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Adult
Hirschsprung's Disease: What is the Best Management?
Khalid A. Ismail,
Mohamed H. Mazhar Ashour, Osama. H. El Khadrawy, Hamdy Abdel Hady,
Ali M. Turky, Mohammed F. Metwally, & Amal A. Hashish
Department of Surgery, Tanta University Hospital, Tanta, Egypt
Tanta Med. Sc. J 2009; 4(3):113-118
ICID: 907316
Article type: Case reportIC™ Value: 2.72
| Abstract provided by Publisher |
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Background/Aim: Although rare, adult Hirschsprung's disease (HD)
should be suspected in patients who have lifelong constipation. The
purpose of this study was to find out the best management strategy
for HD at this specific age group. Patients & Methods: This study
included 15 adult patients (9 males and 6 females) with proven
tissue diagnosis of HD. These patients were treated, between January
1995 and December 2008, at Tanta University Hospital and affiliated
hospitals. The clinical data, diagnostic tools, details of surgical
management and outcome were analyzed. The diagnosis was confirmed by
barium enema and full-thickness rectal biopsy in all patients. Seven
patients had anorectal manometry. Different surgical techniques were
used either as one or 2 stage. Results: The patients' age ranged
from 16-48 years (mean 28±3 years). All patients presented with
chronic constipation requiring enemas, cathartics and multiple
hospital admissions for management. The aganglionic segments were
confined to the rectum in 6 patients and included the distal part of
sigmoid in nine. The definitive procedures were; primary Soave
endorectal pull through (ERPT) (4 patients), two-stage Soave ERPT (6
patients), primary transanal ERPT (3 patients) and Duhamel
retrorectal pull through after a failed myectomy (2 patients).
Anastomotic leak occurred in 2 patients (13.33%), anal stricture in
one patient (6.66%), cuff abscess in one patient (6.66%) and two
patients (13.33%) suffered from partial incontinence that improved
by conservative treatment. Conclusion: Although rare, adult
Hirschsprung's disease must always be considered whenever a patient
complains of severe chronic constipation. One stage ERPT either
conventional transabdominal or transanal approach is feasible after
prolonged bowel preparation but a two stage approach is technically
easier and safer.
ICID 907316
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