1、,Kienbck病资料汇总,月骨缺血性（无菌性）坏死,女性，55岁，主诉：右腕疼痛2年加重1年入院现病史：2年前起无诱因右腕肿胀，疼痛，活动后明显，近一年来症状加重。查体：右腕肿胀、腕背部压痛，腕关节活动受限，背伸45掌屈30尺桡偏活动正常，轴向叩击第3掌骨可诱发腕部疼痛。否认外伤史、烟酒史、药物史。,术后2月,1843年Peste发现该病,1910年奥地利-维也纳 放射科医生Dr.Robert Kienbck(1871-1953)详细报道该病临床症状和体征 。,流行病学 ,月骨无菌性坏死是腕痛的要原因之一，好发于15-40岁男性体力劳动者的优势手腕。Kienbocks disease is
2、classified as a rare disorder, meaning that it affects fewer than 200,000 people in the US population. 美国：被定义为“罕见病”，全美发病率少于20万人。,病 因 尚不明确,病 因 1 内因学说月骨血供特点,月骨是近侧列腕骨的中心，同时又是腕关节中央列即头-月-桡骨关节链的中心，与邻近骨构成5个关节面，仅掌侧和背侧韧带附着处有小血管进入。,月骨血供,Y型 X型 I型Ge1berman 等描述了血管进入月骨的3 种方式，单一血管或者一面接受血液供应的月骨（占月骨的20% ）发生骨坏死的危险性最大
3、，Gelberman R H ,Bauman T , Menon J , et al, The vascularity of the lunate bone and Kienbocks disease, JHandSurg 5A ,272 , 1980,病 因 2 外因学说外伤,外力诱发的血液灌注减少（如风镐、冲击电钻操作员）反复微损伤导致月骨附着韧带损伤及滋养血管断裂，易导致月骨无菌性坏死。,目前尚无证据证明急性月骨骨折与Kienbock病有联系，单纯骨块移位、韧带损伤甚至月骨脱位均不足以引起月骨缺血坏死。公认的观点是复合因素致病。,病 因 3 内因学说基因缺陷 predisposition
4、,Schiltenwulf and associates548 have shown that the intraosseous pressure of the normal lunate is greater in 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及其同伴发现正常月骨在腕关节伸展位时骨内压比中立位
5、状态下高40mmHg，而正常头状骨的骨内压差别只有7 mmHg。,This rise in intraosseous pressure may help explain the lunates predisposition for osteonecrosis. This mechanism may also explain the increased prevalence of Kienbcks disease in patients with spastic hemiplegia.303 这一现象可以解释为什么月骨更易发生缺血坏死基因缺陷，同时可以解释为什么强直性偏瘫患者月骨缺血性坏死发生率
6、较正常人群高。,病 因 4 内因学说 尺骨负变异,尺骨负变异？（腕关节正位片上显示尺桡骨长度的不同统称为尺骨变异正常桡骨茎突超过尺骨关节端9-12mm。若尺骨关节面与桡骨关节面在一相同水平，则形成中性尺骨变异。偶尔，尺骨投影于更近侧-负尺骨变异，尺骨投影更远侧-正尺骨变异）其中，负尺骨变异，腕关节近侧关节面为桡骨远端承载力的传导，此时月骨恰好为主要承重点，易发生无菌性坏死。,尺骨负变异时，月骨负荷相对集中，月骨因压力增加而发生骨折、骨内微血管损伤，从而发生月骨无菌性坏死。,支持者,Hultn的研究结果（ 1928 ）,HultnO:ber anatomische Variationen der
7、 Handgelenkknochen.Acta Radiol Scand1928;9:155-168,支持者,Gelberman等也发现了尺骨负变异和Kienbocks 病在统计学上有明显的关联性。,支持者,J Hand Surg Am. 1986 Jan;11(1):95-7.Traumatic ulnar variance in Kienbcks disease.Stahl S, Reis ND.Abstract A case of unilateral ulnar minus variant, almost certainly caused by trauma to the distal
8、 ulnar growth plate, and ipsilateral Kienbcks disease is described. The contralateral wrist was normal in all respects. This constitutes additional evidence for the etiologic relationship between Kienbcks disease and the presence of the ulnar minus variant.PMID: 3944454 PubMed - indexed for MEDLINES
9、tahl和Reis等观察到患者单侧腕关节创伤后尺骨负变异畸形而导致的Kienbocks 病的典型病理过程，他们认为这为Kienbocks 病的病因学因素增加了证据。,支持者,反对者,但是，如果尺骨负变异的机械力学因素作为一个独立致病因素，那么在尺骨小头切除术及尺骨截骨短缩术后因该会有更高的Kienbocks 病发病率，这点来说与实际不符。另一方面，如果尺骨负变异是机械性因素，那么负变异值越大则月骨承受的力学刺激就越明显，导致月骨坏死的时间就越早，塌陷和碎裂的程度就越严重。Mirabello等人的研究显示Kienbocks 病患者的发病年龄、月骨塌陷程度与尺骨负变异没有相关性。,Negative
10、 ulnar variance is not a risk factor for Kienbcks disease K. DHoore MD, L. De Smet MD,K. Verellen MD The Journal of Hand Surgery Volume 19, Issue 2, March 1994, Pages 229-231 尺、桡骨远端解剖变异与月骨缺血性坏死关系的研究田光磊王澍寰韦加宁杨克非 中华手外科杂志.1997年3期,反对者,Mirabello427 and Werner and Palmer689 both pointed out that radial sl
11、ope in the anteroposterior projection can have a significant effect on both the forces transmitted to the lunate and the age at onset of Kienbcks disease. Mirabello 、 Werner 和 Palmer均指出桡骨远端尺偏角不仅牵涉桡月关节力量传递，同时对Kienbock病初始发病年龄有明显影响。,病 因 5 内因学说 桡骨远端尺偏角,J Hand Surg Br. 1993 Feb;18(1):70-5.Title： Anatomic
12、al risk factors for Kienbcks disease.Author： Tsuge S, Nakamura R.Department of Orthopaedic Surgery, Branch Hospital, Nagoya University School of Medicine, Japan.Abstract Contralateral unaffected wrists from 41 males with Kienbcks disease were compared with wrists from 66 normal males. From X-rays, v
13、arious features of the lunate and radius were measured. In patients with Kienbcks disease, the lunate tended to be smaller and inclined more radially than in normal subjects and the radial inclination was flatter. Discriminant analysis showed that 85% of the unaffected contralateral wrists in patien
14、ts with Kienbcks disease and 74% of the wrists in normal subjects were accurately discriminated to their respective groups. It may be possible to identify subjects who are at risk for Kienbcks disease prior to onset using discriminant analysis.PMID: 8436868 PubMed - indexed for MEDLINE,但是使用楔形截骨术（进一步
15、减小了桡骨远端尺偏角）治疗月骨缺血性坏死，却取得了良好的疗效;究竟是小尺偏角还是大尺偏角与月骨缺血坏死有关，目前尚不清楚。,晕,病 因 n.,导致骨坏死的各种因素，诸如： 胶原沉着病（硬皮病）、系统性红斑狼疮、 链状细胞性贫血、长期应用激素等等,病 理,Symptoms症状,月骨缺血性坏死临床表现为腕部疼痛、酸胀，并有一定程度的活动受限，尤以腕背伸受限为主。在腕背部月骨部位有局限性压痛，叩击第二三掌骨头时可诱发腕部疼痛。本病的进展期可有腕关节运动疼痛和握力下降。,诊断,X-ray片是确定月骨无菌性坏死的基本依据，且可了解其进展程度。MRI对腕骨缺血性改变比较敏感，在T1、T2上表现为低信号。在
16、早期病变单纯X-ray片不易发现时，MRI检查对本病诊断有重要意义。,分期,Kienbck 病的分期是制定治疗方案的主要依据。目前使用最广泛的是Lichtman分期。,月骨结构正常，无X线表现或者线形或压缩性骨折的征象，增强MRI 扫描可显示有所变化但在X 线片上无变化。,The lunate is not collapsed. Carpal alignment is normal. There is no osteoarthritis 月骨无塌陷、腕骨排列正常，无骨性关节炎。,期,Lunate fragmentation and initial lunate collapse. Carpal
17、 height is normal. There is no osteoarthritis.月骨断裂，塌陷初期，腕高正常，无骨性关节炎。,月骨外形正常，但内部有确切的密度改变。,期,Lunate fragmentation and collapse with carpal shortening and instability. There is no evidence of osteoarthritis.月骨碎裂、塌陷，腕高丢失并腕关节不稳，无明显骨性关节炎。,月骨有塌陷或碎裂，头状骨向近侧移位(腕骨高度比例 0.54 + 0 .03),期,a期,Weiss又将期分为：A 期骨折有硬化或塌陷或
18、两者均有。B 期舟骨发生旋转伴A 期的变化。,b期,a期,The last stage, when perilunate osteoarthritic changes are present.终末期，近月点骨性关节炎。,腕关节内有广泛的退行性改变。,期,关节镜下分型,BainG等7在关节镜下根据坏死月骨关节面累及的部分将月骨坏死分成4期，期：坏死月骨关节面单纯累及桡骨关节面，期：坏死月骨关节面单纯发生于头状骨关节面，期：坏死月骨关节面同时累及桡骨关节面和头状骨关节面，期：所有月骨关节面均累及。BainG,BeggM:Arthroscopic assessment and classificat
19、ion of Kienbocks disease.Tech Hand Up Extrem Surg2006;10:8-13.,关于腕骨高度比率判断月骨塌陷及腕关节稳定性,3 个运动学指数:第3 掌骨高度（L1 ） 、腕骨高度( L2 )和腕尺距离( L3 )。腕骨高度比是L2/L 1 ，腕尺距离比是L3/L1,如Kienbck 病患者的腕骨与第3 掌骨高度比减小。Youm 等证明此比率为0.54 士0.03 ，比率明显减小，表明腕骨出现广泛塌陷。,L1=69L2=35L2/L1=0.507Youm 等证明此比率为0.54 士0.03比率减小，表明腕骨濒临塌陷。本例患者病情处于期a期过渡阶段。,
20、治 疗Treatment,治疗,非手术,手术,针对易患因素（目的：延缓月骨塌陷）,针对塌陷月骨（目的：缓解症状）,非手术治疗Nonoperative Treatment,有学者建议对早期的病例( 1 期或2 期，即在出现硬化、碎裂或塌陷之前) ，进行简单的管型石膏固定。这种治疗包括用管型石膏固定数周; 如果有必要，随后复查X 线片，寻找后期可能变明显的隐匿性骨折或月骨缺血性改变或是其他异常， 包括以前漏诊的腕舟骨骨折。这种治疗方法一般需要制动4 个月以上， 且结果不确定，所以通常令人无法接受。,支持者,Stahl585 recommended prolonged immobilization
21、as the treatment of choice for Kienbcks disease. Evidence has been presented that suggests that patients not subjected to operative treatment rarely change their occupations whereas different surgical procedures result in an inability to resume occupations in nearly half of all cases.536推荐长期制动结果：保守治
22、疗患者基本都在原有工作岗位，而手术治疗患者有一半不能重返工作。,支持者,Tajima surveyed 80 wrists with Kienbcks disease seen during a 42-year period and noted no appreciable difference in the end results of nonoperative versus surgical treatment.604用时42年随访了80腕结果：认为手术和保守治疗效果无差别。,支持者,Kristensen and coworkers monitored 46 nonsurgical pat
23、ients for 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。仅1/4患者还在疼.341 Evans and associates173 also found satisfactory wrist function in long-term follow-up of patients treated nonsurgically.腕关节功能满意
24、 Taniguchi and colleagues610 reported radiographic worsening in 70% of the 20 patients they monitored for 35 years, but only 20% of their patients had disabling symptoms. 虽然70%的患者影像片上骨关节炎在恶化，但仅20%患者关节功能障碍在加重。,反对者,Other long-term follow-up studies, however, have shown failure rates from nonoperative
25、treatment to approach 80%42,416 and inferior results when compared with radial shortening procedures.538 然而另有长期随访报道证实非手术治疗失败率达80%，效果比桡骨短缩术差。,手术治疗Operative Treatment,There are many treatment options for Kienbcks disease, ranging from observation to complex surgical reconstruction. The search for a un
26、iversally acceptable treatment plan continues some 80 years after Kienbcks description. A review of the literature shows that many different surgical procedures have been proposed to correct any of the multiple factors that may lead to lunate collapse or to treat the collapsed lunate itself.,治疗Kienb
27、ck 病有很多种手术方案。关节“ 平均” 术包括尺骨延长术和挠骨短缩术，适用于I 期至IIl A 期Kienbck 患者， 伴尺侧负变异， 无桡月关节或者头月关节退行性变。桡骨楔形植骨通过减少腕骨远端桡尺倾斜来减少月骨的负重。月骨血管成形术通过移植各种带蒂的骨移植物来保护月骨结构。这些血管成形术通常通过舟骨、头状骨关节或者舟大小多角骨关节插入钢丝或者使用外固定架来保护月骨。月骨切除可以得到短期的缓解。月骨假体修复也可以得到缓解。部分腕骨间融合术可以在月骨切除后防止近端腕骨移位， 同时帮助减少月骨假体所承受的压力。当腕关节出现广泛继发性关节炎改变后( IV 期) ，治疗方法通常为近侧列腕骨切除术
28、或腕关节融合术。,针对易患因素,月骨血运重建术I 期至IIl A 期关节平衡术I 期至IIl A 期伴 尺骨负变异 月骨减压术 ：如外固定、舟-大-小多角骨(STT)关节融合术、舟-头关节融合术、头状骨短缩术(适用于无尺骨负变异病例),桡骨短缩术,尺骨延长术,楔形截骨,横形或梯形截骨,血管束植入,游离骨移植（通常选用髂骨）,带蒂骨瓣移植,针对月骨塌陷,月骨摘除术 近排腕骨切除术IV 期腕关节融合术 IV 期腕部支配神经离断术 IV 期,摘除+替代物植入期a期,单纯摘除期a期,摘除+替代物植入+部分关节融合IIIB期,Kienbcks Disease in Children,Kienbcks d
29、isease is rare in children. Until recently, only single case reports had been published; these suggested that a nonoperative approach might benefit children more than adults. Recently, Kim and coworkers324 reported seven children aged 9 to 15 with Kienbcks disease. All were initially treated nonoper
30、atively. 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 seem reasonable to manage children with Kienbcks disease who were younger than 12 with 6
31、to 12 weeks of cast immobilization. This approach also may be tried in older children, but it seems likely that children age 12 or older will respond more like adults.,谢谢！,血管束植入,Hori and colleagues described a technique for vascular bundle implantation using the second dorsal intermetacarpal artery.
32、70 Hori描述了第二骨间背侧血管束移植技术 70. HoriY,TamaiS,OkudaH,et al:Blood vessel transplantation to bone.J Hand Surg Am1979;4:23-33.,J Hand Surg Am. 1979 Jan;4(1):23-33.Blood vessel transplantation to bone.Hori Y, Tamai S, Okuda H, Sakamoto H, Takita T, Masuhara K.Abstract In dogs an artery, a vein, and a vascula
33、r bundle were transplanted into intact bone, isolated bone segments, necrotized bone, and 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 isolate
34、d bone, 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 Kienboecks disease, and avascular necrosis of the scaphoid, the femoral head, the talus, and other conditions.PMID: 759500 PubMed - i
35、ndexed for MEDLINE狗 脉管束植入完整骨、游离骨、坏死骨、同种异体骨，均有新生血管张入。为Kienbock病、舟状骨缺血坏死、股骨头坏死、距骨坏死提供新的治疗方法。,可被移植的带蒂骨瓣有：桡骨远端旋前方肌附丽点骨瓣 桡骨远端截取以第4 和第5 伸肌间室动脉为蒂的骨瓣 豌豆骨骨瓣 第2、3掌骨背侧基底部骨瓣 头状骨骨瓣,Shin 和Bishop等描述了从桡骨远端截取以第4 和第5 伸肌间室动脉为蒂的移植骨来使月骨血管重建。采用血管化技术治疗的月骨有60% - 95% 恢复了正常结构和血液供应。这个技术对于90% 的患者可以有效缓解疼痛和改善功能。,适应症：适用于I 期至IIl A
36、 期Kienbck 患者， 伴尺侧负变异， 无桡月关节或者头月关节退行性变。,头状骨短缩术,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 lunate (black arrows). The triquetrum is m
37、ore 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) results in lunate failure (see Arner and Hagberg27). (From Taleisnik J: The Wrist. New York, Churchill Livingstone, 198
38、5. 1985, Elizabeth Roselius.),胡桃夹效应,月骨摘除,Although several authors have reported good results after simple excision of the lunate,59,103,161,228,391,525 others have called this procedure a “crippling” or a “mutilating” operation584 and a useless procedure.59有作者报道单纯月骨摘除可取得良好效果，但也有作者称该术式可能导致腕部不稳甚至功能障碍，
39、并对其效果持怀疑态度。,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 tissues167,288,317,319,643 have been proposed.主张使用替代物来防止月骨摘除后其它腕骨滑移，钴铬合金、树脂、硅橡胶及生物材料假体均有尝试。,Vitallium and acrylic prostheses have ha
40、d only limited clinical trials, without widespread acceptance. A Silastic implant has been used extensively in the past, but problems with silicone synovitis have sharply reduced acceptance of this implant.*钴铬合金及树脂假体仅进行了有限临床试验，并未广泛普及；硅胶假体曾大量使用，但由于并发症多，尤其硅胶性滑膜炎的限制也使用量锐减。,A review of the literature su
41、ggests that the clinical results of most soft tissue lunate replacement procedures288,317,319,643 are similar to those of silicone implants.8,317,367,368,589,603 Lunate replacement will not prevent carpal collapse97,252,317,588; however, some studies have reported long-term satisfactory results with fascial replacement.98 有文献报道软组织替代物和硅胶植入物均不能防止腕骨塌陷，但也有文献报道使用筋膜组织替代物后长期疗效满意。,Indications for lunate excision and arthroplasty should probably be confined to stage III patients until further long-term studies are available. 月骨摘除+关节成形术的适应症是：3期病变+能保证长期随访的患者。,
