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.

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 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.