Clinical Pearls in Anesthesia for Xeroderma Pigmentosum: A Case Report

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.

Difficult Intubation and General Anesthesia Management in a Pediatric Patient with Xeroderma Pigmentosum Requiring Emergency Laparotomy

“…It is an outstanding finding that short-acting muscle relaxants are used in reduced doses. The monitorization of neuromuscular blockade may be useful in avoiding prolonged drug effects (7). Some studies have reported using an agent to reverse the neuromuscular blockade without specifying the reversal agents (5).…”