Session Title: Free Paper Session 12: Imaging I
Session Date/Time: Friday 08/09/2017 | 14:30-16:00
Paper Time: 14:42
Venue: Room 111
First Author: : S.Luthra INDIA
Co Author(s): : V. Aggarwal S. Das N. Sanduja G. Luthra M. Luthra P. Mruthyunjaya
To describe two cases of the rare entity BDUMP (Bilateral Diffuse Uveal Melanocytic Proliferation) associated with Renal Cell Carcinoma and Non Small Cell Lung Cancer. The natural history of progression in one case due to refusal of patient for treatment is a contrast to the good response to plasmapheresis in the other case.
These were patients seen at the Retina Clinics of Drishti Eye Institute, Dehradun, India and Duke University Eye Centre, USA
A detailed clinical history was obtained and detailed examination was done. Then the patients were subjected to mosaic fundus photos (Topcon) or wide-field fundus imaging (Optos), Fundus Fluorescein Angiography (FFA) (Topcon) or wide-field FFA (Optos), swept source OCT (Topcon), Fundus Autofluorescence (FAF) (Heidelberg) or wide-field FAF (Optos), and ultrasound B Scan (Nidek). While one patient refused active intervention, the other received 9 cycles of plasmapheresis. The oncologist continued the systemic treatment for the primary malignancy. Both of the patients were serially followed up with documentation of their investigations.
The very similar clinical presentation with widespread RPE mottling at the posterior pole, starry sky appearance on FFA, multiple pockets of neurosensory elevation with fine undulations at RPE level on swept source OCT, and well-delineated extent of lesions showing mottled hypoautofluorescence with background hyperautofluorescence on FAF, and shallow retinal detachment on Ultrasound B Scan is described. While the patient who refused treatment showed progression to bilateral total exudative retinal detachments going on to develop neovascular glaucoma, the patient who received plasmapharesis showed significant continuous improvement with resolution of neurosensory elevations and significant improvement in visual acuity.
Bilateral Diffuse Uveal Melanocytic Proliferation (BDUMP) is a rare entity. It is a paraneoplastic ocular syndrome occurring in patients with systemic, often occult but advanced carcinoma. The ocular signs precede systemic manifestation of the malignancy by 3-12 months. BDUMP is a hallmark of poor prognosis, hence highlighting the need for a high index of suspicion and appropriate and prompt evaluation. Diagnosis of the occult primary malignancy can be challenging. Various treatment modalities have been described for BDUMP including corticosteroids, surgery, external beam radiotherapy and management of the underlying systemic malignancy. Recently plasmapheresis has been shown to have encouraging results for this condition. In our experience also the patient who accepted to undergo plasmapheresis showed a good response to treatment. However, since this entity is a hallmark of advanced systemic malignancy, the impact of early diagnosis of BDUMP on the overall prognosis and survival is debatable.