Table 4.

Treatment of Manifestations in Individuals with X-linked Spondyloepiphyseal Dysplasia Tarda

Manifestation/
Concern
TreatmentConsiderations/
Other
Odontoid
hypoplasia
Precautions during intubation/surgery to avoid hyperextensionObtain cervical spinal films prior to any surgical procedure involving general anesthesia to assess for clinically significant odontoid hypoplasia.
Scoliosis/
Kyphoscoliosis
  • Treatment per orthopedic surgeon
  • Spine surgery (correction of scoliosis or kyphosis) may be indicated.
Osteoarthritis
  • Chronic pain management
  • Surgical intervention may incl joint replacement (hip, knee, shoulder).

From: X-Linked Spondyloepiphyseal Dysplasia Tarda

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