Background:

Hepatopulmonary syndrome is a rare complication characterized by liver disease associated with hypoxemia and intrapulmonary vascular dilatations. The prevalence reported in the few studies in children with cirrhosis is 3-8%. Although uncommon, it is important for physicians to recognize this condition because of its progressive course.

Case report:

We report the case of an 8-year-old girl diagnosed with liver cirrhosis and portal hypertension with symptoms of dyspnea and cyanosis. On physical examination the patient was found malnourished with jaundice, telangiectasias in abdomen, severe clubbing, acrocyanosis, platypnea and orthodeoxia; arterial blood gas showed PaO2 of 59 mmHg. Echocardiography with agitated saline test was positive and lung perfusion scan with albumin macroaggregates reported 15% right-to-left short circuit, thus demonstrating the existence of intrapulmonary shunt. Diagnosis of severe hepatopulmonary syndrome was made. Liver transplantation is recommended as the only effective treatment.

Conclusions:

In children with liver disease presenting dyspnea and hypoxemia and those enrolled in a liver transplant protocol, hepatopulmonary syndrome must be intentionally searched because the prognosis will depend on timely diagnosis.