Neurometabolism and brain morphometry in an adolescent female with an extra-hepatic congenital portosystemic shunt

Isaline Chabbey; Cristina Cudalbu; Eugénie Barras; Sylviane Hanquinet; Bénédicte Maréchal; Anne-Laure Rougemont; Julie Wacker; Florence Zangas-Gheri; Valérie A McLin

doi:10.1002/jpr3.12035

Neurometabolism and brain morphometry in an adolescent female with an extra-hepatic congenital portosystemic shunt

JPGN Rep. 2023 Dec 28;5(1):35-42. doi: 10.1002/jpr3.12035. eCollection 2024 Feb.

Authors

Isaline Chabbey¹, Cristina Cudalbu^{2

3}, Eugénie Barras⁴, Sylviane Hanquinet⁴, Bénédicte Maréchal^{5

6

7}, Anne-Laure Rougemont⁸, Julie Wacker⁹, Florence Zangas-Gheri¹⁰, Valérie A McLin¹¹

Affiliations

¹ Department of Pediatrics, Gynecology and Obstetrics, Swiss Pediatric Liver Center, Division of Pediatric Surgery University of Geneva Geneva Switzerland.
² CIBM Center for Biomedical Imaging Vaud Switzerland.
³ Animal Imaging and Technology Ecole Polytechnique Fédérale de Lausanne Lausanne Switzerland.
⁴ Diagnostic Department, Pediatric Radiology Unit, Radiology Division Geneva University Hospitals Geneva Switzerland.
⁵ Advanced Clinical Imaging Technology Siemens Healthineers International AG Lausanne Switzerland.
⁶ Department of Radiology, Lausanne University Hospital (CHUV) University of Lausanne Vaud Switzerland.
⁷ LTSS, Ecole Polytechnique Fédérale de Lausanne (EPFL) Lausanne Switzerland.
⁸ Division of Clinical Pathology, Swiss Pediatric Liver Center Geneva University Hospitals Geneva Switzerland.
⁹ Department of Pediatrics, Gynecology and Obstetrics, Pediatric Cardiology Unit University of Geneva, Geneva, Switzerland Geneva Switzerland.
¹⁰ Pediatric Neurology Unit Geneva University Hospitals Geneva Switzerland.
¹¹ Department of Pediatrics, Gynecology and Obstetrics, Swiss Pediatric Liver Center, Pediatric Gastroenterology, Hepatology and Nutrition Unit University of Geneva Geneva Switzerland.

Abstract

Background: Chronic hepatic encephalopathy (CHE) has been reported both in patients with congenital porto-systemic shunts (CPSS) and chronic liver disease. CHE is difficult to recognize in children as there is no clear definition and its manifestations are highly variable. CHE is associated with variations in brain volumes and metabolites that have already been demonstrated using 1.5-3T MRI systems. However, the in-depth study of brain metabolism requires the high spectral resolution of high magnetic fields.

Objectives and methods: We analyzed the neurometabolic profile, brain volumes and T₁ relaxation times of a child with a CPSS using high field proton magnetic resonance spectroscopy (¹H MRS, 7T) combined with MRI and compared it to an age-matched control group. We also evaluated the impact of shunt closure on neurocognitive symptoms using adapted neuropsychological tests.

Results: 7T MRS revealed a significant increase in glutamine compared to controls, a decrease in brain osmolytes, and a slight elevation in NAA concentrations. 7T MRI scans showed morphological abnormalities but no changes in the signal intensity of the globus pallidus. Neurocognitive testing revealed attention deficit disorder, language difficulties, and mild intellectual disability. Most of these areas improved after shunt closure.

Conclusions: In this paediatric case of type B HE with normal fasting ammonia, neurometabolic profile was compatible with what has been previously shown in chronic liver disease, while also demonstrating an isolated glutamine peak. In addition, neurocognitive function partially improved after shunt closure, arguing strongly for shunt closure in both presymptomatic and symptomatic patients.

Keywords: chronic liver disease; congenital porto‐systemic shunts; hepatic encephalopathy; proton magnetic resonance spectroscopy.

© 2023 The Authors. JPGN Reports published by Wiley Periodicals LLC on behalf of The European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.