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.

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.

Resolution of idiopathic intracranial hypertension after sustained lowering of cerebrospinal fluid pressure

Idiopathic intracranial hypertension(IIH) is a syndrome of headache due to raised intracranial pressure(ICP) where the cerebrospinal fluid(CSF) is normal and there is no alternative pathology on imaging. The aetiology is unknown. This review questions many of the prevailing views regarding aetiology and treatment of IIH. It explores the concept that there is a vicious cycle of fluctuating raised ICP leading to secondary compression of the transverse sinuses and further elevation of ICP. It also raises the question as to whether this vicious cycle could be relieved by prolonged drainage of CSF as seen in Lumbar puncture induced low-pressure headache or alternatively a lumbar drain.

Cerebrospinal Fluid Diversion Procedures for Treatment of Idiopathic Intracranial Hypertension: Single Center Experience

Idiopathic intracranial hypertension (IIH) is a disorder characterized by increased intracranial pressure without any identifiable etiology with normal brain imaging and normal cerebrospinal fluid (CSF) content. CSF diversion procedures are commonly used for treatment if medical treatment failed. The aim of this study is to report our experience in treatment of IIH with lumboperitoneal (LP) and stereotactic guided ventriculoperitoneal (VP) shunts. The clinical data of 43 consecutive patients with IIH refractory to medical treatment and underwent CSF diversion procedures between 2009 and 2014 were analyzed. 29 patients underwent LP shunts and the remaining 14 patients underwent stereotactic guided Ventricular shunts. All patients underwent clinical, imaging and CSF manometry evaluation. 38 (88.4%) patients were female and the remaining 5 (11.6%) patients were male. The mean age was 27.2 years. The opening pressure was above 300 mm H2O in 26 (69.8%) patients. Headache (100%) and blurring and/or diminution of vision (81.4%) were the commonest clinical presentation. 36 (83.7%) patients reported recovery of their headache and 30 (69.7%) patients showed complete resolution of papilledema. The clinical outcome between both procedures was not significant. The incidence of perioperative complications (20.7% vs. 0%) and shunt revisions (27.6% vs. 7.1%) were higher in patients with LP shunt than patients with stereotactic Ventricular shunts. The results of this study demonstrate that both LP and Ventricular shunts are valid diversion procedures for treatment of IIH. Stereotactic guided Ventricular shunt has lower incidence of complications and revisions and seems to be safe, effective and feasible alternative procedure for treatment of IIH.

Intracranial hypertension as the primary symptom of malignant melanoma:A case report

BACKGROUND The etiological diagnosis of intracranial hypertension is quite complicated but important in clinical practice.Some common causes are craniocerebral injury,intracranial space-occupying lesion,subarachnoid hemorrhage,and hydrocephalus.When a patient presents with intracranial hypertension,the common causes are to be considered first so that other causes would be dismissed.With the morbidity lower than 9%,neuromelanin is very rare.Common symp-toms include nerve damage symptoms,epilepsy,psychiatric symptoms,and cognitive disorders.CASE SUMMARY We present a patient with melanoma which manifested with isolated intracranial hypertension without any other neurological signs.A 22-year-old male had repeated nausea and vomiting for 2 mo with Babinski sign(+)on both sides,nuchal rigidity,and subarachnoid hemorrhage.He had been diagnosed with melanoma and was given surgery and whole-brain radiation.Ultimately,the patient died 2 mo later.CONCLUSION Malignant melanoma should be taken into consideration in the differential diagnosis of intracranial hypertension.

Microvascular Changes in the Retrobulbar Optic Nerve in Idiopathic Intracranial Hypertension

Purpose : To examine the microvascular changes in the retrobulbar optic nerve in idio-pathic intracranial hypertension (PTC).Methods: Both optic nerves from a 29-year-old man with a two year history of PTC were examined histologically and morphometrically. A semi-automated image analysis system and paraphenylenediamine (PPD) stain were employed to resolve sufficiently the microvascular images for counts and measurement.Results: There were 150 vessels distributed in the optic nerves which revealed the following: The average lumen of the vessels in outer sectors were larger than those of the inner sector vessels (168. 17μm2vs. 46. 99μm2; p = 0. 0338;OD; and 251. 96μm2vs. 130.02μm2;p = 0.029;OS) while in the normal control optic nerve the outer and inner area lumens were reversed in size-differential, but this did not show a statistical difference. The thickness of the PTC optic nerve vessel walls in the outer sectors was also greater than that of the walls in the inner sectors (4. 95μm vs. 2. 67μm;

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.

Idiopathic Intracranial Hypertension in Sickle Cell Disease: A Paediatric Case Report

In this article, we present the case of a 12-year-old girl with sickle cell disease (SCD), who presented with the severe headache. She had bilateral 6th cranial nerve palsy and papilloedema. The common sickle cell-related vascular causes of headache were ruled out by neuro-imaging. She then had a lumbar puncture and was diagnosed with idiopathic intracranial hypertension (IIH). This case demonstrates that IIH can affect younger children with SCD and should form a part of differential diagnosis when investigating causes of headache in SCD.

Idiopathic Intracranial Hypertension in Pregnancy Treated with Serial Lumbar Punctures

Idiopathic intracranial hypertension (IIH) is a syndrome of elevated intracranial pressure with normal CSF composi-tion and no evidence of hydrocephalus or mass lesion. We describe the anesthetic management of a parturient with IIH who required multiple lumbar punctures during pregnancy and delivery secondary to worsening neurological symp-toms.

Brain edema and intracranial hypertension in fulminant hepatic failure:Pathophysiology and management

Intracranial hypertension is a major cause of morbidity and mortality of patients suffering from fulminant hepatic failure. The etiology of this intracranial hypertension is not fully determined, and is probably multifactorial, combining a cytotoxic brain edema due to the astrocytic accumulation of glutamine, and an increase in cerebral blood volume and cerebral blood flow, in part due to inflammation, to glutamine and to toxic products of the diseased liver. Validated methods to control intracranial hypertension in fulminant hepatic failure patients mainly include mannitol, hypertonic saline, indomethacin, thiopental, and hyperventilation. However all these measures are often not sufficient in absence of liver transplantation, the only curative treatment of intracranial hypertension in fulminant hepatic failure to date. Induced moderate hypothermia seems very promising in this setting, but has to be validated by a controlled, randomized study. Artificial liver support systems have been under investigation for many decades. The bioartiflcial liver, based on both detoxification and swine liver cells, has shown some efficacy on reduction of intracranial pressure but did not show survival benefit in a controlled, randomized study. The Molecular Adsorbents Recirculating System has shown some efficacy in decreasing intracranial pressure in an animal model of liver failure, but has still to be evaluated in a phase Ⅲ trial.

Intracranial Hypertension Syndrome in Systemic Lupus Erythematosus: Clinical Analysis and Review of the Literature

In order to better understand the clinical manifestation of systemic lupus erythematosus （SLE） with intracranial hypertension syndrome （IHS）, we analyzed the clinical features and treatment of a typical SLE patient with IHS. SLE is one of the most unpredictable autoimmune diseases involving multiple organ systems that is defined clinically and associated with antibodies directed against cell nuclei. IHS is an uncommon manifestation of neuropsychiatric SLE （NPSLE） and is characterized by an elevated intracranial pressure, papilledema, and headache with occasional abducens nerve paresis, absence of a space-occupying lesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents. IHS has been reported in a few sporadic cases in patients with SLE worldwide, but rarely has been reported in China. In this study, a 34-year-old female SLE patient with IHS was. reported and pertinent literature reviewed. The clinical presentation, image logical features, and investigatory findings were discussed.

Pharmacological potential of acetazolamide in traumatic intracranial hypertension

Traumatic brain injuries are an important cause of morbidity and mortality around the world. These types of lesions are often associated with increased intracranial pressure and cerebral edema, proper management of this can reduce tissue damage of the brain and improve brain perfusion. The use of acetazolamide is not indicated in guidelines for the management of intracranial hypertension, which is used to a great extent for the management of idiopathic intracranial hypertension. However, it is not yet known in the management of traumatic intracranial hypertension.

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.

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".

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.

Duodenal variceal bleeding secondary to idiopathic portal hypertension treated with transjugular intra-hepatic portosystemic shunt plus embolization: A case report

BACKGROUND Duodenal varices are a lesser-known complication with non-cirrhotic portal hypertension. We report a circuitous route from missed diagnosis of duodenal varices to correction. An extremely rare case of duodenal variceal bleeding secondary to idiopathic portal hypertension(IPH) is expounded in this study, which was controlled by transjugular intra-hepatic porto-systemic shunt(TIPS) plus embolization. CASE SUMMARY A 46-year-old woman with anemia for two years was frequently admitted to the local hospital. Upon examination, anemia was attributed to gastrointestinal tract bleeding, which resulted from duodenal variceal bleeding detected by repeated esophagogastroduodenoscopy. At the end of a complete workup, IPH leadingto duodenal varices was diagnosed. Portal venography revealed that the remarked duodenal varices originated from the proximal superior mesenteric vein. TIPS plus embolization with coils and Histoacryl was performed to obliterate the rupture of duodenal varices. The anemia resolved, and the duodenal varices completely vanished by 2 mo after the initial operation. CONCLUSION TIPS plus embolization may be more appropriate to treat the bleeding of large duodenal varices.

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.

Acute Epidural Hematoma Compressing the Dominant Sigmoid Sinus as an Unusual Cause of Intracranial Hypertension: Case Report and Review of Literature

Post traumatic dural sinus vein stenosis has been rarely described in pediatric population. We present a case of a 9-year-old child that had sustained a head injury after a fall from height causing an acute epidural hematoma compressing the dominant sigmoid sinus. The patient had developed sub acutely signs and symptoms of increased intracranial pressure. Prophylactic treatment with anticoagulants was initiated despite the presence of an intracranial bleeding. Clinical and radiological improvements were achieved. We had also reviewed the literature regarding this uncommon entity and discussed other existing diagnostic and therapeutic alternatives. Further gathering of information is essential in order to form a therapeutic protocol.