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Sample records for mediastinal cystic lymphangioma

  1. A Case of Mediastinal Cystic Lymphangioma.

    PubMed

    Suehisa, Hiroshi; Ueno, Tsuyoshi; Sawada, Shigeki; Yamashita, Motohiro; Teramoto, Norihiro

    2015-01-01

    A 35-year-old Japanese man's routine chest radiography revealed an abnormal opacity. Chest computed tomography and magnetic resonance imaging showed a 5.5 cm in dia. cystic tumor located at the left anterior mediastinum. The tumor was suspected to be an asymptomatic thymic cyst, and we chose observation for the tumor. At the 3-year follow up, the cystic tumor had gradually enlarged to 7.5 cm in dia. and we thus performed a surgical resection via left video-assisted thoracic surgery. An immunohistochemical analysis showed that the cystic tumor was not a thymic cyst but rather a mediastinal cystic lymphangioma. Mediastinal cystic lymphangiomas are very rare, and they are difficult to diagnose preoperatively. Complete surgical resection is proposed for the treatment of such tumors. PMID:26690247

  2. Cystic Lymphangioma of Pancreas.

    PubMed

    Bihari, Chhagan; Rastogi, Archana; Rajesh, S; Arora, Ankur; Arora, Asit; Kumar, Nikhil

    2016-03-01

    Lymphangiomas are benign hamartomatous malformations which can arise either from congenitally sequestered lymphatic channels or due to acquired obstruction caused by fibrosis of lymph channels. They are common in the pediatric age group in the soft tissue of neck and the axilla. Abdominal lymphangiomas are rare; even rarer is the primary involvement of pancreas. It occurs more frequently in females and is often located in the distal pancreas. The authors report the case of cystic lymphangioma of pancreas in a 26-year old female presenting with recurrent episodes of upper abdominal pain that was treated with laparoscopic cyst excision. Although exceptionally rare, lymphangioma of the pancreas should be considered in the differential diagnosis of pancreatic cystic lesions, especially in young women. PMID:27065694

  3. Mesenteric cystic lymphangioma mimicking malignancy.

    PubMed

    Hureibi, Khalid; Sunidar, Osama A

    2014-01-01

    Mesenteric cystic lymphangiomas are benign tumours arising from the mesentery, and have no known aetiology. Patients might be discovered incidentally to have asymptomatic mesenteric cysts, or they can present with symptoms such as pain, nausea and vomiting. A 27-year-old man presented with vague abdominal pain, loss of appetite, postprandial fullness and significant weight loss. There was no lymphadenopathy, and abdominal examination was unremarkable. CT showed a mesenteric mass and a diagnosis of abdominal lymphoma was suggested. There was no evidence of pulmonary tuberculosis on chest X-ray and the purified protein derivative test was negative. On laparotomy, a 5×9×7 cm sessile cyst containing thick white fluid and arising from the ileal mesentery was found and completely removed. Histopathology proved a diagnosis of mesenteric cystic lymphangioma. The patient made uneventful recovery, and was asymptomatic on clinical follow-up after 6 weeks. PMID:25178885

  4. Mesenteric cystic lymphangioma mimicking malignancy

    PubMed Central

    Hureibi, Khalid; Sunidar, Osama A

    2014-01-01

    Mesenteric cystic lymphangiomas are benign tumours arising from the mesentery, and have no known aetiology. Patients might be discovered incidentally to have asymptomatic mesenteric cysts, or they can present with symptoms such as pain, nausea and vomiting. A 27-year-old man presented with vague abdominal pain, loss of appetite, postprandial fullness and significant weight loss. There was no lymphadenopathy, and abdominal examination was unremarkable. CT showed a mesenteric mass and a diagnosis of abdominal lymphoma was suggested. There was no evidence of pulmonary tuberculosis on chest X-ray and the purified protein derivative test was negative. On laparotomy, a 5×9×7 cm sessile cyst containing thick white fluid and arising from the ileal mesentery was found and completely removed. Histopathology proved a diagnosis of mesenteric cystic lymphangioma. The patient made uneventful recovery, and was asymptomatic on clinical follow-up after 6 weeks. PMID:25178885

  5. Cardiac lymphangioma presenting as intrapericardial cystic mass

    PubMed Central

    Lone, Nazir A.; Naikoo, Bashir A.; Khan, Naseer A.

    2016-01-01

    Cystic lymphangioma usually confined to head and neck is a well-recognized tumor that occurs during childhood. However, a cardiac lymphangioma is exceptionally uncommon and a particularly rare form of disease. We report a case of cystic lymphangioma arising from the right ventricular wall, and presenting as pericardial mass in a young female, who presented with a history of exercise intolerance in the form of breathlessness on exertion and palpitations. The management of such a case was a difficult task; however, she underwent near total resection of the mass, and is doing well for the last 2 years. PMID:26739981

  6. Bilateral Cystic Lymphangioma of Ovary Associated with Chylous Ascites

    PubMed Central

    Arakeri, Surekha Ulhas; Patil, Vijaya L.; Mulay, Himanshu Dilip

    2015-01-01

    Intraabdominal cystic lymphangiomas are rare and are located in retroperitoneum, mesentery, omentum and other visceral organs. Lymphangiomas of the ovary are rare and are usually unilateral. Cases with bilateral cystic lymphangiomas of the ovary are reported very rarely in literature. We report a rare case of bilateral cystic lymphangioma of ovary associated with chylous ascites in a 35-year-old lady who presented with complaints of severe dysmenorrhoea and oligomenorrhoea since 6 months with history of chyluria for the past 3 years. PMID:26435959

  7. Rare Case of Ovarian Cystic Lymphangioma Managed at Laparoscopy.

    PubMed

    Naik, Sejal Arunbhai

    2011-01-01

    Lymphangiomas are rare, usually benign tumors of the lymphatic system. Lymphangiomas may arise in any part of the body. Lymphangioma of the ovary is very rare tumor, which is usually slow-growing, remains asymptomatic for a long time, and identified incidentally at histopathological examination after excision. It is advisable to excise the lesion with microscopically clear margins. Many times, diagnosis becomes difficult because of confusion with malignant ovarian mass, especially in post-menopausal woman. A 72-year-old post-menopausal woman had a symptomatic pelvic mass. Imaging studies demonstrated a complex right ovarian cyst. Laparoscopic total hysterectomy with bilateral salpingo-oophorectomy was performed successfully. Cystic Lymphangioma should be included in the differential diagnosis of an ovarian cystic mass, and laparoscopic excision may be the method of treatment. PMID:26085753

  8. Rare Case of Ovarian Cystic Lymphangioma Managed at Laparoscopy

    PubMed Central

    Naik, Sejal Arunbhai

    2011-01-01

    Lymphangiomas are rare, usually benign tumors of the lymphatic system. Lymphangiomas may arise in any part of the body. Lymphangioma of the ovary is very rare tumor, which is usually slow-growing, remains asymptomatic for a long time, and identified incidentally at histopathological examination after excision. It is advisable to excise the lesion with microscopically clear margins. Many times, diagnosis becomes difficult because of confusion with malignant ovarian mass, especially in post-menopausal woman. A 72-year-old post-menopausal woman had a symptomatic pelvic mass. Imaging studies demonstrated a complex right ovarian cyst. Laparoscopic total hysterectomy with bilateral salpingo-oophorectomy was performed successfully. Cystic Lymphangioma should be included in the differential diagnosis of an ovarian cystic mass, and laparoscopic excision may be the method of treatment. PMID:26085753

  9. Infected retroperitoneal cystic lymphangioma masquerading as psoas abscess.

    PubMed

    Pratap, Akshay; Tiwari, Awadhesh; Sah, Birendra Prasad; Sinha, Arvind Kumar; Shakya, Vikal Chandra; Niels, Koopmans G

    2008-01-01

    A 2-year-old male is described who presented with fever, fixed flexion deformity of the right hip and a tender mass in the right iliac fossa. A clinical diagnosis of psoas abscess was made. Abdominal ultrasound was suggestive of an echogenic retroperitoneal cyst but could not accurately determine the nature of the cyst. A contrast CT scan showed a retroperitoneal cyst. At laparotomy, a large retroperitoneal cyst containing turbid fluid was found and was excised completely. Histopathological examination was suggestive of cystic lymphangioma. Clinical presentation of retroperitoneal lymphangioma may be variable and misleading, therefore complex imaging studies are necessary in the evaluation of this condition. PMID:18480641

  10. Multiple cystic lymphangiomas of the spleen: radiologic and histopathologic findings.

    PubMed

    Kim, Se-Young; Kwon, Heon-Ju; Park, Hae Won; Lee, So-Yeon; Son, Byung Ho; Kim, Mi Sung

    2015-07-01

    Splenic cyst(s) may be noted as an incidental finding on conventional imaging techniques, or as a result of evaluation of a patient with left upper quadrant pain, left shoulder pain, abdominal enlargement, or splenomegaly. Lymphangioma of the spleen is an extremely rare and benign neoplasm in adults, which is characterized by cystic dilatation of the lymphatic vessels in splenic parenchyma. This report describes a case of multiple splenic lymphangiomas in a 41-year-old female. She underwent abdominal ultrasonography for a health check-up. She had no symptoms, and physical examination did not reveal any abnormalities apart from splenomegaly. Ultrasonography and computed tomography showed multiple variable-sized cysts replacing the normal parenchyma of the spleen. There were wall calcifications in several cysts. The patient underwent laparoscopic splenectomy, and the final diagnosis was multiple lymphangiomas of the spleen. PMID:26576795

  11. [Cystic lymphangioma of the adrenal gland. Three misleading cases].

    PubMed

    Berthet, B; Christophe, M; Siméoni, J; Jean, F; Le Treut, Y P; Bricot, R; Assadourian, R

    1993-01-23

    Three cases of adrenal cystic lymphangioma are reported. In 1 patient the lesion was complicated by intracystic haemorrhage. The remaining 2 patients had a hepatic lesion which was treated in the same surgical operation as the adrenal cyst. Ultrasonography and computerized tomography play a major role in the exploration of these cysts. Their unexpected discovery has become more frequent since these methods have multiplied, and this raises therapeutic problems. The nature of adrenal cysts is determined at histology. In asymptomatic cysts percutaneous needle aspiration can only have an indicative value. PMID:8493206

  12. Percutaneous Sclerotherapy With OK-432 of a Cervicomediastinal Lymphangioma.

    PubMed

    Golinelli, Gloria; Toso, Andrea; Borello, Giovanni; Aluffi, Paolo; Pia, Francesco

    2015-11-01

    The present study reports a case of percutaneous sclerotherapy of a giant cystic cervicomediastinal lymphangioma using OK-432. To the best of our knowledge, percutaneous sclerotherapy of a mediastinal lymphangioma using OK 432 has not previously been reported in the English literature. PMID:26522530

  13. OK432 (picibanil) efficacy in an adult with cystic cervical lymphangioma. A case report.

    PubMed

    Alonso, Juan; Barbier, Luis; Alvarez, Julio; Romo, Laura; Martín, Jesús C; Arteagoitia, Iciar; Santamaría, Joseba

    2005-01-01

    Cervical cystic lymphangioma (CCL) is a rare and benign tumour involving congenital and cystic abnormalities derived from lymphatic vessels. The most accepted treatment continues to be surgical excision. However, when this infiltrates vital neurovascular neck structures, complete excision is difficult and if only partial, the recurrence rate is very high. The most frequently used alternative treatment is to inject sclerosants into the lesion. The use of these techniques has reported good results in children; however, there are few references thereof with regard to adults. We are reporting on a cervical cystic lymphangioma in a male aged 22, treated with an intra-lesion injection of 20 cc with 0.01 mg/cc dilution of OK-432 (picibanil) in physiological serum. Sole complications were fever and local reaction where the solution was injected. One month after treatment the lymphangioma had totally remitted and sixteen months later continues in remittance. PMID:16056191

  14. An Interesting Association of Cystic Hygroma of the Neck and Lymphangioma Causing a Paediatric Swollen Tongue

    PubMed Central

    Beech, A. N.; Farrier, J. N.

    2016-01-01

    Up to 75% of lymphatic malformations occur in the head and neck region. Of these, cystic hygromas and lymphangiomas have been widely reported; however they rarely occur in the same patient. We report the case of a 5-year-old girl who presented to the Department of Paediatrics of a district general hospital with a short history of recurrent, painful swelling of the anterior one-third of her tongue. She was reviewed under the joint care of the Oral and Maxillofacial Surgery and Otolaryngology Teams. Relevant past medical history included a previously excised cystic hygroma from her right neck when she was aged 2 years. Diagnosis of lymphangioma was made and of the potential management options available active monitoring was favoured due to the patient's age. To our knowledge the occurrence of both tongue lymphangioma and cystic hygroma has not been previously reported in a paediatric patient. This case report therefore shows a rare association between a cystic hygroma of the neck and lymphangioma of the tongue. PMID:27069707

  15. [The diagnosis and treatment of one huge cystic lymphangioma in etropharyngeal space].

    PubMed

    Zhang, Hua; Song, Xicheng; Jia, Chuanliang

    2016-01-01

    One child was hospitalized because of repeated cough and sputum. The biopsy diagnosis in local hospital was cystic lymphangioma in retropharyngeal space. We carried out transoral incision and drainage by catheter under general anesthesia. Put into the surgical cavity a suction drainage tube, and injected 5 mg dexamethasone and 8mg Bleomycin. He had nasogastric liquid diet after operation. We removed the suction drainage tube two weeks later. No recurrence was found following up over two years. PMID:27192920

  16. [Spontaneous rupture of mediastinal cystic teratoma (case report)].

    PubMed

    Ege, Gürkan; Akman, Haluk; Kuzucu, Kismet; Kalayci, Göksel

    2004-06-01

    Teratomas are rare tumors in the mediastinum. Benign cystic teratomas of anterior mediastinum are rarely complicated by rupture into an adjacent body cavity. Such rupture, however, is usually associated with life-threatening complications. We present a case with spontaneous rupture of mediastinal cystic teratoma. The patient was evaluated with chest radiograph, computed tomography (CT) and magnetic resonance imaging (MRI). A complex mass including predominantly cystic components was detected in the left anterior mediastinum. After surgery, pathologic diagnosis was reported as mature cystic teratoma. High levels of amylase and lipase were detected in both the cystic fluid and serum. This finding supported the hypothesis of autolysis for the explanation of rupture. In addition, carbohydrate antigen (CA) 19-9, CA 125 and carcinoembryonic antigen (CEA) levels were high in the cystic fluid. PMID:15236127

  17. Mediastinal Tuberculosis in an Adult Patient with Cystic Fibrosis▿

    PubMed Central

    Morand, Philippe C.; Burgel, Pierre-Régis; Carlotti, Agnès; Desmazes-Dufeu, Nadine; Farhi, David; Martin, Clémence; Kanaan, Reem; Mangialavori, Luigi; Palangié, Estelle; Dusser, Daniel; Poyart, Claire; Hubert, Dominique

    2011-01-01

    Tuberculosis (TB) is rarely observed in cystic fibrosis (CF) patients. We report the first case of mediastinal TB, associated with leg pain and skin rash, in an adult patient with CF, and discuss factors suggestive of TB in the course of CF. PMID:21106788

  18. Peripancreatic cystic lymphangioma diagnosed by endoscopic ultrasound/fine-needle aspiration: a rare mesenchymal tumour

    PubMed Central

    Tanimu, Sabo; Rafiullah; Resnick, Jeffrey; Onitilo, Adedayo A

    2013-01-01

    A 73-year-old man presented with a 5-month history of intermittent nausea, vomiting, central abdominal discomfort and a 17-pound weight loss over the past year. Laboratory testing, including a complete blood count with differential, liver function testing, amylase and lipase studies were normal. A CT scan showed a bilobed cystic lesion inferior to the body of the pancreas. An endoscopic ultrasound revealed a 5.3×3.9 cm, anechoic, bilobed cystic lesion, extrinsic to the body of the pancreas with a 1–2 mm septation and a normal pancreas. Fine-needle aspiration revealed a milky-white aspirate with negative cytology. Laboratory assessment of the cystic aspirant revealed carcinoembryonic antigen 1.7 ng/mL, amylase 148 units/L, cholesterol 300 mg/dL, and carbohydrate antigen 19-9 3 units/mL. He underwent resection of the mass, with the histopathology confirming a diagnosis of peripancreatic lymphangioma. He did well after the surgery with interval resolution of his symptoms. PMID:24092605

  19. Retroperitoneal lymphangioma: A report of 2 cases and a review of the literature regarding the differential diagnoses of retroperitoneal cystic masses

    PubMed Central

    DI MARCO, MARIACRISTINA; GRASSI, ELISA; VECCHIARELLI, SILVIA; DURANTE, SANDRA; MACCHINI, MARINA; BIASCO, GUIDO

    2016-01-01

    Cystic lymphangioma is a type of benign tumor originating from the lymph vessels. The tumor commonly occurs in childhood, in the head or neck regions, and retroperitoneal localization and presentations in adulthood are rare. Determining a pre-operative diagnosis is often challenging, and in the majority of cases, a diagnosis is only possible subsequent to the histological examination of the surgical specimen. A radical resection is the recommended treatment for cystic lymphangioma, and recurrence is usually due to an incomplete excision of the mass. The present study reports 2 cases of cystic lymphangioma, localized in the pancreatic gland and duodenal wall respectively, which were treated with surgical resection. The study also briefly reviews the literature regarding the differential diagnosis of retroperitoneal cystic masses. PMID:27123082

  20. Volvulus of the jejunum on cystic lymphangioma: About a clinical case

    PubMed Central

    Coulibaly, Yacaria; Keita, Soumaila; Doumbia, Aliou; Togo, Adegne

    2016-01-01

    Intestinal volvulus on mesenteric cysticum lymphangioma (CL) is rare in children. The clinical picture is not very suggestive. We report a case of intestinal volvulus on CL in a 7-year-old girl after an abdominal trauma. Resection and anastomosis were made. The confirmation diagnosis was done by anatomopathological examination. Early diagnosis of intra-abdominal CL will allow avoiding complication. PMID:27251660

  1. Mediastinitis

    MedlinePlus

    ... heart valve surgery, or coronary artery bypass surgery. Did You Know... A child who swallows a button ... treatment for fibrosing mediastinitis. Resources In This Article Did You Know 1 Did You Know... Pleural and ...

  2. Mediastinitis

    MedlinePlus

    ... Trauma Other causes of mediastinitis include: Histoplasmosis Radiation Sarcoidosis Tuberculosis Breathing in anthrax Cancer Risk factors include: ... of breath If you have tuberculosis, histoplasmosis, or sarcoidosis and develop any of these symptoms, contact your ...

  3. Case report of solitary giant hepatic lymphangioma

    PubMed Central

    Lee, Hwan Hyo

    2016-01-01

    A hepatic lymphangioma is a rare benign neoplasm that is usually associated with systemic lymphangiomatosis. A solitary hepatic lymphangioma is extremely rare. Therefore, we present a rare case of a female patient who underwent right hepatectomy for solitary giant hepatic lymphangioma. A 42-year-old female presented to the emergency department with complaint of severe abdominal pain of the right upper quadrant. Abdominal computed tomography showed an approximately 23×30-cm sized, giant, relatively well-defined, homogenous cystic mass with few septa in the right liver (segments VII and VIII). The preoperative diagnosis was a giant hepatic cystadenoma or cystadenocarcinoma. We performed right hepatectomy. The permanent histopathological report revealed cystic lymphangioma of the liver. Although the prognosis of solitary hepatic lymphangioma after surgical resection is favorable, recurrence has been reported in literature. PMID:27212994

  4. [Bleomycin therapy for lymphangioma].

    PubMed

    Kertész, Zs; Bălă, G; Bancu, S; Gozar, H; Virgil, G; Horváth, E; Pávai, Z

    2011-01-01

    Lymphangiomas are uncommun congenital malformations of the lymphatic system, that involve the skin and subcutaneous tissues. Of the several types of treatment, surgical excision has been the preferred. There is a high recurrence rate because lymphangiomas tend to infiltrate the surrounding tissues. The bleomycin is a cytotoxic antitumoral antibiotic, that causes modifications of DNA. It has been also successfully used in intralesional injection treatment of cystic hygromas and haemangiomas, based specifically on a high sclerosing effect on vascular endothelium. We report the cases of five patients, with congenital lymphangioma, localized on the leg, in cervical and latero-thoracal region, treated with repeated intralesional bleomycin injections. The treatment indication was given by the location of this lesions and the infiltration of the surrounding vital tissues, that made the complete surgical excision impossible. Intralesional injection of bleomycin into the lymphangiomas was given at a dose, not exceeding 0,5 mg/kg of body weight, at intervals of 4 weeks. Complete resolution (n = 4) or significant improvement (n = 1) occurred in all patients treated. No other treatment was needed. We didn't notice local or general adverse effects. With this method we set the purpose to treat effectively this congenital malformations, obviating the need for invasive primary surgery or systemic treatment regimens. Toward other methods, intralesional bleomycin injections have a minimal risk of side effects (ulceration, pulmonary fibrosis). PMID:21523964

  5. Cases of Atypical Lymphangiomas in Children

    PubMed Central

    Minocha, Prashant K.; Persad, Rambachan

    2014-01-01

    Background. Lymphatic malformations or lymphangiomas are rare benign hamartomas that result from maldevelopment of primitive lymphatic sacs. They are most frequently found in the neck and axilla, while intra-abdominal and mediastinal lymphangiomas are uncommon. These are primarily tumours of infancy and childhood and are successfully treated with surgical excision. Summary of Cases. Five cases of lymphangioma comprising three intra-abdominal lymphangiomas and two unilateral axillary lymphangiomas presenting at one institution in Trinidad W.I. between 2005 and 2012 were examined. The presentations, location, workup, treatment, and outcome of these patients were studied. Conclusion. This paper discusses a range of extracervical lymphangioma cases seen at San Fernando General Hospital, Trinidad W.I. We report three intra-abdominal cases and the most common clinical presentations were abdominal pain and distension. Also two axillary cases were reported, which presented as painless axillary masses. The major concerns for excision of axillary lymphangioma by parents and surgeons were cosmesis and feasibility of complete resection without disruption of developing breast tissue and axillary vessels. We believe that ultrasound scan is very good at detection of the lesion, while CT is better at determining tumour content and planning for the operation. It is our opinion that complete surgical excision can be achieved. PMID:25328741

  6. Cases of atypical lymphangiomas in children.

    PubMed

    Minocha, Prashant K; Roop, Lakhan; Persad, Rambachan

    2014-01-01

    Background. Lymphatic malformations or lymphangiomas are rare benign hamartomas that result from maldevelopment of primitive lymphatic sacs. They are most frequently found in the neck and axilla, while intra-abdominal and mediastinal lymphangiomas are uncommon. These are primarily tumours of infancy and childhood and are successfully treated with surgical excision. Summary of Cases. Five cases of lymphangioma comprising three intra-abdominal lymphangiomas and two unilateral axillary lymphangiomas presenting at one institution in Trinidad W.I. between 2005 and 2012 were examined. The presentations, location, workup, treatment, and outcome of these patients were studied. Conclusion. This paper discusses a range of extracervical lymphangioma cases seen at San Fernando General Hospital, Trinidad W.I. We report three intra-abdominal cases and the most common clinical presentations were abdominal pain and distension. Also two axillary cases were reported, which presented as painless axillary masses. The major concerns for excision of axillary lymphangioma by parents and surgeons were cosmesis and feasibility of complete resection without disruption of developing breast tissue and axillary vessels. We believe that ultrasound scan is very good at detection of the lesion, while CT is better at determining tumour content and planning for the operation. It is our opinion that complete surgical excision can be achieved. PMID:25328741

  7. Pancreatic Lymphangioma as a Rare Pancreatic Mass: A Case Report

    PubMed Central

    Talaiezadeh, Abdolhasan; Ranjbari, Nastaran; Bakhtiari, Mohammad

    2016-01-01

    Introduction Cystic lymphangiomas of abdomen has mostly involved mesentery and retro peritoneum that should be considered as a differential diagnosis of abdominal masses. Pancreatic lymphangiomas were extremely rare that should be differentiated from neoplastic pancreatic cysts. Patients have commonly presented with epigastric pain and a relevant palpable epigastric mass. Case Presentation A 65-year-old lady who has presented with epigastric pain, then during investigations, a cystic tumor which located in the tail of pancreas, has found. Whereas definite diagnosis of tumor with routine procedures was impossible, the tumor has completely resected by distal pancreatectomy and splenectomy. Pathology and IHC was suggestive of benign lymphangioma. Conclusions According to this presentation diagnosis of cystic lymphangioma of the tail of pancreas should be considered as a differential diagnosis of pancreatic cystic lesions and complete excision has been the treatment of choice. PMID:27366308

  8. Giant facial lymphangioma.

    PubMed

    Sanger, Claire; Wong, Lindsey; Wood, Jeyhan; David, Lisa R; Argenta, Louis C

    2011-07-01

    Lymphatic malformation (LM) is a benign cystic entity resulting from aberrant lymphatic drainage. Often evident at birth, most LMs have declared themselves by 2 years of age. They can be concerning when they occur near vital structures such as the airway or orbit. The natural history varies considerable from spontaneous gradual regression to long-term growth and debilitation. Depending on the location, structures involved, and clinical course of the LM, therapeutic options include observation, intralesional sclerosis, laser therapy, and surgical excision. The literature provides guidelines for treatment options that must be carefully applied to the facial region. We present a newborn infant who presented to our institution with giant facial lymphangioma who underwent a combination of sclerosis, laser ablation, and surgery with reconstruction. PMID:21772195

  9. Adrenal Lymphangioma Masquerading as a Catecholamine Producing Tumor

    PubMed Central

    Hodish, Israel; Schmidt, Lindsay; Moraitis, Andreas G.

    2015-01-01

    Objective. To report the unusual case of an adrenal lymphangioma presenting in a patient with an adrenal cystic lesion and biochemical testing concerning for pheochromocytoma. The pertinent diagnostic and imaging features of adrenal lymphangiomas are reviewed. Methods. We describe a 59-year-old patient who presented with hyperhidrosis and a 2.2 by 2.2 cm left adrenal nodule. Biochemical evaluation revealed elevated plasma-free normetanephrine, urine normetanephrine, urine vanillylmandelic acid, and urine norepinephrine levels. Elevated plasma norepinephrine levels were not suppressed appropriately with clonidine administration. Results. Given persistent concern for pheochromocytoma, the patient underwent adrenalectomy. The final pathology was consistent with adrenal lymphangioma. Conclusions. Lymphangiomas are benign vascular lesions that can very rarely occur in the adrenal gland. Imaging findings are generally consistent with a cyst but are nonspecific. Excluding malignancy in patients presenting with adrenal cysts can be difficult. Despite its benign nature, the diagnosis of adrenal lymphangioma may ultimately require pathology. PMID:26618011

  10. Lymphangioma of the ovary: etiology and management.

    PubMed

    Radhouane, Achour; Mayada, Saadaoui; Khaled, Neji

    2016-08-01

    Lymphangioma of the ovary is an extremely rare lesion. It was first described in 1908 [1] and there is only 19 cases reports in a 50-year literature survey [2]. Lymphangiomas are benign congenital malformations of lymphatic system. They are thought to happen due to obstruction of local lymph flow system and they can occur anywhere in the skin and the mucous membranes. Most common sites are the head and the neck and sometimes they can be found in the intestines, the pancreas and the mesentery. Lymphangioma of the ovary is a very rare tumor. Typically, lymphangiomas are slow-growing tumors that remain asymptomatic for a long time, with the tumor being identified incidentally during histopathological examination after excision. A 36-year-old women who consulted our department for infertility for 2 years. The systematic ultrasound examination reveals a right ovarian mass. The histologic analysis confirmed the diagnosis of cystic lymphangioma of the right ovary. She was planned for total laparoscopic tumorectomy with removal of all ovarian mass. The evolution was good for one year within Clinical and ultrasound monitoring. We report through this case report a review of the literature. PMID:27401696

  11. A Case of Intraoral Lymphangioma Circumscripta – A Diagnostic Dilemma

    PubMed Central

    Babu, Dara Balaji Gandhi; Kumar, B. Raj; Gannepalli, Ashalata

    2015-01-01

    Lymphangiomas are developmental malformations which present as benign hamartomas of lymphatic channels. They are commonly located in the head and neck region and are less frequently reported in the oral cavity. However affected sites in the oral cavity include the tongue, palate, gingiva, buccal mucosa, lips and alveolar ridge of the mandible. The most common site in the oral cavity is tongue. Lymphangioma of the buccal mucosa is rare as per the available literature and they usually occur as lobular masses or cystic lesions. Histopathologically, lymphangioma appear as proliferated lymphatic vessels lined by plump endothelial cells. Surgical excision, laser therapy and sclerotherapy are the eventual modes of treatment. This paper reports a rare case of lymphangioma of the buccal mucosa in a 60-year old male patient managed by surgical excision. PMID:26557627

  12. Jejunal cavernous lymphangioma

    PubMed Central

    Morris-Stiff, Gareth; Falk, Gavin A; El-Hayek, Kevin; Vargo, John; Bronner, Mary; Vogt, David P

    2011-01-01

    Cavernous lymphangiomas are usually identified in infants and children with the majority of lesions found around the head and neck, trunk or extremities. Tumours affecting the intra-abdominal organs are rare. The authors report a case of small bowel cavernous lymphangioma arising within the jejunum of a 34-year-old woman presenting with dyspnoea and anaemia, and review the existing literature relating to this uncommon tumour. PMID:22696733

  13. Cavernous lymphangioma: Two case reports

    PubMed Central

    Sargunam, Cynthia; Thomas, Jayakar; Raneesha, P. K.

    2013-01-01

    Lymphangiomas are congenital malformation of the lymphatic system that involve the skin and subcutaneous tissues. We are reporting two cases of cavernous lymphangioma. These cases are presented for their rarity. PMID:23984237

  14. Adrenal lymphangioma: clinicopathologic and immunohistochemical characteristics of a rare lesion.

    PubMed

    Ellis, Carla L; Banerjee, Priya; Carney, Erin; Sharma, Rajni; Netto, George J

    2011-07-01

    Adrenal lymphangiomas, also known as cystic adrenal lymphangiomas, are rare, benign vascular lesions that usually remain asymptomatic throughout life. Although previously adrenal lymphangioma lesions were primarily found at autopsy, they are currently detected during imaging work-up for unrelated causes and are likely to imitate other adrenocortical or adrenal medullary neoplasms. We aimed to retrospectively review all adrenal lymphangioma cases at our hospital and further document their lymphatic origin by immunohistochemical staining. A search of surgical pathology records (1984-2008) was conducted. All hematoxylin and eosin sections were retrieved from archives and reviewed by 2 pathologists in the study. Clinical information was gathered from electronic medical records. Representative paraffin-embedded sections from each case were selected for immunohistochemical analysis using monoclonal antibodies D2-40 and AE1/AE3. A total of 9 adrenal lymphangioma cases were identified (6 women and 3 men). All 9 patients were adults at time of diagnosis with a mean age of 42 years (range, 28-56 years). There were 7 white patients, 1 African American patient, and 1 Asian patient. The average size of an adrenal lymphangioma lesion was 4.9 cm (range, 2.0-13.5 cm). Adrenal lymphangioma was twice more frequently located on the right side (6 right-sided and 3 left-sided). Clinically, 4 (44%) of the 9 lesions presented with abdominal, flank, or back pain. One lymphangioma was found during work-up for labile hypertension. The remaining 4 lesions (44%) were asymptomatic and incidentally found during imaging studies for unrelated causes. Surgical removal was achieved by total adrenalectomy in 8 of the 9 lesions and by partial adrenalectomy in the remaining case. No evidence of recurrence or development of a contralateral lesion was encountered in any of the patients. Histologically, our adrenal lymphangiomas showed a typical multicystic architecture with dilated spaces lined by

  15. Laparoscopic segmental colectomy for colonic lymphangiomas: A definitive, minimally invasive surgical option

    PubMed Central

    Zhuo, Chang-Hua; Shi, De-Bing; Ying, Min-Gang; Cheng, Yu-Fan; Wang, Yu-Wei; Zhang, Wen-Ming; Cai, San-Jun; Li, Xin-Xiang

    2014-01-01

    Colonic lymphangioma is an unusual benign malformation. We herein describe two cases. A 36-year-old woman was admitted with one year of intermittent abdominal pain; colonoscopy, abdominopelvic computed tomography and endoscopic ultrasonography (EUS) revealed enlarged cystic masses at the ascending colon. In another 40-year-old man, colonoscopy and EUS revealed an asymptomatic lobulated cystic mass with four small sessile polyps at the sigmoid colon. Both patients underwent laparoscopic segmental colectomy. Both masses were histologically confirmed as cystic lymphangiomas, and the patients were discharged without complications. The management of colonic lymphangioma depends on the individual situation; close surveillance or endoscopic therapy may be appropriate for asymptomatic lesions smaller than 2.5 cm in diameter. Surgical intervention can be considered for larger lesions or in patients who develop complication risks. Laparoscopic segmental colon resection may be recommended to excise relatively large submucosal lesions because it is a definitive, minimally invasive intervention with a fast postoperative recovery. PMID:25024636

  16. A Giant Retroperitoneal Lymphangioma: A Case Report

    PubMed Central

    Hubli, Prabhu; Sachin, Basavaraju M

    2016-01-01

    Lymphangioma is a congenital lesion of childhood. They usually present in head and neck region. Intra-abdominal lymphangioma in an adult is a rare lesion which poses diagnostic difficulty. Although asymptomatic, they may present with an acute abdomen. Here, we report a rare case of huge asymptomatic retro-peritoneal lymphangioma who underwent complete surgical excision with an uneventful postoperative period.

  17. Primary Mediastinal Classical Hodgkin Lymphoma.

    PubMed

    Piña-Oviedo, Sergio; Moran, Cesar A

    2016-09-01

    Primary mediastinal Classical Hodgkin lymphoma (CHL) is rare. Nodular sclerosis CHL (NS-CHL) is the most common subtype involving the anterior mediastinum and/or mediastinal lymph nodes. Primary thymic CHL is exceedingly rare. The disease typically affects young women and is asymptomatic in 30% to 50% of patients. Common symptoms include fatigue, chest pain, dyspnea and cough, but vary depending on the location and size of the tumor. B-symptoms develop in 30% of cases. By imaging, primary mediastinal CHL presents as mediastinal widening/mediastinal mass that does not invade adjacent organs but may compress vital structures as bulky disease. Histopathology is the gold standard for diagnosis. Primary mediastinal NS-CHL consists of nodules of polymorphous inflammatory cells surrounded by broad fibrous bands extending from a thickened lymph node capsule. The cellular nodules contain variable numbers of large Hodgkin/Reed-Sternberg cells, required for diagnosis. Primary thymic CHL may exhibit prominent cystic changes. The histopathologic recognition of NS-CHL can be challenging in cases with prominent fibrosis, scant cellularity, artifactual cell distortion, or an exuberant granulomatous reaction. The differential diagnosis includes primary mediastinal non-HLs, mediastinal germ cell tumors, thymoma, and metastatic carcinoma or melanoma to the mediastinum. Distinction from primary mediastinal non-HLs is crucial for adequate therapeutic decisions. Approximately 95% of patients with primary mediastinal CHL will be alive and free of disease at 10 years after treatment with short courses of combined chemoradiotherapy. In this review, we discuss the history, classification, epidemiology, clinicoradiologic features, histopathology, immunohistochemistry, differential diagnosis, and treatment of primary mediastinal CHL. PMID:27441757

  18. Multiple mediastinal hydatic cyst: a case report.

    PubMed

    Mohammadi, Afshin; Khodabakhsh, Maryam

    2011-01-01

    Hydatid disease is a parasitic infection that is remains endemic in many countries, particularly the Middle East and Central Asia. Mediastinal hydatidosis is very rare (less than 0.1% of all hydatid disease cases) that have been only anecdotally in the literature. To the best of our knowledge only one case of multiple mediastineal hydatid cysts has been reported previously. We report the second cases of multiple mediastineal hydatid cysts and recommend that it can be considered in the differential diagnosis of multiple cystic mediastineal masses in endemic countries. PMID:21554233

  19. Subserous lymphangioma of the sigmoid colon: an uncommon cause of acute abdomen in pediatric patients

    PubMed Central

    Fernandes, Bianca Furlan; Moraes, Érika Neves de Souza; de Oliveira, Francini Rossetto; Felipe-Silva, Aloísio; Ferreira, Cristiane Rúbia; de Alcântara, Paulo Sérgio Martins; Tokeshi, Flavio; Martinês, João Augusto dos Santos; Ferronato, Ângela Espósito

    2015-01-01

    Lymphangioma is a rare, benign lesion derived from a malformation of the lymphatic system, which is more frequently found in the head, neck, and axilla. However, it may be present anywhere in the body, and the diagnosis involves adults as children with some distinct clinical features among them. In pediatric patients, abdominal cystic lymphangioma occurs mostly in the mesentery presenting abdominal pain, intestinal obstruction, or, more rarely, hemorrhage. The authors report the case of a child with a short-course history of fever, abdominal pain, and constipation. The physical examination disclosed the presence of an abdominal mass and signs of peritoneal irritation. Imaging was consistent with a cystic lesion compressing the sigmoid colon and laterally displacing the remaining loops. Exploratory laparotomy was undertaken, and a sigmoidectomy, followed by Hartman’s colostomy, was performed. Histological examination revealed the nature of the lesion as a cystic lymphangioma. The authors highlight the clinical features of this entity and call attention to this disease in the differential diagnosis of acute abdomen or abdominal pain, mainly in pediatric patients. PMID:26894047

  20. Subserous lymphangioma of the sigmoid colon: an uncommon cause of acute abdomen in pediatric patients.

    PubMed

    Fernandes, Bianca Furlan; Moraes, Érika Neves de Souza; de Oliveira, Francini Rossetto; Benevides, Gabriel Núncio; Felipe-Silva, Aloísio; Ferreira, Cristiane Rúbia; de Alcântara, Paulo Sérgio Martins; Tokeshi, Flavio; Martinês, João Augusto Dos Santos; Ferronato, Ângela Espósito

    2015-01-01

    Lymphangioma is a rare, benign lesion derived from a malformation of the lymphatic system, which is more frequently found in the head, neck, and axilla. However, it may be present anywhere in the body, and the diagnosis involves adults as children with some distinct clinical features among them. In pediatric patients, abdominal cystic lymphangioma occurs mostly in the mesentery presenting abdominal pain, intestinal obstruction, or, more rarely, hemorrhage. The authors report the case of a child with a short-course history of fever, abdominal pain, and constipation. The physical examination disclosed the presence of an abdominal mass and signs of peritoneal irritation. Imaging was consistent with a cystic lesion compressing the sigmoid colon and laterally displacing the remaining loops. Exploratory laparotomy was undertaken, and a sigmoidectomy, followed by Hartman's colostomy, was performed. Histological examination revealed the nature of the lesion as a cystic lymphangioma. The authors highlight the clinical features of this entity and call attention to this disease in the differential diagnosis of acute abdomen or abdominal pain, mainly in pediatric patients. PMID:26894047

  1. Mediastinal tumor

    MedlinePlus

    ... contains the heart, large blood vessels, windpipe (trachea), thymus gland, esophagus, and connective tissues. The mediastinum is divided into three sections: Anterior (front) Middle Posterior (back) Mediastinal ... Cancer Browse the Encyclopedia A.D.A.M., ...

  2. Oral lymphangioma: A rare case report

    PubMed Central

    Bhayya, Harsha; Pavani, D.; Avinash Tejasvi, M. L.; Geetha, P.

    2015-01-01

    Lymphangiomas are benign hamartomatous tumors of the lymphatic channels which present as developmental malformations arising from sequestration of lymphatic tissue that do not communicate with the rest of the lymphatic channels. Lymphatic vessels are filled with a clear protein-rich fluid containing few lymph cells. It can also occur in association with hemangioma. The onset of lymphangiomas are either at birth (60% to 70%) or up to two years of age (90%) and rare in adults. Lymphangiomas have marked predilection for the head and neck region (50-70%). The most common location in the mouth is the dorsum of tongue, followed by lips, buccal mucosa, soft palate, and floor of the mouth. On tongue, they may present as a localized or a diffused growth which may enlarge to cause macroglossia, impaired speech and difficulty in mastication. Herewith, we present a rare case of lymphangioma of tongue leading to macroglossia in a 8-year-old boy. PMID:26681873

  3. Thoracic Cavernous Lymphangioma Provoking Massive Chyloptysis

    PubMed Central

    Ferguson, Robert; Hodges, Jeffrey; Harness-Brumley, Cayce; Girod, Carlos; Bartolome, Sonja

    2013-01-01

    Chyloptysis is a relatively rare embodiment of disease that encompasses a lengthy differential and provides many diagnostic and therapeutic challenges. Presented here is the case of a young woman with massive chyloptysis due to a thoracic cavernous lymphangioma arising in the peripartum period. The severity of her condition mandated the use of cardiopulmonary bypass to resect her lymphangioma. We believe that the extent of her symptoms, etiology of disease, and surgical management represent a unique scenario in the literature. PMID:26425583

  4. [A case of lymphangioma of penis].

    PubMed

    Takeyama, M; Kou, E; Kondoh, N; Doi, Y; Fujioka, H; Tokizane, M

    1989-03-01

    A 9-year-old boy was seen with chief complaint of a penile mass with pain. The movable mass was palpable in the subcutaneous tissue of the penis apart from the median raphe. The mass was excised enbloc. Histological examination revealed that the mass was a lymphangioma of penis consisting of several endothelial-lined cysts. Convalescense was uneventful. Eighteen cases of lymphangioma or lymphangiectasia of penis including the present case in the Japanese literature are reviewed. PMID:2660518

  5. [Mediastinal leiomyosarcoma].

    PubMed

    Auliac, J B; Cuvelier, A; Peillon, C; Louvel, J P; Metayer, J; Muir, J F

    1999-04-01

    Leiomyosarcomas are extremely rare tumors which develop from smooth muscle, usually in the esophagus and large vessels (inferior vena cava, pulmonary artery, and superior vena cava). In very rare cases, leiomyosarcomas develop from small vessels in the soft tissue of the mediastinum. Clinical expression of mediastinal leiomyosarcomas (dysphagia, dysphonia) is related to their large size and the subsequent compression of mediastinal structures. At pathology examination, the gross aspect is one of a single cell tumor. Microscopically, the tumor may be highly undifferentiated making it necessary to use specific immune markers (actin and desmin) or ultra-structural analysis to establish the diagnosis. Treatment of localized tumors is based on surgical excision, either alone or in combination with radiotherapy of the mediastinum. Chemotherapy, generally dexorubicin, is indicated in case of metastatic dissemination, but outcome remains uncertain. As for all soft tissue sarcomas, the prognosis of mediastinal leiomyosarcoma depends on the size of the tumor, its histological structure and its resectability. PMID:10339766

  6. Mediastinal hydatid cyst: a case report

    PubMed Central

    2013-01-01

    Introduction Mediastinal localization of hydatidosis is very rare even in endemic areas. The diagnosis is based on typical clinical and radiological criteria. Case presentation We report a case of a mediastinal location of hydatidosis in a 60-year-old Arab man admitted for chest pain. The chest radiograph showed a rounded and homogeneous opacity. Computed tomography showed a right mediastinal cyst, without other thoracic or abdominal sites. Through a posterolateral thoracotomy, we found a cystic mass in the posterior mediastinum. The patient received a cystectomy with medical treatment based on albendazole. He improved a few weeks later. Conclusion Mediastinal cysts remain rare, even in endemic countries, which makes initial diagnosis difficult. Our observation shows the importance of keeping this diagnosis in mind when a patient presents with signs of mediastinal compression. PMID:24099329

  7. [Mediastinal echinococcosis].

    PubMed

    Biriukov, Iu V; Islambekov, E S; Streliaeva, A V; Sagieva, A T; Sadykov, V M; Sakykov, R V; Rafikov, A A; Khaĭdarov, G A

    2002-01-01

    Among 29,875 autopsies 59 cases of mediastinal echinococcosis were revealed. Among 4178 patients with thoracic echinococcosis 55 patients had mediastinal echinococcosis. All the patients were operated, most of them underwent ideal ecinococcectomy. Intraoperative prophylaxis of echinococcosis was performed: plearal cavity was treated by low-frequency ultrasound and glycerin. 7 examined patients demonstrated reduction of immune and phagocytosis indices in blood (DC3+, CD4+, CD8+, CD16+, CD21+). Increase of immunoglobulines A, M, G and circulating immune complexes was revealed. Reactions of scolexprecipitation and lymphocytes antigen-fixing were positive. PMID:11875945

  8. [Minimally Invasive Thoracoscopic Surgery for Mediastinal Lesions].

    PubMed

    Maeda, Sumiko

    2016-07-01

    This review article describes minimally invasive thoracoscopic surgery for anterior mediastinal lesions. The operative procedures for anterior mediastinal lesions have been changed in a couple of decades from open surgery under median sternotomy to complete thoracoscopic mediastinal surgery with sternal lifting or carbon dioxide insufflation. Carbon dioxide insufflation of the thoracic cavity or the mediastinum is now prevailing to improve the surgical field and facilitate the operative procedures. Surgical indications for complete thoracoscopic mediastinal surgery include benign cystic lesions generally regardless of their size and non-invasive anterior mediastinal tumors usually less than 50~60 mm in the greatest dimension. There are currently three surgical approaches in the complete thoracoscopic surgery for the anterior mediastinal lesions. One is the unilateral or bilateral transthoracic approach. The second is the combination of the subxiphoid and the transthoracic approach. The last is the subxiphoid approach. The selection of the surgical approach depends on the surgeon's preference and experiences. When carbon dioxide insufflation is applied during the operation, following complications may occur;hypercapnia, gas embolism, subcutaneous emphysema, endotracheal tube dislocation due to the mediastinal sift, and hypotention. Special safety considerations are necessary during the complete thoracoscopic mediastinal surgery with carbon dioxide insufflation. PMID:27440034

  9. Mediastinal tracheostomy.

    PubMed

    Gomes, M N; Kroll, S; Spear, S L

    1987-05-01

    Upper airway obstruction in primary or recurrent carcinomas of the head and neck extending into the mediastinum may demand surgical intervention despite severe technical difficulties in patients with tumors previously considered inoperable. In fact, many of these tumors may be operable and some perhaps curable. A technique has been developed based in part on our experience with previously described procedures. A preliminary sternal split is used to demonstrate the extent of the mediastinal involvement as well as to provide enhanced exposure and proximal control of the great vessels. The pectoralis major muscle is used with a generous flap of overlying skin comprising nearly half of the anterior portion of the chest. A tracheostomy is then created in a fashion similar to the placement of a cardiac valvular prosthesis by creating a circular defect in the pectoralis major flap and suturing it to the tracheal remnant. This technique offers a reasonably safe and reliable means of creating a low anterior mediastinal tracheostomy for tumors previously considered inoperable. The preliminary sternal split makes the procedure safer and easier to perform, and the use of a very large pectoralis major island flap allows for reliable closure of the resulting mediastinal and sternal defects. PMID:3034176

  10. Echocardiographic features of a mediastinal pancreatic pseudocyst.

    PubMed Central

    Aroney, C N; Nicholson, M R; Shevland, J E

    1985-01-01

    Cross sectional echocardiography detected a mediastinal pancreatic pseudocyst which caused extracardiac compression in a 49 year old man. Computed tomography confirmed the presence of a cystic lesion lying behind the heart and extending from the pancreas to above the carina. Surgical decompression resulted in resolution of the clinical and echocardiographic findings. Images PMID:3994874

  11. Cystic renal neoplasms and renal neoplasms associated with cystic renal diseases in adults: cross-sectional imaging findings.

    PubMed

    Katabathina, Venkata S; Garg, Deepak; Prasad, Srinivasa R; Vikram, Raghu

    2012-01-01

    Cystic renal neoplasms in adults are a heterogeneous group of tumors with characteristic histogenesis, pathological findings, and variable biological profiles. They include disparate entities that are either biologically benign (lymphangioma, cystic nephroma, and mixed epithelial and stromal tumor) or malignant (cystic renal cell carcinoma, multilocular cystic renal cell carcinoma, and primary renal synovial sarcoma). Renal cystic diseases are characterized by cystic changes of the kidneys due to hereditary, developmental, or acquired etiology. Cystic renal diseases such as acquired cystic kidney disease, von Hippel-Lindau disease, and tuberous sclerosis are associated with the development of a wide spectrum of benign and malignant renal neoplasms. Most cystic renal tumors and cystic disease-associated renal neoplasms show characteristic cross-sectional imaging findings that permit accurate diagnosis. In addition, cross-sectional imaging is pivotal in the follow-up and surveillance of adult cystic tumors of the kidney. PMID:23192202

  12. Klippel-Trenaunay Syndrome with Extensive Lymphangiomas

    PubMed Central

    Mneimneh, Sirin; Tabaja, Ali; Rajab, Mariam

    2015-01-01

    Klippel-Trenaunay syndrome (KTS) is a rare disorder characterized by the triad of vascular malformations, venous varicosities, and bone and soft-tissue hypertrophy. We present a case of Klippel-Trenaunay syndrome with limb hypertrophy, port-wine stains, lymphangiomas, and venous varicosities in the limbs. PMID:26587303

  13. [Mediastinal hemangioma (case report)].

    PubMed

    Karakaya, Olcay; Akgül, Erol; Binokay, Figen; Aikimbaev, Kairgueldy

    2004-09-01

    We present radiographic and computed tomographic findings of a mediastinal hemangioma, a very rare benign vascular tumor that comprises less than 0.5% of all mediastinal masses. Posteroanterior chest film showed a homogeneous opacity in the left upper zone that was contiguous with the mediastinum. Computed tomography demonstrated a mediastinal mass with inhomogeneous contrast enhancement and a small calcification. PMID:15470623

  14. Mediastinal mature teratoma in a child- A case report.

    PubMed

    Liew, W X; Lam, H Y; Narasimman, S; Navarasi, S; Mohd Hamzah, K

    2016-02-01

    Mediastinal teratoma is an infrequent germ cell tumour and comprises of 1 to 5% of all mediastinal tumours. We report a case of mediastinal mature teratoma in a 12 year old boy who presented to us with persistent non-productive cough, fever and dyspnoea for the past 7 months. Computed tomographic scan of thorax revealed a large anterior mediastinal mass measuring 11.2x9.9x14cm with calcification within. He subsequently underwent a median sternotomy with left subcostal extension (L-incision) and excision of tumour. Histopathology of the tumour revealed a mature cystic teratoma. We would like to report a case of successful surgical management of a large mediastinal mature teratoma in a child. PMID:27130743

  15. Classical Cases of Lymphangioma - As Multiple Vesicular Eruptions.

    PubMed

    Devi, Anju; Narwal, Anjali; Yadav, Achla Bharti; Singh, Virender; Gupta, Ambika

    2016-06-01

    Lymphangiomas are uncommon congenital hamartomas of the lymphatic system, usually diagnosed in infancy and early childhood. They are rarely situated in oral cavity and most commonly identified on the anterior two-thirds of the tongue. Though rarely seen in the oral cavity, lymphangiomas are the entities which should be taken into consideration by the clinician while examining vesicular lesions of the oral cavity. Early recognition is of utmost importance for the initiation of proper treatment and to avoid serious complications. We hereby report two classical cases of lymphangioma of the buccal mucosa with multiple vesicular eruptions, a rare site. PMID:27504428

  16. Classical Cases of Lymphangioma – As Multiple Vesicular Eruptions

    PubMed Central

    Narwal, Anjali; Yadav, Achla Bharti; Singh, Virender; Gupta, Ambika

    2016-01-01

    Lymphangiomas are uncommon congenital hamartomas of the lymphatic system, usually diagnosed in infancy and early childhood. They are rarely situated in oral cavity and most commonly identified on the anterior two-thirds of the tongue. Though rarely seen in the oral cavity, lymphangiomas are the entities which should be taken into consideration by the clinician while examining vesicular lesions of the oral cavity. Early recognition is of utmost importance for the initiation of proper treatment and to avoid serious complications. We hereby report two classical cases of lymphangioma of the buccal mucosa with multiple vesicular eruptions, a rare site. PMID:27504428

  17. Cystic hygroma

    MedlinePlus

    ... Names Lymphangioma References Richards DS. Ultrasound for pregnancy dating, growth, and the diagnosis of fetal malformations. In: ... the first to achieve this important distinction for online health information and services. Learn more about A. ...

  18. Cystic Fibrosis

    MedlinePlus

    ... and Diseases > Lung Disease Lookup > Cystic Fibrosis Cystic Fibrosis Cystic Fibrosis (CF) is an inherited disease that ... quality of life has improved. Learn About Cystic Fibrosis Cystic fibrosis is a genetic (inherited) condition that ...

  19. Wilms Tumor 1 protein is not expressed in oral lymphangiomas.

    PubMed

    Netto, Ana Carolina de Mesquita; de Oliveira, Mariana Batista; Bernardes, Vanessa Fátima; Gomes, Carolina Cavaliéri; Gomez, Ricardo Santiago

    2012-01-01

    Lymphangiomas are benign hamartomatous lesions of lymphatic vessels. Wilms Tumor 1 (WT1) is a transcription factor that is activated in some human neoplasias. WT1 protein expression is observed in endothelial cells during angiogenesis and is a useful marker to distinguish between vascular proliferations and vascular malformations. The purpose of the present study is to report a case series of oral lymphangiomas together with an immunohistochemical investigation of WT1. Seventeen cases of oral lymphangioma were retrieved and reviewed. Immunohistochemical analysis of WT1 protein was performed and pyogenic granuloma samples were used as positive controls. The male/female ratio was 1.125 and most of the lesions occurred in young subjects. While pyogenic granuloma showed positive staining for WT1, the endothelial cells lining the thin-walled dilated lymphatic vessels of lymphangiomas were negative for this protein. The findings strengthen the idea that oral lymphangioma is a vascular malformation characterized by lymphatic dilatation without significant endothelial proliferation. PMID:23338265

  20. Macroglossia Associated with Lymphangioma: Surgical Management of An Interesting Case.

    PubMed

    Nagpal, Tapan; Shah, Dixit; Manjunatha, B S; Mahajan, Amit

    2015-11-01

    Lymphangioma is considered as benign as well as a hamartomatous lesion of the lymphatic vessels that shows a marked predilection for the head and neck region. The most common site for intraoral lymphangiomas is the anterior two-thirds on the dorsal surface of tongue resulting in macroglossia. Such patients have a tendency of speech disturbances, poor oral hygiene, and bleeding from tongue following a trauma. A child with markedly enlarged tongue presents a unique challenge to the clinicians. Aesthetic, occlusal, functional and psychosocial problems may arise as a result of this condition. The indexed patient presented with an enlarged tongue. Wide excision of the lesion was done as a treatment. PMID:26672763

  1. Macroglossia Associated with Lymphangioma: Surgical Management of An Interesting Case

    PubMed Central

    Nagpal, Tapan; Shah, Dixit; Mahajan, Amit

    2015-01-01

    Lymphangioma is considered as benign as well as a hamartomatous lesion of the lymphatic vessels that shows a marked predilection for the head and neck region. The most common site for intraoral lymphangiomas is the anterior two-thirds on the dorsal surface of tongue resulting in macroglossia. Such patients have a tendency of speech disturbances, poor oral hygiene, and bleeding from tongue following a trauma. A child with markedly enlarged tongue presents a unique challenge to the clinicians. Aesthetic, occlusal, functional and psychosocial problems may arise as a result of this condition. The indexed patient presented with an enlarged tongue. Wide excision of the lesion was done as a treatment. PMID:26672763

  2. Lymphangioma circumscriptum of the vulva: Report of a rare case

    PubMed Central

    Sinha, Anuradha; Phukan, Jyoti Prakash; Jalan, Shilpa; Pal, Subrata

    2015-01-01

    Lymphangioma circumscriptum (LC) is a form of lymphangioma characterised by benign dilation of lymphatic channels, which affects the skin and subcutaneous tissues. The most common sites of LC are mucosa of mouth, tongue, groin, axilla, trunk and proximal region of extremities. Vulva is a rare site of LC. In this report, we are presenting a case of LC of vulva occurring in a 60-year-old female without any obvious reason. The patient presented with multiple genital wart-like papular lesions in the vulva. Biopsy of lesion reveals LC. She was treated with vulvectomy and showed no sign of recurrence till date. PMID:26167062

  3. Torsion of the Greater Omentum Secondary to Omental Lymphangioma in a Child: A Case Report

    PubMed Central

    Mistry, Kewal Arunkumar; Iyer, Dayashankar

    2015-01-01

    Summary Background Omental cyst and omental torsion both are uncommon but important causes of acute abdomen with a difficult clinical diagnosis due to nonspecific features. Here we report a case of an eight year old child with acute abdominal pain referred for USG and CT scan which revealed two cysts in greater omentum leading to secondary omental torsion. Case Report An eight year old male child presented to casualty with severe pain abdomen since 1 day. There was no history of vomiting or altered bowel habits. The patient was febrile with tachycardia on arrival. On examination rigidity and tenderness all over abdomen were present. Serum amylase was within normal range. USG and CECT abdomen were done subsequently. USG showed two well defined cystic lesions in lower abdomen with presence of some internal echogenic debris and calcified foci in their dependent part. There was also presence of omentum with a whirl of blood vessels seen along anterior abdominal wall leading to these lesions suggesting torsion. On colour Doppler the presence of blood flow within the whirl of vessels was seen. Mild amount of free fluid was also seen in the peritoneal cavity. On CECT abdomen the findings of omental cysts and torsion of greater omentum with free fluid in abdomen were confirmed. The cysts measured 60×55 and 65×55mm on CT. The patient was taken for an emergency laparotomy for indication of acute generalized peritonitis. Two large omental cysts were found in the pelvic cavity along with torsed greater omentum along with 150 ml of hemorrhagic fluid in peritoneal cavity. The cysts and twisted necrotic part of the greater omentum were excised at surgery. No postoperative complications were observed. Histopathologic examination was suggestive of lymphangioma of omentum. Conclusions Lymphangioma of the omentum is an not very uncommon however acute presentation with omental torsion and infarction is an unusual entity. Optimal utilization of preoperative imaging with USG, Doppler

  4. Normalization of mediastinal widening after successful treatment of mediastinal tuberculosis.

    PubMed

    Raskin, Jo; Van Bleyenbergh, Pascal

    2016-08-01

    Clinical image of an asymmetrical mediastinal widening due to tuberculosis of mediastinal lymph nodes, without evidence of pulmonary tuberculosis. Image at first presentation and after successful treatment, showing normalization of the mediastinum. PMID:27149681

  5. [Mediastinal germ cell tumors].

    PubMed

    Bremmer, F; Ströbel, P

    2016-09-01

    The mediastinum is among the most frequent anatomic region in which germ cell tumors (GCT) arise, second only to the gonads. Mediastinal GCT (mGCT) account for 16 % of all mediastinal neoplasms. Although the morphology and (according to all available data) the molecular genetics of mediastinal and gonadal GCT are identical, a number of unique aspects exist. There is a highly relevant bi-modal age distribution. In pre-pubertal children of both sexes, mGCT consist exclusively of teratomas and yolk sac tumors. The prognosis is generally favorable with modern treatment. In post-pubertal adults, virtually all patients with malignant mGCT are males; the prognosis is more guarded and depends (among other factors) on the histological GCT components and is similar to GCT in other organs. So-called somatic type malignancies (i. e. clonally related, non-germ cell neoplasias arising in a GCT) are much more frequent in mGCT than in other organs, and the association between mediastinal yolk sac tumors and hematological malignancies, such as myelodysplasias and leukemias, is unique to mediastinal tumors. The prognosis of GCT with somatic type malignancies is generally dismal. PMID:27491549

  6. Mediastinal Mature Teratoma Revealed by Empyema.

    PubMed

    Raoufi, Mohammed; Herrak, Laila; Benali, Anas; Achaachi, Leila; El Ftouh, Mustapha; Bellarbi, Salma; Tilfine, Charaf; Taouarsa, Firdaous

    2016-01-01

    Teratomas are germ cell tumors, manifested with a great variety of clinical features; the most common extragonadal site is the anterior mediastinum. In this case, we report the patient with a large mature mediastinal teratoma with several components of ectodermal and endothermal epithelium. A 24-year-old female patient presented with history of persistent chest pain and progressively aggravating dyspnea for the previous 3 months. A chest X-ray showed a large opacity of the entire left hemithorax. Transcutaneous needle aspiration revealed a purulent fluid. The tube thoracostomy was introduced and the effusion was evacuated. Some weeks later, patient was seen in emergency for persistent cough and lateral chest pain. CT scan revealed a mass of the left hemithorax. The mass showed heterogeneous density, without compressing mediastinum great vessels and left hilar structures. Lipase value was elevated in needle aspiration. The patient underwent a total resection of the mediastinum mass via a left posterolateral thoracotomy. Microscopy revealed a mature teratoma with cystic structures. The patient subsequently made a full recovery. This case provide benign mediastinal teratoma with total atelectasis of left lung and elevated lipase value in needle transcutaneous aspiration; this event is explained by pancreatic component in the cystic tumor. Total removal of the tumor is adequate treatment for this type of teratoma and the prognosis is excellent. PMID:27144046

  7. Mediastinal Mature Teratoma Revealed by Empyema

    PubMed Central

    Raoufi, Mohammed; Herrak, Laila; Benali, Anas; Achaachi, Leila; El Ftouh, Mustapha; Bellarbi, Salma; Tilfine, Charaf; Taouarsa, Firdaous

    2016-01-01

    Teratomas are germ cell tumors, manifested with a great variety of clinical features; the most common extragonadal site is the anterior mediastinum. In this case, we report the patient with a large mature mediastinal teratoma with several components of ectodermal and endothermal epithelium. A 24-year-old female patient presented with history of persistent chest pain and progressively aggravating dyspnea for the previous 3 months. A chest X-ray showed a large opacity of the entire left hemithorax. Transcutaneous needle aspiration revealed a purulent fluid. The tube thoracostomy was introduced and the effusion was evacuated. Some weeks later, patient was seen in emergency for persistent cough and lateral chest pain. CT scan revealed a mass of the left hemithorax. The mass showed heterogeneous density, without compressing mediastinum great vessels and left hilar structures. Lipase value was elevated in needle aspiration. The patient underwent a total resection of the mediastinum mass via a left posterolateral thoracotomy. Microscopy revealed a mature teratoma with cystic structures. The patient subsequently made a full recovery. This case provide benign mediastinal teratoma with total atelectasis of left lung and elevated lipase value in needle transcutaneous aspiration; this event is explained by pancreatic component in the cystic tumor. Total removal of the tumor is adequate treatment for this type of teratoma and the prognosis is excellent. PMID:27144046

  8. Ultrasonic findings of fetal axillary lymphangioma with intralesional hemorrhage.

    PubMed

    Furue, Akiko; Mochizuki, Junko; Onishi, Yoko; Kawano, Shoko; Kanai, Yuji; Kemmochi, Manabu; Tanaka, Kiyoshi; Unno, Nobuya

    2016-04-01

    We report a case of an axillary lymphangioma in a fetus delivered at 30 weeks' gestation with suspected intralesional hemorrhage based on the ultrasonic findings. In the ultrasonic examination at 15 weeks' gestation, the fetus was found to have a multilocular mass spreading from the axilla to the chest wall, which was diagnosed as an axillary lymphangioma. Chromosome analysis by amniocentesis showed a normal karyotype, and no other malformations were observed. At 29 weeks, the mass had increased in size, and color Doppler ultrasound examination revealed that the middle cerebral artery peak systolic velocity (MCA-PSV) reached 80.2 cm/s [1.86 MoM (multiples of the median)]. Intralesional bleeding was suspected because of the multiple images of hemorrhage in which sites of blood spouting in a pulsatile fashion were detected within the mass. Cordocentesis at 30 weeks revealed that fetal hemoglobin concentration was 5.1 g/dL. An emergency Cesarean section was performed. A female weighing 2810 g, including the mass, was delivered, and the blood hemoglobin level was 5.9 g/dL at birth. Blood transfusion, fine-needle aspiration of the fluid in the mass, intralesional injection of OK-432, and partial excision of the lymphangioma were performed after birth. Ultrasonic examination proved useful in the diagnosis of intralesional bleeding in this lymphangioma. PMID:26707998

  9. An Unusual Origin of Fetal Lymphangioma Filling Right Axilla.

    PubMed

    Ersoy, Ali Ozgur; Oztas, Efser; Saridogan, Erdinc; Ozler, Sibel; Danisman, Nuri

    2016-03-01

    Fetal lymphangioma is a hamartomatous congenital anomaly of the lymphatic system, which is embracing the fetal skin (sometimes mucous membranes) and the subcutaneous tissue. The general consensus is that it occurs as a result of failure in lymphatic drainage. A 36-year-old pregnant woman was referred to our perinatology clinic at 22 weeks' gestation, because of a fetal right-sided axillary mass revealed by ultrasonography. The mass measuring 5x7x7cm in three dimensions had a multilocular structure without colour Doppler flow and well-circumscribed borders. Amniocentesis revealed a normal constitutional karyotyping. Lymphangioma was considered as prediagnosis. A healthy female baby weighing 3470 grams was delivered at term. Neonatal examination and the postnatal MRI confirmed the diagnosis. The baby is still on follow-up with the medical treatment of Sirolimus an anti-proliferative drug, and the mass got smaller significantly in 8 months after delivery. PMID:27134953

  10. An Unusual Origin of Fetal Lymphangioma Filling Right Axilla

    PubMed Central

    Oztas, Efser; Saridogan, Erdinc; Ozler, Sibel; Danisman, Nuri

    2016-01-01

    Fetal lymphangioma is a hamartomatous congenital anomaly of the lymphatic system, which is embracing the fetal skin (sometimes mucous membranes) and the subcutaneous tissue. The general consensus is that it occurs as a result of failure in lymphatic drainage. A 36-year-old pregnant woman was referred to our perinatology clinic at 22 weeks’ gestation, because of a fetal right-sided axillary mass revealed by ultrasonography. The mass measuring 5x7x7cm in three dimensions had a multilocular structure without colour Doppler flow and well-circumscribed borders. Amniocentesis revealed a normal constitutional karyotyping. Lymphangioma was considered as prediagnosis. A healthy female baby weighing 3470 grams was delivered at term. Neonatal examination and the postnatal MRI confirmed the diagnosis. The baby is still on follow-up with the medical treatment of Sirolimus an anti-proliferative drug, and the mass got smaller significantly in 8 months after delivery. PMID:27134953

  11. Mediastinal dysgerminoma complicating pregnancy

    PubMed Central

    Manikandan, K; Veena, P; Elamurugan, S; Soundararaghavan, S

    2012-01-01

    Malignancy complicating pregnancy represents one of the most challenging clinical situations. Lack of evidence and the presence of the dependent fetus contribute to the management dilemma. A 26-year-old primigravida presented at 23 weeks of gestation with a bulging substernal mass. Fine-needle aspiration was reported as mediastinal dysgerminoma. She was treated with weekly bleomycin and three weekly cisplatin and etoposide (BEP). Maternal neutropenia after 11 weeks of bleomycin required colony stimulator factor. Fetal growth restriction necessitated delivery at 31 weeks. Significant clinical and radiological tumour regression was noted after chemotherapy. Postnatally mother received external beam radiotherapy but the disease worsened two weeks after the completion of radiotherapy. Mediastinal dysgerminoma differs from the ovarian counterpart and therefore therapeutic success reports on ovarian germ cell tumours complicating pregnancy cannot be extrapolated. The safety of the BEP regimen for the fetus is yet to be established.

  12. [Mediastinal tumors: introduction].

    PubMed

    Trousse, D; Avaro, J-P

    2010-02-01

    Mediastinal tumors are relatively uncommon, usually incidentally discovered on a chest X-ray in asymptomatic patients. Young adults are particularly concerned. Mediastinal masses represent a group of heterogeneous histological type cell. A definite diagnosis is essential leading to an adequate prompt therapeutic strategy when either benign disease or aggressive malignant tumor is conceivable. Indeed the therapeutic management of such tumors could be strictly medical, requiring exclusive surgical approach or includes a multimodal treatment. Clinical examination and imaging are important tools in the diagnostic approach. However the specific diagnosis could be complex and requires histological confirmation by an experienced pathologist after examination of large biopsies of the tumor. Several investigations, including surgical invasive exploration, should be quickly requested in order to achieve a final diagnosis and refer patients in an adequate therapeutic scheme without delay. The aim of this article is to point out the available diagnostic tools in mediastinal masses, including surgical approach, and to identify the role of surgical resection in specific subtypes. PMID:20207291

  13. Lymphangioma of cheek region–an unusual presentation

    PubMed Central

    Gupta, Siddharth; Ahuja, Puneet; Rehani, Usha; Singh, Vishal

    2012-01-01

    Background and Objectives Lymphangiomas are benign hamartomatous tumors of the lymphatic vessels. A lymphatic malformation is a congenital defect that occurs during early embryonic development when the lymphatic vessels do not properly form. The vessels may become blocked and enlarged as lymphatic fluid collects in the vessels, forming a mass or a cyst. About 50% of all lesions are noted at birth and around 90% develop by 2 years of age. Materials and Methods We report the case of lymphangioma, with an unusual presentation in the cheek region resulting into vestibular obliteration and expanding to the left maxilla diagnosed on the basis of histopathology. The review of literature concerning the clinical and histological features as well as the proper management concerning this hamartomatous entity is included. Results and Conclusion The various treatment modalities for lymphangioma are surgical excision, radiation therapy cryotherapy, electrocautery, sclerotherapy, steroid administration, embolization, ligation, laser surgery with Nd-YAG and CO2 and radiofrequency tissue ablation technique. An early diagnosis and intervention helps in reducing the functional, psychological disturbances and cosmetic disfigurement. PMID:25756019

  14. Robotic partial cystectomy for lymphangioma of the urinary bladder in an adult woman

    PubMed Central

    Seyam, Raouf; Alzahrani, Hassan M.; Alkhudair, Waleed K.; Dababo, Mohammed Anas; Al-Otaibi, Mohammed F.

    2012-01-01

    Lymphangioma of the urinary bladder is a very rare tumour in adulthood. Robotic partial cystectomy is evolving for treatment of a limited number of bladder tumours. We describe a case of an adult woman with a bladder dome lymphangioma for which robotic partial cystectomy was carried out. PMID:22396382

  15. Three cases of mediastinal pancreatic pseudocysts.

    PubMed

    Matsusue, Eiji; Fujihara, Yoshio; Maeda, Kazunori; Okamoto, Masaru; Yanagitani, Atsushi; Tanaka, Kiwamu; Nakamura, Kazuhiko; Ogawa, Toshihide

    2016-06-01

    A rare complication of acute or chronic pancreatitis is the formation of a mediastinal pancreatic pseudocyst (MPP), which is caused by tracking of pancreatic fluids through anatomical openings of the diaphragm into the mediastinum. Herein, we report the imaging characteristics of three cases of this condition. Our results revealed three features in common: (i) the connection between the mediastinum and the pancreatic cystic lesion; (ii) the presence of pleural effusions; and (iii) imaging findings consistent with chronic pancreatitis, such as pancreatic atrophy and calcifications and dilatation and/or stricture of main pancreatic duct (MPD). Serial diameter changes of the MPD and of the adjacent pseudocysts were necessary for the determination of the therapeutic strategy used in each case. PMID:27330827

  16. Three cases of mediastinal pancreatic pseudocysts

    PubMed Central

    Fujihara, Yoshio; Maeda, Kazunori; Okamoto, Masaru; Yanagitani, Atsushi; Tanaka, Kiwamu; Nakamura, Kazuhiko; Ogawa, Toshihide

    2016-01-01

    A rare complication of acute or chronic pancreatitis is the formation of a mediastinal pancreatic pseudocyst (MPP), which is caused by tracking of pancreatic fluids through anatomical openings of the diaphragm into the mediastinum. Herein, we report the imaging characteristics of three cases of this condition. Our results revealed three features in common: (i) the connection between the mediastinum and the pancreatic cystic lesion; (ii) the presence of pleural effusions; and (iii) imaging findings consistent with chronic pancreatitis, such as pancreatic atrophy and calcifications and dilatation and/or stricture of main pancreatic duct (MPD). Serial diameter changes of the MPD and of the adjacent pseudocysts were necessary for the determination of the therapeutic strategy used in each case. PMID:27330827

  17. Multiple mediastinal hydatid cysts: a case report.

    PubMed

    Rahimi-Rad, Mohammad Hossein; Mahmodlou, Rahim

    2009-01-01

    Hydatid cyst (HC) in mediastinum is very rare. To the best of our knowledge, a case with multiple HCs in mediastinum is not reported already. We herein report a case of multiple HCs of the mediastinum and liver in a - 50 year-old woman presented with chest pain, cough and dyspnea for about two years. She had been treated for tuberculosis for 20 years. Chest CT scan showed multiple cysts in posterior mediastinum and one cyst in left lobe of liver. Via right posterolateral thoracotomy, multiple cysts were excised in mediastinum. And then, hepatic left lobe cyst was removed trans-diaphragmatically. Histopathologic examination confirmed HCs. Despite its rarity, primary HCs should be considered in the differential diagnosis of mediastinal multiple cystic lesions in endemic regions. PMID:20067057

  18. Minimally invasive mediastinal surgery

    PubMed Central

    Melfi, Franca M. A.; Mussi, Alfredo

    2016-01-01

    In the past, mediastinal surgery was associated with the necessity of a maximum exposure, which was accomplished through various approaches. In the early 1990s, many surgical fields, including thoracic surgery, observed the development of minimally invasive techniques. These included video-assisted thoracic surgery (VATS), which confers clear advantages over an open approach, such as less trauma, short hospital stay, increased cosmetic results and preservation of lung function. However, VATS is associated with several disadvantages. For this reason, it is not routinely performed for resection of mediastinal mass lesions, especially those located in the anterior mediastinum, a tiny and remote space that contains vital structures at risk of injury. Robotic systems can overcome the limits of VATS, offering three-dimensional (3D) vision and wristed instrumentations, and are being increasingly used. With regards to thymectomy for myasthenia gravis (MG), unilateral and bilateral VATS approaches have demonstrated good long-term neurologic results with low complication rates. Nevertheless, some authors still advocate the necessity of maximum exposure, especially when considering the distribution of normal and ectopic thymic tissue. In recent studies, the robotic approach has shown to provide similar neurological outcomes when compared to transsternal and VATS approaches, and is associated with a low morbidity. Importantly, through a unilateral robotic technique, it is possible to dissect and remove at least the same amount of mediastinal fat tissue. Preliminary results on early-stage thymomatous disease indicated that minimally invasive approaches are safe and feasible, with a low rate of pleural recurrence, underlining the necessity of a “no-touch” technique. However, especially for thymomatous disease characterized by an indolent nature, further studies with long follow-up period are necessary in order to assess oncologic and neurologic results through minimally

  19. Minimally invasive mediastinal surgery.

    PubMed

    Melfi, Franca M A; Fanucchi, Olivia; Mussi, Alfredo

    2016-01-01

    In the past, mediastinal surgery was associated with the necessity of a maximum exposure, which was accomplished through various approaches. In the early 1990s, many surgical fields, including thoracic surgery, observed the development of minimally invasive techniques. These included video-assisted thoracic surgery (VATS), which confers clear advantages over an open approach, such as less trauma, short hospital stay, increased cosmetic results and preservation of lung function. However, VATS is associated with several disadvantages. For this reason, it is not routinely performed for resection of mediastinal mass lesions, especially those located in the anterior mediastinum, a tiny and remote space that contains vital structures at risk of injury. Robotic systems can overcome the limits of VATS, offering three-dimensional (3D) vision and wristed instrumentations, and are being increasingly used. With regards to thymectomy for myasthenia gravis (MG), unilateral and bilateral VATS approaches have demonstrated good long-term neurologic results with low complication rates. Nevertheless, some authors still advocate the necessity of maximum exposure, especially when considering the distribution of normal and ectopic thymic tissue. In recent studies, the robotic approach has shown to provide similar neurological outcomes when compared to transsternal and VATS approaches, and is associated with a low morbidity. Importantly, through a unilateral robotic technique, it is possible to dissect and remove at least the same amount of mediastinal fat tissue. Preliminary results on early-stage thymomatous disease indicated that minimally invasive approaches are safe and feasible, with a low rate of pleural recurrence, underlining the necessity of a "no-touch" technique. However, especially for thymomatous disease characterized by an indolent nature, further studies with long follow-up period are necessary in order to assess oncologic and neurologic results through minimally invasive

  20. [Spontaneous mediastinal emphysema].

    PubMed

    Svedbrand, Charlotte; Lange, Peter; Nielsen, Klaus

    2016-01-01

    Spontaneous mediastinal emphysema, also known as spontaneous pneumomediastinum, is defined as radiologically detected free air in the mediastinum, without preceding trauma. It is a rare condition, mainly affecting young adults. It can be caused by coughing, strenuous sports or cocaine inhalation, however, 40% are idiopatic. Common symptoms are chest pain and dyspnoea. 75-90% can be diagnosed with a chest X-ray, and 100% with a computed tomography. Treatment is symptomatic and complications are rare, however, pneumothorax and pneumorrachis have been reported. PMID:26750190

  1. [OK-432 therapy for cervicofacial lymphangioma in adults].

    PubMed

    Peral Cagigal, Beatriz; Serrat Soto, Alberto; Calero, Hermógenes; Verrier Hernández, Alberto

    2007-05-01

    The treatment of cervicofacial lymphangiomas has been changing in recent years. OK-432 appears to be a relatively safe and effective treatment option for patients of all ages. An intracystic injection of OK-432 produces a local inflammatory reaction, leading to resolution of the lesion. Cosmetically, the outcome is excellent, leaving no lesions on the skin at the injection site, unlike other sclerosing agents. It may thus be considered as the treatment of choice, especially in cases where surgical treatment is associated with the possibility of serious functional or cosmetic side effects. PMID:17498476

  2. The 'Pinocchio' nasal deformity due to cavernous lymphangioma.

    PubMed Central

    Hobby, J L; Tiernan, E; Mayou, B J

    1995-01-01

    The 'Pinocchio' or 'Cyrano' nose is a rare condition in which deformity of the nasal tip is produced by an underlying soft tissue tumour. Previously reported cases have been due to either capillary or cavernous haemangiomas (angiolipomas). The deformity is the cause of much teasing in children. There has been debate as to whether surgical intervention is indicated, as a proportion of cases will regress spontaneously. We report a case of 'Pinocchio' nose with a lymphangioma of the nasal tip which is previously undescribed and review the options for management. Images Figure 1 Figure 2 PMID:7562857

  3. Minimally Invasive Management of Complex Recurrent Lymphangioma of the Thorax and Abdomen.

    PubMed

    Knight, Joshua K; Marshall, M Blair

    2016-06-01

    Lymphangioma is a rare benign proliferation of lymph vessels in the head and neck, axilla, and rarely the mediastinum. We report on the minimally invasive management of a complex recurrence of lymphangioma involving bilateral chest, mediastinum, and abdomen. Given the complexity, the patient underwent debulking of the lymphangioma, ligation of the cisterna chyli, and repeated percutaneous drainage and sclerosing procedures. This report suggests that the use of combined minimally invasive techniques including laparoscopy, thoracoscopy, percutaneous drainage, and sclerosis may be the optimal strategy to manage these complex situations. PMID:27211980

  4. Acute mediastinitis arising from pancreatic mediastinal fistula in recurrent pancreatitis

    PubMed Central

    Choe, In Soo; Kim, Yong Seok; Lee, Tae Hee; Kim, Sun Moon; Song, Kyung Ho; Koo, Hoon Sup; Park, Jung Ho; Pyo, Jin Sil; Kim, Ji Yeong; Choi, In Seok

    2014-01-01

    Acute mediastinitis is a fatal disease that usually originates from esophageal perforation and surgical infection. Rare cases of descending necrotizing mediastinitis can occur following oral cavity and pharynx infection or can be a complication of pancreatitis. The most common thoracic complications of pancreatic disease are reactive pleural effusion and pneumonia, while rare complications include thoracic conditions, such as pancreaticopleural fistula with massive pleural effusion or hemothorax and extension of pseudocyst into the mediastinum. There have been no reports of acute mediastinitis originating from pancreatitis in South Korea. In this report, we present the case of a 50-year-old female suffering from acute mediastinitis with pleural effusion arising from recurrent pancreatitis that improved after surgical intervention. PMID:25356062

  5. Transcervical extended mediastinal lymphadenectomy.

    PubMed

    Zielinski, Marcin; Kuzdzal, Jaroslaw; Nabialek, Tomasz; Hauer, Lukasz; Pankowski, Juliusz; Dziadzio, Bogdan

    2006-01-01

    Transcervical extended mediastinal lymphadenectomy (TEMLA) is a new procedure for bilateral excision of all nodal stations of the mediastinum, except for the pulmonary ligament nodes (station 9) and the most distal left lower paratracheal nodes (station 4L). The procedure is performed through a transverse 5-8 cm incision in the neck with elevation of the sternum with a traction device facilitating the access to the mediastinum. Most of the procedure is performed with an open technique, while the removal of the subcarinal (station 7) and periesophageal nodes (station 8) is performed with the mediastinoscopy assisted technique and excision of the paraaortic nodes (station 6), the aorta-pulmonary window nodes (station 5) and, sometimes, the prevascular nodes (station 3A) is performed with the aid of a videothoracoscope introduced to the mediastinum through the neck incision, without violating the pleura. PMID:24413333

  6. Cystic Fibrosis

    MedlinePlus

    ... for the Public » Health Topics » Cystic Fibrosis Explore Cystic Fibrosis What Is... Other Names Causes Who Is at Risk Signs & Symptoms Diagnosis Treatments Living With Clinical Trials Links Related Topics Bronchiectasis ...

  7. Cystic fibrosis

    MedlinePlus

    ... page: //medlineplus.gov/ency/article/000107.htm Cystic fibrosis To use the sharing features on this page, please enable JavaScript. Cystic fibrosis is a disease that causes thick, sticky mucus ...

  8. Endoscopic ultrasound in mediastinal tuberculosis

    PubMed Central

    Sharma, Malay; Ecka, Ruth Shifa; Somasundaram, Aravindh; Shoukat, Abid; Kirnake, Vijendra

    2016-01-01

    Background: Tubercular lymphadenitis is the commonest extra pulmonary manifestation in cervical and mediastinal locations. Normal characteristics of lymph nodes (LN) have been described on ultrasonography as well as by Endoscopic Ultrasound. Many ultrasonic features have been described for evaluation of mediastinal lymph nodes. The inter and intraobserver agreement of the endosonographic features have not been uniformly established. Methods and Results: A total of 266 patients underwent endoscopic ultrasound guided fine needle aspiration and 134 cases were diagnosed as mediastinal tuberculosis. The endoscopic ultrasound location and features of these lymph nodes are described. Conclusion: Our series demonstrates the utility of endoscopic ultrasound guided fine needle aspiration as the investigation of choice for diagnosis of mediastinal tuberculosis and also describes various endoscopic ultrasound features of such nodes. PMID:27051097

  9. Mediastinal fibrosis presenting as asthma.

    PubMed

    Chantaphakul, H; Rock, M J; Steiner, D S; Gern, J E

    1998-01-01

    Asthma is one of the most common chronic medical conditions affecting children. The usual presenting symptoms of asthma include wheezing, shortness of breath, and dyspnea on exertion. Occasionally, children who present with one of these respiratory complaints have a less common disorder. Mediastinal fibrosis is a rare and incurable condition in which an excessive fibrotic reaction in the mediastinum causes progressive cardiopulmonary compromise. The presentation is variable: many patients present with respiratory symptoms such as cough, wheezing, dyspnea, and/or hemoptysis, while others are asymptomatic and present with a mediastinal mass discovered incidentally on a radiograph. With such a broad array of presenting complaints, and a clinical course characterized by slow progression of symptoms, the early stages of mediastinal fibrosis can mimic other diseases such as asthma, chronic bronchitis, or the superior vena cava syndrome. In this report we describe two patients with mediastinal fibrosis who were initially thought to have asthma. PMID:9642434

  10. Lymphangioma of the tongue - a case report and review of literature.

    PubMed

    V, Usha; Sivasankari, T; Jeelani, S; Asokan, G S; Parthiban, J

    2014-09-01

    Lymphangiomas are benign tumours resulting from a congenital malformation of the lymphatic system. They are relatively uncommon and usually diagnosed in infancy and early childhood. Commonly located at head and neck, they rarely occur in the oral cavity. Intraoral lymphangiomas occur more frequently on the dorsum of tongue, followed by palate, buccal mucosa, gingiva, and lips. Lymphangioma of the tongue is a common cause of macroglossia in children associated with difficulty in swallowing and mastication, speech disturbances, airway obstruction, mandibular prognathism, openbite and other possible deformities of maxillofacial structures. We present the case of a 13-year-old female with lymphangioma of tongue. The clinical, radiological, and treatment modalities of this case are discussed. PMID:25386537

  11. Cystic Lesions of the Mediastinum.

    PubMed

    Vargas, Daniel; Suby-Long, Thomas; Restrepo, Carlos S

    2016-06-01

    Cystic lesions are commonly seen in the mediastinum, and they may arise from virtually any organ. The vast majority of these lesions are benign and result in no symptoms. When large, cysts may produce symptoms related to compression of adjacent structures. The most common mediastinal cysts are pericardial and foregut duplication cysts. Both computed tomography and magnetic resonance are routinely used to evaluate these lesions. Although computed tomography offers superior spatial resolution, magnetic resonance is useful in differentiating cysts that contain proteinaceous material from solid lesions. Occasionally, cysts arise from solid lesions, such as thymoma or teratoma. Although cysts are alike in appearance, location helps narrowing the differential diagnoses. PMID:27261346

  12. [Surgical treatment of acute mediastinitis].

    PubMed

    Krüger, M; Decker, S; Schneider, J P; Haverich, A; Schega, O

    2016-06-01

    Despite modern intensive care management, acute mediastinitis is still associated with a high morbidity and mortality (up to approximately 40 %). Effective antibiotic therapy, intensive care management, elimination of the causative sources of infection and drainage of the affected mediastinal compartments are the cornerstones of therapy in a multidisciplinary treatment concept. Early diagnosis, prompt and uncompromising initial therapy and planned computed tomography (CT) control after the first stages of therapy in order to decide on the necessity for surgical re-interventions are essential for achieving optimal results. Knowledge of the specific anatomical characteristics is crucial for the understanding of this disease and its treatment; therefore, the current knowledge on fascial layers and interstitial spaces from the neck to the mediastinum is described and discussed. A possible foudroyant spread of the infection, especially within the posterior mediastinum, has to be anticipated. The approach to the mediastinum depends on the mediastinal compartments affected, on the causative disease and on the patient's clinical situation. The surgical approach should be adapted to the particular clinical situation of the individual patient and to the surgical experience of the surgeon. When in doubt, the more invasive approach to the mediastinum, such as bilateral thoracotomy, is recommended. An ascending mediastinitis due to pancreatitis is a very rare condition; however, as chest pains are often the main clinical sign surgeons should be aware of this differential diagnosis. An intraoperative brown-black serous fluid in the mediastinal tissue is virtually pathognomonic. The treatment results of esophageal perforation as the most frequent cause of mediastinitis have been improved by integration of various interventional procedures. Hyperbaric oxygen therapy or immunoglobulin treatment can play an auxiliary role in selected patients with acute mediastinitis. PMID

  13. Pulmonary Hypertension Complicating Fibrosing Mediastinitis

    PubMed Central

    Seferian, Andrei; Steriade, Alexandru; Jaïs, Xavier; Planché, Olivier; Savale, Laurent; Parent, Florence; Amar, David; Jovan, Roland; Fadel, Elie; Sitbon, Olivier; Simonneau, Gérald; Humbert, Marc; Montani, David

    2015-01-01

    Abstract Fibrosing mediastinitis is caused by a proliferation of fibrous tissue in the mediastinum with encasement of mediastinal viscera and compression of mediastinal bronchovascular structures. Pulmonary hypertension (PH) is a severe complication of fibrosing mediastinitis caused by extrinsic compression of the pulmonary arteries and/or veins. We have conducted a retrospective observational study reviewing clinical, functional, hemodynamic, radiological characteristics, and outcome of 27 consecutive cases of PH associated with fibrosing mediastinitis diagnosed between 2003 and 2014 at the French Referral Centre for PH. Fourteen men and 13 women with a median age of 60 years (range 18–84) had PH confirmed on right heart catheterization. The causes of fibrosing mediastinitis were sarcoidosis (n = 13), tuberculosis-infection confirmed or suspected (n = 9), mediastinal irradiation (n = 2), and idiopathic (n = 3). Sixteen patients (59%) were in NYHA functional class III and IV. Right heart catheterization confirmed moderate to severe PH with a median mean pulmonary artery pressure of 42 mm Hg (range 27–90) and a median cardiac index of 2.8 L/min/m2 (range 1.6–4.3). Precapillary PH was found in 22 patients, postcapillary PH in 2, and combined postcapillary and precapillary PH in 3. Severe extrinsic compression of pulmonary arteries (>60% reduction in diameter) was evidenced in 2, 8, and 12 patients at the main, lobar, or segmental levels, respectively. Fourteen patients had at least one severe pulmonary venous compression with associated pleural effusion in 6 of them. PAH therapy was initiated in 7 patients and corticosteroid therapy (0.5–1 mg/kg/day) was initiated in 3 patients with sarcoidosis, with 9 other being already on low-dose corticosteroids. At 1-year follow-up, 3 patients had died and among the 21 patients evaluated, 3 deteriorated, 14 were stable, and only 4 patients with sarcoidosis improved (4 receiving corticosteroids and 1

  14. Pleural effusion presenting as mediastinal widening

    PubMed Central

    Mohapatra, Prasanta R.; Garg, Kranti; Prashanth, Chikkahonnaiah; Lahoria, Rupali

    2013-01-01

    We report a case of middle-aged female presenting with mediastinal widening on chest radiograph owing to pleural effusion. The pleural effusion presenting as mediastinal widening on chest radiograph is rarely reported. PMID:24339499

  15. Acquired vulvar lymphangioma circumscriptum after cervical cancer treatment: Case report.

    PubMed

    Valente, Kari; Montgomery, Kathleen; Schultenover, Stephen; Desouki, Mohamed Mokhtar

    2016-04-01

    Vulvar lymphangioma circumscriptum (LC) is a rare entity which may present as a painful, warty lesion. In contrast to the congenital form, which occurs in children, the acquired form arises in older adults and may be associated with infection, Crohn's disease, or prior pelvic/regional surgery. We present a case of acquired LC of the vulva in a 55-year-old woman who presented with a 3-4 year history of vulvar pain following chemotherapy, radiation, and brachytherapy for cervical cancer. Vulvar shave biopsies followed by excision revealed a thickened dermis with epidermal hyperkeratosis, parakeratosis, elongated rete ridges and dilated lymphatic channels containing eosinophilic material and scattered thrombi. The differential diagnosis for this unusual lesion includes more common conditions such as condyloma acuminatum, fungating squamous cell carcinoma and molluscum contagiosum. It is important to recognize the clinical presentation as well as the distinct histological appearance of this rare benign entity. PMID:27331134

  16. Unilateral Obstructive Emphysema in Infancy due to Mediastinal Bronchogenic Cyst-Diagnostic Challenge and Management.

    PubMed

    Vimala, Leena Robinson; Sathya, Ravi Kishore Barla Sri; Lionel, Arul Premanand; Kishore, Jeenapalli Srinivasa; Navamani, Kirubakaran

    2015-05-01

    Bronchogenic cysts are the most common cystic mediastinal lesion in children. Bronchogenic cyst causing unilateral obstructive emphysema is a rare presentation. We report the case of a one and half month old infant who presented with respiratory distress which was initially suspected as left pneumothorax on frontal chest radiograph but was later found to be due to hyperinflated left lung and hence the possibility of congenital lobar emphysema was considered. CT thorax and limited MRI sections revealed a cystic lesion in mediastinum causing obstructive emphysema and mediastinal displacement. He underwent an emergency thoracotomy and excision of the cyst via an extrapleural approach. Post operatively, rapid improvement of the infant was noticed both clinically as well as radiologically. Cross sectional imaging like CT or MR is required for reaching the correct and early diagnosis in paediatric patients with respiratory distress, when there is diagnostic dilemma based on chest radiograph. PMID:26155530

  17. [Cystic lymphangioma of the transverse mesocolon simulating neoplasm of the pancreatic tail].

    PubMed

    Zago, A; Tosi, D; Portuese, D; Rosa, G

    1997-01-01

    Mesenteric cysts are uncommon lesions interesting surgeons above all for frequently difficulties in the preoperatory diagnosis. The role of radiology is to demonstrate the nature of these abdominal masses, but only with surgery we can establish a definitive histologic diagnosis. The authors report a case of a lynphangioma of the transverso mesocolon, miming a pancreatic neoplasm. PMID:9235871

  18. Cystic fibrosis

    MedlinePlus

    ... or three times each week. Swimming, jogging, and cycling are good options. Clearing or bringing up mucus ... cannot be prevented. Screening those with a family history of the disease may detect the cystic fibrosis ...

  19. Pericardial Injury from Mediastinal Irradiation

    PubMed Central

    Kumar, P.P.

    1980-01-01

    The absence of radiation induced cardiac damage during the orthovoltage era, during which period much lower doses of radiation were delivered for mediastinal malignancies due to severe skin reactions, was misinterpreted as cardiac radioresistance. However, with the advent of supervoltage x-rays with skin sparing effect, much higher doses of irradiation have been given for mediastinal malignancies. This has resulted in higher doses of radiation to the heart resulting in various types of radiation induced cardiac damage. The most common site of damage is to the pericardium, resulting in pericardial effusion. The radiographic evidence of radiation induced pericardial effusion starts one to six months prior to signs and symptoms due to it. Most of the asymptomatic radiation induced pericardial effusions resolve spontaneously. The factors which appear to play a role in the development of radiation induced pericardial effusion are discussed. ImagesFigure 1Figure 2 PMID:7392078

  20. Cystic fibrosis - resources

    MedlinePlus

    Resources - cystic fibrosis ... The following organizations are good resources for information on cystic fibrosis : Cystic Fibrosis Foundation -- www.cff.org March of Dimes -- www.marchofdimes.org/baby/cystic-fibrosis-and- ...

  1. Primary cystic peritoneal masses and mimickers: spectrum of diseases with pathologic correlation.

    PubMed

    Arraiza, María; Metser, Ur; Vajpeyi, Rajkumar; Khalili, Korosh; Hanbidge, Anthony; Kennedy, Erin; Ghai, Sangeet

    2015-04-01

    Cystic lesions within the peritoneum have been classified classically according to their lining on histology into four categories-endothelial, epithelial, mesothelial, and others (germ cell tumors, sex cord gonadal stromal tumors, cystic mesenchymal tumors, fibrous wall tumors, and infectious cystic peritoneal lesions). In this article, we will proceed to classify cystic peritoneal lesions focusing on the degree of radiological complexity into three categories-simple cystic, mildly complex, and cystic with solid component lesions. Many intra-abdominal collections within the peritoneal cavity such as abscess, seroma, biloma, urinoma, or lymphocele may mimic primary peritoneal cystic masses and need to be differentiated. Clinical history and imaging features may help differentiate intra-abdominal collections from primary peritoneal masses. Lymphangiomas are benign multilocular cystic masses that can virtually occur in any location within the abdomen and insinuate between structures. Ultrasound may help differentiate enteric duplication cysts from other mesenteric and omental cysts in the abdomen. Double-layered wall along the mesenteric side of bowel may suggest its diagnosis in the proper clinical setting. Characteristic imaging features of hydatid cysts are internal daughter cysts, floating membranes and matrix, peripheral calcifications, and collagenous pericyst. Non-pancreatic psuedocysts usually have a fibrotic thick wall and chylous content may lead to a fat-fluid level. Pseudomyxoma peritonei appears as loculated fluid collections in the peritoneal cavity, omentum, and mesentery and may scallop visceral surfaces. Many of the primary cystic peritoneal masses have specific imaging features which can help in accurate diagnosis and management of these entities. Knowledge of the imaging spectrum of cystic peritoneal masses is necessary to distinguish from other potential cystic abdominal mimicker masses. PMID:25269999

  2. Cystic Fibrosis

    MedlinePlus

    Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs. CF causes your mucus to be thick and sticky. The mucus clogs the lungs, causing breathing problems ...

  3. Mediastinal lymph node size in lung cancer.

    PubMed

    Libshitz, H I; McKenna, R J

    1984-10-01

    Using a size criterion of 1 cm or greater as evidence for abnormality, the size of mediastinal lymph nodes identified by computed tomography (CT) was a poor predictor of mediastinal lymph node metastases in a series of 86 patients who had surgery for bronchogenic carcinoma. The surgery included full nodal sampling in all patients. Of the 86 patients, 36 had nodes greater than or equal to 1 cm identified by CT. Of the 21 patients with mediastinal metastases proven at surgery, 14 had nodes greater than or equal to 1 cm (sensitivity = 67%). Of the 65 patients without mediastinal metastases, 22 had nodes greater than or equal to 1 cm. Specificity was 66% (43/65). Obstructive pneumonia and/or pulmonary collapse distal to the cancer was present in 39 patients (45%). Of these, 21 had mediastinal nodes greater than or equal to 1 cm; 10 harbored metastases and 11 did not. Obstructive pneumonia and/or pulmonary collapse is a common occurrence in bronchogenic carcinoma, but mediastinal nodes greater than or equal to 1 cm in this circumstance cannot be presumed to represent metastatic disease. Metastatic mediastinal lymph node involvement was related to nodal size also in patients with evidence of prior granulomatous disease and in patients with no putative benign cause for nodes greater than or equal to 1 cm. In both of these groups, metastatic nodal disease was found in only 25% of nodes greater than or equal to 1 cm. PMID:6332469

  4. Mediastinal Staging in Non-Small Cell Lung Cancer.

    PubMed

    Gamliel, Ziv

    2016-07-01

    In the absence of distant metastases, lung cancer treatment is determined by the results of mediastinal lymph node staging. Occult mediastinal lymph node metastases can be missed by radiologic and needle-based staging methods. Aggressive staging of mediastinal lymph nodes improves staging accuracy. Improved accuracy of mediastinal lymph node staging results in more appropriate lung cancer treatment. Improved accuracy of mediastinal lymph node staging can improve stage-specific survival from lung cancer. PMID:27261911

  5. Mediastinal impalement with a fibreglass sheet

    PubMed Central

    Wimalachandra, Welege Samantha Buddhika; Asmat, Atasha

    2014-01-01

    Mediastinal impalement injuries are uncommon and often fatal. There have been very few reported cases of survival following mediastinal impalement. Patients who present with these injuries always undergo operative intervention regardless of their underlying haemodynamic status or associated injuries. We herein present a case of mediastinal impalement injury, where a sheet of fibreglass had fractured the manubrium and entered thffige anterior mediastinum with no associated great vessel injury. The fibreglass sheet was removed via a partial sternotomy and the patient made an uneventful postoperative recovery. PMID:25273944

  6. Cystic Fibrosis Research

    MedlinePlus

    ... turn Javascript on. Feature: Steady Advances Against Cystic Fibrosis Cystic Fibrosis Research Past Issues / Fall 2012 Table of Contents "Remarkable strides in cystic fibrosis research over the past two decades have culminated ...

  7. Enlarging mediastinal/hilar lymphadenopathy with calcification.

    PubMed

    Adachi, Takashi; Nakahata, Masashi; Moritani, Suzuko; Iida, Hiroatsu; Ogawa, Kenji

    2016-02-01

    A 77-year-old man was referred to our hospital due to enlarging mediastinal/hilar lymphadenopathy with calcification. Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) and bone marrow aspiration were performed. Subsequently, monoclonal gammopathy of undetermined significance (MGUS) associated with mediastinal amyloidosis was diagnosed. We hereby report a case in which EBUS-TBNA led to a successful diagnosis of amyloidosis. PMID:26862422

  8. Mediastinitis and Bronchial Perforations Due to Mucormycosis.

    PubMed

    Dhooria, Sahajal; Agarwal, Ritesh; Chakrabarti, Arunaloke

    2015-10-01

    Diabetes mellitus is the most common predisposing condition for mucormycosis, which is emerging as an important invasive fungal infection worldwide. Isolated mediastinitis is a very rare presentation of mucormycosis. A 57-year-old woman with uncontrolled type 2 diabetes mellitus and ketoacidosis presented with septic shock and was subsequently found to have mucor mediastinitis with multiple bronchial perforations. The organism was identified as Rhizopus oryzae with the help of DNA sequencing. PMID:26348693

  9. Descending necrotizing mediastinitis associated with Lactobacillus plantarum

    PubMed Central

    2013-01-01

    Background Descending necrotizing mediastinitis (DNM), a severe infection with a high fatality rate, develops in mediastinal spaces due mainly to deep cervical abscesses. The majority of causative microbes of DNM are Streptococci and oral anaerobes. DNM associated with Lactobacillus-infection is rather rare. Case presentation A 69-year-old male with an unremarkable past medical history was referred to our hospital for surgical resection of advanced laryngeal cancer. Full examination revealed a neck abscess and DNM with a background of untreated diabetes mellitus. Initially, he was treated with meropenem. However, Lactobacillus plantarum was isolated from surgical drainage of a mediastinal abscess. Despite using antibiotics capable of eradicating all isolates with susceptibilities not differing significantly from those of the neck and mediastinal abscesses, we attributed DNM to the L. plantarum detected only in the mediastinal abscess. After DNM treatment, he underwent total pharyngolaryngectomy with bilateral neck dissection followed by reconstruction using free jejunum. He was discharged fully recovered. Conclusion We concluded that L. plantarum as the sole cause of the mediastinal abscess in the present case cannot be ruled out. As the number of immunocompromised patients increases, we should be cautious regarding this “familiar” microbe. PMID:23987907

  10. Videothoracoscopy in the treatment of mediastinal cysts

    PubMed Central

    Brzeziński, Daniel; Kozak, Józef

    2014-01-01

    Introduction Progress in the development of surgical techniques has led to the growing use of video-assisted thoracoscopic surgery (VATS) techniques in mediastinal cyst treatment. Aim To present our experience of treating mediastinal cysts with the minimally invasive technique. Material and methods Fifty patients with mediastinal cysts were treated from 2001 to 2011. There were 32 women and 18 men. The age of the patients ranged from 17 to 72, the mean age being 42 years. All patients underwent basic preoperative diagnostic tests of the chest: X-ray, computed tomography (CT), bronchoscopy and spirometry; 4 patients underwent endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) and 3 fine needle aspiration biopsy (FNAB) of the described lesions. Results The VATS was performed in each case. Conversion was carried out due to superior mediastinal location in 10 cases and pleural adhesions in 3 cases. The partial resection of a cyst was performed in 3 patients. One patient was treated conservatively due to heart failure. In that patient the transthoracic needle aspiration of a cyst under ultrasound guidance using alcoholisation with 76% ethanol with a good effect was performed twice. Cyst recurrence was observed in 1 case. Conclusions The surgical access depends on the location of a cyst. The VATS resection of a superior mediastinal cyst is not always feasible. Surgery of mediastinal cysts is both diagnostic and curative. PMID:25337163

  11. Mediastinal lymph node size in lung cancer

    SciTech Connect

    Libshitz, H.I.; McKenna, R.J. Jr.

    1984-10-01

    Using a size criterion of 1 cm or greater as evidence for abnormality, the size of mediastinal lymph nodes identified by computed tomography (CT) was a poor predictor of mediastinal lymph node metastases in a series of 86 patients who had surgery for bronchogenic carcinoma. The surgery included full nodal sampling in all patients. Of the 86 patients, 36 had nodes greater than or equal to 1 cm identified by CT. Of the 21 patients with mediastinal metastases proven at surgery, 14 had nodes greater than or equal to 1 cm (sensitivity = 67%). Of the 65 patients without mediastinal metastases, 22 had nodes greater than or equal to 1 cm. Obstructive pneumonia and/or pulmonary collapse distal to the cancer was present in 39 patients (45%). Obstructive pneumonia and/or pulmonary collapse is a common occurrence in bronchogenic carcinoma, but mediastinal nodes greater than or equal to 1 cm in this circumstance cannot be presumed to represent metastatic disease. Metastatic mediastinal lymph node involvement was related to nodal size also in patients with evidence of prior granulomatous disease and in patients with no putative benign cause for nodes greater than or equal to 1 cm.

  12. Pleuropulmonary blastoma in a young adult presenting as a ruptured cystic teratoma in radiology.

    PubMed Central

    Lee, Chang Hun; Kim, Keun Il; Kim, Young Dae; Lee, Min Ki; Kim, Jee Yeon; Park, Do Youn; Sol, Mee Young; Suh, Kang Suek

    2003-01-01

    Pleuropulmonary blastoma (PPB) is a rare malignant dysontogenetic neoplasm primarily affecting children and is characterized histologically by a variably mixed blastematous and sarcomatous patterns. We herein report a very exceptional adult case of PPB. A 21-yr-old male patient presented with a left chest pain of two weeks' duration. A computed tomography scan revealed a large, multicystic tumor occupying the left lower hemithorax, leading to the impression of a ruptured mediastinal cystic teratoma. A thoracotomy for resection of the tumor was performed. On histologic examination, the tumor consisted of cystic walls and associated solid lesions which showed undifferentiated blastemal tissues with focal fibrosarcomatous and rhabdoid features. Immunohistochemically the tumor cells only showed diffuse strong positivity for vimentin. The histologic findings corresponded to a type II PPB. The authors suggest that PPB, especially of type I or II, should be included in the radiologic differential diagnosis of mediastinal cystic neoplasms in a young adult. PMID:12923341

  13. [Rational antibiotic treatment of mediastinitis].

    PubMed

    Ambrosch, A

    2016-06-01

    Mediastinitis occurs as a severe complication of thoracic and cardiac surgical interventions and is the result of traumatic esophageal perforation, conducted infections or as a result of lymphogenic and hematogenic spread of specific infective pathogens. Treatment must as a rule be accompanied by antibiotics, whereby knowledge of the spectrum of pathogens depending on the pathogenesis is indispensable for successful antibiotic therapy. Polymicrobial infections with a high proportion of anaerobes are found in conducted infections of the mediastinum and after esophageal perforation. After cardiac surgery Staphylococci are the dominant pathogens and a nasal colonization with Staphylococcus aureus seems to be a predisposing risk factor. Fungi are the predominant pathogens in immunocompromised patients with consumptive underlying illnesses and can cause acute or chronic forms with granulomatous inflammation. Resistant pathogens are increasingly being found in high-risk patient cohorts, which must be considered for a calculated therapy. For calculated antibiotic therapy the administration of broad spectrum antibiotics, mostly beta-lactams alone or combined with metronidazole is the therapy of choice for both Gram-positive and Gram-negative bacteria inclusive of anaerobes. For patients at risk, additional antibiotic classes with a spectrum against methicillin-resistant Staphylococcus aureus (MRSA) or vancomycin-resistant Enterococcus (VRE) can be administered. Increasing rates of multidrug-resistant Gram-negative bacteria (e.g. Enterobacteriaceae) and non-fermenting bacteria (e.g. Pseudomonas and Acinetobacter) in individual cases necessitates the use of polymyxins (e.g. colistin), new tetracyclines (e.g. glycylglycines) and newly developed combinations of beta-lactams and beta-lactam inhibitors. For treatment of fungal infections (e.g. Candida, Aspergillus and Histoplasma) established and novel azoles, amphotericin B and echinocandins seem to be successful; however

  14. Spontaneous Retropharyngeal and Mediastinal Emphysema.

    PubMed

    Cho, Do-Yeon; Aaron, Geoffrey P; Shepard, Kimberly G

    2016-06-01

    A 14-year-old girl with no significant medical history presented at Emergency Department with sore throat and odynophagia after one episode of nonviolent coughing. She denied any respiratory distress, voice change, foreign body ingestion, retching, substance abuse, dental procedures, or trauma. She was afebrile with normal oxygen saturation and physical examination including the head and neck was unremarkable with the exception of bilateral neck crepitus without tenderness on palpation. Fiberoptic laryngoscopy revealed a patent laryngeal airway with normal vocal fold movement. Lateral neck X-ray demonstrated a linear air-column in the retropharyngeal space and computed tomography confirmed emphysema involving the retropharyngeal space and mediastinum with no evidence of fluid collection or abscess formation. Spontaneous retropharyngeal and mediastinal emphysema are clinical entities where free air is present within the confines of retropharyngeal space and mediastinum without obvious cause. It is benign and self-limited in nature and allows for conservative management. This case is presented with a review of literature. PMID:27090269

  15. [Prognosis of childhood mediastinal lymphoma].

    PubMed

    Okamura, J; Ikuno, Y; Sakata, N; Kai, T; Tasaka, H

    1990-11-01

    Between 1973 and 1989, 16 children with non-Hodgkins lymphoma (NHL) with a mediastinal mass (MM) were treated at our institution with multi-agent chemotherapy and radiotherapy. They also received central nervous system (CNS) prophylaxis including intrathecal methotrexate administration (14 cases) and cranial irradiation (7 cases). Twelve were boys and 4 girls. Median age was 11 +/- 3. One patient died of air way obstruction one day after admission. Fourteen of 15 patients entered into complete remission (CR) and one patient partial remission. Five remains in CR 7 to 175 months after diagnosis (median 76 months). Nine patients relapsed in the bone marrow (3 cases), CNS (3), testicles (1), neck lymph node (1) and bones plus kidneys (1). Of these, 7 patients died within 13 months after initial relapse. The disease free survival (DFS) and overall survival of all patients were 27% and 33%, respectively. Analysis of the prognostic factors among patients with MM+ -NHL revealed that the serum LDH level below 1,000IU/l was a good prognostic sign. Other factors such as age, stage, initial WBC count, size of MM and response of the MM to the initial treatment did not correlate with DFS. Because of its rarity and the poor treatment result, we need more aggressive treatment program by a multiinstitutional study for MM+ -NHL. PMID:2287064

  16. Spontaneous Retropharyngeal and Mediastinal Emphysema

    PubMed Central

    Cho, Do-Yeon; Aaron, Geoffrey P.; Shepard, Kimberly G.

    2016-01-01

    A 14-year-old girl with no significant medical history presented at Emergency Department with sore throat and odynophagia after one episode of nonviolent coughing. She denied any respiratory distress, voice change, foreign body ingestion, retching, substance abuse, dental procedures, or trauma. She was afebrile with normal oxygen saturation and physical examination including the head and neck was unremarkable with the exception of bilateral neck crepitus without tenderness on palpation. Fiberoptic laryngoscopy revealed a patent laryngeal airway with normal vocal fold movement. Lateral neck X-ray demonstrated a linear air-column in the retropharyngeal space and computed tomography confirmed emphysema involving the retropharyngeal space and mediastinum with no evidence of fluid collection or abscess formation. Spontaneous retropharyngeal and mediastinal emphysema are clinical entities where free air is present within the confines of retropharyngeal space and mediastinum without obvious cause. It is benign and self-limited in nature and allows for conservative management. This case is presented with a review of literature. PMID:27090269

  17. Open Reduction Internal Fixation Poststernotomy Mediastinitis

    PubMed Central

    Dionisopoulos, Tassos

    2013-01-01

    Introduction. Mediastinitis has been reported to complicate 5% of sternotomy surgery. We have adopted an open reduction and rigid internal fixation (ORIF) approach during the conventional rescue surgery in the treatment of mediastinitis. Methods. A retrospective review was performed to compare the outcomes of patients that had an ORIF to correct postoperative mediastinitis following median sternotomy. These were compared with the outcome of the patients that did not undergo ORIF. Results. In the 5-year study period, we reviewed 35 mediastinitis patient charts. Postoperatively, the ORIF patient group remained in the Intensive Care Unit (ICU) and on a ventilator for a mean of 1.5 and 0.75 days, respectively. Patients treated without ORIF spent significantly more days in the ICU (mean of 7.5 days, P < 0.05) and on a ventilator (mean of 2.15 days, P = 0.1). Furthermore, it was found that none of the patients (0%) who underwent ORIF complained of any postoperative sternal instability or pain. Preoperatively, however, these rates were as high as 72%. Conclusions. In the select patient, ORIF can be a safe option in the management of mediastinitis, which we have shown to significantly decrease morbidity and mortality by providing anatomic reduction as well as physiologic stabilization. We have shown that ORIF will improve the quality of life of the patient by minimizing abnormal sternal mobility and pain and will also decrease inpatient costs by decreasing days spent in the ICU and ventilator dependence. PMID:23970966

  18. [Clinical types of thoracic cancer. Mediastinal tumours].

    PubMed

    Lemarié, E

    2006-11-01

    Mediastinal germ cell tumours (teratomas, seminomas, and non-seminomatous malignant germ cell tumours) are a heterogeneous group of benign and malignant neoplasms. The standard treatment of mediastinal non-seminomatous malignant germ cell tumours is four cycles of chemotherapy followed by surgical resection of the residual mass. Small localized mediastinal seminomas may be treated with primary resection followed by radiotherapy. In patients with locally advanced disease, the preferred treatment is systemic chemotherapy followed by surgical resection of any residual disease. Thymomas can be locally invasive and associated with parathymic syndromes. Complete surgical excision is attempted in most cases of thymoma. Radiation therapy is usually recommended for invasive or incompletely excised tumours. Invasive thymoma is chemosensitive. PMID:17268355

  19. A modern definition of mediastinal compartments.

    PubMed

    Carter, Brett W; Tomiyama, Noriyuki; Bhora, Faiz Y; Rosado de Christenson, Melissa L; Nakajima, Jun; Boiselle, Phillip M; Detterbeck, Frank C; Marom, Edith M

    2014-09-01

    Division of the mediastinum into compartments is used to help narrow the differential diagnosis of newly detected mediastinal masses, to assist in planning biopsy and surgical procedures, and to facilitate communication among clinicians of multiple disciplines. Several traditional mediastinal division schemes exist based upon arbitrary landmarks on the lateral chest radiograph. We describe a modern, computed tomography-based mediastinal division scheme, which has been accepted by the International Thymic Malignancy Interest Group as a new standard. This clinical classification defines a prevascular (anterior), a visceral (middle), and a paravertebral (posterior) compartment, with anatomic boundaries defined clearly by computed tomography. It is our intention that this definition be used in the reporting of clinical cases and the design of prospective clinical trials. PMID:25396318

  20. Anterior mediastinal presentation of a giant angiomyolipoma.

    PubMed

    Amir, Afzal M I; Zeebregts, Clark J; Mulder, H Jan

    2004-12-01

    Angiomyolipomas are benign, solitary, noninvasive lesions that most often arise in the kidney. Extrarenal manifestations of these tumors include the skin, oropharynx, the abdominal wall, retroperitoneum, gastrointestinal tract, heart, lung, liver, uterus, penis, and spinal cord. We report a patient with a giant angiomyolipoma located in the anterior mediastinum. We believe this is the seventh reported case of mediastinal angiomyolipoma and the largest reported by size. It is the second reported lesion to arise in the anterior mediastinum. Distinction from other pulmonary or thoracic masses relies on the appreciation of the unique and characteristic histologic features of these mediastinal angiomyolipomas. We conclude that, although rare, angiomyolipoma should be considered in the differential diagnosis of a mediastinal tumor. PMID:15561061

  1. Biochemical remission by chemoradiotherapy in male mediastinal choriocarcinoma with diffuse lung metastasis: A case report

    PubMed Central

    ZHANG, JING; WANG, ZHI-JUN; YANG, BIN; WEI, YOU-YING; YANG, LING; HU, YANG; HU, YAN-PING

    2016-01-01

    Primary mediastinal choriocarcinoma is a rare malignancy that is characterized by multiple metastases at the time of diagnosis, poor response to therapy and short survival times. There is no standard treatment for this disease. The present study described the case of a 25-year-old man with metastatic mediastinal choriocarcinoma. The patient completed 8 cycles of standard combination chemotherapy consisting of etoposide [100 mg/m2; intravenous (IV) drip on days 1–3], cisplatin (20 mg/m2; IV drip on days 1–5) and bleomycin (20 mg/m2; intramuscular injection on days 1, 8 and 15 every 21 days). The α-fetoprotein level decreased to 2.36 ng/ml, the serum β-human chorionic gonadotropin (β-HCG) level markedly decreased to 8.69 IU/l, which was slightly higher than the normal upper limit, and the lactate dehydrogenase level decreased to a normal range. The computed tomography (CT) scan revealed that the number and size of the lung lesions was significantly reduced subsequent to 8 cycles of chemotherapy and the size of the mediastinal tumor was evidently reduced, with a less solid component and a more cystic component. The response assessment indicated partial remission. Following chemotherapy, a radiation dose of 50 Gy (2.0 Gy/fraction) was administered to the involved field of the mediastinum. Following radiotherapy, the β-HCG level had also decreased to normal levels, and CT evaluation revealed that the size of the residual lung lesions demonstrated no evident change, and the mediastinal tumor was slightly reduced in size, with a less solid component. The patient refused to undergo surgery and did not receive additional treatment following radiotherapy. At present, the patient has survived >16 months of follow-up without any symptoms. PMID:27073527

  2. Descending Necrotizing Mediastinitis Treated with Tooth Extractions following Mediastinal and Cervical Drainage

    PubMed Central

    Fukuchi, Minoru; Suzuki, Okihide; Nasu, Daisuke; Koizumi, Kazue; Muta, Yu; Imaizumi, Hideko; Ishiguro, Toru; Kumagai, Youichi; Ishibashi, Keiichiro; Mochiki, Erito; Ishida, Hideyuki

    2015-01-01

    Descending necrotizing mediastinitis (DNM) is a rare condition in which oropharyngeal infection spreads to the mediastinum via the cervical fascia. Delayed diagnosis and surgery result in a high mortality rate among patients with DNM. We present a case of DNM resulting from odontogenic infection treated successfully with tooth extraction following mediastinal and cervical drainage. A 43-year-old, previously healthy Japanese man was admitted to our hospital for treatment of acute mediastinitis. Computed tomography revealed gas collection around the mid-thoracic esophagus and bilateral pleural effusion. We performed mediastinal drainage via right thoracotomy. Cervicotomy was performed on postoperative day 14 to drain a residual cervical abscess. The patient required the extraction of ten teeth over three procedures to address primary odontogenic infection before his fever resolved on postoperative day 40. Prompt diagnosis, aggressive drainage and removal of the source of infection can improve survival among patients with this life-threatening disease. PMID:26600767

  3. Carcinosarcoma as a primary mediastinal tumor.

    PubMed

    Bayram, Ahmet Sami; Ozdemir, Bulent; Aydiner, Fatma; Gullulu, Sümeyye

    2004-03-01

    Carcinosarcoma is a rare, biphasic and malignant tumor having a mixture of carcinoma and sarcoma containing differentiated mesenchymal elements. It may occur in such diverse locations as the uterus, breast, thyroid, lung, and upper gastrointestinal system. However, to date a primary mediastinal carcinosarcoma has not been reported in the literature. PMID:17670165

  4. Endoscopic mediastinal staging of lung cancer.

    PubMed

    Khoo, Kay-Leong; Ho, Khek-Yu

    2011-04-01

    The advent of endoscopic ultrasound-guided sampling procedures such as endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) and endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) has lead to significant advances in the mediastinal diagnosis and staging of lung cancer. These endoscopic techniques can be performed in the outpatient setting under conscious sedation and local anesthesia, in contrast to the surgical standard, mediastinoscopy (MS), which requires operating theatre time and general anesthesia. Proponents of mediastinoscopy have always emphasized the advantages of mediastinoscopy, namely its sensitivity even with a low prevalence of mediastinal metastases and its low false negative rate. Newer endoscopic techniques such as EBUS-TBNA are showing sensitivities exceeding that of mediastinoscopy, even in the setting of an equally low prevalence of mediastinal metastases. However, endoscopic techniques have double the false negative rate of mediastinoscopy. As the tracheobronchial route and esophageal route provide almost complete access to mediastinal lymph nodes, these endoscopic techniques are complementary rather than competing. When used in combination, it is possible mediastinoscopy may be superseded. The challenge however, is how best to select the appropriate endoscopic procedures to accurately stage lung cancer in the most cost-effective manner. PMID:21130638

  5. Intralobar pulmonary sequestration and mediastinal bronchogenic cyst.

    PubMed Central

    Grewal, R. G.; Yip, C. K.

    1994-01-01

    A patient with a bronchogenic cyst and intralobar pulmonary sequestration is presented. Chest radiography showed a posterior mediastinal mass and a computed tomographic scan of the chest revealed, in addition, an abnormality suggestive of pulmonary sequestration. This was confirmed by an aortogram. It is important to be aware of the coexistence of these anomalies to make a diagnosis preoperatively. Images PMID:8016803

  6. Mediastinal mass caused by syphilitic aortitis.

    PubMed Central

    Hofmann-Wellenhof, R; Domej, W; Schmid, C; Rossmann-Moore, D; Kullnig, P; Annelli-Monti, M

    1993-01-01

    A 47 year old man presented with hoarseness and chest pain found to be due to proliferative syphilitic aortitis. The case is unusual as the syphilitic aortitis caused a mediastinal mass without affecting the lumen of the aorta. Images PMID:8322248

  7. Learning about Cystic Fibrosis

    MedlinePlus

    ... order to digest food. Cystic Fibrosis: A Single Gene Disease Mutations in a single gene - the Cystic ... the defective gene, or correcting the defective protein. Gene Therapy Research Offers Promise of a Cure for ...

  8. What Causes Cystic Fibrosis?

    MedlinePlus

    ... What Causes Cystic Fibrosis? A defect in the CFTR gene causes cystic fibrosis (CF). This gene makes ... and very salty sweat. Research suggests that the CFTR protein also affects the body in other ways. ...

  9. Living with Cystic Fibrosis

    MedlinePlus

    ... from the NHLBI on Twitter. Living With Cystic Fibrosis If you or your child has cystic fibrosis (CF), you should learn as much as you ... about CF Care Centers, go to the Cystic Fibrosis Foundation's Care Center Network Web page. It's standard ...

  10. A Giant Mature Cystic Teratoma Mimicking a Pleural Effusion

    PubMed Central

    Dorterler, Mustafa Erman; Boleken, Mehmet Emin; Koçarslan, Sezen

    2016-01-01

    The vast majority of teratomas originating from more than a single germ layer are benign. Often, such teratomas are initially asymptomatic. Later symptoms are caused by the weight per se of the teratoma and include chest pain, cough, dyspnea, and/or recurrent attacks of pneumonia. A mediastinal teratoma is treated by total surgical resection of the mass. Here, we report a case of giant mature cystic teratoma mimicking a pleural effusion in the thorax at the 7-month-old female patient with a symptom of persistent pulmonary infection and tachypnea. PMID:26942032

  11. Primary Pulmonary Amyloidosis with Mediastinal Lymphadenopathy

    PubMed Central

    Kim, Dohun; Lee, Yong-Moon; Kim, Si-Wook; Kim, Jong-Won; Hong, Jong-Myeon

    2016-01-01

    We report a case of inadvertent hoarseness after surgery for primary pulmonary amyloidosis. A 55-year-old male was transferred to our facility due to a lung mass. Chest computed tomography revealed a solitary pulmonary nodule. Positron emission tomography–computed tomography showed fluorodeoxyglucose uptake in the main mass and in the mediastinal lymph nodes. To confirm the pathology of the mass, wedge resection and thorough lymph node dissection were performed via video-assisted thoracic surgery (VATS). No complications except for hoarseness were observed; hoarseness developed soon after surgery and lasted for 3 months. The main mass was diagnosed as amyloidosis, but this was not found in the lymph nodes. In conclusion, VATS wedge resection for peripheral amyloidosis is a feasible and safe procedure. However, mediastinal lymph node dissection is not recommended unless there is evidence of a clear benefit. PMID:27298804

  12. DESCENDING NECROTIZING MEDIASTINITIS SECONDARY TO RETROPHARYNGEAL ABSCESS.

    PubMed

    Kovacić, Marijan; Kovacić, Ivan; Dželalija, Boris

    2015-12-01

    Descending necrotizing mediastinitis secondary to a nontraumatic retropharyngeal abscess is very rare. This form of mediastinitis in the era of potent antibiotics often ends up with lethal outcome. It usually occurs in immunocompromised patients and requires intensive multidisciplinary treatment approach. We report a case of nontraumatic retropharyngeal abscess complicated by descending necrotizing mediastinitis in a 70-year-old man with insulin dependent diabetes mellitus. The patient was admitted to our hospital after clinical and radiological diagnosis of retropharyngeal abscess. During treatment for retropharyngeal abscess with antibiotic therapy and transoral incision, the patient showed mild clinical improvement but his condition suddenly aggravated on day 4 of hospital stay. He had high fever, chest pain with tachypnea, tachycardia, hypotension, and showed signs of occasional disorientation. Emergency computed tomography (CT) scan of the neck and thorax showed inflammation in the retropharyngeal space, as well as thickening of the upper posterior mediastinum fascia with the presence of air. Emergency surgery including cervicotomy and drainage of the retropharyngeal space and posterior mediastinum was performed. The patient promptly recovered with improvement of the clinical status and laboratory findings. After 16 days of treatment he was discharged from the hospital in good condition. Descending necrotizing mediastinitis can be a serious and life threatening complication of deep neck infection if the diagnosis is not quickly established. Besides inevitable application of antimicrobial drugs, good drainage of the mediastinum is necessary. We believe that transcervical approach can achieve high-quality drainage of the upper mediastinum, especially if it is done timely as in this case. Its efficacy can be verified by intensive monitoring of the patient clinical condition, by CT scan of the thorax, and by laboratory tests. In the case of inefficacy of this type

  13. Descending necrotizing mediastinitis complicating hyperimmunoglobulin E syndrome

    PubMed Central

    Hayakawa, Takamitsu; Neyatani, Hiroshi; Funai, Kazuhito

    2016-01-01

    Abstract Descending necrotizing mediastinitis (DNM) is a potentially lethal disease that originates from a deep neck infection (DNI); it is often associated with an immunocompromised state. Hyperimmunoglobulin E syndrome (HIES) is an extremely rare complex immune deficiency characterized by recurrent abscesses of staphylococcal etiology. A rare case of a 34‐year‐old woman wherein HIES putatively promoted the development of DNI into DNM is described. She presented with cervical purulent lymphadenitis and retropharyngeal abscess. Despite immediate cervical drainage and use of broad‐spectrum antibiotics, she developed DNI and subsequently DNM. Mediastinal drainage with thoracotomy and subsequent deep neck drainage were performed. Bacterial culture of the abscess isolated methicillin‐resistant Staphylococcus aureus (MRSA). Although a postoperative recurrent deep neck abscess required a second surgery, we succeeded in conservative remission of recurrent mediastinal abscess with long‐term use of anti‐MRSA drugs. Sufficient drainage under thoracotomy and robust administration of postoperative antibiotics resulted in successful management of HIES‐associated DNM. PMID:27512566

  14. A clue in a colour? EBUS-TBNA in the analysis of isolated mediastinal lymphadenopathy

    PubMed Central

    Ryan, Daniel John; McPhillips, Dympna; Bruzzi, John; Breen, David

    2014-01-01

    A 52-year-old female patient with a background history of malignant melanoma was referred to the interventional pulmonology unit at Galway University Hospital for the investigation of possible intrathoracic recurrence. A CT of the thorax (CT thorax) demonstrated mediastinal lymphadenopathy at position 4R, right paratracheal region. An endobronchial ultrasound (EBUS) revealed multiple enlarged lymph nodes at station 4R. The nodes were heterogeneous with solid and cystic elements. Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) obtained a black material consistent with malignant melanoma. Multiple TBNA samples were obtained and the diagnosis was confirmed pathologically by the presence of multiple epithelioid pigmented cells on cell block analysis. This case report emphasises the value of EBUS in the assessment of metastatic disease and, in particular, how the gross appearance of the sample can be suggestive of the diagnosis. PMID:24567182

  15. Anaesthetic Consideration in Macroglossia Due to Lymphangioma of Tongue: A Case Report

    PubMed Central

    Tewari, Anurag; Munjal, Munish; Kamakshi; Garg, Shuchita; Sood, Dinesh; Katyal, Sunil

    2009-01-01

    Summary Successful airway management of an infant or child with macroglossia prerequisites recognition of a potential airway problem. We describe our experience with a debilitated 13-year-old girl who presented with severe macroglossia, secondary to lymphangioma of the tongue. Along with the social discomfort she had inability to speak, eat or drink properly and exposure-induced dryness. Such patients are a challenge for the anaesthesiologists due to the anticipated difficult intubation associated with the oral mucosa occupying lesion. It also becomes pertinent to rule out any of the associated congenital anomalies. The importance of a thorough preoperative evaluation and attention to difficult intubation and maintenance of airway is emphasized. We endeavor to review the available literature regarding patient's perioperative management of such patients. PMID:20640084

  16. [Cystic renal pathology].

    PubMed

    Rosi, P; Cesaroni, M; Bracarda, S; Rociola, W; Virgili, G

    1993-08-01

    Ultrasonography has a great interest in diagnosis of cystic kidney disorders for typical eco-pattern of this pathology. In this work we show the eco-pattern of the most common cystic kidney disorders. Particularly we examine simple cysts (typical, atypical, complicated), multicystic kidney dysplasia, autosomal recessive polycystic kidney disease (infantile) autosomal dominant polycystic kidney disease (adult age). The so-called neoplastic cysts (multiloculated cysts, multiloculated cysts nephroma, cystic nephroblastoma), medullar cysts (medullary sponge kidney, medullary cystic disease), parapyelic cysts, acquired cystic kidney disease in renal failure patients, parasitic cysts, epidermoid cysts. About this disorders we present the more typical and expressive ultrasonographic appearance and we define the role and the opportunity of diagnostic setting by echography, moreover ultrasonography allows us to make a differential diagnosis between cystic kidney disorders and other kidney disease. PMID:8353538

  17. How Is Cystic Fibrosis Diagnosed?

    MedlinePlus

    ... from the NHLBI on Twitter. How Is Cystic Fibrosis Diagnosed? Doctors diagnose cystic fibrosis (CF) based on ... to see whether the baby has CF. Cystic Fibrosis Carrier Testing People who have one normal CFTR ...

  18. Renal cystic disease

    SciTech Connect

    Hartman, D.S.

    1988-01-01

    The book begins with an overview of renal cystic disease and a presentation of simple renal cysts. Subsequent chapters cover cystic disease in association with renal neoplasms and medullary sponge kidney. The chapters addressing autosomal-dominant and autosomal-recessive polycystic kidney disease discuss and differentiate the infantile and adult forms of the disease. There are also separate discussions of medullary cystic disease, multicystic dysplastic kidney, and cysts of the renarenal sinus.

  19. Video Assisted Thoracoscopic Surgery (VATS) for Excision of an Ectopic Anterior Mediastinal Intra-Thymic Parathyroid Adenoma.

    PubMed

    Naik, Dukhabandhu; Jebasingh, Kumaradoss Felix; Ramprasath; Roy, Gnanamuthu Birla; Paul, Mazhuvanchary Jacob

    2016-06-01

    Ectopic anterior mediastinal parathyroid adenoma is a rare cause of Primary Hyperparathyroidism (PHPT). Imaging studies such as Technetium-99m ((99m)Tc) sestamibi parathyroid scan along with a Single Photon Emission Computerized Tomogram (SPECT), and contrast enhanced Computerized Tomogram (CT) of the neck and thorax can precisely localize the ectopic mediastinal parathyroid adenoma. We report a 40-year-old gentleman who presented with persistent pain in the right shoulder following trivial trauma. His biochemical investigations showed an elevated serum calcium of 11.6mg% (Normal: 8.3-10.4 mg%) along with an elevated iPTH of 1443 pg/ml (normal: 8-70 pg/ml) which were suggestive of primary hyperparathyroidism. The localization studies revealed an ectopic cystic parathyroid adenoma in the anterior mediastinum that was not accessible from the neck. He underwent a Video Assisted Thoracoscopic (VAT) excision procedure with normalization of serum calcium and an uncomplicated recovery. The VAT approach is a successful minimally invasive technique for mediastinal parathyroidectomy. PMID:27504353

  20. Video Assisted Thoracoscopic Surgery (VATS) for Excision of an Ectopic Anterior Mediastinal Intra-Thymic Parathyroid Adenoma

    PubMed Central

    Naik, Dukhabandhu; Jebasingh, Kumaradoss Felix; Ramprasath; Roy, Gnanamuthu Birla

    2016-01-01

    Ectopic anterior mediastinal parathyroid adenoma is a rare cause of Primary Hyperparathyroidism (PHPT). Imaging studies such as Technetium-99m (99mTc) sestamibi parathyroid scan along with a Single Photon Emission Computerized Tomogram (SPECT), and contrast enhanced Computerized Tomogram (CT) of the neck and thorax can precisely localize the ectopic mediastinal parathyroid adenoma. We report a 40-year-old gentleman who presented with persistent pain in the right shoulder following trivial trauma. His biochemical investigations showed an elevated serum calcium of 11.6mg% (Normal: 8.3-10.4 mg%) along with an elevated iPTH of 1443 pg/ml (normal: 8-70 pg/ml) which were suggestive of primary hyperparathyroidism. The localization studies revealed an ectopic cystic parathyroid adenoma in the anterior mediastinum that was not accessible from the neck. He underwent a Video Assisted Thoracoscopic (VAT) excision procedure with normalization of serum calcium and an uncomplicated recovery. The VAT approach is a successful minimally invasive technique for mediastinal parathyroidectomy. PMID:27504353

  1. Pathological assessment of mediastinal lymph nodes in lung cancer: implications for non-invasive mediastinal staging.

    PubMed Central

    Kerr, K M; Lamb, D; Wathen, C G; Walker, W S; Douglas, N J

    1992-01-01

    BACKGROUND: The use of computed tomography in mediastinal staging of lung cancer relies on the premiss that malignant lymph nodes are larger than benign ones. This hypothesis was tested by linking node size and presence or absence of malignancy and looking at factors possibly influencing the size of benign nodes. METHODS: All accessible mediastinal lymph nodes were taken from 56 consecutive patients with lung cancer who underwent thoracotomy. Nodes were measured and histologically examined. Resected cancer bearing lung from 44 of these patients was assessed for degree of acute and chronic inflammation. RESULTS: Lymph node size was not significantly related to the presence of metastatic disease, 58% of malignant and 43% of benign lymph nodes measuring over 15 mm. Similarly, there was no statistically significant relation between size of lymph nodes and the likelihood of malignancy, 20% of lymph nodes of 10 mm or more but also 15% of those less than 10 mm being malignant. Thresholds of 15 and 20 mm showed similar results. The maximum size of benign lymph nodes was significantly greater in those patients with histological evidence of acute pulmonary inflammation than in those without. CONCLUSIONS: The study shows that in patients with lung cancer (1) malignant mediastinal lymph nodes are not larger than benign nodes; (2) small mediastinal lymph nodes are not infrequently malignant; and (3) benign adenopathy is more common in patients with acute pulmonary inflammation. Images PMID:1609375

  2. Mediastinal emphysema following fracture of the orbital floor

    PubMed Central

    Abdelrahman, Husham; Shunni, Adam; El-Menyar, Ayman; Ajaj, Ahmad; Afifi, Ibrahim; Zarour, Ahmad; Al-Thani, Hassan

    2014-01-01

    Pneumomediastinum (PM) is mainly an atypical finding among traumatic neck or thoracic injury patients. Moreover, PM secondary to isolated orbital floor fracture remains a rare event which is infrequently associated with severe complications such as mediastinitis, airway obstruction and pneumothorax. Herein, we report an atypical case of mediastinal emphysema consequent to orbital floor fracture along with review of the literature. PMID:24876504

  3. Descending Mediastinitis in Epstein-Barr Virus Infection

    PubMed Central

    van Driel, E. M.; Janssen, M. J. F. M.

    2015-01-01

    Our case report describes a previously healthy 34-year-old male who develops a descending mediastinitis as a complication of an Epstein-Barr virus (EBV) infection. The mediastinitis was suspected to have developed by a breakthrough of a peritonsillar abscess through the space between the alar and prevertebral space. PMID:25740774

  4. Tuberculosis-associated Fibrosing Mediastinitis: Case Report and Literature Review

    PubMed Central

    Tan, Ronald; Martires, Joanne; Kamangar, Nader

    2016-01-01

    Fibrosing mediastinitis is a rare condition defined by the presence of fibrotic mediastinal infiltrates that obliterate normal fat planes. It is a late complication of a previous granulomatous infection, such as histoplasmosis or tuberculosis (TB). Due to its rarity, fibrosing mediastinitis is often under-recognized, and the clinical presentation is variable and dependent on the extent of infiltration or encasement of structures within the mediastinum. We present a case of fibrosing mediastinitis in a man with a prior history of TB, who presented with progressive dyspnea and was found to have chronic mediastinal soft tissue opacities and pulmonary hypertension. His diagnosis was delayed due to the lack of recognition of this clinical/radiographic entity. Fibrosing mediastinitis is a rare entity usually caused by granulomatous disease. Most cases develop as a late complication of histoplasmosis or TB. The presence of calcified mediastinal soft tissue infiltrates on advanced chest imaging can be diagnostic of fibrosing mediastinitis in patients with a prior history of a granulomatous infection once active processes such as malignancy are excluded. PMID:27625898

  5. Tuberculosis-associated Fibrosing Mediastinitis: Case Report and Literature Review.

    PubMed

    Tan, Ronald; Martires, Joanne; Kamangar, Nader

    2016-01-01

    Fibrosing mediastinitis is a rare condition defined by the presence of fibrotic mediastinal infiltrates that obliterate normal fat planes. It is a late complication of a previous granulomatous infection, such as histoplasmosis or tuberculosis (TB). Due to its rarity, fibrosing mediastinitis is often under-recognized, and the clinical presentation is variable and dependent on the extent of infiltration or encasement of structures within the mediastinum. We present a case of fibrosing mediastinitis in a man with a prior history of TB, who presented with progressive dyspnea and was found to have chronic mediastinal soft tissue opacities and pulmonary hypertension. His diagnosis was delayed due to the lack of recognition of this clinical/radiographic entity. Fibrosing mediastinitis is a rare entity usually caused by granulomatous disease. Most cases develop as a late complication of histoplasmosis or TB. The presence of calcified mediastinal soft tissue infiltrates on advanced chest imaging can be diagnostic of fibrosing mediastinitis in patients with a prior history of a granulomatous infection once active processes such as malignancy are excluded. PMID:27625898

  6. Mediastinal angiomatosis: a rare case report

    PubMed Central

    Pak, Min Gyoung; Choi, Phil Jo; Choi, Won Suk; Kang, Eun Ju

    2015-01-01

    Angiomatosis refers to a rare condition of large hamartomatous vascular lesions that extensively affect a region of the body or several different tissue types in a contiguous way. Several cases have been reported in the mediastinum. We experienced a histologically proven case of mediastinal angiomatosis in a 56-year-old woman that was incidentally detected as multiple conglomerated masses mimicking metastatic lymph nodes on chest radiography. Despite its rareness, our case emphasizes that pathologists and radiologists need to be aware of the rare diagnosis of angiomatosis in the mediastinum. PMID:26793386

  7. Mediastinal bronchogenic cyst mimicking congenital lobar emphysema.

    PubMed

    Arun, Sumitha; Kumar, Manish; Ross, Benjamin Jeyanth

    2016-01-01

    Bronchogenic cyst (BC) is a rare congenital malformation of the lung. Most patients remain asymptomatic until adulthood while some are symptomatic in the first few years of life. However, symptoms in newborn period are rare. We report a case of a 3-day-old preterm baby with respiratory distress diagnosed as congenital lobar emphysema on chest X-ray. A CT scan revealed a mediastinal cyst causing obstructive lobar emphysema. The cyst was excised and pathological examination was suggestive of BC. PMID:27609589

  8. [A case of mediastinal cavernous hemangioma].

    PubMed

    Maebeya, S; Nishimura, O; Yokoi, H; Shimizu, T; Yoshimasu, T; Naito, Y

    1990-03-01

    A 6-year-old boy had an abnormal shadow on the chest X-ray film. It showed a tumor shadow with calcification on the right hilum. The plain CT scan showed an anterior mediastinal mass and its density was similar to that of large vessels. On the angio CT scan the lesion displayed a much lower enhancement than large vessels. The tumor was resected completely by median sternotomy. It was 5.6 X 3.6 X 3.0 cm in size and contained a phlebolith 5 mm in diameter. The histological diagnosis was cavernous hemangioma. PMID:2348129

  9. Posterior mediastinal extramedullary hematopoiesis secondary to hypoxia

    PubMed Central

    Solazzo, A; D’Auria, V; Moccia, LG; Vatrella, A; Bocchino, M; Rea, G

    2016-01-01

    Two mediastinal masses were incidentally detected at high resolution computed tomography (HRCT) of a 72 year-old male patient, former smoker, affected by chronic obstructive pulmonary disease with worsening dyspnea and 2-year medical history of polycythemia secondary to hypoxia. Integration with a multidetector computed tomography (MDCT) scan after administration of intravenous injection contrast medium showed slightly inhomogeneous increase of enhancement of masses, suggesting in the first case potential malignancy. Diagnosis of extramedullary hematopoiesis was achieved by fine needle aspiration citology (FNAC). Extramedullary hematopoiesis must be considered in differential diagnosis in patients with medical history of polycythemia and severe hypoxia. PMID:27326388

  10. Posterior mediastinal extramedullary hematopoiesis secondary to hypoxia.

    PubMed

    Solazzo, A; D'Auria, V; Moccia, L G; Vatrella, A; Bocchino, M; Rea, G

    2016-05-01

    Two mediastinal masses were incidentally detected at high resolution computed tomography (HRCT) of a 72 year-old male patient, former smoker, affected by chronic obstructive pulmonary disease with worsening dyspnea and 2-year medical history of polycythemia secondary to hypoxia. Integration with a multidetector computed tomography (MDCT) scan after administration of intravenous injection contrast medium showed slightly inhomogeneous increase of enhancement of masses, suggesting in the first case potential malignancy. Diagnosis of extramedullary hematopoiesis was achieved by fine needle aspiration citology (FNAC). Extramedullary hematopoiesis must be considered in differential diagnosis in patients with medical history of polycythemia and severe hypoxia. PMID:27326388

  11. Asymptomatic peritoneal carcinomatosis originating from benign cystic peritoneal mesothelioma

    PubMed Central

    Iacoponi, S; Calleja, J; Hernandez, G; de la Cuesta, R Sainz

    2015-01-01

    Benign multicystic mesothelioma is a rare tumour that originates from the abdominal peritoneum with a predisposition to the pelvic peritoneum. It typically affects women of reproductive age. There have been less than 200 cases of this rare neoplasia reported to date. We present the case of a 35-year-old woman who was referred to our centre because of the detection of a peritoneal carcinomatosis during a gynaecological exam. A diagnostic laparoscopy was performed. The findings included multiple cysts appearing as ‘a bunch of grapes’ occupying the omentum. Biopsies were taken during the surgery and the results showed benign multicystic peritoneal mesothelioma. Benign multicystic mesothelioma can simulate other conditions, such as malignant ovarian tumours or cystic lymphangioma. It is often diagnosed accidentally during surgery performed for another reason. The diagnosis is interoperative, observing multicystic structures grouped as a ‘bunch of grapes’ containing clear fluid with thin walls made of connective tissue. Immunohistochemistry confirmed mesothelial origin. Surgery is considered the treatment of choice and is based on the removal of the cysts from the abdominal cavity. Hyperthermic intraperitoneal chemotherapy can be considered as a primary treatment in patients with recurrences or even as a part of primary treatment associated with surgery. Survival at 5 years is 100% and invasive or malignant progression is extraordinary. The treatment approach should be multidisciplinary, and the patient should be referred to a referral centre. PMID:26715942

  12. Cranial mediastinal carcinomas in nine dogs.

    PubMed

    Liptak, J M; Kamstock, D A; Dernell, W S; Ehrhart, E J; Rizzo, S A; Withrow, S J

    2008-03-01

    Nine dogs were diagnosed with cranial mediastinal carcinomas. Based on histological and immunohistochemical analysis, four dogs were diagnosed with ectopic follicular cell thyroid carcinomas, one dog with ectopic medullary cell thyroid carcinoma, two dogs with neuroendocrine carcinomas and two dogs with anaplastic carcinomas. Clinical signs and physical examination findings were associated with a space-occupying mass, although one dog was diagnosed with functional hyperthyroidism. Surgical resection was attempted in eight dogs. The cranial mediastinal mass was invasive either into the heart or into the cranial vena cava in three dogs. Resection was complete in six dogs and unresectable in two dogs. All dogs survived surgery, but four dogs developed pulmonary thromboembolism and two dogs died of respiratory complications postoperatively. Adjunctive therapies included pre-operative radiation therapy (n=1) and postoperative chemotherapy (n=3). Three dogs had metastasis at the time of diagnosis, but none developed metastasis following surgery. The overall median survival time was 243 days. Local invasion, pleural effusion and metastasis did not have a negative impact on survival time in this small case series. PMID:19178660

  13. Inherited renal cystic diseases.

    PubMed

    Kim, Bohyun; King, Bernard F; Vrtiska, Terri J; Irazabal, Maria V; Torres, Vicente E; Harris, Peter C

    2016-06-01

    A number of inherited renal diseases present with renal cysts and often lead to end-stage renal disease. With recent advances in genetics, increasing number of genes and mutations have been associated with cystic renal diseases. Although genetic testing can provide a definite diagnosis, it is often reserved for equivocal cases or for ongoing investigational research. Therefore, imaging findings are essential in the routine diagnosis, follow-up, and detection of complications in patients with inherited cystic renal diseases. In this article, the most recent classification, genetic analysis, clinical presentations, and imaging findings of inherited cystic renal diseases will be discussed. PMID:27167233

  14. Management of large mediastinal masses: surgical and anesthesiological considerations

    PubMed Central

    van Boven, Wim Jan P.; Annema, Jouke T.; Eberl, Susanne; Klomp, Houke M.; de Mol, Bas A.J.M.

    2016-01-01

    Large mediastinal masses are rare, and encompass a wide variety of diseases. Regardless of the diagnosis, all large mediastinal masses may cause compression or invasion of vital structures, resulting in respiratory insufficiency or hemodynamic decompensation. Detailed preoperative preparation is a prerequisite for favorable surgical outcomes and should include preoperative multimodality imaging, with emphasis on vascular anatomy and invasive characteristics of the tumor. A multidisciplinary team should decide whether neoadjuvant therapy can be beneficial. Furthermore, the anesthesiologist has to evaluate the risk of intraoperative mediastinal mass syndrome (MMS). With adequate preoperative team planning, a safe anesthesiological and surgical strategy can be accomplished. PMID:27076967

  15. Aortic Pseudoaneurysm Secondary to Mediastinitis due to Esophageal Perforation

    PubMed Central

    Zuluaga, Claudia Patricia; Aluja Jaramillo, Felipe; Velásquez Castaño, Sergio Andrés; Rivera Bernal, Aura Lucía; Granada, Julio Cesar; Carrillo Bayona, Jorge Alberto

    2016-01-01

    Esophageal perforation is a condition associated with high morbidity and mortality rates; it requires early diagnosis and treatment. The most common complication of esophageal rupture is mediastinitis. There are several case reports in the literature of mediastinitis secondary to esophageal perforation and development of aortic pseudoaneurysm as a complication. We report the case of a patient with an 8-day history of esophageal perforation due to foreign body (fishbone) with mediastinitis and aortic pseudoaneurysm. The diagnosis was made using Computed Tomography (CT) with intravenous and oral water-soluble contrast material. An esophagogastroduodenoscopy did not detect the perforation. PMID:26977330

  16. A rheumatoid nodule in an unusual location: mediastinal lymph node

    PubMed Central

    Yachoui, Ralph; Ward, Celine; Kreidy, Mazen

    2013-01-01

    Rheumatoid arthritis (RA) is a multisystem inflammatory disease characterised by destructive synovitis and varied extra-articular involvement. Rheumatoid lung nodules are the most common pulmonary manifestations of RA. Rheumatoid nodules in mediastinal lymph nodes are extremely uncommon. We describe a male patient with long-standing RA and subcutaneous rheumatoid nodules presenting with multiple lung nodules and mediastinal lymphadenopathies. Definite histopathology of a lymph node was consistent with necrobiotic granuloma due to RA. Clinicians should be aware of rheumatoid nodules as a potential cause of mediastinal lymphadenopathies, mainly in advanced rheumatoid arthritis. PMID:23645652

  17. Recurrent primary mediastinal liposarcoma: A case report

    PubMed Central

    ZHAO, CHUN; ZHANG, FANGBIAO; ZHANG, XIANGYAN; TU, SHAOSONG; WU, ZHIJUN; LI, XIA; XIANG, YINGMING; ZHENG, CHUNHUI; ZENG, QINGHUI

    2016-01-01

    Primary mediastinal liposarcomas are extremely rare. The current study reports the case of a 63-year-old man presenting with a primary liposarcoma arising from the posterior mediastinum. The patient reported a 6-month history of chest pain with increasing dyspnea for 2 months. Enhanced computed tomography revealed a 10×16-cm mass in the posterior mediastinum. Other physical examinations were normal. Radical resection was performed under the agreement of patient. Subsequent pathological analysis indicated a liposarcoma. The patient recovered and was successfully discharged. However, at a follow-up examination 12 months after surgery, recurrence was identified in the anterior mediastinum. Therefore, the patient underwent surgery. The postoperative course was uneventful, however, there was evidence of disease recurrence 2 years after the second surgery. The patient refused any treatment and succumbed after 3 months. PMID:27313694

  18. Cystic Adenomatoid Odontogenic Tumor

    PubMed Central

    Grover, Sonal; Rahim, Ahmed Mujib Bangalore; Parakkat, Nithin Kavassery; Kapoor, Shekhar; Mittal, Kumud; Sharma, Bhushan; Shivappa, Anil Bangalore

    2015-01-01

    Adenomatoid Odontogenic Tumor (AOT) is a well-established benign epithelial lesion of odontogenic origin. Rightfully called “the master of disguise,” this lesion has been known for its varied clinical and histoarchitectural patterns. Not only does AOT predominantly present radiologically as a unilocular cystic lesion enclosing the unerupted tooth (which is commonly mistaken as a dentigerous cyst) but the lesion also presents rarely with a cystic component histopathologically. We present one such unusual case of cystic AOT associated with an impacted canine, mimicking a dentigerous cyst. The present case aims to highlight the difference between cystic AOT and dentigerous cyst radiographically. The exact histogenesis of AOT and its variants still remains obscure. An attempt has been made to hypothesize the new school of thought regarding the origin of AOT. PMID:26579317

  19. Acquired Cystic Kidney Disease

    MedlinePlus

    ... a kidney transplant or blood-filtering treatments called dialysis. The cysts are more likely to develop in people who are on kidney dialysis. The chance of developing acquired cystic kidney disease ...

  20. Epidural Cystic Spinal Meningioma

    PubMed Central

    Zhang, Ji; Chen, Zheng-he; Wang, Zi-feng; Sun, Peng; Jin, Jie-tian; Zhang, Xiang-heng; Zhao, Yi-ying; Wang, Jian; Mou, Yong-gao; Chen, Zhong-ping

    2016-01-01

    Abstract Cystic spinal meningioma (CSM) is an uncommon meningioma variant. Extradural CSMs are particularly rare and difficult to distinguish from other intraaxial tumors. This study presents a case of a 36-year-old woman with intraspinal extradual CSM at the thoracolumbar spine. She experienced persistent weakness, progressive numbness, and sensory disturbance in the right lower limb. Magnetic resonance imaging (MRI) of the patient revealed an irregular cystic mass at the thoracic 11 to lumbar 3 levels dorsally. This case was misdiagnosed as other neoplasms prior to surgery because of the atypical radiographic features and location of the tumor. Extradural CSMs should be considered in the differential diagnosis of intraspinal extradural cystic neoplasms. Complete removal of cystic wall provides an optimal outcome, rendering the lesion curable. PMID:26986119

  1. Cystic Fibrosis (CF) Respiratory Screen: Sputum

    MedlinePlus

    ... Cystic Fibrosis (CF) Chloride Sweat Test Lungs and Respiratory System Cystic Fibrosis: Diet and Nutrition Cystic Fibrosis Cystic Fibrosis: Diet and Nutrition Lungs and Respiratory System Contact Us Print Resources Send to a friend ...

  2. Troubleshooting thoracoscopic anterior mediastinal surgery: lessons learned from thoracoscopic lobectomy

    PubMed Central

    Hirji, Sameer A.; Balderson, Scott S.; Berry, Mark F.

    2015-01-01

    Video-assisted thoracoscopic surgery (VATS) lobectomy is safe, oncologically effective, and increasingly utilized for lung cancer resection. Lessons from VATS lobectomy experience can guide the use of a VATS approach to resect mediastinal masses. Exposure and dissection when using VATS to resect anterior mediastinal masses has unique challenges. Several maneuvers acquired from experience with VATS lobectomy can reduce the technical difficulty and often prevent conversion to an open approach. In this troubleshooting guide, we offer ‘tips’ to both avoid and manage numerous intra-operative technical difficulties that commonly arise during VATS anterior mediastinal procedures. Avoiding an open approach may improve outcomes, although conversion for safety or complete resection can be necessary. Techniques and experiences derived from VATS lobectomy can facilitate VATS resection of mediastinal masses. PMID:26693151

  3. Descending necrotizing Mediastinitis caused by Kocuria rosea: a case report

    PubMed Central

    2013-01-01

    Background Kocuria species are gram-positive, non-pathogenic commensals. However, in immunocompromised patients such as transplant recipients, cancer patients, or patients with chronic medical conditions, they can cause opportunistic infections. Case presentation We report the first case of descending necrotizing mediastinitis in a 58-year-old, relatively healthy woman caused by Kocuria rosea. Conclusion Descending necrotizing mediastinitis due to Kocuria rosea can be successfully treated with prompt surgical drainage combined with antimicrobial therapy. PMID:24112281

  4. Multiple cystic lung disease.

    PubMed

    Ferreira Francisco, Flavia Angélica; Soares Souza, Arthur; Zanetti, Gláucia; Marchiori, Edson

    2015-12-01

    Multiple cystic lung disease represents a diverse group of uncommon disorders that can present a diagnostic challenge due to the increasing number of diseases associated with this presentation. High-resolution computed tomography of the chest helps to define the morphological aspects and distribution of lung cysts, as well as associated findings. The combination of appearance upon imaging and clinical features, together with extrapulmonary manifestations, when present, permits confident and accurate diagnosis of the majority of these diseases without recourse to open-lung biopsy. The main diseases in this group that are discussed in this review are lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis and folliculin gene-associated syndrome (Birt-Hogg-Dubé); other rare causes of cystic lung disease, including cystic metastasis of sarcoma, are also discussed. Disease progression is unpredictable, and understanding of the complications of cystic lung disease and their appearance during evolution of the disease are essential for management. Correlation of disease evolution and clinical context with chest imaging findings provides important clues for defining the underlying nature of cystic lung disease, and guides diagnostic evaluation and management. PMID:26621970

  5. Acute Mediastinitis in Children: A Nine-Year Experience

    PubMed Central

    Mirshemirani, Alireza; Rouzrokh, Mohsen; Mohajerzadeh, Laili; Tabari, Nasibeh Khaleghnejad; Ghaffari, Parand

    2013-01-01

    Background Acute mediastinitis is a serious medical condition with a mortality rate of 30 to 40% or even higher. Early diagnosis with prompt and aggressive treatment is essential to prevent its rapid progression. We evaluated acute mediastinitis cases and analyzed the outcomes. Materials and Methods A retrospective chart review was conducted on patients diagnosed with acute mediastinitis who were admitted to Mofid Children's Hospital from January 2001 to January 2010. Results Seventeen patients aged 1 to 10 yrs. (mean =3.8 yrs) were evaluated including 12 (70%) boys and 5 (30%) girls. The most common symptoms were fever, dyspnea, cyanosis, tachycardia and tachypnea. The etiology of mediastinitis was iatrogenic esophageal perforation (EP), and related to manipulation in 13(77%), and leakage of esophageal anastomosis in 4 cases (33%). The underlying diseases were esophageal atresia in 2(12%), corrosive injury of the esophagus in 13(76%), congenital esophageal stenosis in one (6%), and gastroesophageal reflux esophagitis also in one (6%) patient. Patients with clinical symptoms were evaluated by immediate chest radiography, and gastrografin swallow. After early diagnosis, the patients received wide spectrum antibiotics and immediate mediastinal or thoracic drainage, followed by esophagostomy and gastrostomy. Only one case of endoscopic perforation was managed by NG tube. Fifteen patients (88%) survived successfully. We had 2(12%) cases of mortality in our study (one patient after esophageal substitution, mediastinal abscess and septicemia, and the other one developed esophageal perforation 6 months after early management and died of cardiac arrest during endoscopic dilation). Conclusion Prevention of acute mediastinitis is still a difficult challenge. As the prognosis is not good and patients have high mortality, rapid management is mandatory. PMID:25191462

  6. Effective treatment for malignant mediastinal teratoma.

    PubMed Central

    Parker, D; Holford, C P; Begent, R H; Newlands, E S; Rustin, G J; Makey, A R; Bagshawe, K D

    1983-01-01

    Primary malignant mediastinal teratoma is a rare tumour previously regarded as inevitably fatal. In a series of eight male patients with a mean age of 24 years five remain alive and well. All patients showed raised serum concentrations of human chorionic gonadotrophin or alpha fetoprotein. The patients were treated with intermittent combination chemotherapy that included cisplatin. Six patients responded to chemotherapy with a fall in human chorionic gonadotrophin or alpha fetoprotein to near normal levels and they then had radical excision of the remaining tumour. Living malignant tumour was found in four of the specimens and these patients received postoperative chemotherapy. One patient died after eight months and the remaining five patients are alive and well 13-136 months after the start of treatment. The two patients who did not undergo surgery died at one month and 15 months. Intermittent combination chemotherapy and carefully timed radical excision of these tumours would appear to have produced better results than have been reported in other series. Images PMID:6198739

  7. Effective treatment for malignant mediastinal teratoma.

    PubMed

    Parker, D; Holford, C P; Begent, R H; Newlands, E S; Rustin, G J; Makey, A R; Bagshawe, K D

    1983-12-01

    Primary malignant mediastinal teratoma is a rare tumour previously regarded as inevitably fatal. In a series of eight male patients with a mean age of 24 years five remain alive and well. All patients showed raised serum concentrations of human chorionic gonadotrophin or alpha fetoprotein. The patients were treated with intermittent combination chemotherapy that included cisplatin. Six patients responded to chemotherapy with a fall in human chorionic gonadotrophin or alpha fetoprotein to near normal levels and they then had radical excision of the remaining tumour. Living malignant tumour was found in four of the specimens and these patients received postoperative chemotherapy. One patient died after eight months and the remaining five patients are alive and well 13-136 months after the start of treatment. The two patients who did not undergo surgery died at one month and 15 months. Intermittent combination chemotherapy and carefully timed radical excision of these tumours would appear to have produced better results than have been reported in other series. PMID:6198739

  8. Immature mediastinal teratoma with unusual histopathology

    PubMed Central

    Mustafa, Osama M.; Mohammed, Shamayel F.; Aljubran, Ali; Saleh, Waleed N.

    2016-01-01

    Abstract Germ cell tumors (GCTs) represent a well-recognized group of heterogeneous neoplasms with diverse clinical, histopathological, diagnostic, and prognostic characteristics. We present a rare case of a locally aggressive, chemotherapy-resistant immature mediastinal teratoma with a peculiar histological finding of a multilineage somatic-type malignant degeneration. A 21-year-old male patient presented with a 3-week history of persistent, blood-tinged productive cough and shortness of breath. A contrast-enhanced computed tomography (CT) scan of the chest showed a heterogeneous mass occupying the right hemithorax and abutting on adjacent structures. CT-guided biopsy was consistent with immature teratoma. Combination chemotherapy with bleomycin, etoposide, and cisplatin was initiated, albeit without success; the mass showed interval progression in size, and surgical resection through clamshell incision was performed. Histological assessment of the resected mass confirmed the diagnosis of immature teratoma and revealed an extensive multilineage malignant differentiation into sarcomatous, carcinomatous, and melanomatous components. The patient underwent an uneventful recovery but presented 2 months later with extensive liver and bone melanomatous metastases. In this report, relevant findings from the literature are also highlighted. Despite being exceptionally rare, such tumors carry poor prognosis. Understanding the clinicopathological characteristics and biological behavior of such tumors may provide an insight into interventions tailored to improve the otherwise dismal disease outlook. PMID:27367976

  9. Giant mediastinal parathyroid adenoma: a case report.

    PubMed

    Costanzo, Mario; Terminella, Alberto; Marziani, Alessia; Chisari, Antonio; Missiato, Alfredo; Cannizzaro, Matteo Angelo

    2009-01-01

    A 65 year old female patient suffering from multinodular goitre and from hypercalcaemic syndrome which had been and was being treated with drugs for a number of years. The patient undergoes total thyroidectomy. Surgical neck and anterior mediastinum exploration was negative for sick parathyroids. Neck and chest TC and 'TC sestamibi scintigraphy showed the presence of a 7 cm hyperfunctional ectopic parathyroid tissue in the postero-superior mediastinum infiltrating oesophagus. Video bronchoscopy revealed an extrinsic compression of the trachea. The patient underwent surgery. Through right postero-lateral thoracotomy and mediastinal pleura incision, the adenoma excision was carried out. The histological examination revealed a potentially malignant parathyroid adenoma with infiltration, without over-reaching the capsule. The surgical treatment resulted in an immediate resolution of the clinical symptomatology. One year later, the follow up was shown no observable relapse with normal calcium and PTH values. Today, the tendency to minimally invasive surgery, an accurate preoperative localization of the adenoma and the use of techniques such as intra-surgical nuclear mapping through manual gamma probes, selective angiography, venous intraoperative dosage of PTH and intra-surgical ultrasound scan. PMID:19537125

  10. Cystic and nodular lung disease.

    PubMed

    Richards, J Caleb; Lynch, David A; Chung, Jonathan H

    2015-06-01

    Diffuse cystic and nodular lung diseases have characteristic imaging findings. The most common causes of cystic lung disease are lymphangioleiomyomatosis and Langerhans cell histiocytosis. Other less common cystic lung diseases include Birt-Hogg-Dube syndrome, lymphocytic interstitial pneumonitis, and light chain deposition disease. Computed tomography is used to differentiate cystic lung disease from emphysema, honeycombing, cavities, and bronchiectasis, which mimic cystic lung disease. Diffuse nodular lung disease are categorized as centrilobular, perilymphatic, and random types. In diffuse nodular lung disease, a specific diagnosis is achieved through a combination of history, physical examination, and imaging findings. PMID:26024606

  11. Single port VATS mediastinal tumor resection: Taiwan experience

    PubMed Central

    Wu, Ching-Yang; Heish, Ming-Ju

    2016-01-01

    Background To present the technique of single-port video-assisted thoracoscopic mediastinal tumor resection, which includes limited thymectomy, extended thymectomy, cyst excision and posterior mediastinal tumor excision, and the early results of resection with the use of this technique. Methods Forty patients with mediastinal tumors were treated with single-port thoracoscopic mediastinal resection at Chang Gung Memorial Hospital between April 2014 and September 2015. The surgical intervention was performed through the fourth or fifth intercostal space at the anterior axillary line. A 5 or 10 mm 30 degree video camera and working instruments were employed simultaneously at this incision site throughout the surgery. Results Among the 40 cases included in the final analysis, 10 extended thymectomies, 7 limited thymectomies, nine cyst excisions and 14 tumor excisions were performed successfully without the need for conversion. For the 40 patients who underwent single-port video-assisted thoracoscopic surgery (VATS), the mean operation time was 97.3±31.2 min and the average blood loss was 29.75±39.77 mL. The average length of the incision wound was 3.22±0.79 cm and the average length of postoperative hospital stay was 3.72±1.63 days. There were no mortalities and mobility was achieved within 30 days postoperatively. Conclusions Our preliminary report suggests that uniportal VATS for mediastinal tumor resection is a promising and safe technique within a short-term period. PMID:27134836

  12. Primary cranial mediastinal hemangiosarcoma in a young dog

    PubMed Central

    2014-01-01

    Primary cranial mediastinal hemangiosarcomas are uncommon tumors. A 30-kg, 2-year-old, intact female German shepherd was presented for evaluation of cachexia and respiratory distress of a few days’ duration. Lateral radiographic projection of the thorax revealed significant pleural effusion. Computed tomography revealed a cranial mediastinal mass effect adjacent to the heart. On surgical exploration, a pedunculated mass attached to the esophagus, trachea, brachiocephalic trunk, left subclavian artery and cranial vena cava without attachment to the right atrium and auricular appendage was removed and debrided by use of blunt dissection and dry gauzes, respectively. Histopathology results described the cranial mediastinal mass as hemangiosarcoma. At 8 months and 5 days post-operatively, the patient died. Primary cranial mediastinal hemangiosarcomas, although a seemingly rare cause of thoracic pathology in young dogs, should be considered in the differential diagnosis for pleural effusion and soft tissue mass effect in the cranial mediastinum. This is the first case report in a dog to describe primary cranial mediastinal hemangiosarcoma. PMID:25089185

  13. Primary cranial mediastinal hemangiosarcoma in a young dog.

    PubMed

    Yoon, Hun-Young; Kang, Hye-Mi; Lee, Mi-Young

    2014-01-01

    Primary cranial mediastinal hemangiosarcomas are uncommon tumors. A 30-kg, 2-year-old, intact female German shepherd was presented for evaluation of cachexia and respiratory distress of a few days' duration. Lateral radiographic projection of the thorax revealed significant pleural effusion. Computed tomography revealed a cranial mediastinal mass effect adjacent to the heart. On surgical exploration, a pedunculated mass attached to the esophagus, trachea, brachiocephalic trunk, left subclavian artery and cranial vena cava without attachment to the right atrium and auricular appendage was removed and debrided by use of blunt dissection and dry gauzes, respectively. Histopathology results described the cranial mediastinal mass as hemangiosarcoma. At 8 months and 5 days post-operatively, the patient died. Primary cranial mediastinal hemangiosarcomas, although a seemingly rare cause of thoracic pathology in young dogs, should be considered in the differential diagnosis for pleural effusion and soft tissue mass effect in the cranial mediastinum. This is the first case report in a dog to describe primary cranial mediastinal hemangiosarcoma. PMID:25089185

  14. Disseminated necrotic mediastinitis spread from odontogenic abscess: our experience

    PubMed Central

    Filiaci, Fabio; Riccardi, Emiliano; Mitro, Valeria; Piombino, Pasquale; Rinna, Claudio; Agrillo, Alessandro; Ungari, Claudio

    2015-01-01

    Summary Aims Deep neck infections are rare but potentially fatal complication of pulpal abscess of the teeth. If an infection can progress rapidly from a toothache to a life threatening infection, then it is critical that dentists be able to recognize the danger signs and identify the patients who are at risk. Mediastinitis is a severe inflammatory process involving the connective tissues that fills the intracellular spaces and surrounds the organs in the middle of the chest. This pathology has both an acute and a chronic form and, in most cases, it has an infectious etiology. This study want to expose the experience acquired in the Oral and Maxillo-facial Sciences Department, Policlinico Umberto I, “Sapienza” University of Rome, regarding two clinical cases of disseminated necrotizing mediastinitis starting from an odontogenic abscess. Methods We report two clinical cases of disseminated necrotic mediastinitis with two different medical and surgical approaches. The radiographic and photographic documentation of the patients was collected in the pre-and post-operatively. All patients underwent a CT scan and MRI. Results Mediastinitis can result from a serious odontogenic abscess, and the extent of its inflammation process must be never underestimated. Dental surgeons play a key role as a correct diagnosis can prevent further increasing of the inflammation process. Conclusions A late diagnosis and an inadequate draining represent the major causes of the elevated mortality rate of disseminated necrotizing mediastinitis. PMID:26330907

  15. [News in cystic fibrosis].

    PubMed

    Delaisi, B

    2013-08-01

    The improvement over the last two decades in the treatment of cystic fibrosis led to an increase in life expectancy approaching 40 years at birth. Logically, the population of adult patients has been increasing and is currently 50% of patients followed in France. These therapeutic advances have justified the establishment in 2003 of a generalized neonatal screening for cystic fibrosis. The latest data of this screening show an incidence of CF of 1/5359 live births, far below the incidence of 1/2500 which was widely accepted twenty years ago. The performance of this screening is currently based on the dosage of trypsin immuno reactive, followed in case of exceeding the threshold of a search of the 30 most common mutations, can detect around 96% of 150 to 200 CF cases every year. Therefore, the possibility of a false negative of the screening cannot be excluded and evocative symptoms of cystic fibrosis, even for children born after 2003, will lead to prescribe a sweat test. While treatments available so far goal consequences of cystic fibrosis, a new therapeutic class to correct the functional defect of the mutated protein, called CFTR modulators, is emerging. Ivacaftor, leader of this new class, belonging to the category of "CFTR potentiator" got its access on the market in September 2012 for patients carrying the G551D mutation. New other molecules, named "CFTR correctors" which can have synergistic effect with ivacaftor and concern patients carrying the most common mutation--DF 508--are under development. PMID:23856023

  16. [US-guided transhepatic drainage of a mediastinal abscess].

    PubMed

    Simón-Yarza, I; Viteri-Ramírez, G; García-Lallana, A; Benito, A

    2014-01-01

    Anastomoses often leak after esophageal surgery; if they are not detected in time, leaks can give rise to complications like fluid collections, superinfections, and mediastinitis. Although these complications usually require surgical treatment, different series of patients successfully treated with conservative or minimally invasive approaches have been reported. We present the case of a patient who developed a mediastinal abscess after epiphrenic diverticulectomy. We treated the abscess with US-guided percutaneous transhepatic drainage to avoid surgical reintervention. Although other cases of a transhepatic approach to thoracic lesions have been reported, to our knowledge this is the first report of this technique in mediastinal abscesses. We recommend that transhepatic drainage be considered a therapeutic option in the management of these complications. PMID:22464798

  17. Cervical necrotizing fasciitis associated with descending necrotizing mediastinitis

    PubMed Central

    2011-01-01

    We report a case of potentially fatal cervical necrotizing fasciitis and descending necrotizing mediastinitis due to deep neck infection in a 66-year-old male patient with no history or evidence of immunocompromising disorders. On admission, he had painful neck movements and the skin over his neck was red, hot and tender. A computerized tomography (CT) scan of his neck and chest showed evidence of air collection in soft tissues. He was treated with broad-spectrum intravenous antibiotics and early massive cervical drainage. Prompt diagnosis by CT of the neck and chest enabled an early surgical treatment of cervical necrotizing fasciitis. Although acute mediastinitis is a fatal infection involving the connective tissues that fill the interpleural spaces and surround the median thoracic organs, an extensive cervicotomy combined with appropriate antibiotics can prevent the need for mediastinal drainage. PMID:22958302

  18. Concurrent cranial mediastinal Blastomyces granuloma and carcinoma with cranial vena caval syndrome in a dog

    PubMed Central

    Evans, Natashia A.; Viviano, Katrina R.

    2015-01-01

    This report describes an unusual progression of blastomycosis in a dog with concurrent mediastinal carcinoma. The dog was evaluated for respiratory distress. Diagnostic results revealed chylothorax and a cranial vena caval thrombus. Histopathology of the cranial mediastinal mass diagnosed mediastinal carcinoma and fungal granuloma. Intercurrent disease may complicate the clinical presentation and clinical course of blastomycosis. PMID:26538674

  19. Bulky mediastinal aspergillosis mimicking cancer in an immunocompetent patient.

    PubMed

    Stern, Jean-Baptiste; Wyplosz, Benjamin; Validire, Pierre; Angoulvant, Adela; Fregeville, Aude; Caliandro, Raffaele; Gossot, Dominique

    2014-10-01

    We describe the case of a previously healthy 42-year-old woman who presented with a chronic cough and occasional night sweats. Radiologic exploration showed a bulky mediastinal mass surrounding the aortic arch, associated with a left subclavicular lymph node and a cerebral round lesion, mimicking a disseminated lung cancer. Surgical left subclavicular and computed tomography-guided mediastinal biopsy specimens showed granulomatous patterns. Mycologic culture of both samples grew Aspergillus flavus. Resolution was obtained after 9 months of oral voriconazole therapy. PMID:25282220

  20. Endobronchial ultrasound for mediastinal staging in lung cancer patients.

    PubMed

    Guarize, Juliana; Pardolesi, Alessandro; Donghi, Stefano; Filippi, Niccolò; Casadio, Chiara; Midolo, Valeria; Petrella, Francesco; Spaggiari, Lorenzo

    2014-01-01

    Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) has changed the way mediastinal staging is performed in lung cancer patients. EBUS-TBNA is probably the most important non-invasive procedure for mediastinal staging and the currently preferred approach in many reference cancer centres worldwide. EBUS-TBNA is a less invasive technique than mediastinoscopy with low morbidity and no mortality and can be performed in an outpatient setting with excellent results. This study describes the technical aspects of EBUS-TBNA and our personal experience with the procedure. PMID:25332380

  1. Video-assisted thoracoscopic surgery for complex mediastinal mass resections

    PubMed Central

    DeMarchi, Lorenzo; Emerson, Dominic A.; Holzner, Matthew L.

    2015-01-01

    Minimally invasive surgery has changed the way operative procedures are performed in many specialties. As surgeons have become progressively facile with these techniques, the opportunities to use them have expanded. In thoracic surgery, many surgeons now use minimally invasive techniques to resect small, uncomplicated pathologies of the mediastinum as well as to perform thymectomy for myasthenia gravis. Experience with these techniques has allowed new knowledge to be gained and expansion of the use of these techniques for more complicated mediastinal pathology. This keynote address will outline the instrumentation and techniques that we have adopted over a decade of using these techniques for more complicated mediastinal pathology. PMID:26693146

  2. Surgical Management of Necrotizing Mediastinitis With Large Aortic Pseudoaneurysm.

    PubMed

    Chevalier, Andrew T; Khaja, Minhaj S; Yang, Bo

    2016-05-01

    We report a patient with necrotizing mediastinitis complicated by a giant retrosternal mycotic pseudoaneurysm and prosthetic valve endocarditis successfully managed with a redo sternotomy under hypothermic circulatory arrest. The approach then included extensive débridement of the mediastinum, replacement of the ascending aorta and aortic arch with selective antegrade cerebral perfusion, redo aortic valve replacement, and wound closure with omental flap and myocutaneous flap. After a 2-year survival, the patient suffered reinfection from hemodialysis. Our approach is also applicable to more common presentations of mediastinitis. PMID:27106461

  3. [Mediastinal lymphatic spread of bronchopulmonary cancer].

    PubMed

    Riquet, M

    1991-01-01

    The mediastinum may be divided into 4 zones divided by the tracheo-bronchial axis in which are situated the lymphatic chains involved in the lymphatic drainage of the lungs. In the upper right zone there are 2 chains which are frequently involved, the right paratracheal chain (PTD) and the tracho-oesophageal chain (TO) and 2 lymphatic chains which are less often involved, the superior right phrenic chain (PH Dt) and the lymphatic chain which crosses the Azygos vein (AZM). In the superior left zone are found 2 chains which are frequently infiltrated: The pre-aortic carotid chain (AO) and the left superior bronchial chain (BSG) and 2 chains which are more rarely involved: The left superior phrenic (PHG) and the chain which crosses the aorta (the minor aorta Azygos; AOmi). At the level of the right and left inferior zones are found important groups of lymphatic ganglia at the intratracheo-bronchial bifurcation (ITB) and of one other part of the tracheo-oesophageal axis, the juxta-oesophageal ganglia (OE) and those of the triangular ligament (LT). The lymph coming from the pulmonary segments crosses the ganglia (LN) of the segments of the lobe and of the hilum before reaching the mediastinum and then at the final stage the lymph nodes situated on the margins of the mediastinum considered as N3 in cancer assessment. This schema is not the rule. In less than 5% of cases the lymph may drain without any lymph node relay either to the subclavicular hollow or to the thoracic duct in the mediastinum. More frequently (in 20-35% cases according to the segment considered) the lymph returns directly to the mediastinum ganglia without relaying through the intrapulmonary ganglia. Finally there are those cases where only the perilobar LN are involved. In these cases it is not necessarily the LN of the lobe drained but sometimes of another pulmonary lobe. These direct paths (N2) confirmed in study of cancer, demand and justify the need for a systematic cure of mediastinal LN. It is

  4. The Cystic Fibrosis Intestine

    PubMed Central

    De Lisle, Robert C.; Borowitz, Drucy

    2013-01-01

    The clinical manifestations of cystic fibrosis (CF) result from dysfunction of the cystic fibrosis transmembrane regulator protein (CFTR). The majority of people with CF have a limited life span as a consequence of CFTR dysfunction in the respiratory tract. However, CFTR dysfunction in the gastrointestinal (GI) tract occurs earlier in ontogeny and is present in all patients, regardless of genotype. The same pathophysiologic triad of obstruction, infection, and inflammation that causes disease in the airways also causes disease in the intestines. This article describes the effects of CFTR dysfunction on the intestinal tissues and the intraluminal environment. Mouse models of CF have greatly advanced our understanding of the GI manifestations of CF, which can be directly applied to understanding CF disease in humans. PMID:23788646

  5. Cervicothoracic cystic dysraphism.

    PubMed

    Valeur, Natalie S; Iyer, Ramesh S; Ishak, Gisele E

    2016-09-01

    Cystic dysraphism of the cervical and upper thoracic spine is very rare. It differs from the much more common lumbosacral dysraphism in appearance and structure, and usually portends a better prognosis due to lack of functional neurological tissue in the dysraphic sac and absent or less severe intracranial anomalies. There is ambiguity in the literature regarding terminology because of the paucity of cases. We present cases of the most common type of cervicothoracic cystic dysraphism and emphasize differences from lumbosacral myelomeningocele. Patient outcome depends on the presence of associated anomalies and whether complete surgical resection is performed. Imaging plays a critical role in surgical planning, screening the central nervous system for additional anomalies, and in the postoperative setting for evaluation of retethering. PMID:27147079

  6. Cystic pulmonary hydatidosis

    PubMed Central

    Sarkar, Malay; Pathania, Rajnish; Jhobta, Anupam; Thakur, Babu Ram; Chopra, Rajesh

    2016-01-01

    Cystic echinococcosis (CE) is a zoonotic parasitic disease caused by the larval stages of the cestode Echinococcus granulosus. Worldwide, pulmonary hydatid cyst is a significant problem medically, socially, and economically. Surgery is the definitive therapy of pulmonary hydatidosis. Benzimidazoles may be considered in patients with a surgical contraindication. This review will focus on pathogenesis, lifecycle, clinical features, and management of pulmonary hydatid disease. PMID:27051107

  7. Epidemiology of Cystic Fibrosis.

    PubMed

    Spoonhower, Kimberly A; Davis, Pamela B

    2016-03-01

    Improved quality of care and rapidly emerging therapeutic strategies to restore chloride transport profoundly impact the epidemiology and pathobiology of cystic fibrosis (CF) in the twenty-first century. CF now serves as a model for chronic illness management, continuous quality improvement via registry data, and a seamless link between basic science research, translational studies, clinical trials, and outcomes research to enable rapid expansion of treatment options. PMID:26857763

  8. Video-assisted thoracic surgery for mediastinal extramedullary haematopoiesis.

    PubMed Central

    Ng, C. S. H.; Wan, S.; Lee, T. W.; Sihoe, A. D. L.; Wan, I. Y. P.; Arifi, A. A.; Yim, A. P. C.

    2002-01-01

    Extramedullary haematopoiesis is a rare cause of an intrathoracic mass. We report a case of posterior mediastinal extramedullary haematopoietic mass in a 50-year-old man who presented with non-specific symptoms and a paravertebral mass on chest X-ray. Diagnosis was achieved by using video-assisted thoracic surgery. Images Figure 1 Figure 2 PMID:12092864

  9. Life-threatening giant mediastinal goiter: a surgical challenge.

    PubMed

    Veronesi, G; Leo, F; Solli, P G; D'Aiuto, M; D'Ovidio, F; Mazzarol, G; Spaggiari, L; Pastorino, U

    2001-06-01

    Mediastinal goiter is a well known benign disease, usually resectable through a cervical approach with minimal morbidity and mortality. Only occasionally a median sternotomy or a lateral thoracotomy may be required. The present case is worthy of presentation because of the exceptional dimension of the disease and the surgical challenge that it presented. In a 72-year-old woman a large intrathoracic goiter of the right thorax caused a severe dyspnoea due to an important contralateral mediastinal shift with compression of the lung, superior vena cava system and trachea. At surgical exploration, through a cervico-sternotomic approach, the mediastinal structures dislocation and the strong adherences between the anomalous neovascularized capsula of the mass and the surrounding structures, complicated the surgical dissection. An accidental lesion of the innominate artery required its reimplantation on the ascending aorta. An immense mass, was finally removed and pathological examination revealed a rare case of neovascularized, pseudosarcomatoid capsula among a benign hyperplastic proliferation. In spite of its benign nature, a giant goiter caused a life-threatening compression of the respiratory tract and lung parenchyma in this patient. The dimension of the lesions, the mediastinal anatomy alterations and the severe intraoperative haemorrhage represented major technical difficulties during surgical resection. PMID:11501515

  10. Invasive Esophageal Candidiasis with Chronic Mediastinal Abscess and Fatal Pneumomediastinum.

    PubMed

    Aghdam, Mohammad Reza F; Sund, Ståle

    2016-01-01

    BACKGROUND Invasive candidiasis is a potential problem for patients receiving long-term immunosuppressive treatment. Psoriatic arthritis is one of many chronic diseases that can be successfully treated with immunosuppressive drugs, in spite of a documented and accepted risk for infectious complications. Critical awareness of possible infection must be part of the surveillance of such patients. CASE REPORT This is the case of a 68-year-old Norwegian male, treated with long-term immunosuppression for psoriatic arthritis, hospitalized with acute subcutaneous and mediastinal emphysema of unknown cause. He died of acute respiratory failure with circulatory collapse shortly after admission. The autopsy revealed mediastinal and subcutaneous emphysema and a mediastinal abscess containing Candida with probable entrance from the esophagus. CONCLUSIONS We consider invasive candidiasis of the esophagus to be the cause of both the chronic abscess and the acute mediastinal emphysema. This case illustrates the importance of awareness of invasive candidiasis as a possible complication in a patient with long-term immunosuppression. PMID:27389822

  11. Invasive Esophageal Candidiasis with Chronic Mediastinal Abscess and Fatal Pneumomediastinum

    PubMed Central

    Aghdam, Mohammad Reza F.; Sund, Ståle

    2016-01-01

    Patient: Male, 68 Final Diagnosis: Invasive esophageal candiasis Symptoms: Chest discomfort Medication: — Clinical Procedure: — Specialty: Infectious Diseases Objective: Unusual clinical course Background: Invasive candidiasis is a potential problem for patients receiving long-term immunosuppressive treatment. Psoriatic arthritis is one of many chronic diseases that can be successfully treated with immunosuppressive drugs, in spite of a documented and accepted risk for infectious complications. Critical awareness of possible infection must be part of the surveillance of such patients. Case Report: This is the case of a 68-year-old Norwegian male, treated with long-term immunosuppression for psoriatic arthritis, hospitalized with acute subcutaneous and mediastinal emphysema of unknown cause. He died of acute respiratory failure with circulatory collapse shortly after admission. The autopsy revealed mediastinal and subcutaneous emphysema and a mediastinal abscess containing Candida with probable entrance from the esophagus. Conclusions: We consider invasive candidiasis of the esophagus to be the cause of both the chronic abscess and the acute mediastinal emphysema. This case illustrates the importance of awareness of invasive candidiasis as a possible complication in a patient with long-term immunosuppression. PMID:27389822

  12. Cystic lesions of the pancreas

    PubMed Central

    Hruban, Ralph H.; Fukushima, Noriyoshi

    2008-01-01

    Summary In contrast to the relatively uniform pathology and the unyielding dismal outcome associated with infiltrating ductal adenocarcinoma of the pancreas, cystic lesions have a broad spectrum of gross and microscopic pathologies, and a range of clinical outcomes. The common cystic lesions of the pancreas are reviewed with emphasis on practical tips for distinguishing between the main entities. PMID:20953247

  13. Mature Cystic Teratoma of Liver

    PubMed Central

    Gupta, Richa; Bansal, Kalpana; Manchanda, Vivek

    2013-01-01

    A four-year-old boy presented with constipation and mild abdominal distention for one year. Radiologic investigations showed a multiloculated cystic lesion in the caudate lobe of liver with focal calcification in the wall. The child underwent laparotomy with marsupialization of the cystic lesion. Histopathologic examination showed mature teratoma of liver. PMID:24040591

  14. Cystic Fibrosis Therapeutics

    PubMed Central

    Ramsey, Bonnie W.

    2013-01-01

    A great deal of excitement and hope has followed the successful trials and US Food and Drug Administration approval of the drug ivacaftor (Kalydeco), the first therapy available that targets the underlying defect that causes cystic fibrosis (CF). Although this drug has currently demonstrated a clinical benefit for a small minority of the CF population, the developmental pathway established by ivacaftor paves the way for other CF transmembrane conductance regulator (CFTR) modulators that may benefit many more patients. In addition to investigating CFTR modulators, researchers are actively developing numerous other innovative CF therapies. In this review, we use the catalog of treatments currently under evaluation with the support of the Cystic Fibrosis Foundation, known as the Cystic Fibrosis Foundation Therapeutics Pipeline, as a platform to discuss the variety of candidate treatments for CF lung disease that promise to improve CF care. Many of these approaches target the individual components of the relentless cycle of airway obstruction, inflammation, and infection characteristic of lung disease in CF, whereas others are aimed directly at the gene defect, or the resulting dysfunctional protein, that instigates this cycle. We discuss how new findings from the laboratory have informed not only the development of novel therapeutics, but also the rationales for their use and the outcomes used to measure their effects. By reviewing the breadth of candidate treatments currently in development, as well as the recent progress in CF therapies reflected by the evolution of the therapeutics pipeline over the past few years, we hope to build upon the optimism and anticipation generated by the recent success of Kalydeco. PMID:23276843

  15. Ultrasound techniques in the evaluation of the mediastinum, part 2: mediastinal lymph node anatomy and diagnostic reach of ultrasound techniques, clinical work up of neoplastic and inflammatory mediastinal lymphadenopathy using ultrasound techniques and how to learn mediastinal endosonography

    PubMed Central

    Jenssen, Christian; Annema, Jouke Tabe; Clementsen, Paul; Cui, Xin-Wu; Borst, Mathias Maximilian

    2015-01-01

    Ultrasound imaging has gained importance in pulmonary medicine over the last decades including conventional transcutaneous ultrasound (TUS), endoscopic ultrasound (EUS), and endobronchial ultrasound (EBUS). Mediastinal lymph node (MLN) staging affects the management of patients with both operable and inoperable lung cancer (e.g., surgery vs. combined chemoradiation therapy). Tissue sampling is often indicated for accurate nodal staging. Recent international lung cancer staging guidelines clearly state that endosonography should be the initial tissue sampling test over surgical staging. Mediastinal nodes can be sampled from the airways [endobronchial ultrasound combined with transbronchial needle aspiration (EBUS-TBNA)] or the esophagus [endoscopic ultrasound fine needle aspiration (EUS-FNA)]. EBUS and EUS have a complementary diagnostic yield and in combination virtually all MLNs can be biopsied. Additionally endosonography has an excellent yield in assessing granulomas in patients suspected of sarcoidosis. The aim of this review in two integrative parts is to discuss the current role and future perspectives of all ultrasound techniques available for the evaluation of mediastinal lymphadenopathy and mediastinal staging of lung cancer. A specific emphasis will be on learning mediastinal endosonography. Part 1 deals with an introduction into ultrasound techniques, MLN anatomy and diagnostic reach of ultrasound techniques and part 2 with the clinical work up of neoplastic and inflammatory mediastinal lymphadenopathy using ultrasound techniques and how to learn mediastinal endosonography. PMID:26623120

  16. [Acute mediastinitis except in a context of cardiac surgery].

    PubMed

    Doddoli, C; Trousse, D; Avaro, J-P; Djourno, X-B; Giudicelli, R; Fuentes, P; Thomas, P

    2010-02-01

    Acute mediastinitis is a life-threatening complication (20 to 40 % of mortality) secondary to oropharyngeal abscesses, neck infections or oesophageal leak spreading into the mediastium. Early diagnosis and optimal therapeutic approach are crucial for patient survival. CT scanning of the cervical and thoracic area is a useful tool for diagnosis and follow-up. Treatment is based on broad-spectrum antibiotherapy, adequate surgery, mediastinal drainage, and treatment of possible organ failure. There is no surgical standardized attitude. Mini-invasive approach could be satisfactory when prompt diagnosis is established and the thoracic drainage is effective. Repeated postoperative CT scanning and close clinical and laboratory monitoring could make an additional thoracotomy a second-line procedure. PMID:20207299

  17. Perforation of esophagus and subsequent mediastinitis following mussel shell ingestion

    PubMed Central

    Park, Il Hwan; Lim, Hyun Kyo; Song, Seung Woo

    2016-01-01

    Esophageal perforation is a very rare occurrence because accidental swallowing of foreign bodies is uncommon in adults. Thus, perforation due to swallowing of a foreign body and subsequent development of mediastinitis is rarely encountered by physicians. We experienced such a case and described an adult male patient who had perforated esophagus after accidentally swallowing a mussel shell. The patient visited our emergency department complaining of painful dysphagia for 4 days. A review of history revealed that he consumed a spicy seafood noodle soup containing mussel shells 4 days ago. Computed tomography (CT) of the chest showed the foreign body in the esophagus and pneumomediastinum was identified. We removed the mussel shell fragment using rigid esophagoscopy; explo-thoracotomy, debridement of mediastinal abscess and irrigation were performed.

  18. Surgical resection of a huge ruptured mature mediastinal teratoma.

    PubMed

    Acharya, Metesh Nalin; De Robertis, Fabio; Popov, Aron-Frederik; Anastasiou, Nikolaos

    2016-09-01

    Usually slow-growing and benign, mature mediastinal teratomas are rare clinical entities. They may be complicated by rupture into the pleural or pericardial spaces, lungs, or bronchi. Complete surgical resection is the treatment of choice and is usually curative. We report the unusual case of a 24-year-old woman presenting 15 weeks postpartum with a huge ruptured mature mediastinal teratoma superinfected with Mycobacterium avium Catastrophic bleeding from the superior vena cava was encountered on mobilization of adhesions attached to it, requiring extracorporeal membrane oxygenator support for control. Histopathological examination confirmed a 12.0 × 7.8 × 4.5-cm differentiated teratoma without malignant transformation. PMID:27440933

  19. Resection of Posterior Mediastinal Tumors by Video Assisted Thoracic Surgery.

    PubMed

    Ali, Taimur Asif; Fatimi, Saulat Hasnain; Naeem, Syed Saad

    2015-07-01

    This case report illustrates successful Video Assisted Thoracic Surgery (VATS) performed on a 45-year-old woman and 52-year-old man presenting with a mass in left and right paravertebral space on the CT scan respectively. VATS has many benefits over traditional open operation (thoracotomy), resulting in less pain and shorten recovery time. However, VATS has higher equipment cost but when an experienced surgeon performs the surgery, better outcomes are achieved. VATS is not common in Pakistan's surgical setup as it is an expensive method of eradicating mediastinal pathologies and not every patient undergoes VATS. The primary objective of presenting these cases is to promote the use of VATS specifically for removal of posterior mediastinal tumors and improve the surgical outcomes. PMID:26208561

  20. Automatic mediastinal lymph node detection in chest CT

    NASA Astrophysics Data System (ADS)

    Feuerstein, Marco; Deguchi, Daisuke; Kitasaka, Takayuki; Iwano, Shingo; Imaizumi, Kazuyoshi; Hasegawa, Yoshinori; Suenaga, Yasuhito; Mori, Kensaku

    2009-02-01

    Computed tomography (CT) of the chest is a very common staging investigation for the assessment of mediastinal, hilar, and intrapulmonary lymph nodes in the context of lung cancer. In the current clinical workflow, the detection and assessment of lymph nodes is usually performed manually, which can be error-prone and timeconsuming. We therefore propose a method for the automatic detection of mediastinal, hilar, and intrapulmonary lymph node candidates in contrast-enhanced chest CT. Based on the segmentation of important mediastinal anatomy (bronchial tree, aortic arch) and making use of anatomical knowledge, we utilize Hessian eigenvalues to detect lymph node candidates. As lymph nodes can be characterized as blob-like structures of varying size and shape within a specific intensity interval, we can utilize these characteristics to reduce the number of false positive candidates significantly. We applied our method to 5 cases suspected to have lung cancer. The processing time of our algorithm did not exceed 6 minutes, and we achieved an average sensitivity of 82.1% and an average precision of 13.3%.

  1. Supradiaphragmatic Hodgkin's disease: significance of large mediastinal masses

    SciTech Connect

    Prosnitz, L.R.; Curtis, A.M.; Knowlton, A.H.; Peters, L.M.; Farber, L.R.

    1980-07-01

    In order to assess the significance of large mediastinal masses in patients with Hodgkin's disease, we analyzed all patients with pathological stage (PS) IA or IIA disease evaluated and treated at Yale between 1969 and 1978. There were 131 such patients treated initially with radical radiotherapy only, combination chemotherapy being reserved for those who failed radiation. Actuarial 5 and 10 year survivals were 95%. The presence of a mediastinal mass regardless of size did not affect survival. Relapse-free survival was 77% at 5 years, 74% at 10 years in the entire group. Patients with any mediastinal involvement had a 65% relapse-free survival, 72% if the mass was < 33% of transverse chest diameter, 55% if the mass was > 33%. These differences are suggestive of a greater tendency of such patients to fail radiotherapy but the differences were not statistically significant. Patients who did fail radiotherapy were for the most part successfully retreated with combined modality therapy (chemotherapy and radiation), accounting for the most part successfully retreated with combined modality therapy (chemotherapy and radiation), accounting for the overall survival of 95%. Only 6 patients died of causes related to Hodgkin's disease and 2 of these deaths were related to combined modality therapy complications. Because of the serious potential long term consequences of combined modality treatment, it should be used with great caution and on an individual basis only in PSIA and IIA patients.

  2. CT of chronic infiltrative lung disease: Prevalence of mediastinal lymphadenopathy

    SciTech Connect

    Niimi, Hiroshi; Kang, Eun-Young; Kwong, S.

    1996-03-01

    Our goal was to determine the prevalence of mediastinal lymph node enlargement at CT in patients with diffuse infiltrative lung disease. The study was retrospective and included 175 consecutive patients with diffuse infiltrative lung diseases. Diagnoses included idiopathic pulmonary fibrosis (IPF) (n = 61), usual interstitial pneumonia associated with collagen vascular disease (CVD) (n = 20), idiopathic bronchiolitis obliterans organizing pneumonia (BOOP) (n = 22), extrinsic allergic alveolitis (EAA) (n = 17), and sarcoidosis (n = 55). Fifty-eight age-matched patients with CT of the chest performed for unrelated conditions served as controls. The presence, number, and sites of enlarged nodes (short axis {ge}10 mm in diameter) were recorded. Enlarged mediastinal nodes were present in 118 of 175 patients (67%) with infiltrative lung disease and 3 of 58 controls (5%) (p < 0.001). The prevalence of enlarged nodes was 84% (46 of 55) in sarcoidosis, 67% (41 of 61) in IPF, 70% (14 of 20) in CVD, 53% (9 of 17) in EAA, and 36% (8 of 22) in BOOP. The mean number of enlarged nodes was higher in sarcoidosis (mean 3.2) than in the other infiltrative diseases (mean 1.2) (p < 0.001). Enlarged nodes were most commonly present in station 10R, followed by 7, 4R, and 5. Patients with infiltrative lung disease frequently have enlarged mediastinal lymph nodes. However, in diseases other than sarcoid, usually only one or two nodes are enlarged and their maximal short axis diameter is <15 mm. 11 refs., 2 figs., 1 tab.

  3. [Curative report of post sternotomy mediastinitis due to bacterofungal infection].

    PubMed

    Katsumata, T; Shimakura, T; Nakano, H; Shimamura, Y; Ishitoya, H

    1994-01-01

    We describe a 72-year-old patient with mediastinitis caused by Methicillin-resistant Staphylococcus aureus (MRSA) and Candida albicans after median sternotomy who was successfully treated with wound closure with pectoral musculocutaneous flap and closed continuous mediastinal irrigation. The irrigation device consisted of two pairs of irrigation and suction tubes which placed upper and lower half of mediastinum respectively, in which high rate irrigation technique (200 ml/h) was employed using 0.01% of Vancomycin hydrochloride as a base agent and additional 0.1% of Povidone-iodine in early phase and 0.01% of Fluconazole in late phase. After 12-days irrigation, the drainage culture turned negative and the wound was healed and tomographically granulated. High rate irrigation with sufficient concentration of antimicrobial agents selected according to each organism sensitivity could eliminate redundant irrigation and contribute to avoid antimicrobial toxication. We experienced also another four cases (two caused by MRSA, two caused by Serratia liquefaciens) treated successfully with this technique. These results led us to believe that continuous mediastinal irrigation technique could be carried out with safe and effectiveness so far as high rate irrigation with low concentration of Povidone-iodine is employed. PMID:8308391

  4. Mediastinal Mass with Hyper-eosinophilia in a Young Boy -A Diagnostic Dilemma

    PubMed Central

    Prem, Shruti; Jacob, Priya Mary; Nair, Rekha Appukuttan

    2016-01-01

    Mediastinal masses in children comprises of a heterogeneous group of tumours. In such cases, biopsy and histological analysis are mandatory for planning of treatment. We have reported an unusual aetiology for a mediastinal mass in a young boy presenting with features of Superior Vena Caval Obstruction (SVCO) who also had marked blood and marrow eosinophilia mimicking Chronic Eosinophilic Leukaemia (CEL). We have also discussed the differential diagnoses of mediastinal tumours with hyper-eosinophilia and possible therapeutic implications.

  5. Giant Chordoma of the Upper Thoracic Spine with Mediastinal Involvement: A Surgical Challenge

    PubMed Central

    Rena, Ottavio; Allegra, Giuliano; Casadio, Caterina; Turello, Davide

    2014-01-01

    Thoracic chordomas are very rare malignant tumours originating from notochordal remnants. These tumours develop within a vertebral body and enlarge involving the mediastinal compartment. Because of their slow-growing attitude, they become symptomatic only when they invade or compress the spinal cord and/or mediastinal organs. We present a rare case of a thoracic spine chordoma presenting with increasing paraparesis with a huge mediastinal component which was surgically debulked to decompress the spinal cord and medistinal organs. PMID:24967050

  6. Giant chordoma of the upper thoracic spine with mediastinal involvement: a surgical challenge.

    PubMed

    Rena, Ottavio; Davoli, Fabio; Allegra, Giuliano; Casadio, Caterina; Turello, Davide

    2014-06-01

    Thoracic chordomas are very rare malignant tumours originating from notochordal remnants. These tumours develop within a vertebral body and enlarge involving the mediastinal compartment. Because of their slow-growing attitude, they become symptomatic only when they invade or compress the spinal cord and/or mediastinal organs. We present a rare case of a thoracic spine chordoma presenting with increasing paraparesis with a huge mediastinal component which was surgically debulked to decompress the spinal cord and medistinal organs. PMID:24967050

  7. Complete transthoracic resection of giant posterior mediastinal goiter: case report and review of surgical strategies

    PubMed Central

    Zhao, Honglin; Ren, Dian; Liu, Yi; Li, Xin; Wu, Yi; Chen, Gang; Chen, Jun

    2016-01-01

    Intrathoracic goiters generally occupy anterior mediastinum, rarely involving the posterior mediastinal space. Reported herein is a 54-year-old female with a giant posterior mediastinal mass that was successfully resected via right posterolateral thoracotomy. The final pathologic diagnosis was giant posterior mediastinal goiter. This patient has done well postoperatively, with no evidence of local recurrence at 12-month follow-up. Related surgical strategies in past publications are summarized. PMID:27217766

  8. Gastrointestinal Manifestations of Cystic Fibrosis

    PubMed Central

    2016-01-01

    Cystic fibrosis has historically been considered a pulmonary disease, but with the increasing life expectancy of these patients, gastrointestinal manifestations are becoming more important. Furthermore, nutritional status is closely linked to pulmonary function and, thus, overall mortality. This article discusses gastrointestinal manifestations (which involve nutritional, pancreatic, hepatobiliary, and, in particular, gastrointestinal tract issues) of cystic fibrosis as well as management of the disease. In addition, the article discusses studies that have been critical to our understanding of gastrointestinal manifestations of cystic fibrosis. PMID:27330503

  9. Chloride impermeability in cystic fibrosis

    NASA Astrophysics Data System (ADS)

    Quinton, Paul M.

    1983-02-01

    Cystic fibrosis is the most common fatal genetic disease affecting Caucasians and is perhaps best characterized as an exocrinopathy involving a disturbance in fluid and electrolyte transport1. A high NaCl concentration in the sweat is characteristic of patients with this disease; the basic physiological reason for this abnormality is unknown. We have microperfused isolated sweat ducts from control subjects and cystic fibrosis patients, and report here results which suggest that abnormally low Cl- permeability in cystic fibrosis leads to poor reabsorption of NaCl in the sweat duct, and hence to a high concentration of NaCl in the sweat.

  10. A case of giant mediastinal cyst initially detected and diagnosed by echocardiography.

    PubMed

    Li, XiaoDong; Wang, XiaoCong; Duan, XiuMei; Xu, Hui

    2015-07-01

    Mediastinal cysts are a group of benign mediastinal lesions, and their diagnoses are primarily depended on x-ray and computed tomography. The development of ultrasound instruments and inspection techniques in recent years has led to the ability to use transthoracic echocardiography to clearly display structures surrounding the heart according to the dark fluid space (blood) of the atrium, ventricles, and large vessels as an acoustic window. This technique offers improved detection rates of mediastinal lesions. We report a case of a mediastinal cyst that was first detected and diagnosed on thoracic echocardiography examination and then confirmed by thoracoscopic surgery and pathological analysis. PMID:25640147

  11. Neutrophils in cystic fibrosis.

    PubMed

    Laval, Julie; Ralhan, Anjali; Hartl, Dominik

    2016-06-01

    Cystic fibrosis (CF) lung disease is characterized by chronic infection and inflammation. Among inflammatory cells, neutrophils represent the major cell population accumulating in the airways of CF patients. While neutrophils provide the first defensive cellular shield against bacterial and fungal pathogens, in chronic disease conditions such as CF these short-lived immune cells release their toxic granule contents that cause tissue remodeling and irreversible structural damage to the host. A variety of human and murine studies have analyzed neutrophils and their products in the context of CF, yet their precise functional role and therapeutic potential remain controversial and incompletely understood. Here, we summarize the current evidence in this field to shed light on the complex and multi-faceted role of neutrophils in CF lung disease. PMID:26854289

  12. [Cystic kidney diseases].

    PubMed

    Zerres, K; Ortiz Brüchle, N

    2012-04-01

    Cystic kidney diseases are clinically and genetically heterogeneous. The most important entities are autosomal-dominant and autosomal-recessive polycystic kidney diseases. The proteins encoded by the involved genes are referred to as cystoproteins, which are located predominantly in the primary cilia. Primary cilia play an important role in cyst formation. Inherited polycystic kidney diseases belong to the increasing number of reported ciliopathies, including several syndromic entities. An exact diagnosis is the basis for medical care and genetic counselling; thus, the diagnostic algorithm should include clinical, ultrasonographic and morphological features of the underlying kidney disease, knowledge about further features and family history. Molecular genetic testing may contribute important information towards a definite diagnosis. PMID:22410941

  13. [Rhinosinusitis in cystic fibrosis].

    PubMed

    Mainz, J G; Gerber, A; Arnold, C; Baumann, J; Baumann, I; Koitschev, A

    2015-11-01

    In cystic fibrosis (CF) mucociliary clearance of the entire respiratory system is impaired. This allows pathogens, such as Pseudomonas aeruginosa to persist and proliferate, which by progressive pulmonary destruction causes 90 % of premature deaths due to this inherited disease. The dramatic improvement in life expectation of patients due to intensive therapy has resulted in the inevitable but variably expressed sinonasal involvement coming into the clinical and scientific focus. Thereby, almost all CF patients reveal sinonasal pathology and many suffer from chronic rhinosinusitis. Recently, the sinonasal niche has been recognized as a site of initial and persistent colonization by pathogens. This article presents the pathophysiological background of this multiorgan disease as well as general diagnostic and therapeutic standards. The focus of this article is on sinonasal involvement and conservative and surgical options for treatment. Prevention of pathogen acquisition is an essential issue in the otorhinolaryngological treatment of CF patients. PMID:26495450

  14. Cystic fibrosis in pregnancy.

    PubMed Central

    Kent, N E; Farquharson, D F

    1993-01-01

    OBJECTIVE: To review the outcomes of pregnancies in women with cystic fibrosis (CF) and to address issues pertinent to the obstetric care of such women. DATA SOURCES: English-language case reports and case series published from 1960 to 1991 identified through a search of MEDLINE and Index Medicus. The terms of reference were "cystic fibrosis" and "pregnancy". Not all the reports reviewed addressed all the outcomes under consideration. STUDY SELECTION: A total of 20 reports citing cases of pregnancy in women with CF. DATA EXTRACTION: Outcomes included the number of spontaneous abortions, pregnancies continued beyond 20 weeks, preterm deliveries, maternal deaths at 6 months and 2 years after delivery and perinatal deaths. Breast-feeding was addressed. Measures to assess the severity of maternal disease included the mean age at diagnosis of CF, weight gain during pregnancy, pulmonary function studies if available and the need for pancreatic enzyme replacement therapy. DATA SYNTHESIS: Of 217 pregnancies in 162 women spontaneous abortion occurred in 10 (4.6%). Pregnancy progressed beyond 20 weeks in 81.6% of cases; 24.3% of the deliveries were preterm. The maternal death rate did not exceed that among age-related women with CF who were not pregnant. The rate of perinatal death was 7.9%. Breast milk was not hypernatremic. Poor outcomes were associated with a weight gain of less than 4.5 kg and a forced vital capacity of less than 50% of the predicted value. CONCLUSIONS: Premature labour and delivery remain a significant risk for pregnant women with CF, contributing to a high rate of perinatal death. Maternal illness and death result from deteriorating pulmonary function. Breast-feeding is not contraindicated. Attention to energy intake and pulmonary function is important. PMID:8374843

  15. Genetics Home Reference: cystic fibrosis

    MedlinePlus

    ... protects the linings of the airways, digestive system, reproductive system, and other organs and tissues. In people with ... experience health problems affecting the respiratory, digestive, and reproductive systems. Most men with cystic fibrosis have congenital bilateral ...

  16. Steady Advances Against Cystic Fibrosis

    MedlinePlus

    ... age 2, he grew up playing everything from football and lacrosse to ice hockey and golf. And ... Who's at Risk? Cystic fibrosis affects males and females from all racial and ethnic groups. It is ...

  17. Cystic lesions of the pancreas

    PubMed Central

    Karoumpalis, Ioannis; Christodoulou, Dimitrios K.

    2016-01-01

    Different types of benign or malignant cystic lesions can be observed in the pancreas. Pancreatic cystic lesions are classified under pathology terms into simple retention cysts, pseudocysts and cystic neoplasms. Mucinous cystic neoplasm is a frequent type of cystic neoplasm and has a malignant potential. Serous cystadenoma follows in frequency and is usually benign. Intraductal papillary mucinous neoplasms are the most commonly resected cystic pancreatic neoplasms characterized by dilated segments of the main pancreatic duct and/or side branches, the wall of which is covered by mucus secreting cells. These neoplasms can occupy the pancreatic head or any part of the organ. Solid pseudopapillary tumor is rare, has a low tendency for malignancy, and is usually located in the pancreatic body or tail. Endoscopic ultrasound with the use of fine-needle aspiration and cytology permits discrimination of those lesions. In this review, the main characteristics of those lesions are presented, as well as recommendations regarding their follow up and management according to recent guidelines. PMID:27065727

  18. Cystic lesions of the pancreas.

    PubMed

    Karoumpalis, Ioannis; Christodoulou, Dimitrios K

    2016-01-01

    Different types of benign or malignant cystic lesions can be observed in the pancreas. Pancreatic cystic lesions are classified under pathology terms into simple retention cysts, pseudocysts and cystic neoplasms. Mucinous cystic neoplasm is a frequent type of cystic neoplasm and has a malignant potential. Serous cystadenoma follows in frequency and is usually benign. Intraductal papillary mucinous neoplasms are the most commonly resected cystic pancreatic neoplasms characterized by dilated segments of the main pancreatic duct and/or side branches, the wall of which is covered by mucus secreting cells. These neoplasms can occupy the pancreatic head or any part of the organ. Solid pseudopapillary tumor is rare, has a low tendency for malignancy, and is usually located in the pancreatic body or tail. Endoscopic ultrasound with the use of fine-needle aspiration and cytology permits discrimination of those lesions. In this review, the main characteristics of those lesions are presented, as well as recommendations regarding their follow up and management according to recent guidelines. PMID:27065727

  19. Pre-operative mediastinal evaluation in primary bronchial carcinoma--a review of staging investigations.

    PubMed Central

    Elliott, J. A.

    1984-01-01

    A review of staging investigations in the preoperative evaluation of mediastinal involvement in primary bronchial carcinoma is presented. The following conclusions are offered as guidelines for the use of mediastinal staging procedures in clinical practice: Surgical staging methods have the over-riding advantage of superior specificity over indirect imaging techniques. Where 67Ga-imaging or CT scanning are not available, routine pre-operative mediastinoscopy or, when appropriate, mediastinotomy will identify most patients with non-resectable disease but this approach entails a high proportion of true negative examinations. Radioisotope ventilation and perfusion lung imaging has no place in the pre-operative staging of lung cancer. Where the techniques are available, 67Ga-imaging and CT scanning have a use in selecting patients for mediastinal exploration. A negative mediastinal 67Ga scan or a negative CT examination suggest that mediastinal exploration will be unrewarding in the vast majority of cases and may be omitted prior to thoracotomy. A positive mediastinal 67Ga scan or the demonstration of abnormal mediastinal nodes by CT is an indication for mediastinal exploration which, if negative should be followed by thoracotomy. PMID:6369288

  20. Autologous hematopoietic stem cell transplantation for mediastinal extramedullary plasmocytoma.

    PubMed

    Abdelkefi, Abderrahmene; Ben Othman, Tarek; Torjman, Lamia; Ladeb, Saloua; Ben Ghorbel, Imed; Lakhal, Amed; Ben Amor, Ramzi; Miled, Mohamed; Kchir, Mohamed-Nidhameddine; Ben Abdeladhim, Abdeladhim

    2003-07-01

    Extramedullary plasmocytoma (EMP) is a rare cell neoplasm most frequently localised in the upper respiratory tract. We report the case of a 43 year-old-man, with an unusual presentation of EMP developing in the mediastinum, two years after a diagnosis of solitary plasmocytoma of the bone which was successfully treated by local irradiation. In this aggressive presentation, we decided to perform an autologous hematopoietic stem cell transplantation. Two months after transplantation, CT scan showed disappearance of the mediastinal mass and immunofixation of the serum was normal. Selected cases of diffuse EMP, could benefit from intensive treatment followed by autologous hematopoietic stem cell transplantation. PMID:14534964

  1. Descending necrotizing mediastinitis: trends in a developing country.

    PubMed

    Chaudhary, Neena; Agrawal, Sanjay; Rai, Anil K

    2005-04-01

    Descending necrotizing mediastinitis is believed to be a rare and serious complication of odontogenic and oropharyngeal infections. It is associated with a high (up to 40%) mortality rate, which can be attributed to delays in diagnosis and inadequate surgical drainage. Between May 1999 and September 2002, we treated 7 cases at our institution in New Delhi, indicating that such fulminating infections are not so rare in developing countries. In our 7 cases, a high index of suspicion and early computed tomography helped us make a rapid diagnosis and initiate prompt treatment, which resulted in a favorable outcome in 6 cases (mortality rate: 14.3%). PMID:15929325

  2. [Early diagnosis of a newborn with a mediastinal mass].

    PubMed

    Flores-Hernández, Salomón Sergio; Ahumada Mendoza, Héctor; Santana-Montero, Blanca Lilia; González Flores, María de Lourdes

    2005-01-01

    Neuroblastoma is an embryonal tumour that evolves from the neural crest cell. This neoplasm may arise at any site in the sympathetic nervous system, including the brain, the cervical region, the posterior mediastinum, the para-aortic sympathetic ganglia, the pelvis, and the adrenal medulla. The clinical presentation in neonatal age is rare and the differential diagnosis includes congenital lung malformations, pneumoniae, atelectasia, etc. The case of a newborn with a mediastinal neuroblastoma is presented. This case illustrates how a patient with an X-ray image compatible with a thoracic tumor should be studied. PMID:16381510

  3. Detection of mediastinitis after heart transplantation by gallium-67 scintigraphy

    SciTech Connect

    Quirce, R.; Serano, J.; Arnal, C.; Banzo, I.; Carril, J.M. )

    1991-05-01

    We report the findings of a patient with post-cardiac transplant mediastinitis detected by {sup 67}Ga-citrate imaging. Fever and leukocytosis were the first clinical signs suggesting infection. The usual diagnostic modalities, including CT and ultrasound, failed to identify the site of infection. A {sup 67}Ga scan showed intense abnormal uptake behind the sternum. The site of uptake was shown by necropsy to be necrotic tissue involving cardiac sutures, pulmonary arteries, and the aorta due to infection with Haemophilus aphrophilus.

  4. A case report of severe ulcerative colitis with mediastinal and subcutaneous emphysema.

    PubMed

    Terasaki, Kei; Okuyama, Yusuke; Ueda, Tomohiro; Matsuyama, Kiichi; Urata, Yoji; Yoshida, Norimasa

    2016-03-01

    A 17-year-old boy developed prominent mediastinal and subcutaneous emphysema while receiving treatment with 5-aminosalicylic acid (5-ASA) and oral corticosteroids for severe ulcerative colitis. We ruled out infection and initiated oral administration of tacrolimus, after which both the underlying disease and mediastinal and subcutaneous emphysema improved. However, he continued to experience repeated bouts of ulcerative colitis, so we ultimately opted for surgical intervention. Although mediastinal and subcutaneous emphysema is rare, it is one of the known extra-intestinal complications and can be particularly concerning. In this patient, mediastinal and subcutaneous emphysema might have been caused by the vulnerability of pulmonary alveolar walls to steroid medication and the increase of pulmonary alveolar pressure with abdominal pain and breath holding. Here, we report a case of inflammatory bowel disease with mediastinal and subcutaneous emphysema, along with a review of the literature. PMID:26947047

  5. Mucolytics in cystic fibrosis.

    PubMed

    Henke, Markus O; Ratjen, Felix

    2007-03-01

    Mucus accumulation in the lower airways is a key feature of cystic fibrosis (CF) lung disease. The major component of mucus in CF is not mucin derived from mucus producing cells but rather pus that includes viscous material such as polymerized DNA derived from degraded neutrophils. This has important implications for mucolytic therapy aiming to improve mucus clearance from the airways, since degradation of mucin may not be a suitable treatment strategy. In addition, thinning of secretions may not always be beneficial, since it may negatively affect certain aspects of mucus transport such as cough clearance. While inhaled N-acetylcysteine has been used as a mucolytic drug in CF for decades, there is little evidence that it has any beneficial effect. Dornase alfa has been shown to reduce pulmonary exacerbations and improve lung function and is currently the only mucolytic agent with proven efficacy in CF. Newer agents targeting other components of CF mucus, such as filamentous actin, are currently in development. Ultimately, drugs that are mucokinetic, which preserve viscoelasticity, rather than mucolytic may prove to be beneficial for CF lung disease in the future. PMID:17419975

  6. PULMONARY CYSTIC ECHINOCOCCOSIS

    PubMed Central

    Santivanez, Saul; Garcia, Hector H.

    2012-01-01

    Pulmonary cystic echinococosis, a zoonosis caused by the larvae of the dog tapeworm Echinococcus granulosus, is considered as a major public health problem in those countries where dogs are used to care for large herds because of the incapacitating effects produced in affected population. The ratio lung:liver involvement is higher in children than in adults. A higher proportion of lung cases are discovered incidentally on a routine x-ray evaluation; the majority of infected people remain asymptomatic until the cyst enlarges sufficiently to cause symptoms. The majority of symptoms are caused by mass effect from the cyst volume; the presence of complications caused by cysts broke changes the clinical presentation; the principal complication is cyst rupture, producing cough, chest pain, hemoptysis, or vomica. Diagnosis is obtained by imaging evaluation (Chest X-ray or CT scan), supported by serology in the majority of cases. Surgery is the main therapeutic approach, having as principal objective, the removal of the parasite, preventing intraoperative dissemination; the use of pre surgical chemotherapy reduces the chances of seeding and recurrence; treatment using benzimidazoles is the preferred treatment when surgery is not available, or complete removal is not feasible PMID:20216420

  7. Cystic Fibrosis Sinusitis.

    PubMed

    Le, Christopher; McCrary, Hilary C; Chang, Eugene

    2016-01-01

    Cystic fibrosis (CF) is an autosomal recessive genetic disorder caused by mutations in the CF transmembrane conductance regulator gene(CFTR) resulting in impaired ion transport. Nearly all people with CF will develop chronic rhino-sinusitis (CRS) and present with the characteristic viscous mucus, impaired mucociliary clearance and chronic inflammation/infection of the sinonasal cavity. While some individuals with CF can appear relatively asymptomatic in terms of their sinus disease, commonly reported symptoms include anosmia, headache, facial pain, nasal obstruction, chronic congestion and nasal discharge. Nasal endoscopy typically reveals mucosal edema, purulent discharge and nasal polyposis. Computed tomography (CT) imaging classically demonstrates the distinguishing findings of sinus hypoplasia or aplasia with generalized opacification, medial bulging of the lateral sinonasal sidewall and a demineralized uncinate process. Current treatment for CF sinusitis includes the use of hypertonic saline, topical and systemic steroids, antibiotics and endoscopic surgery. Research investigating novel therapies designed at targeting the primary defect of CF is showing promise for reversal of CF sinus disease, in addition to potential for disease prevention. PMID:27466844

  8. Cystic Lesions in Autoimmune Pancreatitis.

    PubMed

    Gompertz, Macarena; Morales, Claudia; Aldana, Hernán; Castillo, Jaime; Berger, Zoltán

    2015-01-01

    Autoimmune pancreatitis (AIP) can be chronic or recurrent, but frequently completely reversible after steroid treatment. A cystic lesion in AIP is a rare finding, and it can mimic a pancreatic cystic neoplasm. Difficulties in an exact diagnosis interfere with treatment, and surgery cannot be avoided in some cases. We report the history of a 63-year-old male presenting with jaundice and pruritus. AIP was confirmed by imaging and elevated IgG4 blood levels, and the patient completely recovered after corticosteroid therapy. One year later, he presented with a recurrent episode of AIP with elevated IgG4 levels, accompanied by the appearance of multiple intrapancreatic cystic lesions. All but 1 of these cysts disappeared after steroid treatment, but the remaining cyst in the pancreatic head was even somewhat larger 1 year later. Pancreatoduodenectomy was finally performed. Histology showed the wall of the cystic lesion to be fibrotic; the surrounding pancreatic tissue presented fibrosis, atrophy and lymphoplasmacytic infiltration by IgG4-positive cells, without malignant elements. Our case illustrates the rare possibility that cystic lesions can be part of AIP. These pseudocysts appear in the pancreatic segments involved in the autoimmune disease and can be a consequence of the local inflammation or related to ductal strictures. Steroid treatment should be initiated, after which these cysts can completely disappear with recovery from AIP. Surgical intervention may be necessary in some exceptional cases. PMID:26675058

  9. Using Cystic Fibrosis Therapies for Non-Cystic Fibrosis Bronchiectasis.

    PubMed

    ElMaraachli, Wael; Conrad, Douglas J; Wang, Angela C C

    2016-03-01

    Non-cystic fibrosis bronchiectasis (NCFB) is an increasingly prevalent disease that places a significant burden on patients and health systems globally. Although many of the therapies used to treat NCFB were originally developed as cystic fibrosis (CF) therapies, not all of them have been demonstrated to be efficacious in NCFB and some may even be harmful. This article explores the evidence for which therapeutic strategies used to treat CF have been translated into the care of NCFB. The conclusion is that therapies for adult NCFB cannot be simply extrapolated from CF clinical trials, and in some instances, doing so may actually result in harm. PMID:26857775

  10. Clinical and pathological characteristics of adrenal lymphangioma treated by laparoscopy via a retroperitoneal approach: experience and analysis of 7 cases

    PubMed Central

    Gao, Liang; Zhang, Shu; Wang, Huan; Qiu, Yan; Yang, Lu; Yuan, Jiuhong; Wei, Qiang; Han, Ping

    2015-01-01

    To describe the clinical and pathological characteristics of adrenal lymphangioma (AL) and share our experiences of the treatment of AL with retroperitoneal laparoscopic surgery. All patients pathologically diagnosed with AL were examined. The clinical and pathological characteristics, process of diagnosis, and preparation and treatment of all patients, especially patients treated with laparoscopic surgery, were summarized and retrospectively analyzed. From January 2008 to May 2014, 8 patients underwent adrenalectomies and were diagnosed with AL in our hospital. The median age was 45.5 years. All of these patients experienced a smooth adrenalectomy: 7 performed by laparoscopy via a retroperitoneal approach and 1 performed by open surgery. Five were female and the other 3 were male. These patients had unilateral adrenal lesions. Four were located on the right which to be same as the contralateral. In addition, 1 specimen was assayed by immunohistochemistry (IHC), which revealed positive results for CD31, CD34, Factor VIII-related antigen and D2-40, and negative results for cytokeratin AE1/AE3. During a brief follow up, all patients exhibited favorable results without discomfort. AL is a benign lesion with mild bio-behavior and patients are generally asymptomatic. The use of computerized tomography (CT) combined with enhanced CT has a superior advantage in diagnosis. Laparoscopic adrenalectomies that are performed via a retroperitoneal approach would be a very safe and efficient choice for AL treatment. D2-40 can be considered as a specific IHC marker in the pathological diagnosis of AL. However, pheochromocytoma and adrenal tuberculosis should be ruled out before and during the operation. PMID:26064332

  11. Acute neck cellulitis and mediastinitis complicating a continuous interscalene block.

    PubMed

    Capdevila, Xavier; Jaber, Samir; Pesonen, Pertti; Borgeat, Alain; Eledjam, Jean-Jacques

    2008-10-01

    We report a case of acute neck cellulitis and mediastinitis complicating a continuous interscalene brachial plexus block. A 61-yr-old man was scheduled for an elective arthroscopic right shoulder rotator cuff repair. A continuous interscalene block was done preoperatively and 20 mL of 0.5% bupivacaine and 20 mL of 2% mepivacaine were injected through the catheter. Postoperative analgesia was provided by a continuous infusion of bupivacaine, 0.25% at 5 mL/h for 39 h using a 240-mL elastomeric disposable pump. The day after surgery, the patient complained of neck pain. The analgesic block was not fully effective. He was discharged home. Three days later, the patient was readmitted with neck edema and erythema, fever and fatigue. Neck ultrasonography and computed tomographic scan revealed an abscess of the interscalene and sternocleidomastiod muscles and cellulitis, as well as acute mediastinitis. Two blood cultures and surgical samples were positive for Staphylococcus aureus. The infection was treated with surgery, the site was surgically debrided, and a 2-mo course of vancomycin, imipenem, and oxacilline. The technique of drawing local anesthetic from the bottle and filling the elastomeric pump was the most likely cause of infection. This case emphasizes the importance of strict aseptic conditions during puncture, catheter insertion, and management of the local anesthetic infusate. PMID:18806062

  12. Belatacept and mediastinal histoplasmosis in a kidney transplant patient

    PubMed Central

    Trimarchi, Hernán; Rengel, Tatiana; Andrews, José; Paulero, Matías; Iotti, Alejandro; Forastiero, Agustina; Lombi, Fernando; Pomeranz, Vanesa; Forrester, Mariano; Iriarte, Romina; Agorio, Iris

    2016-01-01

    Background: In transplantation immunosuppression enhances the appearance of opportunist infections. An ideal balance between the prevention of rejection, the lowest risk of infections and the highest rates of graft survival is a continuous challenge. Lower doses of immunosuppression may diminish the risk of infections, metabolic and hemodynamic complications or even of malignancy, but may expose patients to episodes of acute rejection. New drugs are being developed to improve graft survival at the lowest risk of side effects. Belatacept has recently been introduced in kidney transplantation to inhibit the co-ligand signal of T cell stimulation. It is a drug with a safe profile, is well-tolerated and appears to improve long-term survival of kidney grafts. However, there may be an increase in opportunistic infections which may be facilitated by T cell depression, as Aspergillus sp., Cryptococcus neoformans or tuberculosis. Case Presentation: We describe a 59-year-old female who developed fever, clinical wasting and a mediastinal mass 31 months after receiving a living non-related kidney transplant while on belatacept therapy. A mediastinal node biopsy disclosed the presence of Histoplasma capsulatum. Infection successfully resolved after appropriate antifungal treatment. Conclusions: To our knowledge, this is the first reported case of Histoplasma capsulatum in a kidney transplanted patient on belatacept therapy PMID:27152295

  13. [Cystic cancer of the kidney].

    PubMed

    el Moussaoui, A; Dakir, M; Sarf, I; Aboutaeib, R; el Mrini, M; Benjelloun, S

    1997-01-01

    Cystic renal cancer is uncommon and raises real preoperative diagnostic problems, requiring the use of medical imaging, and sometimes even surgery. The authors report 3 cases of cystic renal cancer in 2 men and 1 woman, aged 87, 67 and 20 years, respectively. Three patients presented with the urological triad (haematuria, pain and lumbar mass). Ultrasonography suggested the diagnosis of cystic cancer in all 3 cases. Computed tomography was performed in 2 patients and more precisely confirmed the ultrasound findings. Selective arteriography, performed in one patient, confirmed the hypothesis of malignancy. Surgical exploration resulted in radical total nephrectomy. Histology confirmed the diagnosis of adenocarcinoma. The course was favourable in 2 cases after a follow-up of 4 years. One patient presented a local recurrence with pulmonary metastases 6 months after the operation. A review of the literature illustrates the diagnostic difficulties of this form of renal cancer. PMID:9509236

  14. Congenital cystic adenomatoid malformation in the fetus: natural history and predictors of outcome.

    PubMed

    Miller, J A; Corteville, J E; Langer, J C

    1996-06-01

    Cystic adenomatoid malformation (CCAM) is a rare lesion that often is diagnosed by prenatal ultrasonography. Outcome varies from hydrops and fetal death to resolution before birth. The authors reviewed their 7-year experience with 17 fetuses diagnosed with CCAM by prenatal ultrasonography to determine the natural history of the lesion and to identify factors that might predict outcome. Of the 17 fetuses, five died during intrauterine life (3 terminations, 2 fetal deaths); four of them had hydrops. Twelve fetuses were carried to term. Only one had prenatal intervention: a thoracoamniotic shunt at 24 weeks. All 12 infants survived and underwent resection. Only four required neonatal support (1 extracorporeal membrane oxygenation, 2 ventilator, 1 oxygen). The initial CCAM:chest ratio, degree of mediastinal shift at time of diagnosis, location of CCAM, and age at time of diagnosis did not correlate with outcome. Sonographically predicted pathological type did not correlate with pathological diagnosis after surgery, or with outcome. The only accurate predictors of outcome were presence of hydrops (all died) and decrease in size of CCAM during gestation (all survived). The outcome for fetuses with CCAM may be better than previously recognized. Many of the lesions decrease in size, despite significant mediastinal shift and lung compression at the time of diagnosis. Fetal intervention should be considered only for fetuses with hydrops. Others should be monitored with serial ultrasonography. Parental counseling, especially regarding pregnancy termination, should reflect the positive outcome noted in most cases. PMID:8783108

  15. [Historical compilation of cystic fibrosis].

    PubMed

    Navarro, Salvador

    2016-01-01

    Cystic fibrosis is the most common life-shortening recessively inherited disorder in the Caucasian population. The genetic mutation that most frequently provokes cystic fibrosis (ΔF508) appeared at least 53,000years ago. For many centuries, the disease was thought to be related to witchcraft and the "evil eye" and it was only in 1938 that Dorothy H. Andersen characterized this disorder and suspected its genetic origin. The present article reviews the pathological discoveries and diagnostic and therapeutic advances made in the last 75 years. The review ends with some considerations for the future. PMID:26070393

  16. Diagnostic Testing in Cystic Fibrosis.

    PubMed

    Brewington, John; Clancy, J P

    2016-03-01

    Cystic Fibrosis (CF) is a rare, multisystem disease leading to significant morbidity and mortality. CF is caused by defects in the cystic fibrosis transmembrane conductance regulator protein (CFTR), a chloride and bicarbonate transporter. Early diagnosis and access to therapies provides benefits in nutrition, pulmonary health, and cognitive ability. Several screening and diagnostic tests are available to support a diagnosis. We discuss the characteristics of screening and diagnostic tests for CF and guideline-based algorithms using these tools to establish a diagnosis. We discuss classification and management of common "diagnostic dilemmas," including the CFTR-related metabolic syndrome and other CFTR-associated diseases. PMID:26857766

  17. Mediastinal mass and pericardial tamponade in a renal transplant recipient: A rare case of nocardia infection

    PubMed Central

    Salazar, Maria Nieva; Wray, Dannah; Denlinger, Chadrick; Srinivas, Titte; Thomas, Beje; Posadas, Aurora

    2013-01-01

    Patient: Female, 30 Final Diagnosis: Nocardiosis Symptoms: Cardiac tamponade • cough • dyspnea • hoarseness • mediastinal mass • pericardial effusion • short of breath Medication: — Clinical Procedure: — Specialty: Transplantology Objective: Rare disease Background: Nocardia infections can complicate solid organ transplantation. The usual clinical presentations include pulmonary infiltrates with or without cavitation and subcutaneous and brain abscesses. We report an unusual case of nocardia infection in a kidney transplant recipient that presented as mediastinal mass and was associated with pericardial tamponade. Case Report: A 30 year old African American renal transplant recipient presented with cough, hoarseness and shortness of breath nine months after kidney transplantation. She received basiliximab perioperatively and her maintenance immunosuppression included tacrolimus, mycophenolate mofetil and prednisone. Computed tomography (CT) showed a large mediastinal mass with a large pericardial effusion. An echocardiogram revealed collapse of the right ventricle consistent with tamponade. We performed emergent pericardiocentesis to treat the tamponade. A mediastinoscopic biopsy of the mediastinal mass was done to establish a diagnosis. The mediastinal biopsy confirmed the growth of Nocardia. After 2 weeks of imipenem and 6 weeks of linezolid, there was marked radiographic improvement in the size of the mediastinal mass. Conclusions: We report a rare case of a large mediastinal mass associated with pericardial tamponade from nocardia infection in a renal transplant recipient. An invasive approach may be necessary to obtain tissue diagnosis to direct treatment in these cases. Prompt and appropriate medical therapy leads to marked radiographic improvement. PMID:23940824

  18. The place of clinical features and standard chest radiography in evaluation of mediastinal masses.

    PubMed

    Adegboye, V O; Brimmo, A I; Adebo, O A; Ogunseyinde, O O; Obajimi, M O

    2003-06-01

    One hundred and five primary mediastinal masses were seen between 1975 and 1998, at the Cardiothoracic surgical Unit of the University College Hospital Ibadan. These were studied to establish the importance of clinical features and plain chest radiography in preoperative evaluation of these masses. The sources of information were unit's record, cancer registry and the patients case note. The age range of the 75 male patients was 3 to 80 years (mean 35.2 +/- 22.1 year) and for the 30 female patients was 2.5 to 70 years (mean 30.9 +/- 18.6 years). Anterosuperior mediastinal masses were the most common (63.8%) followed by posterior mediastinal masses (22.9%). Middle mediastinal masses made up the remaining (13.3%). The most common primary mediastinal tumour was lymphoma (21.9%) next to which were thymus gland neoplasia and thymus cysts/hyperplasia (18.1%). Endocrine tumours (mainly goiters) constituted 17.1% of the masses. On the whole, 45 (42.9%) of the mediastinal tumours were malignant and 60 (57.1%) were benign. Eighty one patients (77.1%) were symptomatic at presentation, 24 patients (22.9%) were asymptomatic. Malignancy was more associated with symptoms in this series (82.2%) and benign lesion were more frequently (70.8%) asymptomatic. Cervical lymphadenopathy (78.6%), was more frequent in patients with malignant lesion. Tracheal deviation (60.3%) and neurological signs (78.9%) were more frequent in benign disease. Superior vena cava syndrome was more frequently associated with primary malignant mediastinal tumours. All masses (100%) were visualized on plain chest x-ray. On the basis of clinical features and chest x-ray, majority of patients (76.2%) with primary mediastinal masses had exploratory thoracotomy, sternotomy or biopsy of their mediastinal mass. PMID:14529228

  19. Accidental fracture of EBUS-TBNA needle during sampling of an enlarged mediastinal lymph node

    PubMed Central

    Tariq, Syed Mohammad

    2016-01-01

    Endobronchial ultrasounded-guided transbronchial needle aspiration (EBUS-TBNA) for sampling enlarged hilar and mediastinal lymph nodes, and mediastinal tumours is a safe procedure and can be performed under sedation and local anaesthesia. Rare complications of this procedure include haemorrhage, pneumothorax, pneumonia, mediastinitis and pericarditis (Asano F et al., Complications associated with endobronchial ultrasound-guided transbronchial needle aspiration: a nationwide survey by the Japan Society for Respiratory Endoscopy. Respir Res 2013;14:50). I report an unexpected fracture of the tip of the EBUS-TBNA needle as it hit a bronchial cartilage ring during attempted insertion into a subcarinal node. PMID:27099769

  20. [Mediastinal tracheotomy in cervicofacial oncologic surgery. Contribution of the pectoralis major myocutaneous flap].

    PubMed

    Hamoir, M; Calteux, N; Robillard, T; Remacle, M; De Coninck, A; Van Den Eeckhaut, J

    1985-01-01

    Four cases of anterior mediastinal tracheostomy with myocutaneous Pectoralis Major flap are described. Resection of the manubrium, clavicular heads and the cartilages of the first and second ribs is necessary. The use of myocutaneous Pectoralis Major flap give good functional results. No infection or hemorrhage of mediastinal great vessels occurred. The indications of Anterior mediastinal tracheostomy are discussed. Stomal and peristomal recurrences following laryngectomy for carcinoma represents the indication of choice. Stomal recurrences are difficult to treat and the prognostic is poor. Prevention of the stomal recurrences is discussed. PMID:4014936

  1. A Rare Case of Primary Anterior Mediastinal Yolk Sac Tumor in an Elderly Adult Male

    PubMed Central

    Nakhla, Sammy G.; Sundararajan, Srinath

    2016-01-01

    Mediastinal germ cell tumors are extragonadal germ cell tumors (EGGCTs) commonly seen in children and young adults. They are more common in men. Clinically they are classified as teratomas, seminomas, and nonseminomatous germ cell tumors. Primary mediastinal yolk sac neoplasm is an extremely rare tumor. We present here a very rare case of primary yolk sac tumor of the anterior mediastinum in a 73-year-old male. Mediastinal germ cell tumors have a worse prognosis than gonadal germ cell tumors. Chemotherapy followed by adjuvant surgery improves overall response in EGGCTs. However, comorbidities can render treatment with chemotherapy and surgery challenging in elderly patients. PMID:27144043

  2. ANESTHETIC MANAGEMENT OF A PATIENT WITH CENTRAL AIRWAY COMPRESSION DUE TO POSTERIOR MEDIASTINAL MASS.

    PubMed

    Sulen, Nina; Petani, Barbara; Bacić, Ivan; Morović, Domagoj

    2016-03-01

    Patients with mediastinal masses present unique challenge to anesthesiologists. Patients with anterior mediastinal masses have well documented cases of respiratory or cardiovascular collapse during anesthesia and in postoperative period. Masses in the posterior mediastinum have been traditionally regarded to carry a significantly lower risk of anesthesia related complications but cases of near fatal cardiorespiratory complications have been reported. We describe anesthetic management of a patient with posterior mediastinal mass compressing the trachea and the left main bronchus presented for left thoracotomy and tumor excision. The patient experienced pain and cough, and exhibited positional dyspnea. Airway was successfully secured with awake nasotracheal intubation and placement of single lumen endobronchial tube. PMID:27276782

  3. Cystic pineocytoma--case report.

    PubMed

    Momozaki, N; Ikezaki, K; Abe, M; Fukui, M; Fujii, K; Kishikawa, T

    1992-03-01

    Pineocytoma and pineoblastoma, originating from pineal parenchyma, are rare and usually solid. An unusual case of totally cystic pineocytoma in a 37-year-old female is reported. The tumor showed neuronal differentiation and had a good outcome. Prominent calcification associated with pineocytoma and pineoblastoma is an useful finding to differentiate these from benign pineal cysts. PMID:1377802

  4. Cutaneous manifestations of cystic fibrosis.

    PubMed

    Bernstein, Megan L; McCusker, Meagen M; Grant-Kels, Jane M

    2008-01-01

    Cystic fibrosis is an autosomal recessive disease reported in 1 in 2500 live births in Northern American and Northern European Caucasian populations. Classic disease findings include chronic bacterial infection of airways and sinuses, malabsorption of fat, infertility in men, and elevated concentrations of chloride in sweat. Less well-recognized findings associated with cystic fibrosis include cutaneous findings, which can be primary or secondary manifestations of the disease process. Patients demonstrate more atopic and drug hypersensitivity reactions than the general population, but have similar rates of urticaria compared with the general population. In atypical presentations of cystic fibrosis, the nutrient deficiency dermatitis of the disease may aid with diagnosis, and notably can be the presenting sign. Other dermatologic manifestations of cystic fibrosis include early aquagenic skin wrinkling and cutaneous vasculitis, which can be associated with arthralgias. Familiarity with the nutrient deficiency dermatitis of this entity may play a role in the timely diagnosis of the disease, and the other cutaneous findings add to our understanding of the protean nature of its manifestations. PMID:18429769

  5. [Therapeutic update in cystic fibrosis].

    PubMed

    Durupt, S; Nove Josserand, R; Durieu, I

    2014-06-01

    We present the recent therapeutic advances in the cystic fibrosis care. It concerns improvements in symptomatic treatment with the development of dry powder inhaled antibiotics that improved quality of life, and innovative treatments namely the modulators of the cystic fibrosis transmembrane protein conductance regulator (CFTR), molecules which act specifically at the level of the defective mechanisms implied in the disease. The life expectancy of cystic fibrosis patients born after 2000, is estimated now to be about 50 years. This improvement of survival was obtained with the organization of the care within the specialized centers for cystic fibrosis (Centre de ressource et de compétences de la mucoviscidose) and remains still based on heavy symptomatic treatments. Dry powder inhaled antibiotics constitute a significant time saving for patients to whom all the care can achieve two hours daily. Since 2012, the modulators of CFTR, molecules allowing a pharmacological approach targeted according to the type of the mutations, allows a more specific approach of the disease. Ivacaftor (Kalydeco(®)) which potentialises the function of the CFTR protein expressed on the cellular surface is now available for patients with the G551D mutation. Lumacaftor is going to be tested in association with ivacaftor in patients with the F508del mutation, that is present in at least 75% of the patients. The ataluren which allows the production of a functional protein CFTR in patients with a no sense mutation is the third representing of this new therapeutic class. We presently have numerous symptomatic treatments for the cystic fibrosis care. The development of CFTR modulators, today available to a restricted number of patients treated with ivacaftor represents a very promising therapeutic avenue. It will represent probably the first step to a personalized treatment according to CFTR genotype. PMID:24309546

  6. Nonsurgical resolution of caudal mediastinal paraesophageal abscess in a cat

    PubMed Central

    JUNG, Joohyun; CHOI, Mincheol

    2014-01-01

    A one-year-old, castrated male domestic short hair cat was admitted with a history of anorexia, regurgitation and pyrexia for two days. Fever and leukocytosis were identified. There were a large soft tissue density oval mass in the caudal mediastinum on thoracic radiographs, a fluid-filled oval mass in the caudal mediastinum on ultrasonography, and left-sided and ventrally displaced and compressed esophagus on esophagram. On esophageal endoscopy, there were no esophageal abnormalities. CT findings with a fluid filled mass with rim enhancement indicated a caudal mediastinal paraesophageal abscess. The patient was treated with oral antibiotics, because the owner declined percutaneous drainage and surgery. The patient was admitted on emergency with severe respiratory distress; and ruptured abscess and deteriorated pleuropneumonia were suspected. With intensive hospitalization care and additional antibiotic therapy, the patient had full recovery. PMID:25648207

  7. [Kodamaea ohmeri infection in a newborn with a mediastinal mass].

    PubMed

    Alvarado Socarras, Jorge; Rojas Torres, Juan P; Vargas Soler, José A; Guerrero, Carlos

    2016-10-01

    Invasive fungal infections are a considerable cause of morbidity, mortality, increased hospital stay durations, and high health care costs, during neonatal period. In this period, the premature infants are the most affected. Candida species are the leading cause of invasive fungal infections. The majority of neonatal Candida infections are caused by C. albicans, C. parapsilosis, C. glabrata and C. tropicalis, although other fungus species are being reported. One such emerging pathogen is K. ohmeri. This organism has been reported as a pathogen in the neonatal period, principally in premature infants. The risk factors associated with fungal infection are central line, immunosuppression, prolonged hospital stay, endotracheal intubation and exposure to antibiotics. We present a term baby with a mediastinal mass, who required several procedures, as pericardiocentesis, central catheters, mechanical ventilation, antibiotics. During his evolution, he presented infection by K. ohmeri. The baby was treated with amphotericin B, with satisfactory clinical course. PMID:27606654

  8. Nonsurgical resolution of caudal mediastinal paraesophageal abscess in a cat.

    PubMed

    Jung, Joohyun; Choi, Mincheol

    2015-04-01

    A one-year-old, castrated male domestic short hair cat was admitted with a history of anorexia, regurgitation and pyrexia for two days. Fever and leukocytosis were identified. There were a large soft tissue density oval mass in the caudal mediastinum on thoracic radiographs, a fluid-filled oval mass in the caudal mediastinum on ultrasonography, and left-sided and ventrally displaced and compressed esophagus on esophagram. On esophageal endoscopy, there were no esophageal abnormalities. CT findings with a fluid filled mass with rim enhancement indicated a caudal mediastinal paraesophageal abscess. The patient was treated with oral antibiotics, because the owner declined percutaneous drainage and surgery. The patient was admitted on emergency with severe respiratory distress; and ruptured abscess and deteriorated pleuropneumonia were suspected. With intensive hospitalization care and additional antibiotic therapy, the patient had full recovery. PMID:25648207

  9. Trans-mediastinal gunshot wounds: are "stable" patients really stable?

    PubMed

    Nagy, Kimberly K; Roberts, Roxanne R; Smith, Robert F; Joseph, Kimberly T; An, Gary C; Bokhari, Faran; Barrett, John

    2002-10-01

    Gunshot wounds that traverse the mediastinum frequently cause serious injury to the cardiac, vascular, pulmonary, and digestive structures contained within. Most patients present with unstable vital signs signifying the need for emergency operation. An occasional patient will present with stable vital signs. Work-ups for such a patient may range from surgical exploration to radiographic and endoscopic testing to mere observation. We report our experience with diagnostic work-up of the stable patient with a transmediastinal gunshot wound. All stable patients who present to our urban level I trauma center following a transmediastinal gunshot wound undergo diagnostic work-up consisting of chest radiograph, cardiac ultrasound, angiography, esophagoscopy, barium swallow, and bronchoscopy. The work-up is dependent on the trajectory of the missile. Information on these patients is kept in a prospective database maintained by the trauma attending physicians. This database was analyzed and comparisons were made using Student's t-test and the Fisher exact c2 as appropriate. Over a 68-month period, 50 stable patients were admitted following a transmediastinal gunshot wound. All of these patients had a chest radiograph followed by one or more of the above tests. 8 patients (16%) were found to have a mediastinal injury (4 cardiac, 3 vascular, and 1 tracheo-esophageal) requiring urgent operation (group 1). The remaining 42 patients (84%) did not have a mediastinal injury (group 2). There was no difference between groups with respect to blood pressure, pulse, respiratory rate, pH, base deficit, or initial chest tube output. There was one death in each group, and three complications in group 2. Patients may appear stable following a transmediastinal gunshot wound, even when they have life-threatening injuries. There is no difference in vital signs, blood gas, or hemothorax to indicate which patients have serious injuries. We advocate continued aggressive work-up of these patients

  10. Mediastinal Desmoid Tumor With Remarkably Rapid Growth: A Case Report.

    PubMed

    Lee, Joon Hyung; Jeong, Jae Seok; Kim, So Ri; Jin, Gong Yong; Chung, Myoung Ja; Kuh, Ja Hong; Lee, Yong Chul

    2015-12-01

    Desmoid tumors (DTs) are a group of rare and benign soft tissue tumors that result from monoclonal proliferation of well-differentiated fibroblasts. Since DTs tend to infiltrate and compress adjacent structures, the location of DTs is one of the most crucial factors for determining the severity of the disease. Furthermore, DTs can further complicate the clinical course of patients when the growth is remarkably rapid, especially for DTs occurring in anatomically critical compartments, including the thoracic cavity.The authors report a case of a 71-year-old man with a known mediastinal mass incidentally detected 4 months ago, presenting dyspnea with right-sided atelectasis and massive pleural effusion. Imaging studies revealed a 16.4 × 9.4-cm fibrous mass with high glucose metabolism in the anterior mediastinum. The mass infiltrated into the chest wall and also displaced the mediastinum contralaterally. Interestingly, the tumor had an extremely rapid doubling time of 31.3 days.En bloc resection of the tumor was performed as a curative as well as a diagnostic measure. Histopathologic examination showed spindle cells with low cellularity and high collagen deposition in the stroma. Immunohistochemical staining was positive for nuclear β-catenin. Based on these pathologic findings, the mass was diagnosed as DT. After surgery, there has been no evidence of recurrence of disease in the patient.This patient presents a mediastinal DT with extremely rapid growth. Notably, the doubling time of DT in our case was the shortest among reported cases of DT. Our experience also highlights the benefits of early interventional strategy, especially for rapidly growing DTs in the thoracic cavity. PMID:26717381

  11. Therapeutic Outcomes of Pectoralis Major Muscle Turnover Flap in Mediastinitis

    PubMed Central

    Bagheri, Reza; Tashnizi, Mohammad Abbasi; Haghi, Seyed Ziaollah; Salehi, Maryam; Rajabnejad, Ata’ollah; Safa, Mohsen Hatami Ghale; Vejdani, Mohammad

    2015-01-01

    Background This study aimed to evaluate the therapeutic results and safety of pectoralis major muscle turnover flaps in the treatment of mediastinitis after coronary artery bypass grafting (CABG) procedures. Methods Data regarding 33 patients with post-CABG deep sternal wound infections (DSWIs) who underwent pectoralis major muscle turnover flap procedures in the Emam Reza and Ghaem Hospitals of Mashhad, Iran were reviewed in this study. For each patient, age, sex, hospital stay duration, remission, recurrence, and associated morbidity and mortality were evaluated. Results Of the 2,447 CABG procedures that were carried out during the time period encompassed by our study, DSWIs occurred in 61 patients (2.5%). Of these 61 patients, 33 patients (nine females [27.3%] and 24 males [72.7%]) with an average age of 63±4.54 years underwent pectoralis major muscle turnover flap placement. Symptoms of infection mainly occurred within the first 10 days after surgery (mean, 10.24±13.62 days). The most common risk factor for DSWIs was obesity (n=16, 48.4%) followed by diabetes mellitus (n=13, 39.4%). Bilateral and unilateral pectoralis major muscle turnover flaps were performed in 20 patients (60.6%) and 13 patients (39.4%), respectively. Complete remission was achieved in 25 patients (75.7%), with no recurrence in the follow-up period. Four patients (12.1%) needed reoperation. The mean hospitalization time was 11.69±6.516 days. Four patients (12.1%) died during the course of the study: three due to the postoperative complication of respiratory failure and one due to pulmonary thromboembolism. Conclusion Pectoralis major muscle turnover flaps are an optimal technique in the treatment of post-CABG mediastinitis. In addition to leading to favorable therapeutic results, this flap is associated with minimal morbidity and mortality, as well as a short hospitalization time. PMID:26290837

  12. Idiopathic tension mediastinal emphysema cured by video-assisted thoracic surgery: a case report

    PubMed Central

    Li, Xiao-Ping; Zhang, Liang; Li, Lei; Yang, Bo; Zhang, Wei-Dong

    2015-01-01

    Mediastinal emphysema is a status of gas retention in mediastinum, usually caused by airway system injury or esophagus rupture. Healthy people without trauma and basic disease emerging unexplained mediastinal emphysema are called idiopathic mediastinal emphysema. While tension pneumomediastinum is an exceptional and potentially lethal condition, the increased intramediastinal pressure with a severe oppression of heart, vena cava and pulmonary vein leads to impaired central venous return through the cavae system, restricted right heart diastolic filling, and collapse of the cardiac chambers, resulting in reduced stroke volume, cardiac output and dysaemia. This young people-prone disease was initially reported by Hamman in 1939 and usually has a good prognosis so that no special treatments were needed. In this study, however, we presented a young boy with idiopathic tension mediastinal emphysema combined with dysaemia and respiratory failure. Fortunately, he was finally successful treated by VATS after heteropathy and incision on suprasternal fossae. PMID:26770618

  13. Mediastinal stabilization by an expansion prosthesis in postoperative congenital diaphragmatic hernia with severe pulmonary hypoplasia.

    PubMed

    Becmeur, F; Horta, P; Christmann, D; Donato, L; Livolsi, A; de Geeter, B; Sauvage, P

    1995-10-01

    The authors illustrate a case of right neonatal congenital diaphragmatic hernia (CDH) of Bochdalek with major pulmonary hypoplasia and postoperative massive mediastinal displacement to the right, leading to tracheal compression and recurrent respiratory distress at every attempt to wean the infant from the ventilator. Mediastinal stabilization was obtained by placing an expansion prosthesis (of the cutaneous type) in the right hemithorax, to prevent mediastinal obstruction of the main airways. This procedure may prove to be an original and efficient solution for the critical postoperative respiratory obstruction due to severe pulmonary hypoplasia and mediastinal mass effect in the newborn with CDH. However, this may not prevent progressive scoliosis, a serious problem frequently seen in the absence of one lung. PMID:8555135

  14. Mediastinitis and Mycotic Aneurysm of the Aorta after Orthotopic Heart Transplantation

    PubMed Central

    Anthuber, Matthias; Kemkes, Bernhard M.; Kreuzer, Ekkehard; Gokel, Michael; Schuetz, Albert; Kugler, Christian; Sudhoff, Frank

    1991-01-01

    After cardiac transplantation, bacterial mediastinitis is a rare but dangerous early complication. Of the 113 patients who underwent heart or heart-lung transplantation at our hospital from August 1981 to April 1989, 8 developed purulent mediastinitis. Treatment involved surgical débridment, local irrigation, drainage, and high-dose systemic antibiotics. No patient died of an acute mediastinal infection. In 2 cases, however, chronic mediastinitis led to the formation of a huge mycotic aneurysm of the ascending aorta. Eleven days after surgical intervention for rupture, 1 patient died of aneurysmal rerupture; the 2nd patient remains well 16 months after prosthetic replacement of the ascending aorta and reconstruction of the necrotic proximal portion of the left coronary artery with a saphenous vein patch. (Texas Heart Institute Journal 1991;18:186-93) Images PMID:15227478

  15. Disseminated cryptococcosis presenting as mediastinal and hilar lymphadenopathy in an immunocompetent patient

    PubMed Central

    Oki, Masahide; Saka, Hideo; Kajikawa, Shigehisa; Murakami, Ayuka; Ishida, Akane

    2016-01-01

    Abstract We herein report a rare case of disseminated cryptococcosis presenting as mediastinal and hilar lymphadenopathy in a young immunocompetent man. A previously healthy 26‐year‐old man presented with persistent headache and nonproductive cough. Chest computed tomography indicated mediastinal and hilar lymphadenopathy. Cryptococcal lymphadenitis and meningitis was confirmed by endobronchial ultrasound‐guided transbronchial needle aspiration and central spinal fluid examination, respectively. He received liposomal amphotericin B and flucytosine followed by fluconazole and finally improved. PMID:27512567

  16. Ultrasound-guided biopsy as a diagnostic aid in three horses with a cranial mediastinal lymphosarcoma.

    PubMed

    De Clercq, D; van Loon, G; Lefère, L; Deprez, P

    2004-06-01

    An ultrasound examination of the thorax of three horses which were performing poorly or had mild signs of colic showed that they had a cranial mediastinal mass and a pleural effusion. A cytological examination of the pleural fluid showed that it did not contain neoplastic cells. A histological examination of an ultrasound-guided core biopsy of the cranial mediastinal mass showed that in each of the three horses it was a lymphosarcoma. PMID:15214516

  17. Efficacy of antimicrobial activity of slow release silver nanoparticles dressing in post-cardiac surgery mediastinitis.

    PubMed

    Totaro, Pasquale; Rambaldini, Manfredo

    2009-01-01

    We report our preliminary experience in post-cardiac surgery mediastinitis using a recently introduced silver-releasing dressing claiming prompt antibacterial activity. Acticoat, a silver nanoparticles slow release dressing was used in four patients with documented post-cardiac surgery mediastinitis and persistently positive microbiological cultures despite vacuum-assisted closure (VAC) therapy. In all four patients negative cultures were obtained within a maximum of 72 h and patients were discharged within a maximum of 20 days. PMID:18948308

  18. Optimal surgical options for descending necrotizing mediastinitis of the anterior mediastinum.

    PubMed

    Guan, Xin; Zhang, Wei Jie; Liang, Xi; Liang, Xiang; Wang, Feng; Guo, Xiang; Zhou, Yaodong

    2014-09-01

    The mortality rates from descending necrotizing mediastinitis (DNM) are between 25 and 40 % mainly because of delayed diagnosis and inappropriate surgical treatment. This study was undertaken to examine two surgical options for DNM and determine the optimal surgical option for DNM of the anterior mediastinum. Fifteen cases of DNM of the anterior mediastinum, January 2001 and October 2010, were retrospectively reviewed. Eleven were anterosuperior mediastinitis, with infection located above the tracheal bifurcation and four had infections involving the entire anterior mediastinum. Depending on the location of mediastinitis, open drainage of the submandibular and neck abscesses, in addition to other surgical treatments, was performed. If the infection was anterosuperior, transcervical mediastinal drainage or thoracotomy was performed. If the entire anterior mediastinum was involved, necrotic tissue was removed with thoracoscopic via subxiphoid incision, the bilateral pleurae were opened for drainage, and a tunnel connecting the neck incision and the subxiphoid incision through the whole anterior mediastinum was made for drainage. The anterosuperior mediastinitis cases were treated with either transcervical mediastinal drainage (n = 8) or thoracotomy (n = 3). Patients healed after an average of 24.5 and 20.0 days in the hospital, respectively. For the four other cases, one patient died of septic shock, while the other three patients were healed after and an average of 43.3 days in the hospital. Mortality rate was 6.7 %. The surgical procedure used to treat DNM should be selected according to the location of the infection. DNM involving the anterosuperior mediastinum can be treated by transcervical mediastinal drainage. If anterosuperior mediastinitis spreads to the side of the trachea, open thoracotomy is a suitable therapy. If the entire anterior mediastinum is involved, debridement and drainage of the anterior mediastinum should be performed with a thoracoscope via

  19. Disseminated cryptococcosis presenting as mediastinal and hilar lymphadenopathy in an immunocompetent patient.

    PubMed

    Murakami, Yasushi; Oki, Masahide; Saka, Hideo; Kajikawa, Shigehisa; Murakami, Ayuka; Ishida, Akane

    2016-07-01

    We herein report a rare case of disseminated cryptococcosis presenting as mediastinal and hilar lymphadenopathy in a young immunocompetent man. A previously healthy 26-year-old man presented with persistent headache and nonproductive cough. Chest computed tomography indicated mediastinal and hilar lymphadenopathy. Cryptococcal lymphadenitis and meningitis was confirmed by endobronchial ultrasound-guided transbronchial needle aspiration and central spinal fluid examination, respectively. He received liposomal amphotericin B and flucytosine followed by fluconazole and finally improved. PMID:27512567

  20. Controlled Trachea Suspension for Tracheomalacia After Resection of Large Anterior Mediastinal Mass.

    PubMed

    Liu, Zhengcheng; Yang, Rusong; Shao, Feng; Pan, Yanqing

    2015-06-01

    Tracheomalacia is a disorder of the large airways that is often caused by a large anterior mediastinal mass. This study describes 7 patients who underwent controlled trachea suspension as a surgical intervention to prevent severe tracheomalacia and provide potent relief of airway symptoms. All patients recovered well. The results demonstrate this procedure may be safe and effective for resection of a large mediastinal mass compressing the trachea with collapsed segments. PMID:26046890

  1. Pharmacogenetics of cystic fibrosis treatment.

    PubMed

    Carter, Suzanne C; McKone, Edward F

    2016-08-01

    Cystic fibrosis (CF) is genetic autosomal recessive disease caused by reduced or absent function of CFTR protein. Treatments for patients with CF have primarily focused on the downstream end-organ consequences of defective CFTR. Since the discovery of the CFTR gene that causes CF in 1989 there have been tremendous advances in our understanding of the genetics and pathophysiology of CF. This has recently led to the development of new CFTR mutation-specific targeted therapies for select patients with CF. This review will discuss the characteristics of the CFTR gene, the CFTR mutations that cause CF and the new mutation specific pharmacological treatments including gene therapy that are contributing to the dawning of a new era in cystic fibrosis care. PMID:27490265

  2. [Cystic fibrosis and associated complications].

    PubMed

    Schwarz, C; Staab, D

    2015-03-01

    Cystic fibrosis (CF) is an autosomal recessive inherited metabolic disease. The mutation is located on the long arm of chromosome 7. Due to a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, chloride ion transport is reduced across the cell membrane. As a result, the disease can be described as an exocrinopathy. In all organs with exocrine glands, disorders occur in association with the defective chloride transport. The main impact of this defect is manifested in the lungs. Therefore, the most common cause of death is pulmonary disease with respiratory insufficiency due to recurrent infections. Unfortunately, a cure for the disease is still not available. However, new therapies that may affect the CFTR mutation more specifically give new hope for better therapeutic options in the future. The long-term goal of therapy is to develop a causal therapy for all six different mutation classes and thus for about 2000 mutations. PMID:25693903

  3. Etanercept-induced cystic acne.

    PubMed

    Kashat, Maria; Caretti, Katherine; Kado, Jessica

    2014-07-01

    Tumor necrosis factor α antagonists are potent biologics used to treat a variety of autoimmune disorders such as rheumatoid arthritis, ankylosing spondylitis, Crohn disease, psoriasis, and psoriatic arthritis. These medications are known to have many side effects (eg, infusion reactions, cytopenia, risk for infection, heart failure); however, only a few cases of acne vulgaris have been associated with the use of these biologics, particularly infliximab and adalimumab. We report a rare case of etanercept-induced cystic acne. PMID:25101341

  4. [Imaging spinal cord cystic lesions in adults].

    PubMed

    Kremer, S; Bierry, G; Abu Eid, M; Bogorin, A; Koob, M; Zöllner, G; Dietemann, J L

    2007-05-01

    Intrarachidian cystic lesions are frequent, with highly varied causes. They can be classified according to their location into intramedullary cystic lesions and extramedullary cystic lesions. In these two categories, they can then be regrouped according to the tissue from which they develop. MRI is the first-choice examination for the study of the intracanal contents and the differential diagnosis between the various lesions. PMID:17541357

  5. Congenital Cystic Lesions of the Lung: Congenital Cystic Adenomatoid Malformation and Bronchopulmonary Sequestration

    PubMed Central

    Sfakianaki, Anna K; Copel, Joshua A

    2012-01-01

    Congenital cystic lesions of the lung in fetuses are rare. The most common malformations of the lower respiratory tract are congenital cystic adenomatoid malformation and bronchopulmonary sequestration. With the increased use of obstetric ultrasound, cystic lung lesions are detected more often antenatally, which allows for proper planning of peripartum and neonatal management. This article discusses a range of diagnostic and management options. PMID:22866187

  6. Echocardiographic Manifestation of Esophagitis Mimicking a Posterior Mediastinal Mass

    PubMed Central

    Swarup, Supreeya; Kantamneni, Sowmya; Kabir, Sarah; Zeltser, Roman; Makaryus, Amgad N

    2014-01-01

    Incidental extracardiac findings (ECFs) are commonly noted on cardiac imaging. The majority of the ECFs are noticed on computed tomography (CT), cardiac magnetic resonance scanning, and myocardial perfusion imaging. Although transthoracic echocardiography (TTE) is a widely used cardiac modality, there is scarcity of data describing ECF on TTE. ECFs have the potential to alter patient management. We present a rare case of a cystic mass seen in the posterior mediastinum on TTE, which led to further evaluation and diagnosis of esophagitis with ulceration. PMID:25780343

  7. Imaging guided mediastinal percutaneal core biopsy--technique and complications.

    PubMed

    Azrumelashvili, T; Mizandari, M; Magalashvili, D; Dundua, T

    2015-05-01

    165 percutaneous biopsies of anterior, middle and posterior mediastinum lesions were performed to 156 patients. Procedure was guided by US in 40 cases, by CT - in 125 cases. Hydrodissection was used in 5 cases, artificial pneumothorax - in 3 cases in order to avoid transpulmonary needle pass. Post-biopsy CT scan was performed and patients observed for any complications. Adequate tissue for histological diagnosis was obtained in 156 (94.5%) cases at the first attempt; in 9 (5.5%) cases the repeated procedure was needed. No major complications were detected after biopsy procedures; minor complications (pneumothorax, hemothorax and hemophtysis) were detected in 23 (13.9%) cases. No complications were detected after US guided procedures; In 17 (10.3% of all complications) cases pneumothorax, in 4 (2.4%) cases - hemothorax and in 2 (1.2%) cases hemophtisis was detected on CT guided procedures. All hemothorax and hemophtisis and 10 pneumothorax cases happened to be self-limited; in 3 pneumothorax cases aspiration and in 4 cases - pleural drainage was needed. Percutaneous image-guided core biopsy of mediastinal lesions is an accurate and safe procedure, which enables to get the tissue material from all mediastinum compartments. Ultrasound is the most efficient for biopsy guidance, if the target is adequately imaged by it; the advantages of US guidance are: a) possibility of real-time needle movement control b) possibility of real-time blood flow imaging b) noninvasiveness c) cost-effectiveness d) possibility to perform the biopsy at the bedside, in a semiupright position; so, ultrasound is a "Gold Standard" for procedure guidance if the 'target" can be adequately imaged by this technique. If US guidance is impossible biopsy should be performed under CT guidance. Hydrodissection and artificial pneumothorax enables to avoid the lung tissue penetration related complications. Pneumothorax was associated with multiple Needle passes and larger diameter needle use. The safety

  8. POMB/ACE chemotherapy for mediastinal germ cell tumours.

    PubMed

    Bower, M; Brock, C; Holden, L; Nelstrop, A; Makey, A R; Rustin, G J; Newlands, E S

    1997-05-01

    Mediastinal germ cell tumours (MGCT) are rare and most published series reflect the experiences of individual institutions over many years. Since 1979, we have treated 16 men (12 non-seminomatous germ cell tumours and 4 seminomas) with newly diagnosed primary MGCT with POMB/ACE chemotherapy and elective surgical resection of residual masses. This approach yielded complete remissions in 15/16 (94%) patients. The median follow-up was 6.0 years and no relapses occurred more than 2 years after treatment. The 5 year overall survival in the non-seminomatous germ cell tumours (NSGCT) is 73% (95% confidence interval 43-90%). One patient with NSGCT developed drug-resistant disease and died without achieving remission and 2 patients died of relapsed disease. In addition, 4 patients with bulky and/or metastatic seminoma were treated with POMB/ACE. One died of treatment-related neutropenic sepsis in complete remission and one died of relapsed disease. Finally, 4 patients (2 NSGCT and 2 seminomas) referred at relapse were treated with POMB/ACE and one was successfully salvaged. The combination of POMB/ACE chemotherapy and surgery is effective management for MGCT producing high long-term survival rates. PMID:9291802

  9. Mixed Phenotypic Acute Leukemia Presenting as Mediastinal Mass-2 Cases.

    PubMed

    Vardhan, Rig; Kotwal, Jyoti; Ganguli, Prosenjit; Ahmed, Rehan; Sharma, Ajay; Singh, Jasjit

    2016-06-01

    Mixed phenotype acute leukemia symbolizes a very small subset of acute leukemia that simply cannot be allocated as lymphoid or myeloid lineage. The 2008 World Health Organisation classification established stringent standard for diagnosis of mixed phenotype acute leukemia, accentuating myeloperoxidase for myeloid lineage, cytoplasmic CD3 for T lineage and CD19 with other B markers for B lineage obligation. Mixed phenotype leukemia is rare and 3-5 % of acute leukmias of all age groups, is associated with poor outcome with overall survival of 18 months. We wish to present two cases of mixed phenotypic acute leukemia who presented with mediastinal masses, were suspected to be T cell lymphoma/leukemia clinically and radiologically. In one case, tissue diagnosis was given as lymphoma for which treatment was given. These cases show that patients diagnosed as lymphoma on histopathology can be cases of mixed phenotype acute leukemia and varying specific treatment protocols and follow up are required. Awareness of these entities will help in proper diagnosis and treatment. PMID:27408360

  10. Cystic fibrosis presenting with bilateral facial palsy.

    PubMed

    Basu, Anna P; Kumar, Prashant; Devlin, Anita M; O'Brien, Christopher J

    2007-07-01

    A 15-week old male infant presented with bilateral lower motor neuron facial palsy of unknown cause. Subsequently his growth deteriorated and he developed progressively worsening cough and wheeze. A diagnosis of cystic fibrosis was confirmed and hypovitaminosis A detected. Improvement of the facial palsy was noted following standard management of cystic fibrosis including vitamin A supplementation. PMID:17287135

  11. A combination therapy for cystic fibrosis.

    PubMed

    Brodsky, Jeffrey L; Frizzell, Raymond A

    2015-09-24

    The most prevalent form of cystic fibrosis arises from an amino acid deletion in the cystic fibrosis transmembrane conductance regulator, CFTR. A recently approved treatment for individuals homozygous for this mutation combines a chemical corrector, which helps CFTR fold, and a potentiator that increases CFTR channel activity. PMID:26406363

  12. Calcified Cystic Lesion of the Pancreas.

    PubMed

    Li, Tao; Chen, Zhi-Qiang; Meng, Zhi-Xin; Hong, Jian-Guo; Zhi, Xu-Ting

    2016-06-01

    Pancreatic cystic lesion is a relatively uncommon condition with an estimated prevalence of 2 % in the general population. In the past two decades, there has been a dramatic increase in the prevalence of pancreatic cystic lesions because of the widespread use of high-resolution imaging, as well as the aging of the population. Pancreatic cystic lesions cover a wide spectrum of pathology and can range from obviously benign to borderline malignant potential lesions to overt malignancy. Though the presence of mural nodules, septa-like structures, or calcification on imaging examination contributes to the differential diagnosis, preoperatively determining the biological nature of these cystic lesions is sometimes challenging. In this paper, we report a rare case of pancreatic cystic lesion with an egg-shell like calcification. Complete resection was performed and histological examination confirmed the diagnosis of calcified pancreatic pseudocyst. PMID:26992398

  13. Risk factors for mediastinitis after cardiac surgery – a retrospective analysis of 1700 patients

    PubMed Central

    Diez, Claudius; Koch, Daniel; Kuss, Oliver; Silber, Rolf-Edgar; Friedrich, Ivar; Boergermann, Jochen

    2007-01-01

    Background Mediastinitis is a rare, but serious complication of cardiac surgery. It has a significant socioeconomic impact and high morbidity. The purpose of this study was to determine pre-, intra-, and postoperative predictors of mediastinitis. Methods and results In 1700 consecutive patients, who underwent cardiac surgery in 2001, 49 variables were retrospectively assessed. Forty-five patients (2.65%, 95% CI [1.88; 3.41]) developed postoperative mediastinitis. None of these patients died during their hospitalization. Multivariate analysis identified three of the 49 variables as highly significant independent predictors for the development of mediastinitis: obesity (OR 1.03, 95% CI [1.01; 1.04] p = 0.001), chronic obstructive pulmonary disease (OR 3.30, 95% CI [1.58; 6.88], p = 0.001), and bilateral grafting of the internal mammary artery (OR 3.18, 95% CI [1.20; 8.43] p = 0.02). The model is reliable in terms of its goodness of fit, it also discriminates well. Additionally, univariate analysis identified diabetes mellitus, CCS class and the number of intraoperatively transfused units of fresh frozen plasma as variables with a significant impact. Conclusion The present study suggests that bilateral IMA grafting, chronic obstructive pulmonary disease and obesity are important predictors of mediastinitis. PMID:17511885

  14. Endocrine Disorders in Cystic Fibrosis.

    PubMed

    Blackman, Scott M; Tangpricha, Vin

    2016-08-01

    Cystic fibrosis is frequently complicated by endocrine disorders. Diabetes can be expected to affect most with CF and pancreatic insufficiency and varies widely in age of onset, but early identification and treatment improve morbidity and mortality. Short stature can be exacerbated by relative delay of puberty and by use of inhaled corticosteroids. Bone disease in CF causes fragility fractures and should be assessed by monitoring bone mineral density and optimizing vitamin D status. Detecting and managing endocrine complications in CF can reduce morbidity and mortality in CF. These complications can be expected to become more common as the CF population ages. PMID:27469183

  15. Pancreatic pathophysiology in cystic fibrosis.

    PubMed

    Gibson-Corley, Katherine N; Meyerholz, David K; Engelhardt, John F

    2016-01-01

    The pancreas is one of the earliest, and most commonly affected, organs in patients with cystic fibrosis (CF). Studying the pathogenesis of pancreatic disease is limited in CF patients, due to its early clinical onset, co-morbidities and lack of tissue samples from the early phases of disease. In recent years, several new CF animal models have been developed that have advanced our understanding of both CF exocrine and endocrine pancreatic disease. Additionally, these models have helped us to better define the influence of pancreatic lesions on CF disease progression in other organs, such as the gastrointestinal tract and lung. PMID:26365583

  16. Pulmonary complications of cystic fibrosis.

    PubMed

    Ng, M Y; Flight, W; Smith, E

    2014-03-01

    The life expectancy of patients with cystic fibrosis (CF) has steadily increased over recent decades with a corresponding increase in the frequency of complications of the disease. Radiologists are increasingly involved with managing and identifying the pulmonary complications of CF. This article reviews the common manifestations of CF lung disease as well as updating radiologists with a number of less well-known complications of the condition. Early and accurate detection of the pulmonary effects of CF are increasingly important to prevent irreversible lung damage and give patients the greatest possibility of benefiting from the new therapies becoming available, which correct the underlying defect causing CF. PMID:24361142

  17. Fulminant mediastinitis due to extended-spectrum beta-lactamase-producing Klebsiella pneumoniae: atypical presentation and spreading following cardiac surgery†

    PubMed Central

    Valenzuela, Horacio; Carrascal, Yolanda; Maroto, Laura; Arce, Nuria

    2013-01-01

    Mediastinitis due to Klebsiella pneumoniae, related to thoracic wall contamination after cardiac surgery, has rarely been described. We aim to report a case of fulminant mediastinitis due to extended-spectrum beta-lactamase-producing K. pneumoniae, secondary to a disseminated concomitant pulmonary infection. The patient remained pauci-symptomatic until clinical manifestations of sepsis acutely appeared. PMID:23416348

  18. Managing diabetes in cystic fibrosis.

    PubMed

    Laguna, T A; Nathan, B M; Moran, A

    2010-10-01

    Cystic fibrosis related diabetes (CFRD) is the most common co-morbidity in persons with cystic fibrosis (CF). As the life expectancy of persons with CF continues to increase, the need to proactively diagnose and aggressively treat CFRD and its potential complications has become more apparent. CFRD negatively impacts lung function, growth and mortality, making its diagnosis and management crucial in a population already at high risk for early mortality. Compared to type 1 and type 2 diabetes, CFRD is a unique entity, requiring a thorough understanding of its unique pathophysiology to facilitate the creation and utilization of an effective medical treatment plan. The physiology of CFRD is complex, likely consisting of a combination of insulin deficiency, insulin resistance and a genetic predisposition towards the development of diabetes. However, the hallmark of CFRD is insulin deficiency, necessitating the use of exogenous insulin as the mainstay of therapy. Insulin administration, in combination with a multidisciplinary team of health professionals with expertise in the care of patients with CF and CFRD, is the cornerstone of the care for these patients. The goals of treatment of the CFRD population are to reverse protein catabolism, maintain a healthy weight, and reduce acute and chronic diabetes complications. Creating a partnership between the treatment team and the patient is the ideal way to accomplish these goals and is essential for successful diabetes care. PMID:20920037

  19. Cystic Fibrosis: Brazilian ENT Experience

    PubMed Central

    Sih, Tania; Godinho, Ricardo; Franco, Leticia Paiva; Piltcher, Otávio

    2012-01-01

    Most published studies about Cystic Fibrosis (CF) are European or North American. There are still few publications about the characteristics of fibrocystic populations in developing countries. The incidence of cystic fibrosis (CF) in Brazil varies among different regions (1 : 10,000 in Minas Gerais, 1 : 9,500 in Paraná, 1 : 8,700 in Santa Catarina, and 1 : 1600 in Rio Grande do Sul). The prevalence of the DF508 mutation also varies according to population: 33% in Sao Paulo, 49% in Rio Grande do Sul, 27% in Santa Catarina, and 52% in Minas Gerais. Cough and nasal obstruction are the most common symptoms. The variation in nasal polyposis prevalence may be explained by population genotypic characteristics in a country that spans a continent. Findings on nasal endoscopy and computed tomography (CT) have better correlation than do this information compared with surgical and clinical history. Microbiologic studies suggest a high level of early contamination of the airways. Sensorineural hearing loss (SNHL) occurs in these patients as a result of ototoxic antibiotics. The data compiled in this paper is useful, but also lead to the general agreement that more research would be welcome due to the unique characteristics of this country. PMID:22611403

  20. Aspergillus infections in cystic fibrosis.

    PubMed

    King, Jill; Brunel, Shan F; Warris, Adilia

    2016-07-01

    Patients with cystic fibrosis (CF) suffer from chronic lung infection and airway inflammation. Respiratory failure secondary to chronic or recurrent infection remains the commonest cause of death and accounts for over 90% of mortality. Bacteria as Staphylococcus aureus, Pseudomonas aeruginosa and Burkholderia cepacia complex have been regarded the main CF pathogens and their role in progressive lung decline has been studied extensively. Little attention has been paid to the role of Aspergillus spp. and other filamentous fungi in the pathogenesis of non-ABPA (allergic bronchopulmonary aspergillosis) respiratory disease in CF, despite their frequent recovery in respiratory samples. It has become more apparent however, that Aspergillus spp. may play an important role in chronic lung disease in CF. Research delineating the underlying mechanisms of Aspergillus persistence and infection in the CF lung and its link to lung deterioration is lacking. This review summarizes the Aspergillus disease phenotypes observed in CF, discusses the role of CFTR (cystic fibrosis transmembrane conductance regulator)-protein in innate immune responses and new treatment modalities. PMID:27177733

  1. Sonographic findings in horses with mediastinal lymphosarcoma: 13 cases (1985-1992).

    PubMed

    Garber, J L; Reef, V B; Reimer, J M

    1994-11-15

    Sonographic findings correlated with necropsy findings in 8 of 9 horses in which the cranial portion of the mediastinum was evaluated by use of both methods. Cranial mediastinal masses were imaged as multilobular and homogeneously hypoechoic; a complex echogenic pattern was observed with necrosis within the mass. Pleural effusion was a common finding in horses with mediastinal lymphosarcoma. Cytologic evaluation of pleural fluid samples was useful in diagnosis of lymphosarcoma in 10 of 12 horses that had thoracentesis. Sonographic examination of the thorax and cranial portion of the mediastinum can aid in the diagnosis of mediastinal lymphosarcoma in horses. Such examination should be performed in horses with clinical signs of cranial vena cava obstruction in which pleural effusion is detected, or when thoracic lymphosarcoma is suspected. PMID:7698925

  2. The surprising outcome of a giant primary mediastinal synovial sarcoma treated with neoadjuvant chemotherapy.

    PubMed

    Balieiro, Marcos Alexandre; Lopes, Agnaldo José; Costa, Bruno Pinheiro; Veras, Gustavo Perissé Moreira; Perelson, Paulo Sergio; Acatauassú Nunes, Rodolfo; Saito, Eduardo Haruo

    2013-02-01

    There are only a few cases of primary mediastinal synovial sarcoma in the literature. Normally, they do not respond well to chemotherapy. In our case, a 30-year-old patient was admitted due to thoracic pain, dyspnea, orthopnea, cough, hoarseness and weight loss over a 3-month period as well as a dramatic worsening a week before the admission. A chest radiography showed a completely white left hemithorax and contralateral mediastinal shift; in addition, a chest tomography revealed a giant heterogeneous mediastinal mass, lung atelectasia and a small pleural effusion. The patient was submitted to Chamberlain procedure (biopsy) under local anesthesia and the diagnosis of a synovial sarcoma was obtained after immunohistochemical analysis. Due to his poor general condition, he received chemotherapy first, with a dramatic response, after what, the mass that had been reduced was removed surgically. After a 5-year- follow-up period there are no signs of disease recurrence. PMID:23372956

  3. The surprising outcome of a giant primary mediastinal synovial sarcoma treated with neoadjuvant chemotherapy

    PubMed Central

    Balieiro, Marcos Alexandre; Costa, Bruno Pinheiro; Veras, Gustavo Perissé Moreira; Perelson, Paulo Sergio; Acatauassú Nunes, Rodolfo; Saito, Eduardo Haruo

    2013-01-01

    There are only a few cases of primary mediastinal synovial sarcoma in the literature. Normally, they do not respond well to chemotherapy. In our case, a 30-year-old patient was admitted due to thoracic pain, dyspnea, orthopnea, cough, hoarseness and weight loss over a 3-month period as well as a dramatic worsening a week before the admission. A chest radiography showed a completely white left hemithorax and contralateral mediastinal shift; in addition, a chest tomography revealed a giant heterogeneous mediastinal mass, lung atelectasia and a small pleural effusion. The patient was submitted to Chamberlain procedure (biopsy) under local anesthesia and the diagnosis of a synovial sarcoma was obtained after immunohistochemical analysis. Due to his poor general condition, he received chemotherapy first, with a dramatic response, after what, the mass that had been reduced was removed surgically. After a 5-year- follow-up period there are no signs of disease recurrence. PMID:23372956

  4. Lipomatous hypertrophy of the interatrial septum and fibrosing mediastinal lymphadenopathy causing superior vena cava obstruction

    PubMed Central

    Baikoussis, Nikolaos G.; Argiriou, Orestis; Kratimenos, Theodoros; Dedeilias, Panagiotis; Argiriou, Michalis

    2015-01-01

    Lipomatous hypertrophy of the interatrial septum (LHIS) is an uncommon cause of superior vena cava syndrome (SVCS). Fibrosing mediastinal lymphadenopathy is another cause of SVCS. We present a 65-year-old female patient with a history of tuberculosis (TB) and the coexistence of LHIS and fibrosing mediastinitis due to TB of the lung. Fibrosing or sclerosing mediastinitis is a rare entity with few cases published in the western literature. She presented with mild symptomatology of SVCS and she underwent on transthoracic and transesophageal echocardiography, computed tomography scan, magnetic resonance imaging, and venography. Due to the development of an abundant collateral venous system seen on venography and her negation for any treatment, she did not undergo yet on any intervention. To our knowledge, this is the first case reported in the international bibliography in which LHIS and sclerosing lymphadenopathy are simultaneously diagnosed in the same patient. PMID:26440257

  5. Mediastinal lymph node metastasis of renal cell carcinoma: A case report

    PubMed Central

    MIYAZAKI, KUNIHIKO; SATO, SHINYA; KODAMA, TAKAHIDE; KURISHIMA, KOICHI; SATOH, HIROAKI; HIZAWA, NOBUYUKI

    2016-01-01

    Renal cell carcinoma (RCC) may metastasize to mediastinal lymph nodes without any abdominal lymph node involvement. The present study describes an autopsy-proven case of RCC presenting with a large mediastinal mass; the case had been previously misdiagnosed as small cell lung carcinoma due to imaging analysis results, an elevated serum level of neuron-specific enolase and the presence of small atypical cells with a high nuclear/cytoplasmic ratio. Despite RCC occurrence being rare, it should be considered in the differential diagnosis, particularly when a mass located in the kidneys presents with metastases to the mediastinal lymph nodes, even if there is no involvement of the abdominal lymph nodes and the primary lesion is of a small size. PMID:26893788

  6. Successful Use of Extracorporeal Membrane Oxygenation for Respiratory Failure Caused by Mediastinal Precursor T Lymphoblastic Lymphoma

    PubMed Central

    Oto, Masafumi; Inadomi, Kyoko; Chosa, Toshiyuki; Uneda, Shima; Uekihara, Soichi; Yoshida, Minoru

    2014-01-01

    Precursor T lymphoblastic lymphoma (T-LBL) often manifests as a mediastinal mass sometimes compressing vital structures like vessels or large airways. This case was a 40-year-old male who developed T-LBL presenting as respiratory failure caused by mediastinal T-LBL. He presented with persistent life threatening hypoxia despite tracheal intubation. We successfully managed this respiratory failure using venovenous (VV) ECMO. Induction chemotherapy was started after stabilizing oxygenation and the mediastinal lesion shrank rapidly. Respiratory failure caused by compression of the central airway by tumor is an oncologic emergency. VV ECMO may be an effective way to manage this type of respiratory failure as a bridge to chemotherapy. PMID:25580133

  7. Post-surgical mediastinitis due to carbapenem-resistant Enterobacteriaceae: Clinical, epidemiological and survival characteristics.

    PubMed

    Abboud, C S; Monteiro, J; Stryjewski, M E; Zandonadi, E C; Barbosa, V; Dantas, D; Sousa, E E; Fonseca, M J; Jacobs, D M; Pignatari, A C; Kiffer, C; Rao, G G

    2016-05-01

    Invasive infections due to carbapenem-resistant Enterobacteriaceae (CRE), including polymyxin-resistant (PR-CRE) strains, are being increasingly reported. However, there is a lack of clinical data for several life-threatening infections. Here we describe a cohort of patients with post-surgical mediastinitis due to CRE, including PR-CRE. This study was a retrospective cohort design at a single cardiology centre. Patients with mediastinitis due to CRE were identified and were investigated for clinically relevant variables. Infecting isolates were studied using molecular techniques. Patients infected with polymyxin-susceptible CRE (PS-CRE) strains were compared with those infected with PR-CRE strains. In total, 33 patients with CRE mediastinitis were studied, including 15 patients (45%) with PR-CRE. The majority (61%) were previously colonised. All infecting isolates carried blaKPC genes. Baseline characteristics of patients with PR-CRE mediastinitis were comparable with those with PS-CRE mediastinitis. Of the patients studied, 70% received at least one agent considered active in vitro and most patients received at least three concomitant antibiotics. Carbapenem plus polymyxin B was the most common antibiotic combination (73%). Over 90% of patients underwent surgical debridement. Overall, in-hospital mortality was 33% and tended to be higher in patients infected with PR-CRE (17% vs. 53%; P=0.06). In conclusion, mediastinitis due to CRE, including PR-CRE, can become a significant challenge in centres with CRE and a high cardiac surgery volume. Despite complex antibiotic treatments and aggressive surgical procedures, these patients have a high mortality, particularly those infected with PR-CRE. PMID:27155944

  8. Feline mediastinal lymphoma: a retrospective study of signalment, retroviral status, response to chemotherapy and prognostic indicators.

    PubMed

    Fabrizio, Francesca; Calam, Amy E; Dobson, Jane M; Middleton, Stephanie A; Murphy, Sue; Taylor, Samantha S; Schwartz, Anita; Stell, Anneliese J

    2014-08-01

    Historically, feline mediastinal lymphoma has been associated with young age, positive feline leukaemia virus (FeLV) status, Siamese breed and short survival times. Recent studies following widespread FeLV vaccination in the UK are lacking. The aim of this retrospective multi-institutional study was to re-evaluate the signalment, retroviral status, response to chemotherapy, survival and prognostic indicators in feline mediastinal lymphoma cases in the post-vaccination era. Records of cats with clinical signs associated with a mediastinal mass and cytologically/histologically confirmed lymphoma were reviewed from five UK referral centres (1998-2010). Treatment response, survival and prognostic indicators were assessed in treated cats with follow-up data. Fifty-five cases were reviewed. The median age was 3 years (range, 0.5-12 years); 12 cats (21.8%) were Siamese; and the male to female ratio was 3.2:1.0. Five cats were FeLV-positive and two were feline immunodeficiency-positive. Chemotherapy response and survival was evaluated in 38 cats. Overall response was 94.7%; complete (CR) and partial response (PR) rates did not differ significantly between protocols: COP (cyclophosphamide, vincristine, prednisone) (n = 26, CR 61.5%, PR 34.0%); Madison-Wisconsin (MW) (n = 12, CR 66.7%, PR 25.0%). Overall median survival was 373 days (range, 20-2015 days) (COP 484 days [range, 20-980 days]; MW 211 days [range, 24-2015 days] [P = 0.892]). Cats achieving CR survived longer (980 days vs 42 days for PR; P = 0.032). Age, breed, sex, location (mediastinal vs mediastinal plus other sites), retroviral status and glucocorticoid pretreatment did not affect response or survival. Feline mediastinal lymphoma cases frequently responded to chemotherapy with durable survival times, particularly in cats achieving CR. The prevalence of FeLV-antigenaemic cats was low; males and young Siamese cats appeared to be over-represented. PMID:24366846

  9. On your toes: Detecting mediastinal air on the chest radiograph in ecstasy abusers.

    PubMed

    Naidoo, Mergan; Govind, Mayuri

    2016-01-01

    Abnormal mediastinal air may be caused by inhalational illicit drug use subsequent to barotrauma resulting from coughing after deep inhalation and breath holding. It may also arise from oesophageal rupture due to retching after ingestion of the illicit drug. The history can alert the practitioner to this cause of chest pain. As chest radiographs are widely accessible and mediastinal air is easily recognisable, the chest radiograph should be included and carefully scrutinised in the diagnostic workup of chest pain in the recreational drug abuser. It is prudent to exclude oesophageal rupture, particularly in the setting of retching, before deciding on conservative and expectant management. PMID:27138662

  10. Candida famata mediastinitis. A rare complication of open heart surgery. Case report and brief review.

    PubMed

    Sanchez Betancourt, Alfredo Alonso; Sibaja Alvarez, Pablo; Camacho, Rolando Arguedas; Guevara Espinoza, Edward

    2016-01-01

    Candida mediastinitis is a rare complication of open heart surgery with high mortality and morbidity usually associated with C. albicans. We are reporting the case of a 57 year old male who after having a triple coronary artery bypass graft procedure, had mediastinitis caused by Candida famata, a yeast, that had only been reported once before as the causal agent of this condition. It is of vital importance, that future cases be reported, due to the fact that both reported cases have led to patient demise. PMID:27419075

  11. An uncommon presentation of Kikuchi-Fujimoto disease as mediastinal lymphadenopathy.

    PubMed

    Hino, Haruaki; Nishimura, Takashi; Nitadori, Jun-Ichi; Miyakoshi, Shigesaburo; Seki, Atsuko; Arai, Tomio; Nakajima, Jun

    2016-05-01

    We experienced an uncommon presentation of Kikuchi-Fujimoto disease (KFD) with sole mediastinal lymphadenopathy in senior age, which was histologically diagnosed by thoracoscopic biopsy leading to appropriate therapy. A 69-year-old man was admitted due to intermittent high fever, general malaise, and appetite loss lasting over 3 months along with 10-kg weight loss in 6 months. Chest computed tomography (CT) showed isolated mediastinal lymphadenopathy, and malignant diseases including malignant lymphoma or metastatic carcinoma, tuberculous lymphadenitis, and sarcoidosis were considered. Therefore thoracoscopic biopsy should be performed for the correct diagnosis, even in uncommon portion of lymph node swelling and age for KFD. PMID:27162693

  12. EBUS-guided mediastinal lung cancer staging: monitoring of quality standards improves performance.

    PubMed

    Evison, Matthew; Crosbie, Philip; Martin, Julie; Shah, Rajesh; Doran, Helen; Borrill, Zoe; Hoyle, Jennifer; Rana, Durgesh; Bailey, Simon; Booton, Richard

    2016-08-01

    This audit examined key performance indices related to endobronchial ultrasound (EBUS)-guided mediastinal lung cancer staging before and after the introduction of defined quality standards, at four independent EBUS centres in one cancer network. Data from 642 procedures were prospectively collected and analysed. The introduction of standards was associated with a significant increase (p<0.001) in sampling of key mediastinal lymph node stations (4R, 4L and 7) and a reduction in the variability of staging sensitivity between centres. These data reinforce the requirement for an appropriate regulatory framework for EBUS-transbronchial needle aspiration provision that includes quality assurance and performance monitoring. PMID:27146201

  13. Treatment of thoracic hemorrhage due to rupture of traumatic mediastinal hematoma.

    PubMed

    Yu, Hui-Jie; Zhang, Ling-Fang; Cao, Wei-Zhong

    2016-02-01

    Patients in traffic accidents are usually presented with pain and bleeding due to fractures or soft tissue injury. On some occasions, more severe complications may be triggered by the trauma. A review of the published English language literature reveals no survival case once the traumatic mediastinal hematoma is ruptured. In our case, a 54-year-old man suffering motorcycle accident was admitted to emergency department. Computed tomography scan revealed subdural hematoma combined with posterior mediastinal hematoma. The patient was saved and discharged with a satisfactory outcome. Here we hope to share our treatment experience in dealing with the patient with severe multiple trauma. PMID:27033275

  14. Anesthetic management of superior vena cava syndrome due to anterior mediastinal mass

    PubMed Central

    Chaudhary, Kapil; Gupta, Anshu; Wadhawan, Sonia; Jain, Divya; Bhadoria, Poonam

    2012-01-01

    Anesthetic management of superior vena cava syndrome carries a possible risk of life-threatening complications such as cardiovascular collapse and complete airway obstruction during anesthesia. Superior vena cava syndrome results from the enlargement of a mediastinal mass and consequent compression of mediastinal structures resulting in impaired blood flow from superior vena cava to the right atrium and venous congestion of face and upper extremity. We report the successful anesthetic management of a 42-year-old man with superior vena cava syndrome posted for cervical lymph node biopsy. PMID:22557753

  15. An uncommon presentation of Kikuchi-Fujimoto disease as mediastinal lymphadenopathy

    PubMed Central

    Nishimura, Takashi; Nitadori, Jun-ichi; Miyakoshi, Shigesaburo; Seki, Atsuko; Arai, Tomio; Nakajima, Jun

    2016-01-01

    We experienced an uncommon presentation of Kikuchi-Fujimoto disease (KFD) with sole mediastinal lymphadenopathy in senior age, which was histologically diagnosed by thoracoscopic biopsy leading to appropriate therapy. A 69-year-old man was admitted due to intermittent high fever, general malaise, and appetite loss lasting over 3 months along with 10-kg weight loss in 6 months. Chest computed tomography (CT) showed isolated mediastinal lymphadenopathy, and malignant diseases including malignant lymphoma or metastatic carcinoma, tuberculous lymphadenitis, and sarcoidosis were considered. Therefore thoracoscopic biopsy should be performed for the correct diagnosis, even in uncommon portion of lymph node swelling and age for KFD. PMID:27162693

  16. Giant cystic pheochromocytoma: A silent entity

    PubMed Central

    Gupta, Amit; Bains, Lovenish; Agarwal, Manish Kumar; Gupta, Renu

    2016-01-01

    Pheochromocytoma is a catecholamine secreting tumor that originate from chromaffin cells. Usually, it is solid neoplasm of the adrenal medulla, however cystic pheochromocytoma is a rare neuro-endocrine tumour that is frequently asymptomatic and often diagnosed incidentally on imaging or intra-operatively. Only a few cases of cystic pheochromocytomas have been reported in the world literature. We present a case of giant cystic pheochromocytoma in a 65 years old lady who presented with a large retroperitoneal lump, which is probably the world's third largest pheochromocytoma as per the available indexed literature. PMID:27453669

  17. Heart involvement in cystic fibrosis: A specific cystic fibrosis-related myocardial changes?

    PubMed

    Labombarda, Fabien; Saloux, Eric; Brouard, Jacques; Bergot, Emmanuel; Milliez, Paul

    2016-09-01

    Cystic fibrosis is a complex multi-systemic chronic disease characterized by progressive organ dysfunction with development of fibrosis, possibly affecting the heart. Over the last four decades pathological, experimental, and clinical evidence points towards the existence of a specific myocardial involvement in cystic fibrosis. Multi-modality cardiac imaging, especially recent echocardiographic techniques, evidenced diastolic and/or systolic ventricular dysfunction in cystic fibrosis leading to the concept of a cystic fibrosis-related cardiomyopathy. Hypoxemia and inflammation are among the most important factors for heart involvement in cystic fibrosis. Cystic Fibrosis Transmembrane Regulator was found to be involved in the regulation of cardiomyocyte contraction and may also account for cystic fibrosis-related myocardial dysfunction. This review, mainly focused on echocardiographic studies, seeks to synthesize the existing literature for and against the existence of heart involvement in cystic fibrosis, its mechanisms and prognostic implications. Careful investigation of the heart function may be helpful for risk stratification and therapeutic decisions in patients with cystic fibrosis. PMID:27578468

  18. Chronic pancreatitis and cystic fibrosis

    PubMed Central

    Witt, H

    2003-01-01

    Recent discoveries of trypsinogen and trypsin inhibitor mutations in patients with chronic pancreatitis (CP) support the hypothesis that an inappropriate activation of pancreatic zymogens to active enzymes within the pancreatic parenchyma starts the inflammatory process. Current data suggest that CP may be inherited dominant, recessive, or complex as a result of mutations in the above mentioned or yet unidentified genes. Evaluation of patients with CP should include genetic testing. Cystic fibrosis (CF) is an autosomal recessive inherited disorder caused by mutations in the CF transmembrane conductance regulator (CFTR) gene and is characterised by pancreatic insufficiency and chronic bronchopulmonary infection. The progression and severity of pulmonary disease differs considerably between people with identical CFTR mutations and does not seem to correlate with the type or class of the CFTR mutation. The identification of further disease modifying genetic factors will increase the pathophysiological understanding and may help to identify new therapeutic targets. PMID:12651880

  19. Aspergillus bronchitis in cystic fibrosis.

    PubMed

    Shoseyov, David; Brownlee, Keith G; Conway, Steven P; Kerem, Eitan

    2006-07-01

    Aspergillus fumigatus, a widely distributed spore-bearing fungus, is commonly grown in sputum cultures of patients with cystic fibrosis (CF). A fumigatus may cause allergic bronchopulmonary aspergillosis (ABPA), a complex condition that leads to worsening of airway inflammation and progressive damage and is diagnosed by specific criteria. In this report, we present six CF patients with respiratory deterioration that did not respond to appropriate antibiotic treatment. All had had A fumigatus in sputum cultures but did not fulfill the criteria of ABPA. Treatment with antifungal agents was followed by improvement in clinical condition. We suggest that in patients with CF, A fumigatus should be considered as a pathogen that may directly cause respiratory exacerbations. Antifungal therapy should be considered when deteriorating respiratory function is not responding to antibacterial therapy and A fumigatus is growing in sputum cultures. PMID:16840406

  20. Biliary complications of cystic fibrosis.

    PubMed Central

    O'Brien, S; Keogan, M; Casey, M; Duffy, G; McErlean, D; Fitzgerald, M X; Hegarty, J E

    1992-01-01

    One hundred and four adult patients with cystic fibrosis were evaluated for the presence of liver disease as defined by abnormal liver function tests of six months' duration, histological evidence of fibrosis or cirrhosis, or the presence of portal hypertension, or both. Twenty patients fulfilled these criteria and were evaluated further for the presence of biliary tract abnormalities with biliary scintigraphy using 99Tc diisopropylphenyl-carboxymethyl iminodiacetic acid (DISIDA) and endoscopic retrograde cholangiography. Clearance of 99Tc DISIDA from the liver and biliary tree was diminished at 45 (E45) and 60 (E60) minutes in the patients with liver disease compared with those without liver disease; E45 = 37.8% and 65.8%, p less than 0.01; E60 = 48.2% and 77.5%, p less than 0.01 respectively. Serial analogue images of the extrahepatic biliary tree were consistent with common bile duct obstruction with retention of DISIDA and tapering of the common bile duct in seven of 18 patients with and two of 10 patients without liver disease. Endoscopic retrograde cholangiography showed changes consistent with sclerosing cholangitis, with beading and stricturing of the intrahepatic ducts in 12 of the 14 patients. In all 14 patients, including those in whom biliary scintigraphy had suggested obstruction, no abnormality of the common bile duct was identified. These results indicate that abnormalities of the bile ducts in patients with cystic fibrosis related liver disease are confined to the intrahepatic biliary tree and that common bile duct strictures do not contribute to either the progression or development of liver disease in these patients. Images Figure 2 PMID:1568661

  1. Epidural Cystic Spinal Meningioma: A Case Report.

    PubMed

    Zhang, Ji; Chen, Zheng-He; Wang, Zi-Feng; Sun, Peng; Jin, Jie-Tian; Zhang, Xiang-Heng; Zhao, Yi-Ying; Wang, Jian; Mou, Yong-Gao; Chen, Zhong-Ping

    2016-03-01

    Cystic spinal meningioma (CSM) is an uncommon meningioma variant. Extradural CSMs are particularly rare and difficult to distinguish from other intraaxial tumors. This study presents a case of a 36-year-old woman with intraspinal extradual CSM at the thoracolumbar spine. She experienced persistent weakness, progressive numbness, and sensory disturbance in the right lower limb. Magnetic resonance imaging (MRI) of the patient revealed an irregular cystic mass at the thoracic 11 to lumbar 3 levels dorsally. This case was misdiagnosed as other neoplasms prior to surgery because of the atypical radiographic features and location of the tumor.Extradural CSMs should be considered in the differential diagnosis of intraspinal extradural cystic neoplasms. Complete removal of cystic wall provides an optimal outcome, rendering the lesion curable. PMID:26986119

  2. [Epidemiology of cystic echinococcosis in the world].

    PubMed

    Tünger, Özlem

    2013-01-01

    The incidence and prevalence of cystic echinococcosis have fallen dramatically over the past several decades. Nonetheless, cystic echinococcosis remains a major public health issue in several countries and regions as a result of a reduction of control programmes due to economic problems. Geographic distribution differs by country and region depending on the presence of large numbers of nomadic or semi-nomadic sheep and goat flocks that represent the intermediate host of the parasite, and their close contact with the final host, the dog, which mostly provides the transmission of infection to humans. The greatest prevalence of cystic echinococcosis in human and animal hosts is found in countries of the temperate zones, including Mediterranean regions, southern and central parts of Russia, central Asia, China, Australia, South America and north and east Africa. In this article, the geographic distribution and epidemiology of cystic echinococcosis worldwide are reviewed. PMID:23619047

  3. Mediastinal paraganglioma presenting as an intracardiac mass with superior vena caval obstruction.

    PubMed Central

    Sharma, S. K.; Sharma, S.; Mukhopadhyay, S.

    1993-01-01

    A case of mediastinal paraganglioma presenting with superior vena caval obstruction is reported. The tumour extended into the right atrium and ventricle. Tru-Cut biopsy under ultrasonographic guidance was performed safely to provide a diagnosis before death. Images PMID:8296267

  4. Intra and Interfraction Mediastinal Nodal Region Motion: Implications for Internal Target Volume Expansions

    SciTech Connect

    Thomas, Jonathan G.; Kashani, Rojano; Balter, James M.; Tatro, Daniel; Kong, F.-M.; Pan, Charlie C.

    2009-07-01

    The purpose of this study was to determine the intra and interfraction motion of mediastinal lymph node regions. Ten patients with nonsmall-cell lung cancer underwent controlled inhale and exhale computed tomography (CT) scans during two sessions (40 total datasets) and mediastinal nodal stations 1-8 were outlined. Corresponding CT scans from different sessions were registered to remove setup error and, in this reference frame, the centroid of each nodal station was compared for right-left (RL), anterior-posterior (AP), and superior-inferior (SI) displacement. In addition, an anisotropic volume expansion encompassing the change of the nodal region margins in all directions was used. Intrafraction displacement was determined by comparing same session inhale-exhale scans. Interfraction reproducibility of nodal regions was determined by comparing the same respiratory phase scans between two sessions. Intrafraction displacement of centroid varied between nodal stations. All nodal regions moved posteriorly and superiorly with exhalation, and inferior nodal stations showed the most motion. Based on anisotropic expansion, nodal regions expanded mostly in the RL direction from inhale to exhale. The interpatient variations in intrafraction displacement were large compared with the displacements themselves. Moreover, there was substantial interfractional displacement ({approx}5 mm). Mediastinal lymph node regions clearly move during breathing. In addition, deformation of nodal regions between inhale and exhale occurs. The degree of motion and deformation varies by station and by individual. This study indicates the potential advantage of characterizing individualized nodal region motion to safely maximize conformality of mediastinal nodal targets.

  5. [A case of miliary tuberculosis and esophageal perforation secondary to tuberculous mediastinal lymphadenitis].

    PubMed

    Ishikawa, Shigenori; Yano, Shuichi; Wakabayashi, Kiryo; Kadowaki, Toru; Kimura, Masahiro; Kobayashi, Kanako; Ikeda, Toshikazu; Takeyama, Hiroyasu

    2009-04-01

    An 80-year-old woman was admitted to a local hospital following transient disturbance of consciousness after a fall. High intermittent fever developed after hospitalization and she was diagnosed as having mediastinal abscess with esophageal perforation. She underwent mediastinal drainage and surgical repair of the esophagus. Acid-fast bacilli were detected in her sputum. Chest CT scanning showed a diffuse granular shadow. Then she was diagnosed as having miliary tuberculosis and treated with combination of INH, RFP, EB, and PZA. However, five days after treatment was initiated, fever and skin eruption appeared and treatment has to be stopped after one month. Then she was referred to our hospital. We gradually increased the dosages of INH and RFP, which resulted in pyrexia. Therefore, we changed EB to SM. Fever subsided and we were able to administer the full dose of drugs from the beginning of January 2007. Thereafter, the patient improved gradually. However, she died in February 2007. At autopsy, we identified tuberculous mediastinal lymphadenitis, inflammatory granuloma under the esophageal mucosa and miliary tuberculosis. We report this case as a rare case of miliary tuberculosis and esophageal perforation secondary to tuberculous mediastinal lymphadenitis. PMID:19425392

  6. Mediastinitis caused by Aspergillus fumigatus with ruptured aortic pseudoaneurysm in a heart transplant recipient: case study.

    PubMed

    Byl, B; Jacobs, F; Antoine, M; Depierreux, M; Serruys, E; Primo, G; Thys, J P

    1993-01-01

    The case of a heart transplant recipient with a ruptured aortic pseudoaneurysm caused by an Aspergillus fumigatus mediastinitis is reported. Contamination of surgical fields occurring by air seeding during surgery appears to be the most probable source of infection. Subtle infectious signs of the wound and subacute course are remarkable features of this case. PMID:8449757

  7. Mediastinal lymphadenopathy and pulmonary arterial hypertension in mixed connective tissue disease

    SciTech Connect

    Guit, G.L.; Shaw, P.C.; Ehrlich, J.; Kroon, H.M.; Oudkerk, M.

    1985-02-01

    A case of mixed connective tissue disease (MCTD) is presented in which mediastinal lymphadenopathy was the most prominent radiological finding detected by plain chest radiographs and computed tomography. Pulmonary arterial hypertension, which is a rare and often fatal complication of MCTD, also developed in this patient.

  8. The flow and composition of lymph from the caudal mediastinal lymph node of sheep.

    PubMed Central

    Spencer, J; Hall, J G

    1984-01-01

    By cannulating the efferent duct of the caudal mediastinal lymph node in sheep, lymph from the lower respiratory tract was collected under physiological conditions for several days. In 18 such preparations the flow rate varied from 4 to 12 ml/hr between individuals and the lymphocyte count between 4000 and 117,000/mm3. The protein content of the lymph plasma averaged nearly 60% of that of the blood, and this indication of the high permeability of the capillary bed of the lungs was confirmed by measuring the time taken for intravenous doses of 125I-albumin to equilibrate between the blood and mediastinal lymph plasma. The concentration of immunoglobulin A was higher in the mediastinal lymph than in blood serum, while the reverse was true of the concentrations of IgG1, IgG2, and IgM. This evidence for the local production of IgA by the intra thoracic lymphoid tissue was supported by the demonstration by immunoperoxidase techniques of IgA-containing plasma cells in sections cut from the caudal mediastinal nodes, and of IgA-containing immunoblasts in the lymph. Images Figure 3 PMID:6715022

  9. [Renal failure and cystic kidney diseases].

    PubMed

    Correas, J-M; Joly, D; Chauveau, D; Richard, S; Hélénon, O

    2011-04-01

    Cystic kidney diseases often are discovered at the time of initial work-up of renal failure through ultrasound or family history, or incidentally at the time of an imaging test. Hereditary diseases include autosomal dominant or recessive polycystic kidney disease (PKD), tuberous sclerosis (TS) and medullary cystic kidney disease (MCKD). Autosomal dominant PKD is characterized by large renal cysts developing in young adults. Renal failure is progressive and becomes severe around 50-60 years of age. Atypical cysts (hemorrhagic or hyperdense) are frequent on CT and MRI examinations. Imaging plays a valuable role in the management of acute complications such as cyst hemorrhage or infection. Autosomal recessive PKD is often detected in neonates, infants or young adults. It is characterized by renal enlargement due to the presence of small cysts and liver disease (fibrosis and biliary ductal dilatation). Late manifestation or slow progression of autosomal recessive PKD may be more difficult to distinguish from autosomal dominant PKD. These cystic kidney diseases should not be confused with non-hereditary incidental multiple renal cysts. In tuberous sclerosis, renal cysts are associated with angiomyolipomas and sometimes pulmonary lymphangioleiomyomatosis. Renal failure is inconstant. Other hereditary cystic kidney diseases, including MCKD and nephronophtisis, are usually associated with renal failure. Non-hereditary cystic kidney diseases include multicystic renal dysplasia (due to complete pelvi-ureteric atresia or hydronephrosis), acquired multicystic kidney disease (chronic renal failure, chronic hemodialysis) and varied cystic kidney diseases (multicystic renal disease, glomerulocystic kidney disease, microcystic kidney disease). PMID:21549887

  10. Cystic fibrosis from the gastroenterologist's perspective.

    PubMed

    Ooi, Chee Y; Durie, Peter R

    2016-03-01

    Cystic fibrosis is a life-limiting, recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Increased survival outcomes and the multisystem nature of the disease, including the involvement of hepatobiliary and gastrointestinal tracts, now require the need for more extensive knowledge and expertise in cystic fibrosis among gastroenterologists. Manifestations are either a direct consequence of the primary defect in cystic fibrosis or a secondary complication of the disease or therapy. Adult patients with cystic fibrosis also have an increased risk of malignancy in the gastrointestinal and pancreatico-biliary tracts compared with the general population. Novel treatments that target the basic defects in the CFTR protein have emerged, but to date not much is known about their effects on the gastrointestinal and hepatobiliary systems. The introduction of such therapies has provided new opportunities for the application of intestinal endpoints in clinical trials and the understanding of underlying disease mechanisms that affect the gut in cystic fibrosis. PMID:26790364

  11. Primary mediastinal seminomas: a comprehensive immunohistochemical study with a focus on novel markers.

    PubMed

    Weissferdt, Annikka; Rodriguez-Canales, Jaime; Liu, Hui; Fujimoto, Junya; Wistuba, Ignacio I; Moran, Cesar A

    2015-03-01

    Primary mediastinal seminomas are unusual tumors that can present in a pure form or as part of a mixed germ cell tumor. Contrary to testicular seminomas, little is known about the expression of novel immunohistochemical markers in mediastinal seminomas. This study investigates the immunohistochemical features of these tumors with a focus on novel markers. Thirty-two cases of primary mediastinal seminomas were reviewed; and representative whole-tissue sections were selected for immunohistochemical studies using antibodies directed against high molecular weight cytokeratin 5/6 (CK5/6), low molecular weight cytokeratin (CAM5.2), octamer-binding transcription factor 3/4 (OCT3/4), spalt-like transcription factor 4 (SALL4), GATA binding protein 3 (GATA-3), sry-related HMG box 2 (SOX2), SOX17, human T cell leukemia/lymphoma 1 (TCL1), glypican 3, melanoma associated antigen C2 (MAGEC2), and paired box gene 8 (Pax8). The percentage of positive tumor cells as well as the intensity of staining was evaluated and scored. Thirty-one cases (97%) expressed SOX17, whereas 29 cases (91%) were positive for OCT3/4 and SALL4, respectively. Twenty-eight cases (88%) expressed MAGEC2 and CAM5.2, respectively. Two cases (6%) were positive for Pax8, and a single case (3%) was positive for TCL1. None of the cases stained with CK5/6, GATA-3, SOX2, or glypican 3. Similar to testicular seminomas, mediastinal seminomas show consistent expression of OCT3/4, SALL4, SOX17, and MAGEC2 and are negative for SOX2, glypican 3, GATA-3, and CK5/6. Pax8 positivity is only inconsistently identified in mediastinal seminomas. Contrary to their testicular counterparts, mediastinal tumors show diffuse expression of low-molecular-weight cytokeratin in up to 90% of cases and are commonly negative for TCL1. Although there is some immunohistochemical overlap between testicular and mediastinal seminomas, considerable differences also exist and should be acknowledged when dealing with these tumors. PMID:25576290

  12. A Life-Threatening Mediastinal Hematoma After Central Venous Port System Implantation

    PubMed Central

    Sarach, Janine; Zschokke, Irin; Melcher, Gian A.

    2015-01-01

    Patient: Female, 68 Final Diagnosis: Mediastinal hematoma Symptoms: Agitation • severe hemodynamic instability • severe respiratory distress Medication: — Clinical Procedure: Cardiopulmonary resuscitation • reintubation • thoracic drain Specialty: Surgery Objective: Diagnostic/therapeutic accidents Background: We report a case of surgical central venous port system implantation using Seldinger’s technique with a life-threatening mediastinal hematoma due to the perforation of the superior vena cava. Case Report: A 68-year-old woman was admitted to our institution for port implantation. Open access to the cephalic vein and 2 punctures of the right subclavian vein were unsuccessful. Finally, the port catheter could be placed into the superior vena cava using Seldinger’s technique. As blood aspiration via the port catheter was not possible, fluoroscopy was performed, revealing mediastinal contrast extravasation without contrasting the venous system. A new port system could be placed in the correct position without difficulties. After extubation, the patient presented with severe respiratory distress and required consecutive cardiopulmonary resuscitation and reintubation. The CT scan showed a significant hematoma in the lower neck and posterior mediastinum with tracheal compression. We assumed a perforation of the superior vena cava with the tip of the guidewire using Seldinger’s technique. Long-term intensive treatment with prolonged ventilation and tracheotomy was necessary. The port system had to be subsequently explanted due to infection. Conclusions: Mediastinal hematoma is a rare but life-threatening complication associated with central venous catheterization using Seldinger’s technique. Perforation occurs most often during central venous catheterization in critical care. Mediastinal hematoma is an example of a mechanical complication occurring after central venous catheterization, which has been described only a few times in the literature to

  13. Feasibility of thoracoscopic approach for retrosternal goitre (posterior mediastinal goitre): Personal experiences of 11 cases

    PubMed Central

    Bhargav, Panchangam Ramakanth; Amar, Vennapusa; Mahilvayganan, Sabaretnam; Nanganandadevi, Vimala

    2016-01-01

    INTRODUCTION: Posterior mediastinal goitres constitute of a unique surgical thyroid disorder that requires expert management. Occasionally, they require thoracic approach for the completion of thyroidectomy. In this paper, we describe the feasibility and utility of a novel thoracoscopic approach for such goitres. MATERIALS AND METHODS: This is a retrospective study conducted at a tertiary care endocrine surgery department in South India over a period of 5 years from January 2010 to December 2014. We developed a novel thoracoscopic technique for posterior mediastinal goitres instead of a more morbid thoracotomy or sternotomy. All the clinical, investigative, operative, pathological and follow-up data were collected from our prospectively filled database. Statistical analysis was done with SPSS 15.0 version. Descriptive analysis was done. OPERATIVE TECHNIQUE OF THORACOSCOPIC THYROIDECTOMY: Single lumen endotracheal tube (SLETT) was used of anaesthetic intubation and general inhalational anaesthesia. Operative decubitus was supine with extension and abduction of the ipsilateral arm. Access to mediastinum was obtained by two working ports in the third and fifth intercostal spaces. Mediastinal extension was dissected thoracoscopically and delivered cervically. RESULTS: Out of 1,446 surgical goitres operated during the study period, 72 (5%) had retrosternal goitre. Also, 27/72 (37.5%) cases had posterior mediastinal extension (PME), out of which 11 cases required thoracic approach. We utilised thoracoscopic technique for these 11 cases. The post-operative course was uneventful with no major morbidity. There was one case of recurrent laryngeal nerve (RLN) injury and hoarseness of voice in the third case. Histopathologies in 10 cases were benign, out of which two had subclinical hyperthyroidism. One case had multifocal papillary microcarcinoma. CONCLUSIONS: We opine that novel thoracoscopic technique is a feasibly optimal approach for posterior mediastinal goitre

  14. Mediastinal transposition of the omentum reduces infection severity and pharmacy cost for patients undergoing esophagectomy

    PubMed Central

    Ye, Peng; Cao, Jin-Lin; Li, Qiu-Yuan; Wang, Zhi-Tian; Yang, Yun-Hai; Lv, Wang

    2016-01-01

    Background The greater omentum has been found to be immunologically competent in protecting abdominal organs from inflammation. Anastomotic omentoplasty has been used and proven effective in preventing anastomotic leaks after an esophagectomy. However, pulmonary complications are still a substantial problem after an esophagectomy. This study investigated the benefits of mediastinal transposition of the omentum, a modification of the conventional omental wrapping technique, in controlling overall postoperative intrathoracic complications. Methods From January 2010 to March 2015, 208 consecutive patients receiving an open Ivor-Lewis esophagectomy at our institution were retrospectively reviewed. One hundred twenty-one patients with omentum mediastinal transposition were assigned to the transposition group and 87 patients without omental transposition were placed in the non-transposition group. The patients’ demographics, postoperative short-term outcomes, and in-hospital cost were documented and analyzed. Results Mediastinal transposition of the omentum led to a shorter postoperative hospital stay (14 vs. 16 d, P=0.038) and a lower intrathoracic infection rate (30.6% vs. 48.3%, P=0.009). Intrathoracic infection was milder in the transposition group (P=0.005), though a non-significant was found in overall complications (P=0.071). The multivariate logistic regression analyses identified omentum mediastinal transposition (P=0.007, OR=0.415) as an independent protective factor for postoperative intrathoracic infection. The total in-hospital cost was comparable in both groups (P>0.05), whereas the pharmacy cost was lower in the transposition group than in the non-transposition group (¥21,668 vs. ¥27,012, P=0.010). Conclusions Mediastinal transposition of the omentum decreases the rate and severity of postoperative intrathoracic infection following an open Ivor-Lewis esophagectomy. This result in decreased pharmacy costs, rather than resulting in an increased economic

  15. Abnormal Ion Permeation through Cystic Fibrosis Respiratory Epithelium

    NASA Astrophysics Data System (ADS)

    Knowles, M. R.; Stutts, M. J.; Spock, A.; Fischer, N.; Gatzy, J. T.; Boucher, R. C.

    1983-09-01

    The epithelium of nasal tissue excised from subjects with cystic fibrosis exhibited higher voltage and lower conductance than tissue from control subjects. Basal sodium ion absorption by cystic fibrosis and normal nasal epithelia equaled the short-circuit current and was amiloride-sensitive. Amiloride induced chloride ion secretion in normal but not cystic fibrosis tissue and consequently was more effective in inhibiting the short-circuit current in cystic fibrosis epithelia. Chloride ion-free solution induced a smaller hyperpolarization of cystic fibrosis tissue. The increased voltage and amiloride efficacy in cystic fibrosis reflect absorption of sodium ions across an epithelium that is relatively impermeable to chloride ions.

  16. Laparoscopic resection of an intra-abdominal cystic mass: a cystic mesothelioma

    PubMed Central

    Birch, Daniel W.; Park, Adrian; Chen, Vicki

    1998-01-01

    The clinical features of a patient with an intra-abdominal cystic mass do not lead to a specific diagnosis. Aspiration is usually ineffective because the mass recurs and cytologic investigation is often non-diagnostic. Conservative management is unsuccessful because symptoms often persist. Surgical management of cystic masses is required for definitive management and pathologic diagnosis. A laparoscopic approach to the diagnosis and treatment can provide essential anatomic information and a complete resection with minimal morbidity. A laparoscopic technique using 3 trocars and maintaining the integrity of the mass allows complete excision and removal of large intra-abdominal cystic masses as reported in a 43-year-old patient with a large intra-abdominal cystic mass identified as a benign cystic mesothelioma. PMID:9576001

  17. Surgical outcome in cystic vestibular schwannomas

    PubMed Central

    Nair, Suresh; Baldawa, Sachin S.; Gopalakrishnan, Chittur Viswanathan; Menon, Girish; Vikas, Vazhayil; Sudhir, Jayanand B.

    2016-01-01

    Background: Cystic vestibular schwannomas (VS) form a rare subgroup that differs from the solid variant clinically, radiologically, and histopathologically. These tumors also vary in their surgical outcome and carry a different risk of post-operative complications. We analyzed our series of 64 patients with cystic VS and discuss the technical difficulties related to total excision of these tumors and focus on complication avoidance. Materials and Methods: A retrospective review of cystic VS surgically managed over a span of 11 years. The case records were evaluated to record the clinical symptoms and signs, imaging findings, surgical procedure, complications, and follow-up data. Post-operative facial nerve palsy was analyzed with respect to tumor size and tumor type. Results: Progressive hearing impairment was the most common initial symptom (76.6%). Atypical initial symptoms were present in 15 patients (23.4%). Preoperatively, 78% patients had good facial nerve function (HB grade 1, 2) and 22% had intermediate (HB grade 3, 4) to poor (HB grade 5 and 6) function. Mean tumor size was 4.1 cm. Complete tumor removal was achieved in 53 patients (83%). The facial nerve was anatomically intact but thinned out after tumor excision in 38 patients (59.4%). Ninety percent patients had either intermediate or poor facial nerve function at follow-up. Poor facial nerve outcome was associated with giant tumors and peripherally located, thin-walled cystic tumors. Conclusion: Resection of cystic VS is complicated by peritumoral adhesions of the capsule to the nerve. Extensive manipulation of the nerve in order to dissect the tumor–nerve barrier results in worse facial nerve outcome. The outcome is worse in peripherally located, thin-walled cystic VS as compared to centrally located, thick-walled cystic tumors. Subtotal excision may be justified, especially in tumors with dense adhesion of the cyst wall to the facial nerve in order to preserve nerve integrity. PMID:27366248

  18. Antibiotic allergy in cystic fibrosis.

    PubMed

    Parmar, J S; Nasser, S

    2005-06-01

    Allergic reactions to antibiotics are more common in cystic fibrosis (CF) than in the general population. This in part is due to the improving survival in adults with CF and the increased use of high dose intravenous antibiotics. While some are immediate anaphylaxis type (IgE mediated) reactions, the majority are late onset and may have non-specific features such as rash and fever. Piperacillin has consistently been found to have the highest rate of reported reactions (30-50%). There is a low risk of cross reactions between penicillins and other non-beta-lactam classes of antibiotics in penicillin skin prick positive patients. Carbapenems should only be used with extreme caution in patients with positive skin prick tests to penicillin. However, aztreonam can be used safely in patients who are penicillin allergic with positive skin prick reactions. The aminoglycosides are a relatively uncommon cause of allergic reactions, but patients who react to one member of the family may cross react with other aminoglycosides. Desensitisation relies on the incremental introduction of small quantities of the allergen and has been used for penicillins, ceftazidime, tobramycin and ciprofloxacin and must be repeated before each course. Personalized cards should be regularly updated for patients who develop allergic reactions. Written instructions on the emergency treatment of allergic reactions should be provided to patients self-administering intravenous antibiotics at home. Further research is required to identify risk factors and predictors for antibiotic allergy. PMID:15923254

  19. Evaluation of hepatic cystic lesions.

    PubMed

    Lantinga, Marten A; Gevers, Tom J G; Drenth, Joost P H

    2013-06-21

    Hepatic cysts are increasingly found as a mere coincidence on abdominal imaging techniques, such as ultrasonography (USG), computed tomography (CT) and magnetic resonance imaging (MRI). These cysts often present a diagnostic challenge. Therefore, we performed a review of the recent literature and developed an evidence-based diagnostic algorithm to guide clinicians in characterising these lesions. Simple cysts are the most common cystic liver disease, and diagnosis is based on typical USG characteristics. Serodiagnostic tests and microbubble contrast-enhanced ultrasound (CEUS) are invaluable in differentiating complicated cysts, echinococcosis and cystadenoma/cystadenocarcinoma when USG, CT and MRI show ambiguous findings. Therefore, serodiagnostic tests and CEUS reduce the need for invasive procedures. Polycystic liver disease (PLD) is arbitrarily defined as the presence of > 20 liver cysts and can present as two distinct genetic disorders: autosomal dominant polycystic kidney disease (ADPKD) and autosomal dominant polycystic liver disease (PCLD). Although genetic testing for ADPKD and PCLD is possible, it is rarely performed because it does not affect the therapeutic management of PLD. USG screening of the liver and both kidneys combined with extensive family history taking are the cornerstone of diagnostic decision making in PLD. In conclusion, an amalgamation of these recent advances results in a diagnostic algorithm that facilitates evidence-based clinical decision making. PMID:23801855

  20. Respiratory Conditions Update: Cystic Fibrosis.

    PubMed

    Pritchard, Lyle L

    2016-09-01

    Cystic fibrosis (CF) is an autosomal recessive genetic disease that occurs in approximately 1 in 2,500 white live births. It is less common in nonwhite individuals. A dysfunctional epithelial chloride channel leads to excessively thick mucus affecting multiple organ systems. Common issues include mucous plugging of the airway, lung inflammation, chronic pulmonary infections, intestinal malabsorption, and malnutrition. Universal screening of newborns for CF is recommended in many countries. CF can be diagnosed based on clinical evidence of disease along with genetic testing or other laboratory evidence of chloride channel dysfunction. Pulmonary system dysfunction causes the most morbidity and mortality. Pulmonary function testing is the primary modality used to monitor CF progression. Therapies include chest physiotherapy, mucolytics, antibiotics, anti-inflammatory drugs, targeted therapies, and vaccines. Dysfunction of the exocrine pancreas and gastrointestinal tract leads to malabsorption, malnutrition, and intestinal obstruction. Nutrition should be optimized with adequate calories, pancreatic enzymes, and appropriate dietary supplements. Complications, including acute pulmonary exacerbations, gastrointestinal conditions, chronic rhinosinusitis, CF-related diabetes, osteoporosis, infertility, and psychosocial issues, must be managed. At the appropriate time, lung transplantation and end-of-life issues must be addressed. PMID:27576234

  1. Lung Transplantation for Cystic Fibrosis

    PubMed Central

    Adler, Frederick R.; Aurora, Paul; Barker, David H.; Barr, Mark L.; Blackwell, Laura S.; Bosma, Otto H.; Brown, Samuel; Cox, D. R.; Jensen, Judy L.; Kurland, Geoffrey; Nossent, George D.; Quittner, Alexandra L.; Robinson, Walter M.; Romero, Sandy L.; Spencer, Helen; Sweet, Stuart C.; van der Bij, Wim; Vermeulen, J.; Verschuuren, Erik A. M.; Vrijlandt, Elianne J. L. E.; Walsh, William; Woo, Marlyn S.; Liou, Theodore G.

    2009-01-01

    Lung transplantation is a complex, high-risk, potentially life-saving therapy for the end-stage lung disease of cystic fibrosis (CF). The decision to pursue transplantation involves comparing the likelihood of survival with and without transplantation as well as assessing the effect of wait-listing and transplantation on the patient's quality of life. Although recent population-based analyses of the US lung allocation system for the CF population have raised controversies about the survival benefits of transplantation, studies from the United Kingdom and Canada have suggested a definite survival advantage for those receiving transplants. In response to these and other controversies, leaders in transplantation and CF met together in Lansdowne, Virginia, to consider the state of the art in lung transplantation for CF in an international context, focusing on advances in surgical technique, measurement of outcomes, use of prognostic criteria, variations in local control over listing, and prioritization among the United States, Canada, the United Kingdom, and The Netherlands, patient adherence before and after transplantation and other issues in the broader context of lung transplantation. Finally, the conference members carefully considered how efforts to improve outcomes for lung transplantation for CF lung disease might best be studied. This Roundtable seeks to communicate the substance of our discussions. PMID:20008865

  2. Postoperative recurrence of cystic hydatidosis

    PubMed Central

    Prousalidis, John; Kosmidis, Christophoros; Anthimidis, Georgios; Kapoutzis, Konstantinos; Karamanlis, Eleutherios; Fachantidis, Epaminondas

    2012-01-01

    Background Surgical management is the basic treatment for hydatid disease. Overall, the recurrence rate appears to be high (4.6%–22.0%). The purpose of this study was to report our results in the management of recurrent hydatid disease, evaluating the methods for identifying recurrence, prognostic factors and therapeutic options. Methods We retrospectively reviewed the medical records of patients who underwent surgery for cystic hydatidosis between 1970 and 2003. Results Of the 584 patients who underwent surgery during our study period, follow-up was complete for 484 (82.8%). Cysts recurred in 51 patients (8.7%). Abdominal ultrasonography and computed tomography appeared to be efficient for diagnosing recurrence. The 2 most important determinants for recurrence were minute spillage of the hydatid cyst and inadequate treatment owing to missing cysts or incomplete pericystectomy. All but 2 recurrences required surgery. There were 14 postoperative complications for a rate of 27.0%. Thirteen re-recurrences were observed in the follow-up of these patients and also required surgery. Conclusion Avoidance of minute spillage of cyst contents and cautious removal of the parasite with as much of the pericyst as possible are fundamental objectives of primary hydatid surgery. Conservative surgery (removal of the cyst contents plus partial pericystectomy with drainage when necessary) plus chemotherapy and local sterilization is suggested for both primary and secondary operations and appears to achieve satisfactory long-term results. Radical surgery (resection, cystopericystectomy) is preferred only in select patients. PMID:21939605

  3. Infection Control in Cystic Fibrosis

    PubMed Central

    Saiman, Lisa; Siegel, Jane

    2004-01-01

    Over the past 20 years there has been a greater interest in infection control in cystic fibrosis (CF) as patient-to-patient transmission of pathogens has been increasingly demonstrated in this unique patient population. The CF Foundation sponsored a consensus conference to craft recommendations for infection control practices for CF care providers. This review provides a summary of the literature addressing infection control in CF. Burkholderia cepacia complex, Pseudomonas aeruginosa, and Staphylococcus aureus have all been shown to spread between patients with CF. Standard precautions, transmission-based precautions including contact and droplet precautions, appropriate hand hygiene for health care workers, patients, and their families, and care of respiratory tract equipment to prevent the transmission of infectious agents serve as the foundations of infection control and prevent the acquisition of potential pathogens by patients with CF. The respiratory secretions of all CF patients potentially harbor clinically and epidemiologically important microorganisms, even if they have not yet been detected in cultures from the respiratory tract. CF patients should be educated to contain their secretions and maintain a distance of >3 ft from other CF patients to avoid the transmission of potential pathogens, even if culture results are unavailable or negative. To prevent the acquisition of pathogens from respiratory therapy equipment used in health care settings as well as in the home, such equipment should be cleaned and disinfected. It will be critical to measure the dissemination, implementation, and potential impact of these guidelines to monitor changes in practice and reduction in infections. PMID:14726455

  4. Cell therapy for cystic fibrosis.

    PubMed

    Murphy, Sean V; Atala, Anthony

    2015-03-01

    Currently there is no cure for cystic fibrosis (CF). Treatments are focused on addressing the disease symptoms, with varying degrees of success. Regenerative medicine holds the promise of regenerating dysfunctional or damaged tissues and to enhance the body's own endogenous repair mechanisms. The discovery of endogenous and exogenous stem cells has provided valuable tools for development of novel treatments for CF. The ability of stem cells to differentiate into functional pulmonary cells, modulate inflammatory responses and contribute to pulmonary function has provided researchers with multiple approaches to develop effective treatment strategies. Several approaches show promise to produce viable therapeutic treatments to treat the underlying cause of CF, reduce the symptoms and mitigate long-term damage, and generate functional replacement organs for end-stage transplantation. This review provides an overview of the rapidly progressing field of cell therapy for CF, focusing on the various cell types utilized and current strategies that show promise to improve life expectancy and quality of life for CF patients. PMID:23894126

  5. CXCR4+ granulocytes reflect fungal cystic fibrosis lung disease.

    PubMed

    Carevic, Melanie; Singh, Anurag; Rieber, Nikolaus; Eickmeier, Olaf; Griese, Matthias; Hector, Andreas; Hartl, Dominik

    2015-08-01

    Cystic fibrosis airways are frequently colonised with fungi. However, the interaction of these fungi with immune cells and the clinical relevance in cystic fibrosis lung disease are incompletely understood.We characterised granulocytes in airway fluids and peripheral blood from cystic fibrosis patients with and without fungal colonisation, non-cystic fibrosis disease controls and healthy control subjects cross-sectionally and longitudinally and correlated these findings with lung function parameters.Cystic fibrosis patients with chronic fungal colonisation by Aspergillus fumigatus were characterised by an accumulation of a distinct granulocyte subset, expressing the HIV coreceptor CXCR4. Percentages of airway CXCR4(+) granulocytes correlated with lung disease severity in patients with cystic fibrosis.These studies demonstrate that chronic fungal colonisation with A. fumigatus in cystic fibrosis patients is associated with CXCR4(+) airway granulocytes, which may serve as a potential biomarker and therapeutic target in fungal cystic fibrosis lung disease. PMID:25929952

  6. Genetics Home Reference: medullary cystic kidney disease type 1

    MedlinePlus

    ... disease type 1 medullary cystic kidney disease type 1 Enable Javascript to view the expand/collapse boxes. ... Close All Description Medullary cystic kidney disease type 1 (MCKD1) is an inherited condition that affects the ...

  7. Rehabilitation with Cystic Fibrosis: From Utopia to Reality.

    ERIC Educational Resources Information Center

    Goldberg, Richard T.; And Others

    1980-01-01

    The paper dispels some of the myths regarding cystic fibrosis (a genetic metabolism disorder), provides information on the latest developments in rehabilitation, summarizes research in the field, and projects future needs of the patient with cystic fibrosis. (SBH)

  8. Cystic Meningioma Masquerading as a Metastatic Tumor: A Case Report.

    PubMed

    Ramanathan, Nithya; Kamaruddin, Khairul Azmi; Othman, Aizzat; Mustafa, Fadhli; Awang, Mohamed Saufi

    2016-05-01

    Cystic meningioma is a rare form of intracranial meningioma. Meningiomas are typically solid tumors but may rarely have cystic components. The diagnosis of cystic meningioma is clinically challenging as the finding of multiple intra-axial tumors, including metastatic tumors, is relatively common. We report a case of cystic meningioma initially diagnosed as a metastatic tumor from a recurrence of acute lymphoid leukemia. However, postoperative histopathological examination demonstrated an atypical meningioma. PMID:27418876

  9. Cystic Meningioma Masquerading as a Metastatic Tumor: A Case Report

    PubMed Central

    Ramanathan, Nithya; Kamaruddin, Khairul Azmi; Othman, Aizzat; Mustafa, Fadhli; Awang, Mohamed Saufi

    2016-01-01

    Cystic meningioma is a rare form of intracranial meningioma. Meningiomas are typically solid tumors but may rarely have cystic components. The diagnosis of cystic meningioma is clinically challenging as the finding of multiple intra-axial tumors, including metastatic tumors, is relatively common. We report a case of cystic meningioma initially diagnosed as a metastatic tumor from a recurrence of acute lymphoid leukemia. However, postoperative histopathological examination demonstrated an atypical meningioma.

  10. Occult Mediastinal Yolk Sac Tumor Producing α-Fetoprotein Detected by 18F-FDG PET/CT.

    PubMed

    Liu, Meng; Chen, Guoqian; Fu, Zhanli; Li, Ziao; Li, Qian

    2016-07-01

    Malignant mediastinal yolk sac tumor (YST), especially adult onset, is rare. Herein, we report a case of occult mediastinal YST with α-fetoprotein production revealed by F-FDG PET/CT in a young adult, in which the intense tracer uptake was demonstrated in the anterior mediastinum. This case indicates F-FDG PET/CT may be a useful tool for detecting the occult primaries of YST. PMID:27088388

  11. Multilocular cystic renal tumor in children: radiologic-pathologic correlation.

    PubMed

    Agrons, G A; Wagner, B J; Davidson, A J; Suarez, E S

    1995-05-01

    Multilocular cystic renal tumor is a term that encompasses two histologically distinct but grossly indistinguishable lesions: cystic nephroma and cystic partially differentiated nephroblastoma (CPDN). Cystic nephroma is a segmental, purely cystic mass characterized by multiple septations composed entirely of differentiated tissues, without blastemal elements. CPDN is also a multiloculated lesion without nodular solid components, but its septa contain embryonal cells. Multilocular cystic tumors primarily affect boys during early childhood, with a substantial number of the lesions containing blastema (CPDN), and adult women, with lesions that more commonly lack septal blastema (cystic) nephroma). As a rule, nephrectomy is curative and the clinical course benign, but CPDN may recur locally. Although cystic nephroma and CPDN cannot be distinguished radiologically, failure to do so has no practical impact on management, since all of these tumors are surgically removed. However, the differential diagnosis includes other pediatric cystic renal masses that may require different treatment stratagems: Wilms tumor with cyst formation due to hemorrhage and necrosis, cystic clear cell sarcoma, cystic mesoblastic nephroma, cystic renal cell carcinoma, multicystic dysplastic kidney, and segmental multicystic dysplasia in a duplicated renal collecting system. PMID:7624570

  12. Living with Cystic Fibrosis: A Guide for the Young Adult.

    ERIC Educational Resources Information Center

    Cystic Fibrosis Foundation, Atlanta, GA.

    Intended for the young adult with cystic fibrosis, the booklet provides information on dealing with problems and on advances in treatment and detection related to the disease. Addressed are the following topics: description of cystic fibrosis; inheritance of cystic fibrosis; early diagnosis; friends, careers, and other matters; treatment;…

  13. Acute Scedosporium apiospermum Endobronchial Infection in Cystic Fibrosis.

    PubMed

    Padoan, Rita; Poli, Piercarlo; Colombrita, Domenico; Borghi, Elisa; Timpano, Silviana; Berlucchi, Marco

    2016-06-01

    Fungi are known pathogens in cystic fibrosis patients. A boy with cystic fibrosis boy presented with acute respiratory distress. Bronchoscopy showed airways obstruction by mucus plugs and bronchial casts. Scedosporium apiospermum was identified as the only pathogen. Bronchoalveolar lavage successfully resolved the acute obstruction. Plastic bronchitis is a new clinical picture of acute Scedosporium endobronchial colonization in cystic fibrosis patients. PMID:26967814

  14. Premalignant cystic neoplasms of the pancreas.

    PubMed

    Dudeja, Vikas; Allen, Peter J

    2015-02-01

    Due to increasing utilization of cross-sectional imaging, asymptomatic pancreatic cysts are frequently being diagnosed. Many of these cysts have premalignant potential and offer a unique opportunity for cancer prevention. Mucinous cystic neoplasm and intraductal papillary mucinous neoplasm are the major premalignant cystic neoplasms of pancreas. The prediction of the risk of malignancy (incidental and future risk of malignant transformation) and balancing the risks of watchful waiting with that of operative management with associated mortality and morbidity is the key to the management of these lesions. We review the literature that has contributed to the development of our approach to the management of these cystic neoplasms. We provide an overview of the key features used in diagnosis and in predicting malignancy. Particular attention is given to the natural history and management decision making. PMID:25726053

  15. Do brine shrimp diagnose cystic fibrosis?

    PubMed

    Hodes, M E; Thomas, J; Morgan, S; Merritt, A D

    1975-11-01

    The nauplii of the brine shrimp Artemia salina are dependent upon the function of their salt gland to maintain osmotic pressure within narrow limits. A number of drugs interfere with this function and are lethal to the nauplii. Saliva and serum from normal persons, patients with cystic fibrosis, and obligate heterozygotes were tested for lethal effect against brine shrimp nauplii. At salt concentrations between 100 mM and 2.5 no difference was found among the phenotypes. At lower concentrations a difference was noted occasionally between some normal subjects and some individuals carrying one or two genes for cystic fibrosis. Data from an independent series of experiments indicate that the naupliar deaths result from distorted ratios of Na+/K+ and not from a specific gene product. No difference was noted in the O2 uptake of nauplii treated with saliva or serum obtained from normal subjects, patients with cystic fibrosis, or obligate heterozygotes. PMID:1187245

  16. Unique origin of the cystic artery.

    PubMed

    Hlaing, K P P; Thwin, S S; Shwe, N

    2011-12-01

    The cystic artery (CA) is known to exhibit variations in its origin and branching pattern. This is attributed to the developmental changes occurring in the primitive ventral splanchnic arteries. During routine dissection of a male cadaver, we observed that the CA originated from the middle hepatic artery (MHA) at a distance of about 1 cm from its origin, and the MHA originated from the right hepatic artery at a distance of 2.1 cm from its origin. The CA traversed for a distance of 1.5 cm, giving off a branch to the cystic duct. It then passed anterior to the cystic duct. The origin of the CA was located to the left of the common hepatic duct, outside the Calot's triangle. The topographical anatomy of the arterial system of the hepatobiliary region and their anomalous origin should be considered during hepatobiliary surgeries. This knowledge is also important for interventional radiologists in routine clinical practice. PMID:22159949

  17. Cystic Fibrosis Transmembrane Conductance Regulator

    PubMed Central

    Smith, Stephen S.; Steinle, Erich D.; Meyerhoff, Mark E.; Dawson, David C.

    1999-01-01

    The cystic fibrosis transmembrane conductance regulator (CFTR) Cl channel exhibits lyotropic anion selectivity. Anions that are more readily dehydrated than Cl exhibit permeability ratios (PS/PCl) greater than unity and also bind more tightly in the channel. We compared the selectivity of CFTR to that of a synthetic anion-selective membrane [poly(vinyl chloride)–tridodecylmethylammonium chloride; PVC-TDMAC] for which the nature of the physical process that governs the anion-selective response is more readily apparent. The permeability and binding selectivity patterns of CFTR differed only by a multiplicative constant from that of the PVC-TDMAC membrane; and a continuum electrostatic model suggested that both patterns could be understood in terms of the differences in the relative stabilization of anions by water and the polarizable interior of the channel or synthetic membrane. The calculated energies of anion–channel interaction, derived from measurements of either permeability or binding, varied as a linear function of inverse ionic radius (1/r), as expected from a Born-type model of ion charging in a medium characterized by an effective dielectric constant of 19. The model predicts that large anions, like SCN, although they experience weaker interactions (relative to Cl) with water and also with the channel, are more permeant than Cl because anion–water energy is a steeper function of 1/r than is the anion–channel energy. These large anions also bind more tightly for the same reason: the reduced energy of hydration allows the net transfer energy (the well depth) to be more negative. This simple selectivity mechanism that governs permeability and binding acts to optimize the function of CFTR as a Cl filter. Anions that are smaller (more difficult to dehydrate) than Cl are energetically retarded from entering the channel, while the larger (more readily dehydrated) anions are retarded in their passage by “sticking” within the channel. PMID:10578016

  18. Drug disposition in cystic fibrosis.

    PubMed

    Rey, E; Tréluyer, J M; Pons, G

    1998-10-01

    There are many pathological changes in patients with cystic fibrosis (CF) which can lead to alterations in drug disposition. Although, in patients with CF, the extent of drug absorption varies widely and the rate of absorption is slower, bioavailability is not altered. Plasma protein binding for the majority of drugs studied did not differ in patients with CF compared with control groups. The difference in volume of distribution of most drugs between patients with CF and healthy individuals vanished when corrected for lean body mass. Despite hepatic dysfunction, patients with CF have enhanced clearance of many, but not all, drugs. Phase I mixed-function oxidases are selectively affected: cytochrome P450 (CYP) 1A2 and CYP2C8 have enhanced activity, while other CYP isoforms such as CYP2C9 and CYP3A4 are unaffected. Increased phase II activities are also demonstrated: glucuronyl transferase, acetyl transferase (NAT1) and sulfotransferase. The increased hepatic clearance of drugs in the presence of CF may be the consequence of disease-specific changes in both enzyme activity and/or drug transport within the liver. The renal clearance (CLR) of many drugs in patients with CF is enhanced although there has been no pathological abnormality identified which could explain this finding: glomerular filtration rate and tubular secretion appear normal in patients with CF. The precise mechanisms for enhanced drug clearance in patients with CF remain to be elucidated. The optimisation of antibiotic therapy in patients with CF includes increasing the dose of beta-lactams by 20 to 30% and monitoring plasma concentrations of aminoglycosides. The appropriate dosage of quinolones has not been definitively established. PMID:9812180

  19. Solitary mediastinal lymph node recurrence after curative resection of colon cancer.

    PubMed

    Matsuda, Yasuhiro; Yano, Masahiko; Miyoshi, Norikatsu; Noura, Shingo; Ohue, Masayuki; Sugimura, Keijiro; Motoori, Masaaki; Kishi, Kentaro; Fujiwara, Yoshiyuki; Gotoh, Kunihito; Marubashi, Shigeru; Akita, Hirofumi; Takahashi, Hidenori; Sakon, Masato

    2014-08-27

    We report two cases of solitary mediastinal lymph node recurrence after colon cancer resection. Both cases had para-aortic lymph node metastasis at the time of initial surgery and received adjuvant chemotherapy for 4 years in case 1 and 18 mo in case 2. The time to recurrence was more than 8 years in both cases. After resection of the recurrent tumor, the patient is doing well with no recurrence for 6 years in case 1 and 4 mo in case 2. Patients should be followed up after colon cancer surgery considering the possibility of solitary mediastinal lymph node recurrence if they had para-aortic node metastasis at the time of initial surgery. PMID:25161766

  20. A Case of Ruptured Aneurysm of the Proper Esophageal Artery with Symptomatic Mediastinal Hematoma.

    PubMed

    Liu, Jiajia; Sato, Yusuke; Takahashi, Satoshi; Motoyama, Satoru; Yoshino, Kei; Sasaki, Tomohiko; Imai, Kazuhiro; Saito, Hajime; Minamiya, Yoshihiro

    2016-08-01

    Mediastinal aneurysms are rare but potentially life-threatening. Among these, bronchial artery aneurysms are most frequently reported, whereas up to now aneurysms of the proper esophageal artery had never been reported. A 69-year-old woman was referred to our hospital for treatment of a massive mediastinal hematoma. Enhanced computed tomography and selective proper esophageal arteriography revealed a 5-mm aneurysm in the proper esophageal artery that arises from the thoracic aorta at the Th8 level and has an anastomotic branch with the bronchial artery peripherally. Transcatheter arterial embolization was successfully performed using a mixture of N-butyl cyanoacrylate and lipiodol (1:3 ratio, 0.3 ml). Post-embolization angiography showed no filling into the aneurysm. The patient recovered with no complications and was discharged on the 25th post-procedure day. PMID:27094689

  1. [Mediastinal Nonseminomatous Germ Cell Tumor with Sarcoid-like Reaction in the Regional Lymph Nodes].

    PubMed

    Hamada, Akira; Kanauchi, Naoki; Suzuki, Katsuyuki; Watanabe, Hikaru; Watanabe, Isamu

    2015-06-01

    A 26-year-old man was admitted because of an abnormal shadow on a chest roentgenogram. Computed tomography(CT) revealed a very large tumor in the anterior mediastinum and bilateral mediastinal lymphadenopathy. Examination of a CT-guided biopsy specimen revealed a yolk-sac tumor. The patient received 4 courses of bleomycin, etoposide, and cisplatin chemotherapy. After chemotherapy, the tumor was markedly reduced in size, but the lymphadenopathy remained. The patient underwent thoracoscopic biopsy of the mediastinal lymph nodes. Sarcoid nodules were found in all the biopsied nodes, and the lymphadenopathy was thought to be a sarcoid-like reaction associated with the germ cell tumor. Resection of the residual tumor was performed according to the treatment algorithm of the International Germ Cell Cancer Collaborative Group. There were no viable tumor cells in the resected tissue. The patient is free of recurrence and without any sign of generalized sarcoidosis 3 years after the surgery. PMID:26066869

  2. Mediastinal mass-a rare presentation of desmoplastic small round cell tumour

    PubMed Central

    Nayak, Hemanta K; Vangipuram, Deepak Rajkumar; Sonika, Ujjwal; Kar, Premashish; Kumar, Naresh; Kapoor, Neha

    2011-01-01

    Primary mediastinal desmoplastic small round cell cancer is an uncommon tumour usually located in the abdomen and pelvis. Here the authors report an extremely rare case of a young male with a primary desmoplastic small round cell tumour in the anterior and middle mediastinum. The patient had non-specific complaints but an abnormal shadow was seen in a routine chest x-ray. He was diagnosed as having mediastinal mass with few lung parenchymal deposits on CT. Mediastinoscopy and guided biopsy revealed desmoplastic small round cell tumour. Desmoplastic small round cell tumour is a rare and aggressive tumour which rarely involves the mediastinum as a primary site. The nature of the lesion and its prognosis were explained to the patient. He was offered chemotherapy and radiotherapy for the tumour management. He refused treatment and left against medical advice. PMID:22670008

  3. Mediastinal infusion with tracheal necrosis: an unusual complication of Port-a-cath devices.

    PubMed

    Renaud, Stéphane; Santelmo, Nicola; Falcoz, Pierre Emmanuel; Massard, Gilbert

    2011-06-01

    The Port-a-cath (PAC) is a catheter totally implanted under the skin. It is commonly used in oncology for permanent venous access. It provides a more simple way to infuse chemotherapies, antibiotics or parenteral nutrition, while offering improved comfort to patients. The usual complications of these devices (infections and catheter obstructions) are well documented. More exceptional events are catheter fractures with systemic migration, and endopleural perfusions due to a wrong positioning of the catheter. Since 1998, 10 cases of mediastinal infusion of cytotoxics have been reported. Surgical management was necessary in only two cases. We are reporting the case of a 57-year-old female suffering from a multimetastatic sigmoid adenocarcinoma. A mediastinal infusion of Folfiri and bevacizumab with a tracheal necrosis complicated the PAC use and required a latissimus dorsi myoplasty to fill up the tracheo-bronchial defect. PMID:21362735

  4. A case of TAFRO syndrome with a large mediastinal mass treated with debulking surgery.

    PubMed

    Nagano, Masaaki; Matsumoto, Jun

    2016-12-01

    Multicentric Castleman's disease is a polyclonal lymphoproliferative disorder. Recently, a new variant of the disease was reported and named TAFRO syndrome, an acronym for thrombocytopenia, ascites, myelofibrosis, renal dysfunction, and organomegaly. A 55-year-old woman presented to our hospital with dyspnea on exertion and high fever. Laboratory tests revealed anemia, thrombocytopenia, and proteinuria. Computed tomography (CT) revealed a large anterior mediastinal mass, mild splenomegaly, bilateral pleural effusion, pericardial effusion, and mild systemic lymphadenopathy. A CT-guided biopsy was unable to establish a definitive diagnosis, so we resected the mediastinal mass for diagnostic and therapeutic purposes. Pathological findings were consistent with the hyaline vascular type of Castleman's disease (CD), and she was diagnosed with TAFRO syndrome. There has been no description of a patient with TAFRO syndrome with a large mass, and this is the first case of TAFRO syndrome treated with debulking surgery. PMID:27316721

  5. Thoracic CT scanning for mediastinal Hodgkin's disease: results and therapeutic implications

    SciTech Connect

    Rostock, R.A.; Siegelman, S.S.; Lenhard, R.E.; Wharam, M.D.; Order, S.E.

    1983-10-01

    Thoracic CT scans were performed on 42 newly diagnosed patients with Hodgkin's disease. Five of 10 patients with negative chest X ray (CXR) had abnormal thoracic CT scans. Among the remaining 32 patients with mediastinal Hodgkin's disease (MHD) on CXR, pericardial (Ep) and chest wall invasion (Ec) were the two most common sites of involvement which were detectable by CT scan alone. Ep and Ec accounted for 16 of 19 of the changes in treatment portal or philosophy based on CT scan findings. Because of the high risk of cardiac or pulmonary radiation toxicity in Ep or Ec, radiation treatment alone may be inadequate. Treatment of mediastinal Hodgkin's disease is reviewed here. The use of CT scans for identification of Ep, Ec, and other abnormalities will allow for more precise treatment, further define the use of conventional radiotherapy, combined modality therapy or whole lung irradiation, and allow more accurate analysis of treatment results.

  6. Mediastinal Yolk Sac Tumor Producing Protein Induced by Vitamin K Absence or Antagonist-II.

    PubMed

    Akutsu, Noriyuki; Adachi, Yasushi; Isosaka, Mai; Mita, Hiroaki; Takagi, Hideyasu; Sasaki, Shigeru; Yamamoto, Hiroyuki; Arimura, Yoshiaki; Ishii, Yoshifumi; Masumori, Naoya; Endo, Takao; Shinomura, Yasuhisa

    2015-01-01

    Extragonadal yolk sac tumors (YSTs) are rare. We herein report the case of a 66-year-old man with mediastinal, lung and liver tumors. The largest mass was located in the liver and contained a high concentration of protein induced by vitamin K absence or antagonist-II (PIVKA-II) and alpha-fetoprotein. Therefore, the lesion was difficult to distinguish from hepatocellular carcinoma. Finally, YST was diagnosed based on the results of a liver biopsy. Although chemotherapy was effective, the patient died of respiratory failure. The autopsy revealed primary mediastinal YST. In the current report, we describe this case of PIVKA-II-producing YST and review previous cases of PIVKA-II-producing tumors other than hepatoma. PMID:26073245

  7. Facial, Cervical, and Mediastinal Emphysema of the Clarinet Player: Case Report

    PubMed Central

    Biçer, Yusuf Özgür; Kesgin, Selcan; Tezcan, Erkan; Köybaşı, Serap

    2014-01-01

    Background: Cervicofacial emphysema may arise due to the leakage of air from a defect in the aerodigestive tract to the fascial layers of neck and face. Rarely, it may be caused by insufflation of air through the Stensen’s duct. Case Report: We present a case with diffuse facial, cervical and mediastinal emphysema due to playing a wind instrument immediately after a facial trauma. There was no mucosal defect or laceration noticed by examination which could explain the origin of the emphysema. Despite the widespread cervicofacial emphysema with mediastinal involvement, the patient significantly improved within 48 hours without any intervention. Conclusion: Even though cervicofacial emphysema ameliorates spontaneously, increased care must be taken, especially when there is pneumomediastinum and/or pneumothorax. PMID:25667794

  8. Management issues for adolescents with cystic fibrosis.

    PubMed

    Withers, Adelaide Lindsay

    2012-01-01

    The healthy adolescent will encounter major changes in biological and psychosocial domains. The adolescent period can be greatly affected by a chronic illness. Cystic fibrosis is a terminal illness that can significantly affect an adolescent's biological, mental and psychosocial health. This paper discusses general issues to consider when managing an adolescent with a chronic medical condition, and specifically how cystic fibrosis may impact upon puberty, body image, risk-taking behaviours, mental health, independence, nonadherence, reproductive health, transition, lung transplantation, and end of life care. PMID:22991662

  9. Cystic fibrosis, intravenous antibiotics, and home therapy.

    PubMed

    Hammond, L J; Caldwell, S; Campbell, P W

    1991-01-01

    The survival rate of patients with cystic fibrosis has improved considerably in the last 20 years. Although not all of the factors accounting for this change are understood, aggressive nutritional management and treatment of pulmonary exacerbations certainly play a role. Home intravenous (IV) antibiotic delivery for pulmonary exacerbation has proved to be as effective as hospital treatment and offers significant advantages to the patient and family. This article examines the microbiology of pulmonary infections in patients with cystic fibrosis, as well as antimicrobial therapy, methods of IV administration, home IV therapy, and the nurse practitioner's role in this home program in the future. PMID:1990112

  10. Diagnosis of Adult Patients with Cystic Fibrosis.

    PubMed

    Nick, Jerry A; Nichols, David P

    2016-03-01

    The diagnosis of cystic fibrosis (CF) is being made with increasing frequency in adults. Patients with CF diagnosed in adulthood typically present with respiratory complaints, and often have recurrent or chronic airway infection. At the time of initial presentation individuals may appear to have clinical manifestation limited to a single organ, but with subclinical involvement of the respiratory tract. Adult-diagnosed patients have a good response to CF center care, and newly available cystic fibrosis transmembrane receptor-modulating therapies are promising for the treatment of residual function mutation, thus increasing the importance of the diagnosis in adults with unexplained bronchiectasis. PMID:26857767

  11. Precision Genomic Medicine in Cystic Fibrosis.

    PubMed

    Chang, Eugene H; Zabner, Joseph

    2015-10-01

    The successful application of precision genomic medicine requires an understanding of how a person's genome can influence his or her disease phenotype and how medical therapies can provide personalized therapy to one's genotype. In this review, we highlight advances in precision genomic medicine in cystic fibrosis (CF), a classic autosomal recessive genetic disorder. We discuss genotype-phenotype correlations in CF, genetic and environmental modifiers of disease, and pharmacogenetic therapies that target specific genetic mutations thereby addressing the primary defect of cystic fibrosis. PMID:26073768

  12. Genetics of Cystic Fibrosis: Clinical Implications.

    PubMed

    Egan, Marie E

    2016-03-01

    Cystic fibrosis (CF) is a common life-shortening autosomal recessive genetic disorder caused by mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator protein (CFTR). Almost 2000 variants in the CFTR gene have been identified. The mutational classes are based on the functional consequences on CFTR. New therapies are being developed to target mutant CFTR and restore CFTR function. Understanding specific CF genotypes is essential for providing state-of-the art care to patients. In addition to the variation in CFTR genotype, there are several modifier genes that contribute to the respiratory phenotype. PMID:26857764

  13. Incidentally Detected Mediastinal Vascular Anatomical Variants on PET-CT: A Pictorial Essay

    PubMed Central

    Paidipati, Kathyayini Gopalkrishna Murthy; Kashyap, Raghava; Tadimeti, Hima; Peyyeti, Mahidhar

    2015-01-01

    Positron emission tomography with computed tomography (PET–CT) is an integral part of oncology practice in the current-day scenario. The hybrid anatomical and metabolic imaging information in this imaging modality provides access to a wealth of incidental findings which can have implications in the management of the patient, especially when surgical options are being considered. In this pictorial essay, we present incidentally detected mediastinal vascular anomalies on PET–CT, along with a review of associated literature. PMID:25883859

  14. The value of computed tomography in the diagnosis and management of pediatric mediastinal abnormalities

    SciTech Connect

    Siegel, M.J.; Sagel, S.S.; Reed, K.

    1982-01-01

    The utility of computed tomography (CT) in evaluation of mediastinal abnormalities was assessed in 23 pediatric patients. By comparison with standard chest radiography, CT provided additional diagnostic information in 82% of the patients. In 65%, the CT findings contributed to a change in clinical management. CT was most useful in documenting a benign process (near-water-density mass or a normal vascular or soft-tissue structure) and in demonstrating the full extent of a malignant tumor.

  15. Transcriptional Profiling of Endobronchial Ultrasound-Guided Lymph Node Samples Aids Diagnosis of Mediastinal Lymphadenopathy

    PubMed Central

    Tomlinson, Gillian S.; Thomas, Niclas; Chain, Benjamin M.; Best, Katharine; Simpson, Nandi; Hardavella, Georgia; Brown, James; Bhowmik, Angshu; Navani, Neal; Janes, Samuel M.; Miller, Robert F.; Noursadeghi, Mahdad

    2016-01-01

    Background Endobronchial ultrasound (EBUS)-guided biopsy is the mainstay for investigation of mediastinal lymphadenopathy for laboratory diagnosis of malignancy, sarcoidosis, or TB. However, improved methods for discriminating between TB and sarcoidosis and excluding malignancy are still needed. We sought to evaluate the role of genomewide transcriptional profiling to aid diagnostic processes in this setting. Methods Mediastinal lymph node samples from 88 individuals were obtained by EBUS-guided aspiration for investigation of mediastinal lymphadenopathy and subjected to transcriptional profiling in addition to conventional laboratory assessments. Computational strategies were used to evaluate the potential for using the transcriptome to distinguish between diagnostic categories. Results Molecular signatures associated with granulomas or neoplastic and metastatic processes were clearly discernible in granulomatous and malignant lymph node samples, respectively. Support vector machine (SVM) learning using differentially expressed genes showed excellent sensitivity and specificity profiles in receiver operating characteristic curve analysis with area under curve values > 0.9 for discriminating between granulomatous and nongranulomatous disease, TB and sarcoidosis, and between cancer and reactive lymphadenopathy. A two-step decision tree using SVM to distinguish granulomatous and nongranulomatous disease, then between TB and sarcoidosis in granulomatous cases, and between cancer and reactive lymphadenopathy in nongranulomatous cases, achieved > 90% specificity for each diagnosis and afforded greater sensitivity than existing tests to detect TB and cancer. In some diagnostically ambiguous cases, computational classification predicted granulomatous disease or cancer before pathologic abnormalities were evident. Conclusions Machine learning analysis of transcriptional profiling in mediastinal lymphadenopathy may significantly improve the clinical utility of EBUS

  16. Colon cancer metastasis to mediastinal lymph nodes without liver or lung involvement: A case report.

    PubMed

    El-Halabi, Mustapha M; Chaaban, Said A; Meouchy, Joseph; Page, Seth; Salyers, William J

    2014-11-01

    Colon cancer is the second most common type of cancer in females and the third in males, worldwide. The most common sites of colon cancer metastasis are the regional lymph nodes, liver, lung, bone and brain. In this study, an extremely rare case of colon adenocarcinoma with extensive metastasis to the mediastinal lymph nodes without any other organ involvement is presented. A 44-year-old Caucasian male presented with abdominal pain, a change in bowel habits, melena and weight loss. Colonoscopy revealed a large friable, ulcerated, circumferential mass in the ascending colon. Biopsies were consistent with the diagnosis of invasive moderately differentiated adenocarcinoma. Subsequently, right colon resection was performed, and pathological analysis revealed moderately differentiated adenocarcinoma of the right colon with extensive regional lymph node involvement. Computed tomography (CT) scans of the chest, abdomen and pelvis were performed preoperatively as part of routine staging for colon cancer. No liver or lung pathology was identified; however, multiple pathologically enlarged mediastinal lymph nodes were observed. Endoscopic ultrasound with fine needle aspiration of the largest mediastinal lymph node, which measured 5.2×3.5 cm on CT scans, was performed. The pathology was again consistent with the diagnosis of metastatic colorectal primary adenocarcinoma. At present, no optimum treatment has been identified for metastatic colon cancer to the mediastinal lymph nodes. The patient in the current case received chemotherapy with folinic acid, fluorouracil and oxaliplatin (FOLFOX), as well as with bevacizumab. Initial follow-up CT scans of the chest revealed a positive response to treatment. Physicians, in particular, radiologists, must consider the mediastinum during the first evaluation and further follow-up of patients with colorectal carcinoma even in the absence of metastasis. PMID:25289100

  17. Aggressive solitary intracranial metastatic malignant melanoma from a primary mediastinal tumour.

    PubMed

    Sivaraju, Laxminadh; Aryan, Saritha; Hegde, Vinay S; Ghosal, Nandita; Hegde, Alangar S

    2016-08-01

    Malignant melanoma is the third most common tumour to cause cerebral metastases, following breast and lung cancer. Central nervous system metastases occur in 10-40% of patients with melanoma. Intracranial metastasis from a primary malignant melanoma of the anterior mediastinum is uncommon. We report a case of solitary intracranial metastatic melanoma arising from a primary mediastinal tumour. We then discuss the clinico-radiological features and treatment options. PMID:27145991

  18. Concurrent Myelomatous Pleural Effusion and Extramedullary Mediastinal Involvement as an Initial Manifestation of Multiple Myeloma

    PubMed Central

    Williams, George; Kadaria, Dipen; Sodhi, Amik

    2016-01-01

    Patient: Female, 72 Final Diagnosis: Myelomatous pleural effusion Symptoms: Dyspnea Medication: — Clinical Procedure: Thoracentesis Specialty: Pulmonology Objective: Rare disease Background: Myelomatous pleural effusion (MPE) is a rare occurrence in patients with multiple myeloma (MM). Fewer than 20 cases of MPE have been reported as an initial manifestation of MM. Extramedullary plasmacytoma (EMP) occurs in fewer than 5% patients with MM, and mediastinal EMP is even rarer, with only about 80 cases reported in the literature. We present a case study involving a patient with concurrent MPE and mediastinal EMP as an initial manifestation of MM. Case Report: The patient was a 74-year-old nonsmoking female with a 3-month history of exertional dyspnea and back pain. On exam, the patient was afebrile (temperature 37.2°C), blood pressure was 160/74 mm Hg, heart rate was 92 bpm, respiratory rate was 22/min, and oxygen saturation was 87% on room air. Patient was in mild distress and had decreased breath sounds over right lung fields about halfway up with dullness to percussion. Computed tomography of the chest showed a moderate-sized right pleural effusion and an anterior mediastinal mass. Thoracentesis showed a lymphocyte-predominant exudate. Cytology showed numerous plasma cells including immature forms. Stains for CD138 were positive, confirming plasma cell origin of cells. The anterior mediastinal mass was also biopsied and showed diffuse infiltrate of lymphocytes with plasma cell features that were also positive for CD138. Systemic protein electrophoresis showed a monoclonal immunoglobulin G kappa spike, and bone marrow biopsy was consistent with MM. Conclusions: MPE and EMP are extremely rare manifestations in MM. In addition, it is extremely rare for these to be the presenting features of MM. We report concurrently occurring MPE and EMP in a patient as her initial manifestation of MM. PMID:27396960

  19. [Aspergillus fumigatus mediastinitis in an immunocompetent pediatric patient after heart surgery].

    PubMed

    Acuña, Mirta; Farfán, Felipe; Cofré, Fernanda; Benadof, Dona

    2016-02-01

    Postsurgical aspergillosis occurs primarily in immunocompetent patients whose main predisposing factor is the loss of skin and mucosal integrity during surgery. Local infection tends to be destructive and refractory to treatment and relapses are common. It is important to consider aspergillosis in the differential diagnosis of slowly progressive and destructive surgical site infections with negative bacterial cultures. We present the case of a child who developed Aspergillus fumigatus mediastinitis months after heart surgery. PMID:26965883

  20. Concurrent Myelomatous Pleural Effusion and Extramedullary Mediastinal Involvement as an Initial Manifestation of Multiple Myeloma.

    PubMed

    Williams, George; Kadaria, Dipen; Sodhi, Amik

    2016-01-01

    BACKGROUND Myelomatous pleural effusion (MPE) is a rare occurrence in patients with multiple myeloma (MM). Fewer than 20 cases of MPE have been reported as an initial manifestation of MM. Extramedullary plasmacytoma (EMP) occurs in fewer than 5% patients with MM, and mediastinal EMP is even rarer, with only about 80 cases reported in the literature. We present a case study involving a patient with concurrent MPE and mediastinal EMP as an initial manifestation of MM. CASE REPORT The patient was a 74-year-old nonsmoking female with a 3-month history of exertional dyspnea and back pain. On exam, the patient was afebrile (temperature 37.2°C), blood pressure was 160/74 mm Hg, heart rate was 92 bpm, respiratory rate was 22/min, and oxygen saturation was 87% on room air. Patient was in mild distress and had decreased breath sounds over right lung fields about halfway up with dullness to percussion. Computed tomography of the chest showed a moderate-sized right pleural effusion and an anterior mediastinal mass. Thoracentesis showed a lymphocyte-predominant exudate. Cytology showed numerous plasma cells including immature forms. Stains for CD138 were positive, confirming plasma cell origin of cells. The anterior mediastinal mass was also biopsied and showed diffuse infiltrate of lymphocytes with plasma cell features that were also positive for CD138. Systemic protein electrophoresis showed a monoclonal immunoglobulin G kappa spike, and bone marrow biopsy was consistent with MM. CONCLUSIONS MPE and EMP are extremely rare manifestations in MM. In addition, it is extremely rare for these to be the presenting features of MM. We report concurrently occurring MPE and EMP in a patient as her initial manifestation of MM. PMID:27396960

  1. Challenging posterior mediastinal mass resection via a minimally invasive approach with neurological monitoring.

    PubMed

    Smail, Hassiba; Baste, Jean Marc; Melki, Jean; Peillon, Christophe

    2013-02-01

    We report a novel surgical strategy for the resection of a rare type of posterior mediastinal tumour in a young patient. A melanotic schwannoma arose from the left thoracic sympathetic chain, adjacent to the origin of the artery of Adamkiewicz. Successful excision of this tumour via a minimally invasive approach without arterial or spinal cord injury was possible with the aid of neurological monitoring using spinal-evoked potentials. PMID:23190618

  2. Mediastinal follicular dendritic cell sarcoma involving bone marrow: a case report and review of the literature.

    PubMed

    Jiang, Liuyan; Admirand, Joan H; Moran, Cesar; Ford, Richard J; Bueso-Ramos, Carlos E

    2006-12-01

    We report a rare case of mediastinal follicular dendritic cell (FDC) sarcoma involving the bone marrow. The patient, a 46-year-old woman, had a clinically aggressive tumor in the anterior mediastinum that was initially diagnosed as a diffuse B-cell lymphoma. She received chemotherapy but showed no significant improvement. One year later, the patient presented at our institution with pelvic bone metastases. Biopsy specimens of the sacrum lesion and bone marrow were obtained. The diagnosis of FDC sarcoma was made based on histological examination and immunohistochemical findings, including strong positive staining of tumor cells for CD21, CD23, clusterin, and epidermal growth factor receptor (EGFR) and negative staining for CD20, CD30, CD45, CD1a, S-100, vimentin, and keratin cocktail. Histological examination and immunohistochemical studies of a previous biopsy of the mediastinal mass confirmed the diagnosis of mediastinal FDC sarcoma. The patient was treated with an appropriate chemotherapy regimen; 1 month later, follow-up bone marrow biopsy revealed no tumor cells. Although FDC sarcoma is considered a low-grade tumor, the tumor in the present case not only developed at an unusual location with bone metastasis but also involved bone marrow. To our knowledge, this is the first such case ever reported. This case also highlights the utility of EGFR as an immunohistochemical marker of dendritic cell tumors that could be used as a diagnostic tool and guide for choosing appropriate chemotherapy regimens. PMID:17126255

  3. Rationale for and Preliminary Results of Proton Beam Therapy for Mediastinal Lymphoma

    SciTech Connect

    Li Jing; Dabaja, Bouthaina; Reed, Valerie; Allen, Pamela K.; Cai, Haihong; Amin, Mayankkumar V.; Garcia, John A.; Cox, James D.

    2011-09-01

    Purpose: To evaluate the potential of three-dimensional proton beam therapy (3D-PBT) for reducing doses to normal structures in patients with mediastinal lymphomas compared with conventional photon radiation therapy (RT). Methods and Materials: We treated 10 consecutive patients with mediastinal masses from lymphomas with 3D-PBT between July 2007 and February 2009 to 30.6-50.4 cobalt-Gray equivalents (CGE). Of those patients, 7 had primary refractory or recurrent disease, and 8 had Hodgkin lymphoma. Dosimetric endpoints were compared with those from conventional RT plans. Results: PBT delivered lower mean doses to the lung (6.2 vs. 9.5 Gy), esophagus (9.5 vs. 22.3 Gy), and heart (8.8 vs. 17.7 Gy) but not the breasts (5.9 vs. 6.1 Gy) than did conventional RT. Percentages of lung, esophagus, heart, and coronary artery (particularly the left anterior descending artery) volumes receiving radiation were consistently lower in the 3D-PBT plans over a wide range of radiation doses. Of the 7 patients who had residual disease on positron emission tomography before PBT, 6 (86%) showed a complete metabolic response. Conclusions: In patients with mediastinal lymphomas, 3D-PBT produced significantly lower doses to the lung, esophagus, heart, and coronary arteries than did the current conventional RT. These lower doses would be expected to reduce the risk of late toxicities in these major organs.

  4. Primary malignant mediastinal germ cell tumours: improved prognosis with platinum-based chemotherapy and surgery.

    PubMed Central

    Childs, W. J.; Goldstraw, P.; Nicholls, J. E.; Dearnaley, D. P.; Horwich, A.

    1993-01-01

    A retrospective analysis was performed of 18 patients with primary malignant germ cell tumours of the mediastinum treated with platinum-based chemotherapy between 1977 and 1990. All seven patients with pure seminoma were treated initially with chemotherapy and four of these patients received additional mediastinal radiotherapy. Only one patient relapsed; his initial therapy had included radiotherapy and single-agent carboplatin and he was successfully salvaged with combination chemotherapy. With a follow-up of 11 to 117 months (median 41 months) all seven patients with seminoma remain alive and disease free giving an overall survival of 100%. Eleven patients had malignant non seminoma; following chemotherapy eight of these had elective surgical resection of residual mediastinal masses. Complete remission was achieved in nine (82%) patients, however, one of these patients died from bleomycin pneumonitis. With a follow-up of 12 to 113 months (median 55 months) eight of 11 (73%) patients with malignant mediastinal teratoma remain alive and disease free. PMID:8494705

  5. An anterior mediastinal lesion in TAFRO syndrome showing complete remission after glucocorticoid and tocilizumab therapy.

    PubMed

    Sakashita, Kentaro; Murata, Kengo; Inagaki, Yuji; Oota, Souichi; Takamori, Mikio

    2016-09-01

    Thrombocytopenia (T), anasarca (A), myelofibrosis (F), renal dysfunction (R), and organomegaly (O) (TAFRO) syndrome is a variant of multicentric Castleman's disease. We describe here a 57-year-old man who presented with persistent fever, pleural effusion, and ascites. He was negative for human immunodeficiency virus and human herpes virus-8. A computed tomography scan showed an anterior mediastinal mass and small inguinal lymphadenopathy. Although a biopsy of the anterior mediastinum showed fatty tissue infiltrated with CD20 (+) and CD45RO (+) lymphocytes, a biopsy of the left inguinal lymph node revealed a hyaline vascular type of Castleman's disease. He subsequently developed severe thrombocytopenia and renal dysfunction. In addition, his bone marrow biopsy showed myelofibrosis. TAFRO syndrome was diagnosed based on the lymph node pathology and the characteristic manifestations of the syndrome. Tocilizumab and glucocorticoid therapy achieved complete remission and regression of the mediastinal mass. To our knowledge, this is the first report of TAFRO syndrome accompanied by an anterior mediastinal mass, which responded very well to therapy. PMID:27516889

  6. Proposal for a recovery prediction method for patients affected by acute mediastinitis

    PubMed Central

    2012-01-01

    Background An attempt to find a prediction method of death risk in patients affected by acute mediastinitis. There is not such a tool described in available literature for that serious disease. Methods The study comprised 44 consecutive cases of acute mediastinitis. General anamnesis and biochemical data were included. Factor analysis was used to extract the risk characteristic for the patients. The most valuable results were obtained for 8 parameters which were selected for further statistical analysis (all collected during few hours after admission). Three factors reached Eigenvalue >1. Clinical explanations of these combined statistical factors are: Factor1 - proteinic status (serum total protein, albumin, and hemoglobin level), Factor2 - inflammatory status (white blood cells, CRP, procalcitonin), and Factor3 - general risk (age, number of coexisting diseases). Threshold values of prediction factors were estimated by means of statistical analysis (factor analysis, Statgraphics Centurion XVI). Results The final prediction result for the patients is constructed as simultaneous evaluation of all factor scores. High probability of death should be predicted if factor 1 value decreases with simultaneous increase of factors 2 and 3. The diagnostic power of the proposed method was revealed to be high [sensitivity =90%, specificity =64%], for Factor1 [SNC = 87%, SPC = 79%]; for Factor2 [SNC = 87%, SPC = 50%] and for Factor3 [SNC = 73%, SPC = 71%]. Conclusion The proposed prediction method seems a useful emergency signal during acute mediastinitis control in affected patients. PMID:22574625

  7. Complete atrioventricular block following mediastinal irradiation: A report of six cases

    SciTech Connect

    Slama, M.S.; Le Guludec, D.; Sebag, C.; Leenhardt, A.R.; Davy, J.M.; Pellerin, D.E.; Drieu, L.H.; Victor, J.; Brechenmacher, C.; Motte, G. )

    1991-07-01

    Complete atrioventricular block (AVB) following radiotherapy has been reported rarely, usually after high dose mediastinal irradiation for Hodgkin's disease or lung or breast carcinoma. The authors report six new cases of episodic complete infranodal AVB, requiring permanent pacemaker implantation. The mean age was 48-years old (ranging from 25-60) at the first Adams Stokes attack, mean delay was 12 years after irradiation (10-18), and mean radiation dose was 5,200 rads (4,000-6,500). All patients had abnormal interval electrocardiograms (right bundle branch block in two, left bundle branch block in three, alternating left and right bundle branch block in one). Electrocardiograms during the episode of AVB or Holter recordings were consistent with infranodal block in all patients; electrophysiological study performed in five patients confirmed infranodal AVB in four, and one was normal. Pericardial disease was constant, which included pericardial constriction in four patients. Two patients died after failure of pericardiectomy to improve congestive heart failure, due to epicardial, myocardial, and endocardial involvement. Noncardiac mediastinal lesions were present in four cases. Since this delayed complication may occur in patients of such age that the relation between the AVB and the chest irradiation is questionable, they propose the following etiologic criteria; high radiation dose (over 4,000 rads); delay of 10 years or more; abnormal interval tracings; pericardial involvement; and associated cardiac or mediastinal radiation-induced lesions.

  8. A Case of Nocardia farcinica Pneumonia and Mediastinitis in an Immunocompetent Patient

    PubMed Central

    Kim, Jinyoung; Kang, Minkyu; Kim, Juri; Jung, Sohee; Park, Junhung; Lee, Dongkyu

    2016-01-01

    Nocardia species are aerobic, gram-positive pathogens found worldwide in soil. Nocardia is considered an opportunistic pathogen, and its infection mostly occurs in immunocompromised patients. We report a case of Nocardia farcinica induced mediastinitis and pneumonia that occurred in a 64-year-old male patient who had no significant medical history except for hypertension. He visited another hospital with a complaint of dyspnea and left chest wall pain. The symptoms arose 7 days ago without any trauma and they worsened. A mediastinal mass was found on computed tomography scan. After being transferred to our hospital for further evaluation, he was diagnosed with mediastinitis and pneumonia. As N. farcinica was found to be the causative organism by 16S rRNA sequencing, proper antibiotic therapy including trimethoprim/sulfamethoxazole was initiated immediately. After this, the patient improved and he was discharged. If an infection has a disseminating course, nocardiosis cannot be excluded even in immunocompetent patients. Once the diagnosis is established, prompt antibiotic therapy should be performed based on the severity. PMID:27066088

  9. Diagnostic yield of endobronchial ultrasound-guided transbronchial needle aspiration for mediastinal staging in lung cancer*

    PubMed Central

    Fernández-Bussy, Sebastián; Labarca, Gonzalo; Canals, Sofia; Caviedes, Iván; Folch, Erik; Majid, Adnan

    2015-01-01

    OBJECTIVE: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive diagnostic test with a high diagnostic yield for suspicious central pulmonary lesions and for mediastinal lymph node staging. The main objective of this study was to describe the diagnostic yield of EBUS-TBNA for mediastinal lymph node staging in patients with suspected lung cancer. METHODS: Prospective study of patients undergoing EBUS-TBNA for diagnosis. Patients ≥ 18 years of age were recruited between July of 2010 and August of 2013. We recorded demographic variables, radiological characteristics provided by axial CT of the chest, location of the lesion in the mediastinum as per the International Association for the Study of Lung Cancer classification, and definitive diagnostic result (EBUS with a diagnostic biopsy or a definitive diagnostic method). RESULTS: Our analysis included 354 biopsies, from 145 patients. Of those 145 patients, 54.48% were male. The mean age was 63.75 years. The mean lymph node size was 15.03 mm, and 90 lymph nodes were smaller than 10.0 mm. The EBUS-TBNA method showed a sensitivity of 91.17%, a specificity of 100.0%, and a negative predictive value of 92.9%. The most common histological diagnosis was adenocarcinoma. CONCLUSIONS: EBUS-TBNA is a diagnostic tool that yields satisfactory results in the staging of neoplastic mediastinal lesions. PMID:26176519

  10. Cervico-mediastinal goiter: is telescopic exploration of the mediastinum (video mediastinoscopy) useful?

    PubMed

    Migliore, Marcello; Costanzo, Mario; Cannizzaro, Matteo A

    2010-03-01

    Surgeons are aware that most mediastinal goiters can be excised through a Kocher transverse collar incision, but in rare circumstances a partial-complete median sternotomy or a thoracotomy are mandatory. During an operation to remove a large cervico-mediastinal goiter (CMG) a profound, not massive, bleeding in the anterior mediastinum developed. Bleeding was unsuccessfully treated with packing. Instead, to perform an urgent sternotomy we used telescopic imaging to identify the source of hemorrhage, and a metallic clip was used to stop the bleeding. Since then we have prospectively used the telescope in the case of large CMG causing compression of an adjacent structure. This report is a preliminary communication demonstrating the technique. Telescopic exploration of the mediastinum was performed in seven patients. The goiters were located in the middle mediastinum in five patients and in the anterior and middle mediastinum in one, respectively. The use of a telescope can help the surgeon during the removal of a large mediastinal goiter. It facilitates a) the visualization of the intrathoracic tributaries reducing the risk of hemorrhage, b) the research of ectopic thyroid gland, and finally c) minimizes the risk of complications of a median sternotomy. PMID:20022881

  11. A Case of Nocardia farcinica Pneumonia and Mediastinitis in an Immunocompetent Patient.

    PubMed

    Kim, Jinyoung; Kang, Minkyu; Kim, Juri; Jung, Sohee; Park, Junhung; Lee, Dongkyu; Yoon, Heejung

    2016-04-01

    Nocardia species are aerobic, gram-positive pathogens found worldwide in soil. Nocardia is considered an opportunistic pathogen, and its infection mostly occurs in immunocompromised patients. We report a case of Nocardia farcinica induced mediastinitis and pneumonia that occurred in a 64-year-old male patient who had no significant medical history except for hypertension. He visited another hospital with a complaint of dyspnea and left chest wall pain. The symptoms arose 7 days ago without any trauma and they worsened. A mediastinal mass was found on computed tomography scan. After being transferred to our hospital for further evaluation, he was diagnosed with mediastinitis and pneumonia. As N. farcinica was found to be the causative organism by 16S rRNA sequencing, proper antibiotic therapy including trimethoprim/sulfamethoxazole was initiated immediately. After this, the patient improved and he was discharged. If an infection has a disseminating course, nocardiosis cannot be excluded even in immunocompetent patients. Once the diagnosis is established, prompt antibiotic therapy should be performed based on the severity. PMID:27066088

  12. Posterior Mediastinal Adenomatoid Tumor: A Case Report and Review of the Literature

    PubMed Central

    Parekh, Vishwas; Winokur, Thomas; Cerfolio, Robert J.; Stevens, Todd M.

    2016-01-01

    Adenomatoid tumor is an uncommon benign neoplasm of mesothelial differentiation that distinctively arises in and around the genital organs. In rare instances, it has been described in extragenital locations. There have been only two reports documenting its occurrence in the anterior mediastinum, and no reports documenting its occurrence in the posterior mediastinum. We report the first case of posterior mediastinal adenomatoid tumor. A 37-year-old Caucasian woman presented with symptoms of bronchitis. Imaging studies identified a 2.0 cm posterior mediastinal mass abutting the T9 vertebral body, clinically and radiologically most consistent with schwannoma. Histologic sections revealed a lesion composed of epithelioid cells arranged in cords and luminal profiles embedded in a fibrotic to loose stroma and surrounded by a fibrous pseudocapsule. Lesional cells showed vacuolated eosinophilic cytoplasm and peripherally displaced nuclei with prominent nucleoli. There was focal cytologic atypia but no mitotic figures or necrosis was identified. The lesional cells expressed cytokeratin, calretinin, and nuclear WT1 but were negative for PAX8, TTF1, p53, chromogranin, CD31, and CD34, and Ki67 showed <2% proliferation rate, diagnostic of adenomatoid tumor. Three years after resection, the patient is in good health without tumor recurrence. Thus, our encounter effectively expands the differential diagnosis of posterior mediastinal neoplastic entities. PMID:27293940

  13. Detection of cystic structures using pulsed ultrasonically induced resonant cavitation

    NASA Technical Reports Server (NTRS)

    Bar-Cohen, Yoseph (Inventor); Kovach, John S. (Inventor)

    2002-01-01

    Apparatus and method for early detection of cystic structures indicative of ovarian and breast cancers uses ultrasonic wave energy at a unique resonance frequency for inducing cavitation in cystic fluid characteristic of cystic structures in the ovaries associated with ovarian cancer, and in cystic structures in the breast associated with breast cancer. Induced cavitation bubbles in the cystic fluid implode, creating implosion waves which are detected by ultrasonic receiving transducers attached to the abdomen of the patient. Triangulation of the ultrasonic receiving transducers enables the received signals to be processed and analyzed to identify the location and structure of the cyst.

  14. Ultrasound techniques in the evaluation of the mediastinum, part I: endoscopic ultrasound (EUS), endobronchial ultrasound (EBUS) and transcutaneous mediastinal ultrasound (TMUS), introduction into ultrasound techniques

    PubMed Central

    Annema, Jouke Tabe; Clementsen, Paul; Cui, Xin Wu; Borst, Mathias Maximilian; Jenssen, Christian

    2015-01-01

    Ultrasound imaging has gained importance in pulmonary medicine over the last decades including conventional transcutaneous ultrasound (TUS), endoscopic ultrasound (EUS), and endobronchial ultrasound (EBUS). Mediastinal lymph node staging affects the management of patients with both operable and inoperable lung cancer (e.g., surgery vs. combined chemoradiation therapy). Tissue sampling is often indicated for accurate nodal staging. Recent international lung cancer staging guidelines clearly state that endosonography (EUS and EBUS) should be the initial tissue sampling test over surgical staging. Mediastinal nodes can be sampled from the airways [EBUS combined with transbronchial needle aspiration (EBUS-TBNA)] or the esophagus [EUS fine needle aspiration (EUS-FNA)]. EBUS and EUS have a complementary diagnostic yield and in combination virtually all mediastinal lymph nodes can be biopsied. Additionally endosonography has an excellent yield in assessing granulomas in patients suspected of sarcoidosis. The aim of this review, in two integrative parts, is to discuss the current role and future perspectives of all ultrasound techniques available for the evaluation of mediastinal lymphadenopathy and mediastinal staging of lung cancer. A specific emphasis will be on learning mediastinal endosonography. Part I is dealing with an introduction into ultrasound techniques, mediastinal lymph node anatomy and diagnostic reach of ultrasound techniques and part II with the clinical work up of neoplastic and inflammatory mediastinal lymphadenopathy using ultrasound techniques and how to learn mediastinal endosonography. PMID:26543620

  15. Zinc supplementation in children with cystic fibrosis

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Cystic fibrosis (CF) leads to malabsorption of macro- and micronutrients. Symptomatic zinc deficiency has been reported in CF but little is known about zinc homeostasis in children with CF. Zinc supplementation (Zn suppl) is increasingly common in children with CF but it is not without theoretcial r...

  16. Nutritional assessment in children with cystic fibrosis

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Optimal nutrition, including consuming 35–40% of calories (kcal) as fat, is a vital part of the management of cystic fibrosis (CF), and involves accurate assessment of dietary intake. We compared 3 methods of nutritional assessment in 8– to 14-year-old children (n=20) with CF: 1) a 24-h Dietary Reca...

  17. [Macrolides, Pseudomonas aeruginosa and cystic fibrosis].

    PubMed

    Guillot, M; Amiour, M; El Hachem, C; Harchaoui, S; Ribault, V; Paris, C

    2006-10-01

    Long-term low dose azithromycin treatment in cystic fibrosis patients with chronic Pseudomonas aeruginosa infection is safe and reduces the decline in lung function, the number of acute exacerbations and improves nutritional status; underlying efficacy mechanisms are multiple and synergistic. PMID:17370396

  18. Diabetes mellitus in patients with cystic fibrosis.

    PubMed

    Alves, Crésio de Aragão Dantas; Aguiar, Renata Arruti; Alves, Ana Cláudia S; Santana, Maria Angélica

    2007-01-01

    Cystic fibrosis-related diabetes (CFRD) is the principal extra-pulmonary complication of cystic fibrosis, occurring in 15-30% of adult cystic fibrosis patients. The number of cystic fibrosis patients who develop diabetes is increasing in parallel with increases in life expectancy. The aim of this study was to review the physiopathology, clinical presentation, diagnosis and treatment of CFRD. A bibliographic search of the Medline and Latin American and Caribbean Health Sciences Literature databases was made. Articles were selected from among those published in the last twenty years. Insulin deficiency, caused by reduced beta-cell mass, is the main etiologic mechanism, although insulin resistance also plays a role. Presenting features of type 1 and type 2 diabetes, CFRD typically affects individuals of approximately 20 years of age. It can also be accompanied by fasting, non-fasting or intermittent hyperglycemia. Glucose intolerance is associated with worsening of nutritional status, increased morbidity, decreased survival and reduced pulmonary function. Microvascular complications are always present, although macrovascular complications are rarely seen. An oral glucose tolerance test is recommended annually for patients > or = 10 years of age and for any patients presenting unexplained weight loss or symptoms of diabetes. Patients hospitalized with severe diseases should also be screened. If fasting hyperglycemia persists for more than 48 h, insulin therapy is recommended. Insulin administration remains the treatment of choice for diabetes and fasting hyperglycemia. Calories should not be restricted, and patients with CFRD should be managed by a multidisciplinary team. PMID:17724542

  19. Cystic lesion around the hip joint.

    PubMed

    Yukata, Kiminori; Nakai, Sho; Goto, Tomohiro; Ikeda, Yuichi; Shimaoka, Yasunori; Yamanaka, Issei; Sairyo, Koichi; Hamawaki, Jun-Ichi

    2015-10-18

    This article presents a narrative review of cystic lesions around the hip and primarily consists of 5 sections: Radiological examination, prevalence, pathogenesis, symptoms, and treatment. Cystic lesions around the hip are usually asymptomatic but may be observed incidentally on imaging examinations, such as computed tomography and magnetic resonance imaging. Some cysts may enlarge because of various pathological factors, such as trauma, osteoarthritis, rheumatoid arthritis, or total hip arthroplasty (THA), and may become symptomatic because of compression of surrounding structures, including the femoral, obturator, or sciatic nerves, external iliac or common femoral artery, femoral or external iliac vein, sigmoid colon, cecum, small bowel, ureters, and bladder. Treatment for symptomatic cystic lesions around the hip joint includes rest, nonsteroidal anti-inflammatory drug administration, needle aspiration, and surgical excision. Furthermore, when these cysts are associated with osteoarthritis, rheumatoid arthritis, and THA, primary or revision THA surgery will be necessary concurrent with cyst excision. Knowledge of the characteristic clinical appearance of cystic masses around the hip will be useful for determining specific diagnoses and treatments. PMID:26495246

  20. Cystic lesion around the hip joint

    PubMed Central

    Yukata, Kiminori; Nakai, Sho; Goto, Tomohiro; Ikeda, Yuichi; Shimaoka, Yasunori; Yamanaka, Issei; Sairyo, Koichi; Hamawaki, Jun-ichi

    2015-01-01

    This article presents a narrative review of cystic lesions around the hip and primarily consists of 5 sections: Radiological examination, prevalence, pathogenesis, symptoms, and treatment. Cystic lesions around the hip are usually asymptomatic but may be observed incidentally on imaging examinations, such as computed tomography and magnetic resonance imaging. Some cysts may enlarge because of various pathological factors, such as trauma, osteoarthritis, rheumatoid arthritis, or total hip arthroplasty (THA), and may become symptomatic because of compression of surrounding structures, including the femoral, obturator, or sciatic nerves, external iliac or common femoral artery, femoral or external iliac vein, sigmoid colon, cecum, small bowel, ureters, and bladder. Treatment for symptomatic cystic lesions around the hip joint includes rest, nonsteroidal anti-inflammatory drug administration, needle aspiration, and surgical excision. Furthermore, when these cysts are associated with osteoarthritis, rheumatoid arthritis, and THA, primary or revision THA surgery will be necessary concurrent with cyst excision. Knowledge of the characteristic clinical appearance of cystic masses around the hip will be useful for determining specific diagnoses and treatments. PMID:26495246

  1. Cystic Renal Disease in the Domestic Ferret

    PubMed Central

    Jackson, Courtnye N; Rogers, Arlin B; Maurer, Kirk J; Lofgren, Jennifer LS; Fox, James G; Marini, Robert P

    2008-01-01

    Cystic renal diseases in domestic ferrets are a common anecdotal finding but have received scant systematic assessment. We performed a 17-y, case-control retrospective analysis of the medical records of 97 ferrets housed at our institution between 1987 and 2004, to determine the prevalence and morphotypes of cystic renal diseases in this species. Histologic sections stained with hematoxylin and eosin, Masson trichrome, or periodic acid–Schiff were evaluated by a comparative pathologist, and statistical analysis of hematologic and serum chemistry values was correlated with morphologic diagnosis. Of the 97 available records, 43 were eliminated due to lack of accompanying tissues. Of the 54 remaining cases, 37 (69% prevalence) had documented renal cysts, and 14 of the 54 ferrets (26%) had primary polycystic disease consisting of either polycystic kidney disease affecting renal tubules or, more commonly, glomerulocystic kidney disease. Secondary polycystic lesions were identified in 11 ferrets (20%), and 12 ferrets (22%) exhibited focal or isolated tubular cysts only as an incidental necropsy finding. Ferrets with secondary renal cysts associated with other developmental anomalies, mesangial glomerulopathy, or end-stage kidney disease had hyperphosphatemia and elevated BUN in comparison with those with primary cystic disease and elevated BUN compared with those without renal lesions. Although reflecting institutional bias, these results implicate primary and secondary cystic renal diseases as highly prevalent and underreported in the domestic ferret. In addition to the clinical implications for ferrets as research subjects and pets, these findings suggest a potential value for ferrets as a model of human cystic renal diseases. PMID:18524174

  2. Nutrient Status of Adults with Cystic Fibrosis

    PubMed Central

    GORDON, CATHERINE M.; ANDERSON, ELLEN J.; HERLYN, KAREN; HUBBARD, JANE L.; PIZZO, ANGELA; GELBARD, RONDI; LAPEY, ALLEN; MERKEL, PETER A.

    2011-01-01

    Nutrition is thought to influence disease status in patients with cystic fibrosis (CF). This cross-sectional study sought to evaluate nutrient intake and anthropometric data from 64 adult outpatients with cystic fibrosis. Nutrient intake from food and supplements was compared with the Dietary Reference Intakes for 16 nutrients and outcomes influenced by nutritional status. Attention was given to vitamin D and calcium given potential skeletal implications due to cystic fibrosis. Measurements included weight, height, body composition, pulmonary function, and serum metabolic parameters. Participants were interviewed about dietary intake, supplement use, pulmonary function, sunlight exposure, and pain. The participants’ mean body mass index (±standard deviation) was 21.8±4.9 and pulmonary function tests were normal. Seventy-eight percent used pancreatic enzyme replacement for malabsorption. Vitamin D deficiency [25-hydroxyvitamin D (25OHD)<37.5 nmol/L] was common: 25 (39%) were deficient despite adequate vitamin D intake. Lipid profiles were normal in the majority, even though total and saturated fat consumption represented 33.0% and 16.8% of energy intake, respectively. Reported protein intake represented 16.9% of total energy intake (range 10%–25%). For several nutrients, including vitamin D and calcium, intake from food and supplements in many participants exceeded recommended Tolerable Upper Intake Levels. Among adults with cystic fibrosis, vitamin D deficiency was common despite reported adequate intake, and lipid profiles were normal despite a relatively high fat intake. Mean protein consumption was adequate, but the range of intake was concerning, as both inadequate or excessive intake may have deleterious skeletal effects. These findings call into question the applicability of established nutrient thresholds for patients with cystic fibrosis. PMID:18060897

  3. Systemic inflammatory mediators and cystic fibrosis genotype.

    PubMed

    Augarten, A; Paret, G; Avneri, I; Akons, H; Aviram, M; Bentur, L; Blau, H; Efrati, O; Szeinberg, A; Barak, A; Kerem, E; Yahav, J

    2004-10-01

    Morbidity and mortality in cystic fibrosis patients is mainly attributed to pulmonary infection and inflammation. Chemokines play a pivotal role in the inflammatory process. Although genotype-phenotype correlation in cystic fibrosis patients has been defined, a clear relationship between the defect in the cystic fibrosis transmembrane regulator (CFTR) gene and pulmonary inflammation has not been established. The aim of this study was to assess whether serum chemokines levels in cystic fibrosis patients correlate with genotype and pulmonary function tests, as well as with other clinical characteristics. Serum levels of interleukin-8, RANTES, and monocyte chemoattractant protein-1 were measured in 36 cystic fibrosis patients grouped according to their genotype. Group A included 25 patients who carried two mutations associated with a pathological sweat test and pancreatic insufficiency (deltaF508, W1282X, G542X, N1303K, S549R). Group B included 11 compound heterozygote patients who carried one mutation known to cause mild disease with borderline or normal sweat test and pancreatic sufficiency (3849+10kb C to T, 5T). Associations between chemokine levels, genotype, pulmonary function, Pseudomonas aeruginosa colonization, age, sweat chloride level, and pancreatic and nutritional status were examined. Mean interleukin-8 and monocyte chemoattractant protein-1 levels were significantly higher in group A than group B (11.4 +/- 2.1 pg/ml vs. 5 +/- 0.9 pg/ml and 157 +/- 16 pg/ml vs. 88.8 +/- 16.4 pg/ml, respectively) (P < 0.01). No difference in RANTES levels were found between groups. interleukin-8 levels were inversely related to forced expiratory volume in 1 s (r = -0.37, P < 0.02), while there was no association between the latter and RANTES and monocyte chemoattractant protein-1 levels. The Pseudomonas colonization rate was higher among group A patients than group B (88% vs. 40%, P < 0.01). No relationship was found between measured chemokines and age, sweat chloride

  4. Systematic Endobronchial Ultrasound-guided Mediastinal Staging Versus Positron Emission Tomography for Comprehensive Mediastinal Staging in NSCLC Before Radical Radiotherapy of Non-small Cell Lung Cancer: A Pilot Study.

    PubMed

    Steinfort, Daniel P; Siva, Shankar; Leong, Tracy L; Rose, Morgan; Herath, Dishan; Antippa, Phillip; Ball, David L; Irving, Louis B

    2016-02-01

    Despite known limitations of positron emission tomography (PET) for mediastinal staging of non-small cell lung cancer (NSCLC), radiation treatment fields are generally based on PET-identified disease extent. However, no studies have examined the accuracy of FDG-PET/CT on a per-node basis in patients being considered for curative-intent radiotherapy in NSCLC.In a prospective trial, patients with NSCLC being considered for definitive thoracic radiotherapy (± systemic chemotherapy) underwent minimally invasive systematic mediastinal evaluation with endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) following noninvasive staging with integrated PET-CT.Thirty patients underwent EBUS-TBNA, with TBNA performed from a mean 2.5 lymph node (LN) stations per patient (median 3, range 1-5). Discordant findings between PET-CT and EBUS-TBNA were observed in 10 patients (33%, 95% CI 19%-51%). PET-occult LN metastases were demonstrated by EBUS in 4 patients, whereas a lesser extent of mediastinal involvement, compared with FDG-PET, was demonstrated by EBUS in 6 patients, including 2 patients downstaged from cN3 to pN2. LNs upstaged by EBUS were significantly smaller than nodes downstaged by EBUS, 7.5 mm (range 7-9) versus 12 mm (range 6-21), P = 0.005.A significant proportion of patients considered for definitive radiotherapy (+/-chemotherapy) undergoing systematic mediastinal evaluation with EBUS-TBNA in this study have an extent of mediastinal NSCLC involvement discordant with that indicated by PET-CT. Systematic EBUS-TBNA may aid in defining the extent of mediastinal involvement in NSCLC patients undergoing radiotherapy. Systematic EBUS-TBNA has the potential to contribute significantly to radiotherapy planning and delivery, by either identifying occult nodal metastases, or demonstrating FDG-avid LNs to be disease-free. PMID:26937894

  5. [A case of mucinous cystic neoplasm of the liver].

    PubMed

    Tatsumi, Ryoji; Amizuka, Hisato; Matsubara, Yu; Yoshizaki, Koji; Sakamoto, Jun; Sato, Ryu; Kimura, Keisuke; Nishimori, Hiroyuki; Ohta, Tomoyuki

    2015-07-01

    We present a case of resected mucinous cystic neoplasm of the liver in a 71-year-old woman admitted to our hospital with epigastric discomfort. Abdominal ultrasonography and computed tomography revealed a multi-locular cystic tumor measuring 35 mm in diameter in segment IV of the liver. Left hepatic lobectomy was performed based on the diagnosis of mucinous cystic neoplasm of the liver; subsequent histology revealed that the tumor was multi-locular, cystic, and lined with a single layer of columnar epithelium with low-grade atypia and was associated with a typical ovarian-like stroma. There was no evidence (imaging or histological) to support communication of the cyst with the intrahepatic bile duct, despite modest bile deposition being observed in the cystic wall. The definitive diagnosis was mucinous cystic neoplasm with low-grade intrahepatic epithelial neoplasia. PMID:26155869

  6. Pregnancy and cystic fibrosis: Approach to contemporary management.

    PubMed

    Geake, James; Tay, George; Callaway, Leonie; Bell, Scott C

    2014-12-01

    Over the previous 50 years survival of patients with cystic fibrosis has progressively increased. As a result of improvements in health care, increasing numbers of patients with cystic fibrosis are now considering starting families of their own. For the health care professionals who look after these patients, the assessment of the potential risks, and the process of guiding prospective parents through pregnancy and beyond can be both challenging and rewarding. To facilitate appropriate discussions about pregnancy, health care workers must have a detailed understanding of the various important issues that will ultimately need to be considered for any patient with cystic fibrosis considering parenthood. This review will address these issues. In particular, it will outline pregnancy outcomes for mothers with cystic fibrosis, issues that need to be taken into account when planning a pregnancy and the management of pregnancy for mothers with cystic fibrosis or mothers who have undergone organ transplantation as a result of cystic fibrosis. PMID:27512443

  7. Pregnancy and cystic fibrosis: Approach to contemporary management

    PubMed Central

    Tay, George; Callaway, Leonie; Bell, Scott C

    2014-01-01

    Over the previous 50 years survival of patients with cystic fibrosis has progressively increased. As a result of improvements in health care, increasing numbers of patients with cystic fibrosis are now considering starting families of their own. For the health care professionals who look after these patients, the assessment of the potential risks, and the process of guiding prospective parents through pregnancy and beyond can be both challenging and rewarding. To facilitate appropriate discussions about pregnancy, health care workers must have a detailed understanding of the various important issues that will ultimately need to be considered for any patient with cystic fibrosis considering parenthood. This review will address these issues. In particular, it will outline pregnancy outcomes for mothers with cystic fibrosis, issues that need to be taken into account when planning a pregnancy and the management of pregnancy for mothers with cystic fibrosis or mothers who have undergone organ transplantation as a result of cystic fibrosis. PMID:27512443

  8. Mediastinal micro-vessels clipping during lymph node dissection may contribute to reduce postoperative pleural drainage

    PubMed Central

    Yan, Shi; Wang, Xing; Lv, Chao; Phan, Kevin; Wang, Yuzhao; Wang, Jia; Yang, Yue

    2016-01-01

    Background Postoperative pleural drainage markedly influences the length of postoperative stay and financial costs of medical care. The aim of this study is to retrospectively investigate potentially predisposing factors related to pleural drainage after curative thoracic surgery and to explore the impact of mediastinal micro-vessels clipping on pleural drainage control after lymph node dissection. Methods From February 2012 to November 2013, 322 consecutive cases of operable non-small cell lung cancers (NSCLC) undergoing lobectomy and mediastinal lymph node dissection with or without application of clipping were collected. Total and daily postoperative pleural drainage were recorded. Propensity score matching (1:2) was applied to balance variables potentially impacting pleural drainage between group clip and group control. Analyses were performed to compare drainage volume, duration of chest tube and postoperative hospital stay between the two groups. Variables linked with pleural drainage in whole cohort were assessed using multivariable logistic regression analysis. Results Propensity score matching resulted in 197 patients (matched cohort). Baseline patient characteristics were matched between two groups. Group clip showed less cumulative drainage volume (P=0.020), shorter duration of chest tube (P=0.031) and postoperative hospital stay (P=0.022) compared with group control. Risk factors significantly associated with high-output drainage in multivariable logistic regression analysis were being male, age >60 years, bilobectomy/sleeve lobectomy, pleural adhesion, the application of clip applier, duration of operation ≥220 minutes and chylothorax (P<0.05). Conclusions This study suggests that mediastinal micro-vessels clipping during lymph node dissection may reduce postoperative pleural drainage and thus shorten hospital stay. PMID:27076936

  9. Multi-Modality Mediastinal Staging for Lung Cancer Among Medicare Beneficiaries

    PubMed Central

    Farjah, Farhood; Flum, David R.; Ramsey, Scott D.; Heagerty, Patrick J.; Symons, Rebecca Gaston; Wood, Douglas E.

    2009-01-01

    Introduction The use of non-invasive and invasive diagnostic tests improves the accuracy of mediastinal staging for lung cancer. It is unknown how frequently multi-modality mediastinal staging is used, or whether its use is associated with better health outcomes. Methods A cohort study was conducted using SEER-Medicare data (1998–2005). Patients were categorized as having undergone single (CT only), bi- (CT and PET or CT and invasive staging), or tri-modality (CT, PET, and invasive staging) staging. Results Among 43,912 subjects, 77%, 21%, and 2% received single, bi-, and tri-modality staging, respectively. The use of single modality staging decreased over time from 90% in 1998 to 67% in 2002 (p-trend <0.001), whereas the use of bi- and tri-modality staging increased from 10% to 30% and 0.4% to 5%, respectively. After adjustment for differences in patient characteristics, the use of a greater number of staging modalities was associated with a lower risk of death (bi- versus single modality: HR 0.58, 99% CI 0.56–0.60; tri- versus single modality: HR 0.49, 99% CI 0.45–0.54; tri- versus bi-modality: HR 0.85, 99% CI 0.77–0.93). These associations were maintained even after excluding stage IV patients or adjustment for stage. Conclusions The use of multi-modality mediastinal staging increased over time and was associated with better survival. Stage migration and unmeasured patient and provider characteristics may have affected the magnitude of these associations. Cancer treatment guidelines should emphasize the potential relationship between staging procedures and outcomes, and health care policy should encourage adherence to staging guidelines. PMID:19156000

  10. Motion Analysis of 100 Mediastinal Lymph Nodes: Potential Pitfalls in Treatment Planning and Adaptive Strategies

    SciTech Connect

    Pantarotto, Jason R.; Piet, Anna H.M.; Vincent, Andrew; Soernsen de Koste, John R. van; Senan, Suresh

    2009-07-15

    Purpose: The motion of mediastinal lymph nodes may undermine local control with involved-field radiotherapy. We studied patterns of nodal and tumor motion in 41 patients with lung cancer. Methods and Materials: Four-dimensional (4D) computed tomography planning scans were retrospectively evaluated to identify patients with clearly visible mediastinal lymph nodes. One hundred nodes from 14 patients with Stage I and 27 patients with Stage III were manually contoured in all 4D computed tomography respiratory phases. Motion was derived from changes in the nodal center-of-mass position. Primary tumors were also delineated in all phases for 16 patients with Stage III disease. Statistical analysis included a multivariate mixed-effects model of grouped data. Results: Average 3D nodal motion during quiet breathing was 0.68 cm (range, 0.17-1.64 cm); 77% moved greater than 0.5 cm, and 10% moved greater than 1.0 cm. Motion was greatest in the lower mediastinum (p = 0.002), and nodes measuring 2 cm or greater in diameter showed motion similar to that in smaller nodes. In 11 of 16 patients studied, at least one node moved more than the corresponding primary tumor. No association between 3D primary tumor motion and nodal motion was observed. For mobile primary tumors, phase offsets between the primary tumor and nodes of two or more and three or more phases were observed for 33% and 12% of nodes, respectively. Conclusions: Mediastinal nodal motion is common, with phase offsets seen between the primary tumor and different nodes in the same patient. Patient-specific information is needed to ensure geometric coverage, and adaptive strategies based solely on the primary tumor may be misleading.

  11. Cardiac Magnetic Resonance Imaging Findings in 20-year Survivors of Mediastinal Radiotherapy for Hodgkin's Disease

    SciTech Connect

    Machann, Wolfram; Beer, Meinrad; Breunig, Margret; Stoerk, Stefan; Angermann, Christiane; Seufert, Ines; Schwab, Franz; Koelbl, Oliver; Flentje, Michael; Vordermark, Dirk

    2011-03-15

    Purpose: The recognition of the true prevalence of cardiac toxicity after mediastinal radiotherapy requires very long follow-up and a precise diagnostic procedure. Cardiac magnetic resonance imaging (MRI) permits excellent quantification of cardiac function and identification of localized myocardial defects and has now been applied to a group of 20-year Hodgkin's disease survivors. Methods and materials: Of 143 patients treated with anterior mediastinal radiotherapy (cobalt-60, median prescribed dose 40 Gy) for Hodgkin's disease between 1978 and 1985, all 53 survivors were invited for cardiac MRI. Of those, 36 patients (68%) presented for MRI, and in 31 patients (58%) MRI could be performed 20-28 years (median, 24) after radiotherapy. The following sequences were acquired on a 1.5-T MRI: transversal T1-weighted TSE and T2-weighted half-fourier acquisition single-shot turbo-spin-echo sequences, a steady-state free precession (SSFP) cine sequence in the short heart axis and in the four-chamber view, SSFP perfusion sequences under rest and adenosine stress, and a SSFP inversion recovery sequence for late enhancement. The MRI findings were correlated with previously reconstructed doses to cardiac structures. Results: Clinical characteristics and reconstructed doses were not significantly different between survivors undergoing and not undergoing MRI. Pathologic findings were reduced left ventricular function (ejection fraction <55%) in 7 (23%) patients, hemodynamically relevant valvular dysfunction in 13 (42%), late myocardial enhancement in 9 (29%), and any perfusion deficit in 21 (68%). An association of regional pathologic changes and reconstructed dose to cardiac structures could not be established. Conclusions: In 20-year survivors of Hodgkin's disease, cardiac MRI detects pathologic findings in approximately 70% of patients. Cardiac MRI has a potential role in cardiac imaging of Hodgkin's disease patients after mediastinal radiotherapy.

  12. Cystic Adventitial Disease in Former Athlete.

    PubMed

    Fatic, Nikola; Nikolic, Aleksandar; Maras, Dejan; Bulatovic, Nikola

    2015-09-15

    In this paper we present a 39-year old former athlete complaining with pain in his legs during long walk resembling to intermittent claudication. Color duplex scan described a popliteal artery with 10 mm in diameter with mural thrombus that caused stenosis 75% of lumen. Digital subtraction angiography demonstrated a stenosis of right popliteal artery. The suspicion for Cystic adventitial disease was set. The patient was operated on by posterior direct approach. After incision, a yellowish viscous material was observed in adventitia. Partial resection of the affected popliteal artery and replacement by an autogenous great saphenous vein graft was performed. Patient was dismissed on the seventh postoperative day, in good condition and without any complication. Cystic adventitial disease of the popliteal artery should be considered in the differential diagnosis of intermittent claudication, especially in former sportsmen patients. Partial resection of the affected popliteal artery and replacement by an autogenous great saphenous vain graft produces excellent results. PMID:27275264

  13. MicroRNA Dysregulation in Cystic Fibrosis

    PubMed Central

    McKiernan, Paul J.; Greene, Catherine M.

    2015-01-01

    The cystic fibrosis lung is a complex milieu comprising multiple factors that coordinate its physiology. MicroRNAs are regulatory factors involved in most biological processes and it is becoming increasingly clear that they play a key role in the development and manifestations of CF lung disease. These small noncoding RNAs act posttranscriptionally to inhibit protein production. Their involvement in the pathogenesis of CF lung disease stems from the fact that their expression is altered in vivo in the CF lung due to intrinsic and extrinsic factors; to date defective chloride ion conductance, endoplasmic reticulum stress, inflammation, and infection have been implicated in altering endogenous miRNA expression in this setting. Here, the current state-of-the-art and biological consequences of altered microRNA expression in cystic fibrosis are reviewed. PMID:26185362

  14. Diffuse Cystic Lung Diseases: Diagnostic Considerations.

    PubMed

    Xu, Kai-Feng; Feng, Ruie; Cui, Han; Tian, Xinlun; Wang, Hanping; Zhao, Jing; Huang, Hui; Zhang, Weihong; Lo, Bee Hong

    2016-06-01

    Diffuse cystic lung disease (DCLD) is a group of heterogeneous diseases that present as diffuse cystic changes in the lung on computed tomography of the chest. Most DCLD diseases are rare, although they might resemble common diseases such as emphysema and bronchiectasis. Main causes of DCLD include lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, pulmonary Langerhans cell histiocytosis, lymphoid interstitial pneumonia, amyloidosis, light-chain deposition disease, Sjögren syndrome, and primary or metastatic neoplasm. We discuss clinical factors that are helpful in the differential diagnosis of DCLDsuch as sex and age, symptoms and signs, extrapulmonary presentations, cigarette smoking, and family history. Investigations for DCLD include high-resolution computed tomography, biochemical and histopathological studies, genetic tests, pulmonary function tests, and bronchoscopic and video-assisted thoracoscopic biopsies. A proposed diagnostic algorithm would enhance ease of diagnosing most cases of DCLD. PMID:27231867

  15. Inflammation and its genesis in cystic fibrosis.

    PubMed

    Nichols, David P; Chmiel, James F

    2015-10-01

    The host inflammatory response in cystic fibrosis (CF) lung disease has long been recognized as a central pathological feature and an important therapeutic target. Indeed, many believe that bronchiectasis results largely from the oxidative and proteolytic damage comprised within an exuberant airway inflammatory response that is dominated by neutrophils. In this review, we address the longstanding argument of whether or not the inflammatory response is directly attributable to impairment of the cystic fibrosis transmembrane conductance regulator or only secondary to airway obstruction and chronic bacterial infection and challenge the importance of this distinction in the context of therapy. We also review the centrality of neutrophils in CF lung pathophysiology and highlight more recent data that suggest the importance of other cell types and signaling beyond NF-κB activation. We discuss how protease and redox imbalance are critical factors in CF airway inflammation and end by reviewing some of the more promising therapeutic approaches now under development. PMID:26335954

  16. Acquired Cystic Fibrosis Transmembrane Conductance Regulator Deficiency.

    PubMed

    Cho, Do-Yeon; Woodworth, Bradford A

    2016-01-01

    In the genetic airway disease cystic fibrosis (CF), deficiency or dysfunction of the cystic fibrosis membrane conductance regulator (CFTR) alters anion transport in respiratory epithelium and consequently disrupts mucociliary clearance. An enriched understanding of the role of CFTR in the maintenance of normal epithelial function has revealed that mild and variable CFTR mutations play a causative role in a number of diseases not classically associated with CF. Furthermore, recent evidence indicates that acquired defects in wild-type CFTR protein processing, endocytic recycling and function can contribute to the pathogenesis of airway diseases, such as chronic obstructive pulmonary disease. In this chapter, we discuss emerging findings implicating acquired CFTR dysfunction in the pathogenesis of chronic rhinosinusitis and propose a new and leading edge approach to future CRS therapy using CFTR potentiators. PMID:27466849

  17. Diffuse Cystic Lung Disease. Part I.

    PubMed

    Gupta, Nishant; Vassallo, Robert; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X

    2015-06-15

    The diffuse cystic lung diseases (DCLDs) are a group of pathophysiologically heterogenous processes that are characterized by the presence of multiple spherical or irregularly shaped, thin-walled, air-filled spaces within the pulmonary parenchyma. Although the mechanisms of cyst formation remain incompletely defined for all DCLDs, in most cases lung remodeling associated with inflammatory or infiltrative processes results in displacement, destruction, or replacement of alveolar septa, distal airways, and small vessels within the secondary lobules of the lung. The DCLDs can be broadly classified according to underlying etiology as those caused by low-grade or high-grade metastasizing neoplasms, polyclonal or monoclonal lymphoproliferative disorders, infections, interstitial lung diseases, smoking, and congenital or developmental defects. In the first of a two-part series, we present an overview of the cystic lung diseases caused by neoplasms, infections, smoking-related diseases, and interstitial lung diseases, with a focus on lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis. PMID:25906089

  18. Diffuse Cystic Lung Disease. Part II.

    PubMed

    Gupta, Nishant; Vassallo, Robert; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X

    2015-07-01

    The diffuse cystic lung diseases have a broad differential diagnosis. A wide variety of pathophysiological processes spanning the spectrum from airway obstruction to lung remodeling can lead to multifocal cyst development in the lung. Although lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis are perhaps more frequently seen in the clinic, disorders such as Birt-Hogg-Dubé syndrome, lymphocytic interstitial pneumonia, follicular bronchiolitis, and light-chain deposition disease are increasingly being recognized. Obtaining an accurate diagnosis can be challenging, and management approaches are highly disease dependent. Unique imaging features, genetic tests, serum studies, and clinical features provide invaluable clues that help clinicians distinguish among the various etiologies, but biopsy is often required for definitive diagnosis. In part II of this review, we present an overview of the diffuse cystic lung diseases caused by lymphoproliferative disorders, genetic mutations, or aberrant lung development and provide an approach to aid in their diagnosis and management. PMID:25906201

  19. Cystic Adventitial Disease in Former Athlete

    PubMed Central

    Fatic, Nikola; Nikolic, Aleksandar; Maras, Dejan; Bulatovic, Nikola

    2015-01-01

    In this paper we present a 39-year old former athlete complaining with pain in his legs during long walk resembling to intermittent claudication. Color duplex scan described a popliteal artery with 10 mm in diameter with mural thrombus that caused stenosis 75% of lumen. Digital subtraction angiography demonstrated a stenosis of right popliteal artery. The suspicion for Cystic adventitial disease was set. The patient was operated on by posterior direct approach. After incision, a yellowish viscous material was observed in adventitia. Partial resection of the affected popliteal artery and replacement by an autogenous great saphenous vein graft was performed. Patient was dismissed on the seventh postoperative day, in good condition and without any complication. Cystic adventitial disease of the popliteal artery should be considered in the differential diagnosis of intermittent claudication, especially in former sportsmen patients. Partial resection of the affected popliteal artery and replacement by an autogenous great saphenous vain graft produces excellent results.

  20. Mediastinal mass diagnosed by endobronchial ultrasound as recurrent hepatocellular carcinoma in a post-liver transplantation patient

    PubMed Central

    Alraiyes, Abdul Hamid; Tunsupon, Pichapong; Kheir, Fayez; Salerno, Daniel A

    2016-01-01

    Objective: We presented a rare case of recurrent hepatocellular carcinoma after liver transplant manifested as an isolated mediastinal mass. Methods: A 62-year-old man was referred for evaluation of atypical chest pain and abnormal finding of a computed tomography of the chest. He had history of chronic hepatitis C liver cirrhosis and hepatocellular carcinoma underwent orthotopic liver transplant as a curative treatment three years earlier. Results: The computed tomography of the chest demonstrated paratracheal mediastinal lymphadenopathy. He subsequently underwent endobronchial ultrasound with transbronchial needle aspiration (EBUS-TBNA). The right paratracheal lymph node station 4R was sampled. Rapid on-site cytology evaluation demonstrated recurrent metastatic hepatocellular carcinoma. Conclusion: Pulmonologist should be cognizant of diagnostic utility of EBUS-TBNA in this clinical setting as more transplant patients on immunosuppressive medications with enlarged mediastinal lymphadenopathy of unknown origin will be referred for further evaluation. PMID:27489713

  1. Mediastinal Bronchogenic Cyst With Acute Cardiac Dysfunction: Two-Stage Surgical Approach.

    PubMed

    Smail, Hassiba; Baste, Jean Marc; Melki, Jean; Peillon, Christophe

    2015-10-01

    We describe a two-stage surgical approach in a patient with cardiac dysfunction and hemodynamic compromise resulting from a massive and compressive mediastinal bronchogenic cyst. To drain this cyst, video-assisted mediastinoscopy was performed as an emergency procedure, which immediately improved the patient's cardiac function. Five days later and under video thoracoscopy, resection of the cyst margins was impossible because the cyst was tightly adherent to the left atrium. We performed deroofing of this cyst through a right thoracotomy. The patient had an uncomplicated postoperative recovery, and no recurrence was observed at the long-term follow-up visit. PMID:26434484

  2. Severe conduction disturbances and ventricular arrhythmias complicating mediastinal irradiation for Hodgkin's disease: a case report

    SciTech Connect

    Mary-Rabine, L.; Waleffe, A.; Kulbertus, H.E.

    1980-09-01

    A 27-year-old male had undergone mediastinal and retroperitoneal irradiation for stage IIIA Hodgkin's disease at age 19. When he was admitted to the hospital, because of lightheadedness and syncope, trifascicular bundle branch block was noted and the patient underwent a clinical electrophysiological investigation. AH and HV intervals were prolonged and ventricular fibrillation was induced during programmed right ventricular stimulation. Serial electrophysiological studies allowed us to prescribe effective antiarrhythmic drug therapy with no recurrence of symptoms during a follow-up period of 12 months.

  3. Left atrial mass 16 years after radiation therapy for mediastinal neuroblastoma

    SciTech Connect

    Ensing, G.J.; Driscoll, D.J.; Smithson, W.A.

    1987-01-01

    Tumors involving the heart during childhood are rare. However, neuroblastoma, a common pediatric malignancy, has been described to involve the cardiovascular system in 3%-12% of patients dying with this tumor. Rarely is such involvement diagnosed ante mortem and never, to our knowledge, has a benign cardiac tumor been reported to present in childhood after successful eradication of neuroblastoma. We describe the identification and surgical resection of a nodular, hypertrophied, calcified, pedunculated left atrial mass in a 16-year-old boy who was complaining of exercise-associated presyncope and headaches 16 years after irradiation and chemotherapy for mediastinal neuroblastoma.

  4. A Rare Cause of Crazy-Paving and Mediastinal Lymphadenopathy: Congestive Heart Failure

    PubMed Central

    Senturk, Aysegul; Karalezli, Aysegul; Soyturk, Ayse Nur; Hasanoglu, H. Canan

    2013-01-01

    Crazy-paving sign is a pattern seen on multislice computed tomography images of the lungs. It is characterized by a reticular pattern superimposed on ground-glass opacity. It was first described in the late 1980s in patients with pulmonary alveolar proteinosis, but has now been described in some other diseases of the lung. Enlarged mediastinal lymph nodes can be seen in infectious and specific inflammatory diseases and malignancies. The present report describes a case of a 44-year-old man in whom congestive heart failure presented with a crazy-paving appearance and enlarged lymph nodes of the lungs on the chest computed tomography scan. PMID:24083067

  5. Mediastinal teratoma in a free-ranging American black bear (Ursus americanus).

    PubMed

    Munk, Brandon A; Turner, J Chris; Keel, M Kevin

    2013-12-01

    A 2.75-yr-old female American black bear (Ursus americanus) was found emaciated and unable to rise. It was euthanized due to the perceived poor prognosis. An approximately 18 x 8 x 10-cm, multiloculated, well-demarcated mass that extended from the bifurcation of the trachea, laterally displacing the lung lobes and caudally displacing the heart. The mass contained fibrous connective tissue, cartilage, bone, nervous tissue, smooth muscle, and a variety of epithelial structures. A mediastinal teratoma was diagnosed based on the microscopic features of the neoplasm. PMID:24450083

  6. Progressive Dyspnea in a 40-Year-Old Man Caused by Giant Mediastinal Thymolipoma

    PubMed Central

    Rad, Kamelia

    2016-01-01

    Thymolipomas are rare tumors of the anterior mediastinum containing both thymic stroma and an abundance of fat. We present a 40-year-old man with no underlying disease who presented with cough and progressive dyspnea. Clinical evaluation revealed a giant mass of lipid density filling almost the entire left hemithorax with mediastinal shift. Total excision of the 40 × 33 × 8 cm mass weighing 4 kg was performed via a left thoracotomy and the histopathologic diagnosis of the mass was reported as thymolipoma. The patient remains alive and disease-free, twelve months after the intervention. PMID:27293949

  7. Potter sequence complicated by congenital cystic lesion of the bladder.

    PubMed

    Abe, Yoshifusa; Mizuno, Katsumi; Horie, Hiroshi; Matsumoto, Michihiro; Yamaoka, Akiko; Mizutani, Kayo; Takeuchi, Toshio; Iikura, Yoji

    2002-07-01

    We report a case complicated by oligohydramnios, pulmonary hypoplasia, bilateral renal dysplasia, and cystic lesion of the bladder. He was clinically compatible with Potter sequence. Congenital cystic bladder is the rarest form of the bladder. We can find no report of Potter sequence complicated by cystic lesion of the bladder. This lesion was similar to multilocular bladder. The diagnosis was confirmed it by autopsy, magnetic resonance imaging, and urography after his death. PMID:12152145

  8. Calcifying cystic odontogenic tumor radiographically mimicking a sinus mucocele.

    PubMed

    de Santana Santos, Thiago; Frota, Riedel; de Souza Andrade, Emanuel Sávio; de Oliveira E Silva, Emanuel Dias

    2013-03-01

    The calcifying cystic odontogenic tumor or Gorlin cyst is an uncommon lesion with a variable clinical behavior and considerable histopathologic diversity. The authors report a case of calcifying cystic odontogenic tumor that was being treated as a maxillary sinus mucocele. The possibility of mimicking numerous odontogenic and nonodontogenic lesions makes the calcifying cystic odontogenic tumor difficult for a clinical diagnosis. The present case demonstrates that a specific knowledge in oral pathology is required to differentiate odontogenic lesions. PMID:23524811

  9. Lung Cancer Detected 5 Years after Resection of Cancer of Unknown Primary in a Mediastinal Lymph Node: A Case Report and Review of Relevant Cases from the Literature.

    PubMed

    Kawasaki, Hidenori; Arakaki, Kazunari; Taira, Naohiro; Furugen, Tomonori; Ichi, Takaharu; Yohena, Tomofumi; Kawabata, Tsutomu

    2016-04-20

    We report the rare and interesting case of a primary lung cancer detected 5 years after cancer of unknown primary (CUP) of a mediastinal lymph node (LN) was resected. A 40-year-old male was diagnosed with adenocarcinoma of unknown primary in a mediastinal lymph node after resection of the mediastinal tumor. Five years after resection of the CUP in mediastinal LN, a small, abnormal nodular shadow in left upper lobe was detected by chest CT. This pulmonary tumor was diagnosed as a lung adenocarcinoma. The pathological and immunohistological findings of the resected pulmonary tumor resembled those of the LN resected 5 years before. We speculated that the pulmonary lesion represented primary lung cancer that enlarged later than the metastatic mediastinal LN. This case illustrates the importance of careful observation and long-term follow-up in patients treated for CUP of a thoracic LN. PMID:26328596

  10. [Molecular epidemiology of cystic fibrosis in Tunisia].

    PubMed

    Messaoud, T; Bel Haj Fredj, S; Bibi, A; Elion, J; Férec, C; Fattoum, S

    2005-01-01

    Cystic fibrosis is the most frequent autosomal recessive genetic disease in North European population. This pathology seems to not be rare in Tunisia. On another hand, development of molecular biology techniques has largely contributed to implement the study of the different mutations in the CFTR gene where over 1,300 mutations were reported. Herein, we describe the strategy used to detect molecular defects responsible of cystic fibrosis on 390 children (383 families) in Tunisian population. Several techniques were performed for genotype diagnosis: DNA extraction was from peripheral blood. Polymerase chain reaction (PCR) and polyacylamide gel electrophoresis, and reverse dot blot procedures were used to detect known point mutations. Denaturant gradient gel electrophoresis (DGGE) were used in a next step searching for the unknown point mutations that are later identified by automated sequencing on ABIprism 310. This strategy allowed us to detect 17 different mutations located on the different exons of the CFTR gene. The most frequent was the F508del (50.74%) followed by three other mutations (G542X, W1282X and N1303K) known to be common in the Mediterranean area. For mutations (T665S, 2766 del8, F1166C, L1043R) were exclusively found, up to now, in the Tunisian population. Our results permitted to establish cystic fibrosis mutations and their distribution in Tunisia and to implement an appropriate prevention program of these diseases through the genetic council and prenatal diagnosis. PMID:16330381

  11. Progress in therapies for cystic fibrosis.

    PubMed

    De Boeck, Kris; Amaral, Margarida D

    2016-08-01

    Standard follow-up and symptomatic treatment have allowed most patients with cystic fibrosis to live to young adulthood. However, many patients still die prematurely from respiratory insufficiency. Hence, further investigations to improve these therapies are important and might have relevance for other diseases-eg, exploring how to increase airway hydration, how to safely downscale the increased inflammatory response in the lung, and how to better combat lung infections associated with cystic fibrosis. In parallel, development of modulators that target the underlying dysfunction in the cystic fibrosis transmembrane conductance regulator (CFTR) is fast moving forward. Existing treatments are specific to certain mutations, or mutation class, in CFTR. An effective, although not yet entirely corrective, treatment is available for patients with class III mutations, and a treatment with modest effectiveness is available for patients who are homozygous for Phe508del, albeit at a very high cost. Corrective treatments that are non-specific to mutation class and thus applicable to all patients-eg, gene therapy, cell-based therapies, and activation of alternative ion channels that bypass CFTR-are being explored, but they are still in early stages of development. In view of the large number of patients with very rare mutations, a plan to advance personalised biomarkers to predict treatment effect is also being investigated and validated. PMID:27053340

  12. [Treatment of Cystic Fibrosis with CFTR Modulators].

    PubMed

    Tümmler, B

    2016-05-01

    Personalized medicine promises that medical decisions, practices and products are tailored to the individual patient. Cystic fibrosis, an inherited disorder of chloride and bicarbonate transport in exocrine glands, is the first successful example of customized drug development for mutation-specific therapy. There are two classes of CFTR modulators: potentiators that increase the activity of CFTR at the cell surface, and correctors that either promote the read-through of nonsense mutations or facilitate the translation, folding, maturation and trafficking of mutant CFTR to the cell surface. The potentiator ivacaftor and the corrector lumacaftor are approved in Germany for the treatment of people with cystic fibrosis who carry a gating mutation such as p.Gly551Asp or who are homozygous for the most common mutation p.Phe508del, respectively. This report provides an overview of the basic defect in cystic fibrosis, the population genetics of CFTR mutations in Germany and the bioassays to assess CFTR function in humans together with the major achievements of preclinical research and clinical trials to bring CFTR modulators to the clinic. Some practical information on the use of ivacaftor and lumacaftor in daily practice and an update on pitfalls, challenges and novel strategies of bench-to-bedside development of CFTR modulators are also provided. PMID:26894479

  13. Vorinostat in Treating Patients With Locally Advanced, Recurrent, or Metastatic Adenoid Cystic Carcinoma

    ClinicalTrials.gov

    2016-03-10

    Recurrent Oral Cavity Adenoid Cystic Carcinoma; Recurrent Salivary Gland Carcinoma; Salivary Gland Adenoid Cystic Carcinoma; Stage III Major Salivary Gland Carcinoma; Stage III Oral Cavity Adenoid Cystic Carcinoma; Stage IVA Major Salivary Gland Carcinoma; Stage IVA Oral Cavity Adenoid Cystic Carcinoma; Stage IVB Major Salivary Gland Carcinoma; Stage IVB Oral Cavity Adenoid Cystic Carcinoma; Stage IVC Major Salivary Gland Carcinoma; Stage IVC Oral Cavity Adenoid Cystic Carcinoma; Tongue Carcinoma

  14. Cystic lesions of the pancreas: challenging issues in clinical practice.

    PubMed

    Oh, Hyoung-Chul; Kim, Myung-Hwan; Hwang, Chang Yun; Lee, Tae Yoon; Lee, Sang Soo; Seo, Dong-Wan; Lee, Sung Koo

    2008-01-01

    Cystic lesions of the pancreas are being recognized with increasing frequency and have become a common finding in clinical practice. Cystic lesions of the pancreas display a wide spectrum of histopathology and biologic behavior. Differentiating among lesions and choosing an optimal therapy is challenging, and evidence-based guidelines for diagnosis, management, and follow-up for cystic lesions of the pancreas are needed. This review describes the epidemiology and typical features of cystic lesions of the pancreas, including a summary of commonly used descriptive terms, as well as the primary issues in the differential diagnosis and management of these lesions. PMID:18076739

  15. Chloride and potassium channels in cystic fibrosis airway epithelia

    NASA Astrophysics Data System (ADS)

    Welsh, Michael J.; Liedtke, Carole M.

    1986-07-01

    Cystic fibrosis, the most common lethal genetic disease in Caucasians, is characterized by a decreased permeability in sweat gland duct and airway epithelia. In sweat duct epithelium, a decreased Cl- permeability accounts for the abnormally increased salt content of sweat1. In airway epithelia a decreased Cl- permeability, and possibly increased sodium absorption, may account for the abnormal respiratory tract fluid2,3. The Cl- impermeability has been localized to the apical membrane of cystic fibrosis airway epithelial cells4. The finding that hormonally regulated Cl- channels make the apical membrane Cl- permeable in normal airway epithelial cells5 suggested abnormal Cl- channel function in cystic fibrosis. Here we report that excised, cell-free patches of membrane from cystic fibrosis epithelial cells contain Cl- channels that have the same conductive properties as Cl- channels from normal cells. However, Cl- channels from cystic fibrosis cells did not open when they were attached to the cell. These findings suggest defective regulation of Cl- channels in cystic fibrosis epithelia; to begin to address this issue, we performed two studies. First, we found that isoprenaline, which stimulates Cl- secretion, increases cellular levels of cyclic AMP in a similar manner in cystic fibrosis and non-cystic fibrosis epithelial cells. Second, we show that adrenergic agonists open calcium-activated potassium channels, indirectly suggesting that calcium-dependent stimulus-response coupling is intact in cystic fibrosis. These data suggest defective regulation of Cl- channels at a site distal to cAMP accumulation.

  16. Downsized Contegra graft as a right ventricle-to-pulmonary artery conduit in the setting of mediastinitis.

    PubMed

    MacIver, Robroy H; Permut, Lester C; McMullan, David M

    2013-09-01

    Polytetrafluoroethylene (PTFE) grafts are commonly used for construction of the right ventricle-to-pulmonary artery conduit in the modified Norwood procedure. Dehiscence of a PTFE conduit in the setting of purulent mediastinitis presents a challenging clinical problem because of limited availability of appropriately sized replacement vascular homografts. The Contegra bovine jugular vein graft is an alternative to placing another PTFE graft in an infected space when a homograft of appropriate size is not available. We describe the use of a downsized Contegra conduit to replace an infected PTFE right ventricle-to-pulmonary artery graft in a neonate with life-threatening purulent mediastinitis. PMID:23992706

  17. Mediastinal irradiation in a patient affected by lung carcinoma after heart transplantation: Helical tomotherapy versus three dimensional conformal radiotherapy

    PubMed Central

    Iorio, Vincenzo; Cammarota, Fabrizio; Toledo, Diego; Senese, Rossana; Francomacaro, Ferdinando; Muto, Matteo; Muto, Paolo

    2016-01-01

    Abstract Patients who have undergone solid organ transplants are known to have an increased risk of neoplasia compared with the general population. We report our experience using mediastinal irradiation with helical tomotherapy versus three‐dimensional conformal radiation therapy to treat a patient with lung carcinoma 15 years after heart transplantation. Our dosimetric evaluation showed no particular difference between the techniques, with the exception of some organs. Mediastinal irradiation after heart transplantation is feasible and should be considered after evaluation of the risk. Conformal radiotherapy or intensity‐modulated radiotherapy appears to be the appropriate treatment in heart‐transplanted oncologic patients. PMID:27148425

  18. Mediastinal irradiation in a patient affected by lung carcinoma after heart transplantation: Helical tomotherapy versus three dimensional conformal radiotherapy.

    PubMed

    Giugliano, Francesca M; Iorio, Vincenzo; Cammarota, Fabrizio; Toledo, Diego; Senese, Rossana; Francomacaro, Ferdinando; Muto, Matteo; Muto, Paolo

    2016-04-26

    Patients who have undergone solid organ transplants are known to have an increased risk of neoplasia compared with the general population. We report our experience using mediastinal irradiation with helical tomotherapy versus three-dimensional conformal radiation therapy to treat a patient with lung carcinoma 15 years after heart transplantation. Our dosimetric evaluation showed no particular difference between the techniques, with the exception of some organs. Mediastinal irradiation after heart transplantation is feasible and should be considered after evaluation of the risk. Conformal radiotherapy or intensity-modulated radiotherapy appears to be the appropriate treatment in heart-transplanted oncologic patients. PMID:27148425

  19. Right Gaze Palsy and Hoarseness: A Rare Presentation of Mediastinal Tuberculosis with an Isolated Prepontine Cistern Tuberculoma

    PubMed Central

    Agu, Chidozie Charles; Aina, Olufemi; Basunia, Md; Oke, Vikram; Schmidt, Marie Frances; Quist, Joseph; Enriquez, Danilo; Gayam, Vijay

    2015-01-01

    We describe a previously healthy young man who presented with headaches, diplopia with right lateral gaze palsy, dysphagia, and hoarseness over a 2-month period. Magnetic resonance imaging of the brain revealed a small enhancing mass at the prepontine cistern and chest CT showed a left mediastinal mass. Mediastinoscopy and lymph node biopsy were performed. DNA probe and culture of the biopsy specimen were confirmed to be Mycobacterium tuberculosis complex. Resolution of neurologic symptoms was noted after 6 weeks, in addition to regression of brain stem and mediastinal lesions after 12 weeks of antituberculous therapy. PMID:26693365

  20. Mediastinal B-Cell Lymphoma Presenting with Jugular-Subclavian Deep Vein Thrombosis as the First Presentation

    PubMed Central

    Eltawansy, Sherif Ali; Ceniza, Sidney; Sharon, David

    2015-01-01

    Jugular venous thrombosis infrequently could be secondary to malignancy and has seldom been reported secondary to mediastinal large B-cell lymphomas. The postulated mechanisms are mechanical compression that leads to stagnation of blood in the venous system of the neck and/or an increase in the circulating thrombogenic elements that could cause venous thromboembolism as a paraneoplastic phenomenon. We report the case of a middle aged male presenting with right sided neck pain and arm swelling secondary to ipsilateral jugular-subclavian deep vein thrombosis. Investigations revealed it to be secondary to a mediastinal mass shown on CT scan of the chest. PMID:25821628

  1. Predictive Factors of Superior Mediastinal Nodal Metastasis from Papillary Thyroid Carcinoma—A Prospective Observational Study

    PubMed Central

    Woo, Joo Hyun; Park, Ki Nam; Lee, Jae Yong; Lee, Seung Won

    2016-01-01

    Objectives The purpose of this study was to demonstrate the incidence rates and predictive factors of superior mediastinal lymph node (SMLN) metastasis in PTC (papillary thyroid carcinoma) patients. Methods A prospective observational study was performed between January 2009 and January 2011. PTC patients who had tumors with a maximal diameter greater than 1 cm and clinically negative SMLNs were included in this study. Finally, a total of 217 patients who underwent total thyroidectomy with central compartment neck dissection (CND) and elective superior mediastinal lymph node dissection (SMLND), with or without modified radical neck dissection (MRND) and revisional CND, were included. Results Occult SMLN metastasis was present in 15.7% (34/217). Cytological classifications of tumor, BRAFV600E mutation, Tumor size, T-stage, perithyroidal extension, lymphovascular invasion, multifocality, and paratracheal pN(+) were not predictive of SMLN metastasis (P > .05), while revision surgery, pretracheal pN(+), and multiple lateral pN(+) were associated with SMLN metastasis. There were no major complications related to SMLND. Transient and permanent hypoparathyroidism was observed in 69 cases (31.8%) and 8 cases (3.6%), respectively. Conclusions Despite clinically negative SMLN in preoperative evaluation, SMLN metastasis can be predicted for patients with a PTC tumor size larger than 1 cm, pretracheal LN metastasis, multiple lateral metastasis, and revisional surgery. PMID:26848952

  2. Mediastinal vacuum phenomenon: atypical pneumomediastinum caused by gas replacement of diminished fat

    PubMed Central

    Hagiwara, Hiroaki; Torii, Ikuo

    2015-01-01

    We report a case involving an 83-year-old man with interstitial lung disease who developed atypical pneumomediastinum caused by gas replacement of diminished fat. The patient presented with a complaint of worsening symptoms of respiratory difficulty since a diagnosis of chronic eosinophilic pneumonia 5 months back. He had been under observation with no particular treatment for 5 months. Computed tomography performed on admission revealed pneumomediastinum. When the current scan was compared with that obtained 5 months ago, it was evident that the fat surrounding the mediastinum had been replaced by gas density. There was no mediastinal enlargement, pneumothorax, or pneumopericardium. Because the patient was elderly, home oxygen therapy was initiated for the interstitial pneumonia with no steroid therapy. Computed tomography performed 10 months after discharge showed the reappearance of mediastinal fat and no evidence of gas density. This case is unique because the pneumomediastinum was distinct from spontaneous pneumomediastinum caused by alveolar air leaks and resembled the vacuum phenomenon caused by intervertebral disc degeneration. PMID:26604834

  3. Mediastinal Single Nodal Relapse of a Nasal Nk/T cell Lymphoma

    PubMed Central

    Rhee, Kyoung Hoon; Hong, Seok Chan; An, Jeong Min; Huh, Jooryung; Sook, Ryu Jin; Lee, Jin Seong

    2007-01-01

    A nasal NK/T cell lymphoma is a very aggressive form of lymphoma. Patterns of relapse after treatment have not been systematically evaluated, and mediastinal nodal relapse at a primary site has never been documented. We describe here a 40-year old man who presented with a nasal obstruction caused by a protruding mass that was identified as a nasal NK/T cell lymphoma. The initial work-up, including chest and abdominopelvic computed tomography (CT) and positron emission tomography (PET), showed no regional or distant metastasis. A CT scan performed following three cycles of chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) showed that the mass had nearly disappeared. Radiation therapy undertaken following chemotherapy was given to the primary site. However, PET performed following radiotherapy revealed a single mediastinal lymphadenopathy, with no evidence of residual tumor in the nasal cavity. A biopsy using video-assisted thoracoscopy (VATS) showed the presence of a recurrent NK/T cell lymphoma with an immunophenotype identical to that of the primary nasal lymphoma. An additional three cycles of CHOP chemotherapy were administered, and the patient remains alive, with no evidence of disease 30 months after the initial relapse. These findings indicate that early detection with PET and prompt surgical excision with the use of VATS can lead to successful treatment of a relapsed nasal NK/T cell lymphoma. PMID:17939339

  4. Mediastinal single nodal relapse of a nasal NK/T cell lymphoma.

    PubMed

    Rhee, Kyoung Hoon; Hong, Seok Chan; An, Jeong Min; Huh, Jooryung; Sook, Ryu Jin; Lee, Jin Seong; Suh, Cheolwon

    2007-09-01

    A nasal NK/T cell lymphoma is a very aggressive form of lymphoma. Patterns of relapse after treatment have not been systematically evaluated, and mediastinal nodal relapse at a primary site has never been documented. We describe here a 40-year old man who presented with a nasal obstruction caused by a protruding mass that was identified as a nasal NK/T cell lymphoma. The initial work-up, including chest and abdominopelvic computed tomography (CT) and positron emission tomography (PET), showed no regional or distant metastasis. A CT scan performed following three cycles of chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) showed that the mass had nearly disappeared. Radiation therapy undertaken following chemotherapy was given to the primary site. However, PET performed following radiotherapy revealed a single mediastinal lymphadenopathy, with no evidence of residual tumor in the nasal cavity. A biopsy using video-assisted thoracoscopy (VATS) showed the presence of a recurrent NK/T cell lymphoma with an immunophenotype identical to that of the primary nasal lymphoma. An additional three cycles of CHOP chemotherapy were administered, and the patient remains alive, with no evidence of disease 30 months after the initial relapse. These findings indicate that early detection with PET and prompt surgical excision with the use of VATS can lead to successful treatment of a relapsed nasal NK/T cell lymphoma. PMID:17939339

  5. An unusual mediastinal parathyroid carcinoma coproducing PTH and PTHrP: A case report

    PubMed Central

    CAO, CHUANGJIE; DOU, CHENGYUN; CHEN, FUQIN; WANG, YAN; ZHANG, XIAOLI; LAI, HONG

    2016-01-01

    Parathyroid carcinoma (PTCA) is a rare disease, and ectopic PTCA is particularly rare. Parathyroid hormone-related protein (PTHrP) expression in PTCA has not been previously described in the relevant literature to the best of our knowledge. The present study reports a unique case with a mediastinal parathyroid carcinoma producing parathyroid hormone (PTH) and PTHrP. A 53-year-old man presented with hyperparathyroidism symptoms, including fatigue, chest pain, dizziness, muscular soreness, polyuria, night sweats and renal stones. However, neck ultrasound revealed no significantly abnormal thyroid or parathyroid nodules. Tc99m methoxyisobutylisonitrile (Tc99m-MIBI) scintigraphy scanning indicated an ectopic mediastinal parathyroid adenoma. Histopathological examination revealed PTCA, and the tumor tissue was coproducing PTH and PTHrP. The patient underwent successful surgical operation. Serum calcium and PTH levels remained within normal ranges, and there was no tumor recurrence observed at a 3-year follow-up appointment. Although rare, ectopic parathyroid glands may lead to malignant disease. Clinical symptoms, biochemical tests, ultrasound and Tc99m-MIBI scintigraphy scanning may assist with the diagnosis of this disease. Hypersecretion of PTHrP and PTH contributed collaboratively to the pathogenesis of hypercalcemia due to PTCA. Complete surgical resection with microscopically negative margins is the recommended treatment for PTCA and offers the best chance of a cure. PMID:27313750

  6. [Surgical Resection for Mediastinal Lymph Node Metastasis of Combined Hepatocellular and Cholangiocarcinoma--A Case Report].

    PubMed

    Yoshida, Mitsuhiko; Shimizu, Hiroaki; Ohtsuka, Masayuki; Kato, Atsushi; Yoshitomi, Hideyuki; Furukawa, Katsunori; Takayashiki, Tsukasa; Kuboki, Satoshi; Takano, Shigetsugu; Okamura, Daiki; Suzuki, Daisuke; Sakai, Nozomu; Kagawa, Shingo; Nojima, Hiroyuki; Miyazaki, Masaru

    2015-11-01

    We report the case of a 77-year-old woman with mediastinal lymph node metastasis of combined hepatocellular and cholangiocarcinoma who was successfully treated with S8 segmentectomy and lymphadenectomy. A hepatic nodule was detected in segment 8 during follow-up computed tomography (CT) after left iliac arterial aneurysm repair. The patient was diagnosed with a hepatocellular carcinoma (HCC), and transcatheter arterial chemoembolization (TACE) was selected for HCC because of the patient's condition. The levels of tumor markers did not change after TACE was performed twice. Therefore, TACE treatment was considered to be ineffective for HCC, and the patient was admitted to our hospital for surgical resection. In addition to the primary lesion, a lymph node with a diameter of 20 mm was detected in the anterior mediastinum using CT and magnetic resonance imaging(MRI). We did not find any other metastases, and therefore, S8 segmentectomy and lymphadenectomy in the anterior mediastinum were performed. Recovery was uneventful, and the patient was discharged from the hospital on postoperative day 12. Based on histopathologic findings, combined hepatocellular and cholangiocarcinoma with mediastinal lymph node metastasis was confirmed. Levels of tumor markers normalized, and the patient survived without recurrence for 6 months. PMID:26805196

  7. Follicular variant of peripheral T cell lymphoma with mediastinal involvement in a child: a case report.

    PubMed

    Delas, Audrey; Gaulard, Philippe; Plat, Geneviève; Brousset, Pierre; Laurent, Camille

    2015-03-01

    Peripheral T cell lymphomas are rare in young patients. We report the first case of a follicular variant of peripheral T cell lymphoma not otherwise specified in an 11-year-old boy, who presented with a large mediastinal mass. Microscopic examination of the mediastinal biopsy revealed nodular infiltration of medium- to large-sized atypical lymphocytes. Immunohistochemistry showed expression of follicular helper T cell markers (CD10, PD1, CXCL13, and BCL6) in tumor T cells. Epstein-Barr virus (EBV) was not detected by an in situ hybridization assay for EBV-encoded RNA. Interestingly, fluorescence in situ hybridization detected the presence in the tumor cells of the t(5;9)(q33;q22) translocation, involving ITK and SYK rearrangement. T cell clonality was detected by multiplex PCR analysis of TRG and TRD gene rearrangements. After 4 cycles of systemic chemotherapy, the patient was in complete remission. Although this entity is very rare, our observations show that lymphomas arising from T follicular helper cells may occur in children and that this should be distinguished from other lymphomas, such T-lymphoblastic lymphomas, which require a specific therapeutic approach. PMID:25604350

  8. [Complete resection of a posterior mediastinal tumor after preoperative identification of artery of Adamkiewicz].

    PubMed

    Shiiya, Haruhiko; Tanaka, Akihiko; Sakuraba, Motoki; Nakamura, Masanori; Shibayama, Yui; Tsuji, Takahiro; Fukasawa, Yuichiro

    2014-05-01

    The thoracolumbar spinal cord receives its blood supply primarily from the artery of Adamkiewicz (AA), a branch of thoracolumbar intercostal arteries. Aortic cross-clamping during operation for descending aortic aneurysms can cause paraplegia due to spinal cord ischemia secondary to low blood flow through the AA. A 69-year-old woman was diagnosed with a left posterior mediastinal tumor measuring 66 mm. The tumor was adjacent to the thoracic aorta between Th10 to Th12 vertebral levels. Preoperative 3-dimensional computed tomography (3D-CT) imaging revealed 2 AAs originated from the 10th and 11th left intercostal arteries just near the tumor. The patient underwent a left thoracotomy and the 2 intercostal arteries were carefully dissected from the encapsulated tumor. Complete resection was safely achieved with preservation of the AAs. Pathology revealed a schwannoma. There were no complications. In performing thoracic surgery for posterior mediastinal tumors, it is important to identify the AAs preoperatively and preserve them. PMID:24917281

  9. Adult renal cystic disease: a genetic, biological, and developmental primer.

    PubMed

    Katabathina, Venkata S; Kota, Gopi; Dasyam, Anil K; Shanbhogue, Alampady K P; Prasad, Srinivasa R

    2010-10-01

    Renal cystic diseases in adults are a heterogeneous group of disorders characterized by the presence of multiple cysts in the kidneys. These diseases may be categorized as hereditary, acquired, or developmental on the basis of their pathogenesis. Hereditary conditions include autosomal dominant polycystic kidney disease, medullary cystic kidney disease, von Hippel-Lindau disease, and tuberous sclerosis. Acquired conditions include cystic kidney disease, which develops in patients with end-stage renal disease. Developmental cystic diseases of the adult kidney include localized renal cystic disease, multicystic dysplastic kidney, and medullary sponge kidney. In recent years, many molecular and cellular mechanisms involved in the pathogenesis of renal cystic diseases have been identified. Hereditary renal cystic diseases are characterized by genetic mutations that lead to defects in the structure and function of the primary cilia of renal tubular epithelial cells, abnormal proliferation of tubular epithelium, and increased fluid secretion, all of which ultimately result in the development of renal cysts. A better understanding of these pathophysiologic mechanisms is now providing the basis for the development of more targeted therapeutic drugs for some of these disorders. Cross-sectional imaging provides useful information for diagnosis, surveillance, prognostication, and evaluation of treatment response in renal cystic diseases. PMID:21071372

  10. The Cystic Vein: The Significance of a Forgotten Anatomic Landmark

    PubMed Central

    1997-01-01

    The cystic vein, a portion of biliary anatomy whose insignificance in open gallbladder surgery led to its being relegated to mythology, has been rediscovered by the magnified view of laparoscopic surgery. Its presence is an important anatomic feature that helps distinguish between cystic duct and common hepatic duct, thus diminishing the risk of inadvertent bile duct injury during laparoscopic cholecystectomy. PMID:9876684

  11. The cystic vein: the significance of a forgotten anatomic landmark.

    PubMed

    Fine, A

    1997-01-01

    The cystic vein, a portion of biliary anatomy whose insignificance in open gallbladder surgery led to its being relegated to mythology, has been rediscovered by the magnified view of laparoscopic surgery. Its presence is an important anatomic feature that helps distinguish between cystic duct and common hepatic duct, thus diminishing the risk of inadvertent bile duct injury during laparoscopic cholecystectomy. PMID:9876684

  12. First isolations of Segniliparus rugosus from patients with cystic fibrosis.

    PubMed

    Butler, W Ray; Sheils, Catherine A; Brown-Elliott, Barbara A; Charles, Nadege; Colin, Andrew A; Gant, Mary J; Goodill, John; Hindman, Diane; Toney, Sean R; Wallace, Richard J; Yakrus, Mitchell A

    2007-10-01

    We report three cases of the new genus Segniliparus isolated from patients with cystic fibrosis. All isolates were unambiguously identified by 16S rRNA gene sequencing as Segniliparus rugosus (GenBank accession no. AY 60892). Drug susceptibility results that may enhance treatment for cystic fibrosis patients with this opportunistic pathogen are presented. PMID:17670929

  13. Rare cystic liver lesions: A diagnostic and managing challenge

    PubMed Central

    Bakoyiannis, Andreas; Delis, Spiros; Triantopoulou, Charina; Dervenis, Christos

    2013-01-01

    Cystic formations within the liver are a frequent finding among populations. Besides the common cystic lesions, like simple liver cysts, rare cystic liver lesions like cystadenocarcinoma should also be considered in the differential diagnosis. Thorough knowledge of each entity’s nature and course are key elements to successful treatment. Detailed search in PubMed, Cochrane Database, and international published literature regarding rare cystic liver lesions was carried out. In our research are included not only primary rare lesions like cystadenoma, hydatid cyst, and polycystic liver disease, but also secondary ones like metastasis from gastrointestinal stromal tumors lesions. Up-to date knowledge regarding diagnosis and management of rare cystic liver lesions is provided. A diagnostic and therapeutic algorithm is also proposed. The need for a multidisciplinary approach by a team including radiologists and surgeons familiar with liver cystic entities, diagnostic tools, and treatment modalities is stressed. Patients with cystic liver lesions must be carefully evaluated by a multidisciplinary team, in order to receive the most appropriate treatment, since many cystic liver lesions have a malignant potential and evolution. PMID:24282350

  14. The Spectrum of Nocardia Lung Disease in Cystic Fibrosis.

    PubMed

    Mei-Zahav, Meir; Livnat, Galit; Bentur, Lea; Mussaffi, Huda; Prais, Dario; Stafler, Patrick; Bar-On, Ophir; Steuer, Guy; Blau, Hannah

    2015-08-01

    We reviewed all cases of Nocardia infection in cystic fibrosis patients at 2 centers. Eight of 200 patients had Nocardia in sputum. Four developed severe lung disease, including 3 with associated allergic bronchopulmonary aspergillosis; 4 remained clinically stable. Nocardia is often associated with significant lung disease in cystic fibrosis, possibly associated with allergic bronchopulmonary aspergillosis or steroids. PMID:25973994

  15. Pandoraea pulmonicola chronic colonization in a cystic fibrosis patient, France

    PubMed Central

    Kokcha, S; Bittar, F; Reynaud-Gaubert, M; Mely, L; Gomez, C; Gaubert, J-Y; Thomas, P; Rolain, J-M

    2013-01-01

    Pandoraea are considered emerging multidrug resistant pathogens in the context of cystic fibrosis. We report herein for the first time the case of a 30-year-old woman with cystic fibrosis, living in France, who was chronically infected with Pandoraea pulmonicola and who died of Pseudomonas aeruginosa sepsis 3 weeks after bilateral lung transplantation. PMID:25356323

  16. Rare cystic liver lesions: a diagnostic and managing challenge.

    PubMed

    Bakoyiannis, Andreas; Delis, Spiros; Triantopoulou, Charina; Dervenis, Christos

    2013-11-21

    Cystic formations within the liver are a frequent finding among populations. Besides the common cystic lesions, like simple liver cysts, rare cystic liver lesions like cystadenocarcinoma should also be considered in the differential diagnosis. Thorough knowledge of each entity's nature and course are key elements to successful treatment. Detailed search in PubMed, Cochrane Database, and international published literature regarding rare cystic liver lesions was carried out. In our research are included not only primary rare lesions like cystadenoma, hydatid cyst, and polycystic liver disease, but also secondary ones like metastasis from gastrointestinal stromal tumors lesions. Up-to date knowledge regarding diagnosis and management of rare cystic liver lesions is provided. A diagnostic and therapeutic algorithm is also proposed. The need for a multidisciplinary approach by a team including radiologists and surgeons familiar with liver cystic entities, diagnostic tools, and treatment modalities is stressed. Patients with cystic liver lesions must be carefully evaluated by a multidisciplinary team, in order to receive the most appropriate treatment, since many cystic liver lesions have a malignant potential and evolution. PMID:24282350

  17. Endovascular Treatment of Bilateral Pulmonary Artery Stenoses and Superior Vena Cava Syndrome in a Patient with Advanced Mediastinal Fibrosis

    PubMed Central

    Kuban, Joshua D.; Ramanathan, Rohit; Whigham, Cliff J.

    2016-01-01

    Vascular stenosis is a relatively uncommon and often fatal sequela of mediastinal fibrosis. There are very few reports in the medical literature of endovascular treatment for concomitant bilateral pulmonary artery stenoses and superior vena cava syndrome. We report the endovascular treatment of these conditions in a 54-year-old man, and the long-term outcome. PMID:27303243

  18. Bilateral Breast Enlargement: An Unusual Presentation of Superior Vena Cava Obstruction in a Hemodialysis Patient with Fibrosing Mediastinitis

    SciTech Connect

    Goo, Dong Erk Kim, Yong Jae; Choi, Deuk Lin; Kwon, Kui Hyang; Yang, Seung Boo

    2011-02-15

    A 67-year-old woman with end-stage renal disease presented with profound edema of both breasts. The presence of a patent hemodialysis basilic transposition fistula and superior vena cava obstruction (SVC), due to fibrosing mediastinitis, was demonstrated by the use of fistulography. Endovascular treatment with a balloon and stent caused immediate resolution of the breast edema.

  19. Mediastinal infusion of epirubicin and 5-fluorouracil. A complication of totally implantable central venous systems. Report of a case.

    PubMed

    Rodier, J M; Malbec, L; Lauraine, E P; Batel-Copel, L; Bernadou, A

    1996-01-01

    Perforation of the wall of the superior vena cava by a central venous catheter is reported. The resultant inadvertent infusion of 5-fluorouracil and epirubicin caused a severe acute inflammatory reaction in the right-lobe bronchus, mediastinal infiltration and pleural and pericardial effusions. The patient recovered but has residual mild oesophageal dysfunction. PMID:8781572

  20. Enterococcus faecium Mediastinitis Complicated by Disseminated Candida parapsilosis Infection after Congenital Heart Surgery in a 4-Week-Old Baby.

    PubMed

    Renk, Hanna; Neunhoeffer, Felix; Hölzl, Florian; Hofbeck, Michael; Kumpf, Matthias

    2015-01-01

    Background. Cardiac surgery offers multiple treatment options for children with congenital heart defects. However, infectious complications still remain a major cause of morbidity and mortality in these patients. Mediastinitis is a detrimental complication in children undergoing cardiac surgery. The risk of mediastinitis after delayed sternal closure is up to 10%. Case Presentation. We report a case of Enterococcus faecium mediastinitis in a 4-week-old female baby on extracorporeal membrane oxygenation after Norwood procedure. Although repeated antibiotic irrigation, debridement, and aggressive antibiotic treatment were started early, the pulmonary situation deteriorated. Candida parapsilosis was isolated from bronchoalveolar lavage after pulmonary hemorrhage. Disseminated C. parapsilosis infection with pulmonary involvement was treated with liposomal amphotericin B. Subsequently, inflammatory markers increased again and eventually C. parapsilosis was isolated from the central venous catheter. Conclusion. Children undergoing delayed sternal closure have a higher risk of mediastinitis. Therefore, antibiotic prophylaxis, for example, for soft tissue infection seems justified. However, long-term antibiotic treatment is a risk factor for fungal superinfection. Antifungal treatment of disseminated C. parapsilosis infection may fail in PICU patients with nonbiological material in place due to capacity of this species to form biofilms on medical devices. Immediate removal of central venous catheters and other nonbiological material is life-saving in these patients. PMID:26605096

  1. Enterococcus faecium Mediastinitis Complicated by Disseminated Candida parapsilosis Infection after Congenital Heart Surgery in a 4-Week-Old Baby

    PubMed Central

    Renk, Hanna; Neunhoeffer, Felix; Hölzl, Florian; Hofbeck, Michael; Kumpf, Matthias

    2015-01-01

    Background. Cardiac surgery offers multiple treatment options for children with congenital heart defects. However, infectious complications still remain a major cause of morbidity and mortality in these patients. Mediastinitis is a detrimental complication in children undergoing cardiac surgery. The risk of mediastinitis after delayed sternal closure is up to 10%. Case Presentation. We report a case of Enterococcus faecium mediastinitis in a 4-week-old female baby on extracorporeal membrane oxygenation after Norwood procedure. Although repeated antibiotic irrigation, debridement, and aggressive antibiotic treatment were started early, the pulmonary situation deteriorated. Candida parapsilosis was isolated from bronchoalveolar lavage after pulmonary hemorrhage. Disseminated C. parapsilosis infection with pulmonary involvement was treated with liposomal amphotericin B. Subsequently, inflammatory markers increased again and eventually C. parapsilosis was isolated from the central venous catheter. Conclusion. Children undergoing delayed sternal closure have a higher risk of mediastinitis. Therefore, antibiotic prophylaxis, for example, for soft tissue infection seems justified. However, long-term antibiotic treatment is a risk factor for fungal superinfection. Antifungal treatment of disseminated C. parapsilosis infection may fail in PICU patients with nonbiological material in place due to capacity of this species to form biofilms on medical devices. Immediate removal of central venous catheters and other nonbiological material is life-saving in these patients. PMID:26605096

  2. Cystic fibrosis: need for mass deployable screening methods.

    PubMed

    Sengar, Aditya Singh; Agarwal, Anirudh; Singh, Manish K

    2014-10-01

    Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. CFTR is a member of the adenosine triphosphate (ATP)-binding cassette superfamily of proteins and it functions as a chloride channel. CFTR largely controls the working of epithelial cells of the airways, the gastrointestinal tract, exocrine glands, and genitourinary system. Cystic fibrosis is responsible for severe chronic pulmonary disorders in children. Other maladies in the spectrum of this life-limiting disorder include nasal polyposis, pansinusitis, rectal prolapse, pancreatitis, cholelithiasis, insulin-dependent hyperglycemia, and cirrhosis. This review summarizes the recent state of art in the field of cystic fibrosis diagnostic methods with the help of CF literature published so far and proposes new research domains in the field of cystic fibrosis diagnosis. PMID:24880895

  3. Cystic Fibrosis: Microbiology and Host Response.

    PubMed

    Zemanick, Edith T; Hoffman, Lucas R

    2016-08-01

    The earliest descriptions of lung disease in people with cystic fibrosis (CF) showed the involvement of 3 interacting pathophysiologic elements in CF airways: mucus obstruction, inflammation, and infection. Over the past 7 decades, our understanding of CF respiratory microbiology and inflammation has evolved with the introduction of new treatments, increased longevity, and increasingly sophisticated laboratory techniques. This article reviews the current understanding of infection and inflammation and their roles in CF lung disease. It also discusses how this constantly evolving information is used to inform current therapeutic strategies, measures and predictors of disease severity, and research priorities. PMID:27469179

  4. Cystic Neoplasms of the Exocrine Pancreas

    PubMed Central

    Ribaux, C.; Schnyder, P.

    1990-01-01

    Cystic neoplasms of the pancreas are rare and their diagnosis and treatment can be difficult. This report details 7 patients who had histologically proven serous cystadenoma4, mucinous cystadenoma2 and cystadeno carcinoma1. Computed tomography and sonography allowed excellent preoperative assessment but to attempt a distinction between the histological variants may be hazardous. Two tumours were only autopsy findings and 5 patients underwent laparotomy. It is confirmed that potentially malignant mucinous cystadenomas and cytadenocarcinomas should be resected whenever possible; serous cystadenomas are always benign and should therefore be resected only when the diagnosis is doubtful or if they cause symptoms. PMID:2278913

  5. Cystic masses of neck: A pictorial review

    PubMed Central

    Mittal, Mahesh Kumar; Malik, Amita; Sureka, Binit; Thukral, Brij Bhushan

    2012-01-01

    Cystic masses of neck consist of a variety of pathologic entities. The age of presentation and clinical examination narrow down the differential diagnosis; however, imaging is essential for accurate diagnosis and pretreatment planning. Ultrasound is often used for initial evaluation. Computed tomography (CT) provides additional information with regard to the extent and internal composition of the mass. Fine-needle aspiration cytology (FNAC) has a supplementary role for confirmation of diagnosis. Magnetic resonance imaging may be needed in some cases for preoperative assessment. PMID:23833426

  6. Cystic Fibrosis Diagnosis and Newborn Screening.

    PubMed

    Rosenfeld, Margaret; Sontag, Marci K; Ren, Clement L

    2016-08-01

    The diagnosis of cystic fibrosis (CF) has evolved over the past decade as newborn screening has become universal in the United States and elsewhere. The heterogeneity of phenotypes associated with CF transmembrane conductance regulator (CFTR) dysfunction and mutations in the CFTR gene has become clearer, ranging from classic pancreatic-insufficient CF to manifestations in only 1 organ system to indeterminate diagnoses identified by newborn screening. The tools available for diagnosis have also expanded. This article reviews the newest diagnostic criteria for CF, newborn screening, prenatal screening and diagnosis, and indeterminate diagnoses in newborn-screened infants and symptomatic adults. PMID:27469178

  7. The Changing Microbial Epidemiology in Cystic Fibrosis

    PubMed Central

    LiPuma, John J.

    2010-01-01

    Summary: Infection of the airways remains the primary cause of morbidity and mortality in persons with cystic fibrosis (CF). This review describes salient features of the epidemiologies of microbial species that are involved in respiratory tract infection in CF. The apparently expanding spectrum of species causing infection in CF and recent changes in the incidences and prevalences of infection due to specific bacterial, fungal, and viral species are described. The challenges inherent in tracking and interpreting rates of infection in this patient population are discussed. PMID:20375354

  8. Vitamin K supplementation for cystic fibrosis

    PubMed Central

    Jagannath, Vanitha A; Fedorowicz, Zbys; Thaker, Vidhu; Chang, Anne B

    2015-01-01

    Background Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction. Malabsorption of fat and fat-soluble vitamins (A, D, E, K) may occur and can cause subclinical deficiencies of some of these vitamins. Vitamin K is known to play an important role in both blood coagulation and bone formation. Supplementation with vitamin K appears to be one way of addressing the deficiency, but there is very limited agreement on the appropriate dose and frequency of use of these supplements. Objectives To assess the effects of vitamin K supplementation in people with cystic fibrosis and to determine the optimal dose and route of administration of vitamin K for both routine and therapeutic use. Search methods We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group’s Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Most recent search: 08 October 2014. Selection criteria Randomised and quasi-randomised controlled trials of all preparations of vitamin K used as a supplement compared to either no supplementation (or placebo) at any dose or route and for any duration, in children or adults diagnosed with cystic fibrosis (by sweat test or genetic testing). Data collection and analysis Two authors independently screened papers, extracted trial details and assessed their risk of bias. Main results Two trials (total of 32 participants) each lasting one month were included in the review and were assessed as having a moderate risk of bias. One was a dose-ranging parallel group trial in children (aged 8 to 18 years); and the other (with an older cohort) had a crossover design comparing supplements to no treatment, but no separate data were reported for the first intervention period. Neither of the trials addressed any of the primary outcomes (coagulation, bone formation and quality of life). Both trials reported the restoration

  9. Nontuberculous Mycobacterial Infections in Cystic Fibrosis.

    PubMed

    Martiniano, Stacey L; Nick, Jerry A; Daley, Charles L

    2016-03-01

    Nontuberculous mycobacteria (NTM) are important emerging cystic fibrosis (CF) pathogens, with estimates of prevalence ranging from 6% to 13%. Diagnosis of NTM disease in patients with CF is challenging, as the infection may remain indolent in some, without evidence of clinical consequence, whereas other patients suffer significant morbidity and mortality. Treatment requires prolonged periods of multiple drugs and varies depending on NTM species, resistance pattern, and extent of disease. The development of a disease-specific approach to the diagnosis and treatment of NTM infection in CF patients is a research priority, as a lifelong strategy is needed for this high-risk population. PMID:26857770

  10. Vaccine strategies against cystic fibrosis pathogens.

    PubMed

    Le Moigne, Vincent; Gaillard, Jean-Louis; Herrmann, Jean-Louis

    2016-03-01

    A great number of cystic fibrosis (CF) pathogens such as Pseudomonas aeruginosa, the Burkholderia cepacia and the Mycobacterium abscessus complex raised difficult therapeutic problems due to their intrinsic multi-resistance to numerous antibiotics. Vaccine strategies represent one of the key weapons against these multi-resistant bacteria in a number of clinical settings like CF. Different strategies are considered in order to develop such vaccines, linked either to priming the host response, or by exploiting genomic data derived from the bacterium. Interestingly, virulence factors synthesized by various pathogens might serve as targets for vaccine development and have been, for example, evaluated in the context of CF. PMID:26618824

  11. Benign Cystic Mesothelioma Misdiagnosed as Peritoneal Carcinomatosis

    PubMed Central

    Shin, Hyun Deok; Kim, Suk Bae

    2016-01-01

    Benign cystic mesothelioma (BCM) is a rare benign disease that forms multicystic masses in the abdomen, pelvis, and retroperitoneum. It occurs predominantly in young to middle-aged women. The majority of cases were associated with a history of abdominal or pelvic operation, a history of endometriosis, and pelvic inflammatory disease. We present a unique case of BCM which is different to the previous cases. The patient was a 52-year-old man showing features of peritoneal carcinomatosis accompanied by ascites on abdominal computed tomography scans. We herein report a case of BCM misdiagnosed with peritoneal carcinomatosis.

  12. Targeting the Root Cause of Cystic Fibrosis.

    PubMed

    Trescott, Laura; Holcomb, Joshua; Spellmon, Nicholas; Mcleod, Cathy; Aljehane, Leala; Sun, Fei; Li, Chunying; Yang, Zhe

    2015-01-01

    Cystic Fibrosis (CF) is a serious genetic condition caused by CF transmembrane conductance regulator (CFTR) mutation. CF patients have shortened lifespan due to airway obstruction, infection, and end-stage lung failure. However, recent development in CF therapy suggests a brighter future for CF patients. Targeting specific CFTR mutations aims to potentiate the channel gating activity of impaired CFTR and restore protein trafficking to the plasma membrane. Gene therapy introduces correct CFTR gene into the affected airway epithelium leading to the functional expression of CFTR in CF patients. This review will sum up the current status in CF-cause targeting therapy. PMID:25316272

  13. Nutrition and Growth in Cystic Fibrosis.

    PubMed

    Lusman, Sarah; Sullivan, Jillian

    2016-08-01

    Close attention to nutrition and growth is essential in caring for children with cystic fibrosis (CF). Growth and nutritional status should be monitored as part of routine CF care. Children with CF should achieve growth and nutritional status comparable with that of well-nourished children without CF. Children with CF are at risk for nutritional deficiencies. Optimal nutritional and growth status may be difficult to attain in this population given risk of insufficient caloric intake and likelihood of increased caloric expenditure. Various methods to attain optimal nutritional status may be used, including oral supplementation, behavioral treatment, pharmacotherapy, and enteral nutrition. PMID:27469181

  14. Fluoroscopically guided nose tube drainage of mediastinal abscesses in post-operative gastro-oesophageal anastomotic leakage

    PubMed Central

    Xu, Q Y; Yin, G W; Chen, S X; Jiang, F; Bai, X J; Wu, J D

    2012-01-01

    Objective The aim of this study was to retrospectively evaluate the technical success rates and clinical effectiveness of fluoroscopically guided nose tube drainage of mediastinal abscesses and a nasojejunum feeding tube in post-operative gastro-oesophageal anastomotic leakage (GEAL). Methods From January 2006 to June 2011, 18 cases of post-operative GEAL with mediastinal abscesses after oesophagectomy with intrathoracic oesophagogastric anastomotic procedures for oesophageal and cardiac carcinoma were treated by insertion of a nose drainage tube and nasojejunum feeding tube under fluoroscopic guidance. We evaluated the feasibility of two-tube insertion to facilitate leakage site closure and complete resolution of the abscess, and the patients’ nutritional benefit was also evaluated by checking the serum albumin level between pre- and post-enteral feeding via the feeding tube. Results The two tubes were placed successfully under fluoroscopic guidance in 18 patients (100%). The procedure time for two-tube insertion ranged from 20 to 40 min (mean 30 min). 17 patients (94%) achieved leakage site closure after two-tube insertion and had a good tolerance of two tubes in the nasal cavity. The serum albumin level was significant, increased from pre-enteral feeding (2.49±0.42 g dl−1) to the post-enteral feeding (3.58±0.47 g dl−1) via the feeding tube (p<0.001). The duration of follow-up ranged from 1 to 49 months (mean 19 months). Conclusion The insertion of nose tube drainage and a nasojejunum feeding tube under fluoroscopic guidance is safe, and it provides effective relief from mediastinal abscesses in GEAL after oesophagectomy. Moreover, our findings indicate that two-tube insertion may be used as a selective procedure to treat mediastinal abscesses in post-operative GEAL. Advances in knowledge Directive drainage of mediastinal abscesses in post-operative GEAL may be an effective treatment. PMID:22806622

  15. Mucinous Cystic Neoplasm of the Liver Masquerading as an Echinococcal Cyst: Radiologic-pathologic Differential of Complex Cystic Liver Lesions

    PubMed Central

    Jeong, Daniel; Jiang, Kun; Anaya, Daniel A.

    2016-01-01

    Although simple liver cysts are common, complex cystic liver lesions are infrequent and represent a diagnostic and therapeutic challenge. The differential diagnosis of complex cystic liver lesions can be grouped into neoplastic, infectious or inflammatory, and miscellaneous pathologic entities. Clinicians should remember to consider mucinous cystic neoplasm and echinococcal cysts in the differential, which are uncommon etiologies for liver lesions but may expose unique challenges. We present a case of a 49-year-old female who was referred for evaluation of a new complex cystic liver lesion. The following brief review describes how radiologic imaging and pathologic testing can help distinguish between the broad spectrum of diseases that may produce cystic liver lesions. PMID:27195178

  16. Cystic echinococcosis is an occupational disease?

    PubMed

    Youssefi, Mohammad Reza; Mirshafiei, Sepide; Moshfegh, Zeinab; Soleymani, Norredin; Rahimi, Mohammad Taghi

    2016-09-01

    Cystic echinococcosis (CE) is considered as an occupational infection and among different careers greengroceries have a close contact with raw vegetables which can transmit eggs of Echinococcus granulosus. Therefore, the objective of this survey was to determine the seroprevalence of CE among greengroceries using ELISA test in Mazandaran and Gilan Provinces, northern Iran. Overall, 160 serum samples (60 male and 100 female) were collected from greengroceries in Mazandaran and Gilan Provinces, 2012. Sera were surveyed employing ELISA assay for diagnosis of CE. In addition, before sampling, a questionnaire was filled out for each subject. Seropositivity was 2.4 % (four cases) and females 1.8 % (three cases) were more positive compared to males 0.8 % (one case). Age group of 40-60 years encompassed the highest rate of positivity (1.8 %) and the least rate was in 20-40 years old age group (0 %). Besides, 1.8 % of seropositive subjects were dog owner, unaware of the disease and lives in rural areas. In conclusion, seroprevalence of cystic hydatid disease among greengroceries is relatively high in Mazandaran and Gilan Provinces. More continuous serological researches and preventive measures should be taken into consideration owing to the significance of the disease. PMID:27605752

  17. CFTR protein repair therapy in cystic fibrosis.

    PubMed

    Quintana-Gallego, Esther; Delgado-Pecellín, Isabel; Calero Acuña, Carmen

    2014-04-01

    Cystic fibrosis is a single gene, autosomal recessive disorder, in which more than 1,900 mutations grouped into 6 classes have been described. It is an example a disease that could be well placed to benefit from personalised medicine. There are currently 2 very different approaches that aim to correct the basic defect: gene therapy, aimed at correcting the genetic alteration, and therapy aimed at correcting the defect in the CFTR protein. The latter is beginning to show promising results, with several molecules under development. Ataluren (PTC124) is a molecule designed to make the ribosomes become less sensitive to the premature stop codons responsible for class i mutations. Lumacaftor (VX-809) is a CFTR corrector directed at class ii mutations, among which Phe508del is the most frequent, with encouraging results. Ivacaftor (VX-770) is a potentiator, the only one marketed to date, which has shown good efficacy for the class iii mutation Gly551Asp in children over the age of 6 and adults. These drugs, or a combination of them, are currently undergoing various clinical trials for other less common genetic mutations. In the last 5 years, CFTR has been designated as a therapeutic target. Ivacaftor is the first drug to treat the basic defect in cystic fibrosis, but only provides a response in a small number of patients. New drugs capable of restoring the CFTR protein damaged by the most common mutations are required. PMID:24095197

  18. Cystic fibrosis chronic rhinosinusitis: A comprehensive review

    PubMed Central

    Chaaban, Mohamad R.; Kejner, Alexandra; Rowe, Steven M.

    2013-01-01

    Background: Advances in the care of patients with cystic fibrosis (CF) have improved pulmonary outcomes and survival. In addition, rapid developments regarding the underlying genetic and molecular basis of the disease have led to numerous novel targets for treatment. However, clinical and basic scientific research focusing on therapeutic strategies for CF-associated chronic rhinosinusitis (CRS) lags behind the evidence-based approaches currently used for pulmonary disease. Methods: This review evaluates the available literature and provides an update concerning the pathophysiology, current treatment approaches, and future pharmaceutical tactics in the management of CRS in patients with CF. Results: Optimal medical and surgical strategies for CF CRS are lacking because of a dearth of well-performed clinical trials. Medical and surgical interventions are supported primarily by level 2 or 3 evidence and are aimed at improving clearance of mucus, infection, and inflammation. A number of novel therapeutics that target the basic defect in the cystic fibrosis transmembrane conductance regulator channel are currently under investigation. Ivacaftor, a corrector of the G551D mutation, was recently approved by the Food and Drug Administration. However, sinonasal outcomes using this and other novel drugs are pending. Conclusion: CRS is a lifelong disease in CF patients that can lead to substantial morbidity and decreased quality of life. A multidisciplinary approach will be necessary to develop consistent and evidence-based treatment paradigms. PMID:24119602

  19. The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

    PubMed Central

    Rosenberg, Mark F.; O'Ryan, Liam P.; Hughes, Guy; Zhao, Zhefeng; Aleksandrov, Luba A.; Riordan, John R.; Ford, Robert C.

    2011-01-01

    Cystic fibrosis affects about 1 in 2500 live births and involves loss of transmembrane chloride flux due to a lack of a membrane protein channel termed the cystic fibrosis transmembrane conductance regulator (CFTR). We have studied CFTR structure by electron crystallography. The data were compared with existing structures of other ATP-binding cassette transporters. The protein was crystallized in the outward facing state and resembled the well characterized Sav1866 transporter. We identified regions in the CFTR map, not accounted for by Sav1866, which were potential locations for the regulatory region as well as the channel gate. In this analysis, we were aided by the fact that the unit cell was composed of two molecules not related by crystallographic symmetry. We also identified regions in the fitted Sav1866 model that were missing from the map, hence regions that were either disordered in CFTR or differently organized compared with Sav1866. Apart from the N and C termini, this indicated that in CFTR, the cytoplasmic end of transmembrane helix 5/11 and its associated loop could be partly disordered (or alternatively located). PMID:21931164

  20. Lung Infections Associated with Cystic Fibrosis

    PubMed Central

    Lyczak, Jeffrey B.; Cannon, Carolyn L.; Pier, Gerald B.

    2002-01-01

    While originally characterized as a collection of related syndromes, cystic fibrosis (CF) is now recognized as a single disease whose diverse symptoms stem from the wide tissue distribution of the gene product that is defective in CF, the ion channel and regulator, cystic fibrosis transmembrane conductance regulator (CFTR). Defective CFTR protein impacts the function of the pancreas and alters the consistency of mucosal secretions. The latter of these effects probably plays an important role in the defective resistance of CF patients to many pathogens. As the modalities of CF research have changed over the decades from empirical histological studies to include biophysical measurements of CFTR function, the clinical management of this disease has similarly evolved to effectively address the ever-changing spectrum of CF-related infectious diseases. These factors have led to the successful management of many CF-related infections with the notable exception of chronic lung infection with the gram-negative bacterium Pseudomonas aeruginosa. The virulence of P. aeruginosa stems from multiple bacterial attributes, including antibiotic resistance, the ability to utilize quorum-sensing signals to form biofilms, the destructive potential of a multitude of its microbial toxins, and the ability to acquire a mucoid phenotype, which renders this microbe resistant to both the innate and acquired immunologic defenses of the host. PMID:11932230

  1. Targeting ion channels in cystic fibrosis.

    PubMed

    Mall, Marcus A; Galietta, Luis J V

    2015-09-01

    Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause a characteristic defect in epithelial ion transport that plays a central role in the pathogenesis of cystic fibrosis (CF). Hence, pharmacological correction of this ion transport defect by targeting of mutant CFTR, or alternative ion channels that may compensate for CFTR dysfunction, has long been considered as an attractive approach to a causal therapy of this life-limiting disease. The recent introduction of the CFTR potentiator ivacaftor into the therapy of a subgroup of patients with specific CFTR mutations was a major milestone and enormous stimulus for seeking effective ion transport modulators for all patients with CF. In this review, we discuss recent breakthroughs and setbacks with CFTR modulators designed to rescue mutant CFTR including the common mutation F508del. Further, we examine the alternative chloride channels TMEM16A and SLC26A9, as well as the epithelial sodium channel ENaC as alternative targets in CF lung disease, which remains the major cause of morbidity and mortality in patients with CF. Finally, we will focus on the hurdles that still need to be overcome to make effective ion transport modulation therapies available for all patients with CF irrespective of their CFTR genotype. PMID:26115565

  2. Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

    PubMed Central

    Corradi, Valentina; Vergani, Paola; Tieleman, D. Peter

    2015-01-01

    The cystic fibrosis transmembrane conductance regulator (CFTR) is a member of the ATP-binding cassette (ABC) transporter superfamily. CFTR controls the flow of anions through the apical membrane of epithelia. Dysfunctional CFTR causes the common lethal genetic disease cystic fibrosis. Transitions between open and closed states of CFTR are regulated by ATP binding and hydrolysis on the cytosolic nucleotide binding domains, which are coupled with the transmembrane (TM) domains forming the pathway for anion permeation. Lack of structural data hampers a global understanding of CFTR and thus the development of “rational” approaches directly targeting defective CFTR. In this work, we explored possible conformational states of the CFTR gating cycle by means of homology modeling. As templates, we used structures of homologous ABC transporters, namely TM(287–288), ABC-B10, McjD, and Sav1866. In the light of published experimental results, structural analysis of the transmembrane cavity suggests that the TM(287–288)-based CFTR model could correspond to a commonly occupied closed state, whereas the McjD-based model could represent an open state. The models capture the important role played by Phe-337 as a filter/gating residue and provide structural information on the conformational transition from closed to open channel. PMID:26229102

  3. Purely cystic adrenal lesion in a newborn evolving into a solid neuroblastoma.

    PubMed

    Gali, Shapira; Anat, Ilivitzki

    2015-02-01

    Purely cystic neuroblatomas are often discovered prenatally. As the main differential diagnosis is adrenal hemorrhage, follow-up sonography is warranted after birth. Cystic neuroblastomas are expected to evolve into lesions of mixed echogenicity with cystic and solid components. We present a rare case of a purely cystic left-sided adrenal lesion in a newborn, suggesting an adrenal hemorrhage, which on follow-up sonography evolved into a purely solid mass with poor vascularization, diagnosed as a cystic neuroblastoma. We suggest that even purely cystic adrenal masses in the newborn should be closely followed up with sonography, as they may represent purely cystic neuroblastomas. PMID:24947197

  4. Superior vena caval syndrome and ipsilateral pleural effusion: A rare presentation of anterior mediastinal thymoma.

    PubMed

    Das, Anirban; Pandit, Sudipta; Choudhury, Sabyasachi; Das, Sibes K; Basuthakur, Sumitra

    2014-10-01

    Incidence of thymic malignancies is very low. Thymoma, a tumor of thymus gland, is of epithelial origin and is most common anterior mediastinal tumor. In most cases, thymomas are localized and locally advanced thymomas may rarely present with superior vena caval obstruction (SVCO) and malignant pleural deposits. Microscopically, capsular invasion is noted in case of locally advanced thymomas, which behave like a malignant neoplasm. Complete surgical removal of the tumor along with intact capsule is the treatment modality of choice in case of localized tumors. Neoadjuvant radiotherapy (RT) and chemotherapy followed by surgical resection of residual tumor is useful in case of locally advanced tumors. RT is especially useful in case of SVCO to relieve the distressing respiratory symptoms. Here, we report a rare case of locally advanced thymoma, complicated by SVCO and ipsilateral pleural effusion in a 53-year-old male patient. PMID:25378848

  5. [Video-assisted thoracic surgery, lung transplantation and mediastinitis: major issues in thoracic surgery in 2010].

    PubMed

    Borro, José M; Moreno, Ramón; Gómez, Ana; Duque, José Luis

    2011-01-01

    We reviewed the major issues in thoracic surgery relating to the advances made in our specialty in 2010. To do this, the 43(rd) Congress of the Spanish Society of Pneumology and Thoracic Surgery held in La Coruña and the articles published in the Society's journal, Archivos de Bronconeumología, were reviewed. The main areas of interest were related to the development of video-assisted thoracic surgery, lung transplantation and descending mediastinitis. The new tumor-node-metastasis (TNM) classification (7(th) edition), presented last year, was still a topical issue this year. The First Forum of Thoracic Surgeons and the Update in Thoracic Surgery together with the Nurses' Area have constituted an excellent teaching program. PMID:21300211

  6. Concurrent thymoma, thymic carcinoma, and T lymphoblastic leukemia/lymphoma in an anterior mediastinal mass.

    PubMed

    Ito, Junko; Yoshida, Akihiko; Maeshima, Akiko Miyagi; Nakagawa, Kazuo; Watanabe, Shun-ichi; Kobayashi, Yukio; Fukuhara, Suguru; Tsuta, Koji

    2015-09-01

    We report a case of a 62-year-old man with concurrent thymoma, thymic carcinoma, and T lymphoblastic leukemia/lymphoma. Computed tomography revealed a 5.5-cm anterior mediastinal mass, and surgical resection was performed. Histologically, the mass showed concurrent thymoma (type AB), thymic carcinoma, and T lymphoblastic leukemia/lymphoma. Lymphoma cells infiltrated in the left lung, pulmonary hilar lymph nodes, and involved bone marrow. The patient underwent chemotherapy for T lymphoblastic leukemia/lymphoma and achieved remission. One year after surgery, he remains free of both thymoma and thymic carcinoma, and T lymphoblastic leukemia/lymphoma remains complete remission under maintenance therapy. Thymoma and T lymphoblastic leukemia/lymphoma can combine in the same mass, although this is quite rare. At the time of the diagnosis of thymoma, additional attention should be directed toward lymphocytes in the background. PMID:26150396

  7. [Transmanubrial Osteomuscular-sparing Approach for Resection of Superior Mediastinal Schwannoma].

    PubMed

    Aragaki, Masato; Iimura, Yasuyuki; Sato, Nagato; Fukuda, Naoya; Miyazaki, Dai; Umemoto, Kazufumi; Yoshida, Yusuke; Hasegawa, Naoto

    2016-08-01

    Various approaches can be applied to resect superior mediastinal tumor. It is important to choose the procedure according to the location, size, and characteristics of the tumor in order to perform safe procedure surgery because of distinctive anatomy of this site. We hereby report on a case of Schwannoma of the superior mediastinum resected by the transmanubrial approach. A 67-year-old woman was referred to our department to examine an abnormal chest shadow found at a regular health checkup. Computed tomography revealed a tumor 58×52 mm in size extending from the left supraclavicular fossa to the upper border of the aortic arch. The surgery was performed under the diagnosis of neurogenic tumor using the transmanubrial approach. The tumor was resected safely and the pathological diagnosis was a Schwannoma. Transmanubrial approach was found to be quite useful in securing an adequate visual field and enabling the safe separation of blood vessels and nerves from the tumor. PMID:27476565

  8. [Undetected hypopharyngeal perforation with deep neck abscess and mediastinitis due to transesophageal echocardiography].

    PubMed

    Eichhorn, K W G; Bley, T A; Ridder, G J

    2003-11-01

    Hypopharyngeal perforation is a rare but dangerous complication caused by diagnostic procedures. If there is any suspicion of perforation of the upper airways and/or upper digestive tract, immediate diagnostic and therapeutic procedures are indicated. Possible complications of a hypopharyngeal perforation are deep neck infection with subsequent mediastinitis and haemorrhage from major cervical vessels, both of which have high mortality rates. We present the case of a 65 year old male patient with perforation of the hypopharynx after transesophageal echocardiography which was unrecognized for more than 1 week. Clinical symptoms of hypopharyngeal perforation may initially be unspecific. Esophagography (Gastrografin administration) and computed tomography as well as esophagoscopy are needed to certify the diagnosis and to evaluate the extent of the lesion. Pathological findings as well as diagnostic and therapeutic needs are demonstrated and discussed. PMID:14605709

  9. Technetium-99m-sulfur colloid identification of a mediastinal mass: Case report

    SciTech Connect

    Eisenberg, B.; Heyman, S.; Velchik, M.G. )

    1989-09-01

    The authors present a case of an 8-yr-old boy who had a tracheoesophageal fistula repaired shortly after birth. At that time, a right posterior chest mass was noted. Since he remained asymptomatic and all other studies were negative, he was followed with serial chest x-rays. A question arose as to whether the mass was enlarging out of proportion to the growth of the thorax, and the etiology of the mass was sought. A liver-spleen scan was performed, which demonstrated liver parenchyma in the area of the mass noted on chest x-ray. Diaphragmatic herniations (congenital or traumatic) should always be considered whenever mediastinal masses are noted on chest x-ray.

  10. Evaluation of the effects of mediastinal radiation therapy on autonomic nervous system.

    PubMed

    Hoca, Ayse; Yildiz, Metin; Ozyigit, Gokhan

    2012-12-01

    In this prospective study, the effects of mediastinal radiation therapy (RT) on autonomic nervous system (ANS) were investigated by heart rate variability (HRV) analysis that is accepted as a non-invasive indicator of ANS. Study was performed with the eligible patients had a histopathologically confirmed diagnosis of malignant disease with no known congestive heart failure, coronary heart disease, hypertension, valvular cardiac disease or arrhythmia history. Electrocardiograms of 14 voluntary patients were recorded for duration of 5 min just before and after irradiation for the first and the 15th fractions. ANS-related HRV analysis parameters were calculated as which were recommended by Task Force of ESC/NASPE (Circulation 93:1043-1065, 1996). HRV parameters that belong to pre- and post-RT treatment of patients were compared statistically. We found that there is not effect of single-dose radiation on HRV parameter. The mean RRI (782.29 ± 115.65-738.93 ± 111.01, P < 0.014) and HF power of HRVs PSD (156.94 ± 229.37-60.71 ± 77.99, P < 0.045) decreased, and LF/HF ratio (1.38 ± 0.79-2.03 ± 1.25, P < 0.039) increased significantly with 28-Gy external radiation dose. As the effect of cumulative dose was investigated on HRV parameters, the above changes were continued to increase with 30-Gy external radiation dose. We found that mediastinal RT involving heart directly in the radiation field decreased vagal and sympathetic ANS activities, and autonomic balance shifted toward sympathetic dominance. PMID:22528518

  11. Diagnosis of Malignancy of Adult Mediastinal Tumors by Conventional and Transesophageal Echocardiography

    PubMed Central

    Zhou, Wei-Wei; Wang, Hong-Wei; Liu, Nan-Nan; Li, Jing-Jing; Yuan, Wei; Zhao, Rui; Xiang, Liang-Bi; Qi, Miao

    2015-01-01

    Background: Transesophageal echocardiography (TEE) is a well-established method for detecting and diagnosing heart tumors. In contrast, its role in assessing the presence, growth and evidence of malignant tumors originating from mediastinal sites remains unclear. The aim of this study was to compare the diagnostic impact of TEE and transthoracic echocardiography (TTE) for determining the localization, growth and malignancy of adult mediastinal tumors (MTs). Methods: In a prospective and investigator-blinded study, we evaluated 144 consecutive patients with MT lesions to assess the diagnostic impact of TEE and TTE for detecting the presence of tumors spreading both inside and outside of the heart and for determining infiltration and invasion using pathological examination results as a reference. Results: All tumor lesions were diagnosed and carefully evaluated by biopsy. Biopsy revealed malignant tumors in 79 patients and benign tumors in 65 patients. When compared to histological findings, TEE predicted malignancy from the presence of tumors spreading both inside and outside of the heart and from infiltration and invasion in 49/79 patients (62.0%). TTE predicted malignancy in only 8/79 patients (10.1%, P < 0.005). TEE visualized tumor lesions in 130 patients (90.3%) while the TTE visualized tumor lesions in 110 patients (76.4%) and was less effective at detecting MT lesions (P < 0.001). TTE and TEE could detect anterior MTs and adequately verified MTs (P > 0.05); TEE detected medium MTs better than TTE (P < 0.001). Conclusions: TEE is effective and superior to TTE for predicting the localization and growth of MTs as well as for accessing evidence of tumor malignancy. TTE and TEE were able to detect anterior MTs; TEE was able to detect medium MT better than TTE. PMID:25881598

  12. Hydraulic conductivity, albumin reflection and diffusion coefficients of pig mediastinal pleura.

    PubMed

    Parameswaran, S; Brown, L V; Ibbott, G S; Lai-Fook, S J

    1999-09-01

    Hydraulic conductivity (L), albumin reflection coefficient (sigma), and albumin diffusion coefficient (D) were measured across pig mediastinal pleura. The tissue (7 mm diameter) was bonded between two chambers. Flow (Q) of lactated Ringer solution between the chambers was measured in turn at driving pressures (DeltaP) of 2, 4, and 6 cm H(2)O. Value of L was proportional to the slope of the Q-DeltaP curve. Then Q was measured in turn at three albumin osmotic pressure differences (Deltapi equivalent to -1, -2, and -3 g/dl albumin concentration difference, DeltaC) with DeltaP constant at either 2, 3, 4, or 6 cm H(2)O. From Starling's equation, magnitude of sigma was the slope of the Q-Deltapi curve divided by the slope of the Q-DeltaP curve. We measured the diffusion of 0, 2, 5, and 10 g/dl albumin with tracer (125)I-albumin. Tracer mass (M) that diffused across the pleura was measured for 10 h using a well-type NaI(T1) detector. D was calculated from the slope of the M-time curve. Values of L averaged 2.0 x 10(-8) cm(3). s(-1). dyne(-1) (n = 23). Values of sigma were small (0.02-0.05) and sigma increased as flow increased 20-fold. D (n = 24) increased 3-fold from 2.7 x 10(-8) cm(2)/s as DeltaC increased from 0 to 10 g/dl. The small values of sigma indicated that mediastinal pleura provided little restriction to the passage of protein. PMID:10458927

  13. Myeloid Sarcoma: An Unusual Case of Mediastinal Mass and Malignant Pleural Effusion with Review of Literature.

    PubMed

    Sahu, Kamal Kant; Tyagi, Ruchita; Law, Arjun Datt; Khadwal, Alka; Prakash, Gaurav; Rajwanshi, Arvind; Varma, Subhash Chander; Malhotra, Pankaj

    2015-12-01

    Myeloid sarcoma is an extramedullary tumor seen most commonly in patients with acute myeloid leukemia and less frequently in chronic myeloid leukemia, myelodysplastic syndrome and rarely, in an isolated form without any other underlying malignancy. Malignant pleural effusion in hematological malignancies is rare when compared with solid tumors. We present an unusual case of myeloid sarcoma in which a mediastinal mass with pleural effusion was the initial presentation. A 27 year old gentleman presented with complaints of fever, chest pain and swelling in the anterior chest wall for 6 months. Examination revealed a lump measuring 5 × 5 cm on the left side of the chest wall. Hematological evaluation showed hemoglobin-14.2 g/dL, platelet count-233 × 10(9)/L, TLC-117 × 10(6)/L with normal differential counts. Contrast enhanced computerised tomography (CECT) confirmed the presence of a soft tissue mass in the superior mediastinum abutting against the chest wall. Core biopsy was suggestive of myeloid sarcoma and immunohistochemistry was positive for myeloperoxidase and negative for CD3, CD 20 and CD 23. Pleural fluid analysis showed the presence of malignant cells. Bone marrow examination did not show an excess of blasts. A final diagnosis of extramedullary myeloid sarcoma with malignant pleural effusion was made. The patient was given induction chemotherapy (3 + 7 regimen) with daunorubicin and cytosine arabinoside. Repeat CECT done on day 28 showed complete resolution of pleural effusion and significant reduction in the size of mediastinal mass. The patient has successfully completed three cycles of consolidation therapy following which there has been complete resolution of the mass. He remains asymptomatic on close follow up. PMID:26306072

  14. Randomized Clinical Trial to Assess the Efficacy of Radiotherapy in Primary Mediastinal Large B-Lymphoma

    SciTech Connect

    Aviles, Agustin; Neri, Natividad; Fernandez, Raul; Huerta-Guzman, Judith; Nambo, Maria J.

    2012-07-15

    Purpose: We developed a controlled clinical trial to assess the efficacy and toxicity of adjuvant-involved field radiotherapy (IFRT) in patients with primary mediastinal B-cell lymphoma that achieved complete response after the patients were treated with cyclophosphamide, doxorubicin, vincristine, prednisone, and rituximab (R-CHOP-14). Methods and Materials: Between January 2001 and June 2004, 124 consecutive patients who were in complete remission after dose dense chemotherapy and rituximab administration (R-CHOP14) were randomly assigned to received IFRT (30 Gy). Sixty-three patients received IFR, and 61 patients did not (control group). Results: The study aimed to include 182 patients in each arm but was closed prematurely because in a security analysis (June 2004), progression and early relapse were more frequent in patients that did not received IFRT. Patients were followed until March 2009, at which point actuarial curves at 10 years showed that progression free-survival was 72% in patients who received IFR and 20% in the control group (p < 0.001), overall survival was 72% and 31%, respectively (p < 0.001). Acute toxicity was mild and well tolerated. Discussion: Adjuvant radiotherapy to sites of bulky disease was the only difference to have an improvement in outcome in our patients; the use of rituximab during induction did not improve complete response rates and did affect overall survival; patients who received rituximab but not IFRT had a worse prognosis. Conclusions: The use of IFRT in patients with primary mediastinal B-cell lymphoma who achieved complete response remain as the best treatment available, even in patients that received rituximab during induction.

  15. Chylothorax and Recurrent Laryngeal Nerve Injury Associated with Robotic Video-Assisted Mediastinal Lymph Node Dissection

    PubMed Central

    Sarkaria, Inderpal S.; Finley, David J.; Bains, Manjit S.; Adusumilli, Prasad S.; Rizk, Nabil P.; Huang, James; Downey, Robert J.; Rusch, Valerie W.; Jones, David R.

    2016-01-01

    Summary Objective Although the technical aspects of robotic video-assisted thoracic surgery (RVATS) for lung resections may be advantageous, compared with standard thoracoscopy, complications of chylothorax and recurrent laryngeal nerve injury (RLNI) associated with mediastinal lymph node dissection (MLND) may be significant. Methods Consecutive patients who underwent RVATS anatomic lung resection for suspected or confirmed cancer and experienced RLNI or chylothorax were identified and reviewed from a prospectively maintained database. Complications were graded according to the Common Terminology Criteria for Adverse Events version 3.0. Results From July 28, 2010, to December 20, 2013, 251 patients underwent RVATS segmentectomy, lobectomy, or bilobectomy with mediastinal lymph node dissection (MLND). Eleven patients (4.4%) experienced MLND-related complications and composed the study group; 81.8% were right-sided resections, and median lymph node counts in right station IV and station VII were 9 (range, 1–23) and 5.5 (range, 1–10); 72.7% of cases were performed for early-stage I and II lung cancers. Chylothorax (6/251 [2.4%]) and RLNI (6/251 [2.4%]) were significantly more common in the RVATS group than in the open thoracotomy and standard VATS groups. Complications requiring procedural intervention (Grade 3) are as follows: 4 cases of RLNI in patients undergoing percutaneous vocal cord medialization and 3 cases of chylothorax in patients undergoing image-guided thoracic duct embolization or maceration. No operative interventions were required. Conclusions RVATS MLND may be associated with increased rates of chylothorax and RLNI. Attention must be paid to identifying potential technical pitfalls with RVATS lung resections, adjusting surgical techniques accordingly, and minimizing patient morbidity. PMID:26165562

  16. Cystic renal tumors: new entities and novel concepts.

    PubMed

    Moch, Holger

    2010-05-01

    Cystic renal neoplasms and renal epithelial stromal tumors are diagnostically challenging and represent some novel tumor entities. In this article, clinical and pathologic features of established and novel entities are discussed. Predominantly cystic renal tumors include cystic nephroma/mixed epithelial and stromal tumor, synovial sarcoma, and multilocular cystic renal cell carcinoma. These entities are own tumor entities of the 2004 WHO classification of renal tumors. Tubulocystic carcinoma and acquired cystic disease-associated renal cell carcinoma are neoplasms with an intrinsically cystic growth pattern. Both tumor types should be included in a future WHO classification as novel entities owing to their characteristic features. Cysts and clear cell renal cell carcinoma frequently coexist within the kidneys of patients with von Hippel-Lindau disease. Sporadic clear cell renal cell carcinomas often contain cysts, usually as a minor component. Some clear cell renal cell carcinomas have prominent cysts, and multilocular cystic renal cell carcinoma is composed almost exclusively of cysts. Recent molecular findings suggest that clear cell renal cancer may develop through cyst-dependent and cyst-independent molecular pathways. PMID:20418675

  17. Air Trapping and Airflow Obstruction in Newborn Cystic Fibrosis Piglets

    PubMed Central

    Adam, Ryan J.; Michalski, Andrew S.; Bauer, Christian; Abou Alaiwa, Mahmoud H.; Gross, Thomas J.; Awadalla, Maged S.; Bouzek, Drake C.; Gansemer, Nicholas D.; Taft, Peter J.; Hoegger, Mark J.; Diwakar, Amit; Ochs, Matthias; Reinhardt, Joseph M.; Hoffman, Eric A.; Beichel, Reinhard R.; Meyerholz, David K.

    2013-01-01

    Rationale: Air trapping and airflow obstruction are being increasingly identified in infants with cystic fibrosis. These findings are commonly attributed to airway infection, inflammation, and mucus buildup. Objectives: To learn if air trapping and airflow obstruction are present before the onset of airway infection and inflammation in cystic fibrosis. Methods: On the day they are born, piglets with cystic fibrosis lack airway infection and inflammation. Therefore, we used newborn wild-type piglets and piglets with cystic fibrosis to assess air trapping, airway size, and lung volume with inspiratory and expiratory X-ray computed tomography scans. Micro–computed tomography scanning was used to assess more distal airway sizes. Airway resistance was determined with a mechanical ventilator. Mean linear intercept and alveolar surface area were determined using stereologic methods. Measurements and Main Results: On the day they were born, piglets with cystic fibrosis exhibited air trapping more frequently than wild-type piglets (75% vs. 12.5%, respectively). Moreover, newborn piglets with cystic fibrosis had increased airway resistance that was accompanied by luminal size reduction in the trachea, mainstem bronchi, and proximal airways. In contrast, mean linear intercept length, alveolar surface area, and lung volume were similar between both genotypes. Conclusions: The presence of air trapping, airflow obstruction, and airway size reduction in newborn piglets with cystic fibrosis before the onset of airway infection, inflammation, and mucus accumulation indicates that cystic fibrosis impacts airway development. Our findings suggest that early airflow obstruction and air trapping in infants with cystic fibrosis might, in part, be caused by congenital airway abnormalities. PMID:24168209

  18. Cystic Fibrosis Transmembrane Conductance Regulator. Implications in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.

    PubMed

    Cantin, André M

    2016-04-01

    Cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD) have traditionally been viewed as two distinct entities of unrelated origins. However, molecular, cellular, and clinical studies have revealed that cystic fibrosis transmembrane conductance regulator (CFTR) protein dysfunction is common to both conditions, one (CF) being defined genetically and the other (COPD) as an acquired CFTR deficiency. Multiple molecular mechanisms of cigarette smoke-induced CFTR dysfunction have been reported. More importantly, considerable evidence of cigarette smoke-induced CFTR dysfunction in several respiratory and nonrespiratory tissues have been confirmed, making CFTR a target that cannot be overlooked in our quest to understand COPD and improve therapies for individuals affected by this disease. This review summarizes the molecular, cellular, and clinical evidence that CFTR dysfunction is induced by cigarette smoke exposure both in vitro and in vivo, and explores how this may contribute to the development of COPD. PMID:27115950

  19. Remnant cystic duct adenocarcinoma presenting as gastric outlet obstruction

    PubMed Central

    Lo, Samuel Tsoon Wuan; Cheng, Yue; Cheung, Frances; Tang, Chung Ngai

    2016-01-01

    Only a few case reports of remnant cystic duct carcinoma exist. The presented case of remnant cystic duct carcinoma with invasion to pylorus and bulbus of duodenum leading to gastric outlet obstruction was the first of its kind. We reviewed all cases of remnant cystic duct carcinoma that we found in the literature and summarized its definition, presentation, extent of invasion and clinical outcome after operation. The diagnosis can be difficult due to the rarity of disease, locally advanced nature of disease and distorted postoperative anatomy. A high index of suspicion can increase the likelihood of a preoperative diagnosis. PMID:27154747

  20. Cystic change in primary paediatric optic nerve sheath meningioma.

    PubMed

    Narayan, Daniel; Rajak, Saul; Patel, Sandy; Selva, Dinesh

    2016-08-01

    Primary optic nerve sheath meningiomas (PONSM) are rare in children. Cystic meningiomas are an uncommon subgroup of meningiomas. We report a case of paediatric PONSM managed using observation alone that underwent cystic change and radiological regression. A 5-year-old girl presented with visual impairment and proptosis. Magnetic resonance (MR) imaging demonstrated a PONSM. The patient was left untreated and followed up with regular MR imaging. Repeat imaging at 16 years of age showed the tumour had started to develop cystic change. Repeat imaging at 21 years of age showed the tumour had decreased in size. PMID:27310300

  1. [Pancreatic cystic fibrosis in Mexicans over 15 years of age].

    PubMed

    Quezada, R; Hernandez, N; Sada, E

    1990-01-01

    A better knowledge of cystic fibrosis of the pancreas has contributed to raise the detection of cystic fibrosis in adults. We describe nine Mexican patients older than 15 years with cystic fibrosis. Respiratory symptoms were predominant and they were secondary to bronchiectasis. All patients were infected by mucoid Pseudomona aeruginosa and in some cases, the finding of this microorganism in sputum suggested the diagnosis. In Mexican population the cystyc fibrosis of the pancreas can be found in adult patients, and it should be considered in the differential diagnosis of chronic respiratory diseases in adults. PMID:2125356

  2. Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis

    PubMed Central

    Sloane, Peter A.; Rowe, Steven M.

    2013-01-01

    Purpose of review Recent progress in understanding the production, processing, and function of the cystic fibrosis gene product, the cystic fibrosis transmembrane conductance regulator (CFTR), has revealed new therapeutic targets to repair the mutant protein. Classification of CFTR mutations and new treatment strategies to address each will be described here. Recent findings High-throughput screening and other drug discovery efforts have identified small molecules that restore activity to mutant CFTR. Compounds such as VX-770 that potentiate CFTR have demonstrated exciting results in recent clinical trials and demonstrate robust effects across several CFTR mutation classes in the laboratory. A number of novel F508del CFTR processing correctors restore protein to the cell surface and improve ion channel function in vitro and are augmented by coadministration of CFTR potentiators. Ongoing discovery efforts that target protein folding, CFTR trafficking, and cell stress have also indicated promising results. Aminoglycosides and the novel small molecule ataluren induce translational readthrough of nonsense mutations in CFTR and other genetic diseases in vitro and in vivo and have shown activity in proof of concept trials, and ataluren is now being studied in confirmatory trials. Summary An improved understanding of the molecular mechanisms underlying the basic genetic defect in cystic fibrosis have led to new treatment strategies to repair the mutant protein. PMID:20829696

  3. Mediastinal Packing for Intractable Coagulopathy in Acute Aortic Dissection (Types 1 and 2 DeBakey): A Life-Saving Technique—Report of Experiences

    PubMed Central

    Moeinipour, Aliasghar; Fathi, Mehdi; Sepehri Shamloo, Alireza; Amini, Shahram; Hoseinikhah, Hamid; Kianinejad, Akram

    2015-01-01

    Nonsurgical bleeding after complex thoracic aortic procedures (such as aortic dissection and aortic aneurysm) is a great challenge for cardiac surgeons because of severe coagulopathy, exsanguinous bleeding, and inevitable death. Temporary mediastinal packing (with sponge) in such cases is the only life-saving technique with good result in most cases. Herein, we presented three cases with acute aortic dissection with intractable bleeding that was successfully managed with mediastinal packing. PMID:26435855

  4. A rare case of mediastinal metastasis of ovarian carcinoma diagnosed by endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA).

    PubMed

    Carbonari, Augusto; Camunha, Marco; Binato, Marcelo; Saieg, Mauro; Marioni, Fabio; Rossini, Lucio

    2015-10-01

    Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) has become a minimally invasive tool with excellent diagnostic accuracy and low risk of complications in the diagnosis of thoracic diseases, including lung cancers and primary mediastinal lesions. Occasionally, EBUS-TBNA may be useful in identifying thoracic metastasis from distant tumors. Here we report an interesting and rare case of mediastinal metastasis of ovarian carcinoma diagnosed by EBUS-TBNA. PMID:26623131

  5. Poly Cystic Ovarian Syndrome: An Updated Overview

    PubMed Central

    El Hayek, Samer; Bitar, Lynn; Hamdar, Layal H.; Mirza, Fadi G.; Daoud, Georges

    2016-01-01

    Poly Cystic Ovarian Syndrome (PCOS) is one of the most common metabolic and reproductive disorders among women of reproductive age. Women suffering from PCOS present with a constellation of symptoms associated with menstrual dysfunction and androgen excess, which significantly impacts their quality of life. They may be at increased risk of multiple morbidities, including obesity, insulin resistance, type II diabetes mellitus, cardiovascular disease (CVD), infertility, cancer, and psychological disorders. This review summarizes what the literature has so far provided from guidelines to diagnosis of PCOS. It will also present a general overview about the morbidities associated with this disease, specifically with its more severe classic form. Finally, the review will stress on the various aspects of treatment and screening recommendations currently used in the management of this condition. PMID:27092084

  6. Control programme for cystic echinococcosis in Uruguay

    PubMed Central

    Irabedra, Pilar; Ferreira, Ciro; Sayes, Julio; Elola, Susana; Rodríguez, Miriam; Morel, Noelia; Segura, Sebastian; dos Santos, Estela; Guisantes, Jorge A

    2016-01-01

    Cystic echinococcosis is a highly endemic parasitic zoonosis that is present in the Southern Cone countries of America. For several decades, various prevention and control programmes have been implemented in different countries and regions, with varying results. In Uruguay, a new control programme was implemented in 2006 that employed new strategies for canine diagnosis and treatment, dog population control, diagnosis in humans, epidemiological surveillance, and health education, including community participation. The control programme in Uruguay addresses the control and surveillance of the disease from a holistic perspective based on Primary Health Care, which has strengthened the community’s participation in developing and coordinating activities in an interdisciplinary manner. Similarly, the control programme that is currently implemented is based on a risk-focused approach. The surveillance and control measures were focused on small villages and extremely poor urban areas. In this study, the strategies used and the results obtained from 2008-2013 are analysed and discussed. PMID:27223652

  7. Paediatric nasal polyps in cystic fibrosis.

    PubMed

    Mohd Slim, Mohd Afiq; Dick, David; Trimble, Keith; McKee, Gary

    2016-01-01

    Patients with cystic fibrosis (CF) are at increased risk of nasal polyps. We present the case of a 17-month-old Caucasian patient with CF who presented with hypertelorism causing cycloplegic astigmatism, right-sided mucoid discharge, snoring and noisy breathing. Imaging suggested bilateral mucoceles in the ethmoid sinuses. Intraoperatively, bilateral soft tissue masses were noted, and both posterior choanae were patent. Polypectomy and bilateral mega-antrostomies were performed. Histological examination revealed inflammatory nasal polyposis typical of CF. The role of early functional endoscopic sinus surgery (FESS) in children with CF nasal polyposis remains questionable as the recurrence rate is higher, and no improvement in pulmonary function has been shown. Our case, however, clearly demonstrates the beneficial upper airway symptom relief and normalisation of facial appearance following FESS in a child with this condition. PMID:27329094

  8. Cystic fibrosis transmembrane regulator correctors and potentiators.

    PubMed

    Rowe, Steven M; Verkman, Alan S

    2013-07-01

    Cystic fibrosis (CF) is caused by loss-of-function mutations in the CF transmembrane conductance regulator (CFTR) protein, a cAMP-regulated anion channel expressed primarily at the apical plasma membrane of secretory epithelia. Nearly 2000 mutations in the CFTR gene have been identified that cause disease by impairing its translation, cellular processing, and/or chloride channel gating. The fundamental premise of CFTR corrector and potentiator therapy for CF is that addressing the underlying defects in the cellular processing and chloride channel function of CF-causing mutant CFTR alleles will result in clinical benefit by addressing the basic defect underlying CF. Correctors are principally targeted at F508del cellular misprocessing, whereas potentiators are intended to restore cAMP-dependent chloride channel activity to mutant CFTRs at the cell surface. This article reviews the discovery of CFTR potentiators and correctors, what is known regarding their mechanistic basis, and encouraging results achieved in clinical testing. PMID:23818513

  9. Cystic Fibrosis Transmembrane Regulator Correctors and Potentiators

    PubMed Central

    Rowe, Steven M.; Verkman, Alan S.

    2013-01-01

    Cystic fibrosis (CF) is caused by loss-of-function mutations in the CF transmembrane conductance regulator (CFTR) protein, a cAMP-regulated anion channel expressed primarily at the apical plasma membrane of secretory epithelia. Nearly 2000 mutations in the CFTR gene have been identified that cause disease by impairing its translation, cellular processing, and/or chloride channel gating. The fundamental premise of CFTR corrector and potentiator therapy for CF is that addressing the underlying defects in the cellular processing and chloride channel function of CF-causing mutant CFTR alleles will result in clinical benefit by addressing the basic defect underlying CF. Correctors are principally targeted at F508del cellular misprocessing, whereas potentiators are intended to restore cAMP-dependent chloride channel activity to mutant CFTRs at the cell surface. This article reviews the discovery of CFTR potentiators and correctors, what is known regarding their mechanistic basis, and encouraging results achieved in clinical testing. PMID:23818513

  10. Developmental and psychosocial issues in cystic fibrosis.

    PubMed

    Ernst, Michelle M; Johnson, Mark C; Stark, Lori J

    2011-08-01

    Cystic fibrosis (CF) is a multisystemic life-limiting genetic disorder, primarily affecting respiratory functioning. Most patients with CF are diagnosed by 2 years of age, and the current median predicted survival rate is 37.4 years old, with 95% of patients dying from complications related to pulmonary infection. Given the chronic, progressive, and disabling nature of CF, multiple treatments are prescribed, most on a daily basis. Thus, this illness requires children, with the aid of their families, to adopt multiple health-related behaviors in addition to managing more typical developmental demands. The morbidity and mortality factors pose cognitive, emotional, and behavioral challenges for many children with CF and their families. This article applies a developmental perspective to describing the psychosocial factors affecting psychological adjustment and health-related behaviors relevant to infants, preschool and school-age children, and adolescents with CF. Topics particularly pertinent to developmental periods and medical milestones are noted, with clinical implications highlighted. PMID:21855711

  11. Intraorbital Cystic Lesions: An Imaging Spectrum.

    PubMed

    Pahwa, Shivani; Sharma, Sanjay; Das, Chandan J; Dhamija, Ekta; Agrawal, Saurabh

    2015-01-01

    Presence of a cyst or a cystic component in an intraorbital mass often narrows the list of differential diagnoses to specific entities. Such a lesion in the orbit may arise from structures within the orbit, globe, and lacrimal system or from neighboring paranasal sinuses or meninges. Common congenital and developmental lesions encountered within the orbit include dermoids and epidermoids, and infrequently coloboma. Parasitic cysts (cysticercus), orbital abscess, mucocele, and vascular lesions are the most common acquired pathologies giving rise to fluid-containing lesions within the orbit. The role of a radiologist is crucial in expediting the diagnosis of orbital lesions with the help of characteristic imaging features on ultrasound, computed tomography, or magnetic resonance imaging. It also helps in identifying complications in others where formulation of an early and effective management strategy is vital for preserving vision. PMID:25908230

  12. Poly Cystic Ovarian Syndrome: An Updated Overview.

    PubMed

    El Hayek, Samer; Bitar, Lynn; Hamdar, Layal H; Mirza, Fadi G; Daoud, Georges

    2016-01-01

    Poly Cystic Ovarian Syndrome (PCOS) is one of the most common metabolic and reproductive disorders among women of reproductive age. Women suffering from PCOS present with a constellation of symptoms associated with menstrual dysfunction and androgen excess, which significantly impacts their quality of life. They may be at increased risk of multiple morbidities, including obesity, insulin resistance, type II diabetes mellitus, cardiovascular disease (CVD), infertility, cancer, and psychological disorders. This review summarizes what the literature has so far provided from guidelines to diagnosis of PCOS. It will also present a general overview about the morbidities associated with this disease, specifically with its more severe classic form. Finally, the review will stress on the various aspects of treatment and screening recommendations currently used in the management of this condition. PMID:27092084

  13. Clinical monitoring of steatorrhoea in cystic fibrosis.

    PubMed Central

    Walters, M P; Kelleher, J; Gilbert, J; Littlewood, J M

    1990-01-01

    In 100 patients with cystic fibrosis the severity of steatorrhoea was assessed by three separate methods. Using chemical faecal fat assay as the gold standard, two other rapid and inexpensive methods were compared with it. The steatocrit method proved unreliable in our hands and gave little indication of the presence or severity of steatorrhoea. The more simple microscopy method was highly sensitive (97%) and only three of 80 patients with steatorrhoea were missed. All patients with severe steatorrhoea (greater than 60 mmol fat/day) were clearly demonstrated. The method is applicable to spot faecal samples and can readily be carried out on an outpatient basis. In centres where faecal fat assays are not available, the simple and cheap microscopic examination will give some indication of the response to enzyme treatment and may also help to identify non-compliant individuals. PMID:2301990

  14. Imaging of Cystic Fibrosis and Pediatric Bronchiectasis.

    PubMed

    Murphy, Kevin P; Maher, Michael M; O'Connor, Owen J

    2016-03-01

    1. CT is superior to pulmonary function tests and chest radiography for the assessment and monitoring of cystic fibrosis (CF)-related lung disease and, also, of pediatric bronchiectasis not caused by CF (hereafter referred to as non-CF bronchiectasis). 2. Low-dose CT protocols that impart radiation doses similar to those used in chest radiography are feasible for the surveillance of patients with bronchiectasis. 3. Chest radiography is still most commonly used as the first-line imaging examination of choice for the assessment of acute complications related to bronchiectasis. 4. Pulmonary MRI, with or without the use of inhaled hyperpolarized gas, can be performed to obtain functional information, and, in dedicated centers, it may yield imaging results comparable to those obtained by CT. 5. Gastrointestinal and pancreaticobiliary manifestations of CF are observed with greater frequency in adults, because of increased life expectancy. PMID:26901001

  15. Adventitial cystic disease of common femoral vein

    PubMed Central

    Suh, Bo-Yang

    2011-01-01

    Adventitial cystic disease (ACD) of venous system is an extremely rare condition. Very few reports of ACD in venous system have been described. In this report we discuss two cases of common femoral vein ACD that presented with a swollen leg by the obstruction of the vein. Ultrasound imaging showed the typical hypoechoic fluid filled cyst with a posterior acoustic window. Computed tomography scan and ascending venogram showed a stenosis to flow in the common femoral vein caused by an extrinsic mass. Trans-adventitial evacuation of cyst with removal of vein wall was performed for both cases. During operation we found the gelatinous material in the cysts arising in the wall of the common femoral vein and compressing the lumen. The patients were released after short hospitalization and have remained symptom free with no recurrence. PMID:22066091

  16. Scoliosis in cystic fibrosis - an appraisal

    SciTech Connect

    Paling, M.R.; Spasovsky-Chernick, M.

    1982-03-01

    An unusually high prevalence (10%) of scoliosis is described in a series of 151 patients aged four years and older with cystic fibrosis. The scolioses were of the late onset (juvenile and adolescent) type, being typically thoracic with the curve convex to the right, although there was no significant preference for either sex. No direct relationship was found between the spinal curvature and the severity or distribution of the lung disease, although the worse scolioses tended to occur in patients with relatively severe pulmonary involvement. There was no evidence of metabolic bone disease as a predisposing cause. Some indication of a familial tendency towards scoliosis was apparent, and a genetic or constitutional basis is postulated with an unknown precipitating factor.

  17. Two Cases of the Laryngeal Cystic Lesions

    PubMed Central

    Kusunoki, Takeshi; Wada, Ryo; Homma, Hirotomo; Kidokoro, Yoshinobu; Yanai, Aya; Ikeda, Katsuhisa

    2016-01-01

    We experienced two rare cases with laryngeal cystic lesions (laryngocele and laryngeal cyst). In the first case, the laryngocele case was removed by laryngomicrosurgery using an oral approach under general anesthesia. In the second case, the magnetic resonance imaging demonstrated a dumbbell-type cyst with mucus widely extending from the laryngeal lumen to the neck through the thyroid cartilage. The patient had undergone chemotherapy for renal carcinoma with multiple lung and bone metastases. Therefore, we performed only fine needle aspiration rather than aggressive surgery for extirpation of the cyst using an external approach. This fine needle aspiration could improve the quality of life by decreasing both the left laryngeal swelling and the resulting pain, which were the chief complaints. PMID:27162605

  18. Chronic Rhinosinusitis in Patients with Cystic Fibrosis.

    PubMed

    Hamilos, Daniel L

    2016-01-01

    Chronic rhinosinusitis (CRS) is highly prevalent in patients with cystic fibrosis (CF) and accounts for significant morbidity and contribution to CF lung disease. Mutations of the cystic fibrosis transmembrane regulator gene occur with increased prevalence in patients with CRS without CF, suggesting some contribution to CRS pathophysiology. Nasal polyps (NPs) occur with increased prevalence in patients with CF of all ages and have a more neutrophilic appearance with fewer eosinophils and increased submucosal glandular elements in comparison to NPs from patients without CF. Mainstays of medical treatment include isotonic saline irrigations and topical intranasal glucocorticoids, with some evidence that topical intranasal glucocorticoids reduce NP size. Although inhaled hypertonic saline (7%) has been widely studied as a mucolytic agent for CF lung disease, there are no reports of its use in CF CRS. Mucolytics have also not been studied as a treatment for CRS in CF, and most evidence does not support their use for CF lung disease. Nasally nebulized dornase alfa (recombinant human deoxyribonuclease) following sinus surgery shows promise for treatment. Other unproven therapies include addition of baby shampoo to isotonic saline to potentially thin mucus and help prevent biofilm formation. There are no data to support the use of low-dose oral macrolide antibiotics or the use of prophylactic oral antibiotics for CRS in patients with CF. However, there is some support for the use of topical antibiotics, including colistimethate sodium or tobramycin, administered as a sinus irrigation or antral lavage in patients following sinus surgery when susceptible bacteria are cultured. Key components of CF sinus surgical management include extensive surgery to ensure that the maxillary, frontal, sphenoid, and ethmoid sinuses are all widely opened with smoothing of bony overhangs to prevent mucus retention and bacterial recolonization, postoperative meticulous daily nasal irrigations

  19. Surgical treatment of multiple cystic echinococcosis

    PubMed Central

    Prousalidis, J; Kosmidis, CH; Fahantidis, E; Aletras, O

    2004-01-01

    Background Multiple hydatid disease is a complex surgical problem, and its treatment can follow either conservative principles (drainage or obliteration of the cavity) or radical principles (cystoperi-cystectomy or liver or lung resection). Methods A total of 220 patients with multiple cystic echinococcosis (428 cysts) were managed between 1967 and 1998 with conservative operations (group A) or radical operation (group B). There were 90 men and 130 women, with a mean age of 52 years (range 18–77 years). There were two cysts in 124 patients, three cysts in 40 patients, four in 15 and more than four in 41 patients. These multiple cysts were located at one anatomical site (n=140) or at more than one site (n=80). Multiple (2–3) hepatic cysts occurred in 142 patients, multiple (2–3) lung cysts in 15 and multiple peritoneal cysts in 13 patients. Hepatic cysts co-existed with lung cysts in another 32 patients, with peritoneal cysts in 14 patients and once each with splenic, splenic plus lung cysts and renal cysts, one retroperitoneal cyst coincided with small peritoneal cysts. Results The operative procedure employed was dependent on the type and site of the parasite and the condition of the host. Three of 208 patients operated conservatively (group A) died postoperatively as opposed to receiving radical treatment. Morbidity rates were 8.8% and 12.5% in group A and B and mean hospital stay was 15.8 and 15.1 days, respectively. In group A there was an 8.6% recurrence rate, and recurrent disease was finally managed in each group the overall result could be considered satisfactory. Discussion We conclude that conservative surgery can provide good results in multiple cystic echinococcosis. Radical surgery, with its time-consuming major procedures, is ideal but only in properly selected cases. PMID:18333060

  20. AEROSOL DEPOSITION AS A FUNCTION OF AIRWAY DISEASE: CYSTIC FIBROSIS

    EPA Science Inventory

    Progressive lung disease associated with cystic fibrosis (CF) is a continuous interaction of the processes of airway obstruction, infection and inflammation. ecent literature has suggested that the manifestation of CF could compromise the successful administration of pharmacologi...