Background and aims

Portoenterostomy is the initial surgical treatment for biliary atresia (BA); however, no curative therapy exists for BA. Varix bleeding is a major complication of end-stage liver disease and must be determined in patients with BA, necessitating routine surveillance using esophagogastroduodenoscopy (EGD). We attempted to validate criteria to identify BA patients requiring EGD.

Methods

From January 2007 to December 2017, we selected BA patients who underwent Kasai surgery, transient elastography (TE), and EGD at Severance hospital. In total, 190 cases were included; laboratory tests and EGDs were carried out from 3 months before TE to 3 months after TE.

Results

Based on the cut-off value (< 10) of the liver stiffness measurement (LSM), 35 (81.4%) patients with low-risk varix (LRV) and 8 (18.6%) with high-risk varix (HRV) were identified. Based on platelet counts (> 150,000), 87 (77.68%) patients with LRV and 25 (22.32%) with HRV were identified. Based on this, the BAVENO VI criteria, which identify patients who can safely avoid screening EGD, missed 9/68 (13.24%) of HRV patients. The expanded BAVENO VI criteria missed 21/68 (30.88%) of HRV patients. However, the criteria using LSM <10 and platelet count > 150,000 missed identifying only 4/68 (5.88%) HRV patients.

Conclusions

The BAVENO criteria may be as useful in children with BA as in adults with liver cirrhosis. Regular laboratory tests, imaging studies, and EGD may avoid missing diagnoses of varices in BA patients. However, LSM < 10 and platelet count > 150,000 may provide more accurate criteria and help identify patients who does not need endoscopy.

中文翻译：

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验证 BAVENO VI 标准以识别没有内窥镜检查食管静脉曲张的低风险胆道闭锁患者。

背景和目标

肠门吻合术是胆道闭锁 (BA) 的初始手术治疗；然而，BA 尚无治愈性疗法。静脉曲张出血是终末期肝病的主要并发症，必须在 BA 患者中确定，需要使用食管胃十二指肠镜 (EGD) 进行常规监测。我们试图验证识别需要 EGD 的 BA 患者的标准。

方法

从 2007 年 1 月到 2017 年 12 月，我们选择了在 Severance 医院接受 Kasai 手术、瞬时弹性成像 (TE) 和 EGD 的 BA 患者。共纳入190例；从 TE 前 3 个月至 TE 后 3 个月进行实验室检查和 EGD。

结果

根据肝脏硬度测量 (LSM) 的临界值 (< 10)，确定了 35 (81.4%) 名低危静脉曲张 (LRV) 患者和 8 名 (18.6%) 高危静脉曲张 (HRV) 患者. 根据血小板计数 (> 150,000)，确定了 87 (77.68%) 名 LRV 患者和 25 (22.32%) 名 HRV 患者。基于此，识别可以安全避免筛查 EGD 的患者的 BAVENO VI 标准漏掉了 9/68 (13.24%) 的 HRV 患者。扩大的 BAVENO VI 标准遗漏了 21/68 (30.88%) 的 HRV 患者。然而，使用 LSM <10 和血小板计数 > 150,000 的标准未能识别仅 4/68 (5.88%) HRV 患者。

结论

BAVENO 标准对 BA 儿童和肝硬化成人同样有用。定期实验室检查、影像学检查和 EGD 可以避免漏诊 BA 患者静脉曲张的诊断。然而，LSM < 10 和血小板计数 > 150,000 可能会提供更准确的标准并有助于识别不需要内窥镜检查的患者。