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

Abstract

Summary 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.

A retrospective study to evaluate the anaesthetic choices and complications for patients with osteogenesis imperfecta at a quaternary referral hospital

“…As expected, and similarly to our surgical cohort, the surgical populations described by Hall et al and Rothschild et al had a high incidence of type III OI as type III OI is more severe and often requires more surgery. 10,11 Furthermore, in our study, only nine patients had a positive family history of OI, which differs from the Swedish cohort, where 87% of their cohort had a positive family history and a causative mutation identified. 13 In our study we found a very high incidence (65%) of preoperative anaemia.…”

“…7 All of these have to be carefully considered before anaesthetising these patients. 10,11 Our study is, to our knowledge, the first study describing management of such cases in a low-middle-income country.…”

“…Fibreoptic bronchoscopy and/or a videolaryngoscopy with in-line stabilisation have been suggested to facilitate intubation and to decrease manipulation of the cervical spine 19 especially in patients at high risk for subluxation. 11 They can also have a short neck and pigeon chest which can make laryngoscopy difficult. 8 In our study, most of our patients had their airway secured using a SGAD; only five per cent were intubated, none of which were documented as difficult.…”

Management of Endocrine Disease: Osteogenesis imperfecta: an update on clinical features and therapies

“…As a result, patients are prone to fracture above or below the implant because of stress riser formation (70,71). When surgery is indicated, potential challenges with anesthesia should be considered, especially in patients with moderate to severe OI (72).…”

Assessing bleeding risk in 18 children with Osteogenesis imperfecta

“…Diagnosis is based on clinical presentation, family history, radiographic features and genetic analysis. A 10–30% incidence of bleeding diathesis is reported in patients with OI undergoing surgery 1 . Bleeding may occur despite normal haemostatic tests, making intraoperative bleeding difficult to predict 2 .…”