Anesthetic and Surgical Considerations in a Patient with Epidermolysis Bullosa

A patient with epidermolysis bullosa (EB) often requires special care in the perioperative period in order to prevent unnecessary complications.

General recommendations for perioperative management:

(1) Schedule the patient as the first case of the day and for twice the usual duration.

(2) Ensure adequate anxiolytic therapy for the patient.

(3) Educate all persons involved in the patient's care about the unique problems encountered with these patients, especially skin fragility.

(4) Minimize abrasive forces (see below).

(5) Protect the eyes with saline-soaked gauze or with gel pads.

Problems Associated with Skin Fragility	Intervention to minimize abrasive forces
shearing forces while being moved	allow the patient to move self whenever appropriate; use a slide sheet and not a rigid plastic board to move the patient
complications of adhesive tape	avoid adhesives (tape, pads, etc) whenever possible, using an alternative
need for lubrication to minimize abrasion	liberally lubricate tubing, intubation blades and skin
intravenous access	identify potential sites well in advance
pressure	use a soft, pressure-relieving mattress; use padding under pressure cuffs

Evaluate and correect common complications of EB:

(1) anemia (transfuse if necessary)

(2) cardiomyopathy

(3) microstomia (evaluate for a difficult orotracheal intubation)

(4) loose and/or carious teeth (evaluate for loss during intubation)

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