[Anesthetic management of a patient with May-Hegglin anomaly]

The May-Hegglin anomaly is a rare hereditary disorder characterized by the presence of giant platelets and platelet deficiency. This report presents a successful management of a patient with May-Hegglin anomaly. A 5-year-old girl with May-Hegglin anomaly was scheduled for closure of fistulae of her ears. Although preoperative physical examination revealed platelet count of 8,000 mm-3, she had no bleeding tendency. No premedication was given. Anesthesia was induced with inhalational agents of a mixture of sevoflurane, nitrous oxide and oxygen. Neuromuscular blockade was achieved with vecuronium 0.1 mg.kg-1 and the trachea was gently intubated. Anesthesia was maintained with sevoflurane, nitrous oxide and oxygen. Five units of platelet were transfused during the operation. No bleeding tendency was observed perioperatively. It is important to plan the management of anesthesia for a patient with bleeding tendency.