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Abstract
Pai syndrome is a rare disorder, first described in 1987. Diagnostic criteria is 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.
REFERENCES
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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.
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).
Table 1.
Clinical Summary of Pai syndrome Patients Described in the Literature1-9 and Our Patient
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