Ectopia Lentis et Pupillae
In the autosomal recessive disorder ectopia lentis et pupillae, the lens and the pupil are displaced in opposite directions. The pupil is irregular, usually slit shaped, and displaced from the normal position. The dislocated lens may bisect the pupil or may be completely absent from the pupillary space. This disorder is usually bilateral but not symmetric. Characteristically, the iris dilates poorly. Associated ocular anomalies include severe axial myopia, retinal detachment, enlarged corneal diameter, cataract, and abnormal iris transillumination.
Beebe DC. The lens. In: Kaufman PL, Alm A, eds. Adler’s Physiology of the Eye: Clinical Application. 11th ed. Mosby; 2011:131–163.
Congdon NG, Chang MA, Botelho P, Stark WJ, Datiles MB III. Cataract: clinical types. In: Tasman W, Jaeger EA, eds. Duane’s Clinical Ophthalmology. Lippincott Williams & Wilkins; 2006; vol 1, chapter 73.
Hiles DA, Kilty LA. Disorders of the lens. In: Isenberg SJ, ed. The Eye in Infancy. 2nd ed. Mosby; 1994:336–373.
Streeten BW. Pathology of the lens. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology. 2nd ed. Saunders; 2000:3685–3749.
Excerpted from BCSC 2020-2021 series: Section 11 - Lens and Cataract. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.