Dupuytren Disease Case File

Dupuytren Disease Case File

Eugene C. Toy, MD, Andrew J. Rosenbaum, MD, Timothy T. Roberts, MD, Joshua S. Dines, MD

CASE 31

A 64-year-old, right-hand dominant male is referred to your office with complaints of progressive hand stiffness and deformity over the past 2 years. The small finger of the right hand is most affected. The left hand is also affected, but not as severe. He denies any history of trauma or previous hand problems, and he has no complaints of pain, nor finger locking or clicking. His father had similar problems with his hands.

    The patient’s past medical history includes high cholesterol and blood pressure. More than 5 years ago, he underwent surgery for early-stage colon cancer, and since then has been cancer-free. He currently does not drink alcohol, although he drank heavily during his younger years. He is of English-Irish decent.

    Examination of both hands reveals nodules and skin thickening in the palm along the small finger ray. The right small finger metacarpophalangeal (MCP) joint is fixed at 45 degrees of flexion, and the proximal interphalangeal (PIP) joint is fixed at 20 degrees. There is no redness, tenderness, edema, or pain with passive flexion. The left hand has a nodule and cord with a resting flexion contracture of the small finger of 20 degrees that is improved with passive extension. Additionally, examination of his left foot reveals nontender nodules along the first ray proximal to the metatarsal head.

► What is the most likely diagnosis?

► What is the most appropriate treatment for this patient?

ANSWER TO CASE 31:

Dupuytren Disease                             

Summary: This right-hand dominant, 64-year-old male patient has a progressive soft tissue condition leading to digital flexion deformity or contracture. The stiffness is associated with decreased functionality. The contracture in the right hand is not passively correctible.

• Most likely diagnosis: Dupuytren disease.
• Most appropriate treatment: Collagenase injection(s), needle aponeurotomy, or  surgical fasciotomies, depending on the extent of disease and surgeon preference.

ANALYSIS

Objectives

1. Understand the clinical presentation, etiology, risk factors, anatomic features, and pathoanatomy of Dupuytren disease.
2. Be familiar with basic treatment options and associated complications.
3. Identify criteria to guide choice of treatment.
4. Recognize prognostic factors for recurrence and poor outcome.

Considerations

This patient presents with progressive right small finger stiffness and deformity. His MCP joint is fixed in 45 degrees of flexion and his PIP joint is fixed in 20 degrees of flexion. His deformity (see Figure 31–1 ) is most obvious as he tries to extend all his fingers. Furthermore, the patient denies any locking symptoms, and there is no redness, tenderness, edema, or pain with passive flexion. The differential diagnosis for digital flexion contractures includes Dupuytren disease, trigger finger(s), stenosing tenosynovitis, ganglion cysts, soft-tissue masses, hyperkeratosis, intrinsic joint disease, and traumatic scarring. Despite this broad differential, there are several components of this patient’s presentation that favor a diagnosis of Dupuytren disease. These include a family history of similar pathology, a history of heavy alcohol use, being of Northern European decent, and presence of similar, nontender nodules in his left foot. After the clinical diagnosis of Dupuytren disease is made, treatment options can be considered, including nonoperative and operative modalities.

Figure 31–1. Dupuytren disease of the small finger. Note the MCP and PIP flexion contracture as the

patient attempts to extend his fingers.

APPROACH TO:

Dupuytren Disease                                 

DEFINITIONS

DUPUYTREN DISEASE: A benign fibroproliferative disorder involving the palmar and digital fascia.

SPIRAL CORD: An arrangement of abnormal fibrous tissue associated with PIP joint flexion contractures. Spiral cords can displace digital neurovascular bundles centrally and volarly (palmar superficially), making them especially prone to injury during surgical release.

DUPUYTREN DIATHESIS: Refers to patients with an aggressive form of the disease, manifesting with early onset, rapid progression, frequent bilaterality, and/or involvement of the radial digits.

CLINICAL APPROACH

Etiology and Anatomic Features

Dupuytren disease is a benign fibroproliferative disorder involving the fascia of the hand and fingers, most commonly involving the ring and small fingers. It can present locally as a nodule in the palm or a dimple in the skin crease. Dupuytrens may then progress distally to involve the joints of the fingers.

    The exact etiology remains unclear, although a strong genetic predisposition exists. It occurs more commonly in males of northern European descent. Dupuytren disease has been associated with tobacco and alcohol use, diabetes, epilepsy, chronic pulmonary disease, prior myocardial infarction, human immunodeficiency virus (HIV) infection, trauma, and tuberculosis. However, idiopathic presentation is most common.

    The pathophysiology of Dupuytren disease is not completely understood, but has been found to involve abnormal proliferations of myofibroblasts, cells normally responsible for producing contractile scar tissue when the body attempts to close a wound. In Dupuytrens, hyperactive myofibroblasts deposit disproportionate quantities of extracellular collagen that form collections of fibrous bundles. Additionally, the deposited fibrous matrix is itself abnormal, consisting of greater concentrations of relatively thick and immature collagen type III, versus the mature collagen type I that is found in normal fascia. Dupuytren disease develops in 3 distinct stages: (1) the proliferative stage, in which abnormal numbers of oversized myofibroblasts are produced, (2) the involutional stage, in which abnormal concentrations and quantities of collagen type III are deposited, and (3) the residual stage, in which the myofibroblasts disappear, leaving behind their dense fibrous deposits. Simply put, Dupuytrens results from a pathological triggering of tissue healing in the absence of inciting trauma. Thick scar-like deposits of abnormal collagen coalesce into the characteristic Dupuytren nodules and cords.

    Structural anomalies result from contraction of the collagenous palmar fascia and overlying skin and fingers. Normal structures involved in the disease process include the pretendinous band, natatory band, spiral band, Grayson ligament, retrovascular band, and lateral digital sheet. These normal structures, or bands, when diseased, become cords and are thereafter referred to as the pretendinous cord, central cord, lateral cord, spiral cord, abductor digiti minimi cord, and intercommissural cord of the first web space. Spiral cords lead to PIP contractures and receive special attention for putting the neurovascular bundle at risk by displacing it more centrally and volarly as the PIP joint contracture increases.

    The natural history and risk for progression for a single individual remain indeterminable. Patients with Dupuytren diathesis have a more aggressive form of the disease that is associated with onset before age 40 years, bilateral hand involvement, thumb and index finger contractures, and fibrotic disease of the feet and penis. Patients need to be informed that recurrence is as high as 40% to 50%. When the fibroproliferative disease results in permanent curvature of the penis, it is known as Peyronie disease; similarly, if it involves the plantar foot, it is known as Ledderhose disease.

Clinical Presentation and Evaluation

Patients may present early or with mild forms that manifest as a tender palmar nodule (arising over a pretendinous band), skin dimpling, or a palpable cord. Classic features include fixed finger flexion deformities that impair function of daily living or work and indicate more advanced disease. There can be fixed flexion of either or both the MCP and PIP joints. (The distal interphalangeal joints are rarely involved and, when they are, are believed to result from formation of the retrovascular cord.) Disease involvement occurs most often in the ring and little fingers, with decreasing order of frequency in the long, thumb, and index digits. It is bilateral in 45% of patients.

    Dupuytren disease is diagnosed clinically. Flexion contractures are rarely subtle. MCP flexion may result from a pretendinous cord or a spiral cord. Limitation in finger abduction is frequently present and results from involvement of the web space and the presence of a natatory cord. Fixed flexion of the PIP joint may arise from contracture of the central cord that continues from a pretendinous cord in the palm that extends into the finger. Involvement of the thumb may manifest as contracture of the web space in addition to a fixed flexion contracture.

TREATMENT

The indications for surgery include functional impairment such as inability to wear a glove, reach into pockets, or securely hold or grasp objects. Physical exam criteria include a positive tabletop test (the inability to place the hand flat on a tabletop), MCP flexion contracture of greater than 30 degrees, and any PIP flexion contracture.

    Different surgical techniques have been described. These include percutaneous needle aponeurotomy, limited fasciectomy, and total palmar fasciectomy. Total palmar fasciectomy has recently fallen from favor because recurrence rates after surgery were no better than those of less extensive procedures and it was associated with higher rates of wound complications. The open-palm technique of McCash leaves the wounds open after surgery, resulting in diminished edema and hematoma formation and allowing for early postoperative motion. This historic technique was once preferred for older patients at increased risk for stiffness; but because of wound complications and poor acceptance on the part of the patient, this technique has since been abandoned. Skin coverage deficits are not uncommon after surgical treatment and may be addressed with skin grafting, Z-plasty, or healing by secondary intention.

    Nonoperative treatment of Dupuytren disease is evolving. In early stages, corticosteroid injections may provide some relief by softening and flattening prominent or painful nodules. For more advanced disease, an injectable collagenase (clostridial collagenase histolyticum or Xiaflex, which was approved by the US Food and Drug Administration in 2010) is receiving increasing popularity. This drug has been shown to chemically dissolve the Dupuytren’s cord, allowing for the subsequent manipulation necessary to mechanically disrupt the contracted tissue and correct the flexion deformity.

Outcomes and Complications

The most common complications after collagenase injection include edema, bruising, and redness localized to the site of injection. More serious but less frequent complications include flexor tendon rupture or neurovascular injury.

    The most common complication after operative treatment is recurrence, with long-term rates as high as 40% to 50%. Attention to postoperative therapy with active range of motion and splinting is a major determinant of improved outcomes. Potential complications include infection, digital neurovascular injury, complex regional pain syndrome, hematoma, skin loss, and amputation.

COMPREHENSION QUESTIONS

31.1 A right-hand dominant 48-year-old woman presents with worsening hand stiffness and deformity. On exam, you note nodules and skin thickening in the palm along the small finger ray. You ultimately diagnose the patient with Dupuytren disease and begin discussing the specifics of this condition with her. Which of the following statements regarding Dupuytren disease is true?

A. Dupuytren disease is rarely bilateral.

B. Dupuytren disease is associated with pain.

C. Females are at greater risk than males.

D. Recurrence after surgery is as high as 50%.

E. Rapidly progressive disease suggests malignancy.

31.2 Which of the following patients with Dupuytren contracture would benefit the most from total palmar fasciectomy?

A. 50-year-old police officer with ring and small finger involvement of only the MCP joints

B. 70-year-old sedentary male with small finger involvement including the MCP and PIP joints

C. 45-year-old female tennis player with ring and small finger involvement including MCP and PIP joints

D. None of the above, as total palmar fasciectomy has fallen out of popularity due to its associated recurrence rates and complications, as opposed to regional, or limited fasciectomy

31.3 A 40-year-old man presents to your hand clinic with a diagnosis of Dupuytren disease involving his left small finger. The patient was given this diagnosis last week while at a routine follow-up appointment with your partner for his right total hip arthroplasty. Your partner felt that you, a hand surgeon, would be best equipped to manage the Dupuytren. After receiving this diagnosis, the patient did an internet search on this condition and comes in today inquiring about treatment with Xiaflex. Which of the following statements regarding Xiaflex is most accurate?

A. This drug has been shown to chemically dissolve the Dupuytren cord.

B. Xiaflex is a time-tested and effective means of treating Dupuytren disease.

C. Xiaflex is great because it has no known side effects.

D. Xiaflex can be purchased as an over-the-counter medication.

ANSWERS

31.1 D. Patients with Dupuytren disease should be counseled about the high risk for recurrence after surgery. Disease is frequently bilateral, most common in middle-aged men of northern European descent, and not histologically malignant, although patients with Dupuytren diathesis or with a genetic predisposition may have a more clinically aggressive form with rapid progression and high recurrence.

31.2 D. As stated in D, total palmar fasciectomy has fallen out of popularity as a result of its associated recurrence rates and complications, as opposed to regional, or limited fasciectomy.

31.3 A. Xiaflex has been shown to chemically dissolve the Dupuytren cord, allowing for the subsequent manipulation necessary to mechanically disrupt the contracted tissue and correct the flexion deformity. Although data are promising, Xiaflex was only approved by the US Food and Drug Administration in 2010. Serious side effects include tendon rupture, ligament damage, nerve injury, and allergic reactions. Common side effects include swelling, bleeding, bruising, pain, and/or tenderness at the injection site or hand.

    CLINICAL PEARLS    

► Dupuytren disease is a clinical diagnosis, and imaging or laboratory tests are needed only to assess or rule out other incidental conditions. ► Treatment is indicated when the fixed contractures interfere with hand function; standard criteria to provide treatment include the contractures of the MCP of 30 degrees and any degree of fixed PIP flexion, but decision to treat should be tailored to the patient. ► Patients with aggressive disease should be warned that there is high recurrence after treatment. ► Patients should be informed of irreversible complications from treatment including flexor tendon rupture, wound complications (eg, hematoma, infection), and injury to neurovascular structures.

REFERENCES

Desai SS, Hentz VR. The treatment of Dupuytren’s disease. J Hand Surg Am. 2011;36:936-942. 

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;361:968-979. 

Miller MD. Review of Orthopaedics. 5th ed. Philadelphia: Saunders Elsevier; 2008. 

Terek RM, Jiranek WA, Goldberg MJ, Wolfe HJ, Alman BA. The expression of platelet-derived growthfactor gene in Dupuytren’s contracture. J Bone Joint Surg Am. 1995;77:1-9.

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