Corneal Endothelium in Brown-McLean Syndrome: In-vivo Confocal Microscopy Finding

When Brown-McLean syndrome (BMS) was first described, there was no confocal microscopy to document corneal endothelial status, and it was understood as a disease entity involving a diseased endothelial layer. In the advent of confocal microscopy demonstrating healthy endothelium in BMS within the affected cornea, it can be inferred that BMS is a spectrum of disease ranging from healthy to decompensated corneal endothelium, rather than just a specific disease entity. Here we report a case of BMS with normal healthy corneal endothelium using corneal confocal microscopy. Any patient with BMS should be followed up to observe for any disease progression and should be educated regarding the signs and symptoms of corneal surface problems. Confocal microscopy documentation is a convenient and informative way and should form part of the follow-up of any BMS patient.     

Cataract surgery in a patient with Brown-McLean syndrome

The Brown-McLean syndrome is defined by corneal edema that involves the peripheral 2.0 to 3.0 mm of cornea, sparing the central cornea. This syndrome is usually associated with previous cataract surgery but has been reported rarely in patients with other predisposing factors such as angle-closure glaucoma or spontaneous lens absorption with iridodonesis. We describe the clinical course of a 50-year-old man with myotonic dystrophy who had Brown-McLean syndrome with no identifiable predisposition for peripheral corneal edema. Although this syndrome appears to be the result of peripheral endothelial dysfunction, this patient was able to tolerate cataract extraction without developing central corneal edema.     

Bilateral Implantation of Scleral-Fixated Cionni Endocapsular Rings and Toric Intraocular Lenses in a Pediatric Patient with Marfan's Syndrome

The management of ectopia lentis in Marfan''s syndrome is challenging. Multiple disease-associated factors conspire to deprive these patients of adequate vision. While optical correction with glasses and contact lenses is usually advocated early on, the irregular astigmatism and even partial aphakia that accompanies advanced cases generally warrant surgical intervention. Several surgical strategies have been devised to manage these challenging cases, including the combination of endocapsular or pars plana lensectomy and iris or scleral fixation of the intraocular lens (IOL) or IOL-bag complex. All of the reported cases have been implanted with IOLs that correct for myopia only. With toric lenses, it is now possible to correct for corneal astigmatism in these patients as well, provided that the capsular bag is maintained and can be properly centered. We report the combination of scleral-fixated Cionni endocapsular rings and toric IOLs in a pediatric patient with bilateral ectopia lentis secondary to Marfan''s syndrome.Key words: Ectopia lentis, Marfan''s syndrome, Toric intraocular lens, Cionni endocapsular ring     

CMV Retinitis in a Patient with Good Syndrome

Purpose: To describe cytomegalovirus (CMV) retinitis in a patient with Good syndrome. Methods: A 48-year-old patient with Good syndrome presented with a necrotizing retinitis in the left eye. Quantitative touchdown real-time polymerase chain reaction (PCR) was performed on aqueous fluid. Results: Quantitative PCR showed 152 copies of CMV per ml and was negative for varicella zoster virus (VZV), Epstein-Barr virus (EBV), herpes simplex virus (HSV-1), and HSV-2. The positive CMV PCR suggested CMV retinitis and the patient was treated with intravitreal ganciclovir injections (2.5 mg/0.05 ml), followed by ganciclovir implant. The retinal lesions showed decreasing activity two weeks after the onset of the therapy. A repeat PCR showed a decreasing number of CMV copies at one and two weeks (122 copies/ml and 0 copies/ml, respectively) that correlated clinically with the decreasing retinitis activity. Conclusions: Quantitative PCR can be useful in diagnosing as well as assessing the response to therapy of CMV retinitis in patients with Good syndrome.     

Anterior stromal puncture in Brown-McLean syndrome

We describe 2 cases of Brown-McLean syndrome with corneal edema successfully treated by anterior stromal puncture (ASP). Ophthalmologic examinations showed peripheral edema and aphakia in both patients. The patients had ASP to control the severe foreign-body sensation. The procedure was effective over a long-term follow-up, suggesting that peripheral ASP may be an effective therapeutic option for patients with symptomatic Brown-McLean syndrome.     

Pseudoexfoliation Syndrome in a Patient with Lattice Corneal Dystrophy

We report the case of a 70-year-old female who presents lattice corneal dystrophy type I in association with pseudoexfoliation syndrome. This association has never been reported in patients not affected by systemic amyloidosis. This revised version was published online in July 2006 with corrections to the Cover Date.     

Atypical VZV Retinitis in a Patient with Good Syndrome

Purpose: To describe atypical varicella zoster virus (VZV) retinitis in a patient with Good syndrome.

Methods: A 63-year-old patient with Good syndrome presented with bilateral necrotizing retinitis starting from the posterior pole. He had a history of thymoma status post thymectomy 4 years previously, left-sided sinusitis, and recent pulmonary aspergillosis. Qualitative PCR was performed on aqueous fluid.

Results: Immunological investigations revealed reduced levels of CD4⁺ T cells and immunoglobulins. Qualitative PCR was positive for VZV and negative for cytomegalovirus, herpes simplex virus (HSV)-1, and HSV-2. The patient was treated with oral valacyclovir and three courses of immunoglobulin supplementation. The atypical retinitis showed improvement after therapy.

Conclusion: Good syndrome should be considered in a patient with opportunistic infections and history of thymoma in the absence of human immunodeficiency virus. Atypical retinitis can occur in patients with Good syndrome and quantitative PCR is important for accurate diagnosis.     

Choroidal Tuberculoma in a Patient with Acquired Immunodeficiency Syndrome

Background: In Japan, since the introduction of antituberculosis chemotherapy, the typical choroidal tuberculoma has been considered uncommon. A patient with acquired immunodeficiency syndrome (AIDS), because of the suppression of cell mediated immunity, faces the risk of tuberculous infection.Case: A 30-year-old Malayan man had continuous cough for 6 months. He was diagnosed as having miliary tuberculosis of the lung and spine. Because the serum test of human immunodeficiency virus (HIV) was positive, he was also diagnosed as having AIDS.Findings: Fundus examination showed a yellow white swollen lesion of the choroid with distinct border in his right eye, probably caused by tuberculosis. After 3 months of therapy with antituberculosis and anti-HIV drugs, his systemic and ocular findings were markedly improved.Conclusion: Because of the recent increase in the incidence of tuberculosis with the epidemic of HIV prevailing in the world, the recognition of ocular tuberculosis is important.     

Anterior Ischemic Optic Neuropathy in a Patient with Churg-Strauss Syndrome

We describe a patient with Churg-Strauss syndrome who developed unilateral anterior ischemic optic neuropathy. A 54-year-old man with a history of bronchial asthma, allergic rhinitis, and sinusitis presented with sudden decreased visual acuity in his right eye that had begun 2 weeks previously. The visual acuity of his right eye was 20 / 50. Ophthalmoscopic examination revealed a diffusely swollen right optic disc and splinter hemorrhages at its margin. Goldmann perimetry showed central scotomas in the right eye and fluorescein angiography showed remarkable hyperfluorescence of the right optic nerve head. Marked peripheral eosinphilia, extravascular eosinophils in a bronchial biopsy specimen, and an increased sedimentation rate supported the diagnosis of Churg-Strauss syndrome. Therapy with methylprednisolone corrected the laboratory abnormalities, improved clinical features, and preserved vision, except for the right central visual field defect. Early recognition of this systemic disease by ophthalmologists may help in preventing severe ocular complications.     

Retinal Infarcts in a Patient with an Acute Confusional Syndrome

A 46-year-old man presented with acute confusional syndrome, ataxia, dysarthria, and right hemiparesthesia. Brain MRI showed small bilateral infarcts and fluorescein angiography revealed multiple peripheral retinal infarcts bilaterally. No visual loss was present, and no other organs were involved. The diagnosis of Susac syndrome (microangiopathy of the brain, retina and cochlea) was made and immunosuppressive therapy begun.     

Subretinal Fibrosis and Uveitis Syndrome in a Patient with Ectodermal Dysplasia

Purpose: To report a case of subretinal fibrosis and uveitis syndrome in association with ectodermal dysplasia.

Methods: Chart review of a patient with subretinal fibrosis and uveitis syndrome and ectodermal dysplasia.

Results: The authors identified one case of a patient with the diagnoses of subretinal fibrosis and uveitis syndrome and ectodermal dysplasia.

Conclusions: This represents an uncommon case of a patient with the diagnoses of both subretinal fibrosis and uveitis syndrome and ectodermal dysplasia.     

Idiopathic Intracranial Hypertension in a Prepubertal Pediatric Japanese Patient Complicated by Severe Papilledema

Purpose

To report a rare case of idiopathic intracranial hypertension (IIH) in a prepubertal pediatric patient.

Case Report

The patient was an 11-year-old Japanese boy. Initially, an ophthalmologist found severe papilledema, and the patient was diagnosed with IIH. He was unresponsive to conservative therapy, and a ventriculoperitoneal shunt was inserted in the Neurosurgery Department because the visual impairment was severe. Twelve months after the shunt, the improvement in vision was limited due to optic disc atrophy.

Conclusion

Ophthalmologists need to be more aware of II, especially in cases with severe papilledema that can lead to permanent reduction of vision.Key Words: Idiopathic intracranial hypertension, Papilledema, Ventriculoperitoneal shunt     

Bilateral Corneal Infiltrates and Uveitis in a Pediatric Patient with Presumed Ocular Sarcoidosis

Purpose: To report a pediatric case with presumed ocular sarcoidosis presented with bilateral corneal infiltrates and uveitis.

Design: Single case report.

Methods: A 9-year-old girl presented with a 1-month history of blurred vision and redness in both eyes. Ophthalmologic evaluation revealed midstromal infiltrates in cornea, granulomatous anterior uveitis, vitritis, snowball opacities, and localized perivenous exudates.

Results: She was treated with topical and oral steroids. During steroid tapering, oral cyclosporine A therapy was added.

Conclusion: In patients with corneal infiltrates and uveitis, sarcoidosis should be considered in the differential diagnosis.     

Variability in the clinical presentation of the Brown-McLean syndrome

CASE REPORT: We report two aphakic patients with Brown-McLean syndrome. DISCUSSION: One patient was affected by Marfan syndrome, after having undergone lens subluxation surgery and aphakia 23 years previously. The other patient was aphakic due to cataract surgery with complications three years before. Our cases demonstrate that this syndrome can show a variety of clinical characteristics, but peripheral corneal edema is always present. A full understanding of the clinical signs of presentation is of great importance in order to detect this syndrome.