Also called as the benign fibromatosis of the palmar and digital fascia, Dupuytrens’s disease develops in the palmar ligaments causing pathological changes in them.
It is considered to be a genetic disorder with autosomal dominance (Variable penetrance). An altered beta catenin pathway has been implicated in the pathogenesis of the disease. Common in the 6th decade with males affected more than the females 3:1. The digits more commonly includes the 4th and the 5th.
- Early presentation associated with high recurrence rates.
- More prevalent in Diabetics with more complication rates after surgery for dupuytrens.
- Also associated with smoking, chronic alcoholism, AIDS and vascular diseases.
- It is a pathologic fibroproliferative disorder.
Diathesis Group by Hueston ( Associated with multiple surgeries and significant hand impairment)
- Positive history of 1st and 2nd degree relatives.
- ectopic deposits beyond the palmar surface (knuckle pads, plantar surface, penile tissue like peyronies disease)
- Early Age younger than 50 years (some say 40 )
- Bilateral severe radial disease
Palmar skin is attached to the skeleton by the retention ligaments which keep the Skin in place during processes like gripping and pinch. This connective tissue can be divided into:
- Digital fascia
- Palmar fascia
- Palmar-digital junction fascia
- Lateral digital sheath.
- Superficial fibrofatty palmar/dorsal fascia.
- Cleland’s ligament (dorsal to neurovascular bundle).
- Grayson ligament (volar to the neurovascular bundle).
- **Cleland ligament not involved in the disease process.
- Pretendinous bands.
- Superficial transverse bands.
- Vertical septa (these structures are mentioned superficial to deep)
Palmar digital fascia
- Spiral band.
- Natatory ligament.
- Vertical fibre of Legueu and Juvara.
- ** In digit these attach to proximal portion of Grayson ligament and lateral digital sheath
- ** In palm they connect the pretendinous bands with the superficial transverse ligaments.
Dupuytrens’s results from deformation of normal anatomic structures (ligaments or bands) to abnormal structures (cords).
- Pretendinous aponeurosis changes to pretendinous cords.
- Natatory ligament changes to natatory cords (natatory cords contract the web space from side to side and prevents fingers from separating)
- Central cord originates from the pretendinous bands and the palmar superficial fibrofatty fascia. This cord causes combined MP and PIP joint contractures.
- Similarly lateral cords, retrovascular cords are also formed.
- Spiral cords form by 5 separate structures namely pretendinous bands, spiral band of gosset, lateral digital sheath, vertical band and grayson ligament. Natatory ligament not involved with the spiral band.
- spiral cords pass deep to the neurovascular bundle to reach the side of the finger . Then it passes superficial to the bundle to reach its attachment at the base of the middle phalynx.
- As it shortens it pulls the neurovascular bundle to midline and in a more superficial position prone to damage during surgery.
- Retrovascular ligament itself does not cause a PIP contracture but with lateral cord it can hyperextend the DIP contracture.
- Lateral sheath proximally coalesce to form natatory ligaments and the spiral ligaments. They are deficient on the ulnar side of little fingers. But the tendon of ADM can be found pathological in 25% of affected cases. it does not displace neurovascular bundle.
- Histology: clinical nodule has a lot of smaller nodules that are non-encapsulated but surrounded by connective tissue and collagen bundles. Predominant cell in early disease is fibroblast making collagen (type 3 instead of type 1). Diseased state one finds myofibroblasts with smooth muscle actin production.
MP joints usually affected by the pretendinous cords. MP joint of thumb never crosses 30 degrees due to weak pretendinous cord.
PIP joint affected by the following cords in the order as central cord (maximum) followed by spiral and lateral cords (minimum)
DIP joint is rarely involved ( if it gets involved retrovascular and lateral bands are to blame)
Difference between MP and PIP joint contracture
MP joint has a cam like effect with its eccentric centre of orientation relative to the collateral ligaments which are stretched when the joint is flexed. Thus after the release of the contracting cords don’t tether the joints and MP joint easily returns to extension. MP joint contracture more than 30 degrees is an indication for surgery.
PIP joint contractures when present lead to collateral ligament and volar plate shortening leading to some intrinsic contracture even after release of cords. PIP contracture of 20 degrees or progressing is indication for surgery (some say any PIP contracture should be intervened)
- Functional limitations
- Measure the angles of each contracture ( especially Dynamic contractures). MP if held in neutral extension maximises the PIP contracture. if MP joint is flexed the recordable PIP contracture is reduced significantly.
- Quality of overlying skin with nodules in palmar, plantar and penile areas
- History of Carpal tunnel syndrome.
- clinical diagnosis only: no biopsy needed, no imaging needed until arthritis present.
- joint movements in sequence from thumb to little, deviation of fingers from axis.
- Kaplan line
There is no surgical cure for Dupuytrens’s disease. Surgical goal is to improve the hand function. Surgery does not eliminate the disease. Options include
- Needle fasciotomy
- closed fasciotomy
- open fasciectomy
- enzymatic fasciectomy (experimental)
Scenario 1: No contracture, no pain : tightness only no contractures. These patients are treated with reassurance and follow up at regular interval for progression of the disease.
Scenario 2: Painful Palmar nodules: Don’t excise, put steroids. excision only if it is associated with trigger finger or causing unrelenting pain.
Scenario 3: Dorsal Dupuytrens’s disease: Diathesis Group by Hueston, monitoring or excision as per patients choice.
Scenario 4: Established contractures
- Needle aponeurectomy: cord weakened by percutaneous needle insertion and broken by finger manipulation. It is more effective for MCP than PIP.
- may cause some neuropraxia, tendon rupture and frequent recurrences.
- recurrence rate expected high as diseased tissue not removed.
- Put local Anesthetic only superficially so that the digital nerve is spared and patient can warn paresthesia indicating needle is close to the nerve.
- 25 gauge needle used to perforate the cord in multiple region along the area of 5 square mm up and down motion not sawing action.
- After multiple passes finger manipulates and ruptures cord.
- Release any residual cords if any.
- useful when skin is not involved.
- Limited fasciectomy: standard treatment.
- Removal of diseased cord.
- Amount of tissue removed is individualised.
- splintage till suture removal (10-14 days) followed by active physiotherapy several times a day.
- Night splintage continued later on 3-4 months.
- reduces recurrence rates, more durable
- May cause nerve or vessel injury (level of palmar digital nerve and vessel should be identified), hand stiffness
- long rehabilitation time.
- Dermatofasciectomy and SSG:
- More aggressive procedure.
- especially for PIP
- Skin involved, Residual disease or young patients.
- FTG applied.
- Distraction : for PIP contracture where distractor used for stretching the soft tissue. After adequate stretching limited fasciectomy is done and distractor removed.
- Collagenase (clostridium histolyticum)
- effective for MCP joint
- hand injected with medicated injection as per label.
- hand manipulated after 24 hours to rupture the coed
- bruising and swelling are expected
- McCash technique: open fasciectomy without skin closure.
- Segmental open fasciectomy.