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| CASE REPORT |
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| Year : 2016 | Volume
: 3
| Issue : 2 | Page : 73-74 |
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Fibromuscular Dysplasia: A Case Study from China
Shibchurn Mithilesh
Vascular and Endovascular Surgery, Neurology, Sir Run Run Shaw Hospital, College of Medicine, Zhejiang University Loma Linda University, Zhejiang, China
| Date of Web Publication | 8-Jun-2016 |
Correspondence Address:
Shibchurn Mithilesh
Vascular and Endovascular Surgery, Neurology, Sir Run Run Shaw Hospital, College of Medicine, Zhejiang University Loma Linda University, Zhejiang
China
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0972-0820.183642
A 59-year-old Chinese patient was diagnosed with fibromuscular dysplasia (FMD) which is a rare genetic disorder among Asians. Neck vessels angiography showed a classic ''string of bead appearance'' affecting vertebral and internal carotid arteries. Carotid artery surgical reconstruction and vertebral intervention were proposed by vascular surgeon to prevent future strokes, but patient's family refused for intervention/surgery. However, on optimal medical management his health improved during follow-up of over 8 months. This case shows unique multivessel FMD involving bilateral vertebral and one internal carotid artery which was managed successfully with medical management.
Keywords: Active intracranial hemorrhage with a midline shift, fibromuscular dysplasia, stroke in evolution
How to cite this article:
Mithilesh S. Fibromuscular Dysplasia: A Case Study from China. Indian J Vasc Endovasc Surg 2016;3:73-4 |
| Introduction | |  |
Fibromuscular dysplasia (FMD) is an uncommon non-atheromatous, non-inflammatory arteriopathy of unknown origin affecting mostly the renal arteries (70-75%) in middle aged population having a “string of beads” appearance. Besides the renal arteries, the mid-cervical segment ofthe internal carotid artery (ICA) is the next most frequently affected region (25-30%) and 8-12% of patients with carotid involvement have associated vertebral artery involvement. This case presents a rare multivessel involvement of vertebral and carotid arteries.
| Case Report | | |
A 59-year-old male with one year history of poorly controlled hypertension (158/106 mmHg on presentation in the emergency department) presented with left side hemiparesis with headache, vertigo, and a Glascow Coma Score of 15 points. Brain computed tomography (CT) revealed infarction of the right occipital lobe. Aspirin was started promptly. Three days later radiology disclosed infarction on the right thalamus (stroke-in-evolution) [Figure 1]a. Low molecular weight heparin was given for 3 days. Two weeks later, he had a suddenly unbearable headache, and his vision became blurred. His hemiparesis deteriorated. Brain CT showed postinfarction hemorrhage involving the thalamus and breaking into the lateral ventricle with a midline shift of 8.7 mm to the left side compressing the right optic nerve [Figure 1]b. Cranial nerve examination showed anisocoria (diameter of pupils; right: 2.5 mm, left: 4 mm). Digital subtraction angiography revealed a classic ''beaded appearance'' with multiple constrictions of the lumen affecting vertebral arteries and left internal carotid artery (ICA) [Figure 1]c and [Figure 2]. He was diagnosed with fibromuscular dysplasia (FMD). He was discharged on day 21 without any neurologic sequelae, and aspirin (81 mg daily) was prescribed for primary stroke prevention. | Figure 1: (a) Stroke-in-evolution. (b) Active intracranial hemorrhage with a midline shift. (c) Typical ''string-of-beads'' sign affecting the vertebral arteries (arrows)
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 | Figure 2:(a) Left internal carotid artery angiogram demonstrating a beaded appearance (arrow) anterior-posterior view. (b) Selective left internal carotid artery angiogram. (c) Left vertebral artery
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| Discussion | | |
FMD, a genetic disorder, is rare among the Asian population.[1] This paper reports the first case from China. Less than 10% of FMD patients have problems with vertebral arteries.[1] The association of segmental FMD involving ICA, FMD affecting bilateral vertebral arteries causing subarachnoid hemorrhage is rare in medical literature. Research shows that alpha 1 antitrypsin deficiency is associated with arterial FMD.[2] However, despite having FMD our patient's lung and liver functions were normal. Bleeding could be due to (a) posterior cerebellar artery dissection, (b) recanalization of the artery, or (c) rupture of a very small aneurysm.[3] Although no rupture of the aneurysm was detected, we take this eventuality under consideration. The neurosurgeon advised draining the hematoma. The vascular surgeon suggested surgical correction by end-to-end anastomosis or carotid, and vertebral endovascular stenting. These measures were expected to provide excellent long-term patency and help prevent strokes in the future. However, such interventions should ideally be carried out by high volume surgeons as they have a higher success rate.[4] Surgical approach has only been adopted for symptomatic lesions.[5] Artery grafting has been advocated as the best treatment by Van Damme et al.[6] His patient neither suffered from postoperative mortality nor stroke after the artery grafting. In the case of this patient, his family refused surgical procedures. The patient was treated with calcium channel blocker for appropriate hypertension control and antiplatelets for stroke prevention. Interestingly, during the 8 months of follow-up, he reported improved health and was asymptomatic. As a precautionary measure, the family members of FMD patients should be screened in their 50 s (mean age 51.9 years) for arteries supplying the head and neck, heart, kidneys, and pelvis to prevent catastrophic complications because FMD is autosomal dominant.[5],[6] This case demonstrates that such patients may have a favorable prognosis even without any surgical procedure, but the question is for how long. However, FMD should be considered as a differential diagnosis for stroke patients.
Acknowledgment
I would like to thank Dr. Hong Jie Hu for granting me access to radiological data and Dr. Xiaojing Yu for advising on the selection and interpretation of the radiological images from Sir Run Run Shaw Hospital, College of Medicine, Zhejiang University Loma Linda University. I, Mithilesh Shibchurn, had full access to all the data in the study and take responsibility for the integrity of the data and accuracy of the data analysis.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Liu CH, Wu D, Chin SC, Fu SC, Wu TC, Chang CH, et al. Cervicocranial fibromuscular dysplasia in Taiwanese ischemic stroke patients. Eur Neurol 2012;67:129-35.  |
| 2. | Schievink WI, Katzmann JA, Piepgras DG. Alpha-1-antitrypsin deficiency in spontaneous intracranial arterial dissections. Cerebrovasc Dis 1998;8:42-4. |
| 3. | Lim SM, Choi IS, Hum BA, David CA. Dissecting aneurysms of the distal segment of the posterior inferior cerebellar arteries: Clinical presentation and management. AJNR Am J Neuroradiol 2010;31:1118-22. |
| 4. | Maas MB, Jaff MR, Rordorf GA. Risk adjustment for case mix and the effect of surgeon volume on morbidity. JAMA Surg 2013;148:532-6. |
| 5. | 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;99:163-8. |
| 6. | Olin JW, Froehlich J, Gu X, Bacharach JM, Eagle K, Gray BH, et al. The United States registry for fibromuscular dysplasia: Results in the first 447 patients. Circulation 2012;125:3182-90. |
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