儿童先天性腹主动脉瘤一例并文献回顾

Congenital Abdominal Aortic Aneurysm in Children: A Case Report and Literature Review

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 acquire more knowledge about congenital abdominal aortic aneurysms （AAA）. Methods A 2-year-old boy with congenital AAA diagnosed on Novermber 1 st, 2012, was presented. The clinical features and its treatment of other 24 cases with congenital AAA reported previously at home and abroad were reviewed. Results The boy was admitted with gross hematuria and gradually presented with hypertension, proteinuria and recurrent thrombocytopenia. Multi-slice spiral CT angiography （MSCTA） revealed an AAA with left renal artery stenosis. Color Doppler ultrasound showed that the AAA expanded to bilateral common iliac artery with mural thrombus and calcification formation and that the left kidney was atrophy. The boy was only treated with conservative medical therapy. Seven months later, the boy died suddenly. Before his death, cerebral infarction was found through cranial CT. In other 24 cases with congenital AAA, which were 6 fetus and 18 patients （including 5 neonates and 8 infants）, 15 were with infrarenal AAA type, 5 were with juxtarenal AAA type, 2 were thoracoabdominal AAA, and others were unspecified AAA. These 18 patients diagnosed after birth presented with abdominal mass （n=8）, vomiting （n=4）, dyspnea （n=2） and lumbago （n=l）, and 3 patients were found during treating other disorders. Imaging studies such as vascular ultrasound （n=21）, MSCTA （n=16） and magnetic resonance angiography （n=9） were used in diagnosing congenital AAA. Thirteen patients with infrarenal AAA underwent surgery, others received conservative medical treatment. Five patients died of ruptured aneurysms, and 2 patients died of heart failure. Conclusions Congenital AAA mainly occurs in infants and infrarenal type of congenital AAA is most common. Abdominal pulsatile mass is most common clinical manifestations. MSCTA is a preferable tool to confirm the diagnosis. Early individualized surgery is advocated to treat congenital AAA.