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. 2022 Jul 20;37(9):1693–1709. doi: 10.1111/jgh.15927

Table 4.

Checklist for a patient presenting with gastrointestinal symptoms potentially associated with HSD/hEDS

Actions Notes
Screen patients for hEDS with 5‐point questionnaire
  • 5‐point questionnaire 10
1. Can you now (or could you ever) place your hands flat on the floor without bending your knees?
2. Can you now (or could you ever) bend your thumb to touch your forearm?
3. As a child, did you amuse your friends by contorting your body into strange shapes, or could you do the splits?
4. As a child or teenager did your shoulder or kneecap dislocate on more than one occasion?
5. Do you consider yourself double‐jointed?
  • Consider new diagnosis of hEDS in patients with multisystemic symptoms and DGBI
  • Early referral to multi‐disciplinary teams if available
Exclusion of organic gastrointestinal conditions

  • Screening for celiac disease (e.g. celiac‐specific serology if consuming gluten) and inflammatory bowel disease (e.g. fecal calprotectin, colonoscopy if high suspicion) as recommended in any patient with chronic gastrointestinal symptoms

  • In patients with Crohn's disease, consider HSD/hEDS overlap with spondyloarthropathy 34

  • Judicious use of investigations and procedures to minimize duplication of care and iatrogenic risk from low yield procedures. Endoscopic procedures should be performed on their clinical merits. No evidence of increased procedural risks (perforation, post‐procedural pain in HSD; possible increased risk of bleeding in hEDS patients with minor bleeding disorder)
Institute integrated management for DGBI symptoms
Evidence for management specifically in HSD/hEDS lacking–attention to:
  • Integrated care and behavioral therapies 85
  • Dietary management: FODMAP diet efficacious in HSD/hEDS‐related DGBI 89
  • Pelvic floor dysfunction: consider early referral for anorectal physiological assessment and biofeedback/pelvic floor physiotherapy
  • Psychology input to address psychological comorbidity
Consider nutritional and dietary issues
  • Assess nutritional status as undernutrition is common and multifactorial 79
  • Optimization of bone health: vitamin D and calcium supplementation as required
  • Screen for weight loss and disordered eating patterns (dietitian)
  • Consider the following
  • ○
    Underlying eating disorder, in particular avoidant/restrictive food intake disorder (ARFID)
  • ○
    Dental and oral mucosal health, temporomandibular joint (dys)function
  • ○
    Presence of underlying chemosensory disorder (altered taste and smell)
  • ○
    Alteration of diet due to presence of DGBI symptoms
  • ○
    Consider the impact of eating disorder itself on gastrointestinal function (e.g. generation of IBS‐like symptoms, constipation, postprandial fullness, bloating, and early satiety) 81
Address extra‐intestinal manifestations—consider referral to appropriate healthcare professional

  • Increased risk of psychiatric comorbidities

  • Increased risk of ‘at risk’ substance use (alcohol, tobacco)

  • Musculoskeletal involvement often widespread, affecting joints beyond those listed in diagnostic criteria

  • Chronic pain syndromes common—individualized pain management appropriate, awareness of opiate use

  • Chronic fatigue symptoms common—multidisciplinary approach 84

  • Consider contribution of autonomic nervous system‐related symptoms

  • Consider physical deconditioning, which may exacerbate autonomic dysfunction and musculoskeletal symptoms, fatigue and pain

  • Consider referral to cardiologist for surveillance in those with positive family history of cardiac/aortic disease or abnormal cardiovascular clinical examination findings on auscultation; no clear guideline regarding routine/baseline echocardiographic surveillance 2 , 90

Pharmacological considerations

  • Consider the effects of medication on symptoms (e.g. fatigue, sleep quality, and gastrointestinal dysfunction)

  • Caution with opiates, particularly in those with gastrointestinal symptoms
Support

  • Referral to support group/local hEDS organization

  • Providing patients with pathways to obtain further information about the condition and allow family members to consider this diagnosis where appropriate
Professional education and training

  • Further healthcare professional training is available through EDS Society (EDS ECHO), established 2019 with evidence for improved outcomes and physician confidence 20