International Journal of Keratoconus and Ectatic Corneal Diseases

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2018 | July-December | Volume 7 | Issue 2

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Original Article

Maria A Henriquez, Nadia Canorio, Carmen Maldonado, Mirel Rincon, Luis Izquierdo Jr

Snake-like Chromatin Cell Protein as an Indicator of Chronic Eye Rubbing in Patients with Keratoconus and Allergic Conjunctivitis

[Year:2018] [Month:July-December] [Volume:7] [Number:2] [Pages:5] [Pages No:91 - 95]

Keywords: Conjunctivitis, Eye, Habit, Keratoconus, Rubbing

   DOI: 10.5005/jp-journals-10025-1163  |  Open Access |  How to cite  | 


Purpose: To evaluate the presence of snake-like chromatin (SLC) cell protein (related to eye rubbing) in patients with keratoconus or allergic conjunctivitis and in controls. Materials and methods: Prospective experimental study included 193 eyes of 193 patients between January 2015 and July 2016; 86 had a diagnosis of new keratoconus, 88 were controls, and 19 had a diagnosis of allergic conjunctivitis and underwent impression cytology (IC). Specimens were stained with a combination of periodic acid-Schiff (PAS) and Gill's modified papanicolaou stains to detect the presence of SLC cell protein and morphological characterization of dry eye and allergic conjunctivitis. A questionnaire regarding eye-rubbing habits was administered. Results: Of the patients, 56.97% (49/86 eyes) in the keratoconus group, 84.21% (16/19 eyes) in the allergy group, and 10.11% (9/89 eyes) in the control group reported rubbing their eyes. The SLC was detected in 24.41% (21/86), 73.68% (14/19), and 3.37% (3/89) of the keratoconus, allergy, and control groups respectively, who reported rubbing their eyes. In contrast, SLC was detected in 2.32% (2/86), 0% (0/19), and 1.12% (1/89) of the keratoconus, allergy, and control groups respectively, who reported not rubbing their eyes. Conclusion: The presence of SLC cells was higher in keratoconus, allergy, and control group patients who reported rubbing their eyes. Keratoconus is independent of eye-rubbing habits and the presence of SLC cells.


Original Article

Chintan Malhotra, Balamurugan Ramatchandirane, Amit Gupta, Deepika Dhingra

Transepithelial Collagen Cross-linking versus Contact Lens-assisted Collagen Cross-linking for Progressive Keratoconus with Borderline Corneal Thickness: A Prospective Comparative Study

[Year:2018] [Month:July-December] [Volume:7] [Number:2] [Pages:9] [Pages No:96 - 104]

Keywords: Cornea, Corneal collagen crosslinking, Ectasia, Keratoconus, Riboflavin

   DOI: 10.5005/jp-journals-10025-1164  |  Open Access |  How to cite  | 


Aim: To compare outcomes of transepithelial collagen crosslinking (TECXL) and contact lens-assisted collagen crosslinking (CACXL) for progressive keratoconus with borderline corneal thickness. Materials and methods: In this prospective, comparative, interventional series, twenty eyes with progressive keratoconus and a preoperative ‘epithelium on’ minimal corneal thickness (MCT) of 350-420 μm, were randomized to undergo TECXL (n = 11 eyes) or CACXL (n = 9 eyes) using hydroxypropyl methylcellulose (HPMC) based 0.25% and 0.1% riboflavin respectively. Primary outcomes evaluated were demarcation line depth on anterior segment optical coherence tomography (ASOCT) 1 month postoperatively: change in maximum keratometry (Kmax) and endothelial cell density (ECD) at 6 months from baseline. Postoperative pain in the first 4 days and haze at 6 months were also compared between the two techniques. Results: Preoperative ‘epithelium on’ MCT in the TECXL (394.3 ± 12.6 μm)and CACXL (385.6 ± 13.8 μm) groups was comparable (p = 0.15); mean demarcation line depth was 74.6% (294.4 ± 57.1 μm) and 80% (308.2 ± 84.2 μm) respectively (p = 0.66). Regression (reduction of Kmax by >1 diopter) or stabilization (change in Kmax of < ± 1D) was seen in 91% (n = 10) and 89% (n = 8) eyes of the TECXL and CACXL groups, respectively. ECD at baseline and last follow up was comparable in each group (p >0.05). Postoperative pain in the first four days and haze at 6 months post the CXL procedure were also comparable (all p's >0.05). Conclusion: Using HPMC riboflavin, both TECXL and CACXL had good clinical efficacy and equivalent patient comfort without compromising endothelial safety, in keratoconus patients with thin corneas. Clinical significance: TECXL and CACXL are simple, costeffective techniques to arrest progression in keratoconus patients with borderline corneal thickness and may decrease the need for future surgical interventions like lamellar or penetrating keratoplasty.


Original Article

Marco Abbondanza, Gabriele Abbondanza, Valentina De Felice

Long-term Results of Mini Asymmetric Radial Keratotomy for the Treatment of Keratoconus

[Year:2018] [Month:July-December] [Volume:7] [Number:2] [Pages:5] [Pages No:105 - 109]

Keywords: Keratoconus, MARK, Mini asymmetric radial keratotomy

   DOI: 10.5005/jp-journals-10025-1165  |  Open Access |  How to cite  | 


Aim: To present long-term results of mini asymmetric radial keratotomy (MARK) for the treatment of mild to moderate keratoconus, with an average follow-up of 7.3 years and a minimum of 6 years. Design: Retrospective clinical study. Materials and methods: The postoperative outcomes of 66 eyes (45 patients) were selected and retrospectively analyzed. Inclusion criteria were progressive stages I and II keratoconus (Kc) and contact lens intolerance, while exclusion criteria were advanced stages of the pathology and chronic or recurrent ocular infections. Tomography- and topography-guided miniincisions were customised and performed sectorally with a calibrated diamond knife and custom-made corneal markers. Results: After a minimum follow-up of 6 years, mean keratometry improved in 91% of cases (from 47.25 D to 44.61 D), and mean pachymetry improved in 98% of cases (from 446 μm to 484 μm), while best spectacle-corrected visual acuity improved for 95% of patients, from 0.23 LogMAR (5.9/10) to 0.095 LogMAR (7.7/10). Conclusion: MARK improved both mean keratometry and mean pachymetry, thus halting the progression of keratoconus, while improving visual acuity. The long-term results suggest that MARK should be considered as one of the conservative treatments for moderate to mild progressive keratoconus.


Original Article

Arthur B Cummings, Parker J Shaw, Gabrielle Kelly

Epithelium-on Corneal Cross-linking for Progressive Keratoconus: Two-year Outcomes

[Year:2018] [Month:July-December] [Volume:7] [Number:2] [Pages:5] [Pages No:110 - 114]

Keywords: Accelerated corneal cross-linking, Blurred vision, Corneal cross-linking, Corneal ectasia, Corneal tomography, Corneal topography, Corneal transplant, Eye rubbing, Femtosecond- laser assisted DALK, Keratoconus, Steep corneas, Quality of vision, Vogt's striae

   DOI: 10.5005/jp-journals-10025-1166  |  Open Access |  How to cite  | 


Corneal cross-linking (CXL) has been established as a successful treatment tool for the treatment of progressive keratoconus in terms of slowing or halting progressive corneal steepening and thinning and even on some occasions, reversing the steepening. To date the Dresden epithelium-off protocol is regarded as the gold standard and the epithelium-on (epi-on) approaches have met with less success. Both doctors and patients would welcome an epi-on CXL procedure that provided good outcomes as the morbidity with epi-on CXL is so much less and the safety is enhanced. Patient comfort is greater with the epi-on techniques when compared to epi-off. This study looked at 82 eyes that had documented progression of keratoconus and then underwent epi-on CXL using the CXLO system. The results show that corneal steepening can be halted and even reversed over a 2-year follow-up period with no complications noted. Over the 24 months post treatment on average there was a decrease in all keratometry values, BAD and ISV when compared to before treatment with IHD being marginally increased. Further studies over a longer follow-up period are required but recent publications using the same approach are validating the findings seen in this study.



Marcella Q Salomão, Ana L Höfling-Lima, Fernando Faria-Correia, Bernardo T Lopes, Daniel G Dawson, Renato Ambrósio

Mild Keratoconus in the Mother of a Patient with Down Syndrome: Case Report and Clinical Hypothesis

[Year:2018] [Month:July-December] [Volume:7] [Number:2] [Pages:6] [Pages No:115 - 120]

Keywords: Corneal tomography, Corneal topography, Down syndrome, Forme fruste keratoconus, Keratoconus

   DOI: 10.5005/jp-journals-10025-1167  |  Open Access |  How to cite  | 


The purpose of this study is to report a case of mild or forme fruste keratoconus (FFKC) in the mother of a patient with Down syndrome (DS) who presented for cataract surgery and to propose a possible association of mothers with mild forms of ectasia and higher risk for delivering babies with DS. Advanced corneal imaging including corneal topography, corneal tomography, and the integrated corneal tomography and biomechanical assessment allowed the identification of a mild form of keratoconus in both eyes of this patient, and the correct characterization of the corneal structure and whole optics of the eye were crucial for IOL selection and planning cataract surgery.



Dana Garzozi, Hanna J Garzozi

Post-femtosecond-assisted Laser-assisted In Situ Keratomileusis Ectasia in a Hyperopic Eye

[Year:2018] [Month:July-December] [Volume:7] [Number:2] [Pages:7] [Pages No:121 - 127]

Keywords: Astigmatism, Ectasia, Hyperopia, Laser, LASIK

   DOI: 10.5005/jp-journals-10025-1168  |  Open Access |  How to cite  | 


We report on a case of post-femtosecond-assisted laserassisted in situ keratomileusis (LASIK) (FA-LASIK) corneal ectasia, which developed in a young hyperopic male who underwent surgery in both eyes (BE) and eighteen month later, an enhancement procedure in his left eye (LE) due to residual hyperopia.   Ten years later, the patient complained about deterioration of his visual acuity (VA) in his LE. An examination of the eyes and imaging of the cornea revealed post-LASIK ectasia in his LE.   Post-LASIK ectasia in hyperopic eyes has already been reported in few cases, but this is the first report on post-FALASIK ectasia in a hyperopic eye.



Ramon J Hallal Jr, Renato Ambrósio

Importance of Screening for Ectatic Corneal Disease Prior to Multifocal Intraocular Lens

[Year:2018] [Month:July-December] [Volume:7] [Number:2] [Pages:6] [Pages No:128 - 133]

Keywords: Cataract surgery, Screening, Subclinical keratoconus

   DOI: 10.5005/jp-journals-10025-1169  |  Open Access |  How to cite  | 


Aim: This article reports the case of a patient with severe symptoms related to bad quality of vision after toric multifocal intraocular lens (IOL) that was diagnosed with mild keratoconus. Materials and methods: To present a case report and prospectively review of the literature, considering the relevance of screening ectaticconreal disease prior to refractive cataract (or lens) surgery. Results: The patient was dissatisfied, seeking a second opinion after the implantation of a toric multifocal IOL in the left eye. The IOL was properly centered and surgery had no complications. The preoperative refraction was −4.00 − 1.50 × 160, giving 20/40. Patient denied any history of keratoconus or transplant in his family. After cataract surgery, uncorrected distance vision acuity (UDVA) was 20/60, and J4 for near. Manifest refraction was + 2.00 − 0.50 × 130, giving 20/30. Corneal topography, tomography, and biomechanical assessments indicated the diagnosis of mild keratoconus. The patient was advised for IOL exchange, which was successfully done for a monofocal aspheric IOL. The patient was satisfied with the final result, presenting a final UDVA of 20/25, J3, and manifest refraction of +0.75 − 0.50 × 105, giving 20/20. Conclusion: Screening for corneal abnormalities including ectatic corneal disease is fundamental prior to the indication of a premium lens. Advanced corneal imaging plays a fundamental role to help select candidates for multifocal IOLs, as for individualized planning of refractive cataract surgery.



James McKelvie, Cameron Andrew McLintock, Samer Hamada

Coincident Pellucid Marginal Degeneration and Fuchs’ Endothelial Dystrophy

[Year:2018] [Month:July-December] [Volume:7] [Number:2] [Pages:3] [Pages No:134 - 136]

Keywords: Ectasia, Fuchs endothelial dystrophy, Pellucid marginal degeneration

   DOI: 10.5005/jp-journals-10025-1170  |  Open Access |  How to cite  | 


Aim: We report a rare case of coincident pellucid marginal degeneration and Fuchs' endothelial dystrophy. Background: As far as the authors are aware there have been no previous reports of this combination of corneal disorders in the same patient. Case description: A 45-year-old woman presented with progressive pellucid marginal degeneration and Fuchs' endothelial dystrophy. Progressive changes in corneal topography and specular microscopy imaging have been documented over 13 years since presentation. Declining vision has been successfully managed in this patient, to date, with serial crescentic corneal wedge excision biopsies to maintain acceptable spectacle-corrected visual acuity. Conclusion: This rare combination of corneal disorders presents an interesting and unique challenge for surgical management in the future, where corneal decompensation and cataract are likely to become limiting factors for visual acuity. Clinical significance: This is the first report of coincident pellucid marginal degeneration and Fuchs' endothelial dystrophy.


Surgical Technique

Andrea Romani, Alessandro Burroni

Pachymetry-based Accelerated Crosslinking: The “M Nomogram” for Standardized Treatment of All-thickness Progressive Ectatic Corneas

[Year:2018] [Month:July-December] [Volume:7] [Number:2] [Pages:8] [Pages No:137 - 144]

Keywords: Crosslinking, Keratoconus, M nomogram, Pachymetry, Thin corneas

   DOI: 10.5005/jp-journals-10025-1171  |  Open Access |  How to cite  | 


Purpose: To assess the safety and efficacy of a new customized epithelium-off accelerated crosslinking (ACXL) nomogram “M nomogram” based on preoperative corneal optical thinnest point for progressive keratoconus and iatrogenic corneal ectasia. Methods: Comparative analysis including the measured depths of the demarcation lines by in vivo confocal microscopy (IVCM) and corneal OCT in 20 eyes treated with conventional 3 mW/cm2 CXL, 20 eyes treated with 30 mW/cm2 ACXL with continuous (10 eyes) and pulsed (10 eyes) UV-A exposure (1 sec on, 1 sec off), 20 eyes treated with 15 mW/cm2 pulsed light ACXL and 20 eyes using the 9 mW/cm2 ACXL protocol. IVCM was performed by the HRT II Rostock Cornea Module (Heidelberg, Germany) and corneal OCT by the OptoVue (Freemont, Irvine, USA). The mathematical cross-linking profile was determined according to a calculated depth of the demarcation line and the threshold cross-link concentration adopting the conventional 3 mW/cm2 protocol as a benchmark. Results: The average demarcation depths were 350 ± 50 μm for the 3 mW/cm2 conventional protocol, 200 ± 50 μm for the 30 mW/cm2 continuous light ACXL, 250 ± 50 μm for the 30 mW/cm2 pulsed light ACXL and 280 ± 30 μm for the 15 mW/cm2 pulsed light ACXL. There was a very high correlation between the depth of the demarcation line between the measured and calculated data with a slope of m = 1.03 and an R2 value 0.73. Conclusion: ACXL M nomogram allows safe and efficacious CXL parameters setting based on preoperative minimum corneal thickness also including a more standardized treatment of thin ectatic corneas between 250 μm and 400 μm.


Surgical Technique

Sandro Coscarelli, Leonardo Torquetti

Surgical Management of Type II Big Bubble in Deep Anterior Lamellar Keratoplasty

[Year:2018] [Month:July-December] [Volume:7] [Number:2] [Pages:6] [Pages No:145 - 150]

Keywords: Deep anterior lamellar keratoplasty, Dua's layer, Type II big bubble

   DOI: 10.5005/jp-journals-10025-1172  |  Open Access |  How to cite  | 


Purpose: To present some simple surgical maneuvers to manage a type 2 big bubble (BB) in deep anterior lamellar keratoplasty (DALK). Methods: Four DALK procedures with type 2 or mixed BB were performed. One was in the right eye of a 25-year-old female. The corrected distance visual acuity (CDVA) was 20/200 due to advanced keratoconus. The second case was in a 28-year old male with central-anterior stromal scarring and neovascularization due to infectious keratitis and CDVA <20/400. The third case was in a 20-year-old male with advanced keratoconus and CDVA <20/400. The fourth case was a 49-year-old female, with high irregular astigmatism due to radial keratotomy and her CDVA was 20/150. Results: At the last follow-up at 12 months, the first case of advanced keratoconus had a clear graft with CDVA = 20/50 and some Descemet's folds that were in the visual axis, corresponding to the are of type 2 BB that was not deflated intraoperatively. The second case achieved a CDVA = 20/40, without folds at the visual axis at 12 months of follow-up. In the third and fourth patients with mixed BB where DL was retained, the CDVA was 20/25 and 20/30, respectively. No intraoperative DM perforation or postoperative complications were noted. Conclusion: Type 2 BB in DALK can be properly managed in order to avoid perforation and to attain a good visual outcome.


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