2018 Volume 46 Issue 1 Pages 13-15
Cornelia de Lange syndrome (CdL) is characterized by growth disturbance, mental retardation, congenital heart disease and multiple malformations.
We report the case of a 21-year-old patient with CdL and tetralogy of Fallot (TOF) who was scheduled to undergo dental treatment under general anesthesia because of multiple tooth caries. Radical cardiac surgery had not been performed. We consulted a cardiologist regarding the patient’s cardiac condition prior to the operation. Furthermore, mental retardation prevented us from making a precise assessment of the general status. TOF is a complex of congenital cardiac malformations requiring careful attention to hemodynamics stability and infectious endocarditis. The main points of anesthetic management were the avoidance of anoxic spells and trismus. An anoxic spell can be caused by stress, and its mechanism results in an increase in right-to-left blood flow because of pulmonary spasm. We planned a slow induction with the maintenance of spontaneous breathing and fiberoptic intubation as the airway management. Then, a mask with one hole was prepared. The purpose of this device was to administer the inhalation anesthetic to the patient while operating via fiberoptic intubation. Using this device, nasal intubation was successfully established. Anesthesia was maintained with remifentanil and propofol. During the operation, the patient’s blood pressure, heart rate and arterial oxygen saturation remained stable.
The general anesthesia was well maintained, and no perioperative complications occurred. The preoperative creation of an anesthetic plan in cooperation with medical specialists that is capable of coping with various situations, including hospitalization after the operation, is important.
