Hereditary Reversion Pigmentation of the Eyelids with Heterochromia of the Iris

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Hereditary Reversion Pigmentation of the Eyelids with Heterochromia of the Iris 874 LEE MASTEN FRANCIS enucleated. The following report on the Other cells were round with hyper- specimen was submitted from the New chromatic nuclei; while scattered thruout York State Institute for the Study of the tumor were large deeply staining Malignant Diseases: cells with one or two nuclei but free from The gross appearance of a cross sec- pigment. There were apparently two tion of the eye shows a tumor lying in types of pigmented cells, the one a large the lower temporal quadrant of the eye, irregular cell with long protoplasmic evidently springing from the choroid processes densely filled with fine yellow- near the margin of the optic disc. This ish granules, evidently chromatophores. tumor measured 15x10 mm. and was The other type of pigmented cell was a slightly nodular irregular ovoid tumor. evidently a tumor cell of the type men- The surface appeared smooth, was dark tioned above but containing fewer gran- gray in color and was of a soft consist- ules than the chromatophores. Thruout ency. The retina was markedly detached the tumor were small areas of hemor- and contained a clear serous fluid. Cross rhage and between the cells could be section of the tumor mass showed a demonstrated here and there, free pig- deeply pigmented homogeneous surface. ment granules. Microscopically, the tumor varied as From this picture, we would make a to the cellular constituents. There were diagnosis of malignant melanoma, fre- areas showing many pigment cells and quently called melanosarcoma, but by other areas almost free from the same. some authorities considered as melanotic The tumor was very vascular showing many fine capillaries around which in carcinoma. many places elongated cells arranged This case is reported because of co- themselves. Other areas showed cords existing and independent choroidal of epithelial like cells. While in some changes; the gradually decreasing intra- places there was distinct stroma, other ocular tension; the persistent absence of areas seemed almost free of connective frequent collateral symptoms of intra- tissue. ocular tumor, hypertension and engorge- Three types of tumor cells could be ment of the anterior ciliary veins; and differentiated. The most common was a the consequent confusion in making a large spindle shape cell, having a large differential diagnosis between exudate, vesicular nucleus with a single nucleus. tuberculoma and neoplasm. HEREDITARY REVERSION PIGMENTATION OF THE EYELIDS WITH HETEROCHROMIA OF THE IRIS. HARRY VANDERBILT WURDEMANN, M.D., F.A.C.S., SEATTLE, WASHINGTON. This is the report of a case of anomalous pigmentation of the skin and iris. The illustration is not a photograph of the patient but is drawn upon a photograph of another person to show the location. In January, 1911, a girl of sixteen an ancestry of English and Irish blood. years, came to me for examination into Indeed, they have traced their family the causes of eye strain. No particular trees back for generations. cause was found and this disappeared The girl was a typical brunette with after following hygienic advice. brown eyes of even color, but my notes The father is a well known business of that time, show pigmentation of the man and has always been accepted as a somewhat bluish sclera of the left. It pureblood white, tho, to the ethnologist, was a shock to me to note, in the child traces of Ethiopian ancestry are apparent of a friend, the evidences of a throw- in his somewhat kinky hair and rugged back, as her features were distinctly cast of countenance. Yet, this particular negroid, her hair kinky and there was no family claims to be pure American with meniscus of the finger nails; the latter PIGMENTATION OF LIDS WITH HETEROCHROMIA 875 well known to the Southerners of the old to those sometimes seen in old negroes slavery days and a sure sign of negro and more particularly in half castes with blood. The father has this, too. speckled skins. The fundi of both eyes In June, 1920, comes the case again, is nonpigmented, but of the brunette now a married woman with two children, type. A peculiar bluish black discol- on account of eye strain and questions oration of the eyelids on the left side, me as to the cause of the pigmentation which extends above the temple on to the of the eyelids, and as to why her eyes are forehead, looks just like a "black eye," of different color. Glasses were pre- but there is no history of injury and the scribed for low grade astigmatism. coloration is on the Malpighian layer of Interest centers in the general change the skin, showing thru- the translucent of her countenance and the development outer layers. Questioning of the patient Diagram showing distribution of pigmentation of Wiirdemann's of hereditary reversion. of onesided pigmentation limited to the elicits evident authentic information that left eye and surroundings. Her hair is there are no other discolored areas on her no longer kinky, but is wavy—perhaps body, a part of which was submitted for the result of much endeavor by the hair- examination. Altho she has had two dresser. A sample obtained, shows that children, the mammillary ring around it is oval on section. Her countenance the nipple is not at all pigmented. is of the European type, but examination For obvious reasons, this young wo- of her fingers shows no meniscus of the nails. Her two children have the men- man's photograph was not obtained; in iscus present. fact, a photograph or even a wash draw- The right iris is light brown in color ing would not show the appearances, sat- and has distinct markings, while that of isfactorily. (The diagrammatic represen- the left eye, is a very dark seal brown tation herewith illustrated shows the with no distinct markings. The right area that is implicated.) sclera is white and the conjunctiva is COMMENTS. This case is not lentigo normal, while the sclera of the left eye or freckles; it is not chloasma or liver is bluish, blotched with pigment and the spots, for the shade of color is different conjunctiva has pigment patches similar and is localized. There is no other dis- 876 H. V. WuRDEMANN ease and no history; in fact, the family Heterochromia iridis is not so uncom- history has been investigated and negro mon, altho it constituted only about 0.2% blood is denied. Yet the ethnologic as- of cases at the Zurich Clinic (Lutz). pect is such, that the diagnosis of a color For discussion of pigmentation of the reversion from an infinitesimal amount conjunctiva and of the iris, I refer to of negrism, is apparent to the educated article on "Congenital Anomalies of eye. the Eye," by William Frederick Hardy, This case is an evidence of the Haeckel Volume 4, American Encyclopedia of and Neo-Lamarckian law, a manifesta- Ophthalmology, and to his references, all tion of the inheritance of characteristics of which deal with these conditions in from forebears, causing variation of disease, rather than from an ethnologic type, rather than an acquired anomaly. aspect. ORBITAL, EPIDURAL AND BRAIN ABSCESS. AUTOPSY AND MICROSCOPIC STUDIES. EUGENE M. BLAKE, M.D., From the Ophthalmological Department, Yale Univ., School of Medicine. NEW HAVEN, CONN. This is the report of a case which illustrates the danger which may attend acute disease first manifested by ocular symptoms. It was a mixed streptococcus and staphy- loccocus infection apparently starting with coryza. The patient, a white male, aged 37 was much swollen. The headache re- years, was admitted to the-New Haven mained sharply localized. He had some Hospital on June 23, 1920, on my service, nausea but no vomiting. complaining of a swollen and painful The physical examination showed the right eye. patient apparently in some pain. The left The family history was unimportant. eye was normal. The right upper lid . The past history showed he had never was swollen, red, and could not be been ill in bed, but had had occasional opened. There was marked edema ex- colds. He had gonorrhea fifteen years tending below the ramus of the right before. A few days before the onset of mandible. Induration and reddening were this illness he had a coryza which per- present on the forehead and temporal sisted. region. There was a slight thin dis- On June 17 the patient first noticed a charge from the eye and marked chem- frontal headache, which was sudden, se- osis. The cornea was clear; the pupil vere, and soon localized over the right was small and reacted slightly to light. eye. It became rapidly worse and about The movements were present but limited. two and one-half hours later he suddenly The fundus showed a white disc and became blind in the right eye. After dilated veins. The pharynx was injected five minutes vision partially returned, ev- and there was a marked discharge from erything appearing dark and blurred; this the nasopharynx. The teeth were carious impairment was present at time of ad- and pyorrhea was present. The general mission to hospital. The following morn- physical examination was negative except ing the right eye was slightly swollen for the urine which showed albumin, and the pain still more severe. On the granular casts, red and white blood cells. 20th he had a chill, followed by fever His blood urea was 22 and nitrogen 44. and sweating. From that time be became The white blood count on the 23rd was rapidly worse, and on the 23rd, entered 20,800 and on the 28th was 26,000. the hospital. On the 24th he had a chill June 24th under local anesthesia, I lasting fifteen minutes.
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