摘要
目的总结肝脏遗传性出血性毛细血管扩张症（HHHT）的诊断和治疗经验。方法回顾性分析2002年1月至2010年9月山东大学齐鲁医院、山东省临沂市蒙阴县人民医院、山东省聊城市人民医院、河南省人民医院、河北医科大学第二医院、浙江大学医学院附属第一医院收治的15例HHHT患者的临床资料。总结该病的临床表现、影像学及实验室检查，探讨该病的诊断、治疗和预后。结果HHHT患者早期无特异性临床表现，多表现为上腹痛、气短、贫血、肝区杂音等，但病情可迅速进展并出现门静脉高压症、肝硬化等并发症；彩超、CT检查示肝内毛细血管扩张、动静脉瘘、肝动脉瘤；数字减影血管造影检查对多支肝动脉的HHHT患者，血管显影欠佳，而CT血管造影检查则显示清晰。15例患者根据病变程度和发病阶段被分为无症状型、简单型和复杂型，针对不同患者采用个体化治疗。6例患者采用手术治疗，其中5例接受肝动脉结扎或缩扎手术，随访效果满意。3例患者采用介入治疗，其中1例复杂型患者介入栓塞失败，药物治疗30个月后死亡。6例患者采用内科保守治疗，2例起始无症状，21个月、35个月后分别出现肝功能异常、腹腔积液等，其中1例死亡；4例仅行药物治疗的患者，随访示肝脏病变逐渐加重。结论依据肝内毛细血管扩张、动静脉瘘、肝动脉瘤三大特征，影像学检查具有重要诊断意义；HHHT为进展性疾病，早期、积极的个体化治疗具有较好的临床效果；保留肝周韧带及胆道供血的肝动脉结扎或缩扎手术疗效满意。
Objective To summarize the experiences in the diagnosis and treatment of the hepatic hereditary hemorrhagic telangiectasia （HHHT）. Methods The clinical data of 15 HHHT patients who were admitted to the Qilu Hospital, People＇s Hospital of Mengyin, People＇s Hospital of Liaoeheng, Henan Provincial People＇s Hospital, the Second Hospital of Hebei Medical University, First Affiliated Hospital of Zhejiang University were retrospectively analyzed. The clinical manifestation, features of imaging and laboratory examination were summarized, and the diagnosis, treatment and prognosis of the disease were investigated. Results HHHT patients had nonspecific symptoms in the early stage, and some patients presented with right upper quadrant discomfort, shortness of breath, anemia and liver bruit. The condition of HHHT patients could be worsened by liver cirrhosis or portal hypertension rapidly. The results of color doppler ultrasound and computed tomography showed intrahepatic telangiectasia, arteriovenous fistula and hepatic artery aneurysm in the 15 patients. Digital subtraction angiography was not clear enough for 2 HHHT patients with more than 1 enlarged hepatic arteries, but computed tomographic angiography was feasible. According to the degree and stages of the HHHT, all the 15 patients were divided into asymptomatic HHHT, simple HHHT and complex HHHT. Among the 6 patients who underwent surgical treatment, 5 received ligation or banding of the enlarged hepatic arteries with subsequent disappearance of symptoms. Three patients received interventional treatment, and the treatment for 1 patient with complex HHHT was failed, and the patient died 30 months after medical treatment. Six patients were treated by conservative treatment, 2 patients of them had no symptoms at the beginning, then they suffered from hepatic dysfunction and aseites at 21 and 35 momhs, respectively, and 1 of thenl died 6 mouths later. Four patients received medical treatment, and tile results of color doppler ultrasound and computed tomography showed the pathological changes were aggravated gradually. Conclusions Telangieetasia, intrahepatie arteriovenous fistula and hepatic artet7 aneurysm are the main imaging eharacteristies of HHHT, and imaging diagnosis has significant value in the diagnosis of HHHT. HHItT is a progressive disease, early, active and iudividualized treatmmlt is beneficial to the patients. The outcome of ligation or banding of the hepatic arteries is satisfatctory.
出处
《中华消化外科杂志》
CAS
CSCD
北大核心
2012年第6期566-569,共4页
Chinese Journal of Digestive Surgery
关键词
肝脏疾病
毛细血管扩张
遗传性出血性
诊断
治疗
Liver diseases
Telangieclasia, hereditatT hemorrhagic
l）iagnosis
Therapy
