| 词条 | Ollier disease | |||||
| 释义 |
| name = Ollier disease | image = Ollier radio2.jpg | caption = X-ray image showing enchondromas localized in the lower part of the radius of a 7-year-old girl with Ollier disease. | | pronounce = | field = | synonyms = | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }}Ollier disease is a rare nonhereditary sporadic disorder where intraosseous benign cartilaginous tumors (enchondroma) develop close to growth plate cartilage. Prevalence is estimated at around 1 in 100,000.[1] Clinical featuresNominally, the disease consists of multiple enchondromas which usually develop in childhood. The growth of these enchondromas usually stops after skeletal maturation.[2] The affected extremity is shortened (asymmetric dwarfism) and sometimes bowed due to epiphyseal fusion anomalies. Persons with Ollier disease are prone to breaking bones and normally have swollen, aching limbs. Associated conditionsOllier disease carries a high risk of skeletal, visceral and brain malignancy which occurs in approximately 25% of patients. Juvenile granulosa cell tumour has been associated with the disease.[3] The incidence of secondary chondrosarcoma in Ollier disease is not known, but may be as high as 25%, pelvis and shoulder girdle being the commonest locations.[4] A related disorder called Maffucci syndrome named after Angelo Maffucci is characterized by enchondromas associated with multiple hemangiomas which usually occur in the hands and feet. Maffucci syndrome carries a higher risk for cancer. Cause{{Empty section|date=August 2017}}DiagnosisOn radiographs, streaks of low density are seen projecting through the diaphyses into the epiphyses of the long bones, due to ectopic cartilage deposits. With age, the cartilage may calcify in the typical "snowflake" pattern. TreatmentThe deformities are managed surgically to preserve the function of the limb.[4] EpidemiologyOne person in every 100,000 is affected. Ollier disease is not normally diagnosed until toddler years because it is not very visible. EponymsThe disorder is named after French surgeon Louis Léopold Ollier.[5] Additional imagesSee also
References1. ^{{cite journal |vauthors=Silve C, Jüppner H |title=Ollier disease |journal=Orphanet J Rare Dis |volume=1 |issue= |pages=37 |year=2006 |pmid=16995932 |pmc=1592482 |doi=10.1186/1750-1172-1-37 |url=http://www.ojrd.com/content/1//37}} 2. ^{{cite book|title=Turek's orthopaedics principles and their application|year=2005|publisher=Lippincott Williams & Wilkins|location=Philadelphia|isbn=9780781742986|pages=438|edition=6th}} 3. ^{{cite book|author=Stephen C. Rubin|title=Ovarian cancer|year=2001|publisher=Lippincott Williams and Wilkins|location=Philadelphia [u.a.]|isbn=9780781724081|edition=2nd}} 4. ^1 {{cite book|title=Lovell and Winter's pediatric orthopaedics.|year=1990|publisher=Lippincott|location=Philadelphia|isbn=9780397509140|edition=3rd}} 5. ^{{WhoNamedIt|synd|1813}} External links{{Medical resources| DiseasesDB = 9212 | ICD10 = {{ICD10|Q|78|4|q|65}} | ICD9 = {{ICD9|756.4}} | ICDO = | OMIM = 166000 | MedlinePlus = | eMedicineSubj = radio | eMedicineTopic = 247 | MeshID = D004687 }}{{Osteochondrodysplasia}} 3 : Skeletal disorders|Genetic disorders with OMIM but no gene|Rare diseases |
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