{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,17]],"date-time":"2026-05-17T09:03:10Z","timestamp":1779008590867,"version":"3.51.4"},"reference-count":14,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2025,10,23]],"date-time":"2025-10-23T00:00:00Z","timestamp":1761177600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Background\/Objectives: Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor of infancy, often complicated by Kasabach\u2013Merritt phenomenon (KMP), a consumptive coagulopathy characterized by severe thrombocytopenia and hypofibrinogenemia. Airway involvement at birth is exceptionally rare and can be life-threatening. This study reports the clinical presentation and treatment course of a full-term male neonate with severe airway obstruction caused by KHE with KMP. Case Presentation: The patient had unremarkable prenatal imaging but presented at birth with severe respiratory distress requiring emergent intubation. Physical examination revealed firm violaceous swelling over the right cervicothoracic region. Laboratory tests showed profound thrombocytopenia (22,000\/\u03bcL), hypofibrinogenemia (75 mg\/dL), and coagulopathy. Imaging findings were consistent with KHE complicated by KMP. Due to bleeding risk, the biopsy was not performed. Initial treatment included platelet and plasma transfusions, intravenous immunoglobulin (IVIG), corticosteroids, and antithrombin III replacement. Vincristine was discontinued owing to gastrointestinal toxicity. Sirolimus therapy was initiated on day 14. Following sirolimus initiation, rapid platelet recovery was observed. At three months, marked tumor regression was documented. After mild recurrence, sirolimus was reintroduced, and the patient remained stable at 16-month follow-up. Conclusions: This case underscores the critical importance of prompt airway stabilization, early recognition of consumptive coagulopathy, and sirolimus-based therapy in managing neonatal KHE with airway involvement.<\/jats:p>","DOI":"10.3390\/children12111429","type":"journal-article","created":{"date-parts":[[2025,10,23]],"date-time":"2025-10-23T02:03:48Z","timestamp":1761185028000},"page":"1429","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Neonatal Kaposiform Hemangioendothelioma with Kasabach\u2013Merritt Phenomenon Presenting as Severe Airway Obstruction at Birth: A Case Report"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8328-1087","authenticated-orcid":false,"given":"Soyoung","family":"Shin","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Keimyung University School of Medicine, Daegu 42601, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5047-3493","authenticated-orcid":false,"given":"Ye Jee","family":"Shim","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, School of Medicine, Kyungpook National University, Daegu 41944, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,10,23]]},"reference":[{"key":"ref_1","first-page":"457","article-title":"Kaposiform haemangioendothelioma: Clinical features, complications and risk factors for Kasabach-Merritt phenomenon","volume":"179","author":"Ji","year":"2018","journal-title":"Br. J. Dermatol."},{"key":"ref_2","first-page":"593","article-title":"Kasabach\u2013Merritt phenomenon: Clinical, radiologic, and histologic features and response to treatment of 30 cases","volume":"50","author":"Enjolras","year":"2004","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1016\/j.jpeds.2012.06.044","article-title":"Kaposiform hemangioendothelioma: Atypical features and risks of Kasabach\u2013Merritt phenomenon in 107 referrals","volume":"162","author":"Croteau","year":"2013","journal-title":"J. Pediatr."},{"key":"ref_4","first-page":"349","article-title":"Clinical improvement of patients with Kaposiform hemangioendothelioma and Kasabach\u2013Merritt phenomenon treated with sirolimus","volume":"63","author":"Ozeki","year":"2016","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1016\/j.jpeds.2013.03.080","article-title":"Consensus-derived practice standards plan for complicated Kaposiform hemangioendothelioma","volume":"163","author":"Drolet","year":"2013","journal-title":"J. Pediatr."},{"key":"ref_6","first-page":"467","article-title":"Kaposiform hemangioendothelioma: A review with emphasis on Kasabach\u2013Merritt phenomenon","volume":"32","author":"Croteau","year":"2010","journal-title":"J. Pediatr. Hematol. Oncol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1486","DOI":"10.1016\/j.jpedsurg.2006.04.013","article-title":"Kaposiform hemangioendothelioma of the mediastinum in a 7-month-old boy: A case report","volume":"41","author":"Iwami","year":"2006","journal-title":"J. Pediatr. Surg."},{"key":"ref_8","first-page":"539","article-title":"Treatment of childhood kaposiform hemangioendothelioma with vincristine","volume":"22","author":"Blatt","year":"2000","journal-title":"J. Pediatr. Hematol. Oncol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1002\/pbc.25241","article-title":"Successful treatment of Kaposiform hemangioendothelioma with everolimus","volume":"62","author":"Uno","year":"2015","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1055\/s-0036-1593383","article-title":"Sirolimus in the treatment of vascular anomalies","volume":"27","author":"Dore","year":"2017","journal-title":"Eur. J. Pediatr. Surg."},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Vrinceanu, D., Dumitru, M., Marinescu, A., Dorobat, B., Palade, O.D., Manole, F., Muresian, H., Popa-Cherecheanu, M., and Ciornei, C.M. (2024). New insights into cervicofacial vascular anomalies. J. Clin. Med., 13.","DOI":"10.3390\/jcm13123515"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1018","DOI":"10.1002\/pbc.23124","article-title":"Sirolimus for the treatment of complicated vascular anomalies in children","volume":"57","author":"Hammill","year":"2011","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"956","DOI":"10.1111\/1346-8138.15076","article-title":"Sirolimus therapy for kaposiform hemangioendothelioma with long-term follow-up","volume":"46","author":"Wang","year":"2019","journal-title":"J. Dermatol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"600","DOI":"10.1002\/ijc.34509","article-title":"Long-term outcomes of sirolimus treatment for Kaposiform hemangioendothelioma: Continuing successes and ongoing challenges","volume":"153","author":"Zhou","year":"2023","journal-title":"Int. J. Cancer"}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/12\/11\/1429\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T08:08:19Z","timestamp":1761552499000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/12\/11\/1429"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,23]]},"references-count":14,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2025,11]]}},"alternative-id":["children12111429"],"URL":"https:\/\/doi.org\/10.3390\/children12111429","relation":{},"ISSN":["2227-9067"],"issn-type":[{"value":"2227-9067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10,23]]}}}