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Acetabular Protrusion in a Cohort of Patients with Osteogenesis Imperfecta Evaluated in a Pediatric Hospital
Rosario Ramos-Mejía1, Francisco Monterroza-Quintana1, Claudio Primomo1
1Department of Growth and Development, Paediatric Hospital Dr Juan P. Garrahan, Buenos Aires, Argentina.
Insights
Acetabular protrusion (AP) is common in osteogenesis imperfecta (OI). This study found AP prevalence in 22.5% of pediatric OI patients, particularly those with moderate to severe forms, and linked it to age and non-ambulatory status.
Area of Science:
- Pediatric Orthopedics
- Skeletal Dysplasias
- Radiology
Background:
- Acetabular protrusion (AP) affects a significant portion of osteogenesis imperfecta (OI) patients.
- AP is under-described in pediatric populations, necessitating further investigation.
Purpose of the Study:
- To determine the incidence and associations of acetabular protrusion (AP) in pediatric patients with osteogenesis imperfecta (OI).
Main Methods:
- Retrospective and cross-sectional evaluation of 71 pediatric OI patients (ages 2-19.5).
- Radiographic assessment included center-edge (CE) angle and acetabular line crossing Kohler's line.
- Statistical analysis using univariate and multivariate logistic regression to identify risk factors and complications.
Main Results:
- Acetabular protrusion (AP) was present in 22.5% of all pediatric OI patients and 41% of those with moderate to severe OI.
- Abnormal CE angles were found in 71.8% of children, irrespective of OI severity.
- AP was significantly associated with older age (p=0.0062) and non-ambulatory status (p=0.0093).
Conclusions:
- Acetabular protrusion (AP) is highly prevalent in pediatric patients with moderate to severe osteogenesis imperfecta (OI), even at younger ages.
- Abnormal hip morphology, indicated by CE angle, is common across all OI severity groups.
- OI severity and patient age are key factors associated with AP, while ambulatory status shows a negative correlation.
Abstract:
Acetabular protrusion (AP) is present in 33 to 55% of patients with osteogenesis imperfecta (OI). Even though the finding is relatively common, it is poorly described in pediatric patients. The objective of this study was to describe the incidence and associations of AP in pediatric OI patients. We retrospectively and cross-sectionally evaluated clinical histories and radiographic findings of OI patients aged 2 to 19.5 years, recording sex, age, severity, anthropometric measurements, ambulation status, femoral fractures history, and occurrence of orthopaedic surgeries and nephropathy. AP was considered present when the center-edge (CE) angle was more than 35 degrees and the acetabular line crossed the Kohler's line by more than 1 and 3 mm in boys and girls, respectively, and 3 and 6 mm in adult males and females, respectively. The association with risk factors and complications was analyzed through univariate and multivariate logistic regression. A total of 71 children were evaluated. The median age was 8.6 years, and 54.9% of them had moderate to severe forms of OI. In 71.8% of the children, an abnormal CE angle was found, being frequent in mild, moderate, and severe cases. AP was present in 22.5% of all patients and in 41% of children with moderate to severe OI, and was significantly associated with older ages ( p = 0.0062) and nonwalking status ( p = 0.0093). We found a high prevalence of AP in children with moderate to severe forms of OI, which was present even at younger ages. In addition, we found a significant increase in the number of children with abnormal CE angles even in those with mild forms of OI. The presence of AP was associated with the severity of the OI and age, and in a negative association with the ambulatory status.
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