Congenital intrahepatic portohepatic shunt managed by interventional radiologic occlusion: a case report and literature review.
Congenital intrahepatic portosystemic shunts are rare hepatic vascular anomalies that often lead to severe secondary conditions. A 6-year-old boy was referred for assessment of severe hypoxia, and a large liver mass was diagnosed with such a malformation and was managed by direct closure of the venous fistula by interventional radiology. Follow-up assessment shows normalization of the respiratory condition and a progressive reduction of the vascular liver lesion. Review of literature suggests that radiologic interventions are currently the criterion standard for managing these conditions, with surgery reserved for patients who are not eligible for radiologic procedure and those requiring liver transplantation.
Liver surgery and Transplantation Unit, Children's Hospital Bambino Gesù, Rome, Italy.
Chiara
C
Grimaldi
Lidia
L
Monti
Piergiorgio
P
Falappa
Giuseppe
G
d'Ambrosio
Antonio
A
Manca
Jean
J
de Ville de Goyet
D021721:Balloon Occlusion; D002648:Child; D003937:Diagnosis, Differential; D004417:Dyspnea / Q000209:etiology; D020518:Focal Nodular Hyperplasia / Q000175:diagnosis; D006503:Hepatic Veins / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D020065:Hepatopulmonary Syndrome / Q000209:etiology; D006801:Humans; D000860:Hypoxia / Q000209:etiology; D033162:Incidental Findings; D008099:Liver / Q000098:blood supply / Q000000981:diagnostic imaging / Q000473:pathology; D008102:Liver Circulation; D008279:Magnetic Resonance Imaging; D008297:Male; D011169:Portal Vein / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D011658:Pulmonary Fibrosis / Q000175:diagnosis; D011859:Radiography; D013152:Splanchnic Circulation; D018615:Ultrasonography, Doppler, Color; D016157:Vascular Fistula / Q000151:congenital* / Q000000981:diagnostic imaging / Q000601:surgery