Severe, rigid cervical kyphotic deformity associated with SAPHO syndrome successfully treated with three-stage correction surgery combined with C7 vertebral column resection: a technical case report.

STUDY

DESIGN

Case report.

OBJECTIVES

To describe the first reported use of corrective surgery combined with C7 vertebral column resection (VCR) to treat an extremely rare case of severe, rigid cervical kyphotic deformity associated with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome.

BACKGROUND DATA: Spinal lesions reportedly occur in 32-45% in SAPHO syndrome. However, bone and joint lesions are usually repaired such that severe joint destruction is rare. Therefore, there have been few reported cases of surgical treatment for spinal lesions.

METHODS

A 22-year-old woman had been diagnosed with cervical kyphotic deformity associated with SAPHO syndrome. She had difficulty looking upward. On radiography, the C4-C7 vertebral bodies were fused and exhibited severe rigid kyphotic deformity. Right convex scoliosis with a Cobb angle of 22° was apparent at C5-T2, and the C2-C7 angle of kyphosis was 75°. Corrective three-stage surgery was carried out from the anterior, posterior, and anterior, with C7 VCR.

RESULTS

The C2-T1 angle improved to 21° and the patient was capable of looking up. At present, 2 years postoperatively, complete bony fusion has been achieved. Her cervical spine function and quality of life were markedly improved.

CONCLUSION

Only a very limited number of patients have a deformity sufficiently severe that VCR of cervical vertebra is required, but as this is the technique that provides the greatest multiplanar alignment correction, its choice in the present case was appropriate.

LEVEL OF EVIDENCE: IV.