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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

Background: Liver disease in patients with cystic fibrosis (CF) is an understudied and increasingly common concern. The prevalence of cystic fibrosis liver disease (CFLD) in Canada has not been clearly established, although it is now the third leading cause of death among patients with CF. The current literature identifies a range in prevalence from 4% to 65%, which implies the need for further research. This study aimed to determine the prevalence of CFLD among adult patients with CF in Newfoundland and Labrador. Methods: Charts of patients with CF from the St. John’s, Newfoundland, CF clinics were reviewed retrospectively for the presence of elevated liver enzymes, imaging or biopsy of the liver, and other etiologies of liver disease. Prevalence was determined for patients meeting the criteria for CFLD in the population as a whole and for those who had undergone all pertinent investigations. Results: The diagnostic guidelines for CFLD were met in 14 of 57 cases (24.6% prevalence). Severe CFLD was present in 9 patients (15.8%). Among all patients with CF, 33 (57.9%) had isolated liver enzyme elevation. Eleven patients had not had the requisite imaging performed for accurate diagnosis. Among the subset who had undergone imaging, the prevalence of CFLD was 30%. Conclusions: This study attempted to fill the gap in Canadian CFLD prevalence data by examining the population with CF in Newfoundland and Labrador. The prevalence of CFLD was found to be between 25% and 30%. More accurate determination of prevalence could be done with future cross-sectional or prospective studies.

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