Lymphangiography as a Treatment for Refractory Congenital Chylothorax Due to RASopathies: A Report of Two Cases

• Kinota Naoya

  Department of Radiology, Hyogo College of Medicine

• Kodama Hiroshi

  Department of Radiology, Ashiya Municipal Hospital

• Moriyama Taiki

  Department of Radiology, Hyogo College of Medicine

• Taniguchi Junichi

  Department of Radiology, Hyogo College of Medicine

• Maruyama Mitsunari

  Department of Radiology, Shimane University

• Ogasawara Atsushi

  Department of Radiology, Hyogo College of Medicine

• Kako Yasukazu

  Department of Radiology, Hyogo College of Medicine

• Kobayashi Kaoru

  Department of Radiology, Hyogo College of Medicine

• Takaki Haruyuki

  Department of Radiology, Hyogo College of Medicine

• Shibata Akio

  Department of Pediatrics, Hyogo College of Medicine

• Minagawa Kyoko

  Department of Pediatrics, Hyogo College of Medicine

• Takeshima Yasuhiro

  Department of Pediatrics, Hyogo College of Medicine

• Yamakado Koichiro

  Department of Radiology, Hyogo College of Medicine

Abstract

In this study, we report two cases in which intranodal lymphangiography helped improve congenital chylothorax due to RASopathies. We performed lymphangiography after conservative treatments failed to improve chylothorax in an 8-year-old girl with cardiofaciocutaneous syndrome and a 2-month-old boy with Noonan syndrome. Inguinal lymph nodes were punctured with 25-gauge needles under ultrasonographic guidance, and 4 and 1 mL of iodized oil were injected, respectively, showing a backflow of iodized oil into the lungs. Chylothorax had improved in both patients after nodal lymphangiography. However, the second child experienced worsening of disease-associated extremity edema and died of sepsis 4 months later.

Journal

• Interventional Radiology

  Interventional Radiology 7 (1), 17-20, 2022-03-01

  Japanese Society of Interventional Radiology

Keywords

• Lymphangiography
• Rasopathies
• Pediatrics
• Chylothorax
• Chyle leak

Details

• CRID: 1390573242454755712
• NII Article ID: 130008166448
• DOI: 10.22575/interventionalradiology.2021-0014
• ISSN: 24320935
• Web Site: https://www.jstage.jst.go.jp/article/interventionalradiology/7/1/7_2021-0014/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2023116232
• Text Lang: en
• Data Source:
  • JaLC
  • Crossref
  • CiNii Articles
  • KAKEN
• Abstract License Flag: Disallowed