X-linked Juvenile Retinoschisis in a Young Female.

X-linked juvenile retinoschisis has recessive inheritance which occurs due to RS1 gene mutation. We report an instance in a female managed with systemic and topical carbonic-anhydrase inhibitors. 18-year female presented with bilateral blurred vision for two years. Best corrected vision was 6/24 right eye and 6/12 left eye. Fundus examination, ocular coherence tomography and fundus fluorescein angiography supported the diagnosis. Systemic and topical carbonic-anhydrase inhibitors were advised and followed for six months with scrutinization of possible adverse drug reaction. Juvenile retinoschisis being rare among females, prompt diagnosis and management helps for the restoration of the vision and foveal anatomy. Keywords: Carbonic anhydrase inhibitors; female; retinoschisin ; X linked juvenile retinoschisis.

Rhegmatogenous retinal detachment in Coats' disease: a case report.

BACKGROUND: Coats' disease was originally defined as a unilateral idiopathic exudative retinopathy in young males, characterized by abnormal retinal vascular telangiectasia with intraretinal and subretinal lipid exudation. The retinal detachment is usually exudative. Herein, we describe a case of rhegmatogenous retinal detachment with detectable retinal break in a patient with Coats' disease. CASE PRESENTATION: A 15-year-old Indo-Aryan male patient presented with sudden painless diminution of vision in his right eye of 4 days duration. Upon examination, the anterior segment in both eyes and left fundus was within normal limits. Dilated fundus evaluation of the right eye revealed telangiectasia of the retinal vessels, with subretinal exudation in superotemporal and superonasal quadrants and presence of subretinal fluid in the superotemporal area extending into fovea. There was also presence of single flap horseshoe tear in the superotemporal quadrant at around the 10 o'clock position in the equatorial region, with no secondary changes. The retina was reattached with encircling band buckle combined with vitrectomy and silicon oil tamponade. Seven months post vitrectomy, lenticular opacification developed, for which he underwent silicon oil removal, along with lens aspiration and implantation of foldable intraocular lens. Over the period of 1 year, his best corrected visual acuity improved from 6/60 to 6/18 in the affected eye at the last follow-up visit. The recovery was uneventful following the subsequent surgery. CONCLUSION: Coats' disease has a remarkable diversity in clinical presentation and morphology. The disease can also present with an underlying break, which may not be attributed to any iatrogenic modality. The treatment modalities in coats' disease should be tailored individually due to the low incidence of the disease and the great variation in severity upon presentation. Prompt management restores the best possible anatomical outcome and maintains good vision.

Central retinal artery occlusion in optic disk melanocytoma.

Optic disk melanocytoma (ODM) is a rare benign tumor of the optic disk. We report a rare occurrence of profound visual loss due to central retinal artery occlusion associated with ODM in a 78-year-old female with no significant medical history. The clinical findings were supplemented by ancillary investigations.

Comparative Study of Dry Eye Indices Following Cataract Surgery.

BACKGROUND: This study aims to assess dry eye indices following cataract surgery. MATERIALS AND METHODS: A single center descriptive and comparative study was performed. A total of 100 eyes of 100 cases fulfilling the inclusion criteria from 1st June 2017 to 30th May 2018 were enrolled. Out of 100 eyes, 50 eyes each went through manual small incision cataract surgery (MSICS) and phacoemulsification respectively. For objective analysis : schirmer 1 test(ST-I), tear breakup Time(TBUT) along with lissamine Green Surface Staining(LGSS) was performed on pre-operative day, 1st, 4th and 12th week respectively. Ocular Surface Disease Index (OSDI) was done for subjective analysis on pre-operative day and at 12th week. RESULTS: The mean age of the patient was 53.66 ± 7.839 years with 34 (68%) being female in a small incision cataract surgery group. In the phacoemulsification group, mean age was 54.72 ± 7.985 years and 32 (64%) were female. On analyzing the objective dry eye indices: ST-I,TBUT and LGSS at 12thweek was 18.80 ± 7.393 mm, 11.30 ± 5.456 seconds and 1.62 ± 1.193 in Small incision cataract surgery group and 27.10 ± 6.326 mm, 16.60 ± 4.699 seconds and 0.38 ± 0.602 in Phacoemulsification group respectively which was statistically significant. (p< 0.001). CONCLUSION: Regardless of the type of cataract surgery, dry eye disease is unavoidable affecting both tear quality and quantity postoperatively. In our study, phacoemulsification had lesser effect in dry eye indices than small incision cataract surgery.

Evolution of Lightning Maculopathy: Presentation of Two Clinical Cases and Brief Review of the Literature.

Lightning is a naturally occurring atmospheric phenomenon. Though uncommon, it is a potentially devastating and underreported natural calamity. Lightning accounts for the second leading cause of weather-related death in most parts of the world. Among the survivors of lightning injury, more than half of the victims may suffer from some form of ophthalmic injury. The lightning-associated ocular injury varies from a range of anterior segment to posterior segment pathologies. We report on two clinical cases of ocular injuries among the survivors of lightning injury. Anatomical involvement is seen at different levels with presentation as uveitis, pupillary abnormality, maculopathy, and later development of lenticular opacification. Optical coherence tomography (OCT), a noninvasive diagnostic tool, is particularly useful in the evaluation of lighting maculopathy as well as to monitor its progression through the course of time. Visual prognosis depends upon the structures of the eyes affected in the injury. The presence of irreversible retinal damage as well as optic nerve damage often result in poor visual outcome in the absence of significant anterior segment pathology. This report highlights the evolution of maculopathy through the course of time and signifies the importance of long-term follow-up postlightning injury.

Outcomes of the Perplexed Surgical Management of Retinal Detachment in Eyes with Coloboma.

PURPOSE: To determine the anatomical and visual outcomes of retinal detachment in eyes with chorioretinal coloboma managed by pars plana vitrectomy, endolaser photocoagulation and silicone oil (SO) tamponade. METHODS: Retrospective review of 29 eyes of 29 patients with retinal detachment associated with chorioretinal coloboma. All the cases were managed by vitrectomy procedures concluding with SO tamponade. Encircling band was placed based on pre-operative evaluation and/or surgeon's discretion. Endolaser photocoagulation was applied around the peripheral retina, all around the peripheral breaks and around the colobomatous area. The outcome measures were evaluated with regard to functional and anatomical success. RESULTS: The average age at the time of surgery was 21.76 ± 9.58 years (range, 10-50 years). The mean follow-up duration was 12.28 ± 4.8 months (range, 6-24 months). Primary attached retina was obtained in 21 / 29 (72.4%) eyes after single surgery. Re-detachment in 8 / 29 (27.6%) eyes which required revision surgery was the most frequent postoperative complication followed by raised intraocular pressure in 4 / 29 (13.8%) with SO in situ. Out of 29 eyes, 23 were followed up after the removal of SO. The mean duration of SO removal was 7.91 ± 3.9 months (range, 4-18 months). Implantation of encircling band, lens removal and cryotherapy provided no added advantage. At the final examination, improvement in vision was observed in 21 (72.4%) eyes and the anatomical attachment of the retina was attained in 27 (93.1%) eyes. CONCLUSIONS: Complete pars plana vitrectomy, endolaser photocoagulation along with SO tamponade is effective for retinal detachment associated with chorioretinal coloboma. This technique improves the anatomical outcome and helps in regaining significant visual acuity.

Posterior Scleritis Simulating Choroidal Melanoma: A Case Report.

Nodular posterior scleritis (NPS) is rare; however, it is critical to differentiate it from similar intraocular pathologies to prevent inappropriate and potentially irreversible treatment. Multimodal imaging is beneficial in the diagnosis of NPS. This report describes the diagnosis and management of a case of NPS with corticosteroid therapy that led to complete resolution.

Chemotherapy-Induced Central Retinal Artery Occlusion in Gestational Trophoblastic Neoplasia: Case Report.

The use of anticancer chemotherapy (ACC) has resulted in longer patient survival but has also increased drug-related adverse effects. A 22-year-old female receiving cisplatin-based intravenous chemotherapy for high risk variant of gestational trophoblastic neoplasia (GTN) presented with complaints of sudden painless loss of vision in her right eye for a duration of 4 hours. Ocular findings were suggestive of central retinal artery occlusion (CRAO). After exclusion of other potential aetiological risk factors, the patient was diagnosed with CRAO associated with cisplatin. Cancer patients are prone to thromboembolic events (TEE) not only due to primary disease but also due to underlying comorbidities and treatment modalities. The high incidence of TEE in patients under cisplatin therapy mandates a high degree of suspicion among the treating physicians. This rare possibility of irreversible visual toxicity should also be considered among the patients under cisplatin chemotherapy.

Ocular Manifestations and Biometrics in Marfan's Syndrome from Eastern Nepal.

PURPOSE: To evaluate the ocular characteristics of Marfan's syndrome (MFS) fulfilling the revised Ghent-2 nosology in Eastern Nepal. MATERIALS AND METHODS: A hospital-based observational and cross-sectional study was conducted. Ocular manifestations and biometrics were incorporated. Patients were subdivided into adults (16 years or older) and children (5-15 years). Ocular biometric parameters consisted of values of refractive error, keratometry readings, anterior chamber depth (ACD), central corneal thickness (CCT), lens thickness (LT) and axial length (AL). RESULTS: A total of 34 eyes of 17 patients with MFS were included, where 32 eyes were phakic. Mean age of the study participants was 14.5 ± 9.1 years. The mean best corrected visual acuity (BCVA) of phakic eyes was 0.99 ± 0.82 LogMAR. Myopia greater than -3 diopters (D) was present in 28/34 (82.35%) eyes. The average spherical equivalent was -12.34 ± 8.85 D. Ectopia lentis (EL) was present in 24/32 (75%) eyes where superonasal was the most common subluxation in 10/24 (41.7%) eyes. AL was longer in adults 26.54 ± 4.42 mm compared to 25.21 ± 1.93 mm in children. Likewise, LT in adults was 4.9 ± 0.70 mm and 4.40 ± 0.59 mm in pediatric participants. Flat corneas were noted in both the groups with an average of 41.53 ± 2.21 D. The mean CCT and ACD were 524.62 ± 21.74 µm and 3.64 ± 0.80 mm, respectively. There was a negative association between the AL and the average corneal curvature (Kmed, correlation coefficient -0.11, p=0.54). CONCLUSION: Myopia is the foremost ocular involvement with significant visual disability in MFS. Though, AL and corneal curvature are not included in the revised Ghent-2 nosology, we strongly recommend these parameters to be considered during ophthalmic evaluation in suspected and diagnosed cases of MFS in the absence of genetic testing.

Angle Closure Glaucoma in Retinitis Pigmentosa.

BACKGROUND: Angle closure glaucoma (ACG) whether primary or secondary lens induced has rare occurrence in cases with retinitis pigmentosa (RP). METHOD: Five patients with history of diminished vision, ocular pain, and nyctalopia were clinically evaluated. Four patients had unilateral presentations of circumciliary congestion, corneal edema, and high intraocular pressure (IOP), while one had bilateral presentation, respectively. Anterior chambers were shallow; fundoscopy revealed the features of RP and gonioscopy affirmed closed angles in all the cases. The management strategies were individualized based on the specific ocular condition. RESULT: The raised IOP were not well controlled with conventional medical treatment. Neodymium yttrium aluminium garnet laser peripheral iridotomy (LPI) was performed in two patients and in the fellow eye in other two patients as a prophylactic measure. Phacoemulsification surgery with implantation of intraocular lens (IOL) was performed in three patients, whereas phacoemulsification only without IOL and trabeculectomy performed in one patient. Among them, two patients had subluxated lens, where one was managed with capsular tension ring and the other was left aphakic, respectively. However, the vision was not improved significantly in these patients. CONCLUSION: RP may be associated with ACG in rare instances. In these patients, angle closure-related high IOP can have a detrimental effect on the pre-existing visual impairment. However, this can be prevented by thorough clinical examination and timely intervention in those susceptible eyes.

Outcomes of Conventional External Dacryocystorhinostomy with and without Mitomycin C: Experience from Eastern Nepal.

INTRODUCTION: Dacryocystorhinostomy (DCR) is the commonest surgery for nasolacrimal duct obstruction. Inhibition of the scarring process within the anastomosis and rhinostomy site which has been attributed to the failure of this procedure, might improve the success rate of DCR. The objective of this study was to evaluate the outcomes of DCR with Mitomycin-C (MMC) and to compare the results of DCR with and without MMC. MATERIALS AND METHODS: A hospital based, prospective study was conducted in patients with primary acquired nasolacrimal duct obstruction. Standard conventional DCR was performed upto the level of creation of flaps. Application of MMC 0.2 mg/ml in and around the ostium and underneath the created flaps for two minutes was effected with cotton pledgets. The area was thoroughly washed with normal saline after removal of the pledgets. Rest of the surgery was completed as usual. The patients were followed up on the first postoperative day, one month and three months post surgery. RESULTS: A total of 60 cases, 30 in each group were allocated. The success rate of DCR with MMC was found to be 96.7% compared to 86.7% (p=0.35) in the DCR group at the end of three months duration. CONCLUSIONS: Intraoperative application of MMC during conventional DCR surgery provides a comparatively higher success rate than DCR without MMC without posing any extra financial burden and adverse drug reaction to the patient.

Confrontation with a live subretinal parasite.

BACKGROUND: Parasitic infestation of the eyes are a major cause of ocular diseases across the globe. Filarial and filarial-like nematodes top the list of the nematodes that affect the eye. CASE: A rare case of live sub retinal worm is reported in a 25 years old apparently healthy young male. The case presented with unilateral loss of vision and floaters in the affected eye. Upon examination a live subfoveal worm was identified with continuous wriggling movements and diffuse retinal edema. The worm was removed surgically and sent for parasitological examination. OBSERVATION: The worm was identified microscopically as Loa Loa. However detailed histopathological examination could not be incorporated. The patient's vision improved to 6/12 (0.30 Log MAR) from the initial presentation of 3/60 (1.30 Log MAR) after three months follow-up. CONCLUSION: The rare sub retinal live worm presents a challenge in management. The management depends upon the location and viability of the parasite. Surgical management is aimed at worm removal and vision preservation.

Orbital Teratoma: A rare congenital tumour.

BACKGROUND: Orbital teratomas are congenital, unilateral germ cell tumors, which are present at birth with moderate to massive proptosis. The rare tumour has to be managed individually and is at times difficult. CASE: A female child in her 2nd day of life was brought to our department with complains of forward bulging of the left eye which was noticed since the time of her birth. OBSERVATION: Examination and investigations revealed a mass with scattered foci of calcification. The mass was causing expansion of left orbit with thinning and scalloping of bony boundaries of left orbit with no intracranial component. The scan suggests the diagnosis of orbital teratoma which was further confirmed by histopathological examination. CONCLUSION: The rare tumour presents a challenge in management. The aim of management depends upon the extent of tumour, preservation of vision whenever possible, promotion of normal orbital growth and cosmesis.