Neuronal intestinal malformations: a retro- and prospective study on 203 patients

A total of 203 patients with neuronal intestinal malformations were analyzed. A retrospective study was performed on 122 patients who had been treated from 1963-1988 and 119 (97.5%) of these patients underwent follow-up examination after a mean of 11.5 years. Subsequently 81 patients treated from 1989-1993 were included in a prospective trial. These patients were investigated preoperatively by standardized questionnaires, x-ray examination, electromanometry, transit time studies, and underwent follow-up after a mean of 3.2 years. All biopsy specimens of the prospective trial were analyzed by the Institute for Pathology of the University of Basel. Before 1989 the incidence of neuronal intestinal malformations was 4.9 per year as compared to 18 per year from 1989-1993. The percentage of classical aganglionosis decreased from 77.9% to 35.8% and aganglionosis associated with NID B was increased from 9% to 29.6% (p < 0.001). The prospective trial showed that only 54.7% of 53 children with aganglionosis had classical Hirschsprung's disease, 45.3% were combined with NID B. Out of 37 patients with NID 64.9% had associated aganglionosis. Preoperative symptoms showed no pathognomonic criteria for any specific neuronal intestinal disorder. However, 75% of the patients with aganglionosis combined with NID suffered from ileus as compared to 41.1% of the patients with classical aganglionosis and 23.1% of patients with isolated NID (p < 0.05). This indicates an additive effect of both lesions. X-ray examinations, electromanometry, and transit time studies did not show pathognomonic criteria for specific neuronal intestinal malformations.(ABSTRACT TRUNCATED AT 250 WORDS)