These are the two Xrays  taken from the 65 year old male complaining of pain in the lower limbs and progressively deviating his limbs away from the midline.And he also noticed that his hat was quite tight for his head which was not previously noticed.Comment on the Xray and What is the Diagnosis?







ANSWER - PAGET'S DISEASE 

It is a chronic disorder that typically results in enlarged and deformed bone due to excessive and disorganised bone resorption and formation.Paget's disease is the second most common metabolic bone disease after osteoporosis.Men are more commonly affected than women.Onset is usually over age 50 years.Etiology is unknown but it is associated with certain viral infection(mealses) and has a genetic association also.

Pagetic bone is generally thickened, irregular and brittle
Cysts and stress fractures may be seen in both vertebrae and long bones
A tile-like mosaic of osteoid cement lines demarcate the old resorption cavities. This pattern is pathognomonic of Paget's disease of bone.(Top Xray shown this pattern)
Pelvis (72%),Lumbar spine (58%),Thoracic spine (45%),Skull (42%),Femur (55%) are very commonly affected.

CLINICAL FEATURES
Constant Bone pain
Limb shortening or bowing(Bottom Xray)
Chalkstick fractures
Curvature of spine(Kyphosis)
Loose teeth
Damage to joint cartilage - Arthritis
Increased head size
Skull thickening may also be associated with visual loss, facial palsy and trigeminal neuralgia
Hearing loss may occur when Paget's disease affects the skull
Drowsiness due to vascular steal syndrome of skull

INVESTIGATIONS

  • X-Ray
     Osteolytic stage(Earlier)
       Patchy areas of increased radiolucency
       Flame shaped areas of osteopenia
     Sclerotic phase(later) - enlarge and irregular bones with coarse trabeculation
  Fine crackseen in the Tibia and Femur

  • Isotope bone scans
  • Biochemical tests
   Increased plasma alkaline phosphatase
   Increased urinary hydroxyproline but no longer used as marker

MANEGEMENT

  • Bisphosphonates - Reduce Bone turnover
  • Calcitonin
  • Calcium Supplementation 1500 mg qd
  • Vitamin D 400 IU per day
  • Surgery is indicated if   there were pathological fractures,painful osteoarthritis,nerve entrapment or severe spinal stenosis
  • Low impact Exercise

Complications

  • Pathological fractures
  • Osteosarcoma
  • Osteoarthritis
  • Nephrolithiasis
  • Nerve compression syndromes

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3 comments

  1. Anonymous Says:
  2. Paget's disease.

     
  3. Anonymous Says:
  4. paget s disease

     
  5. Anonymous Says:
  6. pagets disease

     

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