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KIENBO ¨ CK DISEASE BY BRIAN DIVELBISS, MD, AND MARK E. BARATZ, MD Kienbo ¨ck disease is a disorder of undetermined etiology that results in osteonecrosis of the lunate. It most commonly affects the dominant hand in men aged 20 to 40 years. The natural history of the disorder is uncertain. Treatment ranges from immobilization to revascularization of the lunate, but no single treatment has been proven superior. In many cases, the radiographic results do not correlate with the clinical outcome. Copyright © 2001 by the American Society for Surgery of the Hand I n 1910, Robert Kienbo ¨ck, a radiologist in Vi- enna, described the radiographic and clinical characteristics of a disease he termed “lunatoma- lacia.” 1 He believed that the underlying cause of this condition was rupture of the ligaments and vessels surrounding the lunate, which resulted in fracture and subsequent collapse. Although this condition would eventually bear his name, the initial description of lunate collapse secondary to suspected trauma was presented in 1843 by Peste. 2 The treatment of Kien- bo ¨ck disease was significantly advanced by Hulte ´n, 3 who in 1928 noted an association between the disease and the presence of negative ulnar variance. He was the first to present radial shortening as a possible form of treatment, and this was followed shortly thereafter by Persson, 4 who advocated lengthening the ulna. Several decades later, intercarpal fusions and revascu- larization procedures were introduced for the treat- ment of Kienbo ¨ck disease. The myriad number of treatments that have been described for this disorder is evidence that we are far from solving its riddle. Our treatment is hampered because a clearly defined eti- ology and natural history have not been determined. ETIOLOGY A lthough the underlying etiology of Kienbo ¨ck disease is still unclear, it is generally agreed that the endpoint of fragmentation and collapse is second- ary to osteonecrosis. Both extrinsic and intrinsic fac- tors have been offered as causes of the disease. 5 Ex- trinsic factors include the relationship between the radius of curvature of the capitate head and distal lunate, concentration of axial loads preferentially through the lunate, and ulnar variance; intrinsic fac- tors include the vascular supply and trabecular ar- rangement of the lunate. The radius of curvature of the capitate is less than that of its articulating surface with the lunate. Com- pressive forces are therefore concentrated on the distal articular surface of the lunate. Late in the disease, as the capitate migrates proximally, it can act as a wedge, splitting the lunate into dorsal and volar por- tions. Conventional wisdom has suggested that repetitive microtrauma plays a role in the disease, but there are no specific data to support this contention. The ab- sence of a single traumatic event is common. Curi- ously, Kienbo ¨ck disease is not seen after perilunate From the Department of Orthopaedic Surgery, Allegheny General Hospital, Pittsburgh, PA. Address reprint requests to Mark E. Baratz, MD, Department of Orthopaedic Surgery, Allegheny General Hospital, 490 East North Avenue, #500, Pittsburgh, PA 15212. Email: [email protected] Copyright © 2001 by the American Society for Surgery of the Hand 1531-0914/01/0101-0001$35.00/0 doi:10.1053/jssh.2001.19885 JOURNAL OF THE AMERICAN SOCIETY FOR SURGERY OF THE HAND z VOL. 1, NO. 1, FEBRUARY 2001 61

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Page 1: KIENBOCK DISEASE - Northwestern Universitysites.surgery.northwestern.edu/reading/Documents/curriculum/Hartig… · disease. Lichtman et al15 modified Stahl’s classification of

KIENBOCK DISEASE

BY BRIAN DIVELBISS, MD, AND MARK E. BARATZ, MD

Kienbock disease is a disorder of undetermined etiology that results in osteonecrosisof the lunate. It most commonly affects the dominant hand in men aged 20 to 40years. The natural history of the disorder is uncertain. Treatment ranges fromimmobilization to revascularization of the lunate, but no single treatment has beenproven superior. In many cases, the radiographic results do not correlate with theclinical outcome.

Copyright © 2001 by the American Society for Surgery of the Hand

In 1910, Robert Kienbock, a radiologist in Vi-enna, described the radiographic and clinicalcharacteristics of a disease he termed “lunatoma-

lacia.”1 He believed that the underlying cause of thiscondition was rupture of the ligaments and vesselssurrounding the lunate, which resulted in fracture andsubsequent collapse. Although this condition wouldeventually bear his name, the initial description oflunate collapse secondary to suspected trauma waspresented in 1843 by Peste.2 The treatment of Kien-bock disease was significantly advanced by Hulten,3

who in 1928 noted an association between the diseaseand the presence of negative ulnar variance. He wasthe first to present radial shortening as a possible formof treatment, and this was followed shortly thereafterby Persson,4 who advocated lengthening the ulna.Several decades later, intercarpal fusions and revascu-larization procedures were introduced for the treat-ment of Kienbock disease. The myriad number oftreatments that have been described for this disorder

is evidence that we are far from solving its riddle. Ourtreatment is hampered because a clearly defined eti-ology and natural history have not been determined.

ETIOLOGY

A lthough the underlying etiology of Kienbockdisease is still unclear, it is generally agreed that

the endpoint of fragmentation and collapse is second-ary to osteonecrosis. Both extrinsic and intrinsic fac-tors have been offered as causes of the disease.5 Ex-trinsic factors include the relationship between theradius of curvature of the capitate head and distallunate, concentration of axial loads preferentiallythrough the lunate, and ulnar variance; intrinsic fac-tors include the vascular supply and trabecular ar-rangement of the lunate.

The radius of curvature of the capitate is less thanthat of its articulating surface with the lunate. Com-pressive forces are therefore concentrated on the distalarticular surface of the lunate. Late in the disease, asthe capitate migrates proximally, it can act as awedge, splitting the lunate into dorsal and volar por-tions.

Conventional wisdom has suggested that repetitivemicrotrauma plays a role in the disease, but there areno specific data to support this contention. The ab-sence of a single traumatic event is common. Curi-ously, Kienbock disease is not seen after perilunate

From the Department of Orthopaedic Surgery, Allegheny GeneralHospital, Pittsburgh, PA.Address reprint requests to Mark E. Baratz, MD, Department ofOrthopaedic Surgery, Allegheny General Hospital, 490 East NorthAvenue, #500, Pittsburgh, PA 15212. Email: [email protected]

Copyright © 2001 by the American Society for Surgery of the Hand1531-0914/01/0101-0001$35.00/0doi:10.1053/jssh.2001.19885

JOURNAL OF THE AMERICAN SOCIETY FOR SURGERY OF THE HAND z VOL. 1, NO. 1, FEBRUARY 2001 61

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dislocations. Although early signs of osteonecrosissuch as increased radiodensity are often seen, progres-sion to collapse has not been reported.

Much has been written about the relationship be-tween Kienbock disease and negative ulnar vari-ance.3,6-9 Hulten found that 23% of the general pop-ulation have negative ulnar variance, whereas 74% ofhis patients with Kienbock disease were ulnar nega-tive. Although a causal relationship cannot be proven,a statistical relationship appears evident. The ulnar-negative variant produces a situation in which loadingacross the radiocarpal joint is not shared normallybetween the ulna and radius. The lunate bears thebrunt of this abnormal loading. The triangular fibro-cartilage complex is thicker in these patients, and thedifference in resistance to loading between it and theulnar edge of the radius is accentuated.

The vascular supply of the lunate has been exten-sively studied. Most lunates have a dual blood supplywith both dorsal and palmar inflow and a rich anas-tomotic network.10 Given this finding, it is hard toascribe the osteonecrosis to a major vessel disruptionin most cases. Increased intraosseous pressure has beenshown in femoral heads with osteonecrosis, and thisfinding has been duplicated in lunates with Kienbockdisease.11 This suggests that impairment of venousdrainage may contribute to bone necrosis.

NATURAL HISTORY

The natural history of Kienbock disease is not wellunderstood because there are few studies that

focus primarily on nonoperative treatment. Kristensenet al12 reviewed 49 patients who were managed non-operatively with an average follow-up time of 20.5years. They found that 80% of these patients had nopain or experienced pain only with hard work. Themajority reported a gradual decrease in symptomsover time, and Kristensen described this as a benigncourse. The radiographic course of the disease, how-ever, did not mirror the clinical course. All 49 lunateswere deformed, and two thirds of patients showedevidence of osteoarthritis in the radiocarpal or carpaljoints. Mikkelson and Gelineck13 also found that 80%of their 25 patients, followed up for 8 years, had nopain or experienced pain only with hard work. How-ever, their conclusion was that nonoperative treatmentis unsuccessful. Nearly 60% of the patients had daily

problems with their wrists, and 7 required a change inoccupation.

DIAGNOSIS

The most common clinical presentation is a managed 20 to 40 years who complains of intermit-

tent wrist pain, decreased wrist motion, and a weakgrip in the dominant hand. Many of the patients aremanual laborers. There may be a long history ofsymptoms before presentation. Usually, the pain isactivity related and abates with rest. A history oftrauma is variable and may be in the distant past. Thewrist may be swollen and tender to palpation over theradiocarpal joint. Motion is predictably diminished inthe flexion/extension arc, and grip strength is com-monly decreased to 50% of the unaffected side.14

Evaluation of plain radiographs forms the basis forthe staging and treatment of patients with Kienbockdisease. Lichtman et al15 modified Stahl’s classificationof Kienbock disease16 by dividing the disease into 4stages (Table 1, Figs 1A-D). Initially, the lunateappears to be more radiodense, which is consistentwith the radiologic appearance of osteonecrosis. Acoronal fracture that splits the lunate into dorsal andvolar fragments may be noted. If the lunate does notrevascularize, the fractures will not heal and the lunatewill collapse. This is especially evident on the lateralview, where the capitate may be imagined to be thepestle in the mortar of the lunate, splitting it in half.Scaphoid flexion occurs secondary to the proximaldescent of the capitate into the area of lunate collapse.Finally, the altered carpal mechanics result in radio-graphically visible degenerative disease around thelunate.

Other imaging modalities can be helpful. Bonescintigraphy may be useful to exclude Kienbock dis-

TABLE 1Staging of Kienbo ¨ck Disease

Stage Radiographic Findings

I Normal (MRI shows decreased signal intensity onT1 image)

II Increased radiodensity, possible mild decrease inlunate bone height on radial side

IIIA Lunate bone collapse, no scaphoid bone rotationIIIB Lunate bone collapse, fixed scaphoid bone flexion

IV Degenerative changes around the lunate bone

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ease, but is not sensitive enough to eliminate othermore common causes of wrist pain. It may be helpfulin patients with known disease in one wrist whodevelop mild symptoms on the contralateral side. In apatient whose diagnosis is unclear, the examinationmost commonly used is magnetic resonance imaging(MRI). This is an extremely sensitive test for detectingchanges in marrow fat that are consistent with, butnot diagnostic of, osteonecrosis. On the T1-weightedimage, a lunate with Kienbock disease will show lowto medium signal intensity instead of high signalintensity (Fig 2). This decreased signal may occupythe entire bone or only focal portions.17 MRI providesa method for diagnosing this disorder in its earlieststage, giving the opportunity for early intervention.High-quality tomograms have been shown to enhance

the staging of Kienbock disease by better showingearly collapse, thus upgrading many stage II patientsto stage III.18

TREATMENT

Treatment is based on the stage of disease and themeasurement of ulnar variance. There is no agree-

ment on the optimal way to treat Kienbock disease. Theoptions range from immobilization to revasculariza-tion procedures. Although the natural history of pro-gressive collapse leading to perilunate arthritis is un-questioned, these changes do not universally lead to apoor clinical outcome. Although comparison of tech-niques is difficult because most studies have smallnumbers and inadequate follow-up periods, the results

FIGURE 1A. Stage II disease.

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do not vary significantly between the options fortreatment.

Immobilization. Several researchers, in long-termfollow-ups of patients treated conservatively versusthose treated surgically, noted no significant differ-ence in pain and function between the 2 groups.14,19

Caution should be used in interpreting these results,because the patients were not randomized and surgerywas reserved for patients who failed nonoperative care.

Revascularization. The intuitive answer for thetreatment of avascular necrosis is to provide a newsource of arterial blood. This is a well documentedapproach to femoral head osteonecrosis, and it hasbeen used for Kienbock disease as well. In 1979, Horiet al20 presented the initial results of a novel revascu-larization technique that implanted a dorsal metacar-pal vascular pedicle directly into the lunate. Results oflonger-term follow-up studies of this technique21

showed disappearance or reduction of pain in 50 of 51patients and increased strength in all 51. These im-provements in clinical symptoms were not necessarilyassociated with radiographic improvement, however,and increased degenerative changes were noted inalmost 20% and fragmentation in nearly 10% ofpatients. In a number of these cases, additional pro-cedures such as bone grafting, scaphotrapeziotrapezoi-dal (STT) fusion, or external fixation were performedas an adjunct. Newer methods for providing a bloodsupply use vascularized bone pedicles that can berotated and implanted into the lunate. Sheetz et al22

elegantly showed the blood supply of the distal radiusand ulna and identified several vascular pedicles thatprovide a wide arc of rotation and can be harvestedwith relative ease (Figs 3 and 4). As with other bonesaffected with osteonecrosis, there is a concern that

FIGURE 1B. Stage IIIA disease. (Courtesy Joseph Imbriglia, MD.)

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collapse may follow revascularization unless additionalsupport is provided to decrease the load across thelunate during the healing phase.23 This has beenaccomplished with the use of external fixation span-ning the wrist.21

Biomechanical basis for joint leveling and inter-carpal fusion procedures. Biomechanical studies onvarious options of treatment include both cadaver andcomputer modeling experiments. Iwasaki et al,24 in a3-dimensional modeling study of intercarpal fusions,found that although both scaphocapitate (SC) andSTT fusions significantly reduced the load across thelunate, this load was preferentially shifted to theunfused articulations around the scaphoid, includingthe radioscaphoid joint. A cadaveric experiment25

comparing intercarpal fusions with joint-leveling pro-cedures noted a 70% strain reduction at the lunate

with STT fusion, ulnar lengthening, and radial short-ening. Only the STT fusion, however, resulted inmotion loss. Ninety percent of the strain reductionafter joint-leveling procedures occurs within the first2 mm of shortening or lengthening. Peak pressure atthe lunate, both in the midcarpal and radiocarpaljoints, is reduced with a radial closing-wedge osteot-omy as well.26 Kinematic studies comparing the SCand STT fusions in a cadaver model showed no supe-riority in motion of one over the other.27 They didnote that although the range of motion lost wasmodest, the kinematic relationships between thescaphoid and lunate are dramatically altered. Capito-hamate (CH) fusion without shortening was unsuc-cessful in reducing load at the lunate in both studies.

Joint-leveling procedures. The most commonlyused procedures for the treatment of Kienbock disease

FIGURE 1C. Stage IIIB disease. Note scaphoid signet ring sign on AP film indicating scaphoid flexion. (Courtesy JosephImbriglia, MD.)

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have focused on leveling the distal radioulnar joint.Both radial shortening and ulnar lengthening havebeen described. The goal in either procedure is toobtain approximately neutral ulnar variance. In 2 ofthe largest studies of radial shortening,28,29 approxi-mately 90% of the patients had decreased pain (Figs 5and 6). Improvement in wrist motion and gripstrength were also noted at 3- to 4-year follow-upexaminations. In both studies, there was a low rate ofcomplication when the osteotomy was performed witha 3.5-mm dynamic compression plate. The radio-

graphic appearance of the lunate, however, showed nosignificant improvement despite a good clinical out-come. This has also been supported in a study ofpatients who had undergone radial osteotomy, inwhom follow-up wrist arthroscopy showed progres-sion of degenerative changes around the lunate in twothirds of the cases, despite clinical outcomes.30 Inulnar-neutral or ulnar-positive patients, successfuloutcomes have also been obtained by performing lat-eral radial closing-wedge osteotomies to decrease ra-dial inclination.31 Ulnar lengthening with intercalary

FIGURE 1D. Stage IV disease.

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bone graft and plate fixation has also resulted in painrelief and improvements in grip strength and range ofmotion.32 Studies comparing the 2 leveling tech-niques have shown essentially equivalent clinical out-comes, but more nonunions and delayed unions resultfrom ulnar lengthening.33,34 The rates of nonunionand delayed union for ulnar lengthening and radialshortening range from 8% to 19% and 0% to 6%,respectively.28,32-34

Intercarpal procedures. Intercarpal fusions areused to treat a variety of conditions in the wrist. Theywere applied to Kienbock disease for their ability tocontrol carpal malalignment and decrease the loadexperienced by the lunate. They may be more useful inulnar-neutral or ulnar-positive patients who are notamenable to joint leveling. Several different variationshave been used for stage II and stage III disease.

STT fusion. As the lunate collapses into stage IIIdisease, the capitate migrates proximally and the

FIGURE 2. T1-weighted MRI showing decreased signal in-tensity in the lunate bone.

FIGURE 3. Potential vascularized pedicled bone grafts from the distal radius.22 (A) Graft based on 1,2 intercompartmentalsupraretinacular artery. (B) Graft based on fourth extensor compartment artery. (Reprinted with permission.22)

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scaphoid begins to palmar flex. STT fusion was ap-plied to this disorder because of concern that thisprogressive rotatory subluxation of the scaphoidwould lead to continued pain and degenerativechanges (Fig 7). Watson et al35 reviewed their expe-rience with 28 STT fusions for Kienbock disease at anaverage follow-up time of 51 months and found that78% of patients had good or excellent pain relief.Range of motion and grip strength were also im-proved. They emphasized that the scaphoid should befused at 55° to 60° of flexion, because a position ofrelative extension compromises motion and accentu-ates the load across the radioscaphoid joint. Unfortu-nately, 43% of these patients required additionaloperative procedures (lunate excision, proximal rowcarpectomy, or wrist arthrodesis) to obtain this result.Watson believes a collapsed lunate is a contraindica-tion to a joint-leveling procedure because it fails toaddress scaphoid rotation. This concern has not beensupported in the radial shortening literature, however,as 20 of 28 patients in Weiss’s study28 were in stageIII (16 stage IIIA, 4 stage IIIB).

SC fusion. SC fusion in Kienbock disease36,37

provided good pain relief but somewhat less motion ascompared with STT fusion. The advantage of thisfusion is that it is technically easier to perform andrequires only 1 fusion site (Fig 8).

Capitate shortening with CH fusion. Origi-nally described as a CH fusion alone,38 this variant hasnow been modified to include capitate shortening.39

This approach reduces load on the lunate by prevent-

FIGURE 5. Radial shortening osteotomy performed with aT-plate for compression.

FIGURE 4. Intraopera-tive photograph show-ing a harvested fourthextensor compartmentpedicled bone graft.

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ing the proximal migration of the capitate. There areno long-term studies to document the success of thistechnique (Fig 9).

Salvage procedures. Proximal row carpectomy(PRC) and wrist fusion are salvage procedures thatare reserved for advanced stages of Kienbock diseaseand failures with other treatments. A number ofresearchers40-42 have reported relatively good resultswhen PRC was used to treat various conditionsincluding Kienbock disease. One can expect toachieve approximately 75° of motion in the flexion/extension arc and a grip strength of 75% of theunaffected side. Pain relief has been achieved inmore than 80% of the patients. These results were

confirmed in a study that specifically addressedPRC in patients with stage III Kienbock disease.43

The obvious advantage of PRC over arthrodesis ispreservation of motion. Some researchers40,41 alsorecommend the addition of a radial styloidectomy ifimpingement of the triquetrum on the radialstyloid in radial deviation is noted intraoperatively.A common dilemma is determining whether thearticular damage to the capitate head or lunate fossais too severe to perform a PRC. The appearance mayrange from a small partial-thickness defect to com-plete eburnation. The maximal amount of damagewith which a PRC can be successfully performed isunknown, and there are no good data to aid in this

FIGURE 6. Radial shorten-ing osteotomy performedwith a 6-hole dynamic com-pression plate (A) allowingcompression with a lagscrew through the plate (B).Alternatively, a transverseosteotomy can be used witha 6-hole plate.

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decision. Imbriglia et al40 recommend adding acapsular imbrication if there is full-thickness dam-age less than 3 mm in size. Larger lesions may

necessitate wrist fusion. A failed PRC can success-fully be converted to arthrodesis.

Other options. Despite encouraging early results,silicone implants into the lunate space15 were noteffective because the implants were found to deterio-rate secondary to particulate synovitis.44 Theseimplants can no longer be considered a primary treat-ment modality. Zelouf and Ruby45 performed cancel-lous bone grafting with external fixation and foundthat it provided good pain relief in 80% of patientsand contributed to improvement in both gripstrength and range of motion. They also detectedevidence of revascularization on postoperative MRIscans in a third of their patients. Lunate excision hasgenerally been avoided because of concerns about sub-sequent carpal collapse. The addition of a fascial in-terposition graft after lunate excision has been advo-cated and has yielded good long-term results in asmall series.46 Arthroscopic debridement in patientswith stage II disease was successful in reducing pain,increasing range of motion, and providing completerelief of mechanical symptoms.47 Although this studyhad a small number of patients and a relatively shortfollow-up period, it does show that this simple pro-cedure may provide relief of symptoms. Finally, in theEuropean community, wrist denervation has been ad-vocated both as an adjunct and as a primary proce-dure.48

FIGURE 7. STT fusion should be combined with a radialstyloidectomy.

FIGURE 8. SC fusion provides a large surface area for fusionto occur and easy surgical access.

FIGURE 9. Capitate bone shortening is often combined withfusion of the CH joint.

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TREATMENT BY STAGE

Because there is no literature that discusses themanagement of stage I disease, it seems prudent

to begin with immobilization in treating these cases.Most patients, however, will not present this early inthe disease course. Stage II disease with negativeulnar variance can be effectively managed withradial shortening. Vascularized bone grafting isparticularly attractive for young patients with stageII or stage IIIA disease who manifest neutral orpositive ulnar variance. A lateral closing-wedgeosteotomy of the radius is also an option for thepatient with an ulnar-positive wrist. Stage IIIAdisease with negative ulnar variance can also beeffectively treated with radial shortening, and al-though good results have been obtained in a fewpatients with stage IIIB disease, many surgeonsnow favor PRC. Stage IV wrists can also be man-aged with PRC, provided the damage to the capi-

tate head or lunate fossa is not too severe. The exactrole for capsular interposition in addition to PRC isunknown. Total wrist arthrodesis is reserved forcases of failed PRC or if significant cartilage dam-age is noted on the head of the capitate or in thelunate fossa.

CONCLUSION

Anumber of issues are unresolved with regard toKienbock disease. The etiology and natural history

of the disorder are not clearly defined. This makes itdifficult to determine the value of operative intervention.The role of intercarpal fusions, with their requisite loss ofmotion and transfer of loads to the radioscaphoid joint, isdebatable. Further work needs to focus on the role ofrevascularization procedures and whether they provideadded benefit over joint-leveling procedures. Radialshortening and proximal row carpectomy remain themainstays of treatment of this disease.

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13. Mikkelsen SS, Gelineck J. Poor function after nonoperativetreatment of Kienbock’s disease. Acta Orthop Scand 1987;58:241-243.

14. Beckenbaugh RD, Shives TC, Dobyns JH, Linscheild RL.Kienbock’s disease: The natural history of Kienbock’s diseaseand consideration of lunate fractures. Clin Orthop 1980;149:98-106.

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19. Delaere O, Dury M, Molderez A, Foucher G. Conservativeversus operative treatment for Kienbock’s disease. J HandSurg Br 1998;23B:33-36.

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22. Sheetz KK, Bishop AT, Berger RA. The arterial blood supplyof the distal radius and ulna and its potential use in vascu-

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