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American Academy of Ophthalmology and Otolaryngology 1976
AMERICAN JOURNAL OF OPHTHALMOLOGY FRANK
W.
NEWELL,
Editor-in-Chief
233 East Ontario St., Chicago, Illinois 60611 EDITORIAL BOARD Mathea R. Allansmith, Boston Douglas R. Anderson, Miami Crowell Beard, San Jose Bernard Becker, St. Louis Benjamin F. Boyd, Panama Charles J. Campbell, New York Thomas Chalkley, Chicago Claes H. Dohlman, Boston Sir Stewart Duke-Elder, London Fred Ederer, Bethesda J. Terry Ernest, Chicago
Published
DuPont Guerry III, Richmond Michael J. Hogan,J San Francisco Robert W. Hollenhorst, Rochester Herbert E. Kaufman, Gainesville Arthur H. Keeney, Louisville Bertha A. Klien, Tucson Carl Kupfer, Bethesda James E. Lebensohn, Chicago Irving H. Leopold, Irvine A. Edward Maumenee, Baltimore Irene H. Maumenee, Baltimore Edward W. D. Norton, Miami
monthly by the Ophthalmic
G. Richard O'Connor, San Francisco Arnall Patz, Baltimore Steven M. Podos, New York Albert M. Potts, Louisville Algernon B. Reese, New York Robert D. Reinecke, Albany Marvin L. Sears, New Haven David Shoch, Chicago Bruce E. Spivey, San Francisco Bradley R. Straatsma, Los Angeles Gunter K. von Noorden, Houston
Publishing
Company
233 East Ontario St., Chicago, Illinois 60611 Directors: A. EDWARD MAUMENEE, President; MICHAEL J. HOGAN.J Vice President; FRANK W. NEWELL, SECRETARY AND TREASURER; EDWARD W. D. NORTON, DAVID SHOCH, BRADLEY R. STRAATSMA
JDeceased November 6, 1976.
AMERICAN ACADEMY O F OPHTHALMOLOGY AND OTOLARYNGOLOGY 1976 The 81st Annual Meeting of the Ameri can Academy of Ophthalmology and Otolaryngology attracted some 15,076 par ticipants to the spacious Las Vegas Con vention Center October 5-10,1976. Those who attended had an exhausting week: Satellite meetings were devoted to many special techniques and specialties; the scientific sessions in the convention cen ter arena and the instruction courses were filled to overflowing; and there were alumni gatherings and unlimited busi ness was to be transacted. The scientific ex hibits were superb and the technical ex hibits filled the display floor in a manner that did not seem possible ten years ago. J. Donald M. Gass, in the XXXIII Edward Jackson Memorial Lecture, "Problems in the differential diagnosis of choroidal nevi and melanomas," empha
sized the benign nature of spindle cell melanomas and the malignant nature of those with epithelioid cells. H e urged caution in enucleation of eyes with tu mors and stressed continued observation for evidence of growth. H e believed the phosphorus uptake test ( 32 P) was not indi cated unless the tumor enlarged. In con trast, his lecture was followed by William S. Hagler, William H. Jarrett, and John H. Killian who thought 3 2 P testing to be most important in the diagnosis of malignant melanoma. J. Elliott Blaydes described 8-0, 9-0, and 10-0 Vicryl and Dexon su tures, which appear most promising in ophthalmic surgery. H. Stanley Thompson led a symposium on the pupil in clinical diagnosis with consideration of afferent pupillary de fects, failure of light reaction, simple anisocoria, Homer's syndrome, Adie's tonic pupil, and anisocoria. Stephen M. Litinsky, Robert N. Shaffer, John Hethering-
944
VOL. 82, NO. 6 ton, and H. Dunbar Hoskins described their extensive experience with goniotomy in congenital glaucoma and urged surgery of both eyes during one anesthe sia to avoid problems of multiple general anesthesias. Richard J. Simmons, David K. Dueker, and Richard L. Kimbrough described successful treatment of angle neovascularization using an argon laser with a 50-μ spot size. Cryotherapy was described for treatment of a variety of conjunctival skin tumors and for retinal angiomas. Andrew P. Ferry showed per manent destruction of the pigmented epi thelium of the ciliary body after cyclocryotherapy. A symposium on retinal vascular dis ease and its treatment suggested that laser photocoagulation in central vein closure, although not associated with particular visual improvement, prevented complica tions such as glaucoma. H. MacKenzie Freeman chaired an excellent symposi um, cosponsored by the Retina Society, on the treatment of macular disorders with the argon laser. Laser coagulation was recommended in persistent serous choroidopathy, for recurrent cases, and when early resolution is necessary. It is recommended for nonproliferative dia betic maculopathy particularly if there is no severe capillary loss. Arnall Patz warned against a short exposure time and small spot size in treating subretinal neo vascularization. A retrospective study of 23 patients by Robert A. Nozik indicated exacerbation of ocular toxoplasmosis after periocular corticosteroids were administered. A sympo sium on keratoprosthesis led by Arthur G. DeVoe contrasted the occasional dramatic improvement in vision with a bleak over all statistical prognosis. Administratively, the divisions of oph thalmologists and otolaryngologists oper ated nearly independently amidst great peace and harmony. President John F. Daly announced that the entire Council
EDITORIAL
945
of the Academy favored incorporation and that the fellows would be provided with information necessary to make a wise decision concerning reorganization. Named to honorary fellowships in oph thalmology were Juan Arentsen of Chile, G. Battista Bietti of Italy, Jules François of Belgium, Hans Goldmann of Switzer land, Barry Jones of England, Gerd Meyer-Schwickerath of West Germany, and Saiichi Mishima of Japan. W. How ard Morrison was given a scroll and a standing ovation in appreciation for his active editorial direction of the Academy since 1940. Jules Stein awarded the Re search to Prevent Blindness Trustees Award to A. Edward Maumenee for his contributions in nearly every phase of ophthalmology. David Weeks of Research to Prevent Blindness was presented with the Academy's Distinguished Service Award. The ophthalmology film program spon sored by the Continuing Education Ex hibit and Film Program Committee awarded first prize to Louis J. Girard for a film on the anterior adnexa; second prize to Arnall Patz, David H. Orth, and Stuart L. Fine for a film on the principles and clinical application of fluorescein angiography; and third prize to Richard K. Fors ter for a film on the diagnosis of keratomycoses. The Council approved meeting in Dal las, Texas, October 2-6, 1977. Thereafter, the divisions of ophthalmology and otolaryngology will meet separately, possi bly at different times and places. The division of ophthalmology has an option to meet in Las Vegas in 1978 but the final decision will be made after survey of the convention facilities in Chicago and Kan sas City. San Francisco was approved as the site for the 1982 meeting when the United States will host the International Con gress of Ophthalmology October 31 to November 5. The Academy will meet
946
AMERICAN JOURNAL OF OPHTHALMOLOGY
immediately before or immediately after the International Congress. Bradley R. Straatsma, who this year guided the division of ophthalmology with exceptional statesmanship and com petence as president-elect, jaecomes presi dent of the Academy January 1, 1977. Ophthalmic officers elected were: first vice-president, Robert W. Hollenhorst; councillor, Marshall M. Parks; and mem bers at large for the Division of Oph thalmology, Byron H. Demorest of Sacra mento and Richard O. Schultz of Mil waukee. Stanley Truehlson continues as editor of the Academy publications. The Ophthalmic Secretariat continues with Frederick C. Blodi as secretary for oph thalmology; David Shoch, secretary for instruction; and Bruce Spivey, secretary for continuing education. As the new au tonomous ophthalmic division develops, Bruce Spivey will undertake the main administrative role. David Paton will be assistant secretary of continuing educa tion in ophthalmology, and Robert D. Reinecke will be assistant secretary of ophthalmology. K E N N E T H R. D I D D I E F R A N K W. N E W E L L
CORRESPONDENCE Letters to the Editor must be typed double-spaced on 8V2 x 11-inch bond paper, with lV2-inch margins on all four sides, and limited in length to two manuscript pages.
A Variant of Neimann-Pick Disease Editor: In 1970 Dawson and Stein 1 reported the biochemical findings in a child with a severe neurovisceral disease. Lactosyl ceramide was found to be accumulated in abnormal concentrations in the central
DECEMBER, 1976
nervous system, sympathetic ganglia, liv er, spleen, lymph nodes, lungs, and bone marrow. The liver biopsy also demon strated increased storage of other glycolipids including glucocerebroside and G M 3 ganglioside. The activity of lactosyl ceramide ß-galactosidase was measured in liver and cultured fibroblasts from this patient and had decreased (10 to 20% of normal). In keeping with the known path ological chemistry of other lipid storage diseases, this decrease in the catabolic enzyme was expected and the entity has come to be called "lactosyl ceramidosis." We evaluated the eyes of the patient in consultation and noted pale optic disks and pale grayish maculae. The eyes were obtained at autopsy and histochemical and electron microscopic studies revealed a marked decrease in the number of reti nal ganglion cells. The existing ganglion cells contained lysosomal inclusion bodies. 2 Subsequently, this entity has been widely reported as a distinct entity. Recently two assay methods have been developed to measure two genetically dis tinct enzymes that degrade lactosyl cera mide. 3 - 4 Accordingly, fibroblast cultures from the previously described patient with lactosyl ceramidosis were devaluat ed for lactosyl ceramide cleaving activity by using these two new assay methods. 5 No deficiency of either of the lactosyl ceramide-cleaving enzymes was ob served. In addition, sphingomyelinase ac tivity was reduced to one-sixth normal, while all other enzymes were within the normal ranges. The discrepancy in the activity of the lactosyl ceramide ß-galactosidase from that measured pre viously 1 resides in the need to use sodium taurocholate in the assay. 5 Dawson, Matalon, and Stein's original assay 6 did not use sodium taurocholate causing a facti tiously low value for lactosyl ceramide ß-galatosidase. Lactosyl ceramide has been stored to some extent in certain organs in children
W.
NEWELL,
Editor-in-Chief
233 East Ontario St., Chicago, Illinois 60611 EDITORIAL BOARD Mathea R. Allansmith, Boston Douglas R. Anderson, Miami Crowell Beard, San Jose Bernard Becker, St. Louis Benjamin F. Boyd, Panama Charles J. Campbell, New York Thomas Chalkley, Chicago Claes H. Dohlman, Boston Sir Stewart Duke-Elder, London Fred Ederer, Bethesda J. Terry Ernest, Chicago
Published
DuPont Guerry III, Richmond Michael J. Hogan,J San Francisco Robert W. Hollenhorst, Rochester Herbert E. Kaufman, Gainesville Arthur H. Keeney, Louisville Bertha A. Klien, Tucson Carl Kupfer, Bethesda James E. Lebensohn, Chicago Irving H. Leopold, Irvine A. Edward Maumenee, Baltimore Irene H. Maumenee, Baltimore Edward W. D. Norton, Miami
monthly by the Ophthalmic
G. Richard O'Connor, San Francisco Arnall Patz, Baltimore Steven M. Podos, New York Albert M. Potts, Louisville Algernon B. Reese, New York Robert D. Reinecke, Albany Marvin L. Sears, New Haven David Shoch, Chicago Bruce E. Spivey, San Francisco Bradley R. Straatsma, Los Angeles Gunter K. von Noorden, Houston
Publishing
Company
233 East Ontario St., Chicago, Illinois 60611 Directors: A. EDWARD MAUMENEE, President; MICHAEL J. HOGAN.J Vice President; FRANK W. NEWELL, SECRETARY AND TREASURER; EDWARD W. D. NORTON, DAVID SHOCH, BRADLEY R. STRAATSMA
JDeceased November 6, 1976.
AMERICAN ACADEMY O F OPHTHALMOLOGY AND OTOLARYNGOLOGY 1976 The 81st Annual Meeting of the Ameri can Academy of Ophthalmology and Otolaryngology attracted some 15,076 par ticipants to the spacious Las Vegas Con vention Center October 5-10,1976. Those who attended had an exhausting week: Satellite meetings were devoted to many special techniques and specialties; the scientific sessions in the convention cen ter arena and the instruction courses were filled to overflowing; and there were alumni gatherings and unlimited busi ness was to be transacted. The scientific ex hibits were superb and the technical ex hibits filled the display floor in a manner that did not seem possible ten years ago. J. Donald M. Gass, in the XXXIII Edward Jackson Memorial Lecture, "Problems in the differential diagnosis of choroidal nevi and melanomas," empha
sized the benign nature of spindle cell melanomas and the malignant nature of those with epithelioid cells. H e urged caution in enucleation of eyes with tu mors and stressed continued observation for evidence of growth. H e believed the phosphorus uptake test ( 32 P) was not indi cated unless the tumor enlarged. In con trast, his lecture was followed by William S. Hagler, William H. Jarrett, and John H. Killian who thought 3 2 P testing to be most important in the diagnosis of malignant melanoma. J. Elliott Blaydes described 8-0, 9-0, and 10-0 Vicryl and Dexon su tures, which appear most promising in ophthalmic surgery. H. Stanley Thompson led a symposium on the pupil in clinical diagnosis with consideration of afferent pupillary de fects, failure of light reaction, simple anisocoria, Homer's syndrome, Adie's tonic pupil, and anisocoria. Stephen M. Litinsky, Robert N. Shaffer, John Hethering-
944
VOL. 82, NO. 6 ton, and H. Dunbar Hoskins described their extensive experience with goniotomy in congenital glaucoma and urged surgery of both eyes during one anesthe sia to avoid problems of multiple general anesthesias. Richard J. Simmons, David K. Dueker, and Richard L. Kimbrough described successful treatment of angle neovascularization using an argon laser with a 50-μ spot size. Cryotherapy was described for treatment of a variety of conjunctival skin tumors and for retinal angiomas. Andrew P. Ferry showed per manent destruction of the pigmented epi thelium of the ciliary body after cyclocryotherapy. A symposium on retinal vascular dis ease and its treatment suggested that laser photocoagulation in central vein closure, although not associated with particular visual improvement, prevented complica tions such as glaucoma. H. MacKenzie Freeman chaired an excellent symposi um, cosponsored by the Retina Society, on the treatment of macular disorders with the argon laser. Laser coagulation was recommended in persistent serous choroidopathy, for recurrent cases, and when early resolution is necessary. It is recommended for nonproliferative dia betic maculopathy particularly if there is no severe capillary loss. Arnall Patz warned against a short exposure time and small spot size in treating subretinal neo vascularization. A retrospective study of 23 patients by Robert A. Nozik indicated exacerbation of ocular toxoplasmosis after periocular corticosteroids were administered. A sympo sium on keratoprosthesis led by Arthur G. DeVoe contrasted the occasional dramatic improvement in vision with a bleak over all statistical prognosis. Administratively, the divisions of oph thalmologists and otolaryngologists oper ated nearly independently amidst great peace and harmony. President John F. Daly announced that the entire Council
EDITORIAL
945
of the Academy favored incorporation and that the fellows would be provided with information necessary to make a wise decision concerning reorganization. Named to honorary fellowships in oph thalmology were Juan Arentsen of Chile, G. Battista Bietti of Italy, Jules François of Belgium, Hans Goldmann of Switzer land, Barry Jones of England, Gerd Meyer-Schwickerath of West Germany, and Saiichi Mishima of Japan. W. How ard Morrison was given a scroll and a standing ovation in appreciation for his active editorial direction of the Academy since 1940. Jules Stein awarded the Re search to Prevent Blindness Trustees Award to A. Edward Maumenee for his contributions in nearly every phase of ophthalmology. David Weeks of Research to Prevent Blindness was presented with the Academy's Distinguished Service Award. The ophthalmology film program spon sored by the Continuing Education Ex hibit and Film Program Committee awarded first prize to Louis J. Girard for a film on the anterior adnexa; second prize to Arnall Patz, David H. Orth, and Stuart L. Fine for a film on the principles and clinical application of fluorescein angiography; and third prize to Richard K. Fors ter for a film on the diagnosis of keratomycoses. The Council approved meeting in Dal las, Texas, October 2-6, 1977. Thereafter, the divisions of ophthalmology and otolaryngology will meet separately, possi bly at different times and places. The division of ophthalmology has an option to meet in Las Vegas in 1978 but the final decision will be made after survey of the convention facilities in Chicago and Kan sas City. San Francisco was approved as the site for the 1982 meeting when the United States will host the International Con gress of Ophthalmology October 31 to November 5. The Academy will meet
946
AMERICAN JOURNAL OF OPHTHALMOLOGY
immediately before or immediately after the International Congress. Bradley R. Straatsma, who this year guided the division of ophthalmology with exceptional statesmanship and com petence as president-elect, jaecomes presi dent of the Academy January 1, 1977. Ophthalmic officers elected were: first vice-president, Robert W. Hollenhorst; councillor, Marshall M. Parks; and mem bers at large for the Division of Oph thalmology, Byron H. Demorest of Sacra mento and Richard O. Schultz of Mil waukee. Stanley Truehlson continues as editor of the Academy publications. The Ophthalmic Secretariat continues with Frederick C. Blodi as secretary for oph thalmology; David Shoch, secretary for instruction; and Bruce Spivey, secretary for continuing education. As the new au tonomous ophthalmic division develops, Bruce Spivey will undertake the main administrative role. David Paton will be assistant secretary of continuing educa tion in ophthalmology, and Robert D. Reinecke will be assistant secretary of ophthalmology. K E N N E T H R. D I D D I E F R A N K W. N E W E L L
CORRESPONDENCE Letters to the Editor must be typed double-spaced on 8V2 x 11-inch bond paper, with lV2-inch margins on all four sides, and limited in length to two manuscript pages.
A Variant of Neimann-Pick Disease Editor: In 1970 Dawson and Stein 1 reported the biochemical findings in a child with a severe neurovisceral disease. Lactosyl ceramide was found to be accumulated in abnormal concentrations in the central
DECEMBER, 1976
nervous system, sympathetic ganglia, liv er, spleen, lymph nodes, lungs, and bone marrow. The liver biopsy also demon strated increased storage of other glycolipids including glucocerebroside and G M 3 ganglioside. The activity of lactosyl ceramide ß-galactosidase was measured in liver and cultured fibroblasts from this patient and had decreased (10 to 20% of normal). In keeping with the known path ological chemistry of other lipid storage diseases, this decrease in the catabolic enzyme was expected and the entity has come to be called "lactosyl ceramidosis." We evaluated the eyes of the patient in consultation and noted pale optic disks and pale grayish maculae. The eyes were obtained at autopsy and histochemical and electron microscopic studies revealed a marked decrease in the number of reti nal ganglion cells. The existing ganglion cells contained lysosomal inclusion bodies. 2 Subsequently, this entity has been widely reported as a distinct entity. Recently two assay methods have been developed to measure two genetically dis tinct enzymes that degrade lactosyl cera mide. 3 - 4 Accordingly, fibroblast cultures from the previously described patient with lactosyl ceramidosis were devaluat ed for lactosyl ceramide cleaving activity by using these two new assay methods. 5 No deficiency of either of the lactosyl ceramide-cleaving enzymes was ob served. In addition, sphingomyelinase ac tivity was reduced to one-sixth normal, while all other enzymes were within the normal ranges. The discrepancy in the activity of the lactosyl ceramide ß-galactosidase from that measured pre viously 1 resides in the need to use sodium taurocholate in the assay. 5 Dawson, Matalon, and Stein's original assay 6 did not use sodium taurocholate causing a facti tiously low value for lactosyl ceramide ß-galatosidase. Lactosyl ceramide has been stored to some extent in certain organs in children