French-Belgian consensus statement to managing spinal deformities in children with spinal muscular atrophy treated with SMN restoring therapies.

BACKGROUND

Spinal muscular atrophy (SMA) affects the motor neurons of the anterior spinal cord, causing progressive muscle weakness and atrophy. SMN restoring therapies have led to the emergence of new phenotypes in spinal muscular atrophy (SMA), including early-onset scoliosis. Guidelines regarding the optimal treatment for scoliosis and its consequences are lacking.

OBJECTIVE

To provide guidelines to help clinical decision-making and to standardise the management of neuromuscular scoliosis (NMS) in children with SMA.

METHODS

We conducted a Delphi study with 31 experts between July 2023 and February 2024. Three rounds were conducted using anonymous electronic questionnaires to determine consensus on items grouped into 5 main domains: Respiratory monitoring (7 items), Trunk orthoses (4 items), Surgical approaches (13 items), Preoperative care (17), post-operative care (10 items). Experts were asked to rate their agreement with each item on a scale from 1 (strongly disagree) to 9 (strongly agree). Consensus was considered to have been achieved if the median value of the responses was ≥ 7. Items that did not reach consensus in one round were reformulated in the following round using comments provided by the experts.

RESULTS

The experts included 12 orthopaedic surgeons, 9 physical medicine and rehabilitation physicians and 10 paediatricians/child neurologists. Fourty-seven items achieved consensus. In the first round, consensus was achieved for 33 of the 51 items. The second round included 15 items, and a consensus was achieved for 11 items. In the third round, 3 of the 4 items included achieved consensus and the fourth item was dropped due to a lack of agreement.

CONCLUSIONS

The recommendations generated from the questionnaire provide a relevant consensus-based guidance for the multidisciplinary management of spinal deformities in children with SMA.