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$Unique_ID{BRK03781}
$Pretitle{}
$Title{Gorham's Disease}
$Subject{Gorham's Disease Disappearing Bone Disease Gorham's Syndrome
Gorham-Stout Syndrome Idiopathic Massive Osteolysis Massive Gorham Osteolysis
Massive Osteolysis Morbus Gorham-Stout Disease Progressive Massive Osteolysis
Vanishing Bone Disease Osteonecrosis Gaucher Disease Kienboeck Disease
Legg-Calve-Perthes Syndrome}
$Volume{}
$Log{}
Copyright (C) 1991 National Organization for Rare Disorders, Inc.
832:
Gorham's Disease
** IMPORTANT **
It is possible that the main title of the article (Gorham's Disease) is
not the name you expected. Please check the SYNONYM listing to find the
alternate names and disorder subdivisions covered by this article.
Synonyms
Disappearing Bone Disease
Gorham's Syndrome
Gorham-Stout Syndrome
Idiopathic Massive Osteolysis
Massive Gorham Osteolysis
Massive Osteolysis
Morbus Gorham-Stout Disease
Progressive Massive Osteolysis
Vanishing Bone Disease
Information on the following disorders can be found in the Related
Disorders section of this report:
Osteonecrosis
Gaucher Disease
Kienboeck Disease
Legg-Calve-Perthes Syndrome
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.
Gorham's Disease is an extremely rare bone disorder characterized by bone
loss often associated with swelling or abnormal blood vessel growth
(angiomatous proliferation). Bone loss can occur in just one bone or spread
to soft tissue and adjacent bone.
Symptoms
Gorham's Disease is an extremely rare bone disorder. Normally bones
replenish themselves through a cycle of bone dissolution and regrowth. In
people with Gorham Disease bone loss occurs and progresses in certain areas
of the body but no new bone growth takes place. This may occur in just one
bone or may spread to adjacent areas of the affected bone.
Fibrous tissue may appear in areas of bone loss. If fractures occur,
which is common in this disorder, the disease may progress more quickly.
Angiomas often occur in Gorham's Disease in conjunction with bone loss. An
angioma is abnormal growth of tissue formed by small blood or lymphatic
vessels. Angiomas cause swelling.
Bone loss may occur in such places as the hand, arm, shoulder, ribs, part
of the pelvis (hemipelvis), thighbone (femur), or jaw. When the lower jaw,
upper jaw, tooth sockets, or other bones in the face, neck or head are
affected possible symptoms may include pain, loose teeth, fractures, facial
deformity, and/or recurrent meningitis. (For more information choose
"meningitis" as your search term in the Rare Disease Database).
In some patients a fluid build-up (pleural effusion) in the space between
the membrane that surround each lung and line the chest cavity may occur in
conjunction with Gorham's Disease.
Causes
The exact cause of Gorham's Disease is unknown. Scientists are conducting
research to try and identify what causes this disorder.
Affected Population
Gorham's Disease is an extremely rare bone disorder. It affects males
slightly more often than females, and occurs in all age groups.
Related Disorders
Symptoms of the following disorders can be similar to those of Gorham's
Disease. Comparisons may be useful for a differential diagnosis:
Osteonecrosis is the destruction of a bone (necrosis) due to an
inadequate circulation of blood. It most commonly affects the joints and
bones of the hips, knees or shoulder. It often occurs as a result of bone
injuries or in conjunction with other diseases and conditions. (For more
information on this disorder, choose "Osteonecrosis" as your search term in
the Rare Disease Database).
Gaucher's Disease is an inherited disease of lipid metabolism caused by
the failure to produce the enzyme glucocerebrosidase. There are three types
of Gaucher's Disease - Types I, II, and III. In Types I and III bone
deterioration is the major problem and can affect any part of the body.
Other symptoms of Types I and III may include an enlarged spleen or liver, a
low blood count, bone pain, gastric problems or delayed growth. In Type III
seizures, mental retardation, abnormal eye movement, or jerking motions of
the limbs, head, and upper body may also occur. (For more information on
this disorder, choose "Gaucher" as your search term in the Rare Disease
Database).
Kienboeck Disease is an acquired bone disorder of the wrist caused by
inflammation or injury. Degenerative changes of the lunate bone occur such
as softening, deterioration, fragmentation or compression. These changes may
produce pain, swelling, tenderness, thickening and/or stiffness in the
overlying tissues of the wrist. The range of motion may become restricted.
Healing occurs through formation of new bone in some cases. (For more
information on this disorder, choose "Kienboeck" as your search term in the
Rare Disease Database).
Legg-Calve-Perthes Syndrome is a rare bone disorder affecting the hip
joint. Abnormalities in bone growth early in life may result in permanent
deformity of the hip joint several years later. The bone may become shorter
than normal, causing a noticeable limp. (For more information on this
disorder, choose "Legg-Calve-Perthes" as your search term in the Rare Disease
Database).
Therapies: Standard
Testing for Gorham's Disease includes imaging techniques such as X-rays or
computerized tomographic (CT) scans. Diagnosis can be made by biopsy.
Gorham's Disease may be treated with radiation therapy, surgery, and/or bone
grafting. Drugs may also be prescribed.
Fluid build-up (pleural effusion) in the membrane surrounding each lung
and lining the chest cavity may be treated by draining the fluid if
necessary.
Other treatment is symptomatic and supportive.
Therapies: Investigational
Scientists are trying to find the cause of Gorham's Disease by studying the
cells and enzymes which may be related to it. Research is ongoing.
This disease entry is based upon medical information available through
June 1991. 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 Gorham's Disease, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
The National Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Information Clearinghouse
Box AMS
Bethesda, MD 20892
(301) 495-4484
References
CECIL TEXTBOOK OF MEDICINE, 18th Ed.: James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1988. Pp. 1474-1475.
A 20-YEAR FOLLOW-UP STUDY OF A CASE OF SURGICALLY TREATED MASSIVE
OSTEOLYSIS. S. Turra, et al.; Clin Orthop (Jan 1990; issue 250). Pp. 297-
302.
CYTOCHEMICAL LOCALIZATION OF ALKALINE AND ACID PHOSPHATASE IN HUMAN
VANISHING BONE DISEASE. G. R. Dickson, et al.; Histochemistry (1987; issue
87 (6)). Pp. 569-572.
"DISAPPEARING BONE DISEASE" IN THE HAND. R. S. Carneiro and V. Steglich;
J Hand Surg [Am] (Jul 1987; issue 12 (4)). Pp. 629-634.
GORHAM'S DISEASE AFFECTING THE MAXILLOFACIAL SKELETON. Y. Anavi, et al.;
Head Neck (Nov-Dec 1989; issue 11 (6)). Pp. 550-557.
GORHAM'S SYNDROME: A CASE REPORT AND REVIEW OF THE LITERATURE. N. D.
Choma, et al.; Am J Med (Dec 1987; issue 83 (6)). Pp. 1151-1156.
MASSIVE GORHAM OSTEOLYSIS OF THE RIGHT HEMIPELVIS COMPLICATED BY
CHYLOTHORAX: REPORT OF A CASE IN A 9-YEAR OLD BOY SUCCESSFULLY TREATED BY
PLEURODESIS. N. Hejgaard and P. R. Olsen; J Pediatr Orthop (Jan-Feb 1987;
issue 7 (1)). Pp. 96-99.
MASSIVE OSTEOLYSIS OF THE FEMUR (GORHAM'S DISEASE): A CASE REPORT AND
REVIEW OF THE LITERATURE. A. A. Mendez, et al.; J Pediatr Orthop (Sep-Oct
1989; issue 9 (5)). Pp. 604-608.
RADIOTHERAPY OF MORBUS GORHAM-STOUT: THE BIOLOGICAL VALUE OF LOW
IRRADIATION DOSE. L. Handl-Zeller and G. Hohenberg; Br J Radiol (Mar 1990;
issue 63 (747)). Pp. 206-208.