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目的提高对先天性腹主动脉瘤(AAA)的认识。方法报道2012年11月1日先天性AAA 1例,回顾国内外报道的24例先天性AAA的临床资料。结果患儿男,2岁,以肉眼血尿起病,伴高血压、蛋白尿和反复血小板降低;多层螺旋CT血管成像(MSCTA)见AAA伴左肾动脉狭窄;彩色多普勒超声见AAA累及双侧髂总动脉伴动脉壁间血栓及钙化,左肾萎缩;保守治疗7个月后猝死,临终前头颅CT见脑梗死。回顾分析24例先天性AAA,包括肾下型AAA 15例,肾上型AAA 5例,胸腹部AAA 2例,未具体指明类型2例;产前诊断6例,出生后诊断18例(其中包括新生儿5例和1个月～3岁婴幼儿8例);以腹部搏动性包块起病8例,呕吐4例,呼吸困难2例,腰部疼痛1例,因其他疾病就诊3例;采用血管超声21例,MSCTA 16例,磁共振血管造影9例;13例肾下型AAA接受手术治疗;死于AAA破裂5例,死于心力衰竭2例。结论先天性AAA以肾下型为主,多为婴幼儿,常表现为腹部肿块,确诊该病首选MSCTA,主张行早期个体化手术。
Objective To improve the understanding of congenital abdominal aortic aneurysm (AAA). Methods One case of congenital AAA was reported on November 1, 2012, and the clinical data of 24 cases of congenital AAA reported at home and abroad were reviewed. Results Male, 2 years old, had gross hematuria with hypertension, proteinuria and recurrent thrombocytopenia. MSCT showed AAA with left renal artery stenosis; color Doppler ultrasound showed AAA involvement Bilateral common iliac artery thrombosis and calcification of the wall, left atrophy; conservative treatment of sudden death after 7 months, see the head of brain before death CT See cerebral infarction. Retrospective analysis of 24 cases of congenital AAA, including 15 cases of subtype of AAA, 5 cases of suprarenal AAA, 2 cases of thoracoabdominal AAA, unspecified type 2 cases; prenatal diagnosis in 6 cases, 18 cases were diagnosed after birth (including 5 cases of newborns and 8 cases of infants and children aged from 1 month to 3 years); 8 cases of abdominal pulsatile mass onset, 4 cases of vomiting, 2 cases of dyspnea, 1 case of lumbar pain and 3 cases of other diseases; 21 cases of vascular ultrasound, 16 cases of MSCTA, 9 cases of magnetic resonance angiography; 13 cases of subtype of AAA underwent surgery; 5 died of AAA rupture and 2 died of heart failure. Conclusions Aneurysmal AAA is predominantly subtype of kidney, mostly infants and young children. It often manifests as abdominal mass. MSCTA is the first choice of the disease and advocates early individualized operation.