1. Virtual Children's Hospital: Paediapaedia: Enchondromatosis A look at enchondromatosis by Michael P. D'Alessandro, M.D. Includes 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. Last Updated May 14, 2002 http://www.emedicine.com/radio/topic247.htm

(advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Radiology Musculoskeletal 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, Excerpt from Enchondroma and enchondromatosis. Synonyms, Key Words, and Related Terms chondroma, Ollier's disease, http://www.emedicine.com/radio/byname/enchondroma-and-enchondromatosis.htm

(advertisement) 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. Uhrad.com Pediatric Imaging Teaching Files uhrad.com Pediatric Imaging Teaching Files Case Fifty Five - Multiple enchondromatosis Click on Images for Enlarged View Clinical History Trauma to second right digit. Diagnosis Multiple enchondromatosis. Discussion enchondromatosis (Ollier's Disease) is a non hereditary disorder of 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.

5. 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.

6. Virtual Children's Hospital: Paediapaedia: Musculoskeletal Disease Index Thanatophoric; enchondromatosis (Ollier Disease); Ewing Sarcoma; FibrousDysplasia, Monostotic; Fibrous Dysplasia, Polyostotic; Fracture http://www.vh.org/pediatric/provider/radiology/PAP/MSDiseases/MSDisIndex.html

Paediapaedia: Musculoskeletal Diseases Musculoskeletal Disease Index Michael P. D'Alessandro, M.D. Peer Review Status: Internally Peer Reviewed Normal Neonatal Pelvis and Hips Achondroplasia Aneurysmal Bone Cyst (ABC) Battered Child Syndrome, Bone Injuries (Child Abuse) (Trauma X) ... Title Page See related Provider Topics Bone Diseases Bones, Joints and Muscles Ear, Nose and Throat Endocrine System (Hormones) ... Scoliosis or Thyroid Diseases See related Patient Topics Bone Diseases Bones, Joints and Muscles Ear, Nose and Throat Endocrine System (Hormones) ... Scoliosis or Thyroid Diseases Virtual Children's Hospital Home Virtual Hospital Home Site Map ... UI Health Care Home http://www.vh.org/pediatric/provider/radiology/PAP/MSDiseases/MSDisIndex.html

7. HONselect - Enchondromatosis Translate this page English enchondromatosis, - Enchondroma, Multiple - Enchondrosis, Multiple- Maffucci Syndrome - Ollier's Disease - Ollier Disease - Disease, Ollier. 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 Yes Español No Português No Home About us Site map Feedback ... HONewsletter http://www.hon.ch/HONselect/RareDiseases/C05.116.099.708.338.html Last modified: Thu Jul 25 2002

8. ELibrary.com - The Mosby Medical Encyclopedia 10-01-1996, eLibrary is the subscription based online library for fun or research. Find out more about securing your guaranteed Free 7day trial with your credit card and retrieve 'eLibrary.com - The Mosby Medical Encyclopedia 10-01-1996, 'enchondromatosis'' http://redirect-west.inktomi.com/click?u=http://ask.elibrary.com/getdoc.asp%3Fpu

9. HON - List Of Rare Diseases Eisenmenger Complex, EllisVan Creveld Syndrome. Encephalitis, enchondromatosis.Epidermal Necrolysis, Toxic. Fabry Disease, Facial Hemiatrophy. http://www.hon.ch/HONselect/RareDiseases/

List of rare diseases: English Deutsch Acrocephalosyndactylia Acrodermatitis ... HONewsletter http://www.hon.ch/HONselect/RareDiseases/index.html Last modified: Thu Sep 26 2002

10. Enchondromatosis Children with enchondromatosis may be susceptible to pathologic fractures, and adults may be at risk of malignant http://www.amersham-health.com/medcyclopaedia/Volume%20III%201/ENCHONDROMATOSIS.

Medcyclopaedia About Medcyclopaedia Amersham Health Search for: Type a word or a phrase. All forms of the word are searchable. Advanced search Browse entry words starting with: A B C D ... Other characters Try our Medcyclopaedia Premium Edition with added tools and functionality tailored to make your working day easier. The following tools are presently available: Expanded search *For Medical Professionals only, registration required 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

11. Enchondromatosis Medical Professionals only. enchondromatosis,, Print this article,presence of enchondromatosis, Fig. 1 a. AP radiograph demonstrates http://www.amershamhealth.com/medcyclopaedia/Volume III 1/ENCHONDROMATOSIS.asp

Medcyclopaedia About Medcyclopaedia Amersham Health Search for: Type a word or a phrase. All forms of the word are searchable. Advanced search Browse entry words starting with: A B C D ... Other characters Try our Medcyclopaedia Premium Edition with added tools and functionality tailored to make your working day easier. The following tools are presently available: Expanded search *For Medical Professionals only, registration required 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

12. ORPHANET® : Enchondromatosis Base de données sur les maladies rares et les médicaments orphelins ORPHANET database access. enchondromatosis. Direct access to details http://orphanet.infobiogen.fr/static/GB/enchondromatosis.html

ORPHANET database access Enchondromatosis Direct access to details Alias : Home Page

13. Enchondromatosis, Multiple enchondromatosis, multiple,, Print this article, enchondromatosis, multiple,Fig. 1 Radiograph of the lower limbs in an affected sixyear-old child. http://www.amershamhealth.com/medcyclopaedia/Volume VII/ENCHONDROMATOSIS MULTIPL

Medcyclopaedia About Medcyclopaedia Amersham Health Search for: Type a word or a phrase. All forms of the word are searchable. Advanced search Browse entry words starting with: A B C D ... Other characters Try our Medcyclopaedia Premium Edition with added tools and functionality tailored to make your working day easier. The following tools are presently available: Expanded search *For Medical Professionals only, registration required 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 (

14. Multiple Enchondromatosis For Medical Professionals only. Multiple enchondromatosis . see enchondromatosis multiple http://www.amersham-health.com/medcyclopaedia/Volume%20VII/MULTIPLE%20ENCHONDROM

Medcyclopaedia About Medcyclopaedia Amersham Health Search for: Type a word or a phrase. All forms of the word are searchable. Advanced search Browse entry words starting with: A B C D ... Other characters Try our Medcyclopaedia Premium Edition with added tools and functionality tailored to make your working day easier. The following tools are presently available: Expanded search *For Medical Professionals only, registration required Multiple enchondromatosis, see enchondromatosis multiple HC The Encyclopaedia of Medical Imaging Volume VII Welcome to Medcyclopaedia. This site is open to a public audience, still we want to know a little more about our visitors. Please tick off the boxes that match your profile. Do you live in Europe? Yes No Are you a medical professional? Yes No This frame will disappear when submitted Legal Contact us Making Waves

15. ENCHONDROMATOSIS, GENERALISED Features Listed For enchondromatosis, GENERALISED.McKusick 166000. Chromosome(s) http://www.hgmp.mrc.ac.uk/dhmhd-bin/hum-look-up?540

16. Ollier's Disease (multiple Enchondromatosis) Ollier's disease (multiple enchondromatosis) Describe the radiographic features of solitary enchondroma Describe the clinical and radiographic features of Ollier's disease Describe orthopaedic problems secondary to Ollier's disease http://www.posna.org/CoreCurriculum/Book3/Ollier%27s%20disease%20%28multiple%20e

17. ENCHONDROMATOSIS-VERTEBRAL ANOMALIES Features Listed For enchondromatosisVERTEBRAL ANOMALIES. McKusick Asymmetric legs/hindlimbs;Cutis laxa; Enchondromata; Enlarged joints; Excessive skin wrinkling; http://www.hgmp.mrc.ac.uk/dhmhd-bin/hum-look-up?539

18. Pediatric Orthopaedic Core Curriculum cortical defect Fibrous dysplasia Giant cell tumor Hereditary multiple exostoses(HME) Ollier's disease (multiple enchondromatosis) Osteochondroma Osteoid http://www.posna.org/CoreCurriculum/CoreCurriculum.htm

Pediatric Orthopaedic Core Curriculum General Regional You'll need Adobe Acrobat Reader to see and print these files General Prenatal testing Developmental milestones Growth Enchondral ossification ... Walking gait Genetics Genetics Chromosomal disorders Autosomal dominant disorders Autosomal recessive disorders ... Sex-linked recessive disorders Congenital Deficiencies Congenital deficiency of the upper limb Skeletal Dysplasias Achondroplasia Infantile hyperostosis Arthrogryposis Multiple epiphyseal dysplasia (MED) ... Short stature Diseases of Muscle Becker muscular dystrophy Congenital muscular dystrophy Congenital myopathies Duchenne muscular dystrophy ... Myotonic dystrophy Blood Dyscrasias Hemophilia Sickle cell disease and related hemoglobinopathies (return to top) Benign Tumors Aneurysmal bone cyst Chondroblastoma Eosinophilic granuloma Fibrous cortical defect ... Unicameral bone cyst Malignant Tumors Ewing's sarcoma Osteosarcoma Neurologic Disorders Cerebral palsy Friedreich ataxia Hereditary motor sensory neuropathy Poliomyelitis ... Thyroid disorders Infection Acute osteomyelitis Chronic osteomyelitis Septic arthritis Subacute osteomyelitis Trauma Child abuse Fractures associated with head injury Fractures of the physis Overuse injuries ... The multiply injured child (Other systems) Miscellaneous Heavy metal intoxication Idiopathic chondrolysis Juvenile arthritis Marfan syndrome ... Prosthetics and orthotics Regional

19. ORPHANET® : Enchondromatosis Translate this page ORPHANET. ORPHANET database access. enchondromatosis. Direct accessto details Alias Maffucci syndrome,Ollier disease. Home Page. http://www.orpha.net/static/GB/enchondromatosis.html

ORPHANET database access Enchondromatosis Direct access to details Alias : Home Page

20. Service Page - Pathologie Information DISEASE enchondromatosis, Synonym(s) Maffucci syndrome Ollier disease,CIM Q78.4, Maffucci's syndrome is characterized by the presence http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=296

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