1Rohit Agarwal,1Abhai Verma, 1Abhai Verma, 1Gaurav Chauhan
1Medanta the Medicity
Abernethy malformation is an extrahepatic congenital portosystemic shunt (CPSS), an extremely rare condition characterized by the diversion of portal blood away from the liver. In this anomaly blood from splanchnic circulation drains into the inferior vena cava (IVC) through a shunt bypassing the liver, thereby causing an alteration in the metabolism of pulmonary vasoactive substances resulting in pulmonary vasodilatation, diffusion-perfusion mismatch, and eventually hypoxemia. The clinical manifestations of Abernethy malformation are highly variable and can be asymptomatic or symptomatic due to the shunting of blood such as hepatic encephalopathy or hepatopulmonary syndrome (HPS). Abernethy malformation can be classified into two types. Type I is defined by an absence of intrahepatic portal veins, and a lack of liver perfusion with portal blood. In type Ia, superior mesenteric and splenic veins drain separately into the IVC and in type Ib splanchnic blood drain via the common trunk into IVC . Type II is defined by side to side anastomosis of the portal vein (PV) with IVC in which hypoplastic intrahepatic portal vein supplies some degree of portal flow to liver parenchyma. Prolonged untreated severe hypoxemia can result in irreversible changes in the pulmonary vasculature and its consequences become incapacitating for the patient. Early shunt closure resolves hypoxemia and clinical symptomatology.
Material(s) and Method(s):
Endovascular plug embolization of the porto systemic shunt.
Complete blockage of congenital porto systemic shunt leading to improvement in symptoms of hypoxia and exercise tolerance.
Abernathy malformation is masquerader and most of the times early diagnosis is missed and patient keep visting cardiothoracic department for suspected treatment of cyanotic heart disease. However, with good clinical vigil and suspicion these patients can be easily diagnosed and most of the times can be treated with pin hole surgery with simple embolization technique.