FROM T H E OPHTHALMIC D E P A R T M E N T OF T H E F I N S E N I N S T I T U T E A N D R A D I U N STATION.

(CHIEF: OLAF BLEGVAD. M . 0.).

IRIDOCYCLITIS AND DISEASE OF THE JOINTS IN CHILDREN.

By Olaf Blegvad.’)

It is a recognised fact that iridocyclitis in childhood is very seldom encountered, and the rheumatic Iridocycl’itis common to adults is so rare in the case of children, that it is practically nonexistent.

Gilbert, writing on ailments of the eye in Phaundler and Schlossmann’s ))Handbuch der Kinderheilkundecc remarks that iridocyclitis rarely occurs in childhood, and that the chronic rheumatism which plays such an important part with adults, means little in childhood. Further, Vallude, who is the author of a similar section in a French handbook on children’s com- plaints, maintains that the rheumatic intis is never found in children.

On those few occasions when a child appears suffering from iridocyclitis, the affection is always of an insidious, chronic type. As the affection is accompanied by little con- gestion and few inconveniences, i t is often quite by chance that it is discovered, It has no resemblance to the rheumatic iridocyclitis occurring in adults and one is therefore Iiable to associate it with syphilis or tuberculosis. Thus, when a Wassermann test is taken and the result is negative, only tuberculosis remains as a possible diagnosis. That the tuber- culin test is perhaps also negative means very little, as this test is frequently negative even in the most typical adult cases of tuberculous iridocyclitis. This probably explains the

*) Received Sept. 22th 41.

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appearance of six cases of children suffering from iridocyclitis a t the Ophthalmic department of the Finsen Institute, where we specially treat tuberculous ailments of the eye. As, in all six cases, we found a disease of the joints of a distinct type, comparable to that previously described by S f i l l , I have ar- rived at the conclusion that the affection has a definite clinical picture, a further description of which might be of interest.

I. The first patient E. N. (identical with A. Friedlander’s patient nr. 1) girl, horn in 1924, is now 1’7 years old.

At the age of three, iritis on the right eye. Six months later, there appeared a disease of the joints, first in the right knee followed by affection of both ankles and accompanied by slight increase of tem- perature. She was treated for articular tuberculosis at various hospi- tals. The X-ray examination shewed no sign of destructive pro- cesses although there was osseous rarefaction. For a time she was at Refsnas Kysthospital. Mantoux test was perhaps or perhaps not positive Adenotonsillotomy was performed. In October 1932, swelling and soreness of both knee-joints.

Temperature subfehrile. Was admitted to hospital. Mantoux test negative (1110 mg.). In 1933 she was again admitted.

No swelling of the spleen and no periphic glandular swelling. Was- sermann test negative. Gonorrheal test negative. Further, there now appeared an iritis on the left eye and a nephritis hamorrhagica. In addition, the right wrist was affected, and there was inuscular atrophy of the lower extremities.

I saw the patient at Fuglebakken Cliildrens Hospital in 1933. Tlierc were then the remains of iridocyclitis and dense cataract on the right eye. On the left eye were keratic precipitates, opacity of aqueous humor, solid synecliia and a commencing cataract. An examination in August 1933 shewed light perception with false projection on the right eye and 3/60 on the left. The patient was treated by various physicians with, amongst other things, iodine, streptococcus vaccine, thyreoidin and Ovex, and the disease of the joints somewhat improved. As she could read with the aid of + 8,OO sph., she was able to attend school.

Several times during 1936 and 1937 she was admitted to the Oph- thalmic department of the Finsen Institute.

For cosmetic reasons, her parents desired the removal of the brilliant white cataract. This was therefore performed with 4 dis- cissions and the operation was in some degrees successful. The situation has since been rather cliangeahle. The joint disease re- curred with serious contractures of botli knee-joints.

Wrists and ankles were also gravely affected and there was

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distinct muscular atrophy in the region of the affected joints. Blood sedimentation test 40. Various treatments were applied, the last being Sanocrysin. This, however, the patient could not stand. She is now lying in the Orthopzdic HQspital.

In spite of all that had befallen her, the patient had shewn un- believable energy, passed the entrance examination and had begun a t a secondary school. The last time I saw her was in October 1940. L.V. 3/18 with correction. The condition of the eyes was stationary but there was a band-shaped keratitis on both eyes. In spite of cataract, visual acuity on the left eyes, with cylinder correction, permitted reading of Jaeger nr. 3.

II. C. G. C. Boy, born 1930. In 1937 the patient was sent from Refsnm Kysthospital, where he was lying with arthroitis talo- cruralis sin., to the Finsen Institute’s Ophthalmic department for examination. The disease of the joints commenced a t the age of 3 years and was regarded as of tuberculous origin. Bandages and light treatment had been applied at a provincial hospital. He was then well until March 1937 when pains occurred in the ankle, and some weeks later he was admitted to Refsnaes Kysthospital. Con- siderable circular swellings of the pericapsular tissue of the ankle were found. The skin was natural with very little tenderness. Con- siderable muscular atrophy in the left lower extremity. Movement of the ankle limited, but no pain. In the meantime, the Mantoux test is slightly positive with 1/10 mg. and an X-ray of the joint shews only osseous atrophy. No definite signs of tuberculosis.

The eye disease was said to have begun on 11th May 1937 and I saw him for the first time a fortnight later, when vision was 619 on both eyes. A very typical band-formed keratitis was found across both corneae and vessels were visible in the iris.

At a later examination, keratic precipitates could be observed on both eyes and exudates on the anterior surface of the lens. Mantoux test was now 0. Blood sedimentation test 4. Stethoscopy and X-ray of lungs showed nothing abnormal. Temperature normal. Disease of the joints stationary. No swelling in the ankle, only a limitation of movement. He goes to school, runs and plays like other children. The eyes are free from congestion, the band-formed kera- titis is unchanged, and occasionally a few precipitates can be seen. R. V. < G / G . L. V. 6/18.

I l l . J. I. Boy, born 1926. The father died of pulmonary tuber- culosis two months before the patient was born. When 8 years old, the patient was admitted to Frederiksberg Hospital with diagnosis adenitis colli, and was treated with compresses, light treatment, iron and cod-liver-oil.

On discharge, the glandular swellings had disappeared. He was then well until September 1937 when, without previous

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trauma, the right knee began to swell. Tyeatment a t home with rest and compresses, but a s the swelling did not diminish, he was admitted to the Surgical department of the Finsen Institute. There was pronounced microadenitis on both sides of the neck. Owing to considerable swelling, the extension of the right knee is limited to 160°. The contours around patella are completely effaced. Skin natural. Flexion only to 70°.

Measurement over the right patella 30,5 cm., over the left 27,5 cm. Mantoux test negative. Wassermann test negative, X-ray of the knee shews no sign of osseous or articular alterations. No atrophy of the bones. X-ray photograph of lungs shews nothing abnormal- Arc-light treatment. Referred to the Eye department on 26th Janu- ary 1938.

Visual acuity of both eyes 6J6. Hm. 1,OO. Left eye slightly congested. Numerous keratic precipitates and synechieae on the front surface of the lens. The retina is seen somewhat hazy but seems to be normal. Right eye normal. When treated with Atropin, the left eye remained slightly inflamed, without pain, but still many precipitates. Folds in membrana Descemeti, a few vessels visible in the iris but no nodules.

The state of the eye gradually came to a standstill and on 1 November the visual acuity was 6/6 Hm. 1,50, there being no in- jection, precipitates or visible vessels in the iris. Treatment was therefore discontinued. Since this date, the eye has remained quiet. All that can be seen now is fine dustlike precipitates on the posterior surface of the cornea and the remains of synechia together with a trace of band-shaped keratitis. After a time, the swelling of the right knee almost disappeared and movement became virtually free. In February 1938, pains occur in the left knee with increase of synovial fluid together with an increase of synovial fluid in the right knee. The patient is now ordered to bed. Occasionally the temperature is a trifle too high but otherwise, examination of lungs, abdomen etc., shew nothing abnormal except microadenitis on the neck.

On the 23rd March he is allowed up, limps somewhat but manages quite well with the aid of a walking stick, and on the 9th April he is discharged. He spent the summer by the sea and the walking-stick was quickly dispensed with. A capsular swelling remained until the beginning of 1939 since when, both knee-joints have been normal. He walks with ease, plays and even indulges in gymnastics a t school.

IV . Aa. G. Girl, born 1937. About the middle of July 1939 in- jection of left eye. A colleague diagnosed a lef t-sided iritis. Complete rest, compresses and Atropin (later Scopolamin) resulted in an improvement, but shortly afterwards, iritis appeared on the right

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eye. In consequence, the child was admitted to Queen Louise’s Child rens Hospital for observation.

No explanation was found for the iritis. Mantoux test 1 mg. negative. Blood sedimentation test 16. Blood normal. Tonsils some- what enlarged. Tonsillotomy performed. Temperature was normal during the whole of the hospital stay. M & B and Streptamid were administered and the condition of the eyes improved. The patient was discharged on the 14th October 1939 but on the following day, congestion reappeared with photophobia, and she was then admitted to the Ophthalmic department of the Finsen Institute on the 16th October. The results of the examination here were as follows: - Wassermann test negative. X-ray of lungs shewed nothing ab- normal. The eyes were moderately injected but subsequent to the examination they became greatly so.

There were keratic precipitates on the right eye, but probably nothing on the left. There were synechiea on both eyes but no vessels visible in the iris. The pupils shone red fairly well. The retina could just be seen, though dimly, owing to opacities.

Tension subnormal. At this point, a possible diagnosis was imagined to be sympathetic ophthalmia resulting from an un- observed lesion. An X-ray was accordingly taken of the first af- fected left eye, but no foreign body was found. Light treatment, Scopolamiri and compresses were administered. With this treat- ment the eye improved and became almost devoid of inflammation, with no photophobia. During her stay, she suffered from frequent cold in the nose, although no increase of temperature, and the eyes were often inflamed. Some few weeks after admittance, a child was admitted to the department who was later found to be suf- fering from measles. The patient was therefore inoculated with measle serum. This caused a slight increase of temperature which however, quickly disappeared. About two months after this in- oculation, there was an increase of temperature to 39,6’ C., with cold in the nose, cough and vomiting. Stethoscopy was normal. After a few days the temperature fell to normal. The child was then allowed to get up - this was in the middle of February 1940 - and it was discovered she was limping, the right knee was thicker than the left. There was considerable increase of synovial fluid in the knee but no tenderness. X-ray of the knee shewed nothing ab- normal except that the articular line was broader than that of the left knee. Our children’s specialist was first of the opinion that it was a case of serum infection, localised in the knee, and a similar opinion was xoiced by the surgeons who later examined the knee. Ephidrin tablets were therefore prescribed. Some capsular swelling of the knee-joint remained and a short-wave treatment of this was now begun.

In March 1940, the child’s parents expressed the desire for her

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to be at home for Easter. She was therefore discharged and made a n out-patient a t Gentofte Amtssygehus, where the joint was treated with extension and plaster of Paris, and during her stay here, the left knee became similarly affected, though to a lesser degree. During the summer she remained at home and exercised the leg with walking, tricycling and the like. The right knee however, could not be completely straightened, and later, in the autumn, she was admitted to st. Joseph’s Hospital’s orthopaedic department. Thc right knee was here treated with splint immobilisation whereafter she was discharged and has since remained a t home. The right knee has still slight swelling and limited movement. Left knee now normal.

An iridocyclitis remains on both eyes, with slight injection, fine precipitates and opacities.

V . B. N. Girl, born 1937. Measles when 7 months old. In March 1940, when about 3 years old, the eyes became injected after a cold. This injection disappeared on the left eye but remained on the right. I saw the child on 20th March 1940. There was a fine peri- corneal injection in the right eye, fine kerativ precipitates, numer- ous vessels visible in iris and synechiz. Left eye only slightly in- jected, but here too there were fine precipitates, vessels in iris corneal injection in the right eye, fine keratie precipitates, nunier- and synechiz. On 4 ApriOl 1940 the patient was admitted to the eye department of the Finsen Institute.

Examination here shewed the following. Mantoux test < 1 - in other words, practically negative. Blood sedimentation test 15. Wassermann test negative. X-ray of lungs, nothing abnormal. The child was an unusually healthy-looking little girl in good nutrition and the eye disease did not seem to trouble her. Atropin and light treatment resulted in an improvement. There was no increase of temperature.

About three weeks after admission, we discovered - due to the fact that our attention had already been drawn to the possible connection between iridocyclitis and children’s joint diseases - that the patient had a diffused swelling around the right knee-joint and fluid in the knee.

Flexion of the knee was not limited and the child walked per- fectly naturally. There was no adenitis colli, axillz or cubiti, but pea-sized adenitis inguinalis. No enlargement of the liver or spleen. Stethoscopy and further examinations normal. X-ray of the knee shewed nothing abnormal.

During the course of one or two months, the swelling in the knee-joint and the knee exudation decreased considerably, and as the condition of the eyes was greatly improled, with no injection, precipitates or \essels in iris but, however, a commencing band- formed keratitis, she was discharged, Just two months after ad-

mittance. She has since attended regularly for observation. The band-shaped keratitis has become more pronounced, especially on the right eye. Some cataract on the right eye. No injection or photophobia.

The knee slowly improved, and at the present moment, ap- proximately six months from the commencement, the knee ailment is almost imperceptible other than that the affected knee is slightly thicker than the left. Other joints have not been affected. There is no muscular atrophy. Visual acuity is at least 6/12 on the least affected eye.

VI . IN. K. Girl, born 1930. Four cyars ago, lmmathom in the right knee after a lesion. In September 19/10, pains in the right knee, and a fortnight later, congestion of both eyes. As vision be- came worse, she was admitted to tlie ophthalmic department of the State Hospital on the 19th October 1940.

Examination shewed R. V. 6/60, L. V. 6/36 without glasses. The eyes were slightly injected, there mere fine keratic precipitates, numerous vessels in iris, total circular synechiae and iris bombe. In consequence, a n upward peripherical iridectomia on both eyes was performed in November 1940. Maiitoux test taken a t the State Hospital was positive with 1/100 mg., but later, a t the Finsen Insti- tute, is was negative with 1/10 mg. Wassermann test negative. Blood sedimentation test GO. Stethoscopy normal. There was no general adenitis and no enlargement of liver or spleen. An X-ray taken a t the Statc Hospital shewed halisteresis but no significant basis for tubcrculosis. On the 23rd October, a t the State Hospital, a flexion contracture of the right elbow-joint was observed. There was considerable swelling of a fungoid nature around this joint and the right knee-joint. No increase of synovial fluid in the knee, y2 centimeter muscular atrophy of the thigh. At the surgical out- patient's department, it was the opinion that tlie affection must be a tuberculous arthritis of the elbow and knee, and the patient was accordingly admitted to the surgical department of the Finsen Institute. New X-ray photographs were here taken of the affected joints. As these shewed only capsular swelling with no destructive processes, as the Maiitoux test was negative up to 1/10 mg., and as no sign was otherwise found of tuberculosis, the patient was transferred to the ophthalmic department on the 17th January 1941. Congestion of both eyes still prevailed, and there were many pre- cipitates, hyperaemic irises, no nodules, extensive synechia? and exudate deposits on the antcrior surface of the lens. Coloboma iridis upwards. Pupillary reflex to light is absent. Tension normal. There continues to be considerable enlargement of the right knee, but flexion has lately undergone a great improvement. She can bend more than 90° and walks quite well with just a slight limp.

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The right elbow-joint is still greatly enlarged and flexion lacks 45'. Moderate atrophy of the muscles around the affected joints. Pea-sized glandular swellings at angul. mandib. and spread about the neck, but nothing in axillae or inguina. No liver or spleen enlargement. Stethoscopy and X-ray of the lungs shewed nothing abnormal. She is still a patient in the department but is allowed up. There is some occasional congestion alternating on both eyes, but she is almost free from photophobia, and, on the whole, the eye trouble affects her very little. There are however, many pre- cipitates and vessels visible in the iris so that the eye disease is not yet a t a standstill. Visual acuity is reported as R. V. 2/36, 1.. V. 6/24.

As is evident from the above reports, all six patients are children who, in early childhood, suffer from a iridocyclitis and a disease of the joints, the one arising comparatively rapidly after the other. In all cases, the secondary affection - eye or joint disease respectively - commenced whilst the primary affection was still present.

When the infrequency of iridocyclitis in children is now taken into consideration, it appears quite remarkable that in all the six cases observed in recent years, the eye disease occurs in conjunction with an affection of the joints.

I have reviewed the number of cases of iridocyclitis for the years 1929 to. 1939 taken from the annual reports of the Communal and State Hospitals' eye departments, in order to ascertain the number of childrens cases included.

A statistic shewed that 896 cases of iridocyclitis were treated at these two departments, twenty of which were children under the age of 15, eight of these being from the State Hospital. On examning the reports of these cases, I find that only four of the eight were under 11 years old. One of these four cases revealed itself to be the second of the two cases described by Axel Friedlaender, and in type similar to those discussed here. Yet another of the four cases is possibly of the same type, as an affection of columna cervicalis was discovered which was impossible to diagnose with certainty from the X-ray pictures. It did not seem to be spondylitis tub. as the Mantoux test was negative with weaker solutions, whilst with stronger solutions (1/100 mg.) only slightly

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positive after a lapse of 24 hours but again negative after 48 hours.

Of the two remaining cases, the first seems to have been an iridocyclitis after morbilli, and the other a slight irido- cyclitis with belt-formed keratitis in a child suffering from stenosis laryngis and polypi in the larynx.

I have examined the otological departments reports on the latter case, but have been unable to find any indication whatsoever leading to an opinion on the etiology of the irido- cyclitis. It seems therefore, that also in Denmark, iridocyclitis in children under 10 years of age is very infrequent, and I have not the slightest doubt that amongst such cases there are a number of them with articular diseases comparable with the four cases from the State Hospital’s ophthalmic de- partment.

W . Gilbert, in his section on diseases of the Uvea in Kurzes Handbuch der Ophthalmologie Vol. IV, page 4, presents a statistic of 600 cases of iritis. Only eleven of these are children under the age of 11 years, and Gilbert writes, that in the first 10 years, syphilis plays the primary, and infection the second- ary part. Such diseases as morbilli, scarlatina and meningitis can result in iridocyclitis in children, although here, tuber- culosis is already of some importance.

I am of the opinion, that i f those cases due to syphilis and infection are eliminated, iridocyclitis in children is ex- ceedingly rare. I think that really tuberculous iridocyclitis in children occurs very seldom. It almost appears that the primary focus of the tuberculous iridocyclitis - as a rule the hilus glands - must attain a certain age in order to cause iridocyclitis, and it is for this reason iridocyclitis tuber- culosa first appears at the age of 20-30 years.

The tuberculous eye disease observed in children is often a miliary tuberculosis, especially in chorioidea but occasionally also in iris (primary complex), but i t is an entirely different disease from the insidious iridocyclitis mentioned in the cases in question. It must otherwise be admitted, that this eye disease can be compared with the clinical picture commonly seen in adults and which here, with perhaps more or less justification, is considered as tuberculous. Syphilis and irido-

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cyclitis occurring after infectious diseases can be disregarded, so there only remains the rheumatic iritis. The acute rheu- matic iritis in no way resembles the insidious disease under discussion. This is so slight in the commencing stages that in many cases the eye disease is only observed quite casually. The child does not complain of pains, is perhaps slightly averse to light and there is very little congestion.

Moreover, rheumatic iritis, as far as is known, is not found in children. Gilbert writes, in the aforementioned section in Kurzes Handbuch, that rheumatic iritis practically always attacks young and middle-aged persons, children and elderly people being spared. I therefore think that the rheumatic iritis in these cases can be excluded.

What then, is the nature of the iridocyclitis? If there is a connection between the eye and the joint

diseases, as I am inclined to believe there is, the nature of the articular complaint should give us some indication of the etiology of the disease. Those who have described this form of juvenile articular disease, have associated it with one of the diseases depicted in 1897 by the English children’s spe- cialist Georg F . S t i l l . Usually regarded as infrequent and as a special children’s disease, it occurs just as frequently in girls as in boys. It begins before the secondary dentition and is characterised by protracted, remittent febrile periods, pro- gressive polyarthritis, polyadenitis, anzmia, enlargement of spleen and muscular atrophy together with a few other in- constant symptoms. Temperature is only high during acute exacerbations, otherwise it is in the neighbourhood of 38’ Centigrade.

The polyarthritis shews itself as a periarthritis, but with a slight tendency to exudation. Changes in the cartilage and bone are only seen in inveterate cases, and the lack of these changes in the Commencing stage is regarded as characteristic for the disease. The X-ray photographs shew therefore very little except halisteresis of varrying degrees. Pains are not occurrent in all cases. The disease begins in one joint and then extends to the others, often symmetrically, and all joints are liable to affection. Polyadenitis can become established in all the regional glands. In some cases, only a few groups of

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glands are affected, but in others the adenitis is only very slightly pronounced. Microscopy of the glands shews simple inflammatory hyperplasia. Enlargement of the spleen is fre- quent but not constant (for example, i t is lacking in three of Still’s twelve cases). Simple anaemia and muscular atrophy are symptoms which are characteristic. The etiology of the disease is unknown. It’s origin is thought to be of an infectious nature, the type of which is not known.

The disease has a chronic progression, but prognosis in slight cases is favourable. Cases of complete recovery are known. A few more or les typical cases have been described in subsequent literature.

Cases are also mentioned by Scandinavian authors viz:- H . Hirschsprung, who calls the disease multiple, chronic, infectious articular disease in childhood, and S . Monrad arthroitis multiplex chronic infantilis.

It will be noticed that these writers do not use the desig- nation >>Still’s Diseasecc as, amongst other things, several of the symptoms are not present (fever, enlargement of spleen, general glandular swellings). In my opinion, it would appear that Still has taken too many symptoms into account, and that the main importance must be centred upon a chronic articular disease not embodying destruction of the points but only periarthritis, often slight glandular swelling and mus- cular atrophy around the affected joint. I would therefore consider it more correct, to designate the disease as chronic juvenile periarthritis.

Diversity of opinion prevails as to the cause of the joint disease.

J . Husler classifies chronic articular diseases in childhood into the infectious and the non-infectious. Under the infec- tious are ( 1 ) primary chronic arthritis, which attacks the small joints first and later spreads to the others. It is pro- gressive and results in serious invalidity owing to ankylosis, (2 ) secondary chronic arthritis - after acute polyarthroitis, syphilis and gonorrheal infection, (3 ) chronic septic poly- arthritis - Still’s disease. This classification however, does not give much information regarding it’s etiology.

Some writers (Leichentritt and E . Kuhn) attribute the

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disease to streptococcus infection, others (Sundt, Olympia and Pollitzer) to tuberculosis. Monrad pays special attention to the muscular atrophy and favours the theory that it is a neurogene affection, whilst Johannesen thinks that the disease is due to endocrine disturbances. In the main, the articular disease gives no indication whatever of the cause of the oph- thalmic disease, but I am of the opinion that it must be regarded as a universal infectious disease in the same way as syphilis, gonorrheal infection, tuberculosis or Boeck’s Sar- coid.

No mention is made of iridocyclitis in Still’s own cases. The first to describe cases of this kind was Axel Fried-

Iznder. In a paper written in 1933, he mentions two cases of chronic septic polyarthritis in childhood accompanied by eye complications. The first case is identical with report nr. 1 mentioned here. The second is a girl aged 6 years. When two years old, her ankle-joint was affected. There followed an affection of columna cervicalis and other joints, especially left elbow and right knee. A t four years, when the joint disease became worse, she was admitted to the ophthalmic department of the State Hospital for a supposed tuberculous affection of the eye. Nevertheless, the Mantoux test was negative, as were also the Wassermann test and Coniplement fixation test for gonococci. Blood sedimentation test 24. X-ray of the affected joints shewed rarefaction, otherwise nothing abnormal. A commencing band-shaped keratitis subsequently appeared on both eyes. Friedlznder does not think i t possible to maintain the designation ))Still’s Diseasecc in these two cases, as the glandular swellings of the spleen and lymphe- gland are absent, but he presumes that they are transient stages and therefore calls the disease chronic septic polyar- thritis. The appearance of the disease was in both cases mon- articular and devoid of the symmetry described as charac- teristic of Still’s disease, but the X-ray photographs of the affected joints were typical. The two cases were first thought to be of a tuberculous nature and Friedlamder stresses the importance of the tuberculin test in diagnosing. His opinion on the possible cause of the disease is not altogether plain, but he has perhaps in mind the possibility of a rheumatic

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complaint as he writes that even if the therapy of these affec- tions is not very encouraging, an anti-rheumatic treatment, rigorously applied at an early stage and an early attack on the eventual centre of infection (caries - tonsils) could per- haps make the progress of the disease milder and the threaten- ing invalidity less serious.

Friedlxnder has the honour of being the first to shew the association between children’s iridocyclitis and the typical accompanying articular disease, and there have subsequently appeared one or two papers on the same subject.

A t the Oxford Congress in 1935, Ej le r Holm described a case of iridocyclitis and band-shaped keratitis in a child suf- fering from infantile polyarthritis (Still’s Disease). When Holm first saw the patient she was 15 years old. At this stage there was band-shaped keratitis and synechiz, the condition of the eyes otherwise stationary. The complaint had begun when the patient was 5 years old and there was swelling of finger-joints, wrist and knee-joint, muscular atrophy and glandular swelling in axil12 and inguina. Mantoux test nega- tive. Liver and spleen not palpable. X-ray of the joints shewed no osseous changes. This case therefore resembles Still’s Disease.

Holm reviews literature from 1918 to 1922 regarding band- shaped keratitis and finds, among the twelve cases therein described, an articular disease in seven of them, - the author takes special regard to the band-shaped keratitis, but the initial factor must in any case be iridocyclitis. As Holm em- phasises, band-shaped keratitis is often seen in children after inflammatory conditions of the eye, whereas adult cases of a similar nature are frequently followed by phthisis bulbi. It seems that Holm assumes that both the eye and joint diseases are of a rheumatic nature.

Apart from the above two Danish papers on iridocyclitis in childhood, I have only succeeded in finding one other publi- cation on the same subject in the literature‘) viz: E . Hiissler’s

- ) A work by Z e e m c m n i n the Sederlandsche Tijdschrift f . Geneeskund. 1940 pp. 134-141 is referred to in the Zentralblatt f. Augenheilkunde, but owing to present circumstances, the original paper is not available for perusal.

Acta Ophthalmol.. Vol. 19. 3-4. 1G

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article in Monatschrift fiir Kinderheilkunde 1939 Vol. 7 7 . : Eyecomplications with polyarthritis chron. Infantil. and Still's Disease, with remarks on the pathogenesis of the rheumatic infection. In this work, Hassler mentions four children suf- fering from articular and eye diseases. Of these, there were only three with iridocyclitis ( the fourth had a scrofulous keratitis).

The first of these three cases was a boy aged nine who, at the age of two years became affected with poly- arthritis chron. infant. The hip, knee, elbow and some finger- joints were affected. X-ray examination shewed only osseous porosity. No enlargement of the spleen but slight glandular swelling. Tuberculin test positive with 1/10,000 Alt-Tuberculin. Wassermann test negative. Blood sedimentation test 58 but normal after Tonsillectomi. The joints were punctured but the irrigation fluid shewed no signs of tubercular bacillte when inoculated on animals. Ophthalmic examination shewed band- shaped keratitis and signs of previous iritis.

Patient nr . 2 was a six year old boy. From the age of two until four years, had periods of fever with swelling of the knee-joint, subsequently also of wrist, elbow and ankle joints but with only slight limitation of movement and no pains. X-ray examination shewed halisteresis, otherwise nothing ab- normal. Spleen and Iiver palpable. Tuberculin test negative. Wassermann test negative. Blood sedimentation test 42. The eye affection was of a serious character, iridocyclitis on both eyes. Three months later, detachment of the retina on both eyes, phthisis bulbi and band-shaped keratitis, keratic pre- cipitates and occlusio pupillz. Iridectomi was attempted, but the eyes remained blind. Hassler is of the opinion, and he i s probably right, that the above is a case of Still's Disease - the progress of the eye affection was much more serious than usual.

In the third case there was no iridocyclitis but only a keratitis. Finally, the fourth case, a girl (age unknown). A t the age of 2 years, tuberculosis in the left foot.

When 5 years old, tuberculosis in the left knee, at 9 in the left foot and left knee, and at 12 years in the left knee again.

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She was treated with plaster of Paris and heat, and the affection was cured after a few weeks. In September 1938 eye symptoms. On examination a month later, a not very charac- teristic affection of the left ankle was discovered. X-ray shewed nothing abnormal in the joint. Band-shaped keratitis on both eyes, iridocyclitis and cataracta coniplicata incipiens.

Hassler regards this case a s polyarthritis chron. infantil. Apparently Hassler is not acquainted with the Danish litera- ture. He is of the opinion that polyarthritis chron. infantil. is an abortive form of Still’s Disease and, in agreement with Leichtentritt seems to think that Still’s Disease belongs to the rheumatic infections. At the same time, in agreement with Weil , he assumes that an injury which can very fre- quently become septic i n children, often leads to allergical forms of disease in adults.

If we now take my six cases, Friedlznders case nr. 2, Holm’s case and Hassler’s three cases, i t will be seen that we have eleven childrens cases with the common feature of iridocyclitis and an articular disease of a distinct type.

Both ophthalmic and articular complaints can be of a more or less serious nature. I t appears moreover, that there is some kind of association in the extent of the two affec- tions, i n such a way that when the joint complaint is only slight and of short duration with only one or perhaps two joints affected, the eye trouble is also less serious, for example in my cases nr. 2, 3 and 5. On the other hand, where the articular disease is serious, as in those cases which present a perfect clinical picture of Still’s Disease, the eye complaint is also serious and ultimately results in blindness as in Hussler’s case nr. 2 .

The disease, which is very infrequent, attacks children between the ages of 2 to 10 years. As a rule, the joints are primarily affected. Subsequently, often during a remission of the articular disease and in some cases up to two years after, the eyes are affected. Sometimes the disease begins with the eye symptoms but the disease of the joints then follows quicker - at the latest six months after - and always whilst the eye complaint is still present. The eye complaint, which is nearly always bilateral, is an iridocyclitis, com-

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niencing slowly with slight injection, fine keratic precipitates, vessels i n iris but never but never nodules, synechiq now and then occlusio pupilla: and iris bomb&, and possible catar- acts complicata. In the final stage, band-shaped Iteratitis is frequently found. There is therefore nothing really charac- teristic, only a chronic iridocyclitis.

In most cases the affection is mild, especially where there is only slight articular disease, but grave cases with compli- cations resulting in blindness can and do occur where the joint complaint is serious and accompanied by all the allied symptoms - fever periods, swelling of spleen, glandular swelling - which appear in the complete clinical picture of SfiZZ’s disease. In the commencement, the joint complaint is often monarticular and can remain so for some months with gradual recovery.

All joints, but more often the knee, elbow and wrist are open to attack, often symmetrically, so that first one knee is affected then the other and so on.

The disease begins with capsular swelling - sometimes with hydarthron which, a s a rule, quickly disappears, whereas the swelling of the capsule can continue for some length of time. There are no pains when the joint is a t rest, but in the initial stage there are often pains when the joint is in motion. X-ray examination, in the main, shews halisteresis and never osseous or cartilage alterations. There is often muscular atrophy around the affected joints. In most cases, the articular disease comes to a standstill, though often after some years and with repeated relapses. Contractures a re not infrequent, especially when the joint is treated with long periods of immobility, and this can result in a considerable limitation of flexion. In addition, various symptoms com- parable with Still’s disease can appear, such as glandular enlargements, swelling of the spleen and periods of fever.

In most cases, prognosis is favourable both for the eye and the joint diseases. Severe and permanent decrease of vision only occurs in the minority of cases where there are complications (cataract - increase of tension). The majority recover with good vision but often with a band-shaped kera- titis a s the last trace of the disease. As a rule. the articular

disease ends with complete recovery of mobility and cessation of muscular atrophy. Contractures add sub-luxations, which compromise a favourable result, occur in some instances, seemingly in those cases where a faulty diagnosis (tuber- culosis) has resulted in too forceful a treatment.

The treatment of the eye complaint has been the usual: Atropin instillation, and during the more acute periods such as during colds and the like, hot compresses. The patient’s general condition should be improved with light treatment, and admission to hospital should be recommended. When the articular disease is in the acute stage, the patient must be confined to bed with slight immobility, compresses etc., but as soon as movement can be made without pain, and an eventual hydarthron has disappeared, it is my impression that it is an advantage i f the joint is used again. As early as 1919, Monrad recommended massage and active and pas- sive exercise as a good treatment, and I consider this to be correct.

When two such infrequent diseases as iridocyclitis in child- hood and the special articular disease - whether it is de- signated Still’s disease or infectious, chronic juvenile peri- arthritis - appear together in so many cases as mentioned here, it must be permissible to assume that here is a definite clinical picture which should be remembered when dealing with children’s cases of iridocyclitis.

REFERENCES: Friedlcender, A.: Ugeskrift for LEger 1933, S. 1190. Gilbert, W.: Pfaundler u. Sclilossmaiin: Handbucli der Kinderheil-

Holm, E.: Transactions of the Opht’rialmological SOC. Vol. LV, 1935,

Husler, b.: Pfaundler u. Schlossmnnn: Handbucli der Kinderheil-

Hirschsprung, H.: Ilospitalstidende. 1901, S. (121. HBssZer, S.: Monatschrift f. Kinderheilkunde. Bd. 77, 1939, S. 23. MoZkte, 0.: Acta med. scandin. B. LXXX, 1933, S . 427. Monrad, S.: Ugeskrift for Iager. 1919, S. 1472. Still, G. F.: Cit. from 0. Molkte. S u n d t , H.: Acta orthoptedica. €3. VII, 1936. S. 2005. Vallude. E.: Cit. from Friedlznder.

kunde. B. 6, S. 113-114.

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kunde. B. 4, S. 637.