Prospective controlled evaluation on radiotherapy of subfoveolar neovascularization

Summary Despite the successful therapy of subretinal neovascular membranes by laserphotocoagulation there are many problems to be overcome. In the case of subfoveolar neovascularization, photocoagulation leads to a sudden decrease in visual acuity.Recently radiotherapy is considered as an alternative. Complications and effectivity were evaluated in this prospective and randomized trial. The initial results are presented. Patients and methods: There are 76 patients (51 women, 25 men, average age 77.7 ± 8.6 years) included in the prospective randomized study. All of them show subfoveolar neovascular membranes in FLA and a decrease in visual acuity between 0.05 and 0.5. They were randomly assigned to either the radiotherapy or the control group. Radiotherapy was done within 6 days by 6 × 2 Gy (6 MV photons). The follow-up was at 4 weeks, after 3 months, after 6 months and then every 6 months after the end of radiotherapy. On average the follow-up is at 15.1 months. Results: Concerning age and visual acuity before therapy, the control group and the radiotherapy group were not significantly different. At 4 weeks after radiotherapy, visual acuity was 0.13 ± 0.46 (LogMAR). After 12 months, visual acuity at a distance was 0.11 ± 0.30 in the therapy group and 0.09 ± 0.13 (P = 0.838) in the control group. Patients with a preoperative visual acuity better than 0.2 improved more after radiotherapy. Metamorphopsy improved in 75 % of the therapy group. The following complications could be observed: In the control group 3 patients suffered subretinal bleeding, in the radiotherapy group 3 patients, respectively. Conclusions: At present, the follow-up is too short to recommend radiotherapy as a standard procedure in the case of subfoveolar neovascularization. The results in patients with a better preoperative visual acuity encourage us to continue this study.      

Repeat laser coagulation of subretinal neovascularization in age-related macular degeneration

Introduction: Retinal laser coagulation (LC) of extrafoveal subretinal neovascularization (SRN) due to age-related macular degeneration (AMD) is the only sure therapy. Diagnostics, therapy and follow-up are presented in a case report. Case report: A 55-year-old woman had suffered from progressive reduction of visual acuity (with metamorphopsia) in both eyes (LE > RE) for 3 years. In the RE the visual acuity was 0.32 and in the LE 0.05. Funduscopy and fluorescein angiography revealed in both eyes the wet form of AMD.The extrafovealy located SRN of the RE was photocoagulated and had to be retreated three-times in 6 weeks due to persistence. For 8 months the findings in the RE have been stable. VA has been improved to 0.4 and no metamorphopsia has been found. Conclusion: Extrafoveal SRN should be treated by LC. Short-term follow-up (including FA) and in most cases several LCs are necessary to close the SRN and save the patients' reading vision.      

Combined immunosuppressive and surgical therapy of necrotic keratitis in rheumatoid arthritis

Introduction: Areactive forms of keratitis in patients with seropositive rheumatoid arthritis are inflammations threatening the visual acuity and integrity of the eye. They commonly occur in a rheumatologically inactive interval and have a poor prognosis. A retrospective evaluation of medicamentous and surgical strategies for a curative therapy with optical rehabilitation is necessary to optimise the treatment of patients with necrotic sclerokeratitis. Patients and methods: A total of 27 eyes of 22 patients (14 women and 8 men, ranging in age at the time of operation from 40 to 88 years; mean 68.7 years) with seropositive rheumatoid arthritis and secondary Sj?gren's syndrome were reviewed retrospectively. There were 17 eyes with necrotic keratitis and 9 eyes with necrotic sclerokeratitis. In one eye, necrotic sclerokeratitis with bacterial transmigrating keratitis and hypopyon occurred. Operations: In 8 cases we performed a perforating mini-keratoplasty, in 16 cases a tectonic and optical perforating keratoplasty, in 3 cases a tectonic sclerokeratoplasty, in 9 patients a combined keratoplasty and cataract extraction with posterior chamber lens implantation and in 1 case a partial conjunctival plasty. Follow-up ranged from 7 months to 4 years (average 2.8 years). Results: In all eyes, a sufficient tectonic and primary curative effect was achieved only under cyclophosphamide immunosuppression. In 3 cases, a rekeratoplasty had to be performed because of recurrent keratitis after changing the systemic cyclophosphamide therapy to methotrexate, glucocorticosteroids or non-steroid antiphlogistic agents. Visual acuity outcome was depending on the eccentricity of the keratoplasty and earlier affections of the eye. Postoperatively, the visual acuity improved in 23 eyes. In 3 cases, no change of visual acuity was achieved. Visual acuity deteriorated in one case from counting fingers to hand motions. Peri- and postoperative complications during the follow-up period were corneal infiltration around sutures in 4 eyes, graft rejecting reactions in 3 cases, and sicca syndrome in 6 cases. Conclusions: The intensive cooperation of ophthalmologists and rheumatologists enables the successful treatment of apparently hopeless situations in necrotic sclerokeratitis in patients with seropositive rheumatoid arthritis. The rate of complications under an immunosuppressive therapy with cyclophosphamide was found at average 2.8 years follow-up to be low. The indication for the combined therapy depends on the ophthalmological findings; rheumatologists and ophthalmologists should decide on the appropriate dosage for the systemic cyclophosphamide therapy. Topical glucocorticosteroid therapy alone is contra-indicated.      

Effect of exposure time using single Landolt-rings on visual acuity in normal individuals and in patients with nystagmus

Background: The recommendations of the German Society of Ophthalmology (DOG) concerning the examination of candidates or holders of a driving licence allow a maximum time interval of 1 s for identifying a single Landolt-C for normal individuals as well as for patients with nystagmus. The aim of this study was to determine whether visual acuity decreases with shortening of the exposure time. Patients and methods: We measured the visual acuity of 18 patients with manifest nystagmus and 26 normal individuals with single Landolt-C at exposure times of 10 s, 1 s, 0.25 s and 0.1 s. Results: Visual acuity dropped significantly at exposure times of 1 s in the group of patients with nystagmus (P = 0,01). In normal individuals, visual acuity decreased significantly only at exposure times below 1 s. Conclusion: Determining visual acuity according to the recommendations of the DOG shows a significant drop of visual acuity in nystagmus patients, but not in normal individuals. Applying the maximum time limit of 1 s exposure time to these patients will ensure that they will be able to identify traffic signs quickly and orient themselves safely.      

Surgery of Marfan-associated and idiopathic lens subluxations

Background: The ideal and safe surgical method for Marfan-associated or idiopathic lens subluxations is still a matter of debate. Patients and methods: Between 1990 and 1995, 23 eyes were operated for lens subluxations, mainly because of decreased visual acuity, but also because of conservatively uncontrolled secondary glaucoma. Marfan patients were 27.0 (5–62) years old at surgery; patients with idiopathic lens subluxations were 38.5 (11–63) years old. Surgical procedure depended on patient age and anatomical conditions. Results: All patients achieved an increase in visual acuity. Amblyopia existed in six patients. All problems due to glaucoma were controlled postoperatively. Our greatest concern was rhegmatogenous retinal detachment. It occurred in only one eye of a non-Marfan patient. Conclusion: The prognosis for lens surgery in Marfan-associated and idiopathic lens subluxations is good. The implantation of a posterior chamber lens provides a good and secure means of optical rehabilitation. Our preferred primary transscleral suture technique guarantees high security and stability of position. Previously feared surgical risks have been reduced by modern surgical procedures.      

A prospective comparison of two multifocal lens models

Summary Background: The functional results of two different types of multifocal intraocular lenses (based on the diffractive and refractive principle, respectively) were investigated prospectively. Materials and methods: Altogether 50 patients who had a multifocal lens implanted were examined 4–6 weeks postoperatively. Visual acuity for distance and near vision, contrast sensivity, low contrast visual acuity and glare visual acuity were investigated. Results: Distance visual acuity, contrast sensitivity, low contrast visual acuity and glare visual acuity did not show significantly different results. Near visual acuity was statistically significantly better with the diffractive type of multifocal lens (because of a stronger adjustment for near vision). When the focus depth was tested by defocus curves, both lenses had better results within different areas of defocus. Conclusions: All patients in both groups showed satisfactory results. When choosing a multifocal intraocular lens, the individual needs of each patient should be taken into consideration.      

Central Serous Chorioretinopathy: Clinical Characteristics Associated with Visual Outcomes

Purpose: Retrospective cohort study to identify clinical characteristics associated with poor visual acuity in central serous chorioretinopathy (CSC). Materials and Methods: Charts of patients in a tertiary referral clinic diagnosed with CSC over a 13-year period were reviewed. Multivariate logistic regression analyses were performed to assess the relationship between several clinical characteristics and final visual acuity. Results: Of 353 subjects with CSC, 258 had a minimum of 2 clinical assessments and adequate follow-up. Multivariate analysis showed that the followings were significantly associated with worse final visual acuity: older age at diagnosis, history of photodynamic therapy, choroidal neovascularization (CNV), hypertension, and either prostate cancer or benign prostatic hypertrophy. Diabetes mellitus was associated with better final visual acuity. In a subgroup analysis of 150 subjects with at least 1 year of follow-up, CNV, hypertension, and gastroesophageal reflux disease were significantly associated with worsening of visual acuity over the study period. Use of a psychiatric medication at presentation was protective. Conclusion: Poor visual outcomes in CSC are associated with older age at diagnosis, CNV, hypertension, and history of prostate disease. Several clinical characteristics that have been identified as risk factors for developing CSC also appear to be associated with worse visual outcomes.     

Pseudophakia in children with juvenile arthritis

Background: Cataract secondary to juvenile rheumatoid arthritis is a severe, vision-threatening complication in early childhood. Intraocular lens implantation is controversial. The follow-up of four pseudophakic eyes of three patients and their perioperative therapeutic regimen were retrospectively analyzed. Early and late postoperative complications are reported. Patients and methods: Both girls had lens aspiration and posterior lens implantation at the ages of 6 and 12 years, the boy at the age of 10 and 14 years. All patients had relapsing anterior uveitis. The follow-up time was 3 years (1–6 years). One girl was diagnosed with sarcoidosis, causing juvenile arthritis. Both girls had perioperative methothrexate and prednisolone therapy. The boy had azathioprine therapy at the time of his first cataract surgery; later he had no systemic therapy. Both girls' intraocular lenses were implanted at different eye hospitals. Results: Both girls had severe inflammatory reactions after surgery. At the hospitals both eyes had surgical revision for iris capture. In one case this was combined with exchanging the intraocular lens. Iris capture persisted for this eye and later vitrectomy with silicone oil filling was necessary to delay phthisis, resulting in amaurosis. For two pseudophakic eyes vitrectomy was necessary later because of severe vitreous opacities, but visual acuity was severely diminished by chronic cystoid macular edema and epiretinal membranes. The boy developed in his second eye intermittent iris bombata and persistent secondary glaucoma, visual acuity was stabilized at 0.5. Conclusions: Secondary cataract due to juvenile rheumatoid arthritis or sarcoidosis is a difficult situation for phacoemulsification with intraocular lens implantation in children. For severe inflammatory complications intense local and systemic anti-inflammatory therapy is mandatory. Visual prognosis is reduced for the uveitic posterior segment and glaucoma complications. IOL implantation can be recommended for only a very few patients.      

Ability to drive after surgery for retinal detachment

Background: After vitreoretinal surgery the patient is at least temporarily unable to drive. Buckling procedures may cause refractive changes, reduced motility or impaired binocular vision. We examined the ability of these patients to drive a car according to German law after retinal surgery. Patients and methods: The frequency and extent of changes interfering with driving ability were examined in 112 unselected patients 3.5 years (2–5 years) after successful buckling procedures. We used half-round buckling elements of 3–11 mm diameter or encircling bands (2–3 mm). We examined visual acuity, frequency of permanent diplopia and field of normal binocular vision. Driving ability was considered as impaired, when visual acuity was below 0.4 in the better eye or 0.2 in the worse eye, or when diplopia occurred within a gazing field of 20 ° of diameter. Results: Eighteen of 112 (16 %) patients reported diplopia within the first 3 months, so they were temporarily unable to drive. Two to five years later 14 of 112 patients did not have sufficient visual acuity according to the German requirements. Another 2 patients had an impaired binocular vision, resulting in driving disability. Conclusion: Driving ability may be temporarily restricted by double vision in 15 % of patients after successful buckling for retinal detachment. Permanent driving ability is mainly impaired by macular involvement in retinal detachment. The type of buckling procedure is of minor importance.      

One-year results after radiotherapy of choroidal neovascularization in age-related macular degeneration

Summary Choroidal neovascularization (CNV) associated with age-related macular degeneration is the major cause of legal blindness in Europe and the USA in patients aged more than 65 years, but Chakravarthy et al. has reported that radiotherapy has a beneficial effect on visual acuity. Methods: Since March 1996 we have treated 56 patients in cooperation with the Department of Radiotherapy at the Technical University in Munich. The total dose with external beam radiotherapy was 16 Gy in 8 fractions, delivered through an anterior oblique axis to spare the lens. Before the treatment and 3, 6 and 12 months after therapy, we performed a standardized visual acuity and contrast-sensitivity test (ETDRS, Pelli Robson Chard) and fluorescin angiography 6 and 12 months after therapy. Results: Twenty-five angiograms showed well-defined CNV and 31 not well-defined CNV. Six months after the treatment 15 patients had stable visual acuity within one line. Twenty-seven patients had lost more than one line of visual acuity. There was no difference between well and not well defined CNV's. One year after treatment the visual acuity remained stable within one line in 4 patients, no patient had an increase of two lines or more and 17 patients lost more then 2 lines of vision. We saw no side effects other than sicca symptoms in 3 patients. Conclusion: In our opinion, these results do not show that radiation treatment has a real beneficial effect on visual acuity. Further randomized studies are needed to demonstrate the efficiency of this treatment for choroidal neovascularization in AMD.      

Focal proliferation of retinal pigment epithelium as risk factor in age-related macular degeneration

Background: Clinical studies have demonstrated the relevance of focal RPE proliferations in early AMD as risk factors for visual loss caused by late AMD. Angiographically these focal RPE proliferations are characterized as small hypofluorescent spots with hyperfluorescent rim without leakage. Corresponding to histological and experimental studies they can be interpreted as small areas of occult choroidal neovascularizations covered by proliferated RPE cells. The characterization of the long-term prognosis of these lesions was the aim of the present study. Patients and methods: Ninety-eight patients (52 female, 46 male) were reexamined clinically and angiographically with a follow-up of 2–12 years (mean 6.5 years). Results: Visual loss of two lines or more could be observed in 64.5 % of patients with final visual acuity less than 20/100 in 24.5 % of patients. Morphologically the changes in visual acuity were related to the progression towards classical choroidal neovascularizations in 32.7 % of patients. In addition 11.2 % of patients demonstrated a regression of the small occult membrane with the development of small areas of RPE atrophy covering the size of the original occult neovascularization. In 10.2 % of the patients enlargement of the lesion was observed, resulting in a large occult choroidal neovascularization without signs of classical membranes, and in 45.9 % of patients the clinical and angiographical situation was unchanged. The most important prognostic factor correlating with visual loss was the presence of a disciform lesion in the fellow eye and of multiple drusen in the examined eye. Other factors like the size or location of the focal RPE proliferation and the duration of follow-up did not correspond with visual loss. Conclusions: Focal RPE proliferations in early AMD interpreted as small occult choroidal neovascularizations are associated with a high risk of visual loss. Especially if these lesions are associated with multiple drusen and a disciform lesion in the fellow eye, nearly all patients are at risk for visual loss. These changes may therefore characterize a special high-risk group for future prophylactic treatments in early AMD, but because of the high risk for the development of classical choroidal neovascularizations in this group, these results are also very important for the planning of prophylactic laser trials for drusen in early AMD.      

Management of submacular hemorrhage by tissue plasminogen activator and SF6 gas injection

Summary The formation of platelet-fibrin clots causes degeneration of foveolar photoreceptors by subretinal hemorrhage. Patients: Twenty-two patients with submacular hemorrhages were treated by intravitreal injection of 50 μg tpa and 0.5 ml SF₆ gas within 1 h. Biomicroscopy, fundus photography and fluorescein angiography were performed pre- and postoperatively. Further multifocal electroretinograms were recorded. Results: The subfoveolar hemorrhages were dissoluted and displaced out of the macular region. The visual acuity increased in 18 cases and ranged from hand movement and 0.3 to values between 1/10 and 0.5. Conclusions: Toxic and hypoxic damage of foveolar photoreceptors by subretinal hemorrhage can be prevented by early and minimal invasive fibrinolytic therapy. After localization of choroidal neovascularizations, treatment by focal laser coagulation is possible in some cases.      

Neurophysiological examinations in patients with pituitary surgery

Background: Pituitary tumors are adenomas of a region of the sella turcica which can produce compression of the anterior visual pathway. Patients and methods: Besides clinical signs of visual improvement such as enhanced visual acuity and visual field, the use of electroophthalmological methods can help monitor patients after neurosurgery. Results: The N₇₅ P₁₀₀ amplitude of the p-VEP has proven to be the most sensitive marker for measuring-improvements after surgery. This amplitude significantly increased, whereas latency time, visual acuity and visual field showed no statistically significant changes after surgery. Conclusions: The p-VEP is an important tool in treating patients with compression of the visual pathway.      

A prospective study investigating the long-term stability of changes in astigmatism following arcuate lamellar keratotomy (ALK): 3-year results

Introduction: The correction of low to moderate astigmatism is possible today by means of photoablation (PRK), while the treatment of moderate and higher astigmatism still involves refractive keratotomy. Experience has shown that cataract surgery, using modern tunnel techniques with self-healing incisions, results in earlier stability in both the refractive outcome and wound healing. In this study, we attempted to combine the advantages of lamellar keratotomy with those of a pair-wise T-incision as arcuate lamellar keratotomy (ALK). Patients and methods: The clinical outcome of 41 patients who underwent ALK was investigate in a prospective study over a period of 3 years. The pre- and postoperative investigations undertaken included the measurement of astigmatism using a Zeiss keratometer, uncorrected visual acuity, and corrected glare vision using a Humphrey autorefractor. All patients had astigmatism between 2.0 and 7.0 D. Together with a uniform arcuate incision, we used 7 mm (n = 26) and 8 mm (n = 15) mm zones for correction. Results: The average preoperative astigmatism was 4.01 ± 1.90 (median, 3.50) D. After a 3-year follow-up the average postoperative astigmatism was 1.59 ± 1.29 (median, 1.38) D. The astigmatic change induced (Jaffé) after 3 years was about 3.23 ± 2.23 (median, 3.16) D. The average uncorrected visual acuity (log MAR) before ALK was 0.20 ± 0.12 (median, 0.22) and after follow-up, 0.41 ± 0.14 (median, 0.39). Corrected glare vision before surgery was 0.23 ± 0.19 (median, 0.10) and afterwards, 0.25 ± 0.22 (median, 0.14). Conclusions: Arcuate lamellar keratomy (ALK) stood the test as a routine clinical procedure for correction of moderate astigmatism with stable postoperative functional outcomes. We did not find impairment of glare vision following this procedure.      

North Carolina macular dystrophy: a hereditary macular dystrophy with good visual prognosis

Background: North Carolina macular dystrophy (NCMD) is a rare autosomal dominant maculopathy with highly variable expressivity. Genetic analysis of an American family consisting of 247 members out of which 96 were affected with NCMD allowed chromosomal assignment of the NCMD locus to 6q14-q16.2. Few families with NCMD are known in Europe, one of these is living in Germany. By routine investigation, a second family affected with NCMD was detected in Germany. As some authors still doubt the good prognosis of this disease, our results should be added to the experience of others. Patients and methods: In a total of 18 family members from three generations between the age of 2 and 65 years, clinical investigations and genetic analysis was carried out. Some individuals had additional examinations such as colour contrast sensitivity, EOG, ERG, and microperimetry. Results: Ten of 18 family members turned out to be affected. All grades of NCMD were present with great variability. Visual acuity ranged from 0.32 to 1.0 and did not correlate to the grade of the disease or to the age of the person. In those patients who underwent microperimetry, central fixation was confirmed. Genetic linkage analysis further narrowed the region harbouring the NCMD locus and supported the assumption that the central areolar pigment epithelial dystrophy (CAPED) is an allelic disorder. Conclusion: Similar visual acuity in three generations of NCMD patients supports the observation that NCMD is not a progressive disorder. If geographic atrophy is found in a patient with good visual acuity, NCMD should be considered and genetic analysis should be carried out.      

Electrophysiologic evaluation according to ISCEV standards in infants less than 10 years of age

Purpose: To analyze the value of electrophysiologic testing according to standards of the International Society for Clinical Electrophysiology of Vision (ISCEV) in infants less than 10 years of age. Patients and methods: In 64 infants less than 10 years, the results from of age electroretinograms (n = 47), visual evoked potentials (n = 30), or electro-oculograms (n = 1) were recorded. Twenty-nine infants were less than 6 years of age, and 17 infants were less than 3 years of age. Indications for examination were unexplained visual loss in 54 infants and familial hereditary retinal dystrophies in ten infants. Sedation with chloral hydrate was performed in seven children. Results: In 40/64 infants (62 %), the results of electrophysiologic examination were pathologic. In 29 infants visual loss was due to retinochoroidal dystrophies, and in 11 infants it was due to disturbances in the optic pathway. In 7/64, infants the suspected retinochoroidal dystrophy could be excluded. Therefore electrophysiologic testing was diagnostically accurate in 47/64 (73 %) of cases. Conclusion: Electrophysiologic testing according to ISCEV standards can be reliably performed in infants less than 10 years of age. When the indication for electrophysiologic testing is made following meticulous ophthalmologic examination, diagnostic accuracy is given in at least 73 % of cases.      

Therapy-resistant unilateral chronic blepharokeratoconjunctivitis as the main finding in autoaggression syndrome. Clinicohistopathological findings

Summary Autoaggressive syndromes as causes of diseases underlying chronic blepharitis and keratoconjunctivitis that are refractory to treatment are often difficult to recognize. Patients: Three female patients (age 21, 25, 41 years) and one male patient (age 42 years) had suffered from a right-(4 × ) or left-(1 × ) handed treatment-refractory blepharokeratoconjunctivitis for 1, 2 ,11 and 30 months prior to admission. In each case more than 5 ophthalmologists and 2–6 eye hospitals had been consulted, and extraocular surgery had been performed 1–4 times. Results: Patients presented with a visual acuity of 0.3 p (1 × ), 0.1 (1 × ), FC (1 × ), HM (1 × ). In three patients contact eczema of the facial skin and lids and a corneal pannus were observed; in two patients we saw purulent pseudomembranous and in two patients chronic cicatrizing keratoconjunctivitis. Conjunctival smears grew P. aeruginosa, and S. aureus; impression cytology showed infiltration with neutrophils and epithelial keratinization; histopathology indicated chronic inflammatory, partly purulent subepithelial and stromal conjunctival infiltrate with hyper- and parakeratosis fibrous strands and epithelial cell loss; the lower lids showed parakeratosis, focal necrosis, intercellular edema and a lymphohistiocytic round-cell infiltrate. Furthermore, multiple allergies to antibiotics and preservatives (4 × ), lacerations of the arms and legs (2 × ) and an irritative-toxic dermatitis (1 × ) were substantiated. In the patients who agreed to a psychiatric consultation, somatized-agitated longing for care combined with a dependent and infantile personality (1 × ) and reactive depression (2 × ) were verified. Conclusions: In patients suffering from treatment-refractory unilateral chronic blepharokeratoconjunctivitis correlated with the hand, one must take into consideration the fact that other factors may be involved: possible exacerbation prior to examinations; multiple inpatient diagnostic and surgical procedures in different locations; histopathological mixed inflammatory patterns; and psychiatric syndromes.      

Multifocal electroretinography (MF-ERG) in age-related macular degeneration

Purpose: Small areas of retinal pathology may pose diagnostic difficulties. The noninvasive multifocal electroretinogram (MF-ERG) provides a topographical mapping of retinal function. Its role in the diagnosis of macular diseases is examined in age-related macular degeneration (AMD). AMD is a main cause of central visual loss in the elderly population, affecting the second eye in 75 %. Methods: MF-ERG recordings of three patients with AMD were compared to the findings of fundus photography and fluorescein angiography. During the MF-ERG recordings the central 50 ° of the retina was stimulated. The visual stimulus consisted of 241 hexagons that alternated, independently and pseudorandomly, between black and white according to a special predetermined binary sequence. Local retinal response components were extracted using the Fast m-Transform Algorithm. Results: Three of six eyes had undergone cataract surgery with implantation of a posterior chamber lens (PCL). In accordance with an increase in light transmission through PCLs, these eyes showed an increase in the MF-ERG responses. MF-ERG allowed accurate topographic mapping of focal areas of retinal dysfunction in all patients tested. There was good correspondence to anatomical changes detected by fluorescein angiography. Conclusion: The high resolution of the MF-ERG enables detection of small areas of retinal pathology. It thus presents a clinically useful, noninvasive method in the early diagnosis and follow-up of macular disease.      

Oligocone trichromasy – a rare form of incomplete monochromatism

Summary Oligocone trichromasy is a rare form of congenital incomplete monochromatism. Patients and methods: An 11-year-old girl presented because of reduced visual acuity while color vision was almost normal. Besides a general ophthalmological examination, special psychophysical tests, such as perimetry, color vision tests using pseudoisochromatic plates, arrangement tests, the Nagel anomaloscope and spectral sensitivity measurement, and electrophysiological tests (electroretinogram and electrooculogram) were conducted. Results: The tests yielded the following: congenital nystagm, normal results at ophthalmoscopy, best visual acuity of 0.1 monocular and 0.2 binocular. Perimetry revealed a relatively central scotoma. All color vision tests showed only mild dysfunction of the blue-sensitive cones. Findings at photopic electroretinogram were almost completely lacking. There was no sign of progression in the last 6 years. Conclusion: Differential diagnosis includes all diseases associated with congenital nystagm, such as aniridia, diseases of the optic nerve, albinism and all forms of hereditary cone dysfunction, cone dystrophies and complete and incomplete congenital stationary monochromatism. In the present case the findings are most congruent with oligocone trichromasy.