Difficulties in the diagnostics and treatment of near-total congenital megacolon

Near total colonic aganglionosis is one of the rarest forms of Hirschsprung's disease and until recent years it has been considered deadly. Establishing a correct diagnosis has proven to be challenging, because while the clinical and radiological features can be useful, they are not pathognomon...

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Veröffentlicht in:Chirurgia (Bucharest, Romania : 1990) Romania : 1990), 2014-09, Vol.109 (5), p.701-704
Hauptverfasser: Topor, L, Ulici, A, Mălureanu, D, Stoica, I, Moga, A
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container_start_page 701
container_title Chirurgia (Bucharest, Romania : 1990)
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creator Topor, L
Ulici, A
Mălureanu, D
Stoica, I
Moga, A
description Near total colonic aganglionosis is one of the rarest forms of Hirschsprung's disease and until recent years it has been considered deadly. Establishing a correct diagnosis has proven to be challenging, because while the clinical and radiological features can be useful, they are not pathognomonic. Chronic intestinal obstruction and long-term parenteral nutrition dependency are associated with a high mortality risk for these patients. While there is no current consensus with regards to a superior operative method, the patients benefit from surgical techniques aimed at lengthening the intestine, as well as from intestine 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 of digestive tract malformation, the diagnosis of near total congenital megacolon was established with great difficulty and the infant underwent serial surgeries, ending up with an extended myotomy-myectomy (Ziegler's procedure) as a curative approach, with favorable immediate postoperative evolution.However, the patient developed sepsis and although the infection was treated accordingly, the baby's general condition kept deteriorating and exitus was recorded 77 days after admission.
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source MEDLINE; EZB-FREE-00999 freely available EZB journals; Alma/SFX Local Collection
subjects Anti-Bacterial Agents - therapeutic use
Bacteremia - drug therapy
Bacteremia - microbiology
Chronic Disease
Fatal Outcome
Female
Hirschsprung Disease - complications
Hirschsprung Disease - diagnosis
Hirschsprung Disease - surgery
Humans
Ileostomy
Infant, Newborn
Intestinal Obstruction - etiology
Intestinal Obstruction - surgery
Jejunostomy
Parenteral Nutrition - methods
title Difficulties in the diagnostics and treatment of near-total congenital megacolon
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