The Incidence, Localization and Clinical Relevance of Arterial Fenestrations and Their Association to Brain Aneurysms: A Case-Control Study Based on the STROBE Guidelines.

Background: Fenestrations are rare, but well-known, vascular variations of the cerebral arteries. They are mostly incidental, asymptomatic angiographic findings and might precipitate vascular lesions such as AVM, aneurysmal dilatation, or even ischemic symptoms. However, association between arterial fenestration and brain aneurysms has not been clearly established. Objective: To evaluate whether incidence of arterial fenestrations are associated with brain aneurysm development and investigate the prevalence and most-common localizations of arterial fenestrations of the human brain. Design: Case−control study. Setting: All patients examined by CT angiography in University Hospital No. 4 in Lublin from 2009 to 2019. Patients: Each patient showing at least one cerebral aneurysm was included in the case group and each patient without cerebral aneurysm on CT angiography was included in the control group. Measurements: CT angiography examinations were conducted using the standard protocol used in the 1st Department of Radiology, Medical University of Lublin, Poland. The database and statistical research were conducted by use of the Statistica software (ver. 13.3, Tibco Software Inc., Palo Alto, CA, USA). Results: A total of 6545 CTA examinations were included in the study. Most of the aneurysms were located on the MCA: 629 (38.59%), ICA: 466 (28.59%) and AComA: 192 (11.78%). Cerebral arterial fenestration showed a non-statistically significant elevated risk for brain aneurysms in the entire study population (OR: 1.157; 95% CI: 0.826−1.621; p = 0.39). Among 6545 cranial CTA examinations, cerebral vessel fenestration was found in 49 of them, which constituted 0.75%. The most common vascular fenestrations were those located in the ACA (30.61%), BA (30.61%) and AComA (22.45%), while other fenestrations occurred infrequently. There were no significant differences in the age of patients in the individuals with vascular fenestration (p > 0.05). VA fenestration was slightly more common in men (16.67%) than in women (5.41%). However, these differences were not statistically significant (p = 0.216). Limitations: Our study has several limitations, including selection bias regarding examined population. Second, we assume that the total number of fenestrations detected in our study was underestimated due to the limitations of the CT method in comparison to other radiologic modalities. Conclusions: Cerebral arterial fenestrations are rare vascular malformations. The ACA is the most common localization of fenestrations, followed by BA and AComA. Fenestrations of cerebral arteries insignificantly increase the risk of cerebral aneurysm formation. Further prospective studies are necessary to make this association more precise.

Accuracy of SCORTEN in predicting mortality in toxic epidermal necrolysis.

BACKGROUND: Toxic epidermal necrolysis (TEN) patients require multi-directional and multi-disciplinary treatment. In most cases, they are hospitalised at intensive care units and require multi-directional, burn-complication preventive care. Choosing the most appropriate treatment option might be troublesome even when predicting scores are used. SCORTEN is the most renowned prognostic score for TEN patients, however, there are some data indicating that the accuracy of this test may be limited. The credibility of not just the predicted mortality risk, but also componential laboratory results and clinical features subject to debate. The aim of this study was to evaluate the efficacy and credibility of SCORTEN in clinical practice, on proprietary material. METHODS: A retrospective analysis of 35 patients with diagnosed in histopathology TEN was performed. The inclusion criteria were as follows: day of submission before 5th day from the onset of the symptoms, full protocol of plasmaphereses and IVIGs according to our scheme. Our protocol includes cycle of plasmapheresis with frozen fresh plasma twice daily for the first 2 days following admission, and once daily for the subsequent 5 to 7 days. IVIGs were administered after the first two sessions of plasmapheresis, for 4 to 7 days. The dosage was calculated according to body weight, at 0.4 to 0.5 g/kg per dose. RESULTS: The sensitivity of SCORTEN for the analysed cohort was 100%, with a specificity of 24%. The estimated death was 41,9%, while the actual death rates were 12,5%. Our protocol improved the survival, OR = 26,57, RR = 6,34, p = 0,022. Decrease in mortality was caused by a combined treatment protocol we use- plasmaphereses with IVIGs. No independent risk factor was significant in death evaluation. CONCLUSION: Our data suggest that the scoring system for predicting death among TEN patients are reliable when they are high. New prognostic factors should be found to improve the evaluation of patients with low SCORTEN.

Immunomodulatory Treatment of Lyell's Syndrome: A Simultaneous Plasmapheresis and Intravenous Immunoglobulins Therapy.

Lyell's syndrome, or toxic epidermal necrolysis (TEN) is a rare but life-threatening condition. It manifests with blistering of skin and mucous due to subepidermal bullae and keratinocyte necrosis. In most cases, it is an immune response to drugs or their metabolites. The mortality in TEN is high despite optimal infection and wound control. There are no unequivocal treatment guidelines in TEN. Immunosuppressive treatment may increase the wound infection risk and mortality. The aim of the study was to evaluate a 10-year experience with immunomodulatory therapy in TEN. We perform a combination of plasmapheresis and intravenous immunoglobulins to control the disease. There were 35 patients in the group and we performed a post hoc evaluation. Twenty-eight patients received the full protocol and there were seven patients who did not complete the treatment (single therapy group). The mortality in the test group was 14.29%, and the difference reached statistical significance in comparison with the single therapy group (P < .05). Our protocol reduced the mortality risk five times. Our study proved that simultaneous plasmaphereses with intravenous immunoglobulins administration were safe and improved patients' outcome in TEN.