Korean J Perinatol.  2014 Mar;25(1):17-21. 10.14734/kjp.2014.25.1.17.

A Case of Pai Syndrome: First Reported Case in Korea

• Hong M
• Nam SW
• Lee YK
• Ko SY
• Shin SM

• KMID: 1917228
• DOI: http://doi.org/10.14734/kjp.2014.25.1.17

Abstract

Pai syndrome is a rare disorder, first described in 1987. Diagnostic criteria are the presence of the nasal polyp and one of the following: midline cleft lip, congenital polyp of mid-anterior alveolar process, and pericallosal lipoma. Thirty-six cases of Pai syndrome have been described so far. We report 1 case of Pai syndrome accompanied by congenital nasal polyp and callosal lipoma with partial agenesis of corpus callosum, the first time in Korea.

Keyword

Pai syndrome; Nasal polyp; Pericallosal lipoma

MeSH Terms

Agenesis of Corpus Callosum
Alveolar Process
Cleft Lip
Korea
Lipoma
Nasal Polyps
Polyps

Figure

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  Fig. 1. Patient showing congenital nasal polyp (A) and post-excision status (B).

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  Fig. 2. Brain ultrasonogrphy: sagittal (A) and coronal (B) image showing lipoma (arrow) at body to tail portion of corpus callosum.

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  Fig. 3. Brain magnetic resonance imaging: sagittal (A) and coronal (B) T-1 weighted images showing lipoma in the body and tail portion of corpus callosum (arrow) and tail portion of the corpus callosum was replaced by the mass (arrow head).

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