cures for retinitis pigmentosa

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AMERICAN JOURNAL OF OPHrrHALMOLOGY Published Monthly by the Ophthalmic Publishing Company EDITORIAL STAFF LAWRENCE T. POST, Editor PARK LEWIS 640 S. Kingshighway, Saint Louis 454 Franklin Building, Buffalo WILLIAM H. CRISP, Consulting Editor 530 Metropolitan Building, Denver C. S. O'BRIEN EDWARD JACKSON, Consulting Editor The State University of Iowa, College of Republic Building, Denver Medicine, Iowa City HANS BARKAN M. URIBE TRONCOSO Stanford University Hospital, San Francisco 350 West 85th Street, New York HARRY S. GRADLE 58 East Washington Street, Chicago JOHN M. WHEELER H. ROMMEL HILDRETH 635 West One Hundred Sixty-fifth Street, 824 Metropolitan Building, Saint Louis New York EMMA S. Buss, Manuscript Editor 4907 Maryland Avenue, Saint Louis Directors: EDWARD JACKSON, President, LAWRENCE T. POST, Vice-President, DR.F. E. WOOD- RUFF, Secretary and Treasurer, WILLIAM L. BENEDICT, WILLIAM H. CRISP, CHARLES P. SMALL. Address original papers, other scientific communications including correspondence, also books for review and reports of society proceedings to Dr. Lawrence T. Post, 640 S. Kingsltighway, Saint Louis. Exchange copies of medical journals should be sent to Dr. William H. Crisp, 530 Metropolitan Building, Denver. Subscriptions, applications for single copies, notices of change of address, and communications with reference to advertising should be addressed to the Manager of Subscriptions and Advertis ing, 640 S. Kingshighway, Saint Louis. Copy of advertisements must be sent to the manager by the fifteenth of the month preceding its appearance. Author's proofs should be corrected and returned within forty-eight hours to the manuscript editor. Twenty-five reprints of each article will be supplied to the author without charge. Addi- tional reprints may be obtained from the printer, the George Banta Publishing Company, 450-458 Ahnaip Street, Menasha, Wisconsin, if ordered at the time proofs are returned. But reprints to contain colored plates must be ordered when the article is accepted. CURES FOR RETINITIS PIGMENTOSA What is the status of cervical sympa- thectomy as a treatment for retinitis pig- mentosa? Whenever, in the course of one or two generations, a great number of remedies have been proposed, tried, and abandoned one after the other as cures for a disease which has therefore come to be regarded as incurable, there is ample justification for skepticism as to the chances of suc- cess of any new therapeutic proposal. Above all is such skepticism justified with regard to a disorder which has been in- cluded in the group of defects to which Treacher Collins and others have applied the name "abiotrophy"; that is, defects whose development was predetermined by some failure in the hereditary genes. Karsch has recently prepared a critical review of attempts at therapeutic control of retinitis pigmentosa, as disclosed in the literature of ophthalmology between 1877 and 1935 (Zeitschrift fur Augen- heilkunde, 1936, volume 90, page 157). It is of interest to note that preparation of the review was apparently stimulated to some extent by the German Nationalist law providing for sterilization, in order to prevent transmission of hereditary dis- eases to future generations. The long list of remedies which have been resorted to for this purpose is classi- fied by Karsch under the headings of (1) general treatment, vascular measures; (2) special applications to the eye; (3) surgical treatment; (4) radiotherapy; 311

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Page 1: Cures for Retinitis Pigmentosa

AMERICAN JOURNAL OF OPHrrHALMOLOGYPublished Monthly by the Ophthalmic Publishing Company

EDITORIAL STAFF

LAWRENCE T. POST, Editor PARK LEWIS640 S. Kingshighway, Saint Louis 454 Franklin Building, Buffalo

WILLIAM H. CRISP, Consulting Editor530 Metropolitan Building, Denver C. S. O'BRIEN

EDWARD JACKSON, Consulting Editor The State University of Iowa, College ofRepublic Building, Denver Medicine, Iowa City

HANS BARKAN M. URIBE TRONCOSOStanford University Hospital, San Francisco 350 West 85th Street, New York

HARRY S. GRADLE58 East Washington Street, Chicago JOHN M. WHEELER

H. ROMMEL HILDRETH 635 West One Hundred Sixty-fifth Street,824 Metropolitan Building, Saint Louis New York

EMMA S. Buss, Manuscript Editor4907 Maryland Avenue, Saint Louis

Directors: EDWARD JACKSON, President, LAWRENCE T. POST, Vice-President, DR. F. E. WOOD­RUFF, Secretary and Treasurer, WILLIAM L. BENEDICT, WILLIAM H. CRISP, CHARLES P. SMALL.

Address original papers, other scientific communications including correspondence, also booksfor review and reports of society proceedings to Dr. Lawrence T. Post, 640 S. Kingsltighway,Saint Louis.

Exchange copies of medical journals should be sent to Dr. William H. Crisp, 530 MetropolitanBuilding, Denver.

Subscriptions, applications for single copies, notices of change of address, and communicationswith reference to advertising should be addressed to the Manager of Subscriptions and Advertis­ing, 640 S. Kingshighway, Saint Louis. Copy of advertisements must be sent to the manager bythe fifteenth of the month preceding its appearance.

Author's proofs should be corrected and returned within forty-eight hours to the manuscripteditor. Twenty-five reprints of each article will be supplied to the author without charge. Addi­tional reprints may be obtained from the printer, the George Banta Publishing Company, 450-458Ahnaip Street, Menasha, Wisconsin, if ordered at the time proofs are returned. But reprints tocontain colored plates must be ordered when the article is accepted.

CURES FOR RETINITISPIGMENTOSA

What is the status of cervical sympa­thectomy as a treatment for retinitis pig­mentosa?

Whenever, in the course of one or twogenerations, a great number of remedieshave been proposed, tried, and abandonedone after the other as cures for a diseasewhich has therefore come to be regardedas incurable, there is ample justificationfor skepticism as to the chances of suc­cess of any new therapeutic proposal.Above all is such skepticism justified withregard to a disorder which has been in­cluded in the group of defects to whichTreacher Collins and others have appliedthe name "abiotrophy"; that is, defectswhose development was predetermined

by some failure in the hereditary genes.Karsch has recently prepared a critical

review of attempts at therapeutic controlof retinitis pigmentosa, as disclosed inthe literature of ophthalmology between1877 and 1935 (Zeitschrift fur Augen­heilkunde, 1936, volume 90, page 157).It is of interest to note that preparationof the review was apparently stimulatedto some extent by the German Nationalistlaw providing for sterilization, in orderto prevent transmission of hereditary dis­eases to future generations.

The long list of remedies which havebeen resorted to for this purpose is classi­fied by Karsch under the headings of (1)general treatment, vascular measures;(2) special applications to the eye; (3)surgical treatment; (4) radiotherapy;

311

Page 2: Cures for Retinitis Pigmentosa

312 EDITORIALS

(5) hormone and vitamin treatments.Since narrowing of the retinal blood

vessels is a striking feature of the dis­ease, vasodilators have been widely em­ployed. Thus, Mellinger reported im­provement from sweating. Especially be­fore the Wassermann era antisyphiliticmeasures were credited with successfulresults. Strychnine, amyl nitrite, subcon­junctival injections of sodium chloride,and retrobulbar injections of atropine mayall be reckoned in the class of treatmentsaimed at improvement in retinal circu­lation. Mayou and Wheeler are men­tioned among those who used the Elliottrephine operation for the same purpose.

Sgrosso's claims of material improve­ment of visual acuity after Roentgenradiation have been supported by a num­ber of Japanese writers, especially Suga­numa, who thought he obtained improve­ment in eight out of fifteen cases whichwere systematically subjected to thistreatment.

In view of the unquestionable influenceof vitamins upon other conditions inwhich the dark adaptation of the retinais affected, it is by no means surprisingthat vitamin treatment should have beensuggested for retinitis pigmentosa. In aseries of cases Doyne administered rawretina of oxen and sheep. Clegg tried aglycerin emulsion of sheep retina, with­out result. Seggel combined injections ofstrychnine with the use of cod-liver oiland liver.

Starting from the observation thatretinitis pigmentosa is more frequent inmen than in women, whereas the pub­lished genealogies of retinitis pigmentosado not suggest that the disease is sex­linked, Wibaut believes that the femaleorganism is particularly rich in hormoneswhich have a favorable influence uponthe degenerative process. He, and otherswith him, have therefore employed ahormonic preparation (menformon). Butthis line of treatment has led to very few

claims of success.The assumption that retrmtrs pigmen­

tosa depends upon degeneration of theretinal vessels induced Royle to proposeresection of the sympathetic nerve trunk,in order to produce permanent dilatationof the vessels. 'In five cases, in which hedivided the sympathetic above the secondthoracic ganglion, he reported improve­ment of visual acuity and of the visualfield. In the six years which have elapsedsince Royle's preliminary report in theMedical Journal of Australia, this treat­ment has been extensively tried in variouscountries.

There has been some argument as towhich part of the sympathetic trunk isbest adapted to the purpose. Campbelland Meighen removed the superior cervi­cal ganglion. Magitot stripped the carotidartery of its periarterial plexus. Othershave done a cervicodorsal sympathec­tomy.

Takats and Gifford (Archives ofOphthalmology, 1935, volume 14, page441) take exception to Royle's methodon the grounds that mere section of thesympathetic trunk is rapidly followed byregeneration, that it denervates the upperextremity, and that the preganglionicfibers to the eye may escape division un­less the stellate ganglion is removed. Intheir eleven operations on six patients,they used cervicodorsal sympathectomyfour times and superior cervical sympa­thectomy seven times.

These authors point out that there hasbeen a good deal of discussion as to thepermanency of vasodilatation followingsympathectomy; and that vascular tonusis regained within a few weeks, althoughthe possibility of a permanently increasedsupply of blood to the part cannot beruled out. They evidently feel consid­erable doubt as to the success of this lineof treatment.

Several authors feel that there is littleprospect of success from sympathectomy

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EDITORIALS 313

in the late stages of retinitis pigmentosa.In the early stages of the disease, on theother hand, its development is usualyvery slow, and yet the visual conditionfluctuates appreciably from time to time.Young patients who still possess fairlygood vision, and who are honestly ad­vised as to the extreme uncertainty ofbeneficial results and also as to the un­pleasant disturbances which commonlyfollow sympathectomy, are hardly likelyto show enthusiasm for the experiment.Conscientious surgeons, too, may prop­erly feel a good deal of reluctance to ex­pose patients to these inconveniences inthe earlier stages.

It seems at least possible that some ofthe favorable results reported have de­pended partly upon an excess of credulityin the patient or even in the surgeon, andpartly upon natural fluctuations in thepatient's condition. It is a suggestive factthat after unilateral sympathectomy im­provement has been reported as to botheyes. One patient showed no increase invisual field or acuity, but "believed" hernight blindness had improved.

Walsh and Sloan (Archives of Oph­thalmology, 1935, volume 14, page 699)conclude that the merit of the operationis not yet proved, although they wouldnot discard the procedure until furthercases have been thoroughly studied.Karsch's review satisfies him that, inview of the unpleasant disturbances pro­duced, the method has not been suffi­ciently successful to encourage its con­tinuance or further development.

As a general principle, it would beunscientific to argue that failure of previ­ous experiments condemned trial of newmeasures. To what extent the hazardsassociated with further experimentationare justifiable is a problem for the surgi­cal conscience. In the presence of sub­stantial risks, it may be questionedwhether operations should be urged forthe mere purpose of adding to the sur-

geon's statistical material, especially whenthe evidence previously accumulated af­fords no significant prospect of success.

W. H. Crisp.

GRADUATE STUDY

The need for a physician to continueto study so long as he continues in prac­tice, is being more widely recognizedevery year. The Research Study Club ofLos Angeles met for its Sixth AnnualMid-Winter Course on Ophthalmologyand Otolaryngology, January 18th to29th; and the American Board of Oph­thalmology held an examination in theLos Angeles County Hospital, on Satur­day, January 23d. The registration for theCourse was over two hundred, and aboutthirty took the examination for the Cer­tificate of the Board.

This year the foreign guest-teacher wasFelix R. Nager, Professor of Otolaryn­gology in the Medical School of the Uni­versity of Zurich, Switzerland. He provedto be a good teacher, with a broad viewof his subject, of general pathology, andof the public responsibility of a medicalman, whatever branch of medicine hemay practice.

In ophthalmology the principal coursewas given by Dr. Meyer Wiener, of St.Louis. He gave about twenty lectures on"The surgery of the eye." Beginningwith the preparation of the patient, hediscussed the details of operations, fromthose for cataract to those on the lidsand lacrimal passages. He amply sus­tained his reputation as a skillful teacher.These courses were well iIIustrated,largely by lantern slides and drawings onan illuminated background.

There were also valuable lectures byDr. Frederick C. Cordes, Professor ofOphthalmology in the Medical School ofthe University of California, San Fran­cisco, on the "Histopathology of the eye"