Broto J, Infante D, Tormo R, Marhuenda C, Gil Vernet J M, Boix-Ochoa J
Departamento de Cirugía Pediátrica, H. Universitario Materno-Infantil Vall d'Hebron, Barcelona.
Cir Pediatr. 1995 Jul;8(3):99-101.
The authors present 14 patients affected with portal cavernoma, diagnosed and controlled over the last 12 years. The splenomegaly (60%) and the digestive hemorrhages (40%) were the initial study causes. The cavernoma appeared idiopathic in 10 patients or secondary either to a catheterization of umbilical vein (3 cases) or onfalitis (1 case). The first clinical manifestation oscillated between the ages of 3 months to nine years. The diagnosis was performed in the initial cases (4 cases) using splenoportography (3 cases) and superior mesenteric arteriography (SMA) in the other. In the remaining cases the ultrasonography together with the endoscopic evidence of the varies backed up the diagnosis. Before the intervention, a SMA was performed in all cases. The surgical decision was taken with regards to the persistence of the digestive bleeding after the start of medical treatment with propranolol, cimetidine, and sucralfate, along with the existence of dangerous varices. Eight patients were operated on, two by the Warren technique, four by mesocaval jugular graft interposition (MJI) and two by azigo portal disconnection (APD). The shunts initially controlled the bleeding in all cases. There was one rebleeding instance at eleven months in a patient operated on by MJI, and in another by APD. Four patients continue medical treatment without problems, and two are free of treatment. Our experience with varies sclerosis is limited to two cases, although since the beginning of the treatment, one year ago, neither of them has bled.
作者报告了过去12年中确诊并接受治疗的14例门静脉海绵样变性患者。脾肿大(60%)和消化道出血(40%)是最初的研究病因。10例患者的海绵样变性为特发性,3例继发于脐静脉插管,1例继发于脐炎。首发临床表现出现在3个月至9岁之间。最初的4例病例通过脾门静脉造影(3例)和肠系膜上动脉造影(SMA)进行诊断,其他病例则通过SMA诊断。其余病例通过超声检查以及不同内镜检查结果辅助诊断。在进行干预之前,所有病例均进行了SMA检查。在使用普萘洛尔(心得安)、西咪替丁和硫糖铝进行药物治疗后,若消化道出血持续存在且存在危险的静脉曲张,则做出手术决定。8例患者接受了手术,2例采用沃伦技术,4例采用肠系膜上腔静脉-颈内静脉搭桥术(MJI),2例采用奇静脉-门静脉断流术(APD)。分流术最初在所有病例中均控制了出血。1例接受MJI手术的患者和1例接受APD手术的患者在术后11个月出现了再次出血。4例患者继续接受药物治疗,无任何问题,2例患者无需治疗。我们在静脉曲张硬化治疗方面的经验仅限于2例,尽管自1年前开始治疗以来,这2例患者均未出血。