[Aortic disruption after operation for pectus excavatum in a infant with Marfanoid hypermobility syndrome].

An elective correction of pectus excavatum was carried out in a 5-year-old boy who subsequently died on the sixth postoperative day from rupture of descending aorta. The rupture which was longitudinal disruption 5 cm in length occurred 3.2 cm distal to the orifice of the left subclavian artery. Histological examination revealed that cystic medial necrosis was present in the thoracic and abdominal aorta. This boy had a slightly tall frame, arachnodactylia, hyperextensibility of the joint, but no ocular symptoms. His skin was excessively elastic and friable. Although he had been identified with characteristics to neither Marfan syndrome nor Ehlers-Danlos syndrome, he was thought to be with Marfanoid hypermobility syndrome. Since aortic dilatation was 4.6 cm in diameter and no valvular involvement was detected preoperatively, only the cosmetic surgery for the pectus excavatum was made. The correction of pectus excavatum might be a trigger of aortic disruption. We must mention that aortic disruption may be occur besides the dilated portion in case of connective tissue disease.