[Isolated palpebral tumor revealing Von Recklinghausen's neurofibromatosis: a case report].
BACKGROUND
Von Recklinghausen's neurofibromatosis is the most frequent of phakomatoses. Its incidence is 1/2,500-3,000 births. It is inherited through a dominant autosomal mode with a strong prevalence and changing expression. Eye and skin signs are essential to diagnosis and this case provided a noteworthy example of a unilateral upper palpebral tumor revealing this disease.


METHODS
We report a case of a 6-year-old boy whose original clinical manifestation was a tumor on the upper eyelid of the right eye, present at birth. According to the anamnesis, this palpebral tumor gradually increased in size, causing a full ptosis. A biopsy exeresis of the tumor was carried out with an anatomicopathological study of the biopsy specimen, confirming the diagnosis.


CONCLUSIONS
The anatomicopathological study of the biopsy specimen enabled diagnosis of a neurofibromatosis revealed by this isolated palpebral localization responsible for significant aesthetic and functional damage.
Fany
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A
Service d'Ophtalmologie, CHU Treichville, 18 BP 160 Abidjan 18, Côte d'Ivoire, France.
Gbe
K
K
Konan-Toure
M L
ML
Adjorlolo
C
C
Koffi
K
K
Coulibaly
F
F
Boni
S
S
D001706:Biopsy; D001763:Blepharoptosis / Q000209:etiology; D002648:Child; D005142:Eyelid Neoplasms / Q000151:congenital / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D005143:Eyelids / Q000473:pathology; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery