[Anal sphincter dysplasia as cause of chronic defecation disorders: a clinical and genetic study].

Anal sphincter dysplasia is a congenital, often familial malformation of the anal canal. In the literature, the anomaly is poorly represented and usually referred to as anteriorly or ventrally displaced anus. The range of symptoms includes chronic constipation, severe straining at defecation, encopresis and chronic paradoxical diarrhea with fecal incontinence.

Isolation of respiratory syncytial virus from liver tissue and extrahepatic biliary atresia material.

Respiratory syncytial virus (RSV) was isolated from liver tissue in a 7-month-old infant with extrahepatic biliary atresia. To our knowledge this is the first report of RSV isolation from human liver tissue.

[Perianal fistula as a cause of meningitis?].

In childhood perianal fistulas are frequent. The origin of the fistula is the anorectal line, especially from the Morgagni crypts. Local recurrence of abscesses are observed.

The importance of oral sodium replacement in ileostomy patients.

The main function of the colon is fluid and sodium conservation. In ileostomy patients these colonic functions are lacking. The consequence is excessive loss of fluid and sodium, failure to thrive, and skin excoriation around the ileostomy.

The influence of small bowel contamination on the pathogenesis of bowel obstruction.

Altered motility of the intestine after laparotomy, adynamic bowel segments, blind bowel loops following bypass operations, or diverticula may cause pathological growth of intestinal microflora and thus lead to contaminated small bowel syndrome (CSBS). As a result of malabsorption in the jejunum and ileum, loss of weight, growth arrest, diarrhea, steatorrhea, megaloblastic anemia, and hypoproteinemia may occur. In addition to these, the acute symptoms of small bowel contamination, intestinal obstruction and secretory diarrhea, are less well known.

[The significance of pathophysiologic principles of intestinal secretion for the diagnosis and therapy of ileus].

Intestinal obstruction is always accompanied by intestinal hypersecretion. This phenomenon explains the initial symptoms like bilious vomiting and abdominal distension as well as the later clinical signs of hypovolaemia and shock. The proximal hypersecretion in intestinal obstruction is incompletely understood and in the surgical literature on ileus only little attention has been paid to this crucial observation.

[Diagnosis and therapy of dumping syndrome following Nissen fundoplication with reference to pathogenetic aspects].

Between 1982 and 1987 27 Nissen's fundoplications were carried out in our institution. Postoperatively 7 infants showed a typical dumping syndrome. The symptoms were irritability, pallor, sweating, tachycardia, lethargy, diarrhoea and vomiting.

[Intraoperative spasmolysis with glucagon in an irreducible invagination].

We present 4 cases in which atraumatic reduction of an intussusception was achieved only after intraoperative intravenous administration of Glucagon. Because of this observation all our cases of the past 10 years with intussusceptions where bowel resection was necessary, were reviewed. Intraoperative macroscopic and histological findings revealed that bowel resections were not only necessary because of necrosis but also because of bowel wall lesions caused by traumatic reduction or the impossibility to reduce the intussusception.

[Painful pronation--a diagnostic pitfall in septic arthritis and osteomyelitis of the elbow in infancy].

Pulled elbow is a common injury in infancy and childhood. The history and clinical findings are usually classical, but one should never forget that the classical history can always be easily reconstructed by the examining physician. It can be estimated that in 93% of all cases a dramatic response to therapy can be observed.

Deleterious consequences of gastroesophageal reflux in cleft larynx surgery.

Our series of 17 children with laryngotracheal clefts is reported. In three of four cases with a complicated postoperative course, gastroesophageal reflux (GER) has been found. Three breakdowns of the surgical repair opposed to be due to GER.

Infant dumping syndrome: reversal of symptoms by feeding uncooked starch.

Two infants with dumping syndrome after Nissen's fundoplication were bolus-fed with regular cows' milk formula and with test meals containing either cooked or uncooked starch. Cows' milk formula and test meals made with cooked starch provoked dumping symptoms, hyperglycaemia and hyperinsulinaemia. Dumping symptoms vanished and normoglycaemia was established when meals contained uncooked starch as the sole carbohydrate.

Neuronal intestinal dysplasia--clinical and histochemical findings and its association with Hirschsprung's disease.

Neuronal intestinal dysplasia (NID) is a disturbance of the innervation of the gut. Its symptoms resemble the ones seen in Hirschsprung's Disease. Contrary to aganglionosis, however, there is hyperplasia of the ganglia.

[Contamination of the small intestine as a cause for ileus attacks in children after intestinal surgery].

During the last two years eight children aged 3 months to 7 years were treated successfully for contaminated small bowel syndrome (CSBS). All patients had a history of a laparotomy in the neonatal period and showed bile stained vomiting and diarrhoea. On examination, a painful distended abdomen with hyperactive bowel sounds was found.

Prenatal diagnosis improves the prognosis of children with obstructive uropathies.

Five neonates with gross obstructive uropathies diagnosed in utero were operated during the first few days of life. Follow-up studies of up to 2 years postoperatively showed normal development of all the children. Radiologic investigations showed no obstruction and normal growth of renal parenchyma in all but one case, thus differing markedly from infants with gross obstructive uropathies who were diagnosed and referred to us some months after birth.

Familial incidence of congenital anorectal anomalies.

We describe two families with pedigrees over three and two generations with nine members affected with anorectal malformations. Both pedigrees are compatible with autosomal dominant inheritance with variable penetrance and expression or with multifactorial inheritance with a high genetic load. The recurrence risk is thought to be in the range of 10% to 20% for first degree relatives of affected members.

Dumping syndrome following Nissen's fundoplication: a cause for refusal to feed.

Two cases of infantile dumping syndrome which developed following Nissen fundoplication for gastroesophageal reflux are described. Both infants were fed postoperatively via a gastrostomy and showed the typical clinical picture of dumping with failure to thrive, intermittent diarrhea, lethargy and pallor postprandially. Several glucose tolerance tests were highly pathological with marked hyperglycemia immediately after a gastrostomy meal followed by hypoglycemia two hours later.

Prolonged foreign body impaction in the oesophagus.

The severe consequences of prolonged impaction in the oesophagus of a fairly radiolucent foreign object (aluminium can top) in 2 children are reported. The value of endoscopy in cases of unexplained dysphagia, even in the presence of normal radiographic examination, is stressed.

Limitations in the diagnosis of valvular dysfunction using the Rickham reservoir.

The majority of shunt dysfunctions can be easily diagnosed by puncturing the Rickham reservoir with a fine needle and estimating the intracranial pressure. Experience has, however, shown that definite shunt dysfunctions can be missed by this method. These limitations of the technique are better understood by constructing a model which enables us to simulate the pressure and flow situation in a ventricular drainage system.

Hemiparesis--an uncommon symptom of hydrocephalus or shunt dysfunction.

We describe a boy with asymmetrical hydrocephalus in whom a marked hemiparesis of recent origin resolved completely following insertion of a shunt. In 2 children with shunted obstructive hydrocephalus due to a midline tumour a newly developed hemiparesis improved markedly in one and resolved in the other case subsequent to revision of a blocked shunt. A hemiparesis should therefore not necessarily be attributed to assumed progression of an underlying disease.

Early differential diagnosis between neonatal hepatitis and biliary atresia.

More and more refined laboratory investigations for differential diagnosis of neonatal direct hyperbilirubinemia have been developed, but not one of them is absolutely diagnostic. All of them take time and by the time the infant is referred to the surgeon it is often too late, as the prognosis for successful surgical correction becomes rapidly worse with increasing age. Most authorities have now agreed that in cases of extrahepatic biliary atresia cirrhosis has already well developed after the second month of life.

The importance of hepatic lymph drainage in experimental biliary atresia. Effect of omentopexy on prevention of cholangitis.

It is believed that partial destruction of the hepatic lymph drainage during the operation of hepato-portoenterostomy is probably the cause of postoperative attacks of cholangitis. Experimental evidence is described that appears to show that omentopexy to the porta hepatis will aid hepatic lymph drainage and may thus prevent postoperative cholangitis.

The risks of small intestinal transplantation for the recipient: experimental results in young minipigs.

In a previous series of 36 small intestinal transplants in young minipigs, a monitoring system, based on a special transplantation model, clinical and histologic criteria has been worked out. The spontaneous evolution was observed and no attempt was made to save the animal when complications on the graft occurred. Most of the animals died.

Experimental investigations into the etiology of cholangitis following operation for biliary atresia.

Experimental operations carried out on young minipigs appear to show that cholangitis does not occur after simple ligation of the bile duct, but develops nearly invariably is the lymphatic drainage from the liver is also interrupted. The so-called "ascending" cholangitis observed after hepato-porto-entero anastomosis is probably not ascending at all, but blood-borne. When a hepatic portoenterostomy is carried out for biliary atresia, trauma to the lymphatics in the porta hepatis should be avoided and an additional omento-hepato-portopexy should be done.

Laparoscopy in childhood.

The indications and technique for laparoscopy in childhood are discussed. During the last 5 years 59 laparoscopies were carried out in the Surgical Department of the University Children's Hospital, Zürich, Switzerland, These cases are described in some detail. No complications were encountered.