1. A. Atiq Durrani, MD
Center For Advanced Spine Technologies
Cincinnati, OH

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. 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

5. NECK PAIN

6. Symptoms

7. Symptoms

8. Symptoms

9. Symptoms

10. Symptoms

11. Dx Tests

12. 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

13. Measurements

16. Subluxation of C2 on C3

17. Cranial Settling or Chiari Malformation

18. 3D CT Neutral

19. 3D CT Left

20. 3D CT Right

21. % 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.

22. Treatment
Asymptomatic- Observation

23. Treatment
 Symptomatic
 Physical Therapy-
 Cranio-Sacral Alignment.
 Cervical Collar.

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

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

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

27. KH Pre-Op

31. KH 1 yr Post-Op

34. JM Pre-Op

38. JM 1 yr Post-Op

41. Outcomes

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

43. 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%.

44. 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.

45. Pain at Best

46. Pain at Worst

47. Pain on Average

49. NEWMAN

50. NEWMAN

52. 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)

53. 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)

54. 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)

55. 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

56. 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.