Lawrence Y Ho

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.

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.

Surgical outcomes of idiopathic macular hole repair with limited postoperative positioning.

Surgical repair of idiopathic macular holes was first described in 1991 by Kelly and Wendel. Patients in their study underwent pars plana vitrectomy, gas– fluid exchange, and postoperative face down patient positioning for 7 days to 10 days. Since then, this postoperative positioning period has been adopted by many retinal surgeons. However, there have been multiple complications reported secondary to extended postoperative positioning: neck or back pain, pressure sores, ulnar neuropathy, and time lost from work. In recent years, internal limiting membrane (ILM) peeling has been thought to relieve tangential traction on the macula and improve surgical outcomes. The improved success rate of macular hole surgery has led some retinal surgeons to question the need for prolonged postoperative face down positioning. Several studies have suggested that the use of ILM peeling and a shorter postoperative positioning or no postoperative positioning may be adequate in achieving equivalent surgical outcomes. There have also been reports showing postoperative macular hole closure on ocular coherence tomography (OCT) by postoperative Day 2 or Day 3, further challenging the need for postoperative positioning beyond that period. To provide evidence in support of limited postoperative positioning, we present the outcomes of idiopathic macular hole repair at our institution, using ILM peeling, followed by 1 day of face down positioning.

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 others 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.

Retinal Pigment Epithelial Tear in Shaken Baby Syndrome

R etinal hemorrhages are the most commonly reported ocular findings in shaken baby syndrome (SBS) in children. The intraocular hemorrhages can vary considerably not only in size and severity but also in location. Shaken baby syndrome can also cause several structural retinal abnormalities including perimacular folds, traumatic retinoschisis, vitreomacular traction, retinal pigmentary changes, and macular holes. Although these observations have been well documented in the literature, retinal pigment epithelial (RPE) tears have not been described in the setting of SBS.

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.

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.