Identify the problems – Since EDS is an umbrella term for multiple connective tissue disorders, preoperative focus should be on identifying specific associated complications.

Airway evaluation – temporomandibular dysfunction, premature spondylosis, or occipitalatlantoaxial instability may create difficulties during intubation, etc.

Ask about bleeding problems – Laboratory results are usually within normal range and generally not helpful for estimating the bleeding risk. Fragile vascular connective tissue may be the cause of spontaneous bleeding and/or dissection. Positioning, tourniquets, etc may result in abnormal bleeding/bruising. There is a high risk of hematoma and compartment syndromes, as well untreatable diffuse bleeding in EDS subtypes with vascular fragility. Non-invasive monitoring should be employed where possible. Recommended to type and cross blood and to be transfusion-ready.

Desmopressin (DDAVP) – use in patients with positive bleeding history. Desmopressin improves the bleeding time and reduces transfusion requirements by increasing plasma levels of Factor VIII and von-Willebrand-Factor (vWF).

A-line with ultrasound – If invasive monitoring such as an A-line or CVP is absolutely necessary, ultrasound guidance is considered mandatory.

Avoid Pneumothorax – High peak inspiratory pressure (PIP) and laparoscopic surgeries requiring insufflation of the abdomen may lead to a pneumothorax

Ask about muscle weakness – Some EDS patients exhibit muscle weakness which can complicate spontaneous breathing on emergence.

Skin fragility – Avoid any unnecessary sheering forces and use of medical tape when the patient exhibits skin fragility. Occular traction during positioning or surgery (MD leaning on the face) can cause retinal detachment/blindness.

Regional/Neuraxial anesthetic considerations – local anesthetics have shown, in some cases to be ineffective. Ask the patient about past experiences (dental or otherwise) where the block was ineffective. Patients with EDS may also have scoliosis or other structural abnormalities that can complicate neuraxial anesthesia.

Caution for neuraxial anesthesia – Recommendations are to avoid spinal and epidural anesthesia in patients with vascular EDS due to high risk of bleeding.

Control PONV – Reports have shown that patients with vascular EDS can rupture their esophagus as a result of PONV.

This condition is an umbrella term for a number of disorders of connective tissue. Patients with this disorder have skin hyperextensibility, joint hypermobility, and/or vascular and soft tissue fragility. Clinical manifestations range from mild to life-threatening such as arterial aneurisms and dissections.

Most EDS subtypes are transmitted as autosomal-dominant or recessive traits. Incidence is 1:10,000 to 1:25,000 with no ethnic predisposition.

The most common organs susceptible to spontaneous rupture include the gastro-intestinal tract and gravid uterus, and, less commonly, lungs, spleen and liver.