Man With Rare Disorder Undergoes Removal of 21 Baby Teeth – A Groundbreaking Cleidocranial Dysplasia Case
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Introduction: A Rare Genetic Disorder That Keeps Baby Teeth Into Adulthood
Most people lose their baby teeth by age 12 or 13, making room for permanent adult teeth. However, for one 28-year-old man from New York, those baby teeth never left. The patient was diagnosed with a rare genetic condition known as Cleidocranial Dysplasia (CCD), which affects the development of the bones, skull, and teeth. This unique case led oral surgeons at the University of Rochester Medical Center’s Eastman Institute for Oral Health to perform an extraordinary procedure—removing 21 retained baby teeth in a single treatment.
This groundbreaking surgery not only helped the patient regain function and confidence but also shined a spotlight on the power of modern dental science and compassionate care in treating rare conditions.
What Is Cleidocranial Dysplasia (CCD)? Causes and Symptoms Explained
Cleidocranial Dysplasia (CCD) is a rare, inherited bone disorder that affects approximately 1 in 1 million people worldwide. It is caused by mutations in the RUNX2 gene, which plays a critical role in bone and dental development. CCD follows an autosomal dominant inheritance pattern, meaning only one affected gene copy from a parent can cause the disorder.
Individuals with CCD may have underdeveloped or absent collarbones (allowing unusual shoulder movement), delayed closure of skull sutures, and numerous dental abnormalities such as:
- Delayed loss of primary (baby) teeth
- Delayed eruption of permanent teeth
- Multiple supernumerary (extra) teeth
- Malocclusion (misalignment) and crowding
The dental manifestations often lead to functional challenges in chewing, speech, and facial development, as well as significant psychological impacts due to aesthetic concerns.
Source: National Organization for Rare Disorders (NORD); Journal of Oral and Maxillofacial Surgery
The Complex Dental Surgery: Removal of 21 Retained Baby Teeth
The patient’s case was managed by a multidisciplinary team at the University of Rochester Medical Center’s Eastman Institute for Oral Health, involving oral surgeons, orthodontists, and prosthodontists. Over time, the team created a personalized treatment plan to safely remove 21 retained deciduous teeth—an exceptionally rare surgical scenario.
The surgical procedure involved careful planning through 3D imaging and digital modeling. Surgeons needed to account for unerupted permanent teeth and abnormal bone density typical in CCD patients. Each tooth was methodically extracted under general anesthesia with measures to minimize trauma and promote healing.
Key challenges included:
- Preserving bone integrity around unerupted permanent teeth
- Preventing nerve damage due to altered jaw anatomy
- Ensuring proper alignment for future orthodontic treatment
Following extraction, the patient underwent guided tissue healing and ongoing monitoring to assess eruption progress of remaining permanent teeth. The long-term plan includes orthodontic management and possible prosthetic rehabilitation to achieve optimal function and esthetics.
Source: Eastman Institute for Oral Health, University of Rochester Medical Center
Innovations in Craniofacial & Dental Treatment for CCD Patients
The field of craniofacial and dental surgery has seen remarkable progress, particularly for genetic bone disorders like CCD. Digital dentistry and advanced cone beam computed tomography (CBCT) have become essential tools for diagnosis and surgical mapping.
Key innovations in treatment include:
- 3D-guided surgical planning for precision tooth extractions
- Orthodontic traction to assist eruption of impacted permanent teeth
- Use of temporary anchorage devices (TADs) for controlled tooth movement
- Custom dental prosthetics to enhance oral function and facial symmetry
A multidisciplinary team approach—combining geneticists, orthodontists, oral surgeons, and restorative dentists—maximizes outcomes and ensures a holistic treatment plan tailored to each CCD patient.
Source: American Journal of Orthodontics and Dentofacial Orthopedics; Journal of Clinical Oral Science
Why Early Diagnosis of Cleidocranial Dysplasia Is Crucial
Early recognition of CCD plays a vital role in minimizing long-term complications and promoting healthy dental development. Because the condition can often mimic normal variations in delayed tooth eruption, vigilance among parents and dentists is key.
Signs to Watch for in Early Childhood:
- Persistent baby teeth beyond age 13
- Missing or delayed adult teeth on X-rays
- Wider skull sutures or underdeveloped collarbones
Genetic testing can confirm a CCD diagnosis through identification of RUNX2 gene mutations. Once confirmed, a coordinated long-term care plan—including routine imaging, orthodontic evaluations, and oral surgery when appropriate—can greatly improve quality of life.
Source: National Institutes of Health (NIH) Genetic and Rare Diseases Information Center
Raising Awareness and Hope for Individuals With Rare Disorders
This case from Rochester underscores the importance of increasing awareness about rare dental disorders and promoting collaboration across medical and dental specialties. Each case contributes to a deeper understanding of the condition and fuels advancements in both treatment and patient education.
Equally important is the emotional aspect of care. Patients living with conditions like CCD often face daily challenges—from appearance-related concerns to functional dental difficulties. As healthcare providers and community members, supporting these individuals through empathy, education, and resources can transform their experience.
Organizations such as the Cleidocranial Dysplasia Support Group and Little People of America provide vital peer networks, advocacy, and latest research updates. Continued awareness fosters not just medical progress, but also acceptance, empowerment, and hope.
Frequently Asked Questions (FAQ)
1. What causes Cleidocranial Dysplasia?
CCD is caused by mutations in the RUNX2 gene, which regulates bone and tooth formation. It may be inherited from an affected parent or occur as a new, spontaneous mutation.
2. Can adults with CCD still grow new teeth?
Adults with CCD often have unerupted permanent teeth that can sometimes be guided into position through surgical and orthodontic techniques, depending on the bone structure and individual case.
3. How common is it to retain baby teeth into adulthood?
Retention of baby teeth past adolescence is rare, affecting less than 2% of the population. However, in people with CCD, it is a hallmark characteristic due to delayed eruption patterns.
4. What treatment options are available for CCD?
Treatment involves coordinated care including oral surgery to remove baby teeth, orthodontics to guide permanent teeth, and restorative dentistry to enhance function and aesthetics.
5. How can genetic testing help with early diagnosis?
Genetic testing confirms CCD by identifying changes in the RUNX2 gene. Early testing allows families to plan multidisciplinary care and monitor dental and skeletal development proactively.
Conclusion: Progress in CCD Treatment and Future Perspectives
The removal of 21 retained baby teeth from this 28-year-old patient represents more than a surgical achievement—it symbolizes progress in rare disease management and compassionate dentistry. With each innovation, patients with Cleidocranial Dysplasia are gaining access to safer, more personalized care that restores health, confidence, and quality of life.
Every small step in awareness and prevention reflects the same principle that shapes great oral health: consistent habits and mindful improvements. Discover how small habits can create big life changes in The Habit Method.
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