A type II Abernethy malformation that started with hypersplenism: a case report

Abstract

BACKGROUND: Abernethy malformations are rare congenital vascular malformations defined by diverting portal blood away from the liver. We present a case of Abernethy malformation that started with hypersplenism.CASE DISCUSSION: A 44-year-old female had leukopenia and thrombocytopenia 14 years ago and was diagnosed with hypersplenism without manifestations of cirrhosis. Since then, the disease gradually progressed, and the number of erythrocytes, leukocytes, and platelets significantly decreased. In addition, liver perfusion was insufficient for an extended period due to portosystemic shunt, and abnormal liver function was observed. The laboratory investigation excluded viral, alcoholic, drug, and autoimmune factors; magnetic resonance imaging and colour ultrasonography revealed a splenorenal shunt, and type II Abernethy malformation was considered. After shunt ligation, the leukocyte and platelet counts and haemoglobin level quickly returned to normal values, improving liver function. In this report, we discuss the common clinical manifestations, associated anomalies, diagnostic workup, and treatment options of this disorder.CONCLUSION: Abernethy malformation is a rare congenital vascular malformation. This vascular abnormality’s clinical importance and manifestations range from asymptomatic cases to liver or metabolic dysfunctions of various degrees. Treatment strategies are decided according to shunt types, locations, symptoms, severities, and clinical course shunt closure should be advised to cure or prevent complications for type II Abernethy malformation. Long-term follow-up is indicated for all patients.