New study suggests radiation often underused for palliation

BACKGROUND: The exact pathogenic mechanism of the accumulation of subretinal fluid at the posterior pole of the fundus in cases of central serous chorioretinopathy (CSC) is not well established. Recently, it was reported that CSC is more frequent among patients with endogenous Cushing's syndrome. Thus, it has been suggested that glucocorticoids might be involved in the pathogenesis of CSC. Subsequently, additional observations, have confirmed the relationship between glucocorticoids and CSC. We present preliminary data on the endogenous cortisol secretion in patients with CSC. PATIENTS AND METHOD: Sixteen patients (14 men and 2 women, 35-65 years of age) suffering from CSC, not exposed to exogenous glucocorticoids and without clinical and/or biological stigmata of endogenous Cushing's syndrome, have been examined. Twenty four hour urinary free cortisol (24 h-UFC) secretion was measured within one week of their CSC episode. Twenty four hour urinary free cortisol of age and sex matched controls were also measured. RESULTS: Twenty four hour urinary free cortisol was 188.20 nmol/l +/- 34.1 for the patients suffering from CSC and 115.3 nmol/l +/- 63.4 for the control group (p < 0.05). CONCLUSION: These results give additional evidence that glucocorticoids may play a role in the pathogenesis of CSC. However, given the substantial variability of urinary free cortisol levels, as indicated by the increased SD, additional number of patients should be examined.     

Physical and linkage mapping of human chromosome 17 loci to dog chromosomes 9 and 5

We performed fluorescein and indocyanine green (ICG) angiographies in 56 patients with central serous chorioretinopathy, and studied the choroidal lesions. In the early phase, choroidal filling with ICG was delayed in 77% in the area including focal leakage. Hypofluorescent findings around the site of focal leakage persisted through the phase in 23%, and we think this finding was caused by filling defect of the choriocapillaris. In the late phase, choroidal tissue staining by ICG was present in 82% in the area including focal leakage. Multiple areas of choroidal staining were also present in unaffected areas in 43% and in 62% of fellow eyes. Choroidal tissue staining by ICG was revealed in 48% in the area of choroidal filling delay, and this finding persisted after focal leakage had disappeared following photocoagulation. We think this finding was caused by choroidal vascular hyperpermeability. These findings suggest that choroidal circulatory disturbance and choroidal vascular hyperpermeability play a causative role in damage to the retinal pigment epithelium in central serous chorioretinopathy.     

Clinical grand rounds. Idiopathic central serous chorioretinopathy

Idiopathic central serous chorioretinopathy is a very commonly seen disease process involving atypical RPE cells allowing the development of a neuroepithelial retinal detachment. Typically, this disease is self-contained and resolves spontaneously; however, on occasion, one must intervene by treating the area of atypical RPE with laser photocoagulation. Patients with ICSC should be monitored closely for any signs of choroidal neovascular membrane.     

Digital indocyanine green angiography in chorioretinal diseases

BACKGROUND: Fluorescein angiography (FA) is important in the diagnosis of chorioretinal diseases; however, it has some limitations. We conducted this study to evaluate whether digital indocyanine green (ICG) angiography can offer enhanced image in chorioretinal diseases. METHODS: Digital indocyanine green angiography with scanning laser ophthalmoscope (SLO) was performed in 314 patients with various chorioretinal diseases. This coupled digital imaging system can offer instant images, process and store data by computer, and give convenient hardcopy generation. RESULTS: The digital ICG angiography provided enhanced image resolution of the choroid compared with FA. The disease categories included choroidal neovascularization (CNV), choroidal tumor, inflammatory choroidal disease, ischemic choroidal disorder, idiopathic central serous chorioretinopathy and retinal macroaneurysm. CONCLUSIONS: ICG angiography is a useful adjunctive diagnostic technique to fluorescein angiography for various chorioretinal diseases. It is especially useful in the diagnosis of occult and recurrent CNV, as well as choroidal tumor and choroiditis. With greater clinical experience, ICG angiography is promising in giving additional clinical information for many other chorioretinal diseases.     

Choroidal vascular lesions in serous retinal detachment viewed with indocyanine green angiography

In serous retinal detachment due to damaged retinal pigment epithelium (RPE), fluorescein angiography shows dye leakage into the subretinal space from the choroid. We performed indocyanine green (ICG) angiography in 110 eyes with serous retinal detachment comprising 71 eyes with central serous chorioretinopathy (CSC), 19 with bullous retinal detachment, 18 with Harada's disease, and 2 with toxemia of pregnancy. Choroidal tissue staining was present around the site of subretinal leakage in late-phase ICG angiograms from 63 eyes with CSC and 18 with bullous retinal detachment. ICG angiography also showed leakage from choroidal vessels in 16 eyes with Harada's disease and 2 with toxemia of pregnancy. As a common feature, ICG angiography showed choroidal vascular hyperpermeability in various types of serous retinal detachment. Choroidal circulation was delayed in Harada's disease and toxemia of pregnancy. Choroidal hypoperfusion and hyperpermeability of choroidal vessels probably contribute to the damage of RPE, and choroidal vascular hyperpermeability probably provides fluid pressure to move fluid into the subretinal space from the choroid.     

Macular imaging with optical coherence tomography

PURPOSE: Optical Coherence Tomography (OCT) is a novel noninvasive and noncontact imaging technique providing cross-sectional representations of the eye structures. OCT is analogous to Ultrasound B-scan, except that it analyzes the reflection of a 850 nm light wave. The aim of this study was to assess the potential of ocular coherence tomography for diagnosing and monitoring macular diseases. METHODS: Cross-sectional images were performed with the Zeiss-Humphrey OCT. Over one year period, we examined approximately 300 patients with idiopathic full thickness macular hole, lamellar hole, cystoid macular edema, choroidal new vessels, epiretinal membrane, diabetic maculopathy, and central serous chorioretinopathy. RESULTS: OCT can provide new information concerning the posterior pole diseases mentioned above. OCT can also be useful in thickness measurements. CONCLUSION: OCT allows tomographic analysis of macular diseases. The information obtained is different from that obtained by histologic study which is sometimes hard to interprete. OCT is mostly useful in studying internal layers of the retina. Further applications may be developed.     

Central serous chorioretinopathy. Clinical, fluorescein angiography and demographic aspects

The purpose of this retrospective study was to analyze the demographic characteristics of central serous chorioretinopathy (CSC). METHODS: Findings of 100 consecutive subjects with CSC were evaluated. Clinical and fluorescein angiographic findings, demographic characteristics, and visual acuity were analyzed. RESULTS: The age of the patients ranged from 28 to 68 years with a mean of 43 years. No significant sex differences were found concerning age and other parameters. The highest age peak was in the group of women. The male to female ratio was 5:1. Patients with chronic CSC were significantly older (P = 0.015) than patients with the other angiographic findings. Median visual acuity was 0.5. In 40% bilateral characteristics of CSC were found. Clinical and fluorescein angiographic findings showed no significant correlation with visual acuity. CONCLUSION: The range of age distribution in CSC is wide. In older patients distinguishing CSC from age-related macular degeneration can be difficult.     

What''s in a name? The 22q11.2 deletion

PURPOSE: To document that lacunar, atrophic lesions of the retinal pigment epithelium, previously reported as a complication of treatment, can result from the natural course of retinopathy of prematurity. METHODS: We reviewed photographs of patients diagnosed with retinopathy of prematurity at the Casey Eye Institute between 1979 and 1996. Lacunar atrophic lesions of the retinal pigment epithelium were correlated with the clinical records of the affected patients. RESULT: Three untreated eyes of three patients with retinopathy of prematurity had lacunar atrophic lesions of the retinal pigment epithelium. CONCLUSIONS: The spectrum of findings associated with untreated retinopathy of prematurity includes lacunar, atrophic lesions of the retinal pigment epithelium. These lesions are distinct from scars secondary to treatment and are possibly linked to macular dragging and exudative or serous retinal detachment.     

Long-term follow-up of severe central serous chorioretinopathy using indocyanine green angiography

BACKGROUND: The severe types of central serous chorioretinopathy (CSC) have a chronic nature, suggesting that a pathological process persists subclinically. Indocyanine green (ICG) angiography recently revealed intrachoroidal dye leakage and its static nature in CSC. As the intrachoroidal dye leakage was suspected to be relevant to the disease process, the long-term persistence of intrachoroidal ICG leakage was examined in four patients of the severe types of CSC. METHODS: ICG angiography was performed periodically over more than three years in three patients and two years in one patient. One patient had CSC with bullous retinal detachment, and the other three had chronic CSC or diffuse retinal pigment epitheliopathy. RESULTS: Intrachoroidal ICG leakage persisted in all the patients. However, a change in location of persistent intrachoroidal leakage or disappearance of intrachoroidal leakage regardless of no progression of retinal pigment epithelial alteration was noted in one eye of two patients. CONCLUSIONS: Pathology causing intrachoroidal ICG leakage persisted subclinically for a long period. However, location and extent of the intrachoroidal leakage could change during a long-term follow-up period.     

Choledocholithiasis: endoscopic versus laparoscopic management

Although small retinal bleeding has been evaluated as a complication of filtering surgery, long-standing retinal bleeding is rare. We report 3 rare cases of young patients with massive retinal hemorrhages following trabeculectomy with 5-fluorouracil or mitomycin C. The bleeding in 1 case resolved spontaneously, whereas the bleeding in the other 2 cases did not resolve and the visual acuity remained poor. Every patient showed markedly delayed retinal circulation. Possible dysfunction of the retinal blood vessels may be responsible for the development of the other two types of massive bleeding, especially in advanced glaucoma with a total cup disk: one is decompression retinopathy and another central retinal vein occlusion or hemorrhagic retinopathy.     

Central serous chorioretinopathy associated with inhaled or intranasal corticosteroids

OBJECTIVE: The purpose of the study is to investigate the relationship between inhaled or intranasal adrenergic agonists and corticosteroids and the development of central serous chorioretinopathy (CSC). DESIGN: The medical records of three patients with CSC who were found to use inhaled adrenergic agents or corticosteroids or both were identified prospectively. A survey of members of the Retina, Macula, and Vitreous societies and the National Registry of Drug-Induced Ocular Side Effects identified three additional cases. RESULTS: Six patients with CSC were found to be chronic users of corticosteroid (four patients) or both beta adrenergic agonist and corticosteroid (two patients) metered dose inhalers or nasal sprays. In three cases, there was a close temporal correlation between the use of a corticosteroid nasal spray and the development of CSC. CONCLUSIONS: These findings suggest that, in patients who are susceptible, the periocular or systemic absorption of inhaled corticosteroids may be sufficient to produce CSC in humans, supporting previous hypotheses regarding the pathogenesis of the disorder. Further studies are needed to confirm this association and to determine whether inhaled adrenergic agents also contribute to the development of this disorder. Patients in whom CSC develops while using corticosteroid inhalers or nasal sprays should be alerted to the possible relationship between CSC and these agents.     

Solar retinopathy in a hospital-based primary care clinic

BACKGROUND: Most reports of solar retinopathy describe epidemics of patients who go to the eye doctor after viewing a solar eclipse. Rarely is it encountered by the primary eye care provider during a routine eye examination. METHODS: For 26 months, patients who went to the primary care eye clinic and found to have macular lesions consistent with solar retinopathy were identified from the total clinic population. These patients were documented in a coded log and fundus photographs were obtained (when possible). RESULTS: Twenty-six eyes of twenty patients (0.14% incidence) were determined to have macular lesions consistent with solar retinopathy. Visual acuity was 20/25 or better in 100% of the patients and 85% were 20/20. Patients were predominantly men (75%) of middle age (average age, 43 years; SD, 11 years) with a history relevant for solar retinopathy (80%)--consisting of sungazing, 60%; looking at welding light without eye protection, 15%; substance abuse, 15%; and psychiatric condition, 5%. Forty percent had solar lesions in both eyes. Amsler grid testing revealed a defect in only 20%, and macular threshold visual-field testing was normal in all the eyes tested. CONCLUSIONS: This is the first report to characterize solar retinopathy in a primary eye care population. Management includes correct differentiation from other macular disorders, acquisition of a careful detailed history, and provision of patient education regarding the dangers of sungazing.     

Plasma cell content of main and accessory lacrimal glands and conjunctiva

Chloroquine compounds are known to cause a retinopathy which typically begins in the central fundus giving rise to a "bull's eye" macula. Ultimately peripheral changes may become apparent. In the routine eye examination of such patients emphasis has been laid on the central area of the fundus. A case is presented where the retinopathy was not diagnosed until marked peripheral changes had occurred with peripheral pigment changes, attenuated retinal vessels, slight optic atrophy, peripheral visual field restriction and a subnormal electroretinogram. The typical "bull's eye" changes were not apparent. Routine examination of the peripheral fundus by means of ophthalmoscopy and perimetry is necessary to avoid missing any such retinopathy.     

Undifferentiated carcinoma of the liver with neuroendocrine features: a case report

The authors present an account on their first experience with treatment of central serous chorioretinopathy (CSCHR) by beta-blocking agents. In 21 patients with CSCHR Trimepranol (metipranololum) a beta non-selective blocker, 2 x 10 mg/day for two to three months was used. In 30 patients with CSCHR Vasocardin (Metoprololi tartas) was used i.e. a beta-1 selective blocking agent, 2 x 50 mg/day for two to three months. In all patients remission of the disease occurred, on average 4.5 to 4.8 weeks after the onset of treatment. During treatment of CSCHR by beta-blocking agents no significant difference was found in the action of beta selective and non-selective blockers regardless of the duration of the disease before onset of treatment and the number of relapses.     

Progression of retinopathy after change of treatment from oral antihyperglycemic agents to insulin in patients with NIDDM

OBJECTIVE: In insulin-dependent diabetes mellitus, institution of good glycemic control has been shown to retard development of retinopathy even though temporary progression has occurred. Few data have been available from patients with non-insulin-dependent diabetes mellitus (NIDDM). To determine the impact of improved glycemic control on retinopathy in patients with NIDDM, we examined, in a case-control study, the progression of retinopathy in 94 patients who changed treatment from oral antihyperglycemic agents to insulin. RESEARCH DESIGN AND METHODS: We used the Wisconsin retinopathy scale and related progression of retinopathy during a 2-year observation period to changes in HbA1c after institution of insulin therapy. RESULTS: Progression of retinopathy > or = 3 levels occurred in 23% of the patients and was significantly more common in the patient group in which HbA1c was lowered > or = 3% compared with progression in the group in which HbA1c was lowered < 3% (P = 0.0001; relative risk 3.2; 95% confidence interval 1.5-6.9). CONCLUSIONS: Improved glycemic control as achieved by insulin therapy may be associated with worsening of retinopathy in patients with NIDDM.     

Infectious simian varicella virus expressing the green fluorescent protein

PURPOSE: To determine if vascular occlusion and nonperfusion is associated with the outer retinal atrophy, retinopathy, and choroidopathy (chorioretinopathy) that occurs in the alpha H beta S[beta MDD] and alpha H beta S [alpha MD beta MDD] transgenic mouse models of sickle cell disease. METHODS: Mice from the alpha H beta S[beta MDD] and alpha H beta S[alpha MD beta MDD] transgenic mouse lines that express high levels of human beta S globin were anesthetized and administered horseradish peroxidase (HRP) intracardially. After 1 min, the animals were sacrificed, and the retina from one eye was excised, fixed, and developed in diaminobenzidine (DAB). The contralateral eye was fixed, embedded whole in glycol methacrylate, and HRP developed in 2.5 microns sections. RESULTS: HRP reaction product (HRP-RP) and stained erythrocytes (RBCs) (due to endogenous peroxidase) were diffusely distributed within all vascular lumens in flatmount retinas from control animals (littermates homozygous for the mouse Beta Major deletion not expressing the beta S transgene). In 42.5% of the transgenic mice expressing beta S without any proliferative retinopathy, many blood vessels contained RBC plugs and lacked lumenal HRP-RP. In addition to packed RBCs, fibrin was sometimes present at sites of occlusion. In sections from whole eyes of the same animals, foci of photoreceptor degeneration were associated with areas of choriocapillaris nonperfusion (lumen that lacked HRP-PR). In areas with normal photoreceptors, the choriocapillaris appeared perfused (HRP-RP was present). In animals with proliferative chorioretinopathy, some neovascular formations lacked luminal HRP-RP, suggesting autoinfarction. CONCLUSIONS: Nonperfused retinal and choroidal vessels were observed in mice from the alpha H beta S[beta MDD] and alpha H beta S[alpha MD beta MDD] lines without retinal and choroidal neovascularization, whereas, all mice with neovascularization had nonperfused areas. Furthermore, small foci of PR loss were associated with areas of nonperfused choriocapillaris. These results suggest that sickle cell-mediated vaso-occlusions are an initial event in the chorioretinopathy and outer retinal atrophy that occurs in these models.     

The long-term outcome of central serous chorioretinopathy

OBJECTIVE: To assess the long-term outcome of central serous chorioretinopathy (CSR) among a group of patients who previously participated in a prospective argon laser photocoagulation study of CSR. DESIGN: Thirty-eight of 41 surviving patients with CSR participating in an earlier study were invited to participate in a follow-up study that included history taking, ophthalmoscopy, biomicroscopy, and fundus photography. RESULTS: Thirty-seven (38 eyes) of 38 surviving patients (97%) were available for follow-up between 11 and 15 years after participation in the earlier study. There were no clinically documented or historical recurrences of CSR among the six eyes previously treated by direct laser photocoagulation. There were 13 clinically documented recurrences and four historical recurrences among the 32 eyes not treated with direct laser photocoagulation. The difference in recurrences was statistically significant (P = .02). Pigment changes indistinguishable from age-related macular degeneration frequently occurred in eyes with CSR. The difference in the development of such pigment changes between eyes with CSR (33 of 38) and nonaffected fellow eyes (12 of 35) was significant (P = .001). CONCLUSIONS: The decreased rate of CSR recurrence after direct laser photocoagulation reported in an earlier study was sustained with follow-up beyond 10 years. Pigmentary changes in the fundus indistinguishable from those associated with age-related macular degeneration developed in eyes affected with CSR, probably as a consequence of the presence of subretinal fluid accompanying the CSR rather than from early age-related macular degeneration.     

Effect of tubule orientation in the cavity wall on the seal of dental filling materials: an in vitro study

BACKGROUND: Surgery for macular gliosis and macular holes has become increasingly successful with regard to anatomical outcome. Assessment of the damage to the receptors by these processes is still difficult, but is important in predicting functional outcome. METHODS: Examination with the Nagel II or the Neitz OT anomaloscope was performed in 36 patients with macular gliosis, 23 patients with full-thickness macular holes and 47 patients with central serous choroidopathy. The anomaloscope matches were expressed as the quotient of anomaly. RESULTS: In macular gliosis the mid-matching point is usually 1.0; there is no pseudoprotanomaly. In macular holes the mid-matching point is 1.0 when visual acuity is 0.3 or greater; in eyes with lower visual acuity there may be signs of diminished red sensitivity, but anomaloscope examination becomes difficult. In central serous choroidopathy the mid-matching point is shifted towards red, and pseudoprotanomaly is present, even when visual acuity is normal. CONCLUSIONS: Diseases of the inner retina, in early stages, do not alter colour vision substantially, whereas diseases of the outer retina give rise to early colour vision deficiency. In macular gliosis and macular holes, anomaloscope examination enables estimation of macular receptor misalignment.     

Retinitis pigmentosa inversa

BACKGROUND: Retinitis pigmentosa (RP) is one of the most common inherited retinal diseases, with a prevalence of about 1 in 3500 to 4500. Retinitis pigmentosa inversa is a rare variant of this disorder characterized by areas of choroidal degeneration with pigment migration and bony spicule formation in the macular area. In contrast to more typical forms of RP, this anomaly destroys central vision, leaving peripheral vision intact. CASE REPORT: A 47-year-old white male was followed for about 7 years with evidence of progressive retinal pigment epithelial atrophy and hyperpigmentation affecting both maculae. Since 1970, he had noted difficulty seeing at night as well as an acquired hearing deficit that appeared to be getting worse, ultimately impairing his ability to safely drive a truck. Medical history was positive for either chloroquine or hydroxychloroquine use for 2 to 3 years as malaria prophylaxis while he served in Vietnam. In addition, his father in Louisiana had visual loss of unknown cause. During the 7-year period, the condition progressed rapidly. The patient became virtually blind secondary to visual acuity loss with dense central and paracentral scotomas. The peripheral visual fields remained intact. After several years of extensive examinations, including laboratory, electroretinography, and genetic testing, a definitive diagnosis of RP inversa was made. DISCUSSION: RP inversa is a rare form of tapetoretinal degeneration that is characterized by decreased central vision with normal peripheral vision. A recessive form of inheritance has been postulated but never substantiated. Although there is currently no treatment, recent studies have indicated that 15,000 IU of vitamin A palmitate daily may slow the progression of retinitis pigmentosa; however, it is unknown whether this treatment would be effective for the inverse form of RP. Differential diagnoses include Leber's congenital amaurosis, central gyrate atrophy, central areolar choroidal sclerosis, progressive cone-rod dystrophy, syphilitic retinopathy, retinal toxicity from phenothiazine use, and chloroquine/hydroxychloroquine retinopathy.     

The platysma myocutaneous flap. Indications and caveats

Arterial hypertension has been identified as a major secondary risk factor for diabetic retinopathy. However, the mechanisms by which hypertension worsens retinopathy are unknown. Inhibition of advanced glycation product formation prevents the development of experimental diabetic retinopathy in normotensive diabetic rats. In this study the effect of hypertension on the rate of diabetic retinopathy development and the formation of arteriolar thrombosis was evaluated. We also evaluated the effect of aminoguanidine, an inhibitor of advanced glycation and product formation on retinal pathology of diabetic hypertensive rats. After 26 weeks of diabetes, hypertension accelerated the development of retinopathy despite a lower mean blood glucose level than in the non-hypertensive group (diabetic spontaneous hypertensive rats (SHR) 16.00 +/- 6.83 mmol/l; diabetic normotensive Wistar Kyoto rats (WKY) 34.9 +/- 3.64 mmol/l; p < 0.0001). Diabetic SHR had nearly twice as many acellular capillaries as diabetic WKY (SHR diabetic: 91.9 +/- 7.5 acellular capillaries per mm2 of retinal area vs WKY diabetic: 53.7 +/- 8.5 acellular capillaries per mm2 of retinal area), and a 3.8-fold increase in the number of arteriolar microthromboses (SHR diabetic 23,504 +/- 5523 microns2 vs SHR non-diabetic 6228 +/- 2707 microns2). Aminoguanidine treatment of SHR diabetic rats reduced the number of acellular capillaries by 50%, and completely prevented both arteriolar deposition of PAS-positive material and abnormal microthrombus formation. These data suggest that hypertension-induced deposition of glycated proteins in the retinal vasculature plays a central role in the acceleration of diabetic retinopathy by hypertension.