Effects of Endoscopic Thoracic Sympathectomy on Raynaud's Disease

Abstract

Introduction:

Raynaud's disease is a disorder that is characterized by attacks of pain, cyanosis, redness, and numbness in the upper extremities caused by vasospasm of digital arteries due to cold or emotional stress. We aimed at demonstrating our experiences with endoscopic thoracic sympathectomy (ETS) in the treatment of Raynaud's disease.

Methods:

From 48 patients who underwent ETS for various reasons at our department between January 2014 and January 2015, we reviewed 9 patients with Raynaud's disease (18.7%) with respect to their demographic characteristics such as gender and age, postoperative complications, short-term results, side effects, recurrence of symptoms, and long-terms results.

Results:

The symptoms and findings reappeared and the number and dosage of the drugs used returned to their preoperative levels in 66.6% of the patients at month 6, and in all patients except 1 at the end of the 1st year.

Conclusion:

ETS should be considered an ultimate choice for patients with Raynaud's disease who have treatment-resistant severe symptoms and serious complications, disturbed social and daily lives, and impaired quality of life, and all patients should be properly informed before the surgery about the possibility of a high rate of recurrence.

Introduction

Having no underlying disease, Raynaud's disease is characterized by symmetrical involvement of the upper extremities by attacks of pain, cyanosis, redness, and numbness caused by vasospasm of digital arteries due to cold or emotional stress. Its etiology has not been fully explained. The prevalence of Raynaud's disease is 3%–4% with the predominance of women.^1,2 Raynaud's disease usually has a benign prognosis rarely leading to a digital gangrene or tissue loss, but there is no curative treatment method for it. The therapeutic goal is to achieve palliation through conservative methods such as protection from cold, reduction of emotional stress, and quitting smoking in addition to medical treatment.

The endoscopic thoracic sympathectomy (ETS) procedure is being used increasingly in recent years to eliminate the circulation disorder and pain in the upper extremities in Raynaud's disease cases where frequent and severe attacks, tissue damages such as nonhealing digital ulceration and necrosis, as well as dystrophic changes occur in the extremities despite correct and adequate medical treatment. ETS reduces the peripheral vascular resistance, hence increasing the blood flow in the peripheral vascular system.³ Our literature search showed that the short- and long-term success rates, side effects, and recurrence rates of ETS performed for Raynaud's disease cases differed from study to study, and a clearly defined consensus has not been established yet.^4–8 In this study, which we planned for the reason cited earlier, we wished to demonstrate concern with ETS in the treatment of Raynaud's disease, including short- and long-term results, side effects, and recurrence rates, on the basis of patient satisfaction and to discuss and present the results we obtained.

Materials and Methods

Population and study design

From 48 patients who underwent ETS for various reasons at our department between January 2014 and January 2015, we reviewed 9 patients diagnosed with Raynaud's disease (18.7%) at least for the past 3 years who had frequent episodic attacks, digital ulcers, or dystrophic disorders in their extremities despite adequate treatment with respect to their demographic characteristics such as gender and age, postoperative complications, short-term results, side effects, recurrence of symptoms, and long-terms results. The study began in 2014 on the approval of the local ethics committee. Information was given to all cases before ETS and informed consent forms were signed by patients. The work was carried out in accordance with the principles of the Helsinki Declaration revised in 2000.

Surgical procedure

Under double-lumen intubated general anesthesia, all patients were administered a single-session bilateral ETS operation in the supine semi-sitting position (semi-Fowler's position) with abduction of both arms by using a single port of a 1-cm incision in the third intercostal space from the lateral pectoral muscle on the anterior axillary margin. After introduction of the 30-degree videothoracoscope, the parietal pleura was opened and the sympathetic chain and accessory nerve fibers were transected over the third ribs and at the T3 ganglion level with electrocauterization. The efficacy of ETS is tested intraoperatively with the presence of dryness and increased heat in the hands immediately after the sympathectomy. While the lungs were expanded by the anesthesiologist in all patients, the air in the pleural space was evacuated and the incisions were closed without placing any thoracic drain. Postoperative chest radiography was performed routinely to exclude any pulmonary and pleural complications.

Statistical analysis

The data obtained were analyzed statistically by using the SPSS software. In descriptive analyses, the continuous variables were given as mean ± standard deviation and categorical variables were indicated as percentages and counts.

Results

The patients consisted of 6 female (66.6%) and 3 male patients (33.4%). The mean age of the patients was 33.7 years (range 21–42 years). All the patients had been diagnosed with Raynaud's disease 3 years ago or earlier. Despite this, the diagnoses of all patients were verified through diagnostic procedures such as anamnesis, physical examination, and cold water stress test. Four of the patients (44.4%) had been using medical therapy with nifedipine (60 mg/day)+acetylsalicylic acid (100 mg/day) and 5 of them (55.6%) with nifedipine (60 mg/day)+pentoxifylline (800 mg/day)+acetylsalicylic acid (100 mg/day) at least for the past 1.5 years, but none achieved a satisfactory result. The patients had five or more attacks a year, most of which occurred in winter months. Seven patients (77.8%) had chronic digital ulcers involving medical treatment-resistant necrotic tissues in their upper extremities. Severe ischemia-induced dystrophic changes such as atrophy, persistent redness, falling hair, and weakened nails were found in the cutaneous and subcutaneous tissues of the hands in two patients (22.2%). The demographics and clinical characteristics of the patients are shown in Table 1.

Table 1.

Demographics and Clinical Characteristics of the Patients

Patient no.	Gender	Age (years)	Disease duration (years)	Comorbidities	Smoking status	Episodic attacks (mean/years)	Digital ulcer	Ischemia induced dystrophic changes
1	Male	34	6	None	Former	6	Yes	None
2	Male	21	4	None	Never	9	Yes	None
3	Female	25	3	None	Former	5	None	Yes
4	Female	29	6	None	Never	5	Yes	None
5	Female	34	9	Bronchial asthma	Former	7	Yes	None
6	Male	38	11	None	Former	5	None	Yes
7	Female	42	7	None	Never	6	Yes	None
8	Female	41	4	None	Former	9	Yes	None
9	Female	40	3	None	Former	5	Yes	None

A bilateral ETS was applied to all patients at the T3 level in a single session. There was no preoperative mortality and no conversion to an open thoracotomy. No complications developed in any of the patients in the early postoperative period. The median length of the hospital stay was 1.5 ± 0.2 days (range 1–3 days).

Within the first postoperative month, a close-to-full recovery was seen in all patients with chronic digital ulcers. The entire 9 patients stated that there was a decrease in the frequency and severity of their vascular spasm attacks. The number and frequency of using drugs also decreased in all patients. When the patients were asked about their grade of satisfaction in the light of all these results, 8 patients (88.9%) stated that they extremely benefited from the operation and 1 patient (11.1%) stated that the operation met his expectations with a satisfactory result. The immediate effects of the operations as obtained by questioning the patients of their satisfaction are shown in Table 2.

Table 2.

Immediate and Late Effects of Endoscopic Thoracic Sympathectomy on Patients with Raynaud's Disease

Effects of the operation	Patient immediate effects(postoperative 1st month)	Patient late effects(postoperative 1st year)
Excellent	8 (88.9%)	1 (11.1%)
Satisfactory	1 (11.1%)	0
Some effect	0	2 (22.2%)
No effect	0	6 (66.6%)

In the postoperative 3rd month, 1 of the patients (11.1%) had compensatory sweating (CS) localized in the back area with moderate level of irritation.

The median follow-up time was 14 months (range 12–17 months). All patients were reassessed at postoperative 6th month. No problems were found in 3 patients (33.4%), whereas there was an increase in the frequency of attacks with recurrent symptoms in the other 6 patients (66.6%) and the number and dosage of the drugs they used reached their preoperative levels. Digital ulcers reappeared in 4 patients (44.4%). Also, 4 patients (44.4%) stated that the recurrent symptoms had the same severity as those that had occurred before the sympathectomy and 2 (22.2%) stated that the severity of the recurrent symptoms was less compared with those before the sympathectomy.

All 9 patients were invited to our hospital at the end of the 1st year to be questioned and physically examined. With the exception of 1 female patient (11.1%), attack frequencies and symptoms relapsed and the number and dosage of the drugs used reached their preoperative levels in the remaining 8 patients (88.9%). Six (66.6%) of these 8 patients stated that they had been operated unnecessarily and they would not have had the operation if they knew about the outcome. Two of them (22.2%) stated that they had benefited from the operation, although partially, and did not regret having been operated. Among all the patients, only the earlier mentioned 1 female patient (11.1%) stated that the excellent benefit she had in the postoperative early period continued to prevail just as well at the end of the 1st year. The satisfaction levels of the patients with respect to their operations at the end of the 1st year are shown in Table 2.

Discussion

This study underlines five points: (1) The Raynaud's disease cases in this series were found frequent in women and in the second and third decades; (2) During the study period, 18.7% of the 48 patients who were administered ETS were operated due to Raynaud's disease; (3) In the ETSs applied at the T3 level, only 1 patient had CS with moderate degree of irritation; the other patients did not have any side effects; (4) The symptoms and findings reappeared and the number and dosage of the drugs used returned to their preoperative levels in 66.6% of the patients at month 6 and in all patients except 1 at the end of the 1st year; (5) At the end of the postoperative 1st year, 66.6% of the patients expressed their dissatisfaction, saying that they were operated unnecessarily and they would not have had the operation if they knew about the outcome.

In the course of this study, 9 patients (18.7%) were operated due to Raynaud's disease out of the 48 patients who were administered ETS. This seems to be a high rate when compared with classical ETS series.³ We think this is because many patients with peripheral vascular diseases in our region are being monitored by the vascular diseases outpatient clinic that was founded and is being managed by the Cardiovascular Surgery clinic in the health center where we work and any complications that patients develop are detected at an early stage, on which they are referred to surgery.

The most commonly seen sweat-related complications that occur after an ETS are gustatory sweating and CS. Gustatory sweating is a sweating complaint that occurs when ingesting hot or sour food, and its cause is unknown. Gustatory sweating was observed in 1%–30% of patients.^9,10 It might be caused by an aberrant anastomosis between the sympathetic trunk and the vagal nerve.¹¹ CS is a condition in which excessive sweating occurs in the back, waist, groins, and legs of the body to irritating extents. According to the literature, CS is the most common and undesirable long-term complication of ETS, which occurs at a rate between 3% and 98%, and is reported to be the “quality marker” of an ETS.^12,13 The physiopathology of CS occurrence is not fully known. There is no adequate and appropriate treatment for CS, which is a factor that directly affects patient satisfaction, and it becomes more important at this point to prevent the occurrence of CS rather than trying to deal with it.

As the level of sympathectomy goes up in the ETS surgery, the effectiveness of treatment increases, but there is also an increase in the risk of CS. To prevent this irritating complication, wide resections have been avoided in years and sympathectomy has been administered from the lower ganglion levels.¹⁴ In this way, the rates of CS have been reduced. We have been trying to perform the ETS procedure only at the T3 level in our clinic in recent years. With this practice, we realized a serious decrease in the number and severity of CS, the complication that affects patient satisfaction the most. Also in this study, all patients were administered ETS at the T3 level and only 1 patient had CS, which produced a moderate degree of irritation but did not affect the patient's quality of life negatively. Generally, patients have warm and dry hands, a decline in the severity of pain, and improvement in skin circulation immediately after the surgery. We think that all of these positive outcomes can be obtained with much less CS complication in all patients who undergo ETS at the T3 level, and attempting sympathectomy with more dissections and at different ganglion segments is unnecessary.

We see a decrease in effect over time with recurrence of symptoms in almost all post–ETS Raynaud's disease patients. Recurrence of symptoms typically begins at month 6 after the sympathectomy.^15,16 This may be linked to incomplete surgical procedures, but it is more common here that those symptoms relapse very early, sometimes with no improvement at all and patients usually complain that they have not benefited from the operation. The relapsing symptoms at month 6 and thereafter could be associated with nerve regeneration over the years, but no results could be found to support this hypothesis.¹⁷ However, there are also papers reporting that the factors precipitating recurrence of symptoms may include hypersensitivity of the noradrenergic receptors regulating the pre-capillary sphincters reacting on very small amounts of circulating catecholamines. The same authors also reported that the reason for the recurrence seen after ETS particularly in patients who have also a collagen disease could be progressive vasculitis.⁷ In our study, the symptoms and findings relapsed and the number and doses of the drugs used returned to their preoperative levels in 66.6% of the patients at month 6 and in all patients except 1 at the end of postoperative year 1. The reason for this cannot possibly be insufficient surgical procedure because improvement in symptoms and recovery in lesions occurred in all patients in the early postoperative period. Moreover, after transecting the sympathetic chain with electrocautery at the T3 level, we additionally electrolyze the proximal and distal endings of the nerve and a 6–7 cm area of the posterior surface of the rib at that level. The possibility of this procedure to prevent the sympathetic chain and its branches from regenerating and restoring the connection between proximal and distal is high. At this point, we believe that there is a need to conduct studies at molecular level to explore the effects of immunological mediators, neurotransmitters, enzymes, or hormones on the sympathetic nerve system and capillary circulation after ETS, so that the mechanism of nerve regeneration that causes symptom recurrence can be understood.

The sympathetic nerve regeneration and resulting symptom recurrences led to so much dissatisfaction that 66.6% of the patients in this study thought that they were operated unnecessarily. However, very little positive effect remained in 2 patients (22.2%) at the end of year 1. It is not normal for a surgical procedure to produce so much dissatisfaction at the end of the 1st year and makes it debatable whether to perform such procedures at all. This holds true also for ETSs carried out in patients with Raynaud's disease, and there is failure resulting from recurrence in nearly all patients at the end of year 1 as evidenced in many studies in the literature.^4,17,18 Therefore, we think that performing an ETS procedure is not appropriate except for patients with severe Raynaud's disease accompanied by serious ischemic complications. Even so, this situation of recurrence should be explained to such patients in detail before the operation and a surgery should be decided for those who agree after making sure that they have adequate information on the issue. In this way, it will be possible to avoid any medicolegal problems in the future.

This study has clear limitations. Limited number of cases stands at the forefront of these constraints. In addition, this study was conducted at a single center. We believe that the results obtained from this study can gain more meaning by further multicenter studies including a greater number of patients.

Conclusion

Careful patient selection is required in Raynaud's disease; ETS should be considered an ultimate choice only for those who have treatment-resistant severe symptoms and serious complications, disturbed social and daily lives, and impaired quality of life; and all patients should be properly informed before the surgery about the possibility of a high rate of recurrence.

Footnotes

Disclosure Statement

No competing financial interests exist.

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