Objective. To retrospectively assess, with a sufficiently long followup (mean 11.6 years; median 9 years), the long-term outcome of chronic recurrent multifocal osteitis (CRMO), a multifocal, inflammatory bone disease.Methods. Patients included were 8 children/ adolescents and 7 adults with no family history of rheumatic disease who had been diagnosed as having CRMO between 1979 and 1995. Ten patients had undergone at least 1 bone biopsy of the lesions, with histologic examination and multiple cultures. In 1996, in addition to an in-depth interview, 12 patients underwent an extensive physical examination, laboratory evaluation, HLA-A, B, C, and DR typing, bone radiography and scintigraphy, and computed tomography scan of the sternoclavicular and sacroiliac joints.Results. Remission was observed in 3 patients. The other 12 patients developed various associations of vertebral (n ؍ 10), sacroiliac (n ؍ 6), anterior thoracic (n ؍ 7), peripheral articular (n ؍ 2), enthesopathic (n ؍ 4), or dermatologic (palmoplantar pustulosis in 3 cases and psoriasis in 2) involvements. Spine involvement was the most common and occurred the earliest (median time to appearance after the onset of osteitis 5.63 years). Clinical sacroiliitis was always unilateral. No patients carried the HLA-B27 haplotype. CRMO responded well to nonsteroidal antiinflammatory drugs. Twelve patients met the European Spondylarthropathy Study Group criteria for spondylarthopathy.
Current knowledge of the causes and risk factors of Legg-Calvé-Perthesdisease (LCPD) does not allow effective preventive strategies. The outcome in adulthood is usually good. Hip osteoarthritis rarely develops before 50 years of age. The risk of osteoarthrosis depends chiefly on the final degree of joint incongruence. Age at onset and the lateral pillar classification are the two main outcome predictors and serve to guide the surgical indications based on the studies by Herring's group. Non-operative treatment is not effective. In contrast, femoral varus osteotomy and Salter's innominate osteotomy provide good outcomes. In severe forms, however, combining these two techniques or performing a triple pelvic osteotomy seem preferable. Surgery is now performed considerably less often than in the past, as it is effective only in patients with lateral pillar group B or B/C disease with onset after eight years of age. In other situations, therapeutic abstention is recommended.
Amniotic band syndrome or amniotic disruption complex is a well-known congenital limb abnormality, which occurs in 1 in 1200 to 1 in 15,000 live births. In cases of an isolated band constriction, it has been speculated that the bands lead to decreased blood flow in the constricted limb and subsequent natural amputation. Fetal surgery could be considered in these situations in order to release the constriction band in threatened limb amputation. We present a case of a tight constriction ring secondary to amniotic band syndrome with a favourable outcome, despite the failure of an attempted surgical procedure.
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