Assessment of the perioperative status in children with SMA receiving nusinersen and undergoing reconstructive orthopedic interventions

Abstract

Surgical methods for treatment of spinal muscular atrophy (SMA) in children are aimed at improving their positioning and maintaining the ability of verticalization. Assessment of neurological, somatic and motor status of patients, individual selection of drugs for anesthesia and sedation at the stage of planning surgical interventions allow the anesthesiologist/resuscitator to avoid perioperative adverse events. Purpose To compare the course of the perioperative period in children with SMA who received pathogenetic therapy with nusinersen with the control group during orthopedic correction of acquired skeletal deformities. Materials and methods The retrospective analysis for the period from 2019 to 2021 included 23 children. Depending on the ongoing pathogenetic therapy, children with SMA were divided into 2 groups. The main group (SMA+N) included 9 children who received nusinersen; the control group (CG) included 14 children without antisense oligonucleotide therapy. Co-morbidities, hemodynamic parameters, blood loss, need for analgesics and complications were studied. Results Insignificant differences between the groups were recorded based on the Hammersmith Extended Scale (HFMSE). At the same time, similar comorbid pathology, the severity of respiratory failure, and the absence of differences in the frequency of the NIV application indicated that the groups were comparable. This is probably due to the late start of SMA treatment, degenerative changes in motor neurons, and fatty degeneration of muscle tissue. Conclusion Intake of nusinersen in patients with SMA type II-III and a long period of illness, severe neurological and respiratory disorders do not lead to a significant regression of symptoms in the perioperative period. The therapy with antisense oligonucleotides in severe muscle hypotonia does not exclude the risk of adverse events in the perioperative period in children with SMA type II-III during orthopedic correction of skeletal deformities.

Keywords

• spinal muscular atrophy
• neurological status
• pathogenetic therapy
• orthopaedic interventions