Airway management for patients with craniofacial disorders poses many challenges. Congenital infiltrating lipomatosis of the face (CILF) is an extremely rare disorder in which mature lipocytes invade adjacent tissues in the head and neck. The manifestations are typically unilateral, often with associated hypertrophy of both the hard and soft tissues of the face. This is a case report regarding the anesthetic management for a 5-year-old intellectually disabled female with CILF involving the right side of her face who underwent a successful intubated general anesthetic for dental treatment. Awake fiber-optic intubations are recommended and routinely used for patients with suspected or confirmed difficult airways. In this case, substantial distortion of the normal facial anatomy was observed clinically with noted hypertrophy of the right maxilla, mandible, and right side of the tongue. Further complicating matters was the patient's inability to fully cooperate because of her intellectual disability, precluding the option of an awake fiber-optic intubation. To secure the airway following mask induction of anesthesia, spontaneous ventilation was carefully maintained using sevoflurane, nitrous oxide, and oxygen combined with the application of a nasopharyngeal airway. Despite compression of the oral cavity and upper pharyngeal space by the hypertrophic tissues due to CILF, the space in and around the glottis was preserved. Intubation was completed easily with the use of a fiber-optic scope without any serious complications.

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