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   Table of Contents      
ARTICLES
Year : 1982  |  Volume : 30  |  Issue : 6  |  Page : 641-646

Angiomatosis retinae-clinical features & management


Sankara Nethralaya Medical Research Foundation, Madras-6, India

Correspondence Address:
B Sridhar Rao
Sankara Nethralaya Medical Research Foundation, 18 College Road, Madras-6
India
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Source of Support: None, Conflict of Interest: None


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How to cite this article:
Rao B S, Abraham C, Abraham M, Surendran T S, Badrinath S S. Angiomatosis retinae-clinical features & management. Indian J Ophthalmol 1982;30:641-6

How to cite this URL:
Rao B S, Abraham C, Abraham M, Surendran T S, Badrinath S S. Angiomatosis retinae-clinical features & management. Indian J Ophthalmol [serial online] 1982 [cited 2019 Jun 16];30:641-6. Available from: http://www.ijo.in/text.asp?1982/30/6/641/29303

Table 2

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Table 2

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Table 1

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Table 1

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The term Angiomatosis Retinae is used to describe a hamartomatous tumour of retinal tissue which is developmental in origin. A classical primary lesion consists of a vascular mass, red or whitish in colour supplied by dilated and tortous feeder vessels. Some of these lesions are non-progressive while the eventual outcome in many is progression to secondary retinal detachment, massive exuda­tion, haemorrhage, uveitis, intractable glau­coma or phthisis bulbi. Photocoagulation, cryo therapy and diathermy are the currently employed modes of therapy which when ap­plied at the appropriate time help to arrest the progress of the disease.


  MATERIALS, METHODS AND OBSERVATIONS Top


14 eyes of 12 patients with angiomatous masses seen at Sankara Nethralaya form the basis of this study. Six patients were males and six females-their age ranging between 11 and 50 years. All patients had a detailed ophthal­mological examination and a thorough medical work-up by the Physician. Fluorescein angio­graphy was performed in 7 eyes where the media was clear enough and the situation of the lesion within the reach of the fundus

camera. A detailed fundus drawing was made in all other cases. The ultrasonographic features of the angiomatous lesions were studied in 3 eyes using the Ocuscan 400 contact A and B scan. Eyes in which an angioma had produced a complication and uncomplicated angiomas in patients who had lost a fellow eye due to com­plications of angiomatosis were subject to treatment.

The modalities of treatment employedare shown in [Table - 1]. Photocoagulation was direct­ed towards the mass and carried out in diff­erent sessions until the tumour showed signs of regression. Spot sizes of 4.5° and 6° with the xenon and 200 microns and 500 microns with the argon laser were used with an ex­posure time of 0.5 sec and 0.05-0.5 sec respec­tively. The power was adjusted to produce a mild blanching of the tumour. The triple-free­zethaw technique described by Amoils was used in cryocoagulation. This was combined with scleral buckling in one. The period of follow up ranges from three months to more than one year. Three have been followed for less than three months. The results of the treatment were judged by shrinkage of the tumour, re­duction in size of feeders, absorption of ex­udates, settlement of secondary retinal detach­ment. Fluorescein angiography was used to document some of these changes,


  Clinical features Top


1. Ophthalmoscopic fndings

The 12 patients in this study harboured 50 angiomatous masses within their eyes. 39 of them were situated on the temporal side, 11 were nasal and 2 were on the optic disc. Tumours within each eye varied in number and size. They had produced varying degrees of exudation in 9 eyes, varying extent of secondary retinal detachment in 6 and vitreous haemor­rhage in 2. There were no complications in ;. The relationship between number and size of the tumours to these complications is shown in [Table - 2].

Five of the fellow eyes were normal. 3 eyes which presented with complicated cataract and 2 with extensive secondary retinal detachment were most likely due to the complications pro­duced by angiomatosis retinae.

None of the patients had any associated systemic involvement.

2. Fluorescein Angiographic Features

The tumour masses filled in a slow and steady manner in 2 eyes. While most tumours leaked the dye in varying degrees, the leak was minimal in 2. The emptying in this case was complete in the delayed phase and was remark­ably rapid. Capillary drop-out was observed in 4 eyes, and was seen in the region surrounding the masses and distal to it. Compensatory dila­tation of the remaining capillaries was an in­variably associated feature. Angiography helped in identifying a small angioma in a patient in whom the lesion simulated a retinal hole ophthalmoscopically. In another, it helped in confirming the diagnosis of angioma that was suspected on ophthalmoscopy.

3 Ultrasonographic Features

B scan ultrasonography showed these angiomas to be round and smooth, comprising of clusters of dot like echoes. The correspond­ing A-scan syowed a tall amplitude spike from the surface of the tumour which on decreasing the sensitivity continued to remain tall. This was followed by a number of closely spaced mid amplitude spikes from the centre of the mass. These features were constant in all the cases studied. An accompanying secondary re­tinal detachment showed the usual characteris­tic ultrasonographic features.

Ophthalmoscopic shrinkage of the tumour was seen in 9 eyes. It was complete in 5 and partial but significant in the remaining. Smaller masses disappeared completely while the larger ones persisted. Some developed a fibrous envelope around them. 4 of the 9 eyes were treated with the laser, 3 with the xenon, I with both laser and xenon and another with cryo.

Smaller masses regressed more readily than larger ones. Signs of regression of exudates were observed in two eyes-one of whicn had cryo and the other both xenon and laser, Secondary retinal detachment had subsided totally in both the eyes treated with cryo and partially in one treated with the laser. In 2 eyes there was neither regression of the mass nor improvement in the secondary retinal detach­ment. These tumours were very large (more than 10 DD) and had been treated with laser. Fluorescein angiography helped in document­ing the reduction in size of the tumour and its feeders in 3 eyes. Some degree of dye leakage persisted in 2 eyes and there was none in I. Visual acuity was maintained in 11 eyes where treatment was considered successful in 8, and unsuccessful in 3. 2 eyes showed an improve­ment due to absorpsion of vitreous haemor­rhage. A retinal detachment with extensive preretinal membrane developed in I eye follow ing xenon arc photocoagulation causing re­duction of vision and loss of the eye. The other complications with photocoagulation were a minor bleed from the mass in 2, secondary re­tinal detachment in 1. They were of no con­seqaence and needed no special attention.


  Discussion Top


The predilection for the angiomas to be situated on the temporal side is very significant. [Table - 2] shows that the complications that an angioma can cause is related to its number and size. Small but multiple angiomas and large solitary angiomas had invariably been associate with complications except for case 1. Larger angiomas (more than 2 DD) were most often seen to be associated with a secon­dary retinal detachment, irrespective of the number of tumours. The 1 rger the tumour the more extensive was the retinal detachment.

Fluorescein angiography may be considered unnecessary by some but it has shown us certain interesting features. In eyes where the pictures were centered initially around the mas; and its feeders, we found the filling to be slow and steady. This is to be expected as the column of blood or fluorescein passing through a dilated channel with other features remaining constant is likely to be slower if at all than normal. The usual concept is that a retinal angioma fills rapidly. This notion arises pro­bably from the fact that there is always increa­sed fluoresceince from the dilated feeder and the mass which is simply due to the quantita­tive increase in fluorescein within these struc­tures. The minimal leakage of dye in two cases show that the endotheliah in these instances was fairly competent. Both these patients had a secondary retinal detachment and I had ex­udation in addition showing thereby that fluorescein leakage is not always equivalent to leakage of material from within the vascular tumour. May be time duratton also counts. We were able to observe that the dye had emptied fairly rapidly from one of these tumours demonstrating the absence of stasis within. The capillary drop out and compensa­tory capillary dilatation around tumour which we have observed seems to have received very little attention before Angiomatosis retinae thus shares a common feature with the other vascular retinopathies. Fluorescein angiography helped in establishing the diagnosis of angioma in two suspicious cases. This could be impor­tant when following up of a fellow eye in a patient with retinal angioma in the other. The consistent features observed in A&B scan ultrasonography will possibly us to pick up these lesions in an eye when the media is opaque and help us make a more definitive diagnosis However the mass has to be poster­iorly situated when using a contact scanner. Both photocoagulation and cryo therapy have been equally effective in causing a shrinkage of the mass and there has been no significant diff­erence between the argon laser and the xenon are. The bleed during photocoagulation was fortunately minimal and without consequence. However, one should always be aware of the fact that a massive bleed with its sequlae is a distinct possibility. This and the occurence of an extensive preretinal membrane with retinal detachment inspite of regression of the masses calls for the greatest caution while treating these lesions. The masses which responded only partially or did not respond at all were the lar­ger ones-indicating that treatment is more like­ly to be successful when the mass is still small. We expect the eyes in which vision has been maintained and where treatment has been con­sidered successful to maintain their vision in the long run. These are eyes which are likely to have deteriorated if they had been left alone We feel that there is a definite need for treat­ing uncomplicated lesions in an eye when the fellow eye has been lost by the same disease and lesions which have produded retinal deta­chment, exudation or vitreous haemorrhage. Photocoagulation (xenon or argon laser) and cn o when properly applied give good re­sults.


  Summary Top


Twelve cases of Angiomatosis Retinae are presented. The detailed ophthalmoscopic features in all and the fluorescein angiographic and ultrasonogr iphic features in a selected few are discussed. The techniques, results, compli­cations and the need for treatment are outlined.


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]
 
 
    Tables

  [Table - 1], [Table - 2]



 

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