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L´urétérocèle est une dilatation pseudo-kystique de l´uretère terminal sous muqueux. C´est une uropathie malformative rare surtout si elle survient sur un uretère simplex. Il s´agit d´une étude rétrospective menée sur dix ans, de 12 dossiers de malades colligés au Service de Chirurgie Pédiatrique de l´Hôpital Fattouma Bourguiba de Monastir entre 2006 et 2016. L´âge moyen de nos malades est de 2,7 ans avec des limites allant de 7 jours à 11 ans, le sex-ratio est de 1. Le tableau clinique a été dominé par la fièvre en rapport avec une infection urinaire haute. Le diagnostic a été posé essentiellement par l´échographie rénale et vésicale, l´urographie intraveineuse (UIV) et l´uréthro-cystographie rétrograde (UCR). L´urétérocèle était unilatéral dans 10 cas et bilatérale dans 2 cas soit un total de 14 cas d´urétérocèles. Tous ont été sur uretère simplex et ont été opérées par voie endoscopique. Aucun incident peropératoire n´a été noté. Les suites opératoires étaient simples. Une amélioration clinique et radiologique a été obtenue dans tous les cas. L´urétérocèle sur uretère simplex est une malformation urinaire très rare. Non diagnostiquée à temps, elle peut retentir sur le haut appareil. Le traitement endoscopique est une alternative séduisante avec des résultats satisfaisants.

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.

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.

Neonatal congenital pancreatic cyst is rare. It can be isolated or associated to other malformations. Early management is recommended because of possible severe complications. The aim of our work is to report our experience with three newborns with congenital pancreatic cyst. A five year (2010-2015) retrospective study is carried. Data were collected from medical files of three different patients. Diagnosis was prenatal in two patients. The Cyst was associated to Ivemark syndrome in two cases and solitary in one. Cystic complications were infection in one case and pancreatitis with liver cirrhosis in another case. These pancreatic cysts were successfully managed by surgery. One patient succumbed as a result of heart failure. To our knowledge we report the first case of congenital pancreatic cyst complicated with liver cirrhosis. We concluded that prompt diagnosis is important to prevent severe complications. Prognosis is dependent on early management and on life threatening associated malformations.