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.