IS HISTOLOGICAL DIAGNOSIS OF NEURONAL INTESTINAL DYSPLASIA RELATED TOCLINICAL AND MANOMETRIC FINDINGS IN CONSTIPATED CHILDREN - RESULTS OFA PILOT-STUDY

Neuronal intestinal dysplasia (NID) of the colon has been reported in adults and children with chronic constipation. However, it is unknown whether these histological abnormalities are related to the severity o f symptomatology or the findings of anorectal manometry. We studied 57 children (2 weeks to 17 years old, 33 boys) who had chronic constipat ion and/or soiling or obstructive symptoms early in life and evaluated anamnestic data, symptoms, outcome after 6 months of conventional the rapy, rectal biopsies for signs of dysganglionosis, and results of ano rectal manometry. In cooperative patients, defectaion dynamics were re corded during manometry with simultaneous surface electromyogram of th e external anal sphincter. In 30 older patients, severity of constipat ion was assessed by measurement of colonic transit time with radiopaqu e markers. Histology confirmed Hirschsprung's disease in nine (exclude d from further analysis), hyperganglionosis of the plexus submucosus ( classic NID) in six, heterotopic ganglion cells without hypergangliono sis (abortive NID) in 18, and no signs of dysganglionosis in 24 patien ts. The rectoanal inhibitory reflex was more often absent or abnormal in children with classic NID (six of six) and abortive NID (11 of 17) compared with children with normal histology (four of 22) (p < 0.001). All other manometric parameters analyzed, colonic transit times, and clinical outcomes were not related to histological diagnosis. In contr ast, the severity of constipation and outcome was significantly worse in children with abnormal defecation dynamics. These results suggest t hat histological signs of NID in the submucous plexus are of uncertain value in assessing the clinical picture and should not influence deci sions concerning further treatment, especially surgical interventions.