Follow-up report of fundus findings of tuberous sclerosis-associated retinal astrocytoma of two siblings.

A late adolescent with tuberous sclerosis (TS) presented with reduced vision in one eye to our tertiary care university hospital 4 years ago. Fundus examination revealed multiple retinal astrocytic hamartomas (RAHs) in both eyes. His younger sibling, who also had TS, was found to have RAH on retinal screening. The swept-source optical coherence tomography (SS-OCT) findings were typical of RAH. We further noted that some of the RAH lesions showed segmental whitening of the outer walls of the arterioles, which traversed through them. The segmental whitening may suggest the enveloping of normal retinal vessels by the tumour. En-face and B-scan SS-OCT angiography of patients with TS showed vascularity within the tumour. The vessels within the tumour appeared to be in continuity with the retinal vasculature. Both siblings were reviewed annually. At the end of 4 years, there was no change in visual acuity, tumour size, number, vascularity and behaviour.

Clinical applications of aqueous angiography in glaucoma.

Aqueous humor outflow (AHO) pathways are the main site of resistance causing elevated intraocular pressure in glaucoma, especially primary open-angle glaucoma patients. With the recently introduced technique of aqueous angiography (AA); functional, real time assessment of AHO from proximal (trabecuar meshwork) to distal pathways under physiological conditions has been made possible. AHO pathways are segmental, and AA can identify high-flow region (increased angiographic signals) and low flow region (decreased angiographic signals) in an individual. With the introduction of canal-based minimally invasive glaucoma surgeries (MIGS), the assessment of AHO can help guide the placement of stents/incisions during MIGS procedures. This can allow individualized and targeted MIGS procedures in glaucoma patients for better results. Based on the density of AHO pathways visualized on AA, surgeons can decide whether to perform MIGS or conventional glaucoma surgery for improved outcomes for the patient. Immediate intraoperative assessment for functionality of the MIGS procedure performed is possible with AA, allowing for surgical adjustments of MIGS procedure in the same sitting, if needed. This review provides a summary of the studies performed with AA to date, with a special focus on Indian patients. It covers the basics and clinical applications of AA for improving surgical outcomes in glaucoma patients.

Primary congenital glaucoma: An iridotrabeculodysgenesis?

PURPOSE: To analyze primary congenital glaucoma (PCG) anterior chamber and angle anomalies over 360° as possible biomarkers of severity and prognosis. METHODS: A cross-sectional observational study was conducted analyzing anterior segment anomalies of PCG patients over 4 years of age who underwent trabeculectomy combined with trabeculotomy and age-matched controls using anterior segment optical coherence tomography (ASOCT), CASIA-2. Anterior iridotrabecular adhesions or anterior iris insertion was identified and quantified from the scleral spur using the iridotrabecular contact (ITC) index parameter as a surrogate. RESULTS: There was a variable but significantly increased anterior iridotrabecular adhesion on ITC index, ITC area, corneal volume, anterior chamber volume, iris volume, anterior chamber depth, and small/absent trabecular meshwork in PCG eyes compared to control eyes. In PCG eyes, anterior iridotrabecular adhesion had a positive correlation with pre-operative central corneal thickness (CCT) (r = 0.53, P = 0.02), review iris thickness (r = 0.4, P = 0.04), and ITC area (r = 0.85, P < 0.001). Review iris thickness had a negative correlation with pre-operative vertical cup-disc ratio (r = -0.51, P = 0.008). Iris hypoplasia with fewer or absent folds, collarette, pupillary ruff, and pupillary ruff to collarette distance was significantly different from controls. CONCLUSION: ASOCT in PCG eyes has shown that they have variable anterior iridotrabecular tissue adhesions, anomalous tissue/membranes in the angle, and iris hypoplasia correlating with pre-operative cup-disc ratio. These features could be used as gonioscopic and clinical biomarkers to assess the severity and prognosis of the disease. The presence of abnormal iris morphology and iridotrabecular tissue anomalies in PCG suggests that it is more than just isolated trabeculodysgenesis and is probably best considered as part of the anterior segment dysgenesis spectrum.

Recruitment of Temporal Aqueous Outflow Channels After Bent Needle Ab-Interno Goniectomy Demonstrated by Aqueous Angiography.

PURPOSE: To demonstrate the utility of operating on the temporal trabecular meshwork with in vivo - aqueous angiography demonstrating new aqueous outflow channels. METHOD: In a patient with primary open angle glaucoma, nuclear sclerosis, and medically uncontrolled intraocular pressure, Indocyanine green aqueous angiography (0.5%) was performed to visualize baseline functional aqueous outflow channels. This was followed by 30 degrees bent needle ab-interno goniectomy in the temporal quadrant, where no aqueous outflow channels were initially visualized. Aqueous angiography was repeated using 2% fluorescein to visualize aqueous outflow channels after bent needle ab-interno goniectomy. RESULTS: Prebent needle ab-interno goniectomy, aqueous angiography revealed functional outflow channels in the nasal quadrant although none were visible in the temporal quadrant. Postbent needle ab-interno goniectomy in temporal quadrant aqueous angiography demonstrated 2 new aqueous outflow channels. CONCLUSION: In a patient with open angle glaucoma, following temporal quadrant ab-interno goniectomy, the recruitment of aqueous outflow channels was demonstrated using aqueous angiography.