Part VI • Hand, Wrist, And Upper Extremity

Chapter 97: Dupuytren’S Disease

Questions: 10
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Question 1 of 10 ETIOLOGIC FACTORS IN DUPUYTREN’S DISEASE
Which one of the following is correct regarding Dupuytren’s disease?
A
It occurs almost exclusively in white males over the age of 40.
B
Excess alcohol intake has no association with its development.
C
Inheritance is normally autosomal recessive with variable penetrance.
D
The middle and index fingers are the most commonly affected digits.
E
No clear correlation exists between the use of vibration tools and cord development.
✓ Correct Answer: E. No clear correlation exists between the use of vibration tools and cord development.
Clinical Explanation

Dupuytren’s disease is a benign fibroproliferative disorder that occurs in the palmar fascia leading to nodules, cords, and contractures of the fingers. There is no proven correlation with vibration tools or other manual work in development of Dupuytren’s disease, although there are theories that a microtrauma mechanism may act to direct myofibroblast contracture. The link between alcohol intake and Dupuytren’s disease has been questioned over the years. A review by Hindocha et al1 concluded that alcohol excess is associated with the disease that is more likely to require surgical correction than conservative management, although the mechanism for this is unclear. A previous study by this group has shown that excess alcohol intake was associated with an increased severity of the disease.2 The inheritance of Dupuytren’s disease is thought to be autosomal dominant (not recessive) with variable penetrance. As better epidemiological data emerge, it is recognized that white men of Northern European descent are most commonly affected. The disease is also regularly encountered in women and in other ethnic groups such as Japanese individuals. There appears to be both a genetic and environmental influence on the condition. The ring and little fingers (not the middle and ring) are most commonly affected, followed by the middle finger. The thumb and index finger can be affected, but this is less common.

References:
  • 1. Hindocha S, McGrouther DA, Bayat A. Epidemiological evaluation of Dupuytren’s disease incidence and prevalence rates
  • in relation to etiology. Hand (N Y) 2009;4(3):256–269
  • 2. Hindocha S, Stanley JK, Watson JS, Bayat A. Revised Tubiana’s staging system for assessment of disease severity in Du-
  • puytren’s disease-preliminary clinical findings. Hand (N Y) 2008;3(2):80–86
Question 2 of 10 DUPUYTREN’S DIATHESIS
A 38-year-old patient presents to clinic with palpable nontender lumps in both palms affecting the ring finger and thumb. They have been present for 6 months and are now limiting his ability to fully open his hands. His father and grandfather had similar problems, and he would like to discuss treatment options with you. Which one of the following is correct?
A
All of the risk factors for Dupuytren’s diathesis are present.
B
This is a typical age of onset for Dupuytren’s contracture.
C
If he has sisters, they are unlikely to be affected.
D
His risk of recurrence after treatment is high.
E
Dorsal knuckle pads would be unusual in this patient.
✓ Correct Answer: D. His risk of recurrence after treatment is high.
Clinical Explanation

father and grandfather had similar problems, and he would like to discuss treatment options with you. Which The presence of multiple Dupuytren’s diathesis factors in a patient such as this man increases the risk of recurrent disease by up to approximately 70%, compared with a baseline recurrence risk of 20–25% in patients without diathesis factors.1 The initial description of Dupuytren’s diathesis by Hueston included four factors2: 1. Ethnicity 2. Family history 3. Bilateral disease 4. Ectopic lesions (outside the palm) The scenario gives only two of these factors. He may develop ectopic disease, such as dorsal knuckle pads (Garrod’s pads) and may also be affected by plantar or penile fibromatosis. Subsequent to Hueston’s description, additional items have been added by Hindocha et al1 who suggested two new factors: male sex and age at onset less than 50 years (for which this patient meets both criteria). These authors also proposed that the family history should include one or more affected siblings/parents and that only ectopic lesions in the knuckles (Garrod’s pads) should count. Finally, because inheritance is autosomal dominant, any of his siblings including sisters are also at risk, although penetrance of the phenotype is variable.

References:
  • 1. Hindocha S, Stanley JK, Watson S, Bayat A. Dupuytren’s diathesis revisited: evaluation of prognostic indicators for risk
  • of disease recurrence. J Hand Surg Am 2006;31(10): 1626–1634
  • 2. Hueston JT. Dupuytren’s Contracture. Edinburgh: E & S Livingstone; 1963
Question 3 of 10 STRUCTURES AFFECTED BY DUPUYTREN’S DISEASE
Which one of the following structures is typically spared in Dupuytren’s contracture?
A
Cleland’s ligament
B
Grayson’s ligament
C
Spiral cord
D
Lateral digital sheet
E
Natatory ligament
✓ Correct Answer: A. Cleland’s ligament
Clinical Explanation

Dupuytren’s disease affects much of the palmar fascia, but Cleland’s ligaments are usually spared in this condition. These fascial bands pass dorsal to the neurovascular bundles in the fingers from the periosteum and flexor tendon sheath to the lateral skin. Grayson’s ligament passes transversely from the volar aspect of the flexor tendon sheath to the skin. It prevents bowstringing of the neurovascular bundle during digital flexion. In Dupuytren’s disease Grayson’s ligament is often tightly adherent to the bundle, making dissection more challenging. The natatory ligaments are found just deep to the skin, extending transversely between the web spaces and, when involved in Dupuytren’s disease, may restrict both metacarpophalangeal (MCP) joint abduction and proximal interphalangeal (PIP) joint extension. The lateral digital sheet is located lateral to the neurovascular bundles within the digit and, when involved in Dupuytren’s disease, can restrict proximal interphalangeal (PIP) and distal interphalangeal (DIP) joint function.1–3

References:
  • 1. McFarlane RM. Patterns of the diseased fascia in the fingers in Dupuytren’s contracture. Displacement of the neurovas-
  • cular bundle. Plast Reconstr Surg 1974;54(1):31–44
  • 2. McFarlane RM. On the origin and spread of Dupuytren’s disease. J Hand Surg Am 2002;27(3):385–390
  • 3. Rayan GM. Palmar fascial complex anatomy and pathology in Dupuytren’s disease. Hand Clin 1999;15(1):73–86, vi–vii
Question 4 of 10 CORD COMPONENTS IN DUPUYTREN’S DISEASE
Your resident is presenting a case of Dupuytren’s disease to you in clinic and states that the patient has a “spiral cord” on examination. Which one of the following structures does not form part of the spiral cord?
A
Lateral digital sheet
B
Grayson’s ligament
C
Landsmeer’s ligament
D
Pretendinous band
E
Spiral band
✓ Correct Answer: C. Landsmeer’s ligament
Clinical Explanation

The presence of a spiral cord can complicate the surgical management of Dupuytren’s disease, as the pliable neurovascular bundle can become distorted around the shortened fibrous structures, spiraling around the disease as it tightens up. Great care must therefore be taken when dissecting the Dupuytren’s disease from the neurovascular bundle in the presence of a spiral cord, as the abnormal anatomy risks iatrogenic damage to the nerve and vessel which may be displaced to or beyond the midline of the digit. Spiral cords may cause flexion contractures of the PIP joint. The common components of a spiral cord are: • Lateral digital sheet • Pretendinous band • Spiral band • Grayson’s ligament • Natatory ligaments (included by many, but not all, authors) Landsmeer’s ligament is not part of the spiral cord. It is also known as the oblique retinacular ligament and originates on the volar aspect of the middle phalanx and inserts on the dorsal aspect of the distal phalanx. It helps coordinate PIP joint and DIP joint motion.1–3

References:
  • 1. Ritter MA. The anatomy and function of the palmar fascia. Hand 1973;5(3):263–267
  • 2. Rayan GM. Palmar fascial complex anatomy and pathology in Dupuytren’s disease. Hand Clin 1999;15(1):73–86, vi–vii
  • 3. Macey ARM, Thomas R. “The serpentine zone”: a surgeon’s guide to the surface anatomy of the digital neurovascular
  • spiral in Dupuytren’s contracture. J Hand Microsurg 2018;10(1):54–56
Question 5 of 10 INDICATIONS FOR SURGERY IN DUPUYTREN’S DISEASE
Which one of the following is most likely to be an indication for surgical intervention in Dupuytren’s disease?
A
A palpable spiral cord in a straight finger
B
The presence of Garrod’s pads
C
A PIP joint contracture of 10 degrees
D
An MCP joint contracture of 20 degrees
E
A negative Hueston’s test
✓ Correct Answer: C. A PIP joint contracture of 10 degrees
Clinical Explanation

Established flexion contractures of the PIP joint are the most difficult to correct because the volar structures shorten when the joint is left in flexion for a prolonged time. The MCP joint is far more forgiving, as the collateral ligaments are taut when the joint is flexed (i.e., the “safe” position for splinting). MCP joint contractures greater than 30 degrees and any PIP joint contracture are commonly quoted as indications for surgery, although lesser contractures of the MCP joint may be sufficiently troubling to warrant treatment, so the decision is tailored to the individual. Garrod’s pads are rarely an indication for surgery but may be so if they are symptomatic. A positive Hueston’s test is where the hand cannot be placed flat on a tabletop and may be an indication for surgery if there are associated functional problems.1–4

References:
  • 1. Smith AC. Diagnosis and indications for surgical treatment. Hand Clin 1991;7(4):635–642, discussion 643
  • 2. Rodrigues JN, Becker GW, Ball C, et al. Surgery for Dupuytren’s contracture of the fingers. Cochrane Database Syst Rev
  • 2015;(12):CD010143
  • 3. Watt AJ, Leclercq C. Management of Dupuytren’s disease. In: Neligan PC, Chang J, Van Beek AL, eds. Plastic Surgery, Vol.
  • 6 – Hand and Upper Extremity. London: Elsevier; 2013:346–362
  • 4. Hueston JT. The table top test. Hand 1982;14(1):100–103
Question 6 of 10 COLLAGENASE INJECTIONS FOR DUPUYTREN’S CONTRACTURE
A patient comes to clinic having read about “an enzyme injection therapy” for Dupuytren’s disease. He has unilateral disease with palpable pretendinous cords affecting the ring and little fingers. The flexion contractures are 40 degrees at the MCP joint and 10 degrees at the PIP joint. Which one of the following is correct regarding collagenase injections for this patient?
A
Even if full correction is achieved, he has a 75% chance of recurrence within 3 years.
B
Each cord will require two office visits, and both can be treated simultaneously.
C
This treatment will avoid the risk of developing a posttreatment wound.
D
His risk of flexor tendon rupture is approximately 5% following this treatment.
E
He is likely to develop antibodies to Streptococcus histolyticus spp. following this treatment.
✓ Correct Answer: B. Each cord will require two office visits, and both can be treated simultaneously.
Clinical Explanation

Collagenase injection for the treatment of Dupuytren’s disease has more recently become popular. The treatment involves injecting collagenase into a palpable cord and then allowing it to weaken before physically breaking the cord by external manipulation 24–48 hours later. Initial recommendations were that one cord should be treated at a time, but this has been relaxed and it is considered appropriate to treat the two cords simultaneously. Two office visits remain the minimum per cord, assuming that one injection and one manipulation is sufficient. Some cords require more than one injection/manipulation cycle. Recurrence is dependent on which joint is affected as well as by other factors including disease severity. At 5 years posttreatment, significant recurrence at the MCP joint is reported to be around 20–40%, while this is increased to 60–70% at the PIP joint.1–3 Collagenase treatment does not obviate any risk of a wound, as skin tears can commonly occur with postinjection manipulation. Therefore, all patients must be warned about wounds due to skin tears developing. These do tend to heal quickly with nonsurgical management and are not usually of great concern. The collagenase used in Dupuytren’s contracture is a blend of two clostridial collagenases, AUX I and AUX II, from Clostridium histolyticum (not Streptococcus). Streptococcus pyogenes, Staphylococcus aureus, and Clostridium perfringens all produce hyaluronidase (rather than collagenase) as a mechanism of penetrating soft tissues. Methods of producing hyaluronidase (Hyalase) have led to its use in local anesthesia and correction of hyaluronic acid facial fillers. All patients will have developed antibodies to the injection by their second injection, but this has not been associated with any adverse outcomes. Subsequent tendon rupture is extremely rare, with three reports in 2,600 injections performed within the Collagenase Option for Reduction of Dupuytren’s (CORD’s) clinical trials.1–4

References:
  • 1. Peimer CA, Blazar P, Coleman S, et al. Dupuytren contracture recurrence following treatment with collagenase clostrid-
  • ium histolyticum (CORDLESS study): 3-year data. J Hand Surg Am 2013;38(1):12–22
  • 2. Werlinrud JC, Hansen KL, Larsen S, Lauritsen J. Five-year results after collagenase treatment of Dupuytren disease. J
  • Hand Surg Eur Vol 2018;43(8):841–847
  • 3. Peimer CA, Blazar P, Coleman S, et al. Dupuytren contracture recurrence following treatment with collagenase clostrid-
  • ium hystolyticum (CORDLESS study): 5-year data. J Hand Surg Am 2015;40(8):1597–1605
  • 4. Hurst LC, Badalamente MA, Hentz VR, et al; CORD I Study Group. Injectable collagenase clostridium histolyticum for
  • Dupuytren’s contracture. N Engl J Med 2009;361(10):968–979
Question 7 of 10 THE IP JOINTS IN DUPUYTREN’S DISEASE
When planning a limited fasciectomy for a finger with Dupuytren’s causing a PIP joint contracture, which one of the following is correct?
A
If a boutonniere deformity is present it is likely that the PIP joint correction will be maintained.
B
A lazy-S volar incision is the preferred approach.
C
Contracture of the accessory collateral ligaments and volar plate at the MCP joint is common.
D
Correction of the PIP joint contracture may be limited by tolerance of the neurovascular bundles.
E
In severe PIP joint contracture, aggressive joint release gives a better long-term result than fasciectomy alone.
✓ Correct Answer: D. Correction of the PIP joint contracture may be limited by tolerance of the neurovascular bundles.
Clinical Explanation

Following a severe, prolonged contracture, the neurovascular bundles may be restricted and unable to tolerate full extension of the digit. The risk of vascular compromise is also increased in revision surgery. Although aggressive release of a contracted PIP joint capsule may give improved correction of the finger intraoperatively, this may be at the expense of joint stability, or result in a loss of flexion if care is not taken. Loss of flexion may be more troublesome than loss of extension for most functional activities. Many surgeons advocate careful serial release of the accessory collateral ligaments and volar plate to improve correction, but many would advise against a forced capsulotomy. The reported outcomes are mixed, with some series demonstrating greater early loss of correction with joint release, but comparable outcomes to fasciectomy alone in the longer term.1,2 A lazy-S incision is not recommended as it is difficult to deal with a skin deficit using local flaps with this incision pattern. Problems with the MCP joint volar plate or ligaments would be highly unusual. A boutonniere deformity indicates central slip attenuation, and a PIP joint correction is unlikely to be maintained without this being addressed.

References:
  • 1. Weinzweig N, Culver JE, Fleegler EJ. Severe contractures of the proximal interphalangeal joint in Dupuytren’s disease:
  • combined fasciectomy with capsuloligamentous release versus fasciectomy alone. Plast Reconstr Surg 1996;97(3):560–
  • 566, discussion 567
  • 2. Ritchie JF, Venu KM, Pillai K, Yanni DH. Proximal interphalangeal joint release in Dupuytren’s disease of the little finger.
  • J Hand Surg [Br] 2004;29(1):15–17
Question 8 of 10 TREATMENT OPTIONS FOR DUPUYTREN’S CONTRACTURE
A 55-year-old man has Dupuytren’s contractures affecting the ring and little fingers of his right hand. Both MCP joints have contractures of 45 degrees and the ring finger PIP joint has a contracture of 20 degrees. There are pretendinous and spiral cords. Which one of the following statements is correct?
A
Reported recurrence rates after radical fasciectomy are less than 10% at 5 years.
B
The risk of neurovascular injury during surgery is minimal, given the anatomy of his disease.
C
Needle fasciotomy may achieve complete correction, but recurrence would be more likely than with fasciectomy.
D
Because both MCP and PIP joints are affected, a primary dermofasciectomy is recommended.
E
The PIP joint is unlikely to be fully corrected with limited fasciotomy.
✓ Correct Answer: C. Needle fasciotomy may achieve complete correction, but recurrence would be more likely than with fasciectomy.
Clinical Explanation

joints have contractures of 45 degrees and the ring finger PIP joint has a contracture of 20 degrees. There are fasciectomy. There are variable recurrence rates reported worldwide following treatment of Dupuytren’s disease, and confusion arises because there is debate about whether any recurrence of disease is included in outcome figures, or whether only recurrent disease requiring further treatment should be included.1,2 Despite these variations, it is generally accepted that needle fasciotomy shows earlier recurrence than fasciectomy. However, the trade-off of greater downtime after fasciectomy means that many surgeons and patients find needle fasciotomy preferable. Some surgeons do report comparable outcome figures with extensive needle fasciotomy. Reported recurrence rates after limited fasciectomy are in the order of 21% at 5 years, compared with 85% for needle fasciotomy.3 Dermofasciectomy is usually reserved for cases with extensive skin involvement or for aggressive or recurrent disease. It is most often required over the proximal phalanx. Wounds are closed with skin grafts or left to heal by secondary intention. Radical fasciotomy involves extensive resection of volar and digital fascia including diseased and nondiseased tissue. It does not decrease the recurrence rate and carries higher rates of complication than limited fasciectomy. In this scenario, the PIP joint contractures are mild, and a full correction is likely whichever technique is performed.

References:
  • 1. Crean SM, Gerber RA, Le Graverand MP, Boyd DM, Cappelleri JC. The efficacy and safety of fasciectomy and fasciot-
  • omy for Dupuytren’s contracture in European patients: a structured review of published studies. J Hand Surg Eur Vol
  • 2011;36(5):396–407
  • 2. Werker PM, Pess GM, van Rijssen AL, Denkler K. Correction of contracture and recurrence rates of Dupuytren contracture
  • following invasive treatment: the importance of clear definitions. J Hand Surg Am 2012;37(10):2095–2105.e7
  • 3. van Rijssen AL, Ter Linden H, Werker PMN. Five-year results of a randomized clinical trial on treatment in Dupuytren’s
  • disease: percutaneous needle fasciotomy versus limited fasciectomy. Plast Reconstr Surg 2012;129(2):469–477
Question 9 of 10 SURGICAL PRINCIPLES IN FASCIECTOMY
When undertaking a fasciectomy for Dupuytren’s disease, which one of the following is true?
A
Skin flaps are best designed with a narrow base to optimize movement.
B
Dissection should start in the palm and proceed distally.
C
All palmar fascia should normally be removed.
D
Wounds should always be fully closed or otherwise skin grafted.
E
A tourniquet must remain inflated until the wounds are closed.
✓ Correct Answer: B. Dissection should start in the palm and proceed distally.
Clinical Explanation

When undertaking a fasciectomy for Dupuytren’s disease, dissection should start in the palm to safely identify the neurovascular bundles. Skin flaps are elevated to expose the normal and abnormal palmar fascia. In the palm the nerves and vessels will be deep to this fascia and, once identified, they can be traced distally under direct vision. As dissection proceeds distally into the digits, the course of the nerves and vessels may be abnormal with them encased in and distorted by disease. The risk of iatrogenic damage is minimized using this approach. When designing skin flaps in the palm and digits, a balance must be made between narrow bases that reduce flap vascularity and wide bases that do not transpose well. Z-plasties in the palm need to be small, as there is little movement here, whereas larger Z-plasty flaps can work over the proximal phalanx. Skin flap thickness is carefully judged to minimize residual disease attached to skin while maintaining vascularity. Where there is obvious involvement of disease with the overlying skin, dermofasciectomy should be performed. Only diseased fascia needs to be removed, as there is no benefit in radical fasciectomy, which is now only of historical interest. Complete wound closure on the palm is not necessary and in fact, leaving areas to heal by secondary intent works very well. Grafts may, however, be preferred in larger defects in the digits. Tourniquet release (if used) before wound closure allows assessment of digit vascularity and hemostasis, and reduces the risk of hematoma. Vascularity of each digit must always be checked and if the finger is white secondary to tension on the contracted digital vessels, then splinting the finger in the least amount of flexion that allows it to be pink is advocated. In clinic, extension can be progressively increased over 1–2 weeks.1,2

References:
  • 1. Watt AJ, Leclercq C. Management of Dupuytren’s disease. In: Neligan PC, Chang J, Van Beek AL, eds. Plastic surgery, Vol.
  • 6 – Hand and Upper Extremity. London: Elsevier; 2013:346–362
  • 2. Rodrigues JN, Becker GW, Ball C, et al. Surgery for Dupuytren’s contracture of the fingers. Cochrane Database Syst Rev
  • 2015;(12):CD010143
Question 10 of 10 COMPLICATIONS FOLLOWING FASCIECTOMY FOR DUPUYTREN’S
Following surgery for Dupuytren’s disease, a patient develops a disproportionate amount of swelling, redness, and stiffness to the hand in the absence of any signs of clinical infection. Which one of the following should be initiated in this scenario?
A
Splinting in the safe position
B
Oral antibiotics
C
Plan to return to theater
D
A regional block
E
Steroid and gabapentin
✓ Correct Answer: E. Steroid and gabapentin
Clinical Explanation

The patient in this scenario is displaying signs of a Dupuytren’s flare, which is sudden-onset development of swelling, redness, and stiffness to the operated hand similar to, or a subtype of, complex regional pain syndrome. This tends to occur toward the end of the first month following surgery after an apparently normal early healing response. At week 3 or 4 postsurgery, there is typically a deterioration in rehabilitation progression and function which may be linked in some way to therapy and splinting regimens. The key steps to take are early and frequent hand therapy (not immobilization, which will lead to additional stiffness), together with methylprednisolone (Medrol) and gabapentin (Neurontin). Regular nonsteroidal medications may also be useful where not contraindicated. Sympathetic blockade (stellate ganglion block) may be helpful, rather than a regional nerve anesthetic block. Antibiotics are indicated only in cases where there is evidence of active infection with wound healing issues or erythema and cellulitis. Likewise, a return to theater would only be indicated in this scenario if underlying infection with an abscess requiring drainage was suspected.1,2

References:
  • 1. Rivlin M, Osterman M, Jacoby SM, Skirven T, Ukomadu U, Osterman AL. The incidence of postoperative flare reaction
  • and tissue complications in Dupuytren’s disease using tension-free immobilization. Hand (N Y) 2014;9(4):459–465
  • 2. Prosser R, Conolly WB. Complications following surgical treatment for Dupuytren’s contracture. J Hand Ther
  • 1996;9(4):344–348

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