Paget’s Disease of Long Bones: Microstructural Analyses of Historical Bone Samples

Abstract

Although Paget's disease of bone (PDB) is the second most common metabolic bone disease, there is only limited information about the microarchitecture of affected bones. Therefore, the aim of this study was to determine cortical and trabecular bone properties in clinically relevant locations by microcomputed tomography (µCT). Ten femora and ten tibiae affected by Paget's disease taken from the Natural History Museum Vienna were compared to 13 femora and 10 tibiae of non-affected body donors. Digitization of the cortical and trabecular bone microarchitecture was performed with an X-ray-based µCT scanner. Additionally, semi-quantitative gradings of trabecular and cortical architectural parameters of the femora and the tibiae were generated. Microcomputed tomography images showed changes in the thickness of cortices, cortical porosity, and trabecularization of cortical structures. Moreover, severe disorganization of trabecular structures, trabecular defects, and thickening of (remaining) trabeculae were detected. Numerical cortical analyses showed lower total bone volume (BV) and lower BV in the outer region (66-100%) (− 36%, p = 0.004, and − 50%, p < 0.001, respectively), lower total volume (TV) in the outer region (66-100%) (− 42%, p < 0.001), lower total bone volume fraction (BV/TV) and BV/TV in the outer region (66-100%) (− 23%, and − 12%, p < 0.001, respectively), higher BV and TV in the middle region (33-66%) and higher BV/ TV in the inner region (0-33%) (123%, p = 0.011, 147%, p = 0.010, and 33%, p = 0.025, respectively) in Pagetic compared to non-affected bones. Trabecular analyses showed higher BV/TV (96%, p = 0.008) and Tb.Th (43%, p = 0.004) in Pagetic compared to non-affected bones. There is a major and consistent structural alteration of PDB at cortical and trabecular sites in weight-bearing long bones. Our findings are relevant for the differential diagnosis of PDB and for the pathogenesis of associated complications, since the disorder produces abnormalities in the structure that might lead to bone fragility.

Microarchitecture of historic bone samples with tuberculosis

“…In Paget’s disease, similar as in tuberculosis, trabecular defects and cortical porosis were seen; nevertheless, in Paget’s disease cortical thickness was increased and no signs of ankylosis were detected [ 22 ], which is in contrast to tuberculosis.…”

“…The microstructure of the femora and tibiae with tuberculosis was compared to that of 10 femora and 10 tibiae from recent body donors provided by the Division of Anatomy, Medical University of Vienna used in a previous study [ 22 ]. The mounted vertebral columns presenting signs of tuberculosis also contained macroscopically unaffected vertebrae.…”

“…In addition to computed tomography, microcomputed tomography has also been successfully used for several years in paleopathology and evolutionary biology [ 18 – 21 ]. Among others, our research group previously used this approach for the analysis of historical samples affected by bone diseases such as Paget’s disease and osteomyelitis [ 22 , 23 ].…”

Micro-CT evaluation of historical human skulls presenting signs of syphilitic infection

“…Based on our previous experience [ 12 , 13 ], this study paid special attention to five alterations, i.e. cortical porosity, cortical thinning, cortical loss, sclerotic reorganization, and perforating defects.…”

The pathological anatomical collection of the Natural History Museum Vienna

“…Additionally, Pagetic bone is also characterized by hypertrophy and alteration of trabecular parameters. In a further study, Nebot Valenzuela et al (2019) [39] compared the microstructure of bones with and without Paget's disease using an X-ray-based µCT scanner. They could not only confirm the higher porosity at the microstructural level by the use of this approach, but also that the femoral heads and tibial condyles were thickened due to increased trabecularization-important findings of relevance for diagnostic purposes.…”