Leucocoria : Most Common Initial Sign of Retinoblastoma

Retinoblastoma is the most common intraocular cancer of childhood. We report the case of a 3-year-old child with unilateral leucocoria noticed by parents who revealed a retinoblastoma. Leucocoria can also indicate other vision threatening conditions : Coats’ disease, cataract, toxocariasis, retinopathy of prematurity for which prompt medical attention is needed.

Pseudoretinoblastoma: Distribution Based on Gender, Age, and Laterality

Purpose: To investigate the distribution of pseudoretinoblastoma (PSRB) cases based on gender, age, and lateralityMethods: The clinical records of 607 patients (851 eyes) who were referred for suspicion of retinoblastoma between October 1998 and May 2021 were retrospectively evaluated. Patients were stratified by age as follows: ≤1 year, >1-3 years, >3-5 years, and >5 years.Results: PSRB conditions were detected in 190/607 (31.3%) patients. Of 190 patients, 129 (67.9%) were males and 61 (32.1%) were females (p=0.001). The 3 most common diagnoses were persistent fetal vasculature (PFV; 16.3%), Coats disease (15.3%), and optic nerve head drusen (ONHD; 5.3%). In males, the 3 most common diagnoses were Coats disease (20.2%), PFV (14.0%), and chorioretinal coloboma (6.2%). The 3 most common diagnoses in females included PFV (21.3%), retinal dysplasia, congenital glaucoma, and combined hamartoma (each accounting for 6.6%). PFV was the most common diagnosis in ≤1 year old patient group (26.6%). Coats disease and PFV were the most common diagnoses in >1-3 years old patient group (16.7%, for each diagnosis). Coats disease was the most common diagnosis in >3-5 years old (30.8%) and >5 years old patient groups (13.1%). PSRBs were unilateral in 121/190 (63.7%) patients and bilateral in 69/190 (36.3%). The most common unilateral and bilateral diagnoses were Coats disease (24.0%) and PFV (24.6%) respectively.Conclusion: In our study, 31.3% of patients referred for suspicion of retinoblastoma received the diagnosis of PSRB. PSRB spectrum encompasses different diseases, the distribution of which differ depending on gender, age, and laterality.

Coats Disease in 9 Patients: A Hispanic Case Series

Background/Aim To describe demographic and clinical characteristics, treatment, and visual prognosis of Coats disease in Hispanic patients. Methods A retrospective chart review was performed on nine patients (ten eyes) diagnosed with Coats disease in our two clinical centrers from 2004 – 2017. Results Mean age at diagnosis was 5.5 years (range 1 – 12 years) and mean follow-up time was 48 months (range 9 – 108 months). Eight patients (89%) were male and had unilateral disease and one (11%) female patient had bilateral disease. In 40% of the cases, patients were asymptomatic. Visual acuity at first presentation was worse than hand motion in 60% of the eyes. Half of the eyes (5/10 eyes, 50%) had exudative retinal detachment (≥ stage IIIA). Vascular ablation with cryotherapy combined with retinal photocoagulation was the most frequent therapeutic approach (40%). Despite anatomical success at 6 months in 100% of the treated eyes, visual outcome at 1 year of treatment was poor (worse than 20/200) in 70% of the cases. Conclusions In our case series, patients were mostly asymptomatic on presentation, with severe stages of Coats disease. Even with anatomical success after surgical treatment in all treated cases, long-term visual prognosis remained very limited.

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.

Vitrectomy and external drainage of subretinal fluid containing high concentration of vascular endothelial growth factor for advanced coats disease

This study investigated the surgical outcomes of Coats disease and the role of external drainage (XD) of subretinal fluid (SRF). The study is a multicenter retrospective interventional case series of 26 consecutive eyes of 26 patients who underwent surgeries for advanced Coats disease with retinal detachment. Main outcomes measured were: 1) comparison of complete SRF resolution with or without XD, and 2) variables that were associated with functional postoperative best-corrected visual acuity (BCVA) defined as BCVA of 0.1 or better, 3) intraocular vascular endothelial growth factor (VEGF) levels. Complete SRF resolution was achieved in all 14 eyes in which XD had been performed and in 75% of 12 eyes in which XD had not been performed (P = .03). Multivariable logistic regression analysis revealed that initial BCVA was the only variable associated with functional postoperative BCVA (odds ratio 3.24, 95% CI 0.93–11.33; P = .04). Markedly elevated VEGF levels were noted in the SRF compared with those in the vitreous humor (49,760 ± 52,990 vs. 707 ± 611 pg/mL, P = .03). XD seems to provide better anatomical success than without XD in the treatment of advanced Coats disease as XD could effectively eliminate substantial amount of VEGF in the SRF.