27. Peutz Jeghers syndrome - A rare cause of recurrent intussusception in older children: A case report
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Abstract
Peutz Jeghers syndrome is a rare genetic syndrome, characterized by mucocutaneous pigmentation and hamartomatous polyposis of the gastro-intestinal (GI) tract. This is one of many causes of recurrent intussusception in older children. The incidence of this condition is estimated to be between 1 in 50,000 to 1 in 200,000 live births. Up to now, there is no specific treatment method, other than regular re-examination, removal of large polyps and screening for cancer risk. We report a case of a 13-years-old female hospitalized due to recurrent intussusception with flat brown-black pigmented macules concentrated on the face and scattered on the fingers and toes, with a healthy family history. Diagnostic imaging examination discovered many polyps stretching from the stomach - small intestine - colon, especially large polyps in the gastric antrum that had penetrated through the pyloric, causing pyloric stenosis, large polyps with sizes > 5cm in the small intestine causing intussusception of the small intestine over a long segment that does not spontaneously detach. Pathological results of all polyps removed were hamartoma polyps. Polyps > 1cm in size were removed through standard gastroesophageal endoscopy and colorectal endoscopy, and surgery intervention for small intestinal polyps unmanageable through standard endoscopy.
Article Details
Keywords
Peutz Jeghers syndrome, pigmentation, intussusception, family history
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