POSTERIOR POLAR ANNULAR CHOROIDAL DYSTROPHY: A CASE SERIES.

PURPOSE: To describe the multimodal imaging findings of posterior polar annular choroidal dystrophy (PPACD). METHODS: Retrospective case series of 2 patients diagnosed with PPACD. Spectral-domain optical coherence tomography, fundus autofluorescence, and fluorescein angiography findings of PPACD are described. Electroretinography results are also presented. RESULTS: Both patients presented with bilateral peripapillary atrophy extending to involve the temporal arcades in an annular, foveal sparing pattern. Both cases demonstrated outer retinal atrophy with foveal sparing on spectral-domain optical coherence tomography, and fluorescein angiography illustrated corresponding window defects with late staining. Fundus autofluorescence showed hypoautofluorescence in the center of atrophic areas but hyperautofluorescence at the leading edge. Electroretinography findings included cone loss with rod preservation. Inflammatory and infectious workup was unremarkable in both cases. CONCLUSION: This report describes the multimodal imaging findings of a rare and poorly described chorioretinal disorder, PPACD, and will serve to guide clinicians regarding the evaluation and management of this elusive condition.

A NEW MACULAR DYSTROPHY SECONDARY TO A NOVEL MUTATION IN THE PROMININ 1 (PROM1) GENE.

PURPOSE: To report a case of a new macular dystrophy caused by a novel mutation in the PROM1 gene. METHODS: History and clinical examination, fluorescein angiography, optical coherence tomography, autofluorescence, electrophysiology, and genetics. RESULTS: We report a case of a 51-year-old man with progressive central visual loss in both his eyes. On fundus examination and imaging, there were asymmetric atrophic macular lesions in both his eyes. Electrophysiology revealed a bilateral macular atrophy; genetic testing revealed a novel PROM1 mutation as the probable cause. CONCLUSION: A novel PROM1 mutation as the cause of a new bilateral macular atrophy is revealed.

Multifocal serous detachments as the presenting sign of thrombotic thrombocytopenic purpura.

PURPOSE: To report a case of bilateral multifocal serous detachments as the presenting sign of thrombotic thrombocytopenic purpura. METHODS: Observational case report. RESULTS: A 59-year-old woman presented with decreased vision and was found to have bilateral multifocal serous detachments. Her review of symptoms prompted further investigations that revealed evidence of acute intravascular hemolysis, renal failure, and cerebral ischemia. This led to the diagnosis of thrombotic thrombocytopenic purpura. CONCLUSION: Thrombotic thrombocytopenic purpura is a life-threatening condition that may present to an ophthalmologist. Recognizing its ophthalmic manifestations can be life saving for the patient.

Histologic findings after surgical excision of optic nerve head drusen.

PURPOSE: To report the histopathologic findings of a case of bilateral massive optic nerve head (ONH) drusen. METHODS: A 59-year-old man presented with bilateral massive ONH drusen and secondary compromise of the retinal vasculature leading to vision of counting fingers at 1 ft in the left eye and 20/30 in the right eye. The inferior one half of the left ONH druse was excised resulting in no light perception vision postoperatively. RESULTS: Energy-dispersive spectroscopy suggested the composition of the ONH druse to be calcium phosphate (Ca3[PO4]2), which has implications on pathogenesis of neuronal cell death in ONH drusen. Scanning electron microscopy demonstrated embedded metal particles, likely to be remnants of the instruments used. CONCLUSION: Massive ONH drusen are actually one large druse that has multiple excrescences on its surface giving it the variegated appearance. Although surgical excision of ONH drusen may be technically possible, consideration should be given to improvements in instrumentation and patient selection criteria.

Anterior ischemic optic neuropathy after uncomplicated scleral buckling surgery.

BACKGROUND: To report a case of non-arteritic anterior ischemic optic neuropathy (NAION) observed after uncomplicated scleral buckle placement. METHODS: A 57-year-old man presented with a rhegmatogenous retinal detachment of the left eye. He underwent uncomplicated scleral buckle placement and C3F8 injection. Perioperative intraocular pressures were normal. RESULTS: On postoperative day 24, fundus examination revealed swelling of the left optic nerve and features of an optic neuropathy (visual field defect, relative afferent pupillary defect). Fluorescein angiography did not reveal delayed choroidal filling. Review of systems and laboratory investigations were not consistent with giant cell arteritis. CONCLUSIONS: Non-arteritic anterior ischemic optic neuropathy may develop after uncomplicated scleral buckling surgery.

Minimally invasive method for repair of rhegmatogenous retinal detachment following treatment for retinoblastoma.

A minimally invasive method for repair of rhegmatogenous retinal detachment following treatment of retinoblastoma is described. Two children with retinoblastoma developed rhegmatogenous retinal detachments following treatment with a combination of chemotherapy and cryotherapy. Each eye underwent a non-drainage scleral buckling procedure without retinopexy of the retinal break. Postoperatively, the retinas reattached and retinopexy with indirect laser photocoagulation was performed during subsequent examinations under anesthesia. The retinas have remained attached with 2 to 5 years of follow-up. The tumor recurred in case 2, was treated with additional chemotherapy, and has undergone type 1 regression. Non-drainage scleral buckling without retinopexy is a useful technique for repairing rhegmatogenous retinal detachment in eyes with retinoblastoma. The retinal break can be treated postoperatively with indirect laser photocoagulation to minimize the chance of viable tumor cell dissemination.

Subretinal hemorrhage in cytomegalovirus retinitis.

A case of subretinal hemorrhage associated with cytomegalovirus (CMV) retinitis in a human immunodeficiency virus-positive woman is described. The patient was found to have an exudative retinal detachment involving the fovea, subretinal hemorrhage, cotton wool spots, and active CMV retinitis temporal to the exudative detachment. No evidence of systemic coagulopathy was found. Although unusual, subretinal hemorrhage may be observed in association with CMV retinitis in the absence of a systemic coagulopathy.

Intravitreal triamcinolone for treatment of complicated proliferative diabetic retinopathy and proliferative vitreoretinopathy.

BACKGROUND: We present a retrospective evaluation of the clinical outcome and complications associated with intravitreal injection of unaltered triamcinolone acetonide in conjunction with pars plana vitrectomy and silicone oil injection for the treatment of complicated proliferative diabetic retinopathy with tractional retinal detachment and severe proliferative vitreoretinopathy. METHODS: Thirteen eyes of 12 consecutive patients were identified from a computerized patient database. All eyes were operated on by the same surgeon and received 4 mg of unaltered, commercially available triamcinolone acetonide intravitreally, before silicone oil injection. The patients were followed for a mean of 4.7 months (range 1-15 months), and demographic as well as pertinent preoperative and postoperative clinical information was gathered. RESULTS: At the last follow-up visit, vision had improved in 4 eyes, remained stable in 5 eyes, and worsened in 4 eyes. The retina was attached at the end of follow-up in 10 of the 13 eyes. Eight of the 13 eyes did not show any clinical signs of re-roliferation or redetachment during the course of follow-up. The mean intra-ocular pressure did not increase (preoperative value was 10.8+/- 6.22 mm Hg with a range of 0-22 mm Hg; at last follow-up, mean pressure was 9.6 +/-3.86 mm Hg with a cumulative postoperative range of 0-26 mm Hg). Steroid crystals were visible at 1 month postoperatively in 3 eyes and did not hinder fundus examination significantly. INTERPRETATION: The intravitreal injection of low-dose, unaltered triamcinolone acetonide in the setting of pars plana vitrectomy and silicone oil injection for the treatment of proliferative vitreoretinopathy and complicated proliferative diabetic retinopathy appears to be well tolerated. Further controlled study is needed to clearly define the potential beneficial effects of intravitreal steroids in these 2 disease processes.

Determination of the incidence and clinical characteristics of subsequent retinal tears following treatment of the acute posterior vitreous detachment-related initial retinal tears.

PURPOSE: To determine the incidence of subsequent retinal tears (SRT) in patients who were previously treated for acute posterior vitreous detachment (PVD)-related retinal tears. DESIGN: Retrospective chart review. METHODS: A retrospective chart review of 155 eyes of 137 consecutive patients treated for acute PVD-related retinal tears was performed. RESULTS: During a median follow-up of 13 months (range 3 to 157 months) after treatment, SRT developed in 19 (12.2%) of 155 treated eyes. Most of the SRT occurred during the first 6 months (12 of 19 eyes) after initial treatment. Subsequent vetinal tears developed within the first 12 months in 15 of 19 treated eyes, however only six of 15 of these eyes were symptomatic. Of those patients who developed SRT after 12 months, all had new visual symptoms. CONCLUSION: Patients who present with acute PVD-related retinal tears are at a low but significant risk for developing SRT. A significant number of patients treated for PVD-associated retinal tears present within 1 year with SRT without symptoms.

Pseudohypopyon following intravitreal triamcinolone acetonide injection.

OBJECTIVE: To report a case of a pseudohypopyon that developed after intravitreal injection of triamcinolone acetonide for choroidal neovascularization from age-related macular degeneration. METHODS: Observational case report. RESULTS: A 62-year-old woman received an intravitreal injection of triamcinolone acetonide for the treatment of a choroidal neovascular membrane that developed as a result of age-related macular degeneration. A layer of yellowish deposits was observed in the anterior chamber 1 day after the injection. The patient denied any pain or reduced vision, and there was no redness noted on examination. The deposits cleared spontaneously on the fourth postoperative day. CONCLUSIONS: Pseudohypopyon may develop after intravitreal injection of triamcinolone acetonide. Distinguishing this from a true hypopyon is important because the treatment and prognosis are very different for the two conditions.

Pressure alopecia following vitreoretinal surgery.

PURPOSE: To describe a patient with pressure alopecia following vitreoretinal surgery. DESIGN: Case report. METHODS: An 11-year-old boy underwent vitreoretinal surgery for left retinal detachment. One week postoperatively, his parents noticed a patch of alopecia where his head may have been in contact with the wrist-rest assembly placed around the head during the surgical procedure. RESULTS: Pressure alopecia on the parieto-occipital region of the scalp was observed. Hair regrowth occurred during the follow-up visits. CONCLUSION: Pressure alopecia is a rare complication of lengthy surgery and is underrecognized in ophthalmic practice. Precautions should be taken to avoid this preventable complication.

Rhegmatogenous retinal detachment in the fellow phakic eyes of patients with pseudophakic rhegmatogenous retinal detachment.

PURPOSE: To determine the risk and clinical characteristics of rhegmatogenous retinal detachment (RD) in the fellow phakic eyes of patients with a pseudophakic retinal detachment (PPRD) in one eye. METHODS: The authors conducted a computer-generated chart review to find 64 consecutive patients with a PPRD in one eye and were phakic in the fellow eye. The clinical information was collected on all patients to investigate the risk and clinical characteristics of RD in the fellow eyes while still phakic. Information was also obtained on the fellow eyes that underwent cataract surgery and developed a PPRD. RESULTS: Sixty-four patients with a PPRD in one eye were phakic in the fellow eye. During an average follow-up of 57.4 months, five (7.8%) fellow eyes developed retinal detachment while still phakic. In addition to the five eyes with a phakic RD, 10 originally phakic fellow eyes underwent cataract surgery. Of these, one (10%) suffered an RD. CONCLUSION: Fellow eyes of patients with a PPRD have a significant risk of RD even if they do not undergo cataract surgery.

Benign lymphoid infiltrate of the iris simulating a malignant melanoma.

PURPOSE: To report a clinicopathologic correlation of an unusual benign lymphocytic iris mass in a patient who had no systemic lymphoproliferative disease. METHODS: Case report. RESULTS: A 49-year-old man developed a circumscribed, tan lesion in his left iris. The lesion was suspected clinically to be an atypical iris melanoma. Histopathologic studies of the resected mass revealed a solid tumor that was comprised of lymphocytes and histiocytes. Immunohistochemical studies identified that most of the cells were T lymphocytes. The histopathologic diagnosis was atypical lymphoid infiltrate. Workup for systemic lymphoma and Epstein-Barr virus infection was negative. CONCLUSION: Lymphoid infiltrate can manifest as a solitary mass that can simulate an iris melanoma.