Peutz-Jeghers Syndrome


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Abstract: Peutz--Jeghers syndrome is an autosomal dominant inherited polyposis disorder where diffuse characteristic gastrointestinal polyps, mainly involving the small bowel, coexist with mucocutaneous pigmentation. Until the advent of balloon-assisted enteroscopy, intraoperative enteroscopy and surgical treatment were the sole treatment options for patients with Peutz--Jeghers syndrome. Balloon-assisted enteroscopy, allowing direct visualization of the small bowel and therapeutic maneuvres, paved the way for nonsurgical small bowel therapy. In the context of patients diagnosed with Peutz--Jeghers syndrome, balloon-assisted enteroscopy with polypectomy allows prevention of polyp-related complications such as intussusception/obstruction or bleeding and reduces the need for future operative polypectomies and either elective or emergent laparotomies, thus avoiding the risk of multiple enterotomies or small bowel resections leading to short bowel syndrome. Furthermore, clearance of small bowel polyps could potentially decrease malignancy risk, although low, by removal of precursor lesions.
Here different small bowel polypectomy techniques are described in a patient affected by Peutz--Jeghers syndrome presenting with multiple jejunal polyps detected at prior videocapsule endoscopy surveillance. This article is part of an expert video encyclopedia.
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Entendendo a Polipose adenomatosa familiar e Peutz Jegher!!
Aqui no HARDWORK MEDICINA descomplicamos a Medicina
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