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 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 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. 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. 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- 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 craniocervical 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) 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 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.