Neonatal Medicine

The Korean Society of Neonatology (대한신생아학회)
권호 표지

A Case of Intestinal Hemangioma Complicated with Thrombocytopenia (Kasabach-Merritt syndrome) in Premature Infant

미숙아에서 혈소판 감소증에 의해 발견된 장 혈관종(Kasabach-Meritt 증후군) 1례

  • Lee, Young-Jin (Department of Pediatrics, Chonnam University Hospital) ;
  • Bae, Sul-Hee (Department of Pediatrics, Chonnam University Hospital) ;
  • Song, Eun-Song (Department of Pediatrics, Chonnam University Hospital) ;
  • Choi, Soo-Jin-Na (Department of Pediatric Surgery, Chonnam University Hospital) ;
  • Kim, Yoon-Ha (Department of Obstetrics and Gynecology, Chonnam University Hospital) ;
  • Choi, Young-Youn (Department of Pediatrics, Chonnam University Hospital)
  • 이영진 (전남대학교 의과대학 소아과학교실) ;
  • 배설희 (전남대학교 의과대학 소아과학교실) ;
  • 송은송 (전남대학교 의과대학 소아과학교실) ;
  • 최수진나 (전남대학교 의과대학 소아외과학교실) ;
  • 김윤하 (전남대학교 의과대학 산부인과학교실) ;
  • 최영륜 (전남대학교 의과대학 소아과학교실)
  • Published : 2010.05.31
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Abstract

Kasabach-Merritt syndrome is a rare thrombocytopenic consumptive coagulopathy associated with a giant hemangioma. We experienced a case of unexplained ascites with thrombocytopenia in a 32 week premature infant. An exploratory laparotomy was performed to determine the cause of the refractory ascites and thrombocytopenia. An intestinal hemangioma was found, but, surgical removal was not performed due to the extensive involvement. Hemangioma was confirmed by SPECT (single-photon emission computed tomography) and the thrombocytopenia was treated with steroid therapy. It is recommended that hemangioma of the visceral organs should be suspected when unexplained thrombocytopenia and disseminated intravascular coagulopathy persist.

Kasabach-Merritt 증후군은 거대한 혈관종에 의해 혈소판이 소모되어 감소되는 드문 질환이다. 저자들은 재태주령 32주 미숙아에서 설명되지 않은 복수와 혈소판 감소증의 원인을 찾기 위해 시험적 개복술을 시행하여 장 혈관종을 발견하였으나 광범위하여 수술적 제거를 하지 못하고, SPECT로 확인 후 스테로이드 치료로 혈소판 감소증이 호전된 1례를 경험하였다. 따라서 다른 원인에 의해 설명되지 않는 혈소판 감소증과 파종성 혈관내 응고병증이 있으면 내부 장기의 혈관종을 의심해 볼 필요가 있다고 사료되었다.

Keywords

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