Diagnosis and treatment of congenital abdominal aortic aneurysm: a systematic review of reported cases (Orphanet Journal of Rare Diseases)

Background: Congenital abdominal aortic aneurysm (AAA) is distinctly rare in infants and children and carries a high mortality rate. Our objective was to summarize the experience of the diagnosis and treatment in patients with congenital AAA. Methods: Reported cases of congenital AAA published prior to November 8, 2014, were identified through PubMed, EMBASE, Web of Science, and reference lists. All selected cases were evaluated for main clinical characteristics. Results: Twenty-six cases of congenital AAA were identified in the English language literature. Congenital AAA occurred primarily in children under three years old, but it was also found in young adults and fetuses. With regards to the localization, the great majority of congenital AAA was infrarenal AAA. The majority of the AAA patients lacked specific symptoms, and a painless pulsatile abdominal mass was the most common clinical presentation. The diagnosis of AAA was based on ultrasound scanning in twenty-five cases, multi-slice spiral computed tomography angiography (MSCTA) in sixteen cases, and magnetic resonance angiography (MRA) in nine cases. Histopathological analyses were available in seven cases. Seven patients received conservative management. Surgical treatment was performed in seventeen cases, and open repair with an artificial graft was the main surgical intervention. The mortality associated with congenital AAA was high (30.76%). Ruptured aneurysm and renal failure were the main causes of death. Conclusions: Good outcomes can be achieved in children with early identification of congenital AAA and individualized surgical repair with grafts.