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CAVERNOMAS: SURGICAL STRATEGY

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,

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CAVERNOMAS: SURGICAL STRATEGY

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  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 I ) Classical III) Punctate II) Acute 5 TYPES 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 ANGIOMACLINICAL 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 - 11 • 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. cavernoussinus) : 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 BORNARE0.57 C E Deopujari, Mumbai

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

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

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

  26. BLEED IN RESIDUAL LESION KC

  27. AFTER SECOND SURGERY KC

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

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

  30. THALAMIC CAVERNOMA WITH IV BLEEDIN 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. 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- 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

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

  36. PONTINE CAVERNOMA12 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. BRAIN STEM CAVERNOMA SS DORSALLY PLACED IN MEDULLA C E Deopujari, Mumbai

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

  41. PRE OP POST OP C E Deopujari, Mumbai

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

  43. BRAIN STEM CAVERNOMA PRE - OP • Antero-lateral approach : Pre sigmoid 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. AD MULTIPLE CAVERNOMAS : FAMILIAL C E Deopujari, Mumbai

  49. RA 2002 C E Deopujari, Mumbai

  50. RA July, 2003 C E Deopujari, Mumbai

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