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ORIGINAL RESEARCH article

Front. Pediatr.

Sec. Neonatology

This article is part of the Research TopicNew Trends in Infant and Neonatal PulmonologyView all articles

Causes and risk factors for death in infants with congenital chylothorax

Provisionally accepted
Yuichi  KuboYuichi Kubo1*Satoshi  IbaraSatoshi Ibara1Takuya  TokuhisaTakuya Tokuhisa2Masaya  KibeMasaya Kibe3Kazuyoshi  SueyoshiKazuyoshi Sueyoshi2Masato  KamitomoMasato Kamitomo2Motoi  KatoMotoi Kato4
  • 1Shonan Fujisawa Tokushukai Hospital, Kanagawa, Japan
  • 2Kagoshima Shiritsu Byoin, Kagoshima, Japan
  • 3Imakiire Sogo Byoin, Kagoshima, Japan
  • 4Kagoshima Daigaku Byoin, Kagoshima, Japan

The final, formatted version of the article will be published soon.

Aim: To identify mortality risk factors in infants with congenital chylothorax (CC) and analyze the causes of death, including pathological examination. Methods: For this single-center retrospective study, we included 27 patients with CC. We divided them into the ALIVE and the DEAD groups, with the DEAD group subdivided into the Early-DEAD and the Late-DEAD groups to compare patient characteristics and risk factors using robust statistical methods. Causes of death were reviewed, including pathological findings from autopsies. Results: The mortality rate was 44% (12 of 27 infants, with 15 survivors). Univariate analysis showed associations between mortality and chromosomal abnormalities and fetal ascites. A longer duration of hydrothorax in utero was specifically associated with early neonatal death. Multivariate regression analysis identified fetal ascites as the strongest independent predictor of mortality. Pathological examination of six autopsied fatal cases revealed lymphangiectasia, including systemic lymphangiectasia in all five whole-body autopsies. Conclusion: Poor prognosis in infants with CC is strongly associated with fetal ascites, which we hypothesize serves as a key clinical marker for underlying Generalized Lymphatic Dysplasia (GLD). This systemic lymphatic disorder, identified pathologically as lymphangiectasia in fatal cases, may be the primary driver of mortality. Chromosomal abnormalities appear to be a significant predisposing factor for this severe phenotype.

Keywords: Congenital chylothorax, Cause of Death, Lymphangiectasia, Infant Mortality, Risk factors, Chromosome abnormalities

Received: 05 Sep 2025; Accepted: 31 Oct 2025.

Copyright: © 2025 Kubo, Ibara, Tokuhisa, Kibe, Sueyoshi, Kamitomo and Kato. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Yuichi Kubo, kubo_u1@yahoo.co.jp

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