Non-cirrhotic Portal Hypertension Research Articles

Pregnancy with Non-cirrhotic Portal Hypertension: A Case Report

Pregnancy with non cirrhotic portal hypertension is an uncommon condition. Due to physiological changes in pregnancy, the patients with portal hypertension may exaggerate and produce life threatening complicationslike variceal haemorrhage and increased incidents of adverse maternal and fetal outcomes. Pregnancy also predisposes the patient to develop hepatic decompensation. So, management of such cases requires a multi-disciplinary approach. In this case report,21-year-old primigravida presented with known case of non cirrhotic portal hypertension at her 33 weeks of pregnancy with huge splenomegaly and history of hematemesis.The pregnancy ran uneventfully, and she underwent elective LSCS at 38+ weeks and gave birth of a live female baby, weighing 3kg was delivered. Post operative recovery was uneventful. The patient was discharged on the 4th post operative day. We want to focus upon different aspects of pregnancy with portal hypertensionto prevent complications and manage the case. J Rang Med Col. March 2024; Vol. 9, No. 1: 86-88

Case report: Primary myelofibrosis presenting with portal hypertension mimicking cirrhosis

Primary myelofibrosis (PMF) is an infrequent etiology of noncirrhotic portal hypertension (PH). In clinical settings, non-cirrhotic PH is often misdiagnosed as cirrhotic PH. This case report details a patient who exhibited recurrent esophageal variceal hemorrhage and was initially misdiagnosed with cirrhosis. Initially poised for liver transplantation, the patient’s liver biopsy revealed no significant cirrhosis but showed signs of extramedullary hematopoiesis (EMH). Following the accurate diagnosis of PMF, the patient underwent standard treatment, leading to an absence of recurrent gastrointestinal hemorrhage due to esophageal varices for nearly three years.

Hepatic Cirrhosis with Esophageal Varices: A Case Report

Background: In adults, portal hypertension is generally caused by hepatic cirrhosis, whereas in children it is more commonly caused by extrahepatic abnormalities with normal liver function. Portal hypertension causes hemodynamic abnormalities. Gastrointestinal bleeding is the most severe clinical manifestation of portal hypertension in both children and adults. Pathogenetically, increased pressure in the portal vein can be caused by increased vascular resistance and increased portal blood flow. The site of obstruction can be prehepatic (portal vein obstruction), intrahepatic (presinusoidal: eg congenital hepatic fibrosis; para sinusoidal: cirrhosis, hepatotoxic drug therapy, vitamin A hepatotoxicity; post sinusoidal: venocclusive disease) and/or post hepatic (Budd-Chiari syndrome, constrictive pericarditis). Case presentation: The study reports the results of observations of a case of a boy, FAA, aged 12 years and 2 months who came to the emergency room at Dr. M. Djamil General Hospital Padang with the main complaint of hematemesis and splenomegaly from physical examination. Non cirrhotic portal fibrosis is a cause  that is not uncommon in the population in the early second decade of life. Some children with non-cirrhotic portal fibrosis as adults can end up with end stage liver disease. Conclusion: Patients with noncirrhotic or cirrhotic portal hypertension can be assessed using the Child Pugh instrument as an instrument that is still used to determine the survival rate if patients with portal hypertension.

Non-cirrhotic portal hypertension in pregnancy due to schistosomiasis: A case series

The presence of non-cirrhotic portal hypertension in pregnancy poses a challenging clinical scenario as it predisposes women to several life-threatening complications such as variceal haemorrhage, splenic artery aneurysm, pulmonary hypertension and bacterial peritonitis. The haemodynamic changes in pregnancy along with the demands of a growing fetus may worsen the severity of pre-existing non-cirrhotic portal hypertension. In this case series, we discuss four cases of non-cirrhotic portal hypertension in pregnancy in a low to middle income setting and review the literature related to this condition.

Evaluation of the histologic and immunohistochemical (CD34, glutamine synthetase) findings in idiopathic non-cirrhotic portal hypertension (INCPH).

Idiopathic non-cirrhotic portal hypertension (INCPH) is a vascular disorder of uncertain origin. Diagnosis can be challenging on liver biopsy. Despite diverse histomorphologic findings documented in literature, studies on the frequency of these findings are lacking. This study aims to assess both the histomorphologic features and the immunoexpression patterns of CD34 and glutamine synthetase (GS) in liver biopsies and searched for their contribution to the pathologic diagnosis of INCPH. Hematoxylin-eosin, CD34, and GS-stained liver needle biopsy sections of 16 patients clinically diagnosed with INCPH were retrospectively analyzed. Histologic findings such as portal vein narrowing, obliteration, or loss were grouped as major findings, while portal vein herniation, hypervascularized portal tracts, and periportal abnormal vessels were grouped as minor findings, and their frequency were evaluated. Periportal endothelial CD34 stained areas were measured via ocular micrometer. The distribution of GS immunoexpression was evaluated. Eighteen healthy liver donor biopsies were evaluated as controls. In INCPH cases, 58% of portal tracts showed major findings, compared to 15% in the control group (p < 0.001). Minor findings were observed in 16% of INCPH cases and 7% of controls (p = 0.014). The number of portal tracts with histologic findings is significantly higher in INCPH than in control liver biopsies. Abnormal portal tract distribution, like being close to each other, was seen in 75% of INCPH cases but not in controls (p < 0.001). Nodular regenerative hyperplasia (NRH) was present in 31% of cases. Periportal CD34 expression was higher in INCPH, and affected areas were larger than in controls (p < 0.001). Irregular GS staining, i.e. GS staining with patchy distribution in zone 3, and/or periportal and zone 2 hepatocytes, was found in 62% of INCPH cases, while controls showed the usual pattern (p < 0.001). In the biopsy diagnosis of INCPH, in addition to the presence of major histologic findings and the amount of portal tracts displaying these features, the expression of endothelial CD34 in periportal areas, and irregular hepatocellular GS expression can also be considered as supporting feature.

Anaesthetic management of a case of non-cirrhotic portal hypertension with severe thrombocytopenia for elective cesarean section

Pregnancy with non-cirrhotic portal hypertension usually presents with portal hypertension due to Physiological hemodynamic changes associated with pregnancy, thereby putting the mother at risk of potentially dangerous complications like variceal bleeding and splenic aneurysmal rupture. Management of such cases requires a multidisciplinary approach involving obstetricians, gastroenterologist and, anesthesiologist Here through the case report of a 36-year-old primigravida with Non Cirrhotic portal hypertension with at 37 weeks gestational age in labor. We intend to focus upon different aspects of anesthetic management of pregnancy with portal hypertension and its complications.

Alterations in the fecal microbiota in patients with advanced cystic fibrosis liver disease after 6 months of elexacaftor/tezacaftor/ivacaftor

BackgroundCystic fibrosis associated liver disease (CFLD) carries a significant disease burden with no effective preventive therapies. According to the gut-liver axis hypothesis for CFLD pathogenesis, dysbiosis and increased intestinal inflammation and permeability permit pathogenic bacterial translocation into the portal circulation, leading to hepatic inflammation and fibrosis. Evaluating the effect of CFTR (cystic fibrosis transmembrane conductance regulator) modulation with elexacaftor/tezacaftor/ivacaftor (ETI) may help determine the role of CFTR in CFLD and increase understanding of CFLD pathogenesis, which is critical for developing therapies. We aimed to characterize the fecal microbiota in participants with CF with and without advanced CFLD (aCFLD) before and after ETI. MethodsThis is an ancillary analysis of stool samples from participants ages ≥12 y/o enrolled in PROMISE (NCT04038047). Included participants had aCFLD (cirrhosis with or without portal hypertension, or non-cirrhotic portal hypertension) or CF without liver disease (CFnoLD). Fecal microbiota were defined by shotgun metagenomic sequencing at baseline and 1 and 6 months post-ETI. ResultsWe analyzed 93 samples from 34 participants (11 aCFLD and 23 CFnoLD). Compared to CFnoLD, aCFLD had significantly higher baseline relative abundances of potential pathogens Streptococcus salivarius and Veillonella parvula. Four of 11 aCFLD participants had an initially abnormal fecal calprotectin that normalized 6 months post-ETI, correlating with a significant decrease in S. salivarius and a trend towards decreasing V. parvula. ConclusionsThese results support an association between dysbiosis and intestinal inflammation in CFLD with improvements in both post-ETI, lending further support to the gut-liver axis in aCFLD.

Transjugular Intrahepatic Portosystemic Shunt in Nonmalignant Noncirrhotic Portal Vein Thrombosis and Portosinusoidal Vascular Disorder.

Transjugular intrahepatic portosystemic shunt (TIPS) emerges as a key treatment for portal hypertension (PH) complications. While international guidelines provide clear indications for its use in cirrhosis, empirical knowledge is notably scarcer in non-cirrhotic PH, particularly in nonmalignant noncirrhotic portal vein thrombosis (NNPVT) and in patients with portosinusoidal vascular disorder (PSVD). Patients afflicted by these rare diseases exhibit distinct clinical profiles compared to their cirrhotic counterparts, often characterized by a younger age, predominantly preserved hepatic functionality even in cases of severe PH, and a higher propensity for extensive splanchnic thrombosis, which intricately complicates TIPS placement, posing unique challenges for its creation. The objective of this review is to synthesize existing literature on the effectiveness, safety, specific indications, and clinical outcomes of TIPS in adult patients with NNPVT or PSVD, focusing also on the technical challenges of TIPS insertion in the presence of portal cavernoma.

Splenic artery aneurysm rupture in a pregnant woman with hepatosplenic schistosomiasis: case report and literature review

BackgroundPregnancy is not uncommon in patients with non-cirrhotic portal hypertension. Rupture of a splenic artery aneurysm remains a rare complication, associated with a very poor maternal–fetal prognosis. Our aim is to report a case of ruptured splenic aneurysm and to show the maternal–fetal over-risks during the association of pregnancy and portal hypertension, even in non-cirrhotic patients.Case presentationWe report a case of a 34-year-old woman, pregnant at 24 weeks gestation, with non-cirrhotic portal hypertension due to hepatic schistosomiasis. She was hospitalized for variceal bleeding. Patient had undergone endoscopic variceal band ligation and no bleeding recurrence. An unexplained hypovolemic shock appeared during the hospitalization with the occurrence of an in utero fetal death. The fetus was delivered by vaginal delivery. Abdominal CT scan angiogram showed a splenic artery aneurysm rupture. The patient underwent an emergency laparotomy with ligation of the splenic artery associated with splenectomy. Postoperative course was simple. Management of portal hypertension was continued at discharge (diuretic, beta-blockers, and esophageal varices ligation).ConclusionThe association of pregnancy and portal hypertension remains a serious situation with a high risk of maternal–fetal complications. Splenic artery aneurysm rupture is one of the rare complications of this association with a very poor maternal–fetal prognosis. Open repair is the surgical treatment of choice with a non-negligible morbi-mortality.

Rare Complication of Chronic Pancreatitis: Chronic Portal Vein Thrombosis and Splenic vein thrombosis causing non cirrhotic portal hypertension

Splenic vein thrombosis is a well-established complication of chronic pancreatitis, while portal vein thrombosis is a rare complication. In this case, we present an interesting instance of chronic pancreatitis associated with both portal and splenic vein thrombosis, causing non-cirrhotic portal hypertension. A fortysix-year-old male patient presented to us with clinical features of chronic pancreatitis. He underwent a CECT abdomen, which showed chronic pancreatitis with portal vein and splenic vein thrombosis. Screening for thrombophilic conditions and malignancies were negative. An upper gastrointestinal endoscopy showed fundal varices. Chronic portal vein thrombosis and splenic vein thrombosis are rare complications of chronic pancreatitis, which can cause non-cirrhotic portal hypertension.

Hepatic Schistosomiasis with Abnormal Ultrasound Findings

Schistosomiasis is an endemic disease in many countries in Africa and the Middle East, and affects millions of individuals. When schistosome eggs are deposited in the liver, an inflammatory response occurs, and if this response becomes chronic, it can lead to non-cirrhotic portal hypertension. A 46-year-old female patient who was diagnosed with hepatic schistosomiasis who recently traveled to the Philippines presented to our hospital with a one-week history of abdominal pain and diarrhea. On transabdominal ultrasound, a reticular echogenic pattern and periportal wall thickening were noted. If the patient has a recent history of travel and characteristic findings on abdominal ultrasound, hepatic schistosomiasis should be suspected and can be diagnosed by detecting ova on liver biopsy.

Case report: Oxaliplatin-induced idiopathic non-cirrhotic portal hypertension: a case report and literature review

Oxaliplatin has become a widely used agent in neoadjuvant chemotherapy for gastrointestinal tract tumors and is an integral part of the therapeutic approach for managing colorectal cancer recurrences and metastases, resulting in a more favorable prognosis for patients. Nevertheless, oxaliplatin can give rise to idiopathic non-cirrhotic portal hypertension (INCPH). The emergence of INCPH can disrupt tumor chemotherapy and incite persistent adverse reactions in later stages, significantly complicating clinical management. Consequently, we have presented a case report of INCPH induced by oxaliplatin chemotherapy with the aim of advancing the diagnosis and treatment of this condition, with a particular focus on the clinical manifestations. This study has ascertained that the condition is primarily attributed to complications related to portal hypertension, such as gastrointestinal bleeding, splenomegaly, and hypersplenism. The pathological features primarily involve hepatic sinus dilation and congestion, portal obstruction, absence, stenosis, shunting, localized venous and perisinusoidal fibrosis, as well as hepatocellular atrophy. Treatment primarily concentrates on strategies typically employed for cirrhosis. Endoscopic ligation, sclerotherapy, and non-selective beta-blockers (NSBBs) can be selected to prevent and treat variceal hemorrhage. Transjugular intrahepatic portosystemic shunt (TIPS) and liver transplantation can also be chosen for severe cases. Notably, despite the timely discontinuation of oxaliplatin, most patients continue to experience disease progression, ultimately resulting in a poor prognosis due to either tumor advancement or the ongoing progression of portal hypertension. This emphasizes the importance for physicians to be aware of and consider the risk of INCPH when prescribing oxaliplatin.

Non-Cirrhotic Portal Hypertension: An Update of Diagnosis and Management

Portal hypertension is not the only caused by liver cirrhosis. Non-cirrhotic portal hypertension (NCPH) is characterized by elevated portal pressure in the absence of cirrhosis and is often underdiagnosed in daily clinical practice. The clinical manifestations of NCPH are similar to liver cirrhosis. The diagnosis of NCPH is still challenging due to the various underlying etiologies and often require either non-invasive or invasive examination. Laboratory examination, abdominal ultrasound, endoscopic procedure, hepatic venous gradient measurement, and liver biopsy have role in diagnosis of NCPH. The principle management of NCPH is to treat portal hypertension and its complications, such as prophylaxis and acute management of variceal bleeding, anticoagulants, surgery, splenectomy, and liver transplantation.

Liver Transplantation in a Woman with Mahvash Disease

SummaryMahvash disease is an exceedingly rare genetic disorder of glucagon signaling characterized by hyperglucagonemia, hyperaminoacidemia, and pancreatic α-cell hyperplasia. Although there is no known definitive treatment, octreotide has been used to decrease systemic glucagon levels. We describe a woman who presented to our medical center after three episodes of small-volume hematemesis. She was found to have hyperglucagonemia and pancreatic hypertrophy with genetically confirmed Mahvash disease and also had evidence of portal hypertension (recurrent portosystemic encephalopathy and variceal hemorrhage) in the absence of cirrhosis. These findings established a diagnosis of portosinusoidal vascular disease, a presinusoidal type of portal hypertension previously known as noncirrhotic portal hypertension. Liver transplantation was followed by normalization of serum glucagon and ammonia levels, reversal of pancreatic hypertrophy, and resolution of recurrent encephalopathy and bleeding varices.

Interventional Radiology and Surgical Treatment Options for Non-Cirrhotic Portal Hypertension

Interventional Radiology and Surgical Treatment Options for Non-Cirrhotic Portal Hypertension

Endoscopic variceal ligation in primary and secondary prevention of variceal bleeding: a retrospective study in Digestive Endoscopy Unit, University Hospital Joseph Raseta Befelatanana, Antananarivo, Madagascar

IntroductionEndoscopic variceal ligation (EVL) is a crucial procedure for the primary and secondary prevention of variceal bleeding. The objective of this study was to evaluate the efficacy and tolerability of EVL in the prevention of variceal bleeding.MethodsThis was a retrospective, single-center study over 8 years, from January 2013 to December 2020, including all patients who came for EVL in primary or secondary prevention.ResultsFifty-seven patients (male/female: 39/18) were included. The mean age of the patients was 40.02 ± 12.32 years (range: 19–68). Portal hypertension was secondary to cirrhosis in 13 patients (22.8%) and to a non-cirrhotic cause in 44 patients (77.2%). EVL was indicated for primary and secondary prevention in 5.3% and 94.7%, respectively. All patients had received propranolol with a mean daily dose of 108.07 ± 38.52 mg (extremes: 80–160). Eradication of varices was achieved in 33 patients (57.9%) with an average of 3.06 ± 0.70 sessions (extremes: 1–5) and an average duration of 10.12 ± 6.21 months (extremes: 1–24). Ten patients (17.5%) had variceal bleeding, and one patient (1.8%) died. There was no significant difference between patients with cirrhotic and non-cirrhotic portal hypertension in terms of varices eradication, variceal bleeding, and mortality. Gender (HR: 37.18; CI: 0.14–18.4; p = 0.009) and the number of previous bleeds (HR: 1.34; CI: 1.01–1.80; p = 0.041) were independent predictors of variceal bleeding during EVL. Dysphagia (73.7%) and retrosternal pain (78.9%) were the main adverse events after ligation.ConclusionEVL is an efficient technique to eradicate varices. Its tolerance is good with post-ligation signs that are rapidly regressive.

A Narrative Review on Non-Cirrohotic Portal Hypertension: Not All Portal Hypertensions Mean Cirrhosis.

Non-cirrhotic portal hypertension (NCPH), also known as idiopathic non-cirrhotic portal hypertension (INCPH) and porto-sinusoidal vascular disorder (PSVD), is a rare disease characterized by intrahepatic portal hypertension (IPH) in the absence of cirrhosis. The precise etiopathogenesis of IPH is an area of ongoing research. NCPH diagnosis is challenging, as there are no specific tests available to confirm the disease, and a high-quality liver biopsy, detailed clinical information, and an expert pathologist are necessary for diagnosis. Currently, the treatment of NCPH relies on the prevention of complications related to portal hypertension, following current guidelines of cirrhotic portal hypertension. No treatment has been studied that aimed to modify the natural history of the disease; however, transjugular intrahepatic porto-systemic shunt (TIPS) placement, shunt and liver transplantation are considerable symptomatic options. In this review, we discuss the heterogeneity of NCPH as well as its etiopathogenesis, clinical presentation and management issues. Starting from the assumption that portal hypertension does not always mean cirrhosis, cooperative studies are probably needed to clarify the issues of etiology and the possible genetic background of this rare disease. This knowledge might lead to better treatment and perhaps better prevention.

Focused panel sequencing points to genetic predisposition in non-cirrhotic intrahepatic portal hypertension patients in India.

Non-cirrhotic intrahepatic portal hypertension (NCIPH), a portal microangiopathy affecting small portal vein radicles, is a disease of Indian sub-continent. NCIPH appears to be a complex disease with interactions between inherited and acquired factors, though the exact pathophysiological mechanism is unknown. We aimed at investigating the genetic variants that might contribute to susceptibility to NCIPH. In this case-control study, we analyzed genes associated with microangiopathy-VWF-ADAMTS13 (von Willebrand factor and its cleavase enzyme-a disintegrin and matrix metalloprotease with thrombospondin type-1 motifs member 13) and alternative complement system vitamin B12 metabolismand withfamilial NCIPH. Eighty-four Indian patients with liver biopsy-proven NCIPH (cases) and 103 healthy controls (matched for residential region of India) were included in the study. Targeted next-generation sequencing (NGS) panel, comprising 11 genes of interest, was done on 54 cases. Genotyping of selected variants was performed in 84 cases and 103 healthy controls. We identified variants in MBL2, CD46 and VWF genes either associated or predisposing to NCIPH. We also identified a single case with a novel compound heterozygous mutation in MBL2 gene, possibly contributing to development of NCIPH. In this first of a kind comprehensive gene panel study, multiple variants of significance have been noted, especially in ADAMTS13-VWF and complement pathways in NCIPH patients in India. Functional significance of these variants needs to be further studied.

S3567 Systemic Mastocytosis - A Rare Cause of Non-Cirrhotic Portal Hypertension and Anchoring Bias

S3567 Systemic Mastocytosis - A Rare Cause of Non-Cirrhotic Portal Hypertension and Anchoring Bias

S3561 Idiopathic Non-Cirrhotic Portal Hypertension With Type B Hepatic Encephalopathy Due to Obliterative Portal Venopathy Secondary to Systemic Lupus Erythematosus and Mixed Connective Tissue Disease

S3561 Idiopathic Non-Cirrhotic Portal Hypertension With Type B Hepatic Encephalopathy Due to Obliterative Portal Venopathy Secondary to Systemic Lupus Erythematosus and Mixed Connective Tissue Disease