Congenital Portosystemic Shunts: Variable Clinical Presentations Requiring a Tailored Endovascular or Surgical Approach

Abstract

Congenital portosystemic shunts (CPSS) are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation. These shunts allow intestinal blood to reach the systemic circulation directly, and if persistent or large, may lead to long-term complications. CPSS can have a variety of clinical presentations that depend on the substrate that is bypassing hepatic metabolism or the degree of hypoperfusion of the liver. Many intrahepatic shunts spontaneously close by 1 year of age, but extrahepatic and persistent intrahepatic shunts require intervention by a single session or staged closure with a multidisciplinary approach. Early detection and appropriate management are important for a good prognosis. The aim of this case series is to describe the varied clinical presentations, treatment approaches, and outcomes of 5 children with CPSS at our institution. Management of these patients should involve a multidisciplinary team, including interventional radiology, surgery, hepatology, and other medical services as the patient's clinical presentation warrants. Regardless of clinical presentation, if a CPSS persists past 1-2 years of age, closure is recommended.

Keywords: endovascular closure; hepatic tumors; hyperinsulinism; interventional radiology; portosystemic shunt.

FIGURE 1.

Imaging and embolization of the intrahepatic shunt. A and B) The patient’s ultrasound and CT showed an abnormal connection between the middle hepatic vein (arrow) and right portal vein (arrowhead), near the periphery of the liver. C and D) The shunt (arrow) was embolized from a transjugular approach using detachable coils. CT = computerized tomography.

FIGURE 2.

Imaging and embolization of multiple intrahepatic shunts within hepatic hemangiomas. A and B) Ultrasound with Doppler and MR imaging showed multiple hepatic hemangiomas containing portosystemic shunts (arrow). C and D) Coil embolization of four of the dominant portosystemic shunts (arrow) within hemangiomas was performed. MR = magnetic resonance.

FIGURE 3.

Preprocedure imaging, Interventional Radiology test occlusion venograms and post surgical follow-up imaging of the portosystemic shunt. A) Ultrasound showed an ill-defined lesion in the right lobe (arrow). B) MRI confirmed an indistinct right lobe lesion however the enhancement pattern was not specific for a benign or malignant lesion (arrow). C) CT showed an extrahepatic shunt (arrow) with a small left portal vein and a definitive right portal vein could not be detected. D, E) Venograms demonstrated a short and wide portosystemic shunt (arrow) from the diminutive left portal vein which drained directly into the inferior vena cava. Occlusion venogram showed redirected blood flow to the liver and filling of portal vein radicals (arrowhead). However the gradient was elevated and the morphology was favorable for surgical closure. F) Follow-up images show no signs of portal hypertension with good hepatopetal portal flow and resolution of the liver tumors. CT = computerized tomography; MRI = magnetic resonance imaging.

FIGURE 4.

Interventional Radiology test occlusion venograms, embolization and follow-up imaging of the portosystemic shunt. A) Venograms demonstrated cavernous transformation of the main portal vein (arrowhead) and a long and narrow intrahepatic portosystemic shunt (arrow) connecting the superior mesenteric vein/splenic vein confluence with the left hepatic vein. B) Occlusion venogram showed multiple intrahepatic portal vein radicals (arrowhead). C) A 16-mm Amplatzer 2 plug (arrow) was deployed at the proximal aspect of the shunt. D) Follow-up ultrasound imaging shows complete occlusion of the shunt with the Amplatzer 2 plug (arrow) and preservation of flow within the left hepatic vein and left portal vein.

FIGURE 5.

Preprocedure imaging, Interventional Radiology test occlusion venograms, embolization and follow-up imaging of the portosystemic shunts. A and B) Coronal MR image and initial venogram demonstrated two intrahepatic shunts (arrows) connecting the main portal vein to the IVC with aneurysmal drainage at the hepatic dome. C) The first shunt was occluded using a 12-mm Amplatzer 1 plug (arrow) deployed from a transjugular approach. D) Test occlusion of the second shunt performed with a Fogarty balloon did not show a significant increase in portal pressure. E) The second shunt was then occluded using a 14-mm Amplatzer 1 plug (arrowhead). F) Follow-up imaging showed occlusion of both shunt. IVC = inferior vena cava; MR = magnetic resonance.