Abstract
PurposeHirschsprung's associated enterocolitis (HAEC) is a complication of Hirschsprung's Disease (HD) with considerable morbidity and mortality. The variability in presentation leads to a wide variety of the reported prevalence pre-and postoperatively. This systematic review aimed to clarify the prevalence of HAEC in short—(S-HD), long (L-HD), TCA and the type of operation used.MethodsA systematic literature-based search for relevant cohorts was performed using Pubmed/Medline, Cochrane Library from its inception to May 2021. Studies reporting on pre-and postoperative enterocolitis, segment length, and surgical procedure (Soave, Swenson, Duhamel) were included. Pooled prevalence and subgroup analysis have been calculated for pre-and postoperative HAEC.Results4738 articles were identified from the literature search, among which 57 studies, including 9744 preoperative and 8568 postoperative patients, were included. The groups were sorted by length of the aganglionic segment for further analysis. The pooled prevalence for preoperative HAEC was 18.3% for all types, 15.2% for S-HD and 26.1% for TCA. The pooled prevalence for postoperative HAEC was in total 18.2% for all segment lengths and used techniques. Subgroup analysis showed no significant difference in the occurrence of postoperative enterocolitis between the three techniques.ConclusionThe prevalence of preoperative HAEC increases with segment length. However, pooled data suggest that the postoperative risk for developing HAEC, independently of the employed method and segment length, is comparable to the preoperative risk.
Highlights
Hirschsprung's Disease (HD) is a developmental disorder of the enteric nervous system (ENS) with an estimated incidence of 1:5000 newborns
Five studies reported on the pre-and postoperative number of patients with Hirschsprung's associated enterocolitis (HAEC) depending on Trisomy 21
Thirtynine patients were suffering from both Morbus Hirschsprung and Trisomy 21, of which 12 showed preoperative HAEC (30.8%)
Summary
Hirschsprung's Disease (HD) is a developmental disorder of the enteric nervous system (ENS) with an estimated incidence of 1:5000 newborns. The reported incidence of HAEC varies significantly in published series and ranges from 17 to 50%. The incidence of postoperative colitis has been reported to occur in 2–35% of patients [3]. Since HAEC occurs postoperatively in a significantly high percentage, several studies have addressed the type of operation (Soave, Swenson, and Duhamel) as a single risk factor for HAEC, with conflicting results [9,10,11]. Because of the wide variety of the reported prevalence pre-and postoperatively, this systematic review aimed to clarify the prevalence of HAEC in short—(S-HD), long (L-HD) and TCA, and the type of operation used. The study's findings will help to clarify conflicting results reported in the current literature regarding the prevalence of HAEC in HD. The exact prevalence will help facilitate the design of evidence-based preoperative management and follow-up and better care for patients with Hirschsprung's Disease
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