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Year : 1971  |  Volume : 19  |  Issue : 2  |  Page : 77-79

Xanthomatosis as a cause of unilateral proptosis

1 Department of Ophthalmology, Irwin Hospital and Maulana Azad Medical College, New Delhi, India
2 Department of Pathology, Irwin Hospital and Maulana Azad Medical College, New Delhi, India

Correspondence Address:
Hari Mohan
Department of Ophthalmology, Irwin Hospital and Maulana Azad Medical College, New Delhi
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Source of Support: None, Conflict of Interest: None

PMID: 15744974

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How to cite this article:
Mohan H, Sen DK, Chatterjee P K. Xanthomatosis as a cause of unilateral proptosis. Indian J Ophthalmol 1971;19:77-9

How to cite this URL:
Mohan H, Sen DK, Chatterjee P K. Xanthomatosis as a cause of unilateral proptosis. Indian J Ophthalmol [serial online] 1971 [cited 2021 Jun 14];19:77-9. Available from: https://www.ijo.in/text.asp?1971/19/2/77/34987

It is very unusual to come across localized xanthomatosis in the orbit. So much so that it is hardly considered as one of the causes of unilateral proptosis. In our ophthalmic practice over a period of six years in a busy metropolitan hospital we came across 128 cases of unilateral pro­ptosis, 3 of which were found to be caused by xanthomatosis. Two of the cases were females and one was male. All belonged to the age group 20-25 years. There was no history of trauma, no demonstra­ble septic foci in and around the orbit, no hypercholesterolaemia or any other generalized distur­bances in any of the cases. The lesions were characterized by the presence of fat-laden histiocytes ("xanthoma Cells"), chronic infla­mmatory cells and varying amounts of fibrous tissue. In the case recorded below the histolo­gical picture is representative of such lesions. Presence of plenty of cholesterol cleft was the addi­tional feature which evoked con­siderable interest as to the etio­logy of such lesions.

Case Report:­

A 25-year-old, Hindu, female reported to the eye out-patient department with the complaints of a painless swelling in the re­gion of the left lower lid for 6 months which was gradually in­creasing in size. There was no history of trauma. On examina­tion: Right eye and its adnexa were found normal. Left eye, vision 6/9 correctable to 6/6 with glasses. There was an ob­vious bulge of the lower portion of the lower lid. The globe was moderately displa­ced upwards. There was some restriction of ocular movement on looking down. A firm infrabul­bar mass in the anterior portion of the orbit was palpable through the lower lid which was not ten­der. There was no abnormality in the globe itself. Fundus was normal. Skiagrams of the orbits and paranasal sinuses were nega­tive. General physical examina­tion disclosed no abnormality. Urine and blood chemical and serological studies were also nega­tive. Serum cholesterol was 195mg. per cent. The mass was completely removed through an incision along the lower orbital margin and sent for histopatholo­gical examination. Post-operative period was unventful. Follow-up studies for two and half, years did not reveal any recurrence of the lesion.

Histopathological examination revealed a greyish-white mass 2.5 X 2.5 X 2cm. in size, and firm to feel. Sectioned surface had a variegated appearance. On micro­scopy essentially the lesion was granulomatous with abundant amount of chronic inflammatory cells, fat-laden histiocytes ("xan­thoma cells") and clusters of cholesterol clefts in the midst of mononuclear cells [Figure - 1]. These were rimmed at places by multi­nucleated giant cells. The xan­thoma cells were having swollen foamy cytoplasm, round to oval vesicular nuclei centrally or eccentrically placed [Figure - 2]. At­tempt of fibrocollagenous walling off was noted in some areas. No evidence of haemorrhage or necrosis was noted. The lesion was negative for acidfast bacillus and fungus.

  Comments Top

Cholesterol containing granu­loma of the type described here belong to the group of xanthoma­tosis. As such lesions in the orbit present with the clinical features of orbital tumour, the diagnosis can be established only by histo­pathological examination. The etiopathogenesis of these lesions is not well understood. As the lesion is essentially characterized by the presence of fat-laden histiocytes in abundance, it was thought by many to represent disorders of fat metabolism or storage. Loca­lized nature of the lesion and its unassociation with hypercholeste­rolaemia, however, do not favour this theory. Presence of chronic inflammatory cells suggest that some low-grade infective process or trauma may be responsible for initiating the process and acquisi­tion of fat by the histiocytes is secondary. Low-grade infective process is known to cause irrita­tive proliferation of the histiocy­tes. Trauma may as well initiate the process by causing haemorr­hage, which decomposes with the release of blood pigments which in turn may cause irritative pro­liferation of the histiocytes and granuloma formation (Ramsey Laws, Pritchard and elliot [1] )

As there was no history of trauma and no evidence of haemo­rrhage histopathologically we presume that the underlying cause of the lesion is in all proba­bility an unknown infectious agent as suggested by Reese [2].

  Summary Top

Localized xanthomatosis was found to be the cause of unilate­ral proptosis in 3 out of 128 cases. In the case recorded there were plenty of cholesterol celfts in addition to fat-laden histiocytes, chronic inflammatory cells and varying amount of fibrous tissue. The underlying cause of the lesion could not be established. The probabilities are discussed.

  References Top

RAMSEY, G. S.: Laws, H. W.: Pri­tchard, J. E. and Elliot, H. Post­tracmatic granuloma of the bony Orbit Similating tumour. Canad, M.A.J., 59, 206 (1948).  Back to cited text no. 1
REESE, A. B. (1963). Tumours of the eye, Ed. 2. p. 544, New York, Hoeber.  Back to cited text no. 2


  [Figure - 1], [Figure - 2]


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