Pulmonary lymphangiectasia a rare cause of chylothorax: A case report
DOI:
https://doi.org/10.52783/jns.v11.1037Keywords:
Pulmonary lymphangiectasia, Chylothorax, NeonateAbstract
Background: Pulmonary lymphangiectasia (PL) is a rare disease characterized by dilation of the pulmonary lymphatic vessels. PL should be considered in the differential diagnosis in infants with pleural effusion, especially in the presence of chylothorax.
Case Presentation: A 3-week-old neonate presented with tachypnea, cough, and reluctance to feed. Work-up found bilateral chylous pleural effusion. The patient was initially managed conservatively with bilateral tube thoracostomies and octreotide, however output from chest tubes did not subside. A left thoracotomy was performed with wedge biopsy of the apical segment of the left inferior lung lobe and multiple titanium clips were used to control thoracic duct. Postoperatively the patient continued to have high chest tube output, despite placement of clips. Histology of the biopsy confirmed the diagnosis of congenital pulmonary lymphangiectasia.
Conclusion: Pulmonary lymphangiectasia is a rare cause of chylothorax. The treatment is often challenging as found in the index case.
Downloads
Metrics
References
Nikbakhsh N, Zamani M, Noorbaran A, Naghshineh A, Rastergar-Nejad D. Etiological approach of chylothorax in Babol, northern Iran. Caspian J Intern Med. 2017; 8:30-4.
Attar MA, Donn SM. Congenital chylothorax. Semin Fetal Neonatal Med. 2017; 22:234-9.
Yuan SM. Congenital pulmonary lymphangiectasia: A disorder not only of feto neonates. KlinPadiatr. 2017; 229:205-8.
Reiterer F, Grossauer K, Morris N, Uhrig S, Resch B. Congenital pulmonary lymphangiectasis. Paediatr Respir Rev. 2014; 15:275-80.
Bellini C, Boccardo F, Campisi C, Bonioli E. Congenital pulmonary lymphangiectasia. Orphanet J Rare Dis. 2006; 30:1-3.
Esther CR Jr, Barker PM. Pulmonary lymphangiectasia: diagnosis and clinical course. PediatrPulmonol. 2004; 38:308-13.
Baltaxe HA, Lee JG, Ehlers KH, Engle MA. Pulmonary lymphangiectasia demonstrated by lymphangiography in 2 patients with Noonan's syndrome. Radiology. 1975; 115:149-53.
Barker PM, Esther CR Jr, Fordham LA, Maygarden SJ, Funkhouser WK. Primary pulmonary lymphangiectasia in infancy and childhood. Eur Respir J. 2004; 24:413-9.
Published
How to Cite
Issue
Section
License
Copyright (c) 2022 James Conner, Richard Mahon, Juan Camps
This work is licensed under a Creative Commons Attribution 4.0 International License.
You are free to:
- Share — copy and redistribute the material in any medium or format
- Adapt — remix, transform, and build upon the material for any purpose, even commercially.
Terms:
- Attribution — You must give appropriate credit, provide a link to the license, and indicate if changes were made. You may do so in any reasonable manner, but not in any way that suggests the licensor endorses you or your use.
- No additional restrictions — You may not apply legal terms or technological measures that legally restrict others from doing anything the license permits.
