title: Inhalation anesthesia in a patient with xeroderma pigmentosum: a case report.
abstract: BACKGROUND
  Xeroderma Pigmentosum (XP) is a rare autosomal recessive disease, which is defined by extreme sensitivity to sunlight and UV radiation and characterized by skin lesions and neuromuscular abnormalities. It is caused by a molecular defect in nucleotide excision repair genes. It has been reported that volatile anesthetics may cause genotoxic side effects or aggravation of the neurological signs. We report an XP patient with difficult intubation whose airway was controlled with Laryngeal Mask Airway (LMA) and was anesthetized with sevoflurane.
  
  
  METHODS
  A 23-year-old woman, who had been a known case of XP since her childhood, was admitted to our hospital for excision of face mass (SCC) and skin graft surgery. Her airway examination revealed some anatomical and pathological abnormalities, including limitation of mouth opening, jaw protrusion, head extension, and class 4 of mallampati, all predicting difficult intubation. We chose general anesthesia with inhalation induction, LMA insertion and maintenance with sevoflurane without muscle relaxant. The surgery was completed uneventfully and the patient left the hospital the day after the surgery without any new complaint.
  
  
  CONCLUSIONS
  We suggest that for XP patients with compromised air-way, sevoflurane (not all volatiles) may be preferred.
authors.affiliation: Department of Anesthesiology, Beheshti Hospital, Kashan University of Medical Sciences, Kashan, Iran.
authors.forename: Mohammad
authors.initials: M
authors.lastname: Hajijafari
authors.affiliation: School of Public Health, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Mohammad Hossein
authors.initials: MH
authors.lastname: Ziloochi
authors.affiliation: Department of Anesthesiology, Beheshti Hospital, Kashan University of Medical Sciences, Kashan, Iran.
authors.forename: Mohammad Reza
authors.initials: MR
authors.lastname: Fazel
keywords: General Anesthesia; Sevoflurane; Xeroderma Pigmentosum