Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_2821_Библиотеки_им_академика_М_И_Перельмана
.pdf
https://t.me/medicina_free
FIGURE1-6.RadiographicandCTpresentationofmelorheostosis.AP(A)andlateral(B)X-raysoftheleft
kneedemonstratetheclassic“drippingcandlewax”appearanceofmelorheostosiswithcorticalhyperostosisalong
theposteromedialaspectofthedistalfemurandthemedialcortexofthetibia.Smallextraosseoussofttissue
ossificationintheposteriormuscleisseenonthelateralradiograph.C:Frontalradiographoftheleftankleinthe
samepatientdemonstratesalargeossificationinthesofttissuesinferiortothemedialmalleolus,representingan
uncommonextraosseousmanifestationofmelorheostosis.D:CoronalreformattedCTimageofthelefthip
demonstratesaheterogeneousossificmasscontiguouswiththelessertrochanterinthispatientwith
melorheostosis.
MULTIPLEHEREDITARYEXOSTOSIS
Multiple hereditary exostosis (MHE), also known as familial osteochondromatosis or
diaphyseal aclasis, is an autosomal dominant inherited disease presenting with multiple
osteochondromas (exostoses).
15,16
Osteochondromas are classically sessile or pedunculated
lesionswithcorticalandmedullarycontinuitywiththeunderlyingbone,cappedbycartilage.
15
Mostpatientsarediagnosedby5yearsofage.Thelesionscommonlyoccurinthemetaphysis
oflongboneswithapredilectionforthedistalfemuraswellastheproximalanddistaltibia
https://t.me/medicina_free
andfibula.Largerosteochondromascanresultinsignificantskeletaldeformity.15Theexostosis
should cease growth when the closest physis is closed, and continued growth suggests
malignanttransformation(Fig.1-8).
NAIL–PATELLASYNDROME(FONGDISEASE)
Nail–Patellasyndrome,alsoknownasFongdisease,isarareautosomaldominantcondition
classically characterized by a tetrad of nail, elbow, and pelvic and knee abnormalities.
17
Affected individuals have a distinct lean body habitus, and may also present with eye
abnormalitiesandrenaldysfunction.17Radiographsofthepelvisrevealbonyprocessesarising
fromtheposterioraspectoftheiliacbonesinmorethan80%ofpatients,whichareknownas
“iliac horns”.18 These protuberances may be palpable and are characteristically bilateral.
Knee and elbow involvement can be asymmetric. Radiographs of the knee may reveal
dysplasia, hypoplasia, or absence of the patella, often with superolateral displacement.
17
Elbowradiographsmaydemonstratehypoplasiaorabsenceoftheradialheadwithorwithout
radialheaddislocation,aswellasepicondylarandcapitellarabnormalities(Fig.1-9).
17
https://t.me/medicina_free
https://t.me/medicina_free
https://t.me/medicina_free
https://t.me/medicina_free
FIGURE1-7.X-rayandMRfindingsofmetastaticdiseaseintheleftfemurandintheleftfoot.A:Frontal
radiographoftheleftfemurdemonstratesclassicmottledbonemarrowinleftfemur.B:Coronalinversionrecovery
imageofbothfemorademonstratesdiffuseinfiltrativehyperintensityinthefemoralheadandinthemedullarycavity
oftheleftfemoraldiaphysiswithsurroundingperiostealreaction(arrows).C:CoronalT1-weightedimageofboth
femorademonstratesdiffuselow-signal-intensitymarrowreplacementthroughouttheleftfemoraldiaphysisand
femoralhead.D:Axialinversionrecoveryimagedemonstratesdiffusehyperintensemarrowreplacementintheleft
femurwithnearcircumferentialperiostealreaction(arrows).EtoG:MRdemonstratesmetastaticdiseaseinthe
medialcuneiform.Coronalinversionrecoveryimage(E)demonstratesdiffuseinfiltrativehyperintensitythroughout
themedialcuneiform.Axial(F)andsagittal(G)fastspinechoprotondensity-weightedimagesdemonstratea
geographichypointensemarrowreplacementprocesslargelylocalizedtotheplantaraspectofthemedial
cuneiform.
FIGURE1-8.X-rayandMRdemonstrationofMHE.AP(A)andlateral(B)radiographsinthisskeletallyimmature
patientwithMHEdemonstratebothsessileandpedunculatedexostosisofthedistalfemurandproximaltibiaand
fibula.Thesessile-typelesionsusuallydeformandexpandthemedullarymetaphysealregionsoftheinvolved
bones.Alargepedunculatedexostosisextendssuperiorlyfromthemedialcortexofthefemurandterminateswitha
typicalcauliflower-liketuft.C:MRcoronalprotondensity-weightedimagedemonstratesmultiplebroad-basedand
pedunculatedexostosesabouttheknee,withthelargestlocalizedmedially,arisingfromtheproximaltibial
metaphysis.Notethecontinuitywiththemedullarycavityoftheproximaltibia.
https://t.me/medicina_free
FIGURE1-9.RadiographicpresentationofNail-PatellaSyndrome.Frontalviewofthepelvis(A)demonstrates
theclassiciliachorns(arrows)characteristicofNail–Patellasyndrome.Merchantviewoftheknees(B)
demonstratesthebilateralhypoplasticpatellae,shallowfemoralsulcianddislocationoftheleftpatella.
NEUROFIBROMATOSISTYPE1
Neurofibromatosistype1(NF1),alsoknownasvonRecklinghausendisease,isanautosomal
dominantneuroectodermaldisorderthatischaracterizedbytheformationofneurofibromasand
mesodermaldysplasiasaffectingnumerousorgans,withskeletalinvolvementinupto50%of
patients.
19,20
The lower extremity manifestations of dysplasia usually result in tibia and/or
fibulabowing,whichpresentswithinthefirstyearoflifeinthesepatientsandoftenisevident
before the other signs of NF1.
19,20
The bowing commonly results in fractures and
pseudarthrosis(Fig.1-10).
19,20
https://t.me/medicina_free
NEUROPATHIC(CHARCOT)FOOT
Neuropathic foot,also knownasCharcotfoot,isanosteoarthropathycausedbyavarietyof
conditions,themostcommonbeingdiabetes.21Theneuropathicfootcanbeseparatedintoboth
acute and chronic phases. In the acute phase, the disease can present with clinical
manifestations of warmth, swelling, and erythema that can mimic osteomyelitis.22 X-ray
changesmaybesubtle,withtheearliestfindingbeingfocaldemineralizationandflatteningof
the metatarsal heads.23 In theearlystages, MRI examinationmaydemonstrate bonemarrow
edema, joint effusions, fluid collections, and marrow enhancement, which mimics
osteomyelitis,complicatingtheaccuratediagnosis.
22
FIGURE1-10.RadiographicpresentationofNeurofibromatosis.StandingAPradiographofbothtibiasand
fibulasinthispatientwithNF1demonstratesseverebowing.Thereissecondarydeformityoftheanklejoints,right
greaterthanleft,wherethereismarkedmedialslantingofthetibialplafondandanklejoint.
https://t.me/medicina_free
In the chronic phase, neuropathic joint involvement assumes a more specific and
identifiable pattern, with classic findings commonly classified by the “6Ds”: dense bones
(absence of osteopenia), degeneration, destruction of cartilage, deformity, debris (loose
bodies),anddislocation.Inthefoot,themidfootandinvariablythetarsometatarsal(Lisfranc)
jointsaremosttypicallyaffected,whichcanleadtocollapseofthelongitudinalarch.
21,23
A
commontermforaCharcotjointis“abagofbones”(Fig.1-11).
OLLIERDISEASE(MULTIPLEENCHONDROMAS)
Ollier disease is a benign rare nonhereditary chondroid dysplasia involving multiple
asymmetricallydistributedenchondromas.24Enchondromasusuallyinvolvethesmallbonesof
thehandsandfeetoroccurcentrallywithinthemetadiaphysisoftubularbones.
10,24
Theyare
benigncartilaginouslesionsthataretypicallyosteolyticwithchondroidmatrixcalcifications.
Although enchondromasare considered benign, patientswith Ollier disease are at a 5% to
50%riskformalignanttransformationoftheenchondromatochondrosarcoma(Fig.1-12).
24
https://t.me/medicina_free
Соседние файлы в папке Библиотека им академика М.И. Перельмана