title: Peutz-Jeghers syndrome: data from the Singapore Polyposis Registry and a shifting paradigm in management.
abstract: BACKGROUND
  Peutz-Jeghers Syndrome (PJS) is an uncommon autosomal dominant hamartomatous polyposis syndrome. Morbidity arises from polyp-related complications and increased risks of malignancy. We report on PJS patients registered in the Singapore Polyposis Registry, identified principal causes of morbidity and appraised current management strategies. A followup protocol based on recent literature has been proposed.
  
  
  METHODS
  A search of a prospectively collected database in the Singapore Polyposis Registry was made. Only patients who fulfilled the diagnostic criteria of PJS were included. The clinical records were retrieved for review. Information on affected family members was obtained from the Registry's pedigree records.
  
  
  RESULTS
  Seven unrelated patients fulfilled the criteria of having PJS. Principal causes of morbidity include recurrent bouts of abdominal colic, episodes of intestinal obstruction, gastrointestinal bleeding and the need for repeated laparotomies. Six out of 7 patients had initial presentation with acute intestinal obstruction requiring emergency laparotomy. Management was mostly problem-oriented and marked inter-surgeon variation with regard to cancer screening and genetic counselling was observed.
  
  
  CONCLUSIONS
  Patients with PJS suffer gastrointestinal complications from polyposis and are at increased risks for developing cancers. A move towards surveillance and planned comprehensive care may reduce the morbidity of the condition. A protocol driven approach conducted in the setting of a Polyposis Registry is ideally suited to facilitate such care.
authors.affiliation: Department of General Surgery, Singapore General Hospital, Singapore.
authors.forename: Veronique Km
authors.initials: VK
authors.lastname: Tan
authors.forename: Poh Koon
authors.initials: PK
authors.lastname: Koh
authors.forename: Carol Tt
authors.initials: CT
authors.lastname: Loi
authors.forename: Kong Weng
authors.initials: KW
authors.lastname: Eu
authors.forename: Choong Leong
authors.initials: CL
authors.lastname: Tang
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D015331:Cohort Studies; D019091:Critical Pathways; D019468:Disease Management; D005260:Female; D005767:Gastrointestinal Diseases / Q000150:complications* / Q000453:epidemiology; D006801:Humans; D008297:Male; D010580:Peutz-Jeghers Syndrome / Q000150:complications* / Q000628:therapy; D011159:Population Surveillance; D015995:Prevalence; D012042:Registries; D012846:Singapore / Q000453:epidemiology; D055815:Young Adult