Angiographic diagnosis for accurate assessment of congenital porto‐systemic shunt and extrahepatic portal vein obstruction in children

Abstract

Background Congenital porto‐systemic shunt (CPSS) is a rare disease and can cause fatal complications. Accurate angiographic assessment is mandatory for proper treatment. Although technically difficult, we developed assessment techniques and assessed their accuracy. One technique came from evaluating patients with extrahepatic portal vein obstruction (EHPVO). Methods We conducted a single center retrospective study to evaluate the efficacy of angiographic diagnostic procedure for the assessment of CPSS and EHPVO, and its impact on patients’ subsequent interventions and clinical course. Eight patients with CPSS and two patients with EHPVO who underwent diagnostic angiography were included. Assessment of the intrahepatic portal vein was performed in all patients. The route of the shunt, and portal vein pressure under shunt occlusion, were also evaluated for patients with CPSS. Evaluation was first attempted with a balloon angiographic catheter (standard method). Three additional techniques were performed as needed: (i) direct wedge‐catheter injection without balloon inflation, (ii) use of occlusion balloon in two patients, and (iii) hybrid angiography with sheath placement directly into the superior mesenteric vein. Results The standard method was sufficient in four patients. On the other hand, all three techniques were required in two patients each. One lost contact during follow up, but all other patients underwent optimal intervention. There were no complications related to the angiographic procedure. Conclusions Use of direct wedge‐catheter injection without balloon inflation, occlusion balloon, and hybrid catheterization improved the diagnostic yield in patients with CPSS or EHPVO.

Congenital extrahepatic Portosystemic shunt with hypoplasia of the intrahepatic inferior vena cava: A rare case report

“…Type IIB EH shunts may present with ectasia of the PV at the level of the shunt, as seen in our case [11] . CECT is typically performed next to further scrutinize the anatomy and location of the shunt [1] . Our patient had a dilated main PV connected to the IVC in a side-to-side fashion, and faintly visualized left and right PVs.…”

“…Congenital portosystemic shunts (CPS) are rare vascular malformations with a reported incidence of 1 in 30,000 to 1 in 50,000 live births which occur due to partial or complete diversion of portal blood into the systemic circulation [1] , [2] , [3] , [4] . A classification is made based on anatomy into intrahepatic (IH) and extrahepatic (EH) shunts, with physiologic, treatment, and prognostic differences existing between the 2 [ 3 , 5 – 7 ].…”

“…EH shunts arise from the main portal vein (PV) while IH shunts originate from the portal venous branches [8] . CPS usually occur as isolated anomalies; however, they can be associated with cardiac, gastrointestinal, and other vascular malformations as well as genetic syndromes [ 1 , 4 , 9 ]. Vascular abnormalities associated with CPS are uncommon, and among those reported in the literature are splenic artery aneurysms, coronary artery fistulas, primitive hypoglossal artery, cutaneous and liver hemangiomas, azygos continuation of the inferior vena cava (IVC), and agenesis or double IVC [ 3 , 8 , 10 ].…”

“…Doppler ultrasound (US) has been the primary imaging test, allowing estimation of the shunt and subsequent follow-up and treatment monitoring [ 8 , 11 ]. Cross-sectional imaging with contrast-enhanced CT (CECT) and magnetic resonance imaging (MRI) are used to delineate shunt anatomy and characterize potential focal liver lesions [ 1 , 3 ]. Angiography is recently a superior and gold-standard diagnostic technique for CPS [1] .…”

“…Cross-sectional imaging with contrast-enhanced CT (CECT) and magnetic resonance imaging (MRI) are used to delineate shunt anatomy and characterize potential focal liver lesions [ 1 , 3 ]. Angiography is recently a superior and gold-standard diagnostic technique for CPS [1] .…”