Spinal lordosis with marked opisthotonus secondary to dystonia musculorum deformans: case report with surgical management.

STUDY

DESIGN

A case report of severe spinal lordosis with marked opisthotonus and retrocollis secondary to dystonia musculorum deformans is presented.

OBJECTIVE

To describe a case of dystonia musculorum deformans with progressive spinal lordosis and its surgical treatment.

SUMMARY OF BACKGROUND DATA: Four patients with correction of coronal spinal deformity associated with dystonia musculorum deformans have been reported in the literature. No reports of sagittal spinal deformity treated with surgical instrumentation and fusion were found.

METHODS

A retrospective chart and radiographic review of a single case was conducted.

RESULTS

Orthotic management and pharmacologic therapy with botulinum toxin injections were unsuccessful in controlling the deformity. Severe spinal lordosis (170 degrees ) from occiput to sacrum was corrected surgically, allowing an upright posture.

CONCLUSION

Dystonia musculorum deformans is a rare condition resulting in coronal or sagittal plane deformities. When other treatment methods are unsuccessful, surgical instrumentation and arthrodesis may correct the deformity and improve function.