Purpose or Case Report: Pediatric esophageal varices almost uniformly occur as a consequence of portal hypertension in children with chronic liver disease or extrahepatic portal vein obstruction. Varices in these cases typically evolve in the distal esophagus at the intersection of the left gastric and esophageal veins. We report a rare case of “downhill” varices that developed in the proximal esophagus due to extensive chronic thrombosis of the SVC and brachiocephalic veins in a child with intestinal failure requiring lifelong central venous access for parenteral nutrition. We report a case of downhill varices associated with thrombosis of the SVC and brachiocephalic veins, secondary to a central line.
A 14-year-old boy with an ACTG2 (actin gene) mutation-related history of parenteral nutrition dependent chronic intestinal pseudo-obstruction and central venous catheter-associated thrombi on prophylactic anticoagulation presented with new onset dysphagia. On endoscopy, Grade II esophageal varices without stigmata of recent bleeding were unexpectedly identified in the upper and middle esophagus. Imaging was then performed to assess for advanced liver disease or abnormal portal flow. CT of the abdomen and pelvis showed no splenomegaly, ascites, or radiographic signs of cirrhosis. There was, however, partial visualization of dilated para-esophageal vessels coursing from the chest.
Subsequent chest CT demonstrated chronic occlusion of the right internal jugular and brachiocephalic veins, along with severe narrowing of the SVC. There were extensive venous collateral pathways through the azygos and para-esophageal venous system and engorged submucosal veins were present in the esophagus. Echocardiography confirmed a stable chronic thrombus in the SVC.
Given the high risk of intervention, the decision was made to forgo immediate SVC balloon dilation and stenting and to continue active surveillance.
This case highlights the important role of imaging in differentiating “downhill” esophageal varices from their “uphill” counterpart. This distinction is critical, as each entity necessitates a different treatment approach. Definitive management of downhill varices is aimed at alleviating SVC obstruction but carries significant risk and was deferred in this case.
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