Anesthetic management of a patient with epidermolysis bullosa dystrophica

Abstract

A 27-year-old female patient with epidermolysis bullosa dystrophica required a forearm amputation and the dissection of axillary and subclavicular lymph nodes for a squamous cell carcinoma which had developed on her hand. Because any friction on her skin or mucous membrane result in blisters, erosions and scars, mechanical contact with the patient had to be reduced to a minimum. Laryngoscopy and endotracheal intubation might cause bulla formation or bleeding on her lips, tongue, pharynx and epiglottis. She was anesthetized with supraclavicular brachial plexus block for the amputation and neuroleptoanalgesia without N2O for the dissection of nodes. Oxygen was administered via a face mask, which was held over her face but not in contact with it. Every effort was made to avoid trauma to her skin. Soft sponge pads were used over pressure points. No adhesive tape was allowed to contact the skin. ECG electrodes and a small IV catheter were fixed in place with a loose bandage. A soft roll padding was used to wrap around her arm beneath the blood pressure cuff. Satisfactory analgesia was obtained and she was sedated and cooperative during the whole procedure which took 5 hrs 40 min. No serious perioperative complications developed.