Journal of Pediatric Surgery Case Reports (Jan 2021)

Perioperative management of a patient with Xeroderma Pigmentosum: A case report

  • Leah M. Webb,
  • Debnath Chatterjee,
  • Megan A. Brockel

Journal volume & issue
Vol. 64
p. 101710

Abstract

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Perioperative care of patients with Xeroderm Pigmentosum (XP), a rare disorder of DNA repair [1,2], presents many anesthetic challenges. Firstly, harmful ultraviolet radiation can be detected in perioperative areas. Also, anesthetic type must be carefully considered, as exposure to volatile anesthetics may hasten the development of neurodegeneration (Masuda et al., 2002; Reitz and Lanz, 1993; Miyazaki et al., 2007; Fjouji et al., 2013) [3-6]. Furthermore, muscle relaxants can produce a prolonged effect (Soen et al., 2006) [7]. Airway management may be difficult secondary to skin changes and growths (Zarrouki et al., 2017; Hajijafari et al., 2014; Meyer, 1982) [8-10]. Even the risk of using imaging modalities should be considered. Given the above exposure risks, we present considerations for preparing to care for patients with XP in the perioperative period and information on where to purchase supplies to prepare the perioperative area.

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