目的:儿童非肝硬化肝外门静脉阻塞(EHPVO)导致的门静脉高压主要通过Meso-Rex旁路术进行治疗,但只有少数病人有一个可行的雷克斯直肠,需要手术。本研究报告了一系列在门静脉再通(PVR)进行介入放射学尝试的患者,专注于技术方面和安全。
方法:自2022年以来,在单个机构中连续对由于非肝硬化EHPVO引起的严重门静脉高压症患者进行了回顾性回顾,这些患者在PVR中进行了经皮尝试,已执行。技术和临床数据,包括透视时间,辐射暴露,技术和临床成功,记录并发症和随访情况.
结果:11例患者(男6例,女5例;中位年龄7岁,范围1-14)接受了15次经皮肝穿(n=1),跨脾(n=11),或同时经肝/经脾(n=3)手术。雷克斯recesssus在4/11(36%)获得专利。荧光镜检查导致每个程序的123Gycm2(范围17-788Gycm2)的高中位总剂量面积乘积(DAP)。5/11例患者(45%)获得PVR,3/5与消除雷克斯衰退。发生2级和3级两个不良事件,无后遗症。血管成形术后,4/5患者需要支架置入以获得持续通畅,在中位随访6个月(范围6-14)后,所有PVR的彩色多普勒超声检查均显示。
结论:我们的初步经验表明,45%的非肝硬化EHPVO患儿即使在雷克斯闭经时也能恢复门静脉血流。在非肝硬化EHPVO中,PVR可能是一种选择,如果Meso-Rex旁路不可行,尽管辐射暴露值得关注。
结论:在不符合Meso-Rex搭桥手术条件的儿童中,创新的经皮手术可能是传统手术方法的替代选择。
结论:传统上,儿童非肝硬化门静脉高压症通过Meso-Rex旁路手术治疗。经皮PVR可以恢复原生门静脉系统的通畅,即使在雷克斯直肠被切除和手术被排除的情况下也是如此。当Meso-Rex旁路术不可行时,介入放射技术可以在儿童EHPVO的复杂病例中提供微创解决方案。
OBJECTIVE: Portal hypertension resulting from non-cirrhotic extrahepatic portal vein obstruction (EHPVO) in children has been primarily managed with the Meso-Rex bypass, but only a few patients have a viable Rex recessus, required by surgery. This study reports a preliminary series of patients who underwent interventional radiology attempts at portal vein recanalization (PVR), with a focus on technical aspects and safety.
METHODS: A retrospective review of consecutive patients with severe portal hypertension due to non-cirrhotic EHPVO at a single institution from 2022, who underwent percutaneous attempts at PVR, was performed. Technical and clinical data including fluoroscopy time, radiation exposure, technical and clinical success, complications and follow-up were recorded.
RESULTS: Eleven patients (6 males and 5 females; median age 7 years, range 1-14) underwent 15 percutaneous transhepatic (n = 1), transplenic (n = 11), or simultaneous transhepatic/transplenic (n = 3) procedures. Rex recessus was patent in 4/11 (36%). Fluoroscopy resulted in a high median total dose area product (DAP) of 123 Gycm2 (range 17-788 Gycm2) per procedure. PVR was achieved in 5/11 patients (45%), 3/5 with obliterated Rex recessus. Two adverse events of grade 2 and grade 3 occurred without sequelae. After angioplasty, 4/5 patients required stenting to obtain sustained patency, as demonstrated by colour-Doppler ultrasound in all PVR after a median follow-up of 6 months (range 6-14).
CONCLUSIONS: Our preliminary experience suggests that 45% of children with non-cirrhotic EHPVO can restore portal flow even with obliterated Rex recessus. In non-cirrhotic EHPVO, PVR may be an option, if a Meso-Rex bypass is not feasible, although the radiation exposure deserves attention.
CONCLUSIONS: Innovative percutaneous procedures may have the potential to be an alternative option to the traditional surgical approach in the management of non-cirrhotic EHPVO and its complications in children not eligible for Meso-Rex bypass surgery.
CONCLUSIONS: Non-cirrhotic portal hypertension in children has been traditionally managed by surgery with Meso-Rex bypass creation. Percutaneous PVR may restore the patency of the native portal system even when the Rex recessus is obliterated and surgery has been excluded. Interventional radiological techniques may offer a minimally invasive solution in complex cases of EHPVO in children when Meso-Rex bypass is not feasible.