Pai syndrome

"Synonyme: Cleft, median, of upper lip with polyps of facial skin and nasal mucosa

Pai syndrome is a rare disorder. It is characterized by mild hypertelorism, midline cleft lip, nasal and facial polyps, pericallosal lipoma and normal neuropsychologic development [1]. Other accompanying findings include clinodactyly, umbilical hernia, and cryptorchidism [2].

Not all the reported cases have midline cleft lip [3]. The case reported by Rudnik-Schoneborn and Zerres displayed a skin tag of forehead, downward slanting palpebral fissure, bifid uvula, and high plate with main elements of Pai syndrome [4].

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