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A 22-year-old female was referred to our hospital due to thrombocytopenia and esophagogastric varices (EGV) [LmF2CbRC1, Lg-c,F1RC0], therefore we performed endoscopic variceal ligation. Dynamic abdominal computed tomography showed giant portosystemic shunts (PSSs) from the left gastric vein to the superior vena cava and splenomegaly despite normal hepatic contour. Blood tests showed thrombocytopenia and hypoalbuminemia, but there were no abnormalities in hepatic function. Retrograde hepatic venography and transjugular liver biopsy were subsequently performed in order to further examine liver pathology. These examinations revealed anastomosis between the right and middle hepatic veins, with no features to suggest cirrhosis, therefore diagnosed as idiopathic portal hypertension. Splenectomy was performed for the treatment of hypersplenism with thrombocytopenia. Nine months after undergoing a splenectomy, the patient consulted a cardiologist due to exertional dyspnea with WHO functional class II. Echocardiography revealed a mild dilatated right ventricle (RV) with an estimated systolic pressure of 55 mmHg, consistent with pulmonary hypertension. Right heart catheterization determined an increased mean pulmonary arterial pressure of 40 mmHg and pulmonary vascular resistance of 7.5 wood units, but a normal pulmonary capillary wedge pressure value of 7 mmHg, resulting in the diagnosis of portopulmonary hypertension (PoPH). Administration of oral macitentan 5 mg/day was initiated. Exertional dyspnea and the findings from right heart catheterization were improved with macitentan 10 mg/day. No report exists of PoPH occurring within one year after splenectomy, however we report here a very rare case in which a splenectomy brought about the onset of PoPH., (© 2024. Japanese Society of Gastroenterology.)

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. [ABSTRACT FROM AUTHOR]

Rationale: Idiopathic noncirrhotic portal hypertension (INCPH) is a rare liver disorder with elevated portal pressure without cirrhosis, making diagnosis challenging. This case report presents a 46-year-old Chinese male with INCPH, highlighting the crucial role of liver biopsy., Patient Concerns: A 46-year-old male presented with persistent fatigue that lasted for 2 months and significantly worsened over the last 3 days. The patient described his fatigue as a profound lack of energy that persisted throughout the day, which progressively impaired his ability to perform daily activities and maintain his usual work responsibilities. He reported feeling exhausted even after light physical exertion, such as walking or standing for short periods. The severity of the fatigue also led to frequent short rests during the day, and he experienced difficulty concentrating and carrying out routine tasks. In addition, he noted a loss of appetite and mild discomfort in the upper abdomen. Given his previous history of abnormal liver function tests and a liver biopsy showing mild chronic liver damage, the patient was initially diagnosed with cirrhosis at a local hospital. This initial diagnosis caused significant emotional distress, as the patient experienced a state of panic and anxiety over the implications of having a progressive liver disease. The psychological burden was evident in his reported difficulty sleeping and persistent worry about his health and future., Diagnoses: Initial imaging suggested portal hypertension and cirrhosis, but a liver biopsy ruled out cirrhotic changes, confirming INCPH by excluding other causes such as chronic hepatitis., Interventions: The patient received symptomatic treatment (acid suppression, gastric and liver protection) and underwent a liver biopsy. Histological analysis confirmed INCPH, ruling out cirrhosis., Outcomes: After the definitive diagnosis, the patient's anxiety lessened. Fatigue and weakness improved with ongoing symptomatic treatment, and psychological support enhanced his overall well-being. His follow-up plan includes regular liver function monitoring, imaging for portal pressure changes, and potential anticoagulation therapy for thrombosis risks., Lessons: This case highlights the diagnostic difficulty of INCPH and underscores the importance of liver biopsy. Further research is needed to develop specific diagnostic tools and treatments for INCPH., Competing Interests: The authors have no conflict of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Since the Asian Pacific Association for the Study of the Liver (APASL) published guidelines on non-cirrhotic portal fibrosis/idiopathic portal hypertension in 2007, there has been a surge in new information, especially with the introduction of the term porto-sinusoidal vascular disorder (PSVD). Non-cirrhotic intra-hepatic causes of portal hypertension include disorders with a clearly identifiable etiology, such as schistosomiasis, as well as disorders with an unclear etiology such as non-cirrhotic portal fibrosis (NCPF), also termed idiopathic portal hypertension (IPH). This entity is being increasingly recognized as being associated with systemic disease and drug therapy, especially cancer therapy. An international working group with extensive expertise in portal hypertension was assigned with formulating consensus guidelines to clarify the definition, diagnosis, histological features, natural history, and management of NCPF/IPH, especially in the context of PSVD. The guidelines were prepared based on evidence from existing published literature. Whenever there was paucity of evidence, expert opinion was included after detailed deliberation. The goal of this manuscript, therefore, is to enhance the current understanding and help create global consensus on the issues surrounding NCPF/IPH., Competing Interests: Declarations. Conflict of interest: There is no conflict of interest to disclose by any of the authors., (© 2024. Asian Pacific Association for the Study of the Liver.)

Porto-sinusoidal vascular liver disease (PSVD) is a disorder that can cause portal hypertension without liver cirrhosis. TAFRO syndrome is a systemic inflammatory disorder with a background of immunological abnormalities. We report a case of TAFRO syndrome complicated by PSVD with portal hypertension. A 39-year-old man developed refractory ascites and esophageal varices. Lymph node histology revealed multicentric Castleman disease-like features. Intravenous methylprednisolone and tocilizumab therapy improved ascites and renal dysfunction, but the patient developed severe infections. The diagnosis of TAFRO syndrome in patients complicated by PSVD with portal hypertension encourages the consideration of appropriate treatment for these patients. [ABSTRACT FROM AUTHOR]

Backgrounds/aims: Splenectomy is the most frequently performed procedure as definitive management or as part of shunt surgery or devascularization in portal hypertension. Splenectomy is technically challenging because of the frequent coexistence of multiple collateral varices, splenomegaly, poor liver function, and thrombocytopenia. Early arterial ligation and late mobilization (EALDEM) is the traditional method for splenectomy in portal hypertension. Early spleen mobilization offers good control of the hilum. We aim to compare the effect of the early mobilization and delayed arterial ligation (EMDAL) technique with that of the conventional splenectomy technique in patients with portal hypertension., Methods: During the study period from September 2011 to September 2022, 173 patients underwent surgical intervention for portal hypertension at our institution. Among these patients, 114 underwent the conventional method of splenectomy (early arterial ligation and late splenic mobilization) while 59 underwent splenectomy with the EMDAL technique. Demographics were compared between the two groups. Intraoperative and postoperative outcomes were analyzed using the Mann-Whitney test in each group. A minimum follow-up of 12 months was performed in each group., Results: Demographics and type of surgical procedure were comparable in the two surgical method groups. Median blood loss was higher in the conventional group than in the EMDAL method. The median duration of surgery was comparable in the two surgical procedures. Clavien-Dindo grade III/IV complications were reported more frequently in the conventional group., Conclusions: The splenic hilum can be controlled well and bleeding can be minimised with early mobilization and delayed arterial ligation.

Background: Porto-sinusoidal vascular disease (PSVD) and portal vein thrombosis (PVT) are causes of portal hypertension characterized respectively by an intrahepatic and a pre-hepatic obstacle to the flow in the portal system. As PVT may be a consequence of PSVD, in PVT patients at presentation, a pre-existing PSVD should be suspected. In these patients the identification of an underlying PSVD would have relevant implication regarding follow-up and therapeutic management, but it could be challenging. In this setting ultrasonography may be valuable in differential diagnosis. The aim of the study was to use ultrasonography to identify parameters to discriminate between PSVD and "pure" PVT and then to suspect PVT secondary to a pre-existing PSVD., Methods: Fifty-three patients with histologically proven PSVD and forty-eight patients affected by chronic PVT were enrolled and submitted to abdominal ultrasonography with elastography by acoustic radiation force impulse (ARFI)., Results: ARFI was higher and superior mesenteric vein (SMV) diameter was wider in PSVD patients than in PVT patients. Thus, a prognostic score was obtained as linear combinations of the two parameters with a good discrimination capacity between PSVD and PVT (the area under the curve = 0.780; 95% confidence interval: 0.690-0.869)., Conclusions: A score based on ARFI and SMV diameter may be useful to suspect an underlying PSVD in patients with PVT and to identify a subgroup of patients to be submitted to liver biopsy., Competing Interests: Competing interest No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article., (Copyright © 2023 First Affiliated Hospital, Zhejiang University School of Medicine in China. Published by Elsevier B.V. All rights reserved.)

Background and Aim: 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., Materials and Methods: 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., Results: 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., Conclusions: The study confirms that the diagnostic criteria of PSVD lead to the inclusion of a greater number of patients than INCPH., Competing Interests: Conflict of interest The authors declare there is no conflict of interests., (Copyright © 2023 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

Caroli disease is a rare congenital malformation that predisposes to segmental cystic dilatation of the intrahepatic bile ducts. Banti syndrome is characterized by persistent splenomegaly due to chronic congestion, resulting in a low hematocrit and ultimately leading to pancytopenia. In this report, we describe a 29-year-old woman who presented with a >20-year history of hepatitis B surface antigen positivity and a >1-year history of recurrent fatigue and malaise. On examination, the patient had abdominal distension with marked splenomegaly (7 cm below the ribs) and ascites with tenderness of the abdominal muscles to palpation. A complete blood count showed a low white blood cell count, red blood cell count, and hemoglobin concentration. During the course of treatment, the patient developed multiple symptoms of pancytopenia and concomitant splenomegaly, and she was discharged after total splenectomy with good recovery. The combination of Banti syndrome and Caroli disease results in severe symptoms of portal hypertension., Competing Interests: Declaration of conflicting interestsThe authors declare that there is no conflict of interest.

Background and Aims: The efficacy of transient elastography (TE) in the differential diagnosis between porto-sinusoidal vascular disease (PSVD) and compensated cirrhosis has not been sufficiently studied. We aimed to investigate the diagnostic performance of TE and identify histological lesions associated with liver stiffness., Methods: We conducted a retrospective cohort study including patients with PSVD and cirrhosis (Child-Turcotte-Pugh class A) and healthy subjects. Both the PSVD and cirrhotic patients had at least one sign of PH. The area under the receiver operating characteristic curve (AUROC) was used for differentiation., Results: Ninety-two patients with PSVD (median age: 53 years, 33% male), 100 patients with compensated cirrhosis and 101 healthy subjects were included. The median TE-LSM in the PSVD patients (10.0 [7.0-13.0] kPa) was significantly lower than that in the cirrhotic patients (21.0 [15.0-28.0] kPa, p < .001) but was significantly higher than that in the healthy subjects (5.1 [4.6-6.0] kPa, p < .001). The AUROCs of TE-LSM for the discrimination of PSVD from the cirrhosis and healthy subjects were 0.886 (95% CI: 0.833-0.928) and 0.913 (95% CI: 0.864-0.949), respectively. The sensitivity and specificity to discriminate PSVD from compensated cirrhosis were 78.3% and 82.0%, respectively, at a cut-off of 13.6 kPa. Furthermore, portal fibrosis and aberrant cytokeratin 7 expression of centrilobular hepatocytes were significantly associated with higher TE-LSM (≥10.0 kPa)., Conclusion: TE-LSM can be used to differentiate PSVD from compensated cirrhosis. Pathological features in association with increased liver stiffness are identified., (© 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background and Aims: Porto-sinusoidal vascular disease (PSVD) has been described as the prominent pathology in liver explants of patients with cystic fibrosis (CF), but data outside the transplant setting are lacking. We aimed to investigate the prevalence of portal hypertension (PH) in CF-associated liver disease (CFLD) and develop an algorithm to classify liver involvement in CF patients., Methods: This is a cross-sectional study of consecutive paediatric and adult patients in a tertiary centre between 2018 and 2019, who underwent ultrasound, liver (LSM) and spleen stiffness (SSM) measurement. CFLD was defined according to physical examination, liver tests and ultrasound findings. PSVD was likely if there were PH signs in the absence of advanced chronic liver disease (CF-ACLD, LSM <10 kPa). A historical cohort was used to validate the prognostic significance of the new definitions., Results: Fifty (27.5%) patients met CFLD criteria. At least one sign of PH was found in 47 (26%) patients, but most (81%) had LSM <10 kPa and were likely to have PSVD; only 9 (5%) had CF-ACLD. PSVD and CFLD (LSM <10 kPa) co-existed in most (23/36) cases. In the historical cohort (n = 599 patients), likely PSVD and CFLD+PH were independently associated with a 2-fold and 3.5-fold increase in mortality compared to patients without PH, respectively. In 34 patients with SSM, values <21 and >50 kPa accurately diagnosed specific signs of PH., Conclusions: PSVD is the prevailing cause of PH in CF patients. We developed a new diagnostic algorithm based on clinical and elastosonography criteria to classify liver involvement in patients with CF., (© 2023 The Authors. Liver International published by John Wiley & Sons Ltd.)

Although cirrhosis is one of the most common causes of portal hypertension, noncirrhotic portal hypertension can result from hemodynamic perturbations occurring in the prehepatic, intrahepatic, and posthepatic circulation. Intrahepatic portal hypertension can be further subclassified relative to the hepatic sinusoids as presinusoidal, sinusoidal, and postsinusoidal. For many of these differential diagnoses, the etiology is known but the cause of idiopathic noncirrhotic portal hypertension, recently included in porto-sinusoidal vascular disease (PSVD), remains poorly understood. Herein, we discuss the diagnostic pathological features of noncirrhotic portal hypertension, with an emphasis on PSVD., (Copyright © 2023 Elsevier Inc. All rights reserved.)

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/mm 3 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., (© 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: The etiopathogenesis of idiopathic non-cirrhotic portal hypertension (INCPH) is so far poorly understood. Altered immunity, blood diseases, infections, congenital defects and drug exposure have been documented in a part of patients with INCPH owing to increased recognition of the disorder in patients with HIV, or various haematological disorders or autoimmune diseases. We aim to discuss the possible etiopathogenesis of INCPH., Case Presentation: We reported that a patient with intestinal infection of T. Marneffei and hyper-IgE syndrome, a group of rare primary immunodeficiency disorders, was finally diagnosed with INCPH for gastroesophageal variceal bleeding. The diagnosis was mainly based on histopathological features. Transjugular intrahepatic portosystemic shunt was performed and there was no recurrence of melena during the six-month follow-up., Conclusion: In the context of immunodeficiency, INCPH may associated with intestinal infections. Thus, screening for enterogenic infection and immunological disorders in patients with unexplained portal hypertension is necessary., (© 2023. The Author(s).)

Background: Porto-sinusoidal vascular disease (PSVD) is a novel nomenclature to describe non-cirrhotic portal hypertension and characteristic histology without portal vein thrombosis (PVT). It is a more inclusive definition than the previously well-recognized entity idiopathic non-cirrhotic portal hypertension. There is a paucity of data on PSVD patients., Methods: A total of 33 patients diagnosed with PSVD and portal hypertension (PH) between 2005 and 2021 were included. Data were retrieved from electronic medical record system and analyzed., Results: Of the 33 patients, 6 (18%) occurred in post-transplant allograft liver. After a median follow-up of 96 months (interquartile range, IQR [52, 139]), 14 deaths occurred (42%), 4 directly related to decompensated liver disease. The Kaplan-Meier survival estimates at 1, 5, and 10 years were 94%, 87% and 58%. PVT occurred in 10 patients (30%). The Nelson-Aalen cumulative risk estimate for PVT at 1, 5 and 10 years were 16%, 25% and 48%. The median model for end-stage liver disease and Child-Pugh score at initial presentation were 8 (IQR [7-12]) and 5 [5-6], and increased to 13 [8, 18] and 7 [5, 8], respectively, at the end of follow-up. Of the 11 patients who presented with splenomegaly and no specific sign of PH, 7 (64%) developed varices and 3 (27%) ascites at a median follow-up of 100 months., Conclusions: PSVD with PH is not a benign entity. Mortality, PVT and hepatic decompensation are common. Patients with PSVD must be closely monitored, including those who only have non-specific clinical signs (e.g., splenomegaly) of PH., (© 2023. Japanese Society of Gastroenterology.)

Background: Noninvasive assessment of high-risk varices (HRV) in idiopathic portal hypertension (IPH) is rare. The purpose of this study was to investigate the performance of spleen stiffness (SS) for evaluating the presence of HRV in IPH patients as compared the measurements in patients with hepatitis B virus (HBV)., Methods: A retrospective single-center study was performed to evaluate the performance of SS for assessing HRV in IPH and HBV-infected patients, in comparison with liver stiffness (LS), spleen stiffness-to-liver stiffness ratio (SS/LS), LS spleen-diameter-to-platelet-ratio score (LSPS), portal hypertension risk score (PH risk score) and varices risk score, by using upper gastrointestinal endoscopy (UGE) as the gold standard. Finally, 86 IPH and 102 HBV-infected patients were enrolled. UGE, two-dimensional shear wave elastography (2D-SWE) and laboratory data were collected, and noninvasive parameters were calculated. Analysis of receiver operating characteristic (ROC) curves was conducted to acquire the optimal area under the ROC curve (AUC) and cutoff value for predicting the presence of HRV., Results: In patients with HRV, the significantly different parameters between IPH (34.9%) and HBV-infected patients (46.1%) were as follows: spleen size (diameter 18.5 ± 3.9 cm vs. 20.8 ± 2.7 cm), SS (50.2 kPa vs. 42.9 kPa), LS (11.1 kPa vs. 18.3 kPa) and PT (prothrombin time 15.1 s vs. 16.7 s). No statistically significant differences were found in liver function, platelet counts, spleen thickness and flow volumes in the portal venous system (p > 0.05). The AUCs of SS were 0.98 and 0.96 for predicting the presence of HRV in IPH (44.0 kPa cutoff value; 0.93 sensitivity; 0.96 specificity) and HBV-infected patients (35.2 kPa cutoff value; 1.00 sensitivity; 0.82 specificity), respectively, which were significantly better than other parameters., Conclusion: SS shows the optimal overall performance for predicting the presence of HRV in IPH and HBV-infected patients, in comparison with other noninvasive parameters., (© 2023. The Author(s).)

Background: Portal hypertension is secondary to either cirrhotic or non-cirrhotic causes, and complicating pregnancy poses a challenge to the treating team. A systematic review was performed to determine maternal and perinatal outcomes in women with portal hypertension. Outcomes were compared among those with cirrhotic (CPH) with non-cirrhotic portal hypertension (NCPH) as well as non-cirrhotic portal fibrosis (NCPF) with extra-hepatic portal vein obstruction (EHPVO)., Methods: Medline and EMBASE databases were searched for studies reporting outcomes among pregnant women with portal hypertension. Reference lists from relevant papers and reviews were hand-searched for appropriate citations. Data were extracted to describe maternal complications, obstetric and neonatal outcomes. A random-effects model was used to derive pooled estimates of various outcomes, and final estimates were reported as percentages with a 95% confidence interval (CI). Cumulative, sequential and sensitivity analysis was studied to assess the temporal trends of outcomes over the period., Results: Information on 895 pregnancies among 581 patients with portal hypertension was included from 26 studies. Portal hypertension was diagnosed during pregnancy in 10% (95% CI 4-24%). There were 22 maternal deaths (0%, 95% CI 0-1%), mostly following complications from variceal bleeding or hepatic decompensation. Variceal bleeding complicated in 14% (95% CI 9-20%), and endoscopic interventions were performed in 12% (95% CI 8-17%) during pregnancy. Decompensation of liver function occurred in 7% (95% CI 3-12%). Thrombocytopenia was the most common complication (41%, 95% CI 23-60%). Miscarriages occurred in 14% (95% CI 8-20%), preterm birth in 27% (95% CI 19-37%), and low birth weights in 22% (95% CI 15-30%). Risk of postpartum hemorrhage was higher (RR 5.09, 95% CI 1.84-14.12), and variceal bleeding was lower (RR 0.51, 95% CI 0.30-0.86) among those with CPH compared to NCPH. Risk of various outcomes was comparable between NCPF and EHPVO., Conclusion: One in ten pregnancies complicated with portal hypertension is diagnosed during pregnancy, and thrombocytopenia is the most common complication. Hepatic decompensation and variceal bleeding remain the most common cause of maternal deaths, with reduced rates of bleeding and its complications reported following the introduction of endoscopic procedures during pregnancy. CPH increases the risk of postpartum hemorrhage, whereas variceal bleeding is higher among NCPH., (© 2022. Asian Pacific Association for the Study of the Liver.)

Introduction: In patients with portal hypertension (PH), the differential diagnosis between porto-sinusoidal vascular disease (PSVD) and cirrhosis is challenging. This study aims to evaluate the diagnostic accuracy of the SSM/LSM index in the diagnosis of PSVD., Methods: Prospective study of patients with PH and PSVD or cirrhosis. Transient liver and spleen elastography were performed and the ratio between spleen stiffness measurement (SSM) and liver stiffness measurement (LSM) was calculated. The relation of SSM/LSM with the diagnosis of PSVD was evaluated., Results: Forty-four patients with PSVD and 44 patients with cirrhosis were evaluated. Median age was 57.5 (IQR 49.0-64.5) years, 66.3% were males. In patients with PSVD, median SSM was 59.4 (33.5-77.7) kPa, median LSM was 6.2 (5.2-10.2) kPa and median SSM/LSM was 5.62 (3.15-9.68). In patients with cirrhosis, median SSM was 47.3 (24.3-60.3) kPa, median LSM was 27.8 (17.7-53.9) kPa and median SSM/LSM was 1.55 (1.06-3.24). The SSM/LSM AUROC was 0.940 (p<0.001). Using 2 as a cut-off, we obtained good sensitivity (86.5%), specificity (92.7%), and accuracy (89.7%) for the diagnosis of PSVD., Conclusion: The SSM/LSM index is useful in the differential diagnosis between liver cirrhosis and PSVD. Using the cut-off of 2 we achieved a good sensitivity and specificity for diagnosing PSVD., Competing Interests: Conflict of Interest None of the authors acted as Reviewer or Editor of this article. There are no conflicts of interest or financial ties to disclose. All authors disclosed no financial relationships relevant to this publication., (Copyright © 2022. Published by Elsevier Ltd.)

Oral contraceptive pills (OCPs) have a known prothrombotic effect. Obliterative portal venopathy (OPV) can be seen in patients with underlying hypercoagulability. We present a case of a 19-year-old female patient taking OCPs who presented with obstructive jaundice. Her main concern was pruritis. An extensive workup was done to reach a diagnosis but it came back negative. A liver biopsy showed OPV. This was thought secondary to her OCP use. Her OCPs were discontinued which resulted in a complete resolution of her symptoms and laboratory abnormalities. Cases with a direct relationship between OPV and OCP use are extremely rare. More studies are required to establish a correlation between OPV and OCPs. OPV should be considered in the differential diagnosis among patients with obstructive jaundice without an obvious cause, especially in patients taking OCPs. Treatment is stopping the OCPs with close follow-up to confirm disease resolution., Competing Interests: There is no conflict of interest to be reported in this case report by any of the authors., (Copyright 2021, Ashraf et al.)

Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are prototype noncirrhotic causes of portal hypertension (PHT), characterized by normal hepatic venous pressure gradient, variceal bleeds, and moderate to massive splenomegaly with preserved liver synthetic functions. Infections, toxins, and immunologic, prothrombotic and genetic disorders are possible causes in IPH, whereas prothrombotic and local factors around the portal vein lead to EHPVO. Growth failure, portal biliopathy, and minimal hepatic encephalopathy are long-term concerns in EHPVO. Surgical shunts and transjugular intrahepatic portosystemic shunt resolve the complications secondary to PHT. Meso-Rex shunt is now the standard-of-care surgery in children with EHPVO., (Copyright © 2019 Elsevier Inc. All rights reserved.)