RESEARCH ARTICLE


Clinical Pearls in Anesthesia for Xeroderma Pigmentosum: A Case Report



S.B. Shah*, U. Hariharan, B.K. Naithani, A.K. Bhargava
Rajiv Gandhi Cancer Institute and Research Centre; Sec-5; Rohini, Delhi-110085, India


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Creative Commons License
© 2015 Shah et al.; Licensee Bentham Open

open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: https://creativecommons.org/licenses/by/4.0/legalcode. This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

* Address correspondence to this author at the Rajiv Gandhi Cancer Institute and Research Centre; sec-5; Rohini, Delhi-110085, India; Tel: 9891769779; E-mail: drshagun_2010@rediffmail.com


Abstract

Xeroderma Pigmentosum (XP) is a rare autosomal recessive (AR) disease characterized by hypersensitivity of the skin to ultra violet (UV) radiation, resulting in a high frequency of UV induced skin tumors and progressive neurological complications at an early age. Through the following case report we emphasize that perioperative management of xeroderma patients entails meticulous evaluation for neurological abnormalities, shielding the skin from OT (operation theatre) lights by using protective clothing, sunscreen and UV blocking film as well as avoidance of genotoxic drugs like volatile anaesthetics and paracetamol. One must be prepared for the possibility of difficult mask ventilation (we used a mask one size larger), difficult intubation and prolonged effect of muscle relaxants (as in our case) due to skin atrophy, neoplasia, joint contracture and neuronal dysfunction.

Keywords: Basal cell carcinoma, genotoxic drugs, muscle relaxants, ultraviolet light, volatile anaesthetics, xeroderma pigmentosum.