Familial Near-Total Intestinal Aganglionosis.

Near total aganglionosis represents the most extreme and rare form of Hirschsprung's disease. It can affect more than one member of family. We report three cases of near total intestinal aganglionosis in a family presenting with intestinal obstruction at birth. All of them were operated and a jejunostomy was performed. Outcome was dismal.

Mesenteric cyst in infancy: presentation and management.

Mesenteric cysts are documented as a rare entity in pediatric population. They are considered as benign intra-abdominal tumors with an unknown etiology. Symptoms are not specific and knowledge of such condition is essential in order to establish a proper management. We report three pediatrics cases of mesenteric cysts managed between 2000 and 2009 in the pediatric surgery Department of Monastir College Hospital. We described the clinical, radiological and operative findings. Two males and a female were managed (age range: 10 days-5years, mean age: 6,3years). Two patients were presented with an intestinal obstruction. A preoperative diagnosis was made basing on imaging. Thus, abdominal ultrasonography was performed in all of our reported cases and showed a cystic mass in all cases. The cystic nature of the mass, its margins and its extension were better described on tomographic images. The mesenteric cyst was completely and successfully removed in all cases. The histopathological report confirmed the diagnosis and showed a multiloculated cyst with columnar mesothelial lining, without any defined muscular layer or cellular atypia and without any evidence of malignancy. The children were evaluated post-operatively with a mean follow-up of 2 years and a half. No recurrence was noted in our patients during the follow-up period. It is known that clinical features are not specific of such anomaly but once the diagnosis is made, the complete surgical removal of the cyst remains the treatment of choice with excellent outcomes.

[Biliary atresia and polysplenia syndrome]. / Atrésie des voise biliaires et syndrome de polysplénie.

Polysplenia syndrome is a rare malformation characterized by the association of multiple rates and other congenital anomalies dominated by cardiac, vascular, intestinal and bile malformations. We report the observation of a patient operated in the neonatal period (3 days) for an upper intestinal obstruction with situs inversus. Surgical exploration noted the presence of multiple rates, a preduodenal vein, a biliary atresia and a duodenal atresia. The surgical procedures performed were a latero-lateral duodeno-duodenostomy and hepatoportoenterostomy of KASAI with simple immediate and delayed outcomes. The follow up was of 23 years. We recall the epidemiological characteristics of this malformative association and we discuss the role played by the prognosis of polysplenia syndrome in the evolution of biliary atresia. The diagnosis and treatment of biliary atresia are always urgent to increase the chances of success of the Kasai, and the chances of prolonged survival with native liver. However, almost all long-term survivors (even anicteric) have biliary cirrhosis, which requires lifelong follow up.

Thoracoscopic treatment of pulmonary hydatid cyst in children: a report of 25 cases.

BACKGROUND: Open surgery is the standard option for the treatment of hydatid pulmonary cysts. Surgeons are able to replicate the principles of conventional surgery using minimally invasive techniques ,in particular thoracoscopy. However, there are few reports about this subject in children. To our knowledge, this is one of the biggest pediatric series ever reported in the literature. aim: the purpose of this study was to determine the best indications of the thoracospic surgery for the treatment of the pulmonary hydatid cysts in children. methods: We report a series of 25 cases with pulmonary hydatid cysts treated using the thoracoscopic approach from 2005 to 2009. We retrospectively analyzed the patients' sex, age, symptoms, biological data, characteristics of hydatid cysts (location, number and size) and the medical treatments. Pulmonary hydatid cyst diagnosis was performed on Chest x-ray, abdominal ultrasound and biological data in all the cases. Tomography was not systematic. All patients underwent video-assisted surgery. A conversion to thoracotomy was conducteded in 2 cases. All the patients had a chest tube and received an antibio-prophylaxy , without Albendazol . A concurrent hydatid cyst at the opposite lung or in the peritoneal cavity was treated later. results: There were 25 cases with a sex ratio of 2.1 and a mean age of 8 years. All the patients underwent a video-assisted surgery. A conversion to thoracotomy was performed in 2 cases because hydatid cysts were large (size > 8 cm). The average duration of the procedure was 75 minutes. In the post-operative course, one patient presented an air leak which required a prolonged chest drainage for 16 days and a second one was treated for lung infection. In all the other cases, the follow-up was uneventful. CONCLUSION: The thoracoscopic approach for pulmonary hydatid cysts in children is feasible. For better results, it is recommended for cysts smaller than 5 cm.