Short answer · Medically reviewed summary · Last updated: 2026-05-08
CLOVES syndrome does not have a unique, dedicated code in the ICD-9 or ICD-10 systems, as it is a rare overgrowth disorder often categorized under broader classifications for congenital malformations or vascular anomalies. Clinicians typically use ICD-10 code Q87.3 (Congenital malformation syndromes involving overgrowth) or Q27.8 (Other specified congenital malformations of peripheral vascular system) to document and bill for CLOVES syndrome. What exactly is CLOVES syndrome? CLOVES syndrome is a rare, complex disorder characterized by Congenital Lipomatous Overgrowth, Vascular malformations, Epidermal nevi, and Spinal/Skeletal anomalies.
ICD10 code of Cloves Syndrome and ICD9 code
ICD-10 and ICD-9 codes for Cloves Syndrome, with classification details for clinicians, coders and patients.
CLOVES syndrome does not have a unique, dedicated code in the ICD-9 or ICD-10 systems, as it is a rare overgrowth disorder often categorized under broader classifications for congenital malformations or vascular anomalies. Clinicians typically use ICD-10 code Q87.3 (Congenital malformation syndromes involving overgrowth) or Q27.8 (Other specified congenital malformations of peripheral vascular system) to document and bill for CLOVES syndrome.
What exactly is CLOVES syndrome?
CLOVES syndrome is a rare, complex disorder characterized by Congenital Lipomatous Overgrowth, Vascular malformations, Epidermal nevi, and Spinal/Skeletal anomalies. Because CLOVES syndrome is caused by somatic mosaic mutations in the PIK3CA gene, it presents with a highly variable clinical phenotype, which makes standardized medical coding challenging for healthcare providers.
How is CLOVES syndrome diagnosed and managed?
Diagnosis of CLOVES syndrome is primarily clinical, supported by targeted genetic testing of affected tissue. Management requires a multidisciplinary team approach to address the specific needs of each patient. Common clinical focus areas for those living with CLOVES syndrome include:
- Surgical management: Debulking of fatty overgrowth and correction of skeletal deformities.
- Vascular intervention: Sclerotherapy or embolization to manage complex vascular malformations.
- Pharmacological therapy: Use of PI3K inhibitors (such as alpelisib) to target the underlying molecular pathway.
- Orthopedic support: Monitoring for scoliosis and limb length discrepancies.
Is there a community for those affected by CLOVES syndrome?
Navigating the diagnostic journey of CLOVES syndrome can feel isolating, but you are not alone. Currently, 7 people with CLOVES syndrome have joined the DiseaseMaps.org community to share their experiences, provide peer support, and exchange information on managing the daily challenges of this condition.
Next steps
- Consult with a geneticist or a specialist in vascular anomalies to confirm a diagnosis of CLOVES syndrome.
- Connect with the 7 members of the CLOVES syndrome community on DiseaseMaps.org for lived-experience insights.
- Request a referral to a center of excellence that specializes in PIK3CA-related overgrowth spectrum (PROS) disorders.
Medical disclaimer: This information is for educational purposes and does not replace professional medical advice, diagnosis, or treatment; always seek the guidance of your physician regarding any medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD): CLOVES syndrome entry.
- Orphanet: Rare disease database entry for CLOVES syndrome.
- OMIM (Online Mendelian Inheritance in Man): #612918 (CLOVES Syndrome).
- The CLOVES Syndrome Community (patient advocacy organization).
