652 AMERICAN JOURNAL OF OPHTHALMOLOGY MAY, 1984

Fig. 2 (Hida and associates). Histologic sections ofthe cornea (top, phosphotungstic acid-hematoxylin,x 160; bottom, Ziehl-Neelsen, x 160).

gelatinous guttate dystrophy after super­ficial keratectomy. 4

REFERENCES

1. Kloucek, F.: Familial band-shaped keratopathyand spheroid degeneration. Clinical and electronmicroscopic study. Albrecht von Graefes Arch. Klin.Exp. Ophthalmol. 205:47, 1977.

2. Garner, A., Morgan, G., and Tripathi, R. C.:Climatic droplet keratopathy. Pathologic findings.Arch. Ophthalmol. 89:198, 1973.

3. Klintworth, G. K.: Chronic actinic keratop­athy. A condition associated with conjunctival elasto­sis (pingueculae) and typified by characteristic extra­ocular concretions. Am. J. Pathol. 67:327, 1972.

4. Kanai, A., and Kaufman, H. E.: Electron­microscopic studies of primary band-shaped keratop­athy and gelatinous, drop-like corneal dystrophy intwo brothers. Ann. Ophthalmol. 14:535, 1982.

UNKNOWN GIANT CELL LESION11 YEARS AFTER SUCCESSFULTREATMENT OFRETINOBLASTOMA

A. HAMBURG, M.D.,AND K. E. W. P. TAN, M. D.

Inquiries to A. Hamburg, M. D. ,Ooglijdersgasthuis, 65 F. C. Dondersstraat, 3572 IEUtrecht, The Netherlands.

Eleven years after successful irradia­tion of an eye with retinoblastoma, anoth­er tumor developed in this eye, in adifferent location. Histopathologic exami­nation disclosed a flat tumorous mass inand beneath the retina, consisting of un­known large polygonal cells forming apseudosyncytium.

The patient, a girl born in 1967 withsporadic bilateral retinoblastoma, wastreated with X-irradiation to both eyes atthe age of 1 year. The tumor in the lefteye, located at the inferior periphery,regressed and remained quiet. The righteye was removed in 1969. Microscopicexamination disclosed considerable re­gression in the tumor with a few calcifiedareas and no evidence of mitoses. The lefteye showed no evidence of reactivation ofthe tumor during the following ten years.In March 1980, the patient's visual acuitysuddenly decreased in the course of a few

VOL. 97, NO. 5 LETTERS TO THE JOURNAL 653

Fig. 1 (Hamburg and Tan). Tumorous mass in andbeneath the retina, clinically suggesting an exophyticretinoblastoma, histologically consisting of large pol­ygonal cells forming a pseudosyncytium (hematoxylinand eosin, x 120).

weeks. After removal of the cataractouslens, ophthalmoscopy disclosed a tumorin the posterior pole and an extensiveretinal detachment nasally. At the site ofthe original tumor was a cicatricial areabeneath that was not connected to theother lesion. It was concluded that therewas "recurrence or new growth of retino­blastoma" and the eye was irradiated asecond time. On repeated ultrasonog­raphy the tumor first increased slightly insize and then decreased in the course ofthe next few months, but the detachmentremained unchanged. Because of increas­ing intraocular pressure this completelyblind eye was removed in November1980.

Microscopic examination disclosed a

Fig. 2 (Hamburg and Tan). Large cell with gran­ules and vacuoles. Large nucleus with prominentnucleolus (hematoxylin and eosin, x 3(0).

flat mass, resembling an exophytic retinaltumor, surrounded by a flat detachment,in the posterior pole. The subretinal fluidcontained numerous bladder cells. Themass consisted of large polygonal, irregu­larly shaped cells, which formed a pseu­dosyncytium and contained large to verylarge monochromatic, sometimes hyper­chromatic nuclei and a large prominentnucleolus (Fig. 1). Some cells containedmore than one nucleus. The glassy, ho­mogenous, and sometimes foamy cyto­plasm contained vacuoles and PAS­positive granules (Fig. 2). It stainedfaintly with the Kluver-Barrera tech­nique. This staining suggested a Nisslsubstance, but the granules were notarranged in the pattern of ganglion cells.The space between the cells was filledwith hyaline connective tissue containing

654 AMERICAN JOURNAL OF OPHTHALMOLOGY MAY, 1984

blood vessels, a few small hemorrhages, afew psammoma-like calcifications, andsome inflammatory cells. The border withthe choroid was indistinct; the pigmentepithelium in this area was lacking. Ante­rior peripheral synechiae were present.It is unlikely that this clinical picture hasanything to do with retinoblastoma; itcould perhaps be interpreted as an un­known late reaction to irradiation or to anunknown infectious agent. The large pol­ygonal cells have been called ganglioncells, histiocytes, and pseudoxanthomat­ous (perhaps endothelial) cells." We favorthe supposition that this lesion is "giantcell pseudoxanthoma."

REFERENCE

1. Ashton, ]1;., in discussion, Taktikos, A.: Retinalhaemangioblastoma. Br. J. Ophthalmoi. 46:692,1962.

COMMEMORATING '84

HARRY H. MARK, M.D.

Inquiries to Harry H. Mark, M. D. , 2 Church St.S. , New Haven, CT 06519.

The year 1984 was made popular byGeorge Orwell's novel of that title. Herein New Haven, however, we think of it asthe bicentennial of the founding of ourCounty Medical Society, probably thethird oldest in the country, the Litchfield(Connecticut) County Medical Societybeing the oldest. Ophthalmology, too,commemorates the year as the anniversa­ry of several milestones in its history.

Three centuries ago the scientific ren­aissance lost one of its more illustriousleaders, Edme Mariotte (1620-1684) ofDijon. We remember him for the discov­ery of the blind spot in 1666. Mariotte

endeavored to settle the question ofwhether the seat of the soul and sensa­tions was in the heart and blood or in thebrain and nerves. Personal study ofanato­my taught him that the vascular choroidwas absent at the entry site of the opticnerve where the nervous retina was yetpresent. To find out which of the two wasthe truly sensitive organ he tested hisvision in the optic disk area, and found itmissing. He concluded of course that thevascular choroid was the true organ ofvision.

Mariette's discovery thus furnishes aninstructive example of scientific method­ology, according to Joseph Priestley':

. . .it is by no means necessary to have justviews, and a true hypothesis, a priori, in orderto make real discoveries. Very lame and imper­fect theories are sufficient to suggest usefulexperiments, which serve to correct those the­ories, and give rise to others more perfect.

Two hundred years ago BenjaminFranklin, a layman, invented bifocals,and thereby saved mankind some timeand effort otherwise spent on endlessswitching from distance to reading glass­es. The invention was first recorded in aletter written on Aug. 21, 1784, by Frank­lin in Paris to his friend Whatley inLondon. Less known is the curious cir­cumstance that it first appeared in theprofessional literature in William Row­ley's notoriously plagiarized book, "ATreatise on One Hundred and EighteenPrincipal Diseases of the Eyes and Eye­lids. "2

Fifty years later, in 1834, HermanSnellen was born in Utrecht. His stan­dards for "optotypic" eyecharts are usedworldwide to this day.

In 1884 Crede summarized his investi­gations in ophthalmia neonatorum in amonograph that remains a model of me­ticulous research and concise writing."

Finally, in 1984 we celebrate the cen­tennial of Carl Koller's introduction oftopical anesthesia, without which we canill imagine doing our work-whether go-