Long-term fundus changes due to Fundus albipunctatus associated with mutations in the RDH5 gene.

Report of a Case. A 56-year-old woman had a 2-month history of decreased vision and mild discomfort in the left eye. She had a history of penetrating injuryat theageof8years that led to traumatic cataract and corneal scarring in her left eye. Two years before we saw her, she had undergone uncomplicatedcombinedcataractextraction and lens implantation with penetrating keratoplasty elsewhere. There had been a satisfactory convalescence, but the visual acuity OS remained counting fingers at 1 m. Amblyopia was therefore suspected. At our first examination of her, the visual acuity was 6/9 OD and hand motion in the left eye. Full ophthalmological workup revealed a graft rejection and a subtotal retinal detachment with a peripheral retinal tear at the 6-o’clock position in the left eye. Intraocular pressure was 22 mm Hg OD and 18 mm Hg OS. In the right eye there was early nuclear cataract formation, but otherwise all findings were normal. No signs of pseudoexfoliation were noted and the cup-disc ratio was 0.3. The patient was admitted and the graft rejection was successfully treated with intensive topical steroids. After the corneal graft inflammation settled, the patient underwent a left pars plana vitrectomy with endolaser and silicone oil filling under retrobulbar anesthesia. No systemic atropine was used. Postoperatively the patient was positioned facedown. The retina was flat under silicone oil on day 1 and the pressure was 40 mm Hg in the eye that had undergone surgery. Systemic treatment with oral dorzolamide hydrochloride reduced the pressure to 27 mm Hg. On day 3 the patient complained about severe pain in the right (fellow) eye. The visual acuity had dropped to 6/60 OD. On examination, acute angle closure with very shallow anterior chamber and angle grade 0 (Schaffer) was found. The intraocular pressure was 45 mm Hg despite continued treatment with dorzolamide hydrochloride, 250 mg 4 times a day, since day 1 after surgery and additional intravenous 20% mannitol (300 mL over 1 hour). Right eye YAG laser iridotomies were performed and the intraocular pressure dropped as the anterior chamber deepened. Pressures remained within normal limits after withdrawal of all systemic and topical treatment. The visual acuity returned to 6/12 OD. Gonioscopy 2 months later showed a grade 2 (Schaffer) anterior chamber angle in the right eye with small patent laser iridotomies at the 11and 2-o’clock positions. The axial length was measured at 22.79 mm. A reliable A-scan was not possible in the left eye because of the silicone oil filling.