Hand surgery in patients with systemic sclerosis (scleroderma)

INTRODUCTION — Systemic sclerosis (SSc, scleroderma) is a connective tissue disorder with unclear etiology. Patients with SSc can experience significant pain and functional limitations in affected hands. Common hand manifestations of SSc include joint contractures, skin ulcerations, digital tip ischemia, Raynaud phenomenon, and calcinosis cutis. Surgery is usually reserved for patients who have failed nonoperative management.

Common patterns of deformity of the hands of patients with SSc and the surgical approaches for treatment of digital tip ischemia, finger and thumb deformities with or without associated skin breakdown, and digital calcific deposits are presented here. Clinical manifestations and medical management of SSc are presented separately. (See "Clinical manifestations and diagnosis of systemic sclerosis (scleroderma) in adults" and "Overview of the treatment and prognosis of systemic sclerosis (scleroderma) in adults".)

The medical management of Raynaud phenomenon, which is a nearly universal problem in patients with SSc, is also presented separately. (See "Treatment of Raynaud phenomenon: Initial management" and "Treatment of Raynaud phenomenon: Refractory or progressive ischemia".)

ISSUES THAT MAY REQUIRE SURGERY — Patients with systemic sclerosis (SSc) and complications involving the hand are best managed by a team that includes a rheumatologist, occupational therapist, and a hand surgeon. Initially, every effort is made to preserve function, optimize perfusion, and regain motion nonsurgically. This is done by medications, using exercises to preserve range of motion, and using appropriate splinting. However, nonoperative treatment to increase motion is usually not successful. Splinting in maximum corrected position without undue pressure may delay progression of the deformity. The impaired circulation, compromised skin, and immunosuppressed condition, characteristic of patients with SSc, present significant challenges to surgical treatment. Nevertheless, selected surgeries may be helpful in relieving pain and in restoring function in certain scenarios when nonoperative treatments have failed.

Surgical procedures on the hands of patients with SSc generally aim to address the following sequelae of disease [1]:

●Digital ischemia (see 'Digital ischemia' below)

●Interphalangeal (IP) and metacarpophalangeal (MCP) joint deformities (see 'Joint deformities' below)

●Calcinosis cutis (see 'Calcinosis cutis' below)

Digital ischemia — Almost all patients with SSc experience Raynaud phenomenon, and nearly half of patients have fingertip ulceration at some point in their clinical course [2-4]. The etiology of digital ischemia in SSc involving the hand is likely multifactorial and may involve Raynaud phenomenon with components of vasospasm as well as intimal fibrosis and narrowing, thrombosis and occlusion, and vascular compression by connective tissue. Early Raynaud phenomenon may manifest as paroxysmal blanching of the finger, especially in states of cold temperature or stress, followed by hyperemia. As the disease progresses, patients may experience painful fingertip ischemia and ulceration, which may be slow to heal due to compromised blood supply. (See "Clinical manifestations and diagnosis of Raynaud phenomenon".)

Joint deformities — Three deformities of the hand are commonly found in patients with limited or diffuse SSc [5,6]. The most frequent deformity pattern is a flexion contracture of the proximal interphalangeal (PIP) joint with compensatory hyperextension of the MCP joint. Hyperextension of the PIP joint (swan neck deformity) and contracture of the skin and muscle of the first web space are less frequently observed.

●Proximal interphalangeal joint flexion contracture – Flexion contractures at the PIP joints are the most common joint deformities seen in SSc. PIP joint flexion contractures may progress to beyond 90 degrees and may be severely functionally limiting in themselves. Moreover, progressive development of PIP joint flexion contractures leads to attenuation or failure of the central slip of the extensor mechanism (picture 1). As PIP joint flexion contractures progress, the dorsal skin overlying these joints may attenuate, blanch, and ultimately break down, exposing the underlying tendon, joint capsule, or bone. Septic arthritis and/or chronic osteomyelitis may further complicate management and, in many cases, will require surgical treatment to eradicate the infection.

Increasing flexion contracture of the PIP joint results in compensatory hyperextension at the MCP joint. Initially, the MCP joints maintain active flexion; however, over time, the collateral ligaments, joint capsule, and overlying skin contract and severely limit MCP joint flexion, resulting in static MCP joint hyperextension contractures.

●Proximal interphalangeal hyperextension contracture – Hyperextension contractures of the PIP joint develop secondary to MCP joint flexion with or without volar subluxation. This condition is similar to the swan neck deformity observed in patients with rheumatoid arthritis (picture 2). As patients lose their ability to extend the fingers at the MCP level, they compensate by hyperextending the PIP joints. The skin over the hyperextended PIP joints is contracted and the dorsal skin ulceration over the PIP joints is not commonly seen in this deformity pattern. Rarely, a prominent metacarpal head can erode the palmar skin overlying MCP joints.

●First web-space contracture – Contracture of the skin, muscles, and fascia of the first web space between the thumb and index finger reduces thumb mobility (picture 3). This abnormality diminishes the ability of the patient to move the thumb out of the plane of the palm and to grasp large objects between the thumb and fingers [7].

Calcinosis cutis — Calcinosis cutis describes intracutaneous or subcutaneous calcium deposition, seen in approximately 15 percent of patients with diffuse SSc and 44 percent of patients with CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) (image 1) [1,6,8,9]. Calcium deposits can occur within one digit or throughout the hand, but commonly occur in the index finger, middle finger, and distal aspect of the thumb. The deposits are firm and produce tender areas in the finger pulp that may interfere with pinch and grasp. They can spontaneously ulcerate and extrude calcific material; secondary infections are a concern. (See "Clinical manifestations and diagnosis of systemic sclerosis (scleroderma) in adults", section on 'Cutaneous manifestations'.)

SURGICAL OPTIONS — Patients with systemic sclerosis (SSc) offer inherent challenges to surgical interventions involving the hands, including poor circulation, compromised and inelastic skin often with ulceration, and immunocompromised systemic state often compounded by immunosuppressive medications. Despite these challenges, some problems affecting the hands of patients with SSc are best treated surgically. These problems include digital ischemia, joint deformities that limit hand function or cause skin ulceration, and calcific depositions.

Treatment of refractory or progressive ischemia — Indications for surgical intervention include severe and disabling Raynaud phenomenon, ischemic digital pain refractory to medical management, and persistent or recurrent digital tip ulcerations [10,11]. (See "Treatment of Raynaud phenomenon: Refractory or progressive ischemia".)

Digital sympathectomy — The mainstay of surgical treatment to improve digital circulation in SSc is ablation of the sympathetic innervation of the digital vessels. This is accomplished by stripping the adventitia of the target vessels, where the sympathetic nerves reside. Various techniques and locations of adventitial stripping have been described. While some surgeons recommend adventitial stripping as distally as practical, at the level of the proper digital arteries in the digit and common digital arteries in the palm [12], other surgeons recommend operating more proximally, at the level of the radial and ulnar arteries at the wrist and the palmar arch and the common digital arteries in the palm (figure 1) [11,13-16]. The extent of adventitial stripping ranges from 3 mm to 2 cm [11,12,17]. Most surgeons strip at least 1 cm of adventitia in the palm involving the common digital arteries [9,10,12,17-19]. Use of the operating microscope is important for performing digital sympathectomy to ensure complete circumferential ablation of the adventitia along a contiguous length of the vessel without damage to the media or intima, which could predispose toward thrombosis [12,17]. (See "Treatment of Raynaud phenomenon: Refractory or progressive ischemia", section on 'Digital sympathectomy'.)

Digital sympathectomy improves digital blood flow, alleviates ischemic pain, and improves healing of fingertip ulcers. However, the effects of digital sympathectomy are frequently short lived, and sympathetic reinnervation occurs over time. Adverse outcomes in patients who have undergone digital sympathectomy include delays in wound healing and development of infection. Progression or recurrence of the disease can lead to amputation [13]. Nonetheless, at two- to four-year follow-up, over 80 percent of patients report better pain control, fewer ulcerations, and decreased time to healing of ulcerations [11,20].

There has been interest in the use of botulinum toxin A for a chemical sympathectomy as opposed to the traditional surgical periarterial sympathectomy [21-23]. The botulinum toxin A is presumed to inhibit sympathetic nerves, but its exact mechanism of action is not known. A total of 50 to 100 reconstituted units of botulinum toxin A are injected per hand, through a dorsal approach, in divided doses in the web spaces. The role of botulinum toxin A for the treatment of refractory or progressive ischemia secondary to Raynaud phenomenon is discussed in detail separately. (See "Treatment of Raynaud phenomenon: Refractory or progressive ischemia", section on 'Botulinum toxin A'.)

Vascular reconstruction — Patients with correctable vascular lesions, such as thrombosis of the ulnar artery, radial artery, or palmar arch, may benefit from microvascular reconstruction of the diseased vessel [1,18,24]. When a reconstructible lesion is suspected, preoperative angiography is useful to delineate the arterial anatomy; however, if the diagnosis is clear or when urgent correction of ischemia is warranted, it may be reasonable to forego preoperative studies [24]. When angiography identifies a vessel amenable to surgical reconstruction, the affected vessel is surgically explored, the segment of artery that is thrombosed is identified and resected, and the artery is reconstructed with a microsurgical technique using a reversed vein interposition graft harvested from the forearm. It should be noted that resection of the diseased vessel provides an effective sympathectomy of that vessel by virtue of excision of the segment of adventitia, which may also aid in treating the ischemia [10,18]. If constrictive fibrous tissue is identified around the vessel in question, arterial decompression may be performed by excision of the constrictive connective tissue.

Amputation — Severe ischemia of the fingertips can lead to ulceration or gangrene, which may require amputation. In spite of optimal medical management (eg, local topical antimicrobials, measures to improve perfusion), ulcerations may be slow to heal or not heal at all. The underlying distal phalangeal tuft may resorb, and if gangrene ultimately develops, auto-amputation may occur. (See "Upper extremity amputation", section on 'Hand and fingers'.)

Secondary infections may occur, more than 90 percent of which are caused by Staphylococcus aureus. Pseudomonas aeruginosa is another potential causative organism [10,18]. Infections may progress to paronychia, felon, osteomyelitis of the distal or middle phalanx, or pyogenic flexor tenosynovitis. In these scenarios, surgical amputation at the distal interphalangeal (DIP) joint level may be necessary (picture 4). The presence of ischemia complicates the usual treatment of osteomyelitis (eg, systemic antibiotics, debridement), and conservative treatment is more likely to fail in patients with SSc.

Amputation is a rare option for the treatment of deformities. (See 'Treatment of deformities' below.)

Treatment of deformities — Surgery may be helpful for treating deformities of the DIP, proximal interphalangeal (PIP), and metacarpophalangeal (MCP) joints of the fingers and at the interphalangeal (IP) and MCP joints of the thumb [1,25-27]. Release of contractures of the first web space (between the thumb and index finger) may help improve function, particularly grasping large objects. (See 'Joint deformities' above.)

Proximal interphalangeal joint — Flexion contractures at the PIP joint are the most commonly seen deformity pattern in SSc. Flexion contractures may progress to extensor tendon and skin attenuation and breakdown, leaving the underlying soft tissues and joint susceptible to infection.

Surgery at the PIP joints is indicated to correct severe deformities, to improve access for hygiene, and to address dorsal skin ulcerations. Most surgeons agree that PIP joint fusion is the appropriate surgical option in most cases [1,26,27]. Early PIP joint fusion once the flexion contracture is greater than 50 degrees may help delay, lessen, or prevent MCP joint extension contractures with minimal loss of function. Headless compression screws, tension band constructs, and crossed Kirschner wires are all options for bony fixation. Buried implants decrease the duration of need for postoperative splinting and the risk of pin-associated infections.

The optimal position of PIP joint fusion varies by digit, ranging from 30 to 40 degrees in the index finger to 45 degrees to 55 degrees in the small finger. The overall functional result of a PIP joint fusion depends on the range of motion at the adjacent MCP joint. With good MCP joint motion, the PIP joint can be fused in less flexion. However, if MCP flexion is reduced, MCP capsulotomy or arthroplasty can be considered. MCP joint surgery to regain flexion in a digit with a PIP flexion deformity should be done at the same time or in a second stage after PIP joint fusion in a more extended position. If MCP joint surgery is performed to regain flexion, a flexed PIP joint will limit the gain in MCP flexion. Alternatively, a more extended PIP joint will help to regain MCP joint flexion. PIP joint flexible implant arthroplasties have been described [1,27], but due to the fixed flexion contracture and extensor tendon attenuation that is so often seen in these patients, the ultimate gain in motion from arthroplasty is small.

It is possible to operate with less risk at the PIP joint because the circulation at the PIP joint level is better than that at the fingertip [26]. However, with severe flexion contractures, an intraoperative circulatory problem may develop as the digit is straightened. In this setting, it may be necessary to accept less deformity correction.

Although not ideal, even infected or exposed joints may be approached surgically. Infection necessitates debridement and appropriate antibiotic therapy.

Skin and bone resection, the latter being a routine part of the fusion procedure, allows primary closure in most cases.

The dorsal PIP joint is a common area of skin ulceration in patients with SSc. These open wounds occurring over the PIP joints can progress to deep infections such as septic arthritis or osteomyelitis, which may threaten the digit. Skin ulcerations on the dorsum of the PIP joints should be distinguished from skin ulcerations at the fingertips. While the common end pathway for both is skin ischemia and breakdown, the former occurs from direct pressure of the phalangeal head in the setting of a severe flexion contracture, while the latter occurs from digital vascular insufficiency such as from vasospasm. Many dorsal open wounds may heal with local wound care, dressing changes, and appropriate splinting or, if needed, fusion in a more extended position.

Following the procedure, range-of-motion exercises should be curtailed until ulcerations have healed, and overly vigorous exercise should be avoided to prevent rupture of the extensor hood. Cases of recalcitrant or recurrent ulcerations should be evaluated for deep infection or bony deformity on exam and with radiographs and may necessitate resection of a bony prominence or PIP joint arthrodesis. PIP joint arthrodesis offers a way to correct the characteristic flexion deformity and offload the pressure on the dorsal skin.

Metacarpophalangeal joint — Perfusion approaches near-normal levels at the MCP joint among patients with SSc. Since the area is often warm to palpation, the options available to the surgeon are more varied with the prospect of more normal wound healing [26]. However, tightness of the dorsal skin poses a significant problem and limits attempts to achieve additional flexion surgically. Surgical options at the MCP joints include flexible implant arthroplasty or resection arthroplasty (image 2). Due to the nature of the disease, resultant motion after either procedure at the MCP joint may be limited. In advanced cases of SSc, even after capsular release and resection arthroplasty, MCP joint motion averages less than 20 degrees [10]. However, the arc of that motion may be in a more functional position, and any pain from arthritis or joint subluxation is typically alleviated.

●For patients with severe ulnar deviation and MCP flexion, a standard dorsal approach is used for resection arthroplasty.

●For patients with shortened dorsal skin and fixed MCP hyperextension, it may be beneficial to approach the metacarpal heads via a palmar approach, allowing for wide resection of the metacarpal heads without implants to restore flexion.

●Resection arthroplasty of the MCP may be combined with PIP fusion (image 2). PIP fusion should be performed after or at the same time as the MCP joint surgery to facilitate the rehabilitation of the MCP joints. Considerable remodeling of the metacarpal heads after resection has been observed, which makes this technique a viable alternative to implant surgery in patients with MCP joint involvement.

Distal interphalangeal joint — Surgery of the DIP joints most commonly consists of either amputation or fusion. Amputation may be required if the patient has gangrene, osteomyelitis of the distal phalanx, or a septic DIP joint (see 'Amputation' above). This surgery is best done with the patient under local anesthesia with tourniquet release before closure to ensure the presence of adequate circulation for skin healing. The skin flaps are closed loosely to avoid any vascular compromise of the skin.

DIP joint fusions are performed to address pain, to correct severe deformity, or to provide stability. Longitudinal headless compression screw or Kirschner wire fixation with the joint in the neutral position is simple and safe options for internal fixation. However, any surgery at the DIP joint level in a digit with diminished circulation is risky and carries the possibility of postoperative digital necrosis and the need for subsequent amputation.

First web-space contracture — Contracture of the skin and soft tissues in the first web space, between the thumb and index finger, may be seen in patients with SSc (picture 3). Adduction contractures of the thumb limit the patient's ability to bring the thumb out of the plane of the palm and grasp large objects. (See 'Joint deformities' above.)

Surgery for these contractures requires addressing the contracted fascia of the adductor pollicis. The skin in the first web space may be released and widened by various Z-plasty operations (see "Z-plasty"). At times, a skin graft or a regional flap may be required to maintain the opening of the first web space. Selected fusions of the IP joint or MCP joint of the thumb are also helpful when deformities reduce the ability to achieve an adequate thumb index pinch. In some patients, resection of the trapezium with or without ligament reconstruction may be helpful to restore abduction of the thumb.

Treatment of refractory calcinosis cutis — Surgery may be a primary or adjunctive therapy for refractory areas of calcinosis cutis [1,6,9]. The medical management of calcinosis cutis is discussed in detail separately. (See "Calcinosis cutis: Management".)

Indications for debulking digital calcifications are palliative and include recalcitrant pain or frequent or infected ulceration. Volar involvement of the digits is often the most symptomatic area because of pressure with grip and pinch. Since the calcifications may be intimately adherent to the undersurface of the dermis, meticulous surgical technique is required to dissect away the calcifications while handling the skin with care. Gentle saline irrigation may be helpful to safely remove calcific material. Some surgeons describe the use of a high-speed micro-burr to express and debulk calcific depositions [28]. Devitalized skin should be excised. In some situations, fingertips should be left open to heal by secondary intension rather than using sutures through compromised skin.

Minor wound breakdown is common but usually heals with time and local wound care. Recurrence is uncommon, and when calcifications recur, they may not be as bothersome as before surgery. Most patients can expect pain relief and improvement in pinch from calcinosis cutis debulking [6]. (See "Calcinosis cutis: Management", section on 'Role of surgery'.)

SUMMARY

●Patients with systemic sclerosis (SSc, scleroderma) offer inherent challenges to surgical interventions involving the hands, including poor circulation, compromised and inelastic skin often with ulceration, and immunocompromised systemic state often compounded by immunosuppressive medications. Despite these challenges, some problems affecting the hands of patients with SSc are best treated surgically. (See 'Introduction' above.)

●Surgical procedures on the hands of patients with SSc generally aim to address the following complications:

•Digital ischemia (see 'Digital ischemia' above)

•Interphalangeal (IP) and metacarpophalangeal (MCP) joint deformities (see 'Joint deformities' above)

•Calcinosis cutis (see 'Calcinosis cutis' above)

●Indications for surgical intervention for refractory or progressive ischemia include severe and disabling Raynaud phenomenon, ischemic digital pain refractory to medical management, and persistent or recurrent digital tip ulcerations. The mainstay of surgical treatment to improve digital circulation in SSc is ablation of the sympathetic innervation of the digital vessels. Patients with correctable vascular lesions, such as thrombosis of the ulnar artery, radial artery, or palmar arch, may benefit from microvascular reconstruction of the diseased vessel. Severe ischemia of the fingertips can lead to ulceration or gangrene, which may require amputation. (See 'Treatment of refractory or progressive ischemia' above and 'Digital sympathectomy' above and 'Vascular reconstruction' above and 'Amputation' above.)

●Surgery may be helpful for treating deformities of the proximal interphalangeal (PIP) joints, the MCP joints, and the distal interphalangeal (DIP) joints of the fingers. Release of contractures of the first web space (between the thumb and index finger) may help improve function, particularly grasping large objects. (See 'Treatment of deformities' above.)

●Surgery may be a primary or adjunctive therapy for refractory areas of calcinosis cutis. Indications for debulking digital calcifications are palliative and include recalcitrant pain or frequent or infected ulceration. (See 'Treatment of refractory calcinosis cutis' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Edward A Nalebuff, MD, now deceased, who contributed to an earlier version of this topic review.