Pelvic congestion syndrome is defined as chronic pelvic pain due to incompetent (dilated and refluxing) pelvic veins. The aim of this study was to investigate the prevalence of this condition by ...examining the prevalence of dilated ovarian and para-uterine veins in pre- and postmenopausal female patients, irrespective of their symptoms. We subsequently investigated how many women with dilated veins suffered from chronic pelvic pain. Additionally, we attempted to define diagnostic criteria that may allow for early identification of affected patients.
We reassessed 2384 abdomino-pelvic computed tomography scans performed on women at our institution. The maximal diameters of the ovarian and para-uterine veins were measured. Patients with a pathological process in the abdomen or pelvis affecting the veins were excluded. We considered ovarian vein dilation to be 6 mm or more in the axial plane. For patients that met these criteria, we performed a retrospective chart review to evaluate the clinical presentation and/or symptoms of these patients.
Dilated pelvic veins were present in 293/2384 (12%) patients, 118/559 premenopausal (21%) and 175/1825 postmenopausal (10%). Chronic pelvic pain of unclear etiology had been documented prior to the CT in 54/293 (18%) women with dilated veins-2% of the whole study collective (54/2384); 8% of all premenopausal (44/559) and 0.5% of all postmenopausal (10/1825). It was often accompanied by urological symptoms such as hematuria, dysuria, and urinary frequency, in the absence of infection (p<0.05). We identified a strong correlation between the presence of dilated ovarian veins and chronic pelvic pain in premenopausal parous patients with hematuria.
Pelvic congestion syndrome appears to be an underdiagnosed and undertreated disease. In our study, 8% of all premenopausal women had documented chronic pelvic pain of unclear etiology and dilated ovarian and pelvic veins on cross-sectional imaging studies. The features we identified in this study as most relevant should enable a faster identification of patients who could benefit from a specific treatment regimen for this condition.
Development of the CVJ is a complex process rarely analyzed by CT. Cartilaginous remnants within the atlas and axis have been shown to variably persist throughout childhood and may be mistaken for ...fractures. The purpose of this study was to better estimate the fusion timeline of the synchondroses at the CVJ.
We retrospectively reviewed singular CT scans in 550 children without known skeletal dysplasia or maturation delay (from neonate to 17 years) and analyzed the ossification of atlas and axis. Normal closing age for synchondroses was defined as soon as a complete ossification rate of 80% or more per age category was achieved.
No separate OC was observed in 38 of 230 incompletely ossified AAAs, whereas single, bipartite, or multiple OCs were observed in, respectively, 196, 86, and 16 of 298 AAAs in which OCs could be identified. Synchondroses at the AAA closed after 12 years (range 4.5-17 years). Posterior midline synchondrosis of the atlas closed after 4 years (range 2-13 years). The axis ossified from 6 OCs and 4 synchondroses. Subdental and neurocentral synchondroses closed simultaneously after 9 years (range 7-9.5 years). Apicodental synchondrosis and chondrum terminale were completely ossified after 10.5 years (range 5.5-13.5 years).
Analysis of the CVJ using triplanar CT reconstructions allows secure identification of characteristic developmental features of the atlas and axis, and helps to separate normal variants from true osseous lesions.
We assessed the efficacy and safety of mechanical thrombectomy (MT) in adult stroke patients with anterior circulation large vessel occlusion presenting in the late time window not fulfilling the ...DEFUSE-3 (Thrombectomy for Stroke at 6 to 16 Hours With Selection by Perfusion Imaging trial) and DAWN (Thrombectomy 6 to 24 Hours After Stroke With a Mismatch Between Deficit and Infarct trial) inclusion criteria.
Cohort study of adults with anterior circulation large vessel occlusion admitted between 6 and 24 hours after last-seen-well at 5 participating Swiss stroke centers between 2014 and 2021. Mismatch was assessed by computer tomography or magnetic resonance imaging perfusion with automated software (RAPID or OLEA). We excluded patients meeting DEFUSE-3 and DAWN inclusion criteria and compared those who underwent MT with those receiving best medical treatment alone by inverse probability of treatment weighting using the propensity score. The primary efficacy end point was a favorable functional outcome at 90 days, defined as a modified Rankin Scale score shift toward lower categories. The primary safety end point was symptomatic intracranial hemorrhage within 7 days of stroke onset; the secondary was all-cause mortality within 90 days.
Among 278 patients with anterior circulation large vessel occlusion presenting in the late time window, 190 (68%) did not meet the DEFUSE-3 and DAWN inclusion criteria and thus were included in the analyses. Of those, 102 (54%) received MT. In the inverse probability of treatment weighting analysis, patients in the MT group had higher odds of favorable outcomes compared with the best medical treatment alone group (modified Rankin Scale shift: acOR, 1.46 1.02-2.10;
=0.04) and lower odds of all-cause mortality within 90 days (aOR, 0.59 0.37-0.93;
=0.02). There were no significant differences in symptomatic intracranial hemorrhage (MT versus best medical treatment alone: 5% versus 2%,
=0.63).
Two out of 3 patients with anterior circulation large vessel occlusion presenting in the late time window did not meet the DEFUSE-3 and DAWN inclusion criteria. In these patients, MT was associated with higher odds of favorable functional outcomes without increased rates of symptomatic intracranial hemorrhage. These findings support the enrollment of patients into ongoing randomized trials on MT in the late window with more permissive inclusion criteria.
Cerebral small vessel disease (SVD) is the major cause of intracerebral hemorrhage (ICH). There is no comprehensive, easily applicable classification of ICH subtypes according to the presumed ...underlying SVD using MRI. We developed an MRI-based classification for SVD-related ICH.
We performed a retrospective study in the prospectively collected Swiss Stroke Registry (SSR, 2013-2019) and the Stroke InvestiGation in North And central London (SIGNAL) cohort. Patients with nontraumatic, SVD-related ICH and available MRI within 3 months were classified as Cerebral Amyloid angiopathy (CAA), Deep perforator arteriopathy (DPA), Mixed CAA-DPA, or Undetermined SVD using hemorrhagic and nonhemorrhagic MRI markers (CADMUS classification). The primary outcome was inter-rater reliability using Gwet's AC1. Secondary outcomes were recurrent ICH/ischemic stroke at 3 months according to the CADMUS phenotype. We performed Firth penalized logistic regressions and competing risk analyses.
The SSR cohort included 1,180 patients (median age interquartile range 73 62-80 years, baseline NIH Stroke Scale 6 2-12, 45.6% lobar hematoma, systolic blood pressure on admission 166 145-185 mm Hg). The CADMUS phenotypes were as follows: mixed CAA-DPA (n = 751 patients, 63.6%), undetermined SVD (n = 203, 17.2%), CAA (n = 154, 13.1%), and DPA (n = 72, 6.3%), with a similar distribution in the SIGNAL cohort (n = 313). Inter-rater reliability was good (Gwet's AC1 for SSR/SIGNAL 0.69/0.74). During follow-up, 56 patients had 57 events (28 ICH, 29 ischemic strokes). Three-month event rates were comparable between the CADMUS phenotypes.
CADMUS, a novel MRI-based classification for SVD-associated ICH, is feasible and reproducible and may improve the classification of ICH subtypes in clinical practice and research.
Background:
The DEFUSE-3 and DAWN trials showed that mechanical thrombectomy (MT) improves the outcome of selected patients with anterior circulation large vessel occlusions (LVO) up to 24 h after ...stroke onset. However, it is unknown whether only those patients fulfilling the trial inclusion criteria benefit, or whether benefit is seen in a broader range of patients presenting between 6 and 24 h.
Aims:
We determined whether fulfilling the DEFUSE-3 and DAWN selection criteria affects outcomes in MT patients in clinical practice.
Methods:
We reviewed adult patients with LVO treated with MT between 6 and 24 h after stroke onset at five Swiss stroke centers between 2014 and 2021. We compared two groups: (1) patients who satisfied neither DEFUSE-3 nor DAWN criteria (NDND) and (2) those who satisfied DEFUSE-3 or DAWN criteria (DOD). We used logistic regression to examine the impact of trial eligibility on two safety outcomes (symptomatic intracranial hemorrhage sICH and all-cause mortality at 3 months) and two efficacy outcomes (modified Rankin Score mRS shift toward lower categories and mRS of 0–2 at 3 months).
Results:
Of 174 patients who received MT, 102 (59%) belonged to the NDND group. Rates of sICH were similar between the NDND group and the DOD group (3% vs. 4%, p = 1.00). Multivariable regression revealed no differences in 3-month all-cause mortality (aOR 2.07, 95% CI 0.64–6.84, p = 0.23) or functional outcomes (mRS shift: acOR 0.81, 95% CI 0.37–1.79, p = 0.60; mRS 0–2: aOR 0.91, 95% CI 0.31–2.57, p = 0.85).
Conclusion:
Among adult patients with LVO treated with MT between 6 and 24 h, safety and efficacy outcomes were similar between DEFUSE-3/DAWN eligible and ineligible patients. Our data provide a compelling rationale for randomized trials with broader inclusion criteria for MT.
Introduction:
The impact of leptomeningeal collateralization on the efficacy of mechanical thrombectomy (MT) in patients with anterior circulation large vessel occlusion (aLVO) presenting in the 6-24 ...h time window remains poorly elucidated.
Patients and methods:
Retrospective multicenter study of aLVO patients presenting between 6 and 24 h after stroke onset who received MT plus Best Medical Treatment (BMT) or BMT alone. Leptomeningeal collateralization was assessed using single-phase computed tomography angiography (grade 0: no filling; grade 1: filling ⩽50%; grade 2: filling >50% but <100%; grade 3: filling 100% of the occluded territory). Inverse probability of treatment weighted ordinal regression was performed to assess the association between treatment and shift of the modified Rankin Scale (mRS) score toward lower categories at 3 months. We used interaction analysis to explore differential treatment effects on functional outcomes (probabilities for each mRS subcategory at 3 months) at different collateral grades.
Results:
Among 363 included patients, 62% received MT + BMT. Better collateralization was associated with better functional outcomes at 3 months in the BMT alone group (collateral grade 1 vs 0: acOR 5.06, 95% CI 2.33–10.99). MT + BMT was associated with higher odds of favorable functional outcome at 3 months (acOR 1.70, 95% CI 1.11–2.62) which was consistent after adjustment for collateral status (acOR 1.54, 95% CI 1.01–2.35). Regarding treatment effect modification, patients with absent collateralization had higher probabilities for a mRS of 0–4 and a lower mortality at 3 months for the MT + BMT group.
Discussion and conclusion:
In the 6-to-24-h time window, aLVO patients with absent leptomeningeal collateralization benefit most from MT + BMT, indicating potential advantages for this group despite their poorer baseline prognosis.
Graphical abstract
Aim To assess image quality and radiation dose in patients with body weights ≤75 kg undergoing abdominal computed tomography (CT) with a tube voltage of either 120 or 100 kVp. Materials and methods ...Eighty patients weighing ≤75 kg were prospectively assigned to receive either 120 or 100 kVp abdominal CT in the portal-venous phase. Attenuation values of abdominal organs and image noise were measured, and the contrast-to-noise ratios (CNRs) were calculated. Subjective image quality was assessed by three independent radiologists. Radiation exposure was assessed by size-specific dose estimate (SSDE). Results The mean attenuation of the kidney increased by 20% at 100 kVp ( p< 0.0001), and the mean image noise was 27% higher in the 100 kVp ( p =0.003). The CNR did not significantly differ between the groups (120 kVp, 6.6±2.8; 100 kVp, 7.4±3.6; p =0.26). Except for subjective image noise ( p< 0.001), no other subjective quality parameters (e.g., contrast, artefacts) were significantly different between the two groups ( p between 0.094 and 0.761). The mean SSDE in the 100-kVp group (9.8±1.8 mGy) was reduced by 19% compared to the 120-kVp group (12.1±1.8 mGy; p< 0.0001). Conclusion Manual reduction of tube voltage from the standard 120 to 100 kVp for portal-venous phase CT in patients with body weights ≤75 kg resulted in a 19% dose reduction while maintaining objective and subjective image quality.
Background:
Paroxysmal nocturnal hemoglobinuria (PNH) is an ultrarare bone marrow insufficiency of childhood consisted of the triad of diagnostic symptoms: hemolytic anemia, thrombosis, and ...peripheral blood cytopenias. Acute abdominal pain is one of the most important prodrome of PNH mostly due to venous thrombosis of intra‐abdominal veins, including hepatic, portal, mesenteric, and splenic blood vessels.
Aims:
To show how a complicated course of diseases due to “abdominal mask” resulted in more than 2 years delay in diagnosis might be fatal to a PNH patients.
Methods:
We report a boy of 15th, suffered from PNH, who presented long before the diagnosis with seasonal (spring and autumn only) paroxysmal acute and severe (8‐9 VAS score) abdominal pain with cramps and tenderness and no other hematological presentation of disease. There were a couple attacks observed during the season with complete resolution of abdominal symptoms in between. He had been diagnosed as acute appendicitis, peptic ulcer disease, pancreatitis or cholecystitis especially when hyperbilirubinemia emerge, Crohn and atypical celiac disease for more than 2 years. Since a peripheral pancytopenia followed by severe anemia required erythrocyte transfusions during the last 2 attacks occurred, hematologist had been asked for consultation. Due to severe anemia with reticulocytosis, hyperbilirubinemia, highly elevated LDH, meaningful splenomegaly and almost nondetectable concentration of serum haptoglobin and a dark urine with confirmed hemoglobinuria, with a weak bone marrow aplasia, the diagnosis of PNH was feasible. The cytometric results, which revealed no expression of GPI proteins in 93.8% of type III PMNs, confirmed the diagnosis (percentage of RBC lacking GPI was not persuasive, as he has been previously transfused). Up to this point of his disease, except of transient, he had no fixed postembolic lessons in liver and kidney, and a negligible bowel signs revealed for the several time in CT/MR‐angiography. He had no other embolic events in other organs as well.
Results:
As the results of HSCT are questionable in PNH, especially in children, we decided to start eculizumab. The complete clinical remission assessed as complete symptoms resolution had been achieved with the 2nd dose, and complete biochemical remission, based on LDH normalization, was confirmed with the 3rd dose of a MoAb therapy. The presence and percentage of PNH clones tested twice since diagnosis in both PNMs and RBCs did not change till now. We have not observed any complications during a year and half of treatment up to date of publication.
Summary/Conclusion:
The insidious and hidden course of thrombosis of intra‐abdominal veins even accompanied by hemolysis may mask the real reason and significantly prolong the diagnosis of ultrarare disease such as a PNH, especially in children. Eculizumab is a treatment of choice of childhood PNH.
Fanconi anaemia (FA) is a genetically heterogeneous autosomal recessive disorder associated with chromosomal fragility, bone-marrow failure, congenital abnormalities and cancer. The gene for ...complementation group A (FAA), which accounts for 60-65% of all cases, has been cloned, and is composed of an open reading frame of 4.3 kb, which is distributed among 43 exons. We have investigated the molecular pathology of FA by screening the FAA gene for mutations in a panel of 90 patients identified by the European FA research group, EUFAR. A highly heterogeneous spectrum of mutations was identified, with 31 different mutations being detected in 34 patients. The mutations were scattered throughout the gene, and most are likely to result in the absence of the FAA protein. A surprisingly high frequency of intragenic deletions was detected, which removed between 1 and 30 exons from the gene. Most microdeletions and insertions occurred at homopolymeric tracts or direct repeats within the coding sequence. These features have not been observed in the other FA gene which has been cloned to date (FAC) and may be indicative of a higher mutation rate in FAA. This would explain why FA group A is much more common than the other complementation groups. The heterogeneity of the mutation spectrum and the frequency of intragenic deletions present a considerable challenge for the molecular diagnosis of FA. A scan of the entire coding sequence of the FAA gene may be required to detect the causative mutations, and scanning protocols will have to include methods which will detect the deletions in compound heterozygotes.
Evidence-based hemostatic treatment for intracerebral hemorrhage (ICH) associated with non-vitamin K antagonist oral anticoagulants (NOACs) is lacking. Tranexamic acid (TXA) is an antifibrinolytic ...drug potentially limiting hematoma expansion. We aimed to assess the efficacy and safety of TXA in NOAC-ICH.
We performed a double-blind, randomized, placebo-controlled trial at 6 Swiss stroke centers. Patients with NOAC-ICH within 12 hours of symptom onset and 48 hours of last NOAC intake were randomized (1:1) to receive either intravenous TXA (1 g over 10 minutes followed by 1 g over 8 hours) or matching placebo in addition to standard medical care via a centralized Web-based procedure with minimization on key prognostic factors. All participants and investigators were masked to treatment allocation. Primary outcome was hematoma expansion, defined as ≥33% relative or ≥6 mL absolute volume increase at 24 hours and analyzed using logistic regression adjusted for baseline hematoma volume on an intention-to-treat basis.
Between December 12, 2016, and September 30, 2021, we randomized 63 patients (median age, 82 years interquartile range, 76-86; 40% women; median hematoma volume, 11.5 4.8-27.4 mL) of the 109 intended sample size before premature trial discontinuation due to exhausted funding. The primary outcome did not differ between TXA (n=32) and placebo (n=31) arms (12 38% versus 14 45%; adjusted odds ratio, 0.63 95% CI, 0.22-1.82;
=0.40). There was a signal for interaction with onset-to-treatment time (
=0.024), favoring TXA when administered within 6 hours of symptom onset. Between the TXA and placebo arms, the proportion of participants who died (15 47% versus 13 42%; adjusted odds ratio, 1.07 0.37-3.04;
=0.91) or had major thromboembolic complications within 90 days (4 13% versus 2 6%; odds ratio, 1.86 0.37-9.50;
=0.45) did not differ. All thromboembolic events occurred at least 2 weeks after study treatment, exclusively in participants not restarted on oral anticoagulation.
In a smaller-than-intended NOAC-ICH patient sample, we found no evidence that TXA prevents hematoma expansion, but there were no major safety concerns. Larger trials on hemostatic treatments targeting an early treatment window are needed for NOAC-ICH.
URL: https://clinicaltrials.gov; Unique identifier: NCT02866838.
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