The Spine in Ehlers-Danlos Syndromes

What it is

The Ehlers-Danlos syndromes (EDS) are a group of thirteen inherited conditions of connective tissue. The common thread is collagen that is built or maintained differently, which makes joints looser and tissues more stretchy or fragile. The spine, a column of joints held together by ligaments, feels the effect. Neck and back pain, scoliosis, a rounded upper back, and a sense that the neck or back is "unstable" are all common. This guide is about the spine. For the syndromes as a whole and how they are diagnosed, see my guide to Ehlers-Danlos syndromes.

Which type matters. Hypermobile EDS (hEDS) is by far the most common and has the mildest effect on the spine. Classical EDS is similar. Kyphoscoliotic EDS is rare and different: it causes a severe, early, progressive curve. Vascular EDS carries a risk to blood vessels and organs that changes every surgical decision.

How it shows up

• Neck and back pain that comes and goes, often worse after being still or after overdoing it, and often out of proportion to what the X-ray or MRI shows.
• Scoliosis, usually mild to moderate and more flexible than idiopathic scoliosis. Severe, early curves point toward the kyphoscoliotic type.
• Headaches, dizziness, and a feeling of a "heavy head," which can come from neck muscles, from blood pressure and heart rate changes on standing (dysautonomia), or both.
• Easy bruising, slow-healing scars, and joints that slip.

How we measure and follow it

The exam looks at joint laxity, posture, muscle strength, and the nerves. Standing X-rays measure any curve. An MRI is used when the exam or symptoms call for it, not routinely. When neck instability is a concern, I use upright X-rays taken in flexion and extension, and sometimes a specialized MRI, and I read them conservatively: a few millimeters of extra motion in a loose-jointed person is usually normal for that person. True structural instability of the neck is uncommon in hEDS. I say that plainly, because the fear of it causes a great deal of worry.

How we treat it

Nearly everyone with EDS and spine symptoms is treated without surgery, and most do well.

• Strength is the brace. Joints that are loose need muscles that are strong. A physical therapist who understands hypermobility builds a slow, graded program for the deep neck and trunk muscles, the hips, and the shoulder blades, and teaches pacing so that flare-ups do not derail progress. Pilates-style and scoliosis-specific exercise fit well.
• Pain care that calms the nervous system. Long-standing pain in EDS often involves nerves that have become oversensitive. Sleep, steady activity, treating anxiety or low mood, and, for some, nerve-calming medicines help more than strong painkillers. I avoid long-term opioids.
• Bracing, sparingly. A scoliosis brace can be used in a growing child with a moderate curve. A soft neck collar can help during a flare, but living in one weakens the very muscles that protect the neck, so it is a short-term tool.
• Treating what travels with EDS. Dysautonomia (POTS), mast cell symptoms, digestive problems, and sleep problems all turn up pain. Treating them is part of treating the spine.
• Surgery, when it is truly needed. A large or progressing curve, especially in the kyphoscoliotic type, a nerve that is clearly compressed, or proven instability can call for surgery. The operation is planned around the tissue: careful handling, a plan for bleeding, a lower threshold to use more anchor points, and attention to wound healing. In vascular EDS, surgery is avoided whenever possible and done only with a vascular team.

What many families have not heard yet

• Hypermobility is not the same as instability. Most people with hEDS and neck symptoms have muscular and autonomic pain, not a loose skull or spine. The internet is full of frightening stories about neck instability and fusion. Fusion of the top of the neck is rarely needed, and it is a serious operation with lasting effects on motion, so I reserve it for proven cases.
• Local anesthetic may wear off faster. Many people with hEDS report that numbing shots are less effective or shorter-acting. I believe you. We plan dental and office procedures, and anesthesia for surgery, with that in mind.
• Loose joints do not mean "do not exercise." The opposite is true. Inactivity makes hypermobile joints more painful. Exercise has to be graded and consistent, but it is the single most effective treatment there is.
• A curve in EDS may be more flexible than it looks. On bending X-rays, EDS curves often correct more than idiopathic curves of the same size. That can make the surgical plan shorter if surgery is ever needed.
• The rare types are the ones to rule out. There is no gene test for hEDS, but there are tests for the others. Confirming that someone does not have vascular, kyphoscoliotic, or classical EDS, or Marfan or Loeys-Dietz syndrome, is the most useful test in the workup.
• Scars and wound healing need planning. In EDS, incisions can stretch and widen. For any surgery, I use techniques that take tension off the skin, and we leave stitches in longer.
• Tethered cord and Chiari-type findings are debated. Some people with EDS are told their symptoms come from a tethered spinal cord that does not show on MRI, or from a slightly low-lying cerebellum. The evidence for operating on these findings is weak. I will give you an honest reading of your imaging, and I will not recommend a surgery the evidence does not support.
• Pregnancy and the spine. Loose ligaments loosen further in pregnancy. Back and pelvic pain are common and usually treatable with therapy and a support belt. Anesthesia for delivery deserves a conversation in advance.

The science: for the curious

Collagen is the rope that holds the spine's joints together. In hEDS the ropes are more elastic; in kyphoscoliotic EDS, an enzyme that cross-links collagen is missing, and the ropes are weak, which is why those curves are severe. Pain in EDS is partly mechanical and partly a nervous system that has learned to amplify signals, which is why exercise, sleep, and nervous-system-calming treatments work better than rest. Studies of spine surgery in EDS show more bleeding, more wound problems, and more hardware-related problems than in people without EDS, and no evidence that surgery helps hypermobility-related pain without a clear structural target. That is the basis for the rehabilitation-first approach above.

Learn more

The Ehlers-Danlos Society: ehlers-danlos.com (opens in a new tab)

Scoliosis Research Society, Patient Brochures: srs.org/patients (opens in a new tab)

My guide to Ehlers-Danlos syndromes covers the thirteen types and how each is diagnosed.

This handout is general education and does not replace the personalized advice we discuss in clinic about your specific case. If anything here does not match what we talked about, what we talked about wins: please ask me.