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Multidisciplinary management of a neonate with kaposiform hemangioendothelioma with extensive cranial fossa destruction
Kaposiform hemangioendothelioma is a rare, benign, locally destructive vascular tumor. Kasabach–Merritt phenomenon, a consumptive coagulopathy, is a life-threatening complication associated with kaposiform hemangioendothelioma. We describe a case of kaposiform hemangioendothelioma complicated by Kas...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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SAGE Publications
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9761797/ https://www.ncbi.nlm.nih.gov/pubmed/36545011 http://dx.doi.org/10.1177/2050313X221142685 |
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author | Khoei, Amelia Aynaz Arias-Shah, AnnaMarie Kralik, Stephen Mahajan, Priya Iacobas, Ionela Fernandes, Caraciolo Joseph |
author_facet | Khoei, Amelia Aynaz Arias-Shah, AnnaMarie Kralik, Stephen Mahajan, Priya Iacobas, Ionela Fernandes, Caraciolo Joseph |
author_sort | Khoei, Amelia Aynaz |
collection | PubMed |
description | Kaposiform hemangioendothelioma is a rare, benign, locally destructive vascular tumor. Kasabach–Merritt phenomenon, a consumptive coagulopathy, is a life-threatening complication associated with kaposiform hemangioendothelioma. We describe a case of kaposiform hemangioendothelioma complicated by Kasabach–Merritt phenomenon in a neonate born with a large facial mass with deep extension toward the cranium and airway. The mass was not identified prenatally. The patient was a 37-week gestation age female neonate born via spontaneous vaginal delivery and noted to have a large left-sided facial mass that was not noted on the most recent prenatal ultrasound at 22 weeks gestation age. At birth, the patient was in respiratory distress and required continuous positive airway pressure support. Imaging revealed a large highly vascularized soft tissue mass adjacent to the airway with intracranial extension and bony destruction. Fine needle aspiration confirmed kaposiform hemangioendothelioma. On day of life 6, the patient was noted to have thrombocytopenia, elevated d-dimer, anemia, and hypofibrinogenemia, consistent with Kasabach–Merritt phenomenon, which resolved at day of life 12. Given the location and extent of the mass, medical therapy with single agent oral sirolimus was chosen over surgery. At 13-month follow-up, the infant is well on sirolimus therapy, and the mass has decreased in size, both clinically and on imaging. This case highlights the importance of prompt diagnosis and management of kaposiform hemangioendothelioma with extensive craniofacial and bony involvement with Kasabach–Merritt phenomenon with single oral therapy of sirolimus. Fibrinogen concentrate may be considered in the Kasabach–Merritt phenomenon refractory to cryoprecipitate. |
format | Online Article Text |
id | pubmed-9761797 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | SAGE Publications |
record_format | MEDLINE/PubMed |
spelling | pubmed-97617972022-12-20 Multidisciplinary management of a neonate with kaposiform hemangioendothelioma with extensive cranial fossa destruction Khoei, Amelia Aynaz Arias-Shah, AnnaMarie Kralik, Stephen Mahajan, Priya Iacobas, Ionela Fernandes, Caraciolo Joseph SAGE Open Med Case Rep Case Report Kaposiform hemangioendothelioma is a rare, benign, locally destructive vascular tumor. Kasabach–Merritt phenomenon, a consumptive coagulopathy, is a life-threatening complication associated with kaposiform hemangioendothelioma. We describe a case of kaposiform hemangioendothelioma complicated by Kasabach–Merritt phenomenon in a neonate born with a large facial mass with deep extension toward the cranium and airway. The mass was not identified prenatally. The patient was a 37-week gestation age female neonate born via spontaneous vaginal delivery and noted to have a large left-sided facial mass that was not noted on the most recent prenatal ultrasound at 22 weeks gestation age. At birth, the patient was in respiratory distress and required continuous positive airway pressure support. Imaging revealed a large highly vascularized soft tissue mass adjacent to the airway with intracranial extension and bony destruction. Fine needle aspiration confirmed kaposiform hemangioendothelioma. On day of life 6, the patient was noted to have thrombocytopenia, elevated d-dimer, anemia, and hypofibrinogenemia, consistent with Kasabach–Merritt phenomenon, which resolved at day of life 12. Given the location and extent of the mass, medical therapy with single agent oral sirolimus was chosen over surgery. At 13-month follow-up, the infant is well on sirolimus therapy, and the mass has decreased in size, both clinically and on imaging. This case highlights the importance of prompt diagnosis and management of kaposiform hemangioendothelioma with extensive craniofacial and bony involvement with Kasabach–Merritt phenomenon with single oral therapy of sirolimus. Fibrinogen concentrate may be considered in the Kasabach–Merritt phenomenon refractory to cryoprecipitate. SAGE Publications 2022-12-17 /pmc/articles/PMC9761797/ /pubmed/36545011 http://dx.doi.org/10.1177/2050313X221142685 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by-nc/4.0/This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access page (https://us.sagepub.com/en-us/nam/open-access-at-sage). |
spellingShingle | Case Report Khoei, Amelia Aynaz Arias-Shah, AnnaMarie Kralik, Stephen Mahajan, Priya Iacobas, Ionela Fernandes, Caraciolo Joseph Multidisciplinary management of a neonate with kaposiform hemangioendothelioma with extensive cranial fossa destruction |
title | Multidisciplinary management of a neonate with kaposiform
hemangioendothelioma with extensive cranial fossa destruction |
title_full | Multidisciplinary management of a neonate with kaposiform
hemangioendothelioma with extensive cranial fossa destruction |
title_fullStr | Multidisciplinary management of a neonate with kaposiform
hemangioendothelioma with extensive cranial fossa destruction |
title_full_unstemmed | Multidisciplinary management of a neonate with kaposiform
hemangioendothelioma with extensive cranial fossa destruction |
title_short | Multidisciplinary management of a neonate with kaposiform
hemangioendothelioma with extensive cranial fossa destruction |
title_sort | multidisciplinary management of a neonate with kaposiform
hemangioendothelioma with extensive cranial fossa destruction |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9761797/ https://www.ncbi.nlm.nih.gov/pubmed/36545011 http://dx.doi.org/10.1177/2050313X221142685 |
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