REPORT OF A CASE OE AKROMEGALY: WITH BRIEF COMMENTS ON THIS DISORDER. BY CHARLES W. DULLES, M.D. Reprinted from the Transactions of the College of Physicians of Philadelphia, for 1892. A CASE OF ACROMEGALY. By CHARLES W. DULLES, M.D. [Read October 5, 1892.] The subject of acromegaly was brought before the College last January and May, when Dr. F. A. Packard read a paper on this subject and a disease that simulates it (osteo-arthro- pathie hypertropliiante pneumique—Marie), and Dr. Dercum exhibited two patients with acromegaly. Since that time, to confirm the commonly entertained opinion that an unusual occurrence is likely to be soon followed by others of the same sort, a case of acromegaly has unexpectedly presented itself to me. On July 7th, in the house of a Syrian client, I was asked to examine and treat a friend who was newly arrived from Con- stantinople. The following account describes what I found : A. P., born in Turkey in Asia, twenty-seven years of age, unmarried; height, five feet seven and a half inches ; weight, about 160 pounds; has a good family and personal history, having lost two brothers in infancy and having five now living and healthy, and both parents living. Six years ago he was in this country, doing business in Chicago. As the accompanying photograph (Fig. 1), taken at that time, shows, he was then a well-formed and healthy young man. After this he went to Constantinople to carry on the Oriental part of a business that consisted in buying rugs, etc., and send- ing them to this country to be sold. Owing to involvement in the affairs of those for whom he acted, he was accused of violating the laws of the Ottoman Empire, and on March 4, 1890, was cast into a Turkish prison, where he remained for about eighteen months—until August 27, 1891. On leaving the prison he had no business, and was under a cloud of distrust and depression until he left Turkey to come to America, where he arrived fifteen days before I saw him. 2 CHARLES W. DULLES, While he was in prison his hands and feet began to enlarge, so that every- body said that he was getting fat, and his shoes (sandals) became too small Fig. 1. for him. About six months ago, he says,1 he began to be oppressed with drowsiness, especially after meals, when he could hardly speak, but had to Fig. 2. lie down and take a nap. For about three months he has had profuse sweats. 1 The periods are not quite trustworthy because the patient does not seem to remember dates accurately. A CASE OF ACROMEGALY. 3 When I first saw him he presented an appearance of melancholy, and a face that at once suggested to my mind the condition described by Marie as “ acromegaly,” and what appeared in the patients exhibited to the College last May by Dr. Dercum. The second photograph (Fig. 2) brings out the peculiar, massive, leonine countenance, the broad forehead, the wide malar eminences, the large mouth, the broad lower jaw, the large chin, the thick lips, the transverse wrinkles of the forehead, which make up the facial picture of acromegaly, and which are in marked contrast to the face in the photograph taken six years ago. Figure 3 shows the feet. In addition to Fig. 3. what the photographs show, it may be stated that there is lateral projection of the rami of the jaw, which is also enlarged, especially in the transverse direction. The form of enlargement generally seen in acromegaly seems to be an increase in the perpendicular and antero posterior measurements, so that the teeth of the lower jaw overlap those of the upper, and the face is of an oval cast. In the present instance the lower jaw seems to be enlarged, in common with the whole of the bones of the face, except that its breadth is disproportionate. Standing behind the patient at a distance of a few feet, both rami are plainly seen extending behind the lines of the neck. This is not the case in normally formed individuals, in whom the rami of the jaws are not visible, or only barely so, when looked at from behind the subject. The wideness of the lower jaw is conspicuous on putting the fingers into the mouth and passing them between the upper maxilla and the ramus of the lower jaw, in which situation the coronoid process can be plainly felt quite outside of the superior maxilla. From the outside the articular process of the lower jaw can be seen and felt through the auditory meatus, sliding far 4 CHARLES W. DULLES, forward when the mouth is opened, and moving back as it is closed. The lateral enlargement of the lower jaw caused the lobes of the ears to protrude and the lower part of the ear (tragus and anti-tragus) to stand further from the middle line than the upper parts of the concha. The teeth of the lower jaw are somewhat separated, but they fall within the line of the upper teeth. The hands and feet are characteristic. The former are large, and have the “sausage-shaped” fingers described by several writers. The feet are very large, and have the fleshy pad along the outer side, which is spoken of as peculiar to this disorder. There is also a thick pad under the os calcis and somewhat in advance of it on the sole of the foot. A careful physical examination did not disclose any evidence of organic lesion of any of the thoracic or abdominal viscera. The urine presented no peculiarities on chemical and microscopic investigation. The eyes were examined by Dr. George E. de Schweinitz, whose friendly interest in the case was increased by the fact that he has already examined the eyes of other persons suffer- ing from acromegaly. Dr. de Schweinitz reports his findings as follows: ‘‘The optic disc of the right eye is irregularly oval, of normal color, having at its nasal side a slight crescent. The fibre-layer of the retina in the neighborhood of the optic papilla is slightly hazy. There is a faint general absorption of the pigment-epithelium. The central vessels are nor- mal in size and carry naturally-colored blood. “ The optic disc of the left eye is a vertical oval; its nasal side is bounded by a pigment-line and its temporal side by a sharply marked greenish border. Otherwise the conditions are the same as in the right eye. “ In the right eye there is compound myopic astigmatism; 1 D. of cor- neal astigmatism with its axis horizontal. In the left eye there is simple myopic astigmatism; 2.50 D. with its axis at 15. With correction of the refractive anomaly: 0. D.—2 31 cyl. ax. H. 6/ix. 0. S.—2.50 cyl. ax. 15 6/ix. “ The pupils are round, equal in size, and react to the changes of light and shade, convergence and accommodation. The pupillary reflex is more marked when a beam of light is thrown upon the right side of each retina than when thrown upon the left, but the hemiopic pupillary inaction (Wernicke’s symptom) is not present. “There is no paralysis of any external ocular muscle. The fusion power is good, and there is esophoria (insufficiency of the external recti) of 2 degrees. A CASE OF ACROMEGALY. 5 “ There is typical left lateral hemianopsia, with great contraction of the preserved field of vision, the contraction being the greater upon the right side. The dividing line between the dark field and the preserved field passes slightly in advance of the fixation-point. The color perception is normal in tha central area, and there is a normal sequence in the appreci- ation of the colors in the area of preserved vision. “ The interesting feature of this case, so far as the ocular symptoms are concerned, is the presence of left lateral hemianopsia—a hemianopsia, more- over, that is probably due to a lesion back of the primary optic centres, inasmuch as there is preservation of the function of the sensori-motor arc of the pupil and the absence of Wernicke’s symptom. The lesion would seem to be located in some portion of the visual tract posterior to the centres just named. Ordinarily in hemianopsia, when there is concentric restriction of the remaining half-fields, this is greatest in the eye opposite to the lesion. In the present instance precisely the contrary condition obtains, the greatest restriction being found upon the right side, while the character of the hemianopsia (left lateral) shows that the lesion that creates it must be upon the right side of the brain. Independent of an organic cerebral lesion, it is perfectly possible that a hemianopsia might be present under the influence of hysterical manifestations. A number of such cases have been reported. In several of the cases of recorded acromegaly, bi-temporal hemianopsia has been present. This, then, is peculiar in being an example of left lateral hemianopsia associated with this affection. It is quite possible that it is purely an association, and has nothing to do with the disease itself.” From this report it will be seen that the patient has a form of hemianopsia that is peculiar, in that it is homonymous, whereas in acromegaly the hemianopsia is usually bi-tem- poral. Taking the picture of acromegaly drawn by Marie, we find this man’s case to resemble it, in that there have been depres- sion of spirits, profuse sweating, intense thirst, voracious appe- tite, great lassitude and drowsiness, especially at and after meals, severe and protracted headaches and marked melancholy of disposition, together with the enlargement of hands, feet, and face that are considered to be peculiar to this curious ailment. Acromegaly is by no means as rare a disorder as might be supposed from the fact that only a few cases have come to the notice of medical men in this country. The often-quoted essay of Dr. Pierre Marie and of Dr. Souza-Leite (London, New 6 CHARLES W. DULLES, Sydenham Society, 1891) contains brief accounts ot forty-eight cases gathered from various authors, the earliest being that of the French surgeon Saucerotte, published 116 years ago (1776), and Dr. 0. T. Osborne, of New Haven, in an interesting re- port of a case he had studied {Am. Joarn. of the Med. Sciences, June, 1892), speaks of it as the eighth case reported in the United States. The reasons for considering this a special disorder, and the differences between it and osteitis deformans and leontiasis ossea, are fully discussed by Marie and his pupil Souza-Leite, and will, I think, satisfy any candid reader. According to Marie, the peculiarity of acromegaly consists in a marked enlargement of the bones and of the overlying tissues in the hands and feet, and also in the face, while the bones of the skull and of the trunk are usually unaffected. The enlarge- ment of the bones causes a typical appearance of the face and of the extremities. The visible changes seem in many cases to be the consequence of profound distress or depression, and they are accompanied by lethargy of mind or even melancholy, with drowsiness, headache, extreme thirst, and sweating. In a number of cases hemianopsia has been observed. This curious manifestation should be looked for in every case until enough cases have been investigated to establish the impor- tance to be attached to its presence as a symptom, and the exact form of it. As already stated, the case that I have de- scribed presents a form of hemianopsia which differs from that which has heretofore been described. The case differs also in the mode of enlargement of the lower jaw, and these very differences make it the more desirable, I think, to put the case on record ; for the literature of the subject is still very re- stricted, and it may be that with a larger material for study, there may be some modification of our ideas as to what is essential to the disorder. An interesting contribution to this literature is an essay1 supplied by Professor Mosler to that remarkable monument in 1 TJeber die sogenannte Akromegalie (Packyacrie). A CASE OF ACROMEGALY. 7 honor of Virchow’s semi-centennial celebration, the Interna- tionale Beitrdge zur wissenschaftlichen Medicin. The patient whose case I have described was sent by me to Dr. Packard and to Dr. de Schweinitz for examination, and would be before you to-night but that he wearied of treatment in a hospital, and left the city. The treatment to which the patient was subjected was directed chiefly to the digestive tract, which was much out of order. An obstinate constipation was broken up; a suspicion of lumbricoids (a very common affliction of Turks and Syrians) was followed up until it proved ungrounded, salol and phen- acetin were given for headache, and strychnine was given as a general and nerve tonic. During the month in which the man was under my care he improved in some respects; but the improvement was solely in regard to the state of his digestive apparatus and the state of his mind. At times he acquired a certain degree of cheerfulness, but this did not last long. On leaving the city for a short summer holiday I had the patient admitted to a hospital, and on my return I found that he had decided to leave it, and that he had also left the city. 4101 Walnut Street.