Peutz-Jeghers syndrome as a cause of intestinal intussusception in children

Abstract

Intestinal intussusception occurs more frequently in the infant younger than 2 years, with a peak between 5 and 9 months of age. In the vast majority of cases it is idiopathic or of unknown cause, probably secondary to a viral infection that causes a growth of the intestinal lymphatic tissue. Only in 2-8% of the patients an anatomical alteration as cause of the intussusception is demonstrated (Meckels diverticulum, polyp). It is called secondary intestinal invagination, which usually occurs in children under 3 months or older than 3 years.
Peutz-Jeguers syndrome (SPJ) is a rare condition and its frequency is probably less than 1 / 50,000. It is characterized by the presence of potentially malignant hamartomatous polyps throughout the digestive tract associated with characteristic mucocutaneous pigmentations. Current data suggest the existence of at least 2 genes involved: STK11 (19p13.3), recently identi ed, is responsible for the disease in 70% of families. We describe the case of two adolescents with intestinal intussusception with a diagnosis of familial polyposis of Peutz Jeghers, requiring both surgical procedures