The Therapeutic Approach to Syndromic Scoliosis: A Case Report
DOI:
https://doi.org/10.25759/spmfr.469Keywords:
Scoliosis/rehabilitation, Williams SyndromeAbstract
Structural scoliosis is a deformity of the spine in all anatomical planes. The diagnosis is made when the lateral curvature in the frontal plane (Cobb angle) is equal or greater to 10o. Severe scoliosis can significantly impair the quality of life of these patients, leading to functional impairment, respiratory dysfunction and aesthetic changes with psychosocial impact. The main goal of the treatment is therefore to minimize curve progression. Williams syndrome
is a rare genetic disorder that causes changes in the normal production of elastin - the most common manifestations are cardiovascular and neurodevelopmental; these patients also are more prone to develop scoliosis. The authors present a case of a pre-pubescent boy with syndromic scoliosis due to Williams syndrome, whilst presenting a narrative review of the therapeutic approaches available.
References
Negrini S, Donzelli S, Aulisa AG, Czaprowski D, Schreiber S, de Mauroy JC, et al. 2016 SOSORT guidelines: orthopaedic and rehabilitation treatment of idiopathic scoliosis during growth. Scoliosis Spinal Disord. 2018;13:3. doi: 10.1186/s13013-017-0145-8.
Knott P, Pappo E, Cameron M, Demauroy J, Rivard C, Kotwicki T, et al. SOSORT 2012 consensus paper: reducing x-ray exposure in pediatric patients with scoliosis. Scoliosis. 2014;9:4. doi: 10.1186/1748-7161-9-4.
Parr A, Askin G. Paediatric scoliosis: Update on assessment and treatment. Aust J Gen Pract. 2020;49:832-7. doi: 10.31128/AJGP-06-20-5477.
Zhang YB, Zhang JG. Treatment of early-onset scoliosis: techniques, indications, and complications. Chin Med J. 2020;133:351-7. doi: 10.1097/ CM9.0000000000000614.
Kozel BA, Barak B, Kim CA, Mervis CB, Osborne LR, Porter M, et al. Williams syndrome. Nat Rev Dis Primers. 2021;7:42.
Damasceno ML, Cristante AF, Marcon RM, Barros Filho TE. Prevalence of scoliosis in Williams-Beuren syndrome patients treated at a regional reference center. Clinics. 2014;69:452-6. doi: 10.6061/clinics/2014(07)02.
Ballhause TM, Moritz M, Hättich A, Stücker R, Mladenov K. Serial casting in early onset scoliosis: syndromic scoliosis is no contraindication. BMC Musculoskelet Disord. 2019;20:554. doi: 10.1186/s12891-019-2938-9.
Ruiz G, Torres-Lugo NJ, Marrero-Ortiz P, Guzmán H, Olivella G, Ramírez N. Early-onset scoliosis: a narrative review. EFORT Open Rev. 2022;7:599-610. doi: 10.1530/EOR-22-0040.
Hasler CC. Early-onset scoliosis: contemporary decision-making and treatment options. J Pediatr Orthop. 2018;38:S13-20.
Osebold WR, King HA. Kyphoscoliosis in Williams syndrome. Spine. 1994;19:367-71.
Mahajan R, Kishan S, Mallepally AR, Shafer C, Marathe N, Chhabra HS. Evolution of casting techniques in early-onset and congenital scoliosis. J Clin Orthop Trauma. 2020;11:810-5. doi: 10.1016/j.jcot.2020.06.034.
Berdishevsky H, Lebel VA, Bettany-Saltikov J, Rigo M, Lebel A, Hennes A, et al. Physiotherapy scoliosis-specific exercises - a comprehensive review of seven major schools. Scoliosis Spinal Disord. 2016;11:20. doi: 10.1186/ s13013-016-0076-9.
Schlösser TPC, Kruyt MC, Tsirikos AI. Surgical management of early-onset scoliosis: indications and currently available techniques. Orthop Trauma. 2021;35:336-46.
Chung AS, Renfree S, Lockwood DB, Karlen J, Belthur M. Syndromic scoliosis: national trends in surgical management and inpatient hospital outcomes: A 12-Year Analysis. Spine. 2019;44:1564-70. doi: 10.1097/BRS.00000000 00003134.
Downloads
Published
How to Cite
Issue
Section
License
Copyright statement
Authors must also submit a copyright statement (as seen below) on article submission.
To the Editor-in-chief of the SPMFR Journal:
The below signed author(s) hereby state that the article
________________________________________ (ref. MFR_________) is
an original unpublished work and all facts stated are a product of the author(s) investigation. This article does not violate any copyright laws or privacy statements. The author(s) also hereby confirm that there is no conflict of interest's issues in this article.
By submitting this article the author(s) agree that after publication all copyrights belong to the SPMFR Journal.
Signed by all authors
Date:
Names (capital letters):
Signatures:
The SPMFR Journal’s contents are follow a Creative Commons licence. After publication the authors can hand out the articles as long as the SPMFR Journal is credited.
