P043: ANESTHETIC MANAGEMENT OF A 13 YEAR OLD MALE WITH EPIDERMOLYSIS BULLOSA UNDERGOING GENERAL ANESTHESIA
Ethan T Lubanski, MD; Christopher Emerson, MD; Langely Chaparro, MD; Luis Rodriquez, MD; Jackson Memorial Hospital
Introduction/Background: Epidermolysis bullosa (EB) is a group of rare genetic conditions affecting the function of keratin, collagen, and laminin proteins, resulting in weakness, fragility, and dehiscence at the dermal layer of skin and mucosal surfaces which as a result requires frequent operations requiring a general anesthetic. Patients with EB are characterized for presenting numerous complications perioperatively. Such obstacles and their resolutions are exhibited in the following case involving a 13 year old male presenting for esophageal dilation and gastric tube insertion.
Methods: Applying monitors proved difficult given the contraindication for using adhesives or any other material that may cause any skin sheering. For monitoring, the following steps were taken; For pulse oximetry, a non-adherent clip-on device was used on the left ear. For cardiac monitoring, a three-lead pediatric electrocardiogram set was used with the adhesive portion completely removed beforehand with alcohol swabs. A small amount of lubricant gel was needed under each lead after having been swabbed away to remove the adhesive portion. These leads were secured under previously present dressings that the patient arrived with. For blood pressure monitoring, a standard appropriately sized non-invasive cuff was used around previously present dressings.
For intravenous access, a 22 gauge catheter was inserted in the left forearm easily and was secured with silicone-based tape prior to beginning IV induction.
For airway management, the following steps were taken; The inflatable portion of the mask was lubricated prior to any contact to skin. Mask ventilation was easy but was minimized. Intubation was made difficult by a limited mouth opening however a grade 2a view of the glottis was achieved with a Pedi Glidescope. A 5.5mm ETT was placed and secured with a surgical facemask with the facemask portion placed over the occiput and the anteriorly facing ties used to secure the ETT. Throughout management of the airway, the clinician had to use meticulous caution in avoiding any sort of traction or manipulation of the patient.
Results: The procedure was without major complication and the patient was extubated awake successfully. However, at one point during the case there was a concern of inadequate paralysis by surgery due to noticing a rigid abdomen upon insertion of the gastric tube. This clinical finding persisted despite multiple redosing of paralytic. Whether or not this was related to a sequela of EB was unclear.
Discussion/Conclusion: Surgical interventions for patients with EB are often children and specific anatomic, physiologic, and pharmacologic considerations of the pediatric population apply. A meticulous review of the record should detail prior anesthesia records and the clinical subtype of EB along with the severity and extracutaneous manifestations. Careful assessment of the patient should seek to identify oral contractures, mucosal bullae, and poor dentition that might predict difficult airway. In addition, special precautions to avoid the formation of new blisters and worsening of pre-existing skin lesions are paramount when utilizing standard ASA monitors in the intraoperative period.