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ARTICLE
Year : 1959  |  Volume : 7  |  Issue : 4  |  Page : 92-95

Miliary retinal aneurysms


The Muslim University Institute of Ophthalmology and the Gandhi Eye Hospital, Aligarh, India

Date of Web Publication7-May-2008

Correspondence Address:
Satya Dev Paul
The Muslim University Institute of Ophthalmology and the Gandhi Eye Hospital, Aligarh
India
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Source of Support: None, Conflict of Interest: None


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How to cite this article:
Paul SD, Shukla B R. Miliary retinal aneurysms. Indian J Ophthalmol 1959;7:92-5

How to cite this URL:
Paul SD, Shukla B R. Miliary retinal aneurysms. Indian J Ophthalmol [serial online] 1959 [cited 2020 Oct 1];7:92-5. Available from: http://www.ijo.in/text.asp?1959/7/4/92/40696

Termed originally by Leber (1912) "Multiple Miliary Aneurysms associat­ed with Retinal degeneration" is now generally called "Miliary Retinal Aneurysms'' Duke-Elder (1941). The disease is very rare and is characteris­ed by a circumscribed area usually in the periphery with hard white and yellowish exudates, more like the circinate retinopathv. The area is often elevated and on it are seen reddish white globules. These globules or globular vascular dilatations are said to occur in the more superficial layers of the retina. Usually it affects young males and is uniocular in distribution. Various reports in literature have agre­ed that one eye is affected and in the affected eve the rest of the fundus is normal. No other consistant general physical findings have been reported to be associated with the disease.

Reese(1951) in a series of five cases of similar findings maintained that his cases formed a separate clinical entity "Capillary Haemangiomas." In the series reported, he found numerous globular vascular dilatations at the periphery of the retina, The lesion was darker in colour, elevated and accom­panied with hard yellowish white exu­dates. There was little or no tendency towards progression. Any visual loss in his series was attributed to macular edema and exudations.


  Case Report Top


S. R., a 19-year-old man, was examined in October, 1957 and has been followed since. His primary com­plaint was gradual diminution of vision in the right eye for the last 2 years. He also complained of discomfort during near work.

External examination : Both the eyes showed Trachoma Stage II and Xerophthalmia. His infra-ocular pres­surc was within normal limits.

Right Eye : A nebulous type of corneal opacity is to be seen. Pupil reacted sluggishly to light and accom­modation.

Vision with -0.50 Sph.,

-0.50 cyl. axis 120 = 6/60.

Left Eye : was normal.

Vision with -0.5o Sph.,

-0.50 cyl. axis 90 = 6/6.

Fundus Examination

Right Eye :
Fundus revealed only moderate amount of periphlebitis and vasculitis. A circumscribed elevated area was noticed along the inferior tem­poral vessels extending up to the hori­zontal meridian. This area was studded with hard white, yellowish waxy exu­dates arranged in a ring like fashion. There were numerous fusiform, sac­cular and globular vascular formations. Some of these formations were white. Aneurysms were present both along the arterial and the venous systems. Macula showed absence of foveal reflex. There were drussen, stippling and exudations at the macula. The terminal venules showed thickening of the walls and dis­tortion of the reflex, [Figure - 1].

Peripheral Field : In the right eye there was an upper nasal contraction with 3/330 White object. Exploration of the central field showed a positive scotoma corresponding to the involved area of the retina.

Left Eve fundus and field were nor­mal.

General physical examination did not reveal any abnormality. Blood pressure was 90/60.

Laboratory investigations for com­plete blood picture, bleeding and coa­sermann and Kahn Reactions, Mantaux halation time, capillary fragility, Was­test, fasting blood sugar, glucose tolerance and urine examination did not reveal anything abnormal.

Hook worm ova were found in the stools. There was no evidence of dia­betes or any other systemic disease. Patient was treated for intestinal para­sites. A diagnosis of multiple aneurysms of the Retina in the right eye was made.


  Discussion Top


Story and Benson (1883) reported a similar case. This was the first case reported in literature. Their patient was a young man of 20 years and revealed the presence of a localised area exhibiting globular vascular dilata­tion-, with associated retinal edema. The patient was followed for 3 Years and the authors noticed progression of the disease. Story (1886).

Doyne (1896) reported a somewhat similar case with the added finding of punctate hemorrhages.

Fisher (1903) reported a similar case in a boy of 13 years with history of family tuberculosis and history of Rheumatic fever.

Leber (1812) after reviewing some cases from the literature and adding 2 cases of his own termed it as a sepa­rate clinical entity and considered multiple emboli such as in Bacterial Endocarditis as the causal factor,

Grubber (1951) described a similar case in a man aged 35. The patient was observed for two years and vari­ous therapeutic agents given which were of no avail.

From the pathological standpoint, Ashton and Langlev (1951) described a typical case of 11liliary Aneurysms. Associated findings in the retina in­cluded a healed lesion of chorioretinitis. Because of this inflammatory focus, the authors believed that the progression and development of the disease could be determined. In previous reports ­Ashton (1951) had reported the find­cnts and felt that superimposed in­flammation exaggerated the condition.

Duke-Elder (1941) on the other hand, considered the disease to be a sort of an angiomatosis, being primarily of a neoplastic and angiomatous na­ture. Junius (1928,1940) believed that Leber's mttiple aneurysms, Coat's disease, Von Hippel disease and Osler disease (Telangectasis) were closely related to one another. Elwyn (1953) believed that miliarv retinal aneurysms belonged within the clinical classifica­tion of Coats disease.

Tour, (1957) while reporting two­ cases of multiple miliarv aneurysms of the retina, considered the causal factor to be either hereditary or congenital malformation of the smaller vessels supplying the retina. He concurred more in favour of Reese's classification of 'Capillar Haemangioma.' The occurrence of Chorioretinitis was con­sidered by him as coincidental. The fundus finding of exudates and retinal edema were considered as secondary manifestation of the primary disease.

On the basis of our present know­ledge and the lack of sufficient patho­logical evidence it would seem that the etiology of miliarv retinal aneurysms is obscure.

However, Leber in 1912 insisted that this was primarily a toxic and embolic arterial disease in which haernorrhage was of secondary importance.

Pathogenetically it has been said that Coat's disease and Leber's disease are variants of the same pathological phenomenon - Houston and Wise (1957). There has been a lot of discussion on this correlation. Coats (1908) believed that his cases were due to organization of hemorrhage from diseased capillaries and veins. On the other hand histopathology of these lesions as described by Leber (1912), Friedenwald (1914) and Leber (1916) indicate obstructed capillaries and small vascular radicles, dilated vascular chan­nels, new blood vessels. aneurysms, preretinal fibrosis and infrequently deli­cate new vessels on the inner retinal surface. The deeper lavers were edematous and cystic. The frequent finding of associated hard white exu­dates or ring of exudates simulating circinate retinopathy has been men­tioned in the literature. It has also been stressed that cases with circinate retinopathv with or without an­eurvsms) often progress to Coats' disease (Gyton 1943, Clausen 1923 and Handman 1937).

There also seems to be a controversy as to the association of aneurysms to the vascular tree. Perhaps it may be the differentiating factor between Coats' disease and miliary retinal aneurysms of Leber. Leber in his survey of 11 cases and two of his Own mentioned the frequency of arterial aneurysms. However a closer study indicated that some of his aneurysms were also ven­ous - Wise(1957)

Mivoshita and Nisyake's (1922) cases showed 3 aneurysms allocated to the venous side and none to the arter­ies.

Kodilinve's case of miliary aneury­sms with retinal degeneration showed 2 aneurysms attached to veins and only two on the arteries.

The case reported in this article had yellowish waxy exudates hard white exudates and arranged in a sort of a ring like fashion. The aneurysms were both arterial and venous.

It is very hard to judge whether it was a case of early Coats' disease or of a full-fledeed Miliarv Retinal Aneurysms. In this connection the authors subscribe to the following opin­ion (Wise 1957) .

Leber's disease is characterised by multiple zones of retinal hypoxia secondary to local capillary or venous obstruction. It is more spotty and milder than Coat's disease and would be to the latter as disseminated is to diffuse chorioretinitis. Its early exuda­tive phase is mild, allowing the retina to remain more nearly adjacent to the choroid. Thus in Leber's disease the hypoxia is greater in inner retinal layers.

Aneurysms and sometimes fibro-blastic vasoproliferation are therfore readily visible on the retinal surface, the latter especially at the centres of these hypoxic areas. When Leber's disease enters its more exudative and degenera­tive stage, the retina is more separated from the choroid by blocking exudation and fatty granular cells. Outer retinal layers then become more hypoxic and neo-vascular fibrosis proceeds subretin­ally. An early stage of Leber's disease with widespread superficial aneurysms which suddenly become exudative, would explain the sporadic report of profuse aneurysrnal formations on the surface of exudative mounds. The explainable relations of Leber', disease to circinate retinopathy is the subject of Reese"s report of two cases of Leber's disease progressing on to Coats' disease a and are thus understandable.


  Summary and Conclusion Top


1. A typical case of Miliary Retinal Aneurysms occurring in young stale in one eve has been described and illustrated.

2. The lesion is more often than not limited to the periphery of the retina, circumscribed and elevated, being characterised by the presence of numer­ous saccular and fusiform vascular dila­tions, aneurysms, hard white and yel­lowish waxy exudates.

3. In th, case described the aneu­rysms seen were both venous and arterial, The terminal vessels showed thick­ening of the vessel wall.

4. No other sign of any systemic disease was noticed except for hook­worm infection.

5. The disease is probably related to Coats' disease and the causal factor is probably hereditary and develop­mental.

6. A brief review of the literature dealing with the clinical and pathologi­cal features is given.[31]

 
  References Top

1.
Ashton, N.(1951) , Brit. 1. Ophthal.,35, 189.  Back to cited text no. 1
    
2.
Ashton, N. and Langley, D.(1951), Brit. 1. Ophthal., 35, .424.  Back to cited text no. 2
    
3.
Berg, F. (1919), Von Graele's Arch Opth., 98,211-242,  Back to cited text no. 3
    
4.
Clausen W(1923) ), Klin. Monatsbl.Augenh., 69, 844 - 845.  Back to cited text no. 4
    
5.
Coats, G (1908) , Roy. London Ophth. Hos. Rep., 17, 440.  Back to cited text no. 5
    
6.
Doyne, R W (1896), Tr. Ophth. Soc.U. K., 16, 94.  Back to cited text no. 6
    
7.
Duke-Elder, W'. S. (1941) , Text Book of Ophthalmology, Vol. III, Henry Kimpton Mosby, St. Louis, p. 2616.  Back to cited text no. 7
    
8.
Elyn, H.(1953) , Diseases of the Re­tina, Blakistan, New York, p. 186.  Back to cited text no. 8
    
9.
Fisher, J, H.(1903), Tr. Ophth. Soc. U. K., 23, 7 - 3.  Back to cited text no. 9
    
10.
Fridenwald, J. H Tr. Am. Ophth. Soc., 13, .440.  Back to cited text no. 10
    
11.
Grubber, 11. (1951), Ophthaln1o1ogica, 121, 91.  Back to cited text no. 11
    
12.
Gyton, J. S. and McGovern, F. H. (10.13), Amer, J. Ophthal., 26,675-684.  Back to cited text no. 12
    
13.
Handman.M.(1937) , Klin. Monat1bi. .Augenh., 96, 613-621.  Back to cited text no. 13
    
14.
Houston, W. R and Wise. G. N.(1957) , Arch. Ophthal., 58, 753.  Back to cited text no. 14
    
15.
Junius, P. (1928), Ztschr. F. Augenh.,62,207.  Back to cited text no. 15
    
16.
Junius, 11. (1940), Ztschr. F. Augenh.,84, 193.  Back to cited text no. 16
    
17.
K lien, B. A. (1037). Amer. 1. Ophthal.,20, 812-819.  Back to cited text no. 17
    
18.
Kodilinve, H. C.(1951) . Tr. Ophth.Soc. U. K., 71, 703.  Back to cited text no. 18
    
19.
Lamb, H.D (1938). Amer.J. Ophthal., 26, 615-641.  Back to cited text no. 19
    
20.
Leber, T. (1915). Handbueh der gesam­ten Augenheil Kunde, edited by T. Saernisch, Wilhelm Engelmann. Leipzig, Vol. 7, Ch.10-.A. pp20-35­  Back to cited text no. 20
    
21.
Leber, T. (1912), :Arch F. Ophth., 81,1.  Back to cited text no. 21
    
22.
Leber, T. (1916) , Handbuch der gesam­ten Augenheil Kunde, edited by T. Saemisch, AVilhelm Engelmann, Leipzig. Vol. 7, edn. 2, pp. 1267-1319.  Back to cited text no. 22
    
23.
Mivoshita, S. and Nisgakey. (1921), Brit. J. Ophthai., 5, .448-153­  Back to cited text no. 23
    
24.
Reese .A. B.(1951) , Tumours of the Eye, Hoeber Newt York, p. 370.  Back to cited text no. 24
    
25.
Reese, A. B. (1956), Amer. J. Ophthal.,42, 1-8.  Back to cited text no. 25
    
26.
Story, J. B. (1886), Tr. Ophth. Soc.U. K., 6, 336.  Back to cited text no. 26
    
27.
Story, J B. and Benson, .A. H. (1883), Tr. Ophth. Soc. U. K., 3, 108.  Back to cited text no. 27
    
28.
Tour, R. L. (1957), Amer. J. Ophthal..43, 426.  Back to cited text no. 28
    
29.
Von Hippel, (1913), Von Graefe's Arch. Ophth.. 86. 443-456.  Back to cited text no. 29
    
30.
Von Hippel E. (1931) Von Graefes­Arch. Ophth., 127, 27-56.  Back to cited text no. 30
    
31.
"Vise, G. N.(1957) A. M. A. Arch Ophthal., 58, 735-746.  Back to cited text no. 31
    


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