Pediatric Gastroenterology, Hepatology & Nutrition

The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition (대한소아소화기영양학회)
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Resolution of Protein-Losing Enteropathy after Congenital Heart Disease Repair by Selective Lymphatic Embolization

  • Kylat, Ranjit I (Department of Pediatrics, The University of Arizona College of Medicine) ;
  • Witte, Marlys H (Department of Surgery, The University of Arizona College of Medicine) ;
  • Barber, Brent J (Department of Pediatrics, The University of Arizona College of Medicine) ;
  • Dori, Yoav (Center for Lymphatic Imaging and Interventions, Children's Hospital of Philadelphia) ;
  • Ghishan, Fayez K (Department of Pediatrics, The University of Arizona College of Medicine)
  • Received : 2018.09.20
  • Accepted : 2019.01.01
  • Published : 2019.11.15
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Abstract

With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, postdouble switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ${\leq}0.9g/dL$) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.

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References

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