A Rare Cause of Gastric Variceal Bleeding

A 68-year-old woman with stage III colon cancer status after right hemicolectomy and adjuvant FOLFOX (5-fluorouracil/leucovorin/oxaliplatin) chemotherapy was hospitalized for melena and found to have new-onset esophageal and gastric varices on esophagogastroduodenoscopy. Her workup did not reveal an underlying liver disease, but her liver biopsy showed noncirrhotic portal hypertension from obliterative portal venopathy (OPV). The development of OPV is likely from her use of oxaliplatin-based chemotherapy.

Interobserver study on histologic features of idiopathic non-cirrhotic portal hypertension

Background Histologic features of idiopathic non-cirrhotic portal hypertension (INCPH) may overlap with those without INCPH. Recently, these features have been recognized as part of the larger spectrum of porto-sinusoidal vascular disease (PSVD). We assessed interobserver agreement on histologic features that are commonly associated with INCPH and studied whether a provision of relevant clinical history improves interobserver agreement. Methods The examined histologic features include lobular (such as anisocytosis, nodular regeneration, sinusoidal dilatation, increased parenchymal draining veins, and incomplete fibrous septa) and portal tract changes (such as paraportal shunting vessel(s), portal tract remnant, increased number of portal vessels, and obliterative portal venopathy). Thirty-four archived liver samples from patients with (group A) and without (group B) INCPH were retrieved. A total of 90 representative images of lobules (L) and portal tracts (P) were distributed among 9 liver pathologists blinded to true clinical history. Each pathologist answered multiple choice questions based on the absence (Q1) or presence (Q2) of clinical history of portal hypertension. Fleiss’ kappa coefficient analysis (unweighted) was performed to assess interobserver agreement on normal versus abnormal diagnosis, in L and P, based on Q1 and Q2. Results The kappa values regarding normal versus abnormal diagnosis were 0.24, 0.24, 0.18 and 0.18 for L-Q1, L-Q2, P-Q1, and P-Q2, respectively. With true clinical history provided, the kappa values were L- 0.32, P-0.17 for group A and L-0.12, P-0.14 for group B. Four pathologists changed their assessments based on the provided history. Interobserver agreement on the interpretation of L and P as normal versus abnormal was slight to fair regardless of provision of clinical history. Conclusions Our findings indicate that the histologic features of INCPH/PSVD are not limited to patients with portal hypertension and are subject to significant interobserver variation.

Cystic Fibrosis–Related Liver Disease in a 12-Year-Old: Important Questions of Portal Hypertension Etiology That Start With Pathology: A Case Presentation

Introduction Cystic fibrosis liver disease (CFLD) has long been postulated to be secondary to dysfunctional cystic fibrosis transmembrane conductance regulator in the apical biliary epithelium, leading to bile stasis and eventually cirrhosis with portal hypertension (PH). We present a considerably young patient with signs of esophageal varices and liver biopsy indicating CFLD with significant obliterative portal venopathy as a result of long-standing portal hypertension. Case Presentation A 12-year-old with CF (diagnosed at 1 year) and pancreatic insufficiency was noted to have chronically elevated liver enzymes with decreasing platelet counts (130,000 K/ µL). These findings prompted an ultrasound, which showed splenomegaly. Upper endoscopy showed grade 1 esophageal varices, and liver needle biopsy performed at this time demonstrated focal neutrophilic lobular inflammation with marked pseudoacinar transformation and biliary metaplasia. Noted was grade 1 macrovesicular steatosis, focal dense fibrosis also known as “focal biliary cirrhosis,” and obliterative portal venopathy. Discussion CF patients with liver fibrosis have significant risk of future morbidity. Of specific interest is identification of PH, and obliterative portal venopathy is a very important feature to distinguish on biopsy as it predicts clinical course. Two recent studies in young adults described obliterative portal venopathy (OPV) and noncirrhotic portal hypertension (NCPH) as the predominant pathophysiology in young adults (median, 22 years) with CFLD. One study found cirrhosis present in only 27% of patients with portal hypertension. In addition, it appears that portal hypertension can precede cirrhotic changes. This unique case in a very young patient with CFLD demonstrates that the underlying pathogenesis of noncirrhotic portal hypertension and CFLD remains to be fully solved. These observations may have important consequences for CFLD management, including opting away from “cholestasis-targeted” treatments and more often considering portosystemic shunting procedures instead of transplant, as liver function remains preserved in numerous CFLD cases.