1. CAVERNOMAS: SURGICAL STRATEGY
Chandrashekhar Deopujari
Professor and Head Neurosurgery
Bombay Hospital Institute of Medical Sciences
Mumbai, India

2. CAVERNOMA
Described as “Angiographically Occult Vascular Malformation” (AOVM)
Variously called :
Cryptic Angioma,
Cavernous Malformation,
Cavernous Hemangioma,
Capillary Hemangioma,
Cavernoma and
Cavernous angioma (Russel, Rubinstein),
Known to occur anywhere in the neuraxis including on cranial / spinal nerves
C E Deopujari, Mumbai

3. CEREBROVASCULAR MALFORMATIONS
Mc Cormick (1984) : 5734 Autopsies : 4 % Incidence
AVM : 0.5 %
Cavernoma : 0.3 %
Capillary Telangiectasis : 0.8 %
Venous Angioma : 3 %
C E Deopujari, Mumbai

4. CAVERNOMA Congenital lesions : Develop in 3rd – 8th week of gestation
Occasionally “ de novo”
Radiation induced
Occur in 2 forms :
genetic studies show abnormality on p 7, first reported in hispanics (CCM1), also observed in other familial types with 2 more mutations (CCM2 & 3), less bleed ?

5. CAVERNOMA : PATHOLOGY Well defined discrete lesions Gross appearance :
“Mulberry like” dark red or purple surrounded usually by gliotic tissue
Cut section :
Honey comb of thin walled vascular spaces
C E Deopujari, Mumbai

6. CAVERNOMA : PATHOLOGY Microscopically :
Irregular sinusoidal spaces with no intervening neural tissue,
Haematomas of various ages present,
Focal Calcifications : Haemangioma calcificans : usually temporal  epilepsy  does not bleed
C E Deopujari, Mumbai

7. CAVERNOMA : IMAGING CT : Diagnostic ≤ 50 % cases, Pop Corn,
Mild enhancement
C E Deopujari, Mumbai

8. CAVERNOMA : IMAGING MRI : High degree of accuracy, Well circumscribed,
Haemorrhages of different age,
Calcifications,
Hemosiderin ring,
Low or minimal enhancement
C E Deopujari, Mumbai

9. CAVERNOMA : IMAGING 5 TYPES I ) Classical III) Punctate II) Acute
IV) Chronic
V) Cystic
C E Deopujari, Mumbai

10. CAVERNOMA : IMAGING Angiographically Occult
Angio may show associated venous angioma
Need for angio only in acute (type I) cases during first event
C E Deopujari, Mumbai

11. CAVERNOUS ANGIOMA CLINICAL PRESENTATION
1) Haemorrhage : 9 – 56 %
2) Seizures : 23 – 52 %
Progressive neurological deficit:20–45 %
Headaches : 6 – 52 %
Incidental
C E Deopujari, Mumbai

12. Cavernous malformations
Data available: 133 cases
Multiple: 6
Familial: 11
Operated: 66 lesions ,
62 patients,
69 surgeries : 56 for hemorrhage
13 for seizures

13. CAVERNOUS MALFORMATIONS
69 operated lesions
Temporal - 22
Frontal
Occipital - 5
Cerebellar – 3
Parietal – 5
Intra Ventricular – 2
Brain Stem – 16
Thalamic - 3
Optic/ Hypothalamic – 1
Spinal – 1
C E Deopujari, Mumbai

14. PEDIATRIC CAVERNOMAS 21 cases surgically excised :
4 for intractable seizures, 17 for hemorrhages
3 had multiple cavernomas ( 1 familial )
9 cavernomas in brainstem
10 other cases being observed
No radiosurgery
C E Deopujari, Mumbai

15. CAVERNOMA : NATURAL HISTORY
RATE OF HAEMORRHAGE
? < AVM
“Symptomatic presence of extralesional blood on MRI”
Per patient / per lesion
Prospective / Retrospective
Asymptomatic increase in size
0.25 – 13 % per patient / year
Event rate (clinical) : 4.2 % per patient / year
C E Deopujari, Mumbai

16. CAVERNOMA : NATURAL HISTORY
HAEMORRHAGE
Size > 10 mm :  Bleeding rate
Age < 35 yrs :  Bleeding rate
Location risk :
Brain stem cavernomas bleed 5-10 times more frequently than other locations ? (up to 21% per year)
3rd Ventricle, spinal cord and extra axial (viz. cavernous sinus) : low incidence
Cluster of events
C E Deopujari, Mumbai

17. CAVERNOMA : NATURAL HISTORY
PAEDIATRIC POPULATION :
Increased tendency for haemorrhage
Increased potential for epilepsy
PREGNANCY :
Effect of pregnancy not statistically proven but an increase in haemorrhage seen.
ASSOCIATED LESIONS :
Venous angiomas (caput medusae): upto 24 percent

18. CAVERNOMA : NATURAL HISTORY
SEIZURES
Presenting symptom in 40 – 70 % patients
More common with frontal and temporal lesions
Frequently focal in nature, secondary generalization
May or may not be associated with haemorrhage
No clear data for long term risk of developing seizures but seizure control becomes more difficult with time
C E Deopujari, Mumbai

19. CAVERNOMA : NATURAL HISTORY
SEIZURES
Mechanism : Break down products of haemorrhage with Ferric ion deposits are highly epileptogenic apart from gliosis around the lesion
Difficult diagnosis in multiple lesions or dual pathology, requiring more detailed assessment
Medically refractory in many cases
C E Deopujari, Mumbai

20. MANAGEMENT OPTIONS Observation Excision Radiosurgery ?
C E Deopujari, Mumbai

21. SELECTION OF TREATMENT MODALITY
Surgical excision
Complete excision including resection of surrounding hemosiderin ring (if safe) to control seizures is effective (Ogilvey, Scott, 1999)
88 % for lesionectomy alone (Zevgaridis)
Less success if > 5 seizures or duration > 2 years
C E Deopujari, Mumbai

22. N BORNARE 0.57
C E Deopujari, Mumbai

23. IMAGE GUIDED EXCISION FOR SEIZURE( short duration) CONTROL
C E Deopujari, Mumbai

24. CEREBELLAR VERMIAN CAVERNOMA
K Charania
CEREBELLAR VERMIAN CAVERNOMA
C E Deopujari, Mumbai

25. CEREBELLAR VERMIAN CAVERNOMA
K Charania
CEREBELLAR VERMIAN CAVERNOMA
POST OP
C E Deopujari, Mumbai

26. BLEED IN RESIDUAL LESIONKC

27. AFTER SECOND SURGERY KC

28. THALAMIC CAVERNOMA WITH ACUTE BLEED IN A 5 YEAR OLD BOY
Idrasi
THALAMIC CAVERNOMA WITH ACUTE BLEED IN A 5 YEAR OLD BOY
TRANSCALLOSAL SURGERY FOR COMPLETE EXCISION
C E Deopujari, Mumbai

29. THALAMIC CAVERNOMA WITH IV BLEED IN A 5 YEAR OLD BOY
TRANSCALLOSAL SURGERY FOR COMPLETE EXCISION
C E Deopujari, Mumbai

30. THALAMIC CAVERNOMA WITH IV BLEED IN A 5 YEAR OLD BOY
TRANSCALLOSAL SURGERY FOR COMPLETE EXCISION
C E Deopujari, Mumbai

31. CAVERNOMA Surgical strategies for epilepsy : include
Image guided technique
Steretotactically guided technique
Functional MRI for pre operative localization
USG : Hyper echoic signal for per operative localization
EcoG tailored resections may be rarely required
Brain mapping in motor or speech area
In multiple cavernomas : subpial transections described
C E Deopujari, Mumbai

32. AWAKE CRANIOTOMY : ECOG GUIDED RESECTION
CHRONIC SEIZURE DISORDER
AWAKE CRANIOTOMY : ECOG GUIDED RESECTION
Uncontrolled seizures for 6 years left temporal localisation
Previous surgery for right parietal cavernoma with large bleed 8 years ago
Complete seizure freedom for last 3 years with no deficit

33. SELECTION OF TREATMENT MODALITY
Surgical excision for haemorrhage
in high risk location viz brain stem, basal ganglia
Zimmerman et al (1991) : 16 cases; no mortality, 1 major , 15 minor/transient deficits
Ojeman et al (1993): 8 cases; no mortality, 2 major, 6 minor/ transient deficits
Amin- Hanjani et al (1998): 14 cases; no mortality, 2 major, 12 minor/ transient deficits
Bertalanffy et al ( 2002 ): 24 cases of brainstem and 12 in basal ganglia: no mortality, 6% long term morbidity
C E Deopujari, Mumbai

34. SELECTION OF TREATMENT MODALITY
Radiosurgery :
Reduction in frequency of seizures as well as risk of haemorhage; (Kondizolka et al, 1995)
Stereotactic radiosurgery is associated with high rate of radiation injury % ; (Amin Hanjani et al , 1998)
Decrease in hemorrhage not well demonstrated (over 8% per year in first 2 years and over 40% on longer follow up)
No obliteration criteria
Randomized trial ?
C E Deopujari, Mumbai

35. BRAIN STEM CAVERNOMA A – Midline supracerebellar B – 4th Ventricular
C – CP Angle
D – Lateral supracerebellar
C E Deopujari, Mumbai

36. PONTINE CAVERNOMA 12 yr old, 2 hge episodes
DORSALLY PLACED
C E Deopujari, Mumbai

37. BRAIN STEM CAVERNOMA
Access to brainstem without damaging nuclei and major fiber tracts :
*Brain stem mapping
*Image guidance
C E Deopujari, Mumbai

38. PONTINE CAVERNOMA EXCISION EXCISION THROUGH THE 4TH VENTRICLE
C E Deopujari, Mumbai

39. DORSALLY PLACED IN MEDULLA
BRAIN STEM CAVERNOMA SS
DORSALLY PLACED IN MEDULLA
C E Deopujari, Mumbai

40. EXCISION THROUGH CERVICO MEDULLARY CISTERNSS
0.40
EXCISION THROUGH CERVICO MEDULLARY CISTERN
C E Deopujari, Mumbai

41. PRE OP
POST OP
C E Deopujari, Mumbai

42. VENTRO LATERALLY PLACED IN PONS
BRAIN STEM CAVERNOMA
VENTRO LATERALLY PLACED IN PONS
C E Deopujari, Mumbai

43. BRAIN STEM CAVERNOMA Antero-lateral approach : Pre sigmoid PRE - OP
POST - OP
C E Deopujari, Mumbai

44. BRAIN STEM CAVERNOMA
POST - OPERATIVE
C E Deopujari, Mumbai

45. CAVERNOUS ANGIOMAS OBSERVATION AND FOLLOW UP :
All asymptomatic / incidentally detected lesions
Symptomatic lesions in deep / critical areas when surgical risk is significant AND recc. haemorrhage and ↑ deficits not present
Familial / multiple cases
Follow up with MRI- Half yearly for 2 yrs. And then annually
C E Deopujari, Mumbai

46. BRAIN STEM CAVERNOMA 18 YR OLD GIRL PRESENTING WITH SEVERE HEADACHES
2 MAJOR EPISODES,
NO NEURODEFICIT KG

47. OSSIFIED CAVERNOMA KG

48. MULTIPLE CAVERNOMAS : FAMILIALAD
MULTIPLE CAVERNOMAS : FAMILIAL
C E Deopujari, Mumbai

49. RA
2002
C E Deopujari, Mumbai

50. RA
July, 2003
C E Deopujari, Mumbai

51. RA
August, 2003
C E Deopujari, Mumbai

52. PRE OP
POST OP
C E Deopujari, Mumbai

53. Follow up 2 patients required surgery for 2nd lesion.
1 patient required repeat surgery for intractable seizures, 2 for residual lesion with recurrent hge.
Transient 6th, 7th N paresis in 4 brainstem cavernomas, persistent 7th paresis in 2
Trunkal ataxia and oscillopsia in 1 ( 1yr)
1 death, ( unrelated cardiac- event )

54. CAVERNOUS MALFORMATIONS
Surgical considerations :
Recent/ recurrent bleed / clustering
Progressive neurological deficit /increase in size of the lesion
Accessibility in eloquent area : lesion near pial or ependymal surface
Lesion size ( > 10 mm )
Brainstem mapping? Image guidance?
C E Deopujari, Mumbai

55. Thank You