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

Naoya Kinota, Hiroshi Kodama, Taiki Moriyama, Junichi Taniguchi, Mitsunari Maruyama, Atsushi Ogasawara, Yasukazu Kako, Kaoru Kobayashi, Haruyuki Takaki, Akio Shibata, Kyoko Minagawa, Yasuhiro Takeshima, Koichiro Yamakado
Hyogo College of Medicine. Ashiya Municipal Hospital. Shimane University.
Japan

Interventional Radiology
Interv Radiol 2022; 7: 17-20
DOI: 10.22575/interventionalradiology.2021-0014

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.

Category
Pulmonary Lymphatic Disease
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease

Age Focus: Pediatric Pulmonary Vascular Disease

Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication

Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No

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