Related Experiment Videos
[Aortic disruption after operation for pectus excavatum in a infant with Marfanoid hypermobility syndrome]
Insights
A child with Marfanoid hypermobility syndrome died from aortic rupture after pectus excavatum surgery. This case highlights the risk of aortic dissection in connective tissue disorders, even outside dilated areas.
Area of Science:
- Cardiovascular Surgery
- Genetics
- Pathology
Background:
- Pectus excavatum correction is typically a safe cosmetic procedure.
- Connective tissue disorders can predispose individuals to aortic complications.
- Marfanoid hypermobility syndrome shares features with Marfan and Ehlers-Danlos syndromes, affecting connective tissue integrity.
Observation:
- A 5-year-old boy with Marfanoid hypermobility syndrome underwent elective pectus excavatum repair.
- Postoperatively, the patient developed a 5 cm longitudinal rupture of the descending aorta.
- Histological examination revealed cystic medial necrosis in the aorta.
Findings:
- The aortic rupture occurred 3.2 cm distal to the left subclavian artery, not within the preoperatively noted 4.6 cm dilatation.
- The patient exhibited tall stature, arachnodactyly, joint hyperextensibility, and hyperelastic skin, consistent with Marfanoid hypermobility syndrome.
- Cystic medial necrosis was identified as the underlying pathology.
Implications:
- Surgical correction of pectus excavatum may act as a trigger for aortic dissection in susceptible individuals.
- Aortic dissection can occur in patients with connective tissue diseases even in non-dilated aortic segments.
- This case underscores the importance of thorough preoperative evaluation for connective tissue disorders in patients undergoing thoracic surgery.
Abstract:
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.