'Pai Syndrome' with anterior alveolar polyp: A variant of a rare clinical entity.

ABSTRACT

'Pai syndrome' (PS) is a rare congenital syndrome. Presented here, a new-born baby-girl who exhibited the characteristic features of having a midline nasal (septal) polyp, an anterior alveolar process polyp, and a pericallosal lipoma associated with corpus callosum dysgenesis of the brain. Both polyps were lined with stratified-squamous epithelium. The overall features were largely consistent with those described by Pai et al., in 1987. A midline cleft-lip (with or without cleft-alveolus) is one of the most common features of the syndrome which was however absent in this case. Instead, an anterior alveolar polyp is present, which is relatively rare.