Not All That Desaturates is Disease: A Rare Encounter with Congenital Methemoglobinemia in Pregnancy

Document Type

Article

Publication Title

Journal of South Asian Federation of Obstetrics and Gynaecology

Abstract

Methemoglobinemia is an uncommon condition in which the iron in hemoglobin is oxidized from its normal ferrous (Fe²+) form to the ferric (Fe³+) form, resulting in the production of methemoglobin that impairs the transport of oxygen throughout the body. While the acquired form is more common, congenital methemoglobinemia—an autosomal recessive condition due to cytochrome b₅ reductase deficiency—is rarely diagnosed and often asymptomatic. Pregnancy may unmask this condition due to physiological stress. Clinical suspicion arises when cyanosis and low oxygen saturation persist despite normal cardiopulmonary findings and oxygen therapy. We report a case of a primigravida at 38 weeks of gestation with congenital methemoglobinemia, detected during cesarean delivery, and managed conservatively with favorable maternal and neonatal outcomes.

First Page

696

Last Page

698

DOI

10.5005/jp-journals-10006-2769

Publication Date

9-1-2025

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