Chronic Hypoxemia in a Child: Thinking Outside the Box.

Background: Chronic hypoxemia is generally attributed to primary cardiac or pulmonary entities. Case characteristics: A 9-year-old boy presenting with cyanosis, clubbing and hypoxemia, without icterus or hepatosplenomegaly. Cardiovascular and respiratory system examinations were normal. Outcome: He was diagnosed as type IB Abernethy malformation, a rare cause of hepatopulmonary syndrome. Message: Pediatricians should consider hepatopulmonary syndrome in the differential diagnosis of chronic hypoxemia, even in the absence of jaundice or hepatosplenomegaly.