Successful Management of Neonatal Kaposiform Haemangioendothelioma and Kasabach-Merritt Phenomenon Using Sirolimus and Surgical Excision
| Author | Lucinda Elizabeth Hill Adams | en |
| Author | Kathleen Wang | en |
| Author | Emma Ryan | en |
| Orcid | Lucinda Elizabeth Hill Adams [0000-0003-2748-3383] | en |
| Issued Date | 2023-09-30 | en |
| Abstract | Kaposiform haemangioendothelioma (KHE) is a rare vascular tumor, occurring predominantly in infancy, with an incidence of 0.091 per 100,000 children. Typically presenting as a solitary large cutaneous lesion, with no distant metastases. Children with KHE are at high-risk of developing Kasabach-Merritt Phenomenon (KMP), a consumptive coagulopathy. KMP is defined as profound thrombocytopenia, hypofibrinogenemia, and elevated D-dimer. KMP has a mortality of 10 - 30%. Due to its rarity, there is little robust evidence for KHE management. Rapamycin (mTOR) inhibitors have been shown to be a safe and effective treatment. This report is regarding a newborn term female who presented at birth with an abdominal cutaneous vascular malformation, subsequently developing life-threatening thrombocytopenia and coagulopathy. She was diagnosed with KHE complicated by KMP, with an excellent response to sirolimus treatment, with subsequent lesion resection. | en |
| DOI | https://doi.org/10.5812/jssc-139177 | en |
| Keyword | Pediatric Dermatology | en |
| Keyword | Vascular Tumors | en |
| Keyword | Kaposiform Haemangioendothelioma | en |
| Keyword | Kasabach-Merritt Phenomenon | en |
| Keyword | Sirolimus | en |
| Publisher | Brieflands | en |
| Title | Successful Management of Neonatal Kaposiform Haemangioendothelioma and Kasabach-Merritt Phenomenon Using Sirolimus and Surgical Excision | en |
| Type | Case Report | en |