Exploiting Phenotypic Plasticity for the Treatment of Hepatopulmonary Shunting in Abernethy Malformation

An 11-year-old boy presented with exercise intolerance due to chronic hypoxemia. Work-up revealed a diagnosis of hepatopulmonary syndrome (HPS) secondary to a congenital extrahepatic portal-venous shunt (Abernethy malformation). Plasticity in the developing liver was exploited as a strategy for the treatment of HPS. With use of a staged endovascular approach, the portosystemic vascular circuitry was modified in a manner that facilitated progressive growth and development of the severely hypoplastic and underdeveloped intrahepatic portal venous system. After completion of the final procedure, the patient's intrahepatic portal veins were normal in appearance; 2 months later, signs and symptoms of HPS completely resolved. The patient remains free of HPS stigmata after 2 years.