DELAYED AWAKENING FROM GENERAL-ANESTHESIA IN A PATIENT WITH HUNTER SYNDROME

Hunter syndrome is one of a heterogeneous group of recessively inherit ed mucopolysaccharide storage diseases (MPS) with similar biochemical defects manifested by impairments in mucopolysaccharide catabolism wit h variable but progressive clinical courses. Abnormal accumulation and deposition of mucopolysaccharides in the tissues of several organs le ad to numerous anatomical, musculoskeletal and neurological abnormalit ies which are known to complicate anaesthetic and airway management. H unter syndrome has a wide variance of clinical phenotypes ranging from mild to severe. We present a patient having physical and neurological features consistent with a severe clinical presentation of Hunter syn drome (MPS, Type II). Following a seemingly uneventful intraoperative anaesthetic course including isoflurane, nitrous oxide and fentanyl (0 .93 mu g.kg(-1)) resumption of spontaneous ventilation and return to c onsciousness were delayed until intravenous naloxone (200 mu g) was ad ministered 110 min after the opioid administration. The cause of delay ed recovery from anaesthesia in this patient is unknown.