Complex Multi-Stage Total Aortic and Subclavian Artery Replacement in a 9-year old boy with Loeys-Dietz-Syndrome.

BACKGROUND

Loeys-Dietz Syndrome is a rare connective tissue disorder that is associated with arterial pathologies such as aortic dissections, tortuosity and aneurysms.We present a child with Loeys-Dietz Syndrome type 2 that received total aortic and bilateral subclavian artery replacement.

CASE REPORT

A 9-year old boy with Loeys-Dietz Syndrome type 2 and acute type B aortic dissection received an urgent complete thoracic and thoraco-abdominal aortic repair within three days. First, the ascending aorta and aortic root were replaced in a Tirone David and Frozen Elephant Trunk procedure. Then, the descending and supramesenteric aorta was replaced by a Dacron interposition graft with direct implantation of the celiac trunk. During the 15 months follow-up, the patient required three more surgical interventions for rapid expanding aneurysms of both subclavian arteries and the infrarenal aorta. No major adverse event nor secondary interventions occurred. Ultrasonographic and magnetic resonance imaging follow-up is continued at 6-months intervals.

CONCLUSION

Children with Loeys-Dietz Syndrome may require extensive aortic repair for aortic dissection and show rapidly expanding aneurysms. Referral to a center with pediatric vascular expertise and long-term follow-up examinations are crucial.