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Peutz-Jeghers syndrome managed with a complete intraoperative endoscopy and extensive polypectomy

2000; Elsevier BV; Volume: 52; Issue: 4 Linguagem: Inglês

10.1067/mge.2000.107213

1097-6779

Rafael Amaro, Gloria Diaz, Jeffrey A. Schneider, Michael D. Hellinger, Neil Stollman,

Metastasis and carcinoma case studies

The Peutz-Jeghers syndrome is an autosomal dominant disorder with incomplete penetrance characterized by GI hamartomatous polyps and mucocutaneous pigmentation. This syndrome was first described in 1921 by Peutz, 1 Peutz JLA Very remarkable case of familial polyposis mucous membrane of intestinal tract and nasopharynx accompanied by peculiar pigmentation of skin and mucous membranes. Ned Maandschr Geneeskd. 1921; 10: 134-146 Google Scholar followed by Jeghers et al. 2 Jeghers H McKusick VA Katz KH Generalized intestinal polyposis and melanin spots of the oral mucosa, lip and digits: a syndrome of diagnostic significance. N Engl J Med. 1949; 241: 1031-1036 Crossref PubMed Scopus (318) Google Scholar in 1949. Although hamartomatous polyps are typically benign lesions, they are responsible for the most common complications of Peutz-Jeghers syndrome such as intussusception, intestinal obstruction, and GI bleeding. Further, malignant change of the hamartomatous polyps has been reported. 3 Konishi F Wyse NE Muto T Sawada T Morioka Y Sugimura H et al. Peutz-Jeghers polyposis associated with carcinoma of the digestive organs. Report of three cases and review of the literature. Dis Colon Rectum. 1987; 30: 790-799 Crossref PubMed Scopus (76) Google Scholar The classical surgical management with multiple enterotomies and removal of all palpable polyps has been modified in recent years to a combined surgical-endoscopic approach using intraoperative enteroscopy. This is a desription of a patient with Peutz-Jeghers syndrome presenting with chronic abdominal pain and iron deficiency anemia who underwent a complete intraoperative endoscopy with extensive removal of hamartomatous polyps.