Question about Scleroderma-related fingertip ulcerations

[mtomaino]

This question was recently sent to me:

Hi Dr. Tomaino, Thank you for taking the time to read my email. I am contacting you because a am a Scleroderma patient at Robert Wood Johnson University Hospital in New Jersey. I am 40 years old and have been a patient since 2004 with connective tissue/scleroderma (thankfully I have no lung, heart or GI issues). What I do have is extreme Raynauds and digital ulcers for approximately 4+years. As I type now, 4 fingers are bandaged with uclers that have not healed for several months. My thumb, index and middle fingers of right and left hands are the ones impacted. I have tried medications including Cialis and am currently taking Adcirca (tadalafil). They provide some relief but not significantly. In the past year, the ulcers have had a big negative impact on my life and I am actively now looking for other options to help the pain, discomfort, limited ability to do normal things without pain (wash dishes, button buttons, throwing a ball to my son, putting earrings in my daughters ears, etc.). My Rheumatologist has brought to my attention last year a surgical procedure called a digital sympathectomy. I have seen a surgeon in NY City who has performs them and he agreed that I was a candidate for the procedure but I am looking for other opinion/consultation. They did an xray and found no other issues (no calstenosis). As you can imagine, for someone who is generally healthy and never had any other surgeries, I am nervous and want to make sure I am in the best hands possible. This is such a specialized microsurgery I am looking to explore and find out who can help me. I am doing the research on my own to specialists to help me. I called The Pittsburgh Scleroderma Center and they gave me your name and that is why I am contacting you today. I have some questions like the ones below… How many have you performed? For how many years? Do you make 1 incision on the palm or one for each finger? What to expect in the healing process? What have been the outcomes? Potential risks? Thank you in advance for your help, advice and time. Sincere thanks.

I am going to respond to the questions asked above, at the outset, and the make some general comments based on my experience. Interestingly, this past week I received a call from a rheumatologist who is currently working in Nebraska, but who worked with me when I was at the University of Pittsburgh Medical Center. She was inquiring about the optimal treatment for nonhealing digital ulcerations, and wanted me to comment on the role of microvascular reconstruction of the ulnar artery in the setting of ulnar artery thrombosis.
How many have you performed?
Over the last 20 years I have performed approximately 50 of these procedures and have published my early outcomes. My initial observations from when I worked at the University of Pittsburgh-at a time when I saw patient's from all over the country because of the expertise of Dr. Thomas Metzger-have not changed.

For how many years?
As mentioned above been doing the surgeries for 20 years.

Do you make 1 incision on the palm or one for each finger?
Based on the current literature, which acknowledges the role of sympathetic nerve fibers on the blood vessels entering the hand at both the wrist level and in the distal palm, I make 2 parallel incisions proximal to the wrist and perform a "adventitial stripping" of the radial and ulnar arteries. If preoperative examination and vascular studies indicate thrombosis of the ulnar artery, which is not uncommon, the thrombosed vessel is resected. In the distal palm a similar adventitial stripping is performed; this is what is referred to as a palmar sympathectomy. It is unusual to have to make incisions into the fingers, but infrequently there may be a role for reconstructing thrombosed vessels in the fingers to bring more blood flow. This is unusual.

What to expect in the healing process? The healing process is characterized by the potential for delayed wound healing and stiffness. However, when patients have not been smokers wounds generally heal without complication by 2-3 weeks, at which time sutures are removed.

What have been the outcomes?
The majority of patients that I have seen, who have undergone palmar sympathectomy have been able to come off of their pain medicines. Ulcerations that have not been severe at healed. However, these patients generally required the use of their vaso-dilator medicines--so the expectation that these can be discontinued after surgery is not what I've observed.

Potential risks?
The risks of this surgery include standard risks of any surgery such as infection and delayed wound healing, however, in this population of patients their increased, and in about 15-20% of patients the wound healing delays necessitate dressing changes and occasionally hyperbaric oxygen. In addition, if ulcerations or severe and do not heal, subsequent digital shortening and closure may be necessary. When ulcerations are very painful and there is exposed bone, my experience and the literature support earlier intervention with partial amputation, at the time of sympathectomy. [/COLOR]

Regarding the need for large vessel reconstruction, as might be suggested in the case of ulnar artery thrombosis--my approach relies on the findings of noninvasive vascular studies. Even though an angiogram may show a clot, suggesting that blood isn't getting to the fingers, if wave form analysis and digital brachial pressure indices suggests that some flow is getting to the fingers, then reconstructing the major vessel at the wrist level is typically not necessary because sympathectomy reverses arteriovenous shunts, and results in improved "nutritional" blood flow to the fingertip. Removing a clotted vessel in this situation without reconstruction is a valid approach and in the process contributes to the "sympathectomy".