ACROMEGALY, WITH OCULAR COMPLICA­TIONS.

By ARTHUR BENSON, M.A., UNlV. DUB., F.R.e.S.;Ophthalmic and Aural Surgeon to the City of Dublin Hospital;

Junior Surgeon to St. Mark's Ophthalmic Hospital.

[Read in the Section of Pathology, November 30, 1894.J

AOROMEGALY is a disease which, of recent years, hasattracted a good deal of attention, and yet about whichvery little is definitely known. I thought .that the notesof a case which I had under observation might not be,vithout some interest to this Section, as such cases are ofequal interest to the ophthalmologist, the phy~ician, andthe pathologist; and each case carefully noted may assistthe statistician in arriving at general conclusions :-

CASE.-M. C. H., aged thirty-eight; bachelor; farmer.Histo1~y.-Fatherdied at theage of eighty; mother died at the age

of fifty-five. Both had been strong, healthy people, and died fronlacute diseases. The patient has two brothers younger than himself,and one sister, older; one sister died at the age of twenty from" coldcaught at schooL" The living menlbers of family are all strongand healthy. His father was always a big, bony man, and hisbrothers also are big, strong, bony men, but light and active, notstout like the patient. He used to weigh twelve stoDe at. his best.".fwelve years ago, iri 1883, he hurt his left knee and leg by thefall of a cart of oats. This prevented him from taking hisusual exerci~e for a long time. He used to ride a great deal andbunt, but since 1883 he had to stop hunting, and gradually gotvery much heavier than before. His general health, however,seemed good, but he got a bit" lazier in himself," and slept at oddtimes during the day more than before, and also at night he sleptvery well. His appetite was good, and all the functions of thebody were regularly performed. He never suffered from headache,

By MR. ARTHUR B,ENSON. 343

but now and again had a sort of feeling as of blood in the head.He never had pain ln the eyes.

In January, 1892, he had an attack of influenza, Dot severe.After this he began to find that he required more light to enablehim to see clearly at night.

On June 8th, 1892, he consulted me on account of a failure ofsight in both eyes, because he found difficulty in c.ounting cattle inthe field, &c. He would count them differently each time and couldnever feel sure that he was right. He then stated that the defectivesight was first noticed about two or three years before, but that ithad got worse for the last six or eight months. He smoked twoor three ounces of twist tobacco in the week. His vision, was 660

in each eye, with an almost total central scotoma for colour, anddefective colour sense over the whole of the temporal half of e~ch

field of vision, the defect in the left eye being decidedly greaterthan in the right eye. 'fhe colour vision of the nasal halves wasfairly perfect. When first seen the central vision was only 660 andperipheral vision 168. After three weeks' nse of iodide of pota~sium

and cessation from smoking, the central vision improved to -~. Therewere perivascular white lines (thickening of tunica adventitia),along both veins and arteries on the disc, and for a short distanceon the retina, otherwise the fundus seerned normal unless for adoubtful paleness of the disc. He was then a large, heavy man,but I regarded him as a naturally bigot heavy man, and made the.diagnosis of toxic amblyopia, advised the stopping of tobacco, .andhe was to come back in a few weeks, which he did, as above stated,with almost perfect vision. He returned home, promising to abstainfrom smoking and return in a few months' time to report progress•.I regret that through stress of circumstances no chart of the fieldwas made at this time.

The next time that I saw him was on November 17th, 1894,i.e., after an interval of two years and five months. He statedthen that he had had perfect health and sight from the time that [saw him before till about two months ago; since then the sight hadrapidly failed. He had taken to smoking again, and partly blamedthat for his trouble-that is to say, that for two years and threemonths, while he had ab3tained from smoking, he had enjoyed"perfect sight" according to his own estimate. He was now adifferent-looking man; his face aud ha.nds in particular struck me,and his figure was bent, and he seemed to require a stick to swady

344 AcromegalJ', with Ocular Complications.

himself with. There was no abnormality discoverable in thyroidor thymus gland.

His face and head had grown large and heavy-looking. Hisnose was large, his under-jaw prognathous, and the ramus long, andthe mouth heavy-looking. The eyelids were puffy, and the eyesprominent, with slight strabismus. The ears were very large,especially the left one. His hands were large and " spade-shaped/'and the fingers round and sausage-like. His feet, also, were mostremarkable...looking, and the joints of ankles, wrists, and elbows,were very massive. His pose was quite characteristic. The backand shoulders were stooped, and the prominent chin alulost touchedhis chest. His speech was rather slow, but his intelligence seemedquite unimpaired.

He complained of little except what he called a "dumb pain"in the arms and legs; he had but little strength in legs and hands,and his grasp was very feeble for such a giant fist and arm. Hewas not sensitive to pain, and complained of feeling "dull inhimself."

As a young man, hunting, he found it bard to get gloves largeenough to fit him, and since then his hands have grown very muchlarger, and he has in every way increased in size. He used to beknown as a strong, active man, and very handsome. The changein the shape of his mouth is what most attracted his friends'attention.

His cranium has increased slightly. He used to wear a hat 6 ~ ;now he wears 7l. He has had to get his boots made larger, andhis legs have numerous large varicose veins, and he has thecharacteristic stoop of the shoulders. He is nlore disposed to sleepthan formerly, and his appetite is excessive. He has no feeling ofthirst, nor does he drink any fluid in excess.

His general health seems to be perfect, and he is free from everyother or~anic lesion. His urine is free from albumen and sugar andindican, but rich in urates and uric acid crystals.

The vision in each eye had again fallen to 10; the right eyebeing the better of the two. 'l'he discs were very similar inappearance to what they were in 1892, but more definitely atrophic,though the perivascular thickening was not increased.

November 20th, 1894.-10 consultation with Dr. HawtreyBenson, it was decided to put him on thyroid extract, one tabloideach day to begin with, and it was suggested that possibly pituitaryextract might be of use.

By MR. ARTHUR BENSON. 345

The next day the patient came to tell me that he found the lefteye was quite blind. On testing him this was found to be thecase-L. V. = bare perception of light. But there was no changein the ophthalmoscopic appearances.

In consequence of this sudden loss of sight in the left eye, Iasked my colleague, Dr. Story, to see him in consultation, and heagreed in the diagnosis of acromegaly, but advised to put him backfor a time on the iodide of potassium mixture, which had donehim, apparently, so much good in 1892. Subsequently I had theadvantage of 0onsultation with Dr. Fitzgerald and Mr. Swanzy.

The right field of vision shows a remarkable confi~uratjon.

l'here is a total central scotonla, combined with a semi-hemian­opsia of the superior temporal quadrant of the field, the inferiorteTnporal quadrant. being the most perceptive position of the wholeiield for white, though every part of the field has now lost itsperception of red and green.

The actual size of the hands can be judged from the accompany­ing measurements, which I took, and his feet ,,'ere very similarlyenlarged. H is feet are terribly squeezed by his boots, but he saJshe "doesn't easily feel pain," and his feet never bother him whenonce he has" crushed them into his boots." His teeth were, at the8ame time, examined by Dr. Arthur Baker, and a cast taken, whichI aID, through his kindness, enabled to show.

Measurelnents of Hands, q-c.

RIGHT. LEFT.

Circumference of wrist - - 9i inches ... 9 inchesDo. palm - - 12 "

12 "Do. thumb - 4

"... 4

"Do. first finger - 4"

... 4"

Do. second finger 4"

4"Do. third finger - 3~

"... S! "4

Do. fourth finger 3~ " ••• S! "Circumference of neck, 18 inches.

Do. of head from chin over vertex, 29 inches.Length of right ear, S! inches.

Do. left ear, 31 inches.Distance between centres of pupils, 76 mm.

On March 17th, 1893, he weighed 17st. 0 ~ lb., and he weighed17~t. 4lbs. on November 29th, 1894; and his brother thinks he isnot now as big as he was a year ago. He says himself that, but

346 A cron'legaly, 'tf)itl~ Ocular Complications.

for his eyes, he is as good a man as ever he was in his life, and' isin perfect health and spirits.

The left eye is almost totally blind, and the sight of the righteye has diminished to fingers at 1 metre. He has to be ledabout, and supports himself on a stick; but his naturally cheerfuldisposition prevents birn from desponding.

December 1st, 1894.-Right 'T. = fingers at O·50m• Left V. =doubtful perception of light. The pupils hardly act to light oraccommodation. There is slight divergent strabismus, and theeyes are proptosed a little, and the lids very full and flabby. Theears are very large, especially the right one, which measures 3iinches in length.

He has been takin~ tabloids of thyroid extract (Burroughs,Wellcome & Co.), two each day, between meals, ana, though theydo not seem in any way to disagree \vith him, bis sight is steadilyand rapidly deteriorating since he began using the tabloids. Onthe suggestion of Dr. James Little, I stopped the tabloids and puthim on fresh thyroid extract, 25 minims three times a week,prepared and sent weekly to him by Messrs. Brady & Martin,Newcastle~on~Tyne.

A fortnight later, December 15th, 1894, his sight had improvedin each eye up to 168 ?? ? And on January 22nd, 1895, sight wasalmost perfect again-in each eye it was ~ ??? and Ja. 1.

'Thus, within seven weeks, the sight of the right eye improvedfrom fingers at O·5m• to ~ ???, and the sight. of the left eye fromdoubtful perception of light to i ?? ? and Ja. 1. And this improve­rneIit seems to date from the taking of the fresh extract, as he wasgetting steadily worse all the time that he was taking the tabloids.

There is still a defect in the upper temporal quadrant of thefield in each eye. And an attempt was made on more than oneoccasion to demonstrate \Vernicke's hemianopic pupil reaction, butwith &nly uncer-tain results.

Two months later, March 29th, 1895, I again saw him. lIesaid that be felt ,~ awfully well," but that his left leg had a sort ofsoreness in "the hip muscles below and in front of the joint; thatleg, he said, had for years been" a sort of a wooden leg" on him.His appetite was splendid, and the-head, he said, was" wonderfullylight" and free from the" full sensation" which he felt before.

All through, his grasp had been very weak, especially in the lefthand. He considers that his wbole left side is weaker than hisright.

By MR. ARTHUR BENSON. 347

On November 29th, 1894, he weighed (naked) 178t. 4lbs. Onl\Iarch 29th, 1895, he weighed 16st. lIb. A loss in four monthsof 1st. BIbs.

His sight still remains almost perfect-R. V. = i slowly.L. V. = ~???? The discs are much as before-paler thannormal, with evidences of past perivasculitis.

Since writing the above I re.ceived a letter from thepatient's brother, which says-" I have to tell you of thedeath of my brother, l\lichael, which occurred on the 25thApril. He continued to go on very well under yourtreatment until he got. rheumatic fev,er,_ whic.h terminatedfatally at the end of a,week."

Dr. Crean, of Clonmel, "rho attended him in his last illness,kindly gave m.e the following information,. he writes-.

" I believe that influenza was the cause of Michael H '8

death. For some months before his last. illness he hadbeen making rapid progress. He recovered his sight; wasno longer drowsy~or apathetic, and attended his ordinar.vbusiness with an llnusual amount of energy. His face""vas not so swollen and expressionless, and the ceden1a(mucoid) of his extremities was decreasing day by day.In his case the thyroid. extract treatment appeared to workwonders. The' 18th· of April he "\vent to the TipperaryHunt Races in fair health and good spirits, ,and was seized,on his return, with a severe rigor, followed by high fever,pains in the limbs, and all the other\' usual symptoms ofinfluenza. The day befo~e·his death he was clear and

II

collected.. The fatal, termination,. in my opinion,' was inno measure due to tumour of the pituitary body."

The pathology of acromegaly seems still a matter ofsome doubt, but in a very large majority of cases thepituitary b.ody has been, on post morte'11t examination, foundto be enlarged, and its pressure on the chiasma accountsfor the ocular complications. The few cases where the

348 AC'r011legaly, 'lvitl" Ocular COl11.plicatio11.s.

pituitary body has been found apparentljT healthy may,perhaps, be explained otherwise.

If the enlargement of the pituitary body be a truehypertrophy, as in the case of many enlargements of thethyroid gland, then it would seem irrational to add furtherto the already excessive pituitary secretion; but if theenlargement be due to some disease of the gland whichdestroys its function, then it would seem rational to sup­plement the fluid which was insufficiently supplied to theorganism by pituitary extract obtained from other animals.So far, there are few recorded cases where pituitaryextract has been used, and, as far as I know, none whereits use gave anything like the same amount of benefitthat fresh thyroid extract gave to my case.

Into the literature of the subject I need not eI!.ter. 1,fydesire being to record a case rather than to exhaust thesubject.

The principal points of interest in the case seem to meto be-

(1.) The early appearance of a central Bcotoma for colours,and probably also hemianopia for colour.

(2.) The almost complete reC01UJry of vision which fol­lowed the use of iodide of potassium and the cessation oftobacco.

(3.) The 'return of visual troubles (after more than t"royears) when the use of tobacco was resumed.

(4.) 1'he rapid deterioration of vision, which occurredwhen the thyroid tabloids were first used.

(5.) -The rapid and continued improvement which followedthe use of the fresh thyroid extract.

(6.) And the many and peculiar changes which haveoccurred in the visual field.

By MR ARTHUR BENSON.

ACROMEGALY.

Clla1~t of v-ision of MICHAEL H--.

349

Date Right Vision Left Vision

-

1892June 8th - 66

0 (central), -/'8 (peripheral) 6~ (central), ft (peripheral)

29th - j! .fi.

" 6 6

1894Nov. 17th - 6'')0 -~60

[fectly)21st

" - 6~ Perception of light (imper-

"26th - if; (badly)

" "

"30th - Fingers at 1 metre

" "(badly)

Dec. 1st - Fingers at o-som. (doubtful)" "

"15th - _6_? ? fa??18 - •

1895Jan. 22nd u §. ? ? ? J'. 1 .§. ? ?? J'. 16 • •• a 6 • •• a

March 29th - ~ slowly !????

DR. BAKER exhibited a cast of the patient's mouth.MR. SWANZY expressed the gratitude the Section owed to Mr..

Benson for the exhibition of this most in teresting case. He con­curred as to the diagnosis and treatment of the disease. Hemi­anopia was one of the most constant ocular symptoms of thisdisease. It existed in this case first as colour blindness of theternporal side of each field, and afterwards as partial blindness ofthe temporal side of each field. Central scotoma was not a usualsymptom, but it is very probable that this was due to a toxicamblyopia caused by excessive tobacco smoking, and had nothingto do with the acromegaly. Dr. Cunningham had endeavoured toshow that the skeleton of M-Grath in 'Irinity College, Dublin,was that of a patient suffering from this di~ease. The pituitaryfo~sa was so large that you could easily put a walnut into it.Neighbouring bony structures were obliterated, and we must sup­po~e that the optic tracts and some of the ocular nerves werepressed upon. It is v~ry probable that his right eye was com-

350 Acromegaly, tvitlt Ocular· Complications.

pletely blind, and that the left half of the left visual field was ah~o

a blank. It is, moreover, very curious that in any acconnt wehave of the giRnt no mention is made of any defect of sight, sothe above conclusions must be more or less speculatory.

MR. BENSON said he. wished to state, in conclusion, that he hadthe patient photographed in variolls postures. The peculiar stoop,the large size of the thorax, and the small size of the buttocks ascompared ,,"ith the legs were well sho\vn.

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