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

Abstract

Background: Osteogenesis imperfecta (OI) is an inherited genetic syndrome affecting connective tissue. Patients often undergo surgery due to an increased susceptibility to bone fractures. Anaesthesia is associated with many perioperative challenges. This study aimed to describe and evaluate the perioperative management of OI paediatric patients presenting for surgery at Inkosi Albert Luthuli Central Hospital (IALCH).
Methods: A retrospective chart review of children under 18 years who had OI and underwent surgical procedures from 2000 to 2017 at a quaternary referral hospital was conducted. Patients were identified from the electronic patient database. The following variables were extracted: demographic data, preoperative history, examination, investigations, chronic medications, intra- and postoperative management and perioperative complications. Simple descriptive statistics were performed using a Microsoft® Excel spreadsheet.
Results: Thirty-nine patients who underwent 93 surgeries were included. The majority (72.1%) had severe type III OI and had elective orthopaedic surgery. Anaemia was identified in 64.5% of patients; 40.8% had a spinal deformity and 37.6% had an abnormality on respiratory examination. A supraglottic airway device (SGAD) was used in 91.9% of patients, with only three airway complications. Eighty-seven per cent of cases had combined general (GA) and regional anaesthesia (RA). No children had documented signs suggestive of hypermetabolism or malignant hyperthermia.
Conclusion: Despite most patients in our study having severe OI, few of the complications and difficulties described in the literature were identified. A combined GA and RA technique with a SGAD was shown to be a safe anaesthesia technique. Improved preoperative investigation, especially a full blood count due to the high incidence of anaemia, should be encouraged to improve overall care.