pagets disease of bone

Physiology and Extra info Osteodystrophy->disorganized formation and remodeling of bone unrelated to functional requirements Abnormal osteoclastic function Radiographic features Pathology Variable : reflect diff phases Microscopic : Earliest phase : osteoporosis Increased bone turnover Patchy osteoclerosis Bone resorbtion and deposition reflects Ill-defined n irregular the disease stages. radiopaque area cotton wool resorbed area filled by cellular and Aetiology : unknown Geographic diff : appearace vascular fibrous marrow within which new Maybe paramyxovirus inf. (measles / > in UK, Australia, North America Skull : bone formsremodelled and replaced by resp. syncytial virus) -> latency in Rare in Asia, Russia and parts of Europe -thickening of outer table of the further new bone. osteoclast progenitor cells vault fully dev. active lesionsimultaneous Genetic predisposition(chr. 18q locus) Lesion : -loss of distinction between the osteoclastic and osteoblastic activity Single or small # of bones tables and diploe bone trabeculaenumerous criss-crossing, Pathogenesis : Disseminated widely throughout the skeleton Jaw : resting and scalloped reversal line (stain (progressive and overlapping phases) (less common) -loss of lamina dura deeply by haematoxylin) Predominantly osteolytic phase Commonest-Weight bearing bones of axial -hypercementosis criss-crossing linemosaic appearance -increased osteoclastic resorption skeleton (sacrum, lumbar, thoracic n cervical -ankylosis reversal linejunction of reversal of Active stage of mixed osteolysis and vertebra) osteoclastic resoption to osteoblastic osteogenesis Skull and femur deposition -abnormal osteoblastic deposition and Jaw- > maxilla scalloped outlinemargin of previously increased vascularity existing howships lacunae Predominantly osteoblastic / sclerotic Gradual onset -> chronic course disjointed trabeculae fusedense phase May be symptomless and an incidental sclerotic masses of mosaic bone -dense bones and decreased radiograph finding less active disease: vascularity -less vascular marrow Clinically : -decreased osteoclastic and osteoblastic st ->1 two phases->bone softened and Varying degree of bony deformity (most activity distorted,overall size markedly cases are mild) + bone pain *Treatment and prognosis : Jaw : enlarged acellular globular increase Increased pathologic fracture Calcitonin and diphosphonate depositsfuse to form dense sclerotic ->sclerotic phase->bone become fixed Enlargement of skull and facial bones inhibit osteoclastic resorption masses in deformed state Narrowing of skull foramina->sensory and Not fatal Cementumhypercementosis fusing with motor disturbances (cranial nerve Increased of malignant alveolar bone->ankylosis compression) neoplasma (<1% of Increasing facial deformity patients)osteosarcoma (most Osteosarcoma/other bone tumorsrare Progressive enlargement of maxilla common. complication (mainly in pt with wide spread Alveolar ridge thickened and widenened disease) Age : > 40 years > common with increasing age 3% overall incidence Clinical Features

 Palate flattened Incompetence of lips Hypercementosis n ankylosed of teeth (difficulty in extraction) Dentate patient : -derangement of occlusion -spacing of teeth -retroclination of incisors -palatoversion of post teeth Edentulous pt : -difficulties in wearing dentures (need to be made periodically) Phases : Osteolytic phase : root resorption Active stage : postextraction hemorrhage (highly vascular marrow) Sclerotic phase : dense and avascular bone, extraction sockets are prone to inf.

Laboratory : Increased AP (markedly in widespread active disease) Serum calcium and phosphorus within normal limit