|Year : 1979 | Volume
| Issue : 1 | Page : 46-48
Racemose aneurysm of retina
PL Chaudhuri, SC Sen, HS Agarwal, AK Das Gupta
Department of Ophthalmology, Institute of Post-graduate Medical Education and Research, Calcutta, India
P L Chaudhuri
Department of Ophthalmology, Institute of Post-graduate Medical Education and Research, Calcutta
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Chaudhuri P L, Sen S C, Agarwal H S, Das Gupta A K. Racemose aneurysm of retina. Indian J Ophthalmol 1979;27:46-8
|How to cite this URL:|
Chaudhuri P L, Sen S C, Agarwal H S, Das Gupta A K. Racemose aneurysm of retina. Indian J Ophthalmol [serial online] 1979 [cited 2020 May 28];27:46-8. Available from: http://www.ijo.in/text.asp?1979/27/1/46/31548
Racemose aneurysm of retina is a rare congenital vascular malformation, in which the retinal vessels are enormously dilated and tortuous and there are direct communications between arteries and veins without the interposition of capillaries. Racemose aneurysm is also known as arteriovenous aneurysm of retina, retinal arteriovenous fistulae, racemose haemangioma and criscoid aneurysm. Magnus first described a case of arterio-venous aneurysm. Leber reviewed the subject and used the term "Racemose aneurysm" Walsh. Wyburn-Mason described a series of 27 cases of arteriovenous aneurysm of retina and cutaneous naevi on the corresponding side and mental changes. Vigorelli also reviewed 49 cases of racemose aneurysm of retina. Racemose aneurysm of the retina without involvement of midbrain have also been reported ,,,, To our knowledge, no case of racemose aneurysm of retina has been reported from India. Because of its rare incidence, a case of racemose aneurysm of retina is being reported.
| Case Report|| |
P.K.R. a male student aged 15 years was admitted in Eye ward of S.S.K.M. Hospital, Calcutta with the complaint of loss of vision in right eye, which the patient had noticed about five months back. The patient had typhoid eight years back. Both the parents were living and healthy. 'The father and the only brother of the patient were examined and their fundus oculi were normal.
Right eye: was divergent to 15° with normal ocular movements. There was no proptosis, pupil was of normal size, reacting to light. There was only perception of light, media was clear and had no refractive error. Tension was 17.3 mm (Schiotz). Examination of fundus oculus revealed greatly dilated and tortuous retinal vessels involving the optic disc and extending to pre-equatorial fundus with presence of fair degree of glial proliferation in association with dilated vessels. [Figure - 1]. Both veins and arteries were involved. No pulsation of the vessels was visible. Colour, size and margin of the optic disc appeared normal. The superior temporal retinal artery and vein, though much dilated could be distinguished from each other. Three arterio-venous communications were detected at different areas [Figure - 2][Figure - 3][Figure - 4]. The inferior temporal vessels were also dilated, but less so than superior ones. No haemorrhage, exudate or any other abnormality was detected.
Left eye: Vision was 6/6, Fundus oculus was healthy. General examination :- Superficial and transvere facial veins on right side were dilated and they became more engorged when the patient stooped down. The skin of the right side of the face was warmer and showed dilatation of small cutaneous veins in comparison to left side. Clinically there was no other systemic anomaly. Haemogram, urine, stool, fasting blood sugar were normal. X-Rays of skull, orbits and optic foramina were normal.
| Discussion|| |
Of the four retinal diseases included under the title anomalous vascular malformations, racemose aneurysm is most rare. There is a familial tendency. The retina in a sector or all over appears to be filled up with large tortuous dilated arteries and veins which cannot be distinguished from each other,,,,. The arterio-venous communication may be single or multiple with a pronounced dilatation of the intervening capillary system,,,. This congenital condition is usually unilateral and non-progressive. The temporal retinal vessels are usually affected. No pulsation is seen in affected vessels. Optic atrophy has not been a feature of any case ,, Clinically retina shows no reaction. Haemorrhage, oedema or exudate are absent. Uninvolved retinal areas appear normal.
The angiomatous malformations are undoubtedly the result of some fault of development and may be distinguished clearly from the tumours of the blood vessels by the presence of traces of compressed nerve tissue between vascular loops comprising the lesion.
Racemose aneurysm of retina may be associated with telangiectases or naevi on the face, scalp or oral mucosa. Sometimes it is associated with similiar vascular malformation of midbrain which give rise to Wyburn-Mason syndrome.
In our case, the lesion was most probably congenital, without any familial history. It was unilateral. Both the superior and inferior vessels were involved in the papillary and peripapillary regions and three arterio-venous communications were detected. It was associated with vascular malformations on the right side of face. Absence of proptosis and neurological symptoms indicated noninvolvement of the orbital and intracranial blood-vessels.
In this vascular anomaly, visual loss is due to retinal atrophy and gliosis. On histopathological examination, Krug and Samuels noticed cystic degeneration of retina and compressed nerve tissue between tortuous dilated vascular loops. Visual loss has also been attributed to various degrees of gliosis, loss of nerve fibres and diminution of ganglion cells.
| Summary|| |
A case of racemose aneurysm of retina is presented with a comprehensive review of literature.
| Acknowledgement|| |
We are indebted to Prof. R.N. Roy of Department of Neuro-surgery, Dr. H.C. Mazumdar of Department of Cardiology and Dr. M.A. Wali of Department of Dermatology for their valuable assistance.
| References|| |
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]