Indian Journal of Ophthalmology

: 1979  |  Volume : 27  |  Issue : 3  |  Page : 52--54

Racemose aneurysm of the retina

GN Seal, TR Kundu 
 Department of Ophthalmology, Medical College Hospital, Calcutta, India

Correspondence Address:
G N Seal
Department of Ophthalmology, Medical College Hospital, Calcutta

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Seal G N, Kundu T R. Racemose aneurysm of the retina.Indian J Ophthalmol 1979;27:52-54

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Seal G N, Kundu T R. Racemose aneurysm of the retina. Indian J Ophthalmol [serial online] 1979 [cited 2021 Jan 21 ];27:52-54
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Racemose aneurysms of the retina are rare congenital vascular anomalies, often referred to arteriovenous communications of the retina. The condition was first described by Magnus in 1874 and since then, more than 60 cases have been reported by different observers. Some­times, the retinal affection is associated with similar lesion in the midbrain and facial angioma on the same side. There may be a familial tendency. Usually, one eye is affected although bilateral cases have been recorded.

The retinal lesion may vary in extent considerably. In lesser manifestations, these anomalies may be restricted to the periphery of the retina, usually in the lower temporal quadrant. Direct arteriovenous communica­tion can, sometimes, be seen between the enlarged and tortuous retinal arteries and veins. In some cases, there is a marked increase in the number, size and tortuosity of the retinal vessels and the arteries and veins are no longer distin­guished from each other. There may be exophthalmos with pulsation of the globe. The pulsation is probably the result of arterilization of the vascular tract. In the presence of a midbrain lesion, there may be attacks of headache, vomiting and neck rigidity with or without loss of consciousness.

We are reporting one such case of racemose aneurysm of the retinal vessels of the right side which is associated with mild degree of proptosis and facial angioma of the same side.

 Case report

A ycung muslim girl, M.K. aged 14 years was first seen in the eye infirmary, medical college hospital, Calcutta on 16.2.78. Her chief complaints were gradual swelling of her right eye for 6 years and dimness of vision in the same eye for 3 years with mild headache. Her mother gave a history of fall at the bathing ghat with injury to her right temporal region 8 years back. About two years after the accident, her right eye started protruding.

On examination, the right half of the face appeared little swollen and boggy.

Right eye:-Both the lids were little thickened. The globe was mildly proptosed. It was axial in nature with no restriction of ocular movements. The proptosed globe was non-pulsatile and non-compressable. Pupil­lary reaction to light was brisk but ill-sustained, the vision was reduced to hand movements and intra.ocular tension was normal.

Ophthalmoscopy: There were numerous, dilated and tortuous retinal vessels with little differentiation between the arteries and veins. These dilated vascular channels formed a convoluted mass in the region of the disc which could not be made out. Pair amount of gliosis was noted within the interstices of the vascular malformation over the region of the disc and the adjoining area.

The left eye was normal in all respects.

Investigations: Haemogram, urine, X-rays of orbits were normal.

Carotid angiography-Right carotid angiography demonstrated a huge arterio-venous malformation in the suprasellar region. There was marked dilatation of the right ophthalmic artery with numerous abnormal vessels in the right orbit.

Frontal venography could not be done due to smallness of the frontal veins. Fluorescein angiography was not carried out for want of dye.


Racemose aneurysm of the retina may be associated with similar lesion of the brain of the affected side. Bonnet et al pointed out the association of retinal, cerebral and facial racemose angiomas and considered the syndrome to be one of the phakomatosis. Wyburn­Mason[4] reviewed the literature and collected 27 cases of retinal arteriovenous aneurysms.

In 22 cases there was evidence of an arterioven ous aneurysm of the midbrain. They also found records of 20 cases of arteriovenous aneurysm of the midbrain, 14 of which had abnormalities of the retinal vessels. Bech and Jensen,[1] however, were of the opinion that many of the patients had no angiographic or pathologic evidence of cerebrovascular malformation.

These abnormal tortuous vessels usually extend as tracts from the retina to the midbrain. They cover and permeate the optic nerve and extend through the chiasma and optic tract to the dorsum of the midbrain. Involvement of the optic pathway subsequently leads to atrophy of the nerve fibres which account for the visual loss. The vessels appear more like arteries than veins although some may retain venous characteristics.

In minor form, the condition remains asymptomatic and is detected during routine examination of the fundus at the time of refraction. The common symptom is visual failure which may be sudden or gradual. Sudden dimness of vision is due to occurrence of thrombosis in or haemorrhage from the vessels. It is usually accompanied by headache, vomiting and exophthalmos. Gradual dimness of vision is due to secondary retinal atrophy and gliosis. The case presented here had a history of fall on the head which was followed by mild headache and gradual visual failure and some proptosis. Patient did not show any symptom suggestive of abnormality of the mid­brain but exhibited mild degree of facial angioma of the right side evidenced by somewhat swollen and asymmetrical appearance of the face.

As far as sight is concerned, the prognosis is doubtful. Some patients develop blindness early while others retain almost normal sight for years. The fundus lesions are frequently so widespread and formidable as to preclude any therapeutic approach.

However, various types of treatment have been employed including deep X-ray, ligation of the common and internal carotid arteries, insertion of radon seeds in the retinal lesion. All of these procedures proved to be useless. Treatment of the cerebro-vascular malformation is problematic and many neurosurgeons will hesitate to go for any surgical intervention in symptomless cases.


A case of racemose aneurysm of the retinal vessels with mild degree of proptosis and facial angioma of the right side is presented. Carotid angiography of the same side shows that the anomaly also affects the ophthalmic artery from its origin. Literature of racemose aneurysm is reviewed regarding its incidence, visual prognosis and management.


1Beck, K. and Jensen, O.A., 1961, Acta Psychiat, Neural (Scand.), 36, 47.
2Desmond, B. 1973, Amer. Jour. Ophthal., 75.
3Reese, A.B., 1963, Tumours of the Eve, Harper and Row Publishers, New York. 2nd td. p. 385.
4Wyburn-Mason, R., 1943, Brain, 66, 163.