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      Pull-Through of Ganglionic Stoma in Children with Hirschsprung’s Disease Living in a Low Resource Context Spares Further Surgery for Colostomy Reversal: A Report from a Tanzanian Regional Referral Hospital

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          Abstract

          Background:

          One-step pull-through for Hirschsprung’s disease (HSCR) is already standard in many tertiary African paediatric surgical centres where radiology and frozen sections are available. However, limited resources in many peripheral areas associated with advanced age at referral or complicated presentation make recourse to colostomy often unavoidable with a consequent three-step surgery. To simplify these children’s clinical course and spare the burden of a third procedure for colostomy reverse, a direct pull-through of the proximal ganglionic stoma is proposed.

          Materials and Methods:

          Twenty children (male/female ratio 19/1) between 3 months and 13 years with a colostomy for a suspected HSCR were selected for stoma pull-through among patients admitted to a regional Tanzanian hospital between 2016 and 2022. Histological diagnosis was always confirmed by rectal biopsies and, in some cases, colonic specimens taken during the stoma confection or revision. A ganglionic stoma was always ascertained before the pull-through procedure (11 Transanal Endorectal, 4 Soave and 5 Duhamel).

          Results:

          One death was registered in a child with Down syndrome, unrelated to surgery, and another due to HSCR acute associated enterocolitis. One stenosis required stricturoplasty, and one anastomotic leakage needed temporary ileostomy. No relationship was found between complications and the procedure of choice. Passage of stools started on the 2 nd day and became regular and without soiling in a week.

          Conclusions:

          Straightforward pull-through of the ganglionic stoma may be a feasible solution for patients with HSCR and a diverting colostomy to avoid a three-step procedure. The correct position of the stoma at the transition zone must be ascertained to avoid the risk of including in the resection segment of the ganglionic bowel.

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          Most cited references15

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          Transanal endorectal pull-through for Hirschsprung's disease.

          A new endorectal pull-through technique using a transanal approach is presented in this report. Mucosectomy, colectomy, and pull-through are performed transanally, and neither laparotomy or laparoscopy are required. Five patients affected with Hirschsprung's disease have been operated on with this technique. During the 6- to 15-month follow-up period, all of them have had postoperative normal bowel movements.
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            Diagnosis of Hirschsprung's disease: a prospective, comparative accuracy study of common tests.

            To compare the diagnostic accuracy of contrast enema (CE), anorectal manometry (ARM), and rectal suction biopsy (RSB) for the detection of Hirschsprung's disease (HD). Following a prospective protocol, infants suspected of HD underwent all 3 index tests. Children with positive results on 2 or more index tests or who continued to have severe bowel problems underwent a full thickness biopsy as reference standard. Clinical follow-up was the reference standard in all other children. Between 2000 and 2003, 111 consecutive patients (67 boys; median age, 5.3 months) in whom HD was suspected were enrolled. HD was found in 28 patients. RSB had the highest sensitivity (93%) and specificity (100%) rates, but values were not significantly different from CE (sensitivity, 76%; specificity, 97%) or from ARM (sensitivity, 83%; specificity, 93%). Inconclusive test results occurred in 8 infants with CE, in 15 infants with ARM because of agitation, and in 2 infants with RSB. RSB is the most accurate test for diagnosing HD, and it has the lowest rate of inconclusive test results.
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              The contrast enema for Hirschsprung disease: predictors of a false-positive result.

              To examine predictors of a false-positive (FP) result on contrast enema (CE) for the diagnosis of Hirschsprung disease (HD). Retrospective analysis, over a 5-year period (1999-2004), of infants (<6 months of age) with suspected HD undergoing rectal biopsy following abnormalities identified on CE (transition zone [TZ], abnormal rectosigmoid ratio, microcolon, retained contrast, or mucosal irregularity). One hundred twenty-nine patients underwent rectal biopsy following an abnormal CE. The FP rate was 48.5% (66 with HD). Age below 30 days (OR, 3.4; 95% CI, 1.1-10.3), female sex (OR, 3.4; 95% CI, 1.6-7.3), and absence of TZ (OR, 6.3; 95% CI, 2.6-15.3) were independently associated with an increased risk for FP on multiple variable logistic regression. A history of bilious emesis decreased the probability of FP (OR, 0.2; 95% CI, 0.06-0.5). Transition zone, sex, age, and bilious emesis are important predictors of FP in those with suspected HD and CE abnormalities. With 100% incidence of FP, infants younger than 30 days with neither bilious emesis nor a TZ and female infants younger than 30 days with these features may represent a subpopulation in whom rectal biopsy can be avoided.
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                Author and article information

                Journal
                Afr J Paediatr Surg
                Afr J Paediatr Surg
                AJPS
                Afr J Paediatr Surg
                African Journal of Paediatric Surgery: AJPS
                Wolters Kluwer - Medknow (India )
                0189-6725
                0974-5998
                Oct-Dec 2023
                19 January 2023
                : 20
                : 4
                : 302-305
                Affiliations
                [1]Department of Surgery, Consolata Hospital Ikonda, Njombe, Tanzania
                [1 ]Department of Pediatric Surgery, San Camillo-Forlanini Hospital, Rome, Italy
                Author notes
                Address for correspondence: Prof. Alessandro Calisti, Via Euclide Turba, 4, 00195 Rome, Italy. E-mail: a.calisti@ 123456tiscali.it
                Article
                AJPS-20-302
                10.4103/ajps.ajps_57_22
                10756406
                9a48494b-967e-4701-812b-d3a269e42c73
                Copyright: © 2023 African Journal of Paediatric Surgery

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                : 04 May 2022
                : 27 July 2022
                : 21 October 2022
                Categories
                Original Article

                children,hirschsprung’s diseases,low resources
                children, hirschsprung’s diseases, low resources

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