Dupuytren’s contracture is due to an abnormal thickening of the soft tissue of the hands and the fingers just beneath the skin. In this condition one or more fingers become bent into a flexion deformity. The thickening involves the palmar fascia in form of thick cords, firm pits, bumps or nodules forcing the fingers involved to bend into the palm. Small hard nodules just under the skin of the palm, worsens over time and may become tender.
People of Scandinavian or North European ancestry have often shown signs of the disease. Many have called this disease, the “Viking disease” even if it is also seen in Mediterranean countries like Spain and Bosnia. It is rarely encountered in people of Chinese or African descents. Men older than 50 in age, are more commonly victims of this condition and in 60 to 70% of the cases, there is a strong genetic predisposition.
The cause of the disease is not well understood although, younger men or women with northern heritage may show earlier signs of the disease around the age of 40. Hand injury has not proven convincingly to be a contributory factor although employees performing in construction work have demonstrated a higher risk in developing the symptoms. Other risk factors like family history, alcoholism, smoking (more than 25 cigarettes a day), thyroid disease, liver disease, diabetes mellitus are reported. People with HIV or Epilepsy have been also found to develop the disease. Thinner people have a tendency to be more prone to the disease as well.
An abnormal connective tissue in the palmar fascia is responsible for the symptoms and deformities. Clinically, the disease starts usually with lumps and pits within the palm, generally, hard in consistency but often they become adherent to the skin. It represents then a thickening of the skin itself or a nodule in the palm which rarely become tender to palpation. Soon a painless increasing loss of range of motion of the affected fingers will progressively be noted.
A typical sign of “puckering” of the skin in the palm is discovered when the flexor tendon become closer to the skin especially at the metacarpo-phalangeal level. Thick cords called “Pre-tendinous bands”, will develop in one or both hands from the palm extending to the fingers especially in the ring and the little fingers. Those bands can involve any finger causing a contracture. Often, the disease is found bilaterally. The cords contractures are painless but if tenosynovitis complicate the picture, patients will develop pain along the tendon with attempts at mobilization of the involved fingers.
A painful triggering can be a striking sign. The ring and little fingers as well as the thumb are most commonly involved. Late in the disease, the proximal interphalangeal (PIP) may also develop contractures. Observation and stretching exercises are recommended at that stage. Many will develop disease in the palm without any progression.
At the beginning, it is always recommended to observe while stretching exercises are enforced. A nodule or a lump in the palm does not mean that the disease will progress but in severe cases with contractures various options of treatment are offered to render the hand functional. Recently injectable medicine has been used to perform the lysis of the tendinous bands showing in good hands, satisfactory results.
The goal of the treatment is to improve motion of the fingers in allowing the hand to be able to apprehend objects. Unfortunately, a complete correction may never happen and the disease can recur more severely after initial treatment. Splinting and hand rehabilitation are imperative for a successful outcome. Steroid injections and physical therapy have been combined to help stretching the cords. Clostridial collagenase injections is becoming extremely popular while radiation therapy which has been used in the past to treat this condition, has failed to show any improvement. The condition may recur in spite of any treatment used.
Males are more commonly affected after the age of 50 and the disease is rare among Asians and Africans descents. There is a higher incidence of Dupuytren’s contracture in Norway where 30% of the population is affected compared to the United States where only 5% of the population is affected by the disease but inversely 20% of the population in the United Kingdom present signs of the disease after the age of 65. It is in 1933 that Guillaume Dupuytren described the disease which bear his name.
Clinically, the palmar fascia within the hands becomes abnormally thick, causing the fingers to curl and lose their mobility. We need to remember that the main function of the palmar fascia is to enhance the strength of the hand so it becomes obvious that the disease impends on the ability to hold on objects. Occasionally one will report pain, aching and itching while the bands are developing in the palm, bringing deformity to the fingers especially in the ring and little fingers.
A normal fascia consists in Type I collagen while in this disease a Type III collagen is also encountered. People with severe involvement of the hands will present with lumps on the back of the fingers (“Garrod’s nodes” or Knuckle pads or dorsal Dupuytren’s nodules). The disease can also involve the plantar fascia affecting the arch support. The same lumps or nodules can become symptomatic and require removal. Once the plantar fascia is involved, this disease takes the name of Plantar Fibromatosis or Ledderhose’s Disease.
The penis can also be involved in this process, presenting in an abnormal curvature but also bearing a risk in malignancy and bringing early death, although many studies have not been able to elucidate this mystery. Finally, many patients suffering from Dupuytren Contracture, will develop adhesive capsulitis of the shoulder.
The adhesive capsulitis and The Dupuytren contracture is a little different in the fact that it is more common in women and generally painful but has been found in relation with the pathology causing causalgia, complex pain syndrome. reflex sympathetic dystrophy, shoulder-hand syndrome.
An American Dupuytren Association was created by Charles Eaton MD who tried to classify the disease in 3 types:
1- An aggressive form of the disease (3%) affecting men under the age of 50 with a family history of Dupuytren presenting with knuckle pads and plantar Fibromatosis (Ledderhose disease). The term of “Dupuytren diathesis” has been coined to this Type 1 clinical picture.
2- The type 2 is the normal form of the disease involving mainly the hands starting after the age of 50 which can be also complicated with Diabetes and heavy manual labor.
3- The type 3 is the milder form, usually found in diabetics or in people taking anti-convulsants to treat epilepsy, or in HIV patients, is generally represented as a localized disease but not inherited.
Practically, another classification stages the disease according to the degree of bent in the contracted fingers. A stage I presented by a bent varying from 6 degrees to 45 degrees. A stage 2 demonstrating a bent of 46 to 90 degrees. A stage 3 presenting a bent finger between 91 degrees to 135 degrees and finally a stage 4l demonstrating a bent greater than 135 degrees.
The goal of the treatment in this condition is to restore the function of the hands by all means. A “table top test” is used to allowing the patient to place the hands flat on a table. The test is negative if one is able to flatten the hands on the table and it is positive if he is unable to do so. Different treatments can then be offered. Unfortunately, finger joints may become permanently stiff with the progression of the disease.
Radiation therapy, Needle Aponeurotomy, Collagenase Injection and surgical treatments have all been used. Recently, collagenase injections have become popular but it has its limitations in the different phases of the disease. Needle Aponeurotomy has been also recently been favored. Finally, limited or total palmar fasciectomy can be effective at any stage of the disease.
This is in June 1831 that Dupuytren performed the first surgical procedure on this condition to restore function of a ring and little fingers. He reported the procedure in the Lancet in 1834 for publication by the Hotel-Dieu de Paris, as a minimally invasive needle procedure. Unfortunately, a high incidence of recurrence was seen with this technique and newer procedures like fasciectomy and dermatofasciectomy have since been introduced helping in a wide resection of the contracted tissue in the hand.
A limited fasciectomy is a common approach to remove the diseased fascia especially when the disease is at an advanced stage. A tourniquet is used while the patient is asleep. Zig-zag incisions are preferred to longitudinal or transverse incisions while exploring the palmar fascia to remove the offending cords and the diseased fascia. Attention is required to avoid damaging the underlying neurovascular bundles. Injuries to the digital nerves or arteries are often seen as well as post-operative hematoma, wound infection and wound dehiscence, complicating the picture.
Occasionally, patients can develop a reflex sympathetic dystrophy or complex regional pain syndrome. Unfortunately, around 30% of the operated cases, will show recurrent disease in 3 to 4 years. Other surgeons in the new millennium have recently used a limited or selective fasciectomy under local anesthesia with epinephrine and no use of the tourniquet.
Dermatofasciectomy in the best way to avoid recurrent disease by removing the diseased cords, fascia and the overlying skin. The skin is then closed with a full-thickness skin graft generally taken from the antecubital fossa or the inner aspect of the arm. The recurrence is low when this procedure is used. Unfortunately, a segmental fasciectomy can only remove the contracted cords but macroscopically, active disease is left in place,
Percutaneous release, percutaneous Aponeurotomy with “lipografting” and collagenase have shown promising hope in the treatment. Needle Aponeurotomy is a minimally invasive procedure able to weaken the cords at their insertion with a needle. These cords are transected at many levels using a 25-gauge needle mounted on a 10 cc syringe. This will allow the mechanical snap of the offending cords by exercising pressure over the fingers.
This procedure is practically performed in the office under local anesthesia and does not require any rehabilitation. The MCP (Metacarpo-phalangeal) joint will gain more range of motion than the PIP’s (Proximal interphalangeal joint). Complications rate are low while the recurrence rate is frequent especially in younger person. The Aponeurotomy can become selective or extensive while 5 to 10cc of the liposuction material (Lipograft), taken from the abdomen or the flanks, is injected into the operated hand. This fat graft results in supple skin.
The digits are then placed on maximal extension to keep the cords under tension and multiple palmar puncture wounds are done to facilitate the cutting of the cords, after the injection of the “lipograft”. The patients are offered an extension splint for about a week, prior to resuming their activities. A night splint may be recommended for 3 weeks after.
Radiation therapy has not been proven to be an effective form of treatment at any stage of the disease. Vitamin E has gained in popularity for a while when the theory of free Radicals was considered as a factor in the pathologic findings of the disease but has since been refuted. Red and Infrared Lasers have been considered also in the treatment but was never been fully evaluated.
Surgery remains in the hand of many surgeons remains the more effective way in treating Dupuytren contracture although post-operative splinting and hand therapy may be needed to prevent stiffness. Static and dynamic splinting have enhanced finger mobility but the choice remains a preferential one. Self-exercises and stretching are encouraged. Personally, I have always added Vitamin C as an adjuvant to the armamentarium.
In conclusion, there is a high recurrence rate encountered in the treatment of this disease and it becomes even more difficult to be treated especially when both hands are involved especially the little fingers. Considerations for early onset and plantar involvement or pads” on the radial aspect of the hands with knuckle pads can be given for surgical removal if symptomatic
Peyronie’s disease is the development of a fibrous scar tissue inside the penis rendering all erections painful while curving the penis. Many patients will not worry about the curvature of their penis and I have met some who refused any consultation with the urologist by fear of losing their successful intercourse.
Significant bent upward, downward or sideward can impend on the function of the penis or can produce pain. The curvature might worsen or stabilize with time. Medication like, Pentoxifylline has been tried to reduce the scar tissue. The FDA has also approved Collagenase Clostridium Histolyticum (Xyaflex) injections and promising results are reported in many studies alone or in combination with other medication like the blood pressure medication Verapamil or Interferon which appear to disrupt the fibrous tissue.
Finally, surgical treatment can be offered when all has failed to restore the anatomical alignment of the penis. Plication of the affected side or removal of any scar tissue in the shaft of the penis may be successful. The last resort remains on the insertion of a penile implant, to restore alignment.
I do not have the pretention of being the expert in the topic but I wanted simply to bring to light all the medical and surgical options offered in the treatment of such debilitating disease. I have practiced as a hand surgeon for years and treated patients with Dupuytren’s contracture to improve their ability to grasp. I would expect all lectors interested in learning more about associated complications to look for consulting with urologists or orthopedists or plastic surgeons, colleagues and specialists involved in the care of patients suffering with such disease, at the AMHE or elsewhere to further discuss the matter, since newer techniques may have surfaced from institutions specializing in this rare pathology.
Maxime Coles MD
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