1. Cervical Instability in the EDS Population
EDNF 2012 Conference
August 2012
Cervical Instability in the EDS Population
A. Atiq Durrani, MD
Center For Advanced Spine Technologies
Cincinnati, OH
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2. Cervical Spine Issues in EDS
C1-C2 instability
Cranio-cervical Instability.
Lower Cervical kyphosis.
Cervical disc degeneration ( Most common at C4-5, C5-6).
Chiari Malformation

3. SYMPTOMS

4. Diagnosing C1-C2 Instability

5. Common Symptoms of Cervical Instability
Occipital headaches
Neck pain
Passing out at the extremes of lateral rotation
Choking sensations
Base of skull tenderness
Jaw pain

6. NECK PAIN

7. Symptoms

8. Symptoms

9. Symptoms

10. Symptoms

11. Symptoms

12. Dx Tests

13. MRI with Flexion and Extension
What we look for:
Cleido-clivus angle
Grubbs Oak distance
Distance between clivus and dens
Cranial settling/Chiari malformation
Subluxation of vertebrae

14. Measurements

17. Subluxation of C2 on C3

18. Cranial Settling or Chiari Malformation

19. 3D CT Neutral

20. 3D CT Left

21. 3D CT Right

22. % uncovering of facets Blue line measures the C2 facet.
EDNF 2012 Conference
August 2012
% uncovering of facets
Blue line measures the C2 facet.
Green line measures the amount of C1 facet that covers C2.
With these numbers, % uncovered can be calculated.
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23. Asymptomatic- Observation
Treatment
Asymptomatic- Observation

24. Treatment Symptomatic Physical Therapy- Cranio-Sacral Alignment.
Cervical Collar.

25. Treatment Interventional Pain Procedures. Occipital nerve Blocks
Cervical Epidural / Foraminal Injections.

26. Treatment
If Conservative means fail to control symptoms , then Cervical Spinal Fusion is the preferred Surgical Treatment.

27. Symptomatic C1-C2 instability in EDS patients can be surgically treated with a C1-C2 fusion

28. KH Pre-Op

32. KH 1 yr Post-Op

35. JM Pre-Op

39. JM 1 yr Post-Op

42. Outcomes

43. Outcomes Between 1/2009 and 8/2011, N= 25. 1 year follow up.
All patients underwent stabilization for C1-C2.

44. Outcomes. Mean Pre-op Pain – 8 Mean post op pain at one year- 2
One patient still had residual pain.
Screw fracture in one patient.
Headaches resolved in 92% of patients.
Will you do this procedure again – 95%.

45. Conclusion
Cervical Spinal Instability is a common reason for EDS patients suffering from headaches and Cranio-Cervical pain.
It is under- appreciated by the spine community and not very well understood.
In many circumstances, patients complaining of such complaints go through extensive work up with no treatment offered in the end.
Stabilization of O-C1-C2, complex resolves cranio-cervical symptoms in EDS patients.

46. Pain at Best

47. Pain at Worst

48. Pain on Average

50. NEWMAN

51. NEWMAN

53. Classical type (formerly Types I and II) EDS
Signs and symptoms include:
Loose joints
Highly elastic, velvety skin
Fragile skin that bruises or tears easily
Redundant skin folds, such as on the eyelids
Slow and poor wound healing leading to wide scarring
Noncancerous fibrous growths on pressure areas, such as elbows and knees; fatty growths on the shins and forearms
Muscle fatigue and pain
Heart valve problems (mitral valve prolapse and aortic root dilation)

54. Hypermobility type (formerly type III) EDS
Signs and symptoms include:
Loose, unstable joints with many dislocations
Easy bruising
Muscle fatigue and pain
Chronic degenerative joint disease
Advanced premature osteoarthritis with chronic pain
Heart valve problems (mitral valve prolapse and aortic root dilation)

55. Vascular type (formerly type IV) EDS
This type of EDS is rare, but it's one of the most serious. It affects an estimated 1 in 100,000 to 200,000 people. Signs and symptoms include:
Fragile blood vessels and organs that are prone to tearing (rupture)
Thin, translucent skin that bruises easily
Characteristic facial appearance, including protruding eyes, thin nose and lips, sunken cheeks and small chin
Collapsed lung (pneumothorax)
Heart valve problems (mitral valve prolapse and others)

56. Complications
Depend on your symptoms and type of EDS, but some common ones include:
Prominent scarring
Difficulty with surgical wounds — stitches may tear out, or healing may be incomplete
Chronic joint pain
Joint dislocation
Early onset arthritis
Premature aging with sun exposure

57. Complications with Vascular EDS
Serious complications can arise with vascular EDS such as
Tearing (rupture) of major blood vessels,
i.e., ruptured or dissected artery or an aneurysm,
rupture of organs, such as the intestines or uterus.
These complications can be fatal. About 1 in 4 people with vascular type EDS develop a significant health problem by age 20, and more than 80 percent develop complications by age 40. The median age of death is 48 years.