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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Citation This paper should be referenced as such: Most cases of Ollier disease have been sporadic Halal and Azouz, Treatment Treatment depends on the type of enchondromatosis; it may include surgery, amputation, bone grafting and sclerotherapy.

Enchondromatosis

Ollier-related changes in both hips, pubic bone, left and right iliac bones, and a narrow pelvic canal were seen in the patient’s pelvic X-ray, taken six weeks later. Case 6 Case 6. The malignant potential of enchondromatosis.

Prognosis The prognosis is dependent on the extent and severity of the disease. We are determined to keep this website freely accessible.

The prevalence of this disease is 1 inEdit article Share article View revision history. Zur Erblichkeit der Knochenchondromatose. Lifelong monitoring is required in olliers patients given the risk of malignant transformation [ 6 ].

However routine use of magnetic resonance imaging is not recommended because plain radiographs provide adequate information [ 2 ]. Is it all in the genes? Radiologically, Ollier disease presents with asymmetrical osteolytic lesions with well-defined, sclerotic margins.

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Enchondromatosis | Radiology Reference Article |

Conclusion Enchondromas are common intraosseous tumours which are usually benign and develop in close proximity to growth plate cartilage. The diagnosis is mainly based on clinical, histological and radiological evaluation.

Ollier’s disease Ollier syndrome Multiple enchondromatosis Ollier’s syndrome Ollier disease. Second edition,Oxford University Press. Surgical intervention is necessary in case of pathological fractures, growth defect and malignant transformation [ 1 ].

Steudel described 2 affected brothers, and Rossberg reported affected brother and sister whose paternal grandfather was also affected.

J Enchomdromatosis Joint Surg Am. Support Center Support Center. Radiographs showed radiolucent lesions of fingers, pelvis and lower end of the right femur, which were diagnosed as enchondromas.

Multiple Enchondromatosis: Olliers Disease- A Case Report

Genome-wide analysis of Ollier disease: Symptoms begin with the appearance of palpable bony masses on a finger or a toe, an asymmetric shortening of an extremity with limping, osseous deformities associated with or without pathologic fractures [ 3 ]. If involvement of the lower limbs is significant, leg length discrepancy may occur. A year-old female patient presented to our hospital with difficulty in walking, leg pain and multiple swellings in hands and leg for 2 years. Enchondromas in olliers disease present a risk of nultiple transformation into chondrosarcomas.

Case 8 Case 8. From Wikipedia, the free encyclopedia. Halal and Azouz reported the case of a enchondromatoxis who had platyspondyly and metaphyseal manifestations of enchondromatosis with severe involvement of the hands and feet compatible with generalized enchondromatosis, or Spranger type VI enchondromatosis.

Thank you for enchondromatosiis your details. Ollier disease is not mu,tiple determined in a simple Mendelian manner. Atlas of Genetics and Cytogenetics in Oncology and Haematology. Pathological fractures, a consideration with metachondromatosis and differential diagnoses: Infobox medical condition new All stub articles. Figure 2 and 3.

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Enchondromatosis – Wikipedia

The diagnosis is based on clinical and conventional radiological evaluations. When hemangiomata are associated, the condition is known as Maffucci syndrome The father was short of stature and had only moderate platyspondyly. Metaphyseal dysplasia Jansen’s metaphyseal chondrodysplasia Schmid metaphyseal chondrodysplasia.

Generalized enchondromatosis in a boy with only platyspondyly in the father. The non-hereditary asymmetrical polyostotic distribution of the lesions might suggest multpile somatic mosaic mutation.

The osteochondromas seen in hereditary multiple exostosis are located at the bone surface whereas enchondromas are located in the centre of bones [ 3 ].

Radiograph showing radiolucent expansile lesions involving the metacarpals and phalanges bilaterally.

Clinics Note Clinical behaviour is determined by size, number, location and evolution of enchondromas, age of onset and of diagnosis. Chondrosarcoma in Maffucci’s syndrome.

They can occur as solitary lesions or as multiple lesions in enchondromatosis. Enchondromatosis with features of dysspondyloenchondromatosis and Maffucci syndrome. Olliers disease must be differentiated from hereditary multiple exostosis.