Wang S Y, Ruggles S, Vade A, Newman B M, Borge M A
Department of Radiology, Loyola University Medical Center, Maywood, IL 60153, USA.
J Pediatr Surg. 2001 Sep;36(9):1456-9. doi: 10.1053/jpsu.2001.26397.
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.
肝紫癜是一种罕见的病症，可影响儿童并导致致命性肝出血或肝衰竭。影像学表现无特异性，可类似于其他肝脏病变，如囊肿、脓肿、转移瘤和肝血管瘤病。肝紫癜可自行消退或通过停用诱发药物而缓解。某些病例可能需要手术切除肝脏的受累部分。最近，有报道称2例患有罕见先天性肌肉疾病——肌管（中央核）肌病的儿科患者发生了致命性肝出血。其中1例患者尸检时发现患有肝紫癜。作者报告了首例成功治疗肌管肌病患儿因肝紫癜引起的危及生命的肝出血的病例。认识到这种情况可能会减少肝紫癜所见的灾难性并发症。
