Subclavian vein epithelioid hemangioendothelioma: Multidisciplinary surgical approach.

Epithelioid hemangioendothelioma (EHE) is a rare primary vascular tumour, usually malignant. We present the case of a 60-year-old woman who presented with hoarseness of voice and a persistent cough. During evaluation, a left supraclavicular mass was discovered. Initial assessments revealed a 30-mm left supraclavicular mass. Computed tomography angiogram and venogram confirmed an enhancing mass arising directly from the proximal left subclavian vein. After discussion in the joint multidisciplinary team, it was decided to resect the tumour owing to its malignant potential. Histopathology after resection confirmed a completely excised EHE. EHE is a rare vascular sarcoma requiring a multidisciplinary approach. Its main challenge is its unpredictable behaviour.

Aortic sodium [18F]fluoride uptake following endovascular aneurysm repair.

OBJECTIVE: In patients with abdominal aortic aneurysms, sodium [18F]fluoride positron emission tomography identifies aortic microcalcification and disease activity. Increased uptake is associated with aneurysm expansion and adverse clinical events. The effect of endovascular aneurysm repair (EVAR) on aortic disease activity and sodium [18F]fluoride uptake is unknown. This study aimed to compare aortic sodium [18F]fluoride uptake before and after treatment with EVAR. METHODS: In a preliminary proof-of-concept cohort study, preoperative and post-operative sodium [18F]fluoride positron emission tomography-computed tomography angiography was performed in patients with an infrarenal abdominal aortic aneurysm undergoing EVAR according to current guideline-directed size treatment thresholds. Regional aortic sodium [18F]fluoride uptake was assessed using aortic microcalcification activity (AMA): a summary measure of mean aortic sodium [18F]fluoride uptake. RESULTS: Ten participants were recruited (76±6 years) with a mean aortic diameter of 57±2 mm at time of EVAR. Mean time from EVAR to repeat scan was 62±21 months. Prior to EVAR, there was higher abdominal aortic AMA when compared with the thoracic aorta (AMA 1.88 vs 1.2; p<0.001). Following EVAR, sodium [18F]fluoride uptake was markedly reduced in the suprarenal (ΔAMA 0.62, p=0.03), neck (ΔAMA 0.72, p=0.02) and body of the aneurysm (ΔAMA 0.69, p=0.02) while it remained unchanged in the thoracic aorta (ΔAMA 0.11, p=0.41). CONCLUSIONS: EVAR is associated with a reduction in AMA within the stented aortic segment. This suggests that EVAR can modify aortic disease activity and aortic sodium [18F]fluoride uptake is a promising non-invasive surrogate measure of aneurysm disease activity.

18F-Sodium Fluoride Positron Emission Tomography and Computed Tomography in Acute Aortic Syndrome.

BACKGROUND: Acute aortic syndrome is associated with aortic medial degeneration. 18F-sodium fluoride (18F-NaF) positron emission tomography (PET) detects microscopic tissue calcification as a marker of disease activity. OBJECTIVES: In a proof-of-concept study, this investigation aimed to establish whether 18F-NaF PET combined with computed tomography (CT) angiography could identify aortic medial disease activity in patients with acute aortic syndrome. METHODS: Patients with aortic dissection or intramural hematomas and control subjects underwent 18F-NaF PET/CT angiography of the aorta. Aortic 18F-NaF uptake was measured at the most diseased segment, and the maximum value was corrected for background blood pool activity (maximum tissue-to-background ratio [TBRmax]). Radiotracer uptake was compared with change in aortic size and major adverse aortic events (aortic rupture, aorta-related death, or aortic repair) over 45 ± 13 months. RESULTS: Aortic 18F-NaF uptake co-localized with histologically defined regions of microcalcification and elastin disruption. Compared with control subjects, patients with acute aortic syndrome had increased 18F-NaF uptake (TBRmax: 1.36 ± 0.39 [n = 20] vs 2.02 ± 0.42 [n = 47] respectively; P < 0.001) with enhanced uptake at the site of intimal disruption (+27.5%; P < 0.001). 18F-NaF uptake in the false lumen was associated with aortic growth (+7.1 mm/year; P = 0.011), and uptake in the outer aortic wall was associated with major adverse aortic events (HR: 8.5 [95% CI: 1.4-50.4]; P = 0.019). CONCLUSIONS: In patients with acute aortic syndrome, 18F-NaF uptake was enhanced at sites of disease activity and was associated with aortic growth and clinical events. 18F-NaF PET/CT holds promise as a noninvasive marker of disease severity and future risk in patients with acute aortic syndrome. (18F Sodium Fluoride PET/CT in Acute Aortic Syndrome [FAASt]; NCT03647566).

Open extent IV thoracoabdominal aneurysm repair: 22-year experience of the Scottish National Service.

BACKGROUND: Since 1999, the Scottish National Service for Thoracoabdominal Aneurysms has offered repair of thoracoabdominal aneurysms (TAAAs) to a population of 5.5 million people. The open operation most commonly performed by the service is the extent IV TAAA repair. METHODS: All extent IV open TAAA repairs performed at the Scottish National Service for TAAAs from June 1999 until April 2021 were evaluated for clinical features, technical details, and clinical outcomes. The primary outcome measure was 30-day mortality; secondary outcomes included short-term (90 days, 6 months, 1 and 2 years) and long-term (5 and 10 years) survival, perioperative complications, and reintervention. Survival was assessed using Kaplan-Meier analysis. RESULTS: Some 248 patients underwent extent IV TAAA repair, with elective surgery in 204 (82.3 per cent). A totally abdominal transperitoneal approach was used for all patients, with a median visceral ischaemia time of 40 (i.q.r. 35-48) min. Overall, 18 patients (7.3 per cent) died within 30 days. The proportion of patients surviving at 90 days, 6 months, 1, 2, 5, and 10 years was 0.91, 0.90, 0.89, 0.85, 0.72, and 0.41, respectively. Ten patients (4.0 per cent) required a reintervention while in hospital, four (1.6 per cent) experienced permanent spinal cord ischaemia, 19 (7.9 per cent) required temporary renal replacement therapy (RRT), and four (1.6 per cent) required permanent RRT. CONCLUSION: Open extent IV TAAA repair performed in a high-volume national centre is associated with favourable short- and long-term survival, and acceptable complication rates.

Aorta, the Innocent Bystander of Bariatric Banding.

INTRODUCTION: Laparoscopic gastric banding (LAGB) is a common bariatric operation performed for obesity. Complications of LAGB commonly evolve around device malfunction and physiological changes secondary to the gastric banding. Complications of LAGB involving the aorta are rare. A case of gastric band misplacement around the aorta and subsequent successful retrieval of the misplaced device is reported. REPORT: A 45 year old obese woman presented as an emergency with food bolus obstruction secondary to gastric banding inserted 10 years previously. Investigations revealed that her gastric band was misplaced around both the oesophagus at the level of the gastro-oesophageal junction and the descending thoracic aorta at the level of T12. Successful and safe retrieval of the misplaced device is reported electively via a two staged approach: first covering the segment of supra-coeliac aorta at the level of the gastric band with a thoracic aortic stent graft (TAG), and, second, assessing for any oesophageal injury via endoscopy and finally extracting the misplaced device via laparoscopy. A Gore C-TAG device size 26 mm × 100 mm was successfully implanted percutaneously via unilateral femoral access during her first stage procedure. Her gastric band was safely retrieved during her second stage procedure with no complications. She recovered well post-operatively. DISCUSSION: Complications of LAGB involving the aorta are rare but potentially life threatening. Multidisciplinary pre-operative planning is necessary for safe removal of the gastric band.

Contemporary outcomes of open repair of thoracoabdominal aortic aneurysm in young patients.

BACKGROUND: Endovascular technology now permits total endovascular thoracoabdominal aortic aneurysm (TAAA) repair with high volume centres reporting encouraging results. The long-term durability of such stent grafts is unknown, leading to concerns regarding their use in younger patients. This study reports contemporary outcomes of open repair in young patients. METHODS: Outcomes for patients age 60 or younger undergoing open TAAA repair between June 1999 and August 2013 with prospective collected data were analysed retrospectively. RESULTS: Thirty-seven patients (31 men, 84%) with a median age of 56 (range 22-60) were identified with a median TAAA diameter of 6.9 cm (range 5.6-11). Aneurysm aetiology included degenerative change (18), dilation of chronic dissection (10), connective tissue disease (7) and mycotic degeneration (2). Crawford Type IV TAAA were most commonly treated (17), followed by Type II (10), Type III (7) and Type I (3). Two (5%) patients died in hospital, one from multiple organ failure and one from respiratory failure. Three patients (8%) developed temporary paraplegia, all of whom made a complete recovery and 4 (11%) patients required temporary renal replacement therapy. Median critical care stay was 5 days (range 2-28) with an in-hospital stay of 14 days (range 7-83). During a median follow-up of 72 months (range 13-171), no patient subsequently required any further aneurysm related surgical or radiological intervention. The mean (SEM) survival time was 138.5 (11) months. The 5 year survival was 79.7% (8.3) including early deaths, with no aneurysm related complications. CONCLUSIONS: The outcome of open TAAA repair in patients aged less than 60 years is favorable. It is against these results that evolving endovascular interventions must be compared.

Ruptured thoracoabdominal aneurysm in a 27-year-old with hyper IgE syndrome.

Hyperimmunoglobulin E syndrome is a rare primary immunodeficiency disorder defined by high serum immunoglobulin E titers and associated with characteristic infectious, dermatologic, skeletal, and dental abnormalities. We present the case of a 27-year-old man presenting acutely with a ruptured type IV thoracoabdominal aortic aneurysm. He was successfully treated by open operative repair and discharged with long-term prophylactic antibiotics. To our knowledge, this is the first such case reported in the English literature.