Brinjikji, Waleed

Abstract Rationale There is sparse published literature on the causes and outcomes of hospitalization of patients with hereditary hemorrhagic telangiectasia (HHT). Objectives To evaluate rates of various complications, comorbidities, and in-hospital outcomes of patients with HHT using a large, multihospital inpatient database. Methods We identified patients with HHT in the U.S. Nationwide Inpatient Sample between 2000 and 2012. Rates of hemorrhagic, neurological, hepatic, and cardiopulmonary complications among hospitalized patients with HHT were evaluated. We also studied procedure use rates for blood transfusion, endoscopy, and epistaxis treatment. Hospitalization outcomes, including in-hospital mortality, discharge status, charges, and length of stay, were evaluated. Measurements and Main Results: We identified 10,293 patients with HHT. The mean age of the HHT population was 60.7 years. Sixty percent of patients were female. More than 75% of HHT hospitalizations occurred in those older than 50 years of age. Patients with HHT had high rates of bleeding-related complications, including anemia (53.3%), epistaxis (16.2%), and gastrointestinal bleeding (10.8%). Overall, bleeding complications accounted for 62.7% of HHT-related complications. Thirty-eight percent of hospitalized patients with HHT received one or more transfusions of a blood product. Cardiopulmonary complications were present in 41.0% of the cases. Congestive heart failure was the second most common individual complication among patients with HHT, affecting 19.9% of patients. The in-hospital mortality rate was 1.9%. Conclusions In this large, nationwide study, we found that nearly two-thirds of patients hospitalized with HHT experienced a bleeding-related complication. Nearly 40% of hospitalized patients with HHT required transfusion of blood products. Cardiopulmonary complications, including congestive heart failure, were the second most common complication. The high burden of bleeding-related complications points to a significant unmet clinical need for these patients.

Background Mycotic and oncotic aneurysms may result in devastating neurologic sequelae if undetected. The objectives of this study were to examine interobserver variability and accuracy of cross-sectional imaging for the detection of distal territory mycotic and oncotic aneurysms. Methods We searched our institutional database for all radiology reports from 2005 to 2015 with an indication or diagnosis of mycotic or oncotic aneurysm. Patients who underwent DSA and either CTA or MRA within 12 weeks of each other were identified. The cross-sectional images from each study were blinded and reviewed by two radiologists. If positive for aneurysm, location and number of aneurysms were reported. Sensitivity, specificity, positive predictive value, negative predictive value, and interobserver variability were determined for MRA and MRA/CTA. Results Twenty-five patients were included in this study. Ten (40%) harbored distal aneurysms. Cross-sectional imaging had a sensitivity of 45.5%, specificity of 90.0%, and kappa value of 0.29 (0.00–0.69) for the detection of cerebral mycotic and oncotic aneurysms. Conclusions Because of the low sensitivity and high interobserver variability of cross-sectional imaging, DSA should remain the gold standard for evaluation of suspected oncotic and mycotic aneurysms. In cases in which cross sectional imaging is negative and there is a high clinical suspicion for mycotic aneurysm, DSA should be strongly considered.

Background While clinical and angiographic risk factors for intracranial aneurysm instability are well established, it is reasonable to postulate that intra-aneurysmal hemodynamics also have a role in aneurysm instability. Objective To identify hemodynamic characteristics that differ between radiologically unstable and stable unruptured intracranial aneurysms. Materials and methods 12 pairs of unruptured intracranial aneurysms with a 3D rotational angiographic set of images and followed up longitudinally without treatment were studied. Each pair consisted of one stable aneurysm (no change on serial imaging) and one unstable aneurysm (demonstrated growth of at least 1 mm diameter or ruptured during follow-up) of matching size (within 10%) and locations. Patient-specific computational fluid dynamics models were created and run under pulsatile flow conditions. Relevant hemodynamic and geometric variables were calculated and compared between groups using the paired Wilcoxon test. Results The area of the aneurysm under low wall shear stress (low shear stress area (LSA)) was 2.26 times larger in unstable aneurysms than in stable aneurysms (p=0.0499). The mean aneurysm vorticity was smaller by a factor of 0.57 in unstable aneurysms compared with stable aneurysms (p=0.0499). No statistically significant differences in geometric variables or shape indices were found. Conclusions This pilot study suggests there may be hemodynamic differences between unstable and stable unruptured cerebral aneurysms. In particular, the area under low wall shear stress was larger in unstable aneurysms. These findings should be considered tentative until confirmed by future larger studies.

Background Although rare, spinal arteriovenous malformations (AVMs) are thought to be more prevalent in the hereditary Hereditary Hemorrhagic Telangiectasia (HHT) population. Methods We report a case of a spinal AVM in a 37-year-old female with HHT treated with endovascular embolization. In addition, we report findings from a systematic review of the literature on the clinical characteristics, angioarchitecture, and clinical outcomes of HHT patients with spinal AVMs. Results The patient is a 37 year-old female with definite HHT who presented with a one-year history of progressive gait difficulty. The spinal fistula was incidentally detected on chest computed tomography (CT). Spinal angiography demonstrated a large perimedullary arteriovenous fistula was supplied by a posterolateral spinal artery. The fistula was treated with detachable coils. The patient made a complete neurological recovery. Our systematic review yielded 25 additional cases of spinal AVMs in HHT patients. All fistulae were perimedullary (100.0%). Treatments were described in 24 of the 26 lesions. Endovascular-only treatment was performed in 16 cases (66.6%) and surgical-only treatment was performed in five cases (20.8%). Complete or near-complete occlusion rates were 86.7% (13/15) for endovascular treated cases, 100.0% (4/4) for surgery and 66.6% (2/3) for combined treatments. Overall, 80.0% of patients (16/20) reported improvement in function following treatment, 100.0% (5/5) in the surgery group and 84.6% (11/13) reported improvement in the endovascular group. Conclusions Spinal fistulae in HHT patients are usually type IV perimedullary fistulae. Both endovascular and surgical treatments appeared to be effective in treating these lesions. However, it is clear that endovascular therapy has become the preferred treatment modality.

Spinal epidural arteriovenous fistulas (SEDAVFs) are rare complex lesions often presenting with protean clinical manifestations secondary to compressive symptoms or congestive myelopathy. The imaging manifestations of SEDAVFs on MR angiography/MRI include high T2 signal in the spinal cord, vascular engorgement of the epidural space, and prominent intradural vascular flow voids. Given the complexity of these lesions, they are best characterized anatomically on catheter angiography where careful inspection of arterial feeders and venous drainage patterns can be performed. The imaging hallmark of an SEDAVF on angiography is the presence of a dilated epidural venous pouch through which spinal and paraspinal veins are secondarily opacified. In the lower thoracic and lumbar spine, SEDAVFs are usually located in the ventral epidural space and fed mainly by the anteriorly coursing epidural arteries. In the cervical and upper thoracic spine, SEDAVFs and their feeding arteries are more typically located laterally in the spinal canal. Current treatment options include transarterial or transvenous endovascular embolization with liquid embolic agents or coils, and surgical resection/disconnection of the fistula. Further research is needed to better characterize how and why these lesions form and to identify the best treatment modalities.