Case Report: Corneal Ulceration from Bilateral Ectropion Due to Congenital Ichthyosis.

SIGNIFICANCE: Ichthyosis is a group of heterogenous inherited skin disorders characterized by abnormal cornification and keratinization of the skin. Autosomal recessive congenital ichthyosis presents with severe lagophthalmos and cicatricial ectropion of both upper and lower lids. Chronic corneal exposure from lid abnormalities may lead to ulcerative keratitis or corneal perforation. PURPOSE: The case highlights a rarely seen condition that presents with potentially serious ocular complications and vision loss. Corneal complications may be avoided or managed with moisture goggles, corneal vaulting with scleral lenses, topical therapeutics, amniotic membrane, and surgical lid repair. CASE REPORT: A 25-year-old woman presented with a painful right eye for 1 week. She had a medical history of autosomal recessive congenital ichthyosis. Her ocular adnexa revealed bilateral lagophthalmos and cicatricial ectropion of both upper and lower lids. The slit lamp of examination revealed an injected eye with corneal ulcer with hypopyon in the right eye and quiet eye with corneal scarring in the left eye. The patient was treated with topical moxifloxacin and polymyxin B sulfate/trimethoprim as well as in-office homatropine 5% in the right eye. The keratitis was treated to resolution, and the patient referred for consultation on lid repair. CONCLUSIONS: Chronic corneal exposure from autosomal recessive congenital ichthyosis may lead to severe dry eye, ulcerative keratitis, or perforation. Patients should be monitored carefully for corneal disease, educated on methods and devices to protect the corneal surface, and referred for surgical repair if indicated. Although rare, this condition presents unique findings that may be visually devastating. Awareness of the condition, as well as the various clinical presentations and appropriate management necessary, will prove beneficial to the patient.

Atypical retinal vaso-occlusion with structural and functional resolution.

PURPOSE: The purpose is to report a patient with primary open-angle glaucoma that developed sudden painless unilateral vision loss, a sequential ophthalmoscopic appearance with features of both central retinal artery and later central retinal vein occlusion, and objective visual system dysfunction in the form of a relative afferent pupil defect, who spontaneously recovered vision along with complete resolution of the pupillary defect over several weeks. CASE REPORT: A 50-year-old woman with a long-standing history of glaucoma presented with acute, painless vision loss in one eye, a pallid retina with a cherry red macula, diffuse retinal hemorrhages, and a relative afferent pupil defect. Spectral domain optical coherence tomography and fluorescein angiography were essentially normal with neither retinal edema nor retinal ischemia to account for the visual dysfunction. Over the course of 2 months, the patient regained vision and the relative afferent pupil defect, typically a permanent manifestation of retinal destruction, resolved. CONCLUSIONS: Not all retinal vaso-occlusive phenomena can be completely attributed to a central retinal vein or artery occlusion. In the patient presented, there was no objective diagnostic testing that revealed a cause for the patient's vision loss or relative afferent pupillary defect. This combined with the complete recovery of vision and resolution of the relative afferent pupillary defect underscores a lack of comprehensive understanding of retinal vaso-occlusive disease.

The diagnosis and management of ocular lymphoma.

PURPOSE: Lymphoma is the most common malignancy of the ocular adnexa. Most of the ocular adnexa lymphomas are non-Hodgkin B-cell lymphomas. The most common type of ocular adnexa lymphoma is primary extranodal marginal zone B-cell lymphoma of the MALT (mucosa-associated lymphoid tissue). Most of these neoplasms are primary extranodal lymphomas, although 10% to 32% are secondary tumors from disseminated disease. CASE REPORT: A 58-year-old woman presented for a comprehensive examination, with the chief complaint of ocular discomfort in both eyes. Anterior segment examination revealed bilateral salmon-colored lesions of the inferior and superior conjunctivae. The patient was referred for systemic evaluation and histopathology of the conjunctival lesions. She was diagnosed as having marginal zone lymphoma of the MALT and underwent radiation therapy (RT). CONCLUSIONS: Ocular lymphoma may present on routine examination or with mild symptoms. Although most commonly a primary extranodal neoplasm, the condition may be associated with disseminated lymphoma and requires thorough evaluation and staging of the disease for determination of appropriate treatment. The primary eye care provider plays an important role in the identification and staging of the disease, as well as managing complications from RT. It is also important to recognize that concurrent conditions requiring treatment with topical medications, such as glaucoma, may be complicated after treatment because of the inflammation and ocular surface irritation after RT. The necessity and benefit of the addition of intraocular pressure medications during that time should be measured on a case-by-case basis. Patients should be followed closely after treatment for relapse of disease and identification of complications from ocular RT.

Central corneal thickness measurements obtained with anterior segment spectral domain optical coherence tomography compared to ultrasound pachymetry in healthy subjects.

INTRODUCTION: Central corneal thickness (CCT) imparts information about an individual's risk of conversion to glaucoma from ocular hypertension, progression of established glaucoma, and the likelihood of developing structural and functional abnormalities in patients with ocular hypertension. Most typically, CCT is measured through ultrasound (US) pachymetry. Currently, optical coherence tomography (OCT) has the ability to image the anterior segment, cornea, and anterior chamber angle. With this ability comes the option of determining CCT. The purpose of this study is to ascertain any significant difference in CCT measurement results as well as quantify the reproducibility of measurements of the two technologies. In addition, by measuring CCT both with traditional US pachymetry as well as spectral domain (SD) OCT technology, we sought to determine if CCT measurement by SD-OCT is an accurate, comparable and viable option; METHODS: Eighty eyes of forty healthy volunteers were used to determine CCT with both SD-OCT and US pachymetry. Three consecutive measurements were collected with each method on every eye. RESULTS: CCT measurements made by US pachymetry and SD-OCT were similar and consistent (r=0.99 for both methods). CCT measurements made by SD-OCT were consistently thinner by approximately 12 micrometers than measurements made by US pachymetry. Repeated measurements of CCT obtained by SD-OCT were more reproducible and had less variability than measurements obtained by US pachymetry. The mean within-subject standard deviation among SDOCT was significantly smaller than that in US pachymetry (1.92 in SD-OCT vs. 2.04 in US pachymetry, p=0.036); CONCLUSIONS: Measurement of CCT by SD-OCT compares favorably with and is at least as accurate as measurements made by US pachymetry. Repeat measurements of CCT by SD-OCT have less variability than those obtained by US pachymetry, are more reproducible, possibly more reliable, and may better represent actual CCT.

Intraocular pressure elevation from topical difluprednate use.

INTRODUCTION: Difluprednate ophthalmic emulsion 0.05% (Durezol™, Alcon, Fort Worth, Texas) is a topical difluorinated derivative of prednisolone with potent anti-inflammatory activity. Difluprednate 0.05% has a reported associated increase in intraocular pressure (IOP) in 3% of patients. Although the occurrence may be low, the possible elevation in IOP may be substantially higher than commonly encountered with other topical steroids. CASE REPORTS: A 49-year-old black man presented with a traumatic anterior uveitis recalcitrant to traditional prednisolone acetate 1% treatment. The patient was switched to difluprednate 0.05% in an attempt to better control the ocular inflammation. Although the patient did not exhibit an IOP response after 4 weeks of treatment with prednisolone acetate 1%, he did experience a pressure response within 2 weeks of initiating difluprednate treatment, resulting in an IOP increase from 9 mmHg to 48 mmHg with subsequent microcystic edema. A 44-year-old black woman presented with recurrent scleritis resistant to topical prednisolone acetate, loteprednol etabonate, and oral nonsteroidal anti-inflammatory therapy. Topical loteprednol 0.5% was replaced by difluprednate 0.05%. All evidence of ocular inflammation was eradicated with the changed therapy. IOP rose in the difluprednate-treated eye from 18 mmHg to 34 mmHg over the course of 18 days. In both cases, the IOP elevation was managed rapidly with the discontinuation of difluprednate and temporary use of IOP-reducing agents with no lasting adverse effects. CONCLUSION: Difluprednate 0.05% is a new topical therapeutic option indicated for the treatment of inflammation and pain management associated with ocular surgery with an off-label potential for treatment of other anterior segment inflammatory conditions. However, clinicians need to be aware of the potential risk for significant and potentially rapid onset of IOP increase with this medication and manage patients accordingly.

Superior segmental optic nerve hypoplasia: The topless disc syndrome.

BACKGROUND: Optic nerve hypoplasia is a well-known congenital maldevelopment presenting with an abnormally small optic nerve head occupying the central aspect of a normally sized chorioscleral canal. Characteristically, the optic nerve head is surrounded by scleral anlage with a "double ring sign." Less commonly appreciated, however, is the fact that optic nerve hypoplasia may be sectorial rather than total and involving only the superior aspect of the optic disc with corresponding inferior visual field loss. CASE REPORT: A 51-year-old woman presented with a previous diagnosis of idiopathic optic atrophy superiorly in the left eye. Detailed observation revealed that the disc was not atrophic superiorly but actually hypoplastic, and the patient received a conclusive diagnosis of superior segmental optic nerve hypoplasia. CONCLUSIONS: It must be appreciated that optic nerve hypoplasia can also affect solely the superior aspect of the disc with subsequent functional deficits. It is important to differentiate this syndrome from true optic atrophy to ensure proper management.

Proliferative vitreoretinopathy as a late complication of blunt ocular trauma.

BACKGROUND: Blunt ocular trauma to the eye can manifest in many ways. However, it is unknown how long patients are at risk for certain complications after the initial injury. Proliferative vitreoretinopathy is one possible complication of ocular trauma. This condition generally results from severe ocular trauma, chronic retinal detachment, or retinal surgery. The nidus for proliferation is unclear; however, once formed, proliferative membranes create traction at the vitreoretinal surface, leading to further complications and ocular morbidity. CASE REPORT: A 34-year-old man presented emergently with decreased vision in the left eye for 3 weeks and pain for 3 days. The patient denied any recent trauma or systemic disease but did report trauma to his left eye approximately 5 years earlier. His best-corrected acuities were 20/20 in the right eye and light perception in the left eye. The anterior segment evaluation found a nearly complete hyphema, iris neovascularization, and superior uveal prolapse through scleral perforation of the left eye. Retinal detachment and proliferative vitreoretinal disease were also discovered, necessitating surgical repair. CONCLUSION: Proliferative vitreoretinopathy is associated with severe ocular trauma. In this case, the patient had a markedly delayed onset of proliferative retinopathy resulting from blunt trauma with partial perforation 5 years before. Clinicians should be aware of the potential for the development of proliferative vitreoretinopathy as a long-term complication of blunt ocular trauma as well as the treatment options for this disease.

Optic atrophy after sildenafil use.

BACKGROUND: It has been well-reported that phosphodiesterase-5 (PDE-5) inhibitors, originally investigated for their effect on smooth muscles and now used widely in treatment of erectile dysfunction, can cause mild transient visual disturbances because of their action on inhibiting enzymes involved in retinal transduction. Recently, these medications have been associated with the development of non-arteritic anterior ischemic optic neuropathy (NAAION) with attendant vision loss. CASE REPORT: An older male patient, previously examined and ocularly healthy, presented asymptomatically with an occult optic neuropathy, not characteristic of NAAION. Neuroimaging and serology failed to reveal any other underlying cause. The patient did, however, report the use of sildenafil during the interval between his previously normal examination and the observation of his optic neuropathy. CONCLUSIONS: This case details the development of an optic neuropathy with atrophy seemingly associated with the use of sildenafil, although no cause and effect could be conclusively found. This may indicate that medications used in the treatment of erectile dysfunction may be responsible for optic neuropathies other than NAAION.

Ocular vascular hamartomas: the relationship with phakomatoses and possible commonalities in pathogenesis.

BACKGROUND: Ocular vascular hamartomas may present as isolated lesions or as part of a multisystemic congenital syndrome known as vascular phakomatoses. These syndromes are characterized by ocular, cerebral, and cutaneous lesions. Although each of these lesions manifests distinct characteristics, there may be overlapping characteristics and manifestations attributable to a common pathogenesis. METHODS: A case series and a literature review are presented illustrating ocular and systemic manifestations associated with vascular hamartomas, with each case representing varying degrees of ocular and systemic expression. CONCLUSION: Because of the potential ocular morbidity and systemic mortality associated with vascular hamartomas and associated syndromes, the optometrist plays an important role in the management of these patients. In general, understanding the pathogenesis of these diseases may contribute to future treatment and prevention opportunities.

Rapid onset phacolysis.

BACKGROUND: Phacolysis involves the breakdown of a hypermature cataract, causing an antigenic reaction to the lens proteins released into the anterior chamber with subsequent inflammation. To date, the time it takes for a crystalline lens to reach hypermaturity and induce a phacolytic response has never been clearly detailed. It is believed that cataract maturation is a slow process. The process by which the lens proteins begin to leak is thought by many to be similarly slow. However, the immune-related inflammatory process that develops when the lens proteins begin to leak may be quite rapid. It may be an error to consider this aspect of the phacolytic process to be slow. METHODS: We present a case with a clear, timed delineation of the phacolytic process. A mature cataract became hypermature with subsequent phacolysis and inflammatory pressure rise over the course of 17 days. It appears that this is the first published account of the time involved in the development of phacolysis and, we believe, the fastest onset of the process. CONCLUSION: While cataract maturation is generally considered to be a slow, insidious process, it should be recognized that the phacolytic process might not be slow. Once a lens reaches hypermaturity, phacolysis could occur quite rapidly over the course of several days. This case could be an important consideration in management of the removal of advanced cataracts. This case may be the shortest reported time from diagnosis of a mature cataract to the development of inflammatory phacolysis and secondary glaucoma, occurring over a period of only 17 days.