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.

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.

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.

[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.

[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).

[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.

[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.

[Bilateral serous retinal detachments in acute posterior multifocal placoid pigmentous epitheliopathy]. / Desprendimientos retinianos serosos bilaterales en la epiteliopatía pigmentaria placoide multifocal posterior aguda.

PURPOSE/METHOD: A particular subtype of acute posterior multifocal placoid pigmented epitheliopathy (APMPPE), different from the classical Gass description, is presented. A 22 year old woman who suffered a sudden decrease in visual acuity associated to bilateral serous retinal detachment concurrent with anterior uveitis is studied. We remark the fact that patient had been using contraceptives and antibiotics (norfloxacin) for the past two months prior to first visit. One month later, visual acuity does not show any recovery. A corticosteroid treatment is prescribed showing improvement. RESULTS/CONCLUSIONS: We believe that although APMPPE usually resolves spontaneously, treatment is needed for clinical subtypes involving serous retinal detachment.