Table 1.
Treatments and outcomes of congenital intrahepatic arterioportal fistula reported in literature, n (%)
| Type of first-line treatment | Overall (n = 44, 100%) | Type-1 IAPF (n = 19, 43.2%) | Type-2 IAPF (n = 10, 22.7%) | Type-3 IAPF (n = 10, 22.7%) | Type not specified (n = 5, 11.4%) |
| Radiological embolization | 39 (88.6) | 17 (89.5) | 10 (100) | 8 (80) | 4 (80) |
| -Overall success rate | 31 (79.5) | 15 (88.2) | 7 (70) | 5 (62.5) | 4 (100) |
| -Success rate for one procedure | 21 (53.8) | 13 (76.5) | 4 (40) | 3 (37.5) | 1 (25) |
| -Success rate for multiple procedures | 10 (25.6) | 2 (11.8) | 3 (30) | 2 (25) | 3 (75) |
| -Patients requiring subsequent surgery | 8 (20.5) | 2 (11.8) (liver resection) | 3 (30) (1 LT, 1 liver resection/arterial ligation, 1 end-to-side porto-caval shunt) | 3 (37.5) (1 endo-to-side portocaval shunt, 1 LT, 1 persistent PH) | - |
| Surgery | 5 (11.4) | 2 (10.5) | - | 2 (20) | 1 (20) |
| Arterial ligation | 4 (9.1) | 2 (10.5) | - | 1 (10) | 1 (20) |
| -Success rate | 2 (50) | 2 (100) | - | 0 (0) (persistent shunt) | 0 (0) (required subsequent embolization) |
| Liver resection | 1 (2.3) | - | - | 1 (10) | - |
| -Success rate | 1 (100) | - | - | 1 (100) | - |
IAFP: Intrahepatic arterioportal fistula; LT: Liver transplantation; PH: Portal hypertension.