{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T13:41:33Z","timestamp":1753882893582,"version":"3.41.2"},"reference-count":20,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2022,1,1]],"date-time":"2022-01-01T00:00:00Z","timestamp":1640995200000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["SAGE Open Medical Case Reports"],"published-print":{"date-parts":[[2022,1]]},"abstract":"<jats:p> Kaposiform hemangioendothelioma is a rare, benign, locally destructive vascular tumor. Kasabach\u2013Merritt phenomenon, a consumptive coagulopathy, is a life-threatening complication associated with kaposiform hemangioendothelioma. We describe a case of kaposiform hemangioendothelioma complicated by Kasabach\u2013Merritt 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\u2009weeks 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\u2013Merritt 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\u2013Merritt phenomenon with single oral therapy of sirolimus. Fibrinogen concentrate may be considered in the Kasabach\u2013Merritt phenomenon refractory to cryoprecipitate. <\/jats:p>","DOI":"10.1177\/2050313x221142685","type":"journal-article","created":{"date-parts":[[2022,12,17]],"date-time":"2022-12-17T11:55:19Z","timestamp":1671278119000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":1,"title":["Multidisciplinary management of a neonate with kaposiform hemangioendothelioma with extensive cranial fossa destruction"],"prefix":"10.1177","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9631-9013","authenticated-orcid":false,"given":"Amelia Aynaz","family":"Khoei","sequence":"first","affiliation":[{"name":"School of Medicine, Baylor College of Medicine, Houston, TX, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"AnnaMarie","family":"Arias-Shah","sequence":"additional","affiliation":[{"name":"Division of Neonatology, Department of Pediatrics, Baylor College of Medicine, Texas Children\u2019s Hospital, Houston, TX, USA"},{"name":"Section of Neonatology, Children\u2019s Health of Orange County, Orange, CA, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephen","family":"Kralik","sequence":"additional","affiliation":[{"name":"Department of Radiology, Baylor College of Medicine, Houston, TX, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Priya","family":"Mahajan","sequence":"additional","affiliation":[{"name":"Division of Neonatology, Department of Pediatrics, Baylor College of Medicine, Texas Children\u2019s Hospital, Houston, TX, USA"},{"name":"Section of Hematology-Oncology, Baylor College of Medicine, Houston, TX, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ionela","family":"Iacobas","sequence":"additional","affiliation":[{"name":"Division of Neonatology, Department of Pediatrics, Baylor College of Medicine, Texas Children\u2019s Hospital, Houston, TX, USA"},{"name":"Section of Hematology-Oncology, Baylor College of Medicine, Houston, TX, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5744-3409","authenticated-orcid":false,"given":"Caraciolo Joseph","family":"Fernandes","sequence":"additional","affiliation":[{"name":"Division of Neonatology, Department of Pediatrics, Baylor College of Medicine, Texas Children\u2019s Hospital, Houston, TX, 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