Two-dimensional (2D) morphologic measurements can quantify the severity of liver disease in children with autosomal recessive polycystic kidney disease (ARPKD)

Adarsh Ghosh; Suraj D Serai; Shyam Sunder B Venkatakrishna; Mohini Dutt; Erum A Hartung

doi:10.1007/s00261-021-03189-3

Two-dimensional (2D) morphologic measurements can quantify the severity of liver disease in children with autosomal recessive polycystic kidney disease (ARPKD)

Abdom Radiol (NY). 2021 Oct;46(10):4709-4719. doi: 10.1007/s00261-021-03189-3. Epub 2021 Jun 26.

Authors

Adarsh Ghosh^{1

2}, Suraj D Serai^{3

4}, Shyam Sunder B Venkatakrishna³, Mohini Dutt⁵, Erum A Hartung^{5

6}

Affiliations

¹ Department of Radiology, Children's Hospital of Philadelphia, 3401 Civic Center Boulevard, Philadelphia, PA, 19104, USA. ghosha2@chop.edu.
² Department of Radiology, Clinical Research, Children's Hospital of Philadelphia, Roberts Center for Pediatric Research, Office 3122, 3rd Floor, 2716 South Street, Philadelphia, PA, 19146, USA. ghosha2@chop.edu.
³ Department of Radiology, Children's Hospital of Philadelphia, 3401 Civic Center Boulevard, Philadelphia, PA, 19104, USA.
⁴ Department of Radiology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.
⁵ Division of Nephrology, Children's Hospital of Philadelphia, 3401 Civic Center Boulevard, Philadelphia, PA, 19104, USA.
⁶ Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.

Abstract

Purpose: To evaluate the correlation of 2D shape-based features with magnetic resonance elastography (MRE)-derived liver stiffness and portal hypertension (pHTN) in children with ARPKD-associated congenital hepatic fibrosis.

Methods: In a prospective IRB-approved study, 14 children with ARPKD (mean age ± SD = 13.8 ± 5.8 years) and 14 healthy controls (mean age ± SD = 13.7 ± 3.9 years) underwent liver MRE. A 2D region of interest (ROI) outlining the left liver lobe at the level of the abdominal aorta was drawn on sagittal T2-weighted images. Eight shape features (perimeter, major axis length, maximum diameter, perimeter to surface ratio (PSR), elongation, sphericity, minor axis length, and mesh surface) describing the 2D-ROI were calculated. Spearman's correlation was calculated between shape features and MRE-derived liver stiffness (kPa) (n = 28). Shape features were compared between participants with ARPKD with pHTN (splenomegaly and thrombocytopenia), (n = 4) and without pHTN (n = 8) using the Mann Whitney U test. Receiver operating characteristic (ROC) curves were generated to examine the diagnostic accuracy of shape features in identifying cases with liver stiffness > 2.9 kPa.

Results: In ARPKD participants and healthy controls, all eight shape features, except elongation, showed moderate to strong correlation with liver stiffness (kPa); the perimeter surface ratio had the strongest correlation (rho = - 0.75, p < 0.001). In ROC analysis, a cut-off of PSR ≤ 0.057 mm^-1 gave 100% (95% CI: 59.0-100.0) sensitivity and 100% (95% CI: 83.9-100.0) specificity in identifying ARPKD participants with liver stiffness > 2.9 kPa, with an area under the ROC curve (AUC) of 1.0 (95% CI: 0.88-1.00). Individuals with pHTN had a lower median PSR (mean ± SD = 0.05 ± 0.01) than those without (0.07 ± 0.01; p = 0.027) with an AUC of 0.91 (95% CI: 0.60-0.99) in differentiating the participants with and without pHTN.

Conclusion: Shape-based features of the left liver lobe show potential as non-invasive biomarkers of liver fibrosis and portal hypertension in children with ARPKD.

Keywords: ARPKD; Congenital hepatic fibrosis; Elastography; Liver stiffness; Shape.

MeSH terms

Child
Elasticity Imaging Techniques*
Humans
Hypertension, Portal* / diagnostic imaging
Hypertension, Portal* / pathology
Liver / diagnostic imaging
Liver / pathology
Liver Cirrhosis / pathology
Polycystic Kidney, Autosomal Recessive* / diagnostic imaging
Prospective Studies

Grants and funding

K23 DK109203/DK/NIDDK NIH HHS/United States