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Outcomes of surgical management of total colonic aganglionosis

Cited 6 time in Web of Science Cited 7 time in Scopus
Authors
Choe, E. K.; Moon, S. B.; Kim, H. Y.; Lee, S. C.; Park, K. W.; Jung, S. E.
Issue Date
2007-11-09
Publisher
Springer Verlag
Citation
World J Surg. 2008 ;32(1):62-8.
Keywords
Body WeightChildChild, PreschoolDigestive System Surgical Procedures/*methodsFemaleHirschsprung Disease/*surgeryHumansInfantInfant, NewbornMaleReoperationTreatment Outcome
Abstract
PURPOSE: The purpose of this study was to assess the long-term clinical outcomes and bowel function of patients with total colonic aganglionosis (TCA) after surgery. METHODS: The hospital records of 17 TCA patients treated surgically during 1985 to 2004 were reviewed. Long-term follow-up was done by telephone interviews with the parents. RESULTS: Primary enterostomy was performed in 13 (76%) patients. In three (17%) patients, TCA was not suspected initially. They underwent conservative surgery primarily, which required a second operation soon after. One had transverse colectomy with ileostomy. By pathologic review, nine (53%) patients had small bowel involvement of aganglionosis. Six (35%) patients died before corrective surgery. They all had extensive small bowel involvement. Among 11 patients who had a corrective operation, 10 were treated with Martin's procedure. Long-term (mean 74 months) follow-up was available in seven patients, and the mean weight-for-age percentiles was 27.1% (range 5-50%), the frequency of defecation was three to five times a day in four patients (57%), one or two times a day in two patients (28%), and more than five times a day in one patient (15%). CONCLUSIONS: TCA is difficult to diagnose; but once it is diagnosed correctly and treated by corrective surgery, outcomes seem promising. Martin's operation brought about a good outcome and enabled patients to have acceptable bowel habits. The prognosis is highly dependent on the extent of aganglionosis.
ISSN
0364-2313 (Print)
Language
English
URI
https://hdl.handle.net/10371/62445
DOI
https://doi.org/10.1007/s00268-007-9270-5
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Appears in Collections:
College of Medicine/School of Medicine (의과대학/대학원)Surgery (외과학전공)Journal Papers (저널논문_외과학전공)
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