A Clinical Case of Successful Anesthesia in Surgical Correction of Severe Scoliotic Deformity of Spine of a Very Severe Degree in a Patient with Pelizeaus–Merzbacher Disease

INTRODUCTION: Pelizeaus–Merzbacher disease is an orphan disorder caused by a mutation in the PLP1 gene, linked with X chromosome. Its incidence is 1.4–1.9 per 100 thousand people in the general population. The disease is a hereditary leukodystrophy which predominantly affects the central nervous system (CNS) and is characterized by a sharp decrease or absence of myelin sheaths in the white matter areas of the CNS. Impaired neuromuscular conduction, reduced muscle tone may lead to scoliotic spinal deformation including that of a very severe degree. The article describes methodical approaches to surgical correction, anesthetic support and postoperative management of a patient with Pelizeaus–Merzbacher disease taking into account the pathogenetic features of a rare nosological form. CONCLUSION. The presented clinical case demonstrates the possibility of successful provision of anesthetic support and early postoperative management of a patient with a rare severe pathology. A thorough preoperative examination including spirometry and echocardiography, permitted to identify potential problems in the postoperative period and prevent them. The use of modern blood conservation methods and online monitoring of hemoglobin level provided stable hemodynamics and tissue oxygenation throughout the entire intervention.