Clinical presentation of familial exudative vitreoretinopathy.

OBJECTIVE: To describe the clinical characteristics, staging and presentation of patients with familial exudative vitreoretinopathy (FEVR) in our clinical practice over the last 25 years. DESIGN: Case series, retrospective review. PARTICIPANTS: We included 273 eyes of 145 patients. METHODS: Data collected from charts included gender, gestational age at birth, birthweight, age at presentation, referring diagnosis, family history, prior ocular surgery, and clinical presentation in each eye. Eyes with invasive posterior segment procedures before initial presentation were excluded. MAIN OUTCOME MEASURES: Demographics on presentation and clinical staging. RESULTS: Patients were slightly male predominant (57%) with a mean birthweight of 2.80 kg (range, 740 g-4.76 kg), mean gestational age of 37.8 weeks (range, 25-42), and mean age at presentation of almost 6 years (range, <1 month-49 years). A positive family history of FEVR was obtained in 18% of patients. A positive family history for ocular disease consistent with but not diagnosed as FEVR was obtained in an additional 19%. Stage 1 FEVR was identified in 45 eyes, stage 2 in 33 eyes, stage 3 in 42 eyes, stage 4 in 89 eyes, and stage 5 in 44 eyes. Radial retinal folds were seen in 77 eyes, 64 of which were temporal or inferotemporal in location. CONCLUSIONS: The FEVR patient population is remarkable for the wide range of age at presentation, gestational age, and birthweight. Although a positive family history on presentation may support the diagnosis of FEVR, a negative family history is of little help. The majority of retinal folds extended radially in the temporal quadrants, but radial folds were seen in almost all quadrants. Fellow eyes demonstrated a wide variation in symmetry. The presentation of FEVR may mimic the presentation of other pediatric and adult vitreoretinal disorders, and careful examination is often crucial in making the diagnosis of FEVR. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any of the materials discussed in this article.

Study of intraocular pressure after 23-gauge and 25-gauge pars plana vitrectomy randomized to fluid versus air fill.

PURPOSE: To determine if a difference in intraocular pressure (IOP) exists after small-gauge pars plana vitrectomy randomized to fluid versus 80% sterile air fill. METHODS: Ninety-two eyes undergoing 23-gauge and 25-gauge transconjunctival pars plana vitrectomy were randomized to fluid versus air fill, and IOP was measured at 5 different time points. RESULTS: There is no difference in the mean IOP for eyes undergoing small-gauge pars plana vitrectomy randomized to fluid versus air fill after vitrectomy. Using 23-gauge instrumentation, the mean immediate and 2-hour postoperative IOPs were statistically lower than the mean preoperative IOP. The mean Postoperative Day 1 and Week 1 IOPs were statistically higher than the mean immediate postoperative IOP. Using 25-gauge instrumentation, the mean IOP was not statistically different at any time points in the 2 groups. When comparing 23-gauge with 25-gauge instrumentation, the immediate postoperative IOP was statistically lower and the rate of suture closure for sclerotomies was higher for 23-gauge vitrectomy. CONCLUSION: When performing 23-gauge or 25-gauge pars plana vitrectomy, there was no difference in mean IOP for fluid- versus air-filled eyes. However, the mean IOP in the immediate postoperative period was statistically lower, and there was a higher rate of suture closure for 23-gauge compared with 25-gauge instrumentation.

Management of avascular peripheral retina in older prematurely born infants.

PURPOSE: To report on the authors' experience with presentation and management of prematurely born infants who do not fully vascularize after 45 weeks of postmenstrual age (PMA). METHODS: Retrospective, noncomparative, consecutive case series consisting of premature infants with retinopathy of prematurity (ROP) who were treated with laser ablation after 45 weeks of PMA at a single tertiary referral institution. RESULTS: A total of 370 premature infants with ROP were treated at this institution from January 1, 2006, to August 1, 2009. Eighteen eyes of 9 patients received laser photocoagulation after 45 weeks of PMA and were included in this series. The mean gestational age for this subgroup was 26 weeks and 2 days, and mean birth weight was 792 g. Type 1 ROP developed in 3 infants after 45 weeks of PMA and received laser treatment, and 6 infants without Type 1 ROP received laser treatment for an avascular peripheral retina, evidence of peripheral neovascularization, and/or ROP in Zone 3 with persistent plus disease. CONCLUSION: This series represents the authors' experience in the management of prematurely born infants with ROP after 45 weeks of PMA. One may consider close observation or laser ablation for the management of infants who have incomplete peripheral retinal vascularization, peripheral neovascularization, and/or ROP in Zone 3 with persistent plus disease.

Endogenous methicillin-resistant Staphylococcus aureus endophthalmitis.

PURPOSE: To report on the presentation, treatment, and outcomes of the largest series to date for patients with endogenous methicillin-resistant Staphylococcus aureus endophthalmitis at a single institution and compare the results from previously published reports of this disease entity. METHODS: Retrospective, noncomparative, consecutive case series of patients who were treated for endogenous methicillin-resistant S. aureus endophthalmitis from a single tertiary referral institution were reviewed. RESULTS: Eight eyes of seven consecutive patients, with a mean age of 58.1 years, were diagnosed with culture-proven endogenous methicillin-resistant S. aureus endophthalmitis. Five patients were male, and six patients had a unilateral presentation. Five of eight eyes were treated with initial vitreous tap and injection of antibiotics, and six eyes showed a retinal detachment during the course of treatment. Final visual acuity ranged from 20/40 to no light perception, and 5 of 8 eyes showed improvement in visual acuity from initial presentation. Only one eye became blind and painful and required enucleation. CONCLUSION: This series represents the largest single-institution report on endogenous methicillin-resistant S. aureus endophthalmitis and associated outcomes. Although previously reported cases cited poor visual outcomes and high enucleation rates, the authors' current series shows that retinal detachments are common and enucleation rates are low.

Intrauterine injury simulating abusive head trauma.

PURPOSE: The purpose of this study was to describe a patient with intrauterine injury simulating abusive head trauma and its management. METHODS: A case report of a premature infant born at 35 weeks 2 days through an uncomplicated vaginal delivery was found to have cerebral hemorrhages on magnetic resonance imaging after presenting with respiratory distress. The infant was referred to us for bilateral subconjunctival hemorrhages and periorbital edema. We performed serial ophthalmologic examinations, reviewed all prenatal history with the mother of the infant, and reviewed all relevant hospital notes, laboratory results, and imaging results. RESULTS: On ophthalmologic examination, the patient was found to have vitreous and retinal hemorrhages bilaterally. Prenatal history showed intrauterine trauma inflicted by the mother in the setting of an atraumatic delivery. The patient required bilateral vitrectomies to prevent amblyopia. CONCLUSION: Intrauterine injury may simulate abusive head trauma, and it is important for clinicians to recognize this presentation to prevent further harm to the infant and prevent devastating visual loss.

Intravitreal bevacizumab and ranibizumab for choroidal neovascularization secondary to endogenous endophthalmitis.

PURPOSE: To report two patients with endogenous endophthalmitis complicated by choroidal neovascularization managed with intravitreal bevacizumab (Avastin, Genentech, Inc., South San Francisco, CA) or ranibizumab (Lucentis, Genentech, Inc., South San Francisco, CA) and their respective outcomes. METHODS: Retrospective chart review of 2 patients, one a 54-year-old diabetic woman on chemotherapy for pemphigus vulgaris and the other a 77-year-old diabetic male with cellulitis, who were treated for endogenous endophthalmitis complicated by choroidal neovascularization. RESULTS: The patients were managed with off-label intravitreal anti-vascular endothelial growth factor injections. The first patient received one dose of intravitreal bevacizumab (Avastin) with improvement in vision and anatomy. The second patient received four doses of intravitreal ranibizumab (Lucentis) with improvement in vision and structural appearance. CONCLUSION: Choroidal neovascularization secondary to endogenous endophthalmitis responds to anti-vascular endothelial growth factor therapy with bevacizumab or ranibizumab.

25-Gauge pars plana vitrectomy for retained lens fragments.

PURPOSE: The purpose of this study was to report the outcomes and complications of 25-gauge pars plana vitrectomy for the management of retained lens fragments after complicated cataract surgery. METHODS: This is a retrospective consecutive case series of 17 patients who presented with retained lens fragments, ranging from mostly cortex to the entire lens, which were managed using only 25-gauge instrumentation. RESULTS: Eight men and 9 women (mean age, 73.1 years) were followed for a mean of 4.5 months after 25-gauge vitrectomy for removal of retained lens fragments. Mean preoperative visual acuity was 20/427 and mean final postoperative visual acuity was 20/62. Mean surgical operating time was 48.5 minutes. Mean preoperative intraocular pressure was 24.5 mmHg and mean 1-day postoperative intraocular pressure was 17.9 mmHg. No cases required the use of a phacofragmatome, no sutures were required to close the sclerotomies, and there were no cases of postoperative hypotony. Cystoid macular edema and glaucoma developed postoperatively in 29.4% and 5.9% of eyes, respectively. There were no cases of postoperative retinal detachment or endophthalmitis. CONCLUSION: A 25-gauge vitrectomy technique, without the use of a phacofragmatome, may be a suitable alternative to 20-gauge vitreous surgery using a phacofragmatome in the management of retained lens fragments after complicated cataract surgery. Clinical outcomes and complication rates are comparable to those found in the literature for 20-gauge surgery.

Microcornea, posterior megalolenticonus, persistent fetal vasculature, and coloboma: a new syndrome.

PURPOSE: To report a newly identified syndrome of bilateral microcornea, posterior megalolenticonus, persistent fetal vasculature, and chorioretinal coloboma (MPPC). DESIGN: Noncomparative case series. PARTICIPANTS: Eight patients with MPPC syndrome. METHODS: Clinical data collected retrospectively included visual acuity, findings on office examination as well as examination under anesthesia, and, in some cases, fluorescein angiography. Intraoperative findings and postoperative visual acuity and clinical findings were recorded when surgical intervention was performed. MAIN OUTCOME MEASURES: Clinical description, intraoperative findings, and surgical outcomes. RESULTS: All patients were found to have microcornea with corneal diameters of less than 8 mm. In all cases, the crystalline lens was found to be retrodisplaced with massive enlargement and a dramatic posterior lenticonus (posterior megalolenticonus), and the ciliary processes frequently were drawn to the lens capsule. A stalk of persistent fetal vascular tissue extended from the posterior pole of the lens to the optic disc. Posterior chorioretinal coloboma was present in all cases. Some cases also exhibited grossly dysplastic retina. Presentation frequently was asymmetric. Eight eyes of 6 patients underwent lensectomy, vitrectomy, membrane peeling, and fluid-Healon exchange (Healon OVD [sodium hyaluronate]; Advanced Medical Optics, Santa Ana, CA) with functional vision in at least 4 of the 5 patients with postsurgical follow-up. CONCLUSIONS: Bilateral microcornea, posterior megalolenticonus, persistent fetal vasculature, and chorioretinal coloboma syndrome is a distinct syndrome previously unreported, to the authors' knowledge, and appropriate surgical intervention may result in significantly improved visual function.

Comparison of Rosenbaum pupillometry card using red and blue light to Colvard and Iowa pupillometers.

PURPOSE: To compare four scotopic pupil measuring techniques to determine whether a statistically significant difference exists among the techniques for each observer or between the observers for each technique. METHODS: Cross-sectional study performed on 200 eyes of 100 healthy individuals. Two observers performed pupillometry four times per pupil using the Rosenbaum card with red light, Rosenbaum card with blue light, Iowa pupillometer, and Colvard pupillometer. All measurements were recorded in scotopic conditions with each examiner masked to the other's results. RESULTS: Both examiners measured mean pupil diameters > 6.0 mm for all modalities except the Colvard device whose mean measurements were the smallest. The Iowa pupillometer and Rosenbaum card with red light produced the largest pupil measurements and were the most similar for both observers. The mean pupil diameter obtained by observer 1 was smaller compared with observer 2 while using the Rosenbaum card with blue light, but this was not significant (P = .2574). The mean pupil diameter obtained by observer 1 was larger compared with observer 2 while using the Colvard pupillometer and this was statistically different after adjusting for eye color (P = .0370). The mean pupil diameters of blue irides and brown irides are significantly higher than the measurements obtained for hazel irides (P = .0271 and P = .0445, respectively). CONCLUSIONS: Due to the complex interaction among observer, pupillometry technique, and iris color, one cannot compare the four techniques to each other with the same observer, nor can one compare the two observers irrespective of the technique.

Clinical predictors and outcomes of pars plana vitrectomy for retained lens material after cataract extraction.

PURPOSE: To determine current clinical predictors and visual outcomes of patients who undergo pars plana vitrectomy (PPV) for retained lens material. DESIGN: Retrospective, noncomparative, consecutive case series. METHODS: Medical records of 166 patients with complicated cataract surgery who underwent PPV for retained lens material at Retina Vitreous Consultants of Pittsburgh between January 1, 2001 and January 1, 2007 were reviewed. Main outcome measures include final visual acuity, development of glaucoma, retinal detachments, and cystoid macular edema. RESULTS: At the final examination, 120 patients (72.3%) had a visual outcome of 20/40 or better and 18 patients (10.8%) had a visual outcome of 20/200 or worse. In patients without any preoperative eye conditions, 82.6% achieved a final vision of 20/40 or better. Multivariable analysis showed that predictors for visual outcomes of 20/40 or better were better presenting vision (P = .001), insertion of a posterior chamber lens (P = .005), and absence of preoperative eye disease (P = .001). Predictors for visual outcomes of 20/200 or worse were the absence of an anterior vitrectomy at cataract surgery (P = .005), absence of a sulcus lens (P = .011), presence of preexisting eye disease (P = .02), and development of glaucoma (P = .001). Performing a PPV within 7 days of cataract surgery was associated with a lower risk of developing glaucoma (P = .005). CONCLUSIONS: Current techniques for management of retained lens material may contribute to improved visual outcomes based on our series. We recommend that the cataract surgeon perform an anterior vitrectomy, place a posterior chamber lens if possible, and consult a retina specialist for a PPV within 7 days to decrease the risk of developing secondary glaucoma.

Effect of audible and visual reminders on adherence in glaucoma patients using a commercially available dosing aid.

We studied the effects of audible and visual alarms on adherence with a recommended dosing regimen in the management of glaucoma. Forty-two patients were begun on therapy with the Travatan((R)) Dosing Aid (TDA) and randomly divided into two observation groups - one with visual and audible alarm functions turned on and the other with alarms off. Dosing information was analyzed for mean rates of adherence, missed days, and dosing at the wrong time. Twenty patients were randomized to the TDA alarm on group and 22 to the alarm off group. The rates of adherence were 87.9% and 79.7% (p = 0.02), rates of missed dosing were 7.6% and 14.4% (p = 0.03), and rates of dosing at the incorrect times were 7.1% and 9.8% (p = 0.19), respectively for alarm on versus alarm off groups. In the alarm on group, the adherence rate was significantly higher and proportion of missed dosing was significantly lower. It is still yet to be determined whether there is a relationship between adherence and progression of glaucoma.

Valsalva retinopathy associated with the choking game.

A 41-year-old man with high myopia presented with a central scotoma in the left eye. Initial visual acuity in the left eye was count fingers. Color fundus photos, fluorescein/indocyanine green angiography, and optical coherence tomography of the macula were performed showing intraretinal and subretinal hemorrhage over the macula. The patient admitted to developing acute vision loss after holding his breath and blowing against a closed glottis to obtain a euphoric sensation. It is extremely important to recognize this possible cause of Valsalva retinopathy due to the potentially life-threatening outcome of this game.