|Year : 1971 | Volume
| Issue : 2 | Page : 77-79
Xanthomatosis as a cause of unilateral proptosis
Hari Mohan1, Dhan K Sen1, PK Chatterjee2
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
Department of Ophthalmology, Irwin Hospital and Maulana Azad Medical College, New Delhi
Source of Support: None, Conflict of Interest: None
|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 2020 Apr 1];19:77-9. Available from: http://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.
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|| |
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  )
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 .
| Summary|| |
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|| |
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).
REESE, A. B. (1963). Tumours of the eye, Ed. 2. p. 544, New York, Hoeber.
[Figure - 1], [Figure - 2]