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.
Our case was a 2-year-old boy who was incidentally diagnosed with anemia during the evaluation of an upper respiratory tract infection. There was no history of hematemesis, melena, or abdominal pain. The child was evaluated by the pediatric gastroenterologist with an upper gastrointestinal endoscopy and was found to have a large sessile gastric polyp that was not amenable to endoscopic resection. Physical examination was insignificant except for a few melanotic spots over the lower lip []. Radiological imaging revealed a large hyper-enhancing mass lesion in the lumen of the stomach, measuring about 8 cm in greatest dimension along with multiple small intestinal polyps. Laparotomy revealed a large sessile polyp involving the greater curvature and posterior wall of the stomach measuring about 10 cm × 8 cm along with multiple small sessile polyps involving the corpus of the stomach sparing the antrum and pylorus, requiring partial gastrectomy []. There were two more polyps, one each in the duodenum and distal jejunum in their antimesenteric border and they could be excised by enterotomy. The child had an uneventful postoperative course. The histopathology revealed the features of tubulovillous adenoma in all the resected polyps. At 36 months of follow-up, the child is asymptomatic and disease-free.