Variants of varices: is it all "downhill" from here?

A 39-year-old male inpatient, with cerebral palsy, endstage renal disease, and previously diagnosed idiopathic non-cirrhotic portal hypertension, was seen in consultation because of an episode of melena. Ten days prior to our consultation, he was admitted for erythema overlying an arteriovenous fistula on his left forearm. Blood cultures had grown methicillin-susceptible Staphylococcus aureus that was treated with intravenous cefazolin. On the night prior to his anticipated discharge, he was noted to have black stools, prompting an evaluation of potential upper gastrointestinal bleeding. His medical record revealed repeated upper gastrointestinal endoscopic investigations of hematemesis, dating back 19 years. Until 8 years ago, only esophagitis had been noted at endoscopy, but more recently, in addition to esophagitis, he was noted to have esophageal varices and portal hypertensive gastropathy. Subsequent endoscopies had also noted that the varices extended proximally to the midand upper esophagus. He had never undergone variceal band ligation. Following his initial endoscopy, he was evaluated for the etiology of portal hypertension. Serologic data were unrevealing of the etiology of cirrhosis. The first of three liver biopsies, performed via a transjugular approach 8 years prior to admission, was notable for a sharp angulation at the superior vena cava. Although the guide wire was passed without difficulty, enabling passage of the catheter into the inferior vena cava (IVC), injection of contrast demonstrated stenosis of the superior vena cava (SVC), just below the entry of the azygos vein, with anatomical distortion due attributed to marked scoliosis. Hepatic vein pressure measurements revealed a wedge pressure of 25 mmHg and a portal-to-hepatic venous pressure gradient of 19 mmHg. Liver biopsy at this time was reported as showing increased iron deposition in Kupffer cells, but no abnormalities specific to any form of liver disease and no cirrhosis. Two years prior to the current admission, two additional percutaneous, ultrasoundguided liver biopsies again revealed Kupffer cell siderosis, but again with no increased hepatic fibrosis, sinusoidal dilation, or cirrhosis. The patient was diagnosed with noncirrhotic portal hypertension for which no risk factors were identified. Because of recurrent hematemesis, 5 years prior to the current hospitalization, a transjugular intrahepatic portosystemic shunt (TIPS) was placed. During this procedure, severe stenosis of the right innominate vein was noted, connecting with a patent but tortuous IVC whose course followed his scoliotic curvature. Following TIPS placement, the portal-to-hepatic venous gradient was decreased to 7 mmHg with cessation of hematemesis for several years. Nonetheless, 2 years prior to this hospitalization, during evaluation for the cause of melena, portal pressures became elevated. A successful revision of the TIPS was performed via the right common femoral vein (due to difficulty cannulating the SVC), with venous drainage through enlarged hemiazygos, chest, and abdominal wall collateral vessels noted. He did not experience any further gastrointestinal bleeding until the current hospitalization. L. Gessel J. Alcorn Division of Gastroenterology and Hepatology, Department of Medicine, University of New Mexico Health Sciences Center, Albuquerque, NM, USA