TAR syndrome with annular pancreas and anal atresia - a case report.

Abstract

TAR syndrome is manifested by the presence of hypomegakaryocytic thrombocytopenia and the bilateral absence of radii. An 8-day-old female newborn was referred to our clinic with diagnosis of TAR syndrome and symptoms of intestinal obstruction. On perineal examination she also had anal atresia with rectovestibular fistula. Because of persistent bile-stained drainage from the nasogastric tube, an upper gastrointestinal contrast study was performed and partial duodenal obstruction was detected. At operation, an annular pancreas was encountered and side-to-side duodenoduodenostomy was performed. A small number of gastrointestinal system malformations associated with TAR syndrome has been reported. Our case is unique; the association with annular pancreas and anal atresia with rectovestibular fistula has never been previously reported.