Cervical Instability in the EDS Population

A. Atiq Durrani, MDCenter For Advanced Spine

TechnologiesCincinnati, 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

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 ExtensionWhat we look for:

Cleido-clivus angleGrubbs Oak distanceDistance between clivus and densCranial settling/Chiari malformationSubluxation of vertebrae

Measurements

Subluxation of C2 on C3

Cranial Settling or Chiari Malformation

3D CT Neutral

3D CT Left

3D CT Right

% uncovering of facetsBlue 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

SymptomaticPhysical Therapy- Cranio-Sacral Alignment. Cervical Collar.

Treatment

Interventional Pain Procedures. Occipital nerve BlocksCervical Epidural / Foraminal Injections.

Treatment

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

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- 2One patient still had residual pain. Screw fracture in one patient. Headaches resolved in 92% of patients. Will you do this procedure again – 95%.

ConclusionCervical 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) EDSSigns and symptoms include: Loose jointsHighly elastic, velvety skinFragile skin that bruises or tears easilyRedundant skin folds, such as on the eyelidsSlow and poor wound healing leading to wide scarringNoncancerous fibrous growths on pressure areas, such as

elbows and knees; fatty growths on the shins and forearmsMuscle fatigue and painHeart valve problems (mitral valve prolapse and aortic root

dilation)

Hypermobility type (formerly type III) EDSSigns 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)

ComplicationsDepend 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 EDSSerious complications can arise with

vascular EDS such asTearing (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.