The deceptive coloboma

Background: Scleral fistulas are known to develop following blunt trauma in patients with retinochoroidal coloboma. These cases can be managed by surgical options such as silicone buckles, or with glue and scleral patch graft. Some cases have been shown to close spontaneously. We report the first?ever case managed by vitrectomy, endophotocoagulation, and gas tamponade. Purpose: We present a rare and interesting case of an atypical choroidal coloboma with traumatic scleral fistula due to blunt trauma manifesting with hypotony?related disc edema, maculopathy, and chorioretinal folds, which was managed surgically with vitrectomy, endophotocoagulation, and gas tamponade with a good anatomical and visual outcome. Synopsis: The video contains the case description and surgical management of a traumatic scleral fistula in a patient with atypical superotemporal choroidal coloboma. The patient developed hypotonic maculopathy and disc edema after 3 months following a blunt trauma sustained in a road traffic accident. A scleral fistula was suspected at the temporal edge of the coloboma but could not be accurately localized. In addition, due to the edge effect of the coloboma, the external repair was difficult. Hence, vitrectomy with internal tamponade was attempted. Highlights: The video highlights a different surgical approach to managing a traumatic scleral fistula at the edge of a retinochoroidal coloboma. There was a risk of leakage of intravitreal fluid into the orbit through the fistula; however, the gas bubble gave a better tamponade due to higher surface tension. It sealed the fistula presumably by creating a trap?door?like effect. The endophotocoagulation helped create adhesion between the tissues at the edge of the coloboma effectively sealing it. This was followed by a rapid recovery of the hypotony?related problems with good vision. Traumatic scleral fistula, at a difficult place such as the edge of a coloboma, can be successfully closed from an internal approach with vitrectomy, endolaser, and gas tamponade.

Retinal laser – Protocols and practical tips

Background: Many a young doctors in training find retinal laser photocoagulation a daunting task. However, if correct protocols are followed and checklists are observed, then it is not difficult to have a successful laser sitting with a happy patient. Most of the complications can be avoided with correct settings and techniques. Purpose: To enumerate the basic protocols of retinal laser photocoagulation and provide practical tips including laser settings and checklists for hassle?free laser experience. Synopsis: Laser settings for a pan?retinal photocoagulation (PRP) for proliferative diabetic retinopathy differ from those for a focal laser for macular edema. A fill in PRP is indicated when an active Proliferative diabetic retinopathy (PDR) is seen after the initial PRP is completed. The settings and protocols for laser photocoagulation for lattice degeneration are different, and various techniques of barrage laser are discussed. Practical tips and checklists are given, which will not be found in any textbooks. Highlights: Animated illustrations and fundus photos are used to explain the correct techniques of performing laser photocoagulation in different indications and scenarios. Detailed instructions and checklists are provided, which can be very useful to avoid complications and medicolegal problems. The practical tips and guidelines in an easy?to?understand manner make this video highly educational for the novice retinal surgeons who want to perfect their technique of retinal laser photocoagulation.

Surgical management for treatment-resistant cases of inflammatory exudative retinal detachment: Mission impossible?

Purpose: To report the outcome of surgical intervention for inflammatory, exudative retinal detachment (ERD). Methods: A retrospective analysis of eyes with ERD that underwent vitrectomy. Results: Twelve eyes (10 patients) with ERD, non?responsive to medical therapy, underwent vitrectomy. The mean age was 35.7 ± 17.7 years. Five eyes (42%) had Vogt–Koyanagi–Harada disease, three (25%) had presumed tuberculosis (TB), two (17%) pars planitis, and one (8%) had sympathetic ophthalmia. The mean time of vitrectomy was 6.76 ± 4.1 months after onset. Six (50%) eyes had a recurrence, two settled with medical treatment, and four underwent re?surgery. The mean follow?up was 2.7 years. At the last visit, 10 (83.3%) eyes had attached retina; the best?corrected visual acuity (BCVA) had reduced to 1.6 ± 0.7 logarithms of the minimum angle of resolution (logMAR) from 1.3 ± 0.7 at baseline. Conclusion: Vitrectomy in ERD can act as an adjuvant to conventional medical therapy and help maintain structural integrity. Early vitrectomy may help preserve visual function.

Next-generation sequencing-based genetic testing and phenotype correlation in retinitis pigmentosa patients from India

Purpose: Inherited retinal dystrophies (IRD) are a heterogeneous group of retinal diseases leading to progressive loss of photoreceptors through apoptosis. Retinitis pigmentosa (RP) is considered the most common form of IRD. Panel?based testing in RP has proven effective in identifying the causative genetic mutations in 70% and 80% of the patients. This is a retrospective, observational, single?center study of 107 RP patients who had undergone next?generation sequencing?based targeted gene panel testing for IRD genes. These patients were inspected for common phenotypic features to arrive at meaningful genotype–phenotype correlation. Methods: Patients underwent complete ophthalmic examination, and blood was collected from the proband for DNA extraction after documenting the pedigree. Targeted Next Generation Sequencing (NGS) was done by panel?based testing for IRD genes followed by co?segregation analysis wherever applicable. Results: Of the 107 patients, 72 patients had pathogenic mutations. The mean age of onset of symptoms was 14 ± 12 years (range: 5–55). Mean (Best Corrected Visual Acuity) BCVA was 6/48 (0.9 logMAR) (range 0.0–3.0). At presentation, over one?third of eyes had BCVA worse than 6/60 (<1 logMAR). Phenotype analysis with the gene defects showed overlapping features, such as peripheral well?defined chorioretinal atrophic patches in patients with CERKL, PROM1, and RPE65 gene mutations and large macular lesions in patients with RDH12 and CRX gene mutations, respectively. Nummular or clump?like pigmentation was noted in CRB1, TTC8, PDE6A, and PDE6B. Conclusion: NGS?based genetic testing can help clinicians to diagnose RP more accurately, and phenotypic correlations can also help in better patient counselling with respect to prognosis and guidance regarding ongoing newer gene?based therapies.

Retinal perfusion density can predict cardiovascular disease risk in patients with retinal vein occlusion

Purpose: To correlate microvascular changes and assess the relationship between microvascular changes and cardiovascular disease (CVD) risk in patients with retinal vein occlusion (RVO). Methods: Patients over 40 years of age with unilateral RVO were included in this prospective study. Those known to have cardiovascular disease were excluded. A detailed medical history was taken and physical exam was done to measure the height, weight, body mass index (BMI), and systolic blood pressure (SBP). A comprehensive eye check?up was followed by optical coherence tomography angiography (OCTA). Microvascular indices such as vessel density (VD) and perfusion density (PD) were noted. A statistical model was developed for prediction of CVD risk and was integrated with the World Health Organization (WHO)’s risk prediction charts. Results: This study included 42 patients with RVO and 22 controls with an age range of 42–82 years. There were 40 males (62.5%) and 24 females (37.5%). Along with age, SBP, and gender, perfusion density was found to have significant impact on CVD risk (P = 0.030). Reduction in PD was associated with increase in CVD risk. PD had a greater influence on CVD in <50 years age than in >70 years group. Using linear regression, a model with accuracy of 72.1% was developed for CVD risk prediction and was converted into color coded charts similar to WHO risk prediction charts. Conclusion: These findings suggest a significant correlation between microvascular parameters and CVD risk in RVO patients. Based on these parameters, an easy?to?use and color?coded risk prediction chart was developed

Scleral cut-down technique of subretinal fluid drainage in scleral buckle surgery for rhegmatogenous retinal detachment

Background: The art of scleral buckle (SB) surgery is being largely forgotten. However, it still remains a good option for selected cases of rhegmatogenous retinal detachment. Drainage of subretinal fluid (SRF) is one of the most crucial steps of the surgery. When performed correctly, it gives the advantage of immediate retinal reattachment, and it hastens recovery. However, it has its own set of complications. Purpose: To demonstrate, step by step, the scleral cut?down technique of SRF drainage in SB surgery for rhegmatogenous retinal detachment (RRD) and to discuss its indications, contraindications, and complications. Synopsis: Longstanding RRDs with bullous detachments, old age, inferior breaks, or multiple breaks are indications of SRF drainage. In shallow RDs or young patients, a non?drainage procedure may be preferred. It is safer to drain at the bed of the buckle. After the sclera is cut and dissected, it is essential to inspect the choroid for the presence of large vessels. Choroidal vessels are avoided from getting injured while perforating. The release of pigments indicates the end of the drainage. Various possible complications can be prevented by being careful in the surgical technique. Highlights: Diagrammatic illustrations explain the steps of the surgical technique. Intraoperative complications have been explained in an easy?to?understand manner with tips to manage such conditions and their prevention. The video highlights the correct way of performing SRF drainage

Recommendations for management of peripheral retinal degenerations

Some degenerations involving the peripheral retina can result in a rhegmatogenous retinal detachment. Currently, there are no clear guidelines for retinal screening and/or management of these peripheral retinal degenerations in patients with or without recent onset posterior vitreous detachment or in those prior to refractive surgery or intraocular procedures. This article aims to provide a set of recommendations for the screening and management of peripheral retinal degenerations based on a common consensus obtained from an expert panel of retinal specialists.

Comparison of three techniques of harvesting full-thickness retinal tissue for large or persistent macular holes

Purpose: To evaluate the success rate of autologous retinal graft (ARG) for the closure of full?thickness macular holes (MHs) and compare the outcomes of three different techniques of harvesting the graft. Methods: Clinic files of all patients who had undergone ARG for MH using intraocular scissors, membrane loop, or retinal punch to harvest retinal tissue were retrospectively reviewed. All patients were evaluated for MH closure, retinal reattachment, and visual improvement. Results: Twenty?two eyes of 22 patients were included. ARG was done for 16 eyes (72.7%) with failed, large persistent MH, and six eyes (27.3%) also underwent simultaneous repair of retinal detachment. The basal diameter of MH was 1103.67 ± 310.09 (range 650–1529) ?m. Intraocular scissors were used in 10 eyes (45.5%), a membrane loop in five eyes (22.7%), and a retinal punch in seven eyes (31.8%). Silicone oil tamponade was used in seven (31.8%) eyes and gas in 15 (68.1%) eyes. The follow?up ranged from 6 to 18 months. The hole closure rate was 72.7% (16/22). Visual improvement was noted in 18 eyes (81.8%). Retinal reattachment was seen in all eyes. Good graft integration with the surrounding area was seen in 17 eyes (77.3%). Graft retraction was seen in four eyes (18.18%) and graft loss in one eye (4.55%). No significant differences were noted among the three groups. Conclusion: ARG is successful in closing large, failed MH with and without retinal detachment. A membrane loop and retinal punch are equally useful in harvesting the graft, but scissors are preferable in case the retina is detached. With all three techniques, integration of the graft with the surrounding tissue can be achieved

Structural and functional phenotypic features and molecular analysis of Indian patients with Bietti crystalline dystrophy

Purpose: Bietti crystalline dystrophy (BCD) is a rare retinal dystrophy, uncommon in Indians. This study describes the various phenotypic features seen in the Indian population. Methods: In this retrospective, descriptive case series, records of patients with either clinical or molecular diagnosis of BCD from 2009 to 2020 were perused. Phenotypic and genotype information was collected and analyzed. Results: This study included 58 patients of BCD (31 males) aged 21–79 years (mean: 47 ± 14 years). The age at onset ranged from 7 to 41 years (mean: 28.8 ± 5.1 years). Vision ranged from 20/20 to counting fingers. There were 18 (31%) patients with stage 1 with crystals and mild retinochoroidal atrophy, 22 (38%) with stage 2 with atrophy extending beyond arcades, and 18 (31%) with absent crystals and extensive atrophy of stage 3. Choroidal neovascular membrane was seen in four patients. The optical coherence tomography showed retinochoroidal thinning (84.6%), outer retinal tubulations (71.8%), and paradoxical foveal thickening with interlaminar bridges (7.7%). Electrophysiology and visual fields showed reduced responses in advanced retinochoroidal changes. Molecular confirmation was available in five patients; five mutations were seen in the CYP4V2. Conclusion: A wide variation is seen in the phenotypic picture of BCD. A molecular diagnosis is helpful in differentiating from other retinal dystrophies. The OCT shows the peculiar feature of the interlaminar bridge in early cases with photoreceptor loss. Further investigations into this OCT feature may provide insights into the pathogenesis of BCD. A genotype–phenotype correlation could not be done.

Concurrent retinitis pigmentosa and pigmented paravenous retinochoroidal atrophy phenotypes in the same patient.

We report a unique case of a patient with retinitis pigmentosa (RP) phenotype in one eye and pigmented paravenous retinochoroidal atrophy (PPRCA) phenotype in the other eye. We describe in detail the symptoms, clinical findings, and investigations done for a 32‑year‑old Indian woman. This patient had phenotypical picture resembling typical RP in the right eye, with characteristic symptoms of night blindness and constricted field of vision and a nonrecordable electroretinogram (ERG). The left eye of the same patient revealed typical PPRCA phenotype, with no night blindness, normal field, and normal ERG. RP and PPRCA phenotypes are part of the same spectrum of genetic disorder. However, it is rare to see them coexist in the same patient.

Retinal arterial occlusions in the young: Systemic associations in Indian population.

Purpose: To determine the systemic associations in retinal arterial occlusions (RAO) in young Indian individuals less than 40 years of age. Materials and Methods: Case records of 32 patients (35 eyes) of less than 40 years, with non-traumatic RAO were analysed. All patients underwent detailed ophthalmic and systemic evaluation including hemogram, lipid profile, coagulation profile, vasculitis screening, carotid Doppler, echocardiogram. Results: In the study 21 were males and 11 were females. The age ranged from 11-39 years (Mean 27.6 ± 8.43). Nine (28%) patients were below 20 years of age. Among 35 eyes, 28 (80%) had central retinal artery occlusion (CRAO), three (8.6%) had branch retinal artery occlusion (BRAO), two (5.7%) each had cilio-retinal (CLAO) and hemi-retinal artery occlusion (HRAO). Vision ranged from no perception of light to 20/20. On systemic evaluation, in 21 (65.6%) patients a hypercoagulable state was responsible for the RAO. Conditions leading to a hypercoagulable state included hyperhomocysteinemia (21.9%), hyperlipidemia (15.6%), anticardiolipin antibody (6.2%), antiphospholipid antibody (6.2%), polycythemia, thrombocytosis, protein S deficiency, use of oral contraceptives and renal disorder (3.1% each). Six (18.7%) patients had cardiac valvular defects. Vasculitis screening was positive in three (9.4%) patients. Two (6.2%) had isolated systemic hypertension. In two (6.2%) patients no abnormality could be detected. Conclusion: The systemic associations of RAOs in the Indian population were distinctly different from those reported in the Western population. Hyperhomocysteinemia was the commonest association found. Whereas associations reported in the Western population such as cardiac abnormalities, coagulation disorders, hemoglobinopathies and oral contraceptive use were uncommon.

Photodynamic monotherapy or combination treatment with intravitreal triamcinolone acetonide, bevacizumab or ranibizumab for choroidal neovascularization associated with pathological myopia.

This retrospective, interventional case series analyses treatment outcomes in eyes with choroidal neovascularization (CNV) secondary to pathological myopia, managed with photodynamic therapy, (PDT), (Group 1, N = 11), PDT and intravitreal triamcinolone acetonide (4 mg/0.1ml) (Group 2, N = 3), PDT and intravitreal anti-vascular endothelial growth factor (anti-VEGF) bevacizumab 1.25 mg/0.05 ml, ranibizumab 0.5 mg/0.05 ml and reduced-fluence PDT and intravitreal ranibizumab 0.5 mg/0.05 ml (Group 3, N=12). All the patients underwent PDT. Intravitreal injections were repeated as required. SPSS 14 software was used to evaluate the data. Wilcoxon signed ranks test was used to evaluate pre- and post-treatment vision. The Kruskal-Wallis test was used for comparison between the groups. All the groups were statistically comparable. All the eyes showed complete regression of CNV, with a minimum follow-up of six months. All groups had visual improvement; significantly in Group 3 (P = 0.003). Combination PDT with anti-VEGF agents appeared to be efficacious in eyes with myopic CNV. However, a larger study with a longer follow-up is required to validate these results.

Intravitreal cysticercosis presenting as neovascular glaucoma.

We report two cases of intraocular cysticercosis which showed a peculiar presentation of neovascular glaucoma which is hitherto unreported. Two young adults presented with symptoms of raised intraocular pressure due to neovascular glaucoma. On dilated fundus examination both were found to have dead intravitreal cysticercosis. The cysts were removed by a three-port vitrectomy and intracameral injection of bevacizumab was given to help in the regression of rubeosis. Trabeculectomy had to be combined in one case. The intraocular pressure returned to normal. No recurrence of rubeosis was seen even after one year.

Endophthalmitis caused by Acinetobacter calcoaceticus. A profile.

PURPOSE: To report the clinical and microbiological profile of endophthalmitis caused by Acinetobacter calcoaceticus. METHODS: A retrospective study of case series of Acinetobacter calcoaceticus endophthalmitis. Outcome measures included ability to sterilise the eye, anatomical result (clear media and attached retina) and visual recovery (visual acuity > 6/60). RESULTS: Of the 20 cases studied, 10 were cases of postoperative endophthalmitis, 3 were posttraumatic, 6 were endogenous and one was bleb-related endophthalmitis. Specific features of interest observed were relative chronicity of presentation and absence of any obvious predisposing factor in endogenous endophthalmitis cases. All cases could be sterilised except one, which needed evisceration. Cases with postoperative endophthalmitis had better anatomical outcome (7/10 with attached retina and clear media) and visual outcome (4/10 regained vision > 6/18). Higher smear positivity was seen in vitreous samples (72.2%) compared to aqueous samples (37.5%). Culture positivity was higher from the vitreous cavity compared to aqueous. The organism was sensitive to ciprofloxacin in a high percentage (88.9%) of cases. CONCLUSIONS: Visual recovery in Acinetobacter calcoaceticus endophthalmitis is modest. Ciprofloxacin is the antibiotic of choice.