Progressive Bilateral Proptosis Research Articles

A possible case of Erdheim–Chester Disease

Aim: To present the case of a 57-year-old male with progressive bilateral proptosis. Material and method: The patient presented with bilateral proptosis and strength deficiency on the upper limbs. During hospitalization, the progression was unfavorable; proptosis progressed, causing a severe loss of vision in the left eye (from 0.8 Snellen to NLP). Results: The imagistic investigation revealed bilateral infiltration of the orbits, infiltrative lesions to the mediastinum and the abdomen. The patient was referred to neurosurgery for further management. Surgical orbital decompression was performed with biopsy. The histopathological examination revealed non-Hodgkin small cell lymphoma. Conclusions: Assembling the clinical and paraclinical data we have suspected the possible diagnosis of Erdheim-Chester disease, however, positive diagnosis has not been achieved.

Exophtalmie bilatérale pseudo-tumorale révélant un syndrome hyper-IgG4 orbitaire

Hyper-IgG4 syndrome is a rare cause of bilateral proptosis. It must always be considered after ruling out orbital lymphoma. We report a case of progressive bilateral proptosis for 4years in a 34-year-old man. Orbital MRI showed an infiltrative process extending to the orbital fat, extraocular muscles and lacrimal glands. Lacrimal gland biopsy with immunohistochemical study showed a lymphoplasmocytic infiltrate rich in IgG4 and fibrosis. The diagnosis of orbital hyper-IgG4 syndrome was suggested. The patient responded well to systemic steroid treatment. Orbital hyper-IgG4 syndrome manifests most often as pseudo-tumoral bilateral proptosis. Elevated IgG4 levels are neither sensitive nor specific. Biopsy with immunohistochemical study is the key to diagnosis. Systemic steroid treatment is the gold standard, but recurrences may occur.

Neglected primary Ewing′s sarcoma of ethmoid presenting as surgical emergency

We present a male child with primary Ewing's sarcoma arising from ethmoid sinuses with intradural and extracranial extension (bilateral nasal cavities, orbits, and maxillary sinuses). This is a rare condition. He presented with recurrent episodes of epistaxis for 2 years, sudden onset rapidly progressive bilateral proptosis, with painful restriction of extraocular movements, and decreased visual acuity for 4 days. Sudden complete loss of vision following admission demanded emergency tumor decompression.

Multiple myeloma presenting as bilateral orbital proptosis

A 58-year-old-man presented with painful rapidly progressive bilateral proptosis with restricted ocular movements of 15 days duration. There was history of significant weight loss in the recent past. Computed tomography scan of the head and orbit revealed bilateral multiple, well-defined, round, soft tissue masses, isointense with muscles in intraconal and extraconal space. Fine needle aspiration cytology and incision biopsy from the lesion, urine for Bence-Jones proteins and immunofixation clinched the diagnosis of multiple myeloma. Skeletal survey did not reveal any bony involvement.The diagnosis of multiple myeloma should be kept in mind in cases of bilateral proptosis. Bony involvement is not universal in cases of orbital myeloma. Early diagnosis can be established with extensive biochemical and histopathological investigations and timely treatment is life saving for these patients.

Atypical presentation and diagnostic pitfalls: A case of rapidly progressive bilateral proptosis in a child aged 18 months

We report an atypical presentation of non-Hodgkin lymphoma (NHL) in a child aged 18 months who presented with rapidly progressive bilateral proptosis. Computerized search using Medline did not reveal a similar presentation of NHL in such a young child. It stresses the need for an early histopathological study including immunohistochemistry and demonstrates the dramatic local response to combined radiotherapy and chemotherapy even in advanced stages without any ocular side-effects.

MYELOID SARCOMA PRESENTING WITH BILATERAL PROPTOSIS AND KIDNEY INFILTRATION

The authors describe a male infant with a history of transient pancytopenia who developed progressive bilateral proptosis associated with diffuse infiltration of the kidney and normal bone marrow. The histopathological examination of the kidney revealed diffuse infiltration of cells of myeloid origin with monocytic differentiation. Although orbital involvement by myeloid sarcoma, with or without concurrent acute myeloid leukemia, is well known, there are distinctive features in this patient that are not reported in the literature, namely bilateral proptosis and simultaneous presence of bilateral kidney infiltration, which enabled diagnosis.

Bilateral Proptosis, Ptosis, and Vision Loss

Abstract: A slowly progressive bilateral proptosis, chemosis, and vision loss are protean manifestations of Graves ophthalmopathy (GO). We describe a patient who had an indolent course with such manifestations and was diagnosed with GO. However, vision loss resulting from central retinal artery occlusion, thickening of the belly, as well as tendons of extraocular muscles on computed tomography and worsening with methyl prednisolone therapy defied the diagnosis of GO. It turned out to be mucorales invasion in rhinoorbital tissues in an immunocompetent subject.

Adult onset primary bilateral orbital xanthogranuloma: Clinical, diagnostic, and histopathologic correlations

Juvenile or infantile xanthogranuloma is a known entity with dermatologic, ocular and systemic involvement. The orbital form is quite rare and is usually associated with other systemic manifestations. Isolated bilateral orbital involvement is known to occur in adults in association with generalized bone involvement and other organ systems as part of the Erdheim-Chester disease syndrome. Primary adult onset bilateral orbital xanthogranuloma not associated with other organ system involvement or dermatologic manifestations, is unusual and should be distinguished from other chronic orbital disorders, such as inflammatory pseudotumor, low grade lymphoma, and infectious orbitopathies. The authors present a case of primary adult onset bilateral orbital xanthogranuloma in a 38-year-old female with painless, progressive bilateral proptosis of 16 years' duration. To our knowledge this is the first case of its kind reported in the ophthalmic literature.