Snapshot A 14-year-old girl presents to the emergency room with acute abdominal pain and constipation. An ultrasound reveals a “target sign”, suggesting intussusception. A CT scan confirms this diagnosis. On closer examination, her palms and oral mucosa are spotted with multiple 1-2 mm blue-gray macules. She is scheduled for emergency surgery to decompress the intussusception with follow-up endoscopy as an outpatient. Introduction Inherited syndrome with login to view 2 more bullets Genetics login to view 4 more bullets Associated conditions login to view 1 more bullet Presentation Symptoms/physical exam login to view 13 more bullets Evaluation Diagnosis with endoscopy login to view 3 more bullets Complete blood count login to view 1 more bullet Differential Diagnosis Familial adenomatous polyposis Juvenile polyposis Cowden syndrome Treatment Routine evaluation with cancer screening every 1-2 years Endoscopic resection of polyps login to view 1 more bullet Prognosis, Prevention, and Complications Prognosis login to view 2 more bullets Complications login to view 7 more bullets