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

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

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

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 ?

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

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

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

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

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

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

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

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

CAVERNOUS MALFORMATIONS 69 operated lesions Temporal - 22 Frontal - 11 Occipital - 5 Cerebellar – 3 Parietal – 5 Intra Ventricular – 2 Brain Stem – 16 Thalamic - 3 Optic/ Hypothalamic – 1 Spinal – 1 C E Deopujari, Mumbai

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

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

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

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

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

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

MANAGEMENT OPTIONS Observation Excision Radiosurgery ? C E Deopujari, Mumbai

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

N BORNARE 0.57 C E Deopujari, Mumbai

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

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

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

BLEED IN RESIDUAL LESION KC

AFTER SECOND SURGERY KC

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

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

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

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

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

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

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- 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 ? C E Deopujari, Mumbai

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

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

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

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

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

EXCISION THROUGH CERVICO MEDULLARY CISTERN SS 0.40 EXCISION THROUGH CERVICO MEDULLARY CISTERN C E Deopujari, Mumbai

PRE OP POST OP C E Deopujari, Mumbai

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

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

BRAIN STEM CAVERNOMA POST - OPERATIVE C E Deopujari, Mumbai

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

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

OSSIFIED CAVERNOMA KG

MULTIPLE CAVERNOMAS : FAMILIAL AD MULTIPLE CAVERNOMAS : FAMILIAL C E Deopujari, Mumbai

RA 2002 C E Deopujari, Mumbai

RA July, 2003 C E Deopujari, Mumbai

RA August, 2003 C E Deopujari, Mumbai

PRE OP POST OP C E Deopujari, Mumbai

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 )

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

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