January 2010, Vol. 39 No. 1 17 Peutz-Jeghers Syndrome in Singapore—Veronique KM Tan et al Original Article Abstract Introduction: Peutz-Jeghers Syndrome (PJS) is an uncommon autosomal dominant hamarto- matous 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 follow- up protocol based on recent literature has been proposed. Materials and 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 pedi- gree records. Results: Seven unrelated patients fulfilled the criteria of having PJS. Principal causes of morbidity include recurrent bouts of abdominal colic, episodes of intestinal obstruc- tion, 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. Conclusion: 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. Ann Acad Med Singapore 2010;39:17-21 Key words: Harmatoma, Intussusception, Polyps, Small bowel Peutz-Jeghers Syndrome: Data from the Singapore Polyposis Registry and a Shifting Paradigm in Management Veronique KM Tan, 1 MBBS, MMed (Surg), MRCS (Edin), Poh Koon Koh, 2 MBBS, FRCSE (Gen), FAMS, Carol TT Loi, 3 RN, MSc (Healthcare), Grad Dip Genetic Counselling, Kong Weng Eu, 1 MBBS, FRCS (Edin), FAMS, Choong Leong Tang, 2 1 Department of General Surgery 2 Department of Colorectal Surgery 3 Singapore Polyposis Registry Singapore General Hospital, Singapore Address for Correspondence: Dr Tang Choong-Leong, Senior Consultant, Department of Colorectal Surgery, Director, Singapore Polyposis Registry, Singapore General Hospital, Outram Road, Singapore 169608. Email: tang.choong.leong@sgh.com.sg Introduction Peutz-Jeghers Syndrome (PJS) is an uncommon autosomal dominant hamartomatous polyposis syndrome associated with mucocutaneous melanocytic macules. Melanin deposition occurs most commonly in the perioral region and buccal mucosa, but these maccules may also be found on the hands, feet and perianal regions. 1,2 Patients with PJS are also at increased risk for developing malignancies of the luminal gastrointestinal organs, pancreas, lungs, breasts and reproductive organs. 3-6 We report on the PJS patients registered in the Singapore Polyposis Registry, highlighting patterns of presentation and the principal causes of morbidity and management to date in our local population. We reviewed the current literature and proposed a shift from the traditional reactive problem-oriented surgery to employing a preventive combined surgical-endoscopic approach in the management of diffuse small bowel polyposis. Materials and Methods A search of a prospectively collected database in the Singapore Polyposis Registry (started in 1989) containing 610 patients with intestinal polyposis was made. Data collection was based on voluntary reporting by participating clinicians islandwide. Only patients who fulfilled the diagnostic criteria of PJS were included in this study, that is, having histopathologically confirmed hamartomatous polyps and at least 2 of the following clinical criteria: i) family history, ii) mucocutaneous hyperpigmentation, and iii) small bowel polyposis. 5 Seven patients with PJS were identified. Their clinical records were retrieved for review. Information on affected family members was obtained from the Registry’s pedigree records. Results All 7 index patients had small bowel hamartomatous polyps confirmed on histology and peri-oral pigmented