Longeville J H, de la Hall P, Dolan P, Holt A W, Lillie P E, Williams J A, Padbury R T
Department of Surgery, Flinders Medical Centre, Adelaide, South Australia.
HPB Surg. 1997;10(3):159-62. doi: 10.1155/1997/10136.
We describe a case of giant cavernous haemangioma of the liver with disseminated intravascular coagulopathy (Kasabach-Merritt syndrome) which was cured by orthotopic liver transplant. A 47 year old man presented with bleeding and tender massive hepatomegaly after tooth extraction. Investigations showed disseminated intravascular coagulopathy and a giant hepatic haemangioma involving both lobes of the liver. Initial treatment failed to resolve the coagulopathy and liver resection was attempted. At laparotomy the tumour was unresectable and the only option for cure was to offer a liver transplantation. The orthotopic liver transplant was performed 20 days after initial laparotomy. Subsequently, all coagulation parameters returned to normal and the patient remains well after 12 months. Orthotopic liver transplant can be considered for giant hepatic haemangioma with Kasabach-Merritt syndrome when resection is necessary and a partial hepatectomy is not technically feasible.
我们描述了一例通过原位肝移植治愈的伴有弥散性血管内凝血(卡萨巴赫 - 梅里特综合征)的巨大肝海绵状血管瘤病例。一名47岁男性在拔牙后出现出血和压痛性肝脏肿大。检查显示弥散性血管内凝血以及累及肝脏两叶的巨大肝血管瘤。初始治疗未能解决凝血障碍,遂尝试肝切除术。剖腹手术时发现肿瘤无法切除,唯一的治愈选择是进行肝移植。在初次剖腹手术后20天进行了原位肝移植。随后,所有凝血参数恢复正常,患者在12个月后情况良好。当有必要进行切除且部分肝切除术在技术上不可行时,对于伴有卡萨巴赫 - 梅里特综合征的巨大肝血管瘤可考虑原位肝移植。