Ollier disease

Ollier disease
Classification and external resources
ICD-10 Q78.4
ICD-9 756.4
OMIM 166000
DiseasesDB 9212
eMedicine radio/247
MeSH D004687
X-ray image showing enchondromas localized in the lower part of the radius of a 7-year-old girl with Ollier disease.
Enchondromas localized in the upper part of the humerus of the same patient

Ollier disease is a rare nonhereditary sporadic disorder where intraosseous benign cartilaginous tumors develop close to growth plate cartilage. Prevalence is estimated at around 1 in 100,000.[1]

Contents

Presentation

Nominally, the disease consists of multiple enchondromas which usually develop in childhood. On 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. 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. One person in every 100,000 is affected. Ollier Disease is not normally diagnosed until toddler years because it is not very visible.

Associated conditions

Ollier Disease carries a high risk of skeletal, visceral and brain malignancy which occurs in approximately 25% of patients. Regular systemic screening is recommended.[2]

Eponyms

The disorder is named after French surgeon Louis Léopold Ollier (1830–1900).[3]

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.

References

  1. ^ Silve C, Jüppner H (2006). "Ollier disease". Orphanet J Rare Dis 1: 37. doi:10.1186/1750-1172-1-37. PMC 1592482. PMID 16995932. http://www.ojrd.com/content/1//37. 
  2. ^ Walid MS, Troup EC (2008). "Cerebellar anaplastic astrocytoma in a teenager with Ollier Disease". J Neurooncol 89 (1): 59–62. doi:10.1007/s11060-008-9583-8. PMID 18414790. 
  3. ^ synd/1813 at Who Named It?

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Look at other dictionaries:

  • Ollier disease — A condition characterized by multiple enchondromas benign masses of cartilage growing within bones. An enchondroma can deform and shorten a limb and predispose to a fracture at the site. The disorder can be caused by a mutation in the gene for… …   Medical dictionary

  • Ollier disease law layer — Ol·lier disease, law, layer (o le aґ) [Lйopold Louis Xavier Edouard Ollier, French surgeon, 1830–1900] see enchondromatosis, and see under law and layer …   Medical dictionary

  • Ollier's disease — see dyschondroplasia L. L. X. E. Ollier (1830 1900), French surgeon …   Medical dictionary

  • Disease — Illness or sickness often characterized by typical patient problems (symptoms) and physical findings (signs). Disruption sequence: The events that occur when a fetus that is developing normally is subjected to a destructive agent such as the… …   Medical dictionary

  • Ollier — Louis X.E.L., French surgeon, 1830–1900. See O. graft, O. disease, O. theory, O. Thiersch graft …   Medical dictionary

  • Ollier's disease — dyschondroplasia …   The new mediacal dictionary

  • Louis Léopold Ollier — Louis Léopold Ollier, full name Louis Xavier Édouard Léopold Ollier (December 2, 1830 November 26, 1900) was a French surgeon whose father and grandfather were also physicians. In 1857 he earned his medical degree from the University of Paris. In …   Wikipedia

  • Maffucci syndrome — Classification and external resources ICD 10 Q78.4 ICD 9 756.4 …   Wikipedia

  • Enchondromatose — Classification et ressources externes Exemple d expression de la maladie d Ollier, chez une fillette de 7 ans (radius) CIM 10 …   Wikipédia en Français

  • Enchondromatosis — A condition characterized by multiple enchondromas benign masses of cartilage growing within bones. The enchondromas can deform and shorten a limb and predispose to a fracture. The condition can be caused by a mutation in the gene for the… …   Medical dictionary


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