The Natural History of Congenital Hepatic Hemangiomas.

OBJECTIVE

To characterize the clinical presentation, radiologic and histologic findings, and management options for congenital hepatic hemangioma (HH) with a focus on describing its natural history and rate of involution.

STUDY

DESIGN

Retrospective review of patients in our HH registry with a congenital HH born between 2004 and 2022 was performed. HH volumes were calculated using the formula for an ellipsoid. Multiple analyses of HH volume change were performed using nonlinear regression with generalized estimating equations, Kaplan-Meier curves, and logrank test.

RESULTS

We identified 96 infants with a congenital HH. Of these, 32% were diagnosed prenatally, 23% developed heart failure, and 23% developed respiratory failure. On average, patients demonstrated 43% residual HH volume at 12 months (95% confidence interval: 27%-60%) and 16% residual volume at 24 months (95% confidence interval: 0%-35%). No difference in time to 50% HH volume reduction between patients with and without medical therapy was observed (P = .894).

CONCLUSION

Congenital HH represents the hepatic counterpart of the cutaneous congenital hemangioma and is clinically, histologically, and radiographically distinct from infantile HH. Most lesions reach >80% involution by 24 months of age with no differences in time to involution observed with medical therapy. A clear understanding of the clinical behavior and evolution of congenital HH is critical to early recognition of associated morbidity and prompt review of an incorrect diagnosis.