Seroprevalence of MOG and AQP4 Antibodies and Outcomes in an Indian Cohort of Pediatric Acquired Demyelinating Syndromes.

This study aimed to determine the seropositivity of myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) and aquaporin-4 antibodies (AQP4-Ab) and outcomes in children with acquired demyelinating syndromes (ADSs). Children (6 months-15 years) with suspected ADS were enrolled and tested for MOG-Ab and AQP4-Ab prospectively over 18 months at a tertiary care hospital in North India. Children with proven nonimmune-mediated neurological disorders were enrolled as controls. Of 79 children with suspected ADS, 66 were enrolled. Among the enrolled children with ADS, acute demyelinating encephalomyelitis (ADEM) (25) was the most common first clinical event followed by optic neuritis (ON) (20) and transverse myelitis (TM) (19; one child had ON and TM simultaneously [neuromyelitis optica spectrum disorders [NMOSDs]]), while two children had clinically isolated syndrome (CIS) apart from ON and TM. Fourteen (21.2%, confidence interval [CI] 11.3-31.1) tested positive for one antibody (12 [18.1%; 95% CI 10.5-25.5%] for MOG-Ab and 2 [3%; 95% CI 0-7.2%] for AQP4-Ab). None of the 62 controls tested positive for any antibody. The final diagnosis in those with the monophasic ADS was ADEM (21), ON (13), TM (16), and other CIS (1) while that in children with recurrent events was multiphasic disseminated encephalomyelitis (MDEM) (2), NMOSD (3), ADEM-ON (4), recurrent ON (4), and MS (2). Among those with the first event, 4/51 (7.8%; 95% CI 0.5-15.2%) were MOG-Ab positive and 2 AQP4-Ab positive, whereas 8/15 (53.3% [95% CI 28.1-78.6%]) with recurrent events (MDEM [2], ADEM-ON [4], recurrent ON [1], and recurrent TM [1]) were MOG-Ab positive. Hence, MOG-Abs are the most common antibodies detected in one in five children with pediatric ADS, especially in relapsing disease. AQP4-Abs are rare in children with ADS.

Surgical outcomes of cataract surgery in anterior and combined persistent fetal vasculature using a novel surgical technique: a single center, prospective study.

PURPOSE: To study the complications and surgical outcomes of cataract surgery in patients of persistent fetal vasculature (PFV) with cataract. METHODS: In this prospective study, phacoaspiration with/without intraocular lens implantation (IOL) was done in 20 children (mean age 14.2 months) with unilateral cataract with anterior (n = 6) or combined (n = 14) PFV. The rentrolental vascularized membrane was cauterized and dissected circumferentially, followed by cauterization and resection of the PFV stalk. The outcome measures included fixation preference using the CSM (central, steady, maintained) method and intraoperative and postoperative complications in an 18-month follow-up. The difference in outcomes of anterior and combined PFV, as well as aphakic and pseudophakic eyes, was studied. RESULTS: CSM fixation was seen in 16 patients after 18 months. The intraocular lens was implanted in 16 eyes and 4 eyes with combined PFV were left aphakic. None of our patients had intraoperative bleeding. Visual axis obscuration was the major complication seen, requiring membranectomy in 8 children. Pupilloplasty was required with membranectomy in one eye. None of our patients developed glaucoma or retinal detachment. CONCLUSION: Timely surgical intervention and aggressive amblyopia therapy led to good visual results in our study. Poor prognosis was seen in combined PFV, aphakia, and microphthalmia.

Causes and Outcomes of Patients Presenting with Diplopia: A Hospital-based Study.

Diplopia or double vision has various aetiologies and often presents to the ophthalmologist. The purpose of our study was to study in detail the aetiology, progression, and outcome in patients of diplopia presenting to the ophthalmology department of a tertiary care centre. In a prospective observational study, all patients presenting to the emergency services and the outpatient department of a tertiary care centre over a period of 1 year were recruited. One hundred and sixty patients who presented with diplopia with a mean age of 40 (range 7-76) years were included. There were 112 (70%) males and 48 (30%) females. The most common cause for binocular diplopia was vasculopathy in 43 (28.66%) followed by trauma in 37 (24.66%). In those less than 20 years decompensating heterophorias were most common and had the best prognosis. Traumatic causes were common in the age group 20-40 years and vascular in the >40 years age group. For all other aetiologies, elderly patients had more resolution than young patients (p = .04). Larger deviation at presentation, younger age group, and a history of trauma were associated with late recovery (p = .03. p = .04 and p = .04, respectively).

Diplopia after Excessive Smart Phone Usage.

Acute comitant esotropia in a child has a mixed set of differentials and we present a report of three cases in children who presented with acute onset diplopia. On careful history taking, all the kids reported an excessive use of the smart phone in the preceding month. We hypothesise that excessive use of the smart phone at near leads to excessive stimulation of ciliary muscle, hence accommodative spasm in these children. This is the first case series to report an association of smart phones and accommodative spasm.

Cataract surgery in infants with microphthalmos.

PURPOSE: To report the postoperative outcomes of cataract surgery in microphthalmic eyes of infants. METHODS: This prospective observational study was carried out on 20 infants with microphthalmos with visually significant cataract. Microphthalmos was defined as axial length of the globe 16.50 mm or less. We excluded eyes with ocular trauma, inflammation, posterior hyperplastic primary vitreous or a tractional retinal detachment, aniridia, or chorioretinal coloboma. All the infants enrolled in this study underwent phacoaspiration with primary posterior capsulotomy, anterior vitrectomy, and peripheral iridectomy. Intraocular lens was not implanted in these children. Post-operative evaluation included refractive errors, irregularity of pupil, posterior synechiae, visual axis obscuration, and intraocular pressure. These children were followed up for a minimum of 12 months. RESULTS: We evaluated 37 eyes of 20 infants, of whom 17 infants had bilateral and three infants had unilateral cataract. The mean age of the children and the mean axial length at the time of surgery were 3.78 ± 2.25 months and 15.76 ± 0.56 mm respectively. The complications observed were irregularity of pupil in seven eyes (18.9 %), glaucoma in five eyes (13.5 %), posterior synechiae in two eyes (5.2 %), visual axis obscuration due to posterior capsule opacification (PCO) in two eyes (5.2 %) and phthisis in one eye (2.7 %). CONCLUSION: Infants achieved a favorable outcome after phacoaspiration with primary posterior capsulotomy with anterior vitrectomy. However, these children must be followed up to detect and treat postoperative complications such as visual axis obscuration, posterior synechiae, and glaucoma to achieve optimal outcome.

Extra-Pituitary Birth Defects May Predict Diagnosis of Congenital Hypopituitarism in a Short Child.

BACKGROUND: Extra-pituitary birth defect (EPBD) in children with congenital hypopituitarism is largely unknown. OBJECTIVE: The study aims to evaluate the incidence and pattern of EPBD in children with congenital hypopituitarism and to evaluate whether it can serve as a clue to diagnose this condition. PATIENTS AND METHODS: Retrospective analysis of hospital record of patients of short stature due to various etiology from which patients with congenital hypopituitarism with age ≥18 years were recruited for the analysis. Clinical, hormonal, radiological and ocular electrophysiological studies were done in all patients and all EPBD were noted. RESULTS: Twenty seven patients (79%) had multiple pituitary hormone deficiency (MPHD) of which growth hormone was universal followed by gonadotropin (62%), TSH (59%), ACTH (44%) and prolactin (12%). Nineteen patients (56%) had multiple EPBD in various combinations. Twenty three ocular abnormalities were present in 12 patients (35%). Nine patients (26%) had other associated EPBD along with ocular abnormalities while 3 had ocular abnormalities without any other associated birth defect. Skeletal defects were present in 10 patients (29.5%). On the contrary, 5 patients in the EPBD group had total 15 visual defects. The most common abnormality of the visual system were abnormal visual evoke response (VER, 18%), followed by strabismus (15%), visual acuity (VA, 12%), electroretinogram (ERG) and electrooculogram (EOG) 8% each and visual field defect 6%. There was a trend towards early age at presentation with EPBD. CONCLUSIONS: Presence of EPBD in a short child is a sensitive marker to diagnose congenital hypopituitarism. Subtle abnormalities of visual pathway without absent septum pellucidum or midline brain defects were common.

Surgical challenges of posterior optic capture in pediatric cataract surgery.

PURPOSE: The efficacy of posterior optic capture (POC) in reducing posterior capsule opacification (PCO) in pediatric cataract is well recognized. The purpose of this paper was to identify the surgical challenges when attempting this technique and highlight the etiquettes to follow when performing this maneuver. METHODS: Prospective observational noncomparative case series. Children diagnosed with congenital or developmental cataracts undergoing cataract surgery and primary IOL implantation with posterior optic capture (and no anterior vitrectomy) from June 2017 to April 2022 at a tertiary care referral institute were included. Records of all intraoperative findings and postoperative complications until the last follow-up were noted. RESULTS: Posterior optic capture was attempted in 53 eyes of 49 children aged 2.4 ± 1.98 years. The mean follow-up of the patients was 16.5 ± 14.2 months (range 6 months-5 years). Successful POC could be performed in 46 eyes (86.8%). Two eyes developed posterior capsular opacification at the last follow-up. In eyes where POC could not be performed, five of these (83%) were children below 12 months of age with half of them having a preexisting posterior capsular defect. CONCLUSION: Posterior optic capture is technically challenging with a steep learning curve that can be mastered over time. Adequate relative sizing of the anterior and posterior capsulorhexis is important. Caution is advised when using this technique in infants and in cases with posterior capsular defects.

Influence of intraocular inflammatory cytokine levels on postoperative complications in congenital cataract surgery.

PURPOSE: Posterior capsular opacification (PCO) accounts for the most significant and most challenging complication after successful cataract surgery in children. Our study aimed to investigate the levels of cytokines in the aqueous humor of children afflicted with congenital cataracts and their association with PCO. METHODS: This was an observational study conducted at a tertiary referral center in India. Consecutive children aged less than 5 years with unilateral/bilateral isolated idiopathic congenital cataracts and planned for cataract extraction with intraocular lens implantation were included. During cataract surgery, 100-500 µl of aqueous samples were collected and the levels of inflammatory cytokines were quantified in the aqueous humor. RESULTS: An analysis of 28 samples was done. The mean age of the patients was 16.7 ± 0.4 months (range 3-60 months) with a mean follow-up 14.2 ± 2.3 months. On binary logistic regression, the level of MCP-1 was significantly related to the development of PCO at 1 year (P = 0.0072). Levels of IL-6 and IL-8 were not associated with the development of PCO (P = 0.05 and P = 0.07, respectively). CONCLUSION: Inflammatory markers like IL-6 and IL-8 were raised in children with pediatric cataracts in our study. Chronic high levels of MCP-1-induced fibrosis may be associated with PCO.

Cataract surgery in children using intracameral mydriatic.

PURPOSE: To study the pupil dynamics with premixed intracameral anesthetic mydriatic combination of phenylephrine (0.31%), tropicamide (0.02%), and lidocaine (1%) in pediatric cataract surgery. METHODS: Consecutive children aged ≤12 years planned for cataract surgery were recruited. A commercially available premixed combination of phenylephrine (0.31%), tropicamide (0.02%), and lidocaine (1%) was injected at the beginning of surgery without any topical/infusion drugs for mydriasis. Pupil sizes at various points of surgery were studied. RESULTS: We recruited 75 patients with a mean age of 24.3 ± 33.4 months (range: 1 month-11 years). Adequate mydriasis with a single injection was achieved in 93.5% (n = 73 eyes of 70 patients) without additional pharmacotherapy or intervention. The mean pupillary diameter increased from 1.8 ± 0.79 to 6.1 ± 1.4 mm after injection (mean change of 4.2 ± 1.25 mm from baseline). The mean variability in pupillary diameter was 0.73 ± 1.3 mm. In five eyes, good dilatation was not possible even after repeat injection. CONCLUSION: Fixed-dose premixed intracameral injection is effective in pupil dilatation. It alleviates the need for any topical dilators or additional intraoperative supplementation for pediatric cataract surgery.

Anterior segment optical coherence tomography (AS-OCT) in strabismus following trauma.

We describe 3 cases of ocular trauma that presented with strabismus. Anterior segment optical coherence tomography was used to delineate the morphology of extraocular muscle insertions and proved helpful in diagnosis and management.

Clinical outcomes and Anti-MOG antibodies in pediatric optic neuritis: A prospective observational study.

OBJECTIVES: The objective of this study was to look at the clinical outcomes, and to determine the proportion of children with visual recovery after the first demyelinating event of optic neuritis (ON). METHODOLOGY: In this observational study, children with the first clinical event of optic neuritis at an age less than 18 years were evaluated. High-contrast visual acuity, colour vision, Expanded Disability Status Scale (EDSS), Anti-MOG and AQP-4 antibodies were assessed. RESULTS: Of the 55 screened, 45 children (77 eyes), median age-98 months, 30 (67%) bilateral were enrolled. Fifty of 77 eyes (67%) had Snellen visual acuity less than 6/60. Twelve children (27%) were MOG seropositive and 3 had AQP-4 positivity. At median follow up of 35 months, 10 (22%) children had one or more relapses. At follow up, the median (IQR) visual acuity improved from nadir of 2.1 (1-2.7) logMAR to 0 (0-0.18) logMAR and 64/77 eyes (83%) had visual recovery. The diagnosis at last follow up was isolated ON in 39/45 (86.6%), relapsing ON (5, 11%), AQP-4 positive NMOSD (3, 7%), MOG antibody associated demyelination (12, 27%), dual seronegative ON (30,67%) and Multiple sclerosis (1, 2%). CONCLUSIONS: Most children with first demyelinating event as ON have a monophasic illness. Despite severe acute-phase visual loss, most eyes with ON will recover good visual functions. The risk of AQP-4 disease and multiple sclerosis is low in this group.

Low-dose atropine 0.01% for the treatment of childhood myopia: a pan-India multicentric retrospective study.

OBJECTIVE: The objective of this study was to assess the efficacy of low-dose atropine 0.01% in controlling myopia progression among Indian children over a 2-year period. METHODS: This retrospective study, conducted across 20 centres in India, monitored the progression of myopia over 2 years after initiating treatment with 0.01% atropine eye drops. This included children between 6 and 14 years with baseline myopia ranging from -0.5 D to -6 D, astigmatism≤-1.5 D, anisometropia ≤ -1 D and documented myopia progression of ≥0.5 D in the year prior to starting atropine. Subjects with any other ocular pathologies were excluded. RESULTS: A total of 732 children were included in the data analysis. The mean age of the subjects was 9.3±2.7 years. The mean myopia progression at baseline (1 year before starting atropine) was -0.75±0.31 D. The rate of myopia progression was higher in younger subjects and those with higher baseline myopic error. After initiating atropine, myopia progression significantly decreased to -0.27±0.14 D at the end of the first year and -0.24±0.15 D at the end of the second year (p<0.001). Younger children (p<0.001) and higher baseline myopia (p<0.001) was associated with greater myopia progression and poor treatment response (p<0.001 for both). CONCLUSION: Low-dose atropine (0.01%) effectively reduces myopia progression over 2 years in Indian children.

Clinico-Radiological Spectrum and Management of Orbital Schwannomas: A Tertiary Care Institute Study.

Abstract Purpose: The aim is to study clinical presentations, radiological features and outcome in histopathologically proven orbital schwannomas. Methods: A retrospective and longitudinal study of 16 patients between January 1999 and July 2011 was undertaken by reviewing the appropriate charts. Clinical and radiological data of all orbital schwannomas patients were analyzed. Results: Mean age was 28.18 years (range 8-40 years). Of 16 patients, 10 were males and 6 females. Duration of symptoms was chronic in 15(93%). Chief complaints were proptosis in 10(62.5%), eyelid swelling in 5(31%) and eyelid mass lesion in 2(12.5%). This included a rare case of cystic schwannoma with microphthalmos. Mass was extraconal in 11(69%). Radiological findings varied from solid to cystic, intraconal to extraconal, no enhancement to intense enhancement and presence of calcification. Thirteen (81%) were excised via anterior orbitotomy route while fronto-orbital craniotomy was done in 3(19%). Postoperatively, vision improved or remained stable in 15(94%) and worsened in one (6%). Final visual acuity of 6/6 was achieved in 11(69%), 6/9 in 2, 6/36 in one. Conclusions: Schwannomas may mimic a variety of mass lesions in the orbit because of variable clinical or imaging features and should be considered in the differential diagnosis of all well circumscribed orbital lesions.

Long-term outcomes of single monocular resection-recession in adult sensory strabismus and factors affecting the postoperative drift.

Purpose: The purpose of our study was to analyze the clinical characteristics and outcome of horizontal strabismus surgery in patients having sensory strabismus and to analyze the factors that affect the postoperative drift in these patients over a follow-up of three years. Methods: This was a retrospective case series. Patients aged ≥18 years, having low vision (visual acuity ≤20/60) in one eye, and undergoing horizontal strabismus surgery (standard recess-resect procedures) in the same eye were recruited. All patients were advised patching of the good eye six weeks prior and continued for six weeks post strabismus surgery. We excluded patients who had paralytic disorders, motility defects, or those with chronic systemic conditions. Patients with a minimum follow-up of three years were recruited. Results: The study included 56 patients whose mean age was 22.9 ± 4.93 years. Exotropia (n = 38; 67.8%) was more common than esotropia (n = 18; 32.1%). Preoperative visual acuity was 1.1 ± 0.85 (range perception of light to 6/18p). The cause of low vision was amblyopia (n = 30; 53.5%) followed by trauma (n = 22; 39.2%). The mean preoperative distance deviation was 57.7 ± 15.5 PD in the primary position (range: 20-65 PD). The success rate of exotropia (78.9%) was more than esotropia (52.9%) at three years. Two patients with esotropia were overcorrected. All patients with exotropia showed an exotropic drift with time. Conclusion: The motor alignment after a single recession-resection procedure was satisfactory at the long-term in our cohort of sensory strabismus. The duration or extent of visual impairment had no relation to the postoperative outcome.

Pharmacological management of intra-operative miosis during cataract surgery.

Cataract surgery requires a well-dilated and stable pupil for a good outcome. Unexpected pupillary constriction during surgery increases the risk of complication. This problem is more pronounced in children. There are now pharmacological interventions that help tackle this unforeseen happening. Our review discusses the simple and quick options available to a cataract surgeon when faced with this dilemma. As cataract surgical techniques continue to improvise and get faster, an adequate pupil size is of paramount importance. Various topical and intra-cameral drugs are used in combination to achieve mydriasis. Despite good pre-operative dilation, the pupil can be quite unpredictable during surgery. Intra-operative miosis limits the field of surgery and increases the risk of complications. For example, if the pupil size decreases from 7 mm to 6 mm, this 1 mm change in pupil diameter will lead to a decrease of 10.2 mm2 in the area of surgical field. Making a good capsulorhexis with a small pupil can be a challenge, even for an experienced surgeon. Repeated touching of the iris increases the risk of fibrinous complications. Removal of cataract and the cortical matter becomes increasingly difficult. Intra-ocular lens implantation in the bag also requires adequate dilation. When dealing with challenging cases like lens subluxation, pseudo-exfoliation, and zonular dehiscence, a small pupil further increases the risk and adversely affects the surgical outcome. Hence, achieving and maintaining adequate mydriasis throughout surgery is essential. This review highlights the risk factors for small pupils during surgery and current management strategies.

Spectrum of congenital and inherited ocular disorders seen in a genetic clinic: Experience of a developing ocular genetic service.

Purpose: Hereditary causes are an important etiological category of childhood blindness. This study reports the real-world experience of a developing ocular genetic service. Methods: The study was carried out from Jan 2020 to Dec 2021 jointly by the Pediatric Genetic Clinic and the Department of Ophthalmology of a tertiary care hospital in North-West India. Children presenting to the genetic clinic with congenital or late-onset ocular disorder(s) and any individual (irrespective of age) suffering from an ophthalmic disorder and referred by an ophthalmologist for genetic counseling for himself/herself and/or his/her family member(s) were included. Genetic testing (exome sequencing/panel-based sequencing/chromosomal microarray) was outsourced to third-party laboratories with the cost of the test being borne by the patient. Results: Exactly 8.6% of the registered patients in the genetic clinic had ocular disorders. Maximum number of patients belonged to the category of anterior segment dysgenesis, followed by microphthalmia anophthalmia coloboma spectrum, lens disorders, and inherited retinal disorders in decreasing numbers. The ratio of syndromic ocular to isolated ocular disorders seen was 1.8:1. Genetic testing was accepted by 55.5% of families. The genetic testing was clinically useful for ~35% of the tested cohort, with the opportunity for prenatal diagnosis being the most useful application of genetic testing. Conclusion: Syndromic ocular disorders are seen at a higher frequency compared to isolated ocular disorders in a genetic clinic. Opportunity for prenatal diagnosis is the most useful application of genetic testing in ocular disorders.

Role of swept-source anterior segment optical coherence tomography in imaging pediatric cataract.

Purpose: To determine the morphology of pediatric cataracts and assess the status of the anterior and posterior capsules preoperatively on swept-source anterior segment optical coherence tomography (ASOCT) and compare the findings to those of intraoperative examination. Secondly, we aimed to obtain biometric measurements on ASOCT and compare them to those obtained on A-scan/optical methods. Methods: This was a prospective and observational study carried out at a tertiary care referral institute. ASOCT scans for anterior segment were obtained preoperatively for all patients, aged <8 years, scheduled for paediatric cataract surgery. The morphology of the lens and capsule and biometry were performed on ASOCT and the same were assessed intraoperatively. The main outcome measures were comparison of ASOCT findings to intraoperative findings. Results: The study included 33 eyes of 29 patients (range 3 months-8 years). The morphological characterization of cataract on ASOCT was accurate in 31/33 (94%) cases. ASOCT accurately identified fibrosis and rupture of the anterior and posterior capsules in 32/33 (97%) cases each. In 30% of eyes, ASOCT gave additional information preoperatively compared to the slit lamp. Intraclass correlation coefficient (ICC) calculation revealed a good agreement between the keratometry values obtained on ASOCT and those obtained preoperatively with a handheld/optical keratometer (ICC = 0.86, P = 0.001). Conclusion: ASOCT is a valuable tool that could provide complete preoperative information of the lens and capsule in pediatric cataract cases. In children as young as 3 months of age, intraoperative risks and surprises could be diminished. The keratometric readings are highly dependent on patient cooperation but show good agreement with the handheld/optical keratometer readings.

Tonic down-rolling and eccentric down-positioning of eyes under sevoflurane anesthesia without non-depolarizing muscle relaxant and its relationship with depth of anesthesia.

Purpose: To analyze the relationship between eccentric downward eye movement/eccentric downward eye-positioning (EDEM/EDEP) encountered in patients undergoing ophthalmic surgeries and its return to a centralized position under general anesthesia (GA) with the depth of anesthesia (DOA). Methods: Patients undergoing ophthalmic surgeries (6 months-12 years) under sevoflurane anesthesia without non-depolarizing muscle relaxant (NDMR) who witnessed a sudden tonic EDEM/EDEP were both retrospectively (R-group) and prospectively (P-group) enrolled (ambispective study). R-group included data-points after induction (AI) till the time surgery lasted while P-group compiled data both during induction (DI) and AI. DOA in terms of MAC (minimum alveolar concentration) at the time of EDEM/EDEP and centralization of eyeball and their timings were noted and compared for both AI and DI data-points. Also, vertical eccentric eye positions were scored and correlated with MAC. Results: AI data included 22 (14R+8P) events and their mean MAC of EDEM/EDEP and centralization were 1.60 ± 0.25 and 1.18 ± 0.17 respectively (p = 0.000). DI data included 62 (P) cases and its mean MAC of EDEM/EDEP and centralization was 2.19 ± 0.43 and 1.39 ± 0.26 respectively (p = 0.000). Median (IQR) eye positions during down-positioning in 84 events was -3 (-3.9 to -2.5). It was preceded by an eccentric upward drift of eyes in 10/22 (6R+4P) AI cases. A strong negative correlation was seen between DOA and eccentric eye positions (r = -0.77, p = 0.000). Conclusions: Tonic down-rolling of eyes is not uncommon in children seen without NDMR with higher depths of sevoflurane anesthesia compared to point of centralization and fluctuations in DOA should be avoided to circumvent inadvertent complications during ocular surgery.