Intrahepatic Cystic Lesions After Hepatic Portoenterostomy for Biliary Atresia With Bile Lake and Dilated Bile Ducts
Intrahepatic Cystic Lesions After Hepatic Portoenterostomy for Biliary Atresia With Bile Lake and Dilated Bile Ducts
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DOI：
10.1097/01.mpg.0000237930.45846.ae
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发表时间：
2007-01
影响因子：
2.9
通讯作者：
T. Tainaka;K. Kaneko;T. Seo;Yasuyuki Ono;W. Sumida;H. Ando
中科院分区：
文献类型：
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作者：
T. Tainaka;K. Kaneko;T. Seo;Yasuyuki Ono;W. Sumida;H. Ando
背景资料：肝内囊性病变发展后，肝门肠吻合术在一些胆道闭锁的患者，并已被认为是一个预后不良的迹象，但是，有混淆的术语和概念的病变。我们调查了囊性病变是否构成一个单一的实体。患者和方法：从1980年到2005年，我们遇到了80例胆道闭锁患者。20例（25%）肝门肠吻合术后腹部超声检查发现肝内囊性病变。对13例肝移植患者的肝内囊性病变进行了影像学和组织学分析。结果：15例为单发单纯囊性病变，5例为多发连续椭圆形或串珠状病变。孤立性病变有纤维化的囊壁，缺乏上皮细胞。连续性病变囊壁被胆管上皮细胞覆盖。13例孤立性囊肿患者死亡或需要肝移植。2例连续性病变行肝门部再开孔术，可消除囊变及黄疸。结论：肝内囊性病变包括两种不同的情况。孤立性囊肿是潴留性假性囊肿，应称为胆汁湖，预后不良。连续的串珠状囊肿是胆管扩张，可以逆转。在考虑治疗策略时，这种区别很重要。
Background: Intrahepatic cystic lesions develop after hepatic portoenterostomy in some patients with biliary atresia and have been regarded as an indication of poor prognosis; however, there is confusion about the terminology and concepts of the lesions. We investigated whether the cystic lesions constituted a single entity. Patients and Methods: From 1980 to 2005, we encountered 80 patients with biliary atresia. Abdominal ultrasonography disclosed intrahepatic cystic lesions in 20 (25%) patients after hepatic portoenterostomy. The cystic lesions were analyzed morphologically with radiologic imaging studies and histologically in 13 patients who underwent liver transplantation. Results: Fifteen patients had solitary simple cystic lesions, and 5 patients had multiple continuous oval or beaded lesions. Solitary lesions had a fibrotic cyst wall and lacked epithelia. Continuous lesions had a cyst wall covered with biliary epithelia. Thirteen patients with solitary cysts died or required liver transplantation. In 2 patients with continuous lesions, surgical reboring of the porta hepatis could eliminate cystic lesions and jaundice. Conclusions: Intrahepatic cystic lesions include 2 different conditions. Solitary cysts are retention pseudocysts, which should be referred to as a bile lake, and are associated with poor prognosis. Continuous beaded cysts are dilated bile ducts, which may be reversed. This distinction is important when considering the treatment strategy.