• Masako Soen, Tetsuro Kagawa, Reiko Uokawa, and Takeshi Suzuki.
• Department of Anesthesiology, Hyogo Prefectural Kobe Children's Hospital, Kobe 654-0081.
• Masui. 2006 Feb 1;55(2):215-7.

AbstractXeroderma pigmentosum (XP) is a rare autosomal recessive disease, which is characterized by hypersensitivity of the skin to ultraviolet (UV)-radiation and progressive neurological complications. Patients with XP show a failure to properly repair UV-induced DNA lesions by the nucleotide excision repair (NER) mechanism. This results predominantly in a high frequency of UV-induced skin tumors at an early age. Therefore, patients with XP must avoid exposure to UV-radiation by use of protective clothing, sunscreen and UV-blocking film. We report a 15-year-old girl, who underwent surgical treatment twice for clubfoot under general anesthesia using propofol and fentanyl. We protected her skin from light with UV blocking film in the operating room. Both surgical procedures were performed uneventfully except for delayed awakening. A number of precautions must be taken in the perioperative management of XP patients, which include proper shielding from damaging light, avoidance of all drugs that harm DNA such as halothane and careful evaluation of neurological abnormalities. There is a possibility of difficult endotracheal intubation or prolonged effect of muscle relaxation due to skin atrophy and joint contracture.

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