Algodystrophy in conjunction with van der Hoeve's syndrome.

Clin Exp Rheumatol

Department of Systemic and Local Treatment of Chronic Inflammatory Rheumatism, G. Pini Orthopaedics Institute, Milan, Italy.

Published: November 1995

Van der Hoeve's syndrome is the all-round variant of Lobstein's disease (late osteogenesis imperfecta) and is characterized by the concurrent presence of osteoporosis, blue sclerae, and deafness. The literature has reported sporadic cases of a combination of algodystrophic syndrome and Lobstein's disease. Our case represents a classic example: a 50-year-old woman with blue sclerae since birth, bilateral deafness, major dental alterations, vitreous osteoporosis and a history of pathological fractures who, two years from the onset of menopause, complained of pain involving the right ankle. After admission to our Department, a diagnosis of algodystrophy combined with van der Hoeve's syndrome was made on the basis of her clinical history, objective signs and x-ray results. Treatment consisted of i.v. clodronate disodium at a dose of 300 mg daily in 250 ml saline solution for 7 consecutive days. At the end of the treatment cycle the patient reported an improvement in her symptoms with nearly total regression of right ankle pain, while swelling disappeared within a few weeks.

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