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Opera-glass hand in rheumatoid arthritis
Opera-glass hand in rheumatoid arthritis Characteristic deformities occur in the fingers, thumb, and wrist in the opera-glass hand in rheumatoid arthritis. Shortening and instability are the result of bone resorption and dislocation and can be severely disabling. Early spontaneous fusion of the proximal interphalangeal joint preserves digital length. Functional improvement can be obtained in the fingers by interphalangeal joint arthrodesis and metacarpophalangeal prosthetic arthroplasty and in the thumb with metacarpophalangeal and/or interphalangeal arthrodesis. With interphalangeal arthrodesis, interposition grafts often are required in order to restore length and securefusion. "Prophylactic" arthrodesis of interphalangeal joints should be considered when resorption seems imminent.
Edward A. Nalebuff, M.D., Brookline, Mass., and John Garrett, M.D., Boston, Mass.
The term main en lorgnette (opera-glass hand) was used initially by Marie and LerjI to describe the deformities of a patient with chronic polyarthritis. The digits were short with excessive skin folds but could be elongated with traction (Fig. 1, A and B). Postmortem studies disclosed resorption of the articular ends of the bones but sparing of the vessels, nerves, and tendons. More recent studies have shown synovial and erosive bony changes consistent with rheumatoid arthritis. 2 Nerve conduction studies have been reported as normal, separating the entity from Charcot neuropathies. 3 The deformity represents an advanced stage of rheumatoid arthritis or one of its variants and often is associated with psoriasis. 4' IO It has been reported to occur with lipoid dermatoarthritis. 11 Doigt en lorgnette (opera-glass finger) describes the disorder in individual digits (Fig. 2).9, 12, 13 Generalized resorptive changes typically referred to as arthritis mutilans may be seen around the wrist and elbow joints, the glenohumeral and acromioclavicular joints, and the ribs, ankle, and toes.5, 9, 14·21 Over 40 cases of opera-glass hand associated with rheumatoid arthritis or psoriasis have been reported. H , 6'10, 12, 13, 16, 17, 19, 22·27 Although loss of digital length and stability can be severely disabling, only one publication deals with reconstructive surgery.6 No mention has been made of operative procedures to prevent the shortening. We have analyzed Received for publication May 11, 1976. Revised for publication Aug. 14, 1976. Reprint requests: Edward A. Nalebuff, M.D., 209 Harvard St., Brookline, Mass. 02140.
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the deformities at individual joints in 13 patients and have outlined the treatment.
Materials and methods Thirteen patients with the deformity of opera-glass hand from the Robert Breck Brigham, Beth Israel, and Massachusetts General Hospitals, Boston, were studied. All of the patients had rheumatoid arthritis; four also had psoriasis. Twelve of the 13 patients were women. The patients' ages ranged from 33 to 72 years, with a mean of 49 years. Arthritis had been present from 8 to 47 years, with a mean of 22 years.
Deformities Fingers. At the carpometacarpal joint, ankylosis was
present in 22 fingers, most often in the index and middle fingers (Fig. 3, A). In 18 hands complete resorption of the carpal bones had taken place, with the metacarpals articulating with the-radius (Fig. 3, B). At the metacarpophalangeal joint level, 29 fingers showed resorption of the metacarpal head, tapering of the metacarpal stump, and an "egg-cup" deformity of the base of the proximal phalanx (Fig. 4). In 40 fingers the metacarpophalangeal joint was dislocated, but in none of these was there the advanced resorption with tapering of the metacarpal shaft or the egg-cup deformity of the proximal phalanx. Metacarpophalangeal dislocations were often associated with swan neck deformities of the fingers. In only one finger was the metacarpophalangeal joint ankylosed. Resorption was present at the proximal interphalangeal joint of 42 fingers. In serial roentgenographs a pattern of degeneration could be followed,
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Fig. 1. A 45-year-old woman with rheumatoid arthritis and opera-glass hands (main en lorgnette): (A) telescoping digits; (B) extensive articular resorption.
Fig. 2. A 67-year-old woman with rheumatoid arthritis and isolated digital shortening (doigt en lorgnette).
beginning with narrowing of the joint space proceeding to a "tongue-in-groove" configuration with retention of some lateral stability (Fig. 5, A). If further resorption occurred, it was in the following order: (1) loss of one or both of the condyles of the proximal phalanx with resultant instability (Fig. 5, B and C); (2) tapering of the remaining stump of the proximal phalanx (Fig . 5, D); (3) tapering of the remaining stump of the middle phalanx (Fig. 5, E). In 18 digits the proximal interphalangeal joint was ankylosed. When ankylosis developed, joint resorption and diaphyseal remodeling ceased. The medullary canals of the phalanges remained broad and digital length was preserved (Fig. 6, A and B). At the distal interp~alangeal joint six fingers were ankylosed. In only one joint had resorption of the condyles of the middle phalanx occurred, and this was the only finger where digital shortening was a result of disease of the distal joint (Fig. 7).
Thumb. Resorption was present at the carpometacarpal joint in 16 thumbs. In all 16 there was loss of the ulnar aspect of the base of the metacarpal, and in 11 resorption of the trapezium and scaphoid had occurred, leaving a free-floating thumb with collapse of the first metacarpal into adduction (Fig. 8, B) . At the metacarpophalangeal joint in II thumbs the proximal phalanx had dislocated volarward (Figure 8, A). In six others there was resorption of the condyles of the metacarpal with early egg-cupping of the base of the proximal phalanx. One was ankylosed. In 14 thumbs the interphalangeal joint had evidence of resorption of the condyles of the proximal phalanx with tapering of the remaining stump and an advanced egg-cup deformity of the distal phalanx (Figure 8, C). All of these thumbs lacked adequate stability for pinch. Wrist. The intercarpal joints of ten wrists showed ankylosis between varying numbers of carpal bones (Fig. 9, A). At the radiocarpal joint resorption of the
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Fig. 3. Changes of the carpometacarpal joints of the fingers: (A) ankylosis of trapezoid and captitate to the second and third metacarpals; (8) massive resorption of the proximal and distal carpal bones with the metacarpals articulating with the radius.
Fig. 4. Metacarpophalangeal joints of the fingers. "Eggcupping" of the middle, ring, and little finger joints. Minimal resorptive changes of the index finger metacarpophalangeal joint, which is dislocated.
distal radius and proximal carpal row was present in II wrists. Complete resorption of all the carpal bones, leaving the radius to articulate with the base of the metacarpals, was present in eight wrists (Fig. 9, B). In five wrists there was radiocarpal ankylosis and in one a dislocation had occurred. At the radioulnar joint there was resorption of the distal ulna in 13 wrists and there was ankylosis in one (Fig. 9, B). Although ankylosis blocked forearm rotation, resorption acted as a resection arthroplasty, allowing free and painless motion. Other joints. Advanced resorption of the radius, ulna, and humerus, giving a "knife-and-fork" deformity, was present in six elbows (Fig. 10, A). Resorption of the head of the humerus occurred twice; resorption
of the acromial end of the clavicle occurred four times (Fig. 10, B). Rib notching was present in four patients (Fig. 10, C), protrusio acetabuli in three, resorption of the femoral condyles in two, and resorption of the talus in two (Fig. 10, D, E. and F.) Changes in the toes similar to those in the fingers were present in six patients (Fig. 10, G). Comment. A review of the published reports of 24 cases of opera-glass deformities in the hand found the same characteristic deformities in the joints of the fingers, thumb, and wrist.l-1o. 13. 16. 17. 19. 22·27 Degeneration had occurred primarily in the metacarpophalangeal and interphalangeal joints. At sites where there was relatively little motion and close coaptation of joint surfaces, such as in the intercarpal and carpometacarpal joints and in the proximal interphalangeal joints with the tongue-in-groove deformity, ankylosis often occurred. When ankylosis occurred, resorption and tapering of the diaphyses ceased. When spontaneous ankylosis occurred, individual digital length was preserved while adjacent digits underwent progressive resorption and shortening. Resorption was more common at condylar than at ginglymus joints and seemed to be the result of motion and wear as well as of synovitis. 16 • 22 Egg-cup and tongue-in-groove deformities probably developed from wear as they were not seen with dislocation. The tapering of diaphyses occurred outside of the synovial cavities and has been attributed to periosteal remodeling. 13. 16 The pace of degeneration was slow, with changes occurring over a 10 to 20 year period, although patients with rapid changes have been reported. 9 Eventually, except for the distal phalanges which typically are spared, only remnants of the diaphyses remain. 16
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Fig. 5. Proximal interphalangeal joint of the fingers : (A) "tongue-in-groove" configuration; (8) loss of one condyle of the proximal phalanx; (C) loss of both condyles; (D) tapering of the proximal phalanx; (£) tapering of the base of the middle phalanx.
Treatment Seventy-six operative procedures were performed in 12 patients; 52 in the fingers, 18 in the thumb, and six in the wrist. The follow-up period ranged from 9 months to 13 years, with a mean of 41/2 years. Fingers. In II patie'nts operations were performed primarily to correct deformities in the index and middle fingers to improve pinch. Seven resection arthroplasties were performed at the metacarpophalangeal joint for dislocation prior to the advent of silicone rubber prostheses. Continued resorption and shortening was demonstrated in serial roentgenographs (Fig. II , A and B). The result was instabil-
ity in all cases. Metacarpophalangeal joint prostheses were inserted into 21 fingers, 16 with dislocation and two with resorption without the egg-cup deformity (Fig. 12, A and B). Some mobility was retained while maintaining length and stability. No additional resorption was noted on serial roentgenographs for periods up to 3 years. Synovectomy was performed in two fingers which later proceeded to dislocation, but there was no evidence of further resorption after 5 years . At the proximal interphalangeal joint, arthrodesis was performed in 14 fingers. Interposition grafts were used to restore length and to enhance fusion (Fig. 13, A and B) . One graft underwent resorption and nonunion
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Fig. 6. A, Spontaneous ankylosis of the proximal interphalangeal joint with preservation of digital length. B, Resultant disparity in digital length.
Fig. 7. Distal interphalangeal joint with resorptive changes limited to the condyles of the middle phalanx.
developed. Swanson prostheses were inserted into three proximal interphalangeal joints. One became septic and was removed. In the other two, no further resorption or shortening occurred, but these have been followed for only 14 months. At the distal interphalangeal joint arthrodesis was performed five times. More recently attempts have been made to prevent digital shortening. In one patient proximal interphalangeal arthrodeses were done because of a tongue-in-groove deformity and roentgenographic evidence of rapidly progressive resorption in adjacent joints (Fig. 14, A and B). No further evidence of resorption or foreshortening of the arthrodesed fingers was present after 2 years. Thumb. Eight patients had operations on the thumb because of marked shortening and instability which interfered with pinch. Metacarpophalangeal and interphalangeal joint fusion each were performed on nine occasions, seven times together. Because of marked loss of bony substance, interposition grafts were required to restore length in six cases of interphalangeal fusion (Fig. 15, A, B, C, and D). There was one painless nonunion after an attempted metacarpophalangeal fusion. Pinch between the tip of the thumb and the side of the index finger was restored in each case. Wrist. Arthrodesis of the wrist was performed six times for marked instability with resorption of the radiocarpal joint. In four wrists there had been total resorption of the carpal bones; the thumb, index, and middle finger metacarpals were articulating with the
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Fig. 8. Thumb: (A) metacarpophalangeal joint with vol ar dislocation; (B) carpometacarpal joint with resorption of the ulnar bea k and collapse of the first m etacarpal into adduction. (Resorption left only a wafer of trapezium between the radius and the thumb metacarpal.); (C) interphalangeal joint with " egg-cup " deformity.
distal radius (Fig. 16, A and B) . One resulted in painless nonunion. Discussion The disability from .the opera-glass deformity is the result of a combination of at least four factors, as follows: (I) unequal shortening of adjacent digits , especially of the thumb and index finger; (2) shortening of all of the digits of the hand with diminution of the
arc of grasp; (3) instability; and (4) angular deformity. In the past the aim of treatment has been to restore length , stability, and alignment. With shortening of all of the digits, we have chosen to focus attention on salvaging pinch between the thumb and the index and middle fingers. In the thumb, metacarpophalangeal and/or interphalangeal fusion restores stability. The carpometacarpal joint is left mobile . In the fingers interphalangeal joint fusion is used to re-establish length
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Fig. 9. Wrist: (A) ankylosis of carpometacarpal, intercarpal, and radiocarpal joints; (8) massive carpal resorption, resorption of the distal radioulnar joint.
Fig. 10. Associated findings : (A) "knife and fork" deformity of the elbow; (8) resorption of the glenohumeral and acromioclavicular joints; (C) toe deformities similar to those in the hands.
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Fig. 11. A. Metacarpophalangeal resection arthroplasty for dislocation. B. Progressive resorption with shortening and instability.
Fig. 12. A. Metacarpophalangeal dislocation and resorption before silicone rubber prostheses. B. Follow-up films revealing no further foreshortening or resorption in the operated middle, ring, and little finger joints, but further resorption and foreshortening in the unoperated index finger.
Fig. 13. Interphalangeal fusions of the thumb, index, and middle fingers to restore pinch.
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Fig. 14. Prophylactic arthrodesis of proximal interphalangeal joints with tongue-in-groove configuration and rapidly progressing resorptive changes of the subjacent metacarpophalangeal joints .
Fig. 15. Thumb reconstruction: (A) foreshortened, unstable thumb; (B) x-rays revealing egg-cup deformity of the proximal interphalangeal joint; (C) interphalangeal arthrodesis with interposition graft; (D) restoration of pinch between the tip of the thumb and side of the index finger .
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Fig. 16. Wrist stabilization: (A) massive carpal resorption; (B) arthrodesis with stabilization with an intramedullary Steinman pin placed down the shaft of the third metacarpal.
and stability, albeit at the expense of motion. An interposition graft usually is required to restore length and to secure fusion. 6 Motion is maintained at the metacarpophalangeal joint. Swanson prostheses are used for dislocation or severe resorption with tapering of the adjacent ends of the metacarpal and proximal phalanx. The metacarpophalangeal egg-cup deformity usually is painless and often sufficiently stable to work at least temporarily as a resection arthroplasty. In these patients surgery may not be needed. At the wrist, arthrodesis may be required to prevent resorption of the carpals and loss of stability between the bases of the digits. The technique of fusion should incorporate internal fixation. We prefer an intramedullary rod passed down the medullary canal of the third metacarpal into the distal radius. 28, 29 The aim of treatment of the opera-glass hand should be to preserve length. Emphasis should be placed upon early treatment to prevent shortening rather than operations' done late to salvage already shortened digits. Since spontaneous fusion of the proximal interphalangeal joint preserves length, we recommend operative fusion where resorption, instability, and shortening seem imminent. An example would be where an interphalangeal joint with a tongue-in-groove deformity is bordered by digits with rapidly progressive resorptive changes. There is insufficient data to predict whether early synovectomy can forestall or prevent gross resorption of the joint. REFERENCES I. Marie, P., and Leri, A.: Une vari6t6 rare de rhumatisme chronique; la main en lorgnette, Bull. Mem. Soc. M6d. Hop. Paris, 36: 104, 1913.
2. Silver, W., and Steinbrocker, 0.: Resorptive osteopathy in inflammatory arthritis ("absorptive arthritis, operaglass hand"), Bull. Hosp. Joint Dis. 14: 211,1954. 3. Swezey, R. L., Bjarnason, N., and Austin, E. S.: Nerve conduction studies in resorptive arthropathies: Operaglass hand, J. Bone Joint Surg. 55A: 1680, 1973. 4. Adrian, c.: Ueber Arthropathia Psoriatica, Mitt. Grenzgeb. Med. Chir. 11: 237, 1903. 5. Clarke, 0.: Arthritis mutilans associated with psoriasis, Lancet 1: 249, 1950. 6. Froimson, A. I.: Hand reconstruction in arthritis mutilans, J. Bone Joint Surg. 53A: 1377, 1971. 7. Nelson, L. S.: The opera-glass hand in chronic arthntis. "La main en lorgnette" of Marie and Uri, J. Bone Joint Surg. 20: 1045, 1938. 8. Shlionsky, H., and Blake, F. G.: Arthritis psoriatica: Report of a case, Ann. Intern. Med. 10: 537, 1936. 9. Solomon, W. M., and Stecher, R. M.: Chronic absorptive arthritis or opera-glass hand: Report of eight cases, Ann. Rheum. Dis. 9: 209, 1950. 10. Williams, L. E., Bland, J. H., and Lipson, R. L.: Cervical spine sublucations and massive osteolysis in the upper extremities in rheumatoid arthritis, Arthritis Rheum. 9: 348, 1960. 11. Albert, J., Bruce, W., Allen, A. c., et al.: Lipoid dermatoarthri tis, Am. J. Med. 28: 661, 1960. 12. Brumpt, M. E.: Doigts en lorgnette aucours d'une atrophic musculaire progressive chez un negre du soudan, Rev. Neurol. 14: 477, 1906. 13. Eisenstadt, H. G., and Eggers, G. W. N.: Arthritis mutilans (doigt, main, pied en lorgnette), J. Bone Joint Surg. 37A: 337, 1955. 14. Alpert, M., and Feldman, F.: The rib lesions of rheumatoid arthritis, Radiology 82: 872, 1964. 15. Alpert, M., and Meyers, M.: Osteolysis of the acromial end of the clavicles in rheumatoid arthritis, Am. J. Roentgenol. Radium Ther. Nucl. Med. 86: 251,1961.
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16. Cohen, H.: Discussion on skeletal changes in metabolic and endocrine disorders, Proc. R. Soc. Med. 31: 1391, 1938. 17. Holroyd, G. T.: Two cases of arthritis mutilans, Br. J. Radiol. 24: 466, 1951. 18 . Kartagener, M.: "Le pied en lorgnette" bei chronischer polyarthritis, Schweiz. Med . Wochenschr. 17: 479, 1936 . 19. Mather, H. G.: Unusual rheumatoid arthritis (arthritis mutilans) , Proc. R. Soc. Med . 47: 457, 1954. 20. Stursberg , H. : Ueber verstummelnde Gelenkentzundung, Dtsch. Med. Wochenschr. 61: 5, 1935. 21. Werthemann, A.: Pied en lorgnette. Arthritis mutilans, Schweiz. Med. Wochenschr. 75: 749, 1945. 22. Nielsen, B., and Snorrason, E.: Arthritis mutilans ("main et doigt en lorgnette"), Acta Radiol. 27: 607, 1946. 23 . Crain, D. c.: Opera-glass hand (Ia main en lorgnette), Med . Ann. D. C. 10: 293 , 1941 .
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24 . Rogers, F . B., and Lansbury , 1.: Atrophy of auricular and nasal cartilages following administration of chorionic gonadotrophins in a case of arthritis mutilans with the Sicca syndrome, Am. J. Med. Sci. 229: 55, 1955. 25. Schuller, J.: Uber die sog. Arthritis mutilans, Munch. Med. Wocheschr. 84: 1381 , 1937. 26. Weigeldt, W. : La main en lorgnette, Munch . Med. Wochenschr. 76: 1270, 1929. 27 . Zellner, E.: Arthropathia psoriatica und Arthritis bei Psoriatikern, Wien. Arch . Inn . Med. 15: 435, 1928. 28 . Mannerfelt, L., and Malmsten , M.: Arthrodesis of the wrist in rheumatoid arthritis. A technique without external fixation, Scand. J. Plast. Reconstr. Surg. 5: 124, 1971. 29. Millender, L. H., and Nalebuff, E. A.: Arthrodesis of th e rheumatoid wrist, J. Bone Joint Surg. 55A: 1026, 1973.
Edward A. Nalebuff, M.D., Brookline, Mass., and John Garrett, M.D., Boston, Mass.
The term main en lorgnette (opera-glass hand) was used initially by Marie and LerjI to describe the deformities of a patient with chronic polyarthritis. The digits were short with excessive skin folds but could be elongated with traction (Fig. 1, A and B). Postmortem studies disclosed resorption of the articular ends of the bones but sparing of the vessels, nerves, and tendons. More recent studies have shown synovial and erosive bony changes consistent with rheumatoid arthritis. 2 Nerve conduction studies have been reported as normal, separating the entity from Charcot neuropathies. 3 The deformity represents an advanced stage of rheumatoid arthritis or one of its variants and often is associated with psoriasis. 4' IO It has been reported to occur with lipoid dermatoarthritis. 11 Doigt en lorgnette (opera-glass finger) describes the disorder in individual digits (Fig. 2).9, 12, 13 Generalized resorptive changes typically referred to as arthritis mutilans may be seen around the wrist and elbow joints, the glenohumeral and acromioclavicular joints, and the ribs, ankle, and toes.5, 9, 14·21 Over 40 cases of opera-glass hand associated with rheumatoid arthritis or psoriasis have been reported. H , 6'10, 12, 13, 16, 17, 19, 22·27 Although loss of digital length and stability can be severely disabling, only one publication deals with reconstructive surgery.6 No mention has been made of operative procedures to prevent the shortening. We have analyzed Received for publication May 11, 1976. Revised for publication Aug. 14, 1976. Reprint requests: Edward A. Nalebuff, M.D., 209 Harvard St., Brookline, Mass. 02140.
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the deformities at individual joints in 13 patients and have outlined the treatment.
Materials and methods Thirteen patients with the deformity of opera-glass hand from the Robert Breck Brigham, Beth Israel, and Massachusetts General Hospitals, Boston, were studied. All of the patients had rheumatoid arthritis; four also had psoriasis. Twelve of the 13 patients were women. The patients' ages ranged from 33 to 72 years, with a mean of 49 years. Arthritis had been present from 8 to 47 years, with a mean of 22 years.
Deformities Fingers. At the carpometacarpal joint, ankylosis was
present in 22 fingers, most often in the index and middle fingers (Fig. 3, A). In 18 hands complete resorption of the carpal bones had taken place, with the metacarpals articulating with the-radius (Fig. 3, B). At the metacarpophalangeal joint level, 29 fingers showed resorption of the metacarpal head, tapering of the metacarpal stump, and an "egg-cup" deformity of the base of the proximal phalanx (Fig. 4). In 40 fingers the metacarpophalangeal joint was dislocated, but in none of these was there the advanced resorption with tapering of the metacarpal shaft or the egg-cup deformity of the proximal phalanx. Metacarpophalangeal dislocations were often associated with swan neck deformities of the fingers. In only one finger was the metacarpophalangeal joint ankylosed. Resorption was present at the proximal interphalangeal joint of 42 fingers. In serial roentgenographs a pattern of degeneration could be followed,
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Fig. 1. A 45-year-old woman with rheumatoid arthritis and opera-glass hands (main en lorgnette): (A) telescoping digits; (B) extensive articular resorption.
Fig. 2. A 67-year-old woman with rheumatoid arthritis and isolated digital shortening (doigt en lorgnette).
beginning with narrowing of the joint space proceeding to a "tongue-in-groove" configuration with retention of some lateral stability (Fig. 5, A). If further resorption occurred, it was in the following order: (1) loss of one or both of the condyles of the proximal phalanx with resultant instability (Fig. 5, B and C); (2) tapering of the remaining stump of the proximal phalanx (Fig . 5, D); (3) tapering of the remaining stump of the middle phalanx (Fig. 5, E). In 18 digits the proximal interphalangeal joint was ankylosed. When ankylosis developed, joint resorption and diaphyseal remodeling ceased. The medullary canals of the phalanges remained broad and digital length was preserved (Fig. 6, A and B). At the distal interp~alangeal joint six fingers were ankylosed. In only one joint had resorption of the condyles of the middle phalanx occurred, and this was the only finger where digital shortening was a result of disease of the distal joint (Fig. 7).
Thumb. Resorption was present at the carpometacarpal joint in 16 thumbs. In all 16 there was loss of the ulnar aspect of the base of the metacarpal, and in 11 resorption of the trapezium and scaphoid had occurred, leaving a free-floating thumb with collapse of the first metacarpal into adduction (Fig. 8, B) . At the metacarpophalangeal joint in II thumbs the proximal phalanx had dislocated volarward (Figure 8, A). In six others there was resorption of the condyles of the metacarpal with early egg-cupping of the base of the proximal phalanx. One was ankylosed. In 14 thumbs the interphalangeal joint had evidence of resorption of the condyles of the proximal phalanx with tapering of the remaining stump and an advanced egg-cup deformity of the distal phalanx (Figure 8, C). All of these thumbs lacked adequate stability for pinch. Wrist. The intercarpal joints of ten wrists showed ankylosis between varying numbers of carpal bones (Fig. 9, A). At the radiocarpal joint resorption of the
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Fig. 3. Changes of the carpometacarpal joints of the fingers: (A) ankylosis of trapezoid and captitate to the second and third metacarpals; (8) massive resorption of the proximal and distal carpal bones with the metacarpals articulating with the radius.
Fig. 4. Metacarpophalangeal joints of the fingers. "Eggcupping" of the middle, ring, and little finger joints. Minimal resorptive changes of the index finger metacarpophalangeal joint, which is dislocated.
distal radius and proximal carpal row was present in II wrists. Complete resorption of all the carpal bones, leaving the radius to articulate with the base of the metacarpals, was present in eight wrists (Fig. 9, B). In five wrists there was radiocarpal ankylosis and in one a dislocation had occurred. At the radioulnar joint there was resorption of the distal ulna in 13 wrists and there was ankylosis in one (Fig. 9, B). Although ankylosis blocked forearm rotation, resorption acted as a resection arthroplasty, allowing free and painless motion. Other joints. Advanced resorption of the radius, ulna, and humerus, giving a "knife-and-fork" deformity, was present in six elbows (Fig. 10, A). Resorption of the head of the humerus occurred twice; resorption
of the acromial end of the clavicle occurred four times (Fig. 10, B). Rib notching was present in four patients (Fig. 10, C), protrusio acetabuli in three, resorption of the femoral condyles in two, and resorption of the talus in two (Fig. 10, D, E. and F.) Changes in the toes similar to those in the fingers were present in six patients (Fig. 10, G). Comment. A review of the published reports of 24 cases of opera-glass deformities in the hand found the same characteristic deformities in the joints of the fingers, thumb, and wrist.l-1o. 13. 16. 17. 19. 22·27 Degeneration had occurred primarily in the metacarpophalangeal and interphalangeal joints. At sites where there was relatively little motion and close coaptation of joint surfaces, such as in the intercarpal and carpometacarpal joints and in the proximal interphalangeal joints with the tongue-in-groove deformity, ankylosis often occurred. When ankylosis occurred, resorption and tapering of the diaphyses ceased. When spontaneous ankylosis occurred, individual digital length was preserved while adjacent digits underwent progressive resorption and shortening. Resorption was more common at condylar than at ginglymus joints and seemed to be the result of motion and wear as well as of synovitis. 16 • 22 Egg-cup and tongue-in-groove deformities probably developed from wear as they were not seen with dislocation. The tapering of diaphyses occurred outside of the synovial cavities and has been attributed to periosteal remodeling. 13. 16 The pace of degeneration was slow, with changes occurring over a 10 to 20 year period, although patients with rapid changes have been reported. 9 Eventually, except for the distal phalanges which typically are spared, only remnants of the diaphyses remain. 16
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Fig. 5. Proximal interphalangeal joint of the fingers : (A) "tongue-in-groove" configuration; (8) loss of one condyle of the proximal phalanx; (C) loss of both condyles; (D) tapering of the proximal phalanx; (£) tapering of the base of the middle phalanx.
Treatment Seventy-six operative procedures were performed in 12 patients; 52 in the fingers, 18 in the thumb, and six in the wrist. The follow-up period ranged from 9 months to 13 years, with a mean of 41/2 years. Fingers. In II patie'nts operations were performed primarily to correct deformities in the index and middle fingers to improve pinch. Seven resection arthroplasties were performed at the metacarpophalangeal joint for dislocation prior to the advent of silicone rubber prostheses. Continued resorption and shortening was demonstrated in serial roentgenographs (Fig. II , A and B). The result was instabil-
ity in all cases. Metacarpophalangeal joint prostheses were inserted into 21 fingers, 16 with dislocation and two with resorption without the egg-cup deformity (Fig. 12, A and B). Some mobility was retained while maintaining length and stability. No additional resorption was noted on serial roentgenographs for periods up to 3 years. Synovectomy was performed in two fingers which later proceeded to dislocation, but there was no evidence of further resorption after 5 years . At the proximal interphalangeal joint, arthrodesis was performed in 14 fingers. Interposition grafts were used to restore length and to enhance fusion (Fig. 13, A and B) . One graft underwent resorption and nonunion
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Fig. 6. A, Spontaneous ankylosis of the proximal interphalangeal joint with preservation of digital length. B, Resultant disparity in digital length.
Fig. 7. Distal interphalangeal joint with resorptive changes limited to the condyles of the middle phalanx.
developed. Swanson prostheses were inserted into three proximal interphalangeal joints. One became septic and was removed. In the other two, no further resorption or shortening occurred, but these have been followed for only 14 months. At the distal interphalangeal joint arthrodesis was performed five times. More recently attempts have been made to prevent digital shortening. In one patient proximal interphalangeal arthrodeses were done because of a tongue-in-groove deformity and roentgenographic evidence of rapidly progressive resorption in adjacent joints (Fig. 14, A and B). No further evidence of resorption or foreshortening of the arthrodesed fingers was present after 2 years. Thumb. Eight patients had operations on the thumb because of marked shortening and instability which interfered with pinch. Metacarpophalangeal and interphalangeal joint fusion each were performed on nine occasions, seven times together. Because of marked loss of bony substance, interposition grafts were required to restore length in six cases of interphalangeal fusion (Fig. 15, A, B, C, and D). There was one painless nonunion after an attempted metacarpophalangeal fusion. Pinch between the tip of the thumb and the side of the index finger was restored in each case. Wrist. Arthrodesis of the wrist was performed six times for marked instability with resorption of the radiocarpal joint. In four wrists there had been total resorption of the carpal bones; the thumb, index, and middle finger metacarpals were articulating with the
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Fig. 8. Thumb: (A) metacarpophalangeal joint with vol ar dislocation; (B) carpometacarpal joint with resorption of the ulnar bea k and collapse of the first m etacarpal into adduction. (Resorption left only a wafer of trapezium between the radius and the thumb metacarpal.); (C) interphalangeal joint with " egg-cup " deformity.
distal radius (Fig. 16, A and B) . One resulted in painless nonunion. Discussion The disability from .the opera-glass deformity is the result of a combination of at least four factors, as follows: (I) unequal shortening of adjacent digits , especially of the thumb and index finger; (2) shortening of all of the digits of the hand with diminution of the
arc of grasp; (3) instability; and (4) angular deformity. In the past the aim of treatment has been to restore length , stability, and alignment. With shortening of all of the digits, we have chosen to focus attention on salvaging pinch between the thumb and the index and middle fingers. In the thumb, metacarpophalangeal and/or interphalangeal fusion restores stability. The carpometacarpal joint is left mobile . In the fingers interphalangeal joint fusion is used to re-establish length
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Fig. 9. Wrist: (A) ankylosis of carpometacarpal, intercarpal, and radiocarpal joints; (8) massive carpal resorption, resorption of the distal radioulnar joint.
Fig. 10. Associated findings : (A) "knife and fork" deformity of the elbow; (8) resorption of the glenohumeral and acromioclavicular joints; (C) toe deformities similar to those in the hands.
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Fig. 11. A. Metacarpophalangeal resection arthroplasty for dislocation. B. Progressive resorption with shortening and instability.
Fig. 12. A. Metacarpophalangeal dislocation and resorption before silicone rubber prostheses. B. Follow-up films revealing no further foreshortening or resorption in the operated middle, ring, and little finger joints, but further resorption and foreshortening in the unoperated index finger.
Fig. 13. Interphalangeal fusions of the thumb, index, and middle fingers to restore pinch.
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Fig. 14. Prophylactic arthrodesis of proximal interphalangeal joints with tongue-in-groove configuration and rapidly progressing resorptive changes of the subjacent metacarpophalangeal joints .
Fig. 15. Thumb reconstruction: (A) foreshortened, unstable thumb; (B) x-rays revealing egg-cup deformity of the proximal interphalangeal joint; (C) interphalangeal arthrodesis with interposition graft; (D) restoration of pinch between the tip of the thumb and side of the index finger .
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Fig. 16. Wrist stabilization: (A) massive carpal resorption; (B) arthrodesis with stabilization with an intramedullary Steinman pin placed down the shaft of the third metacarpal.
and stability, albeit at the expense of motion. An interposition graft usually is required to restore length and to secure fusion. 6 Motion is maintained at the metacarpophalangeal joint. Swanson prostheses are used for dislocation or severe resorption with tapering of the adjacent ends of the metacarpal and proximal phalanx. The metacarpophalangeal egg-cup deformity usually is painless and often sufficiently stable to work at least temporarily as a resection arthroplasty. In these patients surgery may not be needed. At the wrist, arthrodesis may be required to prevent resorption of the carpals and loss of stability between the bases of the digits. The technique of fusion should incorporate internal fixation. We prefer an intramedullary rod passed down the medullary canal of the third metacarpal into the distal radius. 28, 29 The aim of treatment of the opera-glass hand should be to preserve length. Emphasis should be placed upon early treatment to prevent shortening rather than operations' done late to salvage already shortened digits. Since spontaneous fusion of the proximal interphalangeal joint preserves length, we recommend operative fusion where resorption, instability, and shortening seem imminent. An example would be where an interphalangeal joint with a tongue-in-groove deformity is bordered by digits with rapidly progressive resorptive changes. There is insufficient data to predict whether early synovectomy can forestall or prevent gross resorption of the joint. REFERENCES I. Marie, P., and Leri, A.: Une vari6t6 rare de rhumatisme chronique; la main en lorgnette, Bull. Mem. Soc. M6d. Hop. Paris, 36: 104, 1913.
2. Silver, W., and Steinbrocker, 0.: Resorptive osteopathy in inflammatory arthritis ("absorptive arthritis, operaglass hand"), Bull. Hosp. Joint Dis. 14: 211,1954. 3. Swezey, R. L., Bjarnason, N., and Austin, E. S.: Nerve conduction studies in resorptive arthropathies: Operaglass hand, J. Bone Joint Surg. 55A: 1680, 1973. 4. Adrian, c.: Ueber Arthropathia Psoriatica, Mitt. Grenzgeb. Med. Chir. 11: 237, 1903. 5. Clarke, 0.: Arthritis mutilans associated with psoriasis, Lancet 1: 249, 1950. 6. Froimson, A. I.: Hand reconstruction in arthritis mutilans, J. Bone Joint Surg. 53A: 1377, 1971. 7. Nelson, L. S.: The opera-glass hand in chronic arthntis. "La main en lorgnette" of Marie and Uri, J. Bone Joint Surg. 20: 1045, 1938. 8. Shlionsky, H., and Blake, F. G.: Arthritis psoriatica: Report of a case, Ann. Intern. Med. 10: 537, 1936. 9. Solomon, W. M., and Stecher, R. M.: Chronic absorptive arthritis or opera-glass hand: Report of eight cases, Ann. Rheum. Dis. 9: 209, 1950. 10. Williams, L. E., Bland, J. H., and Lipson, R. L.: Cervical spine sublucations and massive osteolysis in the upper extremities in rheumatoid arthritis, Arthritis Rheum. 9: 348, 1960. 11. Albert, J., Bruce, W., Allen, A. c., et al.: Lipoid dermatoarthri tis, Am. J. Med. 28: 661, 1960. 12. Brumpt, M. E.: Doigts en lorgnette aucours d'une atrophic musculaire progressive chez un negre du soudan, Rev. Neurol. 14: 477, 1906. 13. Eisenstadt, H. G., and Eggers, G. W. N.: Arthritis mutilans (doigt, main, pied en lorgnette), J. Bone Joint Surg. 37A: 337, 1955. 14. Alpert, M., and Feldman, F.: The rib lesions of rheumatoid arthritis, Radiology 82: 872, 1964. 15. Alpert, M., and Meyers, M.: Osteolysis of the acromial end of the clavicles in rheumatoid arthritis, Am. J. Roentgenol. Radium Ther. Nucl. Med. 86: 251,1961.
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16. Cohen, H.: Discussion on skeletal changes in metabolic and endocrine disorders, Proc. R. Soc. Med. 31: 1391, 1938. 17. Holroyd, G. T.: Two cases of arthritis mutilans, Br. J. Radiol. 24: 466, 1951. 18 . Kartagener, M.: "Le pied en lorgnette" bei chronischer polyarthritis, Schweiz. Med . Wochenschr. 17: 479, 1936 . 19. Mather, H. G.: Unusual rheumatoid arthritis (arthritis mutilans) , Proc. R. Soc. Med . 47: 457, 1954. 20. Stursberg , H. : Ueber verstummelnde Gelenkentzundung, Dtsch. Med. Wochenschr. 61: 5, 1935. 21. Werthemann, A.: Pied en lorgnette. Arthritis mutilans, Schweiz. Med. Wochenschr. 75: 749, 1945. 22. Nielsen, B., and Snorrason, E.: Arthritis mutilans ("main et doigt en lorgnette"), Acta Radiol. 27: 607, 1946. 23 . Crain, D. c.: Opera-glass hand (Ia main en lorgnette), Med . Ann. D. C. 10: 293 , 1941 .
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24 . Rogers, F . B., and Lansbury , 1.: Atrophy of auricular and nasal cartilages following administration of chorionic gonadotrophins in a case of arthritis mutilans with the Sicca syndrome, Am. J. Med. Sci. 229: 55, 1955. 25. Schuller, J.: Uber die sog. Arthritis mutilans, Munch. Med. Wocheschr. 84: 1381 , 1937. 26. Weigeldt, W. : La main en lorgnette, Munch . Med. Wochenschr. 76: 1270, 1929. 27 . Zellner, E.: Arthropathia psoriatica und Arthritis bei Psoriatikern, Wien. Arch . Inn . Med. 15: 435, 1928. 28 . Mannerfelt, L., and Malmsten , M.: Arthrodesis of the wrist in rheumatoid arthritis. A technique without external fixation, Scand. J. Plast. Reconstr. Surg. 5: 124, 1971. 29. Millender, L. H., and Nalebuff, E. A.: Arthrodesis of th e rheumatoid wrist, J. Bone Joint Surg. 55A: 1026, 1973.