Purpose: To report the beneficial effect of prophylactic methylene blue administration before induction of anesthesia in a patient with congenital methemoglobinemia.
Clinical features: A 26-yr-old male patient known to have congenital methemoglobinemia was scheduled for turbinectomy under general anesthesia. The patient was clinically cyanotic with a pulse oximetry of 91%. Arterial blood gas analysis showed a partial pressure of oxygen (PaO(2)) of 81.3 mmHg associated with a fractional oxyhemoglobin of 80.7%, and a methemoglobin fraction of 0.159. Preoperative iv administration of 1 mg.kg(-1) of methylene blue resulted, within five minutes, in a decrease of methemoglobin fraction down to 0.05 associated with an increase of the fractional oxyhemoglobin saturation up to 94.7%. After two hours, the methemoglobin fraction decreased to 0.01 and the fractional oxyhemoglobin concentration increased to 97.7%. Induction of anesthesia as well as intraoperative and postoperative course were uneventful without any episode of hypoxemia. Postoperatively, the methemoglobin fractions remained low for 24 hr, to be followed by a gradual increase up to 0.02 on the second day to reach 0.094 on the fifth day.
Conclusion: The prophylactic preoperative methylene blue administration in a patient with congenital methemoglobinemia significantly decreased the methemoglobin level and increased the fractional oxygen saturation with a consequent increase of the safety margin against perioperative hypoxemia.