The anesthetic literature contains no focused discussion of the perioperative management and risks of children with malignant autosomal recessive osteopetrosis (osteopetrosis). We retrospectively analyzed the perioperative morbidity and mortality rates encountered in the anesthetic management of children with osteopetrosis. We compared the perioperative mortality rate for this patient population with that for other pediatric patients in our institution and that reported in the literature for children and other high-risk patients. We also investigated the inability to intubate the tracheas of children with osteopetrosis compared with other pediatric patients in our institution. Using Fisher's exact test, patients with osteopetrosis were found to have a higher likelihood of perioperative mortality compared with other children or all ASA physical status III, but not ASA physical status IV, patients (P < 0.05). Finally, we discovered that children with osteopetrosis were more likely to have tracheas that could not be intubated than other pediatric patients in our institution. We conclude that children with osteopetrosis are at risk of adverse respiratory events and mortality associated with these adverse events.
Implications: Osteopetrosis is a rare disease that increases perioperative morbidity and mortality. By performing a retrospective chart review, we found that this increased perioperative morbidity and mortality is primarily related to airway and respiratory factors. Anesthetic management strategies should consider the factors that cause the high frequency of adverse airway events in this patient population.