Coil embolization of bilateral internal mammary artery aneurysms is durable in a patient with Marfan syndrome

Abstract

Internal mammary artery (IMA) aneurysms are very rare, have a high risk of rupture, and can cause hemothorax. Here, we report the case of a 33-year-old man with metachronal and bilateral IMA aneurysms. He had Marfan syndrome diagnosed by genetic testing. We carried out endovascular repair with coil embolization. He has survived without additional treatment for 7 years. Endovascular repair of metachronal and bilateral IMA aneurysms is feasible even in a patient with Marfan syndrome.

Tratamento endovascular de aneurisma de artéria torácica interna esquerda

“…11,12 The etiology of true aneurysms of the internal thoracic artery is generally related to vasculitis (Kawasaki disease, polyarteritis nodosa, and systemic lupus erythematosus), connective tissue diseases (Marfan Syndrome and Ehlers-Danlos Syndrome), type 1 neurofibromatosis, fibromuscular dysplasia, atherosclerosis, or idiopathic causes. 3,7 Although endovascular treatment does not offer the possibility of definitive diagnosis by histopathology, it is presumed that the diagnosis in this case was a true atherosclerotic aneurysm, based on the patient's clinical history of hypertension and diabetes, signs of atherosclerotic disease with calcifications of coronary arteries, negative history of prior medical interventions or traumas, and no diagnosis of connective tissue diseases or vasculitis, and also on the findings of examinations. These elements lead us to assume that the aneurysm was a true aneurysm of atherosclerotic degenerative origins.…”

“…14 True ITAAs are so rare that there is little information on management and prognosis. To date, there are no established criteria for intervention 3 and the decision to treat ITAAs is based on their size, on the presence of symptoms, and on the risk of rupture. 8 Internal thoracic artery aneurysm rupture can be fatal, since hemothorax with shock is the most common initial manifestation.…”

“…8 Internal thoracic artery aneurysm rupture can be fatal, since hemothorax with shock is the most common initial manifestation. 3 Treatment options described in the literature are open surgical repair or endovascular treatment with stenting and/or embolization. In the case of rupture, treatment depends on the patient's hemodynamic condition.…”

“…11,13 Embolization with coils is the treatment of choice for arteriovenous fistulas and smaller aneurysms because the technique is relatively easy in tortuous vessels and cases with short aneurysm necks. 3 There are also other agents used for embolization, such as polymers and sometimes even glues, that can be used in combination treatment with the objective of occluding the proximal and distal portions of the aneurysm to avoid it being fed by collaterals. 11 Exclusion of the aneurysm using covered stents is an option for certain vascular beds and some studies have shown that this is a feasible alternative treatment for ITAA.…”

“…Just 40 cases have been described over the last 40 years, two thirds of which were pseudoaneurysms. 3 True aneurysms are rarer and the first case was reported in 1978 by Den Otter and Stam in a 30-year-old woman with a "coin lesion" found incidentally during a routine X-ray examination. 4 True aneurysms have been described in association with vasculitis, connective tissue disorders, genetic syndromes, and atherosclerosis.…”