Blue Rubber Bleb Nevus Syndrome 1예

A Case of Blue Rubber Bleb Nevus Syndrome

  • 강기수 (제주대학교 의과대학 소아과학교실) ;
  • 오현주 (제주대학교 의과대학 소아과학교실) ;
  • 고재성 (서울대학교 의과대학 소아과학교실) ;
  • 서정기 (서울대학교 의과대학 소아과학교실) ;
  • 박귀원 (서울대학교 의과대학 외과학교실) ;
  • 강경훈 (서울대학교 의과대학 병리과학교실) ;
  • 김우선 (서울대학교 의과대학 영상의학교실)
  • Kang, Ki-Soo (Department of Pediatrics, Cheju National University College of Medicine) ;
  • Oh, Hyun-Ju (Department of Pediatrics, Cheju National University College of Medicine) ;
  • Ko, Jae-Sung (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Seo, Jeong-Kee (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Park, Kwi-Won (Department of Surgery, Seoul National University College of Medicine) ;
  • Kang, Gyeong-Hoon (Department of Pathology, Seoul National University College of Medicine) ;
  • Kim, Woo-Sun (Department of Radiology, Seoul National University College of Medicine)
  • 투고 : 2008.07.02
  • 심사 : 2008.09.09
  • 발행 : 2008.09.30

초록

저자들은 만성적인 철결핍성 빈혈을 보이는 10세 여아에서, 캡슐내시경을 비롯한 내시경적 검사로 위장관계의 다발성 정맥기형을 발견하여 blue rubber bleb nevus syndrome으로 진단하였고, 광범위한 외과적 절제술로 성공적으로 치료하였기에 이에 보고하는 바이다.

We report a 10-year-old girl with the blue rubber bleb nevus syndrome (BRBNS) who had chronic severe anemia caused by chronic occult bleeding in the gastrointestinal (GI) tract. The patient was admitted to the hospital frequently for recurrent pallor and fatigue since the age of 7 years. Gastroduodenoscopy and capsule endoscopy revealed multiple venous malformations with blood oozing in the stomach, small bowel and colon. The patient was treated by aggressive surgical resection of the 23 vascular malformations in the GI tract. The patient is well without anemia 15 months post surgery.

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