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The Exfoliation Syndrome in the Aphakic Eye
NOTES, CASES AND INSTRUMENTS T H E EXFOLIATION SYNDROME IN T H E APHAKIC EYE W I L L I A M C. C A C C A M I S E ,
M.D.
Rochester, New York 1
Sugar recently reported two cases of exfoliation syndrome a number of years after cataract surgery. In 1975, Radian and Radian 2 of Romania described two patients with similar findings. I have treated a fifth patient with this rare condition. CASE REPORT
A 65-year-old man was first examined on Dec. 9, 1966. A slit-lamp examination with the pupils dilated disclosed nuclear and posterior subcapsular cataractous changes in both eyes. There was no evidence of exfoliative material in either eye. On Jan. 5, 1967, the patient underwent an uncomplicated intracapsular cataract extraction with a peripheral iridectomy on the right eye. Three months later, his best corrected visual acuity was 6/6 (20/20). On May 1, 1967, he underwent an operation with similar results on the left eye. From 1967 through 1977 he was re-examined approximately every three years. On each occasion there was no significant change in ocular status. On July 22, 1977, a slit-lamp examination with the pupils dilated disclosed no evidence of exfoliative material. Slit-lamp gonioscopy with the Goldmann lens showed the open angle typical of the aphakic state in both eyes. There was normal pigmentation of the posterior trabeculum. Indirect ophthalmoscopic ex amination of the ora serrata disclosed no fundus abnormalities. Applanation tonometry demonstrated normal intraocular pressures of 16 to 18 mm Hg in each eye. On Sept. 5, 1980, the patient returned to the office for re-examination. He had no specific ocular or visual complaint. No change in visual acuity or refractive error was evident. However, there was a striking change in both eyes on slit-lamp examina tion. The pupillary margin was laden with the scaly material typical of the exfoliation syndrome. The grayish flecks involved the entire circumference of From the Department of Ophthalmology, Univer sity of Rochester Medical Center, Rochester, New York. Reprint requests to William C. Caccamise, M.D., 300 White Spruce Blvd., Rochester, NY 14623.
the pupillary margin. In the pupillary area, the vitreous face was densely impregnated with the same dandruff-like deposits. Slit-lamp gonioscopy dis closed no change in the angle details. There was no increase in the pigmentation of the posterior trabecu lum. Results of an ophthalmoscopic examination were normal. Applanation tonometry again dem onstrated normal intraocular pressures of 16 to 18 mm Hg in each eye. DISCUSSION
The exact source of the exfoliative material evident with the slit-lamp re mains conjectural. Light and electron microscopic studies have been used to support conflicting opinions. The cases reported by Sugar 1 and by Radian and Radian, 2 along with the present case, conclusively indicate that there is at least one type of exfoliation syndrome in which the exfoliative material is not derived from the lens. However, this does not preclude the possibility of two other types of the same syndrome. In one type the lens may be solely responsible for the production of the exfoliative material. Electron micro scopic studies 3 and reports 4 of regression of the exfoliative material after intracapsular cataract extraction have supported this concept. Conversely, there have been reports 5 ' 6 of an increase in the exfoliative material after intracapsular cataract extraction in an eye already manifesting the exfoliation syndrome. There may be a third type of exfoliation syndrome in which the lens and other intraocular structures both contribute to the production of the exfoliative material. To date there have been no studies conclusively supporting or refuting the existence of this third type. SUMMARY
A 79-year-old man developed the classical signs of exfoliation syndrome bi1
112
AMERICAN JOURNAL OF OPHTHALMOLOGY
laterally ten to 13 years after bilateral intracapsular cataract surgery. There is, therefore, at least one type of exfoliation syndrome where the lens does not pro duce the exfoliative material. REFERENCES 1. Sugar, H. S.: Onset of the exfoliation syndrome after intracapsular lens extraction. Am. J. Ophthal mol. 89:601, 1980. 2. Radian, A. B., and Radian, A. L. : Senile pseudoexfoliation in aphakic eyes. Br. J. Ophthalmol. 59:577, 1975.
OPHTHALMIC
JANUARY, 1981
3. Bertelsen, T. I., Drablos, P. A., and Flood, P. R. : The so-called senile exfoliation (pseudoexfoli ation) of the anterior lens capsule, a product of the lens epithelium. Fibrillopathia epitheliocapsularis. Acta Ophthalmol. 42:1096, 1964. 4. Gillies, W. E.: Effect of lens extraction in pseudoexfoliation of the lens capsule. Br. J. Ophthal mol. 57:46, 1973. 5. Gifford, H., Jr.: A clinical and pathologic study of exfoliation of the lens capsule. Am. J. Ophthalmol. 46:508, 1958. 6. Sugar, H. S.: The exfoliation syndrome. Source of the fibrillar material on the capsule. Surv. Oph thalmol. 21:59, 1976.
MINIATURE
Although Teddie blazed away determinedly at the fauna of the Lower Hudson Valley (the Roosevelts had taken a summer house at Dobbs Ferry), he found, to his bewilderment, that he could not hit anything. Even more puzzling was the fact that his friends, using the same gun, seemed to be able to bag the invisible: they fired into the blue blur of the sky, or the green blur of the trees, whereupon specimens mysteriously dropped out of nowhere. The truth was slow to dawn on him. "One day they read aloud an advertisement in huge letters on a huge distant billboard, and I then realized that something was the matter, for not only was I unable to read the sign, but I could not even see the letters. I spoke of this to my father, and soon afterwards got my first pair of spectacles, which literally opened an entirely new world to me. I had no idea how beautiful the world was until I got those spectacles . . . while much of my clumsiness and awkwardness was doubtless due to general characteristics, a good deal of it was due to the fact that I could not see, and yet was wholly ignorant that I was not seeing." Edmund Morris, The Rise of Theodore Roosevelt New York, Ballantine, 1979
M.D.
Rochester, New York 1
Sugar recently reported two cases of exfoliation syndrome a number of years after cataract surgery. In 1975, Radian and Radian 2 of Romania described two patients with similar findings. I have treated a fifth patient with this rare condition. CASE REPORT
A 65-year-old man was first examined on Dec. 9, 1966. A slit-lamp examination with the pupils dilated disclosed nuclear and posterior subcapsular cataractous changes in both eyes. There was no evidence of exfoliative material in either eye. On Jan. 5, 1967, the patient underwent an uncomplicated intracapsular cataract extraction with a peripheral iridectomy on the right eye. Three months later, his best corrected visual acuity was 6/6 (20/20). On May 1, 1967, he underwent an operation with similar results on the left eye. From 1967 through 1977 he was re-examined approximately every three years. On each occasion there was no significant change in ocular status. On July 22, 1977, a slit-lamp examination with the pupils dilated disclosed no evidence of exfoliative material. Slit-lamp gonioscopy with the Goldmann lens showed the open angle typical of the aphakic state in both eyes. There was normal pigmentation of the posterior trabeculum. Indirect ophthalmoscopic ex amination of the ora serrata disclosed no fundus abnormalities. Applanation tonometry demonstrated normal intraocular pressures of 16 to 18 mm Hg in each eye. On Sept. 5, 1980, the patient returned to the office for re-examination. He had no specific ocular or visual complaint. No change in visual acuity or refractive error was evident. However, there was a striking change in both eyes on slit-lamp examina tion. The pupillary margin was laden with the scaly material typical of the exfoliation syndrome. The grayish flecks involved the entire circumference of From the Department of Ophthalmology, Univer sity of Rochester Medical Center, Rochester, New York. Reprint requests to William C. Caccamise, M.D., 300 White Spruce Blvd., Rochester, NY 14623.
the pupillary margin. In the pupillary area, the vitreous face was densely impregnated with the same dandruff-like deposits. Slit-lamp gonioscopy dis closed no change in the angle details. There was no increase in the pigmentation of the posterior trabecu lum. Results of an ophthalmoscopic examination were normal. Applanation tonometry again dem onstrated normal intraocular pressures of 16 to 18 mm Hg in each eye. DISCUSSION
The exact source of the exfoliative material evident with the slit-lamp re mains conjectural. Light and electron microscopic studies have been used to support conflicting opinions. The cases reported by Sugar 1 and by Radian and Radian, 2 along with the present case, conclusively indicate that there is at least one type of exfoliation syndrome in which the exfoliative material is not derived from the lens. However, this does not preclude the possibility of two other types of the same syndrome. In one type the lens may be solely responsible for the production of the exfoliative material. Electron micro scopic studies 3 and reports 4 of regression of the exfoliative material after intracapsular cataract extraction have supported this concept. Conversely, there have been reports 5 ' 6 of an increase in the exfoliative material after intracapsular cataract extraction in an eye already manifesting the exfoliation syndrome. There may be a third type of exfoliation syndrome in which the lens and other intraocular structures both contribute to the production of the exfoliative material. To date there have been no studies conclusively supporting or refuting the existence of this third type. SUMMARY
A 79-year-old man developed the classical signs of exfoliation syndrome bi1
112
AMERICAN JOURNAL OF OPHTHALMOLOGY
laterally ten to 13 years after bilateral intracapsular cataract surgery. There is, therefore, at least one type of exfoliation syndrome where the lens does not pro duce the exfoliative material. REFERENCES 1. Sugar, H. S.: Onset of the exfoliation syndrome after intracapsular lens extraction. Am. J. Ophthal mol. 89:601, 1980. 2. Radian, A. B., and Radian, A. L. : Senile pseudoexfoliation in aphakic eyes. Br. J. Ophthalmol. 59:577, 1975.
OPHTHALMIC
JANUARY, 1981
3. Bertelsen, T. I., Drablos, P. A., and Flood, P. R. : The so-called senile exfoliation (pseudoexfoli ation) of the anterior lens capsule, a product of the lens epithelium. Fibrillopathia epitheliocapsularis. Acta Ophthalmol. 42:1096, 1964. 4. Gillies, W. E.: Effect of lens extraction in pseudoexfoliation of the lens capsule. Br. J. Ophthal mol. 57:46, 1973. 5. Gifford, H., Jr.: A clinical and pathologic study of exfoliation of the lens capsule. Am. J. Ophthalmol. 46:508, 1958. 6. Sugar, H. S.: The exfoliation syndrome. Source of the fibrillar material on the capsule. Surv. Oph thalmol. 21:59, 1976.
MINIATURE
Although Teddie blazed away determinedly at the fauna of the Lower Hudson Valley (the Roosevelts had taken a summer house at Dobbs Ferry), he found, to his bewilderment, that he could not hit anything. Even more puzzling was the fact that his friends, using the same gun, seemed to be able to bag the invisible: they fired into the blue blur of the sky, or the green blur of the trees, whereupon specimens mysteriously dropped out of nowhere. The truth was slow to dawn on him. "One day they read aloud an advertisement in huge letters on a huge distant billboard, and I then realized that something was the matter, for not only was I unable to read the sign, but I could not even see the letters. I spoke of this to my father, and soon afterwards got my first pair of spectacles, which literally opened an entirely new world to me. I had no idea how beautiful the world was until I got those spectacles . . . while much of my clumsiness and awkwardness was doubtless due to general characteristics, a good deal of it was due to the fact that I could not see, and yet was wholly ignorant that I was not seeing." Edmund Morris, The Rise of Theodore Roosevelt New York, Ballantine, 1979