Dents in the skull aren't very common, but they are understandably very distressing. When you see them, it's natural to wonder what in the world could have happened. Here are the most common causes:
- A dent or collapse of the skull can be caused by traumatic injury resulting in a depressed skull fracture, pushing fragments of bone against the brain. This is the most common cause of the problem.
- Skull deformities can be caused by exposure to massive amounts of vitamin A before birth, although this complication is extremely rare.
- And skull indentations appear in a relatively rare condition called Gorham's disease.
There are a number of synonyms for this relatively rare condition, including:
- Disappearing Bone Disease
- Gorham’s Syndrome
- Gorham-Stout Syndrome
- Idiopathic Massive Osteolysis
- Massive Gorham Osteolysis
- Massive Osteolysis
- Morbus Gorham-Stout Disease
- Phantom Bone Disease
- Progressive Massive Osteolysis
- Vanishing Bone Disease
There are several things Gorham's disease isn't. It isn't cancer. It isn't an infection. It isn't the result of an injury.
Gorham's disease occurs when blood vessels grow by dissolving bone. You may have seen a purple birthmark called a hemangioma. In Gorham's disease, something similar happens in bone. Capillaries (the smallest blood vessels) start growing in bone. As you might imagine, a capillary inside a bone doesn't have a lot of room for expansion to accommodate changes in blood flow. As a result oxygen levels in these capillaries tend to be low. When oxygen levels are low, pH is low, too, and enzymes that dissolve the protein component of bone, acid phosphatase and leucine aminopeptidase, become more active. As the bone loosens, tiny microfractures trigger a process called granulation that heals the blood vessels before it heals the bone, causing bone destruction to accelerate. This isn't what happens in osteoporosis, although some of the treatments for this condition are also used to treat osteoporosis.
A capillary may be just 8 microns (1/3000 of an inch) wide. The damage done by a single capillary is minimal. But when dozens and then hundreds and thousands of capillaries destroy bone, the result can be a dent. Actually, the symptoms of the disease appear before that point.
- The first sign of this vanishing bone disease can be a dull ache. It's a "skull ache" rather than a headache. It feels as if you've been hit in the head, even though you haven't.
- As the bone destruction progresses, there can be unexplained weakness. This isn't due to damage to the brain. It's due to changes in the calcium content of the blood.
- Finally, the skull can fracture "on its own." Fractures simply appear without injury to the skull.
This is a condition that almost always starts before the age of 40, usually striking teenagers. It's not hereditary. It's not something your doctor can detect with a laboratory test. It's almost never diagnosed before it causes serious damage to bone, and it's diagnosed by the damage to the bone.
If there's anything good about Gorham's disease, it is that the condition is usually self-limiting. After years and years, it just stops, although the skull continues to be deformed unless the damage can be surgically repaired. Gorham's disease doesn't just affect the skull. It's actually more common in the shoulders, pelvis, and ribs. When the destruction of a bone places pressure on an internal organ, death may result. So what can your doctor do? There are two common interventions:
- Surgery to remove the affected bone, and
- Relatively low-dose radiation to stop the growth of blood vessels in affected bone.
Both treatments, of course, can be complicated. Unfortunately, there isn't a natural, non-surgical, no-radiation approach to dealing with the disease. However, with treatment and reconstructive surgery, most people who have this condition can live relatively normal lives.
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