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 only virtually certain methods of diagnosis are direct inspection of the gall bladder, cholangiogram, and a sizeable liver biopsy. Our clinical experience has convinced us that laparoscopy with liver biospy and simultaneous cholangiography by transhepatic puncture of the gall bladder is the simplest, fastest and most certain method for diagnosis. It allows for early differentiation of direct hyperbilirubinemia in the neonatal age and should be carried out if the hyperbilirubinemia has persisted during the first month of life and before laboratory investigations are instituted.

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.

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. Recent clinical experience appears to confirm this hypothesis.

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. Only one child with a cleft type III died, although a mortality rate of 93% is reported in the literature. GER has to be excluded before tracheoesophageal cleft surgery is undertaken.

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. In one case HbA1c was significantly elevated which is thought to be an expression of recurrent hyperglycemia. In both infants the first and most impressive clinical sign was absolute refusal or oral feeds. Normal oral food intake was slowly re-established after normalization of blood glucose homeostasis.

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.

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.

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. The importance of prenatal diagnosis and hence early post partum treatment of obstructive uropathies appears to us proven.

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. In the present series of 32 small intestinal transplants the monitoring system and explanation criteria are now tested. Twenty-three primarily successfully transplanted animals had complications of the graft, i.e., hemorrhagic infarction, deficient arterial circulation, or rejection. Only 1 of the 23 died before explanation could be done. Twenty-two were explanted according to the previously established criteria. Two died of complications not directly related to the graft, the other 20 animals survived for 1-5 mo. All 22 explanted grafts showed irreversible damages both macroscopically and on histologic examination. The present series confirms that early recognition of complications of the graft and timely removal with survival of the recipient animal is possible. This could be an important step forward to the clinical application of small intestinal transplantation in man.

[Diagnosis and therapy of dumping syndrome following Nissen fundoplication with reference to pathogenetic aspects]. / Diagnose und Therapie des Dumping-Syndroms nach Nissen-Fundoplikation unter Berücksichtigung pathogenetischer Gesichtspunkte.

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. In all cases an absolute refusal of feeding was observed. The diagnosis was confirmed by a typical early postprandial hyperglycaemia with hyperinsulinaemia leading to a reactive hypoglycaemia. Additionally, we were able to demonstrate an increased HbA1c as an expression of recurrent hyperglycaemias in 3 infants. In 6 infants the dumping syndrome was of short duration and the symptoms disappeared after application of a so-called dumping diet. In this diet the easily resorbable carbohydrates are replaced by uncooked starch. But in one case we were forced to use continuous enteral nutrition because of persistence of the symptoms 1 year after the Nissen fundoplication. Complete refusal of feeding is an early symptom of the dumping syndrome. If this symptom is observed after a Nissen's fundoplication, a dumping syndrome must be excluded.

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. The findings suggest that uncooked starch has a place in the dietary control of dumping syndrome in infants and possibly in adults.

[Intraoperative spasmolysis with glucagon in an irreducible invagination]. / Intraoperative Spasmolyse mit Glucagon bei schwer reduzierbarer 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. This aspect and the successful use of Glucagon during operation demonstrate that Glucagon should be administered wherever atraumatic surgical reduction of intussusception is not possible. This procedure may contribute to avoid unnecessary bowel resections.

[The significance of pathophysiologic principles of intestinal secretion for the diagnosis and therapy of ileus]. / Bedeutung pathophysiologischer Grundlagen der intestinalen Sekretion für Diagnose und Therapie des 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. In analogy to secretory diarrhea and because of own clinical observations we conclude that bowel contamination caused by intestinal stasis is mainly responsible for the increased intestinal secretion in bowel obstruction.

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. This experimental model has shown that in cases of partial proximal or distal blockage, the flow through the shunt system is significantly diminished but not totally stopped, and the pressure reading may thus be normal. In these cases, the shunt should be revised on the basis of clinical evidence of dysfunction.

[Anal sphincter dysplasia as cause of chronic defecation disorders: a clinical and genetic study]. / Analsphinkterdysplasie als Ursache chronischer Defäkationsstörungen: eine klinische und genetische Studie.

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. One usually finds dysplasia and absent (type I) or incomplete (type II) fixation of the sphincter complex to the coccyx. Both are demonstrable by computerized tomography (CT) as well as by intraoperative dissection of the sphincter muscles. There ist also shortening of the ectodermal segment of the anal canal which is obviously responsible for the disturbed stool sensation. Posterior butterfly anoplasty combined with fixation of the sphincter complex to the coccyx usually leads to immediate improvement of defecation disorders. A genetic study on 42 patients (age 1-52 years) operated on for anal sphincter dysplasia revealed autosomal dominant inheritance with variable expression and probably incomplete penetrance of the mutated gene.

[Perianal fistula as a cause of meningitis?]. / Perianalfistel als Ursache einer 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. Due to two observations we conclude that the fistulas caused a sepsis with E. coli. In one case meningitis and a consecutive empyema occurred, in a second case pyelonephritis. In the following we describe the patient with meningitis and subdural empyema.

[Painful pronation--a diagnostic pitfall in septic arthritis and osteomyelitis of the elbow in infancy]. / Pronation douloureuse--Ein diagnostischer Reinfall bei eitriger Arthritis und Osteomyelitis des Ellbogens im Kleinkindesalter.

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. In the remaining 7% the most common cause for failure to treatment is an incomplete or recurrent subluxation of the radial head and distortions or fractures of the elbow. But in approx. 1% the examining physician must suspect a hidden osteomyelitis or osteoarthritis of the elbow. With adequate antibiotic therapy the prognosis is generally good. To avoid this diagnostic pitfall, it is essential to re-examine carefully all cases which do not respond to therapy in order to exclude an osteomyelitis.

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. Our study revealed isolated NID (4/49 cases) to be eight times rarer than aganglionosis (33/49 patients). Combination of both diseases has been reported to occur in 20% of NID cases; we did however find it in 75% (12/16) of our patients. NID was located proximal to the aganglionic segment and reached the stomach in 1 case. Combination of both diseases resulted in superposition of symptoms, thus rendering impossible to differentiate between NID group A (with hypoplasia of sympathetic innervation and acute early onset) and group B (with normal sympathetic innervation and chronic, late onset) on clinical grounds only. Major malformations were present in 5/16 patients with NID; 2 of them had trisomy 21.

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. A stenosis in the terminal ileum was experimentally created in Göttingen minipigs and the bacterial flora of the small bowel assessed by quantitative cultures. After 3 months the number of aerobic and anaerobic bacteria in the pre- and poststenotic region had increased by a factor of 10(2)-10(5). The acute form of CSBS was diagnosed by microbiological examination of gastric samples in 14 children. After the children were treated with orally and intravenously administered antibiotics, the symptoms disappeared within 12-36 h. Reoperations for small bowel obstruction can be avoided by conservative treatment of CSBS with antibiotics.