Introduction:Dupuytren's contracture is a inherited proliferative connective tissue disorder resulting in fixed flexion contracture of the hand. [1,2,3] This is a slowly progressive painless disease in which palmar fascia becomes hyperplastic and contracts. This involves most commonly the ring finger and little finger. Men are affected more often than females and incidence increases after age of 40 years. [4] The age wise distribution as per a study done in Norway is shown in picture 4. [5] Pathophysiology:The thickening of palmer fascia is associated with change in type of collagen and affects the grip of the hand. [6] This follows a course from starting as a nodule in the palm to cord in the finger. It is worth noting that not every patient with Dupuytren's disease will develop bent fingers. [7] Specifically when the disease starts beyond the age of 60 many patients never get beyond nodules and possibly cords, and don't experience a severe extension deficit of their fingers. [8]. The study done by G.H dolmans et al. found genetic association to Dupuytrens disease. They found that nine different loci were involved in increased susceptibility to Dupuytrens disease. These include Wnt- signaling pathways that are key to process of fibromatosis in the disease. [9] A few associated conditions in form of Peyronie's disease (curvature of the penis), Ledderhose disease (callus under the foot), Garrod's knuckle (pads on the back of knuckles of fingers) may also be seen along with Dupuytrens contracture. [10, 4] Risk factors for the disease: [11, 12, 13, 14, 15, 16, 17, 18, 6, 7, 10]1)

2) 3) 4) 5)

People of Scandinavian or Northern European ancestry; it has been called the "Viking disease" or "Celtic hand," though it is also widespread in some Mediterranean countries (e.g. Spain and Bosnia) and in Japan; Men Age > 40 years Positive family history People with liver cirrhosis

Trauma, diabetes, alcoholism, epilepsy therapy with phenytoin, and liver disease, occupational factors are also associated with increased incidence. Dupuytrens disease has a high recurrence rate, especially when a patient has so called Dupuytrens diathesis. The term diathesis relates to certain features of Dupuytren's disease and indicates an aggressive course of disease. [19,11,12,13]. The initial description of Dupuytrens disease diathesis included 4 factors:1) 2) 3) 4)

the patient is below the age of 50 years old positive family history both of the hands are affected Ectopic lesions (Peyronies disease, Knuckle pads and Ledderhose disease).[19]

Diagnosis Before making the final diagnosis the following differential diagnosis should also be considered: [4, 7, 13]y y y y y y y y y y

Callus Epithelioid sarcoma Ganglion cyst Stenosing tenosynovitis Giant cell tumor of the tendon sheath Prolapsed flexor tendon Ulnar nerve palsy Camptodactyly Fibromas and fibromatoses Palmar tendinitis.

The disease is best diagnosed by clinically examination of hand which also allows determining stage of the disease as the disease progresses through stages of nodules (lumps of tissue), dimples or pitted marks, thickened skin and flexed (bent) fingers. The condition affects everyone differently, so specific symptoms and problems in carrying out daily activities should be looked for as some people are troubled by quite a minor deformity, while others can cope with a greater one. [7, 10, 11, 12, 13] Another test to diagnose Dupuytrens contracture is the Hueston Table top test which is positive when patient is not able to lay his/her palm flat on the table due du contractures. This test may not be evident in very early stages. [7, 10, 11, 12, 13] Recently, Ultrasound examination of the palm of the hand to see the thickened tissue is also used its cost effectiveness in comparison to clinical diagnosis is not certain. The grading/staging of the disease is done as shown in table and picture 2,3. [20] Treatment Historically first procedure was described by Baron Guillaume Dupuytren in 1831 which was a minimal invasive needle procedure. Since then newer surgical techniques have been introduced, such as the fasiectomy, dermofasciectomy fasciotomy and also amputation. The fasciectomy is seen as the golden standard treatment for Dupuytrens disease.[21] Since the patient burden after open surgery is high, therefore less invasive techniques may be preferred. Newer modalities as percutaneous release, extensive percutaneous aponeurotomy with lipografting, and collagenase. [22,23,24,25,3] Several alternate therapies, including radiation and vitamin E treatment, have been tried in the past.[26,27] None of these treatments

have proved to be a way to stop or cure the condition permanently. In extreme cases, amputation of fingers may be needed for severe or recurrent disease, or after complications in surgery. [28]

A) Non surgical methods 1) Enzyme Injections (Collagenase) Injectable form of enzyme collagenase extracted from Clostridium histolyticum was approved for treatment In February 2010. The enzymatic treatment works by weakening the cords after breaking the peptide bonds in the collagen. Further data on long-term results, complications and rate of recurrence with the use of this emerging treatment option is rewuired for making it as first line of treatment of Dupuytrens disease. [29,30,31 24,25] Although this form of treatment provides the benefits of avoiding the potential surgical complications such as nerve injury, hematoma and skin necrosis, initial studies showed a recurrence rate of 67% in the MCP joint and 100% in the PIP joint. [32] 2) Radiation therapy

Radiation therapy treatment of Dupuytren's contracture with low energy xrays is used, has shown some promising results in trials lacking a control group. Seegenschmiedt et al Radiation therapy is useful in early stages of the disease and has been found to halt disease progression (Picture 5) [33] Treatment with radiation is applied to prevent disease progression. In radiation therapy, the nodules and cords associated with Dupuytren's are irradiated from a distance of 0.5 1 cm either with X-Rays or with electrons This is typically done over five days in a row applying an efficient dose (single dose 3 Gy, total dose 15 Gy). After a break of six weeks, this treatment is repeated. Typically this softens the nodules or cords and prevents contraction of the hand. [34] Betz et al in their 13 years follow up of patients who received radiation therapy concluded that radiotherapy is effective in prevention of disease progression and improves patients' symptoms in early-stage Dupuytren's contracture (stage N, N/I). [35] B) Minimally invasive surgical methods A) Percutaneous Needle Fasciotomy Needle aponeurotomyr or simply needling is a minimally-invasive technique where the cords are weakened through the insertion and manipulation of a

small needle. The cord is sectioned at as many levels as possible in the palm and fingers, depending on the location and extent of the disease. Once weakened, the offending cords can be snapped by putting tension on the finger(s) and pulling the finger(s) straight. After the treatment a small dressing is applied for 24 hours. After these 24 hours patient are able to use their hands normally. No splints are used or physiotherapy is given.[36] The advantage of needle aponeurotomy is the minimal intervention and the very rapid return to normal activities without need for rehabilitation. The major disadvantage of this procedure in high recurrence rate as the nodules are not removed and might start growing again.[37,36] A study reported postoperative gain is greater at the MCP-joint level than at the level of the IP-joint and found a reoperation rate of 24%; complications are scarce.[38] Needle aponeurotomy may be performed on fingers that are severely bent (stage IV), and not just on early-stage Dupuytren's contracture. However a recent studies showed 85% recurrence rate after 5 years.[37,38] B) Extensive Percutaneous Aponeurotomy and Lipografting A recently introduced technique to treat Dupuytrens disease is extensive percutaneous aponeurotomy with lipografting. In this procedure, initially a liposuction is done to the abdomen and ipsilateral flank to collect the lipograft. Percutaneous cutting of cords with needle is done at many levels. The fingers are stretched to break the cords and lipograft is put in the space created. Postoperatively, extension splint is used for 5 to 7 days. Normal activities are advised and night splint is used up to 20 weeks. This technique appears to be promising, because it shortens the recovery time, also the fat graft results in supple skin. [40,23]. Prospective randomized studies with other techniques are in process to fully determine its role in the treatment of Dupuytrens disease. C) Extensive surgical methods 1) Limited fasciectomy The limited or selective fasciectomy is widely been seen as the golden standard treatment for Dupuytrens disease. Therefore, the limited fasciectomy is commonly used procedure around the world.[40,41, 38] The skin is opened with a Zig-Zag incision. After the incision is made, all diseased cords and fascia are excised. The excision of the cords and fascia has to be very precise to spare the neurovascular bundles. After surgery the hand is wrapped in a light compressive bandage for one week. Patients should start practicing bending and extending their fingers as soon as the anesthesia has resolved. Hand therapy is recommended. Approximately 6 weeks after surgery patients are able to completely use their hand. Complications seen with this form or treatment include digital nerve injury, digital artery injury, infection, hematoma and complex regional pain

syndrome. Minor complications such as painful flare and wound healing problems are also seen. [42] 2) Dermofasciectomy Dermofasciectomy is a surgical procedure that is mainly used in recurrent Dupuytrens disease. [43] Just like the limited fasciectomy, with the dermofasciectomy all the diseased cords and fascia are excised. With the cords and the fascia, the overlying skin is taken out as well. Full-thickness skin graft usually taken from the elbow flexion crease or the proximal inner side of the arm is used for cover. After around 2 weeks when the skin graft has stabilized, the mobilization is done intensively. Recurrence of the disease after this procedure is very low. [44] 3) Free vascular flaps In severe cases of Dupuytrens disease a free vascular flap may be preferred to treat the disease. The incidence of recurrence is too low with this form of treatment. O.A. Branford et al recently presented one-year follow-up of one patient where circumex scapular artery perforator ap was used for reconstruction. This patient did not suffer of recurrent disease. [45] Postoperative care: This mainly includes hand therapy and splinting. Hand therapy is prescribed to optimize the hand function after surgery and prevent the patient from joint stiffness. The splint is used to provide prolonged stretch to the healing tissues and prevent flexion contractures. Although splinting is a widely used post-operative intervention, the evidence of a specific indication of splinting and its effectiveness remains scarce. For similar reasons, there is lot of variation in the way of splinting. [46, 47] 4) Recent advances and alternative therapies There are evidences which suggest benefit of different substances in treatment of Dupuytren's patients. These include Quercetin, Bromelain, DMSO, MSM, Acetylcarnitine Hcl, PABA, Nattokinase, Vitamin E, Copper, Vitamin C . Trials are required to ascertain the usage of the above substances in first line treatment of Dupuytrens contracture. [26, 27, 28]

Conclusion Dupuytrens contracture despite being disease, requires early diagnosis and treatment due work progressive course, morbidity and work related problems. Causative associations even at microscopic level are being investigated. Although exact cause is still not clear Variety of treatment methods and their modifications have been mentioned with different results. Precautions during surgery as tensioning the cords tight before cutting and dissecting the tissue with scalpel only may reduce the surgical

complications. Post operative exercises increase the effectiveness of the surgery. Role of postoperative splintage is doubtful. Recurrence of the disease has been found to be high even with surgical procedures, but till now, best results have been found with limited fasciectomy. References

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