Hemodynamic instability during prone spine surgery in a patient with merosin-deficient congenital muscular dystrophy

Trans-esophageal echocardiography (TEE) and/or central venous pressure (CVP) monitoring are important in the anesthetic management of spine fusion of pediatric patients with severe muscular weakness. This case highlights an unusual situation of apparent acute right ventricular mechanical obstruction after prone positioning and its prompt recognition with CVP monitoring. The anesthetic management of a patient with congenital muscular dystrophy, an uncommon neuromuscular disorder, is presented. Good communication and planning between the anesthesiology and surgical teams allowed completion of the procedure using a lateral approach.