Contemporary outcomes after treatment of aberrant subclavian artery and Kommerell's diverticulum.

OBJECTIVE: Aberrant subclavian artery (ASA) and Kommerell's diverticulum (KD) are rare vascular anomalies that may be associated with lifestyle-limiting and life-threatening complications. The aim of this study is to report contemporary outcomes after invasive treatment of ASA/KD using a large international dataset. METHODS: Patients who underwent treatment for ASA/KD (2000-2020) were identified through the Vascular Low Frequency Disease Consortium, a multi-institutional collaboration to investigate uncommon vascular disorders. We report the early and mid-term clinical outcomes including stroke and mortality, technical success, and other operative outcomes including reintervention rates, patency, and endoleak. RESULTS: Overall, 285 patients were identified during the study period. The mean patient age was 57 years; 47% were female and 68% presented with symptoms. A right-sided arch was present in 23%. The mean KD diameter was 47.4 mm (range, 13.0-108.0 mm). The most common indication for treatment was symptoms (59%), followed by aneurysm size (38%). The most common symptom reported was dysphagia (44%). A ruptured KD was treated in 4.2% of cases, with a mean diameter of 43.9 mm (range, 18.0-100.0 mm). An open procedure was performed in 101 cases (36%); the most common approach was ASA ligation with subclavian transposition. An endovascular or hybrid approach was performed in 184 patients (64%); the most common approach was thoracic endograft and carotid-subclavian bypass. A staged operative strategy was employed more often than single setting repair (55% vs 45%). Compared with endovascular or hybrid approach, those in the open procedure group were more likely to be younger (49 years vs 61 years; P < .0001), female (64% vs 36%; P < .0001), and symptomatic (85% vs 59%; P < .0001). Complete or partial symptomatic relief at 1 year after intervention was 82.6%. There was no association between modality of treatment and symptom relief (open 87.2% vs endovascular or hybrid approach 78.9%; P = .13). After the intervention, 11 subclavian occlusions (4.5%) occurred; 3 were successfully thrombectomized resulting in a primary and secondary patency of 95% and 96%, respectively, at a median follow-up of 39 months. Among the 33 reinterventions (12%), the majority were performed for endoleak (36%), and more reinterventions occurred in the endovascular or hybrid approach than open procedure group (15% vs 6%; P = .02). The overall survival rate was 87.3% at a median follow-up of 41 months. The 30-day stroke and death rates were 4.2% and 4.9%, respectively. Urgent or emergent presentation was independently associated with increased risk of 30-day mortality (odds ratio [OR], 19.8; 95% confidence interval [CI], 3.3-116.6), overall mortality (OR, 3.6; 95% CI, 1.2-11.2) and intraoperative complications (OR, 8.3; 95% CI, 2.8-25.1). Females had a higher risk of reintervention (OR, 2.6; 95% CI, 1.0-6.5). At an aneurysm size of 44.4 mm, receiver operator characteristic curve analysis suggested that 60% of patients would have symptoms. CONCLUSIONS: Treatment of ASA/KD can be performed safely with low rates of mortality, stroke and reintervention and high rates of symptomatic relief, regardless of the repair strategy. Symptomatic and urgent operations were associated with worse outcomes in general, and female gender was associated with a higher likelihood of reintervention. Given the worse overall outcomes when symptomatic and the inherent risk of rupture, consideration of repair at 40 mm is reasonable in most patients. ASA/KD can be repaired in asymptomatic patients with excellent outcomes and young healthy patients may be considered better candidates for open approaches versus endovascular or hybrid modalities, given the lower likelihood of reintervention and lower early mortality rate.

A Systematic Review of Contemporary Outcomes from Aortic Arch In Situ Laser Fenestration During Thoracic Endovascular Aortic Repair.

BACKGROUND: In situ laser fenestrated endovascular aortic repair (L-FEVAR) is a novel and creative solution for complex aortic pathologies in the urgent and emergency setting. Outcomes of this technique, however, are poorly reported. We sought to evaluate the efficacy, safety, and outcomes of L-FEVAR in aortic arch pathologies. METHODS: A systematic literature review and analysis were conducted in accordance with the preferred reporting items for systematic reviews and meta-analyses and Cochrane guidelines. A search was conducted using Google, PubMed, and Scopus to identify studies evaluating L-FEVAR. Two independent reviewers determined study inclusion. Case reports and series including < 10 patients were excluded. Reviewers also assessed the methodological quality and extracted data regarding outcomes. A meta-analysis of endoleak event rates was conducted using a fixed-effect model due to small sample size. RESULTS: Eight studies met inclusion criteria between 2013 and 2021. Most studies were retrospective (87.5%) with median follow-up duration of 12.5 months (range 10-42). There were 440 patients included (range 15-148), mostly men (64%). Mean age was 61 years (range 53-68). Included patients were all symptomatic with L-FEVAR being technically successful in 93.3% of cases. The main indication for aortic arch intervention was aortic dissection. Single fenestrations occurred most frequently (68%), followed by triple (22%) then double fenestrations (9%). Meta-analysis of 8 studies (n = 440) demonstrated an endoleak event rate of 0.06 (95% confidence interval 0.04-0.09, P < 0.001) with no observed statistically significant heterogeneity of effects (Q = 7.91, P = 0.34). The median operative time was 162 min (range 53-252) with median length of stay of 10 days (range 7-17). Primary branch patency was 96.6%. Secondary patency rate was 97%. Pooled complication rates such as endoleak occurred in 4.8%, stroke in 2.0%, spinal cord ischemia in 0.2%, retrograde dissection in 0.9%, and 30-day death in 2.0%. Access complications occurred in 0.4%. Antiplatelet regimen was poorly reported in the study cohort. CONCLUSIONS: In situ laser fenestration is a feasible, safe, and effective approach to treat aortic arch disease in patients who are unsuitable for open or custom-made endovascular means. High technical success and excellent short-term branch patency can be achieved. These single-institution series exhibit promising short-term outcomes. In a similar paradigm to investigational device exemptions studies for custom-made and physician modified endografts, these preliminary data make a persuasive argument for larger long-term multi-institutional prospective study of this promising technique.

International Multi-Institutional Experience with Presentation and Management of Aortic Arch Laterality in Aberrant Subclavian Artery and Kommerell's Diverticulum.

BACKGROUND: Aberrant subclavian artery (ASA) with or without Kommerell's diverticulum (KD) is a rare anatomic aortic arch anomaly that can cause dysphagia and/or life-threatening rupture. The objective of this study is to compare outcomes of ASA/KD repair in patients with a left versus right aortic arch. METHODS: Using the Vascular Low Frequency Disease Consortium methodology, a retrospective review was performed of patients ≥18 years old with surgical treatment of ASA/KD from 2000 to 2020 at 20 institutions. RESULTS: 288 patients with ASA with or without KD were identified; 222 left-sided aortic arch (LAA), and 66 right-sided aortic arch (RAA). Mean age at repair was younger in LAA 54 vs. 58 years (P = 0.06). Patients in RAA were more likely to undergo repair due to symptoms (72.7% vs. 55.9%, P = 0.01), and more likely to present with dysphagia (57.6% vs. 39.1%, P < 0.01). The hybrid open/endovascular approach was the most common repair type in both groups. Rates of intraoperative complications, death within 30 days, return to the operating room, symptom relief and endoleaks were not significantly different. For patients with symptom status follow-up data, in LAA, 61.7% had complete relief, 34.0% had partial relief and 4.3% had no change. In RAA, 60.7% had complete relief, 34.4% had partial relief and 4.9% had no change. CONCLUSIONS: In patients with ASA/KD, RAA patients were less common than LAA, presented more frequently with dysphagia, had symptoms as an indication for intervention, and underwent treatment at a younger age. Open, endovascular and hybrid repair approaches appear equally effective, regardless of arch laterality.

The epidemiology of central and extraventricular neurocytoma in the United States between 2006 and 2014.

INTRODUCTION: Central neurocytoma (CN) and extraventricular neurocytoma (EVN) are rare intracranial tumors. There is a paucity of studies reporting the population-based incidence of these tumors. We used the Central Brain Tumor Registry of the United States (CBTRUS), which contains the largest aggregation of population-based data on the incidence of primary central nervous system tumors in the United States to describe these tumors. METHODS: The CBTRUS database, provided by CDC representing approximately 100% of the US population, was queried using the following search criteria: diagnosis years 2006-2014, ICD-0-3 histology codes (9506/0: central neurocytoma, benign; 9506/1: central neurocytoma, uncertain). Annual age-adjusted incidence rates are presented per 100,000 population. Incidence was estimated by age, gender, race, and ethnicity. RESULTS: The combined overall annual incidence rate of CN and EVN was 0.032 [0.030-0.034]. The incidence rates were 0.022 [0.021-0.024] and 0.009 [0.008-0.010] for CN and EVN, respectively. The most frequently documented locations for EVN were frontal lobe and cerebellum, followed by temporal lobe. Peak incidence was found in the 20-34 years range for both CN and EVN. The incidence rate was slightly lower in males compared to females for CN and identical for EVN. The overall incidence rate of CN and EVN combined was lower in Blacks 0.026 [0.021-0.032] and Hispanic Whites 0.020 [0.016-0.025] compared to Non-Hispanic Whites 0.035 [0.033-0.038]. CONCLUSION: CN and EVN are rare tumors with a peak incidence in the 20-34 years age group. This study represents the largest population-based epidemiological study on CN and EVN in the US.

The Art of Sleeve Gastrectomy.

Sleeve gastrectomy (SG) has historically evolved from gastroplasty and anti-reflux procedures into one of the most commonly performed primary metabolic surgeries in the United States and worldwide. Initially initiated in the 1980s as part of the duodenal switch procedure, its standalone effectiveness and simplicity have led to increasing popularity globally. The rise in obesity rates transcends age boundaries, alarmingly affecting not only adults but also the younger demographic. This escalating trend is concerning, as it predisposes these populations to numerous future health complications, as well as highlighting the critical necessity for a safe and potent weight loss strategy. Although sleeve gastrectomy carries a higher risk for gastroesophageal reflux disease (GERD) compared to other bariatric procedures, it stands out as a reliable, safe and effective surgical solution for obesity. It is particularly beneficial for adolescents and patients with complex medical comorbidities, including, but not limited to, heart failure and immunocompromisation. It has also served as a bridge for transplants in morbidly obese patients with end-stage heart, liver and kidney disease due to its favorable safety profile.

Use of Radiofrequency Technology in Endonasal Skull Base and Transcranial Procedures.

Objective Radiofrequency ablation is widely utilized in otorhinolaryngology. It is used for ablation, coagulation and resection, and hemostasis. It causes tissue destruction through a chemical interaction of ions. The potential benefit is to cause less thermal injury to surrounding tissues compared with other coagulative tools. In this article, we present novel uses of radiofrequency ablation in endoscopic endonasal, and transcranial surgery. This is the first study to describe its use in transcranial cases. Design, Setting, and Participants This is a retrospective study of patients between 2016 and 2018 who underwent either endoscopic endonasal or transcranial surgery where radiofrequency ablation was used. Main Outcome Measures We looked at indication for usage, blood loss, postoperative imaging to identify any stroke or edema, and clinical outcomes of these patients. Results The radiofrequency device was used in eight endoscopic endonasal cases and four craniotomies. Four cases were for encephalocele repair and eight were for various intracranial pathologies. In endonasal encephalocele repair, the radiofrequency ablation helped in shrinking the herniated brain while minimizing thermal injury to the surrounding tissue. In tumors resection, the combination of ablation and coagulation effect was particularly effective for highly vascularized tumors. There were no vascular or major neurologic injuries. Postoperative periencephalocele edema was noted in one case. Conclusion Initial experience with radiofrequency ablation showed that it was a safe technique to use in both endonasal skull-base and transcranial procedures. It seemed particularly useful for highly vascularized tumors but a greater experience is needed to further clarify its role in these procedures.

Xanthoma disseminatum with extensive respiratory involvement effectively treated with cladribine: A case report.

Xanthoma disseminatum (XD) is a rare and benign proliferative systemic disease that usually affects the skin and mucosal membranes with variable extent. Extensive systemic involvement can be associated with higher morbidity. There is paucity in the literature describing this rare pathological entity, and the ideal management remains controversial. In this article, we report our experience with cladribine in treating a case of XD. We documented the clinical and pathological manifestations of a 24-year-old woman who was initially diagnosed with rheumatoid arthritis. She presented to our institute with respiratory compromise and was found to have XD affecting skin, mucosal membranes, joints, and bone marrow. The patient received six cycles of cladribine for 6 months, during which she showed a remarkable response in relation to the respiratory lesions. Her hemoglobin also normalized and inflammatory markers gradually decreased to reach normal values. However, her skin lesions did not respond to treatment but no new lesions appeared. With our experience with cladribine, we believe that it could be a promising treatment option for XD. However, more work has to be conducted to determine the efficacy and safety in the long term.

Patient perceptions on mesh use in hernia repair: A prospective, questionnaire-based study.

BACKGROUND: There has been increasing media coverage regarding the controversy of using mesh in various operations. At this time, there are no published studies evaluating the potential influence of this controversy on patients' perceptions. Therefore, our study aimed to assess patient perceptions of hernia repair surgery with mesh as well as factors that may influence patient opinions. METHODS: A 16-item questionnaire evaluated each patient's perceptions of the use of mesh in their upcoming hernia repair. The primary outcomes of interest were their level of comfort regarding the possibility of hernia repair surgery with mesh, aversion to hernia surgery with mesh, and positive belief that mesh is a safe product in hernia repair surgery. RESULTS: We included 100 patients presenting for a hernia repair and 100 patients presenting for other operations. Both groups identified the media as their most common influence (37% and 40%, respectively). Factors leading to a high level of comfort regarding the possibility of mesh repair included believing mesh was a safe product (P < .001) and hearing about the advantages of mesh (P = .012) from medical professionals (P = .001). Factors leading to a positive belief that mesh was a safe product included the male sex (P = .015), a high socioeconomic standing (P = .006), and their own personal experience (P = .013). Factors leading to aversion to mesh use included the female sex (P = .006) and hearing about meshes causing mesh-related (P = .028) and wound-related complications (P = .025) as well as chronic pain (.008). CONCLUSION: Despite the high penetration of non-medical information in the population before presentation for medical care, most patients overall do not seem to be opposed to the concept of the use of mesh in a hernia repair, but there are certain factors associated with aversion to the use of mesh that physicians should acknowledge and should address this potential issue.

Malignant triton tumor diagnosed twelve years after radiosurgically treated vestibular schwannoma.

Stereotactic radiosurgery (SRS) has been used as the primary treatment for vestibular schwannoma (VS) over the last two decades. While literature is available on tumor response following SRS, very long-term follow-ups are not yet available. Malignant transformation of benign VS is very rare. There are only a few cases described in the literature, most of which report malignant transformation occurring between 5-10 years after radiosurgery. Here we report a case of a 65-year-old female, with no family history of neurofibromatosis, who presented with worsening mental status, gait instability and facial weakness. Twelve years prior to her presentation, she was diagnosed with a VS that was treated with stereotactic radiosurgery. The tumor had subsequently been stable on serial interval magnetic resonance imaging. She eventually presented with symptoms related to hydrocephalus and brain stem compression. Histopathologic analysis revealed a malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. She succumbed to her disease and passed away 10 months after her resection surgery. This case highlights the necessity for lifelong follow-up following SRS and the need to be vigilant when performing SRS in large VS.

Extraventricular neurocytoma with ganglioid differentiation of the sellar and parasellar regions in an elderly patient: A case report.

BACKGROUND: Extraventricular neurocytoma (EVN) is a rare variant of central neurocytoma which arises outside of the ventricular system. Diffuse ganglioid differentiation is a characteristic seen in a subset of these tumors which has an uncertain prognostic significance. Typically, EVN presents in children and young adults. Given the rarity of this tumor, the natural history and response to treatments remain unclear. CASE DESCRIPTION: We present a case of EVN with diffuse ganglioid differentiation in a 70-year-old male which arose in the midline parasellar region and extended into the third ventricle. This is the oldest such patient reported. Despite prior reports that extremes of age are associated with more aggressive behavior, the tumor in this case did not exhibit such an aggressive course. CONCLUSION: In this report, we review the natural history and clinical course of this patient and summarize the literature regarding this rare pathological entity. Our patient responded well to therapy despite older age, ganglioid differentiation, and higher mitotic index.

The epidemiology of spinal schwannoma in the United States between 2006 and 2014.

OBJECTIVE: Spinal schwannoma remains the third most common intradural spinal tumor following spinal meningioma and ependymoma. The available literature is generally limited to single-institution reports rather than epidemiological investigations. As of 1/1/2004, registration of all benign central nervous system tumors in the United States became mandatory after the Benign Brain Tumor Cancer Registries Amendment Act took action, which provided massive resources for United States population-based epidemiological studies. This article describes the epidemiology of spinal schwannoma in the United States from January 1, 2006, through December 31, 2014. METHODS: In this study, the authors utilized the Central Brain Tumor Registry of the United States, which corresponds to 100% of the American population. The Centers for Disease Control and Prevention's National Program of Cancer Registries and the National Cancer Institute's Surveillance Epidemiology and End Results program provide the resource for this data registry. The authors included diagnosis years 2006 to 2014. They used the codes per the International Coding of Diseases for Oncology, 3rd Edition: histology code 9560/0 and site codes C72.0 (spinal cord), C70.1 (spinal meninges), and C72.1 (cauda equina). Rates are per 100,000 persons and are age-adjusted to the 2000 United States standard population. The age-adjusted incidence rates and 95% confidence intervals are calculated by age, sex, race, and ethnicity. RESULTS: There were 6989 spinal schwannoma cases between the years 2006 and 2014. The yearly incidence eminently increased between 2010 and 2014. Total incidence rate was 0.24 (95% CI 0.23-0.24) per 100,000 persons. The peak adjusted incidence rate was seen in patients who ranged in age from 65 to 74 years. Spinal schwannomas were less common in females than they were in males (incidence rate ratio = 0.85; p < 0.001), and they were less common in blacks than they were in whites (IRR = 0.52; p < 0.001) and American Indians/Alaska Natives (IRR = 0.50; p < 0.001) compared to whites. There was no statistically significant difference in incidence rate between whites and Asian or Pacific Islanders (IRR = 0.92; p = 0.16). CONCLUSIONS: The authors' study results demonstrated a steady increase in the incidence of spinal schwannomas between 2010 and 2014. Male sex and the age range 65-74 years were associated with higher incidence rates of spinal schwannomas, whereas black and American Indian/Alaska Native races were associated with lower incidence rates. The present study represents the most thorough assessment of spinal schwannoma epidemiology in the American population.