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The hand in the child with juvenile rheumatoid arthritis
ORIGINAL COMMUNICATIONS
The hand in the child with juvenile rheumatoid arthritis Clinical examination of 100 children showed frequent loss of wrist extension and ulnar deviation. Loss of flexion and radial deviation in the metacarpophalangeal joint is more fr equent than in the adult. Statistical review of charts and roentgenograms of 200 patients showed all had ulnar shortening up to 9 mm but there was no correlation with ulnar deviation or metacarpophalangeal radial deviation. Conservative treatment is reviewed; surgery is rarely indicated.
W. Malcolm Granberry , M.D., and Gary L. Mangum, M.D., Houston, Texas
T he hand surgeon is certainly aware of deformities
and problems in the hand of the adult with rheumatoid arthritis . There is , however, a relative paucity of information concerning the hand in the child with rheumatoid arthritis.1-4 I have been the consultant to the JRA Clinic at Texas Children 's Hospital for 14 years and have had the opportunity to see and treat many children with this condition . Observations and experience concerning the hand in the child with juvenile rheumatoid arthritis (JRA) will be given. Some of the problems are opposite to that seen in the adult. Recently pediatric rheumatologists have separated JRA into three basic onset types . The first one, the classic description given by Still,5 does not fulfill today's complete concept of JRA and is now termed "systemic onset type JRA . " The second onset type is described as "polyarticular" and closely resembles the disease seen in the adult. A third separation, "pauciarticular" onset JRA, is characterized by involvement by four joints or less . As a group, children with JRA have a much milder course and are left with less disability than adults. Between 50% and 70% of these children will go into remission. 7 But the child with serious JRA has more stiffness, loss of motion , and ankylosis than an adult. In a growing child reconstructive procedures such as those used in adults are not applicable, as they will produce epiphyseal arrest. If a child continues to have active arthritis and passes through puberty, the disease changes and resembles that seen in the adult. Children are not as cooperative as adults and therapy From the Baylor College of Medicine , Department of Orthopaedic Surgery, Houston , Texas . Received for publication March 6 , 1978; revised May 28, 1979. Reprint requests: W. Malcolm Granberry, M.D., 7000 Fannin, 20th Floor, Houston, TX 77030 (713 790-1818). 0363 -5023/80/020105+09$00.90/0
designed to improve motion and increase strength are not well accepted by the child. The surgeon who treats the child with arthritis should be conservative as surgery is rarely indicated.
Review of literature A significant article by Chaplin et al. 3 in 1969 directly correlates with this investigation. Their roentgenographic review of over 400 patients with JRA showed that 59% of the patients had wrist or hand involvement including 34% with ulnar deviation of the metacarpals (wrist) associated with radial deviation of the fingers . They also reported that many patients had shortening of the ulna. They proposed and thought that their data supported the concept that JRA damaged the distal ulnar epiphysis and produced shortening. This, in tum, produced ulnar deviation of the metacarpals and in tum radial deviation at the metacarpophalangeal joints. Boutonniere deformity was relatively common, but swan-neck deformity was infrequent. Flexion deformity of the interphalangeal joints was common , especially in the younger age group. This study was performed on patients with a diagnosis of JRA, but there are no data in the paper concerning age of the patients at the time of observation . The illustrations were , for the most part, of adult patients . A review of the deformities in 500 adult patients by Pulkki 8 showed that 28% of patients had ulnar deviation of the metacarpophalangeal joints. Vainio and Oka 9 examined almost 300 unselected patients with rheumatoid arthritis and found that 23% of the adults had ulnar deviation of the metacarpophalangeal joints, but only 3 .3% of children had the deformity . The cause of ulnar deviation of the metacarpophalangeal joints in the adult long has been the subject of controversy . 10 Muscle imbalance with intrinsic spasm, unilateral laxity of the capsule, destruction of the collateral liga-
© 1980 American Society for Surgery of the Hand
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The Journal of HAND SURGERY
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I can find no suggestion in the literature as to why there is a significant incidence of radial deviation of the metacarpophalangeal joints in children, and the theories explaining ulnar deviation certainly do not explain radial deviation. Sporadic reports of management and surgery in the hand of the child with JRA are found in the literature. Usually conservative treatment, including splinting and exercises, is advocated. 2 , 13-15 Clinical examination of 100 consecutive children with JRA
Fig. 1. A girl, age 11 years, with juvenile rheumatoid arthritis and various degrees of radial deviation of the metacarpophalangeal joints. Table I. Clinical examination of the hand in juvenile rheumatoid arthritis in 100 patients No.
Metacarpophalangeal joint: Radial deviation Ulnar deviation Decreased flexion Decreased extension Volar subluxation Interphalangeal joint: Boutonniere Swan neck Decreased flexion, PIP Decreased extension, PIP DIP involvement Wrist: Decreased extension Decreased flexion Volar subluxation Ulnar deviation
10 I
23 7 2 7 I
27 10
18 55
22 13 10
Legend: PIP, proximal interphalangeal joint; DIP, distal interphalangeal joint.
ments by synovitis, change in the shape of the joint surfaces, pressure, gravity, daily use, dislocation of the extensor tendons, or subluxation of the flexor tendons all have been implicated as a cause of ulnar deviation. Shapiro l l thought that the cause was radial deviation of the wrist, with linkage breakdown of the metacarpophalangeal joints, comparable to the theory proposed by Landsmeer. 12 Hands with wrists fused in radial deviation have been shown to develop ulnar deviation of the metacarpophalangeal joints. 13
To study a reasonable cross-section of problems in the hand of children with JRA, the author examined 100 consecutive children who had proven JRA for at least 2 years. Examinations were done on children in the hospital and on patients in the outpatient clinic. This examination required approximately 9 months and was completed about 4 years ago. Some had mild or inactive disease, others had severe debilitating arthritis. No attempt at that time was made to correlate various deformities with age, type of onset, length of illness, etc. Table I contains the findings of this examination. Ten percent of the children were found to have significant clinical radial deviation of the metacarpophalangeal joints (Fig. 1). This deformity cannot be measured accurately and depends on the position of extension-flexion, how the hand is placed on the table, and the attention of the child in the attempt to correct or exaggerate the deviation. I found only one child with significant ulnar deviation in this group. Twenty-three percent of these children were found to have loss of flexion of the metacarpophalangeal joints (Fig. 2), but only 7% were found to have loss of extension. This is opposite to that seen in the adult who typically has loss of extension and ulnar deviation. I found only two children with volar subluxation of a metacarpophalangeal joint. Boutonniere deformities were seen in seven children, but they were mild and flexible. One child demonstrated a mild swan-neck deformity. Intrinsic tightness was not seen in this group of children. Twenty-seven children had a clinically significant decrease in flexion of the interphalangeal joint which seemed to be the result of proliferative synovium with volume restriction (Fig. 3). Ten children had loss of extension of the interphalangeal joint. This was usually seen in the child with significant articular disease and deformity of the joint. Eighteen children were found to have distal interphalangeal joint involvement, usually with synovitis and mild flexion contracture. Table I also records the findings in examination of
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Fig. 2. A, Loss of flexion of the metacarpophalangeal joint in a young boy with mild disease . B, Postoperative flexion after collateral ligament release .
the wrist. Fifty-five of these children had detectable loss of extension. This varied from 15° of loss of extension to 30° of flexion contracture . Twenty-two of these children had detectable loss of flexion, but it was much milder and had little clinical consequence (Fig . 4) . Thirteen children had subluxation of the wrist similar to that seen in the adult. Ten children had ulnar deviation of the wrist (ulnar metacarpal angulation with respect to the forearm). In this clinical series, ulnar deviation of the wrist was not usually associated with radial deviation of the metacarpophalangeal joints (Fig. 5). I saw no child with clinical radial deviation of the wrist so common in the adult. No child had symptoms or signs of carpal tunnel syndrome.
This examination of 100 children with JRA correlates well with our experience on this service . At present approximately 500 children with all stages and types of JRA are being treated. Others have recorded a significant problem with loss of extension of the interphalangeal joints, 3 but I found a greater percentage of children with loss of flexion and only mild loss of extension. The conclusion from this evaluation is that the hand of the child with JRA shows more radial deviation and loss of flexion of the metacarpophalangeal joint in contrast to the adult who usually has ulnar deviation and loss of extension. Others have noted this problem,9 but thought it was due to a breakdown in the linkage sec-
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Fig. 3. Loss of flexion of the interphalangeal joints due to proliferative synovitis and increased intraarticular pressure . Secondary periarticular fibrosis later in the disease process will produce the same result. Also there is loss of flexion of the metacarpophalangeal joint .
Table II. Vital data in a record review of 200 children Race Caucasian Black Latin-American Indian Type Polyarticular Pauciarticular Systemic
181 10 8
44% 30% 26%
ondary to ulnar deviation of the wrist. Our data do not confirm this observation . One of the earliest clinical signs of rheumatoid arthritis in a child is mild loss of complete extension of the wrist. This may be detectable prior to the onset of any palpable synovitis or other findings of JRA . It also serves as a barometer of the disease and usually precedes roentgenographic changes by several months.
Review of records and roentgenograms of 200 patients The charts and films of 200 children with proven JRA were reviewed. Charts from 1976 and before were reviewed. Children in the clinical series were excluded. Table II shows the vital data of these patients. There were 134 females and 66 males . There were 181 Caucasian patients, ten blacks , eight Latin-Americans and one Indian. Forty-four percent of the patients were of the polyarticular onset type, 34% were pauciarticular, and 26% had systemic onset type of JRA. Fig. 6 shows the age of onset and age grouping. Our
Fig. 4. A, Decreased extension with relatively good flexion of the wrist, a common early finding in juvenile rheumatoid arthritis. B, The same wrist , showing good flexion.
population includes many children with early onset disease, with a range from 3 months to 16 years and a mean of 6 years. Fig. 7 reviews the age of the patients at the time of the record review, the youngest being 2 years and the oldest 19 years, with a mean of 12 years . The duration of the illness is shown in Fig. 8. It is noted that half of the patients had JRA for 4 years or less. This confirms our clinical impression that many children have a disease of short duration. Observation and recording in these charts of the problems under consideration were for the most part made by the rheumatologists, pediatric residents on the service , and physical therapists. Roentgenographic reports and a personal review of the roentgenogram when available completed the observations. Table III records the incidence of involvement gleaned from the records. There was clinical or roentgenographic involvement of the wrist in 24.5% of these patients. The metacarpophalangeal joint and proximal interphalangeal joint had some type of in-
Vol. 5, No.2
Juvenile rheumatoid arthritis
March 1980
volvement in almost half of the patients. The distal interphalangeal joint was noted to be involved in 19% of the patients. There was mention of tenosynovitis in seven patients, and one patient had clinical shortening of the fingers. There was a boutonniere deformity in 3% of the patients, but there was no mention of any swan-neck contractures. There was some type of hand and/ or wrist involvement in 59.5% of the patients. Critical examination of the small joints in the hand are not well-recorded by the others in this service, and no mention of ulnar or radial deviation was seen in these charts. Goniometric measurements of the small joints in the hand usually were not recorded. Analysis of wrist and metacarpophalangeal deformities was carried out on the roentgenograms available in these patients. It is the practice of technicians in the radiology department to simply place both hands on a single plate and take a posterior-anterior exposure. No attempt is made to correct or increase the deformity. Of the 119 patients who had some type of hand and/or wrist involvement, only 93 roentgenograms in 49 patients were available and suitable for measurements. Wrist deviation was measured by the angle between the radius and the second metacarpal and recorded in degrees either ulnar or radial. The metacarpophalangeal deviation was measured between the long metacarpal and proximal phalanx and recorded in degrees as either ulnar or radial. The amount of ulnar shortening was difficult to measure. The children with severe affliction had marked deformity and articular destruction of varying degrees which complicated measurement. A comparable area of the radius and ulna at their adjacent margins was chosen, and shortening in millimeters was recorded. In the immature skeleton the distal portion of the metaphysis was chosen. In the older child the distal line of the epiphyseal ossification was used (Fig. 9). The data from these measurements are summarized in Table IV. Ulnar shortening of some degree was found in all 93 wrists in the 49 patients with a range of 1 to 10 mm, with an average of 4.1 mm. Wrist deviation was seen in almost all the patients, with 79 roentgenograms demonstrating ulnar deviation between 4° and 25°, with an average of 13°. Twelve roentgenograms showed radial deviation between 5° and 30°, with an average of 11 0. Sixty-two roentgenograms in 43 patients demonstrated metacarpal joint deviation. Thirty-one hands demonstrated ulnar deviation between 2° and 25°, with an average of 8°. Thirty-one hands also demonstrated radial deviation between 2° and 15°, with an average of 7.4° essentially equal findings. Correlation of different variables such as age, duration, age of onset, and sex with various measurements
109
Fig. 5. A boy, age 10 years. with ulnar deviation of the wrist not necessarily associated with radial deviation of the metacarpophalangeal joints or shortening of the ulna.
Table III. Hand involvement* %
Wrist Mep PIP DIP
Tenosynovitis Boutonniere Swan neck
24.5 49.5 49.0 19 7 3 0
'There was hand and/or wrist involvement in 59.5% of patients. Legend: MCP, metacarpophalangeal joint; PIP, proximal interphalangeal
joint; DIP, distal interphalangeal joint.
was made using the advanced computers available today. Ulnar shortening was not found to be statistically related to the ulnar deviation of the metacarpal though the P value was low (P = 0.063). Ulnar shortening was certainly not related to radial deviation of the metacarpophalangeal joints (P = 0.204) and ulnar deviation of the wrist was not correlated to radial deviation of the
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The Journal of HAND SURGERY
Granberry and Mangum
-
45
r--
44
40
42
3 Months to 16 Years Average 6.6 Years Mean 6.0
35 30
a::
~ 15 ~
~
~ 20
22
r--
r--
21
21
-
-
15
15
14
10
r--
12
5
t
4
16
14
12
10 8 AGE AT ONSET
6
Fig. 6. The age at onset and age grouping . 40
Range 2 - 19 Years Mean 11.4
;--
38
35
Median 12 .0
30
a:: 25
-
25
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,-
26
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,-
.----
r--
w ~ 20 ::> z 15
24 r-17
-
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AGE
14
12
18
16
Fig. 7. The age of the patients at the time of the record review.
-60
60
55
Ronge 0 .5 - 17 Yeors Mean 4.8 Median 3.5
50 45
40 a::
35
r--
40
r--
34
~ 30
::;: ~ 25
20
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19
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~
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DURATION
10
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Fig. 8. The duration of illness.
n
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18
Vol. 5, No.2 March 1980
Juvenile rheumatoid arthritis
111
Fig. 9. Examples of roentgenograms showing lines for measurement. A, Metacarpophalangeal deviation was measured as the angle of the third metacarpal and proximal phalanx. B, Wrist deviation was measured along the center of the radius and the second metacarpal. fingers. The only correlation seen in these figures was the age of the patient without hand or wrist involvement was significantly lower than those with hand or wrist involvement (P = 0.035). The finding of equal amounts of ulnar and radial deviation of the metacarpophalangeal joints does not correlate with the clinical analysis. Though the x-ray technician is asked to place the hand on the x-ray plate without change in the position of the fingers, it is thought that x-ray positioning must in some way change the clinical deformity, and correlation between clinical and x-ray position is not reasonable. The amount of ulnar shortening, however, is not altered by positioning and is thought to be reliable. Comparison of this study to a similar study by Vainio and Oka9 does not show similar findings. Their studies indicated that significant correlation between ulnar shortening and ulnar deviation of the wrist and subsequent radial deviation of the metacarpophalangeal joint. Their illustrations demonstrated more severe deformities than in this series and appeared to be of adult patients. It is probable that their patient population was
Table IV. Measurements of x-rays of children with hand and/or wrist involvement Measurement
Joints
Ulnar shortening Wrist ulnar deviation Wrist radial deviation MCP ulnar deviation MCP radial deviation
93 79 12 31 31
Range
1-lOmm 4°_25° 5°_30° 2°_25° 2°_15°
Average
4.lmm 13° 11° 8° 7.4°
Legend: Mep, metacarpophalangeal joint.
older and contained hospitalized patients with more severe disease, rather than the broad cross-section represented by our clinic popUlation. Surgical procedures performed on children with JRA between 1965 and 1975 Surgical procedures are rarely indicated at this age group and only a few were performed over a lO-year period in this clinic. Two young boys had significant loss of metacarpophalangeal flexion and underwent collateral ligament release with excellent results (Fig. 2).
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The Journal of HAND SURGERY
Granberry and Mangum
Fig. 10. A simple light splint for management of flexion contracture of the wrist.
Table V. Synovectomy of the small joints in juvenile rheumatoid arthritis-late result: September 1976* % Good
Fair Poor
o 92 8
'Follow-up 12 to 118 months (average of 76.5 months).
One patient had tenolysis of the flexor tendons for triggering on separate occasions, and one patient had a resection of the superficialis for marked proximal interphalangeal contracture. One patient had a deQuervain's release and one patient had a collateral ligament reconstruction for a gamekeeper's thumb deformity. One older patient (16 years) underwent a release of the volar carpal ligament for carpal tunnel syndrome. Tenosynovitis is seen in children, but no patient had sufficient disease to warrant tenosynovectomy. One patient had terminal finger vasospasm with gangrene and required amputations. A prospective study of synovectomy of all joints in our service was conducted between 1965 and 1970 16 and a late follow-up was performed in 1976. 17 Three patients during this period had synovectomy of the wrist, and two patients had synovectomies of 16 metacarpophalangeal joints, and three patients had 10 proximal interphalangeal joint synovectomies. The late results of these operations in the joints were extracted and recorded in Table V. Though no patient has had recurrent synovitis of these joints under observation in
this small group, the loss of motion after synovectomy of these joints was significant and was responsible for the poor results. Synovectomy of the wrist or fingers is not advised at the present. Two patients who had synovectomies of the wrist were found to have dry or "nonproliferative" type of synovitis and had rapidly progressive ankylosis of the intercarpal and radiocarpal joints following the operation. It is thought that this type of synovitis is a distinct contraindication to synovectomy. Common clinical problems and their management As seen in the preceding portions of this paper, the child tends to lose wrist extension, develop ulnar deviation of the wrist, and lose flexion of the metacarpophalangeal and interphalangeal joints. I have found this to be the most common combination of problems in the hand of the child with JRA. The primary function of the surgeon attending the children is to follow them very closely and to detect these early problems prior to their becoming fixed deformities. Intensive physical therapy with exercise and a home program is a cornerstone in management. Splinting and occasional steroid injections are necessary. The tendency for loss of extensi~n of the wrist can easily be managed by a simple three point extension orthosis (Fig. 10). In the neglected or unresponsive cases, wedging casts can be used to bring the wrist into functional position and then maintained with the orthosis. Combination splints to retard ulnar deviation and flexion may be found to be necessary. Heat-malleable plastic materials are useful in this problem. The main thrust of management of the
Vol. 5, No.2 March 1980
wrist in this age group is to allow intercarpal and perhaps radiocarpal fusion to take place while maintaining the wrist in a neutral functional position. Wrist fusion, arthroplasty, or replacement is left for the adult patient and these have no place in the treatment of the child. The use of the Bunnell knuckle-bender splint is quite helpful in maintaining flexion of the metacarpophalangeal joints which have a tendency to lose flexion. The therapist should teach the use of paraffin, passive stretching, and active exercises to maintain motion in these joints. The child should be seen by the therapist in conjunction with the pediatrician and orthopaedist, and it is most important that the parents see and learn the program and continue it at home as long as necessary.
Summary The problems and management of the hand in the child with juvenile rheumatoid arthritis are different from those in the adult. Loss of extension of the wrist combined with ulnar deviation is a common problem and conservative splinting and therapy is indicated. Radial deviation of the metacarpophalangeal joints is more common than ulnar deviation but rarely requires aggressive therapy. Statistical analysis attempting to link ulnar shortening with wrist and finger deformities did not confirm previous observations, and no correlation was seen between ulnar deviation of the wrist and radial deviation of the metacarpophalangeal joint. Surgical procedures are rarely indicated, and conservative treatment with physical therapy, orthotic devices, and, rarely, injections are the mainstays of management. REFERENCES 1. Athreya BH: The hand in juvenile rheumatoid arthritis, in Proceedings of the first ARA conference on the rheu-
Juvenile rheumatoid arthritis
2.
3.
4.
5.
6. 7.
8. 9. 10. 11. 12. 13.
14.
15.
113
matic diseases of childhood. Arthritis Rheum 20(Suppl 2):573-4, 1976 Granberry WM, Brewer El: Early synovectomy in JRA, in AAOS instructional course lecture series. St. Louis, 1974, The CV Mosby Co, pp 27-32 Chaplin D, Pulkki T, Saarimaa A, Vaninio K: Wrist and finger deformities in juvenile rheumatoid arthritis. Acta Rheumatol Scand 15:206-33, 1969 Sairanen E: On rheumatoid arthritis in children-a clinico roentgenological study. Acta Rheumatol Scand Suppl 2:52-7, 1958 Brewer EJ, et al: Current proposed revision of juvenile rheumatoid arthritis criteria, in Proceedings of the first ARA conference on the rheumatic diseases of childhood. Arthritis Rheum 20(Suppl 2): 195-8, 1976 Still GE: On chronic joint disease in children. Med Surg Trans 80:47-59 , 1897 Hanson V, Konreich H, Bernstein B, King KK, Singsen B: Prognosis of juvenile rheumatoid arthritis, in Proceedings of the first ARA conference on the rheumatic disease of childhood. Arthritis Rheum 20(Suppl 2):279-84, 1976 Pulkki T: Rheumatoid deformities of the hand. Acta Rheumatol Scand 7:85-8, 1961 Vainio K, aka M: Ulnar deviation of the fingers. Ann Rheum Dis 12:122-4, 1953 Swezey RL: Dynamic factors in deformity of the rheumatoid hand. Bull Rheum Dis 22:649-56, 1971-2 Shapiro JS: The etiology of ulnar drift: a new factor. 1 Bone Joint Surg [Am] 50:634, 1968 Landsmeer JMF: Studies in the anatomy of articulation. Acta Morphol Neerl Scand 3:288-304, 1968 Pahle JA, Raunio P: The influence of wrist position on finger deviation in the rheumatoid hand. 1 Bone Joint Surg [Br] 51:664-76, 1969 lakobowski S, Roszczynska P: The possibility of surgical treatment in cases of juvenile rheumatoid arthritis. Acta Rheumatol Scand 13: 113-8, 1967 Edstrom G, Gedda PO: Clinic and prognosis of rheumatoid arthritis in children. Acta Rheumato1 Scand 3:12953, 1957
The hand in the child with juvenile rheumatoid arthritis Clinical examination of 100 children showed frequent loss of wrist extension and ulnar deviation. Loss of flexion and radial deviation in the metacarpophalangeal joint is more fr equent than in the adult. Statistical review of charts and roentgenograms of 200 patients showed all had ulnar shortening up to 9 mm but there was no correlation with ulnar deviation or metacarpophalangeal radial deviation. Conservative treatment is reviewed; surgery is rarely indicated.
W. Malcolm Granberry , M.D., and Gary L. Mangum, M.D., Houston, Texas
T he hand surgeon is certainly aware of deformities
and problems in the hand of the adult with rheumatoid arthritis . There is , however, a relative paucity of information concerning the hand in the child with rheumatoid arthritis.1-4 I have been the consultant to the JRA Clinic at Texas Children 's Hospital for 14 years and have had the opportunity to see and treat many children with this condition . Observations and experience concerning the hand in the child with juvenile rheumatoid arthritis (JRA) will be given. Some of the problems are opposite to that seen in the adult. Recently pediatric rheumatologists have separated JRA into three basic onset types . The first one, the classic description given by Still,5 does not fulfill today's complete concept of JRA and is now termed "systemic onset type JRA . " The second onset type is described as "polyarticular" and closely resembles the disease seen in the adult. A third separation, "pauciarticular" onset JRA, is characterized by involvement by four joints or less . As a group, children with JRA have a much milder course and are left with less disability than adults. Between 50% and 70% of these children will go into remission. 7 But the child with serious JRA has more stiffness, loss of motion , and ankylosis than an adult. In a growing child reconstructive procedures such as those used in adults are not applicable, as they will produce epiphyseal arrest. If a child continues to have active arthritis and passes through puberty, the disease changes and resembles that seen in the adult. Children are not as cooperative as adults and therapy From the Baylor College of Medicine , Department of Orthopaedic Surgery, Houston , Texas . Received for publication March 6 , 1978; revised May 28, 1979. Reprint requests: W. Malcolm Granberry, M.D., 7000 Fannin, 20th Floor, Houston, TX 77030 (713 790-1818). 0363 -5023/80/020105+09$00.90/0
designed to improve motion and increase strength are not well accepted by the child. The surgeon who treats the child with arthritis should be conservative as surgery is rarely indicated.
Review of literature A significant article by Chaplin et al. 3 in 1969 directly correlates with this investigation. Their roentgenographic review of over 400 patients with JRA showed that 59% of the patients had wrist or hand involvement including 34% with ulnar deviation of the metacarpals (wrist) associated with radial deviation of the fingers . They also reported that many patients had shortening of the ulna. They proposed and thought that their data supported the concept that JRA damaged the distal ulnar epiphysis and produced shortening. This, in tum, produced ulnar deviation of the metacarpals and in tum radial deviation at the metacarpophalangeal joints. Boutonniere deformity was relatively common, but swan-neck deformity was infrequent. Flexion deformity of the interphalangeal joints was common , especially in the younger age group. This study was performed on patients with a diagnosis of JRA, but there are no data in the paper concerning age of the patients at the time of observation . The illustrations were , for the most part, of adult patients . A review of the deformities in 500 adult patients by Pulkki 8 showed that 28% of patients had ulnar deviation of the metacarpophalangeal joints. Vainio and Oka 9 examined almost 300 unselected patients with rheumatoid arthritis and found that 23% of the adults had ulnar deviation of the metacarpophalangeal joints, but only 3 .3% of children had the deformity . The cause of ulnar deviation of the metacarpophalangeal joints in the adult long has been the subject of controversy . 10 Muscle imbalance with intrinsic spasm, unilateral laxity of the capsule, destruction of the collateral liga-
© 1980 American Society for Surgery of the Hand
THE JOURNAL OF HAND SURGERY
lOS
106
The Journal of HAND SURGERY
Granberry and Mangum
I can find no suggestion in the literature as to why there is a significant incidence of radial deviation of the metacarpophalangeal joints in children, and the theories explaining ulnar deviation certainly do not explain radial deviation. Sporadic reports of management and surgery in the hand of the child with JRA are found in the literature. Usually conservative treatment, including splinting and exercises, is advocated. 2 , 13-15 Clinical examination of 100 consecutive children with JRA
Fig. 1. A girl, age 11 years, with juvenile rheumatoid arthritis and various degrees of radial deviation of the metacarpophalangeal joints. Table I. Clinical examination of the hand in juvenile rheumatoid arthritis in 100 patients No.
Metacarpophalangeal joint: Radial deviation Ulnar deviation Decreased flexion Decreased extension Volar subluxation Interphalangeal joint: Boutonniere Swan neck Decreased flexion, PIP Decreased extension, PIP DIP involvement Wrist: Decreased extension Decreased flexion Volar subluxation Ulnar deviation
10 I
23 7 2 7 I
27 10
18 55
22 13 10
Legend: PIP, proximal interphalangeal joint; DIP, distal interphalangeal joint.
ments by synovitis, change in the shape of the joint surfaces, pressure, gravity, daily use, dislocation of the extensor tendons, or subluxation of the flexor tendons all have been implicated as a cause of ulnar deviation. Shapiro l l thought that the cause was radial deviation of the wrist, with linkage breakdown of the metacarpophalangeal joints, comparable to the theory proposed by Landsmeer. 12 Hands with wrists fused in radial deviation have been shown to develop ulnar deviation of the metacarpophalangeal joints. 13
To study a reasonable cross-section of problems in the hand of children with JRA, the author examined 100 consecutive children who had proven JRA for at least 2 years. Examinations were done on children in the hospital and on patients in the outpatient clinic. This examination required approximately 9 months and was completed about 4 years ago. Some had mild or inactive disease, others had severe debilitating arthritis. No attempt at that time was made to correlate various deformities with age, type of onset, length of illness, etc. Table I contains the findings of this examination. Ten percent of the children were found to have significant clinical radial deviation of the metacarpophalangeal joints (Fig. 1). This deformity cannot be measured accurately and depends on the position of extension-flexion, how the hand is placed on the table, and the attention of the child in the attempt to correct or exaggerate the deviation. I found only one child with significant ulnar deviation in this group. Twenty-three percent of these children were found to have loss of flexion of the metacarpophalangeal joints (Fig. 2), but only 7% were found to have loss of extension. This is opposite to that seen in the adult who typically has loss of extension and ulnar deviation. I found only two children with volar subluxation of a metacarpophalangeal joint. Boutonniere deformities were seen in seven children, but they were mild and flexible. One child demonstrated a mild swan-neck deformity. Intrinsic tightness was not seen in this group of children. Twenty-seven children had a clinically significant decrease in flexion of the interphalangeal joint which seemed to be the result of proliferative synovium with volume restriction (Fig. 3). Ten children had loss of extension of the interphalangeal joint. This was usually seen in the child with significant articular disease and deformity of the joint. Eighteen children were found to have distal interphalangeal joint involvement, usually with synovitis and mild flexion contracture. Table I also records the findings in examination of
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Fig. 2. A, Loss of flexion of the metacarpophalangeal joint in a young boy with mild disease . B, Postoperative flexion after collateral ligament release .
the wrist. Fifty-five of these children had detectable loss of extension. This varied from 15° of loss of extension to 30° of flexion contracture . Twenty-two of these children had detectable loss of flexion, but it was much milder and had little clinical consequence (Fig . 4) . Thirteen children had subluxation of the wrist similar to that seen in the adult. Ten children had ulnar deviation of the wrist (ulnar metacarpal angulation with respect to the forearm). In this clinical series, ulnar deviation of the wrist was not usually associated with radial deviation of the metacarpophalangeal joints (Fig. 5). I saw no child with clinical radial deviation of the wrist so common in the adult. No child had symptoms or signs of carpal tunnel syndrome.
This examination of 100 children with JRA correlates well with our experience on this service . At present approximately 500 children with all stages and types of JRA are being treated. Others have recorded a significant problem with loss of extension of the interphalangeal joints, 3 but I found a greater percentage of children with loss of flexion and only mild loss of extension. The conclusion from this evaluation is that the hand of the child with JRA shows more radial deviation and loss of flexion of the metacarpophalangeal joint in contrast to the adult who usually has ulnar deviation and loss of extension. Others have noted this problem,9 but thought it was due to a breakdown in the linkage sec-
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Fig. 3. Loss of flexion of the interphalangeal joints due to proliferative synovitis and increased intraarticular pressure . Secondary periarticular fibrosis later in the disease process will produce the same result. Also there is loss of flexion of the metacarpophalangeal joint .
Table II. Vital data in a record review of 200 children Race Caucasian Black Latin-American Indian Type Polyarticular Pauciarticular Systemic
181 10 8
44% 30% 26%
ondary to ulnar deviation of the wrist. Our data do not confirm this observation . One of the earliest clinical signs of rheumatoid arthritis in a child is mild loss of complete extension of the wrist. This may be detectable prior to the onset of any palpable synovitis or other findings of JRA . It also serves as a barometer of the disease and usually precedes roentgenographic changes by several months.
Review of records and roentgenograms of 200 patients The charts and films of 200 children with proven JRA were reviewed. Charts from 1976 and before were reviewed. Children in the clinical series were excluded. Table II shows the vital data of these patients. There were 134 females and 66 males . There were 181 Caucasian patients, ten blacks , eight Latin-Americans and one Indian. Forty-four percent of the patients were of the polyarticular onset type, 34% were pauciarticular, and 26% had systemic onset type of JRA. Fig. 6 shows the age of onset and age grouping. Our
Fig. 4. A, Decreased extension with relatively good flexion of the wrist, a common early finding in juvenile rheumatoid arthritis. B, The same wrist , showing good flexion.
population includes many children with early onset disease, with a range from 3 months to 16 years and a mean of 6 years. Fig. 7 reviews the age of the patients at the time of the record review, the youngest being 2 years and the oldest 19 years, with a mean of 12 years . The duration of the illness is shown in Fig. 8. It is noted that half of the patients had JRA for 4 years or less. This confirms our clinical impression that many children have a disease of short duration. Observation and recording in these charts of the problems under consideration were for the most part made by the rheumatologists, pediatric residents on the service , and physical therapists. Roentgenographic reports and a personal review of the roentgenogram when available completed the observations. Table III records the incidence of involvement gleaned from the records. There was clinical or roentgenographic involvement of the wrist in 24.5% of these patients. The metacarpophalangeal joint and proximal interphalangeal joint had some type of in-
Vol. 5, No.2
Juvenile rheumatoid arthritis
March 1980
volvement in almost half of the patients. The distal interphalangeal joint was noted to be involved in 19% of the patients. There was mention of tenosynovitis in seven patients, and one patient had clinical shortening of the fingers. There was a boutonniere deformity in 3% of the patients, but there was no mention of any swan-neck contractures. There was some type of hand and/ or wrist involvement in 59.5% of the patients. Critical examination of the small joints in the hand are not well-recorded by the others in this service, and no mention of ulnar or radial deviation was seen in these charts. Goniometric measurements of the small joints in the hand usually were not recorded. Analysis of wrist and metacarpophalangeal deformities was carried out on the roentgenograms available in these patients. It is the practice of technicians in the radiology department to simply place both hands on a single plate and take a posterior-anterior exposure. No attempt is made to correct or increase the deformity. Of the 119 patients who had some type of hand and/or wrist involvement, only 93 roentgenograms in 49 patients were available and suitable for measurements. Wrist deviation was measured by the angle between the radius and the second metacarpal and recorded in degrees either ulnar or radial. The metacarpophalangeal deviation was measured between the long metacarpal and proximal phalanx and recorded in degrees as either ulnar or radial. The amount of ulnar shortening was difficult to measure. The children with severe affliction had marked deformity and articular destruction of varying degrees which complicated measurement. A comparable area of the radius and ulna at their adjacent margins was chosen, and shortening in millimeters was recorded. In the immature skeleton the distal portion of the metaphysis was chosen. In the older child the distal line of the epiphyseal ossification was used (Fig. 9). The data from these measurements are summarized in Table IV. Ulnar shortening of some degree was found in all 93 wrists in the 49 patients with a range of 1 to 10 mm, with an average of 4.1 mm. Wrist deviation was seen in almost all the patients, with 79 roentgenograms demonstrating ulnar deviation between 4° and 25°, with an average of 13°. Twelve roentgenograms showed radial deviation between 5° and 30°, with an average of 11 0. Sixty-two roentgenograms in 43 patients demonstrated metacarpal joint deviation. Thirty-one hands demonstrated ulnar deviation between 2° and 25°, with an average of 8°. Thirty-one hands also demonstrated radial deviation between 2° and 15°, with an average of 7.4° essentially equal findings. Correlation of different variables such as age, duration, age of onset, and sex with various measurements
109
Fig. 5. A boy, age 10 years. with ulnar deviation of the wrist not necessarily associated with radial deviation of the metacarpophalangeal joints or shortening of the ulna.
Table III. Hand involvement* %
Wrist Mep PIP DIP
Tenosynovitis Boutonniere Swan neck
24.5 49.5 49.0 19 7 3 0
'There was hand and/or wrist involvement in 59.5% of patients. Legend: MCP, metacarpophalangeal joint; PIP, proximal interphalangeal
joint; DIP, distal interphalangeal joint.
was made using the advanced computers available today. Ulnar shortening was not found to be statistically related to the ulnar deviation of the metacarpal though the P value was low (P = 0.063). Ulnar shortening was certainly not related to radial deviation of the metacarpophalangeal joints (P = 0.204) and ulnar deviation of the wrist was not correlated to radial deviation of the
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The Journal of HAND SURGERY
Granberry and Mangum
-
45
r--
44
40
42
3 Months to 16 Years Average 6.6 Years Mean 6.0
35 30
a::
~ 15 ~
~
~ 20
22
r--
r--
21
21
-
-
15
15
14
10
r--
12
5
t
4
16
14
12
10 8 AGE AT ONSET
6
Fig. 6. The age at onset and age grouping . 40
Range 2 - 19 Years Mean 11.4
;--
38
35
Median 12 .0
30
a:: 25
-
25
5 2
,-
26
25
21
10
,-
.----
r--
w ~ 20 ::> z 15
24 r-17
-
15
fl
4
10
8
6
AGE
14
12
18
16
Fig. 7. The age of the patients at the time of the record review.
-60
60
55
Ronge 0 .5 - 17 Yeors Mean 4.8 Median 3.5
50 45
40 a::
35
r--
40
r--
34
~ 30
::;: ~ 25
20
r--
,---,
19
15
18
~
16
10
5 2
4
6
8
DURATION
10
12
Fig. 8. The duration of illness.
n
14
f3l
r3l 16
18
Vol. 5, No.2 March 1980
Juvenile rheumatoid arthritis
111
Fig. 9. Examples of roentgenograms showing lines for measurement. A, Metacarpophalangeal deviation was measured as the angle of the third metacarpal and proximal phalanx. B, Wrist deviation was measured along the center of the radius and the second metacarpal. fingers. The only correlation seen in these figures was the age of the patient without hand or wrist involvement was significantly lower than those with hand or wrist involvement (P = 0.035). The finding of equal amounts of ulnar and radial deviation of the metacarpophalangeal joints does not correlate with the clinical analysis. Though the x-ray technician is asked to place the hand on the x-ray plate without change in the position of the fingers, it is thought that x-ray positioning must in some way change the clinical deformity, and correlation between clinical and x-ray position is not reasonable. The amount of ulnar shortening, however, is not altered by positioning and is thought to be reliable. Comparison of this study to a similar study by Vainio and Oka9 does not show similar findings. Their studies indicated that significant correlation between ulnar shortening and ulnar deviation of the wrist and subsequent radial deviation of the metacarpophalangeal joint. Their illustrations demonstrated more severe deformities than in this series and appeared to be of adult patients. It is probable that their patient population was
Table IV. Measurements of x-rays of children with hand and/or wrist involvement Measurement
Joints
Ulnar shortening Wrist ulnar deviation Wrist radial deviation MCP ulnar deviation MCP radial deviation
93 79 12 31 31
Range
1-lOmm 4°_25° 5°_30° 2°_25° 2°_15°
Average
4.lmm 13° 11° 8° 7.4°
Legend: Mep, metacarpophalangeal joint.
older and contained hospitalized patients with more severe disease, rather than the broad cross-section represented by our clinic popUlation. Surgical procedures performed on children with JRA between 1965 and 1975 Surgical procedures are rarely indicated at this age group and only a few were performed over a lO-year period in this clinic. Two young boys had significant loss of metacarpophalangeal flexion and underwent collateral ligament release with excellent results (Fig. 2).
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The Journal of HAND SURGERY
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Fig. 10. A simple light splint for management of flexion contracture of the wrist.
Table V. Synovectomy of the small joints in juvenile rheumatoid arthritis-late result: September 1976* % Good
Fair Poor
o 92 8
'Follow-up 12 to 118 months (average of 76.5 months).
One patient had tenolysis of the flexor tendons for triggering on separate occasions, and one patient had a resection of the superficialis for marked proximal interphalangeal contracture. One patient had a deQuervain's release and one patient had a collateral ligament reconstruction for a gamekeeper's thumb deformity. One older patient (16 years) underwent a release of the volar carpal ligament for carpal tunnel syndrome. Tenosynovitis is seen in children, but no patient had sufficient disease to warrant tenosynovectomy. One patient had terminal finger vasospasm with gangrene and required amputations. A prospective study of synovectomy of all joints in our service was conducted between 1965 and 1970 16 and a late follow-up was performed in 1976. 17 Three patients during this period had synovectomy of the wrist, and two patients had synovectomies of 16 metacarpophalangeal joints, and three patients had 10 proximal interphalangeal joint synovectomies. The late results of these operations in the joints were extracted and recorded in Table V. Though no patient has had recurrent synovitis of these joints under observation in
this small group, the loss of motion after synovectomy of these joints was significant and was responsible for the poor results. Synovectomy of the wrist or fingers is not advised at the present. Two patients who had synovectomies of the wrist were found to have dry or "nonproliferative" type of synovitis and had rapidly progressive ankylosis of the intercarpal and radiocarpal joints following the operation. It is thought that this type of synovitis is a distinct contraindication to synovectomy. Common clinical problems and their management As seen in the preceding portions of this paper, the child tends to lose wrist extension, develop ulnar deviation of the wrist, and lose flexion of the metacarpophalangeal and interphalangeal joints. I have found this to be the most common combination of problems in the hand of the child with JRA. The primary function of the surgeon attending the children is to follow them very closely and to detect these early problems prior to their becoming fixed deformities. Intensive physical therapy with exercise and a home program is a cornerstone in management. Splinting and occasional steroid injections are necessary. The tendency for loss of extensi~n of the wrist can easily be managed by a simple three point extension orthosis (Fig. 10). In the neglected or unresponsive cases, wedging casts can be used to bring the wrist into functional position and then maintained with the orthosis. Combination splints to retard ulnar deviation and flexion may be found to be necessary. Heat-malleable plastic materials are useful in this problem. The main thrust of management of the
Vol. 5, No.2 March 1980
wrist in this age group is to allow intercarpal and perhaps radiocarpal fusion to take place while maintaining the wrist in a neutral functional position. Wrist fusion, arthroplasty, or replacement is left for the adult patient and these have no place in the treatment of the child. The use of the Bunnell knuckle-bender splint is quite helpful in maintaining flexion of the metacarpophalangeal joints which have a tendency to lose flexion. The therapist should teach the use of paraffin, passive stretching, and active exercises to maintain motion in these joints. The child should be seen by the therapist in conjunction with the pediatrician and orthopaedist, and it is most important that the parents see and learn the program and continue it at home as long as necessary.
Summary The problems and management of the hand in the child with juvenile rheumatoid arthritis are different from those in the adult. Loss of extension of the wrist combined with ulnar deviation is a common problem and conservative splinting and therapy is indicated. Radial deviation of the metacarpophalangeal joints is more common than ulnar deviation but rarely requires aggressive therapy. Statistical analysis attempting to link ulnar shortening with wrist and finger deformities did not confirm previous observations, and no correlation was seen between ulnar deviation of the wrist and radial deviation of the metacarpophalangeal joint. Surgical procedures are rarely indicated, and conservative treatment with physical therapy, orthotic devices, and, rarely, injections are the mainstays of management. REFERENCES 1. Athreya BH: The hand in juvenile rheumatoid arthritis, in Proceedings of the first ARA conference on the rheu-
Juvenile rheumatoid arthritis
2.
3.
4.
5.
6. 7.
8. 9. 10. 11. 12. 13.
14.
15.
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matic diseases of childhood. Arthritis Rheum 20(Suppl 2):573-4, 1976 Granberry WM, Brewer El: Early synovectomy in JRA, in AAOS instructional course lecture series. St. Louis, 1974, The CV Mosby Co, pp 27-32 Chaplin D, Pulkki T, Saarimaa A, Vaninio K: Wrist and finger deformities in juvenile rheumatoid arthritis. Acta Rheumatol Scand 15:206-33, 1969 Sairanen E: On rheumatoid arthritis in children-a clinico roentgenological study. Acta Rheumatol Scand Suppl 2:52-7, 1958 Brewer EJ, et al: Current proposed revision of juvenile rheumatoid arthritis criteria, in Proceedings of the first ARA conference on the rheumatic diseases of childhood. Arthritis Rheum 20(Suppl 2): 195-8, 1976 Still GE: On chronic joint disease in children. Med Surg Trans 80:47-59 , 1897 Hanson V, Konreich H, Bernstein B, King KK, Singsen B: Prognosis of juvenile rheumatoid arthritis, in Proceedings of the first ARA conference on the rheumatic disease of childhood. Arthritis Rheum 20(Suppl 2):279-84, 1976 Pulkki T: Rheumatoid deformities of the hand. Acta Rheumatol Scand 7:85-8, 1961 Vainio K, aka M: Ulnar deviation of the fingers. Ann Rheum Dis 12:122-4, 1953 Swezey RL: Dynamic factors in deformity of the rheumatoid hand. Bull Rheum Dis 22:649-56, 1971-2 Shapiro JS: The etiology of ulnar drift: a new factor. 1 Bone Joint Surg [Am] 50:634, 1968 Landsmeer JMF: Studies in the anatomy of articulation. Acta Morphol Neerl Scand 3:288-304, 1968 Pahle JA, Raunio P: The influence of wrist position on finger deviation in the rheumatoid hand. 1 Bone Joint Surg [Br] 51:664-76, 1969 lakobowski S, Roszczynska P: The possibility of surgical treatment in cases of juvenile rheumatoid arthritis. Acta Rheumatol Scand 13: 113-8, 1967 Edstrom G, Gedda PO: Clinic and prognosis of rheumatoid arthritis in children. Acta Rheumato1 Scand 3:12953, 1957