Correlation of patient age with CT-measured aorta-sacral promontory distance.

INTRODUCTION AND HYPOTHESIS: Pelvic floor disorders are becoming more prevalent in the elderly population. Since more patients are seeking definitive management for their prolapse, the number of elderly patients undergoing sacral colpopexies will likely increase. During sacral colpopexies, the surgeon must carefully dissect in the presacral space and avoid vital structures. In elderly patients the aorta potentially elongates and the vertebral body height decreases. Consequently, there is a potential for anatomical change of distance from the bifurcation of the aorta to the sacral promontory. This study aimed to correlate the aorta-sacral promontory distance with age. METHODS: From 1 January 2013 to 31 January 2014 computed tomography (CT) images of 241 patients were reviewed in this retrospective study. Radiologists measured the aorta-sacral promontory distance on sagittal acquisition. The corresponding demographic information of age, body mass index, and comorbidities was evaluated using univariate analysis and univariate linear regression. RESULTS: The mean age was 56.6 years, and BMI was 27.6. The mean aorta-sacral promontory measurement based on the CT scan was 63.11 mm. Univariate analysis using a t test and ANOVA demonstrated an inverse correlation with age (p < 0.0001) and hypertension (p = 0.0034) and a positive correlation with BMI categories (p < 0.0017) Under univariate linear regression, the weight of the patient in kilograms demonstrated positive correlation (p = 0.0413). CONCLUSIONS: Based on CT measurements, the aorta-sacral promontory distance is decreased in elderly and hypertensive patients. Heavier patients have an increased aorta-sacral promontory distance. These potential anatomical variants should be considered before operating in the presacral space.

Correlation of Computed Tomography Measured Presacral Thickness with Body Mass Index.

Sacral colpopexy is often chosen as a reliable approach that effectively resolves vaginal vault prolapse. Advancements in minimally invasive technology, robotic and laparoscopic surgery, have helped facilitate surgical dissection and operation when performing this procedure. An increased presacral thickness can potentially present a surgical challenge when operating in the presacral space. We hypothesize that there is a correlation between body mass index and presacral thickness. Computed Tomography (CT) images of 241 patients were reviewed in this retrospective study. The presacral thickness was measured by taking the cross sectional distance from the sacral promontory to the upper aspect of the iliac arteries. The corresponding demographic information of age, body mass index (BMI), and comorbidities were evaluated using univariate analysis, linear regression, and multiple regression analysis. The mean age was 56.6 years, and BMI was 27.6. The mean presacral thickness measurement based on the CT scan was 21.08 mm. Univariate linear regression models demonstrated a positive correlation between presacral thickness and BMI and a negative correlation with age. When adjusting for both age and BMI on multivariate analysis, a positive correlation with hypertension was found. The surgeon should be aware of this potential change in anatomy when operating in the presacral space.

NS-HGlio: A generalizable and repeatable HGG segmentation and volumetric measurement AI algorithm for the longitudinal MRI assessment to inform RANO in trials and clinics.

Background: Accurate and repeatable measurement of high-grade glioma (HGG) enhancing (Enh.) and T2/FLAIR hyperintensity/edema (Ed.) is required for monitoring treatment response. 3D measurements can be used to inform the modified Response Assessment in Neuro-oncology criteria. We aim to develop an HGG volumetric measurement and visualization AI algorithm that is generalizable and repeatable. Methods: A single 3D-Convoluted Neural Network, NS-HGlio, to analyze HGG on MRIs using 5-fold cross validation was developed using retrospective (557 MRIs), multicentre (38 sites) and multivendor (32 scanners) dataset divided into training (70%), validation (20%), and testing (10%). Six neuroradiologists created the ground truth (GT). Additional Internal validation (IV, three institutions) using 70 MRIs, and External validation (EV, single institution) using 40 MRIs through measuring the Dice Similarity Coefficient (DSC) of Enh., Ed. ,and Enh. + Ed. (WholeLesion/WL) tumor tissue and repeatability testing on 14 subjects from the TCIA MGH-QIN-GBM dataset using volume correlations between timepoints were performed. Results: IV Preoperative median DSC Enh. 0.89 (SD 0.11), Ed. 0.88 (0.28), WL 0.88 (0.11). EV Preoperative median DSC Enh. 0.82 (0.09), Ed. 0.83 (0.11), WL 0.86 (0.06). IV Postoperative median DSC Enh. 0.77 (SD 0.20), Ed 0.78. (SD 0.09), WL 0.78 (SD 0.11). EV Postoperative median DSC Enh. 0.75 (0.21), Ed 0.74 (0.12), WL 0.79 (0.07). Repeatability testing; Intraclass Correlation Coefficient of 0.95 Enh. and 0.92 Ed. Conclusion: NS-HGlio is accurate, repeatable, and generalizable. The output can be used for visualization, documentation, treatment response monitoring, radiation planning, intra-operative targeting, and estimation of Residual Tumor Volume among others.

AAV gene therapy for Tay-Sachs disease.

Tay-Sachs disease (TSD) is an inherited neurological disorder caused by deficiency of hexosaminidase A (HexA). Here, we describe an adeno-associated virus (AAV) gene therapy expanded-access trial in two patients with infantile TSD (IND 18225) with safety as the primary endpoint and no secondary endpoints. Patient TSD-001 was treated at 30 months with an equimolar mix of AAVrh8-HEXA and AAVrh8-HEXB administered intrathecally (i.t.), with 75% of the total dose (1 × 1014 vector genomes (vg)) in the cisterna magna and 25% at the thoracolumbar junction. Patient TSD-002 was treated at 7 months by combined bilateral thalamic (1.5 × 1012 vg per thalamus) and i.t. infusion (3.9 × 1013 vg). Both patients were immunosuppressed. Injection procedures were well tolerated, with no vector-related adverse events (AEs) to date. Cerebrospinal fluid (CSF) HexA activity increased from baseline and remained stable in both patients. TSD-002 showed disease stabilization by 3 months after injection with ongoing myelination, a temporary deviation from the natural history of infantile TSD, but disease progression was evident at 6 months after treatment. TSD-001 remains seizure-free at 5 years of age on the same anticonvulsant therapy as before therapy. TSD-002 developed anticonvulsant-responsive seizures at 2 years of age. This study provides early safety and proof-of-concept data in humans for treatment of patients with TSD by AAV gene therapy.

Pneumocele of the frontal sinus producing orbital roof defect: case report and review of literature.

Type III frontal recess air cell as a cause of frontal sinus pneumocele has not been previously reported in literature. A 31-year-old woman with chronic history of sinusitis presented with pressure in the left eye on blowing the nose. Computed tomography examination of the orbits and paranasal sinuses with coronal and sagittal reformatted images showed abnormal collection of gas in the soft tissues at the superior aspect of the left orbit contiguous with the overlying left frontal sinus through a large defect in the orbital roof and a type III frontal recess air cell narrowing the left frontal recess.

Volume and Infusion Rate Dynamics of Intraparenchymal Central Nervous System Infusion in a Large Animal Model.

Thalamic infusion of adeno-associated viral (AAV) vectors has been shown to have therapeutic effects in neuronopathic lysosomal storage diseases. Preclinical studies in sheep model of Tay-Sachs disease demonstrated that bilateral thalamic injections of AAV gene therapy are required for maximal benefit. Translation of thalamic injection to patients carries risks in that (1) it has never been done in humans, and (2) dosing scale-up based on brain weight from animals to humans requires injection of larger volumes. To increase the safety margin of this infusion, a flexible cannula was selected to enable simultaneous bilateral thalamic infusion in infants while monitoring by imaging and/or to enable awake infusions for injection of large volumes at low infusion rates. In this study, we tested various infusion volumes (200-800 µL) and rates (0.5-5 µL/min) to determine the maximum tolerated combination of injection parameters. Animals were followed for ∼1 month postinjection with magnetic resonance imaging (MRI) performed at 14 and 28 days. T1-weighted MRI was used to quantify thalamic damage followed by histopathological assessment of the brain. Trends in data show that infusion volumes of 800 µL (2 × the volume required in sheep based on thalamic size) resulted in larger lesions than lower volumes, where the long infusion times (between 13 and 26 h) could have contributed to the generation of larger lesions. The target volume (400 µL, projected to be sufficient to cover most of the sheep thalamus) created the smallest lesion size. Cannula placement alone did result in damage, but this is likely associated with an inherent limitation of its use in a small brain due to the length of the distal rigid portion and lack of stable fixation. An injection rate of 5 µL/min at a volume ∼1/3 of the thalamus (400-600 µL) appears to be well tolerated in sheep both clinically and histopathologically.

Imaging of Spine Fractures With Emphasis on the Craniocervical Junction.

Spinal cord injury often times is a catastrophic result of trauma. Delay in diagnosis may result in increased morbidity and mortality. Cross-sectional imaging is now increasingly used as a first-line diagnostic modality in the setting of trauma for recognition of spine fractures and ligamentous injuries that might be missed on routine radiographs. The learning objectives of this article are to review the anatomy of the spine and understand the mechanisms of injury in the cervical, thoracic, and lumbosacral column by applying easy and reproducible classification systems to guide clinical management.

Vascular type Ehlers-Danlos Syndrome with fatal spontaneous rupture of a right common iliac artery dissection: case report and review of literature.

Vascular Ehlers-Danlos Syndrome (previously Ehlers-Danlos IV) is a rare autosomal dominant collagen vascular disorder caused by a 2q31 COL3A1 gene mutation encoding pro-alpha1 chain of type III collagen (in contrast to classic Ehlers-Danlos, caused by a COL5A1 mutation). The vascular type accounts for less than 4% of all Ehlers-Danlos cases and usually has a poor prognosis due to life threatening vascular ruptures and difficult, frequently unsuccessful surgical and vascular interventions. In 70% of cases, vascular rupture or dissection, gastrointestinal perforation, or organ rupture is a presenting sign. We present a case of genetically proven vascular Ehlers-Danlos with fatal recurrent retroperitoneal hemorrhages secondary to a ruptured right common iliac artery dissection in a 30-year-old male. This case highlights the need to suspect collagen vascular disorders when a young adult presents with unexplained retroperitoneal hemorrhage, even without family history of such diseases.