RESUMEN
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.
RESUMEN
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.