Contracture, Dupuytren (Dupuytrens Contracture)

Article Author:
Joel Walthall
Article Editor:
Uzma Rehman
Updated:
8/30/2018 4:26:52 PM
PubMed Link:
Contracture, Dupuytren (Dupuytrens Contracture)

Introduction

Dupuytren disease is predominantly a myofibroblastic disease that affects the hand/fingers and results in contracture deformities. The most commonly affected digits are the third (ring) and fourth (small or pinky) digits. The disease begins in the palm as painless nodules that form along longitudinal lines of tension. The nodules form cords that produce contracture deformities within fascial bands and tissues of the hand. [1]

Etiology

Dupuytren disease is an autosomal dominant condition that is associated with diabetes, seizure disorders, smoking, alcoholism, HIV, and vascular disease.[2] Occupation and activities have not been shown to be risk factors. Ectopic manifestations beyond the hand can be seen in Ledderhose disease (plantar fascia), 10% to 30%; Peyronie disease (Dartos fascia of the penis), 2% to 8%; and Garrod disease (dorsal knuckle pads), 40% to 50%.

Epidemiology

This condition is most commonly seen in populations of Northern European/Scandinavian descent.[3] It is relatively uncommon in Southern European and South American populations and is rare in Africans and Asians. Males are affected in a 2:1 ratio compared to women, with the disease also affecting men more severely. Younger age of onset is also associated with increased severity of disease progression.

Pathophysiology

The pathophysiology of Dupuytren disease involves abnormal myofibroblastic growth in the hand. Type III collagen predominates, which under a nondisease state would be Type I collagen. The numerous cytokines involved include interleukin-1, transforming growth factor beta 1, transforming growth factor beta-2, epidermal growth factor, platelet-derived growth factor, and connective tissue growth factor. Dupuytren contracture progresses through three phases: (1) proliferative, (2) involution, and (3) residual. The proliferative phase has a characteristically high concentration of immature myofibroblasts and fibroblasts arranged in a whorled pattern. In the involution phase, fibroblasts become aligned in the longitudinal axis of the hand following lines of tension. In the residual phase, relatively acellular collagen-rich chords remain causing contracture deformity. 

Several cords can develop which can cause unique deformities of the hand. Pretendinous cords cause skin pitting and metacarpal phalangeal (MCP) joint contracture. Natatory cords are responsible for web space contractures. Spiral cords are the most important in the disease process and can cause proximal interphalangeal (PIP) contracture. A spiral cord originates from four main structures: (1) pretendinous band, (2) spiral bands, (3) Lateral digital sheets, and (4) Grayson ligaments.  The spiral cord also causes displacement of the neurovascular bundle centrally, superficially, and proximally.[4] Of note, the Cleland ligament and the transverse ligament of the palmar aponeurosis are not involved in Dupuytren disease.

Risk factors for increased severity and recurrence of disease after treatment include male gender, onset before age 50, bilateral disease, sibling/parent involvement, or the presence of Garrod pads, Ledderhose, or Peyronies diseases.

Histopathology

 A histologic analysis reveals myofibroblasts and fibroblasts.[5] Myofibroblasts have contractile actin microfilaments which align with the long axis of the cell. Myofibroblasts interconnect themselves with fibronectin and extracellular fibrils. Type III collagen predominates in the extracellular matrix.

History and Physical

The patient typically presents with a loss of range of motion of the hand and palpable cords in the palm extending into the affected digits. If presenting early in onset, they may only notice the palpable cord.  The tabletop test (Hueston) is performed by having the patient attempt to place the palm flat on the exam table. If there is any flexion contracture deformity, the patient will be unable to straighten the fingers, resulting in a positive test. [6]

Evaluation

X-rays of the hand should be obtained to examine for other contributing, bony abnormalities that may contribute to the loss of range of motion.

Treatment / Management

Indications for treatment are based on the effects of disease on the patient's quality of life. Many patients with a positive tabletop test, MCP contracture of 30 degrees, or PIP contracture of 15 to 20 degrees will elect to have treatment.

Treatment options consist of needle aponeurotomy, collagenase injection, and/or surgical resection and fasciectomy.[7] 

Needle aponeurotomy is typically reserved for mild contractures. The procedure is minimally invasive and is often performed in an office setting. A needle is used to break up the cord. The digit is then manipulated, and the patient wears a night extension splint.  Metacarpal-phalangeal joint contractures have the greatest improvement. The benefit of this procedure is that it is minimally invasive and has the potential for immediate results. Disadvantages include iatrogenic injury and high recurrence rates.

Collagenase injections provide a minimally invasive treatment derived from Clostridium Histolyticum. The injected enzyme is a metalloprotease that lyses collagen (sparing Type IV collagen which is needed in the basement membrane of blood vessels and nerves). Treatment typically consists of 0.25 mL for MCP and 0.20 mL for PIP contractures delivered subcutaneously directly into the cord with a needle. The affected digit is manipulated under local anesthesia at 24 to 48 hours after injection. Night extension splinting is maintained for 6 months. Collagenase injections have been shown to result in 75% contracture reduction with a 35% recurrence rate. Complications of injections include edema, skin tearing, tendon rupture, complex regional pain syndrome, and pulley rupture.

Surgical fasciectomy can be either limited or radical. Partial palmar fasciectomy entails the limited resection of diseased tissue within a ray.  Dissection generally is performed from proximal to distal as this usually allows for identification of known structures before encountering the spiral cord. Incisions are customized to each patient in a Brunner zigzag pattern. Also, V-Y incision and Z-plasty can be used to lengthen contracted skin. Care must be taken to preserve the neurovascular bundles. Recurrence rate at 1 to 2 years is 30%, 15% at 3 to 5 years, and less than 10% after ten years.

Total palmar fasciectomy can also be performed but is infrequently used as it requires resection of all palmar and digital fascia, including nondiseased tissue.

Complications of fasciectomy include skin necrosis, hematoma (most common complication), flare reaction, neurovascular injury, digital ischemia, swelling, and infection. [8]

Differential Diagnosis

Dupuytren disease should be distinguished from other diseases of the hand including stenosing flexor tenosynovitis, ganglion cysts, and soft tissue masses.

Complications

Complications of surgical treatment include wound edge necrosis, hematoma, nerve injury, digital ischemia, infection, swelling, recurrence, and postoperative flare. Digital ischemia can be due to direct damage to the vascular supply of the digit or because of stretch injury to vessels in digits with a history of prolonged flexion contracture.

Dupuytren flare reaction is pain with diffuse swelling, hyperesthesia, redness, and stiffness. Treatment of this complication consists of steroids, cervical sympathetic block, therapy, and A1 pulley release. There is no increased risk of complex regional pain syndrome (CRPS) if carpal tunnel release is performed with fasciectomy. [9]

Postoperative and Rehabilitation Care

Postoperatively, patients are entered into hand therapy to help maintain the range of motion of the hand. Extension splints often are used in conjunction with other modalities.