Anesthetic management in Kostmann syndrome [Kostmann Sendromlu Olguda Anestezik Yaklaşim]

Kostmann is an autosomal ressesive inherited syndrome characterized by neutropenia. the patients received this diagnosis have frequent infection and growth retardation. neutropenic patients,in order to immuno modulation receive appropiate treatment. anesthetic medications can have affects on immune system. we need to consider anesthetic drugs and anesthetic management in immuno compromised patients. we should avoid the use of immuno supressive drugs in these patients. we believe there is a need to emphasize the importance of preoperative evaluation and peroperative anesthetic management for these patients. in this case,an 3.5-year-old baby with kostmann syndrome who was operated with a diagnosis of spinal cysts is presented. total intravenous anesthesia was administered during the operation due to the necessity of motor evoked potantial monitoring. therefore,in our case we aim to emphasize the approach to the patients with congenital neutropenia who are going to be applied total intravenous anesthesia.