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.

[Anesthetic management of a patient with SAPHO syndrome: a case report].

We report a case of SAPHO syndrome accompanying progressive osteoarthritis. In this 43-year-old woman, difficult intubation was expected due to unilateral hyperostosis of the mandible and spinal degeneration. As far as we know, anesthetic management of this disease has not been reported. Therefore we made an anesthetic plan based on spinal degenerative disease which was considered to have similar problems of intubation and the case was managed without problems. As this syndrome is a progressive disorder, careful perioperative management is required each time.

Intracranial manifestations in SAPHO syndrome: the first case report in literature.

A 41-year-old woman with SAPHO syndrome presented with numbness of her left arm followed by a generalized seizure. Computed tomography and magnetic resonance imaging of the brain revealed a small ring enhancing lesion in the right parietal lobe with adjacent meningeal thickening and enhancement. Surgical removal and histopathology showed evidence of severe chronic sterile inflammation and no malignant cell. Symptoms recurred and a lesion were again detected on radiological follow-up, but improvement occurred following treatment with antibiotics and biphosphonate, achieving a low inflammatory activity, reduction in CNS lesions and relative clinical well-being. This is a first report in literature about the central nervous system involvement in a patient with SAPHO syndrome.

SAPHO syndrome with affection of the mandible: diagnosis, treatment, and review of literature.

The synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare syndrome of unknown etiology. Involvement of the mandible is found in almost 10% of cases. In the literature, conservative treatment is recommended most often, because decortication and partial resection are found to be ineffective and of temporary profit. We report a case of SAPHO syndrome in a 44-year-old women with unilateral hyperostosis of the mandible and massive painful swelling of the surrounding soft tissues. Owing to facial disfiguration and pain, resection of the affected bone followed by immediate reconstruction with a microvascular iliac crest flap were performed. The aim of this paper was to present the necessity of surgical intervention in SAPHO syndrome of the mandible in cases of esthetic and functional limitation.

[Modern specificity of surgical infection of bones and joints in children].

A comparative study of frequency, structure and specific features of the clinical course was fulfilled in 2456 children with surgical infection of bones and joints (SIBJ) in the period from 1970 to 1985 and 1986 to 2006. The work used clinical, instrumental, radiation, laboratory, microbiological, immunological and statistical methods of investigations. Specific features of SIBJ in children at the present time are: 1) pathomorphosis of hematogenic osteomyelitis; 2) the appearance of previously not noted new forms of SIBJ--fetal osteomyelitis and arthritis, chronic recurring multifocal osteomyelitis, syndrome SAPHO and increasing frequency of previously rare BCG-osteomyelitis, exogenous osteomyelitis, sacroiliitis and inervertebral disciitis; 3) widening the spectrum of SIBJ pathogens with an increasing significance of involutionary forms of bacteria, elevation of their resistence to antibiotics and antiseptics, increase of the factors promoting the development of immunodeficient states in childhood.

[Pediatric osteomyelitis]. / Die kindliche Osteomyelitis.

Bacterial osteomyelitis in children must be diagnosed quickly and requires immediate and adequate antibiotic treatment. Surgical interventions may be necessary. Infants as well as immunodeficient patients suffer more often from hematogenic bone infections than immunocompetent patients. According to recent findings, autoinflammatory nonbacterial osteitis is more probable in immunocompetent patients in good general condition and should always be considered as a differential diagnosis. Diagnostic and therapeutic approaches are presented when childhood osteomyelitis is suspected.

SAPHO syndrome - rare case of joint damage treated by knee synovectomy and total hip joint replacement.

This report describes the case of a young male who had been followed-up between the ages of 14-21 years at different health facilities for symptoms initially considered to be caused by tumor, then by chronic osteomyelitis or ankylosing spondylitis and finally diagnosed as the SAPHO syndrome. Musculoskeletal symptoms of the SAPHO syndrome include focal, probably aseptic chronic osteomyelitis, synovitis and formation of hyperostoses. Therapy of the SAPHO syndrome is predominantly conservative. However, in this case we had to employ arthroscopic synovectomy first, for severe synovitis resistant to any conservative therapy approach. Later, left total hip replacement has been performed for advanced hip joint damage accompanied by intense pain and significant range of motion reduction. At the present time, the patient is 51/2 years after synovectomy of the knee and 5 years after the hip joint replacement. The knee joint is without effusion or functional limitations, with intermittent pains only. The hip prosthesis in the risk area is fully integrated without signs of component loosening, with very good functional outcome. While synovectomy can be obviously fully recommended in cases like this, the total joint replacement should be considered unique and indicated only rarely after careful consideration of all circumstances.We consider this approach absolutely exceptional also because we have not found reference to similar case in any national or international professional literature available.