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The usefulness of intra-operative endoscopy in Peutz-Jeghers syndrome: a case report
M C Yagmurdur1, C Daphan, A Ozdemir
1Department of General Surgery, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Insights
Peutz-Jeghers syndrome patients can benefit from combined surgery and perioperative endoscopy. This approach successfully removed 27 polyps in one session, offering a "clean small intestine" and reducing complications.
Area of Science:
- Gastroenterology
- Genetics
- Surgical Oncology
Background:
- Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by mucocutaneous pigmentation and gastrointestinal hamartomatous polyps.
- PJS polyps frequently lead to complications such as intussusception and intestinal hemorrhage, necessitating frequent interventions.
- Long-term management of PJS often involves repeated surgical and endoscopic polyp removal, increasing patient morbidity.
Observation:
- A case report details a PJS patient with a 14-year follow-up history, including multiple previous polyp removals and an intestinal resection.
- The patient underwent a procedure combining surgery and perioperative endoscopy for polyp removal.
- This session resulted in the removal of 27 polyps through only 3 enterotomies, a record number for a single session.
Findings:
- The combined surgical and perioperative endoscopic approach achieved a high polyp burden clearance in a single intervention.
- This technique successfully created a "clean small intestine," a crucial step in managing PJS.
- The procedure minimized the need for extensive resections and multiple laparotomies.
Implications:
- This combined approach offers a potentially superior strategy for managing Peutz-Jeghers syndrome, improving patient outcomes.
- Achieving a "clean small intestine" may extend the interval between necessary surgical interventions, reducing cumulative morbidity.
- The reported technique highlights a significant advancement in the endoscopic and surgical management of complex gastrointestinal polyposis syndromes.
Abstract:
The Peutz-Jeghers syndrome is an autosomal dominant inherited disease manifested by a combination of mucocutaneous pigmentation and gastrointestinal hamartomatous polyps that usually cause intussusception and intestinal hemorrhage. We report a case in which the patient has been followed-up on for 14 years and who underwent surgical and endoscopic polyp removal several times as well as one intestinal resection. This time, with the use of combined surgery and perioperative endoscopy, 27 polyps were removed, performing only 3 enterotomies. This is the highest number in one session to be reported in the literature. The usefulness of this technique is providing a "clean small intestine" that allows the patient a longer time interval between laparotomies and reduces the complications associated with multiple laparotomies and resections.