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Light coagulation in retinal vein thrombosis
369
CURRENTOPHTHALMOLOGY
Farnsworth lOO-Hue Test in Diagnosis of Ethambutol-Induced Damage to Optic Nerve, by Danuta Trusiewicz. Ophthalmologica, Base1 171:425-43 1, 1975 Owing to the high therapeutic value and good tolerance of ethambutol (EMB), this drug is widely applied in treatment of tuberculosis. Toxic changes in the optic nerve are the only severe complication of EMB therapy. Therefore, it is customary to examine during the course of treatment the visual acuity, visual field and color vision. Three cases are presented to illustrate the opinion of Dr. Trusiewicz who suggests that the three recommended examinations not only seem to be excessively time-consuming, but also may not always be the most suitable ones. The author stresses the importance of color vision tests. Case 2. A woman, aged 35, treated for 3 months with EMB (25 mg/kg), complained of deterioration of vision. In spite of these symptoms, examinations of the visual acuity, visual field and color vision (carried out in both eyes simultaneously, using the pseudoisochromatic tables, PIC), which were performed at the same time in another department, gave normal results. Three weeks after the patient had experienced vision disturbances, EMB was withdrawn because of a considerable drop in the visual acuity (VOD = 0.2, vos = 0.3). Case 2. A woman, aged 40, had received EMB in doses of 25 mg/kg over 3 months, and in doses of 15 mg/kg for the last 5 months. She periodically suffered from blurring of vision. In this patient, the color vision defect was the only deviation from normal in the ophthalmological examination. In tests with the Ishihara plates, she misread 5 tables with the right eye (OD) and 17 with the left eye (OS). These results testified to an acquired color vision defect. Examination using the Farnsworth lOO-Hue test confirmed this assumption: total error score was OD = 138, OS = 201. Case 3. A man, aged 62, was treated with EMB (25 mg/kg) for 18 months. At the time of hospitalization VOD = 0.04, VOS = 0.02. After 3 months the results of the Farnsworth lOO-Hue test were as follows: total error score, OD = 461, OS = 472. After 6 months VOD = 0.8, VOS = 0.5. Examination of the color vision pointed to improvement; however, the high total error score persisted (OD = 3 10, OS = 247). In this case the EMB-induced disturbance was superimposed on a congenital color vision defect (deuteranomalia). Dr. Trusiewicz emphasizes that whereas all elements of the customary examination may give normal results (Case l), the acquired color vision defect can be the earliest symptom of the toxic process induced in the optic nerve by EMB (Case 2). When using the PIC tables for the demonstration of the effect of EMB on the optic nerve, it is necessary to perform examinations singly in each eye. The difference in the number of the misread tables between the right and left eye is of diagnostic value in demonstrating the toxic action of EMB. The Farnsworth lOO-Hue test is a valuable diagnostic method which not only permits the detection of the EMB-induced toxic process, but also enables the observation of its dynamics. A decreasing total error score testifies to the recession of this process. (Abstract by D. Trusiewicz)
Comment There is no need to comment at any length on this important paper. The early observation of Carr and Henkind (1962) concerning the ocular damage induced by Ethambutol have been corroborated by several authors. Careful study of the eyes during treatment with substantial doses of the drug is imperative. I am especially pleased with the author’s recommendation to use the Farnsworth lOO-Hue test in preference to any other color vision test. I have come to rely on this magnificent test more and more. True, it takes considerable time to give it and to read it. But the time will be well used. I considered the Farnsworth loOHue test to be the foremost tool for detecting and following pathological changes of color vision. It is superior even to the anomaloscope. ARTHUR LINKSZ
Light Coagulation in Retinal Vein Thrombosis, by H Freyler and St. Nichorlis. Klin Monatsbl Augenheilkd 16.5: 750-755, 1974 The authors present the results of light coagulation treatment in 20 cases with so-called “retinal vein thrombosis.” The study included five cases of central retinal vein occlusion and 15 cases of branch vein occlusion. No cases of preocclusion of the central retinal vein were included. The age of the patients ranged from 24 to 85 years (63.2 average). Ten patients suffered from hypertension, 4 from diabetes mellitus and 4 from both diabetes mellitus and hypertension. The time interval between the tirst subjective symptoms and light coagulation ranged from 4 months to 5 years (average 13.9 months). In all 20 cases, a cystoid edema of the macula and a diffuse intraretinal edema of the drainage area of the affected veins was present. The visual acuity ranged from seeing hand movements to 0.7 (0.16 average). Light coagulation was performed using a Zeiss Xenon-arc-coagulator (field size 3”, intensity l/n green II, time
370
SW
Ophtholmol 21 (4) January-February
1977
CURRENTOPHTHALMOLOGY
of light exposure 1/n about 1 set). Choice of sites to be coagulated was based on fluorescein angiography findings: newly-formed vessels within the retina or on the retinal surface, dye leaking capillaries in the drainage area of the affected v&s and temporally of the macula. The followup period after photocoagulation w.as 9.4 months on the average. In all 20 cases, a regression of retinal hemorrhages was observed. Cystoid edema of the macula disappeared completely in 7 cases and partially in the other 13 cases. In 11 cases, an extramacular edema vanished and in 9 cases it regressed, obviously. In all 20 cases, dilation and tortuosity of the veins diminished. Retinal vasoproliferations disappeared completely in 2 cases and partially in 2 others, the extension of peripapillary neovascularization decreased in 2 cases. The average retinal circulation time diminished from 14.8 set before treatment to 12.1 set after photocoagulation. In 3 cases, visual acuity improved from seeing hand movements before photocoagulation to reading large numbers at a distance of l-3 meters (l/60, l/18,3/60 of the Snellen-screen) and in 17 cases from 0.16 on the average to 0.4. The first signs of improvement were noted 1 to 6 months after photocoagulation (4 months on an average). (Abstract by H. Freyler)
Comment The author has collected a most impressive series of cases which have uniformly responded favorably to photocoagulation treatment. However, it is important to stress that many patients with retinal vein occlusion, both central and branch vein, will spontaneously show improvement as part of the natural course of their condition. To interpret the results more critically, a prospective randomized clinical trial is the only method to document objectively the possible benefits of photocoagulation, or of any other form of therapy. This is especially important as photocoagulation therapy is not without some risk. Although this study lacks controls, the reported success in all 20 cases suggests that photocoagulation has some benefit in the therapy of retinal vascular occlusion. ARNALL PATZ
Significance of the Conjunctival Biopsy in the Diagnosis of Niemann-Pick Disease, by J. Libert and P. Danis. Bull Sot Belge Ophtalmol 168:757-764, 1974 Conjunctival biopsies have been performed in two patients affected with Type A Niemann-Pick disease. The first patient was examined 8 days before death at the age of 3V2years. The second patient was seen during his first admission at the age of 9 months. In both patients, the clinical picture, the macular cherry-red spots, the liver biopsy and the study of sphingomyelin elimination in urine determined the diagnosis of the disease. A very small and superficial fragment of conjunctiva was obtained under topical anesthesia. No suture was necessary. Postoperatively, phenylephrine and an ophthalmic antibiotic ointment were instilled. The tissues were prepared by the usual techniques for electron microscopy. The ultrastructural lesions were similar in the two cases. Stored material was present in the form of dense osmiophilic inclusions, limited by a single membrane, which contained more or less altered lamellar structures. The widespread storage process affected the epithelial cells, the Bbroblasts, the endothelial cells of the blood and lymphatic capillaries. The nerve fibers showed areas of destruction of the myelin sheaths and degeneration of the axons. The Schwann cells were packed with numerous lamellar inclusions and myelin residues. The inclusions found in the conjunctiva were closely similar to those observed in other tissues reported in the literature. This ultrastructural aspect corresponds to the storage of sphingomyelin and cholesterol, and is characteristic of Niemann-Pick disease. The typical alterations were significant at the age of 9 months when the diagnosis was first suspected in one younger patient. They were probably present earlier, even before the clinical signs appeared. This suggests that conjunctival biopsy may offer an easy and reliable way to diagnose type A Niemann-Pick disease. (Abstract by J. Libert)
Comment Niemann-Pick disease is not always easily diagnosed. This is especially true since the broad spectrum of the clinical manifestations of the disease has become recognized (Fredrickson DS, Sloan HR, in Stanbury JB et al (eds): The Metabolic Basis of Inherited Disease, NY, McGraw-Hill, 1972, pp 783-807). Two major forms exist, one in which the disease becomes manifest in childhood; the other with much milder symptomatology, affects adults. A macular cherry-red spot is visible “late” in the clinical course of the childhood variety, earlier signs being hepatosplenomegaly and mental retardation. This paper, and another one recently published by the same authors (Libert J, Danis P: Pathol Europ 10:233-239, 1975), suggest that conjunctival biopsy may be an early technique of definitive diagnosis permitting more accurate prognostication and counseling.
CURRENTOPHTHALMOLOGY
Farnsworth lOO-Hue Test in Diagnosis of Ethambutol-Induced Damage to Optic Nerve, by Danuta Trusiewicz. Ophthalmologica, Base1 171:425-43 1, 1975 Owing to the high therapeutic value and good tolerance of ethambutol (EMB), this drug is widely applied in treatment of tuberculosis. Toxic changes in the optic nerve are the only severe complication of EMB therapy. Therefore, it is customary to examine during the course of treatment the visual acuity, visual field and color vision. Three cases are presented to illustrate the opinion of Dr. Trusiewicz who suggests that the three recommended examinations not only seem to be excessively time-consuming, but also may not always be the most suitable ones. The author stresses the importance of color vision tests. Case 2. A woman, aged 35, treated for 3 months with EMB (25 mg/kg), complained of deterioration of vision. In spite of these symptoms, examinations of the visual acuity, visual field and color vision (carried out in both eyes simultaneously, using the pseudoisochromatic tables, PIC), which were performed at the same time in another department, gave normal results. Three weeks after the patient had experienced vision disturbances, EMB was withdrawn because of a considerable drop in the visual acuity (VOD = 0.2, vos = 0.3). Case 2. A woman, aged 40, had received EMB in doses of 25 mg/kg over 3 months, and in doses of 15 mg/kg for the last 5 months. She periodically suffered from blurring of vision. In this patient, the color vision defect was the only deviation from normal in the ophthalmological examination. In tests with the Ishihara plates, she misread 5 tables with the right eye (OD) and 17 with the left eye (OS). These results testified to an acquired color vision defect. Examination using the Farnsworth lOO-Hue test confirmed this assumption: total error score was OD = 138, OS = 201. Case 3. A man, aged 62, was treated with EMB (25 mg/kg) for 18 months. At the time of hospitalization VOD = 0.04, VOS = 0.02. After 3 months the results of the Farnsworth lOO-Hue test were as follows: total error score, OD = 461, OS = 472. After 6 months VOD = 0.8, VOS = 0.5. Examination of the color vision pointed to improvement; however, the high total error score persisted (OD = 3 10, OS = 247). In this case the EMB-induced disturbance was superimposed on a congenital color vision defect (deuteranomalia). Dr. Trusiewicz emphasizes that whereas all elements of the customary examination may give normal results (Case l), the acquired color vision defect can be the earliest symptom of the toxic process induced in the optic nerve by EMB (Case 2). When using the PIC tables for the demonstration of the effect of EMB on the optic nerve, it is necessary to perform examinations singly in each eye. The difference in the number of the misread tables between the right and left eye is of diagnostic value in demonstrating the toxic action of EMB. The Farnsworth lOO-Hue test is a valuable diagnostic method which not only permits the detection of the EMB-induced toxic process, but also enables the observation of its dynamics. A decreasing total error score testifies to the recession of this process. (Abstract by D. Trusiewicz)
Comment There is no need to comment at any length on this important paper. The early observation of Carr and Henkind (1962) concerning the ocular damage induced by Ethambutol have been corroborated by several authors. Careful study of the eyes during treatment with substantial doses of the drug is imperative. I am especially pleased with the author’s recommendation to use the Farnsworth lOO-Hue test in preference to any other color vision test. I have come to rely on this magnificent test more and more. True, it takes considerable time to give it and to read it. But the time will be well used. I considered the Farnsworth loOHue test to be the foremost tool for detecting and following pathological changes of color vision. It is superior even to the anomaloscope. ARTHUR LINKSZ
Light Coagulation in Retinal Vein Thrombosis, by H Freyler and St. Nichorlis. Klin Monatsbl Augenheilkd 16.5: 750-755, 1974 The authors present the results of light coagulation treatment in 20 cases with so-called “retinal vein thrombosis.” The study included five cases of central retinal vein occlusion and 15 cases of branch vein occlusion. No cases of preocclusion of the central retinal vein were included. The age of the patients ranged from 24 to 85 years (63.2 average). Ten patients suffered from hypertension, 4 from diabetes mellitus and 4 from both diabetes mellitus and hypertension. The time interval between the tirst subjective symptoms and light coagulation ranged from 4 months to 5 years (average 13.9 months). In all 20 cases, a cystoid edema of the macula and a diffuse intraretinal edema of the drainage area of the affected veins was present. The visual acuity ranged from seeing hand movements to 0.7 (0.16 average). Light coagulation was performed using a Zeiss Xenon-arc-coagulator (field size 3”, intensity l/n green II, time
370
SW
Ophtholmol 21 (4) January-February
1977
CURRENTOPHTHALMOLOGY
of light exposure 1/n about 1 set). Choice of sites to be coagulated was based on fluorescein angiography findings: newly-formed vessels within the retina or on the retinal surface, dye leaking capillaries in the drainage area of the affected v&s and temporally of the macula. The followup period after photocoagulation w.as 9.4 months on the average. In all 20 cases, a regression of retinal hemorrhages was observed. Cystoid edema of the macula disappeared completely in 7 cases and partially in the other 13 cases. In 11 cases, an extramacular edema vanished and in 9 cases it regressed, obviously. In all 20 cases, dilation and tortuosity of the veins diminished. Retinal vasoproliferations disappeared completely in 2 cases and partially in 2 others, the extension of peripapillary neovascularization decreased in 2 cases. The average retinal circulation time diminished from 14.8 set before treatment to 12.1 set after photocoagulation. In 3 cases, visual acuity improved from seeing hand movements before photocoagulation to reading large numbers at a distance of l-3 meters (l/60, l/18,3/60 of the Snellen-screen) and in 17 cases from 0.16 on the average to 0.4. The first signs of improvement were noted 1 to 6 months after photocoagulation (4 months on an average). (Abstract by H. Freyler)
Comment The author has collected a most impressive series of cases which have uniformly responded favorably to photocoagulation treatment. However, it is important to stress that many patients with retinal vein occlusion, both central and branch vein, will spontaneously show improvement as part of the natural course of their condition. To interpret the results more critically, a prospective randomized clinical trial is the only method to document objectively the possible benefits of photocoagulation, or of any other form of therapy. This is especially important as photocoagulation therapy is not without some risk. Although this study lacks controls, the reported success in all 20 cases suggests that photocoagulation has some benefit in the therapy of retinal vascular occlusion. ARNALL PATZ
Significance of the Conjunctival Biopsy in the Diagnosis of Niemann-Pick Disease, by J. Libert and P. Danis. Bull Sot Belge Ophtalmol 168:757-764, 1974 Conjunctival biopsies have been performed in two patients affected with Type A Niemann-Pick disease. The first patient was examined 8 days before death at the age of 3V2years. The second patient was seen during his first admission at the age of 9 months. In both patients, the clinical picture, the macular cherry-red spots, the liver biopsy and the study of sphingomyelin elimination in urine determined the diagnosis of the disease. A very small and superficial fragment of conjunctiva was obtained under topical anesthesia. No suture was necessary. Postoperatively, phenylephrine and an ophthalmic antibiotic ointment were instilled. The tissues were prepared by the usual techniques for electron microscopy. The ultrastructural lesions were similar in the two cases. Stored material was present in the form of dense osmiophilic inclusions, limited by a single membrane, which contained more or less altered lamellar structures. The widespread storage process affected the epithelial cells, the Bbroblasts, the endothelial cells of the blood and lymphatic capillaries. The nerve fibers showed areas of destruction of the myelin sheaths and degeneration of the axons. The Schwann cells were packed with numerous lamellar inclusions and myelin residues. The inclusions found in the conjunctiva were closely similar to those observed in other tissues reported in the literature. This ultrastructural aspect corresponds to the storage of sphingomyelin and cholesterol, and is characteristic of Niemann-Pick disease. The typical alterations were significant at the age of 9 months when the diagnosis was first suspected in one younger patient. They were probably present earlier, even before the clinical signs appeared. This suggests that conjunctival biopsy may offer an easy and reliable way to diagnose type A Niemann-Pick disease. (Abstract by J. Libert)
Comment Niemann-Pick disease is not always easily diagnosed. This is especially true since the broad spectrum of the clinical manifestations of the disease has become recognized (Fredrickson DS, Sloan HR, in Stanbury JB et al (eds): The Metabolic Basis of Inherited Disease, NY, McGraw-Hill, 1972, pp 783-807). Two major forms exist, one in which the disease becomes manifest in childhood; the other with much milder symptomatology, affects adults. A macular cherry-red spot is visible “late” in the clinical course of the childhood variety, earlier signs being hepatosplenomegaly and mental retardation. This paper, and another one recently published by the same authors (Libert J, Danis P: Pathol Europ 10:233-239, 1975), suggest that conjunctival biopsy may be an early technique of definitive diagnosis permitting more accurate prognostication and counseling.