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董堯主治醫(yī)師 資陽市中心醫(yī)院 骨科 一、正常髖關(guān)節(jié)（I型）的特點(diǎn)及治療特點(diǎn)：Ⅰ型髖關(guān)節(jié)發(fā)育正常，其中又分Ia型和Ib型。Ia型β角小于55度，Ib型β角在55-77度，兩者α角都大于60度。髖臼角30°，α角60°，β角55°。髖臼對(duì)股骨頭有良好的覆蓋，骨頂外緣銳利成角（Ⅰa型）或輕度變鈍（Ⅰb型），臼頂軟骨狹窄呈三角形1。治療：對(duì)于Ⅰ型髖關(guān)節(jié)，通常不需要特殊治療，只需定期進(jìn)行復(fù)查，觀察髖關(guān)節(jié)的發(fā)育情況。因?yàn)檫@是正常的髖關(guān)節(jié)發(fā)育狀態(tài)，在日常的生長發(fā)育過程中，正常的活動(dòng)和生活不會(huì)對(duì)其產(chǎn)生不良影響。但家長也需要關(guān)注孩子的一些日常行為，例如孩子的活動(dòng)是否自如等，若發(fā)現(xiàn)異常情況應(yīng)及時(shí)就醫(yī)進(jìn)一步檢查。二、髖關(guān)節(jié)發(fā)育稍差（Ⅱ型）的特點(diǎn)與治療特點(diǎn)：Ⅱ型髖關(guān)節(jié)發(fā)育稍差，分為Ⅱa、Ⅱb、Ⅱc、Ⅱd四個(gè)亞型。Ⅱa型指出生12周以內(nèi)嬰兒，α角為50°-59°，β角小于55度；Ⅱb型指12周以上嬰兒髖，α角50°-59°，β角55°；Ⅱc型α角43°-49°，β角小于77度；Ⅱd型α角同Ⅱc型，β角大于77度。髖臼角在30°-43°之間，α角55°-60°，β角55°。該型髖的骨性髖臼外上緣缺少鈣化，骨頂輪廓發(fā)育缺陷的部分由軟骨頂增寬充填，覆蓋在股骨頭上25。治療：對(duì)于Ⅱ型髖關(guān)節(jié)，尤其是Ⅱa和Ⅱb型，因?yàn)樘幱隗y關(guān)節(jié)發(fā)育的臨界狀態(tài)或者骨化延遲狀態(tài)，在早期通常采取觀察和定期復(fù)查的策略。如果沒有進(jìn)一步的惡化或者有好轉(zhuǎn)的趨勢(shì)，可以繼續(xù)觀察。而對(duì)于Ⅱc和Ⅱd型髖臼發(fā)育不良的情況，可采用軟式支具矯治，如Pavlik吊帶等。通過支具將髖關(guān)節(jié)保持在合適的位置，促進(jìn)髖臼的正常發(fā)育。在使用支具期間，需要定期進(jìn)行超聲檢查來評(píng)估治療效果，并且要注意支具的佩戴是否合適，避免對(duì)孩子的皮膚等造成損傷。三、髖關(guān)節(jié)發(fā)育不良（Ⅲ型）的特點(diǎn)與治療特點(diǎn)：Ⅲ型髖關(guān)節(jié)發(fā)育不良，分為Ⅲa和Ⅲb型。Ⅲ型髖關(guān)節(jié)髖臼角43°-55°，α角40°-55°，β角55°-77°。兩類髖中股骨頭均向上外方脫位，Ⅲa軟骨頂為無回聲結(jié)構(gòu)，是透明軟骨成分，Ⅲb的軟骨頂可見有程度不同回聲，說明透明軟骨可能發(fā)生了纖維化或變性改變。在單一冠狀聲像上，脫位的股骨頭和髖臼不能同時(shí)完整顯現(xiàn)，參考測(cè)量點(diǎn)難以辨認(rèn)5。治療：對(duì)于Ⅲ型髖關(guān)節(jié)發(fā)育不良，早期（6-7個(gè)月以下）可采用保守治療，如使用Pavlik吊帶、支具等。從經(jīng)驗(yàn)和文獻(xiàn)的報(bào)道來說，這種保守治療對(duì)于這個(gè)年齡段的孩子成功率可以達(dá)到90%以上。在使用支具或吊帶期間，需要密切關(guān)注孩子髖關(guān)節(jié)的復(fù)位情況，定期進(jìn)行超聲檢查。如果保守治療不成功，可以改用麻醉下石膏復(fù)位。經(jīng)過復(fù)位的關(guān)節(jié)對(duì)髖臼的刺激，局部發(fā)育就會(huì)逐步的改善。同時(shí)，在治療過程中，還需要關(guān)注孩子下肢的血液循環(huán)、神經(jīng)功能等情況，避免因治療導(dǎo)致其他并發(fā)癥的出現(xiàn)。四、髖關(guān)節(jié)脫位（Ⅳ型）的特點(diǎn)與治療特點(diǎn)：Ⅳ型髖關(guān)節(jié)脫位，髖臼角55°，α角40°，β角77°。此型髖的聲學(xué)特點(diǎn)是股骨頭脫位，表面只有薄層關(guān)節(jié)囊覆蓋，髖臼唇盂和軟骨頂也向原始髖臼的內(nèi)下方移位。在B超下α角甚至測(cè)量不出，屬于髖關(guān)節(jié)高位脫位的情況212。治療：對(duì)于Ⅳ型髖關(guān)節(jié)脫位，因?yàn)槊撐磺闆r較為嚴(yán)重，一般需要積極治療。對(duì)于6-7個(gè)月以下的嬰兒，如果保守治療（如Pavlik吊帶等）效果不佳，可考慮麻醉下石膏復(fù)位。而對(duì)于年齡較大的孩子（7-18個(gè)月），可能需要進(jìn)行麻醉下閉合復(fù)位或者切開復(fù)位，然后用石膏固定。在復(fù)位過程中，要確保股骨頭準(zhǔn)確復(fù)位到髖臼內(nèi)，并且要關(guān)注復(fù)位后髖關(guān)節(jié)的穩(wěn)定性。在術(shù)后，需要長期的康復(fù)隨訪，觀察髖關(guān)節(jié)的發(fā)育情況以及孩子的下肢功能恢復(fù)情況，預(yù)防股骨頭壞死、髖關(guān)節(jié)僵硬等并發(fā)癥的發(fā)生。五、髖關(guān)節(jié)半脫位（Ⅴ型）的特點(diǎn)與治療特點(diǎn)：Ⅴ型髖關(guān)節(jié)半脫位，髖臼角55°，α角40°，β角77°-90°。髖關(guān)節(jié)處于半脫位狀態(tài)，股骨頭部分脫離髖臼，關(guān)節(jié)的穩(wěn)定性受到影響，髖臼對(duì)股骨頭的覆蓋不完全，這會(huì)影響髖關(guān)節(jié)的正常發(fā)育和功能1。治療：對(duì)于Ⅴ型髖關(guān)節(jié)半脫位的治療與Ⅲ型髖關(guān)節(jié)發(fā)育不良類似。在早期可嘗試保守治療，如使用Pavlik吊帶或支具，并且定期復(fù)查超聲，觀察髖關(guān)節(jié)復(fù)位情況。如果保守治療失敗，根據(jù)孩子的年齡可選擇麻醉下石膏復(fù)位（6-7個(gè)月以下）或者其他更復(fù)雜的復(fù)位手術(shù)（年齡較大時(shí)）。在整個(gè)治療過程中，同樣要關(guān)注髖關(guān)節(jié)的功能恢復(fù)和發(fā)育情況，避免出現(xiàn)并發(fā)癥。2024年12月21日
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陶可主治醫(yī)師 北京大學(xué)人民醫(yī)院 骨關(guān)節(jié)科 臨界髖關(guān)節(jié)發(fā)育不良BorderlineDDH：什么時(shí)候、怎么判定為異常？作者：SarahDBixby,MichaelBMillis.作者單位:DepartmentofRadiology,BostonChildren'sHospital,Main2,300LongwoodAve.,Boston,MA,02115,USA.sarah.bixby@childrens.harvard.edu.譯者：陶可（北京大學(xué)人民醫(yī)院骨關(guān)節(jié)科）摘要臨界髖關(guān)節(jié)發(fā)育不良是指髖臼形狀和覆蓋范圍輕度低于正常范圍，可能導(dǎo)致兒童易患機(jī)械功能障礙和不穩(wěn)定。臨界發(fā)育不良通常包括外側(cè)中心邊緣角(CEA)為18-24°的兒童。一些具有臨界X線測(cè)量值的兒童具有正常的關(guān)節(jié)力學(xué)和功能，而其他兒童則需要進(jìn)行髖臼截骨手術(shù)。雖然臨界髖關(guān)節(jié)發(fā)育不良的X線檢查結(jié)果可能表明不穩(wěn)定，但最終診斷基于病史和體格檢查以及影像學(xué)相結(jié)合?；加信R界髖關(guān)節(jié)發(fā)育不良的兒童有時(shí)還需要進(jìn)行其他橫斷面成像研究，如MRI成像，以評(píng)估不穩(wěn)定的次要證據(jù)，包括沿髖臼邊緣的損傷或盂唇退化和肥大。CT也有助于描繪3-D髖臼形態(tài)，以進(jìn)行術(shù)前評(píng)估和規(guī)劃。兒童放射科醫(yī)生通常是第一個(gè)在X線片上發(fā)現(xiàn)邊緣性或輕度發(fā)育不良的人。兒童放射科醫(yī)生必須充當(dāng)有效的顧問，并作為針對(duì)這一復(fù)雜患者群體的連貫多學(xué)科診療團(tuán)隊(duì)的一部分提供適當(dāng)?shù)慕ㄗh。圖1?一名16歲女孩的髖關(guān)節(jié)X線片顯示了外側(cè)中心邊緣角(CEA)、髖臼指數(shù)和前CEA的測(cè)量方法。a標(biāo)記了三個(gè)點(diǎn)以便進(jìn)行后續(xù)測(cè)量：點(diǎn)1位于股骨頭(旋轉(zhuǎn))中心，點(diǎn)2位于股骨髖關(guān)節(jié)(負(fù)重關(guān)節(jié)面的)內(nèi)側(cè)邊緣，點(diǎn)3位于股骨髖關(guān)節(jié)(負(fù)重關(guān)節(jié)面的)外側(cè)邊緣。b右髖關(guān)節(jié)的前后位X線片展示了測(cè)量外側(cè)CEA的方法。外側(cè)CEA是連接點(diǎn)1和點(diǎn)3的線與垂直線之間形成的夾角（正常為25-40°）。c髖臼指數(shù)由連接點(diǎn)2和點(diǎn)3的線與水平線之間形成的夾角決定（正常為0-10°）。d右髖關(guān)節(jié)的假斜位片顯示了確定前CEA的測(cè)量方法。前側(cè)CEA由點(diǎn)1到點(diǎn)4（在髖臼前緣）的連線與垂直線之間的角度決定（正常值為25–40°）圖2?一名19歲女性嚴(yán)重雙側(cè)髖關(guān)節(jié)發(fā)育不良。a骨盆前后位X線片顯示右側(cè)髖關(guān)節(jié)外側(cè)中心邊緣角(CEA)為5°，b髖臼指數(shù)為16°。左髖關(guān)節(jié)同樣發(fā)育不良。c右髖關(guān)節(jié)假斜位X線片顯示前方中心邊緣角為8°。圖3?一名右側(cè)髖關(guān)節(jié)疼痛的16歲女孩的外側(cè)中心邊緣角(CEA)。a前后位(AP)骨盆X線片顯示外側(cè)CEA為19°。左側(cè)髖關(guān)節(jié)正常。b同一女孩的假斜位X線片顯示前方CEA為19°。c延遲釓增強(qiáng)軟骨MRI(dGEMRIC)檢查的冠狀T1圖顯示軟骨正常。女孩沒有不穩(wěn)定的跡象，接受了腰肌勞損治療，癥狀在沒有手術(shù)的情況下得到改善。由于腰肌勞損仍然可能與潛在的微不穩(wěn)定有關(guān)，女孩在髖關(guān)節(jié)外科醫(yī)生的護(hù)理下接受長期觀察。圖4?一名有髖關(guān)節(jié)發(fā)育不良家族史的11歲女性足球運(yùn)動(dòng)員的左側(cè)髖關(guān)節(jié)疼痛。a前后位(AP)X線片顯示為正常。b女孩現(xiàn)在13歲，仍然有左側(cè)髖關(guān)節(jié)疼痛。雙側(cè)髖關(guān)節(jié)被認(rèn)為是淺的，左側(cè)測(cè)量的外側(cè)中心邊緣角(CEA)為16°，右側(cè)為23°。左側(cè)髖臼指數(shù)也被認(rèn)為是升高的13°。陽性恐懼測(cè)試反映了體格檢查中左側(cè)髖關(guān)節(jié)的不穩(wěn)定，右側(cè)沒有發(fā)現(xiàn)不穩(wěn)定。C假斜位X線片顯示前方覆蓋充分，前方CEA為27°。d左側(cè)髖臼周圍截骨術(shù)后前后位AP骨盆X線片狀態(tài)顯示側(cè)方覆蓋改善。女孩報(bào)告癥狀有所改善。e一年后的前后位AP骨盆X線片，癥狀緩解并恢復(fù)正常活動(dòng)。她右側(cè)有輕微間歇性疼痛，并定期監(jiān)測(cè)癥狀。圖5?股骨骨骺髖臼頂(FEAR)指數(shù)。a與圖1中相同的16歲女孩的髖關(guān)節(jié)前后位(AP)X線片。FEAR指數(shù)是沿髖臼頂連接點(diǎn)2和點(diǎn)3的線（黑線）與沿股骨骨骺線中央三分之一繪制的線（白線）之間形成的角度。陽性FEAR指數(shù)定義為向外側(cè)傾斜的角度，頂點(diǎn)指向內(nèi)側(cè)。這個(gè)女孩的FEAR指數(shù)小于5°。與FEAR指數(shù)<5°相比，F(xiàn)EAR指數(shù)>5°與不穩(wěn)定性具有更大的相關(guān)性。b一名13歲女孩的髖關(guān)節(jié)前后位X線片顯示左側(cè)髖臼外側(cè)輕微上翻（箭頭）。c一名13歲男孩的右側(cè)髖關(guān)節(jié)疼痛的左側(cè)髖關(guān)節(jié)正常前后位X線片顯示外側(cè)正常。黑線表示髖臼頂部，白線表示骨骺線的中央三分之一。此正常髖關(guān)節(jié)的FEAR指數(shù)顯示FEAR指數(shù)，角度頂點(diǎn)指向外側(cè)。圖6?一名18歲女子田徑運(yùn)動(dòng)員的右側(cè)髖關(guān)節(jié)疼痛影像。a前后位(AP)骨盆X線片顯示右側(cè)髖關(guān)節(jié)發(fā)育不良，左髖關(guān)節(jié)正常。b右側(cè)髖臼周圍截骨術(shù)后前后位(AP)骨盆狀態(tài)顯示外側(cè)覆蓋增加，但該女性報(bào)告癥狀惡化。c術(shù)前（回顧性）進(jìn)行的右髖關(guān)節(jié)矢狀質(zhì)子密度脂肪抑制MR圖像顯示前髖臼內(nèi)有骨樣骨瘤（箭頭）。射頻消融后，該患者的癥狀得到緩解。?Theborderlinedysplastichip:whenandhowisitabnormal?AbstractBorderlineacetabulardysplasiareferstomildlysub-normalpatternsofacetabularshapeandcoveragethatmightpredisposechildrentomechanicaldysfunctionandinstability.Borderlinedysplasiagenerallyincludeschildrenwithalateralcenteredgeangle(CEA)of18-24°.Somechildrenwithborderlineradiographicmeasurementshavenormaljointmechanicsandfunctionwhileothersbenefitfromacetabularreorientingsurgery.Althoughradiographicfindingsofborderlinedysplasiamightsuggestinstability,theultimatediagnosisisbasedonhistoryandphysicalexaminadditiontoimaging.Childrenwithborderlineacetabulardysplasiasometimesbenefitfromothercross-sectionalimagingstudiessuchasMRimagingtoevaluateforsecondaryevidenceofinstability,includingdamagealongtheacetabularrim,orlabraldegenerationandhypertrophy.CTisalsohelpfulfordepictionof3-Dacetabularmorphologyforpreoperativeassessmentandplanning.Pediatricradiologistsareoftenthefirsttoidentifyborderlineormilddysplasiaonradiographs.Itisimperativethatpediatricradiologistsserveaseffectiveconsultantsandofferappropriaterecommendationsaspartofacohesivemultidisciplinaryapproachtothiscomplexpatientpopulation.Fig.1Radiographsofthehipina16-year-oldgirldemonstratemeasurementtechniqueforlateralcenteredgeangle(CEA),acetabularindex,andanteriorCEA.aThreepointsaremarkedforsubsequentmeasurements:Point1atthecenterofthefemoralhead,Point2atthemedialedgeofthesourcil,Point3atthelateraledgeofthesourcil.BAnteroposteriorpelvicradiographconeddowntotherighthipdemonstratestechniqueformeasuringlateralCEA.ThelateralCEAistheangleformedbetweenthelineconnectingPoint1toPoint3andaverticalline(normal25–40°).cAcetabularindexisdeterminedbytheangleformedbetweenalineconnectingPoint2toPoint3andahorizontalline(normal0–10°).dFalseprofileviewoftherighthipdemonstratesmeasurementtechniquefordetermininganteriorCEA.AnteriorCEAisdeterminedbytheanglebetweenalinefromPoint1toPoint4(attheanteriormarginofthesourcil)andaverticalline(normal25–40°)Fig.2Severebilateralhipdysplasiaina19-year-oldwoman.aAnteroposteriorradiographofthepelvisdemonstratesalateralcenteredgeangle(CEA)oftherighthipof5°and(b)acetabularindexof16°.Thelefthipissimilarlydysplastic.cFalseprofileradiographoftherighthipdemonstratesanteriorcenteredgeangleof8°.Fig.3Borderlinelateralcenteredgeangle(CEA)ina16-year-oldgirlwithrighthippain.aAnteroposterior(AP)pelvisradiographrevealsborderlinelateralCEAof19°.Thelefthipisnormal.bFalseprofileradiographofthesamegirlrevealsanteriorCEAof19°.cCoronalT1mapfromdelayedgadolinium-enhancedMRIofcartilage(dGEMRIC)examinationrevealsnormalcartilage.Thegirldidnothavesignsofinstability,wastreatedforpsoasstrain,andsymptomsimprovedwithoutsurgery.Becausepsoasstraincouldstillberelatedtounderlyingmicroinstability,thegirlwasunderlong-termobservationundercareofahipsurgeon.Fig.4Lefthippaininan11-year-oldfemalesoccerplayerwithafamilyhistoryofhipdysplasia.aAnteroposterior(AP)radiographinterpretedasnormal.bGirlnowisage13years,stillwithlefthippain.Bilateralhipswereconsideredshallow,withlateralcenteredgeangle(CEA)measuredat16°ontheleftand23°ontheright.Acetabularindexontheleftwasalsoconsideredelevatedat13°.Positiveapprehensiontestreflectedinstabilityofthelefthiponphysicalexamination,withnoinstabilitynotedontheright.cFalseprofileradiographrevealsadequateanteriorcoverage,withanteriorCEAof27°.dAPpelvisradiographstatuspostleft-sideperiacetabularosteotomydemonstratesimprovedlateralcoverage.Thegirlreportedimprovedsymptoms.eAPpelvisradiograph1yearlater,afterreliefofsymptomsandreturntonormalactivity.Shehadmildintermittentpainontherightandwasbeingmonitoredperiodicallyforsymptoms.Fig.5Femoro-epiphysealacetabularroof(FEAR)index.AAnteroposterior(AP)radiographofthehipinthesame16-year-oldgirlasinFig.1.TheFEARindexistheangleformedbetweenalineconnectingPoint2andPoint3alongtheacetabularroof(blacklines),andalinedrawnalongthecentralthirdofthefemoralphysealscar(whiteline).ApositiveFEARindexisdefinedbyalaterallydirectedanglewiththeapexpointingmedially.TheFEARindexinthisgirlislessthan5°.AFEARindex>5°hasagreatercorrelationwithinstabilitycomparedtoFEARindex<5°.bAPradiographofthehipina13-year-oldgirlwithmildleftacetabularsourcildemonstratesamildlyupturnedlateralsourcil(arrow).cNormalAPradiographofthelefthipina13-year-oldboywithrighthippaindemonstratesanormallateralsourcilforcomparison.Theblacklineindicatestheacetabularroofandthewhitelineindicatesthecentralthirdofthephysealscar.TheFEARindexisdemonstratedinthisnormalhiptoillustrateanegativeFEARindexwiththeapexoftheangledirectedlaterally.Fig.6Imaginginan18-year-oldfemaletrackathletewithrighthippain.aAnteroposterior(AP)pelvisradiographreportedasborderlinerightacetabulardysplasiaandnormallefthip.bAPpelvisstatuspostrightperiacetabularosteotomydemonstratesincreasedlateralcoverage,thoughthewomanhadreportedworseningsymptoms.cSagittalproton-densityfat-suppressedMRimageoftherighthipperformedpreoperatively(retrospectively)revealsanosteoidosteomawithintheanterioracetabulum(arrow).Thewoman’ssymptomsresolvedafterradiofrequencyablation.?AdvancinginvestigationTheterm“borderlinedysplasia”isalsofallingoutoffavor.Advancedimagingmodalitieshaverevealedpatternsofdysplasiathatarenotapparentonradiographs.FocalanteriorandposteriordysplasiagroupshaveanormallateralCEAontheAPradiograph[9].Refinedandupdatedradiographicmeasurementshavebeenproposedthatwouldenablebetteridentificationofchildrenwithfocaldysplasia,suchastheanteriorwallindexandposteriorwallindex[48].Earlystudiesdemonstrateddifferencesintheanteriorandposteriorwallindicesinsymptomaticdysplasticpatientscomparedtothosewithanormalacetabulum[48].Subsequentinvestigationrevealedthatevenasymptomaticpeoplehaveradiographicanteriorandposteriorwallindexmeasurementsthatoverlapthoseofpeoplewithdysplasia[49].Thisindicatesthatsomedegreeofvariationinthe3-Dmorphologyoftheacetabulumisnormal.Furthervalidationoftheseindiceswithcross-sectionalimagingandlongitudinalfollow-upisnecessarybeforethesenewreferencestandardscanbeconsideredreliableindicatorsofdisease.Inthepresenceofinstabilityrelatedtoacetabulardysplasia,thereisoftenovergrowthofsoft-tissuestructuresthatcompensatesforthedeficientbonysupport.Thisincludesenlargementoftheacetabularlabrum[50,51],evenintheabsenceoflabraltearordegeneration.Focalmuscleenlargementhasalsobeennotedinunstablepatients,specificallytheiliocapsularismuscle[52].MRImightbeusefulinidentifyingthesesecondarysignsofinstability.Still,noclearlypositivefindingsconfirmthepresenceofinstability.Femoralversionisalsoakeycomponentindetermininghipstability,asanantevertedfemurisbemoreanteriorlyuncoveredthananeutralfemur[53].Overtfeaturesofacetabularrimdamagealsosupportthediagnosisofdysplasia,includinglabraldegenerationandtearingandcartilageloss,thoughadolescentswhohaveborderlinedysplasiamightnotyethavevisiblemanifestationsofosteoarthritis,evenifinstabilityisthepaingenerator.Giventheseareasofinvestigationanduncertainty,theborderlinedysplastichiphasattractedwell-deservedattentionintheliterature.Specificconcernshavebeenraisedaroundwhethertheterm“borderlinedysplasia”isanadequatelabelandwhetherthisisasinglecondition.ItismorelikelythatchildrenwithaborderlinelateralCEAof18–24°consistofclustersofpatients,someofwhommighthavecamimpingement,andsomeofwhomhavefocalacetabulardeficiency[13].Specificpatternsofacetabulardeficiencyandfemoralmorphologyarebestcharacterizedwithcross-sectionalimagingexaminations,suchasMRIorCT,whichmightalsodetectothercausesforhippain.ItisrecommendedthatanychildinwhomthereisconcernfordysplasiaundergoanMRIaspartofacompleteevaluationbecausetheremightbeanotherfindingthatexplainsthechild’ssymptoms(Fig.6),orevidenceofintra-articulardamagethatsupportsrimloading.MRIiswellsuitedfordetectingcartilageandlabralabnormalitiesaswellasmarrowlesionsthatarepresentinthesettingofalteredbiomechanicsandearlyosteoarthritis[8].Low-dosepelvicCTisalsovaluableforpreoperativeassessmentofthehipmorphologywithprecisecharacterizationofthebonydeficienciesinthreedimensions.Itiscrucialthatthesechildrenaredirectedtoanexperiencedhipspecialistwhoisabletocontextualizetheimagingfindingswithacomprehensivephysicalexamandanappropriatehistory.Thesechildrenshouldbeinterrogatedwithrespecttothenatureandlocationoftheirpainwithspecificquestionsaroundinstability.Acomprehensivephysicalexamshouldfollow,includingattentiontothechild’sgait,pelvicpositionandrotationalprofile.ConclusionTheterm“borderlinedysplasia”referstopatternsofacetabularcoveragethatmightpredisposechildrentoinstability.Somechildrenwithborderlineradiographicmeasurementshavenormaljointmechanicsandfunction(Fig.1ShouldsayFigure3),whileothersbenefitfromacetabularreorientingsurgery(Fig.2ShouldsayFigure4)[11].Itisimportantthatradiologistsreflectthisuncertaintyintheirreportswithappropriatemanagementrecommendations.Instabilitymightbesuggestedbyradiographs,butultimatediagnosisisconfirmedonthebasisofhistoryandphysicalexamassessingforinstability.Thesechildrenshouldundergohigh-resolutionMRimagingofthehiptoevaluateforjointdamage,andCTmightbehelpfulforbetter3-Dcharacterizationofthebonyshapeandcontour.Dynamicultrasonographyhasbeenvalidatedasahelpfuladjunctinthedeterminationofinstabilitybymeasuringanteriorfemoralheadtranslationwithdynamicmaneuversreplicatingtheapprehensiontest[54].Aspediatricradiologists,weareoftenthefirsttoidentifyborderlineormilddysplasiaonthebasisofradiographs.Itisimperativethatweserveaseffectiveconsultantsandofferappropriaterecommendationsaspartofacohesivemultidisciplinaryapproachtothiscomplexpatientpopulation.?文獻(xiàn)出處：SarahDBixby,MichaelBMillis.Theborderlinedysplastichip:whenandhowisitabnormal?ReviewPediatrRadiol.2019Nov;49(12):1669-1677.doi:10.1007/s00247-019-04468-4.Epub2019Nov4.??IntroductionDevelopmentalhipdysplasia(DDH)isoneofthemostimportantandmostcommonpediatricmusculoskeletalconditions.Whileasmanyas80%ofcasesarepresentatbirth,manyremainundiagnosed.Whenpresentininfancy,DDHmightbedetectedonthebasisofphysicalexaminationfindings(i.e.BarlowandOrtolanimaneuvers)andstaticanddynamicultrasoundfeaturesoriginallydescribedbyGraf[1,2].Thelong-termimplicationsofDDHaresignificantbecausetheconditionleadstodevelopmentofosteoarthritisin25–50%ofpatientsbytheageof50years[3].Thereducedsizeandtheincreasedobliquityoftheacetabularweight-bearingsurfacecreateshearingforcesonthearticularcartilageandcausechronicoverloadingoftheanteriorandanterolateralacetabularrim[4].Thismechanicaldysfunction,ifuncorrected,leadsinadulthoodtopain,abductorfatigueandoftensymptomsofinstability,culminatingingradualfailureofthecartilageandleadingtoprogressiveosteoarthritis.Treatmentstrategiesdependonthemechanicalstabilityofthehipandthetypeanddegreeofbonydeformity.IninfantswithmildDDH,capsularlaxityandmildacetabulardysplasiaaretheissues,andsimplepositioningofthehipsinabductionandflexioninaprotectivebraceorPavlikharnessusuallyleadstotighteningofthecapsuleandresolutionofthedysplasia.Inchildrenwithfullcongenitaldislocations,particularlyifdiagnosedafterinfancy,aformalmanipulativereductionmightberequired,withspicacastingforseveralmonths.Atanyage,treatmentisfocusedonreducingandmaintainingthefemoralheadtoaconcentricpositionwithintheacetabulum.IfbonymalalignmentispresentintheolderchildwithDDH,realignmentsurgeryisoftenneededtorestorestability.Infantsandchildrenwithdevelopmentalhipdysplasiamightbetreatedtocurebyvirtueoftheseearlystrategies,ortheymighthavepersistentsubluxationthatrequiresfurthersurgerylaterinadolescenceoryoungadulthood.DysplasiainadolescentsAdolescentsandyoungadultswhohadbeenasymptomaticwithrespecttothehipmightalsohavemildformsofacetabulardysplasiadetectedonradiographsbasedoncriteriaoriginallydefinedbyWiberg(Figs.1and2)[5].Inmanychildrentheindicationforradiographsishippain,thoughforsomechildrenradiographsareperformedforotherindications.Intheabsenceofadedicatedexaminationbyahipspecialistwhocanassessforsignsorsymptomsofinstability,itisnotknownwhethermildorsubtleradiographicabnormalitiesarethesourceofthechild’ssymptoms.Radiographicmeasurementsoffemoralheadcoverageandpositionmightsuggestthepossibilityofmechanicaldysfunctionofthehip,thoughitisthemechanicsthatdefinetheunderlyingdisease,nottheradiographs.IncontrasttoinfantileDDH,adolescentdysplasiahasahighermalepredominanceandismoreoftenbilateral[6].Thedifferenceindemographicsbetweengroupshaspromptedmanytoquestionwhetheradolescentandinfantilehipdysplasiasaretwodistinctentities.Thestandinganteroposterior(AP)radiographofthepelvisremainsthegoldstandardofimagingforadolescenthipdysplasia,supplementedbyotherviewsincludingthefalseprofileradiograph[4].Anumberofradiographicmeasurementshavebeendescribedthatdefinethedysplastichip,thethreemostfundamentalincludingthelateralcenteredgeangle(CEA),theacetabularindexandtheanteriorCEA[7].ThelateralCEAandtheacetabularindexarebothmeasuredonaproperlypositionedstandingAPradiographofthepelvis,whereastheanteriorCEAismeasuredonthefalseprofileradiograph(Figs.1and2).In1939Wiberg[5]definedanormallateralCEAasbeingover25°,anabnormalangleaslessthan20°(Fig.2)andeverythinginbetweenasuncertain.Theserangeswerevalidatedinsubsequentinvestigations[8,9].SimilarcriteriaexistfortheanteriorCEAasmeasuredonafalseprofileradiograph,wherethisangleisconsiderednormalabove25°,borderlineat20–24°anddeficientbelow20°(Fig.2)[10].Incertainchildrentheanterioracetabularroofinsufficiencyismoreseverethanthelateralroofinsufficiency,andthefalseprofileviewmighthelptoidentifythesechildren,withthecaveatthattheanteriorCEAisthemostdependentonradiographictechnique[11].Theacetabularindexisconsiderednormalat0–10°[7],thoughsomehavesuggestedthatanglesupto13°arenormal[8].Valuesabovethisareconsideredindicativeofdysplasia.BorderlinemeasurementsWhilefloridacetabulardysplasiaisincontrovertiblewhenidentifiedradiographically(Fig.2),thecorrectdiagnosisbecomesmoredifficultwhenthemeasurementanglesareonlymildlyoutofthenormalrange,leadingtothecreationofan“uncertain”or“borderline”category(Figs.3and4).Thesechildrenremainasourceofconfusionandcontroversyamongradiologistsandhipspecialists.Ultimately,achildfallingintoanuncertaincategoryhaseitherahealthyoranunhealthyhip,andthisdistinctiondependsonavarietyoffactorsuniquetoeachchildthatgobeyondasimpleradiographicmeasurement.Thelabel“borderlinedysplasia”hasbeenadoptedtodefineagroupthatfallsintoanuncertainmeasurementcategorywithlateralCEAof18–24°[12–14],inwhomfurtherevaluationisnecessarybeforeadiagnosiscanbemade.Giventhemechanicalbasisforthejointdamageinacetabulardysplasia,itisareasonableassertionthathipswithslightlydiminishedcoveragearepredisposedtojointdamagerelatedtoincreasedwearontheacetabularrim.Childrenwithmilddysplasiaareknowntohaveevidenceoflabralandcartilagedamageathiparthroscopy[15].ThisdoesnotmeanthatallchildrenwithalateralCEAof18–24°developosteoarthritis.Earlystudiesevaluatingtherelationshipbetweencenteredgeangleanddysplasiafocusedprimarilyonhipfunctionratherthanspecificevidenceofjointdamage[16],thoughitislikelythatnormallyfunctionalhipsmightovertimealsohavelabraltearsandcartilagelesions.Delayedgadolinium-enhancedMRIofcartilage(dGEMRIC)measurementsDelayedgadolinium-enhancedMRIofcartilage(dGEMRIC)wasdevelopedtoidentifyhipswithearlybiomechanicaldamagetothecartilagematrixinadvanceofmorphologiccartilageloss[17].Whenintroducedviaintravenousorintraarticularinjection,ananionicmoleculesuchasgadopentetate?2(Gd-DTPA?2)distributesovertimeincartilageinverselytotheconcentrationofnegativelychargedglycosaminoglycans.TheconcentrationofGd-DTPA?2canbeindirectlydeterminedwithmeasurementsofT1andisexpectedtobelowerinnormalcartilagecomparedtodegradedcartilagewithlossofglycosaminoglycans.Thismeasurementisreferredtoasthe“dGEMRICindex”(Fig.3).EvaluationofthedGEMRICindexinpeoplewithnoormilddysplasiarevealedthatthedGEMRICindexofmildlydysplastichipsdidnotdiffersignificantlyfromthatofnormalhips[17].ItshouldbenotedthatinthisstudypeoplewithmilddysplasiaweredefinedbyalateralCEA>15°,whichislowerthanwhatwouldnowbeconsideredthethresholdofmilddysplasia.Thesedatasuggestthatchildrenwithmildorborderlinedysplasiamightnotbeatincreasedriskofdevelopingend-stageosteoarthritis,thoughbecausethesepeoplewerenotfollowedlongitudinallyovertimeitisunknownwhethercartilagedegenerationevolvedovertime.ThedGEMRICindexalsodoesnotaddresswhetherchildrenhavesignsorsymptomsofinstability,whichmightbewhatbringsthemtomedicalattention.RadiographiclandmarksMeasurementsobtainedfromconventionalradiographsrelyonidentificationofpreciselandmarks,includingthecenterofthefemoralhead,themedialmarginoftheacetabularsourcil,andthelateralmarginoftheacetabularsourcil(Fig.1).Thesourcilisnotalwayswell-defined,especiallyinchildrenyoungerthan15years.Inyoungerchildrenitisuncommonforthelateralmarginofthesourciltoalsobethelateralmarginoftheacetabulum,promptingsomeinvestigatorstodevelopa“modifiedlateralCEA”thatincludesonlythescleroticportionoftheacetabularsourcil.ThisisincontrasttothetraditionalCEA,whichismeasuredtothelateralacetabularmargin[18].Therangeofnormaldependsontechniquebecausethemodifiedanglehasalowerstandardrange(15–20°)comparedtothetraditionalangle.Withoutstrictattentiontoradiographiclandmarksthevariabilitybetweenmeasurementscanbeextreme[19–21].Themostreliablemethodistoautomatetheprocesswithcomputer-aidedsoftware,eitherbyincorporatingacomputerizedmeasurementprogram[22]orbystandardizingtheprojectionoftheradiograph[23].Unlessrigorouscriteriaarebeingusedformeasurement,amildlyabnormalmeasurementshouldnotbeconsideredamarkofdiseaseunlessthereareothercompellingimagingandclinicalfindings.Anadhocmeasurementperformedby“eyeballing”theradiographisunlikelytobeaccurate.Itisbesttomeasuremultipletimes,especiallyintheabsenceofcomputer-aidedsystems.NormalvariantsCrossoversignRadiologistsarewisetoproceedcautiouslyaroundthehip,giventhefrequencywithwhichpreviouslyreportedradiographicmeasurementsorfindingshavebeensubsequentlydeterminedtorepresentnormalvariants.AnexampleofthisisthecrossoversignontheAPpelvisradiographs.In2007itwasdemonstratedthatthepresenceofacrossoversignwasahighlyreliableindicatorofcranial(superioracetabular)anteversionoflessthan4°[24].Forreference,thesuperioraspectoftheacetabulumisantevertedapproximately10–14°.Atangleslessthan0°,theanteriorwallislateraltotheposteriorwall,leadingtothecrossoversignonradiographswherethetwowallsoverlap.ItbecamestandardforradiologistsandhipspecialiststocommentonthepresenceofacrossoversignonAPradiographsofthepelvisassuggestiveofacetabularretroversion.Inclinicalpractice,however,thesechildrendidnotalwayshavesignsorsymptomsofretroversion,nordidcross-sectionalimagingconfirmretroversion.Overtime,theliteraturerefutedanassociationbetweencrossoversignandclinicallyconfirmedacetabularretroversion.Evenasymptomaticchildrenwithouthipdiseaseorsymptomatologydemonstratedacrossoversignonawellpositionedradiograph,reflectingvariationsinpatientpositioningaswellasthevariablemorphologyoftheanteriorinferioriliacspine[25,26].CoxaprofundaInsimilarfashion,theterm“coxaprofunda”fellinandoutoffavoralmostasquickly.Coxaprofundaisdefinedaspresentiftheflooroftheacetabularfossaliesmedialtotheilioischialline.Itisconsideredanindirectsignofacetabularovercoverageofthefemoralheadandwasproposedasanimagingfeatureofpincer-typefemoroacetabularimpingementin2007[27].Withthisawareness,radiologistsreadilyofferedthisimagingfindingasevidenceofanunderlyingcondition:acetabularover-coverage.Overtime,coxaprofundawasclaimedtobeanormalradiographicfindingthatdoesnotsupportadiagnosisofpincerimpingement[28–30].CamdeformityFinally,thedefinitionof“cam”deformityinchildrenwithfemoroacetabularimpingementhasbeenasubjectofinterestanddebateformanyyears.Camlesionsarebonyprotuberancesalongthefemoralhead/neckjunctionthatimpingeagainsttheacetabularriminhipflexion.Themostobjectivemeansofmeasuringthesizeofacamdeformityisthealphaangle,anangleformedbyalineconnectingthecenterofthefemoralheadtothecenterofthefemoralneck,andalinefromthecenterofthefemoralheadtothepointatwhichthefemoralneckfallsoutsideabest-fitcirclearoundthehead.Intheearly2000sitwasacceptedthatchildrenwithanalphaangleintherangeof50°likelyhadcam-typefemoroacetabularimpingement(FAI)[31–34].OverthelastdecadetherehasbeenincreasingawarenessthatsomepreviouslydefinedcamlesionsinpeoplewithFAImightbepresentinasymptomaticpopulationswithnohipdisease[35–38].Moreoverapositiveimpingementtest,oftenassociatedwiththepresenceofanteriorFAI,hasbeendemonstratedinhealthyyoungadultswhomightnothaveFAI[39],makingthisanunreliableindicatorofdiseaseinisolationofotherevidence.RadiographictechniqueGiventhatourinterestinidentifyingandtreatingpainfulanddebilitatingdiseaseinchildrenmightoutpaceourunderstandingofnormalanatomicalvariation,radiologistsneedguidelinesforinterpretingradiographsthatrevealanuncertaindegreeoffemoralheadcoverage.Acautiousapproachwouldbetosuggestthepossibilityofborderlinehipdysplasiaandrecommendreferraltoahipspecialist.Thisrecommendationshouldbeperformedwhentheimagingfindingshavebeendeemedreliable,whichrequiresstrictadherencetoproperimagingtechnique.ThefollowingshouldbeassessedoneveryAPradiographofthepelvis:(1)Isthepelvistiltedorrotated?Asaguideline,thedistancebetweenthesuperioredgeofthepubicsymphysisandthecoccyxshouldbe1–3cm[40].(2)Howwelldefinedarethemeasurementlandmarks?Ifthereisdoubtastowherethelandmarksarelocated,themeasurementsarelikelytobeinaccurate.(3)Aremeasurementsperformedusingelectroniccalipersorwithavalidatedcomputer-assistedprogram?Ifperformedbyhand,havetheinitialmeasurementsbeenvalidatedwitharepeatattempt?(4)Isthepatientolderthan15years,andifnotisthemodifiedlateralCEAstandardbeingemployedratherthantheclassiclateralCEA?Theanswerstothesequestionshaveagreatimpactonthereportedmeasures.Iftheimagingtechniqueisadequateandthechildstillfallsintoanindeterminatecategoryoftheborderlinedysplastic(alateralCEAthatfallsbetween18°and24°),thisisstillonlythefirststepinacomplexdiagnosticprocess.Ourunderstandingofhipdiseasehasevolvedconsiderablyoverthelastdecade.Itisimpossibletoaccuratelycharacterizeallofthedifferentpatternsofinstabilityandunder-coveragewith2-Dradiographicviews.RelyingsolelyonthelateralCEAtodeterminenormalversusdeficientcoverageassumesalldysplasiaisglobal,orprimarilyinvolvesthesuperioracetabulum.Wenowknowthatatleastthreedistinctpatternsofacetabulardeficiencyexist:anterosuperior,global,andposterosuperiorinsufficiency[41],andAPradiographsarenotdesignedtodetectafocalanteriororposteriordeficiency.Thepresenceofborderlinedysplasiaonradiographsalsodoesnotconfirmthepresenceofinstability,whichisultimatelywhatleadstosymptomsandjointdamage.Thepresenceorabsenceofinstabilityorimpingementmustbedeterminedthroughcarefulhistoryandphysicalexam,aswellasfromstaticandpossiblydynamicimaging.Thefemoroepiphysealacetabularroof(FEAR)indexhasbeenproposedasausefulradiographicmarkerofinstability(Fig.5)[42].Anotherhelpfulradiographiccluetothepresenceofinstabilityistheupslopinglateralsourcilmargin[43].Alloftheseobservations,however,requirefurthervalidationbeforetheycanbeconsideredreliablemarkersofdisease.Wiberg[5]laidtheimportantgroundworkwithhisseminalarticledescribingthe“normal”lateralcoverageofthefemoralhead,andformanydecadesthoseassertionshavenotbeendisproved;subsequentinvestigationshaveonlysubstantiatedhisoriginalfindings[44–46],thoughthelowerendofthenormalthresholdhasshiftedmoretowardthedysplasticendofthespectrum.Largerpopulation-basedstudieshaverecentlysuggestedthattheserangesaregender-specificandthatmaleandfemalepatientsshouldnotbemeasuredagainstthesamestandard.UpdatedreferencestandardsproposedbyLaborieetal.[39]suggestedthatcutoffvaluesformalepatientsshouldbe21°comparedto20°infemalepatients.Updatedupperthresholdvaluesforacetabularindexaccordingtothisstudywere15°formalesand16°forfemales,comparedtopreviouspublishedthresholdof10°forbothgroups.Accordingtothesenewcriteria,manypatientswhohadpreviouslybeencharacterizedasmildlyorborderlinedysplasticmightnowbeconsiderednormal.Additionally,age,gender,heightandbodymassindex(BMI)havebeenfoundtobefactorsinwhatareconsideredtobenormalrangesofacetabularcoverage,shiftingthelowerrangeofnormalcoveragefurtherintothedysplasticrangeforcertainpopulations[47].?2024年08月02日514
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張中禮主任醫(yī)師 天津醫(yī)院 小兒骨科 髖關(guān)節(jié)發(fā)育不良（Developmentaldysplasiaofthehips，DDH）是兒童最常見的肌肉骨骼先天缺陷之一，各地報(bào)道的DDH發(fā)病率在1‰~3.4‰。DDH是導(dǎo)致髖關(guān)節(jié)骨關(guān)節(jié)炎的重要原因，延誤診治或治療不當(dāng)，都將嚴(yán)重的影響成年后生活質(zhì)量。既往文獻(xiàn)報(bào)告，近40%接受髖關(guān)節(jié)置換病人和“髖關(guān)節(jié)發(fā)育不良”有關(guān)。近年來，全髖關(guān)節(jié)置換術(shù)（THA）是當(dāng)今最成功的骨科手術(shù)之一。多種原因?qū)е碌捏y部疼痛時(shí)，THA都可緩解疼痛、恢復(fù)功能并提高生存質(zhì)量。但是，有個(gè)重大的問題是技術(shù)本身克服不了的，那就是，“全髖關(guān)節(jié)置換”的“年限”和初次關(guān)節(jié)置換時(shí)年齡密切相關(guān)，與之相反的是，“翻修手術(shù)”難度大風(fēng)險(xiǎn)高，但效果反而不如初次置換。因此，對(duì)于髖關(guān)節(jié)發(fā)育不良這樣長病程的疾患，“保髖”是“全生命周期治療”中不可或缺的一個(gè)重要環(huán)節(jié)。青少年、兒童期嚴(yán)重型髖關(guān)節(jié)發(fā)育不良/脫位，通過規(guī)范手術(shù)治療可以獲得滿意的中期效果。對(duì)于行走期（walkingage）及以前發(fā)現(xiàn)的髖關(guān)節(jié)脫位孩子，可以通過“閉合復(fù)位石膏褲固定”保守治療的方法，不過，存在一定的“殘余髖關(guān)節(jié)發(fā)育不良”的幾率，部分病例需行二期手術(shù)矯正。和很多與發(fā)育相關(guān)的疾病一樣，髖關(guān)節(jié)脫位同樣需要盡可能早的發(fā)現(xiàn)、診斷，及時(shí)復(fù)位，讓髖臼-股骨頭維持良好對(duì)位，才能讓兒童髖關(guān)節(jié)沿著正?！败壍馈卑l(fā)育，從而“治愈”它。而早期針對(duì)髖關(guān)節(jié)脫位/發(fā)育不良的篩查是解決“早期診斷”的唯一出路?；仡櫩磥?，最早從1879年WilhelmRoser、后來Ortolani等醫(yī)生更多是通過體格檢查的方法檢查出兒童期“脫位”的髖關(guān)節(jié)。在我國，以上海新華醫(yī)院吳守義教授等為代表的小兒骨科前輩也陸續(xù)開展了針對(duì)髖關(guān)節(jié)脫位的篩查工作。自上世紀(jì)80年代，以奧地利Graf、美國Harcke、挪威的Rosendahl、Terjesen醫(yī)生為代表，開始利用髖關(guān)節(jié)超聲技術(shù)來評(píng)估髖關(guān)節(jié)形態(tài)，并逐漸形成了早期髖關(guān)節(jié)超聲檢查技術(shù)及分型系統(tǒng)。從而，將針對(duì)DDH的早期檢出時(shí)間大大提前。并使基于這項(xiàng)技術(shù)的、針對(duì)人群的DDH篩查成為可能。奧地利是最早針對(duì)DDH實(shí)行全民篩查（universalscreeningprogram）的國家之一，他們的數(shù)據(jù)表明，早期全民篩查可以大大降低后期需手術(shù)的比例。從衛(wèi)生經(jīng)濟(jì)學(xué)角度來說，整體上也大大降低了政府的醫(yī)療支出。我國已從政策層面高度重視，國家衛(wèi)健委在《健康兒童行動(dòng)提升計(jì)劃（2021-2025年）》中再次強(qiáng)調(diào)，要“逐步將先天性髖脫位等疾病納入篩查病種”，從而整體提升兒童骨骼發(fā)育健康。然而，西京醫(yī)院嚴(yán)亞波主任的一項(xiàng)針對(duì)兒科、兒保、婦產(chǎn)科、小兒骨科等7個(gè)專業(yè)醫(yī)生，共466份有效問卷的調(diào)查發(fā)現(xiàn)，會(huì)對(duì)嬰幼兒常規(guī)進(jìn)行髖關(guān)節(jié)查體的比例為37.9%，而常規(guī)進(jìn)行髖關(guān)節(jié)超聲的比例僅為20%。理想是豐滿的，而現(xiàn)實(shí)是骨感的。目前，以上海、北京為代表的大城市，實(shí)行的是基于危險(xiǎn)因素的“選擇性篩查”或“區(qū)域性全民篩查”，不過，仍面臨諸如政策、運(yùn)營協(xié)調(diào)等多方面的問題亟待解決?？祻?fù)醫(yī)學(xué)會(huì)修復(fù)重建外科專委會(huì)保髖學(xué)組張洪教授、羅殿中教授、程徽和楊劼教授等，在西藏地區(qū)克服重重困難、開展針對(duì)髖關(guān)節(jié)脫位的系統(tǒng)性早期篩查工作，星星之火已被點(diǎn)燃。在以天津醫(yī)院小兒骨科楊建平主任、天津市婦女兒童保健中心劉功姝主任為代表的兒保、小兒骨科醫(yī)生持續(xù)努力下，在上海第六人民醫(yī)院陳博昌主任等的技術(shù)支持下，新生兒期針對(duì)髖關(guān)節(jié)發(fā)育不良的早期篩查，和先心病、白內(nèi)障等一樣被納入“早期篩查項(xiàng)目”。天津市自2008年開始實(shí)行全市范圍內(nèi)“全民篩查”，將針對(duì)髖關(guān)節(jié)發(fā)育不良的超聲篩查納入到“天津市兒童保健手冊(cè)”、“天津市預(yù)防接種手冊(cè)”（小紅本）內(nèi)，基于信息化管理，采用“2+1”的模式，確保了篩查覆蓋率及良好的質(zhì)量控制。天津市婦女兒童保健中心潘蕾主任總結(jié)了天津市2013~2020年的“天津髖篩經(jīng)驗(yàn)”，題為“天津市嬰兒發(fā)育性髖關(guān)節(jié)發(fā)育不良的篩查結(jié)果及危險(xiǎn)因素分析”的文章發(fā)表于2022年《中華骨科雜志》上。新生兒期檢出，及時(shí)規(guī)范治療，是從臨床、影像學(xué)上“治愈”髖關(guān)節(jié)脫位/發(fā)育不良的前提。有意思的是，天津市的人群數(shù)據(jù)顯示，經(jīng)超聲確診髖關(guān)節(jié)發(fā)育不良的孩子中，僅有不足12%存在例如臀位、家族史等“危險(xiǎn)因素”。上海第六人民醫(yī)院陳博昌主任、揚(yáng)州市婦幼保健院王加寬主任積極探索利用AI技術(shù)，提高髖關(guān)節(jié)超聲檢查的效率、精準(zhǔn)度和同質(zhì)化。針對(duì)髖關(guān)節(jié)發(fā)育不良的早期篩查，意義重大，但仍需多方持續(xù)努力、協(xié)作。不讓每一名孩子輸在起跑線上，保髖，真的需要大家一起努力、從娃娃抓起啊。2024年05月30日435
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吳德超副主任醫(yī)師 深圳市兒童醫(yī)院 骨科 腿紋不對(duì)稱，臀紋不對(duì)稱今天有個(gè)三歲的寶寶來門診，媽媽說寶寶一歲時(shí)體檢發(fā)現(xiàn)腿紋不對(duì)稱，當(dāng)時(shí)做了髖關(guān)節(jié)篩查沒有問題，現(xiàn)在三歲了，體檢腿紋還是不對(duì)稱，該怎么辦呢？其實(shí)腿紋不對(duì)稱本身并不是什么病，大部分的腿紋或臀紋不對(duì)稱是生理性的，即使一直不對(duì)稱也不會(huì)有特別影響。但是有一部分腿紋不對(duì)稱的孩子可能合并髖關(guān)節(jié)發(fā)育的問題，因此建議做髖關(guān)節(jié)篩查，如果髖關(guān)節(jié)篩查沒有問題，那么腿紋不對(duì)稱就不用太關(guān)注了。所以這位媽媽現(xiàn)在不用再擔(dān)心腿紋不對(duì)稱的問題了。2023年05月08日99
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陶可主治醫(yī)師 北京大學(xué)人民醫(yī)院 骨關(guān)節(jié)科 我都20多歲了（30多、40多、50多，甚至60多歲），怎么還會(huì)得髖臼發(fā)育不良(先髖，DDH）？怎么到現(xiàn)在才出現(xiàn)發(fā)育不良？陶可(北京大學(xué)人民醫(yī)院骨關(guān)節(jié)科)在門診就診過程中，經(jīng)常會(huì)有一些朋友會(huì)因?yàn)轶y關(guān)節(jié)周圍，腹股溝區(qū)/股骨大粗隆/臀后區(qū)酸脹、疼痛而行髖關(guān)節(jié)正側(cè)位X線片檢查，當(dāng)認(rèn)真閱片和查體后，告訴很大可能是由于髖關(guān)節(jié)發(fā)育不良導(dǎo)致的髖關(guān)節(jié)周圍疼痛，往往大家會(huì)很驚訝：我都這么大年齡了（20多、30多、40多、50多，甚至60多歲），怎么還會(huì)得髖臼發(fā)育不良(先髖，DDH）？怎么到現(xiàn)在才出現(xiàn)發(fā)育不良？還有一部分朋友可能已經(jīng)就診過，在網(wǎng)上也查閱了一些髖臼發(fā)育不良的資料，但對(duì)自己的病情還不是很確信，可能會(huì)問到：我的髖臼發(fā)育不良嚴(yán)重嗎？如何治療？需要手術(shù)嗎？帶著這些問題，今天就和大家聊一聊髖臼發(fā)育不良的病因和相關(guān)的基礎(chǔ)解剖知識(shí)。首先，為大家科普一下骨盆和髖關(guān)節(jié)的骨骼組成，如下圖：骨盆和雙髖關(guān)節(jié)骨骼結(jié)構(gòu)模式圖pelvis＆hipanatomy骨盆和髖關(guān)節(jié)骨骼解剖組成：femur股骨，pubis恥骨，ischium坐骨，ilium髂骨，sacrum骶骨，coccyx尾骨其次，大家可以看看髖關(guān)節(jié)周圍重要的肌群、血管神經(jīng)：右側(cè)髖關(guān)節(jié)-前方-淺層重要肌群及血管神經(jīng)右側(cè)髖關(guān)節(jié)-前方-深層重要肌群及血管神經(jīng)右側(cè)髖關(guān)節(jié)-后方-重要肌群及血管神經(jīng)影像學(xué)方面，如果在沒有外傷的情況下，骨科醫(yī)生首先可能會(huì)讓您進(jìn)行髖關(guān)節(jié)正側(cè)位X線檢查，當(dāng)X線片無明顯異常，而醫(yī)生又高度懷疑是髖關(guān)節(jié)相關(guān)問題造成的您的髖關(guān)節(jié)周圍的疼痛不適，或當(dāng)不能完全確定髖關(guān)節(jié)病變時(shí)，您的醫(yī)生會(huì)告訴您需要加做：髖關(guān)節(jié)B超、CT或核磁共振MRI等檢查，甚至有些時(shí)候?yàn)榱伺懦恍┎怀Ｒ姷膹?fù)雜疾病(如血液病，痛風(fēng)，感染，急性滑膜炎），您的醫(yī)生可能會(huì)給您開抽血化驗(yàn)申請(qǐng)單等。正常-成年人雙側(cè)髖關(guān)節(jié)正位X線片典型-雙側(cè)髖關(guān)節(jié)發(fā)育不良的X線片大家了解了前面的解剖與影像學(xué)檢查基礎(chǔ)知識(shí)，下面我們系統(tǒng)、深入聊聊第一個(gè)問題：我都這么大年齡了（20多、30多、40多、50多，甚至60多歲），怎么還會(huì)得髖臼發(fā)育不良？怎么到現(xiàn)在才出現(xiàn)發(fā)育不良？成人發(fā)育性髖關(guān)節(jié)發(fā)育不良和髖關(guān)節(jié)脫位是由于嬰幼兒期的髖關(guān)節(jié)遺傳性發(fā)育不良、發(fā)育性髖關(guān)節(jié)發(fā)育不良的相關(guān)髖部病變遷延至成年而來。常見的髖臼發(fā)育不良(先髖，DDH）的高危因素有：父母親或近親屬患有先髖，第一胎，女寶寶，臀位產(chǎn)，不恰當(dāng)?shù)谋юB(yǎng)方式，如北方秋冬季節(jié)為了保暖，把嬰幼兒尤其是剛出生的寶寶的雙下肢和雙髖關(guān)節(jié)嚴(yán)密地包裹起來，俗稱“蠟燭抱”，殊不知此種不恰當(dāng)?shù)倪^分包裹保暖方式，會(huì)讓寶寶的雙髖關(guān)節(jié)極度內(nèi)收，增加髖關(guān)節(jié)囊和股骨近段壓力，可能導(dǎo)致股骨頭從未發(fā)育成熟的髖臼內(nèi)“脫出”風(fēng)險(xiǎn)增大。嬰幼兒時(shí)期寶寶抱養(yǎng)時(shí)，正確與錯(cuò)誤的腿部姿勢(shì)在就診治療之前，有些朋友并未出現(xiàn)明顯髖部周圍酸脹、疼痛不適、跛行、髖關(guān)節(jié)活動(dòng)受限等癥狀，可能的主要原因是：髖臼發(fā)育不良畸形較輕，體重偏輕(瘦小身材），平時(shí)體育鍛煉減少，伏案坐位工作為主，體力活動(dòng)量不大。上述幾種情況的朋友，日常并無臨床不適癥狀，因而在嬰幼兒，青少年等各時(shí)期被忽略，成年后拍片時(shí)，偶然發(fā)現(xiàn)或出現(xiàn)髖關(guān)節(jié)骨關(guān)節(jié)炎(疼痛）表現(xiàn)就診時(shí)，而被發(fā)現(xiàn)。當(dāng)然，還有一部分朋友可能是在嬰幼兒/兒童時(shí)期，有過髖部外傷、不正規(guī)的醫(yī)治經(jīng)歷等，造成了髖關(guān)節(jié)脫位/半脫位/骨質(zhì)結(jié)構(gòu)發(fā)育異常，久而久之身體適應(yīng)了髖關(guān)節(jié)畸形的狀態(tài)。所以，除了可能在身體外觀、行走步態(tài)有異常外，并無髖部疼痛不適癥狀。但是，這部分患者往往會(huì)因腰椎/整個(gè)脊柱代償髖部畸形，而過早地出現(xiàn)腰椎間盤突出、脊柱側(cè)彎等腰背痛癥狀。在掌握了上面的知識(shí)后，我們馬上來學(xué)習(xí)一下第二個(gè)問題：我的髖臼發(fā)育不良嚴(yán)重嗎？如何治療？需要手術(shù)嗎？1.髖臼發(fā)育不良(先髖，DDH）嚴(yán)重程度分型：(1)第一種分型，Crowe分型，早在1979年，美國學(xué)者Crowe就發(fā)表了文章對(duì)髖臼發(fā)育不良(先髖，DDH）嚴(yán)重程度進(jìn)行分型，以便于規(guī)范先髖的治療和同行之間的交流。髖臼發(fā)育不良Crowe分型X線片顯示用于定義Crowe分類的標(biāo)準(zhǔn)。距離A是骨盆的總高度（坐骨結(jié)節(jié)和髂嵴之間的垂直距離）。距離B是在股骨頭與股骨頸的交界處和髖臼淚滴之間距離。Crowe根據(jù)以百分比(B/A)表示的股骨頭近端移位對(duì)髖關(guān)節(jié)發(fā)育不良的嚴(yán)重程度進(jìn)行分類。Crowe1型=B/A＜0.1或股骨頭脫位＜50%，CroweII型=B/A0.10-0.15或股骨頭脫位50-75%，CroweIII型=0.15-0.20或股骨頭脫位＞75%，CroweIV型=B/A＞0.20或股骨頭脫位＞100%.(2)第二種分型，Hartofilakidis分型，隨后，1996年，希臘學(xué)者Hartofilakidis提出另一套髖臼發(fā)育不良分型標(biāo)準(zhǔn)：髖臼發(fā)育不良Hartofilakidis分型I級(jí)是發(fā)育不良，其中股骨頭包含在原來的真髖臼內(nèi)。2級(jí)是低位脫位，股骨頭與假髖關(guān)節(jié)相連，假髖臼的下緣與真髖臼的上緣接觸或重疊，呈現(xiàn)兩個(gè)重疊的髖臼外觀。3級(jí)是高位脫位，股骨頭向后方移位，真假髖臼無接觸。大家也可以搜索閱讀：Hartofilakidis髖臼發(fā)育不良分型原文：髖臼缺損的分類及髖臼成形術(shù)聯(lián)合全髖關(guān)節(jié)置換術(shù)https://station.haodf.com/health/article?healthId=8618339876＆articleId=9391880836分型標(biāo)準(zhǔn)是指導(dǎo)治療方案的關(guān)鍵。上述兩種方法是臨床骨科最常用的髖臼發(fā)育不良(先髖，DDH)分型標(biāo)準(zhǔn)，大家可以對(duì)照自己的雙髖關(guān)節(jié)正位X片，不難得出結(jié)果。當(dāng)然面診時(shí)，有經(jīng)驗(yàn)的保髖專業(yè)醫(yī)生，也會(huì)告訴您具體分型結(jié)論。2.萬一很不幸，您真的被醫(yī)生確診了髖臼發(fā)育不良(先髖，DDH)，那么如何治療呢？需要手術(shù)嗎？其實(shí)，您大可不必驚慌失措，因?yàn)轶y臼發(fā)育不良(先髖，DDH)比較常見，人群發(fā)病率0.6‰-0.2%，是一種可防、可控、可治的疾病。具體理解是：(1)可防：防止或盡可能避免出現(xiàn)髖周疼痛，這是我們治療的核心內(nèi)容之一。(2)可控：控制病情快速進(jìn)展，避免過早發(fā)生髖關(guān)節(jié)過分磨損、破壞，以至于進(jìn)展成為髖骨關(guān)節(jié)炎。(3)可治：髖臼發(fā)育不良(先髖，DDH)有多種治療方法，包括保守治療和手術(shù)治療兩大類。具體治療方法和建議如下：(1)保守療法，健康教育：限制劇烈運(yùn)動(dòng)，避免蹦跳、深蹲、扎馬步、瑜伽劈腿、舞蹈一字胯、反復(fù)上下樓梯/爬山、受涼、勞累，長時(shí)間站立、每天站立行走/步數(shù)、蹺二郎腿等可能造成髖關(guān)節(jié)磨損加快，軟骨/盂唇損傷，髖關(guān)節(jié)積液的活動(dòng)方式。(2)髖關(guān)節(jié)周圍核心肌群、韌帶鍛煉：大家可以在好大夫在線上搜索：髖關(guān)節(jié)核心肌群鍛煉小視頻，或者臀中肌力量訓(xùn)練：7個(gè)小視頻；經(jīng)常做做熱水袋熱敷。(3)控制體重：體重指數(shù)(體重kg/身高m/身高m)在24以內(nèi)。(4)藥物治療：口服消炎止疼藥(如西樂葆膠囊或美洛昔康片等，用藥前一定提前咨詢您的主診醫(yī)生)，邁之靈片(有助于消除靜脈炎/靜脈水腫/滑膜炎/關(guān)節(jié)積液)，乙哌立松片(緩解神經(jīng)肌肉緊張/肌痙攣/神經(jīng)肌肉接頭水腫)，對(duì)于病程比較久，日?；顒?dòng)受限明顯的朋友，您還可能需要預(yù)防骨質(zhì)疏松藥物等。對(duì)于初次就診，剛確診髖臼發(fā)育不良(先髖，DDH)的朋友，您可能尚處于疾病早期，可以考慮上面的保守治療方案1-4周；依據(jù)治療效果，決定進(jìn)一步治療方案。(5)微創(chuàng)關(guān)節(jié)鏡手術(shù)：可以探查髖關(guān)節(jié)軟骨/盂唇等損傷嚴(yán)重程度，也可以配合保髖截骨手術(shù)治療，同期進(jìn)行。(6)保髖截骨手術(shù)：主要有PAO截骨、ITO經(jīng)轉(zhuǎn)子間截骨等。對(duì)于髖臼發(fā)育不良病情較重/影像學(xué)檢查典型、已經(jīng)排除了腰椎/骶椎病變?cè)斐裳甸g盤突出癥/坐骨神經(jīng)痛/梨狀肌綜合征/腰骶筋膜炎/髖關(guān)節(jié)撞擊綜合征/髂腰肌炎癥/單純髖關(guān)節(jié)盂唇損傷/游離體等疾病、且保守治療效果不佳時(shí)，可以考慮選擇您最信任的醫(yī)生進(jìn)行保髖截骨手術(shù)治療。(7)對(duì)于髖關(guān)節(jié)發(fā)育不良繼發(fā)重度骨關(guān)節(jié)炎，持續(xù)性髖關(guān)節(jié)疼痛，活動(dòng)受限的朋友，需考慮進(jìn)行全髖關(guān)節(jié)置換術(shù)THA。(8)對(duì)于一些病程時(shí)間長，髖臼發(fā)育不良病情中等嚴(yán)重程度，髖關(guān)節(jié)軟骨損傷可能比較明顯，年齡偏大(50歲以上)，畏懼或不愿意接受手術(shù)治療的朋友，髖關(guān)節(jié)腔內(nèi)注射藥物(超聲引導(dǎo)下的髖關(guān)節(jié)注射-北京大學(xué)人民醫(yī)院多學(xué)科診治經(jīng)驗(yàn)，或者關(guān)于髖、膝、踝、肩等關(guān)節(jié)潤滑營養(yǎng)封閉)也是一種不錯(cuò)的選擇。髖臼發(fā)育不良患者在PAO截骨術(shù)前，術(shù)后的X線片雙側(cè)髖關(guān)節(jié)發(fā)育不良進(jìn)行了全髖關(guān)節(jié)置換手術(shù)治療雙側(cè)髖關(guān)節(jié)發(fā)育不良進(jìn)行了全髖關(guān)節(jié)置換手術(shù)治療(9)最后，溫馨提醒：患有髖臼發(fā)育不良(先髖，DDH)不一定都有癥狀，臨床經(jīng)驗(yàn)告訴我們約5%的輕度/臨界髖臼發(fā)育不良(先髖，DDH)患者，是可以沒有髖部疼痛不適癥狀的，往往是在拍片時(shí)才能發(fā)現(xiàn)。這部分朋友是不需要治療，只需要密切隨訪觀察即可。另外，不是所有患者都需要全髖關(guān)節(jié)置換手術(shù)治療。目前，國內(nèi)外主流的學(xué)者觀點(diǎn)認(rèn)為，保髖截骨手術(shù)是糾正青壯年髖臼發(fā)育不良(先髖，DDH)髖關(guān)節(jié)骨骼結(jié)構(gòu)畸形，顯著改善髖關(guān)節(jié)疼痛不適、活動(dòng)受限、跛行等最好的治療方式。雙側(cè)髖關(guān)節(jié)發(fā)育不良-輕度-X線片，患者因雙膝關(guān)節(jié)骨關(guān)節(jié)炎住院擬行膝關(guān)節(jié)置換手術(shù)治療前，行雙下肢全長正位X線片檢查時(shí)發(fā)現(xiàn)髖臼發(fā)育不良(先髖，DDH)，但查體和病史詢問，患者無髖部疼痛癥狀，故不做任何治療相信通過今天的科普閱讀，您一定對(duì)髖臼發(fā)育不良(先髖，DDH)的診斷和治療有一個(gè)系統(tǒng)而全面的了解。歡迎大家閱讀、轉(zhuǎn)發(fā)。也希望能幫助到求醫(yī)問藥中的每位朋友。2022年12月07日1297
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陶可主治醫(yī)師 北京大學(xué)人民醫(yī)院 骨關(guān)節(jié)科 青少年和成年人的髖關(guān)節(jié)發(fā)育不良(DDH)髖關(guān)節(jié)發(fā)育異常：診斷和治療方案陶可（北京大學(xué)人民醫(yī)院骨關(guān)節(jié)科）左側(cè)正常髖關(guān)節(jié)的解剖（髖臼包容覆蓋股骨頭達(dá)75-95%，髖臼與股骨頭形合度優(yōu)良，且兩者在同一圓心軌跡活動(dòng)）；右側(cè)明顯髖關(guān)節(jié)發(fā)育不良、合并股骨頭完全脫位。Figure1.Basicanatomyofthehipjoint,AnatomyofaNormalHip.圖2正常右側(cè)髖關(guān)節(jié)外側(cè)視圖（左圖）；髖臼（acetabulum）與股骨頭（femoralhead）組成髖關(guān)節(jié)，盂唇（acetabulumlabrum）、關(guān)節(jié)軟骨（chondralsurfacesoffemoralheadandacetabulum）（右圖）充填于骨骼表面及髖臼邊緣，起到減少摩擦、緩沖跑跳時(shí)的機(jī)械應(yīng)力刺激、并加深髖關(guān)節(jié)窩，以髖臼更好地包裹、覆蓋股骨頭，從而避免髖關(guān)節(jié)脫位。圖3髖臼發(fā)育不良患者術(shù)前正位X線片顯示：雙側(cè)均存在病變，主要表現(xiàn)為髖臼對(duì)股骨頭的包容覆蓋減少，臨床癥狀主要為站立、下蹲、體育鍛煉時(shí)的髖關(guān)節(jié)周圍的酸脹、疼痛（腹股溝區(qū)、大轉(zhuǎn)子附近、臀后區(qū)），甚至部分患者出現(xiàn)行走步態(tài)異常：跛行，從而嚴(yán)重影響患者的日?；顒?dòng)（左圖）；通過髖臼截骨術(shù)（PAO手術(shù)）（右圖）矯正髖關(guān)節(jié)發(fā)育不良的年輕患者髖部區(qū)域的X線圖像。請(qǐng)注意螺釘（以白色顯示），術(shù)中采用螺釘將截骨塊固定于調(diào)整后的髖臼相對(duì)股骨頭包容覆蓋的位置與角度。X-rayimagesofthehipregionofayoungpatientwithhipdysplasia(left)correctedwithPAOsurgery(right).Notethescrews(appearinginwhite),whicharesetinplacetorealigntheacetabuluminamorenormalpositionoverthehead(top)ofthefemur(thighbone).視頻1髖臼截骨術(shù)（PAO）模擬動(dòng)畫介紹雖然慢性髖關(guān)節(jié)疼痛通常與衰老有關(guān)，但青少年和年輕人出現(xiàn)這種癥狀可能是髖關(guān)節(jié)發(fā)育不良的征兆，即髖關(guān)節(jié)的一個(gè)或多個(gè)區(qū)域發(fā)育不正常。在健康的髖關(guān)節(jié)中，股骨（大腿骨）的上端與髖臼相接，像圓球/碗蓋一樣配合在一起，球在髖臼中自由旋轉(zhuǎn)。髖關(guān)節(jié)軟骨是一種光滑的保護(hù)組織，排列在骨骼表面，在運(yùn)動(dòng)過程中限制骨骼表面之間的接觸與摩擦。另一塊稱為髖臼盂唇的軟組織，是由纖維軟骨制成，位于髖關(guān)節(jié)窩邊緣，緩沖關(guān)節(jié)摩擦，并有助于將“圓球”（股骨頭）固定到髖臼窩內(nèi)。在患有髖關(guān)節(jié)發(fā)育不良的患者中，髖臼沒有完全發(fā)育，使其太淺而無法充分容納和支撐股骨頭。當(dāng)出現(xiàn)這種異常時(shí)，髖臼球窩就會(huì)錯(cuò)位，盂唇最終會(huì)承受本應(yīng)分布在整個(gè)髖關(guān)節(jié)的應(yīng)力（很容易在扭傷、屈髖、盤腿、極度一字劈叉、蹺二郎腿等扭轉(zhuǎn)髖關(guān)節(jié)時(shí)，出現(xiàn)盂唇及軟骨損傷，進(jìn)而出現(xiàn)髖明顯疼痛）。此外，更多的應(yīng)力被施加在髖關(guān)節(jié)軟骨和骨骼的較小表面上（應(yīng)力集中），導(dǎo)致數(shù)年就會(huì)出現(xiàn)髖骨關(guān)節(jié)炎的表現(xiàn)（如X線片上可見：髖臼及股骨頭局部硬化、囊性變、骨贅形成等）。盂唇和有助于將關(guān)節(jié)固定到位的髖關(guān)節(jié)囊韌帶受傷會(huì)增加軟骨的疼痛和磨損。髖關(guān)節(jié)發(fā)育不良的嚴(yán)重程度可能有很大差異，從輕微錯(cuò)位到股骨頭和髖臼完全脫位。如果不及時(shí)治療，髖關(guān)節(jié)發(fā)育不良會(huì)導(dǎo)致早期退行性變化——髖骨關(guān)節(jié)炎的開始，其中軟骨磨損，髖關(guān)節(jié)骨頭與骨頭摩擦，疼痛明顯。在晚期病例中，患者可能過早地需要進(jìn)行髖關(guān)節(jié)置換術(shù)。髖臼異常發(fā)育的原因和發(fā)病率髖臼異常發(fā)育的原因尚不清楚，胎兒在子宮中的位置、臀位分娩和發(fā)育不良家族史之間似乎存在關(guān)聯(lián)。髖關(guān)節(jié)發(fā)育不良（也稱為小兒髖關(guān)節(jié)發(fā)育不良或發(fā)育性髖關(guān)節(jié)發(fā)育不良）篩查是美國新生兒的常規(guī)護(hù)理，但不可能檢測(cè)出新生兒期最終發(fā)育不良的所有病例。其中一些異常相對(duì)較輕，可能無法及早發(fā)現(xiàn)或直到個(gè)體進(jìn)入青春期或更晚才引起癥狀。根據(jù)10年期間在挪威髖關(guān)節(jié)置換登記處收集的數(shù)據(jù)，髖關(guān)節(jié)置換術(shù)全部患者中，有高達(dá)26%的患者是未經(jīng)治療的髖關(guān)節(jié)發(fā)育不良。一些專家認(rèn)為這個(gè)數(shù)字更高。雖然髖關(guān)節(jié)發(fā)育不良最常發(fā)生在身體的左側(cè)，但一個(gè)或兩個(gè)髖關(guān)節(jié)都可能受到影響。最近表明，畸形在40%的患者中是雙側(cè)的（發(fā)生在兩側(cè)），兩側(cè)的嚴(yán)重程度各不相同。髖關(guān)節(jié)發(fā)育不良在女性中比在男性中更常見，這種現(xiàn)象可能是由于女性骨盆的解剖學(xué)差異以及女性韌帶更松弛（松弛）的趨勢(shì)所致（女性發(fā)病人數(shù)10-12：男性發(fā)病人數(shù)1）。髖關(guān)節(jié)發(fā)育不良可見于廣泛的年齡范圍，但青少年往往有更嚴(yán)重的發(fā)育不良，因?yàn)轶y關(guān)節(jié)周圍的軟組織更早失效。髖臼發(fā)育不良的診斷除了全面的身體檢查和患者病史外，骨科醫(yī)生還使用X線、磁共振成像(MRI)以及在某些情況下使用3-DCT圖像來確認(rèn)髖關(guān)節(jié)發(fā)育不良的診斷與嚴(yán)重程度，并未手術(shù)設(shè)計(jì)提供影像學(xué)依據(jù)。HSS提供的高度復(fù)雜的MRI技術(shù)特別有助于提供清晰區(qū)分骨骼和軟骨的圖像，并有助于查明盂唇撕裂的范圍（如果存在）。很多情況下，這些圖像上可見的損傷與患者的癥狀不符；例如，患者的軟骨可能有相當(dāng)大的損傷，但疼痛卻很小。并非所有盂唇撕裂都是髖關(guān)節(jié)發(fā)育不良的結(jié)果。髖關(guān)節(jié)盂唇撕裂可能繼發(fā)于髖關(guān)節(jié)疼痛的其他原因，例如股骨髖臼撞擊。髖臼發(fā)育不良的非手術(shù)治療髖關(guān)節(jié)發(fā)育不良的治療重點(diǎn)是盡可能長時(shí)間地保留髖關(guān)節(jié)，這是及時(shí)評(píng)估髖關(guān)節(jié)疼痛的原因之一。骨科醫(yī)生越早發(fā)現(xiàn)問題，就有越多的治療選擇。非手術(shù)治療的初始方案可能適用于發(fā)育非常輕度的年輕人、髖關(guān)節(jié)發(fā)育不良導(dǎo)致關(guān)節(jié)嚴(yán)重?fù)p傷或只能接受髖關(guān)節(jié)置換術(shù)的年輕人。對(duì)于癥狀輕微和發(fā)育不良的患者，骨科醫(yī)生將密切監(jiān)測(cè)病情，以發(fā)現(xiàn)任何可能需要治療的進(jìn)展。對(duì)于上述患者，消炎止疼藥、激素注射、特殊物理治療(如熱敷髖部)和使用手杖、減少或避免站立行走，都可能有助于緩解癥狀。髖臼發(fā)育不良的手術(shù)治療經(jīng)歷疼痛且軟骨損傷有限的髖關(guān)節(jié)發(fā)育不良患者可能適合進(jìn)行髖臼周圍截骨術(shù)(PAO)。該手術(shù)涉及對(duì)髖臼周圍骨骼進(jìn)行一系列切割，以重新定位、并使用螺釘固定完美的角度，以恢復(fù)更正常的解剖結(jié)構(gòu)。在多達(dá)10%的患者中，骨外科醫(yī)生也可能會(huì)進(jìn)行股骨截骨術(shù)（上下聯(lián)合截骨術(shù)）。髖關(guān)節(jié)鏡檢查也可以在選定的患者中與髖臼截骨術(shù)（PAO）一起進(jìn)行（例如，修復(fù)盂唇、軟骨損傷嚴(yán)重程度）。為了符合髖臼截骨術(shù)（PAO）的條件，患者必須已經(jīng)達(dá)到或接近達(dá)到骨骼成熟度；年齡范圍的低端約為11歲（但年齡不是絕對(duì)影響因素，北京大學(xué)人民醫(yī)院做過8歲孩子的保髖上下聯(lián)合截骨術(shù)）。雖然年輕患者的并發(fā)癥發(fā)生率往往最低且恢復(fù)時(shí)間最快，但45歲以上的患者只要符合其他標(biāo)準(zhǔn)（輕微關(guān)節(jié)炎、良好的運(yùn)動(dòng)范圍和輕微癥狀）就有可以接受髖臼截骨術(shù)（PAO）（同樣，年齡不是絕對(duì)影響因素，北京大學(xué)人民醫(yī)院做過54歲患者的保髖上截骨術(shù)）。在美國HSS（同樣地，北京大學(xué)人民醫(yī)院），接受上截髖臼截骨術(shù)（PAO）的患者需要住院5到7天，然后可轉(zhuǎn)至康復(fù)中心或部分患者可回家康復(fù)鍛煉。出院后，患者需要使用雙拐杖4到6周。然后6周時(shí)復(fù)查拍片，進(jìn)一步制定康復(fù)鍛煉方案。絕大多數(shù)患者會(huì)在3到6個(gè)月后恢復(fù)到像術(shù)前一樣的正?；顒?dòng)，而擺脫了髖關(guān)節(jié)疼痛。對(duì)于雙髖發(fā)育不良的患者，手術(shù)間隔4到6個(gè)月。在嚴(yán)重關(guān)節(jié)炎之前進(jìn)行髖臼截骨術(shù)（PAO）的患者即使在手術(shù)后長達(dá)20-25年也有非常好的結(jié)果?！坝山?jīng)驗(yàn)豐富的整形外科醫(yī)生執(zhí)行，髖臼截骨術(shù)（PAO）具有良好的安全性并產(chǎn)生可預(yù)測(cè)的結(jié)果，”“如果您正在考慮接受髖臼截骨術(shù)（PAO），重要的是請(qǐng)去一個(gè)已經(jīng)可以完成上百例此類手術(shù)的醫(yī)學(xué)中心治療?！盚ipDysplasia(DDH)inAdolescentsandYoungAdultsAbnormaldevelopmentinthehipjoint:diagnosisandtreatmentoptionsIntroductionAlthoughchronichippainisoftenassociatedwithaging,theappearanceofthissymptominadolescentsandyoungadultsmaybeasignofhipdysplasia,aconditioninwhichoneormoreareasofthehipjointhavenotdevelopednormally.Inthehealthyhipjoint,theupperendofthefemur(thighbone)meetstheacetabulumtofittogetherlikeaballandsocket/cup,inwhichtheballrotatesfreelyinthehipsocket.Articularcartilage,asmoothprotectivetissue,linesthebonesandlimitsfrictionbetweenthebonesurfacesduringmovement.Anotherpieceofsofttissuecalledthelabrum–whichismadeoffibrouscartilage–linesthehipsocket,cushionsthejoint,andhelpsholdthe“ball”–(thefemoralhead)inplace.Inindividualswithhipdysplasia,theacetabulumdoesnotdevelopfully,makingittooshallowtoadequatelycontainandsupportthefemoralhead.Whenthisabnormalityispresent,theballandsocketaremisalignedandthelabrumcanendupbearingtheforcesthatshouldbedistributedthroughoutthehip.Also,moreforceisplacedonasmallersurfaceofthehipcartilageandbone,resultinginarthritisoveranumberofyears.Injurytothelabrumandtheligamentsthathelpholdthejointinplacecanaddtothepainandwearandtearonthecartilage.Theseverityofhipdysplasiacanvaryconsiderablyfrominstancesinwhichthereisaminormalalignmenttoacompletedislocationofthefemoralheadandacetabulum.Leftuntreated,hipdysplasiacanresultinearlydegenerativechanges-thebeginningsofosteoarthritis,inwhichthecartilagewearsawayandbonerubsagainstbone.Inadvancedcases,thepatientmayrequirehipreplacement.CausesandIncidenceThecauseofabnormaldevelopmentoftheacetabulumisnotyetwellunderstood,althoughthereappearstobearelationshipbetweenthepositionofthefetusinthewomb,breechbirths,andafamilyhistoryofdysplasia.Screeningforhipdysplasia(alsocalledpediatrichipdysplasiaordevelopmentalhipdysplasia)isroutinecarefornewbornsintheUnitedStates,butitisimpossibletodetectallcasesofeventualdysplasiainthenewbornperiod.Someoftheseabnormalitiesarerelativelymildandmaynotbedetectedearlyorcausesymptomsuntiltheindividualreachesadolescenceorlater.Asignificantnumberofhipreplacementsarebelievedtobetheresultofuntreatedhipdysplasia–upto26%,accordingtodatagatheredintheNorwegianArthroplastyRegisterovera10-yearperiod.Someexpertsbelievethisfiguretobehigher.Althoughhipdysplasiaoccursmostfrequentlyontheleftsideofthebody,eitheroneorbothhipsmaybeaffected.Ithasrecentlybeenshownthatthedeformityisbilateral(occurringonbothsides)in40%ofpatients,withvaryingseverityoneitherside.Hipdysplasiaismorecommoninwomenthaninmen,aphenomenonthatmaybeduetoanatomicaldifferencesinthefemalepelvisandthetendencyforwomentohavegreaterlaxity(looseness)intheirligaments.Hipdysplasiacanbeseeninawideagerangeofpatients,butadolescentstendtohavemoreseveredysplasia,asthesofttissuessurroundingthejointfailearlier.DiagnosisInadditiontoathoroughphysicalexamandpatienthistory,orthopedistsuseX-rays,magneticresonanceimaging(MRI),andinsomecases,3-DCTimagestoconfirmadiagnosisofhipdysplasia.ThehighlysophisticatedMRItechniquesavailableatHSSareparticularlyhelpfulinprovidingimagesthatshowcleardistinctionbetweenboneandcartilageandhelpstopinpointtheextentofthelabraltearifpresent.Notinfrequently,thedamagethatisvisibleontheseimagesdoesnotcorrespondtothepatient’ssymptoms;forexample,thepatientmayhaveconsiderabledamagetothecartilage,butexperienceverylittlepain.Notalllabraltearsaretheresultofhipdysplasia.Labraltearsofthehipmaybesecondarytoothercausesofhippain,suchasfemoroacetabularimpingement.NonsurgicalTreatmentTreatmentforhipdysplasiafocusesonpreservingthehipforaslongaspossible,whichisonereasontobeevaluatedforhippainpromptly.Thesoonertheorthopedistdetectstheproblem,themoretreatmentoptionsareavailable.Aninitialtrialofnon-surgicaltreatmentmaybeappropriateforyoungadultswitheitherverymilddysplasiaorthosewhosehipdysplasiahasresultedinsignificantdamagetothejointandwhoseonlysurgicaltreatmentoptionwouldbehipreplacement.Forthosewithminimalsymptomsanddysplasia,theorthopedistwillcloselymonitortheconditiontodetectanyprogressionthatmaywarranttreatment.Forbothgroupsofpatients,anti-inflammatorymedication,steroidinjections,specificphysicaltherapy,anduseofacanemayhelprelievesymptoms.SurgicalTreatmentPatientswithhipdysplasiawhoexperiencepainandhavelimiteddamagetotheircartilagemaybecandidatesforperiacetabularosteotomy(PAO).Thisprocedureinvolvesaseriesofcutstothebonetoreorienttheacetabulumoverthefemoralhead,inordertorestoreamorenormalanatomy.Screwsarethenplacedinthebonestostabilizethisposition.Image-PAOVideoPreviewViewAnimationofPAOInupto10%ofpatientstheorthopedicsurgeonmayalsoperformafemoralosteotomy.HiparthroscopymayalsobeperformedalongwithaPAOinselectedpatients(forexample,torepairthelabrum).InordertobeeligibleforPAO,thepatientmusthavereachedorbeclosetoreachingskeletalmaturity;thelowendoftheagerangeisaboutelevenyears.Whileyoungerpatientstendtohavethelowestrateofcomplicationsandfastestrecoverytime,patientsupintotheirearly40sareeligibleforPAOprovidedtheymeetothercriteria(minimalarthritis,goodrangeofmotion,andmildsymptoms).PatientsinwhomcartilagedamageissignificantandprogressivearenotgoodcandidatesforPAO;initialnon-operativetreatmentandeventualtotalhipreplacementcanbeaneffectivealternative.Inselectedcases,thesurgeonusesarthroscopyasanadjuncttoPAO.Hiparthroscopyaloneisgenerallynotrecommendedforhipdysplasiawithalabraltear,asitcandisrupttheligamentsandmusclesthatsurroundthehip,furtherdestabilizingthejoint.AtHSS,patientswhoundergoPAOarehospitalizedforfivetosevendays.Followingdischarge,thepatientusescrutchesforfourtosixweeks.Mostreturntotheirregularactivitiesbythreetosixmonths.Inpatientswithdysplasiainbothhips,thesurgeriesareperformedfourtosixmonthsapart.PatientswithaPAOperformedbeforesignificantarthritishaveverygoodresultsevenupto20-25yearsaftertheprocedure.[1]“Performedbyanexperiencedorthopedicsurgeon,PAOhasagoodsafetyprofileandproducespredictableresults,”saysErnestL.Sink,MD,anassociateattendingorthopedicsurgeonatHSSwhofrequentlyperformstheprocedure.“IfyouareconsideringundergoingaPAO,it’simportanttogotoacenterwheremanyoftheseproceduresaredone.”https://www.hss.edu/conditions_hip-dysplasia-adolescents-young-adults.asp2022年12月04日1195
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張中禮主任醫(yī)師 天津醫(yī)院 小兒骨科 女孩，12+3歲。幼時(shí)曾因左髖疾患于當(dāng)?shù)匦小爸Ь摺北Ｊ刂委?。因“左髖部疼痛、行走跛行進(jìn)行性加重1年”就診。既往史無殊，無相關(guān)家族史。體格檢查：行走輕度跛行，左髖Trendlenburg(+)、撞擊征(+)。左髖伸髖外展減小、（俯臥位）內(nèi)旋增大。標(biāo)準(zhǔn)骨盆正位X線片（推薦站立位拍片）骨盆正位片示“左側(cè)Shenton線中斷，左側(cè)髖關(guān)節(jié)中心點(diǎn)外移、左側(cè)髖臼發(fā)育不良失去正常眉弓形態(tài)（Tonnis臼頂角減?。?、股骨頭外側(cè)覆蓋不足（coverage，Wiberg外側(cè)CE角顯著減?。瑴I滴形態(tài)異常髖臼內(nèi)壁增厚，左側(cè)小轉(zhuǎn)子稍突出可能存在前傾角較大”。外展內(nèi)旋位骨盆正位片（functionalview，功能位片）示頭臼不能獲得良好“同心圓對(duì)位”，提示頭臼存在不匹配，可能合并存在形態(tài)（臼頂變形）、容積不稱（臼大）假側(cè)位像（65°斜位像、Falseforfileview）：髖臼前緣覆蓋不良雙下肢站立位全長像：雙下肢機(jī)械軸線無明顯異常，左下肢較右側(cè)長4mm雙髖三維CT、疊加法（掃描雙股骨近端與股骨髁部即可，不掃描股骨中部以減少放射線暴露）測(cè)量雙側(cè)股骨頸前傾角：左側(cè)41°，右側(cè)25°雙側(cè)髖臼VRT重建：左髖失去正?！熬省钡男螒B(tài)，呈三角形臼頂髖關(guān)節(jié)直接造影核磁dMRA（稀釋的釓噴酸葡胺）示左髖“盂唇內(nèi)翻入關(guān)節(jié)內(nèi)”2022年10月26日730
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孫永建主任醫(yī)師 南醫(yī)三院 兒童骨科 體檢時(shí)發(fā)現(xiàn)寶寶雙側(cè)腿紋、臀紋不對(duì)稱醫(yī)生建議完善髖關(guān)節(jié)超聲檢查那么，醫(yī)生到底在擔(dān)心什么？髖關(guān)節(jié)發(fā)育不良，了解一下？01什么是髖關(guān)節(jié)發(fā)育不良？發(fā)育性髖關(guān)節(jié)脫位又稱髖關(guān)節(jié)發(fā)育不良（DDH），是兒童常見的骨科疾病，是指孩子的股骨頭或髖白在大小、形狀、方向及組織學(xué)上的異常，或兩者皆有。在出生前后不能夠正常的發(fā)育。髖白發(fā)育不良是以髖白發(fā)育不成熟、變淺為特征，它可以導(dǎo)致股骨頭半脫位或完全脫位。02寶寶得了這個(gè)病，后果嚴(yán)重嗎？如果寶寶得了這個(gè)病，家長不用擔(dān)心，及早到專業(yè)的兒童骨科就診，就可以得到完美的治療，而且越早治療效果越好。如果沒有及時(shí)治療就會(huì)影響孩子以后的生活，可能會(huì)給孩子造成終生的殘疾。03家長如何早期發(fā)現(xiàn)寶寶髖部有問題？（1）觀察雙下肢是否等長：將寶寶平放在床上，觀察寶寶雙腿是否等長。（2）觀察兩腿伸展程度是否不一樣：將寶寶屈膝，并向外展開，正常寶寶的膝蓋外側(cè)可以觸及床面，如果不能，就需要到醫(yī)院骨科做進(jìn)-步檢查。（3）觀察走路是否跛行：普通寶寶在一周歲左右時(shí)會(huì)獨(dú)立行走，如發(fā)現(xiàn)有些寶寶到一歲半還不會(huì)走路，或者勉強(qiáng)行走，步態(tài)不穩(wěn)或姿勢(shì)異常，像只小鴨子，容易摔跤。以上情況需提防DDH可能。（4）觀察臀紋是否不對(duì)稱：臀部、大腿內(nèi)側(cè)皮膚褶皺增多、加深或不對(duì)稱，可能是發(fā)育性髖關(guān)節(jié)脫位，應(yīng)該立即到醫(yī)院行B超、X線或MRI等檢查，以明確診斷，早期進(jìn)行治療。2022年09月18日203
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王煒昌主任醫(yī)師 信宜市人民醫(yī)院 骨科、燒傷整形外科 案例痛心，3歲才發(fā)現(xiàn)，需多處截骨手術(shù)。???來自信宜市丁堡鎮(zhèn)的小姑娘，3歲多就診才發(fā)現(xiàn)患者雙側(cè)DDH，圖為右側(cè)進(jìn)行三聯(lián)截骨手術(shù)治療后，左側(cè)需擇期再手術(shù)治療。DDH科普小知識(shí)一、越早治療效果越好，應(yīng)早至滿月嬰兒。二、①看臀紋，②蛙式試驗(yàn)，③下肢短縮試驗(yàn)。①看臀紋（要對(duì)稱），指大腿與臀部鏈接的紋理，不對(duì)稱要就診，請(qǐng)注意不對(duì)稱并非意味著一定是患有DDH。②蛙式試驗(yàn)（像小青蛙一樣做動(dòng)作），寶寶雙側(cè)髖、膝關(guān)節(jié)屈曲90°后外展，正常新生兒及嬰兒可外展80°左右。（圖示右側(cè)為患側(cè)，圖片源自網(wǎng)絡(luò)）③下肢短縮試驗(yàn)，寶寶雙側(cè)髖、膝關(guān)節(jié)屈曲，足跟平放于床面上，正常兩側(cè)膝頂點(diǎn)等高。（圖示右側(cè)為患側(cè)，圖片源自網(wǎng)絡(luò)）三、科學(xué)就醫(yī)：6月齡內(nèi)查B超，半歲后查正蛙位髖關(guān)節(jié)X線片。1．小于6月齡的寶寶因髖臼和股骨頭主要為軟骨，x線平片不能顯示，超聲可做形態(tài)學(xué)評(píng)估，常用Graf法篩查髖關(guān)節(jié)形態(tài)異常。髖關(guān)節(jié)發(fā)育不良（DDH）X線檢查病情程度分類（圖片源自網(wǎng)絡(luò)）2．髖關(guān)節(jié)發(fā)育不良（DDH）股骨頭近端骨化中心出現(xiàn)（4~6月齡），即可拍前后位片、髖外展位和內(nèi)旋位片，常用骨盆正位及蛙位X線片評(píng)估。2022年09月06日166
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