Presentation of Congenital Portosystemic Shunts in Children

Atessa Bahadori, Beatrice Kuhlmann, Dominique Debray, Stephanie Franchi-Abella, Julie Wacker, Maurice Beghetti, Barbara E Wildhaber, Valérie Anne McLin, On Behalf Of The Ircpss, Atessa Bahadori, Beatrice Kuhlmann, Dominique Debray, Stephanie Franchi-Abella, Julie Wacker, Maurice Beghetti, Barbara E Wildhaber, Valérie Anne McLin, On Behalf Of The Ircpss

Abstract

Background: Congenital portosystemic shunts (CPSS) are rare vascular anomalies resulting in communications between the portal venous system and the systemic venous circulation, affecting an estimated 30,000 to 50,000 live births. CPSS can present at any age as a multi-system disease of variable severity mimicking both common and rare pediatric conditions.

Case presentations: Case A: A vascular malformation was identified in the liver of a 10-year-old girl with tall stature, advanced somatic maturation, insulin resistance with hyperinsulinemia, hyperandrogenemia and transient hematuria. Work-up also suggested elevated pulmonary pressures. Case B: A young girl with trisomy 8 mosaicism with a history of neonatal hypoglycemia, transient neonatal cholestasis and tall stature presented newly increased aminotransferase levels at 6 years of age. Case C: A 3-year-old boy with speech delay, tall stature and abdominal pain underwent abdominal ultrasound (US) showing multiple liver nodules, diagnosed as liver hemangiomas by hepatic magnetic resonance imaging (MRI). Management and outcome: After identification of a venous malformation on liver Doppler US, all three patients were referred to a specialized liver center for further work-up within 12 to 18 months from diagnosis. Angio-computed tomography (CT) scan confirmed the presence of either an intrahepatic or extrahepatic CPSS with multiples liver nodules. All three had a hyperintense signal in the globus pallidus on T1 weighted cerebral MRI. Right heart catheterization confirmed pulmonary hypertension in cases A and C. Shunts were closed either using an endovascular or surgical approach. Liver nodules were either surgically removed if there was a risk of malignant degeneration or closely monitored by serial imaging when benign.

Conclusion: These cases illustrate most of the common chief complaints and manifestations of CPSS. Liver Doppler US is the key to diagnosis. Considering portosystemic shunts in the diagnostic work-up of a patient with unexplained endocrine, liver, gastro-intestinal, cardiovascular, hematological, renal or neurocognitive disorder is important as prompt referral to a specialized center may significantly impact patient outcome.

Keywords: Abernethy malformation; congenital portosystemic shunt; hepatopulmonary syndrome; liver nodules; pulmonary arterial hypertension; tall stature.

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Anatomical forms of congenital portosystemic shunts. Reproduced from Guérin et al. Congenital portosystemic shunts: Vascular liver diseases: Position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver. Clin. Res. Hepatol. Gastroenterol. 452–459. Copyright © 2022 Elsevier Masson SAS. All rights reserved [5].
Figure 2
Figure 2
Case C. Aspect on imaging of the congenital porto-systemic shunt (CPSS) and the liver nodules. (1) Axial view on US B-mode at the level of the portal bifurcation shows the direct communication between the portal bifurcation and the inferior vena cava (IVC) (dotted arrow). There is a large liver tumor in the left hepatic lobe (large white arrow). (2) Axial view on MRI with contrast injection at the arterial phase shows the strong early enhancement of multiple hepatic nodules corresponding to hepatocellular proliferation. (3) Coronal view of an abdominal contrast enhanced computed tomography (CT) at the portal phase shows the direct communication between the portal bifurcation and the IVC. (4) Direct portal venography after occlusion of the CPSS with a balloon inflated in the IVC (star) shows hypoplastic right portal vein that was not visible on non-invasive imaging (plain arrow).

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