Zhuang XiaoJin, He YuanBin, Wu DianMing, Fang YiFan, Lin Yu, Huang YanBing
Department of Pathology, Fujian Children's Hospital (Fujian Branch of Shanghai Children's Medical Center), College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China.
Department of General Surgery, Fujian Children's Hospital (Fujian Branch of Shanghai Children's Medical Center), College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China.
Front Pediatr. 2025 May 13;13:1595238. doi: 10.3389/fped.2025.1595238. eCollection 2025.
The severity of liver fibrosis and optimal surgical timing in infants with prenatally diagnosed choledochal cysts (CDCs) remain contentious. This study aims to identify risk factors for significant liver fibrosis in prenatally diagnosed CDCs and guide optimal surgical timing.
This retrospective case-control study reviewed infants with prenatally diagnosed CDCs between January 2016 and January 2024. Liver fibrosis was staged (S0-S4) using the Batts-Ludwig system. Infants were categorized into mild/no fibrosis (<S2) and significant fibrosis (≥S2) groups based on histopathology. The aspartate aminotransferase to platelet ratio index (APRI) and Fibrosis-4 index (FIB-4) were validated, and multivariate logistic regression analyses were performed to identify independent risk factors. The receiver operating characteristic (ROC) curve was used to assess diagnostic performance.
A total of 50 infants (20 male, 30 female) were enrolled, with a median gestational age at diagnosis of 28 weeks (range: 19-39 weeks) and a median surgical age of 54 days (range: 7-360 days). Liver fibrosis was present in 40 cases (80%), with 34 cases (68%) classified as <S2 and 16 cases (32%) as ≥S2. Univariate analysis showed that infants with significant liver fibrosis had a higher proportion of clinical symptoms and Type IV CDCs, as well as elevated AST, TBil, DBil, GGT, WBC, and cysts width before surgery ( < 0.05). Multivariate logistic regression analysis identified Type IV CDCs (OR = 11.39, 95% CI: 1.04-124.65) and GGT (OR = 1.003, 95% CI 1.00-1.01) as independent influencing factors ( < 0.05). For diagnosing significant fibrosis (≥S2), GGT demonstrated an area under the ROC curve (AUROC) of 0.86, with an optimal cutoff of 327 U/L (sensitivity: 75%, specificity: 88%). APRI showed an AUROC of 0.76 (95% CI 0.61-0.92, < 0.01) with an optimal cutoff of 0.23 (sensitivity: 75%, specificity: 80%).
Liver fibrosis is common in infants with prenatally diagnosed CDCs, primarily presenting as mild. Type IV CDCs and GGT > 327 U/L are significant risk factors, highlighting the need for close monitoring and timely surgical intervention.
产前诊断为胆总管囊肿（CDC）的婴儿的肝纤维化严重程度及最佳手术时机仍存在争议。本研究旨在确定产前诊断为CDC的婴儿发生显著肝纤维化的危险因素，并指导最佳手术时机。
本回顾性病例对照研究纳入了2016年1月至2024年1月期间产前诊断为CDC的婴儿。采用Batts-Ludwig系统对肝纤维化进行分期（S0-S4）。根据组织病理学将婴儿分为轻度/无纤维化（<S2）和显著纤维化（≥S2）组。对天冬氨酸转氨酶与血小板比值指数（APRI）和纤维化-4指数（FIB-4）进行验证，并进行多因素逻辑回归分析以确定独立危险因素。采用受试者工作特征（ROC）曲线评估诊断性能。
共纳入50例婴儿（男20例，女30例），诊断时的中位孕周为28周（范围：19-39周），中位手术年龄为54天（范围：7-360天）。40例（80%）存在肝纤维化，其中34例（68%）分类为<S2，16例（32%）分类为≥S2。单因素分析显示，有显著肝纤维化的婴儿临床症状、IV型CDC的比例更高，术前AST、TBil、DBil、GGT、WBC及囊肿宽度也更高（P<0.05）。多因素逻辑回归分析确定IV型CDC（OR=11.39，95%CI：1.04-124.65）和GGT（OR=1.003，95%CI 1.00-1.01）为独立影响因素（P<0.05）。对于诊断显著纤维化（≥S2），GGT的ROC曲线下面积（AUROC）为0.86，最佳截断值为327 U/L（敏感性：75%，特异性：88%）。APRI的AUROC为0.76（95%CI 0.61-0.92，P<0.01），最佳截断值为0.23（敏感性：75%，特异性：80%）。
产前诊断为CDC的婴儿肝纤维化常见，主要表现为轻度。IV型CDC和GGT>327 U/L是显著危险因素，强调需要密切监测并及时进行手术干预。
