Dupuytren disease





Introduction


Dupuytren’s disease (DD) is an enigma. Histologically, DD is a myofibroplastic disease, resulting in painless cords in the hand that eventually lead to flexion contracture of the fingers. In etiology, it is a genetic disorder, often inherited in an autosomal dominant trait, but it is frequently seen with a multifactorial etiology. Can we identify and treat the problem in the genes and cells before they even produce DD? What can we do to avoid open surgery, since nonoperative care or minimally invasive surgery have become the standard in so many other disciplines—laparoscopic cholecystectomy, knee arthroscopy, and coronary artery stenting to name a few. Percutaneous needle fasciotomy (PNF) recurs, but can we minimize that with adjunctive radiation or chemicals? Can we develop cost-effective and safe enzymes that work as a “surgical drug”? Can we develop outcome measures that are so specific to DD that we really can compare treatments? In this chapter, experts from around the world provide knowledge, updates, and insights into this common yet frustrating condition.


Epidemiology and risk factors (Dominic Furniss, Osaid Alser)


Prevalence


DD is a progressive and irreversible fibroproliferative disorder of the palmar fascia of the hand and considered the most common inherited connective tissue disease. Several studies have estimated the prevalence of DD both in specific countries and worldwide, and the latter ranged from 0.2% to 56%. This wide range in reported prevalence is likely due to several factors including the variation in study design and in population in terms of age, sex, race, and geographical location. The highest prevalence of DD is reported in Western European countries, especially Scandinavian countries.


In the United Kingdom, the estimated prevalence of DD ranges from 0.8% to 25%, with wide geographical variation. Early et al estimated the prevalence of DD in the United Kingdom to be 4% in all ages and increasing to around 20% in the elderly aged 65 and above, demonstrating the increasing incidence with age. In the United States, the prevalence is estimated to be between 2% and 12%. Some studies have also reported high prevalence Africa and some regions in Asia, indicating.


Risk factors


Although the etiology for DD is incompletely understood, DD is known to be a complex disease where genetic and nongenetic factors play a role in pathogenesis. DD usually affects the elderly population, and it is more commonly seen in men than women. The incidence rises sharply after the fourth and fifth decade, with men and women being affected by the same frequency after the eighth decade. , The peak age of onset of DD in men is from 40 to 59 years, whereas for women it is from 40 to 69 years. Several studies have reported overall male-to-female ratio of DD between 3:1 and 9.5:1. , Men tend to have bilateral and more severe disease, and they present, on average, 10 years earlier than women. ,


In a systematic review, the following factors were identified as strong nongenetic risk factors for the development of DD: advanced age, male sex, diabetes mellitus, heavy alcohol drinking, cigarette smoking, and manual work exposure, with the latter three showing a dose-response relationship. Salarito et al showed that the highest prevalence of DD was seen in patients with type 1 diabetes mellitus with 34%, and the second highest prevalence is among patients with type 2 diabetes mellitus with 25%. Interestingly, rheumatoid arthritis and obesity have been shown to protect from the development of DD in both observational and genetic studies. , , , A classical twin study by Larsen et al estimated the heritability of DD to be about 80%, equivalent to type 1 diabetes mellitus, and far higher than ischemic heart disease. The largest genetic study of DD published to date has identified 43 tissue-specific genomic regions associated with increased risk of the development of DD, which confirms the genetic component of DD and provides an unprecedented insight into the biology of the disease that will revolutionize treatment in the coming decades.


Clinically relevant anatomy (Martin Langer, David Warwick)


Understanding the anatomy ensures that surgery is performed thoroughly enough to correct the finger safely and durably. The nodules and cords in DD are proliferations of normal delicate anatomical structures in the fibrous skeleton. New connections can form between different normal fascial structures in DD, either changing the normal course of vessels and nerves (spiral cords) or contracting the finger joints.


Fibrous skeleton of the palm


The palmar aponeurosis , always present even without palmaris longus tendon, is the central structure of the fibrous skeleton in the palm, which develops contracture in DD. Proximally, it originates from the palmaris longus tendon or, if absent, from a subcutaneous fascial point slightly ulnar to the scaphoid tubercle. Strong longitudinal fibers run toward the metacarpophalangeal (MCP) joints, ending in subcutaneous fatty tissue at the distal palmar crease (Kanavel’s line). The more delicate fibers toward the thumb reinforce the thenar fascia ( Fig. 27.1 ).




Fig. 27.1


Palmar view of the hand. 1 –Insertion of Grayson’s fibers to base of second phalanx; 2 –Grayson’s fibers; 3 –Natatory ligament; 4 –Abductor digiti minimi fibers; 5 –Transverse fibers (Skoog) of palmar aponeurosis; 6 –Longitudinal fibers of palmar aponeurosis; 7 –Palmaris brevis muscle; 8 –Palmar cutaneous branch of median nerve; 9 –Thenar branch of palmar aponeurosis; 10 –Palmaris longus tendon; 11 –Median nerve; 12 –Gosset’s fibers; 13 –Distal palmar commissural fibers; 14 –Proximal palmar commissural fibers.

(Courtesy Martin Langer).


From these longitudinal fibers, numerous short Grapow’s fibers (called retinacula curtis ) run to the dermis, especially to the flexor creases of the palm. In DD, these fibers produce small funnel-shaped skin indentations (Hugh-Johnson sign). Distal to the superficial palmar arterial arch, these longitudinal connections form vertical septa to the deep fascia between the flexor tendons and the neurovascular bundles/lumbrical muscles, attaching to the palmar interossei fascia and metacarpal bones. , A little further distally are transverse fibers dorsal to the longitudinal fibers—Skoog ligaments—which are extremely rarely affected by DD. These Skoog ligaments, longitudinal and transverse fasciculi, and the septae of Legueu and Juvara together form the palmar aponeurosis pulley of the flexor tendon. ,


Fibrous skeleton at the border between the palm and the fingers


In a normal hand, the normal fibers of the fibrous skeleton are very faint in the finger, whereas in DD, pathology of these fibers is common. The natatory ligaments are normally only small reinforcing fibers in the fatty tissue in the skin between the fingers not deserving the name “ligament,” but in DD they can form strong strands that contract the web. One must recognize during surgery that these fibers are always palmar to the neurovascular bundles that can spiral around individual strands; they can easily be injured here.


Some fibers arise from the distal parts of the longitudinal fibers, running deep distally and connecting to the fascia on both sides of the metacarpophalangeal joints, from where they send further fibers distally into the fingers (Gosset’s fibers). Fibers also arise from the palmar fascial structures, expanding to the lateral skin of the phalanx of the fingers. , In the first web space, fibers that are radial extensions of both the natatory ligament and Skoog’s ligament can both be affected by DD ( Fig. 27.2 ).




Fig. 27.2


Fibrous skeleton of the finger. 1 –Natatory ligament; 2 –Grapow’s fibers to distal palmar crease; 3 –Longitudinal fibers of palmar aponeurosis; 4 –Transverse fibers (Skoog) of palmar aponeurosis; 5 –Septum of Legueu and Juvara; 6 –Vascular nerve bundle; 7 –Lumbrical muscle; 8 –Flexor tendons; 9 –Deep fascia of interosseus muscles and connections to bone; 10 –Interosseus muscle; 11 –Metacarpal bone; 12 –Cleland’s ligament (distal portion); 13 –Landsmeer’s oblique retinacular ligament; 14 –Cleland’s ligament (proximal portion); 15 –Chorda alligans; 16 –Thomine’s fibers, lateral digital sheet; 17 –Extensor tendon; 18 –Gosset’s fibers; 19 –Deep transverse metacarpal ligament. The insert is a cross-section at proximal phalanx. 1 –Grayson’s ligament; 2 –Grayson’s ligament as palmar sheet of neurovascular bundle; 3 –Neurovascular bundle space; 4 –Cleland’s ligament as dorsal sheet of neurovascular bundle; 5 –A2 pulley; 6 –Flexor digitorum superficialis tendon; 7 –Flexor digitorum profundus tendon; 8 –Palmar digital nerve; 9 –Palmar digital artery; 10 –Chorda alligans; 11 –Cleland’s ligament; 12 –Dorsal cutaneous retinacula; 13 –Proximal phalanx; 14 –Extensor tendon.

(Courtesy Martin Langer).


On the ulnar side of the little finger, there is a fascial system often affected by DD, comprising the fascia of the abductor digiti minimi muscle, connecting with the lateral digital sheath and Grayson’s ligaments. ,


Fibrous skeleton of the fingers


Grayson fibers run from the connective tissue cord along the fibrous palmolateral edge of the phalanges over the flexor tendon sheath, forming the palmar sheath of the neurovascular bundles and stabilizing the skin. These fibers are particularly commonly affected in DD. Between the A3 and A4 pulleys, these ligaments strongly attach to the bone and can even cause osteophytes.


Cleland ligaments arise alongside the interphalangeal joints and pass dorsal to the neurovascular bundles in the lateral skin of the fingers; they are most apparent from the distal PIP joint to the lateral skin of the middle phalanx. DD involvement of these fibers is less common and more proximal at the level of the first phalanx with connections to the lateral digital sheet. Connections between Grayson’s fibers and the oblique retinacular ligament of Landsmeer pull the terminal extensor tendon, thus hyperextending the DIP joint. On the dorsal finger, connections between the transverse retinacular ligament (Landsmeer) and the attachment of the intermediate tract cause Garrod’s pads ( Fig. 27.2 ).


Clinical presentation (Ilse Degreef)


Major findings and progression


Nodules in the palm and fingers and flexion contracture of the fingers are the main clinical presentation. The nodules in the palms are often the initial clinical findings. Over the years, the nodules may enlarge to cause notable and more severe flexion deformities ( Figs. 27.3 and 27.4 ). Although it is often limited to (generally) painless nodules under the palmar skin, one in four will suffer disease progression with an impact on hand function.




Fig. 27.3


Typical disabling Dupuytren’s contractures of the fourth and fifth digits in a left hand.



Fig. 27.4


Tabletop test. A positive tabletop test, when a patient with Dupuytren’s contracture is unable to place the fingers of their affected hand flat on a table, may prompt some surgeons and patients to consider surgery.


The ring and little fingers are most often involved. Multiple fingers and joints can be involved at the same time can also be involved after MCP joint. DD has a slow progression in most patients. Most often, hand function is impacted after years due to finger contractures that reduce the capacity to spread and open the fingers.


Different contractures


The clinical presentation, evolution, and severity of DD differ between individuals. The contractures can be more or less pronounced, resulting in a spectrum of minimal to devastating functional impact on the hand function. Although the severity of these contractures can be measured with goniometry, the clinical presentation, functional impact, treatment, and prognosis of DD are very variable. Functional deficit does not relate strongly to the degree of contracture.


The traditional staging by Tubiana is perhaps not sensitive enough because DD has a spectrum of deformity rather than clear categories. Surgery is more challenging and outcome less predictable in the proximal interphalangeal joint than if the disease is limited to the metacarpal joints. Also, contractures can be compound or fixed ; the first are caused by superficial DD leaving the joints less involved versus the latter, where MCP and PIP joints are in a fixed flexion position, again likely to negatively influence surgical outcome. The involvement of the skin and extension of the disease strongly determine the presentation and treatment options.


Disease diathesis


All fingers can become affected, and, typically, radial hand affection is associated with a more severe presentation and evolution of the disease. Often, these patients with a more aggressive form of DD show signs of fibrosis in other anatomical places than the hand such as the feet (Ledderhose disease) and the penis (Peyronie disease). The simultaneous presence of such comorbidities may point to a probable genetic background of the disease. These patients have, as Hueston described in 1963, a fibrosis diathesis and are prone to scar tissue formation. They often present with more severe and therapy-resisting Dupuytren’s contractures, and recurrence after surgery is common. Other fibrotic processes are often present such as knuckle pads (Garrod nodules on the dorsum of the proximal interphalangeal joints) and even arthrofibrosis of larger joints such as the shoulder.


Patients with severe fibrosis diathesis commonly have a younger age of onset (under 50), diffuse involvement of both hands, and a positive family history of DD. Most likely, if patients present with a family history and ectopic features, a genetic background may predispose to the development, progression, severity, and even therapy resistance of DD. Therefore, the development of a genetic weighted risk score may be a useful future prognostic tool.


Associations


Other comorbidities are reported to be associated with DD, such as diabetes, antiepileptic drugs, excessive drinking of alcohol, or smoking tobacco. It is more common in slimmer people. It is unclear if these associations are influencing the evolution of DD or form a true etiology of it. They may indeed be rather a trigger than a cause. The same goes for chronic or acute trauma of the hands, suspected as being the cause for Dupuytren’s contractures, but possibly a facilitator to disease activation in patients (genetically) prone to DD. Aging and the male gender are also factors that may predispose to DD, since the incidence increases with age and men are more often affected.


Examination tools and differential diagnosis


DD is a clinical diagnosis. Ultrasound may be used to detect the location of the cords. In the early stage, the cords should be differentiated from soft-tissue tumors, such as ganglions or giant cell tumors of the tendon sheath, and trigger fingers. With the development of finger flexion and nodules in the palm, the diagnosis is very straightforward. CT and MRI are not usually necessary. Plain radiograph is necessary for those with longstanding diseases to know any changes in finger joints.


Pathologic and molecular changes (Ilse Degreef)


The histologic basis of DD is a fibroproliferative process that occurs and progresses within the palmar and digital subcutaneous fascia or aponeurosis. Somehow, normal connective tissue cells named fibroblasts appear to be activated and transform to myofibroblasts, as discovered by Gabbiani in 1971. These cells are somewhat larger and develop muscle-cell-like properties such as the expression of actins aligned with the cell axis, which gives them the ability to slowly contract. In addition, they produce collagen, which is deposited within the three-dimensional structure of the palmar fascia. Type III collagen is disproportionally present in DD, similar to wound-healing processes. Clinically, the myofibroblasts appear in nodules under the palmar skin, sometimes causing skin pitting. Once collagen production becomes abundant, fibrous strands appear with a well-described surgical anatomy. These strands then contract the fingers into a bent position, limiting full extension with preserved active flexion.


Several molecular processes are reported to be involved in disease progression, with cytokines such as transforming growth factor (TGF)-β1 and nerve growth factor (NGF). Many of the paracrine molecules that are encountered in Dupuytren’s tissue have proinflammatory properties, suggesting that we may even be dealing with an autoimmune disorder. , The involved cytokines are found to influence the myofibroblast activation, proliferation, contraction, and collagen production and are the subject of translational research to hopefully prevent disease progression and to improve the outcome of treatment for established contractures.


Evaluation of outcomes (David Hunter-Smith, Elisa Jonatan)


One of the hardest things when treating DD is to determine how to measure the success or otherwise of treatment. Is it the straightest finger, the fastest recovery, the most minor recurrence, or the lowest complication rate that wins the race as the most critical factor? The vast heterogeneity of disease based on diathesis, age, gender, working history, comorbidities, genetics, and treating practitioner preferences all play a role in determining the progress and outcome after treatment. Tubiana’s goals in treating DD, that is, to correct contracture, enhance recovery, minimize complications and avoid recurrence, are as relevant today as in 1999 when he wrote. Across studies, there are many ways to describe the measurement of disease severity and treatment outcome of DD.


Goniometry


Range of movement (ROM) is the most frequently reported measure—total active motion (TAM), lack of joint extension, or the percentage of improvement—based on the assumption that the more severe the contracture, the worse the functional disability. The primary outcome measure in the collagenase studies was “ clinical success ” (posttreatment contracture between 0 and 5 degrees) and “ clinical improvement ” (posttreatment improvement of contracture of greater than 50 degrees). , Recurrence is defined as “more than 20 degrees of contracture recurrence in any treated joint at one-year posttreatment compared to six weeks posttreatment.”


Contracture outcome measures are relatively easy to measure, best done with a goniometer, and considered the most objective and reliable tool for measuring range of motion of the joint. The goniometer is inexpensive and readily available, providing speedy measurement in various clinical conditions.


The Tubiana grading measures total flexion deformity (TFD), otherwise known as passive extension deficit (PED), by adding individual finger flexion of the three finger joints. Each of the stages corresponds to 45 degrees TFD of each finger. This system artificially places the contracture into an arbitrary category, which is rather unsuitable for a continuous variable.


Goniometry is commonly used with good reliability. Disadvantages include the reliability of goniometer readings dependent on examiner experience and the joint measured (for example, in deformed joints) and a requirement for consistent positioning, stabilizing, and aligning of joints.


Patient-reported outcome measures (PROMs)


DD interferes with physical functions, restricting daily activities. Therefore, PROMs help clinicians better understand disease severity based on the patient’s functionality and are more frequently used in modern clinical practice.


PROMs are derived from a scored questionnaire containing items the patient completes usually before and after treatment. According to the United States Food and Drug Administration (US FDA), a patient-reported outcome (PRO) is a report of a patient’s health condition acquired directly from the patient, without interpretation by clinicians or anyone else. PROMs derive information that is not obvious or seen by the observer. ,


PROMs are either generic and used across all conditions (e.g., EuroQol 5D, Patient-Specific Functional score [PSFS]), specific for upper limb or hand (e.g., DASH, Michigan Hand Questionnaire, Patient Evaluation Method [PEM]), or specific for a disease or single condition (e.g., the Unite Rheumatologique des Affections de la Main [URAM] or Southampton Dupuytren’s Score Scheme [SDSS]).


Patient-specific functional score (PSFS).


The PSFS scale was developed by Stratford et al in 1995 and has been used in many musculoskeletal conditions (e.g., low back pain, cervical radiculopathy, knee dysfunction). The patient identifies and scores up to five activities affected by their condition on an 11-point scale, with 0 describing “unable to perform” and 10 describing “able to perform at the prior-disease level.” Van Kooiji et al evaluated the PSFS in DD, finding good content validity and responsiveness. PSFS differs from other PROMs because the items of these PROMs are picked by the patient, who also rates the difficulties.


Cosmin.


The COSMIN (Consensus-based Standards for the selection of health status Measurement Instruments) tool aims to improve outcome measurement by providing methodology and practical tools for choosing the most suitable outcome measurement instrument. The COSMIN taxonomy differentiates into three quality domains: reliability, validity, and responsiveness, and each domain comprises one or more measurement properties. ,


Specific hand PROMs


DASH.


The DASH questionnaire is a common tool since its report in 1996, and a shorter 11-item version of DASH (the Quick DASH) provides similar precision. , Although generally reliable, DASH and Quick DASH when used in DD may not be valid, with reports showing a weak correlation with total patient extension deficit (TPED). DD does not impact the arm or shoulder, so DASH is too broad to assess severity in a hand-specific condition such as DD. Also, some items (pain, tingling, sleep) are not affected in DD, thus diluting its validity. A large ceiling effect on DASH scores suggests that any improvement is hard to identify because many patients scored near-perfect scores on the pretreatment questionnaires, so that any improvement cannot be detected on the posttreatment questionnaire score.


Michigan hand questionnaire (MHQ).


In 1998, the MHQ was designed by the University of Michigan using psychometric principles. , MHQ is a hand-specific outcome measurement with 63 questions on six scales: (1) overall hand function, (2) activities of daily living (ADLs), (3) pain, (4) work performances, (5) aesthetics, and (6) patient satisfaction with hand function unlike other questionnaires, MHQ considers aesthetics an essential measurement of patient outcome. MHQ is probably too long (an average of 15 minutes to fill the entire 37 items) for use in the outpatient setting for most practitioners. In 2011, the 12-item brief MHQ was developed as a more efficient and versatile outcome measures.


Patient evaluation measure (PEM).


In the United Kingdom, the PEM is widely used in assessing DD patients. However, there are few data to support its widespread use besides its medium effect size and time needed to complete (often 2–3 minutes). The PEM lacks psychometric measurements and is less responsive than the other PROMs reported. It is not clear whether it is a sufficiently reliable measure specifically for DD.


Dupuytren’s specific PROMs


The southampton dupuytren’s score scheme (SDSS).


Another validated scoring system for assessing disease disability or treatment outcome specifically for DD is the SDSS. The SDSS consists of a 5-item score with five grades specific to DD. The lowest grade is of no problem (scored 0) to a severe problem (scored 5). The minimum score is 0, and the maximum score is 20.


The overall internal consistency, reliability, and content validity are good. In a study by Warwick, preinjection flexion deformity was correlated with both SDSS and Quick DASH. SDSS correlated with deformity (r = 0.2, p ≥ 0.002), whereas QuickDASH did not (r = -0.01, p = 0.86). However, despite being statistically significant, an r of 0.2 was described as a weak correlation between SDSS and angular deformity. It is suggested that a more comprehensive study should be conducted to understand the correlation between deformity and function, also a consideration of the relative merits between objective and subjective outcome measures.


Unite Rheumatologique des Affections de la Main (URAM) scale.


The URAM scale was the first validated patient-reported functional outcome specific for DD comprising nine patient-reported domains with a score ranging from 0 (best) to 45 (worst).


The URAM can be performed very quickly (often less than a minute), making it an easy and reliable tool to be used in the outpatient setting. However, the scale was conceived in French but translated into English, so the first question, “Wash yourself with a flannel, keeping your hands flat?” is not transferable because the French population use a flannel (shaped like a glove) to wash their face, whereas in English a flannel is interpreted as a hand towel (square or rectangular).


The URAM scale shows good internal consistency and test-retest reliability and is responsive in detecting clinically meaningful changes after needle aponeurotomy. It appears to distinguish between patients with and without disease progression and a strong correlation with the worsening of finger contracture as measured by the Tubiana scale. , A recent systematic review and meta-analysis regarding the sensitivity and specificity of the URAM scale concluded that the URAM scale is highly sensitive to changes in Dupuytren contracture but has low specificity.


Recommendations


Outside of the research setting, the authors believe that both a joint contracture measurement and a PROM should be used when assessing DD. The best-performing and practical PROMs for DD appear to be (1) Unite Rheumatologique des Affections de la Main (URAM) scale, (2) the Southampton Dupuytren’s Score Scheme (SDSS), and (3) Patient-Specific Functional Score (PSFS).


We recommend using either the URAM or the SDSS in conjunction with the PSFS. The URAM and SDSS provide functional insight into DD, and the PSFS offers more insight from the patient’s perspective. More studies are needed to better understand the correlation between deformity and function and to consider the relative merits between objective and subjective outcome measures.


Treatment of the mild form of DD


The mild form of DD that does not affect hand function can be tolerated by patients without specific treatment for months or years. The patients usually want to have injections or surgical treatment because they do not like the nodules, pain, or the influence on hand function by the DD. The exact indications and requests for nontreatment vary greatly among patients. In the more advanced disease stage, treatment is necessary as described below.


Fasciectomy (Hitoshi Hirata and Michiro Yamamoto)


Fasciectomy techniques for DD include (1) subcutaneous fasciotomy, (2) limited fasciectomy, (3) complete fasciectomy, and (4) dermofasciectomy with skin grafting. Because higher complication rates have been reported for both complete fasciectomy and dermofasciectomy, we focus on limited fasciectomy, which is probably the most commonly used technique, in this section. ,


Indications


There is no consensus on the indications for fasciectomy. Existing reports suggest that surgery may be indicated when a passive extension deficit of the MCP joint is greater than 30 degrees, that of the PIP joint is greater than 15 degrees, or both. However, a pure numerical indication should be balanced by an understanding that the patient should be involved in the deciding surgery and should usually have identifiable functional problems, against which to judge the risks and possible benefits of surgery. The tabletop test is a common examination to check the flexion contracture of the fingers, although one can often put a ringer flat on the table despite a substantial PIP contracture if there is hyperextension at the MCP joint ( Fig. 27.5 ).




Fig. 27.5


Basic skin incisions. (A) A zigzag incision. (B) A straight midline incision with Z-plasty. A single longitudinal incision is sufficient to treat the palmar lesion. (C) Transverse incisions are used followed by McCash’s open palm technique. (D) L shaped incision to raise a Jacobsen flap to expose DD.


The prognosis and course of the disease varies. The age at onset and the rate of disease progression differ from patient to patient. For example, some patients experience an early onset and rapid progression, some have a late onset and slow progression, and others have a late onset and rapid progression. A multifaceted approach that incorporates patient preference, the likelihood of a successful outcome, recovery time, risk of postoperative complications, and possibility of recurrence is appropriate when evaluating the type and timing of surgery.


Dupuytren’s diathesis involves factors that elevate the risk of a greater disease severity and postoperative recurrence. In 2006, Hindocha et al expanded the factors and calculated odds ratios using logistic regression to more accurately predict the risk of a recurrent Dupuytren’s contracture. In total, 322 patients ranging in age from 25 to 90 years each of whom had a fasciectomy were analyzed. The revised diathesis criteria identified family history, bilateral lesions, ectopic lesions in the knuckles (Garrod’s pads), male gender, and being younger than 50 years old at onset. When treating these patients, it is necessary to explain their higher risk of recurrence before fasciectomy is undertaken.


Surgical setting


The patient is placed in a supine position with their affected arm exposed. They are administered general anesthesia or an axillary block and fitted with a tourniquet on the upper arm. Wide-awake local anesthesia and no tourniquet (WALANT) is an emerging alternative, although some find the view obscured by the volume of anesthetic.


Surgical techniques


There are two popular types of incision used to expose the pathologic tissue ( Figs. 27.5 and 27.6 ). When the PIP flexion contracture is severe, a straight midline incision converted to Z-plasty is useful. If the PIP flexion contracture is mild, a zigzag incision is sufficient ( Fig. 27.7 ). Sometimes the zigzag incision is modified to extend the corner for a Y to V closure using the redundant dorsal skin. A single additional skin incision is usually sufficient for palmar lesions even if multiple fingers are involved. Lesions limited to the palm can be treated with a transverse or oblique incision alone.




Fig. 27.6


A simple, stepwise technique to address complex cords, which is reproducible in the vast majority of cases. Start in the palm and extend the incisional distally either in Bruner’s pattern (good-quality skin) or a straight line (stage 1) , which converts to Z-plasties or a full-thickness skin graft (poor-quality skin) (stage 2 and 3) . For stage 2b , a straight incision is used in the finger; for stage 3b , a skin graft may be needed.

(From Stoneham A, Fetouh S, Kazzam M, Warwick D The ‘Universal Approach’ for Dupuytren’s Disease: a safe and reproducible sequence for planning fasciectomy incisions JPRAS Open 2024;41:372–375.)



Fig. 27.7


(A) An 81-year-old male with Dupuytren disease of the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints of the little finger. (B) A zigzag incision was made to excise the central cord. (C and D) One year postoperatively, the extension deficits of the MCP and PIP joints were zero with almost normal digital motion.


Jacobsen introduced the L-shaped full-thickness flap that is created by making two linear incisions. The first incision is in the transverse crease of the palm, and the second incision is at the mid-lateral line of the little finger to the distal interphalangeal joint. The Jacobsen flap is also an open palm technique ( Fig. 27.5 ).


On the palm, the pathologic tissue can be divided from the transverse palmar ligament, because only the longitudinal palmar ligament becomes the pathologic cord. The neurovascular bundle should be dissected and protected from the proximal site. We use vessel tape to protect and mark the neurovascular bundles. Care should be taken when dissecting the pathologic cord from the level of the MCP to PIP joints, because the neurovascular bundles run superficial to the spiral cord at this level.


Shewring and Rethnam reported that Cleland’s ligaments appeared to be involved in the disease in 18 of 20 patients in their study. They recommend that when fasciectomy is performed for a PIP flexion contracture, Cleland’s ligaments under the neurovascular bundle should be inspected. Cleland’s ligaments should be excised if there is macroscopic involvement with DD. Failure to treat Cleland’s ligaments is associated with early recurrence of PIP flexion contracture.


To complete the procedure, the wound is sutured with 5-0 sutures, preferably a combination of nylon at the corners and dissolvable in the spaces. The lightest dressings are applied to enable early movement. Patients are instructed to keep their hand elevated until the swelling subsides, but the earliest movement of the shoulder, elbow, and hand is encouraged ( Figs. 27.7 and 27.8 ).




Fig. 27.8


(A) A 69-year-old male with Dupuytren disease of the little finger metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints 2 years after a collagenase injection. (B) A recurrence involving the cord and nodule is indicated with arrows and arrowhead, respectively. (C) Full extension of the little finger after excision of the nodule and cord. (D) Six months postoperatively, full finger extension was attained.


Postoperative treatment


The patient is to begin active finger motion exercises overseen by a hand therapist from the second postoperative day. The bandage gradually becomes thinner as the gauze is changed. The use of a night splint ( Fig. 27.9 ) is controversial (see section below). Sutures are removed 10 to 14 days postoperatively.




Fig. 27.9


A night splint may be used for 3 months postoperatively.


Prognosis


We prospectively examined the treatment results after limited fasciectomies in. The scores on patient-reported outcome measures Hand10 and EQ-5D-5L (QOL) and the degrees of extension deficit of the MCP and PIP joints were assessed. Compared to baseline, both Hand10 and QOL scores worsened 1 to 2 weeks postoperatively but improved after 8 weeks. The range of motion of the MP joint improved, but the extension loss of the PIP joint tended to increase gradually.


Dias and Aziz reviewed previous reports and summarized the reported complications after limited fasciectomy. They are stiffness (5.1%), complex regional pain syndrome (5%), skin necrosis (4.1%), nerve injury (3.4%), infection (2.9%), vascular complication (2.1), amputation (0.4%), tendon injury (0.2%), and other (3.3%). Rodrigues et al reported outcomes at 1 and 5 years after fasciectomy by patient interview and examination at five centers in the United Kingdom. Forty-eight out of 126 patients (38%) and 61 of 125 patients (49%) had poor objective outcomes (defined as either the MCP joint or PIP joint having a degree of extension deficit greater than 25 degrees) with or without reoperation at 1 and 5 years, respectively. Although the reoperation rate was 2.4% and 7.1% at 1 and 5 years, respectively, as with all surgical procedures, recurrence is a possibility.


It is important to be familiar with the basic types of incisions that may be used in a limited fasciectomy, although there is insufficient evidence to indicate the superiority of one over another. A surgeon should communicate with the patient about the risk of substantial complications and recurrence that exists regardless of surgical technique.


Radiotherapy (Richard Shaffer, Jarad Martin)


Radiotherapy is the use of radiation for treating DD. Radiotherapy is most commonly used at high doses (e.g., total dose of 60–80 Gy) to treat cancer, but it is also used for the treatment of many noncancer (benign) conditions. At intermediate doses (e.g., 20–30 Gy), radiotherapy has an antiproliferative action, and at very low doses (e.g., 3–6 Gy), it has an antiinflammatory and analgesic effect. Because radiotherapy is used in intermediate doses for DD, side effects tend to be mild.


Radiotherapy may act on the Dupuytren’s tissue through (1) reduction in fibroblast differentiation to myofibroblasts; (2) increased free radicals causing macromolecule (including DNA) damage; (3) modulation of cytokines and signal transduction pathways; (4) affecting macrophages and reducing myofibroblast activation and proliferation; and (5) affecting vascular endothelial cells and reducing white cell adhesion and cytokine secretion.


Dupuytren’s tissue is most sensitive to radiation in the early stages, where there is hyperproliferation and early nodule formation. It retains some efficacy where there is early contracture, but where there are more advanced contractures (where collagen fibers dominate the connective tissue), radiation has little effect.


Radiotherapy for early progressive DD


Several retrospective studies have demonstrated low progression in patients treated with radiotherapy for DD. One study showed an overall 13-year progression rate of 13% of patients with stage N disease. Additionally, there was significantly lower progression than those treated within one year compared with those treated more than two years since the onset of symptoms.


A series of patients were treated with radiotherapy for DD in Essen, Germany, as part of a prospective trial where patients were randomized between two dose levels (Group A = total dose 30 Gy, Group B = total dose 21 Gy). They were treated with orthovoltage x-rays using an Xstrahl X200 machine (Xstrahl Ltd, United Kingdom). Most patients had early disease (nodules, cords, no contracture or up to 10 degrees of contracture) and active progression (worsening of disease, including growth or new nodules/cords). At 12 months follow-up, the overall treatment failure rate was 8%, with 2% needing corrective surgery. Progression by stage was 0% in Stage N, 3% in N/1, 15% in Stage 1, and 40% in Stage II.


Long-term (11-year) follow-up of the Essen study was performed for 594 patients (880 hands). This included 101 patients (141 hands) who after clinical assessment and informed consent decided not to receive radiotherapy but opted for observation, so forming a nonrandomized control group. Table 27.1 shows that both radiotherapy arms (30 Gy, 374 hands and 21 Gy, 365 hands) were superior to the control group, and the 30 Gy arm was superior to the 21 Gy arm. There were mild (grade 1) side effects noted in 14% of treated patients. No higher-grade side effects or radiation-induced cancers were observed.



TABLE 27.1

Outcomes at Mean 11 Years Follow-Up of the Control (Observation) Group, and Two Radiotherapy Groups (21 Gy and 30 Gy Total Doses)
























Groups Regression or Stable (%) Progression (%) Needing Surgery (%)
Control 34 66 48
21 Gy 74 26 14
30 Gy 81 19 8


This trial and other Dupuytren’s radiotherapy literature has been critiqued. The nonrandomized nature of the control group, single-center accrual, lack of peer review, and unique definition of progression have all led to calls for higher-level evidence before wider adoption of radiotherapy for this indication. Patient-reported outcomes have been reported in a survey of 2,235 patients with DD who were asked to rate the outcome of their treatments. The authors summarized the results as “By far the best rating is for radiotherapy.”


Emerging practice in the postoperative setting


Due to the risk of recurrence after management of a contracture, there is interest in bimodal treatment with the combination of surgical release of Dupuytren’s contracture with postoperative radiotherapy. In a recent study, six patients with a high chance of recurrence were treated with a combination of partial aponeurectomy and radiotherapy using surface brachytherapy. This was found to be feasible, and 14-month clinical follow-up showed minimal toxicity and no recurrences.


Additionally, in a retrospective study, 51 patients (55 hands) were treated within three months after needle fasciotomy (NF), collagenase injections (CI), or limited fasciectomy (LF). Seventy-five percent of them had at least two previous ipsilateral interventions. Progression, as defined by >20 degree increase in contracture, was significantly reduced compared with historical controls ( Table 27.2 ). Side effects were tolerable, with no reports of ulceration, deep wounds, or infections. This area is currently under further assessment in a randomized clinical trial.



TABLE 27.2

Recurrence of Contracture After Surgical Release with or Without Postoperative Radiotherapy *
























Groups Recurrence After Surgery Alone Recurrence After Surgery, with Radiotherapy
Follow-up 3–5 years 5.5 years
Early-NF 50%–60% 17%
Early-CI 50%–60% 43%
Early-LF 20% 10%

* Shaffer R, Seegenschmiedt H. (then add 122 as superscript at the end). Previous treatments such needle fasciotomy (NF), collagenase injection (CI) and limited fasciectomy (LF). in the patients with surgery alone was one, and two or more for the most patients with radiotherapy.



Toxicity


The main acute effect of radiotherapy to the palmar aspect of the hand is mild erythema, which resolves over 2 to 4 weeks. In the longer term, the most common change is reduced sweating in the palm. A feared complication is a radiation-induced malignancy (RIM). Only one relevant case (of a cutaneous squamous cell carcinoma) has ever been reported, and that was in a patient given radiotherapy as a teenager to manage hyperhidrosis in the 1960s. From other radiotherapy literature, the risk of a RIM decreases with the age that treatment was delivered, and as part of the informed consent process, a RIM risk of between 1:1000 and 1:5000 has been proposed for the use of low-dose radiotherapy for benign conditions.


Techniques


Treatment is often delivered as an outpatient in 5 daily sessions, each lasting around 10 to 15 minutes, and repeated after 4 to 12 weeks. Given the target is usually the palmar fascia, relatively simple techniques are often adequate to get the right balance between delivering the radiation dose to this structure and minimizing effects on other tissues. Fig. 27.10 shows a representative radiation dose distribution, demonstrating a more intense dose in the superficial aspect of the palm.


Mar 9, 2025 | Posted by in ORTHOPEDIC | Comments Off on Dupuytren disease

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