Analysis of facial features of patients with sagging eye syndrome and intermittent exotropia compared to controls
Kunimi K, Goseki T, Fukaya K, Takahashi S, Ishikawa E, et al. Analysis of facial features of patients with sagging eye syndrome and intermittent exotropia compared to controls. Am J Ophthalmol 18 October 2022;S0002-9394(22)00391–9. doi: 10.1016/j.ajo.2022.10.007. Online ahead of print.
Frontal facial photographs of patients who were greater than 60 years of age and had been diagnosed with sagging eye syndrome or intermittent exotropia were evaluated for three characteristics including sunken upper eyelids, blepharoptosis and baggy lower eyelids. The patients were compared to normal controls and a scoring system was used to grade the degree of these three characteristics.
A total of 86 patients were included in the study. Of these, 23 were diagnosed with sagging eye syndrome, 28 were diagnosed with intermittent exotropia and 35 were in the control group. All patients were Japanese.
The authors noted that only sunken upper eyelids, as opposed to blepharoptosis and baggy lower eyelids, were significantly associated with sagging eye syndrome. This contrasts with previous reports of sagging eye syndrome being associated with blepharoptosis. They also report that baggy lower eyelids were significantly associated with intermittent exotropia. A detailed description is provided of the pathological changes that occur within the orbit leading to these two disorders.
David Bellows
Serial titre and seroconversion in paediatric myelin oligodendrocyte glycoprotein antibody disease
Wendel EM, Thonke HS, Bertolini A, Baumann M, Blaschek A, Merkenschlager A, et al, on behalf of the BIOMARKER Study Group. Temporal dynamics of MOG antibodies in children with acquired demyelinating syndrome. Neurol Neuroimmunol Neuroinflamm 2022;9:e200035.
Myelin oligodendrocyte glycoprotein (MOG) antibody disease (MOGAD) has various clinical phenotypes with acute disseminated encephalitis (ADEM) being the predominant phenotype in younger children, while older children tend to present with optic neuritis (ON) or transverse myelitis (TM). For relapsing MOGAD the current recommended length of treatment per the authors is 2 years after disease remission, independent of MOG-IgG status. It is therefore important to identify the prognostic factors after first MOGAD presentation to guide management.
As part of BIOMARKER study this is a prospective multi-centre hospital-based study involving more than 1000 paediatric patients with a suspected acquired demyelinating syndrome. Those with a first demyelinating attack from MOGAD with complete clinical, radiological and serological data sets as well as follow-up data were recruited.
Among 116 children in the study the most common initial presentation was ADEM (n = 59) followed by optic neuritis (n = 28) with a significant age difference between the two groups. Thirty-eight percent of patients developed relapsing disease over time with the median follow-up being 3 years in monophasic and 5 years in relapsing patients. There was no significant association between disease course and MOG-IgG titres at onset, sex, age at presentation, clinical phenotype or laboratory findings (serum/cerebrospinal fluid) at onset. However, a lower decline during the first 2 years (i.e., persistently higher MOG Ig-G titres) were noted in relapsing patients. Seroconversion to MOG-IgG-negative within 2 years of the initial event showed a significant risk reduction for a relapsing disease course. The median time of seroconversion in relapsing patients with seroconversion was 45 months. In this cohort, no patient experienced a relapse after seroconversion to MOG-IgG-negative.
In this study they found that serial MOG-IgG titres were the only significant predictor for relapsing disease course in paediatric MOGAD. Therefore, serial MOG-IgG testing at least every 6 months for the evaluation of relapse risk was proposed. The authors suggested that seronegativity (defined as an MOG-Ig titre of less than 1:160) during the first and second years pointed against further relapses. Nevertheless, an international standard of seroconversion to MOG-IgG-negative titres is still lacking. The study group proposed using a definition with measurement of endpoint titres. Yet, caution has to be taken as testing of titres after plasma exchange may lead to false-negative results. Also, the effect of immuno-modulating therapies such as intravenous immunoglobulin on MOG-IgG titres has not been fully studied. They also showed that titre at onset has no predictive value for the disease course. It is in contrast to the assumption published in 2017 that stated hat high MOG-IgG titres >1:1280 were associated with a relapsing disease course.
Noel Chan
Asymptomatic optic nerve enhancement in neuromyelitis optica spectrum disorder?
Shah SS, Morris P, Buciuc M, Tajfirouz D, Wingerchuk DM, Weinshenker BG, et al. Frequency of asymptomatic optic nerve enhancement in a large retrospective cohort of patients with aquaporin-4+ NMOSD. Neurology 2022;99:e851-e857. doi:10.1212/WNL.0000000000200838
Unlike multiple sclerosis, asymptomatic optic nerve enhancement in neuromyelitis optica spectrum disorder (NMOSD) is thought to be rare. Do all asymptomatic enhancements signify subclinical optic neuritis (ON)?
In this retrospective cohort, patients with aquaporin-4 (AQP4) – immunoglobulin G (IgG)- positive NMOSD were recruited. A total of 198 magnetic resonance imaging (MRI) scans of orbits in 100 patients, with 107 inter-attack MRIs from 78 patients were reviewed. Asymptomatic optic nerve enhancement occurred in 17% of seropositive NMOSD MRI scans 30 days after an attack, which remained persistent in 15% and 12% at 60 days and 90 days from the attack, respectively. Only one patient with this asymptomatic enhancement eventually developed a clinical episode of ON. New asymptomatic enhancement was seen in two patients, both of whom had a remote history of ON in the affected eye and represented only 2% of inter-attack scans. The authors noted that patients with asymptomatic enhancement on inter-attack MRIs were younger than those without. Nevertheless, long-term visual outcomes between those with and without asymptomatic enhancement were not statistically different.
Given the extended time frame over which the enhancement persisted at the site of prior enhancement without visual compromise, and because all patients with asymptomatic enhancement had a prior ON, intermittent/persistent blood-brain barrier breakdown may account for the asymptomatic enhancement rather than subclinical optic neuritis.
Young patients have more robust autoimmunity at earlier stages of the disease and this may account for these radiological findings. Although rare, new asymptomatic optic nerve enhancement can occur in patients with prior ON.
Noel Chan
A review on genetic diseases that mimic central nervous system inflammatory disorders
Ayrignac X, Carra-Dallière C, Marelli C, Taïeb G, Labauge P. Adult-onset genetic central nervous system disorders masquerading as acquired neuroinflammatory disorders: A review. JAMA Neurol. 1 October 2022;79(10):1069–1078. doi: 10.1001/jamaneurol.2022.2141.
There has been increasing recognition of genetic diseases that can cause magnetic resonance imaging (MRI) changes that mimic central nervous system (CNS) inflammatory disorders. While most of these manifest in childhood, there are some genetic diseases that can present in adulthood and cause significant diagnostic uncertainty. In this recent review in JAMA Neurology, Aryignac et al. discuss the most common adult-onset genetic diseases that can be confused with acquired neuroinflammatory disorders including genetic leukodystrophies, retinal vasculopathy with cerebral leukoencephalopathy (RVCL), Alexander’s disease, cytotoxic T-lymphocyte-associated protein haploinsufficiency, familial haemophagocytic lymphohistiocytosis, and biotinidase deficiency. Table 1 is especially helpful in summarising the MRI findings and suggestive features. Among these diseases, RVCL and biotinidase deficiency are the most likely to present in the neuro-ophthalmology clinic because their presenting symptom can be visual loss. RVCL is an autosomal dominant inflammatory microangiopathy caused by TREX1 gene mutations, which causes a vascular retinopathy, in addition to cognitive impairment, psychiatric symptoms, seizures, and focal neurological deficits. Biotinidase deficiency is an autosomal recessive disease that causes subacute bilateral optic neuropathy with central vision loss that can be accompanied by a myelopathy with longitudinal T2 lesions in the spinal cord.
All of these genetic diseases are important to recognise because their diagnosis can limit unnecessary investigations and avoid potential side effects from unnecessary immunosuppressive treatments. In addition, some of these genetic diseases are treatable if recognised early enough. This review will help neuro-ophthalmologists, neurologists, and ophthalmologists recognise these rare genetic diseases that can mimic adult-onset CNS inflammatory disorders.
John Chen
Orbital/ocular involvement is common in histiocytic disorders, which may compromise visual acuity
Banks SA, Bhatti MT, Go RS, Abeykoon JP, Acosta-Medina AA, Hazim AZ, et al. Ophthalmologic involvement in adults with histiocytic disorders: Clinical presentation and treatment outcomes. Ophthalmology. 3 August 2022:S0161-6420(22)00590–5. doi: 10.1016/j.ophtha.2022.07.031. Online ahead of print.
The authors conducted a retrospective chart review study to investigate ophthalmological involvement in adults with histiocytic disorders. They identified 32 patients, including seven with Langerhans cell histiocytosis (LCH), 15 with Erdheim-Chester disease (ECD), one with mixed LCH/ECD phenotype, eight with Rosai-Dorfman disease (RDD), and one with a mixed RDD/ECD phenotype. Twenty-two (69%) patients had ophthalmological involvement as part of the initial presentations, with eyelid oedema (13/32, 41%) and proptosis (12/32, 38%) being the most common presentations. Isolated orbital or cavernous sinus involvement was most commonly present in patients with LCH (3/7), while patients with ECD usually presented with optic nerve sheath involvement (14/15) and diffuse orbital involvement (12/15). Ocular involvement was seen in six out of eight RDD patients. Visual acuity was affected in 14 patients (14/24, 58%) with a median logarithm of the minimum angle of resolution visual acuity of 0.1 (range, −0.12 to 3). Orbital involvement was most commonly seen in ECD, whereas ocular involvement was most common in RDD. This study shows that visual acuity should be monitored since it may be affected by ocular/orbital involvement by theses histiocytic disorders.
Hui-Chen Cheng
Optic nerve involvement, intra-ocular inflammation, and retinal degeneration may contribute to changes in visual function of patients with ROSAH syndrome
Huryn LA, Kozycki C, Serpen JY, Zein WM, Ullah E, Iannaccone A, et al. Ophthalmic manifestations of ROSAH Syndrome, an inherited NF-kappaB mediated autoinflammatory disease with retinal dystrophy. Ophthalmology 1 November 2022;S0161-6420(22)00858–2. doi: 10.1016/j.ophtha.2022.10.026. Online ahead of print.
ROSAH (Retinal dystrophy, Optic nerve oedema, Splenomegaly, Anhidrosis, and Headache) syndrome is an autosomal dominant-inherited disease associated with innate immune activation, which is caused by mutations in the ALPK1 gene. The authors conducted an observational study to investigate the ocular phenotype of patients with ROSAH syndrome. They enrolled 11 patients with genetically proven ROSAH syndrome, including six females and five males from seven families and age range of 7.3 to 60.2 years. Best-corrected visual acuity ranged from 20/16 to no light perception. Nine patients had signs of intraocular inflammation including keratic precipitates, band keratopathy, anterior chamber cells, cystoid macular oedema and retinal vasculitis. Ten patients had peripapillary retinal nerve fibre layer thickening on optical coherence tomography. Seven patients had retinal degeneration with cone-rod dystrophy. One patient had decreased Arden ratio on electrooculogram. The authors concluded that optic nerve involvement, intra-ocular inflammation, and retinal degeneration may contribute to changes in visual function of patients with ROSAH syndrome.
Hui-Chen Cheng
The epidemiology and phenotypes of ocular manifestations in childhood and juvenile myasthenia gravis: A review
Heckmann JM, Europa TA, Soni AJ, Nel M. The epidemiology and phenotypes of ocular manifestations in childhood and juvenile myasthenia gravis: A review. Front Neurol. 2022;13:834212
The authors reviewed the literature on the phenotypes of childhood- and juvenile-onset ocular myasthenia gravis (MG) among African, Asian and European populations.
There appears to be a four-fold higher incidence rate of MG among younger children from Asia compared with Europe and North America. At least half of the children manifested with MG before the age of 10 with the incidence peaking before the age of 5 years. In Asia, there was a tendency towards more ocular MG amongst the very young and this was not evident in Norwegian children. A multi-racial juvenile MG cohort from Canada with 40% European ancestry showed higher proportion of pre-pubertal onset MG, and most of the very young onset ocular MG cases had Asian ancestry. Similar multi-racial results were seen in cohorts from France and the United Kingdom with African ancestry.
A feature of MG among north European children was that ocular only presentations occurred in less than a third, with >75% of children developing generalised disease within 2 years. Similar findings were made in Canada where white children were more likely to develop generalised MG than Asian.
Acetylcholine receptor antibody (AChR-Ab) positive MG ranged between 50 and 95%. Younger children were more likely to have AChR-Ab negative MG and ocular disease, both of which conferred a higher likelihood of going into remission. Thymoma occurs rarely in juveniles with MG, but concomitant autoimmune disease was mainly thyroid reported in 4–19% of children with Asian or African ancestry. In contrast 30% of Norwegian children had other autoimmune disease in addition to MG.
There remains a significant research gap regarding therapy in juveniles with MG. One study in China showed that treatment with immune therapy and pyridostigmine resulted in only 50% achieving minimal manifestations or better after at least 12 months of follow up. Better outcomes were seen with earlier (within 2 years of symptoms onset) use of prednisone 0.25 mg/kg/day if symptoms did not resolve with pyridostigmine alone, followed by a slow taper and steroid cessation after 6 months of clinical remission. In another study in China, only 17% improved despite immune treatment with prednisone 0.75 mg/kg/day and thymectomy. In South Africa, after 5 years, 31% of children remained with partial or complete treatment-resistant ophthalmoplegia. However, in North America, all patients in a cohort of 22 patients reached minimal manifestation status or better with prednisone 2.5 mg/kg/day for 4–6 weeks followed by alternate day dosing.
An international working group advised that children with juvenile MG should start cholinesterase inhibitors at 0.5 to 1 mg/kg every 4–6 h with the dose increasing to 7 mg/kg/day. Oral steroids between 0.5 and 1 mg/kg daily were advised when there was a poor response to cholinesterase inhibitors. Steroid sparing agents should be considered when there is a poor response to steroids; these include azathioprine, mycophenolate mofetil, methotrexate, and rituximab.
Congenital MG manifests with fatiguable ocular or generalised muscle weakness at birth or within the first year of life with a family history of the same. These patients are seronegative and do not respond to cholinesterase inhibitors. While the HLA region on chromosome 6 has been shown to be associated with MG in adults, the data in children are sparse and need to be further studied.
Treatment resistance is more likely in patients with younger age of onset (<20 years), AChR-Ab positive and African ancestry. Delayed treatment may be associated with muscle atrophy-pathways and mitochondrial metabolic pathways that cannot maintain normal homoeostasis, leading to an irreversible phase of mitochondrial stress, extra-ocular muscular atrophy and fat replacement.
Ultimately, there needs to be more dedicated studies on juvenile MG to create consensus on standardised use of descriptions of ocular involvement and to better guide treatment in these populations.
Peter MacIntosh
Neuro-ophthalmology malpractice: A review of the Westlaw Database
Zhu D, Wong A, Shah PP, Pomeranz HD. Neuro-ophthalmology malpractice: A review of the Westlaw Database. Med Leg J. 2 October 2022:258172221109731. doi: 10.1177/00258172221109731. PMID: 36189929.
As neuro-ophthalmologists we practise on the frontlines and often see patients with vision or life-threatening diagnoses. We are keenly aware of the morbidity and litigation risks associated with these diagnoses but this retrospective study sheds further light on the litigation risk of neuro-ophthalmological diagnoses. The study used a US legal database (Westlaw) to identify 43 cases over the past 33 years that involved ophthalmologists and a neuro-ophthalmological diagnosis. Because cases that are settled confidentially are not discoverable in this database, the data reviewed is incomplete and certainly underestimates the total number of cases. Some of the study findings were not surprising including that failure to diagnose is the predominant indication for litigation and the top missed diagnoses in order of decreasing frequency include stroke, brain tumours, giant cell arteritis, and idiopathic intracranial hypertension. Based upon dissection of the cases by the authors, many of these missed diagnoses could have been avoided by implementing a few routine practices including checking colour vision and visual fields in patients with subjective visual complaints, dilating patients who might be at risk for IIH, including those with headaches, and recognition of amaurosis among patients with transient monocular vision loss. When teaching trainees in neuro-ophthalmology clinics, the vast majority will go on to practice outside the field of neuro-ophthalmology so we should consider sharing this study with unmotivated trainees to drive home the fact that missed neuro-ophthalmological diagnoses can be extremely costly to ophthalmologists. The average payment for plaintiffs including jury decisions for the plaintiff and settlements was nearly $2 million ($1,952,154).
Collin McClelland
Ocular findings in asymptomatic patients with primary antiphospholipid syndrome
Neto TS, Neto ED, Balbi GG, Signorelli F, Higashi AH, Monteiro MLR, et al. Ocular findings in asymptomatic patients with primary antiphospholipid syndrome. Lupus. 1 October 20224:9612033221133687. doi: 10.1177/09612033221133687. PMID: 36239237.
Antiphospholipid syndrome (APAS) can be an elusive diagnosis much like other systemic inflammatory conditions, including lupus. Ophthalmologists and neuro-ophthalmologists are tasked with recognising and testing for APAS amongst patients with peculiar presentations including transient monocular vision loss, migraine-like visual phenomena, and retinal artery occlusions. This study approaches the issue of neuro-ophthalmological disease in APAS from the opposite direction by completing full eye examinations and optical coherence tomography among asymptomatic patients with an established diagnosis of APAS. Neuro-ophthalmological disease was surprisingly plentiful (19.2% of individuals) with the most common retinal change being paracentral acute middle maculopathy (PAMM) in 3/26 subjects. Interestingly, no anterior segment abnormalities were detected among APAS patients. APAS should be considered in patients presenting with PAMM or other retinal vascular occlusive disease, particularly if there is a history of venous/arterial thrombosis or spontaneous miscarriages.
Collin McClelland
Optic nerve head anatomy and vascular risk factors in patients with optic disc drusen associated anterior ischaemic optic neuropathy
Johannesen RG, Lykkebirk L, Jørgensen M, Malmqvist L, Hamann S. Optic nerve head anatomy and vascular risk factors in patients with optic disc drusen associated anterior ischaemic optic neuropathy. Am J Ophthalmol. 2022;242:156–164.
In this case-control study, 34 optic disc drusen (ODD)-associated anterior ischaemic optic neuropathy (ODD-AION) patients and 34 non-arteritic anterior ischaemic optic neuropathy without ODD (nODD-AION) patients had optical coherence tomography using a standardised ODD scanning protocol and were retrospectively analysed and compared regarding demographics, vascular risk factors, clinical characteristics, and specific optic nerve head anatomical characteristics. They found that in patients with ODD-AION, the ODD were predominantly deeply located (82%) but with no significant difference in size (52% large, 48% small). When compared with nODD-AION patients, ODD-AION patients were significantly younger at the time of diagnosis and had fewer vascular risks. The ODD-AION patients had significantly more peri-papillary hyper-reflective ovoid mass-like structures (PHOMS) and pre-laminar hyper-reflective lines as well as smaller Bruch’s membrane opening diameters compared with nODD-AION patients. No significant differences were found between ODD-AION and nODD-AION patients regarding visual acuity, refraction, lamina cribrosa position, ganglion cell layer volume, or retinal nerve fibre layer thickness. The authors concluded that in ODD-AION, location of the ODD within the optic nerve head is important, while the size of the ODD is not. The ODD-AION and nODD-AION patients presented with distinctly different vascular risk factors and anatomical characteristics, establishing ODD and potentially also PHOMS as independent risk factors for developing NA-AION.
Michael Vaphiades
At this junction
Warwick AM, Gospe SM 3rd, Chen JJ. At this junction … . Surv Ophthalmol. 2022;67(6):1711–1716.
The authors present an 81-year-old woman who developed retrobulbar optic neuritis in the right eye. Automated perimetry showed a supero-temporal visual field defect in the asymptomatic left eye, suggestive of a junctional defect. Magnetic resonance imaging demonstrated enhancement of the right optic nerve extending to its junction with the optic chiasm. The patient’s vision failed to improve with intravenous corticosteroids, but demonstrated significant improvement with therapeutic plasma exchange. She was seropositive for aquaporin 4 autoantibodies, confirming the diagnosis of neuromyelitis optic spectrum disorder (NMOSD). The interesting parts of this paper is the patient’s age of onset and Dr. Chen’s discussion of NMOSD and related disorders.
Michael Vaphiades
Sharp edge eye syndrome: A case report and survey of self-identified individuals
Reynolds MS, Katz BJ, Digre KB, Brintz BJ, Olson LM, Warner JEA. Sharp edge eye syndrome: A case report and survey of self-identified individuals. J Neuroophthalmol. 2022;42:524–529. doi: 10.1097/WNO.0000000000001650.
Sharp edge eye syndrome (SEES), sometimes known as visual looming syndrome, is a condition in which the patient experiences ocular pain or discomfort when viewing or mentally picturing sharp objects and edges. The authors describe the syndrome, vision-related quality of life, and psychosocial characteristics in patients with self-identified SEES using a cross-sectional web-based survey made available on social media webpages dedicated to SEES. The study included 22 questions developed by the research team, demographic questions, and four standardised questionnaires. Seventy-seven respondents had a mean age of 29 years and were 57% male. Ninety-two percent reported symptoms starting before the age of 18. The main site of pain or discomfort was the eyes, with onset resulting from viewing or thinking about sharp objects and edges. Symptoms lasted from seconds to hours and could be prolonged even after closing eyes or avoiding viewing the trigger. Anxiety was reported in 58% of participants and depression in 57%. Migraine or headache was reported in 46% of participants. Participants also reported Alice in Wonderland syndrome, visual snow, obsessive-compulsive disorder, attention deficit hyperactivity disorder, stripe-induced visual discomfort, and synaesthesia. The authors concluded that their study provided an understanding of the characteristics of SEES, as well as vision-related quality of life impacts. They postulated that SEES may be a distinct visual phenomenon.
Michael Vaphiades
Alice in Wonderland syndrome: A lesion mapping study
Piervincenzi C, Petsas N, Giannì C, Di Piero V, Pantano P. Alice in Wonderland syndrome: A lesion mapping study. Neurol Sci. 2022;43(5):3321–3332.
Alice in Wonderland syndrome (AIWS) is a rare neurological disorder characterised by an erroneous perception of the body schema or surrounding space. It may be caused by a variety of neurological disorders, but to date, there is no agreement on which brain areas are affected. The authors conducted a literature search for AIWS cases following brain lesions. Patients were classified according to their symptoms as type A (somaesthetic), type B (visual), or type C (somaesthetic and visual). Using a lesion mapping approach, lesions were mapped onto a standard brain template and sites of overlap were identified. Of 30 lesions detected, maximum spatial overlap was present in six cases. Local maxima were identified in the right occipital lobe, specifically in the extra-striate visual cortices and white matter tracts, including the ventral occipital fasciculus, optic tract, and inferior fronto-occipital fasciculus. Overlap was primarily due to type B patients (the most prevalent type, n = 22), who shared an occipital site of brain damage. Type A (n = 5) and C patients (n = 3) were rarer, with lesions disparately located in the right hemisphere (thalamus, insula, frontal lobe, hippocampal/parahippocampal cortex). The authors concluded that lesion-associated AIWS in type B patients could be related to brain damage in visual pathways located preferentially, but not exclusively, in the right hemisphere. Conversely, the lesion location disparity in cases with somaesthetic symptoms suggests underlying structural/functional disconnections requiring further evaluation.
Michael Vaphiades
Diagnosis and classification of optic neuritis
Petzold A, Fraser CL, Abegg M, Alroughani R, Alshowaeir D, Alvarenga R, et al. Diagnosis and classification of optic neuritis. Lancet Neurol. 27 September 2022:S1474-4422(22)00200–9. doi: 10.1016/S1474-4422(22)00200–9. Epub ahead of print. PMID: 36179757.
Currently, there are no established diagnostic criteria and hence no standardised classification of optic neuritis. An international expert panel of 101 neurologists, ophthalmologists, neuro-ophthalmologists, and neuroradiologists sought consensus with a validated Delphi process to address this issue. For the diagnostic criteria and classification, they included all the novel ancillary tests in this rapidly evolving field. Their diagnostic criteria distinguish between definite and possible optic neuritis based on the clinical presentation, validated antibodies (aquaporin 4, myelin oligodendrocyte glycoprotein [MOG], collapsin response-mediator protein 5, intrathecal IgG synthesis), magnetic resonance imaging, and optical coherence tomography. Based on the clinical presentation and time course, as well as the different antibodies, they characterised ten different subtypes, such as MOG-associated optic neuritis, chronic relapsing inflammatory optic neuropathy, and others. This classification is intended to help clinicians avoid misdiagnosis, guide acute treatment, and assess the need for and selection of appropriate long-term immunosuppression, such as monoclonal antibodies.
Konrad P. Weber
Further understanding of the peri-papillary capillary network in papilloedema
Chonsui M, Le Goff M, Korobelnik JF, Rougier MB. Quantitative analysis of radial peripapillary capillary network in patients with papilloedema compared with healthy subjects using optical coherence tomography angiography. J Neuroophthalmol. 2022;42:e109-e115. doi: 10.1097/WNO.0000000000001460. Epub 22 October 2021. PMID: 34860747.
While optical coherence tomography (OCT) has been widely used in clinical setting as a tool for diagnosis and disease monitoring of idiopathic intracranial hypertension (IIH), there has been few studies about OCT angiography (OCTA) change in papilloedema. This article found a decreased peri-papillary capillary density without changes in flux intensity in eyes with papilloedema. There was a positive association between the capillary flux index and the retinal nerve fibre layer (RNFL) thickness and a negative association between the capillary perfusion density and RNFL thickness. It confirmed the discriminatory ability of OCTA in differentiating papilloedema secondary to IIH from a normal optic disc, while providing complementary information for understanding papilloedema pathophysiology.
Xiaojun (June) Zhang