小儿先天性动静脉瘘的诊治

Diagnosis and management of congenital arteriovenous fistula in children

目的 探讨对小儿先天性动静脉瘘 (congenitalarteriovenousfistula ,CAVF)的诊断与治疗。方法 本组共收治 1 5例CAVF患儿 ,男 6例、女 9例 ,年龄 8～ 1 4岁。对全部患儿行多普勒超声 ,动脉造影或MRA检查。发现病变局限者 5例 ,病变范围广泛性者 1 0例 ,病变大多发生于四肢。动脉造影显示 :动静脉之间出现瘘支 ;动脉分支多而紊乱 ,可呈碗蜒扭曲状 ,病变多为弥漫性 ;静脉象早期出现。对 1 1例患儿行手术治疗 ,其中 4例局限性病变者行病灶切除。对 7例病变广泛者行 1或多次减量手术 ,方法是沿动脉主干切除瘘支与病变组织 ,对分流量大又无法切除的病变组织予以缝扎 ,以减少分流量。对 5例患儿行栓塞治疗 (其中 2例为术前栓塞治疗以减少手术出血 )。 1例未治疗。结果 随访 1～ 6年。 5例局限性患儿中 3例治愈 ,2例病情控制。 1 0例广泛性病变中 ,5例病情好转或控制 ,5例病情仍继续发展。结论 影像学检查是CAVF诊断与治疗的依据。CAVF治疗困难。尤其对广泛性病变者 ,无论手术或栓塞治疗疗效均欠理想。但对小儿CAVF应尽早治疗 ,这样可控制病情发展 。

Objective To improve the diagnosis and management of congenital arteriovenous fistula (C AVF) in children. Methods Fifteen children with CAVF (male 6, female 9) ag ing 8～14 years were reviewed. Color Doppler ultrasonography, DSA or MRA were pe rformed on all patients. The lesion was local in five cases and extensive in te n. Most of the lesions were located in the extremities. Arteriography showed ab normal communication between the arterial and venous systems, racemose and exten sive arterial branches, and the venous developed early. Eleven cases were receiv ed operation including lesion resection in 4 local cases, multiple debulking procedure or main artery ligation in 7 cases with extensive lesions. Five cases had embolization therapy of which 2 cases underwent before operation in order to reduce bleeding and one was not managed. Results All cases were followed-up from one to 6 years. Among the 5 localized lesions, 2 cases were under control and 3 cured. Five out of 10 patients with extensive lesions were deteriorative and the other 5 patient s improved. Conclusions Imaging examination is helpful in the diagnosis and treatment of CAVF. The manag ement of CAVF especially the extensive lesions is still difficult. CAVF in chi ldren must be treated as early as possible to control the development of lesions .