Anaesthesia for a child with adrenoleukodystrophy: A case report and review of the literature
Our patient was a 9-year-old Indian male with X-linked cerebral ALD who required general anaesthesia for a permanent catheter insertion via left internal jugular vein for plasmapheresis. The patient was diagnosed in June 2011 at 6 years of age when he presented with partial seizures. He underwent an unrelated donor umbilical cord blood transplant (minor B->O incompatibility) and maintained stable donor chimerism. Post-operative recovery was uneventful and he led a relatively normal seizure free life.\nHe presented on August 2012 with blood-stained urine and chest pain. Investigations determined that he was suffering from severe autoimmune haemolytic anaemia, liver dysfunction and steroid related side effects.\nPhysical examination revealed a 46 kg male with pallor, yellow sclera and cushingoid facies. He was confined to the wheelchair and demonstrated hypotonia. The child showed limited interaction with his environment. Pre-operative medications included levitiracetam, fludrocortisone and famotidine. His pre-operative investigations showed a haemoglobin concentration of 3 g/dL, which eventually improved to 6.9 g/dL after several cycles of plasmapheresis and blood transfusion, and a raised total bilirubin of 97 μmol/L.\nStandard monitoring was attached, and intravenous hydrocortisone 100 mg was administered. The patient was tilted 30 degrees head-up, pre-oxygenated and cricoid pressure was applied. However, ventilation could not be achieved, and decision was made to release cricoid pressure. Anaesthesia was induced with a target propofol concentration of 4 μg/mL and remifentanil infusion rate of 4 ng/mL. Muscle relaxation was provided with rocuronium 0.7 mg/kg. The trachea was intubated orally with a size 6 cuffed tracheal tube. Maintenance of anaesthesia was achieved with a target propofol concentration of 2.5 μg/mL and remifentanil infusion rate of 3 ng/mL. Post-operative analgesia was obtained with 10 mL of lignocaine 1% infiltrated over the surgical site.\nFollowing the procedure that lasted 105 minutes, residual neuromuscular blockade was reversed with neostigmine 2.5 mg and glycopyrrolate 0.4 mg. Neuromuscular function was assessed with electromyography. After the patient demonstrated airway protective reflexes, spontaneous eye opening and a regular respiratory pattern, the trachea was extubated. Recovery was uneventful, and he was subsequently discharged to the general ward.