Historical Review and Update of Surgical Treatment for Corneal Endothelial Diseases
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Ophthalmol Ther (2014) 3:1–15 DOI 10.1007/s40123-014-0022-y REVIEW Historical Review and Update of Surgical Treatment for Corneal Endothelial Diseases Jose´ L. Gu¨ell • Mostafa A. El Husseiny • Felicidad Manero • Oscar Gris • Dani Elies To view enhanced content go to www.ophthalmology-open.com Received: May 13, 2014 / Published online: February 18, 2014 Ó The Author(s) 2014. This article is published with open access at Springerlink.com ABSTRACT extraction. Pseudophakic bullous keratopathy (PBK) is endothelial cell loss caused by surgery The cornea remains in a state of deturgescence, in the anterior chamber. If the corneal maintained by endothelial cell Na?/K? ATPase endothelium is damaged during surgery, the and by tight junctions between endothelial cells same spectrum of symptoms as found in FECD that limit entrance of fluid into the stroma. can develop. In the nineteenth century, Fuchs’ endothelial corneal dystrophy (FECD) penetrating keratoplasty was the only surgical was initially described by Fuchs in 1910 as a procedure available for isolated endothelial combination of epithelial and stromal edema in disease. In the 1960s, Dr. Jose´ Barraquer older patients. It manifests as bilateral, albeit described a method of endothelial keratoplasty asymmetric, central corneal guttae, corneal using an anterior approach via laser-assisted edema, and reduced vision. When edema is in situ keratomileusis (LASIK) flap. In 1999, severe, the corneal epithelium can detach from Melles and colleague described their technique its basement membrane, creating painful bullae of posterior lamellar keratoplasty. Later, Melles on the anterior surface of the cornea. The course et al. started to change host dissection using of this dystrophy can be further accelerated simple ‘‘descemetorhexis’’ in a procedure after intraocular surgery, specifically cataract known as Descemet’s stripping endothelial Electronic supplementary material The online keratoplasty. Following the widespread version of this article (doi:10.1007/s40123-014-0022-y) adoption of Descemet’s stripping automated contains supplementary material, which is available to authorized users. endothelial keratoplasty, the Melles group revisited selective Descemet’s membrane J. L. Gu¨ell (&) Á F. Manero Á O. Gris Á D. Elies transplantation and reported the results of a Cornea and Refractive Surgery Unit, Autonoma University of Barcelona, new procedure, Descemet’s membrane a C/Josep M Llado´, 08035 Barcelona, Spain endothelial keratoplasty (DMEK). Recently, e-mail: [email protected] some eye banks have experimented with the M. A. El Husseiny preparation of DMEK/Descemet’s membrane Cornea and Refractive Surgery Department, Research Institute of Ophthalmology (RIO), automated endothelial keratoplasty donor Giza, Egypt 2 Ophthalmol Ther (2014) 3:1–15 tissue that may help the surgeon avoid the risk Therefore, lost cells cannot be replaced of tissue loss during the stromal separation step. physiologically. Recently, the authors described a new bimanual This review follows the development of technique for insertion and positioning of surgical treatment of endothelial diseases, from endothelium–Descemet membrane grafts in penetrating keratoplasty to different approaches DMEK. of selective endothelial keratoplasty. Keywords: Endothelial; Disease; Endothelial METHODS transplant; Fuch’s dystrophy; Ocular surgery; Ophthalmology; Posterior lamellar For this review, the authors searched PubMed keratoplasty; Pseudophakic bullous edema using the keywords ‘‘Endothelial disease; Endothelial transplant; Fuch’s dystrophy; Ocular surgery; Posterior lamellar keratoplasty; INTRODUCTION Pseudophakic bullous edema’’. They also searched ophthalmology books about cornea The adult human cornea averages 540 lmin and papers published in the last decades thickness [1], with the following layers from discussing the different steps of evolution of anterior to posterior: epithelium, Bowman’s corneal surgeries and management of membrane, stroma, Descemet’s membrane endothelial diseases. The article is divided to (DM), and endothelium. The cornea remains subsequent headlines putting into consideration in a state of deturgescence, maintained by the chronological evolution (abstract, endothelial cell Na?/K? ATPase and by tight introduction, methods, indications for junctions between endothelial cells that limit keratoplasty, keratoplasty in the nineteenth entrance of fluid into the stroma. By century, keratoplasty in the early twentieth maintaining an optimum level of corneal century, use of the human cornea, fixation hydration, endothelial cells preserve the techniques, establishment of eye banks, ordered arrangement of collagen, which is development of endothelial keratoplasty, crucial for corneal transparency [2]. When conclusion and references). The authors used endothelial cell density is low, the loss of tight the statistics of the eye bank association of junctions between cells allows more fluid to America. The abstract, the full article and enter the stroma. The endothelial cells that references were obtained and references checked remain may have a higher concentration of for additional material where appropriate. Na?/K? ATPase in an effort to compensate for the loss [1]. The average human cornea has an INDICATIONS FOR KERATOPLASTY endothelial cell density of 5,000–6,000 cells/ mm2 at birth, decreasing to 2,500–3,000 cells/ Fuchs’ endothelial corneal dystrophy (FECD) mm2 by adulthood. There is an average cell loss was initially described by Fuchs [5] in 1910 as a of 0.6% per year [1]. Corneal edema appears at combination of epithelial and stromal edema in 700–400 cells/mm2 [1, 3]. Adult human corneal older patients. It manifests itself as bilateral, endothelial cells are arrested in the G phase of albeit asymmetric, central corneal guttae, the cell cycle and do not undergo mitosis [4]. corneal edema, and reduced vision [6, 7]. The Ophthalmol Ther (2014) 3:1–15 3 Fig. 1 Corneal edema in a pseudophakic eye with Fuchs’ endothelial corneal dystrophy DM thickens and develops excrescences known occurs during cataract extraction) [3], the same histopathologically as guttae. Stromal edema spectrum of symptoms as found in FECD can develops and corneal thickness may increase to develop, although the histological phenotype of over 1,000 lm. When the edema is severe, the both diseases is different and there is no guttata in corneal epithelium can detach from its basement PBK (Fig. 1). membrane, creating painful bullae on the Toxic anterior segment syndrome (TASS) is a anterior surface of the cornea [2, 8]. FECD is the rare complication of intraocular surgery that most common endothelial dystrophy and is has only recently been recognized [14]. It is an usually seen beyond the fifth decade of life, acute sterile inflammation in the anterior although not all cases are in the elderly; Biswas segment caused by noxious agents such as et al. [9] reported several families with early onset medications, residual viscoelastic agents, and of this dystrophy in the third and fourth decades preservatives, or by an altered osmolarity or pH of life. FECD is, despite its dominant inheritance of the irrigating solution [15–17]. Permanent form, more common and progressive in women corneal endothelial damage can occur in severe [10]. It may also present in a sporadic form and is cases of TASS. thought to be a primary disorder of the endothelium. The total number of endothelial KERATOPLASTY cells is low and existing cells may not function IN THE NINETEENTH CENTURY properly. The course of this dystrophy can be further accelerated after intraocular surgery, In the nineteenth century, penetrating specifically cataract extraction. A cell count of keratoplasty (PK) was the only surgical less than 1,000 cells/mm2 or corneal thickness procedure used for isolated endothelial disease greater than 640 lm are considered major risk whether it was FECD or PBK; however, this factors for corneal decompensation after cataract procedure proved unsuccessful because of a surgery [11–13]. total lack of knowledge about many basic Pseudophakic bullous keratopathy (PBK) is a concepts that could prevent failure, such as term used to describe endothelial cell loss caused sepsis, immunology and tissue biology, by surgery in the anterior chamber. If the corneal anatomy and physiology of the cornea, and endothelium is damaged during surgery (as often anesthesia. 4 Ophthalmol Ther (2014) 3:1–15 The concept of removing a cloudy cornea development of general anesthesia. This was was discussed by Charles Darwin’s first used in 1846, in the Etherdome at grandfather, Erasmus Darwin, in 1796. He Massachusetts General Hospital, and was felt that after an ulcer of the cornea, the scar followed by the introduction of chloroform in could be cut out and have it heal with a 1847. In 1867, Lister first brought attention to transparent scar [18]. In 1824, Franz Reisinger the importance of an aseptic setting for [19] coined the term ‘keratoplasty’ and he is successful surgery. Topical cocaine was credited with this term, although controversy discovered by Kohler in 1884 [18]. surrounds this attribution. During this period, it was believed that the human cornea could be replaced with an animal cornea from KERATOPLASTY IN THE EARLY another species. Wilhelmus Thome, in 1834, TWENTIETH CENTURY was the first to use the term ‘corneal transplantation’, although he did not The first visually successful human corneal undertake such a procedure [18]. Records transplant was performed on December 7, suggest that the first