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Sample records for mediastinal cystic lymphangioma

  1. Mediastinal Lymphangioma and Chylothorax: Thoracic Involvement of Gorham's Disease

    PubMed Central

    Yoo, So Young; Im, Jung-Gi

    2002-01-01

    We report a case of mediastinal lymphangioma associated with Gorham's disease in a 38-year-old man who had suffered recurrent clavicular fractures during a seven-year period. Mediastinal widening associated with osteolysis of the clavicles and the sternal manubrium was revealed by chest radiography, while computed tomography demonstrated a cystic anterior mediastinal mass infiltrating mediastinal fat and associated with osseous destruction of the clavicles and manubrium. Chylothorax recurred during the course of the disease. PMID:12087203

  2. Abdominal cystic lymphangioma mimicking appendicitis.

    PubMed

    Wake, Sarah; Abhyankar, Aruna; Hutton, Kim

    2013-06-01

    A cystic lymphangioma arising within the abdomen is a rare entity in children. It may present with an abdominal mass and symptoms of abdominal pain, vomiting, and anorexia. These nonspecific clinical symptoms are often attributed to more common acute pediatric conditions. In this report, we describe two pediatric cases of intra-abdominal cystic lymphangioma that were initially diagnosed and treated as appendicitis. True diagnosis was only achieved on surgical excision and pathological investigation of cystic material.

  3. CT evaluation of cystic lymphangiomas of the mediastinum

    SciTech Connect

    Pilla, T.J.; Wolverson, M.K.; Sundaram, M.; Heiberg. E.; Shields, J.B.

    1982-09-01

    Two cases of cystic mediastinal lymphangioma are reported, one involving a 12-year-old boy and the other a 53-year-old man. In both cases a mediastinal mass was evident on plain chest radiographs, while CT scans demonstrated a well-circumscribed lesion of low attenuation molding to the mediastinal contours and enveloping the great vessels, suggesting the diagnosis. The display of the precise extent of the lesion in relation to the surrounding structures was helpful in planning surgery.

  4. Mesenteric cystic lymphangioma mimicking malignancy.

    PubMed

    Hureibi, Khalid; Sunidar, Osama A

    2014-09-01

    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. 2014 BMJ Publishing Group Ltd.

  5. Chylopericardium presenting as cardiac tamponade secondary to mediastinal lymphangioma.

    PubMed

    Alsmady, M M; Aladaileh, M A; Al-Zaben, K; Saleem, M M; Alimoglu, O

    2016-11-01

    Mediastinal lymphangioma is a rare entity and chylopericardium is a rare form of pericardial effusion. We report a case of acute chylous cardiac tamponade due to a cervicomediastinal lymphangioma in a one-year-old boy. A chest x-ray revealed marked cardiac enlargement and echocardiography showed massive pericardial effusion. Emergency surgery was performed whereby a pericardial window was created, followed by excision of the lymphangioma.

  6. Cardiac lymphangioma presenting as intrapericardial cystic mass.

    PubMed

    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.

  7. A 36-Year-Old Female with Recurrent Left Sided Pleural Effusion: A Rare Case of Mediastinal Lymphangioma

    PubMed Central

    Swarnakar, Rajesh N.; Hazarey, Jetendra D.; Dhoble, Chetan; Vaghani, Bhavesh; Ainsley, Alaine S.; Khargie, James F.; Likaj, Lorena

    2016-01-01

    Patient: Female, 36 Final Diagnosis: Mediastinal cystic hygroma Symptoms: Chest discomfort Medication: — Clinical Procedure: — Specialty: Pulmonology Objective: Unusual clinical course Background: Lymphangioma is an atypical non-malignant, lymphatic lesion that is congenital in origin. Lymphangioma is most frequently observed in the head and neck, but can occur at any location in the body. About 65% of lymphangiomas are apparent at birth, while 80–90% are diagnosed by two years of age. Occurrence in adults is rare, as evidenced by less than 100 cases of adult lymphangiomas reported in the literature. Case report: A 36-year-old Indian woman with a medical history of recurrent pleural effusions presented with chief complaints of dyspnea on exertion for one year and a low-grade fever for one month. A thorax CT revealed left-sided pleural effusion with thin internal septations. Thoracoscopy revealed a large cystic lesion arising from the mediastinum from the hilum surrounding the mediastinal great vessels. The diagnosis of lymphangioma was confirmed via histopathologic examination of the cyst. It was managed with partial cystectomy along with the use of a sclerosing agent (talc). Conclusions: The size and location of lymphangiomas can vary, with some patients presenting with serious problems like respiratory distress, while others may be asymptomatic. Complete cyst resection is the gold standard treatment for mediastinal cystic lymphangioma. Partial cyst resection along with the use of sclerosing agents can be an effective option when complete cystectomy is not possible. Although lymphangioma is a rare patient condition, it should be included in the differentials for patients presenting with pleural effusions. Also, a biopsy should be done at the earliest opportunity to differentiate lymphangioma from other mediastinal malignant tumors. PMID:27789902

  8. Cystic Pancreatic Lymphangioma - Diagnostic Role of Endoscopic Ultrasound.

    PubMed

    Carvalho, Diana; Costa, Mariana; Russo, Pedro; Simas, Luís; Baptista, Teresa; Ramos, Gonçalo

    2016-01-01

    Pancreatic cystic lymphangiomas are rare benign lesions that arise from lymphatic vessels, accounting for less than 0.2% of all pancreatic cysts. Typically it is asymptomatic and discovery occurs during imaging exams for non-pancreatic disease. In the past, a definite diagnosis was made through surgery, with complete resection of all tumoral tissue to prevent recurrence. Nowadays, the development of endoscopic ultrasound (EUS) made it possible to identify these cysts combining morphologic ultrasound features, macroscopic aspirated fluid appearance, biochemical and cytological evaluation of the sample. We report two cases of cystic pancreatic lymphangioma diagnosed through EUS, allowing conservative management without surgery. These cases show that cystic pancreatic lymphangioma should be considered in the differential diagnosis of cystic pancreatic lesions and that EUS is an important tool for their recognition.

  9. Calcified cystic lymphangioma of the mesentery: case report.

    PubMed

    Buccoliero, Anna Maria; Castiglione, Francesca; Maio, Vincenza; Morelli, Caterina; Martin, Alessandra; Messineo, Antonio; Taddei, Gian Luigi

    2009-01-01

    Mesenteric cysts are rare and are associated with heterogeneous pathological conditions. We describe an extraordinary case of mesenteric calcified cystic lymphangioma in a 10-year-old boy. To the best of our knowledge only 6 mesenteric calcified cystic lymphangioma have been reported. The patient was admitted with abdominal pain and vomiting. Abdominal X-ray and computed tomography scan documented a calcified cyst which dislocated and compressed the ileum. Laparatomy revealed that the cyst arose in the mesentery at 100 cm from the ileocecal valve. The cystic wall was composed of fibrosclerotic calcified tissue and had an endothelial lining. In the adjacent fibrofatty stroma there were distended hemolymphatics. Postoperative recovery was uneventful. At follow-up 6 months after surgery the patient is well. Calcified cystic lymphangioma of the mesentery should be considered in the differential diagnosis of an intra-abdominal calcified cyst in children.

  10. A Huge Cystic Retroperitoneal Lymphangioma Presenting with Back Pain

    PubMed Central

    Kubachev, Kubach; Abdullaev, Elbrus; Babyshin, Valentin; Neronov, Dmitriy; Abdullaev, Abakar

    2016-01-01

    Retroperitoneal lymphangioma is a rare location and type of benign abdominal tumors. The clinical presentation of this rare disease is nonspecific, ranging from abdominal distention to sepsis. Here we present a 73-year-old female patient with 3-month history of back pain. USG and CT revealed a huge cystic mass which was surgically excised and appeared to be lymphangioma on histopathology. PMID:27843456

  11. Mesenteric lymphangioma mimicking a cystic ovarian mass on imaging.

    PubMed

    Hitzerd, Emilie; van Hamont, Dennis; Pijnenborg, Johanna M A

    2016-02-01

    Pelvic cystic masses are frequently observed in women. Most lesions are benign and of ovarian origin. However, non-ovarian lesions can be easily confused with cystic ovarian masses on imaging, which hampers diagnostic and therapeutic management. In this report, a rare case of mesenteric lymphangioma mimicking an ovarian cystic mass, discovered as an incidental finding on orthopaedic MRI in an adult female, is presented. The report highlights the sometimes difficult diagnostic process of pelvic cystic masses, due to an extensive differential diagnosis and the fact that imaging is often inconclusive. Even though most cystic masses are of ovarian origin, non-ovarian causes can mimic ovarian masses and should be considered as differential diagnoses. Surgical exploration may be necessary to exclude malignant causes. 2016 BMJ Publishing Group Ltd.

  12. Retroperitoneal Cystic Lymphangioma: A Diagnostic and Surgical Challenge

    PubMed Central

    Gümüştaş, Oguzhan Güven; Sanal, Murat; Güner, Osman; Tümay, Volkan

    2013-01-01

    A lymphangioma is a benign proliferation of lymph vessels, producing fluid-filled cysts that result from a blockage of the lymphatic system. The incidence of abdominal lymphangiomas is unknown; however they account for from 3% to 9.2% of all pediatric lymphangiomas, with retroperitoneal lymphangioma representing less than 1% of abdominal lymphangiomas. Due to rarity, preoperative diagnosis is often difficult. PMID:23533897

  13. Abdominal cystic lymphangiomas in pediatrics: surgical approach and outcomes.

    PubMed

    Méndez-Gallart, R; Bautista, A; Estévez, E; Rodríguez-Barca, P

    2011-01-01

    Abdominal Cystic lymphangiomas (ACL) are uncommon benign masses usually presented during infancy. Although extremely rare, they may cause complications; therefore, the recommended therapy is surgical excision. The purpose of this study is to report our experience with the diagnosis and surgical treatment of ACL in pediatric population. From January 1994 to December 2009, 10 patients (6 females; 4 males) with diagnostic confirmation of ACL were retrospectively included in study. Children's age ranged between 9 months and 8 years (mean age at diagnosis was 2.5 years). Clinical presentation, cyst location, imaging studies employed, surgical approach and pathologic features were analyzed. The most common symptom was abdominal pain but three cases were incidentally detected. One case had presented with acute abdomen after traumatic haemorrhage of the tumor. All patients were diagnosed with ultrasonography as first-line radiological study. MRI was used in last three cases. At surgery, concomitant bowel resection was necessary in 3 children. Location of the lesion (omentum, mesentery) did not influence the outcome but surgery was more difficult (operative time over three hours) in patients with lymphangioma affecting mesentery of the jejunum. Mean hospital stay after surgery was 6.7 days. Mean follow-up was 5.1 years. No recurrence of the cystic lymphangioma was noticed during follow-up. One case developed an intestinal occlusion due to bowel adhesions 1 year after surgery. ACL usually affect infants and young children and may present with spectrum of symptoms from an incidental finding to an acute life-threatening abdominal obstruction. Complete excision of the tumor is a safe and effective method in the management of ACL in pediatric population. Surgery is mandatory to avoid potential complications.

  14. 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. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  15. Giant cystic lymphangioma of the mesentery: varied clinical presentation of 3 cases.

    PubMed

    Rami, Mohamed; Mahmoudi, Abdelhalim; El Madi, Aziz; Khalid; Khattala; Afifi, Moulay Abderrahmane; Bouabdallah, Youssef

    2012-01-01

    Giant cystic lymphangioma is an uncommon mesenteric tumor which is usually reported in children. In this paper, we describe 3 cases, that was admitted in our department, two with abdominal distension, pain, and an abdominopelvic mass; the other present an acute abdomen. Preoperative studies including abdominal ultrasonography and computed tomography failed to determine the cause of the pain for the female patients. The laparotomy found a giant cystic tumor of the small bowel mesentery. The histological study showed a tumor that was diagnosed as a cystic lymphangioma. Based on those three cases a review of the literature is suggested.

  16. [A cystic mesenteric lymphangioma presented at the colon sigmoid. Case report].

    PubMed

    Ramírez-Ortega, Miguel Angel; Villegas-Romero, Javier; Márquez-Díaz, Adrián; Gómez-Díaz, Antonio

    2010-01-01

    Cystic lymphangioma of the mesentery is an uncommon tumor; its incidence is 1:160,000. Our objective was to present the case of a patient with cystic lymphangioma of mesentery located at the colon. Female 25 years, attending consultation with a clinical picture suggestive of right ovarian cyst and increased abdominal perimeter. On physical examination abdominal painful tumor was identified, with deep palpation and mobilization. Abdominal ultrasound findings suggested giant right ovarian cyst. Laparotomy showed a cystic mass (18 x 11 cm size) depending mesenterium and involving sigmoid colon, surgical intervention was done after two days for bowel preparation. Resection of the cyst, and colon section involving sigmoid colon with termino-terminal anastomosis, was performed. The pathological report was: cystic lymphangioma of sigmoid mesentery. There was no recurrence at six month follow up. Cystic lymphangioma of the mesentery is a benign abdominal tumor, which occurs frequently in children but in adults is rare. We found 49 cases of mesenteric cysts reported in Mexico, of which 21 were lymphangiomas with a range of 1 to 73 years of age.

  17. 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.

  18. Differentiating a large abdominal cystic lymphangioma from multicystic mesothelioma: report of a case.

    PubMed

    Nagata, Hiroshi; Yonemura, Yutaka; Canbay, Emel; Ishibashi, Haruaki; Narita, Makoto; Mike, Makio; Kano, Nobuyasu

    2014-07-01

    We report a case of retroperitoneal cystic lymphangioma in a 30-year-old woman who presented with abdominal distention and pain. Imaging studies revealed a large, thin-walled multicystic mass occupying the whole abdomen. Based on a preoperative diagnosis of multicystic mesothelioma, we performed laparotomy, which revealed a tumor arising from the gastropancreatic ligament in the posterior wall of the omental bursa. We resected the tumor completely, without the adjacent viscera. The final pathological diagnosis was cystic lymphangioma, based on the immunohistochemical findings of positive CD31 and CD34 expression, the presence of smooth muscle confirmed by smooth muscle antigen and desmin, and negative calretinin, WT-1 and cytokeratins 5/6 expression. Multicystic mesotheliomas and cystic lymphangiomas are so similar in morphology that immunohistochemical staining should be fully utilized to differentiate them.

  19. Intra-abdominal cystic lymphangiomas in infancy and childhood.

    PubMed

    Luo, Chih-Cheng; Huang, Chen-Sheng; Chao, Hsun-Chin; Chu, Shih-Ming; Hsueh, Chuen

    2004-07-01

    Cystic lymphangiomas (CL) rarely present as intra-abdominal masses. Abdominal CL is often discussed in conjunction with mesenteric cysts; however, their histology, location and age of presentation differ significantly. In an attempt to establish a best diagnostic and treatment modality, we report our experience dealing with intra-abdominal CL during a 5-year period. Between January 1998 and December 2003, 12 patients, 7 boys and 5 girls, with a diagnosis of CL were reviewed. Modes of clinical presentation, location of CL, methods of diagnosis, surgical intervention and histological examination were all analyzed. The ages of the 12 patients ranged from 8 days to 6 years. Eleven of the 12 patients were symptomatic with abdominal pain, abdominal distention or palpable mass , dysuria and severe acute abdominal pain mimicking appendicitis. Abdominal ultrasound was done preoperatively in all patients. At laparotomy, 5 CL were located in the omentum, 5 in the mesentery, and another 2 in the retroperitoneum. All omental CL were completely excised without difficulty. CL removal required resection of both the cyst and intestine in 2 patients. One of 2 retropritoneal CL was removed with small areas of the posterior wall of the cyst remaining on the inferior vena cava (IVC). There were no major postoperative complications, deaths, or recurrences in this series. Intra-abdominal CL are usually involved in young children and are usually symptomatic. A preoperative diagnosis is possible with ultrasound study. Complete excision of the cysts with or without intestinal resection is mandatory to prevent recurrence. The long-term prognosis is excellent.

  20. Case Report: Multivisceral Transplantation for an Extensive Cystic Lymphangioma of the Mesenteric Root.

    PubMed

    Moulin, L; Rumbo, C; Romero, P; Pedraza, N; Garcia Hervá, D; Orce, G; Trentadue, J; Ramisch, D; Gondolesi, G

    2016-03-01

    We report the case of a 7-year-old girl with intestinal failure owing to a cystic lymphangioma compromising the root of the mesentery, not amenable to resection, leading to intestinal failure. Oncologic treatment was attempted to reduce tumor size with no response; therefore, she was listed for multivisceral transplantation. Resection of the tumor required resection of all abdominal organs with vascular inflow and outflow. A multivisceral graft (liver, stomach, duodenum-pancreas and spleen complex, small bowel, and right colon) was implanted. For vascular reconstruction, donor's superior vena cava was sutured to the recipient's suprahepatic veins in a common patch. For arterial inflow, an arterial conduit was placed directly to the infrarenal aorta, and sutured to an aortic patch of the graft. Cold ischemia time was 8:45 hours; warm ischemia time was 35 minutes. A double-layer gastrogastric anastomosis and piloroplasty was made; and the distal reconstruction was performed with ileocolic side-to-end anastomosis that allowed to perform of a Bishop-Koop ileostomy for endoscopic monitoring. The patient recovered well after the procedure and was discharged 36 days after transplantation with intestinal sufficiency. To the best of our knowledge, this is the first report describing cystic lymphangioma as an indication for multivisceral transplantation. Copyright © 2016 Elsevier Inc. All rights reserved.

  1. Large Omental Cystic Lymphangioma Masquerading as Mucinous Ovarian Neoplasia in an 8-Year-Old Premenarchal Girl: The Findings from Diagnostic Imaging and Laparoscopic-Assisted Excision.

    PubMed

    Takeda, Akihiro; Ito, Hiroaki; Nakamura, Hiromi

    2017-06-16

    Omental cystic lymphangioma is an extremely rare abdominal mass caused by congenital malformation. An 8-year-old premenarchal girl reported abdominal pain. Diagnostic imaging revealed a large multicystic mass measuring 22 cm in diameter, which occupied the entire abdominal cavity with ascites. Emergency laparoscopy revealed a ruptured large cystic mass originating from the greater omentum; this was followed by successful laparoscopic-assisted excision. The pathological diagnosis was omental cystic lymphangioma. The present findings show that omental cystic lymphangioma masquerading as mucinous ovarian neoplasia was a rare cause of acute abdominal events in a young girl. The present case shows that minimally invasive surgery can be a feasible option, which might achieve a favorable outcome in a young patient with an omental cystic lymphangioma. Copyright © 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  2. MRI of mediastinal parathyroid cystic adenoma causing hyperparathyroidism

    SciTech Connect

    Soler, R.; Bargiela, A.; Cordido, F.; Aguilera, C.; Argueeso, R.; Cao, I.

    1996-01-01

    Primary hyperparathyroidism is a common disorder that results from an increased secretion of parathyroid hormone, most often due to a solitary and solid parathyroid adenoma usually found in the inferior group of parathyroid glands. Parathyroid gland is ectopic in approximately 10 to 20% of the cases, and the retrosternal and prevascular mediastinum is the most common location. Most mediastinal parathyroid adenomas are solid and <3 cm, but mediastinal parathyroid cysts are very uncommon and rarely cause hyperparathyroidism. We know of 18 cases of mediastinal parathyroid cysts that have been previously reported and only four of them presented with hyperparathyroidism. We report an unusual case of hyperparathyroidism due to a large cystic parathyroid adenoma located in the anterior mediastinum diagnosed by MRI. 2 refs., 1 fig.

  3. Pediatric chylolymphatic mesenteric cyst - a separate entity from cystic lymphangioma: a case series.

    PubMed

    Rattan, Kamal Nayan; Nair, Vimoj J; Pathak, Manish; Kumar, Sanjay

    2009-11-09

    Chylolymphatic mesenteric cysts are rare entities with variable presentations and this has surgical implications in the pediatric age group. We carried out a retrospective analysis of the clinical and histopathological records of pediatric patients diagnosed and treated for chylolymphatic mesenteric cysts at our institute from 1998 to 2008. Eight patients met the histopathological criteria of chylolymphatic mesenteric cyst. These patients were in the age range 18 months to 10 years with a mean age of 4.5 years. Of these eight patients, four presented with an abdominal lump, and two each with abdominal pain and acute intestinal obstruction. On clinical examination, five out of the eight patients had a palpable abdominal mass. Laparotomy and complete excision of the cyst along with the involved gut was performed in all patients. There were no postoperative complications or any recurrence during the follow-up period which ranged from 4 months to 8 years. Although very rare, chylolymphatic mesenteric cyst should be kept in mind as one of the differential diagnoses of cystic masses of the abdomen including cystic lymphangioma. Ultrasonography and computed tomography suggest the diagnosis but histopathological examination is required for confirmation. Complete excision of the cyst yields excellent results.

  4. Pediatric chylolymphatic mesenteric cyst - a separate entity from cystic lymphangioma: a case series

    PubMed Central

    2009-01-01

    Introduction Chylolymphatic mesenteric cysts are rare entities with variable presentations and this has surgical implications in the pediatric age group. Case presentation We carried out a retrospective analysis of the clinical and histopathological records of pediatric patients diagnosed and treated for chylolymphatic mesenteric cysts at our institute from 1998 to 2008. Eight patients met the histopathological criteria of chylolymphatic mesenteric cyst. These patients were in the age range 18 months to 10 years with a mean age of 4.5 years. Of these eight patients, four presented with an abdominal lump, and two each with abdominal pain and acute intestinal obstruction. On clinical examination, five out of the eight patients had a palpable abdominal mass. Laparotomy and complete excision of the cyst along with the involved gut was performed in all patients. There were no postoperative complications or any recurrence during the follow-up period which ranged from 4 months to 8 years. Conclusion Although very rare, chylolymphatic mesenteric cyst should be kept in mind as one of the differential diagnoses of cystic masses of the abdomen including cystic lymphangioma. Ultrasonography and computed tomography suggest the diagnosis but histopathological examination is required for confirmation. Complete excision of the cyst yields excellent results. PMID:19946589

  5. Abdominal cystic lymphangioma in a term newborn: A case report and update of new treatments.

    PubMed

    Amodeo, Ilaria; Cavallaro, Giacomo; Raffaeli, Genny; Colombo, Lorenzo; Fumagalli, Monica; Cavalli, Riccardo; Leva, Ernesto; Mosca, Fabio

    2017-02-01

    Lymphatic malformations are benign anomalies derived from the abnormal development of lymphatic channels. Usually asymptomatic, they can cause compression on adjacent structures or present acute complications (bleeding or infection). Small asymptomatic lesions can be conservatively managed since the possibility of spontaneous regressions is described, while symptomatic lesions require active management. Less invasive therapeutic options are now preferred instead of surgery (sclerotherapy, laser therapy). However, there are not uniform therapeutic protocols. We present the case of a term newborn with an abdominal cystic lymphangioma extending from the umbilical to the right inguinal area, reaching the medial surface of the right tight. Despite its large dimensions, which classically request surgical management, the patient was by chance asymptomatic, and the mass did not determine compression on the surrounding organs. Therefore, conservative management was tried, and a close clinical and radiological follow-up was started. This approach permitted a spontaneous regression of the mass and to avoid major surgical intervention. Our purpose is to underline the possibility of conservative management of the major multicystic masses and to focus on less invasive therapeutic options, like sclerotherapy, oral therapy, and laser therapy.

  6. Multicystic, peritoneal mesothelioma: a report with electron microscopy of a case mimicking intra-abdominal cystic hygroma (lymphangioma).

    PubMed

    Mennemeyer, R; Smith, M

    1979-08-01

    A 27-year-old Caucasian female complained of persistent, poorly localized abdominal pain of several months' duration, found to be due to a multicystic diffuse lesion involving omentum, peritoneum and pelvic viscera. Three resections were required within a ten-month period for symptomatic control. The tumor showed many of the clinical, operative, gross and light microscopic features of intra-abdominal lymphagioma. Tissues removed at the third operation, however, were examined by electron microscopy, and found to consist of a myriad of small cysts and channels lined by mesothelial cells. In view of these findings, the lesion was regarded as a cystic peritoneal mesothelioma. Similarities between lymphangioma and peritoneal mesothelioma are discussed. It is suggested that electron microscopic examination of lining cells of cystic lesions which are considered grossly consistent with lymphagiomas may yield additional similar cases.

  7. [Mediastinitis].

    PubMed

    Martínez Vallina, Primitivo; Espinosa Jiménez, Dionisio; Hernández Pérez, Lucía; Triviño Ramírez, Ana

    2011-01-01

    Mediastinitis is defined as acute or chronic inflammation of the mediastinal structures and generally has a low incidence. The most frequent acute cause is sternotomy following cardiac revascularization surgery with both internal mammary arteries, with an incidence of 0.4% to 5% and a mortality of 16.5% to 47%. The most frequent vector is Staphylococcus aureus. Esophageal perforation, usually iatrogenic, is the second most frequent cause of acute mediastinitis, produced by common oropharyngeal flora, with a mortality rate of 20% to 60%, depending on the time of diagnosis. The third most frequent cause is descending necrotizing mediastinitis, the origin being an odontogenous focus in 60% and beta-hemolytic streptococcus the causative agent in 71.5% of cases. The most accurate diagnostic imaging technique is computed tomography. Treatment is almost always surgical and survival depends on its early performance. The worst postsurgical prognostic factor is septic shock.

  8. Benign cranial mediastinal lesions in three cats.

    PubMed

    Malik, R; Gabor, L; Hunt, G B; Church, D B; Barrs, V R; Churcher, R; Dixon, R T; Huxley, C; Canfield, P J

    1997-03-01

    Cranial mediastinal lesions were detected in three cats, associated with respiratory impairment (case one), spontaneous pneumothorax (case two) and myasthenia gravis (case three), respectively. On gross and histological examination, the first case was considered either a lymphangioma or a branchial cystic mass of the thymic region of the mediastinum; a cystic lesion was suggested by sonographic detection of multiple anechoic cavitations within a circumscribed mass, while fine needle aspiration cytology excluded lymphosarcoma. The second case was diagnosed histologically as a cystic thymoma, but the third case was not examined microscopically. The masses were amenable to surgical excision in the first two cats, while this proved unnecessary in the third case because of resolution following treatment with dexamethasone. Corticosteroid responsiveness was unhelpful in distinguishing between these benign lesions and lymphosarcoma, as in two cases there was a partial or complete response to dosing with prednisolone or dexamethasone. These cases are presented to emphasise that conditions other than lymphosarcoma can produce cranial mediastinal lesions in cats, and that the prognosis for surgical treatment of lymphangiomas, multilocular thymic cysts and cystic thymomas can be excellent.

  9. Foetal axillary lymphangioma with ipsilateral pes equinovarus: pitfalls in sonographic differential diagnosis (axillary lymphangioma & pes equinovarus).

    PubMed

    Bulbul Baytur, Yesim; Artunc Ulkumen, Burcu; Pala, Halil Gursoy

    2015-01-01

    Lymphangiomas are rare congenital malformations of the lymphatic system. Despite the benign histology, they are likely to grow rapidly and invade the surrounding tissues. In contrast to the cystic hygromas, lymphangiomas at the axillary region tend to have normal karyotype. However, associated hydrops makes the prognosis poor. Due to isolated few cases in the literature, the true incidence of foetal axillary lymphangiomas is not known. We present here a pre-natal ultrasonographic diagnosis of a 15-week foetus with rapidly growing axillary lymphangioma with ipsilateral foot abnormality which had normal karyotype.

  10. Incidental pleural-based pulmonary lymphangioma.

    PubMed

    Benninghoff, Michael G; Todd, William U; Bascom, Rebecca

    2008-09-01

    Adult benign thoracic lymphangiomas typically present as incidental mediastinal lesions, or, more rarely, as solitary pulmonary nodules. Symptomatic compression of vital structures may require lesion resection or sclerotherapy. In the present report, we describe the incidental finding of a solitary pleural-based pulmonary lymphangioma in a 38-year-old woman with chronic arm and shoulder pain. Positron emission tomography revealed that the lesion was highly fluorodeoxyglucose-avid. Biopsy exposed benign tissue consistent with lymphangioma. After continued radiographic tests, the lesion was determined to be an unlikely source of the patient's chronic pain. The present report is, to our knowledge, the first published case of solitary pleural-based pulmonary lymphangioma in the medical literature.

  11. 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

  12. Parotid lymphangioma associated with facial nerve paralysis.

    PubMed

    Imaizumi, Mitsuyoshi; Tani, Akiko; Ogawa, Hiroshi; Omori, Koichi

    2014-10-01

    Parotid lymphangioma is a relatively rare disease that is usually detected in infancy or early childhood, and which has typical features. Clinical reports of facial nerve paralysis caused by lymphangioma, however, are very rare. Usually, facial nerve paralysis in a child suggests malignancy. Here we report a very rare case of parotid lymphangioma associated with facial nerve paralysis. A 7-year-old boy was admitted to hospital with a rapidly enlarging mass in the left parotid region. Left peripheral-type facial nerve paralysis was also noted. Computed tomography and magnetic resonance imaging also revealed multiple cystic lesions. Open biopsy was undertaken in order to investigate the cause of the facial nerve paralysis. The histopathological findings of the excised tumor were consistent with lymphangioma. Prednisone (40 mg/day) was given in a tapering dose schedule. Facial nerve paralysis was completely cured 1 month after treatment. There has been no recurrent facial nerve paralysis for eight years.

  13. Adult Intussusception due to Cecal Lymphangioma: A Case Report

    PubMed Central

    Kim, Dong Il; Kim, Jae Hun; Kim, Hyun Sung; Jo, Hong Jae

    2011-01-01

    We present a rare case of adult intussusception due to cecal lymphangioma. A 30-year-old female was admitted to our hospital with abdominal pain and a palpatable mass on the right lower quadrant. Preoperative radiologic studies by ultrasound and computed tomography showed ileocolic intussusception with a multiseptated cystic tumor as a leading point on the cecum. An ileocecectomy was performed, and the postoperative course was uneventful. Histopathology showed a cecal lymphangioma. Although endoscopic polypectomy or endoscopic mucosal resection is recommended for pedunculated or semi-pedunculated colonic lymphangiomas less than 2 cm in size, it is proper to treat large or symptomatic colonic lymphangiomas with limited a bowel resection or a tumor resection. PMID:21602970

  14. Percutaneous Sclerotherapy Using Acetic Acid After Failure of Alcohol Ablation in an Intra-abdominal Lymphangioma

    SciTech Connect

    Park, Sang Woo Cha, In Ho; Kim, Kyeong Ah; Hong, Suk Joo; Park, Cheol Min; Chung, Hwan Hoon

    2004-09-15

    We report a case of percutaneous sclerotherapy using acetic acid in a 22-year-old woman with an intra-abdominal cystic lymphangioma who was not successfully treated with ethanol despite multiple trials.

  15. Cervical Cystic Hygroma in an Adult

    PubMed Central

    Derin, Serhan; Şahan, Murat; Dere, Yelda; Çullu, Neşat; Şahan, Leyla

    2014-01-01

    Cystic hygromas/lymphangiomas are extremely rare malformations in adults. They are usually seen in infants and children under 2 years of age. En bloc resection is difficult due to the adhesive characteristics of the tumors. Inadequate surgical intervention often leads to recurrent disease. We report herein the case of a cystic hygroma/lymphangioma that presented as an uncommon mass on the cervical region in an adult, together with its histopathological, radiologic, and operative features. PMID:25548704

  16. [Mediastinal fibrosis].

    PubMed

    Roca Calvo, M J; Suero Molina, F F; Mañes Bonet, N; Alix Trueba, A

    1993-04-01

    Even tough the ultimate etiologic and pathogenic mechanisms of mediastinal fibrosis are not quite established, several causes have been mentioned as the more frequent inducers, such as histoplasmosis, tuberculosis and other granulomatous diseases. Generally the diagnosis is suggested by a hilar or mediastinal mass, which is seen in thorax radiography, because 40% of patients are asymptomatic. Exeresis of fibrotic Magma is difficult because usually there is no separation with trachea neither with superior cava vein. That is why many times we have to restricted ourselves to the surgical approach on the complications. The present work discusses three new cases of mediastinal fibrosis.

  17. Mediastinal tumor

    MedlinePlus

    ... contains the heart, large blood vessels, windpipe (trachea), thymus gland, esophagus, and connective tissues. The mediastinum is divided into 3 sections: Anterior (front) Middle Posterior (back) Mediastinal ... Cancer Read more A.D.A.M., Inc. ...

  18. Cystic lumphangioma of the colon

    SciTech Connect

    Agha, F.P.; Francis, I.R.; Simms, S.M.

    1983-10-01

    Cystic lymphangioma is a rare benign lesion of the gastrointestinal tract, in which the colon is the least frequntly involved site. A case is reported displaying the characteristic radiographic features of an extramucosal intramural mass lesion in a patient with concurrent cystadenocarcinoma of the pancreas, in whom the possibility of a metastatic lesion to the colon could not be excluded except by surgical resection.

  19. MEDIASTINAL EMPHYSEMA

    PubMed Central

    Summers, John E.

    1959-01-01

    Mediastinal emphysema may occur due to migration of air from the lungs, from the esophagus or tracheobronchial tree and from the abdomen. Of especial interest is the mechanism starting with the rupture of the perivascular alveoli due to a rapid decrease in intrathoracic pressure from any cause, the development of pulmonary interstitial emphysema and migration of the air into the mediastinum. In one case the patient had severe interstitial emphysema of the left lung, mediastinal emphysema and subcutaneous emphysema without pneumothorax and rapid improvement followed tracheotomy. In another case the patient had interstitial emphysema of the left lung that did not progress to mediastinal emphysema and subcutaneous emphysema. Pneumothorax was not present. Recovery was more rapid than in the first patient. ImagesFigure 1 (Case 1).Figure 2 (Case 1).Figure 3 (Case 1).Figure 4 (Case 1).Figure 5 (Case 1).Figure 7 (Case 2).Figure 6 (Case 2).Figure 8 (Case 2). PMID:13651959

  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.

  1. Laser treatment of retroperitoneal lymphangiomas in children

    NASA Astrophysics Data System (ADS)

    Waldschmidt, U.; Waldschmidt, Juergen; Cholewa, D.; Stroedter, L.

    2000-06-01

    Retroperitoneal Lymphangiomas are rare tumor-like lesions, predominantly found in the new-born period and pediatric age. They are always benign. About 1 percent of Lymphangiomas arise in the retroperitoneal space. Until now surgical excision was the only consistently effective method. Compete excision of the tumor is in more than a half of the cases not possible, and the rate of occurrences is about 50 percent. Because of this background new therapeutic methods were sought. In 1986 we have introduced the interstitial laser therapy in the treatment of the Lymphangiomas and treated more than 200 cases, 10 of them were located in the retroperitoneum. Technique and results are discussed.

  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. Mediastinal masses in children: experience with 120 cases.

    PubMed

    Gun, Feryal; Erginel, Basak; Unüvar, Aysegul; Kebudi, Rejin; Salman, Tansu; Celik, Alaaddin

    2012-03-01

    Primary mediastinal malignancies are rare tumors and can originate from any mediastinal organ or tissue such as thymic, neurogenic, lymphatic, germinal, or mesenchymal. The authors reviewed all cases of primary pediatric mediastinal masses diagnosed over a 25-year period to determine the pattern of presentation, the histology, and the outcome of the surgical treatment. In this study, 120 primary pediatric mediastinal mass cases diagnosed between 1985 and 2011 are retrospectively evaluated according to their age, sex, symptoms, anatomical location, surgical treatment, and histopathological evaluation. The median age of the patients was 5.8 years. There were 34 benign and 86 malign tumors. Thirty patients were asymptomatic. Common symptoms in the patients were cough, dyspnea, fatigue, fever, abdomen pain, back pain, and neurological symptoms. According to their origins, they were presented as neurogenic tumors (38.3%), lymphomas (18.3%), undifferentiated sarcomas (15%), germ cell tumors (7.5%), and the other tumors (22%) thymic pathologies, lymphangiomas, rhabdomyosarcomas, lipomas, hemangiomas, and Wilms' tumor. Complete resection of the tumor was performed in 86 patients, partial resection of the tumor was the intervention in 11 patients. In 23 patients, biopsy was undertaken. Because of the high incidence of asymptomatic or nonspecific presentation such as the upper airway disease, the presentation of a mediastinal mass in children may be challenging. Neurogenic tumors or lymphomas are indicating surgery, if possible complete resection, for both benign and malignant conditions. Although surgery is the mainstay of therapy for most mediastinal tumors, an experienced multidisciplinary approach is necessary.

  4. Lymphangioma of the small bowel mesentery: a case report and review of the literature.

    PubMed

    Suthiwartnarueput, Worapop; Kiatipunsodsai, Siriphut; Kwankua, Amolchaya; Chaumrattanakul, Utairat

    2012-11-21

    Lymphangioma is a rare benign condition characterized by proliferation of lymphatic spaces. It is usually found in the head and neck of affected children. Lymphangioma of the small-bowel mesentery is rare, having been reported for less than 1% of all lymphangiomas. Importantly, it can cause fatal complications such as volvulus or involvement of the main branch of the mesenteric arteries, requiring emergency surgery. Moreover, the gross and histopathologic findings may resemble benign multicystic mesothelioma and lymphangiomyoma. Immunohistochemical study for factor VIII-related antigen, D2-40, calretinin and human melanoma black-45 (HMB-45) are essential for diagnosis. Factor VIII-related antigen and D2-40 are positive in lymphangioma but negative in benign multicystic mesothelioma. HMB-45 shows positive study in the smooth-muscle cells around the lymphatic spaces of the lymphangiomyoma. We report a case of small-bowel volvulus induced by mesenteric lymphangioma in a 2-year-and-9-mo-old boy who presented with rapid abdominal distension and vomiting. The abdominal computed tomography scan showed a multiseptated mass at the right lower quadrant with a whirl-like small-bowel dilatation, suggestive of a mesenteric cyst with midgut volvulus. The intraoperative findings revealed a huge, lobulated, yellowish pink, cystic mass measuring 20 cm × 20 cm × 10 cm, that was originated from the small bowel mesentery with small-bowel volvulus and small-bowel dilatation. Cut surface of the mass revealed multicystic spaces containing a milky white fluid. The patient underwent tumor removal with small-bowel resection and end-to-end anastomosis. Microscopic examination revealed that the cystic walls were lined with flat endothelial cells and comprised of smooth muscle in the walls. The flat endothelial cells were positive for factor VIII-related antigen and D2-40 but negative for calretinin. HMB-45 showed negative study in the smooth-muscle cells around the lymphatic spaces. Thus

  5. Ileo-ileal intussusception caused by lymphangioma of the small bowel treated by single-incision laparoscopic-assisted ileal resection

    PubMed Central

    Kohga, Atsushi; Kawabe, Akihiro; Hasegawa, Yuto; Yajima, Kiyoshige; Okumura, Takuya; Yamashita, Kimihiro; Isogaki, Jun; Suzuki, Kenji; Komiyama, Akira

    2017-01-01

    Intraabdominal lymphangiomas are uncommon; additionally, those affecting the gastrointestinal tract are rare and account for less than 1% of cases. Intussusception caused by a cystic lymphangioma of the small bowel is extremely rare. The patient was a 20-year-old woman who visited our emergency room with a complaint of abdominal pain. A computed tomography image revealed ileo-ileal intussusception with a leading hypovascular mass measuring 1 cm in a diameter. Single-incision laparoscopic-assisted ileal resection was performed. The surgical specimen consisted of a soft polycystic mass. Macroscopically, a pedunculated polyp with a convolutional pattern was found. Microscopically, the inner surfaces of the cysts were covered with a single layer of endothelial cells. On immunohistochemical examination, the endothelial cells were partially positive for D2-40 and CD34. Smooth muscle cells were also found around the cysts. The lesion was diagnosed as a cystic lymphangioma. Dozens of cases of small bowel lymphangiomas have previously been reported. Of these, cases with intussusception were very rare. This is the first case of small bowel intussusception due to lymphangioma treated by single-incision laparoscopic-assisted surgery. PMID:28104992

  6. Ileo-ileal intussusception caused by lymphangioma of the small bowel treated by single-incision laparoscopic-assisted ileal resection.

    PubMed

    Kohga, Atsushi; Kawabe, Akihiro; Hasegawa, Yuto; Yajima, Kiyoshige; Okumura, Takuya; Yamashita, Kimihiro; Isogaki, Jun; Suzuki, Kenji; Komiyama, Akira

    2017-01-07

    Intraabdominal lymphangiomas are uncommon; additionally, those affecting the gastrointestinal tract are rare and account for less than 1% of cases. Intussusception caused by a cystic lymphangioma of the small bowel is extremely rare. The patient was a 20-year-old woman who visited our emergency room with a complaint of abdominal pain. A computed tomography image revealed ileo-ileal intussusception with a leading hypovascular mass measuring 1 cm in a diameter. Single-incision laparoscopic-assisted ileal resection was performed. The surgical specimen consisted of a soft polycystic mass. Macroscopically, a pedunculated polyp with a convolutional pattern was found. Microscopically, the inner surfaces of the cysts were covered with a single layer of endothelial cells. On immunohistochemical examination, the endothelial cells were partially positive for D2-40 and CD34. Smooth muscle cells were also found around the cysts. The lesion was diagnosed as a cystic lymphangioma. Dozens of cases of small bowel lymphangiomas have previously been reported. Of these, cases with intussusception were very rare. This is the first case of small bowel intussusception due to lymphangioma treated by single-incision laparoscopic-assisted surgery.

  7. The surgical management and outcome of congenital mediastinal malformations

    PubMed Central

    Ballouhey, Quentin; Galinier, Philippe; Abbo, Olivier; Andrieu, Guillaume; Baunin, Christiane; Sartor, Agnès; Rittié, Jean Luc; Léobon, Bertrand

    2012-01-01

    We reviewed our institutional experience with congenital mediastinal masses and compared the postnatal management and outcome of patients with or without prenatal diagnosis. Between January 1997 and August 2011, 24 patients underwent surgical procedures for congenital mediastinal mass. For eight patients, the mass was detected by prenatal ultrasonography at 27 weeks of gestation (range 22–35). Postnatal management consisted in open surgery for seven patients at a mean age of 9 months (range 1 day–20 months) and sclerotherapy for one lymphangioma at 5 months of life. Sixteen patients had postnatal diagnosis at 137 months (±194) of median age. Eight bronchogenic cysts, seven bronchopulmonary foregut malformations, five teratomas, three lymphangiomas and one haemangioma were operated on. The median age at resection was 28 months (1 day–15 years). There were four emergency procedures and no surgical mortality. The median follow-up was 45 months (3–144). The duration of mechanical ventilation and hospital stay was, respectively, 4.6 h and 7.5 days for antenatal patients and 24.3 h and 14.3 days for postnatal diagnosed patients. Prenatal diagnosis allows early management of congenital mediastinal malformations. Early resection can be performed prior to the occurrence of symptoms ∼1 year of life and is associated with an excellent outcome and less morbidity. PMID:22394988

  8. Peritoneal cystic mesothelioma: an electron microscopic and immunohistochemical study of two male patients.

    PubMed Central

    Sienkowski, I K; Russell, A J; Dilly, S A; Djazaeri, B

    1986-01-01

    The clinical, pathological, and ultrastructural features of two cases of peritoneal cystic mesothelioma occurring in men were studied. The results of immunohistochemical staining for CAM 5.2, epithelial membrane antigen, carcinoembryonic antigen, and Factor VIII related antigen are reported for the first time and compared with the staining results of two peritoneal cystic lymphangiomas. Although resembling cystic lymphangioma by light microscopy, cystic mesothelioma may have a greater tendency for local recurrence. Staining for CAM 5.2 or epithelial membrane antigen may facilitate the differentiation of these two entities. Images PMID:2422221

  9. Cystic tumors of the pancreas: imaging and management.

    PubMed

    Dewhurst, Catherine E; Mortele, Koenraad J

    2012-05-01

    Cystic tumors of the pancreas are a subset of rare pancreatic tumors that vary from benign to malignant. Many have specific imaging findings that allow them to be differentiated from each other. This article (1) reviews the imaging features of the common cystic pancreatic lesions, including serous microcystic adenoma, mucinous cystic tumor, intraductal papillary mucinous tumor, and solid pseudopapillary tumor, and including the less common lesions such as cystic endocrine tumors, cystic metastases, cystic teratomas, and lymphangiomas; and (2) provides comprehensive algorithms on how to manage the individual lesions, with recommendations on when to reimage patients. Copyright © 2012 Elsevier Inc. All rights reserved.

  10. Lymphangioma circumscriptum of the vulva in a patient with Noonan syndrome.

    PubMed

    Pootrakul, Llana; Nazareth, Michael R; Cheney, Richard T; Grassi, Marcelle A

    2014-06-01

    Lymphangioma circumscriptum (LC) results from the development of abnormal lymphatic vasculature and is characterized by the presence of grouped vesicles filled with clear or colored fluid. Vulvar localization is uncommon. Abnormalities of the lymphatic system, such as lymphedema and cystic hygroma, are well-known features of genetic disorders such as Noonan syndrome (NS) and Turner syndrome. We report the case of a patient with NS who presented with LC of the vulva. We also discuss the expanding spectrum of clinical anomalies associated with the presentation of NS.

  11. Renal lymphangioma: a cause of neonatal nephromegaly.

    PubMed

    Pickering, S P; Fletcher, B D; Bryan, P J; Abramowsky, C R

    1984-01-01

    A newborn male presented with bilateral nephromegaly and mild hypertension. Function of the right kidney was reduced on excretory urography. Ultrasound showed bilaterally enlarged kidneys with increased echogenicity and poorly defined corticomedullary junctions. Areas of decreased medullary enhancement were seen on CT. Renal biopsy demonstrated lymphangioma, probably arising from the peripelvic renal tissues.

  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. Klippel-Trenaunay Syndrome with Extensive Lymphangiomas.

    PubMed

    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.

  14. Cystic Hygroma in an Adult; a Case Report

    PubMed Central

    Veeraraghavan, G; Denny, C; Lingappa, A

    2009-01-01

    Lymphangioma is a benign infiltrative malformation of the lymphatic channels. Cystic lymphangioma or cystic hygroma is a subtype of lymphangioma which exhibits large macroscopic cystic space histologically. The cause of cystic hygroma is believed to be developmental defect or primary Multilocular cystic malformation of dilated lymphatic channels. Cystic hygroma is a common and distinct entity that is not manifested in the oral cavity but occurs in the neck as a large, deep diffuse swelling. They are usually found in the posterior triangle of the neck. They often cross the midline, reaching axilla and mediastinum. Such localization verifies the complexity and extent of the lymphatic system in the cervical region when compared to other regions of the body. The five main locations where cystic hygroma can occur are, cervical (75–90%), axillary (20%), inguinal, retroperitoneal and thoracic. They usually appear as solitary lesions. They are usually infiltrative, often separating fascial planes and incorporating nerves, muscles, and blood vessels. They are fluctuant, freely mobile, compressible, painless and transilluminate well. The skin overlying the lesion is normal and usually there is no associated lymphadenopathy. Various treatment modalities have been tried. Surgery has been the main form of treatment, but total removal is not possible in all cases because of the extent of the lesion, which sometimes involves vital structures. We report a case of cystic hygroma in a young male patient. PMID:21483540

  15. Lymphangioma-like Kaposi sarcoma: case report.

    PubMed

    Posada García, Celia; García-Cruz, Aranzazu; García-Doval, Ignacio; De La Torre, Carlos; Cruces, Manuel José

    2009-09-15

    Kaposi sarcoma (KS) is a multifocal vascular disease with uncertain histogenesis. It is characterized by clinical and histologic polymorphism. The "lymphangioma-like" variant is very uncommon, accounting for less than 5% of all cases. We report the case of a 76-year-old woman, HIV negative, with a 4-year history of classic Kaposi sarcoma treated with cryotherapy who developed new bullous lesions on her lower extremities. Biopsy revealed histologic findings of lymphangioma-like KS (LLKS), together with areas of classic KS; HHV-8 staining was positive. Diagnosis of LLKS was made and the patient was proposed for radiotherapy. The lymphangioma-like Kaposi sarcoma is a rare morphologic expression of KS characterized by dilated and bizarrely shaped vascular channels lined by flattened endothelium permeating the dermis. "Bulla-like" lesions have been considered as a clinical hallmark of this variant. Its histologic appearance suggests a lymphatic origin of KS and it may resemble other vascular tumors. Findings of areas of typical KS and positive staining for HHV-8 may help to make a definitive diagnosis.

  16. Mediastinal mature teratoma with complex rupture into the lung, bronchus and skin: a case report.

    PubMed

    Serraj, Mounia; Lakranbi, Marouane; Ghalimi, Jamal; Ouadnouni, Yassine; Smahi, Mohamed

    2013-06-01

    Mature teratoma is the most common primary germ cell tumor in the mediastinum. On rare occasions, cystic teratomas rupture in adjacent structures, such as pleural space, pericardium, lung or tracheobronchial tree. We present a case of a mediastinal mature cystic teratoma in 16-year-old female with complex rupture into the lung, bronchus and skin. Mature mediastinal teratoma fistulized to the skin has not been previously described.

  17. A rare case of acquired lymphangioma circumscriptum of the penis.

    PubMed

    Adikari, S; Philippidou, M; Samuel, M

    2017-02-01

    Acquired lymphangioma circumscriptum is a rare occurrence on the penis. We report a case of a 47-year-old man who presented with a single lesion of acquired lymphangioma circumscriptum on the penis resembling genital warts. We report the case to increase awareness of this rare condition which may mimic sexually transmitted infections such as genital warts.

  18. Scrotal Lymphangioma – A Rare Cause of Scrotal Swelling

    PubMed Central

    Nain Rattan, Kamal; S Malik, Vivek; Garsa, Vipin

    2013-01-01

    Lymphangioma is an extremely rare cause of scrotal swelling. We are reporting such a tumor in a one and half year old child presenting with a painless, progressive scrotal swelling. The mass was evaluated and excised completely. Histopathology confirmed it as Lymphangioma. PMID:23277887

  19. Intralesional bleomycin and sodium tetradecyl sulphate for haemangiomas and lymphangiomas.

    PubMed

    Harjai, Man Mohan; Jha, Manvendu

    2012-01-01

    To compare the efficacy of intralesional bleomycin and sodium tetradecyl sulphate in treatment of haemangiomas and lymphangiomas. Between July 2007 and May 2009, 120 patients, sixty each of peripheral haemangiomas and lymphangiomas, were administered intralesional injection of bleomycin in a dose of 0.5-1 U/kg in children less than one year of age and 1 to 15 units in children more than one year of age and 1 to 3 ml of 2% sodium tetradecyl sulphate, depending on the size of the lesion at intervals of 14 days. Patients more than 20 years of age and those with diffuse or visceral lesions were excluded from the study. Complete resolution occurred in 16 patients (53%) of haemangiomas and 14 patients (47%) of lymphangiomas treated with bleomycin, while the results were 12 patients (40%) and 10 patients (33%), respectively, in sodium tetradecyl sulphate group. The satisfactory resolution (resolution more than 50%) occurred in eight patients (27%) of haemangiomas and lymphangiomas groups treated with bleomycin, while the results were six patients (20%) and eight patients (27%), respectively, in sodium tetradecyl sulphate group. Poor response rate was observed in six patients (20%) of haemangiomas and eight patients (27%) of lymphangiomas of bleomycin group and 12 patients (40%) of haemangiomas and lymphangiomas in sodium tetradecyl sulphate group. No pulmonary fibrosis or other serious side effects were found. Intralesional bleomycin and sodium tetradecyl sulphate are effective sclerosants in peripheral haemangiomas and lymphangiomas, but bleomycin was found to be more efficacious.

  20. Lymphangioma colli--a new classification contributing to prognosis.

    PubMed

    Schuster, T; Grantzow, R; Nicolai, T

    2003-04-01

    The aim of the study was to introduce a classification of lymphangioma colli, which allows us to predict the expected morbidity and prognosis with surgical treatment. We defined 4 types, dependent on the volume of the lymphangioma as assessed by prenatal ultrasound or clinically: type I included tumors which had no or only a minimal effect on the contour of the neck; type II lymphangiomas were smaller than a line drawn at the lateral border of the head; type III tumors exceeded this line; in type IV the lymphangioma extended beyond the midline of the body. We report on the pre- and postoperative findings in 36 cases operated on between 1988 and 2000, mean follow-up was 4.2 years. 25 % of the cases were classified as type I, 36 % as type II, 22 % as type III, and 17 % as type IV. Cesarean section was carried out for 79 % of type III and IV lymphangiomas following prenatal diagnosis (71 %) and for 14 % of type I and II. MRT was indicated in 38 % of type II compared to 86 % in type III and IV lymphangiomas. Endoscopy was necessary in 25 % of type III and in 66 % of type IV lymphangiomas. In 2 cases (type I and IV), imaging revealed an intrathoracic portion. In 64 % of type I and II cases, resection was complete compared to 21 % in infants with more extended tumors. In all type IV cases, at least one further operation was required, likewise in 25 % of type III lymphangiomas. Postoperative effusion and infection occurred significantly more often in group IV than in groups I to III. 5 nerval lesions remained in children with operated type IV lymphangioma and 2 in type III cases. A visible lack of facial symmetry was evident at the follow-up examination in 83 % of type IV cases and moderate asymmetry was found in 19 % of all type I and II cases. Tracheostomy and gastrostomy were required only in type IV. The introduced classification of lymphangioma colli enables predictive statements to be made concerning the morbidity and prognosis with surgical therapy. The prognosis

  1. Lymphangioma Circumscriptum in an Adult: An Unusual Oral Presentation

    PubMed Central

    Ganesh, C.; Sangeetha, G. S.; Narayanan, Vivek; Umamaheswari, T. N.

    2013-01-01

    Lymphangioma is a benign hamartomatous tumor of lymphatic vessels. This lymphatic malformation is characterized by an abnormal proliferation of lymphatic vessels. Extra-oral lymphangiomas occur more frequently in the neck region predominantly in the posterior triangle, while intra-oral lymphangiomas are commonly seen in the tongue mainly on the dorsum surface. Various imaging modalities such as ultrasound and color Doppler are very useful in viewing the extent of the lesion. In most of the cases, surgical excision is the treatment of choice. The prognosis is good for most patients, but recurrence has also been reported in some cases, presumably because the lesion is interwoven between muscle fibers, preventing complete removal. This case report discusses the clinical features, color Doppler imaging, histopathology, and treatment of lymphangioma. PMID:24228212

  2. Ultrasonography in children of cystic abdominal masses not related to the genitourinary system.

    PubMed

    Milburn, J M; Merritt, C R

    1995-10-01

    A variety of cystic abdominal masses not related to the genitourinary tract may be encountered in the pediatric population. Ultrasound is the most informative imaging modality in the workup of these masses which include lymphangiomas, duplication cysts, enteric cysts, mesothelial cysts, pseudocysts, choledochal cysts, and gastrointestinal teratomas. The clinical and sonographic characteristics of these lesions are presented.

  3. Orbital Lymphangioma: Characteristics and Treatment Outcomes of 12 Cases

    PubMed Central

    Woo, Young Jun; Kim, Chang Yeom; Sgrignoli, Bradford

    2017-01-01

    Purpose To report the patient characteristics and treatment outcomes in 12 cases of orbital lymphangioma. Methods In this study, orbital lymphangioma was diagnosed based on clinical, radiologic (computed tomography, magnetic resonance imaging), and histologic findings when possible. Patients whose vision was not compromised by orbital lymphangioma, or that did not have increased intraocular pressure (IOP), received oral corticosteroids. Orbital lymphangioma that affected vision or increased IOP was treated by surgery, which included aspiration of blood or partial resection with or without injection of a sclerosant. Results Four patients without compromised vision responded well to oral corticosteroids. Eight patients with compromised vision underwent some form of surgery. Bleeding recurred in three patients after aspiration of blood and in two after partial resection and intralesional injection of a sclerosant. Overall, five patients were treated successfully by aspiration of blood, intralesional injection of a sclerosant, and application of continuous negative pressure by appropriate drainage. Partial resection was successful in two patients with organized hematoma. Conclusions Orbital lymphangioma that does not compromise vision can be treated medically using oral corticosteroids. Patients with threatened vision or elevated IOP due to acute hemorrhage should be treated by aspiration of blood, intralesional injection of a sclerosant, and application of continuous negative pressure. Partial resection may be effective only in patients with organized hematoma. PMID:28534344

  4. Orbital Lymphangioma: Characteristics and Treatment Outcomes of 12 Cases.

    PubMed

    Woo, Young Jun; Kim, Chang Yeom; Sgrignoli, Bradford; Yoon, Jin Sook

    2017-06-01

    To report the patient characteristics and treatment outcomes in 12 cases of orbital lymphangioma. In this study, orbital lymphangioma was diagnosed based on clinical, radiologic (computed tomography, magnetic resonance imaging), and histologic findings when possible. Patients whose vision was not compromised by orbital lymphangioma, or that did not have increased intraocular pressure (IOP), received oral corticosteroids. Orbital lymphangioma that affected vision or increased IOP was treated by surgery, which included aspiration of blood or partial resection with or without injection of a sclerosant. Four patients without compromised vision responded well to oral corticosteroids. Eight patients with compromised vision underwent some form of surgery. Bleeding recurred in three patients after aspiration of blood and in two after partial resection and intralesional injection of a sclerosant. Overall, five patients were treated successfully by aspiration of blood, intralesional injection of a sclerosant, and application of continuous negative pressure by appropriate drainage. Partial resection was successful in two patients with organized hematoma. Orbital lymphangioma that does not compromise vision can be treated medically using oral corticosteroids. Patients with threatened vision or elevated IOP due to acute hemorrhage should be treated by aspiration of blood, intralesional injection of a sclerosant, and application of continuous negative pressure. Partial resection may be effective only in patients with organized hematoma.

  5. Orbital lymphangioma: an analysis of 26 patients

    PubMed Central

    Tunc, M.; Sadri, E.; Char, D.

    1999-01-01

    AIM—To evaluate retrospective data on the clinical findings, histological features, radiological diagnosis, and management outcomes in orbital lymphangioma.
METHODS—Data on 26 orbital lymphangioma patients managed over 16 years were re-evaluated. The computed tomograph and magnetic resonance scans and histological slides were reviewed. Parametric techniques were used to assess correlations among clinical, radiological, and histopathological factors.
RESULTS—At presentation proptosis was present in 85%, ptosis in 73%, and restrictive eye movements in 46% of patients. The accuracy of the initial radiology interpretations was 77%. 24 cases required one or more surgeries. The mean follow up was 9.2 years (range 1-14 years). 58% of patients developed recurrences. In cases that recurred, motility restriction was significantly more frequent at initial examination than cases without recurrence (p<0.05). After therapy, 75% of patients were satisfied with their visual function and cosmetic appearance.
CONCLUSIONS—Conservative management of orbital lymphangioma with multiple partial resections may achieve satisfactory results.

 Keywords: orbital lymphangioma PMID:10209440

  6. [An unusual mediastinal opacity].

    PubMed

    Margery, J; Bâ-Fall, K; Rouquet, P; Rique, M; Lefebvre, N; Chevalier, B; Camara, P; Mbaye, P S; Debonne, J-M; Sané, M

    2006-12-01

    A 36-year old immunocompetent male from Senegal with an uneventful history was admitted for exploration of a bullous collection in the posterior mediastinum. Multifocal tuberculosis was diagnosed. Computed tomography-guided drainage removed 600 cc of caseum. The diagnosis as rupture of intrathoracic Pott's abscess complicated by a probably esophageal fistula. The clinical course was rapidly favorable with later development of mediastinal fibrosis. This uncommon case illustrates the contribution of interventional radiology recently developed in the Principal Hospital in Dakar, Senegal.

  7. 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

  8. 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

  9. Fibrosing mediastinitis mimicking bronchogenic carcinoma

    PubMed Central

    Bayiz, Hulya; Mutluay, Neslihan; Koyuncu, Adem; Demirag, Funda; Dagli, Gulfidan; Berktas, Bahadir; Berkoglu, Mine

    2013-01-01

    Fibrosing mediastinitis is a rare but benign disorder characterized by an excessive fibrotic reaction in the mediastinum which can result in compromise of airways, great vessels, and other mediastinal structures. In this paper we presented a patient with fibrosing mediastinitis mimicking bronchogenic carcinoma. The patient was a 32-year-old diabetic male admitting with cough and hemoptysis. There was a right hilar mass and multiple mediastinal conglomerated lymph nodes on chest computed tomography. Positron emission tomography with computed tomography (PET/CT) scan demonstrated increased fluorodeoxyglucose (FDG) uptake at the right hilar mass lesion and mediastinal lymph nodes. Fiberoptic bronchoscopy showed mucosal distortion of right upper lobe. Pathologic examination of the mucosal biopsy revealed inflammation. Endobronchial ultrasound guided transbronchial needle and cervical mediastinoscopic lymph node biopsies were undiagnostic. Diagnostic thoracotomy confirmed the diagnosis fibrosing mediastinitis. Administration of six months of systemic corticosteroid and antituberculous therapy was not beneficial. In conclusion, despite being a rare clinical entity, fibrosing mediastinitis should be kept in mind in the differential diagnosis of mediastinal mass lesions of unknown etiology. The diagnosis is exceptionally difficult in the presence of atypical radiological findings. The treatment is particularly challenging without any proven effective therapy. PMID:23372962

  10. Imaging of Cystic and Cyst-like Lesions of the Mediastinum with Pathologic Correlation

    PubMed Central

    Ödev, Kemal; Arıbaş, Bilgin K.; Nayman, Alaaddin; Arıbaş, Olgun K.; Altınok, Tamer; Küçükapan, Ahmet

    2012-01-01

    Cystic masses of the mediastinum are a heterogenous group of asymptomatic or symptomatic, congenital, infectious, or neoplastic lesions. For early and correct diagnosis, evaluation, and optimal patient management of cystic mediastinal masses in infants, children, or adults imaging plays an important role. A non-invasive and sensitive imaging modality is an efficient and cost-effective tool. Multidetector computed tomography (MDTC) with volumetric acquisition provides fast acquisition of high resolution images and multiplanar reconstruction. Both 2D and 3D imaging in mediastinal imaging help in surgical planning and assessing resectability of mediastinal lesions. MR imaging has many advantages over other modalities for detecting and identifying cystic, or fluid-filled mediastinal masses, because of its intrinsic high soft tissue contrast and direct multiplanar imaging capabilities. However, histological tissue analysis may be required to differentiate a cystic lesion from other cyst-like or low-attenuation lesions. PMID:22919547

  11. 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.

  12. CO2 laser ablation of lymphangioma circumscriptum of the scrotum.

    PubMed

    Treharne, Linda J; Murison, Maxwell S C

    2006-01-01

    Lymphangioma circumscriptum (LC) is an uncommon skin condition characterized by large muscular-coated lymphatic cisterns that lie deep with in the subcutaneous tissue and communicate with dilated dermal lymphatics. Patients suffer from edema and lymphatic leakage. Surgical excision and reconstruction is the gold standard for therapy. However, this can be mutilating. The authors present a patient who suffered widespread disease of his scrotum who had excellent symptomatic relief by treatment with the CO(2) laser.

  13. High-grade squamous intraepithelial lesion arising adjacent to vulvar lymphangioma circumscriptum: a tertiary institutional experience

    PubMed Central

    Bae, Go Eun; Yoon, Gun; Song, Yong Jung; Kim, Hyun-Soo

    2016-01-01

    Lymphangioma circumscriptum of the vulva occurs in patients who have undergone radical hysterectomy, lymph node dissection, or radiation therapy for management of advanced uterine cancer. Since vulvar lymphangioma circumscriptum typically presents as multiple, grossly verrucous vesicles of various sizes, it may be impossible to clinically distinguish vulvar lymphangioma circumscriptum from other vulvoperineal cutaneous diseases. In the present study, 16 (1.6%) out of the 1,024 vulvar biopsy or excision specimens were diagnosed as lymphangioma circumscriptum. In two (12.5%) out of the 16 cases, unusual histopathological findings were observed. Both patients had previously undergone radical hysterectomy with lymph node dissection and postoperative radiation therapy or concurrent chemoradiation therapy for advanced cervical cancer. Microscopic examination revealed high-grade squamous intraepithelial lesions, which were located immediately adjacent to the normal squamous epithelium covering the dilated subepithelial lymphatic vessels. Further, human papillomavirus genotyping confirmed that both patients were infected with high-risk human papillomavirus. High-grade squamous intraepithelial lesion cannot be grossly distinguished from vulvar lymphangioma circumscriptum because the multiple, verrucous vesicles that constitute the characteristic gross appearance of vulvar lymphangioma circumscriptum hinder its distinction. In this regard, our cases of high-grade squamous intraepithelial lesion, located adjacent to vulvar lymphangioma circumscriptum, support the notion that active surgical excision is necessary for the treatment of vulvar lymphangioma circumscriptum. PMID:27329721

  14. 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

  15. [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.

  16. 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

  17. Multicystic benign cystic mesothelioma presenting as a pelvic mass.

    PubMed

    Momeni, Mazdak; Pereira, Elena; Grigoryan, Gennadiy; Zakashansky, Konstantin

    2014-01-01

    Background. Benign cystic mesothelioma (BCM) is a rare tumor that arises from the abdominal peritoneum with a predilection to the pelvic peritoneum. For this reason, it can often mimic gynecologic malignancies. Case. A 47-year-old perimenopausal female presented reporting several weeks of abdominal distention associated with abdominal tenderness and constipation. Computed tomography revealed a 24 cm multiloculated pelvic mass, and tumor markers were notable for an elevated CA-125. The patient was taken to the operating room for an exploratory laparotomy, total abdominal hysterectomy, bilateral salpingoophorectomy, and removal of pelvic mass. Final pathologic evaluation revealed a benign cystic mesothelioma. Conclusion. Classically these tumors present as large multicystic masses with thin-walled septations and on preoperative evaluation BCM can mimic many different disease entities including ovarian malignancies and cystic lymphangioma. Often diagnosis can only be made at time of surgery.

  18. Multicystic Benign Cystic Mesothelioma Presenting as a Pelvic Mass

    PubMed Central

    Pereira, Elena; Zakashansky, Konstantin

    2014-01-01

    Background. Benign cystic mesothelioma (BCM) is a rare tumor that arises from the abdominal peritoneum with a predilection to the pelvic peritoneum. For this reason, it can often mimic gynecologic malignancies. Case. A 47-year-old perimenopausal female presented reporting several weeks of abdominal distention associated with abdominal tenderness and constipation. Computed tomography revealed a 24 cm multiloculated pelvic mass, and tumor markers were notable for an elevated CA-125. The patient was taken to the operating room for an exploratory laparotomy, total abdominal hysterectomy, bilateral salpingoophorectomy, and removal of pelvic mass. Final pathologic evaluation revealed a benign cystic mesothelioma. Conclusion. Classically these tumors present as large multicystic masses with thin-walled septations and on preoperative evaluation BCM can mimic many different disease entities including ovarian malignancies and cystic lymphangioma. Often diagnosis can only be made at time of surgery. PMID:24716035

  19. Diagnostic approach to mediastinal masses.

    PubMed

    Giron, J; Fajadet, P; Sans, N; Jarlaud, T; Verhnet, H; Galy-Fourcade, D; Baunin, C; Durand, G; Sénac, J P; Railhac, J J

    1998-03-01

    Mediastinal masses represent a vast group of tumours and pseudo-tumours which can involve the various compartments of the mediastinum. The authors propose a radiologic diagnostic approach starting from the plain thoracic radiograph with study of the mediastinal lines and oesophageal transit and going on to the classifications made possible by modern CT and MR imaging. The proposed diagnostic procedure is based on nine mediastinal lines and two 'threads of Ariadne' which are the compartments where the masses are located and their behaviour at CT (densitometry before and after administration of an iodinated bolus) and at MRI (T1, T2, gadolinium-enhanced T1-weighted sequences). The definitive aetiological diagnosis may be established by surgery, but also in certain cases by percutaneous needle biopsy.

  20. 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.

  1. Lymphangioma of the Tongue - A Case Report and Review of Literature

    PubMed Central

    V, Usha; Sivasankari, T.; Jeelani, S.; Parthiban, J.

    2014-01-01

    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

  2. Conjunctival Lymphangioma: A Case Report and Brief Review of the Literature

    PubMed Central

    Seca, Mariana; Borges, Pedro; Reimão, Pedro; Gomes, Miguel; Meireles, Angelina

    2012-01-01

    Background. Lymphangioma is a rare venolymphatic lesion, characterized by dilation of lymphatic vessels. It may occur as an isolated lesion or, more often, represent the surface component of a deep orbital lymphangioma. Case. We report a case of a conjunctival lymphangioma on a 58-year-old male that had simultaneously an upper respiratory tract infection. Excision and biopsy confirmed the nature of the lesion, and there has been no relapse to date. Conclusion. Conjunctival lymphangioma is a rare condition in which the diagnose, must be kept in mind in patients with a red eye resistance to topical therapy and in association with an upper respiratory tract infection. Finally, it is also necessary to be aware of possible recurrence of the lesion. PMID:22666617

  3. Vulvar lymphangioma circumscriptum: a rare complication of therapy for squamous cell carcinoma of the cervix

    SciTech Connect

    LaPolla, J.; Foucar, E.; Leshin, B.; Whitaker, D.; Anderson, B.

    1985-11-01

    The clinical and pathological features of a case of multifocal lymphangioma circumscriptum of the vulva are reported in a patient with chronic lymphedema of a lower extremity. Ten years previously the patient had been treated for squamous cell carcinoma of the cervix. Although lymphangioma circumscriptum is an extremely rare complication of altered lymphatic drainage, the presence of multiple noninflammatory vesicular appearing lesions in this setting should suggest the correct diagnosis.

  4. One-stage excision of massive cervicomediastinal lymphangioma in the newborn.

    PubMed

    Cozzi, Denis A; Zani, Augusto; d'Ambrosio, Giuseppe; Piserà, Antonietta; Roggini, Mario; Cozzi, Francesco

    2007-09-01

    We report the first neonate successfully treated with one-stage complete excision of massive cervicomediastinal lymphangioma producing alarming respiratory distress. Early postoperative course was complicated by refractory bilateral chylothorax, which effectively responded to insertion of external pleuroperitoneal shunts. At the 6-year follow-up, the child remains symptom free and recurrence free. One-stage resection for cervicomediastinal lymphangioma is curative and well tolerated even shortly after birth.

  5. Combined mediastinal and retroperitoneal fibrosis

    PubMed Central

    Salmon, H. W.

    1968-01-01

    A case of combined idiopathic mediastinal fibrosis and retroperitoneal fibrosis is described. It is possibly the twelfth case to be reported during life. A review of the literature reveals the `ubiquity' of localized collagenosis and the trend of opinion as regards aetiology and treatment. Images PMID:5654073

  6. Post extubation negative pressure pulmonary edema due to posterior mediastinal cyst in an infant.

    PubMed

    Dubey, Prakash Kumar

    2014-01-01

    A 3-month-old male child underwent uneventful inguinal herniotomy under general anesthesia. After extubation, airway obstruction followed by pulmonary edema appeared for which the baby was reintubated and ventilated. The baby made a complete recovery and extubated after about 2 h. A post-operative computed tomography scan revealed a posterior mediastinal cystic mass abutting the tracheal bifurcation. Presumably, extrinsic compression by the mass on the tracheal bifurcation led to the development of negative pressure pulmonary edema.

  7. PSMA Uptake in Mediastinal Sarcoidosis.

    PubMed

    Ardies, Philip Junior; Gykiere, Pieterjan; Goethals, Lode; De Mey, Johan; De Geeter, Frank; Everaert, Hendrik

    2017-04-01

    Prostate-specific membrane antigen (PSMA) is a cell surface glycoprotein which is frequently overexpressed on prostate cancer cells. Ga-PSMA PET/CT plays an increasing role in prostate cancer management. However, growing evidence suggests increased PSMA uptake in a variety of other malignant tumor entities and in some benign lesions. This report describes PSMA uptake in numerous thoracic lymph nodes in a patient with known mediastinal sarcoidosis. Knowledge and recognition of these possibilities are important to avoid scan misinterpretation.

  8. Mediastinal granuloma: a rare cause of dysphagia.

    PubMed

    Micic, Dejan; Hogarth, Douglas Kyle; Kavitt, Robert T

    2016-06-14

    Dysphagia is commonly attributed to disorders arising from dysfunction of the oesophageal mucosa or oesophageal motility. Mediastinal structures causing compression of the oesophagus remain a rare presenting cause of dysphagia. We report a case of a woman presenting with dysphagia to solid foods and associated symptoms of weight loss. Traditional evaluation for dysphagia was unrevealing until cross-sectional imaging suggested a mediastinal obstructive process. The finding of a mediastinal granuloma, distinct from mediastinal fibrosis, as the aetiology of dysphagia is a rare finding, with specific treatment implications. The patient was treated with itraconazole antifungal therapy with an improvement in her symptoms.

  9. Lack of effect of propranolol in the treatment of lymphangioma in two children.

    PubMed

    Maruani, Annabel; Brown, Shanna; Lorette, Gerard; Pondaven-Letourmy, Soizick; Herbreteau, Denis; Eisenbaum, Allan

    2013-01-01

    Vascular lesions such as hemangiomas and lymphangiomas can cause significant mortality and morbidity, as well as amblyopia when located in the orbit. Oral propranolol can regress infantile hemangioma during infancy and up to 23 months of age, but its effect on lymphangioma has not been demonstrated. We present two cases of lymphatic malformations treated with oral propranolol. Patient 1 is a 2-year-old boy with macrocystic bilateral cervical lymphangioma extending to the pharynx and larynx and microcystic lymphangioma of the tongue. The patient was started on propranolol 2 mg/kg/day starting at 17 months of age, and after 3 months only a very slight decrease in tongue volume was noted. Patient 2 is a 3.5-year-old boy with magnetic resonance imaging evidence of right facial complex lymphangioma with venous malformation. The patient was placed on oral propranolol 2 mg/kg/day. After 3 months of treatment, no change in the lesion was noted except for a transient decrease in the size of the conjunctival telangiectasia. Propranolol 2 mg/kg/day was not effective in treating lymphatic malformations in two children, both older than 17 months at the time of treatment. © 2012 Wiley Periodicals, Inc.

  10. Postoperative radiotherapy for residual tumor of primary mediastinal carcinoid teratoma

    PubMed Central

    Tu, Lingli; Sun, Lan; Zhou, Yu; Gong, Youling; Xue, Jianxin; Gao, Jun; Lu, You

    2013-01-01

    A 36-year-old woman had presented with dry cough for 2 months. Thoracic computed tomography (CT) scan showed a 12 cm × 8 cm ×5 cm mass in the anterior mediastinum. Due to intimately involving the aortic arch, tumor was removed incompletely. Residual tumor remained approximate 2 cm × 3 cm × 4 cm. Histologic diagnosis was a mature cystic teratoma containing a carcinoid. Subsequently, radiotherapy (RT) was administrated on residual tumor for a total dose of 50 Gy at 2 Gy/d fraction in 25 fractions. At 2-year follow-up, the patient had stable disease. In conclusion, adjuvant radiotherapy with 50 Gy is an effective approach for residual tumor of mediastinal carcinoid teratoma. PMID:23585961

  11. Optimal treatment of descending necrotising mediastinitis

    PubMed Central

    Corsten, M. J.; Shamji, F. M.; Odell, P. F.; Frederico, J. A.; Laframboise, G. G.; Reid, K. R.; Vallieres, E.; Matzinger, F.

    1997-01-01

    BACKGROUND: Descending necrotising mediastinitis is caused by downward spread of neck infection and has a high fatality rate of 31%. The seriousness of this infection is caused by the absence of barriers in the contiguous fascial planes of neck and mediastinum. METHODS: The recent successful treatment of seven adult patients with descending necrotising mediastinitis emphasises the importance of optimal early drainage of both neck and mediastinum and prolonged antibiotic therapy. The case is also presented of a child with descending necrotising mediastinitis, demonstrating the rapidity with which the infection can develop and lead to death. Twenty four case reports and 12 series of adult patients with descending necrotising mediastinitis published since 1970 were reviewed with meta-analysis. In each case of confirmed descending necrotising mediastinitis the method of surgical drainage (cervical, mediastinal, or none) and the survival outcome (discharge home or death) were noted. The chi 2 test of statistical significance was used to detect a difference between cases treated with cervical drainage alone and cases where mediastinal drainage was added. RESULTS: Cervical drainage alone was often insufficient to control the infection with a fatality rate of 47% compared with 19% when mediastinal drainage was added (p < 0.05). CONCLUSIONS: Early combined drainage with neck and chest incisions, together with broad spectrum intravenous antibiotics, should be considered standard care for this disease. 


 PMID:9337829

  12. Huge Lymphangioma of the Esophagus Resected by Endoscopic Piecemeal Mucosal Resection.

    PubMed

    Luo, Dinghai; Ye, Liping; Wu, Weidan; Zheng, Haihong; Mao, Xinli

    2017-01-01

    We present an unusual case of a 41-year-old male patient with a large lymphangioma of the esophagus. Endoscopy revealed that the structure measured 60 × 10 mm in the mucosa and the submucosa and had a heterogenous echo pattern. The esophageal mass was successfully resected by endoscopic piecemeal mucosal resection. However, most esophageal lymphangiomas that are larger than 2 cm in diameter reported in the literature can be removed only through open surgery. Thus far, we know of no reported cases of endoscopic resection as a treatment for this case.

  13. Huge Lymphangioma of the Esophagus Resected by Endoscopic Piecemeal Mucosal Resection

    PubMed Central

    Ye, Liping; Wu, Weidan; Zheng, Haihong

    2017-01-01

    We present an unusual case of a 41-year-old male patient with a large lymphangioma of the esophagus. Endoscopy revealed that the structure measured 60 × 10 mm in the mucosa and the submucosa and had a heterogenous echo pattern. The esophageal mass was successfully resected by endoscopic piecemeal mucosal resection. However, most esophageal lymphangiomas that are larger than 2 cm in diameter reported in the literature can be removed only through open surgery. Thus far, we know of no reported cases of endoscopic resection as a treatment for this case. PMID:28408932

  14. Klippel-Trenaunay syndrome with hemimegalencephaly, retroperitoneal lymphangioma and double inferior vena cava.

    PubMed

    Vurucu, S; Battal, B; Kocaoglu, M; Akin, R

    2009-05-01

    Klippel-Trenaunay syndrome (KTS) is a rare disorder characterised by congenital vascular hamartomas, limb hypertrophy, lymphangiomas and atresia of lymph vessels with non-pitting oedema. A 6-year-old girl with KTS was referred to our hospital for evaluation of intractable seizures. In addition to findings consistent with KTS, we also found hemimegalencephaly, retroperitoneal lymphangioma and double inferior vena cava. All of these associations in the same patient with KTS are unique in the English literature. We report on the multidedector CT and MRI features of such an unusual case.

  15. 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. ...

  16. Cystic hygroma

    MedlinePlus

    ... white blood cells. This material is called embryonic lymphatic tissue. After birth, a cystic hygroma most often ... 198. Tower RL II, Camitta BM. Abnormalities of lymphatic vessels. In: Kliegman RM, Stanton BF, St Geme ...

  17. 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- ...

  18. Mediastinitis

    MedlinePlus

    ... and Metabolic Disorders Immune Disorders Infections Injuries and Poisoning Kidney and Urinary Tract Disorders Liver and Gallbladder ... and Metabolic Disorders Immune Disorders Infections Injuries and Poisoning Kidney and Urinary Tract Disorders Liver and Gallbladder ...

  19. Mediastinitis

    MedlinePlus

    ... nodes, lungs, liver, eyes, skin, or other tissues ( sarcoidosis ) Tuberculosis Breathing in anthrax Cancer Risk factors include: ... breath If you have a lung infection or sarcoidosis and develop any of these symptoms, see your ...

  20. [Giant compressive mediastinal lipoma: a case report].

    PubMed

    Ndiaye, As; Diarra, O; Diop, A N; Ciss, A G; Dieng, P A; Dangou, J M; Ndiaye, M

    2006-01-01

    Mediastinal lipoma is a rare mesenchymatous fatty tumor in child. Usually asymptomatic, it can cause asphyxiation. The authors report the case of an 18 month-baby-girl referred to us by the pediatric department for asphyxiation. The chest X ray was evocative of a mediastinal tumor. A sudden cardiorespiratory failure leads us to perform thoracotomy. A fatty tumor was found compressing the heart and the left lung. It was easily extirpated. The postoperative recovery was uneventful. Light microscopy showed a lipoma. This compressive form shows the severity of the mediastinal lipoma when it reaches a large size. The literature on this rare pathology is reviewed.

  1. 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

  2. Ascending necrotizing mediastinitis secondary to emphysematous pyelonephritis.

    PubMed

    Dajer-Fadel, Walid Leonardo; Pichardo-González, Martha; Estrada-Ramos, Sandra; Palafox, Damián; Navarro-Reynoso, Francisco Pascual; Argüero-Sánchez, Rubén

    2014-09-01

    Mediastinal infections usually originate from postoperative complications or in a descending manner from a cervical infectious process; few reports have emerged describing an ascending trajectory. A 56-year-old woman with a Huang class 1 left emphysematous pyelonephritis was referred due to a progression of an ascending necrotizing mediastinitis. A left posterolateral thoracotomy was performed, drainage and thorough lavage were carried out with a successful outcome. We believe this is the first reported case of ascending necrotizing mediastinitis secondary to an emphysematous renal infection. © The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  3. A case of HPV and acquired genital lymphangioma: over-lapping clinical features.

    PubMed

    Cestaro, Giovanni; De Rosa, Michele; Gentile, Maurizio; Massa, Salvatore

    2015-03-25

    Lymphatic malformation or lymphangioma is a benign proliferation of the lymphatics accounting for 4% of all vascular malformations and 26% of all benign vascular tumors. There are several reports about genital lymphangiomas mimicking venereal lesions, such as genital warts. Hereby we described a case of a 24 year old man affected by multiple vesicles and warts in genital area. All hematological and biochemical parameters, Human Immunodeficiency Virus (HIV) and Treponema Pallidum tests, C1-Inhibitor and C1-Q values were within limits. An accurate fulguration and wide excision of bigger lesions were performed. Histological examination showed numerous dilated lymphatic vessels in the superficial dermis with infiltration of inflammatory cells, that is a histopathological picture compatible with genital lymphangioma. Considering our clinical suspicion of condylomatosis, HPV (Human Papilloma Virus) Polimerase Chain Reaction (PCR) Genotyping, named INNOLiPA test, was performed, that revealed a genital infection by HPV - genotype 6. We think that our case can be considered an example of HPV infection and acquired genital lymphangioma overlap clinical syndrome. The patient presented any lesions one year after the procedure at follow-up examination.

  4. Klippel-Trenaunay syndrome presenting with acanthocytosis and splenic and retroperitoneal lymphangioma: a case report.

    PubMed

    Withana, Milinda; Rodrigo, Chaturaka; Shivanthan, Mitrakrishnan Chrishan; Warnakulasooriya, Sachini; Wimalachandra, Manu; Gooneratne, Lallindra; Rajapakse, Senaka

    2014-11-27

    Klippel-Trenaunay syndrome is a rare congenital mesodermal abnormality characterized by bone and soft tissue hypertrophy, extensive hemangioma and venous abnormalities. We report the case of a patient with two additional rare clinical manifestations in the background of Klippel-Trenaunay syndrome, namely, acanthocytosis and splenic and retroperitoneal lymphangioma. A 24-year-old Sri Lankan man from North Central Province in Sri Lanka presented to our general medical unit with symptomatic anaemia. He had been diagnosed with Klippel-Trenaunay syndrome at the age of six years, with hemihypertrophy of his right lower limb and strawberry naevi over both lower limbs. His blood film results were positive for acanthocytes, which accounted for more than 20% of the red blood cell population. He was also found to have extensive splenic lymphangiomas and a large retroperitoneal lymphangioma encasing the mesentric vessels in the right para-aortic region. An extensive battery of tests to identify a secondary cause for the acanthocytosis failed to show any positive results. Retroperitoneal lymphangioma has been reported in association with Klippel-Trenaunay syndrome once before, but an association with acanthocytosis has never been reported. Given the rarity of all three conditions this is not surprising. The cause of acanthocytosis in this setting is currently unresolved. It is plausible that this may be a primary association with Klippel-Trenaunay syndrome, as an alternative aetiology was not found.

  5. Jejunal lymphangioma: an unusual cause of intussusception in an adult patient.

    PubMed

    Limaiem, F; Khalfallah, T; Marsaoui, L; Bouraoui, S; Lahmar, A; Mzabi, S

    2015-03-01

    Adult intussusception is a relatively rare clinical entity. Almost 90% of cases of intussusception in adults are secondary to a pathologic condition that serves as a lead point. Lymphangioma of the small bowel is an unusual tumour that has been rarely reported to cause intussusception. In this paper, we present a rare case of adult intussusception due to jejunal lymphangioma. A 22-year-old female patient with a medical history significant for anaemia presented with intermittent colicky abdominal pain, diarrhoea and oedema of the inferior limbs for the past three months. Ultrasonography and CT scan revealed a typical target sign with dilated intestinal loops. At laparotomy, a jejuno-jejunal intussusception was found. Partial resection of the jejunum was performed. Macroscopic examination of the surgical specimen revealed a pedunculated polyp measuring 2 cm in diameter. Histological sections of the polyp revealed in the lamina propria and submucosal layer of the jejunum several markedly dilated thin-walled lymphatic spaces lined with single layers of flat endothelial cells. The final pathologic diagnosis was submucosal lymphangioma. This case report indicates that intussusception, although rare in adults, should be considered in the differential diagnosis of abdominal pain. Moreover, it should be taken into consideration that lymphangioma is one of the possible lesions that can cause intussusception.

  6. Endoscopic ultrasound in the diagnosis of mediastinal diseases

    PubMed Central

    Wang, Zhiguo; Jiang, Chunmeng

    2015-01-01

    EUS is a useful tool for diagnosis of mediastinal diseases. EUS-FNA plays an important role in staging of lung cancer and in tissue acquisition in patients with mediastinal masses. In this review, the following issues will be addressed: EUS-FNA and EBUS-TBNA, metastatic mediastinal lymph nodes diagnosed by EUS, EUS in assessment of mediastinal lymph node status for staging of lung cancer, mediastinal lymphoma diagnosed by EUS, sarcoidosis and tuberculosis diagnosed by EUS. PMID:28352755

  7. Pyopneumopericardium caused by mediastinal granuloma.

    PubMed

    Gula, Lorne J; Malthaner, Richard A; Quantz, Mackenzie A

    2002-07-01

    We report the case of a previously healthy 32-year-old man who was seen with flulike symptoms, dyspnea, and chest pain. The diagnosis was pyopneumopericardium, and pericardial tap revealed 1.3 L of purulent material. Computed tomography of the chest demonstrated a calcified mass inferior to the carina. Urgent exploration through a right thoracotomy revealed that the mass was adherent to the esophagus and pericardium. The subcarinal mass was resected. Pathological study demonstrated granulomatous lymph nodes, which were likely due to histoplasmosis. This is among the first reports of granulomatous erosion into the pericardium causing pyopneumopericardium. The patient made a good recovery, and his case demonstrates the importance of early imaging and mediastinal exploration for pyopneumopericardium.

  8. Cystic fibrosis.

    PubMed

    Ratjen, Felix; Bell, Scott C; Rowe, Steven M; Goss, Christopher H; Quittner, Alexandra L; Bush, Andrew

    2015-05-14

    Cystic fibrosis is an autosomal recessive, monogenetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The gene defect was first described 25 years ago and much progress has been made since then in our understanding of how CFTR mutations cause disease and how this can be addressed therapeutically. CFTR is a transmembrane protein that transports ions across the surface of epithelial cells. CFTR dysfunction affects many organs; however, lung disease is responsible for the vast majority of morbidity and mortality in patients with cystic fibrosis. Prenatal diagnostics, newborn screening and new treatment algorithms are changing the incidence and the prevalence of the disease. Until recently, the standard of care in cystic fibrosis treatment focused on preventing and treating complications of the disease; now, novel treatment strategies directly targeting the ion channel abnormality are becoming available and it will be important to evaluate how these treatments affect disease progression and the quality of life of patients. In this Primer, we summarize the current knowledge, and provide an outlook on how cystic fibrosis clinical care and research will be affected by new knowledge and therapeutic options in the near future. For an illustrated summary of this Primer, visit: http://go.nature.com/4VrefN.

  9. Primary closure for postoperative mediastinitis in children.

    PubMed

    Ohye, Richard G; Maniker, Robert B; Graves, Holly L; Devaney, Eric J; Bove, Edward L

    2004-09-01

    Mediastinitis affects approximately 1% of children undergoing median sternotomy. Conventional therapy involves debridement followed by open wound care with delayed closure, days to weeks of closed suction or antimicrobial irrigation, and vacuum-assisted closure or muscle flap closure. We hypothesized that primary closure without prolonged suction or irrigation is an effective, less traumatic treatment for mediastinitis in children. From January 1986 to July 2002, 6705 procedures involving median sternotomy were performed at the C. S. Mott Children's Hospital, resulting in 57 cases of mediastinitis (0.85%). Cases were divided into 2 groups, with 42 cases treated with primary closure and 15 cases treated with delayed or muscle flap closure. The 42 cases of primary closure comprised the primary study group of this institutional review board-approved, retrospective analysis. Patient demographics, surgical variables, mediastinitis-related parameters, and outcomes were evaluated. One patient had recurrent mediastinitis for an overall infection eradication rate of 97% (40/41). Three patients (7%) required re-exploration for suspected ongoing infection. Of these re-explorations, 1 patient had evidence of continued mediastinitis. The remaining 2 patients with sepsis of unclear cause had no clinical or culture evidence of recurrent infection. One of these patients ultimately died of sepsis without active mediastinitis for a hospital survival of 97% (41/42). No significant differences could be detected between the treatment successes and failures in this small cohort of patients. Simple primary closure is an effective means to treat selected cases of postoperative mediastinitis in children. The results compare favorably with other more lengthy or debilitating treatments.

  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. [Clinical characteristics and prognosis of mediastinal fibrosis].

    PubMed

    Liao, J P; Hu, Y; Qiu, J X; Jin, Z; Zhang, H; Ma, J; Wang, G F

    2017-03-12

    Objective: To investigate the clinical characteristics and prognosis of mediastinal fibrosis. Methods: Twelve patients with mediastinal fibrosis diagnosed between 2008 and 2015 in our hospital were studied retrospectively. Clinical manifestations, radiological characteristics, endoscopic features, treatment and prognosis were analyzed. Results: There were 3 males and 9 females, with a mean age of 68.8 years.Six patients had previous tuberculosis infection. The most common clinical symptoms were dyspnea on exertion (11 cases), cough (7 cases), and wheezing (6 cases). Chest CT scans revealed an infiltrative mediastinal process, with a discrete mass, enlargement of mediastinal lymph nodes, mediastinal lymph node calcification (9 case). Twelve patients had bronchial and pulmonary artery compression at lobar or segmental levels, 7 cases had localized pulmonary edema, and 6 cases had pulmonary atelectasis. The principal findings of bronchoscopy were distortion of bronchus with stenosis, multiple pigmentation of bronchial mucosa, and bronchial mucosal edema. Pulmonary hypertension (PH) was the main severe complication. One patients suffered from sudden death after bronchoscopy. Eleven patients were followed for 3 month to 7 years, and 5 patients got progression. Anti-tuberculosis therapy with or without corticosteroid was not beneficial. Conclusion: Tuberculosis was the leading cause of mediastinal fibrosis in our study, which was characterized with diffuse bronchial and pulmonary artery compression at lobar or segmental levels, and multiple pigmentation of bronchial mucosa.Anti-tuberculosis therapy with or without corticosteroids was not beneficial.

  13. Prenatal diagnosis of a giant foetal lymphangioma and haemangiolymphoma in the second trimester using 2D and 3D ultrasound.

    PubMed

    Mittermayer, C; Blaicher, W; Deutinger, J; Bernaschek, G; Lee, A

    2003-12-01

    Lymphangiomas are benign tumours of the lymphatic system. Early prenatal diagnosis is important to permit a planned delivery and provide adequate postnatal care. It thereby improves prognosis and allows the option of terminating the pregnancy if poor outcome is predicted. We report two cases, a giant haemangiolymphoma and a lymphangioma. 2D and 3D US findings are presented and differential diagnosis, therapeutic options and prognosis are discussed.

  14. Cystic fibrosis

    MedlinePlus

    ... cannot be prevented. Screening those with a family history of the disease may detect the CF gene in many carriers. Alternative ... FJ. Cystic fibrosis. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap ...

  15. Mediastinal pathology and the contributions of Dr. Juan Rosai.

    PubMed

    Wick, Mark R

    2016-09-01

    Dr. Juan Rosai is one of the most prolific contributors to the literature on mediastinal pathology, and he has added steadily to that body of work over a 50-year period. Rosai has written several landmark articles in this topical area, including articles on thymic epithelial lesions, mediastinal neuroendocrine tumors, mediastinal lymphoma and other hematopoietic lesions, thymolipoma, thymoliposarcoma, mediastinal solitary fibrous tumor, intrathymic langerhans-cell histiocytosis, mediastinal germ cell neoplasms, and multilocular thymic cyst. This review recounts his role as one of the principal figures in the surgical pathology of mediastinal diseases. Copyright © 2016 Elsevier Inc. All rights reserved.

  16. 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.

  17. Mediastinal evaluation utilizing the reverse Trendelenburg radiograph.

    PubMed

    Barker, D E; Crabtree, J D; White, J E; Somberg, L B; Burns, R P

    1999-05-01

    When thoracic aortic rupture is suspected, a 45-degree reverse Trendelenburg (RT) anteroposterior (AP) chest radiograph should place the mediastinal structures in a more appropriate position and allow a more accurate evaluation than a supine AP radiograph. One hundred ninety-one consecutive hemodynamically stable adult patients with major blunt thoracic trauma were initially evaluated for mediastinal abnormalities associated with aortic disruption by both supine AP chest radiograph and an AP chest radiograph with the patient in 45-degree RT position. One hundred four patients underwent contrast aortography based on mediastinal abnormalities detected on the supine AP chest radiograph. Twenty of these patients had abnormal aortograms demonstrating traumatic aortic disruption confirmed at surgery. Supine and RT chest radiographs were retrospectively compared in a blinded fashion to evaluate their specificity and positive predictive value for detection of traumatic thoracic aortic rupture. If RT chest radiographic findings had been used to determine the need for further assessment, 29 angiograms (26%) would have been eliminated, specificity would have increased from 52 per cent to 69 per cent, and positive predictive value would have increased from 19 per cent to 27 per cent. Both supine and RT chest radiographs demonstrated mediastinal widening in all 20 patients with abnormal aortograms, with no missed thoracic aortic disruptions (100% sensitivity). This study indicated that the RT chest radiograph may be used instead of the standard supine radiograph as the initial screen for mediastinal evaluation, maintaining a high sensitivity and eliminating the cost and morbidity of many unnecessary aortograms.

  18. What Causes Cystic Fibrosis?

    MedlinePlus

    ... page from the NHLBI on Twitter. What Causes Cystic Fibrosis? A defect in the CFTR gene causes cystic ... in the severity of the disease. How Is Cystic Fibrosis Inherited? Every person inherits two CFTR genes—one ...

  19. Combined mesothelial cyst and lymphangioma of the small bowel: a distinct hybrid intra-abdominal cyst.

    PubMed

    Wang, Jayson; Fisher, Cyril; Thway, Khin

    2014-09-01

    Intra-abdominal cysts have a variety of origins, of which lymphatic and mesothelial types are the most commonly encountered. Here we describe a combined mesothelial cyst and lymphangioma arising within the small bowel subserosa of an 80-year-old woman. This was found incidentally at laparotomy performed for an unrelated condition. To date, such a hybrid lesion has not been previously reported. The ways by which this lesion might have arisen are discussed. © The Author(s) 2013.

  20. 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

  1. Extramedullary plasmacytoma presenting as a mediastinal mass.

    PubMed

    Shukla, Arpana; Bansal, Vivek; Bhutani, Ritu; Kumar, Gaurav; Sharma, Jai; Solanki, Surinder; Mehta, Anurag

    2011-09-05

    Extramedullary plasmacytoma (EMP) is a plasma cell neoplasm without bone marrow involvement or other systemic characteristics of multiple myeloma. Few large-scale clinical studies have been conducted because of the rarity of EMP, especially when it arises from the mediastinum. Herein we report a rare case of solitary mediastinal plasmacytoma with reactive pleural effusion. A 58-year-old female presented with grade 4 dyspnea and dysphagia, with a mediastinal mass observed with on PET. CT-guided biopsy results were suggestive of IgG kappa-type EMP arising from the anterior mediastinum. The patient was treated with local radiotherapy to the mediastinum, and had clinical and radiological response s were good. Radiotherapy is an effective treatment for mediastinal EMP, but a complete workup is mandatory, including PET, as the majority of such masses coexist with multiple myeloma.

  2. [Primary mediastinal seminoma. Report of three cases].

    PubMed

    Abid, Leïla; Ayadi-Kaddour, Aïda; Braham, Emna; Smati, Belhassen; Ismail, Olfa; Kilani, Tarek; El Mezni, Faouzi

    2007-01-01

    Primary mediastinal seminomas (PMS) are rare tumors that are morphologically similar to their testicular counterparts but may have different biologic behavior due to their particular anatomical location. New cases report of PMS CASES: Three new cases of primary mediastinal seminoma are presented. The patients were men aged of 16, 33 and 47 years. Their clinical symptoms included cough, dyspnea, chest pain and superior vena cava syndrome. None of the patients had a previous history of testicular neoplasm or tumor elsewhere. Mediastinoscopic biopsy was performed and histological examination results revealed a seminoma. Immunohistochemical showed membranous staining with placental alkaline phosphatase in the three cases. Chemotherapy has largely replaced surgical resection and radiotherapy as the initial treatment in patients with mediastinal seminoma.

  3. Diagnostic imaging in mediastinal thyroid tumor

    SciTech Connect

    Shih, W.J.; Cho, S.R.; Purcell, M.; Tsung-Yao, H.; Domstad, P.A.; Liu, C.I.; DeLand, F.H.

    1984-12-01

    Various diagnostic imagings in nine patients with mediastinal goiters were presented. The clinical manifestations of these patients were various, from totally asymptomatic to severe dyspnea. Six of the nine patients underwent surgical intervention, three were follicular adenomas and three were nodular goiters. A chest radiograph (positive in seven out of nine patients) provided the most valuable initial localization of a goiter mass to the anterior, middle, or posterior compartment. Esophagograms (performed in four patients) showed compression of esophagus by the mediastinal mass. I-131 scintigraphy (performed in seven patients) was capable of detection of functional (in three patients) vs nonfunctional status of thyroid status (in four patients). Angiography (performed in five patients), characterized by anatomic continuity with cervical thyroid gland, calcifications, well-defined border of masses and/or contrast enhancement, offered important roles to direct a diagnosis of intrathoracic goiter. The computed tomography becomes increasingly important because all mediastinal goiters are not radioiodine avid.

  4. Adult intussusception secondary to lymphangioma of the cecum: a case report.

    PubMed

    Chen, Chin-Fan; Chuang, Chieh-Han; Lu, Chien-Yu; Hu, Ching; Kuo, Ting-Lu; Hsieh, Jan-Sing

    2009-06-01

    We report the case of a patient with ileocolic intussusception caused by cecal lymphangioma. A 45-year-old man visited our hospital with a 2-month history of frequent episodes of watery diarrhea (>/= 5 times/day) and intermittent abdominal pain. A cecal submucosal tumor with mucosal ulceration and partial obstruction of the colonic lumen was identified by colonoscopy. He was admitted to our hospital 2 days later due to aggravation of his abdominal pain. Physical examination revealed tenderness over the right abdomen with no peritoneal signs. A double-contrast lower gastrointestinal series showed a right-side colonic lesion with indentation and a peripheral, beak-like sign. Abdominal computed tomography scanning revealed an intra-abdominal mass with the characteristic sausage sign, highly suggestive of intussusception. The patient subsequently underwent right hemicolectomy. The final diagnosis was ileocolic intussusception with cecal lymphangioma, which was confirmed by histopathology. He had an uneventful recovery with follow-up in our hospital. This case highlights the possibility of colonic lymphangioma as the leading point of adult intussusception, and this should be taken into consideration as a possible diagnosis in this uncommon clinical condition.

  5. Cryotherapy for massive vulvar lymphatic leakage complicated with lymphangiomas following gynecological cancer treatment.

    PubMed

    Yanazume, Shintaro; Douzono, Haruhiko; Kubo, Hidemichi; Nagata, Tomomi; Douchi, Tsutomu; Kobayashi, Hiroaki

    2014-11-01

    Vulvar lymphatic leakage is a severe complication associated with gynecological cancer treatments. However, standard treatment strategies have not yet been determined. We encountered a rare case of a 76-year-old multiparous woman suffering from massive lymphatic fluid leakage from the entire vulva, and papules developed and were identified as lymphangiomas. A large amount of straw-colored discharge continued from all vulvar papules, which extended over the mons pubis. Nine years ago, the patient had undergone a radical hysterectomy with concurrent chemoradiation for uterine cervical cancer treatment. Her serum albumin level was 1.9 mg/dl, which was attributed to the loss of a large amount of lymph fluid due to leakage from the vulva. Her quality of life gradually decreased because of general fatigue and the need for frequent diaper exchanges every 2 h. The patient received a less-invasive treatment with cryotherapy using liquid nitrogen. She also received a multimodality treatment consisting of the intravenous administration of albumin, massage of the lower limbs and intensive rehabilitation. Cryotherapy was administered once a week for 3 months. Her discharge almost stopped and vulvar lymphangiomas decreased without any major complications. To the best of our knowledge, this is the first case report of massive lymphatic leakage complicated with vulvar lymphangiomas. Additionally, this case may represent the first successful treatment of vulva lymph leakage by cryotherapy without recurrence. Cryotherapy may have the potential to improve the quality of life as a less-invasive treatment for gynecological cancer survivors without serious complications.

  6. Cryptogenic organising pneumonia presenting with bilateral hilar and mediastinal lymphadenopathy

    PubMed Central

    Kahraman, Hasan; Tokur, Mahmut; Sayar, Hamide; Inci, Mehmet Fatih

    2013-01-01

    Cryptogenic organising pneumonia is not considered in the differential diagnosis of bilateral hilar and mediastinal lymphadenopathy. We submitted a patient presenting with bilateral hilar and mediastinal lymphadenopathy. We suspected diagnosis of sarcoidosis, but the patient was diagnosed as cryptogenic organising pneumonia with the histological result. This is the second case report of cryptogenic organising pneumonia presenting with bilateral hilar and mediastinal lymphadenopathy. PMID:23761506

  7. Cryptogenic organising pneumonia presenting with bilateral hilar and mediastinal lymphadenopathy.

    PubMed

    Kahraman, Hasan; Tokur, Mahmut; Sayar, Hamide; Inci, Mehmet Fatih

    2013-06-10

    Cryptogenic organising pneumonia is not considered in the differential diagnosis of bilateral hilar and mediastinal lymphadenopathy. We submitted a patient presenting with bilateral hilar and mediastinal lymphadenopathy. We suspected diagnosis of sarcoidosis, but the patient was diagnosed as cryptogenic organising pneumonia with the histological result. This is the second case report of cryptogenic organising pneumonia presenting with bilateral hilar and mediastinal lymphadenopathy.

  8. Angiographic and scintigraphic findings in fibrosing mediastinitis

    SciTech Connect

    Moreno, A.J.; Weismann, I.; Billingsley, J.L.; Lundy, M.N.; Brown, J.M.; Graham, G.D.; Brown, T.J.

    1983-04-01

    The clinical and morphologic findings in the case of a 47-year-old man with fibrosing mediastinitis, most probably due to histoplasmosis, are described. Radionuclide angiography demonstrated obstruction of the superior vena cava with collateral vascularization. Computed tomography demonstrated a large calcific mass interposed between the pulmonary artery and superior vena cava suggesting potential pulmonary vasculature involvement. For this reason, pulmonary scintigraphy was performed which showed right lung perfusion and ventilation defects. Radionuclide angiography and pulmonary scintigraphy complement each other in determining the extent of vascular involvement with fibrosing mediastinitis. However, contrast venography is necessary to correctly delineate the anatomy of the obstructed superior vena cava and its collaterals.

  9. [Necrotizing mediastinitis in deep neck infections].

    PubMed

    Cárdenas-Malta, Karla Renata; Cortés-Flores, Ana Olivia; Fuentes-Orozco, Clotilde; Martínez-Oropeza, Luz del Carmen; López-Ramírez, María Karina Lizbeth; González-Ojeda, Alejandro

    2005-01-01

    Mediastinitis is a rare complication of deep neck abscesses with a high mortality. An accelerated extension to the mediastinum can happen before the identification of the primary site of infection, delaying diagnosis and treatment. To report the results of treatment of patients with mediastinitis as a complication of deep neck infection. Case series. Consecutive patients with mediastinitis secondary to deep neck abscesses, from March 2001 to February 2004. We studied five patients: three males (60%) and two females (40%), mean age 42.2 +/- 18.4 years. In all patients there was at least a 3-day delay before appropriate diagnosis was made. Hospitalization ranged between 1 and 56 days. Symptoms were fever in five cases (100%), dysphagia in four (80%), dyspnea in four (80%), retrosternal pain in three (60%), orthopnea in two (40%), and tachycardia in one (20%). Primary infection sites were of dental origin in four cases (80%) and upper respiratory tract infection in one. Surgical management consisted of cervical and mediastinal drainage with tracheotomy in all patients (100%). Three also required pleurostomy and two required gastrostomy to improve nutritional status. Mean number of surgical procedures was 5.4 +/- 1.8. All patients developed respiratory insufficiency requiring mechanical ventilation. Mortality was 60%. The delayed diagnosis was common in this case series. The length of hospitalization was long because patients required management with ventilatory support and multiple surgical procedures to limit the infectious process. High mortality is an indication for the early identification and treatment of all cases.

  10. PET imaging of primary mediastinal tumours.

    PubMed Central

    Kubota, K.; Yamada, S.; Kondo, T.; Yamada, K.; Fukuda, H.; Fujiwara, T.; Ito, M.; Ido, T.

    1996-01-01

    Mediastinal masses include a wide variety of tumours and remain an interesting diagnostic challenge for radiologist. We performed positron emission tomography (PET) studies of primary mediastinal tumours in order to predict the malignancy of these tumours preoperatively. Twenty-two patients with primary mediastinal tumours were studied with PET using 2-deoxy-2-[18F]fluoro-D-glucose (FDG). The histological findings of surgical pathology or biopsy, or mediastinoscopy were compared with those of computerised tomography (CT) and PET. PET images were evaluated semiquantitatively using the differential uptake ratio (DUR). Increased FDG uptake was observed in nine of ten patients with malignant tumours, including thymic carcinomas, lymphomas, invasive thymomas and a case of sarcoidosis. A moderate level of FDG uptake was found in a myeloma, non-invasive thymomas, and a schwannoma, whereas a low uptake was observed in a teratoma and various benign cysts. The mean FDG uptake of malignant tumours was significantly higher than that of benign tumours. Both thymic cancer and invasive thymoma showed a high FDG uptake. CT examination resulted in three false-negative and two false-positive cases when used in predicting tumour invasion, while PET was associated with a false-positive and a false-negative case. In conclusion, the use of FDG with PET is clinically helpful in evaluating the malignant nature of primary mediastinal tumours. Our results also suggest that a high FDG uptake reflects the invasiveness of malignant nature of thymic tumours. Images Figure 1 Figure 2 PMID:8611400

  11. The role of FDG PET/CT in evaluation of mediastinal masses and neurogenic tumors of chest wall

    PubMed Central

    Tatci, Ebru; Ozmen, Ozlem; Dadali, Yeliz; Biner, Inci Uslu; Gokcek, Atila; Demirag, Funda; Incekara, Funda; Arslan, Nuri

    2015-01-01

    We evaluated the efficiency of FDG PET/CT for the differentiation of malignant from benign mediastinal masses and neurogenic tumors of chest-wall. Methods: The 88 patients with chest wall-mediastinal masses who underwent examination before operation were retrospectively reviewed. Size, CT density (HU mean) and SUVmax of mediastinal and chest wall lesions were determined. Statistical differences of these parameters were compared between groups by Mann-Whitney U test. Receiver-operating characteristic curve (ROC) analysis with respect to SUVmax was performed to determine the best cutoff value for differentiating benign from malignant masses. Results: The overall sensitivity, specificity, accuracy, positive predictive value (PPV) and negative predictive value (NPV) of PET/CT in detection of malignancy were 90%, 55.17%, 67%, 50.94% and 91.43%, respectively. The SUVmax, HU mean and size were higher in malignant cases (P < 0.05). To distinguish benign and malignant lesions, the cut off value of SUVmax was 4.67. The lesion SUVmax was significantly associated with the lesion size and lesion HU mean values (P < 0.05). The value of SUVmax and HU mean were higher in solid benign lesions than those of cystic benign lesions (P < 0.05). The lesion size was higher in cystic lesions (P = 0.000). The mean SUVmax was significantly higher in invasive thymomas than those of non-invasive forms (P = 0.029). Conclusion: FDG PET/CT may be complementary to conventional imaging methods for the evaluation of mediastinal and chest wall masses. PET/CT may reduce unnecessary invasive investigations for diagnosis in patients with nonavid or low avid FDG lesions. However confirmatory tissue sampling is required to confirm PET positive findings for the definite diagnosis. PMID:26379916

  12. Primary Lymphangioma of the Palatine Tonsil in a 9-Year-Old Boy: A Case Presentation and Literature Review

    PubMed Central

    Iliadou, Eleftheria; Papapetropoulos, Nektarios; Karamatzanis, Eleftherios; Saravakos, Konstantinos

    2016-01-01

    Primary lymphangiomas or lymphangiomatous polyps of the palatine tonsil are rare benign lesions that are described infrequently in the literature. The majority of the published cases concern adults. We report a case of a lymphangiomatous lesion of the right palatine tonsil of a 9-year-old boy. Our clinical suspicion was confirmed by the histological examination after tonsillectomy and the diagnosis of primary lymphangioma of the tonsil was made. In this case we discuss the clinical and histopathological features of this lesion and present a short review of the current literature. PMID:27872780

  13. Cystic Fibrosis

    PubMed Central

    Asay, Lyal D.

    1965-01-01

    Cystic fibrosis, a disease thought to be transmitted as a recessive genetic trait, is found as a disease in about one in 1,000 to one in 10,000 births. It involves all of the exocrine glands with presenting symptoms dependent upon the extent of involvement of any group of glands. Many aspects of the disease can be corrected by substitution therapy. This applies particularly to the use of animal pancreas for the steatorrhea and salt for prevention of heat prostration. Unfortunately, the obstructive pulmonary disease with secondary bronchial infections can only be treated symptomatically by the use of mucus thinning agents, postural drainage, and antibiotics. Nevertheless, longevity can be increased and a great deal of hope offered to the families of these unfortunate children by careful supervision of their medical care. ImagesFigure 1.Figure 2.Figure 3.Figure 4.Figure 5.Figure 6.Figure 7.Figure 8.Figure 9.Figure 10.Figure 11. PMID:14288148

  14. Acquired progressive lymphangioma: Case report with partial response to imiquimod 5% cream.

    PubMed

    Salman, Andac; Sarac, Gonca; Can Kuru, Burce; Cinel, Leyla; Yucelten, Ayse Deniz; Ergun, Tulin

    2017-09-21

    Acquired progressive lymphangioma (APL), or benign lymphangioendothelioma, is an unusual entity derived from vascular structures. Clinically and histopathologically it may resemble Kaposi's sarcoma and well-differentiated angiosarcoma, causing a diagnostic problem. We report an individual with APL initially diagnosed with Kaposi's sarcoma who underwent unnecessary laboratory testing. Imiquimod 5% cream stopped the progression of the lesion. Awareness of this rare entity may prevent patients from undergoing excessive testing. Imiquimod may be used as a safe, effective treatment option. © 2017 Wiley Periodicals, Inc.

  15. Giant mesenteric lymphangioma: a rare cause of a life-threatening complication in an adult

    PubMed Central

    Siddique, Khurram; Bhandari, Santosh; Basu, Sanjoy

    2010-01-01

    Mesenteric cyst lymphangiomas (MCLs) are rare benign tumours of unknown aetiology seen mostly in children. Clinical presentation can be diverse ranging from incidental abdominal cysts to an acute abdomen. A 24-year-old previously fit and healthy young man presented with a 2-week history of cramping, central abdominal pain and vomiting requiring acute hospital admission. He was a diagnostic conundrum despite extensive preoperative investigations. Recurring symptoms required urgent surgical exploration, resection and histological confirmation with a successful outcome. We review the literature and present here this rare case in an adult of MCL that has the potential to grow, invade and develop major life-threatening complications. PMID:22778193

  16. Giant mesenteric lymphangioma: a rare cause of a life-threatening complication in an adult.

    PubMed

    Siddique, Khurram; Bhandari, Santosh; Basu, Sanjoy

    2010-09-07

    Mesenteric cyst lymphangiomas (MCLs) are rare benign tumours of unknown aetiology seen mostly in children. Clinical presentation can be diverse ranging from incidental abdominal cysts to an acute abdomen. A 24-year-old previously fit and healthy young man presented with a 2-week history of cramping, central abdominal pain and vomiting requiring acute hospital admission. He was a diagnostic conundrum despite extensive preoperative investigations. Recurring symptoms required urgent surgical exploration, resection and histological confirmation with a successful outcome. We review the literature and present here this rare case in an adult of MCL that has the potential to grow, invade and develop major life-threatening complications.

  17. Mediastinal Pseudocyst in Acute on Chronic Pancreatitis.

    PubMed

    Mishra, Sushil Kumar; Jain, Pawan Kumar; Gupta, Sukhdev

    2016-03-01

    Pseudocyst is a common complication of Acute and chronic pancreatitis. However, its extension into the mediastinum is a rare entity. We present a case of 52 years male with acute on chronic pancreatitis (alcohol related) who presented with dysphagia and dyspnoea and was found to have a pancreatic pseudocyst extending upto the neck. Ultrasound fails to pick up mediastinal pseudocysts and requires additional imaging modalities - CT and MRI. Management of Mediastinal pseudocyst depends upon underlying etiology, ductal anatomy, size of the pseudocyst, and availability of expertise. Small pseudocysts in asymptomatic patients may resolve spontaneously, but requires prolonged conservative therapy with somatostatin or its analogue and Total Parenteral Nutrition. Ruptured pseudocyst in a symptomatic unstable patient requires surgical resection. Endoscopic ultrasound guided drainage (transmural or transpapillary) and Main Pancreatic Duct stenting are safe and effective treatment modality.

  18. Primary mediastinal chondrosarcoma with Horner's syndrome.

    PubMed

    Jindal, Tarun; Chaudhary, Ramkaran; Sharma, Neeraj; Meena, Monika; Dutta, Roman; Kumar, Arvind

    2011-02-01

    Chondrosarcomas are uncommon tumors of the mediastinum. Cases reported in the literature are chondrosarcomas originating from osteocartilaginous structures; primary chondrosarcomas that have no anatomical relation with cartilaginous structures are rare. They present with myriad symptoms depending on compression of the adjacent structures; but Horner's syndrome, as a symptom, has not been described before. We report a rare case of a large primary mediastinal chondrosarcoma that presented with Horner's syndrome. © The Japanese Association for Thoracic Surgery 2011

  19. Hypertension in pregnancy: An unresectable mediastinal pheochromocytoma.

    PubMed

    Gazala, Sayf; Switzer, Noah; Bédard, Eric L R

    2016-02-01

    Hypertension is a relatively common occurrence during pregnancy, which usually has a benign course with an excellent prognosis. However, physicians caring for pregnant women should have a high index of suspicion for underlying medical conditions that could lead to a more perilous outcome. Herein, we present the case of a pregnant woman who was found to have uncontrollable hypertension late in her pregnancy, secondary to a mediastinal pheochromocytoma, which was deemed unresectable at the time of exploration after her delivery.

  20. Mediastinal bronchogenic cyst mimicking congenital lobar emphysema.

    PubMed

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

    2016-09-08

    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.

  1. Sick sinus syndrome as a complication of mediastinal radiation therapy

    SciTech Connect

    Pohjola-Sintonen, S.; Toetterman, K.J.K.; Kupari, M. )

    1990-06-01

    A 33-year-old man who had received mediastinal radiation therapy for Hodgkin's disease 12 years earlier developed a symptomatic sick sinus syndrome requiring the implantation of a permanent pacemaker. The sick sinus syndrome and a finding of an occult constrictive pericarditis were considered to be due to the previous mediastinal irradiation. A ventricular pacemaker was chosen because mediastinal radiotherapy also increases the risk of developing atrioventricular conduction defects.

  2. Progressive dyspnea in patient with large mediastinal mass

    PubMed Central

    2014-01-01

    Liposarcoma occurs very rarely in the mediastinum. Patients often remain asymptomatic until it grows large enough to cause direct invasion or compression of adjacent organs. We report a case of a 77-year-old male presented with dyspnea of exertion and was found to have a large mediastinal mass which was eventually diagnosed as primary mediastinal well-differentiated liposarcoma. The limited respiratory function at the initial presentation prompted phrenic nerve preserving incomplete resection rather than radical removal of the adjacent mediastinal structures. After surgical removal, the recurrence for well-differentiated mediastinal liposarcomas in the mediastinum is unknown; therefore, close follow-up is crucial. PMID:24393470

  3. Cystic fibrosis - nutritional considerations

    MedlinePlus

    ... this page: //medlineplus.gov/ency/article/002437.htm Cystic fibrosis - nutrition To use the sharing features on this page, please enable JavaScript. Cystic fibrosis (CF) is a life-threatening disease that causes ...

  4. [Giant cystic lymphangioma breast. Report of a case with 20-year follow-up and review of the literature].

    PubMed

    Vargas-Hernández, Víctor Manuel; Tovar-Rodríguez, José María; Moreno-Eutimio, Mario Adán; Acosta-Altamirano, Gustavo

    2014-01-01

    Antecedentes: los linfangiomas son malformaciones congénitas o adquiridas (secundarias a traumatismos, infecciones o neoplasias) de la glándula mamaria sumamente raras. Estas lesiones tienden a infiltrar los tejidos circundantes y la degeneración maligna es excepcional. Su manifestación clínica es en tumores benignos y de crecimiento lento. Se diagnostica clínicamente y por estudios de imagen. El tumor se extirpa por razones estéticas y para establecer el diagnóstico diferencial con otras lesiones más comunes. Caso clínico: paciente femenina de 45 años de edad, con incremento progresivo del tamaño de la mama izquierda, quizá originado por un traumatismo y no tiene otros sintomas. El diagnóstico histológico definitivo fue: linfangioma quístico gigante de la glándula mamaria izquierda. Discusión: los linfangiomas son lesiones extremadamente raras en la glándula mamaria, de comportamiento agresivo local y benignas. El tejido linfático anormal posee cierta capacidad de proliferar y de acumular grandes cantidades de líquido, lo que representa su apariencia quística, como sucedió en el caso aquí reportado. La extirpación quirúrgica local es el tratamiento indicado. Conclusión: este primer caso de linfangioma quístico gigante de mama reportado en México permite corroborar la benignidad de la lesión.

  5. Primary plasmacytoma involving mediastinal lymph nodes: A diagnostic mimicry of primary mediastinal lymphoma.

    PubMed

    Salem, Karma Z; Nishihori, Taiga; Kharfan-Dabaja, Mohamed A; Horna, Pedro; Alsina, Melissa

    2016-03-01

    Plasmacytomas could involve any organ, and at times might pose a diagnostic challenge when the site of involvement is unusual, or if the presentation is similar to other diseases. We describe a 48-year-old man presenting with worsening shortness of breath and chest discomfort with radiologic evidence of mediastinal enlargement, mimicking a lymphoma with mediastinal involvement. An excisional biopsy of a mediastinal lymph node showed a plasma-cell infiltrate strongly positive for CD138, with a flow-cytometry analysis showing a population of lambda-restricted neoplastic plasma cells. He failed to respond to 50Gy involved-field radiotherapy, but achieved a partial response to combination chemotherapy. He underwent high-dose chemotherapy with melphalan (200mg/m(2)) followed by lenalidomide maintenance, and is in complete remission 18months postautografting. This case illustrates a unique and rare presentation of primary lymph-node plasmacytomas involving the mediastinum potentially mistaken as lymphoid malignancy. Clinicians should be aware of the plasma-cell origin of the mediastinal neoplastic process. Copyright © 2015 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd. All rights reserved.

  6. Mediastinal paragangliomas related to SDHx gene mutations

    PubMed Central

    Ćwikła, Jarosław; Prejbisz, Aleksander; Kwiatek, Paweł; Szperl, Małgorzata; Michalski, Wojciech; Wyrwicz, Lucjan; Kuśmierczyk, Mariusz; Januszewicz, Andrzej; Maciejczyk, Anna; Roszczynko, Marta; Pęczkowska, Mariola

    2016-01-01

    Introduction Paragangliomas (PGLs) related to hereditary syndromes are rare mediastinal tumors. Paragangliomas are caused by mutations in genes encoding subunits of succinate dehydrogenase enzyme (SDH). Aim To evaluate clinical, anatomical and functional characteristics of mediastinal paragangliomas related to SDHx gene mutations. Material and methods Retrospective analysis of 75 patients with confirmed SDHx gene mutations (24 patients with SDHB, 5 SDHC, 46 with SDHD mutations) was performed. Patients underwent evaluation using computed tomography (CT), somatostatin receptor scintigraphy (SRS) (99mTc-[HYNIC,Tyr3]-octreotide), 123I mIBG scintigraphy and urinary excretion of total methoxycatecholamines. Results Out of 75 patients, 16 (21%) patients (1 SDHB, 15 SDHD mutations) had 17 PGLs localized in the mediastinum. Fourteen PGLs were localized in the middle mediastinum (intrapericardial) and 3 PGLs in the posterior mediastinum. The median diameter of paragangliomas measured on the axial slice was 24.3 mm (interquartile range (IQR): 14.7–36.6), and the median volume was 2.78 ml (IQR: 0.87–16.16). Twelve out of 16 patients (75%) underwent SRS, and 11 of them (92.3%) had pathological uptake of the radiotracer. Eleven (68.75%) out of 16 patients underwent 123 I mIBG, with only 3 positive results. Symptoms of catecholamine excretion were observed in 3 patients with PGLs localized in the posterior mediastinum. All PGLs were benign except in 1 patient with the SDHB mutation and PGL detected in the posterior mediastinum, who had a metastatic disease. Conclusions Most mediastinal paragangliomas were related to SDHD gene mutations. They were asymptomatic, localized in the medial mediastinum, intrapericardially. PMID:27785149

  7. 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

  8. 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.

  9. Descending necrotizing mediastinitis in the elderly patients

    PubMed Central

    Mazzella, Antonio; Santagata, Mario; Cecere, Atirge; La Mart, Ettore; Fiorelli, Alfonso; Tartaro, Gianpaolo; Tafuri, Domenico; Testa, Domenico; Grella, Edoardo; Perrotta, Fabio; Mazzarella, Gennaro; Santini, Mario

    2016-01-01

    Abstract Descending Necrotizing Mediastinitis (DNM) is a polymicrobic, dangerous and often fatal process, arising from head or neck infections and spreading along the deep fascial cervical planes, descending into the mediastinum. It can rapidly progress to sepsis and can frequently lead to death. It has a high mortality rate, up to 40% in the different series, as described in the literature. Surgical and therapeutic management has been discussed for long time especially in an elderly patient population. The literature has been reviewed in order to evaluate different pathogenesis and evolution and to recognise a correct therapeutic management. PMID:28352835

  10. Harlequin Syndrome Following Resection of Mediastinal Ganglioneuroma

    PubMed Central

    Jeon, Yeong Jeong; Son, Jongbae; Cho, Jong Ho

    2017-01-01

    Harlequin syndrome is a rare disorder of the sympathetic nervous system characterized by unilateral facial flushing and sweating. Although its etiology is unknown, this syndrome appears to be a dysfunction of the autonomic nervous system. To the best of our knowledge, thus far, very few reports on perioperative Harlequin syndrome after thoracic surgery have been published in the thoracic surgical literature. Here, we present the case of a 6-year-old patient who developed this unusual syndrome following the resection of a posterior mediastinal mass. PMID:28382275

  11. Trans-mediastinal herniation of bulla: Semilunar sign

    PubMed Central

    Singh, Sheetu; Singh, Nishtha; Singh, Virendra

    2015-01-01

    Trans-mediastinal herniation of lung is seen occasionally but herniation of bulla across the mediastinum is rare. We report two cases with trans-mediastinal herniation of bullae leading to an unusual line appearing near the mediastinum. We propose the name ‘Semilunar sign’for this radiological sign because of its resemblance to the half-moon shape. PMID:26664170

  12. 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

  13. Postoperative Mediastinitis Due to Finegoldia magna with Negative Blood Cultures▿

    PubMed Central

    Kernéis, Solen; Matta, Matta; Hoï, Annie Buu; Podglajen, Isabelle; Gutmann, Laurent; Novara, Ana; Latremouille, Christian; Mainardi, Jean-Luc

    2009-01-01

    We report a case of Finegoldia magna (formerly known as Peptostreptococcus magnus) mediastinitis following coronary artery bypass in a 50-year-old patient. Even if staphylococci remain the main causative organism of postoperative mediastinitis, the responsibility of anaerobic bacteria must be considered in cases of fever and sternal drainage with negative blood cultures. PMID:19812272

  14. Bilateral Cystic Adrenal Neuroblastoma with Cystic Liver metastasis

    PubMed Central

    Aslan, Mine; Kalyoncu, Ayse Ucar; Habibi, Hatice Arioz; Ozdemir, Gul Nihal; Koc, Basak; Adaletli, Ibrahim

    2017-01-01

    Bilateral congenital cystic adrenal neuroblastoma (NB) with cystic liver metastasis is a very rare condition and only few cases have been reported in the literature. Herein we report a case of a congenital bilateral cystic adrenal NB with cystic liver metastasis and briefly discuss characteristic imaging features of cystic NB. PMID:28163998

  15. Coronary artery disease following mediastinal radiation therapy

    SciTech Connect

    Annest, L.S.; Anderson, R.P.; Li, W.; Hafermann, M.D.

    1983-02-01

    Coronary artery disease occurred in four young men (mean age 41 years) who had received curative irradiation therapy for mediastinal malignancies 12 to 18 (mean 15) years previously. None was at high risk for developing coronary artery disease by Framingham criteria. Angiography demonstrated proximal coronary artery disease with normal distal vessels. Distribution of the lesions correlated with radiation dosimetry in that vessels exposed to higher radiation intensity were more frequently diseased. A total of 163 patients underwent mediastinal irradiation for lymphoma or thymoma between 1959 and 1980. Among the 29 who survived 10 or more years, five (18%) developed severe coronary artery disease, implicating thoracic radiotherapy as an important risk factor. Because of the importance of mantle irradiation in the treatment of lymphomas, the prevalence of these neoplasms, and the survival patterns following treatment, many long-term survivors may be at increased risk for the development of coronary artery disease. Recognition of the relationship between radiotherapy and coronary artery disease may lead to earlier diagnosis and more timely intervention.

  16. Coronary artery disease following mediastinal radiation therapy

    SciTech Connect

    Annest, L.S.; Anderson, R.P.; Li, W.; Hafermann, M.D.

    1983-02-01

    Coronary artery disease occurred in four young men (mean age 41 years) who had received curative irradiation therapy for mediastinal malignancies 12 to 18 (mean 15) years previously. None was at high risk for developing coronary artery disease by Framingham criteria. Angiography demonstrated proximal coronary artery disease with normal distal vessels. Distribution of the lesions correlated with radiation dosimetry in that vessels exposed to higher radiation intensity were more frequently diseased. Three patients had coronary bypass grafting for intractable angina and are asymptomatic at 10 to 43 months. A total of 163 patients underwent mediastinal irradiation for lymphoma or thymoma between 1959 and 1980. Among the 29 who survived 10 or more years, five (18%) developed severe coronary artery disease, implicating thoracic radiotherapy as an important risk factor. Because of the importance of mantle irradiation in the treatment of lymphomas, the prevalence of these neoplasms, and the survival patterns following treatment, many long-term survivors may be at increased risk for the development of coronary artery disease. Recognition of the relationship between radiotherapy and coronary artery disease may lead to earlier diagnosis and more timely intervention. Standard surgical treatment may be particularly beneficial because of the relative youth of most of these patients and because the proximal distribution of typical lesions increases the likelihood of complete revascularization.

  17. 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

  18. How Is Cystic Fibrosis Treated?

    MedlinePlus

    ... page from the NHLBI on Twitter. How Is Cystic Fibrosis Treated? Cystic fibrosis (CF) has no cure. However, ... help oral pancreatic enzymes work better. Treatments for Cystic Fibrosis Complications A common complication of CF is diabetes . ...

  19. Cystic Fibrosis: Diet and Nutrition

    MedlinePlus

    ... Right Sport for You Healthy School Lunch Planner Cystic Fibrosis: Diet and Nutrition KidsHealth > For Teens > Cystic Fibrosis: ... Food Enzyme Supplements Beating the Frustration What Is Cystic Fibrosis? At lunch, Lindsay often gets bored with having ...

  20. Mediastinal Myxoid Liposarcoma with Intrapericardial Involvement and Large Pericardial Effusion.

    PubMed

    Endara, Santiago Adolfo; Davalos, Gerardo Augusto; Vinueza, Ana Lucia; Montalvo, Nelson; Duran, Patricia Graciela; Barzallo, David Esteban

    2015-10-28

    Liposarcoma is the name given to a group of soft tissue sarcomas (STSs) with adipocytic differentiation. As a group, liposarcomas are the second most common STSs in adults. In 1951 Kozonis et al published that in the English language only four cases of liposarcomas originating in the mediastinum had been described. Primary mediastinal liposarcoma is an uncommon neoplasm of intrathoracic origin. We present the case of a 47-year-old woman diagnosed with a large mediastinal mass with intrapericardial invasion and massive pericardial effusion; biopsies showed a mediastinal liposarcoma.

  1. 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

  2. Imaging Manifestations of Mediastinal Fat Necrosis

    PubMed Central

    Bhatt, Malay Y.; Martínez-Jiménez, Santiago; Rosado-de-Christenson, Melissa L.; Watson, Kenneth R.; Walker, Christopher M.; Kunin, Jeffrey R.

    2013-01-01

    Mediastinal fat necrosis (MFN) or epipericardial fat necrosis, as it is commonly referred to in the literature, is a rare self-limiting cause of chest pain of unclear etiology. MFN affects previously healthy individuals who present with acute pleuritic chest pain. Characteristic computed tomography (CT) findings include a fat attenuation lesion with intrinsic and surrounding increased attenuation stranding. There is often associated thickening of the adjacent pericardium and/or pleural effusions. We present two cases of MFN manifesting as ovoid fat attenuation lesions demarcated by a soft tissue attenuation rim with intrinsic and surrounding soft tissue attenuation stranding and review the clinical and pathologic features of these lesions. Knowledge of the clinical presentation of patients with MFN and familiarity with the characteristic imaging findings of these lesions should allow radiologists to prospectively establish the correct diagnosis and suggest conservative management and follow-up. PMID:24369521

  3. Fibrosing mediastinitis: a rare complication of histoplasmosis.

    PubMed

    Patel, Milesh; Lu, Frederic; Hannaway, Maria; Hochman, Katherine

    2015-11-04

    We report a case of a 29-year-old man who presented with intermittent haemoptysis for about 18 months. Previously, his symptoms had been diagnosed as musculoskeletal pain and later as pneumonia. CT found a venous infarct in the right lung in addition to extensive lymphadenopathy in the mediastinum and pulmonary hila, with associated calcifications almost completely occluding the superior vena cava and azygos vein. Further questioning revealed that the patient had once worked on an organic farm in Colorado. Subsequent work up was positive for histoplasmosis yeast antibodies. The patient was diagnosed with fibrosing mediastinitis (FM) and started on itraconazole for 3 months. We note that FM is a rare complication of histoplasmosis and can present as chronic haemoptysis. Travel history is an important aspect of the clinical evaluation. Antifungal agents have shown some efficacy in treating histoplasmosis-related FM.

  4. Mycoplasma hominis Induces Mediastinitis after a Tonsillar Abscess

    PubMed Central

    Grancini, Anna; Colosimo, Manuela; Restelli, Antonella; Colombo, Rosaria; Maraschini, Anna; Pozzi, Cristina; Breda, Giuseppe; Protti, Alessandro; Arghittu, Milena; Maiavacca, Rita

    2016-01-01

    Mycoplasma hominis is commonly involved in genitourinary tract infections. We report a 59-year-old man who developed a M. hominis-associated mediastinitis following acute tonsillar infection. PMID:27957362

  5. An interleukin-6-producing cardiac myxoma associated with mediastinal lymphadenopathy.

    PubMed

    Takizawa, T; Sumino, H; Kanda, T; Kobayashi, I; Nagai, R; Ichikawa, S

    1999-01-01

    We report our experience with a patient whose mediastinal lymphadenopathy resolved after resection of a cardiac myxoma that secreted interleukin-6 (IL-6). The patient was a 68-year-old female who complained of nocturnal chest discomfort related to congestive heart failure. An echocardiogram demonstrated a large left atrial mass. A computed tomogram showed not only the left atrial mass but multiple enlarged mediastinal lymph nodes. The serum IL-6 level was markedly elevated at 13.7 pg/ml. After resection of the cardiac myxoma, serum IL-6 returned to the normal range. A repeat computed tomogram showed no mediastinal lymphadenopathy. We believe that overproduction of IL-6 by the cardiac myxoma was the cause of the mediastinal lymphadenopathy.

  6. Living with Cystic Fibrosis

    MedlinePlus

    ... page 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 can ... with your doctors to learn how to manage CF. Ongoing Care Having ongoing medical care by a ...

  7. Chromomycosis: Subcutaneous cystic type.

    PubMed

    Agrawal, S N; Bhise, P R; Sony, P R

    2000-01-01

    A 38-year -old male farmer presented with a solitary, asymptomatic, cystic lesion on the palm since last four years. He underwent excision of this cyst two times during this period but the lesion recurred near the same site. The histopathology and the microbiological examination led to the diagnosis of the rare subcutaneous cystic type of chromomycosis.

  8. Number of Lymph Nodes Harvested From a Mediastinal Lymphadenectomy

    PubMed Central

    Allen, Mark S.; Decker, Paul A.; Ballman, Karla; Malthaner, Richard A.; Inculet, Richard I.; Jones, David R.; McKenna, Robert J.; Landreneau, Rodney J.; Putnam, Joe B.

    2011-01-01

    Background: Lymph node status is a major determinant of stage and survival in patients with lung cancer; however, little information is available about the expected yield of a mediastinal lymphadenectomy. Methods: The American College of Surgeons Oncology Group Z0030 prospective, randomized trial of mediastinal lymph node sampling vs complete mediastinal lymphadenectomy during pulmonary resection enrolled 1,111 patients from July 1999 to February 2004. Data from 524 patients who underwent complete mediastinal lymph node dissection were analyzed to determine the number of lymph nodes obtained. Results: The median number of additional lymph nodes harvested from a mediastinal lymphadenectomy following systematic sampling was 18 with a range of one to 72 for right-sided tumors, and 18 with a range of four to 69 for left-sided tumors. The median number of N2 nodes harvested was 11 on the right and 12 on the left. A median of at least six nodes was harvested from at least three stations in 99% of patients, and 90% of patients had at least 10 nodes harvested from three stations. Overall, 21 patients (4%) were found to have occult N2 disease. Conclusions: Although high variability exists in the actual number of lymph nodes obtained from various nodal stations, complete mediastinal lymphadenectomy removes one or more lymph nodes from all mediastinal stations. Adequate mediastinal lymphadenectomy should include stations 2R, 4R, 7, 8, and 9 for right-sided cancers and stations 4L, 5, 6, 7, 8, and 9 for left-sided cancers. Six or more nodes were resected in 99% of patients in this study. Trial registry: ClinicalTrials.gov; No.: NCT00003831; URL: clinicaltrials.gov PMID:20829340

  9. Pulmonary scintigraphy in fibrosing mediastinitis due to histoplasmosis

    SciTech Connect

    Park, H.M.; Jay, S.J.; Brandt, M.J.; Holden, R.W.

    1981-04-01

    The pulmonary scintigraphic findings from our patients with fibrosing mediastinitis due to histoplasmosis are reported. The ventilation/perfusion (V/Q) mismatch mimicked pulmonary emboli. However, in these cases the chest radiographs and/or gallium-67 scintigraphy were abnormal, suggesting mediastinal or hilar disease. Awareness of the nonembolic conditions that can result in V/Q mismatches is important in the interpretation of lung scans.

  10. "Salvage" surgery for primary mediastinal malignancies: is it worthwhile?

    PubMed

    Petrella, Francesco; Leo, Francesco; Veronesi, Giulia; Solli, Piergiorgio; Borri, Alessandro; Galetta, Domenico; Gasparri, Roberto; Lembo, Rosalba; Radice, Davide; Scanagatta, Paolo; Spaggiari, Lorenzo

    2008-01-01

    Indications and results of salvage surgery in mediastinal tumors are still unclear. This study analyzes a single-center experience to assess its mortality, morbidity, and long-term results. Mediastinal salvage surgery (MSS) was defined as surgical resection of persistent or recurrent primary mediastinal tumors after previous local treatments with curative intent or exclusive chemotherapy in case of bulky tumors. Clinical data of patients undergoing MSS between 1998 and 2005 were analyzed. Overall and disease-specific long-term survival was calculated. Twenty-one patients (15 men and 6 women, mean age 41 years) underwent MSS. Eleven patients suffered from thymic tumors (eight thymomas, three thymic carcinoma) whereas 10 patients suffered from nonthymic tumors (one lung adenocarcinoma + thymoma, two mediastinal monophasic sinovial sarcoma, one mediastinal neuroendocrine tumor, one mediastinal teratoblastoma, one mediastinal disgerminoma, one Hodgkin's lymphoma, one mediastinal atypic carcinoid, two medullary thyroid carcinoma). MSS required extended vascular resection in 10 cases and cardiopulmonary bypass in one case. Median operation time was 215 minutes (range 140-720). One postoperative death and four major complications were recorded (overall mortality 4.7%, morbidity 19.0%). With a median follow-up of 30.6 months, overall 1-, 3-, and 5-year Kaplan-Meier survival was 89.7, 71.2, and 56.6%, respectively. Thymic neoplasms had a better prognosis (1-, 3-, and 5-year survival was 100, 87.5, 87.5%, respectively) when compared with others (1-, 3-, and 5-year survival was 77.8, 53.3, 26.7%, respectively--logrank p = 0.0128). MSS can offer a chance of curative treatment in selected patients with an acceptable morbidity and mortality. Thymic tumors obtain the best results in term of long-term survival.

  11. Asymptomatic peritoneal carcinomatosis originating from benign cystic peritoneal mesothelioma.

    PubMed

    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.

  12. 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

  13. Mediastinal lymphadenopathy in pediatric tuberculosis in computed tomography scan.

    PubMed

    Mehrain, Payam; Moghaddam, Amin Momeni; Tavakol, Elham; Amini, Afshin; Moghimi, Mehrdad; Kabir, Ali; Velayati, Ali Akbar

    2016-12-01

    Pediatric tuberculosis is usually a primary infection presenting mainly as mediastinal or hilar adenopathy in computed tomography (CT) scan. In this study, we study the distribution and other CT scan characteristics of mediastinal lymphadenopathy in childhood tuberculosis. Chest CT scans of 75 cases of pediatric tuberculosis at Masih Daneshvari Hospital in Tehran, Iran, from 2009 to 2013 were studied regarding characteristics of mediastinal lymphadenopathy. Mean±standard deviation age of cases was 11.2±4.6years. Lymphadenopathy (mediastinal/hilar) was detected in 94.7% of cases. Most of the lymphadenopathies were located in the lower paratracheal (81.7%), upper paratracheal (69.1%), hilar (53.5%), and subcarinal (47.9%) stations. Perilymph node fatty stranding, lymphadenopathy conglomeration, bronchial pressure by the lymph nodes, and lymph node calcification were noted in 74.6%, 52.11%, 4.23%, and 5.6% of cases, respectively. Bilateral, right, and left lung parenchymal involvement were reported in 45%, 25%, and 8% of cases, respectively. Lung parenchymal involvement was significantly correlated with lymphadenopathies in subcarinal (p<0.001), hilar (p<0.001), subaortic (p=0.03), lower paratracheal (p=0.037), and axillary (p=0.006) stations. Right- and left-sided pleural effusions were observed in 12.7% and 7% of cases, respectively. Attention to distribution and characteristics of mediastinal lymphadenopathy can help differentiate tuberculosis from other causes of pediatric mediastinal lymphadenopathy. Copyright © 2016.

  14. Transoral endoscopic mediastinal surgery (TOEMS)—results of a first clinical study for scarless mediastinal lymph node biopsies

    PubMed Central

    Frese, Steffen; Leschber, Gunda; Nemat, André; Wilhelm, Thomas

    2016-01-01

    Background Video-assisted mediastinoscopy (VAM) represents the standard procedure for mediastinal lymph node biopsies. This operation results in a scar at prominent position at the anterior neck. Since there is a trend to less invasive procedures, natural orifice transluminal endoscopic surgery (NOTES) was introduced to different fields of surgery. Based on NOTES we developed a new approach for mediastinoscopy: transoral endoscopic mediastinal surgery (TOEMS). In previous studies using human cadavers and living pigs the feasibility of TOEMS was shown. It was unclear whether TOEMS could be safely applied in patients requiring mediastinal lymph node biopsies. Methods We conducted a clinical phase I study recruiting ten patients with unclear mediastinal lymphadenopathy not resolved by prior bronchoscopy. All patients underwent TOEMS for mediastinal lymph node biopsy. The duration of the procedure and complications were monitored. In addition, all patients were examined for pain, swallowing dysfunction and sensation disturbance. Results TOEMS was accomplished in eight patients. In two patients operation was converted to VAM due to technical problems. Mediastinal lymph nodes were dissected in all patients who finished with TOEMS. On average, two separate lymph stations were reached by TOEMS. Duration of the procedure was 159±22 min. Permanent palsy of the right recurrent laryngeal nerve was noticed in one patient postoperatively. Conclusions This is the first report for a human application of NOTES in thoracic surgery. In fact, transoral endoscopic surgery seems to be a feasible approach for mediastinal lymph node biopsies. Further studies are needed to show whether this procedure has an advantage over VAM in terms of pain, complications and accessibility of mediastinal lymph node stations. PMID:27867546

  15. Primary Mediastinal B-Cell Lymphoma

    PubMed Central

    Pileri, Stefano A.; Gaidano, Gianluca; Zinzani, Pier Luigi; Falini, Brunangelo; Gaulard, Philippe; Zucca, Emanuele; Pieri, Federica; Berra, Eva; Sabattini, Elena; Ascani, Stefano; Piccioli, Milena; Johnson, Peter W. M.; Giardini, Roberto; Pescarmona, Edoardo; Novero, Domenico; Piccaluga, Pier Paolo; Marafioti, Teresa; Alonso, Miguel A.; Cavalli, Franco

    2003-01-01

    Although primary mediastinal (thymic) large B-cell lymphoma has been primarily studied, its precise phenotype, molecular characteristics, and histogenesis are still a matter of debate. The International Extranodal Lymphoma Study Group collected 137 such cases for extensive pathological review. Histologically, the lymphomatous growth was predominantly diffuse with fibrosis that induced compartmentalized cell aggregation. It consisted of large cells with varying degrees of nuclear polymorphism and clear to basophilic cytoplasm. On immunohistochemistry, the following phenotype was observed: CD45+, CD20+, CD79a+, PAX5/BSAP+, BOB.1+, Oct-2+, PU.1+, Bcl-2+, CD30+, HLA-DR+, MAL protein+/−, Bcl-6+/−, MUM1/IRF4+/−, CD10−/+, CD21−, CD15−, CD138−, CD68−, and CD3−. Immunoglobulins were negative both at immunohistochemistry and in situ hybridization. Molecular analysis, performed in 45 cases, showed novel findings. More than half of the cases displayed BCL-6 gene mutations, which usually occurred along with functioning somatic IgVH gene mutations and Bcl-6 and/or MUM1/IRF4 expression. The present study supports the concept that a sizable fraction of cases of this lymphoma are from activated germinal center or postgerminal center cells. However, it differs from other aggressive B-cell lymphomas in that it shows defective immunoglobulin production despite the expression of OCT-2, BOB.1, and PU.1 transcription factors and the lack of IgVH gene crippling mutations. PMID:12507907

  16. Sclerosing mediastinitis and mast cell activation syndrome.

    PubMed

    Afrin, Lawrence B

    2012-03-15

    Sclerosing mediastinitis (ScM) is a rare, potentially life-threatening disorder, idiopathic in roughly half the cases. Systemic symptoms not attributable to sclerosis often appear in idiopathic ScM. Mast cell activation disease (MCAD) is a potential cause of these symptoms and also can cause sclerosis. ScM has not previously been associated with MCAD. Presented here are the first two cases of ScM associated with MCAD, specifically mast cell activation syndrome (MCAS). CASE 1: A 58-year-old chronically polymorbid woman developed ScM following matched sibling allogeneic stem cell transplantation. Eight years later MCAS, likely underlying most of her chronic issues, was identified. CASE 2: A 30-year-old chronically polymorbid woman presented with superior vena cava syndrome and was diagnosed with ScM. On further evaluation, MCAS was identified. Treatment promptly effected symptomatic improvement; sclerosis has been stable. Non-compliance yielded symptomatic relapse; restored compliance re-achieved symptomatic remission. Different MCAS presentations reflect elaboration of different mediators, some of which can induce inflammation and fibrosis. Thus, MCAS may have directly and/or indirectly driven ScM in these patients. MCAS should be considered in ScM presenting with comorbidities better explained by mast cell mediator release. Copyright © 2012 Elsevier GmbH. All rights reserved.

  17. 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

  18. [Thoracoscopic resection of mediastinal hypersecretant parathyroid gland].

    PubMed

    Gramática, Luis; Mercado Luna, Andrés; Verasay, Guillermo; Brizuela, Adrián; Gramática, Luis

    2004-01-01

    In general experienced hands, the inferior parathyroid glands, localized in the thymus or low extrathymus are the main cause or surgical therapeutic failure. When they could not be approached through cervicotomy, or when they were diagnosed after surgery the sternotomy or the angiographic ablation were chosen as treatment. The limited number of thoracoscopic approach whit good results published so far, moved us to use this method. Patient of 49 years with a 7 year secondary hiperprathyroidism due to CRI. After a sub total parathyroidectomy with bilateral thimectomy, the patient did not present clinical or laboratory improvement. Through a thoracic centellogram sixth MIBI, NMR and CAT, a tumor in the middle mediastinum was fond. The mediastinal structures have been easily identified through a left thoracoscopic approach. At the level of the aortopulmonary window, a tumoral mass is located and, with a simple dissection, et is easily extracted in bag. The biopsy through freezing confirms the parathyroid etiology of the gland. Clinical and laboratory evolution has been favorable, with hospital discharged at the 3 day, and being asymptomatic after two years. The hipersecretant parathyroid glands, located in middle mediastinum, that can not be approach through cervicotomy, can be successfully approach through thoracoscopic technique.

  19. Tc-99m Sulfur Colloid Lymphoscintigraphy with Single-photon Emission Computed Tomography/Computed Tomography in a Case of Acquired Vulval Lymphangiomas

    PubMed Central

    Tulsyan, Shruti; Tripathi, Madhavi; Das, Kalpa; Yadav, Divya; Shamim, Shamim Ahmed; Damle, Nishikant; Bal, Chandrasekhar

    2017-01-01

    We describe the lymphoscintigraphy findings of a 25-year-old female patient who was undergoing presurgical workup for lymphangiomas of the vulva. She had a history of treatment for disseminated tuberculosis 6 years back and presented with herpetiform oozing vesicles in the external genitalia. Single-photon emission computed tomography/computed tomography (SPECT/CT) confirmed cutaneous tracer accumulation in the vulval lesions and demonstrated the presence of densely calcified inguinal nodes secondary to healed tuberculosis as the etiology of secondary lymphangioma. PMID:28242996

  20. Learning about Cystic Fibrosis

    MedlinePlus

    ... Cystic Fibrosis: Your Genes, Your Health [yourgenesyourhealth.org] Multimedia educational site including screening and treatment information. Hosted by the Dolan DNA Learning Center at Cold Spring Harbor Laboratory. What is ...

  1. 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

  2. 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

  3. Diabetes in cystic fibrosis.

    PubMed

    Bridges, Nicola

    2013-05-01

    Cystic fibrosis related diabetes (CFRD) is a common complication of cystic fibrosis, caused by a fall in insulin secretion with age in individuals with pancreatic insufficiency. CFRD is associated with worse clinical status and increased mortality. Treatment of CFRD with insulin results in sustained improvements in lung function and nutrition. While clinical experience with insulin treatment in CF has increased, the selection of who to treat and glycaemic targets remain unclear. Copyright © 2013. Published by Elsevier Ltd.

  4. 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

  5. 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

  6. 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 ...

  7. 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 ...

  8. What's it Like to Have Cystic Fibrosis?

    MedlinePlus

    ... CPR: A Real Lifesaver Kids Talk About: Coaches Cystic Fibrosis KidsHealth > For Kids > Cystic Fibrosis Print A A ... strength to deal with cystic fibrosis. What Is CF? Cystic fibrosis (CF) is a disease that causes ...

  9. Isolated mediastinal necrotizing granulomatous lymphadenopathy due to cat-scratch disease.

    PubMed

    Lovis, A; Clerc, O; Lazor, R; Jaton, K; Greub, G

    2014-02-01

    We report a patient suffering from cat-scratch disease limited to mediastinal lymphadenitis. Although rare, cat-scratch disease should be considered in the differential diagnosis of mediastinal lymphadenitis, especially when patients were exposed to cats.

  10. Unusual cause of massive hemothorax: spontaneous rupture of nonfunctioning mediastinal paraganglioma

    PubMed Central

    Lin, Feng; Liu, Chengwu; Ma, Lin; Pu, Qiang; Zhu, Yunke; Xiao, Zhilan; Guo, Chenglin; Zhang, Xiaolong; Li, Chuan

    2016-01-01

    Mediastinal paraganglioma is a rare neurogenic tumor with a hypervascular feature. The spontaneous rupture of mediastinal paraganglioma is an unusual cause of massive hemothorax. Here we present a case of 39-year-old man with massive hemothorax due to the spontaneous rupture of a mediastinal paraganglioma. The man underwent successful resection of tumor and had an uneventful recovery. To our knowledge, this is the first reported case of spontaneous rupture of nonfunctioning mediastinal paraganglioma. PMID:28149584

  11. Mediastinal Abscess Caused by an Expanded Polytetrafluoroethylene Membrane.

    PubMed

    Matsuzaki, Kanji; Tsukada, Toru; Sato, Masataka; Watanabe, Yasunori; Ikeda, Akihiko; Konishi, Taisuke; Jikuya, Tomoaki

    2017-07-01

    A 76-year-old man who had undergone tricuspid annuloplasty 13 years earlier was admitted to our hospital because of a high fever. Although he was treated with antibiotics for pneumonia, a mild fever persisted. Computed tomography and gallium scintigraphy revealed a mediastinal abscess with an expanded polytetrafluoroethylene (ePTFE) membrane. There were no positive cultures or sternal changes indicating poststernotomy mediastinitis. The membrane was removed by a left parasternal approach without resternotomy, and its total removal was essential for the patient's recovery. This is the first reported case of sterile mediastinal abscess caused by an ePTFE membrane after a cardiac operation. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  12. [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. Copyright © 2011 SERAM. Published by Elsevier Espana. All rights reserved.

  13. Correlative Imaging in a Patient with Cystic Thymoma: CT, MR and PET/CT Comparison

    PubMed Central

    Romeo, Valeria; Esposito, Alfredo; Maurea, Simone; Camera, Luigi; Mainenti, Pier Paolo; Palmieri, Giovannella; Buonerba, Carlo; Salvatore, Marco

    2015-01-01

    Summary Background Cystic thymoma is a rare variant of thymic neoplasm characterized by almost complete cystic degeneration with mixed internal structure. We describe a case of a 60 year-old woman with a cystic thymoma studied with advanced tomographic imaging stydies. CT, MRI and PET/CT with 18F-FDG were performed; volumetric CT and MRI images provided better anatomic evaluation for pre-operative assessment, while PET/CT was helpful for lesion characterization based on 18F-FDG uptake. Although imaging studies are mandatory for pre-operative evaluation of cystic thymoma, final diagnosis still remains surgical. Case Report A 60-year-old woman with recent chest pain and no history of previous disease was admitted to our departement to investigate the result of a previous chest X-ray that showed bilateral mediastinal enlargement; for this purpose, enhanced chest CT scan was performed using a 64-rows scanner (Toshiba, Aquilion 64, Japan) before and after intravenous bolus administration of iodinated non ionic contrast agent; CT images demonstrated the presence of a large mediastinal mass (11×8 cm) located in the anterior mediastinum who extended from the anonymous vein to the cardio-phrenic space, compressing the left atrium and causing medium lobe atelectasis; bilateral pleural effusion was also present. Conclusions In conclusion, correlative imaging plays a foundamental role for the diagnostic evaluation of patient with cystic thymoma. In particular, volumetric CT and MRI studies can provide better anatomic informations regarding internal structure and local tumor spread for pre-operative assessment. Conversely, metabolic imaging using 18F-FDG PET/CT is helpful for lesion characterization differentiating benign from malignant lesion on the basis of intense tracer uptake. The role of PET/MRI is still under investigation. However, final diagnosis still remains surgical even though imaging studies are mandatory for pre-operative patient management. PMID:25593635

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

    PubMed

    Evans, Natashia A; Viviano, Katrina R

    2015-11-01

    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.

  15. Mediastinal fibrosis and radiofrequency radiation exposure: is there an association?

    PubMed

    Papandreou, L; Panagou, P; Bouros, D

    1992-01-01

    A 45-year-old officer, working for a period of 18 years at a military radar base, presented with progressive exertional dyspnea, dry cough, and hemoptysis. Subsequent evaluation demonstrated a left pulmonary artery occlusion as well as a left upper lobe bronchus stenosis, due to a dense fibrotic mediastinal mass. Histologically, this proved to be idiopathic mediastinal fibrosis (IMF). The development of IMF in a man exposed for a long period to radio-frequency radiation (RFR) is unique in the literature in English. The possible association of radiation exposure with IMF is discussed.

  16. Giant primary mediastinal liposarcoma: A rare cause of atrial flutter.

    PubMed

    Arrarás-Martínez, Miguel J; Rieger-Reyes, Cristina; Panadero-Paz, Carolina; Landa-Oviedo, Hugo S; García-Tirado, Javier

    2015-11-01

    We report the case of a 68-year-old man who presented with atrial flutter and was observed radiologically to have a large mass in the posterior mediastinum. During surgical removal, spontaneous recovery of sinus rhythm occurred. There was no late recurrence of arrhythmia. The diagnosis was mediastinal liposarcoma of mixed type (extremely rare). Supraventricular arrhythmia associated with mediastinal tumors is exceptional. Surgery appears to be the only potentially curative treatment for these tumors. In cases like ours presenting with arrhythmia, surgery is considered essential for control of the arrhythmia. © The Author(s) 2015.

  17. [Broncho-mediastinal fistula -- a rare manifestation of a tumor].

    PubMed

    Marques, A S; Pinto, F G; Noronha, M M; Vera, J; Monteiro, M

    2004-01-01

    Broncho-mediastinal fistulaes are a very rare condition, existing only a report of a case related with primary lung cancer. The authors present a clinical case of a white 58-year-old man, with previous history of cigarette smoking and diabetes, with fever, productive cough and progressive dyspnea, with radiological evidence of right inferior lobar pneumonia. We noticed a radiological deterioration, despite the high spectrum antibiotic therapy, so he was submitted to thoracic computerized tomography and broncofibroscopy, which revealed extensive infiltration of the principal and intermediary bronchioles with a broncho-mediastinal-esophagic fistula. The histological exam revealed a pavimentam cellular carcinoma.

  18. A Case of Mediastinal Granular Cell Tumor with Horner's Syndrome.

    PubMed

    Shikatani, Yoshinobu; Okazaki, Mikio; Sakao, Nobuhiko; Yukumi, Shungo; Shigematsu, Hisayuki; Kitazawa, Sohei; Sano, Yoshifumi

    2015-01-01

    Granular cell tumor (GCT) is found in various organs but is rare in the mediastinum. We report a case of mediastinal GCT in a 19-year-old woman who presented with left ptosis and miosis. CT and MRI revealed a 29-mm well-circumscribed tumor located close to the first thoracic vertebra with features suggesting a neurogenic tumor. The tumor was completely excised using single-port video-assisted thoracoscopic surgery. Histopathological and immunohistochemical analysis revealed that the tumor was a benign GCT. Postoperatively, left ptosis and miosis had improved slightly. To our knowledge, this is the first report regarding mediastinal GCT presenting with preoperative Horner's syndrome.

  19. Video assisted thoracoscopic resection of a posterior mediastinal Castleman's tumor

    PubMed Central

    2011-01-01

    Castleman's disease (CD) or angiofollicular lymph node hyperplasia is a rare spectrum of lymphoproliferative disorders. CD tumors are commonly localized in the mediastinum and are usually asymptomatic. The mainstay of treatment is surgical resection and has typically been performed using open thoracotomy. Few reports in the literature describe video assisted thoracoscopic resection of these tumors. The differential diagnosis for mediastinal masses is extensive, and CD tumors, although uncommon, should be considered. We describe a case report of a posterior mediastinal Castleman's tumor adherent to the esophagus, which was resected thoracoscopically and review the literature. PMID:21933401

  20. [The management of infectious mediastinitis after the open heart surgery].

    PubMed

    Imada, T; Morishige, N; Nonaka, K; Yamanaka, J

    2000-03-01

    Between October of 1992 and September of 1998, we performed 604 open heart operations. Among them, 12 cases (1.9%) were complicated with postoperative infectious mediastinitis. Five patients (Group A) were treated by conservative therapy which consists of open drainage and intermittent closed irrigation with dilute povidone iodine solution. Seven patients (Group B) were treated surgically in addition to the above-mentioned conservative treatment. Among those patients, one patient developed fatal complication. We have realized that mental care of the patients was also very important when long term hospitalization was necessitated. The hyperbaric oxygen therapy seemed to be also effective for postoperative mediastinitis caused by MRSA.

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

    PubMed

    Marshall, M Blair; DeMarchi, Lorenzo; Emerson, Dominic A; Holzner, Matthew L

    2015-11-01

    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.

  3. [Atypical intraperitoneal cystic masses].

    PubMed

    Domínguez-Pérez, S T; Baeza-Herrera, C; Villalobos-Castillejos, A; González-Mateos, T; Aguilar-Venegas, M

    2010-01-01

    Omental, mesenteric and retroperitoneal cystic masses are very rare in pediatric population. They usually present as asymptomatic abdominal tumors and only occasionally a preoperative diagnosis is made. We presented 4 cases presented to our hospital during a 2-year period. All patients were male with an age ranged from 5 months to 7 years. Three patients had previous diagnosis of cystic mass by abdominal ultrasound. Three patients presented with acute abdomen and one patient with bowel obstruction. All patients underwent successful resection of the mass with no perioperative mortality.

  4. Huge mediastinal liposarcoma resected by clamshell thoracotomy: a case report.

    PubMed

    Toda, Michihito; Izumi, Nobuhiro; Tsukioka, Takuma; Komatsu, Hiroaki; Okada, Satoshi; Hara, Kantaro; Ito, Ryuichi; Shibata, Toshihiko; Nishiyama, Noritoshi

    2017-12-01

    Liposarcoma is the single most common soft tissue sarcoma. Because mediastinal liposarcomas often grow rapidly and frequently recur locally despite adjuvant chemotherapy and radiotherapy, they require complete excision. Therefore, the feasibility of achieving complete surgical excision must be carefully considered. We here report a case of a huge mediastinal liposarcoma resected via clamshell thoracotomy. A 64-year-old man presented with dyspnea on effort. Cardiomegaly had been diagnosed 6 years previously, but had been left untreated. A computed tomography scan showed a huge (36 cm diameter) anterior mediastinal tumor expanding into the pleural cavities bilaterally. The tumor comprised mostly fatty tissue but contained two solid areas. Echo-guided needle biopsies were performed and a diagnosis of an atypical lipomatous tumor was established by pathological examination of the biopsy samples. Surgical resection was performed via a clamshell incision, enabling en bloc resection of this huge tumor. Although there was no invasion of surrounding organs, the left brachiocephalic vein was resected because it was circumferentially surrounded by tumor and could not be preserved. The tumor weighed 3500 g. Pathologic examination of the resected tumor resulted in a diagnosis of a biphasic tumor comprising dedifferentiated liposarcoma and non-adipocytic sarcoma with necrotic areas. The patient remains free of recurrent tumor 20 months postoperatively. Clamshell incision provides an excellent surgical field and can be performed safely in patients with huge mediastinal liposarcomas.

  5. Presternal soft tissue calcifications following mediastinal radiotherapy for Hodgkin's disease

    SciTech Connect

    Vainright, J.R.; Diaconis, J.N.; Haney, P.J.

    1987-01-01

    A patient is described in whom dense calcifications developed in the presternal soft tissues of the chest four years after mediastinal radiotherapy. This is believed to represent dystrophic calcification in previously normal but incidentally radiated tissues following standard dose radiotherapy to the mediastinum for stage IIA Hodgkin's disease.

  6. Unusual cause of right ventricular outflow tract compression: mediastinal lipomatosis.

    PubMed

    Bulakci, Mesut; Yahyayev, Aghakishi; Ucar, Adem; Erer, Burak; Erer, Betul; Dursun, Memduh

    2011-11-01

    Mediastinal lipomatosis (ML) is a benign condition characterized by the accumulation of mature adipose tissue within the mediastinum. ML is usually associated with Cushing syndrome and obesity. Most patients are asymptomatic, but some have thoracic pain, dyspnea, cough, dysphonia, dysphagia, and supraventricular tachycardia. We report a case of ML compressing the right ventricular outflow tract in a patient with Behçet disease.

  7. Vacuum-assisted closure for mediastinitis after pediatric cardiac surgery.

    PubMed

    Hiramatsu, Takeshi; Okamura, Yoshitaka; Komori, Shigeru; Nishimura, Yoshiharu; Suzuki, Hiroyuki; Takeuchi, Takashi

    2008-10-01

    Two children, aged 1 and 14 years with methicillin-resistant Staphylococcus aureus mediastinitis after pediatric open-heart surgery, were fitted with a vacuum-assisted closure system. Complete healing was achieved in both cases, and primary wound closure could be carried out without an omental flap after 6 and 16 days.

  8. [Posterior mediastinal thymus. Apropos of a case in a child].

    PubMed

    Canarelli, J P; Pautard, J C; Baratte, B; Doidy, L; Ricard, J

    1992-01-01

    Posterior and upper mediastinal localization of the thymus gland is uncommon in young children. Presenting symptoms and signs are very variable from incidental diagnosis to bronchopneumonial with atelectasias of the left upper Chest X-Ray and CT Scan are sufficient to make the diagnosis. Treatment of complicated forms with bronchial compression requires thoracotomy and surgical excision.

  9. [Experience of surgical treatment of huge mediastinal tumors].

    PubMed

    Li, Yuanbo; Zhang, Yi; Xu, Qingsheng; Su, Lei; Zhi, Xiuyi; Wang, Ruotian; Qian, Kun; Hu, Mu; Liu, Lei

    2014-09-23

    The diagnosis and surgical treatment of 36 huge mediastinal tumors were summarized in order to evaluate the effect and safety of the operation. Thirty-six huge mediastinal tumor patients treated in our department from June 2006 to June 2013 were retrospective analyzed, of whom clinical manifestations, diagnosis, surgical treatment and prognosis were carefully collected. Twenty-three cases were men and 13 were women. The average age was 39.2 years old. The pathology turned out to be benign in 23 cases and malignant in 13 cases. Complete resection was achieved in 34 cases while palliative resection in 2 cases with no perioperative death. Six cases had developed postoperative complications but all recovered after active treatment. Patients who had been diagnosed with benign tumors were all alive after follow-up periods of 6 months to 7 years. Nine malignat tumor patients developed recurrence or metastasis, including seven deaths. Surgery played a vital role in the diagnosis and treatment of huge mediastinal tumors. Preoperative diagnosis, accurate surgical approach and careful operation were the key to successful treatment. Benign huge mediastinal tumors had excellent prognosis with surgery.

  10. Acute eosinophilic pneumonia accompanied by mediastinal lymphadenopathy and thrombocytopenia.

    PubMed Central

    Esme, Hidir; Sahin, Onder; Sezer, Murat; Fidan, Fatma; Unlu, Mehmet

    2006-01-01

    Acute eosinophilic pneumonia, which was described in 1989, is thought to represent a hypersensitivity reaction to unidentified inhaled antigens. Here, we present a case of a marble mine worker with acute eosinophilic pneumonia complicated with mediastinal lymphadenopathy, neutrophilia, thrombocytopenia and acute respiratory distress syndrome. Images Figure 1 Figure 2 PMID:17128696

  11. [Rare case of congenital cystic adenomatoid malformation associated with polycystic kidney disease].

    PubMed

    Jabłoński, Janusz; Jankowski, Zbigniew; Sitkiewicz, Anna; Lewandowska, Małgorzata; Andrzejewska, Ewa

    2011-01-01

    Congenital cystic adenomatoid malformation (CCAM) is a rare pulmonary abnormality that results from aberrant fetal lung development. It about 4-26% of cases it can be associated with other congenital abnormalities. We describe a case of congenital cystic adenomatoid malformation 2 associated with polycystic kidney disease. The association of these two congenital malformations is exceptional. Only four similar cases have been reported in the literature. A 2-year-old girl was referred to the Department of Paediatric Surgery and Oncology Medical University of Lodz with pneumonia and left pneumothorax. For three weeks prior to referral the patient was treated with antibiotics. Chest x-ray revealed hyperinflation of left upper lobe with mediastinal shift to right. Computer tomographic scan of the lung revealed multiple cyst in the left upper lobe, left-site pneumothorax and mediastinal shift to the right. The patient underwent thoracotomy. Intraoperatively, multiple cysts in the left upper lobe were found and left upper lobectomy was performed. Histologic study was compatible with type 2 congenital cystic adenomatoid malformation. Ultrasound examination showed multilocular cysts in both kidneys. The dimensions of the cysts were: MWR4. 54x45x45 mm and 25x21x24 mm on the left and right sides, respectively. Significant increase in cyst size on the left side was observed. Ten months after first hospitalization resection of the cystic lower pole of the left kidney was performed. The presence of even a single renal cyst in a child with CCAM is an indication for further follow up examinations.

  12. Successful Treatment of Congenital Lymphangioma Circumscriptum of the Vulva with CO2 and Long-Pulsed Nd:YAG Lasers.

    PubMed

    Sasaki, Ryosuke; Negishi, Kei; Akita, Hirotaka; Suzuki, Kayoko; Matsunaga, Kayoko

    2014-01-01

    A 16-year-old girl presented with a 9-year history of vesicles on the vulva. She had initially taken a wait-and-see approach, but required treatment because of bleeding. Histological examination of a biopsied vesicle revealed dilated lymph channels in the upper dermis, suggesting lymphangioma circumscriptum (LC). The challenge for this pathology has been to find a conservative treatment with low morbidity and better results than those reported for surgical excision, which has been the mainstay of therapy. In this case, LC of the vulva was successfully treated using a 10,600-nm CO2 laser and long-pulsed Nd:YAG laser. Use of the 10,600-nm CO2 laser and long-pulsed Nd:YAG laser appeared effective for treating LC.

  13. Diagnosis and surgical treatment of mediastinal parathyroid tumors.

    PubMed Central

    Rothmund, M; Diethelm, L; Brünner, H; Kümmerle, F

    1976-01-01

    Experience and problems in the localization, diagnosis and surgical treatment of mediastinal parathyroid tumors are reported. Arteriography, pneumomediastinum and, especially, selective blood withdrawal with assay of parathyroid hormone, have proven valuable to the authors. Scintigram, intravital staining methods and venography are less productive. Retrosternal parathyroid tumors that can be removed from a Kocher incision should not, for practical reasons, be classified with the mediastinal tumors. The authors recommend the one-phase operation. If, after an intensive search of the neck and behind the sternum, no tumor has been found, it is advisable to incise the sternum step by step and revise the anterior mediastinum in the same session. Images Fig. 1a. Fig. 1b. Figs. 2 a and b. Fig. 3. PMID:1247311

  14. Atypical intermediate-grade mediastinal carcinoid. Case presentation.

    PubMed

    Carrillo-Muñoz, Araceli; Onofre-Borja, Melissa; Borrego-Borrego, Rafael; Chávez-Mercado, Leonora; Navarro-Reynoso, Francisco Pascual; Ibarra-Pérez, Carlos

    2011-01-01

    Approximately 25% of carcinoid tumors develop in the respiratory system. Neuroendocrine carcinoids represent ~5% of all mediastinal tumors and 1-5% of all intrathoracic neoplasms. They contain numerous neurosecretory granules that synthesize, store and release neurohumoral substances that can induce the carcinoid syndrome. A 21-year-old male presented with a rapidly progressive paraneoplastic syndrome unleashed by an acute urethritis. Two left mediastinal masses were identified and resected. Postoperative evolution has been uneventful during the first year. We emphasize the importance of early detection of primary and satellite lesions of these tumors including neurohumoral markers and PET/CT scans as in this case, as well as the participation of a multidisciplinary team.

  15. Giant Mediastinal Germ Cell Tumour: An Enigma of Surgical Consideration

    PubMed Central

    Ali, Nurayub Mohd; Azizan, Nornazirah; Zakaria, Andee Dzulkarnaen; Rahman, Mohd Ramzisham Abdul

    2016-01-01

    We present a case of 16-year-old male, who was referred from private centre for dyspnoea, fatigue, and orthopnea. The chest radiograph revealed complete opacification of left chest which was confirmed by computed tomography as a large left mediastinal mass measuring 14 × 15 × 18 cm. The diagnostic needle core biopsy revealed mixed germ cell tumour with possible combination of embryonal carcinoma, yolk sac, and teratoma. After 4 cycles of neoadjuvant BEP regime, there was initial response of tumour markers but not tumour bulk. Instead of classic median sternotomy or clamshell incision, posterolateral approach with piecemeal manner was chosen. Histology confirmed mixed germ cell tumour with residual teratomatous component without yolk sac or embryonal carcinoma component. Weighing 3.5 kg, it is one of the largest mediastinal germ cell tumours ever reported. We describe this rare and gigantic intrathoracic tumour and discuss the spectrum of surgical approach and treatment of this exceptional tumour. PMID:27807495

  16. Water-Clear Cell Adenoma of the Mediastinal Parathyroid Gland.

    PubMed

    Arik, Deniz; Dündar, Emine; Yilmaz, Evrim; Sivrikoz, Cumhur

    2017-10-06

    Water-clear cell adenoma of the parathyroid gland is a rare neoplasm that consists of cells with abundant clear-pink cytoplasm. There have only been 19 cases reported in the English literature. Here we report a case of water-clear cell adenoma of the mediastinal parathyroid gland. A 70-year-old male patient presented to the hospital with back pain and a mediastinal mass 6 cm in size was detected. After excision and microscopic evaluation, uniform, large clear cells with fine cytoplasmic vacuolization, without nuclear atypia, and arranged in solid and acinar patterns were revealed. The cells formed nests that were separated by fine fibrovascular septae and stained positively with anti-parathyroid hormone. To the best of our knowledge, this has not been previously reported in this location. In the differential diagnosis of clear cell lesions of the mediastinum, water-clear cell parathyroid adenoma should be considered.

  17. Water-Clear Cell Adenoma of the Mediastinal Parathyroid Gland.

    PubMed

    Arik, Deniz; Dündar, Emine; Yilmaz, Evrim; Sivrikoz, Cumhur

    2017-01-01

    Water-clear cell adenoma of the parathyroid gland is a rare neoplasm that consists of cells with abundant clear-pink cytoplasm. There have only been 19 cases reported in the English literature. Here we report a case of water-clear cell adenoma of the mediastinal parathyroid gland. A 70-year-old male patient presented to the hospital with back pain and a mediastinal mass 6 cm in size was detected. After excision and microscopic evaluation, uniform, large clear cells with fine cytoplasmic vacuolization, without nuclear atypia, and arranged in solid and acinar patterns were revealed. The cells formed nests that were separated by fine fibrovascular septae and stained positively with anti-parathyroid hormone. To the best of our knowledge, this has not been previously reported in this location. In the differential diagnosis of clear cell lesions of the mediastinum, water-clear cell parathyroid adenoma should be considered.

  18. Mediastinal lymphadenopathy in ampullary adenocarcinoma: not always metastatic

    PubMed Central

    Rao, Chalapathi A.S.; Rana, Surinder S.; Bhasin, Deepak K.; Nada, Ritambhra; Sampath, Santosh; Mittal, Bhagwant R.; Sharma, Vishal; Dhaliwal, Harpal S.; Singh, Kartar

    2012-01-01

    Malignancies can metastasize through hematogenous or lymphatic routes. Enlarged lymph nodes in a known case of malignancy do not always imply metastasis. A middle-aged female patient presented to us with abdominal pain and jaundice. Investigation revealed ampullary growth due to adenocarcinoma. Positron emission tomography-computerized tomography scan revealed uptake of the tracer in the ampullary region as well as in enlarged mediastinal lymph nodes. Endoscopic ultrasound-guided fine needle aspiration cytology of the mediastinal lymphadenopathy revealed it to be tuberculous. Mere radiologic evidence of a distant nodal spread must not be regarded as final evidence and obtaining a tissue diagnosis should be strongly considered, as potentially curative therapy may be offered. PMID:24714280

  19. Mediastinal lymphadenopathy in ampullary adenocarcinoma: not always metastatic.

    PubMed

    Rao, Chalapathi A S; Rana, Surinder S; Bhasin, Deepak K; Nada, Ritambhra; Sampath, Santosh; Mittal, Bhagwant R; Sharma, Vishal; Dhaliwal, Harpal S; Singh, Kartar

    2012-01-01

    Malignancies can metastasize through hematogenous or lymphatic routes. Enlarged lymph nodes in a known case of malignancy do not always imply metastasis. A middle-aged female patient presented to us with abdominal pain and jaundice. Investigation revealed ampullary growth due to adenocarcinoma. Positron emission tomography-computerized tomography scan revealed uptake of the tracer in the ampullary region as well as in enlarged mediastinal lymph nodes. Endoscopic ultrasound-guided fine needle aspiration cytology of the mediastinal lymphadenopathy revealed it to be tuberculous. Mere radiologic evidence of a distant nodal spread must not be regarded as final evidence and obtaining a tissue diagnosis should be strongly considered, as potentially curative therapy may be offered.

  20. Cystic Fibrosis: Prenatal Screening and Diagnosis

    MedlinePlus

    ... Management Education & Events Advocacy For Patients About ACOG Cystic Fibrosis: Prenatal Screening and Diagnosis Home For Patients Search ... Screening and Diagnosis FAQ171, June 2017 PDF Format Cystic Fibrosis: Prenatal Screening and Diagnosis Pregnancy What is cystic ...

  1. 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

  2. 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

  3. Infected mediastinal bronchogenic cyst in a 12 year old girl.

    PubMed

    Schweigert, M; Wolf, F; Stadlhuber, R J; Ficker, J H; Stein, H J

    2012-04-01

    Bronchogenic cysts are an uncommon congenital malformation deriving from the primitive foregut. They are mainly unilocular, and respiratory distress is the most common presentation in pediatric patients. We describe the case of a 12-year-old girl with a huge infected mediastinal bronchogenic cyst which was resected via an axillary muscle-sparing thoracotomy. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  4. Cervicofacial and mediastinal emphysema due to a dental procedure

    PubMed Central

    Ramnarine, Mityanand; Dubin, Zvi

    2017-01-01

    Dental procedures, though commonplace and usually very safe, are not without complications. We report on a case of extensive cervicofacial and mediastinal emphysema after a routine dental procedure, initially masquerading as an allergic reaction in an otherwise young and healthy woman. A review of the relevant literature on this clinical entity is presented, which serves to underscore the need for awareness by the treating clinician of this condition and its potential sequela. PMID:28243011

  5. Combined endovascular treatment of aorto-oesophageal fistula with mediastinitis

    PubMed Central

    Berna, Pascal; Pequignot, Aurélien; De Temmerman, Pierre; De Dominicis, Florence

    2013-01-01

    False aortic aneurysm is an uncommon complication after oesophageal perforation and results in a high rate of mortality. A 63-year-old patient presented with acute chest pain. Biochemical tests (cardiac enzymes) and electrocardiogram were normal. A thoracic and abdominal CT scan was performed, and showed a foreign body in the posterior mediastinum, with mediastinal cellulitis and a false aortic aneurysm. Surgical endovascular management was performed, with stenting of the thoracic aorta and oesophageal exploration. PMID:23042800

  6. Management of locally advanced primary mediastinal synovial sarcoma

    PubMed Central

    Chatterjee, Ambarish S; Kumar, Rajiv; Purandare, Nilendu; Jiwnani, Sabita; Karimundackal, George; Pramesh, CS

    2017-01-01

    Primary mediastinal synovial sarcoma (PMSS) is a relatively rare disease, and patients are treated predominantly with surgery for resectable disease. Management of locally advanced borderline resectable and unresectable PMSS is not only challenging but also lacks standard guidelines. We present three patients with PMSS, who were unresectable or borderline resectable at presentation and were treated with neoadjuvant chemotherapy followed by surgery and postoperative radiotherapy. PMID:28360472

  7. Primary mediastinal lymphomas, their morphological features and comparative evaluation

    PubMed Central

    Aggarwal, Riti; Rao, Seema; Dhawan, Shashi; Bhalla, Sunita; Kumar, Arvind; Chopra, Prem

    2017-01-01

    Background: Primary mediastinal lymphoma is an uncommon tumor. Hodgkin's lymphoma (HL), primary mediastinal B-cell lymphoma (PMBCL), and T-lymphoblastic lymphoma are the most common primary mediastinal lymphomas. Key morphological and immunohistochemistry (IHC) features play a very crucial role in diagnosis as well as further categorization. Materials and Methods: In this study, the morphological spectrum and histological features of 32 cases of primary mediastinal lymphomas diagnosed over 5 years were studied and morphological and IHC features of PMBCL versus HL were compared. Features of PMBCL were also compared against a control group of systemic diffuse large B-cell lymphoma. Results: Although PMBCL and HL are known to show overlapping morphological features, it was observed that presence of clear cells and compartmentalizing fibrosis in PMBCL; and classical Reed–Sternberg cells and dense inflammatory background in HL are important morphological clues while evaluating the biopsies. PMBCL showed diffuse, strong and uniform CD20 positivity; whereas CD30 showed focal/patchy, weak to moderate and heterogeneous expression, wherever found positive. As against this, HL showed diffuse, strong and uniform CD30 positivity; and focal/patchy, weak to moderate and heterogeneous CD20 expression, if found positive. CD20, CD3, and CD30 were sufficient in most of the cases while diagnosing PMBCL and HL. Conclusion: This study emphasizes the critical examination of IHC markers. Only positive expression in neoplastic cells is not sufficient to make a diagnosis, equal importance should be given to percentage, intensity, pattern, and type of positivity. Apart from basic IHC described above; CD15, leukocyte common antigen and fascin played an important role in differentiating HL and PMBCL in select doubtful cases. PMID:28144055

  8. Cervicofacial and mediastinal emphysema due to a dental procedure.

    PubMed

    Ramnarine, Mityanand; Dubin, Zvi

    2017-01-01

    Dental procedures, though commonplace and usually very safe, are not without complications. We report on a case of extensive cervicofacial and mediastinal emphysema after a routine dental procedure, initially masquerading as an allergic reaction in an otherwise young and healthy woman. A review of the relevant literature on this clinical entity is presented, which serves to underscore the need for awareness by the treating clinician of this condition and its potential sequela.

  9. Minimalist video-assisted thoracic surgery biopsy of mediastinal tumors

    PubMed Central

    Tacconi, Federico; Rogliani, Paola; Cristino, Benedetto; Gilardi, Francesco; Palombi, Leonardo

    2016-01-01

    Background Mediastinal tumors often require surgical biopsy to achieve a precise and rapid diagnosis. However, subjects with mediastinal tumors may be unfit for general anesthesia, particularly when compression of major vessels or airways does occur. We tested the applicability in this setting of a minimalist (M) uniportal, video-assisted thoracic surgery (VATS) strategy carried out under locoregional anesthesia in awake patients (MVATS). Methods We analyzed in a comparative fashion including propensity score matching, data from a prospectively collected database of patients who were offered surgical biopsy for mediastinal tumors through either MVATS or standard VATS. Tested outcome measures included feasibility, diagnostic yield, and morbidity. Results A total of 24 procedures were performed through MVATS. Diagnostic yield was 100%. Median hospital stay and time interval to oncologic treatment were 2 days (IQR, 2–3 days) and 7 days (IQR, 5.5–11.5 days), respectively. At overall comparison (MVATS, N=24 vs. VATS, N=23), there was a significant difference in both frequency and severity of postoperative complication as measured by Clavien-Dindo classification (P<0.006). In a propensity score matched comparison (8 patients per group), grade 3 or 4 complications requiring aggressive management were found only in the general anesthesia group. Global time spent in the operating room was shorter in the MVATS group (P=0.05). Time interval to oncological treatment was the same between groups. Other differences were also found in SIRS score (P=0.05) and PaO2/FiO2 (P=0.04) thus suggesting better adaption to perioperative stress. Conclusions MVATS biopsy appears to be a reliable tool to optimize diagnostic assessment in patients with mediastinal tumors. It can offer high diagnostic accuracy due to large tissue samples, while reducing morbidity rate compared to the same operation under general anesthesia. More robust evaluation is needed to define the appropriateness of

  10. Benign cystic peritoneal mesothelioma.

    PubMed Central

    Bhandarkar, D S; Smith, V J; Evans, D A; Taylor, T V

    1993-01-01

    The well defined but rare entity of benign cystic mesothelioma is reported. The aetiology of this neoplasm remains obscure. The presenting features make a precise preoperative diagnosis difficult; information provided by computed tomography and cytology may help. A firm diagnosis can only come from electron microscopic or immunohistochemical examination of the tumour. Diagnostic accuracy and diligent follow up are essential because, although the tumour is considered benign, it does tend towards local recurrence. Images PMID:8227441

  11. 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

  12. [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. Copyright © 2013. Published by Elsevier Masson SAS.

  13. [Kartagener syndrome with lung cancer and mediastinal tumor].

    PubMed

    Horie, Masafumi; Arai, Hidenori; Noguchi, Satoshi; Suzuki, Masaru; Sakamoto, Yoshio; Oka, Teruaki

    2010-05-01

    A 71-year-old man was admitted to Kanto Central Hospital with hemoptysis. He had had chronic sinusitis and deafness since childhood. Situs inversus, bronchiectasia, and diffuse panbronchiolitis had been also diagnosed at the age of 59. Chest computed tomography demonstrated a 5-cm mass in the anterior mediastinum as well as a 4-cm mass in the upper lobe of the right lung. A transbronchial lung biopsy of the right lung tumor revealed squamous cell carcinoma. Electron microscopic examination of the bronchial epithelial cilia revealed a total defect of both inner and outer dynein arms, leading to a diagnosis of primary ciliary dyskinesia. Biopsy of the mediastinal tumor was not performed. After concurrent chemoradiation therapy, the lung cancer decreased in size partial remission (PR) and the mediastinal tumor disappeared complete remission (CR). Later, a cavity formed in the tumor, where a Pseudomonas aeruginosa infection occurred. He died 1 year after the diagnosis of lung cancer was established. There have been 5 reported cases of Kartagener syndrome complicated with lung cancer, but to the best of our knowledge there have been no reports of Kartagener syndrome with mediastinal tumor.

  14. Mediastinitis in pediatric cardiac surgery: Prevention, diagnosis and treatment

    PubMed Central

    Durandy, Yves

    2010-01-01

    In spite of advances in the management of mediastinitis following sternotomy, mediastinitis is still associated with significant morbidity. The prognosis is much better in pediatric surgery compared to adult surgery, but the prolonged hospital stays with intravenous therapy and frequent required dressing changes that occur with several therapeutic approaches are poorly tolerated. Prevention includes nasal decontamination, skin preparation, antibioprophylaxis and air filtration in the operating theater. The expertise of the surgical team is an additional factor that is difficult to assess precisely. Diagnosis is often very simple, being made on the basis of a septic state with wound modification, while retrosternal puncture and CT scan are rarely useful. Treatment of mediastinitis following sternotomy is always a combination of surgical debridement and antibiotic therapy. Continued use of numerous surgical techniques demonstrates that there is no consensus and the best treatment has yet to be determined. However, we suggest that a primary sternal closure is the best surgical option for pediatric patients. We propose a simple technique with high-vacuum Redon’s catheter drainage that allows early mobilization and short term antibiotherapy, which thus decreases physiological and psychological trauma for patients and families. We have demonstrated the efficiency of this technique, which is also cost-effective by decreasing intensive care and hospital stay durations, in a large group of patients. PMID:21179306

  15. 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.

  16. Rapid onset mediastinal hematoma due to vertebral fracture and review of relevant literature.

    PubMed

    Koksal, Vaner; Coskun, Selcuk; Coskun, Pinar Koksal

    2015-12-01

    Patients with vertebral fractures are frequently encountered and those with thoracic and lumbar spine fractures are likely to have associated injuries. Detection of a widened mediastinum after trauma is very nonspecific and most of the time it is related to aortic injury or mediastinal hematoma. Vertebral or sternal fractures can also be the cause of mediastinal hematoma with or without aortic injury. This report reviews an unusual case of rapid onset mediastinal hematoma due to vertebral fracture after a fall. In the case, there was a mediastinal hematoma adjacent to a burst fracture of the T8 vertebral body. There was a rapid increase in identified hematoma during the emergency follow up and urgent erythrocyte transfusion was carried out. We would like to raise awareness of this infrequent presentation of mediastinal hematoma, as it is insidious and possibly fatal. In the evaluation of mediastinal hematoma, the detection of osseous injuries is a requirement.

  17. Cystic fibrosis and coeliac disease

    PubMed Central

    Goodchild, Mary C.; Nelson, R.; Anderson, Charlotte M.

    1973-01-01

    Two children, unrelated, in whom cystic fibrosis had been diagnosed at the ages of 14 weeks and 10 months, respectively, were found subsequently to suffer from coeliac disease as well. Both children are responding well to dietary gluten withdrawal and to conventional treatment for cystic fibrosis. It is suggested that cystic fibrosis may predispose to the development of coeliac disease. Therefore, jejunal biopsy may be a useful investigation in the occasional child with cystic fibrosis, who presents with unusual features, and who fails to thrive as well as expected. ImagesFIG. 2FIG. 3FIG. 5FIG. 6 PMID:4517654

  18. Neonatal cystic fibrosis screening test

    MedlinePlus

    Cystic fibrosis screening - neonatal; Immunoreactive trypsinogen; IRT test; CF - screening ... better nutrition, growth, and lung function. This screening test helps doctors identify children with CF before they ...

  19. 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

  20. Prone-position thoracoscopic resection of posterior mediastinal lymph node metastasis from rectal cancer.

    PubMed

    Shirakawa, Yasuhiro; Noma, Kazuhiro; Koujima, Takeshi; Maeda, Naoaki; Tanabe, Shunsuke; Ohara, Toshiaki; Fujiwara, Toshiyoshi

    2015-02-12

    Mediastinal lymph node metastasis from colorectal cancer is rare, and barely any reports have described resection of this pathology. We report herein a successful thoracoscopic resection of mediastinal lymph node metastasis in a prone position. A 65-year-old man presented with posterior mediastinal lymph node metastasis after resection of the primary rectal cancer and metachronous hepatic metastasis. Metastatic lymph nodes were resected completely using thoracoscopic surgery in the prone position, which provided advantages of minimal invasiveness, good surgical field, and reduced ergonomic burden on the surgeon. Thoracoscopic resection in the prone position was thought to have the potential to become the standard procedure of posterior mediastinal tumors.

  1. [How can the anatomic mediastinal extension of the tumor be estimated? Endoscopic evaluation].

    PubMed

    Dumon, J F

    1992-01-01

    Tracheobronchial endoscopy is more particularly designed for the exploration of endotracheal and bronchial lesions. However, involvements of the mediastinum can be suggested or demonstrated on the basis of generally indirect signs. The most classical example is the paralysis of the left vocal chord, which expresses a para- or subaortic mediastinal extension. Tracheobronchial endoscopy has an interesting but not determining role in the appreciation of the mediastinal extension of non-small-cell bronchial cancers. It most often allows guiding complementary radiological examinations, and sometimes provides histological evidence of mediastinal extension. In some cases, esophageal endoscopy can demonstrate mediastinal extension, if a tracheoesophageal tumor is discovered.

  2. 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.

  3. Multilocular Cystic Renal Cell Carcinoma or Cystic Nephroma?

    PubMed Central

    Cortez-Betancourt, Roberto; Alías-Melgar, Alejandro; Botello-Gómez, Pedro Jair; Ramírez-Garduño, Emilio; Trujillo-Vázquez, Eric Iván; Torres-Santos, Yosimart; Mata-Martínez, José Antonio; Carreño- de la Rosa, Fernando

    2016-01-01

    The incidence of Multilocular cystic renal cell carcinoma (MCRCC) in literature is very low and confounding MCRCC with cystic nephroma (CN) is even more unusual. The aim of this report is to present a case of MCRCC and emphasize the importance of the preoperative radiologic evaluation and immunohistochemical staining confirmation to obtain an accurate diagnosis. A 73-year-old woman presented with a history of 4-month right flank pain. CT showed a Bosniak type III renal mass. After laparoscopic partial nephrectomy the initial report was cystic nephroma. Immunohistochemical staining was performed being positive for Epithelial Membrane Antigen thus changing the diagnosis to MCRCC. Multilocular cystic renal cell carcinoma cannot reliably be distinguished from cystic nephroma neither by physical examination nor by radiologic evaluation; immunohistochemical staining assay is useful to differentiate between these conditions allowing an accurate diagnosis and proper follow-up. PMID:28074169

  4. Cystic Fibrosis: Diet and Nutrition

    MedlinePlus

    ... Getting an X-ray Cystic Fibrosis: Diet and Nutrition KidsHealth > For Kids > Cystic Fibrosis: Diet and Nutrition Print A A A What's in this article? ... is someone who knows all about food and nutrition. Each kid is different, but most kids with ...

  5. Cystic Fibrosis: Diet and Nutrition

    MedlinePlus

    ... in the Operating Room? Cystic Fibrosis: Diet and Nutrition KidsHealth > For Kids > Cystic Fibrosis: Diet and Nutrition A A A What's in this article? CF ... is someone who knows all about food and nutrition. Each kid is different, but most kids with ...

  6. [Cystic fibrosis in 2008].

    PubMed

    Durieu, I; Josserand, R Nove

    2008-11-01

    To describe the epidemiological, physiopathological, clinical and therapeutic knowledge concerning cystic fibrosis (CF). Important modifications in the health organization of the care concerning this orphan disease have been implemented in France. The life expectancy has dramatically increased, as well as the knowledge concerning the pathological structure and function of the CFTR gene and protein. This will lead to the development of emerging drug treatments for this lethal disease. The life expectancy is predicted to exceed 40 years for children born in the 2000s. As a result, there has been a tremendous growth of the adult population that reached 40% of the overall approximately 5000 patients included in the CF French registry (Observatoire National de la Mucoviscidose). Lung disease remains the primary cause of morbidity and mortality. The characteristic phenotypic presentation associates bronchial and rhinosinusal symptoms, pancreatic insufficiency and liver disease. Bronchial damage leads to progressive chronic respiratory insufficiency. Diabetes mellitus and osteoporosis frequently appears in adulthood. Neonatal screening has been implemented in France since 2002. It will prevent delayed diagnosis and its deleterious consequences. Some atypical cases of CF presenting only with one or two organ system involvement can be diagnosed in adulthood. Isolated chronic rhinosinusitis, bronchiectasis, congenital bilateral absence of vas deferens, recurrent pancreatitis, allergic bronchopulmonary aspergillosis, and some case of cholangitis may so revealed late form of cystic fibrosis. The health care is organized in cystic fibrosis centres. Despite gene discovery, treatment still remains symptomatic, based on intensive pulmonary and nutritional treatments. Challenges for new treatments are to correct the basic defect, either by gene therapy or by pharmacological modulation of the abnormal physiological processes.

  7. Screening for cystic fibrosis.

    PubMed Central

    Dodge, J A; Ryley, H C

    1982-01-01

    Practicable methods are now available for whole population screening of neonates for cystic fibrosis. Although diagnosis and treatment of the disease from birth has not yet been unequivocally shown to improve prognosis, existing evidence suggests that this is likely. Further ethical reasons are proposed in support of neonatal diagnosis and early treatment. The development of tests for prenatal diagnosis and carrier detection is under active investigation, and raises ethical problems for heterozygotes and their medical advisers. The heavy financial and emotional burden this disease imposes on the patient and the family should not be underestimated when policy decisions are made. PMID:7138066

  8. Cystic fibrosis and sleep.

    PubMed

    Katz, Eliot S

    2014-09-01

    Sleep disturbances are frequently observed in cystic fibrosis (CF). The resultant sleep fragmentation, short sleep duration, and gas-exchange abnormalities are postulated to contribute to the neurocognitive, cardiovascular, and metabolic abnormalities associated with CF. There are no outcomes data to establish the optimal procedure for screening and treating CF patients for sleep-related respiratory abnormalities. Therapy with supplemental oxygen and bilevel ventilation are widely considered to be effective in the short term, but there are few evidence-based data to support long-term improvements in morbidity and mortality. Copyright © 2014 Elsevier Inc. All rights reserved.

  9. Cystic fibrosis since 1938.

    PubMed

    Davis, Pamela B

    2006-03-01

    Cystic fibrosis (CF) was distinguished from celiac disease in 1938. Then, it was a pathologic diagnosis, life expectancy was approximately 6 months, and the autosomal recessive disease was believed to arise from abnormal mucus plugging exocrine ducts. Death often occurred from lung infection. Discovery of the sweat electrolyte defect in 1953 and standardization of the sweat test in 1959 allowed identification of milder cases, and CF was no longer considered only a disorder of mucus. In 1955, establishment of centers with programs of aggressive, comprehensive care initiated striking improvement in longevity. The pillars of care established then (attention to nutrition, airway clearance, treatment of lung infection) remain today. In 1983, chloride transport was identified as the basic physiologic CF defect, accompanied by increased sodium reabsorption. In 1980, we learned that inflammation contributes independently to lung disease and constitutes an independent therapeutic target. In 1989, the discovery of the CF gene demonstrated the basic defect to be in a cAMP-regulated chloride channel. This afforded new diagnostic tests, opportunities for research, and prospects for using the gene as therapy. Since then, substantial advances in basic and clinical research catalyzed therapeutic improvements: median survival age now exceeds 30 years. The Cystic Fibrosis Foundation center network provides not only opportunity to conduct clinical trials but also means to disseminate new therapies. In the future, treatments directed at the basic defect can be expected, with concomitant improvements in morbidity and mortality.

  10. Cystic fibrosis in Uruguay.

    PubMed

    Luzardo, Gerardo; Aznarez, Isabel; Crispino, Beatriz; Mimbacas, Adriana; Martínez, Liria; Poggio, Rossana; Zielenski, Julian; Tsui, Lap-Chee; Cardoso, Horacio

    2002-03-31

    We conducted clinical and genetic analyses of 52 cystic fibrosis (CF) patients in Uruguay, which is about half of the known affected individuals in the country. A relatively high proportion had a mild presentation, characterized by pancreatic sufficiency (28%), a strong pulmonary component (97%), and borderline sweat electrolyte measurements (25%). Mutational analysis of CF chromosomes demonstrated a relatively low incidence of the DeltaF508 allele (40%) and a large number of other cystic fibrosis conductance regulator mutations, with an overall detection rate of about 71%. Fifteen different mutations were detected in our patients: DeltaF508, G542X, R1162X, G85E, N1303K, R334W, R75Q, R74W, D1270N, W1282X, DeltaI507, 2789+5G-->A, R1066C, -816C/T, R553X, as well as RNA splicing variant IVS8-5T. This group of Uruguayan CF patients has some characteristics in common with other populations of similar origin (Hispanics), as well as some unique characteristics.

  11. Cystic fibrosis related diabetes.

    PubMed

    O'Shea, Donal; O'Connell, Jean

    2014-08-01

    Improved life expectancy in cystic fibrosis (CF) has led to an expanding population of adults with CF, now representing almost 50 % of the total CF population. This creates new challenges from long-term complications such as diabetes mellitus (DM), a condition that is present in 40 %-50 % of adults with CF. Cystic fibrosis-related diabetes (CFRD) results from a primary defect of insulin deficiency and although sharing features with type 1 (DM1) and type 2 diabetes (DM2), it is a clinically distinct condition. Progression to diabetes is associated with poorer CF clinical outcomes and increased mortality. CFRD is not associated with an increased risk of cardiovascular disease and the prevalence of microvascular complications is lower than DM1 or DM2. Rather, the primary goal of insulin therapy is the preservation of lung function and optimization of nutritional status. There is increasing evidence that appropriate screening and early intervention with insulin can reverse weight loss and improve pulmonary function. This approach may include targeting postprandial hyperglycemia not detected by standard diagnostic tests such as the oral glucose tolerance test. Further clinical research is required to guide when and how much to intervene in patients who are already dealing with the burden of one chronic illness.

  12. Laparoscopic retrosternal gastric pull-up for fistulized mediastinal mass

    PubMed Central

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

    2017-01-01

    We describe the case of a patient successfully reconstructed with laparoscopic retrosternal gastric pull-up after esophagectomy for unresectable posterior mediastinal inflammatory myofibroblastic tumor, eroding into the esophagus and compressing the airways. A partial esophagectomy with esophagostomy was performed for treatment of esophageal pleural fistula and empyema, while the airways were managed with the placement of an endobronchial stent. Gastrointestinal reconstruction was performed using a laparoscopic approach to create a retrosternal tunnel for gastric conduit pull-up and cervical anastomosis. The patient was discharged uneventfully after 6 d, and has done very well at home with normal diet.

  13. [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.

  14. 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.

  15. Cervical and mediastinal emphysema associated with an asthma exacerbation.

    PubMed

    Khan, Waseem Asrar; Abbas, Shoneen; Bright, John

    2013-02-18

    Surgical emphysema associated with an acute asthma exacerbation is very rare. This report presents the case of a 19- year-old male patient with a background of asthma who presented with palpable cervical surgical emphysema and CT evidence of mediastinal emphysema. There are only a limited number of case reports associated with surgical emphysema in the absence of pneumothorax in patients with an asthma exacerbation. Evidence with regard to the management of such cases is limited and is largely consensus based. Below we discuss the case in a greater detail.

  16. Nasal Methicillin-Resistant S. Aureus is a Major Risk for Mediastinitis in Pediatric Cardiac Surgery

    PubMed Central

    2015-01-01

    Background: Mediastinitis caused by methicillin-resistant Staphylococcus aureus (MRSA) is a serious complication after pediatric cardiac surgery. An outbreak of surgical site infections (SSIs) provided the motivation to implement SSI prevention measures in our institution. Methods: Subjects comprised 174 pediatric patients who underwent open-heart surgery after undergoing preoperative nasal culture screening. The incidence of SSIs and mediastinitis was compared between an early group, who underwent surgery before SSI measures (Group E, n = 73), and a recent group, who underwent surgery after these measures (Group R, n = 101), and factors contributing to the occurrence of mediastinitis were investigated. Results: The incidence of both SSIs and Mediastinitis has significantly decreased after SSI measures. With regard to factors that significantly affected mediastinitis, preoperative factors were “duration of preoperative hospitalization” and “preoperative MRSA colonization,” intraoperative factors were “Aristotle basic complexity score,” “operation time,” “cardiopulmonary bypass circuit volume” and “lowest rectal temperature.” And postoperative factor was “blood transfusion volume.” Patients whose preoperative nasal cultures were MRSA-positive suggested higher risk of MRSA mediastinitis. Conclusions: SSI prevention measures significantly reduced the occurrence of SSIs and mediastinitis. Preoperative MRSA colonization should be a serious risk factor for mediastinitis following pediatric cardiac surgeries. PMID:25641035

  17. Nasal methicillin-resistant S. aureus is a major risk for mediastinitis in pediatric cardiac surgery.

    PubMed

    Katayanagi, Tomoyuki

    2015-01-01

    Mediastinitis caused by methicillin-resistant Staphylococcus aureus (MRSA) is a serious complication after pediatric cardiac surgery. An outbreak of surgical site infections (SSIs) provided the motivation to implement SSI prevention measures in our institution. Subjects comprised 174 pediatric patients who underwent open-heart surgery after undergoing preoperative nasal culture screening. The incidence of SSIs and mediastinitis was compared between an early group, who underwent surgery before SSI measures (Group E, n = 73), and a recent group, who underwent surgery after these measures (Group R, n = 101), and factors contributing to the occurrence of mediastinitis were investigated. The incidence of both SSIs and Mediastinitis has significantly decreased after SSI measures. With regard to factors that significantly affected mediastinitis, preoperative factors were "duration of preoperative hospitalization" and "preoperative MRSA colonization," intraoperative factors were "Aristotle basic complexity score," "operation time," "cardiopulmonary bypass circuit volume" and "lowest rectal temperature." And postoperative factor was "blood transfusion volume." Patients whose preoperative nasal cultures were MRSA-positive suggested higher risk of MRSA mediastinitis. SSI prevention measures significantly reduced the occurrence of SSIs and mediastinitis. Preoperative MRSA colonization should be a serious risk factor for mediastinitis following pediatric cardiac surgeries.

  18. Computed tomography evaluation of bilateral bronchostenosis caused by sclerosing granulomatous mediastinitis: a complication of histoplasmosis.

    PubMed

    Shin, M S; Ho, K J

    1984-10-01

    A proved case of histoplasmosis with sclerosing granulomatous mediastinitis complicated by severe bilateral bronchostenosis in a 28-year-old woman is reported. The case demonstrates that this relatively benign disease can lead to grave consequences and that computed tomography is extremely useful in the evaluation of this mediastinal lesion, particularly when plain chest radiography is noncontributory.

  19. A three-dimensional mediastinal model created with rapid prototyping in a patient with ectopic thymoma.

    PubMed

    Akiba, Tadashi; Nakada, Takeo; Inagaki, Takuya

    2015-01-01

    Preoperative three-dimensional (3D) imaging of a mediastinal tumor using two-dimensional (2D) axial computed tomography is sometimes difficult, and an unexpected appearance of the tumor may be encountered during surgery. In order to evaluate the preoperative feasibility of a 3D mediastinal model that used the rapid prototyping technique, we created a model and report its results. The 2D image showed some of the relationship between the tumor and the pericardium, but the 3D mediastinal model that was created using the rapid prototyping technique showed the 3D lesion in the outer side of the extrapericardium. The patient underwent a thoracoscopic resection of the tumor, and the pathological examination showed a rare middle mediastinal ectopic thymoma. We believe that the construction of mediastinal models is useful for thoracoscopic surgery and other complicated surgeries of the chest diseases.

  20. A Three-Dimensional Mediastinal Model Created with Rapid Prototyping in a Patient with Ectopic Thymoma

    PubMed Central

    Nakada, Takeo; Inagaki, Takuya

    2014-01-01

    Preoperative three-dimensional (3D) imaging of a mediastinal tumor using two-dimensional (2D) axial computed tomography is sometimes difficult, and an unexpected appearance of the tumor may be encountered during surgery. In order to evaluate the preoperative feasibility of a 3D mediastinal model that used the rapid prototyping technique, we created a model and report its results. The 2D image showed some of the relationship between the tumor and the pericardium, but the 3D mediastinal model that was created using the rapid prototyping technique showed the 3D lesion in the outer side of the extrapericardium. The patient underwent a thoracoscopic resection of the tumor, and the pathological examination showed a rare middle mediastinal ectopic thymoma. We believe that the construction of mediastinal models is useful for thoracoscopic surgery and other complicated surgeries of the chest diseases. PMID:24633133

  1. Cystic tumors of the pancreas

    PubMed Central

    Morana, Giovanni; Guarise, Alessandro

    2006-01-01

    Cystic tumors of the pancreas are less frequent than solid lesions and are often detected incidentally, as many of these lesions are small and asymptomatic. However, they may be associated with pancreatitis or have malignant potential. With advancements in diagnostic imaging, cystic lesions of the pancreas are being detected with increasing frequency. Many lesions can cause a pancreatic cyst, most being non-neoplastic while approximately 10% are cystic tumors, ranging from benign to highly malignant tumors. With increasing experience it is becoming clear that the prevalence of pseudocyst among cystic lesions of the pancreas is lower than usually presumed. A presumptive diagnosis of pseudocyst based on imaging appearance alone can cause a diagnostic error, and neoplastic cysts of the pancreas are particularly susceptible to this misdiagnosis, which can result in inappropriate treatment. Cystic tumors of the pancreas are formed by serous or mucinous structures showing all stages of cellular differentiation. According to the WHO classification, they can be subdivided on the basis of their histological type and biological behavior into benign tumors, borderline tumors, and malignant tumors. Cystic pancreatic tumors can be subdivided into peripheral (serous cystadenomas, mucinous cystic tumors, solid and papillary epithelial neoplasms, cystic islet cell tumors), which do not communicate with the main pancreatic duct, and ductal tumors (mucinous tumor), according to their site of origin. On the basis of imaging criteria alone, it can be very difficult to differentiate non-tumoral cystic lesions from neoplastic ones. The management of these patients is complex, and it is important to correlate imaging findings with knowledge of the patient’s symptoms and of the natural history and predictors of malignancy in pancreatic cysts. PMID:16861136

  2. 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

  3. Nutritional Issues in Cystic Fibrosis.

    PubMed

    Solomon, Missale; Bozic, Molly; Mascarenhas, Maria R

    2016-03-01

    The importance of maintaining adequate nutrition in patients with cystic fibrosis has been well known for the past 3 decades. Achieving normal growth and maintaining optimal nutrition is associated with improved lung function. Comprehensive and consistent nutritional assessments at regular intervals can identify those at risk of nutritional failure and uncover micronutrient deficiencies contributing to malnutrition. Management of malnutrition in cystic fibrosis should follow a stepwise approach to determine the causes and comorbidities and to develop a nutritional plan. Nutritional management is crucial at every stage in a person's life with cystic fibrosis and remains a cornerstone of management.

  4. A bizarre abdominal cystic lesion.

    PubMed

    Zucchini, Giorgia; Pezzilli, Raffaele; Ricci, Claudio; Casadei, Riccardo; Santini, Donatella; Calculli, Lucia; Corinaldesi, Roberto

    2010-09-06

    In spite of careful intraoperative precautions and gauze counts, mistakes can still occur during surgery. In the case reported, a retained gauze leaved during a surgical approach for removing a solid-cystic papillary tumor localized in the pancreatic tail, caused both persistent abdominal discomfort and the presence of an abdominal cystic lesion at imaging techniques. When a previous operative history is present, a foreign body should be taken into account in the differential diagnosis of a patient with an intra-abdominal cystic mass. Finally, radio-opaque marker should be routinely used by surgeons in order to reach a correct diagnosis in operated patients having retained gauze.

  5. 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

  6. DiGeorge syndrome who developed lymphoproliferative mediastinal mass.

    PubMed

    Kim, Kyu Yeun; Hur, Ji Ae; Kim, Ki Hwan; Cha, Yoon Jin; Lee, Mi Jung; Kim, Dong Soo

    2015-03-01

    DiGeorge syndrome is an immunodeficient disease associated with abnormal development of 3rd and 4th pharyngeal pouches. As a hemizygous deletion of chromosome 22q11.2 occurs, various clinical phenotypes are shown with a broad spectrum. Conotruncal cardiac anomalies, hypoplastic thymus, and hypocalcemia are the classic triad of DiGeorge syndrome. As this syndrome is characterized by hypoplastic or aplastic thymus, there are missing thymic shadow on their plain chest x-ray. Immunodeficient patients are traditionally known to be at an increased risk for malignancy, especially lymphoma. We experienced a 7-year-old DiGeorge syndrome patient with mediastinal mass shadow on her plain chest x-ray. She visited Severance Children's Hospital hospital with recurrent pneumonia, and throughout her repeated chest x-ray, there was a mass like shadow on anterior mediastinal area. We did full evaluation including chest computed tomography, chest ultrasonography, and chest magnetic resonance imaging. To rule out malignancy, video assisted thoracoscopic surgery was done. Final diagnosis of the mass which was thought to be malignancy, was lymphoproliferative lesion.

  7. Clinical Manifestations of Fibrosing Mediastinitis in Chinese Patients

    PubMed Central

    Hu, Yan; Qiu, Jian-Xing; Liao, Ji-Ping; Zhang, Hong; Jin, Zhe; Wang, Guang-Fa

    2016-01-01

    Background: Fibrosing mediastinitis (FM) is a rare disease. FM is thought to be related to prior granulomatous mediastinal infection, such as histoplasmosis or tuberculosis. The majority of cases have been reported in endemic regions for histoplasmosis. The characteristics of cases of FM in China, where the prevalence of tuberculosis is high, have not been reported. We analyzed the clinical, imaging, and bronchoscopic features of Chinese patients with FM to promote awareness of this disease. Methods: Between January 2005 and June 2015, twenty patients were diagnosed with FM in our hospital. Medical records and follow-up data were collected. Imaging and biopsy findings were reviewed by radiologists and pathologists. Results: A total of 20 patients were analyzed (8 males and 12 females). The age ranged from 43 to 88 years with a mean age of 69.5 years. Previous or latent tuberculosis was found in 12 cases. Clinical symptoms included dyspnea (18/20), cough (17/20), expectoration (7/20), and recurrent pneumonia (3/20). Chest computed tomography scans showed a diffuse, homogeneous, soft tissue process throughout the mediastinum and hila with compression of bronchial and pulmonary vessels. Calcification was common (15/20). Pulmonary hypertension was present in 9 of 20 cases. Diffuse black pigmentation in the bronchial mucosa was frequently seen on bronchoscopy (12/13). The patients’ response to antituberculosis treatment was inconsistent. Conclusions: FM in Chinese patients is most likely associated with tuberculosis. Some characteristics of FM are different from cases caused by histoplasmosis. PMID:27824002

  8. All that wheezes: A young infant with a mediastinal mass

    PubMed Central

    Tong, Timothy; Doski, John J

    2014-01-01

    Wheezing infants are frequently encountered in the emergency department. Bronchiolitis is the most commonly seen cause. Radiographs are not recommended in the routine management of bronchiolitis. We present the case of a young wheezing infant with a mildly abnormal chest x-ray whose cystic hygroma caused life-threatening respiratory distress soon after he was admitted to the hospital. PMID:27489658

  9. Pneumothorax in cystic fibrosis

    PubMed Central

    Kioumis, Ioannis P.; Zarogoulidis, Konstantinos; Huang, Haidong; Li, Qiang; Dryllis, Georgios; Pitsiou, Georgia; Machairiotis, Nikolaos; Katsikogiannis, Nikolaos; Papaiwannou, Antonis; Lampaki, Sofia; Porpodis, Konstantinos; Zaric, Bojan; Branislav, Perin; Mpoukovinas, Ioannis; Lazaridis, George

    2014-01-01

    Pneumothorax is recognized as a common and life-threatening complication in cystic fibrosis (CF) patients, especially in those who are infected with P. aeruginosa, B. cepacia or Aspergillus, need enteral feeding, are diagnosed as suffering from allergic bronchopulmonary aspergillosis (ABPA), developed massive hemoptysis, and their respiratory function is seriously compromised. Structural impairment and altered airflow dynamics in the lungs of CF patients are considered as the main predisposing factors, but also inhaled medications and non-invasive positive pressure ventilation (NIPPV) could increase the risk of pneumothorax. Clinical presentation could range from dramatic to very mild. Management of spontaneous pneumothorax occurring to patients with CF is essentially similar to that for non-CF patients. Therapeutic options include intercostal tube drainage, video-assisted thoracoscopic surgery (VATS), and medical or surgical pleurodesis. Pneumothorax increases both short- and long-term morbidity and mortality in CF patients and causes significant deterioration of their quality of life. PMID:25337406

  10. 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

  11. Molecular Diagnosis of Cystic Fibrosis.

    PubMed

    Deignan, Joshua L; Grody, Wayne W

    2016-01-01

    This unit describes a recommended approach to identifying causal genetic variants in an individual suspected of having cystic fibrosis. An introduction to the genetics and clinical presentation of cystic fibrosis is initially presented, followed by a description of the two main strategies used in the molecular diagnosis of cystic fibrosis: (1) an initial targeted variant panel used to detect only the most common cystic fibrosis-causing variants in the CFTR gene, and (2) sequencing of the entire coding region of the CFTR gene to detect additional rare causal CFTR variants. Finally, the unit concludes with a discussion regarding the analytic and clinical validity of these approaches. Copyright © 2016 John Wiley & Sons, Inc.

  12. Haemophilus infection in cystic fibrosis.

    PubMed Central

    Rayner, R J; Hiller, E J; Ispahani, P; Baker, M

    1990-01-01

    Twenty seven patients with cystic fibrosis under the age of 12 years and 27 matched patients with asthma were followed up in a prospective study for one year. The isolation rate of non-capsulated strains of Haemophilus influenzae from cough swabs and sputum specimens taken at routine clinic visits every two months was significantly greater in cystic fibrosis than in asthma. Haemophilus para-influenzae was equally common in both groups. During exacerbations the isolation rate of H influenzae in cystic fibrosis was significantly greater than at other times, whereas in asthma there was no significant difference. The distribution of biotypes of H influenzae and H parainfluenzae was similar in the two groups. In cystic fibrosis, biotype I was associated with exacerbations. Biotype V was more common than in previous studies, but was not associated with exacerbations. PMID:2185699

  13. [Lung physiotherapy in cystic fibrosis].

    PubMed

    Gursli, S; Haanaes, O C

    1991-02-28

    This article is intended as a brief practical guide for physicians and physiotherapists concerned with the treatment of cystic fibrosis. Physiotherapeutic techniques for the treatment of chest diseases have been developed and modified as advances have taken place in the medical management of cystic fibrosis. The article describes forced expiratory technique, positive expiratory pressure, postural drainage, autogenic drainage and other techniques. Patients with cystic fibrosis live longer and have a better quality of life than ever before, but progressive deterioration of lung function will always be their most serious problem. Physical activity and chest physiotherapy are essential parts of all treatment regimens for cystic fibrosis. It is important to realize that the physiotherapist is a very important member of the team which includes nurses, physicians-and the patient.

  14. Newborn screening for cystic fibrosis.

    PubMed

    Castellani, Carlo; Massie, John; Sontag, Marci; Southern, Kevin W

    2016-08-01

    Since the late 1970s when the potential of the immunoreactive trypsinogen assay for early identification of infants with cystic fibrosis was first recognised, the performance of newborn blood spot screening (NBS) has been continually assessed and its use has gradually expanded. NBS for cystic fibrosis is a cost-effective strategy and, if standards of care are fully implemented and robust management pathways are in place, has a positive effect on clinical outcomes. In the past decade, NBS has undergone rapid expansion and an unprecedented number of infants with cystic fibrosis have access to early diagnosis and care. Cystic fibrosis NBS has now moved on from the development phase and is entering an era of consolidation. In the future, research should focus on the rationalisation and optimisation of existing programmes, with particular attention to bioethical implications such as unwanted detection of carriers and inconclusive diagnoses. Copyright © 2016 Elsevier Ltd. All rights reserved.

  15. Physical training for cystic fibrosis.

    PubMed

    Bradley, J; Moran, F

    2008-01-23

    Physical training may form an important part of the care package for people with cystic fibrosis. To determine whether a prescribed regimen of physical training produces improvement or prevents deterioration in physiological and clinical outcomes in cystic fibrosis compared to no training. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Date of the most recent search: September 2007. All randomised and quasi-randomised controlled clinical trials in which a prescribed regimen of physical training is compared to no physical training in people with cystic fibrosis. Two authors independently selected studies for inclusion, assessed methodological quality and extracted data. Of the 26 studies identified, seven studies which included 231 participants, met the inclusion criteria. This review does provide some limited evidence from both short- and long-term studies that aerobic or anaerobic physical training has a positive effect on primary outcomes (exercise capacity, strength and lung function) but improvements are not consistent between studies. Conclusions about the efficacy of physical training in cystic fibrosis are limited by the small size, short duration and incomplete reporting of most of the studies included in this review. Physical training is already part of the care package offered to most people with cystic fibrosis and there is a lack of evidence to actively discourage this. The benefits obtained from including physical training in a package of care may be influenced by the type of training programme. Further research is needed to assess comprehensively the benefits of exercise programmes in people with cystic fibrosis and the relative benefits of the addition of aerobic versus anaerobic versus a combination of both types of physical training to the care

  16. Pneumococcal vaccines for cystic fibrosis.

    PubMed

    Burgess, Laura; Southern, Kevin W

    2014-08-05

    Invasive pneumococcal disease is associated with significant mortality and many countries have introduced routine pneumococcal vaccination into their childhood immunisation programmes. Whilst pneumococcal disease in cystic fibrosis is uncommon, pneumococcal immunisation may offer some protection against pulmonary exacerbations caused by this pathogen. In the USA and UK pneumococcal vaccination is currently recommended for all children and adults with cystic fibrosis. To assess the efficacy of pneumococcal vaccines in reducing morbidity in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Cystic Fibrosis Trials Register, which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. In addition, the pharmaceutical manufacturers of the polysaccharide and conjugate pneumococcal vaccines were approached.Date of the most recent search: 15 May 2014. Randomised and quasi-randomised controlled trials comparing pneumococcal vaccination (with either a polysaccharide or conjugate pneumococcal vaccine) with non-vaccination or placebo in children or adults with cystic fibrosis were eligible for inclusion. No relevant trials were identified. There are no trials included in this review. As no trials were identified we cannot draw conclusions on the efficacy of routine pneumococcal immunisation in people with cystic fibrosis in reducing their morbidity or mortality. As many countries now include pneumococcal immunisation in their routine childhood vaccination schedule it is unlikely that future randomised controlled trials will be initiated. Rigorously conducted epidemiological studies may offer the opportunity to evaluate the efficacy of pneumococcal vaccination in reducing morbidity and mortality in people with cystic fibrosis.

  17. Pneumococcal vaccines for cystic fibrosis.

    PubMed

    Burgess, Laura; Southern, Kevin W

    2012-09-12

    Invasive pneumococcal disease is associated with significant mortality and many countries have introduced routine pneumococcal vaccination into their childhood immunisation programmes. Whilst pneumococcal disease in cystic fibrosis is uncommon, pneumococcal immunisation may offer some protection against pulmonary exacerbations caused by this pathogen. In the USA and UK pneumococcal vaccination is currently recommended for all children and adults with cystic fibrosis. To assess the efficacy of pneumococcal vaccines in reducing morbidity in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Cystic Fibrosis Trials Register, which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. In addition, the pharmaceutical manufacturers of the polysaccharide and conjugate pneumococcal vaccines were approached.Date of the most recent search: 10 July 2012. Randomised and quasi-randomised controlled trials comparing pneumococcal vaccination (with either a polysaccharide or conjugate pneumococcal vaccine) with non-vaccination or placebo in children or adults with cystic fibrosis were eligible for inclusion. No relevant trials were identified. There are no trials included in this review. As no trials were identified we cannot draw conclusions on the efficacy of routine pneumococcal immunisation in people with cystic fibrosis in reducing their morbidity or mortality. As many countries now include pneumococcal immunisation in their routine childhood vaccination schedule it is unlikely that future randomised controlled trials will be initiated. Rigorously conducted epidemiological studies may offer the opportunity to evaluate the efficacy of pneumococcal vaccination in reducing morbidity and mortality in people with cystic fibrosis.

  18. What Are the Signs and Symptoms of Cystic Fibrosis?

    MedlinePlus

    ... Twitter. What Are the Signs and Symptoms of Cystic Fibrosis? The signs and symptoms of cystic fibrosis (CF) ... respiratory, digestive, or reproductive systems of the body. Cystic Fibrosis Figure A shows the organs that cystic fibrosis ...

  19. Endobronchial Ultrasound-directed Transbronchial Needle Aspiration in Diagnosis of Mediastinal Lesions: Initial Egyptian Experience.

    PubMed

    Safwat, Tarek; Khattab, Adel; Haddad, Salwa El; Mostafa, Yasser; Korraa, Emad; Madkour, Ashraf; Fattah, Wael Abd El

    2009-01-01

    Mediastinal lesions represent a diagnostic challenge and often require invasive approaches. We evaluated the role of radial probe endobronchial ultrasound-directed transbronchial needle aspiration (EBUS-TBNA) in the evaluation of mediastinal lesions. Between March 2005 to February 2006, 30 consecutive patients with enlarged mediastinal lymph nodes from unknown etiologies or suspicious for metastatic bronchogenic carcinoma and mediastinal masses underwent EBUS-TBNA and were clinically followed up. EBUS-TBNA was applied under topical anesthesia, midazolam sedation with a mean dose of 4.6+1.7 mg and prolonged the examination by 14.7 minutes on average. EBUS-directed TBNA was performed in 17 lymph nodes and 13 mediastinal masses, achieving specific diagnosis in 82.3% (14/17) and 84.6% (11/13) of examined lesions, respectively, with an overall yield of 83%. The sensitivity, specificity, and accuracy of EBUS-TBNA in distinguishing benign from malignant mediastinal lesions were 89.4%, 100%, and 93.3%, respectively. EBUS was well tolerated by most of the patients with no TBNA-related complications. In conclusion, EBUS-TBNA of mediastinal lesions is a minimally invasive safe diagnostic technique with high yield, even in the hands of those with initial experience. This initial study is convincing and stimulating for widespread application of EBUS-TBNA in Egyptian bronchoscopy practice.

  20. 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

  1. Primary Ectopic Mediastinal Goiter in a Patient With Crohn's Disease Presenting as Myasthenia Gravis.

    PubMed

    Kumar, Sunil; Sultania, Mahesh; Vatsal, Shivam; Sharma, M C

    2015-12-01

    Mediastinum is an uncommon location for ectopic goiter. Primary ectopic mediastinal goiter has been reported to present mostly with compressive symptoms. We report a case of a 62-year-old man with history of Crohn's disease, who presented with symptoms of myasthenia gravis and was found to have an anterior mediastinal mass. The mass was resected completely with successful outcome. On histopathologic examination this mass turned out to be colloid goiter. This is an extremely rare presentation of a primary ectopic mediastinal goiter.

  2. Acute mediastinitis due to extravasation of parenteral nutritional formula via a central venous catheter.

    PubMed

    Marín, Miguel Ruiz; Rodríguez, Maria Encarnación Tamayo; Buleje, Jorge Alejandro Benavides; Valverde, Francisco Miguel González; Martínez, Marcelino Méndez; Pérez, Patricia Pastor; Ruiz, María Vicente; Rodríguez, Ana Ruiz; Sales, Alejandro Puerta; Rodríguez, Pedro Marín; Blázquez, Antonio Albarracín Marín

    2012-07-01

    Mediastinitis is a complication generally associated with thoracic surgery. Its occurrence after placement of a central venous catheter is uncommon, and only a few cases have been reported. An 83-year-old man who had mediastinitis due to extravasation of parenteral nutritional formula via a central venous catheter is presented. The signs and symptoms, diagnosis, and treatment of this unusual complication are described. This complication should be included in the differential diagnosis of mediastinitis in patients with a central venous catheter in place who have not had thoracic surgery.

  3. Thoracoscopic Surgery for Glomus Tumor: An Uncommon Mediastinal Neoplasm and Iatrogenic Tracheal Rupture

    PubMed Central

    Fang, Zhongjie; Ma, Dehua; Luo, Huarong

    2017-01-01

    Mediastinal glomus tumors are rarely recognized, and only seven cases have been reported in the literature. Here, we describe a rare mediastinal glomus tumor and review the characteristics of this rare clinical case. The patient was a 50-year-old female who presented with coughing for 3 months. Her chest computed tomography scan demonstrated a localized tumor in the posterior superior mediastinum. Intraoperatively, we found a longitudinal rupture of the membranous trachea above the carina. We completely resected the tumor and repaired the tracheal rupture under a thoracoscopy using a pedicled muscle flap. The tissue was diagnosed as a mediastinal glomus tumor according to its histological and immunophenotypic characteristics. PMID:28133561

  4. [Methicillin-cephem-resistant Staphylococcus aureus (MRSA) mediastinitis following open heart surgery].

    PubMed

    Yanagisawa, H; Anzai, T; Iijima, T; Sakata, Y; Ishikawa, S; Obayashi, T; Otaki, A; Saito, A; Suzuki, M; Kamoshita, Y

    1991-11-01

    Mediastinitis following cardiac surgery occurs frequently in association with high mortality and morbidity. Patient was a 6-year-old boy suffering from Sotos syndrome with secundum type of ASD. He was operated upon to repair ASD. Following the surgery, he suffered from MRSA mediastinitis and osteomyelitis of the sternum. Routine method of closed irrigation after reopening of the sternal wound was ineffective. Patient recovered following aggressive debridement repeatedly, open drainage and topical irrigation with vancomycin. This appears to be a useful method to treat MRSA mediastinitis of the sternum.

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

    PubMed

    Tariq, Syed Mohammad

    2016-04-01

    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.

  6. Mediastinal seminoma presenting with superior vena cava syndrome.

    PubMed

    Wanous, Amanda; McPhail, Ian R; Quevedo, J Fernando; Sandhu, Nicole P

    2017-06-08

    We present a rare cause of superior vena cava syndrome (SVC) in a previously healthy male aged 31 years. Malignancy was suspected due to unintentional weight loss and childhood exposure to radioactive fallout from a nuclear facility accident. A very large anterior mediastinal mass was identified and demonstrated to be an extragonadal seminoma. Extragonadal germ cell tumours are rare tumours with a high potential for cardiovascular, pulmonary and vascular sequelae. Studies have documented an increased risk of developing seminoma in patients with radioactive exposure. Chemotherapy was initiated, during which the patient experienced progressive and new symptoms, found to be due to extensive thromboembolic disease, which responded well to anticoagulation. Seventy-two months after completing chemotherapy, without need for surgical management, he remains free of the disease. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  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.

  8. Primary Mediastinal Large B-cell Lymphoma Exhibiting Endobronchial Involvement

    PubMed Central

    Shimada, Midori; Fukuda, Minoru; Horio, Kensuke; Suyama, Takayuki; Kitazaki, Takeshi; Hashiguchi, Kohji; Fukuda, Masaaki; Shigematsu, Kazuto; Nakamura, Yoichi; Honda, Takuya; Ashizawa, Kazuto; Mukae, Hiroshi

    2016-01-01

    Primary mediastinal large B-cell lymphoma (PMLBCL) is one of the subtypes of diffuse large B-cell lymphoma. We experienced a rare case of PMLBCL that exhibited endobronchial involvement. A 33-year-old Japanese female with the chief complaints of epigastralgia, back pain, and nausea visited a primary care hospital. Computed tomography of the chest and abdomen demonstrated a bulky mass in the left anterior mediastinum, multiple pulmonary nodules, axillary lymph node swelling, and a pancreatic tumor. Fiberoptic bronchoscopy showed a white-tinged irregularly shaped endobronchial tumor accompanied by capillary vessel dilation in the left upper lobar bronchus. Taken together, these findings resulted in a diagnosis of PMLBCL. PMID:27803409

  9. 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

  10. [Mediastinal adenopathy disclosing Castleman's disease. Apropos of a case].

    PubMed

    Dansin, E; Wallaert, B; Tonnel, A B; Gosselin, B; Sault, M C

    1991-01-01

    Castleman's disease or pseudo-tumoral lymphoid hyperplasia is a rare entity. We report a case of Castleman's tumour in the antero-superior mediastinum which was revealed by a radiological examination in a young woman of 26. We also report immunohistochemical studies. There are two clinical presentations of Castleman's disease; a localised form (mediastinal or extrathoracic) and the multicentric variety. The localised form may be asymptomatic or can be accompanied by general features of inflammatory disease. Surgical excision of these localised forms both confirms the diagnosis and assures a cure. The multicentric variety has various clinical peculiarities (the patients are older and/or immunosuppressed and suffering from multiple enlarged glands also in the viscera) and the disease in above all progressive. There are associations with Kaposi sarcoma and lymphoproliferative complications are described in this multicentric form and their prognosis remains poor.

  11. 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.

  12. Video-assisted thoracic surgery compared with posterolateral thoracotomy for mediastinal bronchogenic cysts in adult patients

    PubMed Central

    Guo, Chenglin; Mei, Jiandong; Liu, Chengwu; Deng, Senyi; Pu, Qiang; Lin, Feng

    2016-01-01

    Background Mediastinal bronchogenic cyst (MBC) is the most common primary cystic lesion of the mediastinum. This study aimed to investigate the efficacy and safety of video-assisted thoracic surgery (VATS) compared with posterolateral thoracotomy (PLT) for the treatment of MBCs in a large series. Methods Patients with MBCs who underwent surgical resection between August 2005 and December 2015 were identified from the electronic database of the Department of Thoracic Surgery, West China Hospital. The patient demographic characteristics, intraoperative findings, postoperative outcomes and follow-up information were reviewed and analyzed. Results A total of 99 patients underwent cystectomy were enrolled for the present study. Of those patients, 65 underwent VATS cystectomy (VATS group) and 34 underwent PLT cystectomy (PLT group) during the same period. The VATS group had shorter operative time than the PLT group (108.77±47.81 vs. 144.62±55.16, P=0.001), less intraoperative blood loss (median 20 vs. 100 mL, P<0.001), and less pleural drainage of the first three days after surgery (median 240 vs. 400 mL, P=0.002). In addition, the length of postoperative hospital stay and duration of chest drainage for the VATS group was also shorter than those of the PLT group (4.94±2.01 vs. 8.64±5.52 days, P=0.001; 2.52±1.29 vs. 3.71±1.55 days, P<0.001, respectively). No statistical significance was revealed among the two groups with regard to the maximum diameter of the cysts, pleural atresia, incomplete resection, surgery-related complications, duration of intensive care unit stay, and postoperative complications. Conclusions Both VATS and PLT are reliable approaches for the surgical resection of MBCs. The VATS approach is superior to PLT with shorter operative time, shorter duration of chest drainage, shorter postoperative hospital stay, less intraoperative blood loss, and less pleural drainage of the first three days after surgery. We conclude that VATS should be the

  13. 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

  14. 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.

  15. Cystic Neoplasms of the Pancreas

    PubMed Central

    Tran Cao, Hop S.; Kellogg, Benjamin; Lowy, Andrew M.; Bouvet, Michael

    2015-01-01

    Whereas pancreatic duct adenocarcinoma (PDA) is a well-studied (but still poorly understood) disease with a dismal prognosis, cystic neoplasms of the pancreas form a more recently recognized group of pancreatic tumors. They are diverse and variable in their pathologic characteristics, clinical course, and outcomes,1–3 although all portend a better overall prognosis than PDA. In recent years, with the improved sensitivity and increasing use of cross-sectional imaging in clinical practice, these lesions are more commonly identified,4 with many being discovered incidentally. Indeed, large radiological series using computed tomography (CT) or magnetic resonance imaging (MRI) have reported detection rates of pancreatic cystic lesions between 1.2% and almost 20%,5,6 approaching the 24.3% prevalence rate in an autopsy series by Kimura and colleagues.7 Although most of these lesions are pseudocysts, a significant portion consist of cystic neoplasms, which are estimated to represent 10% to 15% of all primary pancreatic cystic lesions.8 Given the growing clinical relevance of these tumors, a keen understanding of their natural history and pathophysiology is needed. This article reviews pancreatic cystic neoplasms, with a focus on the challenges encountered in their diagnosis and treatment. PMID:20159515

  16. The history of the management of sternal osteomyelitis and mediastinitis - from Hippocrates until today.

    PubMed

    Ennker, Ina Carolin; Ennker, Jürgen C

    2014-01-01

    Even during the time of Hippocrates, Galen and their colleagues recognized mediastinal affections. However, they were not considered with the surgical treatment. First progress in the treatment options of this severe disease, still denoted as 'terra incognita', over to today's gold standard are pictured. The mediastinitis-registry which was founded by the German Society for Thoracic and Cardiovascular Surgery (DGTHG) in 2011 and the recent establishment of the study group to adopt a guideline 'diagnosis and therapy of postoperative mediastinitis/sternal osteomyelitis following cardiac surgery' are attempts to a standardization of the treatment. Substantial advancement in the treatment of postoperative mediastinitis could be achieved in the past. The mortality dropped as low as less than 10%. With these implementations more benefit for the patients' outcome can be expected.

  17. Tracheal varices caused by mediastinal compression of a large intrathoracic goiter: report of a case

    PubMed Central

    LUCCHINI, R.; SANTOPRETE, S.; TRIOLA, R.; POLISTENA, A.; MONACELLI, M.; AVENIA, S.; SANGUINETTI, A.; PUMA, F.; AVENIA, N.

    2015-01-01

    Introduction Tracheal varices are a rare condition but they can be an important source of massive or recurrent haemoptysis. Usually they are related to increased pressure in the pulmonary veins. Mediastinal goiter is often associated to compressive effects on the surrounding structures, including mediastinal vessels with potential superior vena cava syndrome. Case report We describe a case, not previously reported in literature, of mediastinal goiter with hemoptysis as first clinical manifestation. Bleeding was attributed to a superior vena cava syndrome associated to a tracheal fragile mucosa with an easily bleeding intramural nodule which was diagnosed as tracheal varices after total thyroidectomy. The nodule in fact disappeared together with the venous hypertensive signs after venous decompression of the mediastinum. Conclusions Compressive symptoms including tracheal varices, related to mediastinal goiter, can be treated surgically by total thyroidectomy via cervicotomy and when required with associated median sternotomy. PMID:25827666

  18. The history of the management of sternal osteomyelitis and mediastinitis – from Hippocrates until today

    PubMed Central

    Ennker, Ina Carolin; Ennker, Jürgen C.

    2014-01-01

    Even during the time of Hippocrates, Galen and their colleagues recognized mediastinal affections. However, they were not considered with the surgical treatment. First progress in the treatment options of this severe disease, still denoted as ‘terra incognita’, over to today’s gold standard are pictured. The mediastinitis-registry which was founded by the German Society for Thoracic and Cardiovascular Surgery (DGTHG) in 2011 and the recent establishment of the study group to adopt a guideline ‘diagnosis and therapy of postoperative mediastinitis/sternal osteomyelitis following cardiac surgery’ are attempts to a standardization of the treatment. Substantial advancement in the treatment of postoperative mediastinitis could be achieved in the past. The mortality dropped as low as less than 10%. With these implementations more benefit for the patients’ outcome can be expected. PMID:26504718

  19. Predictors of Mediastinitis Risk after Coronary Artery Bypass Surgery: Applicability of Score in 1.322 Cases

    PubMed Central

    Oliveira, Fabiana dos Santos; de Freitas, Letícia Delfino Oliveira; Rabelo-Silva, Eneida Rejane; da Costa, Laura Maggi; Kalil, Renato Abdala Karam; de Moraes, Maria Antonieta Pereira

    2017-01-01

    Background Mediastinitis is a severe surgical complication of low incidence, but high lethality. Scores used in the preoperative period to stratify the risk of postoperative mediastinitis may contribute to improve the results. Objective To test the applicability of the MagedanzSCORE in predicting the risk factors for mediastinitis in patients undergoing coronary artery bypass grafting at a cardiology reference hospital. Methods Historical cohort study with adult patients who underwent coronary artery bypass grafting. The analyzed variables were contemplated in the MagedanzSCORE: reoperation, chronic obstructive pulmonary disease (COPD), obesity, class IV unstable angina, polytransfusion therapy, mediastinitis and death as outcome variables. Results Of the 1.322 patients examined, 56 (4.2%) developed mediastinitis. Of these, 26 (46.4%) were classified as high risk for mediastinitis and 15 (26.8%) at very high risk for mediastinitis. Three of the five variables of the Magendanz Score showed statistically significant differences: reoperation, COPD and obesity. Class IV unstable angina and postoperative polytransfusion were not associated with mediastinitis after coronary artery by-pass grafting. The area under the ROC curve was 0.80 (CI 95% 0.73 - 0.86), indicating the model's satisfactory ability to predict the occurrence of mediastinitis. Conclusion The tool was useful in the preoperative assessment demonstrating the risk for mediastinitis in this population of intensive care patients. PMID:28832745

  20. Cystic Fibrosis (CF): Chloride Sweat Test

    MedlinePlus

    ... to 2-Year-Old Cystic Fibrosis (CF) Chloride Sweat Test KidsHealth > For Parents > Cystic Fibrosis (CF) Chloride Sweat Test Print A A A What's in this ... en el sudor What It Is A chloride sweat test helps diagnose cystic fibrosis (CF) , an inherited ...

  1. Cystic Fibrosis (CF): Chloride Sweat Test

    MedlinePlus

    ... 1- to 2-Year-Old Cystic Fibrosis (CF) Chloride Sweat Test KidsHealth > For Parents > Cystic Fibrosis (CF) Chloride Sweat Test A A A What's in this ... cloruro en el sudor What It Is A chloride sweat test helps diagnose cystic fibrosis (CF) , an ...

  2. What's it Like to Have Cystic Fibrosis?

    MedlinePlus

    ... Room? What Happens in the Operating Room? Cystic Fibrosis KidsHealth > For Kids > Cystic Fibrosis A A A What's in this article? What ... a condition she's known all her life — cystic fibrosis (say: SIS-tik fi-BRO-sus). Her parents ...

  3. [Suppurative mediastinitis after open heart surgery: in comparison between infants-children and adults].

    PubMed

    Fukasawa, M; Orita, H; Inui, K; Hirooka, S; Iijima, Y; Washio, M

    1993-02-01

    Among 361 consecutive patients who underwent open surgery from Jan. 1987 to Sept. 1991, risk factors and clinical courses were analyzed retrospectively in comparison between infants-children and adults. Seven mediastinitis (4.0%) occurred in 173 adult patients (20 to 75 y/o, mean: 54.4 y/o) and were not associated with age, sex, type of disease, and duration of operation or cardiopulmonary bypass. Postoperative mediastinitis significantly increased in the patients with low output syndrome (LOS) determined as use of IABP and/or assistant circulations (p < 0.001) and reexploration for bleeding or tamponase was associated with an increased risk for mediastinitis (p < 0.01). Five mediastinitis (2.7%) occurred in 188 infants and children (0 to 17 y/o, mean: 4.2 y/o). All patients involved with mediastinitis were less than 12 month old (2.6 +/- 3.3 month). None of the other factors was associated with an increased risk for this complication. Bacterial cultures of exudate were positive in 11 of 12 patients, and identified as MRSA in 10 and Staphylococcus epidermidis in one. In the seven of adult patients, two developed sepsis and four died with other organic failures or mediastinal bleeding. All five of infants healed after postoperative 33 to 145 days. The immature state of immune response might associate with postoperative mediastinitis in infants, whether LOS may be important in the immune suppression by surgical stress in adults, and the prognosis of mediastinitis might be effected by prolonged depression of postoperative cardiac function in adult patients.

  4. [Successful treatment of fungal endocarditis and mediastinitis after fenestrated Fontan operation--a case report].

    PubMed

    Miyaji, K; Shimada, M; Sekiguchi, A; Nishimura, K; Ishizawa, A; Isoda, T

    1993-12-01

    Fenestrated Fontan operation was performed in a 19-year-old male with a diagnosis of right isomerism syndrome. Postoperatively, fungal endocarditis due to Candida Albicans and mediastinitis by Methicilin resistant Staphylococcus Aureus (MRSA) occurred. For Candida endocarditis, combined surgery and medical treatment with amphotericin B was effective. MRSA mediastinitis was successfully treated by continuous closed irrigation with 0.5% povidone-iodine solution. This is the 17th reported case of fungal endocarditis after open heart surgery in Japanese literature.

  5. 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

  6. Radioisotope therapy of cystic craniopharyngeomas

    SciTech Connect

    Strauss, L.; Sturm, V.; Georgi, P.; Schlegel, W.; Ostertag, H.; Clorius, J.H.; Van Kaick, G.

    1982-09-01

    Eighteen patients suffering from cystic craniopharyngeoma were treated with intracavitary irradiation. The beta-emitting radioisotope /sup 90/y (2.25 MeV) was instilled into the cyst following stereotactic puncture of the space-occupying lesion. The surgical approach was planned using angiograms and reconstructed transmission computer tomography (TCT) coronal and saggital sections. Therapy was devised to deliver 20,000 rad to the cyst's wall. Eleven patients received follow-up TCT examinations after four months. Eight of 11 patients had a significant volume decrease in the craniopharyngeoma cyst. In two patients, the cystic volume remained unchanged; one had progression of disease. It is concluded that the intracavitary treatment of cystic craniopharyngeoma will result in a reduction of the size of the space-occupying lesion.

  7. [Intestinal cystic duplication. Case report].

    PubMed

    Herranz Barbero, Ana; Prat Ortells, Jordi; Muñoz Fernández, M Elena; Castañón García-Alix, Montserrat; Figueras Aloy, Josep

    2017-08-01

    Intestinal cystic duplications are rare congenital anomalies, with an estimated incidence of approximately 1:4500 autopsies. The etiopathogenesis is uncertain. These duplications are cystic, tubular or diverticular structures lined with gastrointestinal mucosa. They share a common smooth muscle wall with the gastrointestinal tract but usually their lumens do not communicate with each other. Gastric duplication cysts represent 7-9% of the gastrointestinal tract duplication. They can be diagnosed prenatally by fetal ultrasound; magnetic resonance imaging characterizes the cyst and excludes other malformations. Postnatal ultrasound shows a characteristic double walled cyst. Newborns are usually asymptomatic, although nonspecific gastrointestinal symptoms, intestinal obstruction due to mass effect, volvulus or infection are described. In asymptomatic patients, clinical follow-up and periodic image controls are recommended. Elective surgical resection is the treatment of choice, using minimally invasive technique whenever possible. A case of prenatally suspected intestinal cystic duplication is presented. Sociedad Argentina de Pediatría.

  8. Poststernotomy mediastinitis: a review of conventional surgical treatments, vacuum-assisted closure therapy and presentation of the Lund University Hospital mediastinitis algorithm.

    PubMed

    Sjögren, Johan; Malmsjö, Malin; Gustafsson, Ronny; Ingemansson, Richard

    2006-12-01

    Poststernotomy mediastinitis, also commonly called deep sternal wound infection, is one of the most feared complications in patients undergoing cardiac surgery. The overall incidence of poststernotomy mediastinitis is relatively low, between 1% and 3%, however, this complication is associated with a significant mortality, usually reported to vary between 10% and 25%. At the present time, there is no general consensus regarding the appropriate surgical approach to mediastinitis following open-heart surgery and a wide range of wound-healing strategies have been established for the treatment of poststernotomy mediastinitis during the era of modern cardiac surgery. Conventional forms of treatment usually involve surgical revision with open dressings or closed irrigation, or reconstruction with vascularized soft tissue flaps such as omentum or pectoral muscle. Unfortunately, procedure-related morbidity is relatively frequent when using conventional treatments and the long-term clinical outcome has been unsatisfying. Vacuum-assisted closure is a novel treatment with an ingenious mechanism. This wound-healing technique is based on the application of local negative pressure to a wound. During the application of negative pressure to a sternal wound several advantageous features from conventional surgical treatment are combined. Recent publications have demonstrated encouraging clinical results, however, observations are still rather limited and the underlying mechanisms are largely unknown. This review provides an overview of the etiology and common risk factors for deep sternal wound infections and presents the historical development of conventional therapies. We also discuss the current experiences with VAC therapy in poststernotomy mediastinitis and summarize the current knowledge on the mechanisms by which VAC therapy promotes wound healing. Finally, we suggest a structured algorithm for using VAC therapy for treatment of poststernotomy mediastinitis in clinical

  9. Nutritional management of cystic fibrosis.

    PubMed Central

    MacDonald, A

    1996-01-01

    Nutritional support is an integral part of the management of cystic fibrosis patients. It is arguably best provided by a qualified dietitian and nutritional care sister working in conjunction with the rest of the cystic fibrosis team. The patient's nutritional needs should be assessed, regularly reviewed, and nutritional treatment tailored to meet the changing clinical and psychosocial needs of the patient. Nutritional intervention is not without complications, and in particular attention to normal feeding behaviour and vigilance when instituting supplementary nutrition may prevent many feeding difficulties. PMID:8660059

  10. Progression of mediastinal tuberculosis and superior vena caval obstruction demonstrated by gallium-67 citrate and radiocolloid liver scintigraphy

    SciTech Connect

    Shih, W.J.; DeLand, F.H.; Domstad, P.A.; Mac Vandiviere, H.; Dillon, M.L.

    1986-02-01

    Most causes of superior vena caval (SVC) obstruction are caused by malignant mediastinal neoplasms, especially bronchogenic carcinoma. Less frequently SVC obstruction results from a non-malignant lesion such as mediastinal goiter or tuberculosis. We present a case of mediastinal tuberculosis which progressively enlarged as shown in consecutive Ga-67 citrate studies. As a result, SVC obstruction developed, exhibited by radionuclide SV Cavagram, and demonstrated ''hot'' spots in the radiocolloid scans.

  11. Survival rates in cystic fibrosis.

    PubMed Central

    Wilmott, R W; Tyson, S L; Dinwiddie, R; Matthew, D J

    1983-01-01

    Life tables were calculated for 273 British children with cystic fibrosis for the period 1974-9. There was a marked improvement in survival rates in the meconium ileus group compared with the 1969-73 data, but there was little improvement in patients presenting later with other symptoms. PMID:6639137

  12. Nutritional management of cystic fibrosis.

    PubMed

    Goodchild, M C

    1987-01-01

    Cystic fibrosis patients have an increased requirement for calories and probably for all the major nutrients. The newer, enteric-coated granular preparations of pancreatic enzyme are more effective than preceding preparations and should permit a normal fat intake. Recent work has emphasized the interdependence of respiratory disease and nutrition.

  13. [Ectopic mediastinal parathyroid tumor resected by video-assisted thoracic surgery with intraoperative methylene blue infusion; report of a case].

    PubMed

    Okagawa, Takehiko; Hiramatsu, Yoshinori

    2014-03-01

    We report a surgical case of ectopic mediastinal parathyroid tumor resected by video-assisted thoracic surgery with intraoperative methylene blue infusion. It is often difficult to detect ectopic mediastinal parathyroid tumor during the operation because the tumor is soft, small and buried under mediastinal tissue. After methylene blue 4 mg/kg intravenously administration, the tumor was gradually dyed blue and easily detected and resected by video-assisted thoracic surgery. It is useful of methylene blue for detection of ectopic mediastinal parathyroid tumor.

  14. Transcarinal needle aspiration in the diagnosis of mediastinal adenitis in a patient infected with the human immunodeficiency virus

    PubMed Central

    Serdà, G Julià; de Castro, F Rodriguez; Sánchez-Alarcos, J M Fernandez; Luna, J Caminero; López, F Diaz; Navarro, P Cabrera

    1990-01-01

    Tuberculous mediastinal lymphadenopathy in a patient infected with human immunodeficiency virus (HIV) was diagnosed by means of transcarinal needle aspiration via a fibreoptic bronchoscope. Images PMID:2382248

  15. Severe descending necrotizing mediastinitis: vacuum-assisted dressing did wonder.

    PubMed

    Liew, Y T; Lim, Elizabeth Yenn Lynn; Zulkiflee, A B; Prepageran, N

    2017-04-01

    Descending necrotizing mediastinitis (DNM) is a rapidly progressive disease from the spread of cervical infection. Transcervical and transthoracic drainage was the recognized traditional surgical approach to achieve adequate clearance of infection. Non-invasive vacuum-assisted dressing is a new technique where applied negative pressure can help remove infective fluid and to promote wound healing. A 60-year-old man presented with odynophagia, fever and anterior neck swelling for 2 weeks. He was diagnosed to have anterior neck abscess and underwent surgical drainage. However, it did not respond well, but progressed to involve superior and inferior mediastinum. Vacuum-assisted dressing was applied for total of 2 weeks and the patient recovered without going through usual traditional transthoracic drainage. Vacuum drainage is a simple, safe and non-invasive method of managing DNM, in well selected group with small abscess cavity without airway obstruction and septicemia. This potential technique can lead to paradigm shift in treating life-threatening DNM.

  16. Mediastinal migration of distal occipito-thoracic instrumentation

    PubMed Central

    Joseph, Vivek; Al Jahwari, Ahmed S.

    2007-01-01

    We present the occurrence and management of mediastinal migration of the distal aspect of a posterior occipito-thoracic screw–rod construct. No similar occurrence was found in the literature. This event occurred following an emergency tracheotomy (requiring neck hyperextension) in a patient with severe rheumatoid arthritis, who had previously undergone decompression and an Occiput-T2 instrumented fusion for cranio-cervical and sub-axial cervical spine instability. Imaging showed fracture-subluxation of T1/2 and T2/3 with the bilateral C7, T1 and T2 screws in the mediastinum causing tracheal and esophageal compression. Removal of the instrumentation, decompression (T2 corpectomy) and construct revision down to T10 was safely performed from a posterior approach. Severe osteoporosis, some pre-existing screw loosening and hyperextension of the neck were the predisposing factors of this near catastrophic event. By staying directly posterior to the rod and following the fibrous tract already created, the instrumentation was safely removed from the mediastinum. PMID:18000689

  17. [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.

  18. Apparent Diffusion Coefficient Measurement in Mediastinal Lymphadenopathies: Differentiation between Benign and Malignant Lesions

    PubMed Central

    Ustabasioglu, Fethi Emre; Samanci, Cesur; Alis, Deniz; Samanci, Nilay Sengul; Kula, Osman; Olgun, Deniz Cebi

    2017-01-01

    Objectives: We aimed to prospectively assess the diagnostic value of apparent diffusion coefficient (ADC) measurement in the differentiation of benign and malignant mediastinal lymphadenopathies. Materials and Methods: The study included 63 consecutive patients (28 women, 35 men; mean age 59.3 years) with 125 mediastinal lymphadenopathies. Echoplanar diffusion-weighted magnetic resonance imaging of the mediastinum was performed with b-factors of 0 and 600 mm2/s before mediastinoscopy and mediastinotomy, and ADC values were measured. The ADC values were compared with the histological results, and statistical analysis was done. P < 0.05 was considered statistically significant. Results: The mean ADC value of malignant mediastinal lymphadenopathy (1.030 ± 0.245 × 10−3 mm2/s) was significantly lower (P < 0.05) when compared to benign lymphadenopathies (1.571 ± 0.559 × 10−3 mm2/s). For differentiating malignant from benign mediastinal lymphadenopathy, the best result was obtained when an ADC value of 1.334 × 10−3 mm2/s was used as a threshold value; area under the curve 0.848, accuracy 78.4%, sensitivity 66%, specificity of 86%, positive predictive value 76.7%, and negative predictive value of 79.2%. Interobserver agreement was excellent for ADC measurements. Conclusions: ADC measurements could be considered an important supportive method in differentiating benign from malignant mediastinal lymphadenopathies. PMID:28400998

  19. Mediastinal Lymphadenopathy in Patients Undergoing Cardiac Transplant Evaluation

    PubMed Central

    Van Bakel, Adrian B.; Brand, Timothy M.; Ravenel, James G.; Gilbert, Gregory E.; Silvestri, Gerard A.; Judson, Marc A.

    2011-01-01

    Background: We evaluated the association between hemodynamic parameters of chronic congestive heart failure (CHF) and mediastinal lymphadenopathy (MLA) in heart transplantation (HT) candidates and the effect of HT on MLA. We also described the results of lymph node (LN) biopsies of MLA in the patients. Methods: Patients who underwent HT evaluation over an 8-year period and had chest CT scans were evaluated retrospectively. Data collected included LN sizes pre-HT and post-HT, echocardiographic measurements, radionuclide-derived ejection fraction, and right-sided heart catheterization hemodynamics. MLA was defined as LNs > 1 cm in smallest dimension. Results: Of 118 patients, 53 patients had MLA. MLA had weak statistically significant correlations with elevated mean pulmonary artery pressure (MPAP), mitral regurgitation (MR), tricuspid regurgitation (TR), right atrial pressure (RAP), and pulmonary capillary wedge pressure (PCWP). Thirty-six patients with MLA underwent HT, and nine of the 36 had post-HT chest CT scans. All nine patients showed a decrease in LN size post-HT (mean LN diameter pre-HT = 1.16 ± 0.137 cm, post-HT = 0.75 ± 0.32 cm). Seven of 53 patients with MLA underwent biopsies. Four had benign LNs, one had sarcoidosis, and two had lung cancer. Conclusions: MPAP, MR, TR, RAP, and PCWP had weak statistically significant correlations with MLA. HT led to regression of MLA in patients who underwent CT scans post-HT, implying that MLA is related to CHF. However, we also identified clinically important causes of MLA; therefore, biopsy should be considered if enlarged LNs fail to regress after maximal medical management of CHF. PMID:20966040

  20. [Aeromonas hydrophila septicemia: 3 cases, 1 with mediastinitis].

    PubMed

    Outin, H D; Chatelin, A; Ronco, E; Nauciel, C; Gajdos, P; Barois, A; Goulon, M

    1984-01-01

    Three cases of Aeromonas hydrophila septicaemia are reported. The first case concerned a 51 years old patient suffering from drowning who had previously been in good health and who died after a few days of refractory hypoxaemia. The second case was an 8 years old child with trisomy 21 operated for a congenital cardiac defect. The portal of entry was a catheter and the septicaemia was complicated by A. hydrophila mediastinitis, a complication which has not been described previously. The third case was a 75 years old man with no previous medical history, whose hobby was gardening. He developed septicaemia after a flu-like illness. A. hydrophila is a mobile Gram negative bacillus whose natural habitat is water. It appears to be an exceptional and temporary contaminant of the human G I tract. Clinical infection is rare and usually focal: gastroenteritis, infection of soft tissues after trauma in aquatic surroundings. In 60 out of 82 cases there was a documented deficiency in the patient's immune defenses. A digestive portal of entry was suspected in 63 cases but only rarely proved. In one of our cases the portal of entry was septic thrombophlebitis induced by an indwelling catheter; this complication has not previously been reported. The clinical picture of A. hydrophila septicaemia is characterised by the relative high incidence of Echtyma gangrenosum. The lethal outcome in 50 p. 100 of cases is often related to the patient's general condition. The sensitivity of the three cultures isolated was tested against third generation cephalosporins: they were effective in each occasion.

  1. Laparoscopic management of enlarged cystic duct.

    PubMed

    Nowzaradan, Y; Meador, J; Westmoreland, J

    1992-12-01

    After laparoscopic exploration of the common bile duct, or when a patient has acute cholecystitis, the cystic duct is sometimes edematous and too large to be ligated safely with an Endoclip. In such cases, ligation of the cystic duct with an Endoloop offers a solution to the problem. The standard technique for application of an Endoloop consists of dividing the cystic duct and then applying the Endoloop. This becomes more difficult if, after the cystic duct is divided, loss of traction on the common bile duct results in retraction of the divided cystic stump outside of the laparoscopic field of view. To avoid this difficulty, the authors apply an Endoloop with the grasping forceps on the cystic duct before the duct is divided so that it cannot retract from operative view and for this task developed an instrument that allows simultaneous introduction of both grasping forceps and the Endoloop through a single port.

  2. Biomarkers in Paediatric Cystic Fibrosis Lung Disease.

    PubMed

    Ramsey, Kathryn A; Schultz, André; Stick, Stephen M

    2015-09-01

    Biomarkers in cystic fibrosis are used i. for the measurement of cystic fibrosis transmembrane regulator function in order to diagnose cystic fibrosis, and ii. to assess aspects of lung disease severity (e.g. inflammation, infection). Effective biomarkers can aid disease monitoring and contribute to the development of new therapies. The tests of cystic fibrosis transmembrane regulator function each have unique strengths and weaknesses, and biomarkers of inflammation, infection and tissue destruction have the potential to enhance the management of cystic fibrosis through the early detection of disease processes. The development of biomarkers of cystic fibrosis lung disease, in particular airway inflammation and infection, is influenced by the challenges of obtaining relevant samples from infants and children for whom early detection and treatment of disease might have the greatest long term benefits.

  3. Cystic Fibrosis Research | NIH MedlinePlus the Magazine

    MedlinePlus

    ... please 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 ...

  4. [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. Copyright © 2015 Elsevier España, S.L.U. and AEEH y AEG. All rights reserved.

  5. 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.

  6. Pseudomembranous colitis in cystic fibrosis.

    PubMed

    Nagakumar, Prasad

    2013-05-01

    Cystic fibrosis (CF) patients may require frequent courses of antibiotics and repeated hospital admissions. Although children with CF have high carriage rate for C.difficile, they rarely develop colitis. Pseudomembranous colitis is more common in adult post lung transplant CF patients. Although rare, paseudomembranous colitis should be considered in CF patients presenting with abdominal symptoms even in the absence of diarrhoea. Copyright © 2013 Elsevier Ltd. All rights reserved.

  7. 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.

  8. 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

  9. Hodgkin’s disease presenting as discharging neck sinuses and a mediastinal mass

    PubMed Central

    Zolotar, Meira; Olaleye, Oladejo; Sherif, Ali; Howe, Rachael; Mathews, John

    2011-01-01

    A 23-year-old Asian lady presented with a hard indurated midline neck swelling of 2 months duration without any upper aerodigestive tract or systemic symptoms of note. Her inflammatory markers were elevated and she was commenced on antibiotics. Ultrasound scan and fine needle aspiration cytology (FNAC) were inconclusive. A CT scan showed an ill-defined soft tissue mass anterior to and not well demarcated from the thyroid, and contiguous with a superior and anterior mediastinal mass. Incisional biopsy revealed necrosis and pockets of purulent material. Microbiology suggested a chronic pyogenic abscess negative for acid fast bacilli. She was treated with antituberculous therapy without resolution. She developed a discharging lateral neck mass with progressive increase of the mediastinal mass. She subsequently required a neck exploration and mediastinoscopy. Repeat mediastinal biopsies confirmed the diagnosis of Hodgkin’s disease and no organisms on culture. She was commenced on chemotherapeutic treatment with a satisfactory outcome. PMID:22689859

  10. Resection of giant mediastinal liposarcoma via ‘⊣ shape’ incision

    PubMed Central

    Huang, Wei; Jiang, Ge Ning

    2017-01-01

    Primary mediastinal liposarcomas are extremely rare conditions often resected through standard median sternotomy or lateral thoracotomy. However, the management of a very huge mediastinal tumor involving hemithorax through these two common surgical approaches is always challenging. Herein, we report a case of applying median sternotomy with a sternum transection plus a right fourth intercostal thoracotomy (‘⊣ shape’ incision) to resect a giant primary anterior mediastinal liposarcoma extending into the whole right thorax. The final pathological diagnosis was a well-differentiated liposarcoma. The patient experienced an uneventful recovery. The ‘⊣ shape’ incision is a good backup for the extension of standard median sternotomy and provides a better exposure for both mediastinum and hemithorax. PMID:28044001

  11. An Unusual Presentation of a Posterior Mediastinal Schwannoma Associated with Traumatic Hemothorax

    PubMed Central

    Amin, Ruchi; Waibel, Brett H.

    2015-01-01

    Schwannomas of the thoracic cavity are typically an asymptomatic, benign neurogenic neoplasm of the posterior mediastinum. In this case, we present a traumatic hemothorax as the initial presentation for a previously undiscovered mediastinal mass. The patient presented with shortness of breath and right-sided chest pain after being struck in the chest with a soccer ball. An operative exploration was pursued due to persistent hemothorax with hemodynamic instability despite resuscitation and adequate thoracostomy tube placement. The intraoperative etiology of bleeding was discovered to be traumatic fracture of a large hypervascular posterior mediastinal schwannoma. Surgical resection is the treatment of choice for these tumors. Specific serological markers do not exist for this tumor, and radiographic findings can be variable, so tissue diagnosis is of importance in differentiating benign from malignant schwannomas, as well as other posterior mediastinal tumors. However, most patients have excellent survival following complete resection. PMID:26064757

  12. 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

  13. 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.

  14. From cytology to histology: diagnosis of a relapsed mediastinal lymphoma by endobronchial ultrasound transbronchial histological needle

    PubMed Central

    Ariza-Prota, Miguel Angel; Bango Álvarez, Antonio; Pérez, Liliana; Pando-Sandoval, Ana; Fuentes, Nelson; Casan, Pere

    2015-01-01

    Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is highly accurate in diagnosing mediastinal lymphadenopathies of lung cancer and benign disorders, with the advantage that it is a minimally invasive technique, unlike open surgery and mediastinoscopy. However, the diagnostic accuracy of EBUS-TBNA for the diagnosis of lymphoma in patients with mediastinal lymphadenopathy is not well defined. The lack of tissue architecture obtained by cytological needles decreases the diagnostic accuracy for diagnosis and subtyping of de novo and relapsed mediastinal lymphomas. We present the first described case in the literature of an anaplastic large cell lymphoma relapsed, diagnosed on tissue fragments obtained by EBUS-TBNA with the particularity of using a histological needle. PMID:26090115

  15. Anesthetic management of a large mediastinal mass for tracheal stent placement.

    PubMed

    Rajagopalan, Suman; Harbott, Mark; Ortiz, Jaime; Bandi, Venkata

    2016-01-01

    The anesthetic management of patients with large mediastinal masses can be complicated due to the pressure effects of the mass on the airway or major vessels. We present the successful anesthetic management of a 64-year-old female with a large mediastinal mass that encroached on the great vessels and compressed the trachea. A tracheal stent was placed to relieve the tracheal compression under general anesthesia. Spontaneous ventilation was maintained during the perioperative period with the use of a classic laryngeal mask airway. We discuss the utility of laryngeal mask airway for anesthetic management of tracheal stenting in patients with mediastinal masses. Copyright © 2014 Sociedade Brasileira de Anestesiologia. Published by Elsevier Editora Ltda. All rights reserved.

  16. Cervicofacial subcutaneous and mediastinal emphysema caused by air cooling spray of dental laser.

    PubMed

    Mitsunaga, Sachiyo; Iwai, Toshinori; Aoki, Noriaki; Yamashita, Yosuke; Omura, Susumu; Matsui, Yoshiro; Maegawa, Jiro; Hirota, Makoto; Mitsudo, Kenji; Tohnai, Iwai

    2013-06-01

    Cervicofacial subcutaneous emphysema is a rare complication of dental procedures with an air turbine or syringe, and dentists and oral surgeons sometimes encounter mediastinal emphysema following the presentation of extensive subcutaneous emphysema. Most emphysema occurs incidentally during tooth extraction, restorative treatment, or endodontic treatment, with only a few cases reported of cervicofacial subcutaneous emphysema associated with dental laser treatment. We report a case of cervicofacial subcutaneous and mediastinal emphysema caused by the air cooling spray of dental laser during dental treatment in a 76-year-old woman. After she underwent dental laser treatment, cervicofacial swelling was noted and she was referred to our department. Computed tomography showed both cervicofacial subcutaneous emphysema and mediastinal emphysema. Antibiotics were administered prophylactically and the emphysema disappeared 5 days after the dental laser treatment, without any complications. Copyright © 2013 Elsevier Inc. All rights reserved.

  17. Vacuum-assisted closure (VAC)-Instill(®) with continuous irrigation for the treatment of Mycoplasma hominis mediastinitis.

    PubMed

    Karaca, Saziye; Kalangos, Afksendiyos

    2015-10-01

    A 56-year-old patient who underwent ascending aorta replacement postoperatively developed mediastinitis with atypical Mycoplasma hominis. We present the first successful treatment of M. hominis mediastinitis after cardiac surgery with vacuum-assisted closure (VAC)-Instill(®) therapy combined with dilute antiseptic irrigation for bacterial eradication.

  18. [Successful treatment of Candida albicans mediastinitis after open-heart surgery using vacuum-assisted closure device].

    PubMed

    Fukunaga, Naoto; Nishiya, Kenta; Sakon, Yoshito; Konishi, Yasunobu; Nakamura, Ken; Saji, Yoshiaki; Kanemitsu, Hideo; Koyama, Tadaaki

    2014-10-01

    An 82-year-old man suffered from Candida albicans mediastinitis following emergency aortic valve replacement. After repeated debridement of the anterior portion of the mediastinum, we applied a vacuum assisted closure device with UrgoTul Absorb placed on the right ventricle. Despite relatively short-term application of this device, mediastinitis was cured in combination with transposition of the great omentum.

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

    PubMed

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

    2013-05-01

    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.

  20. Identification of Primary Mediastinal Large B-cell Lymphoma at Nonmediastinal Sites by Gene Expression Profiling.

    PubMed

    Yuan, Ji; Wright, George; Rosenwald, Andreas; Steidl, Christian; Gascoyne, Randy D; Connors, Joseph M; Mottok, Anja; Weisenburger, Dennis D; Greiner, Timothy C; Fu, Kai; Smith, Lynette; Rimsza, Lisa M; Jaffe, Elaine S; Campo, Elias; Martinez, Antonio; Delabie, Jan; Braziel, Rita M; Cook, James R; Ott, German; Vose, Julie M; Staudt, Louis M; Chan, Wing C

    2015-10-01

    Mediastinal involvement is considered essential for the diagnosis of primary mediastinal large B-cell lymphoma (PMBL). However, we have observed cases of diffuse large B-cell lymphoma (DLBCL) with features of PMBL but without detectable mediastinal involvement. The goal was to assess our previously established gene expression profiling (GEP) signature for PMBL in classifying these cases. In a large series of DLBCL cases, we identified 24 cases with a GEP signature of PMBL, including 9 cases with a submission diagnosis of DLBCL consistent with PMBL (G-PMBL-P) and 15 cases with a submission diagnosis of DLBCL. The pathology reviewers agreed with the diagnosis in the 9 G-PMBL-P cases. Among the other 15 DLBCL cases, 11 were considered to be PMBL or DLBCL consistent with PMBL, 3 were considered to be DLBCL, and 1 case was a gray-zone lymphoma with features intermediate between DLBCL and classical Hodgkin lymphoma. All 9 G-PMBL-P and 9 of the 15 DLBCL cases (G-PMBL-M) had demonstrated mediastinal involvement at presentation. Interestingly, 6 of the 15 DLBCL cases (G-PMBL-NM) had no clinical or radiologic evidence of mediastinal involvement. The 3 subgroups of PMBL had otherwise similar clinical characteristics, and there were no significant differences in overall survival. Genetic alterations of CIITA and PDL1/2 were detected in 26% and 40% of cases, respectively, including 1 G-PMBL-NM case with gain of PDL1/2. In conclusion, PMBL can present as a nonmediastinal tumor without evidence of mediastinal involvement, and GEP offers a more precise diagnosis of PMBL.

  1. 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. Copyright © 2016. Published by Elsevier B.V.

  2. 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.

  3. Analysis of treatment results in primary germ cell tumours with mediastinal location: own experience.

    PubMed

    Kowalski, Dariusz M; Knetki-Wróblewska, Magdalena; Winiarczyk, Kinga; Jaśkiewicz, Piotr; Orłowski, Tadeusz; Langfort, Renata; Krzakowski, Maciej; Olszewski, Michał

    2014-01-01

    Primary germ cell tumours with mediastinal location comprise 1-6% of mediastinal tumours and 2-5% of all germ cell tumours occurring in adults. They are identified mostly in the 3rd decade of life, in 90% of cases in men. The most common symptoms are dyspnea, chest pain, cough, fever and weight loss. The aim of the present study was the analysis of our own results of treatment of primary germ cell tumours with mediastinal location, and a review of the literature concerning this subject. Five patients (4 males, 1 female) median age 27.8 years (range 23-30 years) treated in the period from 1999 to 2009 in Maria Sklodowska-Curie Memorial Cancer Centre and Institute of Oncology, Department of Lung Cancer and Chest Tumours in Warsaw, due to germinal tumours with primary mediastinal location, entered the study. All patients received chemotherapy according to the BEP regimen. All patients achieved an objective response to treatment. Two patients died due to disease progression in spite of II- and III-line treatment. Three patients are still in follow-up. The median survival time was 55.8 months (range 8.0-120.0 months). Primary mediastinal germ cell tumours have worse prognosis than do those with gonadal location. Based on our observations and review of the literature, it can be concluded that the results of treatment of non-seminoma type germ cell tumours with primary mediastinal location remain poor. Patients who develop early recurrence or progression during first-line chemotherapy are particularly at risk of unfavourable outcome. Identification of new standards of treatment in tumours resistant to cisplatin require further studies evaluating the effectiveness of new generation cytostatic drugs.

  4. Late Chronic Tamponade after Intraoperative Right Ventricular Rupture Repair with Mediastinal Fat.

    PubMed

    Gualis, Javier; Castaño, Mario; Rodríguez, Miguel Angel; García, Cristina

    2015-12-01

    Advanced age and female sex are known risk factors for ventricular wall rupture during open-heart procedures. We present the case of an 83-year-old female patient with an intraoperative traumatic right ventricular free wall rupture during an aortic valve replacement procedure. Pledgetted interrupted sutures reinforced with large pieces of mediastinal fat were used for rupture repair. After 6 months, the patient was readmitted with the diagnosis of a retrosternal mediastinal mass and clinical signs of cardiac tamponade that required reoperation.

  5. Postoperative mediastinitis due to Candida tropicalis: first reported case in Morocco.

    PubMed

    Maleb, A; Nya, F; Amahzoune, B; Lemnouer, A; Elouennass, M

    2014-09-01

    Mediastinitis is a rare and serious nosocomial complication of cardiac surgery. It is estimated at less than 2% of cases of median sternotomy. We report the case of a postoperative mediastinitis due to Candida tropicalis. A 9-month-old baby was operated for complete repair of tetralogy of Fallot in which we isolated C. tropicalis from sternal purulent fluid and blood culture. The child did not survive, despite the initiation of antifungal therapy. The management of this type of infection requires an early diagnosis and an appropriate prolonged treatment, associated with effective preventive measures. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  6. A retropharyngeal–mediastinal hematoma with supraglottic and tracheal obstruction: The role of multidisciplinary airway management

    PubMed Central

    Birkholz, Torsten; Kröber, Stefanie; Knorr, Christian; Schiele, Albert; Bumm, Klaus; Schmidt, Joachim

    2010-01-01

    A 77-year-old man suffered hypoxemic cardiac arrest by supraglottic and tracheal airway obstruction in the emergency department. A previously unknown cervical fracture had caused a traumatic retropharyngeal–mediastinal hematoma. A lifesaving surgical emergency tracheostomy succeeded. Supraglottic and tracheal obstruction by a retropharyngeal–mediastinal hematoma with successful resuscitation via emergency tracheostomy after hypoxemic cardiac arrest has never been reported in a context of trauma. This clinically demanding case outlines the need for multidisciplinary airway management systems with continuous training and well-implemented guidelines. Only multidisciplinary staff preparedness and readily available equipments for the unanticipated difficult airway solved the catastrophic clinical situation. PMID:21063569

  7. Mediastinal Pseudocyst: Varied Presentations and Management—Experience from a Tertiary Referral Care Centre in India

    PubMed Central

    Abdul Jameel, Abdul Rehman; Kannan, Naveen; Anbalagan, Amudhan; Duraisamy, Benet; Raju, Prabhakaran

    2017-01-01

    Pseudocysts are a recognised complication following acute or chronic pancreatitis. Usually located in peripancreatic areas, they have also been reported to occur in atypical regions like liver, pelvis, spleen, and mediastinum. Mediastinal pseudocysts are a rare entity and present with myriad of symptoms due to their unique location. They are a clinical challenge to diagnose and manage. In this paper, we describe the clinical and radiological characteristics of mediastinal pseudocysts in 7 of our patients, as well as our experience in managing these patients along with their clinical outcome. PMID:28392624

  8. Percutaneous coil embolisation of a false aortic aneurysm following coronary surgery and mediastinitis.

    PubMed

    Barbetakis, Nikolaos; Xenikakis, Theocharis; Efstathiou, Andreas; Fessatidis, Ioannis

    2007-01-01

    A 71-year-old male patient was admitted with methicillin-resistant staphylococcus aureus mediastinitis two months after coronary artery bypass grafting. Treatment with immediate surgical debridement, removal of sternal wires and use of vacuum-assisted closure device was started. Spiral computerised tomography and aortography revealed a false aortic aneurysm at the cannulation site. Active mediastinitis and the patient's objection to further surgery led us to perform percutaneous coil embolisation. No postoperative complication was observed and one year later the patient is in excellent condition.

  9. Acute Leukemia and Concurrent Mediastinal Germ Cell Tumor: Case Report and Literature Review.

    PubMed

    Maese, Luke; Li, K David; Xu, Xinjie; Afify, Zeinab; Paxton, Christian N; Putnam, Angelica

    2017-04-01

    There is a known association of primary nonseminomatous mediastinal germ cell tumors (NSMGCT) and hematologic malignancy in younger males not linked to treatment. When combined these two rare entities convey a very poor prognosis. Here we report a 16-year-old male with an anterior mediastinal mass diagnosed as a malignant germ cell tumor based on elevation of serologic markers. He was found to have acute leukemia with megakaryocytic differentiation several days later. We focus our report on the pathologic findings, including a review of the literature, and a novel molecular analysis of the germ cell tumor.

  10. Preoperative Localization of Mediastinal Parathyroid Adenoma with Intra-arterial Methylene Blue.

    PubMed

    Salman, Rida; Sebaaly, Mikhael G; Wehbe, Mohammad Rachad; Sfeir, Pierre; Khalife, Mohamad; Al-Kutoubi, Aghiad

    2017-06-01

    Ectopic parathyroid is found in 16% of patients with hyperparathyroidism. 2% of ectopic parathyroid adenomas are not accessible to standard cervical excision. In such cases, video-assisted thoracoscopic resection is the recommended definitive treatment. We present a case of mediastinal parathyroid adenoma localized preoperatively by injecting methylene blue within a branch of the internal mammary artery that is supplying the adenoma. Intra-arterial methylene blue injection facilitated visualization and resection of the adenoma. The preoperative intra-arterial infusion of methylene blue appears to be an effective and safe method for localization of ectopic mediastinal parathyroid adenomas and allows rapid identification during thoracoscopic resection.

  11. Localized idiopathic fibrosing mediastinitis as a cause of superior vena cava syndrome: a case report

    PubMed Central

    Baslaim, Ghassan; deVarennes, Benoit

    1998-01-01

    An unusual case of superior vena cava syndrome, caused by localized fibrosing mediastinitis is presented. A 41-year-old woman had swelling of the face and upper extremities, associated with headache and fatigue. Radiologic investigations, which included venography, computed tomography and magnetic resonance imaging of the chest, documented the presence of superior vena caval (SVC) obstruction secondary to what appeared to be an intraluminal tumour. The patient underwent SVC resection and reconstruction with a spiral saphenous vein graft under cardiopulmonary bypass and deep hypothermic circulatory arrest. On histopathological examination localized idiopathic fibrosing mediastinitis causing SVC obstruction was diagnosed. PMID:9492750

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

    PubMed

    Naidoo, Mergan; Govind, Mayuri

    2016-03-30

    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.

  13. Mediastinal emphysema in mountain climbers: report of two cases and review.

    PubMed

    Vosk, A; Houston, C S

    1977-01-01

    Two cases of mediastinal emphysema occurring in healthy individuals climbing to the summit of Mount Rainier (4,393 m.) are described. In both, the condition was probably attributable to a voluntary, forced pressure breathing technique--a modified Valsalva maneuver. The various circumstances in which mediastinal emphysema may occur are discussed, along with its possible serious complication of cardiorespiratory compromise. The means of diagnosis are presented, emphasizing that cases in which the important finding of subcutaneous emphysema is absent may be those in which the danger is greatest. Therapeutic measures that occasionally may be necessary are outlined, though the condition is usually a benign one that subsides without specific treatment.

  14. Non-Candida albicans Candida mediastinitis of odontogenic origin in a diabetic patient.

    PubMed

    Kofteridis, Diamantis P; Mantadakis, Elpis; Karatzanis, Alexander D; Bourolias, Constantinos A; Papazoglou, Georgios; Velegrakis, George A; Samonis, George

    2008-06-01

    Descending mediastinitis occurs as a complication of oropharyngeal or cervical infections and its delayed diagnosis and treatment are associated with high mortality. A rare case of an odontogenic infection in a diabetic patient, complicated by Candida parapsilosis and Candida krusei parapharyngeal space infection, descending mediastinitis and aspiration pneumonia is described. Isolate identification was based on colonial and microscopic morphological characteristics and carbohydrate assimilation test results. The patient was successfully treated with surgical drainage and debridement, broad spectrum antibacterials and liposomal amphotericin B followed by prolonged oral voriconazole therapy.

  15. Primary mature cystic teratoma with carcinoid mimicking an adrenal tumor: Report of a rare association and review of literature.

    PubMed

    Narla, Swetha Lakshmi; Jacob, Sheba; Kurian, Ann; Parameswaran, Ashok

    2016-01-01

    Teratomas are neoplasms comprising of tissues derived from more than one germ layer. Most common sites are gonads, sacrococcygeal, mediastinal, retroperitoneal, and pineal region. Here, we report a case of 2 -year-old previously healthy female toddlerwho presented with fever and right upper quadrant abdominal pain. Magnetic resonance imaging (MRI) of the abdomen revealed a right suprarenal nonenhancing cystic mass. Focal calcification was seen in the inferomedial aspect of the cyst. Complete surgical resection of the right adrenal gland was done. The patient had an uneventful postoperative course and was free of recurrence at 8 months follow-up. Primary retroperitoneal teratomas of the adrenal gland are very uncommon (4% of all primary teratomas) and can mimic a primary adrenal tumor, hence should be included in the differential diagnosis of adrenal masses.

  16. Negative sweat tests and cystic fibrosis.

    PubMed Central

    Sarsfield, J K; Davies, J M

    1975-01-01

    Two brothers are described with chronic suppurative pulmonary disease. One has classical cystic fibrosis with complete pancreatic involvement and abnormal sweat test. The other had incomplete pancreatic disease with repeatedly normal sweat tests. The implications of a negative sweat test in patients with cystic fibrosis are discussed. Images FIG. PMID:1147688

  17. Cystic Lung Diseases: Algorithmic Approach.

    PubMed

    Raoof, Suhail; Bondalapati, Praveen; Vydyula, Ravikanth; Ryu, Jay H; Gupta, Nishant; Raoof, Sabiha; Galvin, Jeff; Rosen, Mark J; Lynch, David; Travis, William; Mehta, Sanjeev; Lazzaro, Richard; Naidich, David

    2016-10-01

    Cysts are commonly seen on CT scans of the lungs, and diagnosis can be challenging. Clinical and radiographic features combined with a multidisciplinary approach may help differentiate among various disease entities, allowing correct diagnosis. It is important to distinguish cysts from cavities because they each have distinct etiologies and associated clinical disorders. Conditions such as emphysema, and cystic bronchiectasis may also mimic cystic disease. A simplified classification of cysts is proposed. Cysts can occur in greater profusion in the subpleural areas, when they typically represent paraseptal emphysema, bullae, or honeycombing. Cysts that are present in the lung parenchyma but away from subpleural areas may be present without any other abnormalities on high-resolution CT scans. These are further categorized into solitary or multifocal/diffuse cysts. Solitary cysts may be incidentally discovered and may be an age related phenomenon or may be a remnant of prior trauma or infection. Multifocal/diffuse cysts can occur with lymphoid interstitial pneumonia, Birt-Hogg-Dubé syndrome, tracheobronchial papillomatosis, or primary and metastatic cancers. Multifocal/diffuse cysts may be associated with nodules (lymphoid interstitial pneumonia, light-chain deposition disease, amyloidosis, and Langerhans cell histiocytosis) or with ground-glass opacities (Pneumocystis jirovecii pneumonia and desquamative interstitial pneumonia). Using the results of the high-resolution CT scans as a starting point, and incorporating the patient's clinical history, physical examination, and laboratory findings, is likely to narrow the differential diagnosis of cystic lesions considerably. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

  18. [Bronchopulmonary infection in cystic fibrosis].

    PubMed

    Munck, Anne; Bingen, Edouard

    2003-01-15

    Bronchopulmonary infection determines the vital prognosis of the patients with cystic fibrosis. Following Staphylococcus aureus infection, patients are colonized or cocolonized by Pseudomonas aeruginosa, greatly involved in the pulmonary deterioration; intensive antibiotic treatment of primocolonisation helps to prevent or delay chronic colonisation. Chronic colonization needs a rational long term antibiotic strategy to prevent the occurrence of multiresistant germs; antibiotic cures are performed every 3 or 4 months before pulmonary exacerbation symptoms. Antibiotherapy, physiotherapy and nutritional management helps to increase the survival and quality of life.

  19. 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. Copyright © 2016 Elsevier Inc. All rights reserved.

  20. Cystic schwannoma of the pelvis.

    PubMed

    Jindal, T; Mukherjee, S; Kamal, M R; Sharma, R K; Ghosh, N; Mandal, S N; Das, A K; Karmakar, D

    2013-01-01

    Schwannomas are benign tumours that arise from the Schwann cells of nerve fibres. They commonly occur in the head and neck, mediastinum and extremities. They are extremely rare in the pelvis. These are usually slow-growing tumours and are often detected incidentally. Preoperative diagnosis is extremely difficult as there are no definitive signs on imaging. Aspiration biopsy is often inconclusive or misleading. Surgical excision is both diagnostic and therapeutic. As these tumours are often large in size, open excision is most commonly performed. We describe a case of a large, cystic schwannoma of the pelvis causing bladder outlet obstruction and bilateral hydroureteronephrosis. Complete surgical excision was performed laparoscopically.

  1. Stridor due to an innominate artery compression and posterior mediastinal mass in a pediatric patient.

    PubMed

    Abraham, Edwin; Parray, Tariq; Poteet-Schwartz, Kim

    2012-06-01

    There are many causes for stridor in a pediatric patient. We present an interesting case of a pediatric patient who had stridor due to an innominate artery compression and posterior mediastinal mass. We discuss the anesthetic complication and management of patients with stridor.

  2. Optimal treatment of cervical necrotizing fasciitis associated with descending necrotizing mediastinitis.

    PubMed

    Karkas, A; Chahine, K; Schmerber, S; Brichon, P-Y; Righini, C A

    2010-04-01

    Cervical necrotizing fasciitis (CNF) associated with descending necrotizing mediastinitis (DNM) is a rapidly evolving and life-threatening condition. The aim of this retrospective study was to describe a treatment strategy for CNF with DNM and present a management algorithm for mediastinal extensions of CNF. Patients diagnosed and treated for CNF with DNM over 14 years in a tertiary referral centre were included. Seventeen adult patients were included. The origin of infection was mainly oropharyngeal. The diagnosis of CNF/DNM was based on clinical and computed tomography findings. All patients underwent cervicotomy for CNF. In ten patients, DNM was located above the carina and could be accessed by a cervical approach. In seven patients, DNM was below the carina, and necessitated sternotomy for anteroinferior mediastinal involvement and posterolateral thoracotomy for posteroinferior mediastinal involvement. All patients received broad-spectrum antibiotics. One patient died 3 days after surgery. The median hospital stay was 30 days. There was no recurrence during long-term follow-up. Prompt diagnosis and early surgical treatment are essential for reducing mortality in CNF/DNM. All patients should undergo extensive cervicotomy. The surgical approach to the mediastinum depends on the supracarinal or infracarinal location of the disease. Copyright (c) 2010 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd.

  3. A case of an anaplastic meningioma metastasizing to the mediastinal lymph nodes

    PubMed Central

    Nishida, Norihiro; Kanchiku, Tsukasa; Imajo, Yasuaki; Suzuki, Hidenori; Yoshida, Yuichiro; Kato, Yoshihiko; Hoshii, Yoshinobu; Taguchi, Toshihiko

    2016-01-01

    Context Grade II and III (World Health Organization classification) meningiomas rarely develop in the spinal cord. However, we experienced a case with an anaplastic meningioma that developed in the spinal cord at the cervicothoracic junction and metastasized to the mediastinal lymph nodes. No such cases have previously been reported. Findings The patient was a 68-year-old man who developed back pain that did not affect his daily living. He developed left lower limb paralysis, and was admitted after magnetic resonance imaging (MRI) revealed an intramedullary tumor at the level of cervical vertebra 7 and thoracic vertebra 1. Positron emission tomography revealed tracer uptake in the intramedullary tumor and the mediastinal lymph nodes, suggesting a metastatic spinal cord tumor or malignant lymphoma. A lymph node biopsy was then performed. Although the tumor was highly malignant, its primary site was not identified. Detailed examinations by several other departments revealed no abnormalities. On hospital day 30, his left lower limb paralysis deteriorated, and MRI revealed that the tumor had grown. Thus, laminaplasty, laminectomy, and tumor resection were performed. The tumor was an anaplastic meningioma that resembled mediastinal lymph node tissue, and other tumor lesions were not found. These findings suggested that an anaplastic meningioma had metastasized to the mediastinal lymph nodes. The patient did not respond to radiotherapy, and he was transferred to another hospital. Conclusion In cases of intramedullary spinal tumors with metastasis without other potential primary tumor lesions, early diagnosis and treatment should be performed while considering anaplastic meningioma. PMID:25738779

  4. Intra and interfraction mediastinal nodal region motion: implications for internal target volume expansions.

    PubMed

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

    2009-01-01

    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 ( approximately 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. The role of surgery in diagnosis and treatment of mediastinal malignancies.

    PubMed

    Neef, H

    1990-01-01

    Primary mediastinal malignancies should be treated aggressively using a multidisciplinary approach since some are curable. Surgery is indicated in almost all cases of mediastinal masses with the exception of malignant lymphomas. The question of operability must be placed at the beginning of diagnostic measures in a mediastinal mass. Exact decisions about local operability are often only possible after thoracotomy. Long lasting diagnostic maneuvers are therefore to be avoided. Histological assessment of masses in the middle or anterior mediastinum is possible by mediastinoscopy or other methods. Thus in malignant lymphomas or metastases thoracotomy can be avoided. In the other mediastinal spaces exact histology remains unknown until thoracotomy. If exploration shows inoperability, mass reduction is performed to decrease the space-taking process and to provide better chances for radiotherapy and chemotherapy. Primary radiotherapy and/or chemotherapy are indicated in malignant lymphomas and germ cell tumors. After these measures the indication for surgical excision in tumors has to be proved. X-ray screening techniques need to be improved to aid in early detection, higher extirpation rates, and thus better chances for cure. Because the amount of cases in individual departments is low multicenter collaboration will be required to define the optimal combined modality approach.

  6. [Thymus in posterior mediastinal localization. Apropos of a case in a child].

    PubMed

    Canarelli, J P; Pautard, J C; Baratte, B; Doidy, L; Ricard, J

    1992-01-01

    Posterior and upper mediastinal localization of the thymus gland is uncommon in young children. Presenting symptoms and signs are very variable from incidental diagnosis to bronchopneumonial with atelectasias of the left upper Chest X-Ray and CT Scan are sufficient to make the diagnosis. Treatment of complicated forms with bronchial compression requires thoracotomy and surgical excision.

  7. Cranial vena cava syndrome secondary to cryptococcal mediastinal granuloma in a cat

    PubMed Central

    Letendre, Jo-Annie; Boysen, Søren

    2015-01-01

    The successful management of cranial vena cava syndrome with suspected secondary chylothorax due to mediastinal cryptococcal granuloma in a 4-year-old male domestic shorthair cat is described. Treatment included long-term antifungal medication, short-term corticosteroids, intermittent thoracocentesis, rutin, octreotide, and enalapril. PMID:25829555

  8. [Mediastinal parathyroid adenomas on a 5th ectopic gland. 2 case reports].

    PubMed

    Emy, P; Combe, H; Marchand, J P; Villeneuve, A; Sicre, G; Chadenas, D

    1992-11-01

    Mediastinal parathyroid adenoma located on the 5th ectopic gland is rare. We report here two new cases diagnosed by scintigraphy. In one case the adenoma was found to be located in the mediastinum prior to cervicotomy. The modern imaging methods capable of locating parathyroid adenomas are evaluated.

  9. Postoperative mediastinitis in cardiovascular surgery postoperation. Analysis of 1038 consecutive surgeries.

    PubMed

    Sá, Michel Pompeu Barros de Oliveira; Silva, Débora Oliveira; Lima, Erika Nibbering de Souza; Lima, Ricardo de Carvalho; Silva, Frederico Pires Vasconcelos; Rueda, Fábio Gonçalves de; Escobar, Rodrigo Renda de; Cavalcanti, Paulo Ernando Ferraz

    2010-01-01

    To report the incidence of mediastinitis in cardiovascular surgery postoperation. The records of all 1038 patients who underwent cardiovascular surgical procedures between May/2007 and June/2009 were reviewed. All operations were performed in Division of Cardiovascular Surgery of Pronto Socorro Cardiológico de Pernambuco - PROCAPE. The complication occurred within, on average, 13 days after operation, in total of 25 (2.4%), eight (32%) deaths occurred. Several risk factors mediastinitis were identified: 56% diabetes, 56% smokers, 20% obeses, 16% with chronic obstructive pulmonary disease and 8% of chronic renal failure. Mediastinitis were reported in 21 (84%) cases of patients submitted to coronary artery bypass grafting, being associated to major risk of infection development (IC 3.448.30, P=0.0001). High rates of complications were observed: respiratory insufficiency (44%), stroke (16%), cardiogenic shock (12%), acute renal failure (28%), pulmonary infection (36%), multiple organs failure (16%) and esternal deiscence (48%). Bacterial cultures of exudates were positive in 84% of patients; Staphylococcus aureus was the most responsible pathogen (28.8%). Mediastinitis stays a serious surgical complication and difficult management in cardiovascular surgery postoperation. The disease stays with low incidence, but still with high lethality. Coronary bypass was associated to major risk of infection development.

  10. Benign Mature Mediastinal Dysembryoma with Pulmonary Extension Revealed by Recurrent Hemoptysis in a Young Woman

    SciTech Connect

    Filaire, M.; Michel-Letonturier, M.; Garcier, J. M.; Escande, G.; Boyer, L.

    2006-06-15

    We report one case of mature mediastinal teratoma with pulmonary extension surgically diagnosed in a 22-year-old woman complaining of recurrent hemoptyses for which no etiological explanation could be found. Thoracic surgery was only decided on after three embolizations proved ineffective.

  11. Thoracoscopic resection with intraoperative use of methylene blue to localize mediastinal parathyroid adenomas.

    PubMed

    Adachi, Yoshin; Nakamura, Hiroshige; Taniguchi, Yuji; Miwa, Ken; Fujioka, Shinji; Haruki, Tomohiro

    2012-03-01

    We report a case of thoracoscopic resection of mediastinal parathyroid adenomas using methylene blue to localize the tumors during the operation. After methylene blue 4 mg/kg was injected intravenously, we easily identified methylene blue-stained parathyroid glands and successfully resected them with sufficient surgical margins. The use of methylene blue for detection of parathyroid adenoma is a useful technique.

  12. Surgical management of cervico-mediastinal goiters: Our experience and review of the literature.

    PubMed

    Di Crescenzo, V; Vitale, M; Valvano, L; Napolitano, F; Vatrella, A; Zeppa, P; De Rosa, G; Amato, B; Laperuta, P

    2016-04-01

    We analyze and discuss the clinical presentation, the diagnostic procedures and the surgical technique in relation to post-operative complications and results in cervico-mediastinal thyroid masses admitted in Thoracic Surgery Unit of AOU Second University of Naples from 1991 to 2006 and in Thoracic Surgery Unit of AOU "S. Giovanni di Dio & Ruggi D'Aragona" of Salerno over a period of 3 years (2011-2014). We reviewed 97 patients who underwent surgical treatment for cervico-mediastinal goiters. 47 patients (49.2%) had cervico-mediastinal goiter, 40 patients (40%) had mediastino-cervical goiter and 10 patients (10.8%) had mediastinal goiter. 73 cases were prevascular goiters and 24 were retrovascular goiters. We performed total thyroidectomy in 40 patients, subtotal thyroidectomy in 46 patients and in 11 cases the resection of residual goiter. In 75 patients we used only a cervical approach, in 21 patients the cervical incision was combined with median sternotomy and in 1 patient with transverse sternotomy. Three patients (3.1%) died in the postoperative period (2 cardio-respiratory failure and 1 pulmonary embolism). The histologic study revelead 8 (7.7%) carcinomas. Postoperative complications were: dyspnea in 9 cases (10.7%), transient vocal cord paralysis in 6 patients (9.2%), temporary hypoparathyroidism in 9 patients (9.2%) and kidney failure in 1 case (0.9%). The presence of a cervico-mediastinal thyroid mass with or without respiratory distress requires a surgical excision as the only treatment option. Thyroid masses extending to the mediastinum can be excised successfully by cervical incision. Bipolar approach (cervical incision and sternotomy) has an excellent outcome, achieving a safe resection, especially in large thyroid masses extending to the mediastinum with close relations to mediastinal structures and in some limited cases (carcinoma, thyroiditis, retrovascular goiter, ectopic goiter). Postoperative mortality and morbidity is very low, independent of

  13. Anterior mediastinal fat in Behçet's disease: qualitative and quantitative CT analysis.

    PubMed

    Lee, Sang Yub; Lee, Jongmin; Lee, Hui Joong; Choi, Sun Ju; Hahm, Myong Hun; Yoon, Sung Won

    2013-12-01

    The fat-rich anterior mediastinum could be a sensitive window for monitoring minute changes in vascularity induced by systemic vasculitis. To evaluate this hypothesis, an analysis of anterior mediastinal fat in patients with Behçet's disease and a control group was conducted. This study included 43 patients diagnosed with Behçet's disease within the last 11 years who underwent CT scan; 55 patients were selected as a control population. Mediastinal fat was classified according to CT morphology. Comparison of serum inflammatory markers was performed for evaluation of disease activity according to morphologic types, and average Hounsfield unit of the anterior mediastinum was measured. Significantly higher mean CT attenuation was observed in the Behçet's disease group, compared with the control group (-48.5 ± 33.5 vs. -67.7 ± 18.7, respectively, P < 0.05). Mediastinal fat types were classified as follows: pure fatty tissue (2 vs. 31 % [Behçet's disease vs. control group]), diffuse soft tissue infiltration (16 vs. 29 %), tubular structures (21 vs. 4 %), mixed infiltration with tubular structures (42 vs. 15 %), and evident thymic tissue (19 vs. 22 %). The value for mean mediastinal attenuation was significantly higher in the group with a high level of C-reactive protein than in the normal level group. The mean CT attenuation of anterior mediastinal fat is significantly higher in the Behçet's disease group, compared with the normal group. Although pathologic confirmation is needed, the cause is postulated to be either inflammatory neovascularization or minimal thymic hyperplasia induced by Behçet's disease.

  14. 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

  15. Machine learning in the prediction of cardiac epicardial and mediastinal fat volumes.

    PubMed

    Rodrigues, É O; Pinheiro, V H A; Liatsis, P; Conci, A

    2017-02-24

    We propose a methodology to predict the cardiac epicardial and mediastinal fat volumes in computed tomography images using regression algorithms. The obtained results indicate that it is feasible to predict these fats with a high degree of correlation, thus alleviating the requirement for manual or automatic segmentation of both fat volumes. Instead, segmenting just one of them suffices, while the volume of the other may be predicted fairly precisely. The correlation coefficient obtained by the Rotation Forest algorithm using MLP Regressor for predicting the mediastinal fat based on the epicardial fat was 0.9876, with a relative absolute error of 14.4% and a root relative squared error of 15.7%. The best correlation coefficient obtained in the prediction of the epicardial fat based on the mediastinal was 0.9683 with a relative absolute error of 19.6% and a relative squared error of 24.9%. Moreover, we analysed the feasibility of using linear regressors, which provide an intuitive interpretation of the underlying approximations. In this case, the obtained correlation coefficient was 0.9534 for predicting the mediastinal fat based on the epicardial, with a relative absolute error of 31.6% and a root relative squared error of 30.1%. On the prediction of the epicardial fat based on the mediastinal fat, the correlation coefficient was 0.8531, with a relative absolute error of 50.43% and a root relative squared error of 52.06%. In summary, it is possible to speed up general medical analyses and some segmentation and quantification methods that are currently employed in the state-of-the-art by using this prediction approach, which consequently reduces costs and therefore enables preventive treatments that may lead to a reduction of health problems.

  16. Comparison of the clinical features and hematopoietic stem cell transplantation outcomes of mediastinal malignant germ cell tumors with nonmediastinal extragonadal placements

    PubMed Central

    Ocal, Nesrin; Yildiz, Birol; Karadurmus, Nuri; Dogan, Deniz; Ozaydin, Sukru; Ocal, Ramazan; Ozturk, Mustafa; Arpaci, Fikret; Bilgic, Hayati

    2016-01-01

    Objective Even though the primary mediastinal extragonadal germ cell tumors (EGCTs) are rare, they are noteworthy in the differential diagnosis of mediastinal masses. In this study, we aimed to identify the clinical features of mediastinal malignant GCTs and compare the results of hematopoietic stem cell transplantation between mediastinal and nonmediastinal malignant EGCTs. Method Data of the patients with EGCT who were treated and underwent hematopoietic stem cell transplantation at our hospital between 1988 and 2015 were retrieved retrospectively. Results were compared between mediastinal and nonmediastinal EGCTs. Results Data of 65 patients diagnosed with EGCT (37 [56.92%] cases with mediastinal EGCT and 28 [43.07%] cases with nonmediastinal EGCT) were assessed. The clinical stages, frequency of pretransplant status, mean pretransplant time, and mean number of chemotherapy lines before hematopoietic stem cell transplantation were not significantly different between groups. Although the overall survival did not significantly differ between groups, the 5-year survival was significantly higher in mediastinal EGCTs (P=0.02). Yolk sac tumor was significantly more common in mediastinal EGCTs (P=0.05). Mortality rates were higher in seminomas and yolk sac tumors in all cases, higher in embryonal carcinomas in mediastinal EGCT group and higher in yolk sac tumors in nonmediastinal EGCT group. While choriocarcinomas had more aggressive courses in mediastinal EGCTs, seminomas and yolk sac tumors had poorer prognosis in nonmediastinal EGCTs. Short pretransplant time and persistence of elevated posttransplant βhCG and AFP levels were the significant mortality risk factors both in mediastinal and nonmediastinal EGCTs. Conclusion Mediastinal placement of EGCT was not a poor prognostic factor; furthermore, the 5-year survival was significantly higher in mediastinal EGCTs. According to our knowledge, this is the first study that compares the clinical outcomes of hematopoietic

  17. 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

  18. Androgen excess in cystic acne.

    PubMed

    Marynick, S P; Chakmakjian, Z H; McCaffree, D L; Herndon, J H

    1983-04-28

    We measured hormone levels in 59 women and 32 men with longstanding cystic acne resistant to conventional therapy. Affected women had higher serum levels of dehydroepiandrosterone sulfate, testosterone, and luteinizing hormone and lower levels of sex-hormone-binding globulin than controls. Affected men had higher levels of serum dehydroepiandrosterone sulfate and 17-hydroxyprogesterone and lower levels of sex-hormone-binding globulin than controls. To lower dehydroepiandrosterone sulfate, dexamethasone was given to men, and dexamethasone or an oral contraceptive pill, Demulen (or both), was given to women. Of the patients treated for six months, 97 per cent of the women and 81 per cent of the men had resolution or marked improvement in their acne. The dose of dexamethasone required to reduce dehydroepiandrosterone sulfate levels was low, rarely exceeding the equivalent of 20 mg of hydrocortisone per day. We conclude that most patients with therapeutically resistant cystic acne have androgen excess and that lowering elevated dehydroepiandrosterone sulfate results in improvement or remission of acne in most instances.

  19. Aspergillus infections in cystic fibrosis.

    PubMed

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

    2016-07-05

    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. Copyright © 2016. Published by Elsevier Ltd.

  20. Internal irradiation for cystic craniopharyngioma

    SciTech Connect

    Kobayashi, T.; Kageyama, N.; Ohara, K.

    1981-12-01

    The authors report the results of internal irradiation with labeled chromic phosphate (32P) and gold-198 (198Au) colloid in eight cases of cystic craniopharyngiomas. They used a newly developed dosimetric formula, by which the radiation dose at the cyst wall and at any point far from the radioactive source can be calculated. Ten courses of irradiation in eight patients were carried out by injection of either 32P or 198Au colloid into the cyst through an Ommaya drainage system that had been placed at craniotomy. Follow-up studies ranging from 13 to 156 months revealed that all cysts were effectively treated, with elimination of fluid or collapse of the cyst. This was confirmed by Conray cystography and/or computerized tomography. Not only the dose delivered to the wall but also the thickness of the cyst wall and the location of the cyst are important factors in planning internal irradiation. A safe and adequate dose to the cyst wall could range between 9000 to 30,000 rads for craniopharyngioma. This treatment is suitable for large cysts that are thought to be difficult to remove radically, recurrent cysts resistant to previous treatment, or multiple cysts. Internal irradiation may also be applicable in other cystic intracranial tumors if dosimetry is calculated accurately.

  1. Two patients with TAFRO syndrome exhibiting strikingly similar anterior mediastinal lesions with predominantly fat attenuation on chest computed tomography.

    PubMed

    Ozawa, Yoko; Yamamoto, Hiroshi; Yasuo, Masanori; Takahashi, Hidekazu; Tateishi, Kazunari; Ushiki, Atsuhito; Kawakami, Satoshi; Fujinaga, Yasunari; Asaka, Shiho; Sano, Kenji; Takayama, Hiroshi; Imamura, Hiroshi; Hanaoka, Masayuki

    2017-03-01

    We herein report on two middle-aged men with TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis or renal failure, and organomegaly) syndrome, a unique clinicopathological variant of multicentric Castleman׳s disease recently proposed in Japan. Strikingly similar anterior mediastinal fat swellings with soft tissue density were observed in the patients on chest computed tomography. In TAFRO syndrome, bilateral pleural effusion and slight lymph node swelling are common in the thoracic region; however, anterior mediastinal lesions have not been previously observed. Although the mechanisms of anterior mediastinal lesions have not been defined, these lesions seem to have a close relationship with TAFRO syndrome.

  2. Voice Disorder in Cystic Fibrosis Patients

    PubMed Central

    Lourenço, Bruna Mendes; Costa, Kauê Machado; da Silva Filho, Manoel

    2014-01-01

    Cystic fibrosis is a common autosomal recessive disorder with drastic respiratory symptoms, including shortness of breath and chronic cough. While most of cystic fibrosis treatment is dedicated to mitigating the effects of respiratory dysfunction, the potential effects of this disease on vocal parameters have not been systematically studied. We hypothesized that cystic fibrosis patients, given their characteristic respiratory disorders, would also present dysphonic symptoms. Given that voice disorders can severely impair quality of life, the identification of a potential cystic fibrosis-related dysphonia could be of great value for the clinical evaluation and treatment of this disease. We tested our hypothesis by measuring vocal parameters, using both objective physical measures and the GRBAS subjective evaluation method, in male and female cystic fibrosis patients undergoing conventional treatment and compared them to age and sex matched controls. We found that cystic fibrosis patients had a significantly lower vocal intensity and harmonic to noise ratio, as well as increased levels of jitter and shimmer. In addition, cystic fibrosis patients also showed higher scores of roughness, breathiness and asthenia, as well as a significantly altered general grade of dysphonia. When we segregated the results according to sex, we observed that, as a group, only female cystic fibrosis patients had significantly lower values of harmonic to noise ratio and an abnormal general grade of dysphonia in relation to matched controls, suggesting that cystic fibrosis exerts a more pronounced effect on vocal parameters of women in relation to men. Overall, the dysphonic characteristics of CF patients can be explained by dysfunctions in vocal fold movement and partial upper airway obstruction, potentially caused by the accumulation of mucus and chronic cough characteristic of CF symptomatology. Our results show that CF patients exhibit significant dysphonia and suggest they may

  3. Voice disorder in cystic fibrosis patients.

    PubMed

    Lourenço, Bruna Mendes; Costa, Kauê Machado; da Silva Filho, Manoel

    2014-01-01

    Cystic fibrosis is a common autosomal recessive disorder with drastic respiratory symptoms, including shortness of breath and chronic cough. While most of cystic fibrosis treatment is dedicated to mitigating the effects of respiratory dysfunction, the potential effects of this disease on vocal parameters have not been systematically studied. We hypothesized that cystic fibrosis patients, given their characteristic respiratory disorders, would also present dysphonic symptoms. Given that voice disorders can severely impair quality of life, the identification of a potential cystic fibrosis-related dysphonia could be of great value for the clinical evaluation and treatment of this disease. We tested our hypothesis by measuring vocal parameters, using both objective physical measures and the GRBAS subjective evaluation method, in male and female cystic fibrosis patients undergoing conventional treatment and compared them to age and sex matched controls. We found that cystic fibrosis patients had a significantly lower vocal intensity and harmonic to noise ratio, as well as increased levels of jitter and shimmer. In addition, cystic fibrosis patients also showed higher scores of roughness, breathiness and asthenia, as well as a significantly altered general grade of dysphonia. When we segregated the results according to sex, we observed that, as a group, only female cystic fibrosis patients had significantly lower values of harmonic to noise ratio and an abnormal general grade of dysphonia in relation to matched controls, suggesting that cystic fibrosis exerts a more pronounced effect on vocal parameters of women in relation to men. Overall, the dysphonic characteristics of CF patients can be explained by dysfunctions in vocal fold movement and partial upper airway obstruction, potentially caused by the accumulation of mucus and chronic cough characteristic of CF symptomatology. Our results show that CF patients exhibit significant dysphonia and suggest they may

  4. Fungi in cystic fibrosis and non-cystic fibrosis bronchiectasis.

    PubMed

    Moss, Richard B

    2015-04-01

    Bronchiectasis is a pathologic bronchial dilatation with loss of function that can result from multiple inflammatory and infectious injuries to the conducting airways of the lung. Molds, particularly the filamentous fungus Aspergillus fumigatus, have been implicated as a common cause of both cystic fibrosis (CF) and non-CF bronchiectasis, the latter primarily in patients with severe asthma. The pathogenesis of mold-associated bronchiectasis is usually due to atopic sensitization to mold allergens in the presence of active chronic endobronchial fungal infection with host innate and adaptive immune deviation to a Th2-dominated inflammation, a condition known as allergic bronchopulmonary aspergillosis (ABPA) (or allergic bronchopulmonary mycosis if a non-Aspergillus mold is implicated). Diagnostic criteria of ABPA continue to evolve, while treatment relies upon downregulation of the allergic inflammatory response with immunomodulatory agents and antifungal pharmacotherapy.

  5. Cystic avascular necrosis of the triquetrum.

    PubMed

    Albtoush, Omar M; Esmadi, Mohammad; Al-Omari, Mamoon H

    2013-01-01

    Carpal bones are rarely affected by avascular necrosis (AVN) in the absence of fractures. The lunate is the most frequently affected carpal bone, followed by the scaphoid and the capitate. The triquetrum is rarely affected by AVN. We report a case of multiple cystic changes in the triquetrum in a patient with a history of trauma. He was treated by below elbow Colles plaster cast for 3 months, with no improvement. Cystic changes resulted from irreversible AVN of the triquetrum. This is the first case to be reported in the literature with cystic AVN changes in the triquetrum.

  6. Cystic Pheochromocytoma Presenting as Adrenal Cyst

    PubMed Central

    Abdulsalam, Mohammed Shafi; Satish, Priyanka; Janakiraman, Raghunath Keddy; Singh, Shivshankar

    2016-01-01

    Pheochromocytomas are usually solid tumours. But it can present as cystic lesions in the adrenal gland. Cystic lesions in adrenal gland with hypertension needs attention to rule out pheochromocytoma. If ignored, it may lead to hypertensive emergency, multisystem crisis and death. Early diagnosis with biochemistry, Computed Tomography (CT) or Magnetic Resonance Imaging (MRI) of abdomen, proper functional imaging like Meta Iodo Benzyl Guanidine (MIBG) scan is essential. Proper preoperative preparation is important to prevent hypertensive crisis during and after surgery. We are reporting a case of cystic pheochromocytoma in a young male. PMID:28050427

  7. Cystic Pheochromocytoma Presenting as Adrenal Cyst.

    PubMed

    Abdulsalam, Mohammed Shafi; Ganapathy, Vijaya; Satish, Priyanka; Janakiraman, Raghunath Keddy; Singh, Shivshankar

    2016-11-01

    Pheochromocytomas are usually solid tumours. But it can present as cystic lesions in the adrenal gland. Cystic lesions in adrenal gland with hypertension needs attention to rule out pheochromocytoma. If ignored, it may lead to hypertensive emergency, multisystem crisis and death. Early diagnosis with biochemistry, Computed Tomography (CT) or Magnetic Resonance Imaging (MRI) of abdomen, proper functional imaging like Meta Iodo Benzyl Guanidine (MIBG) scan is essential. Proper preoperative preparation is important to prevent hypertensive crisis during and after surgery. We are reporting a case of cystic pheochromocytoma in a young male.

  8. 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.

  9. Multiple endocrine neoplasia type 1 with anterior mediastinal parathyroid adenoma: successful localization using Tc-99m sestamibi SPECT/CT

    PubMed Central

    Park, Hye Lim; Kim, Sung Hoon; Lee, Sohee

    2016-01-01

    The most common manifestation of multiple endocrine neoplasia type 1 (MEN1) is hyperparathyroidism. Treatment of hyperparathyroidism in MEN patients is surgical removal of the parathyroid glands, however ectopic parathyroid gland is challenging for treatment. A 51-year-old female, the eldest of 3 MEN1 sisters, had hyperparathyroidism with ectopic parathyroid adenoma in the mediastinal para-aortic region, which was detected by technetium-99m (Tc-99m) sestamibi scintigraphy and single-photon emission computed tomography/computed tomography (SPECT/CT). She underwent total parathyroidectomy with video-assisted thoracoscopic surgery on an anterior mediastinal mass. Anterior mediastinal parathyroid adenoma in MEN1 patients is rare. Precise localization of an ectopic parathyroid gland with Tc-99m sestamibi SPECT/CT can lead to successful treatment of hyperparathyroidism. This is the first reported case in the literature of mediastinal parathyroid adenoma in MEN1 patient visualized by Tc-99m sestamibi SPECT/CT. PMID:27904855

  10. Primary mediastinal large B-cell lymphoma arising from thyroid in a renal recipient with Hashimoto's thyroiditis.

    PubMed

    Wu, Fang; Qu, Lu; Li, Dai-Qiang; Hu, Chun-Hong

    2015-01-01

    Primary mediastinal large B-cell lymphoma is a subtype of diffuse large B-cell lymphoma, arising in the mediastinum from putative thymic B-cell origin with distinctive clinical and genetic features. Generally, primary mediastinal large B-cell lymphoma is believed as only deriving in the mediastinum. The current study presents a rare case of primary mediastinal large B-cell lymphoma which arising from thyroid in a renal recipient with Hashimoto's thyroiditis. Moreover, we devoted a discussion to the relationship among primary mediastinal large B-cell lymphoma, immunomodulatory therapy and autoimmune diseases. The immunologic derangement induced by long-term immunomodulatory therapy and Hashimoto's thyroiditis may be the possible cause for the ectopic lymphoma.

  11. Cystic mesothelioma of the peritoneum.

    PubMed

    Datta, R V; Paty, P B

    1997-10-01

    A 48-year-old man presented with a 3-month history of weight loss and progressive right lower quadrant abdominal pain. His medical history was notable for appendectomy at age 17. Ultrasonography and computed tomography of the abdomen revealed a 12 cm multicystic mass in the right paracolic space. At laparotomy a large serous cyst was found arising from the lateral wall of the cecum, and four additional small cysts were found on the small bowel mesentery, greater omentum, liver capsule, and right hemi-diaphragm. Complete removal of the tumor was accomplished by right colectomy with extraperitoneal dissection of the large cyst and simple excision of the four smaller cysts. Final pathology with immunohistochemical staining confirmed cystic mesothelioma of the peritoneum. In this report we discuss the diagnostic workup and treatment of this rare disease.

  12. Laboratory diagnosis of cystic fibrosis.

    PubMed

    Webster, H L

    1983-01-01

    The demonstration of abnormally high concentrations of electrolytes in eccrine sweat is still the only practical laboratory procedure available for diagnosis of cystic fibrosis. Properly performed, the sweat test is very reliable, but there are many published reports that all of the methods in current use frequently generate incorrect diagnoses. Analysis of potential for error in sweat test methods shows that of the three essential phases involved, stimulation, collection, and analysis, the major cause of intrinsic inaccuracy occurs in the collection process. In this case the problem is due to condensate formation, which leads to the subsequent analysis of nonrepresentative sweat. Human error is also an important cause of false results and is a direct function of the number of critical manual operations involved in the technic. This review provides a critical examination of sweat test methods, identifying problem areas and suggesting ways to improve procedures in the interests of clinically reliable laboratory data in support of diagnosis.

  13. Cystic cerebellar astrocytomas in childhood.

    PubMed

    Griffin, T W; Beaufait, D; Blasko, J C

    1979-07-01

    Thirty-nine patients with low grade cystic cerebellar astrocytomas were treated at the University of Washington and Children's Orthopedic Hospital in Seattle, Washington, between 1955 and 1977; 29 were treated with partial or complete resection alone, and 10 received radiation therapy after various types of surgical procedures. With a mean follow-up time of 7 years, the survival rate for patients who had complete resections of their primary disease was 100%. The relapse-free survival rate was 82%. The relapse-free survival rate for patients treated primarily with partial resection alone was 36%. Postoperative irradiation after partial resection for both primary and recurrent disease resulted in a relapse-free survival rate of 83%. If complete tumor excision is not possible, postoperative radiation therapy is recommended following partial resection.

  14. 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

  15. Cystic echinococcosis in southern Brazil.

    PubMed

    de la Rue, Mario L

    2008-01-01

    Cystic echinococcosis (CE) is very common in the southern part of Rio Grande do Sul State where sheep and cattle raising is the most important economical activity. Prevalence in intermediate hosts is well known due to reports in slaughterhouses while human reports are of discussed value. This is due to underreporting of cases and few epidemiological studies make it difficult to assess the situation of echinococcosis in the population. Whereas cattle infection rate is more or less stable around 12% of the slaughter animals, in sheep there has been an increasing number in the last five years. Some efforts have been done to control the zoonosis but no effective results were obtained so far. Probably educational efforts to change human practices (feeding dogs with raw viscera), periodic treatment of dogs with praziquantel and joined actions with slaughterhouses could bring more attention to improve some control measures.

  16. [New tools in cystic fibrosis].

    PubMed

    Dournes, G; De Boeck, K; Bui, S; Vermeulen, F; Ramalho, A; Chateil, J-F; Laurent, F; Fayon, M

    2016-12-01

    The use of 3 novel tools available for the diagnosis and treatment in cystic fibrosis are described here. 1) The lung clearance index is a sensitive method which can detect functional impairment in the first months after birth. 2) Detailed morphological analyses of the lung can be performed with the new MRI sequences, without any contrast medium or risk of radiation. The analysis of functional MRI data (perfusion, diffusion, ventilation, inflammation) will be possible, and these data will be correlated to morphological data. The exploration of other organs such as the sinuses, liver and abdomen during the same examination represents another definite advantage. 3) Organoïds are a good example of personalized medicine. This tool explores CFTR function and treatment response in each of the 2000 or so known CFTR mutations. These tests are limited to specialized centers, mostly within a research context. However, their generalization after standardization is expected in the near future.

  17. Alcaligenes infection in cystic fibrosis.

    PubMed

    Tan, Kenneth; Conway, Steven P; Brownlee, Keith G; Etherington, Christine; Peckham, Daniel G

    2002-08-01

    The aim of this study was to investigate the effect of chronic Alcaligenes species infection of the respiratory tract on the clinical status of patients with cystic fibrosis. We conducted a retrospective case-controlled study. The microbiological records of all patients attending the Leeds Regional Pediatric and Adult Cystic Fibrosis Units from 1992-1999 were examined. Chronic Alcaligenes infection was defined as a positive sputum culture on at least three occasions over a 6-month period. These patients were compared with controls matched for age, gender, respiratory function, and Pseudomonas aeruginosa infection status. Respiratory function tests, anthropometric data, Shwachman-Kulczycki score, Northern chest x-ray score, intravenous and nebulized antibiotic treatment, and corticosteroid treatment were compared from 2 years before to 2 years after Alcaligenes infection. From a clinic population of 557, 13 (2.3%) fulfilled the criteria for chronic infection. The median age at acquisition of infection was 17.2 years (range, 6.5-33.6). There was no significant difference in the changes of percentage predicted values for FEV(1), FVC, FEF(25-75), or Shwachman-Kulczycki and Northern chest x-ray scores, or in weight, height, and body mass index z-scores between Alcaligenes-infected cases and controls. There was also no significant difference in the use of antibiotics (intravenous and nebulized) or corticosteroids (inhaled and oral). We conclude that in our clinic, chronic infection with Alcaligenes species was uncommon. Chronically infected patients showed no excess deterioration in clinical or pulmonary function status from 2 years before to 2 years after primary acquisition.

  18. 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.

  19. [Cystic fibrosis in a woman aged seventy].

    PubMed

    Ras, Janneke E; van Velzen, Edwin; van Berkhout, Ferdinand Teding; van den Brand, Joop J G

    2010-01-01

    A seventy-year-old woman was admitted to hospital with a Staphylococcus aureus respiratory tract infection. She had a history of extensive bronchiectasis and allergic bronchopulmonary aspergillosis (ABPA). Cystic fibrosis (CF) was suspected and cystic fibrosis transmembrane conductance regulator (CFTR) gene analysis showed F508del and R117H-7T mutations. In these mutations there is residual activity in the chloride channel in the cell membrane coded by the CFTR gene. This results in a much milder disease pattern varying from no disease at all to isolated organ disease. This type of disease is known as non-classical cystic fibrosis. In our patient the diagnosis of cystic fibrosis was made exceptionally late in life.

  20. Radiological description of cystic pancreatic tumors.

    PubMed

    Rodríguez Torres, C; Larrosa López, R

    2016-01-01

    Although most cystic pancreatic lesions are pseudocysts, it is important to do a thorough differential diagnosis with true cystic tumors because cystic tumors are potentially malignant. Sometimes computed tomography and magnetic resonance imaging cannot establish the definitive diagnosis, making it necessary to perform other imaging tests such as endoscopic ultrasound, which in addition to morphological information, can also enable cytologic and biochemical analysis of the lesion through puncture and aspiration of its contents. Combining all these findings nearly always provides enough diagnostic information to allow the appropriate approach in each case. This article describes the specific morphological characteristics for each cystic pancreatic tumor on computed tomography, magnetic resonance imaging, and endoscopic ultrasound and reviews the guidelines for managing these types of lesions.

  1. Antibiotic-associated colitis and cystic fibrosis.

    PubMed

    Pokorny, C S; Bye, P T; MacLeod, C; Selby, W S

    1992-09-01

    The use of antibiotics in patients with cystic fibrosis is widespread, and fecal carriage of Clostridium difficile occurs in up to 50% of these patients; however, antibiotic-associated colitis appears to be a rare occurrence. The reasons why this is so remain unknown. A case of antibiotic-associated colitis occurring in a patient with cystic fibrosis is described. Possible mechanisms for the rarity of antibiotic-associated colitis are reviewed and implications for prompt diagnosis and therapy are discussed.

  2. Cervical solitary long segment cystic Schwannoma.

    PubMed

    Vikram, Muthusubramanian; Pande, Anil; Vasudevan, Madhabushi Chakravarthy; Ravi, Ramamurthi

    2010-04-01

    Cystic neurofibromas are very rare and are of separate entity; occurrence in the cervical spine extending over a long segment intra-durally is very rare. There is a paucity of literature available on cervical cystic Schwannoma and its MRI characteristics. As the preoperative diagnosis of these tumors helps in planning the surgery for this rare tumor the imaging findings reported herein may aid in the preoperative diagnosis and management.

  3. [Retrocervical cystic hygroma: about 35 cases].

    PubMed

    Masmoudi, Aida; Neji, Kaled; Kacem, Sémia; Boudhraa, Kaled; Jabnoun, Samy; Chelli, Hella; Reziga, Hédi; Zouari, Faouzia; Khrouf, Naima; Chaabouni, Habiba; Siala, Soumaya

    2002-03-01

    Retrocervical cystic hygroma is a congenital defect associated to chromosomic anomalies. We report a retrospective study about 35 cystic hygroma autopsies colliged in C.M.N.T in 10 years. Antenatal sonography has a sensibility 94.5%. Genetic abnormalities dominated by trisomie 13 Turner syndrome dad found in 11.5%. Medical abortion has done in 48.5%. A multidisciplinary management autorized to understand etiopathogeny of this defect.

  4. Effect of the respiratory cycle on mediastinal and lung dimensions in Hodgkin's disease. Implications for radiotherapy gated to respiration

    SciTech Connect

    Willett, C.G.; Linggood, R.M.; Stracher, M.A.; Goitein, M.; Doppke, K.; Kushner, D.C.; Morris, T.; Pardy, J.; Carroll, R.

    1987-09-15

    Changes in mediastinal and lung dimensions during respiration were studied to assess the potential of radiotherapy gated to respiration to minimize normal tissue irradiation. Twelve patients with mediastinal Hodgkin's disease were assessed using chest radiographs and thoracic computed tomography (CT) scans both during quiet breathing and at maximum inspiration in the standing, supine, and prone positions. A simple measure of the bulk of mediastinal disease, the ratio of the width of mediastinal mass to thoracic diameter, was determined from posteroanterior (PA) chest radiographs. The volumes of mediastinum, irradiated and protected lung if anteroposterior (AP) and PA mantle fields were used were determined from sequential thoracic CT scans and three-dimensional treatment planning and compared at quiet breathing and deep inspiration. The mediastinal width to thoracic diameter ratio decreased from quiet breathing to deep inspiration an average of 3%, 9%, and 11% for the standing, supine, and prone positions, respectively. Lung volumes as measured from the thoracic CT scans showed that on average, 8% more lung was protected at deep inspiration than at quiet breathing, independent of treatment position. The maximum increase in the percentage of protected lung from quiet breathing to deep inspiration was seen in patients with extensive mediastinal adenopathy suggesting that radiotherapy gated to respiration may be most advantageous in the subset of patients.

  5. A case of descending mediastinitis caused by Streptococcus pyogenes harboring genotype emm25 and sequence type 660.

    PubMed

    Ohya, Hiroaki; Mori, Nobuaki; Hayashi, Tetsuro; Minami, Shujiro; Higuchi, Akiko; Takahashi, Takashi

    2017-02-01

    Descending mediastinitis caused by Streptococcus pyogenes (group A streptococcus, GAS) is rare among cases of invasive GAS infection. In this report, we describe a case of a cervical abscess and secondary descending mediastinitis in a previously healthy 39-year-old Japanese man. The patient presented with a 2-week history of a sore throat, and subsequently developed an abscess and descending mediastinitis. We treated the cervical abscess using ampicillin/sulbactam and drainage, and GAS was subsequently isolated in two blood cultures from the patient's admission. Microbiological analyses revealed that the isolate harbored genotype emm25 and sequence type (ST) 660. This strain was susceptible to erythromycin (minimum inhibitory concentration [MIC]: ≤0.12 μg/mL), resistant to minocycline (MIC: >4 μg/mL), and possessed the tet(M) determinant. Although we have reviewed the literature regarding the clinical and microbiological characteristics of descending mediastinitis cause by GAS, little is known regarding epidemiological and clinical characteristics of emm25/ST660 GAS. Furthermore, to best of our knowledge, this is the first reported case of descending mediastinitis caused by emm25/ST660 GAS. Therefore, physicians should be aware of case with a cervical abscess and secondary descending mediastinitis caused by GAS infection, even if the patient is immunocompetent.

  6. Clinicopathological analysis of mediastinal large B-cell lymphoma and classical Hodgkin lymphoma of the mediastinum.

    PubMed

    Yamamoto, Wataru; Nakamura, Naoya; Tomita, Naoto; Ishii, Yoshimi; Takasaki, Hirotaka; Hashimoto, Chizuko; Motomura, Shigeki; Yamazaki, Etsuko; Ohshima, Rika; Numata, Ayumi; Ishigatsubo, Yoshiaki; Sakai, Rika

    2013-05-01

    Primary mediastinal (thymic) large B-cell lymphoma (PMLBCL) and nodular sclerosing classical Hodgkin lymphoma (NSCHL) are the major histological types of lymphoma affecting the mediastinum. We reviewed 27 patients with PMLBCL and 14 patients with NSCHL. A poor performance status, high serum lactate dehydrogenase level and strong positivity for PAX5 were all significantly more common in patients with PMLBCL than in those with NSCHL. Severe fibrosis was frequent in NSCHL, but not in PMLBCL. PDL1 was expressed by 11/25 PMLBCLs (44.0%) vs. 1/9 NSCHLs (11.1%). Expression of BCL6 was significantly more frequent in PDL1-positive PMLBCL than in PDL1-negative PMLBCL, but there were no clinical differences between these two groups. Two patients with PMLBCL with a poor prognosis had CD20(-), CD79a(+), CD15(-), and CD30(-), possibly representing a subtype of mediastinal gray zone lymphoma.

  7. 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.

  8. Clinical evolution of mediastinitis in patients undergoing adjuvant hyperbaric oxygen therapy after coronary artery bypass surgery

    PubMed Central

    do Egito, Julyana Galvão Tabosa; Abboud, Cely Saad; de Oliveira, Aline Pâmela Vieira; Máximo, Carlos Alberto Gonçalves; Montenegro, Carolina Moreira; Amato, Vivian Lerner; Bammann, Roberto; Farsky, Pedro Silvio

    2013-01-01

    ABSTRACT Objective: To evaluate the use of hyperbaric oxygen therapy as an adjunctive treatment in mediastinitis after coronary artery bypass surgery. Methods: This is a retrospective descriptive study, performed between October 2010 and February 2012. Hyperbaric oxygen therapy was indicated in difficult clinical management cases despite antibiotic therapy. Results: We identified 18 patients with mediastinitis during the study period. Thirty three microorganisms were isolated, and polymicrobial infection was present in 11 cases. Enterobacteriaceae were the most prevalent pathogens and six were multi-resistant agents. There was only 1 hospital death, 7 months after the oxygen therapy caused by sepsis, unrelated to hyperbaric oxygen therapy. This treatment was well-tolerated. Conclusion: The initial data showed favorable clinical outcomes. PMID:24136762

  9. Cervical necrotizing fasciitis and acute mediastinitis of odontogenic origin: A case series

    PubMed Central

    Monsalve-Iglesias, Fernando; Cabello-Serrano, Almudena; Valencia-Laseca, Alfredo; Garcia-Medina, Blas

    2017-01-01

    Necrotising fasciitis (NF) is an uncommon infection. Early signs and symptoms include fever, severe pain and swelling, and redness at the wound site. Moreover, fulminant evolution and high mortality rate are typical of this pathology. In the present report we describes three cases of cervical necrotizing fasciitis complicated by acute mediastinitis. All patients were apparently immunocompetent adults. The main aim of the present report is to show the serious consequences that a dental infection might trigger. Furthermore, we highlight the importance of a multidisciplinary approach in these cases. The constant interaction between different medical specialties is essential for ensuring a proper management of each case. Key words:Cervical necrotizing fasciitis, acute mediastinitis, odontogenic origin , multidisciplinary approach. PMID:28149480

  10. Best approach for posterior mediastinal goiter removal: transcervical incision and lateral thoracotomy.

    PubMed

    Ojanguren Arranz, Amaya; Baena Fustegueras, Juan Antonio; Ros López, Susana; Santamaría Gómez, Maite; Ojanguren Arranz, Iñigo; Olsina Kissle, Jorge Juan

    2014-06-01

    Surgical removal of intrathoracic goiter can be performed by a cervical approach in the majority of patients. Review of literature shows that experienced surgeons need to perform an extracervical approach in 2-3% of cases. In spite of surgical management of substernal goiter is well defined, there is little available information about surgical approach of intrathoracic goiters extending beyond the aortic arch into the posterior mediastinum. We report two cases and propose combination of cervical incision and muscle-sparing lateral thoracotomy for posterior mediastinal goiter removal. In such cases, we do not favour sternotomy as posterior mediastinum is inaccessible due to the presence of heart and great vessels anterior to the thyroidal mass that would lead to perform a perilous blind dissection. Based in our experience, transcervical and thoracotomy approach is indicated for a complete and safe posterior mediastinal goiter removal. Copyright © 2013 SEPAR. Published by Elsevier Espana. All rights reserved.

  11. Mediastinal mass and brachial plexopathy caused by subclavian arterial aneurysm in Behçet's disease.

    PubMed

    Yoo, W H; Kim, H K; Park, J H; Park, T S; Baek, H S

    2000-01-01

    Vascular involvement in Behçet's disease is divided into venous and arterial thrombosis and arterial aneurysmal formation. Subclavian arterial aneurysm rarely occurs in Behçet's disease; however, when it does occur, it causes serious aneurysmal rupture and local complications such as nerve compression and arterial ischemia. We describe the case of a 39-year-old male who presented with neurologic symptoms and signs of brachial plexopathy and mediastinal mass caused by Behçet's subclavian arterial aneurysm. This case shows that the occurrence of brachial plexopathy should be considered a manifestation of Behçet's disease, and that Behçet's aneurysm should be considered in the differential diagnosis of upper mediastinal mass.

  12. Late cardiac effect of radiation therapy on a young woman with mediastinal Hodgkin's lymphoma.

    PubMed

    Kodama, Ken; Takami, Hiroshi; Izumi, Masahiro; Hiramoto, Yoshimune; Yoshida, Kyotaro; Nishioka, Kiyonori; Higashiyama, Masahiko

    2016-01-01

    We experienced an excision of the mediastinal tumor located in left anterior mediastinum found in a 21-year-old woman. The histologic diagnosis of Hodgkin's lymphoma was obtained. After operation, she was treated with mantle field radiotherapy (total 42 Gy) followed by chemotherapy. At 43-year old, she developed constrictive pericarditis and heart failure resulted in the late cardiac effects of mediastinal irradiation. Despite pericardiectomy followed by medical treatment, her cardiac symptoms gradually worsened and she died at 51-year old. At autopsy, her heart was smaller than normal person. The residual pericardium and the surface of the myocardium had fibrous degeneration with tight adhesion and thickness that were dominant at the right atrium and ventricle. Likewise, we demonstrated that pericardiectomy scarcely led to a favorable outcome in this patient. These facts might be based on the radiation-associated cardiac effects involving any components of the cardiac structure.

  13. An anterior mediastinal mass: delayed airway compression and using a double lumen tube for airway patency.

    PubMed

    Lee, Jeounghyuk; Rim, Yong Chul; In, Junyong

    2014-06-01

    Perioperative management of patients with an anterior mediastinal mass is difficult. We present a 35-year-old woman who showed delayed compression of the carina and left main bronchus despite no preoperative respiratory signs, symptoms, or radiologic findings due to an anterior mediastinal mass and uneventful stepwise induction of general anesthesia. Even use of a fiberoptic bronchoscope (FB) after induction of anesthesia was not helpful to predict delayed compression of the airway. Therefore, the anesthesiologist and the cardiothoracic surgeon must prepare for unexpected delayed compression of the airway, even in low risk patients who are asymptomatic or mildly symptomatic without postural symptoms or radiographic evidence of significant compression of structures. We also describe successful management for the compressed carina and left main bronchus with a double lumen tube (DLT) as a stent during surgery. FB guided DLT intubation is a possible solution to maintain airway patency.

  14. 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

  15. Primary mediastinal synovial sarcoma: a case report and review of the literature

    PubMed Central

    Freund, Martin; Zelger, Bettina; Putzer, Daniel; Bonatti, Hugo; Müller, Ludwig; Fiegl, Michael; Geltner, Christian

    2009-01-01

    Primary mediastinal synovial sarcoma is a rare malignancy with only a few cases reported so far. A 56-year-old woman was admitted to our hospital for an investigation of a nodule in the left middle lung on chest radiography. Computed tomography revealed a mediastinal mass first described as a solitary fibrous tumor. The diagnosis of synovial sarcoma was established by computed tomography-guided percutaneous needle biopsy. Work up showed no metastasis to distant organs or contralateral pleural cavity. The mass was surgically resected; pathological and immunohistochemical analyses confirmed the diagnosis of a monophasic spindle cell synovial sarcoma probably originating from phrenic nerve. The patient received adjuvant chemotherapy and radiation and is free of recurrence after a follow up of 16 months. PMID:19918499

  16. Does the Method of Sternal Repair Influence Long-Term Outcome in Management of Treated Mediastinitis?

    DTIC Science & Technology

    2011-03-15

    of cardiac surgery, reducing early and long-term survival. Current management ofPOM is based on sternal debridement, eradication of infection , and...POM were reviewed (1994-2008). Superficial infections and sternal nonunions were excluded. Management principles included sternal debridement...OF (n = 65), and secondary sternal closure (n = 61). Control of mediastinal infection was augmented with negative pressure therapy (NPT) in 73

  17. Mediastinal lymph node detection and station mapping on chest CT using spatial priors and random forest

    PubMed Central

    Liu, Jiamin; Hoffman, Joanne; Zhao, Jocelyn; Yao, Jianhua; Lu, Le; Kim, Lauren; Turkbey, Evrim B.; Summers, Ronald M.

    2016-01-01

    Purpose: To develop an automated system for mediastinal lymph node detection and station mapping for chest CT. Methods: The contextual organs, trachea, lungs, and spine are first automatically identified to locate the region of interest (ROI) (mediastinum). The authors employ shape features derived from Hessian analysis, local object scale, and circular transformation that are computed per voxel in the ROI. Eight more anatomical structures are simultaneously segmented by multiatlas label fusion. Spatial priors are defined as the relative multidimensional distance vectors corresponding to each structure. Intensity, shape, and spatial prior features are integrated and parsed by a random forest classifier for lymph node detection. The detected candidates are then segmented by the following curve evolution process. Texture features are computed on the segmented lymph nodes and a support vector machine committee is used for final classification. For lymph node station labeling, based on the segmentation results of the above anatomical structures, the textual definitions of mediastinal lymph node map according to the International Association for the Study of Lung Cancer are converted into patient-specific color-coded CT image, where the lymph node station can be automatically assigned for each detected node. Results: The chest CT volumes from 70 patients with 316 enlarged mediastinal lymph nodes are used for validation. For lymph node detection, their system achieves 88% sensitivity at eight false positives per patient. For lymph node station labeling, 84.5% of lymph nodes are correctly assigned to their stations. Conclusions: Multiple-channel shape, intensity, and spatial prior features aggregated by a random forest classifier improve mediastinal lymph node detection on chest CT. Using the location information of segmented anatomic structures from the multiatlas formulation enables accurate identification of lymph node stations. PMID:27370151

  18. Clinical Significance of Serum Biomarkers in Pediatric Solid Mediastinal and Abdominal Tumors

    PubMed Central

    Sandoval, John A.; Malkas, Linda H.; Hickey, Robert J.

    2012-01-01

    Childhood cancer is the leading cause of death by disease among U.S. children between infancy and age 15. Despite successes in treating solid tumors such as Wilms tumor, disappointments in the outcomes of high-risk solid tumors like neuroblastoma have precipitated efforts towards the early and accurate detection of these malignancies. This review summarizes available solid tumor serum biomarkers with a special focus on mediastinal and abdominal cancers in children. PMID:22312308

  19. Intra- and Inter-Fraction Mediastinal Nodal Region Motion: Implications for Internal Target Volume Expansions

    PubMed Central

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

    2009-01-01

    Purpose/Objective The purpose of this study is to determine the intra- and inter-fraction motion of mediastinal lymph node regions. Materials/Methods Ten patients with non-small cell lung cancer underwent controlled inhale and exhale CT scans during two sessions (40 total data sets) and mediastinal nodal stations 1–8 [Chapet, et al, IJROBP 2005;63:170–8] were outlined. Corresponding CT scans from different sessions were registered to remove setup error and in this reference frame, the center-of-mass (COM) 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. Intra-fraction displacement was determined by comparing same session inhale-exhale scans. Inter-fraction reproducibility of nodal regions was determined by comparing the same respiratory phase scans between two sessions. Results Intra-fraction displacement of COM 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 inter-patient variations in intra-fraction displacement were large compared to the displacements themselves. Moreover, there was substantial inter-fractional displacement (∼5 mm). Conclusions Mediastinal lymph node regions clearly move during breathing. Additionally, 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. PMID:19410142

  20. Posterior mediastinal paragangliomas: a report of three patients with peculiar tumours.

    PubMed

    Ayadi-Kaddour, Aïda; Braham, Emna; Ismail, Olfa; Smati, Bellahssan; Djilani, Habiba; El Mezni, Faouzi

    2009-04-01

    Paragangliomas of the mediastinum are rare neoplasms, representing less than 0.3% of mediastinal tumours and less than 2% of all paragangliomas. To date, no definitive morphologic criteria exist that correlate with the clinical outcome of these tumours. This report describes three patients with peculiar primary paragangliomas of the posterior mediastinum: gangliocytic, dumbbell and functional paraganglioma. The clinicopathological characteristics of these patients are presented and the literature reviewed.

  1. Colorectal Cancer Metastasis to the Thymus Gland: Rare Presentation of Colorectal Cancer as Anterior Mediastinal Mass.

    PubMed

    Peters, H Charles; Liu, Xiuli; Iqbal, Atif; Cunningham, Lisa A; Tan, Sanda A

    2017-01-01

    Despite improved screening modalities, 15-25% of newly diagnosed colorectal cancers are metastatic at the time of diagnosis. The vast majority of these cases present as hepatic metastasis; however, 22% present with concomitant extrahepatic disease. The thymus gland is an uncommon site of metastasis for any primary malignancy, particularly, colorectal cancer given its vascular and lymphatic drainage. This case report details our experience with a rare case of colorectal cancer metastasis to the thymus gland presenting as a symptomatic mediastinal mass.

  2. The role of endobronchial ultrasonography for mediastinal lymphadenopathy in cases with extrathoracic malignancy.

    PubMed

    Tertemiz, Kemal Can; Alpaydin, Aylin Ozgen; Karacam, Volkan

    2017-07-01

    Many extrathoracic malignancies can metastasize to lungs and mediastinal lymph nodes. Whether mediastinal lesions are metastasis in these patients changes staging, prognosis, and treatment strategy. In this study, we aimed to find out the contribution of EBUS-TBNA to the diagnosis in cases with extrathoracic malignancy. Patients who had been previously diagnosed as extrapulmonary solid organ malignancy and in whom mediastinal or hilar lymphadenopathy developed during their follow-up and EBUS-TBNA was applied for diagnostic purposes were retrospectively included in this study. A total of 91 patients consisting of 35 females (38.5 %) and 56 males (61.5 %) were included in the study. The mean age of the patients was 60.5 (±11.4). Malignancy was not observed in 54 (59.3 %) patients; primary malignancy metastasis was detected in 33 (36.3 %) patients, and primary lung cancer was detected in 4 (4.4 %) patients with EBUS-TBNA. The sensitivity of EBUS-TBNA in extrathoracic malignancies was determined as 90.2 %; its specificity was determined as 100 %, its negative predictive value as 92.5 %, its positive predictive value as 100 %, and its diagnostic accuracy as 95.6 %. The highest rate was determined in the left lower paratracheal lymph node when they were examined in terms of malignancy detection rate in lymph node stations. EBUS-TBNA is a minimally invasive method with quite a low complication rate that does not require general anesthesia. It should be the first step method to be used in the diagnosis of mediastinal and hilar lymphadenopathies seen in extrathoracic malignancies since it has high diagnostic accuracy, sensitivity, and specificity. EBUS-TBNA significantly reduces the need for surgical intervention. Further surgical interventions can be planned in patients in whom diagnostic competence is not ensured.

  3. A new case of Mycoplasma hominis mediastinitis and sternal osteitis after cardiac surgery.

    PubMed

    Le Guern, Rémi; Loïez, Caroline; Loobuyck, Valentin; Rousse, Natacha; Courcol, René; Wallet, Frédéric

    2015-02-01

    We report a case of nosocomial mediastinitis and sternal osteitis due to M. hominis after open-heart surgery in an immuno-competent patient. This infection has been diagnosed by incubating the culture media for an extended period of time, and sequencing 16S rDNA directly from the clinical samples. Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved.

  4. Distinguishing benign mediastinal masses from malignancy in a histoplasmosis-endemic region

    PubMed Central

    Naeem, Fouzia; Metzger, Monika L.; Arnold, Sandra R.; Adderson, Elisabeth E.

    2015-01-01

    Objective To describe the characteristics of benign and malignant mediastinal masses, which may predict their etiology and facilitate the safe and timely management of patients, especially those residing in histoplasmosis-endemic regions. Study design We conducted a retrospective review of the health records of 131 patients aged <19 years who were referred to two tertiary care children's hospitals from 2005-2010 for the evaluation of mediastinal masses. Results Most patients (79%) had benign masses, including 98 with confirmed or suspected histoplasmosis. Overall, patients with benign etiologies were younger, more likely to be African American, more likely to complain of cough and to have pulmonary nodules by chest computed tomographs than patients with cancer. Patients with malignant disease were more likely to complain of malaise and to have neck swelling, abnormal extrathoracic lymphadenopathy, lymphopenia, anterior mediastinal involvement and/or pleural effusion. Positive histoplasmosis serologic tests were specific but insensitive for a benign etiology. No single clinical, laboratory or radiologic feature was sufficiently sensitive and specific to distinguish between benign and malignant masses. For cancer, however, the presence of lymphopenia, anterior mediastinal involvement or enlarged cervical lymph nodes on computerized tomography had a sensitivity of 93%, specificity of 95%, positive predictive value of 86%, and negative predictive value of 97% for cancer. Sixty-four patients (49%) underwent invasive testing, including 37 (36%) of patients with benign masses. Conclusions Patients in this series who had involvement of the anterior mediastinum, lymphopenia or enlarged cervical lymph nodes had a high likelihood of cancer. Expectant management of patients lacking these characteristics may be safe and reduce unnecessary invasive testing. PMID:26009018

  5. Sternitis and mediastinitis after coronary artery bypass grafting. Analysis of risk factors.

    PubMed Central

    Wouters, R; Wellens, F; Vanermen, H; De Geest, R; Degrieck, I; De Meerleer, F

    1994-01-01

    As part of a quality control program, we analyzed possible risk factors in the development of sternitis and mediastinitis after coronary artery bypass grafting. From 1 January 1990 through 31 December 1991, 1,368 consecutive coronary artery bypass grafting procedures were performed at our institution, either alone or in combination with other procedures. Twenty-three patients (1.7%) developed sternitis and/or mediastinitis; 7 (30.4%) of these patients died in an early postoperative phase. Univariate analysis revealed the following statistically significant (p < or = 0.05) risk factors: perfusion time, length of stay in operating room of longer than 5 hours 30 minutes, presence at the operation of a certain surgical resident, revision for bleeding, and postoperative mechanical ventilation lasting longer than 72 hours. After multivariate analysis, statistically significant independent risk factors were: diabetes mellitus, recent cigarette-smoking, reoperation, presence of a certain surgical resident at the operation, revision for bleeding, and length of mechanical ventilation of longer than 72 hours. The use of both internal thoracic arteries was not, in this study, shown to be an independent risk factor. We conclude that although the technique of using both internal thoracic arteries for myocardial revascularization carries no extra risk by itself in the development of sternitis or mediastinitis, associated factors such as prolonged stay in the operating room and reoperation could be responsible for a higher frequency of sternitis-mediastinitis in patients who have undergone this procedure. Therefore, it is advisable to use this technique selectively in high-risk patients. Close surveillance and reporting of wound infections is mandatory to detect risk factor related to the surgical staff (such as Staphylococcus aureus dissemination). PMID:8000263

  6. Clinical significance of serum biomarkers in pediatric solid mediastinal and abdominal tumors.

    PubMed

    Sandoval, John A; Malkas, Linda H; Hickey, Robert J

    2012-01-01

    Childhood cancer is the leading cause of death by disease among U.S. children between infancy and age 15. Despite successes in treating solid tumors such as Wilms tumor, disappointments in the outcomes of high-risk solid tumors like neuroblastoma have precipitated efforts towards the early and accurate detection of these malignancies. This review summarizes available solid tumor serum biomarkers with a special focus on mediastinal and abdominal cancers in children.

  7. [Esophageal perforation and mediastinitis caused by fishbone ingestion: report of 3 cases].

    PubMed

    Vásquez, Julio C; Montesinos, Efraín; Castillo, Luis; Rojas, Luis; Peralta, Julio

    2006-01-01

    We present 3 consecutive cases of fishbone perforation of the esophagus that resulted in acute mediastinitis. All patients were successfully managed with drainage and debridement of the mediastinum and pleural cavity. However, temporary exclusion with external ligation of the esophagus was also performed in one patient, with spontaneous recanalization two weeks later. We discuss the possible role of this surgical maneuver in the management of esophageal perforation.

  8. 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.

  9. Cervical necrotising fasciitis and descending mediastinitis secondary to unilateral tonsillitis: a case report

    PubMed Central

    Islam, Asad; Oko, Michael

    2008-01-01

    Introduction Cervical necrotizing fasciitis is an aggressive infection with high morbidity and mortality. We present a case of cervical necrotizing fasciitis and descending mediastinitis in a healthy young man, caused by unilateral tonsillitis with a successful outcome without aggressive debridement. Case presentation A 41-year-old man was admitted to our unit with a diagnosis of severe acute unilateral tonsillitis. On admission, he had painful neck movements and the skin over his neck was red, hot and tender. Computed tomography scan of his neck and chest showed evidence of cervical necrotizing fasciitis and descending mediastinitis secondary to underlying pharyngeal disease. He was treated with broad-spectrum intravenous antibiotics. His condition improved over the next 3 days but a tender and fluctuant swelling appeared in the suprasternal region. A repeat scan showed the appearance of an abscess extending from the pretracheal region to the upper mediastinum which was drained through a small transverse anterior neck incision. After surgery, the patient's condition quickly improved and he was discharged on the 18th day of admission. Conclusion Less invasive surgical techniques may replace conventional aggressive debridement as the treatment of choice for cervical necrotizing fasciitis and descending necrotizing mediastinitis. PMID:19055812

  10. [Post-operative mediastinitis in a heart hospital of Recife: contributions for nursing care].

    PubMed

    Magalhães, Marina Gabriella Pereira de Andrada; Alves, Ludmila Medeiros Outtes; Alcantara, Lidianne Fábia de Moraes; Bezerra, Simone Maria Muniz da Silva

    2012-08-01

    The objective of this study was to determine the prevalence of post-operative mediastinitis with the purpose to contribute to nursing care knowledge. To do this, an analysis was performed on 896 medical records of patients who underwent heart surgery involving sternotomy at the Cardiology Emergency Room of Recife-PE, in the period between June 2007 and June 2009. The following variables were considered: gender, age, type of surgery, personal history, length of stay, use of antibiotics, and culture of the surgical wound. A high death rate from mediastinitis was observed (33.3%). Several risk factors were identified, including: systemic arterial hypertension (80.9%); smoking (61.9%); diabetes mellitus (42.8%); and obesity (33.3%), most of which (76.2%) were identified in patients who underwent surgery for myocardial revascularization. It is concluded that mediastinitis is a serious infection that needs continuous nursing supervision and preventive measures to assure an early diagnosis and, thus, reduce mortality.

  11. 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

  12. Total mechanical stapled oesophagogastric anastomosis on the neck in oesophageal cancer - prevention of postoperative mediastinal complications.

    PubMed

    Zieliński, Jacek; Jaworski, Radosław; Irga-Jaworska, Ninela; Haponiuk, Ireneusz; Jaśkiewicz, Janusz

    2015-12-01

    Oesophagogastric anastomosis after oesophagus resection is commonly performed on the neck. Even though a few different techniques of oesophagogastric anastomosis have been previously detailed, both manual and mechanical procedures have been burdened with leakages and strictures. Our simple technique of oesophagogastric anastomosis is a modification of mechanical anastomosis with the use of a circular stapler in order to prevent postoperative leak and concomitant mediastinal complications. Since 2008, we have performed nine oesophagogastric anastomoses following oesophagus resection. The mean age of the operated patients was 54 years. There was no mortality among the operated patients in the early post-operative period. The mean follow-up period for the patients operated on in our department was 17 months until the time of the analysis. None of the patients showed any leakage or stricture, and no mediastinal complications were reported in the group. Following our own experience, mechanical anastomosis with the use of a circular stapler seems to decrease the time of the operation as well as significantly reducing the incidence of leakages from the anastomosis. This type of anastomosis may decrease the number of postoperative strictures and the most dangerous mediastinal infections.

  13. 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.

  14. 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.

  15. A canine case with cystic meningioma showing miraculous reduction of the cystic lesion

    PubMed Central

    WADA, Masae; HASEGAWA, Daisuke; HAMAMOTO, Yuji; ASAI, Atsushi; SHOUJI, Akane; CHAMBERS, James; UCHIDA, Kazuyuki; FUJITA, Michio

    2015-01-01

    A 12-year-old spayed female Labrador retriever was presented with forebrain signs. Brain MRI revealed a huge cystic lesion with the thickened falx in the frontal region. The brain parenchyma surrounding the lesion showed significant signs of a mass effect and also increased intracranial pressure. However, the dog suddenly became lucid after about two weeks, and an MRI scan one month after the initial study revealed a dramatically shrunken cystic lesion. The dog survived for over a year until it was euthanized for other reasons, and the brain lesion was diagnosed as a cystic meningioma histologically. To the authors’ knowledge, this is the first report that described the reduction of the cystic lesion of a cystic meningioma in dogs. PMID:26256491

  16. Emerging treatments in cystic fibrosis.

    PubMed

    Jones, Andrew M; Helm, Jennifer M

    2009-10-01

    There are a number of potential drugs for the treatment of cystic fibrosis (CF) currently undergoing clinical studies. A number of antibacterials formulated for delivery by inhalation are at various stages of study; these include dry-powder inhaler versions of colistin, tobramycin and ciprofloxacin, and formulations of azteonam, amikacin, levofloxacin, ciprofloxacin and fosfomycin/tobramycin for nebulization. Clinical trials of anti-inflammatory agents, including glutathione, phosphodiesterase-5 inhibitors such as sildenafil, oral acetylcysteine, simvastatin, methotrexate, docosahexaenoic acid, hydroxychloroquine, pioglitazone and alpha1-antitrypsin, are ongoing. Ion channel modulating agents, such as lancovutide (Moli1901, duramycin) and denufosol, which activate alternate (non-CF transmembrane regulator [CFTR]) chloride channels, and GS 9411, a sodium channel antagonist, are now at the stages of clinical study and if successful, will offer a new category of therapeutic agent for the treatment of CF. Correction of the underlying gene effect, either by agents that help to correct the dysfunctional CFTR, such as ataluren, VX-770 and VX-809, or by gene transfer (gene therapy), is a particularly exciting prospect as a new therapy for CF and clinical studies are ongoing. This article reviews the exciting potential drug treatments for CF currently being evaluated in clinical studies, and also highlights some of the challenges faced by research and clinical teams in assessing the efficacy of potential new therapies for CF.

  17. Cystic fibrosis: a clinical view.

    PubMed

    Castellani, Carlo; Assael, Baroukh M

    2017-01-01

    Cystic fibrosis (CF), a monogenic disease caused by mutations in the CFTR gene on chromosome 7, is complex and greatly variable in clinical expression. Airways, pancreas, male genital system, intestine, liver, bone, and kidney are involved. The lack of CFTR or its impaired function causes fat malabsorption and chronic pulmonary infections leading to bronchiectasis and progressive lung damage. Previously considered lethal in infancy and childhood, CF has now attained median survivals of 50 years of age, mainly thanks to the early diagnosis through neonatal screening, recognition of mild forms, and an aggressive therapeutic attitude. Classical treatment includes pancreatic enzyme replacement, respiratory physiotherapy, mucolitics, and aggressive antibiotic therapy. A significant proportion of patients with severe symptoms still requires lung or, less frequently, liver transplantation. The great number of mutations and their diverse effects on the CFTR protein account only partially for CF clinical variability, and modifier genes have a role in modulating the clinical expression of the disease. Despite the increasing understanding of CFTR functioning, several aspects of CF need still to be clarified, e.g., the worse outcome in females, the risk of malignancies, the pathophysiology, and best treatment of comorbidities, such as CF-related diabetes or CF-related bone disorder. Research is focusing on new drugs restoring CFTR function, some already available and with good clinical impact, others showing promising preliminary results that need to be confirmed in phase III clinical trials.

  18. [Azithromycin therapy in cystic fibrosis].

    PubMed

    Máiz Carro, Luis; Cantón Moreno, Rafael

    2004-03-06

    Progressive lung disease, caused by chronic endobronchial colonization, is the major cause of morbidity and mortality in patients with cystic fibrosis (CF). Several pathogens, including Staphylococcus aureus and Pseudomonas aeruginosa are responsible for this effect. The steadily improving prognosis of CF has been attributed to the use of antibiotics with activity against these organisms. Despite a significant increase in the amount of published material demonstrating the potential role of macrolide antibiotics as antiinflammatory agents and their effects on bacterial virulence, their mechanism of action in CF patients is still unknown. Although there is a limited number of clinical trials assessing the efficacy and safety of azithromycin (AZM) in CF, increasing evidence suggests that 3 to 6-month AZM treatment in CF patients is safe and well tolerated. This treatment results in clinical improvement, decreasing the number of pulmonary exacerbations and increasing pulmonary function. Therefore, chronic treatment with AZM should be considered in CF patients added to conventional therapy. Clinical experience with macrolides other than AZM in CF patients is very limited.

  19. 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)

  20. 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.

  1. 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)

  2. Cystic precursors to invasive pancreatic cancer

    PubMed Central

    Matthaei, Hanno; Schulick, Richard D.; Hruban, Ralph H.; Maitra, Anirban

    2011-01-01

    Improvements in the sensitivity and quality of cross-sectional imaging have led to increasing numbers of patients being diagnosed with cystic lesions of the pancreas. In parallel, clinical, radiological, pathological and molecular studies have improved the systems for classifying these cysts. Patients with asymptomatic serous cystic neoplasms can be managed conservatively with regular monitoring; however, the clinical management of patients with intraductal papillary mucinous neoplasms and mucinous cystic neoplasms is far more challenging, as it is difficult to determine whether these lesions will progress to malignancy. Fortunately, prospective studies have helped to establish that proposed clinical and radiological criteria (the Sendai guidelines) can be used to guide the care of patients with cystic lesions of the pancreas. Despite this progress in imaging and clinical guidelines, sensitive and specific tests have not yet been developed that can reliably predict the histology and biological properties of a cystic lesion. Such biomarkers are urgently needed, as noninvasive precursors of pancreatic cancer are curable, while the vast majority of invasive pancreatic adenocarcinomas are not. PMID:21383670

  3. Fetal case of congenital cystic adenomatoid malformation of the lung: fetal therapy and a review of the published reports in Japan.

    PubMed

    Asabe, Koushi; Oka, Yoichiro; Shirakusa, Takayuki

    2005-09-01

    We herein report a case of type I congenital cystic adenomatoid malformation of the lung (CCAML) with non-immune hydrops fetalis (NIHF), a mediastinal shift and polyhydramnios diagnosed at 24 weeks' gestation by ultrasonography. The fetus was treated with a cyst-amniotic shunt at 29 weeks' gestation. Following a postnatal whole resection of the right lung, postpneumonectomy syndrome appeared and, as a result, the infant died 13 months after delivery due to respiratory failure. Only 19 cases demonstrating CCAML associated with NIHF have been reported previously in Japan. Four cases showed a spontaneous resolution of NIHF, while 5 cases with type I CCAML, which all underwent fetal intervention, demonstrated an excellent outcome.

  4. Cystic duct carcinoma mimicking a middle bile duct tumour

    PubMed Central

    Francisco, Elsa; Mendes, Miguel; Vale, Sílvio; Esteves, Joana

    2015-01-01

    Cystic duct carcinoma was defined by Farrar as a tumour restricted to the cystic duct, making it a rare disease. The authors describe a case of a cystic duct carcinoma that fulfils Farrar’s strict diagnostic criteria and that became clinically relevant by compressing the common hepatic duct, thus causing cholestasis. A cholecystectomy was performed with en bloc resection of the cystic and extrahepatic bile duct with a regional lymphadenectomy. PMID:25819819

  5. Challenges in pulmonary fibrosis. 3: Cystic lung disease.

    PubMed

    Cosgrove, Gregory P; Frankel, Stephen K; Brown, Kevin K

    2007-09-01

    Cystic lung disease is a frequently encountered problem caused by a diverse group of diseases. Distinguishing true cystic lung disease from other entities, such as cavitary lung disease and emphysema, is important given the differing prognostic implications. In this paper the features of the cystic lung diseases are reviewed and contrasted with their mimics, and the clinical and radiographic features of both diffuse (pulmonary Langerhans' cell histiocytosis and lymphangioleiomyomatosis) and focal or multifocal cystic lung disease are discussed.

  6. Challenges in pulmonary fibrosis · 3: Cystic lung disease

    PubMed Central

    Cosgrove, Gregory P; Frankel, Stephen K; Brown, Kevin K

    2007-01-01

    Cystic lung disease is a frequently encountered problem caused by a diverse group of diseases. Distinguishing true cystic lung disease from other entities, such as cavitary lung disease and emphysema, is important given the differing prognostic implications. In this paper the features of the cystic lung diseases are reviewed and contrasted with their mimics, and the clinical and radiographic features of both diffuse (pulmonary Langerhans' cell histiocytosis and lymphangioleiomyomatosis) and focal or multifocal cystic lung disease are discussed. PMID:17726170

  7. 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. PMID:27418876

  8. [Cystic fibrosis in a 70-year-old woman].

    PubMed

    Bruun, Lene Søndberg; Jensen, Michael Skov

    2002-05-06

    Cystic fibrosis is usually diagnosed in early childhood, and patients rarely live beyond the age of 40. We present a case of a 70-year-old woman, in whom cystic fibrosis was diagnosed with the rare mutation, R117C. Cystic fibrosis should therefore also be considered in older patients.

  9. 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;…

  10. 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;…

  11. Florid cystic endosalpingiosis of the uterus

    PubMed Central

    Heatley, M; Russell, P

    2001-01-01

    A 73 year old woman presented with a right sided adnexal cystic mass. At laparotomy, this proved to be a benign serous ovarian cyst and an aggregation of thin walled subserosal and soft tissue cysts and spongy nodules up to 16 mm in diameter involving the side wall of the uterus and adjacent parametrium. These were removed by total abdominal hysterectomy and bilateral salpingo-oophorectomy. Histologically, the cystic spaces and smaller acini were lined by benign tubo-endometrioid epithelium, with smaller areas typical of serous differentiation and rare microfoci of endocervical-type mucinous epithelium. These features indicated multidirectional Mullerian differentiation in a process that, overall, was consistent with so called florid cystic endosalpingiosis. This lesion is to be distinguished from other benign conditions including multicystic mesothelioma, endometriosis, endocervicosis, florid deep glands of the uterine cervix, and deep Nabothian cysts of the uterine cervix. Key Words: uterus • endosalpingiosis • cysts PMID:11328842

  12. Self-management education for cystic fibrosis.

    PubMed

    Savage, Eileen; Beirne, Paul V; Ni Chroinin, Muireann; Duff, Alistair; Fitzgerald, Tony; Farrell, Dawn

    2011-07-06

    Self-management education may help patients with cystic fibrosis and their families to choose, monitor and adjust treatment requirements for their illness, and also to manage the effects of illness on their lives. Although self-management education interventions have been developed for cystic fibrosis, no previous systematic review of the evidence of effectiveness of these interventions has been conducted. To assess the effects of self-management education interventions on improving health outcomes for patients with cystic fibrosis and their caregivers We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register (date of the last search: 23 February 2011).We also searched databases through EBSCO (CINAHL; Psychological and Behavioural Sciences Collection; PsychInfo; SocINDEX) and Elsevier (EMBASE) and handsearched relevant journals and conference proceedings (date of the last searches: 30th March 2011). Randomised controlled trials, quasi-randomised controlled trials or controlled clinical trials comparing different types of self-management education for cystic fibrosis or comparing self-management education with standard care or no intervention. Two authors assessed trial eligibility and risk of bias. Three authors extracted data. Four trials (involving a total of 269 participants) were included. The participants were children with cystic fibrosis and their parents or caregivers in three trials and adults with cystic fibrosis in one trial. The trials compared four different self-management education interventions versus standard treatment: (1) a training programme for managing cystic fibrosis in general; (2) education specific to aerosol and airway clearance treatments; (3) disease-specific nutrition education; and (4) general and disease-specific nutrition education. Training children to manage cystic fibrosis in general had no statistically significant effects on weight after six to eight weeks, mean difference -7.74 lb (95% confidence

  13. Self-management education for cystic fibrosis.

    PubMed

    Savage, Eileen; Beirne, Paul V; Ni Chroinin, Muireann; Duff, Alistair; Fitzgerald, Tony; Farrell, Dawn

    2014-09-08

    Self-management education may help patients with cystic fibrosis and their families to choose, monitor and adjust treatment requirements for their illness, and also to manage the effects of illness on their lives. Although self-management education interventions have been developed for cystic fibrosis, no previous systematic review of the evidence of effectiveness of these interventions has been conducted. To assess the effects of self-management education interventions on improving health outcomes for patients with cystic fibrosis and their caregivers We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register (date of the last search: 22 August 2013).We also searched databases through EBSCO (CINAHL; Psychological and Behavioural Sciences Collection; PsychInfo; SocINDEX) and Elsevier (Embase) and handsearched relevant journals and conference proceedings (date of the last searches: 01 February 2014 ). Randomised controlled trials, quasi-randomised controlled trials or controlled clinical trials comparing different types of self-management education for cystic fibrosis or comparing self-management education with standard care or no intervention. Two authors assessed trial eligibility and risk of bias. Three authors extracted data. Four trials (involving a total of 269 participants) were included. The participants were children with cystic fibrosis and their parents or caregivers in three trials and adults with cystic fibrosis in one trial. The trials compared four different self-management education interventions versus standard treatment: (1) a training programme for managing cystic fibrosis in general; (2) education specific to aerosol and airway clearance treatments; (3) disease-specific nutrition education; and (4) general and disease-specific nutrition education. Training children to manage cystic fibrosis in general had no statistically significant effects on weight after six to eight weeks, mean difference -7.74 lb (i.e. 3.51 kg) (95

  14. [Cystic lesions--diagnosis and conservative treatment].

    PubMed

    Olas, Jacek; Bucka, Jolanta; Dworak, Agata; Golański, Bartłomiej

    2003-01-01

    Effective treatment of cystic lesions that we can apply in the outpatient clinic is the target for seeking a new solution in finding treatment that will produce a better percentage of recovered patients. At this moment non-surgical treatment of cystic lesions is compare with traditional methods of treatment i.e. cyst content aspiration and injection of steroids, with methods which lead to obliteration of cyst lumen and in this way closing the space being the reservoir for the cyst content. Local application of hialuronidase, aspiration, and then steroid injection is gaining more and more attention of clinicians. We also pay much more attention to local fibrinogen injection as a safe and effective method of treatment, which can be useful in the treatment of cystic lesions and topical treatment of bursitis.

  15. Methicillin-resistant Staphylococcus aureus infection: an independent risk factor for mortality in patients with poststernotomy mediastinitis.

    PubMed

    Simşek Yavuz, Serap; Sensoy, Ayfer; Ceken, Sabahat; Deniz, Denef; Yekeler, Ibrahim

    2014-01-01

    The mortality rate of patients with poststernotomy mediastinitis remains very high. The aim of this study was to identify the risk factors associated with mortality in these patients. Surveillance of sternal surgical-site infections including mediastinitis was carried out for adult patients undergoing a sternotomy between 2004 and 2012. Criteria from the US Centers for Disease Control and Prevention were used to make the diagnosis. All data on patients with a diagnosis of mediastinitis who were included in the study and on mortality risk factors were obtained from the hospital database and then analyzed using SPPS 16.0 for Windows. Of the 19,767 patients undergoing open heart surgery, 117 (0.39%) had poststernotomy mediastinitis; 32% of these 117 died. The independent risk factors for mortality were methicillin-resistant Staphylococcus aureus (MRSA) [odds ratio (OR) 12.11 and 95% confidence interval (CI) 3.15-46.47], intensive-care unit stays >48 h after the first operation (OR 11.21 and 95% CI 3.24-38.84) and surgery that included valve replacement (OR 6.2 and 95% CI 1.44-27.13). The mortality rate decreased significantly, dropping from 38% (34/89) between 2004 and 2008 to 14% (4/28) between 2009 and 2012 (p = 0.018). In this study, elimination of MRSA from the hospital setting decreased the rate of mortality in patients with poststernotomy mediastinitis. © 2014 S. Karger AG, Basel.

  16. Combined videothoracoscopic and videomediastinoscopic approach improves radicality of minimally invasive mediastinal lymphadenectomy for early stage lung carcinoma.

    PubMed

    Witte, Biruta; Messerschmidt, Antje; Hillebrand, Hubertus; Gross, Stefan; Wolf, Michael; Kriegel, Elke; Neumeister, Wolfgang; Hürtgen, Martin

    2009-02-01

    To assess the feasibility and radicality of a combined thoracoscopic and mediastinoscopic approach to mediastinal lymphadenectomy compared to thoracoscopy only for minimally invasive management of early stage lung carcinoma. Prospective observational study of patients undergoing anatomical thoracoscopic lung resection for lung carcinoma in our department in 2007. Mediastinal lymphadenectomy was performed either thoracoscopically (VATS group) or by a combination of video-assisted mediastinoscopic lymphadenectomy (VAMLA) and thoracoscopy (VAMLA+VATS group). Inclusion criteria for the study were: stage Ia on CT scan, no central tumor at bronchoscopy, and no contraindications against lobectomy or segmentectomy. Eighteen VAMLA+VATS and fourteen VATS patients were studied. For histology, pTNM stage, type of resection, semiquantitative assessment of the fissure and vascular dissection plane, conversions, blood loss, operation time, adverse events and drainage time, no differences between the two groups were observed. In the VATS group, there was a slight preponderance of women, and right-sided tumors. In the VAMLA+VATS group, both the number of dissected mediastinal lymph node stations (mean, 6.4 stations vs 3.6 stations) and the weight of the mediastinal specimen (median, 11.2 groups vs 5.5 groups), were significantly higher than in the VATS group (p<0.05). A combined approach by VATS and VAMLA improves radicality of minimally invasive mediastinal lymphadenectomy without increase in operation time, morbidity, and drainage time.

  17. Does the presence of mediastinal adenopathy confer a risk for disseminated infection in immunocompetent persons with pulmonary coccidioidomycosis?

    PubMed

    Mayer, Anita P; Morris, Michael F; Panse, Prasad M; Ko, Marcia G; Files, Julia A; Ruddy, Barbara E; Blair, Janis E

    2013-03-01

    Pulmonary coccidioidomycosis is caused by inhaling airborne arthroconidia of Coccidioides, a soil-dwelling fungus endemic to the desert southwestern United States. Although uncommon, disseminated coccidioidal infection can be associated with well-defined risk factors, such as cell-mediated immunodeficiency, certain racial heritages (e.g. African or Filipino), male sex, or pregnancy. Before widespread use of computed tomography (CT), the presence or persistence of mediastinal lymphadenopathy was postulated to be a risk factor for disseminated coccidioidal infection. To investigate the use of CT scanning to identify the presence of mediastinal lymphadenopathy in patients with pulmonary coccidioidomycosis, and to correlate such lymphadenopathy with disseminated coccidioidal infection, we performed a retrospective review of patients with pulmonary coccidioidomycosis who were evaluated by chest CT. Two radiologists independently interpreted 150 CT scans from patients with pulmonary coccidioidomycosis. Forty-nine patients met CT criteria for mediastinal lymphadenopathy, whereas 101 patients did not. Disseminated coccidioidal infection was observed in 5 (10%) of the 49 patients with mediastinal lymphadenopathy and in 6 of the 101 (6%; P = .34) without such adenopathy. Among patients with coccidioidomycosis, patients with mediastinal lymphadenopathy, as assessed by CT, had a higher rate of disseminated infection, but the difference was not statistically significant. © 2012 Blackwell Verlag GmbH.

  18. A rare case of bilateral cystic partially differentiated nephroblastoma recurring as bilateral cystic Wilms tumour.

    PubMed

    Kurian, Jujju Jacob; Ninan, Pradeep Joseph

    2015-04-15

    Childhood cystic partially differentiated nephroblastoma (CPDN) is an uncommon renal neoplasm. Bilateral CPDN or CPDN co-existing with a cystic nephroma/Wilms tumour is extremely rare. Treatment of CPDN is by complete surgical excision. Although local recurrences are uncommon, distant metastases have not been described. We present a case of bilateral CPDN that, after complete excision, recurred as bilateral cystic Wilms tumour. To the best of our knowledge, this is the first reported case in the literature where a bilateral CPDN has recurred as bilateral Wilms tumour.

  19. Cystic angiomatosis, a heterogeneous condition

    PubMed Central

    Najm, Aurélie; Soltner-Neel, Elise; Le Goff, Benoît; Guillot, Pascale; Maugars, Yves; Berthelot, Jean-Marie

    2016-01-01

    Abstract Background: Cystic angiomatosis (CA) is a rare disorder causing bony cysts. It displays some similarity to Gorham–Stout disease (GSD), but has a much better local prognosis, despite the larger number of cysts. These 2 conditions also differ in terms of their location, visceral involvement, and response to treatment. Methods: We report 4 cases of CA, including 1 sclerosing form, which we compare with cases from a literature review performed with PRISMA methodology. Results: We reviewed 38 articles describing 44 other patients. Mean age at diagnosis for the 48 patients (our 4 patients + the 44 from the review) was 22.5 years, and 28 of the patients were men. The femur was involved in 81% (n = 39), the pelvis in 73% (n = 35), the humerus in 52% (n = 25), the skull in 48% (n = 23), and the vertebrae in 44% (n = 21). Visceral lymphangiomatosis (either clinical, or detected on autopsy) was also reported in 35% (n = 18) of the patients. The spleen was the most frequently involved organ (n = 12), followed by the lungs and pleura (n = 8). Liver cysts and/or chylothorax were rarely reported (5 cases), but were invariably fatal. Radiation therapy on bone or soft tissue masses was ineffective, as was interferon alpha, in the 2 patients in which this drug was tested. The efficacy of bisphosphonate was at best equivocal. Conclusion: The progression of CA is unpredictable and treatments effective against GSD, such as bisphosphonates and radiotherapy, have proved ineffective for this condition. New treatments are thus urgently required. PMID:27787381

  20. Venous thromboembolism in cystic fibrosis.

    PubMed

    Takemoto, Clifford M

    2012-02-01

    The incidence of venous thromboembolism (VTE) is increasing in the pediatric population. Individuals with cystic fibrosis (CF) have an increased risk of thrombosis due to central venous catheters (CVCs), as well as acquired thrombophilia secondary to inflammation, or deficiencies of anticoagulant proteins due to vitamin K deficiency and/or liver dysfunction. CVC-associated thrombosis commonly results in line occlusion, but may develop into serious life-threatening conditions such as deep venous thrombosis (DVT), superior vena cava syndrome or pulmonary embolism (PE). Post-thrombotic syndrome (PTS) may be a long complication. Local occlusion of the catheter tip may be managed with instillation of thrombolytics (such as tPA) within the lumen of the catheter; however, CVC-associated thrombosis involving the proximal veins is most often is treated with systemic anticoagulation. Initial treatment with heparin is a standard approach, but thrombolytic therapy, which may carry higher bleeding risks, should be considered for life and limb threatening episodes of VTE. Recommended duration of anticoagulation with low molecular weight heparin (LMWH) or warfarin ranges from 3 to 6 months for major removable thrombotic risks; longer anticoagulation is considered for recurrent thrombosis, major persistent thrombophilia, or the continued presence of a major risk factor such as a CVC. While CVCs are the most common risk for development of VTE in children, studies have not demonstrated a clear benefit with routine use of systemic thromboprophylaxis. The incidence and risk factors of VTE in CF patients will be reviewed and principles of diagnosis and management will be summarized.

  1. Cystic fibrosis in adult age.

    PubMed

    Lerín, M; Prados, C; Martínez, M T; Maíz, L; Girón, R; Solé, A; Cabanillas, J J; Alvarez-Sala, R

    2014-01-01

    To know the prevalence of the patients diagnosed of cystic fibrosis (CF) older than 18 years old of five specific Spanish Units and to analyze their clinical, genetic and microbiological characteristics. Observational, cross-sectional, descriptive study of patients diagnosed with CF at age or older than 18 years. The variables analyzed were: current age, age at diagnosis, sex, nationality, lung function parameters, pathologies presented at diagnosis, microbiological features and genetic findings. Eigthy nine patients (14.8% of the total of 600 CF patients followed at the participating units), of which 45 patients were female (50.6%) and 44 were males (49.4%), were included with a mean age at diagnosis of 36.4 years. Eigthy one patients (91%) were Spaniards. The sweat test was diagnostic in 77 (86.5%) of the patients studied. The sweat test was diagnostic in 77 of the 89 patients studied (86.5%). The most frequently detected mutations were F508del/other and G542X/other, and the most frequent clinical findings at diagnosis were the presence of bronchiectasis in 33 patients (37.1%) followed by sterility in 12 patients (13.5%). The most common colonizing organisms were meticillin-sensitive Staphylococcus aureus (S.aureus) (23.6%) and Pseudomonas aeruginosa (P. aeruginosa) (13.5%). Most patients presented a mild obstructive ventilatory defect and had no pancreatic involvement. The sweat test used to be indeterminate. CF is also a disease which diagnosis can be in adulthood. CF patients diagnosed in adulthood have a mild lung function and lower incidence of pancreatic involvement, so their prognosis tends to be favorable. Copyright © 2014 Elsevier España, S.L. All rights reserved.

  2. Cystic echinococcosis in Southern Israel.

    PubMed

    Ben-Shimol, Shalom; Sagi, Orli; Houri, Ohad; Bazarsky, Elina; Berkowitz, Anat; Bulkowstein, Shlomi; Barrett, Chiya; Greenberg, David

    2016-01-01

    The aim of this retrospective, population-based study was to characterize demographically and clinically cystic-echinococcosis (CE) in southern Israel, between 2005 and 2012. Newly-diagnosed (nd-CE) and past-diagnosed (pd-CE, diagnosed before the study) cases were defined. Two populations live in southern-Israel, receiving medical treatment at a single hospital: the Jewish and the Bedouin populations (resembling resource-rich and resource-poor populations, respectively). 126 CE cases were identified; 55 nd-CE and 71 pd-CE. Mean annual nd-CE incidence per 100,000 in the Bedouin and Jewish populations were 2.7 ± 1.2 and 0.4 ± 0.3, respectively (P<0.001). None of the Bedouin and 86.5% of the Jewish patients were born outside Israel. Liver and lung involvement were recorded in 85.7% and 15.1% of overall-CE, respectively. Abdominal pain, cough, fever, eosinophilia and asymptomatic disease were documented in 63.6%, 32.7%, 27.3%, 41.5% and 12.7% of nd-CE, respectively. Serology sensitivity for first test and any positive test were 67.3% and 83.3%, respectively. Computed tomography, ultrasonography and X-ray diagnosis were documented in 79.2%, 58.4% and 17.0% of overall-CE, respectively, with ultrasonography mainly used in liver-CE and X-ray in lung-CE. Treatment included surgery and albendazole in 50.0% and 55.3% of CE, respectively. We conclude that CE is endemic in southern-Israel among the Bedouin population, while disease is probably mainly imported in the Jewish population. Liver involvement and eosinophilia rates were high compared with those of other endemic regions, possibly due to differences in the timing of diagnosis. These findings may help developing treatment and prevention strategies.

  3. Enteral tube feeding for cystic fibrosis.

    PubMed

    Morton, Alison; Wolfe, Susan

    2015-04-09

    Enteral tube feeding is routinely used in many cystic fibrosis centres when oral dietary and supplement intake has failed to achieve an adequate nutritional status. The use of this method of feeding is assessed on an individual basis taking into consideration the patients age and clinical status. To examine the evidence that in people with cystic fibrosis, supplemental enteral tube feeding improves nutritional status, respiratory function, and quality of life without significant adverse effects. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also contacted the companies that market enteral feeds and reviewed their databases.Date of the most recent search of the Group's Cystic Fibrosis Trials Register: 13 February 2015.Date of the most recent hand search of PubMed and conference abstract books: 13 February 2015. All randomised controlled trials comparing supplemental enteral tube feeding for one month or longer with no specific intervention in people with cystic fibrosis. The searches identified 38 trials; however, none were eligible for inclusion in this review. There are no trials included in this review. Supplemental enteral tube feeding is widely used throughout the world to improve nutritional status in people with cystic fibrosis. The methods mostly used, nasogastric or gastrostomy feeding, are expensive and may have a negative effect on self-esteem and body image. Reported use of enteral tube feeding suggests that it results in nutritional and respiratory improvement; but, efficacy has not been fully assessed by randomised controlled trials. It is acknowledged, however, that performing a randomised controlled trial would be difficult due to the ethics of withholding an intervention in a group of patients whose nutritional status necessitates it.

  4. Liver Disease in Cystic Fibrosis: an Update

    PubMed Central

    Parisi, Giuseppe Fabio; Di Dio, Giovanna; Franzonello, Chiara; Gennaro, Alessia; Rotolo, Novella; Lionetti, Elena; Leonardi, Salvatore

    2013-01-01

    Context Cystic fibrosis (CF) is the most widespread autosomal recessive genetic disorder that limits life expectation amongst the Caucasian population. As the median survival has increased related to early multidisciplinary intervention, other manifestations of CF have emergedespecially for the broad spectrum of hepatobiliary involvement. The present study reviews the existing literature on liver disease in cystic fibrosis and describes the key issues for an adequate clinical evaluation and management of patients, with a focus on the pathogenetic, clinical and diagnostic-therapeutic aspects of liver disease in CF. Evidence Acquisition A literature search of electronic databases was undertaken for relevant studies published from 1990 about liver disease in cystic fibrosis. The databases searched were: EMBASE, PubMed and Cochrane Library. Results CF is due to mutations in the gene on chromosome 7 that encodes an amino acidic polypeptide named CFTR (cystic fibrosis transmembrane regulator). The hepatic manifestations include particular changes referring to the basic CFTR defect, iatrogenic lesions or consequences of the multisystem disease. Even though hepatobiliary disease is the most common non-pulmonary cause ofmortalityin CF (the third after pulmonary disease and transplant complications), only about the 33%ofCF patients presents clinically significant hepatobiliary disease. Conclusions Liver disease will have a growing impact on survival and quality of life of cystic fibrosis patients because a longer life expectancy and for this it is important its early recognition and a correct clinical management aimed atdelaying the onset of complications. This review could represent an opportunity to encourage researchers to better investigate genotype-phenotype correlation associated with the development of cystic fibrosis liver disease, especially for non-CFTR genetic polymorphisms, and detect predisposed individuals. Therapeutic trials are needed to find strategies of

  5. Enteral tube feeding for cystic fibrosis.

    PubMed

    Conway, Steven; Morton, Alison; Wolfe, Susan

    2012-12-12

    Enteral tube feeding is routinely used in many cystic fibrosis centres when oral dietary and supplement intake has failed to achieve an adequate nutritional status. The use of this method of feeding is assessed on an individual basis taking into consideration the patients age and clinical status. To examine the evidence that in people with cystic fibrosis, supplemental enteral tube feeding improves nutritional status, respiratory function, and quality of life without significant adverse effects. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also contacted the companies that market enteral feeds and reviewed their databases.Date of the most recent search of the Group's Cystic Fibrosis Trials Register: 03 September 2012.Date of the most recent hand search of PubMed and conference abstract books: 15 June 2012. All randomised controlled trials comparing supplemental enteral tube feeding for one month or longer with no specific intervention in people with cystic fibrosis. Thirty-one trials were identified by the searches; however, none were eligible for inclusion in this review. There are no trials included in this review. Supplemental enteral tube feeding is widely used throughout the world to improve nutritional status in people with cystic fibrosis. The methods mostly used, nasogastric or gastrostomy feeding, are expensive and may have a negative effect on self-esteem and body image. Reported use of enteral tube feeding suggests that it results in nutritional and respiratory improvement; but, efficacy has not been fully assessed by randomised controlled trials. It is acknowledged, however, that performing a randomised controlled trial would be difficult due to the ethics of withholding an intervention in a group of patients whose nutritional status

  6. The Cystic Fibrosis of Exocrine Pancreas

    PubMed Central

    Wilschanski, Michael

    2013-01-01

    The cystic fibrosis transmembrane conductance regulator (CFTR) protein is highly expressed in the pancreatic duct epithelia, and permits anions and water to enter the ductal lumen. This results in an increased volume of alkaline fluid allowing the highly concentrated proteins secreted by the acinar cells to remain in a soluble state. This work will expound on the pathophysiology and pathology caused by the malfunctioning CFTR protein with special reference to ion transport and acid-base abnormalities both in humans and animal models. We will also discuss the relationship between cystic fibrosis (CF) and pancreatitis, and outline present and potential therapeutic approaches in CF treatment relevant to the pancreas. PMID:23637307

  7. Management Issues for Adolescents with Cystic Fibrosis

    PubMed Central

    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

  8. 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.

  9. 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.

  10. Precision genomic medicine in cystic fibrosis

    PubMed Central

    Chang, Eugene H.; Zabner, Joseph

    2015-01-01

    The successful application of precision genomic medicine requires an understanding of how a person’s genome can influence their 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

  11. Sonographic-pathologic correlation of complex cystic breast lesions

    PubMed Central

    Pongrattanaman, Saravech; Prueksadee, Jenjeera

    2013-01-01

    Objective To understand the pathologic basis for sonographic features of complex cystic lesions. Methods From 2 646 female patients underwent breast sonography at King Chulalongkorn Memorial Hospital from January 2005 through December 2010, 103 cystic lesions were included. Pathologic confirmation was performed by fine-needle aspiration (n=42), core needle biopsy (n=6), excision (n=54) and mastectomy (n=1). Complex cystic breast masses were classified into 3 types as followings; thick outer wall and/or thick internal septa (type I); thick septation and thick wall were defined as equal or more than 0.5 cm, masses containing mixed cystic and solid components (at least 50% of cystic component) (type II), predominantly solid with eccentric cystic foci (at least 50% of solid component) (type III). Results In 103 complex cystic masses, there are 27 lesions (26%) classified as type I cystic breast masses, 37 lesions (36%) as type II cystic breast masses and 39 lesions (38%) type III cystic breast masses, 26 lesions (25.2%) are proved to be malignant. All of type I cystic breast masses in our study are benign, and 14 (38%) of type II cystic breast masses and 12 lesions (31%) of type III cystic breast lesions are proved to be malignant. Conclusions Type II and III lesions should suggest possibility of malignancy and biopsy should be performed in all lesions. All type I lesion in this study are benign. None of other parameters we included in this study (size or margin) can effectively differentiate between benign or malignant cystic breast lesions. Also, grading of the malignant lesions by using type of cystic breast mass cannot be applied.

  12. The Usefulness of Positron-Emission Tomography Findings in the Management of Anterior Mediastinal Tumors

    PubMed Central

    Sano, Fumitoshi; Ohashi, Shinichi; Suzuki, Kosuke; Uematsu, Shugo; Suzuki, Takashi; Kadokura, Mitsutaka

    2017-01-01

    Purpose: We performed a retrospective analysis to evaluate the usefulness of positron-emission tomography/computed tomography (PET/CT) findings in the classification and management of anterior mediastinal tumors. Methods: Between 2006 and 2015, 105 patients with anterior mediastinal tumor received PET/CT. 18F-fluorodeoxyglucose (18F-FDG)-PET images were obtained 60 minutes after the injection of 18F-FDG. Results: The histological classifications were as follows: thymoma (n = 49), thymic carcinoma (TC) (n = 19), malignant lymphoma (ML) (n = 8), teratoma (n = 7), thymic cyst (n = 14), and others (n = 8). Upon visual inspection (SUV max: >2.0), all of the malignant tumors showed 18F-FDG accumulation (with the exception of one type A thymoma). Two of the 14 thymic cysts and three of the seven teratomas showed slight 18F-FDG accumulation. The SUV max values of the low-grade thymomas, high-grade thymomas, TCs and MLs were 3.14 ± 0.73, 4.34 ± 1.49, 8.59 ± 3.05, and 10.08 ± 2.53, respectively, with significant differences between the low- and high-grade thymomas, and between TCs and MLs. The sensitivity, specificity and accuracy of 18F-FDG in the detection of low-grade thymomas and thymomas with a maximum diameter of ≤50 mm and an SUV max of ≤3.4 were 85%, 48%, and 60%, respectively. Conclusion: FDG-PET/CT is an objective and useful modality in the differential diagnosis and management of anterior mediastinal tumors. PMID:28123154

  13. 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.

  14. Atypical presentation of a mediastinal mass in an adolescent: Critical care considerations.

    PubMed

    Yester, Marc A; Ajizian, Samuel J

    2010-07-01

    To describe the clinical course and treatment of a large mediastinal mass with unusual presentation and critical lower airway compression in an adolescent. Case report. Pediatric intensive care unit in a tertiary care, academic children's hospital. A previously well 15-yr-old boy presented to an outside physician with a 2-mo history of widening of his fingernail beds, progressing within a month of admission to fatigue, weight loss, progressive cough, and dyspnea on exertion. One week before admission, he developed facial swelling, headache, and large neck, chest, and abdomen veins. At the time of admission, he was hypoxic and had a large mediastinal mass with severe lower airway compromise, right-sided atelectasis and pleural effusion, as well as significant right atrial compression on chest computed tomography. The patient was placed in the pediatric intensive care unit and underwent emergent tube thoracostomy and drainage of the pleural effusion in the upright position, using a local anesthetic. : The patient developed mild reexpansion pulmonary edema with worsening hypoxia, which was managed using bilevel positive airway pressure. Pleural fluid was nondiagnostic, as was bone marrow aspirate and biopsy done in similar fashion on day 2. The patient then underwent a fine-needle biopsy in the operating room, also nonintubated and upright, which diagnosed non-Hodgkin's lymphoma, nodular sclerosing type. Treatment for tumor lysis syndrome and chemotherapy were initiated, and he progressively improved. Mediastinal mass with true critical airway and vascular compromise is often discussed but infrequently seen in the pediatric intensive care unit. This case shows not only unusual associated signs of lymphoma (clubbing and caput medusae) but more importantly the rapid identification and thoughtful management of the patient's respiratory compromise. This case serves to remind the pediatric intensivist of alternative ways to provide analgesia safely in such patients for

  15. Origin and pharmacological response of atrial tachyarrhythmias induced by activation of mediastinal nerves in canines.

    PubMed

    Armour, J Andrew; Richer, Louis-Philippe; Pagé, Pierre; Vinet, Alain; Kus, Teresa; Vermeulen, Michel; Nadeau, Réginald; Cardinal, René

    2005-03-31

    We sought to determine the sites of origin of atrial tachyarrhythmias induced by activating mediastinal nerves, as well as the response of such arrhythmias to autonomic modulation. Under general anaesthesia, atrioventricular block was induced after thoracotomy in 19 canines. Brief trains of 5 electrical stimuli were delivered to right-sided mediastinal nerves during the atrial refractory period. Unipolar electrograms were recorded from 191 right and left atrial epicardial sites under several conditions, i.e. (i) with intact nervous systems and following (ii) acute decentralization of the intrathoracic nervous system or administration of (iii) atropine, (iv) timolol, (v) hexamethonium. Concomitant right atrial endocardial mapping was performed in 7 of these dogs. Mediastinal nerve stimulation consistently initiated bradycardia followed by atrial tachyarrhythmias. In the initial tachyarrhythmia beats, early epicardial breakthroughs were identified in the right atrial free wall (28/50 episodes) or Bachmann bundle region (22/50), which corresponded to endocardial sites of origin associated with the right atrial subsidiary pacemaker complex, i.e. the crista terminalis and dorsal locations including the right atrial aspect of the interatrial septum. Neuronally induced responses were eliminated by atropine, modified by timolol and unaffected by acute neuronal decentralization. After hexamethonium, responses to extra-pericardial but not intra-pericardial nerve stimulation were eliminated. It is concluded that concomitant activation of cholinergic and adrenergic efferent intrinsic cardiac neurons induced by right-sided efferent neuronal stimulation initiates atrial tachyarrhythmias that originate from foci anatomically related to the right atrial pacemaker complex and tissues underlying major atrial ganglionated plexuses.

  16. Longterm effects of cardiac mediastinal nerve cryoablation on neural inducibility of atrial fibrillation in canines.

    PubMed

    Leiria, Tiago Luiz Luz; Glavinovic, Tamara; Armour, J Andrew; Cardinal, René; de Lima, Gustavo Glotz; Kus, Teresa

    2011-04-26

    In canines, excessive activation of select mediastinal nerve inputs to the intrinsic cardiac nervous system induces atrial fibrillation (AF). Since ablation of neural elements is proposed as an adjunct to circumferential pulmonary vein ablation for AF, we investigated the short and long-term effects of mediastinal nerve ablation on AF inducibility. Under general anesthesia, in 11 dogs several mediastinal nerve sites were identified on the superior vena cava that, when stimulated electrically during the atrial refractory period, reproducibly initiated AF. Cryoablation of one nerve site was then performed and inducibility retested early (1-2 months post Cryo; n=7) or late (4 months post Cryo; n=4). Four additional dogs that underwent a sham procedure were retested 1 to 2 months post-surgery. Stimulation induced AF at 91% of nerve sites tested in control versus 21% nerve sites early and 54% late post-ablation (both P<0.05). Fewer stimuli were required to induce AF in controls versus the Early Cryo group; this capacity returned to normal values in the Late Cryo group. AF episodes were longer in control versus the Early or Late Cryo groups. Heart rate responses to vagal or stellate ganglion stimulation, as well as to local nicotine infusion into the right coronary artery, were similar in all groups. In conclusion, focal damage to intrinsic cardiac neuronal inputs causes short-term stunning of neuronal inducibility of AF without major loss of overall adrenergic or cholinergic efferent neuronal control. That recovery of AF inducibility occurs rapidly post-surgery indicates the plasticity of intrathoracic neuronal elements to focal injury. Copyright © 2011 Elsevier B.V. All rights reserved.

  17. In patients with post-sternotomy mediastinitis is vacuum-assisted closure superior to conventional therapy?

    PubMed

    Yu, Angela W; Rippel, Radoslaw A; Smock, Elliott; Jarral, Omar A

    2013-11-01

    A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether vacuum-assisted closure therapy (VAC) is superior to conventional therapy for treating post-sternotomy mediastinitis. Altogether >261 papers were found using the reported search, of which 9 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. Several studies indicate that VAC therapy is associated with shorter lengths of intensive care and in-hospital stay as well as faster rates of wound healing and fewer dressing changes. It has also been shown that VAC therapy is correlated with a statistically significant reduction in reinfection rates, particularly those that occur in the early postoperative period (at the 1-week follow-up). Patients can be discharged with the dressing in situ and managed in the community with a view to delayed closure or reconstruction. However, the studies comparing VAC with conventional therapy are all retrospective in nature and reinforce the need for randomized controlled trials in order to more accurately establish differences in outcomes between VAC and conventional therapy. Additionally, owing tło the variability of treatment protocols within the non-VAC arm, it is more challenging to draw definitive conclusions regarding the superiority of VAC therapy to every modality that is considered conventional treatment. We conclude that VAC therapy is a portable and an increasingly economical option for the treatment of post sternotomy mediastinitis. Although reductions in mortality rates were not reproduced in all studies, evidence suggests that VAC should still be considered as a first-line therapy for post-sternotomy mediastinitis and as a bridge therapy to musculocutaneous reconstruction or primary closure.

  18. 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.

  19. Response of the mediastinal and thoracic viscera of the dog to intraoperative radiation therapy (IORT)

    SciTech Connect

    Barnes, M.; Pass, H.; DeLuca, A.; Tochner, Z.; Potter, D.; Terrill, R.; Sindelar, W.F.; Kinsella, T.J.

    1987-03-01

    IORT may be a potentially useful adjunctive treatment combined with surgery and/or external beam irradiation in treating locally advanced lung and esophageal tumors. To begin investigation of this modality, the tolerance of intact mediastinal structures to IORT was studied using adult American Foxhounds (wt. 25-30 kg). Groups of six animals received IORT to doses of 20, 30, or 40 Gy to two separate intrathoracic ports, using 9 MeV electrons to treat a portion of the collapsed right upper lobe, and 12 MeV electrons to treat the mediastinal structures. A group of three dogs received thoracotomy with sham irradiation. Two dogs from each treatment dose group, as well as one sham-irradiated control, were sacrificed electively at 1, 3, and 12 months following IORT. There were no acute nor late IORT related mortalities. Post-operative weight loss was minimal (average 4.5% of pre-operative weight) for all dogs. Serial esophagrams showed no inflammation or ulceration. No cardiac nor pulmonary changes were noted clinically. At autopsy, the irradiated lung showed evidence of acute pneumonitis at 1 month with progressive fibrosis at 3 months and 1 year. Esophageal reactions were minimal, with only two dogs (one 30 Gy and one 40 Gy) demonstrating histologically confirmed esophagitis at 1 month. Tracheal changes were minimal. Cardiac damage was evident in the right atrial tissues. In several dogs, this cardiac damage ranged from myocardial vascular changes to frank ischemic necrosis noted at 1 and 3 months, and dense fibrosis at 1 year. The phrenic nerves showed normal function, but had evidence of perineural fibrosis. The large vessels demonstrated only mild histologic evidence of irradiation. The results of this large animal study suggest that intact mediastinal structures will tolerate small volume IORT to doses of 20 Gy without significant clinical sequellae. (Abstract Truncated)

  20. 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

  1. 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.

  2. Cystic hypersecretory ductal carcinoma of the breast: a rare cause of cystic breast mass.

    PubMed

    Song, Sun Wha; Whang, In Yong; Chang, Eun Deok

    2011-11-01

    We present the case of a surgically confirmed, invasive, cystic hypersecretory ductal carcinoma (CHDC) of the breast in a 43-year-old woman. The initial sonography showed a complex cyst, which required a core biopsy; however, the diagnosis was delayed as the patient refused to undergo the biopsy and the cyst decreased in size, as seen on follow-up sonography. Excision biopsy was performed, and invasive CHDC was diagnosed after regrowth of the cystic lesion. Meticulous sonographic evaluation of a cystic breast mass is always important, and pathology confirmation must be considered if the lesion shows features suspicious for malignancy, as a CHDC could be the cause of a cystic breast mass.

  3. Hemomediastinum due to spontaneous rupture of a mediastinal bronchial artery aneurysm – A rare cause of thoracic pain

    PubMed Central

    Vosse, B.A.H.; van Belle, A.F.; de Vries, G.J.; Das, M.

    2014-01-01

    Hemomediastinum is a rare pathological event. Multiple underlying causes and contributory factors can be identified, such as trauma, malignancy, iatrogenic, bleeding disorder or mediastinal organ hemorrhage. Also, a mediastinal bronchial artery aneurysm may be the source of a hemomediastinum. Hemoptysis is an important directive symptom, however occasionally, patients only present with thoracic pain or symptoms related to extrinsic compression of the airways or esophagus. Using contrast-enhanced computed tomography (CT) of the chest, hemomediastinum can be adequately diagnosed, and the involved vascular structures can be revealed. In case of a (ruptured) bronchial artery aneurysm, transcatheter embolization provides a minimally invasive procedure and is treatment of first choice. In this case report, a 76-year-old female is presented with spontaneous rupture of a mediastinal bronchial artery aneurysm resulting in hemomediastinum causing thoracic pain. Superselective embolization of the left bronchial artery was successfully performed. PMID:26029533

  4. Giant pulmonary bulla with mediastinal shift in a 12 1/2 year old girl.

    PubMed

    Fatimi, Saulat Hasnain; Jafferani, Asif; Ashfaq, Awais

    2012-05-01

    Pulmonary bulla in children represent interesting entities. Mostly congenital bronchopulmonary foregut malformations and acquired cysts like pneumatocoeles have also been described. We present a case of a 12 1/2 years old girl with acute onset respiratory distress symptoms harbouring a huge pulmonary cyst exhibiting mass effects, resulting in mediastinal deviation. Following initial workup, cyst excision was carried out which revealed presence of fungal hyphae that was susceptible to Fluconazole therapy post operatively. The case points out how a pulmonary cyst can present in older children with symptoms of respiratory distress and mass effects.

  5. Prostate Cancer Presenting as Huge Mediastinal and Retroperitoneal Masses: Case Report and Review of the Literature.

    PubMed

    Alshaikh, Safa; Harb, Zainab

    2017-01-01

    Mediastinum and retroperitoneum are exceedingly rare sites for metastatic prostate cancer to occur. Here, we present the case of a 67-year-old male patient with incidental findings of mediastinal and retroperitoneal masses which were found to be due to metastatic prostate adenocarcinoma based on histopathology and immunohistochemical studies and later on supported by the significantly elevated Prostate Specific Antigen (PSA) levels. Prostate cancer should always be considered in the differential diagnosis of elderly men presenting with metastatic epithelial tumors even in unusual sites.

  6. 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.

  7. 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.

  8. Pneumomediastinum and Mediastinal Hematoma Secondary to Right Brachiocephalic Vein Thrombectomy Mimicking STEMI

    PubMed Central

    Shukla, Prem; Nivera, Noel

    2017-01-01

    A 50-year-old male with a history of hemodialysis dependent chronic kidney disease presented to our emergency department with acute midsternal crushing chest pain. Patient was diagnosed with acute anterolateral wall Myocardial Infraction due to the presence of corresponding ST segment elevations in EKG and underwent emergent cardiac catheterization which revealed normal patent coronaries without any disease. He continued to have chest pain for which CT of the chest was done which revealed pneumomediastinum with mediastinal hematoma, due to the recent attempted thrombectomy for thrombus in his right brachiocephalic vein. PMID:28804656

  9. Complete Obstruction of Endotracheal Tube in an Infant with a Retropharyngeal and Anterior Mediastinal Abscess

    PubMed Central

    Greene, Nathaniel H.

    2017-01-01

    Intraoperative ventilatory failure is not an uncommon complication; however, acute endotracheal obstruction by a foreign body or blood clot can be difficult to quickly discriminate from other causes. Once the diagnosis is made, quick action is needed to restore ventilation. The ultimate solution is to exchange the endotracheal tube; however, there can be other ways of resolving this in situations where reintubation would be difficult or unsafe. This case report discusses such an event in an infant with multiple airway challenges including a retropharyngeal and anterior mediastinal abscess. We have also formulated a pathway based on various case reports involving complete ETT obstruction. PMID:28299222

  10. 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.

  11. Colorectal Cancer Metastasis to the Thymus Gland: Rare Presentation of Colorectal Cancer as Anterior Mediastinal Mass

    PubMed Central

    Peters, H. Charles; Liu, Xiuli; Iqbal, Atif; Cunningham, Lisa A.

    2017-01-01

    Despite improved screening modalities, 15–25% of newly diagnosed colorectal cancers are metastatic at the time of diagnosis. The vast majority of these cases present as hepatic metastasis; however, 22% present with concomitant extrahepatic disease. The thymus gland is an uncommon site of metastasis for any primary malignancy, particularly, colorectal cancer given its vascular and lymphatic drainage. This case report details our experience with a rare case of colorectal cancer metastasis to the thymus gland presenting as a symptomatic mediastinal mass. PMID:28116210

  12. Mediastinal gray zone lymphoma: clinico-pathological characteristics and outcomes of 99 patients from the Lymphoma Study Association

    PubMed Central

    Sarkozy, Clémentine; Molina, Thierry; Ghesquières, Hervé; Michallet, Anne-Sophie; Dupuis, Jehan; Damotte, Diane; Morsschauser, Franck; Parrens, Marie; Martin, Laurent; Dartigues, Peggy; Stamatoullas, Aspasia; Hirsch, Pierre; Fabiani, Bettina; Bouabdallah, Krimo; da Silva, Maria Gomes; Maerevoet, Marie; Laurent, Camille; Coiffier, Bertrand; Salles, Gilles; Traverse-Glehen, Alexandra

    2017-01-01

    Mediastinal gray zone lymphoma, B-cell lymphomas with intermediate features between classical Hodgkin lymphoma and primary mediastinal B-cell lymphoma, have not been well described in the literature. We report the clinical characteristics and outcomes of a large retrospective series of 99 cases centrally reviewed by a panel of hematopathologists, with a consensus established for the diagnosis. Cases were defined as classical Hodgkin lymphoma-like morphology (64.6%) with primary mediastinal B-cell lymphoma immunophenotype, primary mediastinal B-cell lymphoma-like morphology (30.3%) with classical Hodgkin lymphoma or composite (5.1%) (synchronous occurrence of classical Hodgkin lymphoma and primary mediastinal B-cell lymphoma). The median age was 32 years (13–83 years); 55% were women. Thirteen of 81 evaluable cases (16%) were Epstein-Barr virus-positive. Twenty-eight percent of patients presented primary refractory disease (progression under first-line treatment or relapse within one year). The 3-year event-free and overall survival rates were 63% and 80%, respectively. Patients treated with a standard regimen (RCHOP/ABVD) had worse event-free survival (P=0.003) and overall survival (P=0.02) than those treated with a dose-intensive chemotherapy (high-dose RCHOP/escalated BEACOPP). Rituximab added to chemotherapy was not associated with better event-free survival (P=0.55) or overall survival (P=0.88). Radiotherapy for patients in complete remission had no impact on event-free survival. In multivariate prognostic analysis, ECOG-PS and anemia were the strongest factors associated with a shorter event-free survival and overall survival, respectively. In conclusion, this report describes the largest series of mediastinal gray zone lymphoma. Our data suggest that a dose-intensive treatment might improve the outcome of this rare and aggressive disease. PMID:27758822

  13. Mediastinal gray zone lymphoma: clinico-pathological characteristics and outcomes of 99 patients from the Lymphoma Study Association.

    PubMed

    Sarkozy, Clémentine; Molina, Thierry; Ghesquières, Hervé; Michallet, Anne-Sophie; Dupuis, Jehan; Damotte, Diane; Morsschauser, Franck; Parrens, Marie; Martin, Laurent; Dartigues, Peggy; Stamatoullas, Aspasia; Hirsch, Pierre; Fabiani, Bettina; Bouabdallah, Krimo; da Silva, Maria Gomes; Maerevoet, Marie; Laurent, Camille; Coiffier, Bertrand; Salles, Gilles; Traverse-Glehen, Alexandra

    2017-01-01

    Mediastinal gray zone lymphoma, B-cell lymphomas with intermediate features between classical Hodgkin lymphoma and primary mediastinal B-cell lymphoma, have not been well described in the literature. We report the clinical characteristics and outcomes of a large retrospective series of 99 cases centrally reviewed by a panel of hematopathologists, with a consensus established for the diagnosis. Cases were defined as classical Hodgkin lymphoma-like morphology (64.6%) with primary mediastinal B-cell lymphoma immunophenotype, primary mediastinal B-cell lymphoma-like morphology (30.3%) with classical Hodgkin lymphoma or composite (5.1%) (synchronous occurrence of classical Hodgkin lymphoma and primary mediastinal B-cell lymphoma). The median age was 32 years (13-83 years); 55% were women. Thirteen of 81 evaluable cases (16%) were Epstein-Barr virus-positive. Twenty-eight percent of patients presented primary refractory disease (progression under first-line treatment or relapse within one year). The 3-year event-free and overall survival rates were 63% and 80%, respectively. Patients treated with a standard regimen (RCHOP/ABVD) had worse event-free survival (P=0.003) and overall survival (P=0.02) than those treated with a dose-intensive chemotherapy (high-dose RCHOP/escalated BEACOPP). Rituximab added to chemotherapy was not associated with better event-free survival (P=0.55) or overall survival (P=0.88). Radiotherapy for patients in complete remission had no impact on event-free survival. In multivariate prognostic analysis, ECOG-PS and anemia were the strongest factors associated with a shorter event-free survival and overall survival, respectively. In conclusion, this report describes the largest series of mediastinal gray zone lymphoma. Our data suggest that a dose-intensive treatment might improve the outcome of this rare and aggressive disease.

  14. Psychological interventions for cystic fibrosis.

    PubMed

    Glasscoe, C A; Quittner, A L

    2003-01-01

    As survival estimates for cystic fibrosis (CF) steadily increase long-term management has become an important focus for intervention. Psychological interventions are largely concerned with emotional and social adjustments, adherence to treatment and quality of life, however no systematic review of such interventions has been undertaken for this disease. To describe the extent and quality of effectiveness studies utilising psychological interventions for CF and whether these interventions provide significant psychosocial and physical benefits in addition to standard care. Relevant trials were identified from searches of Ovid MEDLINE, the Cochrane trial registers for CF and Depression, Anxiety and Neurosis Groups and PsychINFO; unpublished trials were located through professional networks and Listserves. Most recent search: April 2003. This review included RCTs and quasi-randomised trials. Study participants were children and adults diagnosed with CF, and their immediate family members. Psychological interventions were from a broad range of modalities and outcomes were primarily psychosocial, although physical outcomes and cost effectiveness were also considered. Two reviewers independently selected relevant trials and assessed their methodological quality. For binary and continuous outcomes a pooled estimate of treatment effect was calculated for each outcome. This review is based on the findings of eight studies, representing data from a total of 358 participants. Studies fell into four conceptually similar groups: (1) gene pre-test education counselling for relatives of those with CF (one study); (2) biofeedback, massage and music therapy to assist physiotherapy (three studies); (3) behavioural intervention to improve dietary intake in children up to 12 years (three studies); and (4) self-administration of treatments to improve quality of life in adults (one study). Interventions were largely educational or behavioural, targeted at specific treatment concerns

  15. The diagnosis of cystic fibrosis.

    PubMed

    De Boeck, Kris; Vermeulen, Francois; Dupont, Lieven

    2017-06-01

    Establishing the diagnosis of cystic fibrosis (CF) is straight forward in the majority of patients: they present with a clear clinical picture (most frequently chronic respiratory symptoms plus malabsorption), the sweat chloride value is>60mmol/L and two known disease causing CFTR mutations are identified. In less than 5% of subjects, mainly those with a milder or limited phenotype, the diagnostic process is more complex, because initial diagnostic test results are inconclusive: sweat chloride concentration in the intermediate range, less than 2 CF causing mutations identified or both. These patients should be referred to expert centers where bioassays of CFTR function like nasal potential difference measurement or intestinal current measurement can be done. Still, in some patients, despite symptoms compatible with CF and some indication of CFTR dysfunction (e.g. only intermediate sweat chloride value), diagnostic criteria are not met (e.g. only 1 CFTR mutation identified). For these subjects, the term CFTR related disorder (CFTR-RD) is used. Patients with disseminated bronchiectasis, congenital bilateral absence of the vas deferens and acute or recurrent pancreatitis may fall in this category. CF has a very wide disease spectrum and increasingly the diagnosis is being made during adult life, mainly in subjects with milder phenotypes. In many countries, nationwide CF newborn screening (NBS) has been introduced. In screen positive babies, the diagnosis of CF must be confirmed by a sweat test demonstrating a sweat chloride concentration above 60mmol/L. To achieve the benefit of NBS, every baby in whom the diagnosis of CF is confirmed must receive immediate follow-up and treatment in a CF reference center. CF NBS is not full proof: some diagnoses will be missed and in some babies the diagnosis cannot be confirmed nor ruled out with certainty. Screening algorithms that include gene sequencing will detect a high number of such babies that are screen positive with an

  16. Treatment of stage i and ii mediastinal Hodgkin disease: a comparison of involved fields, extended fields, and involved fields followed by MOPP in patients stage by laparotomy

    SciTech Connect

    Hagemeister, F.B.; Fuller, L.M.; Sullivan, J.A.; North, L.; Velasquez, W.; Conrad, F.G.; McLaughlin, P.; Butter, J.J.; Shullenberger, C.C.

    1981-12-01

    Three treatment programs for Stage I and II mediastinal Hodgkin disease (established by laparotomy) were compared. Involved-field radiotherapy + MOPP gave a disease-free survival rate of 97%, significantly different from 62% and 55% for involved and extended fields, respectively. Corresponding survival figures of 97%, 88%, and 84% were not signiticantly different statistically due to salvage with radiotherapy and/or chemotherapy. Among patients given radiotherapy alone, the survival figure of 94% for limited mediastinal disease was significantly better than 63% for extensive mediastinal and hilar disease; corresponding disease-free figures of 72% and 35% were also significantly different. Constitutional symptoms were an important prognostic factor in disease-free survival following the use of involved fields; hilar disease was important only with large mediastinal masses. Most relapses were intrathoracic; MOPP alone salvaged only 47%. Treatment of State I and II Hodgkin disease should be based on symptoms, extent of mediastinal disease, and hilar involvement.

  17. Craniofacial morphology in children with cystic fibrosis.

    PubMed

    Hellsing, E; Brattström, V; Strandvik, B

    1992-04-01

    Cystic fibrosis (CF) is a hereditary metabolic disorder with clinical symptoms of abnormal mucus production. This blocks the airways, gives pancreatic insufficiency, and increases sweat electrolytes. The progressive respiratory disease often leads to respiratory insufficiency and cor pulmonale. The aim of the present investigation was to examine the facial morphology in children with cystic fibrosis. The sample comprised 11 children with cystic fibrosis, who were divided in two groups, one with gastrointestinal disorders and the other with predominantly respiratory insufficiency. Eleven healthy children with normal occlusions were selected as controls. Lateral skull radiographs obtained in natural head posture were digitized, and linear and angular variables for the different groups calculated and compared statistically. The cystic fibrosis group showed open bite, decreased posterior facial height, increased mandibular and craniocervical inclination. Additionally, within the CF-group, the children with respiratory insufficiency differed more from the controls than the children with gastrointestinal disorders. Despite the small number of subjects, the facial morphology of the CF children showed a similar pattern to that of children with nasal respiratory obstruction due to enlarged adenoids or tonsils.

  18. 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

  19. Ivacaftor for patients with cystic fibrosis.

    PubMed

    Wainwright, Claire E

    2014-10-01

    Ivacaftor is an oral bioavailable potentiator of the cystic fibrosis transmembrane conductance regulator protein. It is the first therapeutic agent that has been registered for clinical use which targets the basic defect in people with cystic fibrosis who carry a G551D mutation or other rarer specific gating mutations. Clinical trials have shown consistent and impressive clinical benefit that appears to be sustained over time in people with cystic fibrosis who carry a G551D mutation and similar benefits have been seen in those who carry rarer gating mutations. Ivacaftor is orally administered twice daily with a dose that does not vary between children aged 6 years through to adult life in patients with G551D. It appears to be well tolerated although there are potential interactions with drugs that are metabolised through CYPP450 CYP3A. Ivacaftor is also currently being trialled in combination with correctors for patients with the most common mutation of cystic fibrosis transmembrane conductance regulator the F508del mutation.

  20. Cystic fibrosis year in review 2016.

    PubMed

    Savant, Adrienne P; McColley, Susanna A

    2017-08-01

    In this article, we highlight cystic fibrosis (CF) research and case reports published in Pediatric Pulmonology during 2016. We also include articles from a variety of journals that are thematically related to these articles, or are of special interest to clinicians. © 2017 Wiley Periodicals, Inc.