Rett Syndrome: A challenge to an anaesthetist

Abstract

Rett syndrome is a progressive neurological disorder of females characterized by dementia, severe autism, movement disorders, growth retardation, microcephaly, seizures, muscle wasting, scoliosis and an abnormality of respiratory control.These patients may show a characteristic abnormal respiratory pattern consisting of episodes of tachypnea and irregular respiration, intermixed with apnoea, observed only during wakefulness. Anaesthesia is challenging in this rare subgroup of patients with its multiple comorbid presentations. This report provides a practical approach comprising of vigilant monitoring and proactive actions like preoperative oxygenation, anti-epileptic medications, adequate fluid infusion and unilateral spinal anaesthesia restricting level to T 10 to avoid respiratory distress for anaesthesia in a patient with Rett syndrome for sub trochanteric fracture nailing.