|Year : 1955 | Volume
| Issue : 1 | Page : 13-16
Chloroma of the orbit
NS Jain, DV Sethi, OM Prakash
Department of Ophthalmology, Irwin Hospital, New Delhi, India
N S Jain
Department of Ophthalmology, Irwin Hospital, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Jain N S, Sethi D V, Prakash O M. Chloroma of the orbit. Indian J Ophthalmol 1955;3:13-6
Chloroma, a greenish neoplasm of high malignancy is a rare disease. The first description of the rapidly growing neoplasm with an invariably fatal termination was given in 1821 by Allen Burns. The disease was called Chloroma by King in 1853 because of its characteristic colour.
Whereas in the literature, it has been extensively described by several authors, Malkin (1925) described seventy-two cases affecting the orbit. Not many cases are recorded in the literature wherein chloroma presented itself solely in the form of a unilateral proptosis, thus bringing the patient to the ophthalmologist from the first.
| Case Report|| |
Patient, A. B., female, aged 5 years was brought to the Irwin Hospital on 19-12-'53 with the history of a rapidly increasing proptosis of the right eye for the last three weeks. Ophthalmic examination revealed the presence of an axial, irreducible and non-pulsatile proptosis of the right eye with slight limitation of the movements of the globe. There was a hard nodular mass palpable under the supraorbital and infraorbital margins. The skin of the eyelids was oedematous on both sides, although no proptosis of the left eye was present and no retrobulbar mass was palpable in the left orbit. Both fundi were normal.
General physical examination revealed an enlargement of spleen, liver and other lymphatic glands.
Progress of the Disease :- Seven days later i.e. on 26-12-'53 proptosis appeared on the left side and soon after an orbital mass was palpable under the orbital margins. By 31-12-'53 the proptosis had increased considerably on both the sides. On 4-l-'54 immobile shining swellings appeared in the temporal regions and on the glabella of the frontal bone. Fundus examination on this day revealed the presence of retinal oedema and venous engorgement but no hemorrhages or exudates.
For the first time on 11-1-'54 there was a severe bleeding from the right nostril of the patient, followed a few days later by slight anaemia, toxemia and irregular temperature rising to a maximum of 101.8°F. throughout the course of the disease. A few glands appeared in the posterior triangle and proptosis increased markedly on both the sides. Between the dates 20-1-'54 and 28-1-'54, the proptosis reached its extreme causing complete ocular immobility, skin of the eyelids became markedly swollen and tense, blood vessels were prominently traced out in the skin of the eyelids and the frontal and temporal swellings assumed large dimensions. Examination of the oral cavity showed marked induration and thickening of the upper and the lower alveolar margins and involvement of the hard palate causing its narrowing in general. In the nose there was a purulent rhInitis.
Fundus examination in the right eye now showed an advanced leukemic manifestation in the form of a considerable blurring and swelling of the optic disc, retinal oedema, marked dilatation and tortuosity of the veins. There were plenty of superficial flame shaped hemorrhages and soft wooly exudates in the vicinity of the discs and the blood vessels. In the left eye the changes were less marked.
Special Examinations : [Table - 1]
In the final stages, the patient had become markedly toxemic, anemic and semi-comatose. During her stay at the Hospital she was given three blood transfusions without any appreciable improvement. The relatives took the case home against medical advice on 9-2-'54 and a subsequent enquiry brought forth the information of her death seven days after discharge from the Hospital. Autopsy could not be undertaken.
| Discussion|| |
Aetiology of chloroma is not well understood. Age :- Healthy young children usually in the first decade are most commonly affected, the age incidence following a regular curve which rises from, moderate elevation in the first two years of life to a peak of highest incidence in the third and fourth years with a sharp decline in the next three years and more gradual fall in the latter half of childhood.
Sex:- Chloroma is said to be twice as common in males as in females-Kandel's 58 cases (1947).
Clinical Manifestations :- In our case the disease started in the orbit and a rapidly increasing exophthalmos was the first and most prominent feature till the end of the case. Multiple tumours over the skull and the temporal bones which became swollen producing the facies chloromatosa appeared late. In a case recently described by Misra and Mahapatra (1954), besides proptosis there was chronic suppurative otitis media, irregular temperature, marked anaemia, bleeding points and enlarged glands when the patient came under observation of the authors. Enlargement of glands, rise of temperature, extreme asthenia and exhaustion and development of a marked secondary anaemia, leukaemic blood picture with preponderance of immature blast cells occurred in the later stages. Our case did not apparently involve the paranasal sinuses though such is known to occur in chloroma. Fundus examination in the later stages showed papilloedema, marked retinal haemorrhages, exudates and venous engorgement. Corneal ulceration and optic atrophy did not have time to develop in our case.
Pathology :- Macroscopically at the time of taking the tissue from the neoplasm for purposes of biopsy, the neoplasm showed the typical yellowish green colour.
Whereas the neoplastic infiltration can be found in the abdominal viscera this could not be elucidated in our case. Local metastatic deposits in the subperiosteal region in the skull and facial bones are known to be present in about 75% of the cases and were present in our case.
As the tumour has a predilection for the skeletal system especially the bones of the skull and particularly the orbit, the bone-marrow becomes hyperplastic, greyish and liquified and predominantly shows an abnormal collection of myeloid and lymphoid cells in the primitive blast forms. In our case the blast cells were found to be above 50%. The primitive cells are held together by a fine reticular stroma, which is scanty but fairly vascular. The protoplasm and the intercellular tissue is greenish due to the pigment which fades on exposure to light. The nature of this pigment is not clear, some think it to be of lipoid origin (Chiari 1883), whereas others believe it to be hoematogenous and due to protoporphyrin, an intermediary product in the breakdown of haemoglobin to bilirubin (Thomas and Bigwood, 1935). Hydrogen peroxide characteristically restores the pigment in a fresh tissue and Scharlach red stain reveals a few minute fatty droplets in the cytoplasm of the cells resembling the earlier myeloid cells. Goodman and Iverson believe that in the distribution of the cells chloroma is related to myelogenous leukaemia as lymphosarcoma is to lymphatic leukaemia.
Being a neoplasm of high malignancy, the progress of the disease is very rapid and invariably fatal in a few weeks to a few months. No treatment is of any avail.
| Summary|| |
A case of Chloroma in the orbit is described. The aetiology, clinical manifestation, pathology and clinical course of the disease are reviewed with special reference to the case described.
| References|| |
Burns, Allen (1821)--Observations on the Burg. Anat. of the Head & Neck, 386.
Chiari (1883)-Prag. z, f. Heilk.. 4, 177.
Goodman, E. G. & Iverson, L. (1946)-Am. J. M. Sc. 211 : 205-214.
Kandel, E. V. (1947)-Arch. Int. Med.. 59 : 691.
King, A. (1853)-Monthly J. Med. Se. 17 : 97.
Malkin (1925)-Klinik Mon. Augen. 74 : 113.
Misra, M. C. & Mahapatra, G. S. (1954)-J. Ind. Med. Assn. 23 :
Thomas & Bigwood (1935)-C. R. S. Biol., cxviii, 381.
[Figure - 1], [Figure - 2]
[Table - 1]