探讨儿童门静脉海绵样变性（CTPV）特征性侧枝循环代偿路径的临床特点和肠系膜上静脉-门静脉左支搭桥（Rex术）的治疗效果。

回顾性分析2018年5月至2025年3月在湖南省儿童医院行Rex术的36例CTPV患儿。患儿家属均签署知情同意书，符合医学伦理学规定。其中男15例，女21例；年龄1.0~15.0岁，中位年龄4.6岁。原发性CTPV 33例，继发性CTPV 3例。临床表现为全血细胞减少3例，全血细胞减少合并脾大14例，消化道出血18例，无任何临床症状1例。术前腹部超声、腹部增强CT检查，明确诊断为CTPV，术前实施楔入法经肝静脉逆行门静脉造影（WHVP）评估肝内外门静脉发育情况。采用门静脉脱鞘吻合技术及个体化血管移植物选择策略实施经典Rex术和改良Rex术。术后规范抗凝及随访管理。观察患儿Rex手术情况及疗效。术前和术后的肝外门静脉压差比较采用t检验。

36例患儿Rex术均获得成功，其中经典Rex术28例，改良Rex术8例。肝外门静脉压差由术前（30.4±2.6） mmHg（1 mmHg=0.133 kPa）降至术后（17.4±1.1） mmHg（t=30.66，P<0.001）。2例患儿术后乳糜漏，加强营养、抗感染和通畅引流后治愈。其余患儿术后恢复顺利出院，复查CT三维重建示门静脉通畅。随访时间5~84个月，中位随访时间26个月。4例术后出现搭桥血管栓塞，其中2例行了二次Rex术，分别获取患儿颈内静脉和肠系膜下静脉作为血管移植物；其余2例门诊随访观察。3例患儿术后发生搭桥血管狭窄，其中2例行介入下球囊扩张术，1例行血管支架植入术。34例患儿门静脉高压相关症状在半个月内明显缓解并消失，其余2例Plt和WBC未见恢复正常，但未发生食管下段胃底曲张静脉出血。

Rex术能有效重建CTPV患儿的门静脉入肝血流，明显降低肝外门静脉高压。门静脉脱鞘吻合技术及个体化血管移植物选择是手术成功的关键，可明显提高血管吻合成功率及通畅率。

To investigate clinical characteristics of collateral circulation compensation pathway and evaluate clinical efficacy of meso-rex bypass (Rex shunt) in children with cavernous transformation of portal vein (CTPV).

Clinical data of 36 children with CTPV who underwent Rex shunt in Hunan Children's Hospital from May 2018 to March 2025 were analyzed retrospectively. The informed consents of the guardians of all children were obtained and the local ethical committee approval was received. Among them, 15 patients were male and 21 female, aged from 1.0 to 15.0 years, with a median age of 4.6 years. 33 children were diagnosed with primary CTPV and 3 cases of secondary CTPV. Regarding clinical manifestations, 3 cases had pancytopenia, 14 cases of pancytopenia complicated with splenomegaly, 18 cases of gastrointestinal bleeding, and 1 case without any symptoms. The diagnosis of CTPV was confirmed by preoperative abdominal ultrasound and enhanced CT scan. Preoperative wedged hepatic venous portography (WHVP) was performed to evaluate the development of intra-and extra-hepatic portal veins. Classical and modified Rex shunts were performed by portal vein sheath-free anastomosis and individualized vascular graft selection. Standardized anticoagulation and follow-up management were given after surgery. Children's conditions and surgical efficacy of Rex shunts were observed. Extra-hepatic portal vein pressure before and after Rex shunts was compared by t-test.

36 children successfully underwent Rex shunts, including 28 cases of classic Rex shunts and 8 cases of modified Rex shunts. Postoperative extra-hepatic portal vein pressure was decreased from (30.4±2.6) mmHg (1 mmHg=0.133 kPa) before surgery to (17.4±1.1) mmHg (t=30.66, P<0.001). Two children developed postoperative chylous leakage, which were cured after nutritional support, anti-infection and proper drainage. The remaining children were discharged after recovery. Three-dimensional CT reconstruction showed the patency of the portal veins. The follow-up duration was 5-84 months, with a median follow-up of 26 months. 4 children developed postoperative bypass graft embolization, and 2 of them underwent secondary Rex shunts, using internal jugular vein and inferior mesenteric vein as vascular grafts. The other 2 cases were observed during outpatient follow-up. 3 children were complicated with postoperative bypass graft stenosis, 2 of them received interventional balloon dilatation and 1 underwent stent implantation. In 34 children, the symptoms of portal hypertension were significantly relieved and absent within half a month. In the remaining 2 cases, Plt and WBC were not restored to normal, and no variceal bleeding in the lower esophageal fundus was observed.

Rex shunt can effectively reconstruct portal vein blood flow into the liver in children with CTPV, and significantly reduce extra-hepatic portal hypertension. Portal vein sheath-free anastomosis technique and individualized vascular graft selection determine surgical success, which evidently improve the success rate and patency rate of vascular anastomosis.