Thursday, January 3, 2008

26 - rugger jersey spine

Renal Osteodystrophy

· Constellation of musculoskeletal abnormalities occurring with chronic renal failure featuring some combination of

o Osteomalacia (adults)

o Rickets (children)

o 2° hyperparathyroidism

o Soft-tissue calcifications

o Osteosclerosis

o Soft-tissue + vascular calcifications

· Low calcium levels lead to osteomalacia

o Additional factors responsible for osteomalacia are

§ Inhibitors to calcification produced in the uremic state

§ Aluminum toxicity

§ Dysfunction of hepatic enzyme system A

§ Renal insufficiency with diminished filtration results in phosphate retention

· Maintenance of Ca x P product lowers serum calcium directly, which in turn increases parathyroid hormone production (2°hyperparathyroidism)

· Osteopenia

o Combined effect of

§ Osteomalacia (reduced bone mineralization due to acquired insensitivity to vitamin D / antivitamin D factor)

§ Osteitis fibrosa cystica (bone resorption)

§ Osteoporosis (decrease in bone quantity)

o Complications

§ Fracture predisposition (lessened structural strength) with minor trauma

· Spontaneously

§ Fracture prevalence increases with duration of hemodialysis + remains unchanged after renal transplantation

· Sites of fractures

o Vertebral body (3-25%)

o Pubic ramus, rib (5-25%)

o Milkman fracture / Looser zones (in 1%)

o Metaphyseal fractures

o Prognosis

§ Osteopenia may remain unchanged / worsen after renal transplantation + during hemodialysis

· Secondary hyperparathyroidism

o Cause

§ Inability of kidneys to adequately excrete phosphate leads to hyperplasia of parathyroid chief cells (2° hyperparathyroidism)

§ Excess parathyroid hormone affects the development of osteoclasts, osteoblasts, osteocytes

o Hyperphosphatemia

o Hypocalcemia

o Increased PTH levels

o Subperiosteal, cortical, subchondral, trabecular, endosteal, subligamentous bone resorption

o Osteoclastoma = brown tumor = osteitis fibrosa cystica (due to parathyroid hormone -stimulated osteoclastic activity

§ More common in 1° hyperparathyroidism

o Periosteal new-bone formation (8-25%)

o Chondrocalcinosis

§ More common in 1° hyperparathyroidism)

· Osteosclerosis (9-34%)

o One of the most common radiologic manifestations

§ Most common with chronic glomerulonephritis

o May be the sole manifestation of renal osteodystrophy

o Diffuse chalky density

o Thoracolumbar spine in 60% with dense end-plates produce appearance of rugger-jersey (rugger jersey spine)

Rugger-jersey spine in

Renal osteodystrophy

o Also in pelvis, ribs, long bones, facial bones, base of skull (children)

o Prognosis

§ May increase/regress after renal transplantation

· Soft-tissue calcifications

o Metastatic secondary to hyperphosphatemia (solubility product for calcium + phosphate exceeds 60-75 mg/dL in extracellular fluid)

§ Hypercalcemia

§ Alkalosis with precipitation of calcium salts

o Dystrophic secondary to local tissue injury

§ Location

· Arterial (27-83%)

o In medial + intimal elastic tissue

§ Dorsalis pedis a., forearm, hand, wrist, leg

o Pipestem appearance without prominent luminal involvement

· Periarticular (0-52%)

o Multifocal

o Frequently symmetric

o May extend into adjacent joint

o Chalky fluid / pastelike material

o Inflammatory response in surrounding tenosynovial tissue

o Discrete cloudlike dense areas

§ Fluid-fluid level in tumoral calcinosis

o Prognosis

§ Often regresses with treatment

· Treatment

o Decrease of phosphorus absorption in bowel

o Vitamin D3 administration (if vitamin D resistance predominates)

o Parathyroidectomy for 3° hyperparathyroidism (= autonomous hyperparathyroidism)



the picture below shows how a rugger jersey looks like-- with alternating bands .



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