ENTEROCOLITIS ASSOCIATED WITH HIRSCHSPRUNGS-DISEASE - A CLINICAL HISTOPATHOLOGICAL CORRELATIVE STUDY

Enterocolitis associated with Hirschsprung's disease (HEC) remains a m ajor source of morbidity and even mortality, both before and after def initive surgical treatment. This study was undertaken to investigate w hether histopathologic mucosal changes, in the absence of clinical man ifestations of HEC, could predict the subsequent development of this c omplication. The clinical data and histopathology of 25 patients who e ventually developed clinical HEC were compared with a control group of 25 age- and sex-matched patients with Hirschsprung's disease (HD) but with no clinical HEC either preoperatively or postoperatively. The hi stopathologic findings of tissue obtained by rectal biopsy or during l aparotomy were graded from 0 to V according to severity and compared w ith the eventual clinical course. This study showed that (1) histopath ologic findings of HEC appear to predict the clinical development, but not the severity, of future episodes of clinical HEC; (2) a histologi cal grade of greater than or equal to 11 (greater than or equal to 2 c rypt abscesses per high power field) should raise suspicion for subseq uent occurrence of HEC, whereas a grade of greater than or equal to II I (multiple crypt abscesses) places a child at high risk for developme nt of clinical HEC; and (3) contrary to what is generally thought, his topathologic changes of HEC occur in both ganglionic and aganglionic s egments. Based on these findings, it is recommended that histopatholog ic documentation of HEC and its grade, should be an integral part of t he tissue diagnosis of HD. Copyright (C) 1995 by W.B. Saunders Company