Imaging Features of Lymphatic Malformation in Children
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摘要: 淋巴管发育异常性病变是儿童较常见的脉管性病变，本文通过不同的影像学成像技术、成像方法对淋巴回流障碍、淋巴管扩张症、淋巴管畸形、淋巴管瘤病的影像学特点及疾病的解剖基础和病理生理机制进行综述，直观地显示淋巴发育异常性疾病特征性的影像改变，为临床诊治淋巴管疾病提供精准的和适当的诊疗信息及监测疗效的手段。Abstract: Lymphangiodysplasia is a common vascular disease in children. This paper summarizes the imaging characteristics, anatomical basis and pathophysiological mechanism of lymphatic reflux disorder, lymphangiodilation, lymphangiopathy and lymphangiomatosis through different imaging techniques and methods, so as to intuitively display the characteristic imaging changes of lymphatic dysplasia. It provides accurate and appropriate diagnosis and treatment information, as well as the means of monitoring curative effect for clinical diagnosis and treatment of lymphatic diseases.
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Keywords:
- radiology /
- magnetic resonance lymphangiography /
- child /
- lymphatic malformations
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图 1 男，6天。以"生后气促6天"入院。左侧大量胸腔积液，胸水常规检查提示白细胞升高，胸水内甘油三酯含量高—新生儿乳糜胸
Figure 1. Male, 6 days old, admitted to hospital with "postnatal shortness of breath for 6 days". Large amount of pleural effusion of the left side. Routine examination of the pleural effusion indicates elevated leukocytes and high triglyceride content in pleural effusion-neonatal chylothorax
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图 2 男， 1岁半，间断浮肿3月余，加重2周，诊断为上腔静脉阻塞综合征
Figure 2. 1.5 years old boy with intermittent edema for more than 3 months and aggravation for 2 weeks, the diagnosis is superior vena cava obstruction syndrome
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图 3 塑形性支气管炎患儿的动态增强磁共振淋巴管造影（DCMRL），双胸导管（短箭头）和异常肺淋巴流（长箭头）[9]
Figure 3. DCMRL of the patient with PB, demonstrating double Thoracic duct (arrowheads) and abnormal pulmonary lymphatic flow (arrows)
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图 4 男，6天。以“生后呼吸困难6天”入院，诊断为原发性肺淋巴管扩张症
（a）～（d）胸部CT平扫，两肺可见弥漫分布网格影、索条影，肺门区未见病灶。大血管形态位置正常，纵隔内未见明显肿大淋巴结影。
Figure 4. Male, 6 days old. Admitted to hospital with "dyspnea for 6 days after birth", the diagnosis is CPL
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图 5 女，5月，自幼右颈部肿物，近期增大。右侧口咽至下颌骨旁软组织内不规则囊性包块，T1W低信号T2W以高信号为伴等低信号，提示淋巴管瘤伴囊内出血，且口咽及喉咽部气道受压变窄移位
Figure 5. Female, 5 months old recent enlargement of right neck tumor after birth. An irregular cystic mass with T1W low signal and T2W high signal with equal low signal in right oropharynx to paramandibular soft tissue, suggestive of lymphangioma with hemorrhage, as well as the compression of the oropharyngeal and hypopharyngeal airway
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图 6 男，3岁5个月，腹痛一周伴呕吐，肠系膜淋巴管瘤伴肠扭转。右中下腹液性低密度灶，其内可见肠系膜血管穿行和肠系膜根部扭转
Figure 6. Male, 3 years 5 months old, Abdominal pain for a week with vomiting，and diagnosed mesenteric lymphangioma with volvulus. MIP showed a water-like focus in the right middle and lower abdomen with mesenteric vessels and mesenteric root torsion
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