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Algodystrophy in conjunction with van der Hoeve's syndrome

G Chérié-Lignière1, U Tamborini, P Grosso

  • 1Department of Systemic and Local Treatment of Chronic Inflammatory Rheumatism, G. Pini Orthopaedics Institute, Milan, Italy.

Clinical and Experimental Rheumatology
|May 1, 1995
PubMed
Summary

Van der Hoeve's syndrome, a variant of Lobstein's disease, involves osteoporosis, blue sclerae, and deafness. This case highlights successful treatment of algodystrophy combined with van der Hoeve's syndrome using clodronate disodium.

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Area of Science:

  • Endocrinology
  • Genetics
  • Rheumatology

Background:

  • Van der Hoeve's syndrome, a variant of Lobstein's disease (late osteogenesis imperfecta), presents with osteoporosis, blue sclerae, and deafness.
  • Literature reports sporadic cases of combined algodystrophic syndrome and Lobstein's disease.
  • This study details a classic example of this rare combination.

Observation:

  • A 50-year-old woman with congenital blue sclerae, bilateral deafness, dental anomalies, osteoporosis, and a history of fractures presented with right ankle pain post-menopause.
  • Clinical history, objective signs, and X-ray results confirmed a diagnosis of algodystrophy combined with van der Hoeve's syndrome.

Findings:

  • Intravenous clodronate disodium (300 mg daily for 7 days) was administered.
  • The patient experienced significant symptom improvement, with near-complete regression of ankle pain.

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  • Swelling resolved within weeks of treatment completion.
  • Implications:

    • Intravenous clodronate disodium shows promise in managing algodystrophy symptoms in patients with van der Hoeve's syndrome.
    • This case underscores the importance of recognizing and treating combined conditions.
    • Further research into bisphosphonate efficacy for complex bone and connective tissue disorders is warranted.