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1、,Kienböck病資料匯總,月骨缺血性(無菌性)壞死,女性,55歲,主訴:右腕疼痛2年加重1年入院現(xiàn)病史:2年前起無誘因右腕腫脹,疼痛,活動(dòng)后明顯,近一年來癥狀加重。查體:右腕腫脹、腕背部壓痛,腕關(guān)節(jié)活動(dòng)受限,背伸45°掌屈30°尺橈偏活動(dòng)正常,軸向叩擊第3掌骨可誘發(fā)腕部疼痛。否認(rèn)外傷史、煙酒史、藥物史。,術(shù)后2月,,1843年P(guān)este發(fā)現(xiàn)該病,1910年奧地利-維也納 放射科醫(yī)生Dr. 

2、;Robert Kienböck (1871-1953)詳細(xì)報(bào)道該病臨床癥狀和體征 。,流行病學(xué) ——,月骨無菌性壞死是腕痛的要原因之一,好發(fā)于15-40歲男性體力勞動(dòng)者的優(yōu)勢(shì)手腕。Kienbock's disease is classified as a "rare disorder," meaning that it affects fewer than 200,000 people

3、 in the US population. 美國(guó):被定義為“罕見病”,全美發(fā)病率少于20萬人。,,,病 因 尚不明確,病 因 1 內(nèi)因?qū)W說—月骨血供特點(diǎn),月骨是近側(cè)列腕骨的中心,同時(shí)又是腕關(guān)節(jié)中央列即頭-月-橈骨關(guān)節(jié)鏈的中心,與鄰近骨構(gòu)成5個(gè)關(guān)節(jié)面,僅掌側(cè)和背側(cè)韌帶附著處有小血管進(jìn)入。,月骨血供,Y型 X型 I型Ge1berman 等描述了血管進(jìn)入月骨的3 種方式,單一血管或者一面接受血液供應(yīng)的月骨(占月骨

4、的20% )發(fā)生骨壞死的危險(xiǎn)性最大,Gelberman R H ,Bauman T , Menon J , et al, The vascularity of the lunate bone and Kienbock's disease, JHandSurg 5A ,272 , 1980,,病 因 2 外因?qū)W說—外傷,外力誘發(fā)的血液灌注減少(如風(fēng)鎬、沖擊電鉆操作員)——反復(fù)微損傷導(dǎo)致月骨附著韌帶損傷及滋養(yǎng)血管斷裂,易導(dǎo)致月骨無

5、菌性壞死。,,目前尚無證據(jù)證明急性月骨骨折與Kienbock病有聯(lián)系,單純骨塊移位、韌帶損傷甚至月骨脫位均不足以引起月骨缺血壞死。公認(rèn)的觀點(diǎn)是復(fù)合因素致病。,,病 因 3 內(nèi)因?qū)W說—基因缺陷 predisposition,Schiltenwulf and associates[548] have shown that the intraosseous pressure of the normal lunate is greater i

6、n wrist extension than in the neutral position and that the difference, 40 mm Hg, is far greater than the difference seen in the normal capitate (7 mm Hg). Schiltenwulf及其同伴發(fā)現(xiàn)正常月骨在腕關(guān)節(jié)伸展位時(shí)骨內(nèi)壓比中立位狀態(tài)下高40mmHg,而正常頭狀骨的骨內(nèi)壓差別只有7

7、 mmHg。,,This rise in intraosseous pressure may help explain the lunate‘s predisposition for osteonecrosis. This mechanism may also explain the increased prevalence of Kienböck’s disease in patients with spastic hemi

8、plegia.[303] 這一現(xiàn)象可以解釋為什么月骨更易發(fā)生缺血壞死——基因缺陷,同時(shí)可以解釋為什么強(qiáng)直性偏癱患者月骨缺血性壞死發(fā)生率較正常人群高。,病 因 4 內(nèi)因?qū)W說— 尺骨負(fù)變異,尺骨負(fù)變異???(腕關(guān)節(jié)正位片上顯示尺橈骨長(zhǎng)度的不同統(tǒng)稱為尺骨變異.正常橈骨莖突超過尺骨關(guān)節(jié)端9--12mm。若尺骨關(guān)節(jié)面與橈骨關(guān)節(jié)面在一相同水平,則形成中性尺骨變異。偶爾,尺骨投影于更近側(cè)---負(fù)尺骨變異,尺骨投影更遠(yuǎn)側(cè)---正尺骨變異)其中,負(fù)

9、尺骨變異,腕關(guān)節(jié)近側(cè)關(guān)節(jié)面為橈骨遠(yuǎn)端承載力的傳導(dǎo),此時(shí)月骨恰好為主要承重點(diǎn),易發(fā)生無菌性壞死。,,尺骨負(fù)變異時(shí),月骨負(fù)荷相對(duì)集中,月骨因壓力增加而發(fā)生骨折、骨內(nèi)微血管損傷,從而發(fā)生月骨無菌性壞死。,支持者,Hultén的研究結(jié)果( 1928 ),Hultén O: Über anatomische Variationen der Handgelenkknochen.  

10、;Acta Radiol Scand  1928; 9:155-168,支持者,,Gelberman等也發(fā)現(xiàn)了尺骨負(fù)變異和Kienbock’s 病在統(tǒng)計(jì)學(xué)上有明顯的關(guān)聯(lián)性。,支持者,J Hand Surg Am. 1986 Jan;11(1):95-7.Traumatic ulnar variance in Kienböck's disease.Stahl S, Reis ND.Ab

11、stract A case of unilateral ulnar minus variant, almost certainly caused by trauma to the distal ulnar growth plate, and ipsilateral Kienböck's disease is described. The contralateral wrist was normal in al

12、l respects. This constitutes additional evidence for the etiologic relationship between Kienböck's disease and the presence of the ulnar minus variant.PMID: 3944454 [PubMed - indexed for MEDLINE]Stahl和Reis等觀察到

13、患者單側(cè)腕關(guān)節(jié)創(chuàng)傷后尺骨負(fù)變異畸形而導(dǎo)致的Kienbock’s 病的典型病理過程,他們認(rèn)為這為Kienbock’s 病的病因?qū)W因素增加了證據(jù)。,支持者,反對(duì)者,但是,如果尺骨負(fù)變異的機(jī)械力學(xué)因素作為一個(gè)獨(dú)立致病因素,那么在尺骨小頭切除術(shù)及尺骨截骨短縮術(shù)后因該會(huì)有更高的Kienbock’s 病發(fā)病率,這點(diǎn)來說與實(shí)際不符。另一方面,如果尺骨負(fù)變異是機(jī)械性因素,那么負(fù)變異值越大則月骨承受的力學(xué)刺激就越明顯,導(dǎo)致月骨壞死的時(shí)間就越早,塌陷和碎

14、裂的程度就越嚴(yán)重。Mirabello等人的研究顯示Kienbock’s 病患者的發(fā)病年齡、月骨塌陷程度與尺骨負(fù)變異沒有相關(guān)性。,Negative ulnar variance is not a risk factor for Kienböck's disease K. D'Hoore MD, L. De Smet MD, K. Verellen MD The Journal of Hand Surge

15、ry Volume 19, Issue 2, March 1994, Pages 229-231  尺、橈骨遠(yuǎn)端解剖變異與月骨缺血性壞死關(guān)系的研究 田光磊    王澍寰    韋加寧  楊克非  中華手外科雜志 . 1997年3期,反對(duì)者,Mirabello[427] and Wern

16、er and Palmer[689] both pointed out that radial slope in the anteroposterior projection can have a significant effect on both the forces transmitted to the lunate and the age at onset of Kienböck's disease. Mir

17、abello 、 Werner 和 Palmer均指出橈骨遠(yuǎn)端尺偏角不僅牽涉橈月關(guān)節(jié)力量傳遞,同時(shí)對(duì)Kienbock病初始發(fā)病年齡有明顯影響。,病 因 5 內(nèi)因?qū)W說— 橈骨遠(yuǎn)端尺偏角,J Hand Surg Br. 1993 Feb;18(1):70-5.Title: Anatomical risk factors for Kienböck's disease.Author: Tsuge S, Nakamura

18、 R.Department of Orthopaedic Surgery, Branch Hospital, Nagoya University School of Medicine, Japan.Abstract Contralateral unaffected wrists from 41 males with Kienböck's disease were compared with wr

19、ists from 66 normal males. From X-rays, various features of the lunate and radius were measured. In patients with Kienböck's disease, the lunate tended to be smaller and inclined more radially than in normal sub

20、jects and the radial inclination was flatter. Discriminant analysis showed that 85% of the unaffected contralateral wrists in patients with Kienböck's disease and 74% of the wrists in normal subjects were accura

21、tely discriminated to their respective groups. It may be possible to identify subjects who are at risk for Kienböck's disease prior to onset using discriminant analysis.PMID: 8436868 [PubMed - indexed for MEDLI

22、NE],,但是使用楔形截骨術(shù)(進(jìn)一步減小了橈骨遠(yuǎn)端尺偏角)治療月骨缺血性壞死,卻取得了良好的療效;究竟是小尺偏角還是大尺偏角與月骨缺血壞死有關(guān),目前尚不清楚。,暈~,病 因 n….,導(dǎo)致骨壞死的各種因素,諸如: 膠原沉著?。ㄓ财げ。⑾到y(tǒng)性紅斑狼瘡、 鏈狀細(xì)胞性貧血、長(zhǎng)期應(yīng)用激素等等,病 理,Symptoms癥狀,月骨缺血性壞死臨床表現(xiàn)為腕部疼痛、

23、酸脹,并有一定程度的活動(dòng)受限,尤以腕背伸受限為主。在腕背部月骨部位有局限性壓痛,叩擊第二三掌骨頭時(shí)可誘發(fā)腕部疼痛。本病的進(jìn)展期可有腕關(guān)節(jié)運(yùn)動(dòng)疼痛和握力下降。,診斷,X-ray片是確定月骨無菌性壞死的基本依據(jù),且可了解其進(jìn)展程度。MRI對(duì)腕骨缺血性改變比較敏感,在T1、T2上表現(xiàn)為低信號(hào)。在早期病變單純X-ray片不易發(fā)現(xiàn)時(shí),MRI檢查對(duì)本病診斷有重要意義。,,,,分期,,,Kienböck 病的分期是制定治療方案的主要依據(jù)。

24、目前使用最廣泛的是Lichtman分期。,月骨結(jié)構(gòu)正常,無X線表現(xiàn)或者線形或壓縮性骨折的征象,增強(qiáng)MRI 掃描可顯示有所變化但在X 線片上無變化。,,,,,The lunate is not collapsed. Carpal alignment is normal. There is no osteoarthritis 月骨無塌陷、腕骨排列正常,無骨性關(guān)節(jié)炎。,Ⅰ期,Lunate fragmentation and initial

25、lunate collapse. Carpal height is normal. There is no osteoarthritis.月骨斷裂,塌陷初期,腕高正常,無骨性關(guān)節(jié)炎。,月骨外形正常,但內(nèi)部有確切的密度改變。,Ⅱ期,Lunate fragmentation and collapse with carpal shortening and instability. There is no evidence of osteoar

26、thritis.月骨碎裂、塌陷,腕高丟失并腕關(guān)節(jié)不穩(wěn),無明顯骨性關(guān)節(jié)炎。,,月骨有塌陷或碎裂,頭狀骨向近側(cè)移位(腕骨高度比例< 0.54 + 0 .03),Ⅲ期,Ⅲa期,,Ⅰ,,Weiss又將Ⅲ期分為:ⅢA 期骨折有硬化或塌陷或兩者均有。ⅢB 期舟骨發(fā)生旋轉(zhuǎn)伴ⅢA 期的變化。,Ⅲb期,Ⅲa期,The last stage, when perilunate osteoarthritic changes are present.

27、終末期,近月點(diǎn)骨性關(guān)節(jié)炎。,,,,,,腕關(guān)節(jié)內(nèi)有廣泛的退行性改變。,Ⅳ期,關(guān)節(jié)鏡下分型,Bain G等[7]在關(guān)節(jié)鏡下根據(jù)壞死月骨關(guān)節(jié)面累及的部分將月骨壞死分成4期,Ⅰ期:壞死月骨關(guān)節(jié)面單純累及橈骨關(guān)節(jié)面,Ⅱ期:壞死月骨關(guān)節(jié)面單純發(fā)生于頭狀骨關(guān)節(jié)面,Ⅲ期:壞死月骨關(guān)節(jié)面同時(shí)累及橈骨關(guān)節(jié)面和頭狀骨關(guān)節(jié)面,Ⅳ期:所有月骨關(guān)節(jié)面均累及。Bain G, Begg M: Arthroscopic

28、 assessment and classification of Kienbock's disease.  Tech Hand Up Extrem Surg  2006; 10:8-13.,關(guān)于腕骨高度比率—判斷月骨塌陷及腕關(guān)節(jié)穩(wěn)定性,3 個(gè)運(yùn)動(dòng)學(xué)指數(shù):第3 掌骨高度(L1 ) 、腕骨高度( L2 )和腕尺距離( L3 )。腕骨高度比是L2/L 1 ,腕尺距離比是L3/L1,如

29、Kienböck 病患者的腕骨與第3 掌骨高度比減小。Youm 等證明此比率為0.54 士0.03 ,比率明顯減小,表明腕骨出現(xiàn)廣泛塌陷。,L1=69L2=35L2/L1=0.507Youm 等證明此比率為0.54 士0.03比率減小,表明腕骨瀕臨塌陷。本例患者病情處于Ⅱ期Ⅲa期過渡階段。,治 療Treatment,,治療,非手術(shù),手術(shù),,針對(duì)易患因素(目的:延緩月骨塌陷),針對(duì)塌陷月骨(目的:緩解癥狀),非手術(shù)治

30、療Nonoperative Treatment,有學(xué)者建議對(duì)早期的病例( 1 期或2 期,即在出現(xiàn)硬化、碎裂或塌陷之前) ,進(jìn)行簡(jiǎn)單的管型石膏固定。這種治療包括用管型石膏固定數(shù)周; 如果有必要,隨后復(fù)查X 線片,尋找后期可能變明顯的隱匿性骨折或月骨缺血性改變或是其他異常, 包括以前漏診的腕舟骨骨折。這種治療方法一般需要制動(dòng)4 個(gè)月以上, 且結(jié)果不確定,所以通常令人無法接受。,支持者,Stahl[585] recommended prol

31、onged immobilization as the treatment of choice for Kienböck's disease. Evidence has been presented that suggests that patients not subjected to operative treatment rarely change their occupations whereas differ

32、ent surgical procedures result in an inability to resume occupations in nearly half of all cases.[536]推薦長(zhǎng)期制動(dòng)結(jié)果:保守治療患者基本都在原有工作崗位,而手術(shù)治療患者有一半不能重返工作。,支持者,Tajima surveyed 80 wrists with Kienböck's disease seen duri

33、ng a 42-year period and noted no appreciable difference in the end results of nonoperative versus surgical treatment.[604]用時(shí)42年隨訪了80腕結(jié)果:認(rèn)為手術(shù)和保守治療效果無差別。,支持者,Kristensen and coworkers monitored 46 nonsurgical patients for

34、 a minimum of 5 years and a mean of 20 years; although two thirds had arthritic changes in the wrist at follow-up, only a fourth had significant pain。僅1/4患者還在疼.[341] Evans and associates[173] also found satisfactory wris

35、t function in long-term follow-up of patients treated nonsurgically.腕關(guān)節(jié)功能滿意 Taniguchi and colleagues[610] reported radiographic worsening in 70% of the 20 patients they monitored for 35 years, but only 20% of their patie

36、nts had disabling symptoms. 雖然70%的患者影像片上骨關(guān)節(jié)炎在惡化,但僅20%患者關(guān)節(jié)功能障礙在加重。,反對(duì)者,Other long-term follow-up studies, however, have shown failure rates from nonoperative treatment to approach 80%[42,][416] and inferior results when c

37、ompared with radial shortening procedures.[538] 然而另有長(zhǎng)期隨訪報(bào)道證實(shí)非手術(shù)治療失敗率達(dá)80%,效果比橈骨短縮術(shù)差。,手術(shù)治療Operative Treatment,There are many treatment options for Kienböck's disease, ranging from observation to complex surgical r

38、econstruction. The search for a universally acceptable treatment plan continues some 80 years after Kienböck's description. A review of the literature shows that many different surgical procedures have been prop

39、osed to correct any of the multiple factors that may lead to lunate collapse or to treat the collapsed lunate itself.,治療Kienböck 病有很多種手術(shù)方案。關(guān)節(jié)“ 平均” 術(shù)包括尺骨延長(zhǎng)術(shù)和撓骨短縮術(shù),適用于I 期至IIl A 期Kienböck 患者, 伴尺側(cè)負(fù)變異, 無橈月關(guān)節(jié)或者頭月關(guān)節(jié)退行

40、性變。橈骨楔形植骨通過減少腕骨遠(yuǎn)端橈尺傾斜來減少月骨的負(fù)重。月骨血管成形術(shù)通過移植各種帶蒂的骨移植物來保護(hù)月骨結(jié)構(gòu)。這些血管成形術(shù)通常通過舟骨、頭狀骨關(guān)節(jié)或者舟大小多角骨關(guān)節(jié)插入鋼絲或者使用外固定架來保護(hù)月骨。月骨切除可以得到短期的緩解。月骨假體修復(fù)也可以得到緩解。部分腕骨間融合術(shù)可以在月骨切除后防止近端腕骨移位, 同時(shí)幫助減少月骨假體所承受的壓力。當(dāng)腕關(guān)節(jié)出現(xiàn)廣泛繼發(fā)性關(guān)節(jié)炎改變后( IV 期) ,治療方法通常為近側(cè)列腕骨切除術(shù)或腕關(guān)

41、節(jié)融合術(shù)。,針對(duì)易患因素,月骨血運(yùn)重建術(shù)I 期至IIl A 期關(guān)節(jié)平衡術(shù)I 期至IIl A 期伴 尺骨負(fù)變異 月骨減壓術(shù) :如外固定、舟-大-小多角骨(STT)關(guān)節(jié)融合術(shù)、舟-頭關(guān)節(jié)融合術(shù)、頭狀骨短縮術(shù)(適用于無尺骨負(fù)變異病例),,橈骨短縮術(shù),尺骨延長(zhǎng)術(shù),,楔形截骨,橫形或梯形截骨,,血管束植入,游離骨移植(通常選用髂骨),帶蒂骨瓣移植,針對(duì)月骨塌陷,月骨摘除術(shù) 近排腕骨切除術(shù)IV 期

42、腕關(guān)節(jié)融合術(shù) IV 期腕部支配神經(jīng)離斷術(shù) IV 期,,,摘除+替代物植入Ⅱ期Ⅲa期,單純摘除Ⅱ期Ⅲa期,摘除+替代物植入+部分關(guān)節(jié)融合IIIB期,Kienböck's Disease in Children,Kienböck's disease is rare in children. Until recently, only single case reports had been publi

43、shed; these suggested that a nonoperative approach might benefit children more than adults. Recently, Kim and coworkers[324] reported seven children aged 9 to 15 with Kienböck's disease. All were initially treat

44、ed nonoperatively. The three patients younger than 12 years of age all healed with nonoperative treatment; all others required surgery, either capitate shortening or radial shortening.,Based on this information, it would

45、 seem reasonable to manage children with Kienböck's disease who were younger than 12 with 6 to 12 weeks of cast immobilization. This approach also may be tried in older children, but it seems likely that childre

46、n age 12 or older will respond more like adults.,謝謝??!,,,血管束植入,Hori and colleagues described a technique for vascular bundle implantation using the second dorsal intermetacarpal artery.[70] Hori描述了第二骨間背側(cè)血管束移植技術(shù) 70. Ho

47、ri Y, Tamai S, Okuda H, et al: Blood vessel transplantation to bone.  J Hand Surg [Am]  1979; 4:23-33.,,J Hand Surg Am. 1979 Jan;4(1):23-33.Blood vessel transp

48、lantation to bone.Hori Y, Tamai S, Okuda H, Sakamoto H, Takita T, Masuhara K.Abstract In dogs an artery, a vein, and a vascular bundle were transplanted into intact bone, isolated bone segments, necrotized bone, a

49、nd homografts of bone. Active proliferation of new blood vessels and formation of new bone occurred in all instances where the vascular bundle was used, when the vein was implanted into the intact bone and isolated bone,

50、 and only when the artery was implanted into intact bone. As a result of these experiments, vascular bundle transplantation has been performed in patients with Kienboeck's disease, and avascular necrosis of the scaph

51、oid, the femoral head, the talus, and other conditions.PMID: 759500 [PubMed - indexed for MEDLINE]狗 脈管束植入完整骨、游離骨、壞死骨、同種異體骨,均有新生血管張入。為Kienbock病、舟狀骨缺血壞死、股骨頭壞死、距骨壞死提供新的治療方法。,,可被移植的帶蒂骨瓣有:橈骨遠(yuǎn)端旋前方肌附麗點(diǎn)骨瓣 橈骨遠(yuǎn)端截取以第4 和第5 伸肌間室動(dòng)

52、脈為蒂的骨瓣 豌豆骨骨瓣 第2、3掌骨背側(cè)基底部骨瓣 頭狀骨骨瓣,,Shin 和Bishop等描述了從橈骨遠(yuǎn)端截取以第4 和第5 伸肌間室動(dòng)脈為蒂的移植骨來使月骨血管重建。采用血管化技術(shù)治療的月骨有60% - 95% 恢復(fù)了正常結(jié)構(gòu)和血液供應(yīng)。這個(gè)技術(shù)對(duì)于90% 的患者可以有效緩解疼痛和改善功能。,,適應(yīng)癥:適用于I 期至IIl A 期Kienböck 患者, 伴尺側(cè)負(fù)變異, 無橈月關(guān)節(jié)或者頭月關(guān)節(jié)退行性變。,,,頭狀

53、骨短縮術(shù),Stage I, II, or IIIA with Ulnar-Positive or Ulnar-Neutral Variance,,,In extreme dorsiflexion, the radiolunate and lunotriquetral ligaments become tense and produce equal forces in opposite directions acting on the l

54、unate (black arrows). The triquetrum is more likely to shift dorsally and proximally on the more compliant ulnocarpal cartilaginous complex (right arrow). Added compression from the radius (arrow A) and capitate (arrow B

55、) results in lunate failure (see Arner and Hagberg[27]). (From Taleisnik J: The Wrist. New York, Churchill Livingstone, 1985. © 1985, Elizabeth Roselius.),胡桃夾效應(yīng),月骨摘除,Although several authors have reported good resu

56、lts after simple excision of the lunate,[59,][103,][161,][228,][391,][525] others have called this procedure a “crippling” or a “mutilating” operation[584] and a useless procedure.[59]有作者報(bào)道單純?cè)鹿钦扇〉昧己眯Ч?,但也有作者稱該術(shù)式可能導(dǎo)致腕部不

57、穩(wěn)甚至功能障礙,并對(duì)其效果持懷疑態(tài)度。,,In an effort to prevent shift of the remaining carpal bones after excision of the lunate, implants of Vitallium,[375] acrylic,[5,][141] Silastic,[408] and biologic tissues[167,][288,][317,][319,][643

58、] have been proposed.主張使用替代物來防止月骨摘除后其它腕骨滑移,鈷鉻合金、樹脂、硅橡膠及生物材料假體均有嘗試。,,Vitallium and acrylic prostheses have had only limited clinical trials, without widespread acceptance. A Silastic implant has been used extensively in

59、the past, but problems with silicone synovitis have sharply reduced acceptance of this implant.*鈷鉻合金及樹脂假體僅進(jìn)行了有限臨床試驗(yàn),并未廣泛普及;硅膠假體曾大量使用,但由于并發(fā)癥多,尤其硅膠性滑膜炎的限制也使用量銳減。,,,,A review of the literature suggests that the clinical re

60、sults of most soft tissue lunate replacement procedures[288,][317,][319,][643] are similar to those of silicone implants.[8,][317,][367,][368,][589,][603] Lunate replacement will not prevent carpal collapse[97,][252,][31

61、7,][588]; however, some studies have reported long-term satisfactory results with fascial replacement.[98] 有文獻(xiàn)報(bào)道軟組織替代物和硅膠植入物均不能防止腕骨塌陷,但也有文獻(xiàn)報(bào)道使用筋膜組織替代物后長(zhǎng)期療效滿意。,,Indications for lunate excision and arthroplasty should pro

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