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OBJECTIVES The aim of this study was to explore sex and gender differences regarding aortic events in Marfan patients. METHODS We analysed all data from our connective tissue disorder database. Only patients with Marfan syndrome were included. For analysis, patients were divided by sex. Female patients were further divided into 2 subgroups: with versus without children. Aortic events were defined as Stanford type A aortic dissection (TAAD) or type B aortic dissection (TBAD) or any aortic intervention. RESULTS A population of 183 Marfan patients was analysed for the purpose of this study. One hundred four (57%) were male and 79 (43%) were female patients. Thirty-seven (47%) of the 79 female patients had at least 1 child. Male patients had a significantly higher probability of experiencing an aortic event (P = 0.015) compared to female patients. However, there was no increased probability for recurrent events in male patients compared to female patients (P = 0.063). Follow-up revealed no sex and gender differences in the occurrence of Stanford TAAD or TBAD between male and female patients (P = 0.324/P = 0.534). While 11% of women with children suffered from peripartum aortic events, 24% experienced Stanford TAAD unrelated to pregnancy. CONCLUSIONS Male patients have a higher risk of aortic events than female patients. The majority of women were not aware of their Marfan syndrome diagnosis before conceiving. One out of 10 women suffered from peripartum Stanford TAAD or TBAD. Twice as many female patients with children suffered from aortic dissection unrelated to childbirth. There were no sex and gender differences affecting mortality in Marfan patients.

OBJECTIVE The aim of the present comprehensive review was to present an overview of the clinical presentation and treatment options for external (EJVAs) and internal jugular vein aneurysms (IJVAs) to help clinicians in evidence-based decision making. METHODS A systematic literature search was conducted in accordance with the PRISMA (preferred reporting items for systematic reviews and meta-analyses) statement and included MEDLINE, Embase, Cochrane Library, Scopus, WHO (World Health Organization) trial register, ClinicalTrials.gov, and the LIVIVO search portal. The inclusion criteria were studies of patients who had presented with IJVAs or EJVAs. The exclusion criteria were animal and cadaver studies and reports on interventions using the healthy jugular vein for access only (ie, catheterization). Analysis of the pooled data from all eligible case reports was performed. RESULTS From 1840 identified reports, 196 studies were eligible. A total of 256 patients with JVAs were reported, with 183 IJVAs and 73 EJVAs. IJVAs were reported to occur in 66% on the right side compared with the left side (P��= .011). The patients with IJVAs were mostly children (median age, 12��years; interquartile range, 5.8-45.2��years). The patients with EJVAs were young adults (median age, 30��years; interquartile range, 11.0-46.5��years). EJVAs were more frequently reported in women and IJVAs in men (P��= .008). Most of the patients were asymptomatic. Pulmonary embolization in association with thrombosed EJVAs was only reported for one patient. A report of the outcomes after surgery and conservative management was missing for ���50% of the patients. No relevant complications were reported after ligation of the EJVA without reconstruction. Intracranial hypertension after ligation of the right-sided IJVA was reported in three children; in one of them, a pontine infarction was observed. CONCLUSIONS JVAs are a disease of the younger population but can occur at any age. It seems to be safe to observe patients with nonthrombosed JVAs. However, in the presence of thrombus or pulmonary embolization, surgical treatment should be considered. A reconstruction technique of the IJVA with venous patency preservation should be preferred.

Gastric Bypass: Weight Loss with Complications Abstract. Roux-en-Y gastric bypass (RYGBP) is the most often performed bariatric operation worldwide with internal hernia as one of the main long-term complications. To our knowledge, we report the first case of post-ischemic small-bowel strictures observed after a successful operation of an internal hernia after RYGBP. During emergency surgery a Petersen and a Brolin hernia were diagnosed and repaired. The initially ischemic small intestine was recovered. However, a week later the patient presented herself again due to ischemia-induced small-bowel strictures. These were treated successfully by endoscopic balloon dilatation.Zusammenfassung. Der Roux-Y-Magenbypass (RYGBP) stellt weltweit die am häufigsten durchgeführte bariatrische Operation dar – innere Hernien zählen zu den wichtigsten Langzeitkomplikationen. Unser Bericht einer 30-jährigen Patientin beschreibt erstmalig einen Fall einer ischämisch bedingten Dünndarmstenose nach RYGBP. Bei der Erstvorstellung wurden eine Petersen- und eine Brolin-Hernie diagnostiziert und laparoskopisch erfolgreich verschlossen. Der minderperfundierte Dünndarmanteil erholte sich in der Folge gut. Die Zweitvorstellung folgte eine Woche darauf bei postprandialem Erbrechen aufgrund postischämischer Dünndarmstenosen. Diese wurden mittels Ballondilatation erfolgreich behandelt.

BACKGROUND Surgical aortic valve replacement (SAVR) is the treatment of choice in severe symptomatic aortic valve disease. New techniques and prostheses have been recently developed to facilitate the procedure and reduce aortic cross-clamp time (AOx). The aim of this study was to analyse the different procedural steps in order to identify the most time-consuming part during aortic clamping time and to compare impact of experience on procedural aspects. METHODS AOx during SAVR was divided into five consecutive steps. Duration of each step was measured. The first procedural step started with clamping of the aorta and ended with the beginning of the second step starting with the resection of the native aortic valve. The third step started with placement of the first valve anchoring suture, the fourth step started with tying of the first suture and the fifth and final step started after the cut of the last suture and ended with removal of the aortic clamp. Surgeons were divided into two groups based on their experience, which in our analysis was defined as a total SAVR experience of more than 100 procedures. RESULTS From March 2013 to August 2015 57 nonconsecutive patients (33% female; age, median 71.0 years, interquartile range 65.0-76.0) undergoing isolated SAVR for severe aortic valve stenosis in our institution were included in this process analysis. Two different prostheses were implanted. Forty-eight (84%) patients received a tissue valve (Perimount Magna Ease, Edwards Lifesciences, Irvine, USA) and 9 (16%) patients received a mechanical prosthesis (Medtronic AP 360, St-Paul, MN, USA). The mean estimated risk of mortality was 1.1% (0.7-1.6) according to the logistic EUROScore II. Overall duration of AOx was 50.5 ± 13.8 min, with 32.3% (16.4 ± 5.9 min) accounting for placing the sutures into the native annulus and the prosthetic sewing ring and 18.5% (9.2 ± 3.0min) accounting for tying and cutting the sutures. Surgeons with more experience performed 35 operations (61.4%) and needed an average of 44.1 ± 11.5 min versus 60.6 ± 11.0 min (p

Wolfram syndrome (WS) is a rare, autosomic recessive genetic disorder. The mortality rate of WS is about 65% before 35 years of age. It presents diagnostic challenges in the clinical practice due to its incomplete characterization. This report represents the first case of undiagnosed Wolfram syndrome in a patient over 53 years old.A 53-year-old white woman developed a respiratory complication necessitating extended ICU care and respiratory rehabilitation. This respiratory complication proved to be a consequence of undiagnosed WS.The report discusses the clinical elements that suggested the diagnosis, the problems related to the ICU management of this patient, in particular the weaning difficulties, and the need for rehabilitation. Finally, the report considers the ethical aspect of timely diagnosis on the course and outcome of WS.