Cervical Instability in the EDS Population€¦ · Cervical Instability in the EDS Population...

EDNF 2012 Conference August 2012

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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, C5-‐6).

� Chiari Malformation

SYMPTOMS



Diagnosing C1-‐C2 Instability

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

NECK PAIN



Symptoms

Symptoms

Symptoms



Symptoms

Symptoms

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

Measurements



SubluxaFon of C2 on C3

Cranial SeIling or Chiari MalformaFon



3D CT Neutral

3D CT LeL

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.

Treatment

Asymptomatic-‐ Observation

Treatment � Symptomatic

� Physical Therapy-‐ � Cranio-‐Sacral Alignment. � Cervical Collar.



Treatment

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

Treatment

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

SymptomaFc C1-‐C2 instability in EDS paFents 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



Outcomes



Outcomes

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

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%.

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.



Pain at Best

Pain at Worst

Pain on Average



NEWMAN

NEWMAN



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 (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)

ComplicaFons � 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

ComplicaFons 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.

Cervical Instability in the EDS Population€¦ · Cervical Instability in the EDS Population...

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