A proposed Difficulty Grading System For Craniocervical Lesions Mohamed El-Fiki, D.NCh, MD Professor of Neurosurgery. University of Alexandria. Egypt Honorary President ESNS Assistant Secretary CAANS Assistant Treasurer AFNS Member WFNS skull base Committee. Member WFNS Constitution & Bylaws Committee Member WFNS Educational Committee
Rationale There have been no accepted classification for CCx lesions that addressed the selection of operative approach or prognosticate difficulties during exposure, nor the expected outcome after embarking on surgical removal
Transitional Zone classification Benign X malignant Cranial X Cervical Anterior X Posterior MRI features Intraparencymal X Extraparenchymal Neurovascular bundle Cranial nerves, Ry
Simple arachnoid cyst & Syrinx
D e r m o i d & E p i d e r m o i d
Chordoma Glomus
Craniocervical Neurofibroma
Cranio-Cx Meningioma clival CPA
Neurosurgery: December 1996 - Volume 39 - Issue 6 - pp 1086-1095. Clinical Studies Surgical Results for Meningiomas of the Craniocervical Junction, Samii M ; Klekamp J ; Carvalho G Four groups spinocranial meningiomas craniocervical meningiomas: lower clivus lateral meningiomas posterior meningiomas drilling the posterior third of an occipital condyle in a minority of lesions 63% totally removed and 30% of subtotally removed, 2 clinical recurrence. Complications were encountered in 30% of patients, predominantly with recurrent and/or infiltrative or en plaque meningiomas. Whereas motor weakness and gait ataxia tended to improve postoperatively, Cranial nerve deficits usually remained unaltered. CONCLUSION: The relationship of the tumor to neighboring structures, i.e., the vertebral artery in particular, determines its respectability. Using extreme caution with recurrent or en plaque meningiomas and tumors associated with extensive arachnoid scarring.
Craniocervical Intraparencymal
Cystic Intrinsic pontomedullary Cx
Solid Diffuse Pontomedullary-Cx
Diffuse Brain stem Lesions
Posteriorly exophytic Glioma CCx
Anteriorly surfacing Granuloma
Craniocervical Intraparencymal No progress has been made in the outcome of patients with diffuse intrinsic brain stem gliomas in over 3 decades. Little is known about the biology of these tumors due to the limited tissue availability. John-Paul Kilday & Ute Katharina Bartels & Eric Bouffet Curr Neurol Neurosci Rep (2014) 14:441Targeted Therapy in Pediatric Low-Grade Glioma
Cervicocranial intramedullary
BMC Musculoskeletal Disorders 2009, 10(Suppl 1):S1 Malformations of the craniocervical junction (chiari type I and syringomyelia: classification, diagnosis and treatment) Alfredo Avellaneda Fernández et al It is necessary that the physicians involved in the care of people with this condition comprehensively approach the management and follow-up of the patients, and that they organize interdisciplinary teams including all the professionals that can help to increase the quality of life of patients.
Epidermoids, Dermoids, Chordomas, meningiomas, schwanomas & neurofibromas have peculiarities Biology studies performed on DIPG to date have demonstrated significant differences between these tumors and supratentorial gliomas, suggesting a role for the tumor microenvironment. Upper Cx-Cr gliomas?
Romanian Neurosurgery (2012) XIX 4: 251 263 D. Serban, N.A. Calina, Fl. Exergian, M. Podea, C. Zamfir, E. Morosanu, A. Giovani, Gh. Checiu Surgical treatment of upper cervical spine tumors, whether they are vertebral, epidural, subdural or intramedullary, raises technical and decisional difficulties regarding the approach of the region as well as in maintaining its stability C1, C2 cervical tumoral pathology histopathology types Vertebral tumors 24 cases Subdural extramedulary tumors; dumpbell tumors 11 cases Intramedullary tumors 7 cases Epidural tumors 2 cases
Material and Methods 92 pts craniocervical lesions were studied 26 operated pts for CCx lesions at the department of Neurological surgery, University of Alexandria, Egypt were retrospectively analyzed in a pilot study of surgical difficulties contributing to morbidity or mortality as related to Size, Level, Site, MRI features, Relation to vascular structures, Respiratory and cranial nerve deficits.
Material & Methods A grading system was suggested The grading system emphasizes the lesion s relation to important neurovascular structures at the foramen magnum.
Material and Methods Analysis included: epidemiological data, clinical presentation, radiological parameters & histopathological diagnosis. The grading system emphasizes lesion relation to important neurovascular structures at the craniocervical area
Material & Methods The proposed grading was tested prospectively in the following 66 pts to anticipate the difficulty of these lesions & the resultant morbidity & mortality Radio-surgery was used for residual benign tumors recently Aneurysms are Currently Rx endovascular
First 26 cases of CCx Lesions
Old Classification of 92 CCx Lesions Site Retrospective pts Prospective pts # ID-IM (intrins) ID-EM (Lat.) 8 (6 Glioma + 2 Ependymoma) 10 (6 NF + 4 Mening.) 18 (6 Glioma + 4 granulomas + 8 Ependymoma) 24 (12 NF + 10 Mening. + 2 epiderm) 26 34 ID-EM (Ant.) 2 Mening. 18 (12 Mening+ 4 NF+ 2 aneurysms ) 20 ED 2 4 6 (Ant.) Chordoma (2 Chordoma + 2 Mets) ID-E 4 NF 2 Mets 6 Total 26 66 92
Prognostic classification of CCT Site 4/22 Size 4/22 NVS 5/22 Level 3/22 Size Level Site MRI features Relation to vascular structures MRI 3/22 Respiratory and cranial nerve deficits CN's 3/22 Relative weights were assigned to different bullets Maximum score 22
Sex distribution 92 CCx lesions 18 Male Female 16 14 # of patients 12 10 8 6 4 2 0 Pathology
Spinal manifestation Manifestations in 92 CCxT Brachialgia 2 6 Brown Sequard 8 18 Quadriparesis 6 18 Paraparesis 10 24 0 5 10 15 20 25 30 35 # of cases Retrospective Group Prospective Group
Prognostic classification of CC Lesions Size Level Site MRI features Relation to neurovascular structures Respiratory and cranial nerve deficits Size <1 cm in largest dimension 1 1-2 cm 2 2-3 cm 3 > 3 cm 4
Size distribution of 92 CCx 14 Size varies between 1-12 cm in largest dimension Average 6 cm 12 10 8 6 4 2 0 1 3 5 7 9 11 13 15 17 19 21 23 25 27 29 31 33 35 37 39 41 43 45 47 49 51 53 55 57 59 61 63 65 67 69 71 73 75 77 79 81 83 85 87 89 91 Size Linear (Size ) 2 per. Mov. Avg. (Size )
Relation of size to outcome Mortality increases with size, good recovery is not related to size,?? Other factors are operating 12 11 Good Morbidity Mortality 11 12 10 10 8 8 7 7 6 6 5 5 6 4 4 2 0 Good 1 2 3 4 5 Mortality
Size Relation to % total score Size effect on total score 100 90 80 70 60 50 40 30 20 10 0 cases
Prognostic classification of CCx Lesions Size Level Site MRI features Relation to neurovascular structures Respiratory and cranial nerve deficits Level Cervicocranial 1 Craniocervical 2 Extensive 3
Relation of level to outcome score Only extensive lesions are important predictors of outcome 60 level number Averagge score 50 48 40 30 24 20 15 15.5 20 17.5 10 0 1 2 3 4 Cr- Cx Cx- Cr Extensive e
Prognostic classification of CCT Size Level Site MRI features Relation to neurovascular structures Respiratory and cranial nerve deficits Site Extradural 1 Intradural extraparenchymal 2 Intradural and extradural 3 Intradural intraparenchymal 4
Dural based extraparenchymal Intradural & Intraprenchymal
Infiltrating Meningiomas en plaque
Relation of Site to total score intradural extraneural lesions have better prognosis number average score 60 54 50 40 30 24 20 12.6 14 16.6 15.9 10 0 0 0 ED 1 ID-EM 2 ID & ED 3 ID-IM 4
Prognostic classification of CCT Size Level Site MRI features Relation to neurovascular structures Respiratory and cranial nerve deficits MRI features Cystic 1 Solid 2 Mixed intensity 3
Chiari & Syringomyelia
Relation of MRI to total score Mixed MRI intensity increases the risk of morbidity & mortality number Average Score 60 52 50 40 30 24 20 16 12.5 12.7 18 10 0 Cystic Solid Mixed density
Prognostic classification of CCx Lesions Size Level Site MRI features Relation to neurovascular structures Respiratory and cranial nerve deficits Respiratory & cranial nerve deficit Absent 0 Mild 1 Moderate 2 Severe 3
Meningioma, Preop Post op e vascularized muscle fat graft
Post op CT scan, bone resection
Cranial nerve & Ry affection Lower cranial nerve deficits are common & affect respiratory functions in about 17% of pts Retrospective Prospective 30 25 20 18 15 14 8 10 12 5 6 8 8 8 8 0 0 0 2 II III, IV, VI V, VII, VIII IX, X, XI XII Respiratory
Prognostic classification of CCx Lesions Size Level Site MRI features Relation to neurovascular structures Respiratory and cranial nerve deficits Relation to neurovascular structure Posterior 1 Posterolateral or lateral 2 Medial 3 Anterior 4 All 5
Neurofibromatosis lower CNs
Relation to NV structures & score Medial, Anterior or extensive lesions increases difficulty Number Average Score 40 38 35 30 25 20 15 13.5 10.6 14.4 14 14 16 18 18.4 10 8 5 0 Posterior 1 PL or Lat 2 Medial 3 Anterior 4 All 5
A. Rhoton. Neurosurgery, Vol. 47, No. 3, September 2000 Supplement
Neurofibroma & Meningioma After bilateral resection
Relative weight of each category on score Chart Title 45 40 35 30 25 20 15 10 5 0 1 4 7 10 13 16 19 22 25 28 31 34 37 40 43 46 49 52 55 58 61 64 67 70 73 76 79 82 85 88 91 94 Series3 Series5 Series7 Series9 Series11 Series13 Series15
Total score Total Score distribution Score Distribution 25 20 CN's & Ry 15 NVS 10 5 MRI Location Site Size 0 1 4 7 10 13 16 19 22 25 28 31 34 37 40 43 cases 46 CN's & Ry NVS MRI Location Site Size
Score Prognostic Classifications CCxT Histopathology Prognostic difficulty in 92 CCxT Total score, 20 Total score, 19 Total score, 18 Total score, 17 Total score, 16 Total score, 15 Total score, 14 Total score, Total score, Total 13 score, 13 13 Total score, Total score, 11 11 Total score, 20 Total score, 9 Total score, 16 Total score, 15 Total score, 13 Total score, Total score, 12 12 Total score, Total score, 11 11 Total score, 11 Total score, Total score, 10 10 Total score, 10 Total score, 21 Total score, 20 Total score, 9 Total score, 16 Total score, 19 Total score, Total score, 12 Total score, 12 12 Total score, 16 Total score, Total score, 15 15 Total score, Total score, 17 17 Total score, 14 Total score, 16 Total score, 13 Total score, 11 Total score, Total score, 19 19 Total score, 14 Total score, 12 Histopatholoy * Total score
Results Meningiomas were more anterior while neurofibromas tended to be more posterior. This may explain the better results of neurofibromas compared to meningiomas in published series.
Mortality 10/92 4/26 retrospective, 6/66 prospective Four patients died of reoperation in the retrospective group, all had a high score > 18. Two patient re-operated for CCx intradural intramedullary ependymoma & chordoma recurrence after 2 & 3 previous surgeries, died 1 & 3 week after surgery of respiratory failure and aspiration due to lower CNs insults & medullary insult. Two more patients died after reoperation for a grade II glioma & a meningioma 2 & 4 years after initial partial resection. Both had a high score All had limited surgeries for pathological confirmation at first surgery
Changing paradigm More radical resections were performed in the retrospective patients reflecting the traditional importance of radical resection Less mortality in the prospective group was associated e less radical resections. Residual lesions close to neurovascular structures were subjected to radiosurgery
Mortality 10/92 4/26 retrospective, 6/66 prospective Six patients died of aspiration, pulmonary embolism & MI in prospective group 6/66. Two patients e grade II glioma are still surviving 5 & 7 years after staged bony decompression followed 3 weeks & 4 months later by partial resection & dural widening. Both had a high score above 18 and were considered as a bad surgical candidate before the first surgery. Residual benign tumors recently received radiosurgery
Morbidity
Discussion The system thus utilizes a mixture of radiological, clinical and anatomical data that will help to anticipate difficulties during the proposed surgical procedure to resect the tumor. Small posterior cervicocranial extradural cystic tumor in a patient with no or mild respiratory and cranial nerve involvement (score= 0-6) will present minimal surgical difficulties; and carries the best prognosis.
Discussion A large extensive intramedullary craniocervical solid anterior tumor encircling vessels in a patient with marked respiratory compromise & severe multiple lower cranial nerve palsies will present the greatest challenge during surgical extirpation. This patient will have a poor surgical prognosis. A residual benign lesion attached to neurovascular bundle may be subjected to radiosurgery
Discussion Such a lesion may require more than one special craniocervical skull base approach in order to achieve gross total resection without jeopardizing the patient survival and neurological condition. It has a much worse prognosis for smooth postoperative recovery.
Discussion In patients with diffuse infiltrative intraparenchymal solid lesions simple decompression of posterior fossa and upper cervical laminectomy may enable them to enjoy a more prolonged survival for alternative palliative therapeutic modalities to be applied such as stereotactic radiosurgery or combination chemotherapy protocols.
Conclusion Relation to neurovascular structures, lesion size larger than 3 cm diameter, extensive lesions, intradural intra-parenchymal lesions, mixed intensity lesions on MRI & site are detrimental in estimating the expected surgical difficulties and expected outcome in surgery of CCx lesions.
Conclusion Classifying CCx tumors as extradural, extramedullary or intramedullary does not account for critical anatomical neurovascular structures at the foramen magnum which are crucial in anticipating surgical difficulties. The scored prognostic classification proposed here identifies risks involved during surgical management of CCx lesions.
Recommendation Framless stereotaxy and virtual reality simulation of operative approach may define more safe trajectories than standardized approaches. Fixation if instability. Endovascular approaches are advantageous Radiosurgery may be used for residual lesions A tailored surgical approach must be used for each patient to avoid the dreadful complications of CCx lesions.
Mohamed El-Fiki, M.D.
a comprehensive approach to tumors of the brain and surrounding structures. It is unique in its multidisciplinary, collaborative patientcentered philosophy that keeps the patient and treating physicians constantly in touch with the diagnosis, treatment and progress as complex care is rendered. Each case must be discussed in detail attended by all treating physicians and other multidisciplinary team members involved in patient management It is necessary that the physicians involved in the care of people with this condition comprehensively approach the management and follow-up of the patients, and that they organize interdisciplinary teams including all the professionals that can help to increase the quality of life of patients.
Prognostic Classification of 13 CCT No Pathology Size * Level * Site * MRI * NVS * Ry & * T Cm CN 1 Mening 5 4 CR-CX 2 ID-EM 2 Solid 2 Ant 4 Mod 2 16 2 Neurofib 4 4 CR-CX 2 ID-ED 3 Solid 2 PL 2 Sev 3 16 3 Neurofib 2.5 3 CR-CX 2 ID-EM 2 Solid 2 PL 2 Mod 2 13 4 Glioma 12 4 CX-CR 1 ID-IM 4 Mixed 3 All 5 Sev 3 20 5 Glioma 7 4 CX-CR 1 ID-IM 4 Mixed 3 All 5 Mod 2 19 6 Glioma 3 3 CX-CR 1 ID-IM 4 Cystic 1 All 5 Mild 1 15 7 Mening 8 4 CR-CX 2 ID-EM 2 Mixed 3 All 5 Mod 2 18 8 Neurofib 4 4 CX-CR 1 ID-ED 2 Mixed 3 Lat 2 Mild 1 13 9 Mening 9 4 CX-CR 1 ID-EM 2 Solid 2 Lat 2 Mild 1 12 10 Ependymo 4 4 EXTEN 3 ID-IM 4 Mixed 3 PL 2 Mild 2 18 11 Neurofib 4 4 CX-CR 1 ID-EM 2 Solid 2 PL 2 Non 0 11 12 Chordoma 5 4 EXTEN 3 ED 1 Mixed 3 Ant 4 Mild 2 17 13 Neurofib 6 4 CR-CX 3 ID-EM 2 Solid 2 PL 2 Mild 2 17 *: Score T: Total Score
total score Prognostic score & pathology Size score of individual pathological types Total score diff pathology 25 20 15 10 5 0 pathology mening mening mening mening mening mening mening mening mening mening mening mening mening NF NF NF NF NF NF NF NF NF NF NF NF NF Glioma Glioma Glioma Glioma Glioma Glioma Ependymoma Ependymoma Ependymoma Ependymoma Ependymoma Chordoma Chordoma Epidermoid Epdermoid Granuloma Granuloma Mets Mets aneurysm
total score Size score & pathology Size score of individual pathological types Total score diff pathology 25 20 15 10 5 0 pathology mening mening mening mening mening mening mening mening mening mening mening mening mening NF NF NF NF NF NF NF NF NF NF NF NF NF Glioma Glioma Glioma Glioma Glioma Glioma Ependymoma Ependymoma Ependymoma Ependymoma Ependymoma Chordoma Chordoma Epidermoid Epdermoid Granuloma Granuloma Mets Mets aneurysm
Prognostic score & pathology 25 20 15 10 5 0 Total socre Mening Mening Mening Mening Mening Mening Mening mening Mening Mening Mening Mening Mening Neurofibroma Neurofibroma Neurofibroma Neurofibroma Neurofibroma Neurofibroma Neurofibroma neurofibroma Neurofibroma Neurofibroma Neurofibroma AACNS, Neurofibroma WANS 2015, Jeju Island, Neurofibroma Korea Prognostic Glioma classification of CCxT, M. Glioma El-Fiki Glioma Glioma Glioma Glioma
Size score in different lesions 3.5 3 3 3 3 3 3 3 3 3 3 3 3 3 3 3 3 3 3 2.5 2 2 2 2 2 2 2 2 2 2 2 2 2 2 2 2 2 2 1.5 1 1 1 1 1 1 1 1 1 1 1 1 1 0.5 0 * Mening Mening Mening Mening Mening Mening Mening Mening Mening Mening Mening Mening Mening NF NF NF NF NF NF NF NF NF NF NF NF NF Glioma Glioma Glioma Glioma Glioma Glioma Ependymoma ependymoma Ependymoma ependymoma Ependymoma chordoma chordoma Epidermoid Epidermoid granuloma granuloma Mets Mets aneurysm
Score Category contribution in score Contribution of each category in total score category weight in total score 100% 80% Size 60% Level Site 40% 20% Total socre MRI 0% 1 3 5 7 9 11 13 15 17 19 21 23 25 27 29 31 33 35 37 39 41 43 45 47 CN's NVS Cases Size Level Site MRI NVS's CN's & Ry
MRI (Cystic, solid or mixed) 3 Radiological features in CCx in tumors 92 CCx tumors MRI Score 2.5 3 2 MRI 2 1.5 1 1 0 0.5 1 0 1 Pathology Pathology Mening Mening Mening Mening Mening Mening Mening Mening Mening Mening Mening Mening Mening NF Mening NF MeningNF Mening NF Mening NF NF Mening NF Mening NF MeningNF Mening NF Mening NF Mening Mening Mening NF NF NF NF NF NF NF NF NF NF NF GliomaNF Glioma NF Glioma NF Glioma Glioma NF Glioma Glioma EpendyGlioma Ependy Glioma Ependy Glioma Ependy Glioma Glioma Ependy Ependy Ependy Ependy Ependy Chordoma Chordoma Epidermoid Ependy Epidermoid Chordoma Granuloma Chordoma Granuloma Epidermoid MetsEpidermoid Mets Granuloma Granuloma aneurysm MetsSeries47 Mets aneurysm Series47
Prognostic score & pathology 45 Series1 Series2 Series3 Series4 Series5 Series6 Series7 Series8 Series9 Series10 Series11 Series12 Series13 Series14 Series15 Series16 40 35 30 25 20 15 10 5 0
Prognostic score & pathology Histopathology Size Score Level Score Site Score MRI Score NVS Score CNs & Ry Score 5 4.5 4 3.5 3 2.5 2 1.5 1 0.5 0 1 3 5 7 9 1113 15 1719 21 23 2527 29 3133 35 373941 4345 474951 535557 59 6163 65 6769 71 73 7577 79 81 8385 87 8991 93 Score NVS Score Level Histopathology
Diffuse Brain stem Lesions
Cranio-Cx subluxation