Budd–Chiari syndrome as presenting symptom of hepatic sarcoidosis in a child, with recurrence after liver transplantation

Van Brusselen, Daan; Janssen, Carl E.I.; Scott, Christiaan; Bevers, Nanja; Roskams, Tania; Wouters, Carine; Van Damme‐Lombaerts, Rita

doi:10.1111/J.1399-3046.2011.01535.X

Title

Budd–Chiari syndrome as presenting symptom of hepatic sarcoidosis in a child, with recurrence after liver transplantation

Author

Van Brusselen, Daan

Janssen, Carl E.I.

Scott, Christiaan

Bevers, Nanja

Roskams, Tania

Wouters, Carine

Van Damme‐Lombaerts, Rita

Abstract

A seven-yr-old boy presented with a severe Budd-Chiari syndrome, complicated by recurrent thrombosis of several successive TIPSs. Because of liver failure secondary to venous outflow tract obstruction and deterioration of his general condition, an emergency liver transplantation was performed. Steroids were discontinued three months after transplantation, and maintenance immunosuppressive therapy consisted of tacrolimus and azathioprine. Seven years later, this patient presented symptoms of recurrence of venous outflow obstruction in the transplant liver, comparable to the initial event. Histopathology of the liver revealed diffuse granulomatous inflammation with confluent non-caseating granulomas compressing the centrolobular veins. Extensive investigations excluded infections, immune deficiency, and systemic vasculitides. After treatment with a high dose of corticosteroids, the granulomas in the allograft disappeared completely. We report the first case of hepatic sarcoidosis, presenting with venous outflow obstruction and recurring after liver transplantation, in a child.

Language

English

Source (journal)

Pediatric transplantation. - Copenhagen

Publication

Copenhagen : 2012

ISSN

1397-3142

DOI

10.1111/J.1399-3046.2011.01535.X

Volume/pages

16 :2 (2012) , p. E58-E62