An 8-year-old boy was referred with four episodes of melena over the past 10 months, fatigability and refractory anemia. There was no history of abdominal pain, loss of weight/appetite, or drug intake. There was no history suggestive of bleeding dyscrasia or family history suggestive of PJ syndrome. On clinical examination, pallor was noted without perioral mucocutaneous pigmentation. Abdominal examination was normal. All hematological and biochemical investigations were within normal limits, except for hemoglobin of 6.5 g/dL, and stool examination positive for occult blood. Ultrasonography of the abdomen, upper and lower gastrointestinal (GI) endoscopy, and Tc99m Meckel's isotope scan were normal. Capsule endoscopy visualized an approximately 2 cm × 2 cm, single, sessile, submucosal polypoidal lesion in the small bowel. Contrast-enhanced computerized tomography (CECT) enteroclysis done a few days later showed small bowel intussusceptions. On laparoscopy, a 20 cm long jejunojejunal intussusception starting 10–15 cm from the duodenojejunal flexure was seen. This was reduced laparoscopically. An intraluminal mass could be appreciated in this segment. No other abnormality was detected. The involved bowel was delivered through the umbilical camera port incision [] and resection anastomosis performed. The histopathological examination of the polyp was suggestive of a PJ polyp []. There were areas of pseudoinvasion in the lamina, submucosa, and muscularis propria.\nHe had an uneventful postoperative period. Although we could not do a specific chromosomal analysis on the patient for PJ syndrome, a family screening was negative. There was no family history of cancers in the GI tract or elsewhere. At 3-year follow-up, he remains asymptomatic with no manifestations of PJ syndrome.
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.