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Retinopathy of prematurity (ROP) may lead to late-developing complications due to the abnormal vitreoretinal interface. In this case report, three adult patients (ages 39 years, 62 years, and 64 years) with a history of prematurity underwent imaging with ultra-widefield fluorescein angiography (UWFFA). The UWFFA demonstrated late peripheral vascular hyperfluorescence in all three patients, perivascular leakage in two of the patients, hyperfluorescent foveal avascular zone in two of the patients, peripheral avascular retina in two of the patients, vasoproliferative tumor in one of the patients, peripheral neovascularization in one of the patients, and vascular anastomosis in one of the patients. Descriptive analysis of the images is reported. The authors concluded UWFFA reveals details in adults with history of ROP that may gain importance during the follow-up or in the surgical management of these patients; however, prospective studies are needed. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:278-283.]., (Copyright 2018, SLACK Incorporated.)

A 6-day-old female baby with known diagnosis of congenital Zika infection was referred for ophthalmologic examination. The mother (37 years old) was referred for a pruritic rash, conjunctival hyperemia, and malaise at 12 weeks of gestation while still living in Venezuela. Upon arrival to Miami, Zika virus (ZIKV) exposure was confirmed during prenatal screening. At birth, due to the known exposure, a complete congenital ZIKV workup was performed, including brain ultrasound and MRI, which disclosed calcifications in the frontal lobe. Fundus examination revealed a hypopigmented retinal lesion in the left eye that was documented with retinal imaging. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:952-955.]., (Copyright 2016, SLACK Incorporated.)

Purpose: We report a rare case of spontaneous eyeball luxation associated with exophthalmos due to iatrogenic Cushing syndrome (CS). The normalization of serum hormones led to the regression of the picture., Case: A 64-year-old man presented with spontaneous globe luxation of the left eye after a 6-month history of bilateral, painless, and slowly progressive exophthalmos. The patient had been receiving weekly infusions of methylprednisone over the previous 6 months. His best-corrected visual acuity (BCVA) at presentation was 20/40 in the right eye and 20/20 in the left eye. The patient demonstrated full extraocular motility. The intraocular pressure (IOP) was elevated in the right eye (24 mHg) and normal in the left eye (18 mmHg). Exophthalmometry demonstrated bilateral proptosis with measurements of 27 mm in the right eye and 28 mm in the left eye. Computed tomography scan of the brain and orbits revealed increased orbital and cervical fat. Clinical, radiographic and serologic findings ruled out potential diagnoses including orbital metastasis, thyroid orbitopathy, carotid-cavernous fistula, and idiopathic orbital pseudotumor. Clinical suspicion of iatrogenic CS was high, and additional serologic testing confirmed the diagnosis., Conclusion: Exophthalmos is an uncommon sign of CS, but to our knowledge, this is the first reported case of spontaneous globe luxation secondary to CS. In our case, normalization of cortisol was sufficient to resolve the clinical symptoms and eliminated the need for surgical intervention such as orbital decompression surgery.

Chorioretinal folds may occur as a consequence of hypotony and can be a cause of vision loss when associated with macular involvement. In this report, the spectral domain ocular coherence tomography imaging of three patients with chorioretinal folds before and after management are presented. The cases had unique presentations and each underwent different management approaches, but the results included improved visual acuities and lessened chorioretinal folds.

Background and Objective: Retinal nonperfusion is closely associated with vision-threatening complications such as neovascularization and macular edema. The purpose of this study is to investigate the reliability of a calculated ischemic index (ISI) by means of intergrader and intragrader agreement on ultrawide-field fluorescein angiography (UWFFA) in common retinal vascular diseases., Patients and Methods: Eight trained graders evaluated 15 UWFFA images provided digitally and re-graded on a different day. They included five eyes with diabetic retinopathy (DR), five with branch retinal vein occlusion (BRVO), and five with central retinal vein occlusion (CRVO). To assess intergrader and intragrader agreement and variability among different diseases, the replicate inter- and intragrader standard deviations (SDs) and coefficients of variation (CVs) were calculated., Results: Mean ISI was 46% for images of DR, 26% for images of BRVO, and 61.3% for images of CRVO. Combined intragrader and intergrader replicate SDs were 17.8% for DR, 3.8% for BRVO and 13.0% for CRVO. Combined intragrader and intergrader replicate coefficients of variation were 38.6% (percent of mean ISI) for DR, 14.7% for BRVO, and 21.2% for CRVO., Conclusion: Intergrader and intragrader variability was high when assessing DR. This may be due to the chronic nature of DR progression, which can lead to patchy areas of ischemia. Intergrader and intragrader variability was better for CRVO and best for BRVO. This may be due to the acute or subacute nature of retinal vein occlusions., (Copyright 2015, SLACK Incorporated.)

Purpose: To report two cases of central macular sparing of subhyaloid hemorrhages secondary to vitreomacular adhesion documented by spectral-domain ocular coherence tomography findings., Methods: Review of two patients with subhyaloid hemorrhage secondary to proliferative diabetic retinopathy and a macroaneurysm were performed. Treatment was not performed in either patient for management of the hemorrhage, but the patient with proliferative diabetic retinopathy was treated with panretinal photocoagulation for peripheral neovascularization. Foveal sparing of the hemorrhage was documented clinically and on spectral-domain ocular coherence tomography., Results: In Case 1, the proliferative diabetic retinopathy was stabilized with 2 rounds of panretinal photocoagulation treatment, and the visual acuity improved to 20/20 in the affected left eye after 8 months of observation. In Case 2, the visual acuity returned to baseline at 20/60 after 4 months of observation., Conclusion: Macular involvement of subhyaloid hemorrhages may cause visual impairment, but vitreomacular adhesion may block the hemorrhage from obstructing the fovea.

Purpose: The purpose of this study was to report a case of a 34-month-old patient who presented with leukoria and underwent a vitrectomy and vitreous biopsy at an outside hospital followed by definitive enucleation for a nonteratoid medulloepithelioma and to review the literature regarding metastatic rates for this tumor after intraocular surgery., Methods: The authors reviewed the clinical, histopathologic, and neuroimaging records in the unusual case of a child with medulloepithelioma who had previously undergone pars plana vitrectomy and vitreous biopsy. An in-depth literature search was performed for patients with medulloepithelioma with prior biopsy or surgery., Results: Clinical examination showed a subluxed cataractous lens and neovascular glaucoma. Ultrasonography showed a large, cystic lesion with high reflectivity. Enucleation was performed, and histopathologic analysis showed a diffuse nonteratoid medulloepithelioma., Conclusion: Metastasis in medulloepithelioma is very rare. A systematic review of cases of patients with medulloepithelioma undergoing invasive intervention indicated that some patients subsequently developed metastases. Such procedures should be avoided to prevent orbital seeding or creating tracts for tumor migration and ultimate extraocular relapse.

Purpose: To examine the effects of prosthesis refitting on porous orbital implant exposure in 100 patients who underwent enucleation for retinoblastoma., Methods: Parameters analyzed included patient's age at enucleation, gender, implant type, frequency of prosthesis adjustment, and use of chemotherapy or radiation. The main outcome measures consisted of the condition of the fornices, condition of the implant (including conjunctival thinning), and condition and functioning ability of the prosthesis., Results: Increased frequency of visits with the ocularist and number of prosthesis adjustments and refits significantly improved the condition of the implant, fornices, and prosthesis. The patient's age at enucleation, gender, and use of chemotherapy or radiation did not have a significant effect on any of the outcome variables. The condition of the implant was significantly better for porous polyethylene than hydroxyapatite implants (P = .024). No implant exposures were observed., Conclusion: Frequent adjustments and refits by the ocularist are significantly associated with a reduced rate of conjunctival thinning and complete avoidance of implant exposure in patients undergoing enucleation for retinoblastoma. These findings are particularly significant for this population, which historically has demonstrated a high rate of implant exposure., (Copyright 2011, SLACK Incorporated.)

Background: Conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma has been well-described, but rarely do these lesions demonstrate intraocular involvement. We report a case of conjunctival MALT lymphoma with intraocular involvement and novel spectral-domain ocular coherence tomography (SD-OCT) findings., Patient and Methods: A 75-year-old woman with biopsy-proven MALT lymphoma of the conjunctiva presented with ipsilateral yellowish diffuse choroidal infiltrates on fundoscopic examination. Choroidal involvement was documented clinically, on ultrasonography, on fluorescein angiography, and by SD-OCT. Treatment consisted of 3 weeks of oral doxycycline and six cycles of systemic chemotherapy with cyclophosphamide, vincristine, prednisone, and rituximab., Results: There was no evidence of progression or recurrence of MALT lymphoma after 9 months of follow-up. Despite complete resolution of clinical findings, ultrasound, and fluorescein angiography, the choroidal lesions remained unchanged on SD-OCT., Discussion: Choroidal involvement of conjunctival MALT is rare, and can be successfully treated. Persistence of irregularities on SD-OCT did not influence management in the presence of clinical improvement and resolution on ultrasonography and fluorescein angiography.

Purpose: To report a case of a boy with bilateral germinal retinoblastoma and Down syndrome who was successfully treated despite a predilection toward chemotoxicity associated with Down syndrome., Methods: A 3-month-old boy presenting with germinal retinoblastoma and Down syndrome was treated with diode laser and systemic carboplatin and low-dose vincristine, with gradual dose escalation. Etoposide and cyclosporine were later added for a greater tumor response. After cyclosporine was added, the patient was hospitalized due to vincristine toxicity. The vincristine dose was further lowered, and periocular carboplatin injections were given to establish control of intraocular disease., Results: The tumors regressed after 11 sessions of laser and chemotherapy. Because of residual vitreous seeding in the right eye, a series of two periocular carboplatin injections were given. Six more laser sessions were applied to the tumors. There is no evidence of active retinoblastoma after 11 months of follow-up., Conclusion: Because of increased chemosensitivity in patients with Down syndrome, it is important to reduce systemic drug dosages and consider augmenting local therapy in retinoblastoma cases.