Chocarro Gloria, Junco Paloma Triana, Dominguez Eva, Amesty María Virginia, Nuñez Cerezo Vanesa, Hernandez Francisco, Murcia Javier, Tovar Juan Antonio, Lopez Santamaria Manuel
Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
Eur J Pediatr Surg. 2016 Feb;26(1):7-12. doi: 10.1055/s-0035-1563402. Epub 2015 Sep 17.
The mesoportal shunt (MPS) and liver transplantation (LT) have changed the scenario of extrahepatic portal vein obstruction (EHPVO) since the MPS, the only "curative" technique, can now be offered in asymptomatic patients and also thrombotic complications of LT have increased the incidence of EHPVO.
A retrospective study of patients undergoing surgery for EHPVO was conducted between 1990 and 2015. An analysis was done for the shunt permeability and clinical evolution over time.
Of the 73 children with EHPVO, 39 were operated (12 posttransplant and 27 idiopathic). The median age at surgery was 9.36 years (range, 1.60-17.42 years). The MPS was the technique of choice; it was offered in 21 patients but eventually could be performed in only 18 (9 posttransplant and 9 idiopathic). The results of MPS were better in idiopathic (just one thrombosis successfully converted into mesocaval bypass). In the MPS after LT (n = 9), six shunts are permeable, two became thrombotic (one patient requiring retransplantation), and one late thrombosis occurred and the patient died eventually because of gastrointestinal bleeding. In the remaining patients (21 out of the 39), MPS was not performed because of intrahepatic cavernoma, portal vein hypoplasia, or insufficient length of vascular graft (especially in left lateral segment graft LT, since the portal vein is usually located in the right upper quadrant). They underwent 10 distal splenorenal shunts (DSRS) (1 posttransplant and 9 idiopathic), 5 proximal splenorenal shunts (PSRS) (1/4), 6 mesocaval shunts (1/5), and 1 modified Sugiura procedure. The results with DSRS have been favorable (one thrombosis, converted into mesocaval bypass). In the PSRS no thrombosis was identified and in the mesocaval shunt one early thrombosis occurred. Posttransplantation group had higher risk of shunt thrombosis, regardless of the surgical technique (chi-square, 0.021). The total increase of platelets after 6 months was not different in MPS as compared with other surgical techniques (analysis of variance, 0.110).
The MPS is the technique of choice in EHPVO for idiopathic thrombosis as well as secondary to LT. Not all cases are favorable for MPS, so the surgeon must consider the possibility of alternative techniques for EHPVO. The results in terms of shunt patency are much better in idiopathic cavernoma that posttransplant patients.
自中门静脉分流术（MPS）出现以来，中门静脉分流术和肝移植（LT）改变了肝外门静脉阻塞（EHPVO）的治疗局面，因为MPS作为唯一的“治愈性”技术，现在可用于无症状患者，而且肝移植的血栓形成并发症增加了EHPVO的发病率。
对1990年至2015年间接受EHPVO手术的患者进行回顾性研究。对分流的通畅性和随时间的临床演变进行分析。
73例EHPVO患儿中，39例接受了手术（12例为肝移植后，27例为特发性）。手术时的中位年龄为9.36岁（范围1.60 - 17.42岁）。MPS是首选技术；21例患者选择了该技术，但最终仅18例得以实施（9例为肝移植后，9例为特发性）。特发性患者的MPS效果更好（仅1例血栓形成成功转变为中腔静脉分流）。肝移植后的MPS（n = 9）中，6例分流保持通畅，2例发生血栓形成（1例患者需要再次肝移植），1例发生晚期血栓形成，患者最终因胃肠道出血死亡。其余患者（39例中的21例）未进行MPS，原因包括肝内海绵状血管瘤、门静脉发育不全或血管移植物长度不足（特别是在左外侧叶移植肝中，因为门静脉通常位于右上象限）。他们接受了10例远端脾肾分流术（DSRS）（1例为肝移植后，9例为特发性）、5例近端脾肾分流术（PSRS）（1/4）、6例中腔静脉分流术（1/5）和1例改良Sugiura手术。DSRS的效果良好（1例血栓形成，转变为中腔静脉分流）。PSRS未发现血栓形成，中腔静脉分流术有1例发生早期血栓形成。无论采用何种手术技术，肝移植后组发生分流血栓形成的风险更高（卡方检验，0.021）。6个月后血小板总数的增加在MPS组与其他手术技术组之间无差异（方差分析，0.110）。
MPS是EHPVO特发性血栓形成以及肝移植后继发性血栓形成的首选技术。并非所有病例都适合MPS，因此外科医生必须考虑EHPVO的替代技术。特发性海绵状血管瘤患者的分流通畅结果比肝移植后患者好得多。
