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Background: Discovery of serum myelin oligodendrocyte glycoprotein (MOG) antibody testing in demyelination segregated MOG-IgG disease from AQ-4-IgG positive NMOSD. Aims: To study clinico-radiological manifestations, pattern of laboratory and electrophysiological investigations and response to treatment through follow up in MOG-IgG positive patients. Method: Retrospective data of MOG-IgG positive patients was collected. Demographics, clinical manifestations at onset and at follow up and relapses, anti AQ-4-IgG status, imaging and all investigations were performed, treatment of relapses and further immunomodulatory therapy were captured. Results: In our 30 patients, F: M ratio was 2.75:1 and adult: child ratio 4:1. Relapses at presentation were optic neuritis {ON}(60%), longitudinally extensive transverse myelitis {LETM}(20%), acute disseminated encephalomyelitis {ADEM}(13.4%), simultaneous ON with myelitis (3.3%) and diencephalic Syndrome (3.3%). Salient MRI features were ADEM-like lesions, middle cerebellar peduncle fluffy infiltrates, thalamic and pontine lesions and longitudinally extensive ON {LEON} as well as non-LEON. Totally, 50% patients had a relapsing course. Plasma exchange and intravenous immunoglobulin worked in patients who showed a poor response to intravenous methylprednisolone. Prednisolone, Azathioprine, Mycophenolate and Rituximab were effective attack preventing agents. Conclusions: MOG-IgG related manifestations in our cohort were monophasic/recurrent/simultaneous ON, myelitis, recurrent ADEM, brainstem encephalitis and diencephalic Syndrome. MRI features suggestive of MOG-IgG disease were confluent ADEM-like lesions, middle cerebellar peduncle fluffy lesions, LETM, LEON and non-LEON. Where indicated, patients need to go on immunomodulation as it has a relapsing course and can accumulate significant disability. Because of its unique manifestations, it needs to be considered as a distinct entity. To the best of our knowledge, this is the largest series of MOG-IgG disease reported from India.

WHAT THIS PAPER ADDSThis paper provides an overview of trends in peri-operative outcomes of patients who underwent standard endovascular aneurysm repair (EVAR) or open surgical repair (OSR) for an intact abdominal aortic aneurysm between 2014 and 2019 and who were registered in a mandatory nationwide registry. This study shows that all peri-operative outcomes (mortality, major complications, and textbook outcome) of EVAR and OSR for intact abdominal aortic aneurysms have improved since 2014, except for peri-operative mortality following EVAR which remained unchanged.Objective: The Dutch Surgical Aneurysm Audit (DSAA) initiative was established in 2013 to monitor and improve nationwide outcomes of aortic aneurysm surgery. The objective of this study was to examine whether outcomes of surgery for intact abdominal aortic aneurysms (iAAA) have improved over time. Methods: Patients who underwent primary repair of an iAAA by standard endovascular (EVAR) or open surgical repair (OSR) between 2014 and 2019 were selected from the DSAA for inclusion. The primary outcome was perioperative mortality trend per year, stratified by OSR and EVAR. Secondary outcomes were trends per year in major complications, textbook outcome (TbO), and characteristics of treated patients. The trends per year were evaluated and reported in odds ratios per year. Results: In this study, 11 624 patients (74.8%) underwent EVAR and 3 908 patients (25.2%) underwent OSR. For EVAR, after adjustment for confounding factors, there was no improvement in peri-operative mortality (aOR [adjusted odds ratio] 1.06, 95% CI 0.94 e 1.20), while major complications decreased

WHAT THIS PAPER ADDSThe peri-operative outcome of carotid endarterectomy is still mostly reported as a composite end point of combined ipsilateral stroke and death rate, both at individual patient level and at hospital level. This paper shows that textbook outcome, a composite measure achieved for an individual patient when all undesirable outcomes are absent, could be added to individual outcome measures to better evaluate hospital performance, especially in surgical interventions with low baseline risk such as carotid interventions.Objective: Composite measures may better objectify hospital performance than individual outcome measures (IOM). Textbook outcome (TO) is an outcome measure achieved for an individual patient when all undesirable outcomes are absent. The aim of this study was to assess TO as an additional outcome measure to evaluate quality of care in symptomatic patients treated by carotid endarterectomy (CEA). Methods: All symptomatic patients treated by CEA in 2018, registered in the Dutch Audit for Carotid Interventions, were included. TO was defined as a composite of the absence of 30 day mortality, neurological events (any stroke or transient ischaemic attack [TIA]), cranial nerve deficit, haemorrhage, 30 day readmission, prolonged length of stay (LOS; > 5 days) and any other surgical complication. Multivariable logistic regression was used to identify covariables associated with achieving TO, which were used for casemix adjustment for hospital comparison. For each hospital, an observed vs. expected number of events ratio (O/E ratio) was calculated and plotted in a funnel plot with 95% control limits. Results: In total, 70.7% of patients had a desired outcome within 30 days after CEA and therefore achieved TO. Prolonged LOS was the most common parameter (85%) and mortality the least common (1.1%) for not achieving TO. Covariates associated with achieving TO were younger age, the absence of pulmonary comorbidity, higher haemoglobin levels, and TIA as index event. In the case mix adjusted funnel plot, the O/E ratios between hospitals ranged between 0.63 and 1.27, with two hospitals revealing a statistically significantly lower rate of TO (with O/E ratios of 0.63 and 0.66). Conclusion:In the Netherlands, most patients treated by CEA achieve TO. Variation between hospitals in achieving TO might imply differences in performance. TO may be used as an additive to the pre-existing IOM, especially in surgical care with low baseline risk such as CEA.

WHAT THIS PAPER ADDS The new 2019 guideline of the European Society for Vascular Surgery recommends consideration for elective iliac artery aneurysm (IAA) repair when the iliac diameter exceeds 35 mm. This nationwide study from the Netherlands demonstrates that elective IAA repair is carried out at a median maximum diameter of 43 mm, and ruptured IAA repair at 68 mm. The findings appear to support the recent raise of the threshold diameter for elective IAA repair. Objective: The new 2019 guideline of the European Society for Vascular Surgery (ESVS) recommends consideration for elective iliac artery aneurysm (eIAA) repair when the iliac diameter exceeds 3.5 cm, as opposed to 3.0 cm previously. The current study assessed diameters at time of eIAA repair and ruptured IAA (rIAA) repair and compared clinical outcomes after open surgical repair (OSR) and endovascular aneurysm repair (EVAR). Methods: This retrospective observational study used the nationwide Dutch Surgical Aneurysm Audit (DSAA) registry that includes all patients who undergo aorto-iliac aneurysm repair in the Netherlands. All patients who underwent primary IAA repair between 1 January 2014 and 1 January 2018 were included. Diameters at time of eIAA and rIAA repair were compared in a descriptive fashion. The anatomical location of the IAA was not registered in the registry. Patient characteristics and outcomes of OSR and EVAR were compared with appropriate statistical tests. Results: The DSAA registry comprised 974 patients who underwent IAA repair. A total of 851 patients were included after exclusion of patients undergoing revision surgery and patients with missing essential variables. eIAA repair was carried out in 713 patients, rIAA repair in 102, and symptomatic IAA repair in 36. OSR was performed in 205, EVAR in 618, and hybrid repairs and conversions in 28. The median maximum IAA diameter at the time of eIAA and rIAA repair was 43 (IQR 38e50) mm and 68 (IQR 58e85) mm, respectively. Mortality was 1.3% (95% CI 0.7e2.4) after eIAA repair and 25.5% (95% CI 18.0e34.7) after rIAA repair. Mortality was not significantly different between the OSR and EVAR subgroups. Elective OSR was associated with significantly more complications than EVAR (intra-operative: 9.8% vs. 3.6%, postoperative: 34.0% vs. 13.8%, respectively). Conclusion: In the Netherlands, most eIAA repairs are performed at diameters larger than recommended by the ESVS guideline. These findings appear to support the recent increase in the threshold diameter for eIAA repair.

Background: While several observational studies suggested a lower postoperative mortality after minimal invasive endovascular aneurysm repair (EVAR) in patients with a ruptured abdominal aortic aneurysm (RAAA) compared to conventional open surgical repair (OSR), landmark randomized controlled trials have not been able to prove the superiority of EVAR over OSR. Randomized controlled trials contain a selected, homogeneous population, influencing external validity. Observational studies are biased and adjustment of confounders can be incomplete. Instrumental variable (IV) analysis (pseudorandomization) may help to answer the question if patients with an RAAA have lower postoperative mortality when undergoing EVAR compared to OSR. Methods: This is an observational study including all patients with an RAAA, registered in the Dutch Surgical Aneurysm Audit between 2013 and 2017. The risk difference (RD) in postoperative mortality (30 days/in-hospital) between patients undergoing EVAR and OSR was estimated, in which adjustment for confounding was performed in 3 ways: linear model adjusted for observed confounders, propensity score model (multivariable logistic regression analysis), and IV analysis (two-stage least square regression), adjusting for observed and unobserved confounders, with the variation in percentage of EVAR per hospital as the IV instrument. Results: 2419 patients with an RAAA (1489 OSR and 930 EVAR) were included. Unadjusted postoperative mortality was 34.9% after OSR and 22.6% after EVAR (RD 12.3%, 95% CI 8.5e16%). The RD adjusted for observed confounders using linear regression analysis and propensity score analysis was, respectively, 12.3% (95% CI 9.6e16.7%) and 13.2% (95%CI 9.3e 17.1%) in favor of EVAR. Using IV analysis, adjusting for observed and unobserved confounders, RD was 8.9% (95% CI-1.1e18.9%) in favor of EVAR.

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