Short answer · Medically reviewed summary · Last updated: 2026-05-08
Cleidocranial dysplasia is identified in medical coding systems by the ICD-10 code Q74.0 (Other specified congenital malformations of upper limb, including shoulder girdle) and the ICD-9 code 755.59. These codes are essential for healthcare providers to accurately document and process insurance claims for patients managing the skeletal and dental manifestations of this condition. What is the clinical nature of Cleidocranial Dysplasia? Cleidocranial dysplasia is a rare autosomal dominant skeletal disorder characterized primarily by delayed closure of the fontanelles, hypoplastic or absent clavicles, and significant dental anomalies.
ICD10 code of Cleidocranial Dysplasia and ICD9 code
ICD-10 and ICD-9 codes for Cleidocranial Dysplasia, with classification details for clinicians, coders and patients.
Cleidocranial dysplasia is identified in medical coding systems by the ICD-10 code Q74.0 (Other specified congenital malformations of upper limb, including shoulder girdle) and the ICD-9 code 755.59. These codes are essential for healthcare providers to accurately document and process insurance claims for patients managing the skeletal and dental manifestations of this condition.
What is the clinical nature of Cleidocranial Dysplasia?
Cleidocranial dysplasia is a rare autosomal dominant skeletal disorder characterized primarily by delayed closure of the fontanelles, hypoplastic or absent clavicles, and significant dental anomalies. Because Cleidocranial dysplasia affects bone development, patients often experience supernumerary (extra) teeth and delayed eruption of permanent teeth, which requires specialized long-term dental management. Currently, 89 people with Cleidocranial dysplasia have joined the DiseaseMaps community to share their experiences and navigate these complex diagnostic codes.
How is Cleidocranial Dysplasia diagnosed and coded?
Diagnosis of Cleidocranial dysplasia typically involves a combination of clinical evaluation and radiographic assessment. While ICD-10 code Q74.0 is the primary classification, clinicians may also utilize supplementary codes to describe specific skeletal or dental symptoms. Accurate coding for Cleidocranial dysplasia is critical for ensuring that patients receive appropriate coverage for multidisciplinary care, including orthopedics, oral surgery, and genetics.
What are the key clinical features of Cleidocranial Dysplasia?
Patients with Cleidocranial dysplasia often present with a distinct set of physical characteristics that guide the diagnostic process:
- Hypoplasia or total absence of the clavicles (collarbones).
- Persistent open fontanelles and delayed ossification of the skull.
- Multiple supernumerary teeth and malocclusion.
- Short stature and specific pelvic/hip malformations.
- Increased risk of developing osteopenia or osteoporosis.
Next steps
- Consult with a clinical geneticist to confirm the diagnosis through molecular testing of the RUNX2 gene.
- Connect with the DiseaseMaps community to share insights with others living with Cleidocranial dysplasia.
- Schedule regular evaluations with a specialized pediatric dentist or orthodontist familiar with the dental complications of Cleidocranial dysplasia.
Medical disclaimer: This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment; always seek the advice of your physician regarding a medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Cleidocranial dysplasia.
- Orphanet: Cleidocranial dysplasia (ORPHA:206).
- OMIM (Online Mendelian Inheritance in Man): Entry #119600 (Cleidocranial Dysplasia).
- ICD-10 Data: Q74.0 - Other specified congenital malformations of upper limb.
