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Eur J Ophthalmol. 2011 Nov-Dec;21(6):837-40. doi: 10.5301/EJO.2011.8317.

Intraocular neovascularization associated with choroidal ganglioneuroma in neurofibromatosis type 1.

Author information

1
Department of Ophthalmology, Hokkaido University Graduate School of Medicine, Hokkaido, Japan.

Abstract

PURPOSE:

Neurofibromatosis type 1 (NF-1) complicates various tumors originating from the neural crest. The authors report a case of NF-1 presenting with globe enlargement and subretinal and choroidal neovascularization associated with choroidal ganglioneuroma.

METHODS:

A 7-year-old boy with NF-1 had severe ocular pain OS. He has a medical history of congenital glaucoma OS. Left eye showed increased intraocular pressure, buphthalmos, corneal opacity, hyphema, and vitreous hemorrhage. Magnetic resonance imaging indicated prominent vitreous hemorrhage and a choroidal tumor. Enucleation was consequently performed OS.

RESULTS:

Histopathologic diagnosis of the choroidal tumor was ganglioneuroma admixed with microvessels and melanocytes. There was a huge subretinal and choroidal fibrovascular membrane formation originating from the choroidal ganglioneuroma.

CONCLUSIONS:

Severe ocular pain in this patient was caused by intraocular neovascularization and subsequent increased eyeball content by vitreous hemorrhage associated with the choroidal tumor. In NF-1 with orbitofacial involvement, detection of intraocular tumor by imaging tests may predict tumor-associated severe ophthalmic complications.

PMID:
21574161
DOI:
10.5301/EJO.2011.8317
[Indexed for MEDLINE]

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