Malformação de Abernethy: uma doença rara

Abstract

Abernethy malformation is characterized by an extrahepatic portosystemic shunt. We present the case of a 19-month-old boy with recurrent wheezing and thrombocytopenia under investigation for five months, hospitalized for a hypoxemic respiratory infection. Relevant findings included hypoxemia, dyspneia, hemolytic anemia, thrombocytopenia and elevated liver enzymes. He was placed on oxygen and steroidsuntil the 8th day, when there was clinical and laboratory worsening. He started antibiotics and was admitted to our Pediatric Intensive Care Unit. A thoracoabdominal CT scan was performed and revealed: enlarged heart, signs of pulmonary hypertension, bilateral consolidation, rudimentary right portal vein, arterialization of the hepatic circulation, perigastric, peri-esophageal and peri-aortic varices and splenomegaly. Two days after admission, pulmonary hypertension agravated and he developed refractory cardiogenic shock. He ended up dying. Autopsy revealed type II Abernethy malformation. Although rare, hepatosystemic shunts may manifest as recurrent wheezing. This case portrays the importance of early diagnosis to perform treatment before fatal complications.

Keywords

• hypertension
• portal
• pulmonary
• liver circulation
• critical care
• portal system