Lymphomatous pseudo-tumour of the choroid with secondary retinal detachment

K Ananda Kannan

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Year : 1975 | Volume : 23 | Issue : 4 | Page : 28-31

Lymphomatous pseudo-tumour of the choroid with secondary retinal detachment

K Ananda Kannan
Govt. Ophthalmic Hospital, Egmore, Madras-600008, India

Correspondence Address:
K Ananda Kannan
Govt. Ophthalmic Hospital, Egmore, Madras-600008
India

Source of Support: None, Conflict of Interest: None

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PMID: 1031155

How to cite this article:
Kannan K A. Lymphomatous pseudo-tumour of the choroid with secondary retinal detachment . Indian J Ophthalmol 1975;23:28-31

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Kannan K A. Lymphomatous pseudo-tumour of the choroid with secondary retinal detachment . Indian J Ophthalmol [serial online] 1975 [cited 2021 Apr 18];23:28-31. Available from: https://www.ijo.in/text.asp?1975/23/4/28/31436

A rare case of pseudotumour arising probably from the choroid and producing detachment of retina with proptosis which responded well to radiotheraphy is presented herewith.

Case Report

Mr. V. (44 years) was admitted with the complains of defective vision on left eye of one year duration. Proptosis and pain in the same eye of six months duration. On admission, there was acute pain in the left eye. There was history of rise of temperature in evenings but no cough.

On examination-left eye-there was down and in proptosis [Figure - 1]. Conjunctiva was congested and cornea clear. There were firm globular swellings underneath the conjunctiva in the upper nasal [Figure - 2] as well as in the lower temporal quadrants [Figure - 3]. It was tender with dilated tortuous vessels see i over the masses. Anterior chamber was normal. Pupil was dilated and not reacting to light. Upward and lateral movements were restricted.

Exophthalmometry (Hertel's) R.E. 13 m.m. L.E. 22 m.m. Base reading 100 m.m.

Visual acuity-Perception of light.

Fundus : There was extensive detachment of retina of about 20 D. in the lower part and 5 to 6 D. above and nasally. Disc was not seen. Plenty of dark brown pigmentation was seen scattered over the retina.

Transillumination-Transilluminant all around, Right Eye was normal; visual acuity was 6/9, Fundus was normal.

Tension (Schiotz)-R.E. 17.3 m.m. Hg. L.E. 30.4 m.m.Hg. Gonioscopy : R.E.-Angle open. L.E.-Angle was narrow but open.

Slit lamp examinations : L.E.-No flare and cells was seen. Retina was seen lying behind the lens with plenty of brownish pigmentation.

Investigation

Investigation were noncontributory.

A provisional diagnosis of scleritis with secondary retinal detachment was made and the patient had a total dose of 800 i.u. of A.C.T.H, by i/v drip over 15 days in divided doses. Proptosis regressed by 3 m.m.; retinal detachment reduced by 10 D. The disc was not seen yet. Visual acuity improved to hand movement.

Subsequently, she had Streptomycin injection and I.N.H. emperically for 1 month with oral prednisolone 20 mgms. a day. Three months after admission there was proptosis of only 4 m.m. The episcleral masses became more prominent, tender and covered with dilated vessels extending behind the fornices. Fundus picture remained the same with increase of pigmentation scattered over the retina. Visual acuity improved to counting finger 1 metre in the nasal field.

Biopsy of the episcleral tissue was taken under local anaesthesia on. On incising the conjunctiva, shiny homogenous episcleral tissue of several m.ms. thickness was seen. Sclera appeared normal underneath the masses. It was sent for histopathological examination. The report come as lymphoid cells of uniform or monomorphic cytology arranged in diffuse sheets with no follicular arrangement [Figure - 4] a & b. The impression was pseudo-tumour lymphoid type.

A dose of 4000 r of tele-cobalt 60 was delivered in a duration of 5 weeks. On completion of radiotherapy, the eye became normal in size [Figure - 5]; the episcleral masses disappeared; cornea was clear; pupil was middilated and reacting to light; movements were full.

Visual acuity was 6/60. Fundus should atrophic cupping of the disc; retina was flat all around. Extensive scars of healed chorioretinitis were seen.

Exophthalmoitetry (Hertel's)-Both eyes 13 mms. Base reading 100 mm. Tension (Schiotz)-R.E. 17 m.ms. Hg. L.E. 16 m.ms. Hg.

Bone marrow study was done to rule out generalised lymphoma which revealed a normoblastic reaction with hypoplasia; myeloid erythroid ratio decreas ed; mitosis seen; megakaryocytes decreased in number with absence of L.D. bodies. Lymphoblasts slightly increased along with plasma cells. The impression was either a lymphoma or plasmocytoma. An E.P.P. was done which was not in favour of plasmocytoma.

Discussion

Since the visual deterioration started early and the proptosis occured late, it is possible that the pseudo-tumour could have started at the posterior uvea producing an exudative detachment of the retina. The spread to the orbit is possibly through the direct contiguity, eroding the sclera or through the blood vessels thereby producing proptosis. The shiny masses at the episcleral tissues are the anterior extensions of the pseudo-tumour.

In a similar case of inflammatory pseudotumour of the uveal tract with retinal detachment and narrow angle glaucoma, Gass[3] described that the symptoms apparently related to an infiltration of the choroid and secondary macular detachment. This infiltration produced gradual thickening of the choroid and ciliary body and resulted in extensive retinal detachment. This was confirmed by the biopsy of the enucleated globe done at a later stage. He pointed out that some of the visual defects may be due to cellular infiltration of the optic nerve and the infiltration of the retrobulbar tissue was probably responsible for the proptosis.

On the other hand, in pseudo-tumour of the orbit primarily, the proptosis develops more rapidly; exudative retinal detachment may occur. An unusual complication is the formation of a similar inflammatory mass in the choroid. Regression usually takes place in 4 to 6 months and proceed to some degree of enophthalmos and the vision usually returns to normal. Recurrences do not appear.

Simple lymphomas are rare.[1],[2] It may occur at all ages in both sexes. 20% are bilateral.[5] Growth may be slow or rapid associated with oedema, limitation of movements and a mass in the orbit. Intraocular involvement is indirectly through papilloedema and mechanical damage to the nerve and directly by infiltration of the retina and uvea.[4] They may occur without apparent constitutional disease; the absence of general dissemination is only temporary.[6] Pseudo-tumour is classified into three categories:

(1) Malignant form.

(2) A benign form which is reactive or inflammatory showing formation of lymphoid folicles.

(3) In between the two showing pure lymphocytic proliferation; it responds typically to small amount of radiation.

Summary

A case of lymphomatous pseudo-tumour of benign hyperplastic type arising from the posterior uvea which responded very well to small amount of radiation has been discussed.

Acknowledgment

We wish to thank Dr. N.S. Venugopal, M.S., D.O., Superintendent, Government Ophthalmic Hospital, Madras for permitting us to publish this article. We wish to thank Dr. C.P. Gupta, F.R.C.S., for his valuable guidance. We are grateful to Dr. Murugesan, M.D., Professor of Pathology, Kilpauk Medical College and Dr. M. Jagadeesaa, M.D., D.M.R., Reader in Radiology, Barnard Institute of Radiology, Madras for their help and guidance.

References

1.	Aly Mortada., 1964, Amer.. J. Ophthal., 57, 820.
2.	Cook, C., 1954, Brit. J. Ophthal,, 38, 182.
3.	J. Donold M. Gass., 1967, Amer, J. Ophthal,, 64, 612.
4.	Harold Beasley., 1961, Amer. J. Ophthal., 51, 1294.
5.	Duke-Elder., 1952, "Text Book of Ophthalmology" Vol. V. p. 5451 & 5551-Henry Kimpton, London.
6.	Zimmerman L.E., 1964, "Ocular and Adnexal tumours" p. 429 Milton Bonink-The C.V. Moshy Company, Saint Louis.