[Anesthetic management of a patient with hypohidrotic ectodermal dysplasia]

We anesthetized a 10-year-old girl with hypohidrotic ectodermal dysplasia for an ophthalmic surgery. Ectodermal dysplasia involves the abnormalities of ectodermal tissues and has a triad; hypohidrosis, a lack of teeth, and the scarcity of hair. Hyperthermia may occur due to the defect of sweat glands. Therefore, the body temperature must be monitored continuously. Respiratory tract infection occurs frequently due to the absence of seromucosal glands. We recommend humidifying the inspired gases during the operation. Tracheal intubation may be difficult because of maxillary and/or mandibular abnormalities. We conclude that the particular care should be taken such as the management of the body temperature, preparation for the difficult airway and the humidification of respiratory tract.