Phenotypic variability of Pai syndrome: report of two patients and review of the literature

F. Vaccarella, A. Pini Prato, A. Fasciolo, M. Pisano, C. Carlini, P. L. Seymandi

Research output: Contribution to journalArticlepeer-review

Abstract

Pai syndrome is a rare form of frontonasal dysplasia, first described in 1987. It is a triad consisting of midline cleft of the upper lip, facial skin polyps and central nervous system lipomas. Only 14 cases have been reported in the literature. The authors describe the clinical features, diagnostic workup and treatment of two patients. A review of all cases reported in literature is presented to show the phenotypic variability of this rare syndrome.

Original languageEnglish
Pages (from-to)1059-1064
Number of pages6
JournalInternational Journal of Oral and Maxillofacial Surgery
Volume37
Issue number11
DOIs
Publication statusPublished - Nov 2008

ASJC Scopus subject areas

  • Oral Surgery
  • Otorhinolaryngology
  • Surgery

Fingerprint Dive into the research topics of 'Phenotypic variability of Pai syndrome: report of two patients and review of the literature'. Together they form a unique fingerprint.

Cite this