A CHALLENGING CASE: A patient with epidermolysis bullosa presents for multiple dental extractions

Dr. Schlichter discusses a case of epidermolysis bullosae

Presentation:  A patient with a history of epidermolysis bullosae presented for multiple teeth extractions.  The patient is covered with multiple sores in different stages of healing.  There is a history of full facial scarring from ventilation by mask at the age of 8.  The patient is a MP III secondary to limited mouth opening from jaw contractures.

Anesthetic challenge:  Epidermolysis Bullosa consists of a collection of rare mucocutaneous disorders that lead to the formation of skin and mucosal blisters following shearing or pressure to an area.  Direct pressure or friction should be avoided, as should all adhesives.  In a case review of 44 patients over ten years receiving general anesthesia, different airways were used with success including nasal and oral intubations, LMA, and in one case, tracheostomy (1).

How it was done:  An IV was placed in the left antecubital vein and secured with kerlex gauze wrapping thus avoid adhesive tape or adhesive tegaderm.  EKG leads were placed (to be taken off at home during a warm bath), a NIBP cuff was placed with kling wrap between the cuff and the skin, and a pulse oximeter was placed on the left finger.  The patient was given high oxygen via flow - by during induction with IV propofol.  A #3 LMA was placed.  The patient was then given vecuronium and intubation was accomplished with a fiberoptic assistance and an Aintree tube changer via the LMA.  A 6.0 mm oral Rae ET tube was secured with umbilical tape.  The patient was given dexamethasone 10 mg, ondandsetron 4 mg and morphine 5 mg.  The patient had an otherwise uneventful anesthetic and was transported to the APU after a deep extubation on high flow-by oxygen.  The patient was discharged to home 90 minutes after emergence from anesthesia.

For further reading: 1) Griffin RP, Mayou BJ:  The anaesthetic management of patients with dystrophic epidermolysis bullosa.  A review of 44 patients over 10 year period.  Anaesthesia  1993;48: 810-815; 2) Herod J et al:  Epidermolysis bullosa in children: pathophysiology, anaesthesia and pain management.  Pediatric Anaesthesia 2002;12:388-97.

Dr. Schlichter is Assistant Professor of Anesthesiology and Critical Care

Comments

[Andrew Piewola, MD]

There was a "Medically Challenging Case" of this particular scenario at the ASA. Very similar concerns. The patient actually approached the anesthesiology team, requesting not to have an LMA or Intubation, instead choosing to undergo bilateral mastectomy with a thoracic epidural and under Monitored Anesthesia Care (a few miligrams of IV Midazolam).