Subungual enchondrome Q78.4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Dyschondroplasia; Enchondromatosis; Enchondromatosis Ollier; Hemichondrodystrophy; Ollier disease; Ollier M.; Ollier's disease; Ollier's syndrome; Osteochondromatosis Syndromes; subungual enchondrome

History
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Ollier, 1900

Definition
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Skeletal dysplasia is characterized by multiple enchondromas, which occur predominantly on one side of the body and are associated with impaired longitudinal growth. Some authors regard the disease as part of the Maffucci syndrome.

Occurrence/Epidemiology
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Very rare. Incidence: 1/1 million inhabitants/year.

Etiopathogenesis
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Not clear.

Clinical features
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Extremities shortened on one side. Often multiple enchondromas and hemangiomas in the affected bones.

Progression/forecast
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Occasional spontaneous fractures.

Literature
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  1. Al-Ismail K et al (2002) Ollier's disease in association with adjacent fibromatosis. Skeletal radiol 31: 479-483
  2. Auyeung J et al (2003) Maffucci lymphangioma syndrome: an unusual variant of Ollier's disease, a case report and a review of the literature. J Pediatric Orthopaedic Surgery B 12: 147-150
  3. Nguyen BD (2004) Ollier disease with synchronous multicentric chondrosarcomas: scintigraphic and radiologic demonstration. Clin Nucl Med 29: 45-47
  4. Ollier M (1900) De la dyschondrosplasie. Bull Soc Chir Lyon 3: 22-27
  5. Schwarz W et al (2002) Multiple enchondromatosis: Ollier's disease. Trauma surgeon 105: 1139-1142

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Last updated on: 29.10.2020