Short answer · Medically reviewed summary · Last updated: 2026-05-08
Kenny-Caffey Syndrome (KCS) does not have a unique, specific code in the ICD-10 or ICD-9 systems because it is a rare condition often classified under broader categories for skeletal dysplasias. Clinicians typically use the ICD-10 code Q78.8 (Other specified osteochondrodysplasias) or Q77.8 (Other osteochondrodysplasias with defects of growth of tubular bones and spine) to document Kenny-Caffey Syndrome for billing and medical record purposes. What is the clinical classification of Kenny-Caffey Syndrome? Kenny-Caffey Syndrome is a rare genetic disorder characterized by short stature, thickened cortical bones, and narrow medullary cavities.
ICD10 code of Kenny-Caffey Syndrome and ICD9 code
ICD-10 and ICD-9 codes for Kenny-Caffey Syndrome, with classification details for clinicians, coders and patients.
Kenny-Caffey Syndrome (KCS) does not have a unique, specific code in the ICD-10 or ICD-9 systems because it is a rare condition often classified under broader categories for skeletal dysplasias. Clinicians typically use the ICD-10 code Q78.8 (Other specified osteochondrodysplasias) or Q77.8 (Other osteochondrodysplasias with defects of growth of tubular bones and spine) to document Kenny-Caffey Syndrome for billing and medical record purposes.
What is the clinical classification of Kenny-Caffey Syndrome?
Kenny-Caffey Syndrome is a rare genetic disorder characterized by short stature, thickened cortical bones, and narrow medullary cavities. Because of its rarity, Kenny-Caffey Syndrome is often categorized within the group of hypoparathyroidism-related skeletal dysplasias. While there is no dedicated ICD-9 or ICD-10 code specifically for Kenny-Caffey Syndrome, medical coders use the aforementioned "Other" category codes to track the condition within healthcare systems.
How is Kenny-Caffey Syndrome diagnosed?
Diagnosis of Kenny-Caffey Syndrome is primarily based on clinical features and radiographic findings, often confirmed through genetic testing. Key diagnostic indicators include:
- Profound hypocalcemia (low blood calcium levels) often presenting in infancy.
- Characteristic radiographic signs, specifically medullary stenosis (narrowing of the bone marrow cavity).
- Delayed closure of the anterior fontanelle.
- Ocular abnormalities, such as hyperopia or microphthalmia.
Is Kenny-Caffey Syndrome hereditary?
Kenny-Caffey Syndrome can be inherited in either an autosomal dominant or autosomal recessive pattern, depending on the specific genetic mutation. Mutations in the FAM111A gene are associated with the autosomal dominant form of Kenny-Caffey Syndrome, while mutations in the TBCE gene are linked to the autosomal recessive form. Genetic counseling is essential for families affected by Kenny-Caffey Syndrome to understand the specific inheritance risk.
What is the community experience with Kenny-Caffey Syndrome?
Navigating life with a rare condition like Kenny-Caffey Syndrome can feel isolating, but you are not alone. Currently, 4 members have joined the DiseaseMaps.org community to share their experiences with Kenny-Caffey Syndrome. Connecting with others who understand the diagnostic journey and the management of Kenny-Caffey Syndrome can provide invaluable emotional support.
Next steps
- Consult with a clinical geneticist to discuss molecular testing and inheritance patterns.
- Work with an endocrinologist to monitor calcium levels and parathyroid function.
- Connect with the 4 community members at DiseaseMaps.org to share resources and experiences.
Medical disclaimer: This information is for educational purposes only and should not replace professional medical advice, diagnosis, or treatment.
References
- Orphanet: Kenny-Caffey Syndrome (ORPHA:2311).
- NIH GARD: Kenny-Caffey Syndrome.
- OMIM: Kenny-Caffey Syndrome Type 1 (Entry #244460) and Type 2 (Entry #127000).
