Segmental arterial mediolysis (SAM) is an uncommon, nonatherosclerotic, noninflammatory, large- to medium-sized arteriopathy first described in 1976. It is characterized histologically by vacuolization and lysis of the outer arterial media leading to dissecting aneurysms and vessel rupture presenting clinically with self-limiting abdominal pain or catastrophic hemorrhages in the abdomen. Patients of all ages are affected with a greater incidence at the fifth and sixth decades. There is a slight male predominance. Imaging findings overlap with inflammatory vasculitis, collagen vascular disease, and fibromuscular dysplasia. The presence of segmental dissections involving the celiac, mesenteric, and/or renal arteries is the key distinguishing features of SAM. Inflammatory markers, genetic tests for collagen vascular disorders, and hypercoagulable studies are negative. Anti-inflammatory agents and immunosuppressants are not effective. A mortality rate of 50 % has been attributed to the acute presentation with aneurysmal rupture necessitating urgent surgical or endovascular treatments; in the absence of the acute presentation, SAM is a self-limiting disease and is treated conservatively. There are no established guidelines on medical therapy, although optimal control of blood pressure is considered the main cornerstone of medical therapy. The long-term prognosis is not known.
Median arcuate ligament syndrome (MALS) is a rare condition which is due to the compression of celiac trunk by low riding of fibrous attachments of median arcuate ligament and diaphragmatic crura. Technically, MALS is a diagnosis of exclusion, consisting of vague symptoms comprising of postprandial epigastric pain, nausea, vomiting and unexplained weight loss. Different imaging modalities like Doppler ultrasound, computed tomography, magnetic resonance imaging and mesenteric angiogram are helpful to demonstrate celiac axis compression. The goal of treatment is decompression of celiac trunk either by open, laparoscopic or robotic method along with adjuvant interventional procedures like percutaneous transluminal angioplasty (PTA) and stenting. Surgical is the mainstay of treatment. This approach is based on open, laparoscopic or robotic release of compressed ligament along with celiac ganglionectomy and celiac artery revascularization. The role of interventional radiology is limited to angioplasty and stenting to open the stenosis rather than addressing the underlying compression of celiac trunk which has resulted in the symptoms. However, both the diagnosis and therapeutic intervention remains challenging. Extensive evaluation of etiology and pathophysiology of MALS and addressing the same through minimally invasivetechniques may yield best prognosis in future. In this review article, we discuss briefly about the MALS in terms of etiology, diagnosis and its management including the role of interventional radiology.
223 Background: For HCC pts undergoing LT, local regional treatment as a "bridge" is standard to decrease tumor progression. The most common treatment is TACE, but the best bridging modality is unclear. Recently, SBRT has been shown to be both safe and effective when used in pts with locally advanced HCC. We prospectively compare SBRT to TACE as a bridge for HCC pts undergoing LT. Methods: 60 pts planned for accrual. From 9/2014-9/2016, 29 pts within Milan Criteria with C-P Class A/B cirrhosis were randomized to TACE vs. SBRT. TACE pts received 2 treatments one month apart utilizing DEBDOX beads (n = 15). TACE pts were hospitalized after each TACE. Pts receiving SBRT (n = 12) received a median total dose of 45Gy delivered over 5 fractions using fiducials. Mean liver dose, Veff, and NTCP were utilized to determine the prescription dose. Pts were assessed by imaging using mRECIST criteria at 2 months and every 3 months thereafter until LT or death. Toxicity and quality of life were assessed before treatment, during treatment, two weeks post-treatment, and then every three months using the PIQ-6 Pain Impact Questionnaire and the SF-36v2 Health Survey. Primary endpoint was time to retreatment of treated lesion(s). Secondary endpoints include toxicity, pathologic response, radiologic response, number of subsequent treatments, cost, and QOL. Results: A. Demographics/Toxicity. Conclusions: For HCC patients with C-P Class A/B liver cirrhosis, SBRT appears equally effective to TACE as a bridge to liver transplantation, may engender less toxicity, and eliminates hospitalizations. Clinical trial information: NCT02182687. [Table: see text][Table: see text]
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