Dupuytren’s disease

Basic science and disease process

Dupuytren’s disease (DD) is a primary fibroproliferative disorder of the palmar and digital fasciae of the hand. A similar disease process known as Ledderhose and Peyronie’s disease affects the feet and penis, respectively. Recent studies indicate that the condition occurs as a result of a complex interplay between genetic, epigenetic, immunologic, and environmental factors ( Fig. 17.1 ).

Surgical anatomy of the palmar fascia in the hand and fingers and involvement in Dupuytren’s disease

DD follows anatomical pathways, and one of the most enigmatic aspects of DD is how the disease converts the normal anatomy of the fascial bands into diseased cords. In this section, accepted and new insights in the microanatomy of the fascia pertaining to DD are reviewed and related to surgical findings.

DD commonly starts in the palm where it involves the triangular-shaped fascia or palmar aponeurosis (PA) which is connected to the overlying skin by vertical fibers described by Grapow.

Involvement of the Grapow’s fibers results in the development of skin pits, one of the earliest signs of DD. Proximally, the apex of the PA is continuous with the tendon of the palmaris longus (PL) muscle, if present; otherwise, it attaches to the flexor retinaculum. It has been suggested that if the palmaris longus tendon is absent, the flexor carpi ulnaris (FCU) muscle acts as the longitudinal tensor of the palmar fascia. Dupuytren’s nodules have been described at the level of the wrist related to the PL or the FCU. The longitudinal fibers of PA are condensed into pretendinous (PT) bands that radiate distally towards the base of the fingers ( Fig. 17.2 ). A PT band for the thumb is also present, but less prominent than for the fingers. Between the PT bands the more delicate prelumbrical bands complete the palmar aponeurosis ( Fig. 17.3 ). The PA is bordered by the thenar and hypothenar eminences on either side and fixed to the interspatial septa that divide the palm in thenar, midpalmar, and hypothenar spaces. The base of the triangular sheet of the PA is located at the level of the transverse ligament of the palmar aponeurosis (TLPA), which is situated at a slightly deeper plane along a line that joins the proximal and distal palmar creases. TLPA is supported by the ligaments of Legueu and Juvara, which connect it to the deep transverse intermetacarpal ligament (DTIL, Fig. 17.3 ). The DTIL runs between the volar plates of the MCP joints and is not affected by DD.

On cross-section, the TLPA and DTIL, together with the septa of Legueu and Juvara, form seven “boxes” (see Fig. 17.3 ). Each box encloses a lumbrical muscle and a neurovascular bundle, or the flexor tendons of each ray. Vertical septae separate the hypothenar from the midpalmar space, and the midpalmar from the thenar space, and need to be divided to remove the proximal part of the palmar aponeurosis.

The ulnar border of the TLPA merges with fibers of the hypothenar fascia.

At the radial border the TLPA is continuous with the proximal commissural band, which is a condensation of the fascia of the adductor pollicis muscle and runs through the first webspace to end in the thenar fascia at the level of the first metacarpophalangeal (MCP) joint. It is believed that TLPA is not affected by DD, whereas the proximal commissural band can give rise to an adduction contracture. The TLPA lies palmar to the neurovascular bundles but dorsal to the pretendinous and prelumbrical cords. As it is not involved in DD, most surgeons preserve it as it serves as a reference point for future surgery for recurrent disease.

More distally in the palm is located another superficial transverse ligament, the natatory ligament (NL). NL has attachments to the flexor tendon sheet in the midline ( Fig. 17.4 ). It lines the webs (see Fig. 17.2 ) and has extensions towards the fascial sheet on the lateral side of the digits, the lateral digital sheet of Gosset. DD commonly affects the NL leading to restriction of finger abduction. On the radial side of the palm the NL is continuous with the fascia of the first dorsal interosseus muscle, which may also be affected by DD, giving rise to the distal commissural cord ( Fig. 17.5 ).

The normal distal commissural band lines the first webspace and blends with the fascia of the thenar insertions near the MCP joint and the PT band of the thumb. On the ulnar side the NL blends with the fascia of the abductor digiti minimi muscle. This is almost always affected if there is a PT cord of the little finger and, if not excised during limited fasciectomy, predisposes to early recurrence. It may also occur as an isolated cord ( Fig. 17.6 ).

However, it is more common for the abductor cord to blend with fibers that are a continuation of the PT. The proximal edge of the confluence of the abductor and the PT cords is an important anatomical landmark to identify the ulnar digital neurovascular bundle, which can be found in the space immediately deep to where the two structures meet ( Fig. 17.7 ).

The region between the TLPA and NL defines the distal palm and contains a substantial amount of fat in between the flexor tendons of the fingers. Here, the common neurovascular bundles lie proximally and bifurcate more distally, and this is where the palmodigital spiraling system originates.

The pretendinous (PT) bands of the fingers pass volar to the TLPA and divide into three layers ( Fig. 17.8 ). The most superficial layer of the PT bands (layer 1) inserts into the palmar skin. Some fibers of this layer pass to the volar skin of the proximal phalanx, after crossing the NL superficially ( Fig. 17.9 ). Progressing distally, the number of cutaneous insertions reduces. When they turn into cords, these fibers may not be distinguishable from the fibers of the Grayson’s trabecular network of the proximal phalanx (see below). These layer 1 fibers can give rise to a central cord, leading to MCP joint contracture.

The second layer consists of two bands that diverge and run distally, passing deep to the neurovascular bundle. The classical description is that, distally, these fibers blend with the lateral digital sheet (LDS) of Gosset, a fascial condensation in the lateral plane of the finger ( Fig. 17.10 ). However, more recently it has been shown that these fibers have a different course and terminate on the dorsal aspect of NL, spiraling through a full 360° (see Fig. 17.4 ). These fibers are known in the literature as the spiral bands and form the most proximal contribution of the palmodigital spiraling system (PSS). When involved in DD these fibers thicken and shorten to form spiral cords that displace the neurovascular bundle medially and superficially, making the latter very vulnerable to injury during limited fasciectomy ( Fig. 17.11 ). Mobility of the skin overlying the pretendinous cord and the presence of fat in between the skin and the cord should alert the surgeon to the presence of a spiral cord and displacement of the neurovascular bundle (Short–Watson sign) ( Fig. 17.12 ).

The blending of the spiral band with the undersurface of the NL is important because its proximal border defines the location at which a displaced (spiral) digital nerve in the palm crosses a spiral cord superficially. The intrinsic muscle fascia also contributes to the palmodigital spiraling system proximally, just before the spiral band inserts into NL ( Fig. 17.13 ).

The spiral bands of adjoining fingers blend in the distal palm before inserting on the dorsal aspect of NL ( Fig. 17.4 ). This blending explains the formation of a Y-cord that seems to start in one ray and run into adjoining fingers. Therefore, a single strategically placed injection of collagenase can be performed to correct the MCP flexion deformities of two adjacent fingers.

Isolated layer 2 (spiral) as well as layer 1 (central) cords cause MCP joint contractures and can often be treated successfully and safely by percutaneous needle fasciotomy, providing the fasciotomy is not performed too close to the NL, especially in case of a Short–Watson sign. The clinical relevance of the various layers developing into central and spiral cords, and of contributions from the intrinsic fascia, is that not all MCP joint contractures can be completely released by percutaneous needle fasciotomy. The cords that are palpable immediately under the skin are a quick win. However, the spiral cord as well as the intrinsic contributions to PSS cords are located behind the neurovascular bundle and are too deep for safe access during a blind procedure.

The deepest PT fibers dive deep on both sides around the MCP joint to insert dorsally in the extensor sheet. These fibers perforate the DTIL on their way to the extensor sheet and are, therefore, also known as perforating fibers. If both sides of the flexor tendon are affected, they may cause triggering.

In addition to the proximal part of PSS described above, intermediate and distal parts can also be distinguished. The intermediate part of PSS is formed by fibers that originate from the A1 pulley (see Fig. 17.14 ). These fibers rotate up to 180° and join the best-developed part of Grayson's ligament, that covers the neurovascular bundle on the palmar side, and usually end at the A4 pulley, thus giving rise to a digital spiral cord in the finger.

In addition, some of these fibers together with the more distal origin of PSS on the A2 pulley blend with the part of Cleland's ligaments that runs immediately deep to the neurovascular bundle ( Fig. 17.15 ). The fibers that run from PSS to the lateral digital sheet can give rise to a cord that may also contribute to MCP joint contracture and have to be sought behind the neurovascular bundle during limited fasciectomy. We routinely remove them in an effort to prevent or delay recurrence formation.

The anatomical description of the digital fascial structures relevant to DD that bear the names of Cleland and Grayson has changed significantly since their first description and there are conflicting illustrations depicting their course ( Fig. 17.16 ). More recently their microanatomy has been re-evaluated. Grayson’s ligament is the most condensed part of a trabecular network of fibers located volar to the neurovascular bundle. It consist of fibers that originate from the outer surface of the flexor tendon sheath and terminate proximally on the contralateral side of the finger in an inverted V-shaped orientation, which becomes less pointed during flexion ( Fig. 17.17 ). This trabecular network ( Fig. 17.18 ) encompasses fat lobules and facilitates the adaptation of the skin to grasp objects when the fingers are flexed.

On the sides of the fingers, the fibers end in the deep dermis and also connect to the lateral digital sheets (LDS). The Grayson fibers that become affected form a cord that is located proximally in the finger lateral to the neurovascular bundle and distally blends with the A4 pulley. Therefore, it crosses the neurovascular bundle at the level of the proximal interphalangeal (PIP) joint and causes a PIP joint flexion deformity. In the case of a central cord with involvement of the midline part of the Grayson’s trabecular network, the contracture of the fibers can reverse the direction of the inverted V, resulting in the cord running from the midline at the level of the proximal phalanx to laterally beyond the PIP joint and further contributing to the PIP joint flexion contracture ( Fig. 17.19 ).

Cleland’s ligaments form paired structures located on each side of the finger, originating at the level of the PIP and distal interphalangeal (DIP) joints and course proximally and distally ( Fig. 17.20 ). Three layers have been distinguished at the level of the PIP joint: the most volar emerges from the fibers of the C1 and A3 pulleys of the flexor tendon sheet, the middle layer from the volar plate and the most dorsal layer, which is actually a double layer, encases the extensor apparatus. After joining just lateral to the volar plate, they fan out proximally and distally. They run dorsal to the neurovascular bundles (see Fig. 17.18 ). Although typically considered not to be involved with DD, a recent description has challenged this.

Recent microanatomical studies have revealed that the descriptions of the digital ligaments as forming a box-like structure may be too simplistic. Some of the digital fascia has been described as forming a continuous spiral around the neurovascular bundle, starting at the level of the proximal border of the NL and continuing distally at least beyond the PIP joint ( Fig. 17.21 ).

A detailed understanding of the spiraling nature of the normal fascia together with the observation that all parts may be involved in DD provides an explanation for the various patterns of cords encountered in clinical practice. However, whilst some patterns may be expected, there is always a chance of an unexpected finding during limited fasciectomy, leaving the neurovascular bundles vulnerable to inadvertent injury ( Figs. 17.22 & 17.23 ).

Clinical disease process

Approximately 20-40% of patients with early-stage DD progress to develop contractures and present with impaired hand function. Several subgroups can be distinguished based on their clinical presentation and likely outcomes ( Box 17.1 ).

In the first group, which is also the most prevalent, the disease tends to follow a more benign course. The patient is usually a male of over 60 years of age who has had a nodule for more than 10 years, has no associated disorders such as Ledderhose or Peyronie’s or comorbidities such as diabetes mellitus, does not consume more than 14 units of alcohol per week, and is a non-smoker. He usually has an isolated flexion contracture of the MCP joint of the ring or small finger of one hand. Any treatment will result in a good functional outcome, and the risk of recurrence and need for reintervention is inversely related to the aggressiveness of the treatment, so that the less invasive procedures such as needle fasciotomy are associated with a higher rate of recurrence.

In a second group of patients, the disease follows a more aggressive course. This is the group Hueston described as possessing Dupuytren diathesis. These patients usually have a strong family history of DD and disease onset is typically at a young (<40 years) age. It affects both hands, also involves the radial side of the hand and is commonly associated with fibromatoses such as knuckle pads, Peyronie’s and Ledderhose disease. The earlier disease onset and greater propensity to develop contractures mean that treatment is often required at a younger age. These patients also tend to develop recurrence earlier, necessitating additional and repeated interventions. Therefore, it is important to agree to a long-term management strategy with the patient at the outset as each subsequent procedure is associated with its inherent risks.

A third subgroup of patients that imposes an even greater challenge to achieve a good and durable result are the insulin-dependent diabetics. Their convalescence period is longer, and the functional outcome is often suboptimal, despite intensive postoperative hand therapy.

Finally, there is a fourth subgroup of patients that very much resembles the first group, but for unknown reasons, pose challenges during the postoperative period. They do not have any clear risk factors to facilitate identification but often develop a stiff hand despite intensive hand therapy and judicious splinting, and sometimes an early recurrence with worse flexion contractures than before treatment.

Clinical assessment

A detailed history including specific questions about risk factors (family history of DD, alcohol consumption, smoking, exposure to vibration), associated fibromatoses (knuckle pads, Peyronie’s and Ledderhose disease, frozen shoulder), and comorbidities (diabetes type 1 or 2, liver disease, epilepsy), is essential for the initial risk assessment. Physical examination of both hands and, if indicated, the soles of the feet and penis, will further complete the picture. During examination of the hands, attention should be paid to the extent of the disease, the presence of bilateral disease and the location in terms of affected rays and joints, as well as the degree of flexion contractures, which should be accurately recorded using a goniometer (see below). Some authors have described the use of a tumorimeter for nodule size and a durometer for nodule hardness for research purposes, but the benefit of these additional measurements for clinical practice has yet to be defined.