An Unusual Cause of Hematemesis: Esophageal Varices in a Child

Abstract

Portal vein thrombosis (PVT) is characterized by the complete or partial obstruction of the portal vein, due to the presence of a thrombus. This condition eventually results in non-cirrhotic portal hypertension (PHT) and is the leading cause of pediatric PHT and gastrointestinal bleeding. Risk factors for PVT in pediatric patients include those with a history of umbilical vein catheterization, neonatal sepsis, abdominal infection, cardiovascular malformation, coagulation disorder, or previous abdominal surgery. We present a unique case of bleeding esophageal varices due to non-cirrhotic PHT in a child. A 15-year-old male presented with a 1-day history of hematemesis. On presentation, the patient was tachycardic and hypotensive. The patient's initial hemoglobin was 8.0g/dL, and was resuscitated with packed red blood cells and intra-venous fluids. An esophagogastroduodenoscopy was performed which revealed large esophageal varices with nipple sign in the distal esophagus and gastric varices throughout the fundus (Figure 1). The patient underwent variceal band ligation, however continued to have hematemesis. Ultrasound and CT scan of the abdomen showed presence of a chronic portal vein occlusion with significant cavernous transformation (Figure 2). Surgery was consulted and the patient underwent exploratory laparotomy with failed attempt of Meso-Rex bypass and subsequent creation of a splenorenal shunt. Liver biopsy did not show any evidence of cirrhosis. The patient was discharged on post-operative day five after tolerating diet and resolution of symptoms. Prior to discharge, a complete hypercoagulability work-up was performed and the patient was found to have elevated anti-b2 glycoprotein levels, consistent with anti-phospholipid syndrome and the likely cause of this patient's portal vein thrombosis. Patients with chronic PVT may remain asymptomatic for many years, with variceal bleeding usually being the first manifestation of PVT in non-cirrhotic patients, which was the case in our patient. When there is a high clinical suspicion for PVT, ultrasonography (US) of the abdomen is the first line imaging modality. In children, creation of a Meso-Rex bypass, a vascular graft between the superior mesenteric vein and the Rex recessus (left portal vein system), helps improve clinical outcomes. Proper diagnosis of patient's' underlying condition predisposing to PVT is important in preventing further recurrence.Figure: Esophagogastroduodenoscopy showing large esophageal varices with nipple sign (A) and large gastric varices throughout the fundus (B).Figure: Ultrasound of the liver showing main portal vein thrombosis (A), and the use of doppler showing cavernous transformation of the portal vein (B), while CT scan of the abdomen and pelvis (venous phase) showing lack of blood flow through portal vein, findings consistent with presence of portal vein thrombosis, and extensive cavernous transformation (C).