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PubMed · 8941450

Joint hypermobility syndromes.

M L Raff·P H Byers
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Abstract

Inherited connective tissue disorders, among them Ehlers-Danlos syndrome, osteogenesis imperfecta, Marfan syndrome, and Larsen syndrome, are characterized by generalized joint hypermobility. Others, such as Morquio syndrome or achondroplasia, have hypermobility in a more limited distribution. Recent clinical and molecular genetic studies have defined the range of clinical variation and the underlying molecular defects in several disorders. These studies provide hope that more common varieties of joint hypermobility can be understood and that effective therapies are within reach.

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Cervical VertebraeDiagnosis, DifferentialEhlers-Danlos SyndromeHumansJoint InstabilityMarfan SyndromeOsteogenesis ImperfectaSpinal Cord DiseasesSyndromeJournal ArticleReview

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M L Raff, P H Byers. 1996. Joint hypermobility syndromes.. https://doi.org/10.1097/00002281-199609000-00012

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PubMed2007

Surgical treatment of cervical kyphosis in Larsen syndrome: report of 3 cases and review of the literature.

STUDY DESIGN: A retrospective case series. OBJECTIVE: To review the surgical results for midcervical kyphosis in 3 cases with Larsen syndrome, and to discuss the choice of surgical treatments. SUMMARY OF BACKGROUND DATA: Cervical kyphosis is the most hazardous and serious manifestation of Larsen syndrome due to the risk of life-threatening paralysis, and thus usually requires surgical treatment. However, little information has been reported concerning surgical treatments for this challenging condition. METHODS: Three patients with Larsen syndrome were surgically treated for midcervical kyphosis at our institution. RESULTS: An infant with mild cervical kyphosis was successfully treated with posterior arthrodesis using a halo immobilization, and anterior vertebral growth with a mature posterior fusion mass resulted in spontaneous correction of the kyphosis. In the remaining 2 infants with myelopathic symptoms due to severe and structural kyphosis, anterior decompression and fusion via a lateral approach followed by posterior fusion with segmental spinal instrumentation and halo immobilization resulted in improved neurologic symptoms and solid fusion. CONCLUSIONS: Posterior spinal fusion is only indicated for patients with mild and flexible cervical kyphosis, and anterior decompression and circumferential arthrodesis is required for patients with severe kyphotic deformity, who usually develop myelopathic symptoms. Anterior surgery for such a small patient with severe kyphosis involves much higher risk of spinal cord injury during decompression maneuvers and difficulty in stabilization of the reconstructed cervical spine. Therefore, all patients with Larsen syndrome should be screened with radiographs at the first visit to detect cervical kyphosis early so that posterior alone fusion is possible.

Cervical Vertebrae↗