What is the most dangerous complication of SED congenita?

C1-C2 instability from odontoid hypoplasia can cause myelopathy or sudden death; MRI of cervical spine in flexion/extension is essential before any anesthetic; posterior C1-C2 fusion if unstable.

At what scoliosis degree does SED require surgical correction?

Curves >45 degrees or rapidly progressive curves in growing children, particularly thoracic curves causing restrictive lung disease, warrant surgical correction; cast or bracing for 25-40 degree curves.

How is final height predicted in SED?

SED congenita has mean adult height 84-128 cm; SED tarda has less severe short stature (mean 128-157 cm); SED-specific growth charts are used as standard centile charts are not applicable.

What vision problems occur in SED?

Myopia is nearly universal; retinal detachment risk is elevated and requires annual ophthalmological surveillance; cataract and glaucoma also occur with higher frequency than the general population.

What is the difference between SED congenita and SED tarda?

SED congenita (AD, COL2A1) presents at birth with short trunk dwarfism; SED tarda (X-linked or AR) presents in late childhood with short stature and premature osteoarthritis; both affect primarily spine and large joints.

Spondyloepiphyseal Dysplasia Radiological Severity Score

Evaluates skeletal dysplasia severity in SED using thoracic kyphoscoliosis degree, odontoid hypoplasia risk, hip dysplasia severity, and final height prediction using SED-specific growth charts.

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Frequently Asked Questions

What is the most dangerous complication of SED congenita?

At what scoliosis degree does SED require surgical correction?

How is final height predicted in SED?

What vision problems occur in SED?

What is the difference between SED congenita and SED tarda?