All issues > Volume 50(8); 2007

Case Report

Korean J Pediatr. 2007;50(8):794-798. Published online August 15, 2007.

A case of hepatopulmonary syndrome in a child with fatty liver disease secondary to hypopituitarism after craniopharyngioma resection

Sun Ju SJ Im¹, Hyun Seok HS Park¹, Hyoung Doo HD Lee¹, Jae Hong JH Park¹, Hee Ju HJ Park¹

¹Departments of Pediatrics, College of Medicine, Pusan National University, Busan, Korea

Correspondence Hee Ju HJ Park ,Email: phj7294@hanmail.net

Abstract

Hepatopulmonary syndrome is a triad that includes: hepatic dysfunction, intrapulmonary vascular dilatation and abnormal arterial oxygenation. The incidence of intrapulmonary vascular dilatations, in adults with end-stage liver disease, has been reported to be 13% to 47%, however the incidence in children is unclear and the cases in Korean children have never been reported. The hepatopulmonary syndrome may occur as a result of chronic liver disease following nonalcoholic steatohepatitis in children with hypothalamic or pituitary dysfunction. We report a case of hepatopulmonary syndrome in a 13-year- old child who had rapidly progressive liver dysfunction secondary to panhypopituitarism after craniopharyngioma resection. Careful monitoring and treatment of endocrine abnormalities and metabolic status, as well as liver function, are required in all children undergoing pituitary tumor resection.

Keywords :Hepatopulmonary syndrome, Fatty liver disease, Hypopituitarism, Craniopharyngioma