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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_2821_Библиотеки_им_академика_М_И_Перельмана
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continuetoprogressdespiteappropriatetherapeuticefforts.
Any surgery performed in or close to the CRPS affected area may lead to acute and
prolongedexacerbationofpreexistingsymptoms.Inotherwords,toaCRPSpatient,thereisno
minorsurgery.Therefore,adequatepaincontrolbyregionalanestheticand/oropiateinfusion
foraperiodoftimepostoperativelytocreateapain-freewindowforregainingormaintaining
rangeofmotionisofutmostimportance.
SUMMARY
Despitevoluminousliterature,manyaspectsofthetreatmentofthesedisordersarestillbased
on empiricism. For example, it is not yet clear why sympathetic interruption in a warm,
edematous,andvasodilatedextremitydoesnotproducefurtherrelaxationofarteriolarsmooth
muscleandsubsequentlyanincreaseinswellingandpain.36Hypotheseshavebeenproposed,
buttheylackexperimentalsupport.80Thepreciseroleofsteroids alsoremainstobesettled.
There is no explanation either for the phenomenon that LSBs become less effective with
repetition.Partofthedifficultyinstudyingthesepatientshasbeentheubiquitousconfounding
effect ofthe placebo response.81 Establishing a rationale for the treatmentsrequires further
carefulinvestigation.
To the physician, the ultimate goal is restoration of complete functional and anatomic
integrity of the extremities at the earliest possible time and by the simplest therapeutic
procedure. Selecting the treatment best suited to the individual at the earliest moment will
increase the chances of remission and reduce intractability. Long, continued vascular
disturbancesanddisusebecauseofpainarethemajorcausesofpermanentdisability.45Early
diagnosis and specific, goal-directed treatments buttressed by a supportive social structure
offerthebestchanceofreturningthepatienttoameaningfulexistence.
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C
ongenital foot and ankle conditions are common problems that pediatricians and
orthopedic surgeons see throughout their careers. Pediatric foot and ankle surgery is
becomingitsownuniquefieldwithinorthopedics,andknowledgeoftheseconditionsisakey
componentinorthopediceducation.Ingeneral,pediatricfootandankleconditionscanbeseen
atbirth,progressovertime,ordevelopinadolescence.Manyoftheseresolvespontaneously,
but others may require operative management in order to improve ambulation, create a
plantigrade foot,or even permit shoewear. Congenital disorders of the footand ankle can
occurinisolation; however,many occuras sequela of genetic syndromes or neuromuscular
diseases. Orthopedic surgeons, podiatrists, and other musculoskeletal clinicians should be
cognizantofthepotentialunderlyingsystemicimplicationsofthesecongenitalmanifestations.
Justascriticalisanawarenessamongothercliniciansofwhatfootandankledeformitiesmay
beattributedtocongenitaletiologies.Thischapterdiscussesthemostcommoncongenitalfoot,
ankle, and lower extremity conditions, their etiology, associated conditions, and treatment
options.
KNEEDISLOCATIONS
CongenitalDislocationoftheKnee
Congenital anteriordislocationofthetibiarelative tothefemuris arare condition,withan
estimated incidence of less than 1 per 100,000.1 Examination findings range from a
hyperextensiondeformityofthekneeorgenurecurvatumtothemostsevereformdemonstrating
frankdislocation.Radiographsconfirmafixedanteriorsubluxationordislocationofthetibia
on the distal femur. Although it may occur in isolation, there is an association with
arthrogryposis, Larsen syndrome, and myelomeningocele.
2,3
There is also an increased
incidencewithbreechdeliveryandother“packagingdisorders”includingclubfeet,congenital
verticaltalus(CVT),anddevelopmentalhipdysplasia.
2–5
Simple hyperextensiondeformities and dislocations notassociated with other disorders
cangenerallybetreatedearlywithgentle stretching,serial casting, or splintinginaPavlik
harness.
2,5,6
Frankdislocationsorcasesthatfailconservativemanagementmayneedsurgical
treatment with either (1) quadriceps lengthening and anterior capsulotomy or (2) femoral
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shorteningandvariableanteriorreleases.7Withappropriateearlytreatment, conservative or
surgical,patientscangenerallyexpecttohavefunctionalrangeofmotionandambulatewithout
a brace, but may have a stiff-knee gait pattern.
2,7
There are only a few case reports of
congenital dislocations identified and treated in adolescence.
8–10
All required surgical
treatmentwithsome combinationofquadricepsplasty, openreduction,framedistraction,and
femoralshorteningtoreconstructanextremitythatallowedambulation.
CongenitalDislocationofthePatella
Congenitaldislocationofthepatellaischaracterizedbyirreduciblelateraldislocationofthe
patella, flexion contracture of the knee, knock-knee deformity (genu valgum), and rotation
deformity of external tibial torsion.The fixed deformity is not to be confused with patellar
instability or recurrentpatella dislocation,moreoftenseenlater indevelopment,whichcan
oftenbetreatedconservatively.
11–13
Thecongenitaldisorderisalmostifnotalwaysassociated
withsystemicdisorderssuchasarthrogryposisandchondrodysplasiapunctata.Inaddition,at
leasttwopublishedserieshavefoundthatallpatientshaveassociatedfootdeformities.
11,14
Althoughthekneedeformityisnotsubtleandgenerallyrecognizedatbirth,thediagnosis
maybedelayedbecausethepatelladoesnotossifyandisnotvisibleonradiographsuntil3to
5years ofage.Palpation,however,shouldidentify thepatella laterallyanddemonstratethe
absenceofthe patellaanteriorly. Ultrasoundmayconfirm the diagnosis early. Bydefinition,
conservative measures are ineffective, and early surgical correction involves quadriceps
lengthening, lateral release, and medial tightening. Without early surgical correction,
significant disability results including compromised ambulation. If surgery is delayed until
adolescenceorearlyadulthood,treatmentrequiresbonyreconstructionwithdistalfemoraland
tibialtubercleosteotomiescombinedwithsofttissuereconstruction.15Treatmentevenlaterin
lifeafterthedevelopmentofarthritismaybeaddressedwitharthroplastyandconcurrentsoft
tissuereconstruction.
16,17
TIBIALBOWING
AnterolateralBowing
Congenital bowingat the middle and distal third junction of the tibia with theapexin the
anterolateral direction and with a high risk of progressing to pathologic fracture is
characteristicofneurofibromatosistype1(NF1).Althoughthebowingisoftennotedatbirth,
theremaybeadelayinrecognitionofthefractureriskandtheunderlyingsystemicsyndrome.
The bowing in combination with any one of the other seven diagnostic criteria for the
syndromeissufficienttomaketheclinicaldiagnosisofNF1.18PatientswithNF1presentwith
wideclinical variability,however,andlessthan5%havetibialpathology.19Radiographsof
anterolateral bowing associated with NF1 will confirm the direction of bowing and
demonstrate cortical thickening, particularly on the posteromedial aspect, and a narrower
medullarycanal.20 Pathologic fractures, however, frequently occur at the apex, which heal
abnormally leading to nonunion or pseudoarthrosis (Fig. 13-1). Although typical of
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anterolateral bowing, tibial pseudoarthrosis mayalso be seen withamniotic bands, fibrous
dysplasia,intrauterinetrauma,osteogenesisimperfecta,oranyendocrineprocessthatweakens
thebone.
18
Althoughtheentireentityisoftenreferredtoascongenitalpseudoarthrosisofthetibia,this
isamisnomerasthebowingiscongenital,butthefractureandsubsequentpseudoarthrosisare
almost always seen only later.21 Unlike the early stages with cortical thickening, later
radiographs will demonstrate cystic changes, frank fracture, or tapered thin cortices of a
pseudoarthrosis. Even when fractures heal, healing is usually abnormal, and the risk of
refractureishigh.
The goal of treatment in children identified with anterolateral bowing is prevention of
fracture.The conservativeapproachinvolves castinginyoungchildrenandbracinginolder
children until skeletal maturity, with obvious compliance difficulties. Success with purely
conservative measures, at least in patients with NF1, appears to be rare.
21,22
Surgically,
prophylactic bypass strut grafting has been shown to be successful in several series.
21,22
Simple osteotomy of the bowed segment with local bone grafting and intramedullary rod
fixation appears to inevitablyfail andonlyhasten the developmentof a pseudoarthrosis. In
patients who have already fractured and developed a pseudoarthrosis, options include
intramedullary nailing with bone graft, vascularized fibular grafting, and thin-wire external
framing.
2,21,23
Refractureevenafterbonegraftingandfixationisrelativelycommon,andskeletalmaturity
isbelievedbutnotproventobeamilestonefordiminishedrisk.
23,24
Otherlong-termfunctional
issuesincludeleglengthdiscrepancy,anklestiffnessifintramedullaryrodscrossedtheankle
joint,andvalgusankledeformityiftherewasaconcurrentfibularpseudoarthrosis.
21–24
There also exists a separate subset of anterolateral tibial bowing associated with
ipsilateral hallux duplication.
25–30
Recognizing these patients as a different cohort from
patientswithNF1isimportantasthis“benign”formisnotassociatedwiththedevelopmentof
pseudoarthrosis. Although some may still benefit from operative correction, many of these
resolve spontaneously, with onlya subclinical leg lengthdifference. Additionally, fractures
andosteotomiestendtohealnormallyinthesepatientsincontrasttoNF1-associatedbowing.
Otherthanthe obviousduplicationofthehallux,the bowingalso tendstobemoreproximal
andmid-tibial,andtheremaybeaduplicationofthemedullarycanalattheapexofthetibial
bow.
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FIGURE13-1.Anteroposteriorandlateralradiographofanterolateraltibialbowingwithpseudoarthrosis.
AnteromedialBowing
Anterior or anteromedial tibial bowing with the apex pointed away from the fibula is
associatedwithfibularhemimelia.3Radiographsrevealadeficientfibulaandarediagnostic.
Associatedconditionsandprognosisarepertheunderlyingfibularhemimelia.
PosteromedialBowing
Posteromedial bowing of the tibia has an undetermined etiology but is associated with
calcaneovalgusdeformityofthefoot.Thedeformityisobviousatbirth,withthecombination
oftibialandfootdeformitycausingthefoottobeinaseverelydorsiflexedposition.3Although
the majority of the angular deformity spontaneously resolves, residual clinically relevant
shortening typically persists.
31–33
Fracture and pseudoarthrosis do not develop as with
anterolateral bowing. The posterior bowing is more likely to completely correct than the
medial bowing, leading to a residual valgus deformity. Treatment rangesfrom conservative
approachwithbracing andstretching tosurgical with osteotomiestocorrectangulation and
length.Thedeformityisalmostalwaysunilateral,andtheseverityofbowingispredictiveof
the degree of eventual limb length discrepancy.32 With incomplete correction, limb length
discrepancy,valgusdeformity,andweakplantarflexionmayallbesymptomaticlaterinlife.
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LIMBDEFICIENCIES
ProximalFemoralFocalDeficiency
Proximalfemoralfocaldeficiency(PFFD)isacongenitalabnormalityoftheproximalfemur
thatoccursin1in52,000livebirths.34Thespectrumofdiseaserangesfromashortfemur,to
an absent femur, to an absent hip or even a femoral neck pseudoarthrosis. PFFD is often
sporadic, butthere are rare autosomal dominant forms that frequently occur withabnormal
facies.35Thereareseveralassociatedconditionsincludingfibularhemimelia,anteriorcruciate
ligament(ACL)deficiency,kneecontractures,andcoxavara.
Physicalexamandobservationincludeashortandbulkythighthatisflexed,abducted,and
externallyrotated.Instabilityofthehipandkneearecommonphysicalexamfindings.TheACL
isoftenabsent,andtherecanbeanassociatedflexioncontractureoftheknee.Inupto45%of
cases, there is associated fibular hemimelia with a short tibia and equinovalgus foot
deformity.35Radiographsofthefemur,tibia,andfootshouldbeobtainedbilaterally.
ThemostcommonclassificationsystemusedisdescribedbyAitken.Itisdividedintofour
classesbasedonradiographsoftheacetabulum,femoralshaft,femoralhead,and attachment
betweenthefemoralheadandtheshaftatmaturity.
ClassA:Femoralheadpresent,acetabulumnormal
ClassB:Femoralheadpresent,acetabulummildlydysplastic
ClassC:Femoralheadabsent,acetabulumseverelydysplastic
ClassD:Femoralheadabsent,acetabulumabsent
Treatmentisdependent onthefunctionofthefoot,hipjointstability,andultimateleglength
deficiency. Initial decisionmaking includesdetermining the finallimblength discrepancy at
maturity;decidingwhetherornotthefootshouldbesaved,amputated,orrotated;determining
ifthekneeshouldbefused;anddeterminingifthehipstabilityissufficientorifitshouldbe
stabilizedbyfusingtheproximalfemurtothepelvis.
35
FibularHemimelia
Fibularhemimeliaisthemostcommonlongboneagenesisofthebodyoccurringatarateof
7.4 to20per million live births. The fibulacanhavea varying degree ofshorteningor be
completelyabsent.Themajorityofcasesareunilateralandassociatedwithabsentlateralrays.
Fibularhemimeliaalsohasastrongassociationwithshorteningofthelimb,tibialbowing,foot
deformities, tarsal coalitions, ball and socket ankle joint, and proximal femoral focal
deficiency.Thereisnoknowninheritancepattern.
34,36
Physicalexamwillrevealalimblengthinequalityaswellasfootdeformity.Limbfunction
needstobeassessedasmultipleassociatedproblemscanexistinthekneeandproximalfemur.
Radiographsoftheentire limbincluding boththe femur,the foot,and thecontralaterallimb
shouldbeobtained.Classificationsystemsthataremostcommonlyusedincorporateboththe
eventuallimblengthdiscrepancyatskeletalmaturityandthetypeoffootdeformity,asbothof
thesefactorsguidetreatment.
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Treatmentfor fibula hemimelia is individualized anddependent on the future degree of
limb length inequality at maturity as well as stability of the foot and ankle. Nonsurgical
management is indicatedinpatientswith expected limb length deficiency less than2 cm at
maturityandafunctionalfoot.Ifthepatienthasapredictedleglengthdiscrepancyofover25
cm or more and a deformed foot withvalgus deformityof the ankle, then amputation with
prostheticreplacementisrecommended.Inpatientswithanonfunctionalfoot,amputationisthe
treatmentofchoice.Inpatientswithexpectedlimblengthdeficiencyatmaturityof8cmorless
with a functional foot and stable ankle, then lengthening procedures and/or contralateral
shorteningwithepiphysiodesiscanbeconsidered.
35,36
HINDFOOTCONDITIONS
Clubfoot
Clubfoot is themost common congenital footand ankle defect andoccurs at a rate of1 to
2:1,000newbornsintheUnitedStates.Clubfootismorecommoninboys,bilateral50%ofthe
time,andoftenidiopathic.Thereisastronggeneticcomponentassociatedwithclubfoot,and
the familial occurrence is 25%. Despite it being most often idiopathic, there are several
associatedconditionswithclubfoot includingarthrogryposis, amniotic bandsyndrome,tibial
hemimelia,diastrophicdysplasia,myelomeningocele,Larsensyndrome,prunebellysyndrome,
andPierreRobinsyndrome.
36–40
Clubfoot is classified as positional, idiopathic, or teratologic. Positional clubfoot is
attributed to intrauterine positioning and is usually rather flexible. Treatment with serial
castinggenerallyrapidlyresolvesthedeformity.Idiopathicclubfootreferstotheclassicform,
whichismultifactorialincludingbothenvironmentalandgeneticfactors.Thesefeetareusually
stiffer andrequireserial castingandpossible surgical intervention.Teratologic clubfeet are
becauseofsyndromicorneuromuscularconditionsandarethehardesttotreatand generally
requiresurgicalintervention.
35–37,41
Atbirth,thereisacleardeformityofthefoot.Thefootandcalfareoftensmallerthanthe
unaffectedlimb,andthefoothasmedialandposteriorskincreases.Thehindfootisinequinus
andvarus(Fig.13-2).Thedeformitymayberigidonexam.Thepathoanatomyofclubfoothas
beenwelldescribedandtheacronymCAVEiscommonlyused.
Cavusofthemidfoot—becauseoftightperoneuslongusandintrinsics
Adductusoftheforefoot—becauseoftighttibialisposterior
Varusofthehindfoot—becauseoftighttibialisposteriorandtightAchillestendon
Equinusofthehindfoot—becauseoftightAchillestendon
Thebonyanatomyofthefootisalsochangedasthepullfromthetendonsshiftstheosseous
structures.Thetalusisdirectedplantarandmedial,calcaneusisinvarusandrotatedmedially
aroundthetalus,andthenavicularandcuboidaredisplacedmedially.
Imaging of the foot is not usually required. Withimprovements inultrasound, however,
prenataldiagnosisofclubfootatthe16-to20-weekultrasoundhasincreased.Thisallowstime
forcounselingtheparentsonthedeformityandplanningfuturetreatments.
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