Christopher J. Colloca, DC, Bradley S. Polkinghorn, DC
State of the Art Chiropractic Center, PC,
Phoenix, Ariz, and Faculty,
Postgraduate and Continuing Education Department,
New York Chiropractic College,
Seneca Falls, NY, USA
OBJECTIVE: To discuss 2 patients with Ehlers-Danlos syndrome seeking chiropractic evaluation and management of their disabling musculoskeletal pain and associated disorders.
CLINICAL FEATURES: Two disabled patients diagnosed with Ehlers-Danlos syndrome had spinal pain, including neck and back pain, headache, and extremity pain. Commonalities among these 2 cases included abnormal spinal curvatures (kyphosis and scoliosis), joint hypermobility, and tissue fragility. One patient had postsurgical thoracolumbar spinal fusion (T11-sacrum) for scoliosis and osteoporosis. The other patient had moderate anterior head translation.
INTERVENTION AND OUTCOME: Both patients were treated with mechanical force and manually assisted spinal adjustments delivered to various spinal segments and extremities utilizing an Activator II Adjusting Instrument and Activator Methods Chiropractic Technique. Patients were also given postural advice, stabilization exercises, and postural corrective exercises, as indicated in Chiropractic BioPhysics Technique protocols. Both patients were able to reduce pain and anti-inflammatory medication usage in association with chiropractic care. Significant improvement in self-reported pain and disability as measured by visual analog score, Oswestry Low-Back Disability Index, and Neck Pain Disability Index were reported, and objective improvements in physical examination and spinal alignment were also observed following chiropractic care. Despite these improvements, work disability status remained unchanged in both patients.
CONCLUSION: Chiropractic care may be of benefit to some patients with connective tissue disorders, including Ehlers-Danlos syndrome. Low-force chiropractic adjusting techniques may be a preferred technique of choice in patients with tissue fragility, offering clinicians a viable alternative to traditional chiropractic care in attempting to minimize risks and/or side effects associated with spinal manipulation. Psychosocial issues, including patient desire to return to work, were important factors in work disability status and perceived outcome.
From the Full-Text Article:
The 2 cases presented herein for the first time report chiropractic management of patients with Ehlers-Danlos syndrome. Although neither patient experienced resolution of their condition through chiropractic care, several factors emerge that demonstrate a measure of success of chiropractic management in the cases presented. Most notable were the subjective and objective improvements observed in response to chiropractic care resulting in improved function. Also noteworthy was the fact that both patients were able to significantly reduce their dependency on pain medications in association with chiropractic care. Follow-up inquiry during the course of PRN care determined that both patients continued to reduce or abstain from pain medication usage and rather chose to self-manage their mild exacerbations with cryotherapy and exercises performed at home. This reduction of medication usage decreases/resolves the side effects associated with these drugs, improving the health of the patient while reducing health care costs. Further research of the effects of chiropractic care in reducing medication usage among pain sufferers is warranted.
The symptomatic and functional improvement observed in these cases is notable in view of the fact that neck and back pain seen in EDS patients have been reported to be resistive to most allopathic approaches.  Documentation of functional improvement was achieved via the use of the Oswestry Low Back Pain Disability Index and the Neck Pain Disability Index. Both of these instruments have been found to be reliable indicators in assessing a patient’s functional status. [15-17] Functional improvements of 40% and 37% were noted on Oswestry evaluation of the patients, and improvements of 52% and 29% were found in relation to the Neck Pain Disability Index instrument. Pain scores, using a visual analog scale, decreased by 40% and 57% in the respective cases.
These figures are significant because they represent discernible pain reduction in markedly chronic cases of physical disability. The limitations imposed on the activities of daily living of EDS sufferers have been shown to stem, in large part, from physical disability associated with the pain of EDS.  The functional improvement noted in these EDS patients following chiropractic intervention resulted primarily from a reduction in their levels of pain.
Of particular interest in these cases, however, was the observation of not only the aforementioned subjective, symptomatic improvement but also the objective structural changes documented by radiological mensuration of the pretreatment and posttreatment radiographs. Many practicing clinicians are all too familiar with those cases that show subjective improvement, only to exhibit virtually nil structural changes on posttreatment radiological examination. The 2 cases described herein, however, demonstrated measurable improvement in one or both of the following: angle of cervical lordosis, angle of lumbar lordosis, cervicothoracic Cobb angle, lumbar Cobb angle, and anterior head translation.
The mensurations used to document these biomechanical changes have been observed to be reliable indicators of static biomechanics. Intraexaminer and interexaminer reliability for the measurement of cervical lordosis were found to be in the good to excellent range. [18-20] In evaluating the reliability and reproducibility of measuring lumbar lordosis, Polly et al  found intraobserver reliability coefficients to be indicative of excellent reproducibility. Other studies by Harrison et al  and Troyanovich et al  have further supported the reliability of lateral lumbar radiographic analysis used in this study. The utility of the Cobb angle measurement has long been established in the bioscientific literature. 
EDS is a complex disorder, and its varied manifestations include spinal instability, ligamentous laxity, and vertebral deformity. [1, 25] In such cases, we would not usually expect to see any significant measurable changes take place in the structural alignment of the patient’s spine due to the inherent chronic weakness and instability associated with the supportive structures of the vertebral column. However, case number 1, in particular, demonstrated some rather notable structural changes to the spinal column on conclusion of the MFMA chiropractic treatment. Improvements of 20% in the cervicothoracic Cobb Angle and 33% in the anterior head translation were observed, along with an improvement in the cervical lordosis from -2° to +9°. The amounts of the observable changes are significant in as much as one would normally expect that the discoligamentous plastic deformity associated the chronicity of the condition would have prevented such a marked change in structural alignment.
Such improvements might lead one to suspect that the underlying neurological deficits seen in these cases may have been prime factors relating to the patient’s structural problems. Indeed, neurological involvement is not uncommon with EDS, and it has been proposed by Galan et al  that the increased ligamentous laxity seen in EDS may play an important role in the pathogenesis of the associated neuropathy. If so, conservative chiropractic care may be able to offer a benefit to sufferers of EDS who are expressing musculoskeletal related symptoms. Spinal manipulative therapy has been widely recognized in the medical field as a conservative treatment modality for spinal dysfunction and pain.  Stanitski et al  observed that back and/or neck pain was a common complaint among EDS sufferers, affecting over 67% of this patient population group. Interestingly, they found that the musculoskeletal symptoms of EDS patients were independent of the presence of any associated spinal deformity. It has been stated that a certain percentage of the everyday pain experienced by EDS patients is likely secondary to various articular subluxations.  Therefore, it may be possible that a certain percentage of EDS patients might be able to obtain symptomatic relief of their musculoskeletal symptoms if an effective chiropractic approach could be implemented. The challenge in this regard is to provide a chiropractic methodology gentle enough to be able to treat these patients without the risk of iatrogenic complication.
Manipulatory treatment of EDS patients
Inherently, patients with connective tissue disorders, such as EDS, present a challenge in treatment for clinicians, both due to the chronicity of the patient complaints and the risks associated with the disorders. From a musculoskeletal standpoint, patients with EDS have been found to have a greater tendency to suffer fracture,  which was observed on 2 occasions in case number 1. Another significant difference in EDS patients from the general population is that due to defective collagen synthesis, the affected connective tissue results in weaker than normal supportive structures of the joints. Because manipulative therapy generates considerable forces within the holding elements of the articular structures, the clinician contemplating manipulative therapy must be aware of the clinical factors and the associated physical implications involved when treating a patient with EDS. Osteopenia and osteoporosis, often associated with EDS, require a gentle adjusting methodology in any patient.  In addition, EDS patients have a higher prevalence of tissue fragility, which could result in bruising following chiropractic adjustment. The marked tissue fragility with tendency toward easy bruising makes the EDS patient a difficult subject for any forceful manipulation or deep trigger point work. However, the most serious problem for the clinician faced with treating the EDS patient is the issue of the low tensile strength affecting the vascular walls, which can often rupture spontaneously. [29, 30] The fragility of the vascular walls of EDS patients is often accompanied by numerous vascular defects, including multiple aneurysms, and the concomitant risk of arterial dissection.  Mattar et al  report that vascular fragility is marked enough to result in a pronounced tendency of the blood vessels to tear on even minimal manipulation during surgical procedures. Clearly, this presents a difficult situation in which to use forceful high-velocity, low-amplitude (HVLA) manipulation. However, in an interesting paper by Tuling et al,  the authors report on a patient suffering from undetected Marfan syndrome (clinically similar to EDS in terms of vascular fragility) who received HVLA treatment without any adverse effects to the vascular system. They caution, however, that it is inappropriate to knowingly implement HVLA manipulative therapy in patients who have pronounced vascular defects.
Thus, due to the inherent nature of their condition, EDS patients fall into that patient population group where HVLA manual chiropractic adjustments may be relatively contraindicated. [28, 33] Even certain physical medicine procedures, such as physical therapy and exercise, although warranted, can be quite traumatic and physically stressful for this group of patients.  While HVLA manipulation represents the most utilized  and most widely studied  of the various chiropractic methodologies, a gentler form of adjustive therapy is likely more appropriate for sufferers of EDS.
In the cases described above, MFMA chiropractic treatment was rendered via the use of an AAI, utilizing the adjustive protocols specified by AMCT. The AAI has been previously described in the indexed literature,  and AMCT has been reported to be one of the most studied methodologies in chiropractic.  In an informal survey, AMCT was found to be regarded by chiropractors as one of the safest modes of chiropractic treatment available.  Several studies have found the benefits of MFMA adjustments, with regard to pain relief, to be on par with those of HVLA manipulation. [39-41]
Gleberzon  has reported that MFMA adjustments may be more appropriate than HVLA manipulations for patients suffering from osteopenic or vascular disorders, in that the AAI delivers a lower mechanical force to the spine, and AMCT protocols, in particular, specify that cervical adjustments be performed in the neutral prone position, which involves less torque on vascular structures of the cervical spine than HVLA rotatory manipulation.  Cervical rotation has been identified as the position that places a patient at a relatively high risk of injury (notably vascular accident) as compared to nonrotatory positions. [44-46] MFMA manipulation, therefore, may represent a more applicable approach to handling the special needs of EDS patients than HVLA manipulation. In both cases, chiropractic adjustments were delivered in the neutral prone position by means of an AAI. The AAI used in these patients produces an approximate 150 N force, appreciably lower than other spinal manipulation techniques. [36, 47] Such methodology employed in the management of cases such as these may offer a viable alterative to more forceful or rotatory type spinal manipulation techniques.  Further study in this regard is needed.
Proposed mechanism of action
One of the most important components of the vertebral column is its supporting system of paraspinal soft tissues, particularly the ligamentous structures. It has long been known that disrupted joint mechanics can lead to connective tissue changes that can, in turn, predispose joints to accelerated degeneration.  The altered collagen formation seen in EDS results in a weakened ligamentous structure at the outset, which can only be further weakened upon exposure over time to the same biomechanical stresses that affect the population in general. It may be that the etiology of the neck and back pain that EDS patients experience is essentially the same as the general population, except that its intensity is worsened as a result of the inherited structural weakness of the supportive soft tissue structures of the spine. Indeed, the back and neck pain of EDS patients is independent of any EDS associated spinal deformity. 
Therefore, the primary etiology of spinal pain reported by EDS patients may be biomechanical in nature, with a tendency toward increased occurrence resulting from heritable weakness of the supportive ligaments of the spine, including the facet capsular ligaments. These ligaments are some of the most important structures of the spine because of their role in maintaining proper spinal alignment and kinematics. Wyke [49-52] has reported the presence of mechanoreceptors within these ligaments, whose function it is to monitor both static position and motion from the joint through afferent nerve fibers and back to the central nervous system. Disrupted afferent articular input from damaged/dysfunctional mechanoreceptors can occur as a result of altered articular mechanics, resulting in aberrant neurological afferentation. The resulting deafferentation can result in a state of segmental facilitation with associated myospasm and pain. [53, 54]
Chiropractic adjustments, including MFMA thrusts rendered via an AAI, have been proposed to provide benefit in several ways. Those include the stimulation of mechanoreceptors, with resulting depolarization of the affected neuronal pools, to reestablish proper function in the central nervous system. [55, 56] Mechanoreceptor stimulation has an inhibitory effect on afferent pain pathways and efferent motoneuron activity. Pain alleviation following spinal manipulation may be caused by coactivation of mechanically sensitive somatic afferents. Reduction of muscle spasm may result from concomitant inhibition of efferent motoneurons. [57, 58] Indeed, Keller and Colloca  reported observing favorable electromyographic changes in paravertebral musculature following MFMA adjustments of the spine with an AAI. The improvement of muscle tone following spinal manipulation may help to improve segmental articular kinematics and restore normal afferent input to the spinal cord from the involved articular components. This may result in an improvement in the neurological operation of the articular mechanism. The precise nature of the mechanisms involved regarding the neurophysiology of spinal manipulation requires further elucidation  and study in that area is ongoing. [11, 61]
Both patients actively engaged in the performance of CBP mirror-image spinal stabilization exercises both in the office and at home. According to CBP technique protocols, [12, 13] such exercises are specifically designed to reverse patients’ posture, acting to unload, stretch, and strengthen the patient’s spinal joints simultaneously. It is our opinion that the positive outcomes in both cases presented are likely due to a combination of the chiropractic adjustments and exercise regimens adhered to by the patients.
Chiropractic care may be of benefit to some patients with connective tissue disorders, including Ehlers-Danlos syndrome. Low-force chiropractic adjusting techniques may be a preferred technique of choice in patients with tissue fragility, offering clinicians a viable alternative to traditional chiropractic care in attempting to minimize risks and/or side effects associated with spinal manipulation. Psychosocial issues, including patient desire to return to work, were important factors in work disability status and perceived outcome.
While no definitive conclusions can be drawn from a case report, the results observed in these cases are encouraging, given that close to 70% of EDS patients complain of neck and back pain that is usually resistive to pharmacological intervention and is independent of any EDS-associated spinal deformity. Any relief that chiropractic care could offer this group of patients would be significant and make a favorable impact on improving their quality of life. Further study, organized in an academic research venue, will help to clarify the role that chiropractic treatment may be able to provide for sufferers of EDS.