A 36‐year‐old man was seen for preoperative assessment before wound debridement. His history included sickle cell disease previously complicated by superior vena cava (SVC) syndrome, which required surgical jugular‐vein‐to‐right‐atrium bypass grafts. He denied headache, dyspnea, or melena, but reported “food sometimes gets caught in [my] throat.” On exam, his torso veins were prominent (Fig. 1). Pemberton's sign was positive. Heparin anticoagulation was initiated. Initial labs noted Hgb 10.3 g/dL and platelets 59,000/mL. Blood smear visualized sickle cells without schistocytes. CT venogram confirmed thrombus occluding both jugular veins with extension into the SVC and surgical bypass grafts; extensive esophageal venous engorgement was also seen. Endoscopy found markedly dilated varices in the proximal esophagus (Fig. 2a) but no varices at the distal esophagus (Fig. 2b). Variceal banding was not performed. Attempted catheter‐directed thrombolysis was only modestly successful. The patient was clinically improving, however, and thus was treated with anticoagulation alone. He was discharged with resolving thrombocytopenia and no signs of variceal bleeding.
Hospitalists frequently encounter SVC syndrome, especially in patients with malignancy or central venous access. Less commonly, SVC obstruction creates esophageal varices as blood returns from the upper vasculature to the heart through collateral flow. Unlike the familiar “uphill” esophageal varices generated by portal hypertension, these “downhill” varices develop in the submucosa of the proximal esophagus. This location is presumed less prone to erosion by gastric secretions and concomitant bleeding. When hemorrhage does occur, however, the proximal esophagus' location and structural weakness make endoscopic banding technically challenging. In addition, banding “downhill” varices using standard technique may actually worsen intravariceal hemodynamics. Frustratingly, sclerotherapy at the proximal esophagus may cause spinal cord infarction. Recommended first‐line treatment for “downhill” varices is therefore attempted relief of the underlying SVC obstruction. Repeat endoscopy may be needed to evaluate variceal improvement, especially as long‐term anticoagulation is often required.
“Downhill” esophageal varices are a likely underrecognized complication of SVC syndrome. Understanding the physiology of such lesions has notable consequences in the diagnosis and management of patients with this condition.
To cite this abstract:Gandiga P. When Superior Vena Cava (Svc) Syndrome Goes “Downhill”. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 474. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/when-superior-vena-cava-svc-syndrome-goes-downhill/. Accessed April 5, 2020.