Management of Paget-Schroetter Syndrome: a Systematic Review and Meta-Analysis.

OBJECTIVE: Currently, there is no consensus on the optimal management of Paget-Schroetter syndrome (PSS). The objective was to summarise the current evidence for management of PSS with explicit attention to the clinical outcomes of different management strategies. DATA SOURCES: The Cochrane, PubMed, and Embase databases were searched for reports published between January 1990 and December 2021. REVIEW METHODS: A systematic review and meta-analysis was conducted following PRISMA 2020 guidelines. The primary endpoint was the proportion of symptom free patients at last follow up. Secondary outcomes were success of initial treatment, recurrence of thrombosis or persistent occlusion, and patency at last follow up. Meta-analyses of the primary endpoint were performed for non-comparative and comparative reports. The quality of evidence was assessed using the GRADE approach. RESULTS: Sixty reports were included (2 653 patients), with overall moderate quality. The proportions of symptom free patients in non-comparative analysis were: anticoagulation (AC), 0.54; catheter directed thrombolysis (CDT) + AC, 0.71; AC + first rib resection (FRR), 0.80; and CDT + FRR, 0.96. Pooled analysis of comparative reports confirmed the superiority of CDT + FRR compared with AC (OR 13.89, 95% CI 1.08 - 179.04; p = .040, I2 87%, very low certainty of evidence), AC + FRR (OR 2.29, 95% CI 1.21 - 4.35; p = .010, I2 0%, very low certainty of evidence), and CDT + AC (OR 8.44, 95% CI 1.12 - 59.53; p = .030, I2 63%, very low certainty of evidence). Secondary endpoints were in favour of CDT + FRR. CONCLUSION: Non-operative management of PSS with AC alone results in persistent symptoms in 46% of patients, while 96% of patients managed with CDT + FFR were symptom free at end of follow up. Superiority of CDT + FRR compared with AC, CDT + AC, and AC + FRR was confirmed by meta-analysis. The overall quality of included reports was moderate, and the level of certainty was very low.

Long-Term Outcomes of Nonoperative and Surgical Management of Paget-Schroetter Syndrome.

PURPOSE: In Paget-Schroetter Syndrome (PSS), subclavian vein thrombosis is caused by external compression of the subclavian vein at the costoclavicular junction. Paget-Schroetter Syndrome can be treated nonoperatively, surgically, or with a combination of treatments. Nonoperative management consists, in most cases, of anticoagulation (AC) or catheter-directed thrombolysis (CDT). With surgical management, decompression of the subclavian vein is performed by resection of the first rib. No prospective randomized trials are available to determine whether nonoperative or surgical management is superior. We report our long-term outcomes of both nonoperative and surgically treated patients. MATERIALS AND METHODS: We retrospectively analyzed all patients with PSS who were treated between January 1990 and December 2015. Patients were divided based on primary nonoperative or primary surgical therapy. Long-term outcomes regarding functional outcomes were assessed by questionnaires using the "Disability of the Arm, Shoulder, and Hand" (DASH) questionnaire, a modified Villalta score, and a disease-specific question regarding lifestyle changes. RESULTS: In total, 91 patients (95 limbs) were included. Seventy patients (73 limbs) were treated nonoperatively and 21 patients (22 limbs) surgically. Questionnaires were returned by 67 patients (70 limbs). The mean follow-up was 184 months (range, 43-459 months). All functional outcomes were better in the surgical group compared with the nonoperatively treated group (DASH general 3.11 vs 9.86; DASH work 0.35 vs 11.47; DASH sport 5.85 vs 17.98, and modified Villalta score 1.11 vs 3.20 points). Surgically treated patients were more likely to be able to continue their original lifestyle and sports activities (84% vs 40%, p=0.005). Patients with recurrence of thrombosis or the need for surgical intervention after primary nonoperative management reported worse functional outcomes. CONCLUSION: Surgical management of PSS with immediate CDT followed by first rib resection leads to excellent functional outcomes with low risk of complications. The results of nonoperative management in our non-matched retrospective comparative series were satisfactory, but resulted in worse functional outcomes and more patients needing to adjust their lifestyle compared with surgically treated patients. CLINICAL IMPACT: Patients with Paget-Schroetter Syndrome and their attending physicians are burdened by the lack of evidence concerning the optimal treatment of this entity. Case series comparing the outcomes of non-operative treatment with surgical treatment are scarce and often not focussed on functional outcomes. Data from this series can aid in the shared decision making after diagnosis of Paget-Schroetter Syndrome. Functional outcomes of non-operative management can be satisfying although high demand patient who are not willing to alter their daily activities are probably better off with surgical management.

Robot-assisted transthoracic first rib resection for venous thoracic outlet syndrome.

BACKGROUND: Venous thoracic outlet syndrome (vTOS) is caused by external compression of the subclavian vein at the costoclavicular junction. It can be subdivided in McCleery Syndrome and Paget-Schroetter Syndrome (PSS). To improve the venous outflow of the arm and to prevent recurrent thrombosis, first rib resection with venolysis of the subclavian vein can be performed. Open transaxillary, supraclavicular, infraclavicular or combined paraclavicular approaches are well known, but more recent robot-assisted techniques are introduced. We report our short- and long-term results of a minimal invasive transthoracic approach for resection of the anteromedial part of the first rib using the DaVinci surgical robot, performed through three trocars. METHODS: We analyzed all patients with vTOS who were scheduled to undergo robot-assisted transthoracic first rib resection in the period July 2012 to May 2016. Outcomes were: technical success, operation time, blood loss, hospital stay, 30-day complications and patency. Functional outcomes were assessed using the "Disability of the Arm, Shoulder and Hand" (DASH) questionnaire. RESULTS: Fifteen patients (8 male, 7 female; mean age 32.9 years, range 20-54 years) underwent robot-assisted transthoracic first rib resection. Conversion to transaxillary resection was necessary in three patients. Average operation time was 147.9 min (range 88-320 min) with a mean blood loss of 79.5 cc (range 10-550 cc). Mean hospital stay was 3.5 days (range 2-9). In three patients, complications were reported (Clavien-Dindo grade 2-3a). Patency was 91% at 15.5 months' follow-up. DASH scores at one and three years showed excellent functional outcomes (7.1 (SD= 6.9, range 0-20.8) and 6.0 (SD= 6.4, range 0-25)) and are comparable to the scores of the normative general population. CONCLUSION: Robot-assisted transthoracic first rib resection with only three trocars is a feasible minimal invasive approach for first rib resection in the management of vTOS. This technique enables the surgeon to perform venolysis under direct 3D vision with good patency and long-term functional outcome. Studies with larger cohort size are needed to compare the outcomes of this robot-assisted technique with other more established approaches.

The Importance of Early Diagnosis and Treatment of Patients with Aortoenteric Fistulas Presenting with Herald Bleeds.

BACKGROUND: Massive bleeding in patients with aortoenteric fistula (AEF) may be preceded by minor, intermittent gastrointestinal (GI) blood loss, termed the "herald bleed." The aims of this retrospective study were to: (i) analyze the interval between the herald bleed and onset of major GI hemorrhage and/or diagnosis of AEF and (ii) to evaluate the diagnostic roles of endoscopy and computed tomography imaging. METHODS: Analysis of all patients diagnosed with AEF or iliac-enteric fistulas between 1994 and 2013 in a single institution. RESULTS: In 31 of a total of 34 fistula cases, GI bleeding was the presenting symptom. Of these, 17 of 31 presented with herald bleed while 14 of 31 presented with massive GI bleeding. In patients with a herald bleed, median time from first bleeding to diagnosis was 14 (2-137) days. In 5/17 patients, herald bleeding preceded major hemorrhage with a median of 6 (4-92) days before a diagnosis of AEF was made or intervention could be initiated. CT angiography (CTA) showed abnormalities associated with a fistula in 27 (79%) cases, of which in 12 (35%) cases a fistula was actually identified. Esophagogastroduodenoscopy (EGD) demonstrated a fistula in 8 (25%) patients, while 50% of EGDs were completely normal. CONCLUSIONS: Any patient with history of aortic surgery and GI bleeding should be considered to have an AEF until proven otherwise. The sensitivity of CTA for detecting AEF is substantially greater than that of EGD. The time interval between herald bleed and subsequent massive hemorrhage associated with AEF is unpredictable but may be as short as 4 days.

Thoracic sympathectomy for upper extremity ischemia.

INTRODUCTION: Thoracic sympathectomy is performed in the management of a variety of disorders of the upper extremity. To evaluate the contemporary results of thoracic sympathectomy for upper extremity ischemia a systematic review of the literature was conducted. EVIDENCE AQUISITION: We performed a PubMed, EMBASE and Cochrane search of the literature written in the English language from January 1975 to December 2015. All articles presenting original patient data regarding the effect of treatment on symptoms or on the healing of ulcers were eligible for inclusion. Individual analyses for Primary Raynaud's Disease (PRD) and Secondary Raynaud's Phenomenon (SRP) were performed. EVIDENCE SYNTHESIS: We included 6 prospective and 23 retrospective series with a total of 753 patients and 1026 affected limbs. Early beneficial effects of thoracic sympathectomy were noticed in 63-100% (median 94%) of all patients, in 73-100% (median 98%) of PRD patients and in 63-100% (median 94%) of SRP patients. The beneficial effect was noted to lessen over time. Long-term beneficial effects were reported in 13-100% (median 75%) of all patients, in 22-100% (median 58%) of PRD patients, and in 13-100% (median 79%) of SRD patients. Complete or improved ulcer healing was achieved in 33-100% and 25-67% respectively, of all patients. CONCLUSIONS: Thoracic sympathectomy can be beneficial in the treatment of upper extremity ischemia in select patients. Although the effect in patients with PRD will lessen over time, it may still reduce the severity of symptoms. In SRD, effects are more often long-lasting. In addition, thoracic sympathectomy may maximize tissue preservation or prevent amputation in cases of digital ulceration.

Endoscopic thoracic sympathectomy for upper limb ischemia. A 16 year follow-up in a single center.

INTRODUCTION: The aim of our study was to evaluate the long term results of Endoscopic Thoracic Sympathectomy (ETS) in the management of upper limb ischemia (ULI). METHODS: We retrospectively reviewed the records of all consecutive patients who underwent ETS for ULI between January 1994 and May 2009. A standardized questionnaire was used to evaluate the long term success, morbidity and overall patient satisfaction. RESULTS: Thirty-five patients (20 female, mean age 49 years (range 23-79)) underwent bilateral (n = 9) and unilateral (n = 27) ETS procedures, respectively. Six patients had Primary (idiopathic) Raynaud Disease. Twenty-nine patients had upper limb ischemia secondary to systemic disorders (n = 12), embolic disease (n = 10), occlusion of the arteries of the arm (n = 5) or hypothenar hammer syndrome (n = 2). Tissue loss at time of surgery was present in nineteen patients. Short term beneficial effects were reported by 12 patients (63%). Eleven of the 35 patients experienced a total of 13 complications or adverse events, whereof 11 were minor or transient. Limb salvage was unsuccessful in three patients because of major amputations (n = 2) or severe functional impairment (n = 1). Necrotectomies or minor amputations without functional impairment were performed in 9 patients. Medium or long term follow up (mean 98 months (range 18-198) was available in 19 out of 22 living patients(86%). Long term beneficial effects were reported by 10 (53%). Overall patient satisfaction was 56%. Compensatory sweating was experienced by 11 patients (58%). CONCLUSION: Although the long term efficacy of ETS in our study was moderate (53%), due to its low invasiveness ETS is a valuable option in the management of ULI.

Selective sympathectomy for hyperhidrosis: technique of robotic transthoracic selective postganglionic efferent sympathectomy.

Selective postganglionic efferent sympathectomy for hyperhidrosis is associated with excellent relief of hyperhidrosis and a low rate of compensatory hyperhidrosis. However, this technique can be difficult using conventional videoendoscopic techniques. We performed this technique using robotic technology in 55 patients. There was complete relief of hyperhidrosis in 53/55 (96%) patients, partial relief in 2/55 (4%) patients and a compensatory hyperhidrosis rate of 7%.

Robotic selective postganglionic thoracic sympathectomy for the treatment of hyperhidrosis.

BACKGROUND: The surgical management of hyperhidrosis is controversial. Robotic surgical systems with their high-definition magnified 3-dimensional view and increased maneuverability in a confined space may facilitate the technique of selective sympathectomy (ramicotomy). We present a case series of patients undergoing selective postganglionic thoracic sympathectomy using robotic technology. METHODS: This study is a case series analysis of patients who underwent selective postganglionic thoracic sympathectomy from July 2006 to November 2011. The operation was performed on a video-assisted thoracoscopic surgery (VATS) platform. The robot was used for pleural dissection and division of the postganglionic sympathetic fibers and the communicating rami. The success of sympathectomy was assessed by intraoperative temperature measurement of the ipsilateral upper extremity, patient interviews, and scoring of the symptomatic nature of hyperhidrosis based on the Hyperhidrosis Disease Severity Scale. RESULTS: There were 110 sympathectomies performed in 55 patients (25 men, 30 women). Simultaneous bilateral sympathectomy was performed in all patients. Median age was 28 years (range, 16 to 65 years). There was no conversion to thoracotomy. Complications were minor and were seen in 5 of 55 patients (9%). There were no deaths. Median hospital stay was 1 day (range, 1 to 4 days). Of the 55 patients, 53 (96%) had sustained relief of their hyperhidrosis at a median follow-up of 24 months (range, 3 to 36 months), and compensatory sweating was seen in 4 patients (7.2%). CONCLUSIONS: Robotic thoracoscopic selective sympathectomy is an effective, feasible, and safe procedure with excellent relief of hyperhidrosis and low rates of compensatory sweating and complications.

Thoracic sympathectomy for digital ischemia: a summary of evidence.

BACKGROUND: Thoracic sympathectomy is used in the management of a variety of upper limb disorders. We have analyzed the evidence for thoracic sympathectomy in the management of digital ischemia. METHODS: We reviewed the English literature between 1980 and 2010. Our analysis included reports with the clinical end points of relief, recurrence of symptoms or healing of ulcers, or both. Primary Raynaud disease (PRD) and secondary Raynaud phenomenon (SRP) were analyzed separately. RESULTS: An initial postoperative positive effect was reported in 92% of PRD patients and in 89% of SRP patients. Long-term beneficial effect was 58% for PRD and 89% for SRP. Ulcer healing or improvement was achieved in 95%. CONCLUSIONS: The available evidence suggests that thoracic sympathectomy has a role in the treatment of severe PRD and SRP, albeit with better results in SRP patients than in PRD patients. In case of digital ulceration, thoracic sympathectomy may maximize tissue preservation or prevent amputation.

Direct videoscopic approach to the thoracic aorta for aortic endograft delivery: evaluation in a human cadaver circulation model.

PURPOSE: To examine the feasibility of a direct videoscopic approach to the descending thoracic aorta for branched endograft delivery to the aortic arch and abdominal aorta. METHODS: Aneurysms were created in the aortic arch and pararenal aorta of 3 human cadavers, and pulsatile flow was established using a roller pump. Thoracoscopically, 2 double-felted purse-string sutures were placed on the thoracic aorta. Via the most distal trocar, an endoscopic needle was used to insert a stiff guidewire into the aorta through the center of the purse-string suture. Under direct videoscopic control, a 20-F sheath was advanced over the wire into the aorta. Switching to fluoroscopic control, a fenestrated endograft was deployed in the aortic arch followed by placement of a branch graft into the left subclavian artery. The delivery sheath was withdrawn from the aorta while simultaneously tightening the purse-string sutures. A similar procedure was performed in the same cadaver for antegrade branched endograft delivery to the pararenal aorta. Correct deployment of the branched endografts was evaluated by post implant angiography and autopsy. RESULTS: The procedure was successfully completed in all cadavers. "Hemostasis" was obtained in all cadavers without aortic cross clamping. Median fluid loss was 165 mL. Autopsy proved all purse-string sutures to be adequately placed and all branched endografts to be deployed in the correct position. CONCLUSION: A direct videoscopic approach to the descending thoracic aorta proved a feasible technique for branched endograft delivery to the aortic arch and abdominal aorta in a human cadaver model.

Open surgical repair of ruptured juxtarenal aortic aneurysms with and without renal cooling: observations regarding morbidity and mortality.

OBJECTIVES: Little is known about the outcome of ruptured juxtarenal aortic aneurysm (RJAA) repair. Surgical treatment of RJAAs requires suprarenal aortic cross-clamping, which causes additional renal ischemia-reperfusion injury on top of the pre-existing hypovolemic shock syndrome. As endovascular alternatives rarely exist in this situation, open repair continues to be the gold standard. We analyzed our results of open RJAA repair during an 11-year period. DESIGN: Retrospective observational study. MATERIALS AND METHODS: Between July 1997 and December 2008, all consecutive patients with RJAAs were included in the study. Part of these patients received cold perfusion of the kidneys during suprarenal aortic cross-clamping. Perioperative variables, morbidity, and 30-day or in-hospital mortality were assessed. Renal insufficiency was defined as an acute rise of >or=0.5 mg/dL in serum creatinine level. Multiple organ failure (MOF) was scored using the sequential organ failure assessment score (SOFA score). RESULTS: A total of 29 consecutive patients with an RJAA, confirmed by computed tomography-scanning, presented to our hospital. In eight patients, the operation was aborted before the start of aortic repair, because no blood pressure could be regained in spite of maximal resuscitation measures. They were excluded from further analysis. Of the remaining 21 patients, 10 died during hospital stay. Renal insufficiency occurred in 11 out of 21 of the patients. Eleven out of 21 patients developed MOF postoperatively. In a subgroup of patients who received renal cooling during suprarenal aortic clamping, the 30-day or in-hospital mortality was two of 10 vs eight of 11 in patients who did not receive renal cooling (P = .03); renal insufficiency occurred in one out of 10 patients in the subgroup with renal cooling vs 10 out of 11 without renal cooling (P < .001) and MOF in two of 10 vs nine of 11, respectively (P = .009). CONCLUSIONS: Open surgical repair of RJAAs is still associated with high mortality and morbidity. To our knowledge, this is the first report of cold perfusion of the kidneys during RJAA repair. Although numbers are small, a beneficial effect of renal cooling on the outcome of RJAA repair is suggested, warranting further research with this technique.