CONGENITAL TOTAL COLOUR-BLINDNESS

CONGENITAL TOTAL COLOUR-BLINDNESS

626 I the following formula for the vapour Gomenol use Myrtol aa .... :— 5’0 gramme. Chloretone Menthol __ Wa Thymol Camphor trit..... 01. Eu...

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626 I

the following formula for the vapour Gomenol

use

Myrtol

aa

....

:—

5’0 gramme.

Chloretone Menthol __

Wa Thymol Camphor trit..... 01. Eucalypt.....

0’5 10’0 35’0

" "

"

The whole of this quantity is used at a single filling. In this way we introduce into the pleural cavity not simple atmospheric air, but air that is saturated with strongly antiseptic drugs-that is to say we directly sterilise the diseased lung. Thus we accomplish two very important purposes : first, during the period of treatment, carried on for months, we

during

treatment the

detoxication,

and

accordingly

the return of the patients’ temperature to normal, sets in much earlier than with other methods. The bacilli, as shown by the Gaffky scale, disappear from the sputum much sooner and there was a considerable increase in weight. Medicothorax therefore eliminates, on the one hand, the commonest complication of artificial pneumothorax and, on the other, it leads to quicker healing of the diseased lung, and is a more intensive treatment than any other method. For these reasons I think it deserves a place in the modern treatment of

phthisis. REFERENCES 1. Schmidt-Giessel : Die 2. Franck, R. : Moderne

Lungentuberkulose, Leipzig, 1935. Therapie, Leipzig, 1936.

CONGENITAL TOTAL COLOUR-BLINDNESS BY ALLISTER M.

MACGILLIVRAY, M.D. St. And., D.O.M.S.

OPHTHALMIC SPECIALIST TO THE FIFE COUNTY COUNCIL ; LECTURER IN CLINICAL OPHTHALMOLOGY, UNIVERSITY OF ST. ANDREWS ; AND

A. SHEILA

MACLEOD, M.B. Edin., D.P.H.

AREA MEDICAL

The Medicothorax apparatus.-(A) Pneumothorax apparatus. (B) Flask containing the medicated vapour. (c) Flask containing the drug to be vaporised. (D) The electro-inhalator pump.

surround the

lungs

with

a

disinfectant

and, secondly, this disinfecting air-layer

medium,

into contact through the regular respiratory gas-exchange with the whole of the infected organ too. This is confirmed by the fact that the patient’s breath, after an induction by my method, in a few moments acquires the odour of the vapour. The new method adds to artificial pneumothorax two other very important advantages. As is generally known the present treatment causes an exudate in 80 per cent. of the cases.1 Even if the exudate generally runs an uncomplicated course and requires surgery only in exceptional cases, its occurrence is The first and most striking very inconvenient. result of the medico thorax treatment is the almost complete lack of this exudation. This alone is a proof that sterilisation in the pleural cavity actually takes place and that pleurisy is wholly avoidable. This single advantage alone should recommend the method, but we were also able to promote quicker healing. We could establish beyond all doubt that comes

OFFICER, FIFE COUNTY

CONGENITAL total colour-blindness is a condition of extreme rarity. The first case was discovered by Huddart in 1777. After an extensive survey of the previous 147 years, Dr. Julia Bell ’1 writing in 1926, reported that she could find records In 1933 Geldardpublished a of only 119 cases. case, and mentioned that it was only the eleventh case to be recorded in America. To the completely colour-blind person only black, grey, and white are visible, and the spectrum appears to them as a colourless band of varying degrees of brightness. A definite syndrome, consisting of the following signs and symptoms, is associated with the condition : (a) amblyopia; (b) photophobia; (c) nystagmus ; (d) shortening of the red end of the spectrum ; (e) displacement of the brightest portion of the spectrum from the yellow towards the green; (f) central scotoma. No obvious cause for the amblyopia has yet been discovered. The fundi usually appear normal, and any refractive error present is, as a rule, quite insuffi. cient to account for the poor visual acuity. Parsons3 suggests that the condition is due to an absence of cones at the maculae. Bellpoints out that consanof the has been found in 27-4 per cent. guinity parents of the recorded cases. In view of the rarity of the condition, the following case is considered worthy of publication. CASE RECORD

The patient,

a

boy aged 12,

was

kept under observation

at a child welfare clinic from birth until school age. Since then he has been examined periodically at a school clinic. There is no consanguinity of the parents, nor, as far as can be ascertained, is there any family history of total

congenital colour-blindness. It is very unlikely that there has been total congenital colour-blindness within the last three generations. The serious incapacity pro. duced by the three outstanding signs and symptomsamblyopia, nystagmus, and photophobia-would surely have been noted by relatives. No ocular abnormality was noticed at birth. At 10 months, and thereafter for several months, nystagmus was noted, and there was also an intense photophobia. Dr. A. Rowand,4 who has kindly given us access to his

627 records, observed that

the child

was

dazzled

by light

and could not look up in bright light, although there

BRITISH MEDICAL ASSOCIATION

was

sign of any conjunctivitis to account for this. Since then, the photophobia has been a constant and prominent symptom. No nystagmus was visible when the child wae examined at 2, and again at 4. On the latter occasior Dr. Rowand records that " the nystagmus appears tc have ceased, but the child cannot yet face the light.’ During the pre-school period the child could not be per. mitted to play outside, since his inability to face the light made it dangerous for him to move about in traffic, not only in bright sunny weather but even on dull days, When he attained school age he was examined at regulai intervals of six months at a school clinic. Amblyopie was found to be present, the visual acuity being 6/36 ir each eye, not improved after correcting a low degree oj no

This amblyopia was at first regarded lateral nystagmus, which had agair become apparent. Albinism, conjunctivitis, and cornea ulcers having been eliminated, the photophobia waE judged to be a species of habit spasm, since it was not evident in artificial light. This absence of photophobia in artificial light remains the same to-day and is of parti. cular interest, suggesting as it does that the photophobia may be the direct result of an intolerance to ultra-violel rays. The boy is able to tolerate a powerful electric lamp-e.g., the beam of a Mackie slit-lamp, brought quite close to the eyes-without any undue desire to close them. Later he was discovered to be totally colour. blind. At present his visual acuity remains the same-6/36 ir each eye, with and without correcting lenses. The vision was tested in varying degrees of illumination, but nc appreciable improvement could be discovered in lowered illumination, such as has been reported by some observers He can read Jaeger 1 at 7 in. There is a fine lateral nystagmus and marked photophobia. The fundi show nothing pathological, both optic discs being normal. Prof. H. S. Allan and Dr. D. Jack, of the depart. ment of physics in the University of St. Andrews, very kindly examined the boy with the spectroscope in the physics laboratory of the university. The spectrum appeared to him colourless, as a series of graduated bright. nesses. Although the violet end was normal in luminosity, there was a definite shortening of the red end and a dis. placement of the brightest portion from the yellow tc the green. Each eye was examined separately on two Ol three occasions, and the boy was quite definite in his statements. The fields of vision were mapped out and found to be slightly contracted in the lower nasal areas, The colour fields were charted on a Lister perimeter with 5 mm. discs of blue, yellow, red, and green. These discs of course were seen as varying shades of grey, but they approximated to the fields for white, and did not show their usual contraction. It was found impossible to plot out the presence or absence of a central scotoma with any degree of accuracy. Tests for colour-blindness were made by the following methods: (a) Ishihara’s, (b) Nagel’s, (c) Holmgren’s wools, (d) Board of Trade lantern. By them he was found It was also apparent that; to be totally colour-blind. although he had no idea of colours, he nevertheless pos. sessed a keen sense of shade ;that is to say, he could classify all colours according to their degrees of luminosity. When tested by the Board of Trade lantern he designated light red as a dark grey. The deeper shades of red appeared to him as increasingly darker shades of grey, and were finally confused with black. The lightest shades of yellow and green he called white, but insisted that the " whiteness " of the green was brighter than that of the yellow, thus confirming the result obtained by the spectroscopea displacement of the brightest part of the spectrum from

myopic astigmatism. as

the result of

yellow

a

to green. The boy’s general health is good. Although he is a year behind his age at school, his intelligence quotient, as estimated by the Stanford revision of the Binet-Simon test, is 100. Until the nature of the condition was fully recognised, his lack of knowledge of colours was no doubt attributed to "backwardness." Coloured chalks and paper play an important part in the infant class curriculum, and the child must frequently have been at a (Continued at foot of next colunvrc)

MEETING AT OXFORD

SECTIONS OF MEDICINE

AND SURGERY

AT a joint meeting of these sections, with Prof. G. E. GASK, president of the section of surgery, in the chair, a discussion on the Surgery of Pulmonary Tuberculosis was opened by Dr. J. GRAVESEN (Denmark). All effective surgical measures for treating pulmonary tuberculosis were, he said, extensions of the principle of the artificial pneumothorax. The value of this procedure was generally accepted; but its failure in the presence of localised or general pleuritic adhesions made some further operation necessary. The type and extent of this depended on the surgeon’s views about the means by which the pneumothorax achieved its results. He himself thought that relaxation of the elastic expansion of the lung was the factor of importance, allowing, as it did, the retraction of the healing tissues, and more particularly, of the walls of a cavity. He therefore did not aim at maintaining a positive pressure with a view to immobilisation of the lung, nor did he regard total collapse of the lung as desirable. The object of his treatment was " selective relaxation" of the affected part of the lung, whether apical, upper-lobe, or lower-lobe, and his operative measures were directed towards making that relaxation as complete as possible. In a minority of cases, variously given as 5-10 per cent., there were no adhesions, and these cases showed satisfactory relaxation with artificial pneumothorax. This should nearly always be tried first. Unfortunately adhesions tended to be localised around the diseased part of the lung, and the quite unsatisfactory result was that the healthy part of the lung collapsed, leaving the affected part expanded. The patient was worse off than before, since the healthy part of his lung was out of action and the unhealthy part was unable to heal. In such a case he strongly disapproved of attempts to force closure of a cavity by increasing the positive pressure in the pneumothorax. The introduction of the thoracoscope and cauterisation of the adhesions was often of great value and might give a permanently good result. Where it was apparent that division of adhesions was impossible, or would not give effective relaxation, a choice of three more drastic methods of treatment remained-division of the phrenic nerve, apico- or pneumo-lysis, and thoracoplasty. Oleo thorax and a combination of pneumothorax with local operative treatment Dr. Gravesen condemned. Interruption of

(Continued from previous column) loss.

-

this, his poor visual acuity and photomust always have proved a serious handicap.

Added to

phobia

It is difficult to suggest a suitable occupation for this boy in adult life. He is compelled to wear dark glasses in daylight, and this, coupled with the amblyopia and total colour-blindness, will limit his choice very considerably. REFERENCES 1. Bell, Julia : Total Congenital Colour Blindness. Eugenics Library Memoir, XXIII., Treasury of Human Inheritance, vol. ii., London, 1926. 2. Geldard, F. A. : A Case of Congenital Total Colour-blindness, Jour. Optical Soc. of Amer., 1933, xxiii., 256. 3. Parsons, Sir John : Diseases of the Eye, eighth edition, 1936. 4. Rowand, Dr. A.: Records of St. Andrews Child Welfare Centre, and of the James MacKenzie Institute, St. Andrews.

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