Anesthetic management of emergency cesarean section in a woman with lipoid proteinosis

Lipoid proteinosis is a rare autosomal recessive disorder of generalized thickening of the skin, mucosa, and certain viscera due to deposition of numerous small clumps of proteins that give the skin a yellowish color. Lipoid deposits in upper respiratory tract, mucous membranes of the mouth and in vocal cords may interfere with airway management during general anesthesia, and hence the regional techniques are usually preferred in known cases. Here the authors present a typical known case of lipoid proteinosis, which presented for emergency cesarean section due to abruptio placentae. She was meticulously managed with spinal anesthesia.