Resolution of protein-losing enteropathy after congenital heart disease repair by selective lymphatic embolization

Ranjit I. Kylat, Marlys H. Witte, Brent J. Barber, Yoav Dori, Fayez K. Ghishan

Research output: Contribution to journalArticle

2 Scopus citations

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, post-double switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ≤0.9 g/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.

Original languageEnglish (US)
Pages (from-to)594-600
Number of pages7
JournalPediatric Gastroenterology, Hepatology and Nutrition
Volume22
Issue number6
DOIs
StatePublished - 2019

Keywords

  • Cardiac surgical procedures
  • Embolization therapeutic
  • Heart defects congenital
  • Lymphatic abnormalities
  • Protein-losing enteropathies

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hepatology
  • Gastroenterology

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