Prader willi syndrome- A case report

Abstract

Prader willi syndrome (PWS) is a complex multisystem disorder. PWS mainly affects central nervous system and often involves the hypothalamus. Its major clinical features include neonatal hypotonia, developmental delay, short stature, behavior abnormalities, childhood onset obesity, hypothalamic hypogonadism and characteristic appearance. Anaesthetic management with general or regional anaesthesia for these patients is difficult mainly due to morbid obesity. The importance of PWS for anesthesiologist and primary physician is estimated by the circumstances as the prevalence of PWS is nearly as frequent as trisomy -21. We report the anaesthetic management of a 22 year old male patient with genetically proven PWS (small stature, obesity with characteristic behaviour problem) who presented with chronic fissure in ano. Though the clinical course of the patient was uneventful under saddle block. In this case report we discuss our strategy for the prevention of perioperative complications for PWS patients.