Table 4.

Hypermobile Ehlers-Danlos Syndrome: Recommended Surveillance

System/ConcernEvaluationFrequency
Musculoskeletal Assess for joint manifestations & current degree of pain & disability.Annually or at each visit
DXA scanIn those w/height loss >1 inch, atypical/low trauma fractures, or radiographs suggestive of osteopenia
Hematologic Assess for bleeding issues.Annually or at each visit
Gastrointestinal Assess for clinical manifestations of functional bowel disorders.
Cardiac Assess for clinical manifestations of autonomic dysfunction.
EchocardiographyIn those w/aortic root dilatation on initial echocardiogram: 1
  • Follow-up echocardiogram 1 yr after initial echocardiogram & if no change, follow-up echocardiogram in 5 yrs
  • In those w/progressive aortic dilatation: continue annual echocardiography; if progressive dilatation stops, then echocardiogram every 3 yrs
Oral/
Dental 		Assess for orthodontia needs & periodontal disease.Annually or at each visit
ENT/
Pulmonary
  • Assess for phonation & respiratory issues.
  • Assess for sleep issues.
Ocular Assess for xerophthalmia, refractive errors, & diplopia.
Neurologic Assess for headaches, cord & nerve root pathology, dystonia, & peripheral neuropathy.
Inflammation Assess for soft tissue inflammation, co-occurring rheumatologic disorders, & mast cell activation disorders.
Neurobehavioral/
Psychiatric 		Psychosocial assessment for depression, anxiety, affective disorder, autism, & ADHD
Urogynecologic Assess for interstitial cystitis, urinary incontinence, pelvic organ prolapse, dysmenorrhea, menorrhagia & dyspareunia.

ADHD = attention-deficit/hyperactivity disorder; DXA = dual-energy x-ray absorptiometry

1.

Since the risk of progression of aortic root dilatation has not yet been defined by longitudinal studies, surveillance should follow the American College of Cardiology / American Heart Association (ACC/AHA) guidelines for EDS [Isselbacher et al 2022].

From: Hypermobile Ehlers-Danlos Syndrome

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