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.
A 19-year old male Caucasian patient visited the emergency department of our hospital because of melena. He was previously healthy and used no medication but had noticed black stools one week before his visit. There had been no abdominal discomfort, nausea or vomiting. None of his family members had gastrointestinal diseases, and he used no alcohol, did not smoke or use elicit drugs. His occupation is unknown. On physical examination the abdomen was nontender and without palpable masses. Rectal examination revealed black stools. His weight was 62 kg. and his length 1,87 m. Laboratory tests showed an iron deficiency anemia with a hemoglobin concentration of 4.7 mmol/L (normal > 8,5 mmol/L) and a serum ferritin concentration of 4 μg/L (normal > 30 μg/L). Liver function tests, serum creatinine, albumin, CRP and glucose were all normal. Duodenoscopy showed a normal esophageal, gastric and duodenal mucosa without evidence of recent bleeding. A Meckel scan showed no evidence for a Meckel's diverticulum. A small bowel series however, demonstrated a large polyp in the proximal jejunum and possibly a smaller polyp in the distal jejunum (Figure ).\nAs this polyp was the probable cause of the patient's bleeding, a laparotomy with peroperative small bowel endoscopy was performed. Approximately 50 cm beyond Treitz' ligament, a multilobular, 40 mm large polyp was seen, which was subsequently surgically resected (Figure ). There were no other small bowel polyps; the suspected smaller polyp seen on the small bowel series was therefore most likely an artefact.\nPathologic examination of this polyp demonstrated a hamartomatous polyp with arborization of smooth muscle compatible with a PJP (Figure ). No signs of dysplasia were seen and the surrounding tissue had a normal histological appearance. Immunohistochemistry was negative for p53, focally positive for cyclin D1 and the basal parts of the cryps were positive for Ki67/MIB1.\nThe postoperative course has been unremarkable and no further gastrointestinal bleeding has occurred. Total colonoscopy did not show any additional polyps. A repeated, extensive family history, performed by a clinical genetics consultant, did not reveal clues (including pigmentation, nasal or gastrointestinal polyps or typical secondary tumors) to the occurrence of PJS in family members.