Lemoine Caroline, Lokar Joan, McColley Susanna A, Alonso Estella M, Superina Riccardo
Division of Transplant Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Division of Pulmonary Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
J Pediatr Surg. 2019 May;54(5):1076-1082. doi: 10.1016/j.jpedsurg.2019.01.035. Epub 2019 Feb 2.
The management of portal hypertension (PHT) in children with well compensated cirrhosis and cystic fibrosis (CF) is controversial. We present our experience with distal splenorenal shunting (DSRS) for the treatment of PHT as an alternative to liver transplantation (LT).
Between 2008 and 2017, 5 CF children underwent a DSRS at a pediatric hepatobiliary and transplantation referral center. LT (n = 9) was reserved for patients with decompensated cirrhosis. Statistical analysis was done using the paired t-test (p < 0.05 considered significant).
Mean PELD/MELD score was significantly lower for DSRS patients than LT (3 ± 6 vs 28 ± 4, p < 0.001). All 5 DSRS patients had grade III-IV varices. One bled prior to surgery. After DSRS, spleen size decreased significantly from 8.4 ± 1.5 cm to 4.4 ± 1.8 cm (p = 0.019). Mean platelet count remained stable (87.8 ± 48 to 91.8 ± 35, p = 0.9). There were no postoperative complications. No DSRS patient experienced variceal bleeding following shunt creation. Liver function tests remained stable in the DSRS group, and no patient required a liver transplant (median follow up 4.65 years, range 1.24-7.79).
Patients with cystic fibrosis who have well-compensated cirrhosis and symptomatic portal hypertension can be palliated with distal splenorenal shunting and do not need liver transplants. These patients can undergo shunting with minimal morbidity.
Case series with no comparison group.
IV.
对于代偿良好的肝硬化合并囊性纤维化（CF）患儿的门静脉高压（PHT）管理存在争议。我们介绍了我们应用远端脾肾分流术（DSRS）治疗PHT作为肝移植（LT）替代方案的经验。
2008年至2017年间，5例CF患儿在一家儿科肝胆及移植转诊中心接受了DSRS。LT（n = 9）用于失代偿性肝硬化患者。采用配对t检验进行统计分析（p < 0.05认为有统计学意义）。
DSRS患者的平均PELD/MELD评分显著低于LT组（3±6 vs 28±4，p < 0.001）。所有5例DSRS患者均有III-IV级静脉曲张。1例在手术前出血。DSRS术后，脾脏大小从8.4±1.5 cm显著降至4.4±1.8 cm（p = 0.019）。平均血小板计数保持稳定（87.8±48至91.8±35，p = 0.9）。无术后并发症。分流术后无DSRS患者发生静脉曲张出血。DSRS组肝功能检查保持稳定，无患者需要肝移植（中位随访4.65年，范围1.24 - 7.79）。
代偿良好的肝硬化合并有症状门静脉高压的囊性纤维化患者可通过远端脾肾分流术得到缓解，无需肝移植。这些患者可接受分流术，且发病率极低。
无对照组的病例系列。
IV级。
