Abstract

Patient Presentation: A 32 year old female with ESRD secondary to mesangial proliferative glomerulonephritis, recurrent SVC catheters due to failed AVG's, Mixed Connective Tissue Disorder and a failed renal transplant, presented with 2 days of melena and 2 episodes of hematemesis. There was no history of alcoholism, hepatitis, abdominal pain, NSAID or anticoagulant use. Physical examination was significant for tachycardia to 128 BPM, dilated superficial veins on the anterior chest wall, and melenic stool. There was no scleral icterus, abdominal distention, or hepatomegaly. The patient was transferred to the MICU. Work Up/Interventions: Laboratory studies were as follows: Hb 6.0 g/dL, Bilirubin 0.7, μmol/L, AST 7 U/L, ALT 6 U/L, INR 1.06 and Albumin 3.3 g/dL. The patient received transfusion of PRBC. EGD demonstrated F3 esophageal varices in the upper and mid-esophagus and presence of red wale signs and oozing blood. Endoscopic band ligation was performed. The melena resolved and the hemoglobin remained stable. Abdominal ultrasound demonstrated a normal liver. The portal veins were patent with normal direction of flow. The IVC and hepatic veins were patent. CT angiography of the abdomen demonstrated numerous abdominal wall collateral vessels. CT angiography of the chest revealed occlusion of the SVC along with numerous right-sided chest wall collaterals. The occlusion was at the junction of the SVC and the R brachiocephalic vein. A prominent azygous vein was seen. Diagnosis: Downhill esophageal varices secondary to SVC thrombosis due to multiple prior central venous catheters. Therapy: Balloon angioplasty of the SVC successfully improved flow. Subsequent EGD showed improvement of the esophageal varices. Definitive therapy consisted of right axillary vein to right atrium bypass graft.Figure 1Figure 2Figure 3Discussion: Downhill esophageal varices cause less than 0.1% of all variceal bleeds. Occlusion of the SVC results in increased pressure to the azygous and hemiazygous systems responsible for drainage of the venous plexuses of the upper two thirds of the esophagus. If the occlusion does not interfere with azygous drainage to the SVC, only the upper two thirds of the esophagus will be involved, as in the present case. Occlusion of the azygous drainage into the SVC results in varices throughout the esophagus. Treatment of downhill varices focuses on restoring venous drainage. Options include balloon angioplasty, SVC stenting and open surgical therapies. Endoscopic options include proximal banding. Sclerotherapy is avoided due to concerns over embolization.

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