Cause Of Non-cirrhotic Portal Hypertension Research Articles

Congenital hepatic fibrosis as a rare cause of non-cirrhotic portal hypertension

Congenital hepatic fibrosis as a rare cause of non-cirrhotic portal hypertension

Obliterative portal venopathy: A neglected and probably misdiagnosed disease with peculiar etiology in South America.

Obliterative portal venopathy (OPV) is one of the causes of non-cirrhotic portal hypertension. However, many aspects of OPV remain unclear, including the etiology, pathogenesis, and natural history. The aim of this study was to describe the clinical features of OPV in a series of patients in Brazil in whom OPV was diagnosed through liver biopsy. Forty-three consecutive adult patients with OPV were retrospectively selected as a case series based on histologic criteria, defined by the presence of at least portal fibrosis, phlebosclerosis, disappearance and/or reduction of the caliber of portal vein branches, and exclusion of cirrhosis. Clinical and laboratory data were analyzed. Clinically significant portal hypertension was considered in the presence of esophageal varices and/or ascites. The mean age of patients at diagnosis was 44.5 ± 11 years, who were predominantly female (81%). Clinically significant portal hypertension was found in 28% of cases. The most frequent indication for liver biopsy was the elevation of liver enzymes, mostly γ-glutamyl transferase (GGT) in 76% of patients, averaging 222 IU/L (upper limit of normality up to 40 IU/L) and alanine aminotransferase (ALT) in 64%, mean 84 IU/L (38 IU/L). One-third of our patients had exposure to medications, especially herbal medicines, at the time of enzymatic changes. Other risk factors highlighted were features of autoimmunity in 25% of patients or thrombophilia in 20%. OPV can be diagnosed even before the onset of portal hypertension, ALT elevation, and especially GGT elevation in most cases. Its etiology is not defined, but autoimmune diseases, thrombophilia, and the use of medications or herbal medicines may play a role.

S1783 Noncirrhotic Portal Hypertension and Pancreaticogastric Fistula Complicating Acute Pancreatitis With Fluid Collections

Introduction: Noncirrhotic portal hypertension constitutes an increase in pressure of the portal venous system in the absence of cirrhosis. This is a rare condition; however, it is insufficiently acknowledged given its decreased prevalence in North America and Europe compared to other nations. We present a rare case of noncirrhotic portal hypertension and pancreatico-gastric fistula complicating acute pancreatitis with an acute fluid collection and resultant portal vein narrowing. Case Description/Methods: A 55-year-old man with history of alcohol use disorder presented to the emergency department with worsening abdominal pain 2 weeks after discharge for uncomplicated acute pancreatitis. Lipase was 48 IU/L (normal 11-82 IU/L). CT abdomen showed increasing peripancreatic fluid collection with marked narrowing of the main portal vein, which appeared near threadlike, and narrowing of the bifurcation of the portal vein extending into the left portal vein. The splenic vein was also severely narrowed (Figure A). He was discharged home with outpatient follow-up, however, returned to the ED for intractable pain one month later. A CT abdomen showed florid changes of pancreatitis with pseudocyst formation with new foci of air within the collection concerning for fistulous tract formation with the gastric lumen (Figure B). The portal vein and splenic vein narrowing improved, but the narrowing of the right and left portal veins worsened. He underwent upper endoscopy which showed a small area of White "milky" base in the posterior wall of the incisura angularis which was suggestive of fistulous opening in the area (Figure C). Four columns of small distal esophageal varices were also noted. During admission, paracentesis was performed for ascites. Serum ascites albumin gradient was over 1.1 consistent with ascites due to portal hypertension – likely due to the portal vein narrowing. Patient was discharged in stable, improved condition. Discussion: Although portal hypertension normally presents in cirrhotic patients, it may rarely present in the absence of cirrhosis by conditions such as portal vein thrombosis or portosinusoidal vascular disease. Compression of the portal vein from an acute pancreatic fluid collection is an extremely rare cause of noncirrhotic portal hypertension. Clinicians should have a high index of suspicion for complications related to portal hypertension such as ascites or varices in patients with a prolonged course of acute pancreatitis without cirrhosis.Figure 1.: (A) Contrast CT abdomen showing marked narrowing of the main portal vein, which appears near threadlike. (B) One of 2 foci of air in the collection at the anterior superior aspect of the pancreatic body. Appears inseparable from the posterior wall of the gastric body, concerning for fistulous involvement. (C) Area of White “milky” base in the posterior wall of the incisura angularis. This area was rinsed, however re-accumulated despite no obvious opening seen.

Identification and clinical significance of nodular regenerative hyperplasia in primary sclerosing cholangitis.

Background and AimPrimary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of intrahepatic and extrahepatic bile ducts. PSC is frequently associated with inflammatory bowel disease (IBD). Nodular regenerative hyperplasia (NRH) can occur in IBD with the use or even in the absence of thiopurine treatment. We aimed to study the significance of the presence of NRH and obliterative portal venopathy (OPV), both causes of non‐cirrhotic portal hypertension (NCPH), in patients having PSC.MethodsPatients with PSC and concurrent NRH on liver biopsy were identified from the digital pathology database covering the period 2003–2019. Evaluation of liver biopsy and the original diagnoses were confirmed on review based on standard histological features diagnostic for NRH and OPV. Clinical and laboratory data were obtained from electronic medical records.ResultsThirty‐one patients (21 male, 10 female; median age at biopsy 40.1 years) were included in the study. Twelve (38.7%) patients had OPV in addition to NRH on the liver biopsy. Nineteen (61.2%) patients had IBD including 11 with Crohn's disease (CD), 7 with ulcerative colitis (UC), and 1 with indeterminate colitis. Thirteen (41.9%) patients had evidence of portal hypertension, 10 (32.2%) with esophageal varices, 4 (12.9%) with history of variceal bleeding, 6 (19.3%) with ascites, and 14 (12.9%) with splenomegaly. Eleven (35.4%) patients had a cirrhotic‐appearing liver on imaging. Twelve (38.7%) patients had a history of prior or current thiopurine use.ConclusionsThe current study suggests that NRH with or without OPV independently occurs in patients having PSC and may lead to NCPH, even in the absence of concurrent IBD and/or thiopurine therapy.

Ultrasound and Computed Tomography Features of Non Cirrhotic Portal Hypertension caused due to Non Cirrhotic Portal Fibrosis and Extrahepatic Portal Vein Obstruction: A Retrospective Study

Introduction: Non Cirrhotic Portal Hypertension (NCPH) encompasses a wide range of disorders that are characterised by increased portal pressure with the absence of cirrhosis of liver. The disorders are classified anatomically based on the site of blood flow obstruction into prehepatic, hepatic and posthepatic causes. These numerous and variable aetiological factors and the lack of standardised diagnostic criteria, makes NCPH under-recognised both clinically and pathologically and often being falsely labelled as cryptogenic cirrhosis. Therefore, it is important for radiologists to be aware of the imaging features that constitute NCPH for its early recognition, so that appropriate management can be carried out. Aim: To assess the distinct ultrasound and Computed Tomography (CT) features of the two main causes of NCPH: Non Cirrhotic Portal Fibrosis (NCPF) and Extrahepatic Portal Venous Obstruction (EHPVO). Materials and Methods: This retrospective observational study included all radiologically diagnosed cases of NCPH who underwent ultrasound and CT examination at the Institution between June 2020-June 2021. A total of 10 patients met the inclusion criteria. The various imaging features of NCPF and EHPVO were analysed and tabulated. Results: The study showed male predominance with males and females being seven and three, respectively. The mean age of the patients in the study was 35.1 years. The common cause in both NCPF and EHPVO was idiopathic. The most characteristic imaging finding in EHPVO was cavernous transformation of the portal vein seen in all cases and that in NCPF being dilatation of the spleno-portal axis and mural thickening of the main portal vein seen in all the cases. Conclusion: The present study demonstrated the characteristic imaging features of NCPF and EHPVO which are leading cause of NCPH, facilitating radiologist to identify the conditions and not label cases with Portal Hypertension (PHT) as cryptogenic cirrhosis.

Cognitive Impairement in Non-Cirrhotic Portal Hypertension: Highlights on Physiopathology, Diagnosis and Management.

Hepatic encephalopathy (HE) is one of the most frequent complications of cirrhosis. Several studies and case reports have shown that cognitive impairment may also be a tangible complication of portal hypertension secondary to chronic portal vein thrombosis and to porto-sinusoidal vascular disease (PSVD). In these conditions, representing the main causes of non-cirrhotic portal hypertension (NCPH) in the Western world, both overt and minimal/covert HE occurs in a non-neglectable proportion of patients, even lower than in cirrhosis, and it is mainly sustained by the presence of large porto-systemic shunt. In these patients, the liver function is usually preserved or only mildly altered, and the development of porto-systemic shunt is either spontaneous or iatrogenically frequent; HE is an example of type-B HE. To date, in the absence of strong evidence and large cooperative studies, for the diagnosis and the management of HE in NCPH, the same approach used for HE occurring in cirrhosis is applied. The aim of this paper is to provide an overview of type B hepatic encephalopathy, focusing on its pathophysiology, diagnostic tools and management in patients affected by porto-sinusoidal vascular disease and chronic portal vein thrombosis.

Meso-Rex bypass versus portosystemic shunt for the management of extrahepatic portal vein obstruction in children: systematic review and meta-analysis.

Extrahepatic portal vein obstruction (EHPVO) is a major cause of non-cirrhotic portal hypertension in children. Surgical procedures for EHPVO include portosystemic shunts (PSS) and meso-Rex bypass (MRB). We conducted a systematic review and meta-analysis to compare the effectiveness of MRB versus PSS in EHPVO patients. A systematic literature search was performed using four databases. Articles reporting EHPVO and comparing patients who received MRB and PSS were included in the analysis. We retrieved 851 papers, of which five observational studies met the inclusion criteria. There was no difference in shunt complications, mortality, or gastrointestinal bleeding after surgery between MRB and PSS in the meta-analysis. MRB had increased shunt complications compared with PSS in the non-comparative studies. MRB had a potential advantage over PSS in long-term prognosis in one comparative study. Overall, the quality of the evidence was low. Based on available data, our meta-analysis indicates that MRB does not increase shunt complications, mortality, or gastrointestinal bleeding after surgery. The present study did not reveal superiority for either MRB or PSS. The paucity of well conducted trials in this area justifies future multicenter studies and studies that examine long-term outcomes.

NON-CIRRHOTIC PORTAL FIBROSIS CAUSING PORTAL HYPERTENSION

Portal hypertension is more prevalent in patients with liver cirrhosis and occurs infrequently in those without liver cirrhosis. Non-cirrhotic portal brosis (NCPF) and extrahepatic portal venous obstruction (EHPVO) are the two most common causes of non-cirrhotic portal hypertension. Unlike EHPVO, NCPF does not cause thrombosis of the extrahepatic portal vein. Sclerosis of the portal vein's medium and small branches occurs in NCPF. In NCPF, the hepatic venous pressure gradient (HVPG) is normal, in contrast to cirrhosis, where it is increased. Additionally, NCPF is referred to as non-cirrhotic intrahepatic portal hypertension (NCIPH), idiopathic portal hypertension, hepatoportal sclerosis, and benign intrahepatic portal hypertension. It is a disease with an unknown etiology that primarily affects middle-aged males and females and manifests as hematemesis and massive splenomegaly

P-125 REX SHUNT IN A DEVELOPING COUNTRY – IS IT POSSIBLE?

Extrahepatic portal vein obstruction (EHPVO) is a frequent cause of noncirrhotic portal hypertension in children. Describe the experience in the surgical treatment of EHPVO in children, in a developing country. Retrospective case series study, with medical records review of patients with EHPVO, who underwent surgical treatment, by an experienced surgeon, between July 2016 and May 2019. Patient profile, laboratory test, images, liver histology, surgery performed, postoperative complications and shunt patency were analysed. 12 patients, median age of 4 years, umbilical catheterization was present in 8 patients (66,6%). Ten patients performed portography, and 60% had type A by Baveno VI criteria. Despite normal liver tests, liver biopsy revealed ductular proliferation in 83,3% of patients and mild portal fibrosis in 66,7%. Splenomegaly was present in 91,7% and thrombocytopenia in 83,3%. All patients had oesophageal varices and gastrointestinal bleeding occurred in 83,3%. Among the coagulation tests, the deficiency of C and S proteins is noteworthy in most patients, with 72,3% and 63,6% respectively. It was possible to perform meso-Rex bypass in 10 patients (83,3%); in the other 2 distal splenorenal shunt was performed. Early post-operative complications occurred in 58,3% of patients, the most common was ascites in 4 (33,3%), which resolved in less than 1 month. One patient developed shunt thrombosis in the first 7 days after surgery, not resolved with thrombectomy. In outpatient follow-up one patient developed thrombosis in the Rex shunt and another 4 had stenosis. All of them underwent to interventional radiology. Currently 8 of 10 meso-Rex patients (80%) have patent shunt. Rex shunt is possible in developing countries with an experienced surgeon and multidisciplinary team.

Histological analyses of trucut liver biopsies from patients with noncirrhotic portal fibrosis and extra-hepatic portal vein obstruction.

Both noncirrhotic portal fibrosis (NCPF) and extrahepatic portal venous obstruction (EHPVO) are important causes of noncirrhotic portal hypertension (PH) in the Asian region. In this study, we analyzed the histopathological changes of liver needle-core biopsies from patients with NCPF and EHPVO. The patients were diagnosed as per the Asia Pacific Association for the Study of Liver (APASL) criteria. Minimum adequacy criteria for liver core biopsies were defined, and finally, 69 liver biopsies from patients with NCPF and 100 liver biopsies from patients with EHPVO were analyzed. All histological parameters were predefined, and three experienced pathologists analyzed the biopsies after reaching consensus. Institute ethics committee clearance was taken. Although some histological features were overlapping, phlebosclerosis of intra-hepatic branches of the portal vein (PV), periportal aberrant vascular channels, remnant portal tracts, and hepatic fibrosis beyond the portal tracts without the formation of complete hepatic nodules (P < 0.001 for all) were common histological characteristics of NCPF on core-needle liver biopsies; while maintained lobular architecture, nonspecific dilatation of PV branches, absence of intra-hepatic PV phlebosclerosis, aberrant vascular channels, and significant fibrosis were characteristics of EHPVO. Despite the considerable histological overlap between NCPF and EHPVO, careful histological evaluation, supplemented by clinical features, radiological and biochemical findings can help in making a conclusive diagnosis. Patients with NCPF and EHPVO with clinical jaundice show transaminitis, high serum alkaline phosphatase level, more variceal bleed, and histological evidences of nodular regenerative hyperplasia.

Nodular Regenerative Hyperplasia of the Liver in Rheumatic Disease: Cases and Review of the Literature.

Nodular regenerative hyperplasia (NRH) is a rare disease that is characterized by benign transformation of the hepatic parenchyma into small nodules with little to no fibrosis. Nodular regenerative hyperplasia is a cause of noncirrhotic portal hypertension. Symptoms can range from asymptomatic disease to more serious complications of portal hypertension such as esophageal varices and ascites. Nodular regenerative hyperplasia has been described in association with a variety of different rheumatologic, hematologic, and oncologic diseases, as well as in immune deficiency states and with exposures to certain toxins. Diagnosis is made by histology, and the treatment involves addressing the underlying disease. The first description of this rare disease was actually described in a patient with rheumatoid arthritis, neutropenia, and splenomegaly (Felty’s Syndrome). We describe 2 cases of NRH associated with underlying rheumatic disorders, in one of which NRH was actually the presenting feature of the patient’s underlying autoimmune condition. Subsequently, we provide a brief review of the literature of NRH in autoimmune disease with respect to epidemiology, cause, clinical manifestations, diagnosis, and treatment.

2430 Placental Site Trophoblastic Tumor—Unusual Cause of Nodular Regenerative Hyperplasia and Portal Hypertension: Case Report

INTRODUCTION: There are various causes of non-cirrhotic portal hypertension which can be classified as pre hepatic, intra hepatic and post hepatic. One of the causes of idiopathic non cirrhotic portal hypertension is nodular regenerative hyperplasia (NRH). There are various disorders which are associated with NRH. We report a case of placental site trophoblastic tumor as a cause of NRH and portal hypertension. CASE DESCRIPTION/METHODS: A 36 years old Caucasian female with no known medical illness presented with progressive abdominal swelling of 5 months duration. She had a normal vaginal delivery to a healthy baby one year back. She gained almost 40 pounds since she started to notice abdominal distention. She has no history of alcohol intake or medication use. On physical examination she had markedly distended abdomen, with fluid wave and small umbilical hernia. She had Pitting edema of 3+ in the extremities. Investigations revealed that the platelet count was 114, Sodium 124, ALT 41, AST 46 and albumin 2.6. Otherwise white cell count, hemoglobin, bilirubin, PT and INR values were within normal limit. Viral hepatitis panel and work up for other causes of chronic liver disease were unrevealing. Abdominal ultrasound showed ascites and mild splenomegaly. It also showed a normal liver echotexture, no ductal dilatation and a patent portal vein. Ascitic fluid analysis was suggestive of portal hypertension with SAAG of 2. Transjugular liver biopsy was suggestive of nodular regenerative hyperplasia. Portal pressure was elevated at 11 mmHg. On abdominal CT scan an incidental enlarged heterogeneous uterus with prominent sub endometrial and parametrial vessels was noted. Serum HCG was high (762). Subsequent hysteroscopy and endometrial biopsy revealed trophoblastic maturation suggestive of trophoblastic tumor. PET scan showed that the disease was limited to the uterus. The patient underwent total hysterectomy with bilateral salpingectomy. She was also treated with three cycles of chemotherapy. The patient experienced total resolution of here symptoms. Four months after her surgery the patient continues to be symptom free with resolution of ascites and leg swelling without any medications. DISCUSSION: Idiopathic non cirrhotic portal hypertension can be associated with a variety of conditions including hematologic, neoplastic, immunogenic and medication related. To our knowledge, this is the first report of nodular regenerative hyperplasia associated with trophoblastic tumor.

2332 Large Hepatic Arteriovenous Fistula Leading to Non-Cirrhotic Portal Hypertension and Associated Complications

INTRODUCTION: Portal hypertension (and its complications including bleeding, ascites, encephalopathy) often occur in patients with liver cirrhosis as a result of intrahepatic resistance to portal blood flow and increased portal collateral blood flow. Non-cirrhotic portal hypertension (NCPH) is less common, but can also lead to similar complications. Arteriovenous fistulas are an uncommon presinusoidal intrahepatic cause of NCPH. We present a rare case of a patient with a remote history of a liver biopsy who presented with new onset ascites and found on imaging to have a large hepatic arteriovenous fistula which was subsequently treated with embolization. CASE DESCRIPTION/METHODS: A 61 year old male with no significant past medical history presented to clinic for evaluation of progressively worsening abdominal and lower extremity swelling. Laboratory findings on presentation were remarkable for hemoglobin 7.4 g/dL, platelets 59,000/microliter, INR 1.6, Na 137mmol/L, Cr 1.56 mg/dL T bilirubin 2.4 mg/dL. Paracentesis fluid studies showed high serum-ascites albumin gradient (SAAG) and high protein without evidence of heart failure on echocardiogram. Magnetic resonance imaging of the abdomen showed a 6.3 × 4 centimeters hepatic arteriovenous fistula within segment VIII of the liver; the portal vein was noted to be patent. Liver biopsy showed focal perivenular and subsinusoidal fibrosis without bridging (fibrosis stage 1/6) and nodular regenerative hyperplasia changes with bands of hepatocyte atrophy without any evidence of cirrhosis. Right hepatic arteriogram demonstrated a dilated and tortuous vessel with early filling of the portal venous system and a large aneurysm at the communication point between the arterial and venous fistula. Successful embolization of the anterior segmental branch of the right hepatic artery was achieved. The patient’s hospital course was also complicated by upper gastrointestinal bleeding secondary to esophageal varices which required banding x5. DISCUSSION: The presence of portal hypertensive changes with minimal hepatic fibrosis on liver biopsy is best categorized as non-cirrhotic portal hypertension (NCPH). This patient manifested complications of portal hypertension including variceal bleeding and recurrent ascites secondary to a large hepatic arteriovenous fistula which likely formed following his liver biopsy. Closure of large arteriovenous fistulas can be beneficial in preventing worsening portal hypertension and associated potentially fatal complications.

Idiopathic noncirrhotic portal hypertension.

Cirrhosis is the most common cause of portal hypertension but there are many causes of noncirrhotic portal hypertension. Many of these etiologies may be diagnosed by liver biopsy. Idiopathic noncirrhotic portal hypertension is being increasingly diagnosed and has varied histopathological findings as well as overlapping definitions. Many of these histological changes can be subtle, thus making it a challenging diagnosis for the pathologist to make. This review summarizes the clinical aspects of idiopathic noncirrhotic portal hypertension and outlines the different definitions and histological features of the entity. In addition, pearls and pitfalls for the pathologist in making this diagnosis are included.

Long-Term Results after Diversion Surgery in Extrahepatic Portal Vein Obstruction.

Extrahepatic portal vein obstruction (EHPVO) is a frequent cause of noncirrhotic portal hypertension in children. The aim of this study is to analyze long-term results after diversion surgery. Retrospective review of EHPVO patients who underwent diversion surgery analyzing number of platelets, leukocytes, prothrombin activity, splenomegaly, and gastrointestinal bleeding 10 years after surgery. Thirty-three patients were evaluated, mostly males (64%) and presenting with gastrointestinal bleeding (46%). Mesoportal shunt (Rex) was performed in 19 patients, mesocaval in 7, distal splenorenal in 7, and proximal splenorenal in 3. While comparing mesoportal shunt to the other portosystemic shunts, an increase in platelets was found with every technique, but it was clearly higher in mesoportal shunt. The highest increase was 6 months after surgery (p = 0.0015) as well as prothrombin activity (p = 0.0003). Leukocytes level also increased without statistical significance. Spleen size (cm) and spleen size Z score (SSAZ) decreased significantly 6 months after mesoportal shunt (p = 0.0168). Before surgery, over 94% patients suffered gastrointestinal bleeding, which reduced significantly afterward with bleeding episodes in only four (12%) of them. Diversion surgery in EHPVO, especially mesoportal shunt of Rex, improves hepatic function (prothrombin activity), reduces hypersplenism (platelets, leukocytes, and spleen size), and decreases gastrointestinal bleeding episodes.

Non-cirrhotic portal hypertension: clinical case and literature review

Non-cirrhotic portal hypertension can be caused by many factors. The authors presented a clinical case of portal hypertension that was eventually determined to be caused by repeated microthrombotic events on the basis of myeloproliferative disease that probably happened during adult life and involved small and medium branches of the portal vein, thus resulting in the non-cirrhotic portal fibrosis (NCPF). The clinical manifestations included long-lasting splenomegaly, elevations of ALT, AST, gamma-glutamyl transpeptidase levels, and thrombocytosis. The peripheral stigmata of chronic liver disease were absent. The computed tomography scans revealed the early enhancement of the portal branches in the hepatic arterial phase. The patient was treated with propranolol and somatostatin and currently remains in a good condition. In the most cases, NCPF is misdiagnosed due to its rarity; therefore, clinicians should consider NCPF as a potential cause of non-cirrhotic portal hypertension.

A Case of Arterioportal Fistula Leading to Non-cirrhotic Portal Hypertension

Introduction: Arterioportal fistula (APF) is a rare cause of non-cirrhotic portal hypertension. This is a case of a hepatic artery-portal vein fistula presenting with recurrent variceal hemorrhage and ascites in a patient with no known liver disease. Case: A 61-year-old woman with aplastic anemia on cyclosporine, remote colon cancer status post sigmoid resection and workup notable for a percutaneous liver biopsy to evaluate a benign liver lesion as a possible metastasis 10 years prior to current presentation, initially presented with hematemesis following 2-3 weeks of increased abdominal girth and new lower extremity edema. EGD showed bleeding esophageal varices that were successfully band ligated. Over the next 6 weeks, the patient required multiple EGDs with banding for recurrent variceal bleeding, and she was then referred to our center for tranjugular intrahepatic portosystemic shunt (TIPS) evaluation. As part of the TIPS evaluation, an MRI identified a right hepatic lobe arteriovenous malformation between the right hepatic artery and portal vein with evidence of portal hypertension. Diagnostic paracentesis demonstrated a high serum ascites albumin gradient (SAAG) of 3.1. Transjugular liver biopsy with pressures was notable for a mean hepatic venous pressure gradient (HVPG) of 7.5 mmHg. The biopsy showed perisinusoidal fibrosis in zone three without cirrhosis, and intimal hyperplasia of the portal vein (arteriolization) consistent with arterioportal fistula (Figure 1). Rather than pursuing TIPS, we elected to perform coil embolization of the two right hepatic arteries supplying the fistula (Figure 2). Despite technical success, a small fistula persisted and repeat embolizations were required. Following these interventions, rebleeding has not occurred during the 1 year follow-up.Figure 1Figure 2Discussion: APFs are a rare cause of pre-hepatic non-cirrhotic portal hypertension, and can be either acquired or congenital. Acquired etiologies include penetrating hepatic trauma, splanchnic artery aneurysms, surgical complications, or they may occur spontaneously in the setting of cirrhosis or liver tumors. Small APFs can occur following percutaneous liver biopsies, but large APFs complicating a remote percutaneous biopsy, as in our case, are rare. APF should be considered in patients with non-cirrhotic portal hypertension and, when identified, treatment is embolization or surgery, not TIPS.Figure 3

Nodular Regenerative Hyperplasia

A 38-year-old man with a history of two kidney transplants as well as genotype 3A hepatitis C virus (HCV) without cirrhosis presented with hematemesis. The patient was a nonresponder to pegylated IFN and ribavirin. A transjugular liver biopsy 2 years prior revealed stage 0 fibrosis without portal hypertension (HVPG=2 mm Hg). The patient completed a 12-week course of daclatasvir, sofosbuvir, and ribavirin, and achieved viral eradication with SVR12. Following HCV eradication, the patient developed new-onset ascites. Abdominal ultrasound revealed nodular-appearing liver and moderate ascites. Diagnostic paracentesis showed SAAG > 1.1 with low total protein (< 1.0 g/dL) and no evidence of SBP. He presented after one episode of hematemesis and was found to have a hemoglobin of 6.7 g/dL (baseline 10 g/dL). Emergent EGD revealed new medium-sized esophageal varices that were banded. Despite octreotide infusion, he developed recurrent hematemesis on hospital day 2. Repeat EGD revealed evidence of recurrent variceal bleeding. Liver biopsy demonstrated sinusoidal portal hypertension (HVPG=13 mm Hg) and nodular regenerative hyperplasia of the liver. He underwent TIPS insertion for recurrent variceal bleed with significant reduction in portal pressure gradient and resolution of bleeding. Nodular regenerative hyperplasia (NRH) is a form of noncirrhotic portal hypertension (NCPH). Histologically, NRH is distinguished from other causes of NCPH by diffuse parenchymal regenerative nodules and an absence of or minimal fibrosis in the perisinusoidal/periportal regions. The exact pathophysiology is not clearly defined; however, a generally accepted hypothesis involves hepatocyte hyperplasia in response to chronic inflammation, vascular disease, or direct endothelial injury from drugs, autoantibodies, T-cells, malignancies, or viruses. Development of NRH typically occurs within 1 to 6 years of insult, with some cases reporting development of NRH as early as 5-6 months after exposure toxic agents (i.e. azathioprine, oxaliplatin). Treatment involves avoiding and/or discontinuing offending agents and management of portal hypertension. While NRH is a rare but known complication of solid organ transplantation, it has yet to be reported following successful HCV therapy with direct-acting antiviral agents. In conclusion, we report a novel case of clinically significant NCPH secondary to NRH that developed in a kidney transplant recipient immediately following eradication of HCV.

A Case of Hepatoportal Sclerosis in a Patient Receiving Thioguanine Treatment for ALL

Introduction: Hepatoportal sclerosis is a known cause of noncirrhotic portal hypertension. We present the case of a patient with ALL on active chemotherapy with mercaptopurine who presented with upper GI bleeding in the setting of portal hypertension and was later discovered to have hepatoportal sclerosis on liver biopsy. Case: A 20 year old male was seen in the adult GI clinic for management of newly diagnosed hepatosportal sclerosis. He had a history of ALL diagnosed at the age of nine and was started on intrathecal methotrexate, 6-mercaptopurine, vincristine, doxorubicin, and prednisolone. He was treated for approximately three years after which he went into remission. Prior to completing his chemotherapy, he developed splenomegaly and thrombocytopenia. This was initially attributed to chemotherapy and was monitored closely. Five years later, the patient was admitted to the hospital with hematemesis and bloody stools. An EGD showed grade I and II esophageal varices for which he underwent banding and a liver biopsy showed findings of hepatoportal sclerosis. A CT abdomen showed patent portal, renal, and hepatic veins and a repeat transjugular liver biopsy a few years later revealed findings of nodular regenerative hyperplasia. The patient tested negative for hepatitis A,B,C, parasitic diseases, and HIV and an autoimmune workup was negative for rheumatologic diseases, primary biliary cirrhosis, and autoimmune hepatitis. Discussion: There has been only one case report of an association of 6-mercaptopurine and hepatoportal sclerosis. We present a second case where the cause of hepatoportal sclerosis was attributed to mercaptopurine. Clinicians should be aware of mercaptopurine's short and long-term side effects in order to implement appropriate treatment early and prevent long-term complications

Encephalopathy Secondary to a Surgical Splenorenal Shunt

Case: A 56 year old female with history of portal hypertension(PH) secondary to scleroderma (CREST variant) complicated by recurrent esophageal variceal treated by a surgical splenorenal shunt and splenectomy six months prior to presentation was admitted for confusion. Vital signs were within normal limits. Physical exam was notable for mild encephalopathy and asterixis. Labs on admission showed alkaline phosphatase of 154 and ammonia of 288; CBC, BMP, and other liver enzymes were normal. A work up for infection including chest X-ray, urinalysis and blood cultures was negative. CT head was negative for any acute process. CT angiography demonstrated widely patent splenorenal shunt with a decrease in size of the portal vein and preferential drainage into the splenic vein; thus causing minimal hepatic clearance. Patient was started on lactulose and rifaximin with immediate improvement of symptoms. Few weeks later, patient underwent an endovascular splenorenal shunt closure by interventional radiology. Post procedural imaging showed patent portal vein flow. Discussion: Hepatic encephalopathy(HE) is a spectrum of reversible neuropsychiatry abnormalities in patients with liver dysfunction. The exact mechanism of HE is not well understood but thought to be due to an increase in the ammonia level. Typically, hyperarammonemia is seen in cirrhotic patients with PH. However, there several etiologies for non-cirrhotic portal hypertension (NCPH) exist. Pathophysiology behind NCPH is vascular in nature and attributed to endothelial cell lesions, intimal thickening, thrombotic obliterations, or scarring of the intrahepatic portal or hepatic venous circulation. One rare cause of NCPH is scleroderma, an autoimmune connective tissue disease with features including vascular injury and fibrosis of skin and various internal organs including the liver. The main treatment of NCPH is aiming to decrease PH; this can be done surgically by creating portosystemic shunts. In our case, a preferential flow into the shunt and away from the portal vein caused decrease hepatic clearance leading to HE. Although surgical shunts are a rare cause of hyperammonemic encephalopathy, this case highlights the importance of a thorough work up in patients presenting with new encephalopathy.