Natural history of cavernous malformations: 1. They have a sporadic or familial form and can occur anywhere in the CNS but are most common supratentorially. 2. Clinical findings include hemorrhages surrounding the lesion seen on MRI. Lesions can increase in size from repeated small hemorrhages. 3. The risk of rebleeding is higher for symptomatic lesions and those with overt extralesional hemorrhage, ranging from 5-30% per year depending on location in the brain or spinal cord.

1. Natural History of
Carvernous Malformation
Youmans Chapter 392
Joseph M.Zabramski, Iman Feiz-Erfan

2. Outline
• Epidemiology
• Clinical finding
• Natural history

3. Epidemiology
• 1 : 200-250,equal in both sex
• 2 form
– Sporadic form
• Single lesion
– Familial form
• Mutiple lesion
• Autosomal dominant
– chromosome 7q(CCM1)
– chromosome 7p(CCM2)
– chromosome 3q(CCM3)
• Pathognomonic : > or more 3 lesion + family history of
seizure
• T2 80 %, gradient echo 90 % for lesion

4. Epidemilogy
• CNS
– 80% supratentorial
– 15% brainstem and basal ganglia
– 5% spinal cord
• Not all patient symptomatic

5. Clinical finding
• Hemorrhages + Hemosiderin in cerebral tissue
surrounding : unique MRI
• Increase in size : repeat small hemorrhage within lesion
and from spontaneous thrombosis of blood-filled cavern
• Ruptures outside capsule : overt hemorrhage
• Low flow, low pressure, hemorrhage

6. MRI classification

7. Axial T2
-core of lesion reticulate : salt and
pepper
-surround by a halo of low signal
-Patho : hemorrhage and
thrombosis,hemosiderin-stained brain
tissue
-absence of mass effect and edema
-type II

8. Coronal T1
-focal subacute blood outside the capsule of lesion : overt extralesional
-type IA

9. Axial T2
-type II
-type III

10. Supratentorial lesion
• Seizure is the most common symptom
• Do not contain neuronal tissue  not intrinsic
epileptogenic
• Focal gliosis, hemosiderin deposition in cellular and
humoral inflammatory response cause seizure
• MRI evident acute/subacute hemorrhage
• Pathology : surrounded by yellow-brown-stained border
that is infiltrated with hemosiderin-laden macrophages

11. • A : type II cavernous malformation c small type III cavernous malformation
• B : close up view
• C : Prussian blue – hemosiderin and iron deposition

12. Brainstem lesions
• Sudden onset of focal neurological deficit,maximal at
onset
• First episode : tend to resolve
• Recurrent episode : progressive more severe deficit and
increase risk for permanent neurological impairment

13. Pt in first episode
A,B
CT increase density at Rt.pons
C,D
T2 ovet extralesional
Pt in 2 Mo later
E,F
dramatic increase size of lesion

14. Spinal cord lesion
• 2 From
– Major hemorrhage with sudden onset of symptom and
neurological deficit
– Slow progressive myelopathic or radicular pain
• Patients with spinal cord cavernous malformation had
significant risk for intracranial lesion

15. Natural history
• Risk for recurrent hemorrhage higher in symptomatic
lesion
• Overt or extralesional hemorrhage(type IA) : 30% per
year
• Acute lesional hemorrhage(typeIB) or with symptomatic
type II lesion : 5-10% per year

16. Brainstem cavernous malformation
• Eloquence of surrounding structures
• Episode of hemorrhage are much more likely to be
symptomatic
• Risk for hemorrhage 2.5-6.8 % per year
• Risk for rebleeding 5.1-60 per year, recent hemorrhage
or evidence of extralesional hemorrhage on MRI

17. Spinal cord
cavernous malformation
• Rarely diagnosed before the onset of symptom
• After symptomatic,progressive neurological deficit

18. Familial
• Seizure,headaches with supratentorial lesion, focal
neurological deficit
• Clinical silent hemorrhage were common
• New lesion develop

19. Pregnancy and gender
• Pregnancy and puerperium increase risk for hemorrhage
• Cavernous malformation increase size
• Management
– Base on time symptom develop, severity, imaging characteristic
– Need for emergency neurological treatment during pregnancy is
rare
– Method of delivery should be based on obstetric consideration
• Hemorrhage rate equal in both sex, hormonal no effect

20. Thank you