A 24-year-old Caucasian man presented with an acute onset of melaena to the Emergency Department of our hospital. He had no abdominal pain, or history of change in bowel habit or any significant loss of weight or appetite. He was previously healthy and took no regular medications. He had no family history of gastrointestinal disorders. Our patient was also a non-smoker and had no history of illicit drug use.\nUpon admission, he was hypotensive and tachycardic. However, abdominal examination was completely unremarkable. Rectal examination revealed black tarry stool. Multiple dark pigmented patches were noted on the buccal mucosa of our patient. Laboratory tests revealed microcytic, hypochromic anaemia with a haemoglobin of 7.6 gm%. Blood urea was elevated at 12 mmol/litre. Initial assessment showed no other abnormalities.\nHe was resuscitated and required transfusion of six units of blood. Urgent upper gastrointestinal endoscopy revealed polyps in the stomach and duodenum, but no active bleeding sites were found. The presence of buccal pigmentation and multiple polyps on endoscopy suggested a diagnosis of PJS. A computed tomography (CT) scan of the abdomen of our patient was therefore performed. This confirmed multiple small bowel intussusceptions. (Figure )\nUrgent laparotomy was undertaken on our patient and the intussusceptions were reduced. Through enterotomies, eight polyps were removed from the affected segment of the gut. The affected segment of gut was found to be viable. He had a fairly uneventful recovery with no further bleeding. Pathological examination of the polyps confirmed hamartomas with smooth muscle arborisation, compatible with Peutz Jeghers polyps. No features of pseudo-invasion were noticed in any of the polyps. (Figure )
Case 1 is an 84-year-old Japanese man with previous medical history of hypertension, chronic hepatitis C infection, idiopathic thrombocytopenic purpura and colon polyps (tubular adenoma and tubulovillous adenoma). He had no mucocutaneous pigmentation or family history of PJS. An upper gastrointestinal endoscopy revealed a lobular polyp measuring 14 mm in diameter, in the superior duodenal angle (Figure ). Endoscopic mucosal resection was performed without complication and histological examination showed findings suggestive of a hamartomatous polyp--branching bundles of smooth muscle fibers covered by hyperplastic duodenal mucosa--with a focus of well-differentiated adenocarcinoma (Figure ). A colonoscopy and small-intestinal follow-through showed no other polyps.