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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

Boby Varkey Maramattom1, Joe Thomas2, Dilip Panicker3, CV Gopalakrishnan1,  
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

Correspondence Address:
Dr. Boby Varkey Maramattom
Department of Neurology, Aster Medcity, Kochi, Kerala
India




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;70:792-793


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 Aug 8 ];70:792-793
Available from: https://www.neurologyindia.com/text.asp?2022/70/2/792/344601


Full Text



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}

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}{Table 2}

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

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3Fujimoto 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.
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