Cirugía combinada de catarata y queratoplastia lamelar endotelial en el síndrome iridocórneo endotelial / Combined cataract surgery and lamellar endothelial keratoplasty in iridocorneal endothelial síndrome

Reportamos el caso de una mujer de 59 años, que refería disminución de agudeza visual (AV) en el ojo izquierdo (OI). Mediante la exploración, se objetivó en dicho ojo una AV corregida de cuenta dedos a 30 cm, y en la lámpara de hendidura se observó la presencia de edema corneal moderado, con pliegues en la membrana de Descemet. También se apreciaron sinequias iridianas anteriores, atrofia de iris y corectopia. Se diagnosticó de síndrome iridocórneo endotelial (ICE). Se decidió un abordaje terapéutico quirúrgico mediante una cirugía combinada de catarata y queratoplastia endotelial automatizada con disección de la membrana de Descemet (DSAEK). No se reportaron complicaciones intraoperatorias. La recuperación anatómica y funcional fue exitosa, presentando una AV corregida al año de 0,8. Este resultado apoya la eficacia de la DSAEK en el síndrome ICE animando a la realización de más estudios que soporten igualmente su eficacia en este síndrome

The case is reported of a 59-year-old woman, who reported decreased visual acuity (VA) in the left eye (LE). On examination, a corrected VA of finger count/ 30cm was observed in LE. Corneal oedema and folds in the Descemet's membrane were observed using the slit lamp. Anterior synechiae, iris atrophy, and corectopia were also found. ridocorneal endothelial syndrome (ICE) was diagnosed. The treatment option was a combination of cataract surgery and automated endothelial keratoplasty with Descemet's membrane dissection (DSAEK). There were no complications during the operation, with a successful anatomical and functional recovery, with a VA of 0,8 being observed after one year. This result supports the efficacy of DSAEK in ICE syndrome, encouraging more studies to be carried out that should also support its efficacy in this syndrome

Descripción clínica mediante tomografía de coherencia óptica de segmento anterior y microscopía especular de un síndrome endotelial iridocorneal tipo Cogan Reese / Clinical description of a Cogan-Reese type iridocorneal endothelial syndrome using anterior segment optical coherence tomography and specular microscopy

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Ex-PRESS mini-shunt implanted in a pregnant patient with iridocorneal endothelial syndrome.

INTRODUCTION: The failure rate of both filtration surgery and of aqueous shunt implantation is higher for iridocorneal endothelial syndrome than in other scenarios, due to the continuous proliferation of abnormal endothelial cells over the trabecular meshwork and the filtration area and also due to the more pronounced cicatrizing response shown by these young patients. We present the first case ever described in the literature of a pregnant patient with iridocorneal endothelial syndrome and uncontrolled ocular hypertension who was implanted an Ex-PRESS mini-shunt. CLINICAL CASE: A 35-year-old female presented with diminution of vision in the left eye for 2 months. She was 20 weeks pregnant. Her visual acuity was 20/20 in right eye and 20/25 in left eye, and intraocular pressure was 11 mmHg in right eye and 34 mmHg in left eye. Slit lamp biomicroscopic examination revealed no alterations in right eye, whereas left eye showed corectopia and uveal ectropion, stroma of iris' sectoral atrophy and moderate corneal epithelial edema. Gonioscopy showed some anterior iris synechiae in left eye. Fundus evaluation was normal. Based on clinical features and examination, the diagnosis of left eye iridocorneal endothelial syndrome with decompensated intraocular pressure was made. She was prescribed topical timolol (0.5%) and dorzolamide. As a result of uncontrolled intraocular pressure and the impossibility to prescribe other hypotensive treatment available due to her being pregnant, it was decided to perform surgery in left eye using an Ex-PRESS mini-shunt and Ologen®; 6 months post surgery, intraocular pressure was 9 mmHg with no need for hypotensive treatment. The cornea was transparent, and the patient maintained her left eye visual acuity. CONCLUSIONS: Ex-PRESS mini-shunt can be considered a surgical option for iridocorneal endothelial syndrome. Its composition allows the ostium to remain open and the device triggers a milder postoperative inflammatory response. In our particular case, taking into account that the subject was a young, phakic, pregnant woman, whose intraocular pressure had to be closely controlled and we had to ensure that her postoperative care included as few drugs and as few reoperations as possible, we thought that using this device was the most appropriate option.

Cogan-Reese syndrome: image analysis with specular microscopy, optical coherence tomography, and ultrasound biomicroscopy.

Iridocorneal endothelial (ICE) syndrome is a progressive clinical spectrum of corneal endothelial abnormalities affecting the cornea, iris, and iridocorneal angle. Three clinical variations are recognized: essential (progressive) iris atrophy, Chandler syndrome, and Cogan-Reese syndrome. Direct slit-lamp visualization of the cornea and anterior segment in cases of ICE syndrome is inadequate for precise and objective assessment of the affected structures. We describe the evolution of corneal and anterior segment structural changes in a woman with Cogan-Reese syndrome using three different methods of image analysis: specular microscopy, anterior segment optical coherence tomography, and ultrasound biomicroscopy.

Case Report: Iridocorneal Endothelial Syndrome Progression Documented by Anterior Segment Optical Coherence Tomography.

SIGNIFICANCE: Iridocorneal endothelial (ICE) syndrome is characterized by a difficult-to-see membrane of ectopic corneal endothelial cells, called an ICE membrane. This report demonstrates that ICE membranes are detectable by anterior segment optical coherence tomography (AS-OCT) and highlights the benefits of using AS-OCT in ICE syndrome. PURPOSE: A case of ICE syndrome is reported to highlight the utility of AS-OCT in the characterization and diagnosis of the disease. CASE REPORT: A 65-year-old African American man was diagnosed as having the Chandler variant of ICE syndrome of the left eye. He was treated topically for corneal edema. Anterior segment optical coherence tomography and anterior segment photography were used to aid in the diagnosis and document an ICE membrane and multiple peripheral anterior synechiae. Further examinations chronicled the progression of a peripheral anterior synechia. At first, only iridocorneal touch (sparing the trabecular meshwork) was present, but later, full apposition to the trabecular meshwork developed. The patient was diagnosed as having pre-perimetric glaucoma in the left eye and is being treated topically to reduce his intraocular pressure. The right eye remains unremarkable. CONCLUSIONS: Anterior segment optical coherence tomography can support the diagnosis of ICE syndrome by visualizing ICE membranes and differentiating between true peripheral anterior synechiae and iridocorneal touch that does not block the trabecular meshwork. Chronicling ICE membranes longitudinally with AS-OCT will enhance understanding of disease progression. By correlating membranes features (such as location and extent) and patient outcomes, AS-OCT-based ICE membrane classifications can be developed to improve the management and prognosis of ICE syndrome.

Evaluación de los signos de la pseudoexfoliación en el segmento anterior mediante tomografía de coherencia óptica y cámara de Scheimpflug / Pseudoexfoliation signs in the anterior segment assessed by optical coherence tomography and Scheimpflug device

Objetivo: Evaluar diferentes parámetros del segmento anterior en ojos con pseudoexfoliación (PSX), ojos contralaterales y controles mediante tomografía de coherencia óptica (OCT) y cámara Scheimpflug. Métodos: Se estudiaron 3 grupos: 44 ojos de 44 pacientes con PSX, 30 ojos contralaterales no afectos y 148 ojos de 148 controles sanos. Mediante la cámara de Scheimpflug (Pentacam, Oculus Inc.; Wetzlar, Alemania) se midieron la profundidad y volumen de la cámara anterior, volumen corneal y paquimetría, diámetro pupilar y densitometría corneal. Mediante OCT RTVue 100 (Optovue, Fremont, CA, EE. UU.) se midieron la abertura angular, la longitud y el área de la malla trabecular, el grosor del iris, y se valoró la visualización de depósitos PSX. Resultados. No se observaron diferencias en cuanto a la profundidad ni volumen de la cámara anterior, ni en el volumen corneal o paquimetría (p≥0,228 en todos los parámetros) entre grupos. Sin embargo, la densitometría corneal fue mayor en PSX y en los ojos contralaterales que en el grupo control (p < 0,001). En cuanto a los parámetros de OCT no existieron diferencias en la abertura angular ni en el tamaño de la malla entre los 3 grupos, siendo el grosor del iris menor en controles (p = 0,005); identificándose en todos los pacientes el depósito PSX mediante OCT. Conclusiones: No se detectaron diferencias entre las medidas biométricas del segmento anterior entre los pacientes con PSX y controles, salvo en el caso de la densitometría corneal central y el grosor del iris que fueron mayores en el grupo con PSX y en los ojos contralaterales (AU)

Objective: To evaluate different anterior segment parameters in eyes with pseudoexfoliation (PSX), fellow eyes, and controls using optical coherence tomography and a Scheimpflug imaging system. Methods: Three groups were studied: 44 eyes of 44 patients with PSX, 30 clinically unaffected fellow eyes, and 148 eyes of 148 healthy controls. The anterior chamber depth and volume, corneal volume and thickness, pupil diameter and corneal densitometry were measured using a Scheimpflug imaging system (Pentacam, Oculus Inc.; Wetzlar, Germany). The angle width, the length and area of the trabecular meshwork, and the iris thickness were measured using an optical coherence tomography RTVue 100 device (Optovue, Fremont, CA, USA). The presence of PSX deposits was also assessed by OCT. Results: There were no differences in the anterior chamber volume or depth in the corneal volume or central thickness (P ≥ .228). The corneal densitometry was similar between PSX and fellow eyes; however it was greater than in the control group (P < .001). As regards the parameters measured by OCT, there were no differences in the angle width or in the trabecular meshwork size between the 3 groups; however, the iris was thinner in controls (P = .005). In all patients the PSX deposits were correctly visualised by OCT after the identification by biomicroscopy. Conclusions: There were no differences in the anterior segment biometric measurements between patients with PSX and controls, although the corneal densitometry and iris thickness were greater in the PSX and fellow eyes groups (AU)

Síndrome iridocorneoendotelial: variedad Chandler / Iridocorneal endothelial syndrome in Chandler variety

En el síndrome iridocorneoendotelial se unifican diferentes variaciones clínicas de una misma entidad. El denominador común de todas ellas es una peculiar anormalidad de las células endoteliales, alteraciones del iris y el desarrollo de glaucoma, generalmente unilateral y más común en mujeres. Se presenta un caso clínico de la variedad Chandler, quien acudió a la consulta de catarata, con este diagnóstico en el ojo derecho. Se realiza cirugía de catarata en ambos ojos con excelentes resultados y control de la presión intraocular(AU)

Iridocorneal endothelial syndrome comprises different clinical variations of the same disease. The common features of all them are peculiar anomaly of the endothelial cells, alterations of the iris and the onset of glaucoma, generally unilateral and more common in women. A clinical case of the Chandler variety is presented, which is a woman with cataract, and iridocorneal endothelial syndrome diagnosis on the right eye. Cataract surgery was performed in both eyes with excellent results and control of the intraocular pressure(AU)