Intest Res.  2014 Oct;12(4):320-327. 10.5217/ir.2014.12.4.320.

Polyp Clearance via Operative and Endoscopic Polypectomy in Patients With Peutz-Jeghers Syndrome After Multiple Small Bowel Resections

• Lee DH
• Shin HD
• Cho WH
• Shin KH
• Lee S
• Shin JE
• Namgung H
• Gwon JE

• KMID: 2174410
• DOI: http://doi.org/10.5217/ir.2014.12.4.320

Abstract

Peutz-Jeghers syndrome is an autosomal dominant inherited disease that manifests as a combination of mucocutaneous pigmentation and gastrointestinal hamartomatous polyps that usually cause intussusception and intestinal hemorrhage. We report the case of a 40-year-old male patient who was diagnosed 20 years ago and had previously undergone 3 intestinal resection surgeries. This time, with the use of combined operative and endoscopic polypectomy, more than 100 polyps were removed. This technique is useful for providing a "clean" small intestine that allows the patient a long interval between laparotomies and reduces the complications associated with multiple laparotomies and resections.

Keyword

Peutz-Jeghers syndrome; Intussusception; Endoscopy; Polypectomy

MeSH Terms

Adult
Endoscopy
Hemorrhage
Humans
Intestine, Small
Intussusception
Laparotomy
Male
Peutz-Jeghers Syndrome*
Pigmentation
Polyps*

Figure

• 

  Fig. 1 Endoscopic findings. (A) Transient ileal telescoping to the ascending colon and tiny small polyps in ileum. (B) Large ileal polyp almost blocking the lumen. (C) Typical pedunculated hamartoma in ascending colon. (D) Classic hamartoma with multilobulated surface has few distinctive features compared to other polyps. This polyp showed high-grade dysplasia on histologic exam. (E) Multiple tiny gastric polyps on Narrow Band ImagingI view. (F) Severe luminal dilatation of the colon and large pedunculated polyps with thick stalks and large vessels in the proximal jejunum.

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  Fig. 2 Typical mucocutaneous pigmentation of Peutz-Jeghers syndrome. (A) Typical pigmentation on the vermilion border of the lips. (B) Pigmentation on the volar aspect of the hand. (C) A close-up of the hand pigmentation. (D) Classic pigmentation in the buccal mucosa.

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  Fig. 3 Radiologic findings of small bowel series. (A) Tiny scattered filling defects (white arrows) in the ileum and a large well-defined intraluminal filling defect (black arrow) in the jejunum. The filling defects are hamartomatous polyps. (B) Prominent luminal dilation of the jejunum and intussusceptions in the jejunum. The patient had no symptoms during the test.

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  Fig. 4 Abdominopelvic CT findings. (A) Typical intussusceptions in the proximal jejunum. (B) Prominent large fungating polyps (white arrows) and intussusceptions (black arrows) in the jejunum. (C) Intussusceptions containing mesenteric vessels and fat. (D) Intussusceptions containing mesenteric vessels and fat (white arrows) and a large ileal polyp (black arrow) in a coronal image.

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  Fig. 5 Surgical findings. (A) Evagination of the lumen after enterotomy of the proximal jejunum. (B) Stalk base of pedunculated polyp is tied off. (C) After being tied, the polyp is cut off. (D) Total of 76 surgically resected small bowel polyps.

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  Fig. 6 Microscopic findings. (A) Typical hamartomatous polyps characterized by tree-like branching bands of smooth muscle with normal surrounding hyperplastic epithelium (H&E, ×10). (B) Dysplastic change of Fig. 4D (H&E, ×40). (C) Proliferation of neoplastic epithelial cells with nuclear hyperchromasia and pseudostratification (H&E, ×100). (D) Marked increases in mitosis and nuclear pseudostratification in the basal half of the epithelial cells (H&E, ×200).

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