This video shows endoscope withdrawl from the level of the lower esophageal sphincter. This patient is a 39 year old Caucasian female, on chronic hemodialysis for End Stage Renal Disease secondary to Adult Polycystic Kidney Disease. She presented with upper epigastric abdominal pain. Physical exam findings included visible swollen veins over the posterior thorax. An upper endoscopy was performed, which was largely unremarkable. The lower segment of the esophagus, visualized here, showed no pathology. However, on further withdrawl of the endoscope, esophageal varices came into view in the mid-to-upper esophagus. This is the classic location and appearance of so-called “downhill” esophageal varices. These varices are grade II, protruding into the esophageal lumen but not obstructing it, with no signs of hemorrhage.
Several months prior to this procedure, an MRA of the upper extremities revealed the likely cause of the downhill varices. This patient had developed compromise of her superior vena cava secondary to a thrombus around her indwelling hemodialysis catheter. The obstruction caused by this thrombus resulted in the development of this patient''s downhill esophageal varices.
Blood supplying the esophagus is drained by both the azygous system, and the portal system through the coronary (or left gastric) vein. In portal hypertension, elevated pressures through the coronary vein give rise to “uphill” varices in the lower third of the esophagus, a common phenomenon in cirrhosis. In contrast, downhill varices are due to Superior Vena Cava (SVC) syndrome, in which blood flow through the superior vena cava is obstructed. In SVC obstruction superior to the azygous vein, venous return from the head and upper extremities is redirected caudally through various collaterals and then into the azygous vein in order to reach the heart. However, this creates back-pressure in the azygous vein. The resulting esophageal varices will be in the mid- to upper esophagus. The designation “downhill” refers to the direction of the blood flow through the esophageal veins.
Causes of SVC syndrome that have been reported to cause downhill varices include upper mediastinal neoplasms such as lung cancers, thyroid carcinoma, thymoma, or mediastinal lymphoma. Vasculitis due to Behcet''s disease and systemic venulitis can also cause SVC syndrome. Other possible causes include substernal goiter and mediastinal fibrosis. Or, as in this case, the SVC can compromised as a thrombotic complication of upper extremity hemodialysis access.
Hemorrhage from downhill varices is far less common than hemorrhage from varices due to portal hypertension. As the most common causes of SVC obstruction are malignancies, most patients die from their underlying disease. In some cases, if the underlying etiology of the SVC syndrome can be corrected, then the downhill varices may disappear.
David J. Case, B.A., University of Texas Medical Branch
Ronica Case, BSN, University of Texas Medical Branch
Rajasekhara Mummadi, M.D., University of Texas Medical Branch
Gottumukkala S. Raju, MD, MD Anderson Cancer Center