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

被引：14

作者：

Vaccarella, F. ^{[1
]}

Prato, A. Pini ^{[1
]}

Fasciolo, A. ^{[1
]}

Pisano, M. ^{[1
]}

Carlini, C. ^{[1
]}

Seymandi, P. L. ^{[1
]}

机构：

[1] Osped Infantile, Azienda Osped Nazl SS Antonio & Biagio & Cesar Ar, Dept Pediat Surg, I-15100 Spalto Marengo, Alessandria, Italy

来源：

INTERNATIONAL JOURNAL OF ORAL AND MAXILLOFACIAL SURGERY | 2008年 / 37卷 / 11期

关键词：

D O I：

10.1016/j.ijom.2008.06.007

中图分类号：

R78 [口腔科学];

学科分类号：

1003 ;

摘要：

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.

引用

页码：1059 / 1064

页数：6

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