Faculty Bibliography

PURPOSE/OBJECTIVE:PAX6 is a highly conserved protein essential for the control of eye development both in invertebrates and vertebrates. PAX6 expression persists in the adult inner retina, but little is known about its functions after completion of retinal differentiation. Therefore, we investigated PAX6 expression in wild-type and calcitonin receptor-like receptor transgenic (CLR(SMαA)) mice with angle-closure glaucoma. METHODS:Intraocular pressure was measured by indentation tonometry in anesthetized mice. Eyes of mice of both genotypes were enucleated at various ages and retinas were processed for morphological analysis and PAX6 immunostaining. The content of PAX6 in retinal extracts was estimated by Western blot analysis. Retinal expression of glaucoma-related genes was analyzed by reverse transcription-polymerase chain reaction. RESULTS:Control mice showed normal retinal morphology between p22 and p428 with steady PAX6 expression in the ganglion cell layer (GCL) and the inner nuclear layer (INL). CLR(SMαA) mice examined between p22 and p82 exhibited increased intraocular pressure and a progressive decrease in cell number including PAX6-expressing cells in the GCL. The INL was not affected up to postnatal day 42. Later, a significant increase in PAX6-expressing cells concomitant with an overall loss of cells was observed in the INL of CLR(SMαA) as compared with control mice. Retinal up-regulation of glaucoma-related genes was furthermore observed. CONCLUSIONS:Distinctive changes of PAX6 expression in the inner retina of CLR(SMαA) mice suggest a role in regulatory mechanisms involved in glaucoma-related retinal cell death. The selective increase of PAX6 expression in the degenerating INL of CLR(SMαA) mice may represent an attempt to preserve retinal cytoarchitecture.

BACKGROUND:Despite extensive knowledge regarding the diagnosis and management of keratoconus and ectatic corneal diseases, many controversies still exist. For that reason, there is a need for current guidelines for the diagnosis and management of these conditions. PURPOSE/OBJECTIVE:This project aimed to reach consensus of ophthalmology experts from around the world regarding keratoconus and ectatic diseases, focusing on their definition, concepts, clinical management, and surgical treatments. METHODS:The Delphi method was followed with 3 questionnaire rounds and was complemented with a face-to-face meeting. Thirty-six panelists were involved and allocated to 1 of 3 panels: definition/diagnosis, nonsurgical management, or surgical treatment. The level of agreement considered for consensus was two thirds. RESULTS:Numerous agreements were generated in definitions, methods of diagnosing, and management of keratoconus and other ectatic diseases. Nonsurgical and surgical treatments for these conditions, including the use of corneal cross-linking and corneal transplantations, were presented in a stepwise approach. A flowchart describing a logical management sequence for keratoconus was created. CONCLUSIONS:This project resulted in definitions, statements, and recommendations for the diagnosis and management of keratoconus and other ectatic diseases. It also provides an insight into the current worldwide treatment of these conditions.

OBJECTIVE:Corneal collagen cross-linking with riboflavin and UV-A (CXL) decreases corneal oedema and increases visual acuity in human patients with bullous keratopathy. Presumed mechanisms are an increase in collagen packing density and a reduction in stromal swelling pressure. We present two cases in which CXL was used to treat bullous keratopathy in dogs. PROCEDURES/METHODS:Four eyes of two dogs with painful bullous keratopathy-induced corneal erosions that were resistant to prior therapy were treated with CXL. Both corneas of the second patient were dehydrated to ± 400 μm corneal thickness using topical 70% glycerol solution immediately prior to CXL. Follow-up included slit-lamp examination, fluorescein staining and photographic documentation in both cases and high-resolution ultrasound examination in the second patient. RESULTS:All four eyes were comfortable and fluorescein negative at 1-week post-CXL and remained so for the rest of the follow-up period (17.5 months for case 1 and 6 months for case 2). The owner of the first patient reported a less oedematous cornea and improvement in vision that lasted for 6 months. Despite a reported lack of improvement in vision in the second patient, corneal thickness initially decreased, but was back at baseline thickness at the 4-month recheck. CONCLUSIONS:Similar to humans, CXL might become a useful treatment option for bullous keratopathy-induced therapy-resistant corneal erosions in dogs. Patient comfort was greatly improved, but corneal thickness decrease was not as long-lasting as reported for humans. The presently used protocols might need modification to fit the dog cornea.

BACKGROUND:Nasal deformity is a common disorder in Wegener granulomatosis, which is produced by polyangitis of medium-sized vessels. This process may cause necrosis and destruction of the nasal mucosa and osteocartilaginous framework. At this time, there is a lack of published data on nasal deformities associated with Wegener granulomatosis. METHODS:We present a 53-year-old woman with completely necrotized nasal lining and osteocartilage. The patient had a history of granulomatosis with polyangitis that was currently in remission. We reconstructed the patient's nose with fifth and sixth rib cartilages over 3 successive surgeries over a 2-year period. RESULTS:During the 3-year follow-up period, neither infection nor significant cartilage graft resorption was observed, and the aesthetic result was acceptable both to the patient and her physicians. CONCLUSIONS:In spite of contamination to the nasal lining and immune-compromising medications, nasal reconstruction is usually successful when considering these factors. Access to strong and abundant fifth or sixth rib cartilage presents an opportunity to reconstruct nasal destruction due to iatrogenic or autoimmune processes.

PURPOSE/OBJECTIVE:To compare the 1-year results of total versus partial epithelium removal in corneal cross-linking in the treatment of progressive keratoconus. METHODS:This retrospective study compared the results of total (the total group) versus partial (the partial group) approaches of epithelium removal in corneal cross-linking. Eighty eyes of 65 patients (40 eyes in each group) were enrolled. The mean age of the participants was 25.48 ± 4.80 years and 62.5% were male. One-year changes in vision parameters, refraction, and Pentacam (Oculus Optikgeräte GmbH, Wetzlar, Germany) indices were compared between the two groups using repeated measures analysis of variance. RESULTS:One year after corneal cross-linking, uncorrected distance visual acuity in the total and partial group improved by 0.13 ± 0.42 and 0.12 ± 0.36 logMAR (P = .447), respectively, and corrected distance visual acuity improved by 0.00 ± 0.19 and 0.13 ± 0.20 logMAR (P = .001), respectively. Spherical equivalent decreased by 0.44 ± 1.25 diopters (D) in the total group and 0.56 ± 1.47 D in the partial group (P = .710). The decrease in maximum keratometry was 0.39 ± 0.93 and 0.01 ± 0.95 D in the total and partial group, respectively (P = .037), and the decrease in mean keratometry was 0.42 ± 0.93 and 0.00 ± 0.65 D (P = .015), respectively. Central corneal thickness decreased by 18.39 ± 20.66 μm in the total group and 0.11 ± 13.29 μm in the partial group (P < .001). CONCLUSIONS:One year after corneal cross-linking, both approaches showed similar results in terms of uncorrected distance visual acuity. With the partial approach, there was slightly better corrected distance visual acuity improvement and central corneal thickness maintenance, but slightly better corneal flattening was achieved with the total removal. Long-term studies are needed to compare these two approaches in terms of stability of results and stopping the progression of keratoconus.