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Non-immune hydrops fetalis secondary to congenital chylothorax with diffuse interstitial lung disease: a diagnostic conundrum
  1. Alvin Jia Hao Ngeow1,
  2. Mei Yoke Chan2,
  3. Oon Hoe Teoh3,
  4. Sarat Kumar Sanamandra4 and
  5. Daisy Kwai Lin Chan1
  1. 1Department of Neonatal and Developmental Medicine, Singapore General Hospital, Singapore
  2. 2Haematology/Oncology Service, Department of Paediatric Subspecialties, KK Women’s and Children’s Hospital, Singapore
  3. 3Respiratory Medicine Service, Department of Paediatrics, KK Women’s and Children’s Hospital, Singapore
  4. 4Department of Diagnostic Radiology, Singapore General Hospital, Singapore
  1. Correspondence to Dr Alvin Jia Hao Ngeow; alvin.ngeow.j.h{at}


A Chinese male infant was born at 35 weeks weighing 2935 g to a mother with polyhydramnios and prenatal hydrops fetalis. He developed marked respiratory distress secondary to bilateral congenital chylothorax and required pleural drainage, high frequency oscillation and inhaled nitric oxide therapy. He was extubated to non-invasive ventilation by day 14. There was no bacterial or intrauterine infection, haematologic, chromosomal or cardiac disorder. He was exclusively fed medium-chain triglyceride formula. High-resolution CT showed diffuse interstitial lung disease. He received a dexamethasone course for chronic lung disease to facilitate supplemental oxygen weaning. A multidisciplinary team comprising neonatology, pulmonology, haematology, interventional radiology and thoracic surgery considered congenital pulmonary lymphangiectasia as the most likely diagnosis and advised open lung biopsy, lymphangiography or scintigraphy for diagnostic confirmation should symptoms of chylothorax recur. Fortunately, he was weaned off oxygen at 5 months of life, and tolerated human milk challenge at 6 months of life and grew well.

  • paediatrics
  • materno-fetal medicine
  • neonatal and paediatric intensive care
  • neonatal health

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  • Contributors AN, primary physician, was in charge of the patient and wrote the manuscript. DKLC, senior neonatologist, was involved in managing the case, guided the writing and editing, and approved the final manuscript. MYC, senior paediatric haematologist-oncologist, was instrumental in organising the team at several vascular anomalies workgroup meetings and approved the manuscript. Senior paediatric pulmonologist OHT and radiologist SKS were key in making the diagnosis of diffuse interstitial lung disease and guiding a literature review of congenital pulmonary lymphangiectasia.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer-reviewed.

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