ABSTRACT
Cornelia de Lange syndrome presents with various problems which include anatomical anomalies of face and extremities, cardiopulmonary and endocrine disorders, renal dysfunction, epileptic EEG waves, and mental retardation. Difficult airway and aspiration risk due to gastroesophageal reflux and poor esophageal motility are the main challenges in anesthesia management. The choice of anesthetic procedure must be carefully considered in view of these abnormalities. We report a 14years old male child who was a known case of Cornelia De Lange Syndrome for dental extraction with restorations under general anesthesia. The uneventful course of the anesthesia in the presented case was due to the thorough systemic evaluation and careful anesthetic strategy