Outcomes of partial splenic embolization in patients with massive splenomegaly due to idiopathic portal hypertension

AIM To determine the outcomes of partial splenic em-bolization(PSE) for massive splenomegaly due to idiopathic portal hypertension(IPH).METHODS In this prospective study, we evaluated the charac-teristics and prognosis of consecutive patients with IPH who underwent PSE for all indications at a single medical center between June 2009 and January 2015. The inclusion criteria were: presence of hypersplenism, massive splenomegaly, and resultant pancytopenia. The exclusion criteria were: presence of other diseases causing portal hypertension. During the post-PSE period, the patients were hospitalized. All patients underwent abdominal computed tomography imaging 4 wk post-PSE to determine total splenic and non-infarcted splenic volumes.RESULTS A total of 11 patients, with median age of 33.27 ± 4.8 years, were included in the study. Mean spleen size was 22.9 cm(21-28 cm), and severe hypersplenismwas diagnosed in all patients before PSE. Post-PSE, leukocyte and platelet counts increased significantly, reaching peak levels in the second week with gradual decreases thereafter. Liver function tests did not exhibit significant changes during post-intervention follow-up. All patients developed post-embolization syndrome, and one patient experienced serious complications; all complications were successfully treated with conservative therapy and no death occurred. CONCLUSION Our findings showed that PSE has a lower complication rate than previously-reported surgical complication rates, which supports this intervention as a viable alternative for high-risk operable patients with severe hypersplenism.

Clinical characteristics of idiopathic portal hypertension

Idiopathic portal hypertension is one of the interesting causes of portal hypertension. Even in very developed medical centers, this disorder is still one of the most important misdiagnoses of clinical practice. To inexperienced physicians, presenting esophageal varices and upper gastrointestinal bleeding usually prompt an unfortunate diagnosis of cirrhosis. A heterogenous clinical presentation and progression of this disorder should be recognized by physicians, and management should be directed towards some specific problems confined to this disorder. Although a genetic basis and other factors are implicated in its pathogenesis, exact underlying mechanism(s) is (are) unknown. In this review, we discuss the heterogeneity of idiopathic portal hypertension, its etiopathogenesis, clinical presentation and management issues. With the expectation of an excellent prognosis, a practicing gastroenterologist should be aware that "not all varices mean cirrhosis".

Transjugular intrahepatic portosystemic shunt and splenectomy are more effective than endoscopic therapy for recurrent variceal bleeding in patients with idiopathic noncirrhotic portal hypertension

BACKGROUND Transjugular intrahepatic portosystemic shunt(TIPS),splenectomy plus esophagogastric devascularization(SED)and endoscopic therapy+non-selectiveβ-blockers(ET+NSBB)are widely applied in secondary prevention of recurrent gastroesophageal variceal bleeding in patients with liver cirrhosis.These different treatments,however,have not been compared in patients with idiopathic noncirrhotic portal hypertension(INCPH).AIM To compare the outcomes of TIPS,SED and ET+NSBB in the control of variceal rebleeding in patients with INCPH.METHODS This retrospective study recruited patients from six centers across China.Demographic characteristics,baseline profiles and follow-up clinical outcomes were collected.Post-procedural clinical outcomes,including incidence of rebleeding,hepatic encephalopathy(HE),portal vein thrombosis(PVT)and mortality rates,were compared in the different groups.RESULTS In total,81 patients were recruited,with 28 receiving TIPS,26 SED,and 27 ET+NSBB.No significant differences in demographic and baseline characteristics were found among these three groups before the procedures.After treatment,blood ammonia was significantly higher in the TIPS group;hemoglobin level and platelet count were significantly higher in the SED group(P<0.01).Rebleeding rate was significantly higher in the ET+NSBB group(P<0.01).Mortality was 3.6%,3.8%and 14.8%in the TIPS,SED and ET+NSBB groups,respectively,with no significant differences(P=0.082).Logistic regression analysis showed that mortality was significantly correlated with rebleeding,HE,portal thrombosis and superior mesenteric vein thrombosis(P<0.05).CONCLUSION In patients with INCPH,TIPS and SED were more effective in controlling rebleeding than ET+NSBB,but survival rates were not significantly different among the three groups.Mortality was significantly correlated with rebleeding,HE and PVT.

Proteomic analysis of the serum in patients with idiopathic pulmonary arterial hypertension

Idiopathic pulmonary arterial hypertension(IPAH) is a rare disease of unknown etiology.The exact pathogenesis of pulmonary arterial hypertension is still not well known.In the past decades,many protein molecules have been found to be in-volved in the development of IPAH.With proteomic techniques,profiling of human plasma proteome becomes more feasible in searching for disease-related markers.In present study,we showed the protein expression profiles of the serum of IPAH and healthy controls after depleting a few high-abundant proteins in serum.Thirteen spots had changed significantly in IPAH com-pared with healthy controls and were identified by LC-MS/MS.Alpha-1-antitrypsin and vitronectin were down-regulated in IPAH and may be valuable candidates for further explorations of their roles in the development of IPAH.

Peliosis hepatis as an early histological finding in idiopathic portal hypertension: A case report

Peliosis hepatis is a rare condition characterized by dilatation of hepatic sinusoids and blood-filled spaces in the liver mainly observed in subjects exposed to toxic substances or estrogens, which is frequently asymptomatic. Non-cirrhotic idiopathic portal hypertension （NCIPH） is also a vascular disease of the liver rarely observed in European countries, which is usually diagnosed only when the hemorrhagic complications of portal hypertension occur. We report a case of NCIPH in a young Caucasian male who was diagnosed with liver peliosis, showing ultrasonographic and endoscopic signs of portal hypertension four years after. A second biopsy was diagnostic for NCIPH. Even if the pathogenesis remains obscure, peliosis hepatis can be considered as an early sign of vascular disease of the liver, which may progress to more definite conditions.

Optical coherence tomography use in idiopathic intracranial hypertension

Idiopathic intracranial hypertension(IIH)is a condition in which elevated pressure in the cerebrospinal fluid can lead to optic nerve head(ONH)dysfunction and subsequent visual impairment.Physicians are currently limited in their ability to monitor and manage this condition,as clinical symptoms and exam findings are often delayed in response to changes in intracranial pressure.In order to find other biomarkers of disease,researchers are using imaging modalities such as optical coherence tomography(OCT)to observe microscopic changes in the eye in this condition.OCT can create 2-dimensional and 3-dimensional high definition images of the retina of the ONH and has been used to study various conditions such as glaucoma and multiple sclerosis.Numerous studies have used OCT in IIH as well,and they have shown that certain retinal layers and the ONH change in thickness and shape in both the short and long term with intracranial pressure changes.OCT is a promising modality for clinical and scientific evaluation of IIH as it is a noninvasive and practical tool to obtain in depth images.This review will discuss how OCT can be used to assess a patient with IIH,both before and after treatment,along with its limitations and future applications.

Novel therapy for idiopathic pulmonary arterial hypertension： Can hepatocyte growth factor be beneficial？

Idiopathic pulmonary arterial hypertension （IPAH） is a progressive, nearly fatal condition that until recently has had very few treatment options. Median survival time for untreated IPAH was 2.8 years without effective drug intervention. IPAH is characterized by deregulated proliferation of pulmonary arterial endothelial and intimal smooth muscle cells resulting in progressive pulmonary vascular remodeling and an increase in pulmonary arterial pressure. In order to alleviate their symptoms, anticoagulants, diuretics, calcium channel blockers and inotropic agents have been used to treat patients with PAH. Moreover, specific targeted therapies using prostacyclins,

Application value of electrocardiogram in the treatment and prognosis of idiopathic pulmonary hypertension

Objective: To evaluate the treatment and prognostic value of 12-lead electrocardiogram in idiopathic pulmonary hypertension (IPAH). Methods: A total of 200 patients with initial diagnosis of IPAH were included to analyze the correlation between ECG variables and hemodynamics.Univariate and multivariate proportional hazards regression analysis (Cox regression analysis) were used to analyze the changes of ECG parameters and determine the ECG parameters related to IPAH patients. Results: Partial correlation analysis showed that the amplitude of R wave in V1 lead was correlated with mPAP(r=0.343, P<0.001), and the sum of the amplitude of R wave in V1 lead and S wave in V5 lead was correlated with mPAP(r=0.504, P<0.001). The amplitude of R wave in V1 lead was correlated with mortality (HR 3.154, P<0.001), and the sum of the amplitude of R wave in V1 lead and S wave in V5 lead was correlated with mortality (HR 5.056, P<0.001). After 3 months of treatment,ECG parameters and hemodynamic status were improved. Conclusion: The specific 12-lead ECG parameters can reflect the improvement of the treatment of patients with idiopathic pulmonary hypertension and help to evaluate the prognosis of patients with idiopathic pulmonary hypertension.

Idiopathic Intracranial Hypertension without Papilloedema “Case Report”

Purpose: The purpose of this case report is to share and spread the awareness of diagnosing and managing idiopathic intracranial hypertension in atypical scenarios as in this case, who presented with multiple other medical conditions, and had no papilloedema. The presenting signs and symptoms of mere headache were misleading. The other medical conditions could have explained the patient’s headache. These included cervical spine osteoporosis, and middle ear otolith disease. This case is unique because she was found to have IIH without papilloedema, and despite having multiple cancers in the uterus, breasts, and recently the thyroid, she had no malignant cells in the CSF. The patient was treated for IIH, and her malignancies were treated separately by the corresponding physicians. Method: The patient was assessed in 2019, and was followed up closely till 2021, in collaboration with her neurologist, oncologist, and orthopedist. Result: The patient had IIH after her neurologist attempted a lumbar puncture, which showed high CSF pressure. Conclusion: Headache is a common symptom presented by patients of different ages and backgrounds. The absence of papilloedema does not exclude the diagnosis of IIH. When possible etiologies for headache are ruled out (e.g. middle and inner ear infections, anemia, intracranial tumors, glaucoma, etc.), chronic headaches that do not respond to medications, and present with visual field changes may require a lumbar puncture.

Prognostic value of the echocardiographic right/left ventricular end-diastolic diameter ratio in patients with idiopathic pulmonary arterial hypertension

Background Previous studies have shown that an echocardiographic right/left ventricular end-diastolic diameter ratio(RV/LV ratio)≥0.9 is an independent predictor of poor prognosis in patients with acute pulmonary embolism. The prognostic value of the RV/LV ratio in patients with idiopathic pulmonary arterial hypertension(IPAH) is still unknown. Methods We retrospectively enrolled 95 consecutive patients with newly diagnosed IPAH and 16 of them were reevaluated by echocardiography at 3-12 months following targeted therapy.Follow-up data were obtained by telephone interviews and review of the patients’ records.Results The RV/LV ratio was in parallel with the severity of World Health Orgnization(WHO) functional class and mean right atrial pressure.The RV/LV ratio was positively correlated with total pulmonary resistance(P P P 2 saturation(P P = 0.001),weight and absence of targeted therapy were independent predictors of death.No significant changes in the RV/LV ratio before and after targeted therapy were observed. A baseline RV/LV ratio≥0.84 or a further increase in the RV/LV ratio during targeted therapy indicated a poor prognosis. Conclusions The RV/LV ratio helps to assess the severity of IPAH and serves as an independent predictor of prognosis in patients with IPAH.

Shunting,optic nerve sheath fenestration and dural venous stenting for medically refractory idiopathic intracranial hypertension:systematic review and meta-analysis

Background:Cerebrospinal fluid(CSF)-diversion procedures have traditionally been the standard of treatment for patients with medically refractive idiopathic intracranial hypertension(IIH).However,dural venous sinus stent(VSS)placement has been described as a safe and effective procedure for the management of medically refractive IIH.We performed a meta-analysis comparing outcomes and complications of CSF-diversion procedures,VSS and optic nerve sheath fenestration(ONSF)for the treatment of medically refractive IIH.Methods:Electronic searches were performed using six databases from 1988 to January 2017.Data was extracted and meta-analysed from the identified studies.Results:From 55 pooled studies,there were 538 CSF-diversion cases,224 dural venous stent placements,and 872 ONSF procedures.Similar improvements were found in terms of postoperative headaches(CSF vs.VSS vs.ONSF:84%vs.78%vs.62%,P=0.223),papilledema(CSF vs.VSS vs.ONSF:71%vs.86%vs.77%,P=0.192),whilst visual acuity changes favored venous stenting(CSF vs.VSS vs.ONSF:55%vs.69%vs.44%,P=0.037).There was a significantly lower rate of subsequent procedures with venous stent placement(CSF vs.VSS vs.ONSF:37%vs.13%vs.18%,P<0.001),but other complication rates were similar(CSF vs.VSS vs.ONSF:13%vs.8%vs.14%,P=0.28).Subgroup analysis of lumbar-peritoneal vs.ventriculoperitoneal shunts found no differences in symptom improvements,complications and subsequent procedure rates.Conclusions:Our findings suggest that dural venous sinus stenting may be a viable alternative to traditional surgical interventions in patients who are refractory to medical treatment.

Diagnostic challenges in non-cirrhotic portal hypertension-porto sinusoidal vascular disease

Non-cirrhotic portal hypertension consists of a group of diseases characterized by signs and complications of portal hypertension,which differ from cirrhosis through histological alterations,hemodynamic characterization and,clinical outcome.Because of the similarities in clinical presentation and imaging signs,frequently these patients,and particularly those with porto-sinusoidal vascular disease(PSVD),are misdiagnosed as having liver cirrhosis and thus raising difficulties in their diagnosis.The most challenging differentiation to be considered is between PSVD and cirrhosis and,although not pathognomonic,liver biopsy is still the standard of diagnosis.Although they still require extended validation before being broadly used,new non-invasive methods for the diagnosis of porto-sinusoidal vascular disease,like transient elastography,contrast-enhanced ultrasound or metabolomic profiling,have shown promising results.Another issue is the differentiation between PSVD and chronic extrahepatic portal vein obstruction,especially now when it is known that 40%of patients suffering from PSVD develop portal vein thrombosis.In this particular case,once the portal vein thrombosis occurred,the diagnosis of PSVD is impossible according to the current guidelines.Moreover,so far,the differentiation between PSVD and sinusoidal obstruction syndrome has not been clear so far in particular circumstances.In this review we highlighted the diagnostic challenges regarding the PSVD,as well as the current techniques used in the evaluation of these patients.

A rabbit model of non- cirrhotic portal hypertension by repeated injections of E. coli through indwelling cannulation of the gastrosplenic vein

BACKGROUND: Non-cirrhotic portal hypertension is acommon cause of portal hypertension in developing coun-tries. To understand its etiopathogenesis we developed ananimal model by repeated portal endotoxemia inducedthrough the gastrosplenic vein.METHODS: Twenty-nine rabbits (1.5-2.0 kg) were divid-ed into control (group n = 13) and experimental ( groupn = 16) groups. Heat killed E. coli were injected throughan indwelling cannula into the gastrosplenic vein in pre-sensitized animals. The animals were sacriflced at 1, 3 and6 months.RESULTS: The mean portal pressure in group animalswas significantly (P < 0. 05) higher than in group at 1(17.5 ±3.4 vs 10.4±2.2 mmHg), 3 (17.8±1.3 vs7.2 +3.6mmHg), and 6 (19.8±3.1 vs 10.3±4.8 mmHg) months.Similarly, the mean splenic weight in group was signifi-cantly greater than in group (P <0.05). Histopathologi-cally, the spleen showed medullary congestion, hemosid-rin-laden macrophages and mild fibrosis. Histologically,the liver had normal parenchyma with mild portal lympho-cytic infiltrates and kupffer cell hyperplasia. No significantanomalies were detected by liver function tests.CONCLUSIONS: The rabbit model showed significantsplenomegaly with a persistent increase in portal pressureand mild fibrosis without hepatic parenchymal injury, quiteakin to non-cirrhotic portal fibrosis as seen in humans. Re-current intra-abdominal infection may play an importantrole in the pathogenesis of non-cirrhotic portal fibrosis.

Idiopathic Intracranial Hypertension Is Associated with Depression Regardless of Weight: A Controlled Study

Research Background: Idiopathic intracranial hypertension (IIH) is a common disease among obese women during their childbearing years, and it could be associated with depression. It is unclear whether obesity contributes also to depression among these patients. Research Objective: To evaluate patients with IIH for the existence of depression using the Hamilton Depression Rating Scale (HAM-D) questionnaire score, compared to age- and BMI-matched healthy control group. Methods: Controlled IIH patients were prospectively recruited from the neuro-ophthalmology clinic at Tzafon Medical Center, Poria. Following consent, patients were interviewed and answered the HAM-D questionnaire. A healthy age- and body mass index (BMI)-matched participants from the same population (control group), were also recruited and interviewed by the same investigator answering the same questionnaire. Demographics and BMI were documented in both groups. HAM-D score of >10 is significantly associated with depression. Results: Thirty-two and 28 from the patients and control group, respectively, had completed the study. Mean age was 28.6 ± 7.51 and 32.467 ± 8.22 years of both patient and control groups, respectively (P = 0.07). Mean BMI was 32.8 ± 6.87 and 32.1 ± 3.78 respectively (P = 0.621). The IIH group’s mean HAM-D scores was 11.62 ± 8.2 as compared to 3.179 ± 2.407 in the control group (P = 0.000). The most significant differences included the questionnaire items of insomnia initial, work and activities, somatic general and somatic symptoms general. Conclusion: IIH patients had significantly higher HAM-D scores compared to the control group. This score seems to be irrelevant to the weight of the patient. Psychological support in addition to the conventional treatment of IIH, is needed to improve the quality of life of these patients.

Transhepatic catheter-directed thrombolysis for portal vein thrombosis after partial splenic embolization in combination with balloon-occluded retrograde transvenous obliteration of splenorenal shunt

A 66-year-old woman underwent partial splenic embolization （PSE） for hypersplenisrn with idiopathic portal hypertension （IPH）. One week later, contrast-enhanced CT revealed extensive portal vein thrombosis （PVT） and dilated portosystemic shunts. The PVT was not dissolved by the intravenous administration of urokinase. The right portal vein was canulated via the percutaneous transhepatic route under ultrasonic guidance and a 4 Fr. straight catheter was advanced into the portal vein through the thrombus. Transhepatic catheter-directed thrombolysis was performed to dissolve the PVT and a splenorenal shunt was concurrently occluded to increase portal blood flow, using balloon-occluded retrograde transvenous obliteration （BRTO） technique. Subsequent contrast-enhanced CT showed good patency of the portal vein and thrombosed splenorenal shunt. Transhepatic catheter-directed thrombolysis combined with BRTO is feasible and effective for PVT with portosystemic shunts.

Coeliac disease as a potential cause of idiopathic portal hypertension:a case report

Idiopathic portal hypertension is a disorder that has various clinical features.It is mostly characterized by bleeding oesophageal varices,obvious splenomegaly,anaemia and,occasionally,jaundice and ascites.Here we described an interesting case of idiopathic portal hypertension caused by coeliac disease in a 38-year-old woman.By putting this patient on a gluten-free diet,liver function tests became normal and portal vein diameter returned to normal range.This report indicates that,in coeliac disease,repetitive stimulation by antigens along the portal vein—and immune responses to them—can result in the development of idiopathic portal hypertension.

An Unusual Cause of Irritability in a Single Ventricle Patient after Bidirectional Glenn Shunt

The differential diagnosis for irritability in children is broad.In patients with congenital heart disease,one must strongly consider cardiac etiologies such as low cardiac output or elevated central venous pressure(CVP).In patients with single-ventricle physiology,the second stage of palliation includes bidirectional Glenn,which involves anastomosis of the superior vena cava to the pulmonary artery resulting in volume offloading of the single systemic ventricle.Typically,early in the post-operative period,patients may experience a headache due to the acute increase in CVP,and symptoms improve over time.Idiopathic intracranial hypertension(IIH),also known as pseudotumor cerebri,is a rare neurologic disorder in children,characterized by raised intracranial pressure(ICP)in the absence of brain parenchymal lesions or cerebrospinal fluid(CSF)abnormalities.While the pathogenesis of IIH is unknown,early recognition and treatment of IIH are important to prevent permanent vision loss.There are only rare reports of IIH in patients with Fontan circulation.To our knowledge,we report the first case of IIH in a 2-year-old female after bidirectional Glenn.

Pediatric neuro-ophthalmology:not simply neuro-ophthalmology for small adults

Pediatric neuro-ophthalmology is a subspecialty within neuro-ophthalmology.Pediatric neuro-ophthalmic diseases must be considered separate from their adult counterparts,due to the distinctive nature of the examination,clinical presentations,and management choices.This manuscript will highlight four common pediatric neuro-ophthalmic disorders by describing common clinical presentations,recommended management,and highlighting recent developments.Diseases discussed include pediatric idiopathic intracranial hypertension(IIH),pseudopapilledema,optic neuritis(ON)and optic pathway gliomas(OPG).The demographics,diagnosis and management of common pediatric neuro-ophthalmic disease require a working knowledge of the current research presented herein.Special attention should be placed on the differences between pediatric and adult entities such that children can be appropriately diagnosed and treated.