scholarly journals Management of Corneal Clouding in Patients with Mucopolysaccharidosis

2021 ◽  
Vol 10 (15) ◽  
pp. 3263
Author(s):  
Orlaith McGrath ◽  
Leon Au ◽  
Jane Ashworth
Keyword(s):  
Corneal Transplantation ◽  
Research Progress ◽  
Definitive Treatment ◽  
Lamellar Keratoplasty ◽  
Deep Anterior Lamellar Keratoplasty ◽  
Hematopoietic Stem ◽  
Enzyme Replacement ◽  
Corneal Clouding ◽  
Organ Systems ◽  
Anterior Lamellar Keratoplasty

Mucopolysaccharidoses (MPS) are a rare group of lysosomal storage disorders characterized by the accumulation of incompletely degraded glycosaminoglycans (GAGs) in multiple organ systems including the eye. Visual loss occurs in MPS predominantly due to corneal clouding and retinopathy, but the sclera, trabecular meshwork and optic nerve may all be affected. Despite the success of therapies such as enzyme replacement therapy (ERT) and hematopoietic stem-cell transplantation (HSCT) in improving many of the systemic manifestations of MPS, their effect on corneal clouding is minimal. The only current definitive treatment for corneal clouding is corneal transplantation, usually in the form of a penetrating keratoplasty or a deep anterior lamellar keratoplasty. This article aims to provide an overview of corneal clouding, its current clinical and surgical management, and significant research progress.

scholarly journals Multimodal imaging of Hurler syndrome-related keratopathy treated with deep anterior lamellar keratoplasty

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Elodie Da Cunha ◽  
Cristina Georgeon ◽  
Nacim Bouheraoua ◽  
Marc Putterman ◽  
Françoise Brignole-Baudouin ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Multimodal Imaging ◽  
Lamellar Keratoplasty ◽  
Deep Anterior Lamellar Keratoplasty ◽  
Imaging Features ◽  
Hurler Syndrome ◽  
Hematopoietic Stem ◽  
First Choice ◽  
Anterior Lamellar Keratoplasty ◽  
Sd Oct

Abstract Background Hurler syndrome-associated keratopathy is an exceedingly rare corneal disorder that requires corneal transplantation in advanced stages. Precise assessment of the corneal condition is necessary for deciding which type of keratoplasty (i.e., deep anterior lamellar or penetrating) should be proposed. We aimed to confront the results of multimodal imaging with those of histology in a case of Hurler syndrome-associated keratopathy. Case presentation A 16-year-old patient with Hurler’s syndrome treated with hematopoietic stem cell transplantation was referred for decreased vision related to advanced keratopathy. The patient was treated with deep anterior lamellar keratoplasty (DALK) in both eyes with uncomplicated outcome. Visual acuity improved from 0.1 (20/200) preoperatively to 0.32 (20/63) and 0.63 (20/32) after transplantation. The corneal endothelial cell density was 2400 cells/mm2 in both eyes 3 years after transplantation. In vivo confocal microscopy (IVCM) and spectral domain optical coherence tomography (SD-OCT) were performed preoperatively. The corneal buttons retrieved during keratoplasty were processed for histology. In SD-OCT scans, corneal opacities appeared as diffuse stromal hyperreflectivity associated with increased corneal thickness. IVCM showed diffuse cytoplasmic granular hyperreflectivity and rounded/ellipsoid aspects of keratocytes, presence of small intracellular vacuoles, and hyperreflective epithelial intercellular spaces. Bowman’s layer was thin and irregular. The corneal endothelium was poorly visualized but no endothelial damage was observed. Histology showed irregular orientation and organization of stromal lamellae, with the presence of macrophages whose cytoplasm appeared clear and granular. A perinuclear clear halo was visible within the epithelial basal cells. Bowman’s layer featured breaks and irregularities. Conclusions The observed corneal multimodal imaging features in mucopolysaccharidosis-related keratopathy were concordant with histology. Compared with standard histology, multimodal imaging allowed additional keratocyte features to be observed. It revealed both morphological and structural changes of all corneal layers but the endothelium. This information is essential for therapeutic management which should include DALK as the first-choice treatment in case of impaired visual acuity.

scholarly journals Approaches toward enhancing survival probability following deep anterior lamellar keratoplasty

2020 ◽  
Vol 12 ◽  
pp. 251584142091301
Author(s):  
Sepehr Feizi ◽  
Amir A Azari
Keyword(s):  
Graft Rejection ◽  
Graft Failure ◽  
Corneal Transplantation ◽  
Corneal Neovascularization ◽  
Lamellar Keratoplasty ◽  
Deep Anterior Lamellar Keratoplasty ◽  
Vernal Keratoconjunctivitis ◽  
Full Thickness ◽  
Appropriate Treatment ◽  
Anterior Lamellar Keratoplasty

The greatest advantage of deep anterior lamellar keratoplasty over full-thickness corneal transplantation is the elimination of graft failure caused by endothelial rejection. Despite this advantage, a deep anterior lamellar keratoplasty graft can fail because of several factors, such as complications related to the donor–recipient interface, graft epithelial abnormalities, graft vascularization, stromal graft rejection, and recurrence of herpetic keratitis. Increased deep anterior lamellar keratoplasty graft survival is mainly built upon optimization of the ocular surface to provide a hospitable environment for the graft. Any predisposing factors for graft epithelial abnormalities, corneal neovascularization, and preexisting vernal keratoconjunctivitis should be identified and treated preoperatively. Prompt recognition and appropriate treatment of interface-related complications and stromal graft rejection usually result in good anatomic outcomes, with no detrimental effects on vision.

scholarly journals Recurrent Keratoconus

2013 ◽  
Vol 2 (2) ◽  
pp. 65-68
Keyword(s):  
Penetrating Keratoplasty ◽  
Treatment Options ◽  
Corneal Transplantation ◽  
Wound Dehiscence ◽  
Lamellar Keratoplasty ◽  
Deep Anterior Lamellar Keratoplasty ◽  
Time To Recurrence ◽  
Anterior Lamellar Keratoplasty ◽  
Corneal Thinning ◽  
Corneal Disorder

Abstract Keratoconus is an ectatic corneal disorder characterized by progressive corneal thinning and protrusion. Keratoconus recurrence after corneal transplantation although rare is present and has been confirmed histologically. Host, donor, genetic, mechanical and environmental factors have been proposed as predisposing factors to initiate keratoconus recurrence. The time-to-recurrence post-deep anterior lamellar keratoplasty seems to occur earlier than after penetrating keratoplasty. Wound dehiscence and high astigmatism post-transplant are plausible differential diagnosis for this entity. The treatment options are similar to primary keratoconus. Since, the era of collagen corneal cross-linking, early diagnosis is desirable as it could halt the progression of recurrent keratoconus. How to cite this article Barbara R, Barbara A. Recurrent Keratoconus. Int J Kerat Ect Cor Dis 2013;2(2):65-68.

scholarly journals Malapposition of Graft-host Interface after Penetrating Keratoplasty (PK) and Deep Anterior lamellar Keratoplasty (DALK): an optical coherence tomography study

2019 ◽  
Author(s):  
Yujin Zhao ◽  
Hong Zhuang ◽  
Jiaxu Hong ◽  
Lijia Tian ◽  
Jianjiang Xu
Keyword(s):  
Optical Coherence Tomography ◽  
Penetrating Keratoplasty ◽  
Corneal Transplantation ◽  
Anterior Segment ◽  
Lamellar Keratoplasty ◽  
Deep Anterior Lamellar Keratoplasty ◽  
Optical Coherence ◽  
Visual Outcomes ◽  
Total Prevalence ◽  
Anterior Lamellar Keratoplasty

Abstract Backgroud: Previous studies of internal graft-host malappositions have not dealt with the precise ways in which each malapposition affected post-penetrating keratoplasty (post-PK) visual outcomes. In this study, we reviewed our post-PK and post-deep anterior lamellar keratoplasty (post-DALK) keratoconic patients and used anterior segment optical coherence tomography (AS-OCT) to evaluate the correlations between graft-host interface (GHI) characteristics and visual outcomes. Methods: Novel GHI metrics included: mean graft-host touch (GHT), total prevalence of malapposition proportion (Pm), frequency of apposition (F), size of malapposition (Sm), junctional graft thickness (Tg), and the absolute value of difference between Tg and Th (|Tg-Th|). We connected the external and internal junction points of GHI (GHT) and drew a straight line through the central point, perpendicular to both sides of the cornea. Tg and Th were the thicknesses at cross-points 1 mm away from the meeting point on the external side of the graft and host, respectively. Correlations between GHI metrics and postsurgical visual outcomes [ logarithm of minimum angle of resolution best-corrected visual acuity (logMAR BCVA), spherical equivalent diopter (SE), diopter of spherical power (DS), diopter of cylindrical power (DC) and keratometric astigmatism (Astig value)] were evaluated for 45 patients (one eye each). Results: 22 post-PK and 23 post-DALK keratoconic patients were enrolled. Compared with the regular-apposition results, GHT was decreased in step and gape patterns, and increased in hill and tag patterns. SE increased by 6.851, 5.428 and 5.164 times for every 1% increase in: F (step) [b=6.851; 95% Confidence interval (CI)=2.975-10.727; P=0.001]; F (graft step) [b=5.428; 95% CI=1.685-9.171; P=0.005]; and Pm [b=5.164; 95%CI=0.913-9.146; P=0.018], respectively. SE increased by 0.031 times for every 1-μm increment of |Tg-Th| [b=0.031; 95% CI=0.009-0.054; P=0.007]. LogMAR BCVA increased by 0.001 times for every 1-μm increment of both GHT [b=0.001; 95% CI=0-0.002; P=0.030]. and Tg [b=0.001; 95% CI=0.001-0.002; P=0.001]. Astig value increased by 0.017 times for 1-μm increment of Sm [b=0.017; 95% CI=0-0.033; P=0.047]. Conclusion: This investigation of GHI characteristics suggests explanations for varied ametropia in keratoconic eyes and has potential significance as a reference for promoting pre-surgical planning and technology for corneal transplantation.

scholarly journals Malapposition of Graft-host Interface after Penetrating Keratoplasty (PK) and Deep Anterior lamellar Keratoplasty (DALK): an optical coherence tomography study

2020 ◽  
Author(s):  
Yujin Zhao ◽  
Hong Zhuang ◽  
Jiaxu Hong ◽  
Lijia Tian ◽  
Jianjiang Xu
Keyword(s):  
Optical Coherence Tomography ◽  
Penetrating Keratoplasty ◽  
Linear Regression Analysis ◽  
Corneal Transplantation ◽  
Anterior Segment ◽  
Lamellar Keratoplasty ◽  
Deep Anterior Lamellar Keratoplasty ◽  
Optical Coherence ◽  
Visual Outcomes ◽  
Anterior Lamellar Keratoplasty

Abstract Backgroud: Previous studies of internal graft-host malappositions have not dealt with the precise ways in which each malapposition affected post-penetrating keratoplasty (post-PK) visual outcomes. In this study, we reviewed our post-PK and post-deep anterior lamellar keratoplasty (post-DALK) keratoconic patients and used anterior segment optical coherence tomography (AS-OCT) to evaluate the associations between graft-host interface (GHI) characteristics and visual outcomes. Methods: Novel GHI metrics included: mean graft-host touch (GHT), total prevalence of malapposition proportion (Pm), frequency of apposition (F), size of malapposition (Sm), junctional graft thickness (Tg), junctional host thickness (Th) and the absolute value of difference between Tg and Th (|Tg-Th|). We connected the external and internal junction points of GHI (GHT) and drew a straight line through the central point, perpendicular to both sides of the cornea. Tg and Th were the thicknesses at cross-points 1 mm away from the meeting point on the external side of the graft and host, respectively. Linear regression analysis was used to describe associations between GHI metrics and postsurgical visual outcomes [ logarithm of minimum angle of resolution best-corrected visual acuity (logMAR BCVA), spherical equivalent diopter (SE), diopter of spherical power (DS), diopter of cylindrical power (DC) and keratometric astigmatism (Astig value)]. Results: We enrolled 22 post-PK and 23 post-DALK keratoconic patients. Compared with the regular-apposition results, GHT was decreased in step and gape patterns, and increased in hill and tag patterns. SE increased averagely by 6.851, 5.428 and 5.164 diopter per 1% increase in: F (step) [β=6.851; 95% Confidence interval (CI)=2.975-10.727; P=0.001]; F (graft step) [β=5.428; 95% CI=1.685-9.171; P=0.005]; and Pm [β=5.164; 95%CI=0.913-9.146; P=0.018], respectively. SE increased averagely by 0.31 diopter per 10-μm increment in |Tg-Th| [β=0.031; 95% CI=0.009-0.054; P=0.007]. LogMAR BCVA increased (on average) by 0.01 per 10-μm increment in both GHT [β=0.001; 95% CI=0-0.002; P=0.030]. and Tg [β=0.001; 95% CI=0.001-0.002; P=0.001]. Astig value increased on average by 0.17 diopter per 10-μm increment in Sm [β=0.017; 95% CI=0-0.033; P=0.047]. Conclusion: This investigation of GHI characteristics suggests explanations for varied ametropia in keratoconic eyes and has potential significance as a reference for promoting pre-surgical planning and technology for corneal transplantation.

scholarly journals Corneal transplantation for keratoconus

2013 ◽  
Vol 6 (1) ◽  
pp. 1-8
Author(s):  
Sven Jonuscheit ◽  
Michael J Doughty
Keyword(s):  
Corneal Transplantation ◽  
Surgical Techniques ◽  
Lamellar Keratoplasty ◽  
Deep Anterior Lamellar Keratoplasty ◽  
Corneal Transplant ◽  
Eye Care ◽  
Primary Eye Care ◽  
Transplant Centre ◽  
Anterior Lamellar Keratoplasty

This review discusses current techniques of corneal transplantation for moderate to advanced keratoconus. The aim of this article is to provide primary eye care practitioners with an overview of two current corneal transplantation modalities, namely penetrating and deep anterior lamellar keratoplasty. The main surgical techniques, the outcome of the procedures, common complications and the prognosis will be reviewed. Results from ongoing studies at a corneal transplant centre in Glasgow, Scotland will be discussed. 

scholarly journals Glaucoma and Corneal Transplant Procedures

2012 ◽  
Vol 2012 ◽  
pp. 1-9 ◽  
Author(s):  
Ammar M. Al-Mahmood ◽  
Samar A. Al-Swailem ◽  
Deepak P. Edward
Keyword(s):  
Intraocular Pressure ◽  
Treatment Options ◽  
Corneal Transplantation ◽  
Lamellar Keratoplasty ◽  
Deep Anterior Lamellar Keratoplasty ◽  
Corneal Transplant ◽  
Anterior Lamellar Keratoplasty ◽  
Intraocular Pressure Elevation ◽  
High Incidence ◽  
Number Of Treatment

Glaucoma after corneal transplantation is a leading cause of ocular morbidity after penetrating keratoplasty. The incidence reported is highly variable and a number of etiologic factors have been identified. A number of treatment options are available; surgical intervention for IOP control is associated with a high incidence of graft failure. IOP elevation is less frequently seen following deep anterior lamellar keratoplasty. Descemet's striping-automated endothelial keratoplasty is also associated with postprocedure intraocular pressure elevation and secondary glaucoma and presents unique surgical challenges in patients with preexisting glaucoma surgeries. Glaucoma exists in up to three-quarters of patients who undergo keratoprosthesis surgery and the management if often challenging. The aim of this paper is to highlight the incidence, etiology, and management of glaucoma following different corneal transplant procedures. It also focuses on the challenges in the diagnosis of glaucoma and intraocular pressure monitoring in this group of patients.

scholarly journals The ABCD of DALK

2017 ◽  
Vol 10 (02) ◽  
pp. 93
Author(s):  
Jod Mehta ◽  
Keyword(s):  
Corneal Transplantation ◽  
Lamellar Keratoplasty ◽  
Deep Anterior Lamellar Keratoplasty ◽  
Endothelial Keratoplasty ◽  
Anterior Lamellar Keratoplasty

Corneal transplantation has changed rapidly over the last 20 years. In spite of the fact that endothelial keratoplasty has become the most dominant procedure, deep anterior lamellar keratoplasty (DALK), the anterior lamellar procedure, has not become so popular. There are several reasons for this, and this editorial will attempt to give some useful tips, that may not be found in papers or books, in the hope of helping people performing or starting off on their DALK journey.

scholarly journals Senegalese Experience of Keratoplasties in Public and Private Practice (Aristide le Dantec Hospital and Clinique du Cap), Dakar

2019 ◽  
Vol 3 (3) ◽  
Keyword(s):  
Corneal Transplantation ◽  
Primary Objective ◽  
Lamellar Keratoplasty ◽  
Deep Anterior Lamellar Keratoplasty ◽  
Small Scale ◽  
Bullous Keratopathy ◽  
Public And Private ◽  
Anterior Lamellar Keratoplasty ◽  
Corneal Transplants ◽  
One Year

Introduction: The corneal surface is exposed to many external aggressions which can alter its transparency and regularity. Several keratoplasty techniques have been codified such as transfixing keratoplasty (KT) or deep anterior lamellar keratoplasty (KLAP) or rotational auto graft. Our work was carried out in Senegal, in a context where corneal transplants were not frequently performed. The aim of our study was to report results one year after a keratoplasty. Patients and Method: We conducted a retrospective study of patients who underwent prior transfixing or lamellar keratoplasty or rotational auto graft. It was carried out between the ophthalmological clinic Aristide Le Dantec hospital and the ophthalmological center of Clinique du Cap in Dakar, Senegal. It included all records of patients with postoperative follow-up greater than or equal to 1 year. The parameters studied were anamnestic data, preoperative clinical examination (indication of corneal transplantation) and postoperative results. Results: Twenty-six eyes of 22 patients were identified. The decrease in visual acuity evolved on average for 5 years. The operative indications were mainly represented by results of pillow type keratitis (30.76%), bullous keratopathy (23.08%), and keratoconus (23.08%). Surgery consisted of a transfixing keratoplasty in 84.61%, lamellar in 15.39% of the cases. Comments: The primary objective of keratoplasty is to restore the transparency, it can also help to remedy pain or improve the anatomical and aesthetic prognosis of the eyeball. The benefits of keratoplasty in our context of developing countries are the reduced cost and shorter waiting time vis-à-vis western countries. Conclusion: The results of our study are encouraging for surgery, still practiced on a small scale in our regions.

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