胆道闭锁中门静脉板胆管直径与长期预后相关。

Portal plate bile duct diameter in biliary atresia is associated with long-term outcome.

作者信息

Shpoliansky Michael, Tobar Ana, Mozer-Glassberg Yael, Rosenfeld Bar-Lev Michal, Shamir Raanan, Shafir Michal, Gurevich Michael, Waisbourd-Zinman Orith

机构信息

Institute of Gastroenterology, Nutrition and Liver Disease, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

出版信息

Pediatr Surg Int. 2022 Jun;38(6):825-831. doi: 10.1007/s00383-022-05113-2. Epub 2022 Mar 23.

DOI:10.1007/s00383-022-05113-2

PMID:35322291

Abstract

PURPOSE

Kasai portoenterostomy (KPE) is the only treatment currently available for biliary atresia (BA). Age at KPE and surgical experience are prognostic factors for a successful KPE. Here, we aimed to assess whether the size of bile ductules at the porta hepatis during KPE correlates with KPE success and transplant-free survival (TFS).

METHODS

A retrospective analysis of patients diagnosed with BA during 2000-2019. Porta hepatis biopsies were reviewed for diameters of five representative ducts, and a mean ductal diameter (MDD) was calculated. Laboratory values including pre- and postoperative bilirubin levels were analyzed.

RESULTS

The cohort included 77 patients; for 33, ductal plate biopsy was available. KPE was successful in six of eight patients with MDD ≥ 50 µm, and in five of 25 with MDD < 50 µm, p = 0.008, OR = 12.0 (95% CI 1.83-78.3). Ten-year survival with native liver was higher in patients with MDD ≥ 50 µm than in patients with MDD < 50 µm, p < 0.001, HR 0.038 (95% CI 0.007-0.207). Direct bilirubin < 1 mg/dl 3 months post-KPE was associated with improved 2-year post-KPE TFS (27.7% vs. 13.9%, p < 0.0001).

CONCLUSIONS

MDD ≥ 50 µm correlates with KPE success and a higher rate of TFS. Direct bilirubin < 1 mg/dl 3 months post-operation may serve as a marker of successful biliary excretion, and a predictor of 2-year TFS.

摘要

目的

肝门空肠吻合术（KPE）是目前唯一可用于治疗胆道闭锁（BA）的方法。KPE时的年龄和手术经验是KPE成功的预后因素。在此，我们旨在评估KPE期间肝门部胆小管的大小是否与KPE成功及无移植生存率（TFS）相关。

方法

对2000年至2019年期间诊断为BA的患者进行回顾性分析。对肝门部活检组织进行检查，测量五条代表性胆管的直径，并计算平均胆管直径（MDD）。分析包括术前和术后胆红素水平在内的实验室值。

结果

该队列包括77例患者；其中33例有胆管板活检结果。MDD≥50μm的8例患者中有6例KPE成功，MDD＜50μm的25例患者中有5例成功，p = 0.008，OR = 12.0（95%CI 1.83 - 78.3）。MDD≥50μm的患者10年自体肝生存率高于MDD＜50μm的患者，p＜0.001，HR 0.038（95%CI 0.007 - 0.207）。KPE术后3个月直接胆红素＜1mg/dl与KPE术后2年TFS改善相关（27.7%对13.9%，p＜0.0001）。