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 22-year-old male patient was referred to us for evaluation of multiple pigmented lesions over his mouth, present since childhood. Since the age of 9 year, there was a history of occasional hematochezia. However, there was no history of melena. The parents had taken him to a local physician, and the condition was managed with antihelminthic drugs, but the problem used to recur. There was no history of weight loss or any other systemic complaints. Five days back, he had presented to the general emergency with complaints of nonpassage of stool and flatus, pain, and distension of abdomen and vomiting. He had tachycardia, tachypnea, dehydration, hypotension, and distended rigid abdomen. Per rectal examination revealed empty rectum with blood mixed mucus. Straight X-ray abdomen showed dilated bowel loops, multiple air-fluid levels, and the absence of gas in colon and rectum []. Diagnosis of small bowel obstruction was made, and the patient was resuscitated. Hematological profiles and serum electrolytes were within the normal limits. Exploratory laparotomy was done. On exploration, ileocecal intussusception was found with a short segment of gangrenous ileum which was managed with limited ileocecal resection []. Numerous polyps were found along the whole length of the small and large gut. Proximal ileostomy with distal mucous fistula was made. A polyp was found to be the apex of the intussusception, which was histologically found to be a hamartomatous polyp [].\nDuring follow-up, upper gastrointestinal endoscopy and colonoscopy were done which revealed the presence of polyps from the gastric fundus to the rectum. Biopsy from those polyps confirmed a hamartomatous pathology []. Besides, detailed cutaneous examination revealed melanotic mucocutaneous macules in the lips and buccal mucosa [Figures and ]. As per the patient, one of his two younger brothers had similar lesions over the lips, but he was not available for examination. There was no history of hematochezia, melena, or weight loss among the siblings or any other family member.\nAfter 5 months of the first surgery, the patient was reoperated for taking down of ileostoma. During operation, colocolic intussusception was found in the transverse colon with a large mass palpable inside. This colocolic intussusception was resected with ileotransverse anastomosis. Postoperative period was uneventful, and the patient was discharged after 6 days. Histopathology of the mass showed expansion of the marginal zone of the lymphoid tissue along with sheets of heterogeneous population of small lymphocytes, suggestive of MALToma []. Immunohistochemistry showed CD20 positivity. Both proximal and distal margins were negative for malignant cells.\nAfter 1-year follow-up, the patient was asymptomatic. Colonoscopy revealed few pedunculated polyps in the rectum and sigmoid colon, but the surface was smooth. Yearly colonoscopy follow-up was planned to detect early malignant changes.