Perioperative Management of a Patient With Cornelia de Lange Syndrome and Tetralogy of Fallot
This is a case report of a 21-year-old male patient with Cornelia de Lange syndrome (CdL) and unrepaired tetralogy of Fallot scheduled for dental treatment under general anesthesia. Anticipated dental care consisted of restorative treatment and extractions. Surgical correction of the patient's congenital cardiac abnormalities had not occurred by the time of dental treatment. As such, the developed anesthetic plan included the following goals: prevention of any anoxic episodes or spell and preparation for difficult airway management due to micrognathia secondary to CdL. To help ensure adequacy of oxygenation during induction, the normal anesthetic face mask was specially modified with a hole to permit use of the fiberoptic scope during induction and intubation. With preoperative consultation involving a cardiologist as well as the use of the modified mask, general anesthesia was safely administered without any complications.
Anesthesia face mask modified with a hole.
The mask during intubation with the fiberoptic scope. While maintaining spontaneous breathing, the endotracheal tube with the connector removed beforehand was passed through the hole in the mask and into the nostril. The fiberoptic scope was then inserted through the endotracheal tube. After successful intubation, endotracheal tube was reconnected to the anesthetic circuit. This maneuver allowed administration of anesthetic gases throughout the fiberoptic intubation.
Contributor Notes