ICD-10 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.

The Angioma Alliance Scientific Advisory Board has suggested a consensus code moving forward to allow for better tracking. In order to increase sensitivity and specificity, we request that clinicians use the following ICD coding for their cerebral cavernous malformation patients:

ICD-9 code:

228.02  Hemangioma of Intracranial Structures

In ICD-10, this code converts to:

D18.02 Hemangioma of Intracranial Structures

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

In ICD-11, cerebral cavernous malformation will have a unique code:

08  Diseases of the Nervous System

8B22 Certain Specified Cerebrovascular Diseases

H00534 Cerebral Cavernous Malformation


Updated 3.15.20