A Case of Acatalasemia Encountered during Anesthesia

A 71-year-old male was found to have latent acatalasemia when he rapidly developed methemoglobinemia during total laryngectomy. Methemoglobinemia was induced by a 300ml 1.5% hydrogen peroxide solution used to sterilize the oral cavity. Lack of catalase activity in the oral mucous membrane allowed the hydrogen peroxide to oxidize normal hemoglobin to methemoglobin. Initial clinical signs consisted of reduced oxygen saturation (SpO2=80%) with high oxygen tension (PaO2=235mmHg), a darkened blood color near the site of surgery and elevated methemoglobin (11%). His methemoglobinemia was treated with 2, 000mg intravenous ascorbic acid. Following surgery the patient experienced ulceration and edema of the oral mucoua, hemolytic anemia (Hb 7.7g/dl) and liver dysfunction (total bilirubin 5.6mg/dl). Analysis of blood enzymes revealed abnormally low red cell catalase activity. Examination of blood specimens from family members (mother and three children positive) confirmed inherited, asymptomatic acatalasemia.