DUPUYTREN’S CONTRACTURE
Description
References
Dupuytren’s contracture consists of progressive scarring and thickening of the
connective tissue (most commonly in the palm of the hand and most commonly on the
ring and pinky fingers side) that causes the fingers to remain in a flexed position and
prevents their extension.
The appearance of Dupuytren contracture on exam is that of palmar thickening. The
thickening may be nodular (and thus mistaken for a callus) or cord-like. This process
typically involves the longitudinal and vertical components of the fascia but at times
seems to exist apart from anatomically distinct fascia. The skin overlying the sclerosed
fascia may fuse with it and become raised, or it may atrophy, acquiring a puckered
appearance.
Young, DM and Hansen,
SL. 2010. CURRENT
Diagnosis & Treatment:
Surgery, 13e; Doherty GM
ed. Ch. 42: Hand Surgery.
The McGrw Hills
Company; New York.
The histopathology of the disease changes as the condition progresses. Early disease
shows nodules with a predominance of fibroblasts and type III collagen. A contractile
phase follows, during which fibroblasts are replaced by myofibroblasts and
lymphocytes infiltrate the tissue. During this phase, the concentration of proteoglycans Moermans, JP. 1997.
greatly increases. Advanced disease is relatively acellular with a predominance of type I Place of Segmental
Aponeurectomy in the
collagen in tendonlike cords.
Pathology
(organ, cell,
system)
The table below describes the microscopic changes accompanying the evolution of
Dupuytren’s contracture:
Treatment of
Dupuytren's disease;
Chapter 6:
Histopathology. PhD
Thesis. University of
Bruxelles
Balaguer, T et al. 2009.
Histological staging and
Dupuytren's disease
recurrence or extension
after surgical treatment:
a retrospective study of
124 patients. European
Journal of Hand Surgery.
34:493-6.
I speculate that in the beginning, an idiopathic over-proliferation of fibroblasts begins
1
the process of scarring and contraction. The hand, exposed to unusual forces and non physiological traction of the phalanges, likely develops reactive inflammation (hence
the lymphocytic infiltration of the intermediate-stage lesions) and consequently
contributes to the activation of scar tissue deposition, acting synergistically with the
initial idiopathic disease process
Of note, histological staging is a reliable method for predicting recurrence.
Pathophysiology
Like all fascia, the palmar fascia contains a population of resident fibroblasts that allow
for connective tissue renewal. The pathophysiological process underlying Dupuytren
contracture is fibroblast proliferation and collagen deposition. Though it is not known
why this uncontrolled proliferation of palmar fascia occurs, it is thought to be related
increased expression of growth factors, including basic fibroblast growth factor,
platelet-derived growth factor, and transforming growth factor-beta within the
diseased fascia.
Differential
Diagnosis
The differential for Dupuytren disease includes several other conditions that affect the
hand, including trigger finger, palmar tendinitis, stenosing tenosynovitis, a ganglion
cyst, or a soft-tissue mass. Unlike Dupuytren contracture, trigger finger typically
involves pain with flexion followed by the inability to extend the affected digit.
Stenosing tenosynovitis, unlike Dupuytren disease, is also characterized by pain (as well
as a history of overuse or trauma). A small, movable nodule that is tender to palpation
at the metacarpophalangeal (MCP) joint is likely a ganglion cyst (unlike a more fixed
fibrous nodule of Dupuytren’s) A soft-tissue mass must also be excluded from the
diagnosis, especially if the patient is significantly younger than the typical patient with
Dupuytren disease: patient younger than age 40 years without involvement of the
dorsal hand, foot, or penis is unlikely to have Dupuytren disease, and the concerning
possibility of a sarcoma must be ruled out—although the pathologic findings of a
biopsy will most likely reveal a benign etiology (eg, lipoma, inclusion cyst). A firm scar
may also be a simple overuse callus – though the history would likely point to it. The
more advanced phase may be confused with a “hand of benediction” (neuropathic
claw hand conditions caused by median or ulnar nerve injuries). Median claw hand will
show hyper-extension of the MCP and flexion at the IP joints of the 2nd and 3rd digits
(index and middle). Ulnar claw hand will show hyper-extension of the (MCP) and
flexion at the distal and proximal (IP) joints of the 4th and 5th digits (ring and little
finger). In both, clawing will become most obvious during finger extension. Unlike
median and ulnar claw hads, in Dupuytren’s the MCP joints are also flexed; also, the
muscles have normal strength but are limited by the scarring, unlike in the
neuropathies aforementioned, in which the issue is weakness (and only over time may
develop some degree of stiffness due to atrophy).
Revis, DR, Dupuytren’s
Contracture. E-Medicine
Rheumatology:
http://emedicine.medsca
pe.com/article/329414overview, accessed
9/22/2010
Revis, DR, Dupuytren’s
Contracture. E-Medicine
Rheumatology:
http://emedicine.medsca
pe.com/article/329414overview, accessed
9/22/2010
Lee, S Baytion, M, Reinke,
DL, Bogdan, Y. Dupuytren
Contracture. E-Medicine
Orthopaedics:
http://emedicine.medsca
pe.com/article/1238712overview, accessed
9/23/2010
2
Epidemiology
The prevalence of Dupuytren’s contracture varies according to the ethic makeup of a
given country, as Northern European descent confers a predisposition to developing
the disease. Dupuytren contracture is very common in northern Europe and the United
Kingdom and in countries inhabited by immigrants from these areas (eg, Australia,
Canada, United States). In the United Statesm, A 10-year retrospective study using the
Department of Veterans Affairs computer system was conducted to determine the
racial distribution of this disorder: approximately 5-15% of males older than 50 years
are affected. Dupuytren contracture is less common in blacks and Asians (incidence =
3%); Dupuytren contracture is least common (incidence <1%) among Indians, Native
Americans, and individuals of Hispanic descent. While the characteristics of the disease
in blacks are similar to those in whites (late onset, predominantly affecting the ulnar
digits, associated with alcoholism, smoking, liver failure, epilepsy, trauma and
diabetes), an important difference hinting to a genetic component to the pathogenesis
of the disease is that the disease is rarely bilateral in blacks.
Approximately 80% of affected individuals are male in all races studied. Disease onset
is earlier in males than in females, and the course of disease is more rapid and severe.
The mean age of disease onset in males is 49 years; in females, the mean age is 54
years. The mean age among men who seek surgery to treat this condition is also
earlier, at 58 years; the mean age among females is 62 years.
Revis, DR, Dupuytren’s
Contracture. E-Medicine
Rheumatology:
http://emedicine.medsca
pe.com/article/329414overview, accessed
9/22/2010
Saboeiro, AP et al. 2000.
Racial Distribution of
Dupuytren's Disease in
Department of Veterans
Affairs Patients. Plastic &
Reconstructive Surgery.
106 : 71-75
The exact cause of Dupuytren contracture is noty known. The disproportionate
prevalence of Dupuytren contracture in Northern Europeans suggests a genetic
component: studies suggest an autosomal dominant pattern of inheritance with
variable penetrance. HLA-B7 and HLA-DR3 have been identified in a number of
patients, suggesting a possible immunologic influence as well.
Young, DM and Hansen,
SL. 2010. CURRENT
Diagnosis & Treatment:
Surgery, 13e; Doherty GM
ed. Ch. 42: Hand Surgery.
Some researchers suggest that Dupuytren contracture may result from an error in
The McGrw Hills
growth and regulation of the fibroblast, resulting from chromosomal changes similar to Company; New York
Etiology
those seen in cells undergoing neoplastic change. Trisomy 8 has been identified in the
fibroblasts excised from some patients. In support of this theory, ealry nodules display
features of a benign neoplasm.
Because the changes occurring in the palmar fascia approximate the normal stages of
wound repair and remodeling, but to an excessive and detrimental extent, another
theory holds that this disease is a response to trauma. Repeated exposure to
compression, stress, and other mechanical forces may trigger a reparative process in
the palmar fascia. The reason for the progression to severe joint contractures remains
unknown.
Revis, DR, Dupuytren’s
Contracture. E-Medicine
Rheumatology:
http://emedicine.medsca
pe.com/article/329414overview, accessed
9/22/2010
In my opinion, these theories are not mutually exclusive; I tend to believe that the
contracture is a symptom associated with diverse disease processes, and that the
3
palmar fascia is particularly sensitive to these disease processes and it is thus
disproportionately affected. I think it is analogous to a cough in the sense that it is a
symptom, but just like a cough can be due to cancer, infection, heart failure etc, a
Dupuytren’s contracture is due to any processes that cause dysregulation of connective
tissue remodelling. Its association with cancer favors a neoplastic-like process, while its
association with trauma supports the aberrant wound healing theory. Furthermore, I
think that metabolic imbalances (such as those created by DM and liver failure) may
dysregulate the exceedingly complex tissue remodeling signaling cascades, leading to
fibroblast over-proliferation and subsequent scarring. The inflammatory state
established by those diseases further favors scarring.
Dupuytren contracture manifests itself most commonly in the palm by nodular or
cordlike thickening. As aforementioned, the skin may fuse with the underlying fascia
and become raised and hard, or it may shrink, sometimes drawn into a deeply
puckered crevasse. The disorder invades the palm at the expense of fat but is never
adherent to vessels, nerves, or musculotendinous structures (though it may be
adherent to flexor tendon sheaths). It has an unpredictable rate of progression, but the
Young, DM and Hansen,
earlier it starts in life, the more destructive and recurrent it is apt to be.
SL. 2010. CURRENT
Clinical
manifestations
Dupuytren fasciitis may involve any digit or web space, but it affects predominantly the
ring and small fingers. Over time the cord contracts resulting in digital flexion
contracture and reduced hand function. In longstanding cases, the fingers may be
drawn tightly into the palm, resulting in secondary contracture of joint capsule and
ligaments, flexor sheaths, and atrophic muscles. The condition is not usually associated
with pain.
Diagnosis & Treatment:
Surgery, 13e; Doherty GM
ed. Ch. 42: Hand Surgery.
The McGrw Hills
Company; New York
There is a paucity of studies on the specificity/ sensitivity of the clinical findings, as
Dupuytren’s contracture is a diagnosis based on surface anatomy findings and
associated functional impairment. The findings are consistent with a progressive
scarring process.
Late
presentation,
complications
As aforementioned, Dupuytren’s Disease has early, intermediate, and late phases.
While loss of normal architecture and formation of skin pits mark the early phase and
nodules and cords characterize the intermediate phase, contractures mark the late
phase. The late phase tends to go through four stages of contracture,
1. contracture of the MCP joint of the ring finger in stage I;
2. contractures of the MCP and PIP joints of the ring finger and the MCP joint of
the small finger in stage II;
Rayan, GM. 2007.
Dupuytren Disease:
Anatomy, Pathology,
Presentation, and
Treatment. The Journal of
Bone and Joint Surgery
(American). 89:189-198.
3. contractures of the MCP and PIP joints of the ring finger, the MCP and PIP
4
joints of the small finger, and the MCP joint of the long finger in stage III;
4. and stage-III contractures as well as DIP joint hyperextension of the ring or
small finger in stage IV.
This progression is not universal, and in fact palmar fascial disease or contracture
remains confined to the palm and does not progress enough to cause digital flexion
deformity. Palmar involvement usually precedes extension of the disease into the
digits; however, the disease may also begin and remain in the digits. A palpable
interdigital soft-tissue mass is an indication that the neurovascular bundle is involved,
but it is not a reliable indicator.
An early intervention to slow the progression of Dupuytren’s is radiotherapy. A study
showed that regardless of the individual stages, radiotherapy led to stabilization of the
disease in about 50% of patients (with an even greater percentage for earlier stages)
and 10 % improved.
Complication of late stage of the disease have to do with QOL factors (see below)
Nutritional
factors
Radiographic
evidence
Nutrition affects the disease insofar as diabetes and alcoholism both affect the
likelihood of developing the disease. Some studies reported that taking 200–2,000 IU
of vitamin E per day for several months was helpful in the treatment of Dupuytren’s,
suggesting a role for antioxidants in slowing the progression of the fibrosing process;
other studies on the effect of vitamin E, however, did not obtain the same promising
results.
Since Dupuytren’s disease is a surface disease, imaging is not typically obtained.
Research on imaging has shown, however, that MRI is accurate for detecting
Dupuytren's contracture, defining its palmar involvement, and depicting its extent. The
cords of intermediate and late disease have a uniformly low signal intensity similar to
that of tendons on both T1- and T2-weighted images Nodules have an intermediate
signal intensity similar to that of muscle on both T1- and T2-weighted images, with
focal areas of lower signal intensity. The signal characteristics of the lesions correlate
with the degree of cellularity of the lesions as seen histologically. The prognostic
Anonymous author.
Dupuytren’s Contracture.
The Institute for
Optimum Nutrition,
http://www.ion.ac.uk/he
althnotes.php?org=ion&C
ontentID=1205001
accessed 9/23/2010
Thomson GR. Treatment
of Dupuytren’s
contracture with vitamin
E. BMJ 1949;Dec
17:1382–3.
Yacoe ME, BergmanAG,
Ladd AL and Hellman,
BH.1993. Dupuytren's
contracture: MR imaging
findings and correlation
between MR signal
intensity and cellularity of
lesions. American Journal
5
significance is that highly cellular lesions tend to have higher rates of recurrence after
surgery than do hypocellular lesions.
of Roentgenology, 160:
813-817.
Laboratory
evidence
Revis, DR, Dupuytren’s
Contracture. E-Medicine
There are no laboratory studies that are commonly done to detect or stage the disease.
Rheumatology:
However, testing for associated conditions (CAGE screening and LFTs for alcoholism
http://emedicine.medsca
and liver failure, fasting blood glucose or glucose leading test plus HbA1C for diabetes)
pe.com/article/329414may be useful in addressing the patient’s overall health.
overview, accessed
9/22/2010
Psychosocial
impact of disease
A variety of activities can be affected. If it occurs in younger patients who are still
working, their work can be greatly affected. While the ability to write is not as affected
if only the fourth and fifth fingers are contracted, the disease can change one’s
handwriting. It can affect one’s ability to paint, cook, play golf or any other sport that
uses the hands. Aesthetically, it can be disfiguring and can greatly affect the patient,
especially women.
Risk factors
As mentioned in the epidemiology section, being of Northern European descent is a
risk factor in the development of the disease. Medical conditions that increase the risk
of developing the disease include alcoholism, smoking, liver failure, epilepsy, trauma,
and diabetes. Being male and being older than 50 y/o are also risk factors for
developing the disease.
Prevention
There are no proven strategies to prevent Dupuytren’s, though the role of antioxidant
vitamins (such as vitamin E) has been proposed. In light of the existence of early
treatments to slow or halt the progression of the disease, awareness in predisposed
groups and early diagnosis/treatment may be the best “preventative” options.
Saboeiro, AP et al. 2000.
Racial Distribution of
Dupuytren's Disease in
Department of Veterans
Affairs Patients. Plastic &
Reconstructive Surgery.
106 : 71-75
In early-stage Dupuytren's contracture, radiotherapy is applied to prevent disease
progression. Radiation therapy acts in a similar way as it does when applied to the
treatment of sarcomas and other tumors.
Treatment
options
Messina A, Messina J.
1993. The Continouos
Elongation Treatment by
TEC Device for Severe
A number of non surgical techniques have been attempted to treat Dupuytren’s
Dupuytren's Contracture
contracture, including splinting and stretching devices, ultrasonic therapy, and steroids.
of the Fingers. Journal of
None of these methods has been proven to benefit patients on a long-term basis.
Plastic and Reconstructive
Enzymatic fasciotomy using collagenase clostridium histolyticum significantly reduces Surgery; 92:84-90
contractures and improves the range of motion in joints affected by advanced
Dupuytren's disease.
Bird B, Ball C,
Balasuntharam P.
6
Surgery is indicated when the disorder has progressed sufficiently (more than 30
degrees of flexion at the MCP joint or any flexion contracture of the PIP joint).
Fasciectomy is the surgical procedure that gives the best long-term results, although in
selected cases where only the longitudinal pretendinous fascial band is involved and
the skin moves freely over it, subcutaneous fasciotomy done through a small
longitudinal incision may release a contracture and decrease post-op disability time
due to the less invasive nature of the procedure.
The degree of atrophy of the skin overlying the contracture determines whether a skin
graft is required for wound closure after fasciectomy. A skin graft may have the added
benefit of substituting the diseased overlying dermis, which has been implicated as an
inductive mechanism in this process, thus lowering the recurrence rate in severe cases.
Unfortunately, the degree of contracture of the fifth digit may be so severe to require
amputation.
Motion should be started within 3–5 days after surgery. Dynamic splints and
postoperative injection of corticosteroids into joints and the operated areas may
increase patient’s adherence to the physical therapy plan.
In the occasional case with acute and rapid onset of a tender nodule, local
triamcinolone may be used for symptomatic relief. DMSO (Dimethyl sulfoxide) applied
to the affected area may reduce pain by inhibiting transmission of pain messages, and
may also soften the abnormal connective tissue; its effectiveness and safety, however,
remain unproven.
Rehabilitation after
surgery for Dupuytren's
Contracture (Protocol).
Cochrane Database of
Systematic Reviews 2007,
Issue 2. Art. No.:
CD006508. DOI:
10.1002/14651858.CD00
6508.
Hurst,LC, et al.2009.
Injectable Collagenase
Clostridium Histolyticum
for Dupuytren's
Contracture. NEJM,
361:968-979
Betz N, et al. 2010.
Radiotherapy in earlystage Dupuytren's
contracture. Long-term
results after 13 years.
Strahlenther Onkol;
186:82-90.
Lee, S Baytion, M, Reinke,
DL, Bogdan, Y. Dupuytren
Contracture. E-Medicine
Orthopaedics:
http://emedicine.medsca
pe.com/article/1238712overview, accessed
9/23/2010
Young, DM and Hansen,
SL. 2010. CURRENT
Diagnosis & Treatment:
Surgery, 13e; Doherty GM
ed. Ch. 42: Hand Surgery.
The McGrw Hills
Company; New York
Outcomes of
treatment
In terms of early treatment, a study of long term outcomes (13 years) of radiotherapy
showed it is effective in prevention of disease progression and improves patients'
symptoms in early-stage Dupuytren's contracture. In case of disease progression after
radiotherapy, a "salvage" fasciotomy is still feasible with good outcomes despite the
Betz N, et al. 2010.
Radiotherapy in earlystage Dupuytren's
contracture. Long-term
7
tissue changes associated with radiation therapy.
A retrospective study of the outcome of carpal tunnel release was evaluated
retrospectively patients followed for a median of 10 months. Outcome was considered
good in 27% (pain, weakness, and numbness were essentially resolved); fair in 42%
(most of the symptoms improved); and poor in 32% (symptoms persisted or
worsened). Patients whose pre-operative work activity was considered physically
strenuous were associated with a slightly but significantly poorer outcome (60% good
or fair) compared to those in light work or with no employment (89% good or fair).
Proportionately fewer patients returned to their original work when they previously
engaged in strenuous activity, ranging from 27% for those using air guns to 80% in light
work. The highest chance of a poor outcome from carpal tunnel release occurs in
patients who have either associated symptoms of thoracic outlet syndrome or
physically strenuous work activities.
Another study of postoperative management of Dupuytren’s patients with prevention
of applied mechanical tension in the early phases of wound healing showed that this
intervention decreases complications after fasciectomy and that no digital motion is
lost to this protective intervention.
results after 13 years.
Strahlenther Onkol;
186:82-90.
Zyu, G, Firrelll, JG, Tsai,
TM. 1992. Pre-Operative
Factors and Treatment
Outcome Following
Carpal Tunnel Release.
Hand Surg Eur Vol 17:
646-650
Evans RB, Dell PC,
Fiolkowski P. 2002. A
clinical report of the
effect of mechanical
stress on functional
results after fasciectomy
for Dupuytren's
contracture. J Hand Ther.
15:331-9
Bird B, Ball C,
Balasuntharam P.
Rehabilitation after
surgery for Dupuytren's
Contracture (Protocol).
Cochrane Database of
Systematic Reviews 2007,
Issue 2. Art. No.:
CD006508. DOI:
10.1002/14651858.CD00
6508.
Bird B, Ball C,
Balasuntharam P.
hematoma, fibrosis and stiffness, digital nerve injury, recurrence of contractures and
Rehabilitation after
digital ischemia secondary to digital artery injury. Reflex sympathetic dystrophy, a
surgery for Dupuytren's
painful, debilitating neurologic disorder of the hand, can occur after surgery and must Contracture (Protocol).
be treated aggressively. In general, the functional reward for the patient is great at
Cochrane Database of
any age.
Systematic Reviews 2007,
Issue 2. Art. No.:
A Cochrane review showed that although surgery can successfully correct deformity it
CD006508. DOI:
does not cure Dupuytren's disease and recurrence is common after surgery, and that
10.1002/14651858.CD00
reports of recurrence rates following surgery within the first few years range from
6508.
The potential complications of surgery are wound breakdown (loss of skin flaps),
Potential
Complications of
treatment
27% to 34%. After five years it has been reported between 41% and 71%. The
effectiveness of surgery when the contracture involves the metacarpophalangeal
Lee, S Baytion, M, Reinke,
8
(MCP) joint of only one finger is high. However, when two or more joints are involved DL, Bogdan, Y. Dupuytren
complete correction is less likely. At the proximal interphalangeal joint (PIP) the
improvement rate is reduced. PIP contractures in isolated cords improved by 50%
after surgery but is often lost during the healing phase. Other post operative
complications of Dupuytren's contracture include sympathetic flare, hypertrophic
scarring, pain, joint stiffness, loss of digital extension and reduction of function.
Contracture. E-Medicine
Orthopaedics:
http://emedicine.medsca
pe.com/article/1238712overview, accessed
9/23/2010
9