Title: Giant internal carotid artery aneurysms and porcelain aorta in an elderly patient with Marfan syndrome
Hiroki Yagi, MD, PhD 1),2),#, Norifumi Takeda, MD, PhD 1),2), Yumiko Hosoya, MD, PhD 3)
Haruo Yamauchi, MD, PhD 2),4), Issei Komuro, MD, PhD1) 
1)Department of Cardiovascular Medicine,2)Marfan Syndrome Center,3)Department of Therapeutic Strategy for Heart Failure, 4)Department of Cardiac Surgery, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan
#Address for corresponding author : Hiroki Yagi, MD, PhD Department of Cardiovascular Medicine
Graduate School of Medicine
The University of Tokyo 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan Phone: +81-3-3815-5411 Email: hiroki_yagi_19830414@yahoo.co.jp
199 words, 1 reference and 2 figures are included in this manuscript.
Key words : Marfan syndrome, internal carotid artery aneurysm, porcelain aorta, elderly patient
Patient is a 79-year-old obese woman with a BMI of 31.6, complicated with lifestyle-related diseases. At age 61, CT scan identified chronic thoracoabdominal aortic dissection (TAAD) involving multiple visceral and cervical arteries and with severe calcification (porcelain aorta) (Figure1A). At age 76, asymptomatic and marked enlarged bilateral internal carotid artery aneurysms (ICAAs) with tortuosity were incidentally depicted (Figure1B). The maximum right and left diameters were 2.2 cm and 5.6 cm, respectively. Surgical treatment options were considered, however conservative management was chosen because of her greater risk of operation. In April 2017, she was referred to our hospital and was diagnosed with Marfan syndrome (MFS) based on the presence of ectopia lentis and TAAD and a previously-reportedFBN1 pathogenic variant (c.1709G>C; p.Cys570Ser). MFS is an autosomal dominant connective tissue disorder with genetic predisposition to aortic aneurysms and dissections at a relatively young age1). Recent advances in the medical and surgical management have improved life expectancy. ICAAs and porcelain aorta had rarely been reported in MFS, however as the number of elderly patients increases, these unfamiliar late arterial complications might be kept in mind to develop more comprehensive management, because MFS arteries are considered to be easily influenced by lifestyle-related diseases.