Gardner’s syndrome is a rare genetic disorder and is an unknown term among the population.
Gardner’s syndrome is an inherited disorder caused by a defect in a gene that regulates protein production. This gene is called Adenomatous Polyposis Coli.
People with Gardner’s syndrome develop colorectal cancer at a young age due to disordered multiplication of cells caused by the defective gene.
Some of the oral effects of Gardner’s syndrome include the development of supernumerary teeth, osteomas, and odontomas.
The supernumerary teeth are often called Gardner’s teeth and are the key markers to lead to a diagnosis of Gardner’s syndrome.
Gardner syndrome often goes unnoticed. Dental implications may sometimes be the sole factors leading to a diagnosis of Gardner’s syndrome.
A dentist’s knowledge and experience help him to effectively observe the signs of such disorders and detect its presence.
Diagnosis of Gardner syndrome may require several dental x-rays, clinical examinations, confirmation of family history, and a blood test for evidence of the APC gene.
This article will tell you more about how Gardner’s syndrome is clinically diagnosed by a dental professional.
What is Gardner’s syndrome?
Gardner syndrome is an autosomal dominant subtype of familial adenomatous polyposis. It is a rare genetic disorder of the colon.
This disorder is named after the professor who discovered it; Eldon Gardner in 1951.
Typically, the chances of inheritance are 50% among kids who have at least one parent suffering from Gardner’s syndrome.
Gardner syndrome is chiefly characterized by multiple polyp formations in the colon with the development of extracolonic tumors.
The extracolonic tumors include thyroid cancer, fibroma, and epidermoid cysts. (1)
Eye lesions on the retina are also one of the markers of Gardner syndrome. Additionally, Gardner syndrome also has some dental implications that may include –
- Multiple supernumerary teeth also termed as Gardner’s syndrome
What causes Gardner’s syndrome?
As mentioned above, Gardner’s syndrome is a genetic disorder caused by a defect in a protein regulating gene called Adenomatous Polyposis Coli (APC). (2)
This gene regulates cell growth and prevents the cells from dividing too fast or in a disorderly manner. Defect in the function of this gene causes abnormal tissue growth.
- Also read: 4 Most Unusual and Rare Dental Conditions
How is it diagnosed?
Routine dental check-ups and screening at a young age can unfold certain tell-tale signs of Gardner’s syndrome.
Usually, clinical examination of the mouth and dental x-rays, including panoramic x-rays, help to identify the significant oral implications like multiple supernumerary teeth, bony tumors of the jaw that may either be osteomas or odontomas.
Other dental abnormalities may include –
- Impacted teeth
- Poor oral hygiene
- Tooth decay
- Fused molars
- Bony outgrowths
If Gardner’s syndrome is expected, confirmatory family history, blood test and additional x-rays of the mandible along with long bones of the body are taken.
Additionally, monitoring of skin, eyes, and colon is done by a medical specialist to screen for polyps and malignancies.
Studies have shown that about 35 – 70% of people with Gardner’s syndrome have dental abnormalities.
While 68-82% of the people suffer from osteomas, bony outgrowths of the jaw and odontomas. (3)
What is the treatment of Gardner’s syndrome?
Treatment of Gardner’s syndrome is mainly focused on eliminating colon cancer. Medications can help to limit the growth of the colon polyps. These medications may include –
- Nonsteroidal anti-inflammatory drugs (NSAIDS)
- Celecoxib – a COX2 inhibitor
Close monitoring of colon polyps is done using lower gastrointestinal endoscopy to check their malignancy. Once identified, the polyps are removed to prevent the spread of colonic cancer.
Colonoscopies can be performed as early as 10 – 12 years of age and continued every two to three years.
At 25 years of age, upper endoscopy may be performed, which will continue every one to three years to monitor any recurrence of colonic polyps.
Simultaneously, dental abnormalities like supernumerary teeth are extracted. Bony tumors are treated symptomatically.
Lifestyle modification, including proper nutritious diet, exercise, and stress reduction therapy, is advised to control the medical condition.
Follow up for dental as well as medical appointments is mandatory to prevent the progression of Gardner’s syndrome.
What is the outlook of Gardner’s syndrome?
The outlook of Gardner’s syndrome depends on the severity of oral and medical symptoms. Prevention of Gardner’s syndrome is complicated.
Mutation of the adenomatous polyposis coli gene highly increases the risk of developing colonic cancer at a very young age. (4)
Genetic testing can determine the presence of gene mutation.
Take away message
Gardner syndrome is one of the rarest disorders found in dentistry. It is unheard and not popular among the population.
The sole cause for Gardner syndrome is a gene mutation in the Adenomatous polyposis coli gene. This gene primarily regulates cell and protein growth in the body.
Defect in this gene leads to abnormal and disordered growth of cells, especially in the colon leading to the formation of multiple polyps that may convert into malignant tumors.
Gardner syndrome often goes unnoticed. Dental abnormalities may be the first sign of diagnosing Gardner syndrome.
Typical dental abnormalities for Gardner syndrome include the presence of supernumerary teeth, multiple bony tumors like osteoma and odontomas in the lower jaw.
A family history of Gardner syndrome, multiple dental and bone scans, blood tests, and genetic testing are some of the diagnostic aids used to confirm Gardner syndrome.
The prevalence of Gardner syndrome is 50% in a child who has at least one parent positive for this disorder.
Therefore, early dental visits are of utmost importance to detect the presence of this disorder for better prognosis.
Now that you know about Gardner syndrome be aware of its symptoms.
Ask the dentist to check for any dental abnormalities that may indicate this disorder and take the necessary treatment.