S2727 Screening Colonoscopy Reveals Rare Colonic Varices Which Lead to the Diagnosis of Idiopathic Non-Cirrhotic Portal Hypertension

Abstract

INTRODUCTION: Idiopathic non-cirrhotic portal hypertension (INCPH) is a rare disease characterized by the presence of clinically significant portal hypertension in the absence of cirrhosis or other causes of liver disease. INCPH is a diagnosis of exclusion and the underlying pathophysiology is poorly understood. Here we present a case of INCPH diagnosed on screening colonoscopy. CASE DESCRIPTION/METHODS: A 54-year-old male presented for a screening colonoscopy. He was asymptomatic, had no past medical history, no past surgical history, no history of liver disease or significant alcohol intake, and did not take any medications. Colonoscopy revealed several adenomatous polyps as well as large varices in the ascending colon (Figure 1). CT scan showed normal liver morphology without vascular thrombosis. EGD after colonoscopy revealed several columns of esophageal varices (Figure 2). He was sent for liver biopsy with HVPG which showed normal liver parenchyma. HVPG showed a free hepatic pressure of 6 mmHg, wedge of 10mmHg, and gradient of 4 mmHg. Infectious workup including schistosomiasis antibodies were negative, echocardiogram was normal, hypercoagulability and infectious workups were negative, and no other environmental exposures or chemotherapy were identified on review. He was started on carvedilol and scheduled for follow-up in clinic. DISCUSSION: Colonic varices are a rare finding in cirrhosis and occur even more infrequently in NCPH. When varices are identified in the absence of known liver disease, the next steps in the workup involve ruling out other common causes of NCPH including Budd-Chiari syndrome and portal vein thrombosis. Presentation of ICNPH ranges widely depending on the phenotype and can include histologic or imaging findings of PH, presence of EV, or even bleeding. Prevalence varies geographically and peak incidence occurs in the 40s. INCPH is often associated with underlying systemic conditions such as HIV, chronic infections, autoimmune disorders, and prothrombotic factors. Mortality is typically non-liver related and associated with underlying comorbidities, in part due to preserved hepatic function. Management consists of treating underlying portal hypertension and follows similar treatment strategies to those for cirrhotic portal hypertension.Figure 1.: Endoscopic view showing varices in the ascending colon.Figure 2.: Endoscopic view showing varices in the distal esophagus.