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Ectopia lentis et pupillae: case report

The main purpose of this report is to describe a case of ectopia lentis et pupillae syndrome, highlighting the secondary ocular complications and their differential diagnoses. A 27-year-old man presented with complaint of low visual acuity. No evidence of the syndrome was found at presentation. The results of supplementary tests were normal. On ophthalmologic examination, visual acuity was finger count at 2 meters in the right eye and finger count at 1 meter in the left eye. Exotropia of 25 dioptrics. Corneal diameters of 10 mm and 9 mm in the horizontal and vertical meridians, respectively. Keratometry readings of 39.00 at 178 x 43.87 at 88 for the right eye and 37.64 at 22 x 42.75 at 122 for the left eye. The biomicroscopic examination revealed iris atrophy in both eyes, absence of crypt and absent iris transillumination. Centralized right pupil with regular form and left pupil with inferior-temporal dislocation. Opaque lenses with reduced sizes and superior-nasal dislocation. Normal intraocular pressure. Axial length of 26 mm and 30 mm and crystalline axial diameter of 5.4 and 4.5 mm in right and left eyes, respectively, as revealed by ultrasonography. The differential diagnosis encompasses exclusively ocular abnormalities, syndromes and metabolic disorders. Diagnosis of the ectopia lentis et pupillae is fundamental not only to evaluate the risk, prognosis and treatment, but also to assist in differentiating other syndromes with systemic impairment. A visual acuity reduction is normally caused by severe myopia development, cataract, corneal astigmatism, retinal detachment and glaucoma, justifying regular ophthalmologic support for these patients.

Ectopia lentis; Eye abnormalities; Diagnosis,differential; Glaucoma; Iris


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