Abstract
Objective
Fibromuscular dysplasia rarely involves vessels other than the renal and carotid arteries. We present a case of a rare fibromuscular dysplasia involving multiple vascular beds in a young female patient with history of spontaneous coronary artery (SCAD).
Methods
This is a case report with review of the literature using PubMed search for other cases of fibromuscular dysplasia that involves multiple vascular beds and its association with SCAD. The patient agreed to publish her case including her images.
Results
Fibromuscular dysplasia involving multiple vascular beds in a young female patient with prior coronary dissection is rarely reported in the literature.
Conclusion
Fibromuscular dysplasia affecting multiple vascular beds is rare but should be suspected in patients with SCAD, particularly young female patients.
Introduction
Fibromuscular dysplasia (FMD) is a noninflammatory, nonatherosclerotic disease resulting in stenosis, occlusion, aneurysm, or dissection in the affected artery. 1 It is more common in women, classically presenting as elevated blood pressure in an otherwise healthy young female due to renal artery stenosis. However, presentation can vary widely based on the location of the involved artery. Fibromuscular dysplasia is typically diagnosed by radiographic evidence, and therefore classification is primarily based on angiographic appearance. The two main angiographic subtypes of FMD include multifocal FMD and focal FMD. The multifocal subtype has the classic angiographic appearance of “beads on a string,” whereas the focal subtype appears to show more “circumferential or tubular stenosis.” 2
The most commonly involved arteries in FMD include the renal and extracranial carotid arteries, and less commonly, the external iliac and vertebral arteries. 3 Fibromuscular dysplasia rarely involves a mesenteric artery, and cases involving multiple mesenteric arteries are exceedingly rare. One review of over 1100 patients with fibromuscular dysplasia showed celiac involvement in 10 patients and mesenteric artery involvement in 14 patients, 0.8 and 1.2% of patients, respectively. 4
Case report
A 48-year-old white female was referred to vascular surgery by her cardiologist for evaluation of fibromuscular dysplasia and a small superior mesenteric SMA aneurysm. Seven months prior to her clinic visit, she was diagnosed and treated for spontaneous coronary artery (SCAD). In brief, the patient had presented with severe chest pain, elevated troponin, and ST depressions on EKG. Left heart catheterization showed spontaneous flow-limiting dissection of the proximal right coronary artery that was treated with two overlapping 4 mm drug-eluting stents. Other medical history was noncontributory. Of note, the patient has never smoked or used other tobacco products. She has no other significant risk factors for cardiovascular disease. During the patients’ follow-up visits with her cardiologist, she reported intermittent postprandial epigastric pain. When the patient visited out vascular clinic, she endorsed postprandial pain for over a year with associated intermittent nausea and diarrhea, but had not been able to attribute it to anything. Her primary care physician did a trial of holding her Lexapro for concern that these symptoms may be due to side effects; however, she reported no change after holding the medication. Eventually, the patient was sent for a computed tomography (CT) angiogram of the abdomen and pelvis for workup of possible fibromuscular dysplasia. This scan was highly suggestive of multivessel fibromuscular dysplasia (Figure 1). Of note, the patient had no known family history of fibromuscular dysplasia. The celiac artery and SMA were both patent but showed the classic “beads on a strong” appearance indicating fibromuscular dysplasia. The SMA also showed a small branch aneurysm measuring 4 mm (Figure 1). Her common, internal, and external iliac arteries were all normal.
Left: A CTA showing “beads on a string,” most notably in the left renal artery (blue arrow). Right: A CT VR showing the same classic appearance of FMD in the left renal artery. There is also evidence of FMD in the celiac trunk and small aneurysm in distal SMA (red arrow).
In addition, the patient suffered occasional headaches. Therefore, she underwent CT angiogram of the head and neck, as FMD patients can often have cerebrovascular involvement to complete her work up. This scan revealed alternating segments of stenosis and a normal caliber right internal carotid artery starting at approximately the level of C2–C3 and extending into the craniocervical junction. The left side showed similar findings, with mild undulation of the internal carotid artery with segments of mild narrowing, starting at approximately the level of C4 and extending up to the level of C2 (Figure 2). The vertebral arteries were normal bilaterally. She was treated with aspirin, ticagrelor, metoprolol succinate, and atorvastatin. We could not obtain a signed consent from the patient due to the coronavirus crisis and email access; however, we have a verbal consent obtained over the phone in the presence of a witness.
CT VR shows patterns of alternating mild narrowing with normal caliber vessel at the distal cervical internal carotid arteries consistent with fibromuscular dysplasia.
Discussion
Fibromuscular dysplasia involving the mesenteric arteries is a rare occurrence. Even more unique is involvement of multiple vascular beds. Kimura et al. 5 reported a case of renal and superior mesenteric artery FMD. Involvement of the celiac and superior mesenteric arteries as well as the renal and internal carotid arteries in the same patient, to our knowledge, has rarely been reported in the literature. The patient’s history of SCAD also makes this case unique. The clear images of her disease are often difficult to obtain and do not often show the classic findings. The nature of this patient’s vascular disease is clearly widespread. As mentioned, prior to her evaluation with vascular surgery, she already had suffered from SCAD. One study showed that 66% of patients with SCAD will have extracoronary vascular abnormalities (EVA) (a number that has often been thought to be even a low estimate 6 ). Some of these EVAs are mainly dissection or aneurysm formation and could affect multiple vascular beds like carotids, visceral, and intracranial. The authors note that EVAs are prevalent in non-FMD SCAD patients. They also point out that silent intracranial abnormalities are not uncommon. 6 A trial of ACE inhibitor was considered, but ultimately decided against it to avoid hypotension given normotension with metoprolol that she has been on given her history of SCAD. Her headaches correlated to times of low blood pressure. It was decided to initiate conservative management of the patient’s disease. Her abdominal symptoms were vague and too infrequent to justify intervention on the 4 mm SMA aneurysm. The patient will be followed with serial ultrasounds to assess the SMA diameter. The carotid FMD is asymptomatic, and her blood pressure is well controlled on one agent. She was referred to the genetics clinic, and it was discussed with the patient to consider COL3A1 testing to evaluate for Ehlers Danlos Syndrome type 4. It was ultimately decided to wait until her next follow-up appointment next year before deciding to go ahead with the testing, as she does not have any other features of Ehlers Danlos. In terms of follow-up with vascular surgery, her most recent imaging showed no change in her disease. Her FMD lesions are not prominent or severe. She will continue to be observed with yearly carotid, renal, and mesenteric ultrasounds.
The complications of FMD are well known. Aneurysm formation is said to occur in about 17% of patients, and dissection is reported in 19.7% of patients. 2 Our patient had already developed an aneurysm in one of the affected vessels. It can also be reasonably assumed that her underlying FMD could have been the predisposing factor for her SCAD. The patient was educated about her disease and instructed to present immediately if she develops any symptoms related to the multiple vascular beds involved with FMD. In addition, this case report serves to alert physician to have a high suspicion of FMD in young female patients diagnosed with SCAD.
Footnotes
Declaration of conflicting interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.
