Pediatric Lamellar Ichthyosis

Three- and four-year-old brothers with severe lamellar ichthyosis (LI) underwent myringotomy and tympanostomy tube insertion at an ambulatory surgery center. Both had diffuse, thick, brown lamellar scales on the face, trunk, and extremities. The surgeon anticipated a prolonged procedure for debridement of thickened skin within the external auditory canal secondary to LI. Consequently, intravenous access and laryngeal mask airway (LMA) were employed to ensure adequate anesthetic management. Securing the LMA and IV catheter was challenging because emollient-covered, scaly skin prevented standard adhesives, such as plastic tape, from adhering. Therefore, the LMA was secured with a self-adhering wrap around the neck, and moistened gauze was placed over both eyes for corneal protection and secured with a self-adhering wrap (Figure 1). Intravenous access was obtained and secured with a self-adhering wrap (Figure 2). The use of self-adhering wrap minimized the risk of skin injury and helped prevent a devastating loss of airway or IV access intraoperatively. LI is commonly caused by mutations in the TGM1 gene, leading to weakened structural integrity of the stratum corneum [1]. In addition to skin manifestations, LI may present with a difficult airway from restricted neck mobility and mouth opening due to tight skin, increased ocular injury risk from ectropion, and increased susceptibility to hypo- or hyperthermia from impaired thermoregulation [2]. LI presents with hyperkeratotic and fragile skin that is susceptible to injury from minor trauma [2]. Removing adhesives can damage skin, increasing infection risk. The utility of self-adhering elastic wraps, such as Coban wrap, extends beyond LI to other dermatologic conditions, including epidermolysis bullosa and acute burns, in which shearing forces of adhesives pose a similar risk [3]. The use of self-adhering wraps, protective padding, and avoidance of adhesives can help mitigate complications in patients with Lamellar Ichthyosis and other dermatologic disorders. The data that support the findings of this study are available from the corresponding author upon reasonable request.