Polypectomy by Intraoperative Total Gut Endoscopy in a Child with Peutz-Jeghers Syndrome

소아 Peutz-Jeghers 증후군 환자에서 전장 내시경술에 의한 용종 절제술 1례

Peutz-Jeghers syndrome is an autosomal dominant inherited syndrome characterized by mucocutaneous pigmentation and gastrointestinal hamartomatous polyps. The most important complications that increase morbidity are intussusception, bleeding and obstruction. Most patients with Peutz-Jeghers syndrome may undergo multiple laparotomies for complications such as intussusception or bleeding every 2 to 3 years during adolescence and early adulthood. To decrease the relaparotomy rate, intraoperative endoscopy may be useful in the treatment of complications that are related to Peutz-Jeghers syndrome. Use of intraoperative endoscopy can lead to a healthier life and to a longer life expectancy for the patient. We describe a case of Peutz-Jeghers syndrome, who underwent polypectomy by total gut endoscopy in an 11-year-old girl presented with intestinal obstruction and anemia. During the course of the operation, the endoscope was inserted per the enterostomy and colostomy sites, and 16 polyps in the small and large intestine were removed endoscopically using a snare.

수술 중 전장 내시경적 용종 절제술은 내시경이 도달할 수 없는 소장에 발생한 용종의 절제에 유용하며, Peutz-Jeghers 증후군 환자에서 삶의 질을 향상시키고 합병증으로 인한 수술의 빈도를 감소시킨다. 본 저자들은 장중첩증과 장 출혈에 의한 빈혈 및 장 폐쇄가 발생한 Peutz-Jeghers 증후군 환자에서 전장 내시경술에 의한 용종 절제술을 시행한 1례를 경험하였기에 문헌고찰과 함께 보고한다.