New insights into the pathophysiology of achalasia and implications for future treatment

PubMed Central

Furuzawa-Carballeda, Janette; Torres-Landa, Samuel; Valdovinos, Miguel Ángel; Coss-Adame, Enrique; Martín del Campo, Luis A; Torres-Villalobos, Gonzalo

2016-01-01

Idiopathic achalasia is an archetype esophageal motor disorder, causing significant impairment of eating ability and reducing quality of life. The pathophysiological underpinnings of this condition are loss of esophageal peristalsis and insufficient relaxation of the lower esophageal sphincter (LES). The clinical manifestations include dysphagia for both solids and liquids, regurgitation of esophageal contents, retrosternal chest pain, cough, aspiration, weight loss and heartburn. Even though idiopathic achalasia was first described more than 300 years ago, researchers are only now beginning to unravel its complex etiology and molecular pathology. The most recent findings indicate an autoimmune component, as suggested by the presence of circulating anti-myenteric plexus autoantibodies, and a genetic predisposition, as suggested by observed correlations with other well-defined genetic syndromes such as Allgrove syndrome and multiple endocrine neoplasia type 2 B syndrome. Viral agents (herpes, varicella zoster) have also been proposed as causative and promoting factors. Unfortunately, the therapeutic approaches available today do not resolve the causes of the disease, and only target the consequential changes to the involved tissues, such as destruction of the LES, rather than restoring or modifying the underlying pathology. New therapies should aim to stop the disease at early stages, thereby preventing the consequential changes from developing and inhibiting permanent damage. This review focuses on the known characteristics of idiopathic achalasia that will help promote understanding its pathogenesis and improve therapeutic management to positively impact the patient’s quality of life. PMID:27672286

Esophageal achalasia in the Veneto region: epidemiology and treatment. Epidemiology and treatment of achalasia.

PubMed

Gennaro, Nicola; Portale, Giuseppe; Gallo, Costantino; Rocchietto, Stefano; Caruso, Valentina; Costantini, Mario; Salvador, Renato; Ruol, Alberto; Zaninotto, Giovanni

2011-03-01

Achalasia is a rare esophageal motility disorder, incurable but amenable to palliative treatments to relieve dysphagia. Given the rarity of the disease, there is a paucity of data from population-based studies on incidence and outcome of the two treatments most commonly used in clinical practice, i.e., endoscopic pneumatic dilation (PD) and surgical myotomy (SM). A retrospective longitudinal study was conducted on the Veneto region, in north-eastern Italy. All patients with achalasia as their primary diagnosis between 2001 and 2005 were identified and their demographics and treatment details obtained. The overall incidence of achalasia was 1.59 cases/100,000/year. Achalasia patients were mainly seen at University Hospitals. Fifty-five percent of the patients received treatment, 23.3% SM and 31.8% PD. The cumulative risk of any subsequent intervention for achalasia was 20% in treated patients (29.7% in patients treated primarily with PD and 4% in patients treated with SM first). The epidemiology of achalasia in the Veneto Region is in line with the situation reported elsewhere and did not change between 2001 and 2005. Achalasia patients are mostly seen at University Hospitals. We observed a greater risk of subsequent intervention for patients previously treated with PD compared with SM.

[Surgical treatment of achalasia - endoscopic or laparoscopic? : Proposal for a tailored approach].

PubMed

Rahden, B H A von; Filser, J; Al-Nasser, M; Germer, C-T

2017-03-01

Primary idiopathic achalasia is the most common form of the rare esophageal motility disorders. A curative therapy which restores the normal motility does not exist; however, the therapeutic principle of cardiomyotomy according to Ernst Heller leads to excellent symptom control in the majority of cases. The established standard approach is Heller myotomy through the laparoscopic route (LHM), combined with Dor anterior fundoplication for reflux prophylaxis/therapy. At least four meta-analyses of randomized controlled trials (RCTs) have demonstrated superiority of LHM over pneumatic dilation (PD); therefore, LHM should be used as first line therapy (without prior PD) in all operable patients. Peroral endoscopic myotomy (POEM) is a new alternative approach, which enables Heller myotomy to be performed though the endoscopic submucosal route. The POEM procedure has a low complication rate and also leads to good control of dysphagia but reflux rates can possibly be slightly higher (20-30%). Long-term results of POEM are still scarce and the results of the prospective randomized multicenter trial POEM vs. LHM are not yet available; however, POEM seems to be the preferred treatment option for certain indications. Within the framework of the tailored approach for achalasia management of POEM vs. LHM established in Würzburg, we recommend long-segment POEM for patients with type III achalasia (spasmodic) and other hypercontractile motility disorders and potentially type II achalasia (panesophageal compression) with chest pain as the lead symptom, whereas LHM can also be selected for type I. For sigmoid achalasia, especially with siphon-like transformation of the esophagogastric junction, simultaneous hiatal hernia and epiphrenic diverticula, LHM is still the preferred approach. The choice of the procedure for revisional surgery in case of recurrent dysphagia depends on the suspected mechanism (morphological vs. functional/neuromotor).

Achalasia: from diagnosis to management.

PubMed

Vaezi, Michael F; Felix, Valter N; Penagini, Roberto; Mauro, Aurelio; de Moura, Eduardo Guimarães Hourneaux; Pu, Leonardo Zorrón Cheng Tao; Martínek, Jan; Rieder, Erwin

2016-10-01

Achalasia is an esophageal motility disorder associated with abnormalities in peristalsis and lower esophageal sphincter (LES) relaxation. The etiology of the disease remains elusive. It is often misdiagnosed initially as gastroesophageal reflux disease. Patients with achalasia often complain of dysphagia to solids and liquids but may focus on regurgitation as the primary symptom, leading to the early misdiagnosis. Chest pain, weight loss, and occasional vomiting may be additional symptoms encountered in those with achalasia. The disease may be suspected on the basis of clinical presentation, but diagnosis depends on classic findings using high-resolution manometry, showing either failed or simultaneous contractions with associated normal or high LES pressures with no or incomplete relaxation with swallows. There are no cures for achalasia, and, in most patients, treatments have to be repeated over time. Definitive treatment options in achalasia include pneumatic dilation, surgical myotomy, and the new technique of per-oral endoscopic myotomy. Botulinum toxin (Botox) or other medical therapies are often reserved for those who cannot have definitive therapies owing to comorbid conditions. © 2016 New York Academy of Sciences.

Pathogenesis of achalasia cardia.

PubMed

Ghoshal, Uday C; Daschakraborty, Sunil B; Singh, Renu

2012-06-28

Achalasia cardia is one of the common causes of motor dysphagia. Though the disease was first described more than 300 years ago, exact pathogenesis of this condition still remains enigmatic. Pathophysiologically, achalasia cardia is caused by loss of inhibitory ganglion in the myenteric plexus of the esophagus. In the initial stage, degeneration of inhibitory nerves in the esophagus results in unopposed action of excitatory neurotransmitters such as acetylcholine, resulting in high amplitude non-peristaltic contractions (vigorous achalasia); progressive loss of cholinergic neurons over time results in dilation and low amplitude simultaneous contractions in the esophageal body (classic achalasia). Since the initial description, several studies have attempted to explore initiating agents that may cause the disease, such as viral infection, other environmental factors, autoimmunity, and genetic factors. Though Chagas disease, which mimics achalasia, is caused by an infective agent, available evidence suggests that infection may not be an independent cause of primary achalasia. A genetic basis for achalasia is supported by reports showing occurrence of disease in monozygotic twins, siblings and other first-degree relatives and occurrence in association with other genetic diseases such as Down's syndrome and Parkinson's disease. Polymorphisms in genes encoding for nitric oxide synthase, receptors for vasoactive intestinal peptide, interleukin 23 and the ALADIN gene have been reported. However, studies on larger numbers of patients and controls from different ethnic groups are needed before definite conclusions can be obtained. Currently, the disease is believed to be multi-factorial, with autoimmune mechanisms triggered by infection in a genetically predisposed individual leading to degeneration of inhibitory ganglia in the wall of the esophagus.

Pathogenesis of achalasia cardia

PubMed Central

Ghoshal, Uday C; Daschakraborty, Sunil B; Singh, Renu

2012-01-01

Achalasia cardia is one of the common causes of motor dysphagia. Though the disease was first described more than 300 years ago, exact pathogenesis of this condition still remains enigmatic. Pathophysiologically, achalasia cardia is caused by loss of inhibitory ganglion in the myenteric plexus of the esophagus. In the initial stage, degeneration of inhibitory nerves in the esophagus results in unopposed action of excitatory neurotransmitters such as acetylcholine, resulting in high amplitude non-peristaltic contractions (vigorous achalasia); progressive loss of cholinergic neurons over time results in dilation and low amplitude simultaneous contractions in the esophageal body (classic achalasia). Since the initial description, several studies have attempted to explore initiating agents that may cause the disease, such as viral infection, other environmental factors, autoimmunity, and genetic factors. Though Chagas disease, which mimics achalasia, is caused by an infective agent, available evidence suggests that infection may not be an independent cause of primary achalasia. A genetic basis for achalasia is supported by reports showing occurrence of disease in monozygotic twins, siblings and other first-degree relatives and occurrence in association with other genetic diseases such as Down’s syndrome and Parkinson’s disease. Polymorphisms in genes encoding for nitric oxide synthase, receptors for vasoactive intestinal peptide, interleukin 23 and the ALADIN gene have been reported. However, studies on larger numbers of patients and controls from different ethnic groups are needed before definite conclusions can be obtained. Currently, the disease is believed to be multi-factorial, with autoimmune mechanisms triggered by infection in a genetically predisposed individual leading to degeneration of inhibitory ganglia in the wall of the esophagus. PMID:22791940

Conservative management of esophageal perforations during pneumatic dilation for idiopathic esophageal achalasia.

PubMed

Vanuytsel, Tim; Lerut, Toni; Coosemans, Willy; Vanbeckevoort, Dirk; Blondeau, Kathleen; Boeckxstaens, Guy; Tack, Jan

2012-02-01

Esophageal perforation is the most serious adverse event of pneumatic dilation (PD) for achalasia; it is usually managed by surgical repair. We investigated risk factors for esophageal perforation after PD and evaluated safety and long-term outcome of nonsurgical management strategies. We analyzed medical records of patients with achalasia who were treated with PD from 1992-2010 at the University Hospital Gasthuisberg in Leuven, Belgium; all patients with esophageal perforation were contacted to determine long-term outcomes. Achalasia outcomes were assessed by using the Vantrappen criteria. Of 830 PD procedures performed on 372 patients with manometry-confirmed achalasia (57 ± 1 years, 51% male), 16 were complicated by transmural esophageal perforation (4.3% of patients, 1.9% of dilations). Age >65 years was the only significant risk factor for complications (odds ratio, 3.5; 95% confidence interval, 1.2-10.2). All patients were treated conservatively with broad-spectrum antibiotics and nothing by mouth. In 6 patients (38%) the clinical course was further complicated by a pleural effusion, which required a drain in 4 patients. One patient (6%) died of mediastinal hemorrhage within 12 hours after PD. Patients with complications were discharged after 19 ± 2.3 days, compared with 4 ± 0.2 days for those without complications (P < .0001). Long-term outcomes (mean follow-up, 84 ± 14 months) were determined for 12 patients (75%); 11 had excellent or good outcomes (69%), and 1 had a moderate outcome (6%). Age >65 years is a significant risk factor for esophageal perforation after PD. Nonsurgical management of transmural esophageal tears is feasible, with favorable short-term and long-term outcomes, but is not devoid of complications. Copyright © 2012 AGA Institute. Published by Elsevier Inc. All rights reserved.

High-resolution manometry findings in patients with achalasia and massive dilated megaesophagus.

PubMed

Menezes, M A; Andolfi, C; Herbella, F A M; Patti, M G

2017-05-01

Achalasia may present in a non-advanced or an advanced (end stage) stage based on the degree of esophageal dilatation. Manometric parameters and esophageal caliber may be prognostic for the outcome of treatment. The correlation between manometry and disease stage has not been yet fully studied. This study aims to describe high-resolution manometry findings in patients with achalasia and massive dilated megaesophagus. Eighteen patients (mean age 61 years, 55% females) with achalasia and massive dilated megaesophagus, as defined by a maximum esophageal dilatation >10 cm at the barium esophagram, were studied. Achalasia was considered secondary to Chagas' disease in 14 (78%) of the patients and idiopathic in the remaining. All patients underwent high-resolution manometry. Upper esophageal sphincter was hypotonic and had impaired relaxation in the majority of patients. Aperistalsis was seen in all patients with an equal distribution of Chicago type I and type II. No type III was noticed. Lower esophageal sphincter did not have a characteristic manometric pattern. In 50% of the cases, the manometry catheter was not able to reach the stomach. Our results did not show a manometric pattern in patients with achalasia and massive dilated esophagus. © The Authors 2017. Published by Oxford University Press on behalf of International Society for Diseases of the Esophagus. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Laparoscopic Heller myotomy is not superior to pneumatic dilation in the management of primary achalasia

PubMed Central

Cheng, Ji-Wei; Li, Yin; Xing, Wen-Qun; Lv, Hong-Wei; Wang, Hao-Ran

2017-01-01

Abstract Background: Achalasia is an esophageal motility disorder, of unknown cause, which results in increased lower esophageal sphincter tone and symptoms of difficulty swallowing. Current major therapeutic options include laparoscopic Heller myotomy (LHM) and pneumatic dilation (PD). We undertake a systematic review comparing the efficacy and safety of these 2 treatments in the treatment of esophageal achalasia. Methods: PubMed, Embase, and Cochrane Central Register of Controlled Trials were searched for randomized controlled trial investigating LHM versus PD in the treatment of primary achalasia. The primary outcome was symptom remission rates. The Mantel–Haenszel method with fixed-effect or random-effects model was used to calculate relative risks and 95% confidence intervals (CIs). Results: Five studies involving 498 participants were included. The cumulative remission rate was significantly higher with LHM at 3 months and 1 year (short-term), with a risk ratio of 1.16 (95% CI 1.01–1.35, P = 0.04) and 1.14 (95% CI 1.02–1.27, P = 0.02), respectively. There were no significant differences between LHM and PD in 2-year and 5-year remission rate (long-term), with a risk ratio of 1.05 (95% CI 0.91–1.22, P = 0.49) and 1.17 (95% CI 0.84–1.64, P = 0.34), respectively. Rates of major mucosal tears requiring subsequent intervention with LHM were significantly lower than those of esophageal perforation with PD requiring postprocedural medical or surgical therapy, with a risk ratio of 0.25 (95% CI 0.08–0.81, P = 0.02). Postprocedural rates of gastroesophageal reflux, lower esophageal sphincter pressures, and quality of life scores did not differ in trials with sufficient data. Conclusions: There were no significant differences between LHM and PD in 2-year and 5-year remission rate. This study indicates that either treatment can be proposed as initial treatment for achalasia. PMID:28207499

Diagnosis and management of esophageal achalasia.

PubMed

Stavropoulos, Stavros N; Friedel, David; Modayil, Rani; Parkman, Henry P

2016-09-13

Achalasia is a rare esophageal motility disorder that is usually idiopathic in origin. It is characterized by dysphagia, and patients often have chest pain, regurgitation, weight loss, and an abnormal barium radiograph showing esophageal dilation with narrowing at the gastroesophageal junction. Abnormal or absent esophageal peristalsis and impaired relaxation of the lower esophageal sphincter (LES) are typically seen on esophageal manometry. The advent of high resolution manometry (HRM) has allowed more precise diagnosis of achalasia, subtype designation, and differentiation from other esophageal motor disorders with an initial seminal publication in 2008 followed by further refinements of what has been termed the Chicago classification. Potential treatments include drugs, endoscopic botulinum toxin injection, balloon dilation, traditional surgery (usually laparoscopic Heller myotomy; LHM), and a novel, less invasive, natural orifice transluminal endoscopic surgery (NOTES) approach to Heller myotomy termed peroral endoscopic myotomy (POEM). The first human POEM was performed in 2008, with the first publication appearing in 2010 and evidence now rapidly accumulating showing POEM to be comparable to traditional surgery in terms of clinical success and radiologic and manometric post-therapy outcomes. This review discusses the diagnosis and management of achalasia with particular emphasis on the recent developments of HRM and POEM, which arguably represent the most important advances in the field since the advent of laparoscopic Heller myotomy in the 1990s. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

The Pathogenesis and Management of Achalasia: Current Status and Future Directions.

PubMed

Ates, Fehmi; Vaezi, Michael F

2015-07-01

Achalasia is an esophageal motility disorder that is commonly misdiagnosed initially as gastroesophageal reflux disease. Patients with achalasia often complain of dysphagia with solids and liquids but may focus on regurgitation as the primary symptom, leading to initial misdiagnosis. Diagnostic tests for achalasia include esophageal motility testing, esophagogastroduodenoscopy and barium swallow. These tests play a complimentary role in establishing the diagnosis of suspected achalasia. High-resolution manometry has now identified three subtypes of achalasia, with therapeutic implications. Pneumatic dilation and surgical myotomy are the only definitive treatment options for patients with achalasia who can undergo surgery. Botulinum toxin injection into the lower esophageal sphincter should be reserved for those who cannot undergo definitive therapy. Close follow-up is paramount because many patients will have a recurrence of symptoms and require repeat treatment.

The Pathogenesis and Management of Achalasia: Current Status and Future Directions

PubMed Central

Ates, Fehmi; Vaezi, Michael F.

2015-01-01

Achalasia is an esophageal motility disorder that is commonly misdiagnosed initially as gastroesophageal reflux disease. Patients with achalasia often complain of dysphagia with solids and liquids but may focus on regurgitation as the primary symptom, leading to initial misdiagnosis. Diagnostic tests for achalasia include esophageal motility testing, esophagogastroduodenoscopy and barium swallow. These tests play a complimentary role in establishing the diagnosis of suspected achalasia. High-resolution manometry has now identified three subtypes of achalasia, with therapeutic implications. Pneumatic dilation and surgical myotomy are the only definitive treatment options for patients with achalasia who can undergo surgery. Botulinum toxin injection into the lower esophageal sphincter should be reserved for those who cannot undergo definitive therapy. Close follow-up is paramount because many patients will have a recurrence of symptoms and require repeat treatment. PMID:26087861

Prosthetic rehabilitation in patient with achalasia cardia — A rare case report

PubMed Central

Kondaka, Sudheer; Rao, Bathala L.; Reddy, Juturi R.; Akula, Rajesh

2016-01-01

Achalasia Cardia is an idiopathic esophageal motor disorder distinguished by the loss of esophageal peristalsis and insufficient relaxation of the lower esophageal sphincter. The oral manifestation of the disease is dental erosion caused by the regurgitation of the gastric contents and vomiting. A female patient aged 14 years reported to the Department of Prosthodontics, Lenora Institute of Dental Sciences, with complaints concerning aesthetics and pain in relation to several teeth. Patient gave history of loss of tooth structure over the past few years with associated sensitivity and pain in several teeth. Patient also gave history of inability to consume sufficient amount of food owing to the repeated vomiting. This paper aims to present a rare case report of achalasia cardia by rehabiliting the remaining teeth with overlay denture. PMID:27583229

New endoscopic indicator of esophageal achalasia: "pinstripe pattern".

PubMed

Minami, Hitomi; Isomoto, Hajime; Miuma, Satoshi; Kobayashi, Yasutoshi; Yamaguchi, Naoyuki; Urabe, Shigetoshi; Matsushima, Kayoko; Akazawa, Yuko; Ohnita, Ken; Takeshima, Fuminao; Inoue, Haruhiro; Nakao, Kazuhiko

2015-01-01

Endoscopic diagnosis of esophageal achalasia lacking typical endoscopic features can be extremely difficult. The aim of this study was to identify simple and reliable early indicator of esophageal achalasia. This single-center retrospective study included 56 cases of esophageal achalasia without previous treatment. As a control, 60 non-achalasia subjects including reflux esophagitis and superficial esophageal cancer were also included in this study. Endoscopic findings were evaluated according to Descriptive Rules for Achalasia of the Esophagus as follows: (1) esophageal dilatation, (2) abnormal retention of liquid and/or food, (3) whitish change of the mucosal surface, (4) functional stenosis of the esophago-gastric junction, and (5) abnormal contraction. Additionally, the presence of the longitudinal superficial wrinkles of esophageal mucosa, "pinstripe pattern (PSP)" was evaluated endoscopically. Then, inter-observer diagnostic agreement was assessed for each finding. The prevalence rates of the above-mentioned findings (1-5) were 41.1%, 41.1%, 16.1%, 94.6%, and 43.9%, respectively. PSP was observed in 60.7% of achalasia, while none of the control showed positivity for PSP. PSP was observed in 26 (62.5%) of 35 cases with shorter history < 10 years, which usually lacks typical findings such as severe esophageal dilation and tortuosity. Inter-observer agreement level was substantial for food/liquid remnant (k = 0.6861) and PSP (k = 0.6098), and was fair for abnormal contraction and white change. The accuracy, sensitivity, and specificity for achalasia were 83.8%, 64.7%, and 100%, respectively. "Pinstripe pattern" could be a reliable indicator for early discrimination of primary esophageal achalasia.

The Kagoshima consensus on esophageal achalasia.

PubMed

Triadafilopoulos, G; Boeckxstaens, G E; Gullo, R; Patti, M G; Pandolfino, J E; Kahrilas, P J; Duranceau, A; Jamieson, G; Zaninotto, G

2012-05-01

Esophageal achalasia is a primary esophageal motility disorder characterized by lack of peristalsis and a lower esophageal sphincter that fails to relax appropriately in response to swallowing. This article summarizes the most salient issues in the diagnosis and management of achalasia as discussed in a symposium that took place in Kagoshima, Japan, in September 2010 under the auspices of the International Society for Diseases of the Esophagus. © 2011 Copyright the Authors. Journal compilation © 2011, Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.

Barrett's Oesophagus in an Achalasia Patient: Immunological Analysis and Comparison with a Group of Achalasia Patients

PubMed Central

Torres-Landa, Samuel; Coss-Adame, Enrique; Valdovinos, Miguel A.; Alejandro-Medrano, Edgar; Ramos-Ávalos, Bárbara; Martínez-Benítez, Braulio

2016-01-01

The aim of the study was to characterize the presence of diverse CD4 and CD8 T cell subsets and regulatory cells in peripheral blood and lower oesophageal sphincter (LES) from a young patient with BE/achalasia without treatment versus achalasia group. In order to characterize the circulating cells in this patient, a cytometric analysis was performed. LES tissue was evaluated by double-immunostaining procedure. Five healthy blood donors, 5 type achalasia patients, and 5 oesophagus tissue samples (gastrooesophageal junction) from transplant donors were included as control groups. A conspicuous systemic inflammation was determined in BE/achalasia patient and achalasia versus healthy volunteer group. Nonetheless, a predominance of Th22, Th2, IFN-α-producing T cells, Tregs, Bregs, and pDCregs was observed in BE/achalasia patient versus achalasia group. A low percentage of Th1 subset in BE/achalasia versus achalasia group was determined. A noticeable increase in tissue of Th22, Th17, Th2, Tregs, Bregs, and pDCregs was observed in BE/achalasia versus achalasia group. Th1 subset was lower in the BE/achalasia patient versus achalasia group. This study suggests that inflammation is a possible factor in the pathogenesis of BE/achalasia. Further research needs to be performed to understand the specific cause of the correlation between BE and achalasia. PMID:27752370

The feasibility, safety and outcomes of laparoscopic re-operation for achalasia.

PubMed

James, David R C; Purkayastha, Sanjay; Aziz, Omer; Amygdalos, Iakovos; Darzi, Ara W; Hanna, George B; Zacharakis, Emmanouil

2012-05-01

Heller myotomy for achalasia is associated with a recurrence rate of around 10%, thus reoperative surgery is often necessitated. This paper aims to review the available literature on laparoscopic reoperation for achalasia in order to assess its feasibility and effectiveness. A Medline, Embase, Ovid, Cochrane database and Google(TM) Scholar search was performed with the following Mesh terms: "laparoscopic", "redo", "reoperative", "Heller's", "esophagomyotomy" and "achalasia". Outcomes of interest included patient demographics and details of primary procedure, operative details, intra- and post operative complications and symptom scores. Seven studies reported outcomes from 54 cases. Conversion occurred in 7% (4/54) of cases. Thirteen percent (7/54) of patients sustained intra-operative gastric or oesophageal perforation; however these were all noted and repaired intra-operatively leading to no subsequent morbidity. No deaths were reported. Pre- and post operative symptom scores were heterogeneous, however did appear to improve after the procedure. This review demonstrates that laparoscopic reoperation for achalasia is feasible and safe with complication rates comparable to the primary laparoscopic operation. It is recommended that laparoscopic reoperative Heller's myotomy should only be performed by surgeons with special interest in oesophagogastric surgery and adequate experience in laparoscopic surgery for achalasia.

Esophageal Achalasia: Pathophysiology, Clinical Presentation, and Diagnostic Evaluation.

PubMed

Schlottmann, Francisco; Neto, Rafael M L; Herbella, Fernando A M; Patti, Marco G

2018-04-01

Esophageal achalasia is a primary esophageal motility disorder characterized by the absence of esophageal peristalsis and failure of the lower esophageal sphincter to relax in response to swallowing. These abnormalities lead to impaired emptying of food from the esophagus into the stomach with resulting food stasis. Most patients experience severe dysphagia, and regurgitation can lead to aspiration and respiratory problems. Consequently, the quality of life of patients affected by achalasia is severely impacted. A thorough evaluation with upper endoscopy, barium swallow, and esophageal manometry is mandatory to establish the diagnosis and plan the optimal treatment. In selected patients, an ambulatory pH monitoring is recommended to distinguish between gastroesophageal reflux disease and achalasia.

2011 update on esophageal achalasia.

PubMed

Chuah, Seng-Kee; Hsu, Pin-I; Wu, Keng-Liang; Wu, Deng-Chyang; Tai, Wei-Chen; Changchien, Chi-Sin

2012-04-14

There have been some breakthroughs in the diagnosis and treatment of esophageal achalasia in the past few years. First, the introduction of high-resolution manometry with pressure topography plotting as a new diagnostic tool has made it possible to classify achalasia into three subtypes. The most favorable outcome is predicted for patients receiving treatment for type II achalasia (achalasia with compression). Patients with type I(classic achalasia) and type III achalasia (spastic achalasia) experience a less favorable outcome. Second, the first multicenter randomized controlled trial published by the European Achalasia Trial group reported 2-year follow-up results indicating that laparoscopic Heller myotomy was not superior to endoscopic pneumatic dilation (PD). Although the follow-up period was not long enough to reach a convincing conclusion, it merits the continued use of PD as a generally available technique in gastroenterology. Third, the novel endoscopic technique peroral endoscopic myotomy is a promising option for treating achalasia, but it requires increased experience and cautious evaluation. Despite all this good news, the bottom line is a real breakthrough from the basic studies to identify the actual cause of achalasia that may impede treatment success is still anticipated.

2011 update on esophageal achalasia

PubMed Central

Chuah, Seng-Kee; Hsu, Pin-I; Wu, Keng-Liang; Wu, Deng-Chyang; Tai, Wei-Chen; Changchien, Chi-Sin

2012-01-01

There have been some breakthroughs in the diagnosis and treatment of esophageal achalasia in the past few years. First, the introduction of high-resolution manometry with pressure topography plotting as a new diagnostic tool has made it possible to classify achalasia into three subtypes. The most favorable outcome is predicted for patients receiving treatment for type II achalasia (achalasia with compression). Patients with typeI(classic achalasia) and type III achalasia (spastic achalasia) experience a less favorable outcome. Second, the first multicenter randomized controlled trial published by the European Achalasia Trial group reported 2-year follow-up results indicating that laparoscopic Heller myotomy was not superior to endoscopic pneumatic dilation (PD). Although the follow-up period was not long enough to reach a convincing conclusion, it merits the continued use of PD as a generally available technique in gastroenterology. Third, the novel endoscopic technique peroral endoscopic myotomy is a promising option for treating achalasia, but it requires increased experience and cautious evaluation. Despite all this good news, the bottom line is a real breakthrough from the basic studies to identify the actual cause of achalasia that may impede treatment success is still anticipated. PMID:22529685

Update on therapeutic interventions for the management of achalasia.

PubMed

Gunasingam, Nishmi; Perczuk, Adam; Talbot, Michael; Kaffes, Arthur; Saxena, Payal

2016-08-01

Achalasia is a primary esophageal motility disorder. It is the absence of peristalsis in the esophageal body and inability of the lower esophageal sphincter to relax, which characterizes this rare condition. Its features typically include dysphagia, regurgitation, chest pain, and weight loss. The ultimate goal in treating achalasia is to relieve the patient's symptoms, improve esophageal emptying, and prevent further dilatation of the esophagus. Current treatment modalities targeted at achalasia include pharmacological therapy, endoscopic therapy, and surgery. This review focuses on the current therapeutic options and explores the role of peroral endoscopic myotomy in the management armamentarium. © 2016 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.

Reoperations for esophageal achalasia.

PubMed

Omura, Nobuo; Kashiwagi, Hideyuki; Yano, Fumiaki; Tsuboi, Kazuto; Yanaga, Katsuhiko

2012-11-01

To define the factors predisposing to recurrence and evaluate the results of reoperations for achalasia. We reviewed the medical records of ten patients (4 men and 6 women; mean age, 51.5 ± 11.0 years), who underwent reoperations for achalasia between August 1994 and August 2010. The primary surgical procedures were Heller-Dor (HD) cardioplasty in nine patients and Heller myotomy in one patient. The factors contributing to failure of the primary operation included inadequate myotomy (n = 2), recurrent adhesion after myotomy (n = 2), reflux esophagitis (n = 2), difficulty in passage caused by tortuosity of the esophagus (n = 2), difficulty in passage through the thoracic esophagus (n = 1), and severe chest pain (n = 1). The reoperations included repeated HD procedures (n = 4), repair of an esophageal hiatal hernia (n = 2), thoracic esophageal myotomy (n = 2), straightening of the lower esophagus with gastropexy (n = 1), and subtotal esophagectomy (n = 1). The success rate of the reoperations for resolving symptoms was 90 % (9 patients). Selecting surgical procedures based on the causes and conditions of recurrence led to symptomatic improvement and acceptable outcomes.

Laparoscopic Heller Myotomy for Achalasia Technical Aspects.

PubMed

Schlottmann, Francisco; Allaix, Marco E; Patti, Marco G

2018-04-01

Esophageal achalasia is a primary esophageal motility disorder defined by the lack of esophageal peristalsis, and by a lower esophageal sphincter that fails to relax in response to swallowing. Patients' symptoms include dysphagia, regurgitation, aspiration, heartburn, and chest pain. Achalasia is a chronic condition without cure, and treatment options are aimed at providing symptomatic relief, improving esophageal emptying, and preventing the development of megaesophagus. Presently, a laparoscopic Heller myotomy with a partial fundoplication is considered the best treatment modality. A properly executed operation is key for the success of a laparoscopic Heller myotomy.

Outcomes of esophagectomy for esophageal achalasia in the United States.

PubMed

Molena, Daniela; Mungo, Benedetto; Stem, Miloslawa; Feinberg, Richard L; Lidor, Anne O

2014-02-01

While the outcomes after Heller myotomy have been extensively reported, little is known about patients with esophageal achalasia who are treated with esophagectomy. This was a retrospective analysis using the Nationwide Inpatient Sample over an 11-year period (2000-2010). Patients admitted with a primary diagnosis of achalasia who underwent esophagectomy (group 1) were compared to patients with esophageal cancer who underwent esophagectomy (group 2) during the same time period. Primary outcome was in-hospital mortality. Secondary outcomes included length of stay, postoperative complications, and total hospital charges. A propensity-matched analysis was conducted comparing the same outcomes between group 1 and well-matched controls in group 2. Nine hundred sixty-three patients with achalasia and 18,003 patients with esophageal cancer underwent esophagectomy. The propensity matched analysis showed a trend toward a higher mortality in group 2 (7.8 vs. 2.9 %, p = 0.08). Postoperative length of stay and complications were similar in both groups. Total hospital charges were higher for the achalasia group ($115,087 vs. $99, 654.2, p = 0.006). This is the largest study to date examining outcomes after esophagectomy in patients with achalasia. Based on our findings, esophagectomy can be considered a safe option, and surgeons should not be hindered by a perceived notion of prohibitive operative risk in this patient population.

Achalasia: a systematic review.

PubMed

Pandolfino, John E; Gawron, Andrew J

2015-05-12

Achalasia significantly affects patients' quality of life and can be difficult to diagnose and treat. To review the diagnosis and management of achalasia, with a focus on phenotypic classification pertinent to therapeutic outcomes. Literature review and MEDLINE search of articles from January 2004 to February 2015. A total of 93 articles were included in the final literature review addressing facets of achalasia epidemiology, pathophysiology, diagnosis, treatment, and outcomes. Nine randomized controlled trials focusing on endoscopic or surgical therapy for achalasia were included (734 total patients). A diagnosis of achalasia should be considered when patients present with dysphagia, chest pain, and refractory reflux symptoms after an endoscopy does not reveal a mechanical obstruction or an inflammatory cause of esophageal symptoms. Manometry should be performed if achalasia is suspected. Randomized controlled trials support treatments focused on disrupting the lower esophageal sphincter with pneumatic dilation (70%-90% effective) or laparoscopic myotomy (88%-95% effective). Patients with achalasia have a variable prognosis after endoscopic or surgical myotomy based on subtypes, with type II (absent peristalsis with abnormal pan-esophageal high-pressure patterns) having a very favorable outcome (96%) and type I (absent peristalsis without abnormal pressure) having an intermediate prognosis (81%) that is inversely associated with the degree of esophageal dilatation. In contrast, type III (absent peristalsis with distal esophageal spastic contractions) is a spastic variant with less favorable outcomes (66%) after treatment of the lower esophageal sphincter. Achalasia should be considered when dysphagia is present and not explained by an obstruction or inflammatory process. Responses to treatment vary based on which achalasia subtype is present.

New Endoscopic Indicator of Esophageal Achalasia: “Pinstripe Pattern”

PubMed Central

Minami, Hitomi; Isomoto, Hajime; Miuma, Satoshi; Kobayashi, Yasutoshi; Yamaguchi, Naoyuki; Urabe, Shigetoshi; Matsushima, Kayoko; Akazawa, Yuko; Ohnita, Ken; Takeshima, Fuminao; Inoue, Haruhiro; Nakao, Kazuhiko

2015-01-01

Background and Study Aims Endoscopic diagnosis of esophageal achalasia lacking typical endoscopic features can be extremely difficult. The aim of this study was to identify simple and reliable early indicator of esophageal achalasia. Patients and Methods This single-center retrospective study included 56 cases of esophageal achalasia without previous treatment. As a control, 60 non-achalasia subjects including reflux esophagitis and superficial esophageal cancer were also included in this study. Endoscopic findings were evaluated according to Descriptive Rules for Achalasia of the Esophagus as follows: (1) esophageal dilatation, (2) abnormal retention of liquid and/or food, (3) whitish change of the mucosal surface, (4) functional stenosis of the esophago-gastric junction, and (5) abnormal contraction. Additionally, the presence of the longitudinal superficial wrinkles of esophageal mucosa, “pinstripe pattern (PSP)” was evaluated endoscopically. Then, inter-observer diagnostic agreement was assessed for each finding. Results The prevalence rates of the above-mentioned findings (1–5) were 41.1%, 41.1%, 16.1%, 94.6%, and 43.9%, respectively. PSP was observed in 60.7% of achalasia, while none of the control showed positivity for PSP. PSP was observed in 26 (62.5%) of 35 cases with shorter history < 10 years, which usually lacks typical findings such as severe esophageal dilation and tortuosity. Inter-observer agreement level was substantial for food/liquid remnant (k = 0.6861) and PSP (k = 0.6098), and was fair for abnormal contraction and white change. The accuracy, sensitivity, and specificity for achalasia were 83.8%, 64.7%, and 100%, respectively. Conclusion “Pinstripe pattern” could be a reliable indicator for early discrimination of primary esophageal achalasia. PMID:25664812

Treatment of achalasia in the era of high-resolution manometry

PubMed Central

Torresan, Francesco; Ioannou, Alexandros; Azzaroli, Francesco; Bazzoli, Franco

2015-01-01

Esophageal achalasia is a primary motility disorder characterized by impaired lower esophageal sphincter relaxation and absence of esophageal peristalsis leading to impaired bolus transit, manifested with symptoms such as dysphagia, regurgitation, retrosternal pain, and weight loss. The standard diagnostic tool is esophageal manometry which demonstrates incomplete relaxation of the lower esophageal sphincter and impaired esophageal peristalsis. Recently, a new advanced technique, high-resolution manometry (HRM) with the addition of pressure topography plotting, using multiple sensors to capture the manometric data as a spatial continuum, allows a detailed pressure recording of the esophageal motility. This technique, currently the gold standard for the diagnosis of achalasia, has led to a subclassification of three manometric types that seem to have different responsiveness to treatment. Because its pathogenesis is as yet unknown, achalasia treatment options are not curative. Type II achalasia patients respond better to treatment compared to those with types I and III. Low-risk patients with type I or II achalasia have good outcome with both graded pneumatic dilatations and laparoscopic Heller myotomy, while type III achalasia patients respond better to laparoscopic Heller myotomy. Although, type III achalasia patients responds less in comparison to types I and II to laparoscopic Heller myotomy. Peroral endoscopic myotomy is a promising new technique but long-term follow-up studies for its safety and efficacy must be performed. This article reviews the current therapeutic options, highlighting the impact of HRM to predict the outcome and the new insights for the treatment of achalasia. PMID:26130022

Hospitalization for esophageal achalasia in the United States.

PubMed

Molena, Daniela; Mungo, Benedetto; Stem, Miloslawa; Lidor, Anne O

2015-09-25

To assess the outcome of different treatments in patients admitted for esophageal achalasia in the United States. This is a retrospective analysis using the Nationwide Inpatient Sample over an 8-year period (2003-2010). Patients admitted with a primary diagnosis of achalasia were divided into 3 groups based on their treatment: (1) Group 1: patients who underwent Heller myotomy during their hospital stay; (2) Group 2: patients who underwent esophagectomy; and (3) Group 3: patients not undergoing surgical treatment. Primary outcome was in-hospital mortality. Secondary outcomes included length of stay (LOS), discharge destination and total hospital charges. Among 27141 patients admitted with achalasia, nearly half (48.5%) underwent Heller myotomy, 2.5% underwent esophagectomy and 49.0% had endoscopic or other treatment. Patients in group 1 were younger, healthier, and had the lowest mortality when compared with the other two groups. Group 2 had the highest LOS and hospital charges among all groups. Group 3 had the highest mortality (1.2%, P < 0.001) and the lowest home discharge rate (78.8%) when compared to the other groups. The most frequently performed procedures among group 3 were esophageal dilatation (25.9%) and injection (13.3%). Among patients who died in this group the most common associated morbidities included acute respiratory failure, sepsis and aspiration pneumonia. Surgery for achalasia carries exceedingly low mortality in the modern era; however, in complicated patients, even less invasive treatments are burdened by significant mortality and morbidity.

Novel association of achalasia with hereditary sensory and motor neuropathy with sensorineural deafness.

PubMed

Asthana, A K; Lubel, J S; Kohn, G P

2016-08-01

Achalasia is a primary esophageal motility disorder. Unlike diffuse esophageal spasm, it has not previously been described in association with hereditary sensory and motor neuropathy (HSMN). An 18-year-old-male with HSMN with sensorineural deafness presented with a 2-day history of dysphagia to solids and liquids. Achalasia was diagnosed after extensive investigations, and his symptoms resolved with endoscopic and definitive surgical management. His monozygotic twin brother had also been diagnosed with HSMN and suffered from chronic dysphagia, which was also subsequently diagnosed with achalasia. This is the first case to illustrate an association between HSMN with sensorineural deafness and achalasia. © 2013 Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.

Hospitalization for esophageal achalasia in the United States

PubMed Central

Molena, Daniela; Mungo, Benedetto; Stem, Miloslawa; Lidor, Anne O

2015-01-01

AIM: To assess the outcome of different treatments in patients admitted for esophageal achalasia in the United States. METHODS: This is a retrospective analysis using the Nationwide Inpatient Sample over an 8-year period (2003-2010). Patients admitted with a primary diagnosis of achalasia were divided into 3 groups based on their treatment: (1) Group 1: patients who underwent Heller myotomy during their hospital stay; (2) Group 2: patients who underwent esophagectomy; and (3) Group 3: patients not undergoing surgical treatment. Primary outcome was in-hospital mortality. Secondary outcomes included length of stay (LOS), discharge destination and total hospital charges. RESULTS: Among 27141 patients admitted with achalasia, nearly half (48.5%) underwent Heller myotomy, 2.5% underwent esophagectomy and 49.0% had endoscopic or other treatment. Patients in group 1 were younger, healthier, and had the lowest mortality when compared with the other two groups. Group 2 had the highest LOS and hospital charges among all groups. Group 3 had the highest mortality (1.2%, P < 0.001) and the lowest home discharge rate (78.8%) when compared to the other groups. The most frequently performed procedures among group 3 were esophageal dilatation (25.9%) and injection (13.3%). Among patients who died in this group the most common associated morbidities included acute respiratory failure, sepsis and aspiration pneumonia. CONCLUSION: Surgery for achalasia carries exceedingly low mortality in the modern era; however, in complicated patients, even less invasive treatments are burdened by significant mortality and morbidity. PMID:26421106

Management of primary achalasia: The role of endoscopy

PubMed Central

Luján-Sanchis, Marisol; Suárez-Callol, Patricia; Monzó-Gallego, Ana; Bort-Pérez, Inmaculada; Plana-Campos, Lydia; Ferrer-Barceló, Luis; Sanchis-Artero, Laura; Llinares-Lloret, María; Tuset-Ruiz, Juan Antonio; Sempere-Garcia-Argüelles, Javier; Canelles-Gamir, Pilar; Medina-Chuliá, Enrique

2015-01-01

Achalasia is an oesophageal motor disorder which leads to the functional obstruction of the lower oesophageal sphincter (LES) and is currently incurable. The main objective of all existing therapies is to achieve a reduction in the obstruction of the distal oesophagus in order to improve oesophageal transit, relieve the symptomatology, and prevent long-term complications. The most common treatments used are pneumatic dilation (PD) and laparoscopic Heller myotomy, which involves partial fundoplication with comparable short-term success rates. The most economic non-surgical therapy is PD, with botulinum toxin injections reserved for patients with a higher surgical risk for whom the former treatment option is unsuitable. A new technology is peroral endoscopic myotomy, postulated as a possible non-invasive alternative to surgical myotomy. Other endoluminal treatments subject to research more recently include injecting ethanolamine into the LES and using a temporary self-expanding metallic stent. At present, there is not enough evidence permitting a routine recommendation of any of these three novel methods. Patients must undergo follow-up after treatment to guarantee that their symptoms are under control and to prevent complications. Most experts are in favour of some form of endoscopic follow-up, however no established guidelines exist in this respect. The prognosis for patients with achalasia is good, although a recurrence after treatment using any method requires new treatment. PMID:26078828

Laparoscopic Heller myotomy can be used as primary therapy for esophageal achalasia regardless of age.

PubMed

Salvador, Renato; Costantini, Mario; Cavallin, Francesco; Zanatta, Lisa; Finotti, Elena; Longo, Cristina; Nicoletti, Loredana; Capovilla, Giovanni; Bardini, Romeo; Zaninotto, Giovanni

2014-01-01

Laparoscopic Heller-Dor surgery is the current treatment of choice for patients with esophageal achalasia, but elderly patients are generally referred for less invasive treatments (pneumatic dilations or botulinum toxin injections). To assess the effect of age on the surgical outcome of patients receiving laparoscopic Heller-Dor as primary treatment. Demographic and clinical findings were prospectively collected on patients undergoing laparoscopic Heller-Dor from 1992 to 2012. Patients were classified in three age brackets: group A (≤45 years), group B (45-70), and group C (≥70). Treatment was defined as a failure if the postoperative symptom score was >10th percentile of the preoperative score (i.e., >8). We consecutively performed the Heller-Dor in 571 achalasia patients, 305 (53.4 %) in group A, 226 (39.6 %) in group B, and 40 (7 %) in group C. The mortality was nil; the conversion and morbidity rates were both 1.1 %. Group C patients had higher preoperative symptom scores (p = 0.02), while the symptom duration was similar in all three groups. Mucosal tears occurred in 17 patients (3 %): 6 (2 %) in group A, 8 (3.5 %) in group B, and 3 (7.5 %) in group C (p = 0.09). The postoperative hospital stay was slightly longer for group C (p = 0.06). The treatment failure rate was quite similar: 31 failures in group A (10.1 %), 19 in group B (8.4 %), and 3 in group C (7.5 %; p = 0.80). These failures were seen more in manometric pattern III (22.2 %, p = 0.002). Laparoscopic Heller-Dor can be used as the first therapeutic approach to achalasia even in elderly patients with an acceptable surgical risk.

Focal achalasia – case report and review of the literature

PubMed Central

TUTUIAN, RADU

2018-01-01

Esophageal achalasia is a primary smooth muscle motility disorder specified by aperistalsis of the tubular esophagus in combination with a poorly relaxing and occasionally hypertensive lower esophageal sphincter (LES). These changes occur secondary to the destruction of the neural network coordinating esophageal peristalsis and LES relaxation (plexus myentericus). There are limited data on segmental involvement of the esophagus in adults. We report on the case of a 54-year-old man who presented initially with complete aperistalsis limited to the distal esophagus. After a primary good response to BoTox-infiltration of the distal esophagus the patient relapsed two years later. The manometric recordings documented now a progression of the disease with a poorly relaxing hypertensive lower esophageal sphincter and complete aperistalsis of the tubular esophagus (type III achalasia according to the Chicago 3.0 classification system). This paper also reviews diagnostic findings (including high resolution manometry, CT scan, barium esophagram, upper endoscopy and upper endoscopic ultrasound data) in patients with achalasia and summarizes the therapeutic options (including pneumatic balloon dilatation, botulinum toxin injection, surgical or endoscopic myotomy). PMID:29440962

An Overview of Achalasia and Its Subtypes

PubMed Central

Patel, Dhyanesh A.; Lappas, Brian M.

2017-01-01

Achalasia is one of the most studied esophageal motility disorders. However, the pathophysiology and reasons that patients develop achalasia are still unclear. Patients often present with dysphagia to solids and liquids, regurgitation, and varying degrees of weight loss. There is significant latency prior to diagnosis, which can have nutritional implications. The diagnosis is suspected based on clinical history and confirmed by esophageal high-resolution manometry testing. Esophagogastroduodenoscopy is necessary to rule out potential malignancy that can mimic achalasia. Recent data presented in abstract form suggest that patients with type II achalasia may be most likely, and patients with type III achalasia may be least likely, to report weight loss compared to patients with type I achalasia. Although achalasia cannot be permanently cured, palliation of symptoms is possible in over 90% of patients with the treatment modalities currently available (pneumatic dilation, Heller myotomy, or peroral endoscopic myotomy). This article reviews the clinical presentation, diagnosis, and management options in patients with achalasia, as well as potential insights into histopathologic differences and nutritional implications of the subtypes of achalasia. PMID:28867969

Finding the Right Treatment for Achalasia Treatment: Risks, Efficacy, Complications.

PubMed

Moonen, An; Boeckxstaens, Guy

2016-12-01

Achalasia is a primary esophageal motor disorder of the esophagus that is characterized by the absence of esophageal peristalsis and a failure of the lower esophageal sphincter (LES) to relax upon swallowing. The defective relaxation leads to symptoms of dysphagia for solids and liquids, regurgitation, aspiration, chest pain, and weight loss. Achalasia is believed to result from a selective loss of enteric inhibitory neurons, most likely due to an autoimmune phenomenon in genetic susceptible individuals. As there is no curative treatment for achalasia, treatment is confined to disruption of the LES to improve bolus passage. The two most commonly used treatment modalities available are the endoscopic pneumodilation (PD) and the surgical laparoscopic Heller myotomy (LHM). A recent European randomized controlled trial showed that both treatment modalities have comparable success rates after a follow-up of at least 5 years. In view of these data, both treatments can be used as an initial therapy in achalasia and the choice should be based on the expertise available. Recently, a new endoscopic technique, peroral endoscopic myotomy (POEM), has been introduced with excellent short-term success rates. However, longer follow-up and data from randomized controlled trials are needed before accepting this technique as a new treatment option for achalasia in clinical practice.

Esophageal achalasia: current diagnosis and treatment.

PubMed

Schlottmann, Francisco; Patti, Marco G

2018-05-27

Esophageal achalasia is a primary esophageal motility disorder of unknown origin, characterized by lack of peristalsis and by incomplete or absent relaxation of the lower esophageal sphincter in response to swallowing. The goal of treatment is to eliminate the functional obstruction at the level of the gastroesophageal junction Areas covered: This comprehensive review will evaluate the current literature, illustrating the diagnostic evaluation and providing an evidence-based treatment algorithm for this disease Expert commentary: Today we have three very effective therapeutic modalities to treat patients with achalasia - pneumatic dilatation, per-oral endoscopic myotomy and laparoscopic Heller myotomy with fundoplication. Treatment should be tailored to the individual patient, in centers where a multidisciplinary approach is available. Esophageal resection should be considered as a last resort for patients who have failed prior therapeutic attempts.

Clinical management of achalasia: current state of the art

PubMed Central

Krill, Joseph T; Naik, Rishi D; Vaezi, Michael F

2016-01-01

Achalasia is a primary disorder of esophageal motility. It classically presents with dysphagia to both solids and liquids but may be accompanied by regurgitation and chest pain. The gold standard for the diagnosis of achalasia is esophageal motility testing with manometry, which often reveals aperistalsis of the esophageal body and incomplete lower esophageal sphincter relaxation. The diagnosis is aided by complimentary tests, such as esophagogastroduodenoscopy and contrast radiography. Esophagogastroduodenoscopy is indicated to rule out mimickers of the disease known as “pseudoachalasia” (eg, malignancy). Endoscopic appearance of a dilated esophagus with retained food or saliva and a puckered lower esophageal sphincter should raise suspicion for achalasia. Additionally, barium esophagography may reveal a dilated esophagus with a distal tapering giving it a “bird’s beak” appearance. Multiple therapeutic modalities aid in the management of achalasia, the decision of which depends on operative risk factors. Conventional treatments include medical therapy, botulinum toxin injection, pneumatic dilation, and Heller myotomy. The last two are defined as the most definitive treatment options. New emerging therapies include peroral endoscopic myotomy, placement of self-expanding metallic stents, and endoscopic sclerotherapy. PMID:27110134

Achalasia.

PubMed

Islam, Saleem

2017-04-01

Achalasia is a rare neurogenic motility disorder of the esophagus, occurring in approximately 0.11 cases per 100,000 children. The combination of problems (aperistalsis, hypertensive lower esophageal sphincter (LES), and lack of receptive LES relaxation) results in patients having symptoms of progressive dysphagia, weight loss, and regurgitation. Treatment modalities have evolved over the past few decades from balloon dilation and botulinum toxin injection to laparoscopic Heller myotomy and endoscopic myotomy. Most data on achalasia management is extrapolated to children from adult experience. This article describes understanding of the pathogenesis and discusses newer therapeutic techniques as well as controversies in management. Copyright © 2017 Elsevier Inc. All rights reserved.

Progression of diffuse esophageal spasm to achalasia: incidence and predictive factors.

PubMed

Fontes, L H S; Herbella, F A M; Rodriguez, T N; Trivino, T; Farah, J F M

2013-07-01

The progression of certain primary esophageal motor disorders to achalasia has been documented; however, the true incidence of this decay is still elusive. This study aims to evaluate: (i) the incidence of the progression of diffuse esophageal spasm to achalasia, and (ii) predictive factors to this progression. Thirty-five patients (mean age 53 years, 80% females) with a manometric picture of diffuse esophageal spasm were followed for at least 1 year. Patients with gastroesophageal reflux disease confirmed by pH monitoring or systemic diseases that may affect esophageal motility were excluded. Esophageal manometry was repeated in all patients. Five (14%) of the patients progressed to achalasia at a mean follow-up of 2.1 (range 1-4) years. Demographic characteristics were not predictive of transition to achalasia, while dysphagia (P= 0.005) as the main symptom and the wave amplitude of simultaneous waves less than 50 mmHg (P= 0.003) were statistically significant. In conclusion, the transition of diffuse esophageal spasm to achalasia is not frequent at a 2-year follow-up. Dysphagia and simultaneous waves with low amplitude are predictive factors for this degeneration. © 2012 Copyright the Authors. Journal compilation © 2012, Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.

Heartburn in patients with achalasia.

PubMed Central

Spechler, S J; Souza, R F; Rosenberg, S J; Ruben, R A; Goyal, R K

1995-01-01

Heartburn, the main symptom of gastrooesophageal reflux disease (GORD), might be expected to occur infrequently in achalasia, a disorder characterised by a hypertensive lower oesophageal sphincter (LOS) that fails to relax. Nevertheless, it is often described by patients with achalasia. The medical records of 32 patients with untreated achalasia who complained of heartburn, and of 35 similar patients who denied the symptom, were reviewed to explore the implications of heartburn in this condition. Data on endoscopic and manometric findings, and on the onset and duration of oesophageal symptoms were collected. Three patterns of heartburn were observed: (1) in 8 patients (25%) the onset of heartburn followed the onset of dysphagia, (2) in 15 patients (47%) heartburn preceded the onset of dysphagia and persisted as dysphagia progressed, and (3) in 9 patients (28%), heartburn preceded the onset of dysphagia and stopped as dysphagia progressed. The mean (SD) basal LOS pressure in the patients with heartburn (38 (16) mm Hg) was significantly lower than that in patients without the symptom (52 (26) mm Hg); the lowest LOS pressure (29 (11) mm Hg) was observed in the subset of patients whose heartburn preceded the onset of dysphagia and then stopped. It is concluded that patients who have achalasia with heartburn have lower basal LOS pressures than patients who have achalasia without this symptom. In some patients with achalasia, the appearance of dysphagia is heralded by the disappearance of longstanding heartburn. For these patients, it is speculated that achalasia develops in the setting of underlying GORD. PMID:7590421

Update on the endoscopic treatments for achalasia

PubMed Central

Uppal, Dushant S; Wang, Andrew Y

2016-01-01

Achalasia is the most common primary motility disorder of the esophagus and presents as dysphagia to solids and liquids. It is characterized by impaired deglutitive relaxation of the lower esophageal sphincter. High-resolution manometry allows for definitive diagnosis and classification of achalasia, with type II being the most responsive to therapy. Since no cure for achalasia exists, early diagnosis and treatment of the disease is critical to prevent end-stage disease. The central tenant of diagnosis is to first rule out mechanical obstruction due to stricture or malignancy, which is often accomplished by endoscopic and fluoroscopic examination. Therapeutic options include pneumatic dilation (PD), surgical myotomy, and endoscopic injection of botulinum toxin injection. Heller myotomy and PD are more efficacious than pharmacologic therapies and should be considered first-line treatment options. Per oral endoscopic myotomy (POEM) is a minimally-invasive endoscopic therapy that might be as effective as surgical myotomy when performed by a trained and experienced endoscopist, although long-term data are lacking. Overall, therapy should be individualized to each patient’s clinical situation and based upon his or her risk tolerance, operative candidacy, and life expectancy. In instances of therapeutic failure or symptom recurrence re-treatment is possible and can include PD or POEM of the wall opposite the site of prior myotomy. Patients undergoing therapy for achalasia require counseling, as the goal of therapy is to improve swallowing and prevent late manifestations of the disease rather than to restore normal swallowing, which is unfortunately impossible. PMID:27818585

Achalasia following gastro-oesophageal reflux.

PubMed Central

Smart, H L; Mayberry, J F; Atkinson, M

1986-01-01

Five patients initially presenting with symptomatic gastro-oesophageal reflux, proven by radiology or pH monitoring, subsequently developed achalasia, confirmed by radiology and manometry, after an interval of 2-10 years. During this period dysphagia, present as a mild and intermittent symptom accompanying the initial reflux in 3 of the 5, became severe and resulted in oesophageal stasis of food in all. Three of the 5 had a demonstrable hiatal hernia. In none was reflux a troublesome symptom after Rider-Moeller dilatation or cardiomyotomy undertaken for the achalasia. Gastro-oesophageal reflux does not protect against the subsequent development of achalasia. It is suggested that the autonomic damage eventually leading to achalasia may in its initial phases cause gastro-oesophageal reflux. Images Figure 1. A Figure 1. B Figure 2. PMID:3950898

[Surgical treatment of esophageal achalasia--20 years experience].

PubMed

Yaramov, N; Sokolov, M; Angelov, K; Toshev, S; Petrov, B

2009-01-01

Achalasia comes from a Greek word that means "failure to relax." Cardiospasm and achalasia refer to the same condition. This report addresses esophageal achalasia--its history, diagnosis, pathophysiology, and treatment options. We report our experience in treating this disorder surgically using modified Heller myotomy combined or not with partial gastric fundoplication. 47 patients with achalasia surgically operated in 20-years period are reported by authors. These features make it reasonable to reasses the relative indications for surgery and nonsurgical therapy in achalasia of the esophagus.

Minimally invasive surgery for esophageal achalasia.

PubMed

Chen, Huan-Wen; Du, Ming

2016-07-01

Esophageal achalasia is due to the esophagus of neuromuscular dysfunction caused by esophageal functional disease. Its main feature is the lack of esophageal peristalsis, the lower esophageal sphincter pressure and to reduce the swallow's relaxation response. Lower esophageal muscular dissection is one of the main ways to treat esophageal achalasia. At present, the period of muscular layer under the thoracoscope esophagus dissection is one of the treatment of esophageal achalasia. Combined with our experience in minimally invasive esophageal surgery, to improved incision and operation procedure, and adopts the model of the complete period of muscular layer under the thoracoscope esophagus dissection in the treatment of esophageal achalasia.

Minimally invasive surgery for esophageal achalasia

PubMed Central

Chen, Huan-Wen

2016-01-01

Esophageal achalasia is due to the esophagus of neuromuscular dysfunction caused by esophageal functional disease. Its main feature is the lack of esophageal peristalsis, the lower esophageal sphincter pressure and to reduce the swallow’s relaxation response. Lower esophageal muscular dissection is one of the main ways to treat esophageal achalasia. At present, the period of muscular layer under the thoracoscope esophagus dissection is one of the treatment of esophageal achalasia. Combined with our experience in minimally invasive esophageal surgery, to improved incision and operation procedure, and adopts the model of the complete period of muscular layer under the thoracoscope esophagus dissection in the treatment of esophageal achalasia. PMID:27499977

The Treatment of Achalasia in Obese Patients.

PubMed

Wesp, Julie A; Farrell, Timothy M

2018-04-01

Epidemiological studies have demonstrated that obesity is frequently associated with esophageal motility disorders. Morbid obesity and achalasia may coexist in the same patient. The management of the morbidly obese patient with achalasia is complex and the most effective treatment remains controversial. The aim of this study is to review the pathophysiology, clinical presentation, diagnostic evaluation, and treatment of achalasia in morbidly obese patients. PubMed search from January 1990 to July 2017, including the following terms: achalasia, morbid obesity, bariatric, and treatment. Achalasia in the setting of morbid obesity may be successfully treated by endoscopic or surgical methods. Surgeons may choose to add a bariatric procedure, with various strategies present in the literature. A review of the present literature suggests that the preferred approach to achalasia in the morbidly obese patient is to address both disease processes simultaneously with a laparoscopic Heller myotomy and a Roux-en-Y gastric bypass. Roux-en-Y gastric bypass is cited by most experts as the bariatric procedure of choice, given its antireflux benefits. A well-powered study, comparing the various approaches to the treatment of achalasia in the setting of morbid obesity, is required to establish a consensus.

Non-surgical treatment of esophageal achalasia

PubMed Central

Annese, Vito; Bassotti, Gabrio

2006-01-01

Esophageal achalasia is an infrequent motility disorder characterized by a progressive stasis and dilation of the oesophagus; with subsequent risk of aspiration, weight loss, and malnutrition. Although the treatment of achalasia has been traditionally based on a surgical approach, especially with the introduction of laparoscopic techniques, there is still some space for a medical approach. The present article reviews the non-surgical therapeutic options for achalasia. PMID:17007039

Intra and post-operative complications of esophageal achalasia.

PubMed

Pugliese, Luigi; Peri, Andrea; Tinozzi, Francesco Paolo; Zonta, Sandro; di Stefano, Michele; Meloni, Federica; Pietrabissa, Andrea

2013-01-01

To evaluate and discuss all the potential complications affecting morbidity of patients treated with surgery for primary achalasia. A review of the available English literature published to date has been conducted. All articles reporting surgical experience in achalasia were examined and then were selected only those specifically inherent to the topic at issue. Mucosal perforation is the main intra-operative complication while persistence or recurrence of the disease and gastro-esophageal reflux are those mostly affecting patients afterwards, even at long-term follow-up. A few other less common morbidities, as well as the technical considerations useful to minimize and manage each complication mentioned, are reported. Minimally invasive surgery for achalasia consent to treat patients with a low rate of perioperative complications that can be managed with conservative approach in the majority of cases. Risk of esophageal cancer exists in these patients and remains although surgical therapy. Laparoscopic Heller myotomy along with partial fundoplication is a safe and effective procedure that should be considered as the treatment of choice at first evaluation of achalasic patients rather than endoscopic techniques. Robotic technology may add further contribution in diminishing perioperative complications.

[Experience of the surgical management of the esophageal achalasia in a tertiary care hospital].

PubMed

Barajas-Fregoso, Elpidio Manuel; Romero-Hernández, Teodoro; Sánchez-Fernández, Patricio Rogelio; Fuentes-Orozco, Clotilde; González-Ojeda, Alejandro; Macías-Amezcua, Michel Dassaejv

2015-01-01

Achalasia is a primary esophageal motor disorder. The most common symptoms are: dysphagia, chest pain, reflux and weight loss. The esophageal manometry is the standard for diagnosis. The aim of this paper is to determine the effectiveness of the surgical management in patients with achalasia in a tertiary care hospital. A case series consisting of achalasia patients, treated surgically between January and December of 2011. Clinical charts were reviewed to obtain data and registries of the type of surgical procedure, morbidity and mortality. Fourteen patients were identified, with an average age of 49.1 years. The most common symptoms were: dysphagia, vomiting, weight loss and pyrosis. Eight open approaches were performed and six by laparoscopy, with an average length of cardiomyotomy of 9.4 cm. Eleven patients received an antireflux procedure. The effectiveness of procedures performed was 85.7 %. Surgical management offered at this tertiary care hospital does not differ from that reported in other case series, giving effectiveness and safety for patients with achalasia.

Prevalence of gallstones in 1,229 patients submitted to surgical laparoscopic treatment of GERD and esophageal achalasia: associated cholecystectomy was a safe procedure.

PubMed

Sallum, Rubens Antonio Aissar; Padrão, Eduardo Messias Hirano; Szachnowicz, Sergio; Seguro, Francisco C B C; Bianchi, Edno Tales; Cecconello, Ivan

2015-01-01

Association between esophageal achalasia/ gastroesophageal reflux disease (GERD) and cholelithiasis is not clear. Epidemiological data are controversial due to different methodologies applied, the regional differences and the number of patients involved. Results of concomitant cholecistectomy associated to surgical treatment of both diseases regarding safety is poorly understood. To analyze the prevalence of cholelithiasis in patients with esophageal achalasia and gastroesophageal reflux submitted to cardiomyotomy or fundoplication. Also, to evaluate the safety of concomitant cholecistectomy. Retrospective analysis of 1410 patients operated from 2000 to 2013. They were divided into two groups: patients with GERD submitted to laparocopic hiatoplasty plus Nissen fundoplication and patients with esophageal achalasia to laparoscopic cardiomyotomy plus partial fundoplication. It was collected epidemiological data, specific diagnosis and subgroups, the presence or absence of gallstones, surgical procedure, operative and clinical complications and mortality. All groups/subgroups were compared. From 1,229 patients with GERD or esophageal achalasia, submitted to laparoscopic cardiomyotomy or fundoplication, 138 (11.43%) had cholelitiasis, occurring more in females (2.38:1) with mean age of 50,27 years old. In 604 patients with GERD, 79 (13,08%) had cholelitiasis. Lower prevalence occurred in Barrett's esophagus patients 7/105 (6.67%) (p=0.037). In 625 with esophageal achalasia, 59 (9.44%) had cholelitiasis, with no difference between chagasic and idiopathic forms (p=0.677). Complications of patients with or without cholecystectomy were similar in fundoplication and cardiomyotomy (p=0.78 and p=1.00).There was no mortality or complications related to cholecystectomy in this series. Prevalence of cholelithiasis was higher in patients submitted to fundoplication (GERD). Patients with chagasic or idiopatic forms of achalasia had the same prevalence of cholelithiasis. Gallstones

Prevalence of gallstones in 1,229 patients submitted to surgical laparoscopic treatment of GERD and esophageal achalasia: associated cholecystectomy was a safe procedure

PubMed Central

SALLUM, Rubens Antonio Aissar; PADRÃO, Eduardo Messias Hirano; SZACHNOWICZ, Sergio; SEGURO, Francisco C. B. C.; BIANCHI, Edno Tales; CECCONELLO, Ivan

2015-01-01

Background Association between esophageal achalasia/ gastroesophageal reflux disease (GERD) and cholelithiasis is not clear. Epidemiological data are controversial due to different methodologies applied, the regional differences and the number of patients involved. Results of concomitant cholecistectomy associated to surgical treatment of both diseases regarding safety is poorly understood. Aim To analyze the prevalence of cholelithiasis in patients with esophageal achalasia and gastroesophageal reflux submitted to cardiomyotomy or fundoplication. Also, to evaluate the safety of concomitant cholecistectomy. Methods Retrospective analysis of 1410 patients operated from 2000 to 2013. They were divided into two groups: patients with GERD submitted to laparocopic hiatoplasty plus Nissen fundoplication and patients with esophageal achalasia to laparoscopic cardiomyotomy plus partial fundoplication. It was collected epidemiological data, specific diagnosis and subgroups, the presence or absence of gallstones, surgical procedure, operative and clinical complications and mortality. All groups/subgroups were compared. Results From 1,229 patients with GERD or esophageal achalasia, submitted to laparoscopic cardiomyotomy or fundoplication, 138 (11.43%) had cholelitiasis, occurring more in females (2.38:1) with mean age of 50,27 years old. In 604 patients with GERD, 79 (13,08%) had cholelitiasis. Lower prevalence occurred in Barrett's esophagus patients 7/105 (6.67%) (p=0.037). In 625 with esophageal achalasia, 59 (9.44%) had cholelitiasis, with no difference between chagasic and idiopathic forms (p=0.677). Complications of patients with or without cholecystectomy were similar in fundoplication and cardiomyotomy (p=0.78 and p=1.00).There was no mortality or complications related to cholecystectomy in this series. Conclusions Prevalence of cholelithiasis was higher in patients submitted to fundoplication (GERD). Patients with chagasic or idiopatic forms of achalasia had the

Achalasia and chronic opiate use: innocent bystanders or associated conditions?

PubMed

Ravi, K; Murray, J A; Geno, D M; Katzka, D A

2016-01-01

High-resolution manometry identifies three subtypes of achalasia. However, type 3 differs from classic achalasia. Although opiates affect esophageal motility, opiate use and achalasia have not been studied. Patients with a new diagnosis of achalasia at Mayo Clinic Rochester between June 1, 2012 and January 3, 2014 were identified. Clinical records were reviewed to assess symptoms, opiate use, and therapy. Fifty-six patients with achalasia were identified, 14 (25%) were on opiates. Opiate prescription was unrelated to achalasia in all cases, with chronic back and joint pain constituting the majority. Of patients on opiates, five (36%) had type 3 achalasia compared with four (10%) not on opiates (P = 0.02). No patients on opiates had type 1 achalasia. Clinical presentation did not differ with opiates, although those on opiates were more likely to report chest pain (39 vs. 14%, P = 0.05) and less likely to have esophageal dilation (62 vs. 82%, P = 0.13), none with greater than 5-cm diameter. Contractile vigor was greater with opiate use, with distal contractile integral of 7149 versus 2615.5 mmHg/cm/second (P = 0.08). Treatment response was inferior on opiates, with persistent symptoms in 22% compared with 3% without opiates (P = 0.06). Opiate use is common in type 3 achalasia, with the majority of patients on opiates. No patients on opiates were diagnosed with type 1 achalasia. Manometric findings of type 3 achalasia mimic those induced by opiates, suggesting a physiologic mechanism for opiate induced type 3 achalasia. Treatment outcome is inferior with opiates, with opiate cessation perhaps preferable. Further studies assessing opiate use and achalasia are needed. © 2014 International Society for Diseases of the Esophagus.

Laparoscopic Heller-Dor for primary esophageal achalasia: outcomes in 173 patients.

PubMed

Rosati, Riccardo; Fumagalli Romario, Uberto; Ceolin, Martina; Massaron, Simonetta; Peracchia, Alberto

2013-01-01

Laparoscopic Heller myotomy combined with anterior (Dor) fundoplication is the most widely-used surgical procedure for treating esophageal achalasia in Europe From November 1992 through May 2010 we performed laparoscopic Heller-Dor on 173 patients Conversion to laparotomy was required in three cases (1.7%) at the beginning of the experience (for mucosal) perforation which was the most frequent intraoperative complication, managed laparoscopically with the increasing experience. Five (2.9%) cases had minor postoperative complications. Clinical results were satisfactory in 99.4% of cases. One patient (0.6%) had severe persistent dysphagia. Mean esophageal diameter decreased from 50 mm ± 12 (range 20- 90) to 25 mm ± 7 (range 15-80). Lower esophageal sphincter pressure decreased from 32 mmHg (median, range 10- 93) pre-operatively to 11 mmHg (median, range 5-21) at one year follow up and residual pressure from 12 mmHg (median, range 3-30) to 4 mmHg (median, range 1-8). Impedance and pH monitoring showed normal levels in 39/47 (83%) patients who agreed to testing. The good outcomes of this experience, in part due to careful adherence to technical aspects of the operation, confirm that the laparoscopic Heller-Dor is the gold standard surgical treatment for esophageal achalasia.

Esophageal Resection for End-Stage Achalasia.

PubMed

Aiolfi, Alberto; Asti, Emanuele; Bonitta, Gianluca; Siboni, Stefano; Bonavina, Luigi

2018-04-01

Achalasia is a rare disease characterized by impaired lower esophageal sphincter relaxation loss and of peristalsis in the esophageal body. Endoscopic balloon dilation and laparoscopic surgical myotomy have been established as initial treatment modalities. Indications and outcomes of esophagectomy in the management of end-stage achalasia are less defined. A literature search was conducted to identify all reports on esophagectomy for end-stage achalasia between 1987 and 2017. MEDLINE, Embase, and Cochrane databases were consulted matching the terms "achalasia," "end-stage achalasia," "esophagectomy," and "esophageal resection." Seventeen articles met the inclusion criteria and 1422 patients were included in this narrative review. Most of the patients had previous multiple endoscopic and/or surgical treatments. Esophagectomy was performed through a transthoracic (74%) or a transhiatal (26%) approach. A thoracoscopic approach was used in a minority of patients and seemed to be safe and effective. In 95 per cent of patients, the stomach was used as an esophageal substitute. The mean postoperative morbidity rate was 27.1 per cent and the mortality rate 2.1 per cent. Symptom resolution was reported in 75 to 100 per cent of patients over a mean follow-up of 43 months. Only five series including 195 patients assessed the long-term follow-up (>5 years) after reconstruction with gastric or colon conduits, and the results seem similar. Esophagectomy for end-stage achalasia is safe and effective in tertiary referral centers. A thoracoscopic approach is a feasible and safe alternative to thoracotomy and may replace the transhiatal route in the future.

Is Gastroesophageal Reflux Disease and Achalasia Coincident or Not?

PubMed Central

Jung, Da Hyun; Park, Hyojin

2017-01-01

Achalasia and gastroesophageal reflux disease (GERD) are on opposite ends of the spectrum of lower esophageal sphincter dysfunction. Heartburn is the main symptom of GERD. However, heartburn and regurgitation are frequently observed in patients who have achalasia. The diagnosis of achalasia might be delayed because these symptoms are misinterpreted as gastroesophageal reflux. Here, we reviewed the clinical characteristics of patients with the erroneous diagnosis of GERD who actually had untreated achalasia. PMID:27771944

Achalasia-an unnecessary long way to diagnosis.

PubMed

Niebisch, S; Hadzijusufovic, E; Mehdorn, M; Müller, M; Scheuermann, U; Lyros, O; Schulz, H G; Jansen-Winkeln, B; Lang, H; Gockel, I

2017-05-01

Although achalasia presents with typical symptoms such as dysphagia, regurgitation, weight loss, and atypical chest pain, the time until first diagnosis often takes years and is frustrating for patients and nevertheless associated with high costs for the healthcare system. A total of 563 patients were interviewed with confirmed diagnosis of achalasia regarding their symptoms leading to diagnosis along with past clinical examinations and treatments. Included were patients who had undergone their medical investigations in Germany. Overall, 527 study subjects were included (male 46%, female 54%, mean age at time of interview 51 ± 14.8 years). Dysphagia was present in 86.7%, regurgitation in 82.9%, atypical chest pain in 79%, and weight loss in 58% of patients before diagnosis. On average, it took 25 months (Interquartile Range (IQR) 9-65) until confirmation of correct diagnosis of achalasia. Though, diagnosis was confirmed significantly quicker (35 months IQR 9-89 vs. 20 months IQR 8-53; p < 0.01) in the past 15 years. The majority (72.1%) was transferred to three or more specialists. Almost each patient underwent at least one esophagogastroduodenoscopy (94.2%) and one radiological assessment (89.3%). However, esophageal manometry was performed in 70.4% of patients only. The severity of symptoms was independent with regard to duration until first diagnosis (Eckardt score 7.14 ± 2.64 within 12 months vs. 7.29 ± 2.61 longer than 12 months; P = 0.544). Fifty-five percent of the patients primarily underwent endoscopic dilatation and 37% a surgical myotomy. Endoscopic dilatation was realized significantly faster compared to esophageal myotomy (1 month IQR 0-4 vs. 3 months IQR 1-11; p < 0.001). Although diagnosis of achalasia was significantly faster in the past 15 years, it still takes almost 2 years until the correct diagnosis of achalasia is confirmed. Alarming is the fact that although esophageal manometry is known as the gold standard to differentiate primary

Esophageal contractions in type 3 achalasia esophagus: simultaneous or peristaltic?

PubMed Central

Kim, Tae Ho; Patel, Nirali; Ledgerwood-Lee, Melissa

2016-01-01

Absence of peristalsis and impaired relaxation of lower esophageal sphincter are the hallmarks of achalasia esophagus. Based on the pressurization patterns, achalasia has been subdivided into three subtypes. The goal of our study was to evaluate the esophageal contraction pattern and bolus clearance in type 3 achalasia esophagus. High-resolution manometry (HRM) recordings of all patients diagnosed with achalasia esophagus in our center between the years 2011 and 2013 were reviewed. Recordings of 36 patients with type 3 achalasia were analyzed for the characteristics of swallow-induced “simultaneous esophageal contraction.” The HRM impedance recordings of 14 additional patients with type 3 achalasia were analyzed for bolus clearance from the impedance recording. Finally, the HRM impedance along with intraluminal ultrasound imaging was conducted in six patients to further characterize the simultaneous esophageal contractions. Among 187 achalasia patients, 30 were type 1, 121 type 2, and 36 type 3. A total of 434 swallows evaluated in type 3 achalasia patients revealed that 95% of the swallow-induced contractions met criteria for simultaneous esophageal contraction, based on the onset of contraction. Interestingly, the peak and termination of the majority of simultaneous esophageal contractions were sequential. The HRM impedance revealed that 94% of the “simultaneous contractions” were associated with complete bolus clearance. Ultrasound image analysis revealed that baseline muscle thickness of patients in type 3 achalasia is larger than normal but the pattern of axial shortening is similar to that in normal subjects. The majority of esophageal contractions in type 3 achalasia are not true simultaneous contractions because the peak and termination of contraction are sequential and they are associated with complete bolus clearance. PMID:26950858

Esophageal contractions in type 3 achalasia esophagus: simultaneous or peristaltic?

PubMed

Kim, Tae Ho; Patel, Nirali; Ledgerwood-Lee, Melissa; Mittal, Ravinder K

2016-05-01

Absence of peristalsis and impaired relaxation of lower esophageal sphincter are the hallmarks of achalasia esophagus. Based on the pressurization patterns, achalasia has been subdivided into three subtypes. The goal of our study was to evaluate the esophageal contraction pattern and bolus clearance in type 3 achalasia esophagus. High-resolution manometry (HRM) recordings of all patients diagnosed with achalasia esophagus in our center between the years 2011 and 2013 were reviewed. Recordings of 36 patients with type 3 achalasia were analyzed for the characteristics of swallow-induced "simultaneous esophageal contraction." The HRM impedance recordings of 14 additional patients with type 3 achalasia were analyzed for bolus clearance from the impedance recording. Finally, the HRM impedance along with intraluminal ultrasound imaging was conducted in six patients to further characterize the simultaneous esophageal contractions. Among 187 achalasia patients, 30 were type 1, 121 type 2, and 36 type 3. A total of 434 swallows evaluated in type 3 achalasia patients revealed that 95% of the swallow-induced contractions met criteria for simultaneous esophageal contraction, based on the onset of contraction. Interestingly, the peak and termination of the majority of simultaneous esophageal contractions were sequential. The HRM impedance revealed that 94% of the "simultaneous contractions" were associated with complete bolus clearance. Ultrasound image analysis revealed that baseline muscle thickness of patients in type 3 achalasia is larger than normal but the pattern of axial shortening is similar to that in normal subjects. The majority of esophageal contractions in type 3 achalasia are not true simultaneous contractions because the peak and termination of contraction are sequential and they are associated with complete bolus clearance.

Treatments for achalasia in 2017: how to choose among them.

PubMed

Kahrilas, Peter J; Pandolfino, John E

2017-07-01

To review recent advances in achalasia diagnostics and therapeutics. The cardinal feature of achalasia, impaired lower esophageal sphincter (LES) relaxation, can occur in association with varied patterns of esophageal contractility. The Chicago Classification distinguishes among these as follows: without contractility (type I), with panesophageal pressurization (type II), with premature (spastic) distal esophageal contractions (type III), or even with preserved peristalsis [esophagogastric junction (EGJ) outlet obstruction]. Physiological testing also reveals achalasia-like syndromes that also benefit from achalasia therapies. Coincident with this has been the development of peroral endoscopic myotomy (POEM), an endoscopic technique for performing an esophageal myotomy. Hence, the option now exists to either selectively ablate the LES (pneumatic dilation, laparoscopic Heller myotomy, or POEM) or to ablate the sphincter and create a myotomy along some or the entire adjacent smooth muscle esophagus (POEM). Each achalasia syndrome has unique treatment considerations; type II achalasia responds well to all therapies, whereas type III responds best to POEM. Emerging data support the concept that optimal management of achalasia is phenotype-specific, guided by high-resolution manometry, and, in some instance, functional luminal imaging probe studies. This opinion article reviews the varied characteristic and treatment considerations of achalasia syndromes as currently understood.

Endoscopic approaches to treatment of achalasia

PubMed Central

Friedel, David; Modayil, Rani; Iqbal, Shahzad; Grendell, James H.

2013-01-01

Endoscopic therapy for achalasia is directed at disrupting or weakening the lower esophageal sphincter (LES). The two most commonly utilized endoscopic interventions are large balloon pneumatic dilation (PD) and botulinum toxin injection (BTI). These interventions have been extensively scrutinized and compared with each other as well as with surgical disruption (myotomy) of the LES. PD is generally more effective in improving dysphagia in achalasia than BTI, with the latter reserved for infirm older people, and PD may approach treatment results attained with myotomy. However, PD may need to be repeated. Small balloon dilation and endoscopic stent placement for achalasia have only been used in select centers. Per oral endoscopic myotomy is a newer endoscopic modality that will likely change the treatment paradigm for achalasia. It arose from the field of natural orifice transluminal endoscopic surgery and represents a scarless endoscopic approach to Heller myotomy. This is a technique that requires extensive training and preparation and thus there should be rigorous accreditation and monitoring of outcomes to ensure safety and efficacy. PMID:23503707

Surgical Treatment for Achalasia of the Esophagus: Laparoscopic Heller Myotomy

PubMed Central

Torres-Villalobos, Gonzalo; Martin-del-Campo, Luis Alfonso

2013-01-01

Achalasia is an esophageal motility disorder that leads to dysphagia, chest pain, and weight loss. Its diagnosis is clinically suspected and is confirmed with esophageal manometry. Although pneumatic dilation has a role in the treatment of patients with achalasia, laparoscopic Heller myotomy is considered by many experts as the best treatment modality for most patients with newly diagnosed achalasia. This review will focus on the surgical treatment of achalasia, with special emphasis on laparoscopic Heller myotomy. We will also present a brief discussion of the evaluation of patients with persistent or recurrent symptoms after surgical treatment for achalasia and emerging technologies such as LESS, robot-assisted myotomy, and POEM. PMID:24348542

Endoscopic Therapy for Achalasia Before Heller Myotomy Results in Worse Outcomes Than Heller Myotomy Alone

PubMed Central

Smith, C Daniel; Stival, Alessandro; Howell, D Lee; Swafford, Vickie

2006-01-01

Objective: Heller myotomy has been shown to be an effective primary treatment of achalasia. However, many physicians treating patients with achalasia continue to offer endoscopic therapies before recommending operative myotomy. Herein we report outcomes in 209 patients undergoing Heller myotomy with the majority (74%) undergoing myotomy as secondary treatment of achalasia. Methods: Data on all patients undergoing operative management of achalasia are collected prospectively. Over a 9-year period (1994–2003), 209 patients underwent Heller myotomy for achalasia. Of these, 154 had undergone either Botox injection and/or pneumatic dilation preoperatively. Preoperative, operative, and long-term outcome data were analyzed. Statistical analysis was performed with multiple χ2 and Mann-Whitney U analyses, as well as ANOVA. Results: Among the 209 patients undergoing Heller myotomy for achalasia, 154 received endoscopic therapy before being referred for surgery (100 dilation only, 33 Botox only, 21 both). The groups were matched for preoperative demographics and symptom scores for dysphagia, regurgitation, and chest pain. Intraoperative complications were more common in the endoscopically treated group with GI perforations being the most common complication (9.7% versus 3.6%). Postoperative complications, primarily severe dysphagia, and pulmonary complications were more common after endoscopic treatment (10.4% versus 5.4%). Failure of myotomy as defined by persistent or recurrent severe symptoms, or need for additionally therapy including redo myotomy or esophagectomy was higher in the endoscopically treated group (19.5% versus 10.1%). Conclusion: Use of preoperative endoscopic therapy remains common and has resulted in more intraoperative complications, primarily perforation, more postoperative complications, and a higher rate of failure than when no preoperative therapy was used. Endoscopic therapy for achalasia should not be used unless patients are not candidates for

The evolution of the treatment of esophageal achalasia: a look at the last two decades.

PubMed

Allaix, Marco E; Herbella, Fernando A; Patti, Marco G

2012-09-01

Thanks to the development of minimally invasive surgery, the last 20 years have witnessed a revolution in the treatment of benign esophageal disorders, particularly for esophageal achalasia. This has brought a shift in the treatment algorithm of this disease, as today a laparoscopic Heller myotomy with partial fundoplication is considered the primary form of treatment in most Centers in North America. This article reviews the evolution of the treatment of esophageal achalasia during the last two decades, with particular stress on the key technical elements of this operation.

The prevalence of autoimmune disease in patients with esophageal achalasia.

PubMed

Booy, J D; Takata, J; Tomlinson, G; Urbach, D R

2012-04-01

Achalasia is a rare disease of the esophagus that has an unknown etiology. Genetic, infectious, and autoimmune mechanisms have each been proposed. Autoimmune diseases often occur in association with one another, either within a single individual or in a family. There have been separate case reports of patients with both achalasia and one or more autoimmune diseases, but no study has yet determined the prevalence of autoimmune diseases in the achalasia population. This paper aims to compare the prevalence of autoimmune disease in patients with esophageal achalasia to the general population. We retrospectively reviewed the charts of 193 achalasia patients who received treatment at Toronto's University Health Network between January 2000 and May 2010 to identify other autoimmune diseases and a number of control conditions. We determined the general population prevalence of autoimmune diseases from published epidemiological studies. The achalasia sample was, on average, 10-15 years older and had slightly more men than the control populations. Compared to the general population, patients with achalasia were 5.4 times more likely to have type I diabetes mellitus (95% confidence interval [CI] 1.5-19), 8.5 times as likely to have hypothyroidism (95% CI 5.0-14), 37 times as likely to have Sjögren's syndrome (95% CI 1.9-205), 43 times as likely to have systemic lupus erythematosus (95% CI 12-154), and 259 times as likely to have uveitis (95% CI 13-1438). Overall, patients with achalasia were 3.6 times more likely to suffer from any autoimmune condition (95% CI 2.5-5.3). Our findings are consistent with the impression that achalasia's etiology has an autoimmune component. Further research is needed to more conclusively define achalasia as an autoimmune disease. © 2011 Copyright the Authors. Journal compilation © 2011, Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.

Achalasia and thyroid disease: possible autoimmune connection?

PubMed

Quidute, Ana Rosa P; Freitas, Eduardo Vasconcelos de; Lima, Tadeu Gonçalves de; Feitosa, Ana Márcia Lima; Santos, Joyce Paiva dos; Correia, José Walter

2012-12-01

Many cases have been published showing a co-existence of autoimmune thyroid diseases (AITDs) and other autoimmune diseases. About a quarter of patients with achalasia have a concurrent thyroid disease, most commonly associated with hypothyroidism. Although relatively rare, the association of achalasia and hyperthyroidism requires attention. The physiopathology of Grave's Disease (GD) involves B- and T-mediator lymphocytes, which have an affinity for known thyroid antigens: thyroglobulin, thyroid-peroxidase, and thyrotrophin receptor. Currently, however, the real physiopathogenesis of achalasia continues to be unknown. Some important findings are suggestive of an autoimmune mechanism: significant infiltration of the myoenteric plexus by monocytes, presence of the class II-Human Histocompatibility Complex DQwl antigen and antibodies to myoenteric neurons. The present case reports a patient who, despite testing negative for Chagas' disease, had achalasia, progressed to developing significant wasting and worsening of his quality of life, was later diagnosed with hyperthyroidism. After endoscopic esophageal dilatation and radioiodine ablation of the thyroid gland, there was great improvement in the patient clinical condition.

Achalasia: Outcome in children.

PubMed

Meyer, Anell; Catto-Smith, Anthony; Crameri, Joe; Simpson, Di; Alex, George; Hardikar, Winita; Cameron, Donald; Oliver, Mark

2017-02-01

Oesophageal achalasia is well-recognized but relatively rare in children, occasionally appearing as the "triple A" syndrome (with adrenal insufficiency and alacrima). Treatment modalities, as in adult practice, are not curative, often needing further interventions and spurring the search for better management. The outcome for syndromic variants is unknown. We sought to define the efficacy of treatments for children with achalasia with and without triple A syndrome. We conducted a retrospective analysis of presentation and outcomes for 42 children with achalasia presenting over three decades to a major pediatric referral center. Long term impact of the diagnosis was assessed by questionnaire. We identified 42 children including six with triple A syndrome. The median overall age at diagnosis was 10.8 years and median follow-up 1593 days. Initial Heller myotomy in 17 required further interventions in 11 (65%), while initial treatment with botulinum toxin (n = 20) was ultimately followed by myotomy in 17 (85%). Ten out of 35 patients who underwent myotomy required a repeat myotomy (29%). Patients with triple A syndrome developed symptoms earlier, but had delayed diagnosis, were more underweight at diagnosis and at last follow up. Questionnaire results suggested a significant long term deleterious impact on the quality of life of children and their families. Many children with achalasia relapse after initial treatment, undergoing multiple, different procedures, despite which symptoms persist and impact on quality of life. Symptoms develop earlier in patients with triple A syndrome, but the diagnosis is delayed and this has substantial nutritional impact. © 2016 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.

Morphofunctional analysis of experimental model of esophageal achalasia in rats.

PubMed

Sabirov, A G; Raginov, I S; Burmistrov, M V; Chelyshev, Y A; Khasanov, R Sh; Moroshek, A A; Grigoriev, P N; Zefirov, A L; Mukhamedyarov, M A

2010-10-01

We carried out a detailed analysis of rat model of esophageal achalasia previously developed by us. Manifest morphological and functional disorders were observed in experimental achalasia: hyperplasia of the squamous epithelium, reduced number of nerve fibers, excessive growth of fibrous connective tissue in the esophageal wall, high contractile activity of the lower esophageal sphincter, and reduced motility of the longitudinal muscle layer. Changes in rat esophagus observed in experimental achalasia largely correlate with those in esophageal achalasia in humans. Hence, our experimental model can be used for the development of new methods of disease treatment.

Laparoscopic treatment in achalasia of the cardia.

PubMed

Ursut, B; Alecu, L; Tulin, A; Enciu, O

2014-01-01

Achalasia, although a rare disease (an incidence of 1 100 000 individuals each year) is one of the common causes of motor dysphagia and is characterized by loss of peristalsis in the esophageal body and lack of relaxation of the lower esophageal sphincter. The aim of our study was to perform a clinical,therapeutic and evolution evaluation in patients diagnosed with achalasia and operated in our department between 1997 and 2013. We performed a retrospective study using the clinical charts, operatory protocols, imagistic and video database of the 17 patients with achalasia operated in our department. We encountered an equal repartition in women and men and a predominance of urban provenience. Ages were between 24 and 86 years (with an average age of 51). There were two cases of recurrent achalasia at 2, respectively 5 years after the first operation. In all cases, Heller myotomy was used, with the addition of a Dor fundoplication in 12 cases and Toupet fundoplication in five cases, as an antireflux procedure. Mean operation time was 117.6 minutes.There were three iatrogenic perforations of the esophageal mucosa, all of them recognized and treated in the same operative time. No postoperative complications related to the Heller-Dor Heller-Toupet procedure were encountered.The follow-up was between 3 and 72 months. Laparoscopic approach in the treatment of achalasia provides the advantages of minimally invasive surgery, but also and very important, a good visualization of the abdominal esophagus and gastroesophageal junction.Heller esocardiomyotomy is usually associated with anantireflux procedure. A Dor fundoplication is generally used,although the Toupet fundoplication may also be used with the same advantages. It is important to monitor these patients on a yearly basis, knowing the risk of dysplasia carcinoma in achalasia. Celsius.

Esophageal cancer screening in achalasia: is there a consensus?

PubMed

Ravi, K; Geno, D M; Katzka, D A

2015-04-01

Achalasia is an important but relatively uncommon disorder. While highly effective therapeutic options exist, esophageal cancer remains a long-term potential complication. The risk of esophageal cancer in achalasia remains unclear, with current guidelines recommending against routine endoscopic screening. However, given limited data and conflicting opinion, it is unknown whether consensus regarding screening practices in achalasia among experts exists. A 10-question survey to assess screening practices in achalasia was created and distributed to 28 experts in the area of achalasia. Experts were identified based on publications and meeting presentations in the field. Survey responses were received from 17 of 28 (61%) experts. Wide geographic distribution was seen among respondents, with eight (47%) from Europe or Australia, seven (41%) from the United States, and two (12%) from Asia. Screening for esophageal cancer was inconsistent, with nine (53%) experts endorsing the practice and eight (47%) not. Screening practices did not differ among geographic regions. No consensus regarding the risk for esophageal cancer in achalasia was seen, with three experts reporting no increased risk compared with the general population, eight experts a lifetime risk of 0.1-0.5%, three experts a 0.5-1% risk, two experts a 1-2% risk, and one expert a 3-5% risk. However, these differences in perception of risk did not influence screening practices. Upper endoscopy was utilized among all experts who endorsed screening. However, practices still varied with screening commencing at or within 1 year of diagnosis in two practices compared with 5 and 10 years in three respective practices each. Surveillance intervals also varied, performed every 2 years in four practices, every 3 years in four practices, and every 5 years in one practice. Practice variation in the management of achalasia itself was also seen, with initial treatment with Heller myotomy endorsed by eight experts, pneumatic

The muscular expression of RAS in patients with achalasia.

PubMed

Casselbrant, A; Kostic, S; Lönroth, H

2015-09-01

Angiotensin II (AngII) elicits smooth muscle contractions via activation of AngII type 1 receptor (AT1R) in the intestinal wall and in sphincter regions in several species. Achalasia is a rare swallowing disorder and is characterized by a loss of the wave-like contraction that forces food through the oesophagus and a failure of the lower oesophageal sphincter to relax during swallowing. The present study was undertaken to elucidate expression and distribution of a local renin-angiotensin system (RAS) in the muscular layer of distal normal human oesophagus as well as in patients with achalasia using western blot analysis, immunohistochemistry and polymerase chain reaction (PCR). AT1R, together with enzyme renin and cathepsin D expression were decreased in patients with achalasia. In contrast, the mast cells chymase, cathepsin G, neprilysin and the receptor for angiotensin 1-7 peptides, the MAS receptor, were increased in patients with achalasia. The results showed the existence of a local RAS in human oesophageal muscular layer. The enzymes responsible for AngII production are different and there has been a shift in receptor physiology from AT1R to MAS receptor in patients with achalasia. These changes in the RAS might play a significant role in the physiological motor control for patients with achalasia. © The Author(s) 2014.

Long-term outcome of peroral endoscopic myotomy for achalasia treatment in a 9-year-old female patient.

PubMed

Yamashita, Kanefumi; Shiwaku, Hironari; Hirose, Ryuichiro; Kai, Hiroki; Nakashima, Ryo; Kato, Daisuke; Beppu, Richiko; Takeno, Shinsuke; Sasaki, Takamitsu; Nimura, Satoshi; Iwasaki, Akinori; Inoue, Haruhiro; Yamashita, Yuichi

2016-11-01

Achalasia is a primary motility disorder with incomplete lower esophageal sphincter relaxation; it has an annual incidence of 0.11 cases per 100 000 children. Peroral endoscopic myotomy (POEM) is a new endoscopic treatment method for achalasia. Reports about POEM in pediatric patients are rare. We herein report the case of a 9-year-old female patient with achalasia who underwent POEM. The patient underwent endoscopic balloon dilatation because medication was not effective at a previous hospital; however, endoscopic balloon dilatation was not effective either. She then underwent successful POEM upon admission at our hospital. The patient was symptom-free at 2 years postoperatively with no signs of esophagitis in the absence of proton-pump inhibitor therapy. © 2016 Japan Society for Endoscopic Surgery, Asia Endosurgery Task Force and John Wiley & Sons Australia, Ltd.

Functional restoration of the esophagus after peroral endoscopic myotomy for achalasia.

PubMed

Huh, Cheal Wung; Youn, Young Hoon; Chung, Hyunsoo; Lee, Yong Chan; Park, Hyojin

2017-01-01

Peroral endoscopic myotomy (POEM) is a new efficacious treatment option for achalasia. We propose to define "esophageal remodeling" as the functional restoration of the esophagus that involves decreased lower esophageal sphincter (LES) pressure, recovery of esophageal body peristalsis, and reduction of luminal diameter. The aim of this study was to investigate "esophageal remodeling" after POEM for achalasia. We analyzed data from a prospectively collected database of POEM subjects, which included preoperative and 2-month postoperative Eckardt symptom scores, and results from esophageal high resolution manometry (HRM) and barium esophagogram (BE). We recruited 23 patients (13 male; mean age: 53.9 years) whose preoperative and postoperative HRM and BE results were available, from among 30 patients with achalasia who underwent POEM at two institutions between July 2013 and December 2015. All patients achieved clinical treatment success (Eckardt score≤3). Partial recovery of esophageal body peristalsis was noted in 1/5 patients with type I (20%), 6/11 with type II (54.5%), and 7/7 with type III (100%) achalasia after POEM. Pan-esophageal pressurization disappeared after POEM in 10/11 type II achalasia patients. The average diameter of the esophageal body after POEM was significantly decreased in all types of achalasia. POEM provided excellent clinical symptomatic relief and esophageal remodeling in terms of restoration of peristalsis and reduction in diameter of the esophageal body, especially in patients with type III achalasia.

Long-term outcome of peroral endoscopic myotomy for esophageal achalasia in patients with previous Heller myotomy.

PubMed

Kristensen, Helle Ø; Kirkegård, Jakob; Kjær, Daniel Willy; Mortensen, Frank Viborg; Kunda, Rastislav; Bjerregaard, Niels Christian

2017-06-01

Peroral endoscopic myotomy (POEM) is an emerging procedure in the treatment of esophageal achalasia, a primary motility disorder. However, the long-term outcome of POEM in patients, who have previously undergone a Heller myotomy, is unknown. Using a local database, we identified patients with esophageal achalasia, who underwent POEM. We compared patients with a previous Heller myotomy to those, who had received none or only non-surgical therapy prior to the POEM procedure. We conducted follow-up examinations at 3, 12, and 24 months following the procedure. We included 66 consecutive patients undergoing POEM for achalasia, of which 14 (21.2 %) had undergone a prior Heller myotomy. In both groups, the preoperative Eckardt score was 7. Postoperatively, the non-Heller group experienced a more pronounced symptom relief at both 3-, 12-, and 24-month follow-up compared with the Heller group, and there was a tendency for the effect of POEM to reduce over time. We suggest that there is a correlation between preoperative measurements of gastroesophageal sphincter pressures and the chance of a successful POEM. POEM has a place in the treatment of esophageal achalasia in patients with a prior Heller myotomy and persistent symptoms as it is a safe procedure with acceptable long-term results.

Esophageal achalasia: a risk factor for carcinoma. A systematic review and meta-analysis.

PubMed

Tustumi, F; Bernardo, W M; da Rocha, J R M; Szachnowicz, S; Seguro, F C; Bianchi, E T; Sallum, R A A; Cecconello, I

2017-10-01

Achalasia of the cardia is associated with an increased risk of esophageal carcinoma. The real burden of achalasia at the malignancy genesis is still a controversial issue. Therefore, there are no generally accepted recommendations on follow-up evaluation for achalasia patients. This study aims to estimate the risk of esophageal adenocarcinoma and squamous cell carcinoma in achalasia patients. We searched for association between carcinoma and esophageal achalasia in databases up to January 2017 to perform a systematic review and meta-analysis. A total of 1,046 studies were identified from search strategy, of which 40 were selected for meta-analysis. A cumulative number of 11,978 esophageal achalasia patients were evaluated. The incidence of squamous cell carcinoma was 312.4 (StDev 429.16) cases per 100,000 patient-years at risk. The incidence of adenocarcinoma was 21.23 (StDev 31.6) cases per 100,000 patient-years at risk. The prevalence for esophageal carcinoma was 28 carcinoma cases in 1,000 esophageal achalasia patients (CI 95% 2, 39). The prevalence for squamous cell carcinoma was 26 cases in 1,000 achalasia patients (CI 95% 18, 39) and for adenocarcinoma was 4 cases in 1,000 achalasia patients (CI 95% 3, 6).The absolute risk increase for squamous cell carcinoma was 308.1 and for adenocarcinoma was 18.03 cases per 100,000 patients per year. To the best of our knowledge, this is the first meta-analysis estimating the burden of achalasia as an esophageal cancer risk factor. The high increased risk rate for cancer in achalasia patients points to a strict endoscopic surveillance for these patients. Also, the increased risk for developing adenocarcinoma in achalasia patients suggests fundoplication after myotomy, to avoid esophageal reflux and Barret esophagus, a known risk factor for adenocarcinoma. © The Authors 2017. Published by Oxford University Press on behalf of International Society for Diseases of the Esophagus. All rights reserved. For permissions, please

Ling classification describes endoscopic progressive process of achalasia and successful peroral endoscopy myotomy prevents endoscopic progression of achalasia.

PubMed

Zhang, Wen-Gang; Linghu, En-Qiang; Chai, Ning-Li; Li, Hui-Kai

2017-05-14

To verify the hypothesis that the Ling classification describes the endoscopic progressive process of achalasia and determine the ability of successful peroral endoscopic myotomy (POEM) to prevent endoscopic progression of achalasia. We retrospectively reviewed the endoscopic findings, symptom duration, and manometric data in patients with achalasia. A total of 359 patients (197 women, 162 men) with a mean age of 42.1 years (range, 12-75 years) were evaluated. Symptom duration ranged from 2 to 360 mo, with a median of 36 mo. Patients were classified with Ling type I ( n = 119), IIa ( n = 106), IIb ( n = 60), IIc ( n = 60), or III ( n = 14), according to the Ling classification. Of the 359 patients, 349 underwent POEM, among whom 21 had an endoscopic follow-up for more than 2 years. Pre-treatment and post-treatment Ling classifications of these 21 patients were compared. Symptom duration increased significantly with increasing Ling classification (from I to III) ( P < 0.05), whereas lower esophageal sphincter pressure decreased with increasing Ling type (from I to III) ( P < 0.05). There was no difference in sex ratio or onset age among the Ling types, although the age at time of diagnosis was higher in Ling types IIc and III than in Ling types I, IIa, and IIb. Of the 21 patients, 19 underwent high-resolution manometry both before and after treatment. The mean preoperative and postoperative lower esophageal sphincter pressure were 34.6 mmHg (range, 15.3-59.4 mmHg) and 15.0 mmHg (range, 2.1-21.6 mmHg), respectively, indicating a statistically significant decrease after POEM. All of the 21 patients were treated successfully by POEM (postoperative Eckardt score ≤ 3) and still had the same Ling type during a mean follow-up period of 37.8 mo (range, 24-51 mo). The Ling classification represents the endoscopic progressive process of achalasia and may be able to serve as an endoscopic assessment criterion for achalasia. Successful POEM (Eckardt score ≤ 3) seems to have

Functional restoration of the esophagus after peroral endoscopic myotomy for achalasia

PubMed Central

Huh, Cheal Wung; Youn, Young Hoon; Chung, Hyunsoo; Lee, Yong Chan; Park, Hyojin

2017-01-01

Purpose Peroral endoscopic myotomy (POEM) is a new efficacious treatment option for achalasia. We propose to define “esophageal remodeling” as the functional restoration of the esophagus that involves decreased lower esophageal sphincter (LES) pressure, recovery of esophageal body peristalsis, and reduction of luminal diameter. The aim of this study was to investigate “esophageal remodeling” after POEM for achalasia. Materials and methods We analyzed data from a prospectively collected database of POEM subjects, which included preoperative and 2-month postoperative Eckardt symptom scores, and results from esophageal high resolution manometry (HRM) and barium esophagogram (BE). We recruited 23 patients (13 male; mean age: 53.9 years) whose preoperative and postoperative HRM and BE results were available, from among 30 patients with achalasia who underwent POEM at two institutions between July 2013 and December 2015. Results All patients achieved clinical treatment success (Eckardt score≤3). Partial recovery of esophageal body peristalsis was noted in 1/5 patients with type I (20%), 6/11 with type II (54.5%), and 7/7 with type III (100%) achalasia after POEM. Pan-esophageal pressurization disappeared after POEM in 10/11 type II achalasia patients. The average diameter of the esophageal body after POEM was significantly decreased in all types of achalasia. Conclusion POEM provided excellent clinical symptomatic relief and esophageal remodeling in terms of restoration of peristalsis and reduction in diameter of the esophageal body, especially in patients with type III achalasia. PMID:28542509

[Diagnosis and treatment of achalasia cardiae].

PubMed

Belevich, V L; Khokhlov, A V; Eliseev, A V; Ovchinnikov, D V

2014-12-01

Achalasia is a progressive oesophageal motility disorder of unstudied aetiology involving the smooth muscle layer of the oesophagus. Insufficient knowledge of the pathogenesis of achalasia and the lack of clear treatment approaches has led to an increase of advanced disease forms. Common clinical manifestations of the disease are: incomplete LES relaxation, non-specific initial manifestations and as a result late medical seeking. Occurrence of achalasia cardiae is significantly low, therefore it is important to analyse the available clinical data. The article presents an experience of treatment of 30 patients over the last 16 years of observation. The average age of patients was 50, 5 years; insignificant male predominance is noted. III or IV disease state was diagnosed in 86.7% of patients; this diagnosis was confirmed by esophagogastroduodenoscopy. In the group of 24 patients who underwent attempted endoscopic dilatation of the cardiae, most of the included patients with stage II and III disease. Immediate good and satisfactory results of endoscopic treatment were obtained in 20 patients (83.3%), in 4 cases (16.7%) developed recurrent disease.

[Laparoscopic Heller myotomy for esophageal achalasia].

PubMed

Ibáñez, Luis; Butte, Jean Michel; Pimentel, Fernando; Escalona, Alex; Pérez, Gustavo; Crovari, Fernando; Guzmán, Sergio; Llanos, Osvaldo

2007-04-01

Achalasia is characterized by an incomplete relaxation of the lower esophageal sphincter. The best treatment is surgical and the laparoscopic approach may have good results. To assess the results of laparoscopic Heller myotomy among patients with achalasia. Prospective study of patients subjected to a laparoscopic Heller myotomy between 1995 and 2004. Clinical features, early and late operative results were assessed. Twenty seven patients aged 12 to 74 years (12 females) were operated. All had disphagia lasting for a mean of 32 months. Mean lower esophageal sphincter pressure ranged from 18 to 85 mmHg. Eight patients received other treatments prior to surgery but symptoms persisted or reappeared. The preoperative clinical score was 7. No patient died and no procedure had to be converted to open surgery. In a follow up of 21 to 131 months, all patients are satisfied with the surgical results and the postoperative clinical score is 1. Only one patient with a mega esophagus maintained a clinical score of six. In this series of patients, laparoscopic Heller myotomy was an effective and safe treatment for esophageal achalasia.

Therapeutic Outcome of Achalasia Based on High-Resolution Manometry: A Korean Multicenter Study.

PubMed

Lee, Hyuk; Chung, Hyunsoo; Lee, Tae Hee; Hong, Kyoung Sup; Youn, Young Hoon; Park, Jung Ho; Park, Hyung Seok; Park, Hyojin

2017-09-11

Because achalasia subtype is associated with therapeutic response, it is possible that regional differences in subtype distribution could lead to differences in therapeutic outcomes. We aimed to evaluate and compare high-resolution manometry (HRM) profiles among the different subtypes of achalasia and to elucidate predictive factors associated with treatment outcomes. Patients who were diagnosed with achalasia using HRM at 4 Korean university hospitals were retrospectively identified and analyzed. Sixty-four patients with untreated achalasia were divided into 3 subtypes using the Chicago classification system. Clinical characteristics, manometric features, and treatment outcomes were compared. Among 64 patients diagnosed with achalasia, 31 patients were classified as type I, 27 as type II, and 6 as type III. Regarding HRM parameters, there were statistically significant differences in basal lower esophageal sphincter pressure, 4-second-integrated relaxation pressure, residual upper esophageal sphincter pressure, body amplitude, and maximal intrabolus pressure between subtypes. Regarding therapeutic outcome, type II patients (overall success rate of 80.0%) were more likely to respond than type I (55.2%) or type III (33.2%) patients. Multivariate analysis demonstrated that achalasia subtype (type I vs. III, P = 0.072; type II vs. III, P = 0.005), therapeutic modality (dilation vs. pharmacologic, P = 0.013; laparoscopic Heller's myotomy vs. pharmacologic, P = 0.006), and HRM-measured esophageal length (<27.5 vs. ≥27.5 cm, P = 0.014) are independent predictive factors for therapeutic failure. Patients with type II achalasia had better treatment outcomes than patients with other achalasia subtypes. Achalasia subtype, therapeutic modality, and esophageal length are independent predictive factors of therapeutic outcome.

Clinical Practice Update: The Use of Per-Oral Endoscopic Myotomy in Achalasia: Expert Review and Best Practice Advice From the AGA Institute.

PubMed

Kahrilas, Peter J; Katzka, David; Richter, Joel E

2017-11-01

The purpose of this review is to describe a place for per-oral endoscopic myotomy (POEM) among the currently available robust treatments for achalasia. The recommendations outlined in this review are based on expert opinion and on relevant publications from PubMed and EMbase. The Clinical Practice Updates Committee of the American Gastroenterological Association proposes the following recommendations: 1) in determining the need for achalasia therapy, patient-specific parameters (Chicago Classification subtype, comorbidities, early vs late disease, primary or secondary causes) should be considered along with published efficacy data; 2) given the complexity of this procedure, POEM should be performed by experienced physicians in high-volume centers because an estimated 20-40 procedures are needed to achieve competence; 3) if the expertise is available, POEM should be considered as primary therapy for type III achalasia; 4) if the expertise is available, POEM should be considered as treatment option comparable with laparoscopic Heller myotomy for any of the achalasia syndromes; and 5) post-POEM patients should be considered high risk to develop reflux esophagitis and advised of the management considerations (potential indefinite proton pump inhibitor therapy and/or surveillance endoscopy) of this before undergoing the procedure. Copyright © 2017 AGA Institute. Published by Elsevier Inc. All rights reserved.

Radionuclide esophageal transit: an evaluation of therapy in achalasia

DOE Office of Scientific and Technical Information (OSTI.GOV)

McKinney, M.K.; Brady, C.E.; Weiland, F.L.

1983-09-01

We measured quantitative esophageal transit, expressed as percentage of esophageal retention, before and after pneumatic dilatation in two patients with achalasia. In the sitting position they ingested a 500 ml liquid meal containing 500 muCi technetium Tc 99m sulfur colloid. Radioactivity counts of the entire esophagus were plotted at five-minute intervals for 30 minutes. In five normal control subjects the esophagus essentially cleared in less than one minute. Both patients with achalasia had definite retention 30 minutes before dilatation and had quantitative improvement after dilatation. Radionuclide scintigraphic esophageal transit probably correlates better than other parameters with the physiologic degree ofmore » obstruction in achalasia.« less

Endoscopic approach to achalasia

PubMed Central

Müller, Michaela; Eckardt, Alexander J; Wehrmann, Till

2013-01-01

Achalasia is a primary esophageal motor disorder. The etiology is still unknown and therefore all treatment options are strictly palliative with the intention to weaken the lower esophageal sphincter (LES). Current established endoscopic therapeutic options include pneumatic dilation (PD) or botulinum toxin injection. Both treatment approaches have an excellent symptomatic short term effect, and lead to a reduction of LES pressure. However, the long term success of botulinum toxin (BT) injection is poor with symptom recurrence in more than 50% of the patients after 12 mo and in nearly 100% of the patients after 24 mo, which commonly requires repeat injections. In contrast, after a single PD 40%-60% of the patients remain asymptomatic for ≥ 10 years. Repeated on demand PD might become necessary and long term remission can be achieved with this approach in up to 90% of these patients. The main positive predictors for a symptomatic response to PD are an age > 40 years, a LES-pressure reduction to < 15 mmHg and/or an improved radiological esophageal clearance post-PD. However PD has a significant risk for esophageal perforation, which occurs in about 2%-3% of cases. In randomized, controlled studies BT injection was inferior to PD and surgical cardiomyotomy, whereas the efficacy of PD, in patients > 40 years, was nearly equivalent to surgery. A new promising technique might be peroral endoscopic myotomy, although long term results are needed and practicability as well as safety issues must be considered. Treatment with a temporary self expanding stent has been reported with favorable outcomes, but the data are all from one study group and must be confirmed by others before definite recommendations can be made. In addition to its use as a therapeutic tool, endoscopy also plays an important role in the diagnosis and surveillance of patients with achalasia. PMID:23951393

The management of esophageal achalasia: from diagnosis to surgical treatment.

PubMed

Dobrowolsky, Adrian; Fisichella, P Marco

2014-03-01

The goal of this review is to illustrate our approach to patients with achalasia in terms of preoperative evaluation and surgical technique. Indications, patient selection and management are herein discussed. Specifically, we illustrate the pathogenetic theories and diagnostic algorithm with current up-to-date techniques to diagnose achalasia and its manometric variants. Finally, we focus on the therapeutic approaches available today: medical and surgical. A special emphasis is given on the surgical treatment of achalasia and we provide the reader with a detailed description of our pre and postoperative management.

Esophageal motility after peroral endoscopic myotomy for achalasia.

PubMed

Hu, Yue; Li, Meng; Lu, Bin; Meng, Lina; Fan, Yihong; Bao, Haibiao

2016-05-01

Peroral endoscopic myotomy (POEM) has been introduced as a novel endoscopic treatment for achalasia. The aim of this work is to assess the changes in esophageal motility caused by POEM in patients with achalasia. Forty-one patients with achalasia underwent POEM from September 2012 to November 2014. Esophageal motility of all patients was evaluated preoperatively and 1 month after POEM utilizing high-resolution manometry, which was performed with ten water swallows, ten steamed bread swallows, and multiple rapid swallows (MRS). In single swallows, including liquid swallows and bread swallows, all the parameters of lower esophagus sphincter resting pressure (LESP), 4-s integrated relaxation pressure (4sIRP), and intra-bolus pressure (IBP) were decreased between pre- and post-POEM patients (all p < 0.05). Postoperatively, the trend of distal contractile integral (DCI) and distal esophageal peristaltic amplitude declined in subtype II and subtype III (subtype II: p < 0.05; subtype III: p > 0.05), but increased in subtype I (subtype I: p > 0.05). In liquid swallows, the Eckardt score of subtype II patients decreased with DCI, and distal esophageal peristaltic amplitude after POEM was significantly lower compared with those showing increased values of those two parameters (p < 0.05). In MRS, the rate of LES relaxation increased from 66.67 to 95.24%, but without normal response in all achalasia patients. POEM reduces LES pressure in achalasia, and partly restores esophageal motility. POEM displayed varying effect on esophageal motility in patients with different patterns of swallowing. In addition, the changes in parameters associated with esophageal peristalsis correlated with decreases in Eckardt score.

Improvement of respiratory symptoms following Heller myotomy for achalasia.

PubMed

Khandelwal, Saurabh; Petersen, Rebecca; Tatum, Roger; Sinan, Huseyin; Aaronson, Daniel; Mier, Fernando; Martin, Ana V; Pellegrini, Carlos A; Oelschlager, Brant K

2011-02-01

Although patients with achalasia complain mainly of dysphagia, we have observed that they also have a high rate of respiratory problems. We hypothesized that the latter may be due to poor esophageal clearance leading to aspiration. This study examines the effect of Heller myotomy on these symptoms. We studied the course of 111 patients with achalasia who underwent Heller myotomy between 1994 and 2008 and who agreed to participate in this study. All patients completed a questionnaire postoperatively assessing the preoperative and postoperative prevalence and severity of symptoms using visual analog scales. Patients were divided into two groups: one that included all those with respiratory symptoms (dyspnea, hoarseness, cough, wheezing, sore throat, and/or a history of asthma or pneumonia) prior to myotomy and one that included those without those symptoms. All patients presented with dysphagia as their primary complaint, and 63 (57%) reported respiratory symptoms or disease prior to surgery. There were no significant differences in preoperative characteristics between those with and without respiratory manifestations. After a median follow-up of 71 months (range 9-186 months), 55 (87%) patients reported durable improvement of dysphagia. The frequency and severity of all respiratory symptoms decreased significantly. Twenty-four of the 29 patients (82%) who reported a history of pneumonia prior to surgery did not experience recurrent episodes after Heller myotomy. A Heller myotomy is effective in improving esophageal emptying in patients with achalasia. This results in sustained improvement of dysphagia and associated respiratory symptoms/diseases. This suggests that respiratory symptoms/diseases in these patients are likely caused by esophageal retention of food and secretions, and then aspiration.

The Spectrum of Achalasia: Lessons From Studies of Pathophysiology and High-Resolution Manometry

PubMed Central

Kahrilas, Peter J.; Boeckxstaens, Guy

2013-01-01

High-resolution manometry and recently described analysis algorithms, summarized in the Chicago Classification, have increased the recognition of achalasia. It has become apparent that the cardinal feature of achalasia, impaired lower esophageal sphincter relaxation, can occur in several disease phenotypes: without peristalsis, with premature (spastic) distal esophageal contractions, with panesophageal pressurization, or with peristalsis. Any of these phenotypes could indicate achalasia; however, without a disease-specific biomarker, no manometric pattern is absolutely specific. Laboratory studies indicate that achalasia is an autoimmune disease in which esophageal myenteric neurons are attacked in a cell-mediated and antibody-mediated immune response against an uncertain antigen. This autoimmune response could be related to infection of genetically predisposed subjects with herpes simplex virus 1, although there is substantial heterogeneity among patients. At one end of the spectrum is complete aganglionosis in patients with end-stage or fulminant disease. At the opposite extreme is type III (spastic) achalasia, which has no demonstrated neuronal loss but only impaired inhibitory postganglionic neuron function; it is often associated with accentuated contractility and could be mediated by cytokine-induced alterations in gene expression. Distinct from these extremes is progressive plexopathy, which likely arises from achalasia with preserved peristalsis and then develops into type II achalasia and then type I achalasia. Variations in its extent and rate of progression are likely related to the intensity of the cytotoxic T-cell assault on the myenteric plexus. Moving forward, we need to integrate the knowledge we have gained into treatment paradigms that are specific for individual phenotypes of achalasia and away from the one-size-fits-all approach. PMID:23973923

Self-medication of achalasia with cannabis, complicated by a cannabis use disorder.

PubMed

Luquiens, Amandine; Lourenco, Nelson; Benyamina, Amine; Aubin, Henri-Jean

2015-05-28

Achalasia is a rare esophagus motility disorder. Medical, endoscopic and surgical treatments are available, but all endorse high relapse rates. No data has been published to date reporting a therapeutic effect of cannabis use neither in achalasia nor on its influence on manometric measurements. We report the case of a patient diagnosed with achalasia. He could benefit from a large panel of therapeutic interventions, but none of them was effective over the time. He first used cannabis at age 20 and identified benefits regarding achalasia symptoms. He maintained regular moderate cannabis use for 9 years, with minimal digestive inconvenience. A manometry performed without cannabis premedication was realized at age 26 and still found a cardiospasm. Cannabis use could explain the gap between functional symptoms assessment and manometry measurement. Further investigations are warranted to explore a therapeutic effect of cannabis in achalasia and possible influence on outcome measurements.

Current status of achalasia management: a review on diagnosis and treatment.

PubMed

Tuason, Joshua; Inoue, Haruhiro

2017-04-01

Achalasia is a rare esophageal motility disorder that is characterized by loss of peristalsis and failure of relaxation of the lower esophageal sphincter (LES), particularly during swallowing. This review focuses on the diagnosis of esophageal motility disorders as defined by the Chicago Classification ver 3.0, and presents management options with regard to per-oral endoscopic myotomy (POEM) as the treatment of choice. A concise review of literature was performed for articles related to the management of achalasia, and this was contrasted with our institution's current practice. Achalasia is still incompletely understood, and management is focused on establishing a proper diagnosis, and relieving the obstructive symptoms. Achalasia should be considered when dysphagia is present, and not otherwise caused by an obstruction or inflammation, and when criteria is met as per the Chicago Classification ver 3.0. Lowering LES tone and disruption of LES can be accomplished by various methods, most notably pneumatic balloon dilatation and surgical myotomy. POEM has been gaining momentum as a first line therapy for achalasia symptoms, and can be considered an important tool for motility disorders of the esophagus.

New endoscopic classification of the cardiac orifice in esophageal achalasia: Champagne glass sign.

PubMed

Gomi, Kuniyo; Inoue, Haruhiro; Ikeda, Haruo; Bechara, Robert; Sato, Chiaki; Ito, Hiroaki; Onimaru, Manabu; Kitamura, Yohei; Suzuki, Michitaka; Nakamura, Jun; Hata, Yoshitaka; Maruyama, Shota; Sumi, Kazuya; Takahashi, Hiroshi

2016-09-01

Endoscopy, barium esophagram and manometry are used in the diagnosis of achalasia. In the case of early achalasia, characteristic endoscopic findings are difficult to recognize. As a result, the diagnosis of achalasia is often made several years after symptom onset. Therefore, we examined the endoscopic findings of the cardiac orifice in achalasia and propose a new classification. A total of 400 patients with spastic esophageal motility disorders who underwent peroral endoscopic myotomy (POEM) at our hospital between March 2014 and August 2015 were screened for this study. Champagne glass sign (CG) was defined as when the distal end of the lower esophageal sphincter relaxation failure (LESRF) was proximal to the squamocolumnar junction (SCJ) and the SCJ was dilated in the retroflex view. Specifically, CG-1 was defined as a distance from the SCJ to the lower end of LESRF of <1 cm, and CG-2 was defined as a distance ≥1 cm. CG-0 was seen in 73 patients (28.0%), whereas the CG sign was seen in 186 patients (71.3%), of whom 170 (65.1%) were CG-1 and 16 (6.1%) were CG-2. The CG sign is often observed in esophageal achalasia patients. CG-0 (equal to Maki-tsuki) was observed in 28.0% of achalasia patients only. Its absence with dilated SCJ cannot be used to rule out achalasia. Barium esophagram and manometry should be done if esophageal achalasia is strongly suspected. © 2016 Japan Gastroenterological Endoscopy Society.

Per-oral endoscopic myotomy for esophageal achalasia in a case of Allgrove syndrome.

PubMed

Nakamura, Jun; Hikichi, Takuto; Inoue, Haruhiro; Watanabe, Ko; Kikuchi, Hitomi; Takagi, Tadayuki; Suzuki, Rei; Sugimoto, Mitsuru; Konno, Naoki; Waragai, Yuichi; Asama, Hiroyuki; Takasumi, Mika; Sato, Yuki; Irie, Hiroki; Obara, Katsutoshi; Ohira, Hiromasa

2018-01-30

Allgrove syndrome, also known as Triple A syndrome, is a rare autosomal recessive genetic disease characterized by three signs: esophageal achalasia, adrenocorticotropic hormone refractoriness, and alacrima. A 31-year-old male presented to our hospital for treatment of difficulty swallowing caused by esophageal achalasia. Because he had complicating alacrima, a neurologic disease, and a family history of consanguineous marriage, a genetic neurologic disease was suspected. Then, a mutation in the achalasia-addisonianism-alacrima syndrome gene was identified. With the diagnosis of Allgrove syndrome, a per-oral endoscopic myotomy (POEM) was performed for esophageal achalasia. After the POEM, the symptoms and the esophageal pressure findings ameliorated quickly, with no recurrence noted throughout a follow-up of more than 1 year. Here, we report the first case of POEM performed for esophageal achalasia in Allgrove syndrome.

Upper esophageal sphincter abnormalities: frequent finding on high-resolution esophageal manometry and associated with poorer treatment response in achalasia.

PubMed

Chavez, Yamile H; Ciarleglio, Maria M; Clarke, John O; Nandwani, Monica; Stein, Ellen; Roland, Bani C

2015-01-01

Abnormalities of the upper esophageal sphincter (UES) on high-resolution esophageal manometry (HREM) have been observed in both symptomatic and asymptomatic individuals and are often interpreted as incidental findings of unclear clinical significance. Our primary aims were: (1) to assess the frequency of UES abnormalities in consecutive patients referred for HREM studies; and (2) to characterize the demographics, clinical symptoms, and manometric profiles associated with UES abnormalities as compared with those with normal UES function. We performed a retrospective study of 200 consecutive patients referred for HREM. Patients were divided into those with normal and abnormal UES function, including impaired relaxation (residual pressure >12 mm Hg), hypertensive (>104 mm Hg), and hypotensive (<34 mm Hg) resting pressure. Clinical and manometric profiles were compared. A total of 32.5% of patients had UES abnormalities, the majority of which were hypertensive (55.4%). Patients with achalasia were significantly more likely to have UES abnormalities as compared with normal UES function (57.2% vs. 42.9%, P=0.04), with the most frequent abnormality being a hypertensive UES (50%). In addition, patients with impaired lower esophageal sphincter (LES) relaxation (esophagogastric junction outflow obstruction or achalasia) were more likely to have an UES abnormality present as compared with those with normal LES relaxation (53.1% vs. 28.6%, P=0.01). When we assessed for treatment response among patients with achalasia, we found that subjects with evidence of UES dysfunction had significantly worse treatment outcomes as compared with those without UES abnormalities present (20% improved vs. 100%, P=0.015). This remained true even after adjusting for type of treatment received (surgical myotomy, per-oral endoscopic mytotomy, botulinum toxin injection, pneumatic dilatation, medical therapy, P=0.67) and achalasia subtype (P=1.00). UES abnormalities are a frequent finding on HREM

Socio-economic status and lifestyle factors are associated with achalasia risk: A population-based case-control study

PubMed Central

Coleman, Helen G; Gray, Ronan T; Lau, Kar W; McCaughey, Conall; Coyle, Peter V; Murray, Liam J; Johnston, Brian T

2016-01-01

AIM: To evaluate the association between various lifestyle factors and achalasia risk. METHODS: A population-based case-control study was conducted in Northern Ireland, including n = 151 achalasia cases and n = 117 age- and sex-matched controls. Lifestyle factors were assessed via a face-to-face structured interview. The association between achalasia and lifestyle factors was assessed by unconditional logistic regression, to produce odds ratios (OR) and 95% confidence interval (CI). RESULTS: Individuals who had low-class occupations were at the highest risk of achalasia (OR = 1.88, 95%CI: 1.02-3.45), inferring that high-class occupation holders have a reduced risk of achalasia. A history of foreign travel, a lifestyle factor linked to upper socio-economic class, was also associated with a reduced risk of achalasia (OR = 0.59, 95%CI: 0.35-0.99). Smoking and alcohol consumption carried significantly reduced risks of achalasia, even after adjustment for socio-economic status. The presence of pets in the house was associated with a two-fold increased risk of achalasia (OR = 2.00, 95%CI: 1.17-3.42). No childhood household factors were associated with achalasia risk. CONCLUSION: Achalasia is a disease of inequality, and individuals from low socio-economic backgrounds are at highest risk. This does not appear to be due to corresponding alcohol and smoking behaviours. An observed positive association between pet ownership and achalasia risk suggests an interaction between endotoxin and viral infection exposure in achalasia aetiology. PMID:27099443

MicroRNA-130a is highly expressed in the esophageal mucosa of achalasia patients

PubMed Central

Shoji, Hiroyuki; Isomoto, Hajime; Yoshida, Akira; Ikeda, Haruo; Minami, Hitomi; Kanda, Tsutomu; Urabe, Shigetoshi; Matsushima, Kayoko; Takeshima, Fuminao; Nakao, Kazuhiko; Inoue, Haruhiro

2017-01-01

Esophageal achalasia is considered as a risk factor of esophageal cancer. The etiologies of esophageal achalasia remain unknown. Peroral endoscopic myotomy (POEM) has recently been established as a minimally invasive method with high curability. The aims of the present study were to identify the microRNAs (miRs) specific to esophageal achalasia, to determine their potential target genes and to assess their alteration following POEM. RNA was extracted from biopsy samples from middle esophageal mucosa and analyzed using a microarray. Differentially expressed miRs in achalasia patients compared with control samples were identified and analyzed using reverse transcription-quantitative polymerase chain reaction (RT-qPCR). Correlations between specific miR expression levels and the patients' clinical background were also investigated. In addition, alterations of selected miR expression levels before and after POEM were analyzed. The results of RT-qPCR analysis demonstrated that the miR-130a expression levels were significantly higher in patients with achalasia (P<0.0001). In addition, miR-130a expression was significantly correlated with male sex and smoking history in patients with achalasia. However, no significant change in miR-130a expression was observed between before and after POEM. In conclusion, miR-130a is highly expressed in the esophageal mucosa of patients with achalasia and may be a biomarker of esophageal achalasia. PMID:28810541

MicroRNA-130a is highly expressed in the esophageal mucosa of achalasia patients.

PubMed

Shoji, Hiroyuki; Isomoto, Hajime; Yoshida, Akira; Ikeda, Haruo; Minami, Hitomi; Kanda, Tsutomu; Urabe, Shigetoshi; Matsushima, Kayoko; Takeshima, Fuminao; Nakao, Kazuhiko; Inoue, Haruhiro

2017-08-01

Esophageal achalasia is considered as a risk factor of esophageal cancer. The etiologies of esophageal achalasia remain unknown. Peroral endoscopic myotomy (POEM) has recently been established as a minimally invasive method with high curability. The aims of the present study were to identify the microRNAs (miRs) specific to esophageal achalasia, to determine their potential target genes and to assess their alteration following POEM. RNA was extracted from biopsy samples from middle esophageal mucosa and analyzed using a microarray. Differentially expressed miRs in achalasia patients compared with control samples were identified and analyzed using reverse transcription-quantitative polymerase chain reaction (RT-qPCR). Correlations between specific miR expression levels and the patients' clinical background were also investigated. In addition, alterations of selected miR expression levels before and after POEM were analyzed. The results of RT-qPCR analysis demonstrated that the miR-130a expression levels were significantly higher in patients with achalasia (P<0.0001). In addition, miR-130a expression was significantly correlated with male sex and smoking history in patients with achalasia. However, no significant change in miR-130a expression was observed between before and after POEM. In conclusion, miR-130a is highly expressed in the esophageal mucosa of patients with achalasia and may be a biomarker of esophageal achalasia.

A Matched Comparison of Per Oral Endoscopic Myotomy to Laparoscopic Heller Myotomy in the Treatment of Achalasia.

PubMed

Schneider, Andreas M; Louie, Brian E; Warren, Heather F; Farivar, Alexander S; Schembre, Drew B; Aye, Ralph W

2016-11-01

Per oral endoscopic myotomy (POEM) is increasingly utilized to treat patients with achalasia. Early results have demonstrated significant improvement of symptoms, but there are concerns about postoperative reflux. With only limited comparative data available, we sought to compare POEM to laparoscopic Heller myotomy (LHM) with partial fundoplication. This is a retrospective review of 42 POEM and 84 LHM patients undergoing primary myotomy for achalasia. Patients were matched by achalasia type, by Eckardt and dysphagia scores, and by quality of life (QOL) metrics. Analysis at 6-12-month follow-up evaluated these metrics, PPI use, pH, manometric, and endoscopic data. We matched 25 patients with achalasia types I (6), II (13), and III (6). Follow-up was longer for LHM at 158.1 (36.5-272.9) weeks versus 36.2 (22.2-41.2) weeks (p = 0.001). Eckardt scores, QOL metrics, and dysphagia significantly improved in both groups. DeMeester scores and total percent time less than 4 were abnormal in both groups and comparable (p = 0.925 and p = 0.838). Esophagitis was seen in 53.4 % (POEM) and 31.6 % (LHM) (Yates' p = 0.91), and PPI use was equivalent at 36 %. Early clinical outcomes are excellent with POEM and comparable to the standard of care LHM. Long-term follow-up is required as concerns for reflux persist.

Achalasia-Specific Quality of Life After Pneumatic Dilation or Laparoscopic Heller Myotomy With Partial Fundoplication: A Multicenter, Randomized Clinical Trial.

PubMed

Chrystoja, Caitlin C; Darling, Gail E; Diamant, Nicholas E; Kortan, Paul P; Tomlinson, George A; Deitel, Wayne; Laporte, Audrey; Takata, Julie; Urbach, David R

2016-11-01

Achalasia is a chronic, progressive, and incurable esophageal motility disease. There is clinical uncertainty about which treatment should be recommended as first-line therapy. Our objective was to evaluate the effectiveness of pneumatic dilation compared with laparoscopic Heller myotomy with partial fundoplication in improving achalasia-specific quality of life. This was a prospective, multicenter, randomized trial at five academic hospitals in Canada. Fifty previously untreated adults with a clinical diagnosis of primary achalasia, confirmed by manometric testing, were enrolled between November 2005 and March 2010, and followed for 5 years after treatment. Randomization was stratified by site, in random blocks of size four and with balanced allocation. Patients were treated with pneumatic dilation or laparoscopic Heller myotomy with partial fundoplication. The primary outcome was the difference between the treatments in the mean improvement of the achalasia severity questionnaire (ASQ) score at 1 year from baseline. Prespecified secondary outcomes included general and gastrointestinal quality of life, symptoms, esophageal physiology measures (lower esophageal sphincter relaxation and pressure, esophageal emptying, abnormal esophageal acid exposure), complications, and incidence of retreatment. Functional and imaging studies were performed blinded and all outcome assessors were blinded. There were no significant differences between treatments in the improvement of ASQ score at 1 year from baseline (27.5 points in the Heller myotomy arm vs. 20.2 points in the pneumatic dilation arm; difference 7.3 points, 95% confidence interval -4.7 to 19.3; P=0.23). There were no differences between treatments in improvement of symptoms, general and gastrointestinal quality of life, or measures of esophageal physiology. Improvements in ASQ score diminished over time for both interventions. At 5 years, there were no differences between treatments in improvement of ASQ score

Incidence of Achalasia in South Australia Based on Esophageal Manometry Findings.

PubMed

Duffield, Jaime A; Hamer, Peter W; Heddle, Richard; Holloway, Richard H; Myers, Jennifer C; Thompson, Sarah K

2017-03-01

Achalasia is a disorder of esophageal motility with a reported incidence of 0.5 to 1.6 per 100,000 persons per year in Europe, Asia, Canada, and America. However, estimates of incidence values have been derived predominantly from retrospective searches of databases of hospital discharge codes and personal communications with gastroenterologists, and are likely to be incorrect. We performed a cohort study based on esophageal manometry findings to determine the incidence of achalasia in South Australia. We collected data from the Australian Bureau of Statistics on the South Australian population. Cases of achalasia diagnosed by esophageal manometry were identified from the 3 adult manometry laboratory databases in South Australia. Endoscopy reports and case notes were reviewed for correlations with diagnoses. The annual incidence of achalasia in the South Australian population was calculated for the decade 2004 to 2013. Findings were standardized to those of the European Standard Population based on age. The annual incidence of achalasia in South Australia ranged from 2.3 to 2.8 per 100,000 persons. The mean age at diagnosis was 62.1 ± 18.1 years. The incidence of achalasia increased with age (Spearman rho, 0.95; P < .01). The age-standardized incidence ranged from 2.1 (95% CI, 1.8-2.3) to 2.5 (95% CI, 2.2-2.7). Based on a cohort study of esophageal manometry, we determined the incidence of achalasia in South Australia to be 2.3 to 2.8 per 100,000 persons and to increase with age. South Australia's relative geographic isolation and the population's access to manometry allowed for more accurate identification of cases than hospital code analyses, with a low probability of missed cases. Copyright © 2017 AGA Institute. Published by Elsevier Inc. All rights reserved.

Diagnosis and management of esophageal achalasia in children: analysis of 13 cases.

PubMed

Zhang, Yin; Xu, Chun-Di; Zaouche, Abdehaman; Cai, Wei

2009-02-01

Esophageal achalasia is a rare disease and there have been very few reports about it, especially in children. We reviewed our experience in dealing with esophageal achalasia in 13 children. Thirteen children (6 boys and 7 girls), who had been diagnosed with achalasia over a 12-year period between May 1993 and October 2005, were analysed with regard to clinical manifestations, esophageal manometry, endoscopic findings, and treatment. Their age ranged from 3 years to 14 years and 5 months (average 10.3 years) at the time of diagnosis. In the 13 children, 3 had a family history of esophageal achalasia, 2 of them were sisters. All the 3 children suffered from achalasia/alacrimia/ACTH deficiency. Dysphagia was the most common symptom in the affected children. Vomiting/regurgitation, retrosternal pain, retarded growth, and respiratory symptoms were also observed in some patients. Heller's esophagocardiomyotomy was performed in 9 (69.23%) children, among whom 3 had an antireflux operation at the same time. In the remaining 4 children, 3 received a pneumatic dilatation and 1 received regular administration of nifedipine. Twelve patients were followed up: 8 patients by surgery were cured and have been in perfect condition until now, 3 patients recovered fairly, and 1 patient showed improvement. Esophageal manometry combined with X-ray examination proves to be an effective diagnostic method for achalasia. It is also effective in evaluating the result of treatment. Heller's esophagocardiomyotomy is a treatment of choice for children with achalasia because of its safety and long-term effective results after surgery.

Laparoscopic Heller Myotomy and Dor Fundoplication for Esophageal Achalasia: Technique and Perioperative Management.

PubMed

Andolfi, Ciro; Fisichella, P Marco

2016-11-01

Surgical correction of achalasia using laparoscopic Heller myotomy with Dor fundoplication is argued to be the gold standard treatment for patients with achalasia. The goal of this technical report is to illustrate our preferred approach to patients with achalasia and to provide the reader with a detailed description of our operative technique, its rationale, and our pre and postoperative management.

Peroral endoscopic myotomy for the treatment of achalasia in a patient with esophageal varices. A case report.

PubMed

Shen, Naning; Wang, Xin; Zhang, Xiaoyin; Yao, Liping; Xie, Huahong; Zhang, Hongbo

2017-06-01

Achalasia is very uncommon, and rarely does achalasia co-exist with esophageal varices. We present a 62-year-old woman who was diagnosed with both achalasia and esophageal varices in December 2014 and had a past history of hematemesis. The patient's achalasia symptoms' Eckardt score was 9, and her hepatic function was Child-Pugh grade A6. After comprehensive assessment of the patient's health and discussion of the pros and cons of various therapies for achalasia, the patient underwent a peroral endoscopic myotomy. She was symptom-free after the operation and had no recurrence of achalasia symptoms at 20-month follow-up. No adverse events were reported. Peroral endoscopic myotomy for achalasia with esophageal varices has not been previously reported in the English literature.

Multidisciplinary Approach to Esophageal Achalasia: A Single Center Experience.

PubMed

Schlottmann, Francisco; Andolfi, Ciro; Kavitt, Robert T; Konda, Vani J A; Patti, Marco G

2017-04-01

The treatment of achalasia is palliative. Pneumatic dilatation (PD) or laparoscopic Heller myotomy (LHM) just eliminates the outflow obstruction allowing easier emptying of the esophagus. The aim of this study was to evaluate the results of a multidisciplinary approach to esophageal achalasia. A consecutive series of patients with achalasia treated by a multidisciplinary esophageal team consisting of radiologists, gastroenterologists, and surgeons in a quaternary care center between May 2008 and April 2015 were analyzed. A total of 147 patients with achalasia underwent LHM and partial fundoplication. Sixty-two patients (42%) had been treated preoperatively with PD and/or botulinum toxin (BT). The preoperative Eckardt score (ES) was 6.4 ± 2. At a median follow-up of 22 months, 128 patients (87%) did well and required no further treatment (ES 0.1). The remaining 19 patients (13%) had recurrence of symptoms and required further treatment: 12 were treated with PD and improved (ES 0.7); 4 were treated with PD and BT and improved (ES 1.3); 3 failed PD. These 3 patients had been treated with multiple sessions of PD and BT before the myotomy. Overall, 144 patients (98%) did well with laparoscopic (87%) or laparoscopic and endoscopic treatment (11%). The results of this study show that (a) LHM is an effective treatment modality, (b) PD improved symptoms in the majority of patients with recurrent dysphagia after myotomy and (c) multiple preoperative endoscopic treatments seem to affect outcomes of LHM. Patients with achalasia should be treated in a quaternary care center by a multidisciplinary team.

Achalasia and Respiratory Symptoms: Effect of Laparoscopic Heller Myotomy.

PubMed

Andolfi, Ciro; Kavitt, Robert T; Herbella, Fernando A M; Patti, Marco G

2016-09-01

Dysphagia and regurgitation are considered typical symptoms of achalasia. However, there is mounting evidence that some achalasia patients may also experience respiratory symptoms such as cough, wheezing, and hoarseness. The aims of this study were to determine: (1) what percentage of achalasia patients experience respiratory symptoms and (2) the effect of a laparoscopic Heller myotomy and Dor fundoplication on the typical and respiratory symptoms of achalasia. Between May 2008 and December 2015, 165 patients with achalasia were referred for treatment to the Center for Esophageal Diseases of the University of Chicago. Patients had preoperatively a barium swallow, endoscopy, and esophageal manometry. All patients underwent a Heller myotomy and Dor fundoplication. Based on the presence of respiratory symptoms, patients were divided into two groups: group A, 98 patients (59%) without respiratory symptoms and group B, 67 patients (41%) with respiratory symptoms. The preoperative Eckardt score was similar in the two groups (6.5 ± 2.1 versus 6.4 ± 2.0). The mean esophageal diameter was 27.7 ± 10.8 mm in group A and 42.6 ± 20.1 mm in group B (P < .05). The operation consisted of a myotomy that extended for 5 cm on the esophagus and 2.5 cm onto the gastric wall. At a median postoperative follow-up of 17 months, the Eckardt score improved significantly and similarly in the two groups (0.3 ± 0.8 versus 0.3 ± 1.0). Respiratory symptoms improved or resolved in 62 patients (92.5%). The results of this study showed that: (1) respiratory symptoms were present in 41% of patients; (2) patients with respiratory symptoms had a more dilated esophagus; and (3) surgical treatment resolved or improved respiratory symptoms in 92.5% of patients. This study underlines the importance of investigating the presence of respiratory symptoms along with the more common symptoms of achalasia and of early treatment before lung damage occurs.

Robot-assisted Heller's myotomy for achalasia in children.

PubMed

Altokhais, Tariq; Mandora, Hala; Al-Qahtani, Ayed; Al-Bassam, Abdulrahman

2016-12-01

Achalasia is rare in children. Surgical options include open, laparoscopic and robotic approaches. However, Heller's myotomy remains the treatment of choice. This report describes our experience with robot-assisted Heller's myotomy in children and presents a review of the literature. Included in this study are children who underwent robot-assisted Heller's myotomy for esophageal achalasia via the Da Vinci surgical system between 2004 and 2015 at King Saud University Medical City, Riyadh, Saudi Arabia. The medical records of these patients were reviewed for demographic data, presenting symptoms, diagnostic modalities, operative procedures, complications, outcomes and follow-ups. Six patients were identified. The age of the patients at surgery ranged between 2 and 12 years (mean 7.1 years). The most common presenting symptoms were dysphagia, vomiting and nocturnal cough. Contrast swallow and upper gastrointestinal endoscopy established a diagnosis of esophageal achalasia in all of the patients. Four patients underwent esophageal dilatation 2-5 times before the definitive procedure. All patients underwent successful robot-assisted Heller's myotomy with concomitant partial posterior fundoplication. The postoperative course was uneventful. Five patients had a complete resolution of the symptoms and one patient improved. The follow-up assessments have been consistent and have ranged from 0.5 to 11 years (mean 4.4 years). Robotic-assisted Heller's myotomy for esophageal achalasia in children is safe and effective and is a suitable alternative to open and laparoscopic approaches.

Surgical management of esophageal achalasia: Evolution of an institutional approach to minimally invasive repair.

PubMed

Petrosyan, Mikael; Khalafallah, Adham M; Guzzetta, Phillip C; Sandler, Anthony D; Darbari, Anil; Kane, Timothy D

2016-10-01

Surgical management of esophageal achalasia (EA) in children has transitioned over the past 2 decades to predominantly involve laparoscopic Heller myotomy (LHM) or minimally invasive surgery (MIS). More recently, peroral endoscopic myotomy (POEM) has been utilized to treat achalasia in children. Since the overall experience with surgical management of EA is contingent upon disease incidence and surgeon experience, the aim of this study is to report a single institutional contemporary experience for outcomes of surgical treatment of EA by LHM and POEM, with regards to other comparable series in children. An IRB approved retrospective review of all patients with EA who underwent treatment by a surgical approach at a tertiary US children's hospital from 2006 to 2015. Data including demographics, operative approach, Eckardt scores pre- and postoperatively, complications, outcomes, and follow-up were analyzed. A total of 33 patients underwent 35 operative procedures to treat achalasia. Of these operations; 25 patients underwent laparoscopic Heller myotomy (LHM) with Dor fundoplication; 4 patients underwent LHM alone; 2 patients underwent LHM with Thal fundoplication; 2 patients underwent primary POEM; 2 patients who had had LHM with Dor fundoplication underwent redo LHM with takedown of Dor fundoplication. Intraoperative complications included 2 mucosal perforations (6%), 1 aspiration, 1 pneumothorax (1 POEM patient). Follow ranged from 8months to 7years (8-84months). There were no deaths and no conversions to open operations. Five patients required intervention after surgical treatment of achalasia for recurrent dysphagia including 3 who underwent between 1 and 3 pneumatic dilations; and 2 who had redo LHM with takedown of Dor fundoplication with all patients achieving complete resolution of symptoms. Esophageal achalasia in children occurs at a much lower incidence than in adults as documented by published series describing the surgical treatment in children. We

Achalasia Patients Are at Nutritional Risk Regardless of Presenting Weight Category.

PubMed

Newberry, Carolyn; Vajravelu, Ravy K; Pickett-Blakely, Octavia; Falk, Gary; Yang, Yu Xiao; Lynch, Kristle L

2018-05-01

Achalasia is an esophageal motor disorder that leads to swallowing dysfunction and weight loss. Nutritional risk in achalasia patients is not well defined. The aims of this study were to define baseline body mass index (BMI), changes in weight, and nutritional risk over time in a large cohort of achalasia patients. This was a retrospective cohort study of achalasia patients at a tertiary care center with documented BMI, symptom severity as per Eckardt score, and nutritional risk assessment as per the Malnutrition Universal Screening Tool, which considers BMI, degree of recent weight loss, and acuity of disease. Among the 337 patients presenting for achalasia management, 179 had confirmed disease. Upon presentation 69.8% of patients were classified as overweight or obese. Using the Malnutrition Universal Screening Tool, we found 50% of patients to be at moderate or high risk for malnutrition at presentation. Eckardt score (OR 1.15, 95% CI 1.05-1.26), duration of disease (OR for each additional month 1.04, 95% CI 1.01-1.08), and female gender (OR 1.76, 95% CI 1.02-3.03) were independent predictors of increased risk for malnutrition. Nutrition risk score decreased after therapy in 93.3% of patients. Despite a high prevalence of overweight and obese status in achalasia patients, many are at risk of developing nutritional complications secondary to rapid weight loss. This risk frequently resolves post-treatment. Regardless of baseline BMI, we recommend all patients undergo nutritional assessment to identify high-risk patients who may benefit from dietary intervention and expedited therapy.

Hydrogen sulfide synthesis enzymes reduced in lower esophageal sphincter of patients with achalasia.

PubMed

Zhang, L; Zhao, W; Zheng, Z; Wang, T; Zhao, C; Zhou, G; Jin, H; Wang, B

2016-10-01

The etiology of achalasia remains largely unknown. Considerable evidence reveals that the lower esophageal sphincter dysfunction is due to the lack of inhibitory neurotransmitter, secondary to esophageal neuronal inflammation or loss. Recent studies suggest hydrogen sulfide may act as an inhibitory transmitter in gastrointestinal tract, but study about hydrogen sulfide in human esophagus still lack. The aim of the study was to investigate if hydrogen sulfide synthesis enzymes could be detected in human esophagus and if the synthesis of the endogenous hydrogen sulfide could be affected in achalasia patients. Tissue samples in cardia, lower esophageal sphincter, 2 cm and 4 cm above lower esophageal sphincter were obtained from achalasia patients undergoing peroral endoscopic myotomy. Control tissues in lower esophageal sphincter were obtained from esophageal carcinoma patients. Expression of cystathionine-β-synthase and cystathionine-γ-lyase in lower esophageal sphincter of achalasia patients and control were detected by immunohistochemical staining. In addition, expression of cystathionine-β-synthase and cystathionine-γ-lyase were compared among different parts of esophagus in achalasia patients. Compared with control, the expression of cystathionine-β-synthase and cystathionine-γ-lyase in lower esophageal sphincter of achalasia patients was significantly reduced (χ 2 = 11.429, P = 0.010). The expression of cystathionine-β-synthase and cystathionine-γ-lyase were lower in lower esophageal sphincter than that in 2 cm and 4 cm above lower esophageal sphincter, respectively (all P < 0.05). In conclusion, the expression of hydrogen sulfide synthesis enzymes, cystathionine-β-synthase and cystathionine-γ-lyase, can be detected in human esophagus and is reduced in patients with achalasia, which implicates the involvement of the two hydrogen sulfide synthesis enzymes in the pathophysiology of achalasia. © 2015 International Society for Diseases of the

Activated Eosinophils are Present in Esophageal Muscle in Patients with Achalasia of the Esophagus

PubMed Central

Jin, Hong; Wang, Bin; Zhang, Li-li

2018-01-01

Background The aim of this study was to undertake a histological evaluation of the presence of eosinophils in esophageal muscle in patients with achalasia before treatment with peroral endoscopic myotomy (POEM), with clinical follow-up at one year. Material/Methods Before treatment, esophageal biopsies including mucosa and esophageal muscle were obtained from 28 patients with achalasia. Nine patients who had undergone esophagectomy for esophageal carcinoma were included in the control group. The Eckardt Score was used to evaluate the clinical symptoms of achalasia. Histology of routinely processed tissue sections was used to perform eosinophil cell counts (0 to +++), and immunohistochemistry was used to detect expression of eosinophil major basic protein (MBP), eosinophil-derived neurotoxin (EDN), and S100 protein in cases of achalasia (n=28) and controls (n=9). The findings in patients with achalasia were compared before and one year following POEM. Results Esophageal tissue from patients with achalasia showed eosinophils infiltrating into the muscularis externa in 85.7% (24/28), into the muscularis propria in 28.6% (8/28), and in 89% (25/28) there were few remaining myenteric ganglion cells, before POEM. The extent of inflammation was similar in all regions of the esophagus and between subtypes of achalasia. At one year following POEM, the Eckardt Scores between the former eosinophil (0) group and the eosinophil (+++) group were significantly different (Z=3.50, P=0.030). Conclusions Achalasia of the esophagus was associated with infiltration of the esophageal muscle by activated eosinophils and a decrease in the density of ganglion cells in the myenteric esophageal plexus. PMID:29672471

Robotic heller myotomy and Dor fundoplication for achalasia in a woman with morbid obesity.

PubMed

Bedirli, Abdulkadir; Dogan, Ibrahim; Kozan, Ramazan

2012-12-01

Achalasia is a relatively rare condition with a prevalence estimated at less than 0.001 %. Laparoscopic or robotic Heller myotomy is an effective surgical treatment for achalasia. We present the first published case of a morbidly obese achalasia patient treated with robotic Heller myotomy and Dor fundoplication. The operative time was 175 min, with an estimated blood loss of 110 ml. The patient had a normal bowel transit on postoperative day 2, and he was discharged on postoperative day 4 on a liquid diet. A follow-up at 2 months showed significant resolved symptoms of achalasia.

Longitudinal muscle dysfunction in achalasia esophagus and its relevance.

PubMed

Mittal, Ravinder K; Hong, Su Jin; Bhargava, Valmik

2013-04-01

Muscularis propria of the esophagus is organized into circular and longitudinal muscle layers. Goal of this review is to summarize the role of longitudinal muscle in physiology and pathophysiology of esophageal sensory and motor function. Simultaneous manometry and ultrasound imaging that measure circular and longitudinal muscle contraction respectively reveal that during peristalsis 2 layers of the esophagus contract in perfect synchrony. On the other hand, during transient relaxation of the lower esophageal sphincter (LES), longitudinal muscle contracts independently of circular muscle. Recent studies provide novel insights, i.e., longitudinal muscle contraction of the esophagus induces LES relaxation and possibly descending relaxation of the esophagus. In achalasia esophagus and other motility disorders there is discoordination between the 2 muscle layers. Longitudinal muscle contraction patterns are different in the recently described three types of achalasia identified by high-resolution manometry. Robust contraction of the longitudinal muscle in type II achalasia causes pan-esophageal pressurization and is the mechanism of whatever little esophageal emptying that take place in the absence of peristalsis and impaired LES relaxation. It may be that preserved longitudinal muscle contraction is also the reason for superior outcome to medical/surgical therapy in type II achalasia esophagus. Prolonged contractions of longitudinal muscles of the esophagus is a possible mechanism of heartburn and "angina like" pain seen in esophageal motility disorders and possibly achalasia esophagus. Novel techniques to record longitudinal muscle contraction are on the horizon. Neuro-pharmacologic control of circular and longitudinal muscles is different, which provides an important opportunity for the development of novel pharmacological therapies to treat sensory and motor disorders of the esophagus.

Longitudinal Muscle Dysfunction in Achalasia Esophagus and Its Relevance

PubMed Central

Hong, Su Jin; Bhargava, Valmik

2013-01-01

Muscularis propria of the esophagus is organized into circular and longitudinal muscle layers. Goal of this review is to summarize the role of longitudinal muscle in physiology and pathophysiology of esophageal sensory and motor function. Simultaneous manometry and ultrasound imaging that measure circular and longitudinal muscle contraction respectively reveal that during peristalsis 2 layers of the esophagus contract in perfect synchrony. On the other hand, during transient relaxation of the lower esophageal sphincter (LES), longitudinal muscle contracts independently of circular muscle. Recent studies provide novel insights, i.e., longitudinal muscle contraction of the esophagus induces LES relaxation and possibly descending relaxation of the esophagus. In achalasia esophagus and other motility disorders there is discoordination between the 2 muscle layers. Longitudinal muscle contraction patterns are different in the recently described three types of achalasia identified by high-resolution manometry. Robust contraction of the longitudinal muscle in type II achalasia causes pan-esophageal pressurization and is the mechanism of whatever little esophageal emptying that take place in the absence of peristalsis and impaired LES relaxation. It may be that preserved longitudinal muscle contraction is also the reason for superior outcome to medical/surgical therapy in type II achalasia esophagus. Prolonged contractions of longitudinal muscles of the esophagus is a possible mechanism of heartburn and "angina like" pain seen in esophageal motility disorders and possibly achalasia esophagus. Novel techniques to record longitudinal muscle contraction are on the horizon. Neuro-pharmacologic control of circular and longitudinal muscles is different, which provides an important opportunity for the development of novel pharmacological therapies to treat sensory and motor disorders of the esophagus. PMID:23667744

Safety and efficacy of botulinum toxin injection therapy for esophageal achalasia in Japan

PubMed Central

Yamaguchi, Daisuke; Tsuruoka, Nanae; Sakata, Yasuhisa; Shimoda, Ryo; Fujimoto, Kazuma; Iwakiri, Ryuichi

2015-01-01

Botulinum toxin injection is an accepted treatment modality for esophageal achalasia in western countries. This pilot study aimed to clarify the effectiveness of botulinum toxin injection for esophageal achalasia in Japanese patients. We enrolled 10 patients diagnosed with esophageal achalasia between 2008 and 2014. A total of 100 U botulinum toxin A was divided into eight aliquots and injected around the esophagogastric junction. We compared the lower esophageal sphincter pressure before and 1 week after treatment. Scores of subjective symptoms for esophageal achalasia were assessed using a visual analog scale (VAS) before and after 1 week of follow-up of treatment. Barium passage was improved in barium esophagography and passage of contrast agent was also improved. Mean Eckardt score was reduced from 5.5 to 1.6 after treatment (p<0.001). By esophageal manometric study, mean lower esophageal sphincter pressure was reduced from 46.9 to 29.1 mmHg after treatment (p = 0.002). One week after treatment, mean VAS score was reduced from 10 to 3.9 (p<0.001). There were no side effects in any cases. Botulinum toxin injection for esophageal achalasia was safe and effective with few complications. Therefore, botulinum toxin could be used as minimally invasive therapy for esophageal achalasia in Japan. PMID:26566311

Safety and efficacy of botulinum toxin injection therapy for esophageal achalasia in Japan.

PubMed

Yamaguchi, Daisuke; Tsuruoka, Nanae; Sakata, Yasuhisa; Shimoda, Ryo; Fujimoto, Kazuma; Iwakiri, Ryuichi

2015-11-01

Botulinum toxin injection is an accepted treatment modality for esophageal achalasia in western countries. This pilot study aimed to clarify the effectiveness of botulinum toxin injection for esophageal achalasia in Japanese patients. We enrolled 10 patients diagnosed with esophageal achalasia between 2008 and 2014. A total of 100 U botulinum toxin A was divided into eight aliquots and injected around the esophagogastric junction. We compared the lower esophageal sphincter pressure before and 1 week after treatment. Scores of subjective symptoms for esophageal achalasia were assessed using a visual analog scale (VAS) before and after 1 week of follow-up of treatment. Barium passage was improved in barium esophagography and passage of contrast agent was also improved. Mean Eckardt score was reduced from 5.5 to 1.6 after treatment (p<0.001). By esophageal manometric study, mean lower esophageal sphincter pressure was reduced from 46.9 to 29.1 mmHg after treatment (p = 0.002). One week after treatment, mean VAS score was reduced from 10 to 3.9 (p<0.001). There were no side effects in any cases. Botulinum toxin injection for esophageal achalasia was safe and effective with few complications. Therefore, botulinum toxin could be used as minimally invasive therapy for esophageal achalasia in Japan.

Per-oral endoscopic myotomy for achalasia: An American perspective

PubMed Central

Friedel, David; Modayil, Rani; Iqbal, Shahzad; Grendell, James H; Stavropoulos, Stavros N

2013-01-01

Achalasia is an uncommon esophageal motility disorder characterized by the selective loss of enteric neurons leading to absence of peristalsis and impaired relaxation of the lower esophageal sphincter. Per-oral endoscopic myotomy (POEM) is a novel modality for the treatment of achalasia performed by gastroenterologists and surgeons. It represents a natural orifice transluminal endoscopic surgery (NOTES) approach to Heller myotomy. POEM has the minimal invasiveness of an endoscopic procedure that can duplicate results of the surgical Heller myotomy. POEM is conceptually similar to a surgical myotomy without the inherent external incisions and post-operative care associated with surgery. Initial high success and low complications rates promise a great future for this technique. In fact, POEM has been successfully performed on patients with end-stage achalasia as an initial treatment reserving esophagectomy for those without good response. The volume of POEMs performed worldwide has grown exponentially. In fact, surgeons who have performed Heller myotomy have embraced POEM as the preferred intervention for achalasia. However, the niche of POEM remains to be defined and long term results are awaited. We describe our experience with POEM having performed the first POEM outside of Japan in 2009, the evolution of our technique, and give our perspective on its future. PMID:24044040

Current status in the treatment options for esophageal achalasia

PubMed Central

Chuah, Seng-Kee; Chiu, Chien-Hua; Tai, Wei-Chen; Lee, Jyong-Hong; Lu, Hung-I; Changchien, Chi-Sin; Tseng, Ping-Huei; Wu, Keng-Liang

2013-01-01

Recent advances in the treatment of achalasia include the use of high-resolution manometry to predict the outcome of patients and the introduction of peroral endoscopic myotomy (POEM). The first multicenter randomized, controlled, 2-year follow-up study conducted by the European Achalasia Trial group indicated that laparoscopic Heller myotomy (LHM) was not superior to pneumatic dilations (PD). Publications on the long-term success of laparoscopic surgery continue to emerge. In addition, laparoscopic single-site surgery is applicable to advanced laparoscopic operations such as LHM and anterior fundoplication. The optimal treatment option is an ongoing matter of debate. In this review, we provide an update of the current progress in the treatment of esophageal achalasia. Unless new conclusive data prove otherwise, LHM is considered the most durable treatment for achalasia at the expense of increased reflux-associated complications. However, PD is the first choice for non-surgical treatment and is more cost-effective. Repeated PD according to an “on-demand” strategy based on symptom recurrence can achieve long-term remission. Decision making should be based on clinical evidence that identifies a subcategory of patients who would benefit from specific treatment options. POEM has shown promise but its long-term efficacy and safety need to be assessed further. PMID:24023484

Current status in the treatment options for esophageal achalasia.

PubMed

Chuah, Seng-Kee; Chiu, Chien-Hua; Tai, Wei-Chen; Lee, Jyong-Hong; Lu, Hung-I; Changchien, Chi-Sin; Tseng, Ping-Huei; Wu, Keng-Liang

2013-09-07

Recent advances in the treatment of achalasia include the use of high-resolution manometry to predict the outcome of patients and the introduction of peroral endoscopic myotomy (POEM). The first multicenter randomized, controlled, 2-year follow-up study conducted by the European Achalasia Trial group indicated that laparoscopic Heller myotomy (LHM) was not superior to pneumatic dilations (PD). Publications on the long-term success of laparoscopic surgery continue to emerge. In addition, laparoscopic single-site surgery is applicable to advanced laparoscopic operations such as LHM and anterior fundoplication. The optimal treatment option is an ongoing matter of debate. In this review, we provide an update of the current progress in the treatment of esophageal achalasia. Unless new conclusive data prove otherwise, LHM is considered the most durable treatment for achalasia at the expense of increased reflux-associated complications. However, PD is the first choice for non-surgical treatment and is more cost-effective. Repeated PD according to an "on-demand" strategy based on symptom recurrence can achieve long-term remission. Decision making should be based on clinical evidence that identifies a subcategory of patients who would benefit from specific treatment options. POEM has shown promise but its long-term efficacy and safety need to be assessed further.

Cellular and molecular basis of chronic constipation: taking the functional/idiopathic label out.

PubMed

Bassotti, Gabrio; Villanacci, Vincenzo; Creţoiu, Dragos; Creţoiu, Sanda Maria; Becheanu, Gabriel

2013-07-14

In recent years, the improvement of technology and the increase in knowledge have shifted several strongly held paradigms. This is particularly true in gastroenterology, and specifically in the field of the so-called "functional" or "idiopathic" disease, where conditions thought for decades to be based mainly on alterations of visceral perception or aberrant psychosomatic mechanisms have, in fact, be reconducted to an organic basis (or, at the very least, have shown one or more demonstrable abnormalities). This is particularly true, for instance, for irritable bowel syndrome, the prototype entity of "functional" gastrointestinal disorders, where low-grade inflammation of both mucosa and myenteric plexus has been repeatedly demonstrated. Thus, researchers have also investigated other functional/idiopathic gastrointestinal disorders, and found that some organic ground is present, such as abnormal neurotransmission and myenteric plexitis in esophageal achalasia and mucosal immune activation and mild eosinophilia in functional dyspepsia. Here we show evidence, based on our own and other authors' work, that chronic constipation has several abnormalities reconductable to alterations in the enteric nervous system, abnormalities mainly characterized by a constant decrease of enteric glial cells and interstitial cells of Cajal (and, sometimes, of enteric neurons). Thus, we feel that (at least some forms of) chronic constipation should no more be considered as a functional/idiopathic gastrointestinal disorder, but instead as a true enteric neuropathic abnormality.

Achalasia symptom response after Heller myotomy segregated by high-resolution manometry subtypes.

PubMed

Patel, Amit; Patel, Ami; Mirza, Faiz A; Soudagar, Samad; Sayuk, Gregory S; Gyawali, C Prakash

2016-02-01

Achalasia is classified into three HRM subtypes that predict outcomes from diverse management strategies. We assessed if symptomatic response varied when a single management strategy-Heller myotomy (HM)-is employed. Treatment-naive subjects with achalasia referred for HM were followed in this observational cohort study. Chicago criteria designated achalasia subtypes (subtype I: no esophageal pressurization; subtype II: panesophageal pressurization in ≥20 % swallows; subtype III: premature contractions in ≥20 % swallows). Symptom questionnaires assessed symptom burden before and after HM on five-point Likert scales (0 = no symptoms, 4 = severe symptoms) and on 10-cm visual analog scales (global symptom severity, GSS); satisfaction with HM was recorded similarly. Data were analyzed to determine predictors of GSS change across subtypes. Sixty achalasia subjects (56.1 ± 2.4 years, 55 % female) fulfilled inclusion criteria, 15 % with subtype I, 58 % with subtype II, and 27 % with subtype III achalasia. Baseline symptoms included dysphagia (solids: 85 %, liquids: 73 %), regurgitation (84 %), and chest pain (35 %); mean GSS was 7.1 ± 0.3. Upon follow-up 2.1 ± 0.2 years after HM, GSS declined to 1.9 ± 0.4 (p < 0.001), with surgical satisfaction score of 8.7 ± 0.3 out of 10; these were similar across achalasia subtypes. On univariate analysis, female gender, Eckardt score, severity of transit symptoms, and maximal IRP predicted linear GSS improvement; female gender (p = 0.003) and dysphagia for liquids (p = 0.043) remained predictive on multivariate analysis. When a uniform surgical approach is utilized, symptomatic outcome and satisfaction with therapy are similar across achalasia subtypes. Female gender and severity of dysphagia for solids may predict better HM outcome.

Health-related quality of life after laparoscopic Heller myotomy and Dor fundoplication for achalasia.

PubMed

Asti, Emanuele; Sironi, Andrea; Lovece, Andrea; Bonavina, Giulia; Fanelli, Melania; Bonitta, Gianluca; Bonavina, Luigi

2017-04-01

In addition to symptom scores, a person's perception of health and quality of life assessment is an important indicator of quality of treatment and can provide an efficient index to compare different therapeutic modalities in chronic disease states. Only a few studies have investigated quality of life comprehensively in patients with achalasia, and therefore the controversy regarding the best treatment algorithm continues. The primary study outcome was pre- and postoperative quality of life in patients with achalasia undergoing laparoscopic Heller myotomy and Dor fundoplication. The study is a retrospective, observational cohort. The hospital registry and the updated research database were reviewed to identify all patients who were treated for achalasia between 2010 and 2015. Patients were eligible for the study if they had a minimum 1-year follow-up and had pre-and postoperative Eckardt, Short Form-36, and Gastro-Esophageal Reflux Disease Health-Related Quality of Life scores. Patients with previous operative and/or endoscopic treatments for achalasia were excluded. One-hundred and eighteen patients were identified. The median follow-up was 40 months (interquartile range 27). The proportion of patients with Eckardt stage II-III decreased from 94.9-13% (P < .001). The mean Eckardt score decreased from 6.9 ± 1.9 to 1.7 ± 1.2 (P < .001); the mean Short Form-36 scores significantly increased in all 8 domains; the mean Gastro-Esophageal Reflux Disease Health-Related Quality of Life score decreased from 13.9 ± 5.7 to 5.5 ± 5.4 (P < .001). Finally, 88% (confidence interval 81-93) of patients were satisfied regarding their present condition. Quality of life assessed with generic and disease-specific validated instruments significantly improved after laparoscopic Heller myotomy combined with Dor fundoplication. Copyright © 2016 Elsevier Inc. All rights reserved.

Heller Myotomy for Achalasia: Quality of Life Comparison of Laparoscopic and Open Approaches

PubMed Central

Katilius, Marius

2001-01-01

Background: Achalasia is a relatively rare disorder with a variety of treatment options. Although laparoscopic Heller myotomy has become the surgical treatment of choice, little data exist on the overall quality of life of patients undergoing this technique versus standard open approaches. Methods: We prospectively evaluated all patients surgically treated for achalasia by a single surgeon. Laparoscopic Heller myotomy consisted of a long (≥ 6 cm) esophageal cardiomyotomy extending at least 2 cm onto the gastric cardia, with a concomitant Dor fundoplication. Patients were evaluated preoperatively and postoperatively for symptoms and quality of life using the SF-36, a standardized, generic quality of life instrument. Results: A total of 23 patients were surgically treated: 15 patients had a planned laparoscopic procedure, with 3 conversions; 8 had planned open procedures. Dysphagia resolved in 20 of 21 patients, with 1 patient in the laparoscopic group requiring reoperation due to an inadequate gastric myotomy. Compared with preoperative scores, a statistically significant improvement occurred in the general health domain of the SF-36 (70 to 82, P = 0.04). Compared with that in patients undergoing open surgery, the laparoscopic group had better scores in the domains of physical functioning and bodily pain. Conclusions: Laparoscopic Heller myotomy has comparable success to open Heller myotomy, and causes less early detriment to quality of life. This should be the primary treatment in all fit surgical patients with achalasia. PMID:11548827

The early efficacy of Heller myotomy in the treatment of Iranian patients with achalasia.

PubMed

Abdi, Saeed; Forotan, Mojgan; Nikzamir, Abdolrahim; Zomorody, Saeedeh; Jahani-Sherafat, Somayeh

2016-01-01

The purpose of this study was to determine the efficacy of Heller myotomy for the treatment of achalasia in a referral center in Tehran, and investigate the clinical characteristics, manometric results and treatment responses among three achalasia subtypes in Iranian patients. Esophageal achalasia is an unusual swallowing disorder, characterized by high pressure in the lower esophageal sphincter (LES) on swallowing, failure relaxation of the LES and the absence of peristalsis in esophageal. In this cross sectional study, clinical symptom and esophageal manometry before and 2 months after treating with Heller myotomy in 20 patients with achalasia who were referred to Taleghani Hospital, Tehran, in 2013 were evaluated. Patients' demographic, clinical features and response to treatment were analyzed using SPSS software (version 20, Chicago, IL, USA). All the diagnostic criteria measured after the treatment were significantly different (P<0.05) before and after the therapy. The average decline in the length of the esophagus was 1.8 cm and dysphasia score was 7.25 units. Also an average decline in LES Resting Pressure, LES Residual Pressure, PIP, and IRP were 23.2 mmHg, 14.3 mmHg, 3.4 mmHg and 17.8 mmHg, respectively. Results of this study showed that the Heller myotomy is highly effective in relieving dysphasia in patients with achalasia. Also, type II achalasia is the most common subtype of achalasia with a better response to Heller myotomy compared to the other types.

Upper esophageal sphincter (UES) metrics on high-resolution manometry (HRM) differentiate achalasia subtypes.

PubMed

Blais, P; Patel, A; Sayuk, G S; Gyawali, C P

2017-12-01

The upper esophageal sphincter (UES) reflexively responds to bolus presence within the esophageal lumen, therefore UES metrics can vary in achalasia. Within consecutive patients undergoing esophageal high-resolution manometry (HRM), 302 patients (58.2±1.0 year, 57% F) with esophageal outflow obstruction were identified, and compared to 16 asymptomatic controls (27.7±0.7 year, 56% F). Esophageal outflow obstruction was segregated into achalasia subtypes 1, 2, and 3, and esophagogastric junction outflow obstruction (EGJOO with intact peristalsis) using Chicago Classification v3.0. UES and lower esophageal sphincter (LES) metrics were compared between esophageal outflow obstruction and normal controls using univariate and multivariate analysis. Linear regression excluded multicollinearity of pressure metrics that demonstrated significant differences across individual subtype comparisons. LES integrated relaxation pressure (IRP) had utility in differentiating achalasia from controls (P<.0001), but no utility in segregating between subtypes (P=.27). In comparison to controls, patients collectively demonstrated univariate differences in UES mean basal pressure, relaxation time to nadir, recovery time, and residual pressure (UES-RP) (P≤.049). UES-RP was highest in type 2 achalasia (P<.0001 compared to other subtypes and controls). In multivariate analysis, only UES-RP retained significance in comparison between each of the subgroups (P≤.02 for each comparison). Intrabolus pressure was highest in type 3 achalasia; this demonstrated significant differences across some but not all subtype comparisons. Nadir UES-RP can differentiate achalasia subtypes within the esophageal outflow obstruction spectrum, with highest values in type 2 achalasia. This metric likely represents a surrogate marker for esophageal pressurization. © 2017 John Wiley & Sons Ltd.

Laparoscopic esophagomyotomy for achalasia in children: A review

PubMed Central

Pandian, T Kumar; Naik, Nimesh D; Fahy, Aodhnait S; Arghami, Arman; Farley, David R; Ishitani, Michael B; Moir, Christopher R

2016-01-01

Esophageal achalasia in children is rare but ultimately requires endoscopic or surgical treatment. Historically, Heller esophagomyotomy has been recommended as the treatment of choice. The refinement of minimally invasive techniques has shifted the trend of treatment toward laparoscopic Heller myotomy (LHM) in adults and children with achalasia. A review of the available literature on LHM performed in patients < 18 years of age was conducted. The pediatric LHM experience is limited to one multi-institutional and several single-institutional retrospective studies. Available data suggest that LHM is safe and effective. There is a paucity of evidence on the need for and superiority of concurrent antireflux procedures. In addition, a more complete portrayal of complications and long-term (> 5 years) outcomes is needed. Due to the infrequency of achalasia in children, these characteristics are unlikely to be defined without collaboration between multiple pediatric surgery centers. The introduction of peroral endoscopic myotomy and single-incision techniques, continue the trend of innovative approaches that may eventually become the standard of care. PMID:26839646

Peroral endoscopic myotomy for treatment of Guillain-Barre syndrome-associated achalasia: A rare case

PubMed Central

Shin, Seung Kak; Kim, Kyoung Oh; Kim, Eui Joo; Kim, Su Young; Kim, Jung Ho; Kim, Yoon Jae; Chung, Jun-Won; Kwon, Kwang An; Park, Dong Kyun

2017-01-01

Guillain-Barre syndrome (GBS)-associated achalasia is a very rare disease of uncertain cause. We report the case of a patient diagnosed with GBS-associated type I achalasia who was successfully treated with peroral endoscopic myotomy (POEM). A 30-year-old man who was diagnosed with GBS 3 mo before was referred to our department with dysphagia and meal-related regurgitation. The results of esophagography, endoscopy, and high-resolution manometry (HRM) revealed type I achalasia. POEM that utilized a submucosal tunneling technique was performed to treat the GBS-associated type I achalasia. After POEM, smooth passage of a contrast agent into the stomach was shown in follow-up esophagography, and follow-up HRM revealed a decrease in the mean integrated relaxation pressure 22.9 mmHg to 9.6 mmHg. The patient remained without dysphagia for 7 mo, even though the patient’s neurological problems were not fully resolved. POEM may be a safe and effective treatment for GBS-associated type I achalasia. PMID:28223738

Clinical, Endoscopic, and Radiologic Features of Three Subtypes of Achalasia, Classified Using High-Resolution Manometry

PubMed Central

Khan, Mohammed Q.; AlQaraawi, Abdullah; Al-Sohaibani, Fahad; Al-Kahtani, Khalid; Al-Ashgar, Hamad I.

2015-01-01

Background/Aims: High-resolution manometry (HRM) has improved the accuracy of manometry in detecting achalasia and determining its subtypes. However, the correlation of achalasia subtypes with clinical, endoscopic, and radiologic findings has not been assessed. We aimed to evaluate and compare the clinical, endoscopic, and fluoroscopy findings associated with three subtypes of achalasia using HRM. Patients and Methods: The retrospective clinical data, HRM, endoscopy, and radiologic findings were obtained from the medical records of untreated achalasia patients. Results: From 2011 to 2013, 374 patients underwent HRM. Fifty-two patients (14%) were diagnosed with achalasia, but only 32 (8.5%) of these patients had not received treatment and were therefore included in this study. The endoscopy results were normal in 28% of the patients, and a barium swallow was inconclusive in 31% of the achalasia patients. Ten patients (31%) were classified as having type I achalasia, 17 (53%) were classified as type II, and 5 (16%) were classified as type III. Among the three subtypes, type I patients were on average the youngest and had the longest history of dysphagia, mildest chest pain, most significant weight loss, and most dilated esophagus with residual food. Chest pain was most common in type III patients, and frequently had normal fluoroscopic and endoscopic results. Conclusion: The clinical, radiologic, and endoscopic findings were not significantly different between patients with type I and type II untreated achalasia. Type III patients had the most severe symptoms and were the most difficult to diagnose based on varied clinical, radiologic, and endoscopic findings. PMID:26021774

Recurrent achalasia treated with Heller myotomy: A review of the literature

PubMed Central

Wang, Lan; Li, You-Ming

2008-01-01

AIM: To evaluate the efficacy and safety of Heller myotomy (HM) for recurrent achalasia, performed after different methods of first-line treatment. METHODS: We searched for studies published in PubMed from 1966 to March 2008 on treatment of recurrent achalasia with HM after failure with different methods of first-line treatment. The efficacy of HM was assessed by a pooled estimate of response rate with individual studies weighted proportionally to sample size. RESULTS: Sixteen studies were eligible and included in the review. The results showed that HM has a better remission rate for recurrent achalasia after failure of HM [weighted mean (SD)] of 86.9% (21.8%) compared with 81.6% (23.8%) for pneumatic dilatation (PD). One study evaluated the efficacy of HM after failure of PD combined with botulinum toxin injection (83%). The most common complications were perforation and gastroesophageal reflux. CONCLUSION: HM has the best efficacy in patients with recurrent achalasia who were treated with HM as first-line treatment. Future studies should focus on how to increase the success rate and decrease the complications of HM. PMID:19084921

Intra- and interrater reliability of the Chicago Classification of achalasia subtypes in pediatric high-resolution esophageal manometry (HRM) recordings.

PubMed

Singendonk, M M J; Rosen, R; Oors, J; Rommel, N; van Wijk, M P; Benninga, M A; Nurko, S; Omari, T I

2017-11-01

Subtyping achalasia by high-resolution manometry (HRM) is clinically relevant as response to therapy and prognosis have shown to vary accordingly. The aim of this study was to assess inter- and intrarater reliability of diagnosing achalasia and achalasia subtyping in children using the Chicago Classification (CC) V3.0. Six observers analyzed 40 pediatric HRM recordings (22 achalasia and 18 non-achalasia) twice by using dedicated analysis software (ManoView 3.0, Given Imaging, Los Angeles, CA, USA). Integrated relaxation pressure (IRP4s), distal contractile integral (DCI), intrabolus pressurization pattern (IBP), and distal latency (DL) were extracted and analyzed hierarchically. Cohen's κ (2 raters) and Fleiss' κ (>2 raters) and the intraclass correlation coefficient (ICC) were used for categorical and ordinal data, respectively. Based on the results of dedicated analysis software only, intra- and interrater reliability was excellent and moderate (κ=0.89 and κ=0.52, respectively) for differentiating achalasia from non-achalasia. For subtyping achalasia, reliability decreased to substantial and fair (κ=0.72 and κ=0.28, respectively). When observers were allowed to change the software-driven diagnosis according to their own interpretation of the manometric patterns, intra- and interrater reliability increased for diagnosing achalasia (κ=0.98 and κ=0.92, respectively) and for subtyping achalasia (κ=0.79 and κ=0.58, respectively). Intra- and interrater agreement for diagnosing achalasia when using HRM and the CC was very good to excellent when results of automated analysis software were interpreted by experienced observers. More variability was seen when relying solely on the software-driven diagnosis and for subtyping achalasia. Therefore, diagnosing and subtyping achalasia should be performed in pediatric motility centers with significant expertise. © 2017 John Wiley & Sons Ltd.

The Functional Lumen Imaging Probe Detects Esophageal Contractility not Observed with Manometry in Patients with Achalasia

PubMed Central

Carlson, Dustin A.; Lin, Zhiyue; Kahrilas, Peter J.; Sternbach, Joel; Donnan, Erica N.; Friesen, Laurel; Listernick, Zoe; Mogni, Benjamin; Pandolfino, John E.

2015-01-01

Background & Aims The functional lumen imaging probe (FLIP) could improve characterization of achalasia subtypes by detecting non-occlusive esophageal contractions not observed with standard manometry. We aimed to evaluate for esophageal contractions during volumetric distention in patients with achalasia using FLIP topography. Methods Fifty one treatment-naïve patients with achalasia, defined and sub-classified by high-resolution esophageal pressure topography, and 10 asymptomatic individuals (controls) were evaluated with the FLIP during endoscopy. During stepwise distension, simultaneous intra-bag pressures and 16 channels of cross-sectional areas were measured; data were exported to software that generated FLIP topography plots. Esophageal contractility was identified by noting periods of reduced luminal diameter. Esophageal contractions were further characterized by propagation direction, repetitiveness, and based on whether they were occluding or non-occluding. Results Esophageal contractility was detected in all 10 controls: 8/10 had repetitive, antegrade, contractions and 9/10 had occluding contractions. Contractility was detected in 27% (4/15) of patients with type I achalasia and 65% (18/26, including 9 with occluding contractions) of patients with type II achalasia. Contractility was detected in all 10 patients with type III achalasia; 8 of these patients had a pattern of contractility not observed in controls (repetitive, retrograde contractions). Conclusions Esophageal contractility not observed with manometry can be detected in patients with achalasia using FLIP topography. The presence and patterns of contractility detected with FLIP topography may represent variations in pathophysiology, such as mechanisms of pan-esophageal pressurization in patients with type II achalasia. These findings could have implications for additional sub-classification to supplement prediction of the achalasia disease course. PMID:26278501

[Focus on Achalasia].

PubMed

Guillaumot, Marie-Anne; Barret, Maximilien; Leblanc, Sarah; Leconte, Mahaut; Dousset, Bertrand; Oudjit, Ammar; Prat, Frédéric; Chaussade, Stanislas

2018-01-01

The pathophysiology of achalasia is largely unknown, and involves the destruction of ganglion cell in the esophageal myenteric plexus. High-resolution esophageal manometry is the key investigation. Endoscopic pneumodilatation and laparoscopic Heller myotomy have comparable short-term success rates, around 90%. The main complication after pneumodilatation is esophageal perforation, occurring in about 1% of cases. Peroral endoscopic myotomy is a promising treatment modality, however with frequent post-procedural gastroesophageal reflux. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

The early efficacy of Heller myotomy in the treatment of Iranian patients with achalasia

PubMed Central

Abdi, Saeed; Forotan, Mojgan; Nikzamir, Abdolrahim; Zomorody, Saeedeh; Jahani-Sherafat, Somayeh

2016-01-01

Aim: The purpose of this study was to determine the efficacy of Heller myotomy for the treatment of achalasia in a referral center in Tehran, and investigate the clinical characteristics, manometric results and treatment responses among three achalasia subtypes in Iranian patients. Background: Esophageal achalasia is an unusual swallowing disorder, characterized by high pressure in the lower esophageal sphincter (LES) on swallowing, failure relaxation of the LES and the absence of peristalsis in esophageal. Patients and methods: In this cross sectional study, clinical symptom and esophageal manometry before and 2 months after treating with Heller myotomy in 20 patients with achalasia who were referred to Taleghani Hospital, Tehran, in 2013 were evaluated. Patients’ demographic, clinical features and response to treatment were analyzed using SPSS software (version 20, Chicago, IL, USA). Results: All the diagnostic criteria measured after the treatment were significantly different (P<0.05) before and after the therapy. The average decline in the length of the esophagus was 1.8 cm and dysphasia score was 7.25 units. Also an average decline in LES Resting Pressure, LES Residual Pressure, PIP, and IRP were 23.2 mmHg, 14.3 mmHg, 3.4 mmHg and 17.8 mmHg, respectively. Conclusion: Results of this study showed that the Heller myotomy is highly effective in relieving dysphasia in patients with achalasia. Also, type II achalasia is the most common subtype of achalasia with a better response to Heller myotomy compared to the other types. PMID:26744612

Autism and esophageal achalasia in childhood: a possible correlation? Report on three cases.

PubMed

Betalli, P; Carretto, E; Cananzi, M; Zanatta, L; Salvador, R; Galeazzi, F; Guariso, G; Gamba, P; Costantini, M

2013-04-01

Chronic gastrointestinal symptoms are commonly reported in autistic patients. Dysphagia is often present, and it is generally related to behavioral eating disorders. The association between autism and esophageal achalasia has not been described in literature yet. We report our experience with three cases of autistic children we recently treated for esophageal achalasia. In the first case (a 14-year-old male), achalasia was diagnosed with barium swallow and esophageal manometry and was successfully treated with three pneumatic endoscopic dilatations (follow-up: 3 years). In the second case (a 12-year-old female), achalasia was diagnosed with barium swallow and esophageal manometry and was treated with Heller myotomy after two unsuccessful pneumatic endoscopic attempts (follow-up: 3 months). In the last case, a 15-year-old male underwent barium swallow and endoscopy that confirmed achalasia. He was treated with Heller myotomy, and he is asymptomatic at a 6-month follow-up. To our knowledge, this is the first report of a possible association between autism and esophageal achalasia. Because of the rarity of both diseases, their association in the same patient is unlikely to be casual even if speculation on their common etiology is impossible at present. This finding needs further confirmation, but it is sufficient, in our opinion, to indicate proper evaluation with barium swallow and/or manometry in any autistic children with eating difficulty. © 2012 Copyright the Authors. Journal compilation © 2012, Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.

Association Between Gastroesophageal Reflux Disease After Pneumatic Balloon Dilatation and Clinical Course in Patients With Achalasia

PubMed Central

Min, Yang Won; Lee, Jin Hee; Min, Byung-Hoon; Lee, Jun Haeng; Kim, Jae J; Rhee, Poong-Lyul

2014-01-01

Background/Aims The occurrence of gastroesophageal reflux disease (GERD) is known to be associated with lower post-treatment lower esophageal sphincter pressure in patients with achalasia. This study aimed to elucidate whether GERD after pneumatic balloon dilatation (PD) has a prognostic role and to investigate how the clinical course of GERD is. Methods A total of 79 consecutive patients who were first diagnosed with primary achalasia and underwent PD as an initial treatment were included in this retrospective study. Single PD was performed using a 3.0 cm balloon. The patients were divided into two groups: 1) who developed GERD after PD (GERD group) and 2) who did not develop GERD after PD (non-GERD group). GERD was defined as pathological acid exposure, reflux esophagitis or typical reflux symptoms. Results Twenty one patients (26.6%) developed GERD after PD during follow-up. There were no significant differences between the two groups in demographic or clinical factors including pre- and post-treatment manometric results. All patients in GERD group were well responsive to maintenance proton pump inhibitor therapy including on demand therapy or did not require maintenance. During a median follow-up of 17.8 months (interquartile range, 7.1–42.7 months), achalasia recurred in 15 patients (19.0%). However, the incidence of recurrence did not differ according to the occurrence of GERD after PD. Conclusions GERD often occurs after even a single PD for achalasia. However, GERD after PD is well responsive to PPI therapy. Our data suggest that GERD after PD during follow-up does not appear to have a prognostic role. PMID:24840373

Esophagectomy for End-Stage Achalasia: Systematic Review and Meta-analysis.

PubMed

Aiolfi, Alberto; Asti, Emanuele; Bonitta, Gianluca; Bonavina, Luigi

2018-05-01

Indications for surgery and clinical outcomes of esophagectomy in the management of end-stage achalasia are not clearly defined. The aim of this systematic review and meta-analysis was to provide evidence-based information to help in the decision-making and in the choice of surgical technique. An extensive literature search was conducted to identify all reports on esophagectomy for end-stage achalasia patients over the past three decades. MEDLINE, Embase and Cochrane databases were thoroughly consulted matching the terms "achalasia," "end-stage achalasia," "esophagectomy" and "esophageal resection" with "AND" and "OR." Short- and long-term outcome data were extracted. Pooled prevalence of pneumonia, anastomotic leakage and mortality were calculated using Freeman-Tukey double arcsine transformation and DerSimonian-Laird estimator in random effect meta-analysis. Heterogeneity among studies was evaluated using I 2 -index and Cochrane Q test. Meta-regression was used to address the effect of potential confounders. Eight papers published between 1989 and 2014 matched the inclusion criteria. In total, 1307 patients were included. Esophagectomy was performed through a transthoracic (78.7%) or a transhiatal (21.3%) approach. The stomach was used as an esophageal substitute in 95% of patients. Pooled prevalence of pneumonia, anastomotic leakage and mortality were 10% (95% CI 4-18%), 7% (95% CI 4-10%) and 2% (95% CI 1-3%), respectively. Esophagectomy for end-stage achalasia is safe and effective. Based on the results of this study, esophagectomy should be performed without hesitation in patients who are fit for major surgery and present with disabling symptoms, poor quality of life and dolichomegaesophagus recalcitrant to multiple endoscopic dilatations and/or surgical myotomies.

Clinical application of endoscopic ultrasonography for esophageal achalasia.

PubMed

Minami, Hitomi; Inoue, Haruhiro; Isomoto, Hajime; Urabe, Shigetoshi; Nakao, Kazuhiko

2015-04-01

Endoscopic ultrasonography (EUS) has been widely used for evaluating the nature of diseases of various organs. The possibility of applying EUS for esophageal motility diseases has not been well discussed despite its versatility. At present, peroral endoscopic myotomy (POEM) for esophageal achalasia and related diseases has brought new attention to esophageal diseases because POEM provides a more direct approach to the inner structures of the esophageal wall. In the present study, we discuss the clinical utility of EUS in evaluating and treating esophageal motility diseases such as esophageal achalasia and related diseases. © 2015 The Authors. Digestive Endoscopy © 2015 Japan Gastroenterological Endoscopy Society.

Peroral Endoscopic Myotomy for Treating Achalasia and Esophageal Motility Disorders

PubMed Central

Youn, Young Hoon; Minami, Hitomi; Chiu, Philip Wai Yan; Park, Hyojin

2016-01-01

Peroral endoscopic myotomy (POEM) is the application of esophageal myotomy to the concept of natural orifice transluminal surgery (NOTES) by utilizing a submucosal tunneling method. Since the first case of POEM was performed for treating achalasia in Japan in 2008, this procedure is being more widely used by many skillful endosopists all over the world. Currently, POEM is a spotlighted, emerging treatment option for achalasia, and the indications for POEM are expanding to include long-standing, sigmoid shaped esophagus in achalasia, even previously failed endoscopic treatment or surgical myotomy, and other spastic esophageal motility disorders. Accumulating data about POEM demonstrate excellent short-term outcomes with minimal risk of major adverse events, and some existing long-term data show the efficacy of POEM to be long lasting. In this review article, we review the technical details and clinical outcomes of POEM, and discuss some considerations of POEM in special situations. PMID:26717928

The Functional Lumen Imaging Probe Detects Esophageal Contractility Not Observed With Manometry in Patients With Achalasia.

PubMed

Carlson, Dustin A; Lin, Zhiyue; Kahrilas, Peter J; Sternbach, Joel; Donnan, Erica N; Friesen, Laurel; Listernick, Zoe; Mogni, Benjamin; Pandolfino, John E

2015-12-01

The functional lumen imaging probe (FLIP) could improve the characterization of achalasia subtypes by detecting nonocclusive esophageal contractions not observed with standard manometry. We aimed to evaluate esophageal contractions during volumetric distention in patients with achalasia using FLIP topography. Fifty-one treatment-naive patients with achalasia, defined and subclassified by high-resolution esophageal pressure topography, and 10 asymptomatic individuals (controls) were evaluated with the FLIP during endoscopy. During stepwise distension, simultaneous intrabag pressures and 16 channels of cross-sectional areas were measured; data were exported to software that generated FLIP topography plots. Esophageal contractility was identified by noting periods of reduced luminal diameter. Esophageal contractions were characterized further by propagation direction, repetitiveness, and based on whether they were occluding or nonoccluding. Esophageal contractility was detected in all 10 controls: 8 of 10 had repetitive antegrade contractions and 9 of 10 had occluding contractions. Contractility was detected in 27% (4 of 15) of patients with type I achalasia and in 65% (18 of 26, including 9 with occluding contractions) of patients with type II achalasia. Contractility was detected in all 10 patients with type III achalasia; 8 of these patients had a pattern of contractility that was not observed in controls (repetitive retrograde contractions). Esophageal contractility not observed with manometry can be detected in patients with achalasia using FLIP topography. The presence and patterns of contractility detected with FLIP topography may represent variations in pathophysiology, such as mechanisms of panesophageal pressurization in patients with type II achalasia. These findings could have implications for additional subclassification to supplement prediction of the achalasia disease course. Copyright © 2015 AGA Institute. Published by Elsevier Inc. All rights

Data analyses and perspectives on laparoscopic surgery for esophageal achalasia.

PubMed

Tsuboi, Kazuto; Omura, Nobuo; Yano, Fumiaki; Hoshino, Masato; Yamamoto, Se-Ryung; Akimoto, Shunsuke; Masuda, Takahiro; Kashiwagi, Hideyuki; Yanaga, Katsuhiko

2015-10-14

In general, the treatment methods for esophageal achalasia are largely classified into four groups, including drug therapy using nitrite or a calcium channel blocker, botulinum toxin injection, endoscopic therapy such as endoscopic balloon dilation, and surgery. Various studies have suggested that the most effective treatment of esophageal achalasia is surgical therapy. The basic concept of this surgical therapy has not changed since Heller proposed esophageal myotomy for the purpose of resolution of lower esophageal obstruction for the first time in 1913, but the most common approach has changed from open-chest surgery to laparoscopic surgery. Currently, the laparoscopic surgery has been the procedure of choice for the treatment of esophageal achalasia. During the process of the transition from open-chest surgery to laparotomy, to thoracoscopic surgery, and to laparoscopic surgery, the necessity of combining antireflux surgery has been recognized. There is some debate as to which type of antireflux surgery should be selected. The Toupet fundoplication may be the most effective in prevention of postoperative antireflux, but many medical institutions have selected the Dor fundoplication which covers the mucosal surface exposed by myotomy. Recently, a new endoscopic approach, peroral endoscopic myotomy (POEM), has received attention. Future studies should examine the long-term outcomes and whether POEM becomes the gold standard for the treatment of esophageal achalasia.

Data analyses and perspectives on laparoscopic surgery for esophageal achalasia

PubMed Central

Tsuboi, Kazuto; Omura, Nobuo; Yano, Fumiaki; Hoshino, Masato; Yamamoto, Se-Ryung; Akimoto, Shunsuke; Masuda, Takahiro; Kashiwagi, Hideyuki; Yanaga, Katsuhiko

2015-01-01

In general, the treatment methods for esophageal achalasia are largely classified into four groups, including drug therapy using nitrite or a calcium channel blocker, botulinum toxin injection, endoscopic therapy such as endoscopic balloon dilation, and surgery. Various studies have suggested that the most effective treatment of esophageal achalasia is surgical therapy. The basic concept of this surgical therapy has not changed since Heller proposed esophageal myotomy for the purpose of resolution of lower esophageal obstruction for the first time in 1913, but the most common approach has changed from open-chest surgery to laparoscopic surgery. Currently, the laparoscopic surgery has been the procedure of choice for the treatment of esophageal achalasia. During the process of the transition from open-chest surgery to laparotomy, to thoracoscopic surgery, and to laparoscopic surgery, the necessity of combining antireflux surgery has been recognized. There is some debate as to which type of antireflux surgery should be selected. The Toupet fundoplication may be the most effective in prevention of postoperative antireflux, but many medical institutions have selected the Dor fundoplication which covers the mucosal surface exposed by myotomy. Recently, a new endoscopic approach, peroral endoscopic myotomy (POEM), has received attention. Future studies should examine the long-term outcomes and whether POEM becomes the gold standard for the treatment of esophageal achalasia. PMID:26478674

Short fourth and fifth metacarpals in a case of idiopathic primary hypoparathyroidism

PubMed Central

Valizadeh, Neda; Mehdizadeh, Alireza; Nazarbaghi, Surena

2013-01-01

Shortening of metacarpals is a useful diagnostic marker in patients with pseudohypoparathyroidism type Ia (PHP-Ia) with Albright's hereditary osteodystrophy (AHO) phenotype or pseudopseudohypoparathyroidism (PPHP). There are very rare reports of metacarpals shortening in idiopathic primary hypoparathyroidism (IPH) cases in the literature. Here we described a young woman with IPH who presented with hypocalcaemia and generalized tonic-clonic seizure. She had shortening of forth and fifth metacarpals which was prominent in her right hand. Based on our finding and other previous case reports we conclude that metarpals shortening is not a specific finding of PHP-Ia or PPHP and it may be found in IPH cases. PMID:24083181

The Impact of Heller Myotomy on Integrated Relaxation Pressure in Esophageal Achalasia.

PubMed

Salvador, Renato; Savarino, Edoardo; Pesenti, Elisa; Spadotto, Lorenzo; Capovilla, Giovanni; Cavallin, Francesco; Galeazzi, Francesca; Nicoletti, Loredana; Merigliano, Stefano; Costantini, Mario

2016-01-01

A new high-resolution manometry (HRM) parameter, the integrated relaxation pressure (IRP), has been proposed for the assessment of esophageal-gastric junction (EGJ) relaxation. Our aim was to assess the effect of Heller myotomy on IRP in achalasia patients. We prospectively collected data on achalasia patients who underwent HRM between 2009-2014. Barium swallow was used to assess esophageal diameter and shape. Manometric diagnoses were performed by using the Chicago Classification v3. All patients with a confirmed diagnosis of achalasia were treated surgically with Heller Myotomy One hundred thirty-nine consecutive achalasia patients (M:F = 72:67) represented the study population. All the patients had 100% simultaneous waves but 11 had an IRP < 15 mmHg. At median follow-up of 28 months, the median of IRP was significantly lower after surgery (27.4 [IQR 20.4-35] vs 7.1 [IQR 4.4-9.8]; p < 0.001), and so were the lower esophageal sphincter (LES) resting pressure (27 [IQR 18-33] vs 6 [IQR 3-11]; p < 0.001). At univariate analysis, IRP correlated with the gender, LES resting residual pressure, and dysphagia score. This is the first study to have examined the role of IRP in achalasia, and how it changes after surgical treatment. An increased preoperative IRP correlated directly with a more severe dysphagia. The IRP was restored to normal by Heller myotomy.

Surgery or Peroral Esophageal Myotomy for Achalasia

PubMed Central

Marano, Luigi; Pallabazzer, Giovanni; Solito, Biagio; Santi, Stefano; Pigazzi, Alessio; De Luca, Raffaele; Biondo, Francesco Giuseppe; Spaziani, Alessandro; Longaroni, Maurizio; Di Martino, Natale; Boccardi, Virginia; Patriti, Alberto

2016-01-01

Abstract To date very few studies with small sample size have compared peroral esophageal myotomy (POEM) with the current surgical standard of care, laparoscopic Heller myotomy (LHM), in terms of efficacy and safety, and no recommendations have been proposed. To investigate the efficacy and safety of POEM compared with LHM, for the treatment of achalasia. The databases of Pubmed, Medline, Cochrane, and Ovid were systematically searched between January 1, 2005 and January 31, 2015, with the medical subject headings (MeSH) and keywords “achalasia,” “POEM,” “per oral endoscopic myotomy,” and “peroral endoscopic myotomy,” “laparoscopic Heller myotomy” (LHM), “Heller myotomy.” All types of study designs including adult patients with diagnosis of achalasia were selected. Studies that did not report the comparison between endoscopic and surgical treatment, experimental studies in animal models, single case reports, technical reports, reviews, abstracts, and editorials were excluded. The total number of included patients was 486 (196 in POEM group and 290 in LHM group). There were no differences between POEM and LHM in reduction in Eckardt score (MD = −0.659, 95% CI: −1.70 to 0.38, P = 0.217), operative time (MD = −0.354, 95% CI: −1.12 to 0.41, P = 0.36), postoperative pain scores (MD = −1.86, 95% CI: −5.17 to 1.44, P = 0.268), analgesic requirements (MD = −0.74, 95% CI: −2.65 to 1.16, P = 0.445), and complications (OR = 1.11, 95% CI: 0.5–2.44, P = 0.796). Length of hospital stay was significantly lower for POEM (MD = −0.629, 95% CI: −1.256 to −0.002, P = 0.049). There was a trend toward significant reduction in symptomatic gastroesophageal reflux rate in favors of LHM compared to POEM group (OR = 1.81, 95% CI: 1.11–2.95, P = 0.017). All included studied were not randomized. Furthermore all selected studies did not report the results of follow-up longer than 1 year and

Surgery for achalasia: 1998.

PubMed

Shiino, Y; Filipi, C J; Awad, Z T; Tomonaga, T; Marsh, R E

1999-01-01

Technical controversies abound regarding the surgical treatment of achalasia. To determine the value of a concomitant antireflux procedure, the best antireflux procedure, the correct length for gastric myotomy, the optimal surgical approach (thoracic or abdominal), and the equivalency of minimally invasive surgery, a literature review was carried out. The review is based on 23 articles on open transabdominal or transthoracic myotomy, 14 articles on laparoscopic myotomy, and four articles on thoracoscopic myotomy. Postoperative results of traditional open thoracic or transabdominal myotomy as determined by symptomatology were better with fundoplication than without fundoplication. The incidence of postoperative reflux as proved by pH monitoring was high in patients who had an open transabdominal myotomy without fundoplication. The type of antireflux procedure used and the length of gastric myotomy had little effect on results. The results of transthoracic Heller myotomy do not require a concomitant fundoplication. Laparoscopic and thoracoscopic myotomy had excellent results at short-term follow-up. A fundoplication must be added if the myotomy is performed transabdominally. A randomized prospective study is required to determine the best fundoplication and the extent of gastric myotomy. Although minimally invasive surgery for achalasia has excellent initial results, longer follow-up in a larger population of patients is needed.

The Effect of Race in Patients with Achalasia Diagnosed With High-Resolution Esophageal Manometry.

PubMed

Chedid, Victor; Rosenblatt, Elizabeth; Gandhi, Kunjal Komal; Dhalla, Sameer; Nandwani, Monica C; Stein, Ellen M; Clarke, John O

2018-02-01

The advent of the Chicago Classification for esophageal motility disorders allowed for clinically reproducible subgrouping of patients with achalasia based on manometric phenotype. However, there are limited data with regards to racial variation using high-resolution esophageal manometry (HREM). The aim of our study was to evaluate the racial differences in patients with achalasia diagnosed with HREM using the Chicago Classification. We evaluated the clinical presentation, treatment decisions and outcomes between blacks and non-blacks with achalasia to identify potential racial disparities. We performed a retrospective review of consecutive patients referred for HREM at a single tertiary referral center from June 2008 through October 2012. All patients diagnosed with achalasia on HREM according to the Chicago Classification were included. Demographic, clinical and manometric data were abstracted. All studies interpreted before the Chicago Classification was in widespread use were reanalyzed. Race was defined as black or non-black. Patients who had missing data were excluded. Proportions were compared using chi-squared analysis and means were compared using the Student's t-test. A total of 1,268 patients underwent HREM during the study period, and 105 (8.3%) were manometrically diagnosed with achalasia (53% female, mean age: 53.8 ± 17.0 years) and also met the aforementioned inclusion and exclusion criteria. A higher percentage of women presented with achalasia in blacks as compared to whites or other races (P < 0.001). Non-blacks were more likely to present with reflux than blacks (P = 0.01), while blacks were more likely to be treated on the inpatient service than non-blacks (P < 0.001). There were no other significant differences noted in clinical presentation, treatment decisions and treatment outcomes among blacks and non-blacks. Our study highlights possible racial differences between blacks and non-blacks, including a higher proportion of black women

Incidence of Esophageal Carcinomas After Surgery for Achalasia: Usefulness of Long-Term and Periodic Follow-up.

PubMed

Ota, Masaho; Narumiya, Kosuke; Kudo, Kenji; Yagawa, Yohsuke; Maeda, Shinsuke; Osugi, Harushi; Yamamoto, Masakazu

2016-11-14

BACKGROUND Patients with esophageal achalasia are considered to be a high-risk group for esophageal carcinoma, and it has been reported that this cancer often arises at a long interval after surgery for achalasia. However, it is unclear whether esophageal carcinoma is frequent when achalasia has been treated successfully and the patient is without dysphagia. In this study, we reviewed patients with esophageal carcinoma who were detected by regular follow-up after surgical treatment of achalasia.   CASE REPORT Esophageal cancer was detected by periodic upper GI endoscopy in 6 patients. Most of them had early cancers that were treated by endoscopic resection. All 6 patients had undergone surgery for achalasia and the outcome had been rated as excellent or good. Annual follow-up endoscopy was done and the average duration of follow-up until cancer was seen after surgery was 14.3 years (range: 5 to 40 years). Five patients had early cancer. Four cases had multiple lesions.   CONCLUSIONS In conclusion, surgery for achalasia usually improves passage symptoms, but esophageal cancer still arises in some cases and the number of tumors occurring many years later is not negligible. Accordingly, long-term endoscopic follow-up is needed for detection of malignancy at an early stage.

Trypanosoma cruzi Detection in Colombian Patients with a Diagnosis of Esophageal Achalasia.

PubMed

Panesso-Gómez, Santiago; Pavia, Paula; Rodríguez-Mantilla, Iván Enrique; Lasso, Paola; Orozco, Luis A; Cuellar, Adriana; Puerta, Concepción J; Mendoza de Molano, Belén; González, John M

2018-03-01

Achalasia is a motility disorder of the esophagus that might be secondary to a chronic Trypanosoma cruzi infection. Several studies have investigated esophageal achalasia in patients with Chagas disease (CD) in Latin America, but no related studies have been performed in Colombia. The goals of the present study were to determine the presence of anti- T. cruzi antibodies in patients with esophageal achalasia who visited a referral hospital in Bogotá, Colombia, and to detect the presence of the parasite and its discrete typing units (DTUs). This cross-sectional study was conducted in adult patients (18-65 years old) who were previously diagnosed with esophageal achalasia and from whom blood was drawn to assess antibodies against T. cruzi using four different serological tests. Trypanosoma cruzi DNA was detected by conventional polymerase chain reaction (cPCR) and quantitative polymerase chain reaction (qPCR). In total, 38 patients, with an average age of 46.6 years (standard deviation of ±16.2) and comprising 16 men and 22 women, were enrolled. Five (13.15%) patients were found to be positive for anti- T. cruzi antibodies by indirect immunofluorescence assay (IFA), and two patients who were negative according to IFA were reactive by both enzyme-linked immunosorbent assay and immunoblot (5.3%). Parasite DNA was detected in two of these seven patients by cPCR and in one of these by qPCR. The parasite DTU obtained was TcI. In summary, this study identified T. cruzi in Colombian patients with esophageal achalasia, indicating that digestive compromise could also be present in patients with chronic CD.

Training in peroral endoscopic myotomy (POEM) for esophageal achalasia

PubMed Central

Eleftheriadis, Nicholas; Inoue, Haruhiro; Ikeda, Haruo; Onimaru, Manabu; Yoshida, Akira; Hosoya, Toshihisa; Maselli, Roberta; Kudo, Shin-ei

2012-01-01

Peroral endoscopic myotomy (POEM) has been developed in the context of natural orifice transluminal endoscopic surgery (NOTES) as a minimally invasive endoscopic treatment for symptomatic esophageal achalasia, which is a chronic progressive benign disease with severe morbidity and difficult management. Since September 2008, POEM has been successfully performed in more than 200 consecutive patients with symptomatic achalasia at the Digestive Disease Center of Showa University, Northern Yokohama Hospital, Yokohama, Japan, with excellent short- and long-term results and absence of serious complications. International experience of POEM within clinical studies is also promising. According to these results, POEM is considered as a safe procedure that can be applied to all achalasia patients. However, the low incidence of achalasia (0.3%–1% per 100,000 population), in combination with the potential serious complications related to the technically demanding POEM procedure, has made training difficult. There is therefore an urgent need for an animal model for training to decrease the learning curve. Further, there are other ethical and training issues to address. The pig is the most appropriate animal model for training in POEM due to its anatomy being similar to that of humans. The porcine esophagus has the advantage of easy mobilization due to absence of tight junctions to surrounding organs. A non-survival porcine model would be a simple, inexpensive, and reproducible animal model for training in POEM, without the need for concern about complications. A possible training process might first involve observation of POEM performed by specialists, then training on non-survival and survival porcine models, followed by training in humans under specialist guidance and finally, performance of POEM in humans. PMID:22888256

Heller myotomy for achalasia. From the open to the laparoscopic approach.

PubMed

Allaix, Marco E; Patti, Marco G

2015-07-01

The last three decades have witnessed a progressive evolution in the surgical treatment of esophageal achalasia, with a shift from open to a minimally invasive Heller myotomy. The laparoscopic approach is currently the standard of care with better short-term outcomes and similar long-term functional results when compared to open surgery. More recently, the laparoscopic single-site approach and the use of the robot have been proposed to further improve the surgical outcome in achalasia patients.

Laparoscopic Heller Myotomy versus Per Oral Endoscopic Myotomy: Evidence-Based Approach to the Treatment of Esophageal Achalasia.

PubMed

Schlottmann, Francisco; Patti, Marco G

2018-04-01

Esophageal achalasia is a rare disorder characterized by a failure of the lower esophageal sphincter to relax during swallowing, combined with aperistalsis of the esophageal body. Treatment is not curative, but aims to eliminate the outflow resistance caused by the nonrelaxing lower esophageal sphincter. Current evidence suggests that both laparoscopic Heller myotomy and per oral endoscopic myotomy (POEM) are very effective in the relief of symptoms in patients with achalasia. Specifically, for type III achalasia, POEM may achieve higher success rates. However, POEM is associated to a very high incidence of pathologic reflux, with the risk of exchanging one disease-achalasia-with another-gastroesophageal reflux.

Per-oral endoscopic myotomy (POEM) for esophageal achalasia.

PubMed

Pescarus, Radu; Shlomovitz, Eran; Swanstrom, Lee L

2014-01-01

Per-oral endoscopic myotomy (POEM) is a new minimally invasive endoscopic treatment for achalasia. Since the first modern human cases were published in 2008, around 2,000 cases have been performed worldwide. This technique requires advanced endoscopic skills and a learning curve of at least 20 cases. POEM is highly successful with over 90 % improvement in dysphagia while offering patients the advantage of a low impact endoscopic access. The main long-term complication is gastroesophageal reflux (GER) with an estimated incidence of 35 %, similar to the incidence of GER post-laparoscopic Heller with fundoplication. Although POEM represents a paradigm shift in the treatment of achalasia, more long-term data are clearly needed to further define its role in the treatment algorithm of this rare disease.

For patients with primary achalasia the clinical success of pneumatic balloon dilatation can be predicted from the residual fraction of radionuclide during esophageal transit scintigraphy.

PubMed

Jeon, Han Ho; Youn, Young Hoon; Rhee, Kwangwon; Kim, Jie-Hyun; Park, Hyojin; Conklin, Jeffrey L

2014-02-01

Esophageal transit scintigraphy (ETS) and esophagography have long been used to evaluate patients with achalasia. The objectives of our study were to evaluate the efficacy of endoscopic pneumatic dilatation (EPD) as treatment for Koreans with achalasia and to determine which findings from ETS and esophagography predict successful treatment of achalasia. Patients with achalasia who were treated by EPD between April 2002 and January 2012 were recruited. We defined the success of EPD as 6 months or more of clinical remission without symptoms or a decrease in the Eckardt scores by at least two points and a total Eckardt score not exceeding 3. We reviewed the percentage of maximum scintigraphic activity retained in the esophagus at 30 s (R 30) and the post-PD rate of reduction of R 30 ((Pre R 30 - Post R 30)/Pre R 30 × 100) by ETS. Possible predictive factors determined by ETS and esophagography were analyzed. Our study included 53 eligible patients. The median symptom score (Eckardt score) was 5 (4-8). R 30 and T 1/2 were, respectively, 61.8 % and 38.5 min before EPD and 20 % and 4.19 min after EPD. Successful EPD was achieved for 40 of 53 (75.47 %) patients. Age (≥40, p = 0.027) and post-PD rate of reduction of R 30 (>20 %, p = 0.003) were best prognostic indicators of clinical success. There were no perforations related to EPD. Older age and a post-PD rate of reduction of R 30 were strongly associated with better outcomes. Examination with ETS before and after EPD can be used to objectively assess a patient's short-term response to EPD.

Repeated Surgical or Endoscopic Myotomy for Recurrent Dysphagia in Patients After Previous Myotomy for Achalasia.

PubMed

Fumagalli, Uberto; Rosati, Riccardo; De Pascale, Stefano; Porta, Matteo; Carlani, Elisa; Pestalozza, Alessandra; Repici, Alessandro

2016-03-01

Surgical myotomy of the lower esophageal sphincter has a 5-year success rate of approximately 91 %. Peroral endoscopic myotomy can provide similar results for controlling dysphagia. Some patients experience either persistent or recurrent dysphagia after myotomy. We present here a retrospective analysis of our experience with redo myotomy for recurrent dysphagia in patients with achalasia. From March 1996 to February 2015, 234 myotomies for primary or recurrent achalasia were performed in our center. Fifteen patients (6.4 %) had had a previous myotomy and were undergoing surgical redo myotomy (n = 9) or endoscopic redo myotomy (n = 6) for recurrent symptoms. Patients presented at a median of 10.4 months after previous myotomy. Median preoperative Eckardt score was 6. Among the nine patients undergoing surgical myotomy, three esophageal perforations occurred intraoperatively (all repaired immediately). Surgery lasted 111 and 62 min on average (median) in the surgical and peroral endoscopic myotomy (POEM) groups, respectively. No postoperative complications occurred in either group. Median postoperative stay was 3 and 2.5 days in the surgical and POEM groups, respectively. In the surgical group, Eckardt score was <3 for seven out of nine patients after a mean follow-up of 19 months; it was <3 for all six patients in the POEM group after a mean follow-up of 5 months. A redo myotomy should be considered in patients who underwent myotomy for achalasia and presenting with recurrent dysphagia. Preliminary results using POEM indicate that the technique can be safely used in patients who have undergone previous surgical myotomy.

Results after laparoscopic Heller-Dor operation for esophageal achalasia in 100 consecutive patients.

PubMed

Tsuboi, Kazuto; Omura, Nobuo; Yano, Fumiaki; Kashiwagi, Hideyuki; Yanaga, Katsuhiko

2009-01-01

The laparoscopic Heller-Dor operation has been the procedure of choice for the treatment of achalasia. However, because the incidence of achalasia is low, reports on the outcome of surgical treatment for achalasia are limited. In this study, the therapeutic results after laparoscopic Heller-Dor operation for achalasia at a single university hospital were evaluated. Between August 1994 and July 2006, 100 consecutive patients underwent laparoscopic Heller-Dor operation. The therapeutic results after laparoscopic Heller-Dor operation were assessed based on complications, operation time, blood loss, postoperative hospital stay, and the standardized questionnaire for satisfaction by telephone or outpatient clinic interview. With respect to perioperative complications, lower esophageal mucosal perforation occurred in 14 patients, but all of them could be suture-obliterated laparoscopically. One patient was converted to laparotomy because of uncontrolled bleeding from the short gastric artery. The mean operative time was 169 minutes, and the mean perioperative blood loss was 22 mL. The median postoperative hospital stay was 7 days. Reflux esophagitis, which was seen in five patients, was treated successfully with a proton pump inhibitor. According to the standardized questionnaire for satisfaction, 77 patients rated their recovery as 'excellent', 17 as 'good', 4 as 'fair', and 2 as 'poor'; thus, the overall success rate was 94%. There were no significant differences in surgical outcomes by morphologic type and severity of esophageal dilatation; however, the success rate deteriorated significantly with progression of the morphologic type. Laparoscopic Heller-Dor operation is a safe and effective surgical treatment for achalasia.

Management of achalasia in the UK, do we need new guidelines?

PubMed

El Kafsi, Jihene; Foliaki, Antonio; Dehn, Thomas C B; Maynard, Nicholas D

2016-12-01

It is recommended that management of complex benign upper gastrointestinal pathology is discussed at multi disciplinary team (MDT) meetings. American College of Gastroenterology (ACG) guidelines further recommend that treatment delivery is provided by high volume centres, with objective post-procedural investigations, in order to improve patient outcomes. We aimed to survey the current UK practice in the management of achalasia. 443 Upper gastrointestinal (UGI) specialist surgeons throughout the UK were sent a surveymonkey.com questionnaire about the management of achalasia. 100 responses were received. The majority of patients with achalasia are referred directly to surgeons (80%) and only 15% of units have a MDT meeting for discussing such patients. Diagnosis was mainly with oesophagogastroduodenoscopy (OGD) and contrast swallow, and only 61% of units have access to high resolution manometry (HRM). 89% of younger patients were offered surgery initially, whilst in the elderly surgery was offered as first line treatment in 55%. Partial fundoplication was carried out by 91% of responders as part of the operation, and 58% responders carry out an intraoperative OGD. The average number of operations carried out per annum is 4 per responder. Most responders (66%) did not perform routine post-intervention investigations and follow-up varied from none to lifelong. Diagnosis and management of achalasia within the UK is relatively standardised, although there remains limited access to HRM. Discussion at benign MDTs however is poor and follow-up differs widely. UK guidelines may help to make these more uniform.

Oesophageal food impaction in achalasia treated with Coca-Cola and nifedipine.

PubMed

Koumi, Andriani; Panos, Marios Zenon

2010-01-01

Achalasia is characterised by the loss of peristaltic movement in the distal oesophagus and failure of the lower oesophageal sphincter relaxation, which results in impaired oesophageal emptying. We report a case of a 92-year-old frail woman with a history of achalasia, who presented with acute oesophageal obstruction due to impaction of a large amount of food material. She was treated successfully with nifedipine, in combination with Coca-Cola (original product, not sugar free), so avoiding the risks associated with repeated endoscopic intubation and piecemeal removal of the oesophageal content.

Oesophageal food impaction in achalasia treated with Coca-Cola and nifedipine

PubMed Central

Koumi, Andriani; Panos, Marios Zenon

2010-01-01

Achalasia is characterised by the loss of peristaltic movement in the distal oesophagus and failure of the lower oesophageal sphincter relaxation, which results in impaired oesophageal emptying. We report a case of a 92-year-old frail woman with a history of achalasia, who presented with acute oesophageal obstruction due to impaction of a large amount of food material. She was treated successfully with nifedipine, in combination with Coca-Cola (original product, not sugar free), so avoiding the risks associated with repeated endoscopic intubation and piecemeal removal of the oesophageal content. PMID:22242073

A case report of achalasia

NASA Astrophysics Data System (ADS)

Sarumpaet, F.; Dairi, L.

2018-03-01

Achalasia is characterized by esophageal nonperistaltic contraction and incomplete relaxation of the lower esophageal sphincter. The most common symptom are dysphagia, regurgitation, and heartburn. A 48 years old male was admitted into HAM General Hospital Medan with achief complaint of dysphagia since adulthood. The patient had to drink a lot of water to help swallow solid or soft food. Complaint worsened in the last three months followed by odynophagia, nausea, and vomiting undigested, retained food. The patient also complained about heartburn and was previously diagnosed with a variant of angina pectoris but his symptoms didn’t improve with medication. The patient had a history of weight loss but no anorexia, no prior history of corrosive ingestion. The patient was an active smoker. Physical examination revealed no abnormality. Oesophagogram showed dilated distal esophagus with rat tail appearance. Gastroscopy revealed dilatation on the lower third of the esophagus. Computed Tomography revealed dilatation of distal esophagus. Based on the results of the exams, we concluded the diagnosis as achalasia. The patient was treated with calcium channel blocker and proton pump inhibitor. The patient showed clinical improvement after treated and was discharged. The patient was planned for once a month follow up in the outpatient clinic.

Laparoscopic treatment for esophageal achalasia: experience at a single center.

PubMed

Agrusa, A; Romano, G; Bonventre, S; Salamone, G; Cocorullo, G; Gulotta, G

2013-01-01

Achalasia is a not frequent esophageal disorder characterized by the absence of esophageal peristalsis and incomplete relaxation of the lower esophageal sphincter (LES). Its cause is unknown. The aim of treatment is to improve the symptoms. We report the results of the treatment of this condition achieved in one center. We conducted a retrospective study of patients with esophageal achalasia. In the period 2010-2012 we observed 64 patients, of whom 19 were referred for medical treatment. Three of the remaining patients underwent botulinum toxin injection, 17 underwent multiple endoscopic dilation procedures and 25 underwent laparoscopic surgery. There were no complications in the group undergoing endoscopic therapy, but symptom remission was only temporary. Patients undergoing surgery showed a significant improvement in symptoms and no recurrence throughout the follow-up period, that is still ongoing (3 years). There were no major complications in any case and no morbidity or mortality. Surgical treatment of esophageal achalasia with laparoscopic Heller myotomy and Dor fundoplication gives the best and longest-lasting results in suitably selected patients. The extension of the myotomy and reduction in LES pressure are the most important parameters to achieve a good result.

Efficacy of pneumodilation in achalasia after failed Heller myotomy.

PubMed

Saleh, C M G; Ponds, F A M; Schijven, M P; Smout, A J P M; Bredenoord, A J

2016-11-01

Heller myotomy is an effective treatment for the majority of achalasia patients. However, a small proportion of patients suffer from persistent or recurrent symptoms after surgery and they are usually subsequently treated with pneumodilation (PD). Data on the efficacy of PD as secondary treatment for achalasia are scarce. Therefore, this study aimed to investigate the efficacy of PD as treatment for achalasia patients suffering from persistent or recurrent symptoms after Heller myotomy. Patients with recurrent or persistent symptoms (Eckardt score >3) after Heller myotomy were selected. Patients were treated with PD, using a graded distension protocol with balloon sizes ranging from 30 to 40 mm. After each dilation symptoms were assessed to evaluate whether a subsequent dilation with a larger balloon size was required. Patients with recurrent or persistent symptoms (Eckardt score >3) after treatment with a 40-mm balloon were identified as failures. Twenty-four patients were included in total; 15 patients with achalasia type I, seven with achalasia type II and two with achalasia type III. Median relapse time was 2.5 years after Heller myotomy (IQR: 9 years and 3 months). Three patients were not suitable for PD; one patient was morbidly obese and not fit for any form of sedation and two had a siphon-shaped esophagus leaving 21 patients to treat. Eight patients were successfully treated with a single 30-mm balloon dilation (median follow-up time: 6.5 years; IQR: 7.5 years). Four patients required dilations with 30- and 35-mm balloons (median follow-up time: 11 years; IQR: 3 years). Nine patients failed on the 35-mm balloon dilation and underwent a subsequent dilation with a 40-mm balloon, and all failed on this balloon as well. Thus, PD was successful in 12 of the 21 treatable patients, resulting in a success rate of 57% for treatable patients or 50% for all patients. Baseline Eckardt scores were also higher in those that failed (median: 8; IQR: 2) than those that

The preoperative manometric pattern predicts the outcome of surgical treatment for esophageal achalasia.

PubMed

Salvador, Renato; Costantini, Mario; Zaninotto, Giovanni; Morbin, Tiziana; Rizzetto, Christian; Zanatta, Lisa; Ceolin, Martina; Finotti, Elena; Nicoletti, Loredana; Da Dalt, Gianfranco; Cavallin, Francesco; Ancona, Ermanno

2010-11-01

A new manometric classification of esophageal achalasia has recently been proposed that also suggests a correlation with the final outcome of treatment. The aim of this study was to investigate this hypothesis in a large group of achalasia patients undergoing laparoscopic Heller-Dor myotomy. We evaluated 246 consecutive achalasia patients who underwent surgery as their first treatment from 2001 to 2009. Patients with sigmoid-shaped esophagus were excluded. Symptoms were scored and barium swallow X-ray, endoscopy, and esophageal manometry were performed before and again at 6 months after surgery. Patients were divided into three groups: (I) no distal esophageal pressurization (contraction wave amplitude <30 mmHg); (II) rapidly propagating compartmentalized pressurization (panesophageal pressurization >30 mmHg); and (III) rapidly propagating pressurization attributable to spastic contractions. Treatment failure was defined as a postoperative symptom score greater than the 10th percentile of the preoperative score (i.e., >7). Type III achalasia coincided with a longer overall lower esophageal sphincter (LES) length, a lower symptom score, and a smaller esophageal diameter. Treatment failure rates differed significantly in the three groups: I = 14.6% (14/96), II = 4.7% (6/127), and III = 30.4% (7/23; p = 0.0007). At univariate analysis, the manometric pattern, a low LES resting pressure, and a high chest pain score were the only factors predicting treatment failure. At multivariate analysis, the manometric pattern and a LES resting pressure <30 mmHg predicted a negative outcome. This is the first study by a surgical group to assess the outcome of surgery in 3 manometric achalasia subtypes: patients with panesophageal pressurization have the best outcome after laparoscopic Heller-Dor myotomy.

Thoracoscopic Surgery in a Patient with Multiple Esophageal Carcinomas after Surgery for Esophageal Achalasia.

PubMed

Yamasaki, Yuki; Tsukada, Tomoya; Aoki, Tatsuya; Haba, Yusuke; Hirano, Katsuhisa; Watanabe, Toshifumi; Kaji, Masahide; Shimizu, Koichi

2017-01-01

We present a case in which we used a thoracoscopic approach for resection of multiple esophageal carcinomas diagnosed 33 years after surgery for esophageal achalasia. A 68-year-old Japanese man had been diagnosed with esophageal achalasia and underwent surgical treatment 33 years earlier. He was examined at our hospital for annual routine checkup in which upper gastrointestinal endoscopy showed a "0-IIb+IIa" lesion in the middle esophagus. Iodine staining revealed multiple irregularly shaped iodine-unstained areas, the diagnosis of which was esophageal carcinoma. Thoracoscopic subtotal esophagectomy was performed. Esophageal carcinoma may occur many years after surgery for esophageal achalasia, even if the passage symptoms have improved. So, long-term periodic follow-up is necessary for detection of carcinoma at an earlier stage.

Thoracoscopic Surgery in a Patient with Multiple Esophageal Carcinomas after Surgery for Esophageal Achalasia

PubMed Central

Tsukada, Tomoya; Aoki, Tatsuya; Haba, Yusuke; Hirano, Katsuhisa; Watanabe, Toshifumi; Kaji, Masahide; Shimizu, Koichi

2017-01-01

We present a case in which we used a thoracoscopic approach for resection of multiple esophageal carcinomas diagnosed 33 years after surgery for esophageal achalasia. A 68-year-old Japanese man had been diagnosed with esophageal achalasia and underwent surgical treatment 33 years earlier. He was examined at our hospital for annual routine checkup in which upper gastrointestinal endoscopy showed a “0-IIb+IIa” lesion in the middle esophagus. Iodine staining revealed multiple irregularly shaped iodine-unstained areas, the diagnosis of which was esophageal carcinoma. Thoracoscopic subtotal esophagectomy was performed. Esophageal carcinoma may occur many years after surgery for esophageal achalasia, even if the passage symptoms have improved. So, long-term periodic follow-up is necessary for detection of carcinoma at an earlier stage. PMID:28951795

Peroral endoscopic myotomy: Time to change our opinion regarding the treatment of achalasia?

PubMed Central

Tantau, Marcel; Crisan, Dana

2015-01-01

Peroral endoscopic myotomy (POEM) is a new endoscopic treatment for achalasia. Compared to the classical surgical myotomy, POEM brings at least the advantage of minimal invasiveness. The data provided until now suggest that POEM offers excellent short-term symptom resolution, with improvement of dysphagia in more than 90% of treated patients, with encouraging manometric outcomes and low incidence of postprocedural gastroesophageal reflux. The effectiveness of this novel therapy requires long-term follow-up and comparative studies with other treatment modalities for achalasia. This technique requires experts in interventional endoscopy, with a learning curve requiring more than 20 cases, including training on animal and cadaver models, and with a need for structured proctoring during the first cases. This review aims to summarize the data on the technique, outcomes, safety and learning curve of this new endoscopic treatment of achalasia. PMID:25789094

Outcomes promote reoperative Heller myotomy for symptoms of achalasia.

PubMed

Rakita, S; Villadolid, D; Kalipersad, C; Thometz, D; Rosemurgy, A

2007-10-01

Heller myotomy is accepted as first-line therapy for achalasia, yet for a small number of patients, symptoms persist or recur after myotomy. This study was undertaken to report our results with reoperative laparoscopic Heller myotomy for recurrent symptoms of achalasia. We have undertaken laparoscopic Heller myotomy in 275 patients and reoperative myotomy in 12 patients for recurrent dysphagia, of which three had their initial myotomy undertaken by us. For each, studies prior to reoperative Heller myotomy documented a nonrelaxing lower esophageal sphincter without stricture. Patients scored symptoms before and after reoperative myotomy. Before reoperative myotomy, 75% underwent dilation and 42% underwent Botox injection. Ten of twelve reoperative myotomies were undertaken and completed laparoscopically. Median follow-up is 24.1 months (29.0 months + 25.89). Symptom frequency and severity scores improved significantly after reoperative myotomy. Frequency of vomiting and frequency and severity of heartburn were improved after reoperative myotomy, but not to a significant extent. However, they were not particularly notable prior to surgery, compared to obstructive symptoms, such as dysphagia. Excellent or good outcomes were reported in 73%, and notably, 91% stated that they would have the operation again after having been through the process firsthand and knowing their outcomes. Patient outcomes promote the application of reoperative Heller myotomy for recurrent or persistent symptoms of achalasia following Heller myotomy.

Cellular and molecular basis of chronic constipation: Taking the functional/idiopathic label out

PubMed Central

Bassotti, Gabrio; Villanacci, Vincenzo; Creƫoiu, Dragos; Creƫoiu, Sanda Maria; Becheanu, Gabriel

2013-01-01

In recent years, the improvement of technology and the increase in knowledge have shifted several strongly held paradigms. This is particularly true in gastroenterology, and specifically in the field of the so-called “functional” or “idiopathic” disease, where conditions thought for decades to be based mainly on alterations of visceral perception or aberrant psychosomatic mechanisms have, in fact, be reconducted to an organic basis (or, at the very least, have shown one or more demonstrable abnormalities). This is particularly true, for instance, for irritable bowel syndrome, the prototype entity of “functional” gastrointestinal disorders, where low-grade inflammation of both mucosa and myenteric plexus has been repeatedly demonstrated. Thus, researchers have also investigated other functional/idiopathic gastrointestinal disorders, and found that some organic ground is present, such as abnormal neurotransmission and myenteric plexitis in esophageal achalasia and mucosal immune activation and mild eosinophilia in functional dyspepsia. Here we show evidence, based on our own and other authors’ work, that chronic constipation has several abnormalities reconductable to alterations in the enteric nervous system, abnormalities mainly characterized by a constant decrease of enteric glial cells and interstitial cells of Cajal (and, sometimes, of enteric neurons). Thus, we feel that (at least some forms of) chronic constipation should no more be considered as a functional/idiopathic gastrointestinal disorder, but instead as a true enteric neuropathic abnormality. PMID:23864772

Incidence, clinical features and para-clinical findings of achalasia in Algeria: Experience of 25 years.

PubMed

Tebaibia, Amar; Boudjella, Mohammed Amine; Boutarene, Djamel; Benmediouni, Farouk; Brahimi, Hakim; Oumnia, Nadia

2016-10-14

To investigate the incidence of achalasia in Algeria and to determine its clinical and para-clinical profile. To evaluate the impact of continuing medical education (CME) on the incidence of this disease. From 1990 to 2014, 1256 patients with achalasia were enrolled in this prospective study. A campaign of CME on diagnosis involving different regions of the country was conducted between 1999 and 2003. Annual incidence and prevalence were calculated by relating the number of diagnosed cases to 10 5 inhabitants. Each patient completed a standardized questionnaire, and underwent upper endoscopy, barium swallow and esophageal manometry. We systematically looked for Allgrove syndrome and familial achalasia. The mean annual incidence raised from 0.04 (95%CI: 0.028-0.052) during the 1990s to 0.27/10 5 inhabitants/year (95%CI: 0.215-0.321) during the 2000s. The incidence of the disease was two and half times higher in the north and the center compared to the south of the country. One-hundred-and-twenty-nine (10%) were children and 97 (7.7%) had Allgrove syndrome. Familial achalasia was noted in 18 different families. Patients had dysphagia (99%), regurgitation (83%), chest pain (51%), heartburn 24.5% and weight loss (70%). The lower esophageal sphincter was hypertensive in 53% and hypotensive in 0.6%. The mean incidence of achalasia in Algeria is at least 0.27/10 5 inhabitants. A good impact on the incidence of CME was noted. A gradient of incidence between different regions of the country was found. This variability is probably related to genetic and environmental factors. The discovery of an infantile achalasia must lead to looking for Allgrove syndrome and similar cases in the family.

Incidence, clinical features and para-clinical findings of achalasia in Algeria: Experience of 25 years

PubMed Central

Tebaibia, Amar; Boudjella, Mohammed Amine; Boutarene, Djamel; Benmediouni, Farouk; Brahimi, Hakim; Oumnia, Nadia

2016-01-01

AIM To investigate the incidence of achalasia in Algeria and to determine its clinical and para-clinical profile. To evaluate the impact of continuing medical education (CME) on the incidence of this disease. METHODS From 1990 to 2014, 1256 patients with achalasia were enrolled in this prospective study. A campaign of CME on diagnosis involving different regions of the country was conducted between 1999 and 2003. Annual incidence and prevalence were calculated by relating the number of diagnosed cases to 105 inhabitants. Each patient completed a standardized questionnaire, and underwent upper endoscopy, barium swallow and esophageal manometry. We systematically looked for Allgrove syndrome and familial achalasia. RESULTS The mean annual incidence raised from 0.04 (95%CI: 0.028-0.052) during the 1990s to 0.27/105 inhabitants/year (95%CI: 0.215-0.321) during the 2000s. The incidence of the disease was two and half times higher in the north and the center compared to the south of the country. One-hundred-and-twenty-nine (10%) were children and 97 (7.7%) had Allgrove syndrome. Familial achalasia was noted in 18 different families. Patients had dysphagia (99%), regurgitation (83%), chest pain (51%), heartburn 24.5% and weight loss (70%). The lower esophageal sphincter was hypertensive in 53% and hypotensive in 0.6%. CONCLUSION The mean incidence of achalasia in Algeria is at least 0.27/105 inhabitants. A good impact on the incidence of CME was noted. A gradient of incidence between different regions of the country was found. This variability is probably related to genetic and environmental factors. The discovery of an infantile achalasia must lead to looking for Allgrove syndrome and similar cases in the family. PMID:27784974

Pattern of esophageal eosinophilic infiltration in patients with achalasia and response to Heller myotomy and Dor fundoplication.

PubMed

Cools-Lartigue, J; Chang, S-Y; Mckendy, K; Mayrand, S; Marcus, V; Fried, G M; Ferri, L E

2013-01-01

Eosinophilic esophagitis (EoE) is now recognized as a common cause of dysphagia. Eosinophilic infiltration of the esophagus has also been associated with other conditions, such as gastroesophageal reflux disease (GERD); however, the incidence, pattern, and clinical significance of eosinophilic infiltration in achalasia are poorly documented. We sought to characterize this histological finding in patients undergoing Heller myotomy (HM) for achalasia. Ninety-six patients undergoing laparoscopic HM for primary achalasia between 1999 and 2008 were identified from a prospective database. Serial mid and distal per-endoscopic esophageal biopsies taken from patients before and after surgery were assessed for the presence of elevated intraepithelial eosinophils (EIEs). Slides from patients with reports suggestive of EIE were reviewed independently by two pathologists, and the highest eosinophil count/high-power field (eos/hpf) was recorded. Dysphagia scores (0 = none to 5 = severe dysphagia), GERD health-related quality of life scores (0 = best to 45 = worst), and 24-hour pH results were compared before and 3 months after surgery. We related the highest eos to the symptoms and response to HM. Data are presented as median (range). Paired t-test and Wilcoxon signed-rank test determined significance, *P < 0.05. Of 96 patients with achalasia, 50 had undergone pre-HM biopsies revealing EIE in 17/50 (34%), with a median of 3 eos/hpf (1-21). Two patients were found to have superimposed esophageal candidiasis. One patient met the pathologic criteria for EoE. Twenty-five of 50 (50%) postoperative biopsies demonstrated a median of 5 eos/hpf (1-62) for a total of 28/50 patients (56%) with EIE in either the preoperative or postoperative period. Four patients (8%) met the pathologic criteria for EoE, and two demonstrated persistent esophageal candidiasis. A decrease in eosinophils was found in 6/28 patients (21%) from 3/hpf (1-21) to 0.5/hpf (0-4). Increase in eosinophils was found in

Partial recovery of peristalsis after myotomy for achalasia; more the rule than the exception

PubMed Central

Roman, Sabine; Kahrilas, Peter J; Mion, François; Nealis, Thomas B; Soper, Nathaniel J; Poncet, Gilles; Nicodème, Frédéric; Hungness, Eric; Pandolfino, John E

2013-01-01

Hypothesis: Myotomy that alleviates the esophagogastric junction (EGJ) outflow obstruction in achalasia might improve peristalsis. Design: Retrospective study Setting: Two tertiary hospitals (Chicago, USA and Lyon, France) Patients: Thirty patients (18 males; mean age 43 years, range 17-78) were included from 2004 to 2012: 8 type 1 achalasia (27%), 17 type 2 (57%) and 5 type 3 (16%) according to the Chicago Classification. Interventions: Esophageal high resolution manometry (HRM) before and after laparoscopic or endoscopic myotomy. Main outcomes measures: The integrity of peristalsis was characterized as intact, weak, frequent failed, absent, or premature contractions. Results: Whereas peristaltic fragments were evident only in patients with type 3 achalasia before treatment, intact, weak, or frequent failed peristalsis was encountered in 63% of type 1, 47% of type 2 and 80% of type 3 achalasia after myotomy. One type 3 patient had distal esophageal spasm after treatment. In patients with a post-myotomy integrated relaxation pressure (IRP) <15 mmHg, only 10 (40%) persisted in having absent peristalsis. Panesophageal pressurization disappeared after myotomy in 16 of 19 patients. In the 5 patients with post-myotomy IRP >15 mmHg, 4 had weak peristalsis and one had absent peristalsis. Conclusion: Reduction or normalization of the EGJ relaxation pressure achieved by myotomy in achalasia patients was associated with partial recovery of peristalsis in some patients suggesting that the disease process progresses from the EGJ to the esophageal body. Whether or not the return of peristalsis is predictive of an improved therapeutic outcome requires further study. PMID:23426591

Endoscope-guided pneumatic dilation for treatment of esophageal achalasia

PubMed Central

Chuah, Seng-Kee; Wu, Keng-Liang; Hu, Tsung-Hui; Tai, Wei-Chen; Changchien, Chi-Sin

2010-01-01

Pneumatic dilation (PD) is considered to be the first line nonsurgical therapy for achalasia. The principle of the procedure is to weaken the lower esophageal sphincter by tearing its muscle fibers by generating radial force. The endoscope-guided procedure is done without fluoroscopic control. Clinicians usually use a low-compliance balloon such as Rigiflex dilator to perform endoscope-guided PD for the treatment of esophageal achalasia. It has the advantage of determining mucosal injury during the dilation process, so that a repeat endoscopy is not needed to assess the mucosal tearing. Previous studies have shown that endoscope-guided PD is an efficient and safe nonsurgical therapy with results that compare well with other treatment modalities. Although the results may be promising, long-term follow-up is required in the near future. PMID:20101764

Laparoscopic treatment for esophageal achalasia: experience at a single center

PubMed Central

AGRUSA, A.; ROMANO, G.; BONVENTRE, S.; SALAMONE, G.; COCORULLO, G.; GULOTTA, G.

2013-01-01

Summary Background Achalasia is a not frequent esophageal disorder characterized by the absence of esophageal peristalsis and incomplete relaxation of the lower esophageal sphincter (LES). Its cause is unknown. The aim of treatment is to improve the symptoms. We report the results of the treatment of this condition achieved in one center. Patients and methods We conducted a retrospective study of patients with esophageal achalasia. In the period 2010–2012 we observed 64 patients, of whom 19 were referred for medical treatment. Three of the remaining patients underwent botulinum toxin injection, 17 underwent multiple endoscopic dilation procedures and 25 underwent laparoscopic surgery. Results There were no complications in the group undergoing endoscopic therapy, but symptom remission was only temporary. Patients undergoing surgery showed a significant improvement in symptoms and no recurrence throughout the follow-up period, that is still ongoing (3 years). There were no major complications in any case and no morbidity or mortality. Conclusions Surgical treatment of esophageal achalasia with laparoscopic Heller myotomy and Dor fundoplication gives the best and longest-lasting results in suitably selected patients. The extension of the myotomy and reduction in LES pressure are the most important parameters to achieve a good result. PMID:24091178

The natural history of achalasia: Evidence of a continuum-"The evolutive pattern theory".

PubMed

Salvador, Renato; Voltarel, Guerrino; Savarino, Edoardo; Capovilla, Giovanni; Pesenti, Elisa; Perazzolo, Anna; Nicoletti, Loredana; Costantini, Andrea; Merigliano, Stefano; Costantini, Mario

2018-04-01

It is currently unclear if the three manometric patterns of esophageal achalasia represent distinct entities or part of a disease continuum. The study's aims were: a) to test the hypothesis that the three patterns represent different stages in the evolution of achalasia; b) to investigate whether manometric patterns change after Laparoscopic-Heller-Dor (LHD). We assessed the patients diagnosed with achalasia who underwent LHD as their first treatment from 1992 to 2016. Their symptoms were scored using a detailed questionnaire for dysphagia, food-regurgitation, and chest pain. Barium-swallow, endoscopy, and esophageal-manometry were performed before and 6 months after surgery. The study population consisted of 511 patients (M:F=283:228). Patients' demographic and clinical data showed that those with pattern III had a shorter history of symptoms, a higher incidence of chest pain, and a less dilated gullet (p<0.001). All patients with a sigmoid-shaped mega-esophagus had pattern I achalasia. One patient with a diagnosis of pattern III achalasia developed pattern II at a follow-up manometry before surgery. At a median follow-up of 30 months (IQR 12-56), the outcome of surgery was positive in 479 patients (91.7%). All patients with pattern I preoperatively had the same pattern after LHD, whereas more than 50% of patients with pattern III before treatment showed pattern I or II after surgery. This study supports the hypothesis/theory that the different manometric patterns represent different stages in the evolution of the disease-where pattern III is the earliest stage, pattern II an intermediate stage, and pattern I the final stage. Copyright © 2017 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

Giant fibrovascular esophageal polyp misdiagnosed as achalasia.

PubMed

Cordoş, I; Istrate, A; Codreşi, M; Bolca, C

2012-01-01

A 59 years old woman was admitted in our unit accusing longtime dysphagia and regurgitation. On admission, the patient was wearing a 3 month old definitive feeding gastrostomy tube. The contrast swallow, endoscopy and esophageal manometry established the diagnostic--achalasia. We removed the gastrostomy tube and we performed an open Heller myotomy. The postoperative period was uneventful and the patient was discharged one week later with affirmatively unimpaired deglutition. One month later, the patient was admitted via emergency with a giant fibrous tumor arising from her mouth after an episode of strong coughing and vomiting. The repeated endoscopy showed a giant esophageal polyp that was missed by the previous investigations, originating from pharingoesophageal junction. The esophageal polyp was resected by cervical approach with good postoperative outcome. The polyp's particular extreme dimensions (27 cm) prevented the acute asphyxia by blockage at the laryngeal level, possibly provoked by smaller tumors. As postoperative one month barium swallow showed a normal esophageal aspect, a final question remains--was achalasia real or an erroneous diagnosis was established the second time too?

The Prevalence and Characteristics of Primary Headache and Dream-Enacting Behaviour in Japanese Patients with Narcolepsy or Idiopathic Hypersomnia: A Multi-Centre Cross-Sectional Study.

PubMed

Suzuki, Keisuke; Miyamoto, Masayuki; Miyamoto, Tomoyuki; Inoue, Yuichi; Matsui, Kentaro; Nishida, Shingo; Hayashida, Kenichi; Usui, Akira; Ueki, Yoichiro; Nakamura, Masaki; Murata, Momoyo; Numao, Ayaka; Watanabe, Yuji; Suzuki, Shiho; Hirata, Koichi

2015-01-01

Because the prevalence and characteristics of primary headache have yet to be thoroughly studied in patients with hypersomnia disorders, including narcolepsy and idiopathic hypersomnia, we examined these parameters in the Japanese population. In a multicentre cross-sectional survey, among 576 consecutive outpatients with sleep disorders, 68 narcolepsy patients and 35 idiopathic hypersomnia patients were included. Additionally, 61 healthy control subjects participated. Semi-structured headache questionnaires were administered to all participants. The patients with narcolepsy (52.9%) and idiopathic hypersomnia (77.1%) more frequently experienced headache than the healthy controls (24.6%; p<0.0001). The prevalence rates were 23.5%, 41.2% and 4.9% for migraine (p<0.0001) and 16.2%, 23.5% and 14.8% (p = 0.58) for tension-type headache among the narcolepsy patients, the idiopathic hypersomnia patients and the control subjects, respectively. Those who experienced migraine more frequently experienced excessive daytime sleepiness, defined as an Epworth Sleepiness Scale score of ≥10, than those who did not experience headache among the patients with narcolepsy (93.8% vs. 65.6%, p = 0.040) and idiopathic hypersomnia (86.7% vs. 37.5%, p = 0.026). Dream-enacting behaviour (DEB), as evaluated by the rapid eye movement sleep disorders questionnaire, was more frequently observed in the narcolepsy patients than in the idiopathic hypersomnia patients and the control subjects. An increased DEB frequency was observed in the narcolepsy patients with migraines compared to those without headache. Migraines were frequently observed in patients with narcolepsy and idiopathic hypersomnia. DEB is a characteristic of narcolepsy patients. Further studies are required to assess the factors that contribute to migraines in narcolepsy and idiopathic hypersomnia patients.

High-Resolution Manometry Evaluation of the Pharynx and Upper Esophageal Sphincter Motility in Patients with Achalasia.

PubMed

Menezes, Mariano A; Herbella, Fernando A M; Patti, Marco G

2015-10-01

The motility of the pharynx and upper esophageal sphincter (UES) is still poorly understood. It is also unclear if the motility of this area may be compromised in patients with achalasia. This study aims to evaluate the motility of the pharynx, UES, and proximal esophagus in patients with esophageal achalasia. Sixty patients with achalasia underwent high-resolution manometry (HRM) (52 % females, mean age 54 years). Esophageal dilatation was classified according to the radiologic diameter in Type I (<4 cm): 6 %; Type II (4-7 cm): 36 %; Type III (7-10 cm): 34 %; and Type IV (>10 cm): 24 %. HRM classified 43 % of the patients as Chicago Type I and 57 % as Type II. Manometric parameters were compared to normal values obtained from a previous study in volunteers. The motility of the velopharynx showed short, premature, and hypertonic contraction. The epiglottis also showed hypertonic contraction. The UES had increased residual pressure. Chicago classification Type II patients had higher UES residual pressure (p = 0.03). The degree of esophageal dilatation did not correlate with manometric parameters. Achalasia may affect the motility of the pharyngo-upper esophageal area. The changes observed may represent functional alterations to prevent aspiration, especially in patients with Chicago classification Type II achalasia.

Mechanical properties of the gastro-esophageal junction in health, achalasia, and scleroderma.

PubMed

Mearin, F; Fonollosa, V; Vilardell, M; Malagelada, J R

2000-07-01

Manometric assessment of the gastro-esophageal junction (GEJ) is deceptive in that ignores key dynamic properties of the junction, such as resistance to flow and compliance. Our aim was to investigate the mechanical properties of the GEJ comprising intraluminal pressure (measured by manometry), resistance to flow and compliance (measured by resistometry). We studied 8 healthy subjects, 11 patients with achalasia and 11 patients with scleroderma. We used a pneumatic resistometer, previously developed and validated in our laboratory. The resistometer consists of a flaccid polyurethane 5-cm cylinder connected to an electronically regulated nitrogen-injection system; the instrument records nitrogen flow through the cylinder while maintaining a constant pressure gradient between its proximal and distal ends. By placing the cylinder successively in the proximal stomach and along the GEJ we measured the GEJ-gastric resistance gradient (GEJ resistance minus gastric resistance) and were able to calculate the cumulative resistance (sum of resistance exerted at each pressure level), peak resistance (at any injection pressure), nil resistance point (injection pressure in mmHg at which GEJ resistance equals gastric resistance), and compliance slope (flow/pressure relationship). We found that GEJ resistance to flow (cumulative resistance, peak resistance, and nil resistance point) is significantly increased in achalasia and decreased in scleroderma (P < 0.05 versus health) while GEJ compliance is diminished in achalasia (P < 0.05 versus health) and normal in scleroderma. Achalasia is a disease characterized by increased GEJ resistance and rigidity. By contrast, although scleroderma is characterized by decreased GEJ resistance, GEJ compliance may be normal.

Perspective: Update on Idiopathic Intracranial Hypertension

PubMed Central

Bruce, Beau B.; Biousse, Valérie; Newman, Nancy J.

2011-01-01

Purpose Provide an update on various features of idiopathic intracranial hypertension. Design Perspective. Methods Selected articles on the epidemiology, clinical and imaging features, natural history, pathophysiology, and treatment of idiopathic intracranial hypertension were reviewed and interpreted in the context of the authors’ clinical and research experience. Results Idiopathic intracranial hypertension is primarily a disease of obese women of childbearing age, but it can affect patients of any weight, sex, and age. Although a relatively rare disorder, idiopathic intracranial hypertension’s associated costs in the U.S. entail hundreds of millions of dollars. Even following treatment, headaches are frequently persistent and may require the continued involvement of a neurologist. Quality of life reductions and depression are common among idiopathic intracranial hypertension patients. However, visual dysfunction, especially visual field abnormalities, represents the major morbidity of this disorder, and serial automated perimetry remains the primary mode of patient monitoring. Patients who are men, black, very obese, or anemic are at higher risk of visual loss. Vitamin A metabolism, adipose tissue as an actively secreting endocrine tissue, and cerebral venous abnormalities are areas of active study regarding idiopathic intracranial hypertension’s pathophysiology. Treatment studies show that lumbar puncture is a valuable treatment (in addition to its crucial diagnostic role) and that weight management is critical. However, open questions remain regarding the efficacy of acetazolamide, CSF diversion procedures, and cerebral venous stenting. Conclusions Many questions remain unanswered about idiopathic intracranial hypertension. Ongoing studies, especially an ongoing NIH-funded clinical trial of acetazolamide, should provide more insight into this important, yet poorly understood syndrome of isolated intracranial hypertension. PMID:21696699

The Prevalence and Characteristics of Primary Headache and Dream-Enacting Behaviour in Japanese Patients with Narcolepsy or Idiopathic Hypersomnia: A Multi-Centre Cross-Sectional Study

PubMed Central

Suzuki, Keisuke; Miyamoto, Masayuki; Miyamoto, Tomoyuki; Inoue, Yuichi; Matsui, Kentaro; Nishida, Shingo; Hayashida, Kenichi; Usui, Akira; Ueki, Yoichiro; Nakamura, Masaki; Murata, Momoyo; Numao, Ayaka; Watanabe, Yuji; Suzuki, Shiho; Hirata, Koichi

2015-01-01

Background Because the prevalence and characteristics of primary headache have yet to be thoroughly studied in patients with hypersomnia disorders, including narcolepsy and idiopathic hypersomnia, we examined these parameters in the Japanese population. Methods In a multicentre cross-sectional survey, among 576 consecutive outpatients with sleep disorders, 68 narcolepsy patients and 35 idiopathic hypersomnia patients were included. Additionally, 61 healthy control subjects participated. Semi-structured headache questionnaires were administered to all participants. Results The patients with narcolepsy (52.9%) and idiopathic hypersomnia (77.1%) more frequently experienced headache than the healthy controls (24.6%; p<0.0001). The prevalence rates were 23.5%, 41.2% and 4.9% for migraine (p<0.0001) and 16.2%, 23.5% and 14.8% (p = 0.58) for tension-type headache among the narcolepsy patients, the idiopathic hypersomnia patients and the control subjects, respectively. Those who experienced migraine more frequently experienced excessive daytime sleepiness, defined as an Epworth Sleepiness Scale score of ≥10, than those who did not experience headache among the patients with narcolepsy (93.8% vs. 65.6%, p = 0.040) and idiopathic hypersomnia (86.7% vs. 37.5%, p = 0.026). Dream-enacting behaviour (DEB), as evaluated by the rapid eye movement sleep disorders questionnaire, was more frequently observed in the narcolepsy patients than in the idiopathic hypersomnia patients and the control subjects. An increased DEB frequency was observed in the narcolepsy patients with migraines compared to those without headache. Conclusions Migraines were frequently observed in patients with narcolepsy and idiopathic hypersomnia. DEB is a characteristic of narcolepsy patients. Further studies are required to assess the factors that contribute to migraines in narcolepsy and idiopathic hypersomnia patients. PMID:26418536

[New concepts on the physiopathology, diagnosis, and treatment of achalasia].

PubMed

Carmona-Sánchez, R; Valdovinos-Díaz, M A

1998-01-01

To review the most relevant publications on the pathophysiology, clinical manifestations, diagnosis and treatment of esophageal achalasia, and the clinical experience achieved at our institution in order to propose a practical strategy to facilitate the management of these patients. Manual and MEDLINE search of key articles published between January 1986 and July 1997 in addition to publications of our institute of thirty years. All kinds of publications with substantial clinical experience, new information or research protocols. Achalasia is an uncommon disorder of the myenteric plexus of the esophagus. Main symptoms are dysphagia, regurgitations and chest pain. The diagnosis is established by manometric criteria. Esophagogram, endoscopy and radionuclide esophageal emptying test help to differentiate other conditions and evaluate the response to treatment. Pharmacotherapy may provide relief to patients with mild symptoms and is useful for patients with high risk of complications. Dilations and myotomy are safe, effective and long lasting procedures. Botulinum toxin may be effective in selected cases. Predictive factors of response have been described for each therapy. A systematic approach to the management of patients with achalasia is necessary. Introduction of new therapies as botulinum toxin and minimal invasion surgery are changing the therapeutic decisions in this field. Drugs and BoTox are considered the first line of treatment for high risk patients and dilation and surgery for patients with no risk.

Videoscopic Heller myotomy for achalasia--results beyond short-term follow-up.

PubMed

Bloomston, M; Boyce, W; Mamel, J; Albrink, M; Murr, M; Durkin, A; Rosemurgy, A

2000-08-01

Heller myotomy has long been utilized for patients failing nonoperative management of achalasia. Videoscopy has been advocated to decrease the morbidity of Heller myotomy; however, few reports document outcome beyond 1 year after videoscopic Heller myotomy. To determine perioperative morbidity, relief of dysphagia, and the incidence of postoperative reflux symptoms following videoscopic Heller myotomy with follow-up to over 4 years. Patients with achalasia documented by barium esophogram and esophageal manometry underwent videoscopic Heller myotomy beginning in 1992. Intraoperative peroral endoscopy was utilized to guide the cephalad and caudad extent of myotomy. A barium esophogram was undertaken in the immediate postoperative period to evaluate for subclinical leak and assess esophageal emptying. Seventy-eight patients underwent videoscopic Heller myotomy. The mean age was 51 years +/- 19 (range 14 to 91). Most (62%) patients had undergone pneumatic dilation prior to surgical consultation and 54% had previous botox injections. All patients complained of dysphagia and 40% had symptoms of heartburn prior to myotomy. After myotomy, 91% of patients stated that their swallowing was improved with myotomy. Thirteen patients (18%) experience heartburn more than once per week after myotomy. The average length of stay was 2 +/- 2 days, with 72% of patients spending 2 days or fewer in the hospital. Six (7.7%) major complications occurred: five esophageal perforations and one enterotomy without long-term sequellae. Three procedures (3.8%) were converted to "open" procedures. No deaths occurred. We conclude that videoscopic Heller myotomy is safe and efficacious, with low morbidity and mortality. Videoscopic myotomy provides relief beyond the short term for dysphagia due to achalasia with minimal reflux symptoms. We advocate videoscopic Heller myotomy in the treatment of severe dysphagia due to achalasia not adequately palliated by or amenable to nonoperative management

Recurrent achalasia after Heller-Toupet procedure: Laparoscopic extended redo heller myotomy and floppy Dor

PubMed Central

Golash, Vishwanath

2007-01-01

Recurrences of symptoms after the surgery for achalasia cardia are not uncommon. There are several causes of recurrences but the early recurrences are speculated to be secondary to incomplete myotomy and late recurrence due to fibrosis after the myotomy or megaesophagus. These recurrences can be managed by regular dilation failing which a redo surgery is indicated. Laparoscopic approach is now standard because of the obvious benefits for patients and surgeons. Extent of myotomy and addition of fundoplication are debatable issue in the management of achalasia cardia but evidence suggests that some kind of fundoplication would be necessary after the complete division of lower esophageal sphincter. We present our experience in a case of recurrent achalasia, secondary to incomplete myotomy managed laparoscopically by extended myotomy and a floppy anterior fundoplication. Patient is asymptomatic six months after the surgery and radiologically there is free passage of barium in the stomach. PMID:19789666

Angiopoietin-2 polymorphism in women with idiopathic recurrent miscarriage.

PubMed

Pietrowski, Detlef; Tempfer, Clemens; Bettendorf, Hertha; Bürkle, Bernd; Nagele, Fritz; Unfried, Gertrud; Keck, Christoph

2003-10-01

To investigate the relationship between idiopathic recurrent miscarriage and a polymorphism of the gene encoding for angiopoietin-2 (ANGPT2), an autochthonous modulator of angiogenesis during pregnancy. Prospective case control study. Academic research institution. One hundred thirty-one women with a history of three or more consecutive pregnancy losses before 20 weeks' gestation, and 125 healthy, postmenopausal controls with at least two live births and no history of pregnancy loss. Peripheral venous puncture. Polymerase chain reaction and restriction fragment length polymorphism analysis were performed to identify the different ANGPT2 alleles. No association between mutant (mt) allele and the occurrence of idiopathic recurrent miscarriage was found. Between women with primary and secondary idiopathic recurrent miscarriage, no statistically significant differences with respect to allele frequencies were observed. This is the first report on the ANGPT2 gene polymorphism in women with idiopathic recurrent miscarriage, demonstrating that the investigated polymorphism is not associated with idiopathic recurrent miscarriage in a white population.

Leptin Receptor Metabolism Disorder in Primary Chondrocytes from Adolescent Idiopathic Scoliosis Girls.

PubMed

Wang, Yun-Jia; Yu, Hong-Gui; Zhou, Zhen-Hai; Guo, Qiang; Wang, Long-Jie; Zhang, Hong-Qi

2016-07-20

To investigate the underlying mechanisms of low metabolic activity of primary chondrocytes obtained from girls with adolescent idiopathic scoliosis (AIS); AIS is a spine-deforming disease that often occurs in girls. AIS is associated with a lower bone mass than that of healthy individuals and osteopenia. Leptin was shown to play an important role in bone growth. It can also regulate the function of chondrocytes. Changes in leptin and Ob-R levels in AIS patients have been reported in several studies. The underlying mechanisms between the dysfunction of peripheral leptin signaling and abnormal chondrocytes remain unclear; The following parameters were evaluated in AIS patients and the control groups: total serum leptin levels; Ob-R expression in the plasma membrane of primary chondrocytes; JAK2 and STAT3 phosphorylation status. Then, we inhibited the lysosome and proteasome and knocked down clathrin heavy chain (CHC) expression in primary chondrocytes isolated from girls with AIS and evaluated Ob-R expression. We investigated the effects of leptin combined with a lysosome inhibitor or CHC knockdown in primary chondrocytes obtained from AIS patients; Compared with the controls, AIS patients showed similar total serum leptin levels, reduced JAK2 and STAT3 phosphorylation, and decreased cartilage matrix synthesis in the facet joint. Lower metabolic activity and lower membrane expression of Ob-R were observed in primary chondrocytes from the AIS group than in the controls. Lysosome inhibition increased the total Ob-R content but had no effect on the membrane expression of Ob-R or leptin's effects on AIS primary chondrocytes. CHC knockdown upregulated the membrane Ob-R levels and enhanced leptin's effects on AIS primary chondrocytes; The underlying mechanism of chondrocytes that are hyposensitive to leptin in some girls with AIS is low plasma membrane Ob-R expression that results from an imbalance between the rate of receptor endocytosis and the insertion of newly

Idiopathic ophthalmodynia and idiopathic rhinalgia: two topographic facial pain syndromes.

PubMed

Pareja, Juan A; Cuadrado, María L; Porta-Etessam, Jesús; Fernández-de-las-Peñas, César; Gili, Pablo; Caminero, Ana B; Cebrián, José L

2010-09-01

To describe 2 topographic facial pain conditions with the pain clearly localized in the eye (idiopathic ophthalmodynia) or in the nose (idiopathic rhinalgia), and to propose their distinction from persistent idiopathic facial pain. Persistent idiopathic facial pain, burning mouth syndrome, atypical odontalgia, and facial arthromyalgia are idiopathic facial pain syndromes that have been separated according to topographical criteria. Still, some other facial pain syndromes might have been veiled under the broad term of persistent idiopathic facial pain. Through a 10-year period we have studied all patients referred to our neurological clinic because of facial pain of unknown etiology that might deviate from all well-characterized facial pain syndromes. In a group of patients we have identified 2 consistent clinical pictures with pain precisely located either in the eye (n=11) or in the nose (n=7). Clinical features resembled those of other localized idiopathic facial syndromes, the key differences relying on the topographic distribution of the pain. Both idiopathic ophthalmodynia and idiopathic rhinalgia seem specific pain syndromes with a distinctive location, and may deserve a nosologic status just as other focal pain syndromes of the face. Whether all such focal syndromes are topographic variants of persistent idiopathic facial pain or independent disorders remains a controversial issue.

Laparoscopic Heller myotomy for achalasia: results after 10 years.

PubMed

Cowgill, Sarah M; Villadolid, Desiree; Boyle, Robert; Al-Saadi, Sam; Ross, Sharona; Rosemurgy, Alexander S

2009-12-01

Laparoscopic Heller myotomy was first undertaken in the early 1990s, and appreciable numbers of patients with 10-year follow-up periods are now available. This study was undertaken to determine long-term outcomes after laparoscopic Heller myotomy used to treat achalasia. Of 337 patients who have undergone laparoscopic Heller myotomy since 1992, 47 who underwent myotomy more than 10 years ago have been followed through a prospectively maintained registry. Among many symptoms, patients scored dysphagia, chest pain, vomiting, regurgitation, choking, and heartburn before and after myotomy using a Likert scale with choices ranging from 0 (never/not bothersome) to 10 (always/very bothersome). Symptom scores before and after myotomy were compared using a Wilcoxon matched-pairs test. Data are reported as median (mean ± standard deviation). The median length of the hospital stay was 2 days (mean, 3 ± 8.6 days; range, 1-60 days). Notable complications were infrequent after myotomy. There were no perioperative deaths. One patient required a redo myotomy after 5 years due to recurrence of symptoms. At this writing, 33 patients (70%) are still alive. The causes of death after discharge were unrelated to myotomy. The frequency and severity scores for dysphagia, chest pain, vomiting, regurgitation, choking, and heartburn all decreased significantly after laparoscopic Heller myotomy (p < 0.0001 for all). Laparoscopic Heller myotomy can be undertaken with few complications. This procedure significantly decreases the frequency and severity of achalasia symptoms without promoting heartburn. The symptoms of achalasia are durably ameliorated by laparoscopic Heller myotomy during long-term follow-up evaluation, thereby promoting application of this procedure.

Impact of esophageal flexion level on the surgical outcome in patients with sigmoid esophageal achalasia.

PubMed

Tsuboi, Kazuto; Omura, Nobuo; Yano, Fumiaki; Hoshino, Masato; Yamamoto, Se-Ryung; Akimoto, Shunsuke; Masuda, Takahiro; Kashiwagi, Hideyuki; Yanaga, Katsuhiko

2017-11-01

Esophageal achalasia can be roughly divided into non-sigmoid and sigmoid types. Laparoscopic surgery has been reported to be less than optimally effective for sigmoid type. The aim of this study was to examine the impact of the esophageal flexion level on the clinical condition and surgical outcomes of patients with sigmoid esophageal achalasia. The subjects were 36 patients with sigmoid esophageal achalasia who had been observed for >1 year after surgery. The subjects were divided into sigmoid type (Sg) and advanced sigmoid type (aSg) groups based on the flexion level of the lower esophagus to compare their clinical parameters and surgical outcomes. The Sg and aSg groups included 26 (72%) and 10 subjects, respectively. There were no marked differences in the clinical parameters or surgical outcomes between the two groups. However, the clearance rate calculated using the timed barium esophagogram was lower in the aSg group than in the Sg group. No differences were found in the postoperative symptom scores between the two groups, and both reported a high level of satisfaction. Although laparoscopic surgery for symptoms of sigmoid esophageal achalasia was highly successful regardless of the flexion level, the improvement in esophageal clearance was lower when the flexion level was higher.

Correlation Between Timed Barium Esophagogram and Esophageal Transit Scintigraphy Results in Achalasia.

PubMed

Park, Yoo Mi; Jeon, Han Ho; Park, Jae Jun; Kim, Jie-Hyun; Youn, Young Hoon; Park, Hyojin

2015-08-01

Timed barium esophagogram (TBE) and esophageal transit scintigraphy (ETS) have been adopted as useful ways to evaluate achalasia patients. TBE has merit as a simple, non-invasive, and convenient method. The study sought to compare the results of these two tests and verify their usefulness in evaluating treatment response. In addition, we assessed whether TBE could effectively replace ETS through correlation analysis. The medical records of 50 achalasia patients treated between September 2011 and June 2014 were reviewed retrospectively. The height and width of the barium column at 1, 2, and 5 min were measured by TBE. Half-life (T 1/2, min) and R 30 (percentage of remaining radioactivity 30 s after radioisotope ingestion) were measured by ETS. Both tests were performed before and after treatment, and the tests were carried out 1 and 2 days after procedures. And we analyzed the correlation between the parameters from the two tests. The parameters of TBE and ETS were improved after treatment (p < 0.05). Before treatment, the height and width results at 5 min from TBE positively correlated with the T 1/2 parameter from ETS (correlation coefficients of 0.59 and 0.75, respectively). After treatment, the correlation coefficients between the 5-min height and width of the barium column by TBE and T 1/2 by ETS were 0.55 and 0.46, respectively. Both TBE and ETS are useful modalities in assessing esophageal emptying and response to achalasia treatment. TBE and ETS results have a statistically significant correlation both pre- and post-treatment. We suggest that TBE could effectively replace ETS for the assessment of achalasia.

Minimally Invasive Surgical Treatment of Esophageal Achalasia

PubMed Central

Mercantini, Paolo; Amodio, Pietro M.; Stipa, Francesco; Corigliano, Nicola; Ziparo, Vincenzo

2003-01-01

Background and Objectives: A minimally invasive approach is considered the treatment of choice for esophageal achalasia. We report the evolution of our experience from thoracoscopic Heller myotomy (THM) to laparoscopic Heller myotomy (LHM). Our objective is to define the efficacy and safety of these 2 approaches. Methods: Between March 1993 and December 2001, 36 patients underwent minimally invasive surgery for achalasia. Sixteen patients underwent THM without an antireflux procedure, and 20 patients underwent LHM with partial anterior fundoplication (n=13) or closure of the angle of His (n=7). Results: Mean operative time and mean hospital stay were significantly shorter for LHM compared with that of THM (148.3±38.7 vs 222±46.1 min, respectively; P=0.0001) and (2.06±0.65 days vs 5.06±0.85 days, respectively; P=0.0001). Six of 16 patients (37.5%) in the THM group experienced persistent or recurrent dysphagia compared with 1 of 20 patients (5%) in the LHM group (P=0.01). Heartburn developed in 5 patients (31.2%) after THM and in 1 patient (5%) after LHM (P=0.06). Regurgitation developed in 4 patients (25%) after THM and in 2 patients (10%) after LHM (P=0.2). Lower esophageal sphincter (LES) basal pressure decreased significantly from 30.1±5.07 to 15.3±2.1 after THM and from 31.8±6.2 to 10.4±1.7 after LHM (P=0.0001). Mean esophageal diameter was significantly reduced after LHM compared with that after THM (from 53.9±5.9 mm to 27.2±3.3 mm vs 50.8±7.6 mm to 37.2±6.9 mm respectively; P=0.0001). Conclusion: In our experience, LHM is associated with better short-term results and is superior to THM in relieving dysphagia. LHM with partial anterior fundoplication should be considered the treatment of choice for achalasia. PMID:14558709

Self-expandable metal stent placement in a child for treatment of achalasia after failed Heller myotomy.

PubMed

Gugig, Roberto; Muñoz Jurado, Guillermo; Huang, Clifton; Oleas, Roberto; Robles-Medranda, Carlos

2018-01-01

Background and study aims  Childhood achalasia treatment remains inconclusive. What is next after myotomy failure? Repeated pneumatic-dilation put patients at greater risk of perforation with possible symptom recurrence. We report on a 12-year-old patient with a 1-year history of achalasia whom underwent Heller myotomy with fundoplication and recurred with symptoms 1 week after surgery. Pneumatic dilatation was considered but not done because of the risk of esophageal perforation. The decision was made to place a fully covered self-expanding metallic stent (FC-SEMS) for 3 months, which resolved the stenosis as confirmed by esophagram. The patient has remained asymptomatic since the procedure was performed 2 years ago. FC-SEMS is an alternative for treatment of refractory achalasia in children who do not respond to conventional treatment.

Achalasia leading to diagnosis of adenocarcinoma of the oesophagus.

PubMed

Segal, Jonathan; Lagundoye, Ayodele; Carter, Martyn

2017-06-20

A 50-year-old male with a 7 month history of progressive dysphagia to solids then subsequently to liquids. He underwent a diagnostic gastroscopy which was normal. A further barium swallow suggested achalasia. He was referred to a tertiary centre, where he underwent pH and manometry studies which confirmed a diagnosis of achalasia. He was referred for a laparoscopic cardiomyotomy, and at surgery there was a suspected tumour at the gastro-oesophageal junction. A follow-up endoscopy with biopsies was normal. Following this, a positron emission tomography scan showed T3 distal oesophageal cancer with no nodal involvement or distal metastasis. An attempt at oesophagectomy was performed, but at operation there was locally advanced carcinoma infiltrating the coeliac axis. He is currently undergoing palliative chemotherapy. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Idiopathic toe walking.

PubMed

Oetgen, Matthew E; Peden, Sean

2012-05-01

Toe walking is a bilateral gait abnormality in which a normal heel strike is absent and most weight bearing occurs through the forefoot. This abnormality may not be pathologic in patients aged <2 years, but it is a common reason for referral to an orthopaedic surgeon. Toe walking can be caused by several neurologic and developmental abnormalities and may be the first sign of a global developmental problem. Cases that lack a definitive etiology are categorized as idiopathic. A detailed history, with careful documentation of the developmental history, and a thorough physical examination are required in the child with a primary report of toe walking. Treatment is based on age and the severity of the abnormality. Management includes observation, stretching, casting, bracing, chemodenervation, and surgical lengthening of the gastrocnemius-soleus complex and/or Achilles tendon. An understanding of idiopathic toe walking as well as treatment options and their outcomes can help the physician individualize treatment to achieve optimal results.

Response of Esophagus to High and Low Temperatures in Patients With Achalasia

PubMed Central

Ren, Yutang; Fang, Xiucai; Zhu, Liming; Sun, Xiaohong; Wang, Zhifeng; Wang, Ruifeng; Wei, Zhao; Wen, Ping; Xin, Haiwei; Chang, Min

2012-01-01

Background/Aims Achalasia patients would feel exacerbated dysphagia, chest pain and regurgitation when they drink cold beverages or eat cold food. But these symptoms would relieve when they drink hot water. Reasons are unknown. Methods Twelve achalasia patients (mean age, 34 ± 10 years; F:M, 3:9) who never had any invasive therapies were chosen from Peking Union Medical College Hospital. They were asked to fill in the questionnaire on eating habits including food temperature and related symptoms and to receive high-resolution manometry examination. The exam was done in 2 separated days, at swallowing room temperature (25℃) then hot (50℃) water, and at room temperature (25℃) then cold (2℃) water, respectively. Parameters associated with esophageal motility were analyzed. Results Most patients (9/12) reported discomfort when they ate cold food. All patients reported no additional discomfort when they ate hot food. Drinking hot water was effective in 5/8 patients who ever tried to relieve chest pain attacks. On manometry, cold water increased lower esophageal sphincter (LES) resting pressure (P = 0.003), and prolonged the duration of esophageal body contraction (P = 0.002). Hot water decreased LES resting pressure and residue pressure during swallow (P = 0.008 and P = 0.002), increased LES relaxation rate (P = 0.029) and shortened the duration of esophageal body contraction (P = 0.003). Conclusions Cold water could increase LES resting pressure, prolong the contraction duration of esophageal body, and exacerbate achalasia symptoms. Hot water could reduce LES resting pressure, assist LES relaxation, shorten the contraction duration of esophageal body and relieve symptoms. Thus achalasia patients are recommended to eat hot and warm food and avoid cold food. PMID:23105999

Yield of prolonged wireless pH monitoring in achalasia patients successfully treated with pneumatic dilation

PubMed Central

Franchina, Marianna; Elvevi, Alessandra; Pugliese, Delia; Tenca, Andrea; Conte, Dario; Penagini, Roberto

2016-01-01

Background Gastro-oesophageal reflux disease (GORD) is a long-term complication of achalasia treatments. The aim of our study was to evaluate the yield of prolonged wireless pH monitoring in patients with successfully treated achalasia and its influence on proton pump inhibitor (PPI) use. Methods Twenty-five patients with achalasia who underwent prolonged wireless pH monitoring after a successful treatment with pneumatic dilation were enrolled. pH variables were analysed in the first 24 hours of monitoring to determine if tracings were indicative of GORD; the same variables were analysed in the following 24-hour period in order to obtain a worst-day diagnosis of GORD. PPI therapy before and after the test was recorded. Results Five out of 25 patients had GORD diagnosis during the first day of monitoring and four of them had oesophagitis at endoscopy. During the following days of monitoring four more patients had a diagnosis of GORD. Out of the 25 patients, PPIs were started after the test in six asymptomatic GORD-positive ones, whereas prescription of PPIs was stopped without detrimental effect on symptoms in three GORD-negative patients. Conclusions Prolonged wireless pH monitoring is a useful test to be added to endoscopy in order to evaluate GORD and to optimise antisecretory treatment in successfully treated achalasia patients. PMID:29026592

Treatment choices and outcomes of patients with manometrically diagnosed achalasia.

PubMed

Yeung, J C; Finley, C; Hanna, W C; Miller, L; Ferri, L; Urbach, D R; Darling, G E

2016-07-01

This prospective population-based study was designed to evaluate treatment choices in patients with new manometrically diagnosed achalasia and their outcomes. Patients referred to the esophageal function laboratory were enrolled after a new manometric diagnosis of achalasia. Patients completed an initial achalasia symptom score validated questionnaire on their symptom severity, duration, treatment pre-diagnosis and Medical Outcomes Study 36-item Short-Form (SF-36) survey. Treatment decisions were made by the referring physician and the patient. Follow-up questionnaires were completed every 3 months for 1 year. Patients who chose not to undergo treatment at 1-year follow-up completed another questionnaire after 5 years. Between January 2004 and January 2005, 83 of 124 eligible patients were enrolled. Heller myotomy was performed on 31 patients, three patients received botulinum toxin injections, and 25 patients received 29 pneumatic balloon dilatations. Twenty-four patients chose to receive no treatment. Following treatment, patients treated with surgery, dilatation and botulinum toxin had an average improvement in achalasia symptom score of 23 +/- 12.2, 17 +/- 10.9, and 9 +/- 14, respectively. Patients receiving no treatment had worsening symptoms with a symptom score change of -3.5 +/- 11.4. Surgery and dilatation resulted in significant improvement (P < 0.01) relative to no treatment. In univariate logistic regression, symptom severity score (odds ratio [OR] 1.04, 95% confidence interval [CI] 1.00 to 1.08), sphincter tone (OR 1.04, 95% CI 1.00 to 1.09), difficulty swallowing liquids (OR 3.21, 95% 1.15 to 8.99), waking from sleep (OR 2.75, 95% 1.00 to 7.61), and weight loss (OR 5.99, 95% CI 1.93 to 18.58) were all significant in predicting that patients would select treatment. In the multivariate analysis, older age (OR 1.05, 95% CI 1.01 to 1.09) and weight loss (OR 3.91, 95% CI 1.02 to 15.2) were statistically significant for undergoing treatment. At 5 years

Impaired bolus clearance in combined high-resolution esophageal manometry and impedance measurement helps to differentiate between esophagogastric junction outflow obstruction and achalasia.

PubMed

Zizer, Eugen; Seufferlein, Thomas; Hänle, Mark Martin

2017-02-01

Introduction and aims  High-resolution esophageal manometry (HRM) has improved the diagnostic work-up of esophageal motility disorders. Simultaneous evaluation of bolus clearance delivers useful information about the function of tubular esophagus. We assessed bolus clearance in a combined HRM-impedance examination for esophagogastric junction outflow obstruction (EGJOO) in comparison to achalasia patients. The collected data were assessed in a retrospective analysis. Patients and methods  After gastroscopy excluded a mechanical esophageal or gastric obstruction, 142 consecutive patients underwent combined HRM-impedance examination. The assessment and interpretation of the manometry results were done according to the Chicago Classification of esophageal motility disorders v3.0. After classifying the motility disorder, the evaluation of bolus clearance was done according to published studies. Results  All patients with achalasia (n = 24) showed a significantly impaired bolus clearance (< 80 %). Patients with unaffected peristalsis (n = 56) or patients with EGJOO (n = 14) each showed impaired clearance in 7 %, respectively. The evidence of axial hernia was not associated with impaired clearance. Conclusion  Our results demonstrate a significant difference in impedance measurements between EGJOO and achalasia cases. This might be helpful as an additional tool to differentiate between achalasia and EGJOO patients. Furthermore, the role of the combined impedance-HRM investigation for early diagnosis of achalasia in "pre-achalasia" condition or in evaluation of potential progress of EGJOO to achalasia should be evaluated in a prospective study. © Georg Thieme Verlag KG Stuttgart · New York.

Cost-effectiveness of per oral endoscopic myotomy relative to laparoscopic Heller myotomy for the treatment of achalasia.

PubMed

Greenleaf, Erin K; Winder, Joshua S; Hollenbeak, Christopher S; Haluck, Randy S; Mathew, Abraham; Pauli, Eric M

2018-01-01

Per oral endoscopic myotomy (POEM) has recently emerged as a viable option relative to the classic approach of laparoscopic Heller myotomy (LHM) for the treatment of esophageal achalasia. In this cost-utility analysis of POEM and LHM, we hypothesized that POEM would be cost-effective relative to LHM. A stochastic cost-utility analysis of treatment for achalasia was performed to determine the cost-effectiveness of POEM relative to LHM. Costs were estimated from the provider perspective and obtained from our institution's cost-accounting database. The measure of effectiveness was quality-adjusted life years (QALYs) which were estimated from direct elicitation of utility using a visual analog scale. The primary outcome was the incremental cost-effectiveness ratio (ICER). Uncertainty was assessed by bootstrapping the sample and computing the cost-effectiveness acceptability curve (CEAC). Patients treated within an 11-year period (2004-2016) were recruited for participation (20 POEM, 21 LHM). During the index admission, the mean costs for POEM ($8630 ± $2653) and the mean costs for LHM ($7604 ± $2091) were not significantly different (P = 0.179). Additionally, mean QALYs for POEM (0.413 ± 0.248) were higher than that associated with LHM (0.357 ± 0.338), but this difference was also not statistically significant (P = 0.55). The ICER suggested that it would cost an additional $18,536 for each QALY gained using POEM. There was substantial uncertainty in the ICER; there was a 48.25% probability that POEM was cost-effective at the mean ICER. At a willingness-to-pay threshold of $100,000, there was a 68.31% probability that POEM was cost-effective relative to LHM. In the treatment of achalasia, POEM appears to be cost-effective relative to LHM depending on one's willingness-to-pay for an additional QALY.

Gender differences in both the pathology and surgical outcome of patients with esophageal achalasia.

PubMed

Tsuboi, Kazuto; Omura, Nobuo; Yano, Fumiaki; Hoshino, Masato; Yamamoto, Se-Ryung; Akimoto, Shusuke; Masuda, Takahiro; Kashiwagi, Hideyuki; Yanaga, Katsuhiko

2016-12-01

Esophageal achalasia is a relatively rare disease that occurs usually in middle-aged patients. The laparoscopic Heller-Dor (LHD) procedure is the gold-standard surgical treatment for esophageal achalasia. There are many studies on the pathology and surgical outcome of esophageal achalasia from various perspectives, but there are no studies on gender differences in both the pathology and surgical outcome. This study aimed to evaluate gender differences in the surgical outcome with the LHD procedure and in the pathology of esophageal achalasia patients. The study included 474 LHD-treated patients who were postoperatively followed up for 6 months or more. The patients were divided into 2 groups by gender, to compare the preoperative pathology, surgical outcome, symptom scores before and after LHD, symptom score improvement frequency, and patient satisfaction with the surgery. The study population consisted of 248 male and 226 female, having a mean age of 45.1 years. There were no gender differences in the preoperative pathology, but a significantly lower BMI (p < 0.0001) and a smaller esophageal dilation (p = 0.0061) were observed in the female group. The frequency and severity of chest pain before the surgery were significantly higher in the female group (p = 0.0117 and p = 0.0103, respectively), and the improvement in both the frequency and severity of chest pain was significantly higher in the female group (p = 0.0005 and p = 0.003, respectively). No differences were identified in the surgical outcomes and postoperative course. The patient satisfaction with the surgery was high in both groups and comparable (p = 0.6863). The female patients with esophageal achalasia were characterized by low BMI, less esophageal dilation, and increased frequency and severity of chest pain. LHD improved the chest pain in the female patients, whereas the surgical outcome and satisfaction with the surgery were excellent regardless of gender.

Peroral endoscopic myotomy for the treatment of esophageal achalasia: systematic review and pooled analysis.

PubMed

Patel, K; Abbassi-Ghadi, N; Markar, S; Kumar, S; Jethwa, P; Zaninotto, G

2016-10-01

Peroral endoscopic myotomy (POEM) is a novel approach to performing esophageal myotomy for the treatment of achalasia. This review aims to assess subjective and objective metrics of achalasia treatment efficacy, perioperative adverse events and the incidence of postoperative gastroesophageal reflux disease in patients treated with POEM. Secondary aims include a pooled analysis comparison of the clinical outcomes and procedural safety of POEM versus laparoscopic Heller's myotomy (LHM). A systematic review of the literature, up to and including January 15, 2015, was conducted for studies reporting POEM outcomes. Studies comparing POEM to LHM were also included for the purpose of pooled analysis. Outcomes from 1122 POEM patients, from 22 studies, are reported in this systematic review. Minor operative adverse events included capno/pneumo-peritoneum (30.6%), capno/pneumo-thorax (11.0%) and subcutaneous emphysema (31.6%). Major operative adverse events included mediastinal leak (0.3%), postoperative bleeding (1.1%) and a single mortality (0.09%). There was an improvement in lower esophageal sphincter pressure and timed barium esophagram column height of 66% and 80% post-POEM, respectively. Symptom improvement was demonstrated with a pre- and post-POEM Eckardt score ± standard deviation of 6.8 ± 1.0 and 1.2 ± 0.6, respectively. Pre- and post-POEM endoscopy showed esophagitis in 0% and 19% of patients, respectively. The median (interquartile range) points scored for study quality was 15 (14-16) out of total of 32. Pooled analysis of three comparative studies between LHM and POEM showed similar results for adverse events, perforation rate, operative time and a nonsignificant trend toward a reduced length of hospital stay in the POEM group. In conclusion, POEM is a safe and effective treatment for achalasia, showing significant improvements in objective metrics and achalasia-related symptoms. Randomized comparative studies of LHM and POEM are required to determine the

Per-oral endoscopic myotomy (POEM): a new endoscopic treatment for achalasia.

PubMed

Miranda García, Pablo; Casals Seoane, Fernando; Gonzalez, Jean-Michel; Barthet, Marc; Santander Vaquero, Cecilio

2017-10-01

Per-oral endoscopic myotomy (POEM) is a new minimally invasive technique to treat achalasia. We performed a review of the literature of POEM with a special focus on technical details and the results obtained with this technique in patients with achalasia and other esophageal motility disorders. Thousands of POEM procedures have been performed worldwide since its introduction in 2008. The procedure is based on the creation of a mucosal entry point in the proximal esophagus to reach the cardia through a submucosal tunnel and then perform a myotomy of the muscular layers of the cardia, esophagogastric junction and distal esophagus, as performed in a Heller myotomy. The clinical remission rate ranges from 82 to 100%. Although no randomized studies exist and available data are from single-center studies, no differences have been found between laparoscopic Heller myotomy (LHM) and POEM in terms of perioperative outcomes, short-term outcomes (12 months) and long-term outcomes (up to three years). Procedure time and length of hospital stay were lower for POEM. Post-POEM reflux is a concern, and controversial data have been reported compared to LHM. The technique is safe, with no reported deaths related to the procedure and an adverse event rate comparable to surgery. Potential complications include bleeding, perforation, aspiration and insufflation-related adverse events. Thus, this is a complex technique that needs specific training even in expert hands. The indication for this procedure is widening and other motor hypercontractil esophageal disorders have been treated by POEM with promising results. POEM can be performed in complicated situations such as in pediatric patients, sigmoid achalasia or after failure of previous treatments. POEM is an effective treatment for achalasia and is a promising tool for other motor esophageal disorders. It is a safe procedure but, due to its technical difficulty and possible associated complications, the procedure should be performed

Is Dor fundoplication optimum after laparoscopic Heller myotomy for achalasia? A meta-analysis.

PubMed

Wei, Ming-Tian; He, Ya-Zhou; Deng, Xiang-Bing; Zhang, Yuan-Chuan; Yang, Ting-Han; Jin, Cheng-Wu; Hu, Bing; Wang, Zi-Qiang

2013-11-21

To compare the outcome of acid reflux prevention by Dor fundoplication after laparoscopic Heller myotomy (LHM) for achalasia. Electronic database PubMed, Ovid (Evidence-Based Medicine Reviews, EmBase and Ovid MEDLINE) and Cochrane Library were searched between January 1995 and September 2012. Bibliographic citation management software (EndNote X3) was used for extracted literature management. Quality assessment of random controlled studies (RCTs) and non-RCTs was performed according to the Cochrane Handbook for Systematic Reviews of Interventions 5.1.0 and a modification of the Newcastle-Ottawa Scale, respectively. The data were analyzed using Review Manager (Version 5.1), and sensitivity analysis was performed by sequentially omitting each study. Finally, 6 studies, including a total of 523 achalasia patients, compared Dor fundoplication with other types of fundoplication after LHM (Dor-other group), and 8 studies, including a total of 528 achalasia patients, compared Dor fundoplication with no fundoplication after LHM (Dor-no group). Dor fundoplication was associated with a significantly higher recurrence rate of clinical regurgitation and pathological acid reflux compared with the other fundoplication group (OR = 7.16, 95%CI: 1.25-40.93, P = 0.03, and OR = 3.79, 95%CI: 1.23-11.72, P = 0.02, respectively). In addition, there were no significant differences between Dor fundoplication and no fundoplication in all subjects. Other outcomes, including complications, dysphagia, postoperative physiologic testing, and operation-related data displayed no significant differences in the two comparison groups. Dor fundoplication is not the optimum procedure after LHM for achalasia. We suggest more attention should be paid on quality of life among different fundoplications.

Anesthetic management of peroral endoscopic myotomy for esophageal achalasia: a retrospective case series.

PubMed

Tanaka, Eriko; Murata, Hiroaki; Minami, Hitomi; Sumikawa, Koji

2014-06-01

Peroral endoscopic myotomy (POEM) is a newly developed, less invasive treatment for esophageal achalasia that requires general anesthesia under positive pressure ventilation. In this retrospective case series, we describe the anesthetic management of 28 consecutive patients who underwent POEM for esophageal achalasia. Anesthesia was maintained with sevoflurane and remifentanil under positive pressure ventilation through a tracheal tube. Retained contents in the esophagus were evacuated just before anesthesia induction to prevent regurgitation into the trachea. The POEM procedure was performed using an orally inserted flexible fiberscope. Elevation of end-tidal carbon dioxide after initiating esophageal carbon dioxide insufflation was observed in all patients and was treated by minute adjustments to the ventilation volume. Scopolamine butylbromide-induced tachycardia in one patient was treated with landiolol hydrochloride, which is a short-acting beta 1-selective blocker. Minor subcutaneous emphysema around the neck was observed in one patient. POEM was successfully completed, and tracheas were extubated immediately after the procedure in all patients. Our findings suggest that prevention of aspiration pneumonia during anesthesia induction, preparation for carbon dioxide insufflation-related complications, and treatment of scopolamine butylbromide-induced tachycardia play important roles in safe anesthesia management of POEM for esophageal achalasia.

Economic evaluation of the randomized European Achalasia trial comparing pneumodilation with Laparoscopic Heller myotomy.

PubMed

Moonen, A; Busch, O; Costantini, M; Finotti, E; Tack, J; Salvador, R; Boeckxstaens, G; Zaninotto, G

2017-11-01

A recent multicenter randomized trial in achalasia patients has shown that pneumatic dilation resulted in equivalent relief of symptoms compared to laparoscopic Heller myotomy. Additionally, the cost of each treatment should be also taken in consideration. Therefore, the aim of the present study was to perform an economic analysis of the European achalasia trial. Patients with newly diagnosed achalasia were enrolled from to 2003 to 2008 in 14 centers in five European countries and were randomly assigned to either pneumatic dilation (PD) or laparoscopic Heller (LHM). The economic analysis was performed in the three centers in three different countries where most patients were enrolled (Amsterdam [NL], Leuven, [B] and Padova [I]) and then applied to all patients included in the study. The total raw costs of the two treatments per patient include the initial costs, the costs of complications, and the costs of retreatments. Two hundred and one patients, 107 (57 males and 50 females, mean age 46 CI: 43-49 years) were randomized to LHM and 94 (59 males and 34 females, mean age 46 CI 43-50 years) to PD. The total cost of PD per patient was quite comparable in the three different centers; €3397 in Padova, €3259 in Amsterdam and €3792 in Leuven. For LHM, the total costs per patient were highest in Amsterdam: €4488 in Padova, €6720 in Amsterdam, and €5856 in Leuven. In conclusion, the strategy of treating achalasia starting with PD appears the most economic approach, independent of the health system. © 2017 John Wiley & Sons Ltd.

Laparoscopic graduated cardiomyotomy with anterior fundoplication as treatment for achalasia: experience of 48 cases.

PubMed

Etchegaray-Dondé, A; Rodríguez-Espínola, G; Higuera-Hidalgo, F; Ortiz-Higareda, V; Chapa-Azuela, O; Etchegaray-Solana, A

At the Upper Gastrointestinal Tract Clinic of the Hospital General de Mexico, achalasia treatment has been standarized through strictly graduated cardiomyotomy. This procedure guarantees a complete myotomy for the satisfactory resolution of dysphagia, a characteristic symptom of achalasia. To ensure the inclusion of the entire lower esophageal sphincter, an 8cm Penrose drain is placed at the surgical site 6cm above the gastroesophageal junction and 2cm in a caudal direction, for accurate laparoscopic measuring. The aim of our study was to evaluate the results of this technique. A descriptive, retrospective, longitudinal, and observational study was conducted on a cohort of patients diagnosed with achalasia at the Upper Gastrointestinal Tract Clinic of the Hospital General de México "Dr. Eduardo Liceaga". The study included 48 patients, 40 of whom had no prior surgical treatment and 8 that presented with recurrence. Forty-seven patients (97.9%) underwent a laparoscopic procedure and conversion to open surgery was required in 2 of them (4.25% conversion rate). Postoperative progression was satisfactory in all cases, with mean oral diet commencement at 52h and mean hospital stay of 5.7 days. No recurrence was registered during the mean follow-up period of 35.75 months and there were no deaths. Laparoscopic graduated (strictly measured) cardiomyotomy with anterior fundoplication is a reproducible, efficacious, and safe option for the surgical treatment of achalasia. Copyright © 2017 Asociación Mexicana de Gastroenterología. Publicado por Masson Doyma México S.A. All rights reserved.

Long-term results of laparoscopic treatment of esophageal achalasia in children: a multicentric survey.

PubMed

Esposito, Ciro; Riccipetitoni, Giovanna; Chiarenza, Salvatore Fabio; Roberti, Agnese; Vella, Claudio; Alicchio, Francesca; Fava, Giorgio; Escolino, Maria; De Pascale, Teresa; Settimi, Alessandro

2013-11-01

This report describes three Italian centers' experience in the treatment of children with esophageal achalasia. Between June 2000 and June 2012, 31 children (13 girls and 18 boys, with a median age of 8.4 years) affected by esophageal achalasia were treated in three different institutions with an esophagomyotomy according to Heller's procedure via laparoscopy associated with a Dor antireflux procedure. Between 2000 and 2005 (for 14 patients) we used mono- or bipolar coagulation to perform myotomy; after 2005 (for 17 patients) we used the new hemostatic devices to perform it. Median length of surgery was 120 minutes. Median hospital stay was 4 days. We recorded eight complications in our series: 3 patients (9.6%) had a mucosal perforation, and 5 children (16.1%) presented dysphagia after surgery. When comparing the data before and after 2005, it seems that the new hemostatic devices statistically shortened the length of surgery (P<.01, Student's t test). On the basis of our experience, laparoscopic Heller's myotomy associated with an antireflux procedure is a safe and effective method for the treatment of achalasia in the pediatric population. Intraoperative complications were <10%, and they occurred mostly at the beginning of our experience. Residual dysphagia occurred in about 16% of cases. The use of the new hemostatic devices seems to reduce the length of surgery and intraoperative bleeding. Considering the rarity of this pathology, we believe that patients with achalasia have to be treated only at centers with a strong experience in the treatment of this pathology.

Lymphocytic Esophagitis in Non-Achalasia Primary Esophageal Motility Disorders: Improved Criteria, Prevalence, Strength of Association and Natural History

PubMed Central

Putra, Juan; Muller, Kristen E.; Hussain, Zilla H.; Parker, Siddhartha; Gabbard, Scott; Brickley, Elizabeth B.; Lacy, Brian E.; Rothstein, Richard; Lisovsky, Mikhail

2017-01-01

Lymphocytic esophagitis (LE) is a histologic pattern with no established clinical correlates in the majority of patients. The goal of this study was to evaluate the association between non-achalasia primary esophageal motility disorders (PMED) and LE. Sixty-nine patients with PMED and esophageal biopsies, including 22 with nutcracker esophagus, 33 with ineffective motility and 14 with diffuse spasm, constituted the study group. The control group consisted of 70 patients with severe dysmotility-negative gastroesophageal reflux disease (GERD) requiring referral for Nissen fundoplication. To improve the criteria for LE, a lymphocyte reference range at different esophageal levels was first established in 17 healthy volunteers. The cutoffs for normal intraepithelial lymphocytes (IEL), defined as lymphocyte levels not exceeding mean level + 2 standard deviations, were set at 62, 46, and 41 lymphocytes per high power field at 0 to 2 cm, 5 cm, and 10 cm above the gastroesophageal junction, respectively. Predominantly focal peripapillary LE was observed in approximately 40% of patients with nutcracker esophagus or diffuse spasm and 20% of patients with ineffective motility, in comparison to 4% of patients with dysmotility-negative GERD (P < 0.035 versus any subtype of PMED). Overall, LE was strongly associated with PMED in multivariate analysis (adjusted odds ratio, 7.93; 95% CI: 2.26, 27.9; P=0.001). LE had a chronic course in 56% of the patients with follow-up biopsies. In conclusion, LE has a strong association with PMED, suggesting utility of LE in raising the possibility of PMED. PMID:27526295

Idiopathic anaphylaxis.

PubMed

Fenny, Nana; Grammer, Leslie C

2015-05-01

Idiopathic anaphylaxis is a diagnosis of exclusion after other causes have been thoroughly evaluated and excluded. The pathogenesis of idiopathic anaphylaxis remains uncertain, although increased numbers of activated lymphocytes and circulating histamine-releasing factors have been implicated. Signs and symptoms of patients diagnosed with idiopathic anaphylaxis are indistinguishable from the manifestations of other forms of anaphylaxis. Treatment regimens are implemented based on the frequency and severity of patient symptoms and generally include the use of epinephrine autoinjectors, antihistamines, and steroids. The prognosis of idiopathic anaphylaxis is generally favorable with well-established treatment regimens and effective patient education. Copyright © 2015 Elsevier Inc. All rights reserved.

Peroral endoscopic myotomy for achalasia in patients aged ≥ 65 years

PubMed Central

Li, Chen-Jie; Tan, Yu-Yong; Wang, Xue-Hong; Liu, De-Liang

2015-01-01

AIM: To investigate the safety and efficacy of peroral endoscopic myotomy (POEM) for achalasia in patients aged ≥ 65 years. METHODS: From November 2011 to August 2014, 15 consecutive patients (aged ≥ 65 years) diagnosed with achalasia were prospectively recruited and all underwent POEM at our institution. Eckardt score, lower esophageal sphincter (LES) pressure, esophageal diameter, SF-36 questionnaire, and procedure-related complications were used to evaluate the outcomes. RESULTS: All 15 patients were treated successfully by POEM, with a median operation time of 55 min. Median myotomy length was 10 cm. Three patients underwent circular myotomy and 12 full-thickness myotomies. Symptoms remitted in all cases during post-POEM follow-up of 6-39 mo. Eckardt score reduced significantly (pre-operation vs post-operation: 8.0 vs 1.0, P < 0.05). Median LES pressure decreased from 27.4 to 9.6 mmHg (P < 0.05). Median diameter of the esophagus was significantly decreased (pre-operation vs post-operation: 51.0 mm vs 30.0 mm, P < 0.05). Only one patient had reflux, which was resolved with oral proton pump inhibitors. No serious complications related to POEM were found. The quality of life of the 15 patients improved significantly after POEM. CONCLUSION: POEM is a safe and effective technique for treatment of achalasia in patients aged ≥ 65 years, with improvement in symptoms and quality of life. PMID:26290645

Short-term surgical outcomes of reduced port surgery for esophageal achalasia.

PubMed

Omura, Nobuo; Yano, Fumiaki; Tsuboi, Kazuto; Hoshino, Masato; Yamamoto, Se Ryung; Akimoto, Shunsuke; Ishibashi, Yoshio; Kashiwagi, Hideyuki; Yanaga, Katsuhiko

2015-09-01

To clarify the feasibility and utility of reduced port surgery (RPS) for achalasia. Between September 2005 and June 2013, 359 patients with esophageal achalasia, excluding cases of reoperation, underwent laparoscopic Heller myotomy and Dor fundoplication (LHD) according to our clinical pathway. Three-hundred and twenty-seven patients underwent LHD with five incisions (conventional approach), while the other 32 patients underwent RPS, including eight via SILS. The clinical data were collected in a prospective fashion and retrospectively reviewed. We selected 24 patients matched for gender, age and morphologic type with patients in the RPS group from among the 327 patients (C group). The surgical outcomes were compared between the C and RPS groups. There were no significant differences between the two groups in the duration of symptoms, dysphagia score, chest pain score, shape of the distal esophagus and esophageal clearance. The operative time was significantly longer in the RPS group than in the C group (p < 0.001). There were no significant differences between the two groups in the length of postoperative hospital stay or rates of bleeding, mucosal injury of the esophagus and/or stomach and postoperative complications. The symptom scores significantly improved after surgery in both groups (p < 0.001). Furthermore, there were no significant differences between the C group and RPS group in terms of the postoperative symptom scores or satisfaction scores after surgery. The surgical outcomes of RPS for achalasia are comparable to those obtained with the conventional method.

The evaluation of functional results before and after laparoscopic Heller myotomy for achalasia: a single center experience.

PubMed

Pooshani, Abdollah; Frootan, Mojgan; Abdi, Saeed; Jahani-Sherafat, Somayeh; Kamani, Freshteh

2017-01-01

The aim of this study was to evaluate and compare the functional results before and after laparoscopic Heller myotomy for Iranian patients with achalasia. Achalasia is a severe neuromuscular disorder of the esophagus, characterized by the loss of peristalsis and an inability of the lower esophageal sphincter (LES) to reach optimal relaxation. In this cross sectional study, patients who underwent Heller myotomy for achalasia via laparoscopy in Taleghani Hospital Tehran, Iran were evaluated. Symptoms including pressure of residual, integrated relaxation sphincter (IRP), pressure of free drinking, pressure of LES, dysphasia score and peristalsis movement was measured and recorded by manometry before and after (2 months) treating with Heller myotomy. In this study, 23 patients with achalasia (12 females and 11 males) with a mean age of 30±3.5 years (minimum 20, maximum 44 years) who met the inclusion criteria of the study were examined. Results of this study showed that, all the diagnostic criteria that were measured before the treatment was significantly different from after the treatment (P<0.05). The average decline in LES, IRP, Residual Pressure, Free drinking esophagus, and dysphasia score were 23.1 mmHg, 16.24 mmHg, 18,7 mmHg, 18.9 mmHg, and 5.0 unit, respectively. Also the average increase of the peristalsis movement was 8.26±13.7 in 8 patients. Considering the results of Heller myotomy surgery can be as a treatment of choice for achalasia. Free Drinking pressure can be a suitable criteria after treatment for evaluation and prediction of the reducing the dysphasia score after the surgery.

The evaluation of functional results before and after laparoscopic Heller myotomy for achalasia: a single center experience

PubMed Central

Pooshani, Abdollah; Frootan, Mojgan; Abdi, Saeed; Jahani-Sherafat, Somayeh; Kamani, Freshteh

2017-01-01

Aim: The aim of this study was to evaluate and compare the functional results before and after laparoscopic Heller myotomy for Iranian patients with achalasia. Background: Achalasia is a severe neuromuscular disorder of the esophagus, characterized by the loss of peristalsis and an inability of the lower esophageal sphincter (LES) to reach optimal relaxation. Methods: In this cross sectional study, patients who underwent Heller myotomy for achalasia via laparoscopy in Taleghani Hospital Tehran, Iran were evaluated. Symptoms including pressure of residual, integrated relaxation sphincter (IRP), pressure of free drinking, pressure of LES, dysphasia score and peristalsis movement was measured and recorded by manometry before and after (2 months) treating with Heller myotomy. Results: In this study, 23 patients with achalasia (12 females and 11 males) with a mean age of 30±3.5 years (minimum 20, maximum 44 years) who met the inclusion criteria of the study were examined. Results of this study showed that, all the diagnostic criteria that were measured before the treatment was significantly different from after the treatment (P<0.05). The average decline in LES, IRP, Residual Pressure, Free drinking esophagus, and dysphasia score were 23.1 mmHg, 16.24 mmHg, 18,7 mmHg, 18.9 mmHg, and 5.0 unit, respectively. Also the average increase of the peristalsis movement was 8.26±13.7 in 8 patients. Conclusion: Considering the results of Heller myotomy surgery can be as a treatment of choice for achalasia. Free Drinking pressure can be a suitable criteria after treatment for evaluation and prediction of the reducing the dysphasia score after the surgery. PMID:29511469

The efficacy of balloon dilation in achalasia is the result of stretching of the lower esophageal sphincter, not muscular disruption.

PubMed

Borhan-Manesh, F; Kaviani, M J; Taghavi, A R

2016-04-01

Pneumatic dilation (PD) of the lower esophageal sphincter (LES) in achalasia is a major palliative treatment. It is generally believed, although never substantiated, that therapeutic efficacy of ballooning in achalasia is the result of the disruption and tearing of the muscular layers of the LES. To clarify this issue, we investigated the frequency of muscular disruption at the LES, 24 hours after PD, by employing the endoscopic ultrasound (EUS), in a group of 43 consented patients with achalasia. Between July 2009 and March2012, 51 consecutive adult patients with tentative diagnosis of achalasia, some with recurrence of symptoms after an earlier treatment with balloon dilation, were evaluated and underwent PD, using Rigiflex balloon without major adverse effect. Out of the 51 evaluated, 43 eligible and consenting patients who underwent EUS, 24 hours after PD, using Olympus GF-UE 160 echoendoscope and an Aloka Prosound probe at 7.5 MHZ, are the subjects of this study. The EUS in 43 eligible patients revealed an intact LES in 36 (83.7%), small area of muscular disruption in 5 (11.6%) and small hematoma in 2 patients (4.6%). Our data convincingly demonstrate that the clinical effectiveness of balloon dilation in achalasia is not the result of muscular disruption, but of circumferential stretching of the LES. Our findings on the mechanism of action of PD in achalasia could result in modifying the current method of dilation for a safer procedure, by slowing the rate of inflation and allowing the sphincter to slowly stretch itself to the distending balloon. © 2015 The Authors. Diseases of the Esophagus published by Wiley Periodicals, Inc. on behalf of International Society for Diseases of the Esophagus.

Effectiveness of peroral endoscopic myotomy in the treatment of achalasia: a pilot trial in Chinese Han population with a minimum of one-year follow-up.

PubMed

Ling, Ting Sheng; Guo, Hui Min; Yang, Tian; Peng, Chun Yan; Zou, Xiao Ping; Shi, Rui Hua

2014-07-01

To investigate the outcomes of Chinese Han patients who underwent peroral endoscopic myotomy (POEM) for achalasia. Patients undergoing POEM for achalasia at the Affiliated Drum Tower Hospital of Nanjing University Medical School were prospectively enrolled in this study, with a follow-up duration of at least one year. Their outcomes were evaluated by analyzing esophageal manometry, timed barium esophagogram and 36-Item Short Form Health Survey (SF-36), which were performed before surgery, 5 days after surgery and at the last follow-up. Patients' symptom relief was considered the primary outcome. Secondary outcomes included lower esophageal sphincter (LES) pressure, esophageal emptying, patients' quality of life (QoL) and procedure-related complications. Eighty-seven patients were included in the study. Eckardt score after POEM was remarkably lower than the preoperative score (0.4 ± 0.7 vs 7.1 ± 2.1, P = 0.001). The preoperative LES pressure was 32.4 ± 15.3 mmHg, which was decreased to 3.8 ± 3.9 mmHg immediately after surgery. The height of the barium column at 1 min after barium swallow was significantly reduced after treatment (11.7 ± 1.2 cm vs 3.2 ± 1.6 cm, P < 0.001). The patients' QoL was also improved, as indicated by obviously increased physical and mental component summary (PCS and MCS) scores of the SF-36. Complications occurred during POEM included cutaneous emphysema, mucosal injury and pneumothorax. POEM is an effective approach for treating achalasia, which can relieve the symptoms of achalasia by improving esophageal emptying and lowering LES pressure. © 2014 Chinese Medical Association Shanghai Branch, Chinese Society of Gastroenterology, Renji Hospital Affiliated to Shanghai Jiaotong University School of Medicine and Wiley Publishing Asia Pty Ltd.

Treatments for pediatric achalasia: Heller myotomy or pneumatic dilatation?

PubMed

Jung, C; Michaud, L; Mougenot, J-F; Lamblin, M-D; Philippe-Chomette, P; Cargill, G; Bonnevalle, M; Boige, N; Bellaïche, M; Viala, J; Hugot, J-P; Gottrand, F; Cezard, J-P

2010-03-01

The treatment of achalasia consists of reducing distal esophageal obstruction by either Heller myotomy surgery or endoscopic pneumatic dilatation. The aim of the present study was to evaluate the short- and middle-term results of these procedures in children. For technical reasons, children under six years old (n=8) were treated by surgery only, whereas patients over six years old (n=14) were treated by either Heller myotomy or pneumatic dilatation. Of the children aged under six years, 75% were symptom-free at six months and 83% at 24 months of follow-up. Of the patients aged over six years, complete remission was achieved by Heller myotomy in 44.5% vs. 55.5% by pneumatic dilatation after six months, and in 40% vs. 65%, respectively, after 24 months. Both pneumatic dilatation and Heller myotomy showed significant rates of failure. These results suggest that pneumatic dilatation may be considered a primary treatment in children over six years old. Also, where necessary, Heller myotomy and pneumatic dilatation may be used as complementary treatments.

[Effects of peroral endoscopic myotomy on esophageal dynamics in patients with esophageal achalasia].

PubMed

Zhong, Yun-shi; Li, Liang; Zhou, Ping-hong; Xu, Mei-dong; Ren, Zhong; Zhu, Bo-qun; Yao, Li-qing

2012-07-01

To investigate the effects of peroral endoscopic myotomy(POEM) on esophageal dynamics in patients with esophageal achalasia. From September 2011 to November 2011, 20 cases with esophageal achalasia received POEM at the Endoscopic Center in the Zhongshan Hospital of Fudan University. Pre-operation esophageal dynamics of all the patients were evaluated by high resolution manometry(HRM) system and 3 days after operation the test was repeated. Lower esophagus sphincter resting pressure(LESP), 4-second integrated relaxation pressure(4sIRP), lower esophagus sphincter relax rate(LESRR), lower esophagus sphincter length(LESL), and esophageal manometry were analyzed. After POEM, LESP decreased from(29.1±17.0) mm Hg to(14.6±4.9) mm Hg, and decrease rate was 49.8%(P<0.01). However, the decreases in LESRR and LESL were not statistically significant(P>0.05). Esophageal peristaltic contraction was absent in all the 20 patients preoperatively. After POEM, changes in the esophageal contraction were seen in 7 patients, and peristalsis was noticed but was below normal level. There were no significant changes in peristalsis in the remaining 13 patients. POEM can significantly reduce LESP and 4sIRP in patients with achalasia, but can not affect the contraction of the esophagus.

Age and Achalasia: How Does Age Affect Patient Presentation, Hospital Course, and Surgical Outcomes?

PubMed

Downs, Darrell J; Jadick, Giavanna; Swaid, Forat; Ross, Sharona B; Rosemurgy, Alexander S

2017-09-01

Heller myotomy is the "gold-standard" therapy for achalasia, alleviating symptoms by defunctionalizing the lower esophageal sphincter mechanism. Observation has suggested many differences between young and old patients with achalasia, raising the question: is achalasia in younger patients a different disorder than it is in older patients? This study was undertaken to answer this question. With Institutional Review Board approval, 648 patients undergoing laparoscopic Heller myotomy from 1992-2016 were prospectively followed up. Patients self-assessed symptom frequency/severity preoperatively and postoperatively using a Likert scale; 0 (never/not bothersome) to 10 (always/very bothersome). Before myotomy, frequency/severity of many symptoms (e.g., "dysphagia," "chest pain," and "regurgitation") inversely correlated with age (P < 0.01 each). Symptom duration and the number of previous abdominal operations correlated with age, as did intraoperative complications (e.g., gastrotomy), postoperative complications (e.g., atrial fibrillation), and length of stay (P < 0.01 for each). Patients experienced amelioration of all symptoms queried, regardless of age (P < 0.01 each). Age did affect outcome because older patients had less frequent and severe symptoms. Age did not affect improvement of symptoms (e.g., dysphagia) (i.e., differences between preoperative and postoperative scores) (P = 0.88). Age did not influence symptom resolution or patient satisfaction (P = 0.98 and P = 0.15, respectively). The presentation with achalasia, hospital course, and outcome after myotomy are significantly impacted by age, whereas patient improvement after myotomy is constant independent of age. Younger and older patients have different presentations, experiences, and outcomes; these patients seem to have "different disorders", but Heller myotomy provides similar significant amelioration of symptoms independent of age.

Peroral Endoscopic Myotomy Versus Heller Myotomy for Achalasia: Pros and Cons.

PubMed

Tefas, Cristian; Ababneh, Rami; Tanţău, Marcel

2018-01-01

Achalasia is an esophageal motor disorder that has multiple endoscopic and surgical methods of treatment. However, there is no consensus on optimal therapy in patients suffering from this disorder. This review discusses two therapies with similar but technically different concepts, peroral endoscopic myotomy and Heller surgical myotomy. After a brief introduction to the basic problems of achalasia, technical considerations, intra and postprocedural complications are presented and the advantages and disadvantages of the two procedures are discussed, based on the relevant articles in the literature. Heller surgical myotomy and peroral endoscopic myotomy appear to be similar in performance with similar results in terms of gastroesophageal reflux rates. However, with experience being gained in the field of endoscopic myotomy, this procedure seems more advantageous, with similar success rates to those of the established surgical technique, but offering shorter operating times, shorter hospitalizations and, ultimately, lower costs. Celsius.

Idiopathic Ophthalmodynia and Idiopathic Rhinalgia: A Prospective Series of 16 New Cases.

PubMed

Pareja, Juan A; Montojo, Teresa; Guerrero, Ángel L; Álvarez, Mónica; Porta-Etessam, Jesús; Cuadrado, María L

2015-01-01

Idiopathic ophthalmodynia and idiopathic rhinalgia were described a few years ago. These conditions seem specific pain syndromes with a distinctive location in the eye or in the nose. We aimed to present a new prospective series in order to verify the consistency of these syndromes. We performed a descriptive study of all patients referred to our regional neurologic clinics from 2010 to 2014 because of facial pain exclusively felt in the eye or in the nose fulfilling the proposed diagnostic criteria for idiopathic ophthalmodynia and idiopathic rhinalgia. There were 9 patients with idiopathic ophthalmodynia and 7 patients with idiopathic rhinalgia, with a clear female preponderance, and a mean age at onset in the fifth decade. The pain was usually moderate and the temporal pattern was generally chronic. Only one patient reported accompaniments (hypersensitivity to the light and to the flow of air in the symptomatic eye). Preventive treatment with amitriptyline, pregabalin, or gabapentin was partially or totally effective. The clinical features of this new series parallels those of the original description, thus indicating that both idiopathic ophthalmodynia and idiopathic rhinalgia have clear-cut clinical pictures with excellent consistency both inter- and intra-individually. © 2015 American Headache Society.

Diagnosis and Treatment Strategy of Achalasia Subtypes and Esophagogastric Junction Outflow Obstruction Based on High-Resolution Manometry.

PubMed

Ihara, Eikichi; Muta, Kazumasa; Fukaura, Keita; Nakamura, Kazuhiko

2017-01-01

Based on Chicago Classification version 3.0, the disorders of esophagogastric junction outflow obstruction (EGJOO) include achalasia (types I, II and III) and EGJOO. Although no curative treatments are currently available for the treatment of the disorders of EGJOO, medical treatments, endoscopic pneumatic dilation (PD), laparoscopic Heller myotomy (LHM), and per-oral endoscopic myotomy (POEM) are usually the sought-after modes of treatment. Since the etiology and pathogenesis might vary depending on the types of EGJOO disorders, treatment strategies should be considered based on those subtypes. Based on the accumulated evidences, the treatment strategies of our institution are as follows: effects of medical treatments on achalasia are limited. Either PD or LHM/POEM can be considered a first-line in achalasia type I, according to the patient's wish. PD and POEM can be considered first-line in achalasia types II and III, respectively. Conversely, In EGJOO, medical treatments including drugs like acotiamide and/or diltiazem can be tested as a first-line, and PD and POEM will be considered second and third-line treatments, respectively. Key Messages: The classification of subtypes based on high-resolution manometry will help us consider which treatment option can be selected as a first-line treatment depending upon the subtypes of disorders of EGJOO. Acotiamide has the potential to cure patients with EGJOO. © 2016 S. Karger AG, Basel.

Association of High-Resolution Manometry Metrics with the Symptoms of Achalasia and the Symptomatic Outcomes of Peroral Esophageal Myotomy

PubMed Central

Tang, Yurong; Xie, Chen; Wang, Meifeng; Jiang, Liuqin; Shi, Ruihua; Lin, Lin

2015-01-01

Background High-resolution manometry (HRM) has improved the accuracy of manometry in detecting achalasia and has helped distinguish between clinically relevant subtypes. This study investigated whether HRM metrics correlate with the achalasia symptoms and symptomatic outcomes of peroral esophageal myotomy (POEM). Methods Of the 30 patients who were enrolled, 25 were treated with POEM, 12 of who underwent HRM after 3 months. All the patients completed the Eckardt score questionnaires, and those who underwent POEM were followed up for about 6 months. Pearson correlation was used to assess the relationship between the HRM metrics and symptoms and outcomes. Key results The integrated relaxation pressure (IRP) score positively correlated with the total Eckardt score, regurgitation score and weight loss score in all the patients, and with the weight loss score in type I achalasia. In 25 patients (10 patients, type I; 15 patients, type II) who underwent POEM, the total Eckardt scores and individual symptom scores significantly decreased after surgery. Changes in the Eckardt scores were similar between type I and type II. Further, the Eckardt scores and weight loss score changes were positively correlated with baseline IRP. Twelve patients (4 patients, type I; 8 patients, type II) underwent HRM again after POEM. IRP changed significantly after POEM, as did the DEP in type II. The IRP changes after POEM were positively correlated with the Eckardt score changes. Conclusions & Inferences IRP is correlated with the symptoms and outcomes of achalasia patients. Thus, HRM is effective for assessing the severity of achalasia and can predict the efficacy of POEM. PMID:26421919

Association of High-Resolution Manometry Metrics with the Symptoms of Achalasia and the Symptomatic Outcomes of Peroral Esophageal Myotomy.

PubMed

Tang, Yurong; Xie, Chen; Wang, Meifeng; Jiang, Liuqin; Shi, Ruihua; Lin, Lin

2015-01-01

High-resolution manometry (HRM) has improved the accuracy of manometry in detecting achalasia and has helped distinguish between clinically relevant subtypes. This study investigated whether HRM metrics correlate with the achalasia symptoms and symptomatic outcomes of peroral esophageal myotomy (POEM). Of the 30 patients who were enrolled, 25 were treated with POEM, 12 of who underwent HRM after 3 months. All the patients completed the Eckardt score questionnaires, and those who underwent POEM were followed up for about 6 months. Pearson correlation was used to assess the relationship between the HRM metrics and symptoms and outcomes. The integrated relaxation pressure (IRP) score positively correlated with the total Eckardt score, regurgitation score and weight loss score in all the patients, and with the weight loss score in type I achalasia. In 25 patients (10 patients, type I; 15 patients, type II) who underwent POEM, the total Eckardt scores and individual symptom scores significantly decreased after surgery. Changes in the Eckardt scores were similar between type I and type II. Further, the Eckardt scores and weight loss score changes were positively correlated with baseline IRP. Twelve patients (4 patients, type I; 8 patients, type II) underwent HRM again after POEM. IRP changed significantly after POEM, as did the DEP in type II. The IRP changes after POEM were positively correlated with the Eckardt score changes. IRP is correlated with the symptoms and outcomes of achalasia patients. Thus, HRM is effective for assessing the severity of achalasia and can predict the efficacy of POEM.

A single center 26-year experience with treatment of esophageal achalasia: is there an optimal method?

PubMed

Pastor, Aimee C; Mills, Jessica; Marcon, Margaret A; Himidan, Sharifa; Kim, Peter C W

2009-07-01

Treatment modalities for achalasia are evolving and remain controversial. Herein, we report the relative efficacy and outcomes after dilatation or myotomy in children with achalasia. A retrospective analysis of all children treated for achalasia at a tertiary center from 1981 to 2007 was performed (n = 40). Demographics, presenting symptoms, perioperative parameters, and outcomes were analyzed using t tests and chi(2) statistics. Thirty patients were initially treated by esophageal dilatation (ED), whereas 10 were treated by laparoscopic or open Heller myotomy (HM). Both groups were similar with respect to age (10.6 vs 12.4 years; P = .19). There were 18 males and 12 females in the ED group, compared to 5 males and 5 females in the HM group (P = .72). Mean duration of symptoms before diagnosis, including dysphagia, vomiting, food sticking, chest pain, and weight loss, was 15.9 months for ED and 10.7 months for HM (P = .41). Mean time from diagnosis to initial intervention was 76 days in ED vs 86 days in HM (P = .78). Subsequent interventions by myotomy or both dilatation and myotomy were required in 9 (30%) of 30 patients in the ED group and 2 (20%) of 10 patients in the HM group (P = .70). A clear transition from open to laparoscopic approach occurred between 1995 and 2001. Mean operating times were comparable (186.3 vs 156.0 minutes; P = .48). Of 14 laparoscopic myotomies, 11 (79%) had fundoplication, and 2 (18%) of the 11 were converted to open procedure. Intraoperative mucosal perforation rates were similar between open and laparoscopic groups (17% vs 18%). At follow-up, 32% of ED patients vs 43% HM had complete symptom relief (mean follow-up duration, 75.2 months; SD, 196.5). Both dilatation and myotomy are effective immediate treatment of achalasia. A clear transition to and preference for laparoscopic approach has occurred in the treatment of achalasia in children.

[Pneumatic dilation in the treatment of achalasia].

PubMed

Ruiz Cuesta, Patricia; Hervás Molina, Antonio José; Jurado García, Juan; Pleguezuelo Navarro, María; García Sánchez, Valle; Casáis Juanena, Luis L; Gálvez Calderón, Carmen; Naranjo Rodríguez, Antonio

2013-10-01

Pneumatic dilation and surgical myotomy are currently the procedures of choice to treat achalasia. The selection of one or other treatment depends on the experience of each center and patient preferences. To review the experience of pneumatic dilation in patients with achalasia in our center. We included all patients with a clinical, endoscopic and manometric diagnosis compatible with achalasia who underwent pneumatic dilation in a 19-year period. All dilations were routinely performed with a Rigiflex(®) balloon, usually at pressures of 250, 250 and 300mm Hg in three inflations of one minute, each separated by one minute. The success of the dilation was assessed on the basis of the patient's symptoms, the number of sessions, the need for surgery, and the presence of complications. A total of 171 patients were included, 53.2% men and 46.8% women, with a mean age of 51.53±17.78 years (16-87 years), from June 1993 to October 2012. A 35-mm balloon was used in 157 patients, a 30-mm balloon in 9 patients and a 40-mm balloon in 7 patients. A single dilation session was required in 108 patients, two sessions were required in 56 patients, with a mean time between the first and second sessions of 25.23±43.25 months (1-215 months), and 3 sessions were required in 7 patients with a mean time between the second and third sessions of 6.86±5.33 months (1-15 months). Outcome after dilation was successful in 81% of the patients. Of the 140 responders, 121 had complete response (complete disappearance of symptoms without recurrence) and 19 partial response (initial disappearance of symptoms with subsequent reappearance). Surgery (Heller myotomy) was required in 15.8% of the patients. Perforation occurred in 4 of the 171 patients as a complication of the technique; these perforations were satisfactorily resolved, two by conservative treatment and two by surgery. There was no mortality associated with the technique or its complications. In our series, pneumatic dilation had a

Per-Oral Endoscopic Myotomy Versus Laparoscopic Heller Myotomy for Achalasia

PubMed Central

Zhang, Yuan; Wang, Hongjuan; Chen, Xingdong; Liu, Lan; Wang, Hongbo; Liu, Bin; Guo, Jianqiang; Jia, Hongying

2016-01-01

Abstract We aimed to assess the short-term outcomes of per-oral endoscopic myotomy (POEM) compared with laparoscopic Heller myotomy (LHM) for achalasia through a meta-analysis of nonrandomized comparative studies. We searched PubMed, Embase, Medline, Cochrane Library, and Google Scholar for studies that compared POEM and LHM for achalasia and were published between January 1, 2008 and December 31, 2014. The Methodological Index for Nonrandomized Studies (MINORS) was used to evaluate the quality of the studies. Random- and fixed-effects meta-analytical models were used, and between-study heterogeneity was assessed. Four nonrandomized comparative studies that included 317 patients (125 in the POEM group and 192 in the LHM group) met our research criteria and were assessed. There were no differences between the POEM and LHM groups in terms of sex, preoperative Eckhart score, length of myotomy, operation time, length of hospital stay, and complications. The patients in the POEM group were older than those in the LHM group (MD =2.81, 95% CI 0.27–5.35; P = 0.03) with high between-study homogeneity (χ2 = 1.96, df = 2, I2 = 0%; P = 0.38). The patients in the POEM group had a lower Eckardt score after surgery compared with those in the LHM group (MD = −0.30, 95% CI −0.42 to −0.18; P < 0.001) with high between-study homogeneity (χ2 = 0.00, df = 1, I2 = 0%; P = 1.00). The efficacy and safety of POEM appear to be comparable to those of LHM. Multicenter and randomized trials with larger sample size are needed to further compare the efficacy and safety of POEM and LHM for the treatment of achalasia. PMID:26871816

Peroral endoscopic myotomy: An emerging minimally invasive procedure for achalasia

PubMed Central

Vigneswaran, Yalini; Ujiki, Michael B

2015-01-01

Peroral endoscopic myotomy (POEM) is an emerging minimally invasive procedure for the treatment of achalasia. Due to the improvements in endoscopic technology and techniques, this procedure allows for submucosal tunneling to safely endoscopically create a myotomy across the hypertensive lower esophageal sphincter. In the hands of skilled operators and experienced centers, the most common complications of this procedure are related to insufflation and accumulation of gas in the chest and abdominal cavities with relatively low risks of devastating complications such as perforation or delayed bleeding. Several centers worldwide have demonstrated the feasibility of this procedure in not only early achalasia but also other indications such as redo myotomy, sigmoid esophagus and spastic esophagus. Short-term outcomes have showed great clinical efficacy comparable to laparoscopic Heller myotomy (LHM). Concerns related to postoperative gastroesophageal reflux remain, however several groups have demonstrated comparable clinical and objective measures of reflux to LHM. Although long-term outcomes are necessary to better understand durability of the procedure, POEM appears to be a promising new procedure. PMID:26468336

Residual lower esophageal sphincter pressure as a prognostic factor in the pneumatic balloon treatment of achalasia.

PubMed

Park, Jung Ho; Lee, Yong Chan; Lee, Hyuk; Park, Hyojin; Youn, Young Hoon; Park, Hyung Seok; Lee, Tae Hee; Hong, Kyoung Sup

2015-01-01

Pneumatic balloon dilatation (PD) is a mainstay in achalasia treatment. The aim of this study was to identify predictive factors for successful treatment. We retrospectively reviewed 76 patients with a diagnosis of achalasia who underwent PD from June 2010 to May 2013. Clinical symptoms were assessed using Eckardt score and manometry data were analyzed using resting and relaxation pressure (4sIRP) of lower esophageal sphincter (LES) and the distal contractile integral (DCI), which was calculated for 10 s from the start of deglutition between the upper margin of the LES and lower margin of upper esophageal contraction. Patients with achalasia were classified into three groups based on the Chicago classification. Among 76 patients, 52 patients received PD, and the treatment was unsuccessful in 9 patients (6 in class I and 3 in class III). When comparing prognostic factors between successful and unsuccessful treatment groups, the mean value for 4sIRP in the unsuccessful treatment group was significantly lower than that in the successful treatment group (P < 0.05). However, no difference was noticed in resting LES pressure, DCI, age, and sex. Furthermore, a lower mean value of 4sIRP was significantly related to unsuccessful treatment of achalasia (odds ratio, 1.092; 95% confidence interval, 1.001-1.191) even after adjustment for a series of confounding factors. Lower 4sIRP may be a prognostic indicator for poor treatment outcome after PD. © 2014 Journal of Gastroenterology and Hepatology Foundation and Wiley Publishing Asia Pty Ltd.

Efficacy of peroral endoscopic myotomy vs other achalasia treatments in improving esophageal function.

PubMed

Sanaka, Madhusudhan R; Hayat, Umar; Thota, Prashanthi N; Jegadeesan, Ramprasad; Ray, Monica; Gabbard, Scott L; Wadhwa, Neha; Lopez, Rocio; Baker, Mark E; Murthy, Sudish; Raja, Siva

2016-05-28

To assess and compare the esophageal function after peroral endoscopic myotomy (POEM) vs other conventional treatments in achalasia. Chart review of all achalasia patients who underwent POEM, laparoscopic Heller myotomy (LHM) or pneumatic dilation (PD) at our institution between January 2012 and March 2015 was performed. Patient demographics, type of achalasia, prior treatments, pre- and post-treatment timed barium swallow (TBE) and high-resolution esophageal manometry (HREM) findings were compared between the three treatment groups. Patients who had both pre- and 2 mo post-treatment TBE or HREM were included in the final analysis. TBE parameters compared were barium column height, width and volume of barium remaining at 1 and 5 min. HREM parameters compared were basal lower esophageal sphincter (LES) pressures and LES-integrated relaxation pressures (IRP). Data are presented as mean ± SD, median [25(th), 75(th) percentiles] or frequency (percent). Analysis of variance, Kruskal-Wallis test, Pearsons χ(2) test and Fishers Exact tests were used for analysis. A total of 200 achalasia patients were included of which 36 underwent POEM, 22 underwent PD and 142 underwent LHM. POEM patients were older (55.4 ± 16.8 years vs 46.5 ± 15.7 years, P = 0.013) and had higher BMI than LHM (29.1 ± 5.9 kg/m(2) vs 26 ± 5.1 kg/m(2), P = 0.012). More number of patients in POEM and PD groups had undergone prior treatments compared to LHM group (72.2% vs 68.2% vs 44.3% respectively, P = 0.003). At 2 mo post-treatment, all TBE parameters including barium column height, width and volume remaining at 1 and 5 min improved significantly in all three treatment groups (P = 0.01 to P < 0.001) except the column height at 1 min in PD group (P = 0.11) . At 2 mo post-treatment, there was significant improvement in basal LES pressure and LES-IRP in both LHM (40.5 mmHg vs 14.5 mmHg and 24 mmHg vs 7.1 mmHg respectively, P < 0.001) and POEM groups (38.7 mmHg vs 11.4 mmHg and 23.6 mmHg vs 6.6 mm

Efficacy of peroral endoscopic myotomy vs other achalasia treatments in improving esophageal function

PubMed Central

Sanaka, Madhusudhan R; Hayat, Umar; Thota, Prashanthi N; Jegadeesan, Ramprasad; Ray, Monica; Gabbard, Scott L; Wadhwa, Neha; Lopez, Rocio; Baker, Mark E; Murthy, Sudish; Raja, Siva

2016-01-01

AIM: To assess and compare the esophageal function after peroral endoscopic myotomy (POEM) vs other conventional treatments in achalasia. METHODS: Chart review of all achalasia patients who underwent POEM, laparoscopic Heller myotomy (LHM) or pneumatic dilation (PD) at our institution between January 2012 and March 2015 was performed. Patient demographics, type of achalasia, prior treatments, pre- and post-treatment timed barium swallow (TBE) and high-resolution esophageal manometry (HREM) findings were compared between the three treatment groups. Patients who had both pre- and 2 mo post-treatment TBE or HREM were included in the final analysis. TBE parameters compared were barium column height, width and volume of barium remaining at 1 and 5 min. HREM parameters compared were basal lower esophageal sphincter (LES) pressures and LES-integrated relaxation pressures (IRP). Data are presented as mean ± SD, median [25th, 75th percentiles] or frequency (percent). Analysis of variance, Kruskal-Wallis test, Pearsons χ2 test and Fishers Exact tests were used for analysis. RESULTS: A total of 200 achalasia patients were included of which 36 underwent POEM, 22 underwent PD and 142 underwent LHM. POEM patients were older (55.4 ± 16.8 years vs 46.5 ± 15.7 years, P = 0.013) and had higher BMI than LHM (29.1 ± 5.9 kg/m2 vs 26 ± 5.1 kg/m2, P = 0.012). More number of patients in POEM and PD groups had undergone prior treatments compared to LHM group (72.2% vs 68.2% vs 44.3% respectively, P = 0.003). At 2 mo post-treatment, all TBE parameters including barium column height, width and volume remaining at 1 and 5 min improved significantly in all three treatment groups (P = 0.01 to P < 0.001) except the column height at 1 min in PD group (P = 0.11) . At 2 mo post-treatment, there was significant improvement in basal LES pressure and LES-IRP in both LHM (40.5 mmHg vs 14.5 mmHg and 24 mmHg vs 7.1 mmHg respectively, P < 0.001) and POEM groups (38.7 mmHg vs 11.4 mmHg and 23.6 mm

Treatment of idiopathic pulmonary fibrosis with losartan: a pilot project.

PubMed

Couluris, Marisa; Kinder, Brent W; Xu, Ping; Gross-King, Margaret; Krischer, Jeffrey; Panos, Ralph J

2012-10-01

Idiopathic pulmonary fibrosis is a progressive interstitial lung disease with no current effective therapies. Treatment has focused on antifibrotic agents to stop proliferation of fibroblasts and collagen deposition in the lung. We present the first clinical trial data on the use of losartan, an antifibrotic agent, to treat idiopathic pulmonary fibrosis. The primary objective was to evaluate the effect of losartan on progression of idiopathic pulmonary fibrosis measured by the change in percentage of predicted forced vital capacity (%FVC) after 12 months. Secondary outcomes included the change in forced expiratory volume at 1 second, diffusing capacity of carbon monoxide, 6-minute walk test distance, and baseline/transition dyspnea index. Patients with idiopathic pulmonary fibrosis and a baseline %FVC of ≥50 % were treated with losartan 50 mg by mouth daily for 12 months. Pulmonary function testing, 6-minute walk, and breathlessness indices were measured every 3 months. Twenty participants with idiopathic pulmonary fibrosis were enrolled and 17 patients were evaluable for response. Twelve patients had a stable or improved %FVC at study month 12. Similar findings were observed in secondary end-point measures, including 58, 71, and 65 % of patients with stable or improved forced expiratory volume at 1 second, diffusing capacity for carbon monoxide, and 6-minute walk test distance, respectively. No treatment-related adverse events that resulted in early study discontinuation were reported. Losartan stabilized lung function in patients with idiopathic pulmonary fibrosis over 12 months. Losartan is a promising agent for the treatment of idiopathic pulmonary fibrosis and has a low toxicity profile.

Thoracoscopic esophagomyotomy for achalasia: maximum gain, minimal pain.

PubMed

Maher, J W

1997-10-01

Achalasia can be effectively treated by either hydrostatic balloon dilatation or transthoracic modified Heller myotomy. The purpose of this study was to determine whether thoracoscopic methods could be used to achieve surgical results equal to the transthoracic approach with less pain. Twenty-one patients (10 men, 11 women; median age 42 years) had the diagnosis of achalasia confirmed by manometry, radiography, and endoscopy. All had dysphagia; five had weight loss. Median duration of symptoms was 12 months (range: 1 to 360 months). Eleven patients had undergone previous unsuccessful hydrostatic dilatation. Mean esophageal diameter was 5.5 +/- 2.2 cm. All patients underwent attempted modified Heller myotomy through a left thoracoscopic approach. Three patients required conversion to thoracotomy. The myotomy was extended < 1 cm past the squamocolumnar junction. There was one intraoperative perforation and no postoperative complications. All patients were begun on a regular diet on the first postoperative morning. Median length of stay was 2 days, Median follow-up was 22 months (range: 1 to 52 months). Sixteen patients (80%) had excellent relief of their dysphagia. Two patients (10%) had good relief, and two patients had only a fair result, although even they claim to be much improved. Thoracoscopic Heller myotomy reproduces the superior results of open esophagomyotomy with a reduced hospitalization and reduced incisional pain and disability.

Laparoscopic heller myotomy for achalasia cardia-initial experience in a teaching institute.

PubMed

Kaman, Lileswar; Iqbal, Javid; Kochhar, Rakesh; Sinha, Saroj

2013-10-01

Laparoscopic Heller cardiomyotomy and Dor fundoplication is the surgical procedure of choice for esophageal achalasia. The aim of our study was to investigate the clinical outcome and safety of laparoscopic Heller-Dor procedure performed by using Hook electrocautery and as a teaching module for advanced laparoscopic surgery. Between January 2005 and December 2010, 25 consecutive patients with achalasia underwent laparoscopic Heller-Dor operation by a single surgeon. All the patients received upper gastrointestinal series (barium swallow), esophagogastroscopy, and esophageal manometry to exclude esophageal carcinoma and to confirm the diagnosis. All the patients were operated by laparoscopic modified Heller myotomy with Dor fundoplication by using hook electrocautery. Among 25 operated patients, 14 were male and 11 were female with a median age of 43 years (range 18-72 years). The mean operative time was 93.3 min (range 50-50 min), the mean operative blood loss was 90 ml (range 40-200 ml), the median time to oral feeding was 2 days (2-4 days), and the median hospital stay was 4 days (4-7 days). There was no conversion to open surgery. Intraoperative mucosal perforation was encountered in three patients and was repaired in all of them by laparoscopic suture. All the patients had an uneventful recovery without postoperative complication and had excellent clinical response (96 %) during follow-up. Laparoscopic Heller-Dor operation using hook electrocautery is safe, inexpensive, and effective treatment for achalasia which is useful for teaching and training surgical residents in advanced laparoscopic surgery.

Treatment of idiopathic FSGS with adrenocorticotropic hormone gel.

PubMed

Hogan, Jonathan; Bomback, Andrew S; Mehta, Kshama; Canetta, Pietro A; Rao, Maya K; Appel, Gerald B; Radhakrishnan, Jai; Lafayette, Richard A

2013-12-01

Adrenocorticotropic hormone (ACTH) has shown efficacy as primary and secondary therapy for nephrotic syndrome due to membranous nephropathy. The data on using ACTH to treat idiopathic FSGS are limited. This report describes our experience using ACTH for nephrotic syndrome due to idiopathic FSGS in the United States. Twenty-four patients with nephrotic syndrome from idiopathic FSGS were treated with ACTH gel at two academic medical centers between 2009 and 2012, either as part of investigator-initiated pilot studies (n=16) or by prescription for treatment-resistant FSGS (n=8). The primary outcome was remission of proteinuria. The median dose of ACTH was 80 units injected subcutaneously twice weekly. Treatment durations were not uniform. Twenty-two patients had received immunosuppression (mean, 2.2 medications) before ACTH therapy. Six patients had steroid-dependent and 15 had steroid-resistant FSGS. At the time of ACTH initiation, the median serum creatinine (interquartile range) was 2.0 (1.1-2.7) mg/dl, estimated GFR was 36 (28-78) ml/min per 1.73 m(2), and urine protein-to-creatinine ratio was 4595 (2200-8020) mg/g. At the end of ACTH therapy, 7 of 24 patients (29%) experienced remission (n=2 complete remissions, n=5 partial remissions). All remitters had steroid-resistant (n=5) or steroid-dependent (n=2) FSGS. Two responders relapsed during the follow-up period (mean ± SD, 70±31 weeks). Adverse events occurred in 21 of 24 patients, including one episode of new-onset diabetes that resolved after stopping ACTH and two episodes of AKI. Response to ACTH treatment among steroid-resistant or steroid-dependent patients with FSGS is low, but ACTH gel may be a viable treatment option for some patients with resistant nephrotic syndrome due to idiopathic FSGS. Further research is necessary to determine which patients will respond to therapy.

Obesity is a significant susceptibility factor for idiopathic AA amyloidosis.

PubMed

Blank, Norbert; Hegenbart, Ute; Dietrich, Sascha; Brune, Maik; Beimler, Jörg; Röcken, Christoph; Müller-Tidow, Carsten; Lorenz, Hanns-Martin; Schönland, Stefan O

2018-03-01

To investigate obesity as susceptibility factor in patients with idiopathic AA amyloidosis. Clinical, biochemical and genetic data were obtained from 146 patients with AA amyloidosis. Control groups comprised 40 patients with long-standing inflammatory diseases without AA amyloidosis and 56 controls without any inflammatory disease. Patients with AA amyloidosis had either familial Mediterranean fever (FMF) or long-standing rheumatic diseases as underlying inflammatory disease (n = 111, median age 46 years). However, in a significant proportion of patients with AA amyloidosis no primary disease was identified (idiopathic AA; n = 37, median age 60 years). Patients with idiopathic AA amyloidosis were more obese and older than patients with AA amyloidosis secondary to FMF or rheumatic diseases. Serum leptin levels correlated with the body mass index (BMI) in all types of AA amyloidosis. Elevated leptin levels of more than 30 µg/l were detected in 18% of FMF/rheumatic + AA amyloidosis and in 40% of patients with idiopathic AA amyloidosis (p = .018). Finally, the SAA1 polymorphism was confirmed as a susceptibility factor for AA amyloidosis irrespective of the type of the disease. Obesity, age and the SAA1 polymorphism are susceptibility factors for idiopathic AA amyloidosis. Recent advances in treatment of FMF and rheumatic disorders will decrease the incidence of AA amyloidosis due to these diseases. Idiopathic AA, however, might be an emerging problem in the ageing and increasingly obese population.

Idiopathic Hypersomnia: Clinical Features and Response to Treatment

PubMed Central

Ali, Mohsin; Auger, R. Robert; Slocumb, Nancy L.; Morgenthaler, Timothy I.

2009-01-01

Objective: A recent American Academy of Sleep Medicine publication identified a need for research regarding idiopathic hypersomnia. We describe various clinical and polysomnographic features of patients with idiopathic hypersomnia, with an emphasis on response to pharmacotherapy. Methods: A retrospective review of our database initially identified 997 patients, utilizing “idiopathic hypersomnia,” “hypersomnia NOS,” and “primary hypersomnia” as keywords. The charts of eligible patients were examined in detail, and data were abstracted and analyzed. Response to treatment was graded utilizing an internally developed scale. Results: Eighty-five patients were ultimately identified (65% female). Median (interquartile range) ages of onset and diagnosis were 19.6 (15.5) and 33.7 (15.5), respectively. During a median follow-up duration of 2.4 (4.7) years, 65% of patients demonstrated a “complete response” to pharmacotherapy as assessed by the authors' grading schema. Methylphenidate was most commonly used as a first-line agent prior to December 1998, but subsequently, modafinil became the most common first drug. At the last recorded follow-up visit, 92% of patients were on monotherapy, with greater representation of methylphenidate versus modafinil (51% vs. 32%). Among these patients, methylphenidate produced a higher percentage of “complete” or “partial” responses than modafinil, although statistical significance was not reached (38/40 [ 95%] vs 22/25 [88%], respectively, p = 0.291). Conclusions: The majority of patients with idiopathic hypersomnia respond well to treatment. Methylphenidate is chosen more often than modafinil as final monotherapy in the treatment of idiopathic hypersomnia, despite the fact that it is less commonly used initially. Further prospective comparisons of medications should be explored. Citation: Ali M; Auger RR; Slocumb NL; Morgenthaler TI. Idiopathic hypersomnia: clinical features and response to treatment. J Clin Sleep

LATE EVALUATION OF DYSPHAGIA AFTER HELLER ESOPHAGEAL MYOTOMY WITH DOR FUNDOPLICATION FOR ACHALASIA.

PubMed

Câmara, Eduardo Rodrigues Zarco; Madureira, Fernando Athayde Veloso; Madureira, Delta; Salomão, Renato Manganelli; Iglesias, Antonio Carlos Ribeiro Garrido

2017-01-01

All available treatments for achalasia are palliative and aimed to eliminate the flow resistance caused by a hypertensive lower esophageal sphincter. To analyze the positive and negative prognostic factors in the improvement of dysphagia and to evaluate quality of life in patients undergoing surgery to treat esophageal achalasia by comparing findings before, immediately after, and in long follow-up. A total of 84 patients who underwent surgery for achalasia between 2001 and 2014 were retrospectively studied. The evaluation protocol with dysphagia scores compared preoperative, immediate (up to three months) postoperative and late (over one year) postoperative scores to estimate quality of life. The surgical procedure was Heller-Dor in 100% of cases, with 84 cases performed laparoscopically. The percent reduction in pre- and immediate postoperative lower esophageal sphincter pressurewas 60.35% in the success group and 32.49% in the failure group. Regarding the late postoperative period, the mean percent decrease was 60.15% in the success group and 31.4% in the failure group. The mean overall drop in dysphagia score between the pre- and immediate postoperative periods was 7.33 points, which represents a decrease of 81.17%. Reduction greater than 60% percent in lower esophageal sphincter pressurebetween the pre- and postoperative periods suggests that this metric is a predictor of good prognosis for surgical response. Surgical treatment was able to have a good affect in quality of life and drastically changed dysphagia over time.

Clinical remission following endoscopic placement of retrievable, fully covered metal stents in patients with esophageal achalasia.

PubMed

Zeng, Y; Dai, Y-M; Wan, X-J

2014-01-01

Metal stents may represent an alternative therapy in the treatment of achalasia. We therefore evaluated the effectiveness of retrievable, fully covered metal stents in patients with achalasia. Fifty-nine patients with achalasia were treated with retrievable, fully covered metal stents. Symptoms using a global symptom score (0-10), lower esophageal sphincter (LES) resting pressure, LES relaxation, and simultaneous contraction of the esophagus were analyzed before and 1 week and 1 month after intervention. Complications and treatment outcomes were followed up at 6, 12, 18, and 24 months postoperatively. Stent placement was successful, and clinical symptoms resolved (P < 0.01) in all patients. Regurgitation, dysphagia and chest pain improved significantly (all P < 0.01). Therapy improved LES resting pressure (51.4 ± 9.7 mmHg pretherapy vs. 20.9 ± 8.1 mmHg post-therapy), LES relaxation (58.1 ± 17.1% pretherapy vs. 84.5 ± 18.9% post-therapy), and simultaneous contraction of the esophagus (36.1 ± 8.6% pretherapy vs. 69.4 ± 23.1% post-therapy) 1 month after stent placement (all P < 0.01). The cumulative clinical remission rates 6, 12, 18, 24, 30, and 36 months after stent removal were 90.9%, 81.8%, 76.4%, 69.1%, 65.5%, and 49.1%, respectively. All patients tolerated stent placement. Twelve patients (25.5%) complained of substernal pain and five (10.6%) had substernal burning. Stents migrated in four patients (8.5%). Insertion of retrievable, fully covered metal stents is an effective and safe treatment in patients with achalasia. © 2013 Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.

Idiopathic hypersomnia: clinical features and response to treatment.

PubMed

Ali, Mohsin; Auger, R Robert; Slocumb, Nancy L; Morgenthaler, Timothy I

2009-12-15

A recent American Academy of Sleep Medicine publication identified a need for research regarding idiopathic hypersomnia. We describe various clinical and polysomnographic features of patients with idiopathic hypersomnia, with an emphasis on response to pharmacotherapy. A retrospective review of our database initially identified 997 patients, utilizing "idiopathic hypersomnia", "hypersomnia NOS", and "primary hypersomnia" as keywords. The charts of eligible patients were examined in detail, and data were abstracted and analyzed. Response to treatment was graded utilizing an internally developed scale. Eighty-five patients were ultimately identified (65% female). Median (interquartile range) ages of onset and diagnosis were 19.6 (15.5) and 33.7 (15.5), respectively. During a median follow-up duration of 2.4 (4.7) years, 65% of patients demonstrated a "complete response" to pharmacotherapy as assessed by the authors' grading schema. Methylphenidate was most commonly used as a first-line agent prior to December 1998, but subsequently, modafinil became the most common first drug. At the last recorded follow-up visit, 92% of patients were on monotherapy, with greater representation of methylphenidate versus modafinil (51% vs. 32%). Among these patients, methylphenidate produced a higher percentage of "complete" or "partial" responses than modafinil, although statistical significance was not reached (38/40 [95%] vs. 22/25 [88%], respectively, p = 0.291). The majority of patients with idiopathic hypersomnia respond well to treatment. Methylphenidate is chosen more often than modafinil as final monotherapy in the treatment of idiopathic hypersomnia, despite the fact that it is less commonly used initially. Further prospective comparisons of medications should be explored.

Peroral endoscopic myotomy for esophageal achalasia: clinical impact of 28 cases.

PubMed

Minami, Hitomi; Isomoto, Hajime; Yamaguchi, Naoyuki; Matsushima, Kayoko; Akazawa, Yuko; Ohnita, Ken; Takeshima, Fuminao; Inoue, Haruhiro; Nakao, Kazuhiko

2014-01-01

The aim of the present study was to clarify the efficacy of peroral endoscopic myotomy (POEM) for esophageal achalasia. Twenty-eight esophageal achalasia patients who underwent POEM in our institution between August 2010 and October 2012 were enrolled. Under general anesthesia with tracheal intubation, initial incision was made on the anterior wall of the esophagus after submucosal injection. Submucosal tunnel was created and extended below the lower esophageal sphincter (LES) onto the gastric cardia. Subsequently, myotomy was done using triangle tip knife. After confirmation of smooth passage of scope through the esophagogastric junction, the entry was closed. Esophagogram and manometry study was done before and after the procedure. Also, subjective symptom score and Eckardt score were assessed before and 3 months after POEM. POEM was successfully done in all cases without any severe complications such as perforation and mediastinitis.Mean procedure time was 99.1 min (range 61-160) and mean myotomy length was 14.4 cm (range 10-18). Significant improvement was achieved in both esophagogram and endoscopic findings. Mean LES pressure was 71.2 mmHg (35.8-119.0) and 21.0 mmHg (6.7-41.0) before and after the procedure (P < 0.05), respectively. Mean Eckardt score was 6.7 (3-12, median 7) and 0.7 (0-3, median 1) before and 3 months after POEM, respectively (P < 0.05). Symptomatic gastroesophageal reflux disease that was easily controlled by the usual dose of proton pump inhibitor was seen in six cases (21.4%) after the procedure. POEM could be a curative standard treatment of choice for esophageal achalasia. © 2013 The Authors. Digestive Endoscopy © 2013 Japan Gastroenterological Endoscopy Society.

The outcome of laparoscopic Heller myotomy for achalasia is not influenced by the degree of esophageal dilatation.

PubMed

Sweet, Matthew P; Nipomnick, Ian; Gasper, Warren J; Bagatelos, Karen; Ostroff, James W; Fisichella, Piero M; Way, Lawrence W; Patti, Marco G

2008-01-01

In the past, a Heller myotomy was considered to be ineffective in patients with achalasia and a markedly dilated or sigmoid-shaped esophagus. Esophagectomy was the standard treatment. The aims of this study were (a) to evaluate the results of laparoscopic Heller myotomy and Dor fundoplication in patients with achalasia and various degrees of esophageal dilatation; and (b) to assess the role of endoscopic dilatation in patients with postoperative dysphagia. One hundred and thirteen patients with esophageal achalasia were separated into four groups based on the maximal diameter of the esophageal lumen and the shape of the esophagus: group A, diameter<4.0 cm, 46 patients; group B, esophageal diameter 4.0-6.0 cm, 32 patients; group C, diameter>6.0 cm and straight axis, 23 patients; and group D, diameter>6.0 cm and sigmoid-shaped esophagus, 12 patients. All had a laparoscopic Heller myotomy and Dor fundoplication. The median length of follow-up was 45 months (range 7 months to 12.5 years). The postoperative recovery was similar among the four groups. Twenty-three patients (20%) had postoperative dilatations for dysphagia, and five patients (4%) required a second myotomy. Excellent or good results were obtained in 89% of group A and 91% of groups B, C, and D. None required an esophagectomy to maintain clinically adequate swallowing. These data show that (a) a laparoscopic Heller myotomy relieved dysphagia in most patients with achalasia, even when the esophagus was dilated; (b) about 20% of patients required additional treatment; (c) in the end, swallowing was good in 90%.

Laparoendoscopic single-site Heller myotomy with anterior fundoplication for achalasia.

PubMed

Barry, Linda; Ross, Sharona; Dahal, Sujat; Morton, Connor; Okpaleke, Chinyere; Rosas, Melissa; Rosemurgy, Alexander S

2011-06-01

Laparoendoscopic single-site (LESS) surgery is beginning to include advanced laparoscopic operations such as Heller myotomy with anterior fundoplication. However, the efficacy of LESS Heller myotomy has not been established. This study aimed to evaluate the authors' initial experience with LESS Heller myotomy for achalasia. Transumbilical LESS Heller myotomy with concomitant anterior fundoplication for achalasia was undertaken for 66 patients after October 2007. Outcomes including operative time, complications, and length of hospital stay were recorded and compared with those for an earlier contiguous group of 66 consecutive patients undergoing conventional multi-incision laparoscopic Heller myotomy with anterior fundoplication. Symptoms before and after myotomy were scored by the patients using a Likert scale ranging from 0 (never/not severe) to 10 (always/very severe). Data were analyzed using the Mann-Whitney U test, the Wilcoxon matched-pairs test, and Fisher's exact test where appropriate. Patients undergoing LESS Heller myotomy were similar to those undergoing conventional laparoscopic Heller myotomy in gender, age, body mass index (BMI), blood loss, and length of hospital stay. However, the patients undergoing LESS Heller myotomies had operations of significantly longer duration (median, 117 vs. 93 min with the conventional laparoscopic approach) (p<0.003). For 11 patients (16%) undergoing LESS Heller myotomy, additional ports/incisions were required. No patients were converted to "open" operations, and no patients had procedure-specific complications. Symptom reduction was dramatic and satisfying after both LESS and conventional laparoscopic myotomy with fundoplication. The symptom reduction was similar with the two procedures. The LESS approach left no apparent umbilical scar. Heller myotomy with anterior fundoplication effectively treats achalasia. The findings showed LESS Heller myotomy with anterior fundoplication to be feasible, safe, and efficacious

Laparoscopic cardiomyotomy in the treatment of esophageal achalasia.

PubMed

Radovanovic, N; Feussner, H; Stein, H; Siewert, J R

2000-01-01

Tu evaluate the usefulness of the laparoscopic approach as the standard procedure in the surgical treatment of achalasia. Among different competing options of the treatment of esophageal achalasia, extramucosal myotomy of the lower esophageal sphincter--usually combined with anterior fundoplasty--is the most effective but also the most invasive approach. Minimally invasive performance of this operation reduces invasivity and should make the operative treatment a more attractive alternative to other procedures, such as pneumatic dilatation or botox injection. From 1991 till 1997, 27 patients underwent laparoscopic Heller Dor operation (16 males, 11 females, mean age 37 years). Diagnosis was established in all of them by an esophagogram and esophageal manometry. The main symptom was dysphagia in all of the patients. No mortality was observed in this series. There were no conversions to laparotomy. The single intraoperative complication was one case of iatrogenic mucosal laceration. Post operative complications were found in one case of wound infection, and two cases of pneumomedistinum. After a mean follow-up of 33 months (3-77), all patients are without dysphagia and without pathological gastroesophageal reflux. The mean value of residual LES pressure could be reduced from 21 +/- 6.4 mmHg to 7.44 +/- 2.7 mmHg. Laparoscopic cardiomyotomy is at lesat as safe, in terms of morbidity and mortality, as open surgery and similarily effective in alleviating dysphagia. Short hospitalisation and convalascent periods have provided an attractive alternative to repeated dilations for many patients.

Juvenile Idiopathic Arthritis

MedlinePlus

... Is Juvenile Idiopathic Arthritis the same as Juvenile Rheumatoid Arthritis? Yes, Juvenile Idiopathic Arthritis (JIA) is a new ... of chronic inflammatory diseases that affect children. Juvenile Rheumatoid Arthritis (JRA) is the older term that was used ...

Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

MedlinePlus

... Asked Questions Español Condiciones Chinese Conditions Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) En Español Read in Chinese What is idiopathic intracranial hypertension? Idiopathic intracranial hypertension (IIH) is a disorder that ...

Balloon dilation of the esophago-gastric junction affects lower and upper esophageal sphincter function in achalasia.

PubMed

Wauters, L; Van Oudenhove, L; Selleslagh, M; Vanuytsel, T; Boeckxstaens, G; Tack, J; Omari, T; Rommel, N

2014-01-01

Pneumatic dilation of the lower esophageal sphincter (LES) in achalasia has an unappreciated effect on upper esophageal sphincter (UES) function. We studied UES pressure patterns at baseline and alterations in UES parameters resulting from therapy. High-resolution manometry (HRM) tracings from 50 achalasia patients, seen at a tertiary center between January 2009 and July 2011, were reviewed. Manometric parameters studied were (i) LES: resting pressure (restP), 4-second integrated relaxation pressure (IRP4); (ii) UES: resting pressure (restP), minimal relaxation pressure (MRP), peak pressure (PP), relaxation interval (RI), intrabolus pressure (IBP), and deglutitive sphincter resistance (DSR). Mixed models analyses with LES and UES parameters as dependent variables and treatment stage as within-subject independent variable of interest were used. Correlations between treatment-induced changes in LES, UES, and esophageal body (EB) parameters were performed. Pre- and posttreatment HRM tracings were available from 50 patients (mean age 52.7 ± 18.6 years, 29 men). Upper esophageal sphincter parameters MRP (17.9 ± 1.2 vs 15.2 ± 0.9 mmHg; p = 0.02) and IBP (31.5 ± 1.5 vs 27.4 ± 1.2 mmHg; p = 0.009) were significantly reduced after initial balloon dilation and this effect was significant in type II achalasia (p = 0.002 and p = 0.0006). Peak pressure, RI, and DSR were not. The therapeutic effect on LES IRP4 correlated significantly with the change in UES MRP, statistically mediated by the change in EB deglutitive pressure (p = 0.004 and p = 0.0002). We present the first HRM study demonstrating that pneumatic dilation of the LES affects intraesophageal and UES pressures in patients with achalasia. © 2013 John Wiley & Sons Ltd.

Crohn's disease mistaken for long-standing idiopathic mesenteric panniculitis: A case report and management algorithm.

PubMed

Nuzzo, Alexandre; Zappa, Magaly; Cazals-Hatem, Dominique; Bouhnik, Yoram

2016-09-01

Mesenteric panniculitis (MP) is mostly an associated sign of an intra-abdominal or systemic inflammatory primary disease. Nevertheless, etiological and differential diagnosis of idiopathic MP can be challenging when an associate primary cause is not in the foreground. We report here the case of an isolated small bowel Crohn's disease, long time considered as idiopathic MP. This patient presented to our department with a 10-year history of acute abdominal symptoms evolving with flare-up and remission. A diagnosis of idiopathic MP was made based on compatible CT-scan features along with normal laboratory tests and upper and lower bowel endoscopies. As symptoms recurred, a steroid course was proposed which dramatically improved his condition for years. Finally, an explorative laparoscopy was performed because of concern of malignancy when he returned to our unit with a steroid refractory flare-up and weight loss, along with MP nodes growing up to 10 mm. Crohn's disease was eventually diagnosed, based on histopathological middle-gut bowel resection and numerous granulomas in mesenteric nodes without necrosis. This case emphasizes the importance of excluding inflammatory intestinal lesions before making the diagnosis of idiopathic MP (fecal calprotectin, magnetic resonance enterography, wireless capsule endoscopy).

Juvenile idiopathic arthritis.

PubMed

Boros, Christina; Whitehead, Ben

2010-09-01

Juvenile idiopathic arthritis is the most common rheumatic disease in childhood, occurring in approximately 1:500 children. Despite a recent expansion in treatment options and improvement of outcomes, significant morbidity still occurs. This article outlines the clinical manifestations, assessment, detection of complications, treatment options and monitoring requirements, with the aid of guidelines recently published by The Royal Australian College of General Practitioners, which provide practical support for general practitioners to ensure best practice care and to prevent lifelong disability in patients with juvenile idiopathic arthritis. General practice plays an important role in the early detection, initial management and ongoing monitoring of children with juvenile idiopathic arthritis. Early detection involves understanding the classification framework for subtypes of juvenile idiopathic arthritis, and being aware of the clinical manifestations and how to look for them, through history, examination and appropriate investigation. The major extra-articular manifestations of juvenile idiopathic arthritis are uveitis and growth disturbance. Treatment options include nonsteroidal anti-inflammatory drugs, methotrexate, biologic agents, and corticosteroids. Management using a multidisciplinary approach can prevent long term sequelae. Unfortunately, approximately 50% of children will have active disease as adults.

Vocal cord paralysis: What matters between idiopathic and non-idiopathic cases?

PubMed

Özbal Koç, Ayça Eltaf; Türkoğlu, Seda Babakurban; Erol, Ozan; Erbek, Selim

2016-01-01

This study aims to evaluate the demographic and clinical characteristics of patients with idiopathic and non-idiopathic vocal cord paralysis (VCP). This retrospective cohort was performed on data extracted from medical files of 92 consecutive patients (43 males, 49 females; median age 52.1±23.1 years; min. 1 - max. 87) with VCP diagnosed in the otorhinolaryngology department between April 2012 and December 2015. Diagnoses associated with VCP, side of involvement (right, left or bilateral) and previous medical histories were noted and compared between patients with idiopathic and non-idiopathic VCP. Vocal cord paralysis occurred on the left side (n=56, 60.9%), right side (n=28, 30.4%) or bilaterally (n=8, 8.7%). A clinical entity related with VCP was identified in 63 patients (68.5%), while 29 (31.5%) patients had idiopathic VCP. Most common etiologies for VCP were thyroid surgery (n=32, 34.8%), cardiovascular surgery (n=9, 9.8%), lung cancer (n=6, 6.5%) and cardiac anomalies (n=4, 4.3%), respectively. Patients with idiopathic VCP were significantly older (p<0.001), while gender distribution (p=0.121) and side of involvement (p=0.340) did not differ between two groups. Vocal cord paralysis is a relatively common clinical entity with substantial rate of morbidity. Identification of the underlying etiology and awareness on the clinical characteristics are keystones for foreseeing complications and determining the appropriate therapeutic modality.

Redo Heller Myotomy for Achalasia in a Patient with Down Syndrome: a Case Report.

PubMed

Sosa-Stanley, Jessica; Vandendool, KellyAnn; Kiev, Jonathan

2015-01-01

Up to 77% of Down syndrome (DS) patients have associated structural or functional gastrointestinal abnormalities. Functional disturbances, such as processes affecting the enteric nervous system, can often affect the outcome of corrective surgical procedures. Recently, an association between DS and achalasia has been reported. In this report we present a 28-year-old male patient with a history of Down syndrome and achalasia, who presented with recurrent dysphagia, gastroesophageal reflux, and recurrent aspirations. The patient had previously undergone a laparoscopic Heller myotomy with Dor fundoplication. Unfortunately, despite this surgery, he continued to require multiple esophageal dilations, and intraesophageal administration of Botox therapy. Additionally, there were numerous subsequent hospital admissions for recurrent aspiration pneumonia. Evaluation revealed an incomplete myotomy and a revision long Heller myotomy was successfully performed intraabdominally and he is now symptom and aspiration free.

Disease-specific health-related quality of life in patients with esophageal achalasia before and after therapy.

PubMed

Garrigues, V; Ortiz, V; Casanova, C; Bujanda, L; Moreno-Osset, E; Rodríguez-Téllez, M; Montserrat, A; Brotons, A; Fort, E; Ponce, J

2010-07-01

To evaluate disease-specific health-related quality of life (HRQoL) in patients with symptomatic esophageal achalasia before and after therapy. Symptoms and disease-specific HRQoL were evaluated before and 3 months after therapy. Therapy selection, either dilatation or myotomy, depended exclusively on the opinion of the physician on charge of the patient. Symptom severity was graded from 0 to 3, using a scoring system. A disease-specific questionnaire for achalasia developed and validated in Spanish language with 18 items and four subscales (AE-18) was used to evaluate HRQoL. Changes after therapy in HRQoL and its association with clinical improvement were analyzed. Sixty-five patients were prospectively included in eight hospitals in Spain. Of them, 47 were treated with dilatation, and 18 with laparoscopic Heller myotomy. After therapy, AE-18 global and subscales scores improved significantly. Changes in HRQoL were associated with improvement in symptoms. An important improvement in symptoms (>or=50%) was needed to obtain a minimal clinically important improvement (>or=20%) in HRQoL. Disease-specific HRQoL improves in patients with symptomatic achalasia after therapy with dilatation or myotomy. The degree of improvement of HRQoL depends on the degree of improvement of esophageal symptoms.

Idiopathic hypersomnia.

PubMed

Billiard, Michel; Sonka, Karel

2016-10-01

Idiopathic hypersomnia continues to evolve from the concept of "sleep drunkenness" introduced by Bedrich Roth in Prague in 1956 and the description of idiopathic hypersomnia with two forms, polysymptomatic and monosymptomatic, by the same Bedrich Roth in 1976. The diagnostic criteria of idiopathic hypersomnia have varied with the successive revisions of the International classifications of sleep disorders, including the recent 3rd edition. No epidemiological studies have been conducted so far. Disease onset occurs most often during adolescence or young adulthood. A familial background is often present but rigorous studies are still lacking. The key manifestation is hypersomnolence. It is often accompanied by sleep of long duration and debilitating sleep inertia. Polysomnography (PSG) followed by a multiple sleep latency test (MSLT) is mandatory, as well as a 24 h PSG or a 2-wk actigraphy in association with a sleep log to ensure a total 24-h sleep time longer than or equal to 66O minutes, when the mean sleep latency on the MSLT is longer than 8 min. Yet, MSLT is neither sensitive nor specific and the polysomnographic diagnostic criteria require continuous readjustment and biologic markers are still lacking. Idiopathic hypersomnia is most often a chronic condition though spontaneous remission may occur. The condition is disabling, sometimes even more so than narcolepsy type 1 or 2. Based on neurochemical, genetic and immunological analyses as well as on exploration of the homeostatic and circadian processes of sleep, various pathophysiological hypotheses have been proposed. Differential diagnosis involves a number of diseases and it is not yet clear whether idiopathic hypersomnia and narcolepsy type 2 are not the same condition. Until now, the treatment of idiopathic hypersomnia has mirrored that of the sleepiness of narcolepsy type 1 or 2. The first randomized, double-blind, placebo-controlled trials of modafinil have just been published, as well as a double

Laparoscopic Heller myotomy for achalasia: changing trend toward "true" day-case procedure.

PubMed

Agrawal, Sanjay; Super, Paul

2008-12-01

Laparoscopic Heller myotomy is the most effective therapy for achalasia. All case series have reported a minimum length of stay of more than 1 day. "True" day-case laparoscopic Heller myotomy has not been reported, so far. The aim of this study was to review our results with laparoscopic Heller myotomy with respect to the length of stay following the procedure. All patients undergoing laparoscopic Heller myotomy between August 2000 and July 2007 under the care of one surgeon were included in the study. This was performed by incising 6 cm of distal esophageal musculature, extending to 2 cm below the gastroesophageal junction. The myotomy was covered by an anterior fundoplication. All patients were reviewed in the clinic at a median of 6 weeks after surgery and, thereafter, if necessary. Over the 7-year period, 24 consecutive patients with achalasia were treated in this manner. There were 13 women and 11 men, with an age range of 12-73 years. Intraoperative complications included mucosal perforation in 2 patients (sutured immediately) with no postoperative complications or conversion to open surgery. There were no deaths. The average length of stay was 1.9 days (range, 0-4). The last 2 patients were discharged on the same day, and the 5 previous to this were discharged within 23 hours of surgery. There were no adverse outcomes related to early discharge, and there were no readmissions. All patients reported good to excellent results with a relief of dysphagia on follow-up. Three patients (12%) developed recurrent dysphagia after an initial improvement, requiring dilatation only several months later. Based on our own experience, we believe that laparoscopic Heller myotomy with anterior partial fundoplication is safe and achieves a good outcome in the treatment of achalasia. It is well tolerated and can be considered a true day-case procedure.

Treatment of Idiopathic FSGS with Adrenocorticotropic Hormone Gel

PubMed Central

Bomback, Andrew S.; Mehta, Kshama; Canetta, Pietro A.; Rao, Maya K.; Appel, Gerald B.; Radhakrishnan, Jai; Lafayette, Richard A.

2013-01-01

Summary Background and objectives Adrenocorticotropic hormone (ACTH) has shown efficacy as primary and secondary therapy for nephrotic syndrome due to membranous nephropathy. The data on using ACTH to treat idiopathic FSGS are limited. This report describes our experience using ACTH for nephrotic syndrome due to idiopathic FSGS in the United States. Design, setting, participants, & measurements Twenty-four patients with nephrotic syndrome from idiopathic FSGS were treated with ACTH gel at two academic medical centers between 2009 and 2012, either as part of investigator-initiated pilot studies (n=16) or by prescription for treatment-resistant FSGS (n=8). The primary outcome was remission of proteinuria. The median dose of ACTH was 80 units injected subcutaneously twice weekly. Treatment durations were not uniform. Results Twenty-two patients had received immunosuppression (mean, 2.2 medications) before ACTH therapy. Six patients had steroid-dependent and 15 had steroid-resistant FSGS. At the time of ACTH initiation, the median serum creatinine (interquartile range) was 2.0 (1.1–2.7) mg/dl, estimated GFR was 36 (28–78) ml/min per 1.73 m2, and urine protein-to-creatinine ratio was 4595 (2200–8020) mg/g. At the end of ACTH therapy, 7 of 24 patients (29%) experienced remission (n=2 complete remissions, n=5 partial remissions). All remitters had steroid-resistant (n=5) or steroid-dependent (n=2) FSGS. Two responders relapsed during the follow-up period (mean ± SD, 70±31 weeks). Adverse events occurred in 21 of 24 patients, including one episode of new-onset diabetes that resolved after stopping ACTH and two episodes of AKI. Conclusions Response to ACTH treatment among steroid-resistant or steroid-dependent patients with FSGS is low, but ACTH gel may be a viable treatment option for some patients with resistant nephrotic syndrome due to idiopathic FSGS. Further research is necessary to determine which patients will respond to therapy. PMID:24009220

The current state of per oral endoscopic myotomy for achalasia

PubMed Central

Smith, Shane P.

2017-01-01

Achalasia is an acquired neuromuscular disorder that has been treated using a variety of modalities throughout medical history. Recently, the technique of per oral endoscopic myotomy (POEM) was introduced to treat the disease using a truly minimally invasive, natural orifice technique that is rapidly being adopted across the world. This review outlines the development of POEM, the technique itself, and gives a comparison to other procedures, specifically laparoscopic Heller myotomy (LHM). PMID:29078682

Pneumatic balloon dilation in pediatric achalasia: efficacy and factors predicting outcome at a single tertiary pediatric gastroenterology center.

PubMed

Di Nardo, Giovanni; Rossi, Paolo; Oliva, Salvatore; Aloi, Marina; Cozzi, Denis A; Frediani, Simone; Redler, Adriano; Mallardo, Saverio; Ferrari, Federica; Cucchiara, Salvatore

2012-11-01

The use of pneumatic dilation (PD) is well established in adults with achalasia; however, it is less commonly used in children. To evaluate the efficacy of PD in pediatric achalasia and to define predictive factors for its treatment failure. Single-center, prospective cohort study. Academic tertiary referral center. Twenty-four patients with achalasia were enrolled from January 2004 to November 2009 and were followed for a median of 6 years. PD was performed with the patients under general anesthesia. Efficacy and safety of PD. Follow-up was performed by using the Eckardt score, barium swallow contrast studies, and esophageal manometry at baseline; 1, 3, and 6 months after dilation; and every year thereafter. A Cox regression model was used to identify independent predictors of failure after the first PD. The PD success rate was 67%. In 8 patients, the first PD failed, but the parents of one patient refused a second PD and requested surgery. Of the 7 patients who underwent repeated treatment, the second PD failed in 3 (43%). Overall, only 3 of the 24 patients underwent surgery (overall success rate after a maximum of 3 PDs was 87%). Multivariate analysis showed that only older age was independently associated with a higher probability of the procedure success (hazard ratio [HR] 0.66; 95% CI, 0.45-0.97). Small sample size, single-center study. PD is a safe and effective technique in the management of pediatric achalasia. Young age is an independent negative predictive factor for successful clinical outcome. Copyright © 2012 American Society for Gastrointestinal Endoscopy. Published by Mosby, Inc. All rights reserved.

Minimally invasive myotomy for the treatment of esophageal achalasia: evolution of the surgical procedure and the therapeutic algorithm.

PubMed

Bresadola, Vittorio; Feo, Carlo V

2012-04-01

Achalasia is a rare disease of the esophagus, characterized by the absence of peristalsis in the esophageal body and incomplete relaxation of the lower esophageal sphincter, which may be hypertensive. The cause of this disease is unknown; therefore, the aim of the therapy is to improve esophageal emptying by eliminating the outflow resistance caused by the lower esophageal sphincter. This goal can be accomplished either by pneumatic dilatation or surgical myotomy, which are the only long-term effective therapies for achalasia. Historically, pneumatic dilatation was preferred over surgical myotomy because of the morbidity associated with a thoracotomy or a laparotomy. However, with the development of minimally invasive techniques, the surgical approach has gained widespread acceptance among patients and gastroenterologists and, consequently, the role of surgery has changed. The aim of this study was to review the changes occurred in the surgical treatment of achalasia over the last 2 decades; specifically, the development of minimally invasive techniques with the evolution from a thoracoscopic approach without an antireflux procedure to a laparoscopic myotomy with a partial fundoplication, the changes in the length of the myotomy, and the modification of the therapeutic algorithm.

Idiopathic granulomatous mastitis: in search of a therapeutic paradigm.

PubMed

Wilson, Jason P; Massoll, Nicole; Marshall, Julia; Foss, Robin M; Copeland, Edward M; Grobmyer, Stephen R

2007-08-01

Idiopathic granulomatous mastitis, also known as idiopathic granulomatous lobular mastitis, is a benign breast lesion that represents both a diagnostic and therapeutic dilemma. We report two cases of granulomatous mastitis recently evaluated and managed at our institution. To better understand this rare disease, we analyzed treatment outcomes in reported cases of granulomatous mastitis. One hundred sixteen cases were subsequently analyzed. Primary management strategies included observation (n = 9), steroids (n = 29), partial mastectomy (n = 75), and mastectomy (n = 3). Success rates with each treatment were observation, 56 per cent; steroids, 42 per cent; partial mastectomy, 79 per cent; and mastectomy, 100 per cent. Based on this analysis, we propose a clinically useful algorithm for both workup and management of these challenging cases.

Comparison of outcomes of laparoscopic Heller myotomy versus per-oral endoscopic myotomy for management of achalasia

PubMed Central

Burdick, J. S.; Ogola, Gerald O.; Ontiveros, Estrellita

2017-01-01

Achalasia is a rare disorder that has several treatment options. The gold standard of treatment is a surgical myotomy called a laparoscopic Heller myotomy (LHM). More recently, an endoscopic myotomy has become an option as well, called per-oral endoscopic myotomy (POEM). An achalasia registry was queried for patients undergoing either LHM or POEM at Baylor University Medical Center at Dallas. Patient demographics, preoperative and postoperative data points, and Eckardt scores were collected. The patients were further stratified into their follow-up intervals, immediate postoperative and long-term follow-up, to assess surgical success. A subset analysis was done for success of treatment for patients who had redo surgery versus those undergoing the procedure for the first time. There were 12 patients in the POEM group and 11 patients in the LHM group. Both groups demonstrated mean lower esophageal sphincter pressures with failure to relax. Procedure length and hospital length of stay were similar between the two groups. There were three adverse events in each group, but none altered the patient's postoperative clinical course. Eckardt scores, used to assess success of the surgery, were 82% for POEM patients and 66% for LHM patients after 6 months. The outcomes for POEM and LHM in our early experience are similar to those reported in the literature for high-volume centers managing achalasia. PMID:28966450

Comparison of outcomes of laparoscopic Heller myotomy versus per-oral endoscopic myotomy for management of achalasia.

PubMed

Leeds, Steven G; Burdick, J S; Ogola, Gerald O; Ontiveros, Estrellita

2017-10-01

Achalasia is a rare disorder that has several treatment options. The gold standard of treatment is a surgical myotomy called a laparoscopic Heller myotomy (LHM). More recently, an endoscopic myotomy has become an option as well, called per-oral endoscopic myotomy (POEM). An achalasia registry was queried for patients undergoing either LHM or POEM at Baylor University Medical Center at Dallas. Patient demographics, preoperative and postoperative data points, and Eckardt scores were collected. The patients were further stratified into their follow-up intervals, immediate postoperative and long-term follow-up, to assess surgical success. A subset analysis was done for success of treatment for patients who had redo surgery versus those undergoing the procedure for the first time. There were 12 patients in the POEM group and 11 patients in the LHM group. Both groups demonstrated mean lower esophageal sphincter pressures with failure to relax. Procedure length and hospital length of stay were similar between the two groups. There were three adverse events in each group, but none altered the patient's postoperative clinical course. Eckardt scores, used to assess success of the surgery, were 82% for POEM patients and 66% for LHM patients after 6 months. The outcomes for POEM and LHM in our early experience are similar to those reported in the literature for high-volume centers managing achalasia.

Retrograde gastroesophageal intussusception after peroral endoscopic myotomy in a patient with achalasia cardia: A case report.

PubMed

Khan, Samiullah; Su, Shuai; Jiang, Kui; Wang, Bang-Mao

2018-01-01

Retrograde gastroesophageal intussusception (RGEI) is a relatively rare gastrointestinal (GI) disorder in which a portion of the stomach wall invaginates into the esophagus. More recently, peroral endoscopic myotomy (POEM) has emerged as an endoscopic alternative to surgical myotomy for achalasia, and, to the best of our knowledge, our case is the first RGEI after POEM to be reported. A 22-year-old male was presented with a history of vomiting, intractable retching and hematemesis for 3 days. He had a history of achalasia and underwent POEM 3 years ago caused by symptoms of severe dysphagia to solid and liquid. Initially, the patient was diagnosed with a blood-filled esophagus, and the mid esophagus was occluded with a ball-like mass, however, the final diagnosis of RGEI was made by thoracotomy. A therapeutic strategy of conservative treatment and left transthoracic surgery were applied. The surgery and post operative course were uneventful, and he remained asymptomatic 1 year after operation. POEM is a reliable and minimally invasive endoscopic method for esophageal achalasia. Early recognition and severity of RGEI are essential to decrease the unwanted complications. Upper GI series, esophagogastroduodenoscopy and computed tomography scan are helpful for diagnostic purposes of RGEI. Conservative treatment, endoscopic intervention, and surgery are the mainstay of treatments for RGEI. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

Successful peroral endoscopic myotomy performed in Endoscopy Department as a radical, long-term treatment for esophageal achalasia - the Greek experience.

PubMed

Eleftheriadis, Nikolas; Eleftheriadou, Eleni Damianos

2017-01-01

Peroral endoscopic myotomy (POEM) has been considered as a minimal-invasive, innovative technique for long-term treatment of all types of esophageal achalasia and other esophageal motility disorders. We report on 20 consecutive Greek patients with manometrically proved esophageal achalasia (14 patients with type I, 4 with type II, 2 with type III, and 4 with sigmoid esophagus), with an age range of 32-92 years, mean age 59 years, 12 males, successfully treated by POEM from 2013 to 2015. The Eckardt score was 7-12 (type III). Seventeen (85%) POEM procedures were performed in the Endoscopy Department, according to a previous study. During POEM, CO 2 insufflation was mandatory, while the Triangle Tip knife was the only knife used in all procedures. Eckardt score, esophagogram and manometry before and after performing POEM were used for evaluation of our results. The follow-up period was 6 months to 3 years. Selective circular myotomy, 10-13 cm in length, was successfully completed in all patients without severe acute or late complications. Three patients (15%) showed moderate pneumomediastinum and pneumoperitoneum, which was successfully managed by abdominal needle drainage during the procedure. One patient showed mild pleural collection, and in one patient the clip-endoloop technique was used to successfully close the mucosal entry after the completion of POEM. The outcome was uneventful without any further clinical consequences. No other short- or long-term serious complications were reported. Patients were discharged after 1-3 days of hospitalization. Six months to 3 years after the POEM procedure, all patients were alive; the majority (90%) had complete clinical improvement, while two patients with sigmoid-type achalasia showed moderate-to-significant clinical improvement. Erosive esophagitis was reported in 15%. Our results are in accordance with international data, and proved the safety and efficacy of the POEM technique for radical long-term treatment of all

Predictors of short term treatment outcome in patients with achalasia following endoscopic or surgical therapy.

PubMed

Gheorghe, Cristian; Bancila, Ion; Tutuian, Radu; Iacob, Razvan; Tomulescu, Victor

2012-01-01

Pneumatic balloon dilation and surgical myotomy are the most effective treatments for achalasia. While there is controversy which method is best, the aim of the current study was to identify predictors of symptom recurrence after endoscopic or surgical therapy. Patients undergoing pneumatic balloon dilatation (30mm) or laparoscopic Heller myotomy with Dor fundoplication were included in the study. Analyzed parameters include total symptom score (sum of 0-5 point intensity for dysphagia, regurgitation and chest pain), width and height of esophageal column at 2 and 5 minutes after oral barium ingestion, lower esophageal sphincter (LES) length, resting (LESP) and residual pressure (LESRP) before and 3 months after intervention. Patients with symptoms score <3 at the 3-month follow-up visit were considered asymptomatic. Twenty-one patients underwent pneumatic dilation (14) or laparoscopic myotomy (7). Total symptom score improved (p<0.01) from pre- (7.2±2.7) to post-intervention (1.7±2.6). Eleven (85.8%) patients in the endoscopic group vs. 7 (100%) patients in the surgical group were symptom-free 3 months after intervention. Therapies improved LESP (24.4±8.2mmHg pre- vs. 15.4±10.3mmHg post-therapy; p=0.003) and mean LESRP (7.9±4.3mmHg pre- vs. 5.3±6.7mmHg post-therapy; p=0.03). Univariate linear regression analysis identified barium contrast column width >5cm at 2 minutes (p=0.04), LES length <2cm (p=0.003) and LESRP >10mmHg (p=0.02) as predictors for persistent symptoms. While >85% of achalasia patients responded well to 30mm pneumatic balloon dilation, patients with elevated LES pressure, short LES and wide esophagus should be considered as primary surgical candidates.

Peroral endoscopic myotomy (POEM) vs laparoscopic Heller myotomy (LHM) for the treatment of Type III achalasia in 75 patients: a multicenter comparative study.

PubMed

Kumbhari, Vivek; Tieu, Alan H; Onimaru, Manabu; El Zein, Mohammad H; Teitelbaum, Ezra N; Ujiki, Michael B; Gitelis, Matthew E; Modayil, Rani J; Hungness, Eric S; Stavropoulos, Stavros N; Shiwaku, Hiro; Kunda, Rastislav; Chiu, Philip; Saxena, Payal; Messallam, Ahmed A; Inoue, Haruhiro; Khashab, Mouen A

2015-06-01

Type III achalasia is characterized by rapidly propagating pressurization attributable to spastic contractions. Although laparoscopic Heller myotomy (LHM) is the current gold standard management for type III achalasia, peroral endoscopic myotomy (POEM) is conceivably superior because it allows for a longer myotomy. Our aims were to compare the efficacy and safety of POEM with LHM for type III achalasia patients. A retrospective study of 49 patients who underwent POEM for type III achalasia across eight centers were compared to 26 patients who underwent LHM at a single institution. Procedural data were abstracted and pre- and post-procedural symptoms were recorded. Clinical response was defined by improvement of symptoms and decrease in Eckardt stage to ≤ 1. Secondary outcomes included length of myotomy, procedure duration, length of hospital stay, and rate of adverse events. Clinical response was significantly more frequent in the POEM cohort (98.0 % vs 80.8 %; P = 0.01). POEM patients had significantly shorter mean procedure time than LHM patients (102 min vs 264 min; P < 0.01) despite longer length of myotomy (16 cm vs 8 cm; P < 0.01). There was no significant difference between POEM and LHM in the length of hospital stay (3.3 days vs 3.2 days; P = 0.68), respectively. Rate of adverse events was significantly less in the POEM group (6 % vs 27 %; P < 0.01). POEM allows for a longer myotomy than LHM, which may result in improved clinical outcomes. POEM appears to be an effective and safe alternative to LHM in patients with type III achalasia.

Idiopathic Inflammatory Myopathies

PubMed Central

Dimachkie, Mazen M.; Barohn, Richard J.

2012-01-01

The idiopathic inflammatory myopathies are a group of rare disorders including polymyositis (PM), dermatomyositis (DM), and autoimmune necrotizing myopathies (NMs). The idiopathic inflammatory myopathies share many similarities. They present acutely, subacutely, or chronically with marked proximal and symmetric muscle weakness, except for associated distal and asymmetric weakness in inclusion body myositis. The idiopathic inflammatory myopathies also share a variable degree of creatine kinase (CK) elevation and a nonspecifically abnormal electromyogram demonstrating an irritative myopathy. The muscle pathology demonstrates inflammatory exudates of variable distribution within the muscle fascicle. Despite these similarities, the idiopathic inflammatory myopathies are a heterogeneous group. The overlap syndrome (OS) refers to the association of PM, DM, or NM with connective tissue disease, such as scleroderma or systemic lupus erythematosus. In addition to elevated antinuclear antibodies (ANA), patients with OS may be weaker in the proximal arms than the legs mimicking the pattern seen in some muscular dystrophies. In this review, we focus on DM, PM, and NM and examine current and promising therapies. PMID:23117947

Microcatheter Embolization of Intractable Idiopathic Epistaxis

DOE Office of Scientific and Technical Information (OSTI.GOV)

Leppaenen, Martti; Seppaenen, Seppo; Laranne, Jussi

1999-11-15

Purpose: To assess the efficacy and safety of microcatheter embolization in the treatment of intractable idiopathic epistaxis. Methods: Thirty-seven patients underwent microcatheter embolization in 1991-1998. We evaluated retrospectively the technical and clinical outcome, the number of complications, the duration of embolization in each case, and the number of blood transfusions needed. All embolizations were done with biplane digital subtraction angiography (DSA) equipment. The procedure was carried out under local anesthesia using transfemoral catheterization, except in one case where the translumbar route was used. Tracker 18 or 10 microcatheters were advanced as far as possible to the distal branches of themore » sphenopalatine artery. Polyvinyl alcohol (PVA) particles were used for embolization in most cases, while platinum coils or a combination of these two materials were occasionally used. The primary outcome was always assessed immediately by angiography. Follow-up data were obtained from patient records, by interviewing patients on the telephone or by postal questionnaires when necessary. The mean follow-up time was 21 months. Results: The embolization was technically successful in all 37 cases. A curative outcome was achieved in 33 cases (89%). The mean duration of the procedure was 110 min. Four patients (8%) had mild transient complications, but no severe or persistent complications were encountered. Twenty-three patients needed a blood transfusion. Slight rebleeding occurred in three patients during the follow-up; all responded to conservative treatment. One patient suffered two episodes of rebleeding within 2 months after primary embolization. Re-embolizations successfully stopped the bleeding. Conclusion: Embolization is the primary invasive modality for treating intractable idiopathic epistaxis. It proved both safe and effective over a relatively long follow-up.« less

Treatment of esophageal achalasia with Heller myotomy: retrospective evaluation of patient satisfaction and disease-specific quality of life

PubMed Central

Dang, Yen; Mercer, C. Dale

2006-01-01

Background Prospective randomized studies have suggested that surgery palliates esophageal achalasia more effectively than pneumatic dilatation, but for some dilatation is still the procedure of choice for initial treatment. We decided to compare achalasia symptoms before and after Heller myotomy by means of postoperative questionnaires. Methods The study included 22 patients who underwent Heller myotomy for achalasia at the Hotel Dieu Hospital, Queen's University, Kingston, Ont., since July 1990; 5 of them required repeat myotomy for symptom recurrence, for a total of 9 open and 18 laparoscopic procedures. Median follow-up was 43 (range 6–109) months. Preoperative and postoperative data regarding dysphagia, regurgitation, chest pain and overall patient satisfaction were gathered. Symptom scores were calculated by adding severity (0 = none, 2 = mild, 4 = moderate, 6 = severe) to frequency (0 = never, 1 = occasionally, 2 = once a month, 3 = every week, 4 = twice a week, 5 = daily). Patients having a repeat procedure were instructed to evaluate symptoms with respect to their initial myotomy. Results Seventeen (77%) patients were successfully contacted, 4 of them had subsequent repeat myotomy for symptom recurrence. Initially, overall symptom scores decreased for all but 1 patient, with mean preoperative and postoperative values of 23.1 and 7.3 respectively (p < 0.001). The patient in whom symptoms did not improve is a candidate for a repeat procedure. Repeat myotomy was performed after a median of 38 (range 23–75) months, corresponding to an overall 3-year positive outcome in 13 (76%) of the 17 patients. Fifteen (88%) patients considered their myotomies a success and 16 (94%) would choose to have this procedure again given the outcome. Conclusion Heller myotomy appears to be effective in alleviating the symptoms of achalasia. Repeat myotomy is occasionally required. PMID:16948885

Effects of laparoscopic myotomy on the esophageal motility pattern of esophageal achalasia as measured by high-resolution manometry.

PubMed

Salvador, Renato; Savarino, Edoardo; Pesenti, Elisa; Spadotto, Lorenzo; Voltarel, Guerrino; Capovilla, Giovanni; Cavallin, Francesco; Nicoletti, Loredana; Valmasoni, Michele; Ruol, Alberto; Merigliano, Stefano; Costantini, Mario

2017-09-01

Esophageal achalasia can be classified on the grounds of three distinct manometric patterns that correlate well with final outcome after laparoscopic Heller-Dor myotomy (LHM). No analytical data are available, however, on the postoperative picture and its possible correlation with final outcome. The aims of this study were: (a) to investigate whether manometric patterns change after LHM for achalasia; (b) to ascertain whether postoperative patterns and/or changes can predict final outcome; and (c) to test the hypothesis that the three known patterns represent different stages in the evolution of the disease. During the study period, we prospectively enlisted 206 consecutive achalasia patients who were assessed using high-resolution manometry (HRM) before undergoing LHM. Symptoms were scored using a detailed questionnaire. Barium swallow, endoscopy and HRM were performed, before and again 6 months after surgery. Preoperative HRM revealed the three known patterns with statistically different esophageal diameters (pattern I having the largest), and patients with pattern I had the highest symptom scores. The surgical treatment failed in 10 cases (4.9%). The only predictor of final outcome was the preoperative manometric pattern (p = 0.01). All patients with pattern I preoperatively had the same pattern afterward, whereas nearly 50% of patients with pattern III before LHM had patterns I or II after surgery. There were no cases showing the opposite trend. Neither a change of manometric pattern after surgery nor a patient's postoperative pattern was a predictor of final outcome, whereas preoperative pattern confirmed its prognostic significance. The three manometric patterns distinguishable in achalasia may represent different stages in the disease's evolution, pattern III and pattern I coinciding with the early and final stages of the disease, respectively.

Minimally invasive management of achalasia cardia: results from a single center study.

PubMed

Palanivelu, C; Maheshkumar, G S; Jani, Kalpesh; Parthasarthi, R; Sendhilkumar, K; Rangarajan, M

2007-01-01

Since the performance of the first laparoscopic cardiomyotomy for achalasia cardia in 1991, the popularity of the minimally invasive approach for this troublesome disease has been growing. We present our experience of 226 patients who underwent laparoscopic cardiomyotomy and discuss the relevant issues. A retrospective analysis was carried out of 226 patients who have undergone laparoscopic cardiomyotomy since 1993. The preoperative workup, surgical technique, and postoperative management are described. Patients included 146 males and 80 females; average age was 36.4 years (range, 6 to 85). Mean duration of symptoms was 1.4 years. Nearly half of the patients (112) had undergone prior pneumatic dilatation. In 20 patients, myotomy alone was done, 44 patients had a Dor's fundoplication, and 162 had Toupet's fundoplication. The average operating time was 96 minutes. Mean postoperative hospital stay was 2.2 days. Dysphagia was eliminated in 88.9% of the patients with an overall morbidity of 4.4% and nil mortality over a mean follow-up of 4.3 years. Laparoscopic cardiomyotomy with Toupet's fundoplication is a safe and effective treatment of achalasia cardia. Dor's fundoplication is done selectively, especially when suspicion is present of mucosal injury.

Minimally Invasive Management of Achalasia Cardia: Results From a Single Center Study

PubMed Central

Palanivelu, C.; Maheshkumar, G. S.; Parthasarthi, R.; Sendhilkumar, K.; Rangarajan, M.

2007-01-01

Background: Since the performance of the first laparoscopic cardiomyotomy for achalasia cardia in 1991, the popularity of the minimally invasive approach for this troublesome disease has been growing. We present our experience of 226 patients who underwent laparoscopic cardiomyotomy and discuss the relevant issues. Methods: A retrospective analysis was carried out of 226 patients who have undergone laparoscopic cardiomyotomy since 1993. The preoperative workup, surgical technique, and postoperative management are described. Results: Patients included 146 males and 80 females; average age was 36.4 years (range, 6 to 85). Mean duration of symptoms was 1.4 years. Nearly half of the patients (112) had undergone prior pneumatic dilatation. In 20 patients, myotomy alone was done, 44 patients had a Dor's fundoplication, and 162 had Toupet's fundoplication. The average operating time was 96 minutes. Mean postoperative hospital stay was 2.2 days. Dysphagia was eliminated in 88.9% of the patients with an overall morbidity of 4.4% and nil mortality over a mean follow-up of 4.3 years. Conclusion: Laparoscopic cardiomyotomy with Toupet's fundoplication is a safe and effective treatment of achalasia cardia. Dor's fundoplication is done selectively, especially when suspicion is present of mucosal injury. PMID:17931518

SVC obstruction and stridor relieved by nasogastric tube insertion

PubMed Central

Molena, Emma J.; Krishnamoorthy, Ashwin; Praveen, Coimbatore

2016-01-01

Achalasia is an idiopathic motility disorder of the oesophagus of increasing incidence. It is characterized by aperistalsis of the lower oesophagus and failure of relaxation of the lower oesophageal sphincter. Patients classically present with chronic symptoms of dysphagia, chest pain, weight loss and regurgitation, and they commonly suffer pulmonary complications such as recurrent microaspiration of static, retained food contents of the upper oesophagus. However, it has also been described, uncommonly, to present with megaoesophagus and secondary tracheal compression. We present a case of megaoesophagus secondary to achalasia which presented with stridor and signs of acute superior vena caval obstruction. PMID:26933001

Esophageal stasis in achalasia patients without symptoms after treatment does not predict symptom recurrence.

PubMed

van Hoeij, F B; Smout, A J P M; Bredenoord, A J

2017-08-01

After achalasia treatment, a subset of patients has poor esophageal emptying without having symptoms. There is no consensus on whether to pre-emptively treat these patients. We hypothesized that, if left untreated, these patients will experience earlier symptom recurrence than patients without stasis. 99 treated achalasia patients who were in clinical remission (Eckardt ≤3) at 3 months after treatment were divided into two groups, based on presence or absence of esophageal stasis on a timed barium esophagogram performed after 3 months. Two years after initial treatment, patients with stasis after treatment still had a wider esophagus (3 cm; IQR: 2.2-3.8) and more stasis (3.5 cm; IQR: 1.9-5.6) than patients without stasis (1.8 cm wide and 0 cm stasis; both P<.001). In patients with stasis, the esophageal diameter had increased from 2.5 to 3.0 cm within 2 years of follow-up. The symptoms, need for and time to retreatment were comparable between the two groups. Quality of life and reflux symptoms were also comparable between the two groups. Although patients with stasis initially had a wider esophagus and 2 years after treatment also had a higher degree of stasis and a more dilated esophagus, compared to patients without stasis, they did not have a higher chance of requiring retreatment. We conclude that stasis in symptom-free achalasia patients after treatment does not predict treatment failure within 2 years and can therefore not serve as a sole reason for retreatment. © 2017 John Wiley & Sons Ltd.

Outcome of Peroral Endoscopic Myotomy (POEM) for Treating Achalasia Compared With Laparoscopic Heller Myotomy (LHM).

PubMed

Peng, Lijun; Tian, Shuni; Du, Chao; Yuan, Ziying; Guo, Mingxiao; Lu, Lin

2017-02-01

Peroral endoscopic myotomy (POEM) is an emerging endoscopic treatment for achalasia and the long-term efficacy of POEM remains to be evaluated. This study compared the outcomes of POEM with that of the standard laparoscopic Heller myotomy (LHM) for achalasia. Achalasia patients treated by POEM or LHM were retrospectively analyzed, with a minimum postoperative follow-up of 3 years. Perioperative outcomes and long-term outcomes including treatment success (Eckardt score ≤3), occurrence of gastroesophageal reflux disease (GERD) (GerdQ score ≥9) and quality of life (36-item short form) were compared. Thirteen patients who underwent POEM were compared with 18 patients who received LHM. These patients were similar in age, sex, symptoms duration, Eckardt score, and previous therapy (all P>0.05). Mean myotomy lengths were similar (P=0.73). Operation time was shorter in the POEM group (P=0.001). One patient (7.7%) developed pneumothorax after POEM and 1 patient (5.6%) experienced postoperative infection after LHM (P=1.00). Treatment success was achieved in 83.3% (9/12) of POEM patients and 80.0% (12/15) of LHM patients (P=1.00). Both POEM and LHM significantly reduced Eckardt score (both P=0.00). GERD rate was similar (8.3% vs. 6.7%, P=1.00). There was no difference in all aspects of quality of life between the 2 groups. Long-term outcomes indicate that POEM is an effective treatment that is comparable with LHM. More data of randomized trials comparing POEM with LHM will enrich the existing evidence.

Peroral endoscopic myotomy (POEM) vs laparoscopic Heller myotomy (LHM) for the treatment of Type III achalasia in 75 patients: a multicenter comparative study

PubMed Central

Kumbhari, Vivek; Tieu, Alan H; Onimaru, Manabu; El Zein, Mohammad H.; Teitelbaum, Ezra N.; Ujiki, Michael B.; Gitelis, Matthew E.; Modayil, Rani J.; Hungness, Eric S.; Stavropoulos, Stavros N.; Shiwaku, Hiro; Kunda, Rastislav; Chiu, Philip; Saxena, Payal; Messallam, Ahmed A.; Inoue, Haruhiro; Khashab, Mouen A.

2015-01-01

Background and study aims: Type III achalasia is characterized by rapidly propagating pressurization attributable to spastic contractions. Although laparoscopic Heller myotomy (LHM) is the current gold standard management for type III achalasia, peroral endoscopic myotomy (POEM) is conceivably superior because it allows for a longer myotomy. Our aims were to compare the efficacy and safety of POEM with LHM for type III achalasia patients. Patients and methods: A retrospective study of 49 patients who underwent POEM for type III achalasia across eight centers were compared to 26 patients who underwent LHM at a single institution. Procedural data were abstracted and pre- and post-procedural symptoms were recorded. Clinical response was defined by improvement of symptoms and decrease in Eckardt stage to ≤ 1. Secondary outcomes included length of myotomy, procedure duration, length of hospital stay, and rate of adverse events. Results: Clinical response was significantly more frequent in the POEM cohort (98.0 % vs 80.8 %; P = 0.01). POEM patients had significantly shorter mean procedure time than LHM patients (102 min vs 264 min; P < 0.01) despite longer length of myotomy (16 cm vs 8 cm; P < 0.01). There was no significant difference between POEM and LHM in the length of hospital stay (3.3 days vs 3.2 days; P = 0.68), respectively. Rate of adverse events was significantly less in the POEM group (6 % vs 27 %; P < 0.01). Conclusions: POEM allows for a longer myotomy than LHM, which may result in improved clinical outcomes. POEM appears to be an effective and safe alternative to LHM in patients with type III achalasia. PMID:26171430

Endoscopic stent suture fixation for prevention of esophageal stent migration during prolonged dilatation for achalasia treatment.

PubMed

Rieder, E; Asari, R; Paireder, M; Lenglinger, J; Schoppmann, S F

2017-04-01

The aim of this study is to compare endoscopic stent suture fixation with endoscopic clip attachment or the use of partially covered stents (PCS) regarding their capability to prevent stent migration during prolonged dilatation in achalasia. Large-diameter self-expanding metal stents (30 mm × 80 mm) were placed across the gastroesophageal junction in 11 patients with achalasia. Stent removal was scheduled after 4 to 7 days. To prevent stent dislocation, endoscopic clip attachment, endoscopic stent suture fixation, or PCS were used. The Eckardt score was evaluated before and 6 months after prolonged dilatation. After endoscopic stent suture fixation, no (0/4) sutured stent migrated. When endoscopic clips were used, 80% (4/5) clipped stents migrated (p = 0.02). Of two PCS (n = 2), one migrated and one became embedded leading to difficult stent removal. Technical adverse events were not seen in endoscopic stent suture fixation but were significantly correlated with the use of clips or PCS (r = 0.828, p = 0.02). Overall, 72% of patients were in remission regarding their achalasia symptoms 6 months after prolonged dilatation. Endoscopic suture fixation of esophageal stents but not clip attachment appears to be the best method of preventing early migration of esophageal stents placed at difficult locations such as at the naive gastroesophageal junction. © The Authors 2017. Published by Oxford University Press on behalf of International Society for Diseases of the Esophagus. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Comparison of monocyte gene expression among patients with neurocysticercosis-associated epilepsy, Idiopathic Epilepsy and idiopathic headaches in India.

PubMed

Prabhakaran, Vasudevan; Drevets, Douglas A; Ramajayam, Govindan; Manoj, Josephine J; Anderson, Michael P; Hanas, Jay S; Rajshekhar, Vedantam; Oommen, Anna; Carabin, Hélène

2017-06-01

Neurocysticercosis (NCC), a neglected tropical disease, inflicts substantial health and economic costs on people living in endemic areas such as India. Nevertheless, accurate diagnosis using brain imaging remains poorly accessible and too costly in endemic countries. The goal of this study was to test if blood monocyte gene expression could distinguish patients with NCC-associated epilepsy, from NCC-negative imaging lesion-free patients presenting with idiopathic epilepsy or idiopathic headaches. Patients aged 18 to 51 were recruited from the Department of Neurological Sciences, Christian Medical College and Hospital, Vellore, India, between January 2013 and October 2014. mRNA from CD14+ blood monocytes was isolated from 76 patients with NCC, 10 Recovered NCC (RNCC), 29 idiopathic epilepsy and 17 idiopathic headaches patients. A preliminary microarray analysis was performed on six NCC, six idiopathic epilepsy and four idiopathic headaches patients to identify genes differentially expressed in NCC-associated epilepsy compared with other groups. This analysis identified 1411 upregulated and 733 downregulated genes in patients with NCC compared to Idiopathic Epilepsy. Fifteen genes up-regulated in NCC patients compared with other groups were selected based on possible relevance to NCC, and analyzed by qPCR in all patients' samples. Differential gene expression among patients was assessed using linear regression models. qPCR analysis of 15 selected genes showed generally higher gene expression among NCC patients, followed by RNCC, idiopathic headaches and Idiopathic Epilepsy. Gene expression was also generally higher among NCC patients with single cyst granulomas, followed by mixed lesions and single calcifications. Expression of certain genes in blood monocytes can distinguish patients with NCC-related epilepsy from patients with active Idiopathic Epilepsy and idiopathic headaches. These findings are significant because they may lead to the development of new tools to

CONTRAIS: CONservative TReatment for Adolescent Idiopathic Scoliosis: a randomised controlled trial protocol.

PubMed

Abbott, Allan; Möller, Hans; Gerdhem, Paul

2013-09-05

Idiopathic scoliosis is a three-dimensional structural deformity of the spine that occurs in children and adolescents. Recent reviews on bracing and exercise treatment have provided some evidence for effect of these interventions. The purpose of this study is to improve the evidence base regarding the effectiveness of conservative treatments for preventing curve progression in idiopathic scoliosis. Previously untreated girls and boys with idiopathic scoliosis, 9 to 17 years of age with at least one year of remaining growth and a curve Cobb angle of 25-40 degrees will be included. A total of 135 participants will be randomly allocated in groups of 45 patients each to receive one of the three interventions. All three groups will receive a physical activity prescription according to the World Health Organisation recommendations. One group will additionally wear a hyper-corrective night-time brace. One group will additionally perform postural scoliosis-specific exercises. Participation in the study will last until the curve has progressed, or until cessation of skeletal growth. OUTCOME variables will be measured every 6 months. The primary outcome variable, failure of treatment, is defined as progression of the Cobb angle more than 6 degrees, compared to the primary x-ray, seen on two consecutive spinal standing x-rays taken with 6 months interval. Secondary outcome measures include the SRS-22r and EQ5D-Y quality of life questionnaires, the International Physical Activity Questionnaire (IPAQ) short form, and Cobb angle at end of the study. This trial will evaluate which of the tested conservative treatment approaches that is the most effective for patients with adolescent idiopathic scoliosis. NCT01761305.

Laparoscopic Heller Myotomy Versus Peroral Endoscopic Myotomy (POEM) for Achalasia: A Systematic Review and Meta-analysis.

PubMed

Schlottmann, Francisco; Luckett, Daniel J; Fine, Jason; Shaheen, Nicholas J; Patti, Marco G

2018-03-01

To compare the outcome of per oral endoscopic myotomy (POEM) and laparoscopic Heller myotomy (LHM) for the treatment of esophageal achalasia. Over the last 2 decades, LHM has become the primary form of treatment in many centers. However, since the first description of POEM in 2010, this technique has widely disseminated, despite the absence of long-term results and randomized trials. A systematic Medline literature search of articles on LHM and POEM for the treatment of achalasia was performed. The main outcomes measured were improvement of dysphagia and posttreatment gastroesophageal reflux disease (GERD). Linear regression was used to model the effect of each procedure on the different outcomes. Fifty-three studies reported data on LHM (5834 patients), and 21 articles examined POEM (1958 patients). Mean follow-up was significantly longer for studies of LHM (41.5 vs. 16.2 mo, P < 0.0001). Predicted probabilities for improvement in dysphagia at 12 months were 93.5% for POEM and 91.0% for LHM (P = 0.01), and at 24 months were 92.7% for POEM and 90.0% for LHM (P = 0.01). Patients undergoing POEM were more likely to develop GERD symptoms (OR 1.69, 95% CI 1.33-2.14, P < 0.0001), GERD evidenced by erosive esophagitis (OR 9.31, 95% CI 4.71-18.85, P < 0.0001), and GERD evidenced by pH monitoring (OR 4.30, 95% CI 2.96-6.27, P < 0.0001). On average, length of hospital stay was 1.03 days longer after POEM (P = 0.04). Short-term results show that POEM is more effective than LHM in relieving dysphagia, but it is associated with a very high incidence of pathologic reflux.

Clinical, endoscopic and manometric features of the primary motor disorders of the esophagus.

PubMed

Martinez, Júlio César; Lima, Gustavo Rosa de Almeida; Silva, Diego Henrique; Duarte, Alexandre Ferreira; Novo, Neil Ferreira; da Silva, Ernesto Carlos; Pinto, Pérsio Campos Correia; Maia, Alexandre Moreira

2015-01-01

Significant incidence, diagnostic difficulties, clinical relevance and therapeutic efficacy associated with the small number of publications on the primary esophageal motor disorders, motivated the present study. To determine the manometric prevalence of these disorders and correlate them to the endoscopic and clinical findings. A retrospective study of 2614 patients, being 1529 (58.49%) women and 1085 (41.51%) men. From 299 manometric examinations diagnosed with primary esophageal motor disorder, were sought-clinical data (heartburn, regurgitation, dysphagia, odynophagia, non-cardiac chest pain, pharyngeal globe and extra-esophageal symptoms) and/or endoscopic (hiatal hernia, erosive esophagitis, food waste) that motivated the performance of manometry. Were found 49 cases of achalasia, 73 diffuse spasm, 89 nutcracker esophagus, 82 ineffective esophageal motility, and six lower esophageal sphincter hypertension. In relation to the correlations, it was observed that in 119 patients clinical conditions were associated with dysphagia, found in achalasia more than in other conditions; in relationship between endoscopic findings and clinical conditions there was no statistical significance between data. The clinical and endoscopic findings have little value in the characterization of the primary motor disorders of the esophagus, showing even more the need for manometry, particularly in the preoperative period of gastroesophageal reflux disease.

Achalasia—An Autoimmune Inflammatory Disease: A Cross-Sectional Study

PubMed Central

Furuzawa-Carballeda, J.; Aguilar-León, D.; Gamboa-Domínguez, A.; Valdovinos, M. A.; Nuñez-Álvarez, C.; Martín-del-Campo, L. A.; Enríquez, A. B.; Coss-Adame, E.; Svarch, A. E.; Flores-Nájera, A.; Villa-Baños, A.; Ceballos, J. C.; Torres-Villalobos, G.

2015-01-01

Idiopathic achalasia is a disease of unknown etiology. The loss of myenteric plexus associated with inflammatory infiltrates and autoantibodies support the hypothesis of an autoimmune mechanism. Thirty-two patients diagnosed by high-resolution manometry with achalasia were included. Twenty-six specimens from lower esophageal sphincter muscle were compared with 5 esophagectomy biopsies (control). Immunohistochemical (biopsies) and flow cytometry (peripheral blood) analyses were performed. Circulating anti-myenteric autoantibodies were evaluated by indirect immunofluorescence. Herpes simplex virus-1 (HSV-1) infection was determined by in situ hybridization, RT-PCR, and immunohistochemistry. Histopathological analysis showed capillaritis (51%), plexitis (23%), nerve hypertrophy (16%), venulitis (7%), and fibrosis (3%). Achalasia tissue exhibited an increase in the expression of proteins involved in extracellular matrix turnover, apoptosis, proinflammatory and profibrogenic cytokines, and Tregs and Bregs versus controls (P < 0.001). Circulating Th22/Th17/Th2/Th1 percentage showed a significant increase versus healthy donors (P < 0.01). Type III achalasia patients exhibited the highest inflammatory response versus types I and II. Prevalence of both anti-myenteric antibodies and HSV-1 infection in achalasia patients was 100% versus 0% in controls. Our results suggest that achalasia is a disease with an important local and systemic inflammatory autoimmune component, associated with the presence of specific anti-myenteric autoantibodies, as well as HSV-1 infection. PMID:26078981

CONTRAIS: CONservative TReatment for Adolescent Idiopathic Scoliosis: a randomised controlled trial protocol

PubMed Central

2013-01-01

Background Idiopathic scoliosis is a three-dimensional structural deformity of the spine that occurs in children and adolescents. Recent reviews on bracing and exercise treatment have provided some evidence for effect of these interventions. The purpose of this study is to improve the evidence base regarding the effectiveness of conservative treatments for preventing curve progression in idiopathic scoliosis. Methods/design Patients: Previously untreated girls and boys with idiopathic scoliosis, 9 to 17 years of age with at least one year of remaining growth and a curve Cobb angle of 25–40 degrees will be included. A total of 135 participants will be randomly allocated in groups of 45 patients each to receive one of the three interventions. Interventions: All three groups will receive a physical activity prescription according to the World Health Organisation recommendations. One group will additionally wear a hyper-corrective night-time brace. One group will additionally perform postural scoliosis-specific exercises. Outcome: Participation in the study will last until the curve has progressed, or until cessation of skeletal growth. Outcome variables will be measured every 6 months. The primary outcome variable, failure of treatment, is defined as progression of the Cobb angle more than 6 degrees, compared to the primary x-ray, seen on two consecutive spinal standing x-rays taken with 6 months interval. Secondary outcome measures include the SRS-22r and EQ5D-Y quality of life questionnaires, the International Physical Activity Questionnaire (IPAQ) short form, and Cobb angle at end of the study. Discussion This trial will evaluate which of the tested conservative treatment approaches that is the most effective for patients with adolescent idiopathic scoliosis. Trial registration NCT01761305 PMID:24007599

Advances in Management of Esophageal Motility Disorders.

PubMed

Kahrilas, Peter J; Bredenoord, Albert J; Carlson, Dustin A; Pandolfino, John E

2018-04-24

The widespread adoption of high-resolution manometry (HRM) has led to a restructuring in the classification of esophageal motility disorder classification summarized in the Chicago Classification, currently in version 3.0. It has become apparent that the cardinal feature of achalasia, impaired lower esophageal sphincter relaxation, can occur in several disease phenotypes: without peristalsis, with premature (spastic) distal esophageal contractions, with panesophageal pressurization, or even with preserved peristalsis. Furthermore, despite these advances in diagnostics, no single manometric pattern is perfectly sensitive or specific for idiopathic achalasia and complimentary assessments with provocative maneuvers during HRM or interrogating the esophagogastric junction with the functional luminal imaging probe during endoscopy can be useful in clarifying equivocal or inexplicable HRM findings. Using these tools, we have come to conceptualize esophageal motility disorders as characterized by obstructive physiology at the esophagogastric junction, smooth muscle esophagus, or both. Recognizing obstructive physiology as a primary target of therapy has become particularly relevant with the development of a minimally invasive technique for performing a calibrated myotomy of the esophageal circular muscle, the POEM procedure. Now and going forward, optimal management is to render treatment in a phenotype-specific manner: e.g. POEM calibrated to patient-specific physiology for spastic achalasia and spastic disorders of the smooth muscle esophagus, more conservative strategies (pneumatic dilation) for the disorders limited to the sphincter. Copyright © 2018 AGA Institute. Published by Elsevier Inc. All rights reserved.

Laparoscopic myotomy: technique and efficacy in treating achalasia.

PubMed

Ali, A; Pellegrini, C A

2001-04-01

Esophageal Heller myotomy and a partial antireflux procedure for achalasia are the ideal procedures to benefit from the advances in minimally invasive surgery. The magnified view of the operative field provided by the laparoscope allows precise division of the esophageal muscle fibers with excellent results. Laparoscopic Heller myotomy results in reduced postoperative pain, less morbidity, shorter hospitalization, better resolution of dysphagia, and less postoperative heartburn when compared with the open abdominal and even the thoracoscopic approach. A longer myotomy especially at the distal end, and a loose, well-formed partial fundoplication are the keys to a successful outcome. Superior long-term results after surgical myotomy when compared with nonsurgical interventions argue strongly in favor of surgery in any patient who is fit enough to undergo general anesthesia.

Revisional surgery after heller myotomy for treatment of achalasia: a comparative analysis focusing on operative approach.

PubMed

Gouda, Biswanath P; Nelson, Thomas; Bhoyrul, Sunil

2012-08-01

Surgical myotomy is the gold standard in therapy for achalasia, but treatment failures occur and require revisional surgery. A MEDLINE search of peer-reviewed articles published in English from 1970 to December 2008 was performed using the following terms: esophageal achalasia, Heller myotomy, and revisional surgery. Thirty-three articles satisfied our inclusion criteria. A total of 12,727 patients, with mean age of 43.3 years (males 46% and females 50%), underwent Heller myotomy (open 94.8% and laparoscopic 5.2%). Revisional surgery was performed in 6.19%. Procedures performed included revision of the original myotomy or creation of a new myotomy with or without an antireflux procedure or esophagectomy. Reasons for reoperation were incomplete myotomy (51.8%), onset of reflux (34%), megaesophagus (16.2%), and esophageal carcinoma (3.04%). Systematic review of the literature for revisional surgery following Heller myotomy revealed a 6.19% rate of reoperation with a low mortality rate.

Efficacy and Safety of Peroral Endoscopic Myotomy for Treatment of Achalasia After Failed Heller Myotomy.

PubMed

Ngamruengphong, Saowanee; Inoue, Haruhiro; Ujiki, Michael B; Patel, Lava Y; Bapaye, Amol; Desai, Pankaj N; Dorwat, Shivangi; Nakamura, Jun; Hata, Yoshitaka; Balassone, Valerio; Onimaru, Manabu; Ponchon, Thierry; Pioche, Mathieu; Roman, Sabine; Rivory, Jérôme; Mion, François; Garros, Aurélien; Draganov, Peter V; Perbtani, Yaseen; Abbas, Ali; Pannu, Davinderbir; Yang, Dennis; Perretta, Silvana; Romanelli, John; Desilets, David; Hayee, Bu; Haji, Amyn; Hajiyeva, Gulara; Ismail, Amr; Chen, Yen-I; Bukhari, Majidah; Haito-Chavez, Yamile; Kumbhari, Vivek; Saxena, Payal; Talbot, Michael; Chiu, Philip Wai-Yan; Yip, Hon-Chi; Wong, Vivien Wai-Yin; Hernaez, Ruben; Maselli, Roberta; Repici, Alessandro; Khashab, Mouen A

2017-10-01

In patients with persistent symptoms after Heller myotomy (HM), treatment options include repeat HM, pneumatic dilation, or peroral endoscopic myotomy (POEM). We evaluated the efficacy and safety of POEM in patients with achalasia with prior HM vs without prior HM. We conducted a retrospective cohort study of 180 patients with achalasia who underwent POEM at 13 tertiary centers worldwide, from December 2009 through September 2015. Patients were divided into 2 groups: those with prior HM (HM group, exposure; n = 90) and those without prior HM (non-HM group; n = 90). Clinical response was defined by a decrease in Eckardt scores to 3 or less. Adverse events were graded according to criteria set by the American Society for Gastrointestinal Endoscopy. Technical success, clinical success, and rates of adverse events were compared between groups. Patients were followed up for a median of 8.5 months. POEM was technically successful in 98% of patients in the HM group and in 100% of patients in the non-HM group (P = .49). A significantly lower proportion of patients in the HM group had a clinical response to POEM (81%) than in the non-HM group (94%; P = .01). There were no significant differences in rates of adverse events between the groups (8% in the HM group vs 13% in the non-HM group; P = .23). Symptomatic reflux and reflux esophagitis after POEM were comparable between groups. POEM is safe and effective for patients with achalasia who were not treated successfully by prior HM. Although the rate of clinical success in patients with prior HM is lower than in those without prior HM, the safety profile of POEM is comparable between groups. Copyright © 2017 AGA Institute. Published by Elsevier Inc. All rights reserved.

Specific esophagogram to assess functional outcomes after Heller's myotomy and Dor's fundoplication for esophageal achalasia.

PubMed

Tsoukali, E; Gouvas, N; Tsiaoussis, J; Pechlivanides, G; Zervakis, N; Mantides, A; Xynos, E

2011-09-01

Esophageal emptying assessed at the 'timed barium' esophagogram correlates well with symptomatic outcomes after pneumatic dilation for esophageal achalasia, although 30% of patients with satisfactory outcome exhibit partial improvement in emptying. The aim of the study was to investigate any correlation of esophageal emptying to symptomatic response after laparoscopic Heller's myotomy and Dor's fundoplication. 'Bread and barium' (transit time of a barium opaque bread bolus) and 'timed barium' (height of esophageal barium column 5 minutes after ingestion of 200-250 mL of barium suspension) esophagogram was used to assess esophageal emptying in 73 patients with esophageal achalasia before 1 and 5 years (31 cases) after laparoscopic myotomy and anterior fundoplication. Symptoms assessment was based to a specific score. At 1-year follow-up, excellent and good symptomatic results were obtained in 95% of the cases. Esophageal maximum diameter, esophageal transit time, and esophageal barium column were significantly correlated to each other and to symptom score postoperatively (P < 0.001). Complete and partial (<90% and 50-90% postoperative reduction in barium column, respectively) emptying was seen in 55% and 31% of patients with excellent result. Patients with a pseudodiverticulum postoperatively had a more delayed esophageal emptying than those without. Symptomatic outcome and esophageal emptying did not deteriorate at 5-year follow-up. Esophageal emptying assessed by 'barium and bread' and 'timed barium' esophagogram correlated well with symptomatic outcome after laparoscopic myotomy for esophageal achalasia. Complete symptomatic relief does not necessarily reflect complete esophageal emptying. Outcomes do not deteriorate by time. Because of wide availability, esophagogram can be applied in follow-up of postmyotomy patients in conjunction with symptomatic evaluation. © 2011 Copyright the Authors. Journal compilation © 2011, Wiley Periodicals, Inc. and the

Natural history of idiopathic diabetes insipidus.

PubMed

Richards, Gail E; Thomsett, Michael J; Boston, Bruce A; DiMeglio, Linda A; Shulman, Dorothy I; Draznin, Martin

2011-10-01

To determine what percentage of diabetes insipidus (DI) in childhood is idiopathic and to assess the natural history of idiopathic DI. We conducted a retrospective chart review of 105 patients with DI who were born or had DI diagnosed between 1980-1989 at 3 medical centers. A second cohort of 30 patients from 6 medical centers in whom idiopathic DI was diagnosed after 1990 was evaluated retrospectively for subsequent etiologic diagnoses and additional hypothalamic/pituitary deficiencies and prospectively for quality of life. In the first cohort, 11% of patients had idiopathic DI. In the second cohort, additional hypothalamic/pituitary hormone deficiencies developed in 33%, and 37% received an etiologic diagnosis for DI. Health-related quality of life for all the patients with idiopathic DI was comparable with the healthy reference population. Only a small percentage of patients with DI will remain idiopathic after first examination. Other hormone deficiencies will develop later in one-third of those patients, and slightly more than one-third of those patients will have an etiology for the DI diagnosed. Long-term surveillance is important because tumors have been diagnosed as long as 21 years after the onset of DI. Quality of life for these patients is as good as the reference population. Copyright © 2011 Mosby, Inc. All rights reserved.

Laparoscopic Heller myotomy and fundoplication in patients with Chagas' disease achalasia and massively dilated esophagus.

PubMed

Pantanali, Carlos A R; Herbella, Fernando A M; Henry, Maria A; Mattos Farah, Jose Francisco; Patti, Marco G

2013-01-01

Laparoscopic Heller myotomy and fundoplication is considered today the treatment of choice for achalasia. The optimal treatment for end-stage achalasia with esophageal dilation is still controversial. This multicenter and retrospective study aims to evaluate the outcome of laparoscopic Heller myotomy in patients with a massively dilated esophagus. Eleven patients (mean age, 56 years; 6 men) with massively dilated esophagus (esophageal diameter greater than 10 cm) underwent a laparoscopic Heller myotomy and anterior fundoplication between 2000 and 2009 at three different institutions. Preoperative workup included upper endoscopy, esophagram, and esophageal manometry in all patients. Average follow-up was 31.5 months (range, 3 to 60 months). Two patients (18%) had severe dysphagia, four patients (36%) had mild and occasional dysphagia to solid food, and five patients (45%) were asymptomatic. All patients gained or kept body weight, except for the two patients with severe dysphagia. Of the two patients with severe dysphagia, one underwent esophageal dilatation and the other a laparoscopic esophagectomy. They are both doing well. Heller myotomy relieves dysphagia in the majority of patients even when the esophagus is massively dilated.

Vitamin D deficiency in chronic idiopathic urticaria.

PubMed

Movahedi, Masoud; Tavakol, Marzieh; Hirbod-Mobarakeh, Armin; Gharagozlou, Mohammad; Aghamohammadi, Asghar; Tavakol, Zahra; Momenzadeh, Kaveh; Nabavi, Mohammad; Dabbaghzade, Abbas; Mosallanejad, Asieh; Rezaei, Nima

2015-04-01

Chronic urticaria is the most common skin diseases, characterized by chronic cutaneous lesions which severely debilitates patients in several aspects of their everyday life. Vitamin D is known to exert several actions in the immune system and to influence function and differentiation of mast cells, central role players in the pathogenesis of chronic idiopathic urticaria. This study was performed to evaluate the relationship between vitamin D levels and susceptibility to chronic idiopathic urticaria. One hundred and fourteen patients with chronic idiopathic urticaria were recruited in this study along with one hundred and eighty seven sex-matched and age-matched healthy volunteers as the control group. For each patient, urticaria activity score was calculated and autologous serum skin test was done. Vitamin D metabolic statue was measured in serum as 25 hydroxyvitamin D using enzyme immunoassay method. Patients with chronic idiopathic urticaria significantly showed lower levels of vitamin D. Vitamin D deficiency was significantly associated with increased susceptibility to chronic idiopathic urticaria. There was a significant positive correlation between vitamin D levels and urticaria activity score. This study showed that patients with chronic idiopathic urticaria had reduced levels of vitamin D, while vitamin D deficiency could increase susceptibility to chronic idiopathic urticaria.

Laparoscopic calibrated total vs partial fundoplication following Heller myotomy for oesophageal achalasia

PubMed Central

Martino, Natale Di; Brillantino, Antonio; Monaco, Luigi; Marano, Luigi; Schettino, Michele; Porfidia, Raffaele; Izzo, Giuseppe; Cosenza, Angelo

2011-01-01

AIM: To compare the mid-term outcomes of laparoscopic calibrated Nissen-Rossetti fundoplication with Dor fundoplication performed after Heller myotomy for oesophageal achalasia. METHODS: Fifty-six patients (26 men, 30 women; mean age 42.8 ± 14.7 years) presenting for minimally invasive surgery for oesophageal achalasia, were enrolled. All patients underwent laparoscopic Heller myotomy followed by a 180° anterior partial fundoplication in 30 cases (group 1) and calibrated Nissen-Rossetti fundoplication in 26 (group 2). Intraoperative endoscopy and manometry were used to calibrate the myotomy and fundoplication. A 6-mo follow-up period with symptomatic evaluation and barium swallow was undertaken. One and two years after surgery, the patients underwent symptom questionnaires, endoscopy, oesophageal manometry and 24 h oesophago-gastric pH monitoring. RESULTS: At the 2-year follow-up, no significant difference in the median symptom score was observed between the 2 groups (P = 0.66; Mann-Whitney U-test). The median percentage time with oesophageal pH < 4 was significantly higher in the Dor group compared to the Nissen-Rossetti group (2; range 0.8-10 vs 0.35; range 0-2) (P < 0.0001; Mann-Whitney U-test). CONCLUSION: Laparoscopic Dor and calibrated Nissen-Rossetti fundoplication achieved similar results in the resolution of dysphagia. Nissen-Rossetti fundoplication seems to be more effective in suppressing oesophageal acid exposure. PMID:21876635

Dynamic, diagnostic, and pharmacological radionuclide studies of the esophagus in achalasia

DOE Office of Scientific and Technical Information (OSTI.GOV)

Rozen, P.; Gelfond, M.; Zaltzman, S.

1982-08-01

The esophagus was evaluated in 15 patients with achalasia by continuous gamma camera imaging following ingestion of a semi-solid meal labeled with /sup 99m/Tc. The images were displayed and recorded on a simple computerized data processing/display system. Subsequent cine mode images of esophageal emptying demonstrated abnormalities of the body of the esophagus not reflected by the manometric examination. Computer-generated time-activity curves representing specific regions of interest were better than manometry in evaluating the results of myotomy, dilatation, and drug therapy. Isosorbide dinitrate significantly improved esophageal emptying.

An inquiry into chiropractors' intention to treat adolescent idiopathic scoliosis: a telephone survey.

PubMed

Feise, R J

2001-01-01

The primary aim of this study was to (1) determine the clinical management approach of practicing chiropractors with regard to patients with adolescent idiopathic scoliosis and (2) measure the response rate of a telephone survey. A survey instrument was developed and pretested, and a case-specific clinical vignette was generated for a hypothetical typical 12-year-old girl with adolescent idiopathic scoliosis. The instrument addressed 3 domains: the specific management of idiopathic scoliosis, elements guiding the general selection of treatment recommendations, and demographics of respondents. The sample frame consisted of 62,000 US chiropractors, of whom 165 were randomly selected for the survey sample. Interviews were conducted by telephone through use of the tested survey instrument. The response rate was 69% (114/165). Of the 51 nonrespondents, 15 did not have a listed business telephone number and 24 were not in active practice. The response rate of those who met the inclusion criteria (practicing chiropractor with a listed telephone number) was 90% (114/126). The gender, chiropractic college, and years in practice of respondents in this survey were similar to those of respondents in 3 other national surveys. In general, the respondents would provide 6 months of "intensive" chiropractic therapy, then follow the patient for 4 years (near skeletal maturity). Eighty-two percent of respondents named diversified technique as their primary adjustive treatment, 87% would use exercise, and 30% would use electric muscle stimulation as an adjunct to manual therapy. Most surveyed chiropractors would use similar methods (frequency and length of treatment, manipulation technique, and exercise) in the treatment of patients with adolescent idiopathic scoliosis. A high response rate to a national survey can be achieved through use of telephone contact.

Idiopathic ventricular tachycardia and fibrillation.

PubMed

Belhassen, B; Viskin, S

1993-06-01

Important data have recently been added to our understanding of sustained ventricular tachyarrhythmias occurring in the absence of demonstrable heart disease. Idiopathic ventricular tachycardia (VT) is usually of monomorphic configuration and can be classified according to its site of origin as either right monomorphic (70% of all idiopathic VTs) or left monomorphic VT. Several physiopathological types of monomorphic VT can be presently individualized, according to their mode of presentation, their relationship to adrenergic stress, or their response to various drugs. The long-term prognosis is usually good. Idiopathic polymorphic VT is a much rarer type of arrhythmia with a less favorable prognosis. Idiopathic ventricular fibrillation may represent an underestimated cause of sudden cardiac death in ostensibly healty patients. A high incidence of inducibility of sustained polymorphic VT with programmed ventricular stimulation has been found by our group, but not by others. Long-term prognosis on Class IA antiarrhythmic medications that are highly effective at electrophysiologic study appears excellent.

Bayesian comparative effectiveness study of four consensus treatment plans for initial management of systemic juvenile idiopathic arthritis: FiRst-Line Options for Systemic juvenile idiopathic arthritis Treatment (FROST).

PubMed

Nigrovic, Peter A; Beukelman, Timothy; Tomlinson, George; Feldman, Brian M; Schanberg, Laura E; Kimura, Yukiko

2018-06-01

Systemic juvenile idiopathic arthritis is a rare febrile arthritis of childhood characterized by a potentially severe course, including prolonged glucocorticoid exposure, growth failure, destructive arthritis, and life-threatening macrophage activation syndrome. Early cytokine-blocking biologic therapy may improve long-term outcomes, although some systemic juvenile idiopathic arthritis patients respond well to non-biologic treatment, leaving optimal management undefined. Consequently, treatment of new-onset systemic juvenile idiopathic arthritis by expert clinicians varies widely. To describe a pragmatic, observational comparative effectiveness study that takes advantage of diversity in the management of a rare disease: FiRst-Line Options for Systemic juvenile idiopathic arthritis Treatment (FROST), comparing non-biologic and biologic consensus treatment plans for new-onset systemic juvenile idiopathic arthritis within the 60-center Childhood Arthritis and Rheumatology Research Alliance Registry (CARRA). FiRst-Line Options for Systemic juvenile idiopathic arthritis Treatment (FROST) is a multicenter, prospective, non-randomized study that compares four Childhood Arthritis and Rheumatology Research Alliance (CARRA) consensus treatment plans for new-onset systemic juvenile idiopathic arthritis: (1) glucocorticoids alone, (2) methotrexate, (3) interleukin-1 blockade, and (4) interleukin-6 blockade. Patients consenting to participation in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry are started on one of four Consensus Treatment Plans at the discretion of the treating physician. The outcome of primary interest is clinically inactive disease off glucocorticoids at 9 months, comparing non-biologic (Consensus Treatment Plans 1 + 2) versus biologic (Consensus Treatment Plans 3 + 4) strategies. Bayesian analytic methods will be employed to evaluate response rates, using propensity scoring to balance treatment groups for potential

Relation between adolescent idiopathic scoliosis and morphologic somatotypes.

PubMed

LeBlanc, R; Labelle, H; Rivard, C H; Poitras, B

1997-11-01

A prospective and controlled comparative study. To verify the difference in morphologic appearance between a group of adolescents with progressive adolescent idiopathic scoliosis and a control group of normal adolescents. In a previous retrospective study, the possibility of a relation between progressive adolescent idiopathic scoliosis and specific morphotypes was demonstrated. Fifty-two adolescent girls with progressive adolescent idiopathic scoliosis were compared with an age-matched control group of 62 unaffected girls using a classification technique based on morphologic somatotypes. Morphotypes were evaluated with standardized pre-established criteria based on Sheldon's technique. Patients with progressive adolescent idiopathic scoliosis showed significantly less mesomorphism (mean value of 0.88 +/- 0.51) than control girls (mean value of 1.72 +/- 0.52). Adolescent girls with progressive adolescent idiopathic scoliosis have a morphologic somatotype that is different from the normal adolescent population. Subjects with progressive adolescent idiopathic scoliosis are significantly less mesomorphic than control girls. This observation may be of value as a predictive factor for early identification of subjects with adolescent idiopathic scoliosis at greater risk of progression.

The Natural History of Idiopathic Scoliosis During Growth: A Meta-Analysis.

PubMed

Di Felice, Francesca; Zaina, Fabio; Donzelli, Sabrina; Negrini, Stefano

2018-05-01

The aim of the study was to provide a meta-analysis of current literature concerning the natural history of idiopathic scoliosis during growth. A comprehensive search of Medline, Embase, And Scopus databases was conducted up to November 2016. Eligible works were prospective or retrospective studies that enrolled patients with infantile idiopathic scoliosis, juvenile idiopathic scoliosis, or adolescent idiopathic scoliosis, followed up without any treatment from the time of detection. A meta-analysis for proportion was performed. The following studies were grouped per diagnosis: infantile idiopathic scoliosis, juvenile idiopathic scoliosis, and adolescent idiopathic scoliosis. Of the 1797 citations screened, we assessed 61 full-text articles and included 13 of these (2301 participants). Three studies included infantile idiopathic scoliosis patients (347 participants), five studies included a mixed population of juvenile idiopathic scoliosis and adolescent idiopathic scoliosis (1330 participants), and five studies included adolescent idiopathic scoliosis patients only (624 participants). The random pooled estimated progression rate was 49% (95% confidence interval = 1%-97%) for infantile idiopathic scoliosis, 49% in a mixed group of patients affected by juvenile idiopathic scoliosis or adolescent idiopathic scoliosis (95% confidence interval = 19%-79%), and 42% in adolescent idiopathic scoliosis (95% confidence interval = 11%-73%). During growth, idiopathic scoliosis tends to progress in a high percentage of cases. The progression rate varies according to the age at diagnosis, with infantile scoliosis being the most unpredictable. There are many confounders, such as age, Risser sign and baseline Cobb angles that were not consistent among studies, making the data very heterogeneous.

SVC obstruction and stridor relieved by nasogastric tube insertion.

PubMed

Molena, Emma J; Krishnamoorthy, Ashwin; Praveen, Coimbatore

2016-03-01

Achalasia is an idiopathic motility disorder of the oesophagus of increasing incidence. It is characterized by aperistalsis of the lower oesophagus and failure of relaxation of the lower oesophageal sphincter. Patients classically present with chronic symptoms of dysphagia, chest pain, weight loss and regurgitation, and they commonly suffer pulmonary complications such as recurrent microaspiration of static, retained food contents of the upper oesophagus. However, it has also been described, uncommonly, to present with megaoesophagus and secondary tracheal compression. We present a case of megaoesophagus secondary to achalasia which presented with stridor and signs of acute superior vena caval obstruction. Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author 2016.

Major Complications of Pneumatic Dilation and Heller Myotomy for Achalasia: Single Center Experience and Systematic Review of the Literature

PubMed Central

Lynch, Kristle L; Pandolfino, John E; Howden, Colin W; Kahrilas, Peter J

2013-01-01

Background & Aims Pneumatic dilation (PD) and laparoscopic Heller myotomy (LHM) can be definitive therapies for achalasia; recent data suggest comparable efficacy. However, risk must also be considered. We reviewed the major complication rate of PD and LHM in a high volume center and reviewed the corresponding literature. Methods We reviewed 12 years of our institution’s achalasia treatment experience. During this interval a consistent technique of PD was used utilizing Rigiflex dilators. Medical records were reviewed for post-procedure complications. We administered a telephone survey and examined medical records to assess efficacy of treatment. We also performed a systematic review of the literature for comparable clinical data and examined 80 reports encompassing 12,494 LHM and PD procedures. Results At our center, 463 achalasia patients underwent 567 PD or LHM procedures. 78% of the PDs used a 30 mm Rigiflex dilator. 157/184 (85%) patients underwent 1 or 2 PD without any subsequent treatment. There were seven clinically significant perforations; one from PD and 6 from LHM. There were no resultant deaths from these perforations; two deaths occurred within 30 days of LHM from unrelated causes. Complications and deaths post-PD were significantly fewer than those post-LHM (p=.02). Conclusions Esophageal perforation from PD at our high-volume center was less common than often reported and lower than that associated with LHM. We conclude that, in the hands of experienced operators using conservative technique, PD has fewer major complications and deaths than LHM. PMID:23032978

Nifedipine for the poor-risk elderly patient with achalasia: objective response demonstrated by solid meal study

DOE Office of Scientific and Technical Information (OSTI.GOV)

Thomas, E.; Lebow, R.A.; Gubler, R.J.

1984-03-01

We described an 84-year-old woman with symptomatic achalasia who refused both dilation and surgical treatment. She was treated with the calcium channel blocking drug nifedipine, with significant relief of symptoms. Objective evidence of response to the drug was confirmed by using an egg salad sandwich meal labeled with 99mTc-DTPA.

Trends of Heller myotomy hospitalizations for achalasia in the United States, 1993-2005: effect of surgery volume on perioperative outcomes.

PubMed

Wang, Y Richard; Dempsey, Daniel T; Friedenberg, Frank K; Richter, Joel E

2008-10-01

Achalasia is a rare chronic disorder of esophageal motor function. Single-center reports suggest that there has been greater use of laparoscopic Heller myotomy for achalasia in the United States since its introduction in 1992. We aimed to study the trends of Heller myotomy and the relationship between surgery volume and perioperative outcomes. The Healthcare Cost and Utilization Project Nationwide Inpatient Sample (NIS) is a 20% stratified sample of all hospitalizations in the United States. It was used to study the macro-trends of Heller myotomy hospitalizations during 1993-2005. We also used the NIS 2003-2005 micro-data to study the perioperative outcomes of Heller myotomy hospitalizations, using other achalasia and laparoscopic cholecystectomy hospitalizations as control groups. The generalized linear model with repeated observations from the same unit was used to adjust for multiple hospitalizations from the same hospital. The national estimate of Heller myotomy hospitalizations increased from 728 to 2,255 during 1993-2005, while its mean length of stay decreased from 9.9 to 4.3 days. Of the 1,117 Heller myotomy hospitalizations in the NIS 2003-2005, 10 (0.9%) had the diagnosis of esophageal perforation at discharge. Length of stay was negatively correlated with a hospital's number of Heller myotomy per year (correlation coefficient -0.171, P < 0.001). In multivariate log-linear regressions with a control group, a hospital's number of Heller myotomy per year was negatively associated with length of stay (coefficient -0.215 to -0.119, both P < 0.001) and total charges (coefficient -0.252 to -0.073, both P < 0.10). These findings were robust in alternative statistical models, specifications, and subgroup analyses. On a national level, the introduction of laparoscopic Heller myotomy for achalasia was associated with greater use of surgery and shorter length of stay. A larger volume of Heller myotomy in a hospital was associated with better perioperative outcomes

POEM is a cost-effective procedure: cost-utility analysis of endoscopic and surgical treatment options in the management of achalasia.

PubMed

Miller, Heidi J; Neupane, Ruel; Fayezizadeh, Mojtaba; Majumder, Arnab; Marks, Jeffrey M

2017-04-01

Achalasia is a rare motility disorder of the esophagus. Treatment is palliative with the goal of symptom remission and slowing the progression of the disease. Treatment options include per oral endoscopic myotomy (POEM), laparoscopic Heller myotomy (LM) and endoscopic treatments such as pneumatic dilation (PD) and botulinum toxin type A injections (BI). We evaluate the economics and cost-effectiveness of treating achalasia. We performed cost analysis for POEM, LM, PD and BI at our institution from 2011 to 2015. Cost of LM was set to 1, and other procedures are presented as percentage change. Cost-effectiveness was calculated based on cost, number of interventions required for optimal results for dilations and injections and efficacy reported in the current literature. Incremental cost-effectiveness ratio was calculated by a cost-utility analysis using quality-adjusted life year gained, defined as a symptom-free year in a patient with achalasia. Average number of interventions required was 2.3 dilations or two injections for efficacies of 80 and 61 %, respectively. POEM cost 1.058 times the cost of LM, and PD and BI cost 0.559 and 0.448 times the cost of LM. Annual cost per cure over a period of 4 years for POEM, and LM were consistently equivalent, trending the same as PD although this has a lower initial cost. The cost per cure of BI remains stable over 3 years and then doubles. The cost-effectiveness of POEM and LM is equivalent. Myotomy, either surgical or endoscopic, is more cost-effective than BI due to high failure rates of the economical intervention. When treatment is being considered BI should be utilized in patients with less than 2-year life expectancy. Pneumatic dilations are cost-effective and are an acceptable approach to treatment of achalasia, although myotomy has a lower relapse rate and is cost-effective compared to PD after 2 years.

Esophageal achalasia compressing left atrium diagnosed by echocardiography using a liquid containing carbon dioxide in a 21-year-old woman with Turner syndrome.

PubMed

Park, Man Je; Song, Bong Gun; Lee, Hyoun Soo; Kim, Ki Hoon; Ok, Hea Sung; Kim, Byeong Ki; Park, Yong Hwan; Kang, Gu Hyun; Chun, Woo Jung; Oh, Ju Hyeon

2012-01-01

Extrinsic compression of the left atrium by the esophagus, the stomach, or both is an uncommon but important cause of hemodynamic compromise. Achalasia is a motility disorder characterized by impaired relaxation of the lower esophageal sphincter and dilatation of the distal two thirds of the esophagus. Echocardiographic imaging after oral ingestion of liquid containing carbon dioxide allowed for differentiation between a compressive vascular structure and the esophagus. We report a rare case of esophageal achalasia compressing the left atrium diagnosed by echocardiography using a liquid containing carbon dioxide in a 21-year-old woman with Turner syndrome. Copyright © 2012 Elsevier Inc. All rights reserved.

Imaging of juvenile idiopathic arthritis. Part I: Clinical classifications and radiographs

PubMed Central

Matuszewska, Genowefa; Gietka, Piotr; Płaza, Mateusz; Walentowska-Janowicz, Marta

2016-01-01

Juvenile idiopathic arthritis is the most common autoimmune systemic disease of the connective tissue affecting individuals at the developmental age. Radiography is the primary modality employed in the diagnostic imaging in order to identify changes typical of this disease entity and rule out other bone-related pathologies, such as neoplasms, posttraumatic changes, developmental defects and other forms of arthritis. The standard procedure involves the performance of comparative joint radiographs in two planes. Radiographic changes in juvenile idiopathic arthritis are detected in later stages of the disease. Bone structures are assessed in the first place. Radiographs can also indirectly indicate the presence of soft tissue inflammation (i.e. in joint cavities, sheaths and bursae) based on swelling and increased density of the soft tissue as well as dislocation of fat folds. Signs of articular cartilage defects are also seen in radiographs indirectly – based on joint space width changes. The first part of the publication presents the classification of juvenile idiopathic arthritis and discusses its radiographic images. The authors list the affected joints as well as explain the spectrum and specificity of radiographic signs resulting from inflammatory changes overlapping with those caused by the maturation of the skeletal system. Moreover, certain dilemmas associated with the monitoring of the disease are reviewed. The second part of the publication will explain issues associated with ultrasonography and magnetic resonance imaging, which are more and more commonly applied in juvenile idiopathic arthritis for early detection of pathological features as well as the disease complications. PMID:27679726

Idiopathic Hypersomnia: A Study of 77 Cases

PubMed Central

Anderson, Kirstie N.; Pilsworth, Samantha; Sharples, Linda D.; Smith, Ian E.; Shneerson, John M.

2007-01-01

Study Objectives: To review the clinical and polysomnographic characteristics of idiopathic hypersomnia as well as the long-term response to treatment. Setting: The Respiratory Support and Sleep Centre at Papworth Hospital, Cambridge, UK. Patients and Design: A large database of more than 6000 patients with sleep disorders was reviewed. A retrospective study of the clinical and polysomnographic characteristics of 77 patients with idiopathic hypersomnia was performed. Comparison with a similar group of patients with narcolepsy was performed. The response to drug treatment was assessed in 61 patients over a mean follow-up of 3.8 years. Measurements and Results: Idiopathic hypersomnia was 60% as prevalent as narcolepsy. Comparison with a similar group of patients with narcolepsy showed that those with idiopathic hypersomnia were more likely to have prolonged unrefreshing daytime naps, a positive family history, increased slow-wave sleep, and a longer sleep latency on the Multiple Sleep Latency Test. The results of the Multiple Sleep Latency Test were not helpful in predicting disease severity or treatment response. The clinical features were heterogeneous and of variable severity. The majority of patients with idiopathic hypersomnia had symptoms that remained stable over many years, but 11% had spontaneous remission, which was never seen in narcolepsy. Two thirds of patients with idiopathic hypersomnolence had a sustained improvement in daytime somnolence with medication, although a third needed high doses or combinations of drugs. Conclusions: Idiopathic hypersomnolence has characteristic clinical and polysomnographic features but the prolonged latency on the Multiple Sleep Latency Test raises doubt about the validity of this test within the current diagnostic criteria. The disease often responds well to treatment and a substantial minority of patients appear to spontaneously improve. Citation: Anderson KN; Pilsworth S; Sharples LD; Smith IE; Shneerson JM. Idiopathic

Post-Surgical Quality of Life, Psychological State and what Patients Think about the Effectiveness of Heller Myotomy in the Treatment of Achalasia.

PubMed

Vale-Fonseca, Telma; Ferreira-Pinto, Luis; Figueiredo-Braga, Margarida; Carneiro, Silvestre

2016-02-01

The disruption of esophageal motility that characterizes achalasia typically provokes dysphagia, pain, loss of weight and malnutrition. Therefore, patients frequently report a reduction in quality of life and negative emotional states. Laparoscopic Heller myotomy proved to be an effective therapy, enabling the resumption of good quality of life. The authors studied 45 patients previously submitted to laparoscopic Heller myotomy. Postoperative evaluation was performed using a customized version of the achalasia disease-specific quality of life questionnaire. Quality of life and the presence of depressive and anxiety symptoms were assessed using the Portuguese versions of the Medical Outcomes Study SF-36 and the Hospital Anxiety and Depression Scale. Thirty-one patients responded to the survey. Dysphagia was the main clinical symptom before surgery. A clear improvement in dysphagia, regurgitation, pain and weight loss was found after surgery (p < 0.001). The Mental Health domain of SF-36 presented a Pearson correlation coefficient of -0.689 with HADS-D and of -0.557 with HADS-A (p < 0.001 and p = 0.002, respectively). This study demonstrates that the Heller myotomy is associated with a good quality of life in patients with achalasia and strengthens the evidence that this is a safe and reliable procedure.

Aetiology of idiopathic granulomatous mastitis.

PubMed

Altintoprak, Fatih; Kivilcim, Taner; Ozkan, Orhan Veli

2014-12-16

Idiopathic granulomatous mastitis is a rare chronic inflammatory lesion of the breast that can clinically and radiographically mimic breast carcinoma. The most common clinical presentation is an unilateral, discrete breast mass, nipple retraction and even a sinus formation often associated with an inflammation of the overlying skin. The etiology of idiopathic granulomatous mastitis is still obscure. Its treatment remains controversial. The cause may be the autoimmune process, infection, a chemical reaction associated with oral contraceptive pills, or even lactation. Various factors, including hormonal imbalance, autoimmunity, unknown microbiological agents, smoking and α 1-antitrypsin deficiency have been suggested to play a role in disease aetiology. In this review, causing factors in the aetiology of idiopathic granulomatous mastitis are reviewed in detail.

Aetiology of idiopathic granulomatous mastitis

PubMed Central

Altintoprak, Fatih; Kivilcim, Taner; Ozkan, Orhan Veli

2014-01-01

Idiopathic granulomatous mastitis is a rare chronic inflammatory lesion of the breast that can clinically and radiographically mimic breast carcinoma. The most common clinical presentation is an unilateral, discrete breast mass, nipple retraction and even a sinus formation often associated with an inflammation of the overlying skin. The etiology of idiopathic granulomatous mastitis is still obscure. Its treatment remains controversial. The cause may be the autoimmune process, infection, a chemical reaction associated with oral contraceptive pills, or even lactation. Various factors, including hormonal imbalance, autoimmunity, unknown microbiological agents, smoking and α 1-antitrypsin deficiency have been suggested to play a role in disease aetiology. In this review, causing factors in the aetiology of idiopathic granulomatous mastitis are reviewed in detail. PMID:25516860

Laparoscopic Heller Myotomy with Anterior Fundoplication Improves Frequency and Severity of Symptoms of Achalasia, Regardless of Preoperative Severity Determined by Esophagography.

PubMed

Rosemurgy, Alexander; Downs, Darrell; Luberice, Kenneth; Rodriguez, Christian; Swaid, Forat; Patel, Krishen; Toomey, Paul; Ross, Sharona

2018-02-01

This study was undertaken to determine whether postoperative outcomes after laparoscopic Heller myotomy with anterior fundoplication could be predicted by preoperative findings on esophagography. Preoperative barium esophagograms of 135 patients undergoing laparoscopic Heller myotomy with anterior fundoplication were reviewed. The number of esophageal curves, esophageal width, and angulation of the gastroesophageal junction (GEJ) were determined; correlations between these determined parameters and symptoms were assessed using linear regression analysis. The number of esophageal curves correlated with the preoperative frequency of dysphagia, vomiting, chest pain, regurgitation, and heartburn. The width of the esophagus negatively correlated with the preoperative frequency of regurgitation. The angulation of the GEJ did not correlate with preoperative symptoms. Laparoscopic Heller myotomy with anterior fundoplication significantly reduced the frequency and severity of all symptoms, regardless of the number of esophageal curves, esophageal width, or angulation of the GEJ. Laparoscopic Heller myotomy with anterior fundoplication provides dramatic palliation for achalasia. More esophageal curves on preoperative esophagography correlate well with the frequency of a broad range of preoperative symptoms, including the frequency of dysphagia and regurgitation. Patients experience dramatically improved frequency and severity of symptoms after laparoscopic Heller myotomy with anterior fundoplication for achalasia regardless of the number of esophageal curves, esophageal width, or the angulation of the GEJ. Findings on barium esophagogram, in evaluating achalasia, should not deter the application of laparosocopic Heller myotomy with anterior fundoplication.

Comparison of posterior internal anal sphincter myectomy and intrasphincteric botulinum toxin injection for treatment of internal anal sphincter achalasia: a meta-analysis.

PubMed

Friedmacher, Florian; Puri, Prem

2012-08-01

Internal anal sphincter (IAS) achalasia is a clinical condition with presentation similar to Hirschsprung's disease, but with the presence of ganglion cells on rectal suction biopsy (RSB). The diagnosis is made by anorectal manometry (ARM), which demonstrates the absence of the rectosphincteric reflex on rectal balloon inflation. The recommended treatment of choice is posterior IAS myectomy. Recently, intrasphincteric botulinum toxin (Botox) injection has been effectively used for treatment of IAS achalasia. The aim of this meta-analysis was to compare the efficacy of posterior IAS myectomy with intrasphincteric Botox injection for treatment of IAS achalasia. A systematic literature search for relevant articles was conducted using the following databases: MEDLINE( ® ), EMBASE(®), ISI Web of Science(SM) and the Cochrane Library. A meta-analysis was performed with the studies where IAS achalasia was diagnosed based on the results of ARM and RSB. Odds ratio (OR) with 95 % confidence intervals were calculated. Sixteen prospective and retrospective studies, published from 1973 to 2009, were identified. A total of 395 patients with IAS achalasia were included in this meta-analysis. Fifty-eight percent of patients underwent IAS myectomy and 42 % Botox injection. Regular bowel movements were significantly more frequent after IAS myectomy (OR 0.53, [95 % CI 0.29-0.99]; p = 0.04). There was no significant difference in continued use of laxatives or rectal enemas (OR 0.92, [95 % CI 0.34-2.53], p = 0.89) and in overall complication rates between both procedures (OR 0.68, [95 % CI 0.38-1.21]; p = 0.19). Looking at specific complications, the rate of transient faecal incontinence was significantly higher after Botox injection (OR 0.07, [95 % CI 0.01-0.54]; p < 0.01). Constipation and soiling were not significantly different between both procedures (OR 0.66, [95 % CI 0.30-1.48]; p = 0.31 and OR 0.24, [95 % CI 0.03-2.07]; p = 0.25). The rate of non

Per-Oral Endoscopic Myotomy Versus Laparoscopic Heller Myotomy for Achalasia: A Meta-Analysis of Nonrandomized Comparative Studies.

PubMed

Zhang, Yuan; Wang, Hongjuan; Chen, Xingdong; Liu, Lan; Wang, Hongbo; Liu, Bin; Guo, Jianqiang; Jia, Hongying

2016-02-01

We aimed to assess the short-term outcomes of per-oral endoscopic myotomy (POEM) compared with laparoscopic Heller myotomy (LHM) for achalasia through a meta-analysis of nonrandomized comparative studies.We searched PubMed, Embase, Medline, Cochrane Library, and Google Scholar for studies that compared POEM and LHM for achalasia and were published between January 1, 2008 and December 31, 2014. The Methodological Index for Nonrandomized Studies (MINORS) was used to evaluate the quality of the studies. Random- and fixed-effects meta-analytical models were used, and between-study heterogeneity was assessed.Four nonrandomized comparative studies that included 317 patients (125 in the POEM group and 192 in the LHM group) met our research criteria and were assessed. There were no differences between the POEM and LHM groups in terms of sex, preoperative Eckhart score, length of myotomy, operation time, length of hospital stay, and complications. The patients in the POEM group were older than those in the LHM group (MD =2.81, 95% CI 0.27-5.35; P = 0.03) with high between-study homogeneity (χ = 1.96, df = 2, I = 0%; P = 0.38). The patients in the POEM group had a lower Eckardt score after surgery compared with those in the LHM group (MD = -0.30, 95% CI -0.42 to -0.18; P < 0.001) with high between-study homogeneity (χ = 0.00, df = 1, I = 0%; P = 1.00).The efficacy and safety of POEM appear to be comparable to those of LHM. Multicenter and randomized trials with larger sample size are needed to further compare the efficacy and safety of POEM and LHM for the treatment of achalasia.

Acute Idiopathic Scrotal Edema: Systematic Literature Review.

PubMed

Santi, Maristella; Lava, Sebastiano A G; Simonetti, Giacomo D; Bianchetti, Mario G; Milani, Gregorio P

2018-06-01

 Existing information on acute idiopathic scrotal edema relies on small case series and textbooks.  We searched reports with no date limits on acute idiopathic scrotal edema.  Thirty-seven studies were included. Sixteen case series addressed the prevalence of acute idiopathic scrotal edema among males with acute scrotum: among 3,403 cases, the diagnosis of acute idiopathic scrotal edema was made in 413 cases (12%). Twenty-four reports addressed history, findings, management, and course of acute idiopathic scrotal edema in 311 patients. The patients mostly ranged in age from 5 to 8 years, presented with acute scrotal redness and swelling, associated or not with mild pain. Ninety percent or more of the cases developed in patients without atopic diathesis and were not preceded by inguinoscrotal surgery, acute febrile illnesses, or trauma. They were afebrile; in good general condition; and presented without pruritus, nausea or vomiting, or abdominal pain. The lesions were bilateral in two-thirds and unilateral in one-third of the cases. The condition resolved spontaneously within 2 to 3 days without sequelae. Approximately 10% of the cases experienced a recurrence.  Acute idiopathic scrotal edema is a self-limiting condition that accounts for ≥ 10% of cases of acute scrotum in children and adolescents. Georg Thieme Verlag KG Stuttgart · New York.

Idiopathic hypersomnia: a study of 77 cases.

PubMed

Anderson, Kirstie N; Pilsworth, Samantha; Sharples, Linda D; Smith, Ian E; Shneerson, John M

2007-10-01

To review the clinical and polysomnographic characteristics of idiopathic hypersomnia as well as the long-term response to treatment. The Respiratory Support and Sleep Centre at Papworth Hospital, Cambridge, UK. A large database of more than 6000 patients with sleep disorders was reviewed. A retrospective study of the clinical and polysomnographic characteristics of 77 patients with idiopathic hypersomnia was performed. Comparison with a similar group of patients with narcolepsy was performed. The response to drug treatment was assessed in 61 patients over a mean follow-up of 3.8 years. Idiopathic hypersomnia was 60% as prevalent as narcolepsy. Comparison with a similar group of patients with narcolepsy showed that those with idiopathic hypersomnia were more likely to have prolonged unrefreshing daytime naps, a positive family history, increased slow-wave sleep, and a longer sleep latency on the Multiple Sleep Latency Test. The results of the Multiple Sleep Latency Test were not helpful in predicting disease severity or treatment response. The clinical features were heterogeneous and of variable severity. The majority of patients with idiopathic hypersomnia had symptoms that remained stable over many years, but 11% had spontaneous remission, which was never seen in narcolepsy. Two thirds of patients with idiopathic hypersomnolence had a sustained improvement in daytime somnolence with medication, although a third needed high doses or combinations of drugs. Idiopathic hypersomnolence has characteristic clinical and polysomnographic features but the prolonged latency on the Multiple Sleep Latency Test raises doubt about the validity of this test within the current diagnostic criteria. The disease often responds well to treatment and a substantial minority of patients appear to spontaneously improve.

Nephrotic syndrome complicated by idiopathic central diabetes insipidus.

PubMed

Konomoto, Takao; Tanaka, Etsuko; Imamura, Hideaki; Orita, Mayuko; Sawada, Hirotake; Nunoi, Hiroyuki

2014-05-01

There is ongoing discussion regarding the mechanisms underlying edema formation in nephrotic syndrome (NS). Many studies published in the last decade reported that primary renal sodium retention was a major factor in edema formation. However, many of the factors influencing edema formation in NS remain unclear, including the role of arginine vasopressin (AVP). We report a 12-year-old boy with steroid-dependent NS complicated by idiopathic central diabetes insipidus (CDI). He did not develop edema during his first relapse of NS after developing CDI, despite having hypoalbuminemia. He had polydipsia, polyuria, low urine osmolality, and a low serum arginine AVP level. His fractional excretion of sodium was only slightly low. Endocrinological testing and magnetic resonance imaging revealed idiopathic CDI. After starting desmopressin therapy, he developed edema when his NS relapsed. This is the first known reported case of NS in a patient with CDI. The findings suggest that appropriate AVP secretion in response to an increase in serum osmolality caused by renal sodium retention is necessary for excess extracellular fluid accumulation in NS. Further investigation is needed to more fully understand the role of AVP in edema formation in NS.

Effect of peroral esophageal myotomy for achalasia treatment: A Chinese study

PubMed Central

Lu, Bin; Li, Meng; Hu, Yue; Xu, Yi; Zhang, Shuo; Cai, Li-Jun

2015-01-01

AIM: To assess the safety and feasibility of peroral esophageal myotomy (POEM) in patients with achalasia. METHODS: From January 2012 to March 2014, 50 patients (28 men, 22 women; mean age: 42.8 years, range: 14-70 years) underwent POEM. Pre- and postoperative symptoms were quantified using the Eckardt scoring system. Barium swallow and esophagogastroscopy were performed before and after POEM, respectively. Esophageal motility was evaluated in all patients, both preoperatively and one month after POEM treatment, using a high-resolution manometry system. Manometry data, Eckardt scores, lower esophageal sphincter pressure and barium swallow results were used to evaluate the effect of the procedure. RESULTS: POEM was successfully completed for all patients. The mean procedure time was 55.4 ± 17.3 min and the mean total length of myotomy of the circular esophagus was 10.5 ± 2.6 cm. No specific complications occurred, with the exception of two patients that developed asymptomatic pneumomediastinum and subcutaneous emphysema. Clinical improvement in symptoms was achieved in all patients. Approximately 77.5% of patients experienced weight gain 6 mo after POEM, with an average of 4.78 kg (range: 2-15 kg). The lower esophageal sphincter resting pressure, four second integrated relaxation pressure and Eckardt scores were all significantly reduced after POEM (Ps < 0.05). A small segment of proximal esophageal peristalsis appeared postoperatively in two patients, but without normal esophageal peristalsis. The average diameter of the esophageal lumen decreased significantly from 4.39 to 3.09 cm (P < 0.01). CONCLUSION: POEM can relieve achalasia symptoms, improve gastroesophageal junction relaxation and restore esophageal body motility function, but not normal esophageal peristalsis. PMID:25987787

Effect of peroral esophageal myotomy for achalasia treatment: A Chinese study.

PubMed

Lu, Bin; Li, Meng; Hu, Yue; Xu, Yi; Zhang, Shuo; Cai, Li-Jun

2015-05-14

To assess the safety and feasibility of peroral esophageal myotomy (POEM) in patients with achalasia. From January 2012 to March 2014, 50 patients (28 men, 22 women; mean age: 42.8 years, range: 14-70 years) underwent POEM. Pre- and postoperative symptoms were quantified using the Eckardt scoring system. Barium swallow and esophagogastroscopy were performed before and after POEM, respectively. Esophageal motility was evaluated in all patients, both preoperatively and one month after POEM treatment, using a high-resolution manometry system. Manometry data, Eckardt scores, lower esophageal sphincter pressure and barium swallow results were used to evaluate the effect of the procedure. POEM was successfully completed for all patients. The mean procedure time was 55.4 ± 17.3 min and the mean total length of myotomy of the circular esophagus was 10.5 ± 2.6 cm. No specific complications occurred, with the exception of two patients that developed asymptomatic pneumomediastinum and subcutaneous emphysema. Clinical improvement in symptoms was achieved in all patients. Approximately 77.5% of patients experienced weight gain 6 mo after POEM, with an average of 4.78 kg (range: 2-15 kg). The lower esophageal sphincter resting pressure, four second integrated relaxation pressure and Eckardt scores were all significantly reduced after POEM (Ps < 0.05). A small segment of proximal esophageal peristalsis appeared postoperatively in two patients, but without normal esophageal peristalsis. The average diameter of the esophageal lumen decreased significantly from 4.39 to 3.09 cm (P < 0.01). POEM can relieve achalasia symptoms, improve gastroesophageal junction relaxation and restore esophageal body motility function, but not normal esophageal peristalsis.

A possible association of idiopathic osteosclerosis with excessive occlusal forces.

PubMed

Misirlioglu, Melda; Nalcaci, Rana; Baran, Ilgi; Adisen, Mehmet Zahit; Yilmaz, Selmi

2014-03-01

The aim of the study was to determine the relationship between idiopathic osteosclerotic lesions and occlusal forces using the T-Scan II computerized occlusal analysis device, and to test the sensitivity of the system in occlusal analysis. The study was conducted with 21 volunteers with idiopathic osteosclerosis (IO; 14 women, 7 men) aged between 17 and 62 years (mean 29.95). For every patient, seven or eight recordings were made with the T-Scan II occlusal analysis device in maximum intercuspation, and the last two (excluding any with technical problems) were chosen for evaluation. For each lesion-related area, the distribution of high occlusal forces from two different movies was analyzed. In 18 patients (85.71%), lesions were observed in an area of high occlusal force, and in 13 patients (61.9%), the lesions were located at the first area subjected to high occlusal forces. The percentage distribution of high forces at a lesion related area ranged from 0% to 88%. On average, the high forces at an osteosclerotic lesion area accounted for 20% of the maximum total force. No statistical differences were observed between the measurements of the two selected recordings (P > .05). The findings of this study suggest a possible relationship between IO and occlusal forces and primary contacts. T-Scan II was found to be a successful diagnostic device for detecting primary contacts and excessive occlusal forces.

[Idiopathic facial paralysis in children].

PubMed

Achour, I; Chakroun, A; Ayedi, S; Ben Rhaiem, Z; Mnejja, M; Charfeddine, I; Hammami, B; Ghorbel, A

2015-05-01

Idiopathic facial palsy is the most common cause of facial nerve palsy in children. Controversy exists regarding treatment options. The objectives of this study were to review the epidemiological and clinical characteristics as well as the outcome of idiopathic facial palsy in children to suggest appropriate treatment. A retrospective study was conducted on children with a diagnosis of idiopathic facial palsy from 2007 to 2012. A total of 37 cases (13 males, 24 females) with a mean age of 13.9 years were included in this analysis. The mean duration between onset of Bell's palsy and consultation was 3 days. Of these patients, 78.3% had moderately severe (grade IV) or severe paralysis (grade V on the House and Brackmann grading). Twenty-seven patients were treated in an outpatient context, three patients were hospitalized, and seven patients were treated as outpatients and subsequently hospitalized. All patients received corticosteroids. Eight of them also received antiviral treatment. The complete recovery rate was 94.6% (35/37). The duration of complete recovery was 7.4 weeks. Children with idiopathic facial palsy have a very good prognosis. The complete recovery rate exceeds 90%. However, controversy exists regarding treatment options. High-quality studies have been conducted on adult populations. Medical treatment based on corticosteroids alone or combined with antiviral treatment is certainly effective in improving facial function outcomes in adults. In children, the recommendation for prescription of steroids and antiviral drugs based on adult treatment appears to be justified. Randomized controlled trials in the pediatric population are recommended to define a strategy for management of idiopathic facial paralysis. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis.

PubMed

King, Talmadge E; Bradford, Williamson Z; Castro-Bernardini, Socorro; Fagan, Elizabeth A; Glaspole, Ian; Glassberg, Marilyn K; Gorina, Eduard; Hopkins, Peter M; Kardatzke, David; Lancaster, Lisa; Lederer, David J; Nathan, Steven D; Pereira, Carlos A; Sahn, Steven A; Sussman, Robert; Swigris, Jeffrey J; Noble, Paul W

2014-05-29

In two of three phase 3 trials, pirfenidone, an oral antifibrotic therapy, reduced disease progression, as measured by the decline in forced vital capacity (FVC) or vital capacity, in patients with idiopathic pulmonary fibrosis; in the third trial, this end point was not achieved. We sought to confirm the beneficial effect of pirfenidone on disease progression in such patients. In this phase 3 study, we randomly assigned 555 patients with idiopathic pulmonary fibrosis to receive either oral pirfenidone (2403 mg per day) or placebo for 52 weeks. The primary end point was the change in FVC or death at week 52. Secondary end points were the 6-minute walk distance, progression-free survival, dyspnea, and death from any cause or from idiopathic pulmonary fibrosis. In the pirfenidone group, as compared with the placebo group, there was a relative reduction of 47.9% in the proportion of patients who had an absolute decline of 10 percentage points or more in the percentage of the predicted FVC or who died; there was also a relative increase of 132.5% in the proportion of patients with no decline in FVC (P<0.001). Pirfenidone reduced the decline in the 6-minute walk distance (P=0.04) and improved progression-free survival (P<0.001). There was no significant between-group difference in dyspnea scores (P=0.16) or in rates of death from any cause (P=0.10) or from idiopathic pulmonary fibrosis (P=0.23). However, in a prespecified pooled analysis incorporating results from two previous phase 3 trials, the between-group difference favoring pirfenidone was significant for death from any cause (P=0.01) and from idiopathic pulmonary fibrosis (P=0.006). Gastrointestinal and skin-related adverse events were more common in the pirfenidone group than in the placebo group but rarely led to treatment discontinuation. Pirfenidone, as compared with placebo, reduced disease progression, as reflected by lung function, exercise tolerance, and progression-free survival, in patients with

Twenty-year trends in the utilization of Heller myotomy for achalasia in the United States.

PubMed

Haisley, Kelly R; Preston, Jennifer F; Dolan, James P; Diggs, Brian S; Hunter, John G

2017-08-01

Trends in the utilization of Heller myotomy for achalasia in the U.S. over time have not been previously described. Using the Nationwide Inpatient Sample (NIS) database, we analyzed patients undergoing Heller myotomy for achalasia over a 20-year period (1992-2011) to estimate rates of Heller myotomy, locations where the procedures were performed (rural, urban or teaching) and changes in technique (laparoscopic vs open) as well as outcomes of length of stay and in-hospital mortality. Over the last 20 years, the total number of Heller myotomies performed in the U.S. has increased (1576 cases in 1992 to 5046 cases in 2011, p = 0.001). These procedures are now being performed laparoscopically (0.9%-67.0%, p < 0.001) and at urban teaching hospitals (45.4%-77.1%, p < 0.001). In-hospital mortality has decreased (0.9%-0.3%, p = 0.006). Hospital length of stay has decreased from 7 days to 2 days (p < 0.001). These data show a trend of increasing utilization of laparoscopic Heller myotomy at teaching institutions with decreased in-hospital mortality and shorter LOS. Copyright © 2017. Published by Elsevier Inc.

Randomized controlled trial of laparoscopic Heller myotomy plus Dor fundoplication versus Nissen fundoplication for achalasia: long-term results.

PubMed

Rebecchi, Fabrizio; Giaccone, Claudio; Farinella, Eleonora; Campaci, Roberto; Morino, Mario

2008-12-01

To compare in a prospective, randomized trial the long-term results of laparoscopic Heller myotomy plus Dor fundoplication versus laparoscopic Heller myotomy plus floppy-Nissen for achalasia. Anterior fundoplication is usually performed after Heller myotomy to control GER; however, the incidence of postoperative GER ranges between 10% and 30%. Total fundoplication may aid in reducing GER rates. From December 1993 to September 2002, 153 patients with achalasia underwent Heller laparoscopic myotomy plus antireflux fundoplication. Of these, 9 were excluded from the study. The remaining 144 patients were randomly assigned to 2 treatment groups: Heller laparoscopic myotomy plus anterior fundoplication (Dor procedure) or Heller laparoscopic myotomy plus total fundoplication (floppy-Nissen procedure). The primary end point was incidence of clinical and instrumental GER after a minimum of 60 months follow-up. The secondary end point was recurrence of dysphagia. Follow-up clinical assessments were performed at 1, 3, 12, and 60 months using a modified DeMeester Symptom Scoring System (MDSS). Esophageal manometry and 24-hour pH monitoring were performed at 3, 12, and 60 months postoperative. Of the 144 patients originally included in the study, 138 were available for long-term analysis: 71 (51%) underwent antireflux fundoplication plus a Dor procedure (H + D group) and 67 (49%) antireflux fundoplication plus a Nissen procedure (H + N group). No mortality was observed. The mean follow-up period was 125 months. No statistically significant differences in clinical (5.6% vs. 0%) or instrumental GER (2.8% vs. 0%) were found between the 2 groups; however, a statistically significant difference in dysphagia rates was noted (2.8% vs. 15%; P < 0.001). Although both techniques achieved long-term GER control, the recurrence rate of dysphagia was significantly higher among the patients who underwent Nissen fundoplication. This evidence supports the use of Dor fundoplication as the

Ultrasound-guided corticosteroid injection of the subtalar joint for treatment of juvenile idiopathic arthritis.

PubMed

Young, Cody M; Horst, Deanna M; Murakami, James W; Shiels, William E

2015-07-01

The subtalar joint is commonly affected in children with juvenile idiopathic arthritis and is challenging to treat percutaneously. To describe the technique for treating the subtalar joint with US-guided corticosteroid injections in children and young adults with juvenile idiopathic arthritis and to evaluate the safety of the treatment. We retrospectively analyzed 122 patients (age 15 months-29 years) with juvenile idiopathic arthritis who were referred by a pediatric rheumatologist for corticosteroid injection therapy for symptoms related to the hindfoot or ankle. In these patients the diseased subtalar joint was targeted for therapy, often in conjunction with adjacent affected joints or tendon sheaths of the ankle. We used a protocol based on age, weight and joint for triamcinolone hexacetonide or triamcinolone acetonide dose prescription. We describe the technique for successful treatment of the subtalar joint. A total of 241 subtalar joint corticosteroid injections were performed under US guidance, including 68 repeat injections for recurrent symptoms in 26 of the 122 children and young adults. The average time interval between repeat injections was 24.8 months (range 2.2-130.7 months, median 14.2 months). Subcutaneous tissue atrophy and skin hypopigmentation were the primary complications observed. These complications occurred in 3.9% of the injections. With appropriate training and practice, the subtalar joint can be reliably and safely targeted with US-guided corticosteroid injection to treat symptoms related to juvenile idiopathic arthritis.

Juvenile idiopathic arthritis

MedlinePlus

... www.ncbi.nlm.nih.gov/pubmed/21452260 . Long AR, Rouster-Stevens KA. The role of exercise therapy ... nlm.nih.gov/pubmed/21131338 . Wu EY, Bryan AR, Rabinovich CE. Juvenile idiopathic arthritis. In: Kliegman RM, ...

Surgical management for achalasia after coronary artery bypass graft using the right gastroepiploic artery: a case report.

PubMed

Muranushi, Ryo; Miyazaki, Tatsuya; Saito, Hideyuki; Kuriyama, Kengo; Yoshida, Tomonori; Kumakura, Yuji; Honjyo, Hiroaki; Yokobori, Takehiko; Sakai, Makoto; Sohda, Makoto; Kuwano, Hiroyuki

2017-12-01

The right gastroepiploic artery is commonly used in coronary artery bypass grafting. Appropriate strategies are required when performing upper abdominal surgeries after the right gastroepiploic artery has been used in coronary artery bypass grafting because compressing or injuring the graft may cause myocardial ischemia and fatal arrhythmias. To our knowledge, this is the first reported case of surgery for achalasia performed after coronary artery bypass grafting using the right gastroepiploic artery. We have discussed the surgical procedure and particular intraoperative considerations. A 62-year-old man who had undergone coronary artery bypass grafting using the right gastroepiploic artery presented with achalasia. Because medication and balloon dilation had been ineffective and he was having difficulty ingesting food, we performed a Heller-Dor procedure via laparotomy. The right gastroepiploic artery was not damaged during this surgery, and there were no perioperative cardiovascular complications. Adequate control of symptoms was achieved. When performing upper abdominal surgeries after coronary artery bypass grafting with the right gastroepiploic artery, it is necessary to investigate the patient carefully preoperatively and adapt the intraoperative procedure to minimize risk of injury to the graft and consequent cardiovascular complications.

Quality of life comparing dor and toupet after heller myotomy for achalasia.

PubMed

Tomasko, Jonathan M; Augustin, Toms; Tran, Tung T; Haluck, Randy S; Rogers, Ann M; Lyn-Sue, Jerome R

2014-01-01

Laparoscopic Heller cardiomyotomy (LHC) is standard therapy for achalasia. Traditionally, an antireflux procedure has accompanied the myotomy. This study was undertaken to compare quality-of-life outcomes between patients undergoing myotomy with Toupet versus Dor fundoplication. In addition, we investigated overall patient satisfaction after LHC in the treatment of achalasia. One hundred thirty-five patients who underwent LHC over a 13-year period were identified for inclusion. Symptoms queried included dysphagia, heartburn, and bloating using the Gastroesophageal Reflux Disease-Health-Related Quality of Life Scale and a second published scale for the assessment of gastroesophageal reflux disease and dysphagia symptoms. The patients' overall satisfaction after surgery was also rated. Data were compared on the basis of type of fundoplication. Symptom scores were analyzed using chi-square tests and Fisher's exact tests. Sixty-three patients completed the survey (47%). There were no perioperative deaths or reoperations. The mean length of stay was 2.8 days. The mean operative time for LHC with Toupet fundoplication was 137.3±30.91 minutes and for LHC with Dor fundoplication was 111.5±32.44 minutes (P=.006). There was no difference with respect to the incidence or severity of postoperative heartburn, dysphagia, or bloating. Overall satisfaction with Toupet fundoplication was 87.5% and with Dor fundoplication was 93.8% (P>.999). LHC with either Toupet or Dor fundoplication gave excellent patient satisfaction. Postoperative symptoms of heartburn and dysphagia were equivalent when comparing LHC with either antireflux procedure. Dor and Toupet fundoplication were found to have equivalent outcomes in the short term. We prefer Dor to Toupet fundoplication because of its decreased need for extensive dissection and better mucosal protection.

Physiotherapy in pauciarticular juvenile idiopathic arthritis. Case study.

PubMed

Zuk, Beata; Kaczor, Zofia; Zuk-Drążyk, Berenika; Księżopolska-Orłowska, Krystyna

2014-01-01

Juvenile idiopathic arthritis (JIA) is the most common arthropathy of childhood and adolescence. This term encompasses a group of chronic systemic inflammatory diseases of the connective tissue which cause arthritis in patients under 16 years of age lasting at least 6 weeks. The authors presented the characteristic features of physiotherapy based on functional examination results on the basis of two cases of girls with pauciarticular JIA treated according to an established pharmacological regimen. Physiotherapy should be introduced at an early stage of the disease. Kinesiotherapy preceded by history-taking and a functional examination of the patient, has to focus on both primary and secondary joint lesions.

[Panhypopituitarism and dwarfism in a man with a primary empty sella turcica (author's transl)].

PubMed

Turpin, G; Jambart, S; de Gennes, J L

1979-03-10

Endocrine features associated with the primary empty sella turcica syndrom are rare; they are usually related to a pituitary microadenoma and more rarely to an idiopathic panhypop ituitarism. A pituitary dwarfism with an idiopathic panhypopituitarism of hypothalamic origin, associated with an "empty" sella turcica containing functional and stimulable pituitary tissue, is reported in a 27 year old male Moraccan.

Identification of risk factors for mucosal injury during laparoscopic Heller myotomy for achalasia.

PubMed

Tsuboi, Kazuto; Omura, Nobuo; Yano, Fumiaki; Hoshino, Masato; Yamamoto, Se-Ryung; Akimoto, Shusuke; Masuda, Takahiro; Kashiwagi, Hideyuki; Yanaga, Katsuhiko

2016-02-01

Mucosal injury during myotomy is the most frequent complication seen with the Heller-Dor procedure for achalasia. The present study aimed to examine risk factors for such mucosal injury during this procedure. This was a retrospective analysis of patients who underwent the laparoscopic Heller-Dor procedure for achalasia at a single facility. Variables for evaluation included patient characteristics, preoperative pathophysiological findings, and surgeon's operative experience. Logistic regression was used to identify risk factors. We also examined surgical outcomes and the degree of patient satisfaction in relation to intraoperative mucosal injury. Four hundred thirty-five patients satisfied study criteria. Intraoperative mucosal injury occurred in 67 patients (15.4%). In univariate analysis, mucosal injury was significantly associated with the patient age ≥60 years, disease history ≥10 years, prior history of cardiac diseases, preoperative esophageal transverse diameter ≥80 mm, and surgeon's operative experience with fewer than five cases. In multivariate analysis involving these factors, the following variables were identified as risk factors: age ≥60 years, esophageal transverse diameter ≥80 mm, and surgeon's operative experience with fewer than five cases. The mucosal injury group had significant extension of the operative time and increased blood loss. However, there were no significant differences between the two groups in the incidence of reflux esophagitis or the degree of symptom alleviation postoperatively. The fragile esophagus caused by advanced patient age and/or dilatation were risk factor for mucosal injury during laparoscopic Heller-Dor procedure. And novice surgeon was also identified as an isolated risk factor for mucosal injury.

Isosorbide dinitrate and nifedipine treatment of achalasia: a clinical, manometric and radionuclide evaluation

DOE Office of Scientific and Technical Information (OSTI.GOV)

Gelfond, M.; Rozen, P.; Gilat, T.

1982-11-01

The effects of sublingual isosorbide dinitrate (5 mg) and nifedipine (20 mg) were compared in 15 patients with achalasia. The parameters examined included the manometric measurement of the lower esophageal sphincter pressure, the radionuclide assessment of esophageal emptying and the clinical response. The mean basal lower esophageal sphincter pressure fell significantly after both drugs (p less than 0.01), with a maximum fall of 63.5% 10 min after receiving isosorbide dinitrate, but by only 46.7% 30 min after nifedipine. The esophageal radionuclide test meal retention was significantly less (p less than 0.01) only after receiving isosorbide dinitrate. The drug improved initialmore » esophageal emptying by its effect on the lower esophageal sphincter and by relieving the test meal hold-up noted to occur at the junction of the upper and midesophagus. Eight patients cleared their test meal within 10 min after isosorbide dinitrate administration while only two did so after nifedipine. Subjectively, 13 patients had their dysphagia relieved by isosorbide dinitrate and 8 by nifedipine. However, this relief was not confirmed in 4 patients by the radionuclide study and they, as well as the other 3 patients who did not respond to therapy, were referred to pneumatic dilatation. Side effects were more prominent after nitrates. Three of the patients are currently receiving nifedipine and 5 patients received isosorbide dinitrate therapy for 8-14 mo. The radionuclide test meal is currently the best way of objectively evaluating drug therapy in patients with achalasia. Isosorbide dinitrate is more effective than nifedipine in relieving their symptoms.« less

Idiopathic Mast Cell Activation Syndrome With Associated Salicylate Intolerance.

PubMed

Rechenauer, Tobias; Raithel, Martin; Götze, Thomas; Siebenlist, Gregor; Rückel, Aline; Baenkler, Hanns-Wolf; Hartmann, Arndt; Haller, Florian; Hoerning, André

2018-01-01

Idiopathic mast cell activation syndrome can be a rare cause for chronic abdominal pain in children. It remains a diagnosis by exclusion that can be particularly challenging due to the vast variety of possible clinical manifestations. We present a 13-year-old boy who suffered from a multitude of unspecific complaints over a long period of time. In this case, an assessment of mast cell-derived metabolites and immunohistochemical analysis of bioptic specimen was worthwhile. After ruling out, primary (oncologic) and secondary causes for mast cell activation, pharmacologic treatment adapted to the patient's salicylate intolerance resulted in a major relief of symptoms.

Laparoscopic extended cardiomyotomy in children: an effective procedure for the treatment of esophageal achalasia.

PubMed

Tannuri, Ana Cristina Aoun; Tannuri, Uenis; Velhote, Manoel Carlos Prieto; Romão, Rodrigo Luiz Pinto

2010-07-01

Achalasia of the esophagus is characterized by aperistalsis and incomplete relaxation of the lower esophageal sphincter in response to swallowing. The objective of the present study is to present the experience of a modified Heller myotomy via a laparoscopic approach for the treatment of children who had this condition. A retrospective review of medical records of all patients who underwent this procedure from 2000 to 2009 was performed. The procedure consisted of an extended esophagomyotomy beginning on the lower part of the lower esophageal sphincter and continuing 5 to 6 cm above on the lower third of the esophagus, and then extended 3 to 4 cm below to the stomach, associated with an anterior 180-degree hemi-fundoplication according to Dor's technique. Fifteen patients were included in the study. There were 8 female and 7 male patients. Mean operating time was 190 minutes with no intraoperative complications and 1 conversion to open surgery because of difficulty in dissecting an inflamed distal esophagus. In a mean follow-up period of 32.3 months, 2 patients had recurrence of mild dysphagia that disappeared spontaneously, and 1 required a single botulinum toxin injection with complete resolution of symptoms. We conclude that the laparoscopic extended Heller myotomy with Dor fundoplication is a safe and effective method for the treatment for achalasia in the pediatric population even in advanced cases. Copyright 2010 Elsevier Inc. All rights reserved.

Intraspinal anomalies in early-onset idiopathic scoliosis.

PubMed

Pereira, E A C; Oxenham, M; Lam, K S

2017-06-01

In the United Kingdom, lower incidences of intraspinal abnormalities in patients with early onset idiopathic scoliosis have been observed than in studies in other countries. We aimed to determine the rates of these abnormalities in United Kingdom patients diagnosed with idiopathic scoliosis before the age of 11 years. This retrospective study of patients attending an urban scoliosis clinic identified 71 patients satisfying a criteria of: clinical diagnosis of idiopathic scoliosis; age of onset ten years and 11 months or less; MRI screening for intraspinal abnormalities. United Kingdom census data combined with patient referral data was used to calculate incidence. Mean age at diagnosis was six years with 39 right-sided and 32 left-sided curves. Four patients (5.6%) were found to have intraspinal abnormalities on MRI. These consisted of: two combined Arnold-Chiari type 1 malformations with syrinx; one syrinx with a low lying conus; and one isolated syrinx. Overall annual incidence of early onset idiopathic scoliosis was one out of 182 000 (0.0006%). This study reports the lowest rates to date of intraspinal anomalies in patients with early onset idiopathic scoliosis, adding to knowledge regarding current incidences of these abnormalities as well as any geographical variation in the nature of the disease. Cite this article: Bone Joint J 2017;99-B:829-33. ©2017 The British Editorial Society of Bone & Joint Surgery.

Unusual Presentation of Chronic Idiopathic Thrombocytopenic Purpura

PubMed Central

Madhusudhanan, M.; Yusuff, Ali M.

2008-01-01

A snakebite victim presented with normal clotting profile and a low platelet count. A routine CBC in his past records (February 2004) showed a platelet count of 20,000/microlitre, but the patient was not symptomatic. We report a case of chronic idiopathic thrombocytopenic purpura, incidentally found in a patient presenting with snakebite. The patient also has acquired primary testicular failure. After the diagnosis the patient was on regular follow up. He caused trauma to the right external auditory canal and perforated his tympanic membrane. His left tympanic membrane was also scarred and retracted. Establishing a diagnosis of an ITP early is important so that the patient can take precaution to avoid undue trauma and monitor proper follow up. PMID:22567212

Comparison of CSF Distribution between Idiopathic Normal Pressure Hydrocephalus and Alzheimer Disease.

PubMed

Yamada, S; Ishikawa, M; Yamamoto, K

2016-07-01

CSF volumes in the basal cistern and Sylvian fissure are increased in both idiopathic normal pressure hydrocephalus and Alzheimer disease, though the differences in these volumes in idiopathic normal pressure hydrocephalus and Alzheimer disease have not been well-described. Using CSF segmentation and volume quantification, we compared the distribution of CSF in idiopathic normal pressure hydrocephalus and Alzheimer disease. CSF volumes were extracted from T2-weighted 3D spin-echo sequences on 3T MR imaging and quantified semi-automatically. We compared the volumes and ratios of the ventricles and subarachnoid spaces after classification in 30 patients diagnosed with idiopathic normal pressure hydrocephalus, 10 with concurrent idiopathic normal pressure hydrocephalus and Alzheimer disease, 18 with Alzheimer disease, and 26 control subjects 60 years of age or older. Brain to ventricle ratios at the anterior and posterior commissure levels and 3D volumetric convexity cistern to ventricle ratios were useful indices for the differential diagnosis of idiopathic normal pressure hydrocephalus or idiopathic normal pressure hydrocephalus with Alzheimer disease from Alzheimer disease, similar to the z-Evans index and callosal angle. The most distinctive characteristics of the CSF distribution in idiopathic normal pressure hydrocephalus were small convexity subarachnoid spaces and the large volume of the basal cistern and Sylvian fissure. The distribution of the subarachnoid spaces in the idiopathic normal pressure hydrocephalus with Alzheimer disease group was the most deformed among these 3 groups, though the mean ventricular volume of the idiopathic normal pressure hydrocephalus with Alzheimer disease group was intermediate between that of the idiopathic normal pressure hydrocephalus and Alzheimer disease groups. The z-axial expansion of the lateral ventricle and compression of the brain just above the ventricle were the common findings in the parameters for differentiating

Risk Factors for Neurodegeneration in Idiopathic REM sleep Behavior Disorder: A Multicenter Study

PubMed Central

Postuma, RB; Iranzo, A; Hogl, B; Arnulf, I; Ferini-Strambi, L; Manni, R; Miyamoto, T.; Oertel, W; Dauvilliers, Y; Ju, Y; Puligheddu, M; Sonka, K; Pelletier, A; Santamaria, J; Frauscher, B; Leu-Semenescu, S; Zucconi, M; Terzaghi, M; Miyamoto, M.; Unger, MM; Carlander, B; Fantini, ML; Montplaisir, JY

2018-01-01

Objective To assess whether risk factors for Parkinson’s disease and Dementia with Lewy bodies increase rate of defined neurodegenerative disease in idiopathic REM sleep behavior disorder Methods 12 centers administered a detailed questionnaire assessing risk factors for neurodegenerative synucleinopathy to patients with idiopathic REM sleep behavior disorder. Variables included demographics, lifestyle factors, pesticide exposures, occupation, co-morbid conditions, medication use, family history, and autonomic/motor symptoms. After 4-years follow-up, patients were assessed for dementia or parkinsonism. Disease risk was assessed with Kaplan-Meier analysis, and epidemiologic variables were compared between convertors and those still idiopathic using logistic regression. Results Of 305 patients, follow-up information was available for 279, of whom 93 (33.3%) developed defined neurodegenerative disease. Disease risk was 25% at 3 years, and 41% after 5 years. Patients who converted were older (difference=4.5 years, p<0.001), with similar sex distribution. Neither caffeine, smoking, nor alcohol exposure predicted conversion. Although occupation was similar between groups, those who converted had a lower likelihood of pesticide exposure (occupational insecticide=2.3% vs. 9.0%). Convertors were more likely to report family history of dementia (OR=2.09), without significant differences in Parkinson’s disease or sleep disorders. Medication exposures and medical history were similar between groups. Autonomic and motor symptoms were more common among those who converted. Risk factors for primary dementia and parkinsonism were generally similar, except for a notably higher clonazepam use in dementia convertors (OR=2.6). Interpretation Patients with idiopathic RBD are at very high risk of neurodegenerative synucleinopathy. Risk factor profiles between convertors and non-convertors have both important commonalities and differences. PMID:25767079

Intestinal Volvulus in Idiopathic Steatorrhea

PubMed Central

Warner, H. A.; Kinnear, D. G.; Cameron, D. G.

1963-01-01

Volvulus of the intestine has recently been observed in three patients with idiopathic steatorrhea in relapse. Two patients gave a history of intermittent abdominal pain, distension and obstipation. Radiographic studies during these attacks revealed obstruction at the level of the sigmoid colon. Reduction under proctoscopic control was achieved in one instance, spontaneous resolution occurring in the other. The third patient presented as a surgical emergency and underwent operative reduction of a small intestinal volvulus. Persistence of diarrhea and weight loss postoperatively led to further investigation and a diagnosis of idiopathic steatorrhea. In all cases, treatment resulted in clinical remission with a coincident disappearance of obstructive intestinal symptoms. The pathogenesis of volvulus in sprue is poorly understood. Atonicity and dilatation of the bowel and stretching of the mesentery likely represent important factors. The symptoms of recurrent abdominal pain and distension in idiopathic steatorrhea necessitate an increased awareness of intestinal volvulus as a complication of this disease. ImagesFig. 1Fig. 2Fig. 3Figs. 4 and 5Fig. 6 PMID:13998948

Value of routine timed barium esophagram follow-up in achalasia after myotomy.

PubMed

Kachala, Stefan S; Rice, Thomas W; Baker, Mark E; Rajeswaran, Jeevanantham; Thota, Prashanthi N; Murthy, Sudish C; Blackstone, Eugene H; Zanoni, Andrea; Raja, Siva

2018-03-08

The value of routine timed barium esophagram (TBE) in longitudinal follow-up of achalasia after Heller myotomy is unknown. We prospectively prescribed a yearly follow-up TBE. Purposes were to characterize esophageal emptying over time after myotomy, identify preoperative TBE measures associated with follow-up TBE, and characterize follow-up TBE over time in relationship to reintervention. From March 1995 to April 2013, 635 patients underwent Heller myotomy for achalasia; 559 had at least 1 follow-up TBE. Temporal trends of 1335 follow-up TBEs in all nonreintervention and reintervention patients were assessed. Multivariable longitudinal analysis identified preoperative TBE measures associated with follow-up TBE. On average, TBE height and width at 1 and 5 minutes decreased approximately 50% and 60%, respectively, at first postoperative follow-up, and remained stable or slightly decreased for up to 5 years. Wider TBE width at 5 minutes was associated with greater follow-up TBE height and width at 1 minute. Of 118 patients undergoing reintervention, 64 (57%) had only 1 reintervention, with follow-up TBE returning to that of nonreintervention patients. Patients whose follow-up TBE remained abnormal underwent a further reintervention, some normalizing on subsequent TBE, and some not. Follow-up TBE is valuable postmyotomy, particularly if there is substantial esophageal dilatation preoperatively. Follow-up TBE reassures patients with stable or decreasing TBE measures, permitting decreased follow-up intensity. Reintervention should not be considered a myotomy failure, because a successful, single, nonsurgical reintervention often results in long-term successful palliation. More than 1 reintervention requires intensification of TBE follow-up, facilitating treatment planning. Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Quality of Life Comparing Dor and Toupet After Heller Myotomy for Achalasia

PubMed Central

Tomasko, Jonathan M.; Augustin, Toms; Tran, Tung T.; Haluck, Randy S.; Rogers, Ann M.

2014-01-01

Background: Laparoscopic Heller cardiomyotomy (LHC) is standard therapy for achalasia. Traditionally, an antireflux procedure has accompanied the myotomy. This study was undertaken to compare quality-of-life outcomes between patients undergoing myotomy with Toupet versus Dor fundoplication. In addition, we investigated overall patient satisfaction after LHC in the treatment of achalasia. Methods: One hundred thirty-five patients who underwent LHC over a 13-year period were identified for inclusion. Symptoms queried included dysphagia, heartburn, and bloating using the Gastroesophageal Reflux Disease–Health-Related Quality of Life Scale and a second published scale for the assessment of gastroesophageal reflux disease and dysphagia symptoms. The patients' overall satisfaction after surgery was also rated. Data were compared on the basis of type of fundoplication. Symptom scores were analyzed using chi-square tests and Fisher's exact tests. Results: Sixty-three patients completed the survey (47%). There were no perioperative deaths or reoperations. The mean length of stay was 2.8 days. The mean operative time for LHC with Toupet fundoplication was 137.3 ± 30.91 minutes and for LHC with Dor fundoplication was 111.5 ± 32.44 minutes (P = .006). There was no difference with respect to the incidence or severity of postoperative heartburn, dysphagia, or bloating. Overall satisfaction with Toupet fundoplication was 87.5% and with Dor fundoplication was 93.8% (P > .999). Conclusions: LHC with either Toupet or Dor fundoplication gave excellent patient satisfaction. Postoperative symptoms of heartburn and dysphagia were equivalent when comparing LHC with either antireflux procedure. Dor and Toupet fundoplication were found to have equivalent outcomes in the short term. We prefer Dor to Toupet fundoplication because of its decreased need for extensive dissection and better mucosal protection. PMID:25392612

Nonsurgical Management of Adolescent Idiopathic Scoliosis.

PubMed

Gomez, Jaime A; Hresko, M Timothy; Glotzbecker, Michael P

2016-08-01

Pediatric patient visits for spinal deformity are common. Most of these visits are for nonsurgical management of scoliosis, with approximately 600,000 visits for adolescent idiopathic scoliosis (AIS) annually. Appropriate management of scoliotic curves that do not meet surgical indication parameters is essential. Renewed enthusiasm for nonsurgical management of AIS (eg, bracing, physical therapy) exists in part because of the results of the Bracing in Adolescent Idiopathic Scoliosis Trial, which is the only randomized controlled trial available on the use of bracing for AIS. Bracing is appropriate for idiopathic curves between 20° and 40°, with successful control of these curves reported in >70% of patients. Patient adherence to the prescribed duration of wear is essential to maximize the effectiveness of the brace. The choice of brace type must be individualized according to the deformity and the patient's personality as well as the practice setting and brace availability.

Peroral endoscopic myotomy for esophageal achalasia: outcomes of the first over 100 patients with short-term follow-up.

PubMed

Shiwaku, Hironari; Inoue, Haruhiro; Yamashita, Kanefumi; Ohmiya, Toshihiro; Beppu, Richiko; Nakashima, Ryo; Takeno, Shinsuke; Sasaki, Takamitsu; Nimura, Satoshi; Yamashita, Yuichi

2016-11-01

POEM is a recently developed achalasia treatment method, which combines the efficacy of surgical myotomy with the benefits of endoscopic procedures. Very few studies have focussed on the efficacy and usefulness of POEM in over 100 cases. The first 100 adult patients treated according to standard POEM technique in a single center and followed up for 3 months were identified and included in this study (men 42; women 58; mean age 48.2 ± 18.8; range 9-91 years) The pre- and postoperative assessments included Eckardt scores, manometry, endoscopy, and monitoring pH. Mean operative time was 150.8 ± 49.3 min (75-370 min). Adverse events over Grade IIIb by the Clavien-Dindo classification were not encountered. The mean preoperative and postoperative lower esophageal sphincter pressures were 43.6 ± 26.2 and 20.9 ± 12.7 mmHg, respectively, indicating a statistically significant decrease after POEM (P < 0.05). The mean preoperative and postoperative integrated relaxation pressure values were 33.5 ± 15.2 and 15.3 ± 6.5 mmHg (P < 0.05). Furthermore, the Eckardt score significantly decreased from 5.9 ± 2.5 preoperatively to 0.8 ± 0.9 postoperatively (P < 0.05). Clinical success was documented in 99 % of the patients. Short-term outcomes after POEM were extremely good and independent of the age, type of achalasia, and previous treatment method. The percent of monitoring time with a pH < 4 was 25.6 % (22/86) in patients not on proton-pump inhibitors. Nine patients were diagnosed with symptomatic gastroesophageal reflux disease (GERD); patients with reflux esophagitis and symptomatic GERD improved with PPI treatment. Our results confirm the efficacy of POEM in a large patient series and support POEM as one of the first-line achalasia therapies in the near future.

Occurrence of idiopathic pulmonary fibrosis during immunosuppressive treatment: a case report.

PubMed

Cerri, Stefania; Sgalla, Giacomo; Richeldi, Luca; Luppi, Fabrizio

2016-05-25

Immunosuppressive therapy has been-until the recent release of new guidelines on diagnosis and management-the recommended treatment for idiopathic pulmonary fibrosis. However, its efficacy in patients with idiopathic pulmonary fibrosis has always been a matter of debate. We report the occurrence of idiopathic pulmonary fibrosis in a white man receiving chronic immunosuppressive treatment following a heart transplant. This case report suggests that the immune mechanisms targeted by azathioprine and cyclosporine do not play a role in the pathogenesis of idiopathic pulmonary fibrosis.

Per-oral endoscopic myotomy for achalasia. Are results comparable to laparoscopic Heller myotomy?

PubMed

Kumagai, Koshi; Tsai, Jon A; Thorell, Anders; Lundell, Lars; Håkanson, Bengt

2015-05-01

Per-oral endoscopic myotomy (POEM) has recently been introduced as a minimal invasive alternative to conventional treatment for achalasia. This study aimed to clarify the feasibility and the short-term clinical efficacy of POEM as compared to laparoscopic Heller myotomy (LHM). Treatment outcomes were prospectively recorded and compared between the procedures in a nonrandomized fashion. Reduction rate (RR) in timed barium esophagogram (TBE) was calculated at 1, 2 and 5 min after barium ingestion as: RR = 1- postoperative barium height/preoperative barium height. Risk factors for treatment failure defined as the proportion of patients with RR <0.5 (1 min) and gastroesophageal reflux (GER) after POEM were analyzed. Forty-two consecutive patients who underwent POEM were compared to 41 patients who had a LHM during the immediate time period prior to the introduction of POEM. Ninety percent of the cases reported complete symptom relief after POEM. The percentage of esophageal emptying and RR in TBE improved dramatically by both procedures without significant difference. A longer operation time (odds ratio [OR] 32.80, 95%CI 2.99-359.82, p = 0.004) and younger age (OR 26.81, 95%CI 2.09-344.03, p = 0.012) were the independent predictors of treatment failure after POEM. GER was observed in seven patients where previous dilatation (OR 8.59, 95%CI 1.16-63.45, p = 0.035) and higher body mass index (OR 8.69, 95%CI 1.13-66.63, p = 0.037) were the independent predictors for symptomatic GER after POEM. POEM seems to be a safe and effective treatment option for achalasia in the short-term perspective; an effect well comparable to LHM.

Valuing innovative endoscopic techniques: per-oral endoscopic myotomy for the management of achalasia.

PubMed

Shah, Eric D; Chang, Andrew C; Law, Ryan

2018-04-20

Unclear reimbursement for new and innovative endoscopic procedures can limit adoption in clinical practice despite effectiveness in clinical trials. The aim of this study was to determine maximum cost-effective reimbursement for per-oral endoscopic myotomy (POEM) in treating achalasia. We constructed a decision analytic model assessing POEM versus laparoscopic Heller myotomy with Dor fundoplication (LHM) in managing achalasia from a payer perspective over a 1-year time horizon. Reimbursement data were derived from 2017 Medicare data. Responder rates were based on clinically meaningful improvement in validated Eckardt scores. Validated health utility values were assigned to terminal health states based on data previously derived with a standard gamble technique. Contemporary willingness-to-pay (WTP) levels per quality-adjusted life year (QALY) were used to estimate maximum reimbursement for POEM using threshold analysis. Effectiveness of POEM and LHM was similar at one year of follow-up (0.91 QALY). Maximum cost-effective reimbursement for POEM was $1,200.07 to $1,389.85 (33.4-38.7 total 2017 RVUs). This compares to contemporary total reimbursement of 10-15 total RVU for advanced endoscopic procedures. The model was most sensitive to the probability of GERD after procedure. The rate of conversion to open laparotomy due to perforation or bleeding was infrequent in published clinical practice experience, thus did not significantly affect reimbursement. POEM is an example of an innovative and potentially disruptive endoscopic technique offering greater cost-effective value and similar outcomes to the established surgical standard at contemporary reimbursement levels. Copyright © 2018 American Society for Gastrointestinal Endoscopy. Published by Elsevier Inc. All rights reserved.

Therapeutic Options in Idiopathic Burning Mouth Syndrome: Literature Review

PubMed Central

Miziara, Ivan; Chagury, Azis; Vargas, Camila; Freitas, Ludmila; Mahmoud, Ali

2014-01-01

Introduction Burning mouth syndrome (BMS) is characterized by a burning sensation in the tongue, palate, lips, or gums of no well-defined etiology. The diagnosis and treatment for primary BMS are controversial. No specific laboratory tests or diagnostic criteria are well established, and the diagnosis is made by excluding all other possible disorders. Objective To review the literature on the main treatment options in idiopathic BMS and compare the best results of the main studies in 15 years. Data Synthesis We conducted a literature review on PubMed/MEDLINE, SciELO, and Cochrane-BIREME of work in the past 15 years, and only selected studies comparing different therapeutic options in idiopathic BMS, with preference for randomized and double-blind controlled studies. Final Comments Topical clonazepam showed good short-term results for the relief of pain, although this was not presented as a definitive cure. Similarly, α-lipoic acid showed good results, but there are few randomized controlled studies that showed the long-term results and complete remission of symptoms. On the other hand, cognitive therapy is reported as a good and lasting therapeutic option with the advantage of not having side effects, and it can be combined with pharmacologic therapy. PMID:25992157

Idiopathic Pulmonary Fibrosis: Clinically Meaningful Primary Endpoints in Phase 3 Clinical Trials

PubMed Central

Collard, Harold R.; Anstrom, Kevin J.; Flaherty, Kevin R.; Fleming, Thomas R.; King, Talmadge E.; Martinez, Fernando J.; Brown, Kevin K.

2012-01-01

Definitive evidence of clinical efficacy in a Phase 3 trial is best shown by a beneficial impact on a clinically meaningful endpoint—that is, an endpoint that directly measures how a patient feels (symptoms), functions (the ability to perform activities in daily life), or survives. In idiopathic pulmonary fibrosis (IPF), we believe the endpoints that best meet these criteria are all-cause mortality and all-cause nonelective hospitalization. There are no validated measures of symptoms or broader constructs such as health status or funtional status in IPF. A surrogate endpoint is defined as an indirect measure that is intended to substitute for a clinically meaningful endpoint. Surrogate endpoints can be appropriate outcome measures if validated. However, validation requires substantial evidence that the effect of an intervention on a clinically meaningful endpoint is reliably predicted by the effect of an intervention on the surrogate endpoint. For patients with IPF, there are currently no validated surrogate endpoints. PMID:22505745

IgG abnormality in narcolepsy and idiopathic hypersomnia.

PubMed

Tanaka, Susumu; Honda, Makoto

2010-03-05

A close association between narcolepsy and the Human Leukocyte Antigen (HLA)-DQB1*0602 allele suggests the involvement of the immune system, or possibly an autoimmune process. We investigated serum IgG levels in narcolepsy. We measured the serum total IgG levels in 159 Japanese narcolepsy-cataplexy patients positive for the HLA-DQB1*0602 allele, 28 idiopathic hypersomnia patients with long sleep time, and 123 healthy controls (the HLA-DQB1*0602 allele present in 45 subjects). The serum levels of each IgG subclass were subsequently measured. The distribution of serum IgG was significantly different among healthy controls negative for the HLA-DQB1*0602 allele (11.66+/-3.55 mg/ml), healthy controls positive for the HLA-DQB1*0602 allele (11.45+/-3.43), narcolepsy patients (9.67+/-3.38), and idiopathic hypersomnia patients (13.81+/-3.80). None of the following clinical variables, age, disease duration, Epworth Sleepiness Scale, smoking habit and BMI at the time of blood sampling, were associated with IgG levels in narcolepsy or idiopathic hypersomnia. Furthermore we found the decrease in IgG1 and IgG2 levels, stable expression of IgG3, and the increase in the proportion of IgG4 in narcolepsy patients with abnormally low IgG levels. The increase in the proportion of IgG4 levels was also found in narcolepsy patients with normal serum total IgG levels. Idiopathic hypersomnia patients showed a different pattern of IgG subclass distribution with high IgG3 and IgG4 level, low IgG2 level, and IgG1/IgG2 imbalance. Our study is the first to determine IgG abnormalities in narcolepsy and idiopathic hypersomnia by measuring the serum IgG levels in a large number of hypersomnia patients. The observed IgG abnormalities indicate humoral immune alterations in narcolepsy and idiopathic hypersomnia. Different IgG profiles suggest immunological differences between narcolepsy and idiopathic hypersomnia.

Long-term results of pneumatic dilatation for relapsing symptoms of achalasia after Heller myotomy.

PubMed

Legros, Ludivine; Ropert, Alain; Brochard, Charlène; Bouguen, Guillaume; Pagenault, Maël; Siproudhis, Laurent; Bretagne, Jean-François

2014-09-01

The aim of this study was to assess the efficacy and safety of pneumatic dilatation (PD) to treat symptom recurrence after Heller myotomy (HM). Consecutive patients receiving PD for relapsing symptoms following prior HM were included in this retrospective single-center study. Eckardt score ≤3 and/or ∆ Eckardt (difference between Eckardt score before and after dilatation) ≥3 defined the success of initial dilatation. Patients who maintained response longer than 2 months after initial dilatation were defined as short-term responders. Relapsers were offered further on-demand dilatation. Remission was defined as an Eckardt score ≤3 at the study endpoint. Kaplan-Meier survival curves were used to determine relapse rates. Eighteen patients (11 women, seven men) were included from January 2004 to January 2013. Ten patients had type I achalasia, and seven had type III, according to the Chicago classification. Thirty-nine PDs were performed (1.5 [1-2.25] per patient). All patients had short-term responses. The remission rate at the endpoint, after a median follow-up of 33 months, was 78%, but 44% were treated with on-demand PD during the follow-up interval. The proportions of patients without relapse and subsequent PD were 72% at 12 months, 65% at 24 and 36 months, and 49% at 48 months. No factors predictive of long-term response, particularly the type of achalasia, could be identified in this series. There were no perforations. In treating symptom recurrence following HM, PD was safe and effective over the long term when combined with subsequent PD. © 2014 John Wiley & Sons Ltd.

Pathology of idiopathic non-cirrhotic portal hypertension.

PubMed

Guido, Maria; Sarcognato, Samantha; Sacchi, Diana; Colloredo, Guido

2018-04-12

Idiopathic non-cirrhotic portal hypertension is an under-recognized vascular liver disease of unknown etiology, characterized by clinical signs of portal hypertension in the absence of cirrhosis. By definition, any disorder known to cause portal hypertension in the absence of cirrhosis and any cause of chronic liver disease must be excluded to make a diagnosis of idiopathic non-cirrhotic portal hypertension. However, the diagnosis is often difficult because the disease resembles cirrhosis and there is no gold standard test. Liver biopsy is an essential tool: it is able to exclude cirrhosis and other causes of portal hypertension and it allows the identification of the characteristic lesions. Nonetheless, the histological diagnosis of idiopathic non-cirrhotic portal hypertension is not always straightforward, in particular by needle biopsy samples, because there is no pathognomonic lesion, but rather a variety of vascular changes which are unevenly distributed, very subtle, and not all necessarily identified in a single specimen. Pathologists should be able to recognize several patterns of injury, involving portal/periportal areas as well as parenchymal structures.The histological features of idiopathic non-cirrhotic portal hypertension are described in this review, focusing on their interpretation in needle biopsy specimens.

The role of laparoscopic Heller myotomy in the treatment of achalasia.

PubMed

Zonca, P; Cambal, M; Labas, P; Hrbaty, B; Jacobi, C A

2014-01-01

To evaluate the results of laparoscopic Heller myotomy in our group of patients. A retrospective clinical trial was carried out to evaluate the indication, technique and controversies of laparoscopic Heller myotomy in the achalasia treatment. The following symptoms were evaluated prior and after Heller myotomy: dysphagia, heartburn, nausea/vomiting after meal and asthma/coughing. The patients were evaluated by the use of Likert score. Statistical analysis was performed by using Student t test. The intra-operative (operation time, intraoperative complications, blood loss, conversion rate), and peri-operative parameters (morbidity, mortality, hospital stay) were evaluated as well. The patients who underwent laparoscopic Heller myotomy were included in the trial. All patients were perioperatively managed by a multidisciplinary team. The evaluation of fourteen patients was performed (average age: 53.2 yrs., eleven men, two women, BMI 23.6 kg/m(2)). The patients were indicated for surgery in all of the stages (I-III). Previous semiconservative therapeutic modalities were performed in thirteen patients. The standard laparoscopic technique for Heller myotomy with semifundoplication was applied. All the observed symptoms were statistically improved after the surgery (p=0.05). The average operating time was 89 minutes. Intraoperative blood loss was below 20 ml. There was no conversion to open surgery. An average hospital stay was 4.3 days. Morbidity was 14.3 % and mortality 0 %. In one patient esophageal mucosa perforation was intra-operatively identified and sutured. Post-operative course in this patient was without any complications. The laparoscopic Heller myotomy has become the "gold standard" procedure for achalasia. It is an excellent method allowing precise operation technique with good visualization of the esophagogastric junction. The operation with this approach is safe, efficient, and with excellent reproducible operative results. The correct and early indication

[Adolescent idiopathic scoliosis].

PubMed

2016-12-01

Adolescent idiopathic scoliosis is a 3D spinal deformity in frontal, sagittal and axial planes, with high relevance in the pediatric population especially in adolescents and females between 10 years of age and the end of growth spurt and skeletal maturity. The radiographic manifestation is a curve greater than 10° measured by Cobb method associated with vertebral rotation. "Idiopathic" diagnosis has to be done after neuroanatomical anomalies of the posterior cerebral fosa and spinal canal have been ruled out. The physical finding of a thoracic or lumbar hump is the clinical manifestation of vertebral rotation seen in a forward bending test (Adam's Test). It is recommended that all curves with a magnitude greater than 20° have to be controlled and treated by a spinal surgeon being observation, bracing and surgery the different treatment options based on the extent, progression of deformity and basically the clinical condition of the patient. Sociedad Argentina de Pediatría.

Idiopathic pulmonary fibrosis.

PubMed

Xaubet, Antoni; Ancochea, Julio; Molina-Molina, María

2017-02-23

Idiopathic pulmonary fibrosis is a fibrosing interstitial pneumonia associated with the radiological and/or histological pattern of usual interstitial pneumonia. Its aetiology is unknown, but probably comprises the action of endogenous and exogenous micro-environmental factors in subjects with genetic predisposition. Its diagnosis is based on the presence of characteristic findings of high-resolution computed tomography scans and pulmonary biopsies in absence of interstitial lung diseases of other aetiologies. Its clinical evolution is variable, although the mean survival rate is 2-5 years as of its clinical presentation. Patients with idiopathic pulmonary fibrosis may present complications and comorbidities which modify the disease's clinical course and prognosis. In the mild-moderate disease, the treatment consists of the administration of anti-fibrotic drugs. In severe disease, the best therapeutic option is pulmonary transplantation. In this paper we review the diagnostic and therapeutic aspects of the disease. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

CLINICAL CHARACTERISTICS OF IDIOPATHIC FOVEOMACULAR RETINOSCHISIS.

PubMed

Maruko, Ichiro; Morizane, Yuki; Kimura, Shuhei; Shiode, Yusuke; Hosokawa, Mio; Sekiryu, Tetsuju; Iida, Tomohiro; Shiraga, Fumio

2016-08-01

To describe the clinical features of idiopathic foveomacular retinoschisis not in association with myopia, glaucoma, optic disk pit, or juvenile retinoschisis. Retrospective observational case series. Five eyes of five patients with idiopathic foveomacular retinoschisis were included. The patients were 2 men and 3 women (average age, 75.2 years; range, 71-78 years). The average spherical equivalent was +2.40 diopters (range, +0.88 to +5.75 diopters), and the average axial length was 22.0 mm (range, 21.1-23.1 mm). All patients had retinoschisis from the macula to the optic disk in the affected eye. No patients had retinoschisis in the fellow eye. The average best-corrected visual acuity was 20/44 (68 Early Treatment Diabetic Retinopathy Study letter score). Idiopathic foveomacular retinoschisis is not inherited or associated with myopia, vitreomacular traction syndrome, optic pit, or glaucoma but is associated with older age, unilaterality, hyperopia with short axial length, complete posterior vitreous detachment, and weak leakage from the optic disk on fluorescein angiography.

Optimal management of idiopathic scoliosis in adolescence

PubMed Central

Kotwicki, Tomasz; Chowanska, Joanna; Kinel, Edyta; Czaprowski, Dariusz; Tomaszewski, Marek; Janusz, Piotr

2013-01-01

Idiopathic scoliosis is a three-dimensional deformity of the growing spine, affecting 2%–3% of adolescents. Although benign in the majority of patients, the natural course of the disease may result in significant disturbance of body morphology, reduced thoracic volume, impaired respiration, increased rates of back pain, and serious esthetic concerns. Risk of deterioration is highest during the pubertal growth spurt and increases the risk of pathologic spinal curvature, increasing angular value, trunk imbalance, and thoracic deformity. Early clinical detection of scoliosis relies on careful examination of trunk shape and is subject to screening programs in some regions. Treatment options are physiotherapy, corrective bracing, or surgery for mild, moderate, or severe scoliosis, respectively, with both the actual degree of deformity and prognosis being taken into account. Physiotherapy used in mild idiopathic scoliosis comprises general training of the trunk musculature and physical capacity, while specific physiotherapeutic techniques aim to address the spinal curvature itself, attempting to achieve self-correction with active trunk movements developed in a three-dimensional space by an instructed adolescent under visual and proprioceptive control. Moderate but progressive idiopathic scoliosis in skeletally immature adolescents can be successfully halted using a corrective brace which has to be worn full time for several months or until skeletal maturity, and is able to prevent more severe deformity and avoid the need for surgical treatment. Surgery is the treatment of choice for severe idiopathic scoliosis which is rapidly progressive, with early onset, late diagnosis, and neglected or failed conservative treatment. The psychologic impact of idiopathic scoliosis, a chronic disease occurring in the psychologically fragile period of adolescence, is important because of its body distorting character and the onerous treatment required, either conservative or surgical

Dynamic, diagnostic, and pharmacological radionuclide studies of the esophagus in achalasia: correlation with manometric measurements

DOE Office of Scientific and Technical Information (OSTI.GOV)

Rozen, P.; Gelfond, M.; Zaltzman, S.

1982-08-01

The esophagus was evaluated in 15 patients with achalasia by continuous gamma camera imaging following ingestion of a semi-solid meal labeled with /sup 99//sup m/Tc. The images were displayed and recorded on a simple computerized data processing/display system. Subsequent cine' mode images of esophagela emptying demonstrated abnormalities of the body of the esophagus not reflected by the manometric examination. Computer-generated time-activity curves representing specific regions of interest were better than manometry in evaluating the results of myotomy, dilatation, and drug therapy. Isosorbide dinitrate significantly improved esophageal emptying.

Clinical characteristics of aldosterone-producing microadenoma, macroadenoma, and idiopathic hyperaldosteronism in 93 patients with primary aldosteronism.

PubMed

Omura, Masao; Sasano, Hironobu; Saito, Jun; Yamaguchi, Kunio; Kakuta, Yukio; Nishikawa, Tetsuo

2006-11-01

Primary aldosteronism (PA) due to aldosterone-producing adenoma (APA) is a form of surgically curable secondary hypertension, and distinguishing APA from idiopathic hyperaldosteronism (IHA) is important for treatment. We made a differential diagnosis between APA and IHA using imaging tests such as adrenal CT and MRI as well as adrenal venous sampling (AVS) in all 93 cases of PA presenting at our institutions over the last decade. We identified 27 patients with aldosterone-producing microadenoma (APmicroA), all of whom could be diagnosed by AVS but not by the imaging tests. Then, we compared the clinical and roent-genological findings of these 27 patients with those of 42 patients with aldosterone-producing macroadenoma (APmacroA) and of 24 patients with IHA. Using surgically removed adrenal tissues, histopathological examinations and immunohistochemical analyses of steroidogenic enzymes were conducted. The findings for APmicroA were similar to those for APmacroA, except with respect to the diameter of the adrenal adenomas. Endocrinological and roentgenological findings for APmicroA were similar to those for IHA, but not to those for APmacroA. The rate of cure of hypertension was much greater in patients with APmicroA than in patients with APmacroA after the unilateral adrenalectomy (odds ratio, 4.0; p=0.028). In conclusion, it is important to accurately diagnose APmicroA, in which the laterality of the hyperproduction of aldosterone is only detectable by AVS, and to treat these patients by unilateral adrenalectomy in order to avoid long-term medical treatment and prevent hypertensive vascular complications.

History of primary vasculitis in Latin America.

PubMed

Iglesias Gammara, Antonio; Coral, Paola; Quintana, Gerardo; Toro, Carlos E; Flores, Luis Felipe; Matteson, Eric L; Restrepo, José Félix

2010-03-01

A literature review utilizing Fepafem, Bireme, LiLacs, Scielo Colombia, Scielo Internacional, former MedLine, Pubmed, and BVS Colombia as well as manual searches in the libraries of major Latin American universities was performed to study vasculitis in Latin America. Since 1945, a total of 752 articles have been published by Latin American authors. However, only a minority are devoted to primary vasculitides, and even fewer have been published in indexed journals. Approximately 126 are in OLD, Medline, Pubmed, Bireme, and Scielo. Most publications are from Mexico, followed by Brazil and Colombia. Systematic studies of the epidemiology of primary idiopathic vasculitis are available for a few countries, i.e. Brazil, Mexico, Colombia, Chile, and Peru. Takayasu arteritis and ANCA-associated vasculitis are the best studied forms of vasculitis in Latin America. Interest and expertise in vasculitis is growing in Latin America, as reflected in the increased number of published articles from this region of the world in the last decade. Racial and environmental factors are possibly responsible for the differential expression of various types of primary vasculitis observed in Latin America. With time, the unique features, epidemiology, and better treatment strategies for idiopathic vasculitides in Latin America will emerge.

Treatment of the idiopathic scoliosis with brace and physiotherapy.

PubMed

Hundozi-Hysenaj, Hajrije; Dallku, Iliriana Boshnjaku; Murtezani, Ardiana; Rrecaj, Shkurte

2009-01-01

Scoliosis is a three-dimensional deformation of the spine with a lateral curvature or deviation greater than 10 degrees and associated with vertebral rotation. Many conservative treatments are available for adolescents with idiopathic scoliosis, but the evidence for their effectiveness is still questioned. The objective of this study was to define the effectiveness of braces and individual physiotherapy for the comprehensive treatment of idiopathic scoliosis in adolescents. A retrospective study of 57 children with idiopathic thoracic dextroscoliosis with the magnitude of the thoracic curve between 20 degrees-35 degrees, treated in Orthopedic and Physiatrist Clinic as well as National Ortho-prosthetic Center within University Clinical Center of Kosova in Prishtina, during the period of 2003-2006. Inclusion of kinesitherapy in the comprehensive management of idiopathic scoliosis varied in the improvement of the muscle strength (satisfied and moderate) in almost 80% of the children while the correction of the curve was small in approximately 42.1% of cases. For children with idiopathic scoliosis, who require braces, an exercise program helps chest mobility, muscle strength, proper breathing flexibility in the spine, correct posture and keeps muscles in tone so that the transition period after brace removal is easier.

Acute exacerbation of idiopathic pulmonary fibrosis triggered by Aspergillus empyema.

PubMed

Suzuki, Atsushi; Kimura, Tomoki; Kataoka, Kensuke; Matsuda, Toshiaki; Yokoyama, Toshiki; Mori, Yuta; Kondoh, Yasuhiro

2018-01-01

Acute exacerbation (AE) is a severe and life-threatening complication of idiopathic pulmonary fibrosis (IPF). In 2016, the definition and diagnostic criteria for AE-IPF were updated by an international working group. The new definition includes any acute, clinically significant respiratory deterioration (both idiopathic and triggered events) characterized by evidence of new widespread alveolar abnormality in patients with IPF. There are no currently proven beneficial management strategies for idiopathic and triggered AE-IPF. This is the first report describing AE-IPF triggered by Aspergillus empyema, which was improved by a combination of corticosteroid, systemic antifungal therapy, local antifungal therapy, and additional pharmacological therapies. Future research may reveal optimal strategies for both idiopathic and triggered AE-IPF.

Idiopathic intracranial hypertension and sickle cell disease: two case reports.

PubMed

Segal, Laura; Discepola, Marino

2005-12-01

Two patients with sickle cell disease presented with headaches and visual disturbances, typical complaints of this disorder. However, prompt diagnosis of idiopathic intracranial hypertension and initiation of medical therapy lead to improved symptoms and restored vision. Ophthalmologists should consider sickle cell disease to be an independent risk factor for idiopathic intracranial hypertension when a patient is being assessed for visual disturbances. Although a rare condition, idiopathic intracranial hypertension has several key signs useful in establishing a diagnosis. It is critical to recognize the warning signs and symptoms to prevent devastating ophthalmologic complications. We report the first cases of idiopathic intracranial hypertension in patients with the novel Quebec-Chori beta-chain variant of sickle cell disease.

Guidelines for the medical treatment of idiopathic pulmonary fibrosis.

PubMed

Xaubet, Antoni; Molina-Molina, María; Acosta, Orlando; Bollo, Elena; Castillo, Diego; Fernández-Fabrellas, Estrella; Rodríguez-Portal, José Antonio; Valenzuela, Claudia; Ancochea, Julio

2017-05-01

Idiopathic pulmonary fibrosis is defined as chronic fibrosing interstitial pneumonia limited to the lung, with poor prognosis. The incidence has been rising in recent years probably due to improved diagnostic methods and increased life expectancy. In 2013, the SEPAR guidelines for the diagnosis and treatment for idiopathic pulmonary fibrosis were published. Since then, clinical trials and meta-analyses have shown strong scientific evidence for the use of pirfenidone and nintedanib in the treatment of idiopathic pulmonary fibrosis. In 2015, the international consensus of 2011 was updated and new therapeutic recommendations were established, prompting us to update our recommendation for the medical treatment of idiopathic pulmonary fibrosis accordingly. Diagnostic aspects and non-pharmacological treatment will not be discussed as no relevant developments have emerged since the 2013 guidelines. Copyright © 2017 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.

Treatment of oropharyngeal dysphagia secondary to idiopathic cricopharyngeal bar: Surgical cricopharyngeal muscle myotomy versus dilation.

PubMed

Marston, Alexander P; Maldonado, Francisco J; Ravi, Karthik; Kasperbauer, Jan L; Ekbom, Dale C

To compare swallowing outcomes following cricopharyngeal (CP) dilation versus surgical myotomy in patients with dysphagia secondary to idiopathic CP bar. All patients had an idiopathic CP bar without a history of Zenker's diverticulum, head and neck cancer, or systemic neurologic disease treated between 2000 and 2013. The Functional Outcome Swallowing Scale (FOSS) was utilized to assess dysphagia symptoms. Twenty-three patients underwent 46 dilations and 20 patients had a myotomy. Nineteen of 23 (83%) patients in the dilation group and all patients in the myotomy group reported improved swallow function. The median difference in pre- versus post-intervention FOSS scores was not statistically significant (p=0.07) between the dilation and myotomy groups with mean reductions of 1.3 and 1.8, respectively. Seventeen of 23 (74%) dilation patients had persistent or recurrent dysphagia with 13 (57%) requiring repeat dilation and 4 (17%) undergoing CP myotomy. The median time to first reintervention in the dilation group was 13.6months. Nineteen of 20 (95%) surgical myotomy patients did not experience recurrent dysphagia. Both endoscopic CP dilation and myotomy led to similar initial improvement in swallow function for patients with primary idiopathic CP bar; however, dilation is more likely to provide temporary benefit. Copyright © 2016 Elsevier Inc. All rights reserved.

Maternal asthma and idiopathic preterm labor.

PubMed

Kramer, M S; Coates, A L; Michoud, M C; Dagenais, S; Moshonas, D; Davis, G M; Hamilton, E F; Nuwayhid, B; Joshi, A K; Papageorgiou, A

1995-11-15

Previous studies suggest that women with asthma are at increased risk of preterm birth. Moreover, drugs (especially beta-agonists) used to treat asthma are also used to treat preterm labor. The authors carried out a case-control study of 555 women from three hospital centers with idiopathic preterm labor (< 37 weeks), including two overlapping (i.e., non-mutually exclusive) subsamples: cases with early idiopathic preterm labor (< 34 weeks) and cases with idiopathic recurrent preterm labor (< 37 weeks plus a previous history of preterm delivery or second-trimester miscarriage). Controls were matched to cases according to race and smoking history prior to and during pregnancy. All subjects responded in person to questions about atopic, respiratory, obstetric, and sociodemographic histories. Subjects in the early and recurrent preterm labor subsamples were also asked to undergo spirometric testing with methacholine challenge 6-12 weeks after delivery. Cases were significantly more likely to report histories of asthma symptoms and physician-diagnosed asthma (matched odds ratios of 2-3) than controls, particularly those cases with recurrent preterm labor. No significant associations were observed, however, with methacholine responsiveness. These results could not be explained by residual confounding by smoking or other variables, nor by selective recall of asthma symptoms and histories by cases. Women with asthma are at increased risk of idiopathic preterm labor. The fact that no such association was seen with methacholine responsiveness suggests that nonatopic, noncholinergic mechanisms may link bronchial and uterine smooth muscle lability.

Neutrophil to lymphocyte ratio predicts appropriate therapy in idiopathic dilated cardiomyopathy patients with primary prevention implantable cardioverter defibrillator

PubMed Central

Uçar, Fatih M.; Açar, Burak

2017-01-01

Objectives: To investigate whether an inflammatory marker of neutrophil to lymphocyte ratio (NLR) predicts appropriate implantable cardioverter defibrillator (ICD) therapy (shock or anti tachycardia pacing) in idiopathic dilated cardiomyopathy (IDC) patients. Methods: We retrospectively examined IDC patients (mean age: 58.3 ± 11.8 years, 81.5% male) with ICD who admitted to outpatient clinic for pacemaker control at 2 tertiary care hospitals in Ankara and Edirne, Turkey from January 2013-2015. All ICDs were implanted for primary prevention. Hematological and biochemical parameters were measured prior procedure. Results: Over a median follow-up period of 43 months (Range 7-125), 68 (33.1%) patients experienced appropriate ICD therapy. The NLR was increased in patients that received appropriate therapy (4.39 ± 2.94 versus 2.96 ± 1.97, p<0.001). To identify independent risk factors for appropriate therapy, a multivariate linear regression model was conducted and age (β=0.163, p=0.013), fasting glucose (β=0.158, p=0.017), C-reactive protein (CRP) (β=0.289, p<0.001) and NLR (β=0.212, p<0.008) were found to be independent risk factors for appropriate ICD therapy. Conclusions: Before ICD implantation by using NLR and CRP, arrhythmic episodes may be predictable and better antiarrhythmic medical therapy optimization may protect these IDC patients from unwanted events. PMID:28133686

Altered Regional Cerebral Blood Flow in Idiopathic Hypersomnia.

PubMed

Boucetta, Soufiane; Montplaisir, Jacques; Zadra, Antonio; Lachapelle, Francis; Soucy, Jean-Paul; Gravel, Paul; Dang-Vu, Thien Thanh

2017-10-01

Idiopathic hypersomnia is characterized by excessive daytime sleepiness, despite normal or long sleep time. Its pathophysiological mechanisms remain unclear. This pilot study aims at characterizing the neural correlates of idiopathic hypersomnia using single photon emission computed tomography. Thirteen participants with idiopathic hypersomnia and 16 healthy controls were scanned during resting wakefulness using a high-resolution single photon emission computed tomography scanner with 99mTc-ethyl cysteinate dimer to assess cerebral blood flow. The main analysis compared regional cerebral blood flow distribution between the two groups. Exploratory correlations between regional cerebral blood flow and clinical characteristics evaluated the functional correlates of those brain perfusion patterns. Significance was set at p < .05 after correction for multiple comparisons. Participants with idiopathic hypersomnia showed regional cerebral blood flow decreases in medial prefrontal cortex and posterior cingulate cortex and putamen, as well as increases in amygdala and temporo-occipital cortices. Lower regional cerebral blood flow in the medial prefrontal cortex was associated with higher daytime sleepiness. These preliminary findings suggest that idiopathic hypersomnia is characterized by functional alterations in brain areas involved in the modulation of vigilance states, which may contribute to the daytime symptoms of this condition. The distribution of regional cerebral blood flow changes was reminiscent of the patterns associated with normal non-rapid-eye-movement sleep, suggesting the possible presence of incomplete sleep-wake transitions. These abnormalities were strikingly distinct from those induced by acute sleep deprivation, suggesting that the patterns seen here might reflect a trait associated with idiopathic hypersomnia rather than a non-specific state of sleepiness. © Sleep Research Society 2017. Published by Oxford University Press on behalf of the Sleep

Brain MRI findings in patients with idiopathic hypersomnia.

PubMed

Trotti, Lynn Marie; Bliwise, Donald L

2017-06-01

Proper diagnosis of idiopathic hypersomnia necessitates the exclusion of neurologic or medical causes of sleepiness that better explain the clinical syndrome. However, there are no formal guidelines regarding the use of neuroimaging to identify such secondary causes of symptoms. We sought to characterize brain MRI findings in a series of patients with idiopathic hypersomnia. We reviewed medical records on a consecutive series of 61 patients diagnosed with idiopathic hypersomnia to determine the frequency and results of brain magnetic resonance imaging (MRI). One-third of patients had undergone brain MRI, with focal neurologic signs or symptoms being the most common indication for neuroimaging. Although seven patients had an identifiable finding on neuroimaging (e.g., chronic microvascular ischemic changes), clinical management was changed as a result of imaging in only three cases. In all three, the imaging finding was predated by clear clinical abnormalities. Neuroimaging may be a complementary part of an idiopathic hypersomnia evaluation, but the decision to pursue imaging should be made on a case-by-case basis. Copyright © 2017 Elsevier B.V. All rights reserved.

Robotic-assisted Heller myotomy versus laparoscopic Heller myotomy for the treatment of esophageal achalasia: a case-control study.

PubMed

Sánchez, Alexis; Rodríguez, Omaira; Nakhal, Elias; Davila, Hugo; Valero, Rair; Sánchez, Renata; Pena, Romina; Visconti, Maria F

2012-09-01

Minimally invasive surgery has become the gold standard for the treatment of achalasia. The incorporation of robotic technology can improve many limitations of laparoscopic surgery, through, for example, the availability of three-dimensional vision, increasing the degrees of movement, avoiding the fulcrum effect and optimizing ergonomics. The aim of this study was to compare robotic-assisted laparoscopic Heller myotomy (RAHM) with laparoscopic Heller myotomy (LHM) in terms of efficacy and safety. Thirty-one patients with diagnosis of achalasia confirmed by esophagogram and manometry were included. Dysphagia and weight loss were the main complaints in both groups. 18 patients were treated with LHM and 13 patients with RAHM. There was no difference in mean operative time (76 ± 13 vs. 79 ± 20 min; P = 0.73). Intraoperative complications were less frequent in the robotic-assisted procedures (5.5% vs. 0%); however, this was a non-significant difference. 94.5-100% of patients had relief of their symptoms. We conclude that RAHM is a safe and effective procedure. The operative time is no longer than in LHM, but it is necessary to evaluate the technique in randomized clinical trials to determine its advantages in terms of intraoperative complications.

Idiopathic granulomatous mastitis: an institutional experience

PubMed Central

Prasad, Seetharam; Jaiprakash, Padmapriya; Dave, Aniket; Pai, Deepti

2017-01-01

Objective To study idiopathic granulomatous mastitis with respect to its various clinical features, etiologic factors, treatment modalities and complications. Material and methods Retrospective study of all patients who were diagnosed with idiopathic granulomatous mastitis from 1st January 2006 to 31st December 2014 at Kasturba Hospital, Manipal, India (a tertiary care referral centre). The research was performed according to the World Medical Association Declaration of Helsinki. Informed consent was taken from the patient before invasive procedures including surgery. Data was analysed using the Statistical Package for Social Sciences version 16.0 wherever appropriate. Results 73 patients diagnosed with idiopathic granulomatous mastitis during the time period were included. One patient was a male (1.37%), rest were all females (98.63%). The mean age of presentation was 32.67 years (range 23 to 66 years). 70 patients (95.89%) were parous females. Average duration since last childbirth was 4.6 years (range: 3 months to 33 years). 8 patients (10.95%) were lactating. History of oral contraceptive pill use was present in 40 patients (54.79%). The right breast was affected in 44 patients (60.27%), and the left breast in 29 patients (39.73%). None of the patients had bilateral disease. The most common symptom was a painless lump (61.64%). Rest of the patients (38.36%) presented with features of a breast abscess. 19 out of 39 FNACs done (48.72%) were positive for granulomatous mastitis. 59 were primarily managed surgically (lumpectomy/wide excision-33, incision & drainage-26). One patient was treated primarily with prednisolone. 13 patients did not receive specific treatment, and were only kept on regular follow-up. Patients managed with lumpectomy/wide excision had the least rate of complications & recurrence (18.18%). Conclusion Patients with idiopathic granulomatous mastitis can present with a wide variety of symptoms which mimic other more common conditions. Surgical

Idiopathic granulomatous mastitis: a diagnostic dilemma for the breast radiologist.

PubMed

Sripathi, Smiti; Ayachit, Anurag; Bala, Archana; Kadavigere, Rajagopal; Kumar, Sandeep

2016-08-01

Idiopathic granulomatous mastitis is a chronic inflammatory disease of the breast, which is often difficult to differentiate both clinically and radiologically from infectious aetiologies such as tuberculosis, fungal infections, and also from malignancy, thus posing a diagnostic dilemma. We present a pictorial review of the commonly encountered imaging findings in idiopathic granulomatous mastitis on mammography and ultrasound. Mammographic and ultrasound findings of histopathologically proven cases of granulomatous mastitis are discussed. Idiopathic granulomatous mastitis has varied and non-specific appearances on ultrasound and mammography. Histopathology is essential to establish diagnosis. • Idiopathic granulomatous mastitis often poses a diagnostic dilemma for the radiologist by mimicking malignancy. • It has varied and non-specific appearances on mammography and ultrasound. • Histopathology is mandatory to establish the diagnosis and decide management.

A single institution's experience with more than 500 laparoscopic Heller myotomies for achalasia.

PubMed

Rosemurgy, Alexander S; Morton, Connor A; Rosas, Melissa; Albrink, Michael; Ross, Sharona B

2010-05-01

Long-term symptom relief and patient satisfaction after Heller myotomy are being reported. Herein, we report the largest experience of laparoscopic Heller myotomy for the treatment of achalasia. Since 1992, 505 patients have been prospectively followed after laparoscopic Heller myotomy. Until 2004, concomitant fundoplication was undertaken for a patulous hiatus, a large hiatal hernia, or to buttress the repair of an esophagotomy, then concomitant fundoplication became routinely applied. More recently, laparo-endoscopic single site (LESS) Heller myotomy has been performed when possible to improve cosmesis. Before and after myotomy, patients scored their symptoms. Before myotomy, 60% of patients underwent endoscopic therapy; of these patients, 27% had Botox (Allergan) therapy alone, 52% underwent dilation therapy alone, and 21% had both. Esophagotomy occurred in 7% of patients. Concomitant diverticulectomy was undertaken in 7%, fundoplication was performed in 59%, and LESS Heller myotomy was done in 12%. Median length of stay was 1 day. With mean follow-up at 31 months, the severity of all symptoms improved significantly. After myotomy, 95% experienced symptoms less than once per week, 86% believed their outcome is satisfying or better, and 92% would undergo myotomy again, if necessary. Symptoms after myotomy are similar with or without fundoplication and regardless of the laparoscopic approach used. Laparoscopic Heller myotomy safely and durably relieves symptoms of dysphagia. Confinement is short and satisfaction is very high. Relief of esophageal obstruction is paramount; the approach used or the application of a fundoplication has a lesser impact. Laparoscopic Heller myotomy, preferably with anterior fundoplication using a single site laparoscopic approach, is strongly encouraged for patients with symptomatic achalasia and is efficacious even after failures of dilation and/or Botox therapy. Copyright 2010 American College of Surgeons. Published by Elsevier Inc

Idiopathic scrotal elephantiasis.

PubMed

Hornberger, Brad J; Elmore, James M; Roehrborn, Claus G

2005-02-01

Scrotal lymphedema (scrotal elephantiasis) is a condition that has historically been described in areas endemic to filariasis. We present a unique case of a 22-year-old man with idiopathic lymphedema isolated to the scrotum. After acquired causes of lymphedema were ruled out, the patient was treated with scrotectomy and scrotal reconstruction.

Idiopathic diaphragmatic paralysis: Bell's palsy of the diaphragm?

PubMed

Crausman, Robert S; Summerhill, Eleanor M; McCool, F Dennis

2009-01-01

Idiopathic diaphragm paralysis is probably more common and responsible for more morbidity than generally appreciated. Bell's palsy, or idiopathic paralysis of the seventh cranial nerve, may be seen as an analogous condition. The roles of zoster sine herpete and herpes simplex have increasingly been recognized in Bell's palsy, and there are some data to suggest that antiviral therapy is a useful adjunct to steroid therapy. Thus, we postulated that antiviral therapy might have a positive impact on the course of acute idiopathic diaphragm paralysis which is likely related to viral infection. Three consecutive patients with subacute onset of symptomatic idiopathic hemidiaphragm paralysis were empirically treated with valacyclovir, 1,000 mg twice daily for 1 week. Prior to therapy, diaphragmatic function was assessed via pulmonary function testing and two-dimensional B-mode ultrasound, with testing repeated 1 month later. Diaphragmatic function pre- and post-treatment was compared to that of a historical control group of 16 untreated patients. All three subjects demonstrated ultrasound recovery of diaphragm function 4-6 weeks following treatment with valacyclovir. This recovery was accompanied by improvements in maximum inspiratory pressure (PI(max)) and vital capacity (VC). In contrast, in the untreated cohort, diaphragm recovery occurred in only 11 subjects, taking an average of 14.9 +/- 6.1 months (mean +/- SD). The results of this small, preliminary study suggest that antiviral therapy with valacyclovir may be helpful in the treatment of idiopathic diaphragm paralysis induced by a viral infection.

Idiopathic Hypersomnia and Hypersomnolence Disorder: A Systematic Review of the Literature.

PubMed

Sowa, Nathaniel A

2016-01-01

Hypersomnia is a common complaint in medical offices. Often patients are given psychiatric diagnoses, but a primary sleep disorder may be present. The new diagnosis of "hypersomnolence disorder" (HD) in the Diagnostic and Statistical Manual of Mental Disorders, fifth edition is a primary sleep disorder most similar to the diagnosis "idiopathic hypersomnia" (IH) in sleep literature and can be missed in psychiatric settings. A systematic review of the computerized databases PubMed, EMBASE, Web of Science, and Psychinfo using the search criteria "idiopathic AND (hypersomnolence OR hypersomnia)," as well as "hypersomnolence disorder was conducted." Articles were included if they were in English and included information regarding the epidemiology, diagnosis, pathophysiology, or treatment of IH or HD. Where relevant, weighted means and 95% CI were calculated based on the number of subjects in each study. A total of 143 articles discussed IH, whereas no articles were found regarding HD. Most articles were review articles, prospective studies, or studies of pathophysiology. IH is found in approximately 0.02%-0.010% of the general population, has a mean age of onset of 21.8 years, and is associated with several somatic symptoms. Alterations in histaminergic or dopaminergic signaling may be involved in IH. Treatment with modafinil or other stimulants appears moderately effective. IH can be differentiated from psychiatric hypersomnolence by formal polysomnography. IH and HD are relatively uncommon disorders and little is known about them. However, they are distinct from psychiatric disorders and respond well to treatment once properly identified. Copyright © 2016 The Academy of Psychosomatic Medicine. Published by Elsevier Inc. All rights reserved.

Outcome of peroral endoscopic myotomy in achalasia cardia: Experience with a new triangular knife.

PubMed

Nabi, Zaheer; Ramchandani, Mohan; Chavan, Radhika; Kalapala, Rakesh; Darisetty, Santosh; Reddy, D Nageshwar

2018-01-01

Peroral endoscopic myotomy (POEM) is a technically demanding procedure. Recently, a new triangular knife with integrated water jet function (TTJ) has been introduced. The present study was aimed to analyze and compare the operating time, efficacy, and adverse events (AEs) between the conventional (TT knife) and new knife (TTJ). All patients with achalasia cardia (AC) who underwent POEM between August 2015 and November 2016 were analyzed retrospectively. Operating time (OT), technical success, and AEs were assessed and compared between TT and TTJ knife. A total of 193 patients with AC underwent POEM during the specified period. Both groups had equivalent number of different subtypes of AC (I, II, and III). There was no difference in technical success between the two groups (TT, 99% vs TT, 98.9%). OT was significantly less in the TTJ group as compared to TT group (53.8 ± 15.2 vs 66.26 ± 19.2; P = 0.0001). On subanalysis, OT taken for submucosal tunneling was significantly less with TTJ knife (34.6 ± 10.1 vs 45.83 ± 14.80), whereas OT was similar for myotomy and clipping in both the groups. Significantly fewer use of coagulation forceps and exchanges of accessories were required in TTJ knife group (2.92 ± 1.77 vs 10.5 ± 3.58; P = 0.0001). There were no major AEs. Minor AEs were noted in 21.5% and 31% of patients in TTJ and TT knife groups, respectively. New triangular knife reduces procedure time and technical difficulty with POEM. POEM is an efficacious treatment for achalasia and can be safely executed in an endoscopy unit.

[Physical therapy for idiopathic scoliosis].

PubMed

Steffan, K

2015-11-01

The objective is the description and summary of the current state of idiopathic scoliosis treatment with physical therapy based on new scientific knowledge and concluded from more than 15 years of experience as a leading physician in two well-known clinics specializing in the conservative treatment of scoliosis. Based on current scientific publications on physical therapy in scoliosis treatment and resulting from the considerable personal experience gained working with conservative treatment and consulting scoliosis patients (as inpatients and outpatients), the current methods of physical therapy have been compared and evaluated. Physical therapy according to Schroth and Vojta therapy are at present the most common and effective methods in the physical treatment of idiopathic scoliosis. These methods can be applied during inpatient or outpatient treatment or intensified in the practice of specialized therapists. As there are only a few scientific studies on this subject, the author's findings are based mainly on his own experiences of the conservative treatment of idiopathic scoliosis. Athough these experiences are the results of over 15 years of working in the field of therapy, and the Schroth method in combination with corrective bracing presents highly promising results, it would nevertheless be desirable to conduct detailed scientific studies to verify the effectiveness of conservative treatment.

Laparoscopic Heller myotomy provides durable relief from achalasia and salvages failures after botox or dilation.

PubMed

Rosemurgy, Alexander; Villadolid, Desiree; Thometz, Donald; Kalipersad, Candice; Rakita, Steven; Albrink, Michael; Johnson, Milton; Boyce, Worth

2005-05-01

To report outcome after laparoscopic Heller myotomy in a large number of patients. Laparoscopic Heller myotomy has been undertaken for over a decade, but most studies involve small numbers of patients with limited follow-up. Since 1992, 262 patients have undergone laparoscopic Heller myotomy and been prospectively followed. Concomitant fundoplication was undertaken for a patulous hiatus or large hiatal hernia or to buttress the repair of an esophagotomy until recently when it became routinely applied. With mean follow-up at 32 months, symptoms were scored by patients on a Likert scale (frequency: 0 = Never to 10 = Every time I eat/always; severity: 0 = Not bothersome to 10 = Very bothersome). Before myotomy, 79% received Botox or bag dilation: 52% had Botox, 59% underwent dilation, and 36% had both. Inadvertent esophagotomy occurred in 5%. Concomitant diverticulectomy was undertaken in 4%, and fundoplication was undertaken in 30%. Complications were infrequent. Median length of stay was 1 day. After myotomy, the frequency and severity of symptoms of achalasia and reflux significantly decreased. Eighty-eight percent of patients felt their symptoms were greatly improved or resolved, and 90% felt their outcome was satisfying or better. Ninety-three percent felt they would undergo myotomy again, if necessary. Laparoscopic Heller myotomy can safely and durably relieve symptoms of dysphagia while also reducing symptoms of reflux. Length of stay is short and patient satisfaction is very high with extended follow-up. Laparoscopic Heller myotomy is strongly encouraged for patients with symptomatic achalasia and is efficacious even after failures of dilation and/or Botox therapy.

Trunk rotational strength asymmetry in adolescents with idiopathic scoliosis: an observational study

PubMed Central

McIntire, Kevin L; Asher, Marc A; Burton, Douglas C; Liu, Wen

2007-01-01

Background Recent reports have suggested a rotational strength weakness in rotations to the concave side in patients with idiopathic scoliosis. There have been no studies presenting normative values of female adolescent trunk rotational strength to which a comparison of female adolescents with idiopathic scoliosis could be made. The purpose of this study was to determine trunk rotational strength asymmetry in a group of female adolescents with AIS and a comparison group of healthy female adolescents without scoliosis. Methods Twenty-six healthy adolescent females served as the healthy group (HG) (average age 14 years) and fourteen otherwise healthy adolescent females with idiopathic scoliosis served as the idiopathic scoliosis group (ISG) (average age 13.5 years, average Cobb 28°). Participant's isometric trunk rotational strength was measured in five randomly ordered trunk positions: neutral, 18° and 36° of right and left pre-rotation. Rotational strength asymmetry was compared within each group and between the two groups using several different measures. Results The HG showed strength asymmetry in the 36° pre-rotated trunk positions when rotating towards the midline (p < 0.05). The ISG showed strength asymmetry when rotating towards the concavity of their primary curve from the neutral position (p < 0.05) and when rotating towards the concavity from the 18° (p < 0.05) and 36° (p < 0.05) concave pre-rotated positions. The ISG is significantly weaker than the HG when rotating away from the midline toward the concave (ISG)-left (HG) side from the concave/left pre-rotated 18° (p < 0.05) and 36° (p < 0.05) positions. Conclusion The AIS females were found to be significantly weaker when contracting toward their main curve concavity in the neutral and concave pre-rotated positions compared to contractions toward the convexity. These weaknesses were also demonstrated when compared to the group of healthy female adolescent controls. Possible mechanisms for the

Long-Term Outcomes in Idiopathic Membranous Nephropathy Using a Restrictive Treatment Strategy

PubMed Central

van Dijk, Peter R.; Hofstra, Julia M.; Wetzels, Jack F.M.

2014-01-01

Recently published Kidney Disease Improving Global Outcomes (KDIGO) guidelines recommend limiting the use of immunosuppressive drugs in idiopathic membranous nephropathy to patients at the highest risk of kidney failure. However, recommendations are based on natural history rather than direct assessment of a restrictive treatment strategy. Here, we describe the long-term outcomes of treating a large cohort of patients with idiopathic membranous nephropathy according to a restrictive treatment policy. We analyzed data for 254 patients who visited our outpatient clinic between 1995 and 2009. All patients were treated with angiotensin-converting enzyme inhibitors or angiotensin-receptor blockers. Immunosuppressive therapy was recommended in cases of deteriorating renal function or untreatable nephrotic syndrome. Primary outcomes for the present study were renal replacement therapy and death. Secondary outcomes included adverse events during follow-up and remission of proteinuria. In total, 124 patients (49%) received immunosuppressive therapy, which predominantly consisted of cyclophosphamide combined with steroids. Ten-year cumulative incidence rates were 3% for renal replacement therapy and 10% for death. Partial remission rates were 39%, 70%, and 83% after 1, 3, and 5 years, respectively; complete remission rates were 5%, 24%, and 38% at 1, 3, and 5 years, respectively. A serious adverse event occurred in 23% of all patients. The most notable complications were infections (17%), leukopenia (18%), cardiovascular events (13%), and malignancies (8%). In conclusion, the use of a restrictive treatment strategy in this cohort of patients with idiopathic membranous nephropathy yielded favorable outcomes while limiting the number of patients exposed to toxic drugs. These results support current KDIGO guidelines. PMID:24029426

[Idiopathic Nephrotic Syndrome: recommendations of the Nephrology Branch of the Chilean Society of Pediatrics. Part two].

PubMed

Hevia, Pilar; Nazal, Vilma; Rosati, María Pía; Quiroz, Lily; Alarcón, Claudia; Márquez, Sonia; Cuevas, Karen

2015-01-01

Idiopathic nephrotic syndrome is the most common glomerular disease in childhood, affecting 1 to 3 per 100,000 children under the age of 16. It most commonly occurs in ages between 2 and 10. Its cause is unknown, and its histology corresponds to minimal change disease in 90% of cases, or focal segmental glomerulosclerosis. Steroid-resistant nephrotic syndrome represents 10-20% of idiopathic nephrotic syndrome in pediatrics. It has a poor prognosis, and its management is a significant therapeutic challenge. Half of patients evolve to end-stage renal disease within 5 years, and are additionally exposed to complications secondary to persistent NS and to the adverse effects of immunosuppressive therapy. The primary goal of treatment is to achieve complete remission, but even a partial remission is associated with a better renal survival than the lack of response. This paper is the result of the collaborative effort of the Nephrology Branch of the Chilean Society of Pediatrics with aims at helping pediatricians and pediatric nephrologists to treat pediatric idiopathic nephrotic syndrome. In this second part, handling of steroid-resistant nephrotic syndrome as well as nonspecific therapies are discussed. Copyright © 2015 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

Randomized controlled trial of foot reflexology for patients with symptomatic idiopathic detrusor overactivity.

PubMed

Mak, Ho-Leung Jimmy; Cheon, Willy Cecilia; Wong, To; Liu, Yu Sun John; Tong, Wai Mei Anny

2007-06-01

The aim of this study was to examine whether foot reflexology has beneficial effects on patients with idiopathic detrusor overactivity. One hundred and nine women with symptomatic idiopathic detrusor overactivity were randomized into either foot reflexology treatment group or nonspecific foot massage control group. The primary outcome measure was the change in the diurnal micturition frequency. There was significant change in the number of daytime frequency in the reflexology group when compared with the massage group (-1.90 vs -0.55, p = 0.029). There was also a decrease in the 24-h micturition frequency in both groups, but the change was not statistically significant (-2.80 vs -1.04 p = 0.055). In the reflexology group, more patients believed to have received "true" reflexology (88.9 vs 67.4%, p = 0.012). This reflects the difficulty of blinding in trials of reflexology. Larger scale studies with a better-designed control group and an improved blinding are required to examine if reflexology is effective in improving patients' overall outcome.

[Vitrectomy for idiopathic and secondary preretinal macular membrane].

PubMed

Oficjalska-Młyńczak, Jolanta; Jamrozy-Witkowska, Agnieszka

2004-01-01

To evaluate the results of pars plana vitrectomy and membrane stripping for idiopathic and secondary preretinal macular membrane (PMM). Twenty one consecutive subjects (21 eyes) ranging in age from 40 to 78 (mean 66.9) with PMM underwent vitrectomy and membrane peeling. 17 cases had membranes that were considered idiopathic, and 4 cases were associated with other disorders: 3 occurred after successful retinal reattachment surgery, 1--after laserotherapy in the course of diabetic retinopathy. Visual acuity (VA), Amsler grid, and postoperative complications were assessed. The follow-up was 1 to 22 months, mean 5.7. Visual acuity improved postoperatively in 15 eyes (71.4%), at least two lines on the Snellen chart in 8 eyes (38.1%), entirely in patients with idiopathic PMM. It remained unchanged in 3 eyes (14.3%) and deteriorated in 3 eyes (14.3%). Eyes with transparent membrane showed greater visual improvement than opaque ones. The preoperative Amsler test was positive in 15 patients (71.4%), postoperatively--in 4 cases (19%). 2 idiopathic cases with VA of 0.7 showed postoperatively VA of 1.0. Complications included retinal detachment in 2 eyes (1 in idiopathic and 1 in secondary PMM), and development of nuclear sclerotic cataract in 2 eyes. At 6 months of follow-up, a residual membrane formation in 1 cases appeared. Macular pseudohole was observed in 1 eye with no impact on visual results. 1. Vitrectomy with membrane peeling for preretinal macular membrane provides improvement in visual acuity and reduces metamorphopsia 2. Thin, cellophane-like appearance of the membrane gives a better prognosis of visual function improvement.

Idiopathic inflammatory myopathies overlapping with systemic diseases

PubMed Central

Lepreux, Sébastien; Hainfellner, Johannes A.; Vital, Anne

2018-01-01

A muscle biopsy is currently requested to assess the diagnosis of an idiopathic inflammatory myopathy overlapping with a systemic disease. During the past few years, the classification of inflammatory myopathy subtypes has been revisited progressively on the basis of correlations between clinical phenotypes, autoantibodies and histological data. Several syndromic entities are now more clearly defined, and the aim of the present review is to clarify the contribution of muscle biopsy in a setting of idiopathic inflammatory myopathies overlapping with systemic diseases. PMID:29154752

Placental villous hypermaturation is associated with idiopathic preterm birth

PubMed Central

Morgan, Terry K.; Tolosa, Jorge E.; Mele, Lisa; Wapner, Ronald J.; Spong, Catherine Y.; Sorokin, Yoram; Dudley, Donald J.; Peaceman, Alan M.; Mercer, Brian M.; Thorp, John M.; O’Sullivan, Mary Jo; Ramin, Susan M.; Rouse, Dwight J.; Sibai, Baha

2014-01-01

Objective Pregnancy complications such as intra-amniotic infection, preeclampsia, and fetal intrauterine growth restriction (IUGR) account for most cases of preterm birth (PTB), but many spontaneous PTB cases do not have a clear etiology. We hypothesize that placental insufficiency may be a potential cause of idiopathic PTB. Methods Secondary analysis of 82 placental samples from women with PTB obtained from a multicenter trial of repeat versus single antenatal corticosteroids. Samples were centrally reviewed by a single placental pathologist masked to clinical outcomes. The histopathologic criterion for infection was the presence of acute chorioamnionitis defined as neutrophils marginating into the chorionic plate. Placental villous hypermaturation (PVH) was defined as a predominance of terminal villi (similar to term placenta) with extensive syncytial knotting. Idiopathic PTB comprised a group without another known etiology such as preeclampsia, IUGR or infection. Results Acute chorioamnionitis was observed in 33/82 (40%) cases. Other known causes of PTB were reported in 18/82 (22%). The remaining 31/82 (38%) were idiopathic. The frequency of PVH in idiopathic PTB (26/31=84%) was similar to cases with IUGR or preeclampsia (16/ 18=89%), but significantly more common than PVH in the group with acute chorioamnionitis (10/33=30%) (p<0.001). Conclusions PVH, which is a histologic marker of relative placental insufficiency, is a common finding in idiopathic PTB. PMID:23130816

Allelic variation of the FRMD7 gene in congenital idiopathic nystagmus.

PubMed

Self, James E; Shawkat, Fatima; Malpas, Crispin T; Thomas, N Simon; Harris, Christopher M; Hodgkins, Peter R; Chen, Xiaoli; Trump, Dorothy; Lotery, Andrew J

2007-09-01

To perform a genotype-phenotype correlation study in an X-linked congenital idiopathic nystagmus pedigree (pedigree 1) and to assess the allelic variance of the FRMD7 gene in congenital idiopathic nystagmus. Subjects from pedigree 1 underwent detailed clinical examination including nystagmology. Screening of FRMD7 was undertaken in pedigree 1 and in 37 other congenital idiopathic nystagmus probands and controls. Direct sequencing confirmed sequence changes. X-inactivation studies were performed in pedigree 1. The nystagmus phenotype was extremely variable in pedigree 1. We identified 2 FRMD7 mutations. However, 80% of X-linked families and 96% of simplex cases showed no mutations. X-inactivation studies demonstrated no clear causal link between skewing and variable penetrance. We confirm profound phenotypic variation in X-linked congenital idiopathic nystagmus pedigrees. We demonstrate that other congenital nystagmus genes exist besides FRMD7. We show that the role of X inactivation in variable penetrance is unclear in congenital idiopathic nystagmus. Clinical Relevance We demonstrate that phenotypic variation of nystagmus occurs in families with FRMD7 mutations. While FRMD7 mutations may be found in some cases of X-linked congenital idiopathic nystagmus, the diagnostic yield is low. X-inactivation assays are unhelpful as a test for carrier status for this disease.

Thoracoscopic versus laparoscopic modified Heller Myotomy for achalasia: efficacy and safety in 87 patients.

PubMed

Stewart, K C; Finley, R J; Clifton, J C; Graham, A J; Storseth, C; Inculet, R

1999-08-01

The ideal treatment for achalasia permanently eliminates the dysfunctional lower esophageal sphincter, relieving dysphagia and regurgitation; prevents gastroesophageal reflux; and has an acceptable morbidity rate. Controversy exists concerning whether the thoracoscopic Heller Myotomy (THM) or laparoscopic Heller myotomy (LHM) technique is the best approach to a modified Heller myotomy for achalasia. We performed a retrospective comparison of the patient characteristics, operative results, postoperative symptoms, and the learning curves for the procedures of 24 patients undergoing THM and 63 patients undergoing LHM between 1991 and 1998. Preoperative patient variables in each group revealed similar distributions for age, gender, and prevalence of previous pneumatic dilation. Mean operating room (OR) times were 4.3 hours (range 2.9 to 5.6 hours) for THM and 3.0 hours (range 1.5 to 6.5 hours) for LHM (p = 0.01). Three esophageal perforations occurred in the THM group and two in the LHM group. Conversion to an open procedure took place in five THM operations (21%) and one LHM operation (2%) (p = 0.005). There were no postoperative esophageal leaks. Mean postoperative length of stay (LOS) for THM was 6.1 days (range 1 to 17 days) and for LHM was 4.0 days (range 1 to 12 days) (p = 0.03). Learning-curve analysis of the first 24 LHM patients compared with the most recent 24 revealed greater OR time in the first 24 mean 3.6 hours, (range 2.0 to 6.5 hours) versus mean 2.3 hours, (range 1.5 to 3.7 hours; p = 0.01), and greater LOS mean 5.5 days, (range 3 to 12 days) versus mean 3.1 days, (range 1 to 8 days; p < 0.01). One esophageal perforation occurred in each subgroup. A similar analysis in the first 12 THM patients compared with the most recent 12 revealed no significant improvement in OR times or LOS. Three esophageal perforations occurred in the latter subgroup only. All patients had preoperative daily dysphagia to solids. Followup data for LHM (n = 49) (median 17 months

Study on Treatment with Respect to Idiopathic Scoliosis

NASA Astrophysics Data System (ADS)

Takeuchi, Kenzen; Azegami, Hideyuki; Murachi, Shunji; Kitoh, Junzoh; Ishida, Yoshito; Kawakami, Noriaki; Makino, Mitsunori

A hypothesis that the thoracic idiopathic scoliosis is buckling phenomenon of the fourth mode induced by the growth of thoracic vertebral bodies was presented in the previous work by the authors using numerical simulations with finite element model of the spine. If the hypothesis is acceptable, sensitivity function with respect to the critical growth of thoracic vertebrae on the maximization problem of buckling load with the fourth buckling mode gives us useful information to improve and develop treatments for the idiopathic scoliosis. The numerical results analyzed by the finite element method demonstrated that the sensitivity function is high at the articular capsules of the intervertebral joints, the intervertebral disks, the costotransverse joints and the constovertebral joints around the apex of the curvature in the case of the thoracic idiopathic scoliosis.

Adults with idiopathic scoliosis improve disability after motor and cognitive rehabilitation: results of a randomised controlled trial.

PubMed

Monticone, Marco; Ambrosini, Emilia; Cazzaniga, Daniele; Rocca, Barbara; Motta, Lorenzo; Cerri, Cesare; Brayda-Bruno, Marco; Lovi, Alessio

2016-10-01

To evaluate the effects of motor and cognitive rehabilitation on disability in adults with idiopathic scoliosis at lower risk of progression. 130 adults with idiopathic scoliosis (main curve <35°) were randomly assigned to a 20-week rehabilitation programme consisting of active self-correction, task-oriented exercises and cognitive-behavioural therapy (experimental group, 65 subjects, mean age of 51.6, females 48) or general physiotherapy consisting of active and passive mobilizations, stretching, and strengthening exercises of the spinal muscles (control group, 65 subjects, mean age of 51.7, females 46). Before, at the end, and 12 months after treatment, each participant completed the Oswestry disability index (ODI) (primary outcome), the Tampa scale for kinesiophobia, the pain catastrophizing scale, a pain numerical rating scale, and the Scoliosis Research Society-22 Patient Questionnaire. Radiological (Cobb angle) and clinical deformity (angle of trunk rotation) changes were also investigated. A linear mixed model for repeated measures was used for each outcome. Significant effects of time, group, and time by group interaction were found for all outcome measures (P < 0.001). After training, the primary outcome showed a clinically significant between-group change (12 % points), which was preserved at follow-up. At follow-up, the radiological deformities showed a significant, although not clinically meaningful, between-group difference of 4° in favour of the experimental group. The experimental programme was superior to general physiotherapy in reducing disability of adults with idiopathic scoliosis. Motor and cognitive rehabilitation also led to improvements in dysfunctional thoughts, pain, and quality of life. Changes were maintained for at least 1 year.

Idiopathic orbital inflammatory disease successfully treated with rituximab.

PubMed

Schafranski, Marcelo Derbli

2009-02-01

We report a case of a 66-year-old Caucasian female with a diagnosis of idiopathic orbital inflammatory disease (OID) refractory to azathioprine therapy. The coexistence of diabetes mellitus represented a relative contraindication to chronic prednisone use. After two infusions of rituximab, a chimeric anti-CD20+ antibody, ophthalmic signs and symptoms remarkably improved. To our knowledge, this is the first case of idiopathic OID successfully treated with rituximab.

Pathogenesis, Newly Recognized Etiologies, and Management of Idiopathic Anaphylaxis

PubMed Central

Kuhlen, James L.; Virkud, Yamini V.

2018-01-01

Idiopathic anaphylaxis (IA) is a life-threatening allergic disease and the most common diagnosis given to patients following an anaphylactic event. The inability of the healthcare provider and the patient to identify the trigger for anaphylaxis makes standard allergen avoidance measures ineffectual. IA is diagnosed after other causes of anaphylaxis have been excluded. Mast cell activation syndromes (MCAS), mastocytosis, IgE to galactose-alpha-1,3-galactose (α-gal), and certain medications have recently been recognized as causes of anaphylaxis that were previously labeled idiopathic. This review will describe the epidemiology and proposed theories of pathogenesis for IA, its diagnostic approach, its clinical management, and examine newly recognized disorders that were previously labeled as idiopathic anaphylaxis. PMID:25725228

Outcome of peroral endoscopic myotomy in achalasia cardia: Experience with a new triangular knife

PubMed Central

Nabi, Zaheer; Ramchandani, Mohan; Chavan, Radhika; Kalapala, Rakesh; Darisetty, Santosh; Reddy, D. Nageshwar

2018-01-01

Background and Aim: Peroral endoscopic myotomy (POEM) is a technically demanding procedure. Recently, a new triangular knife with integrated water jet function (TTJ) has been introduced. The present study was aimed to analyze and compare the operating time, efficacy, and adverse events (AEs) between the conventional (TT knife) and new knife (TTJ). Patients and Methods: All patients with achalasia cardia (AC) who underwent POEM between August 2015 and November 2016 were analyzed retrospectively. Operating time (OT), technical success, and AEs were assessed and compared between TT and TTJ knife. Results: A total of 193 patients with AC underwent POEM during the specified period. Both groups had equivalent number of different subtypes of AC (I, II, and III). There was no difference in technical success between the two groups (TT, 99% vs TT, 98.9%). OT was significantly less in the TTJ group as compared to TT group (53.8 ± 15.2 vs 66.26 ± 19.2; P = 0.0001). On subanalysis, OT taken for submucosal tunneling was significantly less with TTJ knife (34.6 ± 10.1 vs 45.83 ± 14.80), whereas OT was similar for myotomy and clipping in both the groups. Significantly fewer use of coagulation forceps and exchanges of accessories were required in TTJ knife group (2.92 ± 1.77 vs 10.5 ± 3.58; P = 0.0001). There were no major AEs. Minor AEs were noted in 21.5% and 31% of patients in TTJ and TT knife groups, respectively. Conclusion: New triangular knife reduces procedure time and technical difficulty with POEM. POEM is an efficacious treatment for achalasia and can be safely executed in an endoscopy unit. PMID:29451180

Intravenous immunoglobulin and idiopathic secondary recurrent miscarriage: a multicentered randomized placebo-controlled trial

PubMed Central

Stephenson, Mary D.; Kutteh, William H.; Purkiss, Susan; Librach, Cliff; Schultz, Patricia; Houlihan, Edwina; Liao, Chuanhong

2010-01-01

BACKGROUND Idiopathic secondary recurrent miscarriage may be associated with an abnormal maternal immune response to subsequent pregnancies. Intravenous immunoglobulin (IVIG) has been studied in randomized controlled trials (RCTs) with conflicting results. Therefore, a definitive trial was proposed. METHODS We conducted an investigator-initiated, multicentered, randomized, double-blinded, placebo-controlled trial comparing IVIG with saline in women with idiopathic secondary recurrent miscarriage, defined as a history of at least one prior ongoing pregnancy followed by three or more consecutive unexplained miscarriages. Subjects received either IVIG 500 mg/kg or the equivalent volume of normal saline. Preconception infusions were administered 14–21 days from the projected next menstrual period. With documentation of pregnancy, the subject received the same infusion every 4 weeks until 18–20 weeks of gestation. The primary outcome was an ongoing pregnancy of at least 20 weeks of gestation. RESULTS A total of 82 patients enrolled, of whom 47 had an index pregnancy. All ongoing pregnancies resulted in live births. Therefore, the live birth rates were 70% (16/23) in the IVIG group and 63% (15/24) in the control group (P = 0.760); odds ratio (OR) 1.37 [95% confidence interval (CI) 0.41–4.61]. Including only clinical pregnancies (embryo with cardiac activity at 6 weeks of gestation), the live birth rates were equivalent, 94% (16/17) and (15/16), respectively (P > 0.999); OR 1.07 (95% CI 0.06–18.62). Meta-analysis of randomized controlled trials (RCTs) evaluating IVIG for idiopathic secondary recurrent miscarriage revealed live birth rates of 70% (31/44) in the IVIG group and 62% (28/45) in the control group (P = 0.503); common OR 1.44 (95% CI 0.59–3.48). CONCLUSIONS This is the largest RCT to date in which IVIG was evaluated in women with idiopathic secondary recurrent miscarriage; no treatment benefit was found. The meta-analysis, which combined our study

Laparoscopic Heller Myotomy Provides Durable Relief From Achalasia and Salvages Failures After Botox or Dilation

PubMed Central

Rosemurgy, Alexander; Villadolid, Desiree; Thometz, Donald; Kalipersad, Candice; Rakita, Steven; Albrink, Michael; Johnson, Milton; Boyce, Worth

2005-01-01

Objective: To report outcome after laparoscopic Heller myotomy in a large number of patients. Summary Background Data: Laparoscopic Heller myotomy has been undertaken for over a decade, but most studies involve small numbers of patients with limited follow-up. Methods: Since 1992, 262 patients have undergone laparoscopic Heller myotomy and been prospectively followed. Concomitant fundoplication was undertaken for a patulous hiatus or large hiatal hernia or to buttress the repair of an esophagotomy until recently when it became routinely applied. With mean follow-up at 32months, symptoms were scored by patients on a Likert scale (frequency: 0 = Never to 10 = Every time I eat/always; severity: 0 = Not bothersome to 10 = Very bothersome). Results: Before myotomy, 79% received Botox or bag dilation: 52% had Botox, 59% underwent dilation, and 36% had both. Inadvertent esophagotomy occurred in 5%. Concomitant diverticulectomy was undertaken in 4%, and fundoplication was undertaken in 30%. Complications were infrequent. Median length of stay was 1 day. After myotomy, the frequency and severity of symptoms of achalasia and reflux significantly decreased. Eighty-eight percent of patients felt their symptoms were greatly improved or resolved, and 90% felt their outcome was satisfying or better. Ninety-three percent felt they would undergo myotomy again, if necessary. Conclusions: Laparoscopic Heller myotomy can safely and durably relieve symptoms of dysphagia while also reducing symptoms of reflux. Length of stay is short and patient satisfaction is very high with extended follow-up. Laparoscopic Heller myotomy is strongly encouraged for patients with symptomatic achalasia and is efficacious even after failures of dilation and/or Botox therapy. PMID:15849508

Extended Ponseti method for failed tenotomy in idiopathic clubfeet: a pilot study.

PubMed

Agarwal, Anil; Agrawal, Nargesh; Barik, Sitanshu; Gupta, Neeraj

2018-01-29

We evaluated the outcome of a new protocol of an extended Ponseti method in the management of idiopathic club foot with residual equinus following failed Achilles tenotomy. We also compared the failed with a successful tenotomy group to analyze the parameters for failure. The Ponseti technique-treated idiopathic club foot patients with failed percutaneous Achilles tenotomy (failure to achieve <15° dorsiflexion) were treated by continued stretching casts, with a weekly change for a further 3 weeks. Final dorsiflexion more than 15° if achieved with the above protocol was recorded as a success. Twenty-six (16%) patients with failed Achilles tenotomy and residual equinus out of a total of 161 patients with primary idiopathic club foot were tested with the protocol. Ten (38.5%) failed patients had bilateral foot involvement and 16 (61.5%) had unilateral foot involvement. A total of seven (26.9%) patients achieved the end point dorsiflexion of more than 15° in one further cast, 10 (38.5%) in two casts, and four (15.4%) in three casts, respectively. Overall success of the extended Ponseti protocol was achieved in 21/26 (80.8%) patients. The patient's age, precasting initial Pirani score, number of Ponseti casts, pretenotomy Pirani score, and pretenotomy ankle joint dorsiflexion were statistically different in the failed compared with the successful tenotomy group. The tested extended Ponseti protocol showed a success rate of 80.8% in salvaging failed tenotomy cases. The failed tenotomy group was relatively older at presentation, had high precasting and pretenotomy Pirani scores, received extra number of Ponseti casts, and less pretenotomy ankle joint dorsiflexion compared with successful feet.

MR venography in idiopathic intracranial hypertension: unappreciated and misunderstood

PubMed Central

Higgins, J; Gillard, J; Owler, B; Harkness, K; Pickard, J

2004-01-01

Background: Venous sinus disease must be excluded before diagnosing idiopathic intracranial hypertension but is found only rarely in typical cases. Magnetic resonance venography (MRV) is the technique of choice for investigating this, and provides images that are diagnostic and easy to interpret. However, recent work using more invasive techniques has documented pressure gradients and stenoses in the lateral venous sinuses in many cases of idiopathic intracranial hypertension. Objective: To examine the reason for this discrepancy and to establish whether there are characteristic appearances on MRV in idiopathic intracranial hypertension that are routinely overlooked in clinical practice. Methods: MRVs from 20 patients with idiopathic intracranial hypertension were reviewed, unblinded, by two neuroradiologists, and their appearances rated for focal narrowings and signal gaps. A control group of 40 asymptomatic volunteers, matched for age and sex with the patient group, was recruited prospectively for MRV, and their scans rated in the same way. Results: The lateral sinuses presented a range of appearances with quite different distributions in the two groups (p<0.001). Bilateral lateral sinus flow gaps were seen in 13 of 20 patients with idiopathic intracranial hypertension and in none of 40 controls. Conclusions: A historical failure to use normal healthy controls to establish the boundaries between imaging artefact, normal anatomical variant, and disease means that the pathological significance of the different appearances of the lateral sinuses on MRV has not so far been appreciated. PMID:15026510

Routine magnetic resonance imaging for idiopathic olfactory loss: a modeling-based economic evaluation.

PubMed

Rudmik, Luke; Smith, Kristine A; Soler, Zachary M; Schlosser, Rodney J; Smith, Timothy L

2014-10-01

Idiopathic olfactory loss is a common clinical scenario encountered by otolaryngologists. While trying to allocate limited health care resources appropriately, the decision to obtain a magnetic resonance imaging (MRI) scan to investigate for a rare intracranial abnormality can be difficult. To evaluate the cost-effectiveness of ordering routine MRI in patients with idiopathic olfactory loss. We performed a modeling-based economic evaluation with a time horizon of less than 1 year. Patients included in the analysis had idiopathic olfactory loss defined by no preceding viral illness or head trauma and negative findings of a physical examination and nasal endoscopy. Routine MRI vs no-imaging strategies. We developed a decision tree economic model from the societal perspective. Effectiveness, probability, and cost data were obtained from the published literature. Litigation rates and costs related to a missed diagnosis were obtained from the Physicians Insurers Association of America. A univariate threshold analysis and multivariate probabilistic sensitivity analysis were performed to quantify the degree of certainty in the economic conclusion of the reference case. The comparative groups included those who underwent routine MRI of the brain with contrast alone and those who underwent no brain imaging. The primary outcome was the cost per correct diagnosis of idiopathic olfactory loss. The mean (SD) cost for the MRI strategy totaled $2400.00 ($1717.54) and was effective 100% of the time, whereas the mean (SD) cost for the no-imaging strategy totaled $86.61 ($107.40) and was effective 98% of the time. The incremental cost-effectiveness ratio for the MRI strategy compared with the no-imaging strategy was $115 669.50, which is higher than most acceptable willingness-to-pay thresholds. The threshold analysis demonstrated that when the probability of having a treatable intracranial disease process reached 7.9%, the incremental cost-effectiveness ratio for MRI vs no

Idiopathic scrotal calcinosis.

PubMed

Celik, Orcun; Ipekci, Tumay; Kazimoglu, Hatem

2013-12-01

Idiopathic scrotal calcinosis is a rare scrotal benign disease. Its distinct features are painless, non-pruritic, semi-soft palpable calcific transdermal nodules. We report a 42-year-old-man with asymptomatic multiple calcified scrotal skin nodules for 10 years. Under spinal anesthesia, the affected scrotal skin was excised and the nodules removed. We aim to explain the etiology, pathophysiology, diagnosis, and treatment modalities of this rare disease.

A procedure to detect abnormal sensorimotor control in adolescents with idiopathic scoliosis.

PubMed

Pialasse, Jean-Philippe; Mercier, Pierre; Descarreaux, Martin; Simoneau, Martin

2017-09-01

This work identifies, among adolescents with idiopathic scoliosis, those demonstrating impaired sensorimotor control through a classification procedure comparing the amplitude of their vestibular-evoked postural responses. The sensorimotor control of healthy adolescents (n=17) and adolescents with idiopathic scoliosis (n=52) with either mild (Cobb angle≥15° and ≤30°) or severe (Cobb angle >30°) spine deformation was assessed through galvanic vestibular stimulation. A classification procedure sorted out adolescents with idiopathic scoliosis whether the amplitude of their vestibular-evoked postural response was dissimilar or similar to controls. Compared to controls, galvanic vestibular stimulation evoked larger postural response in adolescents with idiopathic scoliosis. Nonetheless, the classification procedure revealed that only 42.5% of all patients showed impaired sensorimotor control. Consequently, identifying patients with sensorimotor control impairment would allow to apply personalized treatments, help clinicians to establish prognosis and hopefully improve the condition of patients with adolescent idiopathic scoliosis. Copyright © 2017 Elsevier B.V. All rights reserved.

Idiopathic hemifacial spasm responsive to zonisamide: a case report.

PubMed

Siniscalchi, Antonio; Gallelli, Luca; Palleria, Caterina; De Sarro, Giovambattista

2009-01-01

We describe a patient with idiopathic hemifacial spasm (HFS) that was responsive to zonisamide treatment. A 65-year-old woman presented with a 4-year history of left-sided HFS developing gradually, starting from the upper facial muscles. After several analyses, the diagnosis of idiopathic HFS was made, and the clonazepam treatment (0.5 mg every 8 hours) was started, without a complete remission of symptoms. Therefore, zonisamide (150 mg twice a day for a 6-week period) was added, with a complete resolution. The rechallenge with zonisamide after its dechallenge confirmed its effectiveness. During follow-up, the patient remained symptom-free, with no adverse drug reactions. We suggest that zonisamide could represent a useful therapeutic option in the treatment of idiopathic HFS.

Hypertrophy of the muscularis propria of the lower esophageal sphincter and the body of the esophagus in patients with primary motility disorders of the esophagus.

PubMed

Mittal, Ravinder K; Kassab, Ghassan; Puckett, James L; Liu, Jianmin

2003-08-01

Patients with diffuse esophageal spasm (DES) and nutcracker esophagus/high amplitude esophageal contraction (HAEC) have a thicker esophageal muscularis propria than do healthy subjects. The goals of this study were to determine the esophageal muscle cross-sectional area (MCSA), a measure of muscle mass, in patients with achalasia of the esophagus; and to compare it with that in patients with DES, patients with HAEC, and normal subjects. Using a high-frequency ultrasound probe catheter, concurrent manometry and ultrasound images of the esophagus were recorded in four subject groups: normal volunteers, patients with HAEC, patients with DES, and patients with achalasia of the esophagus. Recordings were obtained from the lower esophageal sphincter (LES) and multiple sites in the esophagus 2, 4, 6, 8, and 10 cm above the LES. The LES and esophageal muscle thickness as well as esophageal MCSA were greater in all three patient groups than in the normal subject group. Muscle thickness and MCSA were observed to be greatest in patients with achalasia, which were greater than in patients with DES, which were greater than in those with HAEC, which in turn were greater than in normal subjects. We propose that an increase in the MCSA is an important feature of patients with primary motility disorders of the esophagus. The degree of increase in muscle mass may be an important determinant of the type and the severity of esophageal motor dysfunction.

Nephrogenic diabetes insipidus with idiopathic Fanconi's syndrome in a child who presented as vitamin D resistant rickets.

PubMed

Patra, Soumya; Nadri, Gulnaz; Chowdhary, Harish; Pemde, Harish K; Singh, Varinder; Chandra, Jagdish

2011-10-01

Fanconi's syndrome is a complex of multiple tubular dysfunctions of proximal tubular cells occurring alone or in association with a variety of inherited (primary) or acquired (secondary) disorders. It is characterized by aminoaciduria, normoglycaemic glycosuria, tubular proteinuria without hematuria, metabolic acidosis without anion gap and excessive urinary excretion of phosphorous, calcium, uric acid, bicarbonate, sodium, potassium, and magnesium. Whereas diabetes insipidus is a disease of collecting tubules and child mainly presents with dehydration and hypernatremia. Though all the cases published till date were secondary to drugs, myeloma, hematological disorders, etc., we are reporting the first case of idiopathic Fanconi's syndrome along with nephrogenic diabetes insipidus in a child who presented to us as resistant rickets. Medline search did not reveal any case of nephrogenic diabetes insipidus associated with idiopathic Fanconi syndrome. We hypothesized that the NDI may be due to of severe hypokalemia induced tubular dysfunction.

Mucosal perforation during laparoscopic surgery for achalasia: impact of preoperative pneumatic balloon dilation.

PubMed

Souma, Yoshihito; Nakajima, Kiyokazu; Taniguchi, Eiji; Takahashi, Tsuyoshi; Kurokawa, Yukinori; Yamasaki, Makoto; Miyazaki, Yasuhiro; Makino, Tomoki; Hamada, Tetsuhiro; Yasuda, Jun; Yumiba, Takeyoshi; Ohashi, Shuichi; Takiguchi, Shuji; Mori, Masaki; Doki, Yuichiro

2017-03-01

Controversy remains whether preoperative pneumatic balloon dilation (PBD) influences the surgical outcome of laparoscopic esophagocardiomyotomy in patients with esophageal achalasia. The aim of this study was to evaluate whether preoperative PBD represents a risk factor for surgical complications and affects the symptomatic and/or functional outcomes of laparoscopic Heller myotomy with Dor fundoplication (LHD). A retrospective chart review was conducted on a prospectively compiled surgical database of 103 consecutive patients with esophageal achalasia who underwent LHD from November 1994 to September 2014. The following data were compared between the patients with preoperative PBD (PBD group; n = 26) and without PBD (non-PBD group; n = 77): (1) patients' demographics: age, gender, body mass index, duration of symptoms, maximum transverse diameter of esophagus; (2) operative findings: operating time, blood loss, intraoperative complications; (3) postoperative course: complications, clinical symptoms, postoperative treatment; and (4) esophageal functional tests: preoperative and postoperative manometric data and postoperative profile of 24-h esophageal pH monitoring. (1) No significant differences were observed in the patients' demographics. (2) Operative findings were similar between the two groups; however, the incidence of mucosal perforation was significantly higher in the PBD group (n = 8; 30.7 %) compared to the non-PBD group (n = 6; 7.7 %) (p = 0.005). (3) Postoperative complications were not encountered in either group. The differences were not significant for postoperative clinical symptoms, the incidence of gastroesophageal reflux disease, or necessity of postoperative treatments. (4) Lower esophageal sphincter pressure was effectively reduced in both groups, and no differences were observed in manometric data or 24-h pH monitoring profiles between the two groups. Multivariate logistic regression analysis showed that preoperative PBD and the

Laparoscopic Heller myotomy as the gold standard for treatment of achalasia.

PubMed

Nau, Peter; Rattner, David

2014-12-01

The recent introductions of novel methods for the treatment of achalasia as well as ongoing controversies about the merits of surgical and endoscopic treatment options have created controversy in identifying the optimal treatment for this condition. This lack of clarity prompted this review of 206 consecutive patients treated with a laparoscopic Heller (LH) myotomy over a 16-year period. A retrospective review of a prospectively collected database was performed of 206 consecutive LH performed by a single surgeon. In this cohort, 58 % of patients had undergone a prior therapeutic intervention. Over 90 % of patients had relief of dysphagia post-operatively. There was one intraoperative esophageal perforation. There were no mortalities. Only 4/206 patients sustained complications that required either post-op therapeutic intervention or delayed hospital discharge. This paper outlines an operative technique that has yielded outstanding results and may be used as a benchmark against which other therapies can be judged.

Idiopathic central diabetes Insipidus.

PubMed

Grace, Mary; Balachandran, Venu; Menon, Sooraj

2011-10-01

Idiopathic central diabetes insipidus (CDI) is a rare disorder characterized clinically by polyuria and polydipsia, and an abnormal urinary concentration without any identified etiology. We report a case of central diabetes insipidus in a 60-year-old lady in the absence of secondary causes like trauma, infection, and infiltrative disorders of brain.

Use of isosorbide dinitrate for the symptomatic treatment of patients with Chagas' disease achalasia. A double-blind, crossover trial.

PubMed

Ferreira-Filho, L P; Patto, R J; Troncon, L E; Oliveira, R B

1991-01-01

1. A randomized, double-blind, placebo-controlled trial was carried out to determine the efficacy of isosorbide dinitrate (ISD) on dysphagia in patients with Chagasic achalasia. 2. Twenty-three patients with Chagas' disease and dysphagia entered the study and 20 (87%) completed the two 7-day treatment periods. Subjects were given either 5 mg ISD (12 patients) or placebo (11 patients) by the sublingual route for the first 7 days. On the 8th day, patients crossed over and began another 7-day period during which they received the opposite, identical-appearing tablets. 3. Scores attributed by uninformed investigators for the frequency and severity of dysphagia were significantly lower (P less than 0.05) following ISD treatment than after the placebo period or for the pretreatment condition. A significantly higher degree of improvement of dysphagia was experienced by the patients during ISD treatment than during the placebo period. Fourteen patients experienced meal-related headaches during ISD, but not placebo treatment. The extent of improvement in general well-being due to ISD was the same when the drug was given in the first or second test period. 4. Our results indicate that ISD, 5 mg by the sublingual route, is effective in alleviating dysphagia in patients with Chagasic achalasia but its usefulness is limited by the high rate of headache as a side effect.

Idiopathic thoracic transdural intravertebral spinal cord herniation

PubMed Central

Turel, Mazda K; Wewel, Joshua T; Kerolus, Mena G; O'Toole, John E

2017-01-01

Idiopathic spinal cord herniation is a rare and often missed cause of thoracic myelopathy. The clinical presentation and radiological appearance is inconsistent and commonly confused with a dorsal arachnoid cyst and often is a misdiagnosed entity. While ventral spinal cord herniation through a dural defect has been previously described, intravertebral herniation is a distinct entity and extremely rare. We present the case of a 70-year old man with idiopathic thoracic transdural intravertebral spinal cord herniation and discuss the clinico-radiological presentation, pathophysiology and operative management along with a review the literature of this unusual entity. PMID:29021685

Multiple idiopathic external apical root resorption: report of four cases.

PubMed

Cholia, S S; Wilson, P H R; Makdissi, J

2005-07-01

Multiple idiopathic external root resorption is an unusual condition that may present in a cervical or an apical form. In this article, we review the published literature relating to multiple idiopathic external apical root resorption and present four clinical cases. We consider the aetiology of this condition and discuss the various treatment options.

Etiological Theories of Adolescent Idiopathic Scoliosis: Past and Present

PubMed Central

Fadzan, Maja; Bettany-Saltikov, Josette

2017-01-01

Adolescent idiopathic scoliosis is one of the most common spinal deformities, yet its cause is unknown. Various theories look to biomechanical, neuromuscular, genetic, and environmental origins, yet our understanding of scoliosis etiology is still limited. Determining the cause of a disease is crucial to developing the most effective treatment. Associations made with scoliosis do not necessarily point to causality, and it is difficult to determine whether said associations are primary (playing a role in development) or secondary (develop as a result of scoliosis). Scoliosis is a complex condition with highly variable expression, even among family members, and likely has many causes. These causes could be similar among homogenous groups of AIS patients, or they could be individual. Here, we review the most prevalent theories of scoliosis etiology and recent trends in research. PMID:29399224

Are bilateral idiopathic clubfeet more severe than unilateral feet? A severity and treatment analysis.

PubMed

Agarwal, Anil; Agrawal, Nargesh; Barik, Sitanshu; Gupta, Neeraj

2018-01-01

Evidences suggest that different subgroups of idiopathic clubfoot exist with differences in severity and treatment outcomes. This study compares the severity and treatment outcomes of unilateral and bilateral clubfoot. We retrospectively studied 161 patients (bilateral 66, unilateral 95) with primary idiopathic clubfeet to evaluate the differences in severity and treatment. The parameters analyzed were precasting Pirani score, number of casts required, pretenotomy Pirani score, pretenotomy dorsiflexion, rate of tenotomy, and post-tenotomy dorsiflexion achieved. A Pirani score of at least 5 was classified as very severe and 4.5 or less was classified as less severe. There were 49=(74.24%) male and 17 (25.75%) female patients in the bilateral group and 76 (80%) male and 19 (20%) female patients in the unilateral group. Out of 95 unilateral patients, 34 were left sided (35.8%). Comparing severity, the mean precasting Pirani score in bilateral patients (5.4 ± 0.6) was statistically more than the unilateral patients (4.9 ± 0.7). The number of casts required was significantly more in bilateral feet compared to unilateral (bilateral 5.3 ± 1.7, unilateral 4.7 ± 1.7; p < 0.011). Achilles tenotomy was required in all feet. Post Ponseti treatment, the foot deformity correction achieved (pretenotomy Pirani score, pretenotomy, and post-tenotomy dorsiflexion) was statistically similar in both unilateral and bilateral feet. Idiopathic bilateral clubfoot was more severe than unilateral foot at initial presentation and required more number of corrective casts. Post Ponseti treatment, the deformity correction in bilateral foot was similar to unilateral foot.

Comparison of endogenous and radiolabeled bile acid excretion in patients with idiopathic chronic diarrhea

DOE Office of Scientific and Technical Information (OSTI.GOV)

Schiller, L.R.; Bilhartz, L.E.; Santa Ana, C.A.

Fecal recovery of radioactivity after ingestion of a bolus of radiolabeled bile acid is abnormally high in most patients with idiopathic chronic diarrhea. To evaluate the significance of this malabsorption, concurrent fecal excretion of both exogenous radiolabeled bile acid and endogenous (unlabeled) bile acid were measured in patients with idiopathic chronic diarrhea. Subjects received a 2.5-microCi oral dose of taurocholic acid labeled with 14C in the 24th position of the steroid moiety. Endogenous bile acid excretion was measured by a hydroxysteroid dehydrogenase assay on a concurrent 72-h stool collection. Both radiolabeled and endogenous bile acid excretion were abnormally high inmore » most patients with chronic diarrhea compared with normal subjects, even when equivoluminous diarrhea was induced in normal subjects by ingestion of osmotically active solutions. The correlation between radiolabeled and endogenous bile acid excretion was good. However, neither radiolabeled nor endogenous bile acid excretion was as abnormal as is typically seen in patients with ileal resection, and none of these diarrhea patients responded to treatment with cholestyramine with stool weights less than 200 g. These results suggest (a) that this radiolabeled bile acid excretion test accurately reflects excess endogenous bile acid excretion; (b) that excess endogenous bile acid excretion is not caused by diarrhea per se; (c) that spontaneously occurring idiopathic chronic diarrhea is often associated with increased endogenous bile acid excretion; and (d) that bile acid malabsorption is not likely to be the primary cause of diarrhea in most of these patients.« less

Orthodontic treatment in patient with idiopathic root resorption: a case report.

PubMed

Rey, Diego; Smit, Rosana Martínez; Gamboa, Liliana

2015-01-01

Multiple idiopathic external root resorption is a rare pathological condition usually detected as an incidental radiographic finding. External root resorption of permanent teeth is a multifactorial process related to several local and systemic factors. If an etiological factor cannot be identified for root resorption, the term "idiopathic" is applied. This report presents a case of multiple idiopathic apical root resorption. The condition was found in a young female patient seeking orthodontic treatment due to malocclusion. This kind of resorption starts apically and progresses coronally, causing a gradual shortening and rounding of the remaining root. Patients with this condition are not the ideal candidates for orthodontic treatment; however, the aim of this report is to describe an unusual case of idiopathic root resorption involving the entire dentition, and to present the orthodontic treatment of this patient. It describes the progress and completion of orthodontic therapy with satisfactory end results.

Disease of the year: juvenile idiopathic arthritis--differential diagnosis.

PubMed

Hu-Torres, Sandra; Foster, C Stephen

2014-02-01

The purpose of this review is to comprehensively explain the differential diagnosis of juvenile idiopathic arthritis-associated uveitis. Web-based literature review. Main diagnostic decisions are made through a thorough anterior segment exam and a comprehensive exploration of past medical and family history. High clinical suspicion of other uveitic entities occurring in children is necessary and must be excluded by the practitioner before immediate diagnosis of juvenile idiopathic arthritis is made.

[Coincidence of juvenile idiopathic arthritis and multiple sclerosis: case report].

PubMed

Puszczewicz, Mariusz J; Tuchocka-Piotrowska, Aleksandra; Majewski, Dominik; Kołczewska, Aleksandra

2006-01-01

Juvenile idiopathic arthritis is a systemic pathology of connective tissue characterized by a chronic inflammatory process with an autoimmune background whereas multiple sclerosis is a demyelination disease with an important role of immune disorders in its pathogenesis. The etiology in both cases remains unknown. The coincidence of juvenile idiopathic arthritis and multiple sclerosis was described a just a few patients. We now report on a 31-year-old woman with juvenile idiopathic arthritis and multiple sclerosis. In the present case, the main problem was to find the right proper medication for a very, aggressive course of multiple sclerosis and for arthritis. Treatment with interferon-beta and methylprednisolone led to remission with just minor side-effects.

Evidence for human leukocyte antigen-related susceptibility in idiopathic childhood ischemic stroke.

PubMed

Zou, Li-Ping; Guo, Yu-Hong; Fang, Fang; Jin, Hong; Wu, Hu-Sheng; Mix, Eilhard

2002-01-01

Stroke in children is a relatively uncommon condition and frequently associated with other diseases like cardiopathies, sickle cell disease and chronic smoking. In contrast to stroke in adults, it is rarely caused by atherosclerosis, hypertension or diabetes mellitus. Childhood stroke of unknown causes is called idiopathic stroke. The etiology of idiopathic stroke is unknown. However, several so-called idiopathic diseases develop on the basis of a genetic predisposition. As an approach to investigate this possibility in idiopathic childhood ischemic stroke, we studied the relationship between clinical and immunogenetic features in this disease. We demonstrate that the gene frequencies and relative risk of HLA-B51 were markedly increased in our patients compared with controls (p < 0.001). Thirteen of seventeen HLA-B51-positive patients had had a preceding respiratory infection, which was a higher proportion than in the control group (p < 0.05). In the patient group, the alleles HLA-DRB1*0802, -DRAI*0401 and -DQBI*0402 were also significantly increased, defining the haplotype DRB1*0802-DRA1*0401-DQB1*0402 as a high-risk haplotype for idiopathic childhood ischemic stroke. Transient viral or bacterial infections, which involve vasculitis and vascular occlusion in the brain, can trigger idiopathic childhood ischemic stroke on the basis of an genetic predisposition. Copyright 2002 S. Karger AG, Basel

Methotrexate-induced nausea in the treatment of juvenile idiopathic arthritis.

PubMed

Falvey, Sonja; Shipman, Lauren; Ilowite, Norman; Beukelman, Timothy

2017-06-19

Methotrexate is the most commonly used disease modifying antirheumatic drug in the treatment of juvenile idiopathic arthritis and can be effective in controlling disease in many patients. A significant proportion of patients experience nausea and vomiting induced by methotrexate therapy, which can lead to decreased quality of life and discontinuation of treatment with methotrexate. Many strategies have been employed in attempts to reduce methotrexate-induced nausea, including folate supplementation, switching from oral to subcutaneous methotrexate, anti-emetic therapy, behavioral therapy, and others. Anticipatory nausea can be difficult to treat, making primary prevention of nausea with anti-emetics an attractive approach. Understanding the prevalence and impact of methotrexate-induced nausea, as well as potentially effective interventions, may help maximize the therapeutic benefits of methotrexate.

Milia-like idiopathic calcinosis cutis in a child with Down syndrome.

PubMed

Kumar, Piyush; Savant, Sushil S; Nimisha, Esther; Das, Anupam; Debbarman, Panchami

2016-05-15

Idiopathic calcinosis cutis refers to progressive deposition of crystals of calcium phosphate in the skin and other areas of the body, in the absence of any inciting factor. Idiopathic calcinosis cutis may sometimes take the form of small, milia-like lesions. Most commonly, such milia like lesions are seen in the setting of Down syndrome. Herein, we report a 5-year-old girl with multiple asymptomatic discrete milia-like firm papules distributed over the face and extremities. A diagnosis of milia-like idiopathic calcinosis cutis associated with Down Syndrome was provisionally made and was confirmed by histopathology and karyotyping.

Idiopathic hypertrophic pachymeningitis presenting with occipital neuralgia.

PubMed

Auboire, Laurent; Boutemy, Jonathan; Constans, Jean Marc; Le Gallou, Thomas; Busson, Philippe; Bienvenu, Boris

2015-03-01

Although occipital neuralgia is usually caused by degenerative arthropathy, nearly 20 other aetiologies may lead to this condition. We present the first case report of hypertrophic pachymeningitis revealed by isolated occipital neuralgia. Idiopathic hypertrophic pachymeningitis is a plausible cause of occipital neuralgia and may present without cranial-nerve palsy. There is no consensus on the treatment for idiopathic hypertrophic pachymeningitis, but the usual approach is to start corticotherapy and then to add immunosuppressants. When occipital neuralgia is not clinically isolated or when a first-line treatment fails, another disease diagnosis should be considered. However, the cost effectiveness of extended investigations needs to be considered.

Risk of revision surgery for adult idiopathic scoliosis: a survival analysis of 517 cases over 25 years.

PubMed

Riouallon, Guillaume; Bouyer, Benjamin; Wolff, Stéphane

2016-08-01

Little is known about the long-term status of patients operated for spine deformities. The aim of this study was to determine the survival of primary fusion in adult idiopathic scoliosis and identify the risk factors of revision surgery. Adult patients who underwent primary fusion for idiopathic scoliosis between 1983 and 2011 were included in a continuous monocentric retrospective series. Any additional surgery was registered for survival analysis. Survival and follow-up were estimated by the Kaplan-Meier method and an analysis was performed to identify the risk factors of revision surgery. This series included 447 women (86.5 %) and 70 men (13.5 %) reviewed after a mean follow-up of 7 years (range 0-26.4). Mean age was 44.4 years. Fusion was performed on a median 11 levels (range 3-15); revision rate was 13 % (CI 10-17), 18 % (CI 14-23) and 20 % (CI 16-26) at 5, 10 and 15 years, respectively. Revision surgery was associated with age, anterior release, length of fusion, the inferior limit of fusion, post-operative sagittal balance and junctional kyphosis. The length of fusion (HR 1.13 per vertebrae fused, p = 0.007) and the lower limit of fusion (HR 5.9, p < 0.001) remained independent predictors of revision surgery on multivariate analysis. This series evaluated the risk of revision surgery following spinal fusion for idiopathic scoliosis. Our results show that the risk seemed to increase linearly with a rate of nearly 20 % after 10 years. The length and lower limit of fusion are the main risk factors for revision surgery. Level IV (e.g. case series).

Reduced proliferation and osteocalcin expression in osteoblasts of male idiopathic osteoporosis.

PubMed

Ruiz-Gaspà, Sílvia; Blanch-Rubió, Josep; Ciria-Recasens, Manuel; Monfort, Jordi; Tío, Laura; Garcia-Giralt, Natàlia; Nogués, Xavier; Monllau, Joan C; Carbonell-Abelló, Jordi; Pérez-Edo, Lluis

2010-03-01

Osteoporosis is characterized by low bone mineral density (BMD), resulting in increasing susceptibility to bone fractures. In men, it has been related to some diseases and toxic habits, but in some instances the cause of the primary--or idiopathic--osteoporosis is not apparent. In a previous study, our group compared histomorphometric measurements in cortical and cancellous bones from male idiopathic osteoporosis (MIO) patients to those of control subjects and found reduced bone formation without major differences in bone resorption. To confirm these results, this study analyzed the etiology of this pathology, examining the osteoblast behavior in vitro. We compared two parameters of osteoblast activity in MIO patients and controls: osteoblastic proliferation and gene expression of COL1A1 and osteocalcin, in basal conditions and with vitamin D(3) added. All these experiments were performed from a first-passage osteoblastic culture, obtained from osteoblasts that had migrated from the transiliac explants to the plate. The results suggested that the MIO osteoblast has a slower proliferation rate and decreased expression of genes related to matrix formation, probably due to a lesser or slower response to some stimulus. We concluded that, contrary to female osteoporosis, in which loss of BMD is predominantly due to increased resorption, low BMD in MIO seems to be due to an osteoblastic defect.

From bad to worse: when lung cancer complicates idiopathic pulmonary fibrosis.

PubMed

Strock, Stephen B; Alder, Jonathan K; Kass, Daniel J

2018-04-01

Patients with idiopathic pulmonary fibrosis have a significantly increased risk for the development of lung cancer. The morbidity and mortality of this disease combination are substantial, and, unfortunately, there are currently few data to help guide clinicians in its diagnosis and treatment. In a recent issue of this journal, Hwang et al presented one of the first studies to evaluate lung cancer in patients with idiopathic pulmonary fibrosis at the molecular level. They demonstrate variants in regulators of the cell cycle, which are known to be important in malignant transformation and may also be important in the pathogenesis of idiopathic pulmonary fibrosis. Further understanding of the pathogenic overlap between lung cancer and idiopathic pulmonary fibrosis could help point the direction to specific diagnostic modalities and targeted treatment of both conditions in the future. Copyright © 2017 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd. Copyright © 2017 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

A case report of nephrogenic diabetes insipidus with idiopathic Fanconi syndrome in a child who presented with vitamin D resistant rickets.

PubMed

Patra, Soumya; Nadri, Gulnaz; Chowdhary, Harish; Pemde, Harish K; Singh, Varinder; Chandra, Jagdish

2014-05-01

Fanconi syndrome is a complex of multiple tubular dysfunctions of proximal tubular cells, occurring alone or in association with a variety of inherited (primary) or acquired (secondary) disorders. It is characterized by aminoaciduria, normoglycemic glycosuria, tubular proteinuria without hematuria, metabolic acidosis without anion gap and excessive urinary excretion of phosphorous, calcium, uric acid, bicarbonate, sodium, potassium and magnesium. Diabetes insipidus is a disease of collecting tubules and children mainly present with dehydration and hypernatremia. We are reporting the first case of idiopathic Fanconi's syndrome along with nephrogenic diabetes insipidus in a child who presented to us with vitamin D resistant rickets. Medline search did not reveal any case of nephrogenic diabetes insipidus (NDI) associated with idiopathic Fanconi syndrome. We hypothesized that the NDI may be due to to severe hypokalemia induced tubular dysfunction.

Genetics Home Reference: idiopathic pulmonary fibrosis

MedlinePlus

... structures at the ends of chromosomes known as telomeres . It is not well understood how defects in ... Collard HR, Wolters PJ, Garcia CK. Effect of telomere length on survival in patients with idiopathic pulmonary ...

Radiographic and MRI characteristics of lumbar disseminated idiopathic spinal hyperostosis and spondylosis deformans in dogs.

PubMed

Togni, A; Kranenburg, H J C; Morgan, J P; Steffen, F

2014-07-01

To evaluate clinical signs, describe lesions and differences in the magnetic resonance imaging appearance of spinal new bone formations classified as disseminated idiopathic spinal hyperostosis and/or spondylosis deformans on radiographs and compare degeneration status of the intervertebral discs using the Pfirrmann scale. Retrospective analysis of 18 dogs presented with spinal disorders using information from radiographic and magnetic resonance imaging examinations. All dogs were found to be affected with both disseminated idiopathic spinal hyperostosis and spondylosis deformans. Neurological signs due to foraminal stenosis associated with disseminated idiopathic spinal hyperostosis were found in two dogs. Spondylosis deformans was associated with foraminal stenosis and/or disc protrusion in 15 cases. The Pfirrmann score on magnetic resonance imaging was significantly higher in spondylosis deformans compared with disseminated idiopathic spinal hyperostosis and signal intensity of new bone due to disseminated idiopathic spinal hyperostosis was significantly higher compared to spondylosis deformans. Differences between disseminated idiopathic spinal hyperostosis and spondylosis deformans found on magnetic resonance imaging contribute to an increased differentiation between the two entities. Clinically relevant lesions in association with disseminated idiopathic spinal hyperostosis were rare compared to those seen with spondylosis deformans. © 2014 British Small Animal Veterinary Association.

Giant scrotal elephantiasis: an idiopathic case.

PubMed

Dianzani, C; Gaspardini, F; Persichetti, P; Brunetti, B; Pizzuti, A; Margiotti, K; Degener, A M

2010-01-01

Scrotal elephantiasis is very rare disease in industrialized countries, where it is mainly due to surgery, irradiation or malignancies. It can be defined as idiopathic only when the possible congenital, infectious and compressive causes are excluded. We report a case of massive scrotal lymphoedema in an adult Caucasian patient, in Italy. He presented an extremely voluminous scrotal mass measuring 50 x 47 x 13 cm (weight 18 kg), which extended below his knees, invalidating all his daily activities. The patient was hospitalized in order to undergo to surgical treatment. Although genetic causes were searched and the possible role of infectious agents and compressive factors was evaluated, no etiology was ascertained. Histopathologic examination showed non-specific chronic inflammation, confirming the diagnosis of idiopathic elephantiasis. One year after surgical treatment, the patient is healthy without recurrence signs.

Idiopathic hypersomnia: a report of three adolescent-onset cases in a two-generation family.

PubMed

Janácková, Sona; Motte, Jacques; Bakchine, Serge; Sforza, Emilia

2011-04-01

Idiopathic hypersomnia is an uncommon sleep disorder characterized by prolonged sleep time and excessive daytime sleepiness without cataplexy. This study concerned a case of familial occurrence. The proband expressed an idiopathic hypersomnia with long sleep time at the age of 12 years. Clinical interview and ad libitum polysomnographic study did not reveal any symptoms of narcolepsy or other sleep disorders. Family history revealed that a 20-year-old sister had experienced symptoms of hypersomnia from the age of 16 and their mother had been diagnosed with idiopathic hypersomnia previously. The diagnosis of idiopathic hypersomnia with long sleep time was confirmed in the sister by clinical interview and ad libitum polysomnography. Human leukocyte antigen (HLA) did not reveal the DQB1-0602 phenotype in the proband and relatives. This report confirms the hypothesis of a genetic predisposition in idiopathic hypersomnia.

Screening for Adolescent Idiopathic Scoliosis: US Preventive Services Task Force Recommendation Statement.

PubMed

Grossman, David C; Curry, Susan J; Owens, Douglas K; Barry, Michael J; Davidson, Karina W; Doubeni, Chyke A; Epling, John W; Kemper, Alex R; Krist, Alex H; Kurth, Ann E; Landefeld, C Seth; Mangione, Carol M; Phipps, Maureen G; Silverstein, Michael; Simon, Melissa A; Tseng, Chien-Wen

2018-01-09

Adolescent idiopathic scoliosis, a lateral curvature of the spine of unknown cause with a Cobb angle of at least 10°, occurs in children and adolescents aged 10 to 18 years. Idiopathic scoliosis is the most common form and usually worsens during adolescence before skeletal maturity. Severe spinal curvature may be associated with adverse long-term health outcomes (eg, pulmonary disorders, disability, back pain, psychological effects, cosmetic issues, and reduced quality of life). Early identification and effective treatment of mild scoliosis could slow or stop curvature progression before skeletal maturity, thereby improving long-term outcomes in adulthood. To update the 2004 US Preventive Services Task Force (USPSTF) recommendation on screening for idiopathic scoliosis in asymptomatic adolescents. The USPSTF reviewed the evidence on the benefits and harms of screening for and treatment of adolescent idiopathic scoliosis. The USPSTF found no direct evidence on screening for adolescent idiopathic scoliosis and health outcomes and no evidence on the harms of screening. The USPSTF found inadequate evidence on treatment with exercise and surgery. It found adequate evidence that treatment with bracing may slow curvature progression in adolescents with mild or moderate curvature severity (Cobb angle <40° to 50°); however, evidence on the association between reduction in spinal curvature in adolescence and long-term health outcomes in adulthood is inadequate. The USPSTF found inadequate evidence on the harms of treatment. Therefore, the USPSTF concludes that the current evidence is insufficient and that the balance of benefits and harms of screening for adolescent idiopathic scoliosis cannot be determined. The USPSTF concludes that the current evidence is insufficient to assess the balance of benefits and harms of screening for adolescent idiopathic scoliosis in children and adolescents aged 10 to 18 years. (I statement).

Electronic protocol of respiratory physical therapy in patients with idiopathic adolescent scoliosis.

PubMed

Cano, Danila Vieira Baldini; Malafaia, Osvaldo; Alves, Vera Lúcia dos Santos; Avanzi, Osmar; Pinto, José Simão de Paula

2011-01-01

To create a clinical database of respiratory function in patients with adolescent idiopathic scoliosis; computerize and store this clinical data through the use of a software; incorporate this electronic protocol to the SINPE© (Integrated Electronic Protocols System) and analyze a pilot project with interpretation of results. From the literature review a computerized data bank of clinical data of postural deviations was set up (master protocol). Upon completion of the master protocol a specific protocol of respiratory function in patients with adolescent idiopathic scoliosis was designed and a pilot project was conducted to collect and analyze data from ten patients. It was possible to create the master protocol of postural deviations and the specific protocol of respiratory function in patients with adolescent idiopathic scoliosis. The data collected in the pilot project was processed by the SINPE ANALYZER©, generating charts and statistics. The establishment of the clinical database of adolescent idiopathic scoliosis was possible. Computerization and storage of clinical data using the software were viable. The electronic protocol of adolescent idiopathic scoliosis could be incorporated into the SINPE© and its use in the pilot project was successful.

Periprosthetic Tissue Loss in Patients With Idiopathic Vitreous Inflammation After the Boston Keratoprosthesis.

PubMed

Grassi, Christina M; Cruzat, Andrea; Taniguchi, Elise V; Crnej, Alja; Colby, Kathryn A; Dohlman, Claes H; Chodosh, James

2015-11-01

Idiopathic vitritis is a poorly understood complication after Boston keratoprosthesis surgery with unclear etiology. We sought to determine whether an association exists between periprosthetic corneal tissue loss and the development of idiopathic vitritis in keratoprosthesis recipients. Thirteen Boston type I keratoprosthesis recipient eyes with a history of idiopathic vitritis and 34 type I keratoprosthesis recipient eyes with no history of idiopathic vitritis underwent anterior segment optical coherence tomography (AS-OCT) at a median time postoperatively of 2.4 years versus 1.9 years (range, 0.5-14.2 vs. 0.1-13.6 years), respectively. Areas of corneal graft tissue loss ("gaps") around the keratoprosthesis stem were identified and analyzed by 2 masked observers. The difference in the presence, number, and size of gaps was compared between cases and controls. A periprosthetic gap was identified more commonly in idiopathic vitritis cases than in controls on AS-OCT (11/13, 86% vs. 11/34, 33.3%, P < 0.001). The number of gaps between cases and controls was also significantly different (2.6 ± 1.6 vs. 0.5 ± 0.8, P < 0.001), but not the estimated gap area (0.056 ± 0.049 mm² vs. 0.039 ± 0.025 mm², P = 0.22). A significantly higher proportion of keratoprosthesis recipient eyes with idiopathic vitritis had corneal tissue loss around the keratoprosthesis stem than did controls. Tissue loss could serve as an entry point for debris or bacterial components, triggering idiopathic vitritis. Our study underscores the utility of AS-OCT imaging in the postoperative management of keratoprosthesis patients.

Debate: idiopathic short stature should be treated with growth hormone.

PubMed

Ambler, Geoffrey R; Fairchild, Jan; Wilkinson, Dominic J C

2013-03-01

In this paper we outline the case for and against the treatment of idiopathic short stature with growth hormone. Drs Ambler and Fairchild argue that many of those with 'idiopathic' short stature are not 'short, normal children' and will ultimately receive molecular diagnoses. They also argue that there is a subset of children who suffer negative psychosocial consequences of their stature for whom growth hormone therapy is effective. Growth hormone has a very good safety record and is likely to be as cost-effective in idiopathic short-stature as in some other conditions that are currently funded. Dr Wilkinson counters that short stature is not associated with physical or psychological illness, and that there is no evidence that growth hormone improves psychological or physical wellbeing. Moreover, growth hormone for idiopathic short stature represents a form of enhancement rather than treatment, and is not a fair use of resources. Socially mediated disadvantage should be treated by attention to prejudice and not by hormone treatment. © 2012 The Authors. Journal of Paediatrics and Child Health © 2012 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

Pelvic confined idiopathic retroperitoneal fibrosis mimicking a large tumor.

PubMed

Salemis, N S; Tsiambas, E; Tsohataridis, E

2009-01-01

Idiopathic retroperitoneal fibrosis (IRF) entirely confined to the pelvic cavity is an extremely rare clinical entity. Herein, is described the case of a 36- year old male who presented with clinical and imaging manifestations of a large pelvic tumor. Exploratory laparotomy revealed a large mass in the right pelvis originating from the retroperitoneal space, displacing the right iliac vessels, the right ureter and the urinary bladder completely to the left. A laborious resection of the mass measuring 14 x 10cm was performed. Histopathological examination and detailed immunohistochemistry analysis were suggestive of idiopathic retroperitoneal fibrosis with no evidence of malignancy. This is a very rare case regarding localization and clinical presentation of idiopathic retroperitoneal fibrosis. We conclude that IRF should be included in the differential diagnosis of patients presenting with a pelvic mass even if there is no involvement of the typical para aortic area.

Locomotor skills and balance strategies in adolescents idiopathic scoliosis.

PubMed

Mallau, Sophie; Bollini, Gérard; Jouve, Jean-Luc; Assaiante, Christine

2007-01-01

Locomotor balance control assessment was performed to study the effect of idiopathic scoliosis on head-trunk coordination in 17 patients with adolescent idiopathic scoliosis (AIS) and 16 control subjects. The aim of this study was to explore the functional effects of structural spinal deformations like idiopathic scoliosis on the balance strategies used during locomotion. Up to now, the repercussion of the idiopathic scoliosis on head-trunk coordination and balance strategies during locomotion is relatively unknown. Seventeen patients with AIS (mean age 14 years 3 months, 10 degrees < Cobb angle > 30 degrees) and 16 control subjects (mean age 14 years 1 month) were tested during various locomotor tasks: walking on the ground, walking on a line, and walking on a beam. Balance control was examined in terms of rotation about the vertical axis (yaw) and on a frontal plane (roll). Kinematics of foot, pelvis, trunk, shoulder, and head rotations were measured with an automatic optical TV image processor in order to calculate angular dispersions and segmental stabilizations. Decreasing the walking speed is the main adaptive strategy used in response to balance problems in control subjects as well as patients with AIS. However, patients with AIS performed walking tasks more slowly than normal subjects (around 15%). Moreover, the pelvic stabilization is preserved, despite the structural changes affecting the spine. Lastly, the biomechanical defect resulting from idiopathic scoliosis mainly affects the yaw head stabilization during locomotion. Patients with AIS show substantial similarities with control subjects in adaptive strategies relative to locomotor velocity as well as balance control based on segmental stabilization. In contrast, the loss of the yaw head stabilization strategies, mainly based on the use of vestibular information, probably reflects the presence of vestibular deficits in the patients with AIS.

Fluid Distribution Pattern in Adult-Onset Congenital, Idiopathic, and Secondary Normal-Pressure Hydrocephalus: Implications for Clinical Care.

PubMed

Yamada, Shigeki; Ishikawa, Masatsune; Yamamoto, Kazuo

2017-01-01

In spite of growing evidence of idiopathic normal-pressure hydrocephalus (NPH), a viewpoint about clinical care for idiopathic NPH is still controversial. A continuous divergence of viewpoints might be due to confusing classifications of idiopathic and adult-onset congenital NPH. To elucidate the classification of NPH, we propose that adult-onset congenital NPH should be explicitly distinguished from idiopathic and secondary NPH. On the basis of conventional CT scan or MRI, idiopathic NPH was defined as narrow sulci at the high convexity in concurrent with enlargement of the ventricles, basal cistern and Sylvian fissure, whereas adult-onset congenital NPH was defined as huge ventricles without high-convexity tightness. We compared clinical characteristics and cerebrospinal fluid distribution among 85 patients diagnosed with idiopathic NPH, 17 patients with secondary NPH, and 7 patients with adult-onset congenital NPH. All patients underwent 3-T MRI examinations and tap-tests. The volumes of ventricles and subarachnoid spaces were measured using a 3D workstation based on T2-weighted 3D sequences. The mean intracranial volume for the patients with adult-onset congenital NPH was almost 100 mL larger than the volumes for patients with idiopathic and secondary NPH. Compared with the patients with idiopathic or secondary NPH, patients with adult-onset congenital NPH exhibited larger ventricles but normal sized subarachnoid spaces. The mean volume ratio of the high-convexity subarachnoid space was significantly less in idiopathic NPH than in adult-onset congenital NPH, whereas the mean volume ratio of the basal cistern and Sylvian fissure in idiopathic NPH was >2 times larger than that in adult-onset congenital NPH. The symptoms of gait disturbance, cognitive impairment, and urinary incontinence in patients with adult-onset congenital NPH tended to progress more slowly compared to their progress in patients with idiopathic NPH. Cerebrospinal fluid distributions and

Idiopathic pulmonary fibrosis: evolving concepts.

PubMed

Ryu, Jay H; Moua, Teng; Daniels, Craig E; Hartman, Thomas E; Yi, Eunhee S; Utz, James P; Limper, Andrew H

2014-08-01

Idiopathic pulmonary fibrosis (IPF) occurs predominantly in middle-aged and older adults and accounts for 20% to 30% of interstitial lung diseases. It is usually progressive, resulting in respiratory failure and death. Diagnostic criteria for IPF have evolved over the years, and IPF is currently defined as a disease characterized by the histopathologic pattern of usual interstitial pneumonia occurring in the absence of an identifiable cause of lung injury. Understanding of the pathogenesis of IPF has shifted away from chronic inflammation and toward dysregulated fibroproliferative repair in response to alveolar epithelial injury. Idiopathic pulmonary fibrosis is likely a heterogeneous disorder caused by various interactions between genetic components and environmental exposures. High-resolution computed tomography can be diagnostic in the presence of typical findings such as bilateral reticular opacities associated with traction bronchiectasis/bronchiolectasis in a predominantly basal and subpleural distribution, along with subpleural honeycombing. In other circumstances, a surgical lung biopsy may be needed. The clinical course of IPF can be unpredictable and may be punctuated by acute deteriorations (acute exacerbation). Although progress continues in unraveling the mechanisms of IPF, effective therapy has remained elusive. Thus, clinicians and patients need to reach informed decisions regarding management options including lung transplant. The findings in this review were based on a literature search of PubMed using the search terms idiopathic pulmonary fibrosis and usual interstitial pneumonia, limited to human studies in the English language published from January 1, 2000, through December 31, 2013, and supplemented by key references published before the year 2000. Copyright © 2014 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.

[Physiopathology of idiopathic hypersomnia. Current studies and new orientations].

PubMed

Billiard, M; Rondouin, G; Espa, F; Dauvilliers, Y; Besset, A

2001-11-01

In 1976 Bedrich Roth coined the term "idiopathic hypersomnia" and described two forms of the disease, one monosymptomatic, manifested only by excessive daytime sleepiness, and one polysymptomatic, characterized by excessive daytime sleepiness, nocturnal sleep of abnormally long duration and signs of "sleep drunkenness" on awakening. In comparison with that of narcolepsy, the pathophysiology of idiopathic hypersomnia remains poorly known. There are two main reasons for that: the absence of clinical and polysomnographic criteria pathognomonic or at least characteristic of the condition, as the cataplexies and the sleep onset REM periods of narcolepsy, and also the absence of a natural animal model comparable with the canine model of narcolepsy. The first investigations have stressed the frequent familial pattern of idiopathic hypersomnia. Later on biochemical assays have been performed in the CSF with results in favour of a dysfunction of noradrenergic systems. In the light of the two process model of sleep regulation in which sleep propensity is determined by a homeostatic process S and a circadian process C and of the later three-process model of regulation in which sleepiness/alertness are simulated by the combined action of a homeostatic process, a circadian process and sleep inertia, we suggest that idiopathic hypersomnia is not a pathological entity in itself, but rather the consequence of chronic sleep deprivation in very long sleepers.

Interventional Radiologic Treatment for Idiopathic Portal Hypertension

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hirota, Shozo; Ichikawa, Satoshi; Matsumoto, Shinichi

1999-07-15

Purpose: To evaluate the usefulness of interventional radiological treatment for idiopathic portal hypertension. Methods: Between 1995 and 1998, we performed an interventional radiological treatment in five patients with idiopathic portal hypertension, four of whom had refused surgery and one of whom had undergone surgery. Three patients with gastroesophageal varices (GEV) were treated by partial splenic embolization (PSE), one patient with esophageal varices (EV) and massive ascites by transjugular intrahepatic portosytemic shunt (TIPS) and PSE, and one patient with GEV by percutaneous transhepatic obliteration (PTO). Midterm results were analyzed in terms of the effect on esophageal and/or gastric varices. Results: Inmore » one woman with severe GEV who underwent three sessions of PSE, there was endoscopic confirmation that the GEV had disappeared. In one man his EV shrunk markedly after two sessions of PSE. In two patients slight reduction of the EV was obtained with one application of PSE combined with endoscopic variceal ligation therapy. PTO for GV in one patient resulted in good control of the varices. All patients have survived for 16-42 months since the first interventional treatment, and varices are well controlled. Conclusion: Interventional radiological treatment is effective for patients with idiopathic portal hypertension, whether or not they have undergone surgery.« less

Peroral Endoscopic Myotomy for Esophageal Achalasia: Outcomes of the First 100 Patients With Short-term Follow-up.

PubMed

Familiari, Pietro; Gigante, Giovanni; Marchese, Michele; Boskoski, Ivo; Tringali, Andrea; Perri, Vincenzo; Costamagna, Guido

2016-01-01

Aim of this study is to report the mid-term outcomes of a large series of patients treated with peroral endoscopic myotomy (POEM) in a single European center. POEM is a recently developed treatment of achalasia, which combines the efficacy of surgical myotomy, with the benefits of an endoscopic procedure. Previous studies, including few patients with a short-term follow-up, showed excellent results on dysphagia relief. The first 100 adult patients treated in a single tertiary referral center were retrospectively identified and included in this study (41 men, mean age 48.4 years). Patients were treated according to a standard technique. Follow-up data, including clinical evaluation, and results of esophagogastroduodenoscopy (EGD), manometry, and pH monitoring were collected and analyzed. POEM was completed in 94% of patients. Mean operative time was 83 minutes (49-140  minutes). No complications occurred. Patients were fed after a median of 2 days (1-4 days). A mean follow-up of 11 months (3-24 months) was available for 92 patients. Clinical success was documented in 94.5% of patients. Twenty-four-hour pH monitoring documented Gastro-Esophageal Reflux Disease (GERD) in 53.4% of patients. However, only a minority of patients had heartburn (24.3%) or esophagitis (27.4%), and these patients were successfully treated with proton-pump inhibitors. Our results confirm the efficacy of POEM in a large series of patients, with a mean follow-up of 11 months. Should our results be confirmed by long-term follow-up studies, POEM may become one of the first-line therapies of achalasia in the next future.

Neurodevelopment in preschool idiopathic toe-walkers.

PubMed

Martín-Casas, P; Ballestero-Pérez, R; Meneses-Monroy, A; Beneit-Montesinos, J V; Atín-Arratibel, M A; Portellano-Pérez, J A

2017-09-01

Idiopathic toe walking, a differential diagnosis for neurological and orthopaedic disorders, has been associated with neurodevelopmental alterations. Neurodevelopmental assessment at early ages using specific tests may improve management and follow-up of these patients. The aim of our study is to analyse the neurodevelopmental characteristics of preschool idiopathic toe-walkers (ITW) by comparing them to a control group. Our descriptive cross-sectional study compared possible risk factors, neurodevelopmental characteristics, and scores on the Child Neuropsychological Maturity Questionnaire (CUMANIN) between a group of 56 ITWs aged 3 to 6 and a control group including 40 children. The proportion of males was significantly higher in the ITW group (P=.008). The percentage of patients with a family history (P=.000) and biological risk factors during the perinatal period (P=.032) was also higher in this group. According to the parents' reports, motor coordination in ITWs was significantly poorer (59%; P=.009). ITWs scored significantly lower on CUMANIN subscales of psychomotricity (=0,001) and memory (P=.001), as well as in verbal development (P=.000), non-verbal development (P=.026), and overall development (P=.004). Foot preference was less marked in the ITW group (P=.047). The neurodevelopmental characteristics of our sample suggest that idiopathic toe walking is a marker of neurodevelopmental impairment. However, further studies are necessary to confirm these findings. Copyright © 2016 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

Idiopathic noncirrhotic portal hypertension: current perspectives.

PubMed

Riggio, Oliviero; Gioia, Stefania; Pentassuglio, Ilaria; Nicoletti, Valeria; Valente, Michele; d'Amati, Giulia

2016-01-01

The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis.

Idiopathic pulmonary fibrosis. A rare cause of scintigraphic ventilation-perfusion mismatch

DOE Office of Scientific and Technical Information (OSTI.GOV)

Pochis, W.T.; Krasnow, A.Z.; Collier, B.D.

1990-05-01

A case of idiopathic pulmonary fibrosis with multiple areas of mismatch on ventilation-perfusion lung imaging in the absence of pulmonary embolism is presented. Idiopathic pulmonary fibrosis is one of the few nonembolic diseases producing a pulmonary ventilation-perfusion mismatch. In this condition, chest radiographs may not detect the full extent of disease, and xenon-133 ventilation imaging may be relatively insensitive to morbid changes in small airways. Thus, when examining patients with idiopathic pulmonary fibrosis, one should be aware that abnormal perfusion imaging patterns without matching ventilation abnormalities are not always due to embolism. In this setting, contrast pulmonary angiography is oftenmore » needed for accurate differential diagnosis.« less

Idiopathic Hypersomnia.

PubMed

Trotti, Lynn Marie

2017-09-01

Idiopathic hypersomnia (IH) is a chronic neurologic disorder of daytime sleepiness, accompanied by long sleep times, unrefreshing sleep, difficulty in awakening, cognitive dysfunction, and autonomic symptoms. The cause is unknown; a genetic predisposition is suggested. Autonomic, inflammatory, or immune dysfunction has been proposed. Diagnosis involves a clinical history and objective testing. There are no approved treatments for IH, but modafinil is typically considered first-line. A substantial fraction of patients with IH are refractory or intolerant to standard treatments, and different treatment strategies using novel therapeutics are necessary. Even with current treatment options, quality of life and safety may remain impaired. Copyright © 2017 Elsevier Inc. All rights reserved.

Sensitivity Analyses of the Change in FVC in a Phase 3 Trial of Pirfenidone for Idiopathic Pulmonary Fibrosis.

PubMed

Lederer, David J; Bradford, Williamson Z; Fagan, Elizabeth A; Glaspole, Ian; Glassberg, Marilyn K; Glasscock, Kenneth F; Kardatzke, David; King, Talmadge E; Lancaster, Lisa H; Nathan, Steven D; Pereira, Carlos A; Sahn, Steven A; Swigris, Jeffrey J; Noble, Paul W

2015-07-01

FVC outcomes in clinical trials on idiopathic pulmonary fibrosis (IPF) can be substantially influenced by the analytic methodology and the handling of missing data. We conducted a series of sensitivity analyses to assess the robustness of the statistical finding and the stability of the estimate of the magnitude of treatment effect on the primary end point of FVC change in a phase 3 trial evaluating pirfenidone in adults with IPF. Source data included all 555 study participants randomized to treatment with pirfenidone or placebo in the Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis (ASCEND) study. Sensitivity analyses were conducted to assess whether alternative statistical tests and methods for handling missing data influenced the observed magnitude of treatment effect on the primary end point of change from baseline to week 52 in FVC. The distribution of FVC change at week 52 was systematically different between the two treatment groups and favored pirfenidone in each analysis. The method used to impute missing data due to death had a marked effect on the magnitude of change in FVC in both treatment groups; however, the magnitude of treatment benefit was generally consistent on a relative basis, with an approximate 50% reduction in FVC decline observed in the pirfenidone group in each analysis. Our results confirm the robustness of the statistical finding on the primary end point of change in FVC in the ASCEND trial and corroborate the estimated magnitude of the pirfenidone treatment effect in patients with IPF. ClinicalTrials.gov; No.: NCT01366209; URL: www.clinicaltrials.gov.

Spinal Manipulative Therapy for Adolescent Idiopathic Scoliosis: A Systematic Review.

PubMed

Théroux, Jean; Stomski, Norman; Losco, Christine Dominique; Khadra, Christelle; Labelle, Hubert; Le May, Sylvie

The purpose of this study was to perform a systematic review of clinical trials of spinal manipulative therapy for adolescent idiopathic scoliosis. Search strategies were developed for PubMed, CINHAL, and CENTRAL databases. Studies were included through June 2016 if they were prospective trials that evaluated spinal manipulative therapy (eg, chiropractic, osteopathic, physical therapy) for adolescent idiopathic scoliosis. Data were extracted and assessed by 2 independent reviewers. Cochrane risk of bias tools were used to assess the quality of the included studies. Data were reported qualitatively because heterogeneity prevented statistical pooling. Four studies satisfied the inclusion criteria and were critically appraised. The findings of the included studies indicated that spinal manipulative therapy might be effective for preventing curve progression or reducing Cobb angle. However, the lack of controls and small sample sizes precluded robust estimation of the interventions' effect sizes. There is currently insufficient evidence to establish whether spinal manipulative therapy may be beneficial for adolescent idiopathic scoliosis. The results of the included studies suggest that spinal manipulative therapy may be a promising treatment, but these studies were all at substantial risk of bias. Further high-quality studies are warranted to conclusively determine if spinal manipulative therapy may be effective in the management of adolescent idiopathic scoliosis. Copyright © 2017. Published by Elsevier Inc.

STELLATE NONHEREDITARY IDIOPATHIC FOVEOMACULAR RETINOSCHISIS ACCOMPANIED BY CONTRALATERAL PERIPHERAL RETINOSCHISIS.

PubMed

Ahmed, Daniel; Stattin, Martin; Glittenberg, Carl; Krebs, Ilse; Ansari-Shahrezaei, Siamak

2017-01-16

To present a patient with stellate nonhereditary idiopathic foveomacular retinoschisis on one eye and peripheral retinoschisis without foveal affection on the other eye. A case report with complete workup of family history and clinical examination, including multimodal imaging with optical coherence tomography and angiography, fluorescein angiography, and infrared fundus imaging. Genetic testing for gene mutation XRLS1 was performed. A white woman with unremarkable medical history presented with stellate foveal splitting of the outer plexiform layer on the right eye and peripheral splitting of the outer plexiform layer on both eyes. All known allegeable trigger factors for the existence of a hereditary or acquired foveomacular retinoschisis were ruled out either by clinical presentation or genetic testing. This led to the diagnosis of stellate nonhereditary idiopathic foveomacular retinoschisis with central involvement only present on one eye. Although peripheral schisis of the outer plexiform layer is often concomitant with central splitting in X-linked juvenile retinoschisis, this is the first known report of nonhereditary cleavage of the outer plexiform layer of the peripheral retina without central affection in a patient with documented stellate nonhereditary idiopathic foveomacular retinoschisis on the other eye. These findings suggest an accurate bilateral examination of the peripheral retina while confirming the diagnose of stellate nonhereditary idiopathic foveomacular retinoschisis.

Genetic (idiopathic) epilepsy with photosensitive seizures includes features of both focal and generalized seizures.

PubMed

Xue, Jiao; Gong, Pan; Yang, Haipo; Liu, Xiaoyan; Jiang, Yuwu; Zhang, Yuehua; Yang, Zhixian

2018-04-19

Clinically, some patients having genetic (idiopathic) epilepsy with photosensitive seizures were difficult to be diagnosed. We aimed to discuss whether the genetic (idiopathic) epilepsy with photosensitive seizures is a focal entity, a generalized entity or a continuum. Twenty-two patients with idiopathic epilepsies and photoconvulsive response (PCR) were retrospectively recruited. In the medical records, the seizure types included "generalized tonic-clonic seizures (GTCS)" in 15, "partial secondarily GTCS (PGTCS)" in 3, partial seizures (PS) in 3, myoclonic seizures in 2, eyelid myoclonus in one, and only febrile seizures in one. Seizure types of PCR included GTCS (1/22), PGTCS (6/22), PS (9/22), electrical seizures (ES) (3/22) and GTCS/PGTCS (3/22). Combined the medical history with PCR results, they were diagnosed as: idiopathic (photosensitive) occipital lobe epilepsy (I(P)OE) in 12, genetic (idiopathic) generalized epilepsy (GGE) in one, GGE/I(P)OE in 5, pure photosensitive seizure in one, and epilepsy with undetermined generalized or focal seizure in 3. So, the dichotomy between generalized and focal seizures might have been out of date regarding to pathophysiological advances in epileptology. To some extent, it would be better to recognize the idiopathic epilepsy with photosensitive seizures as a continuum between focal and generalized seizures.

Enlarged Dural Sac in Idiopathic Bronchiectasis Implicates Heritable Connective Tissue Gene Variants

PubMed Central

Birchard, Katherine R.; Lowe, Jared R.; Patrone, Michael V.

2016-01-01

Rationale: Patients with idiopathic bronchiectasis are predominantly female and have an asthenic body morphotype and frequent nontuberculous mycobacterial respiratory infections. They also demonstrate phenotypic features (scoliosis, pectus deformity, mitral valve prolapse) that are commonly seen in individuals with heritable connective tissue disorders. Objectives: To determine whether lumbar dural sac size is increased in patients with idiopathic bronchiectasis as compared with control subjects, and to assess whether dural sac size is correlated with phenotypic characteristics seen in individuals with heritable connective tissue disorders. Methods: Two readers blinded to diagnosis measured anterior–posterior and transverse dural sac diameter using L1–L5 magnetic resonance images of 71 patients with idiopathic bronchiectasis, 72 control subjects without lung disease, 29 patients with cystic fibrosis, and 24 patients with Marfan syndrome. We compared groups by pairwise analysis of means, using Tukey’s method to adjust for multiple comparisons. Dural sac diameter association with phenotypic and clinical features was also tested. Measurements and Main Results: The L1–L5 (average) anterior–posterior dural sac diameter of the idiopathic bronchiectasis group was larger than those of the control group (P < 0.001) and the cystic fibrosis group (P = 0.002). There was a strong correlation between increased dural sac size and the presence of pulmonary nontuberculous mycobacterial infection (P = 0.007) and long fingers (P = 0.003). A trend toward larger dural sac diameter was seen in those with scoliosis (P = 0.130) and those with a family history of idiopathic bronchiectasis (P = 0.149). Conclusions: Individuals with idiopathic bronchiectasis have an enlarged dural sac diameter, which is associated with pulmonary nontuberculous mycobacterial infection, long fingers, and family history of idiopathic bronchiectasis. These findings support our

McArdle's disease: A differential diagnosis of idiopathic toe walking.

PubMed

Pomarino, David; Martin, Stephan; Pomarino, Andrea; Morigeau, Stefanie; Biskup, Saskia

2018-06-01

Idiopathic toe walking (ITW) is a pathological gait pattern in which children walk on their tip toes with no orthopedic or neurological reason. Physiological characteristics of the gastrocnemius muscles, the Achilles tendon, and the foot of toe walkers differ from subjects with a plantigrade walking pattern. McArdle's disease is characterized by the inability to break down muscle glycogen. It is an autosomal-recessive condition, characterized by low exercise tolerance, muscular atrophy at the shoulder girdle, episodes of myoglobinuria after vigorous physical activities and the occurrence of the second wind phenomenon. The aim of this review is to present the case studies of two subjects who were originally diagnosed as idiopathic toe walkers, but were then found to have McArdle's disease. This review will describe some physical characteristics that distinguish McArdle´s disease from Idiopathic toe walkers.

French consensus. Idiopathic hypersomnia: Investigations and follow-up.

PubMed

Leu-Semenescu, S; Quera-Salva, M-A; Dauvilliers, Y

Idiopathic hypersomnia is a rare, central hypersomnia, recently identified and to date of unknown physiopathology. It is characterised by a more or less permanent, excessive daytime sleepiness, associated with long and unrefreshing naps. Night-time sleep is of good quality, excessive in quantity, associated with sleep inertia in the subtype previously described as "with long sleep time". Diagnosis of idiopathic hypersomnia is complex due to the absence of a quantifiable biomarker, the heterogeneous symptoms, which overlap with the clinical picture of type 2 narcolepsy, and its variable evolution over time. Detailed evaluation enables other frequent causes of somnolence, such as depression or sleep deprivation, to be eliminated. Polysomnography and multiple sleep latency tests (MSLT) are essential to rule out other sleep pathologies and to objectify excessive daytime sleepiness. Sometimes the MSLT do not show excessive sleepiness, hence a continued sleep recording of at least 24hours is necessary to show prolonged sleep (>11h/24h). In this article, we propose recommendations for the work-up to be carried out during diagnosis and follow-up for patients suffering from idiopathic hypersomnia. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Do adolescents with idiopathic scoliosis have body schema disorders? A cross-sectional study.

PubMed

Picelli, Alessandro; Negrini, Stefano; Zenorini, Andrea; Iosa, Marco; Paolucci, Stefano; Smania, Nicola

2016-01-01

To date etiology of adolescent idiopathic scoliosis appears complex and still remains unclear. A distorted body schema has been proposed to be a part of a sequence of pathological events in the development of adolescent idiopathic scoliosis. To investigate the awareness of trunk misalignment in adolescents with idiopathic scoliosis. Information about 44 adolescents with idiopathic scoliosis was collected as follows: age; sex; handedness; family history of scoliosis; back pain; sport practice; shoulder and waist line symmetry; leg length; dorsal kyphosis; back hump; rehabilitation; scoliotic curve; Risser sign. We evaluated awareness of trunk misalignment with a graphic table displaying pictures of progressively increasing scoliotic curves. Patients were asked to indicate which picture corresponded to their perceived own spinal alignment. Patients with thoracolumbar scoliosis overestimated their actual thoracic spine curve. Patients with thoracic-thoracolumbar scoliosis underestimated their actual thoracolumbar spine curve and overestimated their actual lumbar spine curve. Scoliotic curve > 15°, double curve, younger age, back pain, family history of scoliosis and lower Risser score related with a misperception of trunk alignment. Our results support the hypothesis that adolescents with idiopathic scoliosis have an altered corporeal awareness of their trunk alignment.

Idiopathic noncirrhotic portal hypertension: current perspectives

PubMed Central

Riggio, Oliviero; Gioia, Stefania; Pentassuglio, Ilaria; Nicoletti, Valeria; Valente, Michele; d’Amati, Giulia

2016-01-01

The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis. PMID:27555800

Idiopathic burning mouth syndrome: a common treatment-refractory somatoform condition responsive to ECT.

PubMed

McGirr, Alexander; Davis, Lindsay; Vila-Rodriguez, Fidel

2014-04-30

Somatic symptom disorders are common causes of disability and suffering, and can pose significant management challenges. Idiopathic burning mouth syndrome is a challenging somatic symptom disorder with relatively high prevalence, particularly among post-menopausal women. Here, we present the case of a woman with severe treatment refractory idiopathic burning mouth syndrome and comorbid major depressive disorder, who was successfully treated with bitemporal electroconvulsive therapy. This case highlights the potential effectiveness of electroconvulsive therapy in idiopathic burning mouth syndrome when other treatment options have been exhausted. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Growth hormone treatment for growth hormone deficiency and idiopathic short stature: new guidelines shaped by the presence and absence of evidence.

PubMed

Grimberg, Adda; Allen, David B

2017-08-01

The Pediatric Endocrine Society recently published new guidelines for the use of human growth hormone (hGH) and human insulin-like growth factor-I (hIGF-I) treatment for growth hormone deficiency, idiopathic short stature, and primary IGF-I deficiency in children and adolescents. This review places the new guidelines in historical contexts of the life cycle of hGH and the evolution of US health care, and highlights their future implications. The new hGH guidelines, the first to be created by the Grading of Recommendations Assessment, Development and Evaluation approach, are more conservative than their predecessors. They follow an extended period of hGH therapeutic expansion at a time when US health care is pivoting toward value-based practice. There are strong supporting evidence and general agreement regarding the restoration of hormonal normalcy in children with severe deficiency of growth hormone or hIGF-I. More complex are issues related to hGH treatment to increase growth rates and heights of otherwise healthy short children with either idiopathic short stature or 'partial' isolated idiopathic growth hormone deficiency. The guidelines-developing process revealed fundamental questions about hGH treatment that still need evidence-based answers. Unless and until such research is performed, a more restrained hGH-prescribing approach is appropriate.

The effect of incremental distal gastric myotomy lengths on EGJ distensibility during POEM for achalasia

PubMed Central

Teitelbaum, Ezra N.; Sternbach, Joel M.; Khoury, Rym El; Soper, Nathaniel J.; Pandolfino, John E.; Kahrilas, Peter J.; Lin, Zhiyue; Hungness, Eric S.

2015-01-01

Background During peroral esophageal myotomy (POEM) for the treatment of achalasia, the optimal distal gastric myotomy length is unknown. In this study we used a functional lumen imaging probe (FLIP) to intraoperatively measure the effect of variable distal myotomy lengths on esophagogastric junction (EGJ) distensibility. Methods EGJ distensibility index (DI) (minimum cross-sectional area divided by intra-bag pressure) was measured with FLIP after each operative step. Each patient's myotomy was performed in four increments from proximal to distal: 1) an esophageal myotomy (from 6cm proximal to the EGJ, to 1cm proximal to it), 2) a myotomy ablating the lower esophageal sphincter (LES) complex (from 1cm proximal to the EGJ, to 1cm distal to it), 3) an initial gastric extension (from 1cm distal to the EGJ, to 2cm distal), and 4) a final gastric extension (from 2cm distal to the EGJ, to 3cm distal). Results Measurements were performed in 16 achalasia patients during POEM. POEM resulted in an overall increase in DI (pre 1.2 vs. post 7.2 mm2/mmHg, p<.001). Initial creation of the submucosal tunnel resulted in a 3-fold increase in DI (1.2 vs. 3.6 mm2/mmHg, p<.001). When the myotomy was then performed in a stepwise fashion from proximal to distal, the initial esophageal myotomy component had no effect on DI. Subsequent myotomy extension across the LES complex resulted in an increase in DI, as did the initial gastric myotomy extension (to 2cm distal to the EGJ). The final gastric myotomy extension (to 3cm distal) had no further effect. Conclusions During POEM, creation of the submucosal tunnel prior to myotomy resulted in a marked improvement in EGJ physiology. Myotomy extension across the LES complex and to 2cm onto the gastric wall resulted in normalization of EGJ distensibility, whereas subsequent extension to 3cm distal to the EGJ did not increase compliance further. PMID:26092005

Selective Arterial Embolization of Idiopathic Priapism

DOE Office of Scientific and Technical Information (OSTI.GOV)

Cohen, Gary S.; Braunstein, Larry; Ball, David S.

1996-11-15

We report a case of idiopathic priapism that was only identified as high-flow or arterial priapism after drainage of the corpora cavernosa. Following failure of conservative and surgical treatment attempts, two consecutive embolizations of a unilateral penile artery were performed with gelgoam particles.

[Clinical features of idiopathic restless legs syndrome in Japanese patients].

PubMed

Kume, Akito; Kume, Hideaki

2010-06-01

Little is known about the diagnosis and management of restless legs syndrome (RLS) in Japanese neurology clinics. To validate the diagnostic criteria of the International RLS Study Group (IRLSSG) and the treatment algorithm of the Mayo Clinic in a Japanese neurology clinic setting and to clarify the features of Japanese patients with idiopathic RLS. Patients with RLS symptoms were examined by a neurologist and the assessment included neurological examination, tests for periodic limb movements (PLM) and dopaminergic response, and the clinical diagnosis was made according to IRLSSG diagnostic criteria. Patients diagnosed with idiopathic RLS were treated with dopaminergic agents and the efficacy was evaluated. The study subjects were 151 Japanese patients who presented with RLS symptoms. Idiopathic RLS was diagnosed in 113 patients, secondary RLS in 16 and RLS mimics in 22. The cause of RLS mimics was either myelopathy, radiculopathy or neuropathy in 11 patients. The mean age of patients with idiopathic RLS was 50.1 (SD 20.0) years, 63% were woman, 97% had daily RLS, 31% had family history (40% of the early-onset subgroup), 86% reported unpleasant sensations in the lower legs, 43% had PLM in the daytime suggested immobilization test, 81% suffered from insomnia, 49% had limitations of work and activities, 71% reported impaired mood, 27% had consulted physicians about their symptoms, 4% had been diagnosed with RLS, 73% improved after dopaminergic treatments, and 33% experienced complete remission. The clinical features of Japanese patients with idiopathic RLS were identical to those reported in western countries, which suggests that IRLSSG diagnostic criteria and Mayo Clinic treatment algorism are valid in Japanese neurology clinics. Both patients and physicians were not fully aware of RLS in this country. Neurological examination was important in excluding RLS mimics and making a diagnosis of RLS.

Significance of Myositis Autoantibody in Patients with Idiopathic Interstitial Lung Disease

PubMed Central

Song, Ju Sun; Hwang, Jiwon; Cha, Hoon-Suk; Jeong, Byeong-Ho; Suh, Gee Young; Chung, Man Pyo

2015-01-01

Purpose Some patients with interstitial lung disease (ILD) related to connective tissue disease (CTD) have a delayed diagnosis of the underlying CTD when the ILD is categorized as idiopathic. In this study, we evaluated the frequency of myositis autoantibodies in patients diagnosed with idiopathic ILD and investigated the clinical significance stemming from the presence of the antibodies. Materials and Methods A total 32 patients diagnosed with idiopathic ILD were enrolled in this study. We analyzed a panel of 11 myositis autoantibody specificities in the patients using a line blot immunoassay. Then, we divided them into myositis autoantibody-positive and -negative groups and compared the clinical features and laboratory data between the two groups. Results Of the 32 idiopathic ILD patients, 12 patients had myositis autoantibodies encompassing 9 specificities, except for anti-Mi-2 and anti-PM-Scl 100 (12/32, 38%). Anti-synthetase autoantibodies including Jo-1, EJ, OJ, PL-7, and PL-12 were present in 7 patients (7/32, 22%). The group with myositis autoantibodies presented more frequently with the symptom of mechanic's hand and showed abnormal pulmonary function test results with low forced vital capacity, diffusing capacity for carbon monoxide, total lung capacity, and high lactate dehydrogenase values in blood when compared with the group without myositis antibodies. Conclusion We strongly suggest that patients undergo an evaluation of myositis autoantibodies, if they are diagnosed with idiopathic ILD in the presence of clinical characteristics including mechanic's hand, arthralgia, and autoantibodies which are insufficient to make a diagnosis of a specific CTD category. PMID:25837172

Significance of myositis autoantibody in patients with idiopathic interstitial lung disease.

PubMed

Song, Ju Sun; Hwang, Jiwon; Cha, Hoon-Suk; Jeong, Byeong-Ho; Suh, Gee Young; Chung, Man Pyo; Kang, Eun-Suk

2015-05-01

Some patients with interstitial lung disease (ILD) related to connective tissue disease (CTD) have a delayed diagnosis of the underlying CTD when the ILD is categorized as idiopathic. In this study, we evaluated the frequency of myositis autoantibodies in patients diagnosed with idiopathic ILD and investigated the clinical significance stemming from the presence of the antibodies. A total 32 patients diagnosed with idiopathic ILD were enrolled in this study. We analyzed a panel of 11 myositis autoantibody specificities in the patients using a line blot immunoassay. Then, we divided them into myositis autoantibody-positive and -negative groups and compared the clinical features and laboratory data between the two groups. Of the 32 idiopathic ILD patients, 12 patients had myositis autoantibodies encompassing 9 specificities, except for anti-Mi-2 and anti-PM-Scl 100 (12/32, 38%). Anti-synthetase autoantibodies including Jo-1, EJ, OJ, PL-7, and PL-12 were present in 7 patients (7/32, 22%). The group with myositis autoantibodies presented more frequently with the symptom of mechanic's hand and showed abnormal pulmonary function test results with low forced vital capacity, diffusing capacity for carbon monoxide, total lung capacity, and high lactate dehydrogenase values in blood when compared with the group without myositis antibodies. We strongly suggest that patients undergo an evaluation of myositis autoantibodies, if they are diagnosed with idiopathic ILD in the presence of clinical characteristics including mechanic's hand, arthralgia, and autoantibodies which are insufficient to make a diagnosis of a specific CTD category.

Prognosis of acute idiopathic neck pain is poor: a systematic review and meta-analysis.

PubMed

Hush, Julia M; Lin, C Christine; Michaleff, Zoe A; Verhagen, Arianne; Refshauge, Kathryn M

2011-05-01

To conduct a systematic review and meta-analysis on the prognosis of acute idiopathic neck pain and disability. EMBASE, CINAHL, Medline, AMED, PEDro, and CENTRAL were searched from inception to July 2009, limited to human studies. Reference lists of relevant systematic reviews were searched by hand. Search terms included: neck pain, prognosis, inception, cohort, longitudinal, observational, or prospective study and randomized controlled trial. Eligible studies were longitudinal cohort studies and randomized controlled trials with a no treatment or minimal treatment arm that recruited an inception cohort of acute idiopathic neck pain and reported pain or disability outcomes. Eligibility was determined by 2 authors independently. Seven of 20,085 references were included. Pain and disability data were extracted independently by 2 authors. Risk of bias was assessed independently by 2 authors. Statistical pooling showed a weighted mean pain score (0-100) of 64 (95% confidence interval [CI], 61-67) at onset and 35 (95% CI, 32-38) at 6.5 weeks. At 12 months, neck pain severity remained high at 42 (95% CI, 39-45). Disability reduced from a pooled weighted mean score (0-100) at onset of 30 (95% CI, 28-32) to 17 (95% CI, 15-19) by 6.5 weeks, without further improvement at 12 months. Studies varied in length of follow-up, design, and sample size. This review provides Level I evidence that the prognosis of acute idiopathic neck pain is worse than currently recognized. This evidence can guide primary care clinicians when providing prognostic information to patients. Further research to identify prognostic factors and long-term outcomes from inception cohorts would be valuable. Copyright © 2011 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

Glymphatic MRI in idiopathic normal pressure hydrocephalus

PubMed Central

Ringstad, Geir; Vatnehol, Svein Are Sirirud; Eide, Per Kristian

2017-01-01

Abstract The glymphatic system has in previous studies been shown as fundamental to clearance of waste metabolites from the brain interstitial space, and is proposed to be instrumental in normal ageing and brain pathology such as Alzheimer’s disease and brain trauma. Assessment of glymphatic function using magnetic resonance imaging with intrathecal contrast agent as a cerebrospinal fluid tracer has so far been limited to rodents. We aimed to image cerebrospinal fluid flow characteristics and glymphatic function in humans, and applied the methodology in a prospective study of 15 idiopathic normal pressure hydrocephalus patients (mean age 71.3 ± 8.1 years, three female and 12 male) and eight reference subjects (mean age 41.1 + 13.0 years, six female and two male) with suspected cerebrospinal fluid leakage (seven) and intracranial cyst (one). The imaging protocol included T1-weighted magnetic resonance imaging with equal sequence parameters before and at multiple time points through 24 h after intrathecal injection of the contrast agent gadobutrol at the lumbar level. All study subjects were kept in the supine position between examinations during the first day. Gadobutrol enhancement was measured at all imaging time points from regions of interest placed at predefined locations in brain parenchyma, the subarachnoid and intraventricular space, and inside the sagittal sinus. Parameters demonstrating gadobutrol enhancement and clearance in different locations were compared between idiopathic normal pressure hydrocephalus and reference subjects. A characteristic flow pattern in idiopathic normal hydrocephalus was ventricular reflux of gadobutrol from the subarachnoid space followed by transependymal gadobutrol migration. At the brain surfaces, gadobutrol propagated antegradely along large leptomeningeal arteries in all study subjects, and preceded glymphatic enhancement in adjacent brain tissue, indicating a pivotal role of intracranial pulsations for glymphatic

Glymphatic MRI in idiopathic normal pressure hydrocephalus.

PubMed

Ringstad, Geir; Vatnehol, Svein Are Sirirud; Eide, Per Kristian

2017-10-01

The glymphatic system has in previous studies been shown as fundamental to clearance of waste metabolites from the brain interstitial space, and is proposed to be instrumental in normal ageing and brain pathology such as Alzheimer's disease and brain trauma. Assessment of glymphatic function using magnetic resonance imaging with intrathecal contrast agent as a cerebrospinal fluid tracer has so far been limited to rodents. We aimed to image cerebrospinal fluid flow characteristics and glymphatic function in humans, and applied the methodology in a prospective study of 15 idiopathic normal pressure hydrocephalus patients (mean age 71.3 ± 8.1 years, three female and 12 male) and eight reference subjects (mean age 41.1 + 13.0 years, six female and two male) with suspected cerebrospinal fluid leakage (seven) and intracranial cyst (one). The imaging protocol included T1-weighted magnetic resonance imaging with equal sequence parameters before and at multiple time points through 24 h after intrathecal injection of the contrast agent gadobutrol at the lumbar level. All study subjects were kept in the supine position between examinations during the first day. Gadobutrol enhancement was measured at all imaging time points from regions of interest placed at predefined locations in brain parenchyma, the subarachnoid and intraventricular space, and inside the sagittal sinus. Parameters demonstrating gadobutrol enhancement and clearance in different locations were compared between idiopathic normal pressure hydrocephalus and reference subjects. A characteristic flow pattern in idiopathic normal hydrocephalus was ventricular reflux of gadobutrol from the subarachnoid space followed by transependymal gadobutrol migration. At the brain surfaces, gadobutrol propagated antegradely along large leptomeningeal arteries in all study subjects, and preceded glymphatic enhancement in adjacent brain tissue, indicating a pivotal role of intracranial pulsations for glymphatic function. In

Idiopathic granulomatous mastitis masquerading as carcinoma of the breast: a case report and review of the literature

PubMed Central

Tuli, Richard; O'Hara, Brian J; Hines, Janet; Rosenberg, Anne L

2007-01-01

Background Idiopathic granulomatous mastitis is an uncommon, benign entity with a diagnosis of exclusion. The typical clinical presentation of idiopathic granulomatous mastitis often mimics infection or malignancy. As a result, histopathological confirmation of idiopathic granulomatous mastitis combined with exclusion of infection, malignancy and other causes of granulomatous disease is absolutely necessary. Case Presentation We present a case of a young woman with idiopathic granulomatous mastitis, initially mistaken for mastitis as well as breast carcinoma, and successfully treated with a course of corticosteroids. Conclusion There is no clear clinical consensus regarding the ideal therapeutic management of idiopathic granulomatous mastitis. Treatment options include expectant management with spontaneous remission, corticosteroid therapy, immunosuppressive agents and extensive surgery for refractory cases. PMID:17662130

The coeruleus/subcoeruleus complex in idiopathic rapid eye movement sleep behaviour disorder.

PubMed

Ehrminger, Mickael; Latimier, Alice; Pyatigorskaya, Nadya; Garcia-Lorenzo, Daniel; Leu-Semenescu, Smaranda; Vidailhet, Marie; Lehericy, Stéphane; Arnulf, Isabelle

2016-04-01

Idiopathic rapid eye movement sleep behaviour disorder is characterized by nocturnal violence, increased muscle tone during rapid eye movement sleep and the lack of any other neurological disease. However, idiopathic rapid eye movement sleep behaviour disorder can precede parkinsonism and dementia by several years. Using 3 T magnetic resonance imaging and neuromelanin-sensitive sequences, we previously found that the signal intensity was reduced in the locus coeruleus/subcoeruleus area of patients with Parkinson's disease and rapid eye movement sleep behaviour disorder. Here, we studied the integrity of the locus coeruleus/subcoeruleus complex with neuromelanin-sensitive imaging in 21 patients with idiopathic rapid eye movement sleep behaviour disorder and compared the results with those from 21 age- and gender-matched healthy volunteers. All subjects underwent a clinical examination, motor, cognitive, autonomous, psychological, olfactory and colour vision tests, and rapid eye movement sleep characterization using video-polysomnography and 3 T magnetic resonance imaging. The patients more frequently had preclinical markers of alpha-synucleinopathies, including constipation, olfactory deficits, orthostatic hypotension, and subtle motor impairment. Using neuromelanin-sensitive imaging, reduced signal intensity was identified in the locus coeruleus/subcoeruleus complex of the patients with idiopathic rapid eye movement sleep behaviour. The mean sensitivity of the visual analyses of the signal performed by neuroradiologists who were blind to the clinical diagnoses was 82.5%, and the specificity was 81% for the identification of idiopathic rapid eye movement sleep behaviour. The results confirm that this complex is affected in idiopathic rapid eye movement sleep behaviour (to the same degree as it is affected in Parkinson's disease). Neuromelanin-sensitive imaging provides an early marker of non-dopaminergic alpha-synucleinopathy that can be detected on an individual

Idiopathic gingival fibromatosis rehabilitation: a case report with two-year followup.

PubMed

Jayachandran, Mahesh; Kapoor, Shalini; Mahesh, Rethi

2013-01-01

Gingival enlargements are quite common and may be either inflammatory, noninflammatory, or a combination of both. Gingival hyperplasia is a bizarre condition causing esthetic, functional, psychological, and masticatory disturbances of the oral cavity. Causes of gingival enlargement can be due to plaque accumulation, due to poor oral hygiene, inadequate nutrition, or systemic hormonal stimulation (Bakaeen and Scully, 1998). It can occur as an isolated disease or as part of a syndrome or chromosomal abnormality. A progressive fibrous enlargement of the gingiva is a facet of idiopathic fibrous hyperplasia of the gingiva (Carranza and Hogan, 2002; Gorlin et al., 1976). It is described variously as fibromatosis gingivae, gingivostomatitis, hereditary gingival fibromatosis, idiopathic fibromatosis, familial elephantiasis, and diffuse fibroma. We present a case of idiopathic gingival fibromatosis with its multidisciplinary approach of management.

Per-oral endoscopic myotomy: Major advance in achalasia treatment and in endoscopic surgery

PubMed Central

Friedel, David; Modayil, Rani; Stavropoulos, Stavros N

2014-01-01

Per-oral endoscopic myotomy (POEM) represents a natural orifice endoscopic surgery (NOTES) approach to laparoscopy Heller myotomy (LHM). POEM is arguably the most successful clinical application of NOTES. The growth of POEM from a single center in 2008 to approximately 60 centers worldwide in 2014 with several thousand procedures having been performed attests to the success of POEM. Initial efficacy, safety and acid reflux data suggest at least equivalence of POEM to LHM, the previous gold standard for achalasia therapy. Adjunctive techniques used in the West include impedance planimetry for real-time intraprocedural luminal assessment and endoscopic suturing for challenging mucosal defect closures during POEM. The impact of POEM extends beyond the realm of esophageal motility disorders as it is rapidly popularizing endoscopic submucosal dissection in the West and spawning offshoots that use the submucosal tunnel technique for a host of new indications ranging from resection of tumors to pyloromyotomy for gastroparesis. PMID:25548473

Idiopathic annular dilation: a rare cause of isolated severe tricuspid regurgitation.

PubMed

Girard, S E; Nishimura, R A; Warnes, C A; Dearani, J A; Puga, F J

2000-03-01

The management of patients with severe tricuspid regurgitation (TR) requires the clinician to clarify the mechanism of regurgitation. Primary disorders of the tricuspid valve, either congenital or acquired, may be readily identified by echocardiography. Severe TR most often results from left-sided heart disease and secondary pulmonary hypertension. Cardiomyopathic processes may also cause right ventricular failure and functional TR. We report three patients with severe TR due to idiopathic annular dilation. The tricuspid valves were otherwise normal on surgical inspection, and the pulmonary pressures were not significantly elevated. Each patient was aged over 65 years and had chronic atrial fibrillation with preserved left ventricular systolic function. Surgical treatment was associated with marked clinical improvement. Clinicians should recognize this unusual but treatable cause of right-sided congestive heart failure.

[Biologic therapy in idiopathic inflammatory myopathy].

PubMed

Selva-O'Callaghan, Albert; Ramos Casals, Manel; Grau Junyent, Josep M

2014-09-15

The aim of this article is to study the evidence-based knowledge related to the use of biological therapies in patients diagnosed with idiopathic inflammatory myopathy (dermatomyositis, polymyositis and inclusion body myositis). In this review the leading published studies related to the use of biological therapy in patients with myositis are analysed; mainly those with high methodological standards, that means randomized and controlled studies. Methodological drawbacks due to the rarity and heterogeneity of these complex diseases are also addressed. Up to now is not possible to ascertain the biologics as a recommended therapy in patients with myositis, at least based in the current evidence-based knowledge, although it can not be neglected as a therapeutic option in some clinical situations, taking into account the scarce of effective treatments in those patients, especially in refractory myositis. Future studies probably will help to better define the role of biological therapies in patients with idiopathic inflammatory myopathy. Copyright © 2013 Elsevier España, S.L.U. All rights reserved.

Bilateral versus single lung transplant for idiopathic pulmonary fibrosis.

PubMed

Lehmann, Sven; Uhlemann, Madlen; Leontyev, Sergey; Seeburger, Joerg; Garbade, Jens; Merk, Denis R; Bittner, Hartmuth B; Mohr, Friedrich W

2014-10-01

It is unknown if uni- or bilateral lung transplant is best for treatment of usual idiopathic pulmonary fibrosis. We reviewed our single-center experience comparing both treatments. Between 2002 and 2011, one hundred thirty-eight patients at our institution underwent a lung transplant. Of these, 58 patients presented with idiopathic pulmonary fibrosis (56.9%) and were the focus of this study. Thirty-nine patients received a single lung transplant and 19 patients a bilateral sequential lung transplant. The mean patient age was 54 ± 10 years, and 69% were male. The intraoperative course was uneventful, save for 7 patients who needed extracorporeal membrane oxygenation support. Three patients had respiratory failure before the lung transplant that required mechanical ventilation and was supported by extracorporeal membrane oxygenation. Elevated pulmonary artery pressure > 40 mm Hg was identified as an independent predictor of early mortality by uni- and multivariate analysis (P = .01; OR 9.7). Using a Cox regression analysis, postoperative extracorporeal membrane oxyge-nation therapy (P = .01; OR 10.2) and the need for > 10 red blood cell concentrate during the first 72 hours after lung transplant (P = .01; OR 5.6) were independent predictors of long-term survival. Actuarial survival at 1 and 5 years was 65.6% and 55.3%, with no significant between-group differences (70.6% and 54.3%). Lung transplant is a safe and curative treatment for idiopathic pulmonary fibrosis. According to our results, unilateral lung transplant for idiopathic pulmonary fibrosis is an alternative to bilateral lung transplant and may affect the allocation process.

Update on diagnosis and treatment of idiopathic pulmonary fibrosis

PubMed Central

Baddini-Martinez, José; Baldi, Bruno Guedes; da Costa, Cláudia Henrique; Jezler, Sérgio; Lima, Mariana Silva; Rufino, Rogério

2015-01-01

Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation. PMID:26578138

Pattern of asymmetry of paraspinal muscle size in adolescent idiopathic scoliosis examined by real-time ultrasound imaging. A preliminary study.

PubMed

Kennelly, K P; Stokes, M J

1993-06-01

The symmetry of lumbar multifidus size was examined in 20 patients with adolescent idiopathic scoliosis, aged 12-19 years. With the subject prone, bilateral real-time ultrasound images were obtained at the level of the 4th lumbar vertebra. Cross-sectional area and linear (horizontal and vertical) measurements were made using on-screen calipers. A pattern of asymmetry of lumbar multifidus cross-sectional area was shown to exist for the different curve types. The cross-sectional area was smaller (P < 0.0001) on the opposite side to the convexity of a primary thoracic curve, and on the convex side of a lumbar or thoracolumbar curve. The combined linear measurements (multiplied) correlated with cross-sectional area (r = 0.95) and could therefore be used for rapid clinical assessment of multifidus size. These preliminary findings provide a basis for further investigation of the role of the musculature in the pathogenesis of adolescent idiopathic scoliosis.

Sensitivity Analyses of the Change in FVC in a Phase 3 Trial of Pirfenidone for Idiopathic Pulmonary Fibrosis

PubMed Central

Bradford, Williamson Z.; Fagan, Elizabeth A.; Glaspole, Ian; Glassberg, Marilyn K.; Glasscock, Kenneth F.; King, Talmadge E.; Lancaster, Lisa H.; Nathan, Steven D.; Pereira, Carlos A.; Sahn, Steven A.; Swigris, Jeffrey J.; Noble, Paul W.

2015-01-01

BACKGROUND: FVC outcomes in clinical trials on idiopathic pulmonary fibrosis (IPF) can be substantially influenced by the analytic methodology and the handling of missing data. We conducted a series of sensitivity analyses to assess the robustness of the statistical finding and the stability of the estimate of the magnitude of treatment effect on the primary end point of FVC change in a phase 3 trial evaluating pirfenidone in adults with IPF. METHODS: Source data included all 555 study participants randomized to treatment with pirfenidone or placebo in the Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis (ASCEND) study. Sensitivity analyses were conducted to assess whether alternative statistical tests and methods for handling missing data influenced the observed magnitude of treatment effect on the primary end point of change from baseline to week 52 in FVC. RESULTS: The distribution of FVC change at week 52 was systematically different between the two treatment groups and favored pirfenidone in each analysis. The method used to impute missing data due to death had a marked effect on the magnitude of change in FVC in both treatment groups; however, the magnitude of treatment benefit was generally consistent on a relative basis, with an approximate 50% reduction in FVC decline observed in the pirfenidone group in each analysis. CONCLUSIONS: Our results confirm the robustness of the statistical finding on the primary end point of change in FVC in the ASCEND trial and corroborate the estimated magnitude of the pirfenidone treatment effect in patients with IPF. TRIAL REGISTRY: ClinicalTrials.gov; No.: NCT01366209; URL: www.clinicaltrials.gov PMID:25856121

Idiopathic granulomatous mastitis: case report and review of the literature.

PubMed

Imoto, S; Kitaya, T; Kodama, T; Hasebe, T; Mukai, K

1997-08-01

We report a case of idiopathic granulomatous mastitis in a 35-year-old Japanese woman, who came to our hospital complaining of a tender mass in her right breast. Because the results of initial aspiration cytology were considered highly suspicious for carcinoma, modified radical mastectomy was performed. However, the final histological diagnosis was granulomatous lobular mastitis with no evidence of malignancy. Idiopathic granulomatous mastitis is a rare inflammatory breast disease of unknown etiology. Since the clinical manifestations are similar to those of mammary carcinoma, this condition has been misdiagnosed as carcinoma and treated as such. A review of the literature revealed that idiopathic granulomatous mastitis has tended to occur in young patients with a history of childbirth or oral contraceptive usage. Clinical or imaging diagnosis has often been difficult. Complete resection or corticosteroid therapy can be recommended as the optimal treatment. Since 38% of patients experience recurrence, long-term follow-up is indicated.

Idiopathic Chronic Parotitis: Imaging Findings and Sialendoscopic Response.

PubMed

Heineman, Thomas E; Kacker, Ashutosh; Kutler, David I

2015-01-01

The purpose of this study was to correlate imaging and sialendoscopic findings to therapeutic response in patients with idiopathic chronic parotitis. We retrospectively reviewed 122 consecutive sialendoscopies performed in an academic medical center by two surgeons between 2008 and 2013. Forty-one (34%) and 54 (44%) patients were excluded on the basis of having parotid or submandibular sialolith, respectively. Nineteen cases were included in the study with idiopathic chronic parotitis. There was a median follow-up of 5 months. Computed tomography (CT) imaging had a sensitivity and specificity of 80.0 and 71.4%, respectively, for predicting abnormal findings on sialendoscopy, while magnetic resonance imaging (MRI) had 100% accuracy in a small set of cases. In glands with noticeable pathology present on preoperative imaging or sialendoscopy, 11 out of 12 glands (92%) treated experienced symptomatic improvement, while 3 out of 7 glands (43%) without pathology on imaging or endoscopy experienced symptomatic improvement (p = 0.038). Sialendoscopy for the treatment of idiopathic chronic parotid disease can improve pain and swelling with a higher frequency of success in patients with abnormalities noted on endoscopy. CT and MRI have a moderate degree of accuracy in predicting which patients will benefit from therapeutic sialendoscopy. © 2015 S. Karger AG, Basel.

Functional single nucleotide polymorphisms of matrix metalloproteinase 7 and 12 genes in idiopathic recurrent spontaneous abortion.

PubMed