ABSTRACT
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.
ABSTRACT
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.
