Özden İlgin, Yavru Ayşen, Güllüoğlu Mine, Alper Aydin, Bilge Orhan, Cantez Serdar, Durmaz Özlem
Department of General Surgery (Hepatopancreatobiliary Surgery Unit), Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Exp Clin Transplant. 2017 Mar;15(Suppl 2):82-85. doi: 10.6002/ect.TOND16.L23.
In this study, we report our experiences on the role of transplantation in 2 patients with large liver tumors in the setting of Abernethy malformation. Patient 1 was a 17-year-old boy who was referred for hepatic masses and recurrent hepatic encephalopathy episodes. Computed tomography and magnetic resonance imaging showed 2 large tumors (4 and 8 cm) in the liver. The portal vein drained directly into the vena cava. Core biopsy of the larger mass revealed fibrosis and regenerative hyperplasia. There were hyperintense signals in the T1-weighted images in the globus pallidus. The Stanford-Binet intelligence scale showed moderate mental retardation (IQ 39); however, the patient showed good ability for caring for himself. His cognitive defect was ascribed partially to chronic encephalopathy. The patient received a right hepatic lobe from his older brother. The congenital portacaval shunt was disconnected to provide inflow to the graft. Pathologic examination of the explanted liver revealed no evidence of malignancy. His IQ improved to 75 at 29 months posttransplant. The hyperintensity of the globus pallidus on magnetic resonance imaging disappeared. The patient has maintained a normal life during 9 years of follow-up. Patient 2 was a 17-year-old girl who was referred for multiple hepatic masses; she had no symptoms at admission. Magnetic resonance imaging showed type 1 Abernethy malformation and multiple hepatic masses (largest was 10 cm), which appeared to be hyperplastic lesions. Because malignancy could not be definitely excluded, she received a right lobe without the middle hepatic vein from her uncle. Pathologic examination of the explanted liver showed localized nodular hyperplasia; there was no evidence of malignancy. She has maintained normal life activities during 3 years of follow-up. Liver transplant is a curative treatment option for patients with large liver tumors, replacing the hepatic parenchyma in the setting of Abernethy malformation.
在本研究中，我们报告了肝移植在2例患有阿伯内西畸形且伴有大肝肿瘤患者中的作用。患者1是一名17岁男孩，因肝脏肿块和反复出现的肝性脑病发作前来就诊。计算机断层扫描和磁共振成像显示肝脏有2个大肿瘤（4厘米和8厘米）。门静脉直接引流至腔静脉。较大肿块的核心活检显示纤维化和再生性增生。苍白球在T1加权图像中有高信号。斯坦福-比奈智力量表显示中度智力发育迟缓（智商39）；然而，该患者自我照料能力良好。其认知缺陷部分归因于慢性脑病。该患者接受了来自其哥哥的右肝叶。先天性门腔分流被切断，以向移植物提供血流。切除肝脏的病理检查未发现恶性肿瘤迹象。移植后29个月时，他的智商提高到了75。磁共振成像上苍白球的高信号消失。在9年的随访期间，该患者维持了正常生活。患者2是一名17岁女孩，因多发性肝脏肿块前来就诊；入院时无症状。磁共振成像显示为1型阿伯内西畸形和多发性肝脏肿块（最大为10厘米），这些肿块似乎是增生性病变。由于不能明确排除恶性肿瘤，她接受了来自其叔叔的不含肝中静脉的右肝叶。切除肝脏的病理检查显示为局限性结节性增生；没有恶性肿瘤的证据。在3年的随访期间，她维持了正常的生活活动。对于患有大肝肿瘤的阿伯内西畸形患者，肝移植是一种治愈性治疗选择，可替代肝脏实质。
