Matsumoto Tomohiro, Kudo Takahiro, Endo Jun, Hashida Kazunobu, Tachibana Nao, Murakoshi Takatsugu, Hasebe Terumitsu
Department of Radiology, Tokai University Hachioji Hospital, Tokai University School of Medicine , Tokyo , Japan.
Minim Invasive Ther Allied Technol. 2015;24(4):246-9. doi: 10.3109/13645706.2014.996162. Epub 2015 Jan 30.
Noonan syndrome, which is a multiple congenital disorder, may be associated with lymphatic abnormalities. Protein-losing enteropathy (PLE) developing in Noonan syndrome is rare. We performed transnodal lymphangiography by directly accessing bilateral inguinal nodes under ultrasound guidance in a 17-year-old female with PLE developing in Noonan syndrome to assess detailed anatomical findings regarding lymphatic vessels. There have been no reports on transnodal lymphangiography for Noonan syndrome. Post-lymphangiographic CT images revealed multiple lymphatic abnormalities and lipiodol extravasation into the duodenum and the proximal jejunum. Transnodal lymphangiography was easy and safe for PLE developing in Noonan syndrome, and post-lymphangiographic CT provided invaluable information.
努南综合征是一种多发性先天性疾病，可能与淋巴系统异常有关。在努南综合征中发生的蛋白丢失性肠病（PLE）很罕见。我们对一名17岁患有努南综合征并发生PLE的女性患者，在超声引导下直接穿刺双侧腹股沟淋巴结进行经淋巴结淋巴管造影，以评估淋巴管的详细解剖学发现。目前尚无关于努南综合征经淋巴结淋巴管造影的报道。淋巴管造影后的CT图像显示多处淋巴系统异常，且碘油渗入十二指肠和空肠近端。经淋巴结淋巴管造影对于努南综合征并发的PLE操作简便且安全，淋巴管造影后的CT提供了宝贵的信息。
