Ea. Elhalaby et al., ENTEROCOLITIS ASSOCIATED WITH HIRSCHSPRUNGS-DISEASE - A CLINICAL RADIOLOGICAL CHARACTERIZATION BASED ON 168 PATIENTS, Journal of pediatric surgery, 30(1), 1995, pp. 76-83
The enterocolitis associated with Hirschsprung's disease (HD) has not
been clearly characterized. This study was undertaken to analyze the c
linical and radiological findings of Hirschsprung's enterocolitis (HEC
) in 168 patients treated from July 1974 through October 1992. HEC occ
urred in 57 patients (33.9%), either preoperatively (13; 7.7%) or post
operatively (36; 21.4%). In eight patients (4.8%), it occurred pre- an
d postoperatively. The number of bouts of HEC per patient ranged from
one to six (mean, 2.2). The major presenting features were abdominal d
istension (83%), explosive diarrhea (69%), vomiting (51%), fever (34%)
, lethargy (27%), rectal bleeding (5%), and colonic perforation (2.5%)
. There were no deaths directly related to HEC. The analysis of 150 pl
ain x-rays of the abdomen, taken at the onset of HEC or in between bou
ts, showed that colonic dilatation was the most sensitive radiological
finding (90% sensitivity), but it had poor specificity (24%). However
, an intestinal cutoff sign (gaseous intestinal distension with abrupt
cutoff at the level of the pelvic brim) was both sensitive (74%) and
specific (86%) for HEC. Barium enema was of limited value in the diagn
osis of HEC bouts because most of the radiographic findings persisted
for prolonged periods after cessation of such bouts. The authors concl
ude that (1) HEC can be characterized as abdominal distension and expl
osive diarrhea associated with the intestinal cutoff sign and (2) the
occurrence of explosive diarrhea in any patient with HD is suggestive
of HEC, even in the absence of systemic symptoms, and should be treate
d to avoid the morbidity and potential mortality of HEC. Copyright (C)
1995 by W.B. Saunders Company