Angelico R, Pietrobattista A, Candusso M, Tomarchio S, Pellicciaro M, Liccardo D, Basso M S, Grimaldi C, Saffioti M C, Torroni F, Dall'Oglio L, Torre G, Spada M
Division of Abdominal Transplantation and Hepatobiliopancreatic Surgery, Bambino Gesù Children's Research Hospital IRCCS, Rome, Italy.
Division of Hepatology and Gastroenterology, Bambino Gesù Children's Research Hospital IRCCS, Rome, Italy.
Transplant Proc. 2019 Jan-Feb;51(1):171-178. doi: 10.1016/j.transproceed.2018.04.074. Epub 2018 Jun 28.
Cirrhosis for biliary atresia (BA) is associated with risk of gastrointestinal bleeding (GB) from gastroesophageal varices due to portal hypertension. Primary prophylaxis of GB is controversial in children who are candidates for liver transplantation (LT). The aim of the study was to define the management of gastroesophageal varices and to identify the benefit of primary prophylaxis for GB in BA children waiting for LT.
A retrospective single-center study including all BA children listed for LT in 2008-2016. Clinical, endoscopical, and biochemical data were analyzed.
Of 82 children, 50 (61%) did not receive primary prophylaxis and did not present any episode of bleeding, 16 (19.5%) underwent primary prophylaxis, and 16 (19.5%) presented spontaneous GB and received secondary prophylaxis. Children without primary prophylaxis and GB were younger than patients with primary prophylaxis and those with GB (7.7 years [range, 4.1-37.9 years] vs 11.2 years [range, 5.1-43 years]; P = .03 vs 10.7 years [range, 6.9-39.9 years], respectively; P = .004). Seventy-five percent of GB occurred in children older than 8 months. Fifteen (93.8%) children with GB presented esophageal varices (grade III = 10 [62.5%]) and 10 (62.5%) required endoscopic treatments, consisting mainly of sclerotherapy. Median time to LT was similar for children with or without bleeding (2 months [range, 0-17.7 months] vs 2.2 months [0-17.9 months], respectively; P = .89). After 45.5 months (range, 13.7-105.5 months) of follow-up, the overall patient survival was 97.6%. At the intention-to-treat analysis, the survival rate was 100% for patients without bleeding episode and 87.5% for children with GB (P = .16).
Despite the risk of GB being not clinically predictable in children with BA waiting for LT, our experience suggests that primary prophylaxis of GB might be unnecessary in children younger than 6 months, while it should be considered in older children. Thus, the occurrence of GB does not delay the timing of transplantation.
胆道闭锁（BA）所致肝硬化与门静脉高压引起的胃食管静脉曲张导致的胃肠道出血（GB）风险相关。对于等待肝移植（LT）的儿童，GB的一级预防存在争议。本研究的目的是确定胃食管静脉曲张的管理方法，并确定GB一级预防对等待LT的BA患儿的益处。
一项回顾性单中心研究，纳入了2008年至2016年所有登记等待LT的BA患儿。对临床、内镜和生化数据进行分析。
82例患儿中，50例（61%）未接受一级预防且未发生任何出血事件，16例（19.5%）接受了一级预防，16例（19.5%）发生自发性GB并接受了二级预防。未接受一级预防且未发生GB的患儿比接受一级预防的患儿以及发生GB的患儿年龄更小（7.7岁[范围4.1 - 37.9岁] vs 11.2岁[范围5.1 - 43岁]；P = 0.03 vs 10.7岁[范围6.9 - 39.9岁]，P = 0.004）。75%的GB发生在8个月以上儿童中。15例（93.8%）发生GB的患儿存在食管静脉曲张（III级 = 10例[62.5%]），10例（62.5%）需要内镜治疗，主要为硬化治疗。有出血和无出血患儿等待LT的中位时间相似（分别为2个月[范围0 - 17.7个月] vs 2.2个月[0 - 17.9个月]；P = 0.89）。随访45.5个月（范围13.7 - 105.5个月）后，总体患者生存率为97.6%。在意向性分析中，无出血事件患者的生存率为100%，发生GB的患儿为87.5%（P = 0.16）。
尽管等待LT的BA患儿GB风险在临床上难以预测，但我们的经验表明，6个月以下儿童可能无需进行GB一级预防，而年龄较大儿童应考虑进行。因此，GB的发生并不延迟移植时机。
