title: Follow-up and surgical management of Peutz-Jeghers syndrome in children.
abstract: BACKGROUND
  Peutz-Jeghers syndrome (PJS) is an autosomal dominant syndrome with an increased risk of polyposis complications and intestinal and extraintestinal tumours.
  
  
  METHODS
  During the last 15 years, we reviewed a series of 11 children with PJS, with special attention to evolution and follow-up. Diagnosis was based on at least 1 hamartomatous polyp associated with 2 of the 3 following criteria: family record of PJS, polyposis localised on small bowel, and mucocutaneous pigmentation. Diagnosis of PJS also could be raised by a single genetic analysis of STK11 gene.
  
  
  RESULTS
  Median age at beginning of symptoms was 6 years old. Seven of the 11 children had genetic tests, which were positive for STK11 gene mutation. Among the 10 children presenting with gastrointestinal complications, 8 were operated on, 6 had at least 1 small bowel resection, and 4 had repeat surgery for recurrent intussusceptions. In case of complications leading to a surgical procedure, we performed intraoperative enteroscopy to remove all large polyps. To prevent any polyposis complications, we suggest a complete check-up of polyposis topography with some of the new endoscopic tools, either double-balloon endoscopy or videocapsule endoscopy.
  
  
  CONCLUSIONS
  Children with PJS have a high risk of numerous laparotomies due to polyps' complications. Therefore, a screening of intestinal polyposis by videocapsule endoscopy is recommended, as well as a screening of the most frequent sites of cancers for the patient's whole life. During any abdominal procedure, they should have an intraoperative endoscopy, this management allowing an increased time interval between 2 laparotomies.
authors.affiliation: Services de Chirurgie Pédiatrique, Institut des Maladies de L'appareil Digestif, Centre Hospitalier Universitaire, Nantes, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Vidal
authors.forename: Guillaume
authors.initials: G
authors.lastname: Podevin
authors.forename: Hugues
authors.initials: H
authors.lastname: Piloquet
authors.forename: Marc
authors.initials: M
authors.lastname: Le Rhun
authors.forename: Benjamin
authors.initials: B
authors.lastname: Frémond
authors.forename: Didier
authors.initials: D
authors.lastname: Aubert
authors.forename: Marc-David
authors.initials: MD
authors.lastname: Leclair
authors.forename: Yves
authors.initials: Y
authors.lastname: Héloury