Short answer · Medically reviewed summary · Last updated: 2026-05-08
Russell-Silver Syndrome (RSS), also known as Silver-Russell Syndrome, is classified under ICD-10 code Q87.1 and ICD-9 code 759.89. These diagnostic codes are essential for medical billing and clinical documentation to ensure patients receive appropriate care for this rare growth disorder. What is the clinical definition of Russell-Silver Syndrome? Russell-Silver Syndrome is a rare, genetically heterogeneous condition characterized primarily by intrauterine growth restriction (IUGR) and postnatal growth failure.
1 people with Russell Silver Syndrome have shared their first-person experience on this question at DiseaseMaps.
ICD10 code of Russell Silver Syndrome and ICD9 code
ICD-10 and ICD-9 codes for Russell Silver Syndrome, with classification details for clinicians, coders and patients.
Russell-Silver Syndrome (RSS), also known as Silver-Russell Syndrome, is classified under ICD-10 code Q87.1 and ICD-9 code 759.89. These diagnostic codes are essential for medical billing and clinical documentation to ensure patients receive appropriate care for this rare growth disorder.
What is the clinical definition of Russell-Silver Syndrome?
Russell-Silver Syndrome is a rare, genetically heterogeneous condition characterized primarily by intrauterine growth restriction (IUGR) and postnatal growth failure. Individuals with Russell-Silver Syndrome often present with a distinct triangular face, a prominent forehead, and body asymmetry. Among the 263 members of our DiseaseMaps community, we see a wide spectrum of these physical features, emphasizing that the condition manifests differently in every patient.
How is Russell-Silver Syndrome diagnosed?
Diagnosis of Russell-Silver Syndrome is primarily clinical, supported by the Netchine-Harbison clinical scoring system. Genetic testing is critical for confirmation, as molecular testing identifies an underlying cause in approximately 60% of cases. The following molecular mechanisms are commonly investigated:
- Loss of methylation on chromosome 11p15.5 (found in roughly 40-50% of cases).
- Maternal uniparental disomy of chromosome 7 (found in approximately 5-10% of cases).
- Rare duplications, deletions, or translocations involving chromosomes 7 or 11.
Is there a cure for Russell-Silver Syndrome?
There is currently no cure for Russell-Silver Syndrome; however, clinical management is highly effective at improving quality of life. Treatment is multidisciplinary and focuses on addressing growth hormone deficiency, feeding difficulties, and potential orthopedic complications. Early intervention is vital for managing the metabolic and developmental challenges associated with Russell-Silver Syndrome.
Next steps
- Consult a pediatric endocrinologist to discuss growth hormone therapy options.
- Coordinate with a clinical geneticist to verify the specific molecular subtype of your Russell-Silver Syndrome diagnosis.
- Connect with the 263 members on DiseaseMaps.org to share experiences and find emotional support within our specialized community.
- Monitor for orthopedic issues such as scoliosis or limb length discrepancy with a pediatric orthopedist.
Medical disclaimer: This information is for educational purposes only and should not replace professional medical advice, diagnosis, or treatment; always seek the guidance of your physician regarding a medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Russell-Silver Syndrome.
- Orphanet: Silver-Russell Syndrome (ORPHA:814).
- OMIM (Online Mendelian Inheritance in Man): Silver-Russell Syndrome (Entry #180860).
- The MAGIC Foundation: Russell-Silver Syndrome resources.
Posted Aug 21, 2017 by Scarlett 2100
