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Cleidocranial Dysplasia: Treatment, Cost and Side Effects

Last Updated: Apr 25, 2024

What is Cleidocranial Dysplasia ?

Cleidocranial Dysplasia (CCD) also referred as cleidocranial dysplasia, is a birth defect that mostly affects the bones and teeth. In this due to absence or poor development of the collar bones the shoulders gets close together. The symptoms may include the abnormal forehead, height often shorter than average, wide set of eyes, abnormal teeth and flat nose. Apart from these symptoms the intelligence is typically seen normal. The initial diagnosis of this disease is often done by physical examination and X-rays of the head, chest and hands. And for detailed diagnosis genetic testing for mutation in the gene that causes the condition, RUNX2, is available but usually isn’t necessary for the initial diagnosis.

As this disease may appear at any part of the body so there is no specific treatment but according to symptoms of individuals the treatment is given accordingly. The orthodontic treatment in a patient with Cleidocranial Dysplasia includes removal of supernumerary teeth, surgical exposure of impacted teeth, and orthodontic treatment. In such patients bone density also recorded below normal, so calcium and vitamin D supplements are given to such patients and preventive treatments for osteoporosis may be started at a young age. If patient is having a frequent ear infection they may be advised for ear tube. Patients with several defects in the cranial vault which is the space in the skull occupied by the brain should be protected by helmets if undergoing any high risk activities. If any patient have various skeletal bone abnormalities then surgery may be required.

How is the treatment done?

The orthodontic treatment was initiated with the goals like- complete dental eruption through surgical exposure of impacted teeth, correction of the anterior crossable and an increase in vertical dimension. The treatment is performed in different steps. The treatment starts with an extraction of the deciduous and supernumerary teeth and surgical exposure of permanent teeth. Then orthodontic traction treatment is carried out with the help of powerful unity of anchorage.

If the abnormalities detected at the age of 5 then surgical corrections may be necessary to prevent any worsening of the deformity. In case if a pregnant woman detected with dysplasia, caesarian delivery is necessary in such cases. Skull defects can be corrected by craniofacial surgery. Orthopedic may advice an appropriate headgear in case of open fontanels for protection from injury. Coxa vara which is a deformity of the hip can be corrected by femoral osteotomies.

Other treatments with minor symptoms and which are not that routine disturbing can be treated with the calcium and vitamin D supplements. In patients with initial or minor symptoms the medication may help and routine check-ups are always advices for updated medical records.

Who is eligible for the treatment?(When is the treatment done ?)

A person who is having difficulties in the routine work due to the symptoms of Dysplasia is eligible for the treatment. Also the patients who have born with some deformities and want to have a treatment due to lack of many activities due to deformity are eligible and advised for the treatment. The people with calcium and vitamin D deficiency are also advised to have a check-up and take supplements as per the medical advisor consultation. A person with lateral skull radiograph showing open skull sutures, large fontanels, multiple wormian bones and underdeveloped Para nasal sinuses. In patients whose panoramic view of the jaws showing multiple unerupted supernumerary teeth, mimicking premolar, missing gonial angles, and underdeveloped maxillary sinuses. Also, patients with poor development of the clavicles and a bell-sharped rib cage can also be advised for the treatment. So, if any of the above reports have been recorded after thorough diagnosis then that candidate is eligible for the treatment.

Who is not eligible for the treatment?

Kids under age 5 years are not eligible for the treatment. But after that they can undergo for a proper treatment as per the symptoms. Also, any person who has not done thorough diagnosis from the authorized medical expert and don’t have relevant reports to cleidocranial dysplasia are not eligible for the treatment. If a person is undergoing some other treatment and consuming any inflammatory medicines then he must stop that treatment and medicines and can start with the treatment for dysplasia.

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Are there any Side Effects?

As everybody reacts differently for every treatment and in this the treatment itself will be different for each individual as per their symptoms. So the side-effects can’t be generalized. Medication may cause lightheadness or dizziness, increased or irregular heart rate. Some medication may have effects like constipation, nausea, rash, and headache. The side-effects can go away once you have completed the treatment. Not all side-effect are bad.

What are the post-treatment guidelines?

If a person has undergone the surgery to cure the dysplasia then that person needs to be under observation for at least 48 hrs. You may require a clinical monitoring and can be discharged once the doctor confirms. The medication related to the treatment post-surgery is required to take as per the schedule. Proper physical therapy should be taken and medical consultation guidelines should be followed.

How long does it take to recover?

Cleidocranial dysplasia has not a specific treatment. But as per the symptoms of the individual the person can be cured. Most of the dysplasia patients need dental care due to various dental abnormalities. And this treatment may go for several months. As this treatment will require many seating and as per the response from the patient the treatment time can be analyzed. It does not have any specific time to be predicted for this treatment.

What is the price of the treatment in India?

As this disease has no specific treatment, so any specific cost cannot be estimated. The result of the success ratio also varies case to case. The duration and sittings are also different as per individual. Hence any specific amount cannot be estimated. But still if you are going under surgical treatment the cost may range from Rs.30, 000/- to Rs.60, 000/-. The cost also varies as per the medical organization in which you are undergoing for the treatment.

Are the results of the treatment permanent?

The result for this treatment for the individuals has been seen permanent. But as this is a genetic condition it may be carried forwarded to next generation. Though this is the case it’s not confirmed that this will pass on to next generation in the same severity.

What are the alternatives to the treatment?

Alternative to dysplasia treatment is not yet considered as a proper treatment. If the bone density is too low and if surgery is avoidable then one can go for the medication treatment. And one can go for the painkillers to avoid the pain due to deformity of bones. But when pain is there at any body part it should be corrected with a proper treatment by medical expert consultation only. But in some cases surgery is important to get rid of the dysplasia. In case of dysplasia teeth alternative treatment is not yet available.

References

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Written ByDr. Sidharth Verma PDCC - Pain Management,MD - Anaesthesiology,DNB Anaesthesiology,MBBSOrthopaedics
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