Anesthetic management of an adult patient with Cornelia de Lange Syndrome

Abstract

Cornelia de Lange Syndrome is a well-known congenital disease characterized by multiple malformations that make anesthetic management a challenge. Since few individuals survive the earliest stage of life, papers describing the details of anesthetic management are mainly reported in the pediatric population. These individuals who move into adulthood develop physical changes that should be taken into consideration. This article reports the case of a 34-year-old patient undergoing an orthopedic procedure. The operation was performed under general anesthesia because the patient showed severe scoliosis and joint contractures, so extraordinary difficulty in neuroaxial and peripheral techniques was expected. The aggressive behavior of the patient impeded the proper assessment of a preoperative airway. Fiber optic intubation was performed under slight sedation after three unsuccessful attempts. Dental crowding, prominent upper central incisors that were more pronounced than in most children, a short and stiff neck, and poor patient cooperation due to mental retardation and occasional autistic behavior made airway management difficult. This case should alert anesthesiologists to the greater difficulties of managing patients with Cornelia de Lange Syndrome when they become adults.