This is to be used as the location for my complied tips / recommendations / general principles for the management of the above condition:

Goal for PCP: emphasize (1) patient education, (2) prevention, and (3) early recognition of injuries to avoid or reduce complications when possible.

Patient Education:

Monitoring:

CARDIOVASCULAR:

• Echocardiogram
  • obtain baseline
  • for detection of cardiovascular involvement including cardiac valvular and vascular disease and aortic dilation
  • frequency: every 2-5 years if baseline is reassuring

OPHTHALMOLOGIC:

• Examinations
  • to detect early retinal or scleral fragility, myopia, and glaucoma.
  • frequency: typically yearly

Scoliosis/Spinal Disease

for patients with scoliosis or spinal disease:

• evaluation of cervical spine instability with cervical spine flexion/extension films and MRI may be indicated
• patient should be evaluated by a spinal specialist if they develop scoliosis, peripheral neuropathy, or new neck pain

Anesthetic and Perioperative Management

Assessment of bleeding history, cardiovascular history, and difficulties with intubation and wound healing.

Use of noninvasive monitoring whenever possible.

Careful attention to patient positioning for the procedure, with appropriate padding and caution with the use of tourniquets in those with tissue fragility.

Careful fluid monitoring and blood pressure control throughout the pre-, intra-, and postoperative periods.

Attention to difficulties with airway management, which are more frequent than in the general population, including potential bleeding, tissue damage, and joint dislocation.

Careful assessment of patients with temporomandibular dysfunction and cervical spine instability.

Management of bleeding, which should be individualized. Some patients benefit from use of desmopressin.

Local anesthesia may be less effective in some patients.

Hypermobile EDS

Musculoskeletal preventative care and symptom management: