A HISTOLOGICAL GRADING SYSTEM FOR THE EVALUATION OF COEXISTING NID WITH HIRSCHSPRUNGS-DISEASE

The significance of dysplastic features in the surgical pullthrough se gment of bowel in patients with Hirschsprung's disease (HD) has not ye t been clarified. The aim of this study was to evaluate prospectively the ganglionated proximal bowel in 26 patients with HD (January 1988 t hrough January 1991). The significance of dysplastic features and thei r influence on post operative outcome were evaluated by means of a new ly devised histological scoring system based on the morphological feat ures. Functional outcome was assessed clinically at follow-up intervie w. Comparison was with control specimens from 22 patients undergoing u nrelated bowel surgery and a further 5 patients with neuronal intestin al dysplasia (NID). Results indicated a wide spectrum of histologicall y identified dysplastic features in patients with NID, the ganglionate d bowel of HD and controls. Although individual abnormal features were noted in the control group, significant degrees of dysplasia were abs ent. The overall degree of dysplasia was less striking than that obser ved in NID and in the 5 patients in whom NID co-existed with HD. Dyspl asia of the ENS in residual bowel could be correlated with postoperati ve dysfunction in 4 out of 5 patients (80 %) with HD and features of c o-existing NID. In addition, milder symptoms were noted in 50 % of pat ients having a borderline score (5-6/12). This study emphasizes the re lationship between clinical obstructive symptoms and a high degree of dysplasia within the ENS. A histological grading system is of value in evaluating the spectrum of abnormal findings and prospectively identi fying those with functional significance in patients with NID co-exist ing with HD.