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.

Although its protective effect is contested and the risk of contracting tuberculosis is rather low nowadays, BCG vaccination is frequently performed. Changes of strain repeatedly led to an increased complication rate. In Austria between 1990 and 1991, of 3386 newborn babies (Strain Pasteur) 116 developed lymphadenitis 3 to 28 weeks after vaccination. The affected children received four types of treatment: nothing specific, isoniazid, or surgery with and without isoniazid. Surgical treatment was found to be necessary in 96 cases. Bacilli were successfully grown in culture in 46% of cases up to week 20 after vaccination; but later than 20 weeks no culture became positive. All cultured bacteria were isoniazid-sensitive. From our data we drew the following conclusions: isoniazid therapy did not prove successful when inflamed lymph nodes exceeded a certain size. Suppurative lymphadenitis in lymph nodes exceeding 1.0 to 1.5 cm usually led to infiltration or even perforation of the skin. Surgery prevents these complications and significantly reduces healing time. Adjuvant isoniazid therapy cannot be recommended, except for generalized BCG tuberculosis.

No abstract

BCG vaccines are the oldest vaccines in use today, but the protective effect of the vaccination is still controversial. The risk of contracting tuberculosis is low compared with the possible complications after this vaccination. In Austria the formerly used BCG vaccine was not available in the required amount and another vaccine was released by the drug authorities. This product, with a more virulent strain, was used between August and December 1990, and this increased the incidence of complications. Eighty four of 1950 vaccinated newborn babies developed severe suppurative lymphadenitis three to 28 weeks after the vaccination, and surgical treatment was found to be necessary. Isoniazid treatment did not prove to be successful when the lymph node exceeded a certain size. Culture was successful in 46% up to week 20; after 20 weeks no culture became positive. AlU cultured bacteria were isoniazid sensitive.The question of general vaccination is raised and several points were considered before we came to the conclusion that except for high risk groups a general vaccination programme for neonates is not justified in Western countries.

In our group, associated malformations were the main factor affecting the long-term quality of life of children with omphalocele and gastroschisis. Although most of the children were developing normally, fear of a repetition of the malformation in a subsequent pregnancy dominated reproductive choices in all couples.