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.

Outcomes and surgical management of persistent fetal vasculature

ObjectiveTo analyse outcomes in different forms of persistent fetal vasculature (PFV).Methods and analysisRetrospective cohort study at a university-based practice of children presenting with PFV between 2011 and 2020. Exclusion criteria was surgical management outside of our institution and follow-up less than 1 month. Wilcoxon and Student’s t-tests were used for statistical analysis.ResultsForty-six eyes of 45 patients presented with PFV at 16.7±31.3 (median 2.8) months old with 32.6±29.8 (median 22.5) months of follow-up. Types of PFV included: mild combined anterior-posterior (23 eyes, 50%), severe combined anterior-posterior (18 eyes, 39%), severe anterior (3 eyes, 7%), mild anterior (1 eye, 2%) and posterior (1 eye, 2%). Thirty-two eyes (70%) underwent PFV surgical correction; lensectomy (13 mild combined), vitrectomy (3 mild combined), sequential lensectomy then vitrectomy (3 severe combined), combined lensectomy-vitrectomy (11 severe anterior or severe combined), laser retinopexy (1 mild combined). Five eyes required additional vitrectomy surgery for retinal detachment, fold or cyclitic membrane. Nine eyes developed glaucoma, six requiring Intraocular pressure (IOP)-lowering surgery. At final follow-up, 32 eyes had at least form vision and 6 eyes were aversive to light. Eight eyes, all which were severe combined, and four that did not undergo PFV surgery, were unable to detect light due to phthisis bulbi (7) and optic nerve hypoplasia (1).ConclusionsClassification of PFV is important in determining surgical approach with severe cases often requiring both lensectomy and vitrectomy for optimal anatomic and functional outcomes.