It has been called many names, from the ‘Viking Disease,’ due to its prevalence among people of Northern European ancestry, to palmar fibromatosis. But to most patients and doctors, it is named for the French surgeon Baron Guillaume Dupuytren, who described an early treatment for the condition.
Dupuytren’s disease is a contraction of the fingers due to abnormal thickening of tissue. At the Hand Center of Western Massachusetts in Springfield, Drs. Richard Martin, Jeffrey Wint, and Bruce Wintman told HCN that the treatment of the disease has just been given a big helping hand. Two of them, in fact, in the form of new procedures that do nothing short of transform the way patients are given relief.
To get a better understanding of the mysteries of Dupuytren’s, which doesn’t have any specifically known causes — only a series of predispositions in patients — Wintman described the symptoms.
“There are different types of tissue in the hand,” he explained. “There’s skin, muscle, bone, and also a tissue that strands throughout the soft parts to give it form, called the fascia. In Dupuytren’s, your body makes a little bit too much of it, and an abnormal type of it at that.
“As a result,” he continued, “you start to get nodules in the palm into the fingers, and sometimes these can coalesce into a cord of tissue that can actually curl the finger down. That cord can be so thick that you can’t actually straighten it out on your own, even though the joints are normal and tendons are working. That abnormal fascial cord is flexing the fingers down. And that’s what patients complain about, that inability to straighten out those fingers.”
There is no pain particular to the abnormal fascia, but the lack of mobility can lead to problems with work and quality of life.
“It can become so cumbersome, and for some people can become so severe,” Wint added, “that we’ve had some patients say, ‘if you can’t do anything for it, cut the hand off. I just can’t do anything with it.’ That’s the extreme, though. Here, we tend to see anything from simple nodules to cords.”
Historically, the treatment for Dupuytren’s involved an open surgical procedure to remove the abnormal tissue, a process which also involves several months of physical therapy to restore mobility in the hand. However, new developments in the treatment of this condition have severed that downtime to the extent that a patient can have one of two new procedures — done in the office and not the surgical theater — and have immediate results with little aftereffect.
“In some cases,” Wintman said, “a patient can have one of these procedures during the week and be golfing by the weekend.”
This month, HCN sat down with the doctors of the Hand Center to find out what these new processes are, and how they are giving an important helping hand to the treatment of a life-altering condition.
Dupuytren’s is, as mentioned, a bit of a mystery, for there are no proven causes. But doctors say there are certain risk factors, including geographic ethnicity, age (over 40), a history of alcohol use, and, perhaps most importantly, those with a family history of the condition. While the disease is most commonly seen in men, it is not uncommon for women to contract it.
When the first signs of Dupuytren’s are noticed, the tendency is not to take immediate action, Martin said.
“If you get a little nodule, a little bump, there’s not a lot that needs to be done,” he explained. “However, when that becomes a cord, that’s the time when most people feel the need to do something about it. In years past, traditionally we would wait on treatment until it became a significant problem for the individual.”
Wintman said the “flat-table test” is a point of entry for his patients. “It’s usually not a pain situation whereby someone will want to seek treatment, but rather a lifestyle condition,” he explained. “If you can’t lay your hand flat on the table, then you may wish to proceed with treatment.”
For many years, what happened next was a pretty straightforward surgical process. If the nodule or cord was not contracting the fingers, nothing would be done, but the affected areas were kept under observation to monitor any marked development. If the progress contracted the fingers significantly, the hand would be incised, and the abnormal tissue cord removed. Wint said that this was a straightforward surgical procedure.
“The problem isn’t taking that cord out,” he explained. “That’s fairly easy to do. After an hour of surgery you can remove it. The problem is what happens to the hand afterwards. If it’s used to being bent, then the skin may be inefficient — it’s used to being pulled down. The tendons may not be springy right away, and the joints may want to reassume that bent position. So, someone who has a simple surgical removal to get rid of that cord needs to now go into a splint, sometimes full-time, up to a month, and into rehabilitation for three to six months after.”
Recurrence rates are a fact of life for patients with Dupuytren’s, and even with full removal of the contracting cord, the abnormal tissue can return in several years’ time. Also, if the original surgery involved the removal of a nodule, there’s a risk of not excising enough tissue, which Wint said could cause the abnormal tissue to return with increased vigor.
Handle with Care
While open surgery had been the only means for patients suffering this condition to return functionality to their fingers and hands, science has come to their rescue.
Two new procedures, as differentiated from surgery since they can be done in physicians’ offices, have come to the market within the last five years. One of these new techniques, a collagenase injection, was made available only one year ago.
That drug is called Xiaflex, and Wintman explained the process.
“You give a small shot of this enzyme in one area in the cord, and it eats through the collagen which the Dupuytren’s fascia is made from,” he said. “So it dissolves through the collagen, and then disrupts the cord at that one level. You come back the next day for manipulation, and the finger can go straight at that level after the manipulation.”
The second process is called needle aponeurotomy (NA), and essentially is a micro-perforation of the cord.
“We take a needle and actually make a series of puncture wounds along the length of the cord that’s keeping the finger bent down,” Martin said. “This breaks up the cord at various levels using the needle, almost like a tiny scalpel. The cord then actually breaks apart at these different levels. And then, the finger can straighten out. This doesn’t remove the tissue; you’re just breaking it up.
“As a result,” he continued, “afterward there are just a series of exterior puncture wounds that need to heal, typically over the course of five to seven days. Patients go home with some Band-Aids and an ice pack, and they’re moving and using their hand right away. Typically they don’t need a splint, although we might give them one to reinforce the gains we’ve made. But that is rare.”
Choosing between the new procedures comes down, ultimately, to dollars and sense: often an insurance carrier might cover one but not the other.
Also, the NA can involve several affected areas in the hand on one visit, whereas the Xiaflex must be administered only in one area every 30 days.
“So if someone comes in with three fingers affected, that’s a bit of a stretch,” Wintman said. “But some people want to try the medication over the four months.”
However, he added, “a patient might have co-morbidities. They might be on a blood thinner, and you wouldn’t want to give them an injection of an enzyme that eats tissue, because there would be too much swelling. Some people may have skin issues and not want to be exposed.”
But it can come down to a patient’s word-of-mouth referral to determine one over the other, Wint said.
Around the Bend
Sadly, science has not cured Dupuytren’s once and for all. The same recurrence rates for the abnormal tissues does still apply to these two new procedures, and, in fact, the time until reappearance of nodules and cords is often just a few years’ time. But, doctors agree, the ease with which both Xiaflex and NA can be administered should allay any fears of a return visit.
But has science sounded the death knell for the surgical open procedure?
“Well, it does reduce that frequency significantly,” Wint said. “Since that used to be the only solution, it was that or nothing. I had some people who, because of their work, really couldn’t take three to four months off. And you had to try to prepare people, also, because it could have even been longer.”
Martin said there are patients who still say, “I want it out,” and that does mean surgery. And for a patient with a history of severe Dupuytren’s, or situations involving prior complex surgeries, the new procedures might not offer any assistance.
“Their anatomy might be markedly distorted; they may have had skin grafts done elsewhere,” Wintman said. “Also, you can have contractures of the joints — the joint itself is scarred in. It’s not that you can feel a clearly defined cord anymore, but there might be scar tissue that keeps the fingers contracted. And, unfortunately, you really can’t do anything with that in the new procedures.”
But one thing the two new techniques will hopefully bring about is a future whereby doctors don’t see such severe cases of Dupuytren’s. “As more and more people read about these procedures, or hear about them from friends, they’ll realize that the need for the surgery will be less and less,” Martin said.
His colleagues nodded in agreement when Wintman said, “it’s incredibly satisfying for us, because when the patient leaves the office, they will have noticeable results. They walk in with a deficit, and they leave here with straight fingers.”