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Reconstructive Hand Surgery for Scleroderma Joint Contractures
TECHNIQUE ARTICLE Reconstructive Hand Surgery for Scleroderma Joint Contractures Ananthila Anandacoomarasamy, MBBS (Hons), Helen Englert, MBBS, PhD, Nicholas Manolios, MD, PhD, Stuart Kirkham, MBBS From the Department of Rheumatology, Royal North Shore Hospital, St. Leonards, North South Wales, Australia; the Departments of Rheumatology and Orthopaedics, Westmead Hospital, Westmead, New South Wales, Australia.
Systemic scleroderma can cause significant hand deformity and functional impairment. Surgery is often avoided due to the perceived risks of wound healing. The most common surgical procedures have been digital sympathectomy, arthrodesis or arthroplasty of the proximal interphalangeal (PIP) or both, and metacarpophalangeal (MCP) joints. We describe herein successful soft tissue hand surgery in 2 patients for treatment of scleroderma claw deformities without the use of arthrodesis or arthroplasty. At the MCP joint, the tight capsules were excised, and the collateral ligaments and volar plates were released. At the PIP joints, the volar plates were released and the tight palmar skin was released, resulting in marked improvement of joint position. Intensive hand therapy was used to maximize function. In these 2 patients with claw deformity, we found that tight volar skin was the main contributor to flexion contracture at the PIP level. In contrast, joint capsule contracture was the main contributor to hyperextension deformity at the MCP level. (J Hand Surg 2007;32A: 1107–1112. Copyright © 2007 by the American Society for Surgery of the Hand.) Key words: Hand surgery, scleroderma.
cleroderma, also known as systemic sclerosis (SS), is a heterogeneous connective tissue disorder characterized by excessive collagen production within the skin and internal organs.1 The 2 clinical subsets—limited cutaneous and diffuse cutaneous—are distinguished by the extent of skin involvement, autoantibody profile and pattern of organ involvement. In limited disease, hand involvement is thought to relate to micro- and macrovascular disease. In diffuse disease, however, hand involvement also includes joint contractures. Claw deformity is common. The pathophysiology of claw deformity is unknown. When scleroderma causes hand contractures, the most typical pattern of clinical deformity is fixed flexion deformities at the proximal interphalangeal (PIP) joints, with associated fixed extension, and even hyperextension deformities at the metacarpophalangeal (MCP) joints. The distal interphalangeal (DIP) joints may show fixed flexion, while the thumb may show some adduction contracture with narrowing of the first web space.2 Contractures can also occur at
S
the wrist. Foot involvement is less common and tends to occur later in the disease process. Typically, the deformities that are observed have excessive collagen affecting the skin (scleroderma), fascia, ligaments, tendons, and joint capsules. Radiological joint destruction, however, is not common, especially in early disease. As a result of prolonged fixed flexion posturing, trauma, and associated poor micro-circulation, these patients also commonly suffer with dorsal PIP joint ulceration. In the hyperextended MCP joints, fascial tissue between extensor tendons and the joint capsule becomes sclerotic, secondarily contributing to extension contracture.3 A study by Herrick et al looked at the functional ability of patients with SS.4 In 140 patients, using an 11-item functional questionnaire, they found that functional limitations were largely related to contractures and decreased range of motion, especially in the hand. Although their functional questionnaire had not been validated and was weighted heavily to upper extremity The Journal of Hand Surgery
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function,5 functional disability seems related, at least in part, to flexion contractures. We report the cases of 2 women with diffuse SS who underwent soft tissue reconstructive surgery to correct finger contractures, which were significantly impeding function. Good results were obtained by releasing the contractures at the PIP and MCP joints. This method carries several functional advantages over arthrodesis. The factors contributing to MCP and PIP joint contractures are discussed.
Case 1 A 24-year-old woman was diagnosed with diffuse SS. Initial treatment consisted of prednisone, methotrexate and D-penicillamine. Ten months after the onset of symptoms, the scleroderma had progressed to involve the limbs, anterior chest wall, and trunk. She also had early pulmonary disease. She was then treated with monthly pulse cyclophosphamide, pulse methylprednisolone, and cyclosporin. She underwent autologous stem cell transplantation (SCT) 14 months after diagnosis, as she continued to have rapidly progressive diffuse disease with inflammatory features. Prior to SCT, she had diffuse scleroderma involving the limbs and most of her trunk. Her modified Rodnan skin score (a measure of skin thickening) was 27/51. Following the procedure, the woman began to experience reversal of the skin thickening and reduction in polyarthralgia after 2 months. Follow-up evaluation at 15 months revealed marked clinical improvement in her musculoskeletal disease status as measured by skin scores, health assessment questionnaire, C-reactive protein, and visual analogue scales. Her skin softened dramatically, and all joint contractures resolved, with the exception of the MCPs and PIPs. In fact, these contractures were progressive, and she had great difficulty in performing activities of daily living. Pain was not a prominent feature. Preoperative evaluation revealed varying degrees of finger deformity (Fig. 1a). The worst affected joints (fifth PIPs, bilaterally) had fixed flexion deformity of 85° to 90°. She had hyperextension of the MCPs and narrowing of the first web spaces, bilaterally. Sensory examination was normal. Allen’s test was slightly abnormal with delay in capillary refill time (4 –5 seconds). Doppler ultrasound of the digital vessels (after SCT) showed good blood flow to both hands. Plain radiographs of both hands showed no evidence of erosive change or loss of joint space. She proceeded to reconstructive surgery of the right hand under general anesthesia.
Figure 1. (A) Prereconstructive hand surgery. (B) Postoperative image demonstrating flexed MCP joints and extended PIP joints.
For exposure of the MCP joints, a transverse incision was made over all 4 MCPs on the dorsal aspect. The sagittal band of the extensor hood was incised for each digit. The extensor tendon was then temporarily sublaxated from the midline of the metacarpal head towards the ulna. Then, the thickened dorsal capsule was excised completely and sent for histological assessment. Following this, the Freer elevator (Aesculap Inc., Center Valley, PA) was used to, first, tease off the collateral ligaments from the metacarpal head and the base of the proximal phalanx. Then, the
Anandacoomarasamy et al / Hand Surgery for Scleroderma
volar plate was released from the distal end of the metacarpal head and neck. Intraoperatively, the following passive ranges of motion were obtained: index finger 0° to 100°, middle finger 0° to 110°, ring finger 0° to 110° and little finger 0° to 110°. The PIPs were then each approached with 4 separate midlateral incisions. The interval between the accessory collateral ligament and the volar plates was incised. The volar plate was then removed as much as possible, though in some digits (especially the ring finger) it was only possible to incise the volar plate. Palmar skin was released at the skin crease for digits 3, 4, and 5. Intraoperatively, the following passive ranges of motion were obtained at the PIPs: index finger 0° to 125°, middle finger 0° to 125°, ring finger 0° to 125°, and little finger 0° to 125°. To keep the fingers splinted in the intrinsic-plus position, intra-osseous K-wires were passed through each of the metacarpal heads to keep the MCPs flexed and the PIPs extended. Extending and straightening the previously pathologically fixed and flexed PIPs resulted in a defect of 1–2 cm on each digit at the PIP joint, requiring skin grafts. A full thickness skin graft measuring 18 ⫻ 1.5 cm was taken from the volar distal forearm. The skin was used to graft the dorsal aspect of the metacarpal heads and the volar aspect of the PIP joint skin creases (Fig. 1b). The donor sites were not sclerodermatous. The pathological findings at surgery were as follows: At the MCP joint level, the dorsal skin was tight. There were adhesions between the extensor apparatus and the capsule. To a lesser extent, there were some tight subcutaneous fibrous bands superficial to the veins in line with the ring and little fingers only. The dorsal capsule was very thick. The capsules were more responsible for lack of MCP joint flexion than the skin. Once the skin, capsule, and adhesions were addressed, the joint underwent a large increase in passive flexion. This corrected posture left a skin defect measuring approximately 18 mm in maximal width. At the PIP joint level, volar relieving skin incisions over each PIP joint allowed the joints to gain considerable passive extension. The volar plate and joint capsule, once released, also contributed to overall joint stiffness to a lesser extent than the skin. These joints did not become free until these structures were all released. The tight volar skin was the main contributing factor to lack of extension. Histopathology showed marked fibrosis of the volar plate of tissue (PIP joint) with hyalinized bundles of collagen fibers (Fig. 2). The dorsal capsule at the
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Figure 2. Histology of the volar plate of the PIP joint obtained at surgery demonstrating marked fibrosis with hyalinized bundles of collagen.
MCP joint had dense avascular fibrous tissue with prominent hyalinization of the collagen. In our volar plate biopsy specimen, vessels were found that showed hyperplasia of the tunica media. The smaller vessels showed a predominant lymphocytic infiltrate around and within the vessel wall. Postoperatively, there were no complications, specifically no ischemic or wound healing problems. Her skin grafts took completely in a normal time frame. Three weeks after surgery, the 4 K-wires were removed. She underwent regular hand therapy sessions with static and dynamic splints. Four months after surgery, she had maintained her hand in the new intrinsic-plus posture. Also, she achieved noticeable improvements in hand function, with return of key grip and ability to wash her hair. She had not, however, regained power grip. Overall the patient was satisfied that she had achieved a modest gain. Eighteen months following reconstructive hand surgery, the patient is able to use her operated hand for functional tasks, which she cannot perform with the non-surgically treated claw hand. The operated hand allows her to open jars and drive a car holding the steering wheel, neither of which she can do with her un-operated hand. She is unable, however, to perform certain tasks with her operated hand, such as reaching into her small handbag. Overall, postoperative hand function has improved. The patient would recommend the procedure to her friends and would agree to have it again under the same circumstances. Her overall satisfaction was relatively high. The current range of motion is only modest. There remains marked bilateral hand stiffness. On the operated side,
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her fingers are stiff in a position of much greater PIP joint extension.
Case 2 A 52-year-old woman was diagnosed with scleroderma. She had diffuse disease with no major organ involvement. Her medications were methotrexate, cyclosporine, and D-penicillamine. Her function was severely impaired by severe contractures of both hands. Preoperatively, the MCP joints of both hands were fully extended while all the PIP joints were fully flexed. The DIP joints were flexed to a lesser extent. The dorsal skin over the MCP joints was very tight. Sensory examination was normal. Angiography of the digital vessels showed good blood flow. Plain radiographs of both hands were normal. Reconstructive surgery of the left hand was performed under general anesthesia. The four MCP joints were approached through a single, dorsal, transverse skin incision. The MCP joint capsules were thickened, and these were excised dorsally. There were minor adhesions between the skin and the tendons. There was definite shortening of the skin requiring full-thickness skin grafts once the defect was corrected. An elliptical full-thickness graft was sutured into place over the dorsal aspect of the MCP joint. The 4 PIP joints were then addressed. A transverse, relieving skin incision was made at the flexion skin crease at the PIP joint level. The incision was then extended by making an anchor-shaped incision with proximal and distal extensions centered in the midlateral aspect of the PIP joint. This was done on the radial border for the index, middle, and ring fingers. The ulnar border was used at the little finger due to difficult access. The neurovascular bundle was identified and protected throughout the procedure. A Beaver 6400 scalpel blade (Robbins Instruments Inc., Chatham, NJ) was used to incise the volar plate and pathological ligaments. The PIP joints were then manipulated from their abnormally flexed position into a fully extended position. This was performed while observing the tension on the neurovascular bundles. Once all 4 fingers were corrected, K-wires were placed across the metacarpal heads and into the proximal and middle phalanges, creating an intrinsic plus position for the entire hand. With the fingers in the extended position, the tourniquet was released, and perfusion in the 4 digits was observed. The index, middle and ring fingers perfused well, but the little finger did not. Therefore, the K-wire was pulled back to allow the finger to flex.
This permitted immediate reperfusion. Following this, the K-wire was repositioned into the little finger but only across the MCP joint (not across the PIP joint to avoid excess tension across the artery). Fullthickness skin grafts obtained from the volar proximal forearm were sutured into place on the volar aspect of the four PIP joints. Each graft measured approximately 2–3 cm in longitudinal length. The total procedure took 4 hours. The intraoperative pathological findings were similar to those seen in the first case. Postoperatively, there were no complications. The skin grafts took completely. Sutures were removed 2 weeks after surgery and K-wires 4 weeks thereafter. Subsequently, she underwent intensive hand therapy to maintain hand position and improve strength. Since removal of K-wires, the fingers have remained in the corrected positions (MCPs flexed and PIPs extended). At the 12-month follow-up visit, her overall hand function showed only small gains. She could hold onto a rowing machine at the gym with the operated hand. Marked bilateral hand stiffness remains, with the operated side showing a much less clawed posture and also a mildly improved ability to perform mono- and bimanual tasks.
Discussion Joint contractures are a major cause of morbidity and disability in scleroderma. Despite this, reconstructive hand surgery in patients with scleroderma has been reported infrequently, probably reflecting the fear that wound healing is compromised in these patients and that surgical intervention might cause further deterioration in the vascularity of the hands and digits.2 A few series have been reported. Most authors advocate and report the outcomes of joint arthrodesis or arthroplasty at the PIP joint and MCP joint capsulotomy or arthroplasty (with or without metacarpal head resection).2,6 –10 Adduction contracture of the thumb requires an opening of the first web space to release the adductor attachment. Resection of the trapezium has been used successfully to restore thumb metacarpal abduction.3 With disease progression, wound healing and the possibility of tissue loss is a genuine concern. The skin over the dorsal aspect of these joints is stretched tightly, and the dermal capillary bed can be reduced by as much as 80%.6 Implant prostheses carry the additional risk of implant failure as well as a higher rate of wound healing complications.3 The earliest small series was reported by Lips-
Anandacoomarasamy et al / Hand Surgery for Scleroderma
comb et al in 6 patients who underwent surgery from 1962. The patients reported an improvement in overall function. The range of motion obtained at the MCP joints, however, was not greatly improved by capsulotomy and arthroplasty.7 Swanson flexible implant arthroplasties have been used at the PIP joints, but the ultimate gain in motion reported is small.7 Jones et al performed 53 PIP joint fusions in 12 patients.2 Arthrodesis was accomplished in a position of 45° to 55° using a combination of K-wires, intraosseous wires, and bony shortening. This improved the position of the fingers for both thumb– index finger pinch and grasp. They also performed 19 MCP capsulotomies and 4 metacarpal head resections in conjunction with PIP joint arthrodesis in 4 patients. The resultant motion at the MCP joints, however, was reported to be disappointing, averaging less than 20°.2 Gilbart et al reported 13 PIP joint fusions and 5 MCP joint excisional arthroplasties.6 Nalebuff reports that in selected cases, capsulotomy will increase the range of motion in the MCP joints.9 The largest series reported thus far by Melone et al included 70 scleroderma patients requiring 272 hand operations.10 They performed 211 interphalangeal joint arthrodeses after complete capsulotomy and jdicious skeletal shortening. Radiographic union of arthrodesis was achieved within 8 weeks of surgery. Twenty-eight implant arthroplasties were performed after wide resection of the metacarpal heads, condyles, and collateral ligaments. The authors report a 50° average arc of motion for these arthroplasties after follow-up evaluations ranging from 1 to 15 years. In most reports to date, severe fixed finger-in-palm deformities have been treated with a combination of MCP joint excisional arthroplasty and PIP joint fusion, involving resection of bone from both the distal metacarpal and proximal phalanges.11 While this procedure improves finger position and function, there is resultant axial shortening of the hand with a loss of the normal MCP joint contour.11 In our patients, a satisfactory functional outcome and aesthetic appearance was gained by positioning the MCP joints in flexed position with the PIP joints in extension. By carefully excising the capsule, and separating the collateral ligaments and volar plates, replacement arthroplasty at the MCP joints was obviated. Likewise, at the PIP joints, careful incision and removal of the volar plates and release of the palmar skin at the skin crease resulted in marked improvement in passive range of motion. Bone resection and joint fusion was avoided by releasing the
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tissues responsible for joint contractures. We believe that soft tissue releases offer obvious advantages compared with bony procedures such as arthrodesis and arthroplasty, especially in light of the fact that so many scleroderma patients have normal joint radiographs. There were 3 important findings from this small case series of 2 patients with relatively early diffuse scleroderma. First, we believe that reconstructive surgery for one hand, leaving the other hand with flexed PIP joints would appear to confer the greatest functional advantage. Second, we were able to elucidate and rank in order of importance the factors contributing to sclerodermatous claw hand at the MCP and PIP joints. At the MCP joint level, capsular tightening and adherence between the latter and overlying tendons was the prime limiting factor. At the PIP level, however, the predominant joint deformity occurred because of overlying skin tightness rather than joint pathology per se. Hence therapeutic intervention to prevent or ameliorate such features in other patients will need to focus on both aggressive control of sclerodactyly (PIP), and joint and periarticular tissue (MCP). Finally we were able to successfully perform surgery in patients with early aggressive diffuse disease without complications relating to vascular compromise, either in the hands or at the area of donor skin. This type of surgery, however, is not without considerable risks. These patients should be advised, preoperatively, of the risks of arterial intimal tears and digital ischemia (including the possibility of losing one or more digits) and that the 3– 4 months of rehabilitation is only likely to achieve a small-tomoderate gain of their overall hand function. Cosmetically the improved posture needs to be weighed against the appearance of the skin grafts. Prolonged flexion may also cause attenuation of the central slip of the extensor digitorum communis. Unlike the normal digit, however, this attenuation does not necessarily lead to any boutonniere deformity because of the very tight skin and joints that prevent the passive imbalance of a boutonniere posture. Our cases also suggest that scleroderma patients should not be denied the benefits of reconstructive hand surgery when they have significant functional limitation from joint deformities. In patients with advanced hand contractures, a nominal measured improvement in position and function may lead to a substantial improvement in the patient’s adaptive ability to perform certain activities of daily living.11
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Hand surgery can improve function but needs to be considered together with the specific surgical risks and a realistic discussion of how long the recovery takes. Hand surgery is an adjunct to medical management and should be considered in the overall management of scleroderma patients with disabling contractures. Further studies may allow us to determine the optimal timing of both medical and surgical treatment in the disease process. Received for publication December 29, 2006; accepted in revised form June 13, 2007. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Corresponding author: Stuart Kirkham, MBBS, Department of Orthopaedics, Westmead Hospital, Westmead, NSW 2145, Australia; e-mail: [email protected]. Copyright © 2007 by the American Society for Surgery of the Hand 0363-5023/07/32A07-0023$32.00/0 doi:10.1016/j.jhsa.2007.06.011
References 1. Lin ATH, Clements PJ, Furst DE. Update on disease-modifying antirheumatic drugs in the treatment of systemic sclerosis. Rheum Dis Clin N Am 2003;29:409 – 426.
2. Jones NF, Imbriglia JE, Steen VD, Medsger TA. Surgery for scleroderma of the hand. J Hand Surg 1987;12A:391– 400. 3. Jakubietz MG, Jakubietz RG, Gruenert JG. Scleroderma of the hand. J Am Soc Surg Hand 2005;5:42– 47. 4. Herrick A, Rooney B, Finn J, Silman A. Lack of relationship between functional ability and skin score in patients with systemic sclerosis. J Rheumatol 2001;28:292–295. 5. Pope JE. Musculoskeletal involvement in scleroderma. Rheum Dis Clin N Am 2003;29:391– 408. 6. Gilbart MK, Jolles BM, Lee P, Bogoch ER. Surgery of the hand in severe systemic sclerosis. J Hand Surg 2004;29B: 599 – 603. 7. Lipscomb PR, Simons GW, Winkelmann RK. Surgery for sclerodactylia of the hand. J Bone Joint Surg 1969A;51:112– 117. 8. Norris RW, Brown HG. The proximal interphalangeal joint in systemic sclerosis and its surgical management. Br J Plast Surg 1985;38:526 –531. 9. Nalebuff EA. Surgery in patients with systemic sclerosis of the hand. Clin Orthop 1999;366:91–97. 10. Melone CP, McLoughlin JC, Beldner S. Surgical management of the hand in scleroderma. Curr Opin Rheumatol 1999;11:514 –520. 11. Bogoch ER, Cross DK. Surgery of the hand in patients with systemic sclerosis: outcomes and considerations. J Rheumatol 2005;32:642– 648.
Systemic scleroderma can cause significant hand deformity and functional impairment. Surgery is often avoided due to the perceived risks of wound healing. The most common surgical procedures have been digital sympathectomy, arthrodesis or arthroplasty of the proximal interphalangeal (PIP) or both, and metacarpophalangeal (MCP) joints. We describe herein successful soft tissue hand surgery in 2 patients for treatment of scleroderma claw deformities without the use of arthrodesis or arthroplasty. At the MCP joint, the tight capsules were excised, and the collateral ligaments and volar plates were released. At the PIP joints, the volar plates were released and the tight palmar skin was released, resulting in marked improvement of joint position. Intensive hand therapy was used to maximize function. In these 2 patients with claw deformity, we found that tight volar skin was the main contributor to flexion contracture at the PIP level. In contrast, joint capsule contracture was the main contributor to hyperextension deformity at the MCP level. (J Hand Surg 2007;32A: 1107–1112. Copyright © 2007 by the American Society for Surgery of the Hand.) Key words: Hand surgery, scleroderma.
cleroderma, also known as systemic sclerosis (SS), is a heterogeneous connective tissue disorder characterized by excessive collagen production within the skin and internal organs.1 The 2 clinical subsets—limited cutaneous and diffuse cutaneous—are distinguished by the extent of skin involvement, autoantibody profile and pattern of organ involvement. In limited disease, hand involvement is thought to relate to micro- and macrovascular disease. In diffuse disease, however, hand involvement also includes joint contractures. Claw deformity is common. The pathophysiology of claw deformity is unknown. When scleroderma causes hand contractures, the most typical pattern of clinical deformity is fixed flexion deformities at the proximal interphalangeal (PIP) joints, with associated fixed extension, and even hyperextension deformities at the metacarpophalangeal (MCP) joints. The distal interphalangeal (DIP) joints may show fixed flexion, while the thumb may show some adduction contracture with narrowing of the first web space.2 Contractures can also occur at
S
the wrist. Foot involvement is less common and tends to occur later in the disease process. Typically, the deformities that are observed have excessive collagen affecting the skin (scleroderma), fascia, ligaments, tendons, and joint capsules. Radiological joint destruction, however, is not common, especially in early disease. As a result of prolonged fixed flexion posturing, trauma, and associated poor micro-circulation, these patients also commonly suffer with dorsal PIP joint ulceration. In the hyperextended MCP joints, fascial tissue between extensor tendons and the joint capsule becomes sclerotic, secondarily contributing to extension contracture.3 A study by Herrick et al looked at the functional ability of patients with SS.4 In 140 patients, using an 11-item functional questionnaire, they found that functional limitations were largely related to contractures and decreased range of motion, especially in the hand. Although their functional questionnaire had not been validated and was weighted heavily to upper extremity The Journal of Hand Surgery
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function,5 functional disability seems related, at least in part, to flexion contractures. We report the cases of 2 women with diffuse SS who underwent soft tissue reconstructive surgery to correct finger contractures, which were significantly impeding function. Good results were obtained by releasing the contractures at the PIP and MCP joints. This method carries several functional advantages over arthrodesis. The factors contributing to MCP and PIP joint contractures are discussed.
Case 1 A 24-year-old woman was diagnosed with diffuse SS. Initial treatment consisted of prednisone, methotrexate and D-penicillamine. Ten months after the onset of symptoms, the scleroderma had progressed to involve the limbs, anterior chest wall, and trunk. She also had early pulmonary disease. She was then treated with monthly pulse cyclophosphamide, pulse methylprednisolone, and cyclosporin. She underwent autologous stem cell transplantation (SCT) 14 months after diagnosis, as she continued to have rapidly progressive diffuse disease with inflammatory features. Prior to SCT, she had diffuse scleroderma involving the limbs and most of her trunk. Her modified Rodnan skin score (a measure of skin thickening) was 27/51. Following the procedure, the woman began to experience reversal of the skin thickening and reduction in polyarthralgia after 2 months. Follow-up evaluation at 15 months revealed marked clinical improvement in her musculoskeletal disease status as measured by skin scores, health assessment questionnaire, C-reactive protein, and visual analogue scales. Her skin softened dramatically, and all joint contractures resolved, with the exception of the MCPs and PIPs. In fact, these contractures were progressive, and she had great difficulty in performing activities of daily living. Pain was not a prominent feature. Preoperative evaluation revealed varying degrees of finger deformity (Fig. 1a). The worst affected joints (fifth PIPs, bilaterally) had fixed flexion deformity of 85° to 90°. She had hyperextension of the MCPs and narrowing of the first web spaces, bilaterally. Sensory examination was normal. Allen’s test was slightly abnormal with delay in capillary refill time (4 –5 seconds). Doppler ultrasound of the digital vessels (after SCT) showed good blood flow to both hands. Plain radiographs of both hands showed no evidence of erosive change or loss of joint space. She proceeded to reconstructive surgery of the right hand under general anesthesia.
Figure 1. (A) Prereconstructive hand surgery. (B) Postoperative image demonstrating flexed MCP joints and extended PIP joints.
For exposure of the MCP joints, a transverse incision was made over all 4 MCPs on the dorsal aspect. The sagittal band of the extensor hood was incised for each digit. The extensor tendon was then temporarily sublaxated from the midline of the metacarpal head towards the ulna. Then, the thickened dorsal capsule was excised completely and sent for histological assessment. Following this, the Freer elevator (Aesculap Inc., Center Valley, PA) was used to, first, tease off the collateral ligaments from the metacarpal head and the base of the proximal phalanx. Then, the
Anandacoomarasamy et al / Hand Surgery for Scleroderma
volar plate was released from the distal end of the metacarpal head and neck. Intraoperatively, the following passive ranges of motion were obtained: index finger 0° to 100°, middle finger 0° to 110°, ring finger 0° to 110° and little finger 0° to 110°. The PIPs were then each approached with 4 separate midlateral incisions. The interval between the accessory collateral ligament and the volar plates was incised. The volar plate was then removed as much as possible, though in some digits (especially the ring finger) it was only possible to incise the volar plate. Palmar skin was released at the skin crease for digits 3, 4, and 5. Intraoperatively, the following passive ranges of motion were obtained at the PIPs: index finger 0° to 125°, middle finger 0° to 125°, ring finger 0° to 125°, and little finger 0° to 125°. To keep the fingers splinted in the intrinsic-plus position, intra-osseous K-wires were passed through each of the metacarpal heads to keep the MCPs flexed and the PIPs extended. Extending and straightening the previously pathologically fixed and flexed PIPs resulted in a defect of 1–2 cm on each digit at the PIP joint, requiring skin grafts. A full thickness skin graft measuring 18 ⫻ 1.5 cm was taken from the volar distal forearm. The skin was used to graft the dorsal aspect of the metacarpal heads and the volar aspect of the PIP joint skin creases (Fig. 1b). The donor sites were not sclerodermatous. The pathological findings at surgery were as follows: At the MCP joint level, the dorsal skin was tight. There were adhesions between the extensor apparatus and the capsule. To a lesser extent, there were some tight subcutaneous fibrous bands superficial to the veins in line with the ring and little fingers only. The dorsal capsule was very thick. The capsules were more responsible for lack of MCP joint flexion than the skin. Once the skin, capsule, and adhesions were addressed, the joint underwent a large increase in passive flexion. This corrected posture left a skin defect measuring approximately 18 mm in maximal width. At the PIP joint level, volar relieving skin incisions over each PIP joint allowed the joints to gain considerable passive extension. The volar plate and joint capsule, once released, also contributed to overall joint stiffness to a lesser extent than the skin. These joints did not become free until these structures were all released. The tight volar skin was the main contributing factor to lack of extension. Histopathology showed marked fibrosis of the volar plate of tissue (PIP joint) with hyalinized bundles of collagen fibers (Fig. 2). The dorsal capsule at the
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Figure 2. Histology of the volar plate of the PIP joint obtained at surgery demonstrating marked fibrosis with hyalinized bundles of collagen.
MCP joint had dense avascular fibrous tissue with prominent hyalinization of the collagen. In our volar plate biopsy specimen, vessels were found that showed hyperplasia of the tunica media. The smaller vessels showed a predominant lymphocytic infiltrate around and within the vessel wall. Postoperatively, there were no complications, specifically no ischemic or wound healing problems. Her skin grafts took completely in a normal time frame. Three weeks after surgery, the 4 K-wires were removed. She underwent regular hand therapy sessions with static and dynamic splints. Four months after surgery, she had maintained her hand in the new intrinsic-plus posture. Also, she achieved noticeable improvements in hand function, with return of key grip and ability to wash her hair. She had not, however, regained power grip. Overall the patient was satisfied that she had achieved a modest gain. Eighteen months following reconstructive hand surgery, the patient is able to use her operated hand for functional tasks, which she cannot perform with the non-surgically treated claw hand. The operated hand allows her to open jars and drive a car holding the steering wheel, neither of which she can do with her un-operated hand. She is unable, however, to perform certain tasks with her operated hand, such as reaching into her small handbag. Overall, postoperative hand function has improved. The patient would recommend the procedure to her friends and would agree to have it again under the same circumstances. Her overall satisfaction was relatively high. The current range of motion is only modest. There remains marked bilateral hand stiffness. On the operated side,
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her fingers are stiff in a position of much greater PIP joint extension.
Case 2 A 52-year-old woman was diagnosed with scleroderma. She had diffuse disease with no major organ involvement. Her medications were methotrexate, cyclosporine, and D-penicillamine. Her function was severely impaired by severe contractures of both hands. Preoperatively, the MCP joints of both hands were fully extended while all the PIP joints were fully flexed. The DIP joints were flexed to a lesser extent. The dorsal skin over the MCP joints was very tight. Sensory examination was normal. Angiography of the digital vessels showed good blood flow. Plain radiographs of both hands were normal. Reconstructive surgery of the left hand was performed under general anesthesia. The four MCP joints were approached through a single, dorsal, transverse skin incision. The MCP joint capsules were thickened, and these were excised dorsally. There were minor adhesions between the skin and the tendons. There was definite shortening of the skin requiring full-thickness skin grafts once the defect was corrected. An elliptical full-thickness graft was sutured into place over the dorsal aspect of the MCP joint. The 4 PIP joints were then addressed. A transverse, relieving skin incision was made at the flexion skin crease at the PIP joint level. The incision was then extended by making an anchor-shaped incision with proximal and distal extensions centered in the midlateral aspect of the PIP joint. This was done on the radial border for the index, middle, and ring fingers. The ulnar border was used at the little finger due to difficult access. The neurovascular bundle was identified and protected throughout the procedure. A Beaver 6400 scalpel blade (Robbins Instruments Inc., Chatham, NJ) was used to incise the volar plate and pathological ligaments. The PIP joints were then manipulated from their abnormally flexed position into a fully extended position. This was performed while observing the tension on the neurovascular bundles. Once all 4 fingers were corrected, K-wires were placed across the metacarpal heads and into the proximal and middle phalanges, creating an intrinsic plus position for the entire hand. With the fingers in the extended position, the tourniquet was released, and perfusion in the 4 digits was observed. The index, middle and ring fingers perfused well, but the little finger did not. Therefore, the K-wire was pulled back to allow the finger to flex.
This permitted immediate reperfusion. Following this, the K-wire was repositioned into the little finger but only across the MCP joint (not across the PIP joint to avoid excess tension across the artery). Fullthickness skin grafts obtained from the volar proximal forearm were sutured into place on the volar aspect of the four PIP joints. Each graft measured approximately 2–3 cm in longitudinal length. The total procedure took 4 hours. The intraoperative pathological findings were similar to those seen in the first case. Postoperatively, there were no complications. The skin grafts took completely. Sutures were removed 2 weeks after surgery and K-wires 4 weeks thereafter. Subsequently, she underwent intensive hand therapy to maintain hand position and improve strength. Since removal of K-wires, the fingers have remained in the corrected positions (MCPs flexed and PIPs extended). At the 12-month follow-up visit, her overall hand function showed only small gains. She could hold onto a rowing machine at the gym with the operated hand. Marked bilateral hand stiffness remains, with the operated side showing a much less clawed posture and also a mildly improved ability to perform mono- and bimanual tasks.
Discussion Joint contractures are a major cause of morbidity and disability in scleroderma. Despite this, reconstructive hand surgery in patients with scleroderma has been reported infrequently, probably reflecting the fear that wound healing is compromised in these patients and that surgical intervention might cause further deterioration in the vascularity of the hands and digits.2 A few series have been reported. Most authors advocate and report the outcomes of joint arthrodesis or arthroplasty at the PIP joint and MCP joint capsulotomy or arthroplasty (with or without metacarpal head resection).2,6 –10 Adduction contracture of the thumb requires an opening of the first web space to release the adductor attachment. Resection of the trapezium has been used successfully to restore thumb metacarpal abduction.3 With disease progression, wound healing and the possibility of tissue loss is a genuine concern. The skin over the dorsal aspect of these joints is stretched tightly, and the dermal capillary bed can be reduced by as much as 80%.6 Implant prostheses carry the additional risk of implant failure as well as a higher rate of wound healing complications.3 The earliest small series was reported by Lips-
Anandacoomarasamy et al / Hand Surgery for Scleroderma
comb et al in 6 patients who underwent surgery from 1962. The patients reported an improvement in overall function. The range of motion obtained at the MCP joints, however, was not greatly improved by capsulotomy and arthroplasty.7 Swanson flexible implant arthroplasties have been used at the PIP joints, but the ultimate gain in motion reported is small.7 Jones et al performed 53 PIP joint fusions in 12 patients.2 Arthrodesis was accomplished in a position of 45° to 55° using a combination of K-wires, intraosseous wires, and bony shortening. This improved the position of the fingers for both thumb– index finger pinch and grasp. They also performed 19 MCP capsulotomies and 4 metacarpal head resections in conjunction with PIP joint arthrodesis in 4 patients. The resultant motion at the MCP joints, however, was reported to be disappointing, averaging less than 20°.2 Gilbart et al reported 13 PIP joint fusions and 5 MCP joint excisional arthroplasties.6 Nalebuff reports that in selected cases, capsulotomy will increase the range of motion in the MCP joints.9 The largest series reported thus far by Melone et al included 70 scleroderma patients requiring 272 hand operations.10 They performed 211 interphalangeal joint arthrodeses after complete capsulotomy and jdicious skeletal shortening. Radiographic union of arthrodesis was achieved within 8 weeks of surgery. Twenty-eight implant arthroplasties were performed after wide resection of the metacarpal heads, condyles, and collateral ligaments. The authors report a 50° average arc of motion for these arthroplasties after follow-up evaluations ranging from 1 to 15 years. In most reports to date, severe fixed finger-in-palm deformities have been treated with a combination of MCP joint excisional arthroplasty and PIP joint fusion, involving resection of bone from both the distal metacarpal and proximal phalanges.11 While this procedure improves finger position and function, there is resultant axial shortening of the hand with a loss of the normal MCP joint contour.11 In our patients, a satisfactory functional outcome and aesthetic appearance was gained by positioning the MCP joints in flexed position with the PIP joints in extension. By carefully excising the capsule, and separating the collateral ligaments and volar plates, replacement arthroplasty at the MCP joints was obviated. Likewise, at the PIP joints, careful incision and removal of the volar plates and release of the palmar skin at the skin crease resulted in marked improvement in passive range of motion. Bone resection and joint fusion was avoided by releasing the
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tissues responsible for joint contractures. We believe that soft tissue releases offer obvious advantages compared with bony procedures such as arthrodesis and arthroplasty, especially in light of the fact that so many scleroderma patients have normal joint radiographs. There were 3 important findings from this small case series of 2 patients with relatively early diffuse scleroderma. First, we believe that reconstructive surgery for one hand, leaving the other hand with flexed PIP joints would appear to confer the greatest functional advantage. Second, we were able to elucidate and rank in order of importance the factors contributing to sclerodermatous claw hand at the MCP and PIP joints. At the MCP joint level, capsular tightening and adherence between the latter and overlying tendons was the prime limiting factor. At the PIP level, however, the predominant joint deformity occurred because of overlying skin tightness rather than joint pathology per se. Hence therapeutic intervention to prevent or ameliorate such features in other patients will need to focus on both aggressive control of sclerodactyly (PIP), and joint and periarticular tissue (MCP). Finally we were able to successfully perform surgery in patients with early aggressive diffuse disease without complications relating to vascular compromise, either in the hands or at the area of donor skin. This type of surgery, however, is not without considerable risks. These patients should be advised, preoperatively, of the risks of arterial intimal tears and digital ischemia (including the possibility of losing one or more digits) and that the 3– 4 months of rehabilitation is only likely to achieve a small-tomoderate gain of their overall hand function. Cosmetically the improved posture needs to be weighed against the appearance of the skin grafts. Prolonged flexion may also cause attenuation of the central slip of the extensor digitorum communis. Unlike the normal digit, however, this attenuation does not necessarily lead to any boutonniere deformity because of the very tight skin and joints that prevent the passive imbalance of a boutonniere posture. Our cases also suggest that scleroderma patients should not be denied the benefits of reconstructive hand surgery when they have significant functional limitation from joint deformities. In patients with advanced hand contractures, a nominal measured improvement in position and function may lead to a substantial improvement in the patient’s adaptive ability to perform certain activities of daily living.11
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The Journal of Hand Surgery / Vol. 32A No. 7 September 2007
Hand surgery can improve function but needs to be considered together with the specific surgical risks and a realistic discussion of how long the recovery takes. Hand surgery is an adjunct to medical management and should be considered in the overall management of scleroderma patients with disabling contractures. Further studies may allow us to determine the optimal timing of both medical and surgical treatment in the disease process. Received for publication December 29, 2006; accepted in revised form June 13, 2007. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Corresponding author: Stuart Kirkham, MBBS, Department of Orthopaedics, Westmead Hospital, Westmead, NSW 2145, Australia; e-mail: [email protected]. Copyright © 2007 by the American Society for Surgery of the Hand 0363-5023/07/32A07-0023$32.00/0 doi:10.1016/j.jhsa.2007.06.011
References 1. Lin ATH, Clements PJ, Furst DE. Update on disease-modifying antirheumatic drugs in the treatment of systemic sclerosis. Rheum Dis Clin N Am 2003;29:409 – 426.
2. Jones NF, Imbriglia JE, Steen VD, Medsger TA. Surgery for scleroderma of the hand. J Hand Surg 1987;12A:391– 400. 3. Jakubietz MG, Jakubietz RG, Gruenert JG. Scleroderma of the hand. J Am Soc Surg Hand 2005;5:42– 47. 4. Herrick A, Rooney B, Finn J, Silman A. Lack of relationship between functional ability and skin score in patients with systemic sclerosis. J Rheumatol 2001;28:292–295. 5. Pope JE. Musculoskeletal involvement in scleroderma. Rheum Dis Clin N Am 2003;29:391– 408. 6. Gilbart MK, Jolles BM, Lee P, Bogoch ER. Surgery of the hand in severe systemic sclerosis. J Hand Surg 2004;29B: 599 – 603. 7. Lipscomb PR, Simons GW, Winkelmann RK. Surgery for sclerodactylia of the hand. J Bone Joint Surg 1969A;51:112– 117. 8. Norris RW, Brown HG. The proximal interphalangeal joint in systemic sclerosis and its surgical management. Br J Plast Surg 1985;38:526 –531. 9. Nalebuff EA. Surgery in patients with systemic sclerosis of the hand. Clin Orthop 1999;366:91–97. 10. Melone CP, McLoughlin JC, Beldner S. Surgical management of the hand in scleroderma. Curr Opin Rheumatol 1999;11:514 –520. 11. Bogoch ER, Cross DK. Surgery of the hand in patients with systemic sclerosis: outcomes and considerations. J Rheumatol 2005;32:642– 648.