Guérin Florent, Charre Lionel, Jasienski Stéphanie, Duché Mathieu, Franchiabella Stéphanie, Bernard Olivier, Jacquemin Emmanuel, Agostini Hélène, Gauthier Frédéric, Branchereau Sophie
Department of Paediatric Surgery, Hôpitaux Universitaires Paris-Sud: Bicêtre (AP-HP), Le Kremlin Bicêtre, France.
Department of Paediatric Surgery, Hôpitaux Universitaires Paris-Sud: Bicêtre (AP-HP), Le Kremlin Bicêtre, France; Department of Surgery, Centre Hospitalier René Dubos, Pontoise, France.
J Pediatr Surg. 2019 Mar;54(3):531-536. doi: 10.1016/j.jpedsurg.2018.06.023. Epub 2018 Jun 25.
To assess the outcome of patients with biliary atresia (BA) who underwent a surgical shunt (SS) for severe portal hypertension (PH) following a Kasai procedure.
We collected and analyzed the data and outcomes of patients with BA who underwent SS for severe PH following a Kasai procedure between 1974 and 2014, focusing on complications related to the procedure, overall survival (OS), and transplant-free survival (TFS).
SS was performed at a median age of 5.5 years [2-13.5] in 38 patients. Conjugated bilirubin level (cBL) was ≤20 μmol/l in 24 patients at time of SS. Median follow-up was 15 years [1-32]. OS at 5 and 10 years was 91% and 87% respectively. TFS at 5 and 10 years was 84% and 70% respectively. Long-term complications included hepatic encephalopathy in 9 patients, and hepatopulmonary syndrome in 3. At last follow-up, 10/14 patients without LT and 18/ 24 who had a delayed LT at a median delay of 11 years [1.5-22] were alive.
Surgical shunt for severe portal hypertension in biliary atresia may delay the need for liver transplantation. However complications are indications for transplantation.
Type of study: Therapeutic. Level of evidence III.
评估在接受葛西手术后因严重门静脉高压（PH）而接受手术分流（SS）的胆道闭锁（BA）患者的预后。
我们收集并分析了1974年至2014年间因严重PH在葛西手术后接受SS的BA患者的数据和预后，重点关注与该手术相关的并发症、总生存期（OS）和无移植生存期（TFS）。
38例患者接受SS时的中位年龄为5.5岁[2 - 13.5岁]。SS时24例患者的结合胆红素水平（cBL）≤20μmol/l。中位随访时间为15年[1 - 32年]。5年和10年的OS分别为91%和87%。5年和10年的TFS分别为84%和70%。长期并发症包括9例肝性脑病和3例肝肺综合征。在最后一次随访时，14例未接受肝移植的患者中有10例存活，24例延迟肝移植的患者中有18例存活，中位延迟时间为11年[1.5 - 22年]。
胆道闭锁严重门静脉高压的手术分流可能会延迟肝移植的需求。然而，并发症是移植的指征。
研究类型：治疗性。证据水平III。
