CAVERNOMAS: SURGICAL STRATEGY

CAVERNOMAS: SURGICAL STRATEGYCAVERNOMAS: SURGICAL STRATEGY

Chandrashekhar DeopujariChandrashekhar DeopujariProfessor and Head NeurosurgeryProfessor and Head Neurosurgery

Bombay Hospital Institute of Medical Sciences Bombay Hospital Institute of Medical Sciences Mumbai, IndiaMumbai, India

Chandrashekhar DeopujariChandrashekhar DeopujariProfessor and Head NeurosurgeryProfessor and Head Neurosurgery

Bombay Hospital Institute of Medical Sciences Bombay Hospital Institute of Medical Sciences Mumbai, IndiaMumbai, India

CAVERNOMACAVERNOMADescribed 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

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, MumbaiC E Deopujari, Mumbai

CEREBROVASCULAR MALFORMATIONSCEREBROVASCULAR MALFORMATIONS

Mc Cormick (1984) : 5734 Autopsies : 4 % Incidence

1) AVM : 0.5 %

2) Cavernoma : 0.3 %

3) Capillary Telangiectasis : 0.8 %

4) Venous Angioma : 3 %

Mc Cormick (1984) : 5734 Autopsies : 4 % Incidence

1) AVM : 0.5 %

2) Cavernoma : 0.3 %

3) Capillary Telangiectasis : 0.8 %

4) Venous Angioma : 3 %


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 ?

CAVERNOMA

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

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

CAVERNOMA : PATHOLOGY


CAVERNOMA : PATHOLOGYCAVERNOMA : 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

Microscopically :

Irregular sinusoidal spaces with no intervening neural tissue,

Haematomas of various ages present,

Focal Calcifications : Haemangioma calcificans : usually temporal epilepsy does not bleed


CAVERNOMA : IMAGINGCAVERNOMA : IMAGING

CT : Diagnostic ≤ 50 % cases,

Pop Corn,

Mild enhancement

CT : Diagnostic ≤ 50 % cases,

Pop Corn,

Mild enhancement


CAVERNOMA : IMAGINGCAVERNOMA : IMAGINGMRI : High degree of accuracy,

Well circumscribed,

Haemorrhages of different age,

Calcifications,

Hemosiderin ring,

Low or minimal enhancement

MRI : High degree of accuracy,

Well circumscribed,

Haemorrhages of different age,

Calcifications,

Hemosiderin ring,

Low or minimal enhancement


IV) Chronic IV) Chronic

II) AcuteII) AcuteI ) ClassicalI ) Classical III) Punctate III) Punctate

CAVERNOMA : IMAGING

V) CysticV) Cystic

5 TYPES5 TYPES


CAVERNOMA : IMAGINGCAVERNOMA : IMAGING

Angiographically Occult Angiographically Occult

Angio may show associated venous angioma Angio may show associated venous angioma

Need for angio only in acute (type I) cases during first event Need for angio only in acute (type I) cases during first event


CAVERNOUS ANGIOMA CLINICAL PRESENTATION

CAVERNOUS ANGIOMA CLINICAL PRESENTATION

1) Haemorrhage : 9 – 56 %

2) Seizures : 23 – 52 %

3) Progressive neurological deficit:20–45 %

4) Headaches : 6 – 52 %

5) Incidental

1) Haemorrhage : 9 – 56 %

2) Seizures : 23 – 52 %

3) Progressive neurological deficit:20–45 %

4) Headaches : 6 – 52 %

5) Incidental


Cavernous malformations

Data available: 133 cases

Multiple: 6

Familial: 11

Operated: 66 lesions ,

62 patients,

69 surgeries : 56 for hemorrhage

13 for seizures

Temporal - 22

Frontal - 11

Occipital - 5

Cerebellar – 3

Parietal – 5

Intra Ventricular – 2

Brain Stem – 16

Thalamic - 3

Optic/ Hypothalamic – 1

Spinal – 1

Temporal - 22

Frontal - 11

Occipital - 5

Cerebellar – 3

Parietal – 5

Intra Ventricular – 2

Brain Stem – 16

Thalamic - 3

Optic/ Hypothalamic – 1

Spinal – 1

CAVERNOUS MALFORMATIONS69 operated lesions

CAVERNOUS MALFORMATIONS69 operated lesions


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

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


CAVERNOMA : NATURAL HISTORY 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

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


CAVERNOMA : NATURAL HISTORY 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

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


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

CAVERNOMA : NATURAL HISTORY

CAVERNOMA : NATURAL HISTORY 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

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


CAVERNOMA : NATURAL HISTORY CAVERNOMA : NATURAL HISTORY

SEIZURESMechanism : 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

SEIZURESMechanism : 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


MANAGEMENT OPTIONS MANAGEMENT OPTIONS

Observation Observation ExcisionExcision Radiosurgery ?Radiosurgery ?


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

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

SELECTION OF TREATMENT MODALITYSELECTION OF TREATMENT MODALITY


N BORNARE

0.57

C E Deopujari, Mumbai

IMAGE GUIDED EXCISION

FOR SEIZURE( short duration) CONTROL

IMAGE GUIDED EXCISION

FOR SEIZURE( short duration) CONTROL


K Charania

CEREBELLAR VERMIAN CAVERNOMACEREBELLAR VERMIAN CAVERNOMA


K Charania

CEREBELLAR VERMIAN CAVERNOMACEREBELLAR VERMIAN CAVERNOMA

POST OP


BLEED IN RESIDUAL LESION

KC

AFTER SECOND SURGERY

KC

Idrasi

THALAMIC CAVERNOMA WITH ACUTE BLEEDIN A 5 YEAR OLD BOY

THALAMIC CAVERNOMA WITH ACUTE BLEEDIN A 5 YEAR OLD BOY

TRANSCALLOSAL SURGERY FOR COMPLETE EXCISIONTRANSCALLOSAL SURGERY FOR COMPLETE EXCISION C E Deopujari, MumbaiC E Deopujari, Mumbai

TRANSCALLOSAL SURGERY FOR COMPLETE EXCISIONTRANSCALLOSAL SURGERY FOR COMPLETE EXCISION

THALAMIC CAVERNOMA WITH IV BLEEDIN A 5 YEAR OLD BOY



TRANSCALLOSAL SURGERY FOR COMPLETE EXCISIONTRANSCALLOSAL SURGERY FOR COMPLETE EXCISION




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

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

CAVERNOMA CAVERNOMA


AWAKE CRANIOTOMY : ECOG GUIDED RESECTIONAWAKE 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

CHRONIC SEIZURE DISORDER CHRONIC SEIZURE DISORDER

Surgical excision for haemorrhagein 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

Surgical excision for haemorrhagein 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



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- 18-27% ; (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 ?

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- 18-27% ; (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 ?



BRAIN STEM CAVERNOMABRAIN STEM CAVERNOMA


A – Midline supracerebellar

B – 4th Ventricular

C – CP Angle

D – Lateral supracerebellar

PONTINE CAVERNOMA12 yr old, 2 hge episodes

PONTINE CAVERNOMA12 yr old, 2 hge episodes

C E Deopujari, MumbaiC E Deopujari, Mumbai DORSALLY PLACED



Access to brainstem without damaging nuclei and major fiber tracts :

*Brain stem mapping

*Image guidance

PONTINE CAVERNOMA EXCISION PONTINE CAVERNOMA EXCISION

C E Deopujari, MumbaiC E Deopujari, MumbaiEXCISION THROUGH THE 4TH VENTRICLE

SS


DORSALLY PLACED IN MEDULLADORSALLY PLACED IN MEDULLA

BRAIN STEM CAVERNOMA

SS0.40


EXCISION THROUGH CERVICO MEDULLARY CISTERN

PRE OPPRE OP

POST OPPOST OP


BRAIN STEM CAVERNOMA

VENTRO LATERALLY PLACED IN PONSVENTRO LATERALLY PLACED IN PONS C E Deopujari, Mumbai

BRAIN STEM CAVERNOMABRAIN STEM CAVERNOMA Antero-lateral approach : Pre sigmoid Antero-lateral approach : Pre sigmoid PRE - OPPRE - OP

POST - OPPOST - OP


BRAIN STEM CAVERNOMAPOST - OPERATIVEPOST - OPERATIVE


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

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

CAVERNOUS ANGIOMAS CAVERNOUS ANGIOMAS



KG

18 YR OLD GIRL

PRESENTING WITH SEVERE HEADACHES

2 MAJOR EPISODES,

NO NEURODEFICIT

OSSIFIED CAVERNOMAOSSIFIED CAVERNOMA

KG

MULTIPLE CAVERNOMAS : FAMILIALMULTIPLE CAVERNOMAS : FAMILIAL

ADAD


20022002RARA


July, 2003July, 2003RARA


RARA

August, 2003August, 2003


POST OPPOST OPPRE OPPRE OP


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 )

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?

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?

CAVERNOUS MALFORMATIONS CAVERNOUS MALFORMATIONS


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CAVERNOMAS: SURGICAL STRATEGY

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