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Research & Science Research & Science Chiropractic Management of Ehlers-Danlos Syndrome: A Case Report By Joseph J. Morley, DC, PhD;* and Terence Parrault, DC, DACBR* Address all correspondence to: Terence Perrault, DC, DACBR Associate Professor of Clinical Sciences University of Bridgeport College of Chiropractic 75 Linden Avenue Bridgeport, CT 06604 Tel: (203)-576-4281 E-mail: [email protected] * Associate Professor of Clinical Sciences, University of Bridgeport College of Chiropractic ABSTRACT Objective: To structure chiropractic care for the symptomatic relief of musculoskeletal symptoms in a patient with Ehlers-Danlos Syndrome (EDS). Clinical Features: A patient with EDS presented with chronic dorsal, neck, and low-back pain. She also had joint hypermobility, skin hyperelasticity and spontaneous glenohumeral dislocations. Intervention: Treatment included soft-tissue work and chiropractic manipulation. Treatment lasted for 7 months. Outcome: The patient reported a noticeable reduction in neck, dorsal, and low-back pain during treatment. Conclusion: Chiropractic care helped relieve some of the more common musculoskeletal symp- toms in this patient and may prove of value in other EDS patients. Doctors of chiropractic who are aware of EDS and its associated symptoms may be able to offer significant relief in selected EDS cases. A large-scale trial of chiropractic management of EDS needs to be done to demonstrate effi- cacy or lack thereof. Key Indexing Terms: Ehlers-Danlos Syndrome; musculoskeletal pain; chiropractic INTRODUCTION • deficiencies of enzymes that produce Ehlers-Danlos Syndrome (EDS) is an inherited collagen disorder of connective tissue characterized by • mutation of collagen alpha chains heterogenicity and variance in clinical symp- toms.1 Mao2 identifies 3 mechanisms by which Enzyme deficiencies or mutations affecting dif- the disease can occur: ferent collagens involved in EDS will become 6 MARCH 2010 Research and Science manifest in tissues in which that particular type logical problems,10 epilepsy,11 cerebral cortical of collagen predominates functionally, i.e., lesions,12 cephalalgia,13 and musculoskeletal skin, ligament, vascular tissue, bowel tissue, pain.14-16 and oral structures. Previously, Ehlers-Danlos was divided into 10 • Haplo-insufficiency types. This was based on single-gene disorders. More recently, the Villefranche classification This results from a lower level of a gene prod- (Table 1) was developed.17 The Villefranche uct and affects the cell’s ability to function nor- classification categorizes Ehlers-Danlos into 6 mally. groups based on the severity of the clinical fea- tures, biochemical defects, genetic defects, and Research has also concentrated on tenascin-X inheritance pattern.1,18 deficiency in connective tissues.3,4 This is an extracellular-matrix protein. However, tenas- cin-X deficiency is not found in a large per- centage of EDS patients but seems to be asso- ciated more with the hypermobility type of EDS.3,5 Malfait suggests that other genetic fac- tors and gender both contribute to EDS. Most of those suffering from the joint hypermobility 1 type of EDS and associated pain were women. Fig. 2. Joint hyperflexibility7 Although joint hypermobility may be the most noticeable symptom, skin hyperelasticity, ex- The most common form of EDS is autosomal tensive bruising and scarring, and connective dominant in the older classification, hyper- tissue fragility can be common features. Typi- mobility type in the newer Villefranche classi- cal examples of skin hyperelasticity and joint fication. The major diagnostic criteria are joint hypermobility associated with EDS are seen in hypermobility and skin described as soft, Figs. 1 and 2, respectively. smooth, and velvety. Joint hypermobility can lead to dislocation, which Beighton17 lists as one of the minor diagnostic criteria. Other mi- nor criteria include chronic joint or limb pain and a positive family history. Beighton17 stated that one or more of the major criteria must be present for a definitive clinical diagnosis. Mi- nor criteria aid in the diagnosis but are not suf- ficient in themselves. Fig. 1. Skin hyperelasticity6 A major symptom, joint hypermobility, is also shared with Marfan’s Syndrome, Benign Joint Familial Hypermobility Syndrome, and Osteo- More serious symptoms associated with EDS 19 can include joint dislocations, rupture of blood genesis Imperfecta. The heterogenicity of vessels and hollow viscera, and mitral valve EDS and the fact that symptoms and severity prolapse. Other reported manifestations of EDS can overlap with other conditions can lead to can include periodontal problems,8,9 psycho- difficulty in diagnosis. JOURNAL OF THE AMERICAN CHIROPRACTIC ASSOCIATION 7 Research and Science CASE REPORT • previous episodes of epileptic seizures, This case study dealt with the hypermobility the last one occurring 2 years ago type of EDS, which may be the type that is • blood loss anemia in the past – heavy characterized by the most functional muscu- menstrual periods and nosebleeds 14 loskeletal problems. • easy bruising • asthma A 5’ 4” 120 lb., 20 year old female college stu- dent suffering from chronic spinal pain sought Previous treatments included: evaluation of her condition at a chiropractic • 2 months of physiotherapy for thoracic clinic. and cervical pain immediately following her car accident This patient had been diagnosed with EDS • massage therapy for thoracic and cervi- prior to seeking chiropractic care. Her family cal pain history included a brother also with EDS. She • spontaneous reductions of dislocations reported that he had a more severe form than or self-reductions of shoulder disloca- she did, but did not elaborate any further. Past tions medical history included pyloric stenosis as an • 2 hours of cardiovascular and weight infant and an appendectomy 2 years previously. exercises regularly • dihydrocodeine for spinal pain Her lifestyle and dietary history revealed that she was a non-smoker, consumed very little • carbamazepine for epilepsy alcohol, had a regular intake of fruits and vege- • sodium valproate for epilepsy tables, and ate chicken in preference to red • phenytoin for epilepsy meats. She did not drink coffee and drank at • beclometasone for asthma least a quart of water per day. She did not re- • salbutamol for asthma port taking any supplements. • iron tablets for blood loss anemia Her major complaints were thoracic and cervi- Informed consent was obtained from the pa- cal pain that worsened after a car accident 3 tient and a physical examination was per- years ago. Prior to this accident, she had had formed. periodic discomfort in these areas, but not to the extent now reported. She reported the pain General Findings as ranging from 3 to 8 on a 10-point scale. The Vital signs were within the normal range for a spinal pain had become worse recently, tending female of her age. The skin was elastic and toward 8. smooth. A scar was noted at the mid-sternal area and the patient reported that a keloid had Her secondary complaints included repeated been removed years ago. There was hyperex- bilateral shoulder dislocations and hip and fin- tension of both knees, both elbows, over- ger dislocations. Other secondary complaints pronation of the feet, and loss of the normal included: medial longitudinal arches. • difficulty sleeping due to the thoracic and cervical pain Cervical Exam • occasional low-back pain that seemed to Hypolordosis worsen with menstruation, but did not Head anterior to gravity line worsen after the accident Cervical distraction produced some pain radiat- ing toward her right shoulder but not in her neck. Passive bilateral rotation, lateral flexion, 8 MARCH 2010 Research and Science flexion, and extension were negative. An active the legs and arms (biceps and triceps curls, trigger point was found in the right upper tra- knee extensions, squats) and walking and jog- pezius. ging. These exercises are beneficial for main- taining muscle mass, minimizing osteoporosis, Thoracic Exam and maintaining the cardiovascular system in Rotation and lateral flexion were restricted bi- good health. Advice regarding the importance laterally from T5-T10. Active trigger points of Vitamin C for connective tissue was given. were found in the rhomboid majors, thoracic paravertebral musculature bilaterally, and mid- Over a 10-month period, she was seen a total of dle trapezii. 20 times. Within a week, after 3 visits, she re- ported an improvement of overall pain, down Lumbo-sacral Exam from an 8 to a 4. By the end of the second week Hypolordosis of treatment (visit 5), she reported that her Right SI motion was restricted. Palpation of menstrual period was lighter and shorter than right SI joint elicited tenderness. normal. At visit 6, she reported an exacerbation of thoracic pain, a 7 on the pain scale. At visit Radiological Exam 7, she reported that she had responded to the A radiological report for lumbar, thoracic and last treatment but the thoracic pain had returned cervical x-rays taken elsewhere stated that after a few days. There had been a spontaneous there were no fractures or dislocations. A loss shoulder dislocation and subsequent neck pain of normal cervical lordosis was also reported. on her right side. By visit 9, she had been under care for almost 2 months. She reported a sig- Diagnoses, Rationale, and Treatment nificant reduction in her neck and back pain Exam and history findings supported a diagno- from the last visit until the current visit. Her sis of myofascial pain related to specific trigger pain was at a 2. She also reported that her men- points in the mid and upper trapezii, rhomboid strual periods were shorter and less painful than majors, and thoracic paravertebral muscles. In normal. addition, there was pain associated with fixa- tion of the right SI joint and vertebrogenic pain She was a student and did not return for 2 associated with restriction of movement of T5- months during the summer break. Her symp- T10. toms were at about a 2 or 3 during this period. At the 12th visit, she reported that her cervical Given the above findings, the connective tissue area had been quite sore after the last treatment.
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