Abstract

Abstract Background Esophageal varices represent a cause of upper gastrointestinal bleeding (UGI) with significant associated morbidity and mortality. The majority of variceal bleeds are due to portal hypertension and cirrhosis. In some very rare cases isolated upper esophageal varices may occur. These present management challenges, as typical management options for varices in cirrhosis are not applicable due to differences in pathophysiology. Infusions to decrease portal pressure and TIPS procedures may be used in cirrhosis, but are not effective in patients with SVC syndrome. Aims We present a literature review on isolated upper esophageal varices due to SVC syndrome, and present a case that provides further confirmation that surgical vascular reconstruction is a viable option in these cases. Methods The case of a 49 year old female with variceal hemorrhage secondary to SVC syndrome was reviewed. A search of the literature was undertaken using the terms “varices + superior vena cava syndrome” and “upper esophageal varices + management.” Results Overall, the majority of cases of isolated upper esophageal varices are secondary to SVC syndrome. They typically occur in cases of benign, rather than malignant SVC syndrome. Only one previous case was reported to be managed with surgical vascular reconstruction. We present the case of a 49-year old woman with SVC syndrome secondary to a thrombosed dialysis line who went on to develop isolated upper esophageal varices and large volume UGI bleeding. The pathophysiology of these “downhill” varices differs enough from portal hypertension that typical variceal management options may not be appropriate. Given a previous history of unsuccessful balloon angiography and the risk associated with variceal bleeding, she was taken back to the operating room for surgical vascular reconstruction. A surgical bypass was performed. She did well post-surgery and was discharged. She had no recurrence of bleeding and had a resolution of her edema at 6 month follow up. Conclusions Isolated upper esophageal varices are a potentially serious complication of SVC syndrome, and should be considered in cases of upper GI bleeding in patients with dialysis lines as they can have limited management options. This case was successfully managed by surgical vascular reconstruction, which represents an important treatment option in these difficult cases. Funding Agencies None

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