Apert Syndrome - caveats of squint management.

Apert Syndrome (AS) is a rare form of acrocephalosyndactyly. The aim of the manuscript was to underline the challenging squint management in a case of Apert Syndrome. A 1.5-year-old male with craniosynostosis, diagnosed at birth, with history of incomplete closure of eyes, more so in the right eye, and squinting of left eye since birth, was brought to eye OPD by the mother. Presence of acrocephaly, prominent forehead with bony irregularity, chin down with left head tilt, fused cervical vertebrae, marked proptosis, cleft palate, dental anomaly and syndactyly confirmed the diagnosis of AS. Old serial photographs of the child were examined to look for progression of squint and proptosis. Squint evaluation revealed 70-75 PD exotropia with 10PD right hypertropia in primary gaze. The right hypertropia increased further in the left gaze, whereas a left hypertropia was noted in the right gaze. The patient underwent bilateral LR recession of 9 mm with full muscle width transposition (upshift) with Inferior Oblique recession of 4:1 mm in the right eye and 3:2 mm in the left eye. Post-operative follow-up after 2 months showed that V pattern collapsed with residual exotropia of 20 PD. Post-operative follow-up after 1 year showed improvement in head posture with pattern collapsed. However, recurrent exotropia was noted on evaluation, for which bilateral medial recti resection was done later. The management of squint in AS and other craniosynostosis poses a multitude of challenges for the ophthalmologists. Frequent follow-ups are needed in patients with AS for the timely management of its ocular manifestations and better visual rehabilitation.

Tuberculosis and neuromyelitis optica spectrum disorder: A conundrum.

Purpose: Infectious atypical optic neuritis (AON), like tubercular, is a vision threatening condition with phenotypic overlap with Neuromyelitis Optica Spectrum Disorder (NMOSD). The overlapping neurological manifestations and negative AQP4-Ab-assay make it difficult to discover the primary cause of neuritis. Case presentation: We report two paediatric cases with NMOSD that did not fulfil the diagnostic criteria. Moreover, associated undiagnosed tuberculosis at the time of presentation and negative AQP4-Ab clouded the diagnosis and delayed the treatment. The first case was initially diagnosed with infectious optic neuropathy. By the time steroids were started, optic atrophy had already set in. The second case had optic neuritis, LETM, and intracranial-tuberculomas with no signs of pulmonary-tuberculosis with negative CSF-analysis. So, systemic steroids were started promptly. The history of LETM in both cases raised the suspicion of NMOSD. Conclusion: The importance of accurate clinical diagnosis and early intervention in cases of AON was emphasized in a limited resource country, that could potentially result in salutary visual outcomes, especially in the paediatric age group Abbreviations: AON = atypical optic neuritis, TB = tuberculosis, NMOSD = neuromyelitis optica spectrum disorder, LETM = longitudinal extensive transverse myelitis, AQP4-Ab = anti-Aquaporin-4 Antibodies, RE = right eye, LE = left eye, MRI = magnetic resonance imaging, CSF = cerebrospinal fluid, ATT = anti-tubercular treatment, DOV = diminution of vision.

Eight years' experience in mobile teleophthalmology for diabetic retinopathy screening.

Background: Screening for diabetic retinopathy in the community without compromising the routine work of ophthalmologists at hospitals is the essence of teleophthalmology. This study was aimed at investigating the efficacy of teleophthalmology practice for screening diabetic retinopathy from 2012 to 2020. It was also aimed at comparing the 2-year prevalence of camps organized by a district hospital in South India, as well as the footfall, reporting, follow-up, patient response, and diagnostic efficacy at these camps. Methods: All patients with diabetes and unexplained vision deterioration attending the mobile camp units underwent non-dilated fundus photography. Patients underwent teleconsultation with the ophthalmologist at the district hospital, and those requiring intervention were called to the district hospital. Trends were studied for the number of patients reporting to the hospital. Patient satisfaction was recorded based on a questionnaire. Results: A total of 682 camps were held over 8 years, and 30 230 patients were examined. Teleconsultation was done for 12 157 (40.21%) patients. Patients requiring further investigations, intervention for diabetic retinopathy, or further management of other ocular pathologies were urgently referred to the district hospital (n= 3293 [10.89%] of 30 230 examined patients). The severity and presence of clinically significant macular edema increased significantly with an increased duration of diabetes mellitus (P < 0.001). The percentage of teleconsultations showed an increasing trend over the years (P = 0.001). Similarly, considering trends of patients reporting to the hospital, the attrition rate decreased over the years (P < 0.05). A total of 10 974 of 12 157 (90.27%) patients who underwent teleophthalmic consultation were satisfied with the service. Conclusions: Teleconsultations over the years showed an increasing trend, and the attrition rate decreased over the years. Teleophthalmology is achieving success in providing high-quality service, easy access to care, and in increasing patient satisfaction. Future studies on the role of teleophthalmology for other leading preventable causes of blindness seem possible and necessary.

Periventricular leukomalacia: an ophthalmic perspective.

Periventricular leukomalacia (PVL) is a common magnetic resonance imaging (MRI) finding in cases of hypoxic ischemic encephalopathy. PVL, in MRI, is identified by the increased signal intensity of periventricular white matter on T2-weighted sequences which is more conspicuous in the posterior cortex. It occurs because of perinatal damage to the cerebral cortex. This insult is in the form of hypoxia, metabolic insults, prematurity, seizures, or infection. Periventricular area is most prone to damage owing to its immaturity and vascular supply. PVL is proven to affect vision in children. Depending on the area and cause of affection, PVL is associated with variable ophthalmic manifestations. It is known that visual function is closely linked to the overall neurodevelopment of a child. A multidisciplinary approach is required to promote the growth and development of these children, and in the midst of multiple disabilities, visual function should not be overlooked. A comprehensive knowledge of the ophthalmological presentation in the developing world can aid us in an early and accurate diagnosis and in intervention for better therapeutic recovery and rehabilitation of these children.

Predisposing Factors, Ophthalmic Manifestations, and Radiological Findings in Children With Cerebral Visual Impairment.

PURPOSE: To describe predisposing factors, ophthalmic manifestations, and magnetic resonance imaging (MRI) findings in children with cerebral visual impairment. METHODS: A retrospective cross-sectional analysis of patients younger than 16 years with neuroradiological and clinical evidence of retrogeniculate visual pathway pathology was performed. Detailed histories, ophthalmic examinations, and MRI findings were compiled and analyzed. RESULTS: Of the 88 cases included in the study, the median age was 32 months (range: 1 to 180 months). Ante-natal history and preterm delivery was positive in 25.0% and 37.5% of patients, respectively. A simple myopic astigmatism was the most common refractive error. Accommodative anomalies were noted in 6 children. CONCLUSIONS: The demographic trends found in the study were similar to those of developed nations, but the frequency of the predisposing factors varied. A comprehensive knowledge of cerebral visual impairment in the developing world can aid an earlier diagnosis, appropriate management, and the development of better rehabilitation teams. [J Pediatr Ophthalmol Strabismus. 2019;56(5):313-318.].

Subconjunctival silicone oil complicating strabismus surgery.

Purpose: To report a case of an otherwise simple strabismus surgery that became complex due to the presence of extensive subconjunctival silicone oil. Methods: A 15-year-old boy underwent strabismus surgery for sensory exotropia. He had undergone two prior vitreoretinal surgeries for traumatic endophthalmitis and retinal detachment, respectively. The second procedure involved injection of silicone oil as an endotamponade. During the strabismus surgery, subconjunctival nodules filled with silicone oil were noted. This made the surgery difficult due to episcleral fibrosis. The multiple silicone oil cysts were removed by rupturing or resecting them along with the tenon's fascia. The extraocular muscles were meticulously isolated and operated using adjustable suture technique. Results: Following surgery, the strabismus was corrected satisfactorily and the patient was relieved of his ocular discomfort and congestion. Conclusions: Subconjunctival leakage of silicone oil can lead to capacious inflammation and complicate strabismus surgery manifold.

Accuracy of noncycloplegic refraction performed at school screening camps.

Purpose: The aim of this study was to compare noncycloplegic refraction performed in school camp with that performed in eye clinic in children aged 6-16 years. Methods: A prospective study of children with unaided vision <0.2 LogMAR who underwent noncycloplegic retinoscopy (NCR) and subjective refraction (SR) in camp and subsequently in eye clinic between February and March 2017 was performed. A masked optometrist performed refractions in both settings. The agreement between refraction values obtained at both settings was compared using the Bland-Altman analysis. Results: A total of 217 eyes were included in this study. Between the school camp and eye clinic, the mean absolute error ± standard deviation in spherical equivalent (SE) of NCR was 0.33 ± 0.4D and that of SR was 0.26 ± 0.5D. The limits of agreement for NCR were +0.91D to - 1.09D and for SR was +1.15D to -1.06D. The mean absolute error in SE was ≤0.5D in 92.62% eyes (95% confidence interval 88%-95%). Conclusion: A certain degree of variability exists between noncycloplegic refraction done in school camps and eye clinic. It was found to be accurate within 0.5D of SE in 92.62% eyes for refractive errors up to 4.5D of myopia, 3D of cylinder, and 1.5D of hyperopia.

The management of congenital superior conjunctival fornix prolapse.

AIM: To present the management of bilateral congenital superior fornicial prolapse and provide its up-to-date review of literature. METHODS: We present the management of congenital bilateral superior fornicial prolapse treated conservatively with topical antibiotics, lubricants and hypertonic saline. One-week, 2-weeks and 1-month follow-up is presented. Results were assessed in terms of reduction of chemosis and eyelid opening. Updated current English peer-reviewed literature was researched. RESULTS: The superior fornicial prolapse resolved with conservative management within 10 days, resulting in normal eyelid motility at the end of 1 month. At present 61 cases have been reported in English peer-reviewed journals. The proposed etiology is varied. Conservative management with topical medication provides relief. CONCLUSIONS: Congenital fornicial prolapse is a rare condition. Topical medication provides complete resolution of prolapse. Authors emphasise the need for awareness of condition among ophthalmologists and neonatologists, because of its gruesome appearance.