Optic nerve sheath fenestration: A second lease at sight

Purpose: To study the safety and efficacy of optic nerve sheath fenestration surgery in patients with optic disc edema due to different etiologies. Methods: Records of 18 eyes of 15 patients who underwent optic nerve sheath fenestration for vision threatening optic disc edema were reviewed retrospectively, and results were analyzed. Improvement of visual acuity was the main measure of outcome. Improved visual fields, resolution of optic disc edema, diplopia, and headache were other benefits that were observed. Results: Fifteen patients between 13 and 54 years of age were included in the study. Three patients underwent successive bilateral surgery. Idiopathic intracranial hypertension was the most common cause for optic disc edema and was found in 80% of the patients. Mean preoperative logMAR acuity was ?1.9789 ± 1.46270, which improved to ?0.9022 ± 1.23181 (p < 0.005) in the operated eye, and mean logMAR acuity of contralateral eye improved from ?1.3378 ± 1.50107 to ?1.0667 ± 1.33813 (p < 0.05). Conclusion: Early optic nerve sheath fenestration is an effective modality for treating optic disc edema due to a wide myriad of causes and helps resolve the associated symptoms.

Hipertensión intracraneal idiopática asociada a infección por COVID 19

Se presenta el caso de una paciente adulta joven con antecedente de ovario poliquístico e infección reciente por COVID 19 que inicia con cuadro de astenopia y visión borrosa junto con cefalea, se realiza fondo de ojo con papiledema bilateral, estudios de laboratorio y neuro imagen sin hallazgos positivos, también punción lumbar con presión de apertura elevada por lo que se diagnostica hipertensión intracraneal idiopática con posterior mejoría post punción.

The case of a young adult patient is presented with a history of polycystic ovary and recent infection by COVID 19 that starts with asthenopia and blurred vision along with headache, fundus examination with bilateral papilledema was performed, laboratory and neuroimaging studies without positive findings, also lumbar puncture with elevated opening pressure so idiopathic intracranial hypertension was diagnosed with subsequent post puncture improvement.

Recognition and progress of idiopathic intracranial hypertension / 中国医师杂志

Idiopathic intracranial hypertension (IIH) is a syndrome of unexplained increased intracranial pressure with normal cerebrospinal fluid and without organic brain lesions. The etiology and pathogenesis of IIH remain unclear, and IIH patients may develop irreversible visual impairment. At present, there are no guidelines and expert consensus on diagnosis and treatment of IIH in China. This review aims to introduce the pathogenesis, diagnosis and treatment of IIH, in order to help clinicians improve their understanding of the disease and to identify, diagnose and treat IIH as early as possible, and improve the prognosis of patients.

STUDY OF PAPILLOEDEMA & ITS CAUSATIVE FACTORS IN A TERTIARY CARE HOSPITAL

To study the etiology of papilloedema in north indian population. Our hospital based retrospective study enrolled 50 patients of papilloedema, who fullfilled our inclusion criteria. Demographic details, chief complains and detailed history was taken from all the patients. All the patients underwent blood pressure measurement by a mercury sphygmomanometer, Visual Acuity testing, Slitlamp Biomicroscopy, IOP measurement, Fundus examination. Fundus photographs were obtained using a fundus camera. Other investigations included B-scan, MRI, MRV, CT and required blood investigations were done wherever necessary. Although papilloedema occured in a vast variety of age group but maximum patients (32%) were from the age group of >18-31 years and males (64%) were more affected than females according to our study. 66% of the patients had good vision with papilloedema. In our study all the patients had bilateral papilloedema and 54% of patients were diagnosed with early papilloedema. Idiopathic Intracranial Hypertension was the main etiology for papilloedema. In this study we concluded IIH (a diagnosis of exclusion) as the most common underlying etiology for papilloedema

Multidisciplinary management of idiopathic intracranial hypertension in pregnancy: case series and narrative review

Abstract Idiopathic intracranial hypertension (IIH) is a neurological condition characterized by raised intracranial pressure of unknown etiology with normal cerebrospinal fluid (CSF) composition and no brain lesions. It occurs in pregnant patients at approximately the same frequency as in general population, but obstetric and anesthetic management of the pregnancy and labor remains controversial. In this article we provide a multidisciplinary review of the main aspects of IIH in pregnancy including treatment options, mode of delivery and anesthetic techniques. Additionally, we report three cases of pregnant women diagnosed with IIH between 2012 and 2019 in our institution.

Idiopathic intracranial hypertension leading to bilateral optic atrophy in a patient with recent COVID-19 infection: a case report

Neurologic complications are common in patients hospitalisedwith COVID-19 infection. Most common complications are myalgias, headaches, encephalopathy and dizziness. Uncommon complications are stroke, motor and sensory deficits, seizures, ataxia and movement disorders. Multiple neuro-ophthalmological manifestations have also been reported in association with COVID-19. These complications may be the result of a range of pathophysiological mechanisms like hypoxic neuronal injury during active COVID-19 infection, RAS dysfunction, immune dysfunction and direct injury by the virus etc throughout the course of the disease. Here we reported a case of neuro-ophthalmic complication of Idiopathic intracranial hypertension (IIH) followed by bilateral optic atrophy in a middle-aged man with recent COVID-19 infection. He presented to the emergency with complaints of headache, dizziness and sudden painless bilateral diminution of vision for 3 days. His fundus examination was suggestive of bilateral papilledema, his MRI brain was normal and opening pressure of CSF was raised on lumbar puncture. His MRV was normal, there was no evidence of CSVT. He was started on steroids and acetazolamide. His headache improved but there was no improvement in visual acuity. Repeat fundus showed pale disc and MRI orbit was suggestive of bilateral optic atrophy.

Endovascular stenting for idiopathic intracranial hypertension with different types of venous sinus stenosis / 中华内科杂志

Objective:To evaluate the efficacy of endovascular stenting of various types of venous sinus stenosis in idiopathic intracranial hypertension (IIH).Method:Clinical, radiological, and manometric data before and after stenting in venous sinus stenosis were retrospectively analyzed in 99 IIH patients who were refractory to medical therapy or rapidly progressed between July 2004 to July 2019. The follow-up period was between 2.3 months to 11 years.Results:Our study enrolled 21 men (21.2%)and 78 women (78.8%) with average body mass index (BMI) 19.2-40.6(27.0±4.4) kg/m2 and median age 37 years. Before stent placement, the mean transverse sinus stenosis gradient was 1-59(26±8) mmHg. Patients with extrinsic stenosis were younger than those with intrinsic and mixed stenosis. In all cases, stenting was effective for papilledema. Fifty patients complained of headaches. Pulsatile tinnitus in twenty-eight patients completely alleviated after stenting. In one patient, replacement of stent did not improve symptoms, and a subsequent CSF diversion procedure was performed and effective.Conclusion:Irrespective of the type of stenosis, stenting of venous sinus stenosis is an effective treatment for IIH. Patients with persistent papilledema post-stenting and elevated transverse pressure pre-stenting should be followed closely as high risk of stenting failure may occur and further diversion procedure is needed.

Clinical and imaging features of idiopathic intracranial hypertension / 中南大学学报(医学版)

OBJECTIVES@#Idiopathic intracranial hypertension (IIH) is a syndrome that excludes secondary causes such as intracranial space-occupying lesion, hydrocephalus, cerebrovascular disease, and hypoxic ischemic encephalopathy. If not be treated promptly and effectively, IIH can cause severe, permanent vision disability and intractable, disabling headache. This study aims to explore the clinical and image features for IIH, to help clinicians to understand this disease, increase the diagnose rate, and improve the outcomes of patients.@*METHODS@#We retrospectively analyzed 15 cases of IIH that were admitted to Xiangya Hospital, Central South University, during January 2015 to September 2020. The diagnosis of IIH was based on the updated modified Dandy criteria. We analyzed clinical data of patients and did statistical analysis, including age, gender, height, weight, medical history, physical examination, auxiliary examination, treatment and outcome.@*RESULTS@#There were 10 females and 5 males. Female patients were 22 to 42 years old with median age of 39.5. Male patients were 27 to 52 years old with the median age of 44.0. The BMI was 24.14-34.17 (28.71±2.97) kg/m@*CONCLUSIONS@#IIH primarily affects women of childbearing age who are overweight. The major hazard of IIH is the severe and permanent visual loss. Typical image signs have high specificity in IIH diagnosis. Prompt diagnosis and effective treatment are significantly important to improve the outcomes of patients.

Hipertensión intracraneal idiopática asociada a nitrofurantoína. Caso clínico / Idiopathic intracranial hypertension associated with nitrofurantoin. Case report

Resumen Introducción: La Hipertensión Intracraneal Idiopática (HICI) es un síndrome neurológico caracterizado por un aumento de la presión intracraneal en ausencia de lesión estructural o hidrocefalia. Los síntomas incluyen cefalea, tinnitus pulsátil, oscurecimientos visuales transitorios y pérdida visual. Dentro de los signos destacan diplopía por parálisis del VI par, edema de papila y disminución de la agudeza visual. Los pacientes no tienen compromiso de conciencia ni signos neurológicos focales. La principal complicación es la pérdida visual que puede ser irreversible. La asociación entre HICI y nitrofurantoína (NTF) se reportó en 1974. Caso clínico: Mujer de 42 años, con sobrepeso, que desarrolló una HICI aproximadamente 18 meses posterior al inicio de nitrofurantoína profiláctica. Consultó por cefalea frontal, opresiva que aumentaba con la maniobra de Valsalva, asociada a disminución fluctuante de la agudeza visual y episodios de oscurecimiento. Al examen destacó edema de papila bilateral, sin déficit neurológico focal. La presión del líquido cefalorraquídeo (LCR) fue de 25,5 cm de agua. La resonancia magnética mostró signos de aumento de la presión del LCR, sin lesiones estructurales ni hidrocefalia. El cuadro se recuperó concomitantemente a la suspensión de la NTF y el uso de topiramato. No se constató daño visual permanente. Conclusiones: Se debe sospechar la HICI en mujeres en edad fértil con sobrepeso. Dentro de los gatillantes del síndrome destacan varios fármacos, entre ellos la NTF. El principal objetivo del tratamiento de la HICI es preservar la función visual.

Abstract Introduction: Idiopathic intracranial hypertension (IIH) is a syndrome characterized by increased intracranial pressure without a space occupying lesion or hydrocephalus. The symptoms are headache, pulsatile tinnitus, transient visual obscurations, and visual loss. Signs are diplopia caused by sixth cranial nerve paresis and papilledema with its associated loss of sensory visual function. The patient maintains an alert and oriented mental state, but has no localizing neurologic findings. The only major morbidity with IIH is visual loss. The association between IIH and nitrofurantoin was reported in 1974. Case: A 42 years old female, overweighed, who developed IIH 18 months after the start of prophylactic nitrofurantoin. She had frontal oppressive headache that increased with the Valsalva maneuver, fluctuant visual loss and transient visual obscurations. She had bilateral papilledema without localizing neurologic findings. The cerebrospinal fluid (CSF) pressure was 25.5 cm H2O. Magnetic resonance imaging showed signs of increased CSF pressure without structural lesions or hydrocephalus. IIH recovered with the withdrawal of nitrofurantoin and the use of topiramate. There was not permanent visual loss. Conclusions: It is recommendable to suspect IIH in obese women in the childbearing years. There are several drugs associated with IIH including nitrofurantoin. The main objective of treatment is to prevent visual loss.

Morphological assessment of lamina cribrosa in idiopathic intracranial hypertension

Purpose: Technological development of optic coherence tomography has enabled a detailed assessment of the optic nerve and deeper structures and in vivo measurements. The aim of this study was to compare the lamina cribrosa morphology of the optic nerve in idiopathic intracranial hypertension (IIH) and healthy individuals. Methods: The lamina cribrosa morphology of optic nerve in 15 eyes with IIH and 17 eyes of healthy individuals were compared. Four parameters such as Bruch membrane opening (BMO), lamina cribrosa thickness (LCT), prelaminar tissue thickness (PTT), and anterior lamina cribrosa surface depth (ALCSD) were retrospectively evaluated. Results: By enhanced depth imaging-optic coherence tomography (EDI-OCT), PTT and BMO were found to be significantly greater (574,35 � 169,20 ?m and 1787,40 � 140,87 ?m, respectively) in IIH patients than healthy individuals (187,18 � 132,15 ?m and 1632,65 � 162,58 ?m, respectively), whereas ALSCD was found to be significantly less in IIH patients (234,49 � 49,31 ?m) than healthy individuals (425,65 � 65,23 ?m). There was not a statistically significant difference regarding LCT between the IIH patients (238,59 � 17,31 ?m) and healthy individuals (244,96 � 15,32 ?m). Conclusion: Increased intracranial pressure causes morphological changes in lamina cribrosa. Assessment of lamina cribrosa with EDI-OCT is important for diagnosis and follow-up of patients with IIH. EDI-OCT is objective, reproducible, and cost-effective assistive imaging tool in IIH patients.

What is the optimal dose of acetazolamide in the treatment of idiopathic intracranial hypertension

@#Objective: Acetazolamide is preferred as the first-line drug for the medical treatment of idiopathic intracranial hypertension. In this study, the efficacy of different doses of the drug on visual functions; visual acuity, optic disc appearance- papilledema grade and visual field-mean deviation (VF-MD) were evaluated. Methods: The medical records of 73 patients diagnosed as idiopathic intracranial hypertension based on Modified Dandy Criteria and treated with acetazolamide who were on follow-up between 2010 and 2017 at the Neuro-ophthalmology Unit of Ege University Medical School, Department of Neurology were analyzed. Improvement in the visual functions at the end of the sixth month in three groups taking different doses of the drug; low (500, 750, 1000 mg/day), moderate (1500, 1750, 2000 mg/day) and high (3000, 4000 mg/day) were compared. Results: Improvement in visual acuity (p: 0.784), was not affected from different doses of the drug whereas papilledema grade (p: 0.014) and VF-MD (p<0.001) were affected. Binary comparisons revealed significant improvement in the high dose group when compared with the moderate and low dose groups both for the papilledema grade (lowhigh: p: 0.003, moderate-high: p: 0.024 ) and VF-MD (low-high: p<0.001, moderate-high: p: 0.001) Conclusion: Treatment with high doses of acetazolamide is associated with improvement in visual field defects and regression of optic disc edema in idiopathic intracranial hypertension.

A Rare Case of Idiopathic Intracranial Hypertension in Prepubertal Age

@#A 2-year-old girl who was under genetic follow up for developmental delay and dysmorphism was accidentally found to have bilateral optic disc swelling during screening examination. She showed response to optokinetic drum examination and the anterior segment examination was unremarkable. Optic disc swellings were seen in both eyes. Lumbar puncture shows high opening pressure of 50 cm H2O with unremarkable CSF analysis. MRI of brain was done and showed features in keeping with mild cerebral atrophy, with no evidence of hydrocephalus or space occupying lesion. She was diagnosed with idiopathic intracranial hypertension and oral acetazolamide 125mg bd was commenced. However, papilloedema persist despite medical therapy. Ventriculoperitonel shunt was inserted to reduce the csf pressure. This case report highlights the importance of considering idiopathic intracranial hypertension as a cause of optic disc swelling in pre-pubertal children because delay in diagnosis and treatment may permanently affect visual function especially in children.

A Case Study on Idiopathic Intracranial Hypertension Management and Outcome.

Background: Pseudo Tumor Cerebri (PTC), also knownby the name Idiopathic Intracranial Hypertension (IIH),is a disorder with increased intracranial pressure (ICP) andassociated headache, nausea, vomiting, transient visualobscuration, double-vision, and visual field defects.Material and methods: Thirty-four cases of idiopathicintracranial hypertension reported to our institute, all reportedpeople are female, and all are over twenty years of age, witha diagnosis of IIH according to the Modified dandy criteriawere included and treated medically with oral acetazolamideat a dose of 500 mg/day.Results: All patients are started on oral acetazolamide 250 mgtwice daily; all patients got resolved of fundus changes andnerve palsy with the therapy within three months of follow up.Conclusions: In this study, administration of low doses ofacetazolamide 250mg twice daily for a duration of 3 monthsfor all the patients, and there were no signs of recurrenceafter cessation of acetazolamide and in the further followup. Acetazolamide plays an important role in cases of IIH forits best outcome. Early diagnosis and early treatment withacetazolamide gave good outcomes in all cases of IdiopathicIntracranial Hypertension.

Lamina cribrosa surface position in idiopathic intracranial hypertension with swept-source optical coherence tomography

Purpose: The purpose of this study is to compare the thickness and depth measurements of the lamina cribrosa (LC) obtained using a swept-source optical coherence tomography (SS-OCT) device in idiopathic intracranial hypertension (IIH) patients and healthy subjects. Methods: This retrospective, cross-sectional observational study included 16 eyes with IIH and 20 control eyes. The LC measurements with serial horizontal B scans of the optic nerve head were obtained using SS-OCT (Topcon 3D DRI OCT Triton). The anterior lamina surface (ALS) depth, posterior lamina surface (PLS) depth, and LC thickness measurements were evaluated. Results: In patients with IIH, the mean ALS depth was 225.00 ± 58.57 ?m and the mean PLS depth was 449.75 ± 63.50 ?m. In the IIH control group, the corresponding values were 359.40 ± 105.38 and 570.10 ± 99.41 ?m (P < 0.05). The difference in LC thickness between the IIH and control subjects was not statistically significant. Conclusion: LC can be evaluated using an SS-OCT device. LC was displaced anteriorly in patients with IIH compared with normal controls. The assessment of LC level with SS-OCT in IIH cases is a valuable and reproducible adjunctive imaging method in terms of diagnosis and follow-up.

Progress in the application of optic nerve sheath fenestration in the treatment of intracranial hypertensive optic neuropathy / 国际眼科杂志(Guoji Yanke Zazhi)

@#Optic nerve sheath fenestration(ONSF), also known as optic nerve sheath decompression, was first proposed by Wecker. The procedure involves cutting incisions or windows in the retrabulbar optic nerve sheath to release the cerebral spinal fluid from the subarachnoid space of the optic nerve, reducing the pressure surrounding the optic nerve. With the development of surgical instruments and procedures, the safety of ONSF has been significantly improved with infrequent complications. Through the past three decades, more and more ophthalmologists realized that ONSF is an effective surgical treatment to stabilize or improve visual loss in idiopathic intracranial hypertension(IIH). Additionally, much progress in the use of ONSF has been made on secondary intracranial hypertension due to cerebral venous sinus occlusion, <i>Cryptococcal</i> meningitis, and intracranial mass or tumors. In this paper we review the application of ONSF in IIH, and secondary intracranial hypertension. From the review, it indicates ONSF is a good surgical alternative for ophthalmologists. It also indicates that ONSF can be used to prevent or halt progressive visual loss associated with intracranial hypertension, particularly when other treatment modalities fail to protect vision.

Landmark studies in neuro-ophthalmology

@#High-quality clinical evidence, derived from well-designed and implemented clinical trials, serves to advance clinical care and to allow physicians to provide the most effective treatments to their patients. The field of ophthalmology, including the subspecialty of neuro-ophthalmology, abounds with such high-quality clinical trials that provide Level 1 clinical evidence. This review article summarizes the research design, key findings, and clinical relevance of select monumental clinical studies in neuro-ophthalmology with the primary goal of providing the readers with the rationale for current standard of care of various neuro-ophthalmic diseases. This includes the Optic Neuritis Treatment Trial, Ischemic Optic Neuropathy Decompression Trial, Idiopathic Intracranial Hypertension Treatment Trial, Rescue of Hereditary Optic Disease Outpatient Study, and Controlled High-Risk Avonex® Multiple Sclerosis Study

Advancement on endovenous therapy for idiopathic intracranial hypertension / 中国脑血管病杂志

Idiopathic intracranial hypertension (IIH) is defined as elevation of cerebrospinal fluid pressure of unknown reason. Hie main symptoms of this disorder include headache and visual disturbances. This review highlights the role of venous outflow obstruction in the pathogenesis of IIH. The literatures on endovascular treatment of IIH were summarized. The unsolved problems and the future research directions were also discussed.

Successful management of peripapillary choroidal neovascular membrane secondary to idiopathic intracranial hypertension with intravitreal ranibizumab

Idiopathic intracranial hypertension (IIH) is a common cause of papilledema in young females and causes headache with transient visual loss. Severe visual loss occurs due to optic atrophy or peripapillary choroidal neovascular membrane (PCNVM). PCNVM in IIH has an incidence of 0.5% with a benign course in the majority of patients. Intravitreal Anti-vascular endothelial growth factor agents have anecdotally been used to manage these patients, with complete resolution reported in all cases after a single injection. Our case of IIH-associated PCNVM was treated with three injections of intravitreal ranibizumab with no recurrence at 6-month follow-up.

Pseudotumor cerebral asociado a hipovitaminosis A, B6 y D: A propósito de dos casos / Pseudotumor cerebri associated with hypovitaminosis A, B6 and D: About two cases

La hipertensión endocraneana idiopàtica se asocia infrecuentemente con la hipovitaminosis A y D. Se presenta el caso de una paciente femenina de 8 años con visión borrosa de 24 horas y papiledema bilateral. Resonancia magnética nuclear normal. Presión de apertura de líquido cefalorraquídeo: 260 mm^O. Presentó déficit de vitamina A y D, e inició un tratamiento sustitutivo. El segundo caso corresponde a un paciente masculino de 12 años con fiebre y odinofagia de 3 días. Con antecedente de glomerulonefritis y sobrepeso. Presentaba edema bipalpebral y papiledema. Tomografia axial computada de la órbita: aumento de líquido en la vaina de ambos nervios ópticos. Resonancia magnética nuclear: aracnoidocele intraselar. Presión de apertura de líquido cefalorraquídeo: 400 mm^O. Presentó déficit de vitamina D y B6, e inició el tratamiento sustitutivo. La elevación de la presión intracraneal desencadena mecanismos de compensación que, al fallar, pueden comprometer la vida o provocar graves discapacidades neurológicas. Reconocer la causa para un enfoque terapéutico preciso es clave para disminuir la morbimortalidad asociada a esta patología.

Idiopathic endocranial hypertension is infrequently associated with hypovitaminosis A and D. The case of an 8-year-old female with 24-hour blurred vision and bilateral papilledema is presented. Nuclear magnetic resonance was normal. Opening pressure of cerebrospinal fluid: 260 mm^O. She presented vitamin A and D deficiency and started replacement therapy. The second case corresponds to a 12-year-old male with fever and odynophagia of 3 days. History of glomerulonephritis and overweight. He had bipalpebral edema and papilledema. Computed tomography scan of the orbit: increase of fluid in the sheath of both optic nerves. Nuclear magnetic resonance: intrasellar arachnoidocele. Opening pressure of cerebrospinal fluid: 400 mmH2O. He presented vitamin D and B6 deficiency and started replacement treatment. The elevation of intracranial pressure triggers compensation mechanisms that, when they fail, can compromise life or cause serious neurological disabilities. Recognizing the cause for an accurate therapeutic approach is key to reduce the morbidity and mortality associated with this pathology.

Pseudotumor Cerebri Presenting Unilateral Papilledema Associated with Iron-deficiency Anemia

Pseudotumor cerebri is a relatively common pathology that is characterized by intracranial hypertension in the absence of mass lesions. It commonly affects young and obese women, and its presentation with visual loss and bilateral papilledema is well-described in the literature. We present a case of a 44-year-old, non-obese, female patient presenting with unilateral papilledema and iron-deficiency anemia. This case emphasizes this unusual presentation and the rare association with iron deficiency.

O pseudotumor cerebral é uma patologia relativamente comum, caracterizada pela hipertensão intracraniana na ausência de lesões causando efeito de massa. Frequentemente afeta mulheres jovens e obesas, e sua apresentação, com perda do campo visual e papiledema bilateral, é bem descrita na literatura. Nós apresentamos um caso de uma paciente de 44 anos, não obesa, com papiledema unilateral e anemia ferropriva. Esse caso destaca essa apresentação incomum e a rara associação com a deficiência de ferro.