Pediatr Gastroenterol Hepatol Nutr.  2019 Nov;22(6):594-600. 10.5223/pghn.2019.22.6.594.

Resolution of Protein-Losing Enteropathy after Congenital Heart Disease Repair by Selective Lymphatic Embolization

Affiliations
  • 1Department of Pediatrics, The University of Arizona College of Medicine, Tucson, AZ, USA. rkylat@gmail.com
  • 2Department of Surgery, The University of Arizona College of Medicine, Tucson, AZ, USA.
  • 3Center for Lymphatic Imaging and Interventions, Children's Hospital of Philadelphia, Philadelphia, PA, USA.

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.

Keyword

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

MeSH Terms

Adhesives
Arteries
Cardiac Surgical Procedures
Child
Child, Preschool
Chyle
Chylous Ascites
Embolization, Therapeutic
Endoscopy, Gastrointestinal
Female
Heart Defects, Congenital*
Heart Septal Defects, Ventricular
Humans
Ligation
Lymphatic Abnormalities
Lymphography
Pleurodesis
Postoperative Complications
Protein-Losing Enteropathies*
Pulmonary Atresia
Rehabilitation
Serum Albumin
Thoracic Duct
Tomography, Emission-Computed
Weight Gain
Adhesives
Serum Albumin

Figure

  • Fig. 1 Lymphangioscintigram and single photon emission computed tomography (SPECT). (A) Bilateral Tc99m sulfur colloid lower-extremity lymphangioscintigraphy showing rapid transport from foot injection sites central to the cisterna chyli (arrow) and later tracer dispersion. (B) SPECT localizing an abnormal area in the duodenal loop (arrow) corresponding to thickening with a loss of wall (arrows) throughout the duodenal loop as seen on magnetic resonance enterography.

  • Fig. 2 Gastrointestinal endoscopy image showing chylous lymphangiectasia localized to the duodenal loop (A, B) and complete resolution after the procedure (C, D).

  • Fig. 3 Magnetic resonance enterography image showing a thickened lymphangiectatic duodenal loop (arrow) corresponding to loss of lymph throughout the duodenal loop.

  • Fig. 4 Selective intranodal magnetic resonance lymphangiogram recorded prior to embolization showing enhanced contrast medium uptake by the lymphatic system and retrograde flow into the intestinal lymphatics from the intestinal lymphatic trunk, confirming a duodenal obstruction.

  • Fig. 5 Simultaneous blue dye injection in the thoracic duct along with endoscopic visualization of the duodenum demonstrating leakage of the dye into the duodenal lumen.

  • Fig. 6 (A) Conventional lymphangiogram showing the stump of the thoracic duct (TD) and the intestinal trunk prior to embolization. (B) After embolization of the intestinal lymphatic trunk with 0.5 cc of TrueFill glue, a glue cast is visible in the intestinal trunk. A microcatheter remains in the remnant of the TD.


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