Primary osteolysis syndromes: beware of difficult airway.
BACKGROUND
Primary osteolysis syndromes represent a number of rare conditions characterized by destruction and resorption of bone that is unrelated to neoplasia, infection, or trauma.


OBJECTIVE
To characterize the periprocedural course of patients with primary osteolysis syndrome undergoing procedures that require anesthesia care.


METHODS
The medical records database from our institution was searched from 1976 to 2013 to identify patients with primary osteolysis syndromes who received anesthesia care. We reviewed demographic characteristics, comorbidities, and perioperative course of patients with different forms of primary osteolysis. A systematic review of the literature was performed to identify reports describing the anesthetic management of patients with these conditions.


RESULTS
We identified 11 patients with a primary osteolysis syndrome who received 111 anesthetics. The patients' 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 patients. Three patients had preexisting tracheostomies. One patient required multiple awake fiberoptic intubations. Another two patients 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. Intraoperative complications were absent. In the postoperative period, three patients required prolonged mechanical ventilation due to airway swelling, respiratory failure, and infection.


CONCLUSIONS
Airway management may be challenging in patients with primary osteolysis syndromes. Because the osteolytic process advances with aging and craniofacial dysmorphism may progressively worsen, the airway should always be reassessed before surgery with an appropriate strategy for potentially difficult intubation. In all patients with primary osteolysis, a potential for pathologic fracture must be considered and careful intraoperative positioning is warranted.
Department of Anesthesiology, College of Medicine, Mayo Clinic, Rochester, MN, USA.
R Scott
RS
Herd
Department of Anesthesiology, College of Medicine, Mayo Clinic, Rochester, MN, USA.
Juraj
J
Sprung
Department of Anesthesiology, College of Medicine, Mayo Clinic, Rochester, MN, USA.
Toby N
TN
Weingarten
D000293:Adolescent; D000328:Adult; D058109:Airway Management / Q000379:methods*; D000402:Airway Obstruction / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D019465:Craniofacial Abnormalities / Q000150:complications; D005260:Female; D006801:Humans; D007431:Intraoperative Complications / Q000517:prevention & control; D008297:Male; D008875:Middle Aged; D010014:Osteolysis / Q000150:complications*; D012189:Retrospective Studies; D013577:Syndrome; D055815:Young Adult
Hajdu-Cheney syndrome; anesthesia complications: difficult intubation; osteolysis, essential; progeria; pycnodysostosis