OBJECTIVE: To detect and characterise visual field (VF) defects using static Octopus perimetry in patients with primary congenital glaucoma (PCG) and to determine VF quality and time duration.
METHODS: Eighty-eight eyes of 70 patients diagnosed with PCG were included. Assessments were performed using an Octopus 900 and each eye was assessed with the tendency-oriented perimetry (G-TOP) algorithm. Quantitative VF data were collected: quality data (false positive and negative response, and time duration) and results of mean deviation (MD) and square root of loss variance (sLV). Qualitative data were collected: the presence of diffuse and localized defects, the affected hemifield and grade of defects using the Aulhorn and Karmeyer classification. Correlations between perimetric results and clinical variables were analysed.
RESULTS: Median age was 11 (8-17) years. 65.9% (58/88) of PCG eyes showed VF defects. Diffuse defects were observed in 10/58 eyes (16.94%) (mean MD = 23.92 [SD: 2.52]) dB) and localized defects in 48/58 eyes (82.75%). The most frequent defect was spot-like/stroke-like/incipient paracentral scotoma (n = 15), nasal step (n = 8), adding arcuate defect (n = 2), half ring-shaped (n = 13) and concentric defect with a central island (n = 9). And the most frequent affected visual hemifield was inferior hemifield. Mean test duration was 2 min 12 s (SD: 21.6 s). MD and sLV values were correlated with best corrected visual acuity (BCVA), cup to disc ratio and number of antiglaucoma surgeries (all P < .001).
CONCLUSIONS: A high number of diffuse and localized defects were identified using Octopus perimetry in PCG patients. The most frequent defect was paracentral scotoma and inferior hemifield was the most affected.

Aniridia is a congenital bilateral ocular disorder with dominant autosomal inheritance. More than 50% of patients will develop aniridic glaucoma (AG) during their lives. Open angle glaucoma is more common in aniridia, but a closed angle mechanism has been described in relation with anterior rotation of the rudimentary iris, occluding trabecular meshwork. Diagnosis and follow-up of AG is difficult in relation with the presence of keratopathy, nystagmus and foveal hypoplasia. Central corneal thickness usually measures more than 600 microns, which prevents achieving a reliable value of intraocular pressure. Medical treatment of AG is no different from the rest of glaucoma. It is recommended to use preservative free formulations, and combined therapy is often required. Surgical treatment is needed in many cases. There is no consensus on the first line surgery for AG, but in open angle AG, angle surgery is usually first choice, and glaucoma drainage devices are the next preferred surgical technique. In closed angle AG glaucoma drainage devices are usually the first choice, with trabeculectomy as the second preferred surgical technique.

Aniridia is a congenital bilateral ocular disorder with dominant autosomal inheritance. More than 50% of patients will develop aniridic glaucoma (AG) during their lives. Open angle glaucoma is more common in aniridia, but a closed angle mechanism has been described in relation with anterior rotation of the rudimentary iris, occluding trabecular meshwork. Diagnosis and follow-up of AG is difficult in relation with the presence of keratopathy, nystagmus and foveal hypoplasia. Central corneal thickness usually measures more than 600 microns, which prevents achieving a reliable value of intraocular pressure. Medical treatment of AG is not different from the rest of glaucoma. It is recommended to use preservative free formulations, and combined therapy is often required. Surgical treatment is needed in many cases. There is no consensus on the first line surgery for AG, but in open angle AG, angle surgery is usually first choice, and glaucoma drainage devices are the next preferred surgical technique. In closed angle AG glaucoma drainage devices are usually the first choice, with trabeculectomy as the second preferred surgical technique.

The neonate has a horizontal diameter of the cornea, usually up to 10mm with growth up to 2mm in the first 2 years of life. We report a case of megalocornea, a rare, recessive, X-linked disorder in a 3-month-old child, seeking to review what the medical literature brings information about the condition, as well as diagnostic and follow-up parameters, of its main differential diagnoses.