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Abstract

RÉSUMÉ
Neurofibromatosis type I (NF1) or Von Recklinghausen disease is one of the most common 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 in children that were complications of NF1 and that were at the origin of stigmatization by their peers and school dropout. Both cases were seen at the National Hospital of Zinder, Niger.


ABSTRACT
La neurofibromatose de type I (NF1) ou maladie de Von Recklinghausen est l’une des maladies génétiques les plus fréquentes à manifestations polyviscérales, l’œil y compris. Sa prévalence était diversement appréciée variant entre 1/7800 à 1/2000. Le diagnostic de la NF1 est essentiellement cliniques basé sur des critères établis par la conférence de consensus de la National Institute of Health (NIH) en 1988. Plusieurs complications ont été rapportées par des nombreux auteurs en cas de neurofibromes plexiformes parfois associés au glaucome congénital. Nous rapportons deux cas de cécité monoculaire chez des enfants, complications de la NF1 et responsables d’une stigmatisation par leurs pairs avec abandon scolaire. Les deux cas ont été vus à l’Hôpital National de Zinder, Niger.

Keywords

Neurofibromatosis type 1, plexiform neurofibroma, monocular blindness, congenital glaucoma, child, Zinder, Niger

Article Details

How to Cite
Laouali Laminou, Hassane A Traoré, Abba Kaka Y, & Amza Abdou. (2022). Monocular Blindness and Plexiform Neurofibromas in Association to Neurofibromatosis Type 1: A Report of Two Pediatric Cases. HEALTH SCIENCES AND DISEASE, 24(1). https://doi.org/10.5281/hsd.v24i1.4111

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