ESPE Abstracts (2021) 94 FC6.6

ESPE2021 Free Communications Bone and Mineral Metabolism (6 abstracts)

Use of lateral DEXA scanning for identifying and monitoring vertebral fractures in patients affected by Duchenne Muscular Dystrophy.

Lucy Turner 1 , Jennifer Lemon 2 , Caren Landes 3 , Rajesh Madhu 3 , Poonam Dharmaraj 3 & Stefan Spinty 3


1St Helen’s & Knowsley NHS Foundation Trust, Liverpool, United Kingdom.;2Warrington & Halton NHS Foundation Trust, Warrington, United Kingdom.;3Alder Hey Children’s Hospital NHS Foundation Trust, Liverpool, United Kingdom


Introduction: International guidelines for management of Duchenne Muscular Dystrophy (DMD) advise active screening for vertebral fractures (VF), complications of which include pain, scoliosis and impact on ambulation. Vertebral fracture assessment (VFA) is a technique of visualising thoracic and lumbar vertebrae with a lateral view on dual energy X-ray absorptiometry (DEXA) to identify VF. This is at reduced cost and radiation exposure when compared to spinal X-ray and is more convenient for the non-ambulant. Previous studies have shown VFA to be a useful tool in actively screening for VF in DMD. Our aims were to assess whether similar outcomes would be seen in our department when introducing routine VFA screening.

Method: We undertook an audit on bone health specifically looking at VF and vitamin D status in boys with DMD exposed to corticosteroids, who were seen in outpatient clinic at a large paediatric neuromuscular centre after routine VFA screening was introduced.

Results: 37 patients were included for analysis. Median age was 10.2yrs (4-5-17.8yrs). Median steroid duration was 4.6yrs (0.99-12.6yrs). 62.2% (n = 23) were taking deflazacort, 32.4% (n = 12) taking prednisolone and in 5.4% (n = 2) steroids had been discontinued. 48.6% (n = 18) of VFA scans showed signs of VF. Of these, 72.2% (n = 13) showed newly identified fractures, 16.7% (n = 3) showed stable old fractures and 11.1% (n = 2) were old fractures that had progressed. Five boys had fractures that were reported as “possible” due to difficulties visualising the vertebrae. One boy with inadequate imaging on VFA underwent subsequent spinal x-ray highlighting multi-level fracture. Of the 19 with VF, 42.1% (n = 8) were single level and 57.9% (n = 11) were multi-level. Median lumbar spine BMD z scores of those with and without VF was -1.65 (-3.1 - -0.2) and -1.05 (-2.9 - +1.1) respectively. Median vitamin D of boys with VF was 57.5nmol/l (38-93) and 44.5nmol/l (19-81) in those without VF. 84.2% (n = 16) of all boys with VF were asymptomatic. Of all 37 who underwent VFA, 35.1% (n = 13) had a change in management following the image findings; five started zoledronic acid, three recommenced zoledronic acid and five were referred for testosterone initiation.

Conclusion: Our findings parallel that of prior studies and support the use of VFA as a screening tool for identifying VF in boys affected by DMD. This adds weight to the current evidence base that forms the recommendations for routine spinal imaging in DMD and promotes proactive bone health surveillance.

Volume 94

59th Annual ESPE (ESPE 2021 Online)

Online,
22 Sep 2021 - 26 Sep 2021

European Society for Paediatric Endocrinology 

Browse other volumes

Article tools

My recent searches

No recent searches.