scholarly journals Diaphyseal Femur Fractures in Osteogenesis Imperfecta: Characteristics and Relationship With Bisphosphonate Treatment

2017 ◽  
Vol 32 (5) ◽  
pp. 1034-1039 ◽  
Author(s):  
Pamela Trejo ◽  
François Fassier ◽  
Francis H Glorieux ◽  
Frank Rauch
Bone ◽  
2010 ◽  
Vol 46 ◽  
pp. S18-S19
Author(s):  
Francesco Doro ◽  
Paolo Cavarzere ◽  
Elena Monti ◽  
Giacomo Venturi ◽  
Silvano Adami ◽  
...  

2015 ◽  
Vol 15 (3) ◽  
pp. 313-320 ◽  
Author(s):  
Heidi Arponen ◽  
Ilkka Vuorimies ◽  
Jari Haukka ◽  
Helena Valta ◽  
Janna Waltimo-Sirén ◽  
...  

OBJECT Cranial base pathology is a serious complication of osteogenesis imperfecta (OI). Our aim was to analyze whether bisphosphonate treatment, used to improve bone strength, could also prevent the development of craniocervical junction pathology (basilar impression, basilar invagination, or platybasia) in children with OI. METHODS In this single-center retrospective study the authors analyzed the skull base morphology from lateral skull radiographs and midsagittal MR images (total of 94 images), obtained between the ages of 0 and 25 years in 39 bisphosphonate-treated OI patients. The results were compared with age-matched normative values and with findings in 70 OI patients who were not treated with bisphosphonates. In addition to cross-sectional data, longitudinal data were available from 22 patients with an average follow-up period of 7.6 years. The patients, who had OI types I, III, IV, VI, and VII, had been treated with zoledronic acid, pamidronate, or risedronate for 3.2 years on average. RESULTS Altogether 33% of the 39 bisphosphonate-treated patients had at least 1 cranial base anomaly, platybasia being the most prevalent diagnosis (28%). Logistic regression analysis suggested a higher risk of basilar impression or invagination in patients with severe OI (OR 22.04) and/or older age at initiation of bisphosphonate treatment (OR 1.45), whereas a decreased risk was associated with longer duration of treatment (OR 0.28). No significant associations between age, height, or cumulative bisphosphonate dose and the risk for cranial base anomaly were detected. In longitudinal evaluation, Kaplan-Meier curves suggested delayed development of cranial base pathology in patients treated with bisphosphonates but the differences from the untreated group were not statistically significant. CONCLUSIONS These findings indicate that cranial base pathology may develop despite bisphosphonate treatment. Early initiation of bisphosphonate treatment may delay development of craniocervical junction pathology. Careful followup of cranial base morphology is warranted, particularly in patients with severe OI.


2006 ◽  
Vol 149 (2) ◽  
pp. 174-179 ◽  
Author(s):  
Franco Antoniazzi ◽  
Giorgio Zamboni ◽  
Silvana Lauriola ◽  
Luisa Donadi ◽  
Silvano Adami ◽  
...  

2014 ◽  
Vol 81 (3) ◽  
pp. 204-210 ◽  
Author(s):  
Andrew Biggin ◽  
Julie N. Briody ◽  
Elizabeth Ormshaw ◽  
Karen K.Y. Wong ◽  
Bruce H. Bennetts ◽  
...  

2018 ◽  
Vol 177 (6) ◽  
pp. 891-902 ◽  
Author(s):  
Argerie Tsimicalis ◽  
Madalina Boitor ◽  
Catherine E. Ferland ◽  
Frank Rauch ◽  
Sylvie Le May ◽  
...  

2015 ◽  
Vol 30 (8) ◽  
pp. 1362-1368 ◽  
Author(s):  
Emad A Anam ◽  
Frank Rauch ◽  
Francis H Glorieux ◽  
François Fassier ◽  
Reggie Hamdy

2019 ◽  
Vol 7 (1) ◽  
pp. 87-96
Author(s):  
Mikhail E. Burtsev ◽  
Aleksandr V. Frolov ◽  
Aleksei N. Logvinov ◽  
Dmitry O. Ilyin ◽  
Andrey V. Korolev

Aim. Osteogenesis imperfecta (OI) is characterized by bone fragility and long bones deformities. Most studies are dedicated to surgical treatment of diaphyseal fractures. To our knowledge, there are no reports giving recommendations about surgical treatment of distal femur intraarticular fractures. Clinical case. We describe the surgical treatment of a 14-year-old girl with OI who had intraarticular fracture of the left distal femur and fracture of a right femur diaphysis. Surgical treatment was complicated by migration of a titanium elastic nail and impaired consolidation, which had to be fixed with a plate and led to peri-implant fracture. Results were assessed before trauma and at 1 and 2 years after trauma with Gillette Functional Assessment Questionnaire (GFAQ) and Bleck score. Discussion. During surgical treatment of comminuted intraarticular distal femur fractures in patients with OI, we had to use big cancellous screw that made implantation in an intramedullary fixator more difficult. Internal fixation with a plate in patients with OI is associated with high risks of peri-implant fracture. Conclusion. For treatment of comminuted intraarticular fracture of the distal femur, it is necessary to have large variety of internal fixators, follow the principles of absolute and relative stability, and be familiar with minimally-invasive techniques.


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