PERIPAPILLARY PACHYCHOROID SYNDROME: New Insights.

PURPOSE: Available literature on peripapillary pachychoroid syndrome (PPS) is very limited. The purpose of this study is to evaluate features of PPS using multimodal imaging and shed further light on this entity. METHODS: This is a retrospective case series at a tertiary eye-care center. Patients with features of pachychoroid with preferential choroidal thickening in nasal macula along with pachyvessels were identified and included for analysis. The patients underwent fundus photography, fundus autofluorescence, optical coherence tomography, fundus fluorescein angiography, and indocyanine angiography. Images were separately analyzed by two different retina specialists. RESULTS: A total of 27 eyes of 14 patients with mean age of 52.2 years were included. Seven patients had bilateral PPS, whereas six had unilateral; one patient had only one seeing eye. A total of 21 eyes with PPS were studied. 52.4% of the eyes revealed retinal pigment epithelium gravitational tracks, outer retinal atrophy, and serous PEDs. None of the eyes showed choroidal folds. Fundus fluorescein angiography showed late leakage at macula in 33% eyes. Fundus autofluorescence showed features of PPS to be similar to central serous chorioretinopathy in most cases. CONCLUSION: Peripapillary pachychoroid syndrome shares common findings with central serous chorioretinopathy and seems to be a subset of central serous chorioretinopathy than a separate entity in pachychoroid disease spectrum.

COMPARATIVE EVALUATION OF OUTCOMES OF DRAINAGE TECHNIQUES IN VITRECTOMY FOR RHEGMATOGENOUS RETINAL DETACHMENT.

PURPOSE: To compare the anatomical and functional outcomes of drainage through posterior retinotomy versus perfluorocarbon liquid (PFCL)-assisted drainage in vitreoretinal surgery for rhegmatogenous retinal detachment and to study intraoperative and postoperative complications. METHODS: This was a prospective randomized study of 52 cases who underwent vitreoretinal surgery for rhegmatogenous retinal detachment. Group 1 underwent PFCL-assisted drainage through preexisting break, whereas Group 2 had posterior retinotomy to drain subretinal fluid. Cases were evaluated for retinal reattachment rates, visual outcomes, optical coherence tomography parameters, and postoperative metamorphopsia. The patients were followed up for minimum period of 3 months. RESULTS: Two groups were comparable in terms of demographic and preoperative parameters. Both groups had single surgery success rate of 100% by the end of follow-up. Final best-corrected visual acuity in Group 1 was 0.61 ± 0.33 and 0.61 ± 0.32 in Group 2 (P = 0.77). Optical coherence tomography parameters (foveal contour, retinal layers, central macular thickness, and epiretinal membrane formation) were similar between the two groups. Subjective metamorphopsia was present in 30.77% (8 of 26) patients in Group-1 and 69.23% (18 of 26) patients in Group-2 (P = 0.034). One eye had retained subretinal PFCL away from the macula in Group 1. CONCLUSION: Anatomical and functional outcomes were similar in vitrectomy using PFCL-assisted drainage versus posterior retinotomy drainage. Postoperative metamorphopsia was lesser in patients who underwent PFCL-assisted drainage through the pre-existing break.

Case Report: Pediatric Ocular Ischemia and Neovascular Glaucoma in Neurofibromatosis Type 1.

SIGNIFICANCE: Neovascular glaucoma is an important subset of secondary glaucoma in neurofibromatosis patients. Vasculopathy of the ophthalmic circulation needs to be borne in mind while evaluating their etiology. PURPOSE: This study aimed to report the presentation, diagnostic work-up and management of an unusual case of neovascular glaucoma in a child. CASE REPORT: A 7-year-old boy presented with uniocular ischemic fundus and secondary neovascular glaucoma. Detailed family history and evaluation led to a diagnosis of familial neurofibromatosis type 1. Fundus fluorescein angiography revealed compromised retinal and choroidal circulations in the affected eye. Ocular ultrasound B scan and neuroimaging did not show any contributory lesions. Cardiovascular evaluation was within normal limits. Ophthalmic Doppler imaging revealed normal proximal ophthalmic arteries in both eyes; however, the central retinal artery of the affected eye showed low flow in its proximal part and absent flow in the distal part, as compared with the fellow eye showing regular flow until the optic disc margin. Corroborating the clinical, fundus fluorescein angiography and Doppler findings, a diagnosis of neurofibromatosis type 1-related vasculopathy of the distal ophthalmic artery was made. Poor visual prognosis for the affected eye was explained, and anterior retinal cryopexy along with cyclocryotherapy was performed to treat the neovascular glaucoma. CONCLUSIONS: Vasculopathy of the ophthalmic circulation is an important cause of neovascular glaucoma in neurofibromatosis patients. The morphology of Lisch nodules may be altered in an ischemic eye, and therefore, careful examination of the other eye and systemic evaluation is vital in such unusual scenarios.

Glycerine assisted vitreoretinal surgery in edematous cornea.

PURPOSE: To describe the use of glycerine in improving media clarity in cases with edematous cornea. METHODS: Retrospective case-series including patients with posterior segment pathologies needing surgical invention and having corneal edema. Each case was studied for the role of topical application of glycerine instead of viscoelastic agents and the course of surgery. The cases were followed up for minimum 3-months duration. RESULTS: Six cases with corneal edema having posterior segment pathology underwent surgery with intraoperative topical application of glycerine. The corneal edema was seen to clear with progression of surgery and was maintained until the end of surgery. Procedures like vitreoretinal surgery, identification of break, internal limiting membrane peeling, intraocular lens explantation, scleral indentation, DSEK graft removal, and subretinal band removal could be performed in these cases. CONCLUSION: Intraoperative glycerine use during vitreoretinal surgeries is helpful in improving the corneal clarity and maintaining it till the end of surgery.

A study of changes in levator muscle in congenital ptosis.

PURPOSE: To study microscopic and ultrastructural changes of levator palpebrae superioris (LPS) muscle in congenital ptosis. METHODS: In this prospective observational study, LPS muscle was studied in 77 eyelids with congenital ptosis; 35-simple congenital ptosis (SCP), 12-Marcus Gunn jaw winking phenomenon (MGJWP), and 30-blepharophimosis epicanthus inversus syndrome (BPES). Light microscopy, enzyme histochemistry, immunohistochemistry and electron microscopy were performed, and results were analyzed. RESULTS: Muscle fibers were detected in 83.33% of MGJWP, 22.86% of SCP and 16.67% of BPES eyelids. Fibers were detected significantly more in individuals with moderate ptosis, LPS action > 4 mm, present eyelid crease and eyelid fold. Severe endomysial and perimysial fibrosis was seen significantly more in individuals with MGJWP. Fat infiltration and nuclei internalization were seen in all three groups. The absence of degenerating or regenerating fibers and inflammatory cells, normal staining pattern on immunohistochemistry and absence of accumulation of any abnormal substance were found in all three groups. Abnormal mitochondrial staining pattern was seen occasionally in three groups. On electron microscopy, muscle was detected in 1 SCP eyelid and 8 MGJWP eyelids out of which 4 had myofibrillary disruption. All other eyelids where muscle fibers were not detected had only fibrocollagenous tissue. CONCLUSION: Fibrocollagenous tissue predominated in all the cases, and muscle fibers detected correlated inversely with the severity of ptosis. The absence of degenerating, regenerating fibers and inflammatory cells supported the theory of dysgenesis of muscle. However, internalization of nucleus seen in all the subtypes is a feature favoring dystrophy.

Ocular decompression retinopathy following bleb needling in a young child.

Ocular decompression retinopathy (ODR) is caused by a sudden lowering of high intraocular pressure. Trabeculectomy is the most common procedure preceding ODR. Various mechanical and vascular etiologies have been proposed to cause ODR, with autoregulation and hemodynamics playing a contributing role. Herein, we report a rare case of ODR occurring after bleb needling in a young child using ultrawide-field fundus photography, fluorescein angiography, and optical coherence tomography.

Intra-ocular hydatid cyst in a child: A rare presentation.

Intra-ocular cystic lesion in a young child can be parasitic lesion, old retinal detachment with cysts or simply a vitreous cyst. Intra-ocular localization of hydatid cysts is extremely rare accounting for less than one percent of all cases. This case describes a young child with left eye pain, redness and progressive diminution of vision with progressively enlarging intra- ocular cyst. Diagnosed as intra-ocular hydatid cyst, the radiological, intraoperative and microbiological features of same are described. Also, the management required in such a case is discussed.

Vitreous hemorrhage - Causes, diagnosis, and management.

Vitreous hemorrhage is associated with a myriad of conditions such as proliferative diabetic retinopathy, proliferative retinopathy following vascular occlusion and vasculitis, trauma, retinal breaks, and posterior vitreous detachment without retinal break. Multiple pathological mechanisms are associated with development of vitreous hemorrhage such as disruption of abnormal vessels, normal vessels, and extension of blood from an adjacent source. The diagnosis of vitreous hemorrhage requires a thorough history taking and clinical examination including investigations such as ultra-sonography, which help decide the appropriate time for intervention. The prognosis of vitreous hemorrhage depends on the underlying cause. Treatment options include observation, laser photo-coagulation, cryotherapy, intravitreal injections of anti-vascular endothelial growth factor, and surgery. Pars plana vitrectomy remains the cornerstone of management. Complications of vitreous hemorrhage include glaucoma (ghost cell glaucoma, hemosiderotic glaucoma), proliferative vitreoretinopathy, and hemosiderosis bulbi.

Cataract in retinopathy of prematurity - A review.

Preterm babies with retinopathy of prematurity (ROP) can become blind if they do not receive appropriate timely intervention. The presence of cataract in these individuals in addition to visual deprivation amblyopia, also delays proper screening, adequate treatment, and makes follow-up assessment difficult. Anatomical differences in these infants and amblyopia management, especially in unilateral cataract, are other important concerns, and hence, management of these cases with cataract and ROP is challenging. In this review, studies where ROP cases were associated with cataract, were evaluated with a focus on preterm individuals less than 6 months age. Preterm babies are at increased risk of developing cataract because of systemic factors. In addition, those with ROP may have cataract associated with retinal detachment or treatment received. The type of cataract, risk factors, and pathophysiology associated with each cause varies. This review highlights these different aspects of cataract in ROP including causes, pathophysiology, types of cataracts, and management. The management of these cases is critical in terms of the timing of cataract surgery and the challenges associated with surgery and posterior segment management for ROP. Anatomical differences, preoperative retina status, pupillary dilatation, neovascularization of iris in aggressive posterior ROP, fundus examination, amblyopia, and follow-up are various important aspects in the management of the same. The preoperative workup, intraoperative challenges, postoperative care, and rehabilitation in these individuals are discussed.

Role of Intralesional Antibiotic for Treatment of Subretinal Abscess - Case Report and Literature Review.

PURPOSE: To describe a rare case of endogenous endophthalmitis due to Citrobacter with subretinal abscess and the role of the novel technique of intralesional antibiotic for its treatment. METHODS: A retrospective case report. RESULT: A 45-year-old male presenting with painful, progressive diminution of vision in the left eye was diagnosed to have endogenous endophthalmitis due to Citrobacter with subretinal abscess. After the failure of the initial intravitreal injection of ceftazidime and vancomycin, successful resolution of abscess was achieved by pars plana vitrectomy with 41-gauge (G) needle assisted intralesional injection of piperacillin and tazobactam combination. CONCLUSION: We treated a case of Citrobacter associated endogenous endophthalmitis with subretinal abscess with intralesional injection of piperacillin and tazobactam combination. 41-G needle can be used safely to inject antibiotic into the subretinal space through a small self-sealing retinotomy with minimum risk of retinal detachment and encouraging results.

Optical coherence tomography angiography in central serous chorioretinopathy: The current clinical role and future perspectives.

Optical coherence tomography angiography (OCTA) images the layers of retinal and choroidal vasculature in the absence of an injectable dye. Since its introduction, OCTA has been utilized in various posterior segment diseases, including central serous chorioretinopathy. We provide a comprehensive review of OCTA's application to central serous chorioretinopathy published between 2014 and 2020.

Intraoperative absent bilateral medial recti in syndromic craniosynostosis.

Patients with syndromic craniosynostosis are usually associated with the complexity of the malformation complex. We describe here detailed oculo-motility disorder and a remarkable finding of hypoplastic bilateral media recti on imaging and its intraoperative absence in patients with phenotypic features resembling Shprintzen-Goldberg syndrome (SGS). SGS is a rare congenital disorder with craniosynostosis affecting multiple systems including mentation and having a considerable overlap of its phenotypic features with Marfan syndrome. Large A-pattern exotropia found in these patients may be related to the craniofacial features and their bearing on extraocular muscle development and function. In this paper, we aimed to sensitise ophthalmologists and strabismologists concerning the necessity to recognise syndromic associations of patients with craniosynostosis presenting with a large squint, be aware of the intraoperative surprises and consider the challenges in its management.

Optical Coherence Tomography Angiography of Early Stage 1a Retinal Hemangioblastoma in Von-Hippel-Lindau.

Von-Hippel-Lindau (VHL) syndrome is characterized by focal vasoproliferative tumors of retinal capillaries called retinal capillary hemangioblastomas (RCH). These tumors are initially small and can be easily missed if not looked for carefully. As they grow, these tumors are more demanding to treat and hence the importance of detecting them early and treating them. Herein, we describe and review the optical coherence tomography angiography (OCTA) of the early- stage lesion, which suggested the involvement of superficial and a deeper retinal capillary plexus. In addition, to helping us detect these lesions earlier, OCTA may also help to understand the in vivo changes occurring at an earlier phase.

Sympathetic ophthalmia following blunt injury to phthisical eye.

Sympathetic ophthalmia is a severe sight-threatening disorder that can lead to blindness if not diagnosed and managed appropriately. It occurs most commonly following penetrating ocular injury and less commonly following surgery. Herein, we report a case of sympathetic ophthalmia following blunt trauma to the phthisical eye along with the pathophysiology and management of such cases.

Macular associations of tilted disc syndrome.

Purpose: The aim of this study was to describe macular changes associated with tilted disc syndrome (TDS) using multimodality imaging. Methods: This is a retrospective observational study of the consecutive TDS cases which were studied for macular changes using color fundus photographs and optical coherence tomography (OCT). Fundus autofluorescence, fundus fluorescein angiography, and OCT angiography were performed wherever required. Results: Twenty consecutive TDS cases (36 eyes) were included. OCT showed inferior depression of all layers in specific scans and macular pathologies seen included lamellar macular hole, full-thickness macular hole, retinal pigment epithelial detachment, acute and resolved subretinal fluid, central serous chorioretinopathy, and choroidal neovascular membrane. Macular involvement was seen in 13 eyes (36.11%) while in the remaining 23 eyes, outer retinal changes were seen on OCT in 9 eyes and normal retinal layers in 14 eyes (38.89%). Conclusion: Various macular pathologies associated with TDS are described using multimodality imaging. These provide understanding of changes which can occur with TDS. It also highlights the need for recognition, differentiation from similar confusing entities, and the necessity to follow-up of these cases carefully to detect the macular changes earlier.

Focal choroidal excavation: review of literature.

Focal choroidal excavation (FCE) is defined as an area of concavity in choroid detected on optical coherence tomography. These are mostly present in macular region without evidence of accompanying scleral ectasia or posterior staphyloma. Though initially considered to be congenital, increasing number of cases have been identified in association with other choroidal pathologies such as central serous choroidopathy, choroidal neovascularisation, polypoidal choroidal vasculopathy, choroiditis, choroidal tumours. In this review article, we aim to elaborate on the morphology, pathogenesis and differential diagnosis of FCE and specifically discuss the spectrum of diseases with known association along with the impact of their treatment on FCE.

An Observational Study of Different Treatment Practices for Aggressive Posterior Retinopathy of Prematurity.

PURPOSE: To evaluate risk factors and outcomes of preferred practice for infants with aggressive posterior retinopathy of prematurity (APROP) at a tertiary eye center in India. METHODS: This was an observational study of infants with APROP where patients were divided into three groups after 2 weeks of initial treatment depending on the treatment received: anti-VEGF only, laser only, and combination of anti-VEGF injection followed by laser within 2 weeks of anti-VEGF injection (combined group). All infants were evaluated for risk factors and followed up at 1, 2, 4, 8, and 12 weeks to determine treatment outcomes in terms of regression, vascular re-growth, progression, and recurrence of the disease. RESULTS: Sixty eyes of 31 infants were included in the study, with 26 eyes in the anti-VEGF only group, 19 eyes in the laser-only group, and 15 eyes in the combined group. Infants in the combined group presented late with lower birth weight (BW), smaller gestational age (GA), and a history of longer duration of ventilatory support. After 3 months of follow-up, regression occurred in 73.08% in the anti-VEGF only group, 89.5% in the laser-only group, and 86.66% in the combined group (P = .07). Zone of vascularization was greater in the anti-VEGF only group and the combined group compared to the laser-only group. Disease recurrence was seen in 27% in the anti-VEGF group, none in the laser-only group, and 13.33% in the combined group (P < .001). However, progression to stage 4 ROP requiring surgery was noted in 2 eyes in the laser-only group. CONCLUSIONS: Infants with APROP who have a lower GA or BW or require longer ventilatory support are possibly a subset best suited for planned combination therapy. A combination of anti-VEGF therapy followed by laser treatment within 2 weeks allows for early regression, a stable course, lower recurrence, and a larger area of retinal vascularization. [J Pediatr Ophthalmol Strabismus. 2021;58(6):370-376.].

Effect of short-term meditation training in central serous chorioretinopathy.

PURPOSE: Stress and Type A personality are established risk factors for the development of central serous chorioretinopathy (CSC). Meditation is known to have a positive effect on reducing stress levels. This study aimed to assess the effect of short-term meditation training in patients of CSC. METHODS: A pilot study was conducted where 40 patients diagnosed with acute and non-resolving CSC were randomly assigned to either of two groups - meditation training and routine care (without meditation). The primary outcome measure was time to resolution of CSC based on optical coherence tomography and fluorescein angiography. Secondary outcome measures were changes in anxiety score (State-Trait Anxiety Inventory [STAI] scores) and blood pressure. The patients were followed up for a minimum period of 4 months. RESULTS: Twenty cases were included in each group. The demographic pattern, baseline swept-source optical coherence tomography parameters, and STAI scores were similar in both groups. The time to disease resolution was 9.4 ± 4.22 weeks in the meditation group and 19.5 ± 2.79 weeks in the nonmeditation group (P < 0.001). At 4 months, CSC had failed to resolve in 60% of patients with routine care compared with 8% in cases following short-term meditation training. STAI scores showed a reduction in stress levels in the meditation group. Furthermore, statistically significant improvement in systolic and diastolic blood pressures was also observed following meditation training. CONCLUSION: Short-term meditation training may be a useful approach in the management of patients with CSC as it tends to reduce stress and prehypertension, and promotes earlier resolution of the condition. However, patient's motivation to complete and pursue the meditation training is a significant barrier.

Ultra-wide field retinal imaging: A wider clinical perspective.

The peripheral retina is affected in a variety of retinal disorders. Traditional fundus cameras capture only a part of the fundus even when montaging techniques are used. Ultra-wide field imaging enables us to delve into the retinal periphery in greater detail. It not only facilitates assessing color images of the fundus, but also fluorescein angiography, indocyanine green angiography, fundus autofluorescence, and red and green free images. In this review, a literature search using the keywords "ultra-widefield imaging", "widefield imaging", and "peripheral retinal imaging" in English and non-English languages was done and the relevant articles were included. Ultra-wide field imaging has made new observations in the normal population as well as in eyes with retinal disorders including vascular diseases, degenerative diseases, uveitis, age-related macular degeneration, retinal and choroidal tumors and hereditary retinal dystrophies. This review aims to describe the utility of ultra-wide field imaging in various retinal disorders.