Percutaneous embolization of lymphatic fistulae as treatment for protein-losing enteropathy and plastic bronchitis in patients with failing Fontan circulation

Geert Maleux, Emma Storme, Bjorn Cools, Ruth Heying, Derize Boshoff, Jacoba J. Louw, Stefan Frerich, Sofie Malekzadeh-Milanii, Jelena Hubrechts, Stephen C. Brown, Marc Gewillig*

*Corresponding author for this work

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Abstract

Background To determine the feasibility and clinical result of selective embolization of hepatoduodenal or paratracheal lymphatics in Fontan patients with protein-losing enteropathy (PLE) or plastic bronchitis (PB). Methods Dilated lymph vessels in periportal (PLE) or paratracheal (PB) position were percutaneously punctured with a 22G Chiba needle. Intralymphatic position was confirmed by water soluble contrast injection with drainage to hepatoduodenal or tracheal fistulae. After flushing with 10% glucose solution, occlusion of hepatoduodenal or paratreacheal lymphatics was effected by injection of 1-4 cc mixture 4/1 of Lipiodol/n-butyl cyanoacrylate (n-BCA; Histoacryl). Results Seven patients with proven PLE were treated with periportal lymphatic embolization 10.7 (range: 6.6-13.5) years after the Fontan operation. The Fontan operation was performed at a median age of 3.7 (range: 2.9-5.7) years and PLE started a median of 3.1 (range: 0.9-4.7) years later. Five patients required a second procedure 2-8 months later. Complications were limited (spillage of glue in portal branch, transient cholangitis, and caustic duodenal bleeding). Six of seven patients reported significant improvement in quality of life and normalization of albumin levels after limited follow-up (p <.01). One patient (Fontan at 2.9 years; age 16.4 years) had PB for 2 years. Selective transthoracic cone-beam-directed puncture of left and right paratracheal lymphatics with n-BCA embolization of distal lymphatic fistulae resulted in lasting absence of tracheal casts (11 months). Conclusions Embolization of periportal/peritracheal lymphatics is a promising technique in Fontan patients with PLE/PB. Larger series are required to determine incidence and reasons of success/failure, with long-term results and effects on liver function.

Original languageEnglish
Pages (from-to)996-1002
Number of pages7
JournalCatheterization and Cardiovascular interventions
Volume94
Issue number7
DOIs
Publication statusPublished - 1 Dec 2019

Keywords

  • congenital heart disease
  • lymphangiography
  • lymphatic embolization
  • n-butyl cyanoacrylate
  • plastic bronchitis
  • protein losing enteropathy
  • THORACIC-DUCT
  • LYMPHANGIOGRAPHY
  • DECOMPRESSION
  • INTERVENTION
  • EXPERIENCE
  • MANAGEMENT
  • ADULTS

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