enchondromatosis

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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.

enchondromatosis

/en·chon·dro·ma·to·sis/ (en-kon″dro-mah-to´sis) hamartomatous proliferation of cartilage cells within the metaphysis of several bones, causing thinning of the overlying cortex and distortion of the growth in length; it may undergo malignant transformation.

enchondromatosis

[en′kəndrō′mətō′sis]
Etymology: Gk, en + chondros, cartilage, oma, tumor, osis, condition
a congenital disorder characterized by the proliferation of cartilage within the extremity of the shafts of bones, causing thinning of the cortex and distortion in length. Also called dyschondroplasia, Ollier's disease. Compare Maffucci's syndrome.

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

enchondromatosis

a condition in humans characterized by hamartomatous proliferation of cartilage cells within the metaphysis of several bones, causing thinning of the overlying cortex and distortion of the growth in length. Called also dyschondroplasia, Ollier's disease.
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.
Somatic mosaic IDH1 or IDH2 mutations are associated with enchondroma and spindle cell hemangioma in Ollier disease and Maffucci syndrome.
Maffucci syndrome and Ollier disease is associated with multiple enchondromas with increased risk of malignant transformation (Fig.
Generalized enchondromatosis, of which Ollier disease is the most common type, is a rare bone dysplasia characterized by multiple intraosseous tumors near growth-plate cartilage.
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.