The clue is in the kidneys: greater renal contrast medium accumulation on ipsilateral side down CT myelogram after lateral decubitus digital subtraction myelogram as a predictor of laterality of cerebrospinal fluid leak.

AIM: To assess the potential correlation of the laterality of a cerebrospinal fluid (CSF)-venous fistula with the laterality of decubitus computed tomography (CT) myelogram (post decubitus digital subtraction myelogram) based on which side demonstrated more renal contrast medium excretion. MATERIALS AND METHODS: Patients with CSF-venous fistulas diagnosed at lateral decubitus digital subtraction myelograms were reviewed retrospectively. Patients who did not have CT myelogram following one or both left and right lateral decubitus digital subtraction myelograms were excluded. Two neuroradiologists independently interpreted the CT myelogram for the presence or absence of renal contrast, and whether subjectively more renal contrast medium was visualised on the left or right lateral decubitus CT myelogram. RESULTS: Renal contrast medium was seen in lateral decubitus CT myelograms in 28 of 30 (93.3%) patients with CSF-venous fistulas. Having more renal contrast medium in right lateral decubitus CT myelogram was 73.9% sensitive and 71.4% specific for the diagnosis of a right-sided CSF-venous fistula, whereas having more renal contrast medium in the left lateral decubitus CT myelogram was 71.4% sensitive and 82.6% specific for a left-sided CSF-venous fistula (p=0.02). CONCLUSION: When the CSF-venous fistula lies on the dependent side of a decubitus CT myelogram performed after decubitus digital subtraction myelogram, relatively more renal contrast medium is visualised compared to when the fistula lies on the non-dependent side.

Fluoroscopically guided transforaminal epidural steroid injections at a quaternary-care teaching institution: effect of trainee involvement and patient body mass index on fluoroscopy time and patient dose.

AIM: To investigate whether there are differences in fluoroscopy time and patient dose for fluoroscopically guided lumbar transforaminal epidural steroid injections (TFESIs) performed by staff radiologists versus with trainees and to evaluate the effect of patient body mass index (BMI) on fluoroscopy time and patient dose, including their interactions with other variables. MATERIALS AND METHODS: Single-level lumbar TFESIs (n=1844) between 1 January 2011 and 31 December 2013 were reviewed. Fluoroscopy time, reference point air kerma (Ka,r), and kerma area product (KAP) were recorded. BMI and trainee involvement were examined as predictors of fluoroscopy time, Ka,r, and KAP in models adjusted for age and gender in multivariable linear models. Stratified models of BMI groups by trainee presence were performed. RESULTS: Increased age was the only significant predictor of increased fluoroscopy time (p<0.0001). Ka,r and KAP were significantly higher in patients with a higher BMI (p<0.0001 and p=0.0009). When stratified by BMI, longer fluoroscopy time predicted increased Ka,r and KAP in all groups (p<0.0001). Trainee involvement was not a statistically significant predictor of fluoroscopy time or Ka,r in any BMI category. KAP was lower with trainees in the overweight group (p=0.0009) and higher in male patients for all BMI categories (p<0.02). CONCLUSION: Trainee involvement did not result in increased fluoroscopy time or patient dose. BMI did not affect fluoroscopy time; however, overweight and obese patients received significantly higher Ka,r and KAP. Male patients received a higher KAP in all BMI categories. Limiting fluoroscopy time and good collimation practices should be reinforced in these patients.

Increases in plasma sheet temperature with solar wind driving during substorm growth phases.

During substorm growth phases, magnetic reconnection at the magnetopause extracts ∼1015 J from the solar wind which is then stored in the magnetotail lobes. Plasma sheet pressure increases to balance magnetic flux density increases in the lobes. Here we examine plasma sheet pressure, density, and temperature during substorm growth phases using 9 years of Cluster data (>316,000 data points). We show that plasma sheet pressure and temperature are higher during growth phases with higher solar wind driving, whereas the density is approximately constant. We also show a weak correlation between plasma sheet temperature before onset and the minimum SuperMAG AL (SML) auroral index in the subsequent substorm. We discuss how energization of the plasma sheet before onset may result from thermodynamically adiabatic processes; how hotter plasma sheets may result in magnetotail instabilities, and how this relates to the onset and size of the subsequent substorm expansion phase.

Enhancement in the Round Window Niche: A Potential Pitfall in High-Resolution MR Imaging of the Internal Auditory Canal.

BACKGROUND AND PURPOSE: There is limited discussion in current literature about the normal imaging appearance of the round window. The purpose of this study was to assess the prevalence and imaging characteristics of gadolinium enhancement in the round window niche on MR imaging to the internal auditory canal. MATERIALS AND METHODS: The presence or absence and laterality of enhancement in the round window niche on MR imaging was retrospectively reviewed in 95 patients from 1 institution. All studies included high-resolution (≤0.5-mm section thickness) pre- and postgadolinium 3D FSE T1 with fat-saturation and postgadolinium 3D FLAIR image sequences. T1 and T2 acquisitions were viewed as coregistered overlays to confirm that enhancement was lateral to the round window membrane within the round window niche. CT was reviewed when available to assess the presence and laterality of soft tissue in the round window niche. RESULTS: Ninety-five patients with internal auditory canal MRIs were included. Enhancement was present in the round window of 15 of 95 patients (15.8%). Of the 27 patients who underwent CT, 4 (14.8%) had concordant soft tissue on CT and MR imaging enhancement in the round window niche. One patient had MR imaging enhancement within the round window niche without a corresponding abnormality on CT. The absence of soft tissue on CT and the corresponding lack of MR imaging enhancement were present in 22 (81.5%) patients. CONCLUSIONS: Enhancement can be visualized within the round window niche on MR imaging as an incidental finding. This enhancement probably represents postinflammatory granulation tissue and does not require further intervention. However, the potential for this enhancement to be misdiagnosed as a pathologic process can be a pitfall in MR imaging.

The Monro-Kellie Doctrine: A Review and Call for Revision.

The Monro-Kellie doctrine is a well-accepted principle of intracranial hemodynamics. It has undergone few consequential revisions since it was established. Its principle is straightforward: The combined volume of neuronal tissue, blood, and CSF is constant. To maintain homeostatic intracranial pressure, any increase or decrease in one of these elements leads to a reciprocal and opposite change in the others. The Monro-Kellie doctrine assumes a rigid, unadaptable calvaria. Recent studies have disproven this assumption. The skull expands and grows in response to pathologic changes in intracranial pressure. In this review, we outline what is known about calvarial changes in the setting of pressure dysregulation and suggest a revision to the Monro-Kellie doctrine that includes an adaptable skull as a fourth component.

Efficacy of Epidural Blood Patching or Surgery in Spontaneous Intracranial Hypotension: A Systematic Review and Evidence Map.

BACKGROUND: Spontaneous intracranial hypotension is an important cause of treatable secondary headaches. Evidence on the efficacy of epidural blood patching and surgery for spontaneous intracranial hypotension has not been synthesized. PURPOSE: Our aim was to identify evidence clusters and knowledge gaps in the efficacy of treatments for spontaneous intracranial hypotension to prioritize future research. DATA SOURCES: We searched published English language articles on MEDLINE (Ovid), the Web of Science (Clarivate), and EMBASE (Elsevier) from inception until October 29, 2021. STUDY SELECTION: We reviewed experimental, observational, and systematic review studies assessing the efficacy of epidural blood patching or surgery in spontaneous intracranial hypotension. DATA ANALYSIS: One author performed data extraction, and a second verified it. Disagreements were resolved by consensus or adjudicated by a third author. DATA SYNTHESIS: One hundred thirty-nine studies were included (median, 14 participants; range, 3-298 participants). Most articles were published in the past decade. Most assessed epidural blood patching outcomes. No studies met level 1 evidence. Most were retrospective cohort or case series (92.1%, n = 128). A few compared the efficacy of different treatments (10.8%, n = 15). Most used objective methods for the diagnosis of spontaneous intracranial hypotension (62.3%, n = 86); however, 37.7% (n = 52) did not clearly meet the International Classification of Headache Disorders-3 criteria. CSF leak type was unclear in 77.7% (n = 108). Nearly all reported patient symptoms using unvalidated measures (84.9%, n = 118). Outcomes were rarely collected at uniform prespecified time points. LIMITATIONS: The investigation did not include transvenous embolization of CSF-to-venous fistulas. CONCLUSIONS: Evidence gaps demonstrate a need for prospective study designs, clinical trials, and comparative studies. We recommend using the International Classification of Headache Disorders-3 diagnostic criteria, explicit reporting of CSF leak subtype, inclusion of key procedural details, and using objective validated outcome measures collected at uniform time points.

Fluoroscopy- and CT-Guided Gold Fiducial Marker Placement for Intraoperative Localization during Spinal Surgery: Review of 179 Cases at a Single Institution-Technique and Safety Profile.

BACKGROUND AND PURPOSE: Wrong-level spinal surgery, especially in the thoracic spine, remains a challenge for a variety of reasons related to visualization, such as osteopenia, large body habitus, severe kyphosis, radiographic misinterpretation, or anatomic variation. Preoperative fiducial marker placement performed in a dedicated imaging suite has been proposed to facilitate identification of thoracic spine vertebral levels. In this current study, we report our experience using image-guided percutaneous gold fiducial marker placement to enhance the accuracy and safety of thoracic spinal surgical procedures. MATERIALS AND METHODS: A retrospective review was performed of all fluoroscopy- or CT-guided gold fiducial markers placed at our institution between January 3, 2019, and March 16, 2022. A chart review of 179 patients was performed detailing the procedural approach and clinical information. In addition, the method of gold fiducial marker placement (fluoroscopy/CT), procedure duration, spinal level of the gold fiducial marker, radiation dose, fluoroscopy time, surgery date, and complications (including whether wrong-level surgery occurred) were recorded. RESULTS: A total of 179 patients (104 female) underwent gold fiducial marker placement. The mean age was 57 years (range, 12-96 years). Fiducial marker placement was performed by 13 different neuroradiologists. All placements were technically successful without complications. All 179 (100%) operations were performed at the correct level. Most fiducial markers (143) were placed with fluoroscopy with the most common location at T6-T8. The most common location for placement in CT was at T3 and T4. CONCLUSIONS: All operations guided with gold fiducial markers were performed at the correct level. There were no complications of fiducial marker placement.

Normal Enhancement within the Vestibular Aqueduct: An Anatomic Review with High-Resolution MRI.

BACKGROUND AND PURPOSE: The normal appearance of the vestibular aqueduct on postcontrast MR images has not been adequately described in the literature. This study set out to characterize the expected appearance of the vestibular aqueduct, with particular emphasis on the enhancement of the structure on both 3D FSE T1 and 3D-FLAIR sequences. MATERIALS AND METHODS: All MR imaging examinations of the internal auditory canals performed between March 1, 2021, and May 20, 2021, were retrospectively reviewed. All studies included high-resolution (≤0.5-mm section thickness) pre- and postgadolinium 3D FSE T1 with fat-saturated and postgadolinium 3D-FLAIR sequences. Two neuroradiologists independently reviewed the MR images of the vestibular aqueduct for the presence or absence of enhancement on both T1 and FLAIR images and compared the relative intensity of enhancement between sequences. The presence or absence of an enlarged vestibular aqueduct was also noted. RESULTS: Ninety-five patients made up the patient cohort, of whom 5 did not have postcontrast FLAIR images available (50 women [55.6%]). On both sides, enhancement was significantly more commonly seen on postgadolinium FLAIR (76/180, 42.2%) than on T1 fat-saturated images (41/190, 21.6%) (P < .001). The intensity of enhancement was significantly greater on postgadolinium FLAIR images than on T1 fat-saturated images (38.9% versus 3.7%, respectively; P < .001). CONCLUSIONS: Enhancement within the vestibular aqueduct is an expected finding on MR imaging and is both more common and more intense on postgadolinium 3D-FLAIR than on T1 fat-saturated sequences. Such enhancement should not be confused with pathology on MR imaging unless other suspicious findings are present.

Utility of Dual-Energy CT to Improve Diagnosis of CSF Leaks on CT Myelography following Lateral Decubitus Digital Subtraction Myelography with Negative Findings.

CSF leaks, including CSF-venous fistulas, which cause spontaneous intracranial hypotension, remain difficult to diagnose, even on digital subtraction myelography and CT myelography. Dual-energy CT technology has been used to improve diagnostic utility within multiple organ systems. The capability of dual-energy CT to create virtual monoenergetic images can be leveraged to increase conspicuity of contrast in CSF-venous fistulas and direct epidural CSF leakage to improve the diagnostic utility of CT myelography. Six cases (in 5 patients) are shown in which virtual monoenergetic images demonstrate a leak location that was either occult or poorly visible on high- or low-kilovolt series. This clinical report describes the novel application of dual-energy CT for the detection of subtle CSF leaks including CSF-venous fistulas.

Diffuse Calvarial Hyperostosis and Spontaneous Intracranial Hypotension: A Case-Control Study.

BACKGROUND AND PURPOSE: Diagnosing spontaneous intracranial hypotension and associated CSF leaks can be challenging, and additional supportive imaging findings would be useful to direct further evaluation. This retrospective study evaluated whether there was a difference in the prevalence of calvarial hyperostosis in a cohort of patients with spontaneous intracranial hypotension compared with an age- and sex-matched control population. MATERIALS AND METHODS: Cross-sectional imaging (CT of the head or brain MR imaging examinations) for 166 patients with spontaneous intracranial hypotension and 321 matched controls was assessed by neuroradiologists blinded to the patient's clinical status. The readers qualitatively evaluated the presence of diffuse or layered calvarial hyperostosis and measured calvarial thickness in the axial and coronal planes. RESULTS: A significant difference in the frequency of layered hyperostosis (31.9%, 53/166 subjects versus 5.0%, 16/321 controls, P < .001, OR = 11.58) as well as the frequency of overall (layered and diffuse) hyperostosis (38.6%, 64/166 subjects versus 13.2%, 42/321 controls, P < .001, OR = 4.66) was observed between groups. There was no significant difference in the frequency of diffuse hyperostosis between groups (6.6%, 11/166 subjects versus 8.2%, 26/321 controls, P = .465). A significant difference was also found between groups for calvarial thickness measured in the axial (P < .001) and coronal (P < .001) planes. CONCLUSIONS: Layered calvarial hyperostosis is more prevalent in spontaneous intracranial hypotension compared with the general population and can be used as an additional noninvasive brain imaging marker of spontaneous intracranial hypotension and an underlying spinal CSF leak.

Percutaneous CT-Guided Core Needle Biopsies of Head and Neck Masses: Review of 184 Cases at a Single Academic Institution, Common and Special Techniques, Diagnostic Yield, and Safety.

BACKGROUND AND PURPOSE: Percutaneous CT-guided core needle biopsies of head and neck lesions can be safely performed with vigilant planning. This largest-to-date single-center retrospective study evaluates multiple approaches with consideration of special techniques and examines the histopathologic yield. MATERIALS AND METHODS: Retrospective review of CT-guided core biopsies of head and neck lesions from January 1, 2010, to October 30, 2020, was performed. We recorded the following: patient demographics, sedation details, biopsy needle type and size, lesion location and size, approach, patient positioning, preprocedural intravenous contrast, proceduralists' years of experience, complications, and pathology results. RESULTS: One hundred eighty-four CT-guided core biopsies were evaluated. The initial diagnostic yield was 93% (171/184). However, of 43/184 (23%) originally "negative for malignancy" biopsies, 4 were eventually positive for malignancy via rebiopsy/excision, resulting in a 2% false-negative rate and an adjusted total diagnostic yield of 167/184 (91%). Biopsies were performed by 16 neuroradiologists with variable experience. The diagnostic yield was essentially the same: 91% (64/70) for proceduralists with ≤3 years' experience, and 90% (103/114) with >3 years' experience. The diagnostic yield was 93% (155/166) for lesions of >10 mm. The diagnostic yield per biopsy needle gauge was the following: 20 ga, 81% (13/16); 18 ga, 93% (70/75); 16 ga, 90% (64/71); and 14 ga, 91% (20/22). There were 4 asymptomatic hematomas, with none requiring intervention. CONCLUSIONS: Percutaneous CT-guided core needle biopsies are safe procedures for superficial and deep head and neck lesions with a high diagnostic yield. Careful planning and special techniques may increase the number of lesions accessible percutaneously while minimizing the risk of complications.

Safety of Consecutive Bilateral Decubitus Digital Subtraction Myelography in Patients with Spontaneous Intracranial Hypotension and Occult CSF Leak.

BACKGROUND AND PURPOSE: Digital subtraction myelography performed with the patient in the lateral decubitus position has the potential for increased sensitivity over prone-position myelography in the detection of spinal CSF-venous fistulas, a well-established cause of spontaneous intracranial hypotension. We report on the safety of performing routine, consecutive-day right and left lateral decubitus digital subtraction myelography in these patients. MATERIALS AND METHODS: In this retrospective case series, all patients undergoing consecutive-day lateral decubitus digital subtraction myelography for suspected spinal CSF leak between September 2018 and September 2019 were identified. Chart review was performed to identify any immediate or delayed adverse effects associated with the procedures. Procedural parameters were also analyzed due to inherent variations associated with the positive-pressure myelography technique that was used. RESULTS: A total of 60 patients underwent 68 pairs of consecutive-day lateral decubitus digital subtraction myelographic examinations during the study period. No major adverse effects were recorded. Various minor adverse effects were observed, including pain requiring analgesics (27.2%), nausea/vomiting requiring antiemetics (8.1%), and transient neurologic effects such as syncope, vertigo, altered mental status, and autonomic dysfunction (5.1%). Minor transient neurologic effects were correlated with increasing volumes of intrathecal saline injectate used for thecal sac prepressurization. CONCLUSIONS: In patients with spontaneous intracranial hypotension and suspected spontaneous spinal CSF leak, consecutive-day lateral decubitus digital subtraction myelography demonstrates an acceptable risk profile without evidence of neurotoxic effects from cumulative intrathecal contrast doses. Higher intrathecal saline injectate volumes may correlate with an increased incidence of minor transient periprocedural neurologic effects.

Spontaneous Intracranial Hypotension: Atypical Radiologic Appearances, Imaging Mimickers, and Clinical Look-Alikes.

Spontaneous intracranial hypotension is a condition characterized by low CSF volume secondary to leakage through a dural defect with no identifiable cause. Patients classically present with orthostatic headaches, but this symptom is not specific to spontaneous intracranial hypotension, and initial misdiagnosis is common. The most prominent features of spontaneous intracranial hypotension on intracranial MR imaging include "brain sag" and diffuse pachymeningeal enhancement, but these characteristics can be seen in several other conditions. Understanding the clinical and imaging features of spontaneous intracranial hypotension and its mimickers will lead to more prompt and accurate diagnoses. Here we discuss conditions that mimic the radiologic and clinical presentation of spontaneous intracranial hypotension as well as other disorders that CSF leaks can imitate.

Imaging Review of Paraneoplastic Neurologic Syndromes.

Paraneoplastic syndromes are systemic reactions to neoplasms mediated by immunologic or hormonal mechanisms. The most well-recognized paraneoplastic neurologic syndrome, both clinically and on imaging, is limbic encephalitis. However, numerous additional clinically described syndromes affect the brain, spinal cord, and peripheral nerves. Many of these syndromes can have imaging findings that, though less well described, are important in making the correct diagnosis. Moreover, imaging in these syndromes frequently mimics more common pathology, which can be a diagnostic challenge for radiologists. Our goal is to review the imaging findings of paraneoplastic neurologic syndromes, including less well-known entities and atypical presentations of common entities. Specifically, we discuss limbic encephalitis, paraneoplastic cerebellar degeneration, paraneoplastic brain stem encephalitis, cranial neuropathy, myelitis, and polyneuropathy. We also demonstrate common diagnostic pitfalls that can be encountered when imaging these patients.

Time to Resolution of Inadvertent Subdural Contrast Injection during a Myelogram: When Can the Study Be Reattempted?

BACKGROUND AND PURPOSE: Inadvertent subdural contrast injections can occur during any myelogram. Currently, there are no guidelines defining when residual subdural iodinated contrast will be cleared and no longer interfere with subsequent procedure and imaging. We investigated the time to resolution of subdural contrast using a 2-day lateral decubitus digital subtraction myelogram and associated CT myelogram data in patients undergoing evaluation for spontaneous intracranial hypotension. MATERIALS AND METHODS: Retrospective review of 63 patients with lateral decubitus digital subtraction myelograms from September 4, 2018, to October 1, 2019, was performed. Patients with 2-day lateral decubitus digital subtraction myelograms on 2 consecutive days, with or without a same-day CT myelogram on day 1 and with a same-day CT myelogram on day 2, were included. Patients with next-day CT covering at least the abdomen and pelvis after either-day injection were also included. In cases of subdural injection, next-day CT scans were evaluated for residual subdural contrast. RESULTS: Of 49 included patients, 5 had subdural injection on day 1, with the second-day CT myelogram available for review. One of these 5 patients had subdural injections on 2 different days and subsequently had chest/abdomen/pelvis CTA a day after the second subdural injection. In all 6 cases of subdural injections, there was complete resolution of subdural contrast on the next-day CT, with the shortest time to resolution of approximately 20.5 hours (range, 20.5-28.5 hours). CONCLUSIONS: Our study suggests that resolution of inadvertently injected subdural contrast occurs within 1 day, and the myelogram can be reattempted as early as the next day.

Lateral Decubitus Digital Subtraction Myelography: Tips, Tricks, and Pitfalls.

Digital subtraction myelography is a valuable diagnostic technique to detect the exact location of CSF leaks in the spine to facilitate appropriate diagnosis and treatment of spontaneous spinal CSF leaks. Digital subtraction myelography is an excellent diagnostic tool for assessment of various types of CSF leaks, and lateral decubitus digital subtraction myelography is increasingly being used to diagnose CSF-venous fistulas. Lateral decubitus digital subtraction myelography differs from typical CT and fluoroscopy-guided myelograms in many ways, including equipment, supplies, and injection and image-acquisition techniques. Operators should be familiar with techniques, common pitfalls, and artifacts to improve diagnostic yield and prevent nondiagnostic examinations.

Ordering the final events in yeast exocytosis.

In yeast, assembly of exocytic soluble N-ethylmaleimide-sensitive fusion protein (NSF) attachment protein receptor (SNARE) complexes between the secretory vesicle SNARE Sncp and the plasma membrane SNAREs Ssop and Sec9p occurs at a late stage of the exocytic reaction. Mutations that block either secretory vesicle delivery or tethering prevent SNARE complex assembly and the localization of Sec1p, a SNARE complex binding protein, to sites of secretion. By contrast, wild-type levels of SNARE complexes persist in the sec1-1 mutant after a secretory block is imposed, suggesting a role for Sec1p after SNARE complex assembly. In the sec18-1 mutant, cis-SNARE complexes containing surface-accessible Sncp accumulate in the plasma membrane. Thus, one function of Sec18p is to disassemble SNARE complexes on the postfusion membrane.

Sec1p binds to SNARE complexes and concentrates at sites of secretion.

Proteins of the Sec1 family have been shown to interact with target-membrane t-SNAREs that are homologous to the neuronal protein syntaxin. We demonstrate that yeast Sec1p coprecipitates not only the syntaxin homologue Ssop, but also the other two exocytic SNAREs (Sec9p and Sncp) in amounts and in proportions characteristic of SNARE complexes in yeast lysates. The interaction between Sec1p and Ssop is limited by the abundance of SNARE complexes present in sec mutants that are defective in either SNARE complex assembly or disassembly. Furthermore, the localization of green fluorescent protein (GFP)-tagged Sec1p coincides with sites of vesicle docking and fusion where SNARE complexes are believed to assemble and function. The proposal that SNARE complexes act as receptors for Sec1p is supported by the mislocalization of GFP-Sec1p in a mutant defective for SNARE complex assembly and by the robust localization of GFP-Sec1p in a mutant that fails to disassemble SNARE complexes. The results presented here place yeast Sec1p at the core of the exocytic fusion machinery, bound to SNARE complexes and localized to sites of secretion.

Imaging Findings Related to the Valsalva Maneuver in Head and Neck Radiology.

Forced exhalation against a closed glottis, known as the Valsalva maneuver, is an important clinical diagnostic and therapeutic tool due to its physiologic effects. Several unique conditions and anatomic changes can occur with repetitive or acute changes in pressure from the Valsalva maneuver. We will discuss and review various pertinent head and neck imaging cases with findings resulting from induced pressure gradients, including the Valsalva maneuver. Additionally, we will demonstrate the diagnostic utility of the Valsalva maneuver in head and neck radiology.

Carotid Artery Tortuosity Is Associated with Connective Tissue Diseases.

BACKGROUND AND PURPOSE: There is a general assumption in the cerebrovascular literature that there is an association between carotid artery tortuosity and connective tissues disease; however, this has not been firmly established. The purpose of this study was to determine the prevalence of carotid artery tortuosity in patients with connective tissue diseases relative to matched controls. MATERIALS AND METHODS: Patients with previous CTA or MRA and a diagnosis of connective tissue diseases were identified and compared with a cohort of age-matched controls. Radiologists blinded to the diagnosis reviewed the images and evaluated the presence of carotid artery tortuosity (including loops, kinks, or coils). Continuous variables were compared using the Student t test, and categoric variables with χ2 tests. RESULTS: One hundred forty-three patients with connective tissue disease and 143 controls were included in this study. Specific diagnoses included Marfan (n = 33), nonvascular Ehlers-Danlos (n = 36), Ehlers-Danlos vascular-type (n = 32), neurofibromatosis type 1 (n = 26), and Loeys-Dietz (n = 16) syndromes. The presence of carotid tortuosity was 44% in connective tissue disease and 16% in controls (P < .001). Of tortuosity manifestations, coils were most prevalent (23% versus 3%; P < .001). Among the various connective tissue diseases, the rates of any carotid tortuosity were 88% for Marfan syndrome, 63% for Loeys-Dietz syndrome, 42% for neurofibromatosis type 1, and 19% for both vascular- and nonvascular-type Ehlers-Danlos syndrome. The positive predictive value of the combination of aortic aneurysm and carotid tortuosity being associated with connective tissue disease was 95.4%. The specificity was 98.6%. CONCLUSIONS: Carotid artery tortuosity is highly associated with connective tissue diseases, particularly Marfan syndrome, Loeys-Dietz syndrome, and neurofibromatosis type 1. Such findings are relevant in risk assessment for vascular complications in connective tissue disease, endovascular treatment planning, and in understanding the pathomechanisms of vascular tortuosity in general.