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.

Immobilisation hypercalciuria after limb fractures in children.

Immobilisation hypercalciuria and hypercalcaemia following limb fractures or paralysis is a frequent occurrence in children. Assessment of calcium metabolism should be performed in such patients, since the formation of kidney stones is possible. The beneficial effect of hydrochlorothiazide (HCT) in the prevention of renal stones is most likely due to a reduction of calcium concentration in urine and a significant decrease of crystalluria. Thus, its administration is recommended for children with hypercalciuria following prolonged immobilisation especially due to fracture treatment or paralysis.

[Dislocated supracondylar femoral fractures, slipped epiphyses and epiphyseal fractures in children]. / Dislozierte suprakondyläre Oberschenkel-Frakturen, Epiphysenlösungen und Epiphysen-Frakturen bei Kindern.

Dislocated supracondylar femoral fractures are usually transverse fractures and are unstable. In our opinion the primary treatment of these fractures should be surgical in order to save the patient a protracted healing period. Osteosynthesis using a straight plate is impossible, as the distal fragment is too short. Condylar plates are contraindicated and angular plates have too little stability. We feel that intramedullary pins are unsuitable in children. They provide too little stability when the distal fragment is short. Up to now we have achieved the best results with external fixation. Slipped epiphyses and epiphyseal fractures do not present problems as far as the surgical procedure is concerned (removal of the displaced periosteum, repositioning and compression). Healing is not delayed. The long-term results are poor, however, due to premature closure of the damaged epiphyseal plate.

[Gastroesophageal reflux in childhood]. / Der gastro-ösophageale Reflux im Kindesalter.

1. Gastro-oesophageal reflux of infancy and childhood leads to vomiting and frequently to aspiration pneumonia and failure to thrive. 2. Two thirds of all cases can be cured conservatively. One third has to undergo surgery. 3. According to our present knowledge, the mechanism of the cardia seems to be competent at birth, however, peristaltism and reflex activity undergoes a maturation process. 4. The aetiology of gastro-oesophageal reflux in childhood is variable. There is a distinct difference between primary and secondary reflux. The latter occurs in children with cerebral palsy as well as following operations of the oesophagus or the hiatus. 5. The indication for an operative intervention is not as much depending upon the radiographic findings as upon the existence of oesophagitis, stenosis, anemia and aspiration pneumonia. 6. Nissen's fundoplication is not the operation of choice in childhood since this intervention is followed by a high morbidity. For uncomplicated cases, reconstruction of the angle of His and repositioning of the abdominal oesophagus into the abdominal cavity in combination with a semiplication of the fundus is preferable.

[Parenteral feeding in child surgery. Results of balance studies]. / Parenterale Ernährung in der Kinderchirurgie Ergebnisse von Bilanzuntersuchungen

After operations in infancy considerable negative nitrogen balances are regularly observed. Balance examinations have demonstrated that there is a minimal requirement of 300 to 350 mg N in the form of a crystalline amino acid solution for the normalization of the catabolic condition of metabolism. The administration of nitrogen, however, is only reasonable, if simultaneously 60 cal/kg of combustible energy is supplied. In severe surgical diseases and in neonates the metabolic requirements can be twice as high. In such cases, if correctly applied and accompanied by controls of the metabolic condition, parenteral nutrition can be useful and may, therefore, be regarded as one of the greatest progresses of modern medicine.