Type I
Complete occlusion of radial or ulnar artery; decreased flow and narrowing in level 2 and 3 arteries
Type II
As I, but with stenosis of radial or ulnar artery
Type IIIa
The main disorder is in the common digital or digital arteries
Type IIIb
A rare subset characterized by selective occlusion of the digital arteries to the index finger, secondary to vibrating machinery use
Type IV
All levels of vessel are stenotic
Type V
Global ischemia, paucity of vessels, scant flow on angiography
Kim and his group use the classification to influence their choice of surgical treatment.
It is notable that the ulnar artery is disproportionately affected in patients with secondary RP; 53.5 % of the patients reported by Kim et al. had disease primarily at the level of the radial or ulnar arteries, and in these, 88.5 % of occlusions and 95.7 % of stenoses affected primarily the ulnar artery. 27.1 % of patients had disease primarily in the palmar arch and common digital vessels. These findings are consistent with those of Janevski [9], who (as discussed above) found occlusion of the ulnar artery and superficial palmar arch on 10 of 24 arteriograms performed in 12 patients with systemic sclerosis and of Park et al. [10] who found occlusion or stenosis of the ulnar artery in 63 % of 19 patients studied with arteriography. Higgins and McClinton describe that by the time patients with collagen vascular disease have ulcers or digit-threatening ischemia, two-thirds have ulnar artery thrombosis at the wrist [3].
Evaluation and Anesthesia
In most instances the patient will be referred to the surgeon by a rheumatologist. The probability is that an extensive work-up will already have been undertaken. If not a careful history and examination will naturally be required. Often the presenting complaint will be of pain and or ulceration. Laboratory investigations will include full blood count and biochemistry. If unilateral disease is present a single source of ischemia, such as hypothenar hammer syndrome, thoracic outlet syndrome, or thromboembolic disease, should be suspected. The vascular supply of the hand may most accurately be assessed by arteriography [6]. Other, less invasive, methods include Doppler evaluation (to assess the presence of pulsatile blood flow in the radial and ulnar arteries), laser Doppler fluxmetry, laser Doppler perfusion imaging, digital:brachial index measurement (the ratio of the pressure in the finger compared to the pressure in the brachial artery), digital plethysmography, measurement of cutaneous surface temperature, isolated cold stress testing, nailfold capillaroscopy, color duplex imaging, and magnetic resonance angiography (see Chap. 14). In practical terms in the hand surgical clinic Doppler ultrasound is convenient and readily performed with a handheld machine. We use angiography if we are contemplating reconstruction, but otherwise start with simple surgical measures and do not routinely arrange angiography for every patient.
Various hand function assessment tools are available to assist in evaluating the results of treatment. These include the Hand Mobility in Scleroderma (HAMIS) test [11], the Scleroderma Health Assessment Questionnaire (SHAQ) [12], the Scleroderma Functional Index (SFI) [13], and the Cochin Hand Function Scale (CHFS) [14]. None of the standard hand assessment tools such as DASH have been validated in Raynaud’s.
Plain radiology may demonstrate acrolysis. It may be very difficult to differentiate between this and osteomyelitis. MRI scanning may be more sensitive in assessing whether there is infection in the terminal phalanx or in other parts of the hand or foot.
Patients with systemic sclerosis or other causes of RP may have significant cardiorespiratory dysfunction and may thus present an increased anesthetic risk. If major surgery is to be undertaken preoperative anesthetic consultation is advisable. Surgery may however readily be performed under regional anesthesia. We have not experienced any problems with performing surgery under metacarpal block anesthesia and in a willing patient this form of anesthesia is ideal for surgery on a single digit. Whilst the use of epinephrine in metacarpal block anesthesia is generally accepted in healthy patients, we do not use it in patients with systemic sclerosis or other forms of RP because of the risk of provoking vasospasm and tissue ischemia.
Ulcer Débridement
Digital ulcers (Figs. 22.1 and 22.2) in patients with RP are often very painful. The simple expedient of debriding the ulcer may be surprisingly effective at relieving pain, especially if, as often happens, there is a bead of pus under an overlying scab.
Fig. 22.1
(a) and (b) Multiple skin ulcers
Fig. 22.2
Painful fingertip ulcer; note the surrounding mild erythema
Technique
Surgery may be performed with the use of local, regional, or general anesthesia. No tourniquet is required. The ulcer is debrided, and the base gently curetted with a No. 15 blade. The surgeon should not expect to see normal bleeding; the finger tip will almost inevitably be relatively avascular. The normal surgical dictum of resecting until healthy tissue is encountered should be resisted, as this will often not be found until a large part of the finger has been amputated. Whilst this may be good for one finger, the patient may be back with other ulcers and poorly thought through amputations may result in the loss of multiple fingers. The patient should be warned that the ulcer will take some time to heal. Mepitel™ (Mölnlycke Health Care) is an excellent choice of dressing as it causes less pain on removal than do some others. Dry scabs may be left exposed if there is no sign of infection.
Sympathectomy
Cervical Sympathectomy
Cervical sympathectomy results in improvement in perfusion in the short term. However, return of sympathetic tone may occur within 6–12 weeks and indeed may be increased. Claes et al. [15] noted return of symptoms after 6 months in all nine operated patients with Raynaud’s phenomenon in his study. Thus, cervical sympathectomy is no longer considered appropriate treatment for the condition.
Periarterial (Digital, Radial, and Ulnar) Sympathectomy
The sympathetic supply to the hand arises not only from the sympathetic chain but also from alternative pathways. Digital sympathectomy was first described by Flatt, who postulated that interrupting the sympathetic nervous supply more distally would be more likely to address all constituents of the supply and thus produce more long-lasting effects than simple division of the cervicothoracic sympathetic trunk [16]. Morgan and Wilgis [17] demonstrated in a rabbit ear model that sympathetic fibers in the adventitia have limited capacity for regeneration even after 1 year.
Balogh et al. [18] emphasized the importance of identifying the nerve of Henlé if possible. The nerve of Henlé sends a consistent branch carrying sympathetic fibers to the ulnar artery [19].
Flatt’s original series of periarterial sympathectomy was of eight patients. The underlying pathology was varied, and Flatt himself describes the difficulty in assessing objectively the results of treatment. Hartzell et al. [20] studied 28 patients with an average follow-up of 96 months (minimum 23 months). Their patients had a mixture of autoimmune and arteriosclerotic disease. Their technique was tailored to each patient’s pattern of disease and involved surgery at any of the levels described previously. Eleven of the 20 patients with autoimmune disease saw complete healing of ulcers, with 15 experiencing some improvement. However, seven eventually underwent amputation (these are included in the unhealed ulcer group).
Kim et al. [8] undertook a combination of digital sympathectomy of the common digital vessels alone and radical sympathectomy including the proper digital vessels for their patients with type IV and V disease. 66.7 % of patients with type IV and 53.3 % of patients with type V disease saw an improvement in symptoms but 22.9 and 33.3 % had no change and 10.4 and 13.3 % experienced a deterioration.
Koman et al. [5] studied the microvascular physiology in seven hands with refractory pain and ulceration undergoing digital sympathectomy. Following surgery all seven hands had diminished pain. The ulcers healed in six hands and improved in the remaining one. The results were assessed further by isolated cold stress testing, digital pulp temperature evaluation, and laser Doppler flowmetry. After sympathectomy, fingertip temperature did not increase (contrary to some other writers’ experiences), suggesting that total blood flow was unaffected. The investigators did, however, note that microvascular perfusion and vasomotion increased. Vasomotion describes the natural rhythmic oscillations in vascular tone caused by local changes in smooth muscle constriction and dilatation. The significance of vasomotion is not certain, but one hypothesis suggests that it may increase tissue oxygenation when blood flow is compromised [21]. On this basis, they hypothesized that the mechanism for the clinical effectiveness of sympathectomy is a preferential increase in flow in nutritional rather than thermoregulatory vessels.
Kotsis and Chung [22] performed a systematic review of the literature on peripheral sympathectomy. 16 papers met their inclusion criteria. Most, but not all, of the patients studied had SSc. Ulcer healing time took from 2 weeks to 7 months. 15 % eventually required amputation despite the sympathectomy, 16 % had recurrence/incomplete healing, and 37 % had a postoperative complication. The authors therefore counsel that long-term prospective studies are still required in this field, and that patients should be warned of the uncertain success rate.
Technique
Peripheral sympathectomy may be performed at the level of the ulnar and radial arteries, the common digital arteries, and the proper digital arteries. The surgery is performed with the use of general or regional anesthesia and an exsanguinating tourniquet. The use of loupe magnification or of an operating microscope is advisable. For digital sympathectomy the common digital artery is approached through a Bruner incision. A 2-cm stretch of the artery is stripped of adventitia. We do not normally drain the wound; the transverse limb may safely be left open. For radial and ulnar artery sympathectomy, the arteries are approached via a straight 3-cm incision. The adventitia is stripped over a 2-cm stretch of artery.
O’Brien et al. [23] describe an extensive sympathectomy of the ulnar artery, superficial palmar arch, and proper digital arteries.
A radical exposure is also described by Koman et al. [38], in which the superficial palmar arch and the three volar common digital vessels are exposed. In addition Koman et al. strip a section of the deep branch of the radial artery and the origin of the deep palmar arch through a fourth incision in the anatomic snuffbox.
Figure 22.3a shows a painful ischemic ring finger in a 53-year-old patient with systemic sclerosis. Sympathectomy was planned (Fig. 22.3b–d).
Fig. 22.3
(a—Part 1 and 2) Ischemic fingertip; (b) incisions for sympathectomy. We plan these to overlie the common digital artery; (c) intraoperative photograph. The wrist is on the left, the fingers on the right. The common digital artery has been exposed. With skin retraction we shall be able to strip the whole 2 cm of artery. The artery (CDA) with the venae comitantes is at the bottom of the picture, the bifurcating common digital nerve (CDN) at the top. Remember that at this level the nerve is volar to the artery. Also it is worth noting that the bifurcation of the artery is well distal to the bifurcation of the nerve; (d) the adventitia is being stripped off the artery
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