An unusual hamartomatous malformation of the rectosigmoid presenting as an irreducible rectal prolapse and necessitating rectosigmoid resection in a 14-week-old infant.
A 14-week-old female infant presented with an irreducible rectal prolapse and a large polypoid tumor at the tip of the prolapsed mucosa. The tumor and prolapsed rectum were resected. Four weeks after the operation, profuse rectal bleeding occurred and a second similar tumor was diagnosed by endoscopy in the sigmoid colon. Laparotomy, rectosigmoid resection, and endorectal pull-through were performed. At operation, the serosal surface showed ragged polypoid lesions and an abnormal angiomatous vascularization. The postoperative course was uneventful. The histology suggested a congenital mucosal malformation. This pathology is unique in our experience and we have been unable to find anything resembling it in the literature. At age two years a Sertoli cell tumor developed in the girl with pubertas precox and a recurrent colonic polyp of the Peutz-Jeghers type.
D012809:Colon, Sigmoid / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D006222:Hamartoma / Q000151:congenital* / Q000473:pathology / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D010580:Peutz-Jeghers Syndrome / Q000175:diagnosis; D012004:Rectal Neoplasms / Q000151:congenital* / Q000473:pathology / Q000601:surgery; D012005:Rectal Prolapse / Q000209:etiology*; D012007:Rectum / Q000601:surgery; D012811:Sigmoid Neoplasms / Q000151:congenital* / Q000473:pathology / Q000601:surgery