4' sensation - &termine if sensorl'loss is localized to a single &rmatome or socking-glove distrihrion

a. pogerior columns-Vibrarory-2 -poinr discrimination-Position sense

b. Anterior spfuntbalamic-Lighr rouch

c. I_areral spinothalamic-Fain and tempexature

5. Reflexes

a Deeprcndon1,4 - Fatellar ren&nSl - Achiltes rendonC5 - Biceps tendonC6 - Brachioradialis rendonC7 - Triceps tendon

Grading ofDTR's

+0 - absent+l - hlpoactive+2 - normal

+3 - hyperactive+4 - pathologrcally brisk / clonu

5 1

b. Pathological reflexes - assesses UMN $sfirnaion

Babinski - not presenl - planurflexon of toes- nesenr - donillexion sf hqllu)i and faruring of lesser toes'

6. Cerebellar rests

a. Rhomberyb. Reboundc. RapidlY alternating movementsd Heel to shine. Tan&m walking (heel to toe)

7. Orthopedic lo$'er $itremJt)' elzrm

a. assess &formities Present- mil4 moderate, or sever€- flotible or rigid- locarion - rearfooL mrdfoot" forefoot or combination of deformities- compensrory mechanisms Present- eraluate rveightbearing and non-weightbearing- gait analysis

-sensor), ataxic - demyelinating peripheral neuropathies, spinocerebellardegeneratior; Vit Bl2 deficiency- M-S.-rrlppug" - peripheral or spinal motor nerve lesions. polio- CMT- progressive

muscular auoPhY-m1'opathic - Muscrnar dlstrophl'and other myopthies-cerebellar ataric - cerebellar lesionsoilmF]'ranidal - Paftinson'soputtia - CVA tumor, n"um4 corticospirul lesion' M'S', C'P'-apractic - Alzleimer's norrral PrEssure h1'drocephalus-hlsterical

8. Labs and SPeoal Studies

rnal.include Cpt! aldolase, EMG/NCV, muscle and nerre biopsies, lumbar Fmcture, CT

scan, MRl, X-rays and other procedrres

lII. Dflerentiation on Upper Motor Neuron GnnD and l,o$'er Motor Neuron (Ii/t]rl) d1'sfirn6ionUMN LMN

Voluntal-v conuolToneReflex arcFathologic reflexesArrophv

lostspstic

IostIlaccidabsenlnol pr€senlsignificail

preseflpresemltde or none

52

IV. Neurologic Diseases affeaing lhe loner exnemrties

---_):talru3-FOot -?solo-of -pailents-u'i& a pes ca\us defornriq'lill-have'an-underlfingneurologc,

problem (Brewerton et al.)Congeniul causes:

CBARCOT-MARIDTOOTE DISEASE @eroneal muscular atrophy)-most colrunon neuromuscular disorder resulting in carrus foot. Srmmericherediurl' molor and sensor)/ neuropatbl' thal is progressve.-3 modes of inheritancel. Autosomal domirunt- aflecs 30/100.000 and is slowh'progressive2. X-iinked recessive- affeas 3.6/100.000 and is more severe, onset dmng

adolescence3. Aulosomal recessive- affeas I.4/100.000, is npidly progressive and scverell'

disabling- fenrales > males 3-5:1. Seen pnmarily in Caucasans

deformities--c:r\41s, czlvo\znrs, equinocavo\anrs due to overpou,ering ofmuscles. Rigid or Flexible

CHARCOT-MARIE-TOOTE DISEASE (conr)- PL > TAprodrces a plantarllexed medial column- TP > PB produces a rearfoot rrans

{onuacnrre of plantar frscia increases cavus-FDL > EDL and intrinsics leads to claunoes- generalized LE arophy lcads to the dev'elopment of ,,$ork leg" or"invened champagrre botd e" deformir-v-.

-presenr u'irh high $eppage or marionene gail-progressive anophy of distal UE leads to "monke\' fist" or "skeleton hand"deforrnity.-Diminished sensation in socking-glove disrr'hrtion-NCV are dela-ved b1' approximately SV/o-Diagnosis based on positive frmily hisory and clinical finding. Recendvarailable is a blood 1.r1 1s r+iegnose the Tlpe I autosornal dominant forn

DLIERTNE-sorrAs DISEAE (rlrperrrophic intersritial pollneuropathy)-autosomal recessive-progressive disease g'ith onset in childhood to earll'nrirlt16,sddistal u'ealoess and sensory loss of primarily tre lou'er exrremities.-peripheral nencs are enlaryed and fiequentll' plpble dre ro "onion bulb"formation of the nerves-nerve biopq'revea.ls concentric proliferation of Schwann cells around an area ofdemyelination

FREIDRICE'S ATA)qA-recessivel-v" inherited disease-idiopathic degeneration of the poserior and laleral columns of the qpinal cord-onser in lrre ghilclhood and early adolescence, rapidll,-rvealmess oflower exremities, anterior and lateral comfrarntenls. I arer uFgrex:remtwwealqess, pnmarill' ulnar newe distn'brtion.-sodcing-glove sensory loss.<erebellar involvemenl nith incoordinatjoL n]st:tgmrrs, {'sarr}uic speectr- auxia-no lab abnormality is diagrrostic how€r.er. senson'aaion potentials are absem ormarkedll'decreased NCV rernain normal or d€cr€zrse slightll'udth disease progression

53

ROUSSY-LE\ry SYNDROME-aulosomal dominant inherited disease+lowlr'-progressive<orolnon n"oi"gt include resdng Eemor, clumsl' Fir, atrophy of the disal LE's

and UE's, abnormal equilibriurq and absent DTR's'

REFSfIM'S DISEASE (Heredopathia arzfrapolyneuritifomds)-autosomal recessive, hyperrrophic sensory-motor polyneuropatlry-meEblic neuropathy caused bV a disturbance rn lipid meubolism $'hich leads to

an accumulauon of Pbltanic acid-males = females-perids of aqtre eracerbtiors-ietiniUs pigmentos4 a1axi4 ichthyosis- deafrress, disral extremiry u'ealcress with

gatr disubanscs. diminished DTR's.-serum phYranic acid levels are >50olo abol'e nonnal'

SPINAL DYSRAPEISM-failure of mesoderm and neuroecloderm to fuse and form the neural tube-Types:

l. Spina bi6da- lack of closure of vertebral archeg primalih'lumbr

2. Spina bifida occr:lta- nol accomparded b' protrusion of nreningeal or

*-o".*,

3. Spim bifida c-rstica- prorusion of meninges

4. Meningoradiculocele- protrusion of meninges aDd nen'e roots

5- Meningoml'elocele- pmtnrsion of meninges and spinal cord

Jeg wea}cness with fi:et in equinovarus positiondiminished DTR, sensory loss feet and saddle area-rnay be associated u'ilh h1'droce'phalus-associared $ith congeniul abnormalities and mental reurdation

CEREBRAL PALSY@esion acguired in urero, duing &livery or monlhs aflerbirth.

<ommonlv due to tra|ntra asphyxia or prerr'ature birt]t-irrwersible, non-progressive brain ddormity+lassification:

-Spastic (657dl. Spastic diplegn- B/L LE $pasticit-v- scissoring gait- rariable menlal

inPainnent2. Spasuc quadriplegia- severe BIL UE and LE spsicity' severe

inellecnul iryaiment3. SpaSic hemiplegia- unilateral spastic hemiparesis. less severe menul

imPairment-Athetoid (20'/A-Ataric-Rigld-Tremor-Atonic

54

-trearrnent of CP-Physical therapl-S4idiilEFr-entrbFtrl-Mude relaxants

MUSCT]I.AR DYSTROPEY-famiiial disorders tlrat lead to degeneration of mrxcle fiben. due to erzlmatic defeo

-T1pes:l. Duchennd Pseudohlperrophic - most cornmon ser-linked inheritedm1'opathl', diaposed dring earll' childhood d.re to slon' motor&velopment 80o/o shou' pseudohlpertrophl' of calf musculatue due tofat deposition uhich can be seen on biopq'. Girdle wealcress lea.l< to theclasslc

"Gou'e/s sign" utilized n'hen going from the- floor to san.+in& Ankle eguinus and

equinovarus deformities are cornmonProgressive and the majoritv of patiens areu'heelchair bound by age 20. Elevarcd CPKIgvels are noted

2. Becker N{-D. - similar pagern of muscle rv'ealirress as Duchemehowever, less aggressive. Fatients with Becker M.D. oflen ualk toappron-uratell'age 35 and have loqger life Tpn<. '3. Facioscaprlohumeral NtD. - mildll' pnogressive, affeos frcial andshoulder muscles with weaJgress and arophy. Lor*'er extrernit' u'ealcnessusually occurs 2G30 1'ean larer.4. Myotonic MD. - multisystemic disordcr, onset mnges from bnh !o 50years of age. hesents with tonic musde spasms. Oflen a fmt dropdevelop.5. Limb girdle M-D. - diaposis of exdusion, he< inconsisent features.Initial pesenution of slroulder and pelvic girdle u'ealgrcss and anophy-

SYRINGOMYEIIA-progressil'e disorder, onsa usual\' 2nd-3rd decade-characterized bv muscle alrophy. dissociatedanestbesi4 uith neurotrophic changes inclu.ting neurogenicoseoarthropathy (Charcot), loss of DTRcused b1' a hlhilar dilrtation in the spinal cord most cornmonly in the cen'icaldiagncis ea<ily rnade with MRI or CT

2. Periphenl neuropathy ( COMMON CAUSES )-Hereditarv

i-lrff -Tangieds disease-Dejerine-Souas -Fa@s dis€ase-Refsum's dis€ase -Abetalipcproteinesua-Amfloidosis -Freidrich's araiia

-Acquir€d-Dabetic -Hlpothyoidism-Myeloma -Sarcoidosis{uillain-Barre' -Alcoholic-lrCalignanq' {ollagen-rascular-Uremia -Acromega\{ryoglobulinemia -ViuminB-Hry

5 5

oxi c{ie drugs-chemicals-ctrloranphenicol-isoniazid-niuofurantorn-Dapsone<rspluin-megOnirtezOle

-hydralazine-Phen1'toin-leadq'anide-thallium-zeil

-InfectiousJeprosr_diphtheria-HIV

-Entrapment-carpal and tarsal tunnd

3. Various neurologic diseaset

I. PARKINSON'SDISEASE-progressive disorder, charaaerized b1' qroplasrnic-eosinophilic inclusions in theneurons of the substantia nigra.-chamaerized b' rigditl', resing bemor, bra$icinesi4 loss of postumt refloies.festimting gail Dementia occurs n 50%oof patients.-malerfemales, onset 4O60 yean of age-multipe drugs rrtilized in neatrnenr including levodop- carbidopa- amantadine.bromocripine, pergolide. Ph1'sical therap' helpful to improvefunction

2. MIJLTIPLE SCLEROSIS-chronic CNS disease of unlcnonn origin<haracterized by irdlammatory demvelinating plagues in w'hite nutler of CNS.lnfrltration of lyrnphocytes, plasma cells and rncrophages.flset at arormd 30,2:l female:rnale-course is rclapsing and retrdttingopfic neuritis is most corrmon presenting symFom-LE affeaed> UE oflen with spasiciry* and f:tigue-cerebellar irrvolvement u'ith auxi4 scaffring dysartlria and intention tremor-autonomic s1'rnFoms- incontinence, impotence and or,tlosutic hlpotension.-oflen see penonaliq'changes, memory loss, dementia and emotional labilitl'.-MRI diagnostic in >90olo of cases-mean sun'iral approL 25ys and is improving-avoid ph)E cal and emotional stress, infections and prolonged er?o$re toe)cremes of temperaure.

3 CEREBRAL VASCI.]I-AR ACCIDENT-rapid orsa of a neurologic deficit irn'ol'ring a rascular rerriorl' of the brain andlasting for grearer than 24 hours. < 24 hours considered a transient ischemic anack(TrA).-mo$ oflen dre to thrombosis or embolic evenl as rvell as hemorrhaee fiomaneunsm or AV malformation.

-lithium-pryridoxinedisuhram+thionamide-vincristine-gold-amiodarone<olchicrne-arsenic-mercury'-nichloroethyleneddr

-herpes zoster-L1'rne disease

56

-prcsenlation raries uith the vessel inrolved and portion of the brain infrrrcted4T scan diagnostically helpfuI----*--------:Gercdly.

ezrly-Tkrcid-paralysis--followed-[-increasinB-spasticin'--arrddevelopment of rynergistic patrems.-Ph1'sical thenp5'and ort}otic fabrication can improve firnaional abilig'

4. EPILEPSY<haraaerized b1' abnormal, recurenl. excessive di scharges from newons.-5(P/o have no underllmg neurologrc disorder-other etiologies include E1OH nithdraual, head rauna blpoglrcemi4 meningltis.encephalitis-most seizures last 3G90 seconds{lassification

l.Focal (Fanial local)- originare from a localized ponion of the brain-Simple focal

- -motor- focal motorwith march Jacksonian-sensorlL somatosensor5', r'isualaudito4', gust:ttorJ'-autonomic- eprgastric sensation, Fllor,sweating flushingpiloereaioa ppillan' rtileurtien-pqychic- dvsplusia affertive, cognitir-e, illusions.hallucinatiors.

{omplex focal-simple focal follon'ed bf imparnnent of conscioumess-imFired conscioumess al onset sometimes qilh automations.

-An-v- focal seizue may evolve lo a secondan,generalized seizure.

2.Generatzed - no focal componenllherefore no prodrome. aura- or focalmolor or sensorJ' qTnFoms.-Absence (Perir }v1al)

-impirment of consciousness only, ma-v show- mildcl onigaronic,tonic component

-Myoclonic seizures-Tonic-Tonic{lonic (Grand rnel)-Atonia (astatic) seizurc

3.Unclassified

-Stan's epl@cns<ondition in u'hich patient has a senes seizures witlrout tolal recovery. ofcorscioumess. Most conrmon cause in compliance with seizuremedications.

-Anticomulsants (ie. phenl'toi:r, calbamazepine) are most commonl5' utilizedNeed to

ohain therapeuic blood levels.

5. MYASTITFNIA GRAVIS-Acet1'lcboline (Ach) recepor anu'bodies desrq' the recepor sites on lhepostqnap.ic memtrane. Resulrs in miniature end plate potentials Orat are r€dlcedin size andnumber.-Due to an auoimmuropathv-Ilallmark is exercise indlced htigue thrt i5 rcsoh'ed nith rest-{flen get double rision and trosis, rlifficulrv- nith ches.ing Onh'invoh'es skeletalmuscle.-Ach receporantibo{'formd in the senmr of >9U/"

) /

-Tensilon (edrophoniurn) is helpful diagnosticalt"-EMG shows classic changes of decrement-S7o-of-patienS,trave-anassociated-fhynoma-ry\ichjs-rnost-easilr'-diagnosed-withCT scan--Trearments include long rcrm anticholineserase meds or disease modi!'ing drugs

such as lmmuran Also plasraphoresis has been found to be effecuve'

MG (cont)- *** gslsn-Iambert s-wdrome is similar to m1'asthenia gral'is hoqever- il im'ohes

a defect tn treuromuscular transmission due to a decreased number of ACh packets

released al lhe pes-maptic rernitral.- use cenain meds such as aminoglycosides and tetraq'cline gith caution

6. G UILLAIN-BARRE' S II\JDROME (Ao.rte idi opathic pollneuritis)-immunologically mediated demyelinating polyneuropathf'-affeos all ages and sexes

- - $rrrr@.l-peaks-� oJoccurrence: 15-35 )rs,and 5G75lrs-s5rralll,preceOeO U1'a t,iral or m1'coplasna infection, sul.gen'. immunization or

rn patienL u'ith q'semic lupus er-rthematosis (SI-E) and lynphomas'

<hancteiuef� q' p6av progressog svmmerical motor wea]<ness in a distal to

pmxirnal fashion Cao toa to severc respiraro4'paralvsis and occasionallY cranial

nerr€ inv'olv'ement-sruses socking-glove paresthesiasdecreased or absenl DTR's-nlay €use auronomic dysfirnaion includingtra$'cardialtachl'cardiahyporensi on/hYPertensiondiagrrosis- ru#tar prncrure reveals elerated CSF prorein and the prcsence of a feu'

mononucls[ leukocYtes.-EMG rweals decreased NCV and prolonged latencies-finctional reoovery ocorrs usually 24 q'eels

ItITIIIIItI

tltTIIITI

BONE TUI\IORS

coBr tclL FttSous D\Tq"{s;ADA/|UMlXurt

tolx! gat lEsrft -

'_ ttlm l.rgcnrlfr|cuJ.n€&! l Eiln$I8.0t$

fltnon Dt:ru3u

lllms tcDr

f, ttEqr5 coFTrc!. ltFEat:nM5D?ltGtlStri L

clA$t gf LilrlrClrll: rEIIFllllEl(

tD('tof

5 8

-CltGfidonj tlsloloeidlylffil on the a=ll or-rime-tj-F-fiom-s'liiehthEl-on-origindes. goe6ii'

CardJaginous and Fibrous are lhe origrn of mos pnmarl' bet igr and maligrranr neoplasms in bone.

Diasrosis:Subj ecti ve qmp oms(NLDOC AT)Pas medical hisoryCiinical .yalrrqrion of objeaive signs

Radi o ganhi c evaluati on :Helps to derermine the rate and aggressiveness of the bonetumor

2.

Panern of bone desruction:a) Geographic - Ieas aggressive, well definedb) ]uloth e aten -more aggssivq less defined- -'c) Permeative - higtrb,aggressive

Sclerotic border:-slou'I1' gron'ing lesions are associatedbone

n'ith reactite sclerosis of the surrounding

-sclerosis can be of rariable thiclsress and paniall5, or completell, srround thelesion

Cortical irrvolvement:$one cortex sen'es as a barrier to rarerar erynsion of the tumor-nonaggressive mefullarJ'lesioru ru)' cause little change in the endosteal surface,

sorue benign lesionssuch as aneul'smal bone qrns can expand rapldl),

-aggressive lesioru cause endosleal erosion and can penetrate the cortes in moret.han one place

4. Perioseal reaction:l. Corniauous

a. Corteri destroved- smooth shell - due to expansile pressurE benigr lesiorx (giant cell,

enchon&oma, fi brous dl,splasa)- lobulated shell _ dre to rzriation of grou,th rarc- ndged sheil - AKA "soap hrbbre" dre to slouing rate of

proliferation (non-ossi$,ing fibronq enchondronu) -

b. Cortex pres€nt- solid 'implies slou' growth rate. can be seen in osteoid osteonra-

encbondroma and eosinophilic granuloma' undurating - rariant of soli{ seen *ith periosrius, hlperrophic

oseoarthroptby- lamellar - rna]'be single or onion skin in appearance- poallel or spiculated "hair on end" oftenassociated with

rnalignanq' @u,ing's sarcoma)2. lnremryed

a. ButUesssolid appearing wedge of bone, forms al marginsof slowll' lesion(chondmmlxoidfibrorya matigrunt change of long-srandingbeni8n lesion)

b- Codman Trianglesugge$s aggressive mdignant lesion seen in

5 9

osteosarcorna and chondrosarcomac. I-amellated Reaction-onion skin appearance,c"n beseenjn malignant- -

(oseosarcoma) and benign lesioru (eosinophilic granulorna)3. Complcx

Sunbum panerr\ higbly zuggestive of osteosarcorna5. Size and Share:

ln general. malignant nlmors are larger than benign tumors. ofien >6crnElongated lesions in u'hich the greates lesional drameter is 1.5 times tbe leasdiameter mal'be indicative of Eu'ing's sarcom4 histioqtic llmphom4chondrosarcoma and angiosarcoma.

6. Sofi tissue involvement:If lesion lea.ts to conical Ueanmugl thm i1 is aggrcssive

7. Skeletal location of lesion:Tumors mal.predominare in areas of red or hematopoietic.rnarro\\'. These include meta$atic disease. plasrna cell myeloma Ewing's sarcornaand hisnooti 9 llmpl-roma,The tendenq'for these tumors 1o irnoh'e appendiarlar and axial skeleton in the),oung and onll'the aiial skeleton in aged is related to the changing distn'hrtion ofred marrow.Some urmors are mor€ prelalent in areas of rapid bone growth ntch as disal femurand proximal ubia.Tibia: increased incjdence of adamantinoma, nonossifyingfibroma and chondromlxoid fibrora osteosarconul osteoido$eoma, Giant cell tumorRbula: non-ossifl'ing fbroma aneur-rsmal bone qrs- Ewing's siuconra lipornaFoor chondromlxoid fibronq anatrysrnal bone cyst"chondroblastorna osteoid osteoma osteoblasorna enchondrroma lipoma

8. Location of lesion in bone: (Fie'l)Transverse plane:

Central.......... Solitary bone cyst, enchondromaEccentric.-......Aneur-vsrnal bone ryst, giant ceil tumor.osteosarconra chon&osarcoma, fibrosarcoma, chondromlxoidfbromaCortical. --. -....Non ossiS'ing fi broma, oseoid osteomaJurraconical/Farosteal....Osteochondroma iuxracorticalclrondrornas parcsteal osteosarcoma

Loneitudinal plane:Epiphysls........ ChondroblastornaMeuphysis.....-.OsteosarcomaDiaphl'sis........Eu'ing's sarcoma, retinaculum cell sarcom4 multiple mveloma and

metasuBesPhyseal.......... Giant cell tumorMeuDiaphl'seal..Unicameral bone cysL enchondroma

9. Trabeculaoon:-rna1' refl ect resi r+rrql uabeculation displaced [' tumor orneu'bone formation dre to the tumorPanern Tumor&bcatq thin Gitnt Cell urmorcoane. thick Chondromlxoid fibrromalobularecl non ossif;"ing fibroma&licare, horizontally aneurysrnal bone q'sorientedsriated radiafrng hemangiorna

60

10. MarixFanern:<enain tumors prodrce matrix tllat calcifies or ossifies

-calcifc circles (ring) dre ro endochondral boneformation

-flocculent-fleck-like (stippled)

I I. hrienr ase: (Fig.21Age: Lesionl. I vear Maasuticneuroblastom.r

2. I 1'ear to l0 vrs. Ewing's sarcoma

3. 101'ean to 20 1rs. Aneunsrnal bone flst

4. l0 yean ro 30 yrs. Osteosarcorna

5. Skeleullf inmrarure Chon&oblasomaAse: ksion6. Skeleull-v nulture to Giant cell tumor

50Yn'

7. 30 yean to 60 1rs. Chon&osarcomahimarylyrnphornaMalignant fibrous histiocyomaFibrosarcoma

8. 50 yean ro g01rs. Multiple mvelomaMetastasis'

Benim verss Maliprrant Bone ksionsBenien lr4alierrant

Size Small lt €eSoff tissue mass None l-argBFeriosteal reacrion Rare C-ommonN4aryins Sclerotic NoneZone of transitjon Narrow WideTrabeculation yes NoCortical destruction Rarc UsualOverall appearance Creognphic Fermeative or Molh-earen

Other diagnostic tests include:-Bone scans

prorides insight on multiple sites of irn,oh'emedIncreased upake of the nucleotide in bone reflects tbehlpermerabolic osreoblastic aaiv$

{onqqtredzed tomographyhelps to determine more precisell'the location and exent of im'olvement of rhe urmor

-Magnaic resorunce irnaEngalso helps to daermine the location and extent of in'olvement of the tumor. howwer, benerin asseSqingthe sofl tisstes and - 'rnor characteristics.

-l:boratory srudiesAlkaline phospnaus{btood and urine)Cornplere blood countChe$ x rav

6 1

prostate qpecifi c antigen@SA)thlroid function tests(fFTs)liver firnction test (LF-Dcannoembr-voni c anti gen(CEA)

-Biopq'lncisional: anning inro the lesion to remove a sample for pathologl'elaluation

-requLed for maltgnanl bone lesionscrries risk of spreading lesion by lymphatic orblood seeding

<omplicatiors are minimized uilh careful incisionplanning stria hemosusis. minimal dissectionavoidance ofneurorascular strucnues and tight wound closure

Excisional: remolal-olentire lesiorr (done for benign tumors)

Common Tumors ofthe Dstal Lee and Foot:

BEMGN TT]MORS:L Osteoid osteoma-+enigr

osteoblastic lesion clraractgri?d by a rvell demarcared cor{nidus)of usually

tlan I crn and a distinctiv'e surrourrring zone of reactive bone forrnation-age : lO-25 1'ean (rare>30 1'ears) male9fernales-location'. fernn32%o fti/a24Vo foot I l7o-presentation: point ten&rness over the lesion nocturnal pai4 local soft tissre ss'elling tn

rhe foot, the lesion usrally appears at the junOion of the anterior mi&third

of the ulus or os calciq salicl'lares ma1'completell'relieve qfmFoms b1'prosaglandin

inhibition+adiographicfeatures.lesion is located most commonly in tbe

corteL The lesion has a radiolucenl nidrs with a suiking

rhickening of adjacenr conical'bone and periooeal new bone formation-pathologicfeorzres: lesion is charaaerized b'rzling degrees of osteoid and highll'

rasorlar supporting osseous tissue.-fteatment: excision of Oe nifus and surroundingbone

2. Osteoblasorna-bet igt, rap,dl]' grouing tumor lhal is hisologicalty similar

ro ost"oiO osleoma. Chzncrrr.:rrdbl' the absence of an1'reactire perilocal bone formation-age:2U30 years of age (W/oare <30 years of age)2:l males:females-location: diaphyseal and meuphvs€al in the foot- lhe tumor

occurs most commonly in the dorsal aqpect of the anterior portion of the talus-presenlation' .itll, achin& localizd pafuL Not noOurnal

parn. Pain is not rebeved bf' salicf'lates. lmlized sn'dling-radi ographi c fealure s: lesions are well cirqrmscribe4 radio

luceni and usuallv expansive u'ith occasionally a thin shelf of reactive bone.-pathologicfearures. urmor is trmorrtragic, grtD'and friable- I-arge lesions na1' sholr'

cenral soflening and q'stic degeneration-trealmenr. cureuage and packing of the defecf

3. Enchondroma- +eip hlaline cartilage grourh rh't &\'elops uithin the

medrllary caliry- of a single bone-age: usualll'nranifest drring 3rd or 4th decade: egual distribJtion betu'een lbe se>ies-location: metaphl'seal to diaphyseal - most occur in the sall

age and gen&r specific studies rnav includeserum prorein elecrophoresis(SPEP)urinepmtein elecrrophoresis(l]P-EP)

I

tIttIIIId

tITtIIITI62

4. Osteochondroma$cnig, slov'groring mushrmm sl,aped hlaiine cartilage cappedlesions. Classicalll'arise from a growth plate in long bonesgrourh ceases u"ith skeletal rnauitv

, age: 2nd decade,2;I male:fernale_, _-location'- metaphl'seal to diaphyseal - exostosis points aua)' from adjacent joint -commonly seen inthe disul femur and proxirnl u'bia-presentation: oflen an incidental foding on x ra)' most commonly a painlesg slon' groninglesion - pain ma5'becausd bJ' impingement of zurrounding anaromic s6ucrures.-radiographic features: sha4rly pedunculared or sessile tumorar tlre level of the metaphl'sis tlut poinrs anay fiom the nean4, joint.-pathologicJeatures: cortex and penosteum of the tumor ate continuous uith un&rl1,ingbone. Hlaline cartilage is usually 2-3 mm- thick and this diffentiares an osleo-chona"r*fionr a suhurgrral exostosis w-hich has a fbrocartilage cap.-ucarnent: excision*rless tran l%o gyve rise to chondrosarcorna

5. Non-ossifiine fibroma- age: lsand2nddecade- location: cortic'lly based metapbyseal lltic lesion eccentricallv located-most cornmon in

disral fernur and proximal ubia- Presentation: usually aslmptomatic unles associaled u"ilh a prhologic fractrre n,ith

accompqing pi4 swelling and disabilig.- radiographic feotures: Lesion is an active and proliferating form of fibrous conical defba

seen in infants and chil&enlucenl eccenEic lesioru *nr may be loculared wi& a scleroticrim

- prhologicfeatures: dense collagen, gianr cells and lipid filled histioores- treatmenf Ctrredage

6. Chondromrxoid fibroma

rubttar bones in the foot seen in meularsals and pralanges'presennrion: lesjons develops slow11' and gradualll'and is well esablshed before clinicalq'mpom. Oflen painless swellingisthefim manifestation-Painful lesioru girhoutraurur shonld arouse suspicion tut a mrlignant rransf,ormadon is occumng.-r a d i o gr aph i c fe at u re s'. l esi ons are well circunscnbed sithdistinct ares of rarefaction-rhe lesion ru1' shou' speckled calcification-pathologtc fearures'. h1'aline cartilage with scatrered calcif cation-reamenl'. cuenage** The qndrome of multiple enchondromas is knoun as Olle/sdisease. If also associared ndth sofl tissue hemaagiomas, the disorder is lgroun asMaftrcci's srn&ome.

rare, benigrr tumor cornposed of chondroi{ fibrurs and myxoid tissuesage:2nd to 3rd decadeg male>femaleslocation: occur in the metaphyses of long bones of foot - l60%presenbtion: usualll' complain of localized, drlt achy pain possibly sg,elling andtendernessradographicfeatures.lucenl tumor, usually >5crn uith welldefined sclerotic borderspathologicfeatures: nodujes ofpoorlv formed hlaline canilage and rr_nxoid tissuedelineated by fibnous sepaetreatmenr. arrfiage

7. Aneunsmal bone o'st- prlmaD'tumor like lesion u'hich iniures an AV fim-rla and therebn' cr€req lia hemo-

{rumic forces. a secondan'reactire bone lesion- age: most occur il patients under 30 1'ean of age, eqrnl ser disrr'bution

63

- location: ru1'affeo an1'bone - fmt{Yopresentaion'. mild to moderate pain as the lesion develops, patient may limpr adi og rap hi afe a lu rc s :small lltic Jesion seen earlp:

- larer, areas resembling Codman's uiangle ma1' develop al the blowoul $age the bone ma1'apqar expanded u'ith no strell around the lesion. l:te lesions as a result of fibrous septae

- and bony spiailes apear loailated- **angographl' ma1'help to define tbe lascular malformaion- rreannenr: curenage and packing u'ith bone chips

8. Simole bone cvst (Unicameral bone cyst)-fl uid containing soliurl' unilocular ryst-age: 8O-9tr/o Q0 yean old, 2:l males:females-locaion: meupbyseal region of Iong tublar bones in juxuposition to the eprphyseal plate-presentation: indistina discomfort makes the q'sts prcsence knoqn In 5V/o, palhologcfiacmre is the cause of diagnoss-radiographicfeatures'. fiom its principle metaphl'seal location the cyS exends into thediaphlsis_.C@qle{rlqcd1lrqocupye.qelrql-lqq!9&_iqlqnglhrs_gleater tlan its n'idh The overlf ing cortex is eggshell lhin tilt alual's inUct-***fallen fasrnent sisr-radiologic finding describing a fiee fiagmenr of cortical bone frllen by'gravig'in the fluidcontaining gu--pathologicfeatures: serous usually bloody tinged fluid is expelled upon opening. C.avi$ isunirystic with a whitish- thin glisening tining.-trealment: ctmtuge and packing with bone chip

9. Giant cell tumor-uncommorl localll'aggressive tumor so named because it contains multinucleated oseo-clas tlpe giant cells.age: afts dring 3rd to 5th decades-location: adrlts: inolve both epiphl'sis and metaphl'sis(adolescents: confined proximally by the gowth plate and arelimited ro the meuphysis-mos arise about &e ln€e)-prcsentation: location of lhe tumor near ends of bones carrsesarthritis Dpe s)'rnploms<ccasionall-v pathologic fracture is presenting concern.Su'elling intermiuenl pin and limited range of rnotion also can be seen.-radi ographic features. large, lytic "soap bubble" lesionsu'hich are eccentric ma1'have a thin, penlesorul sderotic shdl-pathologicfeatures- large and red-broun and freguentlyundergo cysic degeneration Conrposed of unilonn oval mono-nuclear cells which are lhe proliferating component ofthetumor. Scanered u'ithin this background are giant cells having l0O or more nuclei.-treatment'. culeuage. recuTene rate iS 4O4U/o

10. Fibrous dv'splasia

-localize4 progressive replacrmeil of bone by a fibrous proliferation inrermired u'ith

poorly formed, haphazardly arranged uabeculae of wol'en bone.<ge-Zrdto 3rddecade most common-locati on:monostotic-in'olv€mm1 of single bone

polyosotic-im'olves more tban one bone, can beseen n'ith rarious endo-crinoprhies

-presenanon: S'InFolns depend on bon{s)involved and degreeof im'olyement-pain -6 5s'slling ma)r accompanJ' pathologic fiacture.

-radiographic features- "ground glass' appearance nith a nelldefined sclerotic rim

central metadiqhlseal in uhilar bones-pathologic features: lesiornl tissue cornposed of cun'ilinear

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64

trabecuiae of woven bone surroundd b'a moderatell'cellular fibroblasnc proliferation.-treafinent'. excision or olreuaee in monostotic forms.

t

11. Chondroblasoma- age:2nd decade,2:l male:female- location'. epiphl'seat eccentrically located - foot lflt,- presennnon: purfitl and dre to location oflen lead to arthritis type qrnpoms u'ith joint

eftrsions and limited range of morion.- radiographic Jeatures: 'u'elJdefined geographrc lucenq' liral commonll' has spotn

calcif cations- pathologic features: composed of sheets of compact pol_vhedral chondroblasts uith well

defi ned c-vtoplasrni c borders- treatment: curetuge

MALIGNANT TUMORS

l. Osteosarcoma- -ggressive mesenchlmal tumor in n'hich neoplastic cells prodr.rce bone marix- age: most common in persons <20 I'ean old- 2:l male:female- location: 80-90% arise in the medrllarl'caviry' of the meuphyseal ends of long bones hrt

an]'bone can be bvolved- presentation: rEi\ local tenderness and sl'elling - sudden fracnrre is sometimes lhe

presenting qndrome - at time of diagnosis 20olo of patients hare demonsrable lungmetastases

- radtographicfeatures; large desructive-mixed h'tic and blastic nnss llilh permeativemargiru. Perioseal reaction = Codrun's riangle

- rreameil: chemotherapy and irradiation to dehrlk lesron- followedby resection/amprtation-

2. Chon&osarcoma- second rnost common marix-prodrcing tumor of bone behind osteosarcoma- cornmon feauue is lhe profuaion of neoplastic cartilagB- oflen aslmpomatic- age. most corffnonl]. seen in 4060 r-ear age grotp. 1.5:l rnale:female- location. commonly adse in the central ponions of the skeletorl pelvis and shoul&r

girdle. proximal fennrr.- presennnon: pa.inful pogressively enlarging rn?<ses- radiographicfeatures: localized area of bone destruaion punctuated by mottled

densities fiom calcification or ossification.- pathologicfeatures: conventional chondrosarcomas are composed of maligrunt hlaline

cartilage. Mlxoid rariants are 'r'iscous and gelatinous-spottJ' calcif cations are gprcally- presenL- rrealment: amurtadon

3. Fibrosarcoma- rare collagen-prodrcing metasatic neoplasm- oge: czrn occru al an)' age, hou'ever, mo$ corrrmon in 2nd -4t decade, egual sex

distrih.non- location-. arise in metaphlsis of longbones and pelric flal bones - most corrmon in femur- presentanon: enlarging painfuI srasses u'hich nral'be palpaHe and ren&r often present

udth patbologic fiacure- radiographic features: permeative and lltic lesion tlu,t oflen exends inro the soft tissue-

slight sclerosis at maryins of tumor- parhologicfeatures: composed of maligrrant fibroblasts arranged in herringbone pattem- realmen: arnDuladon

65

4. En'ing's Sarcoma- uncommon primarl' snall round ceU nrmor that exlu'bis a

_ ---primitivereual phenotvpe-- age: lGl5 1'ears old uulPfemale, bladrs rarely'amioed- location: arises in the diaphlsis of long tubular bones especialb'the femrn- presennnon: parnful enlarging rnass. Affeaed site is fiequentl-v tender, narm and s*'ollen.

Pathologic fractures nuv occur- radiographic features'. affeqed bones ma1' demonsrrale non*pecific "onion skinning" and

lync lesions- pathologic features: composed of shees of lnifonn small round cells &at are slightll'

larger than lrmphoc-ves u,hichapear clear due to increased glycogen content.- treatmenr chemotherapv and surgical rcsection with or*ithout radiation

5. Multiple Mvelorna- most common malignant bone tumor- age:4G701'ean old male9females- locanon: mqltiplq sitgE, meuphysis of long boneq spine, sku[ and pelvis__ -- presentation; fever, malaise, u,eiglrt losg fatigue, anemia.thromboq'topenia and renal

failure. Fain in one or more bones that increases uith u'eightbcaring- monoclonalgammopthl' on SPEP. hoteinuria dre to rernl damage and charaaeristic Bence-Joneshotein.

- radiographicfeaturcs rague radiolucea[ o\a] lesions

METASTATIC TTIT'ORS-pathuay's of spread include:

l. dircct extension2. lyrybatic and3. rntraq:inal seeding

carcinomas of the breast, prosull€. lung bdnel', and thyoid are most common sourcesskeleul metaslases are Qprcaltf'multifocal. ho$'ever. €rcinonas of the kidnel'and thyoidmay produce soliury lesions.most meustases indrcr a mixed lltic and blasic reactionin lyic lesions lhe metastatic cells secrere substances such as progaglandins, interleukinsand parathgoid hormone related protein lhat stimulates osteoclastic bone resorption.Carcinomas of the ki&re-r-- lung GI and melanoma prodrce this t_vpe of bone desruction.meulstases Ont prodrce a sclerotic response 56inrrlqlg osteoblastic bone formarionhosatic adenocarcinomas Opcally produce this tfpe of response.

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66

Evaluat ion- Visually inspect the lesion for edema, color, overlying skin, location and

transil lumination.- Palpate for size, consislencl', mobility and lenderness/pain.

Mali-qnant- Sarcoma- Aggressive- Rapid destructive growth- Recurrence/met astasis- Large- Solid- Fixed

Malienant- Poorly defined margins- Heterogeneous signal- N-V or bone involvemenr

BionsvDefinition: Removal and examination of tissue, cells and fluid fiom the living body.

Types ofBiopsy:l. Closed - obtain tissue percutaneousll, 1ryi1h a needle

- fine needle - done with 25-gauge needle ro aspirate mass- core needle - done with cannulated needle with inner trocar* Closed biopsies are easy to perform and indicated for local recurrence

or infecrion. However, they oflentimes result in insufficient tissueretrieval and diagnostic inaccuracy.

2. Open - tissue is obrained through a surgical incision- excisional- removal of entire mass

- done for small subcualaneous, obviously benign lesions- incisional- removal of a port ion of mass leaving main mass in situ

- performed for suspected malignant massesIncisionalPlacement:

- longirudinal on exremities- length short as possible- over midponion of mass

----{ofi-TissueMa

Benign verses Mal ignantBenign

- Resemble derivative tissue- Aulomous growh rate- Local invasion rare- Low recurrence rate- Small- Cystic/fluid-filled- Movable

N{RJ - Benign verses lValignantBenign

- Well-defined margins- Homogeneous signal intensitv- No N-V or bone involved

67

Ganglion CYst- well circumscribed, soft, cystic mass- usually in dorsal foot or ankle- close relationto tnndon sheath or joint- aspirate for definitive diagnosis - straw colored fluid- treatmenl - marginal local excision

Plantar Fibroma- subcutaneous thickening of plantar fascia- usually affects medial and central bands- bi lateral1O-15%o- treatment - wide local excision- reculTence cornmon

Lipoma- most common benign tumor- subcutaneous, soff, movable mass- 2 types:

- superficial - well-circumscribed, static- deep - no margin

- treatment - marginal excisionNeurofibroma

- tumor of spindle cell origin- fusiform expansion of nerve- solitary or multiPle- cutaneoud or deeP- multiple associaled with von Recklinghausens disease- lreatmenl - excise culaneous. very difllcult to excise deep without

destructionNeurolemoma

- tumor of PeriPheral nerve sheath- discrete, tender nodule- usually affects major nerye- treatmenl - blunt marginal excision

68

I. DefinidorxA DVT - blood clots n'hich usualll form in fte deepvenous g'$em of the loqer ex'remiq'B. PE - a deuched thrombus. usuallr'fiom the deep verns in the leg (95yo), w'hich trar,el ro and lodges in the

aneries of the lung

II. homoting Faoors - Vircbou/s Triad1. stasis2. abuonnalities of the vessel u,all or rt^quge to the rrall3. "hlpercoagulabiliq"'

III. Pathogenesis / Pathophl'siolog'homoting Faoors (Virchorv's Triad)

White Thrombus - nirtrs of platelets

Red Tlyorubrx ->Fibrinollsis __yResolurion(fibrin thrombus) or

OrganizationhrtnonaryEmbolus

-red thrombus - develops rapidl-v (minutes) and is ven'unstable at fim (It rakes Z-10 da1's to sublize.)-fibrinoll'sis - Ore process br. n'hich fbrin is degadedorganzarion - a 7-10 dav proces during u'hich the thromhrs sabilizes resrhing in vessel narrouing

IV. Conditions associarcd u'ith risk of thrombosisl .postpanum 5. lower exrremilv trauma/fi-acnrre2. vent'icular failure 6. chmnic @p venous insuffciencr3. prolonged bedres 7. using estrogen/oral conrracepf,ive4. carcrnoma 8. recent pelvic or lon'er ercemiq'

surgerl-

V. DiagnosisA D V Tl. Classical - pain heal. swelling in tlre affmed limb2. PE - presenrs as rhe fim sigr in 7tr/oof piena uirh DVT3. Homal's Tes - dorsillexion of Oe foot elicils catf p.ln4 . Prau's Sigrr - compression of calf causes pa.in

B. PE1. sudden onsel2. *classic" triad (onlv ex?erienced in l4%o of parieuu)

a. dl'spneab. ches painc. hemopn'sis (uch1'cardia is acnralll'more common)

3. 85%o of patients u'ilh f:rel PE have some uarning sip such as prior embolic eyenlvenous disease

or s\mF.orruluc

M. Dapostic Tests for DVTA Non-imasive Tests

l. Impedance Plethl'smography - a rapid test to eraluate for D\T proximal to rhe rligh2. Doppler Techdque - an operaror dependent Eghnigue n'hicb demonstrares DVI b\. lack of venouscompression

69

3. Radiofibrinogen - a rery sensitive test for tbe diagrrosis of tbrombosis in lhe calres

B. Inlzsive Testsl. Contrast venograPS'

MI. Dagrrostic Tess forPEA Blood Gas - PaO2 < 80o/omnr Hg PaCO2 < or= to normalB. Ches X-ra1, - 5ff/o are normal (rul)' see a hemidiaphragrn ateleaasis. densin', pu]monary u'edge)

C. EKG - TachycardraD. Ventilation-Perfusion Scan (V/Q Scan) - a mismatch demonsradng an are-l of ventilation but no

perfusion suggess PEl. Perfusion Scan - Injeo technedum 99 labeled human serum albumrn2. Vendanon Scan - lnhzlation of xenon 133

Pulmonarv Angographvl. Definitive rriegnosis Orus indicated s'hen V/Q scan is inconclusive2. Diagrromc sigrrs are: inu-aluminal fi.lling defect- an abrup vessel culofi. or loss of sde branches

Cathererization of lhe rigbt side of the hean and mec-sure the p.rlmcnar-r'aneu'and right ventricular drasolicpresswe (increased pulmornn' resi sunce)

Mtr. hophvlaxisA Subcutaneous Heparin

l. Fe-op - 5.000 u SQ 2 houn before sutgen'2. Mainlenance - 5.000 u SQq 8-12 houn3. Compression sockings4. Interminent pneurnatic compression derices5- Earll'ambulation or range of motion

B. Asptnnl. 325mgpo gd (bb]'ASA 81 mgpo qd)

D( TreaunentA Anticoagulation

l. lntravenous Heparlnl-oading dose - 10,000 - 15.000 uh4ainternnce via fV drip- surt s'rth l.000 u /hr(mix 25.000 u in 250oc of D5 l/2 NSS)

Moniror prg rt^ilv - titrate heparin to rnaintain 1.5 - 2 times patient conrolProamine sulfate reverses heparin (Ratio: I mg protamine strlfate per 100 u heparin). caurion should be used there are manl' possible si& afeos of protamine and dte to haparin's

shon balflife (12 -2 hours)

Nore: Weight Bas€d Pneumogram of Heparin Dosage (rariations exis)Bolus 80 ulkg Hepann IV uith a maintenance rare of I E u/kglttr, check PTT in 6 hours. tlen if:

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PTT BOLUS C}TANGE RATE< 35 80 u/kg (lean u'eight) increase 4 uh.gftv

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3 6 - 4 54 6 - 7 07 l - 9 0> 9 0

40 u/kg increase 2 \/kg/hr--tekg no aCtion

decrEase 2 uAs/hr

70

bold heparin I bourand &reas€ 3 unrgnu

2. Coumadin (narfrrin)a. Onser of aaion is delayed (peak effea 3-5 days)- -------b.---Sundderheparin-is-fterapeuri

c. Dosage 2.5-15 my'da1'd Tirrare dosage to 1.5-2.0 times conuole. ViEmin K rererses Coumadrn

3. Lovenox

A Vetrous irtemrpion operatiotx - intracaval urnbrella- balloon or rvire filter placed in tle ilferjor venacatz beloq'De renel t'ein<

B. Thrombolltic Oterapt'- Urokinase or sreFokimse ro dissolve c)or (mus be initiared u'ithin 244ghours)

C. hrlmorrul' Emboleaomv

X. Diflerenual Diagnosis for DVTA CellulitsB. Conrpa.nmem sndromeC. Venous stasis

)O. Miscellaneous

A Fat Ernbolisml. Usualll' occru's nith large facrures of the lower errremiry' (fiom bone marrou' fat)2- Thee organ svsems u'tth qmptoms: hrlmonan'Olpoxia), Cerebral (confusion). Cuuneous (perechiae)3. Treaunent - largell'suponive

- IV fluirtq. respirarory supporl fracrure care. seroirt<. and hyrenonic Bucose and insulin

B. Srperfi cial Thrombophlebiusl- hesenu as a palpoble cord along the disrn-bution of a 'ein nith enlheme. edem2, and pain locall1..2- Treatmenl - supponive consisrrng of local heal eleradon and rest. Antiinflammator-r'"g.nt rna],69

given.

D. Emboh seconda4'ro endocarditisI. NloS corrrmon cause of endocarditis is Strep. \4ridans2. [VDA- Staph. Aureus

l. Anerial Dsease:Definirions:Arteriosclerosis: generic term for a group of vascular diseases u'hich cause thickenine andrnelasnciq' of aneries. decreasrng the blood suFpl), to the lissuesAlbcrosclerosis: diminished sDe of vessel lumen due to t-be formation of fibrofarry' plagues alongthe intimal surface of the ane4r..Acute aneriaj mclusiop: anerial embolic event usuallv derived from the hean (arial fibrillarionM. rzhular disease, PTCA CABG) u'hich cause occlusion of small end anenes rrirh iscbemia tocorresponding tissues.J\tonckeberg's medial calcific sclerosis: calci-ficarion of tbe media of the anen.. Does notdecrease the r,essel lumen but does falselv elevate AIII's.

7 1

Major fusk Factors:-Diabetes Mellitus

--dypercb ol estero'l em i

ir4inor fusk Factors:-Obesiq

-E1'penension-Smoking

Clinical hesentation:-Acute anerial occlusion

PainPaJlorPulselessnessParestbesiasParall'sis

-Chronic arterial occlusive disease

-Age-Genetic predisposit ion-Decrcased erercise

. lntermituntclaudicationlocailon relared lo level of obstruction calf most corrrrDoncomplain of cramping brought on bv a ceruin amount of exerciseslmploms are reproducible and relieved bv rest

r Rest Painnocrurnal and diffuse in foot u'hile lving n'ith legs elevatedlegs placed in dependenq' for re[ef

. Ischemic ulcerarion / gmgranemost advanced compbcationgeneralll'occur in distal porrion of the footulcers look necrotic and do nol bleed r*irh debridement

Clinical Signs of P\lD'-Color: pallor, q'anosis, rubor on dependenq'-Temperature: decreased

Qualiq'and Quantin'of skin nails and hair:skin: shiny, scall', auoptucnails: thickened, {'strophicbair: diminislted

-Edema: ma1'be increased due lo continuous depcndencl'

Vascular E:ramination-Pulses

palpate - femoral- popliteal. dorsalis pedis and posterior tibial arteriesdoppler

-Ele\ational Bllorrlerale legs abol'e heart for al leas 30 seconds-eraluate color and grade 0 to 4 with +4 indicating marked pallor

-Venous filling time-afier elevational pallor test have patient sit up quicldl'-measure dme it ukes to fi.ll dorsal venous arch-Normal l0-15 seconds. Significant disease 3060 secondsdependent rubor ma1'follos'2-3 minutes larer

N on -ilrasive vascular lestlng-Ankle arm ildex

-ankle q'solic pressru€ / brachial q'stolic pressure

nuick and reliable test- offers liule informarjon aboul anaromic level of occlusion-Segmenul limb pressures

-q'stolic pressures measured u'it-h cuffs at groin above !nee- belon'knee. ankle andrranseutarsal levels-should note a gradual decrease of approx. 20runllg doun the ipselateral limb

72

-both limbs should be u'irhin 2OmmHg ar similar levels-medial calcirosis results in falselv elevated presswes resultrng in unreliable information

lndices1.00- 0.90.89 - 0.70.69 - 0.50.49 - 0.30.29 - 0.0

normdmi ldmoderateimpending gangrenedistal necrosis

-Segmental plethl'smographl'-air cufltechnique (Raines) utilDes same cuffs as segmental pressure measurements-inllate cuffbelon'DBP - allows snrall changes in ex-trenut_v volume to be translaled as apressure chalge n'hich is ploned \/ersus time (P\lR- puJse volu:ne recordrng)

cres- rapid vessel erpansion in svstolenormally <35Yo

dicrotic norch- due to elasic recoilamplirude- normallv 8 -12 mrn decreased in aneriosclerois

-orher plerh'r'smographl, trchniquesimpedence: mq?sures volume shrnges bv assessing d.ifferences in resistancebelween 2 elecuodesmercun' srrarn gauge: eiasic rube filled n'itl mercwf is u'rapped aroundenremin'. as rube elongares u'il-b s'slolic er?ansion resistance changes.Photopletlrl'smographl': sensor probe is placed on drgil q'hich contains inf,aredlight and a sensor. The lighr reflecred and absorbed b1 tissues is relared rocapillan'bed volume.

Transculaneous oxJ'gen measurementsass€sses ussue meubolism as a function of perfusion< 20-30mrnrjg high correlarion s'ith dec. abiliq'/inabiliq'ro bearexlrerxt1' dependenq increases TcpO2 r.alues

Stress testing / Exercise Response:ambulne on a readmill at roovo gradrent ar 2MpH for 5 minutesat complerio4 perform ABI and repeal even'2 mrnutes until pre-exam level obtarned..norrnal palients have no appreciable drop.

Mapetic Resonance Angi ographl':avoids use of ionizing radiation and need for contast media as in aneriognphl-allows multiple imagng planesconsjder in pts. with renal failureSpin Echo (SE) --+lood appears dark ( lon, sigrral inrensir-r)Gradienr Echo (GRE) - brood aFpcars btighl r high signar inrensity)

Aneriographl':involves the injection of a organic iodine conuast medium in the anerial s\-stem u-hich isthen visualized q'ith radiographsusualll'resen'ed for parienrs $at rnal'be cardidares for blpass su'ge$-conrraindicared in patients rvith a iodine allergi,need ro sglilizs Cr to <2.0prs need to be NpoanncoaguIaoon given after procedure (plavix)

Treamrent: (Consenaut'e)

Treamrent: (Surglcal)Balloon angioplaq' - balloon catheter is fed into a localized area of senosis and inJlated ro dilaret}re artery'.Blpass surgery'- utilizes eil-ber autogenous veir grafi or srr:t-betic anerial prosl-besis (gortex) rocircumvent areas of occlusion and improve drstal circulation.Laser angioplasn'- laser probe is passeci percuuneousll'anci Oren activared at the site of occiusion/ senosis to recanalize lhe vessel.Atlerectomr' - utilizes a high spced rotational burr to ablate stenosis and improve flou.

II. \,ENOUS DISEASE:Superfi cial ThronrbophJ ebitis :

-palpable cord along disribution of superficial 'r'ein sith local enthema pain and edema-treatrnenl consists of local \l'ann conrpresses- elevation. resl and anti-inJlarrunator)'medicationsdiscondnue heplock if on the ilt'olved ertremin'

Deep Vein Thrombosis **see seclion on DVT**

XTA G ENTIC RESONANCE IMA GINGGENERAL CONCEPTS AND PATBOLOGY

I. IIRI PhvsicsA Based on the bvdrogen atom.

l. Hvdrogen atom is made up of a single prolon in its nucleus.2. Hvdrogen is abundant througbout our bodl'.3. Hvdrogen has the highest grrornagnetic rario loou'n.

a. Precession is u'hen a proton spins around its axis when placed in a magrretic field. (Example-a top)

b. The frequencv of this precession is determi:red b1' the Larmor Eouaion'.

(D = TX p

o = Precessional fl-armor) Fre4uenq'"l = Gvromagrretic Ratio

B = Srrengrh of Enernal Magrra

c. Since h1'drogen has the largest grromagrretic ratio, it pecesses at a higber fieguenn'.B. Atoms uitlr an odd number of protons and neutrons in their nucleus are small. routing magnets l}:tl

align tlemselves in the direction of the magrretic fieldI. Nuclej ir a sample tissue are rardomly oriented unril a magrreric field is applied2. Bulk A4aqnificaion l/ector (M) is the sum of the magnetic moments of all of tlre hvdrogen nuclei

in a sample tissue.C. A radiofrequencv is applied and protons are stimulated

l. Resonance is a process s'here nuclei make transitions benveen lou' energ' states @mllel to themapetic field) and higb energr sutes (anu-prallel).

2. A radiofreouencv (RF) pulse induces transitions betu'een eDergi' sutes and tends to decrease thenumber of nuclei pointing to the nrain magrretic field

3. Before a 90o RF is induced:a. Protons are in a longitudinal, relaxed state parallel to tlre z aris.

t5ItrlI\

L

rIfrtltTt

1 A

b. Protons precess out of phase u'jth one another

4. Afrer a 90" RF is induced:a. Protons are excited and flip into lhe transterse n, planeb. holons precess in phase q'ith one another.

D. Afler the lem in:rtion of the RF- *re prolons relar- radjoq'ayes are erruned and an image isreconstrucled onlo the computer. The signat produced depends on the number ofnuclei presenl andilre time it lakes for the nuclei to rela:r

l. Tl Relaxatiou (Spin-l-anice Relaration):2. Time it ukes for nuclei to realigrr parallel (assume a lou'energy sute) to the magnetic field.

M must retrun lo lbe z axis.b. AIso called lonsitudinal relaxation.c. Refleos the chemical environmenl of the proron. Snength in s'hich the nuclei are bound to

t}le cbernical bacldone (i.e. nater, fat).2. T2 Relaration (Soin-Spin Rela_riation):

a. An erponential decay of the xv componenl of M back to 0.b. Measures dephasing of the nuclei.c. Also called ftanst'erse relaxaion.d Reflecrs the relationship of tlre prolon to $e surrounding nuclei-

II. Pulse SeouencesA Soin Echo: Utilizes a 90o excitation pulse follou'ed b1' a 180" refocusing pulse. This is the mosr

common pulsesequence used

Spin Echosq 180-11 -

i l t lErclargf l I

-tR

l .1

TE (Time to Echo): Time beru'een a 90o exciutjon pulse and tbe production of a spin echo.fi 6inre to Recoven,): Time bets,een 90" excitation puJses.

6FTER rc- trl tgtst

75

B. Tl-Wciehred Pulse Seoucncel. Short TE (15-30msec).2-J honlILlZ00600msec)3. Main focus is on TR As TR increases. t}re difference in sigral i:rrensin' (SI) decreases.l. Tl is called the Ji{f image. Fat displal's a high

SI.2. Used for norrnalANATOMl'.

=Eo-t

Ez

T2-\\tcighted Pulsc Scqucncc :l. Long TE (60-Somsec).2. l,ong TR (2000msec).3- Main focus is on TE. As TE increases.

Ylmr {TXUrc. t.t. hatnor.rA CnrarEF^i D ErraraJlatiftE \

the difference in Sl increases.

4 . T2 is called the IIATR irnage. The follon'ine displal' a high Sl:a. Flui4 edema hemaloma.b. lnfection. injlammarion.c. Tumor.Used for PATHOLOGT-.

D. Proton Densin' Ontermediate-Weiehred Pulse Sequence):l- Sbort TE (20aomsec).2. Long TR (1500-2000msec).3. Has a high signal to noise ratio (S/IrD.

, . c . . ,F r rs r t raJ

t l t c I t s J

t - l ' t 1 3 . ! n - r r w h - y - -! t ! h E ! @ b d t t r m

t4-I

_rec.

) -

a. SN is the ratio benveen the snengt} of the signal coming back frorn the nuclei and tlreirotensiry of Lbe nojse fiom tbe parient and tle mechine.

4. Good for delineatine bnsic anaromv.

E. Fast Snin Echo:I Quicker than normal spin echo T2 seguence.

a. For each spin echo pulse sequence- rnultiple srmples are usedb. lnsead of one data line per phase pr:lse- tlrere are several sample echoes in the train of each

TR seguence.2. Sacrifice slight resolution for speed.

a. An increase ir speed results in a decrease in phase direction resolution.3. Stigbt shifi in conrast due to longer TR

a. Tlpicalll'use longer TR (4000-5000), but multircho u-ain still makes it fasrer rhan normal T2.

F. STIR (Sbort Tl lnvenion Recoverv):1. A l80o excitation prlse follou'ed bf' a standard spin echo.2. lf T1 is ven' sborl tbe 90o p:lse of Ote spin echo u'ill occur at a time u,ben tbe M of fat is near 0.

a. Anv tissue uitb a short Tl. sucb as fat u'ill be suppressed Nor specitrc for fat3. Tissues u'ith longer Tl values nill be non-zero.

a. An1' tissue s'it-b a long Tl. such as $?ler, nill produce a high Sl.4 Usefirl for evaluating edema in high lipid regions- such as bone marroq'.

a. Also usefirl ir evaluamg afiicular canilage because the joint fluid is brigbt and rhe cardlage isdarker.

LONGITUDINAL FE' 'N'Af1ON I T}

76

5. Shen contrasl agents ftat shonen Tl are used such as gadolinium. the desred contrastenhancement may be suppressed.

G. Fa1 Saturation:Utilizes RF pulses prior ro tbe spi:: ecbo sequence.RF puJse centered at the lipid rcsonarl frequenq'n{ll saturate lipid protons and route them inrothe x1'plane.

a. Subsequent pulses rotale Ore lipid protons out of the x1, plane and their resonance is inhibiredWater prolons are unafeqed because there are differences in l}re resonant fieguencies betrr,eenthe lipid and ualer nuclei.This technigu e is specif c for fat u'hereas. the STIR rechnigue is not.ExceUent technique ro use u-jrh gadolinium ro suppress higb SI of far.

H. Gradient Echo:1. Surts s.ith a RF pulse < 90".

a. hotons are nol completelv rotated into thc x1'ptane.b. Suffcient magnerization remains along the z axis u'hich allows for short TR times. Shorrer

TR times means shorter intagrng times.2. A sbort TR time does nol allou'the nuclei to complelel.r'dephase before lhe ne\1 RF is in&ced.

a. Common in tissues n.ith long T2 relaxation.3. Magnetization is "recvcled".

4. Also called skodv stare magnetization.-5. Good foryoint imaging.6. Bone marrou'has a lou' SI.

l . Gadol in ium:l. Paramagnetic metal ion.

a. Chelated n'ith DTPA lo avoid side effeas.b. Shonens Tl.

2. Good to use with jat saturotion.3- Two merhods of use.

a. lnrravenous:l. Quickly distribured inro irraccllular fluid2 Are^s u'ilh increased vascuJariq', such as neoplasms and inflammation enbance rapidll'

and reuin conEast longer. **Cellulitis and u'alls of abscesses u'ill enhance bur pusuilinol.

b. Inrraarticalar:l. Assess canilage inregrig'. such as talar dome lesions.

III. PatboloerA. Bone Conrusions (Bruises):

1. Trabecular microfracrure q'itlr edema and bemorrhage.2. Poorll'defi-nd inbomogenous, los'SI on Tt.3. Hjgl Sl on T2 because of acute hemorrhage.

B. Str€ss Fractures:1. Gradual ald progressi'r'e resorption of lamellar bone and replacement of dense o$esnal boag.2. Characterized ['local hlperem.ia- edema, and osreoclasric acdviq..3. Tl- linearzone ofdecreased SI surrounded b]'less defined area ofloper SI.

I

2 .

4

5.

77

4. T2- lnear zone continues lo have decreased Sl- but surrounding ederu has a higher Si.

5. STIR- increase in SI because farry* bone marro\r'is suppressed.---€all ous-fsrn utj stt-hasaninterrnediate-Sl-

C. Neuronalhic Osteoarlhronatbv:1. Difrcult lo di.flerentiate fiom osteomyelitis.

a. Marrou,sigrral changeswilhoul iwolventent o_f the periosteum/cortex is not suggestive ofosreomyelitis.

Tl - marked decrease in SI n'ithin medulla4' and cancellous bone.T2- mild incrcase in SI 0ess than u'ith oseomvelitis).STIR- marked increase in SI.

D. AculeOsleomvelit is:I - Tl - involvemenl of cortex/medullarl' bone shou's slight arcas of increased SI ag,ainst background

of normallv low SI cortex.Z. T2 and STIR- increased SI in Ore conerJmedullan'ponion of involved bone.

a. Refleos a decrease i:: the normal fat contenl and localized pus/edena.

E. Cbronic Osteomvelitis:1. Sinus raas- linear areas of increased SI on T2.

2. Foci may demonstmle lhe"rim sign".a. Low Sl iuea $urounded b' an ara of local. active disease.

F- Sofl Tissue lnfections:l. T2 and STIR sequences are preferred for detection ofcellulitis and abscesses.

a. lncreased SI causing disonion of superficial sofl tissues is seen in cellulitis.

b. Wetl-marginated homogenous, high Sl collecrions are seen with abscesses.

c. Gas contained q'ithin abscesses display low SI on all sequences.

G. Oseonecrosis (A\rl{l:

I - Ilallmark is a reocfive interface.a. A distino layer of inflamrnarory fibromesenchrmal tissue between r,iable and infaraed bone.

2. Tl- q'ell defined line of decreased SI.a. Represents granulation replacement of fat.

3. T2- decreased Sl-4. STIR and long T2- "double im sisn" (Mjrchell & Kressel).

a. Inner margin- increase in SI. Represents granulation tissue.

b. Ourer margin- decrease in SI. Represents mineralization.

5. Sueing S1'nem:a- Class A- charaaeristics similar to fat-

l. Tl- increase Sl.2. T2- intermediate SI.

b. Class B- reflects subacule hemorrhage uith characterisrjcs of blood1. Tl and T2- increase SI.

c. With increasing fluid developmenl in necrotic tissue- SI on TI decreases and SI on T2

increases.d- During $e lare stages u'hen fibrosis and sclerosis uke place, there is a decrease in SI on Tl

and T2.H. Osteochondrd l-esions:

l. Tl- decreased SI within subchondral bone.

2- T2- St msy be increased.3. STIR- marked increase in SI.4. Gadolinium ma]- be belpful. Will have a high SI on Tl.

I . Tendons:L l,on' SI on all seguences due lo lower u'aler conlenl.

2. T2 and STIR- optimizes tre contrasl betn'een the dark lendon and the abnormal increase in sater

contenl of tlre rendon.3. Classification of PT Tendon Tears:

a. Tvpe l- i-ncomplete. hlperrophl'4-5 dmes normal.

)

1 .

t

l

IItt

I

I

ltttltttI

7 8

b. Tvne IJ- panial rears- longiruaitral splining Tendon becomes anenualed and atrophic andassumes a long ol'oid confiSuration.

ffiomplele.rear.retraction-ofondst'irlrotnnousjap.-€nds-ma**penrophy--OJeedto distilgrush from TlPe l.)

J. Soft Tissue Ma-sses:1. Most demonstrale loq'to intermediate SI on Tl and high SI on T2.2. Certain benigr lesions. such as liponre. benulg:oma and gangliorx reveal charactenstic

morphologl'.K. Bone Tumors:

I. Most reveal lo$'lo inlermedrate SI on Tl and hlgh SI on T2.2. Lesiorx u'ith high SI on Tl contain eitlter fat or blood3. Very lou, SI seen on T2 images in celluiar. denselr' fibrous iesions- and in lesions conuining

bone.L. Sentic Arrhritis:

1- T2 znd STIR- increased SI uithin surrounding sofi tissues as rvell as joint fluid is suggesti!'e.2. Need clinical correlation.

M. Merall ic Artifact:l. Two mechanisms:

a. FerromaEretic objects lrave their oun magnetic field Thery cannol be utilized in the MRImachine.

b. Mos orthopedic implants are non-ferromagnetic.l. Distons RF uniformitf'.2. Causes lou'SI adjaccnt to implant bul no more tran I cm auar'.

N. Magnetic Resonance Angiogranhv:1. Most of the erperience is u'ith extra-cranial carotid and inra-cranial circulation2. Non-invasive technique.3- lower extrenriN indications:

a. PVD.b. DVT.c. Neoplasms.d. Anatomic srudv preoperativel-v-.

Radiologv of Infection

I. Nuclcar Medicine Studies:A Technetium - 99m:

1. Most commonll' administered in the form hTc methvlene diphosphonare CIc-MDP) &bydrorymethylene diphosphonale (Tc-HMDP;.

2. Binds duectl)'lo calcium h1'drox1'aparirc to form soluble salu. Referred to as a "bone seeking"ageil.

3. Isorope concenuales in acuve nen' bone formation due to osteoblastic activiq' related to boneinjury.

4. Most u'idel1' used nuclear medicine sntdy.5. NON-SPECHC - Positive Scans For:

Osteomvelitis Recenl Sureen'

N euroFthi c Osteoanlroparhl' ArtlriusB6ag Jrrmgrs RSDFracrure Ischemic Necrosis of Bone

79

Half life = 6 hours7. Remains positive for exended perids in cases of osteomvelitis & post-osseous surgen'because

of eoltilr'ffi remodeling and_osreogenesis-8. Increased levels of isorope il the follonilg sires in normal bone.

o Cancellous bone > conicaj bone. Metapbyseal bone. Penarticular regions. especiallv sacroiliac joints. Epiph)'seal plates. Tip6 of the scapula. Petrous ponion of the shrll. Costochondnl nurgln of the rib's. Slernum

9. E>''tra-skeletal siles I'here the isotope occurs:. Kidneys. Bladder. NasophaDrui. Oropharynx. Glandular tissue of the breans. Lacrimal apFrarus

lO. Phases ofBone Scanning:a. Angioeram Ommediale. Earlv. l" nhase):

' Afier IV iljection ofthe isotope, several irnages are laken I-3 seconds apan as theisotope trat'els fo the cxfemiq'.

. Displays arterial blood flou'lo the ex-tremlN.b. Blood Pool (2'd nhase):

. Images are taken 2-5 minures post ry injeaion.

. Quantigrivell' describes "blood pool" occurring in rhe capillary beds & r,eirx.c. Delaved (3d nha-se):

r lsoloPe seeks bone & demonstrales lhe amounl of osteoblastic activiry presenl. lmages are uken 2-4 hours po$ fV injecrion.. Represents the bone upake for repair or mainlenance.

d Fourtb Phase:. Inrages taken 5-24 hours posl IV injection.' The delaf in tlre nme pos injection demonsrales more bone aaivin. and less sofl tissue

activiS'.. There is a decrease in the sensitiviq'becaus€ the half-life is 6 hours.

I l. Interpretation:a. Evaluare all ptrases simuluneouslr'.b. The angiogram & blood pool phases u'ill be "hot" ira both sofi tissue and bone infecdons.c. Use the 3'or 4'phase bone scan lo differendate soft rissue infectiors from bone infections.

6 .7

IIITlIII

IIt

Cellulit is

Scntic Arthritis

diffirse uptake

dinrse uptake

neg

difts€ upuke

neg

diffirse upuke

ITIIII

B. Gallium {7 Citrate:i. Localzauon in areas of acute inJlamrnation & infection [' binding rngghqni5ms.

a. Direa bacterial upuke b1'bacrerial siderophore complex.b. Lacroferrin- a plasma prorein released !r' leukoclres.c. Direo leukocl'te labeling (only 6%).

2. Scan is raken 48-72 hours afier injecrion of the isorope.

80

3. Like technetiuq it is non-specific. lt is not indcared for detecuon of osreomvelitis butse4uential technetium - gllurn scans can be used.-------1. Renrrnslororrnalafierinfectiona.nd-inJlanrrmtionJraveresoh'ed

5. Positive sczm nnv represent:. Infectiono Fractures. Inflammarion. Epiphr-seal plares. Surelcal traunrao |rlggpl35mq. Goul

C. Sequential Technetium -Gall ium Scan:l. 4 hour technetium bone scan follou'ed b1'a .lg-72 hour gallium scan.2. Increases the specificiqv for infection.3. Posili'e seguenrial rc - Ga scar if tbe gallium upake is greater lhan fts rechnetium uptake.1. Normal scan if the gallium uFake is spatialll'congruenl but is less than the lechnetium scan

Acule Uslei0l|lvelnls + -|

Senlic Arthritis + +

Chronic Osleomvelit is +

Ccllulit is - +

lndium - l l l Oxime:1- WBC's are isolated fiom blood taken fiom rbe pdenr & labeled n'ir} indium -l I l. The ugged

cells are tlen reinjeaed back into tlre patienr. The scan is perforrned 24 hoprs later.2. Higlrll' sensitive & spccific for acrle soff tissue & osseous infections.3' Ma1'nol be eflective in diagnosing cluonic osteomvelitis because it is predominantl-v- a

lrmphoqnic response & will nol cause localizarion of indium labeled neunophils. '

a. Acute & subacule oseom'eritis = neuboDhir response = + scan.b. Chronic oseoml'elitis = predominantJl'llmphoqrrc = neg scln.

4. can be helpful in diflerentiating osreomvelitis 'ersus osreoarthropathl,.5. Hatf life = 67 hours6. lnterpretation:

a. (+) Scan - hesence of indium tabeled WBC's- Higher upake t}un surrounding bone

- Acure oneomyelitisb. (-) Scan - No localiz^tion of indjum labeled WBC's

- Chronic osteomvelitis, oneoarthropathl,'1 . False positives may occur uilh:

a. Aseptic sofi tissue and bone inJlammation such as periostitis secondan'to Charcot changes.b. Hl"peremia & hlpenascuJariq'u'hich may or ma\,not be associared qith inflammauon

such as fractures. arthritis- inflammaton,bone disease.

Technefium - 99m - HMPAO lrukocrtic Scintieranbv:l- Developed ia order to have a uged WBC sru$'q'hich is rechnicallv easier & n'ith less radialion

than indium. This allorrys for more radioactive nraterial to be used & increased anaromicalclnEasLTagging molecule = Hexvlmethr4 propvlene amine oxime.Performed $'obtaini-ng 50ml of u'hole blood and spinning off\\GlC's rlren ugging/labelingthem. 2ml of the preparadon is tben reinjected. The scan is performed 3 hours larer

D.

1

3 -

8 l

4. Mav shon' advanuges over olher nuclear medicine studies in diagnosis of earll' osteoml'eliticchanges; however, it is non-specific and an]' area of inllammation will be hot

��--J---A-negative.scan-can-rule.oul+.sepuc-process.lvith_lrighgrecision{94%%)-6. A positive scan mav not discern septic from aseptic rnflammation wilh srfficienl accuraq'.

Positive predictive ralue = 62%. Note - a subtraction technique is a'rzilable and ma1' f ieldhigher results.

n. Maqnetic Rcsonance lmaging:Please refer to the MRI General Concepts & Pathologl Section for basic principles.A. Cellulitis:

l. Increase sigrral intensity (SI) on T2-weighted & STIR rmages.2. Di.ffilse, infilrative panem througbout subcutaneous tissues.3. On Tl -u'eig}ted irnages. a decrcase in SI occurs duc lo lhc in{lammatorl' process rnfilrrating

& replacing norrual hrgh SI for subcutaneous fat.

B- Abscesses:l. Increase in SI on T2 & STIR irnages.2. Localized u'elldemarcated- relativelt' homogeneous signal.3. Can add contrast agen-L such as gadolinium. lo improve contrast between normal tissue &

pathological lesions.4. Note- lf abscess is fi.Ued s'jth necrotic tissue or pumlence- insead of inllammatoq'fluid the

SI decreases.

Osteoml'elitis:1. T2 preferred orer STIR image for detection of osreoml'elitis.2. lncrease SI on T2 & decrease SI on Tl.3. Detects changes in t}te meduJlary canal, conex zurd periosteum.

o Must have changes in both the medullary canal & cortex to be indicative ofoseomyelitis.

Chronic Osteoml'elitis:1. Opposed to acule osteoml'elitis, u'here l}te conex & medullary canal are desrol'ed thel' are

being remodeled in tlre chronic sate.Conical changes are more erlenqive tlnn tbe rrutrrow & are low SI on T2.Areas of active infeaion can still be seen as increase SI on T2 conuasted agains los'er Sl onrhickened surrounding bone. This is lgtoqn as the "rim sign".. The rim of log' SI reflects fibrous fissue surrounding focal. acrive disease."Healed" oseomyelitis displal's an ircrease in SI on Tl. This represents the infiltration of fatback into fte rnarrog'afier resolurion of lhe infeoion.

Septic Arthritis:J. Joint fluid is bes detected uith T2 & STIR images.2. To diferentiate septic artJuitis fron: nou-infectious effusions- an increase of SI of surrourdhg

sofi tissue must also be seen.

Adranuges of MRI:More sensitjve and specific for infection than olher imagtng modalities.Assisunce in preoperatire planninga. Helps localize idection & determi-ne the proximal &distal boundaries.

o Panicularll' helpfuJ uith long bones & tbe calcaneus.. Helpc determire necessaD' level of ampuution or debridement.

Evaluate for other possible sources of hfection:o fsemple: Patient n'ith crllulitis not responding to curenl trsttment protocol- Need

to nrle oul abc€ss or osteomvelitis.

IIIrtIC.

l--- t

)

I

I

I

\D.

2.J .

t .2 .

)I

)

E.

4. Monitor infectious process:

82

IJI. Comnured Tomoerarrhr' (CT):A Basic Princioles:

els€ Flaft-urtageJ ony-Tagrnd}ane unaEes avalldtle vla reconsruclonon compuler.

2. Higb-energr. ionizing radiatjon.3. Meulbc rmplants nill result in anifacts & drsonion of images.4. Windows van'densin'

Highest Ilensitr ....lnq.est Densin-Cortical Bone

CalcificationCancellous Bone

NenreTendon

LigantentC-anilage

FarAir

B. Clintcal application of CT ro evaluale infecrion:l. Use of sofi tissue uindow can demonstrate abnormal sofl tissue densiq' q'hich mav represenr

suppruailoL reacti.r'e granulation tissue. edenu. or fibrosis.Can be used to evaluate planur compartrnents of Oe foot to diagrrose planw smce infections.Use of the bone u'indou' can demonsrate the osseous and articular alterations.a. Early sages of oseoml'elitis is noted as an increase in the density of rlre bone nu[rorr,.b. Can be used to esublish lhe exenl of the infecrion & help derermine the approximare

lwel of amputadon or debridement.

Rheumatic DiseasesGOUT

Gout is a meubolic disorder in u'hich tissue deposition of monosodium urate c4'srals oco.rs fromsupersarumted eruacellular fluids and tesulls in one or more clinical manifesutions uhich incl"de gour]-anhritis, tophi, gouq' nepbropatby, and uric acid calculi in rhe kidnrys. *** As\rnpromatic hlpemricemiain the absence of gout is nol a disease sule.

Gorn is predominate\' a diseas€ of adrlt men u'it} a peak incidence in tbe fiffh decade. Ir is rhe mosr corrunonca"qe of inllamrnalory anhritis in men over age 30. Gout rarely occuls in men before adolescence or womenbefore nlenopause. Serum urare concentrations rise fiom normal childhood mearl wlues of 3.5-{.0 mgldl tosrrrrh lgvsl5 dring PtrrD' in 1'oung men. ln conhasl urate levels remarn rather constant in *'omen untijmenopaus€. The discrepancv in serum urate levels betu,een tp sexes duing the reprodrctve vearr appean losem from t}le action of esrogeru rrhjch promote renal excretion of uric acid Serum urate let'els il rvomen riseafier cessation of the menses. The disease is uidely distrihxed amongst manr'races.

PURINE BIOSYI{TTFSIS ( KEY STT,PS )

1. RATE LIMITING STEPSMg2+

nbose- S-phosphare + ATP PRPP

5 phosph onbos_r'l- I -arnine

PRPPspthetase

ami dopbospbori boql tran sferase

2 .J .

PRPP+gJuramine:

8 3

2.-SALVACTEPAII{VfAYS

H1'poxanthine + PRPPEGPRT

Gaunine + PRPPEGPRT

3. LIRIC ACIDFORMAT]ON

H lpoxanrhine -----> Xanthiqe

> Inosinic Acid

Gaunylic Acid

Uric AcidXanthine Oxidese Xanthine Oxidase

KEYEn4mes listed in bold Print-PRPP = phosphoriboq'l prophosphateH GPRT = Hl'poxanOrine4aunine Phospboriboq'ltransfera-se

PATHOGENESIS

l. Gour in hurnans arises fiom the species-u'ide lack of the enzrme uricase n'hich oxidizes uric acid roallantoin.2. In hunrars unc acid Ls &rived both fiom the ingestion of foods containing purines and endogenous svnthesisof purine nucleotides.3. Under seadl'sare conditions renal excrerion is the major route of ruic acid disposal. The kidne-v accounts forapproximatel-v to'o-thirds of uric acid excretion. Baaerial oxidation of urate excreled into the gut is tbe majormechanism of ooa-renal dtsPosal.4. Urinary uric acid excrerion is the major adjusable mechanism for mainuining urate hemostasis. The capacitl'of ertra-renal uric acid disposal is greatJl' Iimited in comparison to the kidnet'-5. Increased unc acid production ey rtiminishgd uric acid excretion b' the kidnel' operating alone or i:rcombination have been demorstrzted lo contnlxJte substantiali-t'to the hlperuricrmia of gout

PATHOLOGY

l. Urate cn'stallizes as a monosodium salt in over-saturated.iotnt tissue.2. Decreas€d solubiliq, of sodium untrc at lou,er temperaures of peripheral su-ucfures sucb as toes and €rs rna]'erylain nt-v urafe c4stals deposit in tbese areas.3. Hisopothologi' of rophi ret'eals a chronic foreigrr bo$' granulorna surrounding a core of monosodium urate.4. The inllammatory reaction around fhe cr-v'suls consists mostly of mononuclear cells and giant cells.5. Urare cr]'srals apr ro be direc0y able to inidare and srstain intense auacks of asute inJlammation becauseof their capaciq'to stimulate tbe release of several inflammatorl'mediators.

STAGES OFGOI]T

I . As-vmp omatic Hlperuricemia2. Aorte Gorry Arthritis3. lnrcrcritical Cout4. Chronic Tophaceous Gout

CLASSMCATIONS OF GOUTt. pRIMARY : refers to those circumstances in u'hich eletated s€nrm urate levels or urate deposition appear tobe conseguences of inlrerent disorders of uric acid metabo[sm not associared tvitr anotler acguired disorderand in q'hjcb gout is a promi-nent fearure of tbe clinical picnre.

84

2. SECONDARY : refers to those circumsunces in nhjch goul is a minor elinical fearurenumber-ofieneti c -or acquired'processes. _*=-=--=- seconoan'to anv

3. URIC ACID O\ERPRODUCilON : apprroximarell, l0% of patients witb hlperuncemia or goul excrereexcessive quantities of unc acid rnto the unne. Overprroduaion of uric acid occun uith some fiequenq, rn aYaner.r' of acguired and genetic disorders charaacrized bn' exce ssil'e rales of cell and nucleic acid rurnover suchas ntveloproliferative and llmphoproliferain'e drsorders. henrolrtic anenrias. and psoriasis ( i.e. secondary.gout).

Uric acid ol'erproduoion c:rn also ocorr secondan' to in}ented derangemenE in mecbanisns of endogenouspunne bioslnthesis' h both panial def cieno' of HGPRT and PRPP svnther,ase overactiviry eadv onset goulanci renal urate calculi constitule tlre usual clinical manifesarions. Severe HGPRT deficieno,is associated u,irhspasdo4'. cboreoa*retosis menul reurdation and compulsive self-muriladon ( Lesch-N1,han Sl,ndrome ) ( i.e.pnnuS'gorl ).

4' LIRIC ACID LINDERSECREION : the ruajonq' of pauents *'ith h.r"peruricemia or gour ( up tox)vo) shou,a relatil'e defcit in the renaj esffetion of uric acid \rnuallv all plasrna urate is fitrered b1'the $omeruJ,rq, u,ilbgr€EEr l}En 95o/o of tlre fihered load undergorng proximal rubutar resorption. Subse6;i pr,oxiglal tuhilarsecretion conu-l'hrtes the major share of excreled uric acid- A diminished ruutro secrel;r1' rate may contl-h,rteto hlperuricemia as u'ell as increased tubular reabsorpion or diminished uric acid f.luztion

CLIN]CAL FEATURES

l. Acure gor4'arrhdtis is the mosl common early clinical manifesurion2' The ls MTP joint is involved mo$ ofien and is affmed ar some point in 75o,,o of ptrents. ln approrjmarelyl0% of ptients illerE is never a rccuren€. bul up to 60%oof patiens ex?erience a second a[ack in less than a)'ear.3. The ankle. tarsal area and linee are also commonh,aflected-4' The fim episode of acute gouf'anldds fieryenr]r begilrs abnryJf in a single joint oflen dnng rhe nighr sotlut the padenr anakens u'itb dramatic. unerptainedjoinr pain and swelling

- -

5' Afleaed jolnts arE usrnlly red hot, swollen and exremelv render- Drffrse enlhema mav be presenl u,hichcan be confi:sed s'jrh celluliris or thrombophlebitis.6- Acute atucks of gornl'fihritis rna)'be assocjated n'irh higb grade fever and svsremic qmpoms g,hich mavbe filsely inrerpreted as infection - - -; --7

7' The mosl common sites for tophi are tbe base of the great roe. Achilles lendon olecranon hrsae- knees.urisrs. anrl [3a1[5.8- Approximatel\' l0-2V/" of po.tienls ndth prima4'hlperuricemia de.r,elop uric acid kidrq, son6.9. Renal disease is the most corunoD complication of gout exc€F for t_be arthritis.

RADIOGRAPEIC FINDINGS

I - The earles changes in gout are soft d55us su'sring and joinr effirsions2' Alace panern of erosion ru)'be s€en as an earll'change qitr a fire su-iared panern of periosreal reactionaiong the conex ad3acent to a rophus.3. Bonl' erosions tend lo be round or otzl $'it} a sclerotic margin and have been described as . raltite erosions". cvsl-like. or" prnchedout " erosions.4- A feature d$lngulshlng goul fiom otlrer afihritides is t}e presence of destructive lesions in bone that areremote fiom the anicular surface.5. N4anv of the lesiors are expansile *it} o'erhangrng margxs ( Marrel's sigrr ) tJut are displaced aua1,fromthe axis of tlre bone.6. Generalll' joint spaces are presen'ed until late in tbe course of tbe djsease.7. Alkl'losis andjoint subluxation rna\'occur in ren'adrzlced cases.8. Tophi rnai'be visualized uithin the sofi rissues.

8s

LABORATORY FINDINGS

1--rr'!-Denlonstration-of,s-r'novial Iluid nrortosodiu.m urale-cn'sals-is nou,-generally-comidered nundaton' foresubiishing the diaposis of gou as an expla-nanon of acute arthritis. Monosodium urate crystals are needle-shaped and diqrlal'a negative birefrrngence (bright 1'ellou') 'n,hen vien,ed under a polarizing light microscopeparallel to Ore ocis of slou"r'ibratjon marked on the polarizing microscope.2. Slnovial fluid leukoqtes are elerated ( 20.000-100.000 cells per cubic millimeter) u'ith a predomi-narion ofneurrophils.3. The ralue of serum uric acrd levels in the dragnosis of acute goul is iirnired. levels can be normal al the rrmeofan acule SouR'anadr4.The ESR ( enrluocrre sedinrenution lzrte ) is generallv elerared at Lhe tinle of an acule goutv anad(5. An eleratec! serum WBC counl rnav also be seen u'ith an acu.e anad!

TREATMENT

ACI.]TE GOUT

1. COLCHICINE : a polent inhibitor of inllammation brt also a ven'roxic &ug The mechanisrn of actionappears to be rnterference uitt several seps of the hflammaton response in u'hich neutrophfu pla]- acentral role. Intracellular interference u'ith tJre organization of labile librillar mjcrotubJar systerrconcerned q'ith ceU $ructurE and movemeil ma)' lead to micorubular disaggregation and to decreasedneutrophil motiliq'. chemouxis- release of chemouctic faqors and \'sosomal degranutarion.

Dose: 1-2 mg fV over 2-5 minurcs0.5 mg po uken hourlv until pain reduaion GI toxiciqr-. or rnaL dose of 8 mg

11 h:rs been suggested that Oe promtr response of arthritis to colchicine is diapostic of gout bn ir should benoted thal other artlropathies have been shoun to respond to colchicine- and the response to colchicine nu1' berzriable if it is saned 24 hours afier an acule goun'anack

ARTHR OPA THIES WE CH h,IA }' RESIION D TO COLCMCINE ADMINISTRA TI ON

I

II

I

l. Goul2. heudogour3. Familial Mediterranian Fever4. Sarcoidosis5. Behcet's Slndrome6. Amyloidoss

7. Sweet's S1n&ome8- Serum Sidoess9. H1'dro4zpatire10. Er.vthema NodosumI l. Rheumaroid Anhritis

The oral administration of colchicine mzv culs€ gasrointestinal toxiciry' in up to 8tr/o of patients.r.Naus€a- r,omiting diarrbea and cramping abdominal pain mav be s€r'ere. The gradual use of small repeareddoses is meanl lo minimize GI toxicitl'. The marimum dose should not exceed 8 milligrams over a 24 hourpend

Ar.oidance of GI toxicitf is one of tbe adranuges of inuzvenous colchicine. Wilh proper use the onll'antrcipated side effecrs are tluombophlebitis if not properly diluted and slsi:r sloughing if ocranasaredExcessjve in!:n'enous dooges rnal' prod:ce bone marros,suppression- rcnal feilrx€, m)'opthy. dissemrnatedintrarascular coagulario4 s€t'ere h1'pocalcernia cardiopuimonarl'aresl seizures. and deatlr- The marimumrteilt dose should nol exceed gr%ler ftan 4 miliigrans over a 24 hour pedod Affer intavenous lherag' nomore colchjcine sbould be given for at least 7-10 da1s.

2. NSAIDS : are also potenl inhttiton of inllammation The mechanisrn of actjon appears to be inlu-bition oft-he enzlme cyclmq'geruse. Ia geneml NSAIDS are surted ar the rnarinum d^il]'dosages at lhe fim sip ofan acrtre gour\' "n2ck and graeull'upered and conrinued until the arthritis has resoh'ed Althougb less roxicthan colchicine- NSAiDS may €use signifcant side effecrs im'olring the GI tracl- ki&rers. and hemarologic

I86

s_\'$ems. Among NSAIDS mos ph1'sicians prefer indomcthaciD for acule goutv anlritis. Other NSAIDS uithproven efficaq' include suiindac, naproxell puoxcanl ketoproferg rolmetin sodiurn and meclofenanuresrC'-:::l

3. Conicoseroids . generall)'not reconmended for Frenteral use in ao:te goun'arthritis because the effects areincorsistent and rebound anacks are fiequent upon discontinuation. Intra-articular steroid injeaions are ofbenefit i:t conir.utction u'ith colchicine or NSAIDS. or in parjents u'ho are u:uble to take colchicrne or NSAIDSdue to rarious medical conditions.

4. ACTH : is produced [' the pituiu4' gand and causes release of gJucoconicoids fiom a normal functloningadrenal gland Erogenous forms can be adnulisered u"jtlr eflects similar to corucoseroids-

CIIRONIC GOUT

l. COLCHICINE : generaUl'given as 0.5-0.6 mg b1'mout} tu'o to three rimes rlqih' as a prophvlactic measure.

2. ALLOPTIRINOL : potent inhibitor of unc acid fonuation b1' blocking rlre aoion of the enzrme :ranrhinesxirtess. Dosed as 300 mg po qd/bid

3. LIRICOSURICS ( PROBEMCID. SULFINPYRAZO].IE ) : both increase uric acid excrerion by the kidno.sard effrctively lorver serum uric acid ln'cls.

*** Allopurinol- probeniod and sulfinpnrazone shouJd not be sarted during an acute gors' at12ck Rapidmobilization of uric acid and sudden changes rn plasma urare levels rnal' predi5pose an individual to an acupgouqY anadc

4. CFIANGEINDIET

SI]R G I CAL PRO PE}'I-AXISTo prevenl perioperaril'e acute gout)'atucks colchjcine 0.-54.6 mg oralll'r.i.d- 3 da1's before arrd 3 davs afiersurgen'is recommended

DIFT'f,RENTTAL DIA GN OSIS OF FODA GRA

l. Cnut 8. Osreoarrhritis2. CPPDDisease 9. Tlpe II Lipoproreinemra3. Rbeumaloid Arthriris 10. Calcium H1'&oryapaure4. Sarcoidosis ll. Calcium Oxalare5. Sesamoiditis 12. Paget's Disease6. lnfection 13. Calcifc Tendonitis7. Seroneg Spondvlo 14. Trauma

arthropathies

PATIENTS ATRISK AJYD PRECIPITATING FACTORS FOR @UT

- male potient ujth 1s MIP joint artlritis and blperuricemia- patienrs u'ith a hisotl of uric acid renal calculi- alcobo[cs- chronic renal insufrcienq'- post IV contr'as dye- posl trearnenl for heroalologic m:lignano'- stress. Et?trrn2. or sulBen'

87

CONDITIONS ASSOCI.ATED WTTB GOUT AND H}?ERITRICEMIA

- medicaEonslou'dose sallo'latesdrureticsTB medicationsuarfarilrucotinic acid

- hemopoietic and neoplastic disease- ln'pertetxion- isclternic heart drsease. ald h1'perlipidelnia- acule myocardial infarction- drabetes melliurs- chronic lead poisoning ( saturnine gout )- gllcogen $orage disease- sarcoidosis- psoriasis- cluonic renal insufficienq'- sickJe cell anemia and other hemoglobinopathies- pernicious anemia and polycl'themia vera- hlperparathroidism- obesitl- regular alcohol ssnslmtf,ion- mlxedema- losemia of pregrunq'- chronic belnlium disease- Dourl's srmdronre

CPPD cn'sral disease, also cailed pseudogout" is an inflammarory process whereil crystals are

deposired in rendons, ligamenu. anicular capsuleg srnovjurn and canilage. The incidence of clinicalll'

srqpromaric disease is about one-half tlr,ar of classic gottr'. The male ro female ratio is about 1.4:1. Radiologic

mrdies sbou, a sead' increasing prnalence u'ith age so thar near\' 5O%o of furdivir+rr^ls have evidence of

clrondrocalcinosis ( radiognphic appearcnce of calcifed canilage ) by tlre ninth decade of life.

CLASSIRCATION OF CPPD CRYSTAL DEFOSITION DISEASE1. HEREDITARY2. SPORADIC ( IDIOPATHIC )3. ASSOCIATED WTI}I METABOLIC D]SEASE

HlperparathlroidrsmH1'pothlroidismHemochromatossHemosiderosisHlpomagnesemiaGourAml4oidosis

1. ASSOCIATED WITH JOINT TRAUMA OR STJRGERY

CLINTCAL FEATURESl. Acrtre psardogorn is ma*ed bv inltamnration in one or more joints lasting for se'r'eral da1's or more.

2. These self-iimit€d anacls can be as abnrp in onsel and as se\rere as true acute goU, although the av'erage

atrack is less minful.

88

3. The loee is tbe site of alrnos one-half of all auaclis. altbough hvolvement of nearll, all joinrs, including thelst MTPjoinL has been noted--4:Pr-tffionE s@A sress. acure me(fcal iunessei. arioGunu can cause aorre aru.k "f afljrrius.

-5' Due to botb the inflemmrtor-r' and degenerative fearures of CPPD cn,stal dsease, it rna]' simulate drseaseszuch as rheumatoid aflhritis. ankriosing spon$'iitis. and neuroparhic oseoa1hropthl,.

RAD I OGRAPHT C FEA TI]RESl. The Dplcal app€arenc€ of punctate and linear den-sirix in anianlar hlaline or fibrocanilaginous rissues isdiagnosti callS' helpfuI.2. calcif c deposts ma'r'also occur in anicular capsule. ligaments. and lendons.3. Subchondral bone o'sls and hook-like oseophrnes -rj b.,"ol

LABORATORY FTNDINGSl' *'* The precise diagnosis of pseudogoul is made [' identificarion of CppD c4'srals in joinr lluid Thecn'stals generalll' have a neodle-shaped or rhonrboid ag)earenc€ and displar-posri* uo"fringence *henr.ieu,ed under a polarizing Iighr nucroscope-2' Slnovial fluid leukoq'res nErY be ele\aed ( I.uD-50.000 cells per cubic millimerer) sith a predomirntion ofneutrophils.3. A q'semic leuko$'rosis and elerared ESR may be present.

TREATMENTUnlike SouL oerc is no uzv to effectil'el}'remo\re CPPD cn'srals from the-ioint. Acute anacks in largejoinu can be ueated -bl thorough asFrirarion alone or combrned s,ith iniection of rnicrosn,sullineconicosreroids. NSAIDS are often ven' usefiil. colchici-De given inrave""*;;; .ff;;;:-;;;;;;

efiectiveness of oral co-lct-ricrne is les pieaioable. Bu bo$ ft; number and duanon of acute anacks can bercduced b5' colchicine. It is imponanl to nole tlul rearnent of assocjated diseases does nol rcsdr in resorpionof intra-articuatar CPPD crysal deposia.

NEUR OPA TFI C OSTEOARTHROPA THY

witlrorn an1' appreciable exernal cause we may see, bern,een one day and the nexl tbe developmentof a general and ofien enornous tumefacdon of a limb. mosl commonlv u'iihout an1, pain u,hale\€r- or anyfebrile reaction- At the end of a feu' davs the general rumefafiion disapean- hrt a more ", i*. ""mol'#-.1t'eliing of the joint remains. ou'ing to the fornration of a hydrartlrus: and sometimes to lhe accumulation of aliquid in tlre perianicular s€rous bursae also. on rrncrwe being made- a ranspareil lemon<olored liquid hasbeen frrequentJl' dra$n from the joint.

One or tu'o u'eeks afier t}le irnzsion sometimes much sooner- rhe exisence of more or less nrarkedcracking sounds ma)' F nored- betraf ing the aheration of the anicular surfaces u,hiclr- at this penod- is alreadl,profound The hvdranhus becomes quickly resolved- leaving afier it an exrreme -;tb _; the joint- Henceconsecutive lur;rtjons are fiequentJl' found, rheir podrcron being largell, aided b' r1e ",ear,ng auav of theIteads of ore bones u'hich has uken place, I have several times observed a rapict wasting of lhe m ,scular rru*sesof the limbs affmed b-v the anicutar disor&r... Besides the wearing doun of tlre anicular surfaces... \,ou r6ynotice the presence of foreip bodies of all tbe crrslouuln'accompanunens of an-brius oerormans] i;il1;beDane thar.. thq' are all prrodrcod in an accidental rrurnner- and to all app€nmoes chiefl1'[,oe more or lessenergetic mo\'€menls to u'hich tlre patient sometimes continues rc subject the affected limbrs...-J.Ivt Cbarcol 1868

I DEFINMONneuropalhic osleoaflhropathf inplies disease of nen'es that leads ro underlling bone and joinr abnormalitiesflrs descn-bed b'J.N[ Charmr in 1363

tr. PATHOGNESISExact cause is still rrnl-neqrr hl bolh neurorascular and isuron? rmrric tbeories exist and mql, both pla], acrucial role in the development of this disorder

8 9

Neurorascular Tbeon' ( French Theor5') : darnage to rophic nen€ centers uith an aleraion in the- -- qmparhetic-conrol-oFblood-flow lo bones and jornts ]earis-to persisent'hyperemia and-active-bone

r6orFilon

Neurotraumatic Theol' ( Gennan Theory' ) : An cxtreme progresson of degenerative joint disease

follou'ing loss of propriocegion and potecrrve pain sensauon

M. CAUSES

Diabaes IlcllirusS1'ringoml'eliaTabc"s Dorsalis ( Lues )Ercess ethyl alcoholM1'elomeningrceleSpinal cord injun'Polioml'elitisCMTDiseaseRile]'-Daf' SrndromeSpina Bifi&Congeniral lnsensitiviq'Acrod-r'stroPlucneuroparhl'

i*' Diabctcs mcllirug Syingoml'ctia. and Tabcs Dorsalis at'e tbe thrcc most common causcs of Cbarcot

Joints

Spccific discas€s uitb a prcdilection for the lon'cr cxtrcmitl' :

Diabctes Mellitus - ranal- tarsonletatarsal andMTPjoina

Am)'loid neumpathy - alkle, ursal joins

l4l,slsmsningocele - ankle, ursal joints

Acrod-r'srrophic neuropthl' - ankle, MTP joints

Congeniral insensitirtiqv to pin - ankle- ursal joins

IV. INCIDENCE OF C}iARCCTT JOINTS

** Thc mosl commoD cause of Cbarcot Joints is Diabetes Mellitus **

-5-10"/o of patiens q'ith Dabaes Mellins

lOplo of patiens n'ith Tabes Dorsalis

25o/o o{ wieils n'it}t q'ringomyelia

V CLIMCAL PRESENTATION

>red bot- and su'ollen join(s)>&formed unsable/hlpermobile join(s)

>pAIN rnay or mav nol be a fearure ourng to t}e degree. progression and drration of tle underlung

illness. and u'hen preseil is oflen mild corsidering the amounl ofjoint disersion and desruction tut

maY be evtdeil>rariable neurologic exam n'it! decreas€d sensadon auophl'of inuinsic muscles. ald rlimini<hed

parcllar/Achilles refl oies>r'anable rascular exarn but generalll'prlses are eaqily palpable

>spoilaneous fraaure/disl ocari ons

AmyloidossLepro$'TuberculostsUremiaFernicious anemiaperipheral nene inju4'Multiple SclerosisDejerine-Sonas DiseaseTumor imading nen'eBrarn injuriIntra -arti cular corti ePhenl'lhmzone andindomethacin

90

>&formiq'possible in all three cardinal bog' planes. hu especialll' common in the sagiral plane.(rocker- bouom foot )

6ltil ce-rari ois bVeidigacd/diSl ffirectllony proffinences -

\4I. RAD]OGRAPHIC EINDINGSThe nvo classic radiologic forms are atrophic and hlperrophic arrlropathl'. Arophic arthropathl,isencountered more comlnonly in the upper exremitr'. whereas hrpenrophic arthropathv is encounteredmorc comnonlf in the lower owemi$'. Tbe underlf ing disease does not delermine which formpredomiures

ATROP}N C NEUROPATHIC OSTEOART}IROPAT}ry

>enensi\€ reso44ion of bone ends>osteoporosis is presenl and desrruaion of bone nu1'lead to fracrureVdislocarions>no oseophl'res. sclerosis- fiagmenurion or sofl tissue debris is preseil>upering of the disul aspect of the bones mav be presenl u'ilh a " moruu and pestle " or " pencil-in-op " deformiq'

***a poleniial dtagnostic problem s'hich could be misalien for celluljti:"'oseoml-elitis or an aggessil-e bonenrmor

}TYPERTROPH] C NEUROPATH]C OSTEOARTHROPAT}TY

>jofurt space witl marked bonv sclerosis>osleoporosis does not occur with this form>Facrures and fragmenution of lhe artictilar surfaces>a large amounl of bony sofi tissue debris forms and later firses into a larye. dense. u,ell-organized

!6n1,nressu'ith an integnl oono<

>penoseal ngq'bone formation mz)' occw>subluxation and dislocation proceed to destnrctjon malalimment of anicular surfaceg and finallv lototal >disorganizarion of the joint- u'hich appears as if it were pounded hn'a sledge-hamm

VI]. STAGES OF DE\IELOPMENT

Suge I : ACUTE or DE\TLOPMENTALjoint laxity, subluxation ostcochondral fragmentationand debris formation

Suge IJ : COALESCT,NCEabsorprion of debris and fusion of borry f,ragments

Srage III : RECONSfiUCTIONremodelling and rerascularization of bony fragmenu

*Suges are based on pathological findings- **Dsease course is rariable and progression mav be verl'rapid-

VII. DIFFERENTIAL DIAGNOSISAcltr€ septic artbrhis Pigmented Villonodular Svno'r'jtisOsteomvelitis Srnorial Chondrorr,atosis and other tumorsAmrie Cn'sal Deposition Disease GornCPPD Cn'stal Denosition Disease koriatic tuthdrisOsteonecrosis ( Arascular Neoosis ) Rheurnatoid Aflhiris

9 1

DT PATHOLOGY>multiple shards ( fngmens) of bone and canilage embedded in the deep layer of svrlo\4um

characteristi#findings in aonell'resorbed joinu. bone is replaced bv highll'rascular retinaculum

>bone is aaivell' resorbed $' oseoclasts>rn acule sagesqnorium is edematous. congested and infi.ltraed rrift PMNs>lolrinflamnralo4'and serosangi-nous or hemorrlragic effirsions are fitguentll'descnbed>bone and canilage fragmenrs is u'ell as CPPD cr-r'sals can be idendfied in s-r'novial fluid

X. TREATMENTConsenauve

>sria c€ssation of w'eighbeanng>compression ca$ ( Jone's-tlpe casl ) lo conEol edema>cas immobilizanon for fracnres ( generalll'8-12 n'eeks )>gradual increase to full weig}tbearing based on healing>long rerm accorudative fool u,ear (ii. orthopedic shoes- cu.stom molded shocs. AFO. PTB. CROW)

Surgical>sbould Dot b€ donc during tbc acutc phasc>ulcer excision ( primarv closure if possible: may requirc sktn grafttng )>exoseclomJ'>saucerization>digtal sabilization>meEursal head rescedon>Lisfranc artbrcdesrs>Triple and Pantalar arthrodeses>amFlttion

LYME DISEASE

Lrme dlsease is a complex mgldq'sem illness cau-sed b' rle tick-bonre spirochete Borrtllia

burSdor{eri. The spirochae is rransrnineO $marity by cerrain irodid ticks ( biodes darnmini is the principal

vecror in tbe Norrbeasenr and Midu,estern U.S.; Uoa.t pacifiars is the vector in the Wed )- Ttre illnss5 u'hich

closel-r- rnimics otlrer rheumatic diseases usually' occurs suge! uith remissions- exacerbations and different

clinical nunifesations at each suge. It qas firsl recognized in 1975 in Lyne' Connecticut'

CLIMCAL FEATURES

EARLY DISEASE : Afler an inctbarjon of 3-32 da1's crylhcma chronicum mignns occurs al fte site of tjck

bire rn aboul gflo of patienu. Although rhe lesion czrn occur anlu'bere- the thigb' groin- and axilla are

p"rri.,rt"a, cornmon sires. The lesion is \arm to lhe touch and is often painless. Wi&in ss'eral da1's afier rhe

f'ser of eryr;ema chronicum mignns 50plo of pariens dwelop multiple secondaT lesions u'hich may peEist

fiom I Aay fo 14 montbes. Tnese tes;ons are similiar in appearance lo enttema chroniom migraN hn are

u-sull1, smaller, mignte less. and lack indurated cenlers. Skin involvement is commonly accompnied by lou'

grade fever and lynphadenopathy-

INTERMEDIATE DISEASE : Genenlly occurs approximatell' l-6 montbes afler tbe initial eryosur€'

Neurologic abnormalities include meni-ngitis. cranial n*;us. motor and s€nso$ radiculopathy, mononeuritis

muldpei alone or in various combirurions. The mog conunon cardiac abnormaliS'is lluauadng degrees of

arriorienuic'lar blodc Less commonly peri- and myo carditis ma)' occur- Tbe qpcal panern of anhrhis G one

of nugntory pain in jourrs, rcndors. hrsae, muscles and bone'

LATE DISEASE : Occurs ntonthes to ],ears after initial exposue. The mos{ cornmon form of chronic CNS

irn,oh,emenl is a sutnle encephalopathf in'ectrng memory, moo4 or sleep. Most of these parients also have an

arooaf po\neuroparhl, *rrrli'.oi b-r, ii0rer drstal p"nsthesias or spinaVradicular pain Aborl 6o7o of untreared

puo.oo urle u.s. o"rcrop mn5 anlrl*.]b. tiprot panem of i-nvolvement is brief intermiuent auacks of

rrronoarticular or o[goarticuJar anhritis in a feu'large joints especially t-be }cree'

o ?

I-ABORATORY FEATURES

Serologic tening is currendv tlre onll praaical laboramr-r' aid in diagnosis. IgM antr-bodies peak atapproxmatell' -36 n,eels: IgG antibodies peak monthes lo l-ean after e,r?osure. Dreo blood CSF. srnovialfluid and skin lesion cultures are rarelvpositive.

TREATJI{ENT

EARL}'ADULTDoryrycline 100 mgpobidTetnacvcline 250 mg po qidAmoxicillin 500 mg po gidCHILDRENAmoxicillin 50 mglky'daf in divided dosesEn'thromlcin 30 mgkgldzt in dn'ided doses

NEUROLOGIC ABNOMALITIESCeffriaione 2 g/&1'IV x 30 daysPenicillin G 20 million unitg&r'in divided doses

A TR] O\{ENTRJ CI]LAR BLOCKIrnal'enous anb-bi oti csC-onicosteroidsCardiac monitonng

ARTHRTTISOral anubioticslnuaanicular conicosteroidsNSAIDS? qnoveoomf if srmpoms persis > 6-12 montbes

*** Du'ing the initial 2,1 houn afier anubiotic therapry is sarted 1O-15o/" of patiens experience a Jariscb-Eenbeimerreaction charaaerized b-\' high fever and u'orsening of s\TlForns.

LYIVIE DISEASE: TIMETABLE OFA TRIPEASIC n I NESS

STAGE

STAGE I :initialu'eeks of illness

STAGE2:weeksromonrhes afler suge I

STAGE3:montlesto1'ears after sage I

SIGNS AND SYMPTOMS

constitrrional $'lrtFornserytlerna chronicum migrars

recutTefl skin lesionSneurol ogi c manifestati ons

cardiac abnormalities

aflhdtis

93

-f eaffi -i@#ffi*+p"-"prrEdlou*;rueaiseas*sidOnnro,ff eo:nd-plE_-

orgax s_\,slems in addition ro the joins. The average age of or*i fot arirlts is zl0 years of age, tut the disease

nur. $rike for ilre fim dnre al an\.age. Tlre disease-t"nds to aflecl u'omen slighul'nrore cornmonll'tlun nten'

nmum-lrom eBrrnmsRheumarord anhrins rs a q'sremrc auloimmune disorder of unknown etiolory. The ma-ior distinqf

_-- 6::::-:::i=--i:

PATEOLOGYRhzumaroid arrhritis is a anorial disease uith seconda4' parhologic changf raking place rn the

svno*ial fiuid- cartilage. perianicular srructrlres. and rendoru. Attlrough dau are lirnired the irritial patlologic

;i;;;;;; ro * icoi"tion and/or injrul' of rhe qnovial nucrouscular endotbelial cells q'hjch leads ro

edenra in qno'ial ussue and effrsiors lrlil"r rhe jornt caviq'. slnovitis is lbe initial lesion As the disease

progresses to chroruc Sages tlre qnorium bccomes uussivell' hlpemophic and edernatous' The formation of

;;;ff"*d". and inrasive"granulation tissue defined as pannu leads to pcrianiariar bone and cartilage erosions

and destruaion. There is no lstou'lt eriolog"'

CLIMCAL FEATURES

SYI\4PTOMS

- The mode of prresentation can be guite rzriable ( indolent" episodic. or progressive poll'arthralglas )'

- Monoanicular. oligoanicular. or poll'anicular-ioint imolvemeil ImI occtll-- C-onstirutionat qripoms such as nralaise- fatigue. low-grade fever. anroiia- and weigbr loss maY

occur.- Stiftress. parucularll' afler prolonged resl is usuallY severe and lass nearll'60 mrnutes or more'

S]GNS

ARTI CULAR MANIFESTAN ONS

- joint sn'elling and perianiolar tendernes<- local sarmth over affeaed joins- limiution of motion of affected joints- qmmetry ofjoint irrvolvemed- firquenil1, affeoed joinrs include : MCPAvfTPs- PIPs- urists. Imees. shoulders- hips- elbons. anldes,

midarsat joinrs and cervical spine- rcnosrnovitis- deformines ( suan nech boutoruriere, hallui rzlgus. hammenoes. ankle rzlgus. fibular/ulnar

def iation of digis )-calcanealerosons,planurfrsciitis.retroca]canealhrsius

EXTRA-ART] CULAR MANIFESTATI ONS

l. Rheurnatoid nodules : tend to del'elop over pressure points. Rheumatoid nodrles have cba'racteristic

hisoparhologc -

finding u'hen biopsy' is performed- A cenu-al zone of necrosis is surrounded S'

palisadi:rgtioio.lno. -

fibroblasts ud monoclnes. The outer laYer of inflalru'natonr granillation

tissuerscomposedoftlrnphoq'reshisiocytesandpasrnacells2. pulmonary' : the lungs are commonly afieoed $' Rheurnaroid aflhdtis. Commmon compbcarions

include:- pleurisv uirh or uithoul effisions- dift$€ inrerstitial lung disease- Prlmonarl' rheumatoid nodiles- C.aplaa's qndrome ( nodules nitl pnenmoconiosis )- pJmonan' r'asculitis and hlpertersi on

t

tlIlIITIlI

TtIttlttt94

3. Vasculitis : fie mosl conunon organ $'st€rns affeaed are the crilaneous nen'orrs: gasooinrestinaland pimonary' $'slems. Leucoqloclasric angiitis. nail fold infaras. and spliner hemorrhages are-exarnples

of tte-nnal venilifrs---wlila[ mav occur.

4. Cardiorascular: all la'r'ers of lbe hean mr1'be involved Pericarditis is the most corlmon clinicalmanifestation-

5. Hernatologic : mosl patients u'ith clinicelll' acdvs anhritis have anemia of chronic disease. Anotherhematologic rnanifesurion is Felh/s sndrome ( riad of neurropenia, qplenomegall-. andRheumaoid arthritis ).

6. Neurologic : multiple neurologic compllcations ma1'de'r'elop in patienrs n'itr Rheurnatoid anhritis.- m1'elopathl' caused b1, subluration of cen'ical venebrae- peripheral neuropathies

enmpmenl (i.e. tanal tunnel *rrdmme )penpheral sensorJ' neuropathlsensorimoror neuropatlry'( rnay be dre to'r'asculitis )

- aulonomic dvsfuncrron ( pimar/planur en'thema )

7. Ocular : the eyes are also commonly afeaed- keraroconiunctivitis sicca ( dfv e,ves )- episcleritis and scleritis

RADIOGRAPHIC FTNDINGS

L Articular : articular manifesutiors of RA tend lo predonrirute in rhe small joinu of tlre feet and r*rists.

- sofl tissue su'elling u'hich ma1'be secondar-r'to :perianiailar edemajoint eftrsionshlperplasti c srnot'iti s.

- subchondral oseoporosis ( osleoporosts ofjurra-anicular bone is one of tlre cardinal diagnostic crireria forRA ).- ttwginal joid erosions ( erosions at tbe MTPjoinG mosl comnonll'occur al the plantar medial aspecr of the

mesursal heads.)- even joint spe narrou'rng--ioint deformities and sublurations ( marked trallu{ lalgus nirh fibular der"iadon of toes 2,3.and 4 ).- anhritis mutilans.- subluxation of the fim and second c€rvical vertebrae.- lar ch"nges lead ro bony'an$'loqiq- fieSueillv in tbe rarsal bones.

IL EXTRA-ARTICT.JI.AR

- resorption of tbe distal clavicle.- generalized oseoporosis ( associated q'ith disease duration and severin-. menopause, physical activiq'- anddrugs sucb as conicoseroids, ryclosporu1 and mahorerare ).- sress fracnres oflongbones.- Achilles' tendon thickening- erosions may be seen al the poserior and planur aspects of t}te calcaneus. and u'ell marginared planur heelspurs arc oflen presenl

95

ABNORMALTTIES OF SYNOVIAL J OINTS INRHETI]\TATO ID ARTHRITIS

lt

PATEOLOGIC

svnor"ial hflarrunati onand fluid prod:cnon

h1'peremia

pannus destnraion ofcaniiage

pamus desruction of"unprorccted" bone almargins ofjoints

pannus desnuction ofsubchondral bone

laxit-v of capsule, lig-amenls and musoilarconrraction/spasm

RADIOLOGIC

sofl nssue swelhng andwidening of joint spac€s

osteoporosis

narrorring odoint sPace

margnal bonl'erosions

bonl' erosiors and formationof zunchondral qsts

deformiq', sublursti ondislocation fiacnre.fragmenution andsclerosis

tlttttrj

IIrtItt

THE A]I{ERJCAN REEI]MATIS]\I ASSOCHTION 1987 RE\IISEDCRITERIA FOR TEE CLASSM CATI ON OF REEI.I]T'I.A TOID ARTHRITI.S

l. Morning stiffncss : morning stiftress in and around Ore joints lasing al least one hour before maximal

improvement.

2. Artbritis of 3 or more joint arras : al leasl 3 joinr areas bave had sofl tissue svelling or fluid observed by a

pblsician The l4 possible ar€as are right or lefi PIP. MCP, urisl elboq',loee- arikle- and MTPjoints.

3. Arttrritis of hand joinrs : at least one arsr su'oUen ( as defined above ) in a uriE- MCP- or PlPjoint-

4. Slmmef ric arrhritis . sirtuhqneous involvemenl of the same joifl areas ( as defined in 2 ) on both sides of

the bodl'i Uitaremt inyolvement of PIPs- MCPs- or MTPs is accepable withoul absolute s-\nuleq\').

5. R-beumatoid nodules : subcuuneous nodrles, over bony prominences. or e)ilensor surfaces. or rn

juxraanicular regonq observedbS'aph-vsician-

6. Serum rbcumaloid faclor : demonstratjon of abnorrnal anounts of serum rbeumatoid faoor b1' any method

for qrhich the result has been positive in < 5o/o of normal coilrcl subjects.

?. Radiographic changes : radiographic changes ti"rcal of rheumaroid aflhdtis on posteroanErior hand and

*risl -raOograpl,es.

o'tti.tr must inclu& erosions or unequivocal decalcification localized in or mosl markd

adjacem ro tbe irn'olvedjoint ( oseoartbritis ch2nges alone do not quali$ ).

*** For classifcarion rqposs a parient sball be said to have rtermptoid arrlritis if hdsbe has seti<6ed 4 of

these 7 crireria. Crireria l-4 mus have been pres€nl for at least 6 q'eeks. Patiens nith 2 clinical diagnoses are

not excluded- Designation as classic. definite. or probable rheunraoid aflhritis is Dot lo b€ made

96

LABORATORY FINDINGS- elerated erylhrcc1'te{edimenution rate (ESR)--- anemia of chronic disease.- irtllarnmaton' snwial lluid- elerzted uters of rheumatoid faaors in the serum of approximatell'85% of patienrs with RA The rhzumaroidfaoors tend to correlate n'it} severe and unremining disease. nodrles- and exua-arucular di.eece. The mosconlrnon rheumatoid faoor is IgM anubo{'to IgG antibod-r,.

SF.I -FCTED DISEASES ASSOCIATED \ITTE ELEVATED SERITMREEI.NIATOID FACTORS

l. Subacute Baaerial Endocarditis Z. Sarcoidosis2. Irproq' 8. Lir.er disease3. h.rhnorra4'lnterstitial Disease 9. Tuberculosisa. Slphilis 10. Lrme dsease5. Rubella I l. eromegaloyirus6. Infectious Mononucleosis

DTSEASES SEEN IN ASSOCIATION \\'ITB REET]MATOID ARTHRIIIS

l. Sjogren's Slndrome2. Amloidosis3. Felqrs Spdrome - classicaltl'described as a riad hn rra)'include the fo1osing :

Rheurnatoid aflhdtis leukopenia splenomegall,. leg ulcersllmphadenopathl'. and thrombocyopenia.

TREAT�TENT

l. PlTystcAL MEDICINE ( REHABILITATION )

Ph1'sical therapy : goals ilclude improving muscle tone. preventing or correcting deformjties andmaintaining joint mobiliq'' and firnction. Ph1'sical therap-v- is also verJ' imporunr duing the perioperatives€ning

Ocnrpatjonal therap' : goals include assising patients in ad.apdng to tre acti\ities of dai\' litdng (ADL's ), to the limitations of the dise.:se, and supli'ing splinls 3nd rqsi<riys devices to ajd in self care.-

2:MED]CAL TREATh,IENT

Arailable medications cal be considered il two groups : tlrose rlut have the polen'irl for qmgomaticrebef. aod tbose rhet harr the porential to modifl'tbe disease.

97

SYVTPTOMATIC MEDICAL T}IERAIIf :

AspkrnNSAIDSGlucoconicoidsDMARDS ( Disease ltlodifldng AntiR]reumatic DrugS ) :a.ka. SAARDS ( Slou'Acting AntiRheumatic Dru-eS ) :Auronofin : oral gold PreParanonParentenl gold saltsAzothioprine ( lmuran ) : oral crotoxic prine analogHvdroxl'chloroquine ( Plaquenil ) : oral antinralarial compoundMethorexate ( Rheumam>i ) : folic acid anugorusPenicillamins; enrleg of tbe:mino acid c.r'neine

3. SURGICALTREATMENT

SOFTT]SSUE SURGERY- svno\iedom]'n eno$Tovectom]'- release of nerve enFaPnrent- e:rtirpation of rhzumatojd nodules- burseaoml'- rcndon balancing/relocati on- releasc ofjoint contracnu€s- nrarup:lation ofjoints rrnder general anesthesia

OSTEOTOMYARTHROPUISN,+L- rcsection- fascial inrerPosition- metallic bemiart-broPlasS'- pl astic-i sili cone imPlant- roul joint artluoPla*v

.4RTITRODESIS

4. SPECIFIC PROCEDURES FOR FOOT SURGERY

Modification of procedures of Bueler and Ma1'o : resection of the fim meraursal head

Eoffman : resection of metararsal heads 2,3'4-& 5Keller : resection of the base of the first proximal phalarutGocbl : resection of the base of proximal phalanx 2,3,4-& 5Clal'ton : rEsecrion of the base of proximal pbalarui and head of meutarsal 1.2-3-4-& 5

Flint and Sllertnam : amFrution of the forefoot

5. INC]SIONAL APPROACHES TO PANMETATARSAL HEAD RESECTION

Hoffman : transvers€ cuned plantar incision jus behind the u'eb of the roes

FonJer : resection of a planur slcin uedgeClalron : single dorsal uarwerse incisionLarmon : three dorsal longirudinal incisions

- over lhe first metatarsal- berq'een Beuratsals two and three- beru'een meuursals four and five

Ntarmor : fye dorsal longitudinal incisiors centered over the MTP joins and exended onto lbe loes

r

It\

r)

)\

7\

I\

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98

STIR G I CAL CON S IDE RA T] ON S IN' PA TIEI\TS \\TTE RHEI]MATOID ARTHRITIS

t rU@ e nr e n u t i o n : l{-patj ennvho has recei tA ex oe€nou ssterCIaG \\r thln-bnevear of suryetl' mzl'dglrslsp sudden b)'potenson during surgcal sress. The mechanism is based on supresionof ACTH secretion Chronic steroid therap' rm)' be associared rvilh adrenocortical arophv and adrenalrnsuffcienq'. Several methods of suplementation can be emdoved

Hvdroconisone sodium succinal€ 100 mg IV preopl 0 0 m g I V q 8 H r x 2 4 H r

Decrease br\' 5096 each dav andresume oral medicaoons

Solumedrol 40 mg IM pm before surgerl'40 mg IV dring surgery'40 mg IM nighl of surgerl'2O mgIM am and pm POD # I20 mg IM pm FOD # 2 and then resume preop rourine

2. Aspirin or NSAID discontinuation : Aspirin and NSAIDS should be soped ar least 7 days pnor rosurgel'-r' rn order ro prevenl bleeding complications. Aspidn irrn'enibll'acen'lares plarelos inrerfering wirhplatelet aggregarion and prolonging bleeding time. NSAID inhjbition k reversible and persisu on\'u'hile thcdrug is presenl. heoperative laboraton'evaluation should include a bieedurg time, PT. PTT. serum creadrune-and LFTs for patients on long-term a5pirin or NSAIDS.

3. \\'�orkup and propbl'laris of atlanto:r-ial subluration : Arlarnoaxial subluxation ( Cl{2 ) ispresenl in 4O4U/" of patienu tvith rheurnatoid arthrift. and marked flcxion of tlre neck d:ring patient u-ansponand anesthesa ma)'€use severe neurologic disruption ( compression of C2 nerve rool, posterior columndvsfunaion or uhimatel-v quadraparesrs or quadrapalegra ). Subaf al strbluxation rna]' be seen in up lo 30olo ofpadcnts u"ith rheumatoid anhritis. Because there is a poor correlaoon bent'een s\TnFoms and pathologicfindings. it is adtised lo otnain lateral cen'ical spine Xral's in flexion and errension before undertaking generalanestlresia on arn'patient qith rheumatoid arthritis > 5 1'ean duratjon Frequentl.v- a sofi cervical collar or otberform of cen'ical fixation is advised n'lren ilrese patients are being lransporred and operated upon

4- Protcction of skin : The skin tends to be I'en' thin in prients lf i1} lfisrrmeroid arfhritis. The follouingreconrmendaDons can prevenl untouard damage to the skin :

elecu-ic-q'pea. Do not shave the skin because it can be easilr' damaged by a sharp razor. The tr5e of an

razor is safer.b. The use of plasic adherenl drapes ( i.e. beudine impregrured barriers. Op sire ) is

contraindicated because the skrn ma)'be torn q'hen the drapes are removed-c. Mgorous scrubbing of rhe skin is also conrraindicared

-s. Incrcascd risk of infection : O'n'iag to immunologic defecu in both humoral and cellular mechanisms ofhos deferse. paDenE u'ith rheumatoid artlritis have an rncreased susceptibilitv to infection. Tbe risk isaiso increased in patients treared u'ith chronjc corucoseroids. lt is imperauve that serile technigue isemplol'ed in performing inrzsive procedrres sucb as insenion of peripberaVcentral venous acc€ssdericer drring prolonged surgeD'. and drrirgjornt arthroplaq'u'irh implantarion s'ith artifcial joinrs. Thebenefit of prophi-lactic anubiotics is u'ell recognized

6. Dclal'ed n'ound healing : Conicoseroidq penicillarnine. and me*rorexale mal'conrr'hne to delal'edu'ound healtng Anatomrc dssection and gentJe maniprJation of the skin and soff tissues u'ill help to enhanceu,ound healing and decrease the risk of posoperarive complicatiors.

99

TtT.Temporal-ma-ndibularardcricoarytcnoidabnormalities:

SH7lotoof pa.r:ents qi$ rheumaroid arthritis have pathologv of the Tlvlfs. Two main areas of concern are l)

limitation of-mandibular opening rlur-will-prevenLendotracheal innrbtisn-9-loss-ofupper ptnryrn

,-."y space . u,tricb can resrtr in complete airnzl' obsrucdon u'ith sedation or aneshesia. Dffcult c"ces

;. ;d* inrubatjon for general anesthesia bv a fiberopic intubating bronchoscope.

A1lui6 of the cricoa-rnenoid joints is seen in up to 25V" of patients with rheunratoid arthritis'

Arrhriris produces progressve nr^ottrg and fixation of the vocal cords thus compressing the airwal''

Follon,ing a long pt*fo* n'ith endotracheal innrbatiorl e\tlbadon must be performed n'ith greal cauuo[

,i"o ,r*-U-g oitb. alrcadv comprornised airual'rra1'result in complete ainal'obsm"lction in a ptient $'ho

cannot be easih, reinrubated- For this reasorl a ruuro$'diameter should be chosen $'hen selearng an

endoracheal tube.

5. D\T propbl'laris : Fatjents uith rheun'ntoid arthritis m4'be al incteased risk for developing a

DW depending on their lwel of mcapaciunon. Minidose heparin could be

preoperativety on an1'patient undergoing suge4'u'ho mav be bed ridden or incapaciuted for al

errended perid of time posopeiatively-

OSTEOARTHRTTISrDEGE\TRA TT\IE JOINT DISEA SE)

-nlost common joinl disease characlerized [' progressive loss of anicrrlar cartilage- alpositional ne$' bone

formation in tlre subchondral trabecutae. and formation of neu'cartilage and new-bone al the joint margirs.

-tlre exact nrcchanisrns b1'which prinarv oSeoarthritis develops are unlmoqar

-secondary oseoarr}ritis is app[ed ro the disease u'hen it appcars in response lo some oven lo€] or svslemic

patholog'

PATBOLOGY

-rn earfier sages the cartilage is thicker t]ran normal. An increase i:r szter conlent leads to sw'elling of cartilage

and an -"tor" in rhe net rate of proreoglycan slnlhesis. The increase in proteoglycan $nlhesis is an anemp

al reparr ['the chondroq'tes.-$,ith disease progression the joinr surface rhins and proreogll'can srnthesis diminishes. The iltegriq' of rhe

surface is lost and verdcal clefu develop leading ro oPosed bone-

CLINICAL FEATURES

S\}{PTOI\!S6*r-t1. disease occurs aier joinr use- paniorlarlr afier prolonged activit-v of the joint and is relieved

b'rcst.-as the disease progresses pin occun even al r€st-stiftress. rlprcally on arising and afler perids of inactiriry- is usualll' mild and lasts less $an 15 minutes.

-muscle spasm or pressul€ on n"-.s may result in more paln l}an the pnmary joint pain'

S]GNS-usr--euic lenderness and pain on joint range of motion-1o-i .nr"rg.rnent primariJl'due to bone prolifention, sp:rs. secondanv chronic snovjns- or eftrsiors.

-pain on passive range of motion and crepttus

trIITII1

tItTt

subchondral sclerosissubchondral bone q'stspossible joint micefloose osseous bodies)

ttli'

RAD]OLOGIC FIhIDINGS

nrai'appear normal in ea{'sagsuna'en joint space narro$ingrnaryinal osteoPhlres

1 0 0

LABORATORY FINDINGS-nonspecific-snovial-fl uid srudres reveal-urudnulabnormalities--lab m:dres other tha-n radiographs are heipfuJ primarill rn exclurling otherjornt drseases

CLASSMCAT]ON

PRIMAR}' OSTEOARTHRITI S

- euologl'is unlooln: spcci-fic joints im'olved

FIANDS: DIP Jointg>>Heberden's nodesPIP JorntP>>Bouchard's nodesI s Carpo-meucarpal jornts

FEET: I st Meularsophalangeal.fointsKNEESHiPSSPINE

SECONDARY ARTHRITIS-appiied to disease u'hen it appea$ in response lo some oven local or svstemic pathologr'

I traurna(pos-traumatic joint malalignment)2. metabobc or endocrine disorders

-ochronosi s. hemochromalosis. acromegall'. Wilson's disease3. cl-rstal deposition disease4. neuropathic oseoaflhropathl' (Charcot joints)

]\'.ANA G EMENT AND TREATMEI\"7

i. GENERALPROGRAM-paDenl edrcauon-I€ST

-phvsical tlrerapr.- heat/ice. exercises garl trainingoccupatt onal tlrerapr' - splints, joint protecti on assi srjve devi ces

2. DRUG TP€AT\,IENT-analgesics(aq:irin= acetaminophen)-antj-inJlammaro4' agens(NSAIDS. intra-anicular conicoseroids)

3. SURGERY<orrecti on ofjoint malalignmentdebridement of loose osseous bodies and sprrs{$ eotom}' to redi stn'lrrte joint forces-arthrodesis-pamal ortotal joifl replacemeil

PAGET'S DISEASE OF BONE ( OSTEITIS DEFORT{ANS )

Paget's disease of bone ts a condition of udgronn etiologr' *ut occurs in approximarely l -1olo of thepoprlation over $e age of 40. lt uas fim described b' Su James Paget in 1877. lt hes uriabte clinjcalmanifesutiors and mosl czls€s are asrmptomatic. The drsease is charaaerized bn, excessit'e and abnormalrenrodelling of bone. It is more conlmon in nules tlnn females. Aeair tle etiologv is unlgrown htl mosterperimental eridence suppons.....-.-.--." a slon'h'developiag rrral infection of oseoclasts ". as a cause.

1 0 1

SKELETAL DISTRIBUTI OIi

- predilection to involve the arial skeleton 1pelvis. sacmm sprne. and skull ).- also irvolves t}te long bones such as the femur and tibra

( lesioru of the fibula are rare ).- location is srmilar to skeleul melasases: hou'ever the relative fiequenn' of tibial involvemen! spanng of theupper limb. and tnfregueno'of rib inrolvement are fearures of Pagel's disease not shared b-r'skeleul metastases.- tnne invoh'en'renl is poll'osotic in mos cases: ma)'be inidalJl' or louIv monosoric in l0-20o/o of cases.- invoh,ement of the foot is relativel)' uncolnmon rvrtb the calcaneus harang the highes rate of invoh'emenl 1foot invoivement is noled annvhere from 3-16-3%oas noted bv bone scanning ).

RADI OGRAPHI C FEA TI.]RES

- fearures can be explained b1'the underlf ing pathologic prccessSTAGE I - osteolltic phaseSTAGE II - mired pharSTAGE III - sclerotic phase

- earl)' suges mal' nol be visrralized on plain films depcnding on the arnounl of resorpuon bul bone scanning isven'helpfirJ ir detecting earlv osseous involvemenl- late stages are usualJl'u'dl visualized on conventional radiograpbs.- long bones display the following fearures :

1. INCREASED comcal u'idh2.INCREASE in bone length3 coursening of trabeculae4. bonv enlarp.ement

I-ABORATORY FEATTIREJ

- elevated serum alkaline phosphause- incrsrsed urinan' bydrorlproline

COMPLICATIONS

- osseolls deformitres ( bonrng of long bones bonv enlargement )- pathologic fracnrres- malignart u-arsformation ( fibro-. chondro-. oseosarcorns arrd Eu'ing's sarcoma )- oseomvelitis- crysal deposition- neurologrc abnormalties seconda:-r'1o osseous enlargemenl or fracture uith subseguenl nen'e irn'olvemenl- degenerative .l oilt dr sease- higlr output cardrac failure atd cardionregall' secondar-r' to rruuro\\/ exparuion and rncreased blood volume (seal srndromes )

TRI.ATI\{ENT

- sr:nptonratic sith a:ulgesics and appropriare bracing- calcitonil- disodrum etidronate- nutiramf'cin

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SER ONEGA TN|E SPONDYLOARTHR OPA THIESThe seroneganve spondrioarthroq$u:s are an interrelared ggp_o!_UdlqSte1n inflammaton.

disorders. As rheumadc disorden. thq'afrea the spine. peripheraljoints, periani",,tar sr-cnnes. ot aff iirEand thel'are rariabll'associated u'i& charaaerisoc exta-anicular rnanifesutions. The laner inciu& acne orchronic gastrointestirnJ or genitourinary inflammadon sometimes dre to baaerial infection; and- uncommonir.lesiolu of tlre aoruc rool cardiac conductjon s.\'$enl. and pulmonan'apices. Mos but nor all of illese disordersshos'a.n tncreased preralence among indil'iduals s'ho harre inherired the HLA-B27 gene.

RXCOGNI 7FD SEROhIEGATT\iE SPONDYLOARTER OPATHIES

l. Anlg'losing Spondvlitis2. Reireds Sgrdrorne3. Roriaric Anhritis4. Enreropathic Spondvlitis

( Crohn's Disease and Ulceiatir-e Co[tis )5. Reactive Arthritis

PATEOLOGY

I. Sacroiliitis is the patlrclogic lullnutk and usuallv one of the ear[es parhologic manifesutions ofanlvlosing spon{ylitis. The initial lesion in the spire consiss of urllammarorS' granglation tissre at the junctionof the inrcn'enebral disc and the margin of tlre venebral bone. The ourer annular fiben ma1, e'ent'all1, bereplaced bY bone- forming a qrdesmophrre. hogrcssion of this process ieads ro the bamboo spine obsencdradiographieqlh'. Similar adaj pathology Gln occur in other qpondrloart-luopathies as rvell alrbougb uith somedifference.

2. The pothologv of periphenl joint arrhritis in ardaosing spon{'litis car shou, s-rrovial hlperplasin, lyrnphoidinfiltration and parurus formation Sirnilar qnovial pathology can be seen in the ot1,er chronicspon{'loart}uopathies- although Reirc/s svndrome rfpicallr- shows manv more polrmorphonuclear cells inearh'disease.

3. Enthcsitis, inllammation a sires of auachment of tendinous and ligamenrous atuchments lo bone, is anolherpathologic hallmark of the qpondl'loarrhropthies. In ankvlosing spon{'litis il is especially common al sireslocalized around tle spiroe and petvig u'bere it mav evennrally undergo oirifcation ln the otherspon{'loarr}roparhieq it is more colrlmon ar peripheral sites such as ttre calcanqal anectunsnt of t}re AcNlles,tendon

H 1"4 - B 2 7 A SSO C U T] O N S .LVO N G TH E SPO N DYLOAR THR O PA TH] ES

DISORDERSAnkvl osing spondvlitis( n'it}t ureitis or aoniG )

Reactive arthriGand Reite/s qn&ome( rvith sacroiliitis or rn'eitis )

Idlammaton' bos'el dis€as€

ft6daqis tulgaris( uith peripbenal ardritis )( u'ith spon{,liG )

NOrrnal gggcrsi:ns

ELA-B2? EREQUENCY,Wo

near\'100%

ffi-8e/o

nol indeased

not increasednol incrssed

50o/o

6{P/o

( uit}t peripheml arthritis ) nor inoeased( u,ith spondl'litis ) 5U/o

1 0 3

REITER'S SYNDRO]\{E

- -Reiters's-s-vndrome-nas first described by-Hans-Reirer-in-l9l6lt-is-the-most-conmon-czluse of-aninfammrlon' oljgoanlropathf in 1'oung males. lt $s5 x2dldsnqllv sured ftat the rnale to feurale ratio uas20:1. trrt realisticalli'the rado is closer to 5:l. The classic u-iad in Reirer's srndrome consins of non-gonococcaluretirritis. cordrutctivitis. a:rd artlritis. It has becorrre ciear t}at Reiter's slrdronre develops in a genedcalhsuscefble host follonrng a genitourirnn'or gasroinlestinal infection lr is grou@ nrft the other seronegativesponQvl oarthropttues.

PATEOLOGY

The pat}ologic changes in the snovitun are non-speci-fic ( edema- r'ascular congestion and rnf.lu-ation u1ttneurophi-ls. llmphoqres. and sparse plasma cells ). Osteolvsx and perioseal neu'bone formarion are olherfudi:rgs.

CLIMCAL FEATURES

ivTUSCULOSIGLETAL

Aflhritis t)prcalll'begins one 1o lhree u'eeks afier the inciting uret]riris or diarrhea. Joint mfiness. mlzlgias.and lorl' back parn are Fominent erll' features. Tlp,cally onb' a feu._ioints are involved in an asrmmetricfashion : the lglees. ankles. snrall jornts of the feet and tbe qrists are nto6t commonlv affeaed- Joints areusualJy only moderatelv srvollen but tender, stiff and timired in range of motjon. Consrinujonal $mp.oms areusuallv mild and fever, if presenl is usuallv lou,grade.

UROGENTTAL TRACT

Men erperience increased frequenq' of and trrming drring urirntion Eramination of the penis reveats meatalen'$erne and edema' and a clear mucoid drdimge cen be expressed- Fosarids is common and has beenreponed in up to 80o/o of male parients. "Silenl cystitis or cen'icitis" u"ithoul urethritis nu1, be rhe onllurogenital irn'olvemenl in females ufiich accounrs the high male to female ratio fiat \ as once reponed forReirer's qndrome. CNamvdia thrachom:G is a suqreoed patlrogen for r-be deyelopment of Retter's srndrome.

EYE

Upto 4U/o of patients demonsu-ate 'ni- or bilateral non-infectious con-iunqiyitis uhich most ofleD occ*1 earlr.rn the course of the disease. The coniunaivitis is usually mild and ransienr.

MUCOUS MEMBRANES AND SKIN

There are tq'o charaaeristic lesions associaled qith this srndrome.Balanitis Circinata : srnall 5hallsla' ulcen of tbe glanq penis and urethral meatus.

Keratoderma Blennorrhagicum : H1'perkeraotic skin lesiors thal begtn as clear vesicles onenlhematous bases and progress lo nracuies, papies, and tlen ro small kerarodc nodrles. Lesions frequenil1.involve tre soles of the feet- but mav involve Ore toes. palms. scrotrux and scalp. The lesions are seen inapproximarely l2-l4o/o of ptients.

GASTR OINTESTINAL TRACT

The precipiuung episode of diarrhea is often mild and transienl bur ural' be bloodv and prolonged_ Enrericinfecrions b1'boeria such as Shjgella dvsenteriae or floineri Salmonella enreritidis or qphimurinrrm, yersiliaenterolitica or pseudorubercuJosis. and Camp'lobaaer jejuri rnal'pla1' cnrclal roles in the de'elopment of thissrdrome.

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HEART

In--l0o/o-of- patienrs-litlt -s€r'ere-and-long-sunding' disease.--aortic regurgitation develope -as -a-result -of -tnllammation and scarring of the aonic ralve and lalrular cusps. Conducrjon defecs sometimes occur earlv inthe course of rhe disease. t}te most corrrmon being a prolonged p-R intenal.

OT}trR LESS COMMON FEATURES

l. IgA Glomerulonephrins2. Renal Am1'loidosis3. Cranial and Peripheral Neuroparhies4. Thrombophlebitis5. Arrpz6. Livedo Reticularis

G ENERAL RADI OGRAPE] C FEATURES

]. SOFT TISSUE S\4IELLINGSofl tissue su'elhng in the inte4rlnJangeal joins of lhe toes and fingen mal.result in salsage-like deformities ofentire digits. r:rge eftrsons nra-\'be encountered in rhe kree and ank-lejoints

2. OSTEOPOROSISRegional or perianicular osleopotosis accompanies aolte episodes of anhritir but it is corrrmon to derecr se'srecartilagilous and osseous erosions widrout adjacent oseoporosis.

3. JOINT SPACE NARROWINGLoss of the interosseous space is more frequent in the small aniculations of the feeL hands, and 11riss.

4. BONEEROSIONThe mos fequent sites of osseous erosion are the small joinrs of the feet- hands and \riss. Erosiors initialh-arFrar at the joint margiru and ma1' larcr pnogress to irn'olve the subchondral bone ln lhe cenral ponion of thlarticulation Tlre erosions tend to be ildistino rn stnpe zsd srilins.

5. BONEPROLIFERAT]ONBone proliferadon is panioiarlv cbaraaerisric of tbe seronegadve spon{'loarthropathies and is the mosthelpfirJ radiographic fearure in distinguishing these condirions fron Rheunaroid anfrltis. Bone proliferationmav uke several forms.

A Linear or fluS' perioseal proliferadon nuv occru along meaarsals, metacarpalq phalangealshafu- or the malleoli of tlre ankles. Periostitis rna]' occur rithorn sgnifcant articularabnonlreli6'. Tbe periosrids is similiar to H1'penrophic hilmornrv Osteoanlroprly

B. Feriosritis mav ocou al tre sites of rendon and ligament anachmeil to bone ( enrhesitis ).C, lnu'a-anicutar bone formation may occur about sites of osseous erosion

6. TENDINOUS CALCIFICAT]ON AND OSSIFICATIONThese manifesariors are frequent about the lgree, at $hjch sire rhe findings can resemble pelligrini-Stiedasrrrdrone ( pos-u-aumatic calcif cation of tbe medial collareraj Iigamenr ;.

SPECIFI C RADI OGRAPM C FEATI.]RDS

I. FOREFOOTThe forefoot is aflmed n 4u55o/o of patients. An1' joinl' rna)' be affecred- alrhough rhe ls MTp and Ip joimsare espeoalll"r'ulaerable. At an]'Iortion osteoporosis joint space loss. 1tla1g*1 erosionq, as tyell as periostitisof neigbboring diapbl'ses of meuunals and phala:lges ru)' occur. The sesamoid bones can demonsratesignifcant etost-ons and proliferation Subluxzrjon and deforrnin' of the MTp joinrs ma,, be e'idenl an

1 0 5

apqaran(}- tlal hzs been rermed launois's deformig'. ln tlese cases the degree of aniculal mrrtiletion tna't'

resemble that seen rn t\onatic and Rheurnatoid arthntis. or Neuropathic Osteoarthropathi"

2. CALCANEUSThe calcaneus is afleaed in25-5f/oof pauents. Bilarenl changes are frequeil' Retrocalcaneal bu$itis crates a

radiodense shadow that obbrerates the normal iuceil area beroleen the poSero-superior aspeA ofthe calcaneus

and rhe Ashilles tendon Illdefined erosioru on rhe posero-superior and poSenor srrfaces of the caicaneus

mal .bepres€nr .Poser io rca lcanea lq)ursarerareur rhRe i te /sqndrome,hr lgo lnmon* j l1 l lq 'Y i tsoondvbuq Rheumaroid arthritis, and horiauc an]titis. on rhe pla::rar surfrce of the bone osseous erosons'

hlperososis. and ill&fined sprn rna-v dwelop'

3. Alilll-EThe ankle is aflecred rn 30-50o/o of paoents. Sofi Gsue svelling large effusons and fluflr' or lilear periomus

of the diSal tibial and fb-rlar diaphl'ses and mcuphlses are common' Loss ofjoint space and anicular erosions

are much less common in the anHe joinr'

4. KNEEThe IsJree is aflecred in 254ry/o of patients. The most conunon abnormalin is a joint effusior\ although

;;;;"";. penosotis of Oe drsral femur and proxinul ubia' and erosi'e clunges can be deteaed

5. SACROILTAC JOINTThe sagroiliac jornts r|ay bE afleaed tn 4}40o/o of patients. Bilarerzl s.vmmetric or aqmmetric changes are

rnosl colrrmorl.

6. SPINEAlthough abnormalines of the spine occur in Reiter's qr]drome. their frequencl'' and exlenl aIE less tlnn in

ctassic arnq'losing spond-vlitis and Psoriatic artlritis'

LABORATORY TESTS*** Mosl laborarory resrs are verJ non-sp€cific for Reite/s s-rndrome- and the diagrrosis is based 6a slinical and

radiographic finding

l- Set'eral lab tests refled lhe presence 9f inflemm^tis11

- Neurrophilic lankoqrosis- Elerared erythroqre sedimenurion rare- Elerared C-reactive Prolein- Normoqtic anemia- Slnor.ial lluid rs mild to moderatelv innammaror-r' and m4v sho'w'the presence of large macropbagcs t}ut

gqn'ain nuclear debris and enguUbd nzurophils ( Reite/s cells )'

2. HLA-B27 is occasionally belpful'

3. Neu,uends uill be ro doc'ment lhe presence of a specific infecdon from the genitourirlalr or gastroinresrinal

s_\'$ems.

TREATMENTI. NSAIDS2. ROM / strengbening exercises of affmed joints

3. Local conicoseroid injeaions4. Topical corticosteroid and kerarolltic agentsj. $nlfi<alezine6. lrnmunosrppresve therap'( Eelhorevle' azathiopnne )

7. ? use of long rerm anuliotics ( inv'estigatioual )

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PSORIATIC ARTHRITIS--k-;arit -ran-fe

of.-6o6of-pa1Enrs wrih-psoriasis. Tbe al'erage age of ons€l is in the second decade and il ocqJrs more conmonly in mrtes. In mostadult pauents a long hison' of skin disease is erident- although in a feq' indiljrtrrels. the anicr:lar abnormalitiescoincide uift or antedate the apearance of skin lesions. Nail abnormalities appear 10 correlale mos closel'wift anicular disease.

PATEOI,'OGY

hthologic ch:nges of psoriatic aflhritis are basicallv sirnilar lo those of rheumatoid anhritis hl thereare also some pathologic charaaeristics of psoriatic arthdtk tha are distinctive.

l. Slnovial infl:rnrnetion is encountered hrt the degree of cellular inflammation nith lymphoq,tesand plasrna cells is much less nu*ed ftar in rheurnaroid aflhdtis.

2- ln{Iammato[ qlot'ial tissue ( pannus ) is prominent onlv on the surfrce of canilage. u'hereas inrheunratoid anhrins hlperplasic qnor"ium is seen in borh suyrrf cial and deep lal'en of the canilage.

3. Bone proliferation is evident in peri-articular areas.

4. Fibrous an\'losis of the anjcuiation mav be noted as in rheumatoid arthritis- how.ever in psoriaticanhrins bonl'anl'1'loss is also prominen.

CLIMCAL FEATIIRES

- rzriable prcsenlation u'i$ mono, pauci-, or poll-artiarlar disn'hrion- r"im:allv an1' anianlatjon c:m be affeoed althougb tlre small _ioints of the hands and feet arc reponedll.involved r\ 5G-75Vo of patients with anhriG.- sofl tissue 5s'slling can be prominem-- nail changes are generalll' apparenl in the same digir in B'hjch trere is significanr disrat Ip joinr aniorlarabnormaliq'. Nail changes include tbe folloning:

l . P l t ' " 92. tnnsv'erse ridges ( Beau's lines )3. subungual hrperkeratosis &scnbed as a s'hitislr- u'ax\,. grcasy debris which ma.' cruse onvclrolvsis.4. generalized discoloration and desmrction of the entire nail5. oil spot or &oplet belorv the nail.

CHARACTERISTICS OF PSORIATIC ARTERITIS

- i:rvolvertrent of srnovial joints and enLlreses.- asrmnreFic drsribnion is more co[lmon rhan gu3lssD-ic disribrtion- rnvolvemenl of the IPjoints of the hands and feet.- sacroilitis and spon{r'titis uitb pararcnebal ossifcation- bone erosion uith a{acent bony poliferation- destruction of phaiangeal nfrs

107

G ENERAL RA-DI OGRAPM C FEATURES

SOET TISSI]E SWELLING- n rito1ao or srmmeric sofi tissue 5e'slling about involved aniculadon- " 3arrsa!€-Iiks

" s1'slling of endre dgls.

OSTEOPOROSIS- NOT a Prominent feanre.- lack of oseoporosis s a reliable sigr in tlre diflerenuarion of psonatic arrhritis fiom rheunaloid artlmus.

JOINT SPACE NARRO\I/ING AND WIDENING- articular space nu]'be narrowed or nidened- in snrall jornu of the fingen and toes severe desruaion of margunl and subchondral bone can lead to

signif cant widening of the aniailar space'

BONE EROSION- erosive abnonnalities are prominent- inirialll'erosrons predominate in nurginal areas of the aniculatjon. but as thq'progess centml areas can also

be afleaed- o'er a pe;d of time it ma1, appe-lr as if the bones are being g:r.ru'ed al\"]' or have been q'hinled $' a penciJ

slurpener.

BONE PROLIFERATION- profferation of bone is a sriki:rg feature and is recognized as a diagfrosUc sign- itregular excresc€nces crslle a spiculared fi-al'ed Or " paint-brush " aFpearence.- penoseat neu,bone fornution and condensation ofbone on the perioseal and endosteal surhces ofthe conex

along u,ift Fabealfar fiickening in t-he spongrosa c:m ctuse an entire phalange to appear radiodense ( ivory

phalange- nooe protif.ratjon occurs al sites at u'hich Endors and ligarnens irrsen on bones ( entlresopathies )-

TUFTAL RESORPTION- resorprion of the rufls of the distal phalanges of the hands and feet is charaoeristic of psoriatic arthdtis.- the eroded bone nrav be srnoothly upered or irregular in outline'- the nail of the irn'olved digit is almos aluays involved

SPECIFIC RADIOGRAPHIC FEATURES OFTHE FOOT

FOREFOOT- bilareral aqrmmetric changes predominate in tre IP and MTP joins and are charaaerized b!' rnarginal

erosions- bone proliferation alterations iljoint 9pac6 and lack ofoSeoporosis'- exensive desrucrjon of the Ip joinr of tbe great roe is more charaaeristic of psoriatic arthritis tlun ary'otler

drsordsr. although Reite/s q'ndrome and gorn prodrce simifiar hrt less matkd ghangg al lhis ste.- oseoll;sis of phalangeal tufis, phalalgeal and meutarsal shafu can be encountered (aflhritis srrrril:ns )'

CALCANEUS- erosion and proliferadon of the pogerior and inferjor surface of the calcaneus ma)'be present.- retrocalcaneal busitis creztles a radio&nse area a{iacenl to the posterosuperior aspect of the bone.- tle Achilles'rendon may be dickened and irregular excrescences mey develop al irs sile of anachment'- plantar heel sptn.-'lnle;ortl' ..oo* of the ptantar surfac€ freguenrJl' evoke erlensive sclerosis of the surrormding bone.- occasionrllv the entire inferior surfrce of tbe calc"neus becomes ehrrnated

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EXTRA-ARTI CUT.AR FEATURES

SKIN : psoriaric skin lesions are nracular or paprlar u'ith characteristic grey-u,hite scales. Bleeding oflen occursal stles of scale remoral ( Auspiu sign ). Lesions ruke numerous shapes, brl are mosl ofien round and locatedover ex.tensor srrfrces.

EYE : eye inllamrnarion occurs in 3V/o of patients. Frndings indude conjunaivrtis. iritis, episcleritis. ariDkeratoconjunaivitis st cca

HEART : aonic insfrcienq'hr< been reported

NEUROMUSCULAR : an associated myopathy has been suggested

LABORATORY NNDINGS

- s-vnor"ial fluid fionr Ore joina is classicalll' aesa;U"a as being lemon 1'eUon'. Srnovial fluid anallsis t-tpicallldisplays 2,00G.15.000 WBC'c per cubc millim€rer u'ith a predorninadon of neutrophils. .Occasional nrasstveeffr:sions can have leukog'te crnmts in the 100,000/nmi range.

' - abscence of rheurnatoid fraor in mod patients.- mild anemia.- elerzted ESR ( er-flhrocyle sedimenution mte ).- occasionally elerated serum uric acid ( relaled to cdlular tuffover of psoriuic $laneous lesions ).- HLA B.27 k fieguentlypresent

TREATMENT

l. Srrypression of the skin dis€ase rnay be imporrant in helping to conbol tbe arthritis.

local arnlication of ointmentsGoeckerman regimenPttVA tberapylopl cal conicosteroidsinralesional corticoseroid injections

2. NSAIDS3. lnra-anicular conicoseroid injections4. Methouexate5. ? Hy&oxyctrloroquine6. ? Sulfasalazine7. Physical therap8. Surgery9. Ps-vcholgic supon

Enteroo ar hic A-rr britis

lnllamn-nron boq'el disease is a general rerm tlrat refers to both Crohn's disease and ulceratil'e coliris.Both of these diseases are chronic inllammato4' ptocesses of unhown origin s'ith distinct iruestinaldistribution. hisopat-bologr' and radiographic appearance . There is a direo causal Elatisnship bets'een lhernresriles and rhe joint disease. The artbritis may be peripbera! axial or both. The rbenmetologicmanifesutions of lbese t*'o disorders are similarbul not identical. Clinical signs of Crobn's disease usualh'begin in the 2n&3rd decades and can occur anrnvhere bets'een 1560 vears old in ulcerarjve colitis.

r 0 9

ICLIM CAI MANIFESTA TI O NS

PTRIPHERAL-ARTHRITIS-peripberal anjcular manifesations occlu n 10-2V/o of patients (more common in Crohn'sdisease) ard egual male: female predilection-the etiologl' of the artlritis is unlgronn but the close association u'ith thc activiq' of the boweldisease actir.in, suggests a common factor to both processesone h1'pothesis tmplicates absorption of antigeruc matenal through damaged intestinal mucosaand the development of a soluble antigen-antibody complex thel ga1g6 the joint space lo produces-vnovitis-also appears lo be geneticallv-linked-peripheral artiuitis usuallf indicares aaive bowel inJlammation-hon'ever. the artlritic $mplorrs ma1'begn before the bou'el s\Tnploms in Crohn's disease asopposed to ulceratire colitis, s'hich mal'occur u'ithin a feu'1'ears af,er the appearanc€ of bowelslmptoms-1picall1'. the artluitjs is oligoanicular or monoanicular and affects large joints (mosl commonh'the lorees, anklgs. shoulden or urists)-joints of tte lou'er exuemin' are afleded more ofien then the upper errremin,-aqmmeric inflammation of the PIPJs of the toes is particularll'suggestive ("sausage roes-)- -the arthritis is fiequentlt'accompanied b1'fever, painfirl oral ulcerations. uveids or skin lesions(such as pyoderma gangrcnosum in Crohn's disease and eryr}ema nodosum in ulcerative coliris)-tlte more severe the borvel disease and exra-anicular manifestatiorL the increased risk ofperipheral anhritis-arucks of ar0ritis appa:r abruflh' and reach a peak in 4g-72 hours-these arEcks are usualll'selfJirnilsd and resolve u'ithin seyeral g'eeks to several months-recuTences are common-Common sires of peripheral anhritis: tnees (8-16%)

ankles (5-10%)sboulders (24YAurisrs (l-2%)

-cnthesopathies al the Achilles lendon and plantar fascia ma_r' also occur

EXTRA SKELETAL MANIFESTAN ONS

-Besides the associaled bos'el disease- tre mos common errraskeletal invoh'enrenl is ocularinflammailon (uveitis) s'bicb occurs in l0yo of parrents during t-be disease course-oral ulcerations. digiul clubbing hepatobilian' disease. kidnel'soDes and amr4oidosis all areseen in about 57o ofparients-less common manifestatjons ilclude pyoderma gangrenosruL erythema nodosurn- leg uJcers-rhrombophlebitis and anal fimrla formation

RADIOGRAPHIC FEATURES

-Sacroiliitis q'it} or q'ilhoul higher lwels of spon{'litis occurs in I 0- I 57. of patiens-thjs form of arthritis occurs independently of rhe acrjve bowel infl:mm^1jsa-s\mptoms and radiographic changes are indistinguishable from anln'losing spon{'litis-these charges include bilateral sacroiliitis and thin marginal sruuretric srrrdesmophlres (u:hichma1' ultinatel-v lead to bamboo spine)-the spon{'Iitis occurs 2x as fieguentlf in males than females and il ma1'precede t}re bowels.\Tnploms bv several 1'ears-ph1'sical exam reveals sacroiliac joinl tenderness and a decrease of lumbar spine range of motionand resricdon of chest and spinal movemenl-radiographic evaluation of the peripheral aflhrjtis u-sualll- shoq's sofi tissue su'elling andperiani cular oneopenia-it is rerl'uncornmon lo see evidence of osseous or canilagirnus destruction

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LABORATORY FINDINGS

5nli3O%EveaposltiveHI.l:827fl iavE$ild\nifi s-----ftere is no correladon beto'een lil-A-B,27 and the peripberal arthritis or isolated sacroiliitis-increased SED rate. mild anemi4 leukoc-rtosis and rhrom@nosis-negadve rbeunutoid faaor and ANA-synoviat fluid anallsis usually reveals inllammatory fluid u'ith a leucoc-vte counl betn'esn 10.000-50.000/mm3 u'ith norrnal protein and glucose levels

TREATMENT-$eatrnent of tlre bouel disease usually resujG in tJre disappearance of the extracolonicmanifestation<, inclurting the artlritis-patient education-NSAIDS-phlsical therapl-cortico$eroid injections (for s.vmptornatic joints)-sulfasalazine-methotrexate-systemic coni costeroids-bonel surgeSy'colectom)'(resoh'es the peripbeml arthritis bul not the axial anhritis in ulceratirccolitis but has no beneficial effea in Crohn's disease)

ANX}'LO SIN G SPONDYLITIS

enkf'losing spond-vlitis is a chronic q'nemic inllammatory rheusratic disorder that prinur['affecrsrhe aiial skelaon, parzvertebral sofl tissueg spinal apophyseal joins. Sacroiliitis is the hallmark of jotnt

ilwolvemenl Tlre narne is derived fiorn the Greek roots ankylos meaning 'bent" and spondylos nrcaning"spina] venebrae." The disease is strongly associated qdth the lil-A-B.27 andgen Clinical nranifesutions of thedisease usualll'begrn in lare adolescense or earl-v adrlthood Onset afler age 40 is veq'unmrnmon The diseasehas both skelaal and Extra*eletal manifesations and is tluee times more conrmon in men than women-

CLINI CAL MANIFESTATI ONS

SKELETAL MANIFESTAN ONS- rhe mosl corunon and characteriaic early complaint is chronic low back pin of insidious ons€i" usua[ybeglnling in late adolescence or early adulthood- Ore second cornmoD early s1rrqrom is bck siffo"sg rvhicb is s'orse i.n the moming and is eased bJ' rnildphlsical acti@.- extra-anicular or juxu-artiorlar bonl' tenderness due to enbesitis rnay be an earty feature of tbe disease andcan be a major or presenting complaint in some paoems.- im'olyement of peripheral joinrs, other tlran bip and sboulders is infieguent in pnnwl' an\'losngspondvlitis.- mild consitrnional s\TnFoms such as anorexia malaise, or mild fever may occur in some Ftients in earlysuges of rhe disease.- phlsical sigs rna]'be qurte minimal in eartJ' sages of the disease. bou'ever therc is often some limitation ofmodon of tbe lumbar spire uith associated spasrn and soreness of the panspinal muscles.- the Wrigbt-Scbober tes is quire usefirl in detecting limiurion of fonrard flexion of the lumbar spine.- rnvolvemenl of coSovenebml and cosouansverse joinls results in restriction of cbeS evansion ( normally noless than I in& in $'omert and I ll2 incbes in men ) ad breathing tbat becomes prinarily diaphragnatic.- direct pressurc of tbe inflamed sacroiliacjoins frequerrtly caurs pain- bck Fin and stiffness generalll'diminish over Oe years br.u some degree of inflrmmnrorl'pain persiss.- contracurr6 al hip6 and compensatory flexion of the }orees frvor tre sooped over posture so that in adrzncedcases the mtient appealT to be doubled up.- sciatica-lfte pin ma1'alternate fiom one side to anotber ald is nol significandY relieved !v changs in

msition

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EXTRASKELETAL N{ANIFE STAN ONS

- ilre most cornnlon exrraskeletal involvemenl is acute anlerior uveius ( acule intis ) u'hich ocorrs in 25-309i' of

padenu al some time in rhe course of the dtsease'- cardiorasc'rlar invoh,ement is rare and includes ascending aonius, aonic ralve incompetence' and condtclon

abnormalities.liu,rg p"r"ncblnral in,olvement is rare and is charactenzed b' a sloq'lv progressive fibrosis at the upper lobes

ofthe lungs.- neurologic invoS,ement rrun, occur most often relarecl ro spinal fracnrrddislocatjon atlanroarral subluranon

or cauda equina srrdrome. Sponuneous anrerior atlantoaxial subluxatjon is a uell recognized compbcaEon

occurring in about 27o of Pattens.

RAD I OGRAPEI C FT.A TURES

l. Sacroiliac joint : Sacroiliitis is lhe hallrnarli of anl$'losilg spond.rlitis Alrhough an asrmnretric or unilateral

ai*;U.rUon can be evident sn iniriel radiographic eranl radrograpluc charges al later sages of rbe disease are

alnrost irnariablv bilateral and aqmmetncal in dismllxuon'

2. Spine : Abnormalities of the qpine can be seen in lhe discovelebral junaion apophyseal joints'

costovertebral jofuS, posrerior ligamentous anachmenls and atlantoa:rial articrrlaUons

3. Ankle : Rarel1,. clunges in rhe ankle can be notod in ank-vlosirtg spondl'litis. Tlre changes resemble futdrngs

in otler aniculations. pe;ostitis is panicularll'charaOenstic in the disal medial rjbia

4. Forefoot and midfmt : Bilareral srmmeric or aammetric abnormafities of the feel u'hich can be evident in

#ro*io-r"f y, $o/"of parienrs u,i0riong sTd"g ankvlosing spondvlitis. shou'a predilection for MTP and 1s

rarsomerararsal lornrs, and for rhe Ip joinr of the-grear roe. Sofl tissue su'elling diffr'rse joinl space narrol\lng

erosions witl adjacent bonl,protifera-ion predomiiatel)' on tre medial aspea of the melalarsal heads periostitis

of phalangeal and metatarsal sbafu, and inrra-anicular bonl' anlq'losis can be deteaed Subluxarion al MTP

joinr anicularrons consisring of fibular &r,iation of t}le roes is less fiequent and less severe ttan in rheurnatoid

arthritis.

5. Calcanars : Alrhough clinically rnanifest beel abnormalities are infreguent rn anlq'losing spondl'litits.

radiographic changes of the calcaneus are corrrmort- Bilateral abnormalities predominare. well defined plantar

o, po"o.rio, cafcaneaf spurs. or borlu are a corrrmon rranifesation but arc similar in apearance ro those in a

".ro,,oul., poplation Reuocalcaneal srvslling related to bursinq, posterior calceneal erosion and Achilles

rendon thickenrng are also frequenr. Bon1, erosion and proliferarion resulting in ill+find spur formation at the

site of hgamenrous anachmenl to bone on the inferior surface of the calcaneus rul)' occur. Perioseal

pt"fif.-t-or along tlre entjre undersrrface of the calcanzus is evident in a feu'F6ents'

I-ABORATORY FIN'DINGS

- there is no specifc lest for anlq'losing spon$'litis'- HLA-827 bre a go'/"sensitiriq' amoirgcaucasians. hrr is an expensive rest and should not be used for routine

screening_ w to 7\% of parienrs ba'e an elerzted eryhtocl4e sedimenration fle.

- mild to moderare elerarjon of serum lgA is frequentll'obsen'ed- slorial fluid anah'sis is similar ro rbeumaroid aflhritis'

- normocrtic. hlpochromic anemia ma1'be seeru

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il-A-B.27 ASSOCIATIONS

Spond-vloartlropatluBalanins. uveius. etc.Coioreaal czmcerAsbegossChronic inllammaron' bowel diseaseL'rmpboblamc leukemraSecondan'amyloidoss

TREAT]\lENT

- pa0enl education- NSAIDS- sulfasalazine ( rna]'be usefirl for peripheral anhritis )- local conicoscroid injections- rtqily exercises- surgical intervention

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