Kaposiformes kongenitales Hämangioendotheliom als Geburtshindernis und postpartaler Notfall

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• 作者：Dr. E. Verjans ; S. Franzen ; P. Vaeßen ; S. Schrading…
• 关键词：Vascular tumors ; Kasabach ; Merritt phenomenon ; Steroid dependency ; Sirolimus ; Vincristine
• 刊名：Monatsschrift Kinderheilkunde
• 出版年：2016
• 出版时间：April 2016
• 年：2016
• 卷：164
• 期：4
• 页码：318-322
• 全文大小：1,657 KB
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• 作者单位：Dr. E. Verjans (1)
  S. Franzen (1)
  P. Vaeßen (1)
  S. Schrading (2)
  H.P. Berlien (3)
  K. Heimann (1)
  T. Orlikowsky (1)
  N. Wagner (1)
  L. Lassay (1)
  
  1. Klinik für Kinder- und Jugendmedizin, Universitätsklinikum Aachen, RWTH Aachen, Pauwelsstr. 30, 52074, Aachen, Deutschland
  2. Klinik für diagnostische und interventionelle Radiologie, Universitätsklinikum Aachen, RWTH Aachen, Aachen, Deutschland
  3. Abteilung für Lasermedizin, Evangelisches Elisabeth-Krankenhaus, Berlin, Deutschland
  
• 刊物类别：Medicine
• 刊物主题：Medicine & Public Health
  Pediatrics
  General Practice and Family Medicine
  
• 出版者：Springer Berlin / Heidelberg
• ISSN：1433-0474

文摘

Kaposiform hemangioendothelioma (KHE) is a rare, weakly malignant vascular tumor that occurs nearly exclusively during infancy and childhood. It is often complicated by the Kasabach-Merritt phenomenon (KMP), severe thrombocytopenia and consumptive coagulopathy which can lead to severe hemorrhagic complications. This article describes the case of a term newborn with a protracted birth caused by a fulminant swollen, red-colored right arm and a threatening circulation disorder of the ipsilateral hand. The clinical, radiological and laboratory findings including histological analysis were in line with the diagnosis of congenital KHE with KMP. High-dose administration of prednisolone, acetylsalicylic acid and later sirolimus therapy resulted in a clear reduction of the tumor mass and recovery from thrombocytopenia. During the time of treatment the tumor became steroid-dependent and in the further course also vincristine-dependent; therefore, laser therapy was initiated, which successfully caused the tumor to disappear. Additional treatment with propranolol showed no positive effects. Neonatal KHE can present as an emergency and as in this case requires immediate treatment due to thrombocytopenia and imminent loss of hand function from circulation failure.