Complications, treatment, and follow-up of peutz-jeghers syndrome: About 2 case reports.

Case report Gastrointestinal intussusception General surgery Peutz jeghers polyposis Surgical polypectomy

Journal

International journal of surgery case reports
ISSN: 2210-2612
Titre abrégé: Int J Surg Case Rep
Pays: Netherlands
ID NLM: 101529872

Informations de publication

Date de publication:
Aug 2023
Historique:
received: 26 06 2023
revised: 10 07 2023
accepted: 15 07 2023
medline: 29 7 2023
pubmed: 29 7 2023
entrez: 28 7 2023
Statut: ppublish

Résumé

Peutz-Jeghers syndrome is an inherited disorder distinguished by hamartomatous polyps in the gastrointestinal tract and pigmented mucocutaneous lesions. Treatment of the polyps is never definitive, with most patients needing several laparotomies. For this reason, surgeons should be economical in terms of surgical resection to prevent a short bowel syndrome in the long run. In this paper, we report two observations of patients presented a Peutz Jeghers syndrome (PJS). Case report 1: A 32-year-old women, who was operated on for an intestinal perforation related to a Peutz-jeghers hamartoma of the small bowel and was later re operated on for colonic intussusception, Case report 2: A 15-year-old patient that has been operated on three times already for small bowel intussusception and later for duodenal obstruction. In an attempt to reduce complications, the 2010 guidelines updated in 2021 by the European Hereditary Tumor group introduced obligatory monitoring by fibroscopy and colonoscopy associated with an entero-MRI or a videocapsule from the age of 8 years. Laparotomy is indicated when endoscopic treatment is impossible or in emergency setting. When surgery is indicated, intestinal resection should be reserved for rare cases in order to avoid short bowel syndrome. The association of an intraoperative endoscopic treatment is recommended by some authors. Peutz Jeghers syndrome is a rare entity with a complicated surveillance. Adequate polyp mapping is necessary for adequate planning of the treatment. The need for multiple laparotomies makes a comprehensive approach to surgery mandatory to prevent short bowel syndrome.

Identifiants

pubmed: 37506527
pii: S2210-2612(23)00640-5
doi: 10.1016/j.ijscr.2023.108511
pmc: PMC10403703
pii:
doi:

Types de publication

Case Reports

Langues

eng

Pagination

108511

Informations de copyright

Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of competing interest All authors declare they have no conflict of interest.

Auteurs

Yacine Ouadi (Y)

Department of Surgery A La Rabta Hospital, Tunis, Tunisia; Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia. Electronic address: yacine.taha@gmail.com.

Maryem Ben Brahim (MB)

Department of Surgery A La Rabta Hospital, Tunis, Tunisia; Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia.

Emna Trigui (E)

Department of Surgery A La Rabta Hospital, Tunis, Tunisia; Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia.

Wassim Frikha (W)

Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia; Department of Radiology La Rabta hospital, Tunis, Tunisia.

Fadhel Fterich (F)

Department of Surgery A La Rabta Hospital, Tunis, Tunisia; Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia.

Montasser Jameleddine Kacem (MJ)

Department of Surgery A La Rabta Hospital, Tunis, Tunisia; Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia.

Classifications MeSH