title: Anesthetic management of a patient with congenital methemoglobinemia.
abstract: Methemoglobinemia occasionally causes cyanosis particularly in congenital methemoglobinemia. Avoidance of exposure to oxidizing agents is important for patients with congenital methemoglobinemia because of their deficient enzymatic pathways and decreased oxygen-carrying capacity. Here, we present a patient with preoperatively undiagnosed congenital methemoglobinemia who underwent uterine myomectomy under general anesthesia. The patient was a 35-year-old woman who displayed a low pulse oximetry reading of 91% prior to induction of anesthesia. Methemoglobinemia was first suspected intraoperatively because of a mismatch of SpO2 of finger pulse oximetry and SaO2 of arterial blood, and was later confirmed by multiple-wavelength CO-oximetry. The pathophysiology, etiology, clinical manifestations, anesthetic considerations, and treatment options of methemoglobinemia are discussed.
authors.affiliation: Department of Anesthesiology, Mackay Memorial Hospital, Taipei, Taiwan, R.O.C.
authors.forename: Chiao-Yi
authors.initials: CY
authors.lastname: Lin
authors.forename: Jenn-Ming
authors.initials: JM
authors.lastname: Yang
authors.forename: Chiun-Ting
authors.initials: CT
authors.lastname: Chen
authors.forename: Yung-Wei
authors.initials: YW
authors.lastname: Hsu
authors.forename: Chun-Jen
authors.initials: CJ
authors.lastname: Huang
authors.forename: Chien-Chuan
authors.initials: CC
authors.lastname: Chen
authors.forename: Hsin-Jung
authors.initials: HJ
authors.lastname: Tsai
mesh_terms: D000328:Adult; D000758:Anesthesia / Q000379:methods*; D005260:Female; D006801:Humans; D008708:Methemoglobinemia / Q000151:congenital* / Q000628:therapy; D008751:Methylene Blue / Q000627:therapeutic use; D010100:Oxygen / Q000097:blood
chemical_list: D010100:Oxygen; D008751:Methylene Blue