Objective To explore the clinical presentation and diagnosis and treatment of prehepatic portal hypertension （PPH） and discuss its surgical strategies. Methods Forty-six cases of PPH treated in the 2nd Artillery General Hospital and Peking Union Medical College Hospital from January 2000 to May 2009 were analyzed retrospectively, including 2 cases of Abernethy abnormality. All patients were evaluated by indirect portal vein angiography, CT angiography and (or) portal duplex system Doppler ultrasonography before treament. Surgical strategies included: 23 cases with meso-caval shunt, 8 cases with splenectomy and spleno-renal vein shunt, 1 case with porta-caval shunt, 2 cases with paraumbilical vein-jugular vein shunt, 3 cases with portal azygous disconnection, 1 cases with splenectomy and portal azygous disconnection, 1 case with sigmoidostomy and closed the fistula of sigmoid six months later, 1 case with resection of part of small intestine due to acute extensive thrombosis of portal vein system, 4 cases with selective superior mesenteric artery and (or) splenic artery thrombolytic infusion therapy, 2 cases remained no-surgical option and underwent conservative treatment. Results Forty-four patients were followed-up from 2 months to 5 years， average of 23.4 months, one patient without surgical treatment was lost. Satisfactory outcomes were obtained in 34 patients with various shunts, which expressed as a release of hypersplenism and gastrointestinal hemorrhage. Two cases were treated with meso-caval shunt because of rehemorrhage in month 13 and 24 and one died in month 8 after disconnection, one died on day 40 after thrombolytic therapy due to putrescence of intestines, one who remained no-surgical option underwent hemorrhage 4 months later, and then went well by conservative treatment. Conclusion The key of treatment of PPH is to reduce the pressure of hepatic portal vein. Surgical managements of shunt and selective superior mesenteric artery and (or) splenic artery thrombolytic infusion therapy are safe and effective, but individual treatment strategy should be performed.
目的 探讨Abernethy畸形致下消化道出血的诊断及治疗方法。方法 回顾1例Abernethy畸形患者的诊治情况，并结合相关文献进行分析。结果 血管造影确诊后经积极术前准备，行剖腹探查、乙状结肠造瘘术，术后3周开始给予中药保留灌肠至术后7周，6个月关闭造瘘,3周后继续辅以中药保留灌肠4周。患者顺利恢复出院，随访9个月未再次出血。结论 正确的诊断及合理的治疗方案是救治的关键。
目的 提高对儿童Abernethy畸形的认识。 方法 归纳总结2001年1月－2012年11月我国文献报道的13例儿童Abernethy畸形(即先天性肝外门腔分流，临床分为Ⅰa型、Ⅰb 型和Ⅱ型)的临床表现和治疗方法。 结果 13例儿童Abernethy畸形中，11例Ⅰ型（Ⅰa型4例、Ⅰb型7例）和2例Ⅱ型患儿，1岁内发病6例，确诊前病程≥5年6例。在11例Ⅰ型患儿中，便血3例，呕血4例，肝功能异常4例，肝硬化1例，肝性脑病2例，肝脏结节3例，脾脏肿大4例，脾功能亢进3例，门脉高压3例，合并畸形3例。在2例Ⅱ型患儿中，便血1例，肝功能异常伴肝硬化、脾脏肿大和脾功能亢进1例，肝脏结节1例；无呕血和合并畸形。门脉系统血液分流到下腔静脉2例、髂内静脉4例、左肾静脉1例、奇静脉1例、右心房2例和盆静脉丛1例，其余2例不详。患儿多采用保守治疗，如有巨脾、便血或呕血，脾脏切除和结扎乙状结肠周围血管手术也有采用。 结论 Abernethy畸形以Ⅰ型患儿为主，发病年龄较早，临床表现不具特异性。诊断依赖于影像学检查，螺旋CT血管造影对诊断有较高价值。个体化治疗应根据畸形类型和病情而定。