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Physiotherapy Theory and PracticeAn International Journal of Physical Therapy

ISSN: 0959-3985 (Print) 1532-5040 (Online) Journal homepage: http://www.tandfonline.com/loi/iptp20

A multimodal physical therapy approach utilizingthe Maitland concept in the management of apatient with cervical and lumbar radiculitis andEhlers–Danlos syndrome-hypermobility type: Acase report

Adelina Pennetti

To cite this article: Adelina Pennetti (2018): A multimodal physical therapy approach utilizingthe Maitland concept in the management of a patient with cervical and lumbar radiculitis andEhlers–Danlos syndrome-hypermobility type: A case report, Physiotherapy Theory and Practice,DOI: 10.1080/09593985.2017.1422207

To link to this article: https://doi.org/10.1080/09593985.2017.1422207

Published online: 08 Jan 2018.

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RESEARCH REPORT

A multimodal physical therapy approach utilizing the Maitland concept in themanagement of a patient with cervical and lumbar radiculitis and Ehlers–Danlossyndrome-hypermobility type: A case reportAdelina Pennetti, PT, DPT, cert MDT, OCS, COMT

Summit Medical Group, Florham Park, NJ, USA

ABSTRACTThe purpose of this case report is to present a multimodal approach for patient management usingthe Maitland concept framework for cervical and lumbar radiculitis with an underlying diagnosis ofEhlers–Danlos Syndrome-Hypermobility Type (EDS-HT). This case presents care guided by evidence,patient values, and rationale for the selected course of physical therapy treatment provided bytherapist experience. A 35-year-old female with a 2-year history of worsening lumbar and cervicalpain was referred to physical therapy to address these musculoskeletal issues concurrent withdiagnostic testing for EDS. A multimodal approach including manual therapy, therapeutic exercise,postural and bodymechanics education, and a home exercise programwas used. The patient specificfunctional scale (PSFS) was used to gauge patient’s perceived improvements which were demon-strated by increased scores at reevaluation and at discharge. Following the Maitland concept frame-work, the physical therapist was able to make sound clinical decisions by tracking the logical flow ofconstant patient assessment. A 10-month course of treatment designed to maximize recovery offunction was successful with a chronic history of pain and the EDS-HT diagnosis. The role of educationand empowering the patient is shown to be of utmost importance. Optimizing therapeutic outcomeslong-term for this patient population requires maintaining a home exercise program, adaptation andmodifications of work and lifestyle activities.

ARTICLE HISTORYReceived 11 September 2016Revised 5 Februray 2017Accepted 21 March 2017

KEYWORDSEhlers-Danlos syndrome-hypermobility type; Maitlandconcept; physical therapy

Introduction

Ehlers–Danlos Syndrome (EDS) is a group of heritableconnective tissue disorders with many different subtypes.Physical therapy is essential for treatment of this popula-tion who present with chronic musculoskeletal pain andfunctional impairment. Individuals with EDS present withclassic joint hypermobility (JHM) due to increased laxity inconnective tissues and are subject to chronic pain that canlead to marked functional limitations. Musculoskeletalpain is one of the most common reasons for seekingprimary health care (Palmer et al., 2016). JHM is a com-mon cause of musculoskeletal pain and is treated by var-ious physical therapy interventions to address pain,proprioception deficits, poor motor control, joint instabil-ity, and soft tissue injury (Palmer et al., 2016).

A manual therapy approach is not usually chosen as amode of treatment for the hypermobile patient; however,it can be effective with proper clinical decision-making(Simmonds and Keer, 2007). The Maitland concept ispatient-centered using clinical reasoning with specifictechniques of assessment and treatment. An organizedassessment format is followed allowing the therapist to

assess the patient’s signs and symptoms as well as con-sider input from a variety of clinical data to supportsound decision-making (Hengeveld and Banks, 2014a,2014b). The purpose of this case study is to outline amultimodal approach including the use of the Maitlandconcept framework in the management of a patient withEDS who presented with cervical and lumbar radiculitis.This case presents care guided by evidence, patientvalues, and rationale for the selected course of physicaltherapy treatment provided by therapist experience.

Background

EDS is a group of heritable connective tissue disorderscharacterized by abnormal collagen synthesis (DePaepeand Malfait, 2012; Rombaut et al., 2010a; Shirley,DeMaio, and Bodurtha, 2012; Sobey, 2015). It is themost prevalent (1:5,000–1:10,000) among soft tissue dis-orders (Rombaut et al., 2010a). EDS is characterized byvarying degrees of skin hyperextensibility, JHM, andvascular frailty (DePaepe and Malfait, 2012; Rombautet al., 2010a; Shirley, DeMaio, and Bodurtha, 2012;

CONTACT Adelina Pennetti adelina73@msn.com Summit Medical Group, Florham Park, NJ, USA.

PHYSIOTHERAPY THEORY AND PRACTICEhttps://doi.org/10.1080/09593985.2017.1422207

© 2018 Taylor & Francis

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Sobey, 2015). Genetic mutations are used to identifyEDS which affects the synthesis and structure of type I,II, III, and V collagen (DePaepe and Malfait, 2012;Shirley, DeMaio, and Bodurtha, 2012; Sobey, 2015).Categories of EDS include classical, hypermobile, vascu-lar, kyphoscoliotic, arthrochalasic, dematosparactic,Tenascin-X deficient, EDS with scoliosis, myopathy,hearing impairment, and musculocontractural EDS(DePaepe and Malfait, 2012; Shirley, DeMaio, andBodurtha, 2012; Sobey, 2015). Classical and hypermobi-lity types account for more than 90% of cases. The thirdmost common type is vascular EDS and may affect 1 in250,000 people. The other categories are extremely rare(Shirley, DeMaio, and Bodurtha, 2012).

Hypermobility in the joints is commonly seen infemales and children (Castori, 2012; Shirley, DeMaio,and Bodurtha, 2012). Differentiation between benignJHM and a more serious hereditary connective tissuedisorder such as one of the several EDS subtypes is neces-sary to prevent potential systemic complications anddevelop appropriate treatment strategies. JHM isdescribed as the ability of one or more joints to activelyand/or passively move beyond normal limits (Castori,2012). EDS-hypermobility type (EDS-HT) is consideredto be synonymous with joint hypermobility syndrome(JHS) which is also classified as a hereditary connectivetissue disorder (Castori, 2012; Shirley, DeMaio, andBodurtha, 2012). A small percentage of individuals withEDS-HT have mutations in the TNXB gene and patientswith JHS also make a reduced amount of tenascin-Xprotein, making the two conditions one and the same.The typical skin features of EDS are not present in EDS-HT or JHS. Clinical diagnosis is based on generalizedJHM. The Brighton criteria which includes the Beightonnine point score for JHM with clinical symptoms is usedto confirm the diagnosis of benign JHS (BJHS) along withgenetic testing. BJHS is excluded by the presence ofMarfan syndrome or EDS, with the exception of EDS-HT (Clark, 2010; Grahame, 2009; Grahame, Bird, andChild, 2000; Shirley, DeMaio, and Bodurtha, 2012).

Physical therapy plays an important role in the assess-ment and management of hypermobility syndrome. Aftera thorough assessment for individual JHM, issues of jointstability are addressed through motor control and gradedstrengthening exercises. Posture and faulty movementpatterns which develop as a result of hypermobility,pain, fatigue, joint instability, and muscle weakness needto be addressed in a physical therapy program(McKeown, 2015). Patient education and empowermenton the condition are also key components in producing asuccessful outcome.

The Maitland concept for manual therapy requiresskilled clinical reasoning linked with a logical and

methodical process of evaluating cause and effect.Based on the bio-psychosocial model set forth by theWorld Health Organization International Classificationof Functioning Disability and Health, Maitland’s centraltheme necessitates a positive personal commitment tounderstand the patient’s perspective and values onsymptoms such as pain and movement restrictions inactivities (Hengeveld and Banks, 2014a, 2014b).Appropriate problem solving logic, or assessment, willrelate clinical findings to pathology and mechanical dis-order. This process of assessment is the key to successfulmanipulative treatment which should result in reducedpain and increased movement. It has been documentedthat in addition to the physical complaints, the therapistmust be aware of psychological effects of depression andanxiety as a result of the condition, as well as the socialrestrictions in the patient’s life (Baeza-Velasco, Gely-Nargeot, Bulbena-Vilarrasa, and Bravo, 2011). Studiesconfirm that anxiety driven fear of pain results in avoid-ance behavior that will affect a patient’s ability to per-form physical activities which can directly place socialrestrictions in the patient’s life (Scheper, deVries,Verbunt, and Engelbert, 2015). A supportive therapistshould provide affirmation of these psychosocial issuesand, if impacting treatment should refer to appropriatehealthcare practitioners. All of these components, alongwith utilizing the best available research and experiencebased evidence into treatment represent all aspects ofevidence based practice.

No studies presenting the management of any sub-types of EDS using the Maitland concept of manualtherapy were found in a literature search of commonmedical databases. The purpose of this case report is topresent the patient-centered management and outcomesof a multimodal physical therapy approach utilized for apatient with a complex cervical and lumbar radiculitispresentation and a diagnosis of EDS-HT. The clinicaldecision-making framework followed when possible, animpairment based treatment approach utilizing the bestavailable evidence, patient values and therapist expertise.

Case description

The patient is a 35-year-old female referred to outpatientphysical therapy for evaluation of worsening lumbar andcervical radiculitis. The cervical symptoms had beenpresent for 5 months and the lumbar, 7 months. At thetime of evaluation, genetic testing to rule in an EDSdiagnosis had been initiated. She is married, a motherof two children under the age of 12 and is employed in ahome-based profession with high computer use.

Past medical history was not significant for co-morbid-ities. She adopted a physically active lifestyle 8 years prior to

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the episode of care. Over the prior 6 years, the patient hadtrained for and completed six triathlons. Four years beforereferral she developed right lateral lower leg pain andtingling into the lateral border of the foot. This intensifiedto the point that she was forced to stop running because ofpain. An MRI showed right tibia stress fracture and pero-neal tendonitis. Eight months later, a repeat MRI showededema in the peroneal muscle group with no bony involve-ment.One year later, she developed intense pain in the rightbuttock, lumbar spine and groin in addition to the pain andparesthesia from the previous year. AnMRI of the right hiprevealed mild hip dysplasia with a shallow acetabulum andfull thickness tear of the base of the anterior labrum,extending towards the anterior superior labrum. Onemonth later, the patient developed left-sided neck painwith radiating pain to the L medial scapula and paresthesiainto the LUE as well as stabbing pain left-side mid-thoracicregion radiating around fromher leftmedial scapula borderto under her left breast. Along with pain in her cervicalspine, the patient described associated dizziness or light-headedness that affected her daily activities. She was notable to correlate these symptoms with any specific activityor trauma. Five months after the onset of cervical symp-toms she was referred to physical therapy for conservativemanagement.

At the time of evaluation, MRIs of the cervical, thor-acic, and lumbar areas of the spine revealed disc bulging atC5-6, mild disc bulging C6-7 with neural foraminanarrowing, bilateral small cervical ribs at C7, minormid-thoracic bulge with no evidence of cord compres-sion, and L5–S1 degenerative disc with bilateral neuralforamina narrowing.

Prior to the start of physical therapy, the patient wasproactive in educating herself through the literatureregarding her potential diagnosis. One month after thestart of physical therapy, the patient received the genetictesting results, confirming an EDS diagnosis. Thisprompted her to join a support group for EDS on socialmedia which allowed contact with others dealing with thesame diagnosis. According to the genetics report, there isa molecular change in the tenascin XB gene (TNXB)which makes tenascin-X. The molecular change is a keyphysiological finding to confirm the EDS diagnosis.

Examination

Subjective examinationThe patient initially presented with complaints in theleft-side neck and “knife-like” pain in the medial peri-scapular region. There was intermittent tingling inbilateral arms and radiating pain from the left medialscapula region to under the left breast. Patient-reportedpain levels are shown in Table 4. She complained of

intermittent weakness in both arms, right greater thanleft. She reported constant “aching and tightness” painin the lower right lumbar spine and right posterior hipwith intermittent “sharp pain” in the groin and “tight-ness with tingling” into the lower lateral leg. There wereno complaints of numbness in any extremity. Thepatient described her perception of her body balanceas, “I do not feel grounded through my feet when I amstanding. When I am sitting, I feel the weight mostlythrough my left buttock, my body is torqued to the left,and I feel jammed up on the right side of my trunk.”

Systems review and screeningsA review of the patient’s past medical history via theelectronic medical record was not significant for chronicmedical conditions. Her cardiopulmonary and integumen-tary systemswere normal. According to the initial intake bythe geneticist, it was documented under psychiatric that thepatient had normal mood, affect, and behavior. Judgmentand thought content was also considered normal.Neurological screen for deep tendon reflexes for C5, C6,and C7 bilaterally were normal. The patient complained ofdizziness or lightheadedness which required vertebrobasi-lar insufficiency (VBI) screening to rule out vascular com-promise. Associated signs and symptoms of VBI includedizziness, diplopia, dysphagia, dysarthria, drop attacks,ataxia of gait, nausea, numbness, or nystagmus(Huijbregts and Vidal, 2004). The VBI screening was per-formed in sitting by sustained cervical rotation to bothsides for 10 s while monitoring for signs and symptoms(Grant, 1996). The VBI screening was negative.

With VBI ruled out and the history of neck pain with-out trauma, cervicogenic dizziness was considered as thesource of the dizziness and was the working diagnosis.The patient’s cervicogenic dizziness was hypothesized asmost likely being caused by the increased forward headpositioning, which disrupted the upper cervical posturalmechanoreceptors, creating upper cervical hypomobilitywith decreased stimulation of postural mechanoreceptors(Huijbregts and Vidal, 2004). The patient also presentedwith spasms of the upper trapezius and sternocleidomas-toid, another contributing factor of cervicogenic dizziness(Huijbregts and Vidal, 2004; Wrisley, Sparto, Whitney,and Furman, 2000).

Physical examinationPosture assessment in standing revealed moderate for-ward head, bilateral protracted shoulders, elevated leftshoulder girdle and left trunk rotation. An increasedvalgus at the right knee with excessive pronation of theright foot was noted.

Spinal active physiological range of motion (ROM)was measured based on clinical observations from the

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experienced therapist to estimate percentages of avail-able motion in each plane. Studies show the trained eyeis capable of accurately measuring extremity active rangeof motion (AROM) based on the experience level of theobserver. Intraclass correlation coefficients rated fromfair to high (0.73–0.96) between experienced observers(Blonna, Zarkadas, Fitzsimmons, and O’Driscoll, 2012;Holm et al., 2000). A study in observation of cervicalROM by orthopedic surgeons demonstrated good valid-ity compared to using measurement tools and lead to theconclusion that estimated values were appropriate inassessing impairments for disability (Hirsch et al.,2014). Lumbar spinal motions have been reportedlymeasured by a variety of methods including tape mea-sures, inclinometers, protractors, and electronic goni-ometers. No studies of comparative validity orreliability of these tools or comparison with visual esti-mates were found. While ideally measurement of jointmovements is completed using validated goniometrictools, the therapist in this case made the decision to usevisual observation of spinal movements due to clinicaltime constraints and the purpose of the measurementswas to be in assessing spinal impairments as contribut-ing factors in functional disability and pain.

Strength outcomes were measured by manual muscletesting (Kendall and McCreary, 1993) (Tables 2 and 3).The craniocervical flexion test was performed to assessdeep cervical muscle endurance (Painkra et al., 2014).The patient was unable to achieve craniocervical flexionwithout assist. Transverse abdominis (TrA) demon-strated poor recruitment and multifidus recruitmentwas trace via palpation using the abdominal drawing-inexercise (Teyhen et al., 2005).

Passive physiological ROM reproduced the patient’spain complaints in the same motions as active physiolo-gical ROM. Results were consistent with active ROMfindings. Passive accessory intervertebral movementassessment localized hypomobility and comparable signsin the following segments: Unilateral anterior to posterior(UAP) force on the articular pillar of C4, C5, and C6 onthe left; unilateral posterior to anterior (UPA) force on thecosto-transverse joints of T5, T6, T7, and T8 on the right;and UPA to the transverse process of L5 and L4 on theright. Due to paracervical muscular tightness, it was diffi-cult to assess hypermobility in the cervical segments. Thepatient demonstrated normal shoulder AROMbilaterally.Right hip A/PROM was painful and limited into flexionand internal rotation. PROM hip extension was severelylimited, unable to achieve neutral, secondary to tightnessin hip flexor and rectus femoris muscles.

Hypermobility was assessed using the 9-point Beightonscoring system (Table 1) (Beighton, Solomon, and

Soskolne, 1973). A score greater than or equal to 4 of 9points is indicative of general hypermobility. The patientscored 8/9 points, had normal skin extensibility, no abnor-mal scarring, and confirmed TNXB gene mutation; thusencompassing all of the characteristics of EDS-HT.

Palpable myofascial trigger points as defined bySimons, Travell and Simons (1999) were located inthe upper tapezius, levator scapulae, and rhomboidson the left and in the quadratus lumborum, piriformis,gluteus medius, iliopsoas, tensor fascia lata (TFL), andproximal rectus femoris on the right.

Special tests were selected based on the hypothesesdeveloped during the subjective examination and on thediagnostic properties of the individual tests. Positive spe-cial tests for the cervical spine included Spurling maneu-ver and lower cervical extension quadrant. Spurlingmaneuver to the left side causing severe left periscapulardiscomfort and pain into the left arm consistent with theC6 dermatome. Sensitivity of the Spurling test for nerveroot pathology is 95% and specificity is 94% when com-pared to imaging studies (Shabat, Leitner, David, andFolman, 2012). Left lower cervical extension quadrant asdescribed by Maitland was also positive, reproducing thepatient’s pain complaint (Maitland, 1979).

Table 1. The Beighton score for joint hypermobility.Joint mobility Right Left

1.Passive dorsiflexion of the fifth digit metacarpo-phalangeal joint > 90°

1 1

2.Opposition of the thumb to the volar aspect of theipsilateral forearm

1 1

3. Hyperextension of the elbow >10° 1 14. Hyperextension of the knee > 10° 1 15. Forward trunk flexion with knees extended andplace hands flat on the floor

Present = 1

Maximum total 9

Source: Beighton, Solomon L, and Soskolne (1973).

Table 2. Spinal active range of motion.Initial

evaluation Reevaluation Discharge

CervicalFlexion 75% 100% 100%Retraction 50% ERP L 75% 100%Extension 75% ERP L 90% ERP L 100%Rotation R 75% 90% 90%Rotation L 50% ERP L 90% 90%Lateral Flexion R 75% 75% 100%Lateral Flexion L 50% ERP L 75% ERP L 100%

ThoracicRotation R 25% ERP mid

T/S75% 75%

Rotation L 75% 75% 75%LumbarFlexion 75% 100% 100%Extension 25% ERP R 50% ERP R 100%Lateral Flexion R 50% ERP R 50% ERP R 100%Lateral Flexion L 50% ERP R 75% end range

stretch R100%

ERP, end range pain; R, right; L, left.

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Analysis of signs and symptoms suggested an elevatedfirst rib was contributing to her pain. The cervical rota-tion-lateral flexion test was performed as described byLindgren, Leino, and Manninen (1992) and the patientdemonstrated a significant loss of right lateral flexion inleft rotation, indicating an elevated hypomobile rib on theleft. Testing was mildly positive on the right side. Toconfirm rib position, a spring test for the first rib end-feel was positive for loss of caudal mobility in supinebilaterally, left greater than right. Upper limb tensiontests performed as described by Butler for median,ulnar, and radial nerves bilaterally did not reproduce thepatient’s pain complaint and thus were not consideredcomparable (Butler, 1991).

In the lumbar spine, right lumbar extension quadrant asdescribed by Maitland was positive (Hengeveld and Banks,2014b). Lower limb tension test was positive on the rightfor sciatic nerve with common peroneal bias (Butler, 1991).The right hip, being symptomatic, demonstrated positiveflexion/adduction quadrant testing and FABER implicatingintra-articular pathology as her CT-scan confirmed(Hengeveld and Banks, 2014a; Troelsen et al., 2009).

Patient-reported outcome measuresThe patient specific functional scale (PSFS) was chosen asthe self-reported outcome measure. The PSFS is a

validated, patient specific measure, designed to assessfunctional change, primarily in patients presenting withmusculoskeletal disorders (Horn et al., 2012). It is admi-nistered at the start of interventions to establish a baselinemeasure and then re-administered for reevaluation anddischarge measures. The PSFS allows patients to chooseup to five functional activities that are difficult to performdue to their current condition and rate his or her abilityon an 11-point scale with zero representing the inabilityto perform a task and 10 representing the ability to per-form a task without difficulty at prior levels (Horn et al.,2012). The selected items for this patient included sittingat a desk, lifting objects, disrupted sleep, dizziness withactivity, and walking. The patient scored 2 (10/50) pointsat initial evaluation.

Assessment

Analysis of the tests and measures indicated the nature ofthe patient’s pain was mechanical primarily due to pos-tural dysfunction. Contributing results included elevatedpain levels, impaired spinal mobility in the cervical, thor-acic, and lumbar spines, poor muscle performance in thelocal spinal stabilizers, hips, and periscapular region, andsoft tissue tightness with multiple myofascial triggerpoints. Even though imaging showed mild disc bulgingand degenerative disc disease, the primary diagnoses werecervical and lumbar radiculitis based on positive exten-sion quadrants (Hengeveld and Banks, 2014b; Smith et al.,2014) resulting from facet dysfunction. A second diagno-sis was right hip pain and limited movement associatedwith intra-articular pathology and femoral acetabularhypomobility (Troelsen et al., 2009). Cervical and lumbarradiculopathy were ruled out as possible differential diag-noses since signs and symptoms did not satisfy the criteriarequired (Iversen et al., 2013; Wainner et al., 2003).

Based on the patient’s prior history of recovery frommusculoskeletal injury, spinal mobilization to the hypo-mobile segments was included in the treatment plan

Table 3. Manual muscle testing measurements.Initial MMT R Initial MMT L Reeval MMT R Reeval MMT L Discharge MMT R Discharge MMT L

ShoulderFlexion 4-/5 4-/5 4-/5 4-/5 4/5 4/5Abduction 4-/5 4-/5 4-/5 4-/5 4/5 4/5Extension 3+/5 3+/5 4-/5 4-/5 4/5 4/5External rotation 3+/5 3+/5 4-/5 4-/5 4/5 4/5Rhomboids 3+/5 3+/5 3+/5 3+/5 4-/5 4-/5Middle trapezius 3+/5 3+/5 3+/5 3+/5 4-/5 4-/5Lower trapezius 3/5 3-/5 3/5 3/5 4-/5 4-/5

HipFlexion 3+/5 4-/5 3+/5 4-/5 4/5 4/5Abduction 3+/5 3+/5 3+/5 4-/5 4/5 4/5Extension 3+/5 3+/5 3+/5 3+/5 4/5 4/5External rotation 3+/5 4-/5 4-/5 4-/5 4/5 4/5

R, right; L, left; MMT, manual muscle testing.

Table 4. Outcome measure scores.Initial

evaluation Reevaluation Discharge8-monthfollow-up

NPRS (0–10) C/S andT/S at best

5/10 1/10 0/10 1–2/10withoutexertion

C/S and T/S at worst 8–9/10 3–4/10 1–2/10 4/10L/S and hip at best 4/10 2/10 0/10 1–2/10

withoutexertion

L/S and hip at worst 8/10 5/10 1–2/10 4/10PSFS 2 (10/50) 5.6 (28/50) 8.4 (42/50) 7.4 (37/

50)

C/S, cervical spine; T/S, thoracic spine; L/S, lumbar spine; NPRS, numeric painrating scale; PSFS, patient specific functional scale.

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despite the EDS-HT diagnosis. Given the complexity ofthis orthopedic presentation, mobilization would beadministered by a therapist (AP) with advanced practicetraining in the techniques using continuous assessment ofthe patient’s response according to the Maitland concept.Selection of the correct segments to restore spinal arthro-kinematics would be essential due to the radiating pain inorder to achieve successful treatment outcomes. Manualtechniques would be selected according to the patient’spresentation at each visit.

Goals for physical therapy included complete resolu-tion of pain, the ability to return to normal dailyactivities and be able to exercise again. Based on thesubjective and physical examinations, it was reasonableto hypothesize a good prognosis and outcome withskilled physical therapy intervention.

Interventions

The patient was initially seen two times per week for16 weeks. Treatment consisted of postural reeducationexercises, myofascial trigger point release (TPR), spinalmobilization, core stabilization exercises, cervical andscapular stabilization exercises, self- treatment strate-gies and home exercise instruction.

The painful mid-cervical spine and first rib hypo-mobility was addressed with a combination of manualtherapy techniques to restore cervical arthrokinematicsand postural retraining to correct position of theshoulder girdle. Techniques included soft tissue mobi-lization to the sternocleidomastoid, anterior, middle,and posterior scalenes as well myofascial TPR toupper trapezius and levator scapulae. A caudal glideof the first rib was performed to assist in lowering theshoulder girdle. Myofascial TPR technique was utilizedto restore abnormally contracted sarcomeres to theirnormal resting length (McPartland and Dg, 2006).

Evidence supports the use of spinal mobilization toimprove joint mobility and ROM; however in patientswith JHS, pain is usually latent and easily aggravated.High velocity thrust techniques (HVT) or Grade V’sare generally contraindicated in the hypermobilepatient, with the exception of an application to astiff thoracic spine from a skilled therapist(Simmonds and Keer, 2007). In this case, multilevelGrades III- to III++ anterior to posterior mobilizationsin the cervical spine at C3-4, C4-5, and C5-6 withUAP forces directed to the inferior segment wereperformed. Grades III – IV++ transverse mobilizationsin the thoracic and lumbar spines were performedfrom T5 to T8 and L4 to L5 as described byMaitland to address hypomobility found at vertebralsegments that reproduced the patient’s pain

complaints. A posterior pelvic rotation was performedin prone, with stabilization of the sacrum at grade III++ (Hengeveld and Banks, 2014b).

Home program follow-up to compliment the mobili-zations included instruction in self first rib mobilizationand 10-s scalene stretch with a towel (1 set of 10 rep 3x/day) and right thoracic rotation in sitting (2 set of 10 reps2–3x/day). Postural correction and proper ergonomicset-up for her work space included adjustment of chairposition, use of lumbar roll, and maintaining a neutralpelvis position by sitting with both feet on the floor.

Myofascial TPR techniques were performed torestore the arthrokinematics of the lumbar spine andpelvis. TPR to the iliopsoas, quadratus lumborum, pir-iformis, and gluteus medius on the right were per-formed. Although the efficacy and reproducibility ofTPR has not been proven (Remvig, Ellis, and Patijn,2008), use of a test-retest method following TPR forAROM of the R hip, cervical, and lumbar spines allshowed positive change. To compliment these techni-ques at home, the patient was instructed in self TPRusing a tennis ball (6.86 cm diameter) to the posteriorright hip musculature and hip flexor stretch in halfkneeling (30-s hold x 3 reps 2–3x/day).

Postural correction exercises included deep cervicalflexor training incorporated into the patient’s existinghome program to address the decreased endurancefound in the craniocervical flexion test. Given thepatient’s history of neck pain, the deep cervical flexorswere weak due to altered motor control characterizedby impaired activation of the deep cervical flexor mus-cles (Jull, O’Leary, and Falla, 2008). Specific training ofthe deep cervical flexors to increase activation is effec-tive to decrease pain and improve the ability to main-tain an upright posture of the cervical spine duringprolonged sitting (Jull, O’Leary, and Falla, 2008).

Strengthening of the deep cervical stabilizers wasinitiated in supine to address the patient’s forwardhead posture and decreased neck flexor endurance.The patient required assistance to achieve full activecraniocervical flexion. This was initiated at the secondsession and continued until the 10th session when thepatient could perform full AROM independently. Thisprogress was maintained through home exercise (5-shold x 10–15 reps 2x/day progressed as able by 5-s holdincreases. Gentle cervical isometrics for stability intoretraction, bilateral lateral flexion and rotation(Dusuncelli et al., 2009) (5-s hold x 15 reps in eachdirection) and repeated cervical retraction while sittingat computer (5-s hold x15 reps every 2 hours) were alsoincluded in the home exercise program.

The patient was unable to use her upper extremitieswithout pain in the cervical spine at initial evaluation.

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Testing showed lower trapezius and periscapular mus-cular dysfunction as evidenced by difficulty recruitingthe lower scapular musculature for depression anddemonstrated scapular dyskinesis. Patients with EDS-HT present with extremity muscle weakness, whichappears not to be caused by reduced muscle mass butrather by intrinsic muscular dysfunction, associated withmuscle pain and fatigue (Rombaut et al., 2010b).Contributing factors to joint instability include impairedproprioception, postural control and muscular strength(DePaepe and Malfait, 2012). Proprioceptive neuromus-cular facilitation (PNF) for the scapula incorporatesfunctional movement patterns by facilitating propermotor recruitment, motor control, and proprioception(Dayle, 2005). The D1 scapular pattern, anterior eleva-tion and posterior depression, was used to encourageproper scapula positioning thus allowing functionalstrengthening without upper trapezius and levator sca-pulae compensation.

In addition to poor scapular control, the patient haddifficulty recruiting both TrA and multifidus groupsand required tactile and verbal cues for the abdominaldrawing-in exercise. Activation of TrA and mulitifdusmuscles plays an important role in postural control(Teyhen et al., 2005; Van, Hides, and Richardson,2006). Re-education and training of these groupsusing the abdominal drawing-in maneuver facilitates aco-contraction of TrA and multifidus muscles to stabi-lize the trunk with active movement (Teyhen et al.,2005). Full recruitment of the proper muscles wasachieved by visit 6 allowing progression to lowerabdominal and lumbar stabilization exercises.

Hip-strengthening exercises were initiated in anunloaded position and gradually progressed to weightbearing according to the patient’s ability and tolerance.Self-neurodynamic mobilization for the common pero-neal nerve, as described by Butler (1991) in sitting orsupine was also incorporated into the home program.

After 20 physical therapy visits, the patient subjec-tively reported 60% overall improvement since the startof care. She was making a conscious effort to performcervical and scapular retractions to maintain neutralposition when sitting, especially at her computer tominimize pain in the left upper shoulder and mid thor-acic region. The intermittent radiating or “knife-like”pain around the left posterior thorax to the anteriortrunk and tingling into bilateral upper extremities hadresolved completely. Pain, spinal ROM, strength, andoutcome measures are shown in Tables 2, 3, and 4.

Left lower cervical and left lumbar extension quad-rants continued to be provocative motions. While allspinal mobility had improved, continued manual treat-ment was indicated due to persisting motion

limitations. At this time, pain level was stable so thepatient’s frequency of direct treatment was decreased toonce per week. This frequency was continued for anadditional 12 weeks and then decreased to once everyother week until discharge. The home exercise programwas progressed each session as appropriate and qualityof movement monitored for all exercises. Manual treat-ments were performed as needed to normalize musclelength and spinal mobility. Progress during this treat-ment period was variable with some weeks of progressand others with exacerbated pain. Cumulative progressreached a plateau after 10 months from the initialevaluation (Tables 2, 3 and 4). The patient was able tomanage all symptoms and could perform all activitiesinitially listed on PSFS with minimal to no pain. Shewas formally discharged from physical therapy to acomprehensive home exercise and management plan.

Outcomes

At discharge, cervical and lumbar AROM had beenrestored to painfree and functional limits in all planes.The patient continued to have minimal limitation inthoracic rotation right and left without pain (Table 2).Periscapular strength increased to 4-/5 bilaterally andhip musculature strength increased to 4/5 bilaterally(Table 3). The patient was able to efficiently recruitboth TrA and multifidus groups for core stability.Deep neck flexor endurance increased to 23 s. Therewas still a deficit when compared to norms establishedin a cross-sectional study by Painkra et al. (2014) inwhich healthy women, with a mean age of 24.06 years,demonstrated a mean time of 38.43 s.

The PSFS score increased to 8.4 (42/50) and pain levelsdecreased to 1–2/10 without exertion (Table 4). Themini-mally clinically important difference (MCID) has beenreported as 2.0 PSFS points to be a clinically meaningfuloutcome measure (Horn et al., 2012). Pain has a minimaldetectible change (MDC) of approximately three NPRSpoints for patients with musculoskeletal issues (Spadoni,Stratford, Solomon, and Wishart, 2004).

A follow-up phone call was made to the patient 8-month post-discharge. She reported poor compliancewith HEP due to hectic home and work life. Thepatient did report using the strategies given to her intherapy to successfully manage her pain. Her currentscore on the PSFS had dropped from 8.4 at discharge to7.4 (37/50).

Discussion

This study shows the efficacy of physical therapy inter-vention to reduce pain and improve functional mobility

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in a patient with EDS-HT. Rombaut et al. (2011) per-formed a cross-sectional study that confirmed 63.4% ofEDS-HT patients reported positive effects from physi-cal therapy interventions which included musclestrength training, massage, stabilization training, elec-trotherapy, aquatic therapy, and manual therapies.There are published case reports (Russek, 2000;Simmonds and Keer, 2008) demonstrating the efficacyof physical therapy for hypermobility, however nonethat outline specific manual treatment used for thehypo- and hypermobility features documented in thiscase or the 18-month duration of patient management.

Exercise is an essential technique in rehabilitation;however, evidence to determine the most advantageoustype, frequency, dosage, duration, or delivery method islacking in EDS-HT articles (Smith et al., 2014). A recentpilot study (Espi-Lopez, Ingles, Ruescas-Nicolau, andMoreno-Segura, 2016) looked at the effect of low-impactaerobic exercise combined withmusic therapy on patientswith Fibromyalgia, a disease characterized by chronicwidespread pain. Results showed the two groups whoparticipated in aerobic exercise with and without musictherapy both had significant improvements in generaldiscomfort, displayed lower depression levels, andimproved quality of life. It is the belief of the authorsthat patients with chronic pain must be encouraged toparticipate in voluntary enjoyable activities to attain max-imal psychosocial benefits.

The literature supports treatment strategies consist-ing of proprioceptive and motor control re-educationto restore joint stability. Postural and functional train-ing are also key elements in rehabilitation (Grahame,2009; Rombaut et al., 2011; Simmonds and Keer, 2007).The prudent use of mobilization techniques to restorenormal arthrokinematics in hypomobile spinal seg-ments or peripheral joints is appropriate to addressmechanical pain (Grahame, 2009; Simmonds andKeer, 2007). The Maitland framework allowed the phy-sical therapist to make clinical decisions based on thecontinual assessment of the patient’s presentation andresponse to chosen manual techniques. This approachcontributed to a successful outcome in this case.

This case clearly illustrates the importance of using amultimodal patient-centered approach in the manage-ment of musculoskeletal pain in a patient with EDS-HT.Once the patient’s pain is managed and functional mobi-lity is restored, a maintenance program of exercises can beestablished with emphasis on joint protection. The patientin this case was able to accept she would not be able toreturn to a high intensity level of physical training giventhe EDS-HT diagnosis and right hip pathology. She wasable to resume a mild to moderate active lifestyle thatincludes low-impact cycling, walking, and hiking with

family and friends. The patient had high satisfaction atthe conclusion of therapy and she is able to self-manageher condition.

Clinical relevance

Physical therapists must recognize that a slow deliberateprocess is needed to successfully rehabilitate a patientwith EDS-HT. Periods of regression are normal andshould not be considered a negative response to treatmentor reason to discharge before goals are met. The 10-month time frame for the management of this case waslonger in duration than the same chronic musculoskeletalcomplaints without EDS-HT. Monitoring of the patient’sprogress during the 10-month duration ensured shestayed on course to achievemaximal recovery of function.The patient reportedmild regression at the 8-month post-discharge follow-up due to poor compliance with HEPbut her condition was still above baseline and she was ableto return to low-impact activities that she enjoys. It maybe beneficial to have routine monitoring of this popula-tion at consistent intervals to ensure they are continuingto be successful in self management or have the need foradditional physical therapy intervention.

Following a consistent framework is imperative andwill enable the physical therapist to make justified clinicaldecisions. TheMaitland concept is an example of a logicalframework that facilitates sound judgment during patientmanagement. Because no two patients with JHS presentthe same, it is imperative that a treatment plan is custo-mized to his or her particular problems. The role ofeducation and empowering the patient is of utmostimportance. Maintaining a home exercise program andadaptation and modifications of work and lifestyle activ-ities are the key to optimizing therapeutic outcomes long-term for this patient population.

Acknowledgments

The author would like to thank Suzanne Brown, PhD, MPH,PT for being an advisor and for assisting in the editingprocess of this article. This work was completed as a require-ment of Dr. Pennetti’s doctor of physical therapy degree atUniversity of New England, Portland, Maine, USA.

Declaration of Interest

The authors report no declarations of interest.

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