13% of individuals develop a jaw cyst by the age of 10 years and 51% by the age of 20 years. The majority occur after the seventh year. The peak incidence is in the third decade.
Misshapen teeth, missing teeth and a susceptibility to caries are more common in individuals with Gorlin syndrome than in unaffected relatives.
Jaw cysts are thought to form from the rests of tissue close to the soft tissue from which teeth develop in early life. They are therefore to be found in those parts of the jaw which normally bear teeth. In Gorlin syndrome, the cysts are to be found in both jaws with equal frequency, a point of distinction from keratocysts found in non-Gorlin cysts, where the lower jaw predominates.
Treatment for the cysts is surgical removal. This is an operation which requires exposure of the lesion by making a hole in the jaw bone under anaesthetic, finding the lining of the cyst and removing this together with its cheese-like contents. This material is sent to a pathologist for examination under a microscope, to confirm the diagnosis.
Cysts which recur after such treatment, are removed as before but with a layer of bone at the margin of the cyst. This is to ablate any daughter cysts which may be forming in this region. Only in extreme cases is it advisable to resect a whole section of jaw bone (with a bone graft to replace the missing bone).
In the wisdom tooth area, a cyst may form around the crown of the unerupted tooth. The same change may occur around other buried teeth, especially around upper canine. Cysts which appear where a tooth has not developed, are also known as a primordial cyst.
There may be remarkably few symptoms until cysts reach a large size,. Presentation can be with swelling and/or pain of the jaw, pus discharging into the oral cavity or displaces, impacted or loose teeth.
Originally produced by Professor P. A. Farndon, Clinical Geneticist, Jim Costello (deceased) and Margaret Costello. We are reliant on a team of medical advisors for the clinical content of the website. We are grateful for their continuing support.
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