Anesthetic management in a pediatric patient with infantile phase Prader-Willi Syndrome: A case report / 대한마취과학회지

ABSTRACT

Prader-Willi syndrome is a genetic disorder characterized by infantile hypotonia, childhood obesity, characteristic facial appearance, mental retardation, hypogonadism and short stature. It is described as a 2-stage disorder with an infantile hypotonic phase, followed by a childhood obese phase. The first phase, during the newborn and infancy period, is characterized by marked hypotonia, poor sucking, swallowing, coughing, crying, and episodes of asphyxia. Since these signs of poor strength cause poor reversal in the postoperative period, muscle relaxants should be used cautiously, especially in patients in the first phase of the syndrome. We experienced the anesthetic management of a 5-month-old female patient with Prader-Willi syndrome under general anesthesia, without muscle relaxant for excision of BCG lymphadenitis and a preauricular mass.