Shieh, Hester F; Jennings, Russell W
The management of long-gap esophageal atresia remains challenging with limited consensus on the definition, evaluation, and surgical approach to treatment. Efforts to preserve the native esophagus have been successful with delayed primary anastomosis and tension-based esophageal growth induction processes. Esophageal replacement is necessary in a minority of cases, with the conduit of choice and patient outcomes largely dependent on institutional expertise. Given the complexity of this patient population with significant morbidity, treatment and long-term follow-up are best done in multidisciplinary esophageal and airway treatment centers. Copyright © 2017 Elsevier Inc. All rights reserved.
Khan, Khalid M; Krosch, Tara C; Eickhoff, Jeffrey C; Sabati, Arash A; Brudney, James; Rivard, Andrew L; Foker, John E
To determine the pattern of feeding milestones following primary repair of long-gap esophageal atresia (EA). A questionnaire based upon well established feeding milestones was used. Children after long-gap EA repair, n=40, were compared from after primary repair to healthy children from birth, n=102. The age when surveyed of the EA group and controls was different: 6.2+/-4.7 (mean+/-standard deviation) years, range 1.1-20.9, versus 2.5+/-2.4 years, range 0.0-12.1, p=0.00. The esophageal gap length in the EA group was 5.1+/-1.2 cm and age at repair was 5.5+/-5.0 months. There was no statistically significant difference between the atresia group and controls for feeding milestones; Self feeding finger foods approached significance. There was, however, greater variability in the timing of milestones in the atresia group compared to controls. Feeding milestones were negatively correlated with age at primary repair: drinking with a covered sippy cup, rho=-0.51, p=0.01 and self feeding finger foods, rho=-0.36, p=0.04 were statistically significant. Drinking from a cup correlated with gestational age, rho=0.38, p=0.04, and negatively correlated to esophageal gap length, rho=-0.45, p=0.01. Despite delayed onset of feeding, major milestones after EA repair occurred in similar pattern to normal infants. An early referral for primary repair is beneficial for earlier acquisition of milestones for infants with long-gap EA.
Durell, Jonathan; Dagash, Haitham; Eradi, Bala; Rajimwale, Ashok; Nour, Shawqui; Patwardhan, Nitin
The presence of 13 pairs of ribs on pre-operative chest x-ray has been postulated to be an indicator for long gap esophageal atresia (EA). This study sought to determine the validity of this theory and identify associated pathological conditions in patients with EA and abnormal rib count. Babies with EA from January 2005 - December 2012 were retrospectively analyzed. Information was gathered from neonatal health records and operation notes. Chest x-rays were reviewed to determine rib count. Long gap EA was defined as failure to achieve primary esophageal anastomosis. Statistical analysis performed with Fisher's exact test. Seventy-six patients were identified. Eight patients had long gap EA, with none of these patients having 13 pairs of ribs. Paradoxically, 10 patients with esophageal atresia +/- trachea-esophageal atresia (EA +/- TEF) and supernumerary ribs underwent primary repair. Nine patients had 11 pairs of ribs, of which 2 had pure EA and a long gap. Using Fisher's exact test to compare the groups of supernumary ribs and non-supernumary ribs there is a p value of 0.587. VACTERL association was identified in 40% of those with supernumerary ribs. Various associated syndromes and concomitant abnormalities were identified. We found no association between 13 pairs of ribs and long gap in esophageal atresia. Those with 13 pairs of ribs were more likely to have associated anomalies, although this was not statistically significant. Our cohort of patients was found to have a range of pathology related to genetic syndromes, further atresias, and malformations, which is well known to be associated with children born with EA +/- TEF. Prognosis study - level IV. Copyright © 2017 Elsevier Inc. All rights reserved.
Lee, Esmond; Milan, Anna; Urbani, Luca; De Coppi, Paolo; Lowdell, Mark W
Esophageal atresia refers to an anomaly in foetal development in which the esophagus terminates in a blind end. Whilst surgical correction is achievable in most patients, when a long gap is present it still represents a major challenge associated with higher morbidity and mortality. In this context, tissue engineering could represent a successful alternative to restore oesophageal function and structure. Naturally derived biomaterials made of decellularized tissues retain native extracellular matrix architecture and composition, providing a suitable bed for the anchorage and growth of relevant cell types. Areas covered: This review outlines the various strategies and challenges in esophageal tissue engineering, highlighting the evolution of ideas in the development of decellularized scaffolds for clinical use. It explores the interplay between clinical needs, ethical dilemmas, and manufacturing challenges in the development of a tissue engineered decellularized scaffold for oesophageal atresia. Expert opinion: Current progress on oesophageal tissue engineering has enabled effective repair of patch defects, whilst the development of a full circumferential construct remains a challenge. Despite the different approaches available and the improvements achieved, a gold standard for fully functional tissue engineered oesophageal constructs has not been defined yet.
van der Zee, David C.; Bagolan, Pietro; Faure, Christophe; Gottrand, Frederic; Jennings, Russell; Laberge, Jean-Martin; Martinez Ferro, Marcela Hernan; Parmentier, Benoît; Sfeir, Rony; Teague, Warwick
INoEA is the International Network of Esophageal Atresia and consists of a broad spectrum of pediatric specialties and patient societies. The working group on long-gap esophageal atresia (LGEA) set out to develop guidelines regarding the definition of LGEA, the best diagnostic and treatment strategies, and highlight the necessity of experience and communication in the management of these challenging patients. Review of the literature and expert discussion concluded that LGEA should be defined as any esophageal atresia (EA) that has no intra-abdominal air, realizing that this defines EA with no distal tracheoesophageal fistula (TEF). LGEA is considerably more complex than EA with distal TEFs and should be referred to a center of expertise. The first choice is to preserve the native esophagus and pursue primary repair, delayed primary anastomosis, or traction/growth techniques to achieve anastomosis. A cervical esophagostomy should be avoided if possible. Only if primary anastomosis is not possible, replacement techniques should be used. Jejunal interposition is proposed as the best option among the major EA centers. In light of the infrequent occurrence of LGEA and the technically demanding techniques involved to achieve esophageal continuity, it is strongly advised to develop regional or national centers of expertise for the management and follow-up of these very complex patients. PMID:28409148
Fernández, M S; Gutiérrez, C; Ibáñez, V; Lluna, J; Barrios, J E; Vila, J J; García-Sala, C
For distances of over 4-5 cm, esophageal replacement is almost always necessary in esophageal atresias. We present the technical details and describe our experience with esophageal reconstruction by elongation of the lesser curvature (Schärli's technique) in four cases of very long atresias. A retrosternal transposition was made without a thoracotomy in two children, and an orthotopic mediastinal route through a right thoracotomy was done in two others. There were two main complications: anastomotic leaks in three patients that closed spontaneously, and too-rapid gastric emptying, resulting in dumping symptoms that improved with time and diet. One patient developed an anastomotic stricture that responded to bouginage, while another had temporary feeding problems. Esophageal reconstruction by elongation of the lesser curvature provides a relatively simple method of esophageal replacement in children in that all portions of the esophagus are preserved. We propose this technique for early establishment of esophageal continuity in neonates.
Esophageal atresia (EA) is a congenital defect. This means it occurs before birth. There are several types. In most cases, the ... the lower esophagus and stomach. Most infants with EA have another defect called tracheoesophageal fistula (TEF). This ...
Bairdain, Sigrid; Dodson, Brenda; Zurakowski, David; Waisel, David B; Jennings, Russell W; Boretsky, Karen R
Infants with long-gap esophageal atresia (LGEA) undergo repeated thoracotomies for staged surgical repair known as the Foker process (FP). Associated prolonged mechanical ventilation results in exposure to high doses of opioids and benzodiazepines, and prolonged weaning times and ICU stays. The aim of this study was to determine the effectiveness of short-term paravertebral nerve block (PVNB) catheters in reducing opioid/benzodiazepine exposure and effects on clinical variables. The medical records of seventeen infants were retrospectively reviewed; 11 with PVNB and six without (CG). PVNB were placed using ultrasound-guidance and chloroprocaine infusions implemented in the ICU. Opioids and benzodiazepines were administered via the protocol for 5 days following thoracotomies for Foker-I and Foker-II. Foker-I: Average reduction in morphine and midazolam consumption was 36% (2.18 vs 3.40 mg·kg(-1) ·day(-1) ; P < 0.001) and 31% (2.25 vs 3.25 mg·kg(-1) ·day(-1) ; P = 0.033), respectively, in the PVNB compared with CG. Foker-II: Average reduction in morphine and midazolam consumption was 39% (3.19 vs 5.27 mg·kg(-1) ·day(-1) ) and 38% (3.46 mg·kg(-1) ·day(-1) vs 5.62; P < 0.001), respectively in the PVNB compared with CG. 24-h prior to extubation: Average reduction in morphine and midazolam consumption was 50% (2.91 vs 5.85 mg·kg(-1) ·24 h(-1) ; p = 0.023) and 61% (2.27 vs 5.83 mg·kg(-1) ·24 h(-1) ; P = 0.004), respectively, in the PVNB compared with CG. Infusion wean time, (independence from opioid/midazolam infusions) following extubation was 5 days in the PVNB group and 15 days in CG (P = 0.005). Median ICU stay (IQR) was 40 days (34-45 days) in PVNB patients and 71 days (42-106 days) in controls (P = 0.02). PVNB catheters were left an average of 7 days and there were no complications associated with the nerve blocks. Short-term PVNB placement decreases opioid and benzodiazepine exposure, weaning days and ICU stay in infants
Ruiz de Temiño, M; Esteban, J A; Elías, J; González, N; Gracia, J; Romeo, M; Escartín, R; Burgués, P; Sainz, A; Pueyo, C
Treatment of esophageal atresia with "long gap" remains difficult and controversial. According to the idea that esophageal anastomosis is imposible in most cases, several esophageal substitution methods have been proposed, as esophagocoloplasty, gastric transposition or reversed gastric tube. Nevertheless reconstruction of native esophagus is accepted as the best option if posible. "Long gap" definition is imprecise, expressed by variability in percent of these cases in total esophageal atresias reported in different series in literature. We report our experience in seven cases type I esophageal atresia with long gap and the different therapeutic options used, with attention to delayed or early esophageal anastomosis feasibility and outcome. We have treated 121 patients with esophageal atresia from whom we analized 7 cases with pure esophageal atresia with "long gap" (5.8%). Six patients underwent gastrostomy and two gastrostomy and esophagostomy. Five patient underwent primary repair with esophageal anastomosis, delayed between 14 days and 4 months in 4 cases. One patient underwent esophageal anastomosis in the first day without gastrostomy. Retroesternal esophagocoloplasty was performed in 2 patients about their first year of life. Esophagogram was done in first month after surgery and pH monitoring of gastroesophageal reflux. Follow-up ranged from 6 months to 28 years. Esophageal anastomosis was feasible in all 5 patients in whom it was tried. Stricture occurred in two patients, one patient underwent anastomotic resection and new esophageal anastomosis. Esophageal reflux was present in two patients, one of them required funduplication. One patient was dead by complications of cardiac malformation. Remaining patients have normal swallowing and are in normal growth curves. Patients with esophagocoloplasty had not relevant early or late complications. In most pure esophageal atresia, delayed or even early esophageal anastomosis is feasible, making use of surgical
Suzuki, Makoto; Kuwano, Hiroyuki
In this report, we describe the esophageal atresia in terms of current surgical management on the basis of our experience and literatures. Traditionally, infants with esophageal atresia have presented shortly after birth because of an inability to pass an orogastric tube, respiratory distress, or an inability to tolerate feeding. And also, an isolated trachea-esophageal fistula (TEF) usually cases coughing, recurrent pneumonia, or choking during feedings. To ignore these symptoms is to risk a delayed diagnosis. The condition may be associated with other major congenital anomalies such as those seen in the vertebral, anal, cardiac, tracheo-esophageal, renal/radial (VACTER) association, or it may be an isolated defect. Therapeutic strategies for esophageal atresia are a prevention of pulmonary complication by TEF closing and an early establishment of enteral alimentation. We promptly repair healthy infants without performing a gastrostomy and delay repair in infants with high-risk factors such as associated severe cardiac anomaly and respiratory insufficiency. Esophageal atresia has been classically approached through a thoracotomy. The disadvantages of such a thoracotomy have been recognized for a long time, for example winged scapula, elevation of fixation of shoulder, asymmetry of the chest wall, rib fusion, scoliosis, and breast and pectoral muscle maldevelopment. To avoid such disadvantages, thoracoscopic repair was recently reported.
van der Zee, David C; Tytgat, Stefaan H A; van Herwaarden, Maud Y A
Management of esophageal atresia has merged from correction of the anomaly to the complete spectrum of management of esophageal atresia and all its sequelae. It is the purpose of this article to give an overview of all aspects involved in taking care of patients with esophageal atresia between January 2011 and June 2016, as well as the patients who were referred from other centers. Esophageal atresia is a complex anomaly that has many aspects that have to be dealt with and complications to be solved. By centralizing these patients in centers of expertise it is believed that the best care can be given. Copyright © 2017 Elsevier Inc. All rights reserved.
Harne, Swapnil; Pathak, Manish; Rattan, Kamal Nayan
Esophageal atresia with tracheoesophageal fistula (EA/TEF) associated with distal congenital esophageal stenosis (CES) is a well-known entity. We encountered three patients of EA/TEF associated with long and unusual CES. PMID:28083495
Pinheiro, Paulo Fernando Martins; Simões e Silva, Ana Cristina; Pereira, Regina Maria
Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care, neonatal anesthesia, ventilatory and nutritional support, antibiotics, early surgical intervention, surgical materials and techniques. Indeed, mortality is currently limited to those cases with coexisting severe life-threatening anomalies. The diagnosis of EA is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed. The primary surgical correction for EA and TEF is the best option in the absence of severe malformations. There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap EA is still controversial. The primary complications during the postoperative period are leak and stenosis of the anastomosis, gastro-esophageal reflux, esophageal dysmotility, fistula recurrence, respiratory disorders and deformities of the thoracic wall. Data regarding long-term outcomes and follow-ups are limited for patients following EA/TEF repair. The determination of the risk factors for the complicated evolution following EA/TEF repair may positively impact long-term prognoses. Much remains to be studied regarding this condition. This manuscript provides a literature review of the current knowledge regarding EA. PMID:22851858
Charlesworth, Paul; Mahomed, Anies
Long gap oesophageal atresia is a clinically and technically challenging condition to manage. Documentation of the gap between the upper and lower pouches is critical to deciding the timing and feasibility of a primary anastamosis. Integral to this process is the role of radiology. We present a case of long gap oesophageal atresia accompanied by chronological radiography demonstrating its' staged management and highlighting some common complications. PMID:22470672
Dhaliwal, J; Tobias, V; Sugo, E; Varjavandi, V; Lemberg, D; Day, A; Bohane, T; Ledder, O; Jiwane, A; Adams, S; Henry, G; Dilley, A; Shi, E; Krishnan, U
Eosinophilic esophagitis (EoE) has only rarely been reported in esophageal atresia (EA) patients. A retrospective case analysis of all EA patients born at our center between January 1999 and April 2012 was performed. A total of 113 of patients were identified; 10 patients were excluded as a result of inadequate data. Eighteen patients (17%) were diagnosed with EoE. The average number of eosinophilis was 30/high-power field (HPF) (19/HPF-80/HPF). The median age for diagnosis of EoE was 1 year and 6 months (8 months-8 years and 7 months). Children with EoE had a significantly greater incidence of reflux symptoms, dysphagia, tracheomalacia, and 'hypoxic spells' (P < 0.05). EoE patients also underwent significantly more surgery including fundoplication and aortopexy when compared with those without EoE (P < 0.0001). Although the incidence of gastrostomy was greater in the EoE group (33% vs. 13%), this was not statistically significant. Half of the EoE patients had a coexisting atopic condition at time of diagnosis. The commonest condition was asthma 7/18 (38%) followed by specific food allergy 6/18 (33%). EoE was treated in 11 patients with either swallowed fluticasone or budesonide slurry. All improved clinically. Histologically, five had complete resolution and six had partial improvement. Six children with EoE were treated with acid suppression alone. All improved clinically, and 5/6 had subsequent histological resolution. One child who received acid suppression and an exclusion diet also improved. Seven patients (38%) had an esophageal stricture at time of EoE diagnosis. Five were dilated at time of the initial endoscopy, prior to the diagnosis of EoE being available. Two patients had resolution of their strictures on medical treatment of their EoE alone and did not require further dilatation. EoE was seen in 17% of children with EA in this study. EoE should be considered in EA patients with persistent symptoms on standard reflux treatment, increasing
Sanal, Murat; Haeussler, Beatrice; Tabarelli, Walther; Maurer, Kathrin; Sergi, Consolato; Hager, Josef
Isolated esophageal atresia is characterized by a long segment between the 2 esophageal pouches. This article presents a case of pure esophageal atresia with a 1-cm-long segment at the midportion without discontinuity that resembled the subtype II3 according to the Kluth atlas. Resection of the atretic segment and primary anastomosis were performed successfully.
Ortiz, Ruben; Galán, Alba Sánchez; Martinez, Leopoldo; Dominguez, Eva; Hernández, Francisco; Santamaria, Manuel Lopez; Tovar, Juan Antonio
The ideal repair of esophageal atresia (EA) is primary anastomosis with closure of the fistula if present. Long gap or local circumstances prompt other procedures that occasionally lead to disastrous complications. The aim of this study was to analyze the management of these complications in a tertiary referral center. A retrospective review of patients treated for EA between 1993 and 2013 was conducted. Both the patients were primarily treated by us, and referrals from elsewhere after two or more failed operations were included. In total, 23 patients were included (3/176 cases of EA treated primarily by us and 20 referrals). Of the 23 patients, 6 had type I EA, 15 type III (four long gaps), 1 type IV, and 1 type V. Cardiac anomalies were associated in seven cases, duodenal atresia in three, and Down syndrome in two patients. Primary anastomosis was initially achieved in 12 patients. Primary or secondary Foker lengthening was used in seven cases. The causes of the failure were anastomotic leaks in nine, unmanageable strictures in seven, and refistulization in five patients. These patients required 66 reoperations (median of 3 [2-7]) before inclusion in the study. Radical tertiary treatment consisted of 15 esophageal replacements (11 colonic grafts and 4 gastric pull-ups), and 1 esophageal-gastric disconnection. Five patients previously treated with esophageal replacement and referred for graft problems required 13 interventions. Two families did not give consent for one replacement and one disconnection. Complications appeared in 12 patients, and 9 additional operations were required in 7 patients. With a follow-up of 31 months (range, 4-139 months) 15 patients take all their meals per os, 5 occasionally use the gastrostomy, and 2 and 1 are fed exclusively via gastrostomy or jejunostomy. All tracheoesophageal fistulas were closed, but 15 cases are below p3 for weight and 12 for height. Three patients (13%) ultimately died 32 months (range, 9-56 months) after the
Lee, Hui Qing; Hawley, Alisa; Doak, Joe; Nightingale, Michael G; Hutson, John M
Long-gap oesophageal atresia (LGOA) causes significant early and long-term morbidity. We conducted a retrospective 25-year review comparing outcomes of delayed primary anastomosis versus oesophageal replacement with greater curvature gastric tube. Records of 44 consecutive patients undergoing LGOA repair (1986-2010) were obtained from OA database with ethics approval and were analysed for complication and long-term outcomes. Analysis was conducted using Student's t-test for quantitative and Fisher exact test for qualitative data. Thirty (68%) patients underwent delayed primary anastomosis and 14 (32%) had oesophageal replacement. Oesophageal replacement patients had longer gaps (mean 5.5 vertebrae, range 4-9) compared to delayed primary anastomosis (mean 3.9, range 2-6) (p=0.004), but no difference in perioperative complications (p=0.2) (Table 1). Oesophageal replacement had more long-term complications (86%) compared to delayed primary anastomosis (30%) (p=0.005). Almost all patients (>90%) experienced gastro-oesophageal reflux and 21 delayed primary anastomosis patients (70%) underwent fundoplication. 60% of delayed primary anastomosis and 64.3% of oesophageal replacement patients had continued gastrointestinal symptoms years after repair. Our experience indicates that LGOA can be repaired safely using both methods, with no deaths and similar perioperative risk, but high long-term morbidity mandates long-term follow-up of these patients. Delayed primary anastomosis has a better long-term outcome compared to oesophageal replacement with gastric tube. Copyright © 2014 Elsevier Inc. All rights reserved.
Joshi, Shirin S; Dhaded, Sangappa M
Esophageal atresia is the commonest cause of obstruction to esophageal lumen in neonates. Foreign bodies in newborns are extremely rare. We report a rare case of esophageal obstruction closely mimicking atresia due to foreign bodies inserted in a female neonate with homicidal intension. PMID:26023467
Michaud, Laurent; Sfeir, Rony; Couttenier, Frédéric; Turck, Dominique; Gottrand, Frédéric
In childhood, the surgical treatment of gastroesophageal reflux is the main cause of dumping syndrome. We report the cases of 2 children with esophageal atresia who presented with dumping syndrome without any precipitating known factors, such as gastroesophageal reflux surgery or associated microgastria. Our data suggest (1) that dumping syndrome can occur after primary anastomosis of esophageal atresia without antireflux surgery and (2) that dumping syndrome should be considered in every child treated surgically for esophageal atresia presenting with digestive symptoms, malaise, failure to thrive, or refusal to eat.
Kassabian, Sirvart; Baez-Socorro, Virginia; Sferra, Thomas; Garcia, Reinaldo
Esophageal atresia (EA) is defined as a discontinuity of the lumen of the esophagus repaired soon after birth. Dysphagia is a common symptom in these patients, usually related to stricture, dysmotility or peptic esophagitis. We present 4 cases of patients with EA who complained of dysphagia and the diagnosis of Eosinophilic esophagitis (EoE) was made, ages ranging from 9 to 16 years. Although our patients were on acid suppression years after their EA repair, they presented with acute worsening of dysphagia. Esophogastroduodenoscopy and/or barium swallow did not show stricture and biopsies revealed elevated eosinophil counts consistent with EoE. Two of 4 patients improved symptomatically with the topical steroids. It is important to note that all our patients have asthma and 3 out of 4 have tested positive for food allergies. One of our patients developed recurrent anastomotic strictures that improved with the treatment of the EoE. A previous case report linked the recurrence of esophageal strictures in patients with EA repair with EoE. Once the EoE was treated the strictures resolved. On the other hand, based on our observation, EoE could be present in patients without recurrent anastomotic strictures. There appears to be a spectrum in the disease process. We are suggesting that EoE is a frequent concomitant problem in patients with history of congenital esophageal deformities, and for this reason any of these patients with refractory reflux symptoms or dysphagia (with or without anastomotic stricture) may benefit from an endoscopic evaluation with biopsies to rule out EoE. PMID:25548504
Kassabian, Sirvart; Baez-Socorro, Virginia; Sferra, Thomas; Garcia, Reinaldo
Esophageal atresia (EA) is defined as a discontinuity of the lumen of the esophagus repaired soon after birth. Dysphagia is a common symptom in these patients, usually related to stricture, dysmotility or peptic esophagitis. We present 4 cases of patients with EA who complained of dysphagia and the diagnosis of Eosinophilic esophagitis (EoE) was made, ages ranging from 9 to 16 years. Although our patients were on acid suppression years after their EA repair, they presented with acute worsening of dysphagia. Esophogastroduodenoscopy and/or barium swallow did not show stricture and biopsies revealed elevated eosinophil counts consistent with EoE. Two of 4 patients improved symptomatically with the topical steroids. It is important to note that all our patients have asthma and 3 out of 4 have tested positive for food allergies. One of our patients developed recurrent anastomotic strictures that improved with the treatment of the EoE. A previous case report linked the recurrence of esophageal strictures in patients with EA repair with EoE. Once the EoE was treated the strictures resolved. On the other hand, based on our observation, EoE could be present in patients without recurrent anastomotic strictures. There appears to be a spectrum in the disease process. We are suggesting that EoE is a frequent concomitant problem in patients with history of congenital esophageal deformities, and for this reason any of these patients with refractory reflux symptoms or dysphagia (with or without anastomotic stricture) may benefit from an endoscopic evaluation with biopsies to rule out EoE.
Rayyan, Maissa; Allegaert, Karel; Omari, Taher; Rommel, Nathalie
Dysphagia or swallowing disorder is very common (range, 15-52%) in patients with esophageal atresia. Children present with a wide range of symptoms. The most common diagnostic tools to evaluate esophageal dysphagia, such as upper barium study and manometry, aim to characterize anatomy and function of the esophageal body and the esophagogastric junction (EGJ). Using these technologies, a variety of pathological motor patterns have been identified in children with esophageal atresia. However, the most challenging part of diagnosing patients with esophageal dysphagia lies in the fact that these methods fail to link functional symptoms such as dysphagia with the esophageal motor disorders observed. A recent method, called pressure-flow analysis (PFA), uses simultaneously acquired impedance and manometry measurements, and applies an integrated analysis of these recordings to derive quantitative pressure-flow metrics. These pressure-flow metrics allow detection of the interplay between bolus flow, motor patterns, and symptomatology by combining data on bolus transit and bolus flow resistance. Based on a dichotomous categorization, flow resistance at the EGJ and ineffective esophageal bolus transit can be determined. This method has the potential to guide therapeutic decisions for esophageal dysmotility in pediatric patients with esophageal atresia.
Gorter, Ramon R; Heij, Hugo A; van der Voorn, J Patrick; Kneepkens, C M Frank
Eosinophilic esophagitis (EoE) is a relatively new condition resulting in dysphagia or symptoms resembling gastroesophageal reflux disease, symptoms that also are common in patients with a history of esophageal atresia. We present 2 patients with persistent dysphagia after repair of esophageal atresia that was caused by EoE. Although the exact etiology and pathogenesis of EoE remain unclear, it is now generally accepted that it is the result of a T-helper cell 2-type immune response with a crucial role for the eosinophil-specific chemotaxis factor eotaxin 3 and eosinophils. Because there are genetic similarities between esophageal atresia and EoE, we speculate that patients with esophageal atresia are at increased risk for developing EoE.
Spaggiari, Emmanuel; Faure, Giuliana; Rousseau, Veronique; Sonigo, Pascale; Millischer-Bellaiche, Anne-Elodie; Kermorvant-Duchemin, Elsa; Muller, Francoise; Czerkiewicz, Isabelle; Ville, Yves; Salomon, Laurent J
The aim of this study was to evaluate the performance of prenatal diagnosis of esophageal atresia (EA) and its associated abnormalities. We conducted a retrospective study from a pediatric database of EA managed postnatally in a single center. Prenatal data included ultrasound and magnetic resonance imaging parameters including amniotic fluid (AF) volume, stomach visualization, AF biochemistry, and associated malformations. Postnatal data included type of EA, mortality, and postnatal diagnosis of associated malformations. One hundred twenty-two cases were included. The diagnosis was suspected prenatally in 39/122 (32%) cases. Polyhydramnios was noted in 64/122 (52.4%), and the stomach was not visualized or small in 39 (32%). There was 14 (11.5%), 2 (1.6%), 101 (82.8%), 5 (4.1%), and 0 (0%) types I, II, III, IV, and V, respectively. EA was suspected prenatally in 12/14 (85.7%) in type I and in 27/108 (25%) in cases with tracheoesophageal fistula (II + III + IV + V). Magnetic resonance imaging was performed in 28 cases, which confirmed EA in 19/28 (sensitivity 67.8%). AF biochemistry was performed in 17 cases, which confirmed EA in 15/17 (sensitivity 88.2%) cases. Of the 69 syndromic associations, 41/69 (59.4%) cases were detected prenatally. Associated malformation was a strong predictor of postnatal death [19/69 vs 3/53, odds ratio 6.33 (1.76; 22.75), p < 0.01]. Prenatal diagnosis of EA remains challenging. MRI and AF biochemistry may prove useful in the diagnosis of EA. Prenatal ultrasound and MRI examination should also focus on associated anomalies. © 2015 John Wiley & Sons, Ltd. © 2015 John Wiley & Sons, Ltd.
Cozzi, Denis A; Mele, Ermelinda; Totonelli, Giorgia; Ceccanti, Silvia; Frediani, Simone; Cozzi, Francesco
Of 136 infants with repaired esophageal atresia, one presented an unilateral facial flushing and 2 presented a flushing and sweating of one half of the body. The topography of these disorders and/or the associated clinical manifestations suggest that the asymmetry may be related to an instability of unilateral autonomic centers more than to a surgical injury of upper thoracic sympathetic chain during esophageal repair.
Macchini, Francesco; Parente, Giovanni; Morandi, Anna; Farris, Giorgio; Gentilino, Valerio; Leva, Ernesto
Introduction The aim of this study was to stratify anastomotic strictures (AS) following esophageal atresia (EA) repair and to establish predictors for the need of dilations. Material and Methods A retrospective study on children operated on for EA between 2004 and 2014 was conducted. The stricture index (SI) was measured both radiologically (SIXR) and endoscopically (SIEND). A correlation analysis between the SI and the number of dilations was performed using Spearman's test and linear regression analysis. Results In this study, 40 patients were included: 35 (87.5%) presented with Gross's type C EA, 3 (7.5%) type A, 1 (2.5%) type B, and 1 (2.5%) type D. The mean follow-up time was 101 ± 71.1 months (range: 7.8-232.5, median: 97.6). The mean SIXR was 0.56 ± 0.16 (range: 0.15-0.86). The mean SIEND was 0.45 ± 0.22 (range: 0.15-0.85). Twenty-four patients (60%) underwent a mean of 2 endoscopic dilations (range: 1-9). The number of dilations was poorly correlated with SIXR, while significantly correlated with SIEND. Patients who did not need dilations had a SIEND < 0.33, patients who needed only one dilation had 0.33 ≤ SIEND < 0.44, and those with SIEND ≥ 0.44 needed two or more dilations. No significant association with other clinical variables was found. All patients were asymptomatic at the time of the first endoscopy. Conclusion SIEND is a useful tool to classify AS and can represent a predictor of the need for endoscopic dilation. The role of the SIEND becomes even more important as clinical characteristics have a low predictive value for the development of an AS and the need for subsequent endoscopic esophageal dilatations.
Glenn, Ian C; Bruns, Nicholas E; Schomisch, Steve J; Ponsky, Todd A
We previously developed a porcine model of long gap pure esophageal atresia (EA) to aid in the creation of novel devices and techniques for treatment of EA. Shortcomings of the model included difficulty decompressing the proximal esophageal pouch (leading to recurrent aspiration) and a requirement for gastrostomy tube (G-tube) feeds. Therefore, we sought to create a porcine model with a bifurcated esophagus wherein one portion of the esophageal lumen retained patency and the other part mimicked EA. After G-tube placement, thoracotomy was performed with subsequent partial stapled transection of the esophagus in a transverse manner, followed by longitudinal, partial stapled transection of the esophagus proximally and distally. Magnets were placed in the esophageal pouches. Proximal and distal esophageal pouches were created while preserving a parallel, narrower segment of continuous esophagus. G-tube feeds were used initially, but the animal ultimately tolerated full nutrition by mouth. The magnets successfully restored esophageal continuity. The animal regained much of the initial weight lost and survived to planned euthanasia. Necropsy revealed anastomosis formation without evidence of leak. A bifurcated porcine esophagus model was successfully devised, simulating EA while allowing the animal to receive oral feeds and clear oral secretions. This model is anticipated to promote animal well-being and ease of care during future investigations, as well as a platform for the safe development of new therapies for EA.
Tetangco, Eula; Elkhatib, Imad
A 38-year-old male with a history of colonic interposition for esophageal atresia as an infant presented with dysphagia and abdominal pain. On the basis of endoscopy findings, pathology, and response to therapy, he was found to have ulcerative colitis of the colonic conduit. PMID:27847835
Acher, Charles Wynn; Ostlie, Daniel J; Leys, Charles M; Struckmeyer, Shannon; Parker, Matthew; Nichol, Peter F
Introduction Outcome studies of tracheoesophageal fistula (TEF) and/or esophageal atresia (EA) are limited to retrospective chart reviews. This study surveyed TEF/EA patients/parents engaged in social media communities to determine long-term outcomes. Materials and Methods A 50-point survey was designed to study presentation, interventions, and ongoing symptoms after repair in patients with TEF/EA. It was validated using a test population and made available on TEF/EA online communities. Results In this study, 445 subjects completed the survey during a 2-month period. Mean age of patients when surveyed was 8.7 years (0-61 years) and 56% were male. Eighty-nine percent of surveys were completed by the parent of the patient. Sixty-two percent of patients underwent repair in the first 7 days of life. Standard open repair was most common (56%), followed by primary esophageal replacement (13%) and thoracoscopic repair (13%). Out of 405, 106 (26%) patients had postoperative leak. Postoperative leak was least likely in primary esophageal replacement (18%) and standard open repair (19%). Leak occurred in 32% of patients who had thoracoscopic repair; 31% (128/413) reported long-gap atresia, which was significantly associated with increased risk of postoperative leak (54/128, 42%) when compared with standard short-gap atresia (odds ratio, 3.5; p = 0.001). Out of 409, 221 (54%) patients reported dysphagia after repair, with only 77/221 (34.8%) reporting resolution by age 5. Out of 381, 290 (76%) patients reported symptoms of gastroesophageal reflux disease (GERD). There was no difference in dysphagia rates or GERD symptoms based on type of initial repair. Antireflux surgery was required in 63/290, 22% of patients with GERD (15% of all patients) and 27% of these patients who had surgery required more than one procedure antireflux procedure. The most common was Nissen fundoplication (73%), followed by partial wrap (14%). Reflux recurred in 32% of patients after
Bednarczyk, Damian; Śmigiel, Robert; Sąsiadek, Maria Małgorzata
Esophageal atresia and tracheo-esophageal fistula are severe congenital malformations, whose etiology is still poorly understood. So far, numerous genetic and environmental factors that may contribute to the occurrence of these defects have been described and the literature is dominated by the view of their common involvement in the etiology and pathogenesis of congenital esophageal atresia. In this review the authors present current knowledge on the embryogenesis of the esophagus and trachea, discuss environmental risk factors, and also list and describe genetic alterations identified so far in patients with congenital esophageal atresia.
Rothenberg, S S
The first thoracoscopic esophageal atresia with tracheoesophageal fistula (EATEF) repair was performed in March of 2000. This report evaluates the results and evolution of the technique in a single surgeons' experience after the first decade of thoracoscopic EATEF repair. From March 2000 to September 2012, 52 consecutive patients with type 3 EATEF, and an additional nine patients with pure esophageal atresia (EA) were repaired by or under the direct supervision of a single surgeon. Patient weight ranged from 1.2 to 3.8 kg (mean 2.6 kg). Twenty-two patients had significant associated congenital anomalies. The repairs were performed using three ports. The fistula was ligated using a single endoscopic clip, and the anastomosis was performed using a single layer of interrupted sutures. A transanastomotic tube and chest drain were left in all cases. Fifty-one of the 52 procedures were completed successfully thoracoscopically. Operative times ranged from 50 to 120 minutes (average 85 minutes). There were three clinical leaks, one in an EATEF and two in patients with long-gap pure EA, all resolved with conservative therapy. Oral feedings were started on day 5 in all other patients. Twelve of 61 patients required dilations (1-9), and 18 required a Nissen fundoplication for severe reflux. One patient required a thoracoscopic aortopexy for severe tracheomalacia. All patients are currently on full oral feedings. No patient has any evidence of chest wall asymmetry, winged scapula, or clinically significant scoliosis. There have been no recurrent fistulas. Thoracoscopic EA repair has proven to be an effective and safe technique. Initial experience resulted in a higher stricture rate, but this improved with experience and changes in technique. The results are superior to that of documented open series and avoid the morbidity of an open thoracotomy.
Vo, Nghia-Jack; Racadio, John M; Inge, Thomas H
Esophageal atresia with an associated tracheoesophageal fistula is a congenital anomaly requiring surgical correction. Recurrent stricture is the most common complication of surgical repair and is usually treated with mechanical dilation. Rarely, a recurrent completely obstructive stricture can cause obliteration of the anastomosis, preventing passage of a wire for dilation. This condition requires operative correction. In the case presented herein, the obliterated esophageal lumen from an obstructing stricture was operatively corrected with use of a novel transluminal technique. The obstruction was successfully crossed with a modified Chiba biopsy needle covered in a dilator through a gastrostomy. After subsequent balloon dilation, the lumen has remained patent for more than 3 years without significant complication.
Patti, G; Marrocco, G; Mazzoni, G; Catarci, A
The presence of a preduodenal portal vein is an extremely rare event. Even rarer is its association with a preduodenal position of the common bile duct. In the case reported both these abnormalities were simultaneously associated with an esophageal atresia without fistula and atresia of the second duodenal portion.
Boersma, Doeke; Koot, Bart G; van der Griendt, Erik Jonas; van Rijn, Rick R; van der Steeg, Alida F
Communicating bronchopulmonary foregut malformations are extremely rare congenital malformations, characterized by a communicating fistula between an isolated part of the respiratory system and the esophagus or the stomach. In this article, we present a case of esophageal atresia type C, later diagnosed as a rare form of a communicating bronchopulmonary foregut malformation, an esophageal atresia combined with right main bronchus originating from the lower esophagus. Therapeutic resection of the right lung was complicated by postpneumonectomy syndrome.
Chowdhary, Sujit K; Kandpal, Deepak K; Agarwal, Deepak; Balan, Saroja; Jerath, Nameet; Sibal, Anupam; Broor, Sohan L
The aim of the study is to report feasibility and safety of endoscopic esophageal substitution in infants with pure esophageal atresia and wide gap tracheoesophageal fistula with a minimum one year follow-up. This prospective study was conducted from January 2012 for twenty four consecutive months at Apollo Hospital, New Delhi. All babies either followed up or referred for esophageal substitution without any history of mediastinitis or associated major congenital anomaly and weighing greater than 6kg were to be included in the study. The indication, intraoperative details, operative approach, conversion to open, esophageal substitute, postoperative ventilation, ICU and hospital stay, time to solid foods, morbidity and mortality were recorded. Informed consent was obtained from all the parents and ethical clearance was obtained for the study from the hospital ethical committee. Postoperatively babies were followed up monthly for first six months, 3 monthly for next six months and annually thereafter. Between January 2012 and December 2013, in the two year period six infants were admitted for laparoscopic gastric transposition. In five patients the procedure was completed by the laparoscopic approach and one required conversion to open surgery owing to dense adhesions. The age range at the time of surgery was from 8months to 12months with a mean age of 10months. Four patients had pure esophageal atresia (type A) and two had wide gap esophageal atresia with distal tracheoesophageal atresia (type C). Five had primary esophagostomy and gastrostomy as a newborn, the sixth had postoperative anastomotic leak and required subsequent diversion. The mean operating time was 194minutes (range 170-210minutes). The mean stay in ICU was 7days with a range of 4-12days. All patients were ventilated in the postoperative period for an average of 5days with a range of 4-7days. One patient had prolonged gastric ileus which delayed the oral feeds by 14days. The mean time to start the
Sharma, A K; Mangal, D
The survival of the patients with esophageal atresia an tracheo esophageal fistula is believed to be an epitome of the success of the neonatal surgery. Restoring the continuty of the food pipe by esophagus to esophagus anastomosis is the best option. Preservation of natural esophagus by delayed repair in a wide gap esophageal atresia is a preferred technique worldwide, however such a management required prolonged hospitalization and dedicated nursing care, which is often not available in most of the centres in India. Esophageal substitutes in wide gap requires multiple operations and have long term problems, so remains the last option. I use the technique of oblique anastomosis which had distrinct advantage over circular anastomosis in the management of esophageal atresia1. This techniqe helps in bridging wide gap to some extent & minimal stricture formation.
Osaka, Yoshimune; Ando, Takeshi; Kozono, Yuuki; Saito, Ikue; Saito, Rie; Shimada, Muneaki
Trisomy 18 is one of the congenital disorders caused by a chromosomal abnormality. Ninety percent of fetuses with trisomy 18 have various other malformations. The present patient had heart failure due to a complex cardiac malformation and a Gross C type esophageal atresia. Before the esophageal banding, ventilation of the lungs was impossible and respiratory condition was unstable. Considering that direction of the shunt can easily change by hyperventilation and high oxygen concentration, we employed the lowest oxygen concentration and ventilation as possible. In the present case, it was necessary to provide respiratory care for both esophageal atresia and complex cardiac malformation.
Garabedian, Charles; Sfeir, Rony; Langlois, Carole; Bonnard, Arnaud; Khen-Dunlop, Naziha; Gelas, Thomas; Michaud, Laurent; Auber, Fréderic; Gottrand, Fréderic; Houfflin-Debarge, Véronique
Our study aimed at (1) evaluating neonatal treatment and outcome of neonates with either a prenatal or a postnatal diagnosis of esophageal atresia (EA) and (2) analyzing the impact of prenatal diagnosis on outcome based on the type of EA. We conducted a population-based study using data from the French National Register for infants with EA born from 2008-2010. We compared prenatal, maternal, and neonatal characteristics among children with prenatal vs postnatal diagnosis and EA types I and III. We defined a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and death at 1 year. Four hundred sixty-nine live births with EA were recorded with a prenatal diagnosis rate of 24.3%; 82.2% of EA type I were diagnosed prenatally compared with 17.9% of EA type III (P < .001). Transfer after birth was lower in case of prenatal diagnosis (25.6% vs 82.5%; P < .001). The delay between birth and first intervention did not differ significantly among groups. The defect size was longer among the prenatal diagnosis group (2.61 vs 1.48 cm; P < .001). The composite variables were higher in prenatal diagnosis subset (44% vs 27.6%; P = .003) and in EA type I than in type III (58.1% vs 28.3%; P < .001). Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity rate related to the EA type (type I and/or long gap). Even though it does not modify neonatal treatment and the 1-year outcome, prenatal diagnosis allows antenatal parental counselling and avoids postnatal transfers. Copyright © 2015 Elsevier Inc. All rights reserved.
Zaborowska, Kamila; Rogowski, Błażej; Kalińska, Anita; Nosek, Marzena; Golonka, Anna; Lesiuk, Witold; Obel, Marcin
Introduction Thoracoscopic esophageal atresia (EA) repair was first performed in 1999, but still the technique is treated as one of the most complex pediatric surgical procedures. Aim The study presents a single-center experience and learning curve of thoracoscopic repair of esophageal atresia and tracheo-esophageal (distal) fistula. Material and methods From 2012 to 2014, 10 consecutive patients with esophageal atresia and tracheo-esophageal fistula were treated thoracoscopically in our center. There were 8 girls and 2 boys. Mean gestational age was 36.5 weeks and mean weight was 2230 g. Four children had associated anomalies. The surgery was performed after stabilization of the patient between the first and fourth day after birth. Five patients required intubation before surgery for respiratory distress. Bronchoscopy was not performed before the operation. Results In 8 patients, the endoscopic approach was successfully used thoracoscopically, while in 2 patients conversion to an open thoracotomy was necessary. In all patients except 1, the anastomosis was patent, with no evidence of leak. One patient demonstrated a leak, which did not resolve spontaneously, necessitating surgical repair. In long-term follow-up, 1 patient required esophageal dilatation of the anastomosis. All patients are on full oral feeding. Conclusions The endoscopic approach is the method of choice for the treatment of esophageal atresia in our center because of excellent visualization and precise atraumatic preparation even in neonates below a weight of 2000 g. PMID:25960794
Sayari, Arash J; Tashiro, Jun; Wang, Bo; Perez, Eduardo A; Lasko, David S; Sola, Juan E
We hypothesize that weekend esophageal atresia and tracheoesophageal fistula (EA/TEF) repair has worse outcomes compared to procedures performed on weekdays. Kids' Inpatient Database (1997-2009) was searched for EA/TEF in infants admitted at <8days of life. Cases were limited to patients who underwent repair during their hospitalization. Risk-adjusted multivariate analysis (MVA) compared complications, mortality, and resource utilization (length of stay [LOS] total charges [TC]) between weekday and weekend procedures. Overall, 861 EA/TEF cases with known day of repair were identified. Cohort survival was 96%. On risk-adjusted MVA, complication rates were higher with EA/TEF repair on a weekend (OR: 2.2) compared to a weekday. Additionally, complications (OR: 6.5) and LOS (OR: 9.3) were found to be higher among African American children compared to Caucasians. LOS was higher in patients with Medicaid (OR: 2.4) and repairs performed at non-teaching hospitals (OR: 3.2). Weekend vs. weekday procedure had no significant effect on mortality or resource utilization. By risk-adjusted MVA, increased complication rates for EA/TEF are seen in patients undergoing repair on weekends compared to weekdays. Additionally, African American children experienced higher complication rates compared to Caucasians. LOS after repair varies according to race, payer status, and hospital characteristics. Copyright © 2016 Elsevier Inc. All rights reserved.
Nishi, Eriko; Takamizawa, Shigeru; Iio, Kenji; Yamada, Yasumasa; Yoshizawa, Katsumi; Hatata, Tomoko; Hiroma, Takehiko; Mizuno, Seiji; Kawame, Hiroshi; Fukushima, Yoshimitsu; Nakamura, Tomohiko; Kosho, Tomoki
Trisomy 18 is a common chromosomal aberration syndrome involving growth impairment, various malformations, poor prognosis, and severe developmental delay in survivors. Although esophageal atresia (EA) with tracheoesophageal fistula (TEF) is a potentially fatal complication that can only be rescued through surgical correction, no reports have addressed the efficacy of surgical intervention for EA in patients with trisomy 18. We reviewed detailed clinical information of 24 patients with trisomy 18 and EA who were admitted to two neonatal intensive care units in Japan and underwent intensive treatment including surgical interventions from 1982 to 2009. Nine patients underwent only palliative surgery, including six who underwent only gastrostomy or both gastrostomy and jejunostomy (Group 1) and three who underwent gastrostomy and TEF division (Group 2). The other 15 patients underwent radical surgery, including 10 who underwent single-stage esophago-esophagostomy with TEF division (Group 3) and five who underwent two-stage operation (gastrostomy followed by esophago-esophagostomy with TEF division) (Group 4). No intraoperative death or anesthetic complications were noted. Enteral feeding was accomplished in 17 patients, three of whom were fed orally. Three patients could be discharged home. The 1-year survival rate was 17%: 27% in those receiving radical surgery (Groups 3 and 4); 0% in those receiving palliative surgery (Groups 1 and 2). Most causes of death were related to cardiac complications. EA is not an absolute poor prognostic factor in patients with trisomy 18 undergoing radical surgery for EA and intensive cardiac management.
Alshehri, Abdullah; Lo, Andrea; Baird, Robert
Advances in care for neonates with esophageal atresia (EA) has improved overall survival rates. Disease-specific prognostic scores for EA assess mortality risk without assessing patient morbidity. We undertook an analysis of these and generic scoring systems evaluating their ability to predict early nonmortality outcomes. We conducted a retrospective review of all patients with EA at our tertiary care children's hospital. Disease-specific (Spitz, Waterston, and Montreal) and generic prognostic scores (Score for Neonatal Acute Physiology II and Score for Neonatal Acute Physiology-Perinatal Extension) were calculated. Outcomes investigated included mortality and early nonmortality outcomes (leak, stricture, and recurrent fistula). These were assessed individually and as a composite "poor outcome" score. Correlations were sought, and receiver operating characteristic curves were generated. Fifty patients were included for analysis, with 5 deaths (10%) in our series. Eight patients developed a postoperative leak, 18 developed stenosis requiring dilatation, and 2 developed refistulization. Overall, 51% of survivors had a poor composite outcome. Although no prognostic score achieved statistical significance, the generic scores outperformed the disease-specific scores in predicting early nonmortality outcomes. Postoperative morbidity remains common in patients with EA. Disease-specific, preexisting prognostic scoring systems do not delineate surviving patients at risk for early complications and appears to underperform when compared with generic prognostic scores. Copyright © 2012 Elsevier Inc. All rights reserved.
Fragoso, Ana Catarina; Martinez, Leopoldo; Estevão-Costa, José; Tovar, Juan A
Esophageal atresia and tracheoesophageal fistula (EA-TEF) survivors suffer respiratory morbidity of unclear pathogenesis. Defective lung morphogenesis has been described in the rat model. This study examined fetal lung growth and maturity in rats and patients with EA-TEF. Pregnant rats received either adriamycin or vehicle. Control and adriamycin-exposed lungs, with and without EA-TEF, were weighed and processed for RT-PCR, DNA quantification, immunofluorescence and immunoblot analysis of TTF1, VEGF, Sp-B, and α-sma. Twenty human lungs were also processed for immunofluorescence and Alcian-blue staining. Lungs from fetuses with EA-TEF (E21) showed decreased total DNA; FGF7 and TTF1 mRNA expressions were upregulated at E15 and E18, respectively. Protein expression and immunofluorescent distribution of maturity markers were similar. Lungs from stillborns with EA-TEF showed decreased epithelial expression of Sp-B and VEGF whereas those from newborns tended to have less Sp-B and more VEGF and mucous glands. The lungs of rats with EA-TEF were hypoplastic but achieved near-normal maturity. Stillborns with EA-TEF exhibited an apparently disturbed differentiation of the airway epithelium. Newborns with EA-TEF demonstrated subtle differences in the expression of differentiation markers, and increased number of mucous glands that could influence postnatal respiratory adaptation and explain some respiratory symptoms of EA-TEF survivors. Copyright © 2015 Elsevier Inc. All rights reserved.
LeSouëf, P N; Myers, N A; Landau, L I
Recurrent respiratory illnesses are frequent in infants following repair of esophageal atresia and functional abnormalities of respiratory and esophageal function are often seen in older children. Recurrent aspiration is a potential cause of these respiratory abnormalities, but a relationship between abnormalities of gastrointestinal and respiratory mechanics has not been adequately investigated. We sought an association between lower esophageal sphincter (LES) incompetence, gastroesophageal reflux (GER), and respiratory function abnormalities in 18 subjects (age 12 to 21 years) following repair of esophageal atresia (Vogt type 111B). In each subject, measurements were made of spirometry, lung volumes assessed by plethysmography, esophageal manometry recorded using a constantly infused fluid-filled trilumen catheter to assess LES pressure and esophageal motility, and esophageal pH monitoring to detect GER. Subjects were grouped according to the presence or absence of a radiologically supported diagnosis of pneumonia in the first 4 years of life. Lung volumes were mildly but significantly decreased in the "pneumonia" group compared with the "nonpneumonia" group. There was no association between abnormalities of respiratory function and abnormal LES pressure or the presence of GER. These data suggest that pneumonia in esophageal atresia infants is associated with mild long-term lung damage. LES dysfunction and GER do not appear to play a major role in this process.
Hypertrophic pyloric stenosis is brought to attention by its characteristic non-bilious vomiting. In a patient with pure esophageal atresia and a feeding gastrostomy, the symptoms were modified and the diagnosis was delayed. This case report highlights the clinical features of this rare combination, whose diagnosis was easily established once the entity was considered. PMID:24741218
Capeto, F A; Lima, F J B; Okoba, W; Ramos, F L; Messias, T F A; Rigonatto, G A; Sbragia, L; Magalhães, P J C; Melo-Filho, A A
Esophageal atresia (EA) is characterized by esophageal and gastric motility changes secondary to developmental and postsurgical damage. This study evaluated the in vitro contractile profile of the distal esophagus and gastric fundus in an experimental model of EA induced by doxorubicin (DOXO). Wistar pregnant rats received DOXO 2.2 mg/kg on the 8th and 9th gestational days. On day 21.5, fetuses were collected, sacrificed, and divided into groups: control, DOXO without EA (DOXO-EA), and DOXO with EA (DOXO+EA). Strips from the distal esophagus and gastric fundus were mounted on a wire myograph and isolated organ-bath system, respectively, and subjected to increasing concentrations of carbamylcholine chloride (carbachol, CCh). The isolated esophagus was also stimulated with increasing concentrations of KCl. In esophagus, the concentration-effect curves were reduced in response to CCh in the DOXO+EA and DOXO-EA groups compared to the control group (P<0.05). The maximum effect values (Emax) for DOXO+EA and DOXO-EA were significantly lower than control (P<0.05), but the half-maximal effective concentration (EC50) values were not significantly different when the three groups were compared (P>0.05). In response to KCl, the distal esophagus samples in the three groups were not statistically different with regard to Emax or EC50 values (P>0.05). No significant difference was noted for EC50 or Emax values in fundic strips stimulated with CCh (P>0.05). In conclusion, exposure of dams to DOXO during gestation inhibited the contractile behavior of esophageal strips from offspring in response to CCh but not KCl, regardless of EA induction. The gastric fundus of DOXO-exposed offspring did not have altered contractile responsiveness to cholinergic stimulation.
Capeto, F.A.; Lima, F.J.B.; Okoba, W.; Ramos, F.L.; Messias, T.F.A.; Rigonatto, G.A.; Sbragia, L.; Magalhães, P.J.C.; Melo-Filho, A.A.
Esophageal atresia (EA) is characterized by esophageal and gastric motility changes secondary to developmental and postsurgical damage. This study evaluated the in vitro contractile profile of the distal esophagus and gastric fundus in an experimental model of EA induced by doxorubicin (DOXO). Wistar pregnant rats received DOXO 2.2 mg/kg on the 8th and 9th gestational days. On day 21.5, fetuses were collected, sacrificed, and divided into groups: control, DOXO without EA (DOXO-EA), and DOXO with EA (DOXO+EA). Strips from the distal esophagus and gastric fundus were mounted on a wire myograph and isolated organ-bath system, respectively, and subjected to increasing concentrations of carbamylcholine chloride (carbachol, CCh). The isolated esophagus was also stimulated with increasing concentrations of KCl. In esophagus, the concentration-effect curves were reduced in response to CCh in the DOXO+EA and DOXO-EA groups compared to the control group (P<0.05). The maximum effect values (Emax) for DOXO+EA and DOXO-EA were significantly lower than control (P<0.05), but the half-maximal effective concentration (EC50) values were not significantly different when the three groups were compared (P>0.05). In response to KCl, the distal esophagus samples in the three groups were not statistically different with regard to Emax or EC50 values (P>0.05). No significant difference was noted for EC50 or Emax values in fundic strips stimulated with CCh (P>0.05). In conclusion, exposure of dams to DOXO during gestation inhibited the contractile behavior of esophageal strips from offspring in response to CCh but not KCl, regardless of EA induction. The gastric fundus of DOXO-exposed offspring did not have altered contractile responsiveness to cholinergic stimulation. PMID:25760030
Fragoso, Ana Catarina; Ortiz, Ruben; Hernandez, Francisco; Olivares, Pedro; Martinez, Leopoldo; Tovar, Juan A
Both esophageal atresia (EA) and duodenal atresia (DA) involve deficient anti-reflux barrier, poor esophageal function and eventually, duodenogastric reflux. This study aims at examining the upper gastrointestinal functional status in a cohort of patients with both EA and DA. A retrospective survey of patients treated for EA and DA between 1965 and 2012 was conducted. Clinical charts, office visits, imaging, upper gastrointestinal endoscopy and esophageal pH metry/impedance were used to assess the long-term condition of the esophagus, the presence of gastroesophageal reflux disease (GERD) and/or the need for fundoplication. Twenty out of 581 patients treated for EA had associated DA. Ten/twenty children survived; 1 had primary esophageal replacement. With a median follow-up of 9 years, 8/9 had complicated outcomes and 5 still suffered digestive ailments: 2 GER; 1 eosinophilic esophagitis; 1 nodular gastritis, and 1 wrap herniation. A total of 10 procedures were performed: 8 fundoplications, 1 esophagogastric dissociation and 1 replacement with colon. The association of EA with DA involves a poor upper digestive function with high risks of GERD and fundoplication failure. The lifelong synergistic play of esophageal, gastric and duodenal dysfunctions in these patients prompts long-term follow-up, and eventually active treatment. Copyright © 2015 Elsevier Inc. All rights reserved.
Iwańczak, Barbara; Kosmowska-Miśków, Agnieszka; Jamer, Tatiana; Patkowski, Dariusz
A case of a 4.5-year-old girl from a twin pregnancy, who was diagnosed after birth with the congenital esophageal atresia (type 3), and at the age of 4 with the potential coeliac disease. Congenital esophageal atresia was successfully treated surgically in infancy with the thoracoscopic method. The potential coeliac disease was detected in the child with a correct histopathological examination of intestinal villi and showing no enteropathy symptoms based on the presence of antibodies against tissue transglutaminase and against endomysial antibodies of smooth muscles in serum and the presence of HLA DQ2.5. In the treatment of the potential coeliac disease the girl followed a gluten-free diet.
El Azzouzi, Driss
Intrathoracic gastric volvulus in the neonatal period is a rare surgical emergency. Delays in diagnosis and treatment are life-threatening due to progressive deterioration of the gastric walls. Presentation in this period can be confused with the possibility of esophageal atresia or esophageal web. The upper gastrointestinal tract contrast study is diagnostic in this disease. The authors report a case of acute intrathoracic gastric volvulus diagnosis by radiologic-contrast-study in 1-day-old girl that was confirmed at surgery. The physiopathology, classification and different presentations of this entity are briefly reviewed. PMID:25309661
van der Zee, David C; Bax, Klaas N M A
Single center experience with thoracoscopic repair of esophageal atresia with distal fistula and of tracheomalacia. Between May 2000 and December 2006, 51 neonates with an esophageal atresia were presented for thoracoscopic repair. Gestational age varied from 31 3/7 to 42 2/7 weeks (M = 37 2/7). Birth weight was between 1025 g and 4030 g (mean 2620 g). Concomitant anomalies or VACTERL association were encoutered in 31 patients (61%). Duration of the operation was from 90 minutes to 390 minutes (mean 178 minutes). All but 1 patient had an esophageal atresia with a distal fistula. Six patients had tracheomalacia requiring aortopexia, which was performed thoracoscopically. In 2 patients the thoracoscopic procedure had to be converted to a thoracotomy. All other patients underwent a successful thoracoscopic repair. One patient died in the postoperative period because of sepsis. A total of 22 patients (45%) developed a stenosis in the postoperative follow up (1 month-7y 7 month) requiring 1 to 18 dilatations (mean 1.5). Postoperative leakage occurred in 9 patients (18%). Recurrent fistula was encountered in 2 patients. A total of 11 patients (22%) underwent a laparoscopic antireflux procedure for either recurring stenosis (8) or ALTES (3). Six children (12%) underwent thoracoscopic aortopexy for tracheomalacia. In 2 children symptoms recurred for which a successful repeat thoracoscopic aortopexy was undertaken. The thoracoscopic approach to the treatment of esophageal atresia and tracheomalacia is becoming increasingly accepted. The cosmesis is undoubtedly better. The secundary effects like thoracic cage deformities, winged scapula, or scoliosis have not yet been described and are expected to be reduced in comparison to the open technique. Sequelae like leakage, stenosis, recurrent fistulae, and GERD and ALTES will probably remain the same. Whether thoracoscopic dissection has less detrimental effect on disturbed motility remains to be proven. Thoracoscopic aortopexy
Abou Chaar, Mohamad K; Meyers, Mariana L; Tucker, Bethany D; Galan, Henry L; Liechty, Kenneth W; Crombleholme, Timothy M; Marwan, Ahmed I
The antenatal diagnosis of a combined esophageal atresia without tracheoesophageal fistula and duodenal atresia with or without gastric perforation is a rare occurrence. These diagnoses are difficult and can be suspected on ultrasound by nonspecific findings including a small stomach and polyhydramnios. Fetal magnetic resonance imaging adds significant anatomical detail and can aid in the diagnosis of these complicated cases. Upon an extensive literature review, there are no reports documenting these combined findings in a twin pregnancy. Therefore we believe this is the first case report of an antenatal diagnosis of combined pure esophageal and duodenal atresia in a twin gestation. We present a case of a 30-year-old G1P0 white woman at 22-week gestation with a monochorionic-diamniotic twin pregnancy discordant for esophageal atresia, duodenal atresia with gastric perforation, hypoplastic left heart structures, and significant early gestation maternal polyhydramnios. In this case, fetal magnetic resonance imaging was able to depict additional findings including area of gastric wall rupture, hiatal hernia, dilation of the distal esophagus, and area of duodenal obstruction and thus facilitated the proper diagnosis. After extensive counseling at our multidisciplinary team meeting, the parents elected to proceed with radiofrequency ablation of the anomalous twin to maximize the survival of the normal co-twin. The procedure was performed successfully with complete cessation of flow in the umbilical artery and complete cardiac standstill in the anomalous twin with no detrimental effects on the healthy co-twin. Prenatal diagnosis of complex anomalies in twin pregnancies constitutes a multitude of ethical, religious, and cultural factors that come into play in the management of these cases. Fetal magnetic resonance imaging provides detailed valuable information that can assist in management options including possible prenatal intervention. The combination of a cystic
Bastard, François; Bonnard, Arnaud; Rousseau, Véronique; Gelas, Thomas; Michaud, Laurent; Irtan, Sabine; Piolat, Christian; Ranke-Chrétien, Aline; Becmeur, François; Dariel, Anne; Lamireau, Thierry; Petit, Thierry; Fouquet, Virginie; Le Mandat, Aurélie; Lefebvre, Francis; Allal, Hossein; Borgnon, Josephine; Boubnova, Julia; Habonimana, Edouard; Panait, Nicoleta; Buisson, Philippe; Margaryan, Marc; Michel, Jean-Luc; Gaudin, Jean; Lardy, Hubert; Auber, Frédéric; Borderon, Corinne; De Vries, Philine; Jaby, Olivier; Fourcade, Laurent; Lecompte, Jean François; Tolg, Cécilia; Delorme, Benoit; Schmitt, Françoise; Podevin, Guillaume
Thoracotomy as surgical approach for esophageal atresia treatment entails the risk of deformation of the rib cage and consequently secondary thoracogenic scoliosis. The aim of our study was to assess these thoracic wall anomalies on a large national cohort and search for factors influencing this morbidity. Pediatric surgery departments from our national network were asked to send recent thoracic X-ray and operative reports for patients born between 2008 and 2010 with esophageal atresia. The X-rays were read in a double-blind manner to detect costal and vertebral anomalies. Among 322 inclusions from 32 centers, 110 (34.2%) X-rays were normal and 25 (7.7%) displayed thoracic malformations, including 14 hemivertebrae. We found 187 (58.1%) sequelae of surgery, including 85 costal hypoplasia, 47 other types of costal anomalies, 46 intercostal space anomalies, 21 costal fusions and 12 scoliosis, with some patients suffering from several lesions. The rate of patients with these sequelae was not influenced by age at intervention, weight at birth, type of atresia, number of thoracotomy or size of the center. The rate of sequelae was higher following a classical thoracotomy (59.1%), whatever the way that thoracotomy was performed, compared to nonconverted thoracoscopy (22.2%; p=0.04). About 60 % of the patients suffered from a thoracic wall morbidity caused by the thoracotomy performed as part of surgical treatment of esophageal atresia. Minimally invasive techniques reduced thoracic wall morbidity. Further studies should be carried out to assess the potential benefit of minimally invasive approaches to patient pulmonary functions and on the occurrence of thoracogenic scoliosis in adulthood. Level III retrospective comparative treatment study. Copyright © 2017 Elsevier Inc. All rights reserved.
Ethun, Cecilia G; Fallon, Sara C; Cassady, Christopher I; Mehollin-Ray, Amy R; Olutoye, Oluyinka O; Zamora, Irving J; Lee, Timothy C; Welty, Stephen E; Cass, Darrell L
The purpose of this study was to describe prenatal imaging characteristics and outcomes of fetuses with suspected esophageal atresia (EA) in order to improve prenatal diagnosis, counseling, and management. The medical records of all patients referred to our multidisciplinary fetal center for suspected EA from January 2003 to April 2013 were reviewed retrospectively. Thirty-three patients were referred with a prenatal diagnosis of possible EA. Following fetal center evaluation with MRI, EA was deemed unlikely in 6 (18%) fetuses. Of 27 fetuses in whom EA could not be excluded, EA was confirmed postnatally in 15 (56%), excluded in 7 (26%), and unconfirmed in 5 (3 fetal losses; 2 lost to follow-up). Imaging characteristics on fetal MRI associated with the highest positive predictive values (PPV) were an esophageal pouch (100%) and a small stomach (75%). The finding of polyhydramnios had high sensitivity (93%) but low specificity (31%) and PPV (61%) for a diagnosis of EA. Prenatal imaging and fetal center evaluation correctly identify the presence or absence of esophageal atresia in 78% of patients referred on suspicion of this condition. The presence of an esophageal pouch on fetal MRI has significant predictive value for EA. These data may assist with evidence-based prenatal family counseling. Copyright © 2014 Elsevier Inc. All rights reserved.
Hiradfar, Mehran; Gharavifard, Mohammad; Shojaeian, Reza; Joodi, Marjan; Nazarzadeh, Reza; Sabzevari, Alireza; Yal, Nazila; Eslami, Reza; Mohammadipour, Ahmad; Azadmand, Ali
Background: Thoracoscopic treatment of esophageal atresia and tracheoesophageal fistula (EA+TEF) is accepted as a superior technique at least in cosmetic point of view but it is considered as an advance endoscopic procedure that needs a learning curve to be performed perfectly. This is the first report of Iranian group pediatric surgeons in thoracoscopic approach to EA. Methods and Materials: Since 2010, twenty four cases with EA+TEF underwent thoracoscopic approach in Sarvar Children Hospital (Mashhad -Iran). During the first 6 months, thoracoscopic approach to 6 cases of EA+TEF was converted to open procedure because of technical and instrumental problems. The first case of successful thoracoscopic EA repair was accomplished in 2010 and since then, 10 cases of EA+ TEF among 18 patients were treated successfully with thoracoscopic approach Results: Overall conversion rate was 58.3% but conversion rate after the primary learning curve period, was 35.7%. The main conversion causes include difficulties in esophageal anastomosis, limited exposure and deteriorating the patient's condition. Anastomotic leak and stenosis were observed in 20% and 40% respectively. Overall mortality rate was 4.2%. Conclusion: Thoracoscopic repair of esophageal atresia seems feasible and safe with considerable superiorities to the conventional method although acceptable results needs a prolonged learning curve and advanced endoscopic surgical skill. Clear judgment about the best surgical intervention for EA according to all cosmetic and functional outcomes needs further studies. PMID:27471677
Background VATER association was first described in 1972 by Quan and Smith as an acronym which identifies a non-random co-occurrence of Vertebral anomalies, Anal atresia, Tracheoesophageal fistula and/or Esophageal atresia, Radial dysplasia. It is even possible to find out Cardiovascular, Renal and Limb anomalies and the acronym VACTERL was adopted, also, embodying Vascular, as single umbilical artery, and external genitalia anomalies. Methods Data on patients with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) admitted in the Neonatal Intensive Care Unit (NICU) between January 2003 and January 2013 were evaluated for the contingent occurrence of typical VACTERL anomalies (VACTERL-type) and non tipical VACTERL anomalies (non-VACTERL-type). The inclusion criterion was the presence of EA with or without TEF plus two or more of the following additional malformations: vertebral defects, anal atresia, cardiovascular defects, renal anomalies and lower limb deformities, like radial dysplasia. Results Among 52 patients with EA/TEF, 20 (38,4%) had isolated EA and 7 (21,8%) had a recognized etiology such a syndrome and therefore were excluded. Among 32 infants with EA and associated malformations, 15 (46,8%) had VACTERL association. The most common anomalies were congenital heart defects (73,3%), followed by vertebral anomalies (66,6%). Many patients also had additional non-VACTERL-type defects. Single umbilical artery was the most common one followed by nervous system abnormalities and anomalies of toes. Between the groups of infants with VACTERL type and non-VACTERL-type anomalies, there are several overlapping data regarding both the tipically described spectrum and the most frequently reported non-VACTERL-type malformations. Thus, it is possible to differentiate infants with a full phenotype (VACTERL full phenotype) and patients that do not meet all the criteria mentioned above, but with some homologies with the first group (VACTERL partial
Fragoso, Ana Catarina; Tovar, Juan A.
Esophageal atresia with or without tracheoesophageal fistula (EA ± TEF) occurs in 1 out of every 3000 births. Current survival approaches 95%, and research is therefore focused on morbidity and health-related quality of life issues. Up to 50% of neonates with EA ± TEF have one or more additional malformations including those of the respiratory tract that occur in a relatively high proportion of them and particularly of those with vertebral, anal, cardiac, tracheoesophageal, renal, and limb association. Additionally, a significant proportion of survivors suffer abnormal pulmonary function and chronic respiratory tract disease. The present review summarizes the current knowledge about the nature of these symptoms in patients treated for EA ± TEF, and explores the hypothesis that disturbed development and maturation of the respiratory tract could contribute to their pathogenesis. PMID:24829898
Esposito, Ciro; Langer, Jacob C; Schaarschmidt, Klaus; Mattioli, Girolamo; Sauer, Carolien; Centonze, Antonella; Cigliano, Bruno; Settimi, Alessandro; Jasonni, Vincenzo
The validity of open fundoplication after esophageal atresia (EA) repair is still disputed. The authors have retrospectively evaluated the results achieved in their centers using laparoscopic antireflux procedures (LARP) in children operated for EA at birth. From 1998 to 2002, 350 children underwent LARP. Of these, 21 (6%) underwent EA repair at birth. Our study focused only on the management of these 21 patients; 5 of them (23.8%) were neurologically impaired children (NIC). All underwent LARP, 9 patients according to Nissen, 9 according to Thal, and 3 according to Toupet. The 5 NIC with feeding problems underwent concomitant g-tube placement during the same procedure. All the procedures were completed in laparoscopy, without intraoperative complications. The mean operative time was 65 minutes (range 45-140). We had no mortality in our series. Hospital stay varied from 2 to 9 days (median 3 days). At a maximum follow-up of 6 years, all patients were evaluated with a 24-hours pH-metry and barium swallow. The 16 neurologically normal children were free of symptoms at the last follow-up; five of them (31.2%) had mild dysphagia, which disappeared spontaneously within 3-6 months. One girl experienced an important episode of aspiration 2 years after the LARP, although there was no evidence of reflux at the follow-up examinations. As for the 5 NIC, one patient eats only through a g-tube, the other 4 undergo mixed feeding (g-tube and mouth); none have signs of GER, but two of them still present respiratory symptoms, and one has delayed gastric emptying. In our experience laparoscopic antireflux surgery is an appropriate treatment of GER in children operated for EA at birth, independently of the antireflux mechanism adopted; the 31.2% rate of short-term dysphagia presenting as residual respiratory symptoms may be due to a primary dysmotility of the esophagus consequent to the esophageal atresia.
Henriques-Coelho, Tiago; Soares, Tony R; Miranda, Alice; Moreira-Pinto, João; Correia-Pinto, Jorge
Thoracoscopic repair of esophageal atresia has becoming the gold standard in many centers because it allows a better cosmetic result and avoids the musculoskeletal sequelae of a thoracotomy. Natural orifice translumenal endocopic surgery (NOTES) is a new surgical paradigm, and its human application has already been started in some procedures. In the present study, we explore the feasibility of performing an esophagoesophageal anastomosis using a single transthoracic single port combined with a peroral access in a rabbit model to simulate repair of esophageal atresia by hybrid NOTES in a human newborn. Adult male rabbits (Oryctolagus cuniculus, n=28) were used to perform the surgical protocol. We used a transthoracic telescope with a 3-mm working channel and a flexible endoscope with a 2.2-mm working channel by peroral access. We performed total esophagotomy with peroral scissors followed by an esophagoesophageal anastomosis achieved with a rigid transthoracic scope helped by the peroral operator. Extracorporeal transthoracic knots were performed to complete the anastomosis. The anastomoses were examined in loco and ex loco, after animal sacrifice. We successfully accomplished a complete esophageal anastomosis in all rabbits using a combination of transthoracic and peroral 3-mm instruments. This study provides important insights for a possible translation of hybrid NOTES to human newborns with esophageal atresia. Forward studies to accomplish their feasibility in human newborns will still be necessary.
Hagău, N; Cocu, S; Geczi-Toth, I; Studnicska, D; Ciuce, C
The aim of this clinical prospective study was the follow up of a nutritional management protocol for children with esophageal atresia and tracheoesophageal fistula for whom the esophageal substitution was performed with left vascularized colon. In this study entered infants aged 3 months to 18 months old with a major nutritional deficit, due to respiratory infections complications, parastomal leaks and accelerated gastrointestinal transit. All infants were underweight, with a single exception, who had no preoperative complications. The patient was enterally fed postoperatively, all the other patients receiving combined enteral and parenteral nutrition for 5-6 days. The enteral nutrition was delivered early through a trans-anastomotic feeding tube. In the 5th-7th day, complete enteral nutrition was obtained. The parenteral nutrition followed our own recipe: a 10% amino-acid mixture, 50% glucose and Ringer plus electrolytes and vitamins. There was a critical transitional stage between the gastric tube feeding and the oral nutrition. These infants have the suction and the deglutition reflexes modified, followed by oral sensory and motor deficits. After the release from the hospital the patients have been surveyed, the oldest reaching now the age of 7. The earlier the reconstruction was performed, the less problems in oral nutrition were encountered.
Despite early surgical repair, congenital esophageal atresia with or without tracheoesophageal fistula (EA ± TEF) has long-term effects on respiratory and gastrointestinal function. This review updates summarizes research published since 2003 on long-term respiratory complications in patients with a history of EA ± TEF. Pulmonary hypoplasia appears to not be rare in patients with EA ± TEF. Tracheomalacia is common and is associated with respiratory symptoms in childhood. Aspiration, associated with esophageal dysmotility and/or gastroesophageal reflux, may lead to reduced pulmonary function and bronchiectasis. Pulmonary function is generally normal, although lower than in control patients, and restrictive defects tend to be commoner than obstructive defects. Abnormal airway reactivity is common and, along with respiratory symptoms, is associated with atopy. However, the inflammatory profile in EA ± TEF patients based on bronchial biopsies and exhaled nitric oxide differs from typical allergic asthma. Recent studies suggest that in older patients, respiratory symptoms tend to be associated with atopy, but abnormal lung function tends to be associated with gastroesophageal reflux and with chest wall abnormalities. Early detection and management of aspiration may be important to help prevent decrements in pulmonary function and serious long-term complications in EA ± TEF patients.
Zhang, G Y; Tang, Y; Niu, N N; Wu, H T
Objective: To investigate the clinical value of acoustic radiation force impulse (ARFI)technique in predicting esophageal and gastric varices in patients with biliary atresia after Kasai portoenterostomy. Methods: A total of 42 patients with biliary atresia after Kasai portoenterostomy were collected from September 2015 to May 2016 in Tianjin First Central Hospital.ARFI technique was used to measure the stiffness of liver and spleen, and 28 healthy children as control.According to the result of CT examination , patients with biliary atresia were divided into two groups , twenty-three patients with esophageal and gastric varices(A group) and nineteen patients without esophageal and gastric varices (B group), Comparing the difference of liver and spleen stiffness between the two groups.The ROC curve analysis was carried out to test the diagnostic power of effective parameter. Results: The ARFI value of liver (2.98±0.80) m/s and spleen (3.00±0.33) m/s of patients with biliary atresia was significantly higher than that of control group((1.10±0.16) m/s, (2.12±0.32) m/s), the differences had statistical significance (both P<0.01). Between group A and group B, the spleen ARFI value of group A(3.16±0.26) m/s was higher than group B(2.83±0.32) m/s, the difference had statistical significance (P<0.01), whereas there was no statistical difference of liver ARFI value between two group((2.93±0.65), (3.02±0.96) m/s)(P>0.05). The cut-off ARFI value of spleen to diagnose esophageal and gastric varices in biliary atresia was 3.02 m/s, and the biggest area under the ROC curve, sensitivity, and specificity were 0.81, 78.6% and 84.5%, respectively. Conclusion: ARFI can be used as a noninvasive method to predict the presence of esophageal and gastric varices in patients with biliary atresia after Kasai portoenterostomy.
Yalcin, Sule; Demir, Numan; Serel, Selen; Soyer, Tutku; Tanyel, F Cahit
A retrospective study was performed to evaluate the functional disorders of deglutition with videofluoroscopy (VFS), in children operated for esophageal atresia (EA) and/or tracheoesophageal fistula (TEF). Patients with the repair of EA-TEF were evaluated in respect to the type of malformation, operative procedure, postoperative complications, deglutitive and respiratory symptoms, and gastroesophageal reflux disease (GERD). The dysphagia score, VFS findings of oral, pharyngeal and esophageal phases, and penetration-aspiration scale (PAS) score were recorded in the evaluation of the deglutitive functions. Thirty-two cases with a median age of 48months (2-120months), and male to female ratio of 14:18 were included in the study. Most of the cases had Gross type C anomaly (n=26, 81.3%), and the others were type A (n=3), D (n=2) and E (n=1). The incidence of associated anomalies was 71.8%. The patients underwent primary (n=26, 81.3%) or delayed (n=6, 18.7%) anastomosis. Postoperative complications including anastomotic stricture (n=12), leak (n=2) and recurrent fistula (n=2) were managed by dilatation, conservative approach and repair of the fistula, respectively. Recurrent pneumonia (n=13), cough with liquid intake (n=10) and food impaction (n=7) were recorded in the history. Management of GERD included medical (n=11) and surgical (n=7) treatment. The median dysphagia score was 3.5 (min: 0-max: 27). The oral phase of VFS was normal in most of the cases (n=29, 90.6%). Only three had mild or moderate impairment, and none had severe. The pharyngeal phase showed no impairment in 23 of the cases (71.8%), and severe impairment was observed only in 3 of all, for the parameters of hyolaryngeal elevation and airway closure. Opposite to the first two phases of the deglutition, the esophageal phase was normal in only 2 of the cases (6.3%). Among the other 30 cases with impairment, only two had mild, and the rest had moderate to severe problems. Esophageal backflow, motility and
Vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association with Mayer-Rokitansky-Küster-Hauser syndrome in co-occurrence: two case reports and a review of the literature.
Bjørsum-Meyer, Thomas; Herlin, Morten; Qvist, Niels; Petersen, Michael B
The vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome are rare conditions. We aimed to present two cases with the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser co-occurrence from our local surgical center and through a systematic literature search detect published cases. Furthermore, we aimed to collect existing knowledge in the embryopathogenesis and genetics in order to discuss a possible link between the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome. Our first case was a white girl delivered by caesarean section at 37 weeks of gestation; our second case was a white girl born at a gestational age of 40 weeks. A co-occurrence of vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome was diagnosed in both cases. We performed a systematic literature search in PubMed ((VACTERL) OR (VATER)) AND ((MRKH) OR (Mayer-Rokitansky-Küster-Hauser) OR (mullerian agenesis) OR (mullerian aplasia) OR (MURCS)) without limitations. A similar search was performed in Embase and the Cochrane library. We added two cases from our local center. All cases (n = 9) presented with anal atresia and renal defect. Vertebral defects were present in eight patients. Rectovestibular fistula was confirmed in seven patients. Along with the uterovaginal agenesis, fallopian tube aplasia appeared in five of nine cases and in two cases ovarian involvement also existed. The co-occurrence of the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal
Baird, R; Levesque, D; Birnbaum, R; Ramsay, M
While many long-term complications of esophageal atresia (EA) have been well investigated, little is known about feeding difficulties in children after surgical correction of EA and its impact on caregivers. This study investigates the feeding behaviors of children with EA through a validated feeding questionnaire. The Montreal Children's Hospital Feeding Scale (MCH-FS) was filled out by the primary caregiver during patient follow-up visits in the multidisciplinary EA clinic. Demographic information, EA subtype, associated anomalies and outcomes were recorded. Results were compared between groups and to a normative sample. Thirty caregivers have completed the MCH-FS; 26 patients had type C atresia (86.7%). In comparison to controls, 17.5% of EA cases are one standard deviation above the mean feeding difficulty score, while 6.7% (n = 2) cases are greater than two standard deviations above normative values. Typical EA patients (type C who were not born <30 weeks) had mean MCH-FS scores in the subclinical range, whereas one extremely premature child and the patients with non-type C EA (n = 4) all had scores in the severe range. Feeding difficulties of patients with typical EA appear mild. Likely explanations include the use of early protocolized care and intensive multidisciplinary care in follow up. Nonetheless, patients with complicated EA (non-type C) and their caregivers tend to experience significant feeding difficulties. Early targeted care may be required for this patient subset, and additional cases will be investigated to confirm these preliminary findings and explore further risk factors of feeding problem in this cohort.
Demikova, Nataliya S; Vydrych, Yulia V; Podolnaya, Marina A; Lapina, Aleksandra S; Asanov, Aliy Yu
This study examined the prevalence of esophageal atresia (EA) and the relationship between EA and demographic factors in the Russian Federation. Data were obtained from a population-based congenital malformations registry across 14 years (2000-2013) in 24 regions of the Russian Federation and included cases of EA among live births and stillbirths. The total number of births was 6,478,706. There were 1317 cases of isolated EA, resulting in a rate of 2.03 (95% confidence interval [CI], 1.92-2.15) per 10,000 births or 1 case per 4926 births. There were differences in the prevalence of EA among regional registries of the Russian Federation. The prevalence of EA during the study period was stable. 57.3% of all cases were cases of EA with tracheo-esophageal fistula (compared with 42.7% of cases without fistula). The male/female sex ratio was 1.3. The relative risk of EA was higher for live births with birth weight less than 3000 g (relative risk [RR] = 2.58 (95% CI, 2.36-2.82), for older maternal age (RR = 1.47 (95% CI, 1.24-1.75), for males (RR = 1.09; 95% CI, 1.03-1.17), and for the first gravidity (RR = 1.17; 95% CI, 1.09-1.25). In this study, the prevalence of EA across different regions of the Russian Federation was analyzed. The prevalence of EA in the period under study remained stable, and the relative risk of EA was associated with maternal age, birth weight and gravidity. Birth Defects Research (Part A) 106:854-859, 2016. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.
Fragoso, Ana Catarina; Martinez, Leopoldo; Estevão-Costa, José; Tovar, Juan A
Abnormal lung development was recently described in the rat model of esophageal atresia and tracheoesophageal fistula (EA-TEF). Since in this condition the ventral-to-dorsal switch of Shh expression in the foregut is disturbed, the present study tested the hypothesis that this abnormal expression at the emergence of the tracheobronchial bud might be translated into the developing lung. Pregnant rats received either 1.75 mg/kg i.p. adriamycin or vehicle from E7 to E9. Three groups were studied: control and adriamycin-exposed with and without EA-TEF. Embryos were recovered and the lungs were harvested and processed for reverse transcription polymerase chain reaction and immunofluorescence analysis of the Shh signaling cascade. Shh signaling was downregulated at the late embryonic stage of lung development (E13) in embryos with EA-TEF. Throughout the subsequent stages of development, the expression of both Shh and its downstream components increased significantly and remained upregulated throughout gestation. Immunofluorescent localization was consistent with these findings. Defective Shh signaling environment in the foregut is present beyond the emergence of lung buds and probably impairs lung development. Later in gestation, lungs exhibited a remarkable ability to upregulate the Shh cascade, suggesting a compensatory response. These findings may be relevant to understand pulmonary disease suffered by children with EA-TEF.
Brosens, Erwin; de Jong, Elisabeth M; Barakat, Tahsin Stefan; Eussen, Bert H; D'haene, Barbara; De Baere, Elfride; Verdin, Hannah; Poddighe, Pino J; Galjaard, Robert-Jan; Gribnau, Joost; Brooks, Alice S; Tibboel, Dick; de Klein, Annelies
Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) is a relatively common birth defect often associated with additional congenital anomalies such as vertebral, anal, cardiovascular, renal and limb defects, the so-called VACTERL association. Yet, little is known about the causal genetic factors. Rare case reports of gastrointestinal anomalies in children with triple X syndrome prompted us to survey the incidence of structural and numerical changes of chromosome X in patients with EA/TEF. All available (n=269) karyotypes of our large (321) EA/TEF patient cohort were evaluated for X-chromosome anomalies. If sufficient DNA material was available, we determined genome-wide copy number profiles with SNP array and identified subtelomeric aberrations on the difficult to profile PAR1 region using telomere-multiplex ligation-dependent probe amplification. In addition, we investigated X-chromosome inactivation (XCI) patterns and mode of inheritance of detected aberrations in selected patients. Three EA/TEF patients had an additional maternally inherited X chromosome. These three female patients had normal random XCI patterns. Two male EA/TEF patients had small inherited duplications of the XY-linked SHOX (Short stature HOmeoboX-containing) locus. Patients were small for gestational age at birth (
Brosens, Erwin; de Jong, Elisabeth M; Barakat, Tahsin Stefan; Eussen, Bert H; D'haene, Barbara; De Baere, Elfride; Verdin, Hannah; Poddighe, Pino J; Galjaard, Robert-Jan; Gribnau, Joost; Brooks, Alice S; Tibboel, Dick; de Klein, Annelies
Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) is a relatively common birth defect often associated with additional congenital anomalies such as vertebral, anal, cardiovascular, renal and limb defects, the so-called VACTERL association. Yet, little is known about the causal genetic factors. Rare case reports of gastrointestinal anomalies in children with triple X syndrome prompted us to survey the incidence of structural and numerical changes of chromosome X in patients with EA/TEF. All available (n=269) karyotypes of our large (321) EA/TEF patient cohort were evaluated for X-chromosome anomalies. If sufficient DNA material was available, we determined genome-wide copy number profiles with SNP array and identified subtelomeric aberrations on the difficult to profile PAR1 region using telomere-multiplex ligation-dependent probe amplification. In addition, we investigated X-chromosome inactivation (XCI) patterns and mode of inheritance of detected aberrations in selected patients. Three EA/TEF patients had an additional maternally inherited X chromosome. These three female patients had normal random XCI patterns. Two male EA/TEF patients had small inherited duplications of the XY-linked SHOX (Short stature HOmeoboX-containing) locus. Patients were small for gestational age at birth (
Nassar, Natasha; Leoncini, Emanuele; Amar, Emmanuelle; Arteaga-Vázquez, Jazmín; Bakker, Marian K.; Bower, Carol; Canfield, Mark A.; Castilla, Eduardo E.; Cocchi, Guido; Correa, Adolfo; Csáky-Szunyogh, Melinda; Feldkamp, Marcia L.; Khoshnood, Babak; Landau, Danielle; Lelong, Nathalie; López-Camelo, Jorge S.; Lowry, R. Brian; McDonnell, Robert; Merlob, Paul; Métneki, Julia; Morgan, Margery; Mutchinick, Osvaldo M.; Palmer, Miland N.; Rissmann, Anke; Siffel, Csaba; Sìpek, Antonin; Szabova, Elena; Tucker, David; Mastroiacovo, Pierpaolo
BACKGROUND The prevalence of esophageal atresia (EA) has been shown to vary across different geographical settings. Investigation of geographical differences may provide an insight into the underlying etiology of EA. METHODS The study population comprised infants diagnosed with EA during 1998 to 2007 from 18 of the 46 birth defects surveillance programs, members of the International Clearinghouse for Birth Defects Surveillance and Research. Total prevalence per 10,000 births for EA was defined as the total number of cases in live births, stillbirths, and elective termination of pregnancy for fetal anomaly (ETOPFA) divided by the total number of all births in the population. RESULTS Among the participating programs, a total of 2943 cases of EA were diagnosed with an average prevalence of 2.44 (95% confidence interval [CI], 2.35–2.53) per 10,000 births, ranging between 1.77 and 3.68 per 10,000 births. Of all infants diagnosed with EA, 2761 (93.8%) were live births, 82 (2.8%) stillbirths, 89 (3.0%) ETOPFA, and 11 (0.4%) had unknown outcomes. The majority of cases (2020, 68.6%), had a reported EA with fistula, 749 (25.5%) were without fistula, and 174 (5.9%) were registered with an unspecified code. CONCLUSIONS On average, EA affected 1 in 4099 births (95% CI, 1 in 3954–4251 births) with prevalence varying across different geographical settings, but relatively consistent over time and comparable between surveillance programs. Findings suggest that differences in the prevalence observed among programs are likely to be attributable to variability in population ethnic compositions or issues in reporting or registration procedures of EA, rather than a real risk occurrence difference. PMID:22945024
Background Mutations in EFTUD2 were proven to cause a very distinct mandibulofacial dysostosis type Guion-Almeida (MFDGA, OMIM #610536). Recently, gross deletions and mutations in EFTUD2 were determined to cause syndromic esophageal atresia (EA), as well. We set forth to find further conditions caused by mutations in the EFTUD2 gene (OMIM *603892). Methods and results We performed exome sequencing in two familial cases with clinical features overlapping with MFDGA and EA, but which were previously assumed to represent distinct entities, a syndrome with esophageal atresia, hypoplasia of zygomatic complex, microcephaly, cup-shaped ears, congenital heart defect, and intellectual disability in a mother and her two children [AJMG 143A(11):1135-1142, 2007] and a supposedly autosomal recessive oto-facial syndrome with midline malformations in two sisters [AJMG 132(4):398-401, 2005]. While the analysis of our exome data was in progress, a recent publication made EFTUD2 mutations highly likely in these families. This hypothesis could be confirmed with exome as well as with Sanger sequencing. Also, in three further sporadic patients, clinically overlapping to these two families, de novo mutations within EFTUD2 were identified by Sanger sequencing. Our clinical and molecular workup of the patients discloses a broad phenotypic spectrum, and describes for the first time an instance of germline mosaicism for an EFTUD2 mutation. Conclusions The clinical features of the eight patients described here further broaden the phenotypic spectrum caused by EFTUD2 mutations or deletions. We here show, that it not only includes mandibulofacial dysostosis type Guion-Almeida, which should be reclassified as an acrofacial dysostosis because of thumb anomalies (present in 12/35 or 34% of patients) and syndromic esophageal atresia [JMG 49(12). 737-746, 2012], but also the two new syndromes, namely oto-facial syndrome with midline malformations published by Mégarbané et al. [AJMG 132(4): 398
Williams, A K; Qi, B Q; Beasley, S W
The notochord (Nt) is believed to have a role in the development of axial organs. This study was undertaken to reconstruct in three dimensions (3D) the relationship of the Nt to abnormal development of the foregut (Fg) in the adriamycin-induced rat model of esophageal atresia (EA). Pregnant Sprague-Dawley rats were given 1.75 mg/kg adriamycin intraperitoneally on gestational days 6 9 inclusive; control rats received i.p. saline of equal volume, or no injection. Rats were killed between days 11 and 14 and their embryos harvested, histologically sectioned serially, and stained with hematoxylin and eosin. Digitized photographs were taken of serial transverse sections; these photos were traced and used as the basis for 3D reconstruction. From day 11 the normal Nt is no longer in contact with the respiratory or Fg mesenchyme. In adriamycin-treated embryos the Nt branches abnormally as it enters the Fg mesenchyme. Adherence of the Nt to the mesenchyme of the Fg exerts mechanical traction pulling the upper Fg dorsally. The severity of the Fg abnormalities correlates with the length of the ventral extension of the Nt within the Fg mesenchyme: the embryo develops atresia of the esophagus or trachea when the Nt is grossly abnormal. The Nt undergoes reactive thickening in the absence of Fg structures ventral to it. Thus, structural lesions of the Fg (e.g., atresias) are associated with abnormalities of the Nt. The relationship of the Nt to the Fg mesenchyme determines the severity of the abnormality induced by adriamycin: extensive adherence produces tracheal agenesis and EA.
Petrosyan, Mikael; Estrada, Joaquin; Hunter, Catherine; Woo, Russell; Stein, James; Ford, Henri R; Anselmo, Dean M
The surgical management of esophageal atresia with distal tracheoesophageal fistula (EA/TEF) involves early division of the TEF and primary esophageal anastomosis. However, in premature infants, the morbidity associated with primary repair remains high, and the optimal surgical approach has not been well defined. Medical records of very low-birth-weight infants (<1500 g) with EA/TEF from June 1987 to 2008 were retrospectively reviewed. Patients were separated into 2 groups: (1) primary repair and (2) ligation and division of TEF followed by delayed repair of EA. Demographics, anastomotic, and postoperative complications were compared. Twenty-five premature infants with EA/TEF were identified. Sixteen patients (64%) underwent primary repair, and 9 (36%) were repaired in a staged manner. The leak rate confirmed by esophagram was significantly higher after primary repair (50%) compared to staged repair (0%) (P = .034). Strictures occurred significantly more often in the primary repair (81%) vs the staged repair (33%) group (P = .036). Postoperative pneumonia and sepsis were significantly higher in patients treated with primary repair (P = .028). Staged repair of EA/TEF in very low-birth-weight premature infants results in a significantly lower rate of anastomotic complications and overall morbidity and should be considered the preferred surgical approach in this group of patients.
Smigiel, R; Lebioda, A; Blaszczyński, M; Korecka, K; Czauderna, P; Korlacki, W; Jakubiak, A; Bednarczyk, D; Maciejewski, H; Wizinska, P; Sasiadek, M M; Patkowski, D
Esophageal atresia (EA) is a congenital defect of the esophagus involving the interruption of the esophagus with or without connection to the trachea (tracheoesophageal fistula [TEF]). EA/TEF may occur as an isolated anomaly, may be part of a complex of congenital defects (syndromic), or may develop within the context of a known syndrome or association. The molecular mechanisms underlying the development of EA are poorly understood. It is supposed that a combination of multigenic factors and epigenetic modification of genes play a role in its etiology. The aim of our work was to assess the human gene expression microarray study in esophageal tissue samples. Total RNA was extracted from 26 lower pouches of esophageal tissue collected during thoracoscopic EA repair in neonates with the isolated (IEA) and the syndromic form (SEA). We identified 787 downregulated and 841 upregulated transcripts between SEA and controls, and about 817 downregulated and 765 upregulated probes between IEA and controls. Fifty percent of these genes showed differential expression specific for either IEA or SEA. Functional pathway analysis revealed substantial enrichment for Wnt and Sonic hedgehog, as well as cytokine and chemokine signaling pathways. Moreover, we performed reverse transcription polymerase chain reaction study in a group of SHH and Wnt pathways genes with differential expression in microarray profiling to confirm the microarray expression results. We verified the altered expression in SFRP2 gene from the Wnt pathway as well as SHH, GLI1, GLI2, and GLI3 from the Sonic hedgehog pathway. The results suggest an important role of these pathways and genes for EA/TEF etiology. © 2014 International Society for Diseases of the Esophagus.
Tri atresia; Valve disorder - tricuspid atresia; Congenital heart - tricuspid atresia; Cyanotic heart disease - tricuspid atresia ... Tricuspid atresia is an uncommon form of congenital heart disease. It affects about 5 in every 100, ...
R A A, Hassan; Y U, Choo; R, Noraida; I, Rosida
Development of infantile hypertrophic pyloric stenosis during postoperative period in EA with TEF is rare. Postoperative vomiting or feeding intolerance in EA is more common which is due to esophageal stricture, gastroesophageal reflux and esophageal dysmotility. A typical case of IHPS also presents with non-bilious projectile vomiting at around 3-4 weeks of life. The diagnosis of infantile hypertrophic pyloric stenosis in this subset is usually delayed because of its rarity. We report a case of IHPS in postoperative EA and emphasize on high index of suspicion to avoid any delay in diagnosis with its metabolic consequences.
Lepeytre, C; De Lagausie, P; Merrot, T; Baumstarck, K; Oudyi, M; Dubus, J-C
The aim of this study was to evaluate the medium-term outcome (health status, medical and surgical French National Health Authority-recommended follow-up, and quality of life) of children born with type III esophageal atresia (EA). Previous events (during the perinatal period, associated abnormalities, respiratory and digestive complications) of children treated for type III EA at the Marseille university hospitals between 1999 and 2009 were noted. Parents completed a standardized questionnaire concerning the health of their children during the previous year, and a quality-of-life questionnaire (PedsQL 4.0) was also completed by children aged more than 8 years. Among the 68 children treated, 44 responded to our solicitation (mean age, 7.6 years; range, 3-12.8 years). Previous important events were : pneumonia(s) (65%), asthma before the age of 3 years (66%), hospitalization for a respiratory event (45%), fundoplication (20%), and esophageal dilatation (45%). We noted current chronic cough (16%), asthma (30%), dysphagia (39%), and symptomatic gastroesophageal reflux (9%). National guidelines were not respected, except for the surgical indications in children aged less than 6 years. The quality-of-life scores (n=43 children) were similar to healthy controls but were negatively influenced by a gastrostomy procedure (P=0.020), pneumonia (P=0.013), and hospitalization due to a respiratory event (P=0.006) or a digestive event (P=0.010), and also by current asthma (P=0.004). In conclusion, despite recurrent respiratory or digestive symptoms and inadequate recommended follow-up, the quality of life of children treated for type III of EA is good. Copyright © 2013. Published by Elsevier SAS.
Tong, Stanley; Mallitt, Kylie-Ann; Krishnan, Usha
Background Gastroesophageal reflux disease (GERD) and esophageal dysmotility are common in patients with esophageal atresia (EA). The aim of this study was to evaluate GERD and esophageal motility patterns in children with EA using combined multichannel intraluminal impedance and pH (MII-pH) monitoring and high-resolution esophageal manometry (HREM), respectively. The reflux patterns seen in EA patients were also compared with a control group of normal children with suspected GERD. Methods A retrospective chart review was done on 35 patients with EA and 35 age- and sex-matched normal controls with suspected GERD, who had undergone 24-hour MII-pH monitoring. Impedance data were compared between both cohorts. Eight of the EA patients also underwent HREM. Results In the EA cohort, the median age was 53 months, with 21 males, and 71.4% had Type C EA. A total of 85.7% of the EA cohort and 40% of the control group were on proton-pump inhibitor (PPI) therapy during the MII-pH study. There was no significant difference in the total retrograde bolus movements (RBMs) between the EA cohort (1,457) and the control group (1,482). Acidic RBMs was significantly lower in the EA group (208) compared with the control group (689), p = 0.0008. Nonacid reflux index (NARI) was significantly higher in EA children (1.1; 0.0-7.8) compared with controls (0.6; 0.0-5.7), p = 0.0046. In EA patients, only 335/1,183 (28%) total symptom occurrences were associated with RBM. The mean distal baseline impedance (DBI) was significantly lower in EA (1,029.6 [410.9 SD] Ω) compared with controls (2,998.2 [1028.8 SD] Ω) with suspected GERD, p < 0.0001. By logistic regression, only PPI use had a significant effect on DBI, p < 0.0001. HREM was abnormal in all eight EA patients. Four out of eight EA patients had a different peristaltic pattern for their solid swallows compared with their liquid swallows in HREM. Conclusions MII-pH testing allowed increased detection of nonacid
Garabedian, C; Sfeir, R; Langlois, C; Bonnard, A; Khen-Dunlop, N; Gelas, T; Michaud, L; Auber, F; Piolat, C; Lemelle, J-L; Fouquet, V; Habonima, É; Becmeur, F; Polimerol, M-L; Breton, A; Petit, T; Podevin, G; Lavrand, F; Allal, H; Lopez, M; Elbaz, F; Merrot, T; Michel, J-L; Buisson, P; Sapin, E; Delagausie, P; Pelatan, C; Gaudin, J; Weil, D; de Vries, P; Jaby, O; Lardy, H; Aubert, D; Borderon, C; Fourcade, L; Geiss, S; Breaud, J; Pouzac, M; Echaieb, A; Laplace, C; Gottrand, F; Houfflin-Debarge, V
Evaluate neonatal management and outcome of neonates with either a prenatal or a post-natal diagnosis of EA type III. Population-based study using data from the French National Register for EA from 2008 to 2010. We compared children with prenatal versus post-natal diagnosis in regards to prenatal, maternal and neonatal characteristics. We define a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and mortality at 1 year. Four hundred and eight live births with EA type III were recorded with a prenatal diagnosis rate of 18.1%. Transfer after birth was lower in prenatal subset (32.4% versus 81.5%, P<0.001). Delay between birth and first intervention was not significantly different. Defect size (2cm vs 1.4cm, P<0.001), gastrostomy (21.6% versus 8.7%, P<0.001) and length in neonatal unit care were higher in prenatal subset (47.9 days versus 33.6 days, P<0.001). The composite variables were higher in prenatal diagnosis subset (38.7% vs 26.1%, P=0.044). Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity related to the EA type (longer gap). Even if it does not modify neonatal management and 1-year outcome, prenatal diagnosis allows antenatal parental counseling and avoids post-natal transfer. Copyright © 2014 Elsevier Masson SAS. All rights reserved.
Gallo, Gabriele; Vrijlandt, Elianne J L E; Arets, Hubertus G M; Koppelman, Gerard H; Van der Zee, David C; Hulscher, Jan B F; Zwaveling, Sander
Children born with esophageal atresia require an anastomosis between the proximal and distal esophagus. When this distance is too wide (long gap esophageal atresia, LGEA) esophageal replacement strategies have to be deployed. The aim of this study was to assess long-term respiratory morbidity and lung function after esophageal replacement with either stomach (gastric pull-up, GPU) or jejunum (jejunal interposition, JI) for LGEA. Retrospective cohort study. Patients operated with GPU and JI for LGEA (1985-2007) underwent a semi-structured interview and lung function testing (LFT). Seven GPU-patients and eight JI-patients were included. Median age was 12years. One patient per group could not perform LFT. Respiratory symptoms were reported by 13/15 patients (7/7 GPU-patients vs 6/8 JI-patients). All LFT items were lower than reference values; 6/13 patients showed restriction and 6/13 obstruction. All six GPU-patients had abnormal TLC and/or FEV1/FVC vs 3/7 after JI. Restriction was noted in 4/6 GPU-patients vs 2/7 JI-patients. After esophageal replacement for LGEA many children have impaired lung function and respiratory symptoms are common. Lung volumes seem decreased after GPU compared to JI. This may be caused by the intrathoracic stomach which may limit normal lung growth. Respiratory follow-up in adult life is important after esophageal replacement. III. Copyright © 2017 Elsevier Inc. All rights reserved.
Dingemann, Jens; Szczepanski, Rüdiger; Ernst, Gundula; Thyen, Ute; Ure, Benno; Goll, Melanie; Menrath, Ingo
Aim of the Study A history of esophageal atresia (EA) may result in chronic morbidity. The transition of patients from pediatric to adult care has been recognized as an important factor to maintain disease-specific follow-up and prevent exacerbation of chronic disease. Patient education is recognized as a necessary part of transition programs for children with chronic diseases. Structured education programs for patients with EA have not yet been developed. We aimed to evaluate the efficacy of a transition-specific educational program in adolescents with a history of EA. Methods An ethical approval was obtained. Patients with a history of EA (age 14-21 years) and their parents were invited to participate in a 2-day transition-specific educational program. Overall, 29 patients and 25 parents were recruited out of whom 10/7 were allocated to the intervention group (program) and 19/18 to the control group (no program). Subjective satisfaction (ZUF-8) and expected effects of the program on the future course of the disease, transition-specific knowledge (standardized questionnaire addressing organizational and health-related aspects of transition), health-related quality of life (DISABKIDS), and confidence for self-management (Patient Activation Measure-13) were measured with appropriate psychological instruments. Nonparametric tests were used for statistical analysis. Main Results Subjects participating were highly satisfied with the program (patients 26/32 points, parents 25/32; ZUF-8). Overall, 90% of the patients and 67% of the parents anticipated a positive effect on the future course of the disease. Patient's transition-specific knowledge was low before the program (36% correct answers). It improved by 18% after the intervention (56% correct answers; p = 0.004). It did not change in the control group (54 vs. 52%; n.s.). Parent's transition-specific knowledge did not change after the intervention (66 vs. 67% correct answers; ns). In patients, there were no
Zwink, N; Choinitzki, V; Baudisch, F; Hölscher, A; Boemers, T M; Turial, S; Kurz, R; Heydweiller, A; Keppler, K; Müller, A; Bagci, S; Pauly, M; Brokmeier, U; Leutner, A; Degenhardt, P; Schmiedeke, E; Märzheuser, S; Grasshoff-Derr, S; Holland-Cunz, S; Palta, M; Schäfer, M; Ure, B M; Lacher, M; Nöthen, M M; Schumacher, J; Jenetzky, E; Reutter, H
Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) and anorectal malformations (ARM) represent the severe ends of the fore- and hindgut malformation spectra. Previous research suggests that environmental factors are implicated in their etiology. These risk factors might indicate the influence of specific etiological mechanisms on distinct developmental processes (e.g. fore- vs. hindgut malformation). The present study compared environmental factors in patients with isolated EA/TEF, isolated ARM, and the combined phenotype during the periconceptional period and the first trimester of pregnancy in order to investigate the hypothesis that fore- and hindgut malformations involve differing environmental factors. Patients with isolated EA/TEF (n = 98), isolated ARM (n = 123), and the combined phenotype (n = 42) were included. Families were recruited within the context of two German multicenter studies of the genetic and environmental causes of EA/TEF (great consortium) and ARM (CURE-Net). Exposures of interest were ascertained using an epidemiological questionnaire. Chi-square, Fisher's exact, and Mann-Whitney U-tests were used to assess differences between the three phenotypes. Newborns with isolated EA/TEF and the combined phenotype had significantly lower birth weights than newborns with isolated ARM (P = 0.001 and P < 0.0001, respectively). Mothers of isolated EA/TEF consumed more alcohol periconceptional (80%) than mothers of isolated ARM or the combined phenotype (each 67%). Parental smoking (P = 0.003) and artificial reproductive techniques (P = 0.03) were associated with isolated ARM. Unexpectedly, maternal periconceptional multivitamin supplementation was most frequent among patients with the most severe form of disorder, i.e. the combined phenotype (19%). Significant differences in birth weight were apparent between the three phenotype groups. This might be attributable to the limited ability of EA/TEF fetuses to swallow amniotic fluid, thus
Barsness, Katherine A; Rooney, Deborah M; Davis, Lauren M; O'Brien, Ellie
Thoracoscopic esophageal atresia (EA)/tracheoesophageal fistula (TEF) repair is technically challenging. We have previously reported our experiences with a high-fidelity hybrid model for simulation-based educational instruction in thoracoscopic EA/TEF, including the high cost of the tissue for these models. The purposes of this study were (1) to create a low-cost synthetic tissue EA/TEF repair simulation model and (2) to evaluate the content validity of the synthetic tissue simulator. Review of the literature and computed tomography images were used to create computer-aided drawings (CAD) for a synthetic, size-appropriate EA/TEF tissue insert. The inverse of the CAD image was then printed in six different sections to create a mold that could be filled with platinum-cured silicone. The silicone EA/TEF insert was then placed in a previously described neonatal thorax and covered with synthetic skin. Following institutional review board-exempt determination, 47 participants performed some or all of a simulated thoracoscopic EA/TEF during two separate international meetings (International Pediatric Endosurgery Group [IPEG] and World Federation of Associations of Pediatric Surgeons [WOFAPS]). Participants were identified as "experts," having 6-50 self-reported thoracoscopic EA/TEF repairs, and "novice," having 0-5 self-reported thoracoscopic EA/TEF repairs. Participants completed a self-report, six-domain, 24-item instrument consisting of 23 5-point rating scales and one 4-point Global Rating Scale. Validity evidence relevant to test content and response processes was evaluated using the many-facet Rasch model, and evidence of internal structure (interitem consistency) was estimated using Cronbach's alpha. A review of the participants' ratings indicates there were no overall differences across sites (IPEG versus WOFAPS, P=.84) or experience (expert versus novice, P=.17). The highest observed averages were 4.4 (Value of Simulator as a Training Tool), 4.3 (Physical
Connor, Martin J; Springford, Laurie R; Kapetanakis, Venediktos V; Giuliani, Stefano
Esophageal atresia (EA) is a rare congenital anomaly with high infantile survival rates. The aim of this study was to outline the prevalence of common long-term problems associated with EA repair in patients older than 10 years of age. Original papers were identified by systematic searching of MEDLINE and EMBASE databases from January 1993 to July 2014. Fifteen articles (907 EA patients) met inclusion criteria. This is the first systematic review aiming to quantify the prevalence of the long-term problems associated with EA. The main active medical conditions (pooled estimated prevalence) identified were the following: dysphagia (50.3%), gastroesophageal reflux disease with (40.2%) or without (56.5%) histological esophagitis, recurrent respiratory tract infections (24.1%), doctor-diagnosed asthma (22.3%), persistent cough (14.6%), and wheeze (34.7%). The prevalence of Barrett's esophagus (6.4%) was 4 and 26 times higher than the adult (1.6%) and pediatric (.25%) general populations. Adult and pediatric practitioners should focus on how to develop effective long-term follow-up and transitional care for these patients. Copyright © 2015 Elsevier Inc. All rights reserved.
... would. Many infants with duodenal atresia also have Down syndrome. Duodenal atresia is often associated with other birth ... abnormality. Other problems (such as those related to Down syndrome) must be treated as appropriate. Outlook (Prognosis) Recovery ...
Kuppan, Purushothaman; Sethuraman, Swaminathan; Krishnan, Uma Maheswari
The diseases of the esophagus include congenital defects like atresia, tracheoesophageal fistula as well as others such as gastro-esophageal reflux disease (GERD), Barrett's esophagus, carcinoma and strictures. All esophageal disorders require surgical intervention and reconstruction with appropriate substitutes. Primary anastomosis is used to treat most cases but treatment of long gap atresia still remains a clinical challenge. Autologous graft therapies using tissues from colon, and small and large intestine or gastric transplantations have been attempted but have constraints like leakage, infection and stenosis at the implanted site, which leads to severe morbidity and mortality. An alternative for autologous grafts are allogenic and xenogenic grafts, which have better availability but disease transmission and immunogenicity limit their applications. Use of biodegradable and biocompatible scaffolds to engineer the esophagus promises to be an effective regenerative strategy for treatment of esophageal disorders. Nanotopography of the fibrous scaffolds mimics the natural extracellular matrix (ECM) of the tissue and incorporation of chemical cues and tailoring mechanical properties provide the right microenvironment for co-culture of different cell types. Scaffolds cultured with esophageal cells (epithelial cells, fibroblast and smooth muscle cells) might show enhancement of the biofunctionality in vivo. This review attempts to address the various strategies and challenges involved in successful tissue engineering of the esophagus. Copyright © 2012 Elsevier Inc. All rights reserved.
... blood flow from the right ventricle (right side pumping chamber) to the lungs. In pulmonary atresia, a ... Reconstructing the heart as a single ventricle (1 pumping chamber instead of 2) Heart transplant Outlook (Prognosis) ...
Anorectal malformation (ARM) is one of the most common congenital anomaly that requires emergency surgery in the neonatal period. ARMs are frequently associated with other life threatening congenital anomalies. Commonly associated anomalies are genito-urinary, cardiovascular, gastro-intestinal, skeletal and spinal. Alimentary tract anomalies are frequently masked by the intestinal obstruction produced by the anorectal atresia. This retrospective study was carried out to find out the incidence of associated alimentary tract atresias with ARM. In our series, out of 785 cases of high ARM, 14 cases had associated esophageal atresia (1.8%), followed by 7 cases of duodenal atresia (0.89%), and followed by pyloric atresia, jejuno-ileal atresia and colonic atresia. PMID:27896151
Maricic, Maximiliano Alejo; Bailez, María Marcela; Rodriguez, Susana P
We present the results of the validation of an inanimate model created for training thoracoscopic treatment of esophageal atresia with lower tracheoesophageal fistula (EA/TEF). We used different domestic materials such as a piece of wood (support), corrugated plastic tubes (PVC) of different sizes to simulate ribs, intercostal spaces, trachea and spine and tubular latex balloons to simulate the esophagus and lungs to make the basic model. This device was inserted into the thoracic cavity of a rubber dummy simulating a 3kg newborn with a work area volume of 50ml. The model was designed taking into account the experience of doing this procedure in neonates. The cost of the materials used was 50 US$. Regular video endoscopic equipment and 3mm instruments were used. Thirty-nine international faculty or pediatric surgeons attending hands on courses with different levels of training in minimal invasive surgery (MIS) repair of EA/TEF performed the procedure in the model. We compared the performance of the practitioners with their experience in thoracoscopic repair of EA. A Likert-type scale was used to evaluate results. Previous experience in MIS, anatomical appearance of the model, surgical anatomy compared to a real patient, and utility as a training method were analyzed. We also used a checklist to assess performance. We evaluated: number of errors and types of injuries, quality of the anastomosis, and duration of procedure. To analyze the results we used a T-test, chi-square test and Excel® database to match up some results. Thirty-nine questionnaires were completed. Seven surgeons were experts (≥30 TEF/EA repairs as surgeon), 10 had intermediate level of experience (5 to 29 repairs as surgeon) and 22 were beginners (less than 5 repairs). To simplify the analysis we divided the respondents into low experience LE (<5 real procedures-beginners; n=22) and high experience HE (intermediate, 10; and experts, 7; n=17). In relation to the anatomical characteristics of the
... tracheoesophageal fistula (TEF) are 2 disorders of the digestive system. They happen in babies before the babies are ... The main problems EA causes are with the digestive system. TEF usually presents itself through breathing problems. Symptoms ...
Parida, Lalit; Pal, Kamalesh; Buainain, Hussah A.; Al-Umran, Khalid U.
This report describes a successful outcome in a preterm baby with an esophageal atresia and tracheo-esophageal fistula, who initially underwent a primary esophageal repair; but a persistent nonexpanding lung on the side of surgery led to further investigations. A further diagnosis of an esophageal lung resulted in pneumonectomy and prophylactic placement of an intra-thoracic prosthesis to prevent post-pneumonectomy syndrome. To the best of our knowledge, this is the first report of a prophylactic placement of an intra-thoracic prosthesis in a neonate with the condition of esophageal atresia and tracheo-esophageal fistula and associated esophageal lung. PMID:25829674
Famulski, W; Sobaniec-Lotowska, M; Sulkowski, S; Ostapiuk, H; Kemona, A
Congenital biliary tract atresia was found in 0.58% of children dying in the first year of life. Most frequently atresia included the extrahepatic bile ducts, among them the common bile duct. Five cases (35.7%) had atresia associated with other developmental anomalies, which may suggest a genetic determination of the atresia. The most frequent cause of death of the newborns and infants with congenital biliary tract atresia was bronchopneumonia with associated biliary cirrhosis of the liver.
Bassett, Mikelle D.; Murray, Karen F.
Extrahepatic biliary atresia (EHBA), an inflammatory sclerosing cholangiopathy, is the leading indication for liver transplantation in children. The cause is still unknown, although possible infectious, genetic, and immunologic etiologies have received much recent focus. These theories are often dependent on each other for secondary or coexisting mechanisms. Concern for EHBA is raised by a cholestatic infant, but the differential diagnosis is large and the path to diagnosis remains varied. Current treatment is surgical with an overall survival rate of approximately 90%. The goals of this article are to review the important clinical aspects of EHBA and to highlight some of the more recent scientific and clinical developments contributing to our understanding of this condition. PMID:18496390
Lemoine, Caroline; Aspirot, Ann; Morris, Melanie; Faure, Christophe
After surgical correction of esophageal atresia with or without tracheoesophageal fistula, esophageal body motility dysfunction has been reported in nearly all patients. Using high-resolution esophageal manometry before surgical repair in 2 children with isolated tracheoesophageal fistula, we sought to determine whether dysmotility was present before any surgical insult to test the hypothesis that dysmotility associated with esophageal atresia with or without tracheoesophageal fistula is related to intrinsic primary factors linked to abnormal development of the esophagus. Both had an abnormal esophageal motility: one exhibited hypomotility with distal contraction, whereas the other showed a complete aperistalsis pattern. This suggests that esophageal dysmotility is congenital in nature rather than secondary to surgery.
Biliary atresia (BA) is a rare disease characterised by a biliary obstruction of unknown origin that presents in the neonatal period. It is the most frequent surgical cause of cholestatic jaundice in this age group. BA occurs in approximately 1/18,000 live births in Western Europe. In the world, the reported incidence varies from 5/100,000 to 32/100,000 live births, and is highest in Asia and the Pacific region. Females are affected slightly more often than males. The common histopathological picture is one of inflammatory damage to the intra- and extrahepatic bile ducts with sclerosis and narrowing or even obliteration of the biliary tree. Untreated, this condition leads to cirrhosis and death within the first years of life. BA is not known to be a hereditary condition. No primary medical treatment is relevant for the management of BA. Once BA suspected, surgical intervention (Kasai portoenterostomy) should be performed as soon as possible as operations performed early in life is more likely to be successful. Liver transplantation may be needed later if the Kasai operation fails to restore the biliary flow or if cirrhotic complications occur. At present, approximately 90% of BA patients survive and the majority have normal quality of life. PMID:16872500
Soyer, Tutku; Kalkışım, Said; Yalcin, Sule; Müderrisoğlu, Ahmet; Taş, Sadık Taşkın; Tanyel, Feridun Cahit; Ertunç, Mert; Sara, Yıldırım
In long-gap esophageal atresia surgeries, anastomoses can be tensioned by several traction methods in order to establish esophageal continuity. It is unclear whether the etiology of esophageal dysmotility after traction is related with esophageal atresia itself or tensioned esophagus. Therefore, we evaluated the effects of acute in vitro esophageal tension application on esophageal muscle contractility in rats. 26 Wistar rats weighing 250-300 g were included to the study. After diethyl ether anesthesia, proximal segment (PS) and distal segment (DS) of esophagus were removed and suspended in an isolated organ bath kept at 37°C, Krebs-Henseleit solution. Rats were enrolled into four groups including control group (CG, n=14) without tension, 5 g (5G, n=4), 15 g (15G, n=4) and 25 g (25G, n=4) tension groups. In all groups, contractile responses to electrical field stimulation (EFS), carbachol and KCl, and relaxation responses to serotonin were obtained. In CG, higher contractile responses were obtained in PS than DS after EFS. Both PS and DS showed higher contractile amplitudes in 5G with respect to that of CG, 15G and 25G (p<0.05). In 5G, contractile responses to carbachol were significantly increased in both PS and DS with respect to CG (p<0.05). However, contractile amplitudes in response to carbachol were decreased in PS when tension was increased to 15 g and 25 g. In DS, contractile responses in 15G and 25G were lower than 5G, and still higher than CG. Serotonin relaxation responses in PS were decreased when compared to CG at tension levels of 5 g, 15 g and 25 g (p<0.05). In DS, responses to serotonin were also decreased in tension groups. PS had higher contraction amplitudes than DS when contractile responses were obtained by high K(+) (p<0.05). Tension groups of both PS and DS showed increased contractions to high K(+) compared to CG (p<0.05). Increased esophageal tension led to increase in cholinergic responses of smooth muscles as well as in EFS
Bogs, Thomas; Zwink, Nadine; Chonitzki, Vera; Hölscher, Alice; Boemers, Thomas M; Münsterer, Oliver; Kurz, Ralf; Heydweiller, Andreas; Pauly, Marcus; Leutner, Andreas; Ure, Benno M; Lacher, Martin; Deffaa, Oliver Johannes; Thiele, Holger; Bagci, Soyhan; Jenetzky, Ekkehart; Schumacher, Johannes; Reutter, Heiko
Background Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) represents the most common developmental malformation of the upper digestive tract. It is classified into six subtypes according to the classification of Vogt, depending on anatomical variation of this malformation. Around 50% of the patients with EA/TEF present additional anomalies, which often influence, next to the EA/TEF subtype, the overall prognosis of EA/TEF newborns. Here, we investigated the association of the different EA/TEF subtypes with co-occurring congenital anomalies in EA/TEF patients and demonstrate their implications for postnatal diagnostic workup. Materials and Methods We investigated 333 patients of a large German multicenter study born between 1980 and 2012. After evaluation of all available clinical records, 235 patients were included in our analysis. We compared our results with existing data. Results The highest risk for co-occurring anomalies was seen in patients with most common Vogt 3b (p = 0.024), especially for additional gastrointestinal anomalies (p = 0.04). Co-occurring anomalies of the skin were significantly more common in patients with subtype Vogt 2 (p = 0.024). A significant correlation was observed for an impaired neurodevelopmental outcome and EA/TEF Vogt 3a (p = 0.041). Patients with EA/TEF showed a higher risk to present with any additional congenital anomaly compared with the general population (p < 0.001). Conclusion Our results warrant thorough clinical workup for gastrointestinal anomalies especially in patients with Vogt 3b. Moreover, it might be necessary to focus on a thorough aftercare for neurocognitive development in patients with Vogt 3a. The here presented observations need to be confirmed by future studies.
... the chest wall) to drain fluids from the space between the outside of the lung and the inside of the chest cavity. Intravenous (IV) fluids, including nutrition Oxygen Pain medicines as needed If both the TEF and EA ...
Ben Hamida, H.; Hadj Salem, R.; Ben Ameur, K.; Rassas, A.; Chioukh, FZ.; Sakka, R.; Kechiche, N.; Bizid, M.; Sahnoun, L.; Monastiri, K.
Duodenal atresia is rarely associated with situs inversus abdominus. We report a case of duodenal atresia associated with small bowel atresia of apple peel type and situs inversus abdominus. PMID:27896168
Youson, J H
The preceding pages have described an organism that is far removed from mammals on the taxonomic scale of vertebrates but one that has proven to have a unique and most useful system for studies of liver function and, in particular, bile product transport and excretion. It is also an organism in which iron loading can be studied in the liver and other organs and tissues. Many of the events that occur in this animal during its life cycle with regard to bile pigment metabolism as normal programmed phenomena are unparalleled among the vertebrates. In the larval (ammocoete) period of lampreys, there is an intrahepatic gallbladder and a biliary tree that is well equipped for the storage, transport, and elimination of bile products into the intestine for ultimate excretion with the feces. The importance of the patency of these bile ducts to bile excretion is illustrated in one species of lampreys in which the bile ducts of young ammocoetes become infested with larval nematodes to a degree that bile pigment regurgitation into the blood results in a green serum that is identified as biliverdin. Despite having serum levels of biliverdin that would be toxic to humans, these individuals live a complete larval life. The larvae of all lamprey species undergo a phase of metamorphosis in which they transform into adults. During this phase the larval gallbladder, the bile canaliculi of the hepatocytes, and all the intrahepatic bile ducts completely regress in a developmental process called lamprey biliary atresia. The epithelium of the extrahepatic common bile duct transforms and expands into a caudal portion of the endocrine pancreas of the adult. Many of the events of lamprey biliary atresia resemble events occurring during experimental and pathological conditions of mammalian cholestasis, including disruption to the bile-blood barrier (intercellular junctions), accumulation of bile components in the cytoplasmic inclusions, and alteration of the distribution of membrane enzymes
Esophageal varices Overview By Mayo Clinic Staff Esophageal varices are abnormal, enlarged veins in the tube that connects the ... often in people with serious liver diseases. Esophageal varices develop when normal blood flow to the liver ...
Ioannides, Adonis S.; Copp, Andrew J.
Oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) are important human birth defects of unknown aetiology. The embryogenesis of OA/TOF remains poorly understood mirroring the lack of clarity of the mechanisms of normal tracheo-oesophageal development. The development of rat and mouse models of OA/TOF has allowed the parallel study of both normal and abnormal embryogenesis. Although controversies persist, the fundamental morphogenetic process appears to be a rearrangement of the proximal foregut into separate respiratory (ventral) and gastrointestinal (dorsal) tubes. This process depends on the precise temporal and spatial pattern of expression of a number of foregut patterning genes. Disturbance of this pattern disrupts foregut separation and underlies the development of tracheo-oesophageal malformations. PMID:19103415
Wang, Kasper S
Biliary atresia is the most common cause of pediatric end-stage liver disease and the leading indication for pediatric liver transplantation. Affected infants exhibit evidence of biliary obstruction within the first few weeks after birth. Early diagnosis and successful surgical drainage of bile are associated with greater survival with the child's native liver. Unfortunately, because noncholestatic jaundice is extremely common in early infancy, it is difficult to identify the rare infant with cholestatic jaundice who has biliary atresia. Hence, the need for timely diagnosis of this disease warrants a discussion of the feasibility of screening for biliary atresia to improve outcomes. Herein, newborn screening for biliary atresia in the United States is assessed by using criteria established by the Discretionary Advisory Committee on Heritable Disorders in Newborns and Children. Published analyses indicate that newborn screening for biliary atresia by using serum bilirubin concentrations or stool color cards is potentially life-saving and cost-effective. Further studies are necessary to evaluate the feasibility, effectiveness, and costs of potential screening strategies for early identification of biliary atresia in the United States.
Kerkeni, Yosra; Ksia, Amine; Zitouni, Hayet; Belghith, Mohsen; Lassad, Sahnoun; Krichene, Imed; Mekki, Mongi; Nouri, Abdellatif
Polysplenia syndrome is a rare malformation characterized by the association of multiple rates and other congenital anomalies dominated by cardiac, vascular, intestinal and bile malformations. We report the observation of a patient operated in the neonatal period (3 days) for an upper intestinal obstruction with situs inversus. Surgical exploration noted the presence of multiple rates, a preduodenal vein, a biliary atresia and a duodenal atresia. The surgical procedures performed were a latero-lateral duodeno-duodenostomy and hepatoportoenterostomy of KASAI with simple immediate and delayed outcomes. The follow up was of 23 years. We recall the epidemiological characteristics of this malformative association and we discuss the role played by the prognosis of polysplenia syndrome in the evolution of biliary atresia. The diagnosis and treatment of biliary atresia are always urgent to increase the chances of success of the Kasai, and the chances of prolonged survival with native liver. However, almost all long-term survivors (even anicteric) have biliary cirrhosis, which requires lifelong follow up.
Cancer - esophagus ... Esophageal cancer is not common in the United States. It occurs most often in men over 50 years old. There are two main types of esophageal cancer: squamous cell carcinoma and adenocarcinoma. These two types ...
... from your throat to your stomach. Early esophageal cancer usually does not cause symptoms. Later, you may ... You're at greater risk for getting esophageal cancer if you smoke, drink heavily, or have acid ...
Butto, F; Margraf, L; Smith, G; Najmabadi, H
A unique case of aortic atresia and tricuspid atresia associated with transposition of the great vessels is described in a newborn infant. The clinical, echocardiographic, and heart catheterization data are presented. The embryological and surgical implications are discussed.
Bauman, Zachary; Nanagas, Victor
We encountered a rare case of gastroschisis associated with jejunal atresia and colonic atresia. In our case, the jejunal atresia was not discovered for 27 days after the initial abdominal wall closure. The colonic atresia was not discovered for 48 days after initial repair of the gastroschisis secondary to the rarity of the disorder. Both types of atresia were repaired with primary hand-sewn anastomoses. Other than the prolonged parenteral nutrition and hyperbilirubinemia, our patient did very well throughout his hospital course. Based on our case presentation, small bowel atresia and colonic atresia must be considered in patients who undergo abdominal wall closure for gastroschisis with prolonged symptoms suggestive of bowel obstruction. Our case report also demonstrates primary enteric anastomosis as a safe, well-tolerated surgical option for patients with types of intestinal atresia. PMID:26180651
Alalayet, Yasen Fayez; Alkasim, F; Shiba, N; Aldhuayan, I; Alhamaidi, S; Alghamdi, G; Aljobair, F; Shoura, J; Alkhlaif, R
We report a case of a 2-year-old boy who presented with an empty left scrotum. Clinical examination revealed a left palpable undescended testis. During orchidopexy, segmental atresia of the vas deferens was found, and microsurgical repair was carried out. Segmental vasal atresia is an extremely rare condition and is infrequently diagnosed, especially in the pediatric age group. This is the first reported case of segmental vasal atresia without an association with cystic fibrosis. PMID:25419520
Weiss, A H; Iorio, N; Schey, R
Eosinophilic esophagitis (EoE) is characterized by eosinophilic infiltration of the esophagus and is a potential cause of dysphagia and food impaction, most commonly affecting young men. Esophageal manometry findings vary from normal motility to aperistalsis, simultaneous contractions, diffuse esophageal spasm, nutcracker esophagus or hypotonic lower esophageal sphincter (LES). It remains unclear whether esophageal dysmotility plays a significant role in the clinical symptoms of EoE. Our aim is to review the pathogenesis, diagnosis, and effect of treatment on esophageal dysmotility in EoE. A literature search utilizing the PubMed database was performed using keywords: eosinophilic esophagitis, esophageal dysmotility, motility, manometry, impedance planimetry, barium esophagogram, endoscopic ultrasound, and dysphagia. Fifteen studies, totaling 387 patients with eosinophilic esophagitis were identified as keeping in accordance with the aim of this study and included in this review. The occurrence of abnormal esophageal manometry was reported to be between 4 and 87% among patients with EoE. Esophageal motility studies have shown reduced distensibility, abnormal peristalsis, and hypotonicity of the LES in patients with EoE, which may also mimic other esophageal motility disorders such as achalasia or nutcracker esophagus. Studies have shown conflicting results regarding the presence of esophageal dysmotility and symptoms with some reports suggesting a higher rate of food impaction, while others report no correlation between motor function and dysphagia. Motility dysfunction of the esophagus in EoE has not been well reported in the literature and studies have reported conflicting evidence regarding the clinical significance of dysmotility seen in EoE. The correlation between esophageal dysmotility and symptoms of EoE remains unclear. Larger studies are needed to investigate the incidence of esophageal dysmotility, clinical implications, and effect of treatment on
Gupta, Lucky; Bhatnagar, Veereshwar
Background/Purpose: This study aims to analyze the incidence and type of various associated anomalies among infants with extrahepatic biliary atresia (EHBA), compare their frequency with those quoted in the existing literature and assess their role in the overall management. Materials and Methods: A retrospective study was performed on 137 infants who underwent the Kasai procedure for EHBA during the past 12 years. The medical records were reviewed for the incidence and type of associated anomalies in addition to the details of the management of the EHBA. Results: Of the137 infants, 40 (29.2%) were diagnosed as having 58 anomalies. The majority of patients had presented in the 3rd month of life; mean age was 81 ± 33 days (range = 20-150 days). There were 32 males and 8 females; boys with EHBA had a higher incidence of associated anomalies. Of these 40 patients, 22 (37.9%) had vascular anomalies, 13 patients (22.4%) had hernias (umbilical-10, inguinal-3), 7 patients (12.1%) had intestinal malrotation, 4 patients (6.8%) had choledochal cyst, 1 patient (1.7%) had Meckel's diverticulum, 3 patients (5%) had undergone prior treatment for jejunoileal atresias (jejunal-2, ileal-1), 2 patients (3.4%) had undergone prior treatment for esophageal atresia and tracheoesophageal fistula, 2 patients (3.4%) had spleniculi, and 2 patients (3.4%) were diagnosed as having situs inversus. Conclusions: The most common associated anomalies in our study were related to the vascular variation at the porta hepatis and the digestive system. The existence of anomalies in distantly developing anatomic regions in patients with EHBA supports the possibility of a “generalized” insult during embryogenesis rather than a “localized” defect. In addition, male infants were observed to have significantly more associated anomalies as compared with the female infants in contrast to earlier reports. PMID:26862288
Fall, Mbaye; Mbaye, Papa Alassane; Horace, Haingonirina Joelle; Wellé, Ibrahima Bocar; Lo, Faty Balla; Traore, Mamadou Mour; Diop, Marie; Ndour, Oumar; Ngom, Gabriel
Background: Oesophageal atresia is a neonatal emergency surgery whose prognosis has improved significantly in industrialised countries in recent decades. In sub-Saharan Africa, this malformation is still responsible for a high morbidity and mortality. The objective of this study was to analyse the diagnostic difficulties and its impact on the prognosis of this malformation in our work environment. Patients and Methods: We conducted a retrospective study over 4 years on 49 patients diagnosed with esophageal atresia in the 2 Paediatric Surgery Departments in Dakar. Results: The average age was 4 days (0-10 days), 50% of them had a severe pneumonopathy. The average time of surgical management was 27 h (6-96 h). In the series, we noted 10 preoperative deaths. The average age at surgery was 5.7 days with a range of 1-18 days. The surgery mortality rate is 28 patients (72%) including 4 late deaths. Conclusion: The causes of death were mainly sepsis, cardiac decompensation and anastomotic leaks. PMID:26612124
... in chromosomes, or they can be multifactorial. For example, approximately 10 percent of people with CHARGE syndrome , which is usually caused by mutations in the CHD7 gene, have EA/TEF . About ...
... conditions that suppress or weaken your immune system Organisms (germs) that cause esophagitis include fungi, yeast, and viruses. Common organisms include: Candida albicans Cytomegalovirus (CMV) Herpes simplex virus ( ...
Shortsleeve, M.J.; Gauvin, G.P.; Gardner, R.C.; Greenberg, M.S.
Four patients with herpetic esophagitis were examined. In three of them, the presenting symptom was odynophagia. Early in the course of herpetic esophagitis, shallow round and oval ulcers were seen on barium esophagograms. Later, the ulcers filled with fibrinous exudate, forming nodular plaques that projected into the esophageal lumen. Although these findings are diagnostic of esophagitis, they are not specific for a herpes virus infection. The definitive diagnosis must be established by histologic examination, which demonstrates the cytopathic effect of the herpes virus infection within the squamous epithelium.
Castle, Shannon L; Naik-Mathuria, Bindi J; Torres, Manuel B
We present a case of a neonate with VACTERL-like association, with the VACTERL association defined as the non-random association of vertebral, anal, cardiac, esophageal, renal/kidney, and limb defects, as manifested by a hemivertebra, imperforate anus, and digit anomalies, in rare association with duodenal atresia and right-sided diaphragmatic hernia. This constellation is previously undescribed and may offer insight into the pathogenesis of VACTERL and associated birth defects.
Touloukian, R J
A total of 116 cases of intestinal atresia or stenosis were encountered at the Yale-New Haven Hospital between 1970 and 1990. Sites involved were the duodenum (n = 61; 53%), jejunum or ileum (n = 47; 46%), and colon (n = 8; 7%). All but two patients underwent operative correction, for an overall survival rate of 92%. Challenging problems were the management of apple-peel atresia (five patients), multiple intestinal atresia with short-gut syndrome (eight patients), and proximal jejunal atresia with megaduodenum requiring imbrication duodenoplasty (four patients). Major assets in the improved outlook for intestinal atresia are prenatal diagnosis, regionalization of neonatal care, improved recognition of associated conditions, innovative surgical methods, and uncomplicated long-term total parenteral nutrition.
Gupta, Rahul; Gupta, Shilpi; Sharma, Pramila; Bhandari, Anu; Gupta, Arun Kumar; Mathur, Praveen
Gallbladder duplication in association with other GIT anomalies is a rare entity. We report two neonates; one with duodenal atresia and the other newborn with pyloric atresia, ileal atresia and colonic atresia, both were associated with gallbladder duplication which has not been reported earlier. PMID:27123398
Tarasiuk, B A; Iaremenko, V V; Babko, S A; Klimenko, E F; Medvedenko, G F
The assessment of echographic features of biliary atresia was conducted in 65 newborn children ageing up to 3 mo. Their characteristic variants were revealed: the absence or reduction in size of gall-bladder, the presence of hyperechogenic triangular formation in V. portae bifurcation (the symptom of "triangular cicatrix"); the thickening of anterior wall of V. portae right branch. The timely and correct establishment of the diagnosis permits a child to survive and serve the hepatic fibrosis prophylaxis. Echohepatography is a sufficiently trustful method of investigation.
Vakil, Nimish; Affi, Aboud
Despite advances in our knowledge of esophageal cancer, 50% of patients present with incurable disease, and the overall survival after diagnosis is poor. The incidence of esophageal adenocarcinoma of the distal esophagus is rising at a rapid rate in developed countries. Recent advances in the epidemiology of esophageal cancer offer insights into preventive strategies in patients who are at risk. New developments in diagnosis may help detect the disease at an early stage. New diagnostic modalities permit more accurate staging procedures and allow appropriate selection of therapy. New studies provide more information on multimodality therapy for esophageal cancer, and new endoscopic techniques allow resection of small lesions without surgery. New stent designs provide better palliation by providing tumor ingrowth. These developments in the treatment of esophageal cancer are the focus of this review.
El-Asmar, Khaled M.; Abdel-Latif, Mohammed; El-Kassaby, Abdel-Hamid A.; Soliman, Mohamed H.; El-Behery, Mosad M.
Background: Colonic atresia (CA) is a rare form of congenital intestinal atresia. Although CA may be isolated, it is more commonly reported in literature in association with other congenital anomalies. Materials and Methods: This study is a review of prospectively collected data of all the patients with colonic atresia presented to our center (Ain Shams University) during 2008 to 2016. Results: Twelve patients were enrolled in this study. The atresia was of type I in one case, type II in four cases, type IIIa in six cases, type IV in one case. These cases accounted for 4.9 % of intestinal atresias managed in our center during the same period. Five cases were isolated CA, while the other seven cases had associated abdominal congenital anomalies (exomphalos, Hirschsprung's disease, imperforate anus, closing gastroschisis, colonic duplication, and multiple small bowel atresia in two cases). The management in ten cases was by staged procedure with creation of a temporary stoma initially, while primary anastomosis was established in two cases. We had two cases with delayed presentations, one missed diagnosis, and three mortalities in this series. Conclusions: The low incidence of CA may result in delay in the diagnosis and management. Hirschsprung's disease should be excluded in every case of colonic atresia. Early diagnosis and proper surgical management is essential for good prognosis. PMID:27896155
Angotti, R; Molinaro, F; Bulotta, AL; Ferrara, F; Sica, M; Bindi, E; Messina, M
More than 50% of infants with esophageal atresia have associated anomalies. We present a case report of a 46XX neonate with long-gap esophageal atresia and tracheoesophageal fistula (EA/TEF), anorectal malformation, bowel duplication and vaginal agenesis. This is an unusual association of abnormalities which had not yet described in literature. PMID:27433450
Joung, Hae Soo; Guerrero, Alexandra Leon; Tomita, Sandra; Kuenzler, Keith A
Anorectal malformations (ARMs) commonly co-occur with other congenital anomalies, particularly VACTERL (vertebral, anorectal, cardiac, tracheal, esophageal, renal, limb, and duodenal) associations. However, this collection of associations is not comprehensive, and other concurrent anomalies may exist that can be missed during the standard work-up of patients with ARMs. We present a rare case of a neonate with a low ARM with concurrent jejuno-ileal atresia that was diagnosed after the correction of the ARM when the patient developed segmental volvulus. This case illustrates the importance of having a high index of suspicion when deviation from a classic presentation occurs.
Joung, Hae Soo; Guerrero, Alexandra Leon; Tomita, Sandra; Kuenzler, Keith A.
Anorectal malformations (ARMs) commonly co-occur with other congenital anomalies, particularly VACTERL (vertebral, anorectal, cardiac, tracheal, esophageal, renal, limb, and duodenal) associations. However, this collection of associations is not comprehensive, and other concurrent anomalies may exist that can be missed during the standard work-up of patients with ARMs. We present a rare case of a neonate with a low ARM with concurrent jejuno-ileal atresia that was diagnosed after the correction of the ARM when the patient developed segmental volvulus. This case illustrates the importance of having a high index of suspicion when deviation from a classic presentation occurs. PMID:27896167
Narang, Manish; Kumar, Manish; Shah, Dheeraj
Fraser cryptophthalmos syndrome is a severe genetic disorder comprising of cryptophthalmos, syndactyly and genitourinary abnormalities. Gastrointestinal malformations are also increasingly being described. We describe a neonate with this syndrome having colonic atresia leading to cecal rupture and pneumoperitoneum.
Culture - esophageal ... There, it is placed in a special dish (culture) and watched for the growth of bacteria, fungi, ... and Fordtran's Gastrointestinal and Liver Disease Pathophysiology/Diagnosis/Management . 10th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap ...
Kahrilas PJ, Pandolfino JE. Esophageal neuromuscular function and motility disorders. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease . 9th ed. Philadelphia, PA: ...
... is the sixth most common cause of cancer deaths worldwide. Incidence rates vary within different geographic locations. In some regions, higher rates of esophageal cancer cases may be attributed to tobacco and alcohol use or particular nutritional habits and ...
... or gastroesophageal reflux disease: A systematic review. Clinical Gastroenterology and Hepatology. 2015;13:251. Coss-Adame E, et al. ... esophageal (noncardiac) chest pain: An expert review. Clinical Gastroenterology and Hepatology. 2014;12:1224. Castell DO. Chest ...
Dellon, Evan S.
Eosinophilic esophagitis (EoE) is a chronic immune-mediated condition where infiltration of eosinophils into the esophageal mucosa leads to symptoms of esophageal dysfunction. It has rapidly emerged as an important cause of upper GI morbidity in patients of all ages and is encountered in a substantial proportion of patients undergoing diagnostic upper endoscopy. This review discusses the clinical, endoscopic, and histologic features of EoE and presents the most recent guidelines for diagnosis of EoE. It describes selected diagnostic dilemmas including distinguishing EoE from gastroesophageal reflux disease and addressing the newly recognized clinical entity of proton pump inhibitor responsive esophageal eosinophilia. It also highlights evidence to support both pharmacologic and non-pharmacologic treatments, including topical corticosteroids, dietary elimination therapy, and endoscopic dilation. PMID:23452635
Kunisaki, Shaun M; Coran, Arnold G
This article focuses on esophageal replacement as a surgical option for pediatric patients with end-stage esophageal disease. While it is obvious that the patient׳s own esophagus is the best esophagus, persisting with attempts to retain a native esophagus with no function and at all costs are futile and usually detrimental to the overall well-being of the child. In such cases, the esophagus should be abandoned, and the appropriate esophageal replacement is chosen for definitive reconstruction. We review the various types of conduits used for esophageal replacement and discuss the unique advantages and disadvantages that are relevant for clinical decision-making. Copyright © 2017 Elsevier Inc. All rights reserved.
... its ability to move food toward the stomach ( achalasia ) A weak LES, which causes heartburn (GERD) Abnormal ... PA: Elsevier Saunders; 2010:chap 43. Read More Achalasia Esophageal spasm Gastroesophageal reflux disease Review Date 8/ ...
Pimenta, Júlio R; Ferreira, Alexandre R; Fagundes, Eleonora D T; Queiroz, Thaís C N; Baptista, Regiane A N; de Araújo Moreira, Eduardo G; de Resende, Camilo B; Bittencourt, Paulo F S; Carvalho, Simone D; Neto, José A F; Penna, Francisco J
Bleeding of esophageal varices is the main cause of morbidity and mortality in children with portal hypertension. It is important to understand the factors related with a bleeding episode to evaluate more effective primary prophylaxis. The present study aims to describe the endoscopic and laboratory findings associated with upper gastrointestinal bleeding (UGIB) secondary to esophageal varices. A cross-sectional study with 103 children and adolescents with cirrhosis, divided into a group that had experienced an episode of upper UGIB (35 patients) and a group without a history of UGIB (68 patients), was carried out. The esophageal and gastric varices were classified, and the portal hypertensive gastropathy, laboratory findings, and Child-Pugh classification were measured. Factors observed in univariate analysis to be associated with UGIB were the presence of esophageal varices of medium caliber or larger, portal hypertensive gastropathy, presence of red spots on esophageal varices, Child-Pugh class B or C, and hypoalbuminemia (P < 0.05). After multivariate logistic regression analysis, the significant factors were the presence of red spots on esophageal varices and the presence of gastric varices. When separated the autoimmune hepatitis, nonbiliary atresia patients (all patients except the patients with biliary atresia), and biliary atresia groups the findings in the univariate analysis were the presence of esophageal varices of medium or larger caliber, presence of red spots on varices, and presence of gastric varices in the autoimmune hepatitis patients and nonbiliary atresia patients and presence of red spots on esophageal varices, presence of gastric varices, and Child-Pugh classification B or C in biliary atresia group (P < 0.05). After multivariate logistic regression analysis, no statistical significance was found for any factor analyzed in any groups. The presence of gastric varices and red spots on esophageal varices were related to episodes of UGIB
Sathe, Pragati Aditya; Ghodke, Ratnaprabha Kundlikrao; Kandalkar, Bhuvaneshwari Mahendra
We are reporting a rare case of sirenomelia with oesophageal atresia. Sirenomelia is a lethal sporadic defect of which lower gastrointestinal tract anomalies are characteristic findings. Respiratory and upper gastrointestinal tract malformations like oesophageal atresia occur in about 20-35% of cases. Though its occurrence has been described, it has been reported only rarely. This report aims at describing this uncommon association along with its histological features.
Wynchank, S.; Guillet, J.; Leccia, F.; Soubiran, G.; Blanquet, P.
Hepatobiliary scintigraphy using Tc-99m diethyl IDA was performed on 14 jaundiced neonates. It aided greatly the differential diagnosis between neonatal hepatitis and biliary atresia. Limitations in the interpretation of the results are described, as neonatal hepatitis may be accompanied by biliary excretion ranging from zero to normal. Also both biliary atresia (intra- and extrahepatic) and neonatal hepatitis may show no biliary excretion within 24 hours.
Yuan, Y; Wang, K-N; Chen, L-Q
This review gives an overview of the esophageal anastomosis. The history, various techniques and substitution organs, their advantages and disadvantages, healing mechanism, complications, and actual trend of this essential part of esophageal surgery are described. The history of the esophageal anastomosis extending from the first anastomosis in 1901 to today has undergone more than one century. In the early days, the success rate of the anastomosis was extremely low. As the technology progressed, the anastomosis got significant achievement. Various anastomotic techniques are currently being used. However, controversies exist on the choice of anastomotic method concerning the success rate, postoperative complication and quality of life. How to choose the method, no one can give the best answer. We searched the manuscripts about the esophageal anastomoses in recent years and studied the controversy questions about the anastomosis. Performing an esophageal anastomosis is a technical matter, and suture healing is independent of the patient's biologic situation. Every anastomosis technique has its own merit, but the outcomes were different if it was performed by different surgeons, and we also found that the complication rate of the anastomosis was mainly associated with the surgeons. So the surgeons should learn from their previous experience and others to avoid technical errors.
Özçınar, Beyza; Peker, Kıvanç Derya; Demirel, Sertaç; Yanar, Fatih; Tuncer, Koray; İğci, Abdullah
Herein, a case of intramural esophageal dissection is reported and the literature is reviewed. Intramural esophageal dissection is a rare but well described condition that is characterized by a laceration between the esophageal mucosa and submucosa but without perforation. A female patient aged 86 years was hospitalized with a diagnosis of abdominal aortic aneurysm. After placement of an aortic stent, she was started on intravenous heparin. After the procedure, the patient had retching and vomiting due to sedative drugs. On the first day after the procedure, the patient experienced sudden-onset chest pain, hematemesis, back pain and odynophagia. A hematoma was detected in the thoracic esophagus, which was opened during endoscopy and began to bleed suddenly owing to air insufflation. A false lumen was visualized within the esophagus. There was no perforation. The patient was followed up conservatively and discharged from the hospital uneventfully. In conclusion, we propose that esophageal transection, a condition that is widely regarded as relatively benign in the literature, has the potential to lead to perforation. It would be expected that most cases of esophageal transection would be managed conservatively. PMID:28149126
Pueyo, Carlos; Maldonado, Joaquín; Royo, Yolanda; Skrabski, Robert; Di Crosta, Ida; Raventós, Antoni
Intrauterine intussusception is an uncommon cause of intestinal atresia. We report a case of ileal atresia owing to antenatal intussusception revealed as an intraluminal polypoid lesion after surgical intervention.
Russell, Katie W; Rollins, Michael D; Feola, G Peter; Scaife, Eric R
We report a case of rectal atresia treated using magnets to create a rectal anastomosis. This minimally invasive technique is straightforward and effective for the treatment of rectal atresia in children. PMID:25096648
Gupte, Anand R; Draganov, Peter V
Eosinophilic esophagitis is increasingly recognized in adults. The diagnosis is based on the presence of both typical symptoms and pathologic findings on esophageal biopsy. Patients usually present with dysphagia, food impaction and/or reflux-like symptoms, and biopsy of the esophagus shows more than 15 eosinophils per high-power field. In addition, it is essential to exclude the presence of known causes of tissue eosinophilia such as gastroesophageal reflux disease, infections, malignancy, collagen vascular diseases, hypersensitivity, and inflammatory bowel disease. There are no standardized protocols for the therapy of eosinophilic esophagitis. A variety of therapeutic approaches including acid suppression, dietary modifications, topical corticosteroids and endoscopic dilation can be used alone or in combination. PMID:19115464
Genc, Selahattin; Kahraman, Erkan; Ozel, Halil Erdem; Arslan, Ilker Burak; Demir, Ahmet; Selcuk, Adin
The purpose of this study was to show the clinical characteristics of microtia and congenital aural atresia cases in Turkey and to make the classification. For this purpose, records of 28 patients with microtia who were admitted to the ENT Clinic of Eskisehir Military Hospital, Turkey, between 1995 and 2011 and 3 patients admitted to the ENT outpatient clinic of Kocaeli Derince Education and Research Hospital, Turkey, were analyzed retrospectively. Of the total 31 patients with microtia (35 microtic ears), involvement of the right ear of 20 patients (64.5%), the left ear of 7 patients (22.5%), and bilateral involvement in 4 patients (12.9%) were observed. There was a unilateral involvement in 27 patients (87.1%). According to the Marx grading, 2 patients (5.7%) had grade 1 malformation, 3 (8.6%) had grade 2 malformation, 29 (82.9%) had grade 3 malformation, and 1 (2.9%) had grade 4 malformation (anotia). Although the characteristics of microtia vary in different population, the results in Turkey are consistent with those in the literature.
M, Braiek; A, Ksia; I, Krichen; S, Belhassen; K, Maazoun; S, Ben youssef; N, Kechiche; M, Mekki; A, Nouri
Rectal atresia (RA) with a normal anus is a rare anomaly. We describe a case of rectal atresia in a newborn male presenting with an abdominal distension and failure of passing meconium. The rectal atresia was primarily operated by transanal route. PMID:27123404
M, Braiek; A, Ksia; I, Krichen; S, Belhassen; K, Maazoun; S, Ben Youssef; N, Kechiche; M, Mekki; A, Nouri
Rectal atresia (RA) with a normal anus is a rare anomaly. We describe a case of rectal atresia in a newborn male presenting with an abdominal distension and failure of passing meconium. The rectal atresia was primarily operated by transanal route.
Vasudevan, Srikanth; Huang, Jiying; Botterman, Barry; Matloub, Hani S; Keefer, Edward; Cheng, Jonathan
Long-gap peripheral nerve defects arising from tumor, trauma, or birth-related injuries requiring nerve reconstruction are currently treated using nerve autografts and nerve allografts. Autografts are associated with limited supply and donor-site morbidity. Allografts require administration of transient immunosuppressants, which has substantial associated risks. To overcome these limitations, we investigated the use of detergent-free decellularized nerve grafts to reconstruct long-gap nerve defects in a rodent model and compared it with existing detergent processing techniques. Nerve grafts were harvested from the sciatic nerves of 9 donor rats. Twenty-four recipient rats were divided into 4 groups (6 animals per group): (1) nerve grafts (NG, positive control), (2) detergent-free decellularized (DFD) grafts, (3) detergent decellularized grafts, and (4) silicone tube conduits (negative control). Each recipient rat had a 3.5-cm graft or conduit sutured across a sciatic nerve transection injury. All animals were harvested at 12 weeks postimplantation for functional muscle analysis and nerve histomorphometry. Histomorphometry results indicated maximum growth in NG when compared with other groups. DFD and detergent decellularized groups showed comparable regeneration at 12 weeks. Silicone tube group showed no regeneration as expected. Muscle force data indicated functional recovery in NG and DFD groups only. This study describes a detergent-free nerve decellularization technique for reconstruction of long-gap nerve injuries. We compared DFD grafts with an established detergent processing technique and found that DFD nerve grafts are successful in promoting regeneration across long-gap peripheral nerve defects as an alternative to existing strategies.
Vasudevan, Srikanth; Huang, Jiying; Botterman, Barry; Matloub, Hani S.; Keefer, Edward
Background: Long-gap peripheral nerve defects arising from tumor, trauma, or birth-related injuries requiring nerve reconstruction are currently treated using nerve autografts and nerve allografts. Autografts are associated with limited supply and donor-site morbidity. Allografts require administration of transient immunosuppressants, which has substantial associated risks. To overcome these limitations, we investigated the use of detergent-free decellularized nerve grafts to reconstruct long-gap nerve defects in a rodent model and compared it with existing detergent processing techniques. Methods: Nerve grafts were harvested from the sciatic nerves of 9 donor rats. Twenty-four recipient rats were divided into 4 groups (6 animals per group): (1) nerve grafts (NG, positive control), (2) detergent-free decellularized (DFD) grafts, (3) detergent decellularized grafts, and (4) silicone tube conduits (negative control). Each recipient rat had a 3.5-cm graft or conduit sutured across a sciatic nerve transection injury. All animals were harvested at 12 weeks postimplantation for functional muscle analysis and nerve histomorphometry. Results: Histomorphometry results indicated maximum growth in NG when compared with other groups. DFD and detergent decellularized groups showed comparable regeneration at 12 weeks. Silicone tube group showed no regeneration as expected. Muscle force data indicated functional recovery in NG and DFD groups only. Conclusions: This study describes a detergent-free nerve decellularization technique for reconstruction of long-gap nerve injuries. We compared DFD grafts with an established detergent processing technique and found that DFD nerve grafts are successful in promoting regeneration across long-gap peripheral nerve defects as an alternative to existing strategies. PMID:25426384
Furuta, Glenn T.; Katzka, David A.
Once considered a rare condition, eosinophilic esophagitis is now one of the most common conditions diagnosed during the assessment of feeding problems in children and during the evaluation of dysphagia and food impaction in adults.1 The entity exists worldwide but has been most extensively studied in Western countries, where its prevalence has been estimated to be 0.4% among all children and adults.2 Whether eosinophilic esophagitis is truly a new disease or simply a recently recognized one is uncertain.3 In this review, we consider the diagnostic criteria, pathophysiological and clinical features, and treatment of this increasingly prevalent disease. PMID:26488694
Cozzi, F; Steiner, M; Rosati, D; Madonna, L; Colarossi, G
The clinical features of 41 infants with various types of nasal obstruction were reviewed to study the correlation between the degree of nasal obstruction and clinical manifestations. Twenty-one subjects had bilateral choanal atresia/stenosis; 12 had unilateral choanal atresia/stenosis, and eight who were referred with suspected choanal atresia had a simple inflammatory nasal obstruction. Patients with bilateral choanal obstruction and patients with unilateral choanal obstruction or rhinitis showed no differences in clinical picture or in referral age. Many infants with either unilateral or bilateral choanal obstruction had one or more symptoms not fully relieved after surgical repair, although the choanae were widely patent. At long-term follow-up the surviving patients showed spontaneous recovery and good nasal respiration. Overall, five patients died. Since the common syndrome appeared to be related to a dysfunction of the autonomic nervous system, we conclude that any type of nasal obstruction may exacerbate or precipitate an underlying maturational autonomic disturbance.
Govindarajan, Krishna Kumar
The pathway from clinical suspicion to establishing the diagnosis of biliary atresia in a child with jaundice is a daunting task. However, investigations available help to point towards the correct diagnosis in reasonable time frame. Imaging by Sonography has identified several parameters which can be of utility in the diagnostic work up. Comparison of Sonography with imaging by Nuclear medicine can bring out the significant differences and also help in appropriate imaging. The battery of Biochemical tests, available currently, enable better understanding of the line-up of investigations in a given child with neonatal cholestasis. Management protocols enable standardized care with optimal outcome. The place of surgical management in biliary atresia is undisputed, although Kasai procedure and primary liver transplantation have been pitted against each other. This article functions as a platform to bring forth the various dimensions of biliary atresia. PMID:28083081
Govindarajan, Krishna Kumar
The pathway from clinical suspicion to establishing the diagnosis of biliary atresia in a child with jaundice is a daunting task. However, investigations available help to point towards the correct diagnosis in reasonable time frame. Imaging by Sonography has identified several parameters which can be of utility in the diagnostic work up. Comparison of Sonography with imaging by Nuclear medicine can bring out the significant differences and also help in appropriate imaging. The battery of Biochemical tests, available currently, enable better understanding of the line-up of investigations in a given child with neonatal cholestasis. Management protocols enable standardized care with optimal outcome. The place of surgical management in biliary atresia is undisputed, although Kasai procedure and primary liver transplantation have been pitted against each other. This article functions as a platform to bring forth the various dimensions of biliary atresia.
Puri, P; Fujimoto, T
It has been suggested that multiple intestinal atresias result from multiple ischemic infarctions of the intestinal tract. We have studied surgical material from 59 neonates with intestinal atresias seen at our hospital between 1975 and 1986. Forty (68%) patients had single intestinal atresias and 19 (32%) had multiple atresias. There were seven cases of hereditary multiple atresias seen in three families and 12 cases of nonhereditary multiple atresias. All hereditary cases had numerous type I or type II gastrointestinal atresias but none had type IIIa atresia. Six of the seven hereditary cases had multiple atresias in the small as well as large bowel. The 12 patients with nonhereditary atresias had various types of atresias but mesenteric or intestinal interruption was observed in only two patients. All patients with hereditary multiple intestinal atresias showed identical microscopic appearances in the small and large intestine, consisting of sieve-like multiple lumina, each surrounded by its own mucosa and muscularis mucosae but sharing a common muscle coat. There was no evidence of lanugo, bile pigments, or squames within the lumen distal to atretic segments in any of these patients. Six nonhereditary cases who had multiple septal atresias affecting only the small bowel demonstrated essentially similar lesions on microscopic examination as seen in hereditary cases. There was no evidence of arterial occlusion in the mesentery and lanugo, bile pigments, and squames could not be found distally in the intestinal contents in any of these cases. These pathologic findings suggest that all cases of hereditary multiple intestinal atresias and some cases of nonhereditary multiple intestinal atresias are a consequence of a malformative process of the gastrointestinal tract rather than an ischemic process.
... Disease Tricuspid Valve Disease Cardiac Rhythm Disturbances Thoracic Aortic Aneurysm Pediatric and Congenital Heart Disease Heart abnormalities that are present at birth in children, as well as in adults Atrial Septal Defect Ventricular Septal ... Arteriosus Single Ventricle Defects Lung, Esophageal, and ...
... is to complete a test called an esophageal pH test. In this test, a very thin tube is placed through the nose into the esophagus and stomach, or a temporary sensor is placed in the esophagus via endoscopy. Both allow levels of acid in the esophagus to be monitored ...
Hseu, Anne; Anne, Premchand
This is a case report of a three-month-old male who presented to clinic with a cystic lesion under the tongue. On clinical examination, a cystic lesion was observed in the, floor-of-mouth. The patient was referred to Paediatric Otorhinolaryngology service for further management. The differential diagnoses for floor-of-mouth lesions should be reviewed with primary focus on the Wharton’s duct atresia and its management. It is crucial to recognize submandibular duct atresia in the primary Paediatric clinic in order to expedite management of lesion before complications arise including infection, enlargement of cyst, and feeding and breathing difficulties. PMID:27042492
Herpetic esophagitis is a herpes simplex infection causing inflammation and ulcers of the esophagus. Symptoms include difficulty swallowing and pain (dysphagia). Herpetic esophagitis can be effectively ...
Loomba, Rohit S; Geddes, Gabrielle
Rubinstein-Taybi syndrome is a well-characterized condition causing distinctive physical characteristics, intellectual disability, and multiple congenital malformations. Cardiac abnormalities are found in a third of individuals with this condition and usually consist of isolated septal defects or patent ductus arteriosus, although more complex congenital lesions have been described. We present the first reported case of tricuspid atresia and pulmonary atresia with hypoplasia of the right ventricle in the setting of Rubenstein-Taybi syndrome. PMID:26085772
Ferrarini, Alessandra; Osterheld, Maria-Chiara; Vial, Yvan; de Viragh, Pierre A; Cotting, Jacques; Martinet, Danielle; Beckmann, Jacques S; Fellmann, Florence
We report on two familial cases from a non-consanguineous marriage, presenting multiple intestinal and choanal atresia. Massive hydramnios and dilatation of the bowel were observed at 29 weeks of gestation during routine ultrasound scan of a healthy mother. The fetal karyotype was normal and cystic fibrosis screening was negative. Regular scans were performed throughout the pregnancy. The child was born at 34 weeks gestation. Choanal atresia was diagnosed at birth and abdominal investigations showed multiple atresia interesting both the small bowel and the colon. Further interventions were necessary because of recurrent obstructions. During the following pregnancy, a dilatation of the fetal intestinal tract was detected by ultrasonography at 27 weeks of gestation. Pregnancy was interrupted. Post-mortem examination of the fetus confirmed the stenosis of long segments of the small intestine associated with areas of colonic atresia. In both cases, histology and distribution were consistent with those reported in hereditary multiple intestinal atresia (HMIA). An association between multiple intestinal and choanal atresia has never been reported. We suggest it could correspond to a new autosomal recessive entity for which cytogenetic investigations and high-resolution array CGH revealed no visible anomalies.
Hill, L.D.; Mercer, C.D.; McCallum, R.W.; Kozarek, R.
This book integrates gastroenterology and thoracic surgery to detail the comprehensive management of esophageal disease. It describes radiologic and functional evaluation of the esophagus, endoscopy, medical and surgical treatments, and results and also covers gastroesophageal reflux disease, tumors motility, esophageal replacement, intubation, esophageal diverticula, caustic esophageal injury. It presents Dr. Hill's surgical procedures in detail.
Our aim is to present a case with initial diagnosis of non-classified type duodenal atresia operated in our clinic. A patient with prenatally suspected to be duodenal atresia was explored. At laparotomy type 3 duodenal atresia was found between 2(nd) and 3(rd) parts of duodenum. In addition, a web was detected distal to the atresic part. Duodenoduodenostomy together with web excision was performed. He had not any additional pathology. Although duodenal atresia is a very well known pathology by pediatric surgeons, though rarely a non-classified type duodenal atresia can be encountered. Possible presence of a second atresia should be kept in mind, proximal and distal segments of the duodenum and intestinal passage must be explored carefully for the presence of second atresic segment before performing duodenoduodenostomy.
Mahoney, Lisa; Rosen, Rachel
Feeding difficulties such as dysphagia, coughing, choking, or vomiting during meals, slow eating, oral aversion, food refusal, and stressful mealtimes are common in children with repaired esophageal atresia (EA) and the reasons for this are often multifactorial. The aim of this review is to describe the possible underlying mechanisms contributing to feeding difficulties in patients with EA and approaches to management. Underlying mechanisms for these feeding difficulties include esophageal dysphagia, oropharyngeal dysphagia and aspiration, and aversions related to prolonged gastrostomy tube feeding. The initial diagnostic evaluation for feeding difficulties in a patient with EA may involve an esophagram, videofluoroscopic imaging or fiberoptic endoscopic evaluation during swallowing, upper endoscopy with biopsies, pH-impedance testing, and/or esophageal motility studies. The main goal of management is to reduce the factors contributing to feeding difficulties and may include reducing esophageal stasis, maximizing reflux therapies, treating underlying lung disease, dilating strictures, and altering feeding methods, routes, or schedules. PMID:28620597
Antabak, Anko; Bogović, Marko; Vuković, Jurica; Grizelj, Ruža; Babić, Vinka Barbarić; Papeš, Dino; Luetić, Tomislav
Gastric perforation (GP) in neonates is a rare entity with high mortality. Although the etiology is not completely understood, it mostly occurs in premature neonates on assisted ventilation. Combination of duodenal atresia and gastric perforation is very rare. We present a case duodenal atresia who developed gastric perforation after operetion for duodenal atresia. Analysis of the patient medical record and histology report did not reveal the etiology of the perforation. PMID:27896170
Kinhal, Vidyadhar; Desai, Mahesh; Tilak; Choudhari, Fazal UR Rehman
Intestinal atresia is generally caused by intrauterine vascular obstructions involving mesenteric vessels. Intrauterine intussusceptions (IUI) are one of these disruptive events. Intestinal intussusceptions affects children commonly between 3 months and 3 years of age, but it rarely affects in intrauterine life. The relationship between intrauterine intussusception and intestinal atresia has been demonstrated by few cases in literature, suggesting intrauterine intussusception as a rare cause of intestinal atresia. We report a 7-day-old full term neonate presenting with intrauterine intussusceptions (jejuno-jejunal) resulting in jejunal atresia. PMID:26500958
Joshi, Sanjeev B; Kinhal, Vidyadhar; Desai, Mahesh; Tilak; Choudhari, Fazal Ur Rehman
Intestinal atresia is generally caused by intrauterine vascular obstructions involving mesenteric vessels. Intrauterine intussusceptions (IUI) are one of these disruptive events. Intestinal intussusceptions affects children commonly between 3 months and 3 years of age, but it rarely affects in intrauterine life. The relationship between intrauterine intussusception and intestinal atresia has been demonstrated by few cases in literature, suggesting intrauterine intussusception as a rare cause of intestinal atresia. We report a 7-day-old full term neonate presenting with intrauterine intussusceptions (jejuno-jejunal) resulting in jejunal atresia.
Ramareddy, Raghu Sampalli; Alladi, Anand
Aim: To review the patients with esophageal injuries and stenosis with respect to their etiology, clinical course, management, and the lessons learnt from these. Materials and Methods: Retrospective descriptive observation review of children with esophageal injuries and stenosis admitted between January 2009 and April 2015. Results: Eighteen children with esophageal injuries of varied etiology were managed and included, seven with corrosive injury, five with perforation due to various causes, three with mucosal erosion, two with trachea esophageal fistula (TEF), and one wall erosion. The five children who had perforation were due to poststricture dilatation in a child with esophageal atresia and secondary to foreign body impaction or its attempted retrieval in four. Alkaline button cell had caused TEF in two. Three congenital esophageal stenosis (CES) had presented with dysphagia and respiratory tract infection. Six corrosive stricture and two CES responded to dilatation alone and one each of them required surgery. Four of the children with esophageal perforation were detected early and required drainage procedure (1), diversion (1), and medical management (2). Pseudo diverticulum was managed expectantly. Among TEF, one had spontaneous closure and other one was lost to follow-up. All the remaining nineteen children have recovered well except one CES had mortality. Conclusion: Esophageal injuries though rare can be potentially devastating and life-threatening. PMID:27365909
Balaker, Ashley E; Roberson, Joseph B; Goldsztein, Hernan
(1) Determine the prevalence of a non-bony or fibrous incudostapedial (IS) joint in the setting of congenital aural atresia. (2) Assess this anomaly's impact on surgical management and associated hearing outcomes. Retrospective chart review. Subspecialty private practice. Operative reports and audiometric data of patients who underwent congenital aural atresia repair by a single surgeon from 2007 to 2011 were reviewed for operative anatomic findings and audiometric outcomes. Two hundred twenty-eight operations on 206 ears were performed. Median age was 5 years old. Fifty-five (26.7%) of these ears had a fibrous IS joint. The severity of this anomaly was graded as mild in 23 ears, moderate in 20 ears, and severe in 12 ears. Mean postoperative pure tone air conduction (PTA2) in the severely fibrous group was 51 compared to 46 in the moderate group (P = .03) and 41 in the mild group (P = .006). Patients with a fibrous IS joint who underwent successful ossicular chain reconstruction (OCR) had a mean postoperative PTA2 of 30, which was a significantly better outcome than in patients with moderately or severely fibrous IS joints who did not have OCR (P < .05). A fibrous IS joint was seen in 27% of patients undergoing repair of congenital aural atresia. The severity of this anomaly has important implications for postoperative hearing results. These findings suggest that ossicular chain reconstruction should be performed in moderately or severely fibrous cases.
Reilley, J.J.; Malmud, L.S.; Fisher, R.S.; Applegate, G.; DeVegvar, M.L.
Esophageal scintigraphy was developed in order to quantitatively evaluate esophageal transit in patients with a variety of esophageal disorders. The study is performed with orally administered technetium-99m sulfur colloid in water, using a gamma camera on-line to a digital computer. Esophageal transit is expressed as the percent emptying for each of the first 15-sec intervals for 10 min after an initial swallow and at 15-sec intervals after serial swallows. Esophageal transit is significantly decreased in patients with motor disorders of the esophagus, compared to normal controls. In patients with reflux esophagitis, esophageal transit was abnormal when the reflux disease was accompanied by abnormal motor function. The technique we describe is the first quantitative test of esophageal function; it is a useful, sensitive, scintigraphic technique for evaluation of esophageal transit.
Richter, Joel E
Tissue remodeling with scaring is common in adult EoE patients with long standing disease. This is the major factor contributing to their complaints of solid food dysphagia and recurrent food impactions. The best tests to define the degree of remodeling are barium esophagram, high resolution manometry and endoscopy. Many physicians are fearful to dilate EoE patients because of concerns about mucosal tears and perforations. However, multiple recent case series attest to the safety of esophageal dilation and its efficacy with many patients having symptom relief for an average of two years. This chapter will review the sordid history of esophageal dilation in EoE patients and outline how to perform this procedure safely. The key is graduated dilation over one to several sessions to a diameter of 15-18 mm. Postprocedural pain is to be expected and mucosal tears are a sign of successful dilation, not complications. In some healthy adults, occasional dilation may be preferred to regular use of medications or restricted diets. This approach is now supported by recent EoE consensus statements and societal guidelines.
Welch, R W; Luckmann, K; Ricks, P; Drake, S T; Bannayan, G; Owensby, L
The fasting lower esophageal sphincter pressure of 18 normal volunteers was compared to 22 patients with symptoms and objective evidence of gastroesophageal reflux. Lower esophageal sphincter pressure was measured by rapid pull-through using an 8-lumen radially perfused catheter that sampled pressure every45 degrees around the circumference of the sphincter. The 22 reflux patients were subdivided for analysis into two groups, those with an acute inflammatory infiltrate on biopsy and those without inflammation. Those patients without inflammatory esophagitis had normal sphincter pressures. Those with a definite inflammatory infiltrate had pressures significantly less than normal. The least reliable separation between normals and those with inflammatory esophagitis occurred in the anterior orientations. We conclude that while basal lower esophageal sphincter pressure measurement may identify patients with reflux and inflammatory esophagitis, it is of no help in identifying those patients with reflux unassociated with inflammation. Decreased basal fasting LESP does not appear to be the most important primary determinant of gastroesophageal reflux.
Ramírez Degollado, J; Martínez Aguilar, A; Peniche Bojórquez, J
Esophageal moniliasis is found rarely. It has been described mainly in chronically ill patients, who receive antibiotics and corticoesteroids. Early diagnosis and treatment betters their prognosis. Nine patients, 5 males and 4 females were studies in Hospital General del Centro Medico Nacional in Mexico City. Their agesranged from 26 to 77 years, with a mean of 49 years. All patients were chronically ill and 7 of them were treated in the intensive care unit. Three had disphagia, 3 retrosternal pain, and 2 gastrointestinal hemorrhage. Eight patients had high W.B.C., 3 irregular filling defects on X ray studies, and on endoscopy, all showed a pseudomembranous white yellowish exudate, underneath it the mucosa was inflamed, irregular and bled scantily. In 5 out of 9 patients biopsy and a smear confirmed the diagnosis. Eight patients treated with nystatin were cured. This disorder must be suspected in patients with disphagia and retrosternal pain; esophagoscopy is the prefered procedure to establish this diagnosis.
This review is based upon an invited lecture for the 52nd Annual Meeting of the British Association of Paediatric Surgeons, July 2015. The aetiology of biliary atresia (BA) is at best obscure, but it is probable that a number of causes or pathophysiological mechanisms may be involved leading to the final common phenotype we recognise clinically. By way of illustration, similar conditions to human BA are described, including biliary agenesis, which is the normal state and peculiar final pattern of bile duct development in the jawless fish, the lamprey. Furthermore, there have been remarkable outbreaks in the Australian outback of BA in newborn lambs whose mothers were exposed to and grazed upon a particular plant species (Dysphania glomulifera) during gestation. More recent work using a zebrafish model has isolated a toxic isoflavonoid, now named Biliatresone, thought to be responsible for these outbreaks. Normal development of the bile ducts is reviewed and parallels drawn with two clinical variants thought to definitively have their origins in intrauterine life: Biliary Atresia Splenic Malformation syndrome (BASM) and Cystic Biliary Atresia (CBA). For both variants there is sufficient clinical evidence, including associated anomalies and antenatal detection, respectively, to warrant their aetiological attribution as developmental BA. CMV IgM +ve associated BA is a further variant that appears separate with distinct clinical, histological, and immunohistochemical features. In these it seems possible that this involves perinatal obliteration of a normally formed duct system. Although still circumstantial, this evidence appears convincing enough to perhaps warrant a different treatment strategy. This then still leaves the most common (more than 60% in Western series) variant, now termed Isolated BA, whereby origins can only be alluded to.
Yang, Yaqi; Li, Weiguo; Xia, Yu; Yuan, Chuangye
The generation and propagation of a streamer is a significant physical process of air gap discharge. Research on the mechanism of streamers under low-pressure conditions is helpful for understanding the process of long-gap discharge in a high-altitude area. This paper describes laboratory investigations of streamer discharge under alternating current (AC) voltage in a low pressure test platform for a 60 cm rod-plane gap at 30 kPa, and analyzes the characteristics of streamer generation and propagation. The results show that the partial streamer and breakdown streamer all occur in the positive half-cycle of AC voltage near the peak voltage at 30 kPa. The partial streamer could cause the distortion of current and voltage waveform, and it appears as the branching characteristic at the initial stage. With the extension of the streamer, the branching and tortuosity phenomena become gradually obvious, but the branching is suppressed when the streamer crosses the gap. The low-pressure condition has little influence on the tortuosity length and the tortuosity number of the streamer, but affect the diameter of streamer obviously.
Gallo, Amy; Esquivel, Carlos O
It is encouraging that we are improving the technical aspects of treatment modalities for biliary atresia. However, it is clear that more needs to be done to best develop new treatment plans while applying the modalities we have (porto-enterostomy or liver transplantation or both) in a way that will afford the best survival and quality-of-life. This review article will discuss a number of points that are vital to improving care and illustrates the need to further scrutinize treatment decisions.
Read, Andrew J; Pandolfino, John E
Eosinophilic Esophagitis (EoE) is a chronic inflammatory disease of the esophagus triggered by an immune response that leads to symptoms of dysphagia, chest pain, and food impaction. EoE is a clinicopathologic syndrome that requires clinical symptoms and pathologic findings for a diagnosis. The inflammatory process and eosinophilic infiltration of the esophagus in EoE lead to fibrosis and structural changes within the esophagus that cause esophageal dysfunction. The biomechanics of the esophageal function in EoE have been explored using manometry, impedance planimetry, barium esophagograms, and endoscopic ultrasound. These studies have identified several biomechanical changes to the esophagus in EoE including pan-esophageal pressurization on manometry, changes in esophageal compliance with decreased distentisbility by impedance planimetry, decreased esophageal luminal diameter by esophagograms, and dysfunction in the esophageal longitudinal muscles by endoscopic ultrasound. Treatments for the disease involve dietary changes, immunosuppressive drugs, and dilation techniques. However, the data regarding the effect of these therapies on altering mechanical properties of the esophagus is limited. As the pathogenesis of esophageal dysfunction in EoE appears multifactorial, further study of the biomechanics of EoE is critical to better diagnose, monitor and treat the disease.
Pandolfino, John E
Eosinophilic Esophagitis (EoE) is a chronic inflammatory disease of the esophagus triggered by an immune response that leads to symptoms of dysphagia, chest pain, and food impaction. EoE is a clinicopathologic syndrome that requires clinical symptoms and pathologic findings for a diagnosis. The inflammatory process and eosinophilic infiltration of the esophagus in EoE lead to fibrosis and structural changes within the esophagus that cause esophageal dysfunction. The biomechanics of the esophageal function in EoE have been explored using manometry, impedance planimetry, barium esophagograms, and endoscopic ultrasound. These studies have identified several biomechanical changes to the esophagus in EoE including pan-esophageal pressurization on manometry, changes in esophageal compliance with decreased distentisbility by impedance planimetry, decreased esophageal luminal diameter by esophagograms, and dysfunction in the esophageal longitudinal muscles by endoscopic ultrasound. Treatments for the disease involve dietary changes, immunosuppressive drugs, and dilation techniques. However, the data regarding the effect of these therapies on altering mechanical properties of the esophagus is limited. As the pathogenesis of esophageal dysfunction in EoE appears multifactorial, further study of the biomechanics of EoE is critical to better diagnose, monitor and treat the disease. PMID:23105995
Trappey, A Francois; Hirose, Shinjiro
Esophageal duplication and congenital esophageal stenosis (CES) may represent diseases with common embryologic etiologies, namely, faulty tracheoesophageal separation and differentiation. Here, we will re-enforce definitions for these diseases as well as review their embryology, diagnosis, and treatment. Copyright © 2017. Published by Elsevier Inc.
Hanna, Jennifer M; Onaitis, Mark W
Robotic master-slave devices can assist surgeons to perform minimally invasive esophageal operations with approaches that have already been demonstrated using laparoscopy and thoracoscopy. Robotic-assisted surgery for benign esophageal disease is described for the treatment of achalasia, epiphrenic diverticula, refractory reflux, paraesophageal hernias, duplication cysts, and benign esophageal masses, such as leiomyomas. Indications and contraindications for robotic surgery in benign esophageal disease should closely approximate the indications for laparoscopic and thoracoscopic procedures. Given the early application of the technology and paucity of clinical evidence, there are currently no procedures for which robotic esophageal surgery is the clinically proven preferred approach. Copyright © 2014 Elsevier Inc. All rights reserved.
Dall’Oglio, Luigi; Caldaro, Tamara; Foschia, Francesca; Faraci, Simona; Federici di Abriola, Giovanni; Rea, Francesca; Romeo, Erminia; Torroni, Filippo; Angelino, Giulia; De Angelis, Paola
Post-esophageal atresia anastomotic strictures and post-corrosive esophagitis are the most frequent types of cicatricial esophageal stricture. Congenital esophageal stenosis has been reported to be a rare but typical disease in children; other pediatric conditions are peptic, eosinophilic esophagitis and dystrophic recessive epidermolysis bullosa strictures. The conservative treatment of esophageal stenosis and strictures (ES) rather than surgery is a well-known strategy for children. Before planning esophageal dilation, the esophageal morphology should be assessed in detail for its length, aspect, number and level, and different conservative strategies should be chosen accordingly. Endoscopic dilators and techniques that involve different adjuvant treatment strategies have been reported and depend on the stricture’s etiology, the availability of different tools and the operator’s experience and preferences. Balloon and semirigid dilators are the most frequently used tools. No high-quality studies have reported on the differences in the efficacies and rates of complications associated with these two types of dilators. There is no consensus in the literature regarding the frequency of dilations or the diameter that should be achieved. The use of adjuvant treatments has been reported in cases of recalcitrant stenosis or strictures with evidence of dysphagic symptoms. Corticosteroids (either systemically or locally injected), the local application of mitomycin C, diathermy and laser ES sectioning have been reported. Some authors have suggested that stenting can reduce both the number of dilations and the treatment length. In many cases, this strategy is effective when either metallic or plastic stents are utilized. Treatment complications, such esophageal perforations, can be conservatively managed, considering surgery only in cases with severe pleural cavity involvement. In cases of stricture relapse, even if such relapses occur following the execution of well
Shen, O; Sela, H Y; Nagar, H; Rabinowitz, R; Jacobovich, E; Chen, D; Granot, E
Biliary atresia is a progressive disease presenting with jaundice, and is the most common indication for liver transplantation in the pediatric population. Prenatal series have yielded conflicting results concerning a possible association between BA and prenatal nonvisualization of the gallbladder. This retrospective case series was performed to assess the association between biliary atresia, prenatal nonvisualization of the gallbladder and other sonographic signs. We identified biliary atresia patients who underwent a Kasai procedure by a single pediatric surgeon and/or follow up by a single pediatric gastroenterologist. Axial plane images and/or video recordings were scrutinized for sonographic signs of biliary atresia on the second trimester anomaly scan. Proportion of biliary atresia cases with prenatal sonographic signs. Twenty five charts of children with biliary and high quality prenatal images were retrieved. 6/25 (24%) of cases analyzed had prenatal nonvisualization of the gallbladder or a small gallbladder on the prenatal scan. Two cases had biliary atresia splenic malformation syndrome. None of the cases had additional sonographic markers of biliary atresia. Our study suggests that in addition to the well-established embryonic and cystic forms, an additional type can be suspected prenatally, which is characterized by prenatal nonvisualization of the gallbladder in the second trimester. This provides additional evidence that some cases of BA are of fetal rather than perinatal onset and may have important implications for prenatal diagnosis, for counseling and for research of the disease's etiology and pathophysiology. Copyright © 2017 Elsevier B.V. All rights reserved.
Orłowska, Ewa; Czubkowski, Piotr; Socha, Piotr
Biliary atresia is a chronic cholangiopathy leading to progressive fibrosis of both intra- and extrahepatic bile ducts. The cause of the condition is unknown. Fundamental management of biliary atresia is surgical intervention and the outcomes of the treatment depend on the child's age with best results when performed within the first 2 months of life. Thus, the main role of pediatric healthcare is an urgent differential diagnosis and prompt qualification for the surgery, optimal postoperative management and early qualification for the liver transplantation in patients with persistent cholestasis. The authors discuss the clinical presentation, diagnosis and management of biliary atresia.
Shen, Qiuxiang; Shi, Peina; Gao, Mongna; Yu, Xuechan; Liu, Yuxin; Luo, Ling; Zhu, Yabin
The mortality rate from esophageal disease like atresia, carcinoma, tracheoesophageal fistula, etc. is increasing rapidly all over the world. Traditional therapies such as surgery, radiotherapy or chemotherapy have been met with very limited success resulting in reduced survival rate and quality of patients' life. Tissue-engineered esophagus, a novel substitute possessing structure and function similar to native tissue, is believed to be an effective therapy and a promising replacement in the future. However, research on esophageal tissue engineering is still at an early stage. Considerable research has been focused on developing ideal scaffolds with optimal materials and methods of fabrication. This article gives a review of materials and scaffold fabrications currently applied in esophageal tissue engineering research. Copyright © 2013 Elsevier B.V. All rights reserved.
Souza, Rhonda F
Reflux esophagitis causes Barrett's metaplasia, an abnormal esophageal mucosa predisposed to adenocarcinoma. Medical therapy for reflux esophagitis focuses on decreasing gastric acid production with proton pump inhibitors. We have reported that reflux esophagitis in a rat model develops from a cytokine-mediated inflammatory injury, not from a caustic chemical (acid) injury. In this model, refluxed acid and bile stimulate the release of inflammatory cytokines from esophageal squamous cells, recruiting lymphocytes first to the submucosa and later to the luminal surface. Emerging studies on acute reflux esophagitis in humans support this new concept, suggesting that reflux-induced cytokine release may be a future target for medical therapies. Sometimes, reflux esophagitis heals with Barrett's metaplasia, a process facilitated by reflux-related nitric oxide (NO) production and Sonic Hedgehog (Hh) secretion by squamous cells. We have shown that NO reduces expression of genes that promote a squamous cell phenotype, while Hh signaling induces genes that mediate the development of the columnar cell phenotypes of Barrett's metaplasia. Agents targeting esophageal NO production or Hh signaling conceivably could prevent the development of Barrett's esophagus. Persistent reflux promotes cancer in Barrett's metaplasia. We have reported that acid and bile salts induce DNA damage in Barrett's cells. Bile salts also cause NF-x03BA;B activation in Barrett's cells, enabling them to resist apoptosis in the setting of DNA damage and likely contributing to carcinogenesis. Oral treatment with ursodeoxycholic acid prevents the esophageal DNA damage and NF-x03BA;B activation induced by toxic bile acids. Altering bile acid composition might be another approach to cancer prevention.
Esophageal varices Symptoms and causes By Mayo Clinic Staff Symptoms Esophageal varices usually don't cause signs and symptoms unless they bleed. Signs and symptoms of bleeding esophageal varices include: Vomiting and seeing significant amounts of blood ...
Greenhouse, David G; Breitbart, Roger E; Baird, Christopher W
Truncus arteriosus and tetralogy of Fallot with pulmonary atresia may be difficult to differentiate prenatally. We present a case that, on newborn echocardiography, angiography, and intraoperative inspection, shared features of both diagnoses.
Hułas-Stasiak, Monika; Gawron, Antoni
This study was designed to determine follicular atresia in the newborn and the prepubertal spiny mouse. We analyzed the processes of follicle loss using classical markers of apoptosis (TUNEL reaction, active caspase-3) and autophagy (Lamp1). Numerous small clear vacuoles and autophagosomes as well as strong Lamp1 staining were observed in dying oocytes of all follicle types, especially of the primordial and primary ones. Active caspase 3 and the TUNEL reaction were detected only in the granulosa cells of large secondary and antral follicles. The expression of apoptosis and autophagy markers was also changing during the prepubertal period. Western blot analysis indicated that at the moment of birth, females undergo an increased rate of follicular atresia mediated by autophagy, while apoptosis is the dominant form of ovarian atresia in consecutive postnatal days. On the basis of these observations, we concluded that apoptosis and autophagy are involved in follicular atresia and these processes are cell and developmental stage-specific.
Mithwani, Anwar Adil; Hashmi, Asif; Adil, Salman
The association between epidermolysis bullosa (EB) and pyloric atresia (PA) is rare but well documented. Herein, we report a case of EB associated with congenital PA. A female baby, weighing 1480 g, was born vaginally to a 31-year-old gravida 7 lady at 33 weeks of gestation. Polyhydramnios was detected on antenatal assessment. The parents were non-consanguineous Saudis with no family history of significant illness. At birth, well-demarcated areas of peeled skin were present over knees, left leg and periumbilical region. Systemic examination revealed no other abnormality. On second day, the patient developed recurrent vomiting and abdominal distension. An abdominal X-ray revealed a single gastric gas bubble suggesting pyloric obstruction. Following gastroduodenostomy, the baby developed severe sepsis with multiorgan dysfunction and expired on 25th day of life. Skin biopsy showed cleavage within lamina lucida. PMID:24068383
Feldman, Amy; Mack, Cara L.
The cause of biliary atresia (BA) is unknown and in the past few decades the majority of investigations related to pathogenesis have centered on virus infections and immunity. The acquired or perinatal form of BA entails a progressive, inflammatory injury of bile ducts, leading to fibrosis and obliteration of both the extrahepatic and intrahepatic bile ducts. Theories of pathogenesis include viral infection, chronic inflammatory or autoimmune-mediated bile duct injury and abnormalities in bile duct development. This review will focus solely on human studies pertaining to a potential viral trigger of bile duct injury at diagnosis and provide insight into the interplay of the innate and adaptive immune responses in the pathogenesis of disease. PMID:22800972
Dawsey, Sanford M.; Fagundes, Renato B.; Jacobson, Brian C.; Kresty, Laura A.; Mallery, Susan R.; Paski, Shirley; van den Brandt, Piet A.
The following, from the 12th OESO World Conference: Cancers of the Esophagus, includes commentaries on macronutrients, dietary patterns, and risk of adenocarcinoma in Barrett’s esophagus; micronutrients, trace elements, and risk of Barrett’s esophagus and esophageal adenocarcinoma; the role of mate consumption in the development of squamous cell carcinoma; the relationship between energy excess and development of esophageal adenocarcinoma; and the nutritional management of the esophageal cancer patient. PMID:25266021
Sanchez-Quintana, D; Climent, V; Ho, S; Anderson, R
Objective—To compare the atrial and ventricular myoarchitecture in the normal heart and the heart with tricuspid atresia, and to investigate changes in the three dimensional arrangement of collagen fibrils. Methods—Blunt dissection and cell maceration with scanning electron microscopy were used to study the architecture of the atrial and ventricular musculature and the arrangement of collagen fibrils in three specimens with tricuspid atresia and six normal human hearts. Results—There were significant modifications in the myoarchitecture of the right atrium and the left ventricle, both being noticeably hypertrophied. The middle layer of the ventricle in the abnormal hearts was thicker than in the normal hearts. The orientation of the superficial layer in the left ventricle in hearts with tricuspid atresia was irregular compared with the normal hearts. Scanning electron microscopy showed coarser endomysial sheaths and denser perimysial septa in hearts with tricuspid atresia than in normal hearts. Conclusions—The overall architecture of the muscle fibres and its connective tissue matrix in hearts with tricuspid atresia differed from normal, probably reflecting modelling of the myocardium that is inherent to the malformation. This is in concordance with clinical observations showing deterioration in pump function of the dominant left ventricle from very early in life. Keywords: tricuspid atresia; congenital heart defects; connective tissue; fibrosis PMID:9922357
Schmerber, Sébastien; Sheykholeslami, Kianoush; Kermany, Mohammad Habiby; Hotta, Shoko; Kaga, Kimitaka
In an effort to examine the rules by which information of bilaterally applied bone-conducted signals arising from interaural time differences (ITD) and interaural intensity differences (IID) is combined, data were measured for continuous 500 Hz narrow band noise at 65-70 dB HL in 11 patients with bilateral congenital aural atresia. Time-intensity trading functions were obtained by shifting the sound image towards one side using ITD, and shifting back to a centered sound image by varying the IID in the same ear (auditory midline task). ITD values were varied from -600 to +600 micros at 200 micros steps, where negative values indicate delays to the right ear. The results indicate that time-intensity trading is present in patients with bilateral aural atresia. The gross response properties of time-intensity trading in response to bone-conducted signals were comparable in patients with bilateral aural atresia and normal-hearing subjects, though there was a larger inter-subject variability and higher discrimination thresholds across IIDs in the atresia group. These results suggest that the mature auditory brainstem has a potential to employ binaural cues later in life, although to a restricted degree. A binaural fitting of a bone-conducted hearing aid might optimize binaural hearing and improve sound lateralization, and we recommend now systematically bilateral fitting in aural atresia patients.
Wildhaber, Barbara E.
Biliary atresia is a rare neonatal disease of unknown etiology, where obstruction of the biliary tree causes severe cholestasis, leading to biliary cirrhosis and death in the first years of life, if the condition is left untreated. Biliary atresia is the most frequent surgical cause of cholestatic jaundice in neonates and should be evoked whenever this clinical sign is associated with pale stools and hepatomegaly. The treatment of biliary atresia is surgical and currently recommended as a sequence of, eventually, two interventions. During the first months of life a hepatoportoenterostomy (a “Kasai,” modifications of which are discussed in this paper) should be performed, in order to restore the biliary flow to the intestine and lessen further damage to the liver. If this fails and/or the disease progresses towards biliary cirrhosis and life-threatening complications, then liver transplantation is indicated, for which biliary atresia represents the most frequent pediatric indication. Of importance, the earlier the Kasai is performed, the later a liver transplantation is usually needed. This warrants a great degree of awareness of biliary atresia, and the implementation of systematic screening for this life-threatening pathology. PMID:23304557
Sanchez-Quintana, D; Climent, V; Ho, S Y; Anderson, R H
To compare the atrial and ventricular myoarchitecture in the normal heart and the heart with tricuspid atresia, and to investigate changes in the three dimensional arrangement of collagen fibrils. Blunt dissection and cell maceration with scanning electron microscopy were used to study the architecture of the atrial and ventricular musculature and the arrangement of collagen fibrils in three specimens with tricuspid atresia and six normal human hearts. There were significant modifications in the myoarchitecture of the right atrium and the left ventricle, both being noticeably hypertrophied. The middle layer of the ventricle in the abnormal hearts was thicker than in the normal hearts. The orientation of the superficial layer in the left ventricle in hearts with tricuspid atresia was irregular compared with the normal hearts. Scanning electron microscopy showed coarser endomysial sheaths and denser perimysial septa in hearts with tricuspid atresia than in normal hearts. The overall architecture of the muscle fibres and its connective tissue matrix in hearts with tricuspid atresia differed from normal, probably reflecting modelling of the myocardium that is inherent to the malformation. This is in concordance with clinical observations showing deterioration in pump function of the dominant left ventricle from very early in life.
Esteves, Edward; Clemente Neto, Eriberto; Ottaiano Neto, Miguel; Devanir, José; Esteves Pereira, Ruy
Conventional surgery for extrahepatic bile-duct atresia (EHBDA) usually requires a large, painful, muscle-cutting laparotomy, dislodgment of the liver, and wide manipulations, followed by adhesions and possible complications that may disturb the postoperative course and hamper liver transplantation (LT). The main role of laparoscopy in EHBDA has been for diagnostic purposes. Besides all the advantages of minimally-invasive access, it allows excellent visibility and dissection of tiny hilar structures. The authors present the first two cases of successful Roux-en-Y laparoscopic portoenterostomy (LARP) for EHBDA, showing the importance of advanced technical skills and a new approach for extracorporeal enteroanastomosis. Laparoscopic hilar dissection and portoenterostomy was accomplished using four trocars. The umbilical site was used for extracorporeal Roux-en-Y enteroenterostomy, in the first case using a laparoscopic stapler and in the second a hand-sewn suture. Mean operative time was 190 min, and no operative complications were observed. Both girls became anicteric. The first is doing well 15 months after the operation with good hepatic function. The other was anicteric for 6 months, had one episode of cholangitis, developed an umbilical hernia, has shown slow and progressive hepatic failure, and is now being evaluated for possible LT. It is concluded that LARP for EHBDA can be done safely in infants using an extracorporeal transumbilical enteric anastomosis, with several advantages compared with open surgery. The role of LARP in facilitating LT is yet to be defined.
Rosado-Buzzo, A A; Santamaría-Díaz, H; Gómez-Gómez, M; Alva-Espinosa, C; Maulen-Radovan, X; Palacios-Macedo, X
We describe the clinical course of 120 children with tricuspid atresia (TA) attended in the Hospital de Cardiología y Neumología, "Dr. Luis Méndez", del Centro Médico Nacional. There were 61 males and 59 females. The age of presentation was in 79 newborn babies, seventeen between one and six months old, 20 between six and 24 months old, and four with two or more years old. The clinical picture was hypoxic spells in 89% and congestive heart failure in the others. The chest film showed cardiomegaly in 85%, with diminished pulmonary flow in 48%, increased flow in 27.5%, and normal in 9%. The electrocardiogram with superior left axis deviation in 94%, right atrial hypertrophy in 58%, left atrial hypertrophy in 47.5% and left ventricular hypertrophy in 96%. TA was classified as type I in 103 children, type Ic in 70, Ib in 27 and Ia in six, and type II in seventeen children, with eight IIc, six IIb and three IIa. In 44 the management was medical, 63 underwent systemic-pulmonary anastomosis, 37 of them with Blalock-Taussig shunt and Fontan procedure in thirteen children. There were 21 deaths. This survey is compared with the literature and from this point we make management recommendations.
Godart, F; Fall, A L; Francart, C; Brevière, G M; Cajot, M A; Rey, C
We report this centre's experience of the treatment and follow up of pulmonary atresia with an intact septum. 35 infants were seen during the neonatal period. Opening via catheterisation was attempted in 21 patients with initial success in 10, and 11 failures leading to urgent surgery (surgical opening, n=9) and isolated Blalock-Taussig anastomosis (n=2). Fourteen other patients underwent immediate surgery: surgical opening, n=3, and Blalock alone, n=11. Four patients died in the neonatal period: 1 after successful opening via catheterisation, 3 others after Blalock anastomosis. Five others with a Blalock anastomosis died suddenly later. By the end of follow up, 16 patients had undergone biventricular type repair, of which 7 required additional procedures. 10 others had undergone cavo-pulmonary type repair, including 4 infants in whom the initial strategy of biventricular repair had failed. The only predictive factors at birth for subsequent progression to biventricular type repair were: larger tricuspid diameter at echography (10.9 +/- 2.25 mm versus 6.34 +/- 1.74 mm, p = 0.0007) or at angiography (10.07 +/- 2.09 mm versus 8.04 +/- 2.42 mm, p = 0.039), and the right ventricular morphology (p = 0.0011) with more tripartite ventricles, and less bipartite or even unipartite ones in the biventricular group.
Ziv, Y; Lombrozo, R; Dintsman, M
In a 7-day-old infant referred because of bile-stained vomiting, jaundice and lack of meconium, radiological examination revealed the 'double-bubble' sign of duodenal atresia as well as dextrocardia. This infant also had a strawberry haemangioma on the right shoulder. Operation disclosed situs inversus and a preduodenal portal vein as well as duodenal atresia. A side-to-side duodeno-jejunostomy was performed successfully without damage to the anomalous vein. The history of polyhydramnion during gestation, the presence of other anomalies, the rapid onset of bile-stained vomiting and the classic 'double-bubble' sign, together appeared to indicate that the duodenal atresia was intrinsic and not due to the external pressure of the anomalous vein on the duodenum.
Ximenes, Manoel; Piauilino, Marcos Amorim; Oliveira, Humberto Alves; Vaz Neto, Jorge Pinto
We describe the case of a 54 year old woman seen with an esophageal mass diagnosed as a primary esophageal lymphoma. The main symptom was dysphagia of seven months duration. The treatment consisted in resection of the tumor, and reconstruction of the defect with a reversed pleural flap, followed by a chemotherapy regimen that consisted of five drugs, cyclophosphamid, prednisone, doxorubicin, rituximab and vincristine (R-CHOP). The patient developed an esophageal pleural fistula treated with pleural drainage and irrigation that closed in 45 days. Two and one half years later she is doing well and disease free.
Adenocarcinoma of the Gastroesophageal Junction; Esophageal Adenocarcinoma; Stage IB Esophageal Cancer; Stage IIA Esophageal Cancer; Stage IIB Esophageal Cancer; Stage IIIA Esophageal Cancer; Stage IIIB Esophageal Cancer
Manica, Denise; Schweiger, Cláudia; Netto, Cátia C Saleh; Kuhl, Gabriel
Introduction Although it has been more than 250 years since the first description of choanal atresia (CA), there are still doubts about this abnormality. The differences between unilateral and bilateral forms are seldom discussed. Objectives Aggregate data from patients diagnosed with CA, grouping patients with unilateral and bilateral forms. Methods Retrospective study. Results Eighteen patients were included: 12 (66.6%) presented bilateral atresia, of which 77.8% were mixed bony-membranous type and 22.2% were pure bony type. From the 12 patients with bilateral atresia, 10 presented related malformations, 3 of whom had CHARGE syndrome (coloboma, heart defects, choanal atresia, retardation of growth and development, genitourinary problems, ear abnormalities). From the remaining 6 patients with unilateral atresia, only 2 showed malformations, 1 renal and 1 cardiac. All patients with unilateral atresia needed only 1 surgical procedure, and patients with the bilateral form needed a median of 2.85 interventions (p = 0.003). The median age of surgical procedure in the unilateral group was 6 years, ranging from 6 months to 18 years, and in the bilateral group was 25 days, ranging from 6 days to 6 years (p = 0.003). The median interval between diagnosis and surgery was 9 months in the unilateral group, ranging from 1 month to 18 years, and in the bilateral group was 1 day, ranging from 1 day to 2 months (p = 0.001). Discussion and Conclusions Success rates with the endoscopic approach vary from 62 to 100%. Nonetheless, most of these reports present results without considering the number of compromised sides. In our opinion, unilateral and bilateral cases involve distinct patients (taking into account the related malformations), have diverging clinical presentations, and show discrepant restenosis rates and therefore could be considered in different groups of analysis. PMID:25992054
O'Sullivan, O. E.; Crosby, D.; Byrne, B.; Regan, C.
Biliary atresia is a rare idiopathic neonatal cholestatic disease characterized by the destruction of both the intra- and extrahepatic biliary ducts. As the disease is progressive all cases will develop portal fibrosis, cirrhosis, and portal hypertension with the sequelae of varices, jaundice, and eventually liver failure requiring a transplant. Survival rates have improved considerably with many females living well in to be childbearing age. Due to the complexity of the disease these pregnancies are considered, high risk. We report the antenatal, intrapartum, and postpartum managements of a pregnancy complicated by biliary atresia. Furthermore, we highlight the importance of a multidisciplinary team approach in optimizing obstetric care for this high risk group. PMID:24459595
Asma, A; Roslenda, A R; Fadzilah, I; Mazita, A; Marina, M B; Ab Aziz, A
A six-month-old baby with congenital patent ductus arteriosus (PDA), bilateral microtia and canal atresia was referred for hearing assessment. The audiology assessment revealed bilateral profound hearing loss, which is atypical for a case of pure canal atresia. Imaging was performed much earlier than usual and, as suspected, the patient also had bilateral severe inner ear anomaly. It is extremely rare for a person to have both external and inner ear anomaly because of the different embryological origin. The only suitable hearing rehabilitation option for this kind of patients is brainstem implant. However, the parents had opted for sign language as a form of communication.
Kimble, Rebecca; Molloy, Genevieve; Sutton, Bridget
The objective of this study was to report 2 cases of the combined congenital anomalies of complete vaginal atresia and partial cervical agenesis, and highlight the limitations of magnetic resonance imaging for definitive initial diagnosis, and consequently the importance of early definitive management, to avoid life-threatening sepsis. Herein we provide a retrospective case audit of two patients with congenital abnormalities between 2005 and 2013 who were treated in a quaternary statewide pediatric and adolescent gynecology center. Two patients with the combined congenital anomalies of complete vaginal agenesis and partial cervical agenesis highlight the difficulties encountered with the limitations of magnetic resonance imaging in accuracy of diagnosis, as well as development of life-threatening sepsis that requires hysterectomy. Both patients were initially imaged as having distended endometrial cavities and cervical canals with what was thought to be an obstructive upper vaginal septum and absent lower vagina. Both required initial neovagina creation, however the cervices were never clinically or surgically visualized. Partial cervical agenesis is a relatively rare form of Müllerian abnormality which, if not diagnosed and definitively treated early, can have significant morbidity and mortality. Although magnetic resonance imaging is the diagnostic imaging gold standard for Müllerian abnormalities, it is important to recognize the limitations of this modality, the potential sequelae of these limitations, and to appreciate the importance of early accurate diagnosis and treatment of this condition. Importantly, if the imaging diagnosis does not completely correlate with the clinical and surgical findings, then a high suspicion of complete or partial cervical agenesis is prudent, because the consequences of nondefinitive early treatment can be life-threatening and potentially fatal. Crown Copyright © 2016. Published by Elsevier Inc. All rights reserved.
Biliary atresia (BA) is the major cause of cholestasis and the leading indication for liver transplantation (LT). However, the incidence of BA in Korea has not been reported. The aim of this study was to investigate the incidence and clinical outcomes of BA in Korea. We used the Korean universal health insurance database and extracted data regarding BA patients younger than 18 years of age admitted between 2011 and 2015. The incidence of BA was calculated by dividing the number of BA patients by the number of live births. Two hundred forty infants were newly diagnosed with BA. A total of 963 BA patients younger than 18 years of age were followed up for 5 years. The overall incidence of BA was 1.06 cases per 10,000 live births. The incidence of BA was 1.4 times higher for female patients than for male patients. Additionally, significant seasonal variation was observed; in particular, the incidence of BA was 2 times higher from June through August than from December through February. Congenital anomalies were found in 38 of 240 patients (15.8%). Congenital heart diseases were major associated congenital anomalies (6.3%). Several complications developed during the study period, including cholangitis (24.0%), varix (6.2%), and gastrointestinal bleeding (4.4%). Three hundred and one of the 963 BA patients under 18 years of age (31.3%) received LT for BA. The incidence of BA is higher in Korea than that in Western countries. We also report significant gender-associated differences and seasonal variation with respect to the incidence of BA. PMID:28244293
Ksia, Amine; Zitouni, Hayett; Zrig, Ahmad; Laamiri, Rachida; Chioukh, Fatma; Ayari, Eya; Sahnoun, Lassaad; Maazoun, Kais; Krichene, Imed; Mekki, Mongi; Belghith, Mohsen; Nouri, Abdellatif
Pyloric atresia (PA) is uncommon. It occurs in 1:100000 live births. Neonates usually present soon after birth with copious non-bilious vomiting. The treatment is surgical and its prognosis is poor, especially, when it is associated with epidermolysis bullosa (EB). The aim of this study was to evaluate the clinical presentation, diagnosis, operative management, post-operative courses, and outcome in infant with PA, based in our cases and literature review. Charts of 10 patients who underwent surgery for PA in the department of paediatric surgery in a Teaching Hospital in Tunisia (Monastir) between 1990 and 2012 were reviewed. Data were analysed for demographic, clinical, therapeutic, and prognostic characteristics. The average of age at presentation was 2 days and there were six males and four females. The main presenting symptoms were non-bilious vomiting in 90% of cases. Abdominal X-ray showed gastric dilatation with an absence of gas in the rest of the intestinal tract in 90%, and a pneumoperitoneum in one. The surgical approach was laparotomy in all cases. Gastric perforation was observed in one patient and was completely repaired. The distribution of the anatomic variations was type A in nine cases and type B in one. Five patients underwent excision of the diaphragm and Heineke-Mikulicz pyloroplasty and gastroduodenostomy in the other five cases. Identified associated anomalies were Down's syndrome in one and EB in 2 (20%), one family has three affected sibling. Post-operative mortality rate was 70%. No standard surgical approach can be adopted a better management of PA or the associated anomalies depends on an early diagnosis and the availability of neonatal intensive care unit.
... establish common inputs for current and hypothetical screening strategies, chemoprevention and endoscopic therapy. One project aims to advance the understanding of esophageal cancer and the impact of cancer control interventions through a collaborative and comparative modeling project. ...
Putnam, Philip E
Esophagitis is the end result of a variety of insults to epithelial homeostasis. Eosinophilic esophagitis is a manifestation of non-IgE-mediated food allergy that most commonly affects the esophagus of males who have other atopic phenomena. Reflux esophagitis reflects repeated exposure to acidic gastric contents because of failure of the normal protections afforded by the LES. Because certain histologic features can be present in either condition, endoscopic biopsy alone does not distinguish them. Their symptoms overlap, but the treatment options are very different, such that making a formal diagnosis by following consensus guidelines is essential. A treatment protocol designed to manage the inflammation by controlling the provocative factors (acid for GERD and food antigens for EoE) or suppressing the inflammation (ie, topical steroids for EoE) should result in normalization of the mucosa and resolution of symptoms. Eosinophilic esophagitis is a chronic condition that rarely remits spontaneously, so any therapeutic modality will need to be continued indefinitely.
Belío-Castillo, C; Bracho-Blanchet, E; Blanco-Rodríguez, G
The congenital or acquired variety of esophageal diverticulum is a rare childhood disease. Reported is a case-study which deals with a five year old patient who arrived at our hospital complaining of a constricture at the cricopharyngeal level. A month later he returned to the hospital with dysphasia and regurgitation; the X-rays and endoscopic diagnosis showed stenosis of the esophagus and the presence of an esophageal diverticulum. Esophageal dilatations were carried out until an acceptable esophageal diameter was reached. Later on, the surgical removal of the diverticulum was performed without any complications. The histopathological study showed the congenital nature of the diverticulum. The patient's recovery went well and is currently considered as cured.
Aziz, Qasim; Fass, Ronnie; Gyawali, C Prakash; Miwa, Hiroto; Pandolfino, John E; Zerbib, Frank
Functional esophageal disorders consist of a disease category that present with esophageal symptoms (heartburn, chest pain, dysphagia, globus) not explained by mechanical obstruction (stricture, tumor, eosinophilic esophagitis), major motor disorders (achalasia, EGJ outflow obstruction, absent contractility, distal esophageal spasm, jackhammer esophagus), or gastroesophageal reflux disease (GERD). While mechanisms responsible are unclear, it is theorized that visceral hypersensitivity and hypervigilance play an important role in symptom generation, in the context of normal or borderline function. Treatments directed at improving borderline motor dysfunction or reducing reflux burden to sub-normal levels have limited success in symptom improvement. In contrast, strategies focused on modulating peripheral triggering and central perception are mechanistically viable and clinically meaningful. However, outcome data from these treatment options are limited. Future research needs to focus on understanding mechanisms underlying visceral hypersensitivity and hypervigilance so that appropriate targets and therapies can be developed.
Noyer, C M; Simon, D
This article focused on the approach to oral and esophageal disorders in patients with AIDS. Most of these disorders respond to various therapeutic regimens. Some of the oral complications can be prevented with dental prophylaxis, whereas recurrent esophageal disease in some patients may require long-term suppressive therapy. As patients with AIDS live longer with lower CD4 counts, gastroenterologists need to become familiar with the approach to and management of the more common lesions of the mouth and esophagus.
chemokine production13,18; however, it has been observed that activated mast cells can also express TSLP to similarly drive TH2 responses. 12,19,20 Indeed...including eosinophils 61 and mast cells .62 For instance, TSLP activates dendritic cells to adopt a TH2 prim- ing phenotype through the secretion of the...binding pattern to lysates from esophageal epithelial cells , as well as esophageal tissue from patients with active EE. This analysis has not shown
Barsness, Katherine A; Rooney, Deborah M; Davis, Lauren M; Chin, Anthony C
A validated high fidelity simulation model would provide a safe environment to teach thoracoscopic EA/TEF repair to novices. The study purpose was to evaluate validity evidence for performance measures on an EA/TEF simulator. IRB-exempt data were collected from 12 self-reported "novice" and 8 "experienced" pediatric surgeons. Participants evaluated the EA/TEF repair simulator using survey ratings that were analyzed for test content validity evidence. Additionally, deidentified operative performances were videotaped and independently rated by two surgeons using the Objective Structured Assessment for Technical Skills (OSATS) instrument. Novice and experienced OSATS were compared with p<.05 significant. Participants had high overall simulator ratings. Internal structure was supported by high interitem consistency (α=.95 and .96) and interrater agreement (ICC) [.52, .84] for OSATS ratings. Experienced surgeons performed at a significantly higher level than novices for all five primary and two supplemental OSATS items (p<.05). Favorable participant ratings indicate the simulator is relevant to clinical practice and valuable as a learning tool. Further, performance ratings can discriminate experienced and novice performances of EA/TEF repair. These findings support the use of the simulator for performance assessment, representing the first validated measures from a simulator intended for pediatric surgical training. Copyright © 2014 Elsevier Inc. All rights reserved.
Symbas, P. N.; Hatcher, C. R.; Vlasis, S. E.
During a 15-year period from August 1964 to August 1979, 48 patients with gunshot wound of the esophagus (24 of the cervical, 17 of the thoracic, and seven of the abdominal) were treated at Grady Memorial Hospital. In the majority of the patients, the initial history, physical findings, and chest roentgenograms were nondiagnostic for esophageal injury. Esophageal perforation was mainly suspected because the bullet tract was in close proximity to the esophagus or the bullet had traversed the mediastinum. The diagnosis of esophageal perforation was made by esophagography (29 patients), at the time of emergency surgical exploration for suspected other organ injuries (17 patients), or by esophagoscopy (one patient). All but one patient were treated surgically. The surgical procedure most commonly used was primary repair of the esophageal wound with wide drainage of the mediastinum. Thirty-eight (79.2%) of the 48 patients survived, 21 (87.5%) of the 24 patients with cervical, 11 (64.7%) of the 17 patients with thoracic, and six (85.7%) of the seven patients with abdominal esophageal wounds. Ten patients died, three with cervical wound, six with thoracic wound, and one with abdominal esophageal wound. Three patients died intraoperatively from major bleeding and the remaining seven died from the esophageal and/or other associated injuries, four to eight days after surgery. None of the seven patients who underwent primary repair with wide drainage and plication of the suture line with pleural flap or other tissue, died or developed leak at the suture line. This study suggests that the physical and roentgenographic findings in patients with esophageal injury are often nondiagnostic and frequently are masked by coincidental injury to other organs. Hence, a high index of suspicion is required for the diagnosis of esophageal injury from gunshot wounds and esophagography should be performed as soon as the patient's condition is stable in all patients who present with a missile
Ba, I D; Ba, A; Thiongane, A; Ly/Ba, A; Ba, M; Fattah, M; Faye, P M; Cissé, D F; Diouf, F N
Malignant infantile osteopetrosis is a rare genetic disease characterized by increased bone density due to osteoclastic dysfunction. We report on the case of a 3-month-old girl who was referred to our hospital by the ENT department for severe anemia in the context of bilateral choanal atresia. Clinical examination showed failure to thrive, anemia, respiratory distress, bilateral choanal atresia, and chest deformation. The abdomen was soft with large hepatosplenomegaly. We noted a lack of eye tracking, no optical-visual reflexes, and left nerve facial paralysis. The blood count showed normocytic normochromic anemia with severe thrombocytopenia. The infectious work-up and blood smears were negative. The skeleton X-ray showed diffuse bone densification of the skull, long bones, pelvis, vertebrae, and ribs. The facial bone CT confirmed membranous choanal atresia. The molecular biology search for the TCIRG1 gene mutation was not available. The patient had supportive treatment (transfusion, oral steroid, vitamin D, oxygen, nutrition). Bone marrow transplantation was indicated but not available. She died at 6 months in a context of severe anemia and bleeding. Malignant infantile osteopetrosis is rare and symptoms are nonspecific. Diagnosis should be considered in young infants presenting refractory anemia, particularly in the context of choanal atresia. Bone marrow transplantation remains the only curative treatment.
Shankar, Raghu; Rao, Sadashiva P.; Shetty, Kishan B.
We report a rare association of duodenal atresia with situs inversus abdominus in a newborn. The infantogram revealed “reverse double-bubble sign” without dextrocardia. The sonography and echocardiography confirmed the diagnosis of situs inversus abdominus with multiple cardiac anomalies. Laparotomy and a duodenoduodenostomy were carried out. PMID:22529552
Hamilton, J R; Fonseka, S F; Wilson, N; Dickinson, D F; Walker, D R
In six infants with pulmonary atresia and intact ventricular septum operative balloon dilatation was used to achieve continuity between the right ventricle and the main pulmonary artery as the initial procedure. Two of the six subsequently needed an aortico pulmonary shunt. All six are alive and well. Images Fig PMID:3676024
Ducharme, Norm G.; Arighi, Mimi; Horney, F. Don; Barker, Ian K.; Livesey, Michael A.; Hurtig, Mark H.; Johnson, Roger P.
This prospective study was initiated to document the success rate obtained in the treatment of colonic atresia in calves, identify factors that influence survival rate, and to report the histopathological appearance of the proximal blind end of the ascending colon. Forty-three calves with intestinal obstruction due to colonic atresia were admitted to the Ontario Veterinary College between September 1982 and May 1986. Parameters recorded prospectively in this study included age, breed, sex, history, vital signs, acid-base and electrolyte status, location of intestinal atresia, medical and surgical management, and outcome. The typical history and clinical signs included failure to pass meconium or feces, decreased appetite, and progressive depression and abdominal distension. The most common site of colonic atresia was the midportion of the spiral loop of the ascending colon (n = 25). Of the 43 calves, three (7%) were euthanized at surgery, 21 (49%) died in the hospital, and 19 (44%) survived and were discharged from the hospital. Four of the surviving calves died subsequent to discharge giving an overall long-term (mean 15.9 months) survival rate of 35%. No significant risk factors were identified, although experienced surgeons showed a trend towards increased survival rate. ImagesFigure 2.Figure 3.Figure 4.Figure 5. PMID:17423141
Yamataka, Atsuyuki; Lane, Geoffrey J; Cazares, Joel
Minimally invasive surgery in children has evolved to the extent that complex procedures can be performed with safety and outcome comparable with open surgery, with the advantage of minimal scarring. Here we describe the latest laparoscopic techniques used by us at the Juntendo University Hospital, Japan, for treating biliary atresia and choledochal malformation, with presentation of our postoperative management and discussion of preliminary outcomes.
Goldsztein, Hernan; Roberson, Joseph B
Describe intraoperative facial nerve findings in 209 consecutive atresia cases. Identify preoperative and intraoperative anatomical variants that should alert the surgeon to potential high-risk facial nerve anatomy. Case series with chart review. Tertiary care subspecialty private practice. Retrospective review of 209 consecutive atresia cases treated between 2007 and 2011. Descriptive analysis of intraoperative findings. Logistical regression models with generalized estimating equations were used to examine the effect of preoperative variables over the operative findings. Two hundred and nine consecutive patients (ages 2-48) underwent atresia repair between 2007 and 2011. Preoperative Jahrsdoerfer Scale was 9 (23%), 8 (42%), 7 (19%), 6 (2%), 5 or less (2%). The facial nerve was found to have an abnormal course in 39% of the cases and not identified in 1%. It was congenitally dehiscent in 53% of cases and was surgically exposed in 10%. The most common site of congenital dehiscence was in the tympanic segment (57%). Facial-stapes contact was found in 11% of cases. The stapedius tendon was absent in 30% of cases. A single patient had a mild transient postoperative paresis (House-Brackmann 2). Atresia repair remains one of the most challenging procedures in otology. In spite of modern preoperative imaging, the facial nerve remains at risk. When performing surgery on patients with preoperative facial nerve paresis and/or lower Jahrsdoerfer scores, the surgeon should be aware of a higher incidence of facial nerve abnormalities. Thorough knowledge of anatomical variations and meticulous surgical technique are mandatory to safely perform these surgeries.
Minior, Victoria K; Gagner, Jean-Pierre; Landi, Kristen; Stephenson, Courtney; Greco, M Alba; Monteagudo, Ana
Laryngeal atresia is a rare, life-threatening congenital malformation. Prenatal sonographic diagnosis has been described; however, in many cases, the precise diagnosis is established only at autopsy. Our aim was to describe an atypical prenatal presentation of congenital laryngeal atresia in which the final diagnosis was made only at autopsy. Sonographic and postmortem examinations were performed on a fetus with bilateral enlarged echogenic lung fields, an everted diaphragm, and polyhydramnios, which were initially noted on prenatal sonography at 26 weeks' gestation. Unfortunately, the mother was lost to follow up at our inner-city clinic, thus precluding a definitive diagnosis. At birth, tracheostomy was not performed because of the anomalous appearance of the neonate and the suspicion of multiple congenital anomalies. Neonatal death occurred after 18 minutes of life. Autopsy revealed laryngeal atresia with a right hemidiaphragmatic defect and multiple other congenital anomalies. This was a rare case with prenatal sonographic findings in a fetus with congenital laryngeal atresia associated with partial diaphragmatic obliteration.
van Klaveren, R J; Morshuis, W J; Lacquet, L K; Cox, A L; Festen, J; Heystraten, F M
Two cases of congenital bronchial atresia with pectus excavatum are reported. Costosternal retraction during the efforts to overcome the airway obstruction due to encroachment on normal lung tissue by the hyperinflated segments may play a part in causing pectus excavatum. Images PMID:1494776
Hirano, Ikuo; Aceves, Seema S.
In eosinophilic esophagitis (EoE), remodeling changes are manifest histologically in both the epithelium as well as in the subepithelium where lamina propria (LP) fibrosis, expansion of the muscularis propria and increased vascularity occur. The major clinical symptoms and complications of EoE are largely consequences of esophageal remodeling. Important mediators of the process include IL-5, IL-13, TGFβ1, mast cells, fibroblasts and eosinophils. Methods to detect remodeling effects include upper endoscopy, histopathology, barium esophagram, endoscopic ultrasonography, esophageal manometry, and functional luminal imaging. These modalities provide evidence of organ dysfunction that include focal and diffuse esophageal strictures, expansion of the mucosa and subepithelium, esophageal motor abnormalities and reduced esophageal distensibility. Complications of food impaction and perforations of the esophageal wall have been associated with reduction in esophageal caliber and increased esophageal mural stiffness. The therapeutic benefits of topical corticosteroids and elimination diet therapy in resolving mucosal eosinophilic inflammation of the esophagus are evident. Available therapies, however, have demonstrated variable ability to reverse existing remodeling changes of the esophagus. Systemic therapies that include novel, targeted biologic agents have the potential of addressing subepithelial remodeling. Esophageal dilation remains a useful, adjunctive therapeutic maneuver in symptomatic adults with esophageal stricture. As novel treatments emerge, it is essential that therapeutic endpoints account for the fundamental contributions of esophageal remodeling to overall disease activity. PMID:24813517
van der Gaag, I; Tibboel, D
Intestinal atresia was found in 29 animals and stenosis in five. Atresia was found in the duodenum in one pup; in the jejunum in nine calves, two lambs and one piglet; in the ileum in one pup, one lamb and one piglet; and in the colon in one foal, seven calves, one lamb, one piglet and three kittens. Stenosis was found in the duodenum of a foal, in the jejunum in two calves and one pup, and in both the ileum and the colon of a kitten. One lamb showed ileal atresia as well as ileal stenosis. We classified the atresia as type 1, membrane atresia (four cases); type 2, cord atresia (six cases); and type 3, blind-end atresia (19 cases). In the literature and our own study, atresia was found only in the small intestine in puppies, but in foals and kittens it was found only in the colon. Atresia of the colon is more common in animals than in man, but multiple atresia, frequent in man, is rare in animals.
Sundaram, Shikha S.; Alonso, Estella M.; Haber, Barbara; Magee, John C.; Fredericks, Emily; Kamath, Binita; Kerkar, Nanda; Rosenthal, Philip; Shepherd, Ross; Limbers, Christine; Varni, James W.; Robuck, Patricia; Sokol, Ronald J.; Liver, Childhood
Objectives To quantify health related quality of life (HRQOL) of patients with biliary atresia with their native livers and compare them with healthy children and patients with biliary atresia post-liver transplant (LT) and to examine the relationship between HRQOL and medical variables. Study design A cross-sectional HRQOL study of patients with biliary atresia with their native livers (ages 2-25 years) was conducted and compared with healthy and post-LT biliary atresia samples using PedsQL™ 4.0 child self and parent proxy reports, a validated measure of physical/psychosocial functioning. Results 221 patients with biliary atresia with native livers (54% female, 67% white) were studied. patient self and parent proxy reports showed significantly poorer HRQOL than healthy children across all domains (p < 0.001), particularly in emotional and psychosocial functioning. Child self and parent proxy HRQOL scores from patients with biliary atresia with their native livers and post-LT biliary atresia were similar across all domains (p=NS). Child self and parent proxy reports showed moderate agreement across all scales, except social functioning (poor to fair agreement). On multivariate regression analysis, black race and elevated total bilirubin were associated with lower Total and Psychosocial HRQOL summary scores. Conclusions HRQOL in patients with biliary atresia with their native livers is significantly poorer than healthy and similar to post-LT biliary atresia children. These findings identify significant opportunities to optimize the overall health of patients with biliary atresia. PMID:23746866
Luc, Guillaume; Durand, Marlène; Collet, Denis; Guillemot, Fabien; Bordenave, Laurence
Esophageal tissue engineering is still in an early state, and ideal methods have not been developed. Since the beginning of the 20th century, advances have been made in the materials that can be used to produce an esophageal substitute. Three approaches to scaffold-based tissue engineering have yielded good results. The first development concerned non-absorbable constructs based on silicone and collagen. The need to remove the silicone tube is the main disadvantage of this material. Polymeric absorbable scaffolds have been used since the 1990s. The main polymeric material used is poly (glycolic) acid combined with collagen. The problem of stenosis remains prevalent in most studies using an absorbable construct. Finally, decellularized scaffolds have been used since 2000. The promises of this new approach are unfulfilled. Indeed, stenosis occurs when the esophageal defect is circumferential regardless of the scaffold materials. Cell supplementation can decrease the rate of stenosis, but the type(s) of cells and their roles have not been defined. Finally, esophageal tissue engineering cannot provide a functional esophageal substitute, and further development is necessary prior to conducting human clinical studies.
Patel, Ashish; Suntharalingam, Mohan
Radiotherapy and surgery have both played prominent roles in the treatment of esophageal cancer since the beginning of the 20th century. Although the use of radiotherapy alone to treat esophageal cancer has a long history, it has not demonstrated improved outcomes compared with surgery alone. The disappointing rates of survival and local control associated with single-modality therapy and the need for effective nonsurgical management led to the development of definitive chemoradiotherapy paradigms for esophageal cancer. Adding cytotoxic chemotherapy to radiotherapy for additive or synergistic effect was described as early as 1968, and over time, treatment has shifted from single-modality therapy toward combined-modality therapy using chemotherapy and radiotherapy. This approach eventually demonstrated superior outcomes in patients with esophageal cancer when compared to radiotherapy alone. Maximum benefit of this therapy depends on the appropriate addition of surgery and the optimization of radiosensitizing chemotherapy. A burgeoning area of research has focused on improving definitive chemoradiotherapy strategies through the incorporation of newer chemotherapeutic agents and targeted biologic agents. An overview of the history of chemoradiotherapy in the treatment of esophageal cancer is presented, as well as a discussion of ongoing studies and future areas of promising research. PMID:19461907
Kozak, Katarzyna; Rębowski, Marek; Kozak, Józef
Introduction Intramural esophageal tumors (IET) are located between unchanged mucous membrane and muscularis mucosae. They can be both benign and malignant. Aim To evaluate diagnostic and therapeutic difficulties of IET. Material and methods During the years 2010–2015, 11 patients with IET were treated in our clinic. Diagnostics included gastroscopy, computed tomography of the chest, endoscopic ultrasound (EUS) guided fine needle biopsy, and positron emission tomography (PET) of the esophagus in cases with no histopathological confirmation. Results Based on the conducted analysis we diagnosed 1 case of gastrointestinal stromal tumor (GIST), 1 case of adenocarcinoma, and 2 cases of esophageal cysts. In another 7 cases radiological images resembled leiomyoma but with no histopathological confirmation. Esophagectomy was performed in 2 cases of malignant tumors and 1 case of a large benign tumor. In other cases surgical enucleation of tumors was performed. Postoperatively we diagnosed 6 cases of leiomyoma, 1 case of schwannoma, 2 esophageal cysts, 1 case of GIST and 1 of esophageal cancer. Conclusions Intramural esophageal tumors is a very diverse group of tumors, both malignant and benign. In every case of IET we should seek histopathological conformation. Treatment of IET depends on localization, size and histopathological type of lesion. PMID:28096828
Chen, Huan-Wen; Du, Ming
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.
Dysphagia, or difficulty swallowing, is a common problem. Although most cases are attributable to benign disease processes, dysphagia is also a key symptom in several malignancies, making it an important symptom to evaluate. The differential diagnosis of dysphagia requires an understanding of deglutition, in particular the oropharyngeal versus esophageal stages. Stroke is the leading cause of oropharyngeal dysphagia, which is common in older adults and frequently presents as part of a broader complex of clinical manifestations. In esophageal dysphagia, difficulty swallowing is often the main complaint and is caused by localized neuromuscular disorders or obstructive lesions.
Nusrat, Salman; Miner, Philip B
Dramatic progress has been made over the past decade in the sophistication and availability of equipment to test esophageal motility and sensation. High-resolution esophageal manometry and impedance have moved from the research clinic into clinical practice. Some of the testing is costly and time consuming, and requires extensive experience to perform the testing and properly interpret the results. These sensory studies are valuable in the interpretation of clinical problems, and provide important research information. Clinicians should evaluate the research studies to advance their understanding of the pathophysiology of the esophagus. Copyright © 2014 Elsevier Inc. All rights reserved.
Kumar, Manish; Thomas, Niranjan
Oesophageal atresia/trachea-Oesophageal fistula is commonly diagnosed in the newborn period by inability to pass a nasogastric tube (NGT). We present the instance of a newborn baby where the diagnosis of oesophageal atresia was delayed because of an apparent successful passage of nasogastric tube to the stomach. Failure to reinsert the NGT raised the suspicion of oesophageal atresia which was confirmed by contrast study showing blind upper oesophageal pouch.
Baglaj, Maciej; Gerus, Sylwester
Preduodenal portal vein (PDPV) is a rare congenital anomaly. In most patients, it is associated with other congenital defects including situs inversus, malrotation, and biliary atresia or occurs as part of the heterotaxia syndrome or polysplenia syndrome. We describe a newborn affected by high jejunal atresia, malrotation, and a complex cardiac anomaly, in whom PDPV was diagnosed at early relaparotomy because of stenosis of the jejunal anastomosis. Occurrence of PDPV with intestinal atresia has not been previously reported in the literature.
Shashi, V.; Golden, W.L.; Fryburg, J.S.
The authors report on a child with choanal atresia and deletion 9p. A review of the literature documented one previous instance of choanal atresia in a patient with del(9p). Choanal atresia may be part of the spectrum of malformations in the deletion (9p) syndrome and its presence should prompt a search for this particular deletion as part of the differential diagnosis. 9 refs., 3 figs.
Molinaro, F; Sica, M; Mariscoli, F; Bindi, E; Mazzei, O; Ferrara, F; Messina, M
Duodenal atresia is the frequent cause of neonatal intestinal obstruction. The association between duodenal atresia, intestinal malrotation, cardiac anomalies and Down syndrome is infrequently reported. We present a prenatally suspected case of duodenal atresia which was associated with malrotation and atrial septal defect in a patient of Down syndrome. Duodenotomy and resection of web was performed in addition to Ladd’s procedure. Postoperative course remained uneventful. PMID:27170921
Akcora, Bulent; Eris, Ozge
Congenital duodenal atresia complicated by gastric perforation (GP) is a very rare and a very mortal condition. Only three newborns could be cured in the reported 13 cases. We report a successfully treated newborn with this complicated disease. A 2-day-old male was hospitalized with prediagnosis of duodenal obstruction. Twelve hours later, significant abdominal distention occurred promptly. At laparotomy, GP and preampullary duodenal atresia were detected. Gastrorrhaphy and duodenoduodenostomy were performed in the same operation. The patient was discharged on the 15th postoperative day. This complicated disease can be treated by early diagnosis and surgical intervention. We choose one-stage operation because of the clean peritoneal cavity. However, generalized peritonitis may require two-stage operation in delayed cases.
Silva, D; Santos, F; Piedade, S; Morais-Almeida, M
Eosinophilic esophagitis (EoE) is an inflammatory immune-mediated disease with predominant eosinophilic inflammation characterized by the presence of esophageal dysfunction symptoms. Treatment delay can be associated with disease complications, like esophageal strictures, that can justify the use of invasive procedures which are not deprived of side effects. We present a case report of a 14 year old child with severe esophageal stricture secondary to EoE, that was treated with topical and systemic corticosteroid before any invasive procedure was considered. After 26 weeks of medical treatment, significant improvement of esophageal dysfunction occurred with histological remission and stricture resolution. In patients with severe esophageal strictures secondary to EoE, the need for esophageal dilation procedures should be considered only after anti-inflammatory treatment.
Yeung, Fanny; Tam, Yuk Him; Wong, Yuen Shan; Tsui, Siu Yan; Wong, Hei Yi; Pang, Kristine Kit Yi; Houben, Christopher H; Mou, Jennifer Wai Cheung; Chan, Kin Wai; Lee, Kim Hung
Aim: To review nine-year experience in managing jejuno-ileal atresia (JIA) by primary resection and anastomosis and identify factors associated with reoperations. Methods: From April 2006 to May 2015, all consecutive neonates who underwent bowel resection and primary anastomosis for JIA were analyzed retrospectively. Patients with temporary enterostomy were excluded. Patient demographics, types of atresia, surgical techniques, need for reoperations, and long-term outcomes were investigated. Results: A total of forty-three neonates were included, in which nineteen (44.2%) of them were preterm and fourteen (32.6%) were of low birth weight. Thirteen patients (30.2%) had jejunal atresia whereas thirty patients (69.8%) had ileal atresia. Volvulus, intussusception and meconium peritonitis were noted in 12, 8, and13 patients, respectively. Eight patients (18.6%) had short bowel syndrome after operation. Ten patients (23.3%) required reoperations from 18 days to 4 months after the initial surgery due to anastomotic stricture (n=1), adhesive intestinal obstruction (n=1), small bowel perforation (n=2) and functional obstruction (n=6). Prematurity and low birth weight were associated with functional obstruction leading to reoperation (p=0.04 and 0.01 respectively). The overall long-term survival was 97.7%. All surviving patients achieved enteral autonomy and catch-up growth at a median follow-up of 4.7 years. Conclusion: Long-term survival of JIA after primary resection and anastomosis are excellent. However, patients have substantial risk of early reoperations to tackle intraabdominal complications. PMID:27896150
Stage IB Esophageal Adenocarcinoma; Stage IIA Esophageal Adenocarcinoma; Stage IIB Esophageal Adenocarcinoma; Stage IIIA Esophageal Adenocarcinoma; Stage IIIB Esophageal Adenocarcinoma; Stage IIIC Esophageal Adenocarcinoma
Lo, Jacky F. W.; Tsang, Willis S. S.; Yu, Joannie Y. K.; Ho, Osan Y. M.; Ku, Peter K. M.; Tong, Michael C. F.
Congenital aural atresia is the failure of development of the external auditory canal. It usually occurs in conjunction with microtia, which is the malformation of the auricle due to a failure of development of the external ear. Aural atresia, with or without microtia, may significantly affect the hearing and social life of the patients. It is important for every medical practitioner to be aware of the possible treatment options for hearing rehabilitation in this group of patients. In the era of modern technology, new choices, including Bone-Anchored Hearing Aid (BAHA) (Cochlear Ltd. and Oticon Medical), Vibrant Soundbridge (VSB) (MED-EL, Innsbruck, Austria), and Bonebridge system (BB) (MED-EL, Innsbruck, Austria), provide high-end alternatives to traditional Bone Conduction Hearing Aid and Auditory Canal Reconstruction. All these options have advantages and disadvantages, and they are appropriate for different patients and/or at different ages. This paper aims to provide an overview of the management of hearing rehabilitation in congenital aural atresia patients and a discussion of each treatment option. PMID:24883324
Wu, Hui-Chung; Hsia, Jiun-Yi; Hsu, Chung-Ping
Esophageal laceration with intramural dissection is a rare type of injury but without perforation. It is difficult to differentiate from esophageal perforation at presentation time. We report the case of a 46-year-old man who was admitted to our hospital complaining of progressive chest pain, dysphagia, and odynophagia after swallowing a fish bone three days prior to admission. Esophagoscopy revealed a deep longitudinal laceration with pus discharge in the esophagus. Computed tomography of the chest revealed low posterior mediastinal abscess formation. Surgery was performed under the impression of esophageal perforation. The definite diagnosis was esophageal laceration with intramural dissection.
Vernon, Natalia; Mohananey, Divyanshu; Ghetmiri, Ehsan; Ghaffari, Gisoo
Eosinophilic esophagitis (EoE) is a chronic inflammatory process characterized by symptoms of esophageal dysfunction and, histologically, by eosinophilic infiltration of the esophagus. In adults, it commonly presents with dysphagia, food impaction, and chest or abdominal pain. Chronic inflammation can lead to diffuse narrowing of the esophageal lumen which may cause food impaction. Endoscopic procedures to relieve food impaction may lead to complications such as esophageal perforation due to the friability of the esophageal mucosa. Spontaneous transmural esophageal rupture, also known as Boerhaave's syndrome, as a primary manifestation of EoE is rare. In this paper, we present two adult patients who presented with esophageal perforation as the initial manifestation of EoE. This rare complication of EoE has been documented in 13 other reports (11 adults, 2 children) and only 1 of the patients had been previously diagnosed with EoE. A history of dysphagia was present in 1 of our patients and in the majority of previously documented patients. Esophageal perforation is a potentially severe complication of EoE. Patients with a history of dysphagia and patients with spontaneous esophageal perforation should warrant an evaluation for EoE. PMID:24899902
Matsumoto, Hideo; Kubota, Hisako; Higashida, Masaharu; Manabe, Noriaki; Haruma, Ken; Hirai, Toshihiro
Esophageal diverticulum, a relatively rare condition, has been considered to be associated with motor abnormalities such as conditions that cause a lack of coordination between the distal esophagus and lower esophageal sphincter. We herein report a case of esophageal epiphrenic diverticulum associated with diffuse esophageal spasm. A 73-year-old woman presented with dysphagia and regurgitation. Imaging examinations revealed a right-sided esophageal diverticulum located about 10cm above the esophagogastric junction. High-resolution manometry revealed normal esophageal motility. However, 24-h pH monitoring revealed continuous acidity due to pooling of residue in the diverticulum. An esophageal epiphrenic diverticulum was diagnosed and resected thoracoscopically. Her dysphagia recurred 2 years later. High-resolution manometry revealed diffuse esophageal spasm. The diverticulum in the present case was considered to have been associated with diffuse esophageal spasm. The motility disorder was likely not identified at the first evaluation. In this case, the patient's symptoms spontaneously resolved without any treatment; however, longer-term follow-up is needed. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.
Matsumoto, Hideo; Kubota, Hisako; Higashida, Masaharu; Manabe, Noriaki; Haruma, Ken; Hirai, Toshihiro
Introduction Esophageal diverticulum, a relatively rare condition, has been considered to be associated with motor abnormalities such as conditions that cause a lack of coordination between the distal esophagus and lower esophageal sphincter. Presentation of case We herein report a case of esophageal epiphrenic diverticulum associated with diffuse esophageal spasm. A 73-year-old woman presented with dysphagia and regurgitation. Imaging examinations revealed a right-sided esophageal diverticulum located about 10 cm above the esophagogastric junction. High-resolution manometry revealed normal esophageal motility. However, 24-h pH monitoring revealed continuous acidity due to pooling of residue in the diverticulum. An esophageal epiphrenic diverticulum was diagnosed and resected thoracoscopically. Her dysphagia recurred 2 years later. High-resolution manometry revealed diffuse esophageal spasm. Discussion The diverticulum in the present case was considered to have been associated with diffuse esophageal spasm. The motility disorder was likely not identified at the first evaluation. Conclusion In this case, the patient’s symptoms spontaneously resolved without any treatment; however, longer-term follow-up is needed. PMID:26143577
Lacy, Brian E; Weiser, Kirsten
Symptoms of chest pain and dysphagia are common in the adult population. Most patients initially undergo an evaluation to exclude anatomic causes (ie, esophagitis, stricture) and cardiovascular disease as the etiology of these symptoms. Patients with persistent symptoms may then be referred for specialized testing of the esophagus, including esophageal manometry. Disorders of esophageal motility, which include achalasia, diffuse esophageal spasm, nutcracker esophagus, hypertensive lower esophageal sphincter, and ineffective motility are often identified in these patients. Unfortunately, the etiology of these disorders has not been well characterized and the treatment has not been standardized. This review will briefly discuss the impact, etiology, and diagnosis of esophageal motility disorders, and then focus on the medical management of these disorders using evidence from well-designed, prospective studies, where available.
Mathis, J G; Lehman, G A; Shanks, J C; Blom, E D; Brunelle, R L
Gastroesophageal reflux has been incriminated as a factor-inhibiting acquisition of esophageal speech after laryngectomy. Fourteen proficient esophageal speakers and 10 nonproficient speakers underwent esophageal manometry, esophageal pH probe testing, and Bernstein acid perfusion testing. Additionally, 175 laryngectomized members of Lost Chord Clubs answered mailed questionnaires about the frequency of reflux symptoms. Nonproficient and proficient esophageal speakers had a similar frequency of gastroesophageal reflux by pH probe testing, esophageal mucosal acid sensitivity by Bernstein testing, lower esophageal sphincter pressures, and gastroesophageal reflux symptoms. Gastroesophageal reflux does not appear to be a major factor in preventing esophageal speech.
Clark, John F.J.; Hales, Earle; Ma, Peter; Rosser, Samuel B.
A 28-weeks' gestational trisomic-21 male, with duodenal atresia due to annular pancreas, is presented. The atresia was diagnosed in utero by sonographic techniques. A review of the pertinent literature and discussion of management are presented. ImagesFigure 1Figure 2Figure 3Figure 4 PMID:6231382
Corti, R E; Monastra, L; Fernández Marty, P; Barco, J C; Ferro, F E; Galindo, F; Musi, A O; Kogan, Z
During the period included between January 1970 and December 1990, we studied 242 patients with manometric and radiological diagnosis of esophageal achalasia. Eight of these patients (3.3%) developed during the evolution of their disease an esophageal carcinoma. Eight cases showed histologic type of epidermoid carcinoma: 3 differentiated, 3 semi-differentiated and 2 anaplastic. Therapy for achalasia was: one patient, Heller myotomy, 4 patients, dilatations with bougies in numerous opportunities, and the other two patients receive no treatment for achalasia. Two patients reported tracheobronchial fistulas as complication of carcinoma. Treatment received for carcinoma included: three patients, radiotherapy (4000 rads); one patient, chemotherapy; one patient, chemotherapy and radiotherapy, one resection surgery and two patients feeding gastrostomy. All of the eight patients died within the year of diagnosis of epidermoid carcinoma.
Palacios, Fernando; Contardo, Carlos; Guevara, Jorge; Vera, Augusto; Aguilar, Luis; Huamán, Manuel; Palomino, Américo; Yabar, Alejandro
Fibrovascular polyps are extremely rare benign neoplasias of the esophagus, which usually originate in the lower cricoid area. They do not produce any discomfort in the patient for a long time, however it may make itself evident by the patient's regurgitation of the polyp, producing asphyxia or, more frequently, dysphagia. The case of a 58 year old male patient is presented herein, with a 9 month record of dysphagia, weight loss and intermittent melena. The barium x-ray showed a distended esophagus, with a tumor running from the upper esophageal sphincter to the cardia. The endoscopy confirmed the presence of a pediculated tumor, implanted in the cervical esophagus. Surgeons suspected the potential malignancy of the tumor and performed a transhiatal esophagectomy. The final pathologic diagnosis was giant fibrovascular esophageal polyp.
Redd, Matthew; Schey, Ron
Eosinophilic esophagitis (EoE) is a relatively new entity with a significant amount of increased recognition over the last decade. The mainstay treatments of EoE are designed to eliminate the causative allergens or to reduce their effects on the esophageal mucosa. Common treatments include dietary modification, proton pump inhibitors, systemic and topical corticosteroids, and endoscopic treatments. As the pathogenesis of EoE is explored, new and novel treatments are being studied that target specific pathways and chemokines identified in as precipitating agents of EoE. This is a rapidly evolving field with significant ongoing research and clinical studies. Our review will therefore focus on current and novel treatment approaches to the disease.
Eosinophilic esophagitis (EoE) is a newly recognized disease and is an emerging entity throughout developing and developed countries, including the United States. Therefore, understanding the causes, natural history, diagnosis, and management is important for future therapeutic interventions. The pathogenesis of EoE is still not clear, but a growing body of evidence has established that this condition represents a T-cell-mediated immune response involving several proinflammatory mediators and chemoattractants known to regulate eosinophilic accumulation in the esophagus, such as IL-4, IL-5, IL-3 and eotaxin-1, -2, and -3. Determining the mechanism or mechanisms through which human esophageal-derived factors ultimately induce the functional abnormalities observed, and to which antigens patients who have EoE are sensitized that lead to the manifestation of symptoms, is of significant interest.
Smigiel, Robert; Karpiński, Paweł; Patkowski, Dariusz
Oesophageal atresia is a congenital developmental defect of alimentary tract concerning the interruption of oesophagus with or without connection with the trachea. The incidence of oesophageal atresia is 1:3000-3500 of live-born infants. Associated anomalies occur in 50% of patients (syndromic cases). In the rest of the patients with oesophageal atresia these anomalies are isolated (non-syndromic cases). The knowledge of dysmorphic syndromes with oesophageal defects, allows us to diagnose the complex genetic syndromes and to implement the correct treatment and correct genetic counselling concerning the etiology, natural course of the disease, prognosis and possible complications as well as determining the recurrence risk of the disease in the family. The authors describe the chosen embryological, epidemiological and genetic aspects of congenital oesophageal atresia. The clinical aspects, genetic counselling as well as the genetic basis of chosen genetic syndromes with oesophageal atresia are also described in this article.
Riehl, Megan E.; Keefer, Laurie
Hypnotherapy is an evidence based intervention for the treatment of functional bowel disorders, particularly irritable bowel syndrome. While similar in pathophysiology, less is known about the utility of hypnotherapy in the upper gastrointestinal tract. Esophageal disorders, most of which are functional in nature, cause painful and uncomfortable symptoms that impact patient quality of life and are difficult to treat from a medical perspective. After a thorough medical workup and a failed trial of proton pump inhibitor therapy, options for treatment are significantly limited. While the pathophysiology is likely multifactorial, two critical factors are believed to drive esophageal symptoms—visceral hypersensitivity and symptom hypervigilance. The goal of esophageal directed hypnotherapy is to promote a deep state of relaxation with focused attention allowing the patient to learn to modulate physiological sensations and symptoms that are not easily addressed with conventional medical intervention. Currently, the use of hypnosis is suitable for dysphagia, globus, functional chest pain/non-cardiac chest pain, dyspepsia, and functional heartburn. In this article the authors will provide a rationale for the use of hypnosis in these disorders, presenting the science whenever available, describing their approach with these patients, and sharing a case study representing a successful outcome. PMID:26046715
Iftikhar, Imran; Abdelmannan, Dima; Daw, Hamed A
A case of dermatomyositis and esophageal cancer is described. A 58-year-old male recently diagnosed with esophageal cancer was admitted to the hospital with complaints of progressive dysphagia, generalized muscle weakness and skin rash. The weakness started symmetrically in the proximal limb muscles. He also developed a characteristic skin rash on the eyelids, the upper chest and around the nails that was thought to be most indicative of dermatomyositis. Creatine kinase, aspartate aminotransferase and aldolase were elevated. A muscle biopsy showed various degrees of degeneration with perivascular interstitial infiltration of lymphoplasma cells, a finding consistent with "dermatomyositis." The patient was started on corticosteroids and within two weeks, his muscle strength was found to be markedly improved and the rash almost disappeared. Dysphagia can be the presenting symptom of both dermatomyositis and esophageal cancer. In the setting of an underlying malignancy, these symptoms can be misleading and one can miss the diagnosis of dermatomyositis. However, recognition of the characteristic skin rash may provide a clue to the diagnosis. Another aspect of our case that is worth acknowledgment is the quick response to treatment with corticosteroids.
Riehl, Megan E; Keefer, Laurie
Hypnotherapy is an evidence based intervention for the treatment of functional bowel disorders, particularly irritable bowel syndrome. While similar in pathophysiology, less is known about the utility of hypnotherapy in the upper gastrointestinal tract. Esophageal disorders, most of which are functional in nature, cause painful and uncomfortable symptoms that impact patient quality of life and are difficult to treat from a medical perspective. After a thorough medical workup and a failed trial of proton pump inhibitor therapy, options for treatment are significantly limited. While the pathophysiology is likely multifactorial, two critical factors are believed to drive esophageal symptoms--visceral hypersensitivity and symptom hypervigilance. The goal of esophageal directed hypnotherapy is to promote a deep state of relaxation with focused attention allowing the patient to learn to modulate physiological sensations and symptoms that are not easily addressed with conventional medical intervention. Currently, the use of hypnosis is suitable for dysphagia, globus, functional chest pain/non-cardiac chest pain, dyspepsia, and functional heartburn. In this article the authors will provide a rationale for the use of hypnosis in these disorders, presenting the science whenever available, describing their approach with these patients, and sharing a case study representing a successful outcome.
Farmer, Adam D; Ruffle, James K; Aziz, Qasim
The Rome IV diagnostic criteria delineates 5 functional esophageal disorders which include functional chest pain, functional heartburn, reflux hypersensitivity, globus, and functional dysphagia. These are a heterogenous group of disorders which, despite having characteristic symptom profiles attributable to esophageal pathology, fail to demonstrate any structural, motility or inflammatory abnormalities on standard clinical testing. These disorders are associated with a marked reduction in patient quality of life, not least considerable healthcare resources. Furthermore, the pathophysiology of these disorders is incompletely understood. In this narrative review we provide the reader with an introductory primer to the structure and function of esophageal perception, including nociception that forms the basis of the putative mechanisms that may give rise to symptoms in functional esophageal disorders. We also discuss the provocative techniques and outcome measures by which esophageal hypersensitivity can be established.
Shaw, L M; Jones, W A; Brereton, R J
The presenting features and associated abnormalities of imperforate hymen and vaginal atresia were studied in 24 girls under the age of 16 years. Hydrocolpos or hydrometrocolpos occurred in 8 infants, 13 older girls developed haematocolpos, but 3 of the girls had no distension of the genital tract. Seven of the older girls were diagnosed as having appendicitis. Anorectal anomalies were present in 9 of the children. Intravenous pyelography was performed on 16 girls and was normal in only one. Urinary tract investigations are indicated in all girls with vaginal outlet obstruction, and the vaginal orifice should be inspected in all girls with anorectal abnormalities. PMID:6876046
Biliary atresia (BA) is a rare disease of unknown etiology and unpredictable outcome, even when there has been timely diagnosis and exemplary surgery. It has been the commonest indication for liver transplantation during childhood for the past 20 years. Hence much clinical and basic research has been directed at elucidating the origin and pathology of BA. This review summarizes the current clinical variations of BA in humans, its occasional appearance in animals and its various manifestations in the laboratory as an experimental model. PMID:23987231
Del Toro-Valero, Azucena; Estrada-De la Fuente, Alejandro; Velázquez Santana, Héctor; Glicerio González, Jorge; Navarro Meza, María Cristina; Ortega-Hinojosa, Lilia; López-Cardona, María Guadalupe
Amniotic band sequence (ABS) is a group malformation that mainly affects limbs; clinically, constriction rings and lymphedema of the fingers, arms and legs, acrosyndactyly and pseudosyndactyly are observed; also there is congenital amputation of limbs due to distal swelling. Less frequently, craniofacial and trunk involvement are reported in some patients. Etiology is still unknown and most cases are isolated. In this report we present the case of a 45-day-old male with diagnosis of SBA and bilateral choanal atresia as attached finding, and review possible causes of SBA and associated alterations.
O'Rourke, R W; Deveney, C W; McConnell, D B; Wolfe, B M; Jobe, B A
The long-term effects of gastric banding on esophageal function are not well described. This report describes a 28-year-old woman who developed signs and symptoms of abnormal esophageal motility and lower esophageal sphincter hypotension after gastric banding for morbid obesity. The current literature addressing the effects of gastric banding on esophageal function in light of this case report is discussed.
Piepsz, A.; Ham, H.R.; Georges, B.; Delaet, M.H.; Cadranel, S.
Radionuclide methods using Tc-99m have been developed in the last few years for the assessment of the esophageal transit. The method is physiologic, easy to perform and giving thus interesting informations concerning the severity of the disease as well as the effect of the applied treatment. In children, however, one is limited by radiation protection considerations, and the results are often of poor quality due to the low count rate. Furthermore, the risk of external contamination and the impossibility of repeating the test constitute significant disadvantages, particularly in young children. These problems are completely avoided by using Krypton-8lm diluted in a glucose solution. For each swallowing about 8 mCi of Kr-8 lm are administered. Sixty one-second frames are recorded and several parameters of regional transit can be extracted in a few minutes, using time activity curves and parametric images. The test is very sensitive in detecting minor regional transit alterations. The reproducibility of the test is good and phenomenons like intercurrent gastro-esophageal reflux or asynergic peristaltic waves can easily be detected. The test has been applied to 52 children, aged 1 week to 19 years, and provided helpful information concerning the localization and the importance of transit abnormalities in caustic and peptic esophagitis, postoperative cases (atresia or antireflux plasty) and in patients with neuromuscular disorders.
O'Connor, M K; Byrne, P J; Keeling, P; Hennessy, T P
This study examines the scintigraphic transit pattern in a variety of esophageal disorders. Scintigraphy was performed with a semi solid bolus and the patient in an upright position. Condensed esophageal images were obtained from which we derived the esophageal transit time. The pattern of bolus transit was graded by the duration of transit and by the presence of hold up or retrograde motion. Scintigrams were performed in 11 volunteers and 88 patients whose esophageal function had been confirmed by conventional gastroesophageal techniques. Esophageal disorders examined included achalasia (20), scleroderma (9), esophageal carcinoma (8), Barrett esophagus (5), and reflux esophagitis (27). We also examined the effects of gastroesophageal surgery on esophageal function. Transit times distinguished grossly abnormal esophageal function from normal but did not distinguish between different esophageal disorders. Graded transit patterns were a more sensitive indicator of esophageal function and permitted some differentiation between esophageal disorders and allowed evaluation of the effects of gastroesophageal surgery.
Miyazaki, Tatsuya; Sohda, Makoto; Sakai, Makoto; Tanaka, Naritaka; Suzuki, Shigemasa; Yokobori, Takehiko; Inose, Takanori; Nakajima, Masanobu; Fukuchi, Minoru; Kato, Hiroyuki; Kusano, Motoyasu; Kuwano, Hiroyuki
Esophageal motility disorders are classified primary and secondary, and primary esophageal motility disorders are classified esophageal achalasia and other diseases by manometry. An esophageal emptying disorder associated with insufficient relaxation of the lower esophageal sphincter (LES) and elimination of peristaltic waves on the esophageal body is the major abnormality of achalasia. Esophagogram, endoscopy, and manometry are used for diagnosis. As pharmacological therapy, administration of a calcium channel blocker or nitrate is useful. The pharmacological therapy is not recommended as long-term basic therapy but as a temporary treatment. At 1st, the balloon dilation method is chosen in treatment of achalasia Surgical treatment is indicated in the following cases: (1) Patients uneffected by balloon dilation, (2) Flask type with grade II to III dilation, and sigmoid type, (3) the gradual progression to the pathophysiological stage, (4) young patients, (5) complicated with esophageal cancer. Laparoscopic Heller-Dor procedure is the most popular surgical procedure, recently. It is somewhat difficult to perform surgical treatment for this functional disease. We should select the most suitable individualized treatment with efficient comprehension of the pathophysiological situation.
Baulieu, F.; Baulieu, J.; Maurage, C.; Casset, D.; Itti, R.
The aim of this work was to study the correlation between the reflux timing and the presence of esophagitis, an inconstant but serious complication of gastro-esophageal reflux (GER). The hypothesis was that reflux occurring late after meal can be incriminated more than early reflux in esophagitis genesis. 32 children with GER (mean age = 10.5 months, 2 to 30 months) had esophagoscopy and scintigraphy in the same week. The children were classified in two groups according to esophagoscopy: group 1 (n = 18) no esophagitis, group 2 (n = 14) esophaqgitis. The scintigraphy involved the ingestion of 0.5 mCi Tc-99m sulfur colloid milk mixture, followed by esophageal and gastric activity recording (one image per minute for 1 hour). The reflux was assessed from contrast enhanced images and esophageal time activity curves. Reflux intensity was quantitated by reflux index (Re). Mean reflux time was calculated as the mean esophageal activity peaks time (t-bar). Finally a composite parameter was calculated as the mean reflux time weighted by the relative intensity of each reflux peak (t-barw). Re was not found to be different between the two groups. t-bar was significantly higher in group 2: t-bar = 29.6 +- 3.0 mn (mean +- SD) than in group 1: t-bar = 24.5 +- 6.8 mn; rho <0.02. The difference between the two groups was enhanced by intensity weighting: group 1: t-barw = 16.6 +- 6.3 mn, group 2: t-barw = 33.5 +- 7.1 mn rho <0.001. t-barw value was not correlated to esophagitis grade. These results suggest that late reflux is more likely responsible of esophagitis.
Jensen, Elizabeth T.; Shah, Neil D.; Hoffman, Kate; Sonnenberg, Amnon; Genta, Robert M.; Dellon, Evan S.
Summary Background Seasonal variation has been reported in diagnosis of eosinophilic esophagitis (EoE), but results are not consistent across studies and there are no national-level data in the United States. Aim To determine if there is seasonal variation in diagnosis of esophageal eosinophilia and EoE in the U.S., while accounting for factors such as climate zone and geographic variation. Methods This was a cross-sectional study using a U.S. national pathology database. Patients with esophageal eosinophilia (≥15 eosinophils per high-power field) comprised the primary case definition and were compared to those with normal esophageal biopsies. We calculated the crude and adjusted odds of esophageal eosinophilia by season, as well as by day of the year. Sensitivity analyses were performed using more restrictive case definitions of EoE, and after stratification by climate zone. Results 14,524 cases with esophageal eosinophilia and 90,459 normal controls were analyzed. The adjusted odds of esophageal eosinophilia were higher in the late spring and summer months, with the highest odds in July (aOR 1.13; 95%CI: 1.03–1.24). These findings persisted with increasing levels of esophageal eosinophilia, as well as across EoE case definitions. Seasonal variation was strongest in temperate and cold climates, and peak diagnosis varied by climate zone. Conclusions There is a mild but consistent seasonal variation in the diagnosis of esophageal eosinophilia and EoE, with cases more frequently diagnosed during summer months. These findings take into account climate and geographic differences, suggesting that aeroallergens may contribute to disease development or flare. PMID:26059636
Hill, Sarah J; Clifton, Matthew S; Derderian, Sarkis C; Wulkan, Mark L; Ricketts, Richard R
Neonatal obstructive jaundice is frequently explained by biliary atresia (BA) or the presence of a choledochal cyst (CC). Cystic biliary atresia (CBA) has been a proposed as a subtype of BA with projected improved outcomes. We aimed to characterize these lesions further. We conducted an Institutional Review Board-approved review of all patients treated for obstructive jaundice at our tertiary children's hospital over 10 years. Over the decade we evaluated 91 children with obstructive jaundice: 13 CBA, 52 BA, and 26 CC. Patients with isolated CBA and BA were diagnosed significantly earlier than those with CC (15.9, 54, and 281 days, respectively; P = 0.0001). There was a significant delay between diagnosis and surgical intervention for patients with CBA compared with BA: 17 days versus 5.7 days (P = 0.004). There was no difference in rate of transplant between CBA and BA (31 vs. 50%; P = 0.35). The time from surgery until transplant was 13.9 and 18.6 months for CBA and BA, respectively (P = 0.62). Although radiographically similar to CC, CBA behaves similarly to isolated BA. Delay in recognition and surgical treatment may affect outcomes and lead to an increased incidence of liver failure. The presence of a cystic biliary malformation in the setting of neonatal jaundice should be regarded as CBA until proven otherwise.
McKinnon, B J; Dumon, T; Hagen, R; Lesinskas, E; Mlynski, R; Profant, M; Spindel, J; Van Beek-King, J; Zernotti, M
Congenital aural atresia (CAA) poses significant challenges to surgical remediation. Both bone anchored hearing aids (BAHA) and the Vibrant Soundbridge (VSB) have been considered as alternatives or adjuncts to conventional atresiaplasty. A consensus statement on VSB implantation in children and adolescents recommended against implantation when the Jahrsdoerfer score was less than 8. More recent publications suggest that patients with Jahrsdoerfer scores between three and seven may benefit from VSB implantation. The purpose of this study was to further investigate the outcomes of VSB implantation in CAA. The study was a multi-center, retrospective review. A retrospective review of data (patient's demographic, clinical, implant and audiological information) from four collaborating centers that have performed VSB implantation in CAA was performed. Outcomes based on severity of the atresia using the Jahrsdoerfer and Yellon-Branstetter scoring systems were also evaluated. Data from 28 patients from the four centers revealed no iatrogenic facial nerve injuries or change in bone thresholds. Post-operative speech threshold and speech recognition was, respectively, 39 dB and 94%. Jahrsdoerfer and Yellon scores ranged from 4 to 9 and 4 to 12, respectively. The scores did not correlate to or predict outcomes. Three individual elements of the scores did correlate to initial, but not long-term outcomes. Atresiaplasty and BAHA in the management of CAA are not complete solutions. VSB may offer an alternative in these surgically complex patients for achieving amplification, though better metrics for patient selection need to be developed. LEVEL OF EVIDENCE : IV.
Asai, Akihiro; Miethke, Alexander; Bezerra, Jorge A.
Biliary atresia is a severe cholangiopathy of early infancy that destroys extrahepatic bile ducts and disrupts bile flow. With a poorly defined disease pathogenesis, treatment consists of the surgical removal of duct remnants followed by hepatoportoenterostomy. Although this approach can improve the short-term outcome, the liver disease progresses to end-stage cirrhosis in most children. Further improvement in outcome will require a greater understanding of the mechanisms of biliary injury and fibrosis. Here, we review progress in the field, which has been fuelled by collaborative studies in larger patient cohorts and the development of cell culture and animal model systems to directly test hypotheses. Advances include the identification of phenotypic subgroups and stages of disease based on clinical, pathological and molecular features. Stronger evidence exists for viruses, toxins and gene sequence variations in the aetiology of biliary atresia, triggering a proinflammatory response that injures the duct epithelium and produces a rapidly progressive cholangiopathy. The immune response also activates the expression of type 2 cytokines that promote epithelial cell proliferation and extracellular matrix production by nonparenchymal cells. These advances provide insight into phenotype variability and might be relevant to the design of personalized trials to block progression of liver disease. PMID:26008129
Bajin, Münir Demir; Yılmaz, Taner; Günaydın, Rıza Önder; Kuşçu, Oğuz; Sözen, Tevfik; Jafarov, Shamkal
The aim was to evaluate surgical techniques and their relationship to postoperative success rate and hearing outcomes in acquired atresia of the external auditory canal. In this article, 24 patients with acquired atresia of the external auditory canal were retrospectively evaluated regarding their canal status, hearing, and postoperative success. Acquired stenosis occurs more commonly in males with a male: female ratio of 2-3:1; it seems to be a disorder affecting young adults. Previous ear surgery (13 patients, 54.2%) and external ear trauma (11 patients, 45.8%) were the main etiological factors of acquired ear canal stenosis. Mastoidectomy (12/13) and traffic accidents (8/11) comprise the majority of these etiological factors. Endaural incision is performed in 79.2% and postauricular incision for 20.8% of cases during the operation. As types of surgical approach, transcanal (70.8%), transmastoid (20.8%), and combined (8.4%) approaches are chosen. The atretic plate is generally located at the bony-cartilaginous junction (37.5%) and in the cartilaginous canal (33.3%); the bony canal is involved in a few cases only. Preserved healthy canal skin, split- or full-thickness skin grafts, or pre- or postauricular skin flaps are used to line the ear canal, but preserved healthy canal skin is preferred. The results of surgery are generally satisfactory, and complications are few if surgical principles are followed.
James, Frances M; Parente, Eric J; Palmer, Jonathan E
A 1-day-old Standardbred foal with a history of extreme respiratory distress after birth consistent with upper airway obstruction was evaluated. A temporary tracheostomy tube was placed by the referring veterinarian. On initial examination, there was evidence of hypoxic-ischemic syndrome, secondary to perinatal asphyxia. Endoscopy revealed obstruction of both nares at the level of the choanae; a diagnosis of bilateral choanal atresia was made. The foal was anesthetized and underwent transendoscopic laser fenestration of the buccopharyngeal membranes. Three weeks after surgery, cicatricial narrowing of the choanae was apparent and further transendoscopic ablation was performed. Recurrent stenosis necessitated revision surgeries involving a combination of laser ablation with topical administration of mitomycin and, subsequently, a combination of radial incisions into the stenotic tissue and repeated bougienage with a cuffed endotracheal tube. The degree of stenosis decreased, and at 1 year of age, the horse was an appropriate size for its age, had choanae that were almost maximally open (> 85%), and had entered training. Mild stenosis was still evident when the horse was reexamined the following year, although there was no evidence of exercise intolerance or respiratory compromise. Bilateral choanal atresia in a foal can be successfully treated via transendoscopic fenestration of the buccopharyngeal membranes, enabling the horse to subsequently participate in athletic activities. Secondary problems resulting from initial asphyxia and recurrent stenosis at the surgical site can be overcome but may require prolonged and extensive treatment.
Roberson, Joseph B; Goldsztein, Hernan; Balaker, Ashley; Schendel, Stephen A; Reinisch, John F
Describe anatomical and radiological findings in 742 patients evaluated for congenital aural atresia and microtia by a multidisciplinary team. Develop a new classification method to enhance multidisciplinary communication regarding patients with congenital aural atresia and microtia. Retrospective chart review with descriptive analysis of findings arising from the evaluation of patients with congenital atresia and microtia between January 2008 and January 2012 at a multidisciplinary tertiary referral center. We developed a classification method based on the acronym HEAR MAPS (Hearing, Ear [microtia], Atresia grade, Remnant earlobe, Mandible development, Asymmetry of soft tissue, Paralysis of the facial nerve and Syndromes). We used this method to evaluate 742 consecutive congenital atresia and microtia patients between 2008 and January of 2012. Grade 3 microtia was the most common external ear malformation (76%). Pre-operative Jahrsdoerfer scale was 9 (19%), 8 (39%), 7 (19%), and 6 or less (22%). Twenty three percent of patients had varying degrees of hypoplasia of the mandible. Less than 10% of patients had an identified associated syndrome. Patients with congenital aural atresia and microtia often require the intervention of audiology, otology, plastic surgery, craniofacial surgery and speech and language professionals to achieve optimal functional and esthetic reconstruction. Good communication between these disciplines is essential for coordination of care. We describe our use of a new classification method that efficiently describes the physical and radiologic findings in microtia/atresia patients to improve communication amongst care providers. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.
Terenina, Elena; Fabre, Stephane; Bonnet, Agnès; Monniaux, Danielle; Robert-Granié, Christèle; SanCristobal, Magali; Sarry, Julien; Vignoles, Florence; Gondret, Florence; Monget, Philippe; Tosser-Klopp, Gwenola
Ovarian folliculogenesis corresponds to the development of follicles leading to either ovulation or degeneration, this latter process being called atresia. Even if atresia involves apoptosis, its mechanism is not well understood. The objective of this study was to analyze global gene expression in pig granulosa cells of ovarian follicles during atresia. The transcriptome analysis was performed on a 9,216 cDNA microarray to identify gene networks and candidate genes involved in pig ovarian follicular atresia. We found 1,684 significantly regulated genes to be differentially regulated between small healthy follicles and small atretic follicles. Among them, 287 genes had a fold-change higher than two between the two follicle groups. Eleven genes (DKK3, GADD45A, CAMTA2, CCDC80, DAPK2, ECSIT, MSMB, NUPR1, RUNX2, SAMD4A, and ZNF628) having a fold-change higher than five between groups could likely serve as markers of follicular atresia. Moreover, automatic confrontation of deregulated genes with literature data highlighted 93 genes as regulatory candidates of pig granulosa cell atresia. Among these genes known to be inhibitors of apoptosis, stimulators of apoptosis, or tumor suppressors INHBB, HNF4, CLU, different interleukins (IL5, IL24), TNF-associated receptor (TNFR1), and cytochrome-c oxidase (COX) were suggested as playing an important role in porcine atresia. The present study also enlists key upstream regulators in follicle atresia based on our results and on a literature review. The novel gene candidates and gene networks identified in the current study lead to a better understanding of the molecular regulation of ovarian follicular atresia. Copyright © 2017 the American Physiological Society.
The, Natalie S; Honein, Margaret A; Caton, Alissa R; Moore, Cynthia A; Siega-Riz, Anna Maria; Druschel, Charlotte M
Biliary atresia is a rare birth defect that affects 1 in 12,000 to 1 in 19,500 live births. We used data from the National Birth Defects Prevention Study, a multistate case-control study, to identify potential risk factors for isolated biliary atresia (no additional unrelated major birth defects diagnosed). Infants were identified from eight states from 1997 to 2002, with clinical information abstracted from medical records. Potential risk factors assessed include: demographic factors, seasonality, preterm birth, maternal smoking, maternal alcohol use, maternal illicit drug use, maternal health, maternal medication use, maternal vitamin use, and maternal nutrition. Infants of non-Hispanic black mothers were more likely to have biliary atresia than infants of non-Hispanic white mothers (adjusted odds ratio (aOR) = 2.29, 95% confidence interval (CI) 1.07-4.93) and infants conceived during the spring season were more likely to have biliary atresia than infants conceived in winter (aOR = 2.33, 95%CI 1.05-5.16). Low intakes of vitamin E, copper, phosphorus, and beta tocopherol were associated with the occurrence of isolated biliary atresia (borderline significance). Low iron intake had a borderline inverse association with biliary atresia. While this analysis provides support for previous reports of a possible association between seasonal variation and the occurrence of biliary atresia, more data are needed to evaluate whether the seasonal variation is related to infectious agents. The role of nutrients in the development of biliary atresia remains unclear. Further studies of genetic, infectious, and nutrient exposures and the association of biliary atresia are warranted. 2007 Wiley-Liss, Inc
Crespin, O M; Tatum, R P; Yates, R B; Sahin, M; Coskun, K; Martin, A V; Wright, A; Oelschlager, B K; Pellegrini, C A
Nutcracker esophagus (NE), Jackhammer esophagus (JHE), distal esophageal spasm (DES), and hypertensive lower esophageal sphincter (HTLES) are defined by esophageal manometric findings. Some patients with these esophageal motility disorders also have abnormal gastroesophageal reflux. It is unclear to what extent these patients' symptoms are caused by the motility disorder, the acid reflux, or both. The aim of this study was to determine the effectiveness of laparoscopic Nissen fundoplication (LNF) on esophageal motility disorders, gastroesophageal reflux, and patient symptoms. Between 2007 and 2013, we performed high-resolution esophageal manometry on 3400 patients, and 221 patients were found to have a spastic esophageal motility disorder. The medical records of these patients were reviewed to determine the manometric abnormality, presence of gastroesophageal symptoms, and amount of esophageal acid exposure. In those patients that underwent LNF, we compared pre- and postoperative esophageal motility, gastroesophageal symptom severity, and esophageal acid exposure. Of the 221 patients with spastic motility disorders, 77 had NE, 2 had JHE, 30 had DES, and 112 had HTLES. The most frequently reported primary and secondary symptoms among all patients were: heartburn and/or regurgitation, 69.2%; respiratory, 39.8%; dysphagia, 35.7%; and chest pain, 22.6%. Of the 221 patients, 192 underwent 24-hour pH monitoring, and 103 demonstrated abnormal distal esophageal acid exposure. Abnormal 24-hour pH monitoring was detected in 62% of patients with heartburn and regurgitation, 49% of patients with respiratory symptoms, 36.8 % of patients with dysphagia, and 32.6% of patients with chest pain. Sixty-six of the 103 patients with abnormal 24-hour pH monitoring underwent LNF. Thirty-eight (13NE, 2JHE, 6 DES, and 17 HTLES) of these 66 patients had a minimum of 6-month postoperative follow-up that included clinical evaluation, esophageal manometry, and 24-hour pH monitoring
Tomsa, K; Major, A; Glaus, T M
Atresia ani is the most common anorectal anomaly in small animals. In the present study, an anal stricture (atresia ani type I) in five 3 to 8 weeks old kittens and one 4 month old puppy was treated by balloon dilation. In 4 kittens and the puppy the stricture was eliminated permanently and without complications by a single intervention. Only the smallest kitten with the most severe stenosis developed a rectal fistula as a complication of repeated balloon dilation, which necessitated surgical correction. Balloon dilation proved to be an efficient therapeutic method for anal atresia type I, and can be recommended as the treatment of choice.
Yamagiwa, I; Ohta, M; Obata, K; Washio, M
A case report of biliary atresia associated with preduodenal portal vein is presented with a review of 27 similar cases previously reported. The occurrence of associated anomalies in these 28 cases has a much higher frequency (82%) than coincidental association. They were associated with cardiovascular anomalies in 71%, polysplenia in 68%, malrotation of the intestine in 61%, situs inversus in 22% and duodenal atresia in 9%. Developmental anomaly is considered to participate strongly in the aetiology of biliary atresia combined with preduodenal portal vein.
Nwaorgu, Onyekwere G.; Onakoya, Paul A.; Sogebi, Olusola A.; Kokong, Daniel D.; Dosumu, Oluwole O.
OBJECTIVES: This study aims to highlight the problems associated with impacted acrylic dentures and proffers advice to check them. PATIENTS AND METHODS: Retrospective review of all cases of impacted acrylic dentures over a 16-year period. RESULTS: Twenty-two adults had impacted esophageal acrylic dentures of which 16 (72.7%) and six (27.3%) were males and females, respectively (M:F ratio = 2.7:1) with age range 23-77 years. Fourteen patients (63.6%) had worn their dentures for more than 10 years without check-up, and 54.5% presented within 48 hours of impaction. The common symptoms in all the patients were difficulty with swallowing, throat pain and discomfort, followed by tenderness in the neck in 15 (68.2%). Dentures were extracted through esophagoscopy (17 cases) and cervical (three cases) esophagotomy, respectively. Observed complications included pulmonary edema in one and esophageal perforation in five patients. CONCLUSION: Endoscopic extraction of dentures carries a high risk of perforation. Extraction of an impacted denture via esophagoscopy can be undertaken under direct vision and in an ideal situation with judicious use of the Shears forceps. In the absence of these, the safest option is an esophagotomy. Proper treatment planning in the fabrication of dentures with incorporation of radiopaque materials in the dental resins and adequate postdenture delivery instructions are necessary as preventive measures. PMID:15540888
When radiation therapy is used for palliation of obstruction in patients with advanced esophageal carcinoma, an improvement in dysphagia can be expected in approximately 50% of patients. Major objective responses have rarely been quantitied but, in one study, were seen in 33% patients. Recurrence of dysphagia is usually seen within 2-6 months of treatment. Radiation toxicities and complications, even when used with palliative intent, can be substantial and include esophagitis, tracheoesophageal or esophageal-aortic fistula, mediastinitis, hemorrhage, pneumonitis, and myelosuppression. (JMT)
Feldman, Jeremy; Tejerina, Manfred; Hallowell, Michael
Esophageal lipomas are rare tumors, making up 0.4% of all digestive tract benign neoplasms. Most of these lesions are clinically silent as a result of their small size, however, the majority of lesions over 4 cm have been reported to cause dysphagia, regurgitation and/or epigastralgia. We report a case of a 53 year-old African American female who presented with dysphagia. Computed tomography of the chest and esophagram confirmed esophageal lipoma as the cause of the patient’s symptoms. Accurately diagnosing an esophageal lipoma is crucial in order to rule out potential malignant lesions, relieve patient symptoms and plan the appropriate treatment. PMID:23365708
Heider, T Ryan; Behrns, Kevin E; Koruda, Mark J; Shaheen, Nicholas J; Lucktong, Tananchai A; Bradshaw, Barbara; Farrell, Timothy M
Patients with gastroesophageal reflux disease (GERD) and disordered esophageal motility are at risk for postoperative dysphagia, and are often treated with partial (270-degree) fundoplication as a strategy to minimize postoperative swallowing difficulties. Complete (360-degree) fundoplication, however, may provide more effective and durable reflux protection over time. Recently we reported that postfundoplication dysphagia is uncommon, regardless of preoperative manometric status and type of fundoplication. To determine whether esophageal function improves after fundoplication, we measured postoperative motility in patients in whom disordered esophageal motility had been documented before fundoplication. Forty-eight of 262 patients who underwent laparoscopic fundoplication between 1995 and 2000 satisfied preoperative manometric criteria for disordered esophageal motility (distal esophageal peristaltic amplitude < or =30 mm Hg and/or peristaltic frequency < or =80%). Of these, 19 had preoperative manometric assessment at our facility and consented to repeat study. Fifteen (79%) of these patients had a complete fundoplication and four (21%) had a partial fundoplication. Each patient underwent repeat four-channel esophageal manometry 29.5 +/- 18.4 months (mean +/- SD) after fundoplication. Distal esophageal peristaltic amplitude and peristaltic frequency were compared to preoperative data by paired t test. After fundoplication, mean peristaltic amplitude in the distal esophagus increased by 47% (56.8 +/- 30.9 mm Hg to 83.5 +/- 36.5 mm Hg; P < 0.001) and peristaltic frequency improved by 33% (66.4 +/- 28.7% to 87.6 +/- 16.3%; P < 0.01). Normal esophageal motor function was present in 14 patients (74%) after fundoplication, whereas in five patients the esophageal motor function remained abnormal (2 improved, 1 worsened, and 2 remained unchanged). Three patients with preoperative peristaltic frequencies of 0%, 10%, and 20% improved to 84%, 88%, and 50%, respectively
Taillefer, R.; Beauchamp, G.; Duranceau, A.C.; Lafontaine, E.
The principal radionuclide procedures involved in the evaluation of esophageal disorders that are amenable to surgery are illustrated and briefly described. The role of the radionuclide esophagogram (RE) in the diagnosis and management of achalasia, oculopharyngeal muscular dystrophy and its complications, tracheoesophageal fistulae, pharyngeal and esophageal diverticulae, gastric transposition, and fundoplication is discussed. Detection of columnar-lined esophagus by Tc-99m pertechnetate imaging and of esophageal carcinoma by Ga-67 citrate and Tc-99m glucoheptonate studies also is presented. 37 references.
Muhletaler, C A; Gerlock, A J; de Soto, L; Halter, S A
Thirty-nine esophagograms of 24 patients after ingestion of muriatic acid (27% HCI) in suicide attempts were reviewed. All esophagograms were obtained in the acute, subacute, and chronic phases. In the acute and subacute phases, the radiographic findings consisted of mucosal edema, submucosal edema or hemorrhage, ulcerations, sloughing of the mucosa, atony, and dilatation. Strictures of the esophagus were present in the chronic phase. These radiographic findings were not different from those found in alkaline corrosive esophagitis. The severity of the corrosive esophagitis is considered related to the concentration, amount, viscosity, and duration of contact between the caustic agent and the esophageal mucosa.
Harjai, Man Mohan; Badal, Sachendra; Khanna, Sangeeta; Singh, Ajit Kumar
A common upper airway and digestive tract is a rare congenital anomaly that is usually fatal and its exact incidence is not known. It is a diagnostic challenge as it requires high index of suspicion. It should be considered in a neonate with respiratory distress in a non-vigorous baby requiring endotracheal intubation, which is difficult even in expert hand. We present a newborn with suspected tracheo-esophageal fistula that was diagnosed intraoperatively to have absent upper blind pouch of the esophagus and on autopsy found to have laryngeal atresia with absent vocal cords and a common aerodigestive tract continuing distally with trachea. The neonate was ventilated with endotracheal tube (ETT) placement which in retrospect we came to know that it was in the esophagus. The neonate also had associated multiple congenital anomalies of VACTERL association. The importance of teamwork between neonatologist, pediatric surgeon, anesthesiologist, and radiologist is highlighted for diagnosis and management of such rare cases. PMID:25552830
Pasman, Eric A; Heifert, Theresa A; Nylund, Cade M
Focal dermal hypoplasia (FDH) is a rare disorder of the mesodermal and ectodermal tissues. Here we present an eight-year-old female known to have FDH who presents with poor weight gain and dysphagia. She was diagnosed with multiple esophageal papillomas and eosinophilic esophagitis. She was successfully treated with argon plasma coagulation and ingested fluticasone propionate, which has not been described previously in a child. PMID:28405153
Esophageal Neoplasm; Esophageal Neoplasms Malignancy Unspecified; Esophageal Neoplasms Malignant; Cancer of Esophagus; Cancer of the Esophagus; Esophageal Cancer; Esophagus Cancer; Neoplasm, Esophageal
... test can also be done during upper GI endoscopy by clipping a pH monitor to the lining of the esophagus. ... esophagitis : Barium swallow Esophagogastroduodenoscopy (also called upper GI endoscopy)
Kamangar, Farin; Chow, Wong-Ho; Abnet, Christian; Dawsey, Sanford
Synopsis This articles reviews the environmental risk factors and predisposing conditions for the two main histological types of esophageal cancer, esophageal squamous cell carcinoma (ESCC) and esophageal adenocarcinoma (EA). Tobacco smoking, excessive alcohol consumption, drinking maté, low intake of fresh fruits and vegetables, achalasia, and low socioeconomic status increase the risk of ESCC. Results of investigations on several other potential risk factors, including opium consumption, intake of hot drinks, eating pickled vegetables, poor oral health, and exposure to human papillomavirus, polycyclic aromatic hydrocarbons, N-nitroso compounds, acetaldehyde, and fumonisins are also discussed. Gastroesophageal reflux, obesity, tobacco smoking, hiatal hernia, achalasia, and probably absence of H. pylori in the stomach increase the risk of EA. Results of studies investigating other factors, including low intake of fresh fruits and vegetables, consumption of carbonated soft drink, use of H2 blockers, non-steroidal anti-inflammatory drugs, and drugs that relax the lower esophageal sphincter are also discussed. PMID:19327566
This page lists cancer drugs approved by the Food and Drug Administration (FDA) for esophageal cancer. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries.
Cadranel, S.; Di Lorenzo, C.; Rodesch, P.; Piepsz, A.; Ham, H.R. )
The aim of the present study was to investigate esophageal motor function by means of krypton-81m esophageal transit scintigraphy and to compare the results with the functional and morphological data obtained by means of triple lumen manometry and endoscopy. In acute and subacute stages of the disease, all clinical, anatomical, and functional parameters were in good agreement, revealing significant impairment. In chronic stages, the severity of the dysphagia was not correlated to the importance of the residual stenosis. Conversely, 81mKr esophageal transit and manometric's findings were in good agreement with the clinical symptoms, during the entire follow-up period ranging between 3 months to 7 years. The 81mKr test is undoubtedly the easiest and probably the most physiological technique currently available for long-term functional evaluation of caustic esophagitis.
Jaiman, Sunil; Gundabattula, Sirisha Rao; Ratha, Chinmayee
Apple peel deformity is a rare form of upper intestinal atresia of unknown etiology. Umbilical cord ulcers can occur secondary to reflux of gastric juice and bile as a result of the atresia and can cause lethal intrauterine hemorrhage. The authors report 3 instances of congenital apple peel jejunal atresia with helical umbilical cord ulcers afflicting all female offspring in consecutive pregnancies in a single nonconsanguineous family. There was no hemorrhage from the cord ulcers, but all 3 pregnancies resulted in perinatal death. Although familial occurrence is known, our case series is probably the 1st from the Indian subcontinent and warrants further research into the genetic mechanisms and possible ethnic differences of congenital upper intestinal atresia. The causation of sudden fetal demise in the absence of antecedent cord hemorrhage remains elusive.
Lytrivi, Irene D; Avramidis, Dimosthenis; Sfyridis, Panagiotis; Kirvassilis, George; Sarris, George; Papagiannis, John
We report a case of congenital atresia of the superior vena cava (SVC) with stenotic anastomoses between systemic and pulmonary veins, resulting in cyanosis and symptoms consistent with SVC syndrome in an infant.
Fernández González, N; Prieto Espuñes, S; Ibáñez Fernández, A; Fernández Colomer, B; López Sastre, J; Fernández Toral, J
Jacobsen syndrome is a rare chromosomal disorder due to terminal 11q deletion. Prominent features are growth and psychomotor retardation, trigonocephaly and a characteristic facial dysmorphism, but many different abnormalities have been reported. We present the case of a preterm male. Prenatal ultrasonography was suspicious for duodenal atresia. At birth, the boy presented the craniofacial features typical of Jacobsen syndrome, together with diffusely spread petechiae and talipes equinovarus. Hemogram revealed pancytopenia. Ultrasound examination showed left renal agenesis and confirmed the duodenal atresia. Cerebral computed tomography scan, electroencephalogram and cardiac studies showed no abnormalities. Annular pancreas was found during surgery to correct the duodenal atresia. The karyotype was 46,XY,del(11)(q23.2 --> qter), which confirmed Jacobsen syndrome.A wide spectrum of clinical features is described in Jacobsen syndrome, with phenotype-karyotype correlation. This is the first report of duodenal atresia and annular pancreas.
Houben, C H; Lo, A W I; Tsui, S Y; Chan, K W
An acquired ileal atresia is a rare occurrence. A 3-week-old neonate is presented, who developed postnatally a type 2 ileal atresia and an ileal stenosis within a pre-existing internal hernia secondary to an adhesion band. The literature reports a total of eight cases (4 females) with acquired ileal atresia in babies ranging in age from 3 weeks to 2 years (median 4 months). Mechanical forces (eg, adhesion band, intussusception and volvulus) onto the intestine are most frequently (75%) implicated as the primary event. The distal ileum is most often affected and a type 3A atresia is identified in six of eight (75%) cases. PMID:24225736
Baker, Sarah; Fairchild, Alysa
Radiation-induced esophagitis is the most common local acute toxicity of radiotherapy (RT) delivered for the curative or palliative intent treatment of lung cancer. Although concurrent chemotherapy and higher RT dose are associated with increased esophagitis risk, advancements in RT techniques as well as adherence to esophageal dosimetric constraints may reduce the incidence and severity. Mild acute esophagitis symptoms are generally self-limited, and supportive management options include analgesics, acid suppression, diet modification, treatment for candidiasis, and maintenance of adequate nutrition. Esophageal stricture is the most common late sequela from esophageal irradiation and can be addressed with endoscopic dilatation. Approaches to prevent or mitigate these toxicities are also discussed. PMID:28210168
Reinig, J.W.; Stanley, J.H.; Schabel, S.I.
A study of 200 consecutive chest computed tomographic (CT) examinations revealed thickened esophageal walls (over 3 mm) in 35%. While this is the earliest finding of carcinoma of the esophagus on CT, only half of the cases of thickened walls were due to esophageal carcinoma. Other mediastinal malignancies as well as benign inflammatory, vascular, and fibrotic conditions such as reflux and monilial esophagitis, esophageal varices, and postirradiation scarring were found to cause thickened esophageal walls. Distension with air and intravenous enhancement aid in the optimal evaluation of the esophagus by CT. The thickened esophageal wall is always abnormal, but it is nonspecific, seen in both malignant and nonmalignant conditions.
Yazaki, Etsuro; Woodland, Philip; Sifrim, Daniel
Esophageal function testing should be used for differential diagnosis of dysphagia. Dysphagia can be the consequence of hypermotility or hypomotility of the muscles of the esophagus. Decreased esophageal or esophagogastric junction distensibility can provoke dysphagia. The most well established esophageal dysmotility is achalasia. Other motility disorders can also cause dysphagia. High-resolution manometry (HRM) is the gold standard investigation for esophageal motility disorders. Simultaneous measurement of HRM and intraluminal impedance can be useful to assess motility and bolus transit. Impedance planimetry measures distensibility of the esophageal body and gastroesophageal junction in patients with achalasia and eosinophilic esophagitis.
Khan, Seema; Orenstein, Susan R; Di Lorenzo, Carlo; Kocoshis, Samuel A; Putnam, Philip E; Sigurdsson, Luther; Shalaby, Theresa M
Eosinophilic esophagitis, long known to be a feature of acid reflux, has recently been described in patients with food allergies and macroscopically furrowed esophagus. The pathophysiology and optimal management of patients with eosinophilic esophagitis is unclear. We describe our clinical experience related to eosinophilic esophagitis and obstructive symptoms in children and propose etiopathogenesis and management guidelines. Twelve children with obstructive esophageal symptoms (11 male), median age 5 years, and identified to have eosinophilic esophagitis with > 5 eosinophils per high-power field (eos/hpf) are reported. Of these, four had strictures, six had impactions, and two had only dysphagia. A diagnostic evaluation included esophagogastroduodenoscopy with biopsies in all and upper gastrointestinal series, IgE, radioallergosorbent tests, and skin tests for food allergies in some cases. Esophageal histology specimens were independently analyzed for eosinophil density by two authors. Four of five children with > 20 eos/hpf responded to elimination diets/steroids. The fifth child responded to a fundoplication. Seven children had 5-20 eos/hpf and three of them with no known food allergies responded to antireflux therapy alone. Three others in this group with positive food allergies responded to treatment with elimination diets and/or steroids. The seventh patient in this group was lost to follow-up. In conclusion, on the basis of response to therapy, eosinophilic esophagitis can be subdivided into two groups: those with likely gastroesophageal reflux disease if < 20 eos/hpf and no food allergies, and others with allergic eosinophilic esophagitis associated with food allergies and often with > 20 eos/hpf.
Prenatal ultrasound showing a double bubble is considered to be pathognomonic of duodenal atresia. We recently encountered an infant with prenatal findings suggestive of duodenal atresia with a normal karyotype who actually had a jejunal duplication cyst on exploration. A finding of an antenatal double bubble should lead to a thorough evaluation of the gastrointestinal tract and appropriate prenatal/neonatal testing and management as many cystic lesions within the abdomen can present with this prenatal finding. PMID:26023462
Ramachandran, Priya; Safwan, Mohamed; Srinivas, Sankaranarayanan; Shanmugam, Naresh; Vij, Mukul; Rela, Mohamed
Aims: In 1955, Professor Morio Kasai first performed a hepatic portoenterostomy. Since then, the procedure has changed the lives of children with biliary atresia (BA). We report our initial experience in performing “extended” Kasai portoenterostomy (KPE), a modification of the original procedure. Materials and Methods: Since 2013, we have used the technique of “extended KPE” and prospectively recorded data on all children undergoing this operation. Data on demographics, clinical features, liver function tests, and perioperative cholangiogram findings were collected. Outcome of KPE was measured by Jaundice Disappearance Rate (JDR) and Native Liver Survival Rate (NLSR). We present our preliminary results from a 30-month period (February 2013 to May 2015). Results: Thirty-one children underwent KPE during this period (19 males) and only 1 child had biliary atresia splenic malformation (BASM). The mean age at KPE was 73 ± 24 days. Five (16.1%) children were more than 90 days old at the time of KPE. Fourteen children cleared jaundice (JDR 45.2%). Eleven (35.5%) children developed episodes of cholangitis, of whom 8 had early cholangitis (within 3 months of the operation). The proportion of children who survived with their own liver 6 months after KPE (NLSR) was 84.2%. Of those children older than 90 days, 2 cleared jaundice and have survived with their native livers for more than 16 months. Conclusion: In our preliminary report of 31 children, we conclude that the extended KPE leads to increased jaundice clearance and improved NLSR in children with BA. PMID:27046977
Iordanskaia, Tatiana; Malesevic, Miroslav; Fischer, Gunter; Pushkarsky, Tatiana; Bukrinsky, Michael; Nadler, Evan
Biliary atresia (BA) is a devastating liver disease of unknown etiology affecting children generally within the first 3 months of life. The disease is manifested by inflammation and subsequent obstruction of the extra-hepatic bile ducts, fibrosis and liver failure. The mechanisms responsible for disease pathogenesis are not fully understood, but a number of factors controlled by the SMAD signaling pathway have been implicated. In this study, we investigated the role of a known pro-inflammatory factor, extracellular cyclophilin A (CypA), in the pathogenesis of biliary atresia using the rhesus rotavirus (RRV) murine model. We used a unique cyclosporine A derivative, MM284, which does not enter cells and therefore inactivates exclusively extracellular cyclophilins, as a potential treatment. We demonstrated that levels of CypA in plasma of RRV-infected mice were significantly increased, and treatment of mice with MM284 prior to or one day after disease initiation by RRV infection significantly improved the status of mice with experimental BA: weight gain was restored, bilirubinuria was abrogated, liver infiltration by inflammatory cells was reduced, and activation of SMAD pathway and SMAD-controlled fibrosis mediators tissue inhibitors of MMP (TIMP)-4 and matrix metalloproteinase (MMP)-7 was alleviated. Furthermore, treatment of human hepatic stellate cells with recombinant cyclophilin recapitulated SMAD2/3 activation, which was also suppressed by MM284 treatment. In conclusion, our data provide the first evidence that extracellular cyclophilins activate the SMAD pathway and promote inflammation in experimental BA, and suggest that MM284 may be a promising therapeutic agent for treating BA and possibly other intrahepatic chronic disorders.
Zhou, Kejun; Lin, Na; Xiao, Yongtao; Wang, Yang; Wen, Jie; Zou, Gang-Ming; Gu, Xuefan; Cai, Wei
Biliary Atresia (BA), a result from inflammatory destruction of the intrahepatic and extrahepatic bile ducts, is a severe hepatobiliary disorder unique to infancy. Early diagnosis and Kasai operation greatly improve the outcome of BA patients, which encourages the development of early screening methods. Using HPLC coupled tandem mass spectrometry, we detected primary bile acids content in dried blood spots obtained from 8 BA infants, 17 neonatal jaundice and 292 comparison infants at 3-4 days of life. Taurocholate (TC) was significantly elevated in biliary atresia infants (0.98 ± 0.62 µmol/L) compared to neonatal jaundice (0.47 ± 0.30 µmol/L) and comparison infants (0.43 ± 0.40 µmol/L), with p=0.0231 and p=0.0016 respectively. The area under receiver operating characteristic (ROC) curve for TC to discriminate BA and comparison infants was 0.82 (95% confidence interval: 0.72-0.92). A cutoff of 0.63 µmol/L produced a sensitivity of 79.1% and specificity of 62.5%. The concentrations of total bile acids were also raised significantly in BA compared to comparison infants (6.62 ± 3.89 µmol/L vs 3.81 ± 3.06 µmol/L, p=0.0162), with the area under ROC curve of 0.75 (95% confidence interval: 0.61-0.89). No significant difference was found between the bile acids of neonatal jaundice and that of comparison infants. The early increase of bile acids indicates the presentation of BA in the immediate newborn period and the possibility of TC as newborn screening marker.
Hatzirodos, Nicholas; Nigro, Julie; Irving-Rodgers, Helen F.; Vashi, Aditya V.; Hummitzsch, Katja; Caterson, Bruce; Sullivan, Thomas R.; Rodgers, Raymond J.
To examine the detailed composition of glycosaminoglycans during bovine ovarian follicular development and atresia, the specialized stromal theca layers were separated from the stratified epithelial granulosa cells of healthy (n = 6) and atretic (n = 6) follicles in each of three size ranges: small (3–5 mm), medium (6-9 mm) and large (10 mm or more) (n = 29 animals). Fluorophore-assisted carbohydrate electrophoresis analyses (on a per cell basis) and immunohistochemistry (n = 14) were undertaken. We identified the major disaccharides in thecal layers and the membrana granulosa as chondroitin sulfate-derived ∆uronic acid with 4-sulfated N-acetylgalactosamine and ∆uronic acid with 6-sulfated N-acetylgalactosamine and the heparan sulfate-derived Δuronic acid with N-acetlyglucosamine, with elevated levels in the thecal layers. Increasing follicle size and atresia was associated with increased levels of some disaccharides. We concluded that versican contains 4-sulfated N-acetylgalactosamine and it is the predominant 4-sulfated N-acetylgalactosamine proteoglycan in antral follicles. At least one other non- or 6-sulfated N-acetylgalactosamine proteoglycan(s), which is not decorin or an inter-α-trypsin inhibitor family member, is present in bovine antral follicles and associated with hitherto unknown groups of cells around some larger blood vessels. These areas stained positively for chondroitin/dermatan sulfate epitopes [antibodies 7D4, 3C5, and 4C3], similar to stem cell niches observed in other tissues. The sulfation pattern of heparan sulfate glycosaminoglycans appears uniform across follicles of different sizes and in healthy and atretic follicles. The heparan sulfate products detected in the follicles are likely to be associated with perlecan, collagen XVIII or betaglycan. PMID:22057033
Iordanskaia, Tatiana; Malesevic, Miroslav; Fischer, Gunter; Pushkarsky, Tatiana; Bukrinsky, Michael; Nadler, Evan P
Biliary atresia (BA) is a devastating liver disease of unknown etiology affecting children generally within the first 3 months of life. The disease is manifested by inflammation and subsequent obstruction of the extrahepatic bile ducts, fibrosis and liver failure. The mechanisms responsible for disease pathogenesis are not fully understood, but a number of factors controlled by the SMAD signaling pathway have been implicated. In this study, we investigated the role of a known proinflammatory factor, extracellular cyclophilin A (CypA), in the pathogenesis of biliary atresia using the rhesus rotavirus (RRV) murine model. We used a unique cyclosporine A derivative, MM284, which does not enter cells and therefore inactivates exclusively extracellular cyclophilins, as a potential treatment. We demonstrated that levels of CypA in plasma of RRV-infected mice were increased significantly, and that treatment of mice with MM284 prior to or one day after disease initiation by RRV infection significantly improved the status of mice with experimental BA: weight gain was restored, bilirubinuria was abrogated, liver infiltration by inflammatory cells was reduced and activation of the SMAD pathway and SMAD-controlled fibrosis mediators and tissue inhibitor of metalloproteinases (TIMP)-4 and matrix metalloproteinase (MMP)-7 was alleviated. Furthermore, treatment of human hepatic stellate cells with recombinant cyclophilin recapitulated SMAD2/3 activation, which was also suppressed by MM284 treatment. Our data provide the first evidence that extracellular cyclophilins activate the SMAD pathway and promote inflammation in experimental BA, and suggest that MM284 may be a promising therapeutic agent for treating BA and possibly other intrahepatic chronic disorders. PMID:26225831
Momenah, Tarek S; Khan, Muhammad A; Qureshi, Shakeel; Hijazi, Ziyad M
To maintain aortic continuity, aortic arch interruption is usually treated surgically. We present our experience of aortic arch reconstruction using percutaneous implantation of covered stents and mid-term follow-up. To describe the feasibility, safety, and effectiveness using percutaneous placement of covered stents for functional aortic atresia and mid-term follow-up. Nine patients (7 males), mean age of 30.8 ±16.2 years (range 13-58 years) and mean body weight of 65.7± 14.9 kg (range 52-95 kg), were investigated for systemic hypertension and found to have functional aortic interruption. All were treated with percutaneous perforation, combined with balloon dilation and implantation of covered stents. After stent implantation, control angiograms were performed. All the patients had functional aortic interruption and continuity was established by perforating the atretic segment with trans-septal Brockenbrough needle or the stiff end of a guide wire. A covered Cheatham-Platinum CP stent was used to establish the luminal continuity of the aortic arch. Angiograms after stent deployment showed good forward flow through the stent and hemodynamic assessment revealed minimal gradients across the stent. The mean invasive descending aortic systolic blood pressure before stenting was 86.6 ± 14.3 mm Hg, which increased to 116.5 ± 16.3 mm Hg, after stenting (P = 0.004). The mean invasive descending aortic diastolic blood pressure before stenting was 63.6 ± 8.1 mm Hg, which increased to 79.7 ± 13.3 mm Hg after stenting (P = 0.002). Percutaneous treatment of functional aortic atresia with covered stents is feasible, safe, and effective alternative to surgery with excellent short- and mid-term results. © 2015 Wiley Periodicals, Inc.
Chai, Jianyuan; Jamal, M Mazen
Esophageal cancer is mainly found in Asia and east Africa and is one of the deadliest cancers in the world. However, it has not garnered much attention in the Western world due to its low incidence rate. An increasing amount of data indicate that esophageal cancer, particularly esophageal adenocarcinoma, has been rising by 6-fold annually and is now becoming the fastest growing cancer in the United States. This rise has been associated with the increase of the obese population, as abdominal fat puts extra pressure on the stomach and causes gastroesophageal reflux disease (GERD). Long standing GERD can induce esophagitis and metaplasia and, ultimately, leads to adenocarcinoma. Acid suppression has been the main strategy to treat GERD; however, it has not been proven to control esophageal malignancy effectively. In fact, its side effects have triggered multiple warnings from regulatory agencies. The high mortality and fast growth of esophageal cancer demand more vigorous efforts to look into its deeper mechanisms and come up with better therapeutic options. PMID:23236223
Elzenga, N J; Gittenberger-de Groot, A C
Forty-one specimens with pulmonary atresia and confluent pulmonary arteries were studied to establish the frequency and the nature of anomalies of both the ductus arteriosus and the central pulmonary arteries and their possible interrelation. The specimens were divided according to the type of pulmonary atresia: valvar atresia (12 cases) versus "muscular" atresia of the pulmonary orifice (29 cases). Abnormal connexions between the duct and the adjacent great arteries, as well as stenosis of a pulmonary artery, were common in cases with atresia of the pulmonary orifice and relatively rare in cases with imperforate valve. The abnormal connexions consisted of a proximal displacement of the origin of the duct from the aorta and displacement of the entry site of the duct into the pulmonary trunk towards one of the main pulmonary arteries. A left duct thus connected to the left and a right duct to the right pulmonary artery. Pulmonary artery stenosis, exclusively occurring in those specimens with a duct connecting to a pulmonary artery, were located in the segment of pulmonary artery between the pulmonary trunk and the duct. Histological examination revealed ductal tissue in the wall of a pulmonary artery in more than half of the cases with atresia of the pulmonary orifice. Ductal tissue in a pulmonary artery and pulmonary artery stenosis frequently coexisted. It is concluded that stenoses in the central pulmonary arteries may be related to the duct. The relationship resembles that between the duct and coarctation of the aorta.
Halle, Tyler R; Soares, Bruno P; Todd, N Wendell
We aim to define the frequencies of anomalies of the inner ear, oval window, and round window ipsilateral to isolated non-syndromic unilateral aural atresia. Retrospective case series. We reviewed high resolution computed tomography scans of the temporal bones of 70 children with isolated non-syndromic unilateral congenital aural atresia. Scans were reviewed according to the Jahrsdoerfer criteria and further evaluated for anomalies of the vestibule, semi-circular canals, cochlea, internal auditory canal and vestibulocochlear nerve. Inner ear dysplasia was seen in two of 70 atretic ears: one with a dysmorphic lateral semicircular canal and another with a large vestibule assimilating the lateral semicircular canal. Abnormalities of the oval window and round window ipsilateral to the atresia were identified in 21% (15) and 7% (5), respectively, of the atretic ears. Oval window and round window abnormalities were associated with disproportionately lower Jahrsdoerfer scores compared to aural atresia patients without these abnormalities (P < 0.001 and P = 0.04, respectively). Compared to studies that included syndromic or bilateral atresia cases, we found inner ear and oval window abnormalities less common in children with isolated non-syndromic unilateral aural atresia. However, round window anomalies seem to occur at about the same frequency. Copyright © 2017 Elsevier B.V. All rights reserved.
Chen, Emily; Obolensky, Elise; Rauen, Katherine A; Shaffer, Lisa G; Li, Xu
We describe a 14-year-old boy with congenital bilateral cataracts, blepharophimosis, ptosis, choanal atresia, sensorineural hearing loss, short, webbed neck, poor esophageal motility, severe growth and mental retardation, skeletal anomalies, seizures, and no speech. As an infant, he had transient hypogammaglobulinemia requiring IVIG therapy. Cytogenetic studies show an apparently de novo visible duplication at 1p36.3. Fluorescence in situ hybridization (FISH) studies confirm that the common region for the 1p36 deletion syndrome (p58) is duplicated. Probes for D1Z2 at 1p36.3 and the subtelomeric region of 1p (TEL1p) are also duplicated. Array comparative genomic hybridization (aCGH) studies were done at three separate laboratories, each with somewhat different results. BAC whole genome array CGH suggests a single clone gain at the 1p terminus and a single clone deletion at 1p36.3. A targeted BAC array panel with higher resolution at the distal 1p36 region detects a telomeric duplication and an interstitial deletion. Oligonucleotide whole genomic aCGH shows the highest resolution and a more complex rearrangement: two duplications, an interstitial deletion, and a normal region. The MMP23A/B "matrix metalloproteinase 23A/B" genes are within the distal duplication region in our patient, and this patient does not have craniosynostosis. This is the first association of congenital cataracts, choanal atresia, and transient immune abnormalities with 1p36 duplication/deletion. This case illustrates the limitations of different cytogenetic technologies, and shows how three separate aCGH platforms allow for refined delineation and interpretation of the complex cytogenetic rearrangement which would not have been discovered by standard high-resolution chromosome analysis.
... and weighted with mercury or a metal olive-shaped weight that slides on a guide, such as a string or... esophageal or gastrointestinal bougies and the esophageal dilator (metal olive). (b) Classification. Class...
... and weighted with mercury or a metal olive-shaped weight that slides on a guide, such as a string or... esophageal or gastrointestinal bougies and the esophageal dilator (metal olive). (b) Classification. Class...
... and weighted with mercury or a metal olive-shaped weight that slides on a guide, such as a string or... esophageal or gastrointestinal bougies and the esophageal dilator (metal olive). (b) Classification. Class...
Schroeder, Shauna; Robinson, Zachary D; Masterson, Joanne C; Hosford, Lindsay; Moore, Wendy; Pan, Zhaoxing; Harris, Rachel; Souza, Rhonda F; Spechler, Stuart Jon; Fillon, Sophie A; Furuta, Glenn T
Defensins are antimicrobial peptides expressed on mucosal surfaces that contribute to maintaining intestinal homeostasis by providing innate defense mechanisms for the epithelia. Defensin expression is altered in a number of diseases that affect mucosal surfaces, such as atopic dermatitis, allergic rhinitis, and inflammatory bowel disease. Similar to atopic dermatitis, eosinophilic esophagitis (EoE) is a chronic disease in which the squamous epithelial surface is affected by a similar TH2 microenvironment and eosinophil-predominant inflammation. Therefore, we hypothesized that defensin expression would be decreased in EoE. To address this, we measured defensin expression in vitro in cell lines derived from patients with EoE (EoE1-T) or gastroesophageal reflux disease (GERD) (NES-G4T cells) and ex vivo in esophageal mucosal biopsy samples from children with EoE or GERD and control children without esophageal disease. Interleukin-5 induced a decrease in human β-defensin (hBD) -1 and hBD3 expression in EoE1-T but not in NES-G4T cells. Compared with esophageal biopsy specimens from GERD and control children, specimens from EoE pediatric patients revealed a significant decrease in mRNA and protein expression for hBD1 and hBD3. Diminished expression of hBD1 and hBD3 may make the esophageal epithelium more susceptible to the development and/or perpetuation of EoE.
Wen, Jing; Lu, Zhongsheng; Liu, Qingsen
Endoscopic submucosal dissection (ESD) for the treatment of esophageal mucosal lesions is associated with a risk of esophageal stenosis, especially for near-circumferential or circumferential esophageal mucosal defects. Here, we review historic and modern studies on the prevention and treatment of esophageal stenosis after ESD. These methods include prevention via pharmacological treatment, endoscopic autologous cell transplantation, endoscopic esophageal dilatation, and stent placement. This short review will focus on direct prevention and treatment, which may help guide the way forward. PMID:25386186
Vanagunas, A.; Jacob, P.; Olinger, E. )
Radiation esophagitis is a common but frequently unrecognized complication of therapeutic radiation to the neck, chest, or mediastinum. The spectrum of injury ranges from acute self-limited esophagitis to life-threatening esophageal perforation. Complications such as stricture or primary esophageal cancer may occur many years after irradiation, and their linkage to radiation may not be considered. Five cases of radiation-induced injury are described, and the spectrum of radiation-induced esophageal injury is reviewed.
Tripathi, Monika; Swanson, Paul E
In spite of increasing incidence of esophageal adenocarcinoma in the last few decades, esophageal squamous cell carcinoma (SCC) still remains the dominant subtype of esophageal cancer worldwide. Apart from conventional SCC, some rare unconventional tumors of esophageal squamous mucosa are also well known. This study provides an introduction to these and presents a brief review of the literature, including the diagnostic and prognostic importance of each variant.
Rundback, J.H.; Goldfarb, C.R.; Ongseng, F. )
Ga-67 scanning has been used to evaluate esophageal carcinoma. It has demonstrated candidal infection in other body sites and, in one previous case, in the esophagus. The authors present a case of diffuse esophageal uptake of Ga-67 in esophageal candidiasis.
Hong, Su Jin
Esophageal pain that manifests as heartburn or chest pain, is a prevalent problem. Esophageal chest pain is most often caused by gastroesophageal reflux disease (GERD), but can also result from inflammatory processes, infections involving the esophagus, and contractions of the esophageal muscle. The mechanisms and pathways of esophageal chest pain are poorly understood. Vagal and spinal afferent pathways carry sensory information from the esophagus. Recently, esophageal hypersensitivity is identified as an important factor in the development of esophageal pain. A number of techniques are available to evaluate esophageal chest pain such as endoscopy and/or proton-pump inhibitor trial, esophageal manometry, a combined impedance-pH study, and esophageal ultrasound imaging. Proton pump inhibitors (PPIs) have the huge success in the treatment of GERD. Other drugs such as imipramine, trazadone, sertraline, tricyclics, and theophylline have been introduced for the control of esophageal chest pain in partial responders to PPI and the patients with esophageal hypersensitivity. Novel drugs which act on different targets are anticipated to treat esophageal pain in the future.
Koivusalo, A I; Sistonen, S J; Lindahl, H G; Rintala, R J; Pakarinen, M P
Because of an extended gap between esophageal pouches a variety of methods are employed to treat oesophageal atresia (OA) without (type A) or with (type B) proximal tracheooesophageal fistula. This retrospective observational study describes their single centre long-term outcomes from 1947 to 2014. Of 693 patients treated for OA 68 (9.7%) had type A (n=58, 8.3%) or B (n=10, 1.4%). Hospital records were reviewed. Main outcome measures were survival and oral intake. Nine (13%) patients had early and 10 (15%) delayed primary anastomosis, 30 (44%) underwent reconstruction including colonic interposition (n=13), reversed gastric tube (n=11) and jejunum interposition (n=6), whereas19 (28%) had died without a definite repair. Median follow up was 35 (interquartile range, 7.4-40) years. Thirty-one (63%) of 49 patients with definitive repair survived long term. Survival was 22% for early and 80% for delayed primary anastomosis, 57% for colon interposition, 82% for gastric tube and 84% for jejunum interposition. Gastrooesophageal reflux was most common after gastric tube (80%), dysphagia after colon interposition (50%), and 3 (60%) of 5 survivors with jejunum interposition had permanent feeding ostomy because of neurological disorder. Endoscopic follow-up disclosed no oesophageal cancer or dysplasia. Repair in the most recent patients from 1985 to 2014 (n=14) included delayed primary anastomosis (n=7), jejunum interposition (n=6) and gastric tube (n=1) with 93% long-term survival. Morbidity among long-term survivors of type A or B OA is high. With modern management survival is, however, excellent and patients without neurological disorder achieve full oral intake either after primary anastomosis or reconstruction. IV. Copyright © 2017 Elsevier Inc. All rights reserved.
Calvet, Xavier; Villoria, Albert
At Digestive Disease Week (DDW) 2014, developments in esophageal disease were presented. Highlights include: the usefulness of impedancemetry to diagnose reflux disease, or the effectiveness of PPIs for treating non-cardiac chest pain. Concerning Barrett's esophagus, its prevalence is identical in patients with and without reflux symptoms, Barrett segments less than 1cm probably do not require follow-up, and in older patients with long-segment Barrett, initial endoscopies overlooked up to 2% of significant lesions. Regarding achalasia, surgical myotomy is no more effective than endoscopic dilation and may even be less effective than peroral endoscopic myotomy (POEM). In terms of eosinophilic esophagitis, it is important to systematically take biopsies in patients with dysphagia so that cases of eosinophilic esophagitis are not overlooked. In addition, for this condition, routine endoscopic dilations not only do not seem useful in improving the course of the disease, but could also worsen the response to medical treatment.
Zalar, Alberto E; Olmos, Martín A; Piskorz, Eduardo L; Magnanini, Fernando L
Opportunistic esophageal infections (Candida, cytomegalovirus, herpes simplex virus) and idiophatic esophageal ulcerations are commonly found in HIV patients. However, motility disorders of the esophagus have seldom been investigated in this population. The aim of this prospective study was to determine the presence of motility disorders in HIV patients with esophageal symptoms (with or without associated lesions detected by endoscopy) and in HIV patients without esophageal symptoms and normal esophagoscopy. Eigthteen consecutive HIV patients (10 male, 8 female, ages 20-44 years, mean age 33.5; 8 HIV positive and 10 AIDS) were studied prospectively. Nine patients complained of esophageal symptoms, e.g, dysphagia/odynophagia (group 1) and 9 had symptoms not related to esophageal disease, such as diarrhea, abdominal pain, or gastrointestinal bleeding (group 2). All patients underwent upper endoscopy; mucosal biopsies were taken when macroscopic esophageal lesions were identified or when the patients were symptomatic even if the esophageal mucosa was normal. Esophageal manometry was performed in the 18 patients, using a 4-channel water-perfused system according to a standardized technique. Sixteen of the 18 patients (88.8%) had baseline manometric abnormalities. In group 1, 8/9 patients had esophageal motility disorders: nutcrackeresophagus in 1, hypertensive lower esophageal sphincter (LES) with incomplete relaxation in 2, nonspecific esophageal motility disorders (NEMD) in 3, diffuse esophageal spasm in 1, esophageal hypocontraction with low LES pressure in 1. Six of these 9 patients had lesions detected by endoscopy: CMV ulcers in 2, idiopathic ulcers in 1, candidiasis in 1, idiopathic ulcer + candidiasis in 1, nonspecific esophagitis in 1; and 3/9 had normal endoscopy and normal esophageal biopsies. In group 2, 8/9 patients had abnormal motility: hypertensive LES with incomplete relaxation in 1, nutcracker esophagus in 2, esophageal hypocontraction in 3, and NEMD
Raheem, Mayumi; Leach, Steven T; Day, Andrew S; Lemberg, Daniel A
Eosinophilic esophagitis (EoE) is an emerging disease characterized by esophageal eosinophilia (>15eos/hpf), lack of responsiveness to acid-suppressive medication and is managed by allergen elimination and anti-allergy therapy. Although the pathophysiology of EoE is currently unsubstantiated, evidence implicates food and aeroallergen hypersensitivity in genetically predisposed individuals as contributory factors. Genome-wide expression analyses have isolated a remarkably conserved gene-expression profile irrespective of age and gender, suggesting a genetic contribution. EoE has characteristics of mainly TH2 type immune responses but also some TH1 cytokines, which appear to strongly contribute to tissue fibrosis, with esophageal epithelial cells providing a hospitable environment for this inflammatory process. Eosinophil-degranulation products appear to play a central role in tissue remodeling in EoE. This remodeling and dysregulation predisposes to fibrosis. Mast-cell-derived molecules such as histamine may have an effect on enteric nerves and may also act in concert with transforming growth factor-β to interfere with esophageal musculature. Additionally, the esophageal epithelium may facilitate the inflammatory process under pathogenic contexts such as in EoE. This article aims to discuss the contributory factors in the pathophysiology of EoE.
Trifan, Anca; Stoica, Oana; Chihaia, Catalin-Alexandru; Danciu, Mihai; Stanciu, Carol; Singeap, Ana-Maria
Abstract Introduction: Eosinophilic esophagitis (EoE) is a chronic, immune/antigen-mediated disease characterized clinically by symptoms related to esophageal dysfunction and histologically by a marked eosinophilic infiltrate in the esophageal mucosa. What was once considered a rare disease has nowadays become one of the most frequent esophageal diseases in the Western countries, occupying a place just next to the gastroesophageal reflux disease. EoE etiology and pathogenesis remain largely unknown, although most studies consider that allergic and genetic factors play the most important role. Methods: We report the case of EoE in an elderly male (octogenarian), giving a brief review of the current data related to epidemiology, pathogenesis, diagnosis, and treatment of the disease. Results: Dysphagia to solid foods was the leading symptom, and endoscopic findings included white exudates, longitudinal furrows, and concentric mucosal rings, all suggestive for EoE. Diagnosis relied on histological findings in esophageal mucosal biopsies (>30 eosinophils per high power field). He was treated with topical steroids for 8 weeks, symptoms improved gradually and the patient remained in remission at the 8-month follow-up. Conclusion: This case emphasizes that EoE may occur in very old patients and gastroenterologists should have a high index of suspicion of this disorder in any elderly with dysphagia and endoscopic relevant features. PMID:27741150
Watson, T J; DeMeester, T R; Kauer, W K; Peters, J H; Hagen, J A
Benign esophageal diseases constitute a common group of disorders that are generally managed with medical therapy or surgery designed to improve foregut function. A small subset of patients, however, has advanced disease that requires esophageal replacement to achieve symptomatic relief. One hundred four patients with benign esophageal disease who underwent esophageal reconstruction over a 21-year period (1975 to 1996) were reviewed retrospectively. Dysphagia was the major symptom driving surgery in 80% of the patients. Colon was used to reconstruct the esophagus in 85 patients; stomach, in 10 patients; and jejunum, in 9 patients. Forty-two patients who had lived with their reconstruction for 1 year or more answered a postoperative questionnaire concerning their long-term functional outcome. In the 104 patients, the primary underlying abnormality leading to esophageal replacement was end-stage gastroesophageal reflux (37 patients), an advanced motility disorder (37 patients), traumatic, iatrogenic or spontaneous perforation (15 patients), corrosive injury (8 patients), congenital abnormality (6 patients), or extensive leiomyoma (1 patient). Ninety-eight percent of patients reported that the operation had cured or improved the symptom driving surgery. Ninety-three percent were satisfied with the outcome of the operation. The overall hospital mortality rate was 2%, and the median hospital stay was 17 days. Graft necrosis occurred in 3% of patients, and anastomotic leak occurred in 6% of patients (or 2% of the total number of anastomoses). Esophageal replacement for benign disease can be accomplished with a low mortality rate and a marked improvement in alimentation. Reconstruction restores the pleasure of eating and is viewed by the patient to be highly successful.
Totonelli, Giorgia; Maghsoudlou, Panagiotis; Fishman, Jonathan M; Orlando, Giuseppe; Ansari, Tahera; Sibbons, Paul; Birchall, Martin A; Pierro, Agostino; Eaton, Simon; De Coppi, Paolo
A number of congenital and acquired disorders require esophageal tissue replacement. Various surgical techniques, such as gastric and colonic interposition, are standards of treatment, but frequently complicated by stenosis and other problems. Regenerative medicine approaches facilitate the use of biological constructs to replace or regenerate normal tissue function. We review the literature of esophageal tissue engineering, discuss its implications, compare the methodologies that have been employed and suggest possible directions for the future. Medline, Embase, the Cochrane Library, National Research Register and ClinicalTrials.gov databases were searched with the following search terms: stem cell and esophagus, esophageal replacement, esophageal tissue engineering, esophageal substitution. Reference lists of papers identified were also examined and experts in this field contacted for further information. All full-text articles in English of all potentially relevant abstracts were reviewed. Tissue engineering has involved acellular scaffolds that were either transplanted with the aim of being repopulated by host cells or seeded prior to transplantation. When acellular scaffolds were used to replace patch and short tubular defects they allowed epithelial and partial muscular migration whereas when employed for long tubular defects the results were poor leading to an increased rate of stenosis and mortality. Stenting has been shown as an effective means to reduce stenotic changes and promote cell migration, whilst omental wrapping to induce vascularization of the construct has an uncertain benefit. Decellularized matrices have been recently suggested as the optimal choice for scaffolds, but smart polymers that will incorporate signalling to promote cell-scaffold interaction may provide a more reproducible and available solution. Results in animal models that have used seeded scaffolds strongly sug- gest that seeding of both muscle and epithelial cells on scaffolds
Ibáñez-Sanz, Gemma; Rodríguez-Alonso, Lorena; Romero, Natalia M
A 35-year-old man, with a history of rhinitis, eczema and a dubious achalasia was admitted due to chest pain and sialorrhea. Upper endoscopy showed a little hole and a narrowing of the distal esophagus. A CT-scan with oral contrast exposed a discontinuity of the lumen of the middle third of the esophagus and a dissection of submucosal space 16 cm long. The patient recovered after parenteral nutrition. After four months, an esophageal endoscopic showed transient whitish exudates, longitudinal furrows and esophageal lacerations. The biopsies illustrated significant eosinophilic inflammation, eosinophilic microabscesses and basal cell hyperplasia.
Al-Samawi, Abdullah S; Aulaqi, Saleh M
To document the age and gender distribution, histopathologic type as well as grading characteristics of Esophageal Cancer (EC) in Yemen. A case series. Department of Pathology, Sana'a University, Sana'a, Yemen, from January 2005 to December 2011. Three hundred twenty five cases of EC were included for review. The diagnoses were made on hematoxylin and eosin stained sections and the cases were categorized into Squamous Cell Carcinoma (SCC) and adenocarcinoma (ADC). Out of the 325 EC cases, 163 (50%) were SCC (females 67%, males 33%) and 158 (49%) were ADC (females 30%, males 70%). The rest of the cases were 2 adenosquamous carcinoma and 2 non-Hodgkin's lymphoma. The mean age, for SCC was 60 years while the mean age for ADC was 65 years. The peak incidence for SCC was found in the age groups of fifth and sixth decades for females and in fifth and seventh decades for males. The maximum number of patients with ADC was seen in sixth and seventh decades for both gender. Well-differentiated histological grading accounted for 247 (77%) for both genders and types. The moderately differentiated and poorly differentiated accounted, for 17% and 6% respectively. The EC in Yemen had a predominance of SCC in female patients and predominance of ADC in male patients which was usually of a well-differentiated grade.
Allum, William H.; Bonavina, Luigi; Cassivi, Stephen D.; Cuesta, Miguel A.; Dong, Zhao Ming; Felix, Valter Nilton; Figueredo, Edgar; Gatenby, Piers A.C.; Haverkamp, Leonie; Ibraev, Maksat A.; Krasna, Mark J.; Lambert, René; Langer, Rupert; Lewis, Michael P.N.; Nason, Katie S.; Parry, Kevin; Preston, Shaun R.; Ruurda, Jelle P.; Schaheen, Lara W.; Tatum, Roger P.; Turkin, Igor N.; van der Horst, Sylvia; van der Peet, Donald L.; van der Sluis, Peter C.; van Hillegersberg, Richard; Wormald, Justin C.R.; Wu, Peter C.; Zonderhuis, Barbara M.
The following, from the 12th OESO World Conference: Cancers of the Esophagus, includes commentaries on the role of the nurse in preparation of esophageal resection (ER); the management of patients who develop high-grade dysplasia after having undergone Nissen fundoplication; the trajectory of care for the patient with esophageal cancer; the influence of the site of tumor in the choice of treatment; the best location for esophagogastrostomy; management of chylous leak after esophagectomy; the optimal approach to manage thoracic esophageal leak after esophagectomy; the choice for operational approach in surgery of cardioesophageal crossing; the advantages of robot esophagectomy; the place of open esophagectomy; the advantages of esophagectomy compared to definitive chemoradiotherapy; the pathologist report in the resected specimen; the best way to manage patients with unsuspected positive microscopic margin after ER; enhanced recovery after surgery for ER: expedited care protocols; and long-term quality of life in patients following esophagectomy. PMID:25266029
Background. The relation of esophageal food bolus impaction (FBI) to eosinophilic esophagitis (EoE) and lymphocytic esophagitis (LyE) is unclear. The aim of this study was to determine the prevalence of EoE and LyE among adults with FBI. Methods. In this retrospective study we analyzed data from all patients referred for gastroscopy during the past 5 years, because of a present or recent episode of FBI. Results. We found 238 patients with FBI (median age 51 (17–96), 71% males). Endoscopic therapy was required in 143 patients. Esophageal biopsies were obtained in 185 (78%) patients. All biopsies were assessed for numbers of eosinophils and lymphocytes. EoE was found in 18% of patients who underwent biopsy. We found 41 patients (22%) who fulfilled the criteria for both EoE and LyE (EoE/LyE). LyE was found in the 9% of patients with FBI. EoE together with EoE/LyE was the leading cause of FBI in patients ≤50 years (64%). GERD was the leading cause of FBI among patients older than 50 years (42%). Conclusions. Our study showed that EoE was the leading cause of FBI in particular among young adults. Our study highlights the need for esophageal biopsies in any patient with FBI. PMID:27547221
Truskaite, Kotryna; Dlugosz, Aldona
Background. The relation of esophageal food bolus impaction (FBI) to eosinophilic esophagitis (EoE) and lymphocytic esophagitis (LyE) is unclear. The aim of this study was to determine the prevalence of EoE and LyE among adults with FBI. Methods. In this retrospective study we analyzed data from all patients referred for gastroscopy during the past 5 years, because of a present or recent episode of FBI. Results. We found 238 patients with FBI (median age 51 (17-96), 71% males). Endoscopic therapy was required in 143 patients. Esophageal biopsies were obtained in 185 (78%) patients. All biopsies were assessed for numbers of eosinophils and lymphocytes. EoE was found in 18% of patients who underwent biopsy. We found 41 patients (22%) who fulfilled the criteria for both EoE and LyE (EoE/LyE). LyE was found in the 9% of patients with FBI. EoE together with EoE/LyE was the leading cause of FBI in patients ≤50 years (64%). GERD was the leading cause of FBI among patients older than 50 years (42%). Conclusions. Our study showed that EoE was the leading cause of FBI in particular among young adults. Our study highlights the need for esophageal biopsies in any patient with FBI.
Philpott, H; Nandurkar, S; Royce, S G; Thien, F; Gibson, P R
Eosinophilic esophagitis (EoE) is a chronic antigen driven disease, whereby food and/or aeroallergens result in inflammation and luminal narrowing, and the clinical symptoms of dysphagia and food bolus obstruction events (FBOE). Established risk factors are male gender, Caucasian race and atopy. Increased risk amongst family members, and a single nucleotide polymorphism (SNP) in a gene coding thymic stromal lymphopoietin (TSLP) on the pseudoautosomal region of the X and Y chromosomes supports a genetic predisposition. Environmental factors including the timing and nature of food and aeroallergen exposure to the developing immune system may be important, whilst esophageal barrier function integrity and the influence of microbiota are worthy of future research.
Scheppach, Wolfgang; Meesmann, Malte
History | A 77-year-old woman was admitted with severe chest pain, heartburn, dysphagia and odynophagia. She had been on dabigatran for 13 months due to atrial fibrillation and arterial hypertension. Investigations and findings | Endoscopy of the esophagus revealed sloughing of mucosal casts, predominantly in the upper half of the organ. Treatment and course | The patient was placed on pantoprazol, local anaesthetic antacid and i. v. fluids. Dabigatran was discontinued. The symptoms disappeared within 3 days. Control endoscopy after 12 days showed complete healing of the esophageal mucosa. Conclusion | The intake of dabigatran was associated with exfoliative esophagitis, possibly due to caustic tissue damage by prolonged drug contact.
Attaway, Judy; Stone, Christopher L; Sendor, Cindy; Rosario, Emily R
There currently is no guideline regarding amplification or verification for children with aural atresia. This population, with the absence of the ear canal, has obvious congenital hearing loss. Research suggests that delayed treatment for hearing loss can result in speech and language impairments, including poor performance in school. In this study we examined the relationship between amplification and emerging speech and language development in children with aural atresia. Subjects included children, 3 to 6 years of age, with conductive hearing loss due to atresia. Clinical evaluations were used to determine relationships between amplification and speech and language development. Subjects underwent an audiology exam, including pure-tone or warble-tone air and bone conduction using play audiometry techniques, and a speech and language evaluation assessing language and vocabulary skills. Subjects who were fitted before 1 year of age showed better compliance with aid use and exhibited fewer delays in speech and language development. Children with right-sided atresia had significantly greater speech and language delays when compared with age norms from standardized tests. Our findings highlight a relationship between side of atresia, time of first amplification, compliance in aid use, and speech and language abilities.
Alamoudi, Uthman; Hong, Paul
Many parents and children utilize the Internet for health-related information, but the quality of these websites can vary. The objective of this study was to assess the quality and readability of microtia and aural atresia related websites. The search engine Google was queried with the terms 'microtia' and 'aural atresia.' The first 30 results were evaluated, and those websites containing original information written in English were reviewed. Quality of content was assessed with the DISCERN instrument, and readability was assessed with the Flesch-Kincaid Reading Grade Level (FKGL) and the Flesch Reading Ease Score (FRES) tests. Each website was also reviewed for ownership and the date of last update. Sixteen microtia and 14 aural atresia websites were included for full review. The mean DISCERN score for microtia websites was 54.4 (SD=8.3), and for aural atresia websites it was 47.6 (SD=10.7), which indicates 'good' and 'fair' quality of content, respectively. Readability assessments showed an average reading level requiring a grade 10 education on FKGL, and only one microtia (6.3%) and one aural atresia (7.1%) websites were deemed to be at 'reasonable' reading level on FRES. High-quality websites that are considered easily comprehensible to the general public were lacking. Since parents and children may use websites when making treatment decisions, physicians should be aware of the quality of health information pertaining to their area of expertise available on the Internet. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.
Uygun, Ibrahim; Zeytun, Hikmet; Otcu, Selcuk
Background: Isolated oesophageal atresia without tracheo-oesophageal fistula represents a major challenge for most paediatric surgeons. Here, we present our experience with six neonates with isolated oesophageal atresia who successfully underwent immediate primary anastomosis using multiple Livaditis circular myotomy. Materials and Methods: All six neonates were gross type A isolated oesophageal atresia (6%), from among 102 neonates with oesophageal atresia, treated between January 2009 and December 2013. Five neonates were female; one was male. The mean birth weight was 2300 (range 1700-3100) g. Results: All six neonates successfully underwent immediate primary anastomosis using multiple myotomies (mean 3; range 2-4) within 10 (median 3) days after birth. The gap under traction ranged from 6 to 7 cm. One neonate died of a major cardiac anomaly. Another neonate was lost to follow-up after being well for 3 months. Three anastomotic strictures were treated with balloon dilatation, and four anastomotic leaks were treated conservatively. The mean duration of follow-up was 33 months. Conclusions: To treat isolated oesophageal atresia, an immediate primary anastomosis can be achieved using multiple myotomies. Although, this approach is associated with high complication rates, as are other similar approaches, these complications can be overcome. PMID:26712295
Chen, Junming; Liang, Hairong; Wang, Yuejian; Yu, Youjun
The aim of this study was to observe the effect of a titanium tube on external auditory canal reconstruction in congenital aural atresia and to assess the tube's effectiveness in preventing external canal stenosis or atresia after reconstruction. Reconstruction of the external ear canal with a titanium mesh tube was performed in 16 patients (16 ears) with congenital aural atresia at the First People's Hospital of Foshan. The titanium mesh tube was removed 1 year after surgery. The patients were followed up for 2 years (2 ± 0.3 years), and all of the patients had formed a new external ear canal. There was no local infection, granulation tissue, re-stenosis, or atresia in any of the patients after surgery. All of the patients were content with their newly formed external ear canal. Titanium mesh tubing is safe and effective for reconstruction of the external ear canal during surgery for congenital aural atresia.
Chung-Davidson, Yu-Wen; Yeh, Chu-Yin; Li, Weiming
Biliary atresia (BA) is a progressive, inflammatory, and fibrosclerosing cholangiopathy in infants that results in obstruction of both extrahepatic and intrahepatic bile ducts. It is the most common cause for pediatric liver transplantation. In contrast, the sea lamprey undergoes developmental BA with transient cholestasis and fibrosis during metamorphosis, but emerges as a fecund adult with steatohepatitis and fibrosis in the liver. In this paper, we present new histological evidence and compare the sea lamprey to existing animal models to highlight the advantages and possible limitations of using the sea lamprey to study the etiology and compensatory mechanisms of BA and other liver diseases. Understanding the signaling factors and genetic networks underlying lamprey BA can provide insights into BA etiology and possible targets to prevent biliary degeneration and to clear fibrosis. In addition, information from lamprey BA can be used to develop adjunct treatments for patients awaiting or receiving surgical treatments. Furthermore, the cholestatic adult lamprey has unique adaptive mechanisms that can be used to explore potential treatments for cholestasis and nonalcoholic steatohepatitis (NASH). PMID:26101777
Hvass, U; Debauchez, M; Abou-Eid, G; Pansard, Y; Bohm, G; Depoix, J P; Cloez, J L; Worms, A M
Six patients aged 1.5 to 4 years with type 13 tricuspid atresia underwent extracardiac bicavopulmonary repair. This corrective procedure comprises control of palliative aorto-pulmonary anastomosis, the construction of an anastomosis between the superior vena cava and right pulmonary artery and the interposition of an extracardiac tube of autologous pediculated pericardium between the inferior vena cava and the main pulmonary artery. This tube, fashioned from a rectangular flap, remains attached along its right border, conserving its vascular pedicle with the pericardium. Its diameter is calibrated to that of the inferior vena cava. The postoperative course was uncomplicated: all children survived. Assisted respiration was discontinued 24 to 36 hours after surgery, and the pleural drains withdrawn after 48 to 72 hours. The rhythm remained sinusal. Central venous pressure ranged from 8 to 12 mmHg. Peripheral arterial situation was over 95%. At follow-up at 3 to 15 months, the children were very active, reflecting good exercise capacity. Holter monitoring was normal. Saturation was 94 to 97%. Doppler echocardiography showed laminar blood flow. This technique insures good venous drainage without any prosthetic thrombogenic material. In addition, the pediculated pericardium conserves a potential for growth, justifying its use in small children. Long-term evaluation is essential but the initial results are encouraging and encourage perseverance with this method.
Robie, Daniel K; Overfelt, Sarah R; Xie, Li
Diagnosis of biliary atresia (BA) in infants presenting with cholestatic jaundice (CJ) requires exploratory surgery with cholangiography. However, the lack of a standardized approach to preoperative evaluation of infants with CJ can lead to a high number of negative surgical explorations. We reviewed our experience with CJ and BA to determine preoperative variables that might reliably identify BA. Infants explored for possible BA over a 5-year period were retrospectively reviewed. Preoperative clinical indices and liver biopsy results were reviewed. Statistical analysis was conducted by Student's t test and Fisher's exact test (P < 0.05). Twenty patients were identified, 10 with BA and 10 without (50% negative exploration rate). Nuclear cholescintigraphy (HIDA) excretion into the gastrointestinal tract was absent in all BA and in 8 of 10 without BA. Hepatomegaly was more common in the BA group (OR = 9.3, P = 0.07). BA had higher mean (± standard error) serum gamma-glutamyl transpeptidase levels (542 ± 130 vs 139 ± 25.8 U/L in non-BA, P = 0.03). There were insignificant differences in sex, type of feeding, TPN exposure and sepsis between the two groups. Although our small sample size limits conclusions, we suggest screening infants with CJ by measuring GGT levels, absence of hepatomegaly, presence of cholic stools and/or excretion on HIDA scan to undergo pecutaneous liver biopsy given the lower likelihood of BA necessary.
Calder, A L; Co, E E; Sage, M D
The incidence and severity of abnormalities of the coronary arteries were evaluated in 35 necropsy patients with pulmonary atresia and an intact ventricular septum. Right ventricular to coronary artery fistulous connections were found in more than 60% of the cases. All patients with fistulous connections had histologic abnormalities of the coronary arteries. In 50% the lesions were mild, with medial and intimal thickening producing up to moderate luminal stenosis. In 50% there was loss of normal arterial wall structure and severe narrowing or obliteration of the arterial lumen. The coronary arterial fistulas and histologic abnormalities were prevalent in those with underdevelopment of the tricuspid valve and right ventricular cavity but were not found in patients with a normal or dilated tricuspid valve anulus. The coronary arterial abnormalities were found in more than 80% of patients with a tricuspid valve/mitral valve ratio less than 1. A single coronary artery occurred in 6 patients (17%) of this series. In 80% of those with 1 coronary artery arising from the other, the aberrantly arising coronary artery crossed anteriorly to the pulmonary artery and could be at risk in surgical attempts to reconstruct the right ventricular outflow. Aortography is recommended if the coronary arteries are not clearly delineated on ventricular cineangiocardiography.
Lorent, Kristin; Gong, Weilong; Koo, Kyung A.; Waisbourd-Zinman, Orith; Karjoo, Sara; Zhao, Xiao; Sealy, Ian; Kettleborough, Ross N.; Stemple, Derek L.; Windsor, Peter A.; Whittaker, Stephen J.; Porter, John R.; Wells, Rebecca G.; Pack, Michael
Biliary atresia (BA) is a rapidly progressive and destructive fibrotic disorder of unknown etiology affecting the extrahepatic biliary tree of neonates. Epidemiological studies suggest that an environmental factor, such as a virus or toxin, is the cause of the disease, although none have been definitively established. Several naturally occurring outbreaks of BA in Australian livestock have been associated with the ingestion of unusual plants by pregnant animals during drought conditions. We used a biliary secretion assay in zebrafish to isolate a previously undescribed isoflavonoid, biliatresone, from Dysphania species implicated in a recent BA outbreak. This compound caused selective destruction of the extrahepatic, but not intrahepatic, biliary system of larval zebrafish. A mutation that enhanced biliatresone toxicity mapped to a region of the zebrafish genome that has conserved synteny with an established human BA susceptibility locus. The toxin also caused loss of cilia in neonatal mouse extrahepatic cholangiocytes in culture and disrupted cell polarity and monolayer integrity in cholangiocyte spheroids. Together, these findings provide direct evidence that BA could be initiated by perinatal exposure to an environmental toxin. PMID:25947162
Casale, Garrett; Nicholas, Brian D; Kesser, Bradley W
To estimate the prevalence of external auditory canal (EAC) stenosis in patients with congenital aural atresia (CAA) and the prevalence of acquired ear canal cholesteatoma in patients with EAC stenosis and to identify risk factors that may predict the presence of ear canal cholesteatoma in those patients. Retrospective chart review. University tertiary referral center. Patients with EAC stenosis with and without acquired ear canal cholesteatoma were identified from the medical records of 673 patients (770 ears) with CAA. Demographic data, symptoms at presentation, and audiometric data were compared between those with and those without cholesteatoma to identify the risk factors for the presence of ear canal cholesteatoma. Of the 770 ears evaluated, 101 (13.1%) were found to have at least 1 stenotic ear canal. Of this group of 101 ears with canal stenosis, 18 of 94 ears (7 ears were missing data; 19.1%) had a concurrent cholesteatoma, with 1 patient having bilateral cholesteatomas. Demographic, clinical, and audiometric parameters showed that only female sex was associated with a higher rate of ear canal cholesteatoma. Within the cholesteatoma group, right ears in female patients and left ears in male patients predominated. Approximately 1 in 5 patients with congenital aural stenosis were found to have ear canal cholesteatoma. Female sex is a risk factor; basic audiometric parameters provide no diagnostic utility in distinguishing ears with cholesteatoma from those without cholesteatoma.
Reed, Robert; Hubbard, Matthew; Kesser, Bradley W
To compare speech/language development and academic progress between children with right versus left congenital aural atresia (CAA). Case control survey and review of audiometric data. Tertiary care academic practice. Children with unilateral CAA. Demographic and audiometric data; rates of grade retention, use of any hearing or learning resource, and behavioral problems. No significant differences in grade retention rate, utilization of amplification, speech language therapy, use of an individualized education program, or frequency modulated system were found between children with right versus left CAA. Children with left CAA were significantly more likely to be enrolled in special education programs (p = 0.026). Differences in reported communication problems approached significance with more difficulty noted in the right ear group (p = 0.059). Left CAA patients were also more likely to have reported behavioral problems (p = 0.0039). Contrary to the hypothesis that a normal hearing right ear confers a language advantage in patients with unilateral hearing loss, children with left CAA (normal right ear) were statistically more likely to be enrolled in a special education program and have behavioral problems. Reported communication problems were more common in right CAA patients, but this did not reach statistical significance. No differences were found in use of amplification, frequency modulated system, individualized education program, or grade retention. Further investigation of both the clinical implications and underlying psychoacoustics of unilateral hearing loss and the identification and habilitation of "at risk" unilateral hearing loss children is warranted.
Huppert, Peter E.; Goffette, Pierre; Sokal, Emil M.; Schweizer, Paul; Claussen, Claus D.
Purpose: We retrospectively evaluated the technical and long-term clinical results of transjugularintrahepatic portosystemic shunts (TIPS) in children with portal hypertension and biliary atresia (BA). Methods: Nine children with BA and recurrent bleeding from esophagogastric and/or intestinal varices were treated by TIPS at the age of 34-156 months and followed-up in two centers. Different types of stents were used. Results: Shunt insertion succeeded in all patients, but in two a second procedure was necessary. Seven procedures lasted more than 3 hr, mainly due to difficult portal vein puncture.Variceal bleeding ceased in all patients; however, 16 reinterventions were performed in eight patients for clinical reasons (n =11) and sonographically suspected restenosis (n =5). Four patients underwent successful liver transplantation 4-51 months after TIPS and five are in good clinical conditions 64-75 months after TIPS. Conclusions: TIPS in children with BA is technically difficult, mainly due to periportal fibrosis and small portal veins. Frequency of reinterventions seems to be higher compared with adults.
Morita, Keiichi; Fukuzawa, Hiroaki; Maeda, Kosaku
The first-choice therapy for biliary atresia (BA) is Kasai hepatoportoenterostomy, which has been shown to greatly improve outcome. Various long-term complications, however, such as portal hypertension and hepatopulmonary syndrome (HPS), can occur in patients with native liver. A rare case of brain abscess in an 11-year-old girl with HPS associated with BA is reported. The patient underwent hepatoportoenterostomy for BA at 53 days of age, with resolution of hyperbilirubinemia. At 10 years of age, she was diagnosed with severe HPS with right-to-left shunting, and preparations for liver transplantation proceeded. Three months after the diagnosis, she had a right parietal brain abscess. Given that the brain abscess enlarged in size, surgical drainage of the brain abscess was performed. The postoperative course was uneventful, but a slight left hemiplegia remained at discharge. The presumed mechanism of abscess formation in HPS may be right-to-left bacterial transit through intrapulmonary vascular dilatations and/or arteriovenous fistulae. © 2015 Japan Pediatric Society.
Reed, Kent M; Bauer, Miranda M; Mendoza, Kristelle M; Armién, Aníbal G
Choanal atresia (CA) is a common nasal craniofacial malformation in New World domestic camelids (alpaca and llama). CA results from abnormal development of the nasal passages and is especially debilitating to newborn crias. CA in camelids shares many of the clinical manifestations of a similar condition in humans (CHARGE syndrome). Herein we report on the regulatory gene CHD7 of alpaca, whose homologue in humans is most frequently associated with CHARGE. Sequence of the CHD7 coding region was obtained from a non-affected cria. The complete coding region was 9003 bp, corresponding to a translated amino acid sequence of 3000 aa. Additional genomic sequences corresponding to a significant portion of the CHD7 gene were identified and assembled from the 2x alpaca whole genome sequence, providing confirmatory sequence for much of the CHD7 coding region. The alpaca CHD7 mRNA sequence was 97.9% similar to the human sequence, with the greatest sequence difference being an insertion in exon 38 that results in a polyalanine repeat (A12). Polymorphism in this repeat was tested for association with CA in alpaca by cloning and sequencing the repeat from both affected and non-affected individuals. Variation in length of the poly-A repeat was not associated with CA. Complete sequencing of the CHD7 gene will be necessary to determine whether other mutations in CHD7 are the cause of CA in camelids.
Bohm, M; Richter, J E; Kelsen, S; Thomas, R
The goal of this article is to present the results of the long-term treatment with esophageal dilation of a consecutive series of adults with eosinophilic esophagitis (EoE). EoE in adults is a disease of middle aged white males, with recurrent food impactions and dysphagia. The exact treatment of EoE is unknown due to the uncertainty of the pathogenesis. Currently, the long-term follow-up of adult EoE patients is limited. Sixteen consecutive adult patients (12 males/4 females between ages 27 and 58 years) with EoE underwent a detailed history and baseline upper gastrointestinal endoscopy (EGD) with multiple esophageal biopsies. Thirteen had esophageal dilation. Fifteen were on proton pump inhibitor (PPI) therapy. After dilation, one patient was treated with a restrictive diet. One patient took prn fluticasone. Most of the patients had allergy testing for food and aeroallergens. Follow-up evaluation with similar testing was on average 22 months (range: 12-40 months). Six patients were not available for follow-up. None of the remaining 10 patients had a food impaction; one required further esophageal dilation. Only two patients had intermittent dysphagia. The average dysphagia score decreased from 2.1 to 0.3 (P < 0.002). The average number of eosinophils at follow-up was not significantly different from baseline (120 eosinophils/HPF proximally and 165 eosinophils/HPF distally (P= 0.75). The gross endoscopy findings were unchanged in all patients except one who normalized. A total of 62% and 75% of patients had positive tests for aeroallergens and food allergens, respectively. Over an average of two years, esophageal dilation provided excellent symptomatic relief among 10 adult EoE patients despite no improvement in the mucosal eosinophilia or gross endoscopic appearance.
Esophageal Neoplasm; Esophageal Cancer TNM Staging Primary Tumor (T) T2; Esophageal Cancer TNM Staging Primary Tumor (T) T3; Esophageal Cancer TNM Staging Regional Lymph Nodes (N) N0; Esophageal Cancer TNM Staging Distal Metastasis (M) M0
Triadafilopoulos, G; Tsang, H P; Segall, G M
Cold liquid ingestion may precipitate episodes of dysphagia and chest pain in patients with spastic esophageal motility disorders. The effect of hot liquids on esophageal symptoms, esophageal peristalsis, and clearance and any potential therapeutic benefit in such patients has not been examined. Using esophageal scintigraphy and manometry, we have investigated the effects of hot water swallows on dysphagia, chest pain, and esophageal motility and clearance in patients with esophageal motility disorders. We studied 48 men and women with intermittent dysphagia to both solids and liquids, chest pain, and/or regurgitation. All patients underwent upper endoscopy, barium swallow, and esophageal manometry using standard techniques. Esophageal scintigraphy assessed esophageal transit time (ETT) and retrograde intraesophageal movement of bolus at baseline (22 degrees C) and after hot (60 degrees C) water swallows. Esophageal manometry assessed the amplitude and duration of esophageal contractions in response to baseline and hot water swallows. Patients were followed clinically for as long as 6 months to assess symptomatic response. We found that baseline esophageal scintigraphy revealed a mean ETT of 48.5 seconds; after hot water swallow, mean ETT was 27.8 seconds (p < 0.001). The number of secondary peaks at baseline was 3.5; after hot water swallow, it was 2.1 (p < 0.001). Baseline esophageal manometry showed a mean esophageal body contraction amplitude of 188 mm Hg (mean duration, 11.8 seconds) in response to wet swallows and 125 mm Hg (mean duration, 5.7 seconds) with hot water swallows (p < 0.001). Clinically, 28 (58%) of 48 patients noted significant (>50%) improvement of their symptoms and have been ingesting hot water or other hot liquids regularly with their meals. We conclude that hot water accelerates esophageal clearance, decreases the amplitude and duration of esophageal body contractions, and improves symptoms in patients with esophageal motility disorders
Lim, Lynne Hsueh Yee; Xiang, Ling; del Prado, Jocelynne; Ee Chin, Ling; Beltrame, Millo Achille
Aural atresia and severe microtia are associated malformations that result in problems with hearing and cosmesis, associated speech and language difficulties and diminished self-esteem. In cases where middle ear ossiculoplasty and aural atresia canalplasty are expected to give poor hearing outcomes that would eventually require the use of hearing aids, bone anchored hearing aids or active middle ear implants may be better options. This case report describes a simultaneous Vibrant Soundbridge implantation and 2nd stage auricular reconstruction with rib graft cartilage for an 11-year-old boy with grade III microtia and aural atresia 8 months after the 1st stage reconstruction. Audiometric results of the Vibrant Soundbridge aided ear were comparable to that of the contralateral hearing aid aided ear. PMID:26557321
Kendigelen, Pınar; Tütüncü, Ayşe Çiğdem; Erbabacan, Şafak Emre; Kaya, Güner; Altındaş, Fatiş
Kartagener syndrome is an autosomal recessive disorder characterized by primary ciliary dyskinesia accompanied by sinusitis, bronchiectasis, and situs inversus. Synchronous extrahepatic biliary atresia and Kartagener syndrome are very rare. During the preoperative preparation of patients with Kartagener syndrome, special attention is required for the respiratory and cardiovascular system. It is important to provide suitable anaesthetic management to avoid problems because of ciliary dysfunction in the perioperative period. Further, maintaining an effective pain control with regional anaesthetic methods reduces the risk of pulmonary complications. Infants with biliary atresia operated earlier have a higher chance of survival. Hepatic dysfunction and decrease in plasma proteins are important for the kinetics of drugs. In this presentation, the anaesthetic management of patients with synchronous Kartagener syndrome and biliary atresia, both of which are rare diseases, is evaluated. PMID:27366497
Kendigelen, Pınar; Tütüncü, Ayşe Çiğdem; Erbabacan, Şafak Emre; Kaya, Güner; Altındaş, Fatiş
Kartagener syndrome is an autosomal recessive disorder characterized by primary ciliary dyskinesia accompanied by sinusitis, bronchiectasis, and situs inversus. Synchronous extrahepatic biliary atresia and Kartagener syndrome are very rare. During the preoperative preparation of patients with Kartagener syndrome, special attention is required for the respiratory and cardiovascular system. It is important to provide suitable anaesthetic management to avoid problems because of ciliary dysfunction in the perioperative period. Further, maintaining an effective pain control with regional anaesthetic methods reduces the risk of pulmonary complications. Infants with biliary atresia operated earlier have a higher chance of survival. Hepatic dysfunction and decrease in plasma proteins are important for the kinetics of drugs. In this presentation, the anaesthetic management of patients with synchronous Kartagener syndrome and biliary atresia, both of which are rare diseases, is evaluated.
Verma, Ritu; Jana, Manisha; Bhalla, Ashu Seith; Kumar, Arvind; Kumar, Rakesh
Aural atresia is a rare congenital malformation of the external and middle ear. There are several syndromic associations of this anomaly with those involving the first and second branchial arches. Occurrence of aural atresia with sclerosing skeletal dysplasia is unknown and has never been reported. The co-existence of a sclerosing dysplasia can make the surgical treatment in aural atresia difficult and risky; and the auditory improvement may not be as expected. Moreover, internal auditory canal narrowing and hence sensorineural hearing loss in sclerosing dysplasia might add to the already existing conductive hearing loss in such patients. In this case report we have described an unknown association of bilateral microtia with sclerosing skeletal dysplasia (autosomal dominant osteopetrosis) and clinical implications of these two conditions occurring together leading to a change in the management plan. PMID:27170934
Lim, Lynne Hsueh Yee; Xiang, Ling; Del Prado, Jocelynne; Ee Chin, Ling; Beltrame, Millo Achille
Aural atresia and severe microtia are associated malformations that result in problems with hearing and cosmesis, associated speech and language difficulties and diminished self-esteem. In cases where middle ear ossiculoplasty and aural atresia canalplasty are expected to give poor hearing outcomes that would eventually require the use of hearing aids, bone anchored hearing aids or active middle ear implants may be better options. This case report describes a simultaneous Vibrant Soundbridge implantation and 2(nd) stage auricular reconstruction with rib graft cartilage for an 11-year-old boy with grade III microtia and aural atresia 8 months after the 1(st) stage reconstruction. Audiometric results of the Vibrant Soundbridge aided ear were comparable to that of the contralateral hearing aid aided ear.
Caudle, Susan E; Katzenstein, Jennifer M; Karpen, Saul J; McLin, Valérie A
To test the hypothesis that in very young patients with biliary atresia, cognitive deficits are apparent before transplantation. With the Mullen Scales of Early Learning (Mullen), we examined 15 infants (mean age, 7.8 months) with biliary atresia, correlating Mullen scores with standard clinical and biochemical parameters. Overall, participants displayed significant delays in gross motor and language skills, and fine motor and visual reasoning skills were relatively preserved. The international normalized ratio correlated inversely with gross (P < .01) and fine (P < .05) motor skills. Growth parameters correlated positively with expressive language ability, but length of hospitalization and mode of feeding did not. Age at performance of the Kasai procedure was found to correlate with receptive language performance (P < .05). Very young children with biliary atresia display a characteristic profile of early developmental deficits before transplantation. These findings suggest that early intervention and aggressive nutritional management should be the standard of care to minimize neurocognitive effects. Copyright 2010 Mosby, Inc. All rights reserved.
Henry, M A; Mendes, E F; Saad, L H; Rodrigues, P A; Gonçalves, I
The authors report a case of a patient with complaint of progressive disphagia. Stenoses of lower third of esophagus was revealed by radiological and endoscopic examinations. Fungi were showed in biopsy of lesion, with demonstration of Histoplasm capsulate by tissue culture. Endoscopic dilatation was performed because especific medical treatment failed but esophageal rupture was observed. Partial esophagectomy was performed with symptoms remission.
... Disease Tricuspid Valve Disease Cardiac Rhythm Disturbances Thoracic Aortic Aneurysm Pediatric and Congenital Heart Disease Heart abnormalities that are present at birth in children, as well as in adults Atrial Septal Defect Ventricular Septal ... Arteriosus Single Ventricle Defects Lung, Esophageal, and ...
... 15 or more eosinophils per high-powered microscopic field warrants a diagnosis of EoE. A patient may ... Eosinophilic Esophagitis EoE- How do we diagnose? APFED’s Educational Webinar Series © American Partnership for Eosinophilic Disorders (APFED) ...
Developing statistical models that estimate the probability of developing esophageal cancer over a defined period of time will help clinicians identify individuals at higher risk of specific cancers, allowing for earlier or more frequent screening and counseling of behavioral changes to decrease risk.
Leeman, Matthew F.; Richardson, J. David
Background: The long-term success of bariatric operations for weight reduction has been well documented, but their potential effects on the risk of esophageal cancer have not been evaluated. Methods: We performed operations on 3 patients for esophageal cancer following bariatric operations: 2 had Roux-en-Y gastric bypass, and 1 underwent vertical banded gastroplasty. All of these patients had adenocarcinoma at the gastroesophageal junction; 1 involved the entire intrathoracic esophagus. Results: The intervals between the weight-loss operations and cancer diagnoses were 21, 16, and 14 years. All 3 patients had symptoms of reflux for many years before dysphagia developed and cancer was diagnosed. We performed a limited esophagogastrectomy, a classic IvorLewis procedure, and a total esophagectomy with jejunal free-tissue transfer from stomach to cervical esophagus. Two patients had positive lymph nodes. One patient is alive at 6 years; 2 died at 13 and 15 months after undergoing operation for recurrent cancer. Conclusion: The effect of bariatric operations on gastroesophageal reflux is not known, although gastric bypass has been advocated as the “ultimate antireflux procedure.” The presence of esophageal cancer in these 3 patients years after the weight loss operation is worrisome. We believe that patients who develop new symptoms should have endoscopic evaluation and that epidemiologic studies on the incidence of esophageal cancer occurring years after bariatric operation should be performed. PMID:15554284
Gupta, Shilpi; Gupta, Rahul; Ghosh, Soumyodhriti; Gupta, Arun Kumar; Shukla, Arvind; Chaturvedi, Vinita; Mathur, Praveen
Objective: To evaluate the presentation, management, complications and outcome of intestinal atresia (IA) managed at our center over a period of 1 year. Materials and methods: Records of patients of IA admitted in our center from January 2015 to December 2015 were retrospectively analyzed. Demographic data, antenatal history, presenting complaints, location (duodenal, jejunoileal, colonic) of atresia, surgery performed and peri-operative complications were noted. Results: Total 78 cases of IA were included in the analyses. Mean age and weight at the time of presentation was 5.8 days (range 0-50), and 1.9 kg (range 1.1-3.2), respectively. IA included duodenal atresia [DA (32)], jejuno-ileal atresia [JIA (40)], colonic atresia [CA (3)] and atresia at multiple-location (sites) in 3 cases. Ninety percent of patients underwent surgery within 5 to 20 hours of admission. All cases of DA except one underwent Kimura's diamond shaped duodeno-duodenostomy. One case with perforated duodenal web underwent duodenotomy with excision of web. Seven patients with JIA and CA required primary stoma, while rest were managed by excision of dilated proximal segment and primary anastomosis. Complications included anastomotic leak in 5, proximal perforation in 2, functional obstruction in 7, aspiration pneumonitis in 3, and wound infection in 6 patients. Mean hospital stay for survivors was 11 days. Overall survival was 63%. Conclusion: Late presentation, overcrowding in intensive care unit, septicemia, functional obstruction and anastomotic leak are the causes of poor outcome in our series. Early diagnosis, some modification in surgical technique, use of total parenteral nutrition and adequate investigations for other congenital anomalies may improve the outcome. PMID:27896159
Shivakumar, Pranavkumar; Mourya, Reena; Bezerra, Jorge A.
Background and Aims Biliary atresia represents obstructive cholangiopathy in infants progressing rapidly to cirrhosis and end-stage liver disease. Activated NK cells expressing Nkg2d have been linked to bile duct injury and obstruction by establishing contact with cholangiocytes. To define the mechanisms used by cytotoxic cells, we investigated the role of perforin and granzymes in a neonatal mouse model of rotavirus (RRV)-induced biliary atresia. Methods We used complementary cell lysis assays, flow cytometric analyses, quantitative PCRs and in vivo systems to determine the mechanisms of bile duct epithelial injury and the control of the tissue phenotype in experimental biliary atresia. Results RRV-infected hepatic NK and CD8 T cells increased the expression of perforin and injured cholangiocytes in short-term culture in a perforin-dependent fashion. However, the loss of perforin in vivo delayed but did not prevent the obstruction of bile ducts. Based on the increased expression of granzymes by perforin-deficient cytotoxic cells in long-term cytolytic assays, we found that the inhibition of granzymes by nafamostat mesilate (FUT-175) blocked cholangiocyte lysis. Administration of FUT-175 to perforin-deficient mice after RRV infection decreased the development of jaundice, minimized epithelial injury, and improved long-term survival. However, the inhibition of granzymes alone in wild-type mice was not sufficient to prevent the atresia phenotype in newborn mice. In infants with biliary atresia, hepatic Granzymes A and B mRNA, but not Perforin, increased at the time of portoenterostomy. Conclusions Perforin and granzymes have complementary roles mediating epithelial injury by NK and CD8 T cells. The prevention of experimental biliary atresia can only be achieved by inhibiting both granules. PMID:24096050
Shen, Wen-jun; Dong, Rui; Chen, Gong Zheng, Shan
Highlights: • The RRV infected group showed cholestasis, retardation and extrahepatic biliary atresia. • miR-222 was highly expressed, and PPP2R2A was inhibited in the murine biliary atresia model. • miR-222 profoundly modulated the process of fibrosis in the murine biliary atresia model. • miR-222 might represent a potential target for improving biliary atresia prognosis. - Abstract: microRNA-222 (miR-222) has been shown to initiate the activation of hepatic stellate cells, which plays an important role in the pathogenesis of liver fibrosis. The aim of our study was to evaluate the role of miR-22 in a mouse model of biliary atresia (BA) induced by Rhesus Rotavirus (RRV) infection. New-born Balb/c mice were randomized into control and RRV infected groups. The extrahepatic bile ducts were evaluated. The experimental group was divided into BA group and negative group based on histology. The expression of miR-222, protein phosphatase 2 regulatory subunit B alpha (PPP2R2A), proliferating cell nuclear antigen (PCNA) and phospho-Akt were detected. We found that the experimental group showed signs of cholestasis, retardation and extrahepatic biliary atresia. No abnormalities were found in the control group. In the BA group, miR-222, PCNA and Akt were highly expressed, and PPP2R2A expression was significantly inhibited. Our findings suggest that miR-222 profoundly modulated the process of fibrosis in the murine BA model, which might represent a potential target for improving BA prognosis.
Introduction We report for the first time a unique case of VACTERL (vertebral anomalies, anal atresia or imperforate anus, cardiac anomalies, tracheoesophageal fistula, renal and limb defect) spectrum associated with portal hypertension. The occurrence of both VACTERL spectrum and extrahepatic portal hypertension in a patient has not been reported in the literature. We examined whether or not there was any association between extrahepatic portal hypertension and VACTERL spectrum. Case Presentation A two-and-half-year-old Caucasian girl with VACTERL spectrum presented with hematemesis and abdominal distension. She had caput medusae, ascites, splenomegaly, gastric and esophageal varices. Her liver function tests were within normal limits. Magnetic resonance imaging of the liver with contrast showed a thready portal vein with collateral vessels involving both right and left portal veins without intrahepatic duct dilation. Conclusion A thready portal vein, with features of extrahepatic portal hypertension, is a rare non- VACTERL-type defect in patients with VACTERL spectrum. Understandably, clinicians should give low priority to looking for portal hypertension in VACTERL spectrum patients presenting with gastrointestinal bleeding. However before routinely looking for a thready portal vein and/or extrahepatic portal hypertension in asymptomatic VACTERL spectrum patients, we need further evidence to support this rare association. PMID:20444267
Bhurtel, Dilli Raj; Losa, Ignatius
We report for the first time a unique case of VACTERL (vertebral anomalies, anal atresia or imperforate anus, cardiac anomalies, tracheoesophageal fistula, renal and limb defect) spectrum associated with portal hypertension. The occurrence of both VACTERL spectrum and extrahepatic portal hypertension in a patient has not been reported in the literature. We examined whether or not there was any association between extrahepatic portal hypertension and VACTERL spectrum. A two-and-half-year-old Caucasian girl with VACTERL spectrum presented with hematemesis and abdominal distension. She had caput medusae, ascites, splenomegaly, gastric and esophageal varices. Her liver function tests were within normal limits. Magnetic resonance imaging of the liver with contrast showed a thready portal vein with collateral vessels involving both right and left portal veins without intrahepatic duct dilation. A thready portal vein, with features of extrahepatic portal hypertension, is a rare non- VACTERL-type defect in patients with VACTERL spectrum. Understandably, clinicians should give low priority to looking for portal hypertension in VACTERL spectrum patients presenting with gastrointestinal bleeding. However before routinely looking for a thready portal vein and/or extrahepatic portal hypertension in asymptomatic VACTERL spectrum patients, we need further evidence to support this rare association.
Khan, Shoukat H; Madhu, Vijay P; Rather, Tanveer A; Laway, Bashir A
Background/Aims Esophageal dysmotility is associated with gastrointestinal dysmotility in various systemic and neuroregulatory disorders. Hypothyroidism has been reported to be associated with impaired motor function in esophagus due to accumulation of glycosaminoglycan hyaluronic acid in its soft tissues, leading to changes in various contraction and relaxation parameters of esophagus, particularly in the lower esophageal sphincter. In this study we evaluated esophageal transit times in patients of primary hypothyroidism using the technique of radionuclide esophageal transit scintigraphy. Methods Thirty-one patients of primary hypothyroidism and 15 euthyroid healthy controls were evaluated for esophageal transit time using 15–20 MBq of Technetium-99m sulfur colloid diluted in 10–15 mL of drinking water. Time activity curve was generated for each study and esophageal transit time was calculated as time taken for clearance of 90% radioactive bolus from the region of interest encompassing the esophagus. Esophageal transit time of more than 10 seconds was considered as prolonged. Results Patients of primary hypothyroidism had a significantly increased mean esophageal transit time of 19.35 ± 20.02 seconds in comparison to the mean time of 8.25 ± 1.71 seconds in healthy controls (P < 0.05). Esophageal transit time improved and in some patients even normalized after treatment with thyroxine. A positive correlation (r = 0.39, P < 0.05) albeit weak existed between the serum thyroid stimulating hormone and the observed esophageal transit time. Conclusions A significant number of patients with primary hypothyroidism may have subclinical esophageal dysmotility with prolonged esophageal transit time which can be reversible by thyroxine treatment. Prolonged esophageal transit time in primary hypothyroidism may correlate with serum thyroid stimulating hormone levels. PMID:27444283
Khan, Shoukat H; P, Madhu Vijay; Rather, Tanveer A; Laway, Bashir A
Esophageal dysmotility is associated with gastrointestinal dysmotility in various systemic and neuroregulatory disorders. Hypothyroidism has been reported to be associated with impaired motor function in esophagus due to accumulation of glycosaminoglycan hyaluronic acid in its soft tissues, leading to changes in various contraction and relaxation parameters of esophagus, particularly in the lower esophageal sphincter. In this study we evaluated esophageal transit times in patients of primary hypothyroidism using the technique of radionuclide esophageal transit scintigraphy. Thirty-one patients of primary hypothyroidism and 15 euthyroid healthy controls were evaluated for esophageal transit time using 15-20 MBq of Technetium-99m sulfur colloid diluted in 10-15 mL of drinking water. Time activity curve was generated for each study and esophageal transit time was calculated as time taken for clearance of 90% radioactive bolus from the region of interest encompassing the esophagus. Esophageal transit time of more than 10 seconds was considered as prolonged. Patients of primary hypothyroidism had a significantly increased mean esophageal transit time of 19.35 ± 20.02 seconds in comparison to the mean time of 8.25 ± 1.71 seconds in healthy controls (P < 0.05). Esophageal transit time improved and in some patients even normalized after treatment with thyroxine. A positive correlation (r = 0.39, P < 0.05) albeit weak existed between the serum thyroid stimulating hormone and the observed esophageal transit time. A significant number of patients with primary hypothyroidism may have subclinical esophageal dysmotility with prolonged esophageal transit time which can be reversible by thyroxine treatment. Prolonged esophageal transit time in primary hypothyroidism may correlate with serum thyroid stimulating hormone levels.
Stage I Esophageal Adenocarcinoma; Stage II Esophageal Adenocarcinoma; Stage III Esophageal Adenocarcinoma; Stage I Esophageal Squamous Cell Carcinoma; Stage II Esophageal Squamous Cell Carcinoma; Stage III Esophageal Squamous Cell Carcinoma
Colnaghi, M; Condo, V; Gagliardi, L; Mirabile, L; Fumagalli, M; Mosca, F
Laryngeal atresia is a rare congenital cause of high airway obstruction that can lead to death if not correctly recognized and treated at birth. Postnatal management is difficult and the prognosis is often poor. We report a case of prenatal diagnosis of laryngeal atresia in a fetus that was delivered preterm at 29 weeks of gestation. Tracheotomy was performed as an ex utero intrapartum treatment (EXIT) to guarantee patent airway, and laryngotracheoplasty was performed at 22 months of corrected age. A favorable ventilatory and neurodevelopmental outcome was observed at 33 months of age. Copyright (c) 2007 ISUOG.
Megumi, Y; Horii, Y; Matsuda, T; Arai, Y; Takeuchi, H; Yoshida, O
A case of prune belly syndrome associated with urethral stricture and atresia ani is reported. Cystostomy and colostomy were performed on his birthday. Cutaneous vesicostomy, bilateral ureterocystoneoplasty and proctoplasty were performed when the patient was one year old, and urethrotomy was performed when he was four years old. He was closely followed for six years and six months, and had developed without any serious episodes. We collected 128 cases of prune belly syndrome from the Japanese literature including this, and made a brief discussion of this syndrome with urethral stricture and atresia ani.
Chkadua, T Z; Ivanova, M D; Daminov, R O; Brusova, L A; Savvateeva, D M
The paper presents the analysis of clinical case of endoscopic nasal breath restoration and elimination of the secondary cleft lip nasal deformity in 27 years old patient with unilateral choanal atresia and secondary nasal deformity after rhinocheiloplasty. Preoperative examination revealed the absence of nasal breathing on collateral side due to complete bone choanal atresia. Surgical treatment included endoscopic choanal repair, elimination of the secondary nasal deformity, septoplasty, conchotomy and lateroposition of the inferior conchae. The treatment resulted in nasal breath restoration and elimination of nasal deformity. Long-term follow-up at 1 and 12 months post-operatively proved stable positive aesthetic and functional results.
Tou, Sandra P; Keene, Bruce W; Barker, Piers C A
Pulmonary atresia and ventricular septal defect (PA-VSD) was diagnosed in a 2-year-old castrated male Terrier mix. Transthoracic echocardiography identified a large ventricular septal defect, overriding aorta and severe right ventricular hypertrophy. A main pulmonary artery could not be identified, consistent with pulmonary atresia or persistent truncus arteriosus. Transesophageal echocardiography and angiography confirmed PA-VSD with aortopulmonary collateral circulation arising from the descending thoracic aorta. This case report describes the antemortem diagnosis of the rare congenital defect PA-VSD in an adult dog.
Oh, Chaeyoun; Lee, Sanghoon; Lee, Suk-Koo; Seo, Jeong-Meen
Currently, a diamond-shaped anastomosis is preferred for the surgical repair of duodenal atresia (DA) in both open and laparoscopic surgery. We report the results of laparoscopic duodenoduodenostomy with parallel anastomosis (LDPA) in DA. We retrospectively reviewed 22 patients who underwent laparoscopic duodenoduodenostomy from February 2005 to May 2015 in Samsung Medical Center. All patients underwent operation within the first month after birth. Patients who were transversely anastomosed after duodenotomy and patients who underwent simultaneous operation on combined anomalies were excluded. Parallel anastomosis was used in all surgeries. Four trocars were used in laparoscopic repair. After mobilization of both proximal and distal ends, the proximal end was incised transversely and the distal end was incised longitudinally. Duodenoduodenostomy with parallel anastomosis using a 5-0 glyconate monofilament was performed with interrupted sutures. Eleven patients (50 %) were male. Median gestational age was 36 + 6 weeks (32 + 7-40 + 6). Median age at the time of operation and median body weight were 3 days (1-12) and 2.53 kg (1.63-3.18), respectively. All patients were diagnosed prenatally and 16 patients (72.7 %) had associated anomalies. Median operation time was 142 min (96-290) and median postoperative day to start oral feeding was 5 days (3-9) and median postoperative day of reaching full feeding was 11 days (6-19). Median postoperative day was 13 days (10-60). There was no anastomotic leakage or stenosis. Median follow up was 3.5 months (1-21). Currently, there is no late complication. LDPA can be performed easily to patients who have DA in neonatal period. It is anatomically natural and the risk of leakage or stenosis does not seem significant. Therefore, parallel anastomosis should be considered as a safe procedural option for laparoscopic duodenoduodenostomy in DA.
El-Guindi, Mohamed Abdel-Salam; Sira, Mostafa Mohamed; Sira, Ahmad Mohamed; Salem, Tahany Abdel-Hameed; El-Abd, Osama Lotfy; Konsowa, Hatem Abdel-Sattar; El-Azab, Dina Shehata; Allam, Alif Abdel-Hakim
The dilemma of early diagnosis of biliary Atresia (BA), particularly distinguishing it from other causes of neonatal cholestasis is challenging. The aim was to design and validate a scoring system for early discrimination of BA from other causes of neonatal cholestasis. A twelve-point scoring system was proposed according to clinical, laboratory, ultrasonographic, and histopathological parameters. A total of 135 patients with neonatal cholestasis in two sets were recruited to design (n=60) and validate (n=75) a scoring system. Parameters with significant statistical difference between BA (n=30) and non-BA (n=30) patients in the design set were analyzed by logistic regression to predict the presence or absence of BA then a scoring system was designed and validated. The total score ranged from 0 to 37.18 and a cut-off value of >23.927 could discriminate BA from other causes of neonatal cholestasis with sensitivity and specificity of 100% each. By applying this score in the validation set, the accuracy was 98.83% in predicting BA. The diagnosis of BA was proposed correctly in 100% and the diagnosis of non-BA was proposed correctly in 97.67% of patients. By applying this model, unnecessary intraoperative cholangiography would be avoided in non-BA patients. This scoring system accurately separates infants with BA and those with non-BA, rendering intraoperative cholangiography for confirming or excluding BA unnecessary in a substantial proportion of patients. Copyright © 2014 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.
Samo, Salih; Carlson, Dustin A; Kahrilas, Peter J; Pandolfino, John E
The clinical significance of minor esophageal motility disorders is unclear, though they typically carry a benign course. Distal esophageal spasm progressing to achalasia has been reported, although it appears to be rare. We report a case of a patient with dysphagia and chest pain who was found to have ineffective esophageal motility on high-resolution manometry, which developed into distal esophageal spasm and then progressed to type III achalasia.
Carlson, Dustin A.; Kahrilas, Peter J.; Pandolfino, John E.
The clinical significance of minor esophageal motility disorders is unclear, though they typically carry a benign course. Distal esophageal spasm progressing to achalasia has been reported, although it appears to be rare. We report a case of a patient with dysphagia and chest pain who was found to have ineffective esophageal motility on high-resolution manometry, which developed into distal esophageal spasm and then progressed to type III achalasia. PMID:28119934
Liu, Hanjun; Wan, Mingxi; Wang, Supin; Wang, Xiaodong; Lu, Chunmei
The present study attempted to investigate the acoustic characteristics of Mandarin laryngeal and esophageal speech. Eight normal laryngeal and seven esophageal speakers participated in the acoustic experiments. Results from acoustic analyses of syllables /ma/and /ba/ indicated that, F0, intensity, and signal-to-noise ratio of laryngeal speech were significantly higher than those of esophageal speech. However, opposite results were found for vowel duration, jitter, and shimmer. Mean F0, intensity, and word per minute in reading were greater but number of pauses was smaller in laryngeal speech than those in esophageal speech. Similar patterns of F0 contours and vowel duration as a function of tone were found between laryngeal and esophageal speakers. Long-time spectra analysis indicated that higher first and second formant frequencies were associated with esophageal speech than that with normal laryngeal speech.
Rathinam, S; Makarawo, T; Norton, R; Collins, F J
Esophageal perforation is a difficult problem in thoracic surgery. Esophageal perforations can be spontaneous, iatrogenic, or malignant. We report two cases of esophageal perforations caused by thoracic osteophytes and different management strategies leading to successful outcomes. An 80-year-old male presented with chest pain and dysphagia following a fall. On endoscopy, an esophageal perforation and foreign body was noted which was confirmed as a thoracic osteophyte on computed tomography scan. He was managed conservatively as he declined surgery. A 63-year-old male was admitted with dysphagia following a food bolus obstruction. Following esophagoscopy and dilatation, there was clinical and radiological evidence of perforation. During surgery, a thoracic osteophyte was identified as the cause of perforation. The perforation was closed in layers and the osteophyte was trimmed. Both patients recovered well. Thoracic osteophytes are a rare cause of esophageal perforations and a high index of suspicion is required in patients with osteoarthritis who present with esophageal perforations.
Triadafilopoulos, G; Boeckxstaens, G E; Gullo, R; Patti, M G; Pandolfino, J E; Kahrilas, P J; Duranceau, A; Jamieson, G; Zaninotto, G
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.
Tamaki, Tetsuro; Hirata, Maki; Nakajima, Nobuyuki; Saito, Kosuke; Hashimoto, Hiroyuki; Soeda, Shuichi; Uchiyama, Yoshiyasu; Watanabe, Masahiko
Losses in vital functions of the somatic motor and sensory nervous system are induced by severe long-gap peripheral nerve transection injury. In such cases, autologous nerve grafts are the gold standard treatment, despite the unavoidable sacrifice of other healthy functions, whereas the prognosis is not always favorable. Here, we use human skeletal muscle-derived stem cells (Sk-SCs) to reconstitute the function after long nerve-gap injury. Muscles samples were obtained from the amputated legs from 9 patients following unforeseen accidents. The Sk-SCs were isolated using conditioned collagenase solution, and sorted as CD34+/45- (Sk-34) and CD34-/45-/29+ (Sk-DN/29+) cells. Cells were separately cultured/expanded under optimal conditions for 2 weeks, then injected into the athymic nude mice sciatic nerve long-gap model (7-mm) bridging an acellular conduit. After 8-12 weeks, active cell engraftment was observed only in the Sk-34 cell transplanted group, showing preferential differentiation into Schwann cells and perineurial/endoneurial cells, as well as formation of the myelin sheath and perineurium/endoneurium surrounding regenerated axons, resulted in 87% of numerical recovery. Differentiation into vascular cell lineage (pericyte and endothelial cells) were also observed. A significant tetanic tension recovery (over 90%) of downstream muscles following electrical stimulation of the sciatic nerve (at upper portion of the gap) was also achieved. In contrast, Sk-DN/29+ cells were completely eliminated during the first 4 weeks, but relatively higher numerical (83% vs. 41% in axon) and functional (80% vs. 60% in tetanus) recovery than control were observed. Noteworthy, significant increase in the formation of vascular networks in the conduit during the early stage (first 2 weeks) of recovery was observed in both groups with the expression of key factors (mRNA and protein levels), suggesting the paracrine effects to angiogenesis. These results suggested that the human Sk
Hirata, Maki; Nakajima, Nobuyuki; Saito, Kosuke; Hashimoto, Hiroyuki; Soeda, Shuichi; Uchiyama, Yoshiyasu; Watanabe, Masahiko
Losses in vital functions of the somatic motor and sensory nervous system are induced by severe long-gap peripheral nerve transection injury. In such cases, autologous nerve grafts are the gold standard treatment, despite the unavoidable sacrifice of other healthy functions, whereas the prognosis is not always favorable. Here, we use human skeletal muscle-derived stem cells (Sk-SCs) to reconstitute the function after long nerve-gap injury. Muscles samples were obtained from the amputated legs from 9 patients following unforeseen accidents. The Sk-SCs were isolated using conditioned collagenase solution, and sorted as CD34+/45- (Sk-34) and CD34-/45-/29+ (Sk-DN/29+) cells. Cells were separately cultured/expanded under optimal conditions for 2 weeks, then injected into the athymic nude mice sciatic nerve long-gap model (7-mm) bridging an acellular conduit. After 8–12 weeks, active cell engraftment was observed only in the Sk-34 cell transplanted group, showing preferential differentiation into Schwann cells and perineurial/endoneurial cells, as well as formation of the myelin sheath and perineurium/endoneurium surrounding regenerated axons, resulted in 87% of numerical recovery. Differentiation into vascular cell lineage (pericyte and endothelial cells) were also observed. A significant tetanic tension recovery (over 90%) of downstream muscles following electrical stimulation of the sciatic nerve (at upper portion of the gap) was also achieved. In contrast, Sk-DN/29+ cells were completely eliminated during the first 4 weeks, but relatively higher numerical (83% vs. 41% in axon) and functional (80% vs. 60% in tetanus) recovery than control were observed. Noteworthy, significant increase in the formation of vascular networks in the conduit during the early stage (first 2 weeks) of recovery was observed in both groups with the expression of key factors (mRNA and protein levels), suggesting the paracrine effects to angiogenesis. These results suggested that the human Sk
Miwa, Hiroto; Kondo, Takashi; Oshima, Tadayuki; Fukui, Hirokazu; Tomita, Toshihiko; Watari, Jiro
Noxious stimuli in the esophagus activate nociceptive receptors on esophageal mucosa, such as transient receptor potential, acid-sensing ion channel and the P2X family, a family of ligand-gated ion channels responsive to ATP, and this generates signals that are transmitted to the central nervous system via either spinal nerves or vagal nerves, resulting in esophageal sensation. Among the noxious stimuli, gastric acid and other gastric contents are clinically most important, causing typical reflux symptoms such as heartburn and regurgitation. A conventional acid penetration theory has been used to explain the mechanism of heartburn, but much recent evidence does not support this theory. Therefore, it may be necessary to approach the causes of heartburn symptoms from a new conceptual framework. Hypersensitivity of the esophagus, like that of other visceral organs, includes peripheral, central and probably psychosocial factor-mediated hypersensitivity, and is known to play crucial roles in the pathoegenesis of nonerosive reflux disease, functional heartburn and non-cardiac chest pain. There also are esophagitis patients who do not perceive typical symptoms. This condition is known as silent gastroesophageal reflux disease. Although the pathogenesis of silent gastroesophageal reflux disease is still not known, hyposensitivity to reflux of acid may possibly explain the condition.
Weinbrand-Goichberg, Jenny; Segal, Idit; Ovadia, Adi; Levine, Arie; Dalal, Ilan
Eosinophilic esophagitis (EoE) is an emerging disease defined by esophageal dysfunction, by typical endoscopic findings and by abnormal eosinophilic inflammation within the esophagus. Eosinophilic accumulation in the esophagus occurs as a result of esophageal overexpression of pro-inflammatory mediators, including T cells and mast cells, cytokines such as interleukin (IL)-13, IL-5 and IL-15, as well as chemoattractants (eotaxin and transforming growth factor-β1, fibroblast growth factor and the newly characterized gene--thymic stromal lymphopoietin, which is a key regulator of allergic sensitization initiation). The role of allergy, particularly food allergy in EoE is indisputable, as elimination diet is a proven commonly used treatment for the disease. However, unlike classical immediate IgE-mediated reaction to allergen, EoE is associated with an altered immune response, characterized by a combination of IgE-mediated and non-IgE-mediated mechanisms. In this review, we aim to discuss the many typical aspects of EoE as opposed to other entities involving the esophagus, with focusing on the aberrant immune-mediated key players contributing to the pathogenesis of this unique disease.
Martinucci, Irene; de Bortoli, Nicola; Giacchino, Maria; Bodini, Giorgia; Marabotto, Elisa; Marchi, Santino; Savarino, Vincenzo; Savarino, Edoardo
Esophageal motility abnormalities are among the main factors implicated in the pathogenesis of gastroesophageal reflux disease. The recent introduction in clinical and research practice of novel esophageal testing has markedly improved our understanding of the mechanisms contributing to the development of gastroesophageal reflux disease, allowing a better management of patients with this disorder. In this context, the present article intends to provide an overview of the current literature about esophageal motility dysfunctions in patients with gastroesophageal reflux disease. Esophageal manometry, by recording intraluminal pressure, represents the gold standard to diagnose esophageal motility abnormalities. In particular, using novel techniques, such as high resolution manometry with or without concurrent intraluminal impedance monitoring, transient lower esophageal sphincter (LES) relaxations, hypotensive LES, ineffective esophageal peristalsis and bolus transit abnormalities have been better defined and strongly implicated in gastroesophageal reflux disease development. Overall, recent findings suggest that esophageal motility abnormalities are increasingly prevalent with increasing severity of reflux disease, from non-erosive reflux disease to erosive reflux disease and Barrett's esophagus. Characterizing esophageal dysmotility among different subgroups of patients with reflux disease may represent a fundamental approach to properly diagnose these patients and, thus, to set up the best therapeutic management. Currently, surgery represents the only reliable way to restore the esophagogastric junction integrity and to reduce transient LES relaxations that are considered to be the predominant mechanism by which gastric contents can enter the esophagus. On that ground, more in depth future studies assessing the pathogenetic role of dysmotility in patients with reflux disease are warranted.
Del Hierro, Piedad Magdalena
Acute Necrotizing Esophagitis is an uncommon pathology, characterized by endoscopic finding of diffuse black coloration in esophageal mucosa and histological presence of necrosis in patients with upper gastrointestinal bleeding. The first case of acute necrotizing esophagitis followed by duodenal necrosis, in 81 years old woman with a positive history of Type 2 Diabetes Mellitus, Hypertension, and usual intake of Nonsteroidal Anti-inflammatory drugs, is reported. Although its etiology remains unknown, the duodenal necrosis suggests that ischemia could be the main cause given that the branches off the celiac axis provide common blood supply to the distal esophageal and duodenal tissue. The massive gastroesophagic reflux and NSAID intake could be involved.
Tahan, Veysel; Sayrak, Hakan; Bayar, Nevzat; Erer, Burak; Tahan, Gulgun; Dane, Faysal
Introduction Doxycycline-induced esophageal ulcer patients are mostly young persons with no history of esophageal dysfunction. Heartburn, midsternal pain and dysphagia are the most common symptoms. It has generally a benign course. The present case is the first report of doxycycline-induced extensive ulcerations, mimicking esophageal cancer in two esophageal segments alongside, in the literature. Case presentation This report describes a 16-year-old Caucasian girl who, while taking doxycycline capsules100 mg twice a day for acne vulgaris for 3 months, developed these symptoms. An upper endoscopy revealed multiple circumferential deep ulcerations surrounding fragile, irregular, hyperemic and hypertrophic mucosa at the level of the mid-esophagus and concomitantly in the lower esophageal sphincter. The lesions were biopsied to exclude esophageal carcinoma because of the suspicious appearance in the endoscopic examination. The histopathological examination, haematoxylin and eosin stained sections showed ulceration with a mixed inflammatory infiltrate. Doxycycline was discontinued and she was given sucralfate 1 g qid and omeprazole 20 mg bid orally. All symptoms of the patient were resolved on the third day of the treatment. After 4 weeks of the therapy, an upper endoscopic control examination demonstrated normal findings. Conclusion The present case has been an uncommon presentation of doxycycline-induced extensive ulcerations, mimicking esophageal cancer in two esophageal segments, concomitantly. Even the lesions were biopsied to exclude esophageal carcinoma. A modification on the behavior of taking drugs can prevent these unpleasant complications. PMID:18778470
Guillet, J.; Wynchank, S.; Basse-Cathalinat, B.; Christophe, E.; Ducassou, D.; Blanquet, P.
Esophageal transit of a small volume of watery liquid has been observed scintigraphically in 200 studies performed on patients aged between 6 days and 16 years. Qualitative information concerning esophageal morphology and function in the various phases of deglutition, and scintigraphic features of achalasia, stenosis, and other pathologies are described. Measured esophageal transit time and its normal variation, its relevance to the diagnosis of esophagitis, and the monitoring of treatment are discussed. This technique observing distinct deglutitions has proven a useful diagnostic tool. Its advantages and limitations are discussed in comparison with other methods.
Nair, Dhanya Venugopalan; Reddy, A. Gopala
The incidence of esophageal cancer is rapidly increasing especially in developing countries. The major risk factors include unhealthy lifestyle practices such as alcohol consumption, smoking, and chewing tobacco to name a few. Diagnosis at an advanced stage and poor prognosis make esophageal cancer one of the most lethal diseases. These factors have urged further research in understanding the pathophysiology of the disease. Animal models not only aid in understanding the molecular pathogenesis of esophageal cancer but also help in developing therapeutic interventions for the disease. This review throws light on the various recent laboratory animal models for esophageal cancer. PMID:27956773
Atsumi, Kazushige; Shioyama, Yoshiyuki; Arimura, Hidetaka; Terashima, Kotaro; Matsuki, Takaomi; Ohga, Saiji; Yoshitake, Tadamasa; Nonoshita, Takeshi; Tsurumaru, Daisuke; Ohnishi, Kayoko; Asai, Kaori; Matsumoto, Keiji; Nakamura, Katsumasa; Honda, Hiroshi
Purpose: To determine clinical factors for predicting the frequency and severity of esophageal stenosis associated with tumor regression in radiotherapy for esophageal cancer. Methods and Materials: The study group consisted of 109 patients with esophageal cancer of T1-4 and Stage I-III who were treated with definitive radiotherapy and achieved a complete response of their primary lesion at Kyushu University Hospital between January 1998 and December 2007. Esophageal stenosis was evaluated using esophagographic images within 3 months after completion of radiotherapy. We investigated the correlation between esophageal stenosis after radiotherapy and each of the clinical factors with regard to tumors and therapy. For validation of the correlative factors for esophageal stenosis, an artificial neural network was used to predict the esophageal stenotic ratio. Results: Esophageal stenosis tended to be more severe and more frequent in T3-4 cases than in T1-2 cases. Esophageal stenosis in cases with full circumference involvement tended to be more severe and more frequent than that in cases without full circumference involvement. Increases in wall thickness tended to be associated with increases in esophageal stenosis severity and frequency. In the multivariate analysis, T stage, extent of involved circumference, and wall thickness of the tumor region were significantly correlated to esophageal stenosis (p = 0.031, p < 0.0001, and p = 0.0011, respectively). The esophageal stenotic ratio predicted by the artificial neural network, which learned these three factors, was significantly correlated to the actual observed stenotic ratio, with a correlation coefficient of 0.864 (p < 0.001). Conclusion: Our study suggested that T stage, extent of involved circumference, and esophageal wall thickness of the tumor region were useful to predict the frequency and severity of esophageal stenosis associated with tumor regression in radiotherapy for esophageal cancer.
Wood, Michele; Shaw, Paul
Pradaxa (dabigatran) is a direct thrombin inhibitor approved for prevention of stroke and systemic embolism in patients with non-valvular atrial fibrillation. We describe a case of esophageal ulceration associated with Pradaxa administration in a 75-year-old man. The patient reported difficulty swallowing and a burning sensation after taking his first dose of Pradaxa. An esophagogastroduodenoscopy (EGD) revealed linear ulcerations in the mid-esophagus. Pradaxa was held beginning the day before the EGD. The patient reported that his pain and difficulty swallowing resolved on stopping Pradaxa. Pradaxa is formulated with a tartaric acid excipient to reduce variability in absorption. We hypothesise that the capsule lodged in the patient's esophagus and the tartaric acid may have caused local damage resulting in an esophageal ulcer. It is important to educate patients on proper administration of Pradaxa, to decrease the risk of this rare, but potentially serious adverse event.
Wechsler, Joshua B; Bryce, Paul J
Paralleling the overall trend in allergic diseases, Eosinophilic Esophagitis is rapidly increasing in incidence. It is associated with food antigen-triggered, eosinophil-predominant inflammation and the pathogenic mechanisms have many similarities to other chronic atopic diseases, such as eczema and allergic asthma. Studies in animal models and from patients over the last 15 years have suggested that allergic sensitization leads to food-specific IgE and T-helper lymphocyte type 2 cells, both of which appear to contribute to the pathogenesis along with basophils, mast cells, and antigen-presenting cells. This review will outline our current understandings of the allergic mechanisms that drive eosinophilic esophagitis, drawing from clinical and translational studies in humans as well as experimental animal models. PMID:24813516
Ali, Tuncer Ahmet; Afra, Karavelioğlu; Didem, Baskin Embleton; Muhsin, Elmas
Holt–Oram syndrome (HOS) is a rare autosomal dominant disorder, characterized by upper limb dysplasia and congenital cardiac defect. We report two cases with HOS, first associated with renal agenesis, coronal hypospadias, urethral duplication and second associated with duodenal atresia and horseshoe kidney that have not been reported in English literature. PMID:27695215
Mun, Da-Na; Park, Chun Soo; Kim, Young-Hwue; Goo, Hyun Woo
A multistage plan and multidisciplinary approach are the keys to successful repair in patients with pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCAs). In this article, we present a multidisciplinary approach adopted to treat a patient with PA with VSD and MAPCAs associated with left pulmonary artery interruption.
Vallefuoco, Rosario; Alleaume, Charline; Jardel, Nicolas; Maenhoudt, Cindy; Cordonnier, Nathalie
A combination of gastrointestinal and urogenital congenital abnormalities was diagnosed and surgically treated in a kitten. Physical examination, exploratory laparotomy, castration, histological examination, and cytogenetic karyotyping were utilized to determine the true gender of the kitten. The kitten was confirmed to be a male (38 XY) pseudohermaphrodite with Type II atresia ani and rectovaginal fistula. PMID:24155431
Shivakumar, Pranavkumar; Mizuochi, Tatsuki; Mourya, Reena; Gutta, Sridevi; Yang, Li; Luo, Zhenhua; Bezerra, Jorge A.
Biliary atresia is an obstructive cholangiopathy of infancy that progresses to end-stage cirrhosis. Although the pathogenesis of the disease is not completely understood, previous reports link TNFα to apoptosis of the bile duct epithelium in the presence of IFNγ. Here, we investigate if TNFα signaling regulates pathogenic mechanisms of biliary atresia. First, we quantified the expression of TNFA and its receptors TNFR1 and TNFR2 in human livers and found an increased expression of the receptors at the time of diagnosis. In mechanistic experiments using a neonatal mouse model of rhesus rotavirus–induced (RRV-induced) biliary atresia, the expression of the ligand and both receptors increased 6- to 8-fold in hepatic DCs and NK lymphocytes above controls. The activation of tissue NK cells by RRV-primed DCs was independent of TNFα-TNFR signaling. Once activated, the expression of TNFα by NK cells induced lysis of 55% ± 2% of bile duct epithelial cells, which was completely prevented by blocking TNFα or TNFR2, but not TNFR1. More notably, antibody-mediated or genetic disruption of TNFα-TNFR2 signaling in vivo decreased apoptosis and epithelial injury; suppressed the infiltration of livers by T cells, DCs, and NK cells; prevented extrahepatic bile duct obstruction; and promoted long-term survival. These findings point to a key role for the TNFα/TNFR2 axis on pathogenesis of experimental biliary atresia and identify new therapeutic targets to suppress the disease phenotype. PMID:28289704
Vallefuoco, Rosario; Alleaume, Charline; Jardel, Nicolas; Maenhoudt, Cindy; Cordonnier, Nathalie
A combination of gastrointestinal and urogenital congenital abnormalities was diagnosed and surgically treated in a kitten. Physical examination, exploratory laparotomy, castration, histological examination, and cytogenetic karyotyping were utilized to determine the true gender of the kitten. The kitten was confirmed to be a male (38 XY) pseudohermaphrodite with Type II atresia ani and rectovaginal fistula.
Gerestein, C.G.; Berger, R.M.F.; Dalinghaus, M.; Bogers, A.J.J.C.; Witsenburg, M.
Background Pulmonary atresia and intact ventricular septum is characterised by a great morphological variety. Treatment is not uniform. Objective To evaluate our experience with transcatheter valvotomy and balloon dilatation in neonates with pulmonary atresia and intact ventricular septum. Design Retrospective. Methods Between January 1997 and September 2000 five neonates with pulmonary atresia and intact ventricular septum underwent transcatheter valvotomy and balloon dilatation. Results The catheter intervention was performed at a mean age of 27 days (range 3-95 days). The atretic pulmonary valve was successfully perforated in all neonates. Subsequent balloon dilatation was successful in four neonates. Balloon dilatation was unsuccessful in one patient, who underwent an elective surgical valvotomy of the pulmonary valve after five days. Three patients needed a modified Blalock-Taussig shunt after a mean of 23 days. Four patients required repeated balloon dilatation after a mean of 227 days. Mean follow-up was 2.7 years (range 1-5 years). Conclusions Transcatheter perforation of the pulmonary valve membrane and balloon dilatation is a good, safe initial therapy in selected neonates with pulmonary atresia and intact ventricular septum. This procedure can prevent open-heart surgery in these patients in the first months of life. ImagesFigure 1Figure 2 PMID:25696158
Parsons, J M; Rees, M R; Gibbs, J L
A neonate with pulmonary atresia and an intact ventricular septum with a tripartite right ventricle was successfully treated by percutaneous balloon dilatation of the pulmonary valve. This was facilitated by previous laser valvotomy with a hot tip Trimedyne laser wire. There were no major complications. Four weeks later the patient was discharged home on no medication with peripheral oxygen saturations of 70% in air.
Background Several types of congenital lesions can cause complete or incomplete obstruction of the intestine. Our purpose is to present 3 neonates with dual intestinal type I atresia, i.e., simultaneous obstructive lesions at 2 locations in which the atresia manifested as diaphragm-like tissue. Case presentation All 3 cases were female infants ranging in age from 2 to 14 months. The common symptom in all cases was intermittent persistent vomiting. In some cases the vomitus was bilious, and other symptoms included abdominal distention and delayed meconium passage. Prior surgeries at another hospital were unsuccessful at relieving the symptoms in one case. One case had dual lesions in the colon, one dual lesions in the duodenum, and one atresia at both the distal portion of the ileum and the descending colon. Surgical exploration and removal of the lesions at our hospital was successful in all cases, and the infants were discharged in good condition. Conclusions Type I atresia can manifest as a diaphragm-like tissue obstructing the continuity of gastrointestinal tract, and in rare cases multiple areas may be present. Base on the intermittent nature of the associated symptoms, diagnosis can be difficult and is often delayed. Physicians should be aware of this condition during the work-up of an infant with persistent intermittent vomiting. PMID:24928109
Matsuda, Fuko; Inoue, Naoko; Manabe, Noboru; Ohkura, Satoshi
The mammalian ovary is an extremely dynamic organ in which a large majority of follicles are effectively eliminated throughout their reproductive life. Due to the numerous efforts of researchers, mechanisms regulating follicular growth and atresia in mammalian ovaries have been clarified, not only their systemic regulation by hormones (gonadotropins) but also their intraovarian regulation by gonadal steroids, growth factors, cytokines and intracellular proteins. Granulosa cells in particular have been demonstrated to play a major role in deciding the fate of follicles, serving molecules that are essential for follicular growth and maintenance as well as killing themselves by an apoptotic process that results in follicular atresia. In this review, we discuss the factors that govern follicular growth and atresia, with a special focus on their regulation by granulosa cells. First, ovarian folliculogenesis in adult life is outlined. Then, we explain about the regulation of follicular growth and atresia by granulosa cells, in which hormones, growth factors and cytokines, death ligand-receptor system and B cell lymphoma/leukemia 2 (BCL2) family members (mitochondria-mediated apoptosis) are further discussed.
Worku, Tesfaye; Rehman, Zia Ur; Talpur, Hira Sajjad; Bhattarai, Dinesh; Ullah, Farman; Malobi, Ngabu; Kebede, Tesfaye; Yang, Liguo
Our understanding of the post-transcriptional mechanisms involved in follicular atresia is limited; however, an important development has been made in understanding the biological regulatory networks responsible for mediating follicular atresia. MicroRNAs have come to be seen as a key regulatory actor in determining cell fate in a wide range of tissues in normal and pathological processes. Profiling studies of miRNAs during follicular atresia and development have identified several putative miRNAs enriched in apoptosis signaling pathways. Subsequent in vitro and/or in vivo studies of granulosa cells have elucidated the functional role of some miRNAs along with their molecular pathways. In particular, the regulatory roles of some miRNAs have been consistently observed during studies of follicular cellular apoptosis. Continued work should gradually lead to better understanding of the role of miRNAs in this field. Ultimately, we expect this understanding will have substantial benefits for fertility management at both the in vivo or/and in vitro levels. The stable nature of miRNA holds remarkable promise in clinical use as a diagnostic tool and in reproductive medicine to solve the ever-increasing fertility problem. In this review, we summarize current knowledge of the involvement of miRNAs in follicular atresia, discuss the challenges for further work and pinpoint areas for future research.
Worku, Tesfaye; Rehman, Zia Ur; Talpur, Hira Sajjad; Bhattarai, Dinesh; Ullah, Farman; Malobi, Ngabu; Kebede, Tesfaye; Yang, Liguo
Our understanding of the post-transcriptional mechanisms involved in follicular atresia is limited; however, an important development has been made in understanding the biological regulatory networks responsible for mediating follicular atresia. MicroRNAs have come to be seen as a key regulatory actor in determining cell fate in a wide range of tissues in normal and pathological processes. Profiling studies of miRNAs during follicular atresia and development have identified several putative miRNAs enriched in apoptosis signaling pathways. Subsequent in vitro and/or in vivo studies of granulosa cells have elucidated the functional role of some miRNAs along with their molecular pathways. In particular, the regulatory roles of some miRNAs have been consistently observed during studies of follicular cellular apoptosis. Continued work should gradually lead to better understanding of the role of miRNAs in this field. Ultimately, we expect this understanding will have substantial benefits for fertility management at both the in vivo or/and in vitro levels. The stable nature of miRNA holds remarkable promise in clinical use as a diagnostic tool and in reproductive medicine to solve the ever-increasing fertility problem. In this review, we summarize current knowledge of the involvement of miRNAs in follicular atresia, discuss the challenges for further work and pinpoint areas for future research. PMID:28208755
Ramírez Marroquín, E S; Santibáñez Salgado, J A; Calderón Colmenero, J; Molina Méndez, J; Herrera Alarcón, V; Santibáñez Escobar, F; Barragán García, R
Bidirectional cavopulmonary shunt is an alternative palliative procedure for patients with congenital cyanotic heart disease, specially those patients less than "ideal" candidates for a Fontan's procedure. We present our results with this shunt in patients with tricuspid atresia. Twenty patients with tricuspid atresia were operated on with this shunt, with these associated defects: 20 atrial septal defect, 17 ventricular septal defect, 10 pulmonary stenosis, 1 pulmonary atresia and 1 transposition of the great arteries. Sex: 10 males and 10 females; the age was 27 days to 6 years (mean 1.8 years), the weight was 3.2 kg to 24 kg (mean 10.7 kg), the mean pulmonary artery pressure was 11 to 24 mmHg (mean 17 mmHg), pulmonary vascular resistance was 1.5 to 5 UW (mean 3.1 UW). Postoperative oxygen saturation improved 15 to 120%. All patients survived the surgical procedure. Three patients died in the immediate postoperative period, 2 due to a complications in the postoperative period and 1 due to sepsis. There were two late deaths, 1 sudden death after 6 months of the shunt, and 1 due to sepsis after a Fontan's procedure. Four patients presented pleural effusion and 2 pericardial effusion, they resolved well. We have 15 patients alive and well, in functional class I, and minimal cyanosis. We can conclude that this surgical procedure is useful in the management of patients with tricuspid atresia.
A 6-year-old French spaniel and a 14-month-old German shepherd dog were diagnosed with ear canal atresia. Based on presentation, computed tomography, and auditory function evaluation, the first dog underwent excision of the horizontal ear canal and bulla curettage, and the second underwent re-anastomosis of the vertical canal to the external meatus. Both dogs had successful outcomes.
Mun, Da-Na; Park, Chun Soo; Kim, Young-Hwue; Goo, Hyun Woo
A multistage plan and multidisciplinary approach are the keys to successful repair in patients with pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCAs). In this article, we present a multidisciplinary approach adopted to treat a patient with PA with VSD and MAPCAs associated with left pulmonary artery interruption. PMID:27733998
The presentation of extra hepatic biliary-atresia (EHBA) as well as choledochal cyst (CDC) in the neonate may be similar. Since the surgical management and prognosis are entirely different, it is important to differentiate between the two entities. We present a case with co-existing EHBA and CDC which led to a diagnostic dilemma. PMID:26023431
Rentea, Rebecca M; St Peter, Shawn D
Esophageal perforation (EP) is a rare complication that is often iatrogenic in origin. In contrast with adult patients in whom surgical closure of the defect is preferred, nonoperative treatment has become a common therapeutic approach for EP in neonates and children. Principles of management pediatric EP includes rapid diagnosis, appropriate hemodynamic monitoring and support, antibiotic therapy, total parenteral nutrition, control of extraluminal contamination, and restoration of luminal integrity either through time or operative approaches. Copyright © 2017 Elsevier Inc. All rights reserved.
Achem, Sami R; Gerson, Lauren B
Distal esophageal spasm (DES) is an esophageal motility disorder that presents clinically with chest pain and/or dysphagia and is defined manometrically as simultaneous contractions in the distal (smooth muscle) esophagus in ≥20% of wet swallows (and amplitude contraction of ≥30 mmHg) alternating with normal peristalsis. With the introduction of high resolution esophageal pressure topography (EPT) in 2000, the definition of DES was modified. The Chicago classification proposed that the defining criteria for DES using EPT should be the presence of at least two premature contractions (distal latency<4.5 s) in a context of normal EGJ relaxation. The etiology of DES remains insufficiently understood, but evidence links nitric oxide (NO) deficiency as a culprit resulting in a disordered neural inhibition. GERD frequently coexists in DES, and its role in the pathogenesis of symptoms needs further evaluation. There is some evidence from small series that DES can progress to achalasia. Treatment remains challenging due in part to lack of randomized placebo-controlled trials. Current treatment agents include nitrates (both short and long acting), calcium-channel blockers, anticholinergic agents, 5-phosphodiesterase inhibitors, visceral analgesics (tricyclic agents or SSRI), and esophageal dilation. Acid suppression therapy is frequently used, but clinical outcome trials to support this approach are not available. Injection of botulinum toxin in the distal esophagus may be effective, but further data regarding the development of post-injection gastroesophageal reflux need to be assessed. Heller myotomy combined with fundoplication remains an alternative for the rare refractory patient. Preliminary studies suggest that the newly developed endoscopic technique of per oral endoscopic myotomy (POEM) may also be an alternative treatment modality.
Recently published data have shown that an extended lymphadenectomy during the en bloc esophagectomy leads to a significant increased long-term survival for esophageal adenocarcinoma. On the other hand some studies indicate that the increased survival is based on stage migration and that the surgical complication rate is increased after extended lymphadenectomy. The aim of this review was to give an overview about all aspects of an extended lymphadenectomy in patients with esophageal adenocarcinoma. The review of the literature shows clearly that the number of involved lymph nodes is an independent prognostic factor in patients with esophageal adenocarcinoma. Furthermore, an extended lymphadenectomy leads to an increased long-term survival. Some studies describe that 23 lymph nodes should be removed to predict survival; other studies 18 lymph nodes or 15 lymph nodes. Opponents indicate that the survival benefit is based on stage migration. The studies with a large study population have performed a Cox regression analyzes and identified the number of lymph nodes removed as an independent factor for improved survival, which means it is significant independently from other parameters. Under these circumstances is stage migration not an option to explain the survival benefit. An important difficulty is, that there is no standardized definition of an extended lymphadenectomy, which means the localization and number of removed lymph nodes differ depending from the performing centre. The controversies regarding the survival benefit of the lymphadenectomy is based on the lack of standardisation of the lymphadenectomy. The main goal of further studies should be to generate a clear definition of an extended lymphadenectomy in patients with esophageal adenocarcinoma.
Glessner, Joseph; Ashokkumar, Chethan; Ranganathan, Sarangarajan; Min, Jun; Higgs, Brandon W.; Sun, Qing; Haberman, Kimberly; Schmitt, Lori; Vilarinho, Silvia; Mistry, Pramod K.; Vockley, Gerard; Dhawan, Anil; Gittes, George K.; Hakonarson, Hakon; Jaffe, Ronald; Subramaniam, Shankar; Shin, Donghun; Sindhi, Rakesh
Background & Aims Altered extrahepatic bile ducts, gut, and cardiovascular anomalies constitute the variable phenotype of biliary atresia (BA). Methods To identify potential susceptibility loci, Caucasian children, normal (controls) and with BA (cases) at two US centers were compared at >550000 SNP loci. Systems biology analysis was carried out on the data. In order to validate a key gene identified in the analysis, biliary morphogenesis was evaluated in 2-5-day post-fertilization zebrafish embryos after morpholino-antisense oligonucleotide knockdown of the candidate gene ADP ribosylation factor-6 (ARF6, Mo-arf6). Results Among 39 and 24 cases at centers 1 and 2, respectively, and 1907 controls, which clustered together on principal component analysis, the SNPs rs3126184 and rs10140366 in a 3’ flanking enhancer region for ARF6 demonstrated higher minor allele frequencies (MAF) in each cohort, and 63 combined cases, compared with controls (0.286 vs. 0.131, P = 5.94x10-7, OR 2.66; 0.286 vs. 0.13, P = 5.57x10-7, OR 2.66). Significance was enhanced in 77 total cases, which included 14 additional BA genotyped at rs3126184 only (p = 1.58x10-2, OR = 2.66). Pathway analysis of the 1000 top-ranked SNPs in CHP cases revealed enrichment of genes for EGF regulators (p<1 x10-7), ERK/MAPK and CREB canonical pathways (p<1 x10-34), and functional networks for cellular development and proliferation (p<1 x10-45), further supporting the role of EGFR-ARF6 signaling in BA. In zebrafish embryos, Mo-arf6 injection resulted in a sparse intrahepatic biliary network, several biliary epithelial cell defects, and poor bile excretion to the gall bladder compared with uninjected embryos. Biliary defects were reproduced with the EGFR-blocker AG1478 alone or with Mo-arf6 at lower doses of each agent and rescued with arf6 mRNA. Conclusions The BA-associated SNPs identify a chromosome 14q21.3 susceptibility locus encompassing the ARF6 gene. arf6 knockdown in zebrafish implicates early biliary
Hoopmann, M; Kagan, K O; Borgmeier, F; Seitz, G; Arand, J; Wagner, P
Background: The specific recognition of oesophageal atresia (OA) with or without a tracheal fistula in a foetus is a diagnostic challenge for prenatal medicine. The aim of the present work is to analyse the value of the measurement of gastric size in the diagnosis of this significant malformation. Materials and Methods: Altogether, the examinations of 433 pregnancies between the 18.4 and 39.1 weeks of gestation were retrospectively analysed. 59 of these foetuses exhibited an OA. By means of a linear regression analysis with normal foetuses, significant parameters influencing gastric size were examined. Subsequently the gastric sizes were transformed into z values and a comparison was made between OA with and without fistulae with the help of t tests. Results: In the normal foetuses there was a significant association between the gastric circumference and the abdominal circumference (circumference = 6.809 + 0.179 × abdominal circumference, r = 0.686, p < 0.0001). In the normal group the average was 43.0 (standard deviation [SD] 13.7) mm and those in foetuses with and without fistuale were 33.8 (SD 22.7) and 0.9 (SD 3.7) mm. In 34 (57.6 %) foetuses with an OA, the gastric circumference was below the 5th percentile. In detail, there were 13 (34.2 %) foetuses with a fistula and 21 (100 %) without a fistula. The average z values in the normal group and in the groups of OA with fistula and without fistula amounted to 0.0 (SD 1.0), -1.3 (SD 2.2) and -4.5 (SD 1.0). Conclusion: Measurements of the gastric circumference below the 5th percentile should lead to further diagnostic measures, especially when associated with polyhydramnios. Although OA without a fistula is always conspicuous, only about one in three OAs with fistula are associated with a significantly smaller stomach.
Hoopmann, M.; Kagan, K. O.; Borgmeier, F.; Seitz, G.; Arand, J.; Wagner, P.
Background: The specific recognition of oesophageal atresia (OA) with or without a tracheal fistula in a foetus is a diagnostic challenge for prenatal medicine. The aim of the present work is to analyse the value of the measurement of gastric size in the diagnosis of this significant malformation. Materials and Methods: Altogether, the examinations of 433 pregnancies between the 18.4 and 39.1 weeks of gestation were retrospectively analysed. 59 of these foetuses exhibited an OA. By means of a linear regression analysis with normal foetuses, significant parameters influencing gastric size were examined. Subsequently the gastric sizes were transformed into z values and a comparison was made between OA with and without fistulae with the help of t tests. Results: In the normal foetuses there was a significant association between the gastric circumference and the abdominal circumference (circumference = 6.809 + 0.179 × abdominal circumference, r = 0.686, p < 0.0001). In the normal group the average was 43.0 (standard deviation [SD] 13.7) mm and those in foetuses with and without fistuale were 33.8 (SD 22.7) and 0.9 (SD 3.7) mm. In 34 (57.6 %) foetuses with an OA, the gastric circumference was below the 5th percentile. In detail, there were 13 (34.2 %) foetuses with a fistula and 21 (100 %) without a fistula. The average z values in the normal group and in the groups of OA with fistula and without fistula amounted to 0.0 (SD 1.0), −1.3 (SD 2.2) and −4.5 (SD 1.0). Conclusion: Measurements of the gastric circumference below the 5th percentile should lead to further diagnostic measures, especially when associated with polyhydramnios. Although OA without a fistula is always conspicuous, only about one in three OAs with fistula are associated with a significantly smaller stomach. PMID:26719598
Greenhawt, Matthew; Aceves, Seema S; Spergel, Jonathan M; Rothenberg, Marc E
Eosinophilic esophagitis (EoE) is a clinicopathologic, chronic esophageal inflammatory disease resistant to acid suppressive therapy and is associated with variable symptoms indicative of upper gastrointestinal dysfunction. Per current guidelines established by The International Group of Eosinophil Researchers (TIGERS), the diagnosis is made in symptomatic patients after a biopsy that confirms a peak eosinophil level of ≥15 eosinophils/high-powered field (HPF). The esophagus is distinguished by pronounced tissue eosinophilia in which dietary antigens are key inciting factors for disease pathogenesis; EoE being reversed by elimination of triggering food allergens suggests that the disease is mediated in part by allergic sensitization to foods. Moreover, experimental EoE in mice can be induced not only via food exposure but also via aeroallergen exposure. Consistent with an allergic etiology rather than an acid-induced esophagitis, swallowed glucocorticoids are effective for the treatment of EoE. Evaluation by an allergist is a recommended part of the diagnostic workup, especially for management of allergic comorbidities. Clinical practice for the evaluation of patients with EoE mainly relies on prick skin tests due to the ease and validation of these tests in the context of immediate hypersensitivity. However, both atopy patch testing and serum IgE testing have been used in EoE. Herein, we reviewed the basic clinical features of EoE with a focus on the approach to diagnosing causative food allergens and to dietary therapy.
Yeh, S.H.; Wang, S.J.; Wu, L.C.; Liu, R.S.; Tsai, Y.T.; Chiang, T.T.
This study assessed esophageal motility in patients with esophageal varices by radionuclide transit studies. Data were acquired in list mode after an oral dose of 0.5 mCi Tc-99m sulfur colloid in 10 ml of water in the supine position above a low-energy all-purpose collimator of a gamma camera. The condensed image (CI) superimposed with a centroid curve was also produced in each case. Twenty-five normal subjects (N) and 32 patients (pts) with esophageal varices by endoscopy (large varices in Grades IV and V in 8 and small varices in Grade III or less in 24) were studied. TMTT, RTT, RF, and RI were all significantly increased in pts as compared to N. Especially, the transit time for the middle third (6.7 +- 2.6 sec vs 3.5 +- 0.9 sec in N, rho < 0.005) had the optimal sensitivy and specificity of 88% each at the cutoff value of 4.2 sec as determined by ROC analysis. In summary, radionuclide transit disorders occur in the majority of pts with esopageal varices. The middle RTT and CI are both optimal in sensitivity and specificity for detecting the abnormalities.
Cairo, Sarah; Kakembo, Nasser; Kisa, Phyllis; Muzira, Arlene; Cheung, Maija; Healy, James; Ozgediz, Doruk; Sekabira, John
Intestinal atresia is one of the leading causes of neonatal intestinal obstruction (NIO). The purpose of this study was to analyze the presentation and outcome of IA and compare with those from both similar and high-income country settings. A retrospective review of prospectively collected data from patient charts and pediatric surgical database for 2012-2015 was performed. Epidemiological data and patient characteristics were analyzed and outcomes were compared with those reported in other LMICs and high-income countries (HICs). Unmet need was calculated along with economic valuation or economic burden of surgical disease. Of 98 patients, 42.9% were male. 35 patients had duodenal atresia (DA), 60 had jejunio-ileal atresia (JIA), and 3 had colonic atresia. The mean age at presentation was 7.14 days for DA and 6.7 days for JIA. Average weight for DA and JIA was 2.2 and 2.12 kg, respectively. All patients with DA and colonic atresia underwent surgery, and 88.3% of patients with JIA had surgery. Overall mortality was 43% with the majority of deaths attributable to aspiration, anastomotic leak, and sepsis. 3304 DALYs were calculated as met compared to 25,577 DALYs' unmet. Patients with IA in Uganda present late in the clinical course with high morbidity and mortality attributable to a combination of late presentation, poor nutrition status, surgical complications, and likely underreporting of associated anomalies rather than surgical morbidity alone. Level IV, Case series with no comparison group.
Zhao, Shouqin; Han, Demin; Wang, Zhenchang; Li, Jie; Qian, Yanni; Ren, Yuanyuan; Dong, Jiyong
We conducted a prospective study to investigate the abnormalities of the facial nerve canal in patients with congenital aural atresia by computed tomography (CT). Our study population was made up of 99 patients--68 males and 31 females, aged 6 to 22 years (mean: 13.5)--who had unilateral congenital aural atresia without any inner ear malformations. We compared our findings in these patients with those in 50 controls-33 males and 17 females, aged 5 to 22 years (mean: 15.0)-who had normal ears. We classified the congenital aural atresia patients into three groups (A, B, and C) according to their Jahrsdoerfer grading scale score (≥8; 6 or 7; and ≤5, respectively). The course of the facial nerve canal in both the controls and the study patients was determined by temporal bone CT with multiplanar reconstruction. The distances from different parts of the facial nerve canal to surrounding structures were also measured. The course of the facial nerve canal in the normal ears did not vary much, and there were no statistically significant differences according to head side and sex. In groups B and C, the tympanic segment, mastoid segment, and angle of the second genu of the facial nerve canal were all significantly smaller than those of the controls (p < 0.01 in all cases). Statistically, the tympanic segment of the facial nerve canal in patients with congenital aural atresia was downwardly displaced. The mastoid segment of the facial nerve canal in these patients was more anterior than that of the controls. We conclude that congenital aural atresia is often accompanied by abnormalities of the facial nerve canal, especially in the tympanic segment, the mastoid segment, and the second genu. We found that the lower the Jahrsdoerfer score was, the shorter the tympanic segment was and the more forward the mastoid segment was.
Billings, Kathleen R; Qureshi, Hannan; Gouveia, Christopher; Ittner, Colleen; Hoff, Stephen R
To identify the rate of hearing loss related to middle ear disease and the frequency of tympanostomy tube (TT) insertion in the contralateral ear of patients with unilateral microtia/aural atresia. Retrospective case series of patients less than 3 years of age with unilateral microtia/aural atresia treated at an urban, tertiary care children's hospital from 2008 to 2013. Clinical and audiologic data were reviewed. Statistical analysis was performed to determine the relative risk of TT insertion in the normal ear. A total of 72 patients were included for analysis. The average age of patients at their initial otolaryngology visit was 3.3 months (range 0.08-1.67 years); 38 (52.8%) patients were males. Aural atresia involved the right ear in 43 (59.7%) cases. Five (6.9%) patients were syndromic. Abnormal audiometric testing of the normal ear was noted in 12 (16.7%), and 14 (19.4%) underwent TT during the first 3 years of life. Twelve children (85.7%) who had a TT placed were nonsyndromic. When compared to published norms for TT placement in the general population (6.8% of children < 3 year of age), a greater proportion of children with unilateral microtia/aural atresia had TT placement in the normal ear (z = 4.26, P < 0.0001). Patients with unilateral microtia/aural atresia have increased rates of hearing loss and middle ear effusion leading to TT in their normal ear at a higher rate versus the general population. This information can help guide more vigilant care and audiologic follow-up in affected children. 4. Laryngoscope, 126:1470-1474, 2016. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.
Jacobs, John William
Background Eosinophilic esophagitis (EoE) is associated with tissue remodeling that can result in esophageal mucosal fragility, and esophageal dilation for patients with EoE is known to cause painful mucosal lacerations. Clinicians have been admonished that patients with EoE may be exceptionally predisposed to perforation with esophageal dilation, a notion supported primarily by case reports. We have conducted a systematic review of literature on esophageal dilation in EoE in an attempt to better define the risk of perforation. Methods We searched PubMed and abstracts presented at the annual scientific meetings of the American Gastroenterological Association and the American College of Gastroenterology to identify reports on esophageal dilation in EoE. We analyzed reports meeting the following criteria: (1) the diagnosis was established from esophageal biopsy specimens revealing ≥15 eosinophils/hpf, (2) esophageal dilation was described, (3) esophageal perforations described were the result of esophageal dilation. Results We identified 18 reports for inclusion in our systematic review. The studies comprised 468 patients who underwent a total of 671 endoscopic dilations. Esophageal mucosal tears were described in most cases, but there was only one perforation among the 671 dilations (0.1%). Conclusions Our systematic review does not reveal an inordinate frequency of esophageal perforation from dilation in patients with EoE, and it is not clear that dilation is any more hazardous for patients with EoE than for patients with other causes of esophageal stricture. Although esophageal dilation must be performed with caution in all patients, the risk of perforation in EoE appears to have been exaggerated. PMID:20238250
... 21 Food and Drugs 8 2014-04-01 2014-04-01 false Esophageal prosthesis. 878.3610 Section 878.3610 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3610 Esophageal...
... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Esophageal prosthesis. 878.3610 Section 878.3610 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3610 Esophageal...
... 21 Food and Drugs 8 2013-04-01 2013-04-01 false Esophageal prosthesis. 878.3610 Section 878.3610 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3610 Esophageal...
... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Esophageal prosthesis. 878.3610 Section 878.3610 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3610 Esophageal...
... 21 Food and Drugs 8 2012-04-01 2012-04-01 false Esophageal prosthesis. 878.3610 Section 878.3610 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3610 Esophageal...
Ravi, Karthik; Katzka, David A
The development of intraluminal esophageal impedance monitoring has improved our ability to detect and measure gastroesophageal reflux without dependence on acid content. This ability to detect previously unrecognized weak or nonacid reflux episodes has had important clinical implications in the diagnosis and management of gastroesophageal reflux disease (GERD). In addition, with the ability to assess bolus transit within the esophageal lumen, impedance monitoring has enhanced the recognition and characterization of esophageal motility disorders in patients with nonobstructive dysphagia. The assessment of the intraluminal movement of gas and liquid has also been proven to be of diagnostic value in conditions such as rumination syndrome and excessive belching. Further, alternative applications of impedance monitoring, such as the measurement of mucosal impedance, have provided novel insights into assessing esophageal mucosal integrity changes as a consequence of inflammatory change. Future applications for esophageal impedance monitoring also hold promise in esophageal conditions other than GERD. However, despite all of the clinical benefits afforded by esophageal impedance monitoring, important clinical and technical shortcomings limit its diagnostic value and must be considered when interpreting study results. Overinterpretation of studies or application of impedance monitoring in patients can have deleterious clinical implications. This review will highlight the clinical benefits and limitations of esophageal impedance monitoring and provide clinical pearls and pitfalls associated with this technology.
Okeson, Danelle M; Esterline, Meredith L; Coke, Rob L
Four adult, wild caught Parma wallabies (Macropus parma) presented with intermittent, postprandial, midcervical swellings. Esophageal diverticula were discovered in the four animals. One of two wallabies was managed successfully with surgery. A third animal died of other causes. The fourth animal died with possible complications from the diverticulum. This is the first published report of esophageal diverticula in macropods.
Caygill, Christine P J; Gatenby, Piers A C; Herceg, Zdenko; Lima, Sheila C S; Pinto, Luis F R; Watson, Anthony; Wu, Ming-Shiang
The following, from the 12th OESO World Conference: Cancers of the Esophagus, includes commentaries on comparative genomic analysis of esophageal cancers: genomic polymorphisms, the genetic and epigenetic drivers in esophageal cancers, and the collection of data in the UK Barrett's Oesophagus Registry.
... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Esophageal dilator. 876.5365 Section 876.5365 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GASTROENTEROLOGY-UROLOGY DEVICES Therapeutic Devices § 876.5365 Esophageal dilator. (a...
... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Esophageal dilator. 876.5365 Section 876.5365 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GASTROENTEROLOGY-UROLOGY DEVICES Therapeutic Devices § 876.5365 Esophageal dilator. (a...
Adenocarcinoma of the Esophagus; Adenocarcinoma of the Gastroesophageal Junction; Recurrent Esophageal Cancer; Squamous Cell Carcinoma of the Esophagus; Stage III Esophageal Cancer; Stage IV Esophageal Cancer
Burgos, L; Martínez, L; Suárez, O; Andrés, A M; Luis, A L; Encinas, J L; Hernández, F; Murcia, J; Olivares, P; Queizán, A; Lassaletta, L; Tovar, J A
Esophagocoloplasty is one of the most used procedures for esophageal replacement in children. Considering high life expectancy in these patients, long-term results must be considered when evaluating this technique. The aim of our study is to evaluate quality of life of adult patients who underwent surgery at pediatric age. We report a retrospective study of 99 patients who underwent esophageal replacement in our institution between 1966 and 2006. Eight of them have died and 63 out of the remaining 91 are over 18 years now and represent our study serie. Long-term results and actual situation of those patients, considering psychological, physic and social aspects, were evaluated through clinical review and telephonic interview. Karnofsky index was applied to mesure functional ability from 0-100% (bad, medium, good-excellent) according to the answers the patients gave to our questions. We also recorded their health personal experience and subjective evaluation of their quality of life. Sixty-three patients were reviewed (43 males and 20 females) with a mean age of 4.3 +/- 3.4 D.S. Mean follow-up time was 29.6 +/-7.7 years. Indications for esophageal replacement were as follows: caustication (n = 32), type III esophageal atresia (n = 15), type I AE (n = 13) and others (n = 3). In 48 patients the graft was placed in retroestenal position and in 15 cases retromediastic location was used. Postoperative period was uneventful in 44% of the patients, being the most frequent early complications in the remaining, cervical leakage and stenosis. Long-term, 56,8% did not have any sequelae, 28.5% required further surgery and the remaining 43.13% presented the following complications: symptomatic graft reflux (22), scoliosis and thoracic asymmetry (12), colonic redundancy or cervical diverticulum (7), food impaction (6) and failure to thrive (5). Only one 38 year old patient does not have intestinal tract continuity nowadays. Thirty-one patients have a Karnofsky index > or = 80
Yang, Liying; Chaudhary, Noami; Baghdadi, Jonathan; Pei, Zhiheng
The incidence of esophageal adenocarcinoma has increased dramatically in the United States and Europe since the 1970s without apparent cause. Although specific host factors can affect risk of disease, such a rapid increase in incidence must be predominantly environmental. In the stomach, infection with Helicobacter pylori has been linked to chronic atrophic gastritis, an inflammatory precursor of gastric adenocarcinoma. However, the role of H. pylori in the development of esophageal adenocarcinoma is not well established. Meanwhile, several studies have established that a complex microbiome in the distal esophagus might play a more direct role. Transformation of the microbiome in precursor states to esophageal adenocarcinoma-reflux esophagitis and Barrett metaplasia-from a predominance of gram-positive bacteria to mostly gram-negative bacteria raises the possibility that dysbiosis is contributing to pathogenesis. However, knowledge of the microbiome in esophageal adenocarcinoma itself is lacking. Microbiome studies open a new avenue to the understanding of the etiology and pathogenesis of reflux disorders.
Winship, Daniel H.; Zboralske, F. Frank
The response of the normal human esophagus to an obstructing intraluminal bolus was investigated and compared to the response evoked by transient intraluminal distention. A balloon, immobilized within the esophagus by external attachment to a force transducer, was inflated with from 3 to 25 ml of air for from 3 to 210 sec. Pressure phenomena occurring in the esophagus were simultaneously recorded from the body of the esophagus above and below the balloon. Transient distention (5 sec or less) with small volumes (5 ml or less) often evoked a secondary peristaltic wave in the esophagus distal to the balloon, but infrequently resulted in the registration of any force exerted upon the balloon to drive it downward. Conversely, distentions of longer duration and with greater volume elicited an esophageal propulsive force exerted upon the balloon oriented to propel it aborally, and much less often evoked a propagated wave of secondary peristalsis. The propulsive force, obviously resulting from esophageal muscular contraction, occurred promptly, and once initiated, was sustained until deflation of the balloon. It varied widely in magnitude, from 4 to 200 g, and was associated with no motor phenomena recorded from the body of the esophagus proximal or distal to the balloon which could account for its presence, onset, magnitude, or duration. The force was inhibited by deglutition, but arrival of the primary peristaltic wave at the bolus resulted in augmentation of the force. When the obstructing balloon was freed from its attachment, the persistent, stationary force was converted to a propagated one that propelled the balloon before it. It the balloon was arrested before entering the stomach, the moving contraction was also arrested and the persistent propulsive force acting upon the balloon was maintained. The velocity of the moving contraction wave was determined in great part by the resistance offered by the bolus. Unrestrained, the balloon was propelled aborally at 4-8 cm
Meneghetti, Adam T; Tedesco, Pietro; Damani, Tanuja; Patti, Marco G
This study determines the relationship among esophageal dysmotility, esophageal acid exposure, and esophageal mucosal injury in patients with gastroesophageal reflux disease (GERD). A total of 827 patients with GERD (confirmed by ambulatory pH monitoring) were divided into three groups based on the degree of mucosal injury: group A, no esophagitis, 493 patients; group B, esophagitis grades I to III, 273 patients; and group C, Barrett's esophagus, 61 patients. As mucosal damage progressed from no esophagitis to Barrett's esophagus, there was a significant decrease in lower esophageal sphincter pressure and amplitude of peristalsis in the distal esophagus, with a subsequent increase in the number of reflux episodes in 24 hours, the number of reflux episodes longer than 5 minutes, and the reflux score. These data suggest that in patients with GERD, worsening of esophageal mucosal injury may determine progressive deterioration of esophageal motor function with impairment of acid clearance and increase of esophageal acid exposure. These findings suggest that Barrett's esophagus is an end-stage form of gastroesophageal reflux, and that if surgical therapy is performed early in the course of the disease, this cascade of events might be blocked.
Keckler, Scott J; St Peter, Shawn D; Spilde, Troy L; Ostlie, Daniel J; Snyder, Charles L
Duodenal atresia is associated with a wide variety of congenital malformations. Trisomy 21 occurs in approximately one-thirds of infants with duodenal atresia. Congenital heart disease in patients with trisomy 21 and duodenal atresia is well known. However, the frequency and spectrum of congenital heart defects in infants with duodenal atresia and a normal karyotype has not been outlined in the literature. Therefore, we conducted a retrospective chart review to clarify our knowledge about this population. Retrospective review of the medical record was performed on patients with duodenal atresia/stenosis from January 1995 to September 2007. Demographic data included birth weight and gestational age. Variables of interest included cardiac defects and karyotype. Surgical repair for duodenal and cardiac malformations were reviewed. Ninety-four patients with duodenal atresia/stenosis were identified. Average gestational age was 36 weeks and birth weight was 2,536 g. Trisomy 21 was identified in 39 (41%) patients. Overall, 37 patients (39.3%) had a congenital heart defect. Defects were identified in 24 (61.5%) patients with trisomy 21, when compared to 13 (23.6%) patients with a normal karyotype. Of the patients with congenital heart defects and trisomy 21, 11 (28.2%) required operative repair compared to the 6 (10.9%) patients with a defect and normal karyotype. Therefore, in patients with duodenal atresia, the presence of trisomy 21 carries a relative risk of 2.61 for congenital heart defects, and relative risk of 2.59 for open heart surgery. In patients with duodenal atresia, the presence of trisomy 21 carries a 2.5-fold increased risk of cardiac defect and the same increased risk for repairing a cardiac defect.
Lorenz, Julie; Vollenweider, Peter; Vuilleumier, Henri; Schwab, Marcos
Esophageal intramural pseudodiverticulosis is a rare pathology whose etiology is unknown, but which is frequently associated with three highly prevalent entities: esophageal reflux disease, esophageal candidosis and alcoholic esophagitis. With conservative treatment the course of these pathologies is usually benign. However, some severe cases are resistant to conservative treatment and may require more aggressive management. We here present the case of patient suffering from a severe esophagitis complicated by chronic mediastinitis with life-threatening repercussions, requiring esophagectomy as treatment.
Yeh, Chu-Yin; Chung-Davidson, Yu-Wen; Wang, Huiyong; Li, Ke; Li, Weiming
Bile salt synthesis is a specialized liver function in vertebrates. Bile salts play diverse roles in digestion and signaling, and their homeostasis is maintained by controlling input (biosynthesis) and intestinal conservation. Patients with biliary atresia (i.e., obliteration of the biliary tree) suffer liver fibrosis and cirrhosis. In contrast, sea lamprey thrives despite developmental biliary atresia. We discovered that the sea lamprey adapts to biliary atresia through a unique mechanism of de novo synthesis and secretion of bile salts in intestine after developmental biliary atresia, in addition to known mechanisms, such as the reduction of bile salt synthesis in liver. During and after developmental biliary atresia, expression of cyp7a1 in intestine increased by more than 100-fold (P < 0.001), whereas in liver it decreased by the same magnitude (P < 0.001). Concurrently, bile salt pools changed in similar patterns and magnitudes in these two organs and the composition shifted from C24 bile alcohol sulfates to taurine-conjugated C24 bile acids. In addition, both in vivo and ex vivo experiments showed that aductular sea lamprey secreted taurocholic acid into its intestinal lumen. Our results indicate that the sea lamprey, a jawless vertebrate, may be in an evolutionarily transitional state where bile salt synthesis occurs in both liver and intestine. Understanding the molecular basis of these mechanisms may shed light on the evolution of bile salt synthesis and possible therapy for infant biliary atresia. PMID:22733776
Schoepfer, Alain; Safroneeva, Ekaterina
The activity of eosinophilic esophagitis (EoE) can be assessed with patient-reported outcomes and biologic measures. Patient-reported outcomes include symptoms and quality of life, whereas biologic measures refer to endoscopic, histologic, and biochemical activity (e.g. blood biomarkers). So far, a validated tool to assess EoE activity in the above-mentioned dimensions is lacking. Given the lack of a standardized way to assess EoE activity in the various dimensions, the results of different clinical trials may be difficult to compare. For symptom assessment in adult patients, the symptom 'dysphagia' should be evaluated according to different standardized food consistencies. Furthermore, symptom assessment should take into account the following items: avoidance of specific food categories, food modification, and time to eat a regular meal. A distinct symptom recall period (e.g. 2 weeks) has to be defined for symptom assessment. Performing an 'esophageal stress test' with ingestion of a standardized meal to measure symptom severity bears the potential risk of acute food bolus impaction and should therefore be avoided. The description of endoscopic findings in EoE has meanwhile been standardized. Histologic evaluation of EoE activity should report either the size of the high-power field used or count the eosinophils per mm(2). There is a current lack of blood biomarkers demonstrating a good correlation with histologic activity in esophageal biopsies. The development and validation of an adult and pediatric EoE activity index is urgently needed not only for clinical trials and observational studies, but also for daily practice.
Poza Cordon, Joaquin; Froilan Torres, Consuelo; Burgos García, Aurora; Gea Rodriguez, Francisco; Suárez de Parga, Jose Manuel
The rupture of gastric varices results in variceal hemorrhage, which is one the most lethal complications of cirrhosis. Endoscopic therapies for varices aim to reduce variceal wall tension by obliteration of the varix. The two principal methods available for esophageal varices are endoscopic sclerotherapy (EST) and band ligation (EBL). The advantages of EST are that it is cheap and easy to use, and the injection catheter fits through the working channel of a diagnostic gastroscope. Endoscopic variceal ligation obliterates varices by causing mechanical strangulation with rubber bands. The following review aims to describe the utility of EBL and EST in different situations, such as acute bleeding, primary and secondary prophylaxis.
Watson, Thomas J
Endoscopic therapies have become the standard of care for most cases of Barrett's esophagus with high-grade dysplasia or intramucosal adenocarcinoma. Despite a rapid and dramatic evolution in treatment paradigms, esophagectomy continues to occupy a place in the therapeutic armamentarium for superficial esophageal neoplasia. The managing physician must remain cognizant of the limitations of endoscopic approaches and consider surgical resection when they are exceeded. Esophagectomy, performed at experienced centers for appropriately selected patients with early-stage disease can be undertaken with the expectation of cure as well as low mortality, acceptable morbidity, and good long-term quality of life. Copyright © 2017 Elsevier Inc. All rights reserved.
Min, Yang Won; Rhee, Poong-Lyul
Noncardiac chest pain (NCCP) is an often-encountered clinical problem. Although many patients suffer from persistent or recurrent chest pain, treatment remains a challenge owing to its various possible etiologies. Gastroesophageal reflux disease (GERD) is the most common cause of NCCP. In GERD-related NCCP, proton pump inhibitor treatment appears to be effective. However, the pathophysiology remains to be fully elucidated in NCCP patients without GERD. Treatment for non-GERD-related NCCP has been aimed at esophageal motility disorders and visceral hypersensitivity. As there is growing evidence that esophageal visceral hypersensitivity plays a role in NCCP, pain modulators have become the mainstay of therapy in patients with non-GERD-related NCCP. However, there is an unmet need for the treatment of esophageal hypersensitivity in NCCP due to modest evidence for the benefit of pain modulators, including antidepressants, in non-GERD-related NCCP. Recent studies have demonstrated that esophageal mast cell infiltration and impaired mucosal integrity are related to visceral hypersensitivity in patients with NCCP. Thus, esophageal mast cell stabilization and restoration of esophageal mucosal integrity could be considered potential therapeutic targets in selected NCCP patients with hypersensitivity. However, further observations are necessary to shed light on esophageal hypersensitivity in NCCP.
Loske, Gunnar; Schorsch, Tobias; Müller, Christian
Anastomotic insufficiency in esophageal anastomosis and esophageal defects of other etiology are very severe complications. For anastomotic insufficiency in the rectum, endoscopic vacuum therapy has already been used successfully. The authors used vacuum therapy for anastomotic defects and other lesions of the esophagus. Between November 2006 and September 2009, 10 patients (5 men and 5 women, ages 46-82 years) were treated with endoscopic vacuum sponge therapy for anastomotic insufficiency secondary to esophagectomy or gastrectomy (n = 5), iatrogenic esophageal perforation (n = 2), esophageal wall necrosis (n = 1), Boerhaave's syndrome (n = 1), and perforation of esophageal cancer (n = 1). After one to seven changes of the sponge at intervals of 2-7 days and a mean therapy duration of 12 days, the defects were healed in all the surviving patients. During treatment, the patients were fed via an intestinal tube or percutaneous endoscopic gastrostomy (PEG), or enterally past the sponge. One patient died of intercurrent severe colitis. In three cases, a revision laparotomy was necessary at the beginning of treatment. No postinterventional stricture or functional relevant scar formation was observed during a follow-up period of 10-380 days after termination of the vacuum therapy. Esophageal anastomotic insufficiency and esophageal wall defects of other causes can be treated successfully with endoscopic vacuum sponge therapy.
Martinucci, Irene; de Bortoli, Nicola; Giacchino, Maria; Bodini, Giorgia; Marabotto, Elisa; Marchi, Santino; Savarino, Vincenzo; Savarino, Edoardo
Esophageal motility abnormalities are among the main factors implicated in the pathogenesis of gastroesophageal reflux disease. The recent introduction in clinical and research practice of novel esophageal testing has markedly improved our understanding of the mechanisms contributing to the development of gastroesophageal reflux disease, allowing a better management of patients with this disorder. In this context, the present article intends to provide an overview of the current literature about esophageal motility dysfunctions in patients with gastroesophageal reflux disease. Esophageal manometry, by recording intraluminal pressure, represents the gold standard to diagnose esophageal motility abnormalities. In particular, using novel techniques, such as high resolution manometry with or without concurrent intraluminal impedance monitoring, transient lower esophageal sphincter (LES) relaxations, hypotensive LES, ineffective esophageal peristalsis and bolus transit abnormalities have been better defined and strongly implicated in gastroesophageal reflux disease development. Overall, recent findings suggest that esophageal motility abnormalities are increasingly prevalent with increasing severity of reflux disease, from non-erosive reflux disease to erosive reflux disease and Barrett’s esophagus. Characterizing esophageal dysmotility among different subgroups of patients with reflux disease may represent a fundamental approach to properly diagnose these patients and, thus, to set up the best therapeutic management. Currently, surgery represents the only reliable way to restore the esophagogastric junction integrity and to reduce transient LES relaxations that are considered to be the predominant mechanism by which gastric contents can enter the esophagus. On that ground, more in depth future studies assessing the pathogenetic role of dysmotility in patients with reflux disease are warranted. PMID:24868489
Nicodème, Frédéric; Hirano, Ikuo; Chen, Joan; Robinson, Kenika; Lin, Zhiyue; Xiao, Yinglian; Gonsalves, Nirmala; Kwasny, Mary J; Kahrilas, Peter J; Pandolfino, John E
The aim of this study was to assess whether measurements of esophageal distensibility, made by high-resolution impedance planimetry, correlated with important clinical outcomes in patients with eosinophilic esophagitis. Seventy patients with eosinophilic esophagitis (50 men; age, 18-68 y) underwent endoscopy with esophageal biopsy collection and high-resolution impedance planimetry using the functional lumen-imaging probe. The patients were followed up prospectively for an average of 9.2 months (range, 3-14 mo), and the risk of food impaction, requirement for dilation, and symptom severity during the follow-up period was determined from medical records. Esophageal distensibility metrics and the severity of mucosal eosinophilia at baseline were compared between patients presenting with and without food impaction and those requiring or not requiring esophageal dilation. Logistic regression and stratification assessments were used to assess the predictive value of esophageal distensibility metrics in assessing risk of food impaction, the need for dilation, and continued symptoms. Patients with prior food impactions had significantly lower distensibility plateau (DP) values than those with solid food dysphagia alone. In addition, patients sustaining food impaction and requiring esophageal dilation during the follow-up period had significantly lower DP values than those who did not. The severity of mucosal eosinophilia did not correlate with risk for food impaction, the requirement for dilation during follow-up evaluation, or DP values. Reduced esophageal distensibility predicts risk for food impaction and the requirement for esophageal dilation in patients with eosinophilic esophagitis. The severity of mucosal eosinophilia was not predictive of these outcomes and had a poor correlation with esophageal distensibility. Copyright © 2013 AGA Institute. Published by Elsevier Inc. All rights reserved.
Anwar, S.; Purohit, G.N.
A one-day old male camel calf was presented to the Al-Qattara Veterinary Hospital with complaints of abdominal straining and lack of defecation. On examination it was found that the calf had no tail, the posterior sacral margin was blunt and the anal opening was absent. The case was diagnosed as congenital anury with atresia ani. The animal was sedated with 0.1 mg/kg of xylazine administered intramuscularly and under local infiltration with 2% lidocaine a circular incision was made at the anal area to create an anal opening. The animal passed plenty of meconium. The cut edges were sutured with horizontal mattress sutures. The animal was administered penicillin and streptomycin for 5 days and had an uneventful recovery. It is reported that congenital anury rarely occurs in one humped camel and accompanied atresia ani can be surgically treated. PMID:26623295
Boudewyns, An; van den Ende, Jenneke; Boiy, Tine; Van de Heyning, Paul; Declau, Frank
22q11.2 microduplication syndrome is characterized by a large phenotypic variability including facial dysmorphism, developmental delay, and hearing loss. We describe a family in whom 5 of 11 children were affected by a unilateral or bilateral congenital aural atresia. Four of these 5 carried a 22q11.2 microduplication and had typical dysmorphic features. Computed tomography with 3-D reconstructions allowed for a detailed examination of the middle ear structures and classification of the atresia type. Audiometry revealed a moderately severe conductive hearing loss in accordance with the clinical and computed tomography findings. Detailed examination of the ear is warranted in patients with a 22q11.2 microduplication. When outer ear abnormalities are encountered, an additional workup including audiometry and computed tomography with 3-D reconstructions is required.
IJsselstijn, Hanneke; Gischler, Saskia J; Toussaint, Leontien; Spoel, Marjolein; Zijp, Monique H M van der Cammen-van; Tibboel, Dick
Survival rates in oesophageal atresia patients have reached over 90%. In long-term follow-up studies the focus has shifted from purely surgical or gastrointestinal evaluation to a multidisciplinary approach. We reviewed the literature on the long-term morbidity of these patients and discuss mainly issues of physical growth and neurodevelopment. We conclude that growth problems - both stunting and wasting - are frequently seen, but that sufficient longitudinal data are lacking. Therefore, it is unclear whether catch-up growth into adolescence and adulthood occurs. Data on determinants of growth retardation are also lacking in current literature. Studies on neurodevelopment beyond preschool age are scarce but oesophageal atresia patients seem at risk for academic problems and motor function delay. Many factors contribute to the susceptibility to growth and development problems and we propose a multidisciplinary follow-up schedule into adulthood future care which may help improve quality of life.
Jatene, Marcelo; Juaneda, Ignacio; Miranda, Rogerio Dos Anjos; Gato, Rafaella; Marcial, Miguel Lorenzo Barbero
A 9-year-old boy with congenital atresia of the left main coronary artery underwent myocardial revascularization. Coarctation of the aorta and ventricular septal defect were diagnosed at the age of 1 year. At age 7 years, the child presented with syncope while exercising. Preoperative evaluation included cardiac catheterization which revealed the unexpected finding of congenital atresia of the left main coronary artery with origin of the circumflex artery from the right coronary artery. Surgical correction included myocardial revascularization by means of left internal mammary artery graft to the anterior descending coronary artery, coarctation resection, and ventricular septal defect repair. The patient recovered uneventfully. We report the details of this extremely rare case with successful concomitant surgical management of the congenital coronary artery anomaly and the associated structural heart disease.
Sugimoto, Ai; Shiraishi, Shuichi; Moon, Jiyong; Takahashi, Masashi; Tsuchida, Masanori
Atresia of the left coronary artery ostium is extremely rare. We report the case of a 13-year-old boy who played volleyball in school and collapsed with severe chest pain during practice. He was referred to our hospital, and imaging modalities showed atresia of the left main coronary artery ostium. Urgent coronary ostioplasty was performed using a patch of 0.6% glutaraldehyde-treated autologous pericardium. His postoperative course was uneventful, and he has had a normal everyday life without chest pain 8 months postoperatively. Physicians should be aware of the patient's history, as in this case, because prompt imaging diagnosis is essential when there is a high likelihood that the event is related to myocardial ischemia. Since long-term outcome is uncertain even after successful surgical revascularization, close follow-up is required. © The Author(s) 2015.
Anwer, Cona; Schwarz, Tobias; Volk, Susan W; Vite, Charles
A 3 yr old male castrated Labrador retriever presented for evaluation and treatment of bilateral atresia of the external ear canals. The owners reported that the dog could hear only loud and high-pitched noises. Computed tomography of the head revealed intact vertical and horizontal ear canals filled with debris and a debris-filled right tympanic bulla. Air- and bone-conducted brainstem auditory evoked response (BAER) testing revealed an elevated response threshold to air-conducted stimuli and greater amplitude waveforms evoked by bone-conducted stimuli. The ear canals were surgically corrected via lateral ear canal resection. BAER testing postoperatively revealed a decrease in the air-conducted BAER threshold. This case is an example of the use of bone-conducted BAER testing to aid in the diagnosis of conductive deafness, and in determining prognosis for normal hearing after surgical treatment of external ear canal atresia.
Torrisi, J.M.; Haller, J.O.; Velcek, F.T. )
The radiologic findings in five neonates with choledochal cyst associated with extra-hepatic biliary atresia are described. All five patients (age range, 13-72 days) presented with jaundice and acholic stools. In all four patients who underwent sonographic examination, a cystic structure separate from the gallbladder representing the choledochal cyst was shown. The diagnosis of atresia of the distal common bile duct was made preoperatively in all cases by hepatobiliary scintigraphy. Diagnosis was confirmed by surgical findings and was demonstrated by intraoperative cholangiography in four cases. All patients were successfully treated with surgical intervention within 1 month from the time of diagnosis. Early detection of this rare disorder, which may be distinct from choledochal cyst found in children and adults, is important to prevent fatal complications of biliary obstruction. The combined use of sonography and hepatobiliary scintigraphy can correctly identify this subset of patients with persistent neonatal jaundice and provide valuable information for prompt surgical management.
Kocsis, Dorottya; Tulassay, Zsolt; Juhász, Márk
Eosinophilic esophagitis is considered to be a chronic antigen-driven disease whereby food and/or aeroallergens induce a chronic inflammatory infiltrate in the esophagus leading to pathological hyperplasia of the epithelial and muscular layers, fibrosis of the lamina propria and symptoms of dysphagia and food impaction. Eosinophilic esophagitis is often associated with other allergic diseases such as asthma or atopic dermatitis. Current first line treatments of the disease include strict dietary modification and topical anti-inflammatory steroids. In this review the authors summarize currently available treatment strategies of eosinophilic esophagitis.
Montoya, Manuel; Chumbiraico, Robert; Ricalde, Melvin; Cazorla, Ernesto; Hernández-Córdova, Gustavo
Esophageal involvement is an extremely rare complication of tuberculosis even in countries with high prevalence of infection. We report the case of a 57 year-old hiv-seronegative patient with simultaneous diagnoses of oral blastomycosis and laryngeal papillomatosis. Both were confirmed by anatomopathological analysis. The esophageal biopsy revealed granulomatous esophagitis with necrosis and ziehl-neelsen stain showed acid-fast alcohol resistant bacilli suggestive of tuberculosis. The patient's history included pulmonary tuberculosis twice and previous abandonment of therapy. Thus, it was necessary to use oral itraconazole combined with second-line anti-tuberculosis drugs administered through a gastrostomy tube. The clinical development was favorable.
Ahmad, Alsafadi; Wong Kee Song, Louis M.; Absah, Imad
Iatrogenic esophageal perforation (IEP) is a potentially serious adverse event of interventional endoscopy. The approach to IEP varies from surgical repair for large perforations to conservative treatment for small contained perforations. We report a case of an 18-month-old girl with congenital esophageal stenosis suffering a large esophageal perforation after a trial of stricture dilatation, which was successfully managed by the placement of fully covered stent. Hence, in selected cases, esophageal stent placement is a feasible alternative to invasive surgery in managing IEP. PMID:27144142
Song, Ji Hyun; Han, Young-Min; Kim, Won Hee; Park, Jong-Min; Jeong, Migyeong; Go, Eun Jin; Hong, Sung Pyo; Hahm, Ki Baik
The incidence of reflux esophagitis increases in world, affecting approximately 20% of Western populations and its consequent lesion, Barrett's esophagus (BE), established as the primary precursor lesion of esophageal adenocarcinoma (EAC) or Barrett associated adenocarcinoma (BAA), is also increasing in incidence in Asian countries as well as Western countries. The fact that surveillance strategies have not had a major benefit in decreasing the incidence of EAC increased attention to arrest or delay the progression of BE to EAC. Since sustained inflammation and consequent oxidative stress plays core pathogenic role in reflux esophagitis, BE, and BAA, attention paid to anti-inflammatory and antioxidative agents in the treatment of reflux esophagitis. Since the risk of esophagitis is associated with hiatal hernia, body mass index, and duodenogastric reflux, and acid exposure, lifestyle modification and agents to control gastric acidity might be mainstay for treatment, but several studies consistently showed the implication of robust oxidative stress in reflux associated esophageal diseases. In this review article, the pathogenic implication of oxidative stress will be introduced in the development of reflux esophagitis, BE, and EAC. Also, since there is great interest in complete healing of reflux esophagitis and chemoprevention to prevent or slow malignant transformation, the contribution of antioxidants or antioxidative agents, which was delivered during SFRR-Asia 2015 (Chiangmai, Thailand), will be described. Also, the molecular mechanisms how the antioxidative drugs, rebamipide, ecabet sodium, and pantoprazole exerted significant protection from acids or bile acids-associated esophagitis are included.
Ahmad, Alsafadi; Wong Kee Song, Louis M; Absah, Imad
Iatrogenic esophageal perforation (IEP) is a potentially serious adverse event of interventional endoscopy. The approach to IEP varies from surgical repair for large perforations to conservative treatment for small contained perforations. We report a case of an 18-month-old girl with congenital esophageal stenosis suffering a large esophageal perforation after a trial of stricture dilatation, which was successfully managed by the placement of fully covered stent. Hence, in selected cases, esophageal stent placement is a feasible alternative to invasive surgery in managing IEP.
Trabulo, Daniel; Ferreira, Sara; Lage, Pedro; Rego, Rafaela Lima; Teixeira, Gilda; Pereira, A Dias
We report a case of a 56-year-old woman with a history of allogenic bone marrow transplantation for two years, complaining with dysphagia and weight loss. Upper endoscopy revealed esophageal stenosis and extensive mucosa sloughing. Biopsies confirmed the diagnosis of graft-vs-host disease (GVHD). Balloon dilation, corticosteroids and cyclosporin resulted in marked clinical improvement. Gastrointestinal tract is involved in the majority of patients with chronic GVHD. Esophageal manifestations are rare and include vesiculobullous disease, ulceration, esophageal webs, casts or strictures. Sloughing esophagitis along with severe stenosis requiring endoscopic dilation has never been reported in this context. PMID:26290649
Pandya, Kartikey A; Koga, Hiroyuki; Okawada, Manabu; Coran, Arnold G; Yamataka, Atsuyuki; Teitelbaum, Daniel H
The association of vaginal atresia (or Mayer-Rokitansky-Kuster-Hauser Syndrome) with imperforate anus is rare and can present significant diagnostic and therapeutic challenges. This study describes clinical characteristics, surgical treatment and outcomes in this group of complex children. Records of 20 patients were retrospectively analyzed from two pediatric surgical centers. Five patients were excluded from the long-term analysis due to inadequate information, leaving long-term follow-up in 15 patients. Mean follow-up was 10 years (range 1-31.1 years). The diagnosis of vaginal atresia was made pre-operatively in 12 out of 15 patients, and in three patients it was identified during the anoplasty. The anorectal malformations were rectoperineal (N=2), rectovestibular (N=6), recto-bladder neck (N=1) and imperforate anus without fistula (N=6). Satisfactory surgical repair was performed in 13 patients, while one continues to stool through a low perineal fistula awaiting definitive surgery and another underwent a colostomy and mucous fistula. Delayed vaginal reconstruction was due to a failure to identify the problem prior to anoplasty (N=3). Long-term results demonstrated that anorectal continence was much worse than initially appreciated, and many had associated urinary incontinence. Overall stooling score was far lower than in a separate group of children with imperforate anus without vaginal atresia (Levitt and Peña, 2007). Vaginal atresia with imperforate anus is a rare and an extensive pre-operative workup of females with imperforate anus must include assessment of vagina patency. Vaginal reconstruction and anorectal continuity can be performed in a variety of approaches, but long-term continence is often not optimal. We propose a pathway for management of this difficult genito-anorectal disorder. Copyright © 2015 Elsevier Inc. All rights reserved.
Parsons, J M; Rees, M R; Gibbs, J L
A neonate with pulmonary atresia and an intact ventricular septum with a tripartite right ventricle was successfully treated by percutaneous balloon dilatation of the pulmonary valve. This was facilitated by previous laser valvotomy with a hot tip Trimedyne laser wire. There were no major complications. Four weeks later the patient was discharged home on no medication with peripheral oxygen saturations of 70% in air. Images PMID:1854575
Rosenthal, E; Qureshi, S A; Kakadekar, A P; Anjos, R; Baker, E J; Tynan, M
OBJECTIVE--To investigate the efficacy and safety of transcatheter laser-assisted valve dilatation for atretic valves in children with congenital heart disease. DESIGN--Prospective clinical study. SETTING--Supraregional paediatric cardiology centre. SUBJECTS--Eleven children (aged 1 day-11 years; weight 2.1-35.7 kg) with atresia of pulmonary (10) or tricuspid (one) valve underwent attempted laser-assisted valve dilatation as part of the staged treatment of their cyanotic heart disease. INTERVENTION--After delineating the atretic valve by angiography and/or echocardiography a 0.018 inch "hot tip" laser wire was used to perforate the atretic valve. Subsequently the valve was dilated with conventional balloon dilatation catheters up to the valve annulus diameter. RESULTS--Laser-assisted valve dilatation was successfully accomplished in nine children. In two neonates with pulmonary valve atresia, intact ventricular septum, and coexistent infundibular atresia the procedure resulted in cardiac tamponade: one died immediately and one later at surgery. During a follow up of 1-17 months (mean 11) two infants with pulmonary valve atresia and intact ventricular septum died (one with congestive cardiac failure). The remainder are either well palliated and do not require further procedures (three), or are awaiting further transcatheter or surgical procedures because of associated defects (four). CONCLUSIONS--Laser-assisted valve dilatation is a promising adjunct to surgery in this high risk group of patients. It may avoid surgery in some patients, and may reduce the number of surgical procedures in those requiring staged operations. Images PMID:8343325
Yao, H H; Volentine, K K; Bahr, J M
The germinal disc region (GDR), which contains the germinal disc and overlying granulosa cells, is essential for completion of maturation of the preovulatory chicken follicle. The current study was conducted to test the hypothesis that destruction of the GDR (GDRX) of an immature preovulatory chicken follicle blocks ovulation, induces apoptosis, and causes atresia. The GDR of immature preovulatory follicles (F2) were destroyed by freezing with dry ice (3 mm in diameter) 48-50 h before ovulation. As a control for the effect of freezing, a nonGDR portion (a portion of the follicular wall opposite to the GDR relative to the follicular stalk) of other F2 follicles were destroyed (nonGDRX). Treatment of F2 follicles by GDRX caused atresia and blocked ovulation of all treated follicles (6 of 6), whereas none of the nonGDRX follicles (0 of 5) underwent atresia. Treatment of follicles by GDRX induced apoptotic DNA fragmentation (laddering) in theca and granulosa layers obtained from the frozen area and in the theca layer obtained from the follicular wall distal to the frozen area. In contrast, apoptosis was only present in theca and granulosa layers in the frozen area of the nonGDRX follicle. Furthermore, the in situ DNA end-labeling technique demonstrated that in the GDRX follicle 24 h after treatment, cells in the theca interna, endothelial cells in blood vessels of the theca externa, and a few granulosa cells underwent apoptosis. These results indicate that destruction of the GDR of an immature preovulatory follicle causes atresia and apoptosis and blocks ovulation. These novel findings suggest that the GDR maintains development of the chicken preovulatory follicle by producing one or more survival factors. Without the GDR, chicken follicles cannot develop further and they eventually die.
Mahalingam, Sharada; Gao, Liying; Gonnering, Marni; Helferich, William; Flaws, Jodi A.
Equol is a non-steroidal estrogen metabolite produced by microbial conversion of daidzein, a major soy isoflavone, in the gut of some humans and many animal species. Isoflavones and their metabolites can affect endogenous estradiol production, action, and metabolism, potentially influencing ovarian follicle function. However, no studies have examined the effects of equol on intact ovarian antral follicles, which are responsible for sex steroid synthesis and further development into ovulatory follicles. Thus, the present study tested the hypothesis that equol inhibits antral follicle growth, increases follicle atresia, and inhibits steroidogenesis in the adult mouse ovary. To test this hypothesis, antral follicles isolated from adult CD-1 mice were cultured with vehicle control (dimethyl sulfoxide; DMSO) or equol (600 nM, 6 μM, 36 μM, 100 μM) for 48 and 96 h. Every 24 h, follicle diameters were measured to monitor growth. At 48 and 96 h, the culture medium was subjected to measurement of hormone levels, and the cultured follicles were subjected to gene expression analysis. Additionally, follicles were histologically evaluated for signs of atresia after 96 h of culture. The results indicate that equol (100 μM) inhibited follicle growth, altered the mRNA levels of bcl2-associated X protein and B cell leukemia/lymphoma 2, and induced follicle atresia. Further, equol decreased the levels of estradiol, testosterone, androstenedione, and progesterone, and it decreased mRNA levels of cholesterol side-chain cleavage, steroid 17-α-hydroxalase, and aromatase. Collectively, these data indicate that equol inhibits growth, increases atresia, and inhibits steroidogenesis of cultured mouse antral follicles. PMID:26876617
Down's syndrome (DS) is associated with duodenal atresia (DA) in about 8-10% of cases. Transient Myeloproliferative Disorder (TMD)/Acute Myeloid Leukemia (AML) is also associated with the trisomy 21 mutation. The occurrence of the two conditions together complicates the diagnosis and surgical management of the DA. We discuss the technical aspects of management of the DA in this clinical setting. PMID:26023452
Huang, Chao-Cheng; Chuang, Jiin-Haur; Chou, Ming-Huei; Wu, Chia-Lin; Chen, Ching-Mei; Wang, Chih-Chi; Chen, Yaw-Sen; Chen, Chao-Long; Tai, Ming-Hong
Matrix metalloproteinases (MMPs) are the proteases responsible for tissue remodeling during liver fibrosis caused by various disorders including biliary atresia. However, information regarding the relative contribution of these proteases to liver fibrosis is still limited. We studied matrix metalloproteinase-2 (MMP-2), -7, -9 and -13 mRNA expressions in the liver tissue of early-stage biliary atresia at the time of Kasai's procedure, late-stage biliary atresia at the time of liver transplantation with advanced fibrosis and nondiseased control without liver fibrosis. The results of real-time quantitative reverse transcriptase-PCR analysis revealed that only MMP-2 and -7 expressions were significantly different between groups. MMP-2 was significantly higher in Liver Transplantation group than both in Control (P=0.010) and in Kasai's Procedure (P=0.001) groups, whereas the difference of MMP-2 expression between Control and Kasai's Procedure was not significant. However, the relative expression level of MMP-7 was sequentially elevated when comparing Control, Kasai's Procedure and Liver Transplantation groups, and there was significant (P=0.019) difference when comparing Control and Liver Transplantation groups. Moreover, the fold difference in MMP-7 mRNA was much higher than that in MMP-2 mRNA between groups. The expressions of MMP-7 were further confirmed by agarose gel electrophoresis and Western blotting. Immunohistochemical analysis revealed a significant positive correlation of the scores of MMP-7 immunostaining with the stages of liver fibrosis. In situ hybridization demonstrated that the bile ductular epithelial cells, Kupffer cells and hepatocytes were the major producers of matrix metalloproteinase-7 in the liver. Our results imply that MMP-7 is a major MMP associated with the tissue remodeling during the progression of liver fibrosis in biliary atresia.
Yu, Joannie Ka Yin; Wong, Lena Lai Nar; Tsang, Willis Sung Shan; Tong, Michael Chi Fai
Background. Patients with unilateral atresia and microtia encounter problems in sound localization and speech understanding in noise. Although there are four implantable hearing devices available, there is little discussion and evidence on the application of these devices on patients with unilateral atresia and microtia problems. Objective. This paper will review the details of these four implantable hearing devices for the treatment of unilateral atresia. They are percuteaneous osseointegrated bone anchored hearing aid, Vibrant Soundbridge middle ear implant, Bonebridge bone conduction system, and Carina fully implantable hearing device. Methods. Four implantable hearing devices were reviewed and compared. The clinical decision process that led to the recommendation of a device was illustrated by using a case study. Conclusions. The selection of appropriate implantable hearing devices should be based on various factors, including radiological findings and patient preferences, possible surgical complications, whether the device is Food and Drug Administration- (FDA-)/CE-approved, and the finances. To ensure the accurate evaluation of candidacy and outcomes, the evaluation methods should be adapted to suite the type of hearing device. PMID:24991564
Futatsugi, Akiko; Hidaka, Eiko; Kubota, Noriko; Nishijima, Fumie; Yoshizawa, Katsumi; Ishimine, Nau; Sugano, Mitsutoshi; Hori, Atsushi; Hidaka, Hiroya
Lipoprotein-X (LP-X) in cholestatic jaundice causes abnormal reaction in assays for low-density lipoprotein-cholesterol, but the effects on other test items are unknown. Here, we report an infant with biliary atresia showing abnormal reaction in total serum protein assay using the biuret method, and lipoprotein-X (LP-X) was then detected. In this 11-month-old female infant, jaundice was observed at 2 months old, and a diagnosis of biliary atresia was made. On biochemical tests at 12 months old, the total serum protein concentrations detected by the biuret method were very high, and the response curve and linearity of dilution were abnormal. LP-X was detected by agar electrophoresis. In addition and recovery experiments with normal serum fractionation of the patient's LP-X-rich lipoprotein fraction prepared by ultracentrifugation, normal γ-globulin fractionation showed an abnormal reaction by the biuret method. In infants with biliary atresia, we showed that the total serum protein assay by the biuret method was influenced by LP-X-rich lipoprotein, which may be caused by abnormal reaction of LP-X and γ-globulin. [Case Report].
Yue, Fan; Yibei, Wang; Zhen, Wang; Pu, Wang; Xiaowei, Chen
Using questionnaires to evaluate the audiological benefit and satisfaction of bilateral aural atresia patients with bone-anchored hearing aid (Baha). Implanted Baha user questionnaire was applied to 19 patients suffering bilateral aural atresia, and 15 of the patients were evaluated with abbreviated profile of hearing aid benefit (APHAB). Glasgow children's benefit inventory (GCBI) was used to measure subjective benefit of patients under the age of 18. The Baha user questionnaire demonstrated great satisfaction. The mean Baha scores for the subdomains of ease of communication (EC), backgroud noise (BN) and revereration (RV) were decreased by 54.6 ± 10.2 (t = 20.6, P < 0.05), 46.9 ± 11.1 (t = 16.4, P < 0.05) and 58.8 ±15.4 (t = 21.4, P < 0.05) compared to the unaided scores. For the subdomain of aversiveness (AV), the Baha score was 56.7 ± 9.9 (t = 10.8, P < 0.05) higher than the unaided score. The general benefit score GCBI was 41.1 ± 13.0. Baha can significantly improve hearing ability and quality of life of patients with bilateral aural atresia.
Dolenšek, Janez; Cvetko, Erika; Snoj, Žiga; Meznaric, Marija
Fusion of the atlas with the occipital bone is a rare congenital dysplasia known as occipitalization of the atlas, occipitocervical synostosis, assimilation of the atlas, or atlanto-occipital fusion. It is a component of the paraxial mesodermal maldevelopment and commonly associated with other dysplasias of the craniovertebral junction. External auditory canal atresia or external aural atresia is a rare congenital absence of the external auditory canal. It occurs as the consequence of the maldevelopment of the first pharyngeal cleft due to defects of cranial neural crest cells migration and/or differentiation. It is commonly associated with the dysplasias of the structures derived from the first and second pharyngeal arches including microtia. We present the coexistence of the occipitalization of the atlas and congenital aural atresia, an uncommon combination of the paraxial mesodermal maldevelopment, and defects of cranial neural crest cells. The association is most probably syndromic as minimal diagnostic criteria for the oculoariculovertebral spectrum are fulfilled. From the clinical point of view, it is important to be aware that patients with microtia must obtain also appropriate diagnostic imaging studies of the craniovetebral junction due to eventual concomitant occipitalization of the atlas and frequently associated C1-C2 instability.
Sokol, Ronald J.; Shepherd, Ross W.; Superina, Riccardo; Bezerra, Jorge A.; Robuck, Patricia; Hoofnagle, Jay H.
Biliary atresia is the most common cause of end-stage liver disease in the infant and is the leading pediatric indication for liver transplantation in the United States. Earlier diagnosis (<30-45 days of life) is associated with improved outcomes following the Kasai portoenterostomy and longer survival with the native liver. However, establishing this diagnosis is problematic because of its rarity, the much more common indirect hyperbilirubinemia that occurs in the newborn period, and the schedule for routine infant health care visits in the United States. The pathogenesis of biliary atresia appears to involve immune-mediated fibro-obliteration of the extrahepatic and intrahepatic biliary tree in most patients and defective morphogenesis of the biliary system in the remainder. The determinants of the outcome of portoenterostomy include the age at surgery, the center's experience, the presence of associated congenital anomalies, and the postoperative occurrence of cholangitis. A number of screening strategies in infants have been studied. The most promising are early measurements of serum conjugated bilirubin and a stool color card given to new parents that alerts them and their primary care provider to acholic stools. This report summarizes a National Institutes of Health workshop held on September 12 and 13, 2006, in Bethesda, MD, that addressed the issues of outcomes, screening, and pathogenesis of biliary atresia. PMID:17661405
Mboyo, Antoine; Clermidi, Pauline; Podevin, Guillaume; Patkowski, Dariusz; Baglaj, Maciej; Gerus, Sylwester; Lalioui, Abdelfetah; Napoli-Cocci, Stéphan de; Robert, Michel
Pyloric atresia (PA) is a rare condition, and may be misdiagnosed and especially confused for duodenal atresia pre-operatively. We looked for clues to avoiding pre-operative misdiagnosis and hence allow the best neonatal medical and surgical management. A retrospective case-note review was carried out of the five patients managed in four centres with the diagnosis of isolated PA. We focused on antenatal ultrasound findings, postnatal clinical and radiological features, operative findings, surgical procedures and outcomes. Four patients had polyhydramnios and one double bubble sign on antenatal ultrasound. After birth, non-bilious vomiting and upper abdominal distension were the main symptoms. Gastric decompression showed non-bilious gastric fluid. Radiological findings were a large gastric air bubble with no gas beyond in all cases. The diagnosis of duodenal atresia was postulated at first in all cases. The diagnosis of PA was established peroperatively. One patient referred late, died 13-day post-operatively of cardiopulmonary failure secondary to a severe pneumonia that may be related to aspiration syndrome. Outcomes were otherwise satisfactory. Even though it is a rare diagnosis, PA has a specific clinical and radiological presentation underlined here that should be kept in mind when managing a neonate with a gastric outlet obstruction.
Joseph, G; Mandalay, A; Rajendiran, G
Congenital isolated local atresia of the aortic isthmus is anatomically similar and morphogenetically related to congenital coarctation of the aorta and was encountered in 4 out of 26 consecutive adult patients selected for coarctation angioplasty at our center. Anterograde recanalization of the atresia was safely and successfully accomplished in all four patients, using a brachial approach. Balloon dilation in the four patients, with ancillary stent implantation in one patient, resulted in reduction of translesion gradient from 84 +/- 11 mm Hg to 9 +/- 7 mm Hg without complications. Angiography in the three non-stented patients after a mean follow-up of 13 months showed no evidence of restenosis, dissection or aneurysm formation, though one patient had mild dilatation of the posterior aspect of the aortic isthmus. The clinical presentation of patients with isolated local aortic atresia, and their short- to mid-term response to percutaneous treatment, is similar to that of patients with isolated severe coarctation of aorta. Copyright 2001 Wiley-Liss, Inc.
Graham, M Elise; Haworth, Rebecca; Chorney, Jill; Bance, Manohar; Hong, Paul
The benefits of bone-anchored hearing devices (BAHD) in children with unilateral aural atresia are controversial. We sought to determine whether there is parental decisional conflict surrounding elective placement of BAHD for this indication. Caregivers of pediatric patients with unilateral aural atresia and normal contralateral ear undergoing percutaneous BAHD consultation were enrolled. All consultations were carried out by one pediatric otolaryngologist in a consistent manner. After consultation, the participants completed a demographics form and the Decisional Conflict Scale (DCS) questionnaire. Twenty-three caregivers of 15 male (65.2%) and 8 female (34.8%) children (mean age 5.65 years) participated. The overall median DCS score was 15.63 (standard error = 4.21). Significant decisional conflict (DCS score ≥ 25) was found in 10 participants (43.5%). The median DCS score in the group choosing surgery was 5.47, and it was 23.44 in those who did not choose surgery (Mann-Whitney U = 39, Z = -1.391, P = .164). The median DCS score for mothers and fathers was 25 and 3.91, respectively. Many parents experienced significant decisional conflict when considering percutaneous BAHD surgery in children with unilateral aural atresia in our study population. Future research should explore the impact of decisional conflict on health outcomes. © The Author(s) 2015.
Conighi, Maria Luisa; Fasoli, Lorella; Bucci, Valeria; Battaglino, Francesco; Chiarenza, Salvatore Fabio
Ureteral atresia is a rare disease usually associated with a non-functioning dysplastic kidney. The condition may be unilateral or bilateral; focal, short or long and may involve any part of the ureter. Association with other urinary anomalies is rare. We report the case of a 10-month-old boy with prenatal diagnosis of multicystic right kidney. This suspicion was confirmed after birth by ultrasound and static scintigraphy; a right vesicoureteral reflux (VUR) was recorded at cystography. The boy presented a regular renal function but was hospitalized twice for suspected pyelonephritis between the 8th and the 10th month of life and were recorded occasional mild changes in blood pressure. Antibiotic prophylaxis was administered until surgery. When he was 10-month underwent retroperitoneoscopy to perform a nephroureterectomy finding a complete atresia of the upper third of the ureter with the blind end at the level of the uretero-pelvic-junction. The programmed surgery was performed. By a revision of literature, only few cases of imperforate distal ureter have been described. This condition is associated with a kidney dysplasia. The atresia of the ureter with no signs of infection in the dysplastic kidney may be unknown up to adulthood or throughout one’s life. Prognosis usually depends on the severity of the obstruction. PMID:28164035
Bleve, Cosimo; Conighi, Maria Luisa; Fasoli, Lorella; Bucci, Valeria; Battaglino, Francesco; Chiarenza, Salvatore Fabio
Ureteral atresia is a rare disease usually associated with a non-functioning dysplastic kidney. The condition may be unilateral or bilateral; focal, short or long and may involve any part of the ureter. Association with other urinary anomalies is rare. We report the case of a 10-month-old boy with prenatal diagnosis of multicystic right kidney. This suspicion was confirmed after birth by ultrasound and static scintigraphy; a right vesicoureteral reflux (VUR) was recorded at cystography. The boy presented a regular renal function but was hospitalized twice for suspected pyelonephritis between the 8th and the 10th month of life and were recorded occasional mild changes in blood pressure. Antibiotic prophylaxis was administered until surgery. When he was 10-month underwent retroperitoneoscopy to perform a nephroureterectomy finding a complete atresia of the upper third of the ureter with the blind end at the level of the uretero-pelvic-junction. The programmed surgery was performed. By a revision of literature, only few cases of imperforate distal ureter have been described. This condition is associated with a kidney dysplasia. The atresia of the ureter with no signs of infection in the dysplastic kidney may be unknown up to adulthood or throughout one's life. Prognosis usually depends on the severity of the obstruction.
Nigro Stimato, Vittoria; Didier, Dominique; Beghetti, Maurice; Tissot, Cécile
Atresia of the aortic arch is a rare congenital heart defect with a high mortality when associated with other intracardiac defects. Cardiac magnetic resonance (CMR) provides the exact anatomy of the aortic arch and collateral circulation and is useful to diagnose-associated aortic arch anomalies. This report describes the case of a 4-year-old child with atresia of the aortic arch, referred to our institution with the diagnosis of aortic coarctation and bicuspid aortic valve. On clinical exam, the femoral pulses were not palpable and there was a significant differential blood pressure between the upper and lower limbs. The echocardiography showed a severely stenotic bicuspid aortic valve but was limited for the exact description of the aortic arch. CMR showed absence of lumen continuity between the ascending and descending aorta distal to the left subclavian artery, extending over 5 mm, with the presence of a bend in the arch and diverticulum on either side of the zone of discontinuity, suggesting the diagnosis atresia of the aortic arch rather than coarctation or interruption. The patient benefited from a successful surgical commissurotomy of the aortic valve and reconstruction of the aortic arch with a homograft. The post-operative CMR confirmed the good surgical result. This case emphasizes the utility of CMR to provide good anatomical information to establish the exact diagnosis and the operative strategy. PMID:25853109
Background In this study, we investigated the effect of hyperbaric oxygen therapy (HBOT) on the morphology of estradiol valerate (EV) induced polycystic ovary (PCO) to find a new treatment modality for improvement of PCO. Methods The rats were divided into four groups. Group1, control; group 2, PCO group; group 3, PCO with HBOT group and group 4, normal ovary with HBOT. PCO was induced by a single intramuscular injection of 4 mg EV in adult cycling rats. Other rats with normal ovaries had oil injection as placebo. HBOT was applied to third and fourth groups for six weeks. Histopathologic evaluation of ovaries of all groups were performed & compared. Results Six weeks of HBOT was resulted in increase in follicular atresia, decrease in the number of primary, secondary, tertiary follicles and decrease in the number of fresh corpus luteum in normal rat ovary. HBOT on polycystic rat ovary, resulted in significant increase in atretic follicles which were already present. Conclusions HBOT of six weeks itself, changed ovarian morphology in favor of atresia both in PCO group and control group. This result of aggravated follicular atresia after HBOT on EV induced PCO may be due to long-term exposure in our protocol which with this state seems to be inapplicable in the improvement of PCO morphology. PMID:22309835
Background Young age at portoenterostomy has been linked to improved outcome in biliary atresia, but pre-existing biological factors may influence the rate of disease progression. In this study, we aimed to determine whether molecular profiling of the liver identifies stages of disease at diagnosis. Methods We examined liver biopsies from 47 infants with biliary atresia enrolled in a prospective observational study. Biopsies were scored for inflammation and fibrosis, used for gene expression profiles, and tested for association with indicators of disease severity, response to surgery, and survival at 2 years. Results Fourteen of 47 livers displayed predominant histological features of inflammation (N = 9) or fibrosis (N = 5), with the remainder showing similar levels of both simultaneously. By differential profiling of gene expression, the 14 livers had a unique molecular signature containing 150 gene probes. Applying prediction analysis models, the probes classified 29 of the remaining 33 livers into inflammation or fibrosis. Molecular classification into the two groups was validated by the findings of increased hepatic population of lymphocyte subsets or tissue accumulation of matrix substrates. The groups had no association with traditional markers of liver injury or function, response to surgery, or complications of cirrhosis. However, infants with an inflammation signature were younger, while those with a fibrosis signature had decreased transplant-free survival. Conclusions Molecular profiling at diagnosis of biliary atresia uncovers a signature of inflammation or fibrosis in most livers. This signature may relate to staging of disease at diagnosis and has implications to clinical outcomes. PMID:20465800
Cassini, Pamela; Montironi, Alberto; Botti, Sara; Hori, Tetsuo; Okhawa, Haruo; Stella, Alessandra; Andersson, Leif; Giuffra, Elisabetta
Anal atresia is a relatively common congenital malformation that occurs in about 1 out of 5000 infants, caused by abnormal hindgut development of the embryo, often associated with other developmental anomalies (e.g., Currarino, Townes-Brock, Pallister-Hall syndromes, and VATER association). Genetic analysis in human families is exceedingly difficult due to the multifactorial nature of the trait. In pigs, anal atresia occurs at a higher incidence (0.18%) than in humans. A complete genome scan (165 microsatellite markers) was performed using a backcross pedigree previously obtained by crossing affected animals from a partially inbred line, selected for a high incidence of anal atresia, with an unaffected male of a different breed (Meishan). The data set was analyzed with classical linkage (TWOPOINT) and nonparametric genetic methods (NPL, Non-Parametric Linkage, and TDT, Transmission Disequilibrium Test). Both methods support association of the trait with two loci on Chromosomes 9 and 15. GLI2 (GLI-Kruppel family member GLI2) was identified as a positional candidate gene based on comparative mapping; radiation hybrid mapping confirmed that this locus is located within the QTL region.
Verhaert, Nicolas; Fuchsmann, Carine; Tringali, Stéphane; Lina-Granade, Geneviève; Truy, Eric
To describe our surgical and audiometric experience using different active middle ear implant strategies facing various anatomic situations in aural atresia. Retrospective case review. Tertiary academic referral center. Five patients with congenital aural atresia, (3 unilateral and 2 bilateral), with mean age of 22.4 years (range, 12-44 yr), referred for hearing rehabilitation. Active middle ear implant on stapes capitulum. Description of surgical implantation with different active middle ear implants. Preoperative and postoperative air conduction, bone conduction, and aided and unaided thresholds and speech scores were measured, at mid to long term. Subjective benefit analysis was determined through the Abbreviated Profile of Hearing Aid Benefit questionnaire. After activation and fitting of the devices, a mean functional gain of 32.5 dB hearing level was measured. Speech tests in quiet showed a mean functional gain of 20.2 dB. Patients had a mean follow-up period of 12 months. No intraoperative or postoperative complications were noted. Furthermore, we reflected on new coupling possibilities, especially in a difficult case with stapes-footplate fixation where no approach of the round window was feasible because of aberrant facial nerve course. Facing anatomic variations in congenital aural atresia, active middle ear implants can provide substantial hearing improvement in safe conditions and open new strategies for hearing rehabilitation.
Gibbs, J L; Rothman, M T; Rees, M R; Parsons, J M; Blackburn, M E; Ruiz, C E
OBJECTIVE--To assess the possibility of maintaining ductal patency in neonates with complex pulmonary atresia by percutaneous implantation of balloon expandable stents. PATIENTS--Two duct-dependent neonates with long segment pulmonary atresia, right sided aortic arch, and left sided arterial duct. RESULTS--Stents with final diameter of 3.5 or 4 mm and initial length of 7 or 15 mm were successfully positioned in the arterial duct. Two stents were required in one child and four in the other in order to stent the entire length of the duct. After the procedures the ducts remained widely patent and arterial oxygen saturations remained above 80%. Complications of the procedures included perforation of a peripheral pulmonary artery and cardiac perforation, both caused by guide wire manipulation. Both babies died suddenly, one at five weeks, and the other at nine days after successful stenting of the duct. Both ducts were patent at necropsy; the exact cause of one death was not clearly defined, but the second seemed to be caused by pneumococcal septicaemia. CONCLUSIONS--Stenting of the arterial duct is technically feasible. It provides adequate palliation for neonates with pulmonary atresia at least in the short term and it seems to result in balanced, central perfusion of both pulmonary arteries. This preliminary report suggests that this previously untried technique may prove to be a promising and attractive alternative to neonatal aortopulmonary shunt operation. Images PMID:1372815
Peter, C P; Handt, L K; Smith, S M
Animal studies were done using an in vivo dog model to examine the possible mechanism for the esophageal adverse events reported with alendronate sodium tablets. These studies showed that under low pH conditions alendronate sodium can cause esophageal irritation. No esophageal irritation occurred at pH 3.5 or higher where the drug exists primarily as the sodium salt. The animal studies also showed that alendronate sodium can exacerbate preexisting esophageal damage. Exposure of the esophageal mucosa for a prolonged period to alendronate sodium tablet can also cause mild esophageal irritation. These findings suggest that the esophageal irritation in patients taking Fosamax can be from prolonged contact with the tablet, reflux of acidic gastric contents with alendronate sodium, and exacerbation of preexisting esophageal damage. The findings also suggest that other bisphosphonates can cause esophageal injury under similar conditions.
Guo, Jun-Hui; Xing, Guo-Lan; Fang, Xin-Hui; Wu, Hui-Fang; Zhang, Bo; Yu, Jin-Zhong; Fan, Zong-Min; Wang, Li-Dong
AIM To understand the molecular mechanism of esophageal cancer development and provide molecular markers for screening high-risk populations and early diagnosis. METHODS Two-dimensional electrophoresis combined with mass spectrometry were adopted to screen differentially expressed proteins in nine cases of fetal esophageal epithelium, eight cases of esophageal cancer, and eight cases of tumor-adjacent normal esophageal epithelium collected from fetuses of different gestational age, or esophageal cancer patients from a high-risk area of esophageal cancer in China. Immunohistochemistry (avidin-biotin-horseradish peroxidase complex method) was used to detect the expression of peroxiredoxin (PRX)6 in 91 cases of esophageal cancer, tumor-adjacent normal esophageal tissue, basal cell hyperplasia, dysplasia, and carcinoma in situ, as well as 65 cases of esophageal epithelium from fetuses at a gestational age of 3-9 mo. RESULTS After peptide mass fingerprint analysis and search of protein databases, 21 differential proteins were identified; some of which represent a protein isoform. Varying degrees of expression of PRX6 protein, which was localized mainly in the cytoplasm, were detected in adult and fetal normal esophageal tissues, precancerous lesions, and esophageal cancer. With the progression of esophageal lesions, PRX6 protein expression showed a declining trend (P < 0.05). In fetal epithelium from fetuses at gestational age 3-6 mo, PRX6 protein expression showed a declining trend with age (P < 0.05). PRX6 protein expression was significantly higher in well-differentiated esophageal cancer tissues than in poorly differentiated esophageal cancer tissues (P < 0.05). CONCLUSION Development and progression of esophageal cancer result from interactions of genetic changes (accumulation or superposition). PRX6 protein is associated with fetal esophageal development and cancer differentiation. PMID:28293090
Niedzielski, Joshua S.; Yang, Jinzhong; Stingo, Francesco; Martel, Mary K.; Mohan, Radhe; Gomez, Daniel R.; Briere, Tina M.; Liao, Zhongxing; Court, Laurence E.
Purpose To study radiation-induced esophageal expansion as an objective measure of radiation esophagitis in patients with non-small-cell lung cancer (NSCLC) treated with IMRT. Methods and Materials Eighty-five patients had weekly intra-treatment CT imaging and esophagitis scoring according to Common Toxicity Criteria for Adverse Events 4.0, (24 Grade0, 45 Grade2, and 16 Grade3). Nineteen esophageal expansion metrics based on mean, maximum, spatial length, and volume of expansion were calculated as voxel-based relative volume change, using the Jacobian determinant from deformable image registration between the planning and weekly CTs. An anatomic variability correction method was validated and applied to these metrics to reduce uncertainty. An analysis of expansion metrics and radiation esophagitis grade was conducted using normal tissue complication probability (NTCP) from univariate logistic regression and Spearman rank for grade 2 and grade 3 esophagitis endpoints, as well as the timing of expansion and esophagitis grade. Metrics’ performance in classifying esophagitis was tested with Receiver Operating Characteristic (ROC) analysis. Results Expansion increased with esophagitis grade. Thirteen of nineteen expansion metrics had ROC area under the curve (AUC) values >0.80 for both grade 2 and grade 3 esophagitis endpoints, with the highest performance from maximum axial expansion (MaxExp1) and esophageal length with axial expansion ≥30% (LenExp30%) with AUCs of 0.93 and 0.91 for grade2, 0.90 and 0.90 for grade3 esophagitis, respectively. Conclusions Esophageal expansion may be a suitable objective measure of esophagitis, particularly maximum axial esophageal expansion and esophageal length with axial expansion ≥30%, with 2.1 Jacobian Value and 98.6mm as the metric value for 50% probability of grade3 esophagitis. The uncertainty in esophageal Jacobian calculations can be reduced with anatomic correction methods. PMID:26675063
... in the esophagus. One example is acid reflux . Proton-pump inhibitors (PPIs) are medications that help reduce ... other conditions caused by too much stomach acid. Proton-pump inhibitor responsive esophageal eosinophilia or PPI-REE ...
... page: https://medlineplus.gov/news/fullstory_165596.html New Device Approved for Esophageal Birth Defect Condition affects ... 2017 MONDAY, May 15, 2017 (HealthDay News) -- A new medical device has been approved by the U.S. ...
Pathologies that involve the structure and/or function of the esophagus can be life-threatening. The esophagus is a complex organ comprising nonredundant tissue that does not have the ability to regenerate. Currently available interventions for esophageal pathology have limited success and are typically associated with significant morbidity. Hence, there is currently an unmet clinical need for effective methods of esophageal repair. The present article presents a review of esophageal disease along with the anatomic and functional consequences of each pathologic process, the shortcomings associated with currently available therapies, and the latest advancements in the field of regenerative medicine with respect to strategies for esophageal repair from benchtop to bedside. PMID:25813694
Shah, Nisha A; Albert, Dustin M; Hall, Noah M; Moawad, Fouad J
Eosinophilic esophagitis (EoE) is a chronic and progressive immune-mediated condition defined by symptoms of esophageal dysfunction and dense eosinophilic infiltration of the esophageal mucosa. Therapies consist of anti-eosinophilic medications and specialized diets aimed to decrease the progression of EoE and alleviate its symptoms, namely, dysphagia and food impaction. Assessing response to therapy remains challenging, as treatment end points are not well defined and currently consist of clinical, histologic, and endoscopic features. Newer validated measures may help standardize treatment end points. Emerging data support the use of maintenance therapy, which may reduce disease progression. Optimal dosages, delivery techniques, and duration of treatment need to be determined. When features of fibrostenosis develop, esophageal dilation is a safe and effective adjunctive strategy for improving symptoms. In EoE cases refractory to conventional treatments, newer therapies targeting inflammatory mediators and cytokines are on the horizon. PMID:27695356
...) Identification. An esophageal obturator is a device inserted through a patient's mouth to aid ventilation of the... that is attached to a face mask. (b) Classification. Class II (performance standards). ...
Boniface, Megan M; Wani, Sachin B; Schefter, Tracey E; Koo, Phillip J; Meguid, Cheryl; Leong, Stephen; Kaplan, Jeffrey B; Wingrove, Lisa J; McCarter, Martin D
The management of esophageal and gastric cancer is complex and involves multiple specialists in an effort to optimize patient outcomes. Utilizing a multidisciplinary team approach starting from the initial staging evaluation ensures that all members are in agreement with the plan of care. Treatment selection for esophageal and gastric cancer often involves a combination of chemotherapy, radiation, surgery, and palliative interventions (endoscopic and surgical), and direct communication between specialists in these fields is needed to ensure appropriate clinical decision making. At the University of Colorado, the Esophageal and Gastric Multidisciplinary Clinic was created to bring together all experts involved in treating these diseases at a weekly conference in order to provide patients with coordinated, individualized, and patient-centered care. This review details the essential elements and benefits of building a multidisciplinary program focused on treating esophageal and gastric cancer patients. PMID:27217796
... In the United States, esophageal cancer is rare, accounting for only 1 percent of all new cancers ... advanced stage. Stomach cancer, likewise, is also rare, accounting for fewer than 2 percent of all new ...
del Genio, Gianmattia; del Genio, Federica; Schettino, Pietro; Limongelli, Paolo; Tolone, Salvatore; Brusciano, Luigi; Avellino, Manuela; Vitiello, Chiara; Docimo, Giovanni; Pezzullo, Angelo; Docimo, Ludovico
Squamous papilloma of the esophagus is a rare benign lesion of the esophagus. Radiofrequency ablation is an established endoscopic technique for the eradication of Barrett esophagus. No cases of endoscopic ablation of esophageal papilloma by radiofrequency ablation (RFA) have been reported. We report a case of esophageal papilloma successfully treated with a single session of radiofrequency ablation. Endoscopic ablation of the lesion was achieved by radiofrequency using a new catheter inserted through the working channel of endoscope. The esophageal ablated tissue was removed by a specifically designed cup. Complete ablation was confirmed at 3 mo by endoscopy with biopsies. This case supports feasibility and safety of as a new potential indication for BarrxTM RFA in patients with esophageal papilloma. PMID:25789102
Kobylecka, Małgorzata; Olesińska, Marzena
Systemic sclerosis is a rare connective tissue disease, distinctive features of which are fibrosis and microangiopathy. The esophagus is one of the most commonly involved internal organs. Most patients experience dysphagia, difficulties in swallowing and gastro-esophageal reflux. However, in up to one third of cases, the initial onset of esophageal disease may be clinically silent. There are several diagnostic modalities available for assessing both morphological and functional abnormalities of the esophagus. If structural abnormalities are suspected, endoscopy is the method of choice. Functional evaluation is best achieved with manometry. Both endoscopy and manometry are invasive techniques, with low patient acceptance. Barium-contrast study is well tolerated, but qualitative assessment of functional abnormalities is imprecise. Esophageal scintigraphy is an easy, non-invasive, sensitive and specific diagnostic modality. It can detect esophageal dysfunction even in asymptomatic patients. In patients already diagnosed with systemic sclerosis, scintigraphy is useful in evaluating severity and progression of the disease. PMID:27994270
Chojnowski, Marek; Kobylecka, Małgorzata; Olesińska, Marzena
Systemic sclerosis is a rare connective tissue disease, distinctive features of which are fibrosis and microangiopathy. The esophagus is one of the most commonly involved internal organs. Most patients experience dysphagia, difficulties in swallowing and gastro-esophageal reflux. However, in up to one third of cases, the initial onset of esophageal disease may be clinically silent. There are several diagnostic modalities available for assessing both morphological and functional abnormalities of the esophagus. If structural abnormalities are suspected, endoscopy is the method of choice. Functional evaluation is best achieved with manometry. Both endoscopy and manometry are invasive techniques, with low patient acceptance. Barium-contrast study is well tolerated, but qualitative assessment of functional abnormalities is imprecise. Esophageal scintigraphy is an easy, non-invasive, sensitive and specific diagnostic modality. It can detect esophageal dysfunction even in asymptomatic patients. In patients already diagnosed with systemic sclerosis, scintigraphy is useful in evaluating severity and progression of the disease.
Fukuchi, M; Otake, S; Naitoh, H; Shoji, H; Yamagishi, J; Suzuki, M; Yanoma, T; Kuwano, H
Autoimmune blistering skin diseases, including pemphigus vulgaris, rarely involve the esophagus. We report a case of exfoliative esophagitis with pemphigus vulgaris. A sloughing esophageal cast observed by endoscopy was dissected esophageal squamous epithelium in all layers. Our case is the fifth case of pemphigus vulgaris associated with esophageal cast formation recorded in the medical literature. Prednisolone was administered, and both the pemphigus vulgaris and exfoliative esophagitis improved. Upon findings of exfoliative esophagitis by endoscopic examination, we should consider the coexistence of blistering skin diseases, including pemphigus vulgaris.
Minami, Hitomi; Inoue, Haruhiro; Isomoto, Hajime; Urabe, Shigetoshi; Nakao, Kazuhiko
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.
Silverstein, B.D.; Pope, C.E. II
In the evaluation of esophageal disease, the appropriate question must be asked before the correct tests can be selected. Reflux can be demonstrated by radiologic methods, pH testing or radioisotopic techniques. Esophageal mucosal damage is best evaluated by x-ray, endoscopy, or biopsy. Chest pain is demonstrated by acid infusion or by manometry. Two algorithms are presented for the evaluation of chest pain and reflux symptoms.
Davis, Benjamin P.; Rothenberg, Marc E.
Eosinophilic esophagitis (EoE) is a recently recognized inflammatory disease of the esophagus with clinical symptoms derived from esophageal dysfunction. The etiology of EoE is now being elucidated, and food hypersensitivity is emerging as the central cornerstone of disease pathogenesis. Herein, we present a thorough picture of the current clinical, pathologic, and molecular understanding of the disease with a focus on disease mechanisms. PMID:26925500
Stavropoulos, Stavros N; Friedel, David; Modayil, Rani; Parkman, Henry P
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.
Kinoshita, Yoshikazu; Ishimura, Norihisa; Oshima, Naoki; Ishihara, Shunji
To investigate the prevalence and the clinical characteristics of Asian patients with eosinophilic esophagitis. We conducted a systematic search of the PubMed and Web of Science databases for original studies, case series, and individual case reports of eosinophilic esophagitis in Asian countries published from January 1980 to January 2015. We found 66 and 80 articles in the PubMed and Web of Science databases, respectively; 24 duplicate articles were removed. After excluding animal studies, articles not written in English, and meeting abstracts, 25 articles containing 217 patients were selected for analysis. Sample size-weighted mean values were determined for all pooled prevalence data and clinical characteristics. The mean age of the adult patients with eosinophilic esophagitis was approximately 50 years, and 73% of these patients were male. They frequently presented with allergic diseases including bronchial asthma, allergic rhinitis, food allergy, and atopic dermatitis. Bronchial asthma was the most frequent comorbid allergic disease, occurring in 24% of patients with eosinophilic esophagitis. Dysphagia was the primary symptom reported; 44% of the patients complained of dysphagia. Although laboratory blood tests are not adequately sensitive for an accurate diagnosis of eosinophilic esophagitis, endoscopic examinations revealed abnormal findings typical of this disease, including longitudinal furrows and concentric rings, in 82% of the cases. One-third of the cases responded to proton pump inhibitor administration. The characteristics of eosinophilic esophagitis in Asian patients were similar to those reported in Western patients, indicating that this disease displays a similar pathogenesis between Western and Asian patients.
Hanks, J.B.; Fisher, S.R.; Meyers, W.C.; Christian, K.C.; Postlethwait, R.W.; Jones, R.S.
Total laryngectomy for cancer can result in dysphagia and altered esophageal motility. Manometric changes in the upper esophageal sphincter (UES), and in proximal and distal esophageal function have been reported. However, most studies have failed to take into account radiation therapy and appropriate controls. We selected ten male patients (54.3 +/- 1.9 yr) for longitudinal manometric evaluation prior to laryngectomy then at two weeks and again six months later. No patient received preoperative radiation therapy, had a previous history of esophageal surgery, or developed a postoperative wound infection or fistula. Seven of ten patients had positive nodes and received 6,000-6,600 rads postoperative radiation therapy. Preoperatively 4 of 10 patients complained of dysphagia which did not significantly change following surgery and radiation. Two of three patients who did not complain of dysphagia preoperatively and received radiation postoperatively developed dysphagia. No patient without dysphagia preoperatively who received no radiation therapy developed symptoms. Our studies show that laryngectomy causes alterations in the UES resting and peak pressures but not in the proximal or distal esophagus, or the lower esophageal sphincter. These data also imply radiation therapy may be associated with progressive alterations in motility and symptomatology. Further study regarding the effects of radiation on esophageal motility and function are urged.
Esophageal cancer (EC) is the fourth leading cause of cancer death in China. Despite a lot of advances in diagnosis and therapy, the survival rate of patients with EC is low. There is urgent need for a variety of methods and techniques to improve the survival time and alleviate the lesions of EC. Nowadays, alternative and less invasive approaches to the treatment of ECs are being identified. Here, we review several main interventional methods at different stages of EC, including endoscopic resection, stent placement, arterial infusion, photodynamic therapy, and radiofrequency ablation. This review will focus on the indications, methods, clinical outcomes, and complications of these methods, which may help guide the way forward. PMID:27904858
Carrasco, Adriana Elisabeth Aguiar Benavides; Machado, Rodrigo Strehl; Patrício, Francy Reis da Silva; Kawakami, Elisabete
Eosinophilic esophagitis is an emerging disease featured by eosinophilic esophageal infiltrate not responsive to proton pump inhibitors. To characterize histological features of children and adolescents with eosinophilic esophagitis. Cross-sectional study in a tertiary hospital. Biopsies from each esophageal third from 14 patients (median age 7 years) with eosinophilic esophagitis were evaluated. Histological features evaluated included morphometry of esophageal epithelium, esophageal density (per high power field), extracellular eosinophilic granules, eosinophilic microabscesses, surface disposition of eosinophils, epithelial desquamation, peripapillary eosinophilia, basal layer hyperplasia and papillary elongation. Several patients presented a normal esophageal macroscopy in the upper digestive endoscopy (6, 42.8%), and the most common abnormality were vertical lines (7, 50%) and whitish spots over esophageal mucosa (7, 50%). Basal layer hyperplasia was observed in 88.8%, 100% e 80% of biopsies from proximal, middle and lower esophagus, respectively (P=0.22). Esophageal density ranges from 0 to more than 50 per hpf. Extracellular eosinophilic granules (70%-100%), surface disposition of eosinophils (60%-93%), epithelial desquamation (60%-100%), peripapillary eosinophilia (70%-80%) were common, but evenly distributed among each esophageal third. Just one patient did not present eosinophils in the lower third, four in the middle third and four in the upper esophageal third. In the absence of hypereosinophilia, other histological features are present in eosinophilic esophagitis and may contribute to diagnosis. Eosinophilic infiltrate is focal, therefore multiple biopsies are needed for diagnosis.
Kao, C H; Hsieh, J F; Tsai, C S; Ho, Y J; Lee, J K
In 30 patients with syndrome X, esophageal motility was evaluated by radionuclide esophageal transit test (RETT). Esophageal motility measurements included esophageal mean transit time (MTT), residual fraction (RF), and retrograde index (RI). In comparison with 25 age- and sex-matched healthy volunteers, 66% of the patients with syndrome X had abnormal RETT findings including 50% of cases with longer MTT, 50% of cases with higher RF, and 33% of cases with higher RI. In addition, the mean values of MTT, RF, and RI in patients with syndrome X were significantly higher than those of healthy volunteers. We conclude that abnormal esophageal motility occurred in a large portion of syndrome X patients based on an simple and noninvasive RETT.
Korsten, M.A.; Rosman, A.S.; Fishbein, S.; Shlein, R.D.; Goldberg, H.E.; Biener, A. )
OBJECTIVES: To assess the effects of chronic xerostomia on parameters of gastroesophageal reflux and esophagitis. DESIGN: Observational study of a cohort of male patients with xerostomia and age-matched control subjects. SETTING: Tertiary-care Veterans Affairs Medical Center. SUBJECTS: Sixteen male patients with chronic xerostomia secondary to radiation for head and neck cancers or medications. Nineteen age-matched male control subjects with comparable alcohol and smoking histories. MEASUREMENTS AND MAIN RESULTS: Esophageal motility was similar in patients with xerostomia and controls. Clearance of acid from the esophagus and 24-hour intraesophageal pH were markedly abnormal in patients with xerostomia. Symptoms and signs of esophagitis were significantly more frequent in subjects with xerostomia. CONCLUSIONS: Chronic xerostomia may predispose to esophageal injury, at least in part, by decreasing the clearance of acid from the esophagus and altering 24-hour intraesophageal pH. Esophageal injury is a previously unreported complication of long-term salivary deficiency.