ACTA OPHTHALMOLOGICA VOL. 39 1961

From the Ophthalmological Department, Central Hospital, University of Helsinki (Chief: Professor Mauno Vannas, M . D.)

CHOLESTEROL-CRYSTALS IN THE ANTERIOR CHAMBER*)

A Clinical and Chemical Study of 7 Cases

BY

Henrik Forsius

Cholesterol crystal deposition can occur in all tissues of the eye. In the anterior chamber, however, it is rare - most investigations of these questions report only one case.

The disease picture is variable, which suggests that the etiology differs in the different cases. The etiological classification can perhaps be done as follows:

Diseases of the lens

The lens often contains needle-shaped cholesterol crystals which can be freed when the capsule of the lens ruptures. When Schmidt in 1831 opened a lens capsule a cloud of crystals entered the anterior chamber. Gautier (1848), Windsor (1858) and Lloyd (1928) noted spontaneous rupture of the lens in a person whose anterior chamber filled with crystals. Loddoni and Knapp have reported a case in which needle-shaped crystals were seen in the anterior chamber after an extracapsular lens extraction. Safar established flat chole- sterol crystals in secondary glaucoma in a similar case. In three cases of spontaneous rupture of the lens Hubbersty and Gourlay found cholesterol crystals in the lens matter. Gruber reported 6 cases of his own of crystal de- position in the anterior chamber, and reviewed 17 other cases from the litera- ture. He was of the opinion that in the majority of these cases the cholesterol crystals in the anterior chamber derive from the lens, and he pointed out - like many other workers - that the patient usually has mature or hypermature cataract.

*) Received February 18th 1961.

284

Diseases of the cornea

Tsopelas reported on a case of familial hypercholesterolemia, crystals present in the peripheral part of the corneal parenchyma and concomitant deposition of crystals in the anterior chamber.

Xanthomatosis bulbi

Yellow inflammatory fatty degeneration of the cornea, anterior chamber and iris, and often even of the posterior part of the eyeball, occurs in this disease which v. Szily (1, 2) was the first to describe. The patients often suffer from hypercholesterolemia. The eye has usually become blind earlier as the result of a trauma or disease, but sometimes the fatty degeneration is directly related to an inflammation. Sala (3) established histologically that fat drops from the iris were transformed directly into cholesterol crystals in the anterior chamber. The chamber was often filled completely with hyaline connective tissue or fatty substance with fatty granule cells often displaying glittering crystals, presumably cholesterol (Szily (2), Engelking, Sala ( l ) , Bardelli, etc.). The vitreous body may be intact, but similar crystal-rich material to that in the anterior chamber may be found there also.

Causes of diseases in the anterior chamber

In 1951, Seidl found crystals in the hypopyon in a case of iridocyclitis. Rohrschneider and Doggart held that crystals may derive from a hyphema or hypopyon.

Vitreous body

Synchysis scintillans is a disease in which cholesterol crystals are deposited in the vitreous for an unknown reason. If the lens is missing, extracted or dis- located the crystals may migrate to the anterior chamber. A case like this was noted as early as 1851 by Backer. The same observation has been made since by Scholer, v. Wecker, Handmann, Engelking, Berliner, etc. Engelking ex- tracted the lens from a myopic girl of 15 with retinal detachment. The anterior chamber immediately filled with crystals to such an extent that it was im- possible even to see the pupil. Although the chamber was emptied several times the crystals remained. Three months later the majority of the crystals had undergone resorption.

A cause of the presence of cholesterol crystals in the anterior chamber is the following fairly uniform disease group, i. e. eyes that have long been blind because of retinal detachment, a perforating trauma or chronic uveitis. They

285

have a cataract which prevents observation into the vitreous. There is often elevation of intraocular pressure but phthisis bulbi occurs also. There are frequent episodes of recurrent inflammation of the eyes. The patients fre- quently report that the eye has been red and painful for some days or weeks and that they have noted a yellow-white spot in the pupil. This crystal sub- stance in the anterior chamber can grow and fill the chamber in its entirety in a few days (Rehsteiner and Caspar).

The classification performed by the present author is somewhat arbitrary. Sala (1) reported a case which was primarily an intermediary form between xanthomatosis bulbi and the last of the forms described above. It is likewise often difficult to know whether or not the crystals derive from the lens.

Histologically, the picture is often one of moderate inflammation with in- flammatory cells in the iris, choroid, retina and ciliary body. The iridic tissue is often atrophied. Among the crystals are usually seen modest amounts of blood, endothelial cells and lymphocytes (Jaensch). The cornea is usually normal, but Descemet’s membrane is sometimes destroyed by the crystals and the cornea may swell and the crystals migrate into the parenchyma (Mees- mann). The same author injected cholesterol crystals into the anterior chamber of rabbit and produced similar changes in the cornea. Rohrschneider (1) injected olive oil in which cholesterol crystals were dissolved into the anterior chamber of rabbit and found crystal deposits later in the aqueous and fatty degeneration in all tissues of the eye.

Crystals can be seen even in the vitreous and subretinally (Rehsteiner, Mees- mann, Rohrschneider (2), SpaniC, Akiya, Scholer).

The iris may be filled with crystals in the spaces. That the iris need not necessarily have a role in the genesis of the crystals is best evidenced by the fact that they have been reported in aniridia (Lampis). Larger amounts of crystals can themselves cause inflammation in the surrounding tissue (Mees- mann, Akiya, Handmann, Safar, etc.).

The persons affected are mostly of middle age or older. The youngest person mentioned in the literature available to the present author was a boy of 14 (Jaensch), the oldest 88 (Hubbersty and Gourlay).

Information is seldom provided concerning the patient’s general condition, and when it is the annotation usually states no more than that the patient has generally enjoyed good health. Loddoni’s patient had disturbances of liver function, Bardelli’s had hypertonia and cardiac hypertrophy and Mattson’s patient had multiple sclerosis. The serum cholesterol content can be above normal, as reported by de Anquin and Copello, DercaE, Feijer, Fischer & Galatski, Jaensch, Kurz, Loddoni, Ramach, Sala (2), Tsopelas, Zewi. and normal or subnormal as reported by Feijer, Rehsteiner, Mattson, Zewi, Sala (1) (0.12-0.14 per cent), Bardelli (0.115 per cent), and Kurz.

Sala (1) attempted a histological analysis of the contents of the eyeball.

286

He found neutral fat, fatty acids, cholesterol, cholesterol esters and minimal quantities of saturated phosphatides.

Chemical studies of the humours of the eye have, to the present author’s knowledge. been confined to verifying the presence of cholesterol crystals.

Matsuba reported cholesterol-esters in the yellow substance in his xantho- matosis-case.

Cholesterol crystals can be resorbed gradually (Enkelking, Kurz, Clegg, DercaE). By contrast, obstructed resorption on account of closed chamber angle (v. Szily (2)) or aniridia (Lampis) has been assumed to contribute to the develop- ment of the crystals.

OWN INVESTIGATIONS

The present series consisted of 7 persons in whom cholesterol crystals were established in the anterior chamber of one eye. Case H. K. was kindly provided by Dr. B. Knape. The control series consisted of 6 patients with one eye enucleated for chronic uveitis and pain and in which over two years had elapsed from the trauma without any formation of crystals in the anterior chamber.

METHOD

The method distributing arcus senilis corneae, the estimation of serum chole- sterol, cholesterol esters, phospholipids, total fat and paper electrophoresis of proteins and lipoproteins in serum has been reported elsewhere (Forsius 1).

Swahn’s method slightly modified was used in the determination of total fat in the eyeball. The method will be described in greater detail in a future work.

Total protein was determined by the micro-Kjeldahl method.

CASE REPORTS

Table 1 gives the most important data for the 7 patients of the present series. Some additional information is provided here.

H. K. The trauma was caused by il cow butting the patient’s eye. The inflammation subsided rapidly and pressure was normal on discharge from hospital. Vision dis- appeared completely within 7 years of the trauma. The patient had no subjective com- plaints until a week before admission when the eye was painful and reddened. It was enucleated. The anterior chamber was so full of crystals that the iris could not be aeen. Liebermann-Burchard‘s reaction was performed on the crystals; it was strongly positive. Patho-anatomical examination disclosed a pronounced round cell inflamma- tion with isolated granulocytes in the ciliary body and iris.

287

Table 1.

Clinical picture of the anterior

I

I symptoms Other eye I I 1 Crystals I Blood

H. K. 59 Q

0. B. 48 8

E. P. 51 Q

R. J. 13 s

M. K. 5 5 CT

M. S. 53 s

T. T. 50 0

Scleral rupture 9 yrs.11 week Hemophthalmia Dislocation of lens

Hemophthalmia 21 yrs.15 mths. Extraction of con- genital cataract (right eye) (Sympathetic (?) ophthalmia in the left eye)

Malignant myopia 5 yrs./? Retinal detachment

Malignant myopia 7 vrs.11 mth. Contusion of eye- ball. Retinal detachment

Perforating 27 yrs.12 mths. scleral wound Subluxation of lens Hemophthalmia

Perforating 18 yrs.14 mths. corneal wound

Malignant myopia 22 yrs.13 mths. Retinal detachment ?

011.2

O/l/00

011.0

l/m/O

011 .o

1/00/1.3

1/00/0.02

None

Micro- scopical

?

None

Weak

Micro- scopical

Weak

Very profuse

Very profuse

Scanty

Scanty

Profuse

Scanty

Profuse

0. B. Discissio cataractae and extractio cataractae mollis were performed on the patient’s right eye in 1936. The patient left before the eye had calmed and could not be followed up. He did not return until 5 months later when an aqueous flare was

288

Clinical picture of the anterior chamber

Pre- Aqueous Lens cipitate flare

? ? Not 8 Carious teeth 4 207 Eyeball tender on visible Acute throat palpation

injection complaint Marked pericorneal

? + Not 8 Healthy 6 287 Marked pericorneal visible injection

Iris hyperemic

pressure ~~~~~~l Choles- mm Hg condition SR terol

+ ++ Clouded

+ ++ Slight sub- capsular opacity

- ++ Slight sub- capsular opacity

+ ++ Dimmed

some- ++ Central times opacity

with profuse crystals

15 Iris bomb6 Hyperemic and atrophic iris

15 Healthy 19 204 Iris bomb& atrophic. Lens capsulare wrinkled Periph. synechiae Pericorneal inject.

13 Otitis media 11 278 Iris bomb6 Hyperemic and atrophied iris Marked injection

10 Healthy 3 300 Papillary occlusion Hyperemic iris Marked injection

17 Healthy 3 300-407 Marked pericorneal injection

diagnosed in both eyes. The patient refused to submit to treatment and did not return until 9 years later. Vision in the right eye was now 0; there was no sign of inflamma- tion in the left eye.

289

Fig. 1. 0. B. A man of 48 operated on 21 years ago for congenital cataract. Acute inflammatory symptoms for 5 months during which crystal

formation occurred.

In 1957, increasing pain in the right eye drove the patient to consult us. For the patient’s status see Table 1. Deep blood vessels penetrated about 2 mm into the corneal parenchyma where the crystals pressed against the cornea. The anterior chamber was deep. Puncture of the anterior chamber gave 0.15 cu. cm. of fluid which coagulated in the syringe. The anterior chamber reformed within an hour. Some days later, 0.1 CU. cm. was extracted and partly syringed onto a strip of filter paper (for paper electrophoresis).

E. P. In 1950, vision in the right eye was 0.17 (myopia - 12 D). There were profuse opacities in the vitreous. In 1951, the present author diagnosed total retinal detachment. The patient made another appearance in 1957 because of pain in her right eye. She was promised a bed at the Ophthalmological Department but did not turn up.

Fig. 2. 0. B. Cholesterol crystals and isolated erythrocytes extracted by puncture

of the anterior chamber.

290

R. J. The patient’s father was blind with malignant myopia. Myopia, - 12 D, was established in his own eye, the right one, a t the age of 3. In 1951 there was a n un- defined trauma to the eye. Retinal detachment was established shortly afterwards below. Two efforts at surgical intervention failed: first ordinary electrocoagulation. then shortening of the eyeball. On October 5, 1958, he reported that there had been a feeling of some foreign body in the eye for a month. (For the patient’s status see Table 1). The patient was given uveitis treatment. Examination on October 21 revealed no crystals, blood or precipitate. On November 13 pressure was normal, a light aqueous flare was seen and semi-dry precipitate was present. Puncture of the anterior chamber gave 0.2 cu. cm. of clear fluid, partly from the posterior chamber for the pupillary membrane split during the puncture. Microscopical examination displayed in addition to cholesterol crystals profuse erythrocytes, scanty leucocytes. The eye was free from irritation at the follow-up a year later (September 19, 1959). There was no aqueous flare and no crystals. Tonography gave the following results: Po oculi dextri = 15. c = 22, Po oculi sinistri = 29. C = 0.04.

M. K. A large fragment of iron was removed with a magnet from the vitreous body in 1931. The eye was calm in 1955 and pressure normal. Vision = 0. A light aqueous flare was established in the left eye on March 13, 1957. The iris was hyperemic. A light aqueous flare was noted on December 7. The iris was filled with blood. There was a fibrinous deposit on the anterior lens surface. The patient was admitted to hospital on January 21, 1958 (see Table 1): The pupil was small. Cholesterol crystals formed a hypopyon-like mass in the anterior chamber. The eye was observed for a week during which injection, aqueous flare and the crystals increased. Enucleation of the bulbus was performed on February 6. The anterior chamber humour and the vitreous were collected immediately after enucleation. Smears of the centrifuged humour of the anterior chamber displayed profuse cholesterol crystals, erythrocytes and isolated leucocytes. Smears of centrifuged vitreous humour revealed profuse crystals, some erythrocytes, a few leucocytes, one eosinophilic cell, isolated lymphocytes. Isolated plasma cells with vacuoles were observed. Eight white blood corpuscles were counted per hundred red cells. The vitreous was a fairly deep yellow in colour and watery. Total retinal detachment had occurred. Non-protein nitrogen in the vitreous was 231 mg per cent, and after centrifugation 234 mg per cent. The total fat content (bound fat) was smaller in the anterior chamber than in the vitreous.

M. S. The patient received in 1937 a perforating corneal wound from a metal frag- ment which was not removed. Four months before enucleation in 1955 the eye might have again been damaged by a fragment. This fragment (or the old one?) was re- moved a few days later with a magnet. The eye did not calm down. The patient was later hospitalised after 4 weeks of increasing pain. For his status see Table 1. The eye was enucleated. Puncture of the anterior chamber after the enucleation yielded 0.15 cu. cm. of fluid. The vitreous was clear yellow in colour. A small white-red deposit formed during centrifugation showing numerous erythrocytes and leucocytes .among isolated cholesterol crystals. A total of 1.5 cu. cm. of vitreous humour fluid was ob- tained; the rest of the vitreous formed a hard mass. Serum cholesterol esters totalled 211 mg per cent. Total fat was 11.97 per cent.

T. T. The patient was prescribed spectacles at the age of 20 (Myopia). In 1931 vision disappeared in the left eye, without pain. She was prescribed a bandage, but not drops, by the physician. Pain and redness developed in the eye at the beginning of the autumn 1953. Iritis was diagnosed, there were profuse crystals but no precipitate. She

291

Fig. 3. M. S . The vitreous humour was collected after enucleation and centrifuged. Cholesterol crystals and deformed blood cells were seen in the smear preparation.

Fig. 4. T.T. A woman of 5 with malignant myopia. Acute inflammatory symptoms for

3 months. At the follow-up several years later the crystals had disappeared.

was admitted to the hospital on November 16. Eye status (Table 1): Pigment granules were seen on the lens whose superficial central parts were dimmed. The deeper-lying parts were not visible. The lower part of the anterior chamber was filled with crystals. Puncture of the anterior chamber produced the following result: A large part of the crystals was extracted. The liquid coagulated in the test tube. As in the other cases, it consisted of flat, slightly oblique parallelepipeds c. 1/15 mm in size. A new puncture was made on December 9; 0.5 cu.cm. of fluid was obtained and the majority of the crystals came with it. The chamber reformed in 30 min. The third puncture was performed on December 10. The aqueous flare was clearly weakened after this pro-

292

cedure. Puncture No. 4 was performed on February 12, 1954. The anterior chamber was shallower. The aqueous flare was again stronger and there were more crystals than at the first puncture. Examination on May 11, 1954, showed a somewhat smallei number of crystals. The eye was free from irritation. Aqueous flare was weak. Iris hyperemic. There was an episode of pain in the eye in August. On September 7 the aqueous flare was strong. There was considerable fat fresh precipitate. Crystals were seen only temporally down in the anterior chamber.

Chemical tests: Serum cholesterol was 300-325-407 mg per cent, cholesterol esters 237-238 mg per cent. Total fat was 9.7-11.55 per mille. Phospholipids were 228-339 mg per cent.

September 13, 1959. The eye was free from irriiation. It had calmed down in spring 1955 and been free from trouble ever since. Eye status was as follows: A 3 mm X 7-8 mm white, slightly elevated blistery band keratitis with synechiae from the iris. The corneal parenchyma was very swollen. The anterior chamber was shallow and missing downwards. There was neither aqueous flare nor other symptoms of irritation. Tono- graphy gave the following results: Po oculi dextri = 12 mm Hg. C = 0.26. Po oculi sinistri = 9, C = 0.23.

Refraction was -9 in the right eye. The lens contained profuse sparkling crystals. There were numerous vitreous opacities and the vitreous contained crystals. Myopic fundal changes were established.

DISCUSSION

General condition

The mean age of the patients with cholesterol crystals in the anterior chamber was 47 years. Six of the patients were between 48 and 59 while the 7th was only 13 years of age, the youngest patient in the literature seen by the author. The basic causative agent was perforating trauma or operation in 4 casq, while malignant myopia followed by detachment was probably the cause in the other cases. Over 5 years had elapsed in all the cases from the time the eye had become blind.

The degree of arcus senilis was normal for the age in these cases (cf. Forsius 1).

The general condition of the patients was good and the sedimentation rate low. This finding concurs well with reports in the literature.

The serum cholesterol level of all the patients except T. T. was normal. The highest value recorded for T. T. was 407 mg per cent.

I t should be noted that another method of determination, one which gives lower values than that used by the present author, was employed for patient H. K. In some cases total fat, phospholipids and cholesterol esters were also determined, but the values did not differ from normal (cf. Forsius 1).

Rehsteiner recorded low cholesterol and cholesterol ester values and mode- rately high phosphatide values in his case.

Nor did the serum proteins and serum lipoproteins which were studied paper electrophoretically in 4 cases differ from normal (cf. Forsius 1).

293 Acta Ophthalmol. Vol. 39, 11 21

Table 2.

Vision Time Acute Pressure Affected Arcus since state mmmg eye/ senilis Name Diagnosis

Other eye diagnosis

Clinical picture

N. S. Perforating 15 yrs. 2 yrs. 12 0/0.5 Weak + ++ 30 wound 8 Sympathetic

ophthalmia in the other eye

M. S. Perforating 2 yrs. 14 yrs. 12 1/00/1.6 34 wound in the 8 sclera

- +

L. P. Perforating 11 yrs. 4-11 yrs. < 8 0/1.6 Micro- ++ + 41 wound of the scopical 8 cornea

M. T. Perforating 13 yrs. 33 wound of the 9 sclera

Hemophthalmia

52 1/00/1.2 Micro- - - scopical

M. L. Perforating 10 yrs. 4 yr. < 8 0/1.6 None + 20 wound 8

R. P. Keratitis >20yrs. 1 week- 20 1/00/1.0 Micro- ? ? 33 in childhood 4 Y'. scopical 8

294

Table 2.

I I I I n.neral dition

Serum Choles- Total choles- terol fat terol ester 1 SR lc;:

Dimmed Hyper- Humour of the anterior 2 Healthy (280-) 225 8.8 emic chamber yellow, viscid. 310

Pericorneal injection Posterior synechiae Vitreous black-red Phthisis bulbi

Slight Hyper- Humour of the anterior 1 1 Xanthoma 260 150 6.7 opacity emic chamber yellowish, tuberosum

ovatery. Retinal detachment. Vitreous yellow-red, coagulated on puncture

Dimmed Yellowish Anterior chamber low, 7 Healthy 260 200 humout clear, vitreous slightly yellow, clear Phthisis bulbi Corneal leukoma

Dimmed, Hyper- Phthisis bulbi. Shallow 3 Healthy (165-) 170 6.1 calcified emic, anterior chamber 235

atrophied Vitreous and aqueous Posterior humour clear synechiae Secondary glaucoma

Phthisis bulbi. Healthy 185 155 7.4 Low anterior chamber, aqueous humour clear Vitreous fluid and slightly red

? Not Anterior chamber deep 3 Healthy 245 183 8.3 visible Diffuse scars on the

cornea Blistery epithelium Cornea bulged out, showed blood vessels The eyeball very long Vitreous and aqueous humour fluid, clear

295 216

Condition of the eye

According to Gruber and other workers, the crystals derive in most cases from a lens which has suffered damage or perforated spontaneously. Case E. P. may belong to this group, although no rupture was observed in the lens capsule nor lens masses in the anterior chamber. In cases R. J., M. K. and T. T. the lens was intact and its parts could be distinguished. T. T. also had crystals in the lens. 0. B. had undergone a cataract operation 24 years earlier and had crystals in the vitreous, too. Finally, in case H. K., it was hardly likely that the chamber could be so full of crystals from the lens that it was impossible even to see the iris. The iris was usually atrophied and in all cases moderately or markedly injected. No accumulation of fat was seen in it. The only eye that was examined histologically. case H. K., showed moderate lymphocytic infiltration in the uvea and provided no additional information to that obtained in the literature. M. K. and M. S. had crystals even in the vitreous.

Control material

In an earlier work (Forsius 2) the present author examined chemically 44 eyes enucleated for various reasons. Six were patients whose eye had suffered a perforating trauma at least 2 years ago. After a period with no inflammation, the eyes had again become red and painful. One of these 6 cases developed cholesterol crystals and was included in the present series (M. S.). That case was replaced with another, and the data on the earlier series is given in Table 2. An interesting point was that one of them had xanthoma tuberosum, theoretically a predisposing factor if the general condition has something to do with the formation of cholesterol crystals. The cholesterol content, cholesterol esters and total lipid values in the serum were normal or low. Serum proteins and lipids were also normal, as they were in the cholesterol crystal group. The injection in the eye was roughly identical in both groups.

Table 3 combines the chemical findings for the humour of the anterior chamber and the vitreous of the two series. No clear finding emerged. Neither the fat content or the cholesterol content was noticeably high in the eyes with crystals in the vitreous and in the anterior chamber. Nor did the protein values or the protein:lipid ratio of the two series differ. Paper electrophoresis likewise failed to provide a clear answer (Table 4). The comparison (Forsius 2) between the electrophoretic findings for eyes enucleated within 8 months of the trauma and for eyes enucleated at least two years after the trauma showed somewhat lower albumin values for the former in the anterior chamber and vitreous. Lower values in the means - though not in individual cases - could be seen in the eyes with crystals as compared with the eyes in which no crystals had formed.

296

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M. K.

M. S

.

T. T

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M. T

.

N. S

.

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.

M. L.

M. S.

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Patie

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8

Table 4.

Name

Group 1

0. B. R. J. M. K. T. T. M. S.

Lipid Proteins in the anterior Proteins in the vitreous chamber humour

20.6 10.9 4.3 64.2 23.4 19.9 9.0 47.7 25.3 12.1 7.3 55.3 24.6 15.2 7.8 51.8 17.3 82.7 21.2 17.3 14.1 47.4 17.5 12.7 8.4 61.4 16.2 7.8 6.3 69.7

Mean 21.6 14.6 8.6 55.2 20.4 11.8 7.0 60.8 17.3 82.7

Serum protein 21.9 12.9 8.9 56.3 mean

18.5 81.5

Group 2

N. S. 21.7 10.6 7.4 60.3 27.3 16.1 10.2 46.4 M. S. 24.5 18.0 6.5 51.0 15.9 12.5 3.1 68.5 L. P. 13.6 11.3 6.9 68.2 30.2 69.8

M. L. 17.2 6.8 3.0 73.0 M. T. 11.9 11.3 5.3 71.5 14.6 10.6 - 74.8

Mean 19.4 13.3 6.4 60.9 17.7 11.5 4.6 66.2 30.2 69.8

Serum protein 19.1 14.9 10.2 55.8 mean

23.1 76.9

Group 1: Patients with cholesterol crystals in the anterior chamber. Group 2: Patients without cholesterol crystals in the anterior chamber.

The lipid content in these eyes was roughly the same as in other eyes

Why, then, do crystals form in the anterior chamber? It is known that the process takes many years. The present author assumes

that the causative agent of crystals in the anterior chamber is the same as the unknown mechanism that produces synchysis scintillans. The process is more rapid when clinically demonstrable uveitis is present. Proteins and fats enter

enucleated for various reasons. (Unpublished data).

298

the chamber with the flow of fluid in the eye. As cholesterol is not readily soluble in water, crystals are deposited more easily in the chamber. The cases in which an accumulation of crystals filling the chamber as a whole occurs in a few days can be attributed, as some workers have suggested, to a saturated solution or to a rupture in the tissue separating the vitreous from the anterior chamber. The crystals usually provoke no inflammatory symptoms in the vitreous, but produce them in the anterior chamber. The acute phase when the cause of crystal deposition is in the vitreous usually occurs concurrently with the observation of the crystals, and the principal reason for the injection is possibly the mechanical irritation of the chamber tissue by the crystals. The crystals had disappeared and the outflow was normal in two of the patients who reported for a follow-up. The process is consequently reversible. These cases also serve as evidence that the crystals are not as such an indication for enucleation. I t is possible to remove the crystals by puncture, as was done by the present author. They are readily extracted with Amsler’s chamber puncture needle or with a No. 20 injection needle since the crystals are very small, c. 1/15 mm long. The punctures can be repeated without damaging the eye, and the uveitis is kept in check with cortisone therapy and mydriatics.

Sala (1) reported a case with crystals in the anterior chamber in which the eye was enucleated because precipitate originated in the fellow eye and sym- pathetic ophthalmia was suspected. The present series included a similar case (0. B.). The inflammation was inadequately treated in many of the present series and in some of the cases reviewed in the literature. Patients refused to submit to long-term treatment and refused enucleation. I t is possible that there would be a considerably larger number of such cases if all the other patients had behaved in the same way.

SUMMARY

The series comprised 7 patients with cholesterol crystal deposits in one eye. Six of the patients were aged 48-59; the seventh was 13. The basic causative agent of the cholesterol deposition was perforating trauma or surgery in 4 cases and malignant myopia followed by retinal detachment in 3 cases. In all the cases, 5 years had elapsed from the loss of sight in the eye. The patients’ general condition was good and except for one case with hypercholesterolemia. Serum cholesterol and other fat studies gave normal values. The arcus senilis corneae was of normal degree. The crystals probably derived from the lens in one case only. Two of the enucleated eyes had crystals in the vitreous, too.

The control series consisted of 6 cases with eyes enucleated at least 2 years after the trauma on account of pain and redness in the blind eye. There was no clear difference between proteins and lipoproteins in the aqueous humour and

299

the vitreous in the two series (Table 4). The albumin content i n the anterior chamber a n d the vitreous, however. was on the whole slightly lower i n the subjects with crystal deposits i n the anterior chamber.

In Table 3 a r e combined the cholesterol, protein and total fat values for the aqueous humour and the vitreous of the two series.

The f inding was not clear here either. Crystals might well form because of the resorption of the fat-releasing sub-

stances. Chronic uveitis seems to be an important factor in crystal formation. Where the crystals d id not originate through rupture of the lens, it was assumed that the cause was i n the vitreous. Crystal accumulation takes place at this site i n particular owing t o the small crystal-releasing ability of the humour of the anterior chamber.

There were 2 cases i n which the crystals had disappeared and the chamber outflow was normal. Puncture of the chamber and uveitis therapy are re- commended for management of the affection.

REFERENCES

ARiya, S.: Acta SOC. ophth. Japan 1956: 60: 5. Ref. Ophth. Lit. 1956: 10: 352. Anquin, M. H . de & Copello, M. D.: Arch. Oftal. B. Aires 1955: 30: 89. Backer, L. Th.: Schmidts J. b. 1851: 70: 107. Ref. by Mattson. Bardelli, L.: Boll. ocul. 1924: 3: 833. Ref. in Zentralbl. Ophth. 1926: 16: 290. Berliner, M . L.: Biomicroscopy of the eye. vol. 1. Hoeber, New York 1949. p. 593. Caspar, L.: Klin. Monatsbl. Augenh. 1924: 72: 474. Clegg, J . G.: Tr. Ophth. SOC. U. Kingdom 1921: 41: 463. Ref. Zentralbl. Ophth. 1923:

Derkat, V.: LijeZniZki vjesnik 1924: 46: 25. Doggart, J . H.: Tr. Ophth. SOC. U. Kingdom 1952: 72: 15. Engelking, E.: Klin. Monatsbl. Augenh. 1927: 79: 721. Feijer, J.: Am. J. Ophth. 1933: 16: 721. Fischer & Galatski. Ref. by Feijer. Forsius, H.: Arcus senilis corneae, its clinical development and relationship to serum

Forsius, H.: Acta ophth. 1958: 36: 569. Forsius, H.: Lipid Keratopathy. Acta Ophth. 1961: 39: 272. Gautier: Ann. ocul. 1848: 20: 69. Ref. by Gruber. Gruber, E.: Am. J. Ophth. 1955: 40: 817. Handmann: Klin. Monatsbl. Augenh. 1923: 70: 395. Helbron, J.: Ztschr. Augenh. 1900: 4: 200. Hubbersty, F . S. & Gourlay, J . S.: Brit. J. Ophth. 1953: 37: 432. Jaensch, P. A.: Klin. Monatsbl. Augenh. 1926: 76: 476. Kurx, J.: €as. lkk. €esk. 1929: I: 77 and 126. Ref. in Zentralbl. Ophth. 1930: 22: 491. Knapfi, A.: Am. J. Ophth. 1937: 20: 820. Lampis, E.: Boll. ocul. 1933: 12: 547. Ref. in Zentralbl. Ophth. 1933: 30: 10. Lloyd, R. I . : Am. J. Ophth. 1928: 11: 271. Loddoni, G.: Lett. oftal. 1929: 6: 41.

8: 151.

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Mattson, R.: Finska 1ak.-sallsk. handl. 1938: 81: 778. Matsuba, Y.: Chuo-Ganka-Iho 1936: 28: 1. Ref. in Zentralbl. Ophth. 1937: 37: 216. Meesmann, A.: Arch. Augenh. 1924: 94: 56. Ramach. F.: Ztschr. Augenh. 1933: 81: 112. Rehsteiner, R.: Klin. Monatsbl. Augenh. 1932: 89: 291. Rohrschneider, W.: v. Graefes Arch. Ophth. 1927: 118: 131. Rohrschneider, W.: Ztschr. Augenh. 1927: 61: 296. Safar, K.: Ztschr. Augenh. 1926: 39: 397. Sala, G.: Boll. ocul. 1931: 10: 453. Ref. in Zentralbl. Ophth. 1932: 26: 460. Sala, G.: Boll. ocul. 1933: 12: 531. Ref. in Zentralbl. Ophth. 1933: 30: 9. Sala, G.: Boll. ocul. 1934: 13: 385. Ref. in Zekralbl. Ophth. 1934: 31: 717. Schmidt: Ztschr. Ophth. 1831: I: 350. Ref. by Gruber. Scholer: Berl. klin. Wchnschr. 1880: 421. Seidl: Wien. med. Wchnschr. 1851: 34. Ref. by Helbron. Spanit, A.: LijeEniEki vjesnik 1937: 59: 200. Ref. in Zentralbl. Ophth. 1937: 39: 403. Swahn, B.: Scand. J. clin. Lab. Invest. 1952: 4: 247. v. Srily, A.: Ber. deutsch. ophth. Gesellsch. 1922: 42: 285. u. Srily, A.: Klin. Monatsbl. Augenh. 1923: 71: 30. Tsopelas, B.: Bull. SOC. Hell. Ophth. 1948: 16: 262. u. Wecker, V . L.: Die Erkrankungen des Uvealtractus und des GlaskBrpers. In Graefe-

Saemisch: Handbuch der gesamten Ophthalmologie. Vol. 4. Engelmann, Leipzig 1876. p. 697.

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