Multiple myeloma is a plasma cell dyscrasias, associated with the neoplastic proliferation of plasma cells and their precursors. Orbital plasmacytomas are the localised forms, arising in the orbit and comprise 3% of all orbital tumours. Diplopia and ptosis result from interference of mechanical function of the orbital muscles. We present an unusual case of multiple myeloma, where the only initial symptoms were ptosis and diplopia, secondary to mechanical restriction of the ocular muscles (superior rectus and levator palpebrae superioris)
A 63-year-old female, with no significant ophthalmic history presented with drooping of her right eyelid and diplopia of 4 months duration. Examination revealed right upper lid ptosis, normal visual acuity (6/5) in both the eyes, diplopia in all directions of gaze, vertical muscle imbalance and limitation of elevation in the right eye. MRI of the brain demonstrated multiple calvarial lesions with the largest measuring (4.4×2.7×2.8 cm) in the right frontal bone (Figures 1–3). The intra-cranial component of this lesion extended into the extracerebral space, compressed the anterior part of right frontal lobe, extended inferiorly to the right orbital roof, orbital ridge, with intraorbital extension, compressing the superior rectus, levator superioris and indenting the superior margin of the globe.
Haematological investigations revealed normal cell counts with elevated ESR (>140) and biochemical parameters demonstrated reversed albumin/globulin ratio (4.3/4.7 gm). Serum electrophoresis revealed two bands in gamma globulin (possibly dimer and oligomer of IgA). Immune fixation electrophoresis showed two bands in IgA lane and two bands in Kappa lane, confirming the diagnosis of IgA Kappa monoclonal gammopathy. Quantitative estimation of serum immunoglobulins revealed IgA −2471 g/dL and elevated kappa −191.4 g/dL Bone marrow aspiration showed plasmacytosis (12%) and bone marrow biopsy confirmed plasma cell myeloma Patient was diagnosed to have IgA Kappa multiple myeloma ISS stage 1. She was managed with bortezomib, lenalidomide and dexamethasone chemotherapy. She attained very good partial response status and is at present on Lenalidomide maintenance.
Plasma cell tumours rarely affect the orbit and they comprise 3% of orbital tumours (1). In multiple myeloma, orbital involvement is a very rare finding. In four series of orbital tumours, incidence of such a presentation varied from 1 in 200 to 1 in 800 (2). Orbital plasmacytomas can be either primary or secondary. Secondary plasmacytomas are ocular manifestations of multiple myeloma and are more common and more aggressive than primary plasmacytoma. Primary and secondary plasmacytomas cannot be differentiated histologically. They can be distinguished clinically with appropriate diagnostic evaluation for systemic disease involvement (3).
Proptosis is the most common manifestation of orbital plasmacytomas (4). Ptosis, diplopia and eyelid swellings are the other common manifestations. Uncommon manifestations include orbital cellulitis (5), fungating eyelid lesions, Tolosa Hunt syndrome (6), orbital hemorrhage (7) and periorbital ecchymosis (8). Though diplopia and ptosis have been documented as manifestations in 23% and 13% of multiple myeloma patients respectively (9), there has been only one case report where these symptoms have been the sole initial manifestations. Multiple myeloma may cause ocular pathology by direct infiltration or as extramedullary plasmacytomas resulting in compression or displacement of tissues, by immunoglobulin light chain deposition in ocular tissues, or by causing hyperviscosity syndrome. In our patient, the ocular manifestations were due to direct infiltration by extramedullary plasmacytomas. A similar case of myeloma presenting with ptosis and diplopia as the sole presenting features due to frontal bone plasmacytoma has been previously reported by Galea et al. (10).
Conflicts of Interest: The authors have no conflicts of interest to declare.
Informed Consent: Written informed consent was obtained from the patient for publication of this Case report and any accompanying images.
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Cite this article as: Nambiar RK, Nair SG, Mathew SP, Manohar AT. Ptosis and diplopia—sole manifestations in multiple myeloma. Ann Blood 2017;2:9.