Anesthetic considerations in the perioperative management of the patient with Jarcho-Levin Syndrome

Abstract

Jarcho-Levin syndrome is an eponym used to describe a spectrum of small thoracic skeletal dysplasias with variable involvement of vertebrae and ribs. Initially considered lethal, it is currently compatible with life in its mildest forms. Bone alterations that lead to a restrictive respiratory pattern, recurrent respiratory infections and particular phenotype, can make perioperative anesthetic management difficult. The proper assessment of the airway is of special interest because it presents predictors of a difficult airway, as well as the prevention, early diagnosis and adequate treatment of respiratory failure. We present the case of a patient with Jarcho-Levin Syndrome who underwent vertebral distraction surgery, with its most notable implications in anesthetic management.

Keywords: Alteraciones vertebrales; Anestesia; Anesthesia; Disease management; Insuficiencia respiratoria; Jarcho-Levin syndrome; Manejo de la enfermedad; Respiratory insufficiency; Síndrome de Jarcho-Levin; Vertebral defects.