Terson's Syndrome in a Patient with von Hippel-Lindau Disease.

PURPOSE: Terson's syndrome is a condition where a preretinal hemorrhage forms as a result of increased intracranial pressure. The elevated intracranial pressure is thought to be transmitted through the veins and the optic nerve sheath to the optic disc and retina, causing the thin capillary walls to rupture. The authors present a unique case of Terson's syndrome in a patient who underwent recent surgical management for cerebellar hemangioblastomas related to von Hippel-Lindau disease. CASE REPORT: A 17-year-old African American female patient with a history of von Hippel-Lindau disease presented with pain in her right eye. She had recently undergone surgery to remove cerebellar hemangioblastomas. Preliminary fundus imaging was performed, but before formal ophthalmic testing could be conducted, the patient seized and was taken directly to the emergency room. When the patient returned for a formal evaluation 3 weeks later, a new preretinal "boat-shaped" hemorrhage was now present. Additionally, reports from the emergency room suggested that she had bled into the cavity where the previous cerebellar resection had taken place. This hemorrhage likely led to an increase in intracranial pressure, causing a Terson's-like event. CONCLUSIONS: A Terson's event may be caused by high intracranial pressure secondary to the surgical removal of von Hippel-Lindau syndrome-associated cerebellar tumors and should be included as a possible complication of surgical management.

Simple hamartoma of the retinal pigment epithelium with macular edema.

PURPOSE: Presumed congenital simple hamartoma of the retinal pigment epithelium (CSHRPE) is a rare intraocular finding that is described as a focal, nodular, jet black lesion. These lesions frequently occur at or near the macula and have no known association with changes in the surrounding neurosensory retina, retinal pigment epithelium, or choroid, nor have they been related with exudation or hemorrhage. Until now, there have been no cases reported of CSHRPE with associated vascular activity. CASE REPORT: A 14-year-old Hispanic adolescent girl with a presumed CSHRPE presented with adjacent macular edema. The patient was treated with an off-label intravitreal bevacizumab injection to decrease the edema. Two months later, the patient presented with improved visual acuity and decreased macular edema. CONCLUSIONS: This is the first documented case of macular edema secondary to vascular activity being associated with these rare lesions. When CSHRPE is found to have secondary vascular activity and associated macula edema, treatment of intravitreal bevacizumab may lead to improved visual and anatomical outcomes.

Tapioca melanoma of the iris without iris heterochromia.

PURPOSE: A case of a teenage girl with tapioca melanoma of the iris is presented. This case is unusual, as the patient did not have heterochromia and did not present with elevated intraocular pressure. CASE REPORT: A 14-year-old female patient presented with an amelanotic, multinodular, multifocal lesion of the right iris. Pathology confirmed a diagnosis of tapioca melanoma using immunohistologic staining. The patient underwent enucleation of her right eye and has been free of metastatic disease 3 years later. CONCLUSIONS: Tapioca melanoma of the iris must be included among the other differential diagnoses when examining patients with amelanotic iris lesions, even when iris heterochromia is not clearly evident.

Posterior uveal melanoma coexistent with macular hole.

PURPOSE: Three rare cases of macular holes coexistent with posterior uveal melanoma are presented. The possible pathogenesis of a macular hole secondary to uveal melanoma growth is discussed, as well as strategies to treat uveal melanoma before attempting macular hole repair. CASE REPORT: Each patient from all three cases had a macular hole and uveal melanoma at the initial presentation. The macular holes were present before uveal melanoma treatment in cases 1 and 2. The patient in case 3 had already been treated elsewhere with iodine-125 plaque brachytherapy for her melanoma. It was not known if macular hole formation occurred before or after that treatment. Macular hole repair was performed in case 1, and the hole was successfully closed. CONCLUSIONS: To the best of the authors' knowledge, there are now only nine documented cases of macular holes accompanying posterior uveal melanoma. Successful macular hole repair may prove difficult because the melanoma must first be properly treated and then monitored carefully to establish tumor inactivity.

Phacovitrectomy.

Recent advances in vitreoretinal surgical technology combined with advances in small-incision cataract surgery have enabled the ophthalmic surgeon to provide combined management of cataract and retinal pathology. Progressive understanding of the inevitability of cataract progression after pars plana vitrectomy along with the negative impact of early cataract progression on visual acuity outcomes have defined the importance of combined cataract surgery and pars plana vitrectomy in improving surgical outcomes. Combining phacoemulsification with intraocular lens placement with vitrectomy has now been shown to decrease visual rehabilitation time in patients with early/visually significant cataracts. These benefits are especially apparent when complex ocular pathology is present and clear visualization during surgery is needed. This report focuses on specific factors pertaining to combined surgery that include patient selection, mechanics of phacovitrectomy, and potential complications. The authors report that utilization of combined phacovitrectomy at their institution has had an outstanding success rate with 95% of the patients achieving 2-line or greater improvements in visual acuity within 6 weeks of surgery. Combined phacovitrectomy is associated with a significant reduction in overall healthcare costs that may become more important in this era of healthcare reform. Finally, rapid adoption of combined phacovitrectomy has occurred internationally, but remains delayed at most institutions in the USA.

Spontaneous exudative retinal detachment in a patient with sturge-weber syndrome after taking arginine, a supplement for erectile dysfunction.

BACKGROUND: Patients with Sturge-Weber syndrome can have ipsilateral diffuse or circumscribed choroidal hemangiomas. These hemangiomas have been seen to undergo spontaneous exudative or hemorrhagic retinal detachments. There is no definitive treatment for these types of retinal detachments, but radiotherapy, photodynamic therapy, oral propranolol, pegaptinib and bevacizumab have been used. CASE PRESENTATION: A 26-year-old male with Sturge-Weber Syndrome developed an exudative retinal detachment that occurred immediately after taking a supplement containing arginine. The patient was treated with intravitreal bevacizumab 1.25 mg in 0.05 ml solution. Resolution of the retinal detachment was seen after 4 treatments over a six-month period. CONCLUSIONS: Arginine and other medications that cause a release of nitric oxide may lead to intravascular leakage and exudative retinal detachments in patients who have a choroidal hemangioma.