Anesthetic management of percutaneous nephrolithotripsy in an 11 year old girl with Rett Syndrome: a case report

Background: Rett syndrome (RTT) is a rare X-linked neurodevelopmental disorder primarily affecting fe- males, characterized by severe cognitive impairment, au- tonomic dysregulation, epilepsy, and abnormal respira- tory patterns. Anesthetic management in these patients is particularly challenging due to increased sensitivity to medications, airway instability, and potential for postop- erative complications. We report the anesthetic manage- ment of an 11-year-old girl (15 kg) with Rett syndrome undergoing elective left-sided percutaneous nephrolitho- tripsy for staghorn calculus. Premedication was adminis- tered with oral midazolam (0.5 mg/kg) mixed in cherry juice. Stepwise inhalational induction with sevoflurane was performed, followed by successful orotracheal intu- bation without neuromuscular blockade. A single-shot caudal block with bupivacaine 0.5% (50 mg), dexame- thasone 4 mg, and normal saline was performed. No in- traoperative opioids or muscle relaxants were used. In- traoperative hemodynamics and postoperative recovery were stable. This case illustrates that sevoflurane-based anesthesia combined with caudal block, without the use of opioids or neuromuscular blockers, is safe and effec- tive for children with RTT undergoing urological sur- gery. Regional anesthesia techniques can enhance peri- operative stability in patients with complex neurodevel- opmental disorders.

Abstract licence: CC BY

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