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Alagille Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Occurring in up to 95% of ALGS patients but only 15% in the general population, posterior embryotoxon (a prominent Schwalbe ring) is a defect in the anterior chamber of the eye identifiable by slit-lamp examination (Figure 13.2) [16]. Axenfeld−Rieger anomaly (showing an off-center pupil [corectopia] or extra holes in the iris with the appearance of multiple pupils [polycoria], 45%), optic disk drusen (90%), diffuse fundus hypopigmentation (57%), and speckling of the retinal pigment epithelium (33%) are other ocular features associated with ALGS [19].
Ophthalmology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Causes include sector iris hypoplasia, colobomas, ectopia lentis et pupillae and Axenfeld–Rieger anomaly. Intermittent corectopia, with pupils shifting from central to eccentric positions, have been reported during coma and may represent a sign of rostral midbrain dysfunction.
The Developmental Glaucomas
Published in Neil T. Choplin, Carlo E. Traverso, Atlas of Glaucoma, 2014
Carlo E. Traverso, Alessandro Bagnis
Rieger’s anomaly is further along this clinical spectrum and shares many of the findings of Axenfeld’s with a greater degree of anterior iris stromal atrophy. Findings include polycoria, corectopia, and pupillary distortion. Like Axenfeld’s anomaly (and unlike iridocorneal endothelial syndrome), it is bilateral with an autosomal dominant inheritance pattern. Glaucoma is present in roughly 50% of patients with onset in infancy or later. Nonocular anomalies may also be present and include dental (hypo and microdentia), facial (hypotelorism, malar hypoplasia), and systemic findings (short stature, cardiac defects, empty sella, deafness, and mental deficiency) (Figures 13.11, 13.12, and 13.13a and b). The term Rieger’s syndrome is used when nonocular and systemic findings are present.
Corectopia grading: A novel classification system
Published in Seminars in Ophthalmology, 2022
Natalia Anisimova, Lisa Arbisser, Argyrios Tzamalis, Beáta Éva Petrovski, Natalya Shilova, Goran Petrovski, Sergey Anisimov, Boris Malyugin
Corectopia is the medical term describing an eccentric pupil displaced in relation to the apex of the cornea or the visual axis. A slight eccentricity of the pupil can be present in normal individuals as the pupil center is usually displaced about 0.5 mm inferonasal corresponding to the iris center.1 The pupil location is altered with increasing luminance up to 0.5 mm nasally.2 Interestingly, the pupil center shifts up to 0.6 mm temporally under pharmacological dilation.3 As minor eccentricity up to 1.0 mm is clinically and cosmetically insignificant, such displacement is therefore considered within the normal range.1