Anesthesia in children with osteogenesis imperfecta: Retrospective chart review of 83 patients and 205 anesthetics over 7 years.

Background: Osteogenesis imperfecta is the collective term for a heterogeneous group of connective tissue syndromes characterized by bone fragility with multisystem involvement and perioperative implications.

Aims: Literature review of anesthetic management of patients with osteogenesis imperfecta revealed a paucity of data on the incidence of perioperative challenges. We sought to determine the rates of these challenges in our study cohort.

Methods: Data were collected in a specialty orthopedic hospital from 2008 to 2015 for 83 osteogenesis imperfecta patients undergoing 205 surgeries: 203 orthopedic surgeries and 2 mid-face reconstructive surgeries. Airway management, intravenous access, surgical blood loss, use of peripheral nerve blockade and/or neuraxial techniques, presence of perioperative fracture, and peak intraoperative temperature were evaluated and analyzed.

Results: Difficult airway was encountered in 3/205 (1.5%) cases and perioperative fracture in 2/205 (1%) cases. Neuraxial anesthesia was attempted in 64/205 cases with an 87.5% success rate. All peripheral nerve block attempts (33/205 cases) were successful. Difficult intravenous catheter placement was noted in 8/205 (4%) cases. Estimated blood loss >10% of estimated blood volume was considered significant, and occurred in 35/205 (17%) cases. Significant blood loss occurred more often in severe osteogenesis imperfecta types: 18/76 (23.7%) in Type III and 11/65 (16.9%) in Type IV, whereas only 4/47 (8.5%) occurred in mild Type I. In our 205 case cohort, osteogenesis imperfecta Type III had 5.6 times the odds [(95% CI = 1.8-17.2) P = 0.003] of having an anesthetic complication as compared to osteogenesis imperfecta Type I.

Conclusion: Patients with osteogenesis imperfecta undergo frequent anesthetic exposures, but anesthetic challenges in our series were uncommon. Odds of challenges are greater in severe osteogenesis imperfecta Type III, with significant blood loss and difficulty placing intravenous catheters more likely encountered in the more severe types.