An adolescent girl in Hong Kong with type Ib Abernethy malformation complicated by multiple focal nodular hyperplasia

Abstract

Congenital portosystemic venous shunts are developmental anomalies. They represent portal communication with the systemic circulation. The pathogenesis is linked to the complexity of the embryological development of the inferior vena cava and portal vein. We reported a case of an asymptomatic 14-year-old Chinese adolescent girl in Hong Kong with a confirmed congenital portosystemic shunt type 1b. The condition can be diagnosed using contrast-enhanced CT scans and MRIs. Early recognition of the condition is important due to elevated risks of developing hepatocellular tumours. Liver transplantation may be considered curative.

Keywords

• Portosystemic shunt
• Focal nodular hyperplasia
• Abernethy malformation
• Ultra-sonography
• Associations
• Complications