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Diagnosis of Hirschsprung's Disease by Immunostaining Rectal Suction Biopsies for Calretinin, S100 Protein and Protein Gene Product 9.5
Published on: April 26, 2019
Anorectal Malformations and Hirschsprung Disease: A 30-Year Retrospective Outreach Review
Patrick A Dewan1, Patrick J Mill2
1Paediatric Surgery, Sunshine Private Hospital, Melbourne, AUS.
Abstract:
Objective This study analyses the indications for and outcomes of primary and redo surgeries for anorectal malformations (ARMs) and Hirschsprung disease (HD) in developing, resource-limited countries. The study seeks to identify trends in primary surgery complication rates, evaluate the indications for reoperation and explore potential strategies to improve surgical management and long-term outcomes for children with ARM and HD in these settings. Methodology A retrospective cohort analysis was conducted on data collected by the Kind Cut for Kids (KCFK) surgical outreach program, a charitable initiative that provides paediatric surgical care in under-served regions. Data was collected over a 30-year period across 22 developing countries. The dataset contained 2,498 observations linked to ARM or HD, which was filtered to include those with primary surgeries, reoperations for prior complications and management of complications of any surgery done by the visiting team. Clinical data included demographics, pathology classification, surgical details, and postoperative outcomes. Results The final cohort included 496 ARM patients and 224 HD patients, with 65% and 41%, respectively, undergoing primary corrective surgeries. Among ARM cases, 25% required redo surgeries, with malposition (33%), strictures (24%), or prolapse (8%) being the most common indications. In HD, 45% of patients required redo procedures, primarily for strictures (19%), prolapse (9%), or acquired fistulas (4%). The most common redo procedures for ARMs were the posterior sagittal anorectoplasty (PSARP) (58%) or anorectal angle plication (10%). For HD patients, PSARP (11%) and the Swenson procedure (10%) were the most common corrective redo procedures. Due to the focus of KCFK visits, there are significant data gaps pertaining to primary surgical details, reoperation indications and follow-up data. This reflects the challenges of managing these conditions in resource-limited environments and with an outreach program. Conclusion This study highlights high rates of complications from primary surgeries in ARM and HD cases treated in resource-limited settings, which emphasises the need for enhanced surgical precision, structured postoperative care, and consistent follow-up protocols, as well as education of surgeons in the countries visited. Targeted interventions such as capacity-building initiatives, tailored consensus guidelines, and telemedicine integration are critical to addressing disparities in care. Future prospective studies with standardised data collection and outcome metrics are essential to validate these findings and improve care delivery for children with ARM and HD in underserved regions.
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