Short answer · Medically reviewed summary · Last updated: 2026-05-08
Fraser syndrome is classified under the ICD-10 code Q87.0 (Congenital malformation syndromes predominantly affecting facial appearance) and the ICD-9 code 759.89 (Other specified congenital anomalies). These diagnostic codes are essential for medical documentation, insurance billing, and tracking the clinical management of patients living with this rare condition. What is the clinical presentation of Fraser syndrome? Fraser syndrome is a rare autosomal recessive disorder characterized by a specific constellation of developmental features.
ICD10 code of Fraser Syndrome and ICD9 code
ICD-10 and ICD-9 codes for Fraser Syndrome, with classification details for clinicians, coders and patients.
Fraser syndrome is classified under the ICD-10 code Q87.0 (Congenital malformation syndromes predominantly affecting facial appearance) and the ICD-9 code 759.89 (Other specified congenital anomalies). These diagnostic codes are essential for medical documentation, insurance billing, and tracking the clinical management of patients living with this rare condition.
What is the clinical presentation of Fraser syndrome?
Fraser syndrome is a rare autosomal recessive disorder characterized by a specific constellation of developmental features. The clinical hallmarks of Fraser syndrome include cryptophthalmos (hidden eyes where the eyelids fail to separate), syndactyly (webbing of fingers or toes), and renal agenesis or dysplasia. Because Fraser syndrome affects multiple organ systems, a multidisciplinary approach is required to address the complex surgical and developmental needs of affected individuals.
Is Fraser syndrome hereditary?
Yes, Fraser syndrome is inherited in an autosomal recessive pattern, meaning an individual must inherit a pathogenic variant in both copies of the causative gene—most commonly the FRAS1, FREM2, or GRIP1 genes—to manifest the condition. For parents who are both carriers, there is a 25% chance with each pregnancy that the child will have Fraser syndrome. Genetic counseling is strongly recommended for families affected by Fraser syndrome to understand these risks and explore reproductive options.
How is Fraser syndrome managed?
Management of Fraser syndrome requires coordination between several specialists due to the severity of the malformations. Care often involves:
- Ophthalmology: Specialized surgical intervention for ocular surface reconstruction.
- Nephrology/Urology: Monitoring renal function, as renal agenesis is a common and serious feature of Fraser syndrome.
- Plastic Surgery: Correction of syndactyly and other craniofacial anomalies.
- Clinical Genetics: Ongoing support and family screening.
Next steps
- Consult with a clinical geneticist to confirm the diagnosis of Fraser syndrome via molecular testing.
- Connect with the 17 members of the DiseaseMaps.org community who are currently navigating life with Fraser syndrome.
- Seek a referral to a major academic medical center with experience in managing complex congenital syndromes.
Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment; always seek the guidance of your physician regarding a medical condition.
