Orphanet Journal of Rare Diseases (Jan 2022)

Osteogenesis Imperfecta: characterization of fractures during pregnancy and post-partum

  • Eugénie Koumakis,
  • Valérie Cormier-Daire,
  • Azeddine Dellal,
  • Marc Debernardi,
  • Bernard Cortet,
  • Françoise Debiais,
  • Rose-Marie Javier,
  • Thierry Thomas,
  • Nadia Mehsen-Cetre,
  • Martine Cohen-Solal,
  • Elisabeth Fontanges,
  • Michel Laroche,
  • Valérie Porquet-Bordes,
  • Christian Marcelli,
  • Alexandra Benachi,
  • Karine Briot,
  • Christian Roux,
  • Catherine Cormier

DOI
https://doi.org/10.1186/s13023-021-02148-x
Journal volume & issue
Vol. 17, no. 1
pp. 1 – 12

Abstract

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Abstract Background Pregnancy and breastfeeding are associated with bone density loss. Fracture occurrence during pregnancy and post-partum, and its determinants, remain poorly known in Osteogenesis Imperfecta (OI). The aim of this study was to characterize fractures that occurred during pregnancy and post-partum in OI patients. Results We conducted a retrospective multicentric study including a total of 50 previously pregnant OI women from 10 Bone Centers in France. Among these patients, 12 (24%) patients experienced fractures during pregnancy or in the 6 months following delivery, and 38 (76%) did not experience any fracture. The most frequent localizations were: proximal femur (25%), spine (25%), distal femur (12.5%), and pelvis (12.5%). Fractures during pregnancy occurred during the third trimester and post-partum fractures occurred with a mean delay of 2 months following delivery. No fractures occurred during childbirth. We next compared the 12 patients with pregnancy or post-partum fractures with the 38 patients without fractures. Mean age at pregnancy was 32.7 ± 3.1 years-old in the fractured group, vs 29.3 ± 5.0 years-old in the non-fractured group (p = 0.002). Breastfeeding was reported in 85.7% of patients in the fractured group, vs 47.1% in the non-fractured group (p = 0.03). All patients with post-partum fractures were breastfeeding. Bone mineral density was significantly lower in patients with pregnancy-related fractures compared with other patients: spine Z-score − 2.9 ± 1.6DS vs − 1.5 ± 1.7DS (p = 0.03), and total hip Z-score − 2.0 ± 0.7DS vs − 0.5 ± 1.4DS (p = 0.04). At least one osteoporosis-inducing risk factor or disease other than OI was identified in 81.8% vs 58.6% of fractured vs non-fractured patients (not significant). Fracture during pregnancy or post-partum was not associated with the severity of OI. Bisphosphonates before pregnancy were reported in 16.7% and 21.1% of patients with pregnancy-related fractures and non-fractured patients, respectively (not significant). Conclusions OI management during pregnancy and post-partum should aim for optimal control of modifiable osteoporosis risk factors, particularly in patients with low BMD. Breastfeeding should be avoided.

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