Pai syndrome: a case report and review of the literature

What is it about?

Pai syndrome is a rare congenital disorder. There have been few reports on Pai syndrome with a long-term follow-up. Here, we report a female patient with Pai syndrome, who was followed up for 8 years. Additionally, we review 32 articles and discuss the long-term prognosis of Pai syndrome. Our case showed normal mental development at 8 years of age, although an intracranial lipoma grew a little. Her nasal airway was gradually obstructed by the residual intra-nasal polyp. Further information about Pai syndrome is needed to clarify its prognosis

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Why is it important?

There have been few reports on Pai syndrome with a long-term follow-up. Here, we present the treatment approach used in our patient and discussed the clinical course of this patient until childhood at 8 years of age. Additionally, we discuss the etiology and prognosis of Pai syndrome with a literature review.

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