Revista chilena de pediatría
versión impresa ISSN 0370-4106
DONOSO C, CAROLINA y CARVAJAL F, OSCAR. Peutz Jeghers Syndrome and Bowel Obstruction in Children: A Clinical Case. Rev. chil. pediatr. [online]. 2010, vol.81, n.5, pp. 437-444. ISSN 0370-4106. http://dx.doi.org/10.4067/S0370-41062010000500008.
Peutz-Jeghers Syndrome (PJS) is an infrequent genetic disorder characterized by intestinal hamartomas and characteristic mucocutaneous pigmentation. It is frequently the cause of intususception, intentinal obstruction and/or intestinal bleeding. An 8 y.o. patient is described who presented with bowel obstruction and a history of familiar PJS polyposis. Exploratory laparotomy showed that the obstruction was due to a mass of sizable polyps which obstructed the intestinal lumen in the jejunum and colon. Surgical treatment consisted of double intestinal resection and termino-terminal anastomosis. This procedure resolved the acute intestinal obstruction and saved the patient's life.
Palabras llave : Peutz-Jeghers; polyposis; intestinal obstrucion.