Clinical Quiz

Abstract

A 19-year-old white male with multiple medical problems presented to Egleston Children's Hospital, Atlanta, with tarry stools, fatigue, and dizziness. He denied vomiting and abdominal pain. His medications included ibuprofen, digoxin, furosemide, spironolactone, heparin, and calcium. His past medical history included complex congenital heart disease, status post Fontan repair; congenital hepatic fibrosis; heparin-responsive protein-losing enteropathy; and polycystic kidney disease with chronic renal failure. Family history was remarkable for polycystic kidney disease and chronic renal failure in his father. Physical examination revealed a moderately distended but nontender abdomen. Venous congestion was present on the lower thorax and anterior abdominal wall. A firm, hypertrophied left hepatic lobe extended 6 cm below the xiphoid. His spleen was massively enlarged, extending into the pelvis. A fluid wave was elicited indicating the presence of ascites. Admitting laboratory data revealed that his hemoglobin was 7.0 g/L, leukocyte count 3.0 × 109/L, platelet count 161 × 109/L, albumin 22 g/L, prothrombin time 12.1 seconds, creatinine 160 µmol/L, and BUN 33.2 mol/L. His serum bilirubin and hepatic enzymes were normal. Gastrointestinal endoscopy was performed after the patient was stabilized and transfused with packed red erythrocytes. The mucosal appearance of the proximal second portion of the duodenum is shown in Figure 1. What is the diagnosis? What are the treatment options?FIG. 1Answer: Numerous varices were observed throughout the duodenum. Many of the varices had red wales. A small amount of fresh blood was noted in the second portion of the duodenum before advancement of the endoscope into this region. The patient also had dilated submucosal veins in the gastric corpus with overlying mucosal erythema/edema consistent with a hypertensive gastropathy. A superficial aphthous ulceration was noted in the prepyloric antrum. Treatment options were carefully considered because of his multi-organ disease and high risk of continued and subsequent life-threatening gastrointestinal hemorrhage. Decompression of the portal system via a transjugular intrahepatic portosystemic shunt (TIPS) was recommended after cardiac consultation and review of an arteriographic study. His cardiac status was reassessed immediately before the TIPS procedure, and the inferior vena cava pressure was 26 mm Hg and the portal pressure 23 mm Hg at that time. The increased right heart pressure precluded the performance of a TIPS procedure. Anecdotal treatments of duodenal varices have included endoscopic band ligation, sclerotherapy, and embolization; none of these were attempted owing to the extensive number and location of the varices throughout the visualized duodenum. After discussion with the family, it was decided that he not be listed for multi-organ transplantation (heart, liver, and kidney). Palliative supportive measures, including diuretic therapy and periodic blood transfusions, were continued.