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Rare Diseases of the Anterior Segment of the Eye: Update on Diagnosis and Management

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Rare Diseases of the Anterior Segment of the Eye: Update on Diagnosis and Management BioMed Research International Rare Diseases of the Anterior Segment of the Eye: Update on Diagnosis and Management Guest Editors: Alessandro Lambiase, Flavio Mantelli, Marta Sacchetti, Siavash Rahimi, and Giacomina Massaro-Giordano Rare Diseases of the Anterior Segment of the Eye: Update on Diagnosis and Management BioMed Research International Rare Diseases of the Anterior Segment of the Eye: Update on Diagnosis and Management Guest Editors: Alessandro Lambiase, Flavio Mantelli, Marta Sacchetti, Siavash Rahimi, and Giacomina Massaro-Giordano Copyright © òýÔ Hindawi Publishing Corporation. All rights reserved. is is a special issue published in “BioMed Research International.” All articles are open access articles distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Contents Rare Diseases of the Anterior Segment of the Eye: Update on Diagnosis and Management, Alessandro Lambiase, Flavio Mantelli, Marta Sacchetti, Siavash Rahimi, and Giacomina Massaro-Giordano Volume òýÔ , Article ID À¥Þçòâ, ò pages Comparative Study of Anterior Eye Segment Measurements with Spectral Swept-Source and Time-Domain Optical Coherence Tomography in Eyes with Corneal Dystrophies, Anna K. Nowinska, Sławomir J. Teper, Dominika A. Janiszewska, Anita Lyssek-Boron, Dariusz Dobrowolski, Robert Koprowski, and Edward Wylegala Volume òýÔ , Article ID ý çâÞ, Ôý pages Rare Diseases Leading to Childhood Glaucoma: Epidemiology, Pathophysiogenesis, and Management, Solmaz Abdolrahimzadeh, Valeria Fameli, Roberto Mollo, Maria Teresa Contestabile, Andrea Perdicchi, and Santi Maria Recupero Volume òýÔ , Article ID ÞÔòÀ¥, ÔÔ pages Ophthalmic Alterations in the Sturge-Weber Syndrome, Klippel-Trenaunay Syndrome, and the Phakomatosis Pigmentovascularis: An Independent Group of Conditions?, Solmaz Abdolrahimzadeh, Vittorio Scavella, Lorenzo Felli, Filippo Cruciani, Maria Teresa Contestabile, and Santi Maria Recupero Volume òýÔ , Article ID Þâ ÔÀ, ÔÔ pages Diagnosis and Management of Iridocorneal Endothelial Syndrome, Marta Sacchetti, Flavio Mantelli, Marco Marenco, Ilaria Macchi, Oriella Ambrosio, and Paolo Rama Volume òýÔ , Article ID ÞâçýÀç, À pages Pediatric Glaucoma: A Literature’s Review and Analysis of Surgical Results, Gianluca Scuderi, Daniela Iacovello, Federica Pranno, Pasquale Plateroti, and Luca Scuderi Volume òýÔ , Article ID çÀçâÞý, pages Congenital Corneal Anesthesia and Neurotrophic Keratitis: Diagnosis and Management, Flavio Mantelli, Chiara Nardella, Eloisa Tiberi, Marta Sacchetti, Alice Bruscolini, and Alessandro Lambiase Volume òýÔ , Article ID ý Þâ, pages Climatic Droplet Keratopathy in Argentina: Involvement of Environmental Agents in Its Genesis Which Would Open the Prospect for New erapeutic Interventions, María Fernanda Suárez, Leandro Correa, Nicolás Crim, Evangelina Espósito, Rodolfo Monti, Julio Alberto Urrets-Zavalía, and Horacio Marcelo Serra Volume òýÔ , Article ID òÞç , À pages Stem Cell erapy for Corneal Epithelium Regeneration following Good Manufacturing and Clinical Procedures, Beatriz E. Ramírez, Ana Sánchez, José M. Herreras, Itziar Fernández, Javier García-Sancho, Teresa Nieto-Miguel, and Margarita Calonge Volume òýÔ , Article ID ¥ý¥À , ÔÀ pages e Genetics and the Genomics of Primary Congenital Glaucoma, Raaella Cascella, Claudia Strafella, Chiara Germani, Giuseppe Novelli, Federico Ricci, Stefania Zampatti, and Emiliano Giardina Volume òýÔ , Article ID çòÔòÀÔ, Þ pages Fuchs Endothelial Corneal Dystrophy: Strong Association with rsâÔçÞò Not Paralleled by Changes in Corneal Endothelial TCF¥ mRNA Level, Monika Ołdak, Ewelina Ruszkowska, Monika Udziela, Dominika Ozie¸bło, Ewelina Bińczyk, Aneta Ścieżyńska, Rafał Płoski, and Jacek P. Szaik Volume òýÔ , Article ID â¥ýòç¥, â pages SLC¥AÔÔ and the Pathophysiology of Congenital Hereditary Endothelial Dystrophy, Sangita P. Patel and Mark D. Parker Volume òýÔ , Article ID ¥Þ çÀò, Þ pages Cultivated Oral Mucosa Epithelium in Ocular Surface Reconstruction in Aniridia Patients, Dariusz Dobrowolski, Boguslawa Orzechowska-Wylegala, Bogumil Wowra, Ewa Wroblewska-Czajka, Maria Grolik, Krzysztof Szczubialka, Maria Nowakowska, Domenico Puzzolo, Edward A. Wylegala, Antonio Micali, and Pasquale Aragona Volume òýÔ , Article ID òÔÞý, Þ pages Hindawi Publishing Corporation BioMed Research International Volume 2015, Article ID 947326, 2 pages http://dx.doi.org/10.1155/2015/947326 Editorial Rare Diseases of the Anterior Segment of the Eye: Update on Diagnosis and Management Alessandro Lambiase,1 Flavio Mantelli,2 Marta Sacchetti,3 Siavash Rahimi,4 and Giacomina Massaro-Giordano5 1 Department of Sense Organs, Sapienza University of Rome, Viale del Policlinico, No. 155, 00196 Rome, Italy 2DepartmentofBiology,CollegeofScienceandTechnology,TempleUniversity,1900N.12thStreet,Philadelphia,PA19122,USA 3CorneaandOcularSurfaceUnit,SanRaffaeleHospital,ViaOlgettina,No.60,20132Milan,Italy 4QueenAlexandraHospital,SouthwickHillRoad,CoshamPO63LY,UK 5Scheie Eye Institute, University of Pennsylvania, 51 N. 39th Street, Philadelphia, PA 19104, USA Correspondence should be addressed to Alessandro Lambiase; [email protected] Received 17 August 2015; Accepted 24 August 2015 Copyright © 2015 Alessandro Lambiase et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Thisspecialissueisfocusedonthecurrentapproachesused Ascanbeseenfromthevarietyoftopicspresentedin to identify and manage rare diseases of the anterior segment this special issue, the disorders grouped under the umbrella of the eye, which range from congenital to acquired disorders term of rare diseases of the anterior segment of the eye can be that are caused by ocular or systemic conditions and often considered quite heterogenous and numerous and, in turn, have consequences that extend beyond the anterior segment represent a substantial clinical burden on a worldwide basis of the eye. Specifically, the groups of Dr. R. Cascella et al. [1]. In fact, although single disorders are rarely encountered and Dr. G. L. Scuderi et al. provided a complete overview in clinical practice, when taken together, they are not seldom on primary congenital glaucoma and pediatric glaucoma, seen. In addition, these diseases often represent a clinical respectively, from diagnosis to treatment outcomes; the challenge due to the paucity of specific diagnostic criteria groupsofDr.D.Dobrowolskietal.andDr.B.E.Ram´ırez and the lack of specific treatments. In fact, rare disorders et al. described the use of oral mucosa and stem cell therapy, tend to remain orphan drug indications due to the difficulty respectively, for ocular surface reconstruction in rare diseases of running clinical trials, and surgical approaches are often such as aniridia; Dr. F. Mantelli and colleagues reviewed inadequateduetoconcomitantanatomicalandcongenital the diagnosis and management of congenital corneal anes- anomalies. thesia and neurotrophic keratitis; Dr. A. K. Nowinska and colleagues compared different tomographic approaches to To make the clinical picture of the rare diseases of the evaluate rare corneal dystrophies; the groups of Dr. M. Ołdak anterior segment of the eye even more difficult to manage, and Dr. S. P. Patel explained the pathophysiology of Fuchs they often have consequences that extend beyond the anterior endothelial corneal dystrophy and congenital hereditary segment and can severely impair vision. Specifically, while endothelial corneal dystrophy, respectively; Dr. M. Sacchetti corneal opacities and iris anomalies may directly affect and colleagues provide a guide to diagnose and manage visual function, several diseases such as iridocorneal angle the iridocorneal endothelial (ICE) syndrome; Dr. S. Abdol- anomalies may induce glaucoma with consequent damage to rahimzadeh and colleagues described a series of rare diseases the optic nerve. In addition, several congenital diseases of and ophthalmic alterations that may lead to glaucoma; Dr. the anterior segment of the eye are also associated with facial M. F. Suarez´ et al. described how environmental agents can anomalies and other malformations of intraocular structures, influence a rare corneal disease, climatic droplet keratopathy. making this group of disorders particularly complex for 2 BioMed Research International the ophthalmologist, who encounters obstacles in patients’ [7] M. Sacchetti, I. Macchi, A. Tiezzi, M. La Cava, G. Massaro- management from diagnosis to treatment [2]. Giordano, and A. Lambiase, “Pathophysiology of corneal dys- The recent advances in translational research, which trophies: from cellular genetic alteration to clinical findings,” are reshaping modern ophthalmology, allowed identifying Journal of Cellular Physiology,2015. the link between different clinical phenotypes and specific [8] A. Lambiase and M. Sacchetti, “Diagnosis and management of mutations in genes regulating the normal formation and neurotrophic keratitis,” Clinical Ophthalmology,vol.8,pp.571– maturation of the anterior segments of the eye [3]. In 579, 2014. turn, our ability to understand the pathogenesis of rare and [9] R. W. Morris and M. T. Dunbar, “Atypical presentation and complex ophthalmic diseases has improved and, recently, review of the ICE syndrome,” Optometry,vol.75,no.1,pp.13– novel treatments are being proposed for selected disorders
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