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Congenital pulmonary lymphangiectasia

  • Shi-Min Yuan EMAIL logo
Published/Copyright: March 4, 2017
Journal of Perinatal Medicine
From the journal Journal of Perinatal Medicine Volume 45 Issue 9

Abstract

Congenital pulmonary lymphangiectasia (CPL) is a rare but fatal disease, usually having an onset from the first few hours to days after birth. Inconsistent nomenclatures were used for CPL in the past decades. Patients often present with intractable respiratory failure, hydrops fetalis and even sudden death. The etiologies of CPL remain unclear. Previous hypotheses suggested that CPL might be caused by conditions preventing normal regression of the lymphatics after the 18th–20th week of gestation. Up-to-date biological studies on lymphatic development, lymphatic valve formation and occurrence of hydrops fetalis revealed possible causative relations with mutations of genes of the vascular endothelial growth factor receptor (VEGFR), RAS/MAPK, PI3K/AKT and NF-κB signaling pathways. Lung biopsy with subsequent histological and immunohistochemical studies is a gold standard of CPL diagnosis. Apart from symptomatic and supportive treatments, novel regimens including sirolimus, a mammalian target of rapamycin (mTOR) inhibitor, one of the inhibitors of the pertinent signaling pathways and ethiodized oil lymphatic embolization under ultrasound-guided intranodal lymphangiography have shown encouraging short-term therapeutic effects for lymphatic anomalies. Surgical operations (lobectomy or pneumonectomy) can be the treatment of choice for patients with CPL confined to one lobe or one lung. Patients with CPL usually have a poor prognosis and often die during the neonatal period. Their prognoses are expected to improve with the development of modern therapeutic agents.

Keywords: Congenital pulmonary lymphangiectasia; lymphatic vessels; respiratory insufficiency
Corresponding author: Professor Shi-Min Yuan, MD, PhD, Department of Cardiothoracic Surgery, The First Hospital of Putian, Teaching Hospital, Fujian Medical University, 389 Longdejing Street, Chengxiang District, Putian 351100, Fujian Province, People’s Republic of China, Tel.: +86-594-6923117

Author’s statement

  1. Conflict of interest: Authors state no conflict of interest.

  2. Material and methods: Informed consent: Informed consent has been obtained from all individuals included in this study.

  3. Ethical approval: The research related to human subject use has complied with all the relevant national regulations, and institutional policies, and is in accordance with the tenets of the Helsinki Declaration, and has been approved by the authors’ institutional review board or equivalent committee.

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Received: 2016-9-11
Accepted: 2017-2-1
Published Online: 2017-3-4
Published in Print: 2017-12-20

©2017 Walter de Gruyter GmbH, Berlin/Boston

Articles in the same Issue

  1. Frontmatter
  2. Corner of Academy
  3. Perinatal critical care and ethics in perinatal medicine: the role of the perinatologist
  4. Review articles
  5. The impact of psychological distress during pregnancy on the developing fetus: biological mechanisms and the potential benefits of mindfulness interventions
  6. Characteristics and management of mirror syndrome: a systematic review (1956–2016)
  7. Congenital pulmonary lymphangiectasia
  8. Original articles
  9. A definition of gentle ventilation in congenital diaphragmatic hernia: a survey of neonatologists and pediatric surgeons
  10. Three-dimensional ultrasound first trimester fetal volume measurement and its relation to pregnancy outcome
  11. Evidence of altered brain regulatory gene expression in tobacco-exposed fetuses
  12. Planned home birth and the association with neonatal hypoxic ischemic encephalopathy
  13. The impact of maternal obesity on completion of fetal anomaly screening
  14. The effect of fish oil supplementation on maternal and neonatal outcomes: a triple-blind, randomized controlled trial
  15. Letter to the Editor
  16. Respect for professors: an often underappreciated component of professionalism in medical education
  17. Congress Calendar
  18. Congress Calendar
https://doi.org/10.1515/jpm-2016-0407
Keywords for this article
Congenital pulmonary lymphangiectasia; lymphatic vessels; respiratory insufficiency

Articles in the same Issue

  1. Frontmatter
  2. Corner of Academy
  3. Perinatal critical care and ethics in perinatal medicine: the role of the perinatologist
  4. Review articles
  5. The impact of psychological distress during pregnancy on the developing fetus: biological mechanisms and the potential benefits of mindfulness interventions
  6. Characteristics and management of mirror syndrome: a systematic review (1956–2016)
  7. Congenital pulmonary lymphangiectasia
  8. Original articles
  9. A definition of gentle ventilation in congenital diaphragmatic hernia: a survey of neonatologists and pediatric surgeons
  10. Three-dimensional ultrasound first trimester fetal volume measurement and its relation to pregnancy outcome
  11. Evidence of altered brain regulatory gene expression in tobacco-exposed fetuses
  12. Planned home birth and the association with neonatal hypoxic ischemic encephalopathy
  13. The impact of maternal obesity on completion of fetal anomaly screening
  14. The effect of fish oil supplementation on maternal and neonatal outcomes: a triple-blind, randomized controlled trial
  15. Letter to the Editor
  16. Respect for professors: an often underappreciated component of professionalism in medical education
  17. Congress Calendar
  18. Congress Calendar
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