Slit lamp examination OD OS Conjunctiva Normal color Severe congestion Sclera White, silent eye Dilated episcleral vessels Cornea Transparent, normal Mild epithelial edema, no thickness, no KP KP AC Absent in periphery, normal depth in the center; cellular Tyndall= [empty set] Iris "Iris bombe", fine IPS/ 360*; atrophic iris stroma, multiple rolling folds near the iris scleral insertion, supero-temportal rotation of the entire iris (Figure 1) Pupil Medium mydriasis, not reflex, pupillary diameter = 4 mm, mild corectopia
(supero-temporal) Lens Normally positioned, transparent Lacrimal Normal Intense hyperlacrimation adnexa Abbreviations: AC--anterior chamber, KP--keratic precipitates, PIS-- peripheral iris synechiae Table 2.
PATOLOGIA HERENCIA Sindrome de Axenfeld Rieger AD Locus: RIEG1/ RIG2 Cromosomas: 4q25 6p25 Retinosis pigmentaria AR, AD Sindrome de Marfan AD Mutaciones: Gen de la fibrilina 1 (FBN1) Los genes TGFBR 1 y 2 (47) Osteogenesis imperfecta AD Mutaciones en: COL1A1/COL1A2 (49) Sindrome de dispersion AD pigmentaria Cromosomas: 7q35-q36 18q11-21(29) PATOLOGIA CARACTERISTICAS Sindrome de Axenfeld Rieger Penetrancia completa, con Locus: RIEG1/ RIG2 defectos oculares, como Cromosomas: hipoplasia del iris, adherencias 4q25 iridocornea-les, corectopia
, 6p25 policoria y embriotoxon posterior, y otros rasgos menos frecuentes, como cataratas, desprendimiento de retina y microcornea (30).
iris holes and corectopia in combination with the findings of the Axenfeld anomaly.
These include iris stromal hypoplasia, ectropion uveae, corectopia, full-thickness iris defects, severe iris atrophy, prominent, anteriorly displaced Schwalbe's line and extensive peripheral anterior synechiae.
Although MMC significantly reduced the rate of pterygium recurrence, severe complications such as corneal oedema, corneal perforation, scleral calcification, corectopia
, iritis, sudden onset mature cataract, severe secondary glaucoma, incapacitating photophobia, and pain were also reported8.
Posterior polymorphous dystrophy with polycoria and corectopia
Ectopic pupil, also known as corectopia
, causes an abnormally shaped pupil.
2) Rieger described the patients with congenital iris abnormalities including iris hypoplasia, corectopia and polycoria as Rieger anomaly which in case associates with systemic findings such as dental, facial bone defects, umbilical, pituitary abnormalities, termed as Rieger Syndrome.
Anterior chamber was deep and quiet in both eyes, corectopia was noticed and pupil showed grade 2-3 RAPD on left eye.
Rieger described patients with congenital iris abnormalities including iris hypoplasia, corectopia, and polycoria, now referred as rieger anomaly, in 1935(2).
1), eye changes in the form of iris hypoplasia, polycoria (multiple pupils), corectopia (eccentrically placed pupil), posterior embryotoxon (white rim of cornea), which refers to displacement of schwalbe's line anterior to limbus in the cornea.
(or ectopic pupil), is similarly either congenital or acquired, as a result of penetrating trauma, surgery, iridodialysis or inflammation.