OEOFFREY VAUGHAN, M.B., B.S., D.0. (Melb.), F.R.A.C.S., AND GERARD CROCK, F.R.A.C.P., F.R.C.S., F . R . A . C . S . Prom The Prevention o~f Blindness Unit of the Melbourne University Department of Ophthalmology and The Ophthalmic Research Institute of Australia at The Royal Victorian Eye and Ear Hospital

SUMMARY RETINAL ANGIOMATOSIS frequently produces

severe visual impairment, though loss of an eye occurs less commonly. A unique case is reported here in a 19-year-old girl. One eye had been enueleated for unrecognized complications of angiomatosis 12 years before final presentation in 1969, when the remaining eye showed an unusual degree of cholesterol infiltration in the anterior chamber and vitreous.

Pre-operative therapy eradicated cholesterol opacification of the ocular media and made effective surgery possible. The surgical plan was strengthened by re-examination of fresh sections from the old block of the enucleated eye, in which retinal angiomatosis was then identified for the first time.

INTRODUCTION Disappearance of cholesterol deposits from

the retina has been reported frequently following diet and drug administration.

The present case report appears to be unique in that dense opacification of vitreous and aqueous by circulating cholesterol particles obscured fundus details so that a diagnosis of angiomatosis retinae could only be assumed. The original pathology report on the enucleated fellow eye was not initially helpful in supporting the presumptive diagnosis.

Address for repfints : G. .W. Crock, 32 Gisborne Street, East Melbourne, Victorla, Australia.

CASE REPORT Miss K.M. was first seen in 1956 a t the age

of five, because of her habit of holding books too close when reading. The left eye was blind and divergent. A lower temporal retinal fold was described extending from a point lateral to the macula out towards the ciliary body. The lower temporal retinal vein was engorged. The appearances were attributed to a congenital retinal septum. Transillumination was reported as " clear ".

The uncorrected visual acuity in the right eye was 6/36, which corrected to 6/9 with a -2 - S O / -1 -50, axis 180" lens. Near acuity was not recorded a t that time. HorizonLal nystagmus was present on dextroversion. The temporal margin of the right disc was noted to be hazy and the retina between the disc and macula was pale, yet " seemed to contain numerous blood vessels ".

She presented again in 1958 with a three- week history of severe pain in the left eye, which, by then, had absolute glaucoma. She was admitted t o The Royal Victorian Eye and Ear Hospital. The cornea was edematous, the pupil dilated and fixed, and there was an advanced state of rubeosis iridis. The fundal reflex was yellow and a mass was described in the temporal region associated with widespread retinal hemor- rhages and exudates.

In the right fundus the retina temporal t o the disc was flat, greyish in hue, and had horizontal vascularized strie extending almost t o the equator. A " marked gliosis " covered the upper temporal retinal quadrant.

Chest and orbital X-rays, including optic foramina views, were normal. The results of pathology investigations are shown in Table 1.

The most favoured diagnosis was exudative chorioretinitis, though one staff consultant suggested that Coats's disease could not be excluded.

CLOFIBRATE AND LOW FAT DIET 49

TABLE 1 fibres. I n the serous sub-retinal fluid and in the outer retinal layers macrophages are scantv and there are no cholesterol

-- I- Investigation

- Mantoux test . . . . . . W.R. and Kahn .. .. . . Brucella abortus . . . . . . Antistreptolysin titre . . . . Toxoplasmosis :

Sabin and Feldman dye test . . Complement fixation test . .

Negative Negative Negative 20 200 units per ml.

Negative Negative -

crystals. The cribriform plate is slightly depressed and there are areas of early cystic degeneration in the optic nerve immediately behind it. No toxoplasma or other organisms have been seen and there is no definite evidence of vascular malformation.

The absence of either sub-retinal organization or necrosis of the outer retinal layers, and the presence of vitreous fibrinous exudation gra.vitate against a diagnosis of Coats’s disease. The whole picture is more probably due to a meta- static infection but the nature of the organism has not been elucidated.

Diagnosis : “ Chronic metastatic endoph- thalmitis.”

The child continued to attend out-patients’ sporadically for the next three years, during which time cells were occasionally recorded in the anterior chamber and vitreous of the right eye. One entry described “ an obliterated sheathed vessel ” running to an aneurysm in the lower temporal quadrant. She was lost to hospital follow-up in July, 1961.

I n 1966 she again came under ophthalnio- logical care and was considered to be suffering from chronic cyclitis. The visual acuity in the remaining right eye was 616, with -5.0/-1-0 axis 180”. She was given Prednisolone 5 mg. daily. The fundal appearance had remained unaltered from 1961. She was maintained on this dose of steroid until December, 1968, the visual acuity remaining unaltered.

Visual deterioration did not occur until early in January, 1969. Symptoms of clouding became progressively more marked over three days, culminating in a curtain-like opacifica- tion of her vision. Examination revealed gross haziness of the vitreous and a lower retinal detachment. The patient was confined to bed and steroid dosage was increased to 30 mg. daily.

Over the following two weeks golden crystals appeared in the vitreous and exudates were noted in the temporal retina. The detachment increased in extent and took on a dark appearance. Sudden deterioration of vision then occurred in association with a spreading retinal detachment.

The patient was referred to the Melbourne University Department of Ophthalmology in February, 1969, when the following findings

50 AUSTRALIAN JOURNAL O F OPHTHALMOLOGY

were evident : corrected visual acuity of lees than 6/60 (counting fingers at 1 m.), htraocular pressure was 15 mm. Hg (applana- tion), a Grade 111-IV chamber angle with wattered pigment on the trabecular meshwork.

There was a moderate flare and ckculating golden crystals in the anterior chamber. The vitreous was extremely hazy due to numerous pigment granules and refractile cryfit&. Fundal examination was difficult but by indirect ophthalmoscopy virtually total retinal detachment could be made out with many retinal folds.

An elevated whitish-pink lesion was apparent in the lower temporal perjphery with neo-vascularization on its surface. The lower temporal retinal vein draining this wea was engorged. A small retinal hole was present in the 8 o’clock meridian about the equator. There appeared to be a small retinal dialysis in the lower nasal quadrant. It was felt that surgical interference would have been hazardous and uncertain with such poor visibility of the fundus.

General medical and neurological examina- tions were normal.

A provisional diagnosis of retinal angio- matosis was made and a phased withdrawal of steroids was begun. The serum cholesterol was 205mg. per cent. and the non-fasting total serum lipid was 760 mg. per cent. I n spite of these normal values a low-fat diet (30 gm. per day) was instituted, together with a course of Clofibrate, 500 mg. twice daily for the first week then 750 mg. twice daily for the following five weeks. Gutte atropine 1% was instilled daily into the eye.

After six weeks only a faint flare was present jn the anterior chamber and circulating crystals could no longer be seen. The vitreous haze had cleared considerably and by now the visual acuity corrected to 6/24.

Dr Greer was consulted, and he ordered further sections to be cut from the 12-year-old block of the enucleated left eve. He reported the following findings :

“ Further sections cut from this patient’s left eye show a tangle of capillary vessels in the peripheral retina on one side. The retinal features are not described by Coats, but could be interpreted as the bullous detachment of Reese which is possibly an earlier stage of Coats’s disease before exudates appear. There is a vascular anomaly of the retinal vessels in this eye and possibly in the other eye as well.”

By May, 1969, the vitreous had almost cleared. The patient was admitted to hospital and given a course of intravenous Rheo- macrodex, 10% in Dextrose, 500 ml. over 12 hours for the first day then 500ml. daily for three days. This appeared to cause even further clearing of the media and surgery was then considered feasible.

At operation the early impressions of fundal pathology were quickly confirmed through the cleared media. The nasally situated dialysis was found to be a twin crescent tear in the infero-nasal quadrant. There was a smaII round hole a t the equator in the 8 o’clock meridian and peripheral to this was a mass of angiomatous tissue.

A scleral trapdoor was fashioned from the 11 o’clock meridian, beneath the lateral and inferior recti, to the 3 o’clock meridian, without detaching any extraocular muscle. The bed of this trapdoor measured 9 mm. in its antero-posterior dimension, its forward limit being a t the ora serrata.

Diathermy was applied over the full extent of the trapdoor using a M.I.R.A.* machine on a setting of R.F.? 20. Sub-retinal fluid was drained in the 11 o’clock meridian near the back edge of the scleral bed. A cord of stored homologous fascia lata was placed in the trapdoor and the scleral flaps were closed over it with interrupted 5.0 braided dacron sutures (Ethicon). The retina flattened and a high buckle was produced. Beyond the trapdoor limits the fascia1 cord was thinned and encirclement of the globe completed. Intraocular tension was still low a t the conclusion of this procedure. It was restored to within normal limits by intravitreal air injection. Post-operatively infusion of 500 ml. of Rheomacrodex daily was continued for a further four days.

At the first post-operative dressing the patient remarked that she could see things more clearly and could distinguish the colour of objects which she had been unable to do previously. The vision continued to improve gradually. By the fourth post-operative week corrected visual acuity was 6/36 with -5.25/-2.0 axis 180”. A few fine keratic precipitates were noted, together with a faint flare and a few cells in the anterior chamber. The retina was flat and a good pigmentary reaction was evident around the angioma, which was situated on the anterior surface of the buckle.

* Medical Instrument Research Associates, t Radiofrequency.

CLOFIBRATE AND LOW F A T DIET 51

Eight weeks post-operatively the visual acuity corrected to 6/24 and the anterior chamber was clear. At 12 weeks the acuity was 6/18 corrected and she could read N.5. The patient returned to work a t this stage and continues t o enjoy excellent vision, leading an active life to the time of writing.

DISCUSSION The destructive nature of retinal angio-

matosis is well documented (Vail, 1958). The present case would more than likely have terminated in massive disorganization of the globe and with secondary glaucoma, after the pattern of the fellow eye, but for effective medical and surgical intervention.

The beneficial effects of diet (Van Eck, 1959 ; King et al., 1963) and drugs (Cullen et al., 1964 j Duncan et uZ., 1968) on cholester- olosis of the retina have been extensively reported. Vail, Meyer-Schwickerath and Maumenee have all recorded the value of coagulative therapy for angiomatosis retinae (Vail, 1958 j Meyer-Schwickerath, 1961 j Maumenee, 1969).

We are unable to find a case comparable to that described here, with such an unusual juxtaposition of effective pre-operative

medical therapy and re-examination of old pathology material, culminating in successful surgery for near-terminal angiomatosis retins.

REFERENCES CULLEN, J. F., IRELAND, J. T., and OLIVER, M. F.

(1964), ‘‘ A Controlled Trial of Atromid Therapy in Exudative Diabetic Retinopathy ”, Trans. Ophthal. SOC. U.K., 84 : 281.

DUNCAN, L. J. P., CULLEN, J. F., IRELAND, J. T., NOLAN, J., CLARKE, B. F., and OLIVER, M. F. (1968), ‘‘ A Three Year Trial of Atromid Therapy in Exudative Diabetic Retinopathy ”, Diabetes, 17, 7 : 458.

KING, R. C., DOBREE, J. H., KOK, D’A., FOULDS, W. S., and DANGERFIELD, W. G. (1963), ‘‘ Exudative Diabetic Retinopathy : Spontaneous Changes and Effects of a Corn Oil Diet ”, Brit. J. Ophthal., 4 7 : 666.

‘‘ Fluorescein Angio. graphy in the Diagnosis and Treatment of Lesions of the Ocular Fundus”, Trans. Ophthnl. Sot. U.K., 88 : 529.

MEYER-SCHWICKERATH, G. (1961), ‘‘ The Prcservation of Vision by Treatment of Intraocular Tumors with Light Coagulation ”, Arch. Ophthal., 66 : 458.

VAIL, Derrick (1958), ‘‘ Angiomatosis Retinae, Eleven Years After Diathermy Coagulation ”, Amer. J . Ophthal., 46 : 525.

VAN ECK, William F. (1959) : “ The Effect of a Low Fat Diet on the Serum Lipids in Diabetes and its Significance in Diabetic Retinopathy ”, Amer. J. Med., 27 : 196.

MAUMEKEE, A. Edward (1968) :

52 AUSTRALIAN JOURNAL OF OPHTHALMOLOGY