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Article: Natural history of spinal deformity in a patient with Ehlers-Danlos syndrome: case report with 20 year follow-up

TitleNatural history of spinal deformity in a patient with Ehlers-Danlos syndrome: case report with 20 year follow-up
Authors
Issue Date2011
PublisherElsevier BV. The Journal's web site is located at http://www.elsevier.com/locate/spinee
Citation
The Spine Journal, 2011, v. 11 n. 7, p. e1-e4 How to Cite?
AbstractBACKGROUND CONTEXT: Scoliosis can present in patients with Ehlers-Danlos syndrome (EDS) and can be surgically treated despite possible complications. The long-term natural history of the progression of spinal deformity, particularly scoliosis, and associated complications in EDS patients remains relatively unknown. PURPOSE: To assess the progression of spinal deformity and associated complications in a patient with EDS, who was not surgically treated for her spine condition and was followed up for 20 years. STUDY DESIGN: A case report. METHODS: A 9-year-old female with EDS presented to the orthopedic clinic with scoliosis and mild thoracic hyperkyphosis. The patient was followed up for a 20-year period. Progression of her spinal deformity and associated complications were noted. RESULTS: The patient's curve progressed rapidly in the initial follow-up period an 83 degrees worsening from the age of 9 to 10 years. Because she refused surgical intervention, her spinal deformity continued to progress. As such, by the age of 29 years, she presented with 115 degrees from T9 to L4 with severe hyperkyphosis. The patient eventually developed respiratory compromise and diminished functional capacity. CONCLUSIONS: In patients with EDS, scoliosis may progress rapidly and unrelentingly, and if not treated early may lead to respiratory compromise. Close follow-up of such patients is imperative, and appropriate management should be sought to prevent potentially fatal morbidities.
DescriptionCase report
Persistent Identifierhttp://hdl.handle.net/10722/170178
ISSN
2015 Impact Factor: 2.66
2015 SCImago Journal Rankings: 1.153
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorNatarajan, Den_US
dc.contributor.authorSamartzis, Den_US
dc.contributor.authorWong, YWen_US
dc.contributor.authorLuk, KDKen_US
dc.contributor.authorCheung, KMCen_US
dc.date.accessioned2012-10-30T06:05:52Z-
dc.date.available2012-10-30T06:05:52Z-
dc.date.issued2011en_US
dc.identifier.citationThe Spine Journal, 2011, v. 11 n. 7, p. e1-e4en_US
dc.identifier.issn1529-9430en_US
dc.identifier.urihttp://hdl.handle.net/10722/170178-
dc.descriptionCase report-
dc.description.abstractBACKGROUND CONTEXT: Scoliosis can present in patients with Ehlers-Danlos syndrome (EDS) and can be surgically treated despite possible complications. The long-term natural history of the progression of spinal deformity, particularly scoliosis, and associated complications in EDS patients remains relatively unknown. PURPOSE: To assess the progression of spinal deformity and associated complications in a patient with EDS, who was not surgically treated for her spine condition and was followed up for 20 years. STUDY DESIGN: A case report. METHODS: A 9-year-old female with EDS presented to the orthopedic clinic with scoliosis and mild thoracic hyperkyphosis. The patient was followed up for a 20-year period. Progression of her spinal deformity and associated complications were noted. RESULTS: The patient's curve progressed rapidly in the initial follow-up period an 83 degrees worsening from the age of 9 to 10 years. Because she refused surgical intervention, her spinal deformity continued to progress. As such, by the age of 29 years, she presented with 115 degrees from T9 to L4 with severe hyperkyphosis. The patient eventually developed respiratory compromise and diminished functional capacity. CONCLUSIONS: In patients with EDS, scoliosis may progress rapidly and unrelentingly, and if not treated early may lead to respiratory compromise. Close follow-up of such patients is imperative, and appropriate management should be sought to prevent potentially fatal morbidities.en_US
dc.languageengen_US
dc.publisherElsevier BV. The Journal's web site is located at http://www.elsevier.com/locate/spineeen_US
dc.relation.ispartofThe Spine Journalen_US
dc.subject.meshDisease Progressionen_US
dc.subject.meshEhlers-Danlos Syndrome - complications - pathology - radiographyen_US
dc.subject.meshFollow-Up Studiesen_US
dc.subject.meshKyphosis - etiology - pathology - radiographyen_US
dc.subject.meshScoliosis - etiology - pathology - radiographyen_US
dc.titleNatural history of spinal deformity in a patient with Ehlers-Danlos syndrome: case report with 20 year follow-upen_US
dc.typeArticleen_US
dc.identifier.emailSamartzis, D: dspine@hku.hken_US
dc.identifier.emailWong, YW: yatwa@hkucc.hku.hken_US
dc.identifier.emailLuk, KDK: hcm21000@hku.hken_US
dc.identifier.emailCheung, KMC: cheungmc@hku.hk-
dc.identifier.authoritySamartzis, D=rp01430en_US
dc.identifier.authorityLuk, KDK=rp00333en_US
dc.identifier.authorityCheung, KMC=rp00387en_US
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.doi10.1016/j.spinee.2011.02.029en_US
dc.identifier.pmid21640659-
dc.identifier.scopuseid_2-s2.0-80051550253en_US
dc.identifier.hkuros207319-
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-80051550253&selection=ref&src=s&origin=recordpageen_US
dc.identifier.volume11en_US
dc.identifier.issue7en_US
dc.identifier.spagee1en_US
dc.identifier.epagee4en_US
dc.identifier.isiWOS:000293592500001-
dc.publisher.placeNetherlandsen_US
dc.identifier.scopusauthoridCheung, KMC=7402406754en_US
dc.identifier.scopusauthoridLuk, KDK=7201921573en_US
dc.identifier.scopusauthoridWong, YW=36247941700en_US
dc.identifier.scopusauthoridSamartzis, D=34572771100en_US
dc.identifier.scopusauthoridNatarajan, D=35334874800en_US
dc.identifier.citeulike9496794-

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