先天性胆道闭锁（CBA）是一种以肝内/外胆管闭锁所致的肝胆系统病症，且是导致婴幼儿梗阻性黄疸的主要病因。CBA的病因未明，可能与基因变异、病毒感染、免疫损伤相关，缺乏针对性预防手段，目前诊疗核心在于早期诊断和手术干预，但临床仍面临多重挑战。CBA婴儿往往表现为黄疸持续不退，或在生理性黄疸消退后再次出现皮肤、巩膜黄染，伴小便颜色加深，甚至呈浓茶色；大便颜色变浅，甚至可呈白陶土色。腹腔镜或开腹胆道探查术目前被认为是临床诊断胆道闭锁的“金标准”。近年来，新生儿粪便比色卡筛查及新型生物标志物（如血清基质金属蛋白酶7）的应用提升了早期检出率。CBA治疗策略以肝门空肠吻合术（Kasai手术）为首选，理想手术时机为出生后45天内。患儿的基本情况、胆道闭锁的类型、肝脏纤维化的程度都是影响Kasai手术效果的关键因素。术后需长期随访，联合熊去氧胆酸、抗生素及营养支持以延缓肝纤维化。

Congenital biliary atresia (CBA) is a hepatobiliary system disease caused by intrahepatic/extrahepatic biliary atresia, which is the main pathogenesis of obstructive jaundice in infants. The etiology of CBA remains unknown, which may be associated with genetic variation, virus infection and immune damage. Targeted preventive measures are lacking. At present, the core of diagnosis and treatment lies in early diagnosis and surgical intervention. However, multiple challenges remain to be resolved in clinical practice. CBA infants are manifested with persistent jaundice, or yellow discoloration of the skin and sclera recurring after physiological jaundice subsides, accompanied by dark color urine and even dark tea-colored urine. The stool color became lighter, even appearing greyish-white color. Laparoscopic or open bile duct exploration is currently considered as the "gold standard" for clinical diagnosis of CBA. In recent years, use of neonatal stool colorometric scale and novel biomarkers (such as serum matrix metalloproteinase 7) have improved the early detection rate. Kasai portoenterostomy procedure is the optimal treatment for CBA, and the ideal timing is within 45 days after birth. Baseline status, type of biliary atresia and degree of liver fibrosis of CBA infants are key factors to determine the efficacy of Kasai procedure. Long-term postoperative follow-up combined with ursodeoxycholic acid, antibiotics and nutritional support can prevent the progression of liver fibrosis.