Assessment of perioperative bleeding in pediatric osteogenesis imperfecta patients undergoing orthopedic surgeries: A single-center study

Abstract

Background “Osteogenesis imperfecta (OI)” is a heterogeneous group of inherited disorders involving collagen type I with a 10–30% incidence of bleeding diathesis placing these individuals at an increased risk of bleeding. Our study aims to ascertain the risk factors that increase the propensity of perioperative blood transfusions in pediatric population. Methods A single center retrospective observational study of patients aged <18 years undergoing orthopedic surgeries at our tertiary care hospital from January 2016 to August 2024 were included and each surgery was analyzed as a discrete event. Age, type of OI, number of osteotomies, and number of bones operated simultaneously were recorded. The fall in hemoglobin, requirement for blood transfusions and associated factors were analyzed. Results Amongst 53 individuals who underwent surgery, 141 procedures were analyzed. The mean age was 11.07± 5.29 years, majority (n=33;62.2%) were phenotypically type III OI, and 42/53 (79%) patients underwent multiple osteotomies. Significant hemoglobin fall was observed after surgery (p<0.001) and was considerable in patients undergoing various osteotomies (p=0.002). Older children showed a smaller drop in hemoglobin level. Patients with type III OI showed significant hemoglobin fall (1.82 ± 0.86 g/dL). The transfusion requirement was seen in 23 (16.3%) surgeries besides it was significant in those undergoing multiple osteotomies (p= <0.05). Conclusion Our study inferred that the fall in hemoglobin was significant postoperatively in OI patients and was associated with younger age, type III OI, and multiple osteotomies. Before performing orthopedic surgeries on patients with OI, surgeons might consider the latter risk factors to explore therapeutic options aimed at reducing hemorrhage and improving outcomes.