In vivo angle dysgenesis in PHACE syndrome with developmental glaucoma.

A girl in middle childhood presented with glaucoma in her right eye along with segmental haemangiomas on the right side of the face and neck. Magnetic resonance angiography of the brain showed hypoplasia of the right internal carotid artery, leading to the diagnosis of posterior fossa malformations, haemangioma, arterial anomalies, cardiac defects and eye abnormalities (PHACE) syndrome. High-definition anterior segment ocular coherence tomography (AS-OCT) of the right eye showed an absence of Schlemm's canal and a hyperreflective membrane over the trabecular meshwork. The presence of this angle dysgenesis on AS-OCT, a novel finding in this disease, explained the elevated intraocular pressure in the right eye. The embryological basis for the development of angle dysgenesis might help better understand the pathogenesis of PHACE syndrome.

Study 3: Anterior segment optical coherence tomography-guided surgical approach in slipped medial rectus muscle.

Purpose: To discuss the novel swept-source anterior segment optical coherence tomography (SS-ASOCT)-guided surgical approach in slipped medial rectus muscles. Methods: Prospectively (between February 2020 and July 2022), six patients with a clinical suspicion of slipped medial rectus muscle were recruited. After complete ophthalmic and orthoptic evaluation, the missing medial rectus muscle is screened using Anterior Segment Optical Coherence Tomography (ASOCT). In presence of a traceable muscle, its morphology, depth, and distance from a fixed anatomical landmarks were noted; in its absence, the status of other recti was noted. Intraoperatively, the features were confirmed and the intended intervention was performed. Results: The mean age of six patients was 25.66 ± 9.72 years, two with surgical trauma and four with penetrating trauma (66.66%). In five patients, the ASOCT traced the slipped medial rectus muscle successfully (83.33%); intraoperatively, the same was confirmed (within 1-2 millimeters) with favorable outcomes. ASOCT made a significant contribution in all subjects by reducing the number of interventions and muscle surgeries. Conclusions: In eyes with slipped medial rectus muscle, especially those which are within a finite distance from the angle can be traced using ASOCT. This approach impacts the outcomes in many ways.