XXXX : beware of difficult airway.

@entity1 @entity5 @entity281 @entity3061 @entity1576 @entity4238 @entity130 @entity3816
BACKGROUND: @entity3061 represent a number of rare conditions characterized by destruction and resorption of bone that is unrelated to @entity5 , @entity281 , or @entity130 . AIM: To characterize the periprocedural course of @entity1 with primary @entity3061 undergoing procedures that require anesthesia care. METHODS: The medical records database from our institution was searched from 1976 to 2013 to identify @entity1 with @entity3061 who received anesthesia care. We reviewed demographic characteristics, comorbidities, and perioperative course of @entity1 with different forms of primary @entity3061 . A systematic review of the literature was performed to identify reports describing the anesthetic management of @entity1 with these conditions. RESULTS: We identified 11 @entity1 with a primary @entity3061 who received 111 anesthetics. The @entity1 ' ages at the time of surgery ranged from 3 to 62  years. Difficult airway management was a common finding. On preoperative examination, difficulty with endotracheal intubation was predicted in 8 of 11 @entity1 . Three @entity1 had preexisting tracheostomies. One @entity1 required multiple awake fiberoptic intubations. Another two @entity1 where successfully intubated early in life via direct laryngoscopy, but eventually required awake fiberoptic intubations as the disease process caused progressive distortion of airway structures. @entity3816 were absent. In the postoperative period, three @entity1 required prolonged mechanical ventilation due to airway swelling, @entity1576 , and @entity281 . CONCLUSIONS: Airway management may be challenging in @entity1 with @entity3061 . Because the osteolytic process advances with aging and craniofacial @entity4238 may progressively worsen, the airway should always be reassessed before surgery with an appropriate strategy for potentially difficult intubation. In all @entity1 with primary @entity3061 , a potential for pathologic fracture must be considered and careful intraoperative positioning is warranted.
