C1-C2 Instability in the EDS Population

A. Atiq Durrani, MD
Center For Advanced Spine Technologies
Cincinnati, OH
Cervical Spine Issues in EDS
 C1-C2 instability
 Cranio-cervical Instability.
 Lower Cervical kyphosis.
 Cervical disc degeneration ( Most common at C4-5,
 Chiari Malformation
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
Dx Tests
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
Subluxation of C2 on C3
Cranial Settling or Chiari Malformation
3D CT Neutral
3D CT Left
3D CT Right
% 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.
Asymptomatic- Observation
 Symptomatic
 Physical Therapy Cranio-Sacral Alignment.
 Cervical Collar.
 Interventional Pain Procedures.
Occipital nerve Blocks
 Cervical Epidural / Foraminal Injections.
 If Conservative means fail to control
symptoms , then Cervical Spinal Fusion is
the preferred Surgical Treatment.
Symptomatic C1-C2 instability in EDS
patients can be surgically treated with a
C1-C2 fusion
KH Pre-Op
KH 1 yr Post-Op
JM Pre-Op
JM 1 yr Post-Op
 Between 1/2009 and 8/2011, N= 25.
 1 year follow up.
 All patients underwent stabilization for C1-C2.
 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%.
 Cervical Spinal Instability is a common reason for EDS
patients suffering from headaches and Cranio-Cervical
 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 craniocervical symptoms in EDS patients.
Pain at Best
Pain at Worst
Pain on Average
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)
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)
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
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)
 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
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

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