Anaesthetic management of patients with Xeroderma pigmentosum. A series of three cases.
Xeroderma pigmentosum is a rare autosomal recessive disorder with clinical and cellular hypersensitivity to ultraviolet radiation and defective DNA repair. Skin cancer, mainly on the face, head or neck is very common. Inhalational anaesthetic agents and muscle relaxants are best avoided due to the possibility of inducing DNA damage and prolonged effects of muscle relaxants. These patients may have a difficult airway. These patients may have immature brain development which may render them sensitive to synergistic effect of benzodiazepines and opioids as seen in the first case. Total intravenous anaesthesia is preferred. Nonsteroidal anti inflammatory agents and opioids for multimodal analgesia may be beneficial.
Department of Anaesthesiology, Institute of Medicine, Tribhuvan University Teaching Hospital, Kathmandu, Nepal. gentlesunder@hotmail.com
G S
GS
Shrestha
R P
RP
Sah
A G
AG
Amatya
N
N
Shrestha
D000293:Adolescent; D000777:Anesthetics / Q000008:administration & dosage*; D002648:Child; D005153:Facial Neoplasms / Q000150:complications / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008297:Male; D012878:Skin Neoplasms / Q000150:complications / Q000473:pathology / Q000601:surgery*; D014983:Xeroderma Pigmentosum / Q000150:complications / Q000473:pathology / Q000601:surgery*
D000777:Anesthetics