Storylines of family medicine III: core principles-primary care, systems and family.

Storylines of Family Medicine is a 12-part series of thematically linked mini-essays with accompanying illustrations that explore the many dimensions of family medicine, as interpreted by individual family physicians and medical educators in the USA and elsewhere around the world. In 'III: core principles-primary care, systems, and family', authors address the following themes: 'Continuity of care-building therapeutic relationships over time', 'Comprehensiveness-combining breadth and depth of scope', 'Coordination of care-managing multiple realities', 'Access to care-intersectional, systemic, and personal', 'Systems theory-a core value in patient-centered care', 'Family-oriented practice-supporting patients' health and well-being', 'Family physician as family member' and 'Family in the exam room'. May readers develop new understandings from these essays.

Non-contrast MRI of micro-vascularity of the feet and toes.

PURPOSE: This study aimed to develop novel non-contrast MR perfusion techniques for assessing micro-vascularity of the foot in human subjects. METHODS: All experiments were performed on a clinical 3 T scanner using arterial spin labeling (ASL). Seven healthy subjects (30-72 years old, 5 males and 2 females) were enrolled and bilateral feet were imaged with tag-on and tag-off alternating inversion recovery spin labeling for determining micro-vascularity. We compared an ASL technique with 1-tag against 4-tag pulses. For perfusion, we determined signal increase ratio (SIR) at varying inversion times (TI) from 0.5 to 2 s. SIR versus TI data were fit to determine perfusion metrics of peak height (PH), time to peak (TTP), full width at half maximum (FWHM), area under the curve (AUC), and apparent blood flow (aBF) in the distal foot and individual toes. Using analysis of variance (ANOVA), effects of tag pulse and region of interest (ROI) on the mean perfusion metrics were assessed. In addition, a 4-tag pulse perfusion experiment was performed on patients with peripheral artery disease (PAD) and Raynaud's disease. RESULTS: Using our MR perfusion techniques, SIR versus TI data showed well-defined leading and trailing edges, with a peak near TI of 0.75-1.0 s and subsiding quickly to near zero by TI of 2 s, particularly when 4-tag pulses were used. When imaged with 4-tag pulse, we found significantly greater values in perfusion metrics, as compared to 1-tag pulse. The patients with PAD and Raynaud's disease showed a reduced or scattered perfusion curves compared to the healthy control. CONCLUSION: MR perfusion imaging of the distal foot shows greater SIR and perfusion metrics with the 4-tag pulse compared to the 1-tag pulse technique. This will likely benefit those with low perfusion due to aging, PAD, diabetic foot, and other vascular diseases.

Imaging Findings Post Stereotactic Radiosurgery for Vestibular Schwannoma: A Primer for the Radiologist.

Non-invasive tumor control of vestibular schwannomas through stereotactic radiosurgery allows for high rates of long-term tumor control and has been used primarily for small and medium-sized vestibular schwannomas. The post-treatment imaging appearance of the tumor, temporal patterns of growth and treatment response, as well as extra-tumoral complications can often be both subtle or confusing and should be appropriately recognized. Herein, the authors present an imaging-based review of expected changes, as well as associated complications related to radiosurgery for vestibular schwannomas.ABBREVIATIONS: FSRT: Fractionated stereotactic radiotherapy, ISRS: international stereotactic radiosurgery, MPNST: Malignant peripheral nerve sheath tumor, SRS: Stereotactic radiosurgery, VS: Vestibular schwannoma.

Pathophysiology of Facial Nerve Stimulation and Its Implications for Electrical Stimulation in Cochlear Implants.

OBJECTIVES: A small number of cochlear implant (CI) users experience facial nerve stimulation (FNS), which can manifest as facial twitching. In some patients, this can be resolved by adjusting the electrical stimulation parameters. However, for others, facial stimulation can significantly impair CI outcomes or even prevent its use. The exact mechanisms underlying FNS are unclear and may vary among patients. DESIGN: Transimpedance measurements were used to assess lateral and longitudinal spread of current within 15 cochlea of nucleus CI recipients with FNS (13 unilateral recipients and 1 bilateral recipient). We compared the transimpedance measurements with programming parameters from clinical visits and pre- and postoperative temporal bone computed tomography (CT) scans to identify factors that may contribute to FNS in each CI ear. RESULTS: In nine ears, transimpedance curves showed inflection, which suggests a localized current sink within the cochlea. This indicates a low-impedance pathway through which current exits the cochlea and stimulates the labyrinthine segment of the facial nerve canal. Electrodes near this current sink were disabled or underfit to minimize facial stimulation. In the other seven ears, current flow peaked toward the basal end of the cochlea, suggesting that current exits through the round window or other structures near the basal end of the cochlea, stimulating the tympanic segment of the facial nerve. CONCLUSIONS: Objective transimpedance measurements can be used to elucidate the mechanisms of FNS and to develop strategies for optimizing electrical stimulation parameters and speech coding to minimize or eliminate FNS in a small subset of CI users.

Prevalence of Scarpa's ganglion enhancement on high-resolution MRI imaging.

BACKGROUND AND PURPOSE: The vestibular ganglion, or Scarpa's ganglion, is a cluster of afferent vestibular neurons within the internal auditory canal (IAC). There is minimal literature describing enhancement of this region on magnetic resonance imaging (MRI) and its correlation to clinical symptoms. Here, we sought to find the prevalence of enhancement at Scarpa's ganglion, and determine whether such enhancement correlates with demographics or clinical symptoms. MATERIALS AND METHODS: A retrospective review was performed of consecutive patients with an MRI of the IAC between 3/1/2021 and 5/20/2021. Two neuroradiologists independently reviewed for T1 and FLAIR enhancement of the Scarpa's ganglion on post-contrast fat-saturated T1 and post-contrast FLAIR images. Discrepancies were agreed upon by consensus. Clinical variables (hearing loss, vestibular symptoms, tinnitus, and MRI indication) were gathered from a retrospective chart review. RESULTS: Eighty-nine patients were included (51 female); the mean age was 58 (range 19-85). The most common MRI indication was hearing loss (n = 53). FLAIR enhancement was present on the right in 7 patients, on the left in 7 patients, and bilaterally in 6 patients. No enhancement was seen on post-contrast T1 images. There was no statistically significant correlation between consensus FLAIR on at least one side and age (p = .74), gender (p = .29), hearing loss (p = .32), hearing loss side (p = .39), type of hearing loss (p = .87), vestibular symptoms (p = .71), or tinnitus (p = .81). CONCLUSIONS: Enhancement is present in the minority of patients on post-contrast FLAIR images. If seen, it should be considered an uncommon but not unexpected finding with no clinical significance.

Magnetic Resonance Imaging Artifact Associated With Transcutaneous Bone Conduction Implants: Cholesteatoma and Vestibular Schwannoma Surveillance.

OBJECTIVE: To evaluate the magnetic resonance (MR) image artifact and image distortion associated with the two transcutaneous bone conduction implants currently available in the United States. STUDY DESIGN: Cadaveric study. METHODS: Two cadaveric head specimens (1 male, 1 female) were unilaterally implanted according to manufacturer guidelines and underwent MR imaging (General Electric and Siemens 1.5 T scanners) under the following device conditions: (1) no device, (2) Cochlear Osia with magnet and headwrap, (3) Cochlear Osia without magnet, and (4) MED-EL Bonebridge with magnet. Maximum metal mitigation techniques were employed in all conditions, and identical sequences were obtained. Blinded image scoring (diagnostic vs nondiagnostic image) was performed by experienced neuroradiologists according to anatomical subsites. RESULTS: All device conditions produced artifact and image distortion. The Osia with magnet produced diagnostic T1- and T2-weighted images of the ipsilateral temporal bone, however, non-echo planar imaging diffusion-weighted imaging (DWI) was nondiagnostic. The Osia without magnet scanned on the Siemens MR imaging demonstrated the least amount of artifact and was the only condition that allowed for diagnostic imaging of the ipsilateral temporal bone on DWI. The Bonebridge produced a large area of artifact and distortion with the involvement of the ipsilateral and contralateral temporal bones. CONCLUSION: In summary, of the three device conditions (Osia with magnet, Osia without magnet, and Bonebridge), Osia without magnet offered the least amount of artifact and distortion and was the only condition in which diagnostic DWI was available for the middle ear and mastoid regions on the Siemens MR imaging scanner.

Photon Counting Versus Energy-integrated Detector CT in Detection of Superior Semicircular Canal Dehiscence.

BACKGROUND: Superior semicircular canal dehiscence (SSCD), an osseous defect overlying the SSC, is associated with a constellation of audiovestibular symptoms. This study sought to compare conventional energy-integrated detector (EID) computed tomography (CT) to photon-counting detector (PCD)-CT in the detection of SSCD. MATERIAL AND METHODS: Included patients were prospectively recruited to undergo a temporal bone CT on both EID-CT and PCD-CT scanners. Two blinded neuroradiologists reviewed both sets of images for 1) the presence or absence of SSCD (graded as present, absent, or indeterminate), and 2) the width of the bone overlying the SSC (if present). Any discrepancies in the presence or absence of SSCD were agreed upon by consensus. RESULTS: In the study 31 patients were evaluated, for a total of 60 individual temporal bones (2 were excluded). Regarding SSCD presence or absence, there was substantial agreement between EID-CT and PCD-CT (k = 0.76; 95% confidence interval, CI 0.54-0.97); however, SSCD was present in only 9 (15.0%) temporal bones on PCD-CT, while EID-CT examinations were interpreted as being positive in 14 (23.3%) temporal bones. This yielded a false positive rate of 8.3% on EID-CT. The bone overlying the SSC was thinner on EID-CT images (0.66 mm; SD = 0.64) than on PCD-CT images (0.72 mm; SD = 0.66) (p < 0.001). CONCLUSION: The EID-CT examinations tend to overcall the presence of SSCD compared to PCD-CT and also underestimate the thickness of bone overlying the SSC.

A Single-Institution Case Series of Total Endovascular Relining for Type 3 Endoleaks in Traditional Endovascular Aneurysm Repair (EVAR) Grafts with Raised Bifurcations.

BACKGROUND: The endovascular repair of infrarenal abdominal aortic aneurysms can be performed with a wide variety of devices. Many of these grafts elevate the aortic bifurcation which can limit future repairs if the graft material fails thereby creating a type III endoleak to aorto-uniliac grafts. Many manufacturers have grafts susceptible to this, but we have seen this in the Medtronic AneuRx graft. Our goal is to provide technical details and outcomes regarding a novel technique to reline these grafts while maintaining inline flow to the iliac arteries. METHODS: This was a single-institution review of patients who had endoleaks requiring intervention after a previously placed graft with an elevated aortic bifurcation. Primary outcomes included technical success defined as placement of all planned devices, resolution of type III endoleak, aneurysm size at follow-up, and requirement of reintervention. Secondary outcomes included 30-day complications, aneurysm-related mortality, and all-cause mortality. Technical details of the operation include back-table deployment of an Ovation device, modification of the deployment system tether and pre-emptive placement of an up and over 0.014″ wire. The wire is placed up and over and hung outside the contralateral gate. Once the main body is introduced above the old graft, the 0.014" is snared from the contralateral side and externalized. The main body is then able to be seated at the bifurcation as the limb is not fully deployed and then device deployment is completed per instructions for use. RESULTS: Our study consists of 4 individuals, 3 of which had an abdominal aortic aneurysm initially managed with an AneuRx endovascular aneurysm repair and 1 with a combination of Gore and Cook grafts. All 4 patients were male with an average age of 84.5 years at time of reline. All patients had at least 10 years between initial surgery and reline at our institution. Primary outcomes revealed no type 1 or 3 endoleaks at follow-up, technical success was 100% and 1 patient required reintervention for aneurysm growth and type 2 endoleak. In terms of our secondary outcomes, there was 1 postoperative complication which was cardiac dysfunction secondary to demand ischemia, aneurysm-related mortality was 0% and all-cause mortality was 25% at average follow-up of 2.44 years. CONCLUSIONS: As individuals continue to age, there are more patients who would benefit from less invasive reinterventions following endovascular aneurysm repair. Whether this is due to aortic degeneration, stent migration, or stent material damage is not always known. In this study, we present an endovascular approach to treating type III endoleak patients with a previous graft and elevated aortic bifurcation using Ovation stent grafts and found no evidence of type 1 or 3 endoleaks on follow-up imaging. This approach may allow patients with type III endoleak the option of a minimally invasive, percutaneous approach where they previously would not have had one.

Third window lesions of the inner ear: A pictorial review.

PURPOSE: Radiographic review of pathologies that associate with third window syndrome. METHODS: Case series and literature review. RESULTS: Eight unique third window conditions are described and illustrated, including superior, lateral, and posterior semicircular canal dehiscence; carotid-cochlear, facial-cochlear, and internal auditory canal-cochlear dehiscence, labyrinthine erosion from endolymphatic sac tumor, and enlarged vestibular aqueduct. CONCLUSION: The present study highlights the characteristic imaging features and symptoms to differentiate third window pathologies for expedient diagnosis and management planning.

Electronic properties and optical spectra of donor-acceptor conjugated organic polymers.

Organic semiconductors based on conjugated donor-acceptor (D-A) polymers are a unique platform for electronic, spintronic, and energy-harvesting devices. Understanding the electronic structure of D-A polymers with a small band gap is essential for developing next-generation technologies. Here, we investigate the electronic structure and optical spectra of cyclopentadithiophene-based closed/open-shell D-A polymers using density functional theory and the Bethe-Salpeter equation based on G[Formula: see text]W[Formula: see text] approximation. We explored the role of different acceptor units and chemical substitutions on the structural changes and, more importantly, electronic, optical, and dielectric behavior. We found that the computed first exciton peak of the polymers agreed well with the available experimentally measured optical gap. Furthermore, D-A polymers with open-shell character display higher dielectric constant than the closed-shell polymers. We show that the exceptional performance of polycyclopentadithiophene-thiophenylthiadiazoloquinoxaline (PCPDT-TTQ) as a scalable n-type material for Faradaic supercapacitors can be partly ascribed to its elevated dielectric constant. Consequently, these D-A polymers, characterized by their high dielectric constants, exhibit significant potential for various applications, including energy storage, organic electronics, and the production of dielectric films.

Field evaluation of semi-automated moisture estimation from geophysics using machine learning.

Geophysical methods can provide three-dimensional (3D), spatially continuous estimates of soil moisture. However, point-to-point comparisons of geophysical properties to measure soil moisture data are frequently unsatisfactory, resulting in geophysics being used for qualitative purposes only. This is because (1) geophysics requires models that relate geophysical signals to soil moisture, (2) geophysical methods have potential uncertainties resulting from smoothing and artifacts introduced from processing and inversion, and (3) results from multiple geophysical methods are not easily combined within a single soil moisture estimation framework. To investigate these potential limitations, an irrigation experiment was performed wherein soil moisture was monitored through time, and several surface geophysical datasets indirectly sensitive to soil moisture were collected before and after irrigation: ground penetrating radar, electrical resistivity tomography (ERT), and frequency domain electromagnetics (FDEM). Data were exported in both raw and processed form, and then snapped to a common 3D grid to facilitate moisture prediction by standard calibration techniques, multivariate regression, and machine learning. A combination of inverted ERT data, raw FDEM, and inverted FDEM data was most informative for predicting soil moisture using a random regression forest model (one-thousand 60/40 training/test cross-validation folds produced root mean squared errors ranging from 0.025-0.046 cm3/cm3). This cross-validated model was further supported by a separate evaluation using a test set from a physically separate portion of the study area. Machine learning was conducive to a semi-automated model-selection process that could be used for other sites and datasets to locally improve accuracy.

Upper Extremity Access Has Worse Outcomes in F/BEVAR Using the VQI Dataset.

BACKGROUND: Physician-modified endografts and custom-manufactured devices use branched and fenestrated techniques (F/BEVAR) to repair complex aneurysms. Traditionally, many of these are deployed through a combination of upper and lower extremity access. However, with newer steerable sheaths, you can now simulate upper extremity (UEM) access from a transfemoral approach. Single-institution studies have demonstrated increased risks of access site complications and stroke when UEM access is used. This study compares outcomes after F/BEVAR in a national database between total transfemoral (TTF) access and mixed UEM access. METHODS: This study is an analysis of the Vascular Quality Initiative for all patients who underwent F/BEVAR from 2014 to 2021. Patients were stratified based on a TTF delivery of all devices versus any UEM access for deployment of target vessel stents. Primary outcomes included stroke, myocardial infarction (MI), and perioperative death. Secondary outcomes included access site hematoma, occlusion or embolization, operative time, fluoroscopy time, and technical success. Multivariable linear and logistic regression analyses were performed. RESULTS: Three thousand one hundred forty six patients underwent an F/BEVAR: 2,309 (73.4%) TTF and 837 (26.6%) UEM. Logistic regression analysis indicated a two-fold increased risk of death and MI and a three-fold increased risk of stroke in the UEM group. Furthermore, there is decreased operative time (221 vs. 297 min, P < 0.001) and fluoroscopy time (62 vs. 80 min, P < 0.001) in the TTF group and no difference in technical success between groups (96% vs. 97%, P = 0.159). Finally, there was a decrease in access site hematoma 2.54% vs. 4.31% (P = 0.013), access site occlusion 0.61% vs. 1.91% (P = 0.001), and extremity embolization 2.17% vs. 3.58% (P = 0.026) in the TTF versus UEM group. CONCLUSIONS: This study using Vascular Quality Initiative data demonstrates that patients who undergo an F/BEVAR using UEM access have an increased risk of perioperative MI, death, and stroke compared to TTF access.

Peritumoral Signal on Postcontrast FLAIR Images: Description and Proposed Biomechanism in Vestibular Schwannomas.

BACKGROUND AND PURPOSE: Anecdotally, postcontrast FLAIR images of vestibular schwannomas can show peritumoral hyperintense signal, hypothesized to represent gadolinium extravasation. This study assessed the incidence of this phenomenon in a cohort of patients with treatment-naïve sporadic vestibular schwannomas. MATERIALS AND METHODS: A retrospective review of 20 patients with presumed vestibular schwannoma based on characteristic imaging findings and with dedicated internal auditory canal imaging, including postcontrast T1W1 and postcontrast FLAIR, was performed. Tumor size and location were recorded, as was the presence or absence of a fundal fluid cleft. Images were reviewed for the presence of peritumoral hyperintense signal on FLAIR images (a "halo") and for both subjectively and objectively elevated signal in the ipsilateral cochlea and fundus. RESULTS: Patients were randomly selected from an institutional vestibular schwannoma registry. Eleven (55.0%) were women. A peritumoral halo was present in 90% of patients, averaging 1.0 (SD, 0.2) mm in thickness. The maximum mean FLAIR signal in the ipsilateral fundus (205.9 [SD, 110.2]) was significantly greater than on the contralateral side (121.6 [SD, 27.8]) (P = .02). Maximum mean ipsilateral intracochlear signal (167.8 [SD, 104.5]) was also significantly greater than on the contralateral side (113.4 [SD, 40.1]) (P = .04). CONCLUSIONS: A peritumoral halo on postcontrast FLAIR images was present in 90% of our cohort with randomly selected, treatment-naïve sporadic vestibular schwannomas. Although its mechanism is unknown, this signal is hypothesized to represent gadolinium extravasation, given an ipsilateral increased signal in the adjacent internal auditory canal fundus and cochlea.

Exome sequencing findings in children with annular pancreas.

BACKGROUND: Annular pancreas (AP) is a congenital defect of unknown cause in which the pancreas encircles the duodenum. Theories include abnormal migration and rotation of the ventral bud, persistence of ectopic pancreatic tissue, and inappropriate fusion of the ventral and dorsal buds before rotation. The few reported familial cases suggest a genetic contribution. METHODS: We conducted exome sequencing in 115 affected infants from the California birth defects registry. RESULTS: Seven cases had a single heterozygous missense variant in IQGAP1, five of them with CADD scores >20; seven other infants had a single heterozygous missense variant in NRCAM, five of them with CADD scores >20. We also looked at genes previously associated with AP and found two rare heterozygous missense variants, one each in PDX1 and FOXF1. CONCLUSION: IQGAP1 and NRCAM are crucial in cell polarization and migration. Mutations result in decreased motility which could possibly cause the ventral bud to not migrate normally. To our knowledge, this is the first study reporting a possible association for IQGAP1 and NRCAM with AP. Our findings of rare genetic variants involved in cell migration in 15% of our population raise the possibility that AP may be related to abnormal cell migration.

Predicted leukocyte telomere length and risk of myeloid neoplasms.

Maintenance of telomere length has long been established to play a role in the biology of cancer and several studies suggest that it may be especially important in myeloid malignancies. To overcome potential bias in confounding and reverse causation of observational studies, we use both a polygenic risk score (PRS) and inverse-variance weighted (IVW) Mendelian randomization (MR) analyses to estimate the relationship between genetically predicted leukocyte telomere length (LTL) and acute myeloid leukemia (AML) risk in 498 cases and 2099 controls and myelodysplastic syndrome (MDS) risk in 610 cases and 1759 controls. Genetic instruments derived from four recent studies explaining 1.23-4.57% of telomere variability were considered. We used multivariable logistic regression to estimate odds ratios (OR, 95% confidence intervals [CI]) as the measure of association between individual single-nucleotide polymorphisms and myeloid malignancies. We observed a significant association between a PRS of longer predicted LTL and AML using three genetic instruments (OR = 4.03 per ~1200 base pair [bp] increase in LTL, 95% CI: 1.65, 9.85 using Codd et al. [Codd, V., Nelson, C.P., Albrecht, E., Mangino, M., Deelen, J., Buxton, J.L., Hottenga, J.J., Fischer, K., Esko, T., Surakka, I. et al. (2013) Identification of seven loci affecting mean telomere length and their association with disease. Nat. Genet., 45, 422-427 427e421-422.], OR = 3.48 per one-standard deviation increase in LTL, 95% CI: 1.74, 6.97 using Li et al. [Li, C., Stoma, S., Lotta, L.A., Warner, S., Albrecht, E., Allione, A., Arp, P.P., Broer, L., Buxton, J.L., Alves, A.D.S.C. et al. (2020) Genome-wide association analysis in humans links nucleotide metabolism to leukocyte telomere length. Am. J. Hum. Genet., 106, 389-404.] and OR = 2.59 per 1000 bp increase in LTL, 95% CI: 1.03, 6.52 using Taub et al. [Taub, M.A., Conomos, M.P., Keener, R., Iyer, K.R., Weinstock, J.S., Yanek, L.R., Lane, J., Miller-Fleming, T.W., Brody, J.A., Raffield, L.M. et al. (2022) Genetic determinants of telomere length from 109,122 ancestrally diverse whole-genome sequences in TOPMed. Cell Genom., 2.] genetic instruments). MR analyses further indicated an association between LTL and AML risk (PIVW ≤ 0.049) but not MDS (all PIVW ≥ 0.076). Findings suggest variation in genes relevant to telomere function and maintenance may be important in the etiology of AML but not MDS.

Magnetic Resonance Imaging in Patients with Cochlear Implants without Magnet Removal: A Radiology-Administered Protocol to Enhance Operational Efficiency and Improve Workflow.

OBJECTIVE: To describe the development, implementation, and validation of a radiology-administered protocol to obtain magnetic resonance imaging (MRI) in patients with cochlear implants and auditory brainstem implants without magnet removal. STUDY DESIGN: Retrospective review and description of novel care pathway. METHODS: A radiology-administered protocol was designed based on careful input from the radiology safety committee and neurotology. Radiology technologist training modules, consent instructions, patient educational material, clinical audits, and other safeguards were implemented, with samples provided in this report. The primary outcomes measured included instances of magnet displacement during MRI and premature termination of MRI studies secondary to pain. RESULTS: Between June 19, 2018, and October 12, 2021, 301 implanted ears underwent MRI without magnet removal, including 153 devices housing diametric MRI-conditional magnets, and 148 implants with conventional axial (i.e., nondiametric) magnets. Among cases with diametric MRI-conditional magnets, all studies were completed without magnet dislodgement or need to terminate imaging early due to pain. Among cases with conventional axial (nondiametric) magnets, 29 (19.6%) MRI studies were stopped prematurely secondary to pain or discomfort; the overall rate of this event was 9.6% (29 of 301) among the entire study cohort. In addition, 6.1% (9 of 148) experienced confirmed magnet displacement despite headwrap placement; the overall rate among all cases was 3.0% (9 of 301). Eight of these patients received successful external magnet reseating through manual pressure on the external scalp without surgery, and one required surgical replacement of the magnet in the operating room. There were no documented instances of hematoma, infection, device or magnet extrusion, internal device movement (i.e., gross receiver-stimulator migration), or device malfunction in this cohort related to MRI. CONCLUSIONS: We present the successful implementation of a radiology-administered protocol designed to streamline care for cochlear implant and auditory brainstem implant recipients who require MRI and ease clinical demands for otolaryngology providers. Examples of resources developed, including a process map, radiology training modules, consent instructions, patient educational materials, clinical audit, and other procedural safety measures are provided so interested groups may consider adapting and implementing related measures according to need.

Prevalence of intra-osseous veins and venous lakes in the posterior skull base on 3T MRI.

BACKGROUND: Intra-osseous vessels are normal anatomic structures in the calvarium and skull base. On imaging, these structures-particularly venous lakes-can mimic pathologic abnormalities. This study sought to assess the prevalence of veins and lakes in the skull base on MRI. MATERIALS AND METHODS: A retrospective review was completed of consecutive patients that underwent contrast-enhanced MRI imaging of the internal auditory canals. The clivus, jugular tubercles, and basio-occiput were assessed for the presence of both intra-osseous veins (serpentine and/or branching vessels) and venous lakes (well-circumscribed round or oval enhancing structures). Vessels in the adjacent synchondroses major foramina were excluded. Three board-certified neuroradiologists performed independent blinded reviews, with discrepancies agreed upon by consensus. RESULTS: 96 patients were included in this cohort (58.3% female). Mean age was 58.4 years (range = 19-85). At least one intra-osseous vessel was identified in 71 (74.0%) patients. 67 (70.0%) had at least one skull base vein, and 14 (14.6%) had at least one venous lake. Both vessel subtypes were observed in 8.3% of patients. Vessels were more commonly observed in women, though this did not reach statistical threshold (p = 0.56). Age was not associated with the presence of vessels (0.59) or vessel location (p values ranged from 0.44-0.84). CONCLUSIONS: Intra-osseous skull base veins and venous lakes are relatively common findings on MRI. Both vascular structures should be considered normal anatomy, and care should be taken to not confuse these for pathologic entities.

Natural history of facial paraganglioma with 2 decades of follow-up: A case report and literature review.

OBJECTIVE: To report 20 years of natural history data for a facial paraganglioma and provide a comprehensive review of the existing literature. PATIENT: 81-year-old female with a remote history of cardiac arrest while under anesthesia who elected to observe her facial paraganglioma for 20 years. INTERVENTIONS: Observation, clinical documentation, radiographic surveillance. MAIN OUTCOME MEASURES: Tumor progression, patient symptomatology, and review of management options. RESULTS: The initial presentation of the facial paraganglioma was facial spasm. Over the course of observation, symptoms progressed to include complete facial nerve paralysis, pulsatile tinnitus, and otalgia on the affected side. Radiologic surveillance demonstrated incremental growth and erosion of surrounding structures, including the posterior external auditory canal, stylomastoid foramen, and lateral semicircular canal with near-dehiscence. Twenty-four cases of facial paraganglioma were identified in the extended literature search and are summarized herein. CONCLUSIONS: This unique case contributes to the scarce literature surrounding facial paragangliomas by reporting the extended natural history of this disease.

Exploratory Study of the Association of Genetic Factors With Recovery of Adrenal Function in Cushing Disease.

Successful treatment of endogenous Cushing disease (CD) is often followed by a period of adrenal insufficiency (AI). We performed an exploratory study on genetic factors potentially involved in the hypothalamic-pituitary-adrenal (HPA) axis recovery in patients with CD after remission. We identified 90 patients who achieved remission after surgery and had a minimum of 3 months follow-up. Variants in a selected panel of genes that were rare in the general population and predicted as damaging in silico were retrieved from whole exome sequencing analysis. We did not identify any variant with significant correlation with recovery time after adjusting for multiple comparisons. On gene-specific analysis the BAG1 gene showed a correlation with shorter duration of postsurgical AI, but both patients with BAG1 variants later experienced a recurrence. After excluding patients with recurrence, no statistical association was recorded. To conclude, we did not identify a strong genetic modifier of HPA recovery in this exploratory study.