Search

The term porto-sinusoidal vascular disorder (PSVD) was recently proposed to replace that of idiopathic non-cirrhotic portal hypertension (INCPH) to describe patients with typical histological lesions in absence of cirrhosis, irrespective of the presence/absence of portal hypertension (PH), and new diagnostic criteria were defined. The study aimed to compare the applicability between the diagnostic criteria of PSVD and those of INCPH. 53 patients affected by PSVD were enrolled. Biochemical, clinical, ultrasound and histological data, the presence and type of associated diseases were recorded in a database. According to the new criteria, histological data and signs of PH were divided into specific and non-specific. Percutaneous and transjugular biopsies were compared to establish the usability of the two methods for diagnostic purposes. In 85% of the patients the diagnosis of PSVD was obtained by applying the first criterion (25 had specific histological signs with specific signs of PH); one patient presented with specific histological signs but no PH. In 8 patients the diagnosis was obtained by applying the second criterion. 19% of patients had portal vein thrombosis. Finally, the prevalence of the various histological lesions was similar between the patients submitted to percutaneous and transjugular liver biopsy. The study confirms that the diagnostic criteria of PSVD lead to the inclusion of a greater number of patients than INCPH. [ABSTRACT FROM AUTHOR]

Background & Aims: Porto-sinusoidal vascular disorder (PSVD) encompasses a group of liver diseases with vascular abnormalities that can cause portal hypertension in the absence of cirrhosis. The new diagnostic criteria allow for coexistence with other liver diseases, however its relationship with chronic hepatitis B (CHB) remains unclear. This study aimed to assess HBV prevalence in a PSVD cohort and evaluate its clinical impact. Methods: This retrospective study was conducted on patients with PSVD at Hospital Clínic Barcelona. HBV serology was evaluated, and patients were categorized into HBV chronic infection, past infection, or no HBV exposure. Clinical characteristics and outcomes were compared. Results: We included 155 patients with PSVD. Prevalence of CHB and past HBV infection in patients with PSVD was higher than in the general population (5.8% vs. 0.5%, p

Purposeof Review: To review the clinical and histopathological features of obliterative portal venopathy (OPV). Recent Findings: OPV is one cause of non-cirrhotic portal hypertension and is part of the general group of vascular liver diseases termed porto-sinusoidal vascular disease (PSVD), along with nodular regenerative hyperplasia and incomplete septal fibrosis/cirrhosis. It is increasingly being found on liver biopsies in patients having abnormal liver tests and is reliably diagnosable only by liver biopsy. Radiologically, patients may appear to have a cirrhotic liver but typically their liver tests are normal or mildly abnormal, and the liver function is normal. Summary: Most cases of OPV are idiopathic but it can be seen in collagen vascular diseases, in the setting of portal vein thrombosis, in hematologic disorders, and immunodeficiency syndromes, and as a result of certain medications. Liver biopsy may show subtle histological findings so there must be a keener awareness amongst both clinicians and pathologists of this entity, as well as a discourse alerting the pathologist to the presence of portal hypertension. [ABSTRACT FROM AUTHOR]

Purpose of Review: Porto-sinusoidal vascular disease (PSVD) refers to a group of vascular diseases of the liver with lesions encompassing the portal venules and sinusoids, irrespective of portal pressure measurements and the presence or absence of cirrhosis. This article aims to provide an update on the pathologic, clinical, diagnostic, and therapeutic aspects of this underrecognized disease. Recent Findings: The diagnosis of PSVD requires a liver biopsy. Specific histology findings include nodular regenerative hyperplasia, obliterative portal venopathy or portal vein stenosis, and incomplete septal fibrosis/cirrhosis. The natural history of PSVD has not been elucidated, and screening guidelines for sequalae such as portal hypertension or portal vein thrombosis and the outcome of liver transplantation in these patients are unclear. Summary: Patients who have signs of portal hypertension despite unremarkable work-up for portal vein thrombosis and cirrhosis should be actively evaluated for PSVD. A heightened awareness of PSVD as a novel clinical entity should facilitate further collaborative studies and investigations. [ABSTRACT FROM AUTHOR]

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.

Background and Aims: In paediatrics, porto‐sinusoidal vascular disease (PSVD) is relatively unknown and probably underdiagnosed. We aimed to describe clinical phenotypes, histology and outcome of children diagnosed with PSVD. Methods: Retrospective multicentre study of children diagnosed with PSVD. Diagnosis of PSVD was based on histopathology reports; liver specimens were re‐evaluated by two expert liver pathologists. Results: Sixty two children diagnosed with PSVD (M/F = 36/26, median age 6.6 years, range 3.3–10.6), from 7 centres, were included. Thirty‐six presented with non‐cirrhotic portal hypertension, PH, (PH‐PSVD Group = 58%) while 26 had a liver biopsy because of chronic elevation of transaminases without PH (noPH‐PSVD Group = 42%). On histology review, the two groups differed for the prevalence of obliterative portal venopathy (more prevalent in PH‐PSVD, p = 0.005), and hypervascularised portal tracts (more common in noPH‐PSVD, p = 0.039), the other histological changes were equally distributed. At multivariate analysis, platelet count ≤185 000/mm3 was the only independent determinant of PH (p < 0.001). After a median follow‐up of 7 years (range 3.0–11.2), in PH‐PSVD group 3/36 (8%) required TIPS placement, 5/36 (14%) developed pulmonary vascular complications of PH, and 7/36 (19%) required liver transplantation. In noPH‐PSVD none progressed to PH nor had complications. Conclusions: Paediatric patients with PSVD present with two different clinical phenotypes, one characterised by PH and one by chronic elevation of transaminases without PH. PSVD should be included among the conditions causing isolated hypertransaminasaemia. On histology, the differences between the two groups are subtle. Medium‐term outcome is favourable in patients without PH; progression of the disease is observed in those with PH. [ABSTRACT FROM AUTHOR]

Abstract Background and Aim Primary 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. Methods Patients 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. Results Thirty‐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. Conclusions The 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.

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.

ABSTRACT BACKGROUND: Hepatoportal sclerosis HPS or obliterative portal venopathy (OPV), one of the differential diagnoses for non-cirrohtic portal hypertension, is characterized by the disappearance of the portal branches, portal and septal fibrosis, perisinusoidal fibrosis and regenerative nodular hyperplasia (RNH). It is a spectral disease that may progress to severe portal hypertension. Its etiopathogenesis is still little understood, especially in Brazil, it has been probably misdiagnosed due to its histopatological similarities with the hepatosplenic form of schistosomiasis. OBJECTIVE: To analyze the profile of patients with HPS in Northeastern Brazil and to demonstrate the pathological characteristics of HPS. METHODS: We retrospectively analyzed cases of OPV in liver biopsies and explants from a referral center for liver in Bahia - Brazil. The qualitative and quantitative analysis of the portal tracts and liver parenchyma was made so that comparisons could be done among the HPS findings of our population and the findings described by other authors. RESULTS: From the 62 patients identified with HPS, 42% were male, while 58% were female. The average age at diagnosis was 48.3 years. From this group, we analyzed the liver biopsy of 10 patients whose diagnosis of schistosomiasis could be ruled out. From these 100% (10/10) presented dense portal fibrosis and portal venous obliteration. Liver parenchymal atrophy was present in 60% (6/10) of the patients, sinusoidal dilation was present in 30% (3/10), the presence of portal septa occurred in 50% (5/10) and dense portal fibrosis in all patients analyzed. Nodular regenerative hyperplasia was found in 30% (3/10) of the patients. CONCLUSION: HPS seems to be neglected and misdiagnosed in Brazil, due to its similarities with schistossomiasis. In our study dense portal fibrosis, obliteration of the portal vein branches, parenchymal atrophy, sinusoidal dilatation and parenchymal nodular hyperplasia were the main histopathological findings and were similar to that described in other countries.

Background and Aim: Primary 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. Methods: Patients 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. Results: Thirty‐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. Conclusions: The 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. [ABSTRACT FROM AUTHOR]

Aims: Nodular regenerative hyperplasia (NRH) and obliterative portal venopathy (OPV), entities that comprise idiopathic non‐cirrhotic portal hypertension (INCPH), are under‐recognised diseases of uncertain aetiology and the diagnosis can be easily missed on liver biopsy. The expression of CD34 and von Willebrand factor (vWF) in liver sinusoidal endothelial cells (LSEC) and alpha‐smooth muscle actin (ASMA) in hepatic stellate cells (HSCs) is unknown in NRH and OPV. We sought to investigate the pathogenesis and potential immunomarkers that might aid in making the diagnosis of NRH and OPV. Methods and results: Immunohistochemical (IHC) staining for CD34, vWF and ASMA was performed in clinically and histologically well‐characterised NRH (n = 15) and OPV (n = 47) liver specimens. Among the 47 OPV cases, 37 (78.7%) had concurrent features of NRH. CD34 positive staining was mainly confined to small vessels in the portal tracts and LSECs in periportal areas, a finding similar to that in non‐NRH/OPV livers. However, expression of vWF in LSECs was positive in the compressed sinusoids of NRH and in a patchy or geographic pattern, particularly prominent in the perivenular areas and dilated sinusoids of OPV cases. HSCs were negative for ASMA in all NRH and OPV cases. Conclusion: Our findings indicate that NRH may be a subtle but common concurrent morphological feature in OPV. The aberrant expression of vWF in LSECs suggests that endothelial injury may play a role in the pathogenesis, which may thus aid in the recognition and diagnosis of NRH and OPV, particularly when confronted with otherwise apparent normal liver histology on needle biopsy. [ABSTRACT FROM AUTHOR]

In India, an unexplained enteropathy is present in a majority of non-cirrhotic intrahepatic portal hypertension (NCIPH) patients. Small intestinal bacterial contamination and tropical enteropathy could trigger inflammatory stimuli and activate the endothelium in the portal venous system. Groundwater contaminated with arsenic is an environmental factor of epidemic proportions in large areas of India which has similar consequences. Von Willebrand factor (a sticky protein) expressed by activated endothelium may promote formation of platelet microthrombi and occlusion of intrahepatic portal vein branches leading to NCIPH. Environmental factors linked to suboptimal hygiene and sanitation, which enter through the gastrointestinal (GI) tract, predispose to platelet plugging onto activated endothelium in portal microcirculation. Thus, NCIPH, an example of poverty linked thrombophilia, is a disease mainly affecting the lower socio-economic strata of Indian population. Public health measures to improve sanitation, provide clean drinking water and eliminate arsenic contamination of drinking water are urgently needed. Till such time as these environmental factors are addressed, NCIPH is likely to remain 'an Indian disease'. [ABSTRACT FROM AUTHOR]

Idiopathic non‐cirrhotic portal hypertension (INCPH) is a rare vascular liver disease that has attracted new interest in recent years. It is characterised by clinical signs of portal hypertension in the absence of cirrhosis or severe fibrosis and any known cause of portal hypertension. As much uncertainty exists about INCPH pathophysiology, and no definite diagnostic tests are available, liver biopsy is an essential tool for achieving a definite diagnosis. Unfortunately, the histological diagnosis of INCPH is not always straightforward, as the characteristic lesions are unevenly distributed, vary greatly in their severity, are often very subtle, and are not all necessarily present in a single case. Furthermore, specifically for the characteristic portal vessel changes observed in INCPH, the terminology and definition are ambiguous, which adds complexity to the already complex clinicopathological scenario. An international study group of liver pathologists and hepatologists pursued a consensus on nomenclature for the portal vascular lesions of INCPH. Such standardisation may assist pathologists in the recognition of such lesions, and will possibly facilitate further advancement in this field. [ABSTRACT FROM AUTHOR]

Introduction and aim. Non-cirrhotic idiopathic portal hypertension (NCIPH), also known as hepatoportal sclerosis (HPS) is a disease of uncertain etiology. However, many pathophysiological mechanisms has been postulated, including thrombophilia, chronic recurrent infections and exposure to drugs or toxins. In this context, it appears to be of multifactorial etiology or resulting from a portal vascular endothelium aggression. It is important to consider whether the use of dietary supplements and herbs can trigger or contribute to the occurance of HPS. We report a possible association of HPS with the consumption of herbal and / or dietary supplements.Material and methods. We describe two cases of HPS in patients without known etiology causes associated with this disease.Results. Both patients were females who were diagnosed with HPS following the consumption of Herbalife® products and putative anorexigenic agents in the herbal infusions. Image-based analysis and the assessment of the histopathological alterations found in the livers confirmed the diagnosis. The histopatological analysis of liver samples from both patients showed portal tracts enlarged by fibrosis with disappearance or reduction in the diameter of the portal vein branches. In many portal tracts, portal veins branches were replaced by aberrant thin-walled fendiforme vessels. The bile ducts and branches of the hepatic artery show normal aspects.Conclusion. After the exclusion of other etiologic factors and a comprehensive analysis of clinical history, consumption of Herbalife® products and anorexigenic agents was pointed-out as a puttative predisposing factor for the development of the disease.

Oliviero Riggio,1 Stefania Gioia,1 Ilaria Pentassuglio,1 Valeria Nicoletti,1 Michele Valente,2 Giulia d’Amati2 1Department of Clinical Medicine, Center for the Diagnosis and Treatment of Portal Hypertension, 2Department of Radiological, Oncological, and Pathological Sciences, Sapienza University of Rome, Rome, Italy Abstract: The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis. Keywords: idiopathic portal hypertension, obliterative portal venopathy, esophageal varices, splenomegaly

Background & Aims: Porto-sinusoidal vascular disorder (PSVD) encompasses a group of liver diseases with vascular abnormalities that can cause portal hypertension in the absence of cirrhosis. The new diagnostic criteria allow for coexistence with other liver diseases, however its relationship with chronic hepatitis B (CHB) remains unclear. This study aimed to assess HBV prevalence in a PSVD cohort and evaluate its clinical impact., Methods: This retrospective study was conducted on patients with PSVD at Hospital Clínic Barcelona. HBV serology was evaluated, and patients were categorized into HBV chronic infection, past infection, or no HBV exposure. Clinical characteristics and outcomes were compared., Results: We included 155 patients with PSVD. Prevalence of CHB and past HBV infection in patients with PSVD was higher than in the general population (5.8% vs . 0.5%, p <0.0001 and 20% vs. 9.1%, p <0.0001, respectively). Patients with CHB had a significant delay in PSVD diagnosis compared to those without CHB (11 [5-25] vs . 1 [0-3] years, p  = 0.002) and had a more advanced disease (MELD score 12 [9-17] vs . 9 [7-11], p  = 0.012) at the time of PSVD diagnosis. The clinical evolution of PSVD in patients with CHB was marked by a significantly higher transplantation rate at the last follow-up (33% vs. 4.1%, p  = 0.001)., Conclusions: Recognizing the coexistence of PSVD and CHB is important for timely diagnosis and optimal management, highlighting the potential benefits of specialized care for potentially improved outcomes., Impact and Implications: The new diagnostic criteria for porto-sinusoidal vascular disorder (PSVD) allow for coexistence with other liver diseases. The results of the present study highlight, for the first time, a non-negligible prevalence of chronic hepatitis B in the PSVD population that was previously unknown. Coexistence may challenge and delay the PSVD diagnosis and is associated with a more unfavorable clinical course. Our findings will increase awareness of this coexistence and improve PSVD diagnosis and management. Furthermore, the data will encourage new studies to determine the prevalence and clinical behavior of other chronic liver diseases that coexist with PSVD., Competing Interests: The authors who have taken part in this study declare they do not have anything to disclose regarding funding or conflict of interest with respect to this manuscript. Please refer to the accompanying ICMJE disclosure forms for further details., (© 2023 The Authors.)

Résumé: Le terme « maladie vasculaire porto-sinusoïdale » (MVPS) recouvre diverses entités caractérisées par des anomalies des veinules portales et/ou des sinusoïdes, précédemment nommées d'un point de vue histologique « hyperplasie nodulaire régénérative », « veinopathie portale oblitérante », « sclérose hépatoportale », « fibrose portale non cirrhotique » et d'un point de vue clinique « hypertension portale idiopathique », ou « hypertension portale intrahépatique non cirrhotique ». Les maladies ou états suivants peuvent être associés à une maladie vasculaire porto-sinusoïdale : infection VIH, certains médicaments, maladies génétiques, états prothrombotiques, maladies dysimmunitaires ou de système, maladie hématologique. La biopsie hépatique est essentielle pour le diagnostic de maladie vasculaire porto-sinusoïdale. Les principales complications de la maladie vasculaire porto-sinusoïdale sont la thrombose porte et les hémorragies due à l'hypertension portale. Les traitements comprennent la prophylaxie de la rupture de varices œsophagiennes en utilisant des bêta-bloquants et/ou des traitements endoscopiques, et rarement le TIPSS ou la transplantation hépatique pour les formes sévères. La thrombose porte est fréquente chez les malades atteints de cirrhose. Elle est partielle chez la majorité des malades et peut parfois régresser spontanément. La thrombose porte survient d'autant plus volontiers que la maladie du foie est sévère. Un traitement anticoagulant doit être envisagé chez les malades n'ayant pas de contre-indication définitive à une transplantation hépatique et chez ceux ayant une atteinte de la veine mésentérique supérieure. Le risque d'hémorragie ne semble pas augmenté chez les malades atteints de cirrhose traités par anticoagulants lorsque le taux de plaquettes est supérieur à 50 000/mm3. ..LAN-EN The term "porto-sinusoidal vascular disease" (PSD) encompasses various entities characterized by alterations of the portal venules and/or the sinusoids, called idiopathic portal hypertension, intrahepatic non-cirrhotic portal hypertension, obliterative portal venopathy, or nodular regenerative hyperplasia. Different conditions can be associated with PSD including prothrombotic states, immune-based diseases, HIV infection and antiretroviral treatment, other medications and prothrombotic factors. Liver biopsy is crucial for the diagnosis of PSD. The main complications of PSD are variceal bleeding and portal venous system thrombosis. Therapeutic options currently available for patients with PSD include prophylaxis for variceal bleeding using betablockers and/or endoscopic band ligation and TIPSS (transjugular intrahepatic portosystemic shunt) or liver transplantation for severe cases. Portal vein thrombosis (PVT) is common in patients with cirrhosis. PVT is partial in a majority of the patients in whom it develops and may spontaneously resolve in some of them. PVT is associated with features of more severe liver disease. Anticoagulation should be considered in patients with superior mesenteric vein thrombosis, and in patients who are potential candidates for liver transplant. The risk of bleeding does not seem to be increased in patients with cirrhosis receiving anticoagulation therapy, when platelet count is above 50 000/mm3. [ABSTRACT FROM AUTHOR]

Idiopathic non-cirrhotic portal hypertension is an under-recognized vascular liver disease of unknown etiology, characterized by clinical signs of portal hypertension in the absence of cirrhosis. By definition, any disorder known to cause portal hypertension in the absence of cirrhosis and any cause of chronic liver disease must be excluded to make a diagnosis of idiopathic non-cirrhotic portal hypertension. However, the diagnosis is often difficult because the disease resembles cirrhosis and there is no gold standard test. Liver biopsy is an essential tool: it is able to exclude cirrhosis and other causes of portal hypertension and it allows the identification of the characteristic lesions. Nonetheless, the histological diagnosis of idiopathic non-cirrhotic portal hypertension is not always straightforward, in particular by needle biopsy samples, because there is no pathognomonic lesion, but rather a variety of vascular changes which are unevenly distributed, very subtle, and not all necessarily identified in a single specimen. Pathologists should be able to recognize several patterns of injury, involving portal/periportal areas as well as parenchymal structures.The histological features of idiopathic non-cirrhotic portal hypertension are described in this review, focusing on their interpretation in needle biopsy specimens. [ABSTRACT FROM AUTHOR]

The components of the hepatic vascular system (hepatic arteries, portal and hepatic veins, sinusoids, and lymphatics) can be damaged by various types of injury. Each of the resulting conditions is rare, which has limited knowledge and awareness. In the last two decades, international collaborations have allowed to reach critical masses of data, which has driven significant progresses in understanding and management of vascular disorders of the liver. The present paper discusses definitions, denominations, and diagnosis of such vascular disorders with the exception of those affecting hepatic arteries. Evolving pathogenic or pathophysiologic views relevant to the clinical aspects are also overviewed. [ABSTRACT FROM AUTHOR]

Abstract: The significance of post‐transplant HLA DSA and chronic AMR in LT is an emerging field of study. Although OPV has previously been described as a histopathologic finding in DSA‐positive adult LT recipients, it was not included in the recent Banff criteria for chronic AMR. Our aim was to describe the association between OPV and chronic AMR in pediatric LT recipients. A retrospective review of 67 liver biopsies performed between November 2014 and April 2016 in 45 pediatric LT recipients identified four patients with OPV. Clinical status, liver biochemistry, the presence of DSA, and available non‐HLA antibody testing, as well as histopathologic features of chronic AMR, were assessed. All four patients with OPV had class II DSA and histopathologic features of chronic AMR based on the Banff criteria. Two patients were noted to have non‐HLA antibodies. Three patients are undergoing treatment with IVIG but have persistent DSA. Two patients have graft failure and are awaiting retransplantation. In conclusion, OPV is a histopathologic finding associated with chronic AMR in pediatric LT recipients. Further studies are needed to elucidate whether OPV is reversible and/or amenable to medical therapy. [ABSTRACT FROM AUTHOR]

Introduction and aim. Non-cirrhotic idiopathic portal hypertension (NCIPH), also known as hepatoportal sclerosis (HPS) is a disease of uncertain etiology. However, many pathophysiological mechanisms has been postulated, including thrombophilia, chronic recurrent infections and exposure to drugs or toxins. In this context, it appears to be of multifactorial etiology or resulting from a portal vascular endothelium aggression. It is important to consider whether the use of dietary supplements and herbs can trigger or contribute to the occurance of HPS. We report a possible association of HPS with the consumption of herbal and / or dietary supplements. Material and methods. We describe two cases of HPS in patients without known etiology causes associated with this disease. Results. Both patients were females who were diagnosed with HPS following the consumption of Herbalife® products and putative anorexigenic agents in the herbal infusions. Image-based analysis and the assessment of the histopathological alterations found in the livers confirmed the diagnosis. The histopatological analysis of liver samples from both patients showed portal tracts enlarged by fibrosis with disappearance or reduction in the diameter of the portal vein branches. In many portal tracts, portal veins branches were replaced by aberrant thin-walled fendiforme vessels. The bile ducts and branches of the hepatic artery show normal aspects. Conclusion. After the exclusion of other etiologic factors and a comprehensive analysis of clinical history, consumption of Herbalife® products and anorexigenic agents was pointed-out as a puttative predisposing factor for the development of the disease. [ABSTRACT FROM AUTHOR]

Background Nodular regenerative hyperplasia ( NRH) is a rare histological disorder associated with a wide variety of systemic diseases. Aims We aimed (i) to report the prevalence of NRH in a database of liver biopsies ( LBs) and the frequency of portal hypertension ( PHT) at diagnosis, and (ii) to investigate whether associated diseases and/or specific histological lesions, including abnormalities of the microvasculature, were related to PHT. Methods Patients with a histological diagnosis of NRH, referred by seven clinical departments, were retrospectively selected. Clinical, biological, radiological, haemodynamic and endoscopic data at diagnosis were recorded. LBs were reassessed for microvascular abnormalities. Results NRH was diagnosed in 4.4% of LBs ( n = 159, male: 52%, mean age: 54). Among patients referred for unexplained liver enzyme abnormalities, 15% had NRH. PHT was present at diagnosis in 45 patients (38%), including 13 with portal thrombosis; 65% of patients had an associated disorder. Obliteration of portal vein branches, observed in the LBs of 17 patients (11%), was significantly associated with PHT ( P = 0.02). Periportal angiomatosis, observed in 101 patients (63%), was associated with the absence of PHT ( P < 10−4). Conclusion We suggest that NRH is a frequent histological lesion in the setting of unexplained liver enzyme abnormalities. PHT is present at the time of diagnosis in 1/3 of patients regardless of the presence of associated disease. The frequency of periportal angiomatosis in NRH without obliteration of portal vein branches, and its association with the absence of PHT suggest that obstructive portal venopathy would not represent the most frequent mechanism involved in NRH. [ABSTRACT FROM AUTHOR]

Background: Liver involvement in Turner syndrome (TS) patients has been more clearly clarified in recent years. Most of the clinical manifestations in TS are asymptomatic and can be detected as liver test abnormalities; however, a few cases may present with end-stage liver disease and thus require liver transplantation (LT). To the best of our knowledge, only three cases undergoing LT for liver involvements in TS have been previously reported. Case presentation: A 30-year-old female successfully underwent living donor LT for liver dysfunction related to TS syndrome. The diagnosis of TS was established by a cytogenetic analysis at 16 years of age. She received several sessions of endoscopic therapy for recurrent esophageal varices, which was complicated by ascites and spontaneous bacterial peritonitis since 27 years of age. Radiological findings of her liver before LT chronologically showed the progression of atrophy with disturbance of the major portal inflow. And then, she was finally indicated for LT. Pathologic findings of the explanted liver showed vascular abnormalities, obliterative portal venopathy, which may have induced liver dysfunction with severe portal hypertension. The patient's postoperative course was uneventful. Conclusions: The clinicopathologic information obtained by the current case can provide an insight into understanding pathophysiological mechanisms of liver involvement in TS patients. TS patients presenting with severe liver atrophy and disturbance of the major portal inflow should be indicated for LT. [ABSTRACT FROM AUTHOR]

Background & Aims Obliterative portal venopathy without portal hypertension has been described by a single study in a limited number of patients, thus very little is known about this clinical condition. This study aimed to investigate the prevalence of obliterative portal venopathy and its clinical-pathological correlations in patients with cryptogenic chronic liver test abnormalities without clinical signs of portal hypertension. Methods We analysed 482 liver biopsies from adults with non-cirrhotic cryptogenic chronic liver disorders and without any clinical signs of portal hypertension, consecutively enrolled in a 5-year period. Twenty cases of idiopathic non-cirrhotic portal hypertension diagnosed in the same period, were included for comparison. Histological findings were matched with clinical and laboratory features. Results Obliterative portal venopathy was identified in 94 (19.5%) of 482 subjects and in all 20 cases of idiopathic non-cirrhotic portal hypertension: both groups shared the entire spectrum of histological changes described in the latter condition. The prevalence of incomplete fibrous septa and nodular regenerative hyperplasia was higher in the biopsies of idiopathic non-cirrhotic portal hypertension ( P = 0.006 and P = 0.002), a possible hint of a more advanced stage of the disease. The two groups also shared several clinical laboratory features, including a similar liver function test profile, concomitant prothrombotic conditions and extrahepatic autoimmune disorders. Conclusion Obliterative portal venopathy occurs in a substantial proportion of patients with unexplained chronic abnormal liver function tests without portal hypertension. The clinical-pathological profile of these subjects suggests that they may be in an early (non-symptomatic) stage of idiopathic non-cirrhotic portal hypertension. [ABSTRACT FROM AUTHOR]

Competing Interests: The authors have no potential conflicts of interest to disclose.

It is well established that portal hypertension can occur in the absence of cirrhosis, as reported in patients with immune disorders, infections and thrombophilia. However, similar histological abnormalities primarily affecting the hepatic sinusoidal and (peri)portal vasculature have also been observed in patients without portal hypertension. Thus, the term porto-sinusoidal vascular disorder (PSVD) has recently been introduced to describe a group of vascular diseases of the liver featuring lesions encompassing the portal venules and sinusoids, irrespective of the presence/absence of portal hypertension. Liver biopsy is fundamental for PSVD diagnosis. Specific histology findings include nodular regenerative hyperplasia, obliterative portal venopathy/portal vein stenosis and incomplete septal fibrosis/cirrhosis. Since other conditions including alcohol-related and non-alcoholic fatty liver disease, or viral hepatitis, or the presence of portal vein thrombosis may occur in patients with PSVD, their relative contribution to liver damage should be carefully assessed. In addition to histology and clinical diagnostic criteria, imaging and non-invasive tests such as liver and spleen stiffness measurements could aid in the diagnostic workup. The introduction of PSVD as a novel clinical entity will facilitate collaborative studies and investigations into the underlying molecular pathomechanisms encompassed by this term., Competing Interests: Conflict of interest The authors declare no conflicts of interest that pertain to this work. Please refer to the accompanying ICMJE disclosure forms for further details., (Copyright © 2022 The Authors. Published by Elsevier B.V. All rights reserved.)

Non-cirrhotic portal hypertension (NCPH) is characterized by the elevation of the portal pressure in the absence of cirrhosis. Obliterative portal venopathy (OPV) as a cause of NCPH is being increasingly diagnosed, especially after recent reports of its occurrence in patients with HIV using didanosine. Patients usually present with episodes of variceal hemorrhage and other features of portal hypertension including jaundice, ascites, encephalopathy and hepatopulmonary syndrome. Hepatic synthetic function is typically well preserved and the laboratory evaluation in OPV patients typically reveals only mild nonspecific hematological abnormalities chiefly related to hypersplenism. Its diagnosis remains a challenge and patients are often mistakenly diagnosed as having cirrhosis. Despite the increasing recognition of OPV, its etiology and pathogenesis are still unclear. A number of etiologies have been proposed including genetic predisposition, recurrent bacterial infections, HIV infection and highly active antiretroviral therapy, an altered immune response, hypercoagulability, and exposure to chemicals and certain medications. Histopathological evaluation remains critical in excluding cirrhosis and other causes of portal hypertension, and is the only way of definitively establishing the diagnosis of OPV. Clinicians should have a high index of suspicion for OPV in patients who present with variceal bleeding and splenomegaly and who do not have other features of cirrhosis. The purpose of this review is to summarize the known etiologies for OPV and its associated clinical aspects and correlations, and to also provide ample histophotomicrographs of OPV to aid in the diagnosis. It will also help raise awareness of this entity amongst pathologists and clinicians alike. [ABSTRACT FROM AUTHOR]

Nodular regenerative hyperplasia ( NRH) and hepatoportal sclerosis, also known as obliterative portal venopathy ( OPV), are two causes of non-cirrhotic portal hypertension ( NCPH). NCPH is an increasingly recognized entity that can be seen in association with collagen vascular diseases and with the use of medications such as azathioprine and didanosine, but oftentimes the etiology remains unidentified. We herein report a case of NCPH occurring due to OPV and NRH in a 64-year-old woman with myasthenia gravis ( MG), status post-thymectomy. Portal hypertension was diagnosed incidentally on computed tomography in the absence of predisposing factors. Extensive work-up to determine the etiology of any underlying liver disease was unrevealing. NRH and OPV were identified on liver biopsy. Subsequently, the patient had variceal bleeding that necessitated transjugular intrahepatic portosystemic shunt placement. A few similar cases of NCPH occurring in the setting of MG have been previously reported, suggesting that the immunological mechanisms involved in the pathogenesis of myasthenia may also have contributed to the development of NCPH. [ABSTRACT FROM AUTHOR]

Abstract: This review will provide an overview of the pathological features of non-cirrhotic portal hypertension (NCPH) and incomplete septal cirrhosis (ISC), focussing on practical diagnostic approaches and problems. Although many liver diseases may be associated with portal hypertension in the absence of advanced fibrosis, the term NCPH is most widely used to describe a spectrum of changes resulting from occlusion of small portal vein branches, also referred to as “hepato-portal sclerosis” or “obliterative portal venopathy”. Other histological manifestations of NCPH include parenchymal atrophy, nodular regenerative hyperplasia, formation of shunt vessels and foci of sinusoidal dilatation. In more severe cases, foci of parenchymal collapse may be associated with formation of delicate non-linking fibrous septa, corresponding to incomplete septal cirrhosis. Although ISC is generally considered to be part of the morphological spectrum of NCPH, it can also be regarded as part of the spectrum of true cirrhosis – either reflecting cirrhosis that is incompletely developed or partially regressed. [Copyright &y& Elsevier]