Congenital extrahepatic Portosystemic shunt with hypoplasia of the intrahepatic inferior vena cava: A rare case report

Congenital portosystemic shunt (CPS) is a rare vascular anomaly resulting in diversion of splanchnic or portal blood into the systemic circulation. Other vascular malformations associated with this entity are uncommon. A 4-year-old female child with a diagnosis of acute viral hepatitis had an incidental finding of extrahepatic CPS on a Doppler abdominal ultrasound. Contrast-enhanced computed tomography showed a dilated portal vein having H-type side-to-side communication with a hypoplastic intrahepatic portion of the inferior vena cava and a prominent dilated azygos vein. There was retroaortic left renal vein drained into the IVC which was visualized in its entirety. Echocardiography findings were normal and the patient was discharged after symptomatic treatment that achieved improvement. With the expansion of abdominal imaging, incidental cases of CPS are increasingly being diagnosed in children. Although vascular malformations associated with CPS are rare, early diagnosis of cases helps avoid complications during shunt closure.