Paget's Disease of the spine

1. Paget’s Disease of the Spine
Spine conference Friday February 5th, 2015
Upper Chesapeake Medical Center

2. 42 yo man cc: back pain HPI: slip and fall 1/8/16 seen at pat 1st then referred to ER due to L2
fx, CT obtained in ER Dx: fx PMH: very healthy PSH: none FH: bro passed away ca SH: manager
married 2 children nonsmoker Meds: none Exam 6’0” 187 lb nonfocal axial pain

9. HEMAGIOMA
Organized
Trabeculae orientation
Maximized along stress
Areasprominent trabecular pattern
sclerotic vertebra with vertical
trabeculae: Corduroy sign
lytic calvarial lesions with spoke-
wheel appearance
irregular and lytic in long bones,
with a honeycomb appearance
Jail house vertebra

11. Sir James Paget
1814-1899
St Bartholomew Hospital London
British Surgeon and Pathologist
Breast Cancer description, inflammation of
nipple
osteitis deformans.
As a medical student in his first winter session, he
discovered the pathogen for trichinosis, a parasitic disease caused
by Trichinella spiralis, a minute roundworm that infests
the musclesof the human body, and which is usually acquired by
eating infected pork. Paget was friends with Charles Darwin and
Thomas Henry Huxley

12. Prof. Stuart Ralston - Solving the Mysteries of Paget's Disease
The University of Edinburgh 2011
https://youtu.be/9mogcgQqbxg

13. Uncoupling of the normal equilibrium
of bone formation and resorption

16. plywood

17. Osteon
Haversean canal
Osteocytes
Lamellar bone
Contiguous plates

18. Cutting cones

23. Negative CT chest/abd/pelvis
Metastatic w/u

28. L2 L2L3
L3
L3L4

36. epidemiology7-10x more
1970’s England highest rates
Rates now decreased by 50% in general; rare <50
yo; mean 59 +/- 14;
Polyostotic>monostotic; pelvis,lumbar,femur,
tspine, skull, tibia rare in hands/feet

37. 1. Mixed lucent and dense areas
2. ENLARGES the bone itself
3. loss of junction of cortical and
cancellous areas
4. deformity

39. Blade of grass
Osteoclastic phase
Long bone v-shaped
lesion

40. Mixed stage lysis
and sclerosis

42. Sclerotic phase
Enlarged bones

44. PAGETS DISEASE OF BONE

45. Intranuclear inclusion
bodies in an electron
micrograph 9,000
magnification

46. Paramyxovirus (mumps) causes intranuclear inclusion bodies

49. 1995-2008

50. BISPHOPHONATES
• Nitrogen-containing bisphosphonates (N-BPs)
1. Alendronate Fosamax (Merck, Whitehouse Station, New Jersey)
2. Ibandronate Boniva (Roche, San Francisco, California)
3. Pamidronate Aredia (Novartis, East Hanover, New Jersey)
4. Risedronate Actonel (Warner Chilcott, Rockaway, New Jersey)
5. Zoledronate Reclast (Novartis, East Hanover, New Jersey)
• Non-nitrogen-containing bisphosphonates (n-BPs)
1. Etidronate Didronel (Proctor and Gamble, Cincinnati, Ohio)
2. Tiludronate Skelid (Sanofi Aventis, Bridgewater, New Jersey

54. Femur fracture in
women
• Shane E (May 2010). "Evolving data
about subtrochanteric fractures and
bisphosphonates". N. Engl. J. Med.
362 (19): 1825–7
(12 in 14,195 women)
compared to the common hip
fractures (272 in 14,195
women)
Odvina CV, Zerwekh JE, Rao DS, et al.
Severely suppressed bone turnover: a
potential complication of alendronate
therapy. J Clin Endocrinol Metab
2005;90:1294–1301

55. Malignant
transformation
of paget’s in this case
osteosarcoma

56. Thanks