The management of chronic pain in patients diagnosed with Ehlers Danlos Syndrome (EDS) is complex and requires a multimodal approach (Chopra, 2015).  Common types of pain reported include joint, neuropathic, headaches/orofacial, pelvic and gastrointestinal (Chopra et al., 2017).  Often times, these symptoms are heightened and perpetuated by the presence of myofascial trigger points.  It has been proposed that the characteristic taut bands within trigger points are created to counteract joint instability (Levy et al., 2004).  Due to the widespread hypermobility, present in patients with EDS, it is warranted to assess myofascial dysfunction as part of a comprehensive treatment plan.  Particularly, when evaluating patients with the impairments mentioned below or a secondary diagnosis.

Thoracic Outlet Syndrome

Due to numbness, tingling, paresthesia and pain in the extremities, patients with EDS will often times be diagnosed with Thoracic Outlet Syndrome (TOS). While the presence of of neuropathic pain is well documented in this patient population, the mechanism is not completely clear (Camerota et al., 2011).   Hypermobility of the shoulder girdle and secondary myofascial trigger points may play a direct role in brachial plexus irritation (Langley, 1997).  This could add direct compression to neurovascular structures such as the pectoralis minor and scalenes; As well, it could add nociceptive input and referred pain as in the case of the infraspinatus, subscapularis and latissimus dorsi (Qerama et al., 2009).  When treating EDS patients, for TOS symptoms, it is often imperative to treat the involved trigger points, followed with proper proprioceptive and stability training.  Treating the affected areas will help reduce peripheral nociception and better balance the scapula from placing traction on the brachial plexus.

First Carpometacarpal Instability

Thumb pain is often attributed to instability of the first carpometacarpal (CMC) joint in hypermobile patients (Chopra, 2015).  While hypermobility is a concern, there are several myofascial trigger points within extrinsic and intrinsic muscles of thumb that can contribute to these symptoms.  Clinically, when splinting has failed to reduce pain, the abductor pollicis longus and extensor pollicis longus/brevis should be examined for trigger points (Hwang et al., 2005).  These extrinsic muscles are at a mechanical disadvantage for fine motor control and often harbor latent, and sometimes active trigger points.  Additionally, intrinsic muscles including the adductor pollicis and opponens pollicis should also be considered for dysfunction, if pain persists.

Postural Orthostatic Tachycardia Syndrome

One of the most common comorbidities of patients with EDS is Postural Orthostatic Tachycardia Syndrome (Miglis et al., 2017).  POTS is a form of dysautonomia affecting the cardiovascular system which results in dizziness, fatigue, fainting and inability to exercise.  The management of the disorder usually involves a combination of medication and progressive exercise program (Wells et al., 2018).  However, the interplay between trigeminal afferents and the vestibular system provides rationale for assessing myofascial trigger points in patients with severe dizziness (Marano et al., 2005).  Specifically, trigger points that relay information through the trigeminal nucleus should be evaluated.  This would include the sternocleidomastoid (SCM), masseter, upper trapezius, and suboccipitals, at a minimum.  Clinically, if patients are diagnosed with POTS and not responding to therapeutic exercise due to extreme dizziness, it is worth assessing for trigger points in the upper quadrant.

 Lateral Ankle Instability

Impaired proprioception and frequent inversion sprains can lead to lateral ankle instability, in patients with EDS (Levy et al., 2014).  If patients have persistent pain after bracing and traditional stability training, it should be advised to examine the muscles that are subject to repetitive eccentric stress across the subtalar and talocrural joints.  In particular, the fibularis longus/brevis have been found to be impaired ,in strength and neuromuscular function, in patients with chronic ankle instability (Cho et al., 2017).  Treating trigger points, in these muscles, can not only reduce referred pain, but also allow for more efficient recruitment, during stabilization tasks.  In regards to the pain perception, in the area of the lateral ankle ligaments, the extensor hallucis brevis (EHB) is another muscle that crosses the subtalar joint which can harbor active trigger points.  Treatment of the EHB and fibularis, in conjunction with proprioceptive training, should be considered before surgical management of the ligamenouts structures.

 Conclusions

Myofascial pain is a commonly overlooked source of pain and dysfunction in patients with EDS.  At Bethesda Physiocare our physical therapists specialize in the evaluation and treatment of all things muscle and fascial dysfunction.  This involves a variety of manual therapy and needling techniques in conjunction with a detailed biomechanical assessment.  If you are hypermobile and suffering from chronic pain, consider making an appointment with one of our expertly trained clinicians! 

—Dr. Nathan Mayberry, PT, DPT, OCS, CMTPT, CSCS, PHC, CertDxUS