Surgical outcomes of cataract surgery in anterior and combined persistent fetal vasculature using a novel surgical technique: a single center, prospective study.

PURPOSE: To study the complications and surgical outcomes of cataract surgery in patients of persistent fetal vasculature (PFV) with cataract. METHODS: In this prospective study, phacoaspiration with/without intraocular lens implantation (IOL) was done in 20 children (mean age 14.2 months) with unilateral cataract with anterior (n = 6) or combined (n = 14) PFV. The rentrolental vascularized membrane was cauterized and dissected circumferentially, followed by cauterization and resection of the PFV stalk. The outcome measures included fixation preference using the CSM (central, steady, maintained) method and intraoperative and postoperative complications in an 18-month follow-up. The difference in outcomes of anterior and combined PFV, as well as aphakic and pseudophakic eyes, was studied. RESULTS: CSM fixation was seen in 16 patients after 18 months. The intraocular lens was implanted in 16 eyes and 4 eyes with combined PFV were left aphakic. None of our patients had intraoperative bleeding. Visual axis obscuration was the major complication seen, requiring membranectomy in 8 children. Pupilloplasty was required with membranectomy in one eye. None of our patients developed glaucoma or retinal detachment. CONCLUSION: Timely surgical intervention and aggressive amblyopia therapy led to good visual results in our study. Poor prognosis was seen in combined PFV, aphakia, and microphthalmia.

Ophthalmic features and management outcomes of 30 children having Goldenhar syndrome.

PURPOSE: To study the ophthalmic clinical profile and the management outcomes of children having Goldenhar syndrome (GS). METHODS: Retrospective review of included children fulfilling the diagnostic criteria of GS was performed. The demography, ophthalmic features, systemic anomalies, and treatment outcomes were recorded manually from patient files. An ENT, pediatrics, cardiology, and orthopedics consultation was sought for all GS patients before taking up for any ophthalmic surgical procedure. The anatomical (ocular surface and eyelid) and functional {vision and extraocular movements (EOM)} factors were exclusively studied. We ensured a minimum postoperative follow-up of 12 months, and our data were compared with the major studies featuring 'ophthalmic features' of GS. RESULTS: Totally 30 children (females = 18, 60%) were included with a median presenting age of 48 months. Twenty-seven (90%) had unilateral ophthalmic involvement with major features being upper eyelid coloboma (n = 25, 75.76%), lipodermoid (n = 18, 54.55%), and limbal dermoid (n = 10, 30.3%). Diminution of visual acuity was recorded in 22 (73.3%), while five (16.7%) had limitation of EOM. Systemically, the prominent features included hemifacial hypotrophy (100%), auricular anomalies (80%), cardiac anomalies (10%), and scoliosis (6.67%). The eyelid colobomas were repaired with the direct closure ± cantholysis technique or using a Tenzel's flap. All children had satisfactory anatomical and functional outcomes after ophthalmic surgical interventions without any significant complications. CONCLUSION: The tailored ophthalmic surgical intervention(s) provides satisfactory restoration of anatomy and functionality of the eye. These children need specific multi-discipline consultations for the holistic management and complete care.

Efficacy and safety of topical difluprednate in persistent diabetic macular edema.

To evaluate the efficacy and safety of treatment of diabetic macular edema (persistent type) with difluprednate ophthalmic emulsion 0.05 % (off label use). 20 patients with persistent diabetic macular edema were enrolled. In all subjects, more than 4 months had passed since prior treatment. All patients were treated with difluprednate ophthalmic emulsion 0.05 % three times daily for 3 months. At the end of 3 months the visual acuity had increased by two lines to a mean value of 0.61 ± 0.18 on logMAR from a baseline value of 0.885 ± 0.20 and the central retinal thickness had decreased from 423 ± 72.04 microns to 345 ± 68.7 microns. Hence, there was a total of 18.4 % decrease in retinal thickness on difluprednate. Major side effects included raised intraocular pressure in 20 %. Difluprednate is a potent and strong steroid which causes a rapid decrease in persistent diabetic macular edema. However, the potential side effect of raised intraocular pressure limits its use as an adjuvant therapy in non-steroid responders.

Biallelic novel variants in ZNF469 causing Brittle Cornea Syndrome 1: a detailed report of an Indian patient.

BACKGROUND: Variations in ZNF469 have been associated with Brittle Cornea Syndrome that presents with bluish sclera, loss of vision after trivial trauma, arachnodactyly, and joint laxity. MATERIALS AND METHODS: Detailed medical and family history, physical examination, and molecular analysis. RESULTS: A 21-year-old female presented with bluish discoloration of sclera, diminution of vision following trivial trauma in childhood along with hearing loss and systemic features of arachnodactyly and joint laxity. Clinical diagnosis of brittle cornea syndrome was made which was molecularly proven using next-generation sequencing which identified compound heterozygosity in ZNF469 for pathogenic and likely pathogenic nonsense variants. One variant namely NM_001367624.2:c.5882dup was identified in the exon 3 which was novel and classified as likely pathogenic according to American College of Medical Genetics (ACMG) criteria for variant classification. Another variant NM_001367624.2:c.8992C>T in the exon 2 was classified as pathogenic for Brittle Cornea Syndrome 1. CONCLUSIONS: The report adds to the allelic heterogeneity in ZNF469 causative of Brittle Cornea Syndrome 1 and shall acquaint the physicians about this potentially vision threatening, underdiagnosed, rare syndrome.

Multimodal Imaging in Mucolipidosis Type IV: Siblings With Novel Genetic Variant.

PURPOSE: Utilization of multimodal imaging techniques to diagnose cases of mucolipidosis type IV (ML-IV) and report a new genetic variant. METHODS: This study is a case report. RESULTS: Case 1 involves a 4-year-old boy with corneal haziness and global developmental delay who showed an increased reflectivity of the corneal epithelium on anterior segment optical coherence tomography (AS-OCT). In addition, neurologic evaluation was suggestive of ML-IV. Further genetics evaluation confirmed ML-IV. Histology of the button revealed a thickened epithelial basement membrane. Case 2, the younger sibling, showed a milder corneal haze with similar changes on AS-OCT prompting us to further evaluate for ML-IV by genetics (positive MCOLN1 gene mutation). Both instances highlighted varied ML-IV presentations, but a persistent feature was hyperreflective epithelium. CONCLUSIONS: Our study emphasizes AS-OCT's role in screening ML-IV and advocates the role of genetic counseling of affected parents. We present 2 South-Asian siblings with ML-IV with a new genetic variant, emphasizing the utility of detailed ophthalmic and neurologic assessments using multimodal imaging.

Correlation of anterior segment optical coherence tomography and ultrasound biomicroscopy in congenital corneal opacity.

PURPOSE: To investigate the correlation between swept-source anterior segment optical coherence tomography (AS-OCT) and ultrasound biomicroscopy (UBM) in congenital corneal opacity (CCO). METHODS: All children with unilateral or bilateral congenital corneal opacities who underwent examination under anesthesia (EUA) and anterior segment optical coherence tomography (AS-OCT) imaging from January 1, 2022, to December 31, 2022, were included. Main outcome measures were corneal and anterior segment evaluation and correlation of UBM and AS-OCT findings. RESULTS: A total of 22 eyes of 15 patients were imaged using both technologies. The age at first EUA ranged from 11 days to 4 years. Different phenotypes were classified based on the clinical examination, UBM, and AS-OCT findings. Fourteen eyes were diagnosed with Peters anomaly, congenital corneal staphyloma was observed in 4 eyes, 2 eyes had coloboma, 1 eye had peripheral sclerocornea, and 1 eye was diagnosed with congenital primary aphakia. AS-OCT and UBM findings were closely correlated in 18 of 22 eyes (82%) but AS-OCT failed to provide detailed information in 4 eyes (18%) where UBM revealed more details. CONCLUSIONS: Although AS-OCT offers valuable preliminary data for initial assessment and counseling, it may not consistently provide precise assessments in all cases. Therefore, UBM should be considered for definitive evaluation.

Ocular Cicatricial Pemphigoid in Young Patients.

PURPOSE: The purpose of this study is to report ocular cicatricial pemphigoid (OCP) occurring in young patients. Relevant literature is also reviewed. METHODS: Medical records of patients aged 30 years or younger who were treated for OCP between August 2021 and May 2023 at a tertiary care eye institute were reviewed. The most common differential diagnoses of cicatrizing conjunctivitis, such as Stevens-Johnson syndrome sequelae, chemical injury, chronic topical/systemic drug use, autoimmune connective tissue disorders, or allergic eye diseases, were ruled out based on clinical history, examination, and the Cicatrizing Conjunctivitis Score described by Shanbhag et al. The diagnosis of OCP was confirmed by positive direct immunofluorescence of oral mucosal and/or conjunctival biopsy in a majority of the patients. RESULTS: Seven patients fulfilled the criteria for a diagnosis of OCP. The mean age at presentation was 21.86 ± 5.25 years (13-28 years). Some of the patients presented with relatively atypical features for OCP such as corneal immune ring infiltrate and bilateral corneal perforation. Six patients exhibited systemic mucosal lesions, and the direct immunofluorescence yield was 85.71%. All patients required aggressive immunosuppressive treatment. CONCLUSIONS: OCP is classically described as a rare disease that occurs in old age. This case series highlights the importance of a higher index of suspicion for diagnosing OCP at a younger age. Early administration of immunosuppressive agents can potentially control severe ocular surface inflammation and its sequelae.

Bilateral Diffuse Uveal Melanocytic Proliferation in a Patient with Recurrent Frontal Lobe Meningioma.

BACKGROUND: Bilateral diffuse uveal melanocytic proliferation (B-DUMP) is a paraneoplastic disorder that may be seen in patients with a known malignancy but more commonly is seen in those without any history of cancer. It leads to multiple uveal tumors with exudative retinal detachment along with thickening of the choroid and generally carries a poor prognosis. Its etio-pathogenesis is poorly understood but is said to involve factors secreted by the systemic malignancy that lead to proliferation of melanocytes in the uvea. METHODS: We report the presentation and management of a female with history of treated frontal lobe meningioma who presented with neovascular glaucoma along with B-DUMP and was found to have recurrence of the meningioma on neuro-imaging. CONCLUSIONS: Central nervous system meningiomas can cause B-DUMP and management of the malignancy may lead to partial resolution of posterior segment manifestations of B-DUMP.

Ophthalmic Complications in Pediatric Uveitis.

Purpose: We aim to look at the complications encountered by a cohort of pediatric uveitis patients from north India.Methods: Retrospectively, complications seen in patients younger than 16 years diagnosed with uveitis between January 2006 to March 2015 were noted.Results: Data of 104 children, with a mean follow-up of 3.40 ± 2.34 years was studied. Cataract (n = 42, 24.00%), band-shaped keratopathy (n = 32, 18.29%) and Glaucoma/OHT (n = 11, 6.29%) were most encountered complications at presentation. Glaucoma/OHT (29.71%; n = 52), cataract (18.86%; n = 33) and maculopathy (n=12;6.86%) were the most common complications at follow up. Maculopathy (35%) and Glaucoma/OHT (20%) were the most common causes of visual acuity ≤3/60.Conclusions: Cataract is the most critical complication in children with uveitis at presentation and raised intraocular pressure occurs at follow-up, perhaps attributed to the treatment. Maculopathy is the most common cause of blindness in these children.

Interstitial Keratitis with Corneal Perforation as the Presenting Sign of Systemic Tuberculosis.

Background: Ocular surface and corneal involvement in tuberculosis is seldom seen. We report a patient of pulmonary and presumed ocular tuberculosis with immune keratitis along with corneal perforation as the presenting signs.Methods: A middle-aged male presented with stromal keratitis, deep corneal vascularization, and two corneal perforations. Necrotic Mantoux test and cavitary lesion on computerized tomography of the chest clinched the diagnosis of tuberculosis-related interstitial keratitis.Results: Tubercular interstitial keratitis was successfully managed with antitubercular therapy, oral and topical steroids while cyanoacrylate glue was used to seal the corneal perforations.Conclusions: Although corneal involvement is uncommon in ocular tuberculosis, in patients with interstitial keratitis who respond poorly to conventional therapy, a possibility of systemic tuberculosis should be considered in endemic countries.

Comparison of an aspheric monofocal intraocular lens with the new generation monofocal lens using defocus curve.

PURPOSE: The aim of this study was to compare the visual outcomes of two monofocal intraocular lenses (IOLs), with emphasis on the defocus curve. METHODS: A total of 116 consecutive eyes with cataract, undergoing phacoemulsification with IOL implantation were included in the observational case series, and divided into two groups. 71 eyes were implanted with Tecnis Eyhance and 45 with Tecnis 1 monofocal IOL. Eyes with ocular comorbidities, previous ocular surgeries and corneal astigmatism >1 Diopters (D) were excluded from the study. Complete ophthalmic evaluation including uncorrected distance visual acuity (UDVA), corrected distance visual acuity (CDVA), uncorrected intermediate visual acuity (UIVA), corrected intermediate visual acuity (CIVA), uncorrected visual acuity (UNVA), corrected near visual acuity (CNVA) was noted and defocus levels ranging from -4.00 D to + 1.00 D were plotted postoperatively in both groups. RESULTS: Uncorrected intermediate visual acuity (UIVA) and uncorrected near visual acuity (UNVA) was significantly better in Tecnis Eyhance group compared to Tecnis 1 monofocal. Both the IOLs have similar performance for distance vision but visual acuity at intermediate and near is significantly better with Tecnis Eyhance compared to Tecnis 1 piece IOL. CONCLUSION: Tecnis Eyhance IOL with its better defocus curve, not only provides good distance, but intermediate vision as well. With significantly better visual acuity across the range of near and intermediate vision, Tecnis Eyhance IOL can prove to be a viable and reasonable option for patients who are more dependent on intermediate vision in daily activities.

Serpiginous Endothelial Exudates in a 15-Year-Old Boy.

This case report discusses the accumulation of endothelial exudates in a boy aged 15 years after lensectomy for endophthalmitis.

Pigmented Corneal Ulcer.

PURPOSE: To report the clinical characteristics, laboratory findings, and treatment of a rare case of keratitis caused by pigmented fungi Bipolaris hawaiiensis. CASE REPORT: A 55-year-old man presented with a history of trauma with vegetative matter in his left eye. Slit lamp biomicroscopic examination revealed the presence of a brownish-black pigmented plaque with surrounding infiltrates. Corneal scrapings revealed multiple septate hyphae. Culture revealed growth of the Bipolaris species. The patient was treated with topical natamycin 5%, topical voriconazole 1%, and oral itraconazole followed by intracameral amphotericin B (5 µg/mL). The patient responded well to the treatment. CONCLUSION: Brown pigmented infiltrates are an important clinical feature of dematiaceous fungi. B. hawaiiensis is a rare cause of corneal phaeohyphomycosis. Our patient responded well to intracameral amphotericin B, which obviated the need for penetrating keratoplasty.

Visual outcomes of pediatric traumatic cataracts.

INTRODUCTION:: To report visual outcomes and risk factors of pediatric traumatic cataracts in a tertiary care referral center in northern India. METHODS:: We analyzed medical records of traumatic cataracts in the pediatric age group (1-15 years) operated for cataract surgery with or without posterior chamber intraocular lens implantation with or without primary posterior capsulotomy with anterior vitrectomy between 2004 and 2012. Causative agents, types of trauma, demographic factors, surgical interventions, complications, and visual acuity were recorded and compared among different groups. RESULTS:: A total of 147 children were documented to have undergone cataract surgery for traumatic cataract in the study period, male-to-female ratio being approximately 5:1. Mean age was 7.67 ± 3.30 years (range, 1-15 years). Type of primary insult was penetrating injury in 100 (68%) patients and blunt trauma in 47 (32%) patients. Mean interval between injury and cataract surgery in penetrating injury cases was 3.84 ± 7.05 months and in the blunt injury cases was 6.28 ± 11.13 months. Preoperatively, only 110 patients were cooperative for visual acuity. Out of them, none had vision better than 6/18 and only 21 patients (19.9%) had vision of ≥6/60. Visual acuity of 6/18 or better (was considered good visual outcome) was achieved by 87.9%, 97.3%, and 97.9% at 1, 6, and 36 months, postoperatively. Eyes which underwent primary posterior capsulotomy and anterior vitrectomy during cataract surgery showed statistically better visual outcome than those without it. CONCLUSION:: Phacoaspiration with posterior chamber intraocular lens implantation along with primary posterior capsulotomy and anterior vitrectomy and timely introduction of amblyopia therapy helped in gaining good visual outcome in pediatric traumatic cataract patients irrespective of the age of presentation and the type of injury.

Intraocular lens implantation in children with unilateral congenital cataract in the first 4 years of life.

PURPOSE: To describe the outcome of phacoaspiration with intraocular lens implantation in children with unilateral congenital cataract in the first 4 years of life. METHODS: A retrospective chart review of children with visually significant unilateral congenital cataract presenting in the first 4 years of life was done. Children with a minimum postsurgical follow-up of 1 year were included. Outcome measures were mean spherical equivalent, visual axis clarity, visual acuity and complications till the last follow-up. RESULTS: Ninety-three children met the inclusion criteria. The mean age of surgery was 13.23 ± 11.89 months and the mean follow-up period was 24.37 ± 17.35 months. Nearly 40% of children presented during their first year of life. No difference was noted between the subgroups in terms of age ( p = 0.310), sex ( p = 0.475) or laterality ( p = 0.349). Surgical membranectomy was performed in 22 eyes (23.6%) after an average period of 4.85 ± 2.58 months after surgery. One eye underwent piggy back intraocular lens and four eyes underwent intraocular lens exchange after a mean duration of 50 months (range 40-60 months). The mean visual acuity was 0.79 ± 0.11 (log MAR chart). A total of 60.7% of these children ( n = 31) achieved best corrected visual acuity or 20/80 or better. CONCLUSION: The results of our study suggest that primary intraocular lens implantation in children with unilateral congenital cataract gives good structural and functional results. Besides a meticulous surgery, visual outcome is affected by the time of presentation and postoperative compliance to amblyopia therapy.

Nasal endoscopic features and outcomes of nasal endoscopy guided bicanalicular intubation for complex persistent congenital nasolacrimal duct obstructions.

Purpose: To study the clinical presentation, nasal endoscopic features, and outcomes of nasal endoscopy guided (NEG) bicanalicular intubation (BCI) in children with complex persistent congenital nasolacrimal duct obstruction (pCNLDO). Methods: A prospective, interventional study including eligible children (age ≤ 12 years) having complex pCNLDO. The demographics, number of previous probings, nasal endoscopy findings, and outcomes; were noted in all children who underwent NEG-BCI with Crawford's stents. Matting of eyelashes (MoE, upper, and lower eyelid), tear-film height (TFH), and fluorescein dye disappearance test (FDDT) was assessed pre and postoperatively. The minimum stent in-situ period was 12 weeks, and the minimum follow-up was 6 months (after stent removal). Results: Total 32 children (36 eyes) including 18 females (56.25%) were studied. At a mean age of 4.9 years, all children had epiphora and discharge with MoE (both upper and lower), raised TFH and positive FDDT. Previously, all children underwent conventional probing (s)- once in 12 (33.3%), twice in 18 (50%) and thrice in 6 (16.7%) eyes. The general ophthalmologists performed the majority (n = 21, 58.33%) of those. The BCI was performed under GA in all eyes, and at a mean follow-up of 8.5 months, the "complete" success was noted in 29 eyes (80.5%), 'partial' success in 4 (11.1%) and failure in 3 (8.3%). The stent prolapse was seen in three. Conclusion: NEG-BCI may provide a satisfactory resolution to complex pCNLDO after single or multiple failed probings. NEG provides confident and efficient management of coexistent intranasal complexities related to the inferior turbinate and meatus.

Diagnosis and treatment of bilateral Coats disease in a 5-year-old girl.

A 5-year-old girl presented with decreased vision and outward deviation of her right eye. Fundus examination revealed multiple hard exudates in the macula in the right eye and nasal to the disk in the left eye. The patient was lost to follow-up in the near term but presented 9 months later with reduced vision and an increase in exudates in both eyes. RetCam fluorescein angiography confirmed the diagnosis of bilateral Coats disease.

Glaucoma Secondary to Uveitis in Children in a Tertiary Care Referral Center.

Purpose: To study outcome of secondary glaucoma in pediatric uveitis patients. Methods: Retrospective analysis of records of uveitis patients ≤16 years. Results: Of 182 pediatric uveitis patients, secondary glaucoma was seen in 48 patients (75 eyes, 26.23%) with female preponderance (F:M-29:19) . JIA was the most common etiology (35.71%). BCVA of ≥20/40 was seen in 22 eyes at presentation and in 38 eyes at final follow up (p<0.001). Twenty eight children (66.67%) received systemic antiglaucoma therapy while 17 children (21 eyes) required surgery (60.71%). Logistic regression showed pseudophakia could predict the higher use of oral antiglaucoma medication (p=0.03) while anatomical site of involvement was predictive of higher chances of surgery (p=0.003). Overall success was seen in 82.10% and 71.64% using IOP limit of 21 and 18 mmHg respectively at mean follow-up of 3.9 years. Conclusion: Pediatric uveitic glaucoma though require multitude of therapies, can be managed effectively with appropriate therapy.

Long-term outcomes of cataract surgery in children with uveitis.

PURPOSE: To evaluate the long-term outcomes of cataract surgery in children with uveitis. METHODS: Retrospective, noncomparative review of medical records of children (≤16 years) with uveitic cataract who had undergone cataract surgery between January 2001 and December 2014 at a tertiary care center was done. The main outcome measures were visual acuity and postoperative complications. RESULTS: We recruited 37 children (58 eyes) who were diagnosed with uveitic cataract and underwent cataract surgery. The etiology of uveitis included juvenile idiopathic arthritis (n = 19), presumed intraocular tuberculosis (n = 8), idiopathic (n = 4), Behçet's disease (n = 2), Vogt-Koyanagi-Harada syndrome (n = 2), human leukocyte antigen B-27 associated uveitis (n = 1), and toxocariasis (n = 1). Phacoemulsification with intraocular lens (IOL) implantation was performed in 17 patients (27 eyes; 46.55%), while 20 patients (31 eyes; 53.44%) were left aphakic after pars plan lensectomy and vitrectomy. At an average follow-up of 3.69 ± 7.2 (SD) years, all cases had significant improvement in corrected distance visual acuity post cataract extraction; visual acuity of 20/40 or more was achieved in 32 eyes (55.17%). The most common complication was capsular opacification (37.93%). Incidence of secondary procedures as well as glaucoma was not statistically different in patients undergoing IOL implantation from those who were aphakic. CONCLUSION: Even though number of secondary procedures was more in pseudophakic group, meticulous choice of surgical technique and adequate immunosuppression lead to a modest gain of visual acuity in children undergoing IOL implantation in uveitis. However, scrupulous case selection and aggressive control of pre- and postoperative intraocular inflammation are the key factors in the postoperative success of these patients.