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Tricky case of Takayasu arteritis in a young child presenting with heart failure and femoral pulses
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  • Published on:
    Is it really Takayasu arteritis or could it be fibromuscular dysplasia?
    • Kjell Tullus, Consultant Paediatric Nephrologist Great Ormond Street Hospital for Children

    Fabi et al presented in the recent issue of Archives in Diseases in Childhood a 6 year old girl with left renal artery stenosis, occlusion of the right renal artery and narrowed aorta.1 The vascular diagnosis in the child was done very accurately with percutaneous angiography and she also received successful treatment with angioplasty. I would very much support the author’s opinion on the anatomical diagnosis and how it was treated.
    I would however like to challenge their primary diagnoses of this child. They choose to call this Takaysu Arteritis (TA). I would suggest that fibromuscular dysplasia (FMD) or a genetic syndrome like Neurofibromatosis type 1 or Williams’s syndrome is much more likely.2 The angiographic appearances of all these conditions can be indistinguishable.3;4 It is however true that this child, as does all our children in our large renovascular service at Great Ormond Street Hospital, fulfil the published criteria for Takayasu.5 This is actually a very big problem as these conditions very often get confused with each other.
    I would argue that you will need something more for the TA diagnosis. This can be a convincing history and blood parameters of a systemic vasculitis. It could also be that imaging shows a thickened aortic wall or uptake on a PET scan suggestive of vascular inflammation.6;7 Without any such findings I suggest that it is wiser to label this child as having FMD. We have seen several cases where children have been diagnosed...

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    Conflict of Interest:
    None declared.