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.

Comprehensive risk assessments and anesthetic management for children with osteogenesis imperfecta: A retrospective review of 252 orthopedic procedures over 5 years

“…Of these, only four cases (2.6%) encountered difficulties in neuraxial access or catheter placement (Table 2). This ratio is comparable to or lower than those reported by Rothschild et al 17 (8 out of 205) and Cronj et al 25 (8 out of 93). Peripheral nerve blocks were performed preoperatively on ten cases, all of which were guided by ultrasound.…”

“…(8 out of 205) and Cronj et al25 (8 out of 93). Peripheral nerve blocks were performed preoperatively on ten cases, all of which were guided by ultrasound.…”

“…First, many studies spanned long periods of time when anesthesiology and orthopedics significantly evolved. For example, a recent retrospective study of the African population covers a period of 17 years, 25 while data in the seminal work by Hall et al 16 spanned 30 years. Second, the factors and predictors for anesthetic risks were not analyzed quantitatively.…”

Complicaciones relacionadas al manejo anestésico de pacientes pediátricos con osteogénesis imperfecta sometidos a procedimientos ortopédicos

“…Nuestra investigación contiene algunas semejanzas con este autor en cuanto a la utilización de variables de complicaciones anestésicas tales como hemorragia, problemas en la ventilación o presencia de vía aérea difícil. Mohmmad y su equipo (18) en su estudio (2020) realizaron un registro en 17 años de 93 procedimientos anestésicos ortopédicos y no ortopédicos de un total de 39 pacientes. Incluyeron variables de características preoperatorias muy semejantes a las nuestras como características de posible vía aérea difícil (reducción de la apertura oral, Mallampati, disminución del movimiento del cuello), deformaciones de la columna, alteraciones clínicas cardiológicas y pulmonares, características de la anestesia, complicaciones transoperatorias como dificultades en el manejo de la vía aérea, del abordaje de la anestesia regional, transfusión sanguínea transoperatoria.…”

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“…In a contrasting article, Mohmmad and colleagues provide valuable insight into the anaesthetic management of a rare disease. 8 Patients with osteogenesis imperfecta (OI) provide an array of challenges to the anaesthesiologist (depending on the severity of the condition) and often require multiple and complex surgeries representing numerous anaesthetic consultations, as seen in this cohort with 93 procedures for 39 patients.…”