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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
BACKGROUND
 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

 Lorenstan KJ, Schrospshire LC, Ahn HS. Congenital odontoid aplasia and
 posterior circulation stroke in childhood. Ann Neurol 1988;23-410-413
BACKGROUND

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

 Diagnosing even a percentage of the
 patients with VBI as having CVJ
 anomalies may have major therapeutic &
 prognostic implications.
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.
 PROSPECTIVE STUDY DONE
 OVER A SIX MONTH PERIOD
STUDY DESIGN

                  19 PATIENTS WITH FIXED AAD

            Clinical assessment & Brain SPECT on admission

             CONTROL GROUP                         VBI GROUP
                 (7 PTS)                            (12 PTS)


 TOO + PF                                                    TOO + PF


        Rpt SPECT at 4 weeks          Rpt SPECT at 4 weeks
VBI GROUP
 Pts with features suggestive of VBI (Drop
  attacks, episodic vertigo, visual
  disturbances and dysarthria)

CONTROL GROUP
 patients without symptoms of VBI
Operative procedure
 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.
 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.
SPECT
 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.
ABNORMAL SPECT SCAN
 Regional cerebellar perfusion <10% of contralateral
 lobe, or in case of bilateral involvement, less than
 20% of basal ganglia
OBSERVATIONS

Radiology
 AAD                         19
 BI                          15
 Occipitalisation of atlas   14
 kippel-feil anomaly         9
 cerebellar infarcts         2
  (Both in VBI group)
OBSERVATIONS (VBI Group)

Clinical features
 Vertigo and drop attacks   10
 Incoordination             8
 visual symptoms            4
10                            9
                         8

                         6


RESULTS
                         4

                    2             1
(Preoperative SPECT)0        Control Gp           VBI Gp

                                      Number of pts

 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).
RESULTS
 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
 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.
Pathogenesis VBI in CVJ Anomalies
        Chronic low grade micro-trauma
        Chronic low grade micro-trauma
            Rptd flex/extn of vessel
            Rptd flex/extn of vessel

                   Intimal damage
                    Intimal damage

           Thrombosis
           Thrombosis

                   Embolisation
                   Embolisation
Clinical rarity of posterior circulation infarcts in
 CVJ anomalies could be due to:

 Duplication of VA and the adequacy of the
 circulation of Willis

 patientssymptomatic for VBI are not
 routinely evaluated for CVJ anomalies
 which remain undiagnosed
 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
 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.
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
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.
THANK YOU

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Cvj

  • 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  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  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.
  • 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.
  • 6.  PROSPECTIVE STUDY DONE OVER A SIX MONTH PERIOD
  • 7. STUDY DESIGN 19 PATIENTS WITH FIXED AAD Clinical assessment & Brain SPECT on admission CONTROL GROUP VBI GROUP (7 PTS) (12 PTS) TOO + PF TOO + PF Rpt SPECT at 4 weeks Rpt SPECT at 4 weeks
  • 8. VBI GROUP  Pts with features suggestive of VBI (Drop attacks, episodic vertigo, visual disturbances and dysarthria) CONTROL GROUP  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.  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.  Postoperatively the neck was immobilized using a philadelphia collar for a period of three months.
  • 11. SPECT 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.
  • 12. ABNORMAL SPECT SCAN Regional cerebellar perfusion <10% of contralateral lobe, or in case of bilateral involvement, less than 20% of basal ganglia
  • 13. OBSERVATIONS Radiology  AAD 19  BI 15  Occipitalisation of atlas 14  kippel-feil anomaly 9  cerebellar infarcts 2 (Both in VBI group)
  • 14. OBSERVATIONS (VBI Group) Clinical features  Vertigo and drop attacks 10  Incoordination 8  visual symptoms 4
  • 15. 10 9 8 6 RESULTS 4 2 1 (Preoperative SPECT)0 Control Gp VBI Gp Number of pts  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).
  • 16.
  • 17. RESULTS  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
  • 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.  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 Chronic low grade micro-trauma Rptd flex/extn of vessel Rptd flex/extn of vessel Intimal damage Intimal damage Thrombosis Thrombosis Embolisation 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  patientssymptomatic 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
  • 22.  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.
  • 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
  • 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.