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A Rare Case of Multiple Intussusception In An Adult-Puetz-Jeghers Syndrome

Sarath R S and Balasubramanian S .

Abstract


Intussusception in adult is rare with anincidence       of  2-3 in 1000000 population. We present a rare case of multiple intussusceptions in a patient with Peutz-Jeghers syndrome. 20 year old female presented with features of abdominal pain and vomiting. She was pale, dehydrated with tachycardia. The abdomen was minimally distended. A firm mass of size 5×4 cm over left iliac fossa with empty RIF.  Investigations showed multiple air fluid levels with features of intestinal obstruction with the possibility of intussusception. Exploratory laparotomy done revealed multiple segments   of intussusceptions for which multiple resection and   anastomosis was done. There were multiple polyps in the resected specimen and HPE revealed hamartomatous polyps of PeutzJeghers type. Peutz–Jeghers syndrome is a rare autosomal dominant genetic disorder characterized by the development of benign hamartomatous polyps in the  gastrointestinal tract and hyperpig-mented macules on   the lips and oral mucosa.


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