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LETTER TO EDITOR
Year : 2022  |  Volume : 70  |  Issue : 2  |  Page : 792-793

Extensive Fibromuscular Dysplasia in a Young Girl Treated with Bilateral STA-MCA Bypass


1 Department of Neurology, Aster Medcity, Kochi, Kerala, India
2 Department of Rheumatology, Aster Medcity, Kochi, Kerala, India
3 Department of 2Neurosurgery, Aster Medcity, Kochi, Kerala, India

Date of Submission05-Jan-2020
Date of Decision18-Aug-2020
Date of Acceptance19-Aug-2021
Date of Web Publication3-May-2022

Correspondence Address:
Dr. Boby Varkey Maramattom
Department of Neurology, Aster Medcity, Kochi, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.344601

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How to cite this article:
Maramattom BV, Thomas J, Panicker D, Gopalakrishnan C V. Extensive Fibromuscular Dysplasia in a Young Girl Treated with Bilateral STA-MCA Bypass. Neurol India 2022;70:792-3

How to cite this URL:
Maramattom BV, Thomas J, Panicker D, Gopalakrishnan C V. Extensive Fibromuscular Dysplasia in a Young Girl Treated with Bilateral STA-MCA Bypass. Neurol India [serial online] 2022 [cited 2022 May 19];70:792-3. Available from: https://www.neurologyindia.com/text.asp?2022/70/2/792/344601




Dear Editor,

A 14 year-old girl was diagnosed with Takayasu's arteritis [TA] after developing a mid-aortic syndrome [MAS]. Following endovascular stenting, she was started on Wysolone 40mg OD and Mycophenolate mofetil 500 mg BD. Ten days post-op, she developed a right hemiplegia with global aphasia.

MRI brain showed multiple subcortical infarcts and a new left frontal infarct. DSA showed bilateral ICA occlusion and a V-B junction stenosis >90% with highly tortuous vertebral arteries. V-B junction stenting was attempted unsuccessfully. On day 20, she underwent a sequential STA-MCA bypass 2 weeks apart 1 month after admission [right followed by left]; with an STA biopsy on both occasions, which confirmed Fibromuscular dyspasia [FMD] [Figure 1]. Immunosuppressants were stopped, but antiplatelets were continued. At 6 months follow up she was ambulant, but had residual motor aphasia. Whole exome sequencing for genetic causes of stroke was negative.
Figure 1: Panel a; MRA shows bilateral ICA occlusion with tortuous vertebral arteries. Panel b; DWMRI shows acute left frontal infarct [arrow] Panel c; FLAIR axial MRI images shows bilateral subcortical white matter hyperintensities. Panel d; Reconstructed CTA shows the thoracic aorta stent in situ and the cerebrovascular abnormalities [aorta]. Panel e; DSA showing tortuous right vertebral artery with a segmental dissection [arrow]. Panel f; DSA showing tortuous left VA [arrow] with a >90% VB junction stenosis [arrow] Panel g; MIP CTA showing bilateral patent STA-MCA bypasses [arrows]. Panel h and i; histopathology demonstrating intimal FMD

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FMD is a segmentary non-atherosclerotic, non-inflammatory vasculopathy affecting medium and small arteries. It has a female predominance [F; M 9:1] and classically affects the renal and cervico-cranial vasculature [c-FMD].

3 subtypes of FMD are described; The commonest [Medial FMD] is characterized angiographically by a multi-focal alternating stenosis and dilatation [string-of-beads appearance]. The sub-adventitial type shows a tubular stenosis [>1 cm length] and the rarest type, [Intimal type] shows a short focal stenosis of the artery [<1 cm length]. cFMD can be divided into 2 subtypes: a multifocal FMD (≥2 stenoses in any vessel segment ± a string of-beads appearance) or unifocal FMD (a single area of focal or tubular stenosis).[1]

The clinical manifestations and differential diagnosis of FMD are vast [Table 1] and [Table 2]. At present there are no validated diagnostic criteria for FMD. Our child had features of both c-TA and FMD and only STA biopsy was able to confirm FMD.[2]
Table 1: Neurological manifestations of FMD

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Table 2: Differential diagnosis of cervico-cephalic FMD

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FMD is treated with antiplatelets, anti-hypertensives, endovascular interventions or STA-MCA bypass.[3] Immunosuppressants are unhelpful. This is the first report from India of bilateral STA-MCA bypass in FMD and demonstrates the utility of sending STA biopsies during a bypass.[4]

Acknowledgements

We acknowledge the help of Dr Nanda Kacchare from the department of Neuropathology, Aster medcity in the interpretation of arterial biopsy slides.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Savard S, Steichen O, Azarine A, AziziM, Jeunemaitre X, Plouin PF. Association between 2 angiographic subtypes of renal artery fibromuscular dysplasia and clinical characteristics. Circulation 2012;126:30629.  Back to cited text no. 1
    
2.
de Graeff N, Groot N, Brogan P, Ozen S, Avcin T, Bader-Meunier B, et al. European consensus-based recommendations for the diagnosis and treatment of rare paediatric vasculitides-the SHARE initiative. Rheumatology (Oxford) 2019;58:656-71.  Back to cited text no. 2
    
3.
Fujimoto S, Kayama T, Ogawa A, Sakurai Y, Yoshimoto T, Suzuki J. Two cases ofintracranial fibromuscular dysplasia whose repeated angiography disclosed progression of the lesion. No To Shinkei 1987;39:937-45.  Back to cited text no. 3
    
4.
Schmidt WA, Blockmans D. Investigations in systemic vasculitis-The role of imaging. Best Pract Res Clin Rheumatol 2018;32:63-82.  Back to cited text no. 4
    


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