Congenital Clouding of the Cornea 
Affecting Two Sisters. 
BY 
ROBERT L. RANDOLPH, M. D., 
Assistant Surgeon to the Presbyterian Eye and Ear Charity Hospital, Baltimore, Aid. 
FROM 
THE AMERICAN JOURNAL OF THE MEDICAL SCIENCES, 
December, 1888. Extracted from the 
American Journal of the Medical Sciences for December, 1888. 
CONGENITAL CLOUDING OF THE CORNEA AFFECTING 
TWO SISTERS. 
By Robert L. Randolph, M.D., 
ASSISTANT SURGEON TO THE PRESBYTERIAN EYE AND EAR CHARITY HOSPITAL, BALTIMORE, MD. 
This condition is of such rare occurrence that both cases seem to be 
worthy of record. 
Case I.-K. M., four years old. Blue irides. A bluish haze of both 
corneas. Some nystagmus. Slight conjunctival injection. The cloud- 
ing appears to involve the epithelial layers of the cornea, together with 
the upper layers of the substantia propria. Pupils react perfectly. Can 
discern large objects at a short distance. Both eyes of normal size. 
The child prefers shady places and avoids the bright light of the sun. 
Bad teeth, but not the typical Hutchinson's teeth. Very profuse secre- 
tion of saliva. Excoriation on upper lip from a long existing running 
at the nose. 
Head well developed, and intellect in keeping with her age. The skin 
smooth and white. The limbs straight and well formed. Has a good 
appetite, rosy cheeks, and runs all about. The family physician tells 
me that at birth the corneae were almost perfectly blue, and since her 
birth he has noticed that the clouding has grown less and less, and it is 
only within the past year that the irides could be seen. The epithelial 
layers of the cornese have a granular appearance and lack the usual 
lustre. The fundus is not to be seen. 
Case II.-Jeannette M., ten years old; sister of the former. Well 
developed and bright. Blue irides. Her mother says that at birth her 
eyes were exactly like those of her sister. Dr. Cromwell, the family 
physician, has observed since he knew the family-now six years-that 
the corneie have been gradually clearing up. There is present the same 
distinct bluish haze as in the case of the younger sister, but to a much 
less degree. The corneae have not the lustre peculiar to the healthy eye, 
but still they lack the granular appearance seen in Case I. There is no 
nystagmus. The child has suffered off and on during her entire life 
with coryza, the excoriated upper lip giving evidence to this fact. 
Teeth very unsound, but not as much affected as in Case I. Like her 
sister, she dislikes bright, sunny days. It is possible to see the fundus, 
but not distinctly. It appears as though seen through a cloud. She 
uses her eyes for near work, and reads with considerable ease. The 
clouding, as is the case with her sister, pervades the superficial layers 
of the cornese, and is homogeneous throughout, except in the case of the 
left eye, in which the returning transparency seems to be more marked at 
the upper border of the cornea). 2 
RANDOLPH, CONGENITAL CLOUDING OF CORNEA. 
The slight photophobia present in both cases was the only annoying 
subjective symptom. Whether this latter-as is usually the case in 
corneal affections-is a reflex disturbance, having its origin in ciliary 
irritation, I am unable to say. It could have been due to rays of light 
playing upon a retina not yet accustomed to bright light, a retina which 
has always been shaded by what the Germans call a darkening-Ver- 
dunkelung-of the cornea. And this photophobia was less marked in 
the older case, seeming to indicate that with the fading away of the 
corneal cloud the retina was gradually accustoming itself to the increased 
supply of light as well as tojdie brighter quality of the rays. 
I questioned the mother clewidy, but could draw out no specific history. 
The father had become bald quite early in life. The mother had had 
eight children. At the birth of the younger of these two sisters she had 
experienced a great nervous shock, which I do not regard as signifying 
anything pertinent. After Case I. the mother was delivered of one more 
child, which died in convulsions six weeks after birth. 
I should call the affection then a diffuse interstitial keratitis, sluggish in 
character as it frequently is, indeed almost passive, beginning in the 
latter part of pregnancy, and gradually disappearing with the further 
growth and constitutional development of the child. 
As I have said, I was unable to gather any information from the 
mother which would indicate a specific origin, but the whole aspect of 
the disease, its rarity, and its occurrence in sisters, seem to point to some 
single and definite cause, and that was most probably an hereditary 
taint. 
The subject of interstitial keratitis, particularly from the point of view 
of its relations to inherited syphilis, has been the theme of many discus- 
sions, and notable among these latter stands out the work of Mr. Jonathan 
Hutchinson. In his Clinical Memoir,1 Mr. Hutchinson takes the ground 
that this form of keratitis is the effect of inherited syphilis. 
He draws his conclusions from the observation of one hundred and 
two cases of interstitial keratitis. The point in the diagnosis upon 
which he lays most particular stress, as indicating inherited syphilis, is 
the condition of the teeth. " The subjects of this form of corneal 
inflammation," he says, " almost invariably have their upper central 
incisor-teeth of the permanent set dwarfed and notched in a peculiar 
and characteristic manner," the condition commonly known as the 
" Hutchinson teeth." He has never seen a typical form of interstitial 
keratitis in which the teeth were of normal size and shape. 
Parinaud2 regards the miscarriages of the mother, the number of 
deaths among the brothers and sisters as the important factors which 
1 Syphilitic Diseases of the Eye and Ear. A clinical memoir by Jonathan Hutchinson, F.R.C.S. 
London, 1863. 
2 Arch. gen. de Med., Nov. 1883. RANDOLPH, CONGENITAL CLOUDING OF CORNEA. 
3 
should lead us to attribute a case of interstitial keratitis to inherited 
syphilis. In twenty-three out of thirty-two cases he finds these two 
points well marked. He thinks that diffuse interstitial keratitis is a 
manifestation of syphilis attenuee in the parents. In conjunction with the 
disease he has frequently found changes in the teeth. These changes, 
however, may accompany other affections. 
The majority of ophthalmic surgeons of the present day, I think, 
attribute the disease to hereditary syphilis,1 though this theory has been 
strongly opposed by Panas, Buffe, Mooren, and others. Panas doubts 
very seriously the syphilitic origin of the keratitis. " The abnormal 
configuration of the teeth," he says, " is hot always constant, and when 
it does exist it recalls exactly what we see in rachitis." Another point 
which, he thiuks, strengthens the idea that it has a common cause with 
rachitis, is that mercurials avail but little in its treatment, and that 
iodide of potash gives the best results. He suggests the name keratite 
cachectique diffuse instead of keratite heredito-syphilitique. Neither 
Hutchinson nor Panas alludes in his discussion to a congenital form 
of interstitial keratitis; in short, to cases similar to the ones now reported 
by myself. Hutchinson2 asserts that he has never witnessed the occur- 
rence of interstitial keratitis earlier than at the age of two years. The 
youngest case reported by Panas3 in his remarks was of a boy twelve 
years old. 
It is undoubtedly true that in a large proportion of cases the disease 
is developed between the ages of eight and fifteen. 
Although the cases reported by me were congenital, I do not regard 
the disease in itself as differing in one whit from that form of keratitis 
which has been fruitful of so much discussion, and to which I have just 
alluded. The outbreak, however, of the inflammation in utero, and the 
exceeding rarity of such an occurrence would appear to justify me in 
assigning these two cases, and like ones, to a separate and distinct branch 
of the subject. It behooves me, then, to touch more particularly upon 
the congenital forms of interstitial keratitis. 
The following description of the affection was given eighty years ago 
by a French writer:4 
"The child comes into the world with a peculiar form of blindness, due to 
the darkening of the transparent cornea. This latter is of a dull blue color, 
and thicker than in the normal state. This trouble appears to be produced 
by a relaxation of the tissue of the transparent cornea by the presence of a 
lymphatic humor which is absorbed after birth, most often without medical 
attention, the cornea becoming transparent at the end of a few months. In 
such a case the transparency appears first on the outer edge of the cornea, 
then in a circular direction around its border, and so, from place to place, 
until the entire cornea has assumed its natural color." 
1 Gaz. des Hop., 1871, Nos. 139, 140, 142. 2 Clinical Memoir, etc., p. 116. 
3 Gaz. des Hop., No. 142. 
4 Mayor: Essai sur quelque maladies congfenitales des yeux. Une these inaugurale. Montpelier, 
1808. 4 
RANDOLPH, CONGENITAL CLOUDING OF CORNEA. 
The first cases reported were by Wardrop, in 1739, and by Klinkosh, 
in 1766. In 1790 there appeared a very interesting report of three 
cases by Mr. Farar.1 The children were sisters, and they were born 
with corner so opaque that the irides could not be seen. The opacities 
gradually disappeared without treatment. In the first and second cases 
the recovery was complete in ten months after birth. In the third case 
the clouding had markedly diminished at the end of the first year. At 
this point he lost sight of the case. 
In 1841 Mr. S. Crompton,2 of Manchester, reported two cases. The 
children were brothers. One of the cases seems to have been of doubt- 
ful origin, purulent ophthalmia might have been the cause. There was 
no doubt about the younger one. For a history of the older boy the 
author says he relied very largely upon the testimony of the mother. 
In the same year I find two cases reported by Mr. R. Middlemore,3 of 
Birmingham. When first seen the cornese were so opaque that the 
irides were not visible. The cloudings finally disappeared, leaving the 
corneae wholly transparent. 
In 1855 a most interesting history of this subject was given by Dr. 
Fronmuller,4 in his monograph on "Congenital Cloudings of the Cornea." 
According to Fronmuller, the highest grade of the affection is scleroph- 
thalmus, which is an arrest in the development of the eye, taking place 
in the first or second months of pregnancy, at the time when there is no 
difference between the cornea and the sclera, when the former is thick, 
flat, leucomatous. There is, at this stage, no trace of the anterior 
chamber, and the iris lies immediately under the cornea. With this 
malformation we necessarily have microphthalmus. The peripheral 
cloudings generally have their existence in the third and fourth months 
of pregnancy, at the time when the watery elements and the anterior 
chamber form, and when the cornea begins to elevate itself and the 
lamellar formation is in process of growth. The other congenital cor- 
neal cloudings date from the later months of pregnancy. He divides 
the whole subject into two classes: First, congenital leucomatous opacities, 
in which the lamellar structure of the cornea is absent and there is an 
arrest in development, the eye being always reduced in size; the prog- 
nosis is absolutely bad. Second, congenital cloudy opacities, having their 
seat immediately under the external epithelium of the cornea, the latter 
preserving its normal texture; the prognosis here is favorable. 
The only case that I can find reported in the American medical 
journals is that by Dr. Bethune,5 in 1870. The patient was twenty-five 
1 An Account of a Very Uncommon Blindness in the Eyes of Newly Born Children. Medical Com- 
munications, vol. ii , London, 1790. 
2 Medical Gazette, vol. xxvii., London, 1841. 
3 Medical Gazette, vol. xxviii., London, 1841. 
4 Ueber die angeborenen Hornhautverdunklungen. Vierteljahrschrift fiir die prak. Heilkunde, 
B. xlv. S. 57-70. 
6 Boston Medical and Surgical Journal, vol. v., 1870. RANDOLPH, CONGENITAL CLOUDING OF CORNEA. 
5 
years old. The mother had noticed at birth an opacity the size of 
a pin's head, and instead of disappearing it had progressively increased 
in size. This cannot, though, be regarded as a perfectly fair and typical 
case, for too many important points in the history depended for their 
existence simply upon the mother's recollection of what the condition 
was twenty-five years before. It was quite possible that this opacity 
had its origin in purulent ophthalmia. 
The most complete history of the subject I find is that by Hubert.1 
" It may be due," he says, " to an arrest in development, or possibly the 
clearing up of the cornea goes on unusually slowly, and when delivery 
comes it is not yet transparent, or it may be the cornea underwent during 
intra-uterine life an inflammation the traces of which at birth had not 
entirely disappeared." He refers to the fact, however, that there is only 
one case on record in which the condition of the eye was due to a clearly 
defined intra-uterine disease. This case, he says, occurred in the practice 
of M. Panas, and is that of a woman twenty-five years old. The right 
eye was perfect in every respect. The left eye was less than half the 
normal size, and had all the muscular movements except abduction, 
which was rather imperfect. Of course, there was no light-perception. 
The patient affirmed that she was born with the eye just as small as it 
was then, and, to confirm her statement, the conformation of the orbit 
showed an arrest in development in proportion to the duration of the 
condition. The mother had gone through with smallpox during preg- 
nancy, and the patient was born with marks of the disease on her body. 
There were still clearly defined scars on her breasts. Syphilis was abso- 
lutely excluded. Here, then, we have the eye disease due to the variola 
The essay comprises a treatise on the embryology of the cornea, its 
normal histology, and the pathological changes seen in congenital opaci- 
ties. He inclines to Hutchinson's view as to its origin. 
In 1880 M. Leclere,2 in an article on congenital opacities of the cornea, 
reported seven cases in addition to the ones just quoted in this article. 
He concludes that such affections are the results of intra-uterine inflam- 
mation. In 1883 Couzon3 reports a case occurring in the practice of 
Prof. Parinaud. The infant was ten days old when first seen, and was 
born with opaque cornese. The hitter were the seat of an interstitial 
keratitis, characterized by a diffuse infiltration which occupied the 
cornea in its whole extent. The cornea had a pale bluish tint. The 
opacity hid the iris completely from view. Totally blind. No peri- 
corneal injection. History of congenital syphilis. The child was put 
1 Etude sur le development de la cornee et sur les opacites congenitales de cette membrane. These, 
Paris, 1876. 
2 Des opacitfis congenitales de la cornfie. Paris, These, 1880. 
8 Contribution a 1'etude de la keratite interstitielle dans la syphilis hereditaire et dans la syphilis 
acquise. Ibid., 1883. 6 
RANDOLPH, CONGENITAL CLOUDING OF CORNEA. 
on iod. potash, and in three months the clouding on the right cornea 
had nearly disappeared, that on the left cornea had diminished in intensity. 
From the preceding it will be seen that the weight of evidence seems 
to be in favor of the syphilitic theory. If, then, hereditary syphilis be 
the cause of that form of keratitis most often met with in children 
between the ages of eight and fifteen, there would be nothing in conflict 
with such an idea to suppose that this cause can and does sometimes 
exert its activity at a much earlier stage in the life of the child. 
There is, however, another point of view, from which many scientific 
observers have regarded such cases. Hubert, in his essay, though 
inclining to the Hutchinson theory, suggests the possibility of such 
corneal cloudings being due to retarded development. Fromniiller also 
touches upon this point. One of the strongest advocates of this theory 
is Steffan.1 The latter bases his conclusions upon the anatomical exam- 
ination of an eye in which the cornea was leucomatous everywhere, except 
at the periphery. He thinks that all such conditions are due to a retarded 
development on the part of the lens, the sac of the latter being at birth 
adherent to the posterior wall of the cornea, and thus the cornea was 
practically wrapped in darkness. Normally the cornea and lens have 
parted at birth. 
The following three cases, occurring in the practice of M. Jules Gerard,2 
a veterinary surgeon, would seem to speak in favor of this theory; 
certainly the syphilitic theory can be excluded. The colts had a common 
mother and father. The corneae in each case were completely opaque, 
and the affection had evidently commenced in utero. They were treated 
with the ointment of the red oxide of mercury, and they cleared up com- 
pletely. 
Another case is reported by Riickert,3 and is that of congenital cloud- 
ing of the cornea in a hog. Riickert admits the possibility of intra- 
uterine inflammations as the cause of such anomalies, but in his case 
he says that inflammation could be excluded. "Against its inflam- 
matory origin speaks the nature of the tissue in the opaque portion of 
the cornea. It was impossible to distinguish it from scleral tissue. It 
was rather a high process of organization which had taken place, for 
which the peculiar vascular conditions of the area concerned might offer 
a solution." 
It is evident from the brief review of the literature of the subject 
which I have given, that under the designation of congenital clouding 
of the cornea conditions etiologically and pathologically different have 
been described. It seems plain that cases in which the cornea presents 
' Steffan : Beitrag zur Erkliirung angeborener Anomalien der Hornhaut. Monatabliitter fiir Augen- 
heilkunde, July and August, 1867, S. 209-217. 
2 Keratite du foetus de chevaline. Archives Medicales Beiges, xii., 1870. 
3 Riickert: Eiu Beitrag zur Lehre von den angeborenen Hornhauttriibungeu. Diss. Milnchen, 1885. RANDOLPH, CONGENITAL CLOUDING OF CORNEA. 
7 
a fibrous structure like that of the sclera, in which the lens has not 
separated from the cornea, in which there is microphthalmus, and in 
which there is no tendency to improvement of these abnormal conditions, 
should be separated from the cases in which the only pathological 
change in the eye is a superficial bluish cloudiness, which has a marked 
tendency to clear up after birth. The cases belonging to the first cate- 
gory we need not hesitate to refer to arrest or abnormalities of develop- 
ment. 
Furthermore, it is urged that, as we have undoubted evidence of 
arrested development in the first set of cases, cases such as I report may 
be regarded simply as the slighter grades of this abnormal development. 
In opposition to this view it is to be said that there are little analogy 
and not sufficient evidence of transitional forms between the cases of 
undoubted arrest of development, and the cases of simple diffuse 
clouding of the cornese, without other ocular changes. These latter 
cases of diffuse clouding of the corneae are indistinguishable in their 
general aspect from the well-known instances of diffuse interstitial kera- 
titis occurring in childhood, and it is reasonable to suppose that they 
depend upon similar pathological changes. Again, it will be remembered 
that physical defects, which clearly owe their existence to a want of 
completeness in the developmental process of the foetus, rarely, if ever, 
adjust themselves in the infant to the proper order of things. Consider 
the coloboma of the iris, of the choroid, of the lids, the persisting pupillary 
membrane, and hyaloid artery, all these indicate imperfect and unusual 
terminations to the process of development in particular regions of the 
eye. Such imperfections are permanent, they are not remedied under 
the changed conditions surrounding the subject after birth. And this 
is quite as true of other parts of the body as well as of the eye. 
I am inclined, therefore, to refer my cases of congenital corneal cloud- 
ing, and similar ones, to intra-uterine inflammation and not to arrest of 
development. 
It will be observed that in nearly every instance more than one 
member of the same family is afflicted, that relationship plays an im- 
portant role, relationship the result of a common father and mother. 
In other words, that the offspring were exposed to some specific con- 
dition or cause. Such troubles occur in all classes of a population, 
among the well-fed and under-fed, among the residents in the most 
healthy situations as well as those of the most crowded cities. These 
last facts would suggest other than a strumous origin ; they would, on 
the contrary, together with the other well-established symptoms which 
I have mentioned, speak in favor of the syphilitic origin of such 
affections. 
While this is the position which I am inclined to assume regarding 
the pathology and etiology of this affection, it must be admitted that 8 
RANDOLPH, CONGENITAL CLOUDING OF CORNEA. 
considerable caution should be exercised before reaching any positive 
conclusion on these disputed points. The fact that an apparently 
similar affection has been observed in the domestic animals is not easily 
reconcilable with the theory of its syphilitic origin. Undoubtedly, a 
conservative view would admit different causes as capable of producing 
this disease. I am, however, so much impressed with the resemblance 
between the congenital and the post-natal forms of diffuse interstitial 
keratitis and the syphilitic origin seems so well established for the latter, 
and has been demonstrated for a certain number of cases of the former, 
that for the present, at least, I prefer to regard the congenital and the 
post-natal forms as etiologically as well as pathologically the same. THE MONTHLY PUBLICATION OF 
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