Relentless placoid chorioretinitis: A review of four cases in pediatric and young adult patients with a discussion of therapeutic strategies.

Introduction: Relentless placoid chorioretinitis (RPC) is a rare, bilateral disease of the retinal pigment epithelium. The clinical course is prolonged and relapsing. No standard treatment has been established to date. The purpose of this case series is to report four cases of RPC in pediatric and young adult patients in which varying treatments were used, comparing them to previously published cases. Methods: A literature review was conducted to investigate currently published presentations and treatment options for RPC. A multicenter retrospective chart review was also performed on four consecutive patients. These patients were diagnosed with RPC because of new chorioretinitis lesions continuing to appear without or despite therapy for 5-36 months (2 patients), with a clinical course prolonged and relapsing, or because of the atypical location of the multiple lesions (>50) extending from the posterior pole to the equator and mid-peripheral retina (all four patients), which were not consistent with other entities like acute posterior multifocal placoid pigment epitheliopathy and serpiginous choroiditis. Results: All four cases of RPC received oral or IV steroids acutely, and three of these patients were transitioned to a steroid-sparing agent and biologic therapy: anti-TNF alpha or anti-IL-6. Quiescence of the chorioretinitis lesions was obtained after 7 months, 1 month, and 36 months; however, the latter had issues with treatment adherence. Mycophenolate mofetil was insufficient to control the disease in one patient, but tocilizumab and infliximab thereafter were effective after cessation of adalimumab due to side effects. Adalimumab when started the first month after the presentation was effective in controlling the disease in one patient. After the failure of interferon-alpha-2a, one patient displayed long-term control with infliximab. One patient did not require a steroid-sparing agent after oral prednisone taper as there was no evidence of progression or recurrence. Conclusion: This case series adds to the current knowledge regarding potential treatments for RPC, specifically the use of anti-TNF-alpha treatment and anti-IL-6 tocilizumab. In this case study, relapses of RPC were found among patients on mycophenolate mofetil and interferon-alpha-2a, and one case did not relapse on oral steroids without a steroid-sparing agent. Our findings suggest that adalimumab, infliximab, and tocilizumab may be useful medications to obtain quiescence of RPC.

Ocular Manifestations in Patients with Sensorineural Hearing Loss.

Identification of ocular manifestations in patients with sensorineural hearing loss (SNHL) can have a large impact on the outcome and treatment of pediatric patients. Due to the common co-incidence of ocular manifestations and SNHL in children, both ophthalmologic and hearing loss screening and routine examinations must be conducted to minimize adverse outcomes and worsening of pathology. Early evaluation and diagnosis is imperative for intervention and further development of the patient. Co-incidence requires a thorough evaluation that includes a comprehensive history, examination, and diagnostic testing. In this article, a literature review was conducted to analyze the presentations of various diseases and syndromes, such as Alport Syndrome, Waardenburg Syndrome, Norrie Disease, Usher Disease, Stickler Syndrome, Marfan Syndrome, Congenital Rubella, and Hereditary Optic Neuropathies. We divided the various ocular pathologies into anterior and posterior segment presentations and associated systemic findings for better understanding. Additionally, this review aims to include an update on the management of patients with both ocular and hearing loss manifestations.

Spasm of near reflex: a comprehensive management protocol and treatment outcomes.

PURPOSE: To report a consolidated management protocol for patients with spasm of near reflex (SNR), including classification of cases as mild, moderate, and severe based on treatment outcomes. METHODS: Patients with SNR treated at a single institution between August 2016 and November 2018 were included. Management of SNR included modified optical fogging, vision therapy, and pharmacological intervention (cyclopentolate eye drops and, if required, atropine eye drops). Outcome measures were visual acuity (20/25 or better) and refractive error (reduction of excessive myopia). RESULTS: Of 1,306 patients examined during the study period, 66 were diagnosed with SNR, yielding a prevalence of 5% among first-time patients visiting our binocular vision and orthoptics clinic. Of the 45 patients recruited for this study (mean age, 14 ± 5 years; 24 males), all three near-triad components were involved in 11 patients (24%), only the accommodation component in 32 (71%), and only the convergence component in 2 (4%). SNR was relieved in the first post-cyclopentolate refraction visit or with the modified optical fogging technique in 29 patients (66%; mild SNR) and with one-time usage of atropine eyedrops in 10 patients (22%; moderate SNR). In 6 patients (13%), atropine was continued long-term (severe SNR). Of 15 patients with long-term follow-up (1 year), 11 (73%) had persistent relief of SNR. CONCLUSIONS: In our study cohort, SNR with accommodation component was the most common and could be largely relaxed through a one-time use of cycloplegic eye drops and optical intervention. Only severe forms of SNR may require extended use of strong cycloplegics.

Profile of congenital cataract in the first year of life from a tertiary care center in South India - A modern series.

Purpose: To report the etiology, clinical presentation, and morphology of congenital cataract in a tertiary care center. Methods: It is a prospective cohort study conducted at L V Prasad Eye Institute, Hyderabad. All children with congenital cataract ≤ 12 months of age that required surgical intervention between August 2015 and July 2016 were included in the study. 109 such patients were subjected to meticulous history taking, pedigree charting, ocular, and systemic examination, B-scan, TORCH testing, clinical photographs, pediatrician consult and blood tests, which included serum calcium, serum phosphorous and urine for reducing sugars. Results: The mean age of presentation was 4.1 months (±2.6 months) and both the genders were equally affected (P = 0.49). Eighty-five patients (77.9%) presented with bilateral cataracts while 24 patients had a unilateral presentation (22.1%). The common morphological presentation was either a total or a nuclear cataract, both variants noticed in 47 patients (43.1%). TORCH infections were responsible for a maximum (37 patients, 33.4%) number of cases followed by familial (20 patients, 18%) and developmental anomalies (11 patients, 10.1%) while the total number of idiopathic cases were 24% (27 patients). Eighteen patients (16.5%) had congenital heart defects and the majority (16 patients, 88.9%) of these had positive TORCH titres. Conclusion: Familial cataract and those possibly due to TORCH are still the predominant cause of congenital cataract in this series-highlighting the role of vaccination and preventive measures.

Developing a pediatric ophthalmology telemedicine program in the COVID-19 crisis.

PURPOSE: To describe our methodology for implementing synchronous telemedicine during the 2019 novel coronavirus (COVID-19) pandemic. METHODS: A retrospective review of outpatient records at a single children's hospital from March 21 to April 10, 2020, was carried out to determine the outcome of already-scheduled face-to-face outpatient appointments. The week leading up to the March 21, all appointments in the study period were categorized as follows: (1) requiring an in-person visit, (2) face-to-face visit that could be postponed, and (3) consultation required but could be virtual. Teams of administrators, schedulers, and ophthalmic technicians used defined scripts and standardized emails to communicate results of categorization to patients. Flowcharts were devised to schedule and implement telemedicine visits. Informational videos were made accessible on social media to prepare patients for the telemedicine experience. Simultaneously our children's hospital launched a pediatric on-demand e-consult service, the data analytics of which could be used to determine how many visits were eye related. RESULTS: A total of 237 virtual ophthalmology consult visits were offered during the study period: 212 were scheduled, and 206 were completed, of which 43 were with new patients and 163 with returning patients. Following the initial virtual visit, another was required on average in 4 weeks by 21 patients; in-person follow-up was required for 170 patients on average 4.6 months after the initial virtual visit. None needed review within 72 hours. The pediatric on-demand service completed 290 visits, of which 25 had eye complaints. CONCLUSIONS: With proper materials, technology, and staffing, a telemedicine strategy based on three patient categories can be rapidly implemented to provide continued patient care during pandemic conditions. In our study cohort, the scheduled clinic e-visits had a low no-show rate (3%), and 8% of the on-demand virtual access for pediatric care was eye related.

Orbital Apex Syndrome: A Review.

Orbital apex syndrome is characterized by vision loss from optic neuropathy and ophthalmoplegia due to the involvement of ocular motor nerves in the anatomical region of the orbital apex. Patients could present with signs and symptoms deriving from the involvement of structures within the orbital apex, the superior orbital fissure or the cavernous sinus. The primary focus of the ophthalmologist should be to locate the lesion and then identify its etiology. Clinical evaluation holds key to diagnosis which is aided then by certain serological and lab investigations and neuro-imaging modalities including brain and orbital MRI (Magnetic Resonance Imaging) with contrast, CT (Computed Tomography) scans. In rare instances, a biopsy may be needed to aid in diagnosis. Treatment depends on what the nature of the lesion is with inflammatory conditions usually responding to steroids and infections to anti-microbial agents. Through this review, the authors attempt to decode the approach to localizing the lesion, the etiopathology and the management of cases of orbital apex syndrome.

Choroidal Vascularity in Non-arteritic Anterior Ischaemic Optic Neuropathy.

This study evaluates the peripapillary choroidal vascularity in eyes with non-arteritic anterior ischaemic optic neuropathy (NAION) and compares it with the vascularity of healthy fellow eyes and age-matched subjects. The peripapillary choroidal vascularity index (CVI), a new tool of measurement, was calculated using horizontal swept-source optical coherence tomography scans. CVI was calculated using a previously validated automated algorithm. CVI in NAION and fellow eyes of NAION patients were compared with age-matched eyes of healthy individuals using Kruskal-Wallis test. A total of 20 eyes of 20 patients with acute unilateral NAION with healthy fellow eyes (20 eyes) and 40 eyes of 40 healthy patients were included in the study. The average age of patients with NAION was 56 ± 8 and 55 ± 7 years in age-matched healthy controls. NAION eyes had a significantly lower CVI than age-matched controls in both nasal and temporal areas. NAION nasal CVI was 0.47 ± 0.47 compared to 0.62 ± 0.04 in controls (p < 0.001). NAION temporal CVI was 0.45 ± 0.48 compared to 0.58 ± 0.04 in controls (p < 0.001). Temporal CVI was 0.45 ± 0.48 in NAION eyes and was significantly lower than counterpart healthy fellow eyes 0.48 ± 0.02 (p = 0.007). In conclusion, NAION eyes have significantly reduced vascularity in the peripapillary area. CVI is lower in the nasal and temporal of the optic disc compared to healthy individuals. This may suggest those with smaller CVI are more prone to ischaemia from reduced vascularity resulting in NAION.

Orbital apex syndrome secondary to optic nerve cysticercosis.

A 22-year-old male presented to us with complaints of sudden painful loss of vision in left eye 10 days ago along with inward deviation of the left eye. Best-corrected visual acuity (BCVA) in right eye was 20/20 and 20/50 in left eye. Left eye showed limitation of abduction, a relative afferent pupillary defect, normal anterior segment with optic disc oedema. Contrast-enhanced MRI of the brain and orbit showed thickening of left optic nerve along with a cystic lesion near the orbital apex with a central iso- to hyperintense spot resembling a scolex. A diagnosis of left orbital apex syndrome secondary to optic nerve cysticercosis was made. Patient was treated with oral albendazole and intravenous corticosteroids for 3 days followed by oral corticosteroids. Ten weeks post-treatment, his BCVA in the left eye improved to 20/20 and colour vision and visual fields improved. Pallor of the left optic disc was noted, and ocular motility improved completely. MRI after treatment showed a decreased thickness of left optic nerve with disappearance of the cystic lesion.

Cataract surgery in children with retinopathy of prematurity (ROP): surgical outcomes.

PURPOSE: To report the outcomes of cataract surgery in children with retinopathy of prematurity (ROP). METHODS: A retrospective case review of all children diagnosed with ROP from January 2001 to December 2014 was done and those who underwent cataract surgery were included in the study. Details of ROP and cataract treatment, postoperative complications and outcomes were analysed. RESULTS: Of the 2258 children diagnosed to have ROP, 28 eyes of 22 children were included, 14 boys and 8 girls. Mean age at cataract surgery was 18.9 months (range 2 months to 12 years). Most common grade of ROP was stage 4 (13 eyes). Nineteen eyes underwent retinal surgery, scleral buckle (one eye) and laser (three eyes). Five eyes showed spontaneous regression. Mean duration for the development of cataract postretinal surgery was 7.76 months (range 2-32 months). Nine eyes did not receive a primary intraocular lens (IOL). Intraoperative posterior capsular rupture occurred in two eyes. Postoperative complications included visual axis opacification (four), secondary glaucoma (two) and IOL capture (one). Postoperative visual acuity assessment was possible in 23 eyes, 11 had better than 20/200 vision. Eleven patients had a follow-up of at least 2 years and the mean myopic shift at 2 years was -3.07 D in pseudophakes and -8.75 D in aphakes. CONCLUSIONS: Cataracts may develop in children with ROP regardless of the modality of intervention. Postoperative complications and refractive changes are similar to those in eyes without ROP.

Augmented superior rectus transposition with medial rectus recession in patients with abducens nerve palsy.

PURPOSE: To evaluate the surgical outcome of augmented superior rectus transposition (SRT) and medial rectus recession (MRc) in patients with abducens nerve palsy. METHODS: The medical records of consecutive patients with abducens nerve palsy who underwent unilateral or bilateral simultaneous SRT with MRc from January 2012 to December 2014 were analyzed. Patients with previous strabismus surgery or botulinum toxin injection were excluded. Primary outcome measures were esotropia in primary position and abduction deficit. Data collected included age, sex, etiology, pre- and postoperative deviation, pre- and postoperative abduction deficit, anomalous head posture, induced vertical or torsional deviations postoperatively, reoperations, and details of other complications. Success was defined as postoperative alignment within 10Δ of orthotropia; failure, as residual esotropia of ≥20Δ. RESULTS: A total of 15 eyes of 13 patients were included. The most common cause of abducens nerve palsy was trauma (10 patients). The mean preoperative esotropia was 55.4Δ ± 24Δ, which improved postoperatively to 9.9Δ ±10Δ (P = 0.0000). The mean preoperative abduction deficit was -5 units, decreasing postoperatively to -3.1 (P = 0.000). Nine patients (69%) achieved success; 2 were classified as failures. One patient each developed postoperative hypotropia and intorsion; however, these were transient and did not require additional procedures. No patients developed anterior segment ischemia. CONCLUSIONS: Augmented SRT with MRc is effective in the management of abducens nerve palsy; however, its success in large deviations remains variable. Long-term follow-up is essential to determine the incidence of vertical and torsional deviations.

Complex Cases in Pediatric Cataract.

This chapter describes the techniques and pitfalls that the reader may come across when dealing with complex pediatric cataract cases. Each eye in these circumstances is unique, and the examples and general advice shared are intended to help the reader develop a plan for surgery and a road map to avoid potential problems. As in all types of surgery, careful planning is essential. The old saying 'Fail to prepare, then you prepare to fail' is no more true than when dealing with children who have complex cataract. In this chapter, the following circumstances where pediatric cataract may be seen are discussed: retinoblastoma, retinopathy of prematurity, lenticonus, congenital rubella syndrome, trauma, microcornea, pediatric uveitis, Hallermann-Streiff syndrome, Stickler syndrome, Lowe syndrome, subluxated lens, and after previous intraocular surgery (glaucoma, keratoplasty).

Acute unilateral vision loss with optic disc oedema in retinitis pigmentosa.

A 36-year-old woman presented with acute vision loss and was found to have disc oedema and retinitis pigmentosa (RP). She presented with a history of acute, painless vision loss in her left eye over a period of 10 days. Her best-corrected visual acuity was 20/50, N6 in the right eye (OD) and 20/160, N6 in the left eye (OS). She was found to have a swollen optic disc and the examination of her fundus showed changes suggestive of RP. The diagnosis of RP was confirmed by electroretinogram, and after ruling out demyelinating changes in the central nervous system and other possible infectious causes of papillitis, she was treated with intravenous steroids followed by a course of oral steroid therapy. Following treatment, her visual acuity improved to 20/60. Acute vision loss may occur in patients with RP and prompt steroid therapy may result in partial visual recovery.

Ophthalmic profile and systemic features of pediatric facial nerve palsy.

BACKGROUND: Facial nerve palsy (FNP) occurs less frequently in children as compared to adults but most cases are secondary to an identifiable cause. These children may have a variety of ocular and systemic features associated with the palsy and need detailed ophthalmic and systemic evaluation. METHODS: This was a retrospective chart review of all the cases of FNP below the age of 16 years, presenting to a tertiary ophthalmic hospital over the period of 9 years, from January 2000 to December 2008. RESULTS: A total of 22 patients were included in the study. The average age at presentation was 6.08 years (range, 4 months to 16 years). Only one patient (4.54%) had bilateral FNP and 21 cases (95.45%) had unilateral FNP. Seventeen patients (77.27%) had congenital palsy and of these, five patients had a syndromic association, three had birth trauma and nine patients had idiopathic palsy. Five patients (22.72%) had an acquired palsy, of these, two had a traumatic cause and one patient each had neoplastic origin of the palsy, iatrogenic palsy after surgery for hemangioma and idiopathic palsy. Three patients had ipsilateral sixth nerve palsy, two children were diagnosed to have Moebius syndrome, one child had an ipsilateral Duane's syndrome with ipsilateral hearing loss. Corneal involvement was seen in eight patients (36.36%). Amblyopia was seen in ten patients (45.45%). Neuroimaging studies showed evidence of trauma, posterior fossa cysts, pontine gliosis and neoplasms such as a chloroma. Systemic associations included hemifacial macrosomia, oculovertebral malformations, Dandy Walker syndrome, Moebius syndrome and cerebral palsy CONCLUSIONS: FNP in children can have a number of underlying causes, some of which may be life threatening. It can also result in serious ocular complications including corneal perforation and severe amblyopia. These children require a multifaceted approach to their care.

Ocular myasthenia gravis: a review.

Myasthenia gravis (MG) is a disease that affects the neuro-muscular junction resulting in classical symptoms of variable muscle weakness and fatigability. It is called the great masquerader owing to its varied clinical presentations. Very often, a patient of MG may present to the ophthalmologist given that a large proportion of patients with systemic myasthenia have ocular involvement either at presentation or during the later course of the disease. The treatment of ocular MG involves both the neurologist and ophthalmologist. Thus, the aim of this review was to highlight the current diagnosis, investigations, and treatment of ocular MG.

Etiology and clinical profile of childhood optic nerve atrophy at a tertiary eye care center in South India.

BACKGROUND: Optic nerve atrophy is an important ophthalmological sign that may be associated with serious systemic conditions having a significant bearing on the overall morbidity of the child. Studies specific to etiology of childhood optic atrophy are scarce, this being the first such study from India to the best of our knowledge. AIM: The aim was to analyze the clinical features and etiology of diagnosed cases of optic nerve atrophy in children <16 years of age. MATERIALS AND METHODS: Retrospective review of records of children diagnosed with optic nerve atrophy between the ages of 0 and 16 years from 2006 to 2011. RESULTS: A total of 324 children (583 eyes) were identified. Among these 160 (49%) presented with defective vision, 71 (22%) with strabismus, 18 (6%) with only nystagmus. Rest had a combination of two or three of the above symptoms. Sixty-five patients (20%) had a unilateral affection. Hypoxic ischemic encephalopathy seen in 133 patients (41%) was the most frequent cause of childhood optic atrophy, followed by idiopathic in 98 (30%), hydrocephalus in 24 (7%), compressive etiology in 18 (5%), infective in 19 (6%), congenital in 6 (2%), inflammatory in 5 (2%) patients, respectively. CONCLUSION: Hypoxic ischemic encephalopathy appears to be the most common cause of optic atrophy in children in this series. The most common presenting complaint was defective vision.