Peutz-Jeghers syndrome (PJS) is an autosomal dominant disease, characterized by in association with characteristic mucocutaneous pigmentation. Peutz (1921), in his first publication about the syndrome that has his name, presented two cases having polyps in the nasopharynx. Ever since, a few of those cases were published, as time goes, by association was forgotten. This report describes a rare variant of PJS in a fourteen years-old boy, identified by the presence of bilateral nasal polyposis, chronic sinusitis and hamartomatous intestinal polyposis, in an operated patient previously by intestinal oclusion. At macroscopy, multiple white formations were found, with soft consistency and cystic cavities. Microscopically, the polyps showed inflammatory characteristics associated with atypical escamous metaplasia. In pediatric population, nasal polyps present many matters of specific interest. It is an infrequent condition that requires careful diagnoses work up. The objective of this work is to show a rare disease (PJS) with association in Pediatric Surgery and ORL, calling attention of surgeons, pediatricians and specialists to the importance of investigating the etiology of nasal polyposis in the pediatric patients.
Peutz-Jeghers syndrome; nasal polyposis; chronic sinusitis; hamartomas; child
