BACKGROUND AND OBJECTIVES: Mucopolysaccharidosis are a group of heterogenous disorders secondary to the deficiency of a lysosomal enzyme, with accumulation of acid mucopolysaccharides in the tissues. It carries a 20% perioperative mortality rate due mainly to difficulties on handling the airways. The objective of this report was to present the anesthetic approach of a patient with mucopolysaccharidosis who presented in the operating room for emergency surgery. CASE REPORT: A 15 year-old male patient with a diagnosis of mucopolysaccharidosis was admitted to the operating room for treatment of an incarcerated umbilical hernia. After monitoring, a subarachnoid block with hyperbaric bupivacaine was performed with the patient in the sitting position. After identifying failure of the spinal block it was changed to general anesthesia. Anesthetic induction consisted of intravenous clonidine, fentanyl and propofol, followed by endotracheal intubation without intercurrences. After a 45-minute surgery, the patient was extubated and transferred to the postanesthetic care unit where he remained under observation for 12 hours, being discharged to the regular ward without intercurrences. CONCLUSIONS: In the case presented here we did not have any difficulties handling the airways. However, this is the main problem in this group of patients and it is up to the anesthesiologist to choose the better technique to guarantee adequate handling of the airways.
ANESTHESIA, General; COMPLICATIONS; DISEASES
