Nasal and lip polyps: Pai syndrome.

Nasal glial heterotopias, or nasal gliomas, are rare congenital midline lesions composed of ectopic, non-neoplastic glial tissue. They are often mistaken for encephaloceles due to overlapping clinical and embryologic features. Accurate diagnosis is essential to avoid complications associated with surgical intervention. The authors present the case of a newborn female infant born at 35 weeks and 6 days of gestation with a midline nasal mass, respiratory distress, and multiple dysmorphic features. Prenatal and postnatal imaging revealed a pedunculated nasal lesion without intracranial extension and a corpus callosal lipoma suggestive of Pai syndrome. The mass was surgically excised on day of life 2, and tissue was sent for histopathologic evaluation. Pathology confirmed a benign fibroepithelial polyp with rare nerve fibers and negative GFAP staining, consistent with nasal glioma. Postoperatively, the patient exhibited persistent hypotonia, respiratory, and feeding difficulties. Imaging revealed bilateral vestibulocochlear nerve atresia and a hypoplastic facial nerve. Genetic evaluation for Pai syndrome is ongoing. This case highlights the diagnostic and management challenges of nasal gliomas, particularly when associated with syndromic features. MRI is critical for preoperative assessment to exclude intracranial communication. Early surgical excision and multidisciplinary follow-up are essential for optimal outcomes in affected neonates.