Pediatric hepatic sarcoidosis presenting as hypersplenism and portal hypertension.
Rohan Grotra, Himanshu Bhadani, Rajni Yadav et al.
✦ AI-curated · Sources linked
Case in brief
A mid-childhood female presented with massive splenomegaly, pancytopenia, and portal hypertension, but without constitutional or respiratory symptoms. Extensive evaluations ruled out other causes, and a liver biopsy confirmed sarcoidosis. Despite treatment with corticosteroids and azathioprine, severe hypersplenism persisted, leading to an elective splenectomy, after which her hematological parameters normalized and she showed significant clinical improvement.
What made this case unusual
This case is notable for the presentation of pediatric hepatic sarcoidosis without typical pulmonary or systemic features, and normal liver enzymes, which complicates diagnosis.
Diagnostic / clinical pearl
Clinicians should consider hepatic sarcoidosis in pediatric patients presenting with hypersplenism and portal hypertension, even when liver enzymes are normal.
Why it matters
This case underscores the importance of recognizing atypical presentations of sarcoidosis in children, which can lead to significant diagnostic challenges.