Losty P D, Lynch M J, Guiney E J
Department of Paediatric Surgery, Our Lady's Hospital for Sick Children, Dublin, Ireland.
Arch Dis Child. 1994 Nov;71(5):437-40. doi: 10.1136/adc.71.5.437.
The long term outcome of 21 children with extrahepatic portal hypertension secondary to portal vein thrombosis managed by surgical intervention was evaluated. Portosystemic shunts, used primarily in nine patients (eight central splenorenal, one mesocaval) after conservative treatment had failed, had no associated mortality and a 56% patency rate. Five of these shunted patients had no further bleeding episodes and did not show encephalopathic impairment. Direct attack procedures-portoazygos operation (four patients) was associated with significant complications, including one fatality. Other direct approaches-oesophageal transection and variceal plication (five patients) had variable outcome. Splenectomy alone (three patients) ameliorated hypersplenism; however, further surgery for recurrent haemorrhage (two patients) was necessary. Endoscopic sclerotherapy controlled recurrent variceal bleeding (three patients) when it became available to the unit. Conservative treatment practised in five children had little success: two patients survived, two died from further haemorrhage, and one was lost to follow up. These results suggest that in centres without endoscopic expertise, and for patients who are sclerotherapy 'failures', surgery can be performed safely and achieve reasonable long term success rates in childhood extrahepatic portal hypertension.
对21例因门静脉血栓形成继发肝外门静脉高压症并接受手术干预的儿童的长期预后进行了评估。主要在9例患者（8例中央脾肾分流术，1例肠系膜上腔静脉分流术）保守治疗失败后采用门体分流术，无相关死亡病例，通畅率为56%。这些接受分流术的患者中有5例没有再次出血事件，也没有出现脑病损伤。直接攻击手术——门奇静脉断流术（4例患者）伴有严重并发症，包括1例死亡。其他直接方法——食管横断术和曲张静脉折叠术（5例患者）的结果不一。单纯脾切除术（3例患者）改善了脾功能亢进；然而，有2例患者因复发出血需要进一步手术。当该科室能够开展内镜硬化治疗时，其控制了3例患者曲张静脉复发出血。5例儿童采用的保守治疗效果不佳：2例患者存活，2例死于再次出血，1例失访。这些结果表明，在没有内镜专业知识的中心，对于硬化治疗“失败”的患者，手术可以安全进行，并在儿童肝外门静脉高压症中取得合理的长期成功率。
