Abstract
Prader-Willi syndrome (PWS) is a congenital developmental disorder which is related to severe problems during anaesthesia. Of particular interest are the morphological and patho-physiological alterations which may lead to considerable respiratory complications, including a higher risk of regurgitation and aspiration, and the tendency towards post-surgical hypoventilation. Further difficulties may arise from mental retardation, disturbed thermoregulation, and an unstable glucose metabolism.
In our patient these features have been aggravated by burn injuries exceeding 35% of body surface. Appropriate care of circulatory and respiratory function, as well as supervision of blood glucose level and body temperature during a period of several weeks with repeated operations was necessary to avoid further complications. Additional findings we discovered in our patient were narrow airways not corresponding to the patient's age and a significant resistance to non depolarising muscle relaxants that might have been caused by the burn injuries.
The importance of PWS for anaesthesiologists can be estimated by the circumstance, that it is considered to be nearly as frequent as trisomy-21. PWS in connection with extensive burns has yet not been reported. This case and a recent review of the relevant literature on anaesthetic management of PWS are extensively discussed.
Biro, Peter