Extractions: 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:
Extractions: (advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Radiology Musculoskeletal 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
Extractions: (advertisement) 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 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.
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
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
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/
Extractions: 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. 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
Enchondromatosis Information Diseases Database enchondromatosis,Maffucci syndrome,Multiple enchondrosis,Ollier s disease, Disease Database Information. http://www.diseasesdatabase.com/ddb9212.htm
Enchondromatosis Information Diseases Database enchondromatosis Maffucci syndrome Multiple enchondrosis Ollier's disease, Disease Database Information http://www.diseasesdatabase.com/sieve/item1.asp?glngUserChoice=9212
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
Extractions: 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
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
Extractions: 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
Extractions: 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.
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
Extractions: 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:
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
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
Extractions: Philip M. Parker, INSEAD. ENCHONDROMATOSIS Specialty Definition: ENCHONDROMATOSIS Domain Definition 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 per Day Source: compiled by the editor from various references ; see credits. Top Modern Translation: ENCHONDROMATOSIS Language Translations for "ENCHONDROMATOSIS"; alternative meanings/domain in parentheses. enchondromatose various references Enchondromatose various references enchondromatosisay 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.
Extractions: 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
Extractions: 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.
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
Extractions: 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
