3. Introduction
1) Dupuytrenâs disease is a
â Benign fibromatosis of the palmar and digital fascia characterized by nodular
thickening and subsequent contractureâ.
2) Functional limitations result from primary involvement of MCPJ and PIPJ.
3) This disease process presents a challenge in terms of both treatment and prognosis.
4. Epidemiology
ī Highest among individuals of northern European ancestry (2% to 42%)
ī Men are six times more affected (2:1 to 10:1)
ī With aging, the male-to-female ratio starts to equalize
ī Typical onset is
1) Fifth decade of life for men
2) Sixth decade of life for women
5. Etiology
ī Exact cause is unknown.
ī Genetic and environmental factors are thought to play a role.
ī A number of genetic studies show:
1) Autosomal dominant pattern of inheritance.
2) Genes regulating collagen breakdown are inhibited
3) Genes promoting development of collagen in epidermis are upregulated
6. Etiology
Environmental factors thought to be associated with DD include
Environmental Factors implicated in DD
1) Smoking and alcohol consumption
2) Anti-seizure drugs
3) Diabetes mellitus
4) Human immunodeficiency virus
5) Hypercholesterolemia
6) Hypertriglyceridemia
7) Frozen shoulder
8) Rock climbing
7. Pathophysiology
1) There is transformation of fibroblasts to myofibroblasts.
2) There is imbalance of collagen ration
3) Histologically three stages have been described
Stage Histological changes Gross outcome
Proliferative
stage
Fibroblast-myofibroblast proliferation Nodules form
Involutional
stage
- Collage type III increases
- Fibro-myofibroblasts reduced
Partial Cx (<90) of fingers
across MCPJ
Residual stage Collagen type III predominates
Fibro-myofibroblasts highly reduced
Cx >90 in affected fingers
involving MCPJ and PIPJ
8.
9. Clinical features
1) Insidious onset with progression over time.
2) Palmar skin pitting or dimpling (earliest sign)
3) Distortion of the skin creases can appear as a deepening of the crease or widening
of the crease.
4) Nodule formation (over ulnar aspect of ring finger 1st !!)
ī Painless
ī Discomfort while grip
10.
11. Clinical features
5) Longitudinal pathological cords
ī From palm extending into fingers distally.
ī Ring finger > small finger>thumb>middle>index finger
ī ShortâWatson sign.
ī MCPJ involved first followed by PIPJ
(DIPJ typically spared)
12. Clinical features
ī Common complaints include
1) Impairment in hand shaking
2) Difficulty in fitting of gloves
3) Difficulty placing the affected hand in a pocket
4) Impairment in grasping large objects.
ī Cords are divided anatomically into palmar, palmo-digital, digital, thumb and 1st webspace
cords
13. Pertinent Anatomy and Patho-anatomy
Palmar anatomy and patho-anatomy
Pathological
cord
Anatomical entity Origin/insertion Clinical significance
Pre-tendinous
cords
Pre-tendinous
(Longitudinal) bands
Continuation of palmaris fascia
Inserts distally in dermis at distal palmar
crease and at MCPJ
MCPJ contracture
Vertical cords Vertical fibers Join dermis of skin with palmar aponeurosis
and separate flexor tendon sheaths from
septum containing digital NVB and lumbricals
Causes painful triggering
of fingers
14.
15. Pertinent Anatomy and Patho-anatomy
Palmo-digital anatomy and patho-anatomy
Pathological
cord
Anatomical
entity
Origin/Insertion Clinical Significance
Spiral cord Spiral band It consists of Graysonâs ligament, lateral
digital sheath and pre-tendinous band
Displaces the NVB
medially and superficially
Natatory cord Natatory band Extends from the radial border of the index
finger to the ulnar border of the small finger
Webspace adduction Cx
17. Pertinent Anatomy and Patho-anatomy
Digital anatomy and patho-anatomy
Pathological cord Anatomical origin Orign/Insertion Clinical Significance
Central cord Digital slips of
pre-tendinous
bands
Continuation of pre-tendinuous band that
inserts distally at periosteum of middle
phalanx and FTS of PIPJ
PIPJ Flexion Cx
Lateral cord Natatory ligament
and lateral digital
sheath
Continuation from natatory ligament and
lateral digital sheath in digits and inserts at
periosteum of distal phalanx
PIPJ and DIPJ flexion Cx
Retrovascular cord Retrovascular
fascia of Thomine
Longitudinally oriented fascia located dorsal to
the neurovascular bundle
PIPJ and DIPJ flexion Cx
ADM cord ADM fascia Arises from the musculotendinous junction of
ADM.
PIPJ flexion Cx
19. Pertinent Anatomy and Patho-anatomy
Thumb + 1st web space anatomy and patho-anatomy
Pathological cord Anatomical Entity Origin/Insertion Clinical
Significance
Proximal commissural
cord
Proximal commissural ligament Radial continuation of
natatory ligaments into 1st
web space
1st web space
adduction
contracture
Distal commissural
cord
Distal commissural ligament Radial continuation of
proximal transverse palmar
ligament into 1st web space
Thumb
pre-tendinuous cord
Thumb pre-tendinous band Radial longitudinal fibers of
palmar aponeurosis
inserting at MCPJ
MCPJ flexion Cx
21. Clinical features
6) Ectopic disease may be found outside the palmar fascia:
7) Other reported associations with no clear relationship
ī Involvement of auricular concha
ī Presence of nodules in tensor fascia lata in DD
ī Nodules in Achilles tendon in DD
Ectopic disease Description Frequency
Garrodâs pads Dorsum of PIPJ 2-44%
Peyronieâs disease Penile fibromatosis 2-4%
Ledderhoseâs disease Plantar fibromatosis 5-20%
22. Dupuytrenâs Diathesis
ī Refers to the presence of certain patient and/or disease characteristics that may indicate a more
aggressive form of DD in terms of disease severity and recurrence risk
Dupuytrenâs Diathesis
1) Bilateral palmar disease
2) Family history of DD
3) Ectopic disease
4) Ethnicity
5) Early onset of disease (<50yr)
6) Male gender
23. Pre-op Patientâs Evaluation
ī Diagnosis of DD is purely clinical, based on history and physical exam.
1) History taking
ī Family history of DD
ī Age of onset
ī Speed of progression
ī Impact of the disease on the patientâs daily life
ī Ectopic disease
ī Environmental exposures
ī Significant comorbidities
24. Pre-op Patientâs Evaluation
2) Physical examination
ī Looking for skin dimpling, nodules and cords
ī Careful measurements of each contracture in advanced
disease (goniometer)
ī Table top test for mild disease with minimal
/absent Cx
25. Pre-op Patientâs Evaluation
ī Consider the condition of the hand tissues, the rate and extent of the contracture, and
the patientâs capacity to participate in a hand therapy program.
ī Communicate bluntly with the patient about complexity of procedure and
possible complications.
27. Treatment
1) The primary objective is correction of deformity, thereby reducing disability and restoring
hand function with minimal number of operative procedures.
2) Treatment options include:
Treatment Options for Dupuytrenâs Disease
Non-surgical treatment -
Minimally invasive options 1) Percutaneous (Needle) aponeurotomy
2) Colleganse injection
Surgical options 1) Fasiotomy
2) Segmental fasiectomy
3) Limited fasciectomy
4) Total (Radical) fasciectomy
5) Dermatofasciectomy
Adjunctive procedures 1) Skeletal traction
2) Lipofilling
Last resort procedures 1) Amputation
2) Arthrodesis
28. Non-Surgical Options
ī This array of therapies underscores both a physician and patient desire to develop
non-operative alternatives to palmar fasciectomy as well as the general
ineffectiveness of these modalities.
ī These include:
Options Description
Pharmacological therapies Steroid (intralesional, topical, oral, IM), Vitamin E and
hyperbaric oxygen therapy
Physical therapy - Extension splinting
- Ultrasound therapy
- Heat treatment
- Frictional massage
Radiotherapy - External beam radiotherapy
29. Minimally invasive surgeries
1) Needle aponeurotomy
ī Office based procedure done under L/A
ī Uses a hypodermic needle to disrupt the cord/s
responsible for Dupuytren contractures.
Advantages Disadvantages
Rapid recovery Steep learning curve
Minimal
discomfort
Higher recurrence rate
High patient
satisfaction
Small risk of tendon and/or digital nerve
injury
Lower cost -
30. Minimally invasive surgeries
Percutaneous aponeurotomy and lipofilling (PALF)
ī After percutaneous aponeurotomy to disintegrate the Dupuytren cord, infiltration
of the area with autologous lipoaspirate (lipofilling).
ī Prevention of recurrence by replacing the deficient subdermal fat
ī An alternative to limited fasciectomy .
31. Minimally invasive surgeries
2) Collegenase injection
ī Mixture of two enzymes from Clostridium histolyticum that cause lysis and rupture of
contracted collagen cords.
ī Injected percutaneously into a cord, followed by manipulation.
ī Only one cord at a time should be treated (three inj. to each cord 4 wks apart)
Disadvantages
1) Localized bruising and pain
2) Occasional lymphadenopathy
3) Manipulation related skin tears
4) Flexor tendon rupture (rare)
5) Allergic reactions
6) Expensive
33. Surgical options
1) Open Fasciotomy
ī Skin opened by longitudinal incision and cord incised transversely
ī Has largely been replaced by percutaneous needle fasciotomy.
ī Small skin incision allows visualization of the N.V bundles.
ī Postoperative care:
1) Soft bulky dressing removed after 1-3 days
2) Patient encouraged to use there hands as tolerated
3) Extension splinting at night for 3-4 months
34. Surgical options
2) Segmental (local) fasciectomy
ī Short segment of diseased fascia is excised as a âfire breakâ.
ī Less invasive than other fasciectomy techniques.
ī Trade-off between shorter recovery time and higher recurrence rate.
ī Its role is limited and overlaps with that of fasciotomy.
35. Surgical options
3) Limited fasciectomy
ī Most common operation for DD.
ī Only the relevant diseased fascia is excised.
ī Skin incisions preferred for extensive exposure to resect
diseased fascia and release of Cx are:
1) Brunner incisions
2) Modified Brunner incisions
3) Longitudinal incision with Z-plasties
36. Surgical options
ī Skin flaps are elevated and nondiseased subcutaneous tissue should be left on the skin
flaps to preserve as much blood supply to the skin as possible.
ī Dissection of the diseased cord proceeds from proximal to distal in most cases, starting in the
proximal palm.
ī In proximal palm, deeper, transverse fascial fibers remain disease-free. If dissection remains
superficial to these fibers, the underlying superficial arch and common digital vessels and nerves are
protected.
ī Once dissection reaches the distal third of the palm, the transverse fascia is no longer present, and
the neurovascular bundles should be identified before continuing the dissection into the distal palm
and digit
37. Surgical options
ī Excision of diseased fascia should proceed until all contractures are released.
ī If no palpable cords or nodules remain, and joint contractures persist, then some
intrinsic joint contracture may be responsible, part. In PIPJ Cx
ī In such cases, it is best to proceed with PIP joint capsulotomy and/or ligamentous
release in selected cases in following order
1) Check-rein ligaments
2) Transverse incision of the proximal volar plate
3) Accessory collateral ligaments
4) Flexor tendon sheath.
38.
39. Surgical options
ī Full extension may still not be possible due to fibrosis of the NVBs or central slip
attenuation.
ī Both can be addressed by post-operative splintage/hand therapy/transarticular K wire
to maintain joint extension.
ī Such patients should be counselled for higher risk for contracture recurrence.
40. Surgical options
ī Skin closure is performed, incorporating Z-plasties or Y-to-V advancements
ī If complete skin closure is not possible, full thickness skin grafts can be placed which
are believed to act as firebreaks.
ī In contrast wounds can be left open as in McCash open palm technique
41. Surgical options
4) Radical fascietomy
ī All palmar and digital fascia is excised, whether diseased or not.
ī Originally thought it might prevent recurrence.
ī Has fallen out of favour due to high complication rates but similar
recurrence rates to limited fasciectomy.
42. Surgical options
5) Dermato-fasciectomy
ī Skin gets involved in DD due to attachments with palmar fascia.
ī It difficult to find a plane of dissection between an adherent fascial nodule and the
overlying dermis in advanced or recurrent disease.
ī In these cases, wide resection of involved skin and fascia is done
ī Full thickness skin graft is applied afterwards.
44. Surgical options
Postoperative care after fasciectomy
ī Volar splint with MCP and PIP in full extension
ī If no skin graft:
1) Splint removed at 3-5 days with active and passive ROM
2) Antibiotic gauze dressing of suture lines
3) Stiches removed after 14 days
ī If skin graft in place:
1) Bolster taken down at day 5
2) ROM started after day 7
ī Hand therapy
45. Adjunctive procedures
Soft tissue distraction with skeletal traction
ī Involves application of a dorsally based external distraction device
ī Causes slow steady stretching to help overcome problems of foreshortened skin and
other soft tissues.
ī Skin deficiency, vascular compromise due to stretched arteries, and intrinsic PIP joint
contractures are addressed .
ī Makes subsequent surgery easier and avoids amputation
46. Adjunctive procedures
ī Full extension is typically achieved over
the course of 2â4 weeks.
ī Allows for ready access to the palm and
a regional or dermatofasciectomy is
performed concomitant with removal of
the distraction device.
47. Last resort procedures
1) PIPJ Arthrodesis
ī Indications include:
1) Underlying articular or degenerative change
2) Pain upon restoration of motion,
3) Immobility in a non-functional position
ī MCP arthrodesis should be avoided
48. Last resort procedures
2) Amputation
ī Clear indications include
1) Multiple recurrent contractures
2) Severely contracted fingers with nerve or vascular lesions
3) Hygiene issues and ongoing disabilities
ī Ray amputation is preferable for the index, long, and ring fingers.
ī Amputation at the MCP joint level is indicated to address small-finger contracture.
ī Thumb amputation is contraindicated regardless of the severity of contracture.
49. Guidelines for treatment
British Society for Surgery of the Hand (BSSH) guidelines
Mild No functional problems, no contracture or MCPJ
contracture <30â.
1) Observation (table top test)
2) Follow-up in 6â12 months.
Moderate Functional problems or MCPJ contracture 30ââ60â Âą
PIPJ contracture <30â Âą first webspace
contracture.
1) Needle fasciotomy for isolated MCPJ Cx
2) Collagenase
3) Limited fasciectomy
Severe Severe contracture MCPJ >60â and PIPJ >30â. 1) Limited fasciectomy
2) Dermofasciectomy.
50. Guidelines for treatment
ī In addition to absolute degrees of contracture, rate of progression is important
(Huestonâs tabletop test)
ī Mild flexion of MCPJs is not an indication for surgery.
ī In contrast, the PIPJ flexion Cx is a stronger indication for surgery
ī Patients with greater contracture pre-operatively, particularly of the PIPJ, usually have
incomplete correction and higher complication rates.
51. Recurrence
ī Delphi method defines recurrent contracture as
â Passive extension deficit of more than 20° for at least one treated
joint, in the presence of a palpable cord, compared to the result
obtained at time 0. Time 0 is further defined as between 6 weeks
and 3 months after an interventionâ
ī Special considerations include
1) The patient must have an obtainable goal in mind.
2) Full correction of the contracture is unlikely.
3) Dissection is more difficult and takes longer.
52. Recurrence
4) A careful preoperative examination is needed
to identify previously damaged nerves and vessels.
5) Incidence of neurovascular injury is higher during repeat
fasciectomy
6) Recovery and postoperative therapy are more intense.
7) Finally, ancillary procedures, such as PIP joint release, PIP joint fusion, or arthroplasty
or amputation, may be needed.
53. Outcome
ī Physical outcome most commonly reported in DD studies is range of motion.
ī Patient-reported outcomes to evaluate the benefits of one procedure over another from the
patientâs perspective are
1) UnitÊ UnitÊ Rhumatologique des Affections de la Main (URAM) scale (Most validated)
2) Disabilities of the arm, shoulder, and hand (DASH) questionnaire
3) Michigan Hand Outcomes Questionnaire
Superoxide dismutase, matrix metalloproteinase-3, cysteine dioxygenaseι-smooth muscle actin, β-1 integrin, collagen I, V and VIII, fibronectin, laminin)
These associations do not imply a cause-and-effect relationship, and not all studies verify these relationships. It does seem, however, that these subpopulations are more frequently affected with Dupuytrenâs disease with the primary diagnosis in these patients causing changes at the tissue and molecular level.
Type III collagen predominating over type I collagen
The characteristic progression from nodule to fibrotic cord with contracture of the affected palm and digits is pathognomonic for Dupuytrenâs
DiseaseHugh Johnson sign
A soft palpable fullness immediately adjacent to the cord at the level of the MCP joint may indicate displacement of the neurovascular bundle by a pathologic spiral cordâĻâĻâĻâĻDIPJ flexion deformity may occur from involvement of the lateral and retrovascular cord.
DIP hyperextension, in contrast, is compensatory in the setting of PIP flexion contracture, and results from imbalance of the flexor and extensor mechanisms.
ī Initially limitation of hyperextension at MCPJ ī then lack of extension ī then gradual flexion of involved digits ī permanent flexion Cx
The palmar aponeurosis is a triangular-shaped fascial structure consisting of longitudinal, transverse and vertical
fibers with its apex in continuity with the palmaris longus tendon
ī DD patients with all six diathesis factors have recurrence risk of 71%.
ī DD patients with no diathesis factors have recurrence risk of 23%.
The classic history of nodule appearance with insidious progression to cord formation and joint contracture is pathognomonic for Dupuytrenâs disease
Positive table top testâthe palm and fingers cannot be simultaneously placed on the flat surface of the tabletop. This test is often used as a simple tool to help patients monitor their disease: if their contractures progress to a point where they can no longer flatten their hand on a table, they should be reevaluated for additional treatment.
Steroids and radiotherapy have been proved the most effective therapies overall. But radiation therapy adds additional risk of radiation induced malignancies.
Manipulation is performed in a controlled fashion. Placing the wrist in flexion for MCP manipulation and the MCP in flexion
during PIP manipulation relieves stress of the underlying flexor tendons, isolating the cord.
Collagenase,
0.58 mg in 0.25 mL of diluent, One third of 0.58-mg dose placed in three separate areas in cord.
weekly dressings with non-adherent gauze until healed)
Hand therapy (full finger flexion, followed by progressive restoration of finger extension)- in cases of severe Cx
as articular change does not occur with prolonged contracture at this joint and fusion at the MCP joint level is functionally limiting.
Informed consent be taken and counselling regarding phantom sensation or persistent pain due to complex regional pain syndrome should be done before hand.
MCPJ collateral ligaments are stretched in a flexed position. In contrast, the PIPJ volar plate and capsule shorten in a flexed position which allows them to contract over time, resulting in fixed flexion deformity.
The resulting URAM scale is a 9âitem patientâreported questionnaire with total scores for Dupuytren's diseaseâassociated disability ranging from 0 (best) to 45 (worst). High scores suggest high levels of disability and disturbance