Location of Repository

Melaena with Peutz-Jeghers syndrome: a case report

By Sayantan Bhattacharya, Sunondo R. Mahapatra, Ramlal Nangalia, Amitabh Palit, John R. Morrissey, Ernie Ruban, Vijay Jadhav and G. (George) Mathew

Abstract

Introduction: Peutz-Jeghers syndrome (PJS) is a rare familial disorder characterised by mucocutaneous pigmentation, gastrointestinal and extragastrointestinal hamartomatous polyps and an increased risk of malignancy. Peutz-Jeghers polyps in the bowel may result in intussusception. This complication usually manifests with abdominal pain and signs of intestinal obstruction. \ud Case Presentation: We report the case of a 24-year-old Caucasian male who presented with melaena. Pigmentation of the buccal mucosa was noted but he was pain-free and examination of the abdomen was unremarkable. Upper gastrointestinal endoscopy revealed multiple polyps. An urgent abdominal computed tomography (CT) scan revealed multiple small bowel intussusceptions. Laparotomy was undertaken on our patient, reducing the intussusceptions and removing the polyps by enterotomies. Bowel resection was not needed. \ud Conclusion: Melaena in PJS needs to be urgently investigated through a CT scan even in the absence of abdominal pain and when clinical examination of the abdomen shows normal findings. Although rare, the underlying cause could be intussusception, which if missed could result in grave consequences

Topics: RC
Publisher: BioMed Central Ltd.
Year: 2010
OAI identifier: oai:wrap.warwick.ac.uk:3017

Suggested articles

Preview

Citations

  1. (1981). DC: Surgical management of intussusception in the adult. Ann Surg doi
  2. (2008). Dworzanski T: Non-variceal upper gastrointestinal bleeding–guidelines on management. doi
  3. (2007). Eng C: Hamartomatous polyposis syndromes. Nat Clin Pract Gastroenterol Hepatol doi
  4. (1998). Greene MH: The concise handbook of family cancer syndromes. Mayo Familial Cancer Program. doi
  5. (2008). Honkoop PP: A solitary Peutz-Jeghers type polyp in the jejunum of a 19 year-old male. Cases J doi
  6. (2008). Hurlstone DP: Usefulness of prognostic indices in upper gastrointestinal bleeding. Best Pract Res Clin Gastroenterol doi
  7. (2003). Kurda OO: A PeutzJeghers syndrome case with iron deficiency anemia and jejuno-jejunal invagination.
  8. (2008). Lin YS: Tumor spectrum of adult intussusception. doi
  9. (1987). Luk GD: Increased risk of cancer in the Peutz-Jeghers syndrome. doi
  10. (2008). Management of upper gastrointestinal bleeding. Curr Gastroenterol Rep doi
  11. (2002). Minifee PK: Sonographic diagnosis of multiple small-bowel intussusceptions in Peutz-Jeghers syndrome: a case report. Pediatr Radiol doi
  12. (2006). Mirallie E: Clinical spectrum and surgical approach of adult intussusceptions: a multicentric study. doi
  13. (2000). Misplacement of dysplastic epithelium in Peutz-Jeghers Polyps: the ultimate diagnostic pitfall?. Am J Surg Pathol doi
  14. (2004). Simopoulos K: Intussusception of the small bowel due to Peutz-Jeghers syndrome: a case report. Ann Ital Chir
  15. (1992). Sobin LH: Peutz-Jeghers syndrome. Radiographics doi
  16. (2006). Tajiri T: Resection of over 290 polyps during emergency surgery for four intussusceptions with Peutz-Jeghers syndrome: Report of a case. Surg Today doi
  17. (1998). The role of laparoscopy in the management of intussusception in the Peutz-Jeghers syndrome: case report and review of the literature. Surg Laparosc Endosc
  18. (2005). TJ: The hamartomatous polyposis syndromes: a clinical and molecular review. doi

To submit an update or takedown request for this paper, please submit an Update/Correction/Removal Request.