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CC BY 4.0 · European J Pediatr Surg Rep. 2025; 13(01): e55-e58
DOI: 10.1055/a-2699-8104
Case Report

Kaposiform Hemangioendothelioma with Kasabach–Merritt Phenomenon in a Neonate: A Case Report

Authors

  • Yuyang Zheng   

    1   The First Clinical Medical College of Gannan Medical University, Ganzhou, Jiangxi, People's Republic of China

  • Rongjie Wu  

    1   The First Clinical Medical College of Gannan Medical University, Ganzhou, Jiangxi, People's Republic of China

  • Qilin Chen

    1   The First Clinical Medical College of Gannan Medical University, Ganzhou, Jiangxi, People's Republic of China

  • Shiyu Xiong

    1   The First Clinical Medical College of Gannan Medical University, Ganzhou, Jiangxi, People's Republic of China

  • Jialu Yun

    1   The First Clinical Medical College of Gannan Medical University, Ganzhou, Jiangxi, People's Republic of China

  • Wei Peng

    2   Department of Pediatric Surgery, First Affiliated Hospital of Gannan Medical University, Ganzhou, Jiangxi, People's Republic of China

Funding This work was supported by the Science and Technology Project of Jiangxi Provincial Health Commission (grant no. 202310845).
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Corrected by:

Erratum: Corrections to Two Articles in European Journal of Pediatric Surgery Reports, Volume 13, Issue 1European J Pediatr Surg Rep. 2025; 13(01): e59-e59
DOI: 10.1055/a-2706-9632

Abstract

Background

Kaposiform hemangioendothelioma (KHE) is a rare, aggressive vascular tumor frequently complicated by Kasabach–Merritt phenomenon (KMP), a life-threatening consumptive coagulopathy. Neonatal KHE-KMP management requires urgent intervention but is complicated by immunosuppression risks, especially in Bacillus Calmette–Guérin (BCG)-vaccinated infants.

Methods

A full-term male neonate with prenatal right upper limb thickening presented postnatally with a violaceous, firm mass. Laboratory findings confirmed KMP. Due to recent BCG vaccination, sirolimus was initially withheld. First-line therapies failed, prompting sirolimus initiation on day 3, supplemented by fibrinogen transfusions.

Results

Platelets normalized by day 13 (283 × 109/L) with marked tumor regression. Transient fever/diarrhea resolved with supportive care. At discharge (day 27), platelets stabilized (183 × 109/L). Three-month follow-up showed sustained platelet recovery (268–532 × 109/L), near-complete tumor resolution, and age-appropriate development. Prophylactic trimethoprim-sulfamethoxazole prevented infections.

Conclusion

Sirolimus is a critical salvage therapy for refractory neonatal KHE-KMP, even in BCG-vaccinated infants. Timely initiation reverses life-threatening coagulopathy and achieves favorable outcomes, necessitating multidisciplinary monitoring to balance immunosuppression risks.

Keywords

kaposiform hemangioendothelioma - Kasabach–Merritt phenomenon - sirolimus - neonate - thrombocytopenia

* These two authors contributed equally to this work and share the first authorship.


Ethical Compliance

This work was approved by the Institutional Ethics Committee of Gannan Medical University, and written informed consent was obtained.




Publication History

Received: 30 June 2025

Accepted: 19 August 2025

Article published online:
19 September 2025

© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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