NOVEL SURGICAL TECHNIQUE FOR INDUCING POSTERIOR VITREOUS DETACHMENT DURING PARS PLANA VITRECTOMY FOR PEDIATRIC PATIENTS USING A FLEXIBLE LOOP

Vitreoretinal surgical diseases in children are challenging because of the complex features of the vitreous, retina, and the interface between them. Posterior vitreous detachment is a crucial step during vitrectomy. To date, pharmacologic vitreolysis that liquefies the vitreous and disrupts the posterior hyaloid attachment to the retina has been studied. However, there are reports of unexplained adverse effects. We describe a case performed with a reproducible, safe and time-saving technique using a commercially available flexible loop for posterior vitreous detachment induction in a pediatric patient. A 10-year-old boy with previously treated retinopathy of prematurity undergoes a 25-gauge pars plana vitrectomy for retina detachment repair. To achieve a posterior vitreous detachment, a flexible loop was used to grasp and pick the vitreoretinal interface around the optic nerve for dissection of the posterior hyaloid, followed by aspiration and cutting with the vitrectomy probe to complete the vitrectomy. At 3 months follow-up, total retinal reattachment was observed. The patient's vision improved from 20/400 to 20/150. Posterior vitreous detachment induction with a flexible loop instead of pharmacologic vitreolysis seems a promising tool as demonstrated in this clinical case. Further studies to demonstrate long-term safety and anatomical results are needed

Imaging Modalities in Pediatric Vitreoretinal Disorders

This review updates current applications of novel imaging technologies to pediatric vitreoretinal disorders.Ultra-widefield technology has revolutionized retinal imaging in pediatric patients. Color imaging, fluorescein angiography, autofluorescence, and optical coherence tomograpy (OCT) have all been adapted to capture the anterior segment and the posterior pole in children, and serve as valuable tools in diagnosis, management, and documentation of disease processes over time. The role of intraoperative OCT and 3D heads-up visualization technologies continues to evolve with improvement in intraoperative visualization and surgical outcomes.New imaging technologies have the ability to improve our understanding and management of pediatric vitreoretinal disorders

Management of Retinopathy of Prematurity--Use of Anti-VEGF Therapy

Retinopathy of prematurity (ROP) is a leading cause of childhood blindness worldwide. Although laser photocoagulation re-mains the gold standard for treating threshold and prethreshold disease (type 1 ROP), the off-label use of anti‒vascular endothelial growth factor (anti-VEGF) therapy to treat ROP is increasing. Benefits include acute regression of ROP, growth of retinal vasculature beyond the demarcation line, lesser degree of myopia and peripheral visual field loss, and avoidance of sedation and intubation required for laser. However, controversies regarding anti-VEGF in this vulnerable population persist including choice of anti-VEGF agent, dosing, systemic absorption, safety, and late recurrence. This review updates recent evidence regarding the use of anti-VEGF therapy in the management of ROP

Leber Congenital Amaurosis With Coats-Like Reaction and 3 Novel Sequence Variants: A Short Report

Purpose: This report discusses a patient with Leber congenital amaurosis who presented with severe bilateral Coats-like reaction and 3 novel sequence variants in 2 different genes. Methods: The patient underwent complete ophthalmic and retinal evaluations including fluorescein angiography and optical coherence tomography as well as genetic studies. She was treated with intravitreal injection of intravitreal bevacizumab and laser photocoagulation. Results: Genetic analysis of the patient identified 2 novel sequence variants in the CEP290 (centrosomal protein 290) gene and another novel sequence variant in the RPGRIP1 (RPGR interacting protein 1) gene. Conclusions: The clinical presentation of Coats-like reaction in Leber congenital amaurosis can be associated with sequence variants in certain genes. The presence of multiple gene sequence variants in this patient may have contributed to the severity of disease

Retinoschisis in Coats Disease: Clinical Picture, Therapeutic Considerations, and Management Outcomes

Purpose: Retinoschisis in inflammatory and exudative retinopathy is a known but rare entity. We describe the presentation and clinical and surgical outcomes of a series of patients with retinoschisis in the setting of Coats disease. Methods: This retrospective case series of patients with Coats disease recorded visual acuity and the number and type of treatments (eg, angiography-guided photocoagulation, intravitreal bevacizumab, sub-Tenon triamcinolone, or vitreoretinal surgery). Results: Eighteen of 133 patients with Coats disease were diagnosed with retinoschisis by color imaging. All (100%) of the areas of schisis or macrocyst were associated with dense subretinal exudation, whereas only 7 (38.8%) were associated with exudative retinal detachment. Initial best-corrected visual acuity in the schisis cohort was 1.14 ± 1.19 (Snellen equivalent, 20/276) vs 1.42 ± 1.40 (Snellen, 20/526) in those without ( P = .56). Final best-corrected visual acuity was 1.76 ± 1.37 (Snellen, 20/1150) and 1.45 ± 1.44 (Snellen, 20/563), respectively ( P = .43). The mean number of treatments in individuals with schisis was 4.8 ± 2.9 for angiography-guided photocoagulation, 4.5 ± 2.9 for intravitreal bevacizumab, and 1.7±1.2 for sub-Tenon triamcinolone. Intraocular surgery was required in 39% (7 of 18) of patients with schisis vs 22% (25 of 115) for patients without schisis ( P = .14). One eye required enucleation, and histopathology of the schisis pocket was obtained. Conclusions: The pathophysiology of retinoschisis in Coats disease is not completely understood. We propose that telangiectatic leakage, hypoxia, and toxicity to the retinal pigment epithelial cells play a role in cavity development, and therefore may represent a clinical feature of advanced or uncontrolled disease

Revascularization After Intravitreal Bevacizumab and Laser Therapy of Bilateral Retinal Vascular Occlusions in Incontinentia Pigmenti (Bloch-Sulzberger Syndrome)

No consensus exists for the treatment of retinopathy in incontinentia pigmenti (IP). Vascular ischemia leads to tractional retinal detachments if untreated. Ultra-widefield fluorescein angiography (FA) is used to follow the vascular status of the retina. A 13-week-old female with IP presented with bilateral retinal vascular occlusions in both eyes. Ultra-widefield FA showed reperfusion after treatment with intravitreal bevacizumab (IVB) and angiography-guided laser to the avascular retina. Anti-vascular endothelial growth factor treatment reduces neovascularization and allows for growth of retinal vessels. IVB and FA-guided laser to the avascular retina is an option in IP. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:e33-e37.]

A Novel Pathogenic Variant in NDP

Familial exudative vitreoretinopathy (FEVR) is a rare hereditary ocular disorder characterized by incomplete or abnormal development of peripheral retinal vasculature. The genes responsible for this disorder are associated with the wingless-related integration site (Wnt) signaling pathway, a critical pathway for the development of normal retinal vasculature. A pathogenic variant in any one of these genes may disrupt retinal vasculogenesis. Furthermore, the type and number of pathogenic variants may influence the severity of disease and clinical course. Here, the authors identify a novel pathogenic variant in the NDP gene, not previously described in the literature. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:120-124.]

Microcornea, posterior megalolenticonus, persistent fetal vasculature, chorioretinal coloboma (MPPC) syndrome: Case series post vitrectomy

MPPC syndrome has been described as a syndrome that presents with chorioretinal coloboma, posterior megalolenticonus, persistent fetal vasculature, and chorioretinal coloboma. The purpose of our study is to report three patients who present with a variation of MPPC syndrome who each underwent pars plana vitrectomy, pars plana lensectomy, and amblyopic management. Clinical characteristics, ancillary test findings, and post-surgical functional results are compared to what is reported in the literature. Retrospective review of medical records of patients who presented with microcornea, persistent fetal vasculature, chorioretinal coloboma, and microphthalmia who underwent surgical correction at Bascom Palmer Eye Institute. 3 patients (6 eyes) were included, two males and one female. All patients were born full term, vaginally, and had no family history of genetic abnormalities affecting the eye. All patients had color fundus images, fluorescein angiography (FA) and echography. Four eyes underwent surgery. Following surgical intervention, patients demonstrated improved visual acuity, and improved functional status. Patients with microcornea, PFV, chorioretinal coloboma, and microphthalmos, can benefit from surgical intervention when functional decline or media opacities are noted. Though difficult to assess accurate visual acuity and visual improvement pre-operatively and post-operatively, it is evident that our patients demonstrated improvement in functionality and vision following surgical intervention

Sympathetic Ophthalmia in a 22-Month-Old Infant With Sturge–Weber Syndrome With Atypical Histopathological Correlation

Purpose:Sturge–Weber syndrome (SWS) and sympathetic ophthalmia (SO) are unusual pathologies. Their association has not been previously reported. This unusual diagnosis can be elucidated by clinical suspicion.Methods:Case report of a 22-month-old female with SO.Results:SWS with a unilateral diffuse choroidal hemangioma. The exciting eye had multiple surgeries for glaucoma that required enucleation. Clinical picture of SO was found on the sympathizing eye. Atypical histopathology (nongranulomatous choroidal infiltrate and perivascular mononuclear cells) is found in the enucleated eye.Conclusions:To the best of the authors’ knowledge, this is the first description of an association of SWS, diffuse choroidal hemangioma, infantile glaucoma, and SO. Many confounding factors are present (ie, multiple surgeries, risk of infectious endophthalmitis). Therefore, a high index of suspicion must be kept in mind to make the diagnosis. We aim to raise awareness of such a devastating condition in a pediatric patient with many comorbidities.</jats:sec

X-linked peripheral retinoschisis without macular involvement: a case series with RS1 genetic confirmation

Background: Juvenile X-linked Retinoschisis (JXLRS) is a hereditary retinopathy that commonly presents with macular retinoschisis. In this study, we describe a group of patients who presented with peripheral retinoschisis with no macular schisis. Materials and Methods: A retrospective case series of three JXLRS patients with genetically confirmed RS1 genotypes was identified. Presence of macular and/or peripheral retinoschisis as assessed by optical coherence tomography (OCT), wide-field fluorescein angiography, clinical ophthalmoscopy, and color fundus photography. Results: The eyes of the three JXLRS patients with peripheral retinoschisis had no macular schisis or atrophy on OCT. ERG was available in one patient and showed no reduced b-waves on scotopic combined rod-cone response. Conclusions: RS1 mutations can cause a macular-sparing JXLRS phenotype. The diagnosis of JXLRS should be considered for young males presenting with peripheral retinoschisis even if there is no evidence of macular schisis