Splenic artery pathology presentation, operative interventions, and outcomes in 88 patients with vascular Ehlers-Danlos syndrome.

OBJECTIVE: Vascular Ehlers-Danlos syndrome (VEDS) is rare and associated with arteriopathies. The aim of this study is to investigate the presentation, operative interventions, and outcomes of splenic arterial pathology in a population of more than 1500 individuals with genetically confirmed VEDS due to pathogenic COL3A1 variants. METHODS: Cross-sectional analysis of 1547 individuals was performed. The data were assembled by harmonizing data from three overlapping cohorts with genetically confirmed VEDS: the VEDS Collaborative Natural History Study (N = 242), a single-center cohort (N = 75), and the University of Washington Collagen Diagnostic Lab cohort (N = 1231). Duplicates were identified and removed. Patients were selected for analysis if they had splenic artery aneurysm (SAA), pseudoaneurysm, dissection, thrombosis, or rupture. Demographics, COL3A1 variants, interventions, and outcomes were analyzed. Comparisons by splenic artery rupture were made. RESULTS: A total of 88 patients presented between 1992 and 2021 with splenic artery pathology (5.7% of the cohort; mean age at diagnosis, 37 ± 11.1 years; 50% male). One-third were diagnosed with VEDS prior to the splenic artery pathology diagnosis, and 17% were diagnosed post-mortem. Most had a positive family history (61%). Most had COL3A1 variants associated with minimal normal collagen production (71.6%). Median follow up was 8.5 years (interquartile range, 0.9-14.7 years). Initial presentation was rupture in 47% of the cases. Splenic artery rupture overall was 51% (n = 45), including four cases of splenic rupture. There were no major differences in VEDS-related manifestations or COL3A1 variant type by rupture status. SAA was noted in 39% of the cases. Only 12 patients had splenic artery diameter documented in 12 cases with a median diameter of 12 mm (interquartile range, 10.3-19.3 mm). A total of 34 patients (38.6%) underwent 40 splenic arterial interventions: 21 open surgical, 18 embolization, and one unknown procedure. More than one splenic artery intervention was performed in five cases (14.7%). Open repair complications included arteriovenous fistula (n = 1), intestinal or pancreatic injury (n = 1 each), and four intraoperative deaths. There were no deaths or access site complications related to splenic artery embolization. Four patients (23.5%) developed a new SAA in the remaining splenic artery post embolization. All-cause mortality was 35% (n = 31), including 22 related to a ruptured splenic artery. CONCLUSIONS: Splenic arteriopathy in VEDS is associated with variants that affect the structure and secretion of type III collagen and frequently present with rupture. Rupture and open repair are associated with high morbidity and mortality, whereas embolization is associated with favorable outcomes. Suggest repair considerations at SAA diameter of 15 mm. Long-term follow-up is indicated as secondary splenic arteriopathy can occur.

Role of Imaging in Renal Cell Carcinoma: A Multidisciplinary Perspective.

With the expansion in cross-sectional imaging over the past few decades, there has been an increase in the number of incidentally detected renal masses and an increase in the incidence of renal cell carcinomas (RCCs). The complete characterization of an indeterminate renal mass on CT or MR images is challenging, and the authors provide a critical review of the best imaging methods and essential, important, and optional reporting elements used to describe the indeterminate renal mass. While surgical staging remains the standard of care for RCC, the role of renal mass CT or MRI in staging RCC is reviewed, specifically with reference to areas that may be overlooked at imaging such as detection of invasion through the renal capsule or perirenal (Gerota) fascia. Treatment options for localized RCC are expanding, and a multidisciplinary group of experts presents an overview of the role of advanced medical imaging in surgery, percutaneous ablation, transarterial embolization, active surveillance, and stereotactic body radiation therapy. Finally, the arsenal of treatments for advanced renal cancer continues to grow to improve response to therapy while limiting treatment side effects. Imaging findings are important in deciding the best treatment options and to monitor response to therapy. However, evaluating response has increased in complexity. The unique imaging findings associated with antiangiogenic targeted therapy and immunotherapy are discussed. An invited commentary by Remer is available online. Online supplemental material is available for this article. ©RSNA, 2021.

Percutaneous Extra-Anatomic Lymphovenous Bypass Creation: Toward Treatment of Central Conducting Lymphatic Obstructions.

INTRODUCTION: Protein-losing enteropathy manifests as a loss of serum proteins through the gastrointestinal tract, resulting in hypoproteinemia, extravascular fluid retention, and edema. Management consists of nutritional maintenance in conjunction with interventions targeted at treating the underlying etiology. MATERIALS AND METHODS: This report describes a patient with protein-losing enteropathy from a central conducting lymphatic obstruction who was treated with percutaneous extra-anatomic lymphovenous bypass creation. RESULTS: A modified gun-sight technique was used to create a lymphovenous bypass between an occluded terminal thoracic duct and the left internal jugular vein. CONCLUSION: A percutaneous technique to reconstruct the terminal thoracic duct via lymphovenous bypass creation was feasible.

Transhepatic inferior vena cava filter retrieval due to chronic occlusion of jugular and subclavian veins.

Retrieval of inferior vena cava filters is routinely performed via an internal jugular venous access. We present a case of a 55-year-old woman with myeloproliferative disorder, complicated by venous thrombosis. She was referred to interventional radiology for removal of an inferior vena cava filter, which had been placed 5 months prior for mechanical prophylaxis in the setting of femoral orthopedic surgery. Due to the patient's chronic occlusion of the bilateral jugular and subclavian veins, a transhepatic approach was used to retrieve the filter successfully without immediate complications.

The "triple-overlay" technique for percutaneous diagnosis and treatment of lesions of the head and neck: combined three-dimensional guidance with magnetic resonance imaging, cone-beam computed tomography, and fluoroscopy.

OBJECTIVE: Accurate image guidance is an essential component of percutaneous procedures in the head and neck. The combination of preprocedural magnetic resonance imaging (MRI) with cone-beam computed tomography (CBCT) and real-time fluoroscopy (the "triple-overlay" technique) could be useful in image-guided targeting of lesions in the head and neck. METHODS: Three patients underwent percutaneous diagnostic or therapeutic procedures of head and neck lesions (mean, 2.3 ± 2.4 cm). One patient presented for biopsy of a small lesion in the infratemporal fossa only visible on MRI, one presented for preoperative embolization of a nasal tumor, and one presented for sclerotherapy of a parotid hemangioma. Preprocedural MRI for each case was merged with CBCT to create a three-dimensional volume for procedural planning. This was then combined with real-time fluoroscopy to create a triple-overlay for needle trajectory and real-time guidance. RESULTS: The registration of MRI, CBCT, and fluoroscopy was successful for all three procedures, allowing 3D manipulation of the combined images. Percutaneous procedures were successful in all patients without complications. CONCLUSIONS: The combination of MRI, CBCT, and real-time fluoroscopy provides detailed anatomical information for 3D image-guided percutaneous procedures of the head and neck, especially for small lesions or lesions with features visible only by MRI.

Balloon test occlusion with the Doppler velocity guidewire.

Transcranial Doppler ultrasonography is the most commonly used method of cerebrovascular blood flow velocity measurement, but it is subject to certain technical and anatomic limitations. The Doppler velocity guidewire measures blood flow velocity within any vessel during cerebral angiography, overcoming these limitations. We report the first use of this guidewire in the measurement of blood flow velocity during balloon test occlusion, with results similar to simultaneously measured transcranial Doppler ultrasonography. Velocity measurement by Doppler guidewire could be useful in balloon test occlusion for vertebrobasilar circulation, where transcranial Doppler ultrasonography is limited, and provide anatomically specific blood flow velocity measurements in the diagnosis and treatment of stroke and other cerebrovascular diseases.