Type – Implications depend on the type. The riskiest EDS subtype is vascular EDS, with a high risk of vascular and visceral tissue fragility. Make sure to communicate with the patient/family to discover the primary considerations unique to the patient.

PONV – Adequate prophylaxis of postoperative nausea and vomiting is recommended as spontaneous esophageal rupture has been reported as a result of vomiting in vascular-type EDS.

Tissue Fragility – During surgery, gentle patient positioning (avoiding lithotomy position where possible), and intubation techniques are crucial due to tissue fragility and the risk of complications like joint dislocation, cervical spine instability, bleeding, and hematoma formation.

Induction/Intubation – Video laryngoscopy (to avoid unnecessary head/neck movement), and careful temporomandibular joint and cervical spine management are recommended.

Approaches – Volatile and intravenous anesthesia are considered safe. Epidural or spinal anesthesia may be risky due to tissue fragility. Muscle hypoplasia, hypotonia, and myalgia may occur, so close monitoring of neuromuscular blockade is strongly recommended.

Postoperative Considerations – Monitor for surgical emphysema. Early mobilization is crucial to prevent deconditioning.

Ehlers-Danlos syndrome (EDS) is a rare group of inherited connective tissue disorders characterized by skin hyperelasticity, joint hypermobility, and easy bruising. It affects a relatively small portion of the population (1 in 10,000 to 1 in 25,000) and has various subtypes.

There are six subtypes: classic, hypermobility (EDS-HT), vascular, kyphoscoliosis, arthrochalasis, and dermatosparaxis. About 50% of EDS patients have the classic type.