Congenital hepatic fibrosis: clinical presentation, laboratory features and management at a tertiary care hospital of Lahore

Objective: To describe the clinical presentations, laboratory features and management of congenital hepatic fibrosis patients at a tertiary care hospital. Methods: The case series was conducted at The Children Hospital and Institute of Child Health, Lahore, Pakistan, from July 2013 to June 2015, and comprised patients of congenital hepatic fibrosis diagnosed on the basis of liver biopsy. SPSS 20 was used for statistical analysis. Results: The mean age of 25 patients in the study was 8.5 +/- 2.74 years, and the male-to-female ratio was 3:1. Parents of 21(84%) patients had consanguineous marriage, and 14(56%) patients had family history of hematemesis and melena. Besides, 15(60%) patients presented with hematemesis, 12(48%) had abdominal distension, 5(20%) were picked up on screening of siblings and 5(20%) were referred from general practitioners on feeling of hepatosplenomegaly during routine examination. All had hepatomegaly with a mean size of 7.2 +/- 2.3 cm palpable in midline. Splenomegaly was present in 24(96%). Overall, 15(60%) patients had oesophageal varices. Endoscopic band ligations were done in oesophageal variceal patients who were successfully managed, while 5(20%) patients required portosystemic shunt surgeries. Conclusion: Congenital hepatic fibrosis was not uncommon in our population having high rate of consanguinity and most of them were familial cases.