Abstract
Osteogenesis imperfecta (OI) is a rare genetic connective-tissue disorder with bone fragility. To avoid iatrogenic fractures, healthcare providers have traditionally avoided using non-invasive blood pressure (NIBP) cuffs and extremity tourniquets in the OI population in the perioperative setting. Here, we hypothesize that these procedures do not lead to iatrogenic fractures or other complications in patients with OI. A retrospective study of all children with OI who underwent surgery at a single tertiary care children’s hospital from 1998 to 2018 was performed. Patient positioning and the use of NIBP cuffs, arterial lines, and extremity tourniquets were documented. Fractures and other complications were recorded. Forty-nine patients with a median age of 7.9 years (range: 0.2–17.7) were identified. These patients underwent 273 procedures, of which 229 were orthopaedic operations. A total of 246 (90.1%) procedures included the use of an NIBP cuff, 61 (22.3%) an extremity tourniquet, and 40 (14.7%) an arterial line. Pediatric patients with OI did not experience any iatrogenic fractures related to hemodynamic monitoring or extremity tourniquet use during the 20-year period of this study. Given the benefits of continuous intra-operative hemodynamic monitoring and extremity tourniquets, we recommend that NIBP cuffs, arterial lines, and tourniquets be selectively considered for use in children with OI.
Highlights
Osteogenesis imperfecta (OI) is a collection of rare genetic connective tissue disorders that affects bone, tendons, skin, teeth, fibrocartilage, cornea, and endomysium and causes bone fragility and deformities [1,2,3]
An institutional review board (IRB) approved retrospective review of pediatric OI patients who underwent an operative procedure at a single tertiary care center was performed
Seventeen of the 49 patients were formally diagnosed with OI type I, which was the most common subtype in the cohort
Summary
Osteogenesis imperfecta (OI) is a collection of rare genetic connective tissue disorders that affects bone, tendons, skin, teeth, fibrocartilage, cornea, and endomysium and causes bone fragility and deformities [1,2,3]. Subtypes of OI are based on the specific type of collagen affected and display differing frequency and severity of symptoms, ranging from frequent fractures to intrauterine demise [3,4]. Type III is the most severe non-lethal form of the more common autosomal dominant types [3]. There are less common autosomal recessive types that have severe phenotypes, such as types VII–X and XII [3]. These patients typically have a history of multiple fractures from infancy, significant bone deformities, and short stature [3]. Other symptoms of OI include hearing loss, blue sclera, kyphosis and scoliosis, vascular anomalies, poor dentition, and cardiovascular compromise [2,3]
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