Anesthetic Care During Lateral Thoracic Expansion for Asphyxiating Thoracic Dystrophy

Abstract

Jeune syndrome or asphyxiating thoracic dystrophy (JATD) is a rare autosomal recessive disorder with an estimated incidence of 1 in 100,000 to 130,000 live births. The typical phenotype of JATD is osteochondrodysplasia (narrow thorax and shorted ribs) in association with short limb dwarfism. The thoracic dysplasia results in respiratory insufficiency, usually requiring tracheostomy and chronic mechanical ventilation beginning in infancy and childhood. Lateral thoracic expansion is one surgical procedure that has been described in an attempt to enlarge the thoracic cage and alleviate the progressive respiratory failure. In addition to respiratory involvement, patients with JATD may have other co-morbid conditions involving the kidneys, pancreas, liver, and eyes. Given the underlying thoracic dysplasia and other associated comorbid conditions, the intraoperative care of such patients can be challenging. We present two patients with JATD who presented for lateral thoracic expansion. Previous reports of the intraoperative care of such patients are reviewed and options for anesthetic care are discussed.