A Staged, Hybrid Approach to Management of Long-term Complications After Childhood Treatment of Mid-Aortic Syndrome: A Case Report
Selena G. Goss, MD, Mariano Arosemena, MD, Babak Abai, MD, Dawn M. Salvatore, MD, Paul J. DiMuzio, MD.
Thomas Jefferson University Hospital, Philadelphia, PA, USA.
OBJECTIVES: Mid-aortic syndrome in the pediatric population is a rare condition that most commonly presents with malignant hypertension and renal failure. Surgical treatment often involves aortic reconstruction and renal artery bypass. We present a case of a patient treated at age 8 with aorto-aortic bypass and bilateral renal artery bypass who presented at age 38 with claudication, lower extremity embolization necessitating anticoagulation, and a 5.5cm pseudoaneurysm of the left renal artery bypass.
METHODS: Angiography revealed mural thrombus within the aorto-aortic bypass, pseudoaneurysmal degeneration of aortic graft-left renal bypass (see figure, left panel), intact aortic-right renal artery bypass, and normal bilateral run-off. ABIs were 0.9 bilaterally. Nuclear medicine scan of the right and left kidneys revealed split function of 57% and 43%, respectively. The patient underwent thoraco-L CIA-R CFA bypass. He subsequently underwent staged stent-grafting of the aorto-left renal artery pseudoaneurysm and Amplatzer plug occlusion of the previous aorto-aortic graft. RESULTS: Completion arteriography (see figure, right panel) demonstrated exclusion of the pseudoaneurysm, patency of the left renal artery bypass, and thrombosis of the prior aortic bypass. Following surgery, his anticoagulation was stopped. Three months post-operatively, the patient’s claudication had resolved and his blood pressure was normal. On follow-up CTA, the aorto-left renal bypass pseudoaneurysm was excluded.
CONCLUSIONS: The surgical treatment of mid-aortic syndrome in childhood is complicated by the finite durability of the interventions performed, as well as by the impact of patient growth. The most common long-term complications involve stenosis and aneurysmal degeneration of the bypass graft. There is a paucity of literature on the long-term outcomes of surgical treatment for mid-aortic syndrome. Few series exist with decades-long follow up. Contemporary management of long-term complications includes innovative approaches that encompass both open and endovascular strategies, tailored to patient-specific issues. In this report we have outlined what appears to be the second documented case of a hybrid approach to addressing multiple long-term complications that arose from childhood surgical treatment of mid-aortic syndrome. 
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