Anesthetic considerations in Anderman Syndrome. Case report.

Abstract

Anderman syndrome is a hereditary progressive demyelinating sensory-motor neuropathy, of an autosomal recessive nature, due to a mutation of the SLC12A6 gene, responsible for the synthesis of the K-Cl cotransporter that seems to be necessary for the development and sustenance of nervous tissue. These patients present a dysmorphic facies phenotype with hypertelorism and brachycephaly, generalized areflexia, amyotrophy, and severe muscle contractures. Associated comorbidities and anatomical-functional peculiarities can condition perioperative management, posing a challenge for the anesthesiologist. Regarding a case, we will carry out a review of this syndrome, highlighting the points that we consider important in order to serve other anesthetists as a reference when dealing with these patients.