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Mortality predictive factors in congenital hepatic hemangioma: a case–control study

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Abstract

Congenital hepatic hemangiomas (CHHs) are benign vascular tumors whose clinical, histological, and genetic correlation has recently been described in patients with long-term survival, although no mortality risk factors have been identified to date. The aim of this study is to analyze predictors of mortality in patients with CHH. A retrospective single-center case–control study of consecutive CHH patients diagnosed in our institution between 1991 and 2021 was performed, who were classified into two groups according to their survival. Demographic, gestational, imaging, and laboratory data at diagnosis were collected and compared between both groups. A total of 29 patients were included (12 males; 17 females) of whom 5 died as a result of CHH evolution due to cardiac failure and coagulopathy, with a median age of 11 days until death. No differences in demographic or gestational data were reported. There were neither differences when comparing imaging tests, nor in location, number of affected liver segments, or CHH estimated volume. Upon laboratory data at diagnosis, deceased patients had a significant elevation of median liver enzymes [glutamic-oxaloacetic transaminase (359 u/L vs. 45 u/L; p < 0.01) and glutamic-pyruvic transaminase (313 u/L vs. 20 u/L; p < 0. 01)], as well as a decreased median platelet count (85,250/µL vs. 337,000/µL; p < 0.01), prothrombin activity (54% vs. 93%; p < 0.01), and fibrinogen (131 mg/dL vs. 284 mg/dL; p < 0.01), with no differences in blood count or biochemistry data.

Conclusions: CHH clinical behavior can be innocuous or life-threatening. Thrombocytopenia, coagulation disorders, and increased liver enzymes at diagnosis seem to be the main predictors of mortality.

What is Known:

• Congenital Hepatic Hemangiomas (CHHs) are benign vascular tumors whose clinical behavior can be innocuous or life-threatening.

What is New:

• Thrombocytopenia, coagulation disorders and increased liver enzymes at diagnosis seem to be the main predictors of mortality in these patients.

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Data Availability

Data collected in this study would be available upon reasonable request to the authors.

Abbreviations

CHH:

Congenital hepatic hemangiomas

ISSVA:

International Society for the Study of Vascular Anomalies

IHH:

Infantile hepatic hemangiomas

aPPT:

Activated partial thromboplastin time

GOT:

Glutamic-oxaloacetic transaminase

GPT:

Glutamic-pyruvic transaminase

GGT:

Gamma-glutamyl transferase

LDH:

Lactate dehydrogenase

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Authors and Affiliations

  1. Department of Pediatric Surgery, La Paz Children’s University Hospital, Paseo de La Castellana 261, Madrid, Spain

    Carlos Delgado-Miguel, Paloma Triana, Miriam Miguel-Ferrero, Mercedes Díaz, Juan Carlos López-Gutiérrez & Francisco Hernández Oliveros

  2. Institute for Health Research IdiPAZ, La Paz University Hospital, Madrid, Spain

    Carlos Delgado-Miguel & Francisco Hernández Oliveros

  3. Department of Pediatric Hepatology, La Paz Children’s University Hospital, Madrid, Spain

    Loreto Hierro & Paloma Jara

  4. Pediatric Liver Transplantation Unit, La Paz University Hospital, Madrid, Spain

    Francisco Hernández Oliveros

Authors
  1. Carlos Delgado-Miguel
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  2. Paloma Triana
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  3. Miriam Miguel-Ferrero
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  4. Mercedes Díaz
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  5. Loreto Hierro
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  6. Paloma Jara
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  7. Juan Carlos López-Gutiérrez
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  8. Francisco Hernández Oliveros
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Contributions

Conception and design of the work: C. Delgado-Miguel, M. Miguel-Ferrero, and F. Hernández Oliveros. Data collection; C. Delgado-Miguel and P. Triana. Data analysis and interpretation: C. Delgado-Miguel, M. Díaz and JC. López-Gutiérrez. Drafting the article: C. Delgado-Miguel, P. Triana, and M. Miguel-Ferrero. Critical revision of the article: L. Hierro, P. Jara, JC. López-Gutiérrez, and F. Hernández Oliveros. Final approval: all authors.

Corresponding author

Correspondence to Carlos Delgado-Miguel.

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Ethics approval

The study protocol was conformed to the guidelines of the 1975 Declaration of Helsinki and was approved by our Institutional Review Board. Written informed consent was not required due to the retrospective nature of this study and the anonymous collection of analytical data, in line with institutional guidelines.

Competing interest

The authors declare no competing interests.

Additional information

Communicated by Daniele De Luca

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Delgado-Miguel, C., Triana, P., Miguel-Ferrero, M. et al. Mortality predictive factors in congenital hepatic hemangioma: a case–control study. Eur J Pediatr 182, 1657–1663 (2023). https://doi.org/10.1007/s00431-023-04849-w

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  • DOI: https://doi.org/10.1007/s00431-023-04849-w

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