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
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.
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
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.
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: 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
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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/
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.
Extractions: *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
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
Extractions: *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
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
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
Extractions: *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 (
Multiple Enchondromatosis For Medical Professionals only. Multiple enchondromatosis . see enchondromatosis multiple http://www.amersham-health.com/medcyclopaedia/Volume%20VII/MULTIPLE%20ENCHONDROM
ENCHONDROMATOSIS, GENERALISED Features Listed For enchondromatosis, GENERALISED.McKusick 166000. Chromosome(s) http://www.hgmp.mrc.ac.uk/dhmhd-bin/hum-look-up?540
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
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
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
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
