Information Regarding Specific Ailments

Dupuytren’s Contracture

The contracture of the skin of the palm and progression to the contracture of one or more fingers into the palm bears the name Dupuytren’s contracture after the Frenchman who wrote about the disease and operated on patients with it one hundred and seventy years ago. Although sizable nodules can form on the arch of the foot, contracture of the toes does not occur and it is the contracture of the fingers that is disabling and brings patients to seek treatment. Aside from digital contractures following trauma, Dupuytren’s contracture is far and away the most common cause of contractures of the fingers. The process is genetic in origin affecting the fair skinned races and is rarely seen in dark skinned races. It is uncommonly seen under the age of 40 but the incidence increases with every decade so that  in Australia and northern Europe, two of three men aged 80 have the contracture. The incidence is slightly greater in men and usually more pronounced in men than women. Although the cause is still obscure, at some point in time, usually over age 40 there is a genetic trigger that causes fibrous tissue cells in the upper dermis of the skin of the palm to secrete an abnormal protein called type III collagen. Collagen is a protein found in connective tissues all over the body but type III collagen is not normally found in the hand. The cells that secrete this abnormal collagen begin to proliferate in one location most commonly at the distal crease of the palm in line with the ring finger. These cells, along with the type III collagen and new blood vessels, form a nodule which is a small mass that is visible, palpable and sometimes tender. As the process of proliferation continues, it progresses along a scaffolding of normal connective tissue called palmar fascia that is located between the skin and the tendons and extends from the base of the palm into the fingers. The proliferation of abnormal tissue is always found on the superficial side of the fascia and never the deep. It extends towards the fingers producing a thickening called a cord. As the cells proliferate and secrete type III collagen, this collagen attaches to the fascia by chemical cross-linking. A small percentage of the cells in the nodule convert into an energy intensive phase in which for a short period of time they have contractile properties; as they contract, they cause the overlying skin and palmar fascia to which they are cross-linked to bunch up or contract; eventually those cells revert to normal fibroblasts and other fibroblasts develop the contractile properties. As the process proceeds into the fingers, a series of nodules develop; they act as train engines in line with each other, producing a series of contractile sequences that result in one or more fingers being drawn in to the palm. The patient finds him or herself in the awkward situation of not being able to shake hands, put on a glove, or get change out of a pocket; even cleaning one’s face becomes a formidable chore.

The conventional approach to treatment of Dupuytren’s contracture is to do nothing until there is a 20-30 degree contracture of a digit at the metacarpophalangeal or proximal interphalangeal joint. At that point, surgery is usually recommended, the procedure of choice being a limited fasciectomy whereby the visible nodule(s) and cord(s) are excised, which results in significant straightening of the affected digits; however, there are two potential problems with limited fasciectomy. First, there is a recurrence rate of 40-50% usually within the first two to three years postoperatively, as quoted by Tubiana from a series of reports on recurrence in Hueston and Tubiana, the definitive text on Dupuytren’s contracture. Secondly, the limited fasciectomy procedure leaves in place the overlying dermis which has been shown by Fitzgerald et al, using differential histological staining, to be the site of origin of the nodules.

This brings us back to the early stage of the disease when the nodules have been recently formed and diagnosed. It has been our experience that at this early stage before the contracture of a digit develops, a nodule can be softened and flattened by a series of usually three injections of Triamcinolone, a form of cortisone. We have been injecting nodules in patients with Dupuytren’s contracture for 29 years and recently observed a four year window in that experience. Over 90% of the injected nodules responded after a series of injections by softening and flattening and in some cases, the process remained dormant for many years. If it exacerbated, another series of Triamcinolone injections was administered. In this way, if found before digital contracture occurs, a majority of cases can be ameliorated and the course of the disease changed. Complications of the injections are atrophy of the skin around the injection site and depigmentation of the skin; both the atrophy and depigmentation usually resolve within six months of the last injection. The injections are not effective if a digital contracture has developed. In that instance, surgical intervention is the treatment of choice.

At this point, a word about the Dupuytren’s Diathesis is in order. John Hueston, M.D.who has recently been deceased, was for many years the bellweather in the treatment of Dupuytren’s contracture. Through many years of experience with the disease, he observed that if a patient had one or more of five traits, he or she had a partial or total potential for the Dupuytren’s Diathesis, which is the full expression of the disease. Those five traits are: 1) a positive family history of Dupuytren’s contracture; 2) onset of the disease prior to age 40. As mentioned, this is uncommon but I have seen one patient develop it at age 8, two in their teens, and several in their twenties and thirties. Early onset is the harbinger of a more aggressive form of the disease; 3) bilateral disease. There is a subset to this facet of the disease. Dupuytren’s contracture usually involves the ring and/or little finger. Disease on the radial side of the hand i.e. thumb and/or index finger is a signal to expect a more aggressive form of the disease; 4) extrinsic disease. This refers to development of nodules in areas outside of the palmar surface of the hand, namely the plantar surfaces of the feet and dorsum or back of the interphalangeal joints of the fingers. Areas of thickening in this latter site are called knuckle pads and correlate with a high percentage of recurrent disease of the palms; 5) the last facet of the diathesis is recurrent disease, a self-prognosticator of an aggressive form of the disease.

In releasing a significant digital contracture from Dupuytren’s disease, it is frequently necessary to resurface open areas with full thickness skin grafts because of skin contracture and a relative lack of skin when a finger is straightened. Many authors going back to the early 1900’s observed that when a full thickness skin graft is used for that purpose, seldom if ever is recurrent disease seen under the graft. Hueston was the first to put that observation to use in treating patients with a contracture who had one or more facets of the Dupuytren’s Diathesis. He did this as a prophylactic procedure to decrease recurrence. Early in the 1970’s, I began using full thickness skin grafts in this way and found, as others did, that recurrent disease did not occur beneath the grafts; however, tantalizingly, new disease, which is called an extension, did develop and skirted around the grafts. This was very frustrating until I began to enlarge the grafts. If there was no disease on the radial side of the hand (thumb and/or index finger) the procedure I have used for the last 25+ years has been a dermofasciectomy and full thickness skin graft in which a minimum of one centimeter of skin (this includes the dermis) is removed along with diseased fascia all the way down to tendon sheaths and nerves and blood vessels. This resection is done at the distal crease of the palm and from between the index and middle fingers to the mid-lateral line of the little finger, i.e. the mid-point between the palm and dorsum (back) of the hand. If there is Dupuytren’s disease on the radial side of the hand (thumb and/or index finger) the dermofasciectomy extends over the entire width of the hand at the distal crease of the palm with a width of tissue of at least one centimeter. The full thickness skin grafts are taken from the inner side of the upper arm with the scars being very acceptable. By using these large grafts, the incidence of recurrence and extension combined has been decreased from 40-50% to 8%. The dermofasciectomy procedure is only used if an individual has one or more factors of the "Dupuytren’s Diathesis", which indicates a greater tendency toward recurrent disease. Otherwise a conventional limited fasciectomy is employed.

Carpal Tunnel Syndrome

Carpal tunnel syndrome involves primarily numbness of the thumb and adjacent three digits; although the little finger is occasionally involved, it is usually excluded in carpal tunnel syndrome. Aching of the hand and wrist are frequent symptoms, as is weakness and the tendency to drop things, but the essence of the disease is numbness and tingling. Although compression of nerves in the neck from a herniated disc or osteoarthritic spurs can produce similar symptoms, by far the most frequent cause of those symptoms is compression of the median nerve in the carpal (meaning wrist) tunnel. The boundaries of the carpal tunnel are a strong broad ligament (transverse carpal ligament) which forms the roof and the bones of the wrist which form the floor. The tunnel contains ten structures which pass through it, the median nerve which supplies sensation to the thumb and three adjacent digits, as well as motor innervation to most of the muscles of the thumb; in addition, there are nine tendons which flex the fingers (two per finger) and one for the thumb. Each tendon has a sheath around it.

In carpal tunnel syndrome, symptoms occur from compression of the median nerve; such compression interferes with the blood supply to the nerve which leads to diminished function i.e. decreased transmission of electrical impulses and resultant paresthesias (pins and needles) and diminished ability to feel small objects or differentiate textures. The median nerve does not compress itself. There must be a space occupying lesion to compress it, such as a tumor (ganglion cyst or fatty lipoma), a displaced bone i.e. dislocated carpal bone or fracture fragment of the radius, an inflammatory process such as inflammation of the tendon sheaths from rheumatoid arthritis, thickening of the tendon sheaths from a metabolic process such as with pregnancy, hypothyroidism or amyloidosis or thickening of the tendon sheaths from mechanical causes such as work hypertrophy in which repetitive stress on the tendons in people doing moderate to heavy manual work or highly repetitive work (carpenters, plumbers, electricians, etc) results initially in edema of the tendon sheaths and later fibrosis or scarring of those sheaths. This last process is the cause of well over 50% of carpal tunnel syndrome cases.

Carpal tunnel syndrome can occur acutely in a matter of days or weeks but usually it occurs after many years. Although the infrequent acute case can resolve with conservative treatment or with completion of a pregnancy, definitive care usually requires surgical decompression of the median nerve. The procedure that has been the gold standard in treatment of carpal tunnel syndrome has been the open carpal tunnel release where, through an incision 3-4 centimeters at the base of the palm the transverse carpal ligament is divided. Both halves of the ligament separate 3-4 millimeters and that interval fills in with scar tissue re-establishing the ligament but in so doing expanding the capacity of the carpal tunnel and taking the pressure off of the nerve. In most cases this is all that is necessary to definitively treat carpal tunnel syndrome; however in 15-20% of cases so treated, pillar pain, a tender thickening on either side of the scar, can prevent patients who use the base of their palm in their work (carpenters, plumbers, etc) from returning to regular duty for several months.

Endoscopic carpal tunnel release was developed to avoid pillar pain but not only can pillar pain occur after endoscopic carpal tunnel release but since visualization of the contents of the carpal tunnel is less than with direct visualization, incomplete division of the ligament can occur, anatomical variations are not appreciated as readily and there is an increased incidence of damage to tendons, arteries and the median nerve. Furthermore, both procedures do not address the not infrequent presence of robust amounts of hypertrophied flexor tenosynovium (tendon sheath) that is one of the most important causes of increased pressure in the carpal tunnel, because its presence occupies space in the tunnel. With that tissue being left in place, as the patient returns to doing moderate to heavy manual work or highly repetitive skilled work, continued hypertrophy of the tenosynovium can occur, further packing the carpal tunnel and resulting in a recurrence of problems in two to three years in a small but definite percentage of patients up to (15-20%). A repeat carpal tunnel release is of limited value because the scarring from the initial surgery prevents retraction of the edges of the incised transverse carpal ligament which occurred at the first carpal tunnel release. In that situation, removal of the thickened tenosynovium is the answer as that procedure, tenosynovectomy, decompresses the median nerve by removing the space occupying tissue. It has been shown that following a radical tenosynovectomy for rheumatoid arthritis, the recurrence of that synovium is less than 10%.

The hypertrophied tenosynovium from work hypertrophy is a much more benign and less aggressive process than that in rheumatoid arthritis so that a tenosynovectomy for work hypertrophy either alone as a primary procedure for the initial treatment of carpal tunnel syndrome or combined with an open carpal tunnel release for recurrent carpal tunnel syndrome can be expected to yield a recurrence rate well below 10%.

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