A 21-year-old Turkish woman was admitted to our emergency service with complaints of abdominal pain and vomiting that had intensified over three days. This colicky abdominal pain was located in her right upper abdomen and was found to be non-radiating in nature. The pain became stronger after eating or drinking, and vomiting was found to relieve the pain for a while. Her upper abdomen was tender upon palpation, and guarding was significant in her right upper abdomen. Rebound tenderness or rigidity were not detected in our patient. In her right upper abdomen, an approximately 5cm-diameter mass was palpable and bowel sounds were significantly increased at this location. Mucocutaneous pigmentation was noted on her lips and oral mucosa (Figure ).\nHer vital signs were normal except for mild tachycardia (110 beats/min). Her leukocyte count was 13.1×109 cells/L, and her total and direct bilirubin counts were 4.0mg/dL and 2.9mg/dL, respectively. Her blood amylase level was slightly increased (230U/L). Except for the laboratory values noted, all other laboratory tests were deemed normal.\nOur patient had been previously diagnosed with PJS 5 years before; however, she had no history of prior hospitalizations or surgery. Since the age of sixteen, our patient had experienced chronic abdominal pain and perioral hyperpigmentation. Because of her current complaints, further investigation had been performed at another hospital. Endoscopic and computed tomography (CT) enteroclysis findings had only shown a small solitary polyp in her proximal jejunum.\nAbdominal plain radiographs revealed distinct air-fluid levels at the level of her duodenum. An abdominal ultrasound revealed a target sign of invagination at the level of the right upper abdomen. Her stomach and the proximal part of her duodenum were dilated on contrast-enhanced CT, indicating the presence of an obstruction. Her intra- and extra-hepatic biliary tracts were found to be dilated, which was a result of the compression of invaginated jejunal segments on the papilla (Figure ). The CT did not reveal any other polyps in her gastrointestinal tract. A colonoscopy had previously been performed in another clinic two months prior to the presenting symptoms, and the findings of that examination were normal.\nWe performed an esophagogastroduodenoscopy and found polyps in the fourth part of her duodenum. The invaginated jejunal fragments were also visualized. Reduction of the jejunal segments was attempted through gastroscopy, however this attempt was unsuccessful.\nTechnical difficulties with respect to the retroperitoneal position of her duodenum led us to carry out a laparotomy instead of a laparoscopy. Perioperative exposure revealed that the third and fourth parts of her duodenum were dilated. The Treitz ligament was loosened, and the invagination of the jejunal segments was gently reduced. We performed total excision of the polyps through an enterotomy at the last part of her duodenum (Figure ). We subsequently made a thorough examination of her gastrointestinal tract; no other masses were palpated in the remaining parts of visualized gastrointestinal tract.\nGross pathologic findings revealed an 8cm × 4cm × 3cm polypoid tissue mass. Histopathological examination revealed a hamartomatous polyp with adenomatous changes, without evidence of any malignancy. Immunohistochemical examination showed branching smooth muscle bands in the stroma of the polyp as typically seen in hamartomatous polyps of PJS (Figure ).\nOur patient had an uncomplicated postoperative course and was discharged home on postoperative day six. During the two-year follow-up period, our patient did not have any further episodes of intussusception.
We report a case of a 37 year old man who admitted to our hospital with complaint of occasionally painless rectal bleeding since 8 years ago which had exacerbated with no history of weight loss, abdominal pain, loss of appetite or anal pain. He had no significant past medical history or significant family history of GI malignancy. In physical examination he did not had any pigmentation in mucocutaneous and any abnormal finding in abdomen and rectal examination.\nGastrointestinal endoscopy revealed multiple polyps located in the deuodenum and less than 100 polyps in colon with aggregation in left colon and sigmoid with pathology of juvenile polyposis.\nA barium enema he had mucosal lesions in keeping with polyps in sigmoid (). A laparotomy was performed and multiple giant polyps entire colon was found. Total abdominal proctocolectomy and J pouch ileoanal anastomosis and omentectomy was performed and a diverting ileostomy was inserted. Histopathological evaluations of the polyps revealed hamartomatous polyps in keeping with a diagnosis of Peutz Jeghers syndrome ().