Skip constructs in spinal muscular atrophy: outcomes of a novel approach for posterior spinal instrumentation and fusion
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Skip constructs in spinal muscular atrophy: outcomes of a novel approach for posterior spinal instrumentation and fusion Matthew W. Konigsberg1 · Hiroko Matsumoto1 · Jacob R. Ball1 · Benjamin D. Roye1 · Michael G. Vitale1 Received: 11 February 2020 / Accepted: 16 March 2020 © Scoliosis Research Society 2020
Abstract Purpose Many children with spinal muscular atrophy (SMA) develop progressive spinal deformity, worsening already compromised pulmonary function and global spinal balance. Early results demonstrate that intrathecal administration of nusinersen, a recent FDA-approved drug, improves motor function and ventilator-free survival, necessitating preservation of intrathecal access when considering PSIF. The purpose of this study is to assess medium-term outcomes of a specialized approach for posterior spinal instrumentation and fusion (PSIF) to preserve intrathecal access in patients with SMA. Methods A retrospective review of patients with SMA undergoing PSIF at a single tertiary academic medical center during a 3-year period was completed. To facilitate intrathecal drug administration, the traditional approach to PSIF was modified to “skip” one or more intervertebral levels at the thoracolumbar junction. Clinical notes and radiographs were reviewed for postoperative outcomes including major coronal curve correction and complications, including loss of correction, hardware failure and surgical revision. Results Eight patients were identified, with a mean age of 12.7 ± 1.6 years and follow-up of 4 years. These patients had a mean preoperative major coronal curve of 56.4°, with mean curve correction of 35.2°. At follow-up, no patients experienced rod breakage, loss of correction, or postoperative chronic pain. Only one patient required revision surgery due to bony overgrowth at the skipped level after three and a half years. Conclusion Implementing the skip construct approach for PSIF in patients with SMA allows for scoliosis correction without compromising intrathecal drug delivery. Follow-up at 4 years reveals no adverse clinical events, hardware failure or loss of correction. Level of evidence IV. Keywords Spinal muscular atrophy · Posterior spinal fusion · Early onset scoliosis · Nusinersen · Scoliosis
Introduction Spinal muscular atrophy (SMA), a disorder caused by degeneration of the anterior horn cells in the spinal cord, is a leading neuromuscular cause of infant mortality. SMA is the third most commonly diagnosed neuromuscular disorder in pediatric patients and presents with a wide range of clinical manifestations, including proximal muscle weakness, * Michael G. Vitale [email protected] 1
Department of Pediatric Orthopaedic Surgery, Morgan Stanley Children’s Hospital of New York Presbyterian, Columbia University Medical Center, ATTN: Michael Vitale, 3959 Broadway, CHONY 8‑N, New York, NY 10032‑3784, USA
worsening pulmonary function, and global spinal imbalance [1, 2, 3, 4]. Although the severity of SMA is related to the age of clinical onset [5, 6, 7], recent advances in ta
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