Rett Syndrome: a challenge to an anaesthetist

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 1.These patients may show a characteristic abnormal respiratory pattern consisting of episodes of tachypnea and irregular respiration, intermixed with apnoea, observed only during wakefulness. 2 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.