306
September, 1990
AMERICAN JOURNAL OF OPHTHALMOLOGY
Full-field electroretinography was performed postoperatively. Cone, rod, 30-Hz flicker, and single bright flash electroretinograms were no longer recordable in either eye. We again compared the electroretinograms obtained for the right and left eyes in 1970 (Fig. 1), taking into account the 1.2-log unit neutral density filter created by trauma in the right eye. The electroretinograms recorded with comparable stimulus intensities in the traumatized eye never exceeded the response in the untraumatized eye. Light deprivation, even for more than 40 years, did not make the progression of retinitis pigmentosa slower in our patient. Fig. 2 (Miyake and associates). Postoperative findings of the anterior segment in the right eye. but only when the stimulus was intense. Retinitis pigmentosa was diagnosed. There was no family history of the disease. The retinitis pigmentosa gradually progressed and in 1982, visual acuity was R.E.: light perception and L.E.: 20/30. An annular maculopathy was observed in the left fundus. We opened the closed pupil of the right eye by using a vitrectomy instrument to remove the iris of the pupillary zone and the thick membrane behind the iris through the pars plana. The postoperative finding is shown in Figure 2. Before and after the operation, we measured the subjective light threshold of the right eye by changing intensities of full-field stimuli. The postoperative light threshold was 1.2 log units below the preoperative threshold. This result indicates that a filter density of 1.2 log units was removed by surgery. The right eye had been covered with a 1.2-log unit density filter for 42 years. The right and the left fundi were compared in terms of distribution of bone spicule pigmentation, degree of retinal vessel attenuation, color of the optic disk, and macular status. Little difference in fundus appearance was detected. There was no sign that the traumatic damage in the right eye involved the posterior segment. The preoperative visual acuity of light perception increased to hand motions postoperatively, but remained far lower than the visual acuity in the left eye (20/30). The lower visual acuity was most likely caused by stimulus deprivation amblyopia, since the right eye had been deprived of stimulus for 42 years, and the ophthalmoscopic macular findings were essentially the same in both eyes.
References 1. Dowling, J. E., and Sidman, R. L.: Inherited retinal dystrophy in the rat. J. Cell. BioI. 14:73, 1962. 2. La Vail, M. M., and Battelle, B. A.: Influence of eye pigmentation and light deprivation on inherited retinal dystrophy in the rat. Exp. Eye Res. 21:167, 1975. 3. Noell, W. K., and Albrecht, R.: Irreversible effects of visible light on the retina. Role of vitamin A. Science 172:76, 1971. 4. Berson, E. L.: Light deprivation and retinitis pigmentosa. Vision Res. 20:1179, 1980. 5. - - : Light deprivation for early retinitis pigmentosa. A hypothesis. Arch. Ophthalmol. 85:521, 1971.
Familial Anterior Ischemic Optic Neuropathy and Papillophlebitis David Deutsch, M.D., Eva Eting, M.D., Rahamim Avisar, M.D., Tirza Klein, Ph.D., Jacob Teller, M.D., and Hanna Savir, M.D. Department of Ophthalmology, Golda Medical Center, Hasharon Hospital and Tel-Aviv Sackler School of Medicine (D.D., E.E., R.A., J.T., and H.S.); and the Tissue Typing Laboratory, Beilinson Medical Center (T.K.).
Inquiries to David Deutsch, M.D., Department of Ophthalmology, Golda Medical Center, Hasharon Hospital, 7 Keren Kayemet St., Peiach-Tikua, Israel.
Anterior ischemic optic neuropathy is a common cause of significant visual loss in adults.' Papillophlebitis is an uncommon cause of mild
Vol. 110, No.3
Letters to the Journal
visual impairment in young adults." We studied bilateral anterior ischemic optic neuropathy in twin sisters and unilateral papillophlebitis in a third sister. A 41-year-old woman was examined because of pain and mild visual impairment in her right eye for one month. Visual acuity was 20/20 in both eyes. The anterior segments and pupillary reaction were normal. In the right fundus an elevated hyperemic optic disk with blurred margins, edema of the surrounding nerve fiber layer, and marked venous engorgement with perivenous hemorrhages were visible (Fig. 1). Color vision, light brightness, and visual fields were normal as were results of general physical and neurologic examination, serologic, and hematologic studies. Ultrasound Doppler of the carotid arteries and computed tomography of the orbits did not demonstrate any relevant abnormality. A diagnosis of papillophlebitis in the right eye was made, and aspirin treatment was recommended. The clinical picture returned to normal within six months. The patient had two brothers and five sisters, two of whom were 49-year-old identical twins with a history of bilateral nonarteritic anterior ischemic optic neuropathy. We examined all the family members but found no detectable systemic, vascular, hematologic, or ophthalmologic abnormalities. In
Fig. 1 (Deutsch and associates). Optic disk edema, venous engorgement, round and flame-shaped hemorrhages in the fundus of the right eye of a 41-yearold woman.
307
1905 ab
1940 1943 bd
1940 bd
F
311
26
39 DR 6 Q
M
24
1 38
44
DR 5
DR 6
b
1945 bd
~2~ 158 DR 3
c d
3
1922 ed
1947 be
1950 bd
o o
•
et
1951 be
1954 ad
Male Female
A.I.D.N. Papillophlebitis
Fig. 2 (Deutsch and associates). Familial pedigree, dates of birth, and HLA typing distribution. Inset, Parents' haplotypes are represented by a, b (F, father); and c, d (M, mother). One brother was unavailable for blood tests. A.LO.N. indicates anterior ischemic optic neuropathy.
order to define a certain genetic pattern common to the affected sisters, HLA typing was performed (Fig. 2). The affected twins had an identical pattern that was also found in two other younger, unaffected sisters. The sister with papillophlebitis had a different pattern that was shared by another unaffected sister. Berggren, Thorburn, and Fodstad" described the rare occurrence of anterior ischemic optic neuropathy in two members of the same family. A man had five children from his first marriage who were normal. He remarried, and three of seven children from the second marriage were affected by anterior ischemic optic neuropathy, two bilaterally and one unilaterally. We believe the occurrence of ischemic optic neuropathy in two members of the same family, and specifically in identical twins, is not incidental, but related to a common genetic pattern. Such affected individuals are susceptible to vascular damage, which in this case was manifest as anterior ischemic optic neuropathy. Probably environmental factors also playa role. In the case reported by Berggren, Thorburn, and Fodstad," the children from the first marriage were all normal. It was only after the second marriage that a genetic pattern was created, which together with environmental factors led to ischemic optic neuropathy in three of seven siblings. We postulate that the episode of papillophlebitis in the third sister in our case was related to a somewhat different genetic pattern. Although
308
AMERICAN JOURNAL OF OPHTHALMOLOGY
she shared some characteristics with those of the sisters affected by ischemic optic neuropathy the differences made her susceptible to vascular damage of a less severe degree. Probably because she was eight years younger, with better perfusion, was beneficial. We regard all the family members as sharing an increased risk of vascular impairment, and they should be followed up regularly.
References 1. Boghen, D. R., and Glaser, J. S.: Ischemic optic neuropathy. Brain 98:689,1975. 2. Lyle, T. K., and Wybar, K.: Retinal vasculitis. Br. J. Ophthalmol. 45:778,1961. 3. Berggren, 1., Thorburn, W., and Fodstad, H.: Three cases of non inflammatory ischemic optic neuropathy occurring in the same family. Acta OphthaJmol. 52:589,1974.
Near Syncope and Chest Tightness After Administration of Apraclonidine Before Argon Laser Iridotomy Marta H. King, M.D., and David W. Richards, M.D. Department of Ophthalmology, Medical College of Virginia. This study was supported in part by Research to Prevent Blindness, Inc.
Inquiries to Marta H. King, M.D., 5605 E. Tumbleweed Circle, Richmond, VA 23228.
Apraclonidine hydrochloride is an alpha-2 agonist currently approved for topical administration for prophylaxis against intraocular pressure increase after argon laser trabeculoplasty and iridotomy.' The drug has been used increasingly in Nd:YAG laser posterior capsulotomy procedures and has no major reported cardiovascular side effects. We treated a patient with chest tightness, loss of radial pulse, and near syncope after treatment with topical apraclonidine hydrochloride one hour before planned argon laser iridotomy. A 67-year-old white woman with no history of heart disease, arrhythmia, or syncope had chronic angle-closure glaucoma. She had a his-
September, 1990
tory of systemic hypertension, diabetes, and renal stones. Her regular medications were 72 units of insulin isophane, 80 mg of furosemide, and 50 mg of metoprolol three times daily. She had no medical allergies. The patient received one drop of 1 % apraclonidine hydrochloride in the right eye one hour before planned argon laser iridotomy and was seated in a waiting area. Approximately ten minutes after the instillation of the drug, the patient complained of chest tightness. Her radial pulse was checked and found to be strong and regular. Within one to two minutes, the pulse had become undetectable, and the patient complained of feeling faint. She was immediately placed on a stretcher and taken to an adjacent surgical recovery area. An intravenous line was opened, and a cardiac monitor was used. The first blood pressure measurement, taken approximately five minutes after the initial complaint, was 170 j80 mm Hg. At this time her pulse rate was 70 beats per minute and regular, and her blood glucose level was 180 mgjdl. An electrocardiogram showed a sinus rhythm with no acute changes. The patient remained stable and was later discharged. When interviewed, she denied that she had felt particularly anxious before the procedure and explained that her symptoms had a sudden and unexpected onset. A few days later the patient underwent successful argon laser iridotomy without the use of apraclonidine hydrochloride and had no difficulty. The beneficial effects of apraclonidine hydrochloride as a prophylactic agent for acute intraocular pressure increase caused by argon laser iridotomy, trabeculoplasty, and Nd:YAG posterior capsulotomy have been well established.' A study of 21 patients (28 eyes) undergoing argon laser iridotomy and treated prophylactically with apraclonidine hydrochloride showed no eyes with intraocular pressure increases greater than 6 mm Hg, whereas 43% of placebo-treated eyes had intraocular pressure increases of 10 mm Hg or more." The same study reported a vasovagal reaction attributed to anxiety, but the sample was too small to permit conclusions regarding cardiovascular effects. The blood-brain barrier is less permeable to apraclonidine hydrochloride than to clonidine,' therefore diminishing the potential for systemic side effects caused by the former drug. In rats, topical apraclonidine has one tenth the hypotensive effects of clonidine." In healthy human
September, 1990
AMERICAN JOURNAL OF OPHTHALMOLOGY
Full-field electroretinography was performed postoperatively. Cone, rod, 30-Hz flicker, and single bright flash electroretinograms were no longer recordable in either eye. We again compared the electroretinograms obtained for the right and left eyes in 1970 (Fig. 1), taking into account the 1.2-log unit neutral density filter created by trauma in the right eye. The electroretinograms recorded with comparable stimulus intensities in the traumatized eye never exceeded the response in the untraumatized eye. Light deprivation, even for more than 40 years, did not make the progression of retinitis pigmentosa slower in our patient. Fig. 2 (Miyake and associates). Postoperative findings of the anterior segment in the right eye. but only when the stimulus was intense. Retinitis pigmentosa was diagnosed. There was no family history of the disease. The retinitis pigmentosa gradually progressed and in 1982, visual acuity was R.E.: light perception and L.E.: 20/30. An annular maculopathy was observed in the left fundus. We opened the closed pupil of the right eye by using a vitrectomy instrument to remove the iris of the pupillary zone and the thick membrane behind the iris through the pars plana. The postoperative finding is shown in Figure 2. Before and after the operation, we measured the subjective light threshold of the right eye by changing intensities of full-field stimuli. The postoperative light threshold was 1.2 log units below the preoperative threshold. This result indicates that a filter density of 1.2 log units was removed by surgery. The right eye had been covered with a 1.2-log unit density filter for 42 years. The right and the left fundi were compared in terms of distribution of bone spicule pigmentation, degree of retinal vessel attenuation, color of the optic disk, and macular status. Little difference in fundus appearance was detected. There was no sign that the traumatic damage in the right eye involved the posterior segment. The preoperative visual acuity of light perception increased to hand motions postoperatively, but remained far lower than the visual acuity in the left eye (20/30). The lower visual acuity was most likely caused by stimulus deprivation amblyopia, since the right eye had been deprived of stimulus for 42 years, and the ophthalmoscopic macular findings were essentially the same in both eyes.
References 1. Dowling, J. E., and Sidman, R. L.: Inherited retinal dystrophy in the rat. J. Cell. BioI. 14:73, 1962. 2. La Vail, M. M., and Battelle, B. A.: Influence of eye pigmentation and light deprivation on inherited retinal dystrophy in the rat. Exp. Eye Res. 21:167, 1975. 3. Noell, W. K., and Albrecht, R.: Irreversible effects of visible light on the retina. Role of vitamin A. Science 172:76, 1971. 4. Berson, E. L.: Light deprivation and retinitis pigmentosa. Vision Res. 20:1179, 1980. 5. - - : Light deprivation for early retinitis pigmentosa. A hypothesis. Arch. Ophthalmol. 85:521, 1971.
Familial Anterior Ischemic Optic Neuropathy and Papillophlebitis David Deutsch, M.D., Eva Eting, M.D., Rahamim Avisar, M.D., Tirza Klein, Ph.D., Jacob Teller, M.D., and Hanna Savir, M.D. Department of Ophthalmology, Golda Medical Center, Hasharon Hospital and Tel-Aviv Sackler School of Medicine (D.D., E.E., R.A., J.T., and H.S.); and the Tissue Typing Laboratory, Beilinson Medical Center (T.K.).
Inquiries to David Deutsch, M.D., Department of Ophthalmology, Golda Medical Center, Hasharon Hospital, 7 Keren Kayemet St., Peiach-Tikua, Israel.
Anterior ischemic optic neuropathy is a common cause of significant visual loss in adults.' Papillophlebitis is an uncommon cause of mild
Vol. 110, No.3
Letters to the Journal
visual impairment in young adults." We studied bilateral anterior ischemic optic neuropathy in twin sisters and unilateral papillophlebitis in a third sister. A 41-year-old woman was examined because of pain and mild visual impairment in her right eye for one month. Visual acuity was 20/20 in both eyes. The anterior segments and pupillary reaction were normal. In the right fundus an elevated hyperemic optic disk with blurred margins, edema of the surrounding nerve fiber layer, and marked venous engorgement with perivenous hemorrhages were visible (Fig. 1). Color vision, light brightness, and visual fields were normal as were results of general physical and neurologic examination, serologic, and hematologic studies. Ultrasound Doppler of the carotid arteries and computed tomography of the orbits did not demonstrate any relevant abnormality. A diagnosis of papillophlebitis in the right eye was made, and aspirin treatment was recommended. The clinical picture returned to normal within six months. The patient had two brothers and five sisters, two of whom were 49-year-old identical twins with a history of bilateral nonarteritic anterior ischemic optic neuropathy. We examined all the family members but found no detectable systemic, vascular, hematologic, or ophthalmologic abnormalities. In
Fig. 1 (Deutsch and associates). Optic disk edema, venous engorgement, round and flame-shaped hemorrhages in the fundus of the right eye of a 41-yearold woman.
307
1905 ab
1940 1943 bd
1940 bd
F
311
26
39 DR 6 Q
M
24
1 38
44
DR 5
DR 6
b
1945 bd
~2~ 158 DR 3
c d
3
1922 ed
1947 be
1950 bd
o o
•
et
1951 be
1954 ad
Male Female
A.I.D.N. Papillophlebitis
Fig. 2 (Deutsch and associates). Familial pedigree, dates of birth, and HLA typing distribution. Inset, Parents' haplotypes are represented by a, b (F, father); and c, d (M, mother). One brother was unavailable for blood tests. A.LO.N. indicates anterior ischemic optic neuropathy.
order to define a certain genetic pattern common to the affected sisters, HLA typing was performed (Fig. 2). The affected twins had an identical pattern that was also found in two other younger, unaffected sisters. The sister with papillophlebitis had a different pattern that was shared by another unaffected sister. Berggren, Thorburn, and Fodstad" described the rare occurrence of anterior ischemic optic neuropathy in two members of the same family. A man had five children from his first marriage who were normal. He remarried, and three of seven children from the second marriage were affected by anterior ischemic optic neuropathy, two bilaterally and one unilaterally. We believe the occurrence of ischemic optic neuropathy in two members of the same family, and specifically in identical twins, is not incidental, but related to a common genetic pattern. Such affected individuals are susceptible to vascular damage, which in this case was manifest as anterior ischemic optic neuropathy. Probably environmental factors also playa role. In the case reported by Berggren, Thorburn, and Fodstad," the children from the first marriage were all normal. It was only after the second marriage that a genetic pattern was created, which together with environmental factors led to ischemic optic neuropathy in three of seven siblings. We postulate that the episode of papillophlebitis in the third sister in our case was related to a somewhat different genetic pattern. Although
308
AMERICAN JOURNAL OF OPHTHALMOLOGY
she shared some characteristics with those of the sisters affected by ischemic optic neuropathy the differences made her susceptible to vascular damage of a less severe degree. Probably because she was eight years younger, with better perfusion, was beneficial. We regard all the family members as sharing an increased risk of vascular impairment, and they should be followed up regularly.
References 1. Boghen, D. R., and Glaser, J. S.: Ischemic optic neuropathy. Brain 98:689,1975. 2. Lyle, T. K., and Wybar, K.: Retinal vasculitis. Br. J. Ophthalmol. 45:778,1961. 3. Berggren, 1., Thorburn, W., and Fodstad, H.: Three cases of non inflammatory ischemic optic neuropathy occurring in the same family. Acta OphthaJmol. 52:589,1974.
Near Syncope and Chest Tightness After Administration of Apraclonidine Before Argon Laser Iridotomy Marta H. King, M.D., and David W. Richards, M.D. Department of Ophthalmology, Medical College of Virginia. This study was supported in part by Research to Prevent Blindness, Inc.
Inquiries to Marta H. King, M.D., 5605 E. Tumbleweed Circle, Richmond, VA 23228.
Apraclonidine hydrochloride is an alpha-2 agonist currently approved for topical administration for prophylaxis against intraocular pressure increase after argon laser trabeculoplasty and iridotomy.' The drug has been used increasingly in Nd:YAG laser posterior capsulotomy procedures and has no major reported cardiovascular side effects. We treated a patient with chest tightness, loss of radial pulse, and near syncope after treatment with topical apraclonidine hydrochloride one hour before planned argon laser iridotomy. A 67-year-old white woman with no history of heart disease, arrhythmia, or syncope had chronic angle-closure glaucoma. She had a his-
September, 1990
tory of systemic hypertension, diabetes, and renal stones. Her regular medications were 72 units of insulin isophane, 80 mg of furosemide, and 50 mg of metoprolol three times daily. She had no medical allergies. The patient received one drop of 1 % apraclonidine hydrochloride in the right eye one hour before planned argon laser iridotomy and was seated in a waiting area. Approximately ten minutes after the instillation of the drug, the patient complained of chest tightness. Her radial pulse was checked and found to be strong and regular. Within one to two minutes, the pulse had become undetectable, and the patient complained of feeling faint. She was immediately placed on a stretcher and taken to an adjacent surgical recovery area. An intravenous line was opened, and a cardiac monitor was used. The first blood pressure measurement, taken approximately five minutes after the initial complaint, was 170 j80 mm Hg. At this time her pulse rate was 70 beats per minute and regular, and her blood glucose level was 180 mgjdl. An electrocardiogram showed a sinus rhythm with no acute changes. The patient remained stable and was later discharged. When interviewed, she denied that she had felt particularly anxious before the procedure and explained that her symptoms had a sudden and unexpected onset. A few days later the patient underwent successful argon laser iridotomy without the use of apraclonidine hydrochloride and had no difficulty. The beneficial effects of apraclonidine hydrochloride as a prophylactic agent for acute intraocular pressure increase caused by argon laser iridotomy, trabeculoplasty, and Nd:YAG posterior capsulotomy have been well established.' A study of 21 patients (28 eyes) undergoing argon laser iridotomy and treated prophylactically with apraclonidine hydrochloride showed no eyes with intraocular pressure increases greater than 6 mm Hg, whereas 43% of placebo-treated eyes had intraocular pressure increases of 10 mm Hg or more." The same study reported a vasovagal reaction attributed to anxiety, but the sample was too small to permit conclusions regarding cardiovascular effects. The blood-brain barrier is less permeable to apraclonidine hydrochloride than to clonidine,' therefore diminishing the potential for systemic side effects caused by the former drug. In rats, topical apraclonidine has one tenth the hypotensive effects of clonidine." In healthy human
