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Published on: April 26, 2019
Outcomes of Transition Zone Pull-Through in Hirschsprung Disease: A Systematic Review
Simran Sehdev1, Joshua Cave1, Joe Curry1
1Great Ormond Street Hospital for Children, Great Ormond Street, London WC1N 3JH.
Aim:
Transition zone pull-through (TZPT) can be associated with morbidity and the need for redo pull-through (RDPT), but not in all patients. We aimed to systematically review outcomes following TZPT in patients with Hirschsprung disease (HD).
Methods:
A PICO-based systematic search of Embase and Medline was conducted in accordance with PRISMA guidelines. Search terms included variations of: Hirschsprung disease, pull-through (PT), transition zone, and complications. Eligible studies included cohorts of PT with TZ on final histology and reporting functional outcomes, Hirschsprung-associated enterocolitis (HAEC), or reoperation. Cohorts defined solely by redo surgery or postoperative complications were excluded. Risk of bias was assessed for confounding, participant selection, outcome measurement, and missing data.
Results:
The search from 2000-2026 yielded 439 abstracts. Following screening, 47 articles underwent full-text review and six met the inclusion criteria. Overall, the level of evidence was low to moderate. TZPT occurred in 13.4% (one in seven) of consecutive pull-throughs. Earlier cohorts reported higher rates of constipation, obstructive symptoms, HAEC, and redo surgery of up to 54%, as well as stoma formation in up to 38%, both as a bridge to redo surgery and as a permanent strategy. In contrast, more recent contemporary cohorts, some with matched data, suggest that conservative management of patients with TZPT can achieve comparable long-term bowel function and HAEC rates to non-TZPT patients, with redo surgery reserved for refractory cases. Notably, none of these contemporary cohorts reported stoma utilisation.
Conclusion:
Outcomes following TZPT are variable and were historically associated with substantial morbidity. More recent studies suggest potentially similar long-term results compared with ganglionic pull-through, including quality of life. Routine redo surgery for TZPT without comprehensive clinical assessment cannot be recommended. Large dataset outcome reporting is required to better define the true long-term impact of TZPT.