Metahemoglobinemia Congénita. Reporte de un caso

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 pediatric patient with preoperatively undiagnosed congenital methemoglobinemia who underwent catheterization with probably diagnosis of pulmonary hypertension under general anesthesia. The pediatric patient was a 10-year-old who displayed a low pulse oximetry reading of 92% prior to induction of anesthesia. Methemoglobinemia was first suspected intraoperatively because of a mismatch of SpO 2 of finger pulse oximetry and SaO 2 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. Key words: methemoglobinemia, cyanosis, anesthesia, pulse oximetry, pediatric patient.