Cas clinique

DOI of or1Cardiovas

Yokohama, Ja2Departme

Center, Yokoh

CorrespondYokohama CitKu, Yokohama

Ann Vasc Surhttp://dx.doi.or� Annals of V�Edit�e par ELS

Dysplasie fibromusculaire associ�ee �a ladissection spontan�ee simultan�ee de quatreart�eres p�eriph�eriques chez un homme de30 ans

Tadahisa Sugiura,1 Kiyotaka Imoto,1 Keiji Uchida,1 Hiromasa Yanagi,1 Daisuke Machida,1

Makoto Okiyama,1 Shota Yasuda,1 Shigeo Takebayashi,2 Yokohama, Japon

Un homme de 30 ans a eu un acc�es soudain de douleurs abdominales graves. Un scanner mon-trait des dissections des art�eres coeliaque, m�esent�erique sup�erieure, r�enale gauche, et iliaqueexterne droite ; une st�enose de l’art�ere r�enale droite ; et un infarctus du rein gauche. Apr�es�evaluation soigneuse, le diagnostic de dysplasie fibromusculaire (dysplasie m�ediale) �etait port�e,sur les r�esultats du scanner. Ce cas est extremement rare parce que la dysplasie fibromuscu-laire s’est produite en meme temps que des dissections spontan�ees simultan�ees de quatreart�eres p�eriph�eriques chez un jeune homme.

Fibromuscular dysplasia (FMD) is a noninflamma-

tory, nonatherosclerotic disorder that leads to arte-

rial stenosis. It most commonly affects the renal

and internal carotid arteries and is more common

among women than men. We report the case of a

30-year-old man who had FMD associated with

simultaneous spontaneous dissections of the celiac

artery, superior mesenteric artery, left renal artery,

and right external iliac artery.

CASE REPORT

A30-year-oldmanhad a sudden bout of severe abdominal

pain and was taken to a hospital. An enhanced computed

tomographic (CT) scan revealed dissections of the celiac

iginal article: 10.1016/j.avsg.2011.02.018.

cular Center, Yokohama City University Medical Center,pon.

nt of Radiology, Yokohama City University Medicalama, Japon.

ance : Tadahisa Sugiura, Cardiovascular Center,y University Medical Center, 4-57 Urafune-Cho, Minami-232-0024, Japon, E-mail: ssugiura@hij.twmu.ac.jp

g 2011; 25: 838.e9-838.e11g/10.1016/j.acvfr.2012.07.021ascular Surgery Inc.EVIER MASSON SAS

artery (Fig. 1A), superior mesenteric artery, left renal

artery (Fig. 1B), and right external iliac artery (Fig 1C);

stenosis of the right renal artery; and left kidney infarction.

The patient was transferred to our hospital because his

condition did not improve 2 days after admission.

He was given a beta-blocker and a calcium antagonist

for hypertension. He was administered warfarin (target

international normalized ration: 2.0-2.5), and aspirin

(100 mg) for arterial stenosis owing to the dissections.

Laboratory tests gave the following findings: white cell

count, 15,660/mL; platelets, 7.4 � 104/mL, aspartate ami-

notransferase, 65 U/L; alanine aminotransferase, 63 U/L;

lactate dehydrogenase, 1,118 U/L; blood urea nitrogen,

18 mg/dL; serum creatinine, 2.11 mg/dL; and C-reactive

protein, 11.168 mg/dL. Vasculitis was initially suspected,

but was not supported by the results of laboratory tests.

Finally, FMD (medial dysplasia type) was diagnosed on

the basis of enhanced CT findings.

Headache and dizziness developed on the same day as

the abdominal pain. A brain CT scan showed a low-

density area in the left cerebellum. Although the finding

was not compatible with cerebellar infarction, the results

of follow-up CT indicated that the low-density area was a

small infarction. However, CT angiography showed no

dissection of the head or neck vessels.

Renal scintigraphy revealed glomerular filtration rate

(GFR) of 25.6 ml/min/1.73m2 (left kidney) and 6.7 ml/

min/1.73m2 (right kidney). We thought that the right

renal artery stenosis was the cause of decline of the right

894.e9

Fig. 1. (A) An enhanced computed tomographic (CT)

scan revealing dissection of the celiac artery (arrow). (B)

An enhanced CT scan revealing dissection of superior

mesenteric artery and left renal artery (arrow). (C) An

enhanced CT scan revealing dissection of right external

iliac artery (arrow).

Fig. 2. Angiography of the right renal artery. The middle portion of the right renal artery was stenotic (A), and after

successful dilation with stent (B).

894.e10 Cas cliniques Annales de chirurgie vasculaire

renal GFR. Therefore, we performed percutaneous trans-

luminal right renal angioplasty (Fig. 2A, B). After this

procedure, renal function improved gradually; renal

scintigraphy showed GFR of 25.5 ml/min/1.73m2 (left)

and 21.6 ml/min/1.73m2 (right). Amylase and lipase

levels increased 4 days later, ischemic pancreatitis was

then diagnosed. The patient was discharged from the

hospital after 67 days. He is doing well 1 year after dis-

charge. He continued to take warfarin for 3 months and

will continue to take aspirin all his life.

DISCUSSION

The etiology of FMD remains unclear and is thought

to involve a variety of genetic, mechanical, and

hormonal factors. Among adults, FMD is more

common inwomen, with a 2-10 times higher preva-

lence than that in men.1 FMDmost often affects the

renal arteries, accounting for 60-75% of cases.2

Extracranial cerebrovascular arteries are involved in

25-30% of cases, and other miscellaneous arteries,

such as the mesenteric or brachial arteries and

coronary arteries, are involved in up to 30% of

cases. Approximately 25% of patients have invol-

vement of multiple arteries.2

The most commonly accepted classification of

FMD includes three major types: medial dysplasia,

intimal fibroplasia, and adventitial fibroplasia.3

Medial dysplasia is further divided into three

subgroups: medial fibroplasia, medial hyperplasia,

Vol. 25, No. 6, 2011 Cas cliniques 894.e11

and perimedial fibroplasia. Medial fibroplasia is

most common, accounting for 60-70%of all types of

FMD, and is characterized by a ‘‘string of beads’’

appearance on angiography.

Approximately one-half of all dissections

involving visceral arteries are asymptomatic. Occa-

sionally, patients present with intestinal angina or

hemorrhage.4,5 Various symptoms have been des-

cribed depending on the location of the lesions,

including jaundice in association with dissection of

the celiac and hepatic artery, or malabsorption with

involvement of the superior mesenteric artery.6,7

Glehen et al. reported a treatment strategy for the

acute phase of isolated symptomatic celiac artery

dissections.8 Surgery is indicated for management

of aneurysm, occlusive lesions jeopardizing the

lower intestine, arterial rupture, or liver ischemia.

They further suggest that conservative medical

treatment can be proposed for patients with limited

dissection in whom serial examinations have

demonstrated no evidence of rupture or expansion.

Therefore, the patient had conservative treatment in

this case.

Our patient had similar abdominal pain 1 year

before the present episode, but enhanced CT at

that time revealed no dissection, and he was asymp-

tomatic after that. The dissections of the four peri-

pheral arteries (celiac artery, superior mesenteric

artery, left renal artery, and right external iliac

artery)were therefore considered to have developed

spontaneously. To our knowledge, this is the first

documented case of FMD with these characteristics.

This case is extremely rare because FMD occurred

concurrently with spontaneous dissections of four

peripheral arteries in a young man.

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