The Journal of Tehran University Heart Center Case Report Locked-in Syndrome and Blue Toe Syndrome Caused by Cardiopulmonary Bypass Feridoun Sabzi, MD*, Abdolrasoul Moloudi, MD Imam Ali Heart Center, Kermanshah University of Medical Sciences, Kermanshah, Iran. Received 27 December 2008; Accepted 25 April 2009 AAbbsstrtraacctt Severe inflammation after cardiopulmonary bypass with the vasculitis of the acral extremity and vertebro-basilar arterial system leads to the locked-in syndrome and blue toe syndrome. In broad terms, systemic, idiopathic, and environmental factors provoke syndromes that present with digital discoloration or the blue toe syndrome. Painful digital discoloration, accompanied by ulceration, suggests vasculitis, involving small blood vessels. Definitive diagnosis usually requires histological documentation because vasculitic syndromes have no pathognomonic clinical features or laboratory test results. The case introduced herein is that of a woman who developed the locked-in syndrome in conjunction with quadriplegia, loss of facial movement, speech loss, and loss of horizontal eye movements. She had initially presented with severe mitral stenosis and left atrial clot and undergone mitral valve replacement and clot extraction. The patient expired from multiple organ failure despite prolonged ventilatory support, including tracheotomy, and meticulous nursing care and antibiotic prophylaxis. Given the previously reported partial recovery from this syndrome with the use of steroids, we would advocate the use of such pharmacological agents. J Teh Univ Heart Ctr 3 (2010) 150-152 Keywords: Cardiopulmonary bypass • Postoperative complications • Cardiac surgical procedures • Blue toe syndrome Introduction The systemic inflammatory response to cardiopulmonary bypass is a modification of the physiological response to tissue injury or infection. Activation of leukocytes, platelets, complement, and factor XII by contact with the bypass circuit and surgical trauma is followed by the systemic secretion of cytokines and other inflammatory mediators. The induced expression of adhesion molecules on activated leukocytes and endothelial cells can result in the sequestration of white cells within tissues and a clinical syndrome, the systemic inflammatory response syndrome (SIRS), which differs quite widely among patients. In its extreme form, it can lead to multiple organ failure that often includes the adult respiratory distress syndrome, a condition associated with massive leukocyte infiltration in the lung and high mortality.1 The locked-in syndrome is a rare clinical entity consisting of quadriplegia, paralysis of the lower cranial nerve, mutism, and bilateral paresis of horizontal gaze and is associated with unaltered consciousness, with intact vertical eye movements and blinking allowing some form of human communication by way of eye codes. Furthermore, there is usually cranial nerve weakness with the sparing of only the more laterally placed nuclei.2 The locked-in syndrome results most commonly from a lesion in vertical pons, although extensive bilateral destruction of cortical-bulbar and cortico-spinal tracts in the pedunculi cerebri may be responsible. Sensory impairment due to medial lemniscuses involvement and damage to the decussating fibers from the spinal tract of trigeminal nerve are inevitable. The lesion at *Corresponding Author: Feridoun Sabzi, Associate Professor of Cardiac Surgery, Kermanshah University of Medical Sciences, Imam Ali Hospital, Shaheed BeheshtiAve, Kermanshah, Iran. eTl: +98 918 131896. Fax: +98 831 8360043. E-mail: dr_Sabzi@yahoo.com. 150