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1. Virtual Children's Hospital: Paediapaedia: Enchondromatosis
Clinical presentation, etiology and imaging findings.
http://www.vh.org/Providers/TeachingFiles/PAP/MSDiseases/Enchondromatosis.html
Paediapaedia: Musculoskeletal Diseases
Enchondromatosis
Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed Clinical Presentation:
Can have asymmetric limb shortening, defective articulation and swelling of the fingers and toes. The enchondromata are asymptomatic unless a pathologic fracture occurs. Etiology/Pathophysiology:
Multiple nests or streaks of disorganized cartilage cells probably representing persistence of cortical cartilage that was not transformed into osteoblasts and compact bone. The combination of enchondromatosis and multiple hemangiomas is known as Maffuci's Syndrome.They stop forming at puberty, and from then on there is remodeling and replacement of the cartilaginous lesions with bone. Patients can frequently be left with asymmetric limbs. Pathology:
Not applicable Imaging Findings:
Radiolucent metaphyseal defects are seen in tubular or flat bones. The masses expand asymmetrically with thinning of cortex over the affected area. In later stages of the disease the lesions are more frequently linear or striated. DDX:
  • Multiple cartilaginous exostosis
  • Metachondromatosis
  • Metaphyseal chondrodysplasia
  • Fibrous dysplasia
References:
See References Chapter.

2. EMedicine - Enchondroma And Enchondromatosis : Article By Felix S Chew, MD, EdM
Enchondroma and enchondromatosis Enchondromas are benign cartilaginous neoplasms in bone. Enchondroma and enchondromatosis. Last Updated May 14, 2002,
http://www.emedicine.com/radio/topic247.htm
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Enchondroma and Enchondromatosis
Last Updated: May 14, 2002 Rate this Article Email to a Colleague Synonyms and related keywords: chondroma, Ollier's disease, Ollier disease, benign cartilaginous neoplasms, benign bone neoplasms, osseous neoplasms, pathologic bone fracture, hyaline cartilage rests AUTHOR INFORMATION Section 1 of 12 Author Information Introduction Differentials X-ray ... Bibliography
Author: Felix S Chew, MD, EdM , Vice-Chair for Education, Section Head of Musculoskeletal Radiology, Professor, Department of Radiology, Wake Forest University School of Medicine Coauthor(s): Catherine Maldjian, MD , Consulting Staff, Clifton Medical Imaging Felix S Chew, MD, EdM, is a member of the following medical societies: American Roentgen Ray Society , Association of University Radiologists, and Radiological Society of North America Editor(s): Michael A Bruno, MD

3. EMedicine - Enchondroma And Enchondromatosis : Article Excerpt By: Felix S Chew,
Enchondroma and enchondromatosis Enchondromas are benign cartilaginous neoplasms in bone. (advertisement). Excerpt from Enchondroma and enchondromatosis.
http://www.emedicine.com/radio/byname/enchondroma-and-enchondromatosis.htm
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Excerpt from Enchondroma and Enchondromatosis
Synonyms, Key Words, and Related Terms: chondroma, Ollier's disease, Ollier disease, benign cartilaginous neoplasms, benign bone neoplasms, osseous neoplasms, pathologic bone fracture, hyaline cartilage rests
Please click here to view the full topic text: Enchondroma and Enchondromatosis
Background: Enchondromas are benign cartilaginous neoplasms in bone. The primary significance of enchondroma is related to its complications, most notably pathologic fracture, and a small incidence of malignant transformation, which may be associated with pathologic fracture. Enchondromas are usually solitary benign lesions in intramedullary bone. When multiple enchondromas coexist, the diagnosis of enchondromatosis should be considered. Multiple enchondromas may occur in 3 distinct disorders: (1) Ollier disease is a nonhereditary disorder characterized by multiple enchondromas with a predilection for unilateral distribution. The enchondromas can grow large and be disfiguring. (2) Maffucci syndrome is nonhereditary, is less common than Ollier disease. This syndrome results in multiple hemangiomas in addition to enchondromas. (3) Metachondromatosis consists of multiple enchondromas and osteochondromas, and it is the only 1 of the 3 disorders that is hereditary. Metachondromatosis is inherited by autosomal dominant transmission. Pathophysiology: Enchondromas are ectopic hyaline cartilage rests in intramedullary bone. The lesions replace normal bone with mineralized or unmineralized hyaline cartilage, thereby generating a lytic pattern on radiographs or, more commonly, a lytic area containing rings and arcs of chondroid calcifications. The lesions probably arise from cartilaginous rests that are displaced from the growth plate.

4. HONselect - Enchondromatosis
List of rare diseasesEnglish Français - Deutsch - Español - Português. Language MeSH term Accepted terms English enchondromatosis - Enchondroma, Multiple - Enchondrosis, Multiple - Maffucci Syndrome - Ollier's Disease -
http://www.hon.ch/HONselect/RareDiseases/C05.116.099.708.338.html
List of rare diseases: English Deutsch
Language:
MeSH term:
Accepted terms:
English: Enchondromatosis - Enchondroma, Multiple
- Enchondrosis, Multiple
- Maffucci Syndrome
- Ollier's Disease
- Ollier Disease
- Disease, Ollier
Français: ENCHONDROMATOSE - CHONDROMATOSE MULTIPLE - DYSCHONDROPLASIE - OLLIER, MALADIE Deutsch: Enchondromatose - Enchondromatose, multiple - Enchondrosis, multiple - Maffucci-Syndrom - Ollier-Krankheit - Chondrodystrophie-Angiomatose-Syndrom Español: ENCONDROMATOSIS - ENCONDROMA MULTIPLE - ENCONDROSIS MULTIPLE - SINDROME DE MAFFUCI - ENFERMEDAD DE OLLIER Português: ENCONDROMATOSE - ENCONDROMA MULTIPLO - ENCONDROSE MULTIPLA - SINDROME DE MAFUCCI - DOENCA DE OLLIER HONselect ressources Definition: Yes Articles: Yes Images: Yes News: No Conferences: No Clinical trials: No Web sites: English Yes Français Yes Deutsch No Español Yes Português No Home About us Site map Feedback ... HONewsletter http://www.hon.ch/HONselect/RareDiseases/C05.116.099.708.338.html Last modified: Wed Apr 28 2004

5. A Mutant PTH/PTHrP Type I Receptor In Enchondromatosis
Published online 19 February 2002, doi10.1038/ng844. volume 30 no. 3 pp 306 310. A mutant PTH/PTHrP type I receptor in enchondromatosis. Sevan Hopyan1, 2, 3, 4, Nalan Gokgoz2, Raymond Poon1, Robert C. Gensure5, Chunying Yu1, William G. or as multiple lesions in enchondromatosis (Ollier and Maffucci diseases). Clinical problems
http://www.nature.com/cgi-taf/DynaPage.taf?file=/ng/journal/v30/n3/abs/ng844.htm

6. AAMED: American Association Of Multiple Enchondroma Diseases
Together in Our Search for a Cure Ollier's disease, Maffucci's syndrome, enchondromatosis, their families, and physicians. adults and children with enchondromatosis, Multiple Enchondroma, Ollier's disease
http://www.aamed.net/
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Founded in 1998, incorporated in 2002 as a not-for-profit corporation and registered as a tax exempt organization with the US Internal Revenue Service, AAMED is comprised of individuals with Ollier's disease, Maffucci's syndrome, enchondromatosis, their families, and physicians. AAMED is THE source for news and information about bone tumor diseases, research and services for adults and children with Enchondromatosis, Multiple Enchondroma, Ollier's disease, Maffucci's syndrome, and their families. On this site, you'll find links to our quarterly newsletters, message board, pen-pal program for children, latest research information, and much more. We invite interested persons to become part of group. Click on the membership link for more information on joining us.
OUR ACTIVITIES
  • participation in research projects to find treatments and a cure for these diseases
  • helping families cope with the physical/psychological burdens imposed by the disease
  • we provide a forum for patients and families to express their concerns and experiences
  • connect individuals with common symptoms and backgrounds
  • we continuously update a data base of physicians and hospitals with Ollier/Maffucci expertise
  • Videos on limb lengthening and the use of the Ilizarov fixator

7. Enchondromatosis Information Diseases Database
enchondromatosis,Maffucci syndrome,Multiple enchondrosis,Ollier s disease, Disease Database Information.
http://www.diseasesdatabase.com/ddb9212.htm
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4 synonyms or equivalents were found. Enchondromatosis
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Maffucci syndrome
aka/or
Multiple enchondrosis
aka/or
Ollier's disease Enchondromatosis: Definition(s) via UMLS Code translations and terms via UMLS Enchondromatosis: specific sites Send Enchondromatosis to medical search engines (JavaScript enabled browsers only) If your browser has no JavaScript you can still use these:
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8. Enchondromatosis Information Diseases Database
enchondromatosis Maffucci syndrome Multiple enchondrosis Ollier's disease, Disease Database Information
http://www.diseasesdatabase.com/sieve/item1.asp?glngUserChoice=9212

9. Enchondromatosis
enchondromatosis,. Print this article, presence of multiple foci of cartilage enchondromatosis, Fig. 1. a. AP radiograph demonstrates large
http://www.amershamhealth.com/medcyclopaedia/medical/Volume III 1/ENCHONDROMATOS
Amershamhealth.com Search for: Type a word or a phrase. All forms of the word are searchable. Browse entry words starting with: A B C D ... Other characters Enchondromatosis, presence of multiple foci of cartilage within bones and subperiosteal deposition of cartilage, involving one side of the body exclusively or predominantly. This disorder is nonhereditary and is also termed Ollier's disease. The tumours occur most frequently in the extremities but may also be found in the flat bones, such as those of the pelvis. Affected bones may be shortened and deformed. Children with enchondromatosis may be susceptible to pathologic fractures, and adults may be at risk of malignant transformation to chondrosarcoma Radiographically, the tumours may appear as linear or columnar radiolucent regions in the metaphyses or flat bones, which represent sites of persistent cartilaginous tissue ( Fig.1 ). Erosion and proliferation of the bone surface are sometimes observed. A nonhereditary enchondromatosis with soft tissue haemangiomas that may undergo malignant transformation is known as Maffuccis syndrome
DR/RB
The Encyclopaedia of Medical Imaging Volume III:1 Enchondromatosis, Fig. 1

10. Enchondromatosis, Multiple
enchondromatosis, multiple,. Print this article, HC. The Encyclopaedia of Medical Imaging Volume VII. enchondromatosis, multiple, Fig.
http://www.amershamhealth.com/medcyclopaedia/medical/Volume VII/ENCHONDROMATOSIS
Amershamhealth.com Search for: Type a word or a phrase. All forms of the word are searchable. Browse entry words starting with: A B C D ... Other characters Enchondromatosis, multiple, (also called Ollier's disease), multiple enchondromas, i.e. disorganized cartilaginous foci within the bones which causes bony swellings of the affected bones. In the long bones, streaky linear depositions of cartilage within the metaphysis may also occur. The disease often dominantly affects one side of the body. On other occasions it may affect the hands or feet, or be generalized. It is not an inherited disease. Clinically, there are bony swellings, limb shortening and mechanical difficulties, associated with joint disruption and short stature, which may be asymmetrical. The condition usually presents between 2 and 10 years of age. There may be focal restriction of growth at the site of a lesion which may result in limb shortening, Madelung's deformity or kyphosis. The characteristic radiological appearances are columnar radiolucencies extending from the metaphyses into the shafts of tubular bones; there may be associated stippled calcification ( Fig.1

11. Enchondromatosis - Medical Dictionary Definitions Of Popular Medical Terms
enchondromatosis A condition characterized by multiple enchondromas benign masses of cartilage growing within bones. The enchondromas
http://www.medterms.com/script/main/art.asp?articlekey=20861

12. Uhrad.com Pediatric Imaging Teaching Files
uhrad.com Pediatric Imaging Teaching Files. Case Fifty Five - Multiple enchondromatosis. Click Diagnosis Multiple enchondromatosis. Discussion
http://www.uhrad.com/pedsarc/peds055.htm
uhrad.com - Pediatric Imaging Teaching Files
Case Fifty Five - Multiple Enchondromatosis
Click on Images for Enlarged View Clinical History: Trauma to second right digit. Findings: Multiple lucent metaphyseal lesions of the proximal and middle phalanges of the fourth and fifth digits, as well as the distal metacarpals of the fourth and fifth digits. Diagnosis: Multiple enchondromatosis. Discussion: Enchondromatosis (Ollier's Disease) is a non hereditary disorder of enchondral ossification with lesions distributed throughout the tubular and flat bones of the body. The presenting clinical manifestations are masses that increase in size as the child grows, asymmetric limb shortening and either genu varus or genu valgus deformities. Radiographs of the young child could be normal in the first few years of life and later demonstrate radiolucent streaking within the metaphysis of involved bones, or at infancy may demonstrate radiolucent masses which can be round, triangular or linear. Stippled calcifications occur with age within the enchondromas. There may be considerable expansion of the tubular bones especially in the hands, and pathologic fractures may occur. Cartilaginous areas extending from the physis can lead to a growth interference resulting in limb shortening and deformities. Enchondromas typically stabilize or regress in adulthood. However, sarcomatous transformation has been described. Six types of enchondromatosis have been described, of which three are more common. If the multiple enchondromas are purely unilateral or unevenly distributed throughout the metaphyses of the long bones, sparing the cranium and spine, it is called Ollier's Disease. If the enchondromas are seen with multiple cutaneous hemangiomas (soft tissue calcifications/phleboliths) it is called Maffucci's Syndrome. If there is symmetric involvement throughout the body including the cranium, hands and feet, it is known as generalized enchondromatosis. The Ollier's and Maffucci's are more common in boys (2:1). The less frequent types include metachondromatosis which affects the short tubular bones of the hands and feet, and spondyloenchondroplasia in which the enchondromas are associated with platyspondyly, and enchondromas with irregular vertebral bodies.

13. Ollier Disease - Information / Diagnosis / Treatment / Prevention
AAMED is a source for news and information about bone tumor diseases, research and services for adults and children with enchondromatosis, Multiple Enchondroma
http://www.healthcyclopedia.com/rare-disorders/ollier-disease.html

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Related Topics: Musculoskeletal Disorders Medical Definition: University of Newcastle-upon-Tyne Medical Dictionary: "ollier disease" Health News: Search millions of published articles for news on Ollier Disease Modern Medicine Aging The Ardell Wellness Report HealthFacts Medical Post Medical Update Men's Health and the National Women's Health Report Note: Subscription required to access the full text of articles. Web Directory: NORD: Ollier Disease Sample report, plus links to organisations. [Fee required for full report] Virtual Children's Hospital: Enchondromatosis Clinical presentation, etiology and imaging findings. Notes: Healthcyclopedia presents health information in the form of websites and articles that encompass conventional medicine and alternative treatments Under no circumstances can it recommend or endorse a specific drug or therapy or treatment.

14. Genetic Conditions / Rare Conditions Information Site
Maffucci syndrome(Ollier, multiple cartilaginous enchondromatosis) Malignant hyperthermia. Maple syrup urine disease
http://www.kumc.edu/gec/support
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15. Virtual Children's Hospital: Paediapaedia: Enchondromatosis
Paediapaedia Musculoskeletal Diseases enchondromatosis. http//www.vh.org/pediatric/provider/radiology/PAP/MSDiseases/enchondromatosis.html.
http://www.vh.org/pediatric/provider/radiology/PAP/MSDiseases/Enchondromatosis.h
Paediapaedia: Musculoskeletal Diseases
Enchondromatosis
Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed Clinical Presentation:
Can have asymmetric limb shortening, defective articulation and swelling of the fingers and toes. The enchondromata are asymptomatic unless a pathologic fracture occurs. Etiology/Pathophysiology:
Multiple nests or streaks of disorganized cartilage cells probably representing persistence of cortical cartilage that was not transformed into osteoblasts and compact bone. The combination of enchondromatosis and multiple hemangiomas is known as Maffuci's Syndrome.They stop forming at puberty, and from then on there is remodeling and replacement of the cartilaginous lesions with bone. Patients can frequently be left with asymmetric limbs. Pathology:
Not applicable Imaging Findings:
Radiolucent metaphyseal defects are seen in tubular or flat bones. The masses expand asymmetrically with thinning of cortex over the affected area. In later stages of the disease the lesions are more frequently linear or striated. DDX:
  • Multiple cartilaginous exostosis
  • Metachondromatosis
  • Metaphyseal chondrodysplasia
  • Fibrous dysplasia
References:
See References Chapter.

16. DOE Document - Generalized Enchondromatosis With Unusual
Generalized enchondromatosis is a newly delineated type of enchondromatosis.^Radiographically there are multiple enchondromata in almost all metaphyses of the long and short tubular bones and the
http://rdre1.inktomi.com/click?u=http://www.osti.gov/energycitations/product.bib

17. ENCHONDROMATOSIS
Terms of Use. enchondromatosis. Specialty Definition enchondromatosis. Domain, Definition. Health. Top. Frequency of Internet Keywords enchondromatosis.
http://www.websters-online-dictionary.org/definition/english/En/Enchondromatosis
Philip M. Parker, INSEAD.
ENCHONDROMATOSIS
Specialty Definition: ENCHONDROMATOSIS
Domain Definition
Health
Benign growths of cartilage in the metaphyses of several bones. ( references Source: compiled by the editor from various references ; see credits. Top
Frequency of Internet Keywords: ENCHONDROMATOSIS
The following statistics estimate the number of searches per day across the major English-language search engines as identified by various trade publications. Hyperlinks lead to commercial use of the expression at Amazon.com
Expression Frequency
per Day
enchondromatosis
Source: compiled by the editor from various references ; see credits. Top
Modern Translation: ENCHONDROMATOSIS
Language Translations for "ENCHONDROMATOSIS"; alternative meanings/domain in parentheses.
French
enchondromatose various references
German
Enchondromatose various references
Pig Latin
enchondromatosisay
Spanish
encondromatosis
various references Source: compiled by the editor from various translation references Top
Anagrams: ENCHONDROMATOSIS
Words within the letters "a-c-d-e-h-i-m-n-n-o-o-o-r-s-s-t" -3 letters : chondriosomes.

18. Musculoskeletal Diseases
EllisVan Creveld Syndrome - enchondromatosis - Eosinophilia Myalgia Syndrome - Exostoses Silver Syndrome - an overview - MedlinePlus/ADAM. enchondromatosis. About Ollier's disease
http://www.mic.ki.se/Diseases/c5.html
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Diseases and Disorders Links pertaining to Musculoskeletal Diseases Alert! Patients and laypersons looking for guidance among the target sources of this collection of links are strongly advised to review the information retrieved with their professional health care provider. Start Page Contents: Achondroplasia Achondroplasia Acquired Hyperostosis Syndrome Acrocephalosyndactylia ... Tietze's Syndrome
Musculoskeletal Diseases Textbook of Orthopaedics [CR Wheeless] Educational textbook syllabus [Borrill et al.] - Orthoteers (UK) The Belgian OrthoWeb - (BE) Site Map of the Orthopaedics and Sports Medicine resource at Univ of Washington, Seattle (US) The American Academy of Orthopaedic Surgeons A Musculoskeletal Atlas and a set of educational cases (radiol.) - U of Washington (US) OrthoLinx WorldOrtho including A Simple Guide to Orthopaedics [RL Huckstep] - Nepean Hosp., Sydney (AU) A collection of Case discussions at OrthoGate.org An orthopedic Encyclopedia - for patients - DynoMed.com

19. Ollier Disease
Synonyms Multiple enchondromatosis; Multiple Cartilaginous Enchondroses; Dyschondroplasia; enchondromatosis. Disorder Subdivisions None. General Discussion
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Health Topics Symptoms ... For a Complete Report Ollier Disease Important It is possible that the main title of the report Ollier Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms
  • Multiple Enchondromatosis Multiple Cartilaginous Enchondroses Dyschondroplasia Enchondromatosis
Disorder Subdivisions
  • None
General Discussion Ollier disease is a rare skeletal disorder characterized by abnormal bone development (skeletal dysplasia). While this disorder may be present at birth (congenital); it may not become apparent until early childhood when symptoms, such as deformities or improper limb growth, are more obvious. Ollier disease primarily affects the long bones and cartilage of the joints of the arms and legs, specifically the area where the shaft and head of a long bone meet (metaphyses). The pelvis is often involved; and even more rarely, the ribs, breast bone (sternum), and/or skull may also be affected. Ollier disease manifests as greater than normal growth of the cartilage in the long bones of the legs and arms so that growth is abnormal and the outer layer (cortical bone) of the bone becomes thin and more fragile. These masses of cartilage are benign (non-cancerous) tumors known as enchondromas. Enchondromas may occur at anytime. After puberty these growths stabilize as cartilage is replaced by bone. In rare cases, the enchondromas may undergo malignant changes (e.g., chondrosarcomas). The exact cause of Ollier disease is not known, although in some cases it may be inherited as an autosomal dominant genetic trait.

20. Maffucci Syndrome
subdivision(s) covered by this report. Synonyms Dyschondrodysplasia with Hemangiomas; enchondromatosis with Multiple Cavernous Hemangiomas;
http://my.webmd.com/hw/raising_a_family/nord433.asp
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Health Topics Symptoms ... For a Complete Report Maffucci Syndrome Important It is possible that the main title of the report Maffucci Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms
  • Dyschondrodysplasia with Hemangiomas Enchondromatosis with Multiple Cavernous Hemangiomas Hemangiomatosis Chondrodystrophica Kast Syndrome Multiple Angiomas and Endochondromas
Disorder Subdivisions
  • None
General Discussion Maffucci Syndrome is a rare genetic disorder characterized by benign overgrowths of cartilage (enchondromas), skeletal deformities, and dark red irregularly shaped patches of skin (hemangiomas). Enchondromas are most often found in certain bones (phalanges) of the hands and feet. Skeletal malformations may include legs that are disproportionate in length and/or abnormal side-to-side curvature of the spine (scoliosis). In many cases, bones may tend to fracture easily. In most cases, hemangiomas appear at birth or during early childhood and may be progressive. Maffucci Syndrome is inherited as an autosomal dominant genetic trait. Resources Ollier/Maffucci Self-Help Group C/O Hermann Schmid

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