Barry J. Frauens

Peripapillary schisis with serous detachment in advanced glaucoma.

Purpose. To describe features associated with the development and resolution of peripapillary retinoschisis with an underlying serous detachment in a patient with primary open angle glaucoma. This presentation occurred in the absence of an observed optic nerve coloboma, congenital, or acquired optic nerve head pit. Case Report. A patient with advanced glaucomatous optic nerve cupping developed a temporally localized peripapillary serous detachment in the right eye which spontaneously resolved. Results. Optical coherence tomography demonstrated an area of retinoschisis with underlying serous detachment contiguous with the temporal disc margin. Although fluorescein angiography was not performed and the presence of a peripapillary subretinal neovascular membrane could not be ruled out, an atypical coloboma, optic nerve head pit, or peripapillary subretinal neovascular membrane was not observed during biomicroscopy or scanning laser ophthalmoscopy. The retinoschisis and detachment resolved without intervention. Conclusion. Peripapillary retinoschisis with an underlying serous detachment may develop in subjects with advanced glaucoma. Although the occurrence of the findings in this case may be unrelated to glaucomatous optic neuropathy, the likelihood that a pathogenic mechanism linked to advanced glaucoma may be responsible for the development of peripapillary schisis and serous detachment should alternatively be taken into consideration. This case documents its spontaneous resolution without intervention.

Corneal copper deposition secondary to oral contraceptives.

Purpose. To document two cases of corneal pigmented rings and hypercupremia associated with estrogen-based oral contraceptive use. Other causes of corneal pigment rings including Kayser-Fleischer rings and the previously documented association of estrogens and hypercupremia are discussed. Case Reports. Two healthy female patients presenting to a general optometric practice for routine examinations were found to have bilateral bluish-green corneal deposits at the level of endothelium/Descemet’s membrane. The deposits were predominately in the inferior midperiphery in both patients, but formed a complete ring 3 years after initial presentation in one. Both patients were found to have abnormally elevated serum copper and elevated ceruloplasmin levels, and both were taking estrogen-based oral contraceptives. Serum copper levels returned to normal levels soon after cessation of the oral contraceptives, and subsequently re-elevated when a different oral contraceptive agent was tried. Although the pigment rings appeared different from typical Kayser-Fleischer rings, both patients were referred to gastroenterologists to rule out Wilson’s disease. Conclusions. This is believed to be the first report of corneal pigment deposition and hypercupremia associated with oral contraceptive use. Recognition of these potential side effects by eye care practitioners and prescribing physicians may spare patients the expense and morbidity risk of unnecessary medical testing. Further research is needed to determine the exact nature of the deposits (presumed to be a copper compound), and whether it may be accumulating to dangerous levels in other body tissues as in Wilson’s disease.

Choroiditis, pigment epithelial detachment, and cystoid macular edema as complications of poststreptococcal syndrome.

Purpose Two cases of poststreptococcal uveitis are presented. One patient developed nongranulomatous anterior uveitis (case 1). A second patient developed nongranulomatous anterior uveitis followed by granulomatous uveitis with concurrent choroiditis, retinal pigment epithelial detachment, and cystoid macular edema (case 2). Current concepts regarding the pathogenesis of poststreptococcal syndrome and its ocular sequelae are also discussed. Case Reports Case 1: A 31-year-old female patient presented with bilateral nongranulomatous uveitis after a recent onset of sore throat. A past diagnosis of rheumatic fever was revealed during acquisition of the patient’s history. Blood chemistry analysis indicated elevated anti-streptolysin O antibody titers as the only significant elevated result. Case 2: A 33-year-old female presented initially with bilateral nongranulomatous uveitis. A history of recurrent sore throat was reported. Blood chemistry analysis revealed elevated anti-streptolysin O antibody titers only. “Mutton fat” keratic precipitates eventually developed; however, the bilateral uveitis resolved after topical cycloplegic and corticosteroid treatment. An anterior granulomatous uveitis with concurrent choroiditis, retinal pigment epithelial detachment, and cystoid macular edema ensued during a recurrent episode. Blood chemistry analysis was unremarkable at that time. Conclusions. Anterior nongranulomatous/granulomatous and posterior uveitis should be considered clinical manifestations of poststreptococcal syndrome in patients with a clinical history and/or serological evidence indicating possible past streptococcal infection. A variation of host-genetic-predisposed immune response, the patient’s human leukocyte antigen haplotype, pathogen virulence, and/or deposition location of immune circulating complexes may give rise to the diverse spectrum of clinical ocular sequelae in poststreptococcal syndrome. Poststreptococcal uveitis may comprise a similar immunologic pathogenesis to that of acute rheumatic fever, acute glomerulonephritis, and other autoimmune diseases.

The clinical spectrum between posterior polymorphous dystrophy and iridocorneal endothelial syndromes

BACKGROUND There are many conditions affecting the corneal endothelium with similar clinical appearances, though with different prognoses, management approaches, and pathophysiologic development. CASE A 39-year-old black woman with a previous diagnosis of asymmetric corneal posterior polymorphous dystrophy (PPMD) presented complaining of irritation in the left eye, worsening over the last week. On examination, her left eye had profuse edema and bullous keratopathy overlying a large placoid gray lesion at the level of the endothelium. Gonioscopy of the left eye found evidence of a membranous development extending from the endothelium to the trabecular meshwork. Examination of her right eye found a perfectly clear cornea and a normal angle. The patient subsequently had Chandlers syndrome diagnosed in the left eye, an iridocorneal endothelial (ICE) syndrome, rather than PPMD. DISCUSSION PPMD and ICE syndromes present with many similarities; these clinical entities are in fact thought to be variants of the same disease. Unlike PPMD, Chandlers syndrome is unilateral, acquired, and typically symptomatic and progressive. It also characteristically affects middle-age women. CONCLUSION Understanding the clinical features of PPMD and ICE syndromes is important in proper diagnosis and management.

Unilateral granulomatous post-streptococcal uveitis with elevated tension.

Purpose. We document a presumptive case of unilateral post-streptococcal uveitis. The affected eye manifested a granulomatous anterior uveitis with elevated intra-ocular pressure (IOP) compared with the contra-lateral eye. This presentation was preceded by a recent streptococcal pharyngitis. Case Report. An asymptomatic, otherwise healthy 13-year-old black female presented with anterior chamber cells, mutton fat resembling keratic precipitates, and elevated IOP in the left eye relative to the right eye. The patient had been diagnosed with streptococcal pharyngitis approximately 1 week before her eye examination. A blood chemistry analysis, rheumatoid profile panel and antistreptolysin antibody titer were requested. The patient was prescribed predinisolone acetate 1% ophthalmic suspension in the left eye. Results. Anti-streptolysin antibody titers were significantly elevated (291 IU/ml). The patient responded favorably to topical ophthalmic steroid treatment with a reduction of IOP and inflammation. Conclusions. Unilateral, granulomatous anterior uveitis may be a variant manifestation of post-streptococcal uveitis. The IOP elevation in these cases may result secondary to concurrent trabeculitis.

Recurrent serous macular detachment after topical ocular hypotensive medication

We document the recurrence of a CSMD in a patient subsequent to the instillation of topical ocular hypotensive medications and its resolution on discontinuation of therapy. An independent cause or causes contributing to the development of CSMD other than the use of topical ocular hypotensive medications cannot be ruled out in this case, neither can it be considered dissociated from the use of these drugs. We recommend that patients with VTS, those with a history of CSMD or having developed recurrent episodes of CSMD requiring management with topical ocular hypotensive medications, be cautiously monitored for the possible occurrence or exacerbation of CSMD.

More sensitive correlation of afferent pupillary defect with ganglion cell complex

Purpose This study investigated the correlation between the relative afferent pupillary defect (RAPD) and retinal nerve fiber layer thickness (RNFLT) in optic neuropathy. Methods RAPD assessment was performed using a log unit neutral density filter bar. Spectral domain optical coherence tomography RTVue-100 (Optovue) was used to examine the subjects. The optic nerve head pattern (ONH) was subdivided and identified for the purpose of the study into circumpapillary RNFLT (cpRNFLT) and peripheral circumpapillary RNFLT (pcpRNFLT). The cpRNFLT, pcpRNFLT and ganglion cell complex (GCC) parameters were analyzed. Results Eighteen females and twenty three males with asymmetric optic neuropathy and a RAPD participated. Thirty-three subjects had glaucoma and eight had optic neuropathy other than glaucoma. Significant correlations (p < 0.02) were obtained for the RAPD and the percentage difference loss of the GCC and RNFLT parameters. The grouped mean percentage difference loss for RNFLT was significantly different from that of the GCC (p < 0.001). At a 0.6 log unit RAPD, the average mean percentage difference loss was 23% for the CRNFLT, 15% for the GCC, 12% for the global loss volume percentage and 6% for the focal loss volume percentage (FLV%). Conclusions Significant correlations between RNFLT loss for cpRNFLT, pcpRNFLT and GCC parameters with RAPD were observed. Approximately a 35% higher sensitivity was obtained using GCC compared to CRNFL parameters. The expected change in GCC average for every 0.3 log unit increment was approximately 8.49 μm. The FLV% corresponded more sensitively to a RAPD but appeared to be influenced by disease severity.

Focal loss volume best differentiates eyes with afferent pupillary defect

Purpose: To quantify the specificity and sensitivity of RTVue-100, Optovue parameters at determining the presence of a relative afferent pupillary defect (RAPD) by coupling their values with the instrument normative data base (NDB) and the presence of a RAPD. To generate the distribution for the focal loss volume (FLV) percentage, by coupling its value to the instrument NDB and presence of a RAPD. Methods: Forty one subjects with a RAPD and glaucomatous or non-glaucomatous optic neuropathy participated. A neutral density filter bar was used to estimate the RAPD. The specificity and sensitivity of all parameters was analyzed using Fisher’s Exact test. The distribution of the FLV percentage was analyzed using a one-way ANOVA with a Tukey HSD post hoc test. Results: The FLV percentage was more sensitive (100%) but less specific (64%) than other parameters. Eyes of subjects with FLV percentage and NDB p-values < 1% were 33.8 times more likely to have a RAPD [95% CI: 7.2, 325.1]. Confidence limits for the distribution of FLV percentage were; 6.32/10.59 um for eyes with NDB p-values < 1% with a RAPD; 2.04/7.56 um for eyes with NDB p-values >1% with a RAPD and 1.08/6.22 um for eyes with NDB p-values > 1% without a RAPD. Conclusion: Compared to other RTVue-100, Optovue parameters the FLV percentage is more sensitive at determining eyes with a RAPD and may significantly categorize optic neuropathy according to severity.

Comparative tomography of central serouschorioretinopathy

Purpose: To provide spectral domain optical coherence tomography (SDOCT) of subretinal fibrin leakage and concomitant central serous chorioretinopathy (CSC) morphology in four cases. Likewise, to compare the SDOCT of these cases with others documented. Case reports: Four cases of acute onset CSC exhibiting serous neurosensory and retinal pigment epithelium detachment (PED) associated with a hyper-reflective pattern in the subretinal space that appear to emanate from the PED are described. Initially, all cases showed thickening of the photoreceptor outer segment (POS) as well as zones where the inner segment-outer segment junction line (IS/OS) was not visible. All cases had an outer retinal “dipping” appearance. On follow-up visits all cases showed thinning of the POS with an increase of granular deposits on the posterior surface of the detached retina. As the CSC resolved, the IS/OS junction line became visible and the granular deposits diminished. One case exhibited a relative decrease in foveal thickness compared to the unaffected eye. Conclusion: SDOCT imaging of a hyper-reflective pattern at the subretinal space proximal to a PED in CSD may reflect resonance detection of fluid turbulences and density distribution disparities. This occurs as the serous fluid from the choroidal circulation diffuses into the subretinal space from a focal PED leakage site. A clear hypo-reflective area at the core of the hyper-reflective pattern may result from the distribution of less dense constituents centrally while hyper-reflective denser particles surround and concentrate at the peripheral border. The size of the leakage defect in the PED may determine the outline of the hyper-reflective pattern while the location may influence the spatial orientation. The POS swelling may impart pliability to the outer retina. The hydrostatic and fibrin particle pressure flowing from a PED on this retinal layer may contribute to the apparent outer retinal “dipping” imaged on optical coherence tomography.