Paget’s Disease of the Spine

Spine conference Friday February 5th, 2015

Upper Chesapeake Medical Center

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

HEMAGIOMAOrganizedTrabeculae orientationMaximized along stressAreasprominent trabecular patternsclerotic vertebra with vertical trabeculae: Corduroy signlytic calvarial lesions with spoke-wheel appearanceirregular and lytic in long bones, with a honeycomb appearanceJail house vertebra

Sir James Paget 1814-1899St Bartholomew Hospital LondonBritish Surgeon and PathologistBreast Cancer description, inflammation of nippleosteitis 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

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

Uncoupling of the normal equilibrium of bone formation and resorption

plywood

OsteonHaversean canalOsteocytesLamellar boneContiguous plates

Cutting cones

Negative CT chest/abd/pelvisMetastatic w/u

L2 L2L3

L3L3L4

epidemiology7-10x more

1970’s England highest ratesRates now decreased by 50% in general; rare <50 yo; mean 59 +/- 14;Polyostotic>monostotic; pelvis,lumbar,femur, tspine, skull, tibia rare in hands/feet

1. Mixed lucent and dense areas2. ENLARGES the bone itself3. loss of junction of cortical and cancellous areas4. deformity

Blade of grassOsteoclastic phaseLong bone v-shaped lesion

Mixed stage lysisand sclerosis

Sclerotic phaseEnlarged bones

PAGETS DISEASE OF BONE

Intranuclear inclusion bodies in an electron micrograph 9,000 magnification

Paramyxovirus (mumps) causes intranuclear inclusion bodies

1995-2008

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

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: apotential complication of alendronate therapy. J Clin Endocrinol Metab2005;90:1294–1301

Malignant transformationof paget’s in this case osteosarcoma

Thanks