{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T01:25:08Z","timestamp":1760059508309,"version":"build-2065373602"},"reference-count":24,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2025,6,16]],"date-time":"2025-06-16T00:00:00Z","timestamp":1750032000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background\/Objectives: Spinal muscular atrophy (SMA) is a neuromuscular disorder frequently associated with progressive scoliosis requiring posterior spinal fusion (PSF). While Nusinersen offers significant clinical benefit, its intrathecal administration is challenging in patients with extensive spinal instrumentation and solid fusion. This study aimed to evaluate the safety, feasibility, and patient acceptance of lumbar laminectomy as a method to restore intrathecal access for repeated Nusinersen delivery in this population. Methods: A retrospective review was conducted in eleven patients with SMA who underwent lumbar laminectomy following prior PSF and confirmed radiographic fusion. Surgical data, injection outcomes, and patient-reported experiences were collected. A structured questionnaire assessed technical success, imaging requirements, sedation, functional response, and satisfaction. Results: Nine out of eleven patients (81.8%) successfully initiated intrathecal Nusinersen therapy through the laminectomy window, receiving a mean of 11.7 injections (range: 10\u201314). Imaging guidance was used in five cases; three required sedation or anesthesia. Intraoperative dural tears occurred in three patients and were managed without complications. Eight out of nine treated patients reported subjective motor improvement and expressed willingness to undergo the procedure again. No hardware revisions or major adverse events were observed during a mean follow-up of 48.8 months. Conclusions: Lumbar laminectomy is a viable and well-tolerated technique to establish intrathecal access in SMA patients with prior PSF. This approach enables sustained drug delivery and may remain clinically relevant as new intrathecal therapies emerge.<\/jats:p>","DOI":"10.3390\/jcm14124280","type":"journal-article","created":{"date-parts":[[2025,6,16]],"date-time":"2025-06-16T09:51:22Z","timestamp":1750067482000},"page":"4280","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Surgical Access for Intrathecal Therapy in Spinal Muscular Atrophy with Spinal Fusion: Long-Term Outcomes of Lumbar Laminectomy"],"prefix":"10.3390","volume":"14","author":[{"given":"Tomasz","family":"Potaczek","sequence":"first","affiliation":[{"name":"Department of Orthopedic Surgery and Rehabilitation, Faculty of Medicine, Jagiellonian University, 34-500 Zakopane, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5226-6458","authenticated-orcid":false,"given":"S\u0142awomir","family":"Duda","sequence":"additional","affiliation":[{"name":"Department of Orthopedic Surgery and Rehabilitation, Faculty of Medicine, Jagiellonian University, 34-500 Zakopane, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jakub","family":"Adamczyk","sequence":"additional","affiliation":[{"name":"Department of Orthopedic Surgery and Rehabilitation, Faculty of Medicine, Jagiellonian University, 34-500 Zakopane, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,6,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1016\/0092-8674(95)90460-3","article-title":"Identification and characterization of a spinal muscular atrophy-determining gene","volume":"80","author":"Lefebvre","year":"1995","journal-title":"Cell"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1186\/s13023-017-0671-8","article-title":"Prevalence, incidence and carrier frequency of 5q-linked spinal muscular atrophy\u2014A literature review","volume":"12","author":"Verhaart","year":"2017","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1001\/archneur.1995.00540290108025","article-title":"Natural history in proximal spinal muscular atrophy. Clinical analysis of 445 patients and suggestions for a modification of existing classifications","volume":"52","author":"Zerres","year":"1995","journal-title":"Arch. Neurol."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Fujak, A., Raab, W., Schuh, A., Richter, S., Forst, R., and Forst, J. (2013). Natural course of scoliosis in proximal spinal muscular atrophy type II and IIIa: Descriptive clinical study with retrospective data collection of 126 patients. BMC Musculoskelet. Disord., 14.","DOI":"10.1186\/1471-2474-14-283"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"760","DOI":"10.1097\/00007632-198907000-00019","article-title":"Spinal muscular atrophy: Natural history and orthopaedic treatment of scoliosis","volume":"14","author":"Granata","year":"1989","journal-title":"Spine"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"e149","DOI":"10.1212\/WNL.0000000000007742","article-title":"Natural course of scoliosis and lifetime risk of scoliosis surgery in spinal muscular atrophy","volume":"93","author":"Wijngaarde","year":"2019","journal-title":"Neurology"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"388","DOI":"10.1097\/00007632-198405000-00011","article-title":"The Galveston technique of pelvic fixation with L-rod instrumentation of the spine","volume":"9","author":"Allen","year":"1984","journal-title":"Spine"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"S75","DOI":"10.1007\/s00586-011-1758-x","article-title":"Cotrel-Dubousset instrumentation in neuromuscular scoliosis","volume":"20","author":"Piazzolla","year":"2011","journal-title":"Eur. Spine J."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1007\/s00586-009-1198-z","article-title":"Treatment and complications in flaccid neuromuscular scoliosis (Duchenne muscular dystrophy and spinal muscular atrophy) with posterior-only pedicle screw instrumentation","volume":"19","author":"Modi","year":"2010","journal-title":"Eur. Spine J."},{"key":"ref_10","first-page":"A02200100","article-title":"Nusinersen treatment of spinal muscular atrophy\u2014A systematic review","volume":"67","author":"Albrechtsen","year":"2020","journal-title":"Dan. Med. J."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1186\/s13023-023-02769-4","article-title":"Long-term nusinersen treatment across a wide spectrum of spinal muscular atrophy severity: A real-world experience","volume":"18","author":"Baranowski","year":"2023","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"e620","DOI":"10.1212\/WNL.0000000000006006","article-title":"Cervical puncture to deliver nusinersen in patients with spinal muscular atrophy","volume":"91","author":"Veerapandiyan","year":"2018","journal-title":"Neurology"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"986","DOI":"10.3174\/ajnr.A5596","article-title":"Transforaminal Lumbar Puncture: An Alternative Technique in Patients with Challenging Access","volume":"39","author":"Nascene","year":"2018","journal-title":"AJNR Am. J. Neuroradiol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1055\/s-0040-1715481","article-title":"Multicenter Experience with Nusinersen Application via an Intrathecal Port and Catheter System in Spinal Muscular Atrophy","volume":"51","author":"Hahn","year":"2020","journal-title":"Neuropediatrics"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"899","DOI":"10.1177\/0883073815627882","article-title":"Intrathecal Injections in Children With Spinal Muscular Atrophy: Nusinersen Clinical Trial Experience","volume":"31","author":"Swoboda","year":"2016","journal-title":"J. Child Neurol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"E4","DOI":"10.1002\/mus.26082","article-title":"Transforaminal lumbar puncture for intrathecal nusinersen administration","volume":"58","author":"Geraci","year":"2018","journal-title":"Muscle Nerve"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1007\/s00415-018-9124-0","article-title":"Intrathecal administration of nusinersen in adolescent and adult SMA type 2 and 3 patients","volume":"266","author":"Wurster","year":"2019","journal-title":"J. Neurol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1422","DOI":"10.3174\/ajnr.A6131","article-title":"The Complex Spine in Children with Spinal Muscular Atrophy: The Transforaminal Approach-A Transformative Technique","volume":"40","author":"Towbin","year":"2019","journal-title":"AJNR Am. J. Neuroradiol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1007\/s00247-018-4240-7","article-title":"Ultrasound-guided cervical puncture for nusinersen administration in adolescents","volume":"49","author":"Ortiz","year":"2019","journal-title":"Pediatr. Radiol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1007\/s00586-003-0544-9","article-title":"Spinal stenosis surgery in Sweden 1987\u20131999","volume":"12","author":"Jansson","year":"2003","journal-title":"Eur. Spine J."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"217","DOI":"10.3171\/2019.2.SPINE181366","article-title":"Lumbar laminotomy for the intrathecal administration of nusinersen for spinal muscular atrophy: Technical note and outcomes","volume":"31","author":"Ko","year":"2019","journal-title":"J. Neurosurg. Spine"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"e610","DOI":"10.1097\/BPO.0000000000001247","article-title":"Preliminary Safety and Tolerability of a Novel Subcutaneous Intrathecal Catheter System for Repeated Outpatient Dosing of Nusinersen to Children and Adults With Spinal Muscular Atrophy","volume":"38","author":"Strauss","year":"2018","journal-title":"J. Pediatr. Orthop."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"CASE24411","DOI":"10.3171\/CASE24411","article-title":"Fiducial-marked laminectomy window for intrathecal medication administration after spinal fusion: Illustrative case","volume":"8","author":"Still","year":"2024","journal-title":"J. Neurosurg. Case Lessons"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1186\/s13023-023-02972-3","article-title":"Unilateral interlaminar fenestration on the convex side provides a reliable access for intrathecal administration of nusinersen in spinal muscular atrophy: A retrospective study","volume":"18","author":"Wang","year":"2023","journal-title":"Orphanet J. Rare Dis."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/14\/12\/4280\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T17:52:53Z","timestamp":1760032373000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/14\/12\/4280"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,6,16]]},"references-count":24,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2025,6]]}},"alternative-id":["jcm14124280"],"URL":"https:\/\/doi.org\/10.3390\/jcm14124280","relation":{},"ISSN":["2077-0383"],"issn-type":[{"type":"electronic","value":"2077-0383"}],"subject":[],"published":{"date-parts":[[2025,6,16]]}}}