Timing of the definitive procedure and ileostomy closure for total colonic aganglionosis HD: Systematic review

Aim: To establish the cogency of recommendations for the appropriate age for pull-through and ileostomy closure in Total Colonic Aganglionosis-Hirschsprung Disease's (TCA-HD).

Method: Medline, PubMed, Cochrane, and the ClinicalKey databases were searched without date restriction. The studies that reported TCA-HD cases were evaluated for the number of cases, age at the definitive procedure, age at the ileostomy closure, reported complications, and the type of procedure. Perianal excoriation and diaper rash rates were analyzed using SPSS software, with p < 0.05 considered significant.

Results: Twenty-five studies mentioned TCA-HD findings between 1968 and 2019. The total number of patients who had definitive surgery was 218. Analysis showed no correlation between development of diaper rash and the age of the patient at the time of the definitive surgery or ileostomy closure. Studies scored between six and nine of nine possible stars on the NOS scoring system.

Conclusion: There is no correlation between age of surgery and postoperative diaper rash. Delaying the definitive procedure or ileostomy closure for TCA-HD has limited support on a review of current studies. The perianal excoriation/diaper rash is not reported in the literature at a high enough frequency to warrant keeping a diverting ileostomy until toilet trained of urine.

Type of study: Systematic review and meta-analysis. Levels of evidence IV.