Natural History and Management of Congenital Hepatic Hemangiomas

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CA Ostertag-Hill et al. J Pediatr 2025;281:114523. The Natural History of Congenital Hepatic Hemangiomas

Background: “Hemangiomas remain the most common tumor of the liver in newborns and infants…A subtype classification system for hepatic hemangioma (HH) was first proposed based on our Liver Hemangioma Registry in 2007, delineating focal, multifocal, and diffuse HH…Focal hepatic lesions correspond to congenital hemangiomas whereas multifocal and diffuse lesions represent infantile hemangiomas.”

Methods: This was a retrospective review of 96 infants over an 18 year period (2004-2022). Patients with infantile HH were excluded.

Key Findings:

  • 32% were diagnosed prenatally, 23% developed heart failure, and 23% developed respiratory failure
  • There was a balanced sex distribution (50% for each gender)
  • Common clinical features included transient anemia (n = 23/48, 48%) and thrombocytopenia (n = 30/53, 57%).
  • On average, patients demonstrated 43% residual HH volume at 12 months and 16% residual volume at 24 months
  • No difference in time to 50% HH volume reduction between patients with and without medical therapy was observed
  • Larger hemangioma volumes were associated with an increased risk of anemia (P = .005) and thrombocytopenia (P < .001)
  • There was not a significant association between HH volume and congestive heart failure (CHF) or cardiomegaly. For example, the HH volume was 824 mL vs 579 mL (P=0.689) in those with and without CHF, respectively

Discussion Points:

  • “Congenital HH is present at birth and typically does not undergo postnatal growth”
  • “Congenital HH occurs equally in males and females and is immunonegative for GLUT-1. This stands in contrast to infantile hepatic and cutaneous hemangioma, which exhibits an early proliferative phase followed by gradual involution”
  • “HH size at diagnosis was associated with respiratory failure but not with the development of cardiomegaly or CHF, suggesting intralesional shunting may not be related to absolute tumor volume. Hence, all HH regardless of size should be assessed for shunting by doppler US”
  • “We advocate consideration of cross-sectional imaging and/or biopsy if patients with presumed congenital HH do not follow the expected clinical behavior of early signs of involution”
  • “Although corticosteroids and propranolol have proven benefit for infantile HH, we
    demonstrate that there was no significant difference in the rate of congenital HH involution between patients who had received medical therapy and those that did not”
  • “Medical therapy does have a role in the medical management of high-output heart failure occurring secondary to shunting. If refractory to pharmacologic therapy, these patients
    should undergo embolization of symptomatic intrahepatic shunts”
The practice algorithm for evaluation and management of congenital HH suggested by the authors. *Obtain baseline labs and repeat as needed. Repeat CBC if lesion increases in size during
monitoring period and there is concern for intralesional bleeding. Repeat AFP as necessary to rule out hepatoblastoma. TFTs indicated if unclear whether congenital HH or infantile HH
.

My take: This is a very useful study. It is important to distinguish congenital HH from infantile HH. Even in those with congenital HH, “a subset of patients develop life-threatening complications including respiratory failure and CHF that warrant directed medical management.”

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