Peutz-Jeghers syndrome complicated with intussusception: enteroscopic polyps resections through laparotomy.

ABSTRACT

Peutz-Jeghers syndrome is an autosomal dominant inherited disorder characterized by intestinal hamartomatous polyps in association with mucocutaneous pigmentations. Here we present a case of a 30-year-old woman who was hospitalized and underwent diagnostic procedures because of crampy abdominal pain. Physical examination on admission revealed pigmented spots around lips and on the oral mucosa. Multiple polyps were found in stomach, small and large intestine, with signs of initial ileo-ileal intussusception. After endoscopic removal of achievable polyps, we applied gastroscope through laparotomy and enterotomy and removed total number of 34 polyps from small bowel. The polyps were found to be mostly hamartomatous at histological examination. This procedure can provide removal of the most polyps, which are potentially premalignant, also with less complicationes than after multiple intestinal resectiones.