Pre and post-operative otorhinolaryngology surgery care in patients with glycogen storage disease type 1

Abstract

Objective: To discuss aspects of pre and post-operative otorhinolaryngology surgery in patients with glycogen storage disease type 1b. Case description: Description of three clinical cases with probable glycogen storage disease type 1b who underwent otorhinolaryngology surgery, showing the importance of multidisciplinary interaction to avoid episodes of hypoglycemia. Comments:Patientswithglycogenstoragediseasetype1bpresent recurrent infections, including the otorhinolaryngology affections. Whenthereisanindicationforsurgicaltreatment,thecaloricintake shouldbecarefullyfollowedinordertopreventhypoglycemia.Theway to ensure this is to perform the pre and postoperative period in the hospitalward.Inthepostoperativeperiod,itisimportanttomakeaslow transitionbetweentheintravenousandoralroutesandnotsuspendthe infusionofglucoseduringthesurgicalprocedure.Thecasesillustrate the need for the interaction of the otorhinolaryngologic surgeon withtheanesthesiologist,thepediatricianandthegastro-pediatrician inthemanagementofthesepatients,avoidinghypoglycemicepisodes.