Friday, March 23, 2007

Dyschondroplasia Syndrome

Dyschondroplasia Syndrome (Ollier Syndrome; Enchondromatosis)

General:
Chondrodysplasia in which ossification in the epiphyseal region is delayed or absent, with resulting continuation of excessive hypertrophic cartilage formation; dyschondroplasia associated with hemangiomas is referred to as Maffucci syndrome (see Maffucci Syndrome).

Ocular: Narrowing of the optic foramen and supraorbital fissure; ophthalmoplegia; optic atrophy, retinal pigmentation.

Clinical: Joint deformities with functional disturbances; coxa vara or valga; scoliosis; facial asymmetry; unilateral bone involvement with resulting shortening of the extremity; intracranial gliomas; intracavernous chondrosarcoma; clival chondroma.

Brazier DJ, et al. Intracavernous chondrosarcoma associated with Ollier's disease. Br J Ophthalmol 1993; 77: 599-600.

Chang S, Prados MD. Identical twins with Ollier's disease and intracranial gliomas: case report. Neurosurgery 1994; 34:903-906.

Duke-Elder S, ed. System of Ophthalmology, vol. III, part 2. St. Louis: CV Mosby, 1963:1068.

Geeraets WJ. Ocular Syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976.

Ghogawala Z, et al. Clival chondroma in a child with Ollier's disease. Case report. Pediatr Neurosurg 1991; 17:53-56.

Ollier M. Sur une Nouvelle Affection: la Dyschondroplasie. Rev Chir (Paris) 1900; 21:396.

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