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Clinical Reasoning Considerations For Clinical Reasoning Considerations Kaitlin Barnet, SPT1 for the “Flexible” Patient: Megan Barker, PT, DPT, OCS1 1 A Ligamentous Laxity Overview Joseph Signorino, PT, DPT, OCS, FAAOMPT 1The George Washington University, Program in Physical Therapy, Department of Health, Human Function and Rehabilitation Sciences, Washington, DC ABSTRACT supported by a large prevalence range from ate the specific needs in this hypermobile Background and Purpose: Individuals 2-57% for individuals with gJHM indicating population. with excessive joint hypermobility often seek inclusion criteria remains uncertain.3 Typi- The aim of this article is to present a review physical therapy care. Despite the common cally, a Beighton score of 4 out of 9 indicates of the literature regarding JHS and offer clin- clinical occurrence, a consensus is lacking on gJHM in a general adult population.1 There ical information to conservatively manage how to best conservatively address the specific are some additional studies indicating gJHM individuals with suspected or confirmed joint needs of this poorly understood population. is present in women if a score of 5 of 9 is hypermobility syndromes. Lastly, the article The aim is to explore concepts and clinical achieved, and a score of at least 6 out of 9 is could serve to identify knowledge gaps and reasoning considerations when treating a needed to determine the presence of gJHM areas for future research. person with a joint hypermobility syndrome in children.1,2 (JHS). Methods: A pragmatic outline was Joint hypermobility syndrome (JHS) JOINT HYPERMOBILITY established including clinical manifestations, occurs when joint hypermobility becomes CONDITIONS AND CLINICAL evaluation, prognosis, and clinical reason- symptomatic. These symptoms were previ- MANIFESTATIONS ing processes to determine intervention. The ously believed to be only limited to localized Individuals with joint hypermobility literature was identified through PubMed pain, instability, and decreased propriocep- often present to physical therapy due to joint and CINAHL. Clinical Relevance: Only a tion. However, a progressive understanding pain.6 Physical therapists must recognize the subset of individuals with joint hypermobil- appreciates this condition is much more patient’s underlying condition and how it is ity become symptomatic. Joint hypermobil- complex. Due to the nature of the tissues contributing to their current complaint. The ity syndrome includes many ligamentous affected, the condition can present in a physical therapist should appreciate the vari- laxity conditions requiring the clinician to variety of ways. In addition to the musculo- ous characteristics these conditions present appreciate different disease characteristics. skeletal complaints, such as increased likeli- with in order to properly address the indi- There are questionnaires and objective evalu- hood for joint sprains, meniscal injuries, vidual patient’s needs. ation tools available to assist with developing and stress fractures, other body systems are The common characteristics of EDS, individualized treatment. Conclusion: The affected manifesting as disturbances in pain Marfan syndrome, Osteogenesis imperfecta, evaluation and construction of a meaningful perception, anxiety, fatigue, and gastrointes- and Down’s syndrome are listed in Table 1. treatment plan for individuals with JHS can tinal interruptions.4-7 Congenital conditions Table 1 identifies the most common condi- be challenging. Combined clinical knowl- that present with ligamentous laxity and tion characteristics that may help construct edge and sound clinical reasoning processes subsequent joint hypermobility are Down’s a differential diagnosis, although it is not a can assist with optimizing outcome. syndrome, Marfan syndrome, Loeys-Dietz complete list of symptoms related to joint syndrome, and Osteogenesis imperfecta.8-10 hypermobility. Ehlers-Danlos syndrome Key Words: Ehlers-Danlos, generalized Joint hypermobility syndrome is considered presents with many different types. Ehlers- hypermobility, joint hypermobility by some sources to be a mild form of Ehlers- Danlos syndrome, hypermobility type III is Danlos syndrome (EDS) hypermobility type the most common and has an almost iden- INTRODUCTION while other sources indicate JHS is a diagno- tical clinical presentation to JHS.4,12 Unfor- Joint hypermobility is defined as the sis of exclusion and separate from EDS.8 tunately, JHS can often be considered a ability of a joint to move past the clinically Conservative management by a physical diagnosis of exclusion.8 defined normal standards for range of motion therapist is often the preferred first method In addition to musculoskeletal com- (ROM).1 It can occur at one joint or at mul- of treatment for these conditions due to plaints, many individuals may report high tiple joints throughout the body. When their musculoskeletal nature. Treatment can levels of fatigue, depression, and anxiety with excessive motion occurs in multiple joints, vary from stability exercises, proprioception any ligamentous laxity condition.8 Other it is characterized as generalized joint hyper- training, and patient education. Patient edu- clinical observations may include a lack of mobility (gJHM). Generalized joint hyper- cation is focused on modifying movement, proprioception, generalized hyperalgesia, mobility is asymptomatic with no functional lifestyle changes, and addressing persistent various neuropathies including tarsal tunnel loss despite having increased ROM. Identify- pain.11 Unfortunately, there is little consensus and carpal tunnel syndrome, ptosis, varicose ing an individual with gJHM is often made for the best way to manage individuals with veins, low bone density, and postural ortho- using the Beighton score.1 The cut-off scores a joint hypermobility condition; therefore, static tachycardia syndrome. Patients may for the Beighton assessment are inconsistent an increased awareness and understanding present with bowel and bladder dysfunction, and lack a consensus on how to best iden- of JHS is important as physical therapists including pelvic organ prolapse.8,13 tify individuals with gJHM.1,2 This is further are the best health care provider to appreci- Orthopaedic Practice volume 32 / number 3 / 2020 137 9000_OP_July.indd 19 6/22/20 12:33 PM CLINICAL EVALUATION Table 1. Review of Specific Joint Hypermobility Syndromes Along with Common Individuals may arrive to physical therapy Characteristics to Assist with Recognition with an undiagnosed ligamentous laxity con- dition. It would be prudent for the physical Joint Hypermobility Syndromes Common Clinical Presentations therapist to properly screen for ligamentous Ehlers-Danlos syndrome8 Bilateral clubfoot laxity and consider referral to the proper Developmental delays medical provider for diagnosis and additional • Classic Type (I) Dysmorphic facies management. • Vascular Type (IV) Extensive and easy bruising A thorough subjective history is recom- • Kyphoscoliotic Type (VI-A) Large hernias mended during a clinical evaluation for an • Musculocontractural Type (VI-B) Marfanoid habitus • Dermatosparaxis Type Muscle weakness individual with suspected JHS. The subjec- Scleral fragility tive intake should aim to gain an understand- Scoliosis ing of the current and past injury and health Sensory neural hearing loss history, mechanism of injury, and aggravat- Severe muscle dystonia ing and alleviating factors. Identifying how Severe muscle hypotonia Skin hyperextensibility these complaints influence functional loss is Thin translucent skin important. Velvety skin texture The objective evaluation should include ROM measurements while noting if these Marfan syndrome8 Ascending aorta dilation are outside of typical norms. Strength mea- Fingers and toes abnormally long and slender Funnel chest sures and a general neurological screen High palate should be assessed. Blood pressure and heart Muscle hypoplasia rate measurements within the initial session Scoliosis is advisable due to the common occurrence 10 of related hypotension. Functional tasks Osteogenesis imperfecta Aortic root dilation Conductive deafness should be observed to understand the indi- Decreased pulmonary function vidual’s movement strategies, motor control, Heart murmurs and compensations. Both daily functional Scoliosis tasks and sport specific tasks should also be Teeth discoloration observed. Down’s syndrome9 Brachycephaly Flat nasal bridge Subjective Examination Folded ear The Hakim and Grahame questionnaire Gap between 1st and 2nd toes (Table 2) and musculoskeletal and non-mus- Incurved 5th finger culoskeletal screening questions (Table 3) Muscular hypotonia Narrow palate can assist with developing a list of differential Nystagmus diagnoses.8,13,14 It is important to investigate Oblique eye fissure the timeline of symptom development, espe- Short neck cially childhood presentations, to determine a progression or long-standing presentation of related injuries or pain. The individual may describe multi-system involvement, 17,18 including gastrointestinal, vascular, and questions from Table 2 can lead the clini- tibular and somatosensory dysfunction. bowel/bladder issues. They may report clum- cian to perform movements described in the Static measures may include single leg siness, unsteadiness, or coordination deficits. Beighton score during the examination for stance with eyes open, eyes open with cervi- 17,18 After ruling out more serious pathologies, additional objective data. This information cal extension, and
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