Association of retinitis pigmentosa and advanced keratoconus in siblings.

Objective. The aim of the article was to present the rare association of retinitis pigmentosa and bilateral keratoconus in two brothers, one of whom developed corneal hydrops bilaterally, within a short period of time. Methods. A 29-year-old man presented to our service with corneal hydrops in the right eye, complaining of ocular pain and photophobia. He had a history of retinitis pigmentosa, having been diagnosed as an infant. He also had a younger brother carrying the same diagnosis. Slit lamp examination revealed bilateral keratoconus with corneal hydrops in the right eye, posterior subcapsular cataract, macular atrophy and the characteristic retinal signs of retinitis pigmentosa. The patient's brother was also examined, with the same findings being noted, apart from the corneal hydrops. We documented the changes using a slit lamp biomicroscope, a fundus camera, a corneal topography, Anterior Segment Optical Coherence Tomography and visual field testing. Right hydrops regressed in one month after hyperosmolar 5% sodium chloride treatment. However, 4 weeks later, the patient presented with the same corneal findings in the left eye. The same treatment was prescribed for the left eye. Results. Corneal hydrops regressed in both eyes with remaining paracentral corneal scars. However, no other treatment for keratoconus was suitable in the case of this patient. Discussion: Retinitis pigmentosa is currently not amenable to any form of treatment, from vitamin supplementation, medical therapy, gene transfer-based therapy, stem cell-based therapy to retinal implantation. However, molecular genetics may someday provide new therapeutic prospects, that could modify the course of RP. Conclusions. The association of retinitis pigmentosa with keratoconus is a fairly rare finding, worth taking into consideration. Also, presentation with keratoconus in such an advanced state is uncommon and, in our case, it was presumably due to the patient's reduced visual function since childhood, secondary to retinitis pigmentosa, that has prevented him from perceiving any visual modifications caused by keratoconus.

Slit-lamp Pars Plana Needle Hyaloidotomy-Zonulotomy-Iridotomy for the Treatment of Spontaneous Malignant Glaucoma.

An 88-year-old woman with a history of recent complicated pacemaker insertion presented with acute-onset malignant glaucoma recalcitrant to conservative medical therapy. Surgical intervention was discussed; however, given her complex cardiac history and recent postoperative state, the risk of anesthesia-related systemic adverse events was deemed unacceptably high. As such, a slit-lamp procedure was recommended to break the attack of malignant glaucoma. Here within, we report a novel technique of breaking an attack of malignant glaucoma by needling the anterior hyaloid face at the slit lamp. With this technique, a 25-G needle was entered through the pars plana and was advanced through the anterior hyaloid face, zonules, and peripheral iridotomy to create a unicameral eye and successfully break the malignant closure attack.

Salzmann's nodular degeneration of cornea associated with thyroid eye disease.

Salzmann's nodular degeneration (SND) typically occurs in patients who are female, 50-60 years old, and have a history of corneal inflammation and irritation. Multiple case reports have documented associations between SND and trachoma, viral infections, trauma, contact lens wear, corneal surgeries and corneal exposure. The authors describe a patient with bilateral SND confirmed by anterior segment optical coherence tomography (OCT) imaging in the context of thyroid eye disease (TED) and history of LASIK. Treatment involved propylthiouracil (PTU), artificial tear use, loteprednol etabonate ophthalmic gel, eyelid taping and selenium supplementation and prospective superficial keratectomy with diamond burr polish.

A Case of Toxic Keratoconjunctivitis by Self-application of Human Breast Milk

PURPOSE: We report a case of toxic keratoconjunctivitis resulting from the self-application of human breast milk as a traditional folk remedy for allergic conjunctivitis. CASE SUMMARY: An 82-year-old woman presented with pain and conjunctival hyperemia in the right eye that had been worsening for three days. Two months previously, she was treated with antiallergic eye drops for allergic conjunctivitis at another eye clinic. However, the symptoms did not improve. She applied her daughter-in-law's breast milk into her right eye as a folk remedy for three days. The pain and conjunctival hyperemia worsened. At the initial visit, her corrected visual acuity was 0.3 in the right eye. Slit lamp examination demonstrated conjunctival hyperemia, punctate epithelial erosion at the central cornea, corneal keratic precipitates and white-colored deposits in the peripheral cornea combined with irregularly shaped small nodules. There was no anterior chamber inflammation. There was no medical history of rheumatoid arthritis or tuberculosis. Blood tests for serum and other infectious and inflammatory levels for infection and inflammatory markers were performed followed by application of topical steroids and antibiotics with artificial tears. After 3 weeks of treatment, conjunctival hyperemia and corneal deposits had almost resolved and best-corrected visual acuity improved to 1.0. CONCLUSIONS: The self-application of human breast milk may cause toxic keratoconjunctivitis. Therefore, efforts should be made, actively, to inform and educate the elderly in rural areas not to use human breast milk as a folk remedy.

Slit Lamp-Based Ocular Scoring Systems in Toxicology and Drug Development: A Literature Survey.

PURPOSE: To present a survey of the features of published slit lamp-based scoring systems and their applicability in the context of modern ocular toxicology and drug development. METHODS: References describing original or modified slit lamp-based scoring systems for human or veterinary clinical patients or in investigative or toxicologic research were collected following a comprehensive literature review using textbooks and online publication searches. Each system's indications and features were compiled to facilitate comparison. RESULTS: Literature review identified 138 original or modified scoring systems. Most (48%) were published for evaluation of the ocular surface, 34% for the general anterior segment, and 18% for the lens. Most systems were described for assessment of human patients (50%) and small albino laboratory species such as rabbits (19%), rats (12%), and mice (8%). Systems described for pigmented laboratory species and for larger species such as dogs, cats, pigs, and nonhuman primates (NHPs) were comparatively underrepresented. No systems described a lens scoring scheme specific to the dog, cat, pig, or NHP. Scoring schemes for aqueous and vitreous cells were infrequently described for laboratory species. CONCLUSIONS: Many slit lamp-based scoring systems have been published, but the features of each differ and complicate translation of findings between different species. Use and interpretation of any scoring system in toxicology and drug development must be done with awareness of the limitations of the system being used.

Optical coherence tomography of the Kayser-Fleischer ring: an ancillary diagnostic tool for Wilson's disease in children.

This report presents anterior segment optical coherence tomography (AS-OCT) images of Kayser-Fleischer ring (KFR) in a child. The AS-OCT images highlight differential reflectivity of the KFR depending on amount of copper deposited in cornea, thus supporting the role of AS-OCT as a follow-up tool. Utility of AS-OCT for diagnosing and documenting the KFR in children otherwise uncooperative for detailed slit lamp examination is discussed.

A case of atypical nocardia asteroides sclerouveitis resistant to fourth-generation fluoroquinolones / Um caso atípico de esclero-uveíte por Nocardia Asteroides resistente a fluorquinolona de quarta-geração

ABSTRACT We describe an unusual case of Nocardia spp scleritis in a health girl resistant to topical fourth-generation fluoroquinolones. Clinically, there was only partial response of the scleritis to initial therapy. Treatment was changed to meropenem intravenously and topical amikacin. Following several weeks of antibiotic treatment, the patient's infection resolved but her vision was reduced to no light perception. Nocardia asteroides must be considered as a possible agent in cases of necrotizing scleritis in patients without a clear source. Antibiotic sensitivity testing has a definitive role in view of the resistance to these new medications.

RESUMO Nós descrevemos um raro caso de esclerite por Nocardia spp em uma criança sadia resistente a utilização tópica de fluorquinolona de quarta-geração. Clinicamente, a paciente apresentou apenas uma resposta parcial do quadro de esclerite a terapêutica inicial. O tratamento foi então modificado para meropenem intravenoso e amicacina tópica. Após várias semanas de tratamento com antibiótico, o quadro infeccioso regrediu porém a visao da pacientes evoluiu para perda da percepção luminosa. Em casos de esclerite necrotizante em pacientes sem fatores de risco aparente é necessário considerer a Nocardia Asteroides como possível agente causador. Os testes de sensibilidade medicamentosa apresentam importância significativa em virtude do aparecimento de resistência aos novos medicamentos.

A Case of Bilateral Branch Retinal Vein Occlusion after Taking Tamoxifen

PURPOSE: To report a case of bilateral branch retinal vein occlusion development after taking long-term tamoxifen adjuvant therapy. CASE SUMMARY: A 72-year-old female breast cancer patient with a 10-year history of tamoxifen intake presented with decreased visual acuity that began 5 years prior. The patient had no other past history. The best corrected visual acuity (BCVA) was 0.3 in the right eye and 0.04 in the left eye. There was no specific finding of anterior segment on slit lamp examination. On fundus examination, sheathed branch retinal veins were observed in the inferotemporal area of the right eye and superotemporal and inferotemporal areas of the left eye. Microangiopathies were observed around the occluded branch retinal veins in both eyes and macular edema was present in the left eye. Laser photocoagulation was performed at the non-perfusion area in both eyes and an intravitreal injection of bevacizumab and 3 intravitreal injections of triamcinolone were administered into the left eye. The BCVA did not change after 3 years and remained relatively stable. CONCLUSIONS: If a patient presents with decreased visual acuity after taking tamoxifen, fundus examination and fluorescein angiogram should be performed due to the possibility of branch retinal vein occlusion.

Posterior Surface Opacification of a Silicone Intraocular Lens in a Patient with Asteroid Hyalosis

PURPOSE: In the present study, a case of posterior surface opacification of a silicone intraocular lens (IOL) in a patient with asteroid hyalosis (AH) is reported. CASE SUMMARY: 76-year-old male was referred to our clinic with IOL opacification in his left eye. The patient had uneventful cataract surgery 7 years prior with the same silicone IOL implanted in both eyes. Three years after surgery, posterior capsular opacity was observed in his left eye and neodymium:YAG (Nd:YAG) laser capulotomy was performed. After posterior capsulotomy, opacification of the IOL's posterior surface was observed on slit lamp examination. IOL exchange was performed and the explanted IOL was analyzed using a light microscope and a scanning electron microscope with energy dispersive X-ray spectroscopy for elemental analysis of the deposits. The calcification was on the posterior surface of the IOL and composed mainly of calcium and phosphorus, the main components of AH. The right eye showed clear IOL with intact posterior lens capsule. CONCLUSIONS: Surgeons performing cataract surgery should consider the possibility of surface calcification of silicone IOLs in eyes with AH before IOL selection for implantation.

Prophylactic Vancomycin Drops Reduce the Severity of Early Bacterial Keratitis in Keratoprosthesis.

BACKGROUND: Artificial cornea transplantation, keratoprosthesis, improves vision for patients at high risk of failure with human cadaveric cornea. However, post-operative infection can cause visual loss and implant extrusion in 3.2-17% of eyes. Long-term vancomycin drops are recommended following keratoprosthesis to prevent bacterial keratitis. Evidence, though, in support of this practice is poor. We investigated whether prophylactic vancomycin drops prevented bacterial keratitis in an animal keratoprosthesis model. METHODOLOGY: Twenty-three rabbits were assigned either to a prophylactic group (n = 13) that received vancomycin 1.4% drops 5 times/day from keratoprosthesis implantation to sacrifice, or a non-prophylactic group (n = 10) that received no drops. All rabbits had Staphylococcus aureus inoculation into the cornea at 7-12 days post-implantation and were sacrificed at predetermined time-points. Prophylactic and non-prophylactic groups were compared with slit-lamp photography (SLP), anterior segment optical coherence tomography (AS-OCT), and histology, immunohistochemistry and bacterial quantification of excised corneas. Corneal vancomycin pharmacokinetics were studied in 8 additional rabbits. RESULTS: On day 1 post-inoculation, the median SLP score and mean±SEM AS-OCT corneal thickness (CT) were greater in the non-prophylactic than the prophylactic group (11 vs. 1, p = 0.049 and 486.9±61.2 vs. 327.4±37.1 µm, p = 0.029 respectively). On days 2 and 4, SLP scores and CT were not significantly different. Immunohistochemistry showed a greater CD11b+ve/non-CD11b+ve cell ratio in the non-prophylactic group (1.45 vs. 0.71) on day 2. Bacterial counts were not significantly different between the two groups. Corneal vancomycin concentration (2.835±0.383 µg/ml) exceeded minimum inhibitory concentration (MIC) for Staphylococcus aureus only after 16 days of vancomycin drops. Two of 3 rabbits still developed infection despite bacterial inoculation after 16 days of prophylactic drops. CONCLUSIONS: Prophylactic vancomycin drops provided short-term benefit, but did not prevent infection. Achieving MIC in the cornea was not sufficient to prevent Staphylococcus aureus keratitis. Patients should continue to be counselled regarding the risk of infection following keratoprosthesis.

A novel case of chronic conjunctivitis in a 58-year-old woman caused by Raoultella.

A 58-year-old woman presented to eye emergency with a chronic conjunctivitis which was diagnosed by laboratory microbiological testing to be due to the environmental pathogen Raoultella planticola. The organism was sensitive to Chloramphenicol and the patient made a rapid recovery on these drops. This is the first report of this organism infecting the eye.