Anesthetic management of a morbidly obese patient with Prader Willi Syndrome [Prader Willi Sendromlu morbid obez bir olguda anestezi uygulamasi]

Introduction: prader willi syndrome is a rare,biphasic genetic disorder. the clinical course includes hypotonic infantile phase followed by hyperphagia and obese phase. life expectancy reach the fifth decade if morbid obesity is avoided. case report: a 13-year-old morbidly obese (body mass index of 74.4 kg m-2) boy with prader willi syndrome and severe restrictive pulmonary disease underwent a laparoscopic sleeve gastrectomy. intraoperative mechanical ventilation was a challenge. abrupt impairment in oxygenation and ventilation occurred with hemodynamic instability immediately after pneumoperitoneum. herein,we planned to discuss the possible causes,describe our anesthetic management,including alveolar recruitment maneuvers and positive end-expiratory pressure with adjustments in ventilator settings in different parts of the surgical procedure. the patient was succesfully extubated with no need for intensive care unit. conclusion: prader willi syndrome patients with hyperphagia and obese phase are prone to respiratory problems and subsequent death due to morbid obesity. in these patients,it is essential to consider the unique features of disease and surgical procedure for an uneventful perioperative period.