[The Currarino syndrome: two case reports]. / La sindrome di Currarino: due casi clinici.

The association of congenital anal stenosis, or other anal and rectal malformation, sacral defect and a presacral mass is known as the Currarino syndrome described for the first time in 1981. Currarino et al. proposed that abnormal endoectodermal adhesions and notochordal defects in early fetal life may result in a fistula between the gut and the spinal canal with enteric elements ventrally and neural elements dorsally. This abnormality appears to be a variant of the split notochord syndrome. The occurrence of Currarino's triad of anomalies is familial in more than 50% of cases. The most important suggested hypothesis of transmission is an X-linked dominant pattern, but most of the other reports are consistent with an autosomal dominant mode of inheritance. The medical therapy is poorly successful and, therefore, the surgical treatment is recommended for Currarino's syndrome.

Foker's technique in oesophageal atresia with double fistula: a case report.

The authors' starting point is a new surgical approach described by Foker and performed by them on a newborn with long-gap oesophageal atresia. We discuss the possibility of obtaining rapid growth of the atretic oesophagus and performing a primary anastomosis in the case of long-gap oesophageal atresia. With this procedure, primary oesophageal repair could be performed without myotomies, without flaps and with the gastrooesophageal junction below the diaphragm. The need for gastrostomy would be also eliminated if the patient underwent the operation as a neonate.

[Biliary atresia syndrome and splenic malformation. A case report]. / Sindrome da atresia delle vie biliari e malformazione splenica. Caso clinico.

Biliary atresia is a severe neonatal malformation in which the entire extrahepatic biliary tract or part of it is absent and replaced by fibrosclerotic tissue. Having been described for the first time by Thomson in 1891, biliary atresia has an incidence of 1:10,000-15,000 live neonates with a slight predominance in the female sex; it is associated with other malformations in 25% of cases. In 1993 Davenport et al. identified a subgroup of patients with biliary atresia who presented a splenic malformation. This condition was known as biliary atresia and splenic malformation syndrome (BASM). The cases of biliary atresia and BASM are still not clear and a number of etiopathogenetic hypotheses have been put forward. The treatment is surgical and includes a first stage of hepatic-porto-enteroanastomosis using a defunctionalised Roux loop (Kasal), followed by the definitive operation comprising liver transplant. We report the case of a boy who was brought to our attention with jaundice, polypnea and hepatosplenomegalia; instrumental tests allowed the diagnosis of biliary atresia, median liver, stomach and spleen on the right, polysplenia, atresia of the intrahepatic tract of the inferior vena cava, presence of a superior vena cava to the left that drained into the upper left portion of the common atrium. Moreover, the cardiac apex was present on the right, extensive DIA of the common atrium type, extensive DIV and right aortic arch.

[Treatment of Hirschsprung's disease with the Soave-Coran technique: experience with newborns and children]. / Il trattamento del morbo di Hirschsprung con la tecnica di Soave-Coran: esperienza nel neonato e nel bambino.

Authors report their experience with Soave endorectal pull-through (ERPT) procedure modified by Coran for Hirschsprung's disease. At the Santobono Children's Hospital of Naples 30 patients have been operated for Hirschsprung's disease; 21 of them (age 10 days-3 years and 7 months) have undergone a Coran's procedure: a single stage procedure was performed in 16, the other 5 underwent ERPT following colostomy. Four complications occurred: 2 intestinal obstruction and 2 anastomotic leaks. Excellent long-term results reported from literature and proved by Author's experience suggest that one stage Coran's procedure can be safely performed even in neonates avoiding colostomy complications and reducing total duration and costs of hospitalization.