Biliary atresia Q44.2

Biliary atresia is an idiopathic neonatal cholangiopathy characterized by progressive inflammatory obliteration of the intrahepatic or extrahepatic bile ducts. The disease occurs exclusively in the newborn (neonatal period).

The prevalence at birth varies worldwide between 1-9: 100 000 live births. An increase of the disease has been observed in Asia and the Pacific region. In Western Europe, approximately one child in 18,000 falls ill during the neonatal period. In Germany, about 35 cases per year are expected (Mysore KR et al. 2019).

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The cause is still unknown. This statement applies to syndromal biliary atresia (BASM), cystic form and cases associated with CMV IgM antibodies (Lakshminarayanan B et al. 2016).

In about 10% of cases, atresia of the bile ducts is associated with other congenital 1 malformations (e.g. heart defects, polysplenia) and is summarized as syndromal form (BASM).

After birth, the children develop a prolonged jaundice, which in contrast to normal neonatal jaundice is mainly caused by water-soluble "direct" bilirubin. Affected infants defecate in acholic stool; the urine turns brown. The third leading symptom is an enlargement of the liver (hepatomegaly) (Mysore KR et al. 2019). Very often there is an association with facial dysmorphia, eye malformations, heart vitae and skeletal malformations.

The general condition of the children is initially good. Not even their thriving is impaired in the first months. Only later does weight loss and increasing hyperexcitability set in. Finally, splenomegaly and ascites are added as signs of an increase in pressure in the portal vein of the liver (portal hypertension). Fat digestion and thus also the absorption of fat-soluble vitamins, especially vitamin K, is disturbed (risk of bleeding).

Clinic; endoscopic retrograde cholangiopancreaticography or ERCP.

Biliary atresia must be distinguished from type 3 progressive familial intrahepatic cholestasis (see Byler syndrome - Zhang BP et al. 2019).

The prophylactic use of antibiotics to prevent cholangitis is controversial (Decharun K et al. 2016).

Kasai operation (hepatoporto-enterostomy): The aim of the operation is to prepare the hepatic duct in such a way that as many bile ducts as possible are opened. The bile produced by the liver can empty itself into the intestine via this gateway. To catch this "dripping" bile, a loop of intestine is sewn into the hepatic orifice in the shape of a funnel. This surgical technique has dramatically improved results in children with BA. Ultimately, most patients with BA require liver transplantation (LT) even after they have undergone successful Kasai surgery.

In industrialized countries, biliary atresia is the most common cause of the indication for liver transplantation in infants.

The isolated occurrence of atresia of the bile ducts is called a non-syndromal form.

1. Decharun K et al (2016) Prophylactic Antibiotics for Prevention of Cholangitis in Patients With Biliary Atresia Status Post-Kasai Portoenterostomy: A Systematic Review. Clin Pediatr (Phila) 55: 66-72.
2. Kasahara M et al (2017) Liver transplantation for biliary atresia: a systematic review. Pediatrics Surgery Int 33:1289-1295.
3. Lakshminarayanan B et al (2016) Biliary atresia: A comprehensive review. J Autoimmune 73:1-9.
4. Mysore KR et al (2019) Biliary Atresia as a Disease Starting In Utero: Implications for Treatment, Diagnosis, and Pathogenesis. J Pediatr Gastroenterol Nutr doi: 10.1097/MPG.
5. Zhang BP et al (2019) Biliary atresia combined with progressive familial intrahepatic cholestasis type 3: A case report and review of the literature. Medicine (Baltimore) 98:e15593.