This month, “Clinical Communications,” under the clinical editorship of Editorial Board member Andrew B. Mick, OD, FAAO, includes color image content and optional supplementary digital content, with no cost to our authors.
Each article in this section is followed by a clinical comment by our clinical editor, emphasizing the clinical take-home message. We provide these “Clinical Pearls” of the publication following the article’s title, authors, and abstract. The full text and illustrations are found online with the colored images and any video clips.
Dr. Andrew B. Mick provides all of the clinical comments (“Clinical Pearls”) on each article in “Clinical Communications.” This is all part of our OVS program to enrich the clinical content of OVS and make it very accessible, in color and motion, to clinicians, readers, and authors alike.
Andrew B. Mick, OD, FAAO
Clinical Topical Editor
Gyrate Atrophy–Like Phenotype: Normal Plasma Ornithine and Retinal Crystals
Raman Bhakhri and William H. Ridder, 3rd
Purpose. To report an unusual case of a gyrate atrophy–like presentation with retinal crystal deposition in a patient with normal plasma ornithine levels.
Case Report. A 50-year-old Hispanic female patient presented with complaints of blurred vision and nyctalopia. Examination revealed bilateral multiple round islands of peripheral chorioretinal degeneration in addition to small crystal-like deposits in the posterior pole. Spectral domain optical coherence tomography confirmed the crystalline deposits to be above the retinal pigment epithelium. Electrophysiology revealed reduced photopic responses with no recordable scotopic response. Testing for elevated plasma ornithine, which is typical in gyrate atrophy patients, was performed; however, the patient’s levels were normal.
Conclusions. Diagnosis of conditions that cause nyctalopia can be challenging because they are rare and often similar in appearance and presenting symptoms. Retinal crystal deposition and normal plasma ornithine illustrate the phenotypical variation that can be seen in a gyrate atrophy–like phenotype.
Terson’s Syndrome in a Patient with von Hippel-Lindau Disease
Luis A. Acaba, Megan L. Chenworth, Aaron S. Gold, Andrea C. Wildner, Fiona J. Ehlies, Audina M. Berrocal, and Timothy G. Murray
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. ATerson’s event may be caused by high intracranial pressure secondary to the surgical removal of von Hippel-Lindau syndromeYassociated cerebellar tumors and should be included as a possible complication of surgical management.