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Neonatal lymphatic flow disorders: impact of lymphatic imaging and interventions on outcomes

Journal of Perinatology volume 41, pages 494–501 (2021)Cite this article

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Abstract

Neonatal chylothorax (NCTx) and central lymphatic flow disorder (CLFD) are historically challenging neonatal disorders with high morbidity and mortality.

Methods

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.

Results

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).

Conclusions

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.

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Fig. 1: Dynamic contrast MR lymphangiography (DCMRL) in neonatal chylothorax and CLFD.
Fig. 2: Survival and mechanical ventilation outcomes.

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Acknowledgements

This work was supported in part by the Cardiac Center Clinical Research Core at the Children’s Hospital of Philadelphia.

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Authors and Affiliations

  1. Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Erin Pinto, Yoav Dori, Christopher Smith, Aaron DeWitt, Catherine Williams, Heather Griffis, Fernando Escobar, David M. Biko, Ganesh Krishnamurthy, Jonathan Rome, Andrew C. Glatz, Mandi Liu, Chitra Ravishankar, Huayan Zhang & Dalal Taha

  2. University of Pennsylvania, Philadelphia, PA, USA

    Yoav Dori, Christopher Smith, Aaron DeWitt, Fernando Escobar, David M. Biko, Ganesh Krishnamurthy, Jonathan Rome, Andrew C. Glatz, Chitra Ravishankar, Huayan Zhang & Dalal Taha

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  1. Erin Pinto
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Correspondence to Dalal Taha.

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Pinto, E., Dori, Y., Smith, C. et al. Neonatal lymphatic flow disorders: impact of lymphatic imaging and interventions on outcomes. J Perinatol 41, 494–501 (2021). https://doi.org/10.1038/s41372-020-00771-3

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