https://doi.org/10.1177/1120672119841190 European Journal of Ophthalmology 1–8 © The Author(s) 2019 Article reuse guidelines: sagepub.com/journals-permissions DOI: 10.1177/1120672119841190 journals.sagepub.com/home/ejo EJO European Journal of Ophthalmology Introduction Primary congenital glaucoma (PCG) is the most common form of glaucoma in childhood, and a significant cause of vision loss in children. Its diagnosis is defined by two of the following criteria: intraocular pressure (IOP) higher than 21 mmHg, suspicious optic disk cupping, increase in the corneal diameter or Haab Striae, increase in axial length or myopia, and altered visual field. It is classified by the age at diagnosis in neonatal (less than 1 month), infant (between 1 and 24 months), or late (more than 24 months). 1 Cataract extraction in patients with primary congenital glaucoma Laura Morales-Fernández 1 , José María Martínez-de-la-Casa 1 , Blanca Benito-Pascual 1 , Federico Saénz-Francés 1 , Enrique Santos-Bueso 1 , Pedro Arriola-Villalobos 1 , Julio Escribano-Martínez 2 and Julián García-Feijoo 1 Abstract Objective: To assess incidence and risk factors of cataract extraction in patients with primary congenital glaucoma, surgical outcome, and complications. Material and method: Retrospective cohort study, in which 108 patients with primary congenital glaucoma were included. Data collected were need for cataract extraction and at what age, intraocular pressure at diagnosis of primary congenital glaucoma, required antiglaucomatous surgeries, possible mutation in the CYP1B1 gene, and final visual acuity. Among the patients who required cataract extraction were visual acuity, intraocular pressure, and complications, evaluated preoperatively and postoperatively. The data were analysed with STATA. Results: A total of 198 eyes of 108 patients were included, with a median follow-up of 8 years (range: 5–53). In all, 32 eyes (16.2%) of 24 patients (22%) required cataract extraction. The median age for cataract extraction was 12.94 years (interquartile range: 2.42–22). The main identified risk factors associated with cataract extraction were antiglaucomatous surgeries (hazard ratio 1.48, p < 0.001) and valvular implant (hazard ratio 2.78, p < 0.001). Lens was implanted in 30/32 eyes and the main complications were intraocular pressure decontrol (n = 13), capsular fibrosis (n = 7), corneal decompensation (n = 4), lens subluxation (n = 4), and endophthalmitis (n = 1). Visual acuity improvement was observed after cataract extraction in 66.67% of eyes. Conclusions: There is a high incidence of cataract surgery in patients with primary congenital glaucoma, but generally outside of pediatric age. A greater risk of cataract extraction was identified in those patients with a greater number of antiglaucomatous surgeries, especially after valvular implantation. Despite the high rate of complications related to cataract extraction in primary congenital glaucoma, good visual improvement was observed after surgery. Keywords Childhood glaucoma, glaucoma surgery, cataract extraction Date received: 9 November 2018; accepted: 11 March 2019 1 Ophthalmology Department, Clínico San Carlos Hospital, Instituto de Investigación Sanitaria del Hospital Clínico San Carlos (IdISSC), Madrid, Spain 2 Genetics Department, University of Castilla-La Mancha, Ciudad Real, Spain Corresponding author: Blanca Benito-Pascual, Ophthalmology Department, Clínico San Carlos Hospital, Instituto de Investigación Sanitaria del Hospital Clínico San Carlos (IdISSC), Escosura 19 4 D, 28015 Madrid, Spain. Email: blancabenito91@gmail.com 841190EJO 0 0 10.1177/1120672119841190European Journal of OphthalmologyMorales-Fernández et al. research-article 2019 Original research article