Takeaways
Question: What are the treatment options for Dupuytren's disease?
Findings: Percutaneous needle fasciotomy has an immediate improvement but a higher recurrence rate and rare tendon, or nerve complications. Collagenase injections require two visits and have an increased number of minor side effects, with rare tendon injuries. Limited fasciectomy removes the diseased tissue and has a prolonged recovery with a higher complication rate, but has the lowest recurrence rate. Secondary fasciectomy after a previous dermofasciectomy has an unexpected amputation rate as high as 8%.
Meaning: There are multiple options available for patients and those treating this disease.
INTRODUCTION
Dupuytren’s disease (DD) is benign but progressive fibrosis of the hand and digits that leads to flexion deformities that can be disabling to some patients and has vexed surgeons for centuries despite all treatments described.1 Various nonoperative and operative treatment options are available for the management of DD, but the mainstay of treatment remains limited fasciectomy (LF) surgery.1 Other treatments include percutaneous needle fasciotomy (PNF), open fasciotomy (OF), Clostridium collagenase histolyticum (CCH) injections, and dermofasciectomy (DF). In this article, we summarize the highlights of each treatment option as well as its strength and weakness. We also describe practical tips with regard to DD surgical management.
Nonsurgical Management
Observation
As DD is a slowly progressive disease, watchful waiting is an option for patients with minimal contracture and without significant functional disability.2 Many surgeons suggest an intervention when Hueston’s tabletop test is positive, meaning when one cannot put the affected hand down flat on a table facing palm down. Generally, the tabletop test is positive with about a 30-degree metacarpophalangeal joint (MCPJ) contracture. Although the test may be negative with 30-degree proximal interphalangeal joint (PIPJ) contracture due to the ability of the MCPJ to hyperextend. A PIPJ contracture of 5–15 degrees is considered another indication for further intervention.
Certain patients have clinical features (onset before 50 years old, bilateral disease, ectopic disease, and positive family history) called Dupuytren diathesis, which may predict an aggressive disease course and a higher chance of disease recurrence.3 However, genomic analysis and a weighted genetic risk score are more helpful in predicting disease recurrence than is Dupuytren diathesis.3
Physiotherapy
Physical therapy treatments have had some success in treating established DD contractures.4,5 Hand therapy and orthotics are commonly prescribed after corrective DD treatment, but there is a lack of solid evidence to support this intervention.5 However, hand therapy after DD treatment allows multiple modalities such as orthoses, exercise, edema control, pain control, and scar management for each individual.5
Radiation
Low-dose radiotherapy may halt disease progression via inhibition of myofibroblasts.4,6 However, a systematic review of its use showed that the proof of its clinical efficacy is scarce.7 Radiation is encouraging for preventing further progression and symptoms of DD, but it does not correct existing contractures.7,8 Also, follow-up treatment after radiotherapy needs special consideration by hand surgeons due to operating in a radiated field.
Pharmacologic Treatments
Steroids
Steroid injection can cause regression of nodules and cords found in early DD.9 It can be beneficial for treating knuckle pads (Fig. 1).
Fig. 1.: Triamcinolone injection for a knuckle pad. A, Dupuytren disease knuckle pad. B, Result after a ¼ cm3 of triamcinolone 40 injection at 1 year.
Antitumor Necrosis Factor (anti-TNF) Agents
Results from the ongoing phase 2 randomized controlled trial in England showed injecting adalimumab, an anti-TNF agent, directly into DD’s nodules effectively inhibits the myofibroblasts in a dose-dependent fashion compared with saline control at 2-week follow-up.10 These results give hope to a biological therapy for DD.
Clostridium Histolyticum Collagenase
This mixture of two collagenases binds, unwinds, and cleaves type I and type III collagen in the cords synergistically while sparing the neurovascular structures. CCH injection has a favorable safety profile with rare severe complications, and it especially works well on MCPJ contractures or lower severity disease.11–16 The indication for on-label usage is a palpable cord, although private insurance often requires a positive tabletop test for approval to use CCH. Two vial injection allows treatment of two joints or an additional ray at one sitting.16 There was no clinical difference between delayed three-day manipulation and one-day manipulation.16 There was also no change in the complication profile with two vial injections, except for more skin tears: 22% versus 9% with a one vial injection.16 The tendon rupture rate with two vial injections was reduced to 0.1% despite twice the dose.16
Surgical Management
Percutaneous Needle Fasciotomy
PNF can be performed under local anesthesia in an office and can be repeated multiple times. Although PNF has a high recurrence rate, it has many advantages, including immediate result, fast recovery, low cost, and that it does not preclude any future interventions such a fasciectomy.17 PNF can be performed on severe contractures and bring the disease back to a less severe stage. Some patients may have excellent long-term results (Fig. 2).
Fig. 2.: PNF for severe, recurrent Dupuytren contracture. A, Severe stage IV Dupuytren contracture after a previous fasciectomy. B, Result immediately after PNF. C, Result from a single PNF treatment 13 years later.
Open Fasciotomy
Dupuytren introduced OF. Single, double, or triple open fasciotomies were performed at the distal palmer crease and the MCPJ/PIPJ creases, and allowed to heal via secondary intention.18 The success rate was high (93%), and the re-operation rate was only 13.5% at a mean of 46 months.18 More severe diseases required triple fasciotomies and had more recurrence, whereas MCPJ disease needed only single fasciotomies.18 The secondary operation of either repeat OF (13%), LF (33%), or DF (54%) was successful in 97%, with no reported complications.18
Limited Fasciectomy
All affected DD and nodules are removed while the neurovascular bundle is traced out and protected in LF. The recovery is prolonged, and swelling during immobilization can lead to stiffness with flexion. On the other hand, it has a much lower recurrence rate than fasciotomy or CCH injections.15,17
Dermofasciectomy
DF involves removing the affected DD and skin and coverage with a full-thickness skin graft. It has the lowest recurrence rate. A study by Armstrong et al reviewed 143 rays treated with DF and showed a recurrence rate of 8.4% with a 5.8-year follow-up.19 Disadvantages include prolonged recovery, skin graft failure, donor site scarring, a higher complication rate, and poor skin color/texture match.
Amputation
The most common cause of elective amputation of the digits is from DD, and it may be recommended in severe, recurrent cases.20 (Fig. 3). DD may recur in an amputation stump and require further treatment (Fig. 4). The use of distraction devices lowers the amputation rate.21
Fig. 3.: CCH injections for severe, recurrent Dupuytren contracture. A, This patient with severe PIPJ contracture after a previous fasciectomy had an amputation recommended due to the severity of the contracture. Injection markings for CCH in blue. B, After manipulation. C, Extension at 4-year follow-up.
Fig. 4.: Markings for CCH injections into recurrent DD in an amputation stump after distal necrosis developed after a previous LF. Before fasciectomy, the surgeon had advised that PNF with blind poking and the risk of tendon damage with CCH were too dangerous.
Complications
All current treatments may result in devastating complications for the patient.22 Complications of each treatment are summarized in Table 1. The most dangerous procedure is fasciectomy after a previous dermofasciectomy, which has an unplanned amputation rate of 8%.22 This could be due to surgical disruption to the digital arteries resulting from dissecting through a scarred surgical bed.36 A small series of severe PIPJ disease treated with LF had an unexpected amputation rate of 5%.33
Table 1. -
Complications of Dupuytren’s Disease Treatments
Complications |
The Complication Rate for Each Treatment |
CCH |
Two Vial CCH23 |
Primary PNF |
Primary LF |
Primary DF |
Primary and Re-operation (Excluding CCH) |
Edema |
74%24 |
77%23 |
NR |
NR |
NR |
NR |
Contusion |
54.5%–64%24,25 |
59%23 |
NR |
NR |
NR |
NR |
Lymph node tenderness |
9%–10.3%11,24 |
|
NR |
NR |
NR |
NR |
Lymphadenopathy |
3%–11.1%24–26 |
13%23 |
NR |
NR |
NR |
NR |
Pruritus |
12.6%25 |
15%23 |
NR |
NR |
NR |
NR |
Injection site pruritus |
5%24,25 |
|
NR |
NR |
NR |
NR |
Ecchymosis |
17.7%25 |
5%23 |
NR |
NR |
NR |
NR |
Injection site hemorrhage |
34.1%25 |
8%23 |
NR |
NR |
NR |
NR |
Pain in extremity |
26%–36%24,25 |
50%23 |
NR |
20%25 |
NR |
NR |
Pain intensity Visual Analog Pain Scale |
4.9%26 |
NR |
2.7%26 |
NR |
NR |
NR |
Wound complications or skin tears |
11%12 |
22%23 |
3.4%––68%27–29 |
3%–23%24,30 |
0%–25%25 |
NR |
Surgical site infection |
0%25,31 |
NR |
0.1%–1.5%28,29,32 |
2.4%–9.6%24,25,30 |
5%25 |
2.5%–3.2%21,33 |
Neurapraxia or paresthesia |
4.4%–5%24,25 |
NR |
0.1%–1.3%27–29,32 |
5–9.424,25 |
41%–51.3%25 |
NR |
Nerve injury |
0%25,31 |
NR |
0.04%–0.6%27–29,32 |
2%–3.8%24,25,30 |
1.5%–25.3%25 |
3.3%–3.7%21,34 |
Arterial injury |
0%25 |
NR |
NR |
3.3%–5.5%25,30 |
NR |
1.7%–2.5%33,34 |
Hematoma |
5%–39%25,26 |
8%23 |
0%26,27 |
2.1%30 |
14.925 |
1.2%21 |
Stiffness to flexion |
NR |
NR |
NR |
5%30 |
NR |
NR |
Blood blister |
1.4–1424–26 |
12%23 |
NR |
NR |
NR |
NR |
Flare reaction |
2%24 |
NR |
NR |
NR |
NR |
NR |
CRPS |
0.1–0.525,28,31 |
NR |
0%27,32 |
4.5%–5.5%25,30 |
2.6%25 |
NR |
Tendon rupture |
0%–0.3%25,26 |
0.1%23 |
0%–0.2%26–29,32 |
0.1%25 |
NR |
0.2%–2.5%21,25,33 |
Unplanned finger amputation |
Two reported cases35 |
0%32 |
0.2%36 |
0.30%36 |
0.42%36 |
0.55%–5.1%33,36 |
Systemic severe medical complication |
NR |
0.1% (anaphylaxis)23 |
0%27 |
NR |
NR |
0.78%36 |
All current techniques share the risk of tendon rupture. Skin tears are common after PNF or CCH injections.27,37 In some cases, skin tears can be sutured in a horizontal direction, allowing for tissue length to be maintained in a process called diamondplasty.38 Nerve injuries with PNF are rare (0.04%–0.6%).28,32 Other severe complications with CCH injections include tendon rupture and anaphylaxis. Mechanical neurapraxia, but not permanent nerve injury occurs with CCH as nerves are composed of type IV collagen and are spared by CCH treatments.
Comparison Studies of Different Treatments
LF versus PNF
A randomized controlled trial with 166 rays treated with either LF or PNF showed that total passive extension deficit was significantly better with LF than PNF (79% versus 63%).39 The rate of complications was higher in the LF group (5% versus 0%) (level of evidence (LOE): 1).39 The follow-up study showed a higher recurrence rate after 5 years in the PNF group (85% versus 21%), and it occurred sooner in the PNF group, with older age groups having a lower recurrence rate (LOE: II).17
PNF versus CCH
PNF is similar to CCH in results.40–42 One study compared PNF with CCH injections in 50 patients with MCPJ disease with a 3-year follow-up and found an initial correction of 100% versus 89%, a recurrence rate of 68% versus 83%, and no difference in the retreatment rate (LOE: 1).40 Another study compared PNF with CCH in 50 patients with PIPJ disease with a 3-year follow-up finding an initial correction of 67% versus 69% and a recurrence rate of 43% versus 34% (LOE: 1).41 CCH injection was not superior to PNF in the treatment of isolated PIPJ contractures regarding the clinical outcome, and it led to more mainly transient complications than PNF (LOE: I).41 Clinical improvement (reduction in contracture by 50% compared with baseline) was maintained in 29% of PNF patients versus 7% of CCH patients.41 A more extensive study compared PNF with CCH injection in 152 patients with MCPJ disease and 2-year follow-up, finding an initial correction of 91% versus 90% and a recurrence rate of 21% versus 24% (LOE: 1).42 There was no significant difference between CCH and PNF treating MCPJ contractures in range of motion, pain, or QuickDASH score.42 Regression and disappearance of the cords occurring in more than 50% of patients at two years follow-up may indicate that resorption of the pathological cord occurs when the tension in the DD cords is diminished.42 A study in 132 matched patients compared CCH with LF with a three-month follow-up in a nonrandomized study (LOE: III).24 The residual contracture at the MCPJ was 13 degrees for CCH versus 6 degrees for LF.24 PIPJ contractures showed a minor but significantly worse residual contracture than CCH (25 degrees versus 15 degrees with LF; ρ = 0.01).24 CCH patients had zero serious adverse events, but the LF group had three tenosynovitis cases and one nerve injury (ρ = 0.04).24
Treatment Algorithm and Patient Selection
The senior author feels that patients fall into a simple algorithm ladder of treatment. PNF or CCH injections would be the initial treatment. If recurrence is beyond two years, the initial treatment would be repeated. If recurrence is less than 2 years, an alternative would be offered. If the first treatment was PNF, CCH or LF would be offered. If the first treatment was CCH injections, PNF or LF would be offered. PNF or CCH injections can also be repeated. This technique allows for the successes of simple methods to be captured. Those with double failures of PNF and CCH injections would progress to an LF. Further recurrence would be treated with another LF or a dermofasciectomy. This algorithm allows those that achieve long-term success with minimal treatments to be captured.
Techniques and Pearls
Wide Awake Local Anesthetic No Tourniquet
Wide awake local anesthetic no tourniquet surgery for DD avoids the use of the arm tourniquet and a deeper level of anesthesia.34 For those beginning the procedure and having some trepidation, it is easy to add plain lidocaine to the mixture to dilute the epinephrine concentration in half (1: 200,000) or a fourth (1: 400,000) or even more. However, the epinephrine hemostatic effect wears off more rapidly with the more dilute solutions. Wide awake local anesthetic no tourniquet DD surgery is equivalent to general anesthesia or block anesthesia with a tourniquet.34,43 Although current general or regional block anesthesia as currently practiced is exceedingly safe, an extensive review of over 153,376 DD operations showed significant risks for LF and DF surgeries using these techniques versus strictly local anesthesia PNF.36 Serious systemic complications at 90 days after LF and DF included myocardial infarction (0.88%), acute kidney injury (0.09%), and lower respiratory tract infection (0.22%).36
PNF
Mild to severe extension deficits of the MCPJ or PIPJ is treatable with PNF. Apply topical ice and then place small intradermal wheals of 0.1 to 0.2 mL of 1% lidocaine with epinephrine using a 32-gauge needle to minimize pain for superficial dermal anesthesia. This same numbing technique is helpful for CCH injections. (See Video 1 [online], which displays the fast and straightforward PNF release of the MCPJ. The right little finger, not shown, had two previous fasciectomies elsewhere with a residual 70-degree PIPJ contracture.)
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Start distally on the cord with a 21 or 25-gauge needle. PNF generally uses a 5/8 inch 25-gauge needle attached to a 3-mL syringe filled with a local anesthetic. Larger bore needles such as a 21-gauge or 18-gauge needle are useful for thicker cords. Severe contractures need proximal release first to open up the digit. PIPJ contractures with narrow cords will do well with distal to proximal release. During the procedure, check for Tinel signs to prevent nerve injury. The cord is weakened with up and down cutting or pendulum motion using tension on the patient’s distal fingertip with the operator’s nondominant hand. Insert the needle while flexing and extending the digit to rule out a tendon impingement when in doubt. In general, release distal to proximal for PIPJ contractures. Proximal to the distal palmar crease, PNF is very safe even with a larger bore needle and a numb ray. After PNF, more generous digital blocks or joint anesthesia help reduce forceful extension pain. It is okay to perform hyperextension when straightening the MCPJ, but do not try to hyperextend the PIPJ because it can result in a swan neck deformity. To avoid tendon rupture, one can flex and extend the fingers with the needle in place to make sure you are treating a cord, not a tendon. The goal is to disconnect the nodules and cords and inject antifibrotic agents such as triamcinolone or fluorouracil into the divided tissue. Often, closed collateral ligament releases on one side due to a dominant radial or ulnar cord with an 18-gauge needle may be necessary for difficult PIPJ. Morhart and Stromberg (open access) have presented fine articles on PNF technique, and Eaton has an excellent video (See Video 2 [online], which displays the percutaneous needle aponeurotomy of a left ring finger Dupuytren cord with metacarpophalangeal and proximal interphalangeal joint contractures 45 and 65 degrees, respectively).44–46 Advanced PNF techniques are necessary for severe DD. (See Video 3 [online], which displays the advanced PNF technique treating a severe MCPJ and PIPJ contracture.)
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CCH
CCH injections can be used as the primary treatment or for recurrences after PNF, OF, LF, or DF. CCH injections are useful for lateral disease and retrovascular cords where PNF and LF treatment is more complicated (Fig. 5). When the CCH is reconstituted, the bottle cap is removed, and a 1.5-inch 25-gauge needle can easily remove the entire volume of CCH. Use 0.58 mg collagenase for the dominant cord. The residual 0.32 mg may be used more proximally or more distally on the affected cord, other cords in another ray, or on natatory ligaments. Manipulation at seven days is safe and effective (LOE: I).47 Local anesthesia, digital or wrist block for manipulation, helps immensely. One should stretch, massage the cords, and bend in all directions at manipulation. After a skin tear, it is possible to trim exposed Dupuytren cords with a sterile tray and loupe magnification (Fig. 6). CCH injections as an enzymatic tenotomy is helpful for treatment of Dupuytren boutonniere (Figs. 7, 8).48
Fig. 5.: CCH injections for severe, distal Dupuytren contracture. A, PIPJ of -70 degrees and DIPJ of -50 degrees. Three hand surgeons had stated only LF would work. Small doses of CCH 0.1 mg were placed at the PIPJ and DIPJ. B, Result after this one CCH treatment at 2-year follow-up.
Fig. 6.: Management of skin tear with exposed Dupuytren cord. A, Markings for CCH injections. B, Skin tear with exposed cord treated with sterile setup and excision of the exposed tissue. C, At 7-day follow-up.
Fig. 7.: Treatment with CCH injections in Dupuytren boutonniere deformity. A, Small finger with severe PIPJ flexion contracture and DIPJ hyperextension boutonniere. Markings before collagenase injection. “X” marks indicate injection sites for 0.1 mg, and circular dots indicate injection sites for 0.2 mg of CCH. B, Thirteen-month follow-up with PIPJ extension and DIPJ hyperextension improvement after two volar CCH sessions. There was only one CCH tenotomy injection.
Fig. 8.: Distal enzymatic tenotomy for Dupuytren boutonniere deformity. A, Limited distal flexion and injection site of 0.1 mg CCH tenotomy is marked X. B, Markedly improved extension and flexion of the DIPJ after two volar CCH injection sessions, but only one dorsal tenotomy treatment.
LF
Removal of thickened and diseased cords is the goal of LF. Detailed understanding of normal and pathologic anatomy is vital. Incision markings are generally zig-zag Bruner with a V to Y advancement or straight line incisions with Z-plasties. LF dissects the NV bundle off of the cord, being careful looking for a spiral nerve. Littler scissors and tenotomy scissors are helpful. A 15 c blade can be pushed and will cut cords under tension and not the softer NV bundle.49 Once the NV bundles are exposed, the central, spiral, lateral, and retrovascular cords may be safely cut away. The NV bundle may be proximally located or dissected distally beyond the PIPJ, where it is more superficial. Trickett et al showed the effects of sequential releasing of the variously named cords in 99 rays with DD undergoing limited fasciotomy.50 Excision of the central cord corrected 82% of MCPJ contracture and 44% of PIPJ contracture, whereas excision of spiral/lateral cord corrected an additional 12% of the contracture at MCPJ and 19% at PIPJ. The subsequent release of the retrovascular cord and accessory collateral ligament resulted in an extra 23% and 14% correction at PIPJ, showing the complexity of cord structures involved in DD and the need for the release of multiple different cord structures during LF. Look for a spiral nerve when dissecting in fatty areas and identify neurovascular structures before proceeding.51
The central cord has attachments to the skin at the PIPJ crease and into the A4 pulley.51 The retrovascular cord is the leading cause of distal interphalangeal joint (DIPJ) contracture, and it displaces the neurovascular bundle in a volar direction.49 The neurovascular bundle should be retracted medially to expose the retrovascular cord.49 After releasing the central cord, the MCPJ contracture is usually corrected, but residual PIPJ contracture is common.51 At the conclusion of a LF, the PIPJ must be evaluated for residual contracture after releasing the involved cords.33,51 Dissection of Cleland’s and Grayson’s ligaments tethering the skin and contracting the PIPJ. These attachments can be directly excised or released with an 18-gauge needle to untether the skin via subcision. Re-stretch the finger for a minute or two. If there is residual bounce-back, or if the correction is incomplete, progress to the next step. Open the flexor tendon sheath via a transverse incision at the distal end of the A2 pulley. Also, release any taut fascial fibers across the cruciate and A3 pulley. Stretch, then reassess. Divide the transverse retinacular ligament. Asses the volar plate and release the check-rein ligaments. Stretch, then reassess. Capsulotomy and release of the collateral ligaments. Stretch, then reassess. Release of the palmar plate up to its insertion at the middle phalanx base. This stepwise process corrected an average of 81 degrees PIPJ contracture to 29 degrees, an improvement of 64%, although 10% of the patients were worse and 3% were unchanged.33
Recurrence
All current DD treatments have issues with recurrence, and there is no clear definition of recurrence.52 When evaluating a recurrent DD patient, a digital Allen test is useful for assessing potential arterial problems. Collagenase dissolves both scar tissue and DD cords, so previous surgery for DD does not affect the efficacy or safety of CCH injections.53 Repeat LF for recurrent DD is as effective as the initial treatment despite a larger extension deficit (LOE: III).54 Combination therapy using initial wide-awake LF, then touch-up CCH injections can help provide long-term improvement (Fig. 9). Fat grafting held hope for reducing DD recurrence, but whereas percutaneous aponeurotomy with lipofilling (PALF) had good results at 1 year, at 5 years, the correction was less durable than those for LF.55 Further studies are needed to conclude if fat grating can prevent DD recurrence.
Fig. 9.: Use of touch-up CCH injections years later after a previous LF. A, Initial wide awake LF in 2006. B, Touch-up CCH injections (0.5 mg to little finger, 0.1 mg to ring, and 0.3 mg to middle finger) to the right hand in 2011. C, The same hand in 2020.
What Patients Should Know
There is a strong association between DD and older age, positive family history, male gender, diabetes mellitus, alcohol (more than three drinks a day), smoking, and manual work with vibration tools.56,57 Obesity protects against the development of DD.57 In a review of patients receiving DD treatment, about one-fifth had a history of trauma such as a previous surgery or fracture.58 Spontaneous regression can occur in 11% of patients with early DD.59 DD does not follow a linear progression to contracture.60 Millesi followed 150 patients with early signs of DD, finding that 9% showed progression at 1 year, 22% at 3 years, 39% at 5 years, and 48% at 6 or more years.60 In 113 patients with unilateral disease that required operation, the rates of DD progression were 19% at 1 year, 37% at 3–5 years, and 46.5% between 6 and 12 years.60
Those with DD need to know that there is hope with a variety of options available for treatment. The biggest trade-off in DD treatments is fast recovery with higher recurrence rates versus longer recovery with lower recurrence rates.
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