SOCIETY PROCEEDINGS EDITED BY DONA
> J. LYLE,
M.D.
N E W ENGLAND OPHTHALMOLOGICAL SOCIETY
is essentially the same as outlined except that one-half the needle electrode applications are sufficient to cause the tumor to regress.
452nd Meeting, January 20, 1960
OCULAR MANIFESTATIONS OF CERTAIN MET
DR. BRENDAN LEAHEY,
presiding
TREATMENT OF INTRAOCULAR TUMORS WITH DIATHERMY DR. I. OKAMURA: Retinoblastoma and hemangioma of the choroid are two of the intraocular tumors most amenable to dia thermy treatment. The diagnosis of retino blastoma is usually not difficult, the first eye having been removed for an obvious neo plasm. One may encounter difficulty in locat ing all the lesions. The diagnosis of heman gioma of the choroid is more difficult and if there is uncertainty and the differential diag nosis is that of malignant melanoma, it would be safer to enucleate the eye. The shape of a hemangioma is relatively flat, the color is pink, and with pressure on the globe the tumor may be made to blanch. The hemangiomas are usually located next to the disc or in the macular area. They may be bi lateral and complicated by retinal detach ment. The diathermy treatment of retinoblas toma requires that the tumor be completely destroyed, whereas in hemangiomas partial destruction may cause the tumor to regress. The technique requires localization of the le sion under ophthalmoscopic control with a surface electrode. Various lengths of needles must be available. Twelve to 15 applications may be necessary. When gas bubbles form, they are an indication to stop the diathermy and perforate in another location. The trans lucent tumor becomes more opaque. Postop erative monthly ether examinations are" nec essary. The central necrotic tumor decreases in size and, if the therapy has been adequate, a flat scar results. The diathermy treatment of hemangiomas
ABOLIC DISEASES IN CHILDREN DR. S. LIEBMAN: The discussion was lim ited to metabolic diseases in children which are of an inherent nature. There are several large groupings and subdivisions: I. Errors of carbohydrate metabolism. A. Diabetes. There is little new about the ocular manifestations of diabetes. The development of diabetes may be precipitous in children and the ocular manifestations extremely se vere and fulminating. B. Galactosemia. This is defined as a ge netic defect in the ability of the body to con vert galactose into glucose. Cataracts develop in about 50 percent of the cases. The earliest changes appear as a highly ref ractile annular zone around the fetal nucleus, giving the lens an "oil drop" appearance. Later zonular and cortical opacities develop. Early diagno sis is obviously important since restriction of galactose intake by elimination of milk usu ally causes rapid improvement in the clinical picture, and there may be reversal of the cat aract formation if it is still in the "oil drop" stage. The disease is believed due to the ab sence of the enzyme uridyl transferase, which is usually present in all body cells and which is necessary to convert galactose into glucose. C. Hurler's disease (gargoylism). This is known to be a disorder of mucopolysaccharide metabolism. The most striking ocular manifestation is the classical diffuse corneal haze which is usually bilateral and which oc curs before the age of three years. There is interstitial fine-dot clouding of the cornea which can be seen best with the slitlamp. The deposits are yellow-gray, granular and in the deeper layers of the corneal stroma in the early stages. The epithelium and endo-
SOCIETY PROCEEDINGS thelium are usually spared and there is no vascularization. The most pronounced changes in the cornea are confined to the region of Bowman's membrane. The corneal diameters may be increased. The differential diagnosis must include congenital glaucoma. II. Errors in protein metabolism. Cystinosis (Fanconi's syndrome). This is another rare disorder and occurs almost entirely in children. It is due to a hereditary in-born error in the metabolism of many amino acids. A diffuse haziness of the cornea is caused by thousands of tiny highly ref ractile crystals noted by the microscope. III. Errors in fat metabolism. The dis eases are due to a primary constitutional de fect in lipid metabolism as revealed by chem ical and histochemical studies. A. Tay-Sachs disease. The classical fundus picture of a cherry-red spot is due to a cytoplasmic accumulation of a complex glycolipid in the ganglion cells of the retina. The especially abundant ganglion cells in the macula cause the opacity to be most striking in this region. Some vision may remain until 18 to 24 months of age. B. Niemann-Pick disease. A similar cherry-red spot in the macula may be seen in one quarter to one half of the cases. Optic atrophy, blindness, strabismus, and paralysis of vertical gaze have been described. A prominent feature is a widespread intracellular deposition producing the "foam cells." C. Gaucher's disease is similar to Nie mann-Pick disease. There is a deposition of the lipid kerasin in the lymphatic and hematopoietic apparatus. D. Xanthoma tuberosum is a form of fa milial cholesterolemia. Ocular findings in clude arcus juvenilis and xanthelasmas of the eyelids. There is an accumulation of lipid material in the histiocytes. IV. Diseases of varied etiology with occa sional secondary lipid disturbance. A. Eosinophilic granuloma, Hand-Schiiller-Christian syndrome and Letterer-Siewe disease. The diseases may show a disorder of the bony orbit, granulomas in the soft tissue of the orbit, and exophthalmos.
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B. Nevoxanthoendothelioma (xanthoma disseminatum). There are extensive lesions of the skin associated with granulomatous infiltrations of the iris. This occurs only in infants, and there may be spontaneous hem orrhage into the anterior chamber. V. Endocrine disorders. A. Hypoparathyroidism-parathyroid disease itself is rare in children. It may occur on an idiopathic basis and lens changes characterized by small dis crete angular colored crystals may appear. The serum calcium is usually below 7.0 mg. percent. The administration of calcium will not affect the progress of the cataracts. They are said rarely to interfere with vision. B. Pseudohypoparathyroidism. Severe chronic kidney disease produces disturbed calcium metabolism and characteristic bilat eral lenticular opacities. C. Cretinism. VI. Disorders of heavy metal metabolism. A. Wilson's hepaticolenticular degeneration is an excellent example of a hereditary fa milial error in heavy metal metabolism. Chemically, one finds a low serum copper and a reduction in serum copper oxidase. The Kaiser-Fleischer ring is the consistent clinical feature. It is described as golden in color but may be brown or grayish green; when well developed it is two mm. in width. It is seen in the deepest layers of the corneal periphery. It is composed of granules of de posited copper and the pigment is definitely within Descemet's membrane. A L P H A CHYMOTRYPSIN IN CATARACT SURGERY
DR. M. S. WIEDMAN: The clinical and ex
perimental aspects of the use of alpha chy motrypsin in cataract surgery were re viewed. Postoperative complications were noted not to be significantly increased. Wherever striate keratitis was observed it was probably due to protracted fluid infu sion. The author recommended restricting its use to the over 20-year age group. Discus sers of the paper were enthusiastic about the use of this drug. Discussion. DR. BEETHAM: Sixty cases
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SOCIETY PROCEEDINGS
had been tried with the use of the enzyme and, in all instances, Dr. Beetham felt he had met with great success. No capsules had been broken, no vitreous had been lost, striate keratitis was practically absent. DR. T. CAVANAUGH: Having used the erisophake for a number of years Dr. Cava naugh has reverted to the Verhoeff forceps and the use of the enzyme. Frequently the forceps is not even required. DR. E. DOWLING: At the Rhode Island hospital 500 cases have been observed in which the enzyme was used. The effort re quired for delivery of the lens and the num ber of complications have been reduced mark edly.
whereas electromyography records the elec trical activity of the individual muscles. The direct current changes rather than the alter nating current changes are important. It is hoped that these techniques will be used as additional diagnostic tools in selected prob lems of ocular motility. NYSTAGMUS SURGERY
DR. A. KESTENBAUM : Nystagmus is now
divided into three groups based on disturb ances of the fixation, the vestibular, and the gaze mechanisms. Since 1950 we have had a new era with regard to the treatment of nystagmus. Nystagmus surgery is under taken for two reasons: (1) the patient sees less because of his nystagmus, and (2) he PROGRESS REPORT ON SCHOOL VISION TESTING turns his head so that in the new position there is less nystagmus. DR. A. E. SLOANE: The New England Dr. Kestenbaum indicated on a diagram Ophthalmological Society was a pioneer in appointing a committee for the study of a that there was a neutral zone which is the school vision program. Its investigations and optimum position of the eyes. The purpose standards have been followed throughout the of the surgery is to move the eyes in the di country. There have been many testing de rection of the worst nsytagmus, that is, the vices but the Massachusetts Vision Test still direction of the quick phase. Measurements remains extremely reliable and practical be are made with a mm. rule, noting at the cause it includes a study of the visual acuity, limbus the amount of change in position hyperopia, and heterophoria. Studies for when the eye is moved to the position where stereopsis, binocular vision, and color vision the eye is exhibiting the least nystagmus. If were not included in the test because they the distance measured is five mm. the eye appeared to be time consuming and added to must be rotated five mm. into the opposite the complexity of the test. In addition, a direction. Dr. Kestenbaum insists that this larger number of referrals than is necessary will require a five-mm. resection and a fivemm. recession of the antagonist on this eye. would be made. The results of these studies have shown It must be duplicated in the other eye. One that there is less isolation of students in spe mm. of limbus rotation equals eight prism cial classes and more integration into regular diopters. This figure must be kept in mind classes with the aid of special books and and accounted for in cases of strabismus. Two types of cases are specific indications visual aids for those who are visually handi for surgery. The first is an asymmetrical capped. A further detailed report of the past nystagmus present from infancy associated 25 years of these studies is forthcoming. with a head tilt. The second is that which oc ELECTRO-OCULOGRAPHY curs in older people who do not see well in the primary position because of the nystagmus. DR. C. KUPFER: Electro-oculography is a technique whereby the movements of the eye Dr. Kestenbaum commented on latent nys are recorded by means of the corneal-retinal tagmus which occurs when an eye is cov potential of the eye. It is an outgrowth of the ered while fixing with the other eye. He has early investigation of the electroretinogram. observed a severe nystagmus develop in pa It records the movements of the entire globe, tients who have lost one eye. These cases are
SOCIETY PROCEEDINGS good subjects for operation. Turning the eye in the direction of the quick phase will re duce the amount of nystagmus in the primary position. Nystagmus in albinos might be eliminated according to Dr. Kestenbaum, if pinhole gog gles were placed on the infant for the first four months so that a sharp image could be received on the macula and aid in the devel opment of the child's fixation mechanism. In pendular nystagmus which cannot be made to disappear, the recommended meth ods of surgery cannot be applied. D. Robert Alpert, Recorder. N E W YORK SOCIETY FOR CLINICAL OPHTHALMOLOGY May 4, 1959 DR. ARTHUR LINKSZ,
presiding
AQUEOUS VEINS AND CYCLODIALYSIS
DR. KARL W. ASCHER: The detection of
aqueous veins and observation of their be havior in different types of glaucoma and after various manipulations have given an swers to many questions. They offered the first clear demonstration that there is con tinuous production and circulation of aque ous as against the previous stagnation theory, showed that almost all glaucoma is due to retention rather than overproduction of aque ous, and provided the foundation for tonography. Cyclodialysis is often a satisfactory op eration for chronic simple glaucoma, either as a primary procedure or when other sur gery has failed. Three points in the original Elschnig technique were emphasized: (1) massage over the closed lids to lower intraocular pres sure preoperatively, (2) the use of a bent but not curved cyclodialysis spatula, and (3) pressure on the limbus opposite the operative site exerted by the assistant to prevent hem orrhage. Modifications of the original technique
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which make the operation safer are: (1) the pupil should be made miotic; (2) the upper temporal quadrant, rather than the lower, is preferred so that any hemorrhage will drain away from the operative site and to keep the cleft open by the pull of the weight of the lens; (3) a fine-toothed forceps should be used to grasp the sclera between limbus and the incision for fixation; (4) the incision should be bevelled 45 degrees forward; (5) after the spatula has entered the sclera it is preferable to enlarge the dialysis by partially withdrawing the spatula and reinserting it at a slightly different angle, repeating this procedure at increasingly greater angles until the two extreme thrusts are made 180 degrees from each other. This is safer than enlarging the dialysis by upward and down ward sweeps of the spatula while the tip is in the anterior chamber, whereby iridodialysis or folding of the iris and hemorrhages may be induced. H U M A N OCULAR MYOGRAPHY
DR. ARTHUR JAMPOLSKY described the
technique and uses of electromyography. Needle electrodes are placed in the muscle, the amplified signal from either a single fiber or a group is fed to an oscilloscope which is then photographed by a kymographic camera. It is too complicated to be used in routine clinical practice, but is useful in research and in specific neurologic situations. An example of its research value is the light it sheds on the problem as to whether intermittent exotropia is due to "divergence spasm" or simply a relaxation of conver gence. By taking simultaneous records of all four horizontal rectus muscles at the instant of divergence, a picture is obtained of re laxation of the convergence mechanism, rather than activation of the divergence mechanism. Although there is obviously a divergent mechanism, it appears that, when an intermittent exotrope becomes manifestly exotropic, it is a return to the fusion-free position via relaxation of convergence. Alan H. Barnert, Corresponding Secretary.