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

Interventional radiology (Higashimatsuyama-shi (Japan) Pub Date : 2022-01-12 eCollection Date: 2022-03-01 DOI:10.22575/interventionalradiology.2021-0014

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

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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.

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淋巴管造影治疗顽固性先天性乳糜胸(附2例报告)

在这项研究中，我们报告两例结内淋巴管造影有助于改善先天性乳糜胸由于ras病。在保守治疗未能改善一名患有心表皮综合征的8岁女孩和一名患有努南综合征的2个月男孩的乳糜胸后，我们进行了淋巴管造影。超声引导下用25号针穿刺腹股沟淋巴结，分别注射碘化油4 mL和1 mL，可见碘化油回流至肺内。两例患者经淋巴结淋巴管造影后乳糜胸均有改善。然而，第二个孩子经历了疾病相关的四肢水肿恶化，4个月后死于败血症。

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Interventional radiology (Higashimatsuyama-shi (Japan)

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