Uday R. Desai

Pigmentary maculopathy in a patient with Wolfram syndrome

CASE REPORT We describe a rare association of pigmentary maculopathy with Wolfram syndrome not previously reported in the literature. A 12-year-old boy presented to the retina service with a diagnosis of Wolfram syndrome and a history of poor central vision. The patient was found to have bilateral atrophic pigmentary maculopathy that was confirmed with fluorescein angiography. COMMENTS Wolfram syndrome may present with a pigmentary maculopathy and this rare finding may assist the clinician in making appropriate genetic referral when this diagnosis is suspected.

Visual prognosis in patients with traumatic choroidal rupture

BACKGROUND There is a paucity of work in the literature examining the long-term visual prognosis of patients with choroidal ruptures. We performed a study to get a better assessment of long-term visual recovery in these patients as well as to identify prognostic indicators. METHODS We reviewed the charts of 32 patients who experienced traumatic choroidal rupture following blunt ocular trauma. All charts contained International Classification of Diseases code 363.63 (choroidal rupture). For inclusion in the study, the chart had to contain detailed drawings or photographic evidence that could confirm the presence and location (foveal, juxtafoveal or extrafoveal) of the choroidal rupture. In addition, demographic data and visual acuity on follow-up examinations had to be present. The mechanism and location of the injury, initial and final visual acuity, associated ocular findings and length of follow-up were recorded. RESULTS Of the 32 eyes 31 had indirect choroidal ruptures and I had a direct rupture. The mean final visual acuity values in the foveal, juxtafoveal and extrafoveal subgroups were 20/68, 20/35 and 20/60 respectively after a mean duration of follow-up of 4.5, 3.5 and 4.4 years respectively. There was no statistically significant difference in mean final visual acuity or mean length of follow-up between the three groups. The eight patients with multiple choroidal ruptures had a mean final vision of 20/31 after a mean follow-up period of 3.8 years. The 24 patients with a single choroidal rupture achieved a mean final vision of 20/47 over a mean duration of follow-up of 4.1 years. There was no difference in final vision or in length of follow-up between the two groups. The six patients under 15 years of age attained a mean final vision of 20/34 over a mean follow-up period of 4.5 years, whereas the adult group achieved a mean final vision of 20/44 over a mean follow-up period of 3.8 years. Again, there was no difference in final vision or in length of follow-up between the two groups. INTERPRETATION Traumatic choroidal rupture involving the fovea has been thought to have a poor visual prognosis. Our findings show that eyes with foveal choroidal ruptures may regain good central vision over longer follow-up. Furthermore, multiple choroidal ruptures do not necessarily predict a poor visual outcome. Children with choroidal ruptures attained good final visual outcomes.

Incidence of ocular injuries in motor vehicle crash victims with concomitant air bag deployment

OBJECTIVE To report the incidence of ocular and nonocular trauma in patients admitted to the Henry Ford Hospital via the emergency room since 1994 after a motor vehicle crash (MVC) with and without air bag deployment. DESIGN A retrospective cohort study. METHODS The Henry Ford Hospital Trauma Registry database was reviewed for patients involved in MVCs with and without air bag deployment since 1994. RESULTS From 1994 to 1999, there were only seven air bag-related ocular injuries, representing 4.4% of all MVC-related ocular injuries. From 1997 to 1999, MVC-related ocular injuries with and without air bags represented 5.0% and 12.7%, respectively, of all MVC-related injuries. For that same period, the death rate and average Injury Severity Score for MVCs with air bag deployment were 3.4% and 10.75, compared with 8% and 14.5, respectively, for MVCs without air bag deployment. CONCLUSIONS MVC-related ocular injuries associated with air bag deployment are rare, and the incidence of ocular injuries associated with MVCs was lower when air bags were deployed.

Bilateral optic pit-like maculopathy with normal optic nerve heads.

had improved slightly to 20/100. Upon OCT, the central macular thickness had decreased to 165 mm OD and 224 mm OS, indicative of a normalized central macular thickness. The cystic maculopathy and retinoschisis of the parafoveal area had also improved (Figs. 2C, D). OCT is a new imaging modality that provides highresolution, cross-sectional images of the macular region and is helpful in diagnosing and monitoring the clinical course of XLRS and determining the treatment response. Recently, treatments with carbonic anhydrase inhibitors and vitrectomy have been reported to improve or resolve foveal retinoschisis in patients with XLRS. Ghajania and Gorin administered acetazolamide orally to a patient with cystic maculopathy. There was nearly complete resolution of the cystoid spaces in both eyes evident onOCT after 9 months. Apushkin and Fishman and Bastos et al. used topical dorzolamide and confirmed the disappearance of foveal cysts upon OCT. These 2 studies, however, were short term and could not assess whether the lesions recurred over the long term. Recently, Ikeda et al. performed vitrectomies in 5 cases of XLRS. In 4 cases, retinoschisis improved after the initial surgery, whereas a second procedure was required in the remaining case. To our knowledge, this is the first case of spontaneous resolution of retinoschisis in XLRS as observed by OCT. We conclude that, although rare, foveal schisis may improve during the natural course of the illness, as shown in our case. Accordingly, the clinical course of such cases should be monitored with OCT before drug therapy or surgery is considered.

ROLLER COASTER RETINOPATHY: CASE REPORT OF SYMPTOMATIC BILATERAL INTRARETINAL HEMORRHAGES AFTER SHAKING INJURY IN AN OTHERWISE HEALTHY ADULT.

An otherwise healthy young woman develops a symptomatic maculopathy after riding multiple roller coasters at an amusement park. The mechanism is thought to involve gravitational forces acting on the vitreoretinal interface. This may be similar to abusive head trauma seen in children, and further investigation into the interplay between these types of recreational activities and the eye are warranted.

First Described Case of Anterior and Posterior Segment Crystals in Phacolytic Glaucoma

Phacolytic glaucoma is an open-angle glaucoma that occurs when lens proteins from hypermature cataracts seep through an intact anterior capsule and induce obstruction of the trabecular meshwork by inflammatory cells. We review the case of a 66-year-old man who presented with acute pain, a hypermature cataract, prominent anterior chamber crystals, and elevated intraocular pressure. After cataract surgery was performed, iridescent crystals were noted in the posterior chamber. Anterior chamber crystals have been associated with phacolytic glaucoma, but this is the first case demonstrating crystals in the posterior chamber as well.

Crystalline lens dislocation secondary to bacterial endogenous endophthalmitis

Purpose To present an unusual case of endogenous endophthalmitis secondary to Group A streptococcus (GAS) that resulted in dislocation of the crystalline lens. Observations An immunocompetent 51-year-old man presented to the emergency room (ER) with upper respiratory infection (URI) symptoms and painful right eye. He was diagnosed with URI and viral conjunctivitis and discharged on oral azithromycin and polytrim eyedrops. He returned to the ER 30 h later with sepsis and findings consistent with endophthalmitis, including light perception only vision. Ophthalmology was consulted at this time and an emergent vitreous tap and injection was performed. Both blood and vitreous cultures grew an atypical non-hemolytic variant of GAS (Streptococcus pyogenes). The primary source of infection was presumed to be secondary to pharyngitis or cutaneous dissemination. Final vision in the affected eye was no light perception, likely from a combination of anterior segment scarring, posterior segment damage, and hypotony. Interestingly, head computed tomography (CT) at the initial ER presentation showed normal lens position, but repeat CT at re-presentation revealed posterior dislocation of the lens. Conclusions and importance Endophthalmitis secondary to GAS has been sparsely reported in the literature, and this case highlights a unique clinical presentation. We suspect that this atypical non-hemolytic strain may have evaded detection on initial pharyngeal cultures. Additionally, we hypothesize that GAS-mediated protease release resulted in breakdown of the zonular fibers and subsequent lens dislocation. Ophthalmologists should be aware of GAS and its devastating intraocular manifestations.

Vogt-Koyanagi-Harada Syndrome in a Healthy African-American Woman: Case Report and Review of Literature

A previously healthy 18 year-old African-American female presented with unilateral vision loss associated with headaches. Initial ocular examination revealed an exudative retinal detachment involving the macula, disc edema, and choroidal striae worse in the right than the left eye. Fluorescein angiography showed multiple punctate hyperfluorescent lesions with leakage into the subretinal space. Extensive work up led to the diagnosis of Vogt- Koyanagi-Harada syndrome. The patient was treated with a prolonged taper of systemic prednisone and approximately 4 months after presentation, vision improved to 20/20 in the right eye and 20/25 in the left eye.

Surgical technique for removal of a capsular tension ring from the vitreous cavity.

PURPOSE To describe a surgical technique for removing a posteriorly dislocated capsular tension ring (CTR). METHODS Retrospective interventional case report. CASE REPORT A 68-year-old African American man underwent cataract surgery. Early in the procedure, a CTR was placed at the equator of the lens capsule. Subsequently, the posterior capsule ruptured. The CTR was left in place, and an intraocular lens was placed in the sulcus. On postoperative Day 6, the CTR subluxated into the vitreous cavity and was removed without complication by pars plana vitrectomy, using the CTR inserter. CONCLUSION The technique of using the CTR inserter to remove the CTR from the vitreous cavity appears to be safe and effective.

Does Postoperative Near Visual Acuity Predict Macular Hole Closure

To determine whether prone postoperative near visual acuity following macular hole surgery can be used as a reliable indicator of successful hole closure, data from 21 patients undergoing macular hole surgery were collected. Seventeen of the 18 patients with hole closure and all 3 patients with persistent macular holes had a Rosenbaum acuity better than preoperative visual acuity, yielding 94% sensitivity, 0% specificity, 85% positive predictive value, and 0% negative predictive value. Fourteen of the 18 patients with macular hole closure and all 3 patients with persistent macular holes had a Rosenbaum acuity better than 20/40, yielding 78% sensitivity, 0% specificity, 82% positive predictive value, and 0% negative predictive value. Although postoperative near visual acuity can predict macular hole closure with 94% sensitivity, the test is not clinically useful to predict hole closure because of the high surgical success rate of macular hole surgery. The test could be useful in encouraging patients to maintain head prone positioning and alleviate patient anxiety.