Case Report: Clinical Features of Congenital Portosystemic Shunts in the Neonatal Period

Suhua Xu,Peng Zhang,Guoqiang Cheng,Wenhao Zhou,Liyuan Hu

doi:10.3389/fped.2021.778791

Suhua Xu, Peng Zhang + Show 3 more

Open Access

https://doi.org/10.3389/fped.2021.778791

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Abstract

Objective: The aim of this single-center retrospective study was to analyze the clinical characteristics, treatment options, and course of neonatal-onset congenital portosystemic shunts (CPSS).Methods: We included all patients with CPSS who presented with clinical symptoms within the neonatal period in our institution between 2015 and 2020.Results: Sixteen patients were identified, including 13 patients with intrahepatic portosystemic shunts (IPSS) and three patients with extrahepatic portosystemic shunts (EPSS). The median age of diagnosis was 16 days (range prenatal 24 weeks−12 months). Hyperammonemia (60%), neonatal cholestasis (44%), elevated liver enzyme (40%), hypoglycemia (40%), thrombocytopenia (38%), and coagulation abnormalities (23%) appeared in neonatal CPSS. Twelve patients (75%) presented with congenital anomalies, of which congenital heart disease (CHD) (44%) was the most common. Thirteen patients with IPSS initially underwent conservative treatment, but two of them were recommended for the catheter interventional therapy and liver transplantation, respectively, due to progressive deterioration of liver function. Spontaneous closure occurred in nine patients with IPSS. The shunt was closed using transcatheter embolization in one patient with EPSS type II. Another patient with EPSS type II underwent surgical treatment of CHD firstly. The remaining patient with EPSS type Ib received medical therapy and refused liver transplantation.Conclusion: Hyperammonemia, neonatal cholestasis, elevated liver enzyme, hypoglycemia, and thrombocytopenia are the main complications of neonatal CPSS. Moreover, CPSS is associated with multiple congenital abnormalities, especially CHD. Intrahepatic portosystemic shunts may close spontaneously, and conservative treatment can be taken first. Extrahepatic portosystemic shunts should be closed to prevent complications.

Highlights

Congenital portosystemic shunt (CPSS) is a rare vascular malformation in which the venous blood from the intestines and spleen bypasses the liver and diverts directly into the systemic circulation through abnormal vessels
The CPSS was classified as intrahepatic portosystemic shunts (IPSS) and extrahepatic portosystemic shunts (EPSS) based on abdominal ultrasonography (US), contrast-enhanced computerized tomography (CT), contrast-enhanced magnetic resonance imaging (MRI), digital subtraction angiography (DSA), or a combination of these techniques
Intrahepatic portosystemic shunts is not limited to one lobe of the liver and can be composed of multiple portosystemic connections

Summary

Introduction

Congenital portosystemic shunt (CPSS) is a rare vascular malformation in which the venous blood from the intestines and spleen bypasses the liver and diverts directly into the systemic circulation through abnormal vessels. Congenital portosystemic shunt has been associated with congenital malformations, like congenital heart disease (CHD), and Down syndrome, and with multisystem complications including antenatal hemodynamic abnormalities, neonatal cholestasis, hepatopulmonary syndrome, pulmonary hypertension, and even hepatic tumors. The appropriate treatment strategies of CPSS, including conservative therapy, transcatheter embolization, shunt vessel ligation, and liver transplantation, should take into account many factors, mainly the shunt type, shunt ratio, patient age, and the presence of complication [1,2,3]. This study aimed to retrospectively analyze the clinical features, diagnosis, therapeutic treatment, and outcomes of the patients with CPSS who developed symptoms from the neonatal period

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Discussion

Conclusion