​Kembara Xtra - Medicine - Dupuytren Contracture

​Kembara Xtra - Medicine - Dupuytren Contracture 
Introduction 

Palmar fibromatosis, which results in flexion deformities and functional loss due to increasing fibrous proliferation and tightening of the palm fascia. 
Not the same as "trigger finger," which is brought on by thickening of the distal flexor tendon, a similar change in the plantar fascia almost never happens and typically manifests concurrently.
impacted system(s): musculoskeletal
The aggressive heritable variant of Dupuytren's disease is characterized by an initial age of 40, bilateral presentation, including radial digits, plantar fibromatosis (Ledderhose), and penile fibromatosis (Peyronie).

Synonyms include "Celtic hand," "Dupuytren disease," "morbus Dupuytren," palmar fascial fibromatosis, and palmar fascia contracture.

occurrence and prevalence 

Prevalence rises with age; in Western countries, the mean prevalence at ages 55, 65, and 75 is 12%, 21%, and 29%, respectively. More common in Caucasian men of Scandinavian or Northern European descent, the mean age of onset is 60 years, with the average age range of onset between 40 and 80 years. Norway: 30% of males >60 years; Spain: 19% of males >60 years.

Pathophysiology and Etiology 
Unknown definitive cause; possible causes include oxidative stress, improper wound healing, and/or aberrant immunological response.

Three steps are involved (Luck classification): - Myofibroblasts are proliferating and developing nodules on the palmar surface during the proliferative phase. Myofibroblasts extend along the palmar fascia to the fingers during the involutional stage, and more type 3 collagen is produced during the residual phase, which causes the cord to tighten and contractures to form. 
Genetics
Autosomal dominant and only partially penetrant: - Siblings have a threefold risk
68% of the male relatives of afflicted people eventually get sick.
Potential relationship with HLA alleles

Smoking (mean 16 pack-years, odds ratio: 2.8) is one risk factor.
Male/Caucasian; male > female (range: 3.5:1 to 9:1); increasing age
Vibration exposure and manual labor: weekly exposure increases risk
Middle and ring finger involvement; rises with duration of diabetes mellitus; usually moderate; excessive alcohol use; Northern European heritage; family history; hand trauma; low body weight and BMI


Prevention 

Avoid risk factors, especially if there is a history in the family.

Alcoholism is a condition that is related.

Epilepsy (variable data)

Chronic pulmonary disease (DM)

Vibration-related occupational hand injuries, hypercholesterolemia, carpal tunnel syndrome, Peyronie disease, and HIV

Adhesive capsulitis of the shoulder and cancer

Diagnoses are typically made clinically based on the history and physical examination.

A Caucasian male with a history of family illness and the other risk factors mentioned above.
iADLs are frequently reported to be impossible to complete. Gradual emergence of a nodule on the palm that was initially painless progresses to include: - Pain of a palpable nodule - Loss of function of the affected finger.

Most frequently, the ring or middle finger, but any digit can be affected
The proximal and distal interphalangeal joints are rarely damaged; the metacarpophalangeal joint is most frequently impacted.


clinical assessment
Visual examination and palpation of the afflicted area of the palm to check for nodules or bands that resemble cords
Check to see if the injured finger has contracted.
More frequently seen in ulnar digits
- Hueston tabletop test: instruct the patient to put their hands flat on the surface; if they are unable to do so, the test is positive. - Garrod nodes—knuckle calluses linked to a severe disease development
staging for Tubiana  - 0: no illness, no extension deficit
- I: 1 to 45 degrees of extension deficit (surgical referral is indicated) - N: no extension deficit, nodule visible on exam II: Extension Deficit 46 to 90 Degrees III: Extension Deficit 91 to 135 Degrees IV: Extension Deficit >135 Degrees

Multiple Diagnoses 
Early teens with tight fascial bands on the ulnar side of the small finger have camptodactyly. Diabetic cheiroarthropathy affects all four fingers. Volkmann ischemic contracture. Trigger finger (thickening of the distal flexor tendon. Ganglion cyst.

Laboratory Results 
Initial Tests (Lab, Imaging) The diagnosis is made primarily on the history and the physical exam; testing is not always necessary. MRI can evaluate the cellularity of lesions that are associated with post-operative recurrence.

Management 
There is no surefire treatment. Patients need to understand that there is a risk of recurrence with both surgical and nonsurgical treatments.

General Approach: Extension splinting, physical therapy for range of motion, observation for mild disease, and surgical referral as indicated below.

Medication 
Steroid injections can be used to treat painful knuckle pads or acute nodules.
- When used in conjunction with needle aponeurotomy, serial triamcinolone injections enhanced long-term outcomes (4).[B].
- Steroids by themselves are 50% more likely to reoccur in one to three years.
Injections of clostridial collagenase (FDA-approved 2010): Best for isolated cord of MCP joint; 5-year recurrence rate of 47%; comparable to surgical recurrence rates (5). Degrades collagen to enable manual rupture of diseased cord.[B]
- Faster hand function recovery than with restricted fasciectomy, with less severe side effects (6)[B]
Skin tearing and injection site reactions are potential complications. Two cords can be cut simultaneously.

Initial Line

Injections of clostridial collagenase (FDA-approved 2010): Best for isolated cord of MCP joint; 5-year recurrence rate of 47%; comparable to surgical recurrence rates (5). Degrades collagen to enable manual rupture of diseased cord.[B]
- Faster hand function recovery than with restricted fasciectomy, with less severe side effects (6)[B]
Skin tearing and injection site reactions are potential complications. Two cords can be cut simultaneously.
Steroid injections can be used to treat painful knuckle pads or acute nodules.
– When paired with needle aponeurotomy, serial triamcinolone injections improved long-term results.
- Steroid use alone is 50% more likely to reoccur in one to three years.

Referral 

Referrals for orthopedic surgery are necessary when: PIP joints are involved; MCP joints are contracted >30 degrees; function is impaired; or there is a disabling deformity.


Further Treatments 
Better for MCP joints in patients with comorbid diseases; lower complication rate but higher recurrence; improvement of 93% compared to 57% for PIP joint; recurrence common; 50%; shown to be beneficial for recurring disease.

The results of collagenase injections and needle fasciotomy are equivalent at 3 months and 1 year.


Surgical Procedures 
Segmental aponeurectomy, restricted fasciectomy, and dermofasciectomy: - Higher complication rates and more initial correction compared to nonincisional treatment
– Compared to restricted fasciectomy, percutaneous aponeurotomy and lipofilling (PALF) appears to have a faster recovery period, fewer long-term sequelae, similar operative contraction correction, and no appreciable differences in outcomes at one year (8).
Indications include: - Any PIP joint involvement - MCP joints constricted by at least 30 degrees
- A positive Hueston tabletop test (the patient cannot flatten his or her palm on a table).  Severe cutaneous shrinkage wound closure may need skin grafts.  With early surgery, 80% of patients have complete range of motion.
If the fifth digit is severely malformed, amputation MCP joints heal more quickly from surgery than PIP joints, especially if they are contracted more than 45 degrees.


Continuous Care 
patient observation
regular follow-up every six to twelve months
Avoid risk factors, especially if there is a significant family history (alcohol, vibratory exposure, etc.).
Mild disease: Passively extend your fingers twice daily, and refrain from repeatedly clutching objects.
Unpredictable but typically slowly progressing in nature  10% may spontaneously regress.
Dupuytren's disease foretells an aggressive path. Age 50 years, family history, bilateral lesions outside the palm, and ethnicity (Nordic) are characteristics that increase the chance of recurrence by 71% compared to the baseline rate of 23% in the absence of risk factors.
Following surgery and collagenase injection, MCP joints had a better prognosis than PIP joints.


Complications 
Operative nerve damage, Complex Regional Pain Syndrome, and Postoperative Recurrence in 46-80%
Limited hand function, postoperative hand edema, and skin necrosis are all possible side effects.

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