1. HAVE CRANIO-VERTEBRAL JUNCTION ANOMALIES BEEN OVERLOOKED AS A CAUSE OF VERTEBRO-BASILAR INSUFFICIENCY? Deepak Agrawal, Naveen K*, SS kale, C S Bal*, A K Mahapatra Departments of Neurosurgery and *Nuclear medicine, All India Institute of Medical Sciences, New Delhi-110029

2. BACKGROUND association of VBI with CVJ anomalies is severely underestimated association of VBI with CVJ anomalies is severely underestimated x-rays of the Cx spine are done in only 30% of pts with VBI & only 11% pts have proper flex n /ext n x-rays done x-rays of the Cx spine are done in only 30% of pts with VBI & only 11% pts have proper flex n /ext n x-rays done Lorenstan KJ, Schrospshire LC, Ahn HS. Congenital odontoid aplasia and posterior circulation stroke in childhood. Ann Neurol 1988;23-410-413

3. BACKGROUND posterior circulation ischemia has a higher morbidity and mortality posterior circulation ischemia has a higher morbidity and mortality Fifty percent of these patients who are managed conservatively progress to develop infarction Fifty percent of these patients who are managed conservatively progress to develop infarction

4. BACKGROUND Diagnosing even a percentage of the patients with VBI as having CVJ anomalies may have major therapeutic & prognostic implications. Diagnosing even a percentage of the patients with VBI as having CVJ anomalies may have major therapeutic & prognostic implications.

5. Aims and Objectives: Using 99Tc ECD brain SPECT to document the presence of posterior circulation cerebral ischemia in patients with CVJ anomalies and correlate with symptoms of VBI. Using 99Tc ECD brain SPECT to document the presence of posterior circulation cerebral ischemia in patients with CVJ anomalies and correlate with symptoms of VBI.

6. PROSPECTIVE STUDY DONE OVER A SIX MONTH PERIOD PROSPECTIVE STUDY DONE OVER A SIX MONTH PERIOD

7. STUDY DESIGN 19 PATIENTS WITH FIXED AAD Clinical assessment & Brain SPECT on admission CONTROL GROUP (7 PTS) TOO + PF Rpt SPECT at 4 weeks VBI GROUP (12 PTS) TOO + PF Rpt SPECT at 4 weeks

8. VBI GROUP Pts with features suggestive of VBI (Drop attacks, episodic vertigo, visual disturbances and dysarthria) Pts with features suggestive of VBI (Drop attacks, episodic vertigo, visual disturbances and dysarthria) CONTROL GROUP patients without symptoms of VBI patients without symptoms of VBI

9. Operative procedure combined TOO and Occipito-cervical fusion from occiput to C3, using contoured loop and sublaminar wiring with bone graft placement. combined TOO and Occipito-cervical fusion from occiput to C3, using contoured loop and sublaminar wiring with bone graft placement. Both procedures were carried out consecutively in a single sitting. Both procedures were carried out consecutively in a single sitting.

10. Patients with reducible AAD, requiring only occipito-cervical fusion were excluded from the study to maintain uniformity. Patients with reducible AAD, requiring only occipito-cervical fusion were excluded from the study to maintain uniformity. Postoperatively the neck was immobilized using a philadelphia collar for a period of three months. Postoperatively the neck was immobilized using a philadelphia collar for a period of three months.

11. SPECT scanning was done using 99Tcm- ECD on a dual headed GE 'Varicam' scanner. The final data was displayed on a 10 grade color scale and semi quantitative analysis performed. The final data was displayed on a 10 grade color scale and semi quantitative analysis performed. SPECT

12. Regional cerebellar perfusion <10% of contralateral lobe, or in case of bilateral involvement, less than 20% of basal ganglia ABNORMAL SPECT SCAN

13. OBSERVATIONSRadiology AAD19 AAD19 BI15 BI15 Occipitalisation of atlas14 Occipitalisation of atlas14 kippel-feil anomaly9 kippel-feil anomaly9 cerebellar infarcts2 cerebellar infarcts2 (Both in VBI group)

14. OBSERVATIONS (VBI Group) Clinical features Vertigo and drop attacks10 Vertigo and drop attacks10 Incoordination8 Incoordination8 visual symptoms4 visual symptoms4

15. RESULTS (Preoperative SPECT) Decreased cerebellar perfusion in 75% (n=9) of the patients in the VBI group compared to 14% (n=1) in the control group Decreased cerebellar perfusion in 75% (n=9) of the patients in the VBI group compared to 14% (n=1) in the control group (p=0.019, fischer exact, 2 tailed).

17. RESULTS Following surgery, five patients (55%) in the symptomatic group and none in the control group had improvement in cerebellar perfusion. Following surgery, five patients (55%) in the symptomatic group and none in the control group had improvement in cerebellar perfusion. All five patients showing improvement on SPECT also had improvement in their symptoms of VBI following surgery All five patients showing improvement on SPECT also had improvement in their symptoms of VBI following surgery

18. Two pts in VBI group developed meningitis in the postoperative period & had a further decrease in cerebellar perfusion on the follow up SPECT scan. Two pts in VBI group developed meningitis in the postoperative period & had a further decrease in cerebellar perfusion on the follow up SPECT scan. Another 2 pts in VBI group had cerebellar infarcts on MRI & did not show improvement in cerebellar hypoperfusion following surgery. Another 2 pts in VBI group had cerebellar infarcts on MRI & did not show improvement in cerebellar hypoperfusion following surgery.

19. Pathogenesis VBI in CVJ Anomalies Chronic low grade micro-trauma Rptd flex/extn of vessel Intimal damage Thrombosis Embolisation

20. Clinical rarity of posterior circulation infarcts in CVJ anomalies could be due to: Duplication of VA and the adequacy of the circulation of Willis Duplication of VA and the adequacy of the circulation of Willis patients symptomatic for VBI are not routinely evaluated for CVJ anomalies which remain undiagnosed patients symptomatic for VBI are not routinely evaluated for CVJ anomalies which remain undiagnosed

21. In patients with CVJ anomalies currently used imaging modalities such as cervical spine x-rays and CT scans are not adequate to evaluate for vertebro-basilar ischemia In patients with CVJ anomalies currently used imaging modalities such as cervical spine x-rays and CT scans are not adequate to evaluate for vertebro-basilar ischemia

22. Ours is the only study of its kind documenting hypoperfusion in posterior circulation territory in patients with CVJ anomalies. Ours is the only study of its kind documenting hypoperfusion in posterior circulation territory in patients with CVJ anomalies. This hypoperfusion may represent a state of chronic VBI, expected in such patients & MAY BE REVERSIBLE following surgery. This hypoperfusion may represent a state of chronic VBI, expected in such patients & MAY BE REVERSIBLE following surgery.

23. CONCLUSIONS Our study shows that rigid immobilisation (provided by occipito-cervical fusion in our patients) by itself may confer protection from VBI and direct repair of VA may not be necessary Our study shows that rigid immobilisation (provided by occipito-cervical fusion in our patients) by itself may confer protection from VBI and direct repair of VA may not be necessary

24. CONCLUSION We strongly recommend that in patients of VBI, a high index of suspicion should be kept for CVJ anomalies and x-rays of the cervical spine with flexion-extension views be done on all patients. We strongly recommend that in patients of VBI, a high index of suspicion should be kept for CVJ anomalies and x-rays of the cervical spine with flexion-extension views be done on all patients.

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