Pseudosarcoma among the patients with osteogenesis imperfecta: differential diagnosis and features of clinical and radiological course
Introduction. Osteogenesis imperfecta (OI) besides characteristic orthopedic appearance (pathologic bone fractures and bone axial deformities) is associated with such a specific complication as pseudosarcoma which is important in theoretical and practical differential diagnostic aspect.
Goal. Improvement of diagnostics of pseudosarcoma in osteogenesis imperfecta, studying its clinical and radiological features at the different stages as well as differential diagnosis with some true malignant osseous neoplasms.
Materials and methods. Study was conducted with analysis of clinical and radiological picture among 7 patients with osteogenesis imperfecta who have been treated in the state institution «Institute of traumatology and orthopedics of the Ukrainian national academy of medical sciences» (Kyiv, Ukraine) during 1978-2013 and have been diagnosed with pseudosarcoma (age range 2,5-14 years, follow-up duration — 7 years). Among 5 patients pseudosarcoma affected only one bone, among 2 ones multiple pseudosarcomatosis was observed. Pseudosarcoma at the 1st stage was diagnosed in 3 patients, at the 2nd stage — in 2 patients, at the 3rd stage — in 2 patients. Femur was affected in 5 patients, tibia — in 1 patient, metatarsal and calcaneal bones — in 1 patient. Aimed at the differential diagnosis we have analyzed the X-ray images of 3 patients with malignant osseous neoplasms.
Results and discussion. We have studied the features of clinical and radiological manifestations of pseudosarcoma among 7 patients with osteogenesis imperfecta and distributed the course of the mentioned complication into 3 stages — progression, separation and regression. At the first stage clinically we observed the signs typical for malignant neoplasms — progressive pain; dense swelling, soldered to bone and painful during palpation, radiologically at the level of fracture and at some distance from it increase of soft tissues’ volume, as well as periosteal strata and paraosseal area of calcification were evident. At the second stage swelling diminished, pain vanished and skin assumed its natural appearance, radiologically we noted ossification of periosteal strata, clear separation at the periphery of paraosseal calcifications. All paraosseal complex slightly enlarged comparing to the previous X-ray images and assumed «lobulated» structure. At the third stage clinical symptoms disappeared except slight increase of affected limb segment’s volume, radiologically paraosseal calcifications reduced, underwent ossification, became more homogenous and merge into bone. We performed study and comparison of the clinical and radiological course at the 1st and 2nd stage of pseudosarcoma and that of the true malignant osseous neoplasms — osteogenic and paraosseal sarcomas.
Conclusion. In our opinion study of the clinical and radiological evolution of pseudosarcoma contributes to improvement of diagnostics in this complication of osteogenesis imperfecta and clear differentiation in similar nosologies. This would help to avoid diagnostic mistakes and unjustified surgical interventions among patients with osteogenesis imperfecta.
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