Abernethy malformation: a case report

A 5-year-old female child presented with short history of jaundice. A provisional diagnosis of acute viral hepatitis was made in view of clinical presentation and local endemicity of viral hepatitis A. Persistence of jaundice on follow up after 4？weeks led to detailed investigations. Ultrasound and doppler study of abdomen revealed drainage of portal vein into inferior vena cava. CT angiography was performed which confirmed the diagnosis of Type 1 b Abernethy malformation without associated major anomalies. We discuss the common clinical presentations, associated anomalies, diagnostic workup and treatment options of this disorder.The treatment of the patients with congenital porto-systemic shunts depends on the site of the shunt, associated congenital anomalies and the extent of liver damage but the prognosis depends on the complications irrespective of anatomical type. However, the extent of associated abnormalities should not deter paediatricians to refer patients for treatment. Whenever possible closure of the shunt should be advised for cure or to prevent complications. Only symptomatic type I patients with absence of possibility to close the shunt may require liver transplant. Long-term follow-up is indicated for all patients.John Abernethy first reported congenital absence of portal vein and congenital mesenterico-caval shunt in 1793 [1]. Abernethy malformation is defined as congenital diversion of portal blood away from the liver by either end-to-side or side-to-side shunt [2]. Morgan and Superina [3] classified congenital extrahepatic portosystemic shunt (CEPSh) into two types (See Table 1). Till the year 2008 only forty cases of CEPSh type I and twenty-two of CEPSh type II, had been described in literature [4,5]. This is despite the tremendous advances in the diagnostic medical imaging, thus underpinning the rarity of these cases. Only one paediatric case has been reported from India [6]. In this article we describe a patient diagnosed to have type I b Aber