Update on bone fragility in spina bifida.

BACKGROUND CONTEXT: Patients with spina bifida (SB) are at risk for pathological fractures and low bone mineral density (BMD).

PURPOSE AND

METHODS

This article reviews the literature and provides a comprehensive overview of how the characteristics of SB and its associated comorbidities intersect with bone fragility to identify possible pathophysiological mechanisms of fractures and low BMD.

RESULTS

Bone fragility occurs early in the life of patients with SB as a result of a disturbance that determines changes in bone shape, quantity, and quality, as poor mineralization reduces bone stiffness. Bone fragility in SB occurs due to local and systemic factors and may be considered a state of impaired bone quality of multifactorial aetiology, with complex interacting influences of neurological, metabolic, and endocrinological origins and the presence of smaller bones. Bone fragility should be evaluated globally according to skeletal age and Tanner staging. The phases of the evolution of Charcot joints seem to intercept the evolution of epiphyseal fractures. Charcot arthropathy in SB may be initiated by the occurrence of repetitive trauma and fractures in epiphyseal and subepiphyseal regions, where there is a deficit of bone mineralization and greater bone mass deficits.

CONCLUSION

Bone fragility in MMC potentially has a multifactorial neuro-endocrinological-metabolic-renal dimension, with smaller bones, lower bone mass, and mineralization deficits affecting bone strength.