Safety and efficacy of growth-friendly instrumentation for early-onset scoliosis in patients with spinal muscular atrophy type 1 in the disease-modifying treatment era.

Autor: Cetik RM; Orthopedics and Traumatology, Pursaklar State Hospital, Ankara, Turkey., Ovadia D; Dana-Dwek Children's Hospital, Tel Aviv Medical Center, Tel Aviv, Israel., Mladenov K; Department of Pediatric Orthopaedic Surgery, Children's Hospital Hamburg-Altona, University Medical Center Hamburg-Eppendorf, Hamburg, Germany., Kruyt MC; Department of Orthopedic Surgery, University Medical Center Utrecht, Utrecht, The Netherlands., Helenius I; Department of Orthopaedics and Traumatology, Helsinki University Hospital, University of Helsinki, Helsinki, Finland., Ahonen M; Department of Pediatric Orthopedics and Traumatology, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland., Studer D; Department of Orthopedics, University Children's Hospital Basel (Universitats Kinderspital beider Basel), Basel, Switzerland., Yazici M; Department of Orthopedics and Traumatology, Hacettepe University Hospital, Ankara, Turkey.
Jazyk: angličtina
Zdroj: Journal of children's orthopaedics [J Child Orthop] 2023 Nov 27; Vol. 18 (1), pp. 26-32. Date of Electronic Publication: 2023 Nov 27 (Print Publication: 2024).
DOI: 10.1177/18632521231214780
Abstrakt: Purpose: To evaluate the safety of growth-friendly instrumentation for early-onset scoliosis (EOS) in patients with spinal muscular atrophy (SMA) type 1 who received disease-modifying treatment (DMT) and analyze short-term efficacy.
Methods: Retrospective search was conducted between 2017 and 2023. Patients with genetically confirmed SMA type 1 who were surgically treated for spinal deformity and receiving DMTs (nusinersen, risdiplam, or onasemnogene abeparvovec) were included. SMA types 2 and 3 and patients who do not receive DMTs were excluded. Clinical and radiographic data were collected at preoperative, postoperative, and latest follow-up visits.
Results: Twenty-eight patients (mean follow-up: 16 months (range 2-41)) were included. The mean age at surgery was 60 months (range 29-96). Fifteen were treated with dual magnetically controlled growing rods (MCGR), four with unilateral MCGR and a contralateral guided growth system, three with Vertical Expandable Prosthetic Titanium Rib (VEPTR®) implants, five with self-distracting systems, and one with traditional dual growing rods. The mean amount of correction was 57% (44°± 17) for scoliosis and 83% (13°± 11) for pelvic obliquity. The mean T1-12 height gain during surgery was 31 mm (±16 mm), while the mean T1 S1 height gain was 51 mm (±24 mm), and instrumented growth was observed during follow-up. Five patients (18%) developed six serious adverse events: three surgical site infections, two anchor failures, and one rod fracture, and all required unplanned reoperations. No neurologic complication, difficulty during nusinersen injections, or respiratory decline was recorded.
Conclusion: We report that spinal deformity in this population can be safely treated with growth-friendly instrumentation, with similar complication rates when compared with SMA type 2.
Competing Interests: The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: IH reports receiving grants to study group (Finnish Pediatric Research Foundation), research grants to institution (Medtronic, Stryker, Nuvasive and Cerapedics), consultant fees (Medtronic), payment for lectures (Nuvasive), and support for traveling expenses (Medtronic). MA reports receiving research grants (Finnish Pediatric Research Foundation). MCK reports ownership (Cresco Spine) and inventor rights (Spring distraction system). DO reports reimbursement of expenses for a lecture (Nuvasive). MY reports holding the position of Secretary in the Pediatric Spine Foundation.
(© The Author(s) 2023.)
Databáze: MEDLINE