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<p>Pai syndrome, first described in 1987, is characterized by the presence of a congenital nasal lipoma, a median cleft of the upper lip, nasal polyps, and an intracranial lipoma. A 7-year-old male patient presented to our clinic with a chief complaint of nasal obstruction. Imaging studies revealed an intracranial lipoma. Total excision of the nasal mass was performed, and histopathological examination confirmed it as a nasal lipoma. Given that the intracranial lipoma was symptomatic, follow-up was planned. Investigations for a preliminary diagnosis of Pai syndrome did not reveal other findings consistent with this syndrome. As the patient’s relatives declined genetic testing, the patient was closely monitored. In pediatric patients presenting with nasal masses, the presence of an intracranial mass must be ruled out with cranial imaging prior to surgical intervention. When Pai syndrome is suspected, the patient should undergo a multidisciplinary evaluation.</p>

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Pediatric Nasal Lipoma with İntracranial Lipoma-Rare Case of Pai Syndrome

  • 期刊论文

  • 发表日期：

    2025年12月17日

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  • Esra Yılmaz,
  • Merve Mutlu Çekim,
  • Furkan Uyar,
  • Dursun Mehmet Mehel,
  • Doğukan Özdemir

摘要

Pai syndrome, first described in 1987, is characterized by the presence of a congenital nasal lipoma, a median cleft of the upper lip, nasal polyps, and an intracranial lipoma. A 7-year-old male patient presented to our clinic with a chief complaint of nasal obstruction. Imaging studies revealed an intracranial lipoma. Total excision of the nasal mass was performed, and histopathological examination confirmed it as a nasal lipoma. Given that the intracranial lipoma was symptomatic, follow-up was planned. Investigations for a preliminary diagnosis of Pai syndrome did not reveal other findings consistent with this syndrome. As the patient’s relatives declined genetic testing, the patient was closely monitored. In pediatric patients presenting with nasal masses, the presence of an intracranial mass must be ruled out with cranial imaging prior to surgical intervention. When Pai syndrome is suspected, the patient should undergo a multidisciplinary evaluation.