scholarly journals Iridocorneal endothelial syndrome in a patient with keratoconus – a case report

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Michele De Maria ◽  
Danilo Iannetta ◽  
Antonio Moramarco ◽  
Luigi Fontana

Abstract Background To describe a case of a rare association of bilateral keratoconus and unilateral essential iris atrophy and to conduct a literature review of the current strategies of treatment of the corneal disease and glaucoma in patients with Iridocorneal Endothelial Syndrome (ICE). Case presentation We report a rare association of bilateral keratoconus and unilateral essential iris atrophy in a 38-year-old man. Diagnosis of bilateral keratoconus was confirmed by corneal topography. Slit-lamp examination showed extensive iris atrophy with corectopia and policoria in one eye. Corneal specular microscopy revealed an abnormal endothelium morphology in the same eye with extensive peripheral anterior synechiae and closure of the drainage angle at gonioscopy. Intraocular pressure was 26 mmHg, despite maximal topical therapy. Optic disc examination showed severe glaucomatous cupping. Surgery by glaucoma drainage device implantation was performed. Conclusion Essential iris atrophy is a rare clinical variant of ICE syndrome characterized by profound anatomical alterations of the anterior segment associated with corneal edema and secondary glaucoma. In these patients, selective keratoplasties have replaced penetrating keratoplasty to treat corneal decompensation and glaucoma drainage devices are preferred to conventional trabeculectomy for the treatment of secondary glaucoma.

2021 ◽  
Vol 8 (12) ◽  
pp. 1873
Author(s):  
Usha K. Raina ◽  
Shruti Bhattacharya ◽  
Prateeksha Sharma ◽  
Varun Saini

Parry-Romberg syndrome (PRS) is a rare disorder which causes progressive hemifacial atrophy, with ocular manifestations like hypotony, enophthalmos and corneal edema on the ipsilateral atrophic side. This is a report of a unique case of PRS with contalateral manifestations like ectropion uvea, correctopia and endothelial deposits, along with polymegathism and pleomorphism seen on specular microscopy suggestive of Iridocorneal Endothelial (ICE) Syndrome. ICE syndrome and PRS have not been reported together in any literature so far. This case highlights the importance of a thorough glaucoma workup and corneal examination on the atrophic facial side as well as on the apparently normal side in all cases of PRS.


Author(s):  
Benchakroun S ◽  
◽  
Taouri N ◽  
Tagmouti A ◽  
Cherkaoui LO ◽  
...  

We report a case of a 42-year-old-woman, who presented to the ophthalmic consultation for decreased visual acuity complaints of blurred vision, altered pupillary shape since few months of her right eye. The clinical examination found a reduced visual acuity to counting fingers in the right eye and 20/20 in the left eye. intraocular pressures was 38 mm Hg OD and 14 mm Hg OS. Slit lamp examination of the right eye found: Corneal edema, iris atrophy with a deformation of the iris architecture and pupillary anomalies, with polycoria (Figure 1). The evaluation of the angle by gonioscopy found areas of broad synechiae anterior to Schwalbe’s line (Figure 2). While the examination of the left eye was normal (Figure 1B). The posterior segment examination was normal in both eyes. Specular microscopy confirmed the presence of unilateral endothelial pleomorphism and polymegathism. In our case of the retained diagnosis was iridocorneal endothelial syndrome


2015 ◽  
Vol 2015 ◽  
pp. 1-12 ◽  
Author(s):  
Su-Ho Lim

Anterior segment optical coherence tomography (AS-OCT) was recently developed and has become a crucial tool in clinical practice. AS-OCT is a noncontact imaging device that provides the detailed structure of the anterior part of the eyes. In this review, the author will discuss the various clinical applications of AS-OCT, such as the normal findings, tear meniscus measurement, ocular surface disease (e.g., pterygium, pinguecula, and scleromalacia), architectural analysis after cataract surgery, post-LASIK keratectasia, Descemet’s membrane detachment, evaluation of corneal graft after keratoplasty, corneal deposits (corneal dystrophies and corneal verticillata), keratitis, anterior segment tumors, and glaucoma evaluation (angle assessment, morphological analysis of the filtering bleb after trabeculectomy, or glaucoma drainage device implantation surgery). The author also presents some interesting cases demonstrated via AS-OCT.


2021 ◽  
Vol 14 (2) ◽  
pp. e240988
Author(s):  
Sunita Chaurasia ◽  
Sirisha Senthil ◽  
Nikhil Choudhari

To report the outcomes of endothelial keratoplasty (EK) combined with near total iridectomy in the management of iridocorneal endothelial (ICE) syndrome with severely disorganised anterior segment. Three patients with ICE syndrome, who had a severely disorganised anterior segment underwent near total iridectomy, with/without cataract surgery, followed by EK at the same time. Mean age was 35 years. Prior to EK, the intraocular pressure (IOP) was in the range of 12–15 mm Hg. One patient (patient 2) had advanced disc damage prior to EK. Two eyes (patients 1 and 2) had a glaucoma drainage device, and in one, the IOP was controlled with two antiglaucoma medications. All grafts were clear, and IOP was well controlled till the last mean follow-up of 53 (range 30–72) months. The outcomes of EK with this surgical approach are favourable and should be considered in selective cases of ICE syndrome.


2021 ◽  
pp. 112067212110678
Author(s):  
Navjot Singh Ahluwalia ◽  
Rakesh Shakya ◽  
Dhairya Parikh ◽  
Devindra Sood

Purpose To report a case of unilateral Iridocorneal endothelial (ICE) syndrome- Progressive iris atrophy (PIA) with an overlapping chronic angle closure glaucoma (CACG) and to highlight the effect of bilateral Laser peripheral iridotomy (LPI) in such a co-occurrence. Case description A patient presented to us with bilateral gradual painless progressive diminution of vision. Both eyes (BE) had a clear cornea, shallow peripheral anterior chamber depth, grade 2 nuclear sclerosis, raised intraocular pressure and glaucomatous optic neuropathy. In addition, the Left eye (LE) had an irregular anterior chamber, peripheral anterior synechiae (PAS) extending to cornea, patchy iris atrophy, subtle corectopia and a low endothelial cell count on specular microscopy. Indentation gonioscopy led to the diagnoses of CACG BE with ICE syndrome- PIA LE. LPI was performed bilaterally. On Anterior Segment Optical Coherence Tomography (ASOCT), there was evident widening of the angle away from PAS in the Right eye as well as in the LE with PIA post LPI. Conclusion This is a unique case of unilateral PIA with an associated CACG in BE. It is the first case demonstrating the effect of bilateral LPI in such a case scenario. Though not indicated in ICE syndrome, LPI did show short term evidence of significant widening of the angle away from areas of PAS even in the eye with PIA having a limited high PAS and a concurrent primary (chronic) angle closure disease.


2015 ◽  
Vol 2015 ◽  
pp. 1-9 ◽  
Author(s):  
Marta Sacchetti ◽  
Flavio Mantelli ◽  
Marco Marenco ◽  
Ilaria Macchi ◽  
Oriella Ambrosio ◽  
...  

The iridocorneal endothelial (ICE) syndrome is a rare ocular disorder that includes a group of conditions characterized by structural and proliferative abnormalities of the corneal endothelium, the anterior chamber angle, and the iris. Common clinical features include corneal edema, secondary glaucoma, iris atrophy, and pupillary anomalies, ranging from distortion to polycoria. The main subtypes of this syndrome are the progressive iris atrophy, the Cogan-Reese syndrome, and the Chandler syndrome. ICE syndrome is usually diagnosed in women in the adult age. Clinical history and complete eye examination including tonometry and gonioscopy are necessary to reach a diagnosis. Imaging techniques, such as in vivo confocal microscopy and ultrasound biomicroscopy, are used to confirm the diagnosis by revealing the presence of “ICE-cells” on the corneal endothelium and the structural changes of the anterior chamber angle. An early diagnosis is helpful to better manage the most challenging complications such as secondary glaucoma and corneal edema. Treatment of ICE-related glaucoma often requires glaucoma filtering surgery with antifibrotic agents and the use of glaucoma drainage implants should be considered early in the management of these patients. Visual impairment and pain associated with corneal edema can be successfully managed with endothelial keratoplasty.


2021 ◽  
Vol 10 (6) ◽  
pp. 1324
Author(s):  
Cosimo Mazzotta ◽  
Marco Ferrise ◽  
Guido Gabriele ◽  
Paolo Gennaro ◽  
Alessandro Meduri

The purpose of this study was to evaluate the effectiveness and safety of a novel buffered riboflavin solution approved for corneal cross-linking (CXL) in progressive keratoconus and secondary corneal ectasia. Following the in vivo preclinical study performed on New Zealand rabbits comparing the novel 0.25% riboflavin solution (Safecross®) containing 1% hydroxypropyl methylcellulose (HPMC) with a 0.25% riboflavin solution containing 0.10% EDTA, accelerated epithelium-off CXL was performed on 10 patients (10 eyes treated, with the contralateral eye used as control) through UV-A at a power setting of 9 mW/cm2 with a total dose of 5.4 J/cm2. Re-epithelialization was evaluated in the postoperative 7 days by fluorescein dye test at biomicroscopy; endothelial cell count and morphology (ECD) were analyzed by specular microscopy at the 1st and 6th month of follow-up and demarcation line depth (DLD) measured by anterior segment optical coherence tomography (AS-OCT) one month after the treatment. We observed complete re-epithelization in all eyes between 72 and 96 h after surgery (88 h on average). ECD and morphology remained unchanged in all eyes. DLD was detected at a mean depth of 362 ± 50 µm, 20% over solutions with equivalent dosage. SafeCross® riboflavin solution chemically-boosted corneal cross-linking seems to optimize CXL oxidative reaction by higher superoxide anion release, improving DLD by a factor of 20%, without adverse events for corneal endothelium.


2018 ◽  
Vol 11 (1) ◽  
pp. bcr-2018-225806 ◽  
Author(s):  
Archita Singh ◽  
Noopur Gupta ◽  
Vinod Kumar ◽  
Radhika Tandon

Implantable collamer lenses (ICL) have gained popularity for correction of myopia where kerato-refractive procedures are not indicated as in cases of high myopic refractive errors. Toxic anterior segment syndrome (TASS) is a very uncommonly reported postoperative complication following ICL implantation. A young patient developed severe corneal oedema and anterior segment inflammation on the first day after ICL implantation. Analysing retrospectively, possible idiosyncratic response to intracameral pilocarpine was considered as a cause for TASS. Prompt and intensive therapy with oral and topical potent steroids was visually rewarding. TASS, though a sterile inflammation can have catastrophic sequelae such as corneal decompensation and secondary glaucoma. Hence, timely identification and management is important.


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