ABSTRACT

West syndrome is a rare syndrome that consists of a triad of infantile spasms, hypsarrhythmia pattern on electroencephalogram and mental retardation. Tuberous sclerosis complex (TSC) is one of the disorders that can cause it. Radiology suites are considered as remote locations for anaesthesiologists, and the delivery of anaesthesia becomes challenging if a patient with such a rare disease having multiple anaesthetic implications arrives. We present anaesthetic management for the radiological procedure of the MRI brain of a year old paediatric patient with the West syndrome having suspected TSC based on presenting signs and symptoms. Anaesthetic consideration and management of this rare syndrome are discussed. Detailed preoperative assessment, pre-emptive preparation for possible difficult intubation and difficult intravenous access, careful positioning and prevention of seizures should be the goal. Thorough knowledge of the disease process, its manifestation and its management is the key to the successful management of such cases.

Keywords: Anaesthesia, child, infantile, isoflurane, sevoflurane, spasms, tuberous sclerosis