Abstract
Neurofibromatosis type I (NF1) or Von Recklinghausen disease is one of the most frequent genetic diseases with polyvascular manifestations, including the eye. Its prevalence was variously estimated varying between 1/7800 and 1/2000. The diagnosis of NF1 is essentially clinical based on criteria established by the consensus conference of the National Institute of Health (NIH) in 1988. Several complications have been reported by numerous authors in cases of plexiform neurofibromas sometimes associated with congenital glaucoma. We report two cases of monocular blindness, complications of this disease in children, responsible for stigmatization by their peers and school dropout, seen at the National Hospital of Zinder, Niger.
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