Anesthetic implications of Larsen syndrome

In this article I present two children with Larsen syndrome who required anesthetic care during surgical procedures of the ear-nose-throat (ENT) region. The anesthetic implications of this disorder are discussed. Larsen syndrome consists of multiple joint dislocations and a characteristic facies. Associated orthopedic abnormalities include cervical spine vertebral body hypoplasia and hemivertebrae, which may lead to cervical kyphosis and compromise of cervical cord function. Of prime importance to the anesthesiologist are the associated defects of the airway including laryngotracheomalacia, which may predispose the patient to perioperative respiratory compromise. Associated cardiac defects have also been described including atrial and ventricular septal defects.