Leanne Thalji, B.M., M.S.
Pediatric Anesthesia
Mayo Clinic, United States
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Title: Successful Pediatric Mask Ventilation in Epidermolysis Bullosa
Case Report Body:
Introduction: Junctional epidermolysis bullosa is a rare condition in which airway management is not well-described. Patients with this disease have blistering and sloughing of the skin and mucosa with shearing from mild contact. This population presents a challenge with mask ventilation, due to the pressure needed to obtain adequate oxygenation and ventilation. We present a case in which mask ventilation was successfully maintained in a septic pediatric patient using multiple skilled airway providers and a large amount petroleum jelly.
Description: A 2-year-old male with severe junctional epidermolysis bullosa presented with fever, tachycardia, and tachypnea. He had wide-spread skin breakdown, primarily effecting his face. Given his septic presentation, parenteral antibiotic requirement, and impossibility of securing durable peripheral intravascular access, he was urgently listed for central line placement under anesthesia. He had a hoarse cry, likely due to disease involvement of the airway. Given concern for additional trauma caused by endotracheal intubation, the plan was to attempt to maintain spontaneous ventilation throughout the procedure. Unfortunately, the depth of anesthesia required to immobilize the child ultimately resulted in apnea and airway obstruction requiring mask ventilation. Four skilled airway providers were available in the procedure suite. Everything contacting the patient, including the mask and providers’ gloved hands, were coated in petroleum jelly, which minimized friction on the patient’s skin while permitting an adequate seal. Providers were intermittently rotated to rest and re-lubricate their gloves. An additional provider remained in non-lubricated gloves to operate the anesthesia machine and administer medications. The patient tolerated the procedure well and did not suffer additional trauma to his skin or airway as a result, despite requiring intermittent jaw thrusts.
Discussion: Patients with junctional epidermolysis bullosa requiring airway management present competing needs of avoiding friction with medical equipment and the immediate necessity to oxygenate and ventilate. It is possible to mask ventilate using a generous amount of petroleum jelly and multiple providers, which may offer a safer alternative to more invasive airway strategies in select circumstances.