Multistaged hybrid aortic repair successfully excluded aneurysms and restored right arm circulation with symptom-free status at 6 months in a 14-year-old female with Loeys-Dietz Syndrome Type 2.
Case Report (n=1)
No
This case highlights the aggressive vascular manifestations of Loeys-Dietz Syndrome Type 2 and the necessity of a multistaged, hybrid surgical and endovascular approach for complex aortic and arterial aneurysms.
Abstract Loeys-Dietz Syndrome (LDS) is an autosomal dominant connective tissue disorder characterized by aggressive vascular manifestations, often necessitating early surgical intervention. We report on a 14-year-old female with LDS Type 2, with a previous open surgical repair (OSR) of aortic root and arch, who presented with a symptomatic diverticulum of the ductus arteriosus and critical right upper extremity ischemia, due to newly diagnosed occlusion of a subclavian artery (SA) aneurysm. Urgent surgical management included the exclusion of the right SA aneurysm using a 8mm reinforced Dacron graft. In a second procedure two days later, left common carotid artery (LCCA) - SA bypass and thoracic endovascular aortic repair (TEVAR) landing in zone 2 followed. Postoperative scans revealed distal progression of the disease, resulting in distal extension with TEVAR and eventually in an open thoracoabdominal aortic repair.
Koumarelas et al. (Sun,) conducted a case report in 14-year-old female patient with Loeys-Dietz Syndrome Type 2 with previous aortic root and arch repair presenting with symptomatic ductus arteriosus diverticulum and subclavian artery aneurysm causing critical right upper extremity ischemia (n=1). Multistaged hybrid aortic repair including open surgical exclusion of right subclavian artery aneurysm, left common carotid artery to subclavian artery bypass, thoracic endovascular aortic repair (TEVAR), and subsequent open thoracoabdominal aortic repair was evaluated on Successful exclusion of aortic aneurysms and restoration of circulation without symptoms at 6 months follow-up. Multistaged hybrid aortic repair successfully excluded aneurysms and restored right arm circulation with symptom-free status at 6 months in a 14-year-old female with Loeys-Dietz Syndrome Type 2.