Howarth D, Gloviczki P
Department of Nuclear Medicine, John Hunter Hospital, Newcastle, New South Wales, Australia.
J Nucl Med. 1998 Sep;39(9):1635-8.
Chronic genital edema secondary to lymphangiectasia and chylous reflux in a 23-yr-old man with Noonan syndrome was investigated by 99mTc sulfur nanocolloid lymphoscintigraphy and bipedal contrast lymphangiography. Lymphoscintigraphy showed a delayed lymphatic flow pattern in the pelvis, abdomen and chest consistent with lymphangiectasia and abnormal lymphatic flow dynamics. Lymphangiography showed dilated and tortuous abnormal lymphatics in the abdomen and pelvis. Ligation of incompetent retroperitoneal lymph vessels and lymphaticovenous anastamosis were performed, resulting in clinical improvement. Lymphangiectasia has been described previously in Noonan syndrome, but it is relatively uncommon below the diaphragm. This case demonstrates the use of lymphoscintigraphy and lymphangiography in providing important physiological and anatomical information before surgical intervention. Careful presurgical planning using such tests also allows the most appropriate operation to be performed.
一名23岁患有努南综合征的男性因淋巴管扩张和乳糜反流继发慢性生殖器水肿，接受了99m锝硫纳米胶体淋巴闪烁造影和双足对比淋巴管造影检查。淋巴闪烁造影显示盆腔、腹部和胸部淋巴引流延迟，符合淋巴管扩张及异常淋巴流动动力学表现。淋巴管造影显示腹部和盆腔淋巴管扩张且迂曲异常。对功能不全的腹膜后淋巴管进行结扎并进行淋巴静脉吻合术，临床症状得到改善。此前已有努南综合征合并淋巴管扩张的报道，但膈肌以下相对少见。本病例展示了淋巴闪烁造影和淋巴管造影在手术干预前提供重要生理和解剖学信息方面的应用。使用此类检查进行仔细的术前规划也有助于实施最合适的手术。
