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Article: Atlantoaxial rotatory fixation in the setting of associated congenital malformations: A modified classification system

TitleAtlantoaxial rotatory fixation in the setting of associated congenital malformations: A modified classification system
Authors
KeywordsAtlantoaxial
Cervical
Chiari
Congenital
Craniovertebral
Dislocation
Displacement
Fixation
Iatrogenic
Instability
Klippel-Feil
Spinal cord injury
Spine
Subluxation
Issue Date2010
PublisherLippincott, Williams & Wilkins. The Journal's web site is located at http://www.spinejournal.com
Citation
Spine, 2010, v. 35 n. 4, p. E119-E127 How to Cite?
AbstractSTUDY DESIGN. A case report. OBJECTIVE. To raise awareness of the development of atlantoaxial rotatory fixation (AARF) in the setting of congenital vertebral anomalies/malformations. SUMMARY OF BACKGROUND DATA. Klippel-Feil Syndrome (KFS) is a complex, heterogeneous condition noted as congenital fusion of 2 or more cervical vertebrae with or without spinal or extraspinal manifestations. Although believed to be a rare occurrence in the population, KFS may be underreported. Proper diagnosis of KFS and other congenital conditions affecting the spine is imperative to devise proper management protocols and avoid potential complications resulting from the altered biomechanics associated with such conditions and their abnormal vertebral morphology. Craniovertebral dislocation and AARF may cause severe cervicomedullary and spinal cord compression and could thereby be potentially fatal, especially in patients with KFS who present with congenitally-associated comorbidities. METHODS. A 13-year-old boy with Chiari type I malformation, craniofacial abnormalities, and other irregularities underwent thoracolumbar spine surgery for his scoliosis curve correction at another institution, which immediately following surgery he became a quadriparetic. The initial preoperative assessment of his cervical spine was limited and the associated KFS was initially undiagnosed. At 14 years of age, he presented to our clinic with an ASIA-C spinal cord injury. Plain radiographs, normal and 3-dimensional reformatted computed tomographs (CT), and magnetic resonance imaging (MRI) noted assimilation of the patient's occiput to the atlas (occipitalization) with congenital fusion of C2-C3, indicative of KFS, and the presence of anterior craniovertebral dislocation with a Fielding and Hawkins type II AARF. Closed reduction of the craniovertebral dislocation was noted, but his atlantoaxial rotatory subluxation was nonresponsive and fixed (AARF). As such, at the age of 14, the patient underwent posterior instrumentation and fusion from the occiput to C4 to maintain reduction of thecraniovertebral dislocation and reduce his AARF. RESULTS. At 9 months postoperative follow-up of his craniovertebral surgery, the instrumentation remained intact, reduction of the atlantoaxial rotatory subluxation was maintained, and posterior bone fusion was noted. Neurologically, he remained an ASIA-C without any substantial return of function. CONCLUSION. This report raises awareness for the need of a thorough evaluation of the cervical spine to determine patients at high risk for craniovertebral dislocation and atlantoaxial rotatory subluxation, primarily in the context of KFS or other congenital conditions. Three-dimensional CT and MR imaging are ideal radiographic methods to determine the presence and extent of craniovertebral dislocation, AARF, and of abnormal vertebral anatomy/malformations. In addition, the authors propose a modification to the Fielding and Hawkins classification of AARF to include variants and subtypes that account for abnormal anatomy and congenital anomalies/malformations. © 2010 Lippincott Williams & Wilkins, Inc.
Persistent Identifierhttp://hdl.handle.net/10722/92924
ISSN
2015 Impact Factor: 2.439
2015 SCImago Journal Rankings: 1.459
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorSamartzis, Den_HK
dc.contributor.authorShen, FHen_HK
dc.contributor.authorHerman, Jen_HK
dc.contributor.authorMardjetko, SMen_HK
dc.date.accessioned2010-09-22T05:03:55Z-
dc.date.available2010-09-22T05:03:55Z-
dc.date.issued2010en_HK
dc.identifier.citationSpine, 2010, v. 35 n. 4, p. E119-E127en_HK
dc.identifier.issn0362-2436en_HK
dc.identifier.urihttp://hdl.handle.net/10722/92924-
dc.description.abstractSTUDY DESIGN. A case report. OBJECTIVE. To raise awareness of the development of atlantoaxial rotatory fixation (AARF) in the setting of congenital vertebral anomalies/malformations. SUMMARY OF BACKGROUND DATA. Klippel-Feil Syndrome (KFS) is a complex, heterogeneous condition noted as congenital fusion of 2 or more cervical vertebrae with or without spinal or extraspinal manifestations. Although believed to be a rare occurrence in the population, KFS may be underreported. Proper diagnosis of KFS and other congenital conditions affecting the spine is imperative to devise proper management protocols and avoid potential complications resulting from the altered biomechanics associated with such conditions and their abnormal vertebral morphology. Craniovertebral dislocation and AARF may cause severe cervicomedullary and spinal cord compression and could thereby be potentially fatal, especially in patients with KFS who present with congenitally-associated comorbidities. METHODS. A 13-year-old boy with Chiari type I malformation, craniofacial abnormalities, and other irregularities underwent thoracolumbar spine surgery for his scoliosis curve correction at another institution, which immediately following surgery he became a quadriparetic. The initial preoperative assessment of his cervical spine was limited and the associated KFS was initially undiagnosed. At 14 years of age, he presented to our clinic with an ASIA-C spinal cord injury. Plain radiographs, normal and 3-dimensional reformatted computed tomographs (CT), and magnetic resonance imaging (MRI) noted assimilation of the patient's occiput to the atlas (occipitalization) with congenital fusion of C2-C3, indicative of KFS, and the presence of anterior craniovertebral dislocation with a Fielding and Hawkins type II AARF. Closed reduction of the craniovertebral dislocation was noted, but his atlantoaxial rotatory subluxation was nonresponsive and fixed (AARF). As such, at the age of 14, the patient underwent posterior instrumentation and fusion from the occiput to C4 to maintain reduction of thecraniovertebral dislocation and reduce his AARF. RESULTS. At 9 months postoperative follow-up of his craniovertebral surgery, the instrumentation remained intact, reduction of the atlantoaxial rotatory subluxation was maintained, and posterior bone fusion was noted. Neurologically, he remained an ASIA-C without any substantial return of function. CONCLUSION. This report raises awareness for the need of a thorough evaluation of the cervical spine to determine patients at high risk for craniovertebral dislocation and atlantoaxial rotatory subluxation, primarily in the context of KFS or other congenital conditions. Three-dimensional CT and MR imaging are ideal radiographic methods to determine the presence and extent of craniovertebral dislocation, AARF, and of abnormal vertebral anatomy/malformations. In addition, the authors propose a modification to the Fielding and Hawkins classification of AARF to include variants and subtypes that account for abnormal anatomy and congenital anomalies/malformations. © 2010 Lippincott Williams & Wilkins, Inc.en_HK
dc.languageengen_HK
dc.publisherLippincott, Williams & Wilkins. The Journal's web site is located at http://www.spinejournal.comen_HK
dc.relation.ispartofSpineen_HK
dc.subjectAtlantoaxialen_HK
dc.subjectCervicalen_HK
dc.subjectChiarien_HK
dc.subjectCongenitalen_HK
dc.subjectCraniovertebralen_HK
dc.subjectDislocationen_HK
dc.subjectDisplacementen_HK
dc.subjectFixationen_HK
dc.subjectIatrogenicen_HK
dc.subjectInstabilityen_HK
dc.subjectKlippel-Feilen_HK
dc.subjectSpinal cord injuryen_HK
dc.subjectSpineen_HK
dc.subjectSubluxationen_HK
dc.titleAtlantoaxial rotatory fixation in the setting of associated congenital malformations: A modified classification systemen_HK
dc.typeArticleen_HK
dc.identifier.emailSamartzis, D:dspine@hku.hken_HK
dc.identifier.authoritySamartzis, D=rp01430en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1097/BRS.0b013e3181c9f957en_HK
dc.identifier.pmid20160615-
dc.identifier.scopuseid_2-s2.0-77249165646en_HK
dc.identifier.hkuros173072-
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-77249165646&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume35en_HK
dc.identifier.issue4en_HK
dc.identifier.spageE119en_HK
dc.identifier.epageE127en_HK
dc.identifier.eissn1528-1159-
dc.identifier.isiWOS:000275323800025-
dc.publisher.placeUnited Statesen_HK
dc.identifier.scopusauthoridSamartzis, D=34572771100en_HK
dc.identifier.scopusauthoridShen, FH=7201583245en_HK
dc.identifier.scopusauthoridHerman, J=7403275959en_HK
dc.identifier.scopusauthoridMardjetko, SM=6602479638en_HK

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