Liver cirrhosis in children - the role of imaging in the diagnostic pathway

Abstract

Liver cirrhosis in children is a rare disease with multifactorial causes that are distinct from those in adults. Underlying reasons include cholestatic, viral, autoimmune, hereditary, metabolic and cardiac disorders. Early detection of fibrosis is important as clinical stabilization or even reversal of fibrosis can be achieved in some disorders with adequate treatment. This article focuses on the longitudinal evaluation of children with chronic liver disease with noninvasive imaging tools, which play an important role in detecting cirrhosis, defining underlying causes, grading fibrosis and monitoring patients during follow-up. Ultrasound is the primary imaging modality and it is used in a multiparametric fashion. Magnetic resonance imaging and computed tomography are usually applied second line for refined tissue characterization, clarification of nodular lesions and full delineation of abdominal vessels, including portosystemic communications.

Keywords: Children; Cirrhosis; Computed tomography; Elastography; Imaging; Liver; Magnetic resonance imaging; Ultrasound.

Fig. 1

A 9-year-old girl with nephronophthisis (NPHP3 gene mutation) and congenital hepatic fibrosis. Massive splenomegaly can be noted on the coronal T2-TSE (a). The liver has diffuse textural changes depicted in all standard axial sequences (b, T2-W; c, T1-W; d, diffusion weighted imaging, b800; e, contrast-enhanced T1-W). Hypertrophy of segment 2/3, hypotrophy of the right lobe, enlargement of the pericholecystic space (asterisk) and capsular retraction (arrowheads) can be seen. Recanalization of the umbilical vein is noted on standard magnetic resonance imaging (MRI) sequences (arrow), but more clearly depicted on transverse color Doppler US (f). On US elastography (g, linear probe in longitudinal plane, confidence map on the right, elastogram on the left) spleen stiffness was substantially increased (median 2-D shear wave elastography values, 70 kPa) suggestive of congestion by portal hypertension. Grade 3 esophageal varices were noted on endoscopy (not shown). Four-dimensional flow MRI has been used to fully visualize the portosystemic communications (h, coronal reconstruction; ivc inferior vena cava, pv portal vein, smv superior mesenteric vein, sv splenic vein, uv umbilical vein)

Fig. 2

A 2-year-old girl with biliary atresia after a Kasai operation. On axial T2-W magnetic resonance imaging, segmental hypertrophy of liver segment 1 with the formation of a regenerative nodule (asterisk), as well as enlargement of liver segment 2/3 with kissing sign of liver and spleen (arrow), can be noted

Fig. 3

Longitudinal left subcostal ultrasound for the evaluation of liver texture using a linear probe. Regular liver texture in a 5-year-old healthy boy shows a homogenous distribution with relatively low differences in echogenicity between neighboring pixels (a). The liver contour is smooth. By comparison, the irregular appearance of the liver texture is shown in a 3-month old boy with liver cirrhosis due to biliary atresia (b). Note the wider distribution of grey-scale values and the increasing beam attenuation with image depth. The liver contour is nodular (arrowheads)

Fig. 4

Longitudinal left subcostal ultrasound of the left liver lobe in a 10-week-old girl with biliary atresia, who developed progressive fibrosis despite a Kasai procedure. The appearance of textural changes of the left liver lobe depends on the choice of linear transducer and the selected frequencies. Textural changes are more distinct with lower frequency transducers (a, 9 Mhz; b, 14 Mhz). Fibrotic bands are visible on the highest-resolution images (c, 24 Mhz), but beam attenuation notably decreases penetration and visualization of deeper structures

Fig. 5

Ultrasound in a 10-day-old boy with atypical Shwachman-Diamond-like syndrome complicated by liver cirrhosis. Corresponding axial images at the level of the liver veins in color Doppler (a) and b-flow (b) show a large, persistent ductus venosus (arrow) functioning as an intrahepatic portosystemic shunt. Umbilical recess (asterisk)

Fig. 6

A 13-year-old boy with autoimmune hepatitis and liver cirrhosis. Textural changes can be noted on axial magnetic resonance images of the liver on T2 turbo spin echo (a), T1 mDixon (b) and the diffusion-weighted b800 image (c). Areas of diffusion restriction in the right lobe represent fibrosis. Textural changes can also be noted on gray-scale right intercostal ultrasound (d). Right intercostal US with a convex probe, 2-D shear wave elastography (2-D SWE) at the time of diagnosis (e) and 12 months after start of immunosuppressant treatment under complete biochemical remission (f, confidence map, left and elastogram, right). The reduction in liver stiffness (delta of median 2-D SWE values, -6.5 kPa) is explained by elimination of the inflammatory component. The persisting elevation of liver stiffness at 12 months indicates a remaining fibrotic component

Fig. 7

A 2-year-old boy with hereditary tyrosinemia type 1 and hepatocellular carcinoma. Right intercostal ultrasound (a) shows an echogenic lesion within an irregular hepatic texture. The lesion is isointense on axial T2 turbo spin echo (b), hyperintense on axial T1 (c) and only diffusely demarcated on axial diffusion-weighted imaging (b800 image, d). After contrast administration, the lesion shows strong enhancement on the axial T1 in the arterial (e) and portal-venous phase (f) and slight washout in the parenchymal phase (g)