Abstract

BackgroundPeutz-Jeghers Syndrome (PJS) is an uncommon intestinal polyposis disorder. Bowel obstructions are a recurring problem in PJS and as many as 50% of these patients require surgery. The current standard of care for these patients is to perform a flexible enteroscopic polypectomy. The traditional push-pull enteroscopy however, might be unavailable or unsuitable in an emergency setting. Alternatively, repeated laparotomies with multiple small bowel resections can lead to short bowel syndrome. MethodsIn our series, we describe an innovative technique where a short midline laparotomy permitted sufficient access to reduce the intussusception(s) and perform a bowel walk. Rigid laparoscopic instruments were introduced within the small bowel lumen via enterotomies, to perform polypectomies along the entire small bowel length. This precludes the need for small bowel resections which can thwart the development of short bowel syndrome. ResultsTwo patients with PJS presenting with acute small bowel obstruction underwent surgery using the described approach. Another two patients with PJS having multiple intussusceptions on CT underwent an elective prophylactic polypectomy using the same approach. We were able to run the bowel in its entirety and a maximum of 41 polyps were retrieved from the port site enterotomy. The operating times were modest and no unique complications pertaining to this technique were encountered. ConclusionSmall bowel polypectomy using rigid instrumentation employing a limited midline laparotomy is an attractive option for both emergency and elective settings in patients with PJS.

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