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A Single-Institution Review of Portosystemic Shunts in Children:An Ongoing Discussion

机译:儿童门体系统分流的单机构研究:正在进行的讨论

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Purpose. Review the safety and long-term success with portosystemic shunts in children at a single institution.Methods. An IRB-approved, retrospective chart review of all children ages 19 and undergoing surgical portosystemic shunt from January 1990–September 2008.Results. Ten patients were identified, 8 females and 2 males, with a mean age of 15 years (range 5–19 years). Primary diagnoses were congenital hepatic fibrosis (5), hepatic vein thrombosis (2), portal vein thrombosis (2), and cystic fibrosis (1). Primary indications were repeated variceal bleeding (6), symptomatic hypersplenism (2), and significant liver dysfunction (2). Procedures performed were distal splenorenal bypass (4), side-to-side portocaval shunt (3), proximal splenorenal shunt (2), and an interposition H-graft portocaval shunt (1). There was no perioperative mortality and only minor morbidity. Seventy percent of patients had improvement of their symptoms. Eighty percent of shunts remained patent. Two were occluded at a median follow-up of 50 months (range 0.5–13.16 years). Two patients underwent subsequent liver transplantation. Two patients died at 0.5 and 12.8 years postoperatively, one from multisystem failure with cystic fibrosis and one from post-operative transplant complications.Conclusions. The need for portosystemic shunts in children is rare. However, in the era of liver transplantation, portosystemic shunts in selected patients with well-preserved liver function remains important. We conclude that portosystemic shunts are safe and efficacious in the control of variceal hemorrhage and symptoms related to hypersplenism.
机译:目的。在单个机构中回顾儿童门体分流术的安全性和长期成功性。经IRB批准的1990年1月至2008年9月间接受手术门体分流术的所有19岁儿童的回顾性图表回顾。确定了10例患者,女性8例,男性2例,平均年龄15岁(范围5-19岁)。主要诊断为先天性肝纤维化(5),肝静脉血栓形成(2),门静脉血栓形成(2)和囊性纤维化(1)。主要适应症为反复曲张静脉出血(6),症状性脾功能亢进(2)和明显的肝功能障碍(2)。进行的程序为远端脾肾旁路(4),侧向门腔分流(3),近端脾肾分流(2)和插入式H移植门腔分流(1)。没有围手术期死亡,只有轻微的发病率。 70%的患者症状有所改善。百分之八十的分流器保持专利。中位随访时间为50个月(范围0.5–13.16年),其中2例被阻塞。两名患者随后进行了肝移植。两名患者在术后0.5和12.8岁时死亡,一名死于多系统性囊性纤维化衰竭,另一名死于术后移植并发症。儿童很少需要进行门体分流术。然而,在肝移植时代,肝功能良好的部分患者的门体分流仍然很重要。我们得出的结论是,门静脉系统分流术在控制静脉曲张出血和脾功能亢进相关症状方面是安全有效的。

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