Perioperative management of a patient with PEHO - Syndrome : Delayed recovery of neuromuscular blockade after rocuronium

The PEHO syndrome (progressive encephalopathy with brain, facial and peripheral edema, hypsarrhythmia and optic atrophy) is a very rare autosomal recessive disorder with progressive encephalopathy. For diagnosis and therapy, many patients may require deep sedation or even general anesthesia. There are no previous reports on the perioperative management in PEHO-patients. Distinct obstacles have to be obeyed. Lack of pharyngeal control might bear an imminent risk of aspiration. Muscular hypotonia and an immobile state rules out the use of succinylcholine. In the case reported here, a 10-month old boy underwent laparoscopic assisted percutaneous jejunostomy. The anesthetic management consisted of total intravenous anesthesia with propofol and remifentanil, preceded by a rapid sequence induction with 0.6 mg/kg rocuronium. In our patient, recovery from neuromuscular blockade was grossly delayed. Therefore, with all applications of neuromuscular blocking agents in patients with PEHO-syndrome, continuous neuromuscular monitoring is indispensable even on the intensive care unit to determine the end of the neuromuscular block and to exclude residual curarisation. Anestesia Pediatrica e Neonatale, Vol. 8, N. 1, Febbraio-Marzo 2010