A Case of Rhegmatogenous Retinal Detachment in Chronic Myeloid Leukemia.

PURPOSE: Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm characterized by uncontrolled proliferation of granulocytes, caused the BCR-ABL1 fusion gene. While ocular manifestations of CML are rare, the presentations can range from asymptomatic to sudden vision loss. CML associated ocular findings that have been reported include retinal hemorrhages, leukemic infiltrates, and optic disc edema, but a rhegmatogenous retinal detachment (RRD) in the setting of CML has not been described. METHODS: Case report. RESULTS: A 21-year-old man presented with intermittent vision loss in his right eye, tinnitus in the right ear, and abdominal distension. Work up revealed significant leukocytosis, splenomegaly, and a positive BCR-ABL1 mutation. He was diagnosed with CML and started on systemic therapy. Exam of the right eye revealed a large intraocular mass. After two weeks of systemic treatment, the large elevation in the right eye had improved, allowing visualization of diffuse subretinal whitening. At follow up, an RRD secondary to an atrophic hole in an area of prior subretinal infiltrates were noted. He underwent repair with a scleral buckle. Postoperative course was complicated by redetachment with proliferative vitreoretinopathy, which led to an unrepairable detachment, despite multiple surgeries with silicone oil tamponade. CONCLUSIONS: Ocular findings related to CML are rare, with the lowest incidence when compared to other leukemias, and are associated with worse outcomes. Posterior segment findings include intraretinal hemorrhages, Roth spots, and retinal infiltrates. This unique case describes an RRD in CML retinopathy with an aggressive course and poor anatomical result.

Redefining the Spectrum of Pentosan Polysulfate Retinopathy: Multimodal Imaging Findings from a Cross-Sectional Screening Study.

PURPOSE: There is growing evidence of a direct association between pentosan polysulfate (PPS) therapy and the development of macular changes. Using standardized visual acuity (VA) testing and multimodal imaging, we investigated the impact of PPS therapy on vision and described an expanded spectrum of imaging findings among PPS users. DESIGN: Cross-sectional screening study. PARTICIPANTS: Thirty-nine patients who were current or recent users of PPS. METHODS: The participants underwent a brief eye examination and answered a comprehensive medical and ophthalmic history questionnaire. Color fundus photography, fundus autofluorescence (FAF), and spectral-domain OCT (SD-OCT) were performed. The images were evaluated by expert graders at Wisconsin Reading Center. Abnormalities were categorized as definite toxicity (DT) if seen on both FAF and SD-OCT and as questionable toxicity (QT) if seen on either FAF or SD-OCT. MAIN OUTCOME MEASURES: ETDRS and Snellen VA, the dosage and duration of PPS exposure, and the prevalence of retinal toxicity on imaging. RESULTS: The mean ETDRS and Snellen VA of the study cohort were 85 letters and 20/22, respectively. The mean PPS daily dose was 282 mg (range, 88-400 mg), whereas the mean cumulative dose was 915 g (range, 19-3650 g) over a mean period of 8.8 years (range, 2 months-25 years). There was evidence of retinopathy in 41% of the eyes; DT was identified in 24 eyes (31%) and QT in 8 eyes (10%). Retinal pigment epithelium (RPE) abnormalities (thickening or thinning or both) were present in all eyes with DT. Retinal pigment epithelium atrophy was seen in 7 eyes (9%). In addition to well-established findings, the unique SD-OCT features of this cohort included interdigitation zone abnormalities and the presence of a flying saucer-type defect. Fundus autofluorescence abnormalities were seen in 24 eyes (30.8%), with 20 (66.7%) of these exhibiting abnormalities located outside the central subfield and extending beyond the arcades. CONCLUSIONS: Findings from the masked grading of multimodal imaging at a centralized reading center suggest a wider phenotypic spectrum of structural abnormalities among patients taking PPS. Macular changes selectively involve the RPE and outer retina, with a range of findings often seen beyond the arcades. The subtle and atypical findings in this cohort should prompt clinicians to consider lowering the threshold for diagnosing PPS retinopathy.

Management of Iris Retraction Syndrome with Heterochromia and Retinal Detachment.

Iris retraction syndrome (IRS) is an uncommon condition caused by retinal detachment that is characterized by back bowing of the peripheral iris, leading to a deep anterior chamber. It is commonly associated with ocular hypotony, ciliochoroidal detachment, and anterior proliferative vitreoretinopathy. We describe a case of a 66-year-old man presenting with 2 weeks of right eye pain, redness, and iris heterochromia. The patient was diagnosed with IRS secondary to a chronic retinal detachment. Initial management with topical steroids and mydriasis allowed resolution of the iris retraction and heterochromia, normalization of intraocular pressure, and improvement of choroidal detachment. Subsequent vitrectomy with endolaser and oil tamponade led to successful detachment repair. Initial pharmacologic management allows a more controlled approach to the repair of retinal detachment associated with IRS. The patient's presentation is consistent with the hydrodynamic hypothesis of IRS.

Management of Complications and Vision Loss from Proliferative Diabetic Retinopathy.

PURPOSE OF REVIEW: Diabetes can be associated with profound visual loss due to several mechanisms. As the duration of diabetes and blood glucose levels increase, these changes become more severe. The proliferation of new blood vessels, vitreous hemorrhage, and tractional retinal detachments may ultimately result and can be devastating to visual function. New advances, including anti-vascular endothelial growth factor (VEGF) medications and innovative microsurgical instruments, have provided additional methods for the management of diabetic retinopathy in the clinic and in the operating room, leading to improved outcomes. RECENT FINDINGS: Advances in earlier treatment of proliferative diabetic retinopathy, especially with anti-VEGF injections, allow for a reduction in severity, improved vision, and more controlled and successful surgery. Modern surgical techniques and instrumentation have also allowed for improved patient outcomes. Future research into sustained delivery and release of anti-VEGF, reducing the need for frequent in-office injections, may prove to be additionally beneficial. Over the last decade, anti-VEGF has become an increasingly common treatment modality for the management of proliferative diabetic retinopathy, vitreous hemorrhages, and tractional retinal detachments. Further research is needed to determine the ideal method of delivery and timing of the treatment.

DISAPPEARING CHOROIDAL MELANOMA ON OPTOS: THE NOSE ARTIFACT.

PURPOSE: To illustrate a potentially concerning artifact on Optos wide-angle imaging. METHODS: Case report. RESULTS: A healthy 11-year-old girl, with no medical history, was found to have a lightly pigmented superotemporal choroidal tumor, suspicious for melanoma, which was detected on Optos wide-angle imaging by her optometrist. On referral to the retinal specialist, the mass was not visible on funduscopy. B-scan ultrasonography depicted flat retina and no visible mass. Review of the Optos image revealed that the pseudomelanoma represented the patient's nose. CONCLUSION: Optos wide-angle imaging provides broad retinal imaging; however, artifacts from patient positioning can lead to the nose artifact.

Impending aortic aneurysm rupture - a case report and review of the warning signs.

Abdominal aortic aneurysm (AAA) may present with subtle clinical findings. Recognition of the imaging features of an impending rupture is key for timely diagnosis. This report reviews the classic computed tomography findings of impending AAA rupture and presents a recent case which illustrates the key features.

Hemifacial spasm in a patient with basilar artery dolichoectasia caused by uncontrolled hypertension.

A 47-year-old male presented with a 2-year history of hemifacial spasm. Magnetic resonance imaging performed showed his tortuous basilar artery with nerve compression, and the patient was treated conservatively with botulinum toxin injections with complete resolution of symptoms. This rare disease was caused by his long history of hypertension, which led to his major basilar artery dolichoectasia.

Iris metastasis from systemic cancer in 104 patients: the 2014 Jerry A. Shields Lecture.

PURPOSE: To describe the clinical features, treatment, and outcome of patients with iris metastasis. METHODS: Retrospective case series of 160 tumors in 107 eyes of 104 patients with iris metastasis from systemic cancer. RESULTS: The median age at presentation with iris metastasis was 60 years. Iris metastasis was more common in whites (n = 93, 89%) and women (n = 65, 62%). The primary tumor was located in the breast (n = 34, 33%), lung (n = 28, 27%), skin (melanoma) (n = 13, 12%), kidney (n = 7, 7%), esophagus (n = 3, 3%), and others (n = 19, 19%). The main symptoms were pain (n = 33, 32%) or blurred vision (n = 31, 30%). The main findings were corectopia (n = 38, 37%) and secondary glaucoma (n = 38, 37%). The metastasis was unifocal (n = 84, 78%) or multifocal (n = 23, 21%) for a total of 160 tumors. The main tumor was centered at the iris root (n = 72, 69%), midzone (n = 22, 21%), or pupillary margin (n = 10, 10%), with median tumor diameter of 5 mm. Coexistent conjunctival or ciliary body/choroidal metastases were found in 39 patients. Management of iris metastasis included systemic chemotherapy (n = 18, 22%), external beam radiotherapy (n = 34, 41%), plaque radiotherapy (n = 20, 24%), surgical excision (n = 4, 5%), enucleation (n = 3, 4%), or observation (n = 4, 5%). Following treatment, tumor control without recurrence was achieved in 95% cases. In 98 cases with adequate follow-up information, death occurred in 85 (87%) at median 10 months (range, <1-239 months) from the date of iris metastasis. CONCLUSIONS: Metastatic tumors to the iris generally originate from primary malignancies in the breast, lung, or skin (melanoma). Despite successful ocular treatment, life prognosis is poor.