Hepatic rupture caused by peliosis hepatis

Peliosis hepatis is a rare entity that can affect children and cause fatal hepatic hemorrhage or hepatic failure. Radiographic findings are nonspecific and can resemble other hepatic pathologies such as cysts, abscesses, metastases, and hemangiomatosis. Peliosis hepatis can resolve spontaneously or by withdrawal of inciting medications. Certain cases may require surgical resection of the involved portions of the liver. Recently, fatal liver hemorrhage was reported in 2 pediatric patients with a rare congenital muscle disorder known as myotubular (centronuclear) myopathy. One of these patients was found at autopsy to have peliosis hepatis. The authors report the first successful treatment of life-threatening liver hemorrhage caused by peliosis hepatis in a child with myotubular myopathy. Awareness of this condition may reduce the catastrophic complications seen with peliosis hepatis. J Pediatr Surg 36:1456-1459. Copyright (C) 2001 by W.B. Saunders Company.