International Journal of Keratoconus and Ectatic Corneal Diseases

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2020 | January-June | Volume 9 | Issue 1

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Original Article

Rafael Melián, Vicente Rodriguez, Humberto Carreras, David P Piñero Llorens, Jesús M Gonzalez-Martín, Francisco Galván

Long-term Analysis of Epi-ON Corneal Collagen Cross-linking Outcomes in Corneal Ectasia

[Year:2020] [Month:January-June] [Volume:9] [Number:1] [Pages:6] [Pages No:1 - 6]

Keywords: Corneal collagen cross-linking, Corneal ectasia, Corneal tomography, Keratoconus, Pellucid marginal degeneration, Post-LASIK ectasia

   DOI: 10.5005/jp-journals-10025-1187  |  Open Access |  How to cite  | 


Aim and objective: To evaluate the 3-year follow-up clinical outcomes obtained in corneal ectasia using Epi-ON corneal collagen cross-linking (CXL). Materials and methods: This study is a retrospective study enrolling 46 eyes from 32 patients with progressive corneal ectasia and treated with Epi-ON CXL in the period from September 2012 to April 2016. Two groups were differentiated according to the type of corneal ectasia: ectasia post-LASIK group (EPL, 12 eyes) and primary ectasia group (34 eyes). Two different platforms were used for the surgical protocol: VEGA CBM X LINKER platform (CSO, Firenze, Italy) and KXL (Avedro, Waltham, Massachusetts, USA). Visual, refractive, and corneal tomographic outcomes were evaluated during a 3-year follow-up. Results: A statistically significant improvement in the logMAR corrected distance visual acuity (CDVA) was observed in the whole sample (p <0.001) during the follow-up, with half of the sample improving one or more lines of CDVA. Likewise, only significant changes were detected in steepest keratometry (p <0.001), corneal astigmatism (p = 0.012), and index of height asymmetry (p = 0.021), with a trend to increase. Regarding the comparison between groups, more significant improvement in CDVA was found in the EPL group compared to the primary ectasia group (−0.07 ± 0.09 vs −0.15 ± 0.14, p = 0.028). Likewise, a significant trend to more corneal thinning was observed in primary ectasia group (p = 0.034). Conclusion: Epi-ON CXL is efficacious for stabilizing the progression of primary and iatrogenic ectasias for most cases, with significant improvement of visual acuity associated.



Jörg H Krumeich, Nino Hirnschall, Florian Laufer

Femtosecond Circular Keratotomy in Stage I and II Keratoconus

[Year:2020] [Month:January-June] [Volume:9] [Number:1] [Pages:4] [Pages No:7 - 10]

Keywords: Circular corneal cut, Circular keratotomy, Keratoconus

   DOI: 10.5005/jp-journals-10025-1188  |  Open Access |  How to cite  | 


Aim and objective: Authors present clinical results of circular keratotomy (CTK) performed with a femtosecond laser. The 1–3 years results for two separate groups are the first results of a new surgical approach to stop progression of keratoconus. Materials and methods: A circular intraparenchymal central 7-mm cut was made with a Ziemer Z6, Z8 femtosecond laser without affecting the epithelium, Bowman layer, Descemet layer and endothelium. Patients with stage I and II keratoconus according to the Krumeich classification were treated. Results: In total 147 eyes were treated. Statistical evaluation showed stable radii in 87.8% (n = 48) of the eyes treated. Astigmatism was stable in 79.6% (n = 69). BCVA remained stable or improved in 75.9% (n = 29). Conclusion: CKT is a procedure that reliably works in keratoconus stage I and II. Advantages are the ease of the performance using the femtosecond laser and the absence of any severe complication in the eyes treated. The eye remained closed with the consequence that no contact existed from the external world into the cornea.



Ananda Kudari, BR Sumangala

Terrien's Marginal Degeneration: An Uncommon Eye Disease and Treatment Modalities

[Year:2020] [Month:January-June] [Volume:9] [Number:1] [Pages:2] [Pages No:11 - 12]

Keywords: Cornea, Degeneration, Keratoconus, Keratoplasty, Slit lamp

   DOI: 10.5005/jp-journals-10025-1189  |  Open Access |  How to cite  | 


Outermost layer of the eye is cornea which is very clear and transparent. Many diseases can affect the cornea in that Terrien's marginal degeneration is also one of them, and their causes are unknown. But it is associated with other diseases like scleritis, episcleritis, and keratoconus, and this condition can be diagnosed by corneal topography, slit-lamp microscopy, ultrasound biomicroscopy, and scanning-slit topography. Medically, it can be treated by contact lenses and surgically by different types of keratoplasties.



Pedro Manuel Baptista, Rui C Freitas, Nelson Sena Jr, Fernando F Correia, Renato Ambrosio Jr

Very Asymmetric Keratoconus: A Case Report of Long-term Follow-up

[Year:2020] [Month:January-June] [Volume:9] [Number:1] [Pages:7] [Pages No:13 - 19]

Keywords: Corneal cross-linking, Eye rubbing, Keratoconus, Very asymmetric disease, Violet June

   DOI: 10.5005/jp-journals-10025-1190  |  Open Access |  How to cite  | 


Background: Although classically described as a noninflammatory disease, recent evidence suggests that chronic inflammation may play a role in keratoconus pathophysiology. Continued eye rubbing is well established on ectasia development, taking part in the “two-hit” hypothesis, which combines the concept of intrinsic biomechanical susceptibility and external stimuli decompensating the structural integrity of the cornea. Aim and objective: To describe a case of very asymmetric bilateral keratoconus requiring different approaches on both eyes. Case description: A 14-year-old patient with a history of atopy and intense eye rubbing was referred for a cornea consultation because of low vision accompanied with pruritus on both eyes and pain on the left eye. Corneal hydrops was present on the left eye. A diagnosis of grade II keratoconus was established for the right eye. Distance-corrected visual acuity (DCVA) was 20/70 in the right eye and count fingers at 1 meter in the left eye. After 1 month of avoiding eye rubbing, topical mild steroids, and anti-allergic prescription, the patient referred significant improvement in both eyes. DCVA was 20/30, and flattening of 2.5D in the anterior curvature was noted in the right eye. Three months later, ectasia progression was detected in the right eye, and transepithelial cross-linking was performed. More than 6 years after the procedure, the right eye maintained visual and tomographic stability and the left eye had resolution of edema with residual stromal scar. DCVA was 20/30 in the right eye and 20/40 in the left eye. Anterior corneal curvature maintained stable, with a overall decrease of 2.1D in the right eye and 40.9D in the left eye. Conclusion: In the presented clinical case, avoidance of eye rubbing and topical anti-inflammatory drugs were followed by spontaneous partial regression of the ectasia, delaying the necessity for a more invasive treatment. When progression was documented, transepithelial corneal cross-linking was effective in halting disease progression, with topographic stability for more than 6.5 years follow-up period. Clinical significance: Avoidance of deleterious factors like eye rubbing in keratoconic eyes is crucial for clinical stabilization or even improvement. Anti-inflammatory therapy may play a role in ectasia stability.



Jacob D Grodsky, Sean Edelstein

Sterile Corneal Perforation Following Corneal Collagen Cross-linking in a Patient with Down Syndrome

[Year:2020] [Month:January-June] [Volume:9] [Number:1] [Pages:3] [Pages No:20 - 22]

Keywords: Acute hydrops, Corneal collagen cross-linking, Corneal collagen cross-linking complications, Down syndrome, Keratoconus, Perforation

   DOI: 10.5005/jp-journals-10025-1191  |  Open Access |  How to cite  | 


Aim and objective: To report a case of a patient with Down syndrome (DS) and keratoconus who experienced acute hydrops in one eye as well as sterile perforation requiring emergent tectonic penetrating keratoplasty following epithelium-off corneal collagen cross-linking (CXL) in the fellow eye. Background: Keratoconus is a progressive, bilateral, corneal ectasia in which there is stromal thinning and apical protrusion of the cornea resulting in irregular astigmatism. This condition has been shown to have a disproportionately high association with DS. We describe a unique case that highlights this potentially rapidly progressive disease and its manifestations. Case description: An 18-year-old male with history of DS presented with bilateral floppy eyelid syndrome and corneal ectasia as well as acute hydrops of the right eye (OD). Medical management included 5% sodium chloride drops, prophylactic antibiotic ointment, and counseling against eye rubbing and to wear a Fox shield when sleeping. Worsening hydrops OD prompted intracameral injection of 20% SF6 gas which hastened resolution of edema within 3 weeks. Residual severe corneal scarring, however, limited visual improvement to light perception. Meanwhile, progressive ectasia of the left eye (OS) prompted epithelium-off CXL procedure. Sterile corneal perforation was noted 4 days following uneventful surgery, necessitating emergency tectonic penetrating keratoplasty and temporary tarsorrhaphy. Conclusion: This case illustrates that patients with DS and keratoconus are at especially high risk for rapidly progressive disease and for surgery-related complications. Clinical significance: Patients with keratoconus, especially younger patients that can experience rapid progression, need to be closely monitored with early intervention but can still experience uncommon complications. Similar at-risk patients may be considered instead for investigational non-FDA-approved epithelium-on cross-linking, which should intuitively be associated with a lower risk of corneal perforation.


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