Resumo

MELLONI-MAGNELLI, L.F.; DE LA GARZA-GIACOMAN, R.; MARTINEZ-LEIJA, H.  e  GUZMAN-RODRIGUEZ, R.. First clinical case of Pai Syndrome in México. Cir. plást. iberolatinoam. [online]. 2015, vol.41, n.2, pp.183-189. ISSN 1989-2055.  https://dx.doi.org/10.4321/S0376-78922015000200010.

Described by the first time in 1987, Pai's Syndrome is considered a rare variant of the displasia fronto-nasal. It consists of the fault of the closing of the middle line and his signs are encephalo-craneo-facial. The findings of the syndrome are the presence of a variety of polyps (intranasal, cutaneous and alveolar of the upper jaw), bony and lip-palate cleft in the middle line, intracranial lipoma and partial or total agenesia of the corpus callosum. The etiology of this syndrome is not known, and its presentation is sporadic. The incidence is estimated in 1 of every 20.000-40.000 newborn children, being the most affected feminine sex. The aim of this article is to present the first clinical case reported in Mexico with these characteristics and that in agreement to the last case published in 2014 for Mee Hong, it is number 38 of the world literature.

Palavras-chave : Pay´s Syndrome; Nasal polyps; Intracraneal lipona; Agenesia of the corpus callosum; Median lip and cleft palate.

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