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         Enchondromatosis:     more detail

1. Virtual Children's Hospital: Paediapaedia: Enchondromatosis
The combination of enchondromatosis and multiple hemangiomas is known as Maffuci sSyndrome.They stop forming at puberty, and from then on there is
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.

2. 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.

3. Enchondromatosis
A nonhereditary enchondromatosis with soft tissue haemangiomas that may of Medical Imaging Volume III1. enchondromatosis, Fig. 1 a. AP
http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

4. EMedicine - Enchondroma And Enchondromatosis : Article By Felix S Chew, MD, EdM
Enchondroma and enchondromatosis Enchondromas are benign cartilaginous neoplasmsin bone. The primary significance of enchondroma is related to its
http://www.emedicine.com/radio/topic247.htm
(advertisement) Home Specialties Resource Centers CME ... Patient Education Articles Images CME Patient Education Advanced Search Consumer Health Link to this site Back to: eMedicine Specialties Radiology Musculoskeletal
Enchondroma and Enchondromatosis
Last Updated: June 10, 2005 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 , Professor, Vice Chairman for Radiology Informatics, Department of Radiology, University of Washington 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

5. Enchondromatosis, Multiple
N O P Q R S T U V W X Y Z. Other characters. enchondromatosis, multiple, enchondromatosis, multiple, Fig. 1
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6. EMedicine - Maffucci Syndrome : Article By Raymond T Kuwahara, MD
enchondromatosis with multiple cavernous hemangiomas Gorham syndrome Multipleangiomas and endochondromas Ollier disease
http://www.emedicine.com/DERM/topic256.htm
(advertisement) Home Specialties Resource Centers CME ... Patient Education Articles Images CME Patient Education Advanced Search Consumer Health Link to this site Back to: eMedicine Specialties Dermatology Pediatric Diseases
Maffucci Syndrome
Last Updated: June 24, 2003 Rate this Article Email to a Colleague Synonyms and related keywords: dyschondrodysplasia with hemangiomas, enchondromatosis with multiple cavernous hemangiomas, multiple angiomas and endochondromas AUTHOR INFORMATION Section 1 of 9 Author Information Introduction Clinical Differentials ... Bibliography
Author: Raymond T Kuwahara, MD , Dermatologist, Private Practice Coauthor(s): Ron Rasberry, MD , Chief of Dermatology, Veterans Medical Center at Memphis; Associate Professor, Department of Dermatology, University of Tennessee at Memphis Raymond T Kuwahara, MD, is a member of the following medical societies: American Academy of Dermatology Editor(s): Jean Paul Ortonne, MD , Chair, Professor, Department of Dermatology, Hospital L'Archet, Nice University, France; Richard Vinson, MD

7. Virtual Children's Hospital Paediapaedia Enchondromatosis
enchondromatosis
http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

8. Enchondromatosis Information Diseases Database
enchondromatosis,Maffucci syndrome,Multiple enchondrosis,Ollier s disease, DiseaseDatabase Information.
http://www.diseasesdatabase.com/ddb9212.htm
Diseases Database Index Sponsors Contact ... Previous Page
Enchondromatosis information
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4 synonyms or equivalents were found. Enchondromatosis
aka/or
Maffucci syndrome
aka/or
Multiple enchondrosis
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Ollier's disease Enchondromatosis: Definition(s) via UMLS Code translations and terms via UMLS Enchondromatosis: specific web 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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Served 2005-09-09 04:09:50 View metadata Last major update 2005-09-03. The medical information here is presented for education, background reading and general interest. The Diseases Database is not a diagnostic or clinical decision-making tool. Please consult your own licensed physician regarding diagnosis and treatment of any medical condition!

9. EMedicine - Enchondroma And Enchondromatosis Article By Felix S
Enchondroma and enchondromatosis Enchondromas are benign cartilaginous neoplasms in bone. The primary significance of enchondroma is related to its
http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

10. Enchondromatosis Definition - Medical Dictionary Definitions Of Popular Medical
Online Medical Dictionary and glossary with medical definitions.
http://www.medterms.com/script/main/art.asp?articlekey=20861

11. EMedicine - Enchondroma And Enchondromatosis Article Excerpt By
Enchondroma and enchondromatosis Enchondromas are benign cartilaginous neoplasms in bone. The primary significance of enchondroma is related to its
http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

12. Enchondromatosis
enchondromatosis. Printable version. osteochondromatosis, dyschondroplasia,Ollier s disease. Synopsis. cartilagenous tumours (bone, trachea)
http://www.humpath.com/article.php3?id_article=351

13. Uhrad.com Pediatric Imaging Teaching Files
Case Fifty Five Multiple enchondromatosis. Click on Images for Enlarged View Clinical History Trauma to second right digit.
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14. Core Curriculum - POSNA
Ollier s disease is an uncommon condition of multiple enchondromatosis. The incidence of malignant differentiation of multiple enchondromatosis is high,
http://www.posna.org/index?service=page/coreCurriculum&article=olliersDisease.ht

15. ENCHONDROMATOSIS-VERTEBRAL ANOMALIES
Features Listed For enchondromatosisVERTEBRAL ANOMALIES McKusick Asymmetric legs/hindlimbs Cutis laxa Enchondromata Enlarged joints
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16. AAMED: American Association Of Multiple Enchondroma Diseases - Living With Multi
Living with Multiple enchondromatosis a glimpse into the lives of individualsand families living with a rare bone disease multiple enchondromatosis.
http://www.aamed.net/modules.php?name=News&file=article&sid=1

17. OLLIER ENCHONDROMATOSIS
Features Listed For OLLIER enchondromatosis McKusick 166000. Enchondromata Lytic lesions of bone Macrodactyly Multiple fractures
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18. AAMED: American Association Of Multiple Enchondroma Diseases
enchondromatosis of the hand in the context of the Ollier s disease is an infrequent Vertebral involvement in multiple enchondromatosis is very rare.
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.
This site contains general information only and does not constitute specific advice. AAMED

19. Epiphyseal-Metaphyseal Enchondromatosis. A New Clinical Entity
EpiphysealMetaphyseal enchondromatosis. A New Clinical Entity* PETER G. GABOS, M.D. and J. RICHARD BOWEN, M.D. , WILMINGTON, DELAWARE
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20. Uhrad.com Pediatric Imaging Teaching Files
Diagnosis Multiple enchondromatosis. Discussion enchondromatosis (Ollier sDisease) is a non hereditary disorder of enchondral ossification with lesions
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.

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