Anaesthetic Management of Patient with Xeroderma Pigmentosum Posted for Basal Cell Carcinoma Excision

Abstract

Introduction: Xeroderma Pigmentosum (XP) is a hereditary autosomal recessive disorder due to defect in nucleotide repair genes. In this condition patient might require multiple surgeries for removal of skin and ocular lesion. Case report: A 17 year old male patient, weighing 43 kg presented with multiple lesions over face since 2 months. He had cutaneous lesions over full body with no major neurological deficit. General anaesthesia was planned. Patient’s body protection from artificial light and eye protection given in operating room. Patient Pre medicated with Inj glycopyrrolate, Inj Midazolam and Inj fentanyl. Preoxygenation done with 100% oxygen for 3 min. Induced with Inj Propofol. After elimination of reflexes and reaching enough depth of anaesthesia laryngeal mask airway(LMA) number 3 inserted and good lung ventilation confirmed by chest rise and ETCO2 then LMA fixed in place. Anaesthesia maintained with 50% nitrous oxide in oxygen, IV Propofol with intermittent positive pressure ventilation. Further analgesia supplemented by inj.Bupivacaine 0.25% infiltration locally around wound given. The procedure lasted for 40 min. Conclusion: Xeroderma pigmentosum patients have risk of worsening neurological disorder with genotoxic drugs and volatile anaesthesia and prolongation of recovery from muscle relaxants. In this case report demonstrated that patient with XP can safely be managed under general anaesthesia with IV propofol, without muscle relaxant and inhalational volatile anaesthetics.