Paget’s Disease

Paget’s Disease (osteitis deformans or osteodystrophia deformans): 

Paget’s Disease ( osteitis deformans or osteodystrophia deformans ) Rochelle Sansotta Kathaleen McCollum Stephanie Isaacson 2/7/2012

Definition: 

Definition Diffuse bone disorder characterized by: Increased rate of bone breakdown ( lytic activity) and deposition ( blastic activity) in one or multiple bones Enlarged, deformed bones

Phases: 

Phases Osteolytic Increased reabsorption leads to porous and weak bone Not often diagnosed in this stage 1 2. Mixed Lytic / Blastic reabsorption and disorganized deposition 3. Osteosclerotic Increased deposition causing dense bones

Demographics and Etiology: 

Demographics and Etiology Diagnosed in 4% of all persons over the age of 55 1 Depending on the source, males are affected equally 2 or twice as often as females 3 Rare in Africa and Asia 4 Most common in Caucasians in Europe 4 Possible genetic predisposition Possible association with a viral infection

Most Commonly Effected Areas: 

Most Commonly Effected Areas pelvis spine long bones of the extremities Skull * Changes due to Paget’s disease discovered frequently through imaging for unrelated pathologies

Signs and Symptoms: 

Signs and Symptoms Mild forms often go undiagnosed, until the following occurs: Bone pain Patient loses height Bowing of lower extremities Head becomes larger (hats do not fit) Weakness and sensory deficits

Complications: 

Complications Progressive deformities Degenerative arthritis of joints in the area Pathological fractures Osteogenic Sarcoma 1 Neurological complications including cauda equina syndrome

Prognosis : 

Prognosis Varies greatly; patients can be relatively stable or experience a rapid progression of symptoms Generally a slow progression Prognosis good if treatment occurs prior to major change in affected bones

Diagnosis: 

Diagnosis Most often diagnosed by traditional radiograph, may be confirmed by CT or bone scan 6 Bone appears thickened, porous Bone scan most efficient to establish extent in the skeleton, confirmed by radiograph 2 Biochemical markers used to confirm diagnosis

Functional Deficits: 

Functional Deficits Deficits dependent upon location and severity of pathology May include speech and hearing deficits with cranial involvement (compression of cranial nerves) Gait and postural deficits associated with arthritic changes and pathological fractures

Treatment: 

Treatment Treatment involves pain management, anti-inflammatory medications, hormonal therapy, mineral replacement therapy, physical therapy Treatment only slows progression, it is not a cure Surgical intervention for fractures, degenerative arthritis, severe deformity Osteotomy , arthroplasty in the most severe cases

Radiographic Quality: 

Radiographic Quality Due to increased bone density, attenuation of the beam increases. In order to get accurate density in the radiograph, increased exposure time is required 7.

Best Imaging: 

Best Imaging Definitive diagnosis occurs with conventional radiographs CT scans and MRI useful in the spine for assessing back pain 2 Bone scans show extent of involvement and bone metabolic activity; Dx confirmed with conventional radiographs

Conventional Radiograph: 

Conventional Radiograph Depicted to the R: A classic sign, the ivory vertebrae, seen at L3. Results from the increased sclerotic changes that occur with advanced Paget’s Disease. Biomechanical and Physical Findings: Decreased PIVM on mobility testing, decreased pain-free ROM, pt may present with postural abnormality . Question 1: What specific strengthening exercises could be implemented into the TherEx program of this patient to increase stability within the lumbar spine?

CT Scan: 

CT Scan Typical changes seen in the lumbar vertebrae in the patient with Paget’s in the mixed phase are depicted in this axial CT scan of the L3 vertebrae: Thickened, course trabeculae throughout the vertebral body. Lytic destruction throughout the vertebral body, extending into the pedicle, transverse processes, and spinous process. Increased radiodensity in the sclerotic areas of the laminae and pedicles. Biomechanical and Physical Findings: Decreased AROM, gait and postural deficits, increased s usceptibility to fracture Question 2: What are probable contraindications in the treatment a patient with LBP associated with Paget’s Disease?

References: 

References Salter R . Textbook of Disorders and Injuries of the Musculoskeletal System . 6 th ed. Baltimore, MD: Lippincott Williams & Williams; 1999. Lyles KW, Siris ES, Singer FR, & Meunier PJ. A Clinical Approach to Diagnosis and Management of Paget's Disease of Bone. J.Bone Miner. Res . 2001;16:1379-387. Atman RD. Beers MH. The Merck Manual of Medical Information. 2nd Home Edition. Whitehouse Station, N.J.: Merck Research Laboratories division of Merck & Co., Inc.; 2003. 246-348. Resnick D. Paget disease of bone: current status and a look back to 1943 and earlier. American Journal of Roentgeneology . 1988. 150: 249-256. Kline MJ. WebMD LLC. Paget ’s disease Imagining. Medscape Reference Drugs Diseases and Procedures website. 2011. Available at: http://emedicine.medscape.com/article/394165-overview . Accessed February 6, 2012. Singer FR. Evolution of the clinical presentation of paget’s disease of bone. Clinical Review in Bone and Mineral Metabolism . 1(2):95-98 . McKinnis L. Fundamentals of Musculoskeletal Imaging. 2nd ed. Philadelphia, PA: F.A. Davis Company; 2005.