Bilateral optic disc collaterals secondary to high-flow dural arteriovenous fistula: a diagnostic dilemma.

A man in his 80s, with a history of diabetes, hypertension and coronary artery disease, presented with bilateral painless progressive vision loss 2 years prior. His examination showed subnormal best corrected visual acuity of 20/50 and 20/80 in the right eye and left eye (LE), respectively, grade II relative afferent pupillary defect in LE, normal anterior segment, intra-ocular pressure (IOP) and defective colour vision in both eyes (BE). Fundus examination revealed optic disc pallor, disc collaterals and grade 2 hypertensive retinopathy in BE. Automated perimetry showed advanced field loss in BE. MRI of the brain and orbits with contrast showed signs of raised intracranial pressure, and magnetic resonance angiogram of the brain showed multiple arterio-venous channels along with the right transverse and sigmoid sinuses. The patient was referred to a neuroradiologist for further evaluation, and cerebral angiogram confirmed multifocal high-flow dural arterio-venous fistulae at right jugular foramen, transverse and sigmoid sinuses. He underwent Onyx liquid embolization.

Subnormal visual acuity after compliant amblyopia therapy: residual/refractory amblyopia or co-existing pathology? - a retrospective analysis.

Purpose: To assess the prevalence of alternate etiology/co-existing pathology among patients with amblyopia, and to characterize factors contributing to over-diagnosis of amblyopia. Methods: We retrospectively reviewed records of children (from 1 January 2016 to 31 December 2019) who were initially diagnosed as "amblyopia" but later an alternate diagnosis for subnormal vision was established. Patients who had a best corrected visual acuity (BCVA) of ≤20/32 (0.2 logMAR) after compliant amblyopia therapy were divided into 2 groups: those with refractory amblyopia (BCVA improvement from baseline <1 logMAR line) and residual amblyopia (BCVA improvement from baseline >1 logMAR line). Data was collected for presence/absence of amblyogenic risk factors, history, ocular examination, and investigations leading to the final alternate diagnosis. We analyzed the factors that contributed to the initial over-diagnosis of amblyopia using the diagnostic error evaluation and research (DEER) taxonomy tool. Results: During the study period, 508 children with an initial diagnosis of amblyopia met the study criteria. Among these 508 children, 466 were diagnosed to have amblyopia alone, while 26 children (5.1%, median age: 7 years, 17 boys: 9 girls) were revised to have an alternate diagnosis/co-existing pathology. These 26 patients comprised of 2 groups: children referred to us as amblyopia but rediagnosed to have an alternate diagnosis; and a second subset, initially diagnosed by us to have amblyopia, but later found to have alternate diagnosis/co-existing pathology. Subclinical optic neuritis (50%, 13 children), and occult macular dystrophy (OMD) (38.4%, 10 children) were the most frequent alternative diagnoses. Children with ametropic amblyopia (8/26, 30.7%) were most frequently misdiagnosed. Risk factors that led to an initial diagnosis of amblyopia were: high refractive error and heterotropia in 7 patients each (26.9%), anisometropia in 12 (46.1%), and prior pediatric cataract surgery in 4(15.3%). No improvement in BCVA in 21/26 (80.7%) children led to suspicion of co-existing etiology. Other clues were optic disc pallor (11), subnormal color vision (7), history of parental consanguinity in 7, and preceding febrile illness/rhinitis in 1 child. The DEER taxonomy tool suggested that the most common reasons for diagnostic errors were over-emphasis on amblyopia. Conclusion: Our study suggests that 5% of children diagnosed with amblyopia might have co-existing/alternate etiology. Most common co-existing etiologies were subclinical optic neuropathy, and OMD. No improvement in BCVA, subtle history and examination findings prompted further workup. Not considering co-existing etiologies was the most common reason for an initial overdiagnosis of amblyopia.

Pediatric myopic strabismus fixus: clinical features and surgical outcomes of silicone band loop myopexy.

PURPOSE: To evaluate the clinical profile of myopic strabismus fixus (MSF) in children and surgical outcomes of silicone band loop myopexy. METHOD: We retrospectively reviewed records of children presenting with MSF who underwent silicone band loop myopexy between January 2008 and December 2020 at a tertiary eye care center. Data concerning demographics, refractive error, axial length, extra-ocular motility, and ocular alignment pre-operatively and post-operatively, intra- and post-operative complications, ocular and systemic associations, were evaluated. The long-term effects of band loop myopexy on ocular alignment stability, motility improvement, and myopia progression were analyzed. Surgical outcome was defined as post-operative orthotropia or heterotropia less than or equal to 20 PD. RESULTS: A total of0 eyes of 7 patients (median age: 5 years; 5 boys and 2 girls) who underwent band loop myopexy were included in the study. Among them, three children underwent bilateral and four children underwent unilateral band loop myopexy. Medial rectus recession was performed only in two patients as a part of initial procedure. The median follow-up duration was 7 years. Most of the children, i.e. six of them presented with esotropia-hypotropia and only one patient presented with exotropia-hypotropia complex. The median pre-operative measurements were esotropia of 62.5 PD, hypotropia of5 PD, and exotropia of4 PD. Postoperative average primary position deviation measured was close to 9-10 PD of esotropia. The overall motility improved to -1 from -3. CONCLUSION: The clinical profile of MSF in children is almost similar to adults. This condition is a rare entity among adults as well as children. Majority of children with MSF presented with esotropia-hypotropia complex. Silicone band loop myopexy with or without medial rectus recession proves to be a reliable surgical procedure as it provides stable outcomes in terms of ocular alignment and motility among children.

Agreement Between Virtual Reality Perimetry and Static Automated Perimetry in Various Neuro-Ophthalmological Conditions: A Pilot Study.

Our objective was to compare the agreement between virtual reality perimetry (VRP) (order of magnitude, OM) and static automated perimetry (SAP) in various neuro-ophthalmological conditions. We carried out a retrospective analysis of visual field plots of patients with various neuro-ophthalmological conditions who underwent visual field testing using VRP and SAP and between 1 January and 31 May 2022. Two fellowship-trained neuro-ophthalmologists compared the visual field defects observed on both devices. Per cent agreement was used to compare the interpretation of the two examiners on both techniques. The study criteria were met by 160 eyes from 148 patients (mean age 44 years, range 17-74 years). The most common aetiologies were optic atrophy due to various causes, optic neuritis, ischaemic optic neuropathy, and compressive optic neuropathy. Overall, we found good agreement between VRP and SAP for bitemporal (93.8%), hemianopic (90.8%), altitudinal (79.4%), and generalised visual field defects (86.4%). The agreement was acceptable for central/centrocaecal scotomas and not acceptable for enlarged blind spots. Between the two examiners there was good agreement for bitemporal (92.3%), hemianopic (82%), altitudinal (83%), and generalised field defects (76.4%). The results of our study suggest that VRP gives overall good agreement with SAP in various neuro-ophthalmological conditions, especially those likely to produce hemianopic, altitudinal, and generalised visual field defects. This could be useful in various settings; however, future larger studies are needed to explore real-world utilisation.

Differentiating Occult Neuroretinitis and Non-Arteritic Anterior Ischaemic Optic Neuropathy: Clinical and Optical Coherence Tomography Characteristics.

We report clinical and optical coherence tomography (OCT) differences among patients with occult neuroretinitis and non-arteritic anterior ischaemic optic neuropathy (NAAION). We retrospectively reviewed records of patients with a final diagnosis of occult neuroretinitis and NAAION seen at our institute. Data were collected regarding patient demographics, clinical features, concomitant systemic risk factors, visual function, and optical coherence tomography (OCT) findings at presentation and subsequent follow-up. Fourteen and 16 patients were diagnosed to have occult neuroretinitis and NAAION, respectively. Patients with NAAION were slightly older (median age 49, inter-quartile range [IQR]: 45-54 years, versus 41, IQR: 31-50 years) than patients with neuroretinitis. Seventy-five per cent of patients with NAAION were male versus 43% with neuroretinitis (p = 0.07). Systemic risk factors were present in 87.5% of patients with NAAION versus 21.4% in patients with neuroretinitis (p = 0.001). At presentation, all patients presented with blurred vision, had similar visual function, and had optic disc oedema. In addition, none of the patients had evident retinitis lesions, but 10 (71%) showed evident retinitis lesion at follow-up. Neuroretinitis patients had more often vitreous cells (64% versus 6%, p = 0.001), and subretinal fluid (78.6% versus 37.5%, p = 0.03) than the patients with NAAION. In summary, NAAION patients tended to be slightly older, more often male, and had associated systemic diseases more often than those with neuroretinitis. Neuroretinitis patients more often had posterior vitreous cells and subretinal fluid on OCT. However, larger prospective studies are needed.

Circumscribed interests in autism: Can animals potentially re-engage social attention?

BACKGROUND: Circumscribed interests (CI) in autism are highly fixated and repetitive interests, generally centering on non-social and idiosyncratic topics. The increased salience of CI objects often results in decreased social attention, thus interfering with social interactions. Behavioural, biomarker and neuroimaging research points to enhanced social functioning in autistic children in the presence of animals. For instance, neuroimaging studies report a greater activation of reward systems in the brain in response to animal stimuli whereas eye-tracking studies reveal a higher visual preference for animal faces in autistic individuals. This potentially greater social reward attached to animals, introduces the interesting and yet unexplored possibility that the presence of competing animal stimuli may reduce the disproportionately higher visual attention to CI objects. METHOD: We examined this using a paired-preference eye-tracking paradigm where images of human and animal faces were paired with CI and non-CI objects. 31 children (ASD n = 16; TD n = 15) participated in the study (3391 observations). RESULTS: Autistic children showed a significantly greater visual attention to CI objects whereas typical controls showed a significantly greater visual attention to social images across pairings. Interestingly, pairing with a CI object significantly reduced the social attention elicited to human faces but not animal faces. Further, in pairings with CI objects, significantly greater sustained attention per visit was seen for animal faces when compared to human faces. CONCLUSIONS: These results thus suggest that social attention deficits in ASD may not be uniform across human and animal stimuli. Animals may comprise a potentially important stimulus category modulating visual attention in ASD.

Clinical Profile, Neuroimaging Characteristics, and Surgical Outcomes of Patients With Acute Acquired Non-accommodative Comitant Esotropia.

PURPOSE: To report the clinical presentations, neuroimaging findings, and surgical outcomes in patients with acute acquired non-accommodative comitant esotropia (AACE). METHODS: A retrospective review of records of all patients diagnosed as having AACE between January 2011 and December 2019 across three tertiary eye care centers was done. Cases with AACE onset after age 1 year were included. Patients were divided into two groups based on age of onset of esotropia: childhood (16 years or younger) and adult (older than 16 years). Surgical success was defined as postoperative horizontal deviation of 8 prism diopters (PD) or less at the last follow-up visit. RESULTS: A total of 338 patients (220 males and 119 females; mean age at presentation: 12.60 ± 9.8 years) met the study criteria. The mean age at onset of esotropia in the childhood and adult onset groups was 3.61 ± 1.1 and 26.6 ± 8.7 years, respectively. There were significantly more individuals with myopia (30%) in the adult onset group compared to the childhood onset group (6%) (P = .004). Thirty-seven (16%) had positive neuroimaging findings (16.4% adult onset vs 9.4% childhood onset). A total of 148 (44%) patients underwent surgery for esotropia, and the overall success rate was 73%. Mean preoperative esotropia was comparable in either group (P = .20), but surgical success was better in the adult onset group (75.6% vs 66.3% in the childhood onset group). Mean duration of follow-up postoperatively was 13.6 ± 12 months. CONCLUSIONS: Two-thirds of the patients had childhood onset of AACE. Intracranial pathology was found in 1 of 6 patients. Surgical success was better in the adult onset group, which was not influenced by preoperative esotropia, neuroimaging findings, or refractive status, but was dependent on age at onset of esotropia and duration between onset and intervention. [J Pediatr Ophthalmol Strabismus. 2023;60(3):218-225.].

"Peek-a-boo" Sign in Pediatric Cataract Surgery.

The authors demonstrate a "peek-a-boo" sign for intra-operative evaluation of the posterior pole of the fundus during pediatric cataract surgery. [J Pediatr Ophthalmol Strabismus. 2022;59(5):e54.].

Retinal nerve fiber layer thickness in amblyopia.

Purpose: To report retinal nerve fiber layer thickness (RNFLT) in eyes with amblyopia compared with contralateral healthy eyes. Methods: In this cross-sectional study, we included patients with anisometropic amblyopia, strabismic amblyopia, and mixed amblyopia. All subjects underwent complete ophthalmic examination, including RNFLT measurement with time-domain OCT (Stratus OCT) and scanning laser polarimeter (GDX VCC). A paired "t" test was used to compare average and quadrant-wise RNFL thickness between the amblyopic and contralateral normal eyes. In addition, an analysis of variance test was used to compare various RNFL thickness parameters between the three groups. Results: A total of 33 eyes of 33 subjects with anisometropic amblyopia, 20 eyes of 20 subjects with strabismic amblyopia, and 38 eyes of 38 subjects with mixed amblyopia were included. In the anisometropic amblyopia group, the average RNFLT in the amblyopic eye was 98.2 µm and 99.8 µm in the fellow normal eye (P = 0.5), the total foveal thickness was 152.82 µm (26.78) in the anisometropic eye and 150.42 µm (23.84) in the fellow eye (P = 0.38). The difference between amblyopic and contralateral normal eye for RNFL and macular parameters was statistically insignificant in all three groups. The RNFL thickness in four quadrants was similar in the amblyopic and non-amblyopic eye between all three groups and statistically non-significant. Conclusion: Our study showed that RNFL thickness was similar in amblyopic and non-amblyopic eyes between all three amblyopia groups.

Optic chiasm, optic tract and deep white demyelination: an unusual distribution of myelin oligodendrocyte glycoprotein-associated demyelination (MOGAD), case report and review of literature.

A preschool girl presented with sudden-onset bilateral painless loss of vision from 2 days prior. Child's examination showed light perception vision, sluggishly reacting pupils, otherwise normal anterior segment, healthy optic disc and retina in both eyes. MRI of brain and orbit with contrast revealed thickened left part of the optic chiasm with contrast enhancement extending proximally to bilateral optic tract and hyperintensities in the left thalamus and periventricular white mater. Considering the topographical distribution of lesions in the brain, neuromyelitis optica spectrum disorder was suspected. The child was started on intravenous methylprednisolone followed by tapering oral steroids. Serological testing was positive for myelin oligodendrocyte glycoprotein (MOG) and negative for aquaporin-4 antibodies. This case represents an unusual case of MOG associated demyelination disorder where the distribution of lesions showed chiasmal involvement along with optic tract, thalamus and deep white mater lesions.

Systemic Lupus Erythematosus and Third Nerve Palsy: Unusual Presentation and Review of the Literature.

We report the case of a young female with pyrexia of unknown origin, cutaneous macules and an incomplete third cranial nerve palsy, that led to the diagnosis of systemic lupus erythematosus (SLE) with neurological manifestations. Her visual acuity was normal. Fundus examination showed cotton wool spots in both eyes. Neuroimaging was also normal. Systemic work up revealed pancytopaenia, hypocomplementaemia, and the presence of multiple autoantibodies including anti-double stranded deoxyribonucleic acid and lupus anticoagulant. She was effectively treated with intravenous pulsed corticosteroid therapy, cyclophosphamide, and oral hydroxychloroquine. This case highlights the uncommon involvement of cranial nerve mononeuropathy in SLE, the importance of systemic examination and autoimmune workup in young patients with such a presentation.

Superior Rectus Transposition in the Management of Duane Retraction Syndrome: Current Insights.

Various surgical approaches have been described for the management of Duane retraction syndrome (DRS), a type of congenital cranial dysinnervation disorder (CCDD), the goals of which include correcting the primary position deviation and abnormal head posture (AHP), minimizing globe retraction and overshoots and improving the ocular rotations. Vertical rectus transposition (VRT) is one such technique, found more effective in improving abduction and thereby expanding the field of binocular vision, as compared to horizontal muscle surgery. VRT, however, is associated with the risk of inducing vertical deviations and also poses a risk for development of anterior segment ischemia. To overcome these concerns, transposition of only the superior rectus to the lateral rectus was proposed and evaluated to reveal improvement in alignment, AHP and motility comparable to VRT but with lesser surgical time and fewer post-operative complications. With promising results in the management of DRS, superior rectus transposition (SRT) has been extensively studied and has evolved over the last decade with several modifications to further increase the efficiency and reduce the risk of post-operative complications. This article focusses on the pre-operative considerations while planning SRT in DRS, various approaches and surgical techniques described, and the outcomes and complications of SRT in DRS. The role of SRT in the management of other CCDDs may be explored with further studies.

Disc Oedema in A Patient with Chronic Kidney Disease: A Diagnostic Conundrum.

A 47-year-old woman who presented with headache and blurring of vision was referred to us due to suspicion of idiopathic intracranial hypertension or cerebral sinus venous thrombosis. She had chronic kidney disease and underwent dialysis through multiple ports including the right internal jugular vein (IJV). Her examination showed a best corrected visual acuity of 20/20 in each eye, normal anterior segments in each eye but bilateral papilloedema. Magnetic resonance imaging and venography (MRV) of her brain with contrast showed signs of raised intracranial pressure and a hypoplastic left transverse sinus. An MRV of her neck showed a thrombosis of the right IJV. Her symptoms and papilloedema resolved with carbonic anhydrase inhibitors and anticoagulants. This case highlights an uncommon presentation of papilloedema secondary to raised intracranial pressure from IJV thrombosis and its pathogenesis.

Clinical profile, diagnostic challenges, and outcomes in subacute/chronic cerebral sinus venous thrombosis.

PURPOSE: To report clinical profile, diagnostic challenges, and outcomes in cases of subacute/chronic cerebral sinus venous thrombosis (CSVT) presenting to neuro-ophthalmologists/neurologists. METHODS: This was a multicentric, retrospective, observational study. Records of patients with neuroimaging proven subacute/chronic CSVT seen the from January 1, 2016 to March 31, 2020 were analyzed. Data collected included duration of symptoms, diagnosing physician, ophthalmological vs. focal/generalized neurological symptoms, optic disc examination, perimetry, and neuroimaging findings. Statistical analysis was performed using STATA software. RESULTS: Forty-three patients with subacute (30)/chronic (13) CSVT were identified (32 males, 11 females). Median age was 37 (IQR 27-47) years. The presenting complaints were blurred vision 34 (79%), headaches in 25 (58%), vomiting 12 (28%), and diplopia 11 (26%). Eleven patients had associated sixth cranial nerve palsy. All but two patients had either disc edema/optic atrophy; four had unilateral disc edema at presentation. Ophthalmologists and neurologists diagnosed/suspected CSVT correctly in 13/29 (45%) and 11/14 (78.5%) patients, respectively. Most common initial alternate diagnosis was idiopathic intracranial hypertension in 12 (28%). Female gender, age ≤36, unilateral papilledema, not obtaining venogram at initial workup increased chances of initial alternate diagnosis. Median follow-up duration was 21 days. Average visual function remained stable in majority of patients at last follow-up. In total, 47.6% of patients had best-corrected visual acuity ≥20/30 at the final follow-up. CONCLUSION: In our series, subacute or chronic CSVT presented presented primarily with symptoms of intracranial hypertension. Unilateral papilledema, middle-aged patients, female gender, lack of focal/generalized neurological symptoms created diagnostic dilemma. Visual function remained stable in majority of patients.

Working up a child with demyelinating optic neuritis: Striking a balance!

Pediatric optic neuritis (PON) is one of the commonest causes of acute vision loss in children. Although it might often be postinfectious or postvaccination, recent understanding and available evidence suggest that it can be the first manifestation of a neuro-inflammatory syndrome such as multiple sclerosis, neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis or myelin oligodendrocyte glycoprotein associated optic neuritis. Therefore, neuroimaging, serological testing, cerebrospinal fluid analysis, testing for various systemic autoimmune conditions become a part of the workup. However, this can be exhaustive and expensive, especially in countries with limited access to health insurance. Many recent studies suggest that neuroimaging and few clinical features can provide clues to the underlying etiology. However, serological tests can provide a confirmatory evidence. Therefore, in this mini-review, we propose a balanced approach to the evaluation of PON, based on the available literature emanating in the last decade.