Samuel M, Spitz L
Department of Pediatric Surgery, Hospital for Children, London, England.
J Pediatr Surg. 1995 Oct;30(10):1425-9. doi: 10.1016/0022-3468(95)90397-6.
Of 16 infants with infantile hepatic hemangioendothelioma, 14 (88%) presented before age 3 months. For seven cases (44%), the diagnosis was suspected from antenatal ultrasonographic findings. Two (13%) presented with asymptomatic hepatomegaly. The most common presenting features were high-output cardiac failure in 11 (69%), consumptive coagulopathy in 12 (75%), and anemia in 12(75%). Sixty-three percent of the children had associated cutaneous hemangiomata, and disseminated hemangiomatosis was noted in two (13%). Medical measures were effective in stabilizing seven (44%) cases with high-output congestive cardiac failure and/or consumptive coagulopathy. Partial right hepatectomy was successful for four patients; the only death occurred in a newborn, after intraperitoneal rupture of the hepatic hemangioma. Embolization was used in two children to induce involution. Spontaneous involution occurred in two patients. Initially, hepatic hemangiomas should be treated conservatively, with surgery reserved for intractable cardiac failure and/or refractory consumptive coagulopathy.
16例婴儿肝血管内皮瘤患儿中，14例（88%）在3个月龄前发病。7例（44%）根据产前超声检查结果怀疑诊断。2例（13%）表现为无症状肝肿大。最常见的表现为11例（69%）高输出量心力衰竭、12例（75%）消耗性凝血病和12例（75%）贫血。63%的患儿伴有皮肤血管瘤，2例（13%）有弥漫性血管瘤病。内科治疗对7例（44%）高输出量充血性心力衰竭和/或消耗性凝血病患儿病情稳定有效。4例患者行右半肝切除术成功；唯一1例死亡发生在1例新生儿，因肝血管瘤腹腔内破裂。2例患儿采用栓塞治疗促使肿瘤消退。2例患者肿瘤自然消退。最初，肝血管瘤应采取保守治疗，手术仅用于治疗难治性心力衰竭和/或难治性消耗性凝血病。
