Objective: The prevalence of cholestatic jaundice as a presenting feature of congenital hypopituitarism is assessed.
Methodology: A retrospective case record analysis of the presenting features in all patients diagnosed as having congenital hypopituitarism between 1973-93.
Results: Seven of the 20 patients with congenital hypopituitarism presented with cholestatic jaundice as the major initial manifestation of the disorder. Liver biopsy findings in three revealed intracellular bile pigment accumulation and variable giant cell formation.
Conclusion: Cholestatic jaundice was the major manifestation of congenital hypopituitarism in 35% of patients presenting in the neonatal or early infancy period.