[III-AEPCYIA-05] Embolization of hepato-duodenal lymphatic fistulae as treatment for protein losing enteropathy after Fontan
キーワード：protein losing enteropathy, lymphangiography, lymphatic embolization, n-butyl cyanoacrylate, Fontan
Aims & background: To determine early and medium term results of selective embolization of hepato-duodenal lymph vessels in Fontan patients with protein losing enteropathy (PLE).
Methods: Using ultrasound guidance, dilated lymph vessels in periportal position were percutaneously punctured with a 22G Chiba needle. Intralymphatic position was confirmed by water soluble contrast injection with drainage to hepato-duodenal fistulae to the gut. Occlusion of hepato-duodenal lymphatics was effected by injection of 1-4 cc mixture 4-6/1 of lipiodol/n-butyl cyanoacrylate (Histoacryl®).
Results: 18 patients with proven PLE were treated at median age 15.3 (range 6.0 - 38.8) years. Fontan palliation was performed at 3.7 (range: 1.4-10.0) years; clinical PLE started 3.6 (range: 0.9-15.7) years later. Procedural complications were limited: portal thrombus (n = 4), abdominal discomfort in all for 24 up to 48 hours, transient cholangitis (n =1), and caustic duodenal bleeding/melena (n = 1). In 56% of patients (10/18), a lasting improvement in clinical PLE was obtained after one to four embolizations after a median follow-up period of 1.8 (Q 1.3; 2.9 ) years. In these, serum albumin improved significantly from a median of 23.6 (range: 20 – 34) g/l to a median of 38.5 (range: 32.0 – 44.0)g/l [p =0.003]. The procedure tended to be unsuccessful in cachectic patients, long-standing advanced disease, and when ascites was present.
Conclusions: Embolization of hepatico-duodenal lymphatics is a promising technique in Fontan patients with PLE and already outclasses current medical strategies. However in 40% the presumed leaks cannot be reached from the periportal region and will require another diagnostic and therapeutic approach, especially when ascites is present. Larger series with longer follow-up are needed to determine long term results and effects on liver function.
Methods: Using ultrasound guidance, dilated lymph vessels in periportal position were percutaneously punctured with a 22G Chiba needle. Intralymphatic position was confirmed by water soluble contrast injection with drainage to hepato-duodenal fistulae to the gut. Occlusion of hepato-duodenal lymphatics was effected by injection of 1-4 cc mixture 4-6/1 of lipiodol/n-butyl cyanoacrylate (Histoacryl®).
Results: 18 patients with proven PLE were treated at median age 15.3 (range 6.0 - 38.8) years. Fontan palliation was performed at 3.7 (range: 1.4-10.0) years; clinical PLE started 3.6 (range: 0.9-15.7) years later. Procedural complications were limited: portal thrombus (n = 4), abdominal discomfort in all for 24 up to 48 hours, transient cholangitis (n =1), and caustic duodenal bleeding/melena (n = 1). In 56% of patients (10/18), a lasting improvement in clinical PLE was obtained after one to four embolizations after a median follow-up period of 1.8 (Q 1.3; 2.9 ) years. In these, serum albumin improved significantly from a median of 23.6 (range: 20 – 34) g/l to a median of 38.5 (range: 32.0 – 44.0)g/l [p =0.003]. The procedure tended to be unsuccessful in cachectic patients, long-standing advanced disease, and when ascites was present.
Conclusions: Embolization of hepatico-duodenal lymphatics is a promising technique in Fontan patients with PLE and already outclasses current medical strategies. However in 40% the presumed leaks cannot be reached from the periportal region and will require another diagnostic and therapeutic approach, especially when ascites is present. Larger series with longer follow-up are needed to determine long term results and effects on liver function.