Hirschsprung disease managed with one-stage transanal endorectal pullthrough in a low-resource setting without frozen section

Abstract

Background Over the past few decades, surgery for Hirschsprung’s disease (HD) has evolved into a minimally invasive, single-stage procedure with excellent outcomes. Intraoperative frozen section biopsy is critical for this procedure to avoid the potential risk of leaving a retained aganglionic segment. However, this facility is not available in most low-income countries. Therefore, a two-stage procedure with an initial colostomy is still practiced in the developing world. We aimed to evaluate the outcome of single-stage transanal pullthrough performed in a facility without frozen section biopsy. Methods A retrospective review of all patients who underwent transanal pullthrough in two teaching hospitals over a 6-year period (2015–2020). Results Forty-seven children underwent transanal endorectal pullthrough (TERPT). Age at surgery ranged from 2 months to 6 years and mean weight was 8.7 kg. Barium enema did not show transition zone in 6 patients (12.8%) while others demonstrated short segment HD. Intraoperatively, the transition zone was visualized in 40 patients (85%). TERPT alone was performed in 35 (74.5%), TERPT with laparotomy to visualize transition zone in 9 (26.7%) and TERPT with transabdominal mobilization was required in 3 (6.4%). Definitive histopathologic examination revealed aganglionic segment pullthrough in 4 (8.5%) and transitional zone pullthrough in another 4 (8.5%). However, with long term follow up all eight children remained asymptomatic and no intervention was required. Conclusions Transanal pullthrough offers reduced number of surgeries and faster recovery. We have also observed a good functional outcome despite a discrepancy with pathology results. Overall, our data suggests it is a safe and viable option for the treatment of short segment HD in facilities where frozen section is not available.

Diagnostic accuracy of barium enema versus full‐thickness rectal biopsy in children with clinically suspected Hirschsprung's disease: A comparative cross‐sectional study

“…However, the study provided solid evidence that CE has good test accuracy in children and can be used to inform definitive management when pathology services are unavailable. In addition, there are successful stories of surgical treatment of HSD without biopsy, such as endoanal pullthrough performed without a frozen section 10 …”

“…These are the largest centers affiliated with Addis Ababa University, Ethiopia. They have a high burden of pediatric surgical cases, with more than 2000 procedures being performed yearly 10 . All children with clinically suspected HSD in the specified time period were included in the study.…”

“…4 Pathology, especially fresh-frozen biopsy interpretation, is unavailable even in referral centers. 6,10 Thus, relying on relatively simple diagnostic tools with improved accuracy, mainly BE, remains a promising alternative.…”

“…4,6 Although rectal suction biopsy is the preferred method for confirming diagnosis, full-thickness rectal biopsy (FTRB) is performed on patients of all age groups in LMICs due to the unavailability of the former. 10 Many centers in LMICs lack the expertise and infrastructure needed for timely diagnosis of HSD patients. 5 The situation is further exacerbated by the insufficient knowledge of the patients' relatives and the limited availability of primary healthcare services.…”

“…However, the lack of pediatric surgeons and pediatric anesthesia, which is limited to tertiary teaching hospitals, and the backlog of patients on the waiting list for a definitive surgical diagnosis or management represent major obstacles 4 . Pathology, especially fresh‐frozen biopsy interpretation, is unavailable even in referral centers 6,10 . Thus, relying on relatively simple diagnostic tools with improved accuracy, mainly BE, remains a promising alternative.…”