The Asia-Pacific Journal of Ophthalmology

  • May/June 2018 - Volume 7 - Issue 3
    pp: 129-214
    Special Issue on Pediatric Retinal Diseases
    Guest Editors-in-Chief: R.V. Paul Chan, Wai-Ching Lam
    Guest Editors: Michael Chiang, Shunji Kusaka, Chetan Kantibhai Patel, Wei-Chi Wu


Pediatric Retinal Diseases Lam, Wai-Ching; Chan, R.V. Paul
Retinal disorders in children can be very different than those in adults, and there are a number of considerations that are unique to the management of pediatric retina patients. Although we have made significant advances in our understanding of pediatric retinal disease, there is much we are still learning about the physiology and management of these conditions.

Review Article

Pharmacotherapy and ROP: Going Back to the Basics Shulman, Julia P.; Hartnett, M. Elizabeth
Retinopathy of prematurity (ROP) is a leading cause of blindness in preterm infants around the world. Through the development of animal models and clinical trials our understanding of the pathophysiology of this disease and approach to therapy has evolved significantly since ROP was first described in the 1940s in the United States. The mainstay of treatment in ROP remains ablative laser therapy to the avascular retina but pharmacologic agents are being more and more commonly used with new targets for pharmacotherapy emerging. This paper summarizes our current understanding of the pathophysiology of ROP based on the data gleaned from animal models and discusses current approaches to pharmacotherapy
Anti–Vascular Endothelial Growth Factor and the Evolving Management Paradigm for Retinopathy of Prematurity Darwish, Dana; Chee, Ru-Ik; Patel, Samir N.; Jonas, Karyn; Ostmo, Susan; Campbell, J. Peter; Chiang, Michael F.; Chan, R.V. Paul
Diagnosis and management of pediatric retinal conditions such as retinopathy of rematurity (ROP) have been evolving significantly with the availability of new technology and treatments. New imaging systems, telemedicine, tele-education, and anti‒vascular endothelial growth factor (VEGF) intravitreal pharmacotherapy are all changing the way we diagnose and deliver care to children with pediatric retinal disease. Fluorescein angiography and optical coherence tomography have the potential to improve our diagnosis and management of disease, and with improvements in retinal imaging, telemedicine is becoming more feasible. Telemedicine, tele-education, and computer-based image analysis may overcome many of the challenges we face in providing adequate care and access for children with pediatric retinal disease. Treatment options have also expanded with the use of anti-VEGF therapy. Although the use of intravitreal anti-VEGF for ROP has been documented in the literature for more than a decade, many questions still remain about its safety in the pediatric patient population. Several ongoing prospective studies are exploring the utility of anti-VEGF agents for ROP, with attention to the optimal dose of drug, systemic safety, and our understanding of recurrence of disease. This review aims to provide an update on current diagnostic and therapeutic modalities, focusing predominantly on the role of anti-VEGF therapy, for the management of ROP and other pediatric retinal vascular diseases.
Anti-VEGF for ROP and Pediatric Retinal Diseases Wu, An-Lun; Wu, Wei-Chi
Vascular endothelial growth factor (VEGF) is an important mediator of the pathological neovascularization and vascular permeability of the eye. The increasing use of intravitreal therapies targeting VEGF has revolutionized the treatment of pediatric vitreoretinal diseases. In retinopathy of prematurity (ROP), the role of VEGF in pathogenesis has been well recognized and the use of anti-VEGF treatment in phase 2 ROP has demonstrated promising results, particularly in severe cases of posterior disease. This has made VEGF an established target in the treatment of pediatric retinal diseases known to have abnormal vascular activity. However, questions remain about late systemic and neurodevelopmental effects after anti-VEGF therapy for children because intravitreal injection of anti-VEGF may result in systemic circulation of anti-VEGF agent and a corresponding suppression of systemic VEGF. We do not currently know whether the short-term suppression of systemic VEGF affects long-term neurodevelopmental outcome because VEGF is a vital supporting factor during neurodevelopment. This review article focuses on the evidence for the use of anti-VEGF treatment in certain pediatric ocular diseases, including ROP, Coats' disease, and retinoblastoma (RB). More extensive and prospective studies are warranted to further elucidate the role of anti-VEGF therapy in these diseases and illustrate how we can optimally use these agents in pediatric patients.
Plus Disease in Retinopathy of Prematurity: More Than Meets the ICROP? Ghergherehchi, Layla; Kim, Sang Jin; Campbell, J. Peter; Ostmo, Susan; Chan, R.V. Paul; Chiang, Michael F.
Retinopathy of prematurity (ROP), a vasoproliferative retinal disease affecting premature infants, is a leading cause of childhood blindness throughout the world. Plus disease, defined as venous dilatation and arteriolar tortuosity within the posterior retinal vessels greater than or equal to that of a standard published photograph, is the most critical finding in identifying treatment-requiring ROP. Despite an internationally accepted definition of plus disease, there is significant variability in diagnostic process and outcome, producing variable levels of reported intra- and interexpert agreement. Several potential explanations for poor agreement have been proposed, including attention to undefined vascular features such as venous tortuosity, focus on narrower or wider field of view, unfamiliarity with digital images, the magnification and apparent severity of the standard photograph, and cut-off point differences among experts as to the level of tortuosity and dilation sufficient for “plus disease” along a continuum. Moreover, differences in diagnostic consistency among groups of experts separated both geographically and chronologically have been reported. These findings have implications for clinical care, research, and education, and highlight the need for a more precise definition of plus disease and objective diagnostic methods for ROP
Surgical Management of Coats Disease Kusaka, Shunji
Coats disease is a sporadic, chronic retinal vascular disorder with telangiectatic and aneurysmal retinal vessels, with retinal exudation and retinal detachment in severe cases. Various treatment modalities have been used, including laser therapy, cryotherapy, anti-vascular endothelial growth factor (VEGF) therapy, and surgery. Anti-VEGF therapy seems to be effective in reducing exudative changes and is often combined with other treatment modalities. For severe cases with exudative retinal detachment, external drainage of subretinal fluid with or without vitrectomy is commonly performed, combined with laser therapy and/or cryotherapy. Relatively good anatomical outcomes have been reported; however, postoperative functional results, particularly in young patients and/or severe cases, seem to be limited.
Retinoblastoma for Pediatric Ophthalmologists AlAli, Alaa; Kletke, Stephanie; Gallie, Brenda; Lam, Wai-Ching
Retinoblastoma can present in 1 or both eyes and is the most common intraocular malignancy in childhood. It is typically initiated by biallelic mutation of the RB1 tumor suppressor gene, leading to malignant transformation of primitive retinal cells. The most common presentation is leukocoria, followed by strabismus. Heritable retinoblastoma accounts for 45% of all cases, with 80% being bilateral. Treatment and prognosis of retinoblastoma is dictated by the disease stage at initial presentation. The 8th Edition American Joint Committee on Cancer (AJCC) TNMH (tumor, node, metastasis, heritable trait) staging system defines evidence-based clinical and pathological staging for overall prognosis for eye(s) and child. Multiple treatment options are available in 2018 for retinoblastoma management with a multidisciplinary team, including pediatric ocular oncology, medical oncology, radiation oncology, genetics, nursing, and social work. Survival exceeds 95% when disease is diagnosed early and treated in centers specializing in retinoblastoma. However, survival rates are less than 50% with extraocular tumor dissemination. We summarize the epidemiology, genetics, prenatal screening, diagnosis, classification, investigations, and current therapeutic options in the management of retinoblastoma.
Congenital X-Linked Retinoschisis: An Updated Clinical Review Rao, Prethy; Dedania, Vaidehi S.; Drenser, Kimberly A.
We present an updated clinical review of the pathophysiology, progression, and current treatments in pediatric patients with congenital X-linked retinoschisis (CXLRS). CXLRS is an X-linked inherited retinal degeneration characterized by splitting of the superficial layers of the retina. Most recent classification divides CXLRS into 4 distinct clinical phenotypes: type 1, foveal; type 2, foveolamellar; type 3, complex; and type 4, foveoperipheral. The majority of retinoschisis cavities remain stable throughout life and may spontaneously collapse. However, a select number of patients progress to macula-involving peripheral retinoschisis, rhegmatogenous, and combined tractional-rhegmatogenous detachments that require further intervention. Although several advances have been made over the past several decades, medical therapy remains limited to case series‒based carbonic anhydrase therapy and prophylactic laser retinopexy. Recent advances in genetic-based clinical trials with the retinoschisis gene are promising. Vitreoretinal surgical approaches remain complex, case-based, and require careful planning depending on the configuration and location of the retinoschisis cavity.
Familial Exudative Vitreoretinopathy: Pathophysiology, Diagnosis, and Management Tauqeer, Zujaja; Yonekawa, Yoshihiro
Familial exudative vitreoretinopathy (FEVR) is a heritable vitreoretinopathy characterized by anomalous retinal vascular development. The principal feature of the disease is an avascular peripheral retina. This in turn can cause further pathological changes including neovascularization, exudation, hemorrhage, and retinal detachment. The biological basis of the disease is thought to be from defects in the Wnt signaling pathway. Many gene mutations have been implicated, and these can be inherited in an autosomal dominant (most common), autosomal recessive, and X-linked recessive fashion. Examination with wide-field fluorescein angiography is essential and can identify the disease in its earlier stages, enabling timely treatment, in addition to helping identify asymptomatic family members. The current treatment paradigm involves laser photocoagulation of the avascular peripheral retina for neovascular sequelae and vitreoretinal surgery for progressive retinal detachment. Further studies are underway to better characterize this complex vitreoretinopathy.
Caring for Hereditary Childhood Retinal Blindness Jauregui, Ruben; Cho, Galaxy Y.; Takahashi, Vitor K.L.; Takiuti, Julia T.; Bassuk, Alexander G.; Mahajan, Vinit B.; Tsang, Stephen H.
Inherited retinal diseases (IRDs) are a major cause of incurable familial blindness in the Western world. In the pediatric population, IRDs are a major contributor to the 19 million children worldwide with visual impairment. Unfortunately, the road to the correct diagnosis is often complicated in the pediatric population, as typical diagnostic tools such as fundus examination, electrodiagnostic studies, and other imaging modalities may be difficult to perform in the pediatric patient. In this review, we describe the most significant IRDs with onset during the pediatric years (ie, before the age of 18). We describe the pathogenesis, clinical presentation, and potential treatment of these diseases. In addition, we advocate the use of a pedigree (family medical history), electroretinography, and genetic testing as the 3 most crucial tools for the correct diagnosis of IRDs in the pediatric population.
Pediatric Uveitis Chan, Nicole Shu-Wen; Choi, Jessy; Cheung, Chui Ming Gemmy
Pediatric uveitis differs from adult-onset uveitis and is a topic of special interest because of its diagnostic and therapeutic challenges. Children with uveitis are often asymptomatic and the uveitis is often chronic, persistent, recurrent, and resistant to conventional treatment. Anterior uveitis is the most common type of uveitis in children; the prevalence of intermediate, posterior, and panuveitis varies geographically and among ethnic groups. Regarding etiology, most cases of pediatric uveitis are idiopathic but can be due to systemic inflammatory disorders, infections, or a manifestation of masquerade syndrome. Ocular complications include cataracts, hypotony or glaucoma, band keratopathy, synechiae formation, macular edema, optic disc edema, choroidal neovascular membranes, and retinal detachment. These complications are often severe, leading to irreversible structural damage and significant visual disability due to delayed presentation and diagnosis, persistent chronic inflammation from suboptimal treatment, topical and systemic corticosteroid dependence, and delayed initiation of systemic disease‒modifying agents. Treatment for noninfectious uveitis is a stepwise approach starting with corticosteroids. Immunomodulatory therapy should be initiated in cases where quiescence cannot be achieved without steroid dependence. Patients should be monitored regularly for complications of uveitis along with systemic and ocular adverse effects from treatments. The goals are to achieve steroid-free durable remission, to reduce the risk of sight-threatening complications from the uncontrolled ocular inflammation, and to avoid the impact of lifelong burden of visual loss on the child and their family. Multidisciplinary management will ensure holistic care of affected children and improve the support for their families.
Endoscopy for Pediatric Retinal Disease Yeo, Damien C.M.; Nagiel, Aaron; Yang, Unikora; Lee, Thomas C.; Wong, Sui Chien
Endoscopic vitrectomy is a useful and unique adjunct to microincision vitreoretinal surgery. The optical properties of endoscopy allow for some clinically advantageous approaches that are not possible with regular microscope viewing systems, namely, the ability to both bypass optically significant anterior segment opacities and directly visualize difficult-to-access retroirideal, retrolental, and anterior retinal structures in their natural anatomical configuration. The surgical benefits include improved surgical access to the pars plana, pars plicata, ciliary sulcus, ciliary body, and peripheral lens, along with unique access to anterior traction in complex pediatric anterior retinal detachments, particularly in retinopathy of prematurity. This review will focus on the development and surgical utility of intraocular endoscopy, provide an update on its current uses in the era of microincision vitreoretinal surgery, and highlight its role in pediatric vitreoretinal diseases.
Ultra-Widefield Imaging for Pediatric Retinal Disease Patel, Chetan Kantibhai; Buckle, Miranda
This article explores the development of retinal imaging, with particular emphasis on ultra-widefield imaging and the key concept of field of view. Two ultra-widefield imaging platforms are examined in detail—Optomap and Spectralis—noncontact imaging systems that include protocols for performing angiography in infants. Applications of ultra-widefield imaging are illustrated using case studies, including diagnosis, monitoring, and screening.
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