|Year : 1970 | Volume
| Issue : 1 | Page : 25-28
Bilateral orbital involvement in multiple myelomatosis
BS Chohan, IPS Parmar, TD Chugh, AL Jain
Medical College and Hospital, Rohtak, Haryana, India
B S Chohan
Medical College and Hospital, Rohtak, Haryana
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Chohan B S, Parmar I, Chugh T D, Jain A L. Bilateral orbital involvement in multiple myelomatosis. Indian J Ophthalmol 1970;18:25-8
|How to cite this URL:|
Chohan B S, Parmar I, Chugh T D, Jain A L. Bilateral orbital involvement in multiple myelomatosis. Indian J Ophthalmol [serial online] 1970 [cited 2020 May 28];18:25-8. Available from: http://www.ijo.in/text.asp?1970/18/1/25/35056
Multiple myeloma is a bone tumour of multicentric origin characterised by pain, pathological fractures and anaemia. The bones commonly involved are the ribs, sternum, clavicle vertebral bodies, pelvis and occasionally the skull; long bones may be affected but never alone. Ocular involvement in multiple myelomatosis has been described in a small number of cases in ophthalmic literature. Clarke  (1953) reported 14 cases of orbital involvement in the disease.
Handousa, Rose and Taylor  , Kojima have reported a case each, but there was unilateral involvement of the orbit in them. Handousa (1955), reported a case of unilateral proptosis due to multiple myelomatosis having typical changes in the bones and Bence-Jones protienuria. Rose and Taylor (1957) reported another confirmed case of multiple myelomatosis involving the orbit. Another case of myeloma of the orbit was reported by Kojima (1962) in Japan. We recently came across a case of multiple myelomatosis with bilateral orbital involvement. The same is being reported.
| Case Report|| |
Tilak Ram, a male aged 45 years, was admitted to this hospital in October, '68, with gradually increasing swelling of the right orbit for 8 months prior to admission. Along with the appearance of the right orbital swelling, the patient experienced generalised weakness and pains in the whole of his body. Two months before admission he started feeling moderate intensity of pain in the left orbit also. The patient developed symptoms of acute confusional state during his stay in the hospital. He had small-pox infection at the age of 10 years when he lost his left eye.
General physical examination showed anaemia and marked emaciation of the whole body with generalised tenderness of the chest bones and the spine. Except in the right orbital region no other swelling was noticed anywhere on the body. Small-pox scar marks were seen sparsely distributed all over. No abnormality was detected in the cardiovascular, respiratory and central nervous systems and in the gastrointestinal tract. Pyorrhoea alveolaris with absence of teeth in the upper and lower jaws was present.
Examination of the eyes:
The right eye showed eccentric proptosis with forward, downward and inward displacement of the eyeball [Figure - 1]. The proptosis was noncompressible, non-pulsatile, with slight tenderness over its upper and outer limits. The colour of the skin over the lids and the neighbouring areas was normal. The proptosis was due to two almond shaped swellings situated in the superolateral and inferolateral orbital margins which were extending to the right temporal region. The skin over the swellings was mobile.
Mild pressure on the eyelids resulted in subluxation of the eye. Movements of the eyeball were restricted in upper, outer and lower directions. The conjunctiva showed moderate pale white chemosis. Slight xerosis of the lower 1/3 of the cornea was present. The pupil reacted sluggishly to light and accommodation. The examination of the fundus could not be done because of the marked haziness of the vitreous. The vision gradually deteriorated. The left eye showed enophthalmos due to phthisis bulbi resulting from small-pox involving the eye. The eyeball was completely shrunken and the cornea was totally leucomatous with Calcification over its surface. There was no tension in the eye. On eversion of the upper lid a small nodular swelling was visible in the left fornix. It was difficult to reach its posterior limit. The swelling was separate from the eyeball. The movement of the eyeball was negligible. There was no obvious displacement of the eye. The vision was completely absent.
The right orbital cavity was enlarged and there was extensive bony erosion of,-,the superior and lateral orbital margins with slight rarefaction of the orbital plate of the frontal bone. A small translucent punched out area was seen in the region of the greater wing of the sphenoid. The left orbit showed a large translucent area and rarefaction in the orbital plate of the frontal bone. A small bony erosion was observed near the superomedial margin. of the orbital rim. The cavity was normal in size. In the skull, generalised rarefaction with numerous rounded discrete translucent areas of varying sizes were shown [Figure - 2].
The examination of the spine, chest bones and the pelvis showed generalised rarefaction with characteristic multiple punched out translucencies. Few punched out osteolytic and rarefied areas were also seen in the long bones of the upper and the lower extremities. Pathological fractures were seen at the upper end of the left fibula and the 3rd lumber vertebra.
Haemoglobin was 8.00 gm%, total leucocytes 10,000/emm with a differential count of 65% polymorphs, 25% lymphocytes, 7% monocytes and 3% eosinophils. Peripheral blood film showed increased roulaux formation of red cells and a moderate degree of hypochrornia. No immature cells were seen. Platelets were normal in number and morphology. Bleeding and clotting time were within normal limits. Urine examination showed albuminuria (3 plus) and several hyaline and granular casts. BenceJones proteins were absent on repeated examinations. Total serum proteins were 7.5 gm% with albumin 3.4%, alpha, 0.35 gm%, alpha 2 0.35 gm%, Beta 0.2 gm%, Gamma 3.2 gm%. No myeloma proteins were seen. Serum calcium was 11.2 mgm%.
The biopsy material consisted of a few small soft tissue pieces. H. E. stained paraffin section showed sheets and groups of tumour cells ovoid to polygonal in shape with a moderate amount of cytoplasm, each having a single usually large and eccentric nucleus. Chromatin pattern in some of the cells showed a typical cartwheel arrangement. Occasional cells also showed a para-nuclear vacuole or nucleolus. A rare mitotic figure of binucleated cell was also seen.
Sternal marrow examination:
Cellularity was moderately good with a M. E. ratio of 3:1. Numerous myeloma cells and mature plasma cells either in clumps or sin.nly were see [Figure - 3]. The reaction was normoblastic.
Apart from supportive treatment in the form of repeated blood transfusions no other form of treatment was considered worthwhile in view of the extensive involvement of the skeletal system. Radiotherapy to the proptosed eye was also withheld considering the existing visual disability as a result of previous phthisis bulbi in the other eye. Operative interference was abandoned because of diffuse infiltration of myelomatous deposits in the orbits and deteriorating condition of the patient.
| Comments|| |
In view of the presence of eccentric proptosis with marked generalised cachexia and anaemia the possibilities of a malignancy with secondaries in the orbit and a primary growth of lacrimal gland were initially considered. Multiple myelomatosis was not thought of since the involvement of orbit in this disease is extremely rare. However, since the patient complained of generalised bone pains and as no evidence of a growth elsewhere was found on physical examination, skiagrams of the skeletal system were taken to exclude this disease. The x-rays revealed typical bone involvement of myelomatosis and the diagnosis was ultimately confirmed by other investigations.
The patient had enophthalmos of the left eye which had resulted from phthisis bulbi due to previous smallpox infection. Since there was no obvious swelling in this eye the involvement of the left orbit was missed initially. Moreover in none of the reported cases of myelomatosis, bilateral orbital involvement has been described. In the present case, however, subsequent examination revealed a small nodular swelling in the upper fornix which was confirmed on radiological and histopathological examinations to be due to myelomatous deposit. This therefore, probably is the only reported case in recent ophthalmic literature showing a bilateral orbital involvement.
| Summary|| |
Orbital involvement in multiple myelomatosis is rare and only seventeen cases have been reported in the available literature. A case with bilateral involvement of the orbit is reported.
| References|| |
Clarke, E. -- Plasma cell myeloma of the orbit. Brit. J. Ophthal., 37:543-554, (1953).
Handousa, A. -- Multiple myelomatosis with proptosis. Brit. J. Ophthal., 39 41-43, (1955).
Kojima, K; Shimizus; Awaya, S; Tanbat: Takayanagi -- A case of orbital myeloma. J. Clin. Ophth. (Tokyo)., 16:51-55, (15th. Jan. 1962).
Rose and Taylor.,--- Multiple myelomatosis affecting the orbit. Brit. J. Ophthal. 41 : 438-439, (1957).
[Figure - 1], [Figure - 2], [Figure - 3]