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Hyaline cartilage at porta hepatis in extrahepatic biliary atresia: metaplasia or choristoma

机译:肝外胆道闭锁症中肝门的透明软骨:化生或胆管瘤

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摘要

We report a case of a 5-month-old child who presented with the features of obstructive jaundice, anaemia and had no associated congenital anomalies. The child was suspected to have biliary atresia on hepatobiliary iminodiacetic acid (HIDA) scan and underwent Kasai portoenterostomy. Microscopic examination of the atretic gallbladder revealed islands of mature hyaline cartilage, lymphoid aggregates and fibrosis. There was an abrupt transition between the cartilage and the adjacent stroma. Only rare case reports have documented this finding with hypothesis ranging from metaplasia occurring secondary to the associated chronic inflammation or whether it is a choristoma. These cases need to be documented to elucidate the pathways of occurrence and may lead to have a better and detailed understanding of the disease process.
机译:我们报告了一个5个月大的儿童病例,该儿童表现为梗阻性黄疸,贫血且没有相关的先天性异常。肝胆亚氨基二乙酸(HIDA)扫描怀疑这名儿童患有胆道闭锁,并进行了开赛肠胃肠造口术。镜检无定形胆囊显示出成熟的透明软骨,淋巴样聚集和纤维化的岛屿。软骨和相邻基质之间突然过渡。只有极少数的病例报告以这一假设为依据记录了这一发现,其假设为继发于相关的慢性炎症的化生或是否是胆管瘤。这些案例需要记录在案,以阐明发生的途径,并可能导致对疾病过程有更好,更详细的了解。

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