Intro
A 15 month old normal term Caucasian female infant with a history of epidermolysis bullosa presented to the ED with a two day history of new onset hematuria in the setting of a recent URI. The patient was worked up and found to have acute glomerulonephritis with nephrotic range proteinuria. At the time, her etiology was uncertain(possible MPGN vs Post-Strep vs other), requiring a renal biopsy for further workup. Thus, the patient was scheduled for bilateral percutaneous renal biopsy under ultrasound for new onset hematuria. However, her history of epidermolysis bullosa, a rare genetic mechanobullous condition, characterized by excessive fragility of the skin and mucous membranes, resulting in cutaneous blistering and scarring that can lead to debilitating and even life threatening medical conditions; presents anesthetic challenges that require further consideration and unique tailoring of her anesthesia.
Case
The patient had no prior anesthesia and no family history of anesthesia complications. Besides her epidermolysis bullosa, all her other review of system were negative. Her physical exam was significant for molted skin with multiple erythematous patches and bullae throughout the body, in particular, multiple bullae to the right hip, abdomen, back, hands, and elbows and labia majora. Her airway exam was benign, with a mallampati 1 view and full range of motion. Our anesthetic consisted of trying to maintain spontaneous ventilation w/MAC using IV ketamine, dexmedetomidine, and opioids as needed. Given the risks of trauma secondary to the excessive fragility of the skin, special considerations were made to minimize damage. To minimize the risk of skin friction from agitation or uncontrolled movement during induction, 40mg of PO ketamine was given prior to bringing the patient back to the OR. An IV was subsequently obtained with ultrasound to further minimize IV attempts and trauma. Once IV was obtained, IV ketamine and dexmedetomidine was given for sedation while maintaining spontaneous ventilation. To avoid new blisters, Vaseline was applied to all gloves used for contact and the face mask, as well as backup laryngoscope and endotracheal tube. Furthermore, special padding was placed on the OR table to minimize compression and shearing forces.
A major challenge for this patient’s anesthetic management was the use of monitoring technology without damaging the epithelial surface. An ear clip pulse oximeter was placed for oxygenation. To minimize trauma from the BP cuff, we decided to hold off on a BP cuff and use physical exam of palpable pulses for hemodynamic monitoring. In addition, special EKGs were placed with lubrication to minimize sheering.
