Image finding of Abernethy type II malformation in 12 year old patient

Abstract

Malformations of the abdominal venous system are rare vascular disorders. The inciden- ce of this condition is estimated at one in every 30,000 births and is associated with gastrointes- tinal, genitourinary, bone, and cardiovascular malformations. In 2018, 39 cases of type I Aber- nethy and 22 cases of type II Abernethy were registered in the world literature. CLINICAL CASE a 12-year-old female patient with a history of portal hypertension treated 2 years ago, with a his- tory of general malaise and jaundice, went to the private center for complementary studies. A por- tal Doppler ultrasound revealed an isoechoic lesion to the liver parenchyma in the lower aspect of the right lobe. The evaluation was continued by performing a tomography in which it was observed: abnormal configuration of the portal venous system; the splenic and superior mesenteric veins are dilated, in addition, an elongated portosplenic confluence was evidenced, in with which two portal paths derive, one of them draining the aforementioned hepatic lobulation and the second communicates with the right hepatic portal venous system, demonstrating tortuosity of its trajectory, with stenosis of its proximal portion. The findings described above suggest vascular malformation of the portal-splanchnic venous system, which causes Portosystemic shunt concerning type II Abernethy mal- formation. Early diagnosis is recommended. The preferred examination is Doppler ultrasound with subsequent confirmation by abdominal Angiotech. Treatment is extremely important because its delay can lead to irreparable injuries up to liver failure and death.