Pai syndrome

Pai syndrome is an idiopathic developmental disorder characterized by median cleft of the upper lip (MCL), midline polyps of the facial skin and nasal mucosa, and pericallosal lipomas. Hypertelorism with ocular anomalies are also observed, generally with normal neuropsychological development.

Median cleft lip

A type of cleft lip presenting as a midline (median) gap in the upper lip.


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PMID (PMCID)
25359433
 FEMALE Infant, Newborn
Pre- and postnatal imaging of Pai syndrome with spontaneous intrauterine closure of a frontal cephalocele.
Dobrocky T, Ebner L, Liniger B, Weisstanner C, Stranzinger E.
Pediatr Radiol. 2015;45(6):936-40.
Pai syndrome is a rare congenital disorder characterized by cutaneous polyps of the face, pericallosal lipoma and median cleft lip.
23610859
 MIXED_SAMPLE Infant, Newborn
Prenatal detection of Pai syndrome without cleft lip and palate: a case report.
Ocak Z, Yazicioglu HF, Aygun M, Ilter MK, Ozlu T.
Genet Couns. 2013;24(1):1-5.
Pai syndrome is a rarely encountered disease characterized by findings of median cleft lip, facial skin polyps, nasal mucosal polyps and midline central nervous system lipoma.