先天性髋关节发育不全

Developmentaldysplasiaofthehip(DDH)MahdiAlsaleem.DysplasiaofHip:AReview.ClinicalPediatrics2015,Vol.54(10)921–928IntroductionDevelopmentaldysplasiaofthehip(DDH)isoneofthemostcommoncausesofdisabilityamongchildren.DDHrepresentsawidevarietyofpathologicconditions,rangingfromfineacetabulardysplasiatoirreduciblehipdislocationwithproximalfemoraldisplacement.RiskFactorsforDDHGender：Girlsare8timesmorelikelytohaveDDHthanboys.IntrauterineLimitedMobility：Breechposition(especiallyfrankbreech)、First-bornbaby、OligohydramniosFamilyHistory：AboutathirdofthepatientswhowerediagnosedwithDDHhadfamilyhistoryforDDH.Swaddling：keepthehipsintheextendedadductedpositionGeneticFactors：GDF5,TBX4,ASPN,IL-6,TGF-β1,andPAPPA2OtherConditions：cerebralpalsy,spinalcordlesions,ornerveandmuscledisordersClinicalManifestationsClinicalmanifestationsforDDHdependontheageofthechild.Forexample,innewbornitjustmaypresentwithmildlimitationofabduction.Inatoddleritmaypresentwithasymmetricgait,whileinadultswithhippainanddegenerativearthritis.Oneshouldalsoinquireiftheparentsnoticedanyasymmetrybetweenthelimbsoranyabnormalsoundswhenchangingthediapers.TypesofDDHdependingontheseverity:A,normalhip;B,mildhipdysplasia;C,subluxatedfemoralhead;D,dislocatedfemoralhead.PhysicalExaminationRemovethediapers,flexthehips,placethumbonthemedialsideofthethighabovetheknee,andtherestofthehandoverthegreatertrochanterlaterally.ForBarlowtrytoadductthehiptomidlinewithgentleposteriorforcetoseeifthefemoralheadisdislocatable.ForOrtolanitakethehipfromtheadductedpositiontofullabductionwithgentleforwardpressureonthefingersoverthegreatertrochanter,tryingtoreducethehip;ifitisreducibleyouwillfeeltheclunkaspositiveOrtolanitest.PhysicalExaminationLimitedHipAbduction：Inbabiesover3monthsofage,limitedhipabductionmaybetheonlyphysicalsignpresent.Inthiscasethebabyneedsfurtherinvestigationevenifothersignsarenegative,becausethedislocatedhipbecomesstableinthenewdislocatedposition.AsymmetricSkinFolds：Extraskinfoldonthedislocatedsidealsocanbefound.However,extraskinfoldisalsofoundin25%ofnormalbabies.Therefore,extraskinfoldshouldbetakenintoconsiderationalongwithotherfindings.GeleazziSign：Unevenkneeheightswithboththehipsandkneesareflexed.KlisicTest：Itisdonebyplacingtheindexfingerontheanteriorsuperioriliacspineandthemiddlefingeronthetrochanter;inanormalbabythelinebetweenthese2pointspassesthroughtheumbilicusoraboveit.InchildrenwithDDHthelinepassesbelowtheumbilicus.RadiologicalEvaluationUltrasoundisveryusefulforconfirmingthefindingsonclinicalexaminationtill4to6monthsofage.ForUSevaluationofthehipthereare2methods:astaticacetabularimageproposedbyGrafandadynamicstressimageproposedbyHarcke.RadiographsmaybeperformedtoassessthehipsinchildrenwithaclinicaldiagnosisofDDH,tomonitorhipdevelopmentaftertreatment,andtoassesslong-termoutcomes.PlainradiographicevaluationofDDH(Figure3)isdonebydrawing2linesontheplainX-ray:1.Hilgenreiner’slineisalinedrawnhorizontallythroughthesuperioraspectofbothtriradiatecartilages.Itshouldbehorizontal.2.Perkin’slineisalinedrawnperpendiculartoHilgenreiner’sline,passingthoroughthelateral-mostaspectoftheacetabularroof.ThefemoralheadshouldbeseenintheinferomedialquadrantanditshouldliebelowHilgenreiner’sline,andmedialtoPerkin’sline.LateralorsuperiordisplacementofthefemoralheadoccursinDDH.3.Shenton’sline.Itisdrawnpassingfromthemedialborderofthefemoralnecktothesuperiorborderoftheobturatorforamen.Inthenormalhipitiscontinuous,whereasinthehipwithDDHthelinecontourwillbeinterrupted.TheShentonlineremainsintactin“subluxation”butdisruptedin“dysplasia.”4.Acetabularindex.TheacetabularindexistheangleformedbetweenHilgenreiner’slineandatangentiallinetothelateralossificmarginoftheroofoftheacetabulum.Theacetabularindexishelpfulinmeasuringthedevelopmentoftheosseousroofoftheacetabulum.Normalvaluesfortheacetabularindexareasfollows:<35°atbirth;<25°at1year;<20°between1and3years.TreatmentThetreatmentofDDHhasundergonesignificantevolution,butthecurrentgoldstandardisstillthePavlikharness.Avascularnecrosisremainsthemostdevastatingcomplicationofharnessusewithitsincidence(0%to27%).TreatmentforDDHdependsontheageofthechild.Newbornto6MonthsofAgeThecurrentgoldstandardisstillthePavlikharness.Pavlikharnessworksthebestwithsuccessrateof85%to95%andshouldbestartedassoonaspossibletogetthebestresult.Thepurposeoftheharnessistomaintainthehipinflexionandabductionpositionsoastobringthefemoralheadasclosetotheacetabularringaspossible.Children6Monthsto2YearsofAgeTheprincipleatthisagegroupistomaintainthefemoralheadintheusualpositionwithoutdamagingit,soclosedreductionisdoneundergeneralanesthesiaintheoperatingroom.ChildrenOlderThan2YearsOpenreductionisusuallyrequiredabove2yearsofage,mostlybyfemoralosteotomytorelievethepressureoverthefemoralheadandtoreshapetheacetabulum.Thepatientisusuallyimmobilizedbyspicacastfor6to12weeks.Complications(a)grossshorteningofthelimbsandwaddlingGait(b)lowerbackpain(c)prematureosteoarthritisontheaffectedsite.Totalhiparthroplastyremainsthestandardofcarewhenend-stageosteoarthritisleadstosignificantpainandlossoffunction.Thankyou!