朱杰 趙黎
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發(fā)育性髖關(guān)節(jié)發(fā)育不良的超聲篩查:研究進(jìn)展與目前問(wèn)題
朱杰趙黎
發(fā)育性髖關(guān)節(jié)發(fā)育不良(DDH)是引起幼兒及青少年殘疾的主要髖關(guān)節(jié)疾病之一。為了避免其可能導(dǎo)致的嚴(yán)重后果,多個(gè)國(guó)家均已建立DDH早期臨床篩查體系。Graf法已被證實(shí)為臨床可行、結(jié)論準(zhǔn)確的DDH超聲篩查方法之一,已在多個(gè)國(guó)家及地區(qū)使用,可實(shí)現(xiàn)早發(fā)現(xiàn)、早診斷、早治療的目的。該法將DDH分為4型,GrafⅠ型髖關(guān)節(jié)目前無(wú)確切證據(jù)支持會(huì)發(fā)生退變,大部分GrafⅡa型髖關(guān)節(jié)可自發(fā)骨化成熟,而對(duì)骨化延遲及脫位的髖關(guān)節(jié)則應(yīng)及時(shí)采取相應(yīng)治療措施。該文對(duì)目前嬰幼兒DDH超聲篩查研究進(jìn)展作一綜述。
發(fā)育性髖關(guān)節(jié)發(fā)育不良;髖關(guān)節(jié)脫位;超聲篩查
發(fā)育性髖關(guān)節(jié)發(fā)育不良(DDH)是一系列疾病的統(tǒng)稱(chēng),包括髖臼發(fā)育不良、髖關(guān)節(jié)半脫位和髖關(guān)節(jié)全脫位等。DDH在不同年齡段可有不同臨床表現(xiàn),如新生兒DDH表現(xiàn)為髖關(guān)節(jié)不穩(wěn)定,行體檢時(shí)股骨頭既可完全脫出髖臼,也可復(fù)位至髖臼內(nèi),而幼兒及青少年DDH則可表現(xiàn)為髖臼發(fā)育不良,對(duì)股骨頭覆蓋較差,或髖關(guān)節(jié)全脫位。DDH舊稱(chēng)先天性髖關(guān)節(jié)脫位(CDH)[1],但并非所有患者出生時(shí)即存在髖關(guān)節(jié)脫位,命名為DDH可更準(zhǔn)確揭示這一疾病發(fā)育性的特征,即病理改變可隨患者生長(zhǎng)發(fā)生變化。
若DDH得不到早期診斷和治療,晚期可能會(huì)引起跛行、髖關(guān)節(jié)骨關(guān)節(jié)炎等后遺癥,嚴(yán)重影響患者生活質(zhì)量[2-3],且治療時(shí)患者年齡越大預(yù)后越不理想[4]。因此,多個(gè)國(guó)家與地區(qū)均已開(kāi)展DDH早期篩查工作[5]。DDH診斷以影像學(xué)檢查為準(zhǔn),對(duì)于年齡較小、股骨頭骨化核尚未出現(xiàn)的嬰兒,髖關(guān)節(jié)超聲檢查相比其他影像學(xué)檢查更能直觀、清晰地反映髖關(guān)節(jié)內(nèi)部結(jié)構(gòu),為首選檢查方法。
1.1靜態(tài)超聲檢查法
1.1.1Graf法
Graf[6]于1976年起采用標(biāo)準(zhǔn)化超聲檢查技術(shù)進(jìn)行髖關(guān)節(jié)形態(tài)學(xué)檢查,確立了位于髖臼中部冠狀面上的標(biāo)準(zhǔn)平面及一系列解剖標(biāo)志。操作者可根據(jù)該標(biāo)準(zhǔn)平面選取解剖標(biāo)志并進(jìn)行測(cè)量。根據(jù)測(cè)量基線(xiàn)、骨頂線(xiàn)及軟骨頂線(xiàn)構(gòu)成的骨頂角(α角)及軟骨頂角(β角),可將髖關(guān)節(jié)形態(tài)分為4型,通過(guò)該分型可進(jìn)一步判斷髖關(guān)節(jié)發(fā)育情況以及是否存在髖關(guān)節(jié)發(fā)育不良。不同地區(qū)采用Graf法檢查出的DDH發(fā)病率為0.2%~5.1%不等[7-10]。多篇文獻(xiàn)[11-13]報(bào)道,Graf法無(wú)論在圖像的采集、解讀、測(cè)量,觀察者間的差異,還是方法本身的可重復(fù)性,均較其他影像學(xué)檢查方法有明顯優(yōu)勢(shì)。Graf法是一種定義清晰、客觀且可重復(fù)性高的髖關(guān)節(jié)超聲檢查方法,其4種主要分型可對(duì)應(yīng)DDH的一系列病理改變。
1.1.2Morin法
Morin等[14]于1985年報(bào)道了髖關(guān)節(jié)屈曲位冠狀位超聲檢查方法,測(cè)量股骨頭與髖臼內(nèi)側(cè)交界部到髖臼骨性邊緣的垂直距離(d)與其到股骨頭外緣的垂直距離(D),設(shè)定d與D的比值乘以100%為股骨頭骨性髖臼覆蓋率,以此判斷髖關(guān)節(jié)發(fā)育情況。Terjesen等[15]于1989年提出改良Morin法,即改用超聲探頭的平行線(xiàn)替代Morin法中的垂線(xiàn),所測(cè)得比值為股骨頭覆蓋率(FHC)。應(yīng)用此法測(cè)得正常FHC下限為男嬰47%,女?huà)?4%[11,15],新生兒DDH發(fā)病率為1%~6%[11,16]。
1.2動(dòng)態(tài)超聲檢查法
Novick等[17]于1983年采用多平面、動(dòng)態(tài)的方法行髖關(guān)節(jié)超聲檢查,側(cè)重于股骨頭和髖臼的對(duì)應(yīng)關(guān)系及髖關(guān)節(jié)穩(wěn)定度。根據(jù)髖關(guān)節(jié)在中立位及屈曲位時(shí)水平面和冠狀面的超聲圖像,將髖關(guān)節(jié)分為正常髖關(guān)節(jié)、半脫位髖關(guān)節(jié)與全脫位髖關(guān)節(jié)。但由于該法受操作者手法影響較大,對(duì)圖像的解讀較主觀,其精確性、有效性及臨床應(yīng)用價(jià)值值得商榷。
1.3其他超聲檢查法
Rosendahl等[18]于1992年提出改良Graf法,目的為研究髖關(guān)節(jié)形態(tài)異常及髖關(guān)節(jié)不穩(wěn)定對(duì)DDH治療的影響。該方法結(jié)合了靜態(tài)超聲與動(dòng)態(tài)超聲,可彌補(bǔ)動(dòng)態(tài)超聲檢查法用于單純髖臼發(fā)育不良的中心型髖關(guān)節(jié)檢查缺陷。對(duì)于中心型髖關(guān)節(jié),可直接采用Graf法中標(biāo)準(zhǔn)平面評(píng)估髖關(guān)節(jié)形態(tài);對(duì)于非中心型髖關(guān)節(jié),則先稍牽引大腿使髖關(guān)節(jié)復(fù)位,再進(jìn)行形態(tài)評(píng)估。此外,還需結(jié)合體檢評(píng)估是否同時(shí)存在髖關(guān)節(jié)不穩(wěn)定。該法強(qiáng)調(diào)髖關(guān)節(jié)形態(tài)學(xué)在診斷中的主導(dǎo)地位,認(rèn)為超聲影像上形態(tài)學(xué)正常的髖關(guān)節(jié)即使存在髖關(guān)節(jié)不穩(wěn)定,也不需要治療。
上述諸多髖關(guān)節(jié)超聲檢查方法中,Graf法應(yīng)用較廣泛。在我國(guó)及歐洲多個(gè)國(guó)家,DDH的Graf法篩查工作已逐漸開(kāi)展。目前已有諸多文獻(xiàn)[11-13]報(bào)道了Graf法的可行性與準(zhǔn)確性,證實(shí)其具有良好的可靠性,適用于臨床篩查。
2.1GrafⅠ型髖關(guān)節(jié)
GrafⅠ型髖關(guān)節(jié)指形態(tài)學(xué)發(fā)育成熟的穩(wěn)定髖關(guān)節(jié)。除可能會(huì)導(dǎo)致髖關(guān)節(jié)退變的病理情況如全身性神經(jīng)肌肉性疾病、髖關(guān)節(jié)感染等外,目前無(wú)確切證據(jù)表明GrafⅠ型髖關(guān)節(jié)在隨訪(fǎng)過(guò)程中會(huì)出現(xiàn)退變。有學(xué)者[19]認(rèn)為,1月齡內(nèi)的GrafⅠ型髖關(guān)節(jié)存在遲發(fā)型DDH發(fā)生風(fēng)險(xiǎn),但這個(gè)風(fēng)險(xiǎn)極低。
2.2GrafⅡ型髖關(guān)節(jié)
GrafⅡ型髖關(guān)節(jié)尤其值得關(guān)注,因其亞型與行超聲檢查時(shí)嬰兒的年齡密切相關(guān)。GrafⅡ型髖關(guān)節(jié)指形態(tài)學(xué)尚未成熟的穩(wěn)定髖關(guān)節(jié),有骨化成熟的潛力,但需要一定時(shí)間。在判斷早產(chǎn)兒髖關(guān)節(jié)分型時(shí),其年齡必須為矯正年齡(胎齡)。
胎齡為0~6周時(shí),根據(jù)α角是否大于50°可將GrafⅡ型髖關(guān)節(jié)分為GrafⅡa型和GrafⅡc型;由于髖臼在胚胎最初6周骨化程度較快,當(dāng)胎齡達(dá)到6周時(shí),GrafⅡa型髖關(guān)節(jié)可進(jìn)一步分為GrafⅡa(+)型和GrafⅡa(-)型,但這一關(guān)鍵時(shí)間點(diǎn)的臨床意義可能需要進(jìn)一步研究。Roovers等[19]研究發(fā)現(xiàn),95%的GrafⅡa(+)型髖關(guān)節(jié)在胎齡12周時(shí)可轉(zhuǎn)歸為正常髖關(guān)節(jié),而僅84%的GrafⅡa(-)型髖關(guān)節(jié)可轉(zhuǎn)歸為正常髖關(guān)節(jié)。雖然GrafⅡa型髖關(guān)節(jié)總體轉(zhuǎn)歸較樂(lè)觀[8,20-23],但對(duì)于6周這一時(shí)間節(jié)點(diǎn)超聲分型的預(yù)后卻報(bào)道甚少,故需進(jìn)一步研究證實(shí)6周時(shí)髖關(guān)節(jié)超聲分型的臨床意義及是否可指導(dǎo)早期干預(yù)。胎齡12周后,髖關(guān)節(jié)骨化速度減慢,此時(shí)髖關(guān)節(jié)超聲分型如下:發(fā)育成熟的GrafⅠ型髖關(guān)節(jié)、未發(fā)育成熟的GrafⅡb型髖關(guān)節(jié)、嚴(yán)重發(fā)育不良的GrafⅡc型髖關(guān)節(jié)和即將發(fā)生脫位的Graf D型髖關(guān)節(jié)(偏心型)。對(duì)于GrafⅡb、Ⅱc、D型髖關(guān)節(jié)自然轉(zhuǎn)歸的報(bào)道較少,因?yàn)榇蟛糠轴t(yī)師此時(shí)均已采取相應(yīng)治療措施[22-24]。因此,目前對(duì)于Graf Ⅱ型髖關(guān)節(jié)能否在更早(如胎齡6周)時(shí)確定其自發(fā)成熟,還是退化為發(fā)育不良或脫位的髖關(guān)節(jié),尚需進(jìn)一步明確。
2.3GrafⅢ/Ⅳ型髖關(guān)節(jié)
GrafⅢ/Ⅳ型髖關(guān)節(jié)為脫位髖關(guān)節(jié),與GrafⅡb、Ⅱc、D型髖關(guān)節(jié)一樣,檢查時(shí)均已采取治療,故其自然轉(zhuǎn)歸未見(jiàn)文獻(xiàn)報(bào)道。
多種因素如篩查方式不同(體檢、選擇性超聲檢查或超聲普查)、治療指征的把握不同、不同醫(yī)師對(duì)DDH早晚分期界定的理解不同等導(dǎo)致不同研究報(bào)道的新生兒DDH發(fā)病率不盡相同[25-27]。對(duì)于新生兒DDH超聲篩查后是否應(yīng)進(jìn)行治療,目前主要存在2種觀點(diǎn):①髖關(guān)節(jié)形態(tài)學(xué)異常,即髖臼發(fā)育不良是重要治療指征之一;②新生兒髖關(guān)節(jié)不穩(wěn)定是造成DDH的主要病理因素,故需對(duì)存在髖關(guān)節(jié)不穩(wěn)定的患兒進(jìn)行治療。有學(xué)者[28]報(bào)道,采用Graf法進(jìn)行嬰幼兒DDH超聲普查可導(dǎo)致治療率上升。隨著檢查技術(shù)的進(jìn)步、醫(yī)師對(duì)超聲圖像解讀的深入以及DDH超聲篩查流程的優(yōu)化,開(kāi)展超聲篩查后DDH治療率并無(wú)顯著上升。一項(xiàng)涉及534篇文獻(xiàn)的薈萃分析[29]顯示,不同超聲檢查技術(shù)對(duì)DDH治療率無(wú)影響。但Sink等[30]研究認(rèn)為,選擇性超聲篩查模式不足以檢測(cè)出髖臼發(fā)育不良。因此,關(guān)于DDH篩查方式、篩查有效性、治療指征等問(wèn)題目前尚無(wú)定論。
DDH超聲篩查的臨床研究往往受篩查樣本量、隨訪(fǎng)時(shí)間、篩查方式和醫(yī)師對(duì)Graf法檢查結(jié)果的解讀等因素制約。首先,樣本量是否充足直接關(guān)系到結(jié)論是否有臨床意義與統(tǒng)計(jì)學(xué)差異。過(guò)去20多年間雖然諸多文獻(xiàn)[26-27,31-33]均報(bào)道采用Graf法進(jìn)行篩查有降低DDH發(fā)病率的趨勢(shì),但尚無(wú)確切證據(jù)支持Graf法能降低晚期DDH發(fā)病率。其次,診斷遲發(fā)型DDH需要足夠長(zhǎng)的隨訪(fǎng)時(shí)間。2項(xiàng)獨(dú)立的隨機(jī)對(duì)照試驗(yàn)[26-27](隨訪(fǎng)時(shí)間分別為2.3~4.7年和6~11年)均顯示,超聲篩查可降低晚期DDH發(fā)病率,但2篇文獻(xiàn)中數(shù)據(jù)均無(wú)明顯統(tǒng)計(jì)學(xué)差異。第三,目前僅有少數(shù)國(guó)家或地區(qū)可做到髖關(guān)節(jié)超聲普查,大部分地區(qū)由于人力、物力以及醫(yī)療資源配置等問(wèn)題,采用選擇性超聲篩查。此時(shí),對(duì)髖關(guān)節(jié)超聲檢查指征的把握又成為困擾臨床醫(yī)師的新問(wèn)題,即臨床醫(yī)師需在過(guò)度轉(zhuǎn)診與漏診之間作出衡量[30]。由于不同醫(yī)師對(duì)髖關(guān)節(jié)超聲轉(zhuǎn)診指征的看法不盡相同,超聲篩查的“凈利潤(rùn)”仍不明確[34-35]。此外,不同時(shí)間節(jié)點(diǎn)的超聲檢查結(jié)果不同、不同醫(yī)師對(duì)結(jié)果的解讀也不盡相同,也導(dǎo)致DDH診斷率和治療率上的不同[25-27]。
大量文獻(xiàn)研究表明,Graf法篩查DDH臨床可行、結(jié)論準(zhǔn)確。采用Graf法篩查可早期發(fā)現(xiàn)DDH并早期采取治療,故有降低晚期DDH發(fā)病率及手術(shù)率的趨勢(shì)。GrafⅠ型髖關(guān)節(jié)為成熟穩(wěn)定的髖關(guān)節(jié),無(wú)確切證據(jù)支持其會(huì)發(fā)生退變。早期發(fā)現(xiàn)的GrafⅡ型髖關(guān)節(jié)需超聲隨訪(fǎng),大部分GrafⅡa型髖關(guān)節(jié)可自發(fā)骨化成熟,而對(duì)骨化延遲及脫位的髖關(guān)節(jié),則應(yīng)及時(shí)采取相應(yīng)治療措施。
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(收稿:2016-01-23;修回:2016-03-21)
(本文編輯:李圓圓)
200092,上海交通大學(xué)醫(yī)學(xué)院附屬新華醫(yī)院兒童骨科
趙黎E-mail: orthzl@126.com
10.3969/j.issn.1673-7083.2016.03.005