First Author: C.Azrak SPAIN
Co Author(s): J. Elvira Cruanes J. Pastor Bernier
Back to previous
Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a paraneoplastic ocular syndrome occurring in patients with systemic advanced carcinoma, Gass described five cardinal signs related to BDUMP: Multiple red patches at the level of the RPE, early hyperfluorescence of these patches on fluorescein angiography, development of multiple pigmented and non-pigmented uveal melanocytic tumours with diffuse thickening of the uveal track, exudative retinal detachment, and rapidly progressive cataracts. Patients affected by BDUMP have a short life expectancy. We highlight the need for appropriate index of suspicion and prompt evaluation. Treatment options for BDUMP are limited.
Ophthalmology service, Vinalopo University Hospital, Elche, Alicante, Spain.
We report a clinical case of a 52 year old female patient who presented to the primary care clinic with blurred vision, she refers central scotoma in both eyes, began 5 days ago, first in her right eye, then in her left eye. In her personal medical history: She is healthy carrier of hepatitis-B since 1999, laparoscopy secondary to infection from foreign body 1989. While in her family medical history: Her mother had cancer of colon. The corrected distance visual acuity (VA) was 0,4, slit lamp examination (SLE) showed mild facoslerosis, Pupils were 4mm and no afferent pupillary defect was observed, intraocular pressure (IOP) was 18 mmHg, multiple hypopigmentation spots were found in her dilated fundus examination in both eyes. We performed a optical coherence tomography (OCT) of the retina and we observed multiple zone of subretinal fluid and different areas of thickening of the retinal pigment epithelium. In her fundus autofluorescence angiography multiple hyperautofluorescence and hypoautofluorescence were detected. Electroretinogram was altered with a delay response of the photoreceptors.
We thought about paraneoplastic syndrome, bilateral diffuse uveal melanocytic proliferation (BDUMP) and we have referred the patient to internal medicine, Evaluation including a CT scan revealed lymphadenopathy involving lymph nodes. A primary tumour was not found, and the patient was given the diagnosis of carcinoma of unknown primary origin, the patient begin with 6 cycle of chemotherapy. We have performed serial ocular examinations to the patient each 3 monthes, including OCT and fundus autofluorescence angiography, With the course of the disease, the spots of hypoautofluorescence became larger and the external retina more damaged with plane subretinal fluid. The VA fluctuated around 0.4 and the patient refered that the scotoma become larger. The patient has suffered deep venous thrombosis and multiple infections. Unfortunately, The patient died 2 years after the onset of ocular disease.
Bilateral diffuse uveal melanocytic proliferation, although rare, is an intriguing clinical entity. This syndrome is recognized as a benign paraneoplastic syndrome involving the eye in patients whose underlying tumour is usually fatal within about 1 year. As ocular symptoms and signs often precede the onset of systemic problems, early recognition of this condition by an ophthalmologist may have an impact on patient morbidity and mortality. The clinical scenario to be aware of is abrupt bilateral visual loss in a person over 50 years of age with no clear underlying aetiology. Visual loss appears to occur by a combination of mechanisms including destruction of photoreceptors and underlying RPE, serous retinal detachment, Treatment options for BDUMP are limited. Modalities for treatment of the ocular findings include corticosteroids, surgery, external beam radiotherapy, and treatment of the underlying malignant neoplasm have been described, although the vital prognosis is poor. We could follow our patient and document the tests for approximately 2 years, with progressive worsening of the ocular signs and symptoms of the patient despite the implanted treatment for cancer.