Hirschsprung's disease and intestinal neuronal dysplasia--a frequent association with implications for the postoperative course.

Between 1991 and 1993, 106 newly diagnosed cases of Hirschsprung's disease (HD) were prospectively analyzed for the association of HD and intestinal neuronal dysplasia (IND) at ten pediatric surgical departments in central Europe. Hirschsprung-associated IND (HaIND) was found in 40% of cases. IND was disseminated in one-third and localized in two-thirds of the patients. Initial clinical symptoms were related to the length of the aganglionic segment, but not to the presence of HaIND. An enterostomy performed in 72 cases (67.9%) was located in a segment of pathologically innervated bowel in 50% of all cases, but in 72% of cases of HaIND. The proximal margin of the resected bowel showed pathological innervation in 44% of cases. Supplemental biopsies from the intestine (apart from diagnostic suction biopsies and biopsies at the enterostomy site) led to the first identification or definition of length of associated IND in 17.9% of cases. Postoperatively, the presence of long-segment aganglionosis or associated IND implied a delay in the restoration or normal defecation. Persistent constipation was found in 40% of patients with associated disseminated IND at follow-up at 6 months, compared to 20.6% in patients with isolated HD. These children needed secondary interventions more often than patients with associated localized IND or isolated HD. HaIND thus has clinical implications for the postoperative course if IND is disseminated.

[Malignant melanoma in the child--2 case reports]. / Malignes Melanom beim Kind--2 Fallberichte.

Case histories of two infants with malignant melanoma are presented. Both tumors had developed from a congenital mole to advanced disease with lymph node metastasis at time of diagnosis, findings which are commonly correlated with poor outcome. Initially, both patients were surgically treated. From the experience with few described cases of melanoma in childhood and with a large number of adult patients, neither chemotherapy and conventional BCG immunotherapy nor irradiation seem to be an effective treatment and are accompanied by many adverse side effects. Therefore in one patient therapy was limited to surgery alone. In the other child it was followed by treatment with high-dose recombinant alpha-2-interferon (1 Mill. i.u./Kg X d) plus an H2-receptor antagonist. Unexpectedly this girl developed neurological side effects, characterized by spastic paraparesis, indicating damage to the first motoneuron. All symptoms completely resolved after discontinuation of drugs within three months. Both children have been in complete remission for 18+ and 32+ months, respectively.