Huppert P E, Astfalk W, Brambs H J, Schweizer P, Schott U, Pereira P, Duda S H, Dopfer R E, Claussen C D
Abteilung für Radiologische Diagnostik, Eberhard-Karls-Universität Tübingen.
Rofo. 1998 Jun;168(6):595-603. doi: 10.1055/s-2007-1015286.
To present special methodical and clinical findings of transjugular intrahepatic portosystemic shunts (TIPSS) in children and to discuss potential indications.
Between 1993 and 1996, 6 children aged 2-13 years were treated by TIPSS-insertion. In four cases, the underlying disease was extrahepatic biliary atresia (EHBA) and in two cases liver fibrosis secondary to treatment of neoplasms during early childhood. Indications for TIPSS insertion were variceal bleeding resistant to other treatment modalities in three patients, hypersplenism in one patient and both bleeding and hypersplenism in two. Portal vein punctures were performed using 16-gauge needles, because 19-gauge fine-needles showed insufficient stiffness. The mean follow-up was 24.5 months.
Shunt insertion succeeded in all children with a mean procedure time of 5.2 hours. Periportal fibrosis associated with EHBA, atypical course of hepatic veins and small diameters and distances of vessels were conditions making the procedure difficult. Bleeding ceased in all patients, peripheral platelet counts rose by a mean value of 58%. Procedure-related complications were minor extrahepatic bleeding in one child and temporary haemolysis in another child. Restenosis resulted in three patients and were treated successfully by means of transjugular interventions. 5 children remain free of symptoms to this day, one child underwent successful orthotopic liver transplantation 8 months after TIPSS.
TIPSS insertion is technically more difficult in children and has to consider child growth and possible subsequent liver transplantation. Potential indications are recurrent variceal bleeding, also of intestinal origin, resistant to standard treatment and clinically significant hypersplenism.
介绍儿童经颈静脉肝内门体分流术（TIPSS）的特殊方法学和临床发现，并讨论潜在适应证。
1993年至1996年间，6名年龄在2至13岁的儿童接受了TIPSS植入治疗。4例潜在疾病为肝外胆道闭锁（EHBA），2例为幼儿期肿瘤治疗后继发的肝纤维化。3例患者TIPSS植入的适应证是对其他治疗方式耐药的静脉曲张出血，1例是脾功能亢进，2例是出血合并脾功能亢进。由于19号细针刚度不足，门静脉穿刺使用16号针。平均随访时间为24.5个月。
所有儿童分流植入均成功，平均手术时间为5.2小时。与EHBA相关的门周纤维化、肝静脉的非典型走行以及血管直径小和距离短是使手术困难的情况。所有患者出血停止，外周血小板计数平均升高58%。与手术相关的并发症为1例儿童发生轻微肝外出血，另1例儿童发生暂时性溶血。3例患者出现再狭窄，通过经颈静脉介入治疗成功。5名儿童至今无症状，1名儿童在TIPSS术后8个月接受了成功的原位肝移植。
儿童TIPSS植入在技术上更困难，必须考虑儿童生长及可能随后的肝移植。潜在适应证为复发性静脉曲张出血（包括肠道来源且对标准治疗耐药者）以及具有临床意义的脾功能亢进。
