PAGET’S DISEASE ( Osteitis deformans) July 2012DR. FRANTZEN

DEFINITION Non-inflammatory condition of bone Furious bone resorption Chaotic bone deposition Sir John Paget (1876) July 2012DR. FRANTZEN

INCIDENCE Some 3% of European population ( 90% asymptomatic ) RSA : - 1.3% among black population - 2.4% among white population Prevalence increases with age (> 40) Male:Female ratio = 3:2 July 2012DR. FRANTZEN

Pathogenesis Slow virus infection : Paramyxovirus July 2012DR. FRANTZEN

CLINICAL FEATURES Initially asymptomatic Bone pain Stress fractures Increasing head size Blindness Deafness Bowing of legs Kyphosis High output cardiac failure July 2012DR. FRANTZEN

July 2012DR. FRANTZEN

PATHOLOGY 3 PHASES 1.Osteolytic or hot phase 2.Mixed or intermediate phase 3.Sclerotic or cool phase July 2012DR. FRANTZEN

SKELETAL DISTRIBUTION Virtually any bone can be affected Pelvis and tibia most common Next femur, skull, spine and clavicle July 2012DR. FRANTZEN

CLINICAL Asymptomatic in 70% Bone pain Limb feels warm Deformities Neurological symptoms High output heart failure July 2012DR. FRANTZEN

COMPLICATIONS Micro fractures Bowing of femurs and tibias Secondary OA of joints Malignant transformation Compression fractures of spine High output heart failure July 2012DR. FRANTZEN

DIAGNOSIS Clinical picture Radiographs S-ALP, u-DPD, u-Hydroxyproline Bone scan July 2012DR. FRANTZEN

RADIOLOGY OSTEOLYTIC PHASE Active bone resorption Large areas of radiolucency Destruction of cortex and cancellous bone July 2012DR. FRANTZEN

RADIOLOGY INTERMEDIATE PHASE Thickening of cortex and course trabeculation of cancellous bone Trabecular thickening along stress lines July 2012DR. FRANTZEN

RADIOLOGY SCLEROTIC PHASE Enlargement and widening of bone, marked cortical thickening with blurring of cortex-spongiosa demarcation July 2012DR. FRANTZEN

TREATMENT Treatment does not cure Pagets, but provides prolonged periods of remission July 2012DR. FRANTZEN

TREATMENT Pain relief – NSAIDS - Paracetamol - Opioids Pharmacotherapy - Biphosphonates - Calcitonin - Calcium 1000mg/d - vit D - exercise July 2012DR. FRANTZEN

TREATMENT Surgery indicated : 1.Unstable # and delayed union 2.OA for joint replacement 3.Osteotomy for deformities 4.Nerve compression in spine July 2012DR. FRANTZEN