Vanishing Bone Disease

Gorham-Stout disease (GSD), which is also known as vanishing bone disease, disappearing bone disease, massive osteolysis, and more than a half-dozen other terms in the medical literature, is a rare bone disorder characterized by progressive bone loss (osteolysis) and the overgrowth (proliferation) of lymphatic vessels. Affected individuals experience progressive destruction and resorption of bone. Multiple bones may become involved. Areas commonly affected by GSD include the ribs, spine, pelvis, skull, collarbone (clavicle), and jaw. Pain and swelling in the affected area may occur. Bones affected by GSD are prone to reduced bone mass (osteopenia) and fracture. The severity of GSD can vary from one person to another and the disorder can potentially cause disfigurement and functional disability of affected areas.

Causes
The cause of Gorham-Stout is unknown. There is no evidence that the disease is hereditary or caused by environmental factors. However, active research is underway at Boston Children’s and other institutions to possibly identify a mutation that could cause the lymphatic and bone disorder.

What are the symptoms of Gorham-Stout?
Gorham-Stout may affect multiple bones, but in most cases it is a regional disease, meaning it stays in one region of the body. For example, your child may have Gorham-Stout only in the bones of his or her shoulder and arm, but nowhere else in the body.

Bones commonly affected by Gorham-Stout include:
• ribs
• spine
• pelvis
• skull
• collarbone (clavicle)
• shoulder
• jaws

The signs and symptoms of Gorham-Stout can vary widely and can range from mild to severe, depending on the location of the disease and the bones and soft tissues it affects.

A common early sign of the disease is pain and swelling near the affected region without any clear cause. Other children may experience no symptoms until suffering a spontaneous fracture after minor trauma to the bone. A common misbelief is that Gorham-Stout disease is caused by fractures, but these are simply symptoms of the progressive bone loss.
Other symptoms that vary by location include:

Gorham-Stout in the ribs or thoracic vertebrae:
• breathing difficulty
• chest pain
• weight loss
• chylothorax, a condition where a lymphatic fluid called chyle leaks into and accumulates in the chest,  leading to infection or respiratory distress

Gorham-Stout in the spine and skull:
• neurological complications
• acute spinal pain
• paralysis (depending on which vertebrae it affects and how far the disease has progressed)
• occasionally, spinal fluid leak

Gorham-Stout in the jaw (maxillofacial bones):
• jaw pain
• loose teeth
• fractures and facial deformity

Treatment
There is no specific therapy for people with Gorham’s disease. Certain treatments may be effective in some, but not others. Several different methods are often used before finding one that is effective. In some cases, treatment may not be necessary.

Most people require intense treatment, especially if the disease has spread to other areas of the body or if there is extensive involvement in the spine and skull. Treatment options may include vitamin D and calcitonin supplements, radiation therapy, and/or surgery that may involve bone grafting. Some of the best outcomes reported have involved radiation therapy alone or in combination with surgery.Other treatments might include biphosphonates (such as pamidronate or zoledronic acid) and alpha-2b interferon. These treatments have led to improvement of symptoms in some cases. More research is necessary to determine the long-term safety and effectiveness of these therapies in people with Gorham’s disease.

By -Assistant Professor – Mrs Deepika
Department of Nursing
Magazine (YouthRainBow)- Uttaranchal (P.G.) College Of Bio-Medical Sciences & Hospital
Uttaranchal (P.G.) College Of Bio-Medical Sciences & Hospital
Uttaranchal College of Education
College Of Nursing UCBMSH
General Knowledge & Current Affairs

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