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. 2020 Sep 11;5(3):e20.00031.
doi: 10.2106/JBJS.OA.20.00031. eCollection 2020 Jul-Sep.

A Multicenter Study of Intramedullary Rodding in Osteogenesis Imperfecta

Affiliations

A Multicenter Study of Intramedullary Rodding in Osteogenesis Imperfecta

Mercedes Rodriguez Celin et al. JB JS Open Access. .

Abstract

Background: Osteogenesis imperfecta (OI), a heritable connective tissue disorder with wide clinical variability, predisposes to recurrent fractures and bone deformity. Management requires a multidisciplinary approach in which intramedullary rodding plays an important role, especially for moderate and severe forms. We investigated the patterns of surgical procedures in OI in order to establish the benefits of rodding. The main hypothesis that guided this study was that rodded participants with moderate and severe OI would have lower fracture rates and better mobility.

Methods: With data from the Linked Clinical Research Centers, we analyzed rodding status in 558 individuals. Mobility and fracture data in OI Types III and IV were compared between rodded and non-rodded groups. Univariate regression analyses were used to test the association of mobility outcomes with various covariates pertinent to rodding.

Results: Of the individuals with OI, 42.1% had undergone rodding (10.7% of those with Type I, 66.4% with Type III, and 67.3% with Type IV). Rodding was performed more frequently and at a younger age in femora compared with tibiae. Expanding intramedullary rods were used more frequently in femora. In Type III, the rate of fractures per year was significantly lower (p ≤ 0.05) for rodded bones. In Type III, the mean scores on the Gillette Functional Assessment Questionnaire (GFAQ) and Brief Assessment of Motor Function (BAMF) were higher in the rodded group. However, Type-IV non-rodded subjects had higher mean scores in nearly all mobility outcomes. OI type, the use of expanding rods in tibiae, and anthropometric measurements were associated with mobility outcomes scores.

Conclusions: Current practice in 5 orthopaedic centers with extensive experience treating OI demonstrates that most individuals with moderate and severe types of OI undergo rodding procedures. Individuals with severe OI have improved mobility outcomes and lower fracture rates compared with their non-rodded peers, which suggests that early bilateral rodding benefits OI Type III. Our analysis showed a change in practice patterns in the final years of the study in the severe forms, with earlier and more simultaneous rodding procedures performed.

Level of evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

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Conflict of interest statement

Disclosure: The study was supported by the BBDC (1U54AR068069-0), a part of the Rare Diseases Clinical Research Network (RDCRN) of the National Center for Advancing Translational Sciences (NCATS); the Clinical Translational Core of the Baylor College of Medicine Intellectual and Developmental Disabilities Research Center (BCM IDDRC) (1U54HD083092) from the Eunice Kennedy Shriver NICHD (National Institute of Child Health and Human Development); U.S. Department of Health and Human Services/National Institute on Disability, Independent Living, and Rehabilitation Research (HHS/NIDILRR) grant 90AR5022-01; and internal support from Shriners Hospitals for Children. The BBDC is funded through the Osteogenesis Imperfecta (OI) Foundation and the Office of Rare Diseases Research (ORDR) of the NCATS, the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), the National Institute of Child Health and Human Development (NICHD), and the National Institute of Dental and Craniofacial Research (NIDCR). On the Disclosure of Potential Conflicts of Interest forms, which are provided with the online version of the article, one or more of the authors checked “yes” to indicate that the author had a relevant financial relationship in the biomedical arena outside the submitted work (http://links.lww.com/JBJSOA/A198).

Figures

Fig. 1-A
Fig. 1-A
Preoperative radiograph showing the left femur with a mid-diaphyseal femoral fracture sustained while the patient was dancing competitively.
Fig. 1-B
Fig. 1-B
Postoperative radiograph showing a 5.4-mm Fassier-Duval expanding rod.
Fig. 2-A
Fig. 2-A
Fig. 2-B
Fig. 2-B
Fig. 2-C
Fig. 2-C
Postoperative radiograph showing the fragmentation and insertion of expanding 3.2-mm Fassier-Duval rods in both femora and tibiae has been performed in a staged fashion. The surgical procedure was performed when the patient began pulling to stand. The goal of the surgical procedure was to improve alignment and stability, reduce the fracture rate, and increase mobility.
Fig. 3-A
Fig. 3-A
Preoperative radiograph showing the severe deformity of the femora and tibiae.
Fig. 3-B
Fig. 3-B
Postoperative radiograph. When the patient became more mobile and attempted pulling to stand, he underwent fragmentation and rodding of both femora and tibiae, the 2 legs (the tibia and the femur in each) were staged 2 weeks apart. The diameter of the tibiae precluded the use of expanding rods, so non-expanding rods were used in both tibiae (2.38-mm [3/32-inch] Steinmann pins) and expanding rods (3.2-mm Fassier-Duval) were inserted in the femora.
Fig. 4
Fig. 4
Anteroposterior radiograph of the lower limbs of a 4-year-old boy with OI Type IV who underwent isolated rodding of the left femur with the insertion of a 4.0-mm Fassier-Duval expanding rod after a femoral fracture while attempting to run. He has undergone cyclic bisphosphonate treatment since he was 6 months of age. Currently, he is a community ambulator and demonstrates mild bowing of the contralateral femur.
Fig. 5
Fig. 5
Percentage of Type-III subjects responding “yes” to being able to complete each task from the GFAQ: the rodded group compared with the non-rodded group.
Fig. 6
Fig. 6
Percentage of Type-IV subjects responding “yes” to being able to complete each task from the GFAQ: the rodded group compared with the non-rodded group.

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