Presentation and Evolution of Congenital Hepatic Hemangiomas

Source: Ostertag-Hill CA, Fevurly RD, Kulungowski AM, et al. The natural history of congenital hepatic hemangiomas. J Pediatr. 2025;281:114523; doi: 10.1016/j.jpeds.2025.114523.Investigators from Boston Children’s Hospital (BCH), conducted a retrospective study to describe the clinical presentation in children with congenital hepatic hemangioma (HH), assess rate of involution over time, and evaluate the impact of treatment on involution. Study patients were identified by reviewing the Vascular Anomalies Center database, which includes demographic, clinical, and radiographic data on patients with HH diagnosed at BCH or from other institutions through the use of an online registry. Standardized criteria, largely based on radiographic characteristics, were used to define congenital HH. Patients with infantile HH were excluded. The size of the congenital HH was estimated by reviewing imaging studies. Rank sum tests were used to evaluate the association between HH size at diagnosis and development of anemia (hemoglobin or hematocrit >2 standard deviations below mean), thrombocytopenia (platelets <150 x 109/L), congestive heart failure, and respiratory failure. To assess involution over time, data on infants with congenital HH who had sequential imaging studies available for review were included. Change in volume was assessed using regression analyses. Kaplan-Meier curve analysis was used to estimate rates of ≥50% involution at 12 and 24 months and compare involution rates in infants who received treatment for congenital HH or no treatment.A total of 96 infants with congenital HH were included in the analysis, with 37 (39%) diagnosed at BCH. Study participants had a median gestational age of 37 weeks and a median age at diagnosis of 1.0 day; 33 were diagnosed prenatally. Among those with available data, 23/48 (48%) patients had transient anemia and 30/53 (57%) had thrombocytopenia. The size of the HH at diagnosis was associated with an increased risk for anemia (P = 0.005), thrombocytopenia (P <0.001), and respiratory failure (P <0.001), but not congestive heart failure (P = 0.689). Sufficient radiographic information was available to assess change in size of the HH in 47 patients. At 12 and 24 months of age, the residual hemangioma volume was 43% and 16%, respectively. The rate of ≥50% involution was 79% (95% confidence interval [CI], 66, 89) at 12 months and 90% (95% CI, 78, 97) at 24 months. There were 39 (46%) infants who received treatment for congenital HH. There was no significant difference in the rate of involution between patients receiving treatment or no treatment (P = 0.84). The mortality rate of participants with available data was 4% (3/72), with 1 death related to the congenital HH.The authors conclude that in infants with congenital HH, mean involution over 24 months was >80%.Dr Doolittle has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.Congenital HH, although rare, is the most common hepatic tumor in infancy.1 Management of HH varies widely across institutions due to wide variation of presentation and a knowledge gap in their natural history.2 The current authors present the largest case series to date on this complex vascular malformation to close this gap. The investigators excluded patients with infantile HH, which develops after birth and has a histology and natural history different from congenital HH, which is present at birth and often diagnosed in utero.3The main finding of the current study is reassuring to patients, families, and pediatricians alike. By 24 months of age, 90% of HH have spontaneously involuted by more than 50%, irrespective of treatment. Although this was a retrospective cohort and not a randomized controlled trial, it is important to note that those who received corticosteroids or propranolol, the most common treatments, did not have involution rates statistically different from those who received no treatment. The authors suggest that serial monitoring is the most prudent course of action; treatment of HH is indicated only with presenting symptoms, such as congestive heart failure or other hemodynamic evidence of significant shunt. In these cases, medical management or even embolization or resection may be indicated.2Although the current cohort is the largest to date, there were notable gaps in data, which may cast some doubt upon the conclusions. For example, among the 96 participants, investigators had sufficient radiographic evidence in only 47 (49%) and platelet data on only 46 (48%). This may be due to the asymptomatic nature of most patients with HHs; sicker patients likely would have been referred to tertiary care facilities, and data would have been available. Despite this conjecture, this study clarifies the natural history of HHs and provides reassurance that, most of the time, congenital HHs will involute without need for intervention.Most congenital HHs will spontaneously involute by 24 months of age. Serial monitoring, rather than invasive treatment, in otherwise asymptomatic infants is suggested.