CAUSES OF HETEROCHROMIA IRIDIS WITH SPECIAL REFER- ENCE TO PARALYSIS OF THE CERVICAL SYMPATHETIC. F. PHINIZY CALHOUN, M. D. ATLANTA, GA. This abstract of a candidate's thesis presented for membership in the American Ophthal- mological Society, includes the reports of cases, a general review of the literature of the sub- ject, the results of experiments, and histologic observations on the effect of extirpation of the cervical sympathetic in the rab'bit, the conclusions reached from the investigation, and a bib- liography. That curious condition which con- thinks that the word hetcrochromia sists in a difference in the pigmentation should apply to those cases in which of the two eyes, is regarded by the parts of the same iris have different casual observer as a play or caprice of colors. In those cases where a cycli- nature. This phenomenon has for cen- tis accompanies the iris decoloration, turies been noted, and was called hcte- Butler8 uses the term "heterochromic roglaucus by Aristotle1. One who cyclitis," but the "Chronic Cyclitis seriously studies the subject, is at once with Decoloration of the Iris" as de- impressed with the complexity of the scribed by Fuchs" undoubtedly gives a situation, and soon learns that nature more accurate description of the dis- plays a comparatively small part in its ease, notwithstanding its long title. causation. It is however only within The commonly accepted and most uni- a comparatively recent time that the versally used term Hetcrochromia Iri- pathologic aspect has been considered, dis exactly expresses and implies the and in this discussion I especially wish picture from its derivation (irtpoa to draw attention to that part played other, xpw/xa) color. Other newly by the cervical sympathetic. coined words only add disorder to an Many writers have given Hutchin- already chaotic classification of dis- son2 (1869) credit for first describing eases. a difference in the color of the irides, Dr. Willis H. Bocock, my friend and with which cataract was associated. I former professor of Greek at the Uni- by chance discovered that Lawrence'3 versity of Georgia, has suggested the (1853) described' irregularities in the word irideterochromia. "The o (as in color of the iris and cited two cases; in iridoplegia) is not used in composition one of slowly changing color, and an- before a vowel; therefore hid in the other of changed color of the iris with word above. Hetc.ro (as in hetero- cataract. He also quoted Wilde4 who chromia) loses the /j in composition, mentioned the fact that heredity plays therefore irideterochromia." an important part in many of these ab- While it would be advantageous to normalities. have the meaning so expressed in one word, its derivation would not be as NOMENCLATURE obvious as the term heterochromia iri- Various terms have been applied for dis, unless the reader possessed a this inequality in the color of the iri- working knowledge of Greek. des, such as heteroglaucus of the an- cients ; WeilK' uses the word heteroph- CLASSIFICATION thalmus; anisoiridochromia is men- Any exact classification of this con- tioned by Scalinci" and chromohetero- dition is almost an impossible task, and pia is suggested by Malgat7, who also mindful of its limitations I venture to 256 P. P. CALHOUN submit the following as a working ba- development of a pathologic lesion. sis of study: And one reason why more cases are Heredity. Sympathetic par- not so classified is that the observer, in- alysis. different to the causes of heredity, does Cyclitis and cata- { ract. not stress its influence, and also that Primary.. With [-Sympathetic par- too few of our patients (clinical pa- Acquired or J alysis. tients especially) know enough of their without T Cyclitis or Cala- is ract. ancestry. Injuries. Sidero- The history of cases I and II from [" sis bulbi. the same family (more fully described J Uveal infection— ] Myopia. under the heading of symphthetic pa- { I Glaucoma. ralysis) where the paralysis and lighter Strictly speaking, as Sym10 has iris were all on the same side, in a pointed out, there could be no such manner substantiates the fact that- he- thing as a congenital form of hetero- redity does play an important part. If chromia iridis, since it is about the the paralysis was the cause for the twelfth or sixteenth month that the iris decoloration it would be proper to child's iris ceases to be light and takes consider it as the disorder inherited. on color. Brown pigment has however Galezowski'1, Bistis12 and Scalinci" been noted to appear on the iris as report cases of sympathetic paralysis, early as six months. where one or more children in a fam- I am not in accord with Sym and ily had iris decolorations which were Scalinci who maintained that we inherited. But we should bear in mind should reserve the term heterochromia Priestley Smith's13 words that "hered- iridis for the instances in which there ity is not in any real sense a cause. It has not been any obvious pathologic may account for the presence of a dis- process in the eye, such as glaucoma order in an individual or family, but it or iridocyclitis. The term expresses tells us nothing as to its origin. In the condition regardless of the cause. some former generation there must I have recently seen a case in which have been a first departure from the there was a decoloration of the iris in normal." an adult negro, who at first glance pre- Osborne14 as early as 1849, drew at- sented evidences of a sympathetic pa- tention to the difference in color of the ralysis, namely a smaller pupil and a 10 irides of a person (that is they were narrower palpebral aperture than the irregularly marked), who had fifteen opposite eye. The case proved to be brothers and five sisters who all pos- the result of an injury received during sessed the same abnormality which childhood, and the presence of a for- was derived from the mother, whose eign body in the choroid could be three sisters and one brother had the demonstrated. The ptosis was trau- same which were again derived from matic, the myosis was due to posterior the mother's side. Gunn15 mentions adhesions, and the iris decoloration similar cases and classifies them as was probably a siderosis bulbi, or "piebald iris." caused from a low grade iridocyclitis. It has been stated many times that I can see no objection to classifying in congenital heterochromia iridis the this as one of heterochromia of the iris, color of one eye is inherited from the of secondary origin due to an old in- father, while the color of the other is jury (foreign body with uveitis). derived from the mother. In many in- stances this has been true, but the re- HEREDITY verse is as often the case, and even Heredity as a cause for heterochro- eyes of an entirely different color to mia iridis, has not received the same either parent have been observed. This consideration from modern writers as contradictory observation however from those of a generation ago. I am would not refute the hereditary theory firmly convinced that there are many of transmission, bearing in mind the such congenital cases without sign of principles of Mendel's Law. HETEROCHHOMIA IRIDIS 257 The family reported by Gossage10 is facialis) heterochromia iridis as a symp- the only recorded case beyond the sec- tom. Mayou18 in 1910 was probably ond generation that I find mentioned. the first to accurately describe these A detailed description and examination changes, although Homer (quoted by of the eye unfortunately is not given, Samclsohn20) as early as 1875 did altho the author states that " in draw attention, to a case in which a this family hetcrochromia of the iris light iris occurred on the same side tended to appear when one eye, always with a ptosis of sympathetic origin. In the left, was grayish blue in color, with all only thirty-three cases have been chestnut brown patches. Of the off- reported, ten of which were compli- springs of the affected members of the cated cither with cataract or a slow family eight were affected and twenty- uveitis. two free." The following authors reported: From a genetic point of view, Przi- 17 Galezowski", 2 cases, 1 complicated; bram's work on angora cats is inter- Horner's case reported by Samel- esting. He found that the union of an solur"; Dethleffseir1, 5 cases, 1 compli- cated ; Lutz22, 13 cases, 3 complicated; Hutchinson2, 1 case; Alexander and I I Lander"'2, 5 cases, 2 complicated; Bis- M o :!0 18 [ tis , 2 complicated cases; Mayou , 3 A F cases and Scalinci", 1 case. i_ From my own observation I can re- F o port four cases; the first two being of the same family with positive sym- d 4 A F i 4 F A <i toms of a sympathetic paralysis. Case i I III gave less prominent symptoms, and i i i i I case IV presented only slight evidences 6 ° M F F of any sympathetic disturbance. My Fig. 1. Gossan's case. M, affected male; F, af- reason for here reporting case V along fected female; O, sex undetermined. with the others, is that we may assume that the heterochromia was due to cer- asymmetrically colored animal having tain trophic disturbances from a com- one blue and one yellow eye, with a plete hcmiplegia. symmetrically colored animal having Properly it belongs under the ac- two blue or two yellow eyes, resulted quired classification, if the history is in the production of both asymmetri- correct.