Are Peripapillary Hyperreflective Ovoid Mass-like Structures with an Elevated Optic Disc Still a Diagnosis Dilemma?

BackgroundEndoscopic retrograde cholangiopancreatography (ERCP) is an essential procedure in the diagnosis and treatment of biliopancreatic diseases. The most common adverse event of ERCP is post-ERCP pancreatitis (PEP), which can sometimes be severe. Our previous study suggested that injecting ice water at the end of ERCP suppressed PEP, and we decided to investigate this effect in a multicenter randomized controlled trial.MethodsThis study is being conducted at eight hospitals in Japan starting in April 2022. Patients undergoing ERCP will be randomized to ice water group and control group. In the ice water group, 250 ml of ice water is injected toward the papilla at the end of ERCP. The next morning, a physical examination and blood tests are performed to evaluate for the development of pancreatitis. The goal is to have 440 cases in each group.DiscussionThe main cause of PEP is thought to be papilla edema. Cooling the papilla, as everyone naturally does at the time of a burn, is expected to prevent its inflammation and edema. Various methods to suppress PEP have been reported, but so far none of them are reliable. The method we have devised is very simple, easy, and safe. We hope that our study will change the world's ERCP common practice.Trial registration:UMIN000047528. Registered 20 April 2022, https://center6.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000053209

BackgroundThe kaolin induced obstructive hydrocephalus (OHC) model is well known for its ability to increase intracranial pressure (ICP) in experimental animals. Papilledema (PE) which is a predominant hallmark of elevated ICP in the clinic has not yet been studied in this model using high-resolution digital fundus microscopy. Further, the long-term effect on ICP and optic nerve head changes have not been fully demonstrated. In this study we aimed to monitor epidural ICP after induction of OHC and to examine changes in the optic disc. In addition, we validated epidural ICP to intraventricular ICP in this disease model.MethodThirteen male Sprague-Dawley rats received an injection into the cisterna magna containing either kaolin-Ringer’s lactate suspension (n = 8) or an equal amount of Ringer’s lactate solution (n = 5). Epidural ICP was recorded post-operatively, and then continuously overnight and followed up after 1 week. The final epidural ICP value after 1 week was confirmed with simultaneous ventricular ICP measurement. Optic disc photos (ODP) were obtained preoperatively at baseline and after one week and were assessed for papilledema.ResultsAll animals injected with kaolin developed OHC and had significant higher epidural ICP (15.49 ± 2.47 mmHg) compared to control animals (5.81 ± 1.33 mmHg) on day 1 (p < 0.0001). After 1 week, the epidural ICP values were subsided to normal range in hydrocephalus animals and there was no significant difference in epidural ICP between the groups. Epidural ICP after 1 week correlated with the ventricular ICP with a Pearson’s r = 0.89 (p < 0.0001). ODPs from both groups showed no signs of acute papilledema, but 5 out of 8 (62.5%) of the hydrocephalus animals were identified with peripapillary changes.ConclusionsWe demonstrated that the raised ICP at day 1 in the hydrocephalus animals was completely normalized within 1 week and that epidural ICP measurements are valid method in this model. No acute papilledema was identified in the hydrocephalus animals, but the peripapillary changes indicate a potential gliosis formation or an early state of a growing papilledema in the context of lateral ventricle dilation and increased ICP.

Purpose:The aim of this study was to investigate the role of inflammation in the pathogenesis of idiopathic intracranial hypertension (IIH) using the neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) as inflammation markers.Methods:The files of 33 IIH patients and 33 controls were screened for this retrospective study. For each patient, the NLR and PLR values were calculated using a single fasting blood sample. For both eyes, papilledema (PE) grades, best-corrected visual acuity (BCVA), retinal nerve fiber layer thickness (RNFLT), and ganglion cell layer thickness (GCLT) measurements were recorded along with the demographic data, including body mass index (BMI), and complete neurological and ophthalmological findings. Comparisons between the two groups and between the IIH patients with and without PE were made. The associations of NLR and PLR with all other parameters were analyzed independently from age, gender, and BMI.Results:NLR and PLR were higher in patients with IIH than controls (P < 0.05). They were also higher in patients with PE (P < 0.05) in the IIH group. NLR and PLR were found to be associated with BCVA (P < 0.001 and P = 0.023, respectively), global RNFLT (P = 0.004 and 0.012, respectively), RNFLT of the temporal quadrant (P < 0.001 and P = 0.042, respectively) and PE grade (P < 0.001 and P = 0.035, respectively).Conclusion:The NLR and PLR values and their associations with BCVA, RNFLT, and PE support the hypothesis that inflammation is a very important component of the pathogenesis of IIH.

Association between trastuzumab and papilledema

Alagille syndrome (AS) is a rare multisystem disease of the liver, heart, eyes, face, skeleton, kidneys, and vascular system. The occurrence of pseudotumor cerebri syndrome (PTCS) in patients with AS has been reported only exceptionally. Owning to its rarity and a mostly atypical presentation, the diagnosis and natural history of affected patients remain uncertain. We report an atypical case of PTCS in a 4-year-old boy with a known history of AS who presented with bilateral papilledema (PE) on a routine ophthalmological examination. Visual findings deteriorated after treatment with acetazolamide. Continuous intracranial pressure (ICP) monitoring was then utilized to investigate ICP dynamics. Successful treatment with resolution of PE was achieved after ventriculoperitoneal shunting but relapsed due to growth-related dislocation of the ventricular catheter. This report brings new insights into the ICP dynamics and the resulting treatment in this possibly underdiagnosed subgroup of PTCS patients. It also demonstrates that ventriculoperitoneal shunting can provide long-term improvement of symptoms for more than 10 years.

The aim of this study was not only to emphasize the role of clinical signs as well as ophthalmologic evaluation for accurate and differential diagnosis of papilledema (PE), but also to present an instructive algorithm that would help to eliminate unnecessary examinations and treatments. The files of 43 patients (ages 0-18) diagnosed with PE were retrospectively reviewed. The study included 25 patients from our pediatric neurology outpatient clinic, who were thought to have PE, and 18 patients, who were referred from the external centers to our hospital with a pre-diagnosis of PE. Of the 43 patients, 28 had PE, 8 had pseudopapilledema (PPE), and 7 had optic nerve pathologies (ONP). For patients who applied directly to our pediatric neurology unit, a margin of error of 8% was detected based on only a simple ophthalmologic examination and an evaluation of clinical findings. For the patients who were forwarded to our pediatric neurology unit from the external centers without examining any clinical findings and with no details, the margin of error was 72%. For patients with suspected PE, advanced ophthalmologic opinion is a necessary requirement before invasive radiological examinations are used. When the ophthalmologic evaluation is properly elaborated, the distinction can be made more clearly by using noninvasive methods. In order to determine the gold standard in terms of the methods used in the evaluation of patients who are not clinically diagnosed, new prospective studies with more patients should be planned.

Objectives The purpose of this study was to evaluate the changes in retinal nerve fiber layer (RNFL) thickness measured by optical coherence tomography (OCT) in idiopathic intracranial hypertension or pseudotumor cerebri (PTC). Patients and methods This cross-sectional comparative study involved 50 patients with PTC (group I) and 50 age-matched and sex-matched normal persons as controls (group II). Retinal nerve fiber thickness is measured using OCT for all studied population and then recorded and collected to be statistically analyzed. Results Visual acuity and best-corrected visual acuity were measured in both groups. Comparison between both groups shows a statistically highly significant difference (P Conclusion RNFL thickness measurements can provide important information regarding retinal axonal loss in patients with papilledema from PTC. Although axonal loss was documented in the chronic phase of papilledema, our findings, to be confirmed in future prospective studies, suggest a possible use of OCT during the treatment of PTC syndrome.

Medication-induced papilledema

For decades, treatment of compressive optic nerve neuropathy was conservative and involved the intravenous application of high-dose corticoids, which was combined with nerve growth factors in later years. If surgery was considered, transcranial and transfacial access to the orbital apex and the optic nerve were achieved using classical approaches. Over the past decade, endonasal endoscopic procedures have increasingly come into force, based on favorable experiences with functional endoscopic sinus surgery and as a result of innovative developments in endoscopy and image guidance. The purpose of this review is to address the latest literature in this field with regard to the indications, techniques, results, and complications associated with this procedure. There are basically two approaches for endoscopic optic nerve decompression: the extradural supraorbital “keyhole” approach, which provides access to the optic nerve canal roof and which is usually performed by neurosurgeons, and the endonasal endoscopic approach, which is widely used by otorhinolaryngologists. The development and application of endonasal endoscopic surgery was only possible with extensive knowledge of the topographic anatomy. The most important anatomical landmarks and topography have been described based on either cadaveric or CT scan studies, and include the opticocarotid recess, the distance between the optic nerve canal and the internal carotid artery, the ophthalmic artery, and the length of the optic nerve canal. There are two main indications for endonasal endoscopic optic nerve decompression: (1) traumatic optic nerve neuropathy (TON), and (2) optic nerve neuropathy from non-traumatic causes (nTON), such as tumorous conditions, inflammatory diseases, or idiopathic intracranial hypertension (IIH). In trauma cases, rates of postoperative visual improvement of 40–80% have been reported. Factors influencing the outcome include the severity of the injury, initial visual acuity (light perception or better vs. no light perception), the time interval between trauma and intervention, and the type of injury, such as fracture lines directly through the optic nerve canal or probable avulsion trauma. nTON cases generally have a slightly better prognosis, with vision improvement of 54–100% reported. The procedure should be performed before any optic nerve atrophy can occur. In IIH cases, all major symptoms, including vision loss, headache, visual field deterioration, and papilla edema, improved after optic nerve decompression, with rates of 60–100% reported. Earlier studies reported the occurrence of major complications such as cerebral fluid leakage, severe hemorrhage, or optic nerve injury at rates of about 3%. However, more recent studies postulate that no major complications occur, which may reflect the increasing use of the endonasal endoscopic route to the optic nerve and skull base as standard procedure, so that surgeons are becoming more comfortable with the technique. Endonasal endoscopic optic nerve decompression is a safe and highly effective treatment to reduce hydrostatic pressure on the optic nerve in cases of optic nerve neuropathy of various etiologies. In TON cases, about 50% of patients will benefit from the procedure, with vision improvement possible in some cases even with an interval of some days between trauma and surgery. Non-trauma cases have a slightly better prognosis. The intervention should be performed prior to optic nerve atrophy.

Background: Idiopathic intracranial hypertension (IIH) encompasses patients with elevated intracranial pressure (ICP). Generally, it is difficult to make a differential diagnosis between IIH and co-existing migraine headaches. Thus, this article intends to estimate the prevalence of migraine in patients with IIH and explain the occurrence of new-onset migraine after the diagnosis of IIH. Methods: The case group included 108 patients with IIH referred to the neurology wards of three university hospitals. A random sample of controls (n = 103) were recruited from patients hospitalized in the surgery and orthopedics ward. A checklist for migraine diagnosis was filled out. Cerebrospinal fluid (CSF) pressure and presence or absence of papilloedema (PE) in the patients and any necessary data were also recorded from the inpatient medical documents. All statistical analyses were done by SPSS software. Results: There were 70 (64.80%) and 22 (21.40%) migraineurs in the case and control groups, respectively, and the difference was found to be significant (P < 0.001). In 26 (37.14%) migraine cases in the IIH group, the disorder was diagnosed after developing IIH. Also, there was a past medical history of having migraine in 44 (62.85%) migraineurs. In the fully adjusted regression models, the odds of being affected by migraine in patients with IIH was 6.17 times greater than the controls [odds ratio (OR) = 7.15, 95% confidence interval (CI) = 3.56-14.36, P < 0.010]. The patients' mean CSF opening pressure was 32.10 ± 1.03 cmH2O and 93 (81.60%) subjects were found to have PE. Conclusion: It was demonstrated that subjects with IIH might have about a 6-time higher likelihood of developing migraine headache than the general population. These considerations can help prevent misdiagnosis of migraine headache as the recurrence of IIH or uncontrolled IIH and subsequent inappropriate management.

Some recent advances in neuro-ophthalmology including clinical trials, diagnostic testing and newly elucidated disease entitites were summarized in present paper, such as neuromyelitis optica spectrum disorder.The review goes into great detail regarding recent clinical trials in neuro-ophthalmology.These trials have investigated the management of idiopathic intracranial hypertension as well as treatment of Leber hereditary optic neuropathy and optic nerve glioma.The potential gene therapy for Leber hereditary optic neuropathy seems very promising and may have a significant clinical impact within the next few years.This review also includes an extensive discussion about the application of optical coherence tomography (OCT) and OCT-angiography (OCTA) in neuro-ophthalmology.OCT has important clinical relevance not only for the diagnosis and management of optic neuropathies, such as optic neuritis, ischemic optic neuropathy and chiasmal compression, but also for retro-chiasmal injury.In terms of OCTA, recent studies have demonstrated increased vessel density in acute stage of ischemic optic neuropathy (NAION) and optic neuritis, but decreased vessel density in severe papilloedema and late stage of optic atrophy of any cause.These serve as the potential foundation for future clinical applications.Ophthalmologists should pay more attentions to these recent progress in neuro-ophthalmology. Key words: Neuro-ophthalmology; Clinical trials, intracranial hypertension; Leber's optic neuropathy; Optical coherence tomography; Optical coherence tomography angiography; Optic neuritis; Ischemic optic neuropathy

A 59-year-old male presented with episodic headaches and blurring vision, and papilledema (PE) on examination. He had been advised continuous positive airway pressure (CPAP) treatment for moderate obstructive sleep apnea (OSA) and an apneic–hypopneic index of 21 but had not commenced the treatment. Magnetic resonance imaging of the brain was normal, but a contrast examination was not done. Magnetic resonance venography performed to assess the PE, revealed cerebral venous thrombosis (CVT) affecting the superior sagittal, transverse, and sigmoid sinuses. Oral anticoagulants and CPAP resulted in a speedy reversal of both PE and CVT. CVT may complicate OSA and require identification and treatment. Contrast studies are crucial in the evaluation.

Adduction-Induced Deformations Evoke Peripapillary Folds in Papilledema

Objective To explore the effect of psychotherapy in patients with optic papilledema undergoing optic nerve sheath fenestration (ONSF) . Methods Using convenience sampling method, a total of 64 patients with intracranial hypertension and optic papillary edema who were diagnosed by Ophthalmology Department of Xuanwu Hospital of Capital Medical University from 2013 to 2017 were selected as the subjects of study. All the subjects were randomly divided into two groups, with 32 patients in each group. Patients in the control group received routine ophthalmic nursing. The patients in the observation group were given six-step psychological intervention. Self-rating Anxiety Scale (SAS) were applied within 2 days after admission and before discharge to evaluate the patients. Results The score of SAS before discharge in the observation group was (46.28±4.48) , which was lower than that in the control group (56.84±8.25) , and the difference was statistically significant (t=6.363, P<0.01) . Conclusions Relevant psychotherapy can improve the anxiety of patients, so that patients can cooperate well with the treatment, which is worthy of clinical application. Key words: Psychotherapy; Papilledema; Optic nerve sheath fenestration; Increased intracranial pressure

Objective To summarize the clinical features and visual outcome of posterior scleritis presented with symptoms involving affected optic nerve. Methods Retrospective case series study. Twelve eyes of 12 female patients with posterior scleritis were included in this study. The average age was 35.2±14.31 years old. The patients got diagnosed with an average of 24.75±22.91 days. Ocular pain was complained in all patients, and blurred vision in 11 patients. The best corrected visual acuity (BCVA), intraocular pressure (IOP), slit lamp microscope examination, B-scan ultrasound, optical coherence tomography (OCT), fundus photography, fundus fluorescein angiography (FFA) and ocular wall thickness measurement were performed in all patients. Nine eyes received visual field examination. All patients received systemic corticosteroid and steroidal eye drops for 3 months. Clinical features and outcome were retrospectively studied. Results Before treatment, the BCVA was from 0.8. There were 3 eyes with scleral hyperemia, 3 eyes with anterior chamber flares, 12 eyes with papilledema and different degrees of retinal vein dilatation, 3 eyes with star-shaped macular exudates and 2 eyes with macular retinal pigment epithelium detachment. B-scan ultrasound demonstrated that the ocular walls were thickening in all eyes with typical T-sign, and the average thickness was 2.76±0.68 mm. OCT demonstrated optic disc swelling, and the macular retinal detachment in 2 eyes. In the FFA examination, the fluorescein leakage of the disc was enhanced with time. In the Humphrey test, the value of mean deviation (MD) was 12.56±5.73 dB and pattern standard deviation (PSD) was 8.15±4.23 dB in 9 eyes before the treatment. After treatment for 3 months, the symptoms were attenuated and the visual acuity was obviously improved with BCVA>0.1 in all eyes. Scleral hyperemia and anterior chamber flares were only found in 1 eye. The optic disc edema gradually faded away. The ocular wall thickness in the poster part of the eyeball decreased, and the T-sign disappeared in all eyes, the average thickness was 1.53±0.41 mm. Compared with parameters before the treatment, the difference was statistically significant (t=0.003 5, P<0.05). OCT demonstrated the recovery of the macular retinal detachment. There was no abnormal leakage evidenced in FFA in the optic disc and macular. After treatment, the value of MD and PSD was 5.19±4.82 dB and (4.33±3.76) dB, respectively. The difference of MD value between before and after the treatment was significant (t=0.026, P<0.05). Conclusions Posterior scleritis with an initial symptom of optic nerve was tend to affect middle-aged patients, with clinical manifestations of anterior segment signs in some patients and optic disc swelling with retinal vein dilatation in all patients. B ultrasound examination showed typical T sign. Systemic corticosteroid treatment always obtained remission of the ocular inflammatory activity, and could achieve favorable visual outcome. Key words: Scleritis/diagnosis; Scleritis/therapy; Optic neuritis; Disease attributes

Commentary: The Dilemma of Papilledema in Chiari I Malformation.

Association between papilledema and guillian-barré syndrome

Accuracy of Diagnostic Imaging Modalities for Classifying Pediatric Eyes as Papilledema Versus Pseudopapilledema

ABSTRACTThe authors reviewed 93 consecutive cases with optic disc swelling (ODS) to compare clinical manifestations and prognosis among the causes. Among unilateral ODS patients ≥50 years old and without pain, anterior ischaemic optic neuropathy accounted for 87.5%. Furthermore, papilloedema (PE) presented unilateral ODS with an atrophic or hypoplastic disc in the opposite eye. Despite no differences for age and initial visual acuity between PE and pseudopapilloedema, the two main causes of bilateral ODS, some PE patients showed poor visual prognosis. Understanding differences in frequencies and clinical features of ODS related to cause and age group can help to accurately determine cause and predict outcome.