ENCHONDROMATOSIS MULTIPLE PDF

Enchondromatosis is a rare heterogeneous skeletal disease characterized by multiple enchondromas, and it includes several different subtypes of which Ollier . Enchondromas are common benign cartilage tumors of bone. They can occur as solitary lesions or as multiple lesions in enchondromatosis. Enchondromatosis, also known as Ollier disease (see Terminology section), is a non-hereditary, sporadic, skeletal disorder characterised by multiple.

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However, there is a wide variety of presentations in terms of size, number, location, evolution of enchondromas, age of onset and of diagnosis, and requirement for surgery. As there was a bony prominence obstructing the pelvic canal, and she declined elective Cesarean section, controlled rupture of membranes was done. Enchondromatosis is a rare heterogeneous skeletal disease characterized by multiple enchondromas, and it includes several different subtypes of which Ollier disease and Maffucci syndrome are most common.

Multiple Enchondromatosis: Olliers Disease- A Case Report

In the original description of Ollier disease, muktiple enchondromas were predominantly confined to one side and limited to the limbs. The patient presented with various deformations and shortening of the limbs since the age of three years. This article has been cited by other articles in PMC. If pain occurs when the child is older, this should raise the concern of malignant transformation or pathological fracture.

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Enchondromatosis

Most patients have bilateral involvement but often significant asymmetry 4. By using this site, you agree to the Terms of Use and Privacy Policy. In their classification of the enchondromatoses, Spranger et al. C ] – Ovarian juvenile granulosa cell tumor with precocious pseudopuberty Radiology – Enchondromata Inheritance -? Plain radiographs are sufficient for diagnosis.

Anteroposterior radiograph of the pelvis showing columnar, radiolucent lesions in the right ilium extending to the crest and suggestive of cartilaginous rests. Epidemiology in the Arab World View Map. Typical enchondromatous multiple bony lesions in both hands, right proximal humerus, right iliac bone, right scapula, and left enchondromtosis sixth rib were noticed in the skeletal analysis.

However, as the cord was felt at the side of the head, emergency section was performed, uneventfully delivering a healthy enchondromztosis. In a case of human enchondromatosis, Hopyan et al.

Multiple enchondromatosis (Ollier disease)

Radiographically, the lesions of enchondromatosis typically show multiple, radiolucent or mineralized homogeneous well defined lesions with oval or elongated shape.

Views Read Edit View history. Unable to process the form. Achondrogenesis type 2 Hypochondrogenesis.

Enchondromatosis, Multiple – CAGS

Ollier disease can be considered a synonym for enchondromatosis. There are a few instances of familial occurrence. Edit History Tasneem Obeid: While the OMIM database is open to the public, users seeking enchondromatsois about a personal medical or genetic condition are urged to consult with a qualified physician for diagnosis and for answers to personal questions.

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Case 1 Case 1.

Unfortunately, it is not free to produce. J Bone Joint Surg Am. When malignancy is suspected, multipe histopathology investigation is used for grading because different grade requires different management [ 2 ]. Cytogenetics Note Karyotypes of patients with Ollier disease or Maffucci syndrome are normal. CC HPO: The tubular bones of the hand appear globular due to expansion. This rare combination is known as metachondromatosis.

Am J Med Genet A. Ollier disease and Maffucci syndrome are non-inherited disorders while spondyloenchondromatosis is inherited as an autosomal recessive disorder. Both the father and the son had consanguineous parents. Spondyloepiphyseal dysplasia congenita Multiple epiphyseal dysplasia Otospondylomegaepiphyseal dysplasia.

Support Center Support Center. Treatment Treatment depends on the type of enchondromatosis; it may include surgery, amputation, bone grafting and sclerotherapy.

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