Bowel function after transanal endorectal pull-through for Hirschsprung disease - does outcome improve over time?

BACKGROUND: The reported functional outcome in patients operated with transanal endorectal pull-through (ERPT) for Hirschsprung disease (HD) varies greatly. Some studies suggest better functional outcome in older than in younger HD patients, but there are almost no longitudinal studies that have demonstrated such improvement. Therefore, we aimed to compare functional outcome in a cohort of HD patients over time to assess whether bowel function improves with increasing age. METHODS: Functional outcome in HD patients operated with ERPT from 1998 to 2007 was recorded by standardized interviews by an independent investigator during 2008-2011 and again in 2017/2018. Bowel function was assessed using the Krickenbeck questionnaire. Clinical data were collected prospectively. RESULTS: 50 of the original 62 patients responded for a second interview. Median age at the two interviews was 8,1 (3,4-16,6) and 15,4 (9,9-25) years respectively. There was no difference in the rate of soiling at first (52%) and second (52%) follow-up. Constipation was reported in 20% of the patients at first, and in 24% at second follow-up. Bowel management was used by 30% and 32% at first and second interview respectively. CONCLUSION: Soiling and constipation are common in HD patients several years after surgery, and no improvement of bowel function with increasing age could be demonstrated. LEVEL OF EVIDENCE: Level II.

A Nordic multicenter survey of long-term bowel function after transanal endorectal pull-through in 200 patients with rectosigmoid Hirschsprung disease.

OBJECTIVE: Transanal endorectal pull-through (ERPT) is the most popular technique to treat Hirschsprung disease (HD). Still, there is limited knowledge on long-term bowel function. This cross-sectional, multicenter study assessed long-term bowel function in a large HD population and examined predictors of poor outcome. METHODS: Patients older than four years or their parents filled out a validated questionnaire on bowel function. Clinical details were recorded retrospectively from medical records. RESULTS: 73/200 (37%) patients reported absolutely no impaired bowel function, meaning no constipation, fecal accidents, stoma, appendicostomy or need for enemas. Seven (4%) had a stoma, and 33 (17%) used antegrade or rectal colonic enemas. Most disarrangements of fecal control and constipation were significantly less common in older age group, but abnormal defecation frequency and social problems remained unchanged. Syndromic patients (n=31) experienced frequent fecal accidents (46%) more often than nonsyndromic (14%, P<0.001). Having a syndrome (adjusted OR 5.6, 95% CI 2.1-15, P=0.001) or a complete transanal ERPT (adjusted OR 2.4, 95% CI 1.1-5.7, P=0.038) was significantly associated with poor outcome defined as having a stoma, an appendicostomy, daily fecal accidents or need of regular rectal wash outs. CONCLUSION: A significant number of HD patients experience bowel problems many years after definite surgery. Fecal control was significantly better in older than younger HD patients, but some continued to have considerable bowel problems also as adults. A total transanal ERPT was associated with poorer outcome. Long-term follow-up of HD patients is warranted. Prognosis Study: Level II.

Anal endosonography and bowel function in patients undergoing different types of endorectal pull-through procedures for Hirschsprung disease.

BACKGROUND: The reasons for fecal incontinence after surgery for Hirschsprung disease (HD) remain unclear. The aim of this study was to examine the anal sphincters by anal endosonography and manometry after transanal endorectal pull-through, with or without laparotomy or laparoscopy, in HD patients. Furthermore, we aimed to correlate these findings to bowel function. PATIENTS AND METHODS: Fifty-two HD patients were followed after endorectal pull-through. Anal endosonography and manometry were performed without sedation at the age of 3 to 16 years. RESULTS: Endosonographic internal anal sphincter (IAS) defects were found in 24/50 patients, more frequently after transanal than transabdominal procedures (69 vs. 19%, p=0.001). In a multiple variable logistic regression model, operative approach was the only significant predictor for IAS defects. Anal resting pressure (median 40mm Hg, range 15-120) was not correlated to presence of IAS defects. Daily fecal incontinence occurred more often in patients with IAS defects (54 vs. 25%, p=0.03). CONCLUSIONS: Postoperative IAS defects were frequently detected and were associated with daily fecal incontinence. IAS defects occurred more often after solely transanal procedures. We propose that these surgical approaches are compared in a randomized controlled trial before solely transanal endorectal pull-through is performed as a routine procedure.