Jejunojejunal intussusception secondary to a familial solitary Peutz–Jeghers hamartomatous polyp in an adolescent girl

摘要

Intussusception is defined as the telescoping of one segment of thegastrointestinal tract (intussusceptum) into an intestinal segment distalto it (intussuscipiens) and it is the leading cause of abdominal emergency and intestinal obstruction in childhood [1]. Approximately 90% of allpediatric intussusceptions are ileocolic, ileocecal, or ileoileocolic andjejunojejunal type is very uncommon. Pathologic lead points, such asMeckel's diverticulum, polyp or lymphoid hyperplasia are identified inless than 10% of cases [2,3]. Hamartomas are tumors representinganomalous development of tissues natural to an organ. Hamartomatouspolyps in the GI tract are a frequent cause of intussusception and oftenoccur in the setting of Peutz–Jeghers syndrome but these are usuallymultiple and a solitary Peutz–Jeghers polyp (PJP), that too in proximaljejunum, is an extremely rare entity, with an estimated incidence of1:120,000 [4]. We had a patient of PJP recently having a family historyof Peutz–Jeghers jejunal polyps but none of the family members including the patient had the characteristic mucocutaneous melaninpigmentation.