Chonnam Med J.  1999 Sep;35(3):407-412.

A Case of Peutz-Jeghers Syndrome Associated with Jejunojejunal and Colocolic Intussusception

Affiliations
  • 1Division of Pediatric Surgery, Department of Surgery, Chonnam National University Medical School, Kwangju, Korea.
  • 2Department of Pathology, Chonnam National University Medical School, Kwangju, Korea.

Abstract

Peutz-Jeghers syndrome is an inherited syndrome characterized by mucocutaneous melanotic pigmentation and multiple gastrointestinal polyps. It is transmitted as an autosomal dominant trait. The principal complication of Peutz-Jeghers syndrome is intestinal obstruction, which may occur in infancy or childhood. This complication is most prominent in the small intestine because of its narrower diameter. The polyps are really harmatomas, patients with Peutz-Jeghers syndrome are increased risk for cancers. We have treated a case of Peutz-Jeghers syndrome with multiple intussusceptions. This case is a 3-year and 4-month-old girl who had multiple polyps in the stomach, jejunum and sigmoid colon. On physical examination, multiple pigmented lesions were noted in the lips and oral mucosa and there was a large, mild tender, and mobile sausage like mass in the left upper quadrant. We perefored laparotomy, intussusceptions were identified in the jejunum and sigmoid colon. Intussusecptions were reduced manually and then large multiple polyps as the leading point of the intussusception were removed through enterotomy. Gastric polyps were removed by endoscopic resection. Histologic evaluation confirmed all polyps to be harmartomas.

Keyword

Peutz-Jeghers Syndrome; Polyps; Intussusception; Harmartomas
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