Anesthetic Management of Scoliosis Correction in Mainzer-Saldino Syndrome: A Case Report

Abstract

Introduction: Mainzer-Saldino Syndrome is a rare autosomal recessive disease with mutations in genes that encode components involved in ciliary transport. This syndrome is characterized by chronic renal failure, severe retinal dystrophy and skeletal abnormalities including spinal and chest wall deformity resulting in severe respiratory failure. Case presentation: We report the first successful anesthetic management of a 26-year-old man with Mainzer-Saldino Syndrome who underwent scoliosis deformity surgical correction. The severity of respiratory problems and renal dysfunction that characterize this syndrome require a multidisciplinary preoperative assessment and careful planning of intraoperative management, also in relation to surgical complications. Conclusions: A careful preoperative assessment is essential for the correct anesthetic management. General anesthesia was safely administered; however a constant modulation of controlled ventilation is required to avoid barotrauma and an invasive hemodynamic monitoring is critical to allow adequate fluid management. Lastly, intraoperative dialysis can be planned in long-lasting surgery with a significant loss and replacement of fluids. Keywords: Mainzer-Saldino Disease; Ciliopathy; Anaesthesia.