enchondromatosis


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Related to enchondromatosis: chondromyxoid fibroma, Maffucci syndrome

en·chon·dro·ma·to·sis

(en-kon'drō-ma-tō'sis), [MIM*166000 *225795]
A rare disorder characterized by hamartomatous proliferation of cartilage in the metaphyses of several bones, most commonly of the hands and feet, causing distorted growth in length and pathologic fractures; chondrosarcoma may develop. When enchondromatosis is associated with hemangiomas in the cutaneous or visceral regions, the condition is called Maffucci syndrome. Most cases are sporadic but a few instances demonstrate autosomal dominant inheritance with reduced penetrance.

en·chon·dro·ma·to·sis

(en'kon-drō-mă-tō'sis)
A rare familial, and probably hamartomatous, proliferation of cartilage in the metaphyses of several bones, most commonly of the hands and feet, causing distorted growth in length or pathologic fractures; chondrosarcoma frequently develops. When combined with hemangiomas in the cutaneous or visceral regions, it is called Maffucci syndrome.
Synonym(s): dyschondroplasia.

Ollier,

Louis Xavier Edouard Léopold, French surgeon, 1830-1900.
Ollier approach
Ollier disease - proliferation of cartilage in the metaphyses of several bones, causing distorted growth in length or pathological fractures. Synonym(s): enchondromatosis
Ollier graft - a thin split-thickness graft, usually in small pieces. Synonym(s): Ollier-Thiersch graft; Thiersch graft
Ollier incision
Ollier layer
Ollier method
Ollier operation
Ollier rake retractor
Ollier raspatory
Ollier retractor
Ollier technique
Ollier theory - a theory of compensatory growth.
Ollier-Thiersch graft - Synonym(s): Ollier graft
References in periodicals archive ?
(6) Schwartz and coworkers (8) did a retrospective study on 44 patients with enchondromatosis, 7 had Maffucci syndrome while 37 had Ollier disease.
A mutant PTH/PTHrP type I receptor in enchondromatosis. Nat Genet.
Enchondromatosis: insights on the different subtypes.
When multiple enchondromas are present in a single patient, the condition is called generalized enchondromatosis. If a patient demonstrates soft-tissue hemangiomas in addition to multiple enchondromas, the condition is called Maffucci syndrome.
The most common type of enchondromatosis is Ollier disease, a disorder characterized by multiple enchondromas arising in tubular and flat bones and sparing the skull and spine.
The exact cause of generalized enchondromatosis remains unknown.
Imagawa et al reported an enchondroma in the left middle cranial fossa of a patient with generalized enchondromatosis. (5)
The enchondromas in patients with generalized enchondromatosis exhibit a tendency to undergo malignant transformation into chondrosarcomas.
The parotid acinic cell carcinoma in our patient seemed to be unrelated to her generalized enchondromatosis. Our review of the literature found no association of parotid tumors with enchondromatosis.