Liu Jinrong, Yao Chunmei, Xu Baoping, Shen Wenbin, Zhou Chunju, Duan Xiaomin, Zhou Jin, An Ran, Wang Wei, Ding Zhaolu, Zhao Shunying
Department of Respiratory Diseases, Beijing Children's Hospital Affiliated to Capital Medical University,Beijing 100045, China.
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Zhonghua Er Ke Za Zhi. 2014 May;52(5):362-7.
To analyze the clinical characteristics and diagnosis of 2 cases with chylothorax due to primary lymphatic dysplasia and to elevate pediatrician's recognition level for this disease.
Clinical manifestations of the children were retrospectively analyzed. Primary lymphatic dysplasia was diagnosed by lymphoscintigraphy.
The first patient was a male aged 2-year-7-month who presented with a history of tachypnea for 43 days, fever and sore throat for 5 days at the early stage of the illness. He had a history of external injury before his illness. Physical examination showed his left chest bulging and left side diminished breath sound. His pleural effusion showed dark red (It was divided into two layers after standing, the upper layer turned into milky white, and the lower turned into hemorrhagic liquid) . White blood cell (WBC) count was 9 000×10(6)/L, mononuclear cell was 0.9, polykaryocytes was 0.1, triglyceride was 12.37 mmol/L in the pleural effusion. Contrast-enhanced lung CT (revascularization) showed pericardial effusion and a massive left sided pleural effusion. The second patient was a male aged 9 years and 6 months, who presented with a history of cough for 24 days, intermittent fever, vomiting, abdominal pain for 19 days, and edema of lower limbs for 4 days. Physical examination showed edema in both eyelids, lower extremities and scrotum. The level of albumin was 14 g/L and the titer of Mycoplasma pneumoniae IgM was 1: 320 in the serum. His hydrothorax pleural effusion showed milk white. White blood cell (WBC) count was 74×10(6)/L, mononuclear cell was 0.78, polykaryocytes was 0.22, triglyceride was 1.01 mmol/L in the pleural effusion. Chyle test showed positive in his pleural effusion and seroperitoneum. High-resolution CT of the lung revealed bilateral interstitial and parenchymal infiltration and both sided pleural effusion. Abdominal ultrasound showed giant hypertrophy of the gastric mucosa and massive ascites. Gastroscopy showed giant hypertrophy of the gastric mucosa. Lymphoscintigraphy revealed primary lymphatic dysplasia in both children.
Primary lymphatic dysplasia might occur in children and result in dropsy of serous cavity (chylothorax, chylopericardium, chylous ascites). Dropsy of serous cavity showed bloody or milk white. WBC count might elevate with lymphocyte increasing mostly, triglyceride was often higher than 1.0 mmol/L in dropsy of serous cavity. Primary lymphatic dysplasia can be diagnosed by lymphoscintigraphy.
分析2例原发性淋巴管发育异常所致乳糜胸的临床特点及诊断方法，以提高儿科医师对该病的认识水平。
对患儿的临床表现进行回顾性分析。通过淋巴闪烁显像诊断原发性淋巴管发育异常。
第1例患者为2岁7个月男性，病初有43天的呼吸急促史，早期有5天发热、咽痛史。病前有外伤史。体格检查示左胸膨隆，左侧呼吸音减弱。胸腔积液呈暗红色（静置后分为两层，上层变为乳白色，下层变为血性液体）。胸腔积液白细胞（WBC）计数为9 000×10⁶/L，单核细胞0.9，多核细胞0.1，甘油三酯12.37 mmol/L。增强肺CT（血管造影）示心包积液及大量左侧胸腔积液。第2例患者为9岁6个月男性，有24天咳嗽史，19天间歇性发热、呕吐、腹痛，4天下肢水肿史。体格检查示双侧眼睑、下肢及阴囊水肿。血清白蛋白水平为14 g/L，肺炎支原体IgM滴度为1∶320。胸腔积液呈乳白色。胸腔积液白细胞（WBC）计数为74×10⁶/L，单核细胞0.78，多核细胞0.22，甘油三酯1.01 mmol/L。胸腔积液及腹腔积液乳糜试验阳性。肺部高分辨率CT显示双侧间质及实质浸润，双侧胸腔积液。腹部超声显示胃黏膜巨大肥厚及大量腹水。胃镜显示胃黏膜巨大肥厚。淋巴闪烁显像显示2例患儿均为原发性淋巴管发育异常。
原发性淋巴管发育异常可发生于儿童，导致浆膜腔积液（乳糜胸、乳糜心包、乳糜腹水）。浆膜腔积液呈血性或乳白色。白细胞计数可升高，以淋巴细胞升高为主，浆膜腔积液中甘油三酯常高于1.0 mmol/L。原发性淋巴管发育异常可通过淋巴闪烁显像诊断。
