Mitral and aortic valve replacement in a patient with ectodermal anhydrotic dysplasia. A case report.

Ectodermal anhydrotic dysplasia is a rare, usually X-linked recessive malformation of ectodermal tissues and organs. The case of a 17-year-old boy with ectodermal anhydrotic dysplasia and concomitant combined mitral valve disease and aortic insufficiency is described. The surgical risk was considered to be high due to severe atrophia of the laryngo-pharyngeal mucosa complicating endotracheal intubation and the possibility of postoperative temperature control disturbances. Double valve replacement was performed late in the autumn, after laryngo-pharyngeal pharmacological treatment preparing for endotracheal intubation. The peri-operative course was managed without any complication related to the hereditary malformation. Our experience suggests that patients suffering from ectodermal anhydrotic dysplasia may undergo open heart surgery after appropriate preparation. Potential postoperative problems related to the hypohydrosis and impaired spontaneous temperature control could be prevented in our patient.