Children's Hospital of Philadelphia, Philadelphia, PA, USA.
University of Pennsylvania, Philadelphia, PA, USA.
J Perinatol. 2021 Mar;41(3):494-501. doi: 10.1038/s41372-020-00771-3. Epub 2020 Sep 2.
Neonatal chylothorax (NCTx) and central lymphatic flow disorder (CLFD) are historically challenging neonatal disorders with high morbidity and mortality.
We conducted a retrospective study of 35 neonates with pulmonary lymphatic abnormalities at our institution who underwent lymphatic evaluation between December 2015 and September 2018. Patients with only pulmonary lymphatic perfusion syndrome were classified as NCTx and those with multiple flow abnormalities were classified as CLFD. Demographics, clinical characteristics, and outcomes were compared using t-tests/Wilcoxon rank sum tests and Fisher's exact tests.
All 35 patients had intranodal MR lymphangiography and 14 (40%) also had conventional fluoroscopic lymphangiography. Fifteen (42.8%) patients were diagnosed with NCTx and 20 (57.1%) were diagnosed with CLFD. Thirty-four (97.1%) patients had pleural effusions. None of the NCTx group had ascites, anasarca, or dermal backflow compared to 17 (85%) (p < 0.001), 8 (42.1%) (p: 0.004), and 20 (100%) (p < 0.001) of the CLFD group, respectively. In the NCTx group, 11 (73.3%) had ethiodized oil embolization and 4 (26.7%) received conservative therapy. Ten (50%) of the CLFD patients had an intervention; of those, two (10%) had ethiodized oil-only embolization. Eight had non-ethiodized oil embolizations (two (25%) had embolization with glue, three (37.5%) underwent surgical lymphovenous anastomosis, two (25%) underwent thoracic duct (TD) externalization, and one (12.5%) had a non-TD lymphatic channel drain placed). Complete resolution of pleural effusions was achieved in all 15 NCTx patients, whereas 9 (45%) of 20 CLFD patients had resolution of chylothorax (p: 0.001).
Establishing a diagnosis of NCTx or CLFD is paramount in selecting treatment options and providing prognostic information. Development of lymphatic interventions represents a paradigm shift in our understanding of neonatal lymphatic flow disorders and may be associated with improved survival.
新生儿乳糜胸（NCTx）和中央淋巴液流动障碍（CLFD）是具有高发病率和死亡率的具有挑战性的新生儿疾病。
我们对我院 2015 年 12 月至 2018 年 9 月间进行淋巴评估的 35 例肺淋巴异常新生儿进行了回顾性研究。将仅存在肺淋巴灌注综合征的患者归类为 NCTx，而将存在多种流动异常的患者归类为 CLFD。使用 t 检验/Wilcoxon 秩和检验和 Fisher 确切检验比较患者的人口统计学、临床特征和结局。
所有 35 例患者均进行了节内 MR 淋巴管造影术，其中 14 例（40%）还进行了常规荧光透视淋巴管造影术。15 例（42.8%）患者被诊断为 NCTx，20 例（57.1%）患者被诊断为 CLFD。34 例（97.1%）患者存在胸腔积液。与 CLFD 组的 17 例（85%）（p<0.001）、8 例（42.1%）（p：0.004）和 20 例（100%）（p<0.001）相比，NCTx 组无一例出现腹水、全身性水肿或皮肤回流。在 NCTx 组中，11 例（73.3%）接受了碘化油栓塞，4 例（26.7%）接受了保守治疗。CLFD 组中有 10 例（50%）进行了干预；其中 2 例（10%）仅接受了碘化油栓塞。8 例接受了非碘化油栓塞（2 例（25%）采用胶栓塞，3 例（37.5%）进行了外科淋巴静脉吻合术，2 例（25%）进行了胸导管（TD）外置，1 例（12.5%）进行了非 TD 淋巴管引流）。15 例 NCTx 患者的胸腔积液均完全消退，而 20 例 CLFD 患者中有 9 例（45%）乳糜胸消退（p：0.001）。
明确诊断 NCTx 或 CLFD 对于选择治疗方案和提供预后信息至关重要。淋巴介入技术的发展代表了我们对新生儿淋巴液流动障碍的理解发生了重大转变，可能与生存率的提高有关。
