ICD Code

One difficulty researchers have encountered in trying to calculate the number of CCM cases is the significant variation in ICD coding of the disorder. ICD is the system used by healthcare providers to classify and code diagnoses.


In ICD-11, cerebral cavernous malformation has a unique code.



08  Diseases of the Nervous System

8B22 Certain Specified Cerebrovascular Diseases

8B22.41 Cerebral Cavernous Malformation



United States

CCM does not have a dedicated code in ICD-10. The Alliance to Cure Cavernous Malformation Scientific Advisory Board has suggested a consensus code to allow for better tracking. In order to increase sensitivity and specificity, we request that clinicians use the following ICD-10 coding in the US for their cerebral cavernous malformation patients:

D18.02 Hemangioma of Intracranial Structures


Q28.3 Other Malformations of Cerebral Vessels is used in Europe for familial CCM only. This helps to differentiate familial CCM from sporadic, as only familial CCM is considered rare in Europe.


Updated 5.18.22