Download Citation on ResearchGate | Encondromatosis múltiple, enfermedad de Ollier | Multiple enchondromatosis (Ollier’s disease) is a rare disease. PDF | Ollier disease (Spranger type I) is a rare bone disease that is characterized by multiple Encondromatosis múltiple, enfermedad de Ollier. Article. Enchondromatosis, also known as Ollier disease (see Terminology section), is a non-hereditary, sporadic, skeletal disorder characterised by multiple.
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Some authors make a distinction between Ollier disease and enchondromatosis encondrmatosis the basis of distribution. In the original description of Ollier disease, the enchondromas were predominantly confined to one side and limited to the limbs. As such, some authors prefer to use the term Ollier disease in cases where this distribution is present and use enchondromatosis or multiple enchondromatosis for cases where the distribution is more symmetric 5.
Ollier disease is seen in both sexes without gender predilection and usually becomes apparent by early childhood 3,4. The condition is non-hereditary and is thought to occur as a result of random and spontaneous mutations. Clinical presentation is usually with deformity and pain present only during periods of rapid growth 3,4. If pain occurs when the child is older, this should raise the concern of malignant transformation or pathological fracture. If involvement of the lower limbs is significant, leg length discrepancy may occur.
In distinction from solitary lesions, enchondromas of enchondromatosis are more likely to be hypercellular in nature, yet are still considered benign in the absence of other aggressive findings The etiology of enchondromatosis has been associated with post-zygote state mutations during development.
Enchondromatosis | Radiology Reference Article |
More interestingly, it has been reported that the PTHrP parathyroid hormone-related protein receptor has been implicated as a gene responsible for cases of enchondromatosis in patients with a family history of mild skeletal dysplasia in their male parents. Once the individual stops growing then in general, olliier do the enchondromas.
If growth occurs then, this raises the possibility of malignant transformation 4.
Occasionally patients will also be afflicted with multiple osteochondromas. This rare combination is known as metachondromatosis. They can occur anywhere but most frequently involve the short tubular bones of the hands and feet. Most patients have bilateral involvement but often significant asymmetry 4. Radiographs show multiple enchondromas.
For further discussion of appearances, please refer to the article on enchondromas. The extent of disease is highly variable and thus so is the degree of impairment. If only a few bones are involved, then little if any handicap is present, although the affected bones do have a higher rate of fracture.
The differential diagnosis for bone tumours is dependent on the age of the patient, with a very different set of differentials for the pediatric patient. You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Unable to process the form. Check for errors and try again. Thank you for updating your details.
Log in Sign up. Articles Cases Courses Quiz. About Blog Go ad-free. A encondrmoatosis clinical entity. J Bone Joint Surg Am. Related Radiopaedia articles Bone tumours The differential diagnosis for bone tumours is dependent on the age of the patient, with a very different set of differentials for the pediatric patient.
Edit article Share article View revision history. Synonyms or Alternate Spellings: Ollier’s disease Ollier syndrome Multiple enchondromatosis Ollier’s syndrome Ollier disease. Support Radiopaedia and see fewer ads.
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Multiple Enchondromatosis: Olliers Disease- A Case Report
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Encondromatosis múltiple, enfermedad de Ollier | Anales de Pediatría (English Edition)
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