Point shear wave elastography in congenital metabolic liver diseases

Purpose: To evaluate hepatic and splenic shear wave velocity (SWV) values in patients with congenital metabolic liver diseases.

Materials & Methods: Eighty-four patients with different types of congenital metabolic liver diseases (Group 1) and 29 age, gender, body mass index-matched control patients with other metabolic disorders and normal liver functions (Group 2) were enrolled in this study. Hepatic and splenic SWV values were obtained by two sonologists with high interobserver agreement, using standard point shear wave elastography (pSWE) technique. The sonographic examinations were performed without being informed with clinical diagnoses.

Results: Group 1 consisted of 21 patients with sphingolipidosis, 30 patients with mucopolysaccharidosis, 10 patients with glycogenosis and 23 patients suffering intoxication type inborn metabolic diseases. Hepatic and/or splenic enlargement was significantly more common among the patients of Group 1. The SWV values obtained in the livers of the patients in Group 1 had a significantly higher median value (1.36 m/s; IQR = 1.23 – 1.56) than the ones obtained in Group 2 (1.26 m/s, IQR = 1.16 – 1.37). Median splenic SWV values of Group 1 and Group 2 were 2.74 m/s (IQR = 2.52 – 3.02) and 2.65 m/s (IQR = 2.46 – 2.72), respectively. The difference among them was not significant. Similarly, the ratios of splenic/hepatic SWV values were 1.94 m/s (IQR = 1.68 – 2.22) and 2.01 m/s (IQR = 1.83 – 2.26), respectively, and did not differ significantly. Further analysis of pSWE parameters among the patient subgroups and control group did not yield any significant difference.

Conclusion: We concluded that congenital metabolic liver diseases result in higher hepatic SWV values compared to other diseases of metabolism, suggesting increased liver stiffness due to disease processes.