Diagnosis: Osteogenesis imperfectaDescription: Severly deformed femur with a thin and bent shaftAddtitional findings: Death due to right heart failure in a patient with cor pulmonale. Multiple costal fractures, partly not recent, with severe deformation of the thorax and the whole skeleton. Generalised osteopenia. Kyphoscoliosis. Multiple bone marrow embolisms in the lung.Clinical examination: 39-year-old male patient, brittle bone disease, pulmonary hypertensionComment: The impaired bone quality leads to an augmented fragility and augmented tendancy of bone deformation. The lower extremities are preferrably affected. Due to the fractures and leg malposition, the majority of patients cannot walk or stand.(Source: ©PathoPic)
