[A case of Hirschsprung's disease associated with Laurence-Moon-Bardet-Biedl syndrome].
T. MaedaKeinosuke OkazakiMasahito TachibanaYuichiro SakamotoHiroshi SakaedaY. YamamotoKei ItoYoh Watanabe
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Hirschsprung's disease
Bardet–Biedl Syndrome
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Thirty-six patients with Hirschsprung's disease underwent surgery using modified Duhamel operation with the GIA (gastrointestinal autosuture) stapler over a 12-year period at our institution. We now present our experience from the perspective of postoperative complications and anorectal function. Postoperative complications included minimal anal bleeding due to the CIA stapler in 2 patients, and mild enterocolitis in 6 patients. Severe complications such as anastomotic leakage or stricture were not observed in our series. Overall, postoperative anorectal function was good in patients without Down's syndrome or cerebral palsy. Follow-up of our patients showed that a good quality of life was achieved during childhood. We conclude, therefore, that the modified Duhamel operation, with the use of the CIA stapler, is a safe and easy procedure for correction of Hirschsprung's disease.
Hirschsprung's disease
Enterocolitis
Gastrointestinal function
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Introduction: The aim of this study was to test the hypothesis that the early functional outcome for patients with rectosigmoid Hirschsprung's disease (HD) is comparable for the Duhamel pull-through procedure and the transanal endorectal pull-through (TERPT) procedure, with less discomfort for the patient postoperatively after the TERPT technique.
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ObjectiveThe aim of the study was to assess the late postoperative complications after single-stage transanal endorectal pull-through for Hirschsprung's disease.BackgroundHirschsprung's disease is a common cause of bowel obstruction during the newborn period. One-stage surgery for Hirschsprung's disease is well established, and the results are comparable or better than multistage surgery.Patients and methodsThis was a retrospective study conducted at the Pediatric Surgery Unit of Department of General Surgery, Menoufia University Hospital and Abu-El Reesh Insurance Hospital between October 2007 and November 2011. This study included 20 patients with Hirschsprung's disease; there were 15 boys and five girls, their ages ranging from 6 months to 5 years. All these patients were subjected to transanal pull-through, and their data were retrieved by checking their files.ResultsThe mean age of these patients was 32.1 ± 17.47 months. Rectal biopsy was diagnostic in all patients, whereas barium enema was diagnostic only in 16 patients. The mean operative time was 91.3 ± 11.89 min. The commonest postoperative complication was enterocolitis (30%); two patients (10%) were in need for frequent dilatation, three patients (15%) complained of rectal prolapse, two other patients (10%) complained of increased stool frequency, and finally four patients (20%) complained of recurring constipation.ConclusionTransanal pull-through is both feasible and safe for the management of patients with Hirschsprung's disease, and it is associated with acceptable morbidity.
Enterocolitis
Barium enema
Hirschsprung's disease
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Hirschsprung's disease
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From the Pathological Laboratory of the Medical Department of the University of Louisville and the Louisville City Hospital, LOUISVILLE, KY.
Hirschsprung's disease
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At the Department of Paediatric surgery in Stockholm the choice of operative method for Hirschsprung's disease has since 1975 been endorectal pull-through. In 1985 some modifications were introduced, i.e. anal approach of the mucosal dissection, resection of the internal anal sphincter, total parenteral nutrition and fewer colostomies. Thirteen children treated accordingly have been compared to children treated before 1985. It was found that the children gained normal bowel habits earlier and had fewer postoperative problems than the children operated before introduction of the technical modifications.
Hirschsprung's disease
Anal sphincter
Pediatric Surgeon
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Megacolon
Hirschsprung's disease
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Hirschsprung's disease
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Ten years experience with Rehbein's procedure in the surgical treatment of Hirschsprung's disease (23 patients) is reported. Eleven patients were operated with a covering colostomy. Clinically overt anastomotic dehiscence was seen in 6/23 patients, being more common in the patients without a colostomy (5/12). Five of the patients with anastomotic dehiscence recovered completely after surgical intervention (4) or conservative treatment (1), whereas one patient ended up with incontinence. One patient died in enterocolitis apparently without relation to the surgical treatment. Early postoperative constipation and soiling were common, whereas at late follow-up constipation was reported only in one patient. At late follow up barium enemas showed a normal colorectal calibre in most patients. Anorectal manometry (n = 8) showed absence of normal rectoanal inhibition and rectal volumes slightly larger than among controls.
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