MyPACS.net: Radiology Teaching Files > Case 7221475

Never visited FIBROMUSCULAR DYSPLASIA WITH CAROTID ARTERY STENOSIS
Contributed by: patricia burrows, Radiologist, Children's Hospital Boston, Massachusetts, USA.
Patient: 4 year old male
History: 5-year-old boy with known diagnosis of fibromuscular dysplasia, who has vessel disease to his kidneys as well as occasional headaches and abdominal pain.
Images:[small]larger

Fig. 1: Diagnostic Angio dated 06/11/2001

Fig. 2: Interventional Angio dated 06/13/2001

Fig. 3: Interventional Angio dated 06/13/2001
Findings:

CEREBRAL ANGIOGRAM - 06/11/2001:

 

FINDINGS: There are two foci of high-grade, web-like stenosis in the right internal carotid artery. These stenoses are approximately 2 cm apart. The most proximal stenosis is approximately 2 cm from the origin of the bifurcation. The internal maxillary artery from the right external carotid artery injection demonstrates a beaded appearance suggestive of fibromuscular dysplasia. Some irregularity of the superior thyroidal artery on this right side could represent narrowing, however, spasm cannot definitely be excluded. Multiple collaterals from the external carotid artery on the right side to the internal carotid artery circulation is demonstrated including middle meningeal, meningo-hypophyseal and orbital branches. There is mild irregularity of the cavernous and supraclinoid right internal carotid artery. The right middle cerebral artery and both anterior cerebral arteries fill from this right common carotid artery injection. Exuberant collaterals are present from the right external carotid artery circulation filling the right internal carotid artery circulation. From the left external carotid artery injection, there is no significant collateral supply to the internal carotid artery territory. The previously noted collateral supply to the vertebral territory from the occipital artery is not well demonstrated on today's exam due to the more distal position of the catheter on today's study. The left internal carotid artery demonstrates occlusion at the proximal cavernous segment with reconstitution more distally via meningo-hypophyseal branches.

The left vertebral artery is large and tortuous. The portion in the neck is not demonstrated, but is large under fluoro evaluation. Intracranially, there are patent bilateral posterior communicating arteries filling both anterior and both middle cerebral arteries.

 



CEREBRAL ANGIOPLASTY - 06/13/2001:

CLINICAL HISTORY: 4-year-old male with 2 and possibly 3 focal high grade stenoses in the right internal carotid artery which were seen on the cerebral angiogram performed on 6/11/01.

FINDINGS: Three focal high-grade (greater than 95%) stenoses are seen in the cervical portion of the right internal carotid artery. These findings are consistent with the patient's history of fibromuscular dysplasia. The right common carotid artery and external carotid artery are widely patent. The right internal maxillary artery is mildly diffusely diseased. Normal filling of the venous structures is seen on the delayed images.

Following angioplasty of the right internal carotid stenoses, the vessel is widely patent. Mild irregularity in the vessel contour is seen with no focal areas of stenosis.

No intravascular flaps or evidence of dissection are seen post angioplasty.

IMPRESSION: Angioplasty of 3 focal high-grade stenoses in the right internal carotid artery with no residual stenosis post angioplasty.

Diagnosis:

IMPRESSION:

1. Complete occlusion of the distal left internal carotid artery at the proximal cavernous segment. Distally, there is reconstitution via meningo-hypophyseal branches.

2. High-grade foci of stenosis nearly web-like in the right internal carotid artery consistent with fibromuscular dysplasia.

3. Exuberant collaterals from right external carotid artery to the right internal carotid artery including middle meningeal, meningohypophyseal, and orbital branches.

4. Stenosis in the right internal carotid artery, as well as a beaded appearance of the right internal maxillary artery, suggests fibromuscular dysplasia.

5. Large left vertebral artery with patent bilateral posterior communicating arteries filling the left and right anterior and middle cerebral arteries.

 

Discussion:

General Discussion
Fibromuscular dysplasia, commonly called FMD, is a disease that causes one or more arteries in the body to have abnormal cell development in the artery wall. As a result, areas of narrowing, called stenosis, may occur. If enough narrowing causes a decrease in blood flow through the artery, symptoms may result.

FMD is most commonly found in the arteries that supply the kidneys with blood (renal arteries). Up to 75% of all patients with FMD will have disease in the renal arteries. The second most common artery affected is the carotid artery, which is found in the neck and supplies the brain with blood. Less commonly, FMD affects the arteries in the abdomen (supplying the liver, spleen and intestines) and extremities (legs and arms). In 28% of the people with this disease, there will be evidence of FMD in more than one artery.

Symptoms
Many people with this disease do not have symptoms or findings on a physical examination. The signs and/or symptoms that a person with FMD may experience depend on the arteries affected and the degree of narrowing within them. Any pain or clinical sign related to FMD typically comes from the organ that is supplied by that artery. For example, FMD in the kidney arteries may cause high blood pressure.

Progression of disease can also result in ischemic renal atrophy, in which some of the kidney’s tissue dies due to lack of oxygen, and in rare circumstances, kidney failure, if progression of FMD occurs in both the left and right kidney arteries. Patients with impaired carotid arteries may have nonspecific complaints including dizziness, temporary blurring or loss of vision, ringing or buzzing in the ears (tinnitus), feeling as if the room is spinning (vertigo), neck pain, and/or chronic headaches.

However, a person with severe FMD may have neurologic symptoms involving the facial muscles (drooping of the face, for example), stroke or transient ischemic attack. People with carotid FMD have a higher risk for abnormal dilations of the arteries in the brain (intracranial aneurysms). Bleeding in the brain (intracranial hemorrhage) may occur if an aneurysm ruptures.

FMD involving the arteries that supply the intestines, liver and spleen with blood (mesenteric arteries) can result in abdominal pain after eating and unintended weight loss. FMD in the arms and legs can cause limb discomfort with use, cold limbs, weakness, numbness, or skin changes in the fingers and toes due to lack of blood flow.

Causes
The cause of FMD is not yet known, but several theories have been suggested. A number of case reports in the literature have identified the disease in multiple members of the same family including twins. As a result, it is felt that there may be a genetic cause. However, a relative may have different artery involvement, different disease severity, or not develop FMD at all. In fact, not all individuals with FMD have a family member who also has the disease.

FMD is also more commonly seen in women than in men, resulting in the theory that hormones may play an important role in disease development. This theory is further supported by the fact that most women are premenopausal at the time of diagnosis. However, in small population studies one’s reproductive history (number of pregnancies and when they occurred) as well as use of birth control pills did not correlate with the development of FMD.

Other possible causes of FMD include abnormal development of the arteries that supply the vessel wall with blood, resulting in inadequate oxygen supply; the anatomic position of the artery within the body; medications (ergotamine preparations, methysergide); and tobacco use. It is possible that many factors are involved in the development of FMD. This area requires further research.



Courtesy of NORD website:  http://rarediseases.org

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Case Number: 7221475Last Updated: 2011-04-23
Anatomy: Face and Neck   Pathology: Vascular
Modality: AngiographyAccess Level: Readable by all users

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