- Open Access
- Open Peer Review
Color Doppler imaging of cervicocephalic fibromuscular dysplasia
Cardiovascular Ultrasound volume 2, Article number: 7 (2004)
Fibromuscular dysplasia (FMD) is a possible cause of stroke, especially in middle-aged women. However, only few reports are available on ultrasonographic detection and monitoring.
Among the 15,000 patients who underwent color Doppler imaging (CDI) of the cervicocephalic arteries during the study period, all cases fulfilling ultrasound criteria of FMD were included into the case series. Criteria of FMD were: 1. Segmental string-of-beads pattern, 2. Localization in the distal extracranial part of internal carotid artery (ICA) or vertebral artery (VA), and 3. (optional): Direct and/or indirect criteria of stenosis.
CDI detected FMD in 39 vessels (37 ICA and 2 VA segments) of 21 patients. 16 patients had bilateral manifestation on ICA, one of those also on VA, bilaterally. CDI disclosed 4 symptomatic high-grade ICA stenoses, 3 of them underwent endovascular treatment. 5 patients with moderate symptomatic ICA stenoses got medical treatment. In 6 patients FMD was the most likely cause of headache and in one patient FMD was diagnosed as a cause of vertigo.
CDI may be used for detection of cervicocephalic FMD. Due to the unfavourable localisation of FMD for CDI, the sensitivity of CDI is lower in comparison to angiography. However, high-grade FMD stenoses that require invasive treatment can be recognized on the basis of indirect hemodynamic criteria.
Fibromuscular dysplasia (FMD) is a non-atheromatous, non-inflammatory arteriopathy of unknown etiology with segmental manifestation on medium-sized arteries in various regions of the body . Manifestation on the renal arteries with the possible consequence of renovascular hypertension is remarkably frequent . The cervico-cephalic arteries, especially the internal carotid artery (ICA) are attacked with an incidence of about 0.6 – 1%, often bilaterally ; manifestation also occurs on the vertebral artery (VA) . The disease can occur at any age but is usually diagnosed in middle-aged, predominately female individuals .
Angiography reveals in most cases the typical string-of-beads pattern (fig. 1) with alternating regions of lumen narrowing and vessel dilatation over a length of 3 – 5 cm ; the proximal section of the ICA is generally not affected, except in a rare FMD subtype characterised by proximal involvement with a web-like membrane .
Clinical manifestations of FMD on the ICA are transitoric ischemic attacks or cerebral infarctions  as well as unspecific symptoms such as headache and vertigo. In cases of cerebrovascular events, endovascular or surgical treatment is recommended [7–9], therefore detection of FMD is of considerable importance.
Patients and methods
Among the 15,000 patients who attended the neurosonography department of our clinic during the study period, 21 cases were identified fulfilling ultrasound criteria of FMD (Table 1). The presenting symptoms of the patients are listed in table 2.
The color Doppler examinations were performed as described by Arning  and included the common carotid, external carotid, and internal carotid arteries as well as the vertebral arteries.
CDI was performed with 5 MHz and 7 MHz linear array transducers using one of the following systems: Acuson Sequoia (Siemens AG, Erlangen, Germany), Toshiba Powervision 6000 or Toshiba Aplio (Toshiba Medical Systems Europe, Zoetermeer, Netherlands), or ATL HDI 5000 (Philips Medical Systems, Andover, MA).
Using the criteria of table 1, FMD was diagnosed in 21 patients (1 male, 20 female). In total, CDI detected FMD in 39 vessels (37 ICA and 2 VA segments). 16 patients had bilateral manifestation on ICA, one of those also on VA, bilaterally. 5 patients had unilateral manifestation on ICA.
The degree of stenosis was low in 2 patients (Fig. 2) and moderate in the majority of cases (Fig. 3,4,5). 5 patients with moderate symptomatic ICA stenoses got medical treatment. 4 symptomatic high-grade ICA stenoses (Fig. 6,7,8) were detected, 3 of them underwent endovascular treatment (Fig. 9). In 6 patients FMD was the most likely cause of headache and in one patient FMD was diagnosed as the cause of vertigo, involving vertebral artery (fig. 10).
FMD is an uncommon angiopathy with an incidence on the ICA of about 0.6 – 1% . However, the frequency of FMD detection by ultrasound imaging is considerably lower: 0,14% in our case series. Only few reports are available on the detection and monitoring of cervicocephal FMD with ultrasonography [11–14].
Ultrasound criteria of FMD correspond to those of angiography (Fig. 1). CDI reveals the segmental string-of-beads pattern with alternating regions of lumen narrowing and vessel dilatation (Fig. 2,3), distally to a completely normal segment of the vessel (Fig. 6). Dependent on the degree of stenosis, direct (Fig. 8) or indirect hemodynamic criteria may be recognized .
In comparison to angiography, the sensitivity of CDI is low: The vascular lesion can only be visualized sonographically when it is located not too far cranially on the ICA . However, high-grade FMD stenoses will be detected on the basis of indirect hemodynamic criteria. To overlook asymptomatic cases of low grade or medium grade stenosing lesions will not have a negative consequence since they do not require any treatment .
CDI allows diagnosis of FMD in numerous cases. Due to the unfavourable localisation of FMD for CDI, the sensitivity of CDI is low in comparison to angiography. However, high-grade FMD stenoses that require invasive treatment can be recognized on the basis of indirect hemodynamic criteria.
Color Doppler Imaging
Internal Carotid Artery
Russo CP, Smoker WRK: Nonatheromatous carotid artery disease. Neuroimaging Clinics of North America 1996, 6: 811-830.
Slovut DP, Olin JW: Fibromuscular dysplasia. N Engl J Med 2004, 350: 1862-1871. 10.1056/NEJMra032393
Sandok BA: Fibromuscular dysplasia of the internal carotid artery. Neurol Clin 1983, 1: 17-26.
Mas JL, Bousser MG, Hasboun D, Laplane D: Extracranial vertebral artery dissections: a review of 13 cases. Stroke 1987, 18: 1037-1047.
Morgenlander JC, Goldstein LV: Recurrent transient ischemic attacks and stroke in association with an internal carotid artery web. Stroke 1991, 22: 94-98.
Sandmann J, Hojer D, Bewermeyer H, Bamborschke S, Neufang KF: Fibromuscular dysplasia as a cause of cerebral infarct. Nervenarzt 1992, 63: 335-340.
Curry TK, Messina LM: Fibromuscular dysplasia: when is intervention warranted? Semin Vasc Surg 2003, 16: 190-199. 10.1016/S0895-7967(03)00024-3
Chiche L, Bahnini A, Koskas F, Kieffer E: Occlusive fibromuscular disease of arteries supplying the brain: results of surgical treatment. Ann Vasc Surg 1997, 11: 496-504. 10.1007/s100169900081
Van Damme H, Sakalihasan N, Limet R: Fibromuscular dysplasia of the internal carotid artery. Personal experience with 13 cases and literature review. Acta Chir Belg 1999, 99: 163-168.
Arning C: Farbkodierte Duplexsonographie der hirnversorgenden Arterien 3 Edition Stuttgart, New York: Thieme 2002.
Edell SL, Huang P: Sonographic demonstration of fibromuscular hyperplasia of the cervical internal carotid artery. Stroke 1981, 12: 518-520.
Kliewer MA, Carroll BA: Ultrasound case of the day. Internal carotid artery web (atypical fibromuscular dysplasia). Radiographics 1991, 11: 504-505.
Krzanowski M: Fibromuscular dysplasia of the internal carotid artery as a cause of transient cerebral ischemia episodes. Pol Arch Med Wewn 1997, 98: 546-550.
Arning C: Nonatherosclerotic disease of the cervical arteries: Role of ultrasonography for diagnosis. VASA 2001, 30: 160-167.
Wells RP, Smith RR: Fibromuscular dysplasia of the internal carotid artery: a long term follow-up. Neurosurgery 1982, 10: 39-43.
Wesen CA, Elliott BM: Fibromuscular dysplasia of the carotid arteries. Am J Surg 1986, 151: 448-451. 10.1016/0002-9610(86)90100-5
Authors’ original submitted files for images
Below are the links to the authors’ original submitted files for images.
About this article
Cite this article
Arning, C., Grzyska, U. Color Doppler imaging of cervicocephalic fibromuscular dysplasia. Cardiovasc Ultrasound 2, 7 (2004) doi:10.1186/1476-7120-2-7
- Internal Carotid Artery
- Vertebral Artery
- Renovascular Hypertension
- Color Doppler Imaging
- Vessel Dilatation