Approach to Refractory Chylous Effusion


Ann Kulungowski, MD, and Taizo Nakano, MD
Children's Hospital Colorado, Aurora, CO

Guest Presenters

Maxim Itkin, MD, FSIR
Professor of Radiology and Pediatrics
Center for Lymphatic Disorders
University of Pennsylvania Medical Center
Nemours Children's Hospital

Deborah Rabinowitz, MD
Division Chief, Interventional Radiology
Department of Medical Imaging
Nemours Children's Hospital

Motoi Kato, MD
Department of Plastic and Reconstructive Surgery
Saitama Children's Medical Center, Japan

Learning Objectives

  • To increase awareness of and advocate for pediatric patients with vascular anomalies
  • To understand how to establish proper diagnosis and treatment of pediatric vascular anomalies
  • To create an international forum for debate, discussion, and dialog on approaches for treating pediatric patients with pediatric anomalies

Case Summary

  • Baby girl presented with prenatal diagnosis of macrocystic lymphatic malformation of the left chest and axilla
  • Enlarging cyst on 20-wk ultrasound
  • Fetal MRI: multiseptated mass, axillary and left thoracic soft tissues
  • Birth hx: Cesarean at 39 weeks GA
  • Symptomatic (mobility) lesion treated with sclerotherapy x 3
  • Follow up at ~2 yr of age reporting that the malformation took on more bluish hue, concern for venous component, with palpable phleboliths
  • MRI imaging recommended, but not pursued
  • Patient acutely presented with increased WOB
    • Large left pleural effusion with mediastinal shift
    • Admitted with placement of chest tube
    • Output confirmed Chylous
    • Initiation of complete NPO/TPN

Questions Discussed

  • How early should one treat a child with this condition?
  • How can lesions evolve over time?
  • What is the proper diagnosis?
  • How does chylous effusion or ascites differ from other complex congenital lymphatic anomalies?
  • What are options for diagnostic imaging?
  • What are options for therapeutic interventions?
  • What are options for surgical therapeutic interventions?
  • What are options for ongoing managing of the conditon?


Patient Case Discussion

Loading video...


Rate This Presentation: 
Your rating: None
No votes yet