| We present the case of congenital aniridia patient with subluxation of spherophakia in both eyes who developed secondary glaucoma in the right eye. The paitient was a 51-year-old female who come to us with a complain of painful right eye. IOP was 38.8mmHg in the right eye with corneal edema. VA was hand motion in the right eye and 0.1 in the left, which can not be improved by refraction. Except totally aniridia, smaller corneas (horizonal and vertical diameters were 9mm and 8.5 respectively), subluxation of cataractous lens, rotating nystagmus and high myopia were also detected in her both eyes. Gonioscopy manifested angle closure in right eye and N3 angle in left eye. No fovea reflex and smaller optic disc were detected in the fundus examination. With B scan we found that her subluxated lenses were in spherical shape. For treatment, we removed the subluxated cataractous lens of the right eye with intra-vitreous ultrasonic disintegrator and an Ahmed glaucoma valve was implanted into this eye to control the IOP. Consider of the high myopia and poor BCVA, no IOL was implanted and the patient refuse to remove her left lens. After the operation, the IOP of the right eye decreased to 6mmHg and the BCVA was not improved. In addition, her 18-year-old son and 16-year-old daughter had similar congenital conditions: congential aniridia, spherophakia, small cornea, moderate myopia, rotating nystagmus and amblyopia. No fovea can be detected in macular by OCT. There were scatter dot opacities can be observed in their lens. Their refraction errors have been corrected and ocular conditions are screened regularly.
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