The superior mesenteric artery syndrome (SMAS) is an uncommon condition in children. We describe a case of a 7-year-old boy with SMAS that occurred 3 years after a Deloyers' procedure for subtotal colonic Hirschsprung who was admitted for bilious vomit, abdominal pain and diarrhea due to unrecognized celiac disease. This case emphasize that SMAS in children needs a close medical and surgical fo...

MWS is a multiple congenital anomaly syndrome, first clinically delineated by Mowat et al in 1998. Over 45 cases have now been reported. All patients have typical dysmorphic features in association with severe intellectual disability, and nearly all have microcephaly and seizures. Congenital anomalies, including Hirschsprung disease (HSCR), congenital heart disease, hypospadias, genitourinary a...

Total colonic aganglionosis (TCA) is a relatively uncommon form of Hirschsprung disease (HSCR), occurring in approximately 2%-13% of cases. It can probably be classified as TCA (defined as aganglionosis extending from the anus to at least the ileocecal valve, but not >50 cm proximal to the ileocecal valve) and total colonic and small bowel aganglionosis, which may involve a very long segment of...