Four male infants with cystic fibrosis and prolonged neonatal jaundice underwent Kasai procedure to relieve biliary obstruction due to apparent biliary atresia. The excised remnants had viscid mucus accumulation in hypoplastic gallbladders and distended peribiliary glands. Main hepatic ducts were narrow and/or malformed. Microscopic differences between the gallbladder and extrahepatic bile ducts in cystic fibrosis and sporadic biliary atresia were unequivocal, despite some histologic overlap; no erosive or fibro-obliterative lesions typical of biliary atresia were seen. Common in liver, biopsies were small duct cholangiopathy with intense focal cholangiolitis and massive accumulation of ceroid pigment within damaged cholangiocytes, and in portal macrophages, portal fibrosis, and unequivocal features of large duct obstruction were inconspicuous compared with biliary atresia. Plugs of bile in small ducts tended to be pale and strongly periodic acid-Schiff-reactive in cystic fibrosis. Distinguishing the liver lesion from that of biliary atresia is challenging but possible. Liver biopsies from 2 additional infants with cystic fibrosis and prolonged jaundice that spontaneously resolved showed a similar small duct cholangiopathy. Small gallbladders and extrahepatic ducts challenge surgical judgment as findings in liver biopsies challenge the pathologist. The decision to perform a Kasai procedure is reasonable when mimicry of biliary atresia is grossly complete. We hypothesize that a disorder of bile volume/flow during development and/or early infancy linked to the CFTR mutation alone or in combination with the stresses of neonatal intensive care causes destructive cholangiolitis and intrahepatic reduction of bile flow with secondary hypoplasia of extrahepatic biliary structures.

中文翻译：

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囊性纤维化中的肝外胆管树发育不全和肝内胆管病不完全模拟 4 名囊性纤维化婴儿和 Kasai 门肠造口术中的胆道闭锁。

四名患有囊性纤维化和新生儿黄疸延长的男婴因明显的胆道闭锁而接受了 Kasai 手术，以缓解胆道梗阻。切除的残余物在发育不全的胆囊和扩张的胆周腺中有粘液积聚。主肝管狭窄和/或畸形。尽管存在一些组织学重叠，但胆囊和肝外胆管在囊性纤维化和散发性胆道闭锁方面的微观差异是明确的。没有看到胆道闭锁典型的糜烂性或纤维闭塞性病变。在肝脏中，活检常见的是小胆管胆管病，伴有严重的局灶性胆管炎和受损胆管细胞内蜡样色素大量积聚，而在门脉巨噬细胞中，门脉纤维化和大胆管阻塞的明确特征与胆道闭锁相比并不明显。在囊性纤维化中，小管中的胆汁栓往往呈苍白且与高碘酸希夫反应强烈。区分肝脏病变和胆道闭锁虽然具有挑战性，但也是可能的。另外 2 名患有囊性纤维化和长期黄疸但自行消退的婴儿的肝活检显示出类似的小管胆管病。小胆囊和肝外导管对手术判断提出了挑战，就像肝活检的结果对病理学家提出了挑战一样。当胆道闭锁的模仿大致完成时，进行 Kasai 手术的决定是合理的。我们假设，发育和/或婴儿早期胆汁容量/流量的紊乱与 CFTR 突变单独或与新生儿重症监护的压力相结合，导致破坏性胆管炎和肝内胆汁流量减少，并伴有肝外胆管结构继发性发育不全。