Peutz-Jeghers syndrome in children: Report of two cases and review of the literature*

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In this article, we report two new cases ofPeutz-Jeghers syndrome in children and review the literature over the past twenty years. This series of 70 cases demonstrates that the clinical pictures observed in children are similar to those of adults. Rectal prolapse or extrusion of polyps can be the clue to the diagnosis at an early age, even in the absence of pigmentation, which can appear later. Gastroduodenal polyps were strikingly frequent in the ≤16-yr-old group (62%) a circumstance that can create operative difficulties. Five out of the 70 patients (7.14%) had tumors during childhood (two gastrointestinal adenocarcinomas, two ovarian and one testicular neoplasms). A higher risk of tumor development in these patients does exist either as a result of degeneration of the polyps or of a genetic predisposition. Whenever operation becomes necessary, a very cautious approach must be advised in order to preserve as much intestinal length as possible in these patients, who have a lifelong disease which may require repeated operations.

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    *

    Presented before the XXIX Annual Congress of the British Association of Paediatric Surgeons, Madrid, Spain, July 21–23, 1982.

    1

    From the Department of Pediatrics, Pediatric SurgicalUnit, Universidad del Pais Vasco, Residencia Na So de Aránzazu, San Sebastián, Spain.

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