Biko David M, Reisen Breanne, Otero Hansel J, Ravishankar Chitra, Victoria Teresa, Glatz Andrew C, Rome Jonathan J, Dori Yoav
Department of Radiology, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, 3401 Civic Center Blvd., Philadelphia, PA, USA.
Division of Cardiology, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.
Pediatr Radiol. 2019 May;49(5):586-592. doi: 10.1007/s00247-018-04337-6. Epub 2019 Jan 6.
Children with Noonan syndrome are known to have increased risk for lymphatic disorders, the extent and nature of which are poorly understood.
Our objective was to describe the imaging findings of the central lymphatic abnormalities in children with Noonan syndrome who underwent central lymphatic imaging.
We conducted a single-center retrospective review of all children with a confirmed history of Noonan syndrome who presented for lymphatic imaging over a 5-year period. Imaging evaluation was performed on unenhanced T2-weighted (T2-W) imaging, dynamic-contrast MR lymphangiography or conventional lymphangiography. Two readers evaluated the imaging in consensus for the distribution of fluid on T2-W imaging and for lymphatic flow of intranodal contrast agent and thoracic duct abnormalities on dynamic-contrast MR lymphangiography and conventional lymphangiography. We performed a chart review for clinical history and outcomes.
We identified a total of 10 children, all but one of whom had congenital heart disease. Presenting symptoms included chylothorax (n=9) and ascites (n=1). Nine had T2-W imaging, seven had dynamic-contrast MR lymphangiography, and seven had conventional lymphangiography. All with T2-W imaging had pleural effusions. On both dynamic-contrast MR lymphangiography and conventional lymphangiography, perfusion to the lung was seen (n=6), with intercostal flow also seen on dynamic-contrast MR lymphangiography (n=6). The thoracic duct was not present in three children and the central thoracic duct was not present in three. A double thoracic duct was seen in two children.
Children with Noonan syndrome and clinical evidence of lymphatic dysfunction have central lymphatic abnormalities characterized by retrograde intercostal flow, pulmonary lymphatic perfusion, and thoracic duct abnormalities.
已知努南综合征患儿患淋巴系统疾病的风险增加，但其程度和性质尚不清楚。
我们的目的是描述接受中央淋巴系统成像的努南综合征患儿中央淋巴系统异常的影像学表现。
我们对一家单中心在5年期间内接受淋巴系统成像检查且有确诊努南综合征病史的所有患儿进行了回顾性研究。成像评估采用非增强T2加权（T2-W）成像、动态对比磁共振淋巴造影或传统淋巴造影。两名阅片者就T2-W成像上液体的分布、动态对比磁共振淋巴造影和传统淋巴造影上淋巴结内造影剂的淋巴流动以及胸导管异常达成共识进行评估。我们查阅病历以了解临床病史和结果。
我们共确定了10名患儿，除1名外均患有先天性心脏病。主要症状包括乳糜胸（n = 9）和腹水（n = 1）。9名患儿进行了T2-W成像，7名进行了动态对比磁共振淋巴造影，7名进行了传统淋巴造影。所有进行T2-W成像的患儿均有胸腔积液。在动态对比磁共振淋巴造影和传统淋巴造影上均可见肺灌注（n = 6），动态对比磁共振淋巴造影上也可见肋间血流（n = 6）。3名患儿未见胸导管，3名患儿未见中央胸导管。2名患儿可见双胸导管。
有淋巴功能障碍临床证据的努南综合征患儿存在中央淋巴系统异常，其特征为肋间血流逆行、肺淋巴灌注和胸导管异常。
