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Published on: April 26, 2019
Residual aganglionosis after pull-through operation for Hirschsprung's disease: a systematic review and meta-analysis
Florian Friedmacher1, Prem Puri
1National Childrens's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland.
Insights
Residual aganglionosis (RA) and transition-zone bowel (TZB) cause persistent symptoms in one-third of Hirschsprung
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Histopathology
Background:
- Hirschsprung's disease (HD) often requires pull-through (PT) surgery for successful treatment.
- Persistent bowel symptoms post-PT operation necessitate further investigation and potential redo PT.
- Residual aganglionosis (RA) and transition-zone bowel (TZB) are key indications for redo PT in HD patients.
Purpose of the Study:
- To determine the incidence of residual aganglionosis (RA) and transition-zone bowel (TZB) in patients undergoing redo PT for HD.
- To evaluate the outcomes of redo PT operations in children with HD and persistent bowel symptoms.
Main Methods:
- A meta-analysis of published literature on redo PT operations for HD between 1985 and 2011.
- Detailed data extraction on patients with RA and TZB, including clinical presentation, histology, surgical procedures, and outcomes.
- Analysis of recurrent bowel problems, histological findings from repeat rectal biopsies, and types of redo PT operations performed.
Main Results:
- Out of 555 patients in 29 articles, 193 (34.8%) showed abnormal histology, with 144 diagnosed with RA and 49 with TZB.
- Common symptoms included persistent constipation (135 patients) and recurrent enterocolitis (45 patients).
- Following redo PT, 73.9% of 134 evaluated patients achieved normal bowel habits, while 16 experienced recurrent enterocolitis.
Conclusions:
- RA and TZB account for persistent bowel symptoms in approximately one-third of HD patients requiring redo PT.
- Redo PT operations generally yield satisfactory outcomes for most patients with HD.
- Repeat rectal biopsy is recommended for all patients experiencing recurrent bowel issues after initial PT surgery.
Purpose:
Most patients with Hirschsprung's disease (HD) have a satisfactory outcome after pull-through (PT) operation. However, some children continue to have persistent bowel symptoms after the initial operation and may require redo PT. Redo PT operation in HD is usually indicated for anastomotic strictures or residual aganglionosis (RA). We designed this meta-analysis to determine the incidence and outcome of RA among patients with HD following PT operation.
Methods:
A meta-analysis of redo PT operations for HD reported in the literature between 1985 and 2011 was performed. Detailed information was recorded in patients with RA and transition-zone bowel (TZB), including recurrent bowel problems, histological findings on repeat rectal biopsy, type of redo PT operation and outcome.
Results:
Twenty-nine articles reported 555 patients with redo PT operations. 193 (34.8%) patients demonstrated abnormal histological findings on rectal biopsy with 144 patients showing RA and 49 patients showing TZB. These 193 patients presented with persistent constipation (n = 135), recurrent enterocolitis (n = 45) and abnormal histology of the pulled-through bowel (n = 13). Mean age at redo PT was 4.4 years (range 4 months-17 years). Redo procedures were Duhamel (n = 57), transanal endorectal PT (n = 40), Soave (n = 35), Swenson (n = 10), posterior sagittal approach (n = 1) and not reported (n = 50). Follow-up information after redo PT was available in 134 (69.4%) patients and not available in 59 patients. Of the 134 patients, 99 (73.9%) patients had normal bowel habits, 19 patients had persistent constipation/soiling and 16 patients had recurrent enterocolitis.
Conclusion:
This meta-analysis reveals that RA and TZB are the underlying causes of persistent bowel symptoms in one-third of all patients with HD requiring redo PT operation. Most patients have a satisfactory outcome after redo operation. Rectal biopsy should be performed in all patients with recurrent bowel problems after PT operation.
