What Exactly is Gorham’s Disease?
Gorham's Disease is an extremely rare bone disorder; there are few reported cases.
The
bone loss is often associated with swelling or abnormal blood vessel
growth (angiomatous proliferation). This can happen in just one bone or
spread via soft tissue and in to the adjacent bone.Although the disease
may strike any of the bones of the body, it is more often recognized
earlier when the bones at the top of the head (calvarium) and/or the
mandibles are involved.
Due to the rarity of the disease, it
often goes unrecognized. As a result of this and as a lack of agreement
on how best to treat Gorham’s Disease, treatment may often be delayed.
Generally bones replenish themselves naturally through a cycle of bone dissolution and re-growth. With people who suffer with Gorham’s Disease, bone loss occurs and progresses in certain areas of the body but the bone re-growth doesn’t take place.
This can occur in just one bone or may spread to adjacent areas of the affected bone.
Fibrous
tissue may appear in areas of bone loss. It is common in this disorder
for bones to fracture; this can speed up the spreading of the disease.
Angiomas often occur in Gorham's Disease in conjunction with bone loss.
An angioma is abnormal growth of tissue which is formed by small blood
or lymphatic vessels, this causes swelling.
Bone
loss may occur in places such as the hand, arm, shoulder, ribs, and
parts of the pelvis, thighbone, 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.
What is the cause of Gorham’s Disease?
The exact cause of Gorham's Disease is unknown. Doctors and Scientists are conducting research to try and identify what is the cause of the disorder.
Who does Gorham’s Disease Affect?
As previously stated Gorham's Disease really is an extremely, it is believed that there is currently only 200 reported cases 200 cases are reported in the medical literature. It affects males slightly more often than females, and can occur in all age groups.
Are there any related disorders?
The following disorders can produce symptoms similar to those of Gorham's Disease. These 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.
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.
Kienbock 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.
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.
How do you test for Gorham’s Disease?
Testing for Gorham's Disease includes imaging techniques such as X-rays and CT scans. Diagnosis can also be made by biopsy.
Gorham's Disease may be treated with radiation therapy, surgery, and/or bone grafting.
Drugs such as Interpheron Alfa 2B and Bisphosphornates may(should) 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.
Scientists are currently trying to find the cause of Gorham's Disease by studying the cells and enzymes which may be related to it. Research is ongoing.
Are there any Synonyms?
