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Congenital Chylothorax in a Neonate with Cornelia de Lange Syndrome: A Rare Complication Managed with a Novel Indigenously Prepared Milk Formulation

  • Ambika Gupta
  • Kirti M. NaranjeEmail author
  • Anita Singh
  • Aakash Pandita
  • Girish Gupta
  • Kaushik Mandal
  • Mandakini Pradhan
Clinical Brief
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Abstract

Congenital chylothorax is a relatively uncommon condition seen in newborn period. Treatment comprises of adequate pleural fluid drainage, octreotide infusion, total parenteral nutrition followed by medium chain triglyceride (MCT) based low fat milk preparations. The authors present a case of Cornelia de Lange syndrome with a rare presentation of antenatally diagnosed chlyothorax presenting at birth with respiratory distress. The baby was managed with skimmed milk formulation fortified with coconut oil providing low fat and high proteins.

Keywords

Chylothorax Cornelia de Lange Medium chain triglyceride Skimmed milk 

Notes

Authors’ Contributions

AG managed and wrote the case report. KN contributed to patient management, case report writing and proof reading the report. AS, AP and GG contributed intellectually, helped in patient management and writing the manuscript. KM helped in patient management. MP managed the patient’s mother antenatally. GG is the guarantor for this article.

Compliance with Ethical Standards

Conflict of Interest

None.

References

  1. 1.
    van Straaten HL, Gerards LJ, Krediet TG. Chylothorax in the neonatal period. Eur J Pediatr. 1993;152:2–5.CrossRefGoogle Scholar
  2. 2.
    Uzun H, Senses DA, Uluba M, Kocabay K. A newborn with Cornelia de Lange syndrome: a case report. Cases J. 2008;1:329.CrossRefGoogle Scholar
  3. 3.
    Krantz ID, McCallum J, DeScipio C, et al. Cornelia de Lange syndrome is caused by mutations in NIPBL, the human homolog of Drosophilia melanogaster nipped-B. Nat Genet. 2004;36:631–5.CrossRefGoogle Scholar
  4. 4.
    Beghetti M, La Scala G, Belli D, et al. Etiology and management of pediatric chylothorax. J Pediatr. 2000;136:653–8.CrossRefGoogle Scholar
  5. 5.
    Gupta V, Mahendri NV, Tete P, Santhanam S. Skimmed milk preparation in management of congenital chylothorax. Indian Pediatr. 2014;51:146–8.CrossRefGoogle Scholar

Copyright information

© Dr. K C Chaudhuri Foundation 2019

Authors and Affiliations

  • Ambika Gupta
    • 1
  • Kirti M. Naranje
    • 1
    Email author
  • Anita Singh
    • 1
  • Aakash Pandita
    • 1
  • Girish Gupta
    • 1
  • Kaushik Mandal
    • 2
  • Mandakini Pradhan
    • 3
  1. 1.Department of NeonatologySanjay Gandhi Postgraduate Institute of Medical SciencesLucknowIndia
  2. 2.Department of Medical GeneticsSanjay Gandhi Postgraduate Institute of Medical SciencesLucknowIndia
  3. 3.Department of Maternal and Reproductive HealthSanjay Gandhi Postgraduate Institute of Medical SciencesLucknowIndia

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