Difficulties in the Diagnostics and Treatment of Near-Total Congenital Megacolon
L. Topor, A. Ulici, D. Mălureanu, I. Stoica, A. MogaClinical case, no. 5, 2014
Near total colonic aganglionosis is one of the rarest forms of Hirschsprung's disease and until recent years it has beenconsidered deadly. Establishing a correct diagnosis has provento be challenging, because while the clinical and radiologicalfeatures can be useful, they are not pathognomonic. Chronicintestinal obstruction and long-term parenteral nutritiondependency are associated with a high mortality risk for thesepatients. While there is no current consensus with regards to asuperior operative method, the patients benefit from surgicaltechniques aimed at lengthening the intestine, as well as fromintestine transplant. We report the case of a newborn baby girl who was admitted to our clinic for abdominal distension,biliary and fecaloid vomiting. With an initial suspicion ofdigestive tract malformation, the diagnosis of near totalcongenital megacolon was established with great difficulty andthe infant underwent serial surgeries, ending up with anextended myotomy-myectomy (Ziegler's procedure) as a curativeapproach, with favorable immediate postoperative evolution.However, the patient developed sepsis and although the infectionwas treated accordingly, the baby's general condition keptdeteriorating and exitus was recorded 77 days after admission.
