Peutz Jeghers Syndrome Presented as intermittent gastric outlet obstruction

Abstract

Peutz Jeghers Syndrome (PJS), which was first described in 1921 by Peutz, followed by Jeghers etal in 1949, is an uncommon but not a rare disorder characterized by mucocutaneous melanin pigmentation, gastrointestinal hamartomatous polyps and increased risk of gastrointestinal and other
organs cancer. The polyps vary in size from few millimeters to several centimeters, with lobulated surface, and could be pedunculated as in large polyps, or sessile as in the small ones. The clinical symptoms of the disease are recurrent abdominal pain, intestinal intussusception and obstruction, gastrointestinal bleeding with symptoms of iron deficiency anaemia. The symptoms usually take place in the second and third decade of life. The complications of PJS which brings the patient to the doctor is severe anemia due to blood loss from GIT, acute abdomen, intestinal obstruction due to tumor-mass obstruction or intussusception, or rarely due to gastric outlet obstruction as in this presented case. The treatment of polyposis is by endoscopic polypectomy, laprotomy and resection which may lead to complications as short bowel syndrome. The other difficulty which the doctor comes across while managing these cases is to follow-up the occurrence of malignant disease in these patients, malignant changes of hamartomatus polyps and other organs cancers have been reported in patients with PJS.

Keywords: hamartomatus polyps, polypectomy, mucocutaneous.