C1-C2 Instability in the EDS Population

Cervical Instability in the EDS PopulationA. Atiq Durrani, MDCenter For Advanced Spine TechnologiesCincinnati, OH

1August 2012All rights reserved.EDNF 2012 ConferenceCervical Spine Issues in EDSC1-C2 instability Cranio-cervical Instability. Lower Cervical kyphosis. Cervical disc degeneration ( Most common at C4-5, C5-6).Chiari Malformation SYMPTOMS Diagnosing C1-C2 InstabilityCommon Symptoms of Cervical Instability

Occipital headachesNeck painPassing out at the extremes of lateral rotationChoking sensationsBase of skull tendernessJaw 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 Malformation3D 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.

22August 2012All rights reserved.EDNF 2012 ConferenceTreatmentAsymptomatic- Observation Treatment SymptomaticPhysical Therapy- Cranio-Sacral Alignment. Cervical Collar.

TreatmentInterventional 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 fusionKH Pre-Op

KH 1 yr Post-Op

JM Pre-Op

JM 1 yr Post-Op

OutcomesOutcomesBetween 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) 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)

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.