Simultaneous Bilateral Diffuse Melanocytic Uveal Hyperplasia

Rohrbach, Jens Martin; Roggendorf, Wolfgang; Thanos, Solon; Steuhl, Klaus‐Peter; Thiel, Holger

doi:10.1016/s0002-9394(14)76937-5

Cited by 39 publications

(26 citation statements)

References 16 publications

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“…The histopathological changes of BDUMP have been well documented (Barr et al 1982;Prause et al 1984;de Wolff-Rouendaal 1985;Filipic & Ambler 1986;Gass et al 1987;Margo et al 1987;Rohrbach et al 1990;Borruat et al 1992). The uveal tract is diffusely thickened.…”

Section: Discussionmentioning

confidence: 97%

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Bilateral diffuse uveal melanocytic proliferation and uterine cancer. A case report

Ritland¹,

Eide²,

Tausjø

2000

Acta Ophthalmologica Scandinavica

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ABSTRACT.Purpose: To report a case of bilateral diffuse uveal melanocytic proliferation (BDUMP), a rare paraneoplastic syndrome causing visual loss in patients with systemic carcinoma. Results: A 70-year-old woman developed visual symptoms 13 months after surgery and local irradiation therapy for uterine cancer. Following bilateral external beam irradiation supplemented with subsequent drainage of subretinal fluid in the left eye, the visual acuity improved from 0.01 to 0.15 in this eye only. The visual acuity remained at this level until she died 4 1/4 years after the onset of eye symptoms. Conclusion: This is the fourth case that survived longer than 24 months after the onset of visual symptoms of the 22 previously reported cases with BDUMP. It demonstrates that radiotherapy may have a vision-preserving effect in this group of patients. The patient also developed two different paraneoplastic phenomena -a nephrotic syndrome before and BDUMP after treatment for uterine cancer.Key words: bilateral diffuse uveal melanocytic proliferation -drainage of subretinal fluid -irradiation therapy -paraneoplastic syndrome -uterine cancer -uvea. Acta Ophthalmol. Scand. Acta Ophthalmol. Scand. 2000: 78: 366-368 Copyright c Acta Ophthalmol Scand 1999. ISSN 1395-3907 B ilateral diffuse uveal melanocytic proliferation is a rare paraneoplastic ocular syndrome that causes visual loss in patients with systemic carcinoma. The main characteristics are: Multiple round, red patches at the level of the retinal pigment epithelium, a striking pattern of multifocal areas of early hyperfluorescence corresponding with these patches, proliferation of choroidal nevus-like lesions as well as diffuse thickening of the uveal tract, exudative retinal detachment, and rapidly developing cataract (Barr et al. 1982;Gass et al. 1990). In addition glaucoma, dilated episcleral vessels, iridocyclitis, shallow anterior chambers, ciliary body cysts, and iridodonesis have been reported (Murphy et al. 1997). The syndrome frequently causes visual symptoms prior to the presentation of the primary tumour (Gass et al. 1990) and is usually lethal within 12 to 24 months (Gass 1987). Case ReportA 70-year-old woman was treated for a uterine cancer stadium 1B with oophorohysterectomy and additional local irradiation. Before surgery the patient developed a nephrotic syndrome caused by a biopsy-proven membranoproliferative glomerulonephritis. After treatment the renal symptoms disappeared, and the nephrotic syndrome was interpreted as a paraneoplastic phenomenon.One year later she was referred for an eye examination at the local hospital due to reduced vision, and the visual acuity turned out to be 0.5 in both eyes. Dilated episcleral vessels and a bilateral glaucoma with shallow anterior chambers were found. The glaucoma in the left eye was refractory to local antiglaucomatous treatment and systemic acetazolamid. YAG-iridotomy performed subsequently in the left eye revealed a substantial IOP-reduction. During the following months a rapid cataract development occurre...

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Section: Discussionmentioning

confidence: 97%

“…She died 4 1/4 years later, which is an unusually long survival time with BDUMP. Only three previous cases have survived beyond 24 months after onset of BDUMP (Rohrbach et al 1990;Gass & Glatzer 1991), with 6 1/2 years as the longest survival reported (Gass et al 1990). …”

Section: Discussionmentioning

confidence: 99%

Bilateral diffuse uveal melanocytic proliferation and uterine cancer. A case report

Ritland¹,

Eide²,

Tausjø

2000

Acta Ophthalmologica Scandinavica

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“…54,66 Electroretinographic studies have demonstrated a variety of abnormalities including unrecordable electroretinograms or a marked reduction in both cone and rod function. 56,58,60,62 Two further patients were found to have normal or slightly reduced cone function with a severe reduction in rod function. 51,65 In the majority of reported cases there has been an associated systemic malignancy which may have been diagnosed prior to the eye condition or which is found at the time of diagnosis as a result of a systemic examination.…”

Section: Melanocytomasmentioning

confidence: 98%

“…Since then, sporadic reports have appeared in the literature confirming this phenomena as a rare, but definite clinical entity. 51,[55][56][57][58][59][60][61][62][63][64][65][66][67] Gass 51 considered the syndrome clinically to have five cardinal features that accompanied the visual loss. These are: multiple round or oval, subtle red patches at the level of the retinal pigment epithelium in the posterior fundus; a striking pattern of multifocal areas of hyperfluorescence corresponding to these patches on angiography; the development of multiple slightly elevated, pigmented and non-pigmented melanocytic tumours ( Figure 7) together with diffuse thickening of the entire uveal tract; exudative retinal detachment; and rapidly progressive cataracts.…”

Section: Melanocytomasmentioning

confidence: 99%

“…Other features include conjuctival hyperaemia and/or dilated episcleral vessels, 51,54,55,62,65,67 shallowing of the anterior chamber 57 and ciliary body cysts, 55 iridodenesis, 56 uveitis 51 and raised intraocular pressure. 51,54,57,60,62,67 Extrascleral extension of the melanocytic lesion through the emissary channels into the subconjunctival space has also been observed. 54,66 Electroretinographic studies have demonstrated a variety of abnormalities including unrecordable electroretinograms or a marked reduction in both cone and rod function.…”

Section: Melanocytomasmentioning

confidence: 99%

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