Total Percutaneous Revascularization of the Hand to Treat Refractory Digital Ischemia in Advanced Systemic Sclerosis.

We present the case of a young woman with systemic sclerosis (SSc) and refractory digital ulceration who was successfully treated with percutaneous revascularization of chronically occluded ulnar and radial arteries. To our knowledge, this is the first detailed report of limb salvage in SSc-induced hand ischemia in which contemporary endovascular techniques were used. (Level of Difficulty: Advanced.).

History of Presentation

A 39-year-old woman presented to the emergency room with painful ulceration of her right second and third digits. She had been hospitalized multiple times in the year leading up to presentation for similar presentations of intractable pain of these same digits. The patient was treated with analgesic agents for the ulcers and with apixaban and sildenafil for the digital ischemia, and she was admitted for epoprostenol administration.

Learning Objectives

To demonstrate the novel use of percutaneous intervention techniques to treat digital ischemia in the upper extremities.

To understand the role of revascularization in the treatment of SSc-associated chronic digital ischemia.

The patient’s initial physical examination was notable for gangrenous lesions involving the right second digit and third digits, with patchy areas of erythema and violaceous discoloration at the perimeter of the nailbeds (Figure 1). There was no evidence of calcinoses, telangiectasias, or skin thickening. Mobility was severely reduced in both digits, and there was reduced sensation to touch. The patient reported severe pain at rest and was referred for upper extremity angiography on the basis of her presentation and lack of response to standard digital ischemia therapies.

Figure 1

Patient With Dry Gangrene of the Second and Third Digits of the Right Hand

Past Medical History

The patient had a past medical history of autoimmune hepatitis, hypertension, limited cutaneous systemic sclerosis (SSc), and severe Raynaud phenomenon. She received a diagnosis of SSc 1 year before presentation on the basis of symptoms of Raynaud phenomenon, ischemic digital ulcers, abnormal nailfold capillaries, and a positive antinuclear antibody (ANA) 1:1,280 titer.

Differential Diagnosis

The differential diagnosis for our patient’s presentation included a range of connective tissue diseases with the potential to cause Raynaud phenomenon and digital ischemia, including systemic lupus erythematosus, Sjögren syndrome, and mixed connective tissue disease. Other causes of digital ischemia such as vasculitis (eg, cryoglobulinemic vasculitis) were also considered.

Investigations

The patient had a positive ANA 1:1,280 titer in a centromere pattern. Immunologic work-up on admission showed negative titers for anti–Scl-70, anti-RNA polymerase III, anti-PM/Scl, anti–smooth muscle, anti-RNP, and anti-SSA/SSB antibodies, and it showed a rheumatoid factor level of 15 IU/mL. Computed tomography angiography of the right upper extremity 3 months before presentation showed a long occlusion of the right radial artery, a short occlusion of the ulnar artery near the wrist, and partial reconstitution of the superficial palmar arch. Notably, she had not had previous cannulations of the right radial or ulnar arteries before her presentation.

Management

The patient underwent right upper extremity angiography through the right common femoral artery (Figures 2A and 2B). We obtained diagnostic images of the right upper extremity by using a 4-F multipurpose catheter positioned within the brachial artery. We exchanged this for a 6-F 90-cm long sheath for the intervention. We used heparin to maintain an activated clotting time of >300 seconds throughout the procedure.

Figure 2

Right Upper Extremity Angiography

At the level of (A) the forearm and (B) the wrist. There is occlusion of the radial artery in the middle forearm (arrow) and of the ulnar artery at the wrist (arrowheads). The hand is visibly underperfused.

Ulnar artery intervention

We used a Sion (Asahi Intecc) wire to deliver a 150-cm TurnPike LP (Teleflex) microcatheter to the proximal cap of the occluded ulnar artery. We crossed the proximal occlusion by using a Gaia-2 (Asahi Intecc) wire and created a channel by advancing the TurnPike LP catheter over the wire. We then performed sequential angioplasty using 2.0 and 3.0 × 30 mm balloons. We treated an “undilatable” segment of the ulnar artery with a 3.0-mm Chocolate (Medtronic) percutaneous transluminal angioplasty balloon. Postangioplasty angiography showed antegrade flow through the ulnar artery and opacification of the superficial palmar arch and the palmar digital branches (Figures 3A to 3C).

Figure 3

Revascularization of the Occluded Right Ulnar Artery

(A to C) Antegrade flow within the treated segment of the ulnar artery (arrow in C) and opacification of the common palmar digital branches (arrowhead in C) after angioplasty are noted.

Radial artery intervention

We redirected the TurnPike LP catheter into the right radial artery and used a Gaia-2 wire to cross the proximal two-thirds of the occlusion. We initially had difficulty in negotiating the wire across the distal occlusion; however, we were able to form a wire “knuckle” and advance it into the true lumen by means of subintimal tracking and re-entry (Figures 4A to 4C). We used the TurnPike LP catheter to exchange the knuckled wire for a Sion wire, and we treated the radial artery with a 2.5 × 150 mm NanoCross (Medtronic) balloon proximally and a 2.5 × 80 mm Chocolate balloon distally. Final angiography showed brisk antegrade flow through both the ulnar and radial arteries and opacification of both the superficial and deep palmar arches (Figure 5).

Figure 4

Revascularization of the Occluded Right Radial Artery

(A) The TurnPike LP catheter (Teleflex) and Gaia-2 wire (Asahi Intecc) were used to cross the radial artery occlusion proximally. A wire knuckle was used to cross (B) the distal occlusion into (C) the patent princeps pollicis artery.

Figure 5

Final Angiogram Demonstrating Restoration of Antegrade Flow to the Entire Hand

Comparison with Figure 2 shows a stark difference in opacification of the ulnar and radial arteries at the wrist, the palmar branches, and the digital arteries.

The patient had immediate improvement in hand skin color following intervention. She reported being able to “feel her fingers” again and could flex the second and third digits. She was treated with aspirin, clopidogrel, and epoprostenol. She had minimal residual pain in both digits at discharge. Her outpatient regimen included amlodipine, aspirin, sildenafil, and apixaban. The patient was monitored closely in the rheumatology clinic and had complete healing of her fingertips by 4 months (Figure 6).

Figure 6

Patient’s Right Hand at 4-Month Follow-Up Visit

Discussion

As many as 90% of patients with SSc experience vascular complications and symptoms of Raynaud phenomenon, with one-half developing arterial occlusions and subsequent digital ischemia. The etiology of digital ischemia involves a combination of prolonged and recurrent vasospasm of the medium and small arteries of the limb, in situ thrombosis, and chronic changes in vascular anatomy such as vessel wall hypertrophy. The net result consists of vessel occlusion and chronic hypoperfusion of the digits.

Refractory digital ischemia and ulceration associated with SSc are notoriously difficult to reverse. Most patients are treated with supportive care and medical therapy to prevent further tissue damage and avoid amputation. Some patients find symptomatic relief from surgical sympathectomy or microsurgical vein reconstruction, but the data are limited, and the clinical efficacy of these procedures in reversing ischemia is poor.2, 3, 4 Revascularization of the SSc-associated arterial lesions, surgical or otherwise, is seldom performed.

Many patients with SSc and digital ulcers have ulnar artery occlusion (UAO) and interruption of at least 1 of the palmar arches. Radial artery occlusion is less common. A study by Frerix et al reported a UAO prevalence of 21.5% in a cohort of 79 patients with SSc. Interestingly, the prevalence of UAO was higher in patients with localized cutaneous SSc (like our patient) compared with patients with cutaneous disease (24.4% vs 9.0%). Recurrent digital ulcerations occurred more frequently in patients with UAO compared with patients with SSc but without an occlusion.

Percutaneous revascularization is commonly performed for limb salvage in lower extremity atherosclerotic disease and has been shown to be safe in small cohorts of patients with other forms of critical limb ischemia (CLI)., Patients with lower extremity CLI of any cause often present with chronic occlusion of small-caliber (≤3-mm diameter) arteries, which can be successfully revascularized using contemporary endovascular techniques in most cases. We hypothesized that this approach could be used to treat refractory upper extremity digital ischemia in our patient. We decided to treat both arteries in the same setting to expedite wound healing by maximizing flow to the ischemic digits and hopefully minimizing the risk for further tissue loss or recurrence. Although a small case series alluded to the potential utility of angiography and angioplasty in patients with SSc, this case represents the first detailed account of successful arterial revascularization in SSc-associated critical hand ischemia. One potential limitation of the procedure is radiation exposure to the patient’s extremity and the operator. We minimized the patient’s risk by using standard radiation management strategies such as low frame rates (7.5/s), region of interest fluoroscopy, limited cine, and monitoring of the radiation dose to the patient’s hand. Furthermore, we used a ceiling-mounted radiation shield and a table-mounted lead skirt to minimize operator exposure. The total dose delivered to the patient was 315 mGy over 89 minutes, similar to, if not lower than, the dose expected after a complex peripheral intervention.

Follow-up

Our patient was able to flex the digits immediately after intervention and experienced complete healing of both fingers within 4 months. The likely mechanism of healing was reversal of ischemia, similar to treating lower extremity CLI in atherosclerotic disease. It is also possible that reperfusion of the hand improved the delivery of epoprostenol, thereby potentiating its effectiveness. Additional studies are needed to demonstrate the reproducibility of this technique and explore its role in the management of SSc-associated digital ischemia and ulceration.

Conclusions

Our initial experience suggests that reversal of chronic upper extremity digital ischemia is possible in at least some patients with SSc and may offer a potent new therapeutic option for patients who have not responded to other therapies.

Funding Support and Author Disclosures

Dr Arain has served as a consultant for Teleflex. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.