Anaesthetic management of a child with PEHO syndrome

Abstract

SummaryProgressive encephalopathy with oedema, hypsarrhythmia and optic atrophy (PEHO) syndrome is a rare neurodegenerative disorder of probable autosomal recessive inheritance. Specific challenges faced by the anaesthetist include difficult venous access, aspiration risk, and muscular hypotonia contraindicating the use of suxamethonium.A 3‐yr‐old boy with PEHO syndrome underwent tonsillectomy for the management of obstructive sleep apnoea. Our anaesthetic technique consisted of a gaseous induction followed by total intravenous anaesthesia, following which the patient was awoken and admitted electively to the intensive care unit for monitoring. We approached the case adopting techniques used when anaesthetising patients with more common neuromuscular diseases. Although we considered the option of using rocuronium followed by reversal with sugammadex, total avoidance of neuromuscular blocking drugs was felt to be the safest choice. The availability of intensive care in the postoperative period played an important role in our overall management.