Definition

Dupuytren disease (DD) is a benign fibromatosis of the palmar fascias in which nodules and skin pits in the distal palm are usually the first signs. As it progresses, cords are formed which in time may contract limiting extension of the fingers and causing webspace contractures. The disease usually starts in the ring finger and little finger ray, but may affect any ray in a variable pattern. The same is true for the age at which the first symptoms occur. In the majority of cases the disease emerges in people of over 50 years of age. In the younger age groups, males are more affected than females. Systematic meta-analysis of the published work on prevalence has revealed it to range from 0.6 to 31.6%. The disease may affect all races, although prevalence of DD has not been studied extensively in other than Caucasian races (only incidence reports).

Similar fibromatoses may affect the fascias in the instep area of the foot (Ledderhose disease (LD)), the penis (Peyronie disease (PD)), and the knuckles of the finger joints (Garrod pads). LD can cause pain during walking. Toe contractures are only sporadically seen. In PD contraction of plaques formed within the tunica albuginea may cause penile curvature during erection, sometimes hampering sexual intercourse.

Impact of DD and its Reflection in Patient-Rated Outcome Measures

The impact of DD on the patient varies with the demands the patient has. Awkwardness and functional incapacity are usually direct consequences of increasing contracture, especially when the total extension deficit of a finger exceeds 90 degrees, turning the finger into a hook that inadvertently catches objects, that cannot be easily released. Specific common complaints may be difficulty to put on gloves or getting one’s hand into a pocket, eye poking with the bent fingers during facial wash and embarrassment in social hand shaking. Dupuytren contractures may interfere with various job-specific tasks and functionally impair in numerous ways. Primary DD in general does not limit finger flexion.

It is not easy to quantify the disabilities, since most general upper limb impairment scores do not reflect the specific impairment of the contractures, nor their effect on performance of activities or quality of life. There are a few disease-specific questionnaires, but they have been found to have their limitations in use too.

Variable Disease Course of DD

In the majority of DD cases contractures do not develop and it is good to realize that in clinics a subset of the affected population is seen; those who become patients do so mostly because they have developed contractures. Within this group, a further subset of patients can be distinguished that challenges surgeons the most, due to an aggressive disease course (Dupuytren diathesis): in these patients, whose family members often also are affected, the disease typically starts at a young age, affects both hands, also involves the radial side of the hand and is commonly associated with fibromatoses such as knuckle pads, PD and LD. Since these patients develop contractures earlier in life, treatment is often needed at a younger age. Because the disease activity is higher, they often develop (early) recurrences – especially of the little finger – which may demand additional and repeated surgery, which ultimately may inflict damage to neurovascular (NV) bundles and even gangrene.

In the symptomatic population in general, DD is often found to recur or progress and should therefore be regarded as a chronic disease that at present cannot be cured. Therefore, a thoughtful individually tailored treatment strategy is indispensable. On one hand, the aim is to correct the disabling contractures with as minimally invasive treatment options as possible. On the other hand, more aggressive measures sometimes need to be considered in those cases, where recurrence is almost inevitable. This chapter aims to summarize the current knowledge of the various aspects of this disease and give some guidelines for treatment.

Palmar and Digital Fascias

In the palm the normal fascial structures form a three-dimensional network consisting of longitudinally, transversely, and sagittally running fibers. The longitudinal fibers are organized in a triangle and form the palmar aponeurosis. In this triangle, pretendinous and prelumbrical bands can be distinguished, the former being more condensed than the latter. They all pass superficial to the transverse ligament of the palmar aponeurosis (TLPA), which is situated at a line joining the proximal and distal palmar crease. The ulnar border of the TLPA forms a crossroad with fibers of the hypothenar fascia and the radial border is continuous with the proximal commissural band, which runs through the first webspace and ends in the thenar fascia at the level of the first metacarpophalangeal joint. Just beyond TLPA, the pretendinous bands divide into three layers ( Fig. 55.1 ). Layer 1 is the most superficial layer, which has its insertion in either the skin of the distal palm or the proximal phalanx. Layer 2 fibers form the so-called “spiral band,” since it spirals around the NV bundle on its course towards the finger. This band is present on both sides in all long fingers, except for the ulnar side of the little finger. Layer 3 is the deepest layer of fibers, which takes a sagittal course into the hand on both sides of the flexor tendon sheath to insert on either side of the corresponding metacarpophalangeal (MCP) joint.

Schematic drawing of the relevant palmar fascial structures at the palmo-digital junction. The pretendinous fibers can be seen to divide in three layers. Layer 2, the spiral band, courses around the neurovascular bundle. a , pretendinous band (PT); b , Transverse ligament of the palmar aponeurosis (TLPA); c , natatory ligament (NL); d , continuation of the spiral band as it extends into the digit. 1 , layer 1; 2 , layer 2 or spiral band; 3 , layer 3.

From McGrouther DA. The palm. In: Flint MH, McGrouther DA, McFarlane DA. Dupuytren Disease: Biology and Treatment . Edinburgh: Churchill Livingstone; 1990: Fig. 12.3.

Another important transverse fascial structure is the natatory ligament (NL). This ligament lies just underneath the skin in the distal palm and is situated superficial to the NV bundles (see Fig. 55.1 ). On its ulnar end it is continuous with the hypothenar fascia, and on the radial side with the distal commissural band. NL has attachments to the flexor tendon sheet, lines the webs, and has extensions along the lateral sides of the digits. It also blends with fibers from the spiral band and is continuous with the lateral digital sheet. It is important not only because it may be affected, but also because its proximal border defines the place where a spiral nerve superficially crosses a spiral cord, making it vulnerable during surgery.

The vertical or sagittal ligaments in the palm worth mentioning are the septa of Legueu and Juvara that are usually just as wide as TLPA and, except in the thumb, connect the longitudinal fibers at the level of the TLPA, and TLPA itself with the deep transverse palmar ligament that runs between the volar plates of all MCP joints. On cross-section, the TLPA and the deep transverse ligament, together with the ligaments of Legueu and Juvara, form nine boxes, which on an alternating basis hold either the lumbrical muscle and NV bundles, or the flexor tendons of each ray. Much smaller vertical fibers are dispersed throughout the palmar aponeurosis and anchor it to the overlying skin.

The digital fascial structures relevant to Dupuytren disease bear the names of Cleland and Grayson. Their detailed anatomical description has undergone significant changes over time and drawings depicting the exact course of their fibers have been conflicting. Recently their microanatomy has been redefined ( Fig. 55.2 ). ^, Grayson ligament as it was described in 1941 appears to be the most condensed part of a trabecular network of fibers that is located volar to the NV bundle and consists of fibers that originate from the outside of the flexor tendon sheet and end in the deep dermis on the contralateral side of the finger. The fibers have an inverted V-shaped orientation, which becomes less pointed during flexion. Since the trabecular network encompasses fat lobules, it seems to accommodate the adaptation of the skin to objects held in the flexed finger hands.

Schematic cross-section through the distal proximal phalanx, showing Cleland ligament and Grayson trabecular network.

From Zwanenburg RL, McGrouther DA, Werker PMN. Grayson ligament: a revised description of its anatomy and function. J Hand Surg Am. 2019:44(4):341e1–341e6:Fig. 4.

Regarding Cleland ligaments, they have been found to be oriented in pairs located on each side and originating at the level of the proximal interphalangeal (PIP) and distal interphalangeal (DIP) joints, and from there they course proximally and distally. Three layers have been distinguished: the most volar layer emerges from the fibers of the C1 and C3 pulleys of the flexor tendon sheet, the middle layer from the volar plate, and the most dorsal layer, which actually is a double layer, circumvents the extensor apparatus. After joining just lateral from the volar plate, they fan out proximally and distally, and run dorsal to the NV bundles.

At the place where the hypothenar muscles pass into the little finger there is a blending of fibers that run in all three directions, and they all seem to play a role in Dupuytren disease. At the thenar and in the first webspace there is also a complex three-dimensional network of bands, which may also play a part in the disease.

Band and Cords

There is consensus about the role of most of these fascial structures in the disease ( Table 55.1 ). If a well-defined band becomes affected by disease, it is no longer called “band” but becomes “cord.”

At present it is believed that the transverse ligament of the palmar aponeurosis (TLPA) is not affected by DD. Because of this, and since this structure lies palmar to the NV bundles but dorsal to the pretendinous and prelumbrical cords, most surgeons agree that it can be left in situ, and as such serves as a reference point during secondary surgery. Distal to TLPA, however, the dissection always needs to be performed with great care as the NV bundle may be displaced by a spiral cord medially and volarly and may become so intimately related to it that only the experienced eye can discern it. Fat present between a cord and the skin in between TLPA and NT, should raise a high suspicion of a spiral cord. In 2010, Hettiaratchy et al described a number of cases in which a double spiral around the digital nerve was encountered, and in these cases, at least one of the spirals was found in the finger. McFarlane tried to simplify the often complex phenotype of the disease in the finger by defining three anatomical patterns of disease: the central cord, the lateral cord, and the spiral cord. Further work is required to be able to explain all these findings, including those of Hettiaratchy et al, during surgery.