An Uncommon Cause of Hematemesis in a Patient With End-stage Renal Disease

Abstract

Question: A 45-year-old man presented with an acute onset of large volume hematemesis associated with worsening facial and neck swelling for the past 6 months. He has a significant past medical history of end-stage renal disease secondary to congenital dysplastic kidneys and has been on hemodialysis for the past 30 years. He has no history of chronic liver disease. Clinical examination revealed diffuse facial and neck edema. Dilated superficial veins were also seen on his upper anterior chest wall. A pulsatile left brachiocephalic arteriovenous fistula was present. The patient was intubated and urgent esophagogastroduodenoscopy was performed. The following bleeding lesion was visualized 25 cm from the incisors (Figure A). What is the diagnosis? Look on page 405 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. The patient was suspected to have downhill esophageal varices. The bleeding varix located 25 cm from the incisors was band ligated with successful hemostasis (Figure B). A computed tomographic venography of the thorax was performed, which revealed an occluded right brachiocephalic to superior vena cava stent (white arrow) and a prominent azygos vein (red arrow) (Figure C). The patient underwent a percutaneous central venogram and balloon angioplasty of the occluded right brachiocephalic and superior vena cava stent (Figure D). After the procedure, the patient’s facial and neck swelling diminished rapidly and there were no further episodes of bleeding. Downhill varices, or otherwise known as proximal esophageal varices are most commonly due to superior vena cava obstruction secondary to various etiologies such as malignancy, mediastinal fibrosis, and central venous catheter placement.1Ostler P.J. Clarke D.P. Watkinson A.F. Gaze M.N. Superior vena cava obstruction: a modern management strategy.Clin Oncol (R Coll Radiol). 1997; 9: 83-89Abstract Full Text PDF PubMed Google Scholar Although malignancy is the most common etiology of superior vena cava obstruction, bleeding downhill varices tend to occur more in patients with benign etiologies, such as central venous catheter placement.2Loudin M. Anderson S. Schlansky B. Bleeding 'downhill' esophageal varices associated with benign superior vena cava obstruction: case report and literature review.BMC Gastroenterol. 2016 24; 16: 134Crossref PubMed Scopus (13) Google Scholar This phenomenon may be due to increased venous return and pressure secondary to the underlying arteriovenous access for hemodialysis. The underlying superior vena cava obstruction leads to diversion of venous return through the azygos vein, which in turns causes engorgement of the veins draining the proximal to middle aspect of the esophagus, and causing the development of downhill varices.3Marini T.J. Chughtai K. Nuffer Z. et al.Blood finds a way: pictorial review of thoracic collateral vessels.Insights Imaging. 2019; 10: 63Crossref PubMed Scopus (3) Google Scholar During acute bleeding, endoscopic therapies such as variceal band ligation can be used for hemostasis. However, the mainstay of treatment is to treat and relieve the underlying superior vena cava obstruction. Clinicians should have a high index of suspicion for downhill varices if a patient with end-stage renal disease on hemodialysis presents with upper gastrointestinal bleeding, especially if the patient has a history of central venous catheter placement.