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Vitrectomy and Wagner's Vitreoretinal Degeneration: Reply
256
AMERICAN JOURNAL OF OPHTHALMOLOGY
agnosis is mandatory for appropriate treatment, genetic counseling, useful re ferral consultations, and provision of a reliable prognostication. What is now usually called vitreoretinal degeneration of Wagner is one of perhaps several enti ties clearly separable and distinct from the condition originally described by Wagner. Awareness of the genetic hetero geneity in patients with hyaloideoretinal degeneration will permit accumulation of the data necessary to permit assortment of these similar yet genetically separate enti ties. G. FRANK J U D I S O N ,
M.D.
Iowa City, Iowa REFERENCES 1. Wagner, H.: Ein bisher unbekantes Erbleiden des Auges (Degeneratio hyaloideo-retinalis hereditaria), beobachtet im Kanton Zurich. Klin. Monatsbl. Augenheilkd. 100:840, 1938. 2. Bohringer, H. R., Dieterle, P., and Landolt, E.: Zur Klinik und Pathologie der Degeneratio hvaloideo-retinalis hereditaria (Wagner). Ophthalrriol. 139:330, 1960. 3. Ricei, A.: Degenerescence Hyaloideo-retinienne de Wagner. Bull. Des. Soc. Ophtal. Franc. 1961. 4. Stickler, G. B., Belau, P. G. Farrell, F. J., Jones, J. D., Pugh, D. G., Steinberg, A. G., and Ward, L.E.: Hereditary progressive arthro-ophthalmopathy. Mayo Clin. Proc. 40:433, 1965. 5. Popkin, J. S., and Polomeno, R. C.: Stickler's svndrome (hereditary progressive arthro-ophthalmopathy). CM A J. 111:1071, 1974.
Reply Editor: Although nine of Wagner's original 13 patients had myopia of less than or equal to - 4 . 0 diopters, four, or approximately one third, had myopia ranging from - 5 . 2 5 to - 3 0 diopters in the more myopic eye. Wagner's paper states, " . . . without exception, all affected persons exhibited myopia, usually low grade, but some times more severe." Thus the presence of severe myopia in itself does not seem to exclude our patients and those of other
FEBRUARY, 1979
investigators 2,3 from being Wagner's vari ants. Descriptions of cataract are varied in Wagner's original article. Included are anterior and posterior cortical punctate and stellate opacities, subcapsular opaci ties, and diffuse homogenous nuclear tur bidity. One of our patients had a posterior subcapsular lens opacity, whereas two did not. Two of Wagner's juvenile pa tients (ages 5 and 6 years) had clear lenses, and cataracts were not usually prominent until adolescence. Certainly other au thors 2 - 4 have noted these opacities, which are frequently cortical, but may vary. Wagner did not originally describe the association of retinal detachment in his patients, a point we made in our article. Jansen, 4 though, noted the frequent oc currence of retinal detachment, often at an early age, in his patients with mild to moderate (although sometimes severe) myopia, cataracts, optically empty vitre ous with membranes, and peripheral and perivascular retinal pigmentation. His de scriptions are similar to those of Wagner and emphasize the association with reti nal detachment. Palatoschisis has been well noted in Wagner's variants, 2,5 as have hyperextensible joints and chondrodysplasia. 2 Micrognathia and glassoptosis in two pa tients with Wagner's disease and Pierre Robin syndrome have also been de scribed. 2 It was not our objective to dis cuss the systemic associations known to occur with Wagner variants but rather to suggest therapy for recurrent retinal de tachment in the disease. When one considers that most diseases involve a continuum along the spectrum of manifestations, it is not unreasonable to group the original Wagner patients and subsequently described patients. 2 - 5 Cer tain points of selected articles can usually be found to support disassoeiation, as well as association, of patient groups. Whether Wagner's disease and Stickler's
VOL. 87, NO. 2
BOOK REVIEWS
syndrome, described 27 years later at a time of greater medical sophistication, are distinctly separate clinical entities or variants of the same genetic entity is a difficult question to resolve. It is bold to say with certainty that they are clearly separable and distinct. In summary, whether one chooses to label the patients we have described as having Wagner's disease or Stickler's syn drome, both points of view can probably be well supported. Of more importance is the hazard of eponymic terminology where heredogenetic variation tends to occur. Indeed, in retrospect some of our patients probably do more fit Stickler's description, 6 but this should not divert the reader from the knowledge that pa tients with severe myopia and a malig nant form of perivascular and equatorial lattice degeneration associated with an optically empty vitreous cavity and mem brane formation may benefit from vitrectomy if scleral buckling fails. As has Dr. Judison in his letter, we wish to emphasize again the wide variability of hereditary disease. GARY C. B R O W N , W I L L I A M TASMAN,
Philadelphia,
M.D. M.D.
Pennsylvania
REFERENCES 1. Wagner, H.: Ein bisher unbekantes Erbleiden des Auges (Degeneratio hyaloideo-retinalis hereditaria), beobachtet im Kanton Zurich. Klin. Monatsbl. Augenheilkd. 100:840, 1938. 2. Hirose, T., Lee, K., and Schepens, C : Wagner's hereditary vitreo-retinal detachment. Arch. Ophthalmol. 89:176, 1973. 3. Frandsen, E.: Hereditary hyaloideo-retinal degeneration (Wagner) in a Danish family. Acta Ophthalmol. 44:233, 1966. 4. lansen, L.: Degeneratio hyaloideo-retinalis hereditaria. Ophthalmologica 144:458, 1962. 5. Van Balen, A. Th. M., and Falger, E. L. F.: Hereditary hyaloideo-retinal degeneration and palatoschisis. Arch. Ophthalmol. 83:152, 1970. 6. Stickler, G. C , Belau, P. G., Farrell, F. J., Jones, I. D., Pugh, D. G., Steinberg, A. G., and Ward, L. E.: Hereditary progressive arthro-ophthalmopathy. Mayo Clin. Proc. 40:433, 1965.
257
BOOK REVIEWS Ocular Pharmacology. By William H. Havener. St. Louis, C. V. Mosby Co., 1978. Hardcover, index, table of con tents, 377 black and white figures. $45 Ocular pharmacology has grown in leaps and bounds, especially during the past 12 years. This text of ocular pharma cology by Havener is now in its fourth edition. The first one appeared in 1966. This fourth edition contains comprehen sive references of the past 20 years, par ticularly with respect to the use of oph thalmic medications and the responses of eye diseases to such treatments. The au thor continues to review and classify with clarity. Prejudices that have developed because of his own considerable clinical experience do induce some bias into the presentation; however, at all times the author makes his preferences known. This is a monumental accumulation of data and references, well organized, easy to read, and understand. The contents are included under the same 38 different chapter headings as in the third edition. Each chapter contains some new infor mation and there has been a serious at tempt to keep all of the material up to date. This is impossible in any text. For example, this one has no significant men tion of the role of beta blocking agents on ocular functions particularly on intra ocular pressure or glaucoma. The section on drugs useful in fungus endophthalmitis is limited to a discussion of amphotericin. Although triflurthymidine is considered in herpetic ocular infec tions, there is no mention of the cycloquanines. These are not oversights. They simply demonstrate the difficulties and roadblocks encountered with publishing deadlines. Ocular pharmacology is not only a sci ence but also an art and Dr. Havener is thoughtful, articulate, and lucid in deal-
AMERICAN JOURNAL OF OPHTHALMOLOGY
agnosis is mandatory for appropriate treatment, genetic counseling, useful re ferral consultations, and provision of a reliable prognostication. What is now usually called vitreoretinal degeneration of Wagner is one of perhaps several enti ties clearly separable and distinct from the condition originally described by Wagner. Awareness of the genetic hetero geneity in patients with hyaloideoretinal degeneration will permit accumulation of the data necessary to permit assortment of these similar yet genetically separate enti ties. G. FRANK J U D I S O N ,
M.D.
Iowa City, Iowa REFERENCES 1. Wagner, H.: Ein bisher unbekantes Erbleiden des Auges (Degeneratio hyaloideo-retinalis hereditaria), beobachtet im Kanton Zurich. Klin. Monatsbl. Augenheilkd. 100:840, 1938. 2. Bohringer, H. R., Dieterle, P., and Landolt, E.: Zur Klinik und Pathologie der Degeneratio hvaloideo-retinalis hereditaria (Wagner). Ophthalrriol. 139:330, 1960. 3. Ricei, A.: Degenerescence Hyaloideo-retinienne de Wagner. Bull. Des. Soc. Ophtal. Franc. 1961. 4. Stickler, G. B., Belau, P. G. Farrell, F. J., Jones, J. D., Pugh, D. G., Steinberg, A. G., and Ward, L.E.: Hereditary progressive arthro-ophthalmopathy. Mayo Clin. Proc. 40:433, 1965. 5. Popkin, J. S., and Polomeno, R. C.: Stickler's svndrome (hereditary progressive arthro-ophthalmopathy). CM A J. 111:1071, 1974.
Reply Editor: Although nine of Wagner's original 13 patients had myopia of less than or equal to - 4 . 0 diopters, four, or approximately one third, had myopia ranging from - 5 . 2 5 to - 3 0 diopters in the more myopic eye. Wagner's paper states, " . . . without exception, all affected persons exhibited myopia, usually low grade, but some times more severe." Thus the presence of severe myopia in itself does not seem to exclude our patients and those of other
FEBRUARY, 1979
investigators 2,3 from being Wagner's vari ants. Descriptions of cataract are varied in Wagner's original article. Included are anterior and posterior cortical punctate and stellate opacities, subcapsular opaci ties, and diffuse homogenous nuclear tur bidity. One of our patients had a posterior subcapsular lens opacity, whereas two did not. Two of Wagner's juvenile pa tients (ages 5 and 6 years) had clear lenses, and cataracts were not usually prominent until adolescence. Certainly other au thors 2 - 4 have noted these opacities, which are frequently cortical, but may vary. Wagner did not originally describe the association of retinal detachment in his patients, a point we made in our article. Jansen, 4 though, noted the frequent oc currence of retinal detachment, often at an early age, in his patients with mild to moderate (although sometimes severe) myopia, cataracts, optically empty vitre ous with membranes, and peripheral and perivascular retinal pigmentation. His de scriptions are similar to those of Wagner and emphasize the association with reti nal detachment. Palatoschisis has been well noted in Wagner's variants, 2,5 as have hyperextensible joints and chondrodysplasia. 2 Micrognathia and glassoptosis in two pa tients with Wagner's disease and Pierre Robin syndrome have also been de scribed. 2 It was not our objective to dis cuss the systemic associations known to occur with Wagner variants but rather to suggest therapy for recurrent retinal de tachment in the disease. When one considers that most diseases involve a continuum along the spectrum of manifestations, it is not unreasonable to group the original Wagner patients and subsequently described patients. 2 - 5 Cer tain points of selected articles can usually be found to support disassoeiation, as well as association, of patient groups. Whether Wagner's disease and Stickler's
VOL. 87, NO. 2
BOOK REVIEWS
syndrome, described 27 years later at a time of greater medical sophistication, are distinctly separate clinical entities or variants of the same genetic entity is a difficult question to resolve. It is bold to say with certainty that they are clearly separable and distinct. In summary, whether one chooses to label the patients we have described as having Wagner's disease or Stickler's syn drome, both points of view can probably be well supported. Of more importance is the hazard of eponymic terminology where heredogenetic variation tends to occur. Indeed, in retrospect some of our patients probably do more fit Stickler's description, 6 but this should not divert the reader from the knowledge that pa tients with severe myopia and a malig nant form of perivascular and equatorial lattice degeneration associated with an optically empty vitreous cavity and mem brane formation may benefit from vitrectomy if scleral buckling fails. As has Dr. Judison in his letter, we wish to emphasize again the wide variability of hereditary disease. GARY C. B R O W N , W I L L I A M TASMAN,
Philadelphia,
M.D. M.D.
Pennsylvania
REFERENCES 1. Wagner, H.: Ein bisher unbekantes Erbleiden des Auges (Degeneratio hyaloideo-retinalis hereditaria), beobachtet im Kanton Zurich. Klin. Monatsbl. Augenheilkd. 100:840, 1938. 2. Hirose, T., Lee, K., and Schepens, C : Wagner's hereditary vitreo-retinal detachment. Arch. Ophthalmol. 89:176, 1973. 3. Frandsen, E.: Hereditary hyaloideo-retinal degeneration (Wagner) in a Danish family. Acta Ophthalmol. 44:233, 1966. 4. lansen, L.: Degeneratio hyaloideo-retinalis hereditaria. Ophthalmologica 144:458, 1962. 5. Van Balen, A. Th. M., and Falger, E. L. F.: Hereditary hyaloideo-retinal degeneration and palatoschisis. Arch. Ophthalmol. 83:152, 1970. 6. Stickler, G. C , Belau, P. G., Farrell, F. J., Jones, I. D., Pugh, D. G., Steinberg, A. G., and Ward, L. E.: Hereditary progressive arthro-ophthalmopathy. Mayo Clin. Proc. 40:433, 1965.
257
BOOK REVIEWS Ocular Pharmacology. By William H. Havener. St. Louis, C. V. Mosby Co., 1978. Hardcover, index, table of con tents, 377 black and white figures. $45 Ocular pharmacology has grown in leaps and bounds, especially during the past 12 years. This text of ocular pharma cology by Havener is now in its fourth edition. The first one appeared in 1966. This fourth edition contains comprehen sive references of the past 20 years, par ticularly with respect to the use of oph thalmic medications and the responses of eye diseases to such treatments. The au thor continues to review and classify with clarity. Prejudices that have developed because of his own considerable clinical experience do induce some bias into the presentation; however, at all times the author makes his preferences known. This is a monumental accumulation of data and references, well organized, easy to read, and understand. The contents are included under the same 38 different chapter headings as in the third edition. Each chapter contains some new infor mation and there has been a serious at tempt to keep all of the material up to date. This is impossible in any text. For example, this one has no significant men tion of the role of beta blocking agents on ocular functions particularly on intra ocular pressure or glaucoma. The section on drugs useful in fungus endophthalmitis is limited to a discussion of amphotericin. Although triflurthymidine is considered in herpetic ocular infec tions, there is no mention of the cycloquanines. These are not oversights. They simply demonstrate the difficulties and roadblocks encountered with publishing deadlines. Ocular pharmacology is not only a sci ence but also an art and Dr. Havener is thoughtful, articulate, and lucid in deal-