{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T22:33:37Z","timestamp":1791844417072,"version":"4.3.4"},"reference-count":34,"publisher":"Springer Science and Business Media LLC","issue":"4","license":[{"start":{"date-parts":[[2021,3,8]],"date-time":"2021-03-08T00:00:00Z","timestamp":1615161600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,3,8]],"date-time":"2021-03-08T00:00:00Z","timestamp":1615161600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Spine Deform"],"published-print":{"date-parts":[[2021,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Scoliosis often occurs coincident with pulmonary function deterioration in spinal muscular atrophy but a causal relationship has not yet been reliably established. A systematic literature review was performed, with pulmonary function testing being the primary outcome pre- and post-scoliosis surgery. Levels of evidence were determined and GRADE recommendations made. Ninety studies were identified with only 14 meeting inclusion criteria. Four studies were level III and the rest were level IV evidence. The average age at surgical intervention was 11.8\u00a0years (follow-up 6.1\u00a0years). Post-operative pulmonary function progressively declined for the majority of studies. Otherwise, pulmonary function: improved (two studies), were unchanged (two studies), had a decreased rate of decline (three studies), declined initially then returned to baseline (two studies). Respiratory and spine-based complications were common. Given the available evidence, the following GRADE C recommendations were made: (1) surgery is most often associated with decreases in pulmonary function; (2) the impact of surgery on pulmonary function is variable, but does not improve over pre-operative baseline; (3) surgery may result in a decreased rate of decline in pulmonary function post-operatively. Given this lack of evidence-based support, the risk\u2013benefit balance should be taken into consideration when contemplating scoliosis surgery.<\/jats:p>","DOI":"10.1007\/s43390-021-00302-w","type":"journal-article","created":{"date-parts":[[2021,3,8]],"date-time":"2021-03-08T12:02:42Z","timestamp":1615204962000},"page":"913-921","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["The impact of scoliosis surgery on pulmonary function in spinal muscular atrophy: a systematic review"],"prefix":"10.1007","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5721-3029","authenticated-orcid":false,"given":"Abduljabber","family":"Alhammoud","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yahya","family":"Othman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ron","family":"El-Hawary","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William G.","family":"Mackenzie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jason J.","family":"Howard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,3,8]]},"reference":[{"issue":"8","key":"302_CR1","doi-asserted-by":"publisher","first-page":"1179","DOI":"10.2106\/00004623-198264080-00008","volume":"64","author":"H Aprin","year":"1982","unstructured":"Aprin H, Bowen JR, MacEwen GD, Hall JE (1982) Spine fusion in patients with spinal muscular atrophy. J Bone JtSurg 64(8):1179\u20131187","journal-title":"J Bone JtSurg"},{"key":"302_CR2","doi-asserted-by":"crossref","unstructured":"Barsdorf AI, Sproule DM, Kaufmann P (2010) Scoliosis surgery in children with neuromuscular disease: findings from the US National Inpatient Sample, 1997\u20132003. Arch Neurol. 67(2). Available from: http:\/\/archneur.jamanetwork.com\/article.aspx?doi=10.1001\/archneurol.2009.296. Cited 12 Oct 2020","DOI":"10.1001\/archneurol.2009.296"},{"issue":"7","key":"302_CR3","doi-asserted-by":"publisher","first-page":"763","DOI":"10.1097\/00007632-198907000-00020","volume":"14","author":"JC Brown","year":"1989","unstructured":"Brown JC, Zeller JL, Swank SM, Furumasu J, Warath SL (1989) Surgical and functional results of spine fusion in spinal muscular atrophy. Spine (Phila PA 1976). 14(7):763\u2013770","journal-title":"Spine (Phila PA 1976)."},{"issue":"9","key":"302_CR4","doi-asserted-by":"publisher","first-page":"673","DOI":"10.1046\/j.1440-1754.2003.00266.x","volume":"39","author":"SY Chng","year":"2003","unstructured":"Chng SY, Wong YQ, Hui JH, Wong HK, Ong HT, Goh DY (2003) Pulmonary function and scoliosis in children with spinal muscular atrophy types II and III. J Paediatr Child Health 39(9):673\u2013676","journal-title":"J Paediatr Child Health"},{"issue":"2","key":"302_CR5","doi-asserted-by":"publisher","first-page":"82","DOI":"10.4184\/asj.2010.4.2.82","volume":"4","author":"HS Chong","year":"2010","unstructured":"Chong HS, Moon ES, Kim HS, Ankur N, Park JO, Kim JY et al (2010) Comparison between operated muscular dystrophy and spinal muscular atrophy patients in terms of radiological, pulmonary and functional outcomes. Asian Spine J 4(2):82","journal-title":"Asian Spine J"},{"issue":"6","key":"302_CR6","doi-asserted-by":"publisher","first-page":"1721","DOI":"10.1007\/s00586-016-4828-2","volume":"26","author":"S-H Chou","year":"2017","unstructured":"Chou S-H, Lin G-T, Shen P-C, Lue Y-J, Lu C-C, Tien Y-C et al (2017) The effect of scoliosis surgery on pulmonary function in spinal muscular atrophy type II patients. Eur Spine J 26(6):1721\u20131731","journal-title":"Eur Spine J"},{"issue":"1","key":"302_CR7","first-page":"63","volume":"36","author":"K Chua","year":"2016","unstructured":"Chua K, Tan CY, Chen Z, Wong HK, Lee EH, Tay SKH et al (2016) Long-term follow-up of pulmonary function and scoliosis in patients with Duchenne\u2019s muscular dystrophy and spinal muscular atrophy. J PediatrOrthop 36(1):63\u201369","journal-title":"J PediatrOrthop"},{"key":"302_CR8","doi-asserted-by":"publisher","first-page":"1153","DOI":"10.2147\/TCRM.S172291","volume":"15","author":"T Dangouloff","year":"2019","unstructured":"Dangouloff T, Servais L (2019) Clinical evidence supporting early treatment of patients with spinal muscular atrophy: current perspectives. TherClin Risk Manag 15:1153\u20131161","journal-title":"TherClin Risk Manag"},{"issue":"4","key":"302_CR9","doi-asserted-by":"publisher","first-page":"329","DOI":"10.1136\/thx.17.4.329","volume":"17","author":"MS Dunnill","year":"1962","unstructured":"Dunnill MS (1962) Postnatal growth of the lung. Thorax 17(4):329\u2013333","journal-title":"Thorax"},{"issue":"4","key":"302_CR10","first-page":"1037","volume":"55","author":"HJ Farber","year":"2020","unstructured":"Farber HJ, Phillips WA, Kocab KL, Hanson DS, Heydemann JA, Dahl BT et al (2020) Impact of scoliosis surgery on pulmonary function in patients with muscular dystrophies and spinal muscular atrophy. PediatrPulmonol 55(4):1037\u20131042","journal-title":"PediatrPulmonol"},{"issue":"18","key":"302_CR11","doi-asserted-by":"publisher","first-page":"1723","DOI":"10.1056\/NEJMoa1702752","volume":"377","author":"RS Finkel","year":"2017","unstructured":"Finkel RS, Mercuri E, Darras BT, Connolly AM, Kuntz NL, Kirschner J et al (2017) Nusinersen versus Sham control in infantile-onset spinal muscular atrophy. N Engl J Med 377(18):1723\u20131732","journal-title":"N Engl J Med"},{"issue":"12","key":"302_CR12","doi-asserted-by":"publisher","first-page":"1697","DOI":"10.1007\/s00402-012-1610-8","volume":"132","author":"A Fujak","year":"2012","unstructured":"Fujak A, Raab W, Schuh A, Kre\u00df A, Forst R, Forst J (2012) Operative treatment of scoliosis in proximal spinal muscular atrophy: results of 41 patients. Arch Orthop Trauma Surg 132(12):1697\u20131706","journal-title":"Arch Orthop Trauma Surg"},{"issue":"14","key":"302_CR13","doi-asserted-by":"publisher","first-page":"283","DOI":"10.1186\/1471-2474-14-283","volume":"4","author":"A Fujak","year":"2013","unstructured":"Fujak A, Raab W, Schuh A, Richter S, Forst R, 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 MusculoskeletDisord 4(14):283","journal-title":"BMC MusculoskeletDisord"},{"issue":"3","key":"302_CR14","first-page":"207","volume":"3","author":"C Granata","year":"1993","unstructured":"Granata C, Cervellati S, Ballestrazzi A, Corbascio M, Merlini L (1993) Spine surgery in spinal muscular atrophy: long-term results. NeuromusculDisord 3(3):207\u2013215","journal-title":"NeuromusculDisord"},{"issue":"8","key":"302_CR15","first-page":"e505","volume":"37","author":"JB Holt","year":"2017","unstructured":"Holt JB, Dolan LA, Weinstein SL (2017) Outcomes of primary posterior spinal fusion for scoliosis in spinal muscular atrophy: clinical, radiographic, and pulmonary outcomes and complications. J PediatrOrthop 37(8):e505\u2013e511","journal-title":"J PediatrOrthop"},{"key":"302_CR16","doi-asserted-by":"crossref","unstructured":"Howard JJ, Farrelly J (2017) Evidence-based treatment of neuromuscular scoliosis. In: Alshryda S, Huntley JS, Banaszkiewicz PA (eds) Paediatric orthopaedics. Springer International Publishing, Cham, pp 213\u201328. Available from: http:\/\/link.springer.com\/10.1007\/978-3-319-41142-2_24. Cited 12 Oct 2020","DOI":"10.1007\/978-3-319-41142-2_24"},{"issue":"5","key":"302_CR17","first-page":"1323","volume":"469","author":"LA Karol","year":"2011","unstructured":"Karol LA (2011) Early definitive spinal fusion in young children: what we have learned. ClinOrthopRelat Res 469(5):1323\u20131329","journal-title":"ClinOrthopRelat Res"},{"issue":"6","key":"302_CR18","first-page":"552","volume":"17","author":"S Khirani","year":"2013","unstructured":"Khirani S, Colella M, Caldarelli V, Aubertin G, Boul\u00e9 M, Forin V et al (2013) Longitudinal course of lung function and respiratory muscle strength in spinal muscular atrophy type 2 and 3. Eur J PaediatrNeurol 17(6):552\u2013560","journal-title":"Eur J PaediatrNeurol"},{"issue":"8","key":"302_CR19","first-page":"e500","volume":"37","author":"RL Lenhart","year":"2017","unstructured":"Lenhart RL, Youlo S, Schroth MK, Noonan KJ, McCarthy J, Mann D et al (2017) Radiographic and respiratory effects of growing rods in children with spinal muscular atrophy. J PediatrOrthop 37(8):e500\u2013e504","journal-title":"J PediatrOrthop"},{"issue":"13","key":"302_CR20","doi-asserted-by":"publisher","first-page":"E780","DOI":"10.1097\/BRS.0000000000000911","volume":"40","author":"K Livingston","year":"2015","unstructured":"Livingston K, Zurakowski D, Snyder B, Growing Spine Study Group, Children\u02bcs Spine Study Group (2015) Parasol rib deformity in hypotonic neuromuscular scoliosis: a new radiographical definition and a comparison of short-term treatment outcomes with VEPTR and growing rods. Spine (Phila Pa 1976). 40(13):E780-786","journal-title":"Spine (Phila Pa 1976)."},{"issue":"16","key":"302_CR21","doi-asserted-by":"publisher","first-page":"1305","DOI":"10.1097\/BRS.0b013e3182194937","volume":"36","author":"MJ McElroy","year":"2011","unstructured":"McElroy MJ, Shaner AC, Crawford TO, Thompson GH, Kadakia RV, Akbarnia BA et al (2011) Growing rods for scoliosis in spinal muscular atrophy: structural effects, complications, and hospital stays. Spine 36(16):1305\u20131311","journal-title":"Spine"},{"issue":"7","key":"302_CR22","doi-asserted-by":"publisher","first-page":"625","DOI":"10.1056\/NEJMoa1710504","volume":"378","author":"E Mercuri","year":"2018","unstructured":"Mercuri E, Darras BT, Chiriboga CA, Day JW, Campbell C, Connolly AM et al (2018) Nusinersen versus Sham control in later-onset spinal muscular atrophy. N Engl J Med 378(7):625\u2013635","journal-title":"N Engl J Med"},{"issue":"4","key":"302_CR23","doi-asserted-by":"publisher","first-page":"501","DOI":"10.1111\/j.1469-8749.1989.tb04029.x","volume":"31","author":"L Merlini","year":"1989","unstructured":"Merlini L, Granata C, Bonfiglioli S, Marini ML, Cervellati S, Savini R (1989) Scoliosis in spinal muscular atrophy: natural history and management. Dev Med Child Neurol 31(4):501\u2013508","journal-title":"Dev Med Child Neurol"},{"issue":"3","key":"302_CR24","doi-asserted-by":"publisher","first-page":"384","DOI":"10.1007\/s00586-009-1198-z","volume":"19","author":"HN Modi","year":"2010","unstructured":"Modi HN, Suh S-W, Hong J-Y, Cho J-W, Park J-H, Yang J-H (2010) Treatment and complications in flaccid neuromuscular scoliosis (Duchenne muscular dystrophy and spinal muscular atrophy) with posterior-only pedicle screw instrumentation. Eur Spine J 19(3):384\u2013393","journal-title":"Eur Spine J"},{"issue":"5","key":"302_CR25","doi-asserted-by":"publisher","first-page":"336","DOI":"10.1016\/j.ijsu.2010.02.007","volume":"8","author":"D Moher","year":"2010","unstructured":"Moher D, Liberati A, Tetzlaff J, Altman DG, PRISMA Group (2010) Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. Int J Surg. 8(5):336\u2013341","journal-title":"Int J Surg."},{"key":"302_CR26","first-page":"44","volume":"207","author":"JO Piasecki","year":"1986","unstructured":"Piasecki JO, Mahinpour S, Levine DB (1986) Long-term follow-up of spinal fusion in spinal muscular atrophy. ClinOrthopRelat Res 207:44\u201354","journal-title":"ClinOrthopRelat Res"},{"key":"302_CR27","doi-asserted-by":"crossref","unstructured":"Ramirez N, Villarin S, Ritchie R, Thompson KJ (2015) Thoracic insufficiency syndrome: an overview. Razavi Int J Med. 3(4). Available from: http:\/\/razavijournal.neoscriber.org\/en\/articles\/57435.html. Cited 12 Oct 2020","DOI":"10.17795\/rijm33030"},{"issue":"5","key":"302_CR28","doi-asserted-by":"publisher","first-page":"268","DOI":"10.1007\/BF00301032","volume":"4","author":"D Robinson","year":"1995","unstructured":"Robinson D, Galasko CS, Delaney C, Williamson JB, Barrie JL (1995) Scoliosis and lung function in spinal muscular atrophy. Eur Spine J 4(5):268\u2013273","journal-title":"Eur Spine J"},{"issue":"1","key":"302_CR29","first-page":"112","volume":"55","author":"S Rudnik-Sch\u00f6neborn","year":"1994","unstructured":"Rudnik-Sch\u00f6neborn S, Wirth B, Zerres K (1994) Evidence of autosomal dominant mutations in childhood-onset proximal spinal muscular atrophy. Am J Hum Genet 55(1):112\u2013119","journal-title":"Am J Hum Genet"},{"issue":"6","key":"302_CR30","doi-asserted-by":"publisher","first-page":"459","DOI":"10.1016\/j.jspd.2016.09.032","volume":"4","author":"MG Vitale","year":"2016","unstructured":"Vitale MG, Matsumoto H, Mueller JD, Cahill PJ, Sturm PF, Roye DP et al (2016) Paper #27 improvement of pulmonary function measured by patient-reported outcomes in patients with spinal muscular atrophy after VEPTR surgery. Spine Deform 4(6):459","journal-title":"Spine Deform"},{"issue":"8","key":"302_CR31","doi-asserted-by":"publisher","first-page":"1027","DOI":"10.1177\/0883073807305788","volume":"22","author":"CH Wang","year":"2007","unstructured":"Wang CH, Finkel RS, Bertini ES, Schroth M, Simonds A, Wong B et al (2007) Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol 22(8):1027\u20131049","journal-title":"J Child Neurol"},{"issue":"5","key":"302_CR32","doi-asserted-by":"publisher","first-page":"1128","DOI":"10.2106\/JBJS.F.01380","volume":"89","author":"JG Wright","year":"2007","unstructured":"Wright JG (2007) A practical guide to assigning levels of evidence. J Bone JtSurg Am 89(5):1128\u20131130","journal-title":"J Bone JtSurg Am"},{"issue":"9","key":"302_CR33","doi-asserted-by":"publisher","first-page":"1909","DOI":"10.2106\/00004623-200509000-00001","volume":"87","author":"JG Wright","year":"2005","unstructured":"Wright JG, Einhorn TA, Heckman JD (2005) Grades of recommendation. J Bone JtSurg Am 87(9):1909\u20131910","journal-title":"J Bone JtSurg Am"},{"issue":"15","key":"302_CR34","doi-asserted-by":"publisher","first-page":"1196","DOI":"10.1097\/BRS.0000000000000383","volume":"39","author":"WW Yoon","year":"2014","unstructured":"Yoon WW, Sedra F, Shah S, Wallis C, Muntoni F, Noordeen H (2014) Improvement of pulmonary function in children with early-onset scoliosis using magnetic growth rods. Spine (Phila Pa 1976). 39(15):1196\u20131202","journal-title":"Spine (Phila Pa 1976)."}],"container-title":["Spine Deformity"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s43390-021-00302-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s43390-021-00302-w\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s43390-021-00302-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,7,9]],"date-time":"2021-07-09T12:11:52Z","timestamp":1625832712000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s43390-021-00302-w"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,3,8]]},"references-count":34,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2021,7]]}},"alternative-id":["302"],"URL":"https:\/\/doi.org\/10.1007\/s43390-021-00302-w","relation":{},"ISSN":["2212-134X","2212-1358"],"issn-type":[{"value":"2212-134X","type":"print"},{"value":"2212-1358","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,3,8]]},"assertion":[{"value":"15 November 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"30 January 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 March 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"24 May 2021","order":4,"name":"change_date","label":"Change Date","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"Update","order":5,"name":"change_type","label":"Change Type","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The Open Access funding note has been added.","order":6,"name":"change_details","label":"Change Details","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with ethical standards"}},{"value":"Some authors do have a conflict of interest and all submitted in details in ICMJE form for disclosure of potential conflicts of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"Not required as it is a systematic review.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"Not required as it is a systematic review.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}}]}}