$Unique_ID{BRK04062}
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$Title{Ollier Disease}
$Subject{Ollier Disease Multiple Cartilaginous Enchondroses Multiple
Enchondromatosis Ollier Osteochondromatosis Unilateral Chondromatosis}
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Copyright (C) 1987, 1989 National Organization for Rare Disorders, Inc.

337:
Ollier Disease

** IMPORTANT **
It is possible the main title of the article (Ollier Disease) is not the
name you expected.  Please check the SYNONYMS listing to find the alternate
names and disorder subdivisions covered by this article.

Synonyms

     Multiple, Cartilaginous Enchondroses
     Multiple Enchondromatosis
     Ollier Osteochondromatosis
     Unilateral Chondromatosis

General Discussion

** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.


Ollier Disease is a rare abnormal development of the bones (skeletal
dysplasia) usually beginning in childhood.  The disease affects bones and
cartilage in joints of the arms and legs.  Dwarfism is possible if both sides
of the body are affected.  Pain is rare in this disease, occurring usually
only in case of a fracture.

Symptoms

Ollier Disease begins gradually, usually during childhood, and is marked by
possible shortening of the arms and/or legs.  Limping may result from a
shortened leg.  The head and upper body will be normal.  Joint deformities
may occur in wrists and/or ankles.  A dislocation of the elbow or other
joints may occur, possibly causing fractures.  This disease may occasionally
be associated with some forms of cancer.

The abnormal growth of bone end/cartilage areas ceases in time, and
calcification may begin after puberty.  The bone abnormalities may be in
evidence throughout life.

Causes

The origin of Ollier Disease is unknown at this time.  Overgrowth of
cartilage cells in certain skeletal and/or joint areas may cause thinning of
the external layer (cortex) and distortion of growth in the spongy growth
area (metaphysis) of long bones.

Affected Population

Ollier Disease usually begins in childhood, and may be in evidence later in
life.  This disease may affect males and females in equal numbers, and can
occur worldwide.

Related Disorders

Exostosis is a hereditary abnormality of bone end/cartilage (epiphyseal)
growth inherited as a dominant trait.  Although usually without apparent
symptoms, pain may be possible due to pressure from benign bone surface
growths (exostoses).  This is an extremely rare condition, occurring chiefly
among Micronesian Islanders.  It is most frequent and severe in males.
Symptoms may be similar to Ollier Disease, although areas of joint deformity
may differ.

Maffucci Syndrome is a hereditary genetic disease which may begin between
birth and puberty, and is marked by asymmetric distribution of lesions, and
abnormal growth of cartilage in one or more long bones.  Susceptibility to
fractures and skin manifestations are also characteristic of this type of
abnormal bone development (skeletal dysplasia).

Therapies:  Standard

Bone grafts have been of benefit to patients with Ollier Disease, as well as
surgical treatment of limb length.  Fractures usually heal without any
follow-up treatment.  Prosthetic replacement of the limb may be of benefit in
some extreme cases.

Therapies:  Investigational

Research into the causes, as well as orthopedic surgical treatment for Ollier
Disease is ongoing.  For more information, please see the resources section
in this report.

This disease entry is based upon medical information available through
December 1988.  Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate.  Please check with the agencies listed in the Resources section for
the most current information about this disorder.

Resources

For more information on Ollier Disease, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     (203) 746-6518

     Ollier Disease Support Group
     Bridge House
     45 Baring Road
     Beaconsfield, Bucks,
     England  HP9  2NF

     National Information Clearinghouse
     Box NDDIC
     Bethesda, MD  20892
     (301) 468-6344

References

TREATMENT OF MULTIPLE ENCHONDROMATOSIS (OLLIER'S DISEASE) OF THE HAND:  J.F.
Fatti, et. al.;  Orthopedics (April 1986:  issue 9(4)).  Pp.512-518.

OLLIER'S DISEASE. AN ASSESSMENT OF ANGULAR DEFORMITY, SHORTENING, AND
PATHOLOGICAL FRACTURE IN TWENTY-ONE PATIENTS:  F. Shapiro; J Bone Surg (Am)
(Jan. 1982: issue 64-A (1)).  Pp. 95-103.

MULTIPLE CHONDROSARCOMAS IN DYSCHONDROPLASIA (OLLIER'S DISEASE):  S.R.
Cannon, et. al.; Cancer (Feb. 15, 1985:  issue 55(4) ).  Pp. 836-840.