Description

Biliary atresia may be accompanied by extrahepatic malformations. The most common is polysplenia but other conditions may also be present.


 

Features of the biliary atresia splenic malformation (BASM) syndrome:

(1) biliary atresia

(2) splenic anomaly, including polysplenia and asplenia

(3) congenital heart disease (atrial septal defect, ventricular septal defect, tetralogy of Fallot, pulmonary stenosis, anomalous pulmonary venous return, patent ductus arteriosus, etc)

(4) intra-abdominal vascular malformations (absent inferior vena cava, anomalous hepatic artery, portal vein hypoplasia, etc)

(5) pancreatic anomalies

(6) malrotation

(7) situs inversus

(8) skeletal defects

(9) tracheoesophageal atresia or fistula

(10) duodenal or ileal atresia

(11) right lung hypoplasia

(12) genitourinary disorders (hypospadias, etc)

 


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