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\begin{document}

\title{The value of Dynamic Movement Orthoses in the management of a complex
Hypermobile Ehlers-Danlos Syndrome patient: a case report}



\author[1]{Anna Higo}%
\author[1]{Gemma Pearce}%
\author[1]{Shea Palmer}%
\author[2]{Louise Grant}%
\affil[1]{Coventry University}%
\affil[2]{Physiocure}%


\vspace{-1em}



  
  \date{June 15, 2024}


\begingroup
\let\center\flushleft
\let\endcenter\endflushleft
\maketitle
\endgroup





\selectlanguage{english}
\begin{abstract}
The use of Dynamic Movement Orthoses\selectlanguage{ngerman}® is examined in a young woman with
hypermobile Ehlers-Danlos syndrome (hEDS) referred for physiotherapy
with hip dysplasia, prior to a right peri-acetabular osteotomy. Dynamic
Movement Orthoses® plus rigorous subjective examination, therapists'
listening skills and patient-centred goals was useful for this hEDS
patient.%
\end{abstract}\selectlanguage{ngerman}%



\sloppy


The value of Dynamic Movement Orthoses\selectlanguage{ngerman}® in the management of a complex
Hypermobile Ehlers-Danlos Syndrome patient: a case report.

\textbf{Key Clinical Message / Abstract}

The use of Dynamic Movement Orthoses® is examined in a young woman with
hypermobile Ehlers-Danlos syndrome (hEDS) referred for physiotherapy
with hip dysplasia, prior to a right peri-acetabular osteotomy. Dynamic
Movement Orthoses® plus rigorous subjective examination, therapists'
listening skills and patient-centred goals was useful for this hEDS
patient.

\textbf{Key words:} Hypermobile Ehlers Danlos Syndrome, Dynamic Movement
Orthoses®, Hip Dysplasia, Periacetabular Osteotomy, Physiotherapy, joint
subluxation, hypermobility, Postural Tachycardia syndrome.



\textbf{Introduction}

The Ehlers-Danlos Syndromes (EDS) are a group of heritable connective
tissue disorders characterised by joint hypermobility, tissue fragility
and skin extensibility {[}1{]}. Hypermobile EDS (hEDS) is the most
common subtype, and is associated with pain, activity limitations and
participation restrictions {[}2{]}. hEDS is diagnosed through clinical
examination using the 2017 International Classification {[}1,3{]}.
Additional consideration must also be given to the comorbidities that
exist alongside the main condition due to its multisystemic nature.
These include Postural Tachycardia Syndrome (PoTS) {[}4{]}, Mast Cell
Activation Syndrome (MCAS) {[}5{]}, gastrointestinal issues and bladder
and bowel problems {[}6{]}. These comorbidities can play an important
role when it comes to rehabilitation planning {[}7{]}. Comorbidities are
associated with worse health outcomes, more complex clinical management,
and increased health care costs {[}8{]}.

This group of patients are poorly recognised and poorly understood, as
highlighted in recent studies of~healthcare experiences {[}9, 10, 11{]}.
Physiotherapists play a central role in management, yet many
practitioners fail to recognise the complexity of these patients and
that inappropriate treatment may be detrimental to their management
{[}7,12{]}. Existing studies have highlighted the psychological impact
of this condition and recommend early detection to assist optimum
management {[}9,13{]}. Health care practitioners therefore need
increased awareness of hEDS.

Dynamic Movement Orthoses (DMOs)® are fabric elastomeric orthotic
garments that have strategic reinforcement biomechanical panelling
{[}14{]} and are a type of Dynamic Elastomeric Fabric Orthoses (DEFO)
{[}15{]}. Therefore, they may help people with hEDS by exerting a
customised paratonic torsional, compressive and supportive effect that
could positively influence alignment biomechanics, and neuromuscular
activity specific to the individual and their body segments {[}14{]}.
DEFOs are different to traditional braces which may restrict movement
and prevent muscle activity {[}15{]}. They can also be individually
tailored to the patient's needs with specific . Case reports have shown
their successful use in improving form and/or function in a variety of
patients with movement control problems {[}15,16{]}. A study of six hEDS
patients and six matched controls demonstrated that postural control
impairment was partially offset by wearing somatosensory orthoses
{[}17{]}. Simmonds et al {[}18{]} suggested pain, fatigue and fear of
injury are common barriers to exercise. Therefore, in the hEDS patient
who commonly suffers with pain, impaired proprioception, poor muscle
control, increased tissue elasticity {[}19, 20{]}, and reduced function;
DMOs® could be considered as a first line of treatment.

PoTS, a co-morbidity found in EDS {[}21{]}, is defined as a clinical
syndrome lasting at least 6 months that is characterized by: 1) an
increase in heart rate \selectlanguage{english}[?]30 beats per minute within 5 to 10 min of quiet
standing or upright tilt (or \selectlanguage{english}[?]40 beats per minute in individuals 12 to
19 years of age); 2) the absence of orthostatic hypotension
(\textgreater{}20 mm Hg drop in systolic blood pressure); and 3)
frequent symptoms that occur with standing such as light headedness,
palpitations, tremulousness, generalized weakness, blurred vision,
exercise intolerance, and fatigue {[}22{]}. Compression garments have
been highlighted as a tool in the management of PoTS {[}23, 24{]},
therefore the compressive effects of DMOs(r) might help to reduce
symptoms. The management of hEDS must focus on treatment interventions
that allow these patients to function, addressing ``injury prevention
and symptom amelioration rather than a cure'' {[}25{]}.

\textbf{Case History and Examination}

The patient was a 22-year-old female Caucasian (height 160cm, weight
70kg) living in the United Kingdom (UK) with bilateral Developmental
Dysplasia of the Hip (DDH) with a radiographic Lateral Centre Edge Angle
(LCEA) of 15o on the right and 17o. A LCEA of \textless{} 20o is
considered to be dysplastic {[}26{]}. She had been referred for
pre-operative physiotherapy by her hip surgeon, in preparation for a
right hip peri-acetabular osteotomy {[}27{]}. The right hip operation
was scheduled in 16 weeks' time and the left hip operation a year later;
providing surgical outcomes were satisfactory on the right side. The
patient has formally consented to her case being published, having
considered and approved this manuscript.

\textbf{Subjective history}

The patient completed self-reported questionnaires, namely the
international Hip Outcome Tool (iHOT-33) {[}28{]}, the EuroQol 5
dimensions (EQ-5D-5L) {[}29{]} and numerical pain rating scale (NPRS)
{[}30{]} as displayed in \textbf{Table I} . She described right hip pain
with a 9-month history, in a `C-sign distribution' (groin, lateral hip
and buttock) {[}31{]}. Pain was reported at night and affected all
activities, including sitting and standing (both limited to 20 minutes)
which affected her being able to work in her office job. Yoga used to be
her favourite form of exercise as she could adopt the positions easily,
but that was also now painful. She tried physiotherapy locally for the
last 8 months, but the exercises caused her hip pain to flare up, so she
stopped doing any exercise. Physiotherapy exercises previously
prescribed had aimed to improve gluteal strength {[}32{]}.

In addition to her DDH, she reported endometriosis, polycystic ovaries,
hay fever, irritable bowel syndrome, heartburn, reflux, depression and
chronic fatigue. Medications taken were Codeine for pain relief 15mg
once a day, paracetamol 200mg, 2-3 times a day, Duloxetine 60mg once a
day, and Provera 25mg once a day.

\textbf{Objective examination}

The patient walked into the clinic independently mobile with no walking
aid but had a slight bilateral Trendelenberg walking pattern and stood
predominantly weight bearing through the left leg. Initial observations
were that the patient stood in a sway back posture (viewed from a
lateral aspect, the greater trochanter of her hips bilaterally was
positioned anterior to both the lateral aspect of the shoulder and
lateral malleolus), with hyperextended knees and bilaterally pronated
feet. On sitting she had her legs tightly crossed, with her feet tucked
around one of the chair legs. The Beighton's hypermobility scale was 7/9
{[}3, 33, 34{]} with negative findings only for her thumb joints. The
scale was explained to the patient and she reported this had never been
performed on her before and she had never been told about hEDS in any of
her past healthcare experiences. A preliminary hypermobility discussion
ensued and it was explained that some patients experience a range of
common co-morbidities. This resulted in the patient disclosing that she
bruised easily, had unexplained stretch marks on the skin around her
trunk, widened atrophic scars from wounds from childhood injuries (from
falling over), suffered recurrent ankle sprains, previous left wrist
subluxations, allergies, and that she experienced dizziness and light
headedness upon standing (and on a few occasions had fainted and
fallen). She expressed how she often did not tell people about all of
her symptoms as she was afraid of not being believed and being labelled
as a hypochondriac.

Isometric muscle strength tests of the hips using a Hand-Held
Dynamometer (HHD) were carried out to record pre-operative benchmark
measurements. The testing protocol used was as per the Hip Arthroscopy
Pre-habilitation Intervention (HAPI) study {[}35{]} with the addition of
hip internal rotation in prone described by Thorborg et al {[}36{]}.
Measurements are presented in \textbf{Table II} . Hip flexion and hip
extension strength tests were not recorded due to the patient's high
pain level with these movements. Functional movements such as squatting,
bridges, one leg balance, single leg squat, step-ups, and gait were
observed, alongside assessment of proprioception and motor control. All
of these highlighted significant weakness, poor proprioception and poor
neuromuscular control. Due to her history of recurrent ankle sprains,
clinical observation of bilateral foot pronation and the understanding
of the importance of ankle push-off function in reducing pressures
through the anterior hip joint {[}37{]}, the feet and ankles were
assessed in detail. Passive tests of the ankles and feet showed
excessive range of motion and ligament laxity {[}38{]}. Joint
hypermobility can be found in joints outside of the Beighton score so a
whole-body approach was adopted {[}20, 34{]}. Single leg calf raise
ability, in supported one leg stance, was a total of 5 repetitions on
each side before fatigue set in, which is a significantly lower value
than would be expected in an average healthy adult {[}39{]}. It is
normal following peri-acetabular osteotomy surgery for a patient to use
crutches and to have a weight restriction through the operated leg
{[}40{]}; thus, the non-operated leg must be of sufficient strength to
cope with this.

\textbf{Problem list}

\begin{itemize}
\tightlist
\item
  Poor proprioceptive awareness
\item
  Unable to improve pre-operative function and strength, due to pain
\item
  Safety concerns with dizziness, light headedness and fainting
  episodes, combined with reduced mobility
\item
  Low mood due to decreased function
\end{itemize}

\textbf{Differential diagnosis, investigations and treatment} After
discussion, and with the patient's explicit consent, a letter was
written to their Primary Healthcare Physician, General Practitioner
(GP), to request a referral to a specialist Rheumatologist who dealt
regularly with EDS and other connective tissue disorders. The specialist
Rheumatologist diagnosed the patient with hEDS using the 2017
International Classification {[}1, 3{]} and they also suspected PoTS.
Therefore, they made an onward referral to a Cardiologist and for a tilt
table test {[}4, 22, 23{]}. The patient reported that she felt listened
to, felt the value of understanding her own body better and felt having
a `label' helped her explain her problems to family, friends and work
colleagues {[}9{]}. She shared with us that she had previously `lost her
faith' in physiotherapy, as exercises prescribed by past practitioners
had caused pain exacerbation, so it was important for us to build trust
and a relationship to help find a way of improving her strength,
proprioception and function.

To address the issues listed on the problem list we suggested a trial of
DMO(r) leggings which not only provide compression to aid blood pressure
in the legs {[}23{]}, but also provide proprioceptive feedback to aid
her postural control. With this patient we wanted to reduce her sway
back posture and knee hyperextension as we found that this decreased her
pain. Sway back posture has been reported to increase the joint pressure
around the anterior acetabulum, the area which is often inadequately
covered by the hip socket in DDH {[}37{]}. The patient's decreased
awareness of her body posture meant she was unable to maintain the
position which decreased her pain, therefore we wanted to assess to see
if a DMO(r), in the form of leggings which also encompassed the hip and
pelvic region, could assist in reducing her sway back posture. Core
control, hip, lower limb, breathing and proprioceptive exercises
{[}41{]} were tailored to her needs so that they were manageable; paced
to accommodate her pain, chronic fatigue and dizziness; and were
relevant to helping her function post-operatively. In a systematic
review of exercise programmes for hEDS patients, a period of 4-8 weeks
was commonly reported in studies {[}41{]}. Our exercise programme had a
longer time span (16 weeks) as it was dictated by the planned surgery
date.

\textbf{Outcome and follow up}

Patient measurements were reassessed at 8 and 16 weeks,
post-intervention (\textbf{Tables I and II)} . Both subjective and
objective outcome measurements showed positive improvements with the
iHot-33 score increasing by 31 points. The minimal clinically important
difference (MCID) is 6 points {[}28{]}. The patient felt in less pain
and reported the DMO(r) made her feel more co-ordinated and stable. This
resulted in her being able to engage in her physiotherapy exercises to
prepare for hip surgery. Increases in muscle strength were demonstrated
by HHD tests with her hip abductors, hip adductors, hip internal and hip
external rotators. Reduction in her symptoms of light-headedness,
dizziness and palpitations were notable and promising in respect of
improvements to her safety and prevention of falls. \textbf{Figure I}are
photographs showing the patient without the orthoses and their sway back
posture and a comparative photograph to show her posture changes whilst
wearing the orthoses. Her posture in standing whilst wearing the
orthotics, was significantly more vertically aligned. These improvements
also carried over into her walking posture; and most importantly,
resulted in decreased pain and heightened body awareness.

\textbf{Discussion}

This report highlights a novel approach to finding a practical solution
to facilitate pre-operative physiotherapy prior to hip surgery for DDH
in a patient with multiple problems that were presenting as a barrier to
exercise engagement. The use of DMO(r) garments could be considered as an
intervention to allow improved function in people with hEDS and PoTs. A
garment which encompasses the abdomen and lower body, and exerts a
compressive force, has previously been shown to reduce heart rate and
improve PoTS symptoms in adult patients {[}42{]}. Reduced lower limb
proprioception is a common problem in hEDS patients {[}43{]} and joint
hypermobility has been noted to be a key factor in hip dysplasia
{[}44{]}; thus, an understanding of these elements can help support this
group of patients. This case study builds on Dupuy et al's pilot study
improving postural control with DMOs(r) in people with hEDS {[}17{]}.
Fatigue is a common debilitating complaint associated with PoTs {[}4,
45{]}, and emerging evidence supports the use of compression garments to
improve fatigue {[}46{]}.

We have highlighted the multisystemic nature of hEDS, and the importance
of a thorough subjective examination, particularly the past medical
history and the need for appropriate multi-systemic questioning
{[}38{]}. Our case report also emphasises the importance of recognising
when timely onward referral is required to suitable medical specialists.
hEDS patients have often had a long journey to diagnosis, with published
research reporting a mean of 14 years elapsing between the first
clinical manifestations and the actual diagnosis {[}47{]}. Patients
therefore often experience depression and anxiety after years of not
being listened to and being discredited {[}20{]}. To finally have
someone that listens and subsequently acknowledges their multiple issues
can often be the first step in their recovery.

In the example of the patient used in this case study, inappropriate
treatment led to pain-related fear, fear avoidance and further
deconditioning. This is unfortunately common in hEDS patients {[}48{]}.
Pain-related fear occurs when situations that are related to pain are
seen as a main threat {[}49{]}. This can be fear of movement, fear of
injury or reinjury or fear of pain {[}50{]}. Avoidance behaviour is
defined as postponing or preventing a potentially aversive situation
from occurring {[}51{]}. Such behaviour will lead to a diminished
quality of life as the patient has difficulties in physical functioning,
mood and social functioning {[}52{]}. hEDS management overlaps with that
of Hypermobile Spectrum Disorder (HSD) {[}11{]}, so these findings can
be useful for HSD patients too. The majority of patients with HSD/hEDS
have heightened fear of movement {[}53{]} and increased vulnerability to
injury {[}38{]}. Long-term physical inactivity leads to physical
deconditioning {[}54{]}, including decreased muscle strength and
cardio-respiratory fitness. Despite studies identifying kinesiophobia as
a possible barrier to exercise {[}53{]}, the mainstay of treatment for
HSD/hEDs is exercise and pain management {[}41{]}. Literature conveys
that these patients already have higher levels of anxiety {[}55{]},
which may also have a dysautonomia element with symptoms such as
hyperventiliation, nausea and light-headedness which are significantly
more common in HSD/hEDS patients {[}56{]}. Van Meulenbroek et al
{[}57{]} found that adolescents with asymptomatic generalised joint
hypermobility had the same level of physical functioning compared to
non-hypermobile controls. The lower levels of physical functioning
observed in adolescents with HSD/hEDS could therefore not only be
explained by the presence of generalised joint hypermobility; other
issues need to be considered. Within clinical practice, treatment needs
to address other factors such as pain, fatigue, multisystemic
dysfunction, loss of postural control and pain-related fear {[}47,
50{]}.

Via the use of the DMOs(r), we were able to tackle the key factors on this
patient's problem list and bring about positive changes, enabling them
with strategies to overcome barriers to exercise. It is important to
note that there were no adverse effects reported by the patient on
wearing the DMOs(r)\selectlanguage{english}[?]. Considering appropriate DMOs(r)/DEFOs for hEDS/HSD
patients could provide the individual with a beneficial level of
support. This in turn could decrease pain, improve function and increase
confidence in their ability. This confidence might allow them to begin a
carefully graduated, patient-specific strengthening programme, thus
minimising fear-avoidance and potential injury; and in this specific
case report, prepare physically for upcoming major surgery. Not only can
the garments be used for the musculoskeletal system, but as this case
indicates, they are also potentially beneficial in addressing the multi
systemic nature of HSD/hEDS by reducing PoTS symptoms that in this
instance was a safety concern with imminent orthopaedic surgery
{[}58{]}. It was paramount in this case to ensure the patient had a
reduced risk of fainting and falling to avoid undue stress to her
peri-acetabular osteotomy and the newly forming bony union {[}40{]}.
This case study demonstrates the need for further robust studies into
the use of these orthoses for individual joint problems, multiple joint
issues, chronic fatigue and dysautonomia.

\textbf{Acknowledgements}

Thank you very much to the patient who consented to their case study
being used for this publication. We also thank all clinical staff who
participated in the treatment of the patient. `Dynamic Movement
Orthoses(r)\selectlanguage{english}[?] has been used with the kind permission of DM Orthotics'.

\textbf{Authorship Contributions}

Author 1 \& 4: Treated the patient and wrote up the first draft of the
paper.

Author 2 \& 3: Advised author 1 and 4 and edited the draft of the paper
for publication.

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