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

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.

Direct EBUS‐guided transtracheal lymphosclerosis for plastic bronchitis after Fontan

“…If the TD cannot be cannulated, lymph access to the TD can be reduced by destruction of the cisterna chyli by needle maceration 2 . Finally, the distal leaks can be sealed either by sclerosing effect of lipiodol from inguinal node or after direct cannulation of the TD 13 ; more efficient is direct puncture of intrathoracic lymph vessels/nodes with glue injection, either percutaneous 14 or transtracheal as reported in this manuscript.…”

Case Report: Disseminated Systemic Embolism of Lipiodol After Lymphography for Plastic Bronchitis After Fontan Repair

“…Nevertheless, some alternative techniques to treat PB after Fontan repair exist. Lymphatic leaks in PB can also be sealed without transductal access by direct peritracheal puncture techniques either percutaneous or transtracheal (12,13).…”

Liver Lymphatic Imaging and Interventions: Resurrection of the Forgotten Knowledge

“…The application of the liver lymphangiography, followed by interstitial embolization of these abnormal connections, was suggested as a potential treatment method of PLE in this patient group. 23,26 The goal of embolization here is to deliver NBCA glue to the leaking point in the duodenum, all the way through from liver lymphatics to the periduodenal retroperitoneal lymphatics that finally distribute in the duodenal mucosa. Injection of the methylene blue through the liver lymphangiography route and simultaneous endoscopic monitoring of duodenal mucosa helps confirm that liver lymph leakage into the duodenum is the cause of PLE.…”

“…Among them, six patients showed significant improvement in the serum albumin level and quality of life with minimal procedure-related complications. 26…”