Revista chilena de cirugía
versión On-line ISSN 0718-4026
GREZ I, MANUEL; PRADO A, RODRIGO; LAHSEN H, JUAN y HERNANDEZ M, JORGE. Intestinal obstruction in a patient with Peutz-Jeghers syndrome: Report of one case. Rev Chil Cir [online]. 2008, vol.60, n.3, pp. 249-254. ISSN 0718-4026. http://dx.doi.org/10.4067/S0718-40262008000300015.
The Peutz-Jeghers syndrome (PJS) is an uncommon hereditary autosomal dominant disease, characterized by pigmentation of oral mucosa, plantar and palmar skin and gastrointestinal hamartomatous polyposis. When these polyps grow they can cause intussusceptions, intestinal obstruction and hemorrhages. We report a 38 years oíd male admitted for an intestinal obstruction. He had pigmentations of lips and palms of the hands. He was operated, finding a dilatation and thickening of thefirst 50 cm of jejunum. In the zone of obstruction, three intraluminal tumors of approximately 3.5 cm diameter were palpated. Similar tumors were palpated in transverse and descending colon. Approximately 20 cm of dilated proximal jejunum were excised. The pathology report informed the presence of hamartomatous polyps, confirming the diagnosis of Peutz-Jeghers syndrome
Palabras clave : Peutz-Jeghers syndrome; hamartomatous polyp; intestinal obstruction.