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   Table of Contents      
ARTICLES
Year : 1979  |  Volume : 27  |  Issue : 1  |  Page : 46-48

Racemose aneurysm of retina


Department of Ophthalmology, Institute of Post-graduate Medical Education and Research, Calcutta, India

Correspondence Address:
P L Chaudhuri
Department of Ophthalmology, Institute of Post-graduate Medical Education and Research, Calcutta
India
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Source of Support: None, Conflict of Interest: None


PMID: 500182

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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[16]. Magnus[9] first described a case of arterio-venous aneurysm. Leber reviewed the subject and used the term "Racemose aneurysm" Walsh[14]. Wyburn-Mason[15] described a series of 27 cases of arteriovenous aneurysm of retina and cutaneous naevi on the corres­ponding side and mental changes. Vigorelli[17] also reviewed 49 cases of racemose aneurysm of retina. Racemose aneurysm of the retina without involvement of midbrain have also been reported [2],[7],[11],[12],[13] 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 Top


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.

Examination

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 Top


Of the four retinal diseases included under the title anomalous vascular malformations, racemose aneurysm is most rare[16]. There is a familial tendency[10]. 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[1],[3],[5],[10],[16]. The arterio-venous communication may be single or multiple with a pronounced dilatation of the intervening capillary system[3],[6],[10],[15]. This congenital condition is usually unilateral and non-progressive[5]. The temporal retinal vessels are usually affected. No pulsa­tion is seen in affected vessels. Optic atrophy has not been a feature of any case [10],[14],[15] Clinically retina shows no reaction. Haemorrhage, oedema or exudate are absent. Unin­volved retinal areas appear normal[16].

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[8].

Racemose aneurysm of retina may be associated with telangiectases or naevi on the face, scalp or oral mucosa[5]. Sometimes it is associated with similiar vascular malformation of midbrain which give rise to Wyburn-Mason syndrome[15].

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[10]. On histo­pathological examination, Krug and Samuels[8] 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[15], loss of nerve fibres and diminution of ganglion cells[4].


  Summary Top


A case of racemose aneurysm of retina is presented with a comprehensive review of literature.


  Acknowledgement Top


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 Top

1.
Ballantyne, A.J and Michaolson, I.C., 1962, The Text Book of the Fundus of the Eye, 1st. Edition, 311, E.S. Livingstone Ltd., Edinburg and London.  Back to cited text no. 1
    
2.
Cagianut, 1962-quoted by Duke Elder. S, 1964, System of Ophthalmology, 3, Part 2, 787, Henry Kimpton London  Back to cited text no. 2
    
3.
Cameron, M.E., 1958, Brit J. of Ophthal 42, 655.  Back to cited text no. 3
    
4.
Cameron, M.E. and Greer, C.H., 1968, Brit. J. Ophthal., 52, 768.  Back to cited text no. 4
    
5.
Duke Elder, S., 1964, System of Ophthalmology, 3, Part 2, 787, Henry Kimpton, London.  Back to cited text no. 5
    
6.
Greer, C.H., 1972, Ocular Pathology, 2nd. Edition 167, Blackwell Scientific Publications, Oxford, London, Edinburgh, Melbourn.  Back to cited text no. 6
    
7.
Kravitz, D. and Lloyd, R.J., 1935, Arch. of Ophthal., 14, 592.  Back to cited text no. 7
    
8.
Krug, E.F. and Samuels, B., 1932, Arch. of Ophthal., 8, 6, 871.  Back to cited text no. 8
    
9.
Magnus, H., 1874-quoted by Wyburn-Mason (1943) Brain, 66, Part III, 163.  Back to cited text no. 9
    
10.
Reese, A.B., 1963, Tumours of the Eye, 2nd. Edition, 385, Harper & Row Publishers, New York.  Back to cited text no. 10
    
11.
Rentz, 1925-quoted by Wyburn-Mason (1943) in ref. No. 15.  Back to cited text no. 11
    
12.
Seydey, 1899,-quoted by Wyburn-Mason (1943) in ref. No. 15.  Back to cited text no. 12
    
13.
Stokes, W.H., 1934, Arch. of Ophthal., 11, 956.  Back to cited text no. 13
    
14.
Walsh, F.B. and Hoyt W.F., 1969, Clinical Neuro-Ophthalmology, 2, 1690, The Williams and Wilkins Co. Baltimore.  Back to cited text no. 14
    
15.
Wyburn-Mason, R., 1943, Brain, 66, Part III, 163  Back to cited text no. 15
    
16.
Wise, G.N. Dollery, C.T. and Henkind, P., 1971, The retinal circulation, 246, Harper Row, Publishers, New York, Evaston, San Francisco, London.  Back to cited text no. 16
    
17.
Vigorelli 1962--quoted by Duke Elder (1964) in ref. No. 5.  Back to cited text no. 17
    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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Acknowledgement
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