Aniridia and the ocular surface: Medical and surgical problems and solutions.

Congenital aniridia is a multisystemic genetic disease due to a mutation in PAX6 gene which severely affects the development and functionality of the human eyes. In patients affected by the mutation, aside from the absence or defects of iris tissue formation, abnormalities in position or opacities of the crystalline lens, macular hypoplasia, ocular surface disease is the main cause of visual loss and the deterioration of the quality of life of most patients. Limbal stem cell deficiency combined with tear film instability and secondary dry eye cause aniridic keratopathy which, in advanced stages, ends up in corneal opacification. In this paper, the actual knowledge about congenital aniridia keratopathy physiopathology and medical and surgical treatment options and their efficacy are discussed. Indications and results of topical treatments with artificial tears and blood-derivatives in its initial stages, and different surgical techniques as limbal stem cell transplantation, keratoplasty and keratoprostheses are reviewed. Finally, recent advances and results in regenerative medicine techniques with ex vivo stem cell cultivation or other types of cultivated cells are presented.

Trasplante autólogo de limbo como nuevo tratamiento de la distrofia corneal epitelial de Lisch / Keratolimbal autograft transplantation as a possible new treatment of Lisch epithelial corneal dystrophy

CASO CLÍNICO: Presentamos el caso de una mujer de 64 años con agudeza visual de 20/40 en su ojo derecho, debida a una opacificación grisácea y plumosa con microquistes en el epitelio corneal, compatible con una distrofia epitelial de Lisch. Se trató secuencialmente con varios desbridamientos epiteliales, lente de contacto y mitomicina C, a pesar de los cuales, recidivaba. La extirpación de un sector limbar y el trasplante de limbo autólogo obtuvieron buenos resultados y sin recurrencia. CONCLUSIÓN: El trasplante autólogo de limbo tras extirpar la zona de limbo afectado puede ser considerado como una nueva opción terapéutica en el tratamiento de la distrofia epitelial de Lisch asimétrica, cuando otros tratamientos han fallado

CASE REPORT: The case concerns 64-year-old woman with visual acuity of 20/40 in the right eye. Slit-lamp examination revealed a grey, feathery corneal opacification with intraepithelial microcysts compatible with Lisch epithelial corneal dystrophy (LECD). It was treated with epithelial debridements, contact lenses and mitomycin C, but the opacification recurred within months. The removal of limbus sector and autologous limbal transplantation (KLAT) were used successfully without recurrence. Conclusions: After removal of damaged limbus, KLAT should be considered as a treatment option for asymmetric LECD when other treatments have failed

Keratolimbal autograft transplantation as a possible new treatment of Lisch epithelial corneal dystrophy. / Trasplante autólogo de limbo como nuevo tratamiento de la distrofia corneal epitelial de Lisch.

CASE REPORT: The case concerns 64-year-old woman with visual acuity of 20/40 in the right eye. Slit-lamp examination revealed a grey, feathery corneal opacification with intraepithelial microcysts compatible with Lisch epithelial corneal dystrophy (LECD). It was treated with epithelial debridements, contact lenses and mitomycin C, but the opacification recurred within months. The removal of limbus sector and autologous limbal transplantation (KLAT) were used successfully without recurrence. CONCLUSIONS: After removal of damaged limbus, KLAT should be considered as a treatment option for asymmetric LECD when other treatments have failed.

Queratitis por Absidia corymbifera en paciente inmunocompetente sin traumatismo corneal previo / Keratitis caused by Absidia corymbifera in an immunocompetent male with no corneal injuries

CASO CLÍNICO: Varón sano de 55 años sin antecedente de traumatismo corneal acude a urgencias por un infiltrado en el ojo izquierdo que responde parcialmente a tratamiento antibiótico tópico. Tras la introducción de corticoterapia tópica presenta un importante empeoramiento de la úlcera. El examen directo y la tinción de Gram permiten un rápido diagnóstico de las hifas de Absidia. Presenta buena respuesta al tratamiento combinado de anfotericina y posaconazol. DISCUSIÓN: Las queratitis por Zygomycetes son raras. Es rara la afectación de pacientes sanos sin antecedentes de traumatismo corneal. El tratamiento combinado de anfotericina y posaconazol ejerce un efecto sinergico contra hongos filamentosos

CASE REPORT: A healthy 55-years-old male went to emergency due to a white infiltrate in the left eye without corneal trauma which partially responds to antibiotic treatment. The infiltrate worsened by the use of topical steroids. Direct microscopic evaluation and Gram stain are a valuable diagnostic tool for the detection of Absidia filaments. There is a successful treatment with anphotericin and posaconazole. DISCUSSION: Keratitis caused by Zygomicetes are unusual. This is a rare condition in healthy patients with no corneal trauma. The treatment with amphotericin and posaconazole are synergistic against filamentous fungi

Keratitis caused by Absidia corymbifera in an immunocompetent male with no corneal injuries.

Case Report A healthy 55-years-old male went to emergency due to a white infiltrate in the left eye without corneal trauma which partially responds to antibiotic treatment. The infiltrate worsened by the use of topical steroids. Direct microscopic evaluation and Gram stain are a valuable diagnostic tool for the detection of Absidia filaments. There is a successful treatment with anphotericin and posaconazole. Discussion Keratitis caused by Zygomicetes are unusual. This is a rare condition in healthy patients with no corneal trauma. The treatment with amphotericin and posaconazole are synergistic against filamentous fungi.

Relación entre la interfase donante/receptor y la agudeza visual en trasplante corneal tipo DSAEK / Relationship between donor-receptor interface and visual acuity in DSAEK

Objetivo: Evaluar la relación entre la agudeza visual (AV) mejor corregida y la interfase donante-receptor resultante tras realizar una queratoplastia endotelial automatizada con pelado de la membrana de Descemet (DSAEK). Métodos: Se revisaron 46 historias clínicas de pacientes intervenidos con técnica DSAEK, de las cuales cumplían criterios de inclusión 22 pacientes (dos de ellos con DSAEK bilateral). Todos ellos fueron intervenidos por un mismo oftalmólogo desde enero del 2010 hasta abril del 2011. Las indicaciones quirúrgicas fueron: queratopatía bullosa 19 (72,2%), distrofia endotelial de Fuchs tres (12,5%) y agotamiento endotelial tras queratoplastia previa dos (8,3%).Se recogió la AV preoperatoria y al tercer y sexto mes postoperatorio, comparándose en estas dos últimas la interfase existente entre donante y receptor tras DSAEK medida en cámara Scheimpflug de topógrafo corneal (Pentacam-HR, Oculus®, Wetzlar, Alemania). Resultados: Tras la cirugía con DSAEK, la mejora en la AV fue en 19 ojos al tercer mes y en 20 ojos al sexto mes postoperatorio (p < 0,001). Dicho cambio en la AV se comparó con la disminución de la densidad óptica de la interfase donante-receptor resultante, encontrando una relación positiva y significativa entre estas (Rho interfase 3 meses y cambio de AV 6 meses: 0,44; p < 0,001). Sin embargo, la interfase se relacionó negativa y significativamente con la AV (Rho 3 m: –0,41; p = 0,045). Además, los pacientes que presentaban un estroma más denso conseguían AV postoperatorias peores en términos relativos si se comparaban con los que presentaban un estroma menos dañado. Conclusiones: Los pacientes sometidos a DSAEK con AV prequirúrgicas peores experimentan una mejora visual mayor en valores absolutos y, sin embargo, alcanzarían una AV menor relativa que se podría relacionar con una interfase más refringente, como consecuencia de un estroma corneal preoperatorio más alterado (AU)

Objective: To evaluate the relationship between best corrected visual acuity and donor-recipient interface resulting after Descemet's Stripping with Automated Endothelial Keratoplasty (DSAEK). Methods: Retrospective review of 46 clinical histories after DSAEK intervention in which 22 patients who fulfilled the inclusion criteria (two with bilateral DSAEK), were studied. All were operated on by the same ophthalmologist from January 2010 until April 2011. Surgical indications were: 19 (72.2%) bullous keratopathy, 3 (12.5%) Fuchs endothelial dystrophy, and 2 (8.3%) previous failed keratoplasty.The visual acuity (VA) was recorded preoperatively, and at three and six months after the operation; the latter two being compared with the interface between donor and recipient after DSAEK measured in a Scheimpflug corneal topography camera (Pentacam® model). Results: After DSAEK surgery 19 eyes had improved visual acuity at three months, and 20 eyes at six months (P < 0.001). This improvement was compared with the decrease in optical density of the resulting donor-receptor interface, with apositive and significant relationship being found between them (Rho interface 3 months, and improved AV 6 months 0.44, P <0 .001). However, the interface is negatively and significantly related with visual acuity (Rho 3 m: –0.41, P = 0.045). Also, patients who had a denser stroma had a worse postoperative visual acuity in relative terms when compared with those who had a less damaged stroma. Conclusions: Patients undergoing DSAEK with worse preoperative visual acuity had a greater improvement visual in absolute values and however, they may have achieved a lower relative visual acuity than could be related to a higher refractive interface, as a result of a more damaged preoperative corneal stroma (AU)

[Relationship between donor-receptor interface and visual acuity in DSAEK]. / Relación entre la interfase donante/receptor y la agudeza visual en trasplante corneal tipo DSAEK.

OBJECTIVE: To evaluate the relationship between best corrected visual acuity and donor-recipient interface resulting after Descemet's Stripping with Automated Endothelial Keratoplasty (DSAEK). METHODS: Retrospective review of 46 clinical histories after DSAEK intervention in which 22 patients who fulfilled the inclusion criteria (two with bilateral DSAEK), were studied. All were operated on by the same ophthalmologist from January 2010 until April 2011. Surgical indications were: 19 (72.2%) bullous keratopathy, 3 (12.5%) Fuchs endothelial dystrophy, and 2 (8.3%) previous failed keratoplasty. The visual acuity (VA) was recorded preoperatively, and at three and six months after the operation; the latter two being compared with the interface between donor and recipient after DSAEK measured in a Scheimpflug corneal topography camera (Pentacam(®) model). RESULTS: After DSAEK surgery 19 eyes had improved visual acuity at three months, and 20 eyes at six months (P<.001). This improvement was compared with the decrease in optical density of the resulting donor-receptor interface, with apositive and significant relationship being found between them (Rho interface 3 months, and improved AV 6 months 0.44, P<.001). However, the interface is negatively and significantly related with visual acuity (Rho 3 m: -0.41, P=.045). Also, patients who had a denser stroma had a worse postoperative visual acuity in relative terms when compared with those who had a less damaged stroma. CONCLUSIONS: Patients undergoing DSAEK with worse preoperative visual acuity had a greater improvement visual in absolute values and however, they may have achieved a lower relative visual acuity than could be related to a higher refractive interface, as a result of a more damaged preoperative corneal stroma.

Toxicidad corneal por amantadina / Corneal toxicity due to amantadine

Caso clínico: Mujer de 64 años en tratamiento con amantadina durante 2 años por enfermedad de Parkinson, que presentó edema corneal bilateral de inicio brusco. Inicialmente se trató como una endotelitis herpética sin mejoría, al desconocer la medicación empleada por la enferma. Finalmente el edema se resolvió tras la suspensión del fármaco. Discusión: El hidrocloruro de amantadina puede producir disfunción endotelial. El edema corneal puede ser reversible tras su suspensión, pero la densidad endotelial permanece baja. Sería necesario realizar un control oftalmológico previo al inicio del tratamiento para valorar el riesgo/beneficio del mismo, sobre todo en los pacientes que presenten baja densidad endotelial o un endotelio alterado (AU)

Case report: A 64 year-old female with Parkinson disease treated with amantadine for two years who suddenly suffered bilateral corneal oedema. It was initially treated as herpetic endotheliitis without improvement as we lacked information on her chronic treatment. The corneal oedema finally resolved after withdrawing the drug. Discussion: Amantadine hydrochloride may produce endothelial dysfunction. Once the amantadine treatment is stopped, the corneal oedema may be reversible but endothelial density remains low. An ophthalmologist examination should be performed before the initiation of amantadine treatment in order to establish a risk: benefit ratio, especially in those patients with low endothelial density or any endothelial anomaly (AU)

Técnica de extracción de esclera donante mediante extrusión del globo ocular / Technique for removing donor sclera by eyeball extrusion

Objetivo: Describir una técnica quirúrgica de extracción escleral en ojo de donante tras la escisión del botón corneoescleral. Conclusiones: La técnica de extrusión es una técnica fácil de realizar, que permite la extracción escleral completa facilitando además su limpieza total y el aislamiento de la retina y del tejido uveal. Esta técnica podría tener un papel importante en el estudio anatómico y morfológico de las estructuras oculares(AU)

Purpose: To describe a surgery technique for removing donor sclera tissue after corneo-scleral button excision. Results: The extrusion technique is easy to perform. It allows the complete scleral extraction its total clean up to be performed, as well as making easier to isolate the retina and uveal tissue. This technique could have an important role in the anatomical and morphological study of ocular structures(AU)

[Corneal toxicity due to amantadine]. / Toxicidad corneal por amantadina.

CASE REPORT: A 64 year-old female with Parkinson disease treated with amantadine for two years who suddenly suffered bilateral corneal oedema. It was initially treated as herpetic endotheliitis without improvement as we lacked information on her chronic treatment. The corneal oedema finally resolved after withdrawing the drug. DISCUSSION: Amantadine hydrochloride may produce endothelial dysfunction. Once the amantadine treatment is stopped, the corneal oedema may be reversible but endothelial density remains low. An ophthalmologist examination should be performed before the initiation of amantadine treatment in order to establish a risk: benefit ratio, especially in those patients with low endothelial density or any endothelial anomaly.

[Technique for removing donor sclera by eyeball extrusion]. / Técnica de extracción de esclera donante mediante extrusión del globo ocular.

PURPOSE: To describe a surgery technique for removing donor sclera tissue after corneo-scleral button excision. RESULTS: The extrusion technique is easy to perform. It allows the complete scleral extraction its total clean up to be performed, as well as making easier to isolate the retina and uveal tissue. This technique could have an important role in the anatomical and morphological study of ocular structures.

[Argon laser iridoplasty as treatment of plateau-like iris configuration secondary to multiple ciliary body cysts: long-term follow-up by ultrasound biomicroscopy]. / Iridoplastia con láser argón como tratamiento del iris plateau secundario a múltiples quistes del cuerpo ciliar: seguimiento a largo plazo con biomicroscopía ultrasónica.

CASE REPORT: We present a 37 year-old man with secondary angle closure by neuroepithelial ciliary body cysts. An argon laser peripherical iridoplasty was performed and ultrasound biomicroscopy (UBM) was employed to follow-up. Initially, the patient had a favorable response to treatment, but six months later the cysts and the secondary closed angle reappeared. DISCUSSION: Argon laser peripheral iridoplasty has been proposed as treatment of plateau-like iris configuration resulting from neuroepithelial ciliary body cysts. However, the cysts and, the secondary closed angle could recur in a long-term (Arch Soc Esp Oftalmol 2009; 84: 569-572).

Iridoplastia con láser argón como tratamiento del iris plateau secundario a múltiples quistes del cuerpo ciliar: seguimiento a largo plazo con biomicroscopía ultrasónica / Argon laser iridoplasty as treatment of plateau-like iris configuration secondary to multiple ciliary body cysts: long-term follow-up by ultrasound biomicroscopy

Caso clínico: Varón de 37 años de edad diagnosticadode cierre angular secundario a quistes neuroepitelialesdel cuerpo ciliar. Se trató mediante iridoplastiaperiférica con láser argón y se realizó seguimientomediante biomicroscopía ultrasónica(BMU). Inicialmente se consiguió una resolucióndel cuadro, pero a los 6 meses los quistes y el cierreangular secundario reaparecieron.Discusión: La iridoplastia periférica con láserargón se ha propuesto como tratamiento en situacionesde bloqueo angular secundario a quistes neuroepitelialesdel cuerpo ciliar. Sin embargo, losquistes y, secundariamente el cierre angular, podríanrecidivar a largo plazo(AU)

Case report: We present a 37 year-old man withsecondary angle closure by neuroepithelial ciliarybody cysts. An argon laser peripherical iridoplastywas performed and ultrasound biomicroscopy(UBM) was employed to follow-up. Initially, thepatient had a favorable response to treatment, butsix months later the cysts and the secondary closedangle reappeared.Discussion: Argon laser peripheral iridoplasty hasbeen proposed as treatment of plateau-like iris configurationresulting from neuroepithelial ciliarybody cysts. However, the cysts and, the secondaryclosed angle could recur in a long-term(AU)

[Monolayered amniotic membrane transplantation as a palliative treatment for bullous keratopathy]. / Injerto de membrana amniótica monocapa como tratamiento paliativo de la queratopatía bullosa.

PURPOSE: To determine the efficacy of monolayered amniotic membrane transplantation for short and medium-term symptomatic improvement in those cases of bullous keratopathy where a penetrating keratoplasty is not indicated. METHODS: Amniotic membrane transplantation was performed in five patients with symptomatic bullous keratopathy in which penetrating keratoplasty was not indicated. Data about bandage contact lens need before and after surgery, existence of ocular pain, occurrence and extension of bullae, epithelial defects, and persistence of amniotic membrane, were obtained at days 4 and 14, and months 1, 2, 4 and 6 after surgery. RESULTS: No pain, bullae or epithelial defects were present at day 14 in any patient. However, progressive dissolvement of the amniotic membrane was accompanied with more bullae and pain. At 6th month, 4 of 5 patients required bandage contact lens. CONCLUSION: Amniotic membrane transplantation alleviates short-term symptomatic bullous keratopathy and preserves corneal anatomy. Six months after surgery, anatomical and symptomatic improvement does not avoid the need of bandage contact lens.

Injerto de membrana amniótica monocapa como tratamiento paliativo de la queratopatía bullosa / Monolayered amniotic membrane transplantation as a palliative treatment for bullous keratopathy

Objetivo: Comprobar la eficacia del injerto de membrana amniótica en monocapa en la mejoría sintomática a corto y medio plazo de la queratopatía bullosa no susceptible de queratoplastia. Sujetos y métodos: Se realizó injerto de membrana amniótica en cinco pacientes con queratopatía bullosa no susceptibles de queratoplastia. Se valoró la necesidad de lente terapéutica pre y post operatoria, la existencia de dolor, la presencia de bullas (y número de cuadrantes a los que afectaba), de defectos epiteliales, y de restos de membrana amniótica a los 4 y 14 días, y a los 1, 2, 4, y 6 meses de la cirugía. Resultados: A los 14 días ningún paciente presentaba dolor, defecto epitelial o bullas. Sin embargo, la desaparición progresiva de la membrana iba acompañada de aumento de la sintomatología y de las bullas. A los 6 meses, 4 de 5 pacientes necesitaban la utilización de lente terapéutica. Conclusiones: El injerto de membrana amniótica reduce la sintomatología de la queratopatía bullosa a corto plazo y preserva la anatomía corneal. A los 6 meses de la cirugía, la mejoría anatómica y sintomática no evita la necesidad de la utilización de lente de contacto terapéutica (AU)

[Analytic management in ophthalmology: towards a cost per hospital out- and in-patient]. / La contabilidad analítica en oftalmología: hacia el coste por paciente hospitalario.

PURPOSE: To determine the total annual cost of the activities in the Ophthalmology Department at the Hospital La Mancha-Centro, Homogeneous functional group (HFG) of the INSALUD, and to estimate the cost per patient/process of both ambulatory patients and hospital in-patients. METHODS: The study was designed over 1 year period, from January to December 1997, and applied by using analytic accounting in 17,857 patients seen at the out-patient ophthalmology clinic and 973 hospital in-patients who were classified by diagnosis-related groups (DRG) and sorted out by analysis of Case Mix index. RESULTS: We have determined the annual cost of our activity in both the ambulatory patient group and the hospital in-patient group classified by DRG and obtained the cost per process. CONCLUSIONS: To know systematically the annual cost of our medical and surgical activity is nowadays a necessary tool in the framework of the Total Quality Plan developed by the INSALUD's SIGNO Group since 1991. This leads us to the analytic management area and places us in the never ending search for the ideal model of cost per process determination.

[Subconjunctival mitomycin C for the treatment of ocular cicatricial pemphigoid]. / Mitomicina C subconjuntival en el tratamiento del penfigoide ocular cicatricial.

PURPOSE: We study the efficacy of treating ocular cicatricial pemphigoid (OCP) with subconjuctival mitomycin C (MMC). METHODS: The patients were eligible for treatment under two criteria: 1. Significant complications of systemic immunosuppressant therapy. 2. Avanced stage of OCP. All patients received an injection of 0.25 ml of 0.2 mg/ml mitomycin C to both the upper and lower bulbar conjunctiva in both eyes. RESULTS: Four patients were treated with subconjunctival mitomycin C (both eyes) and were followed for an average of 17 months (range 9-26 months). Three of four patients showed progression of the conjunctival disease and required concomitant systemic immunosuppressive therapy after subconjuctival mitomycin C. CONCLUSION: No complications of subconjunctival mitomycin C treatment were noted but, contrary to what other authors had referred, its use was not effective in preventing progression of conjunctival cicatrization in our patients with OCP.