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Skeletal Radiology
Published in: Skeletal Radiology 4/2023

10-08-2022 | Kasabach-Merritt Syndrome | Case Report

Kaposiform haemangioendothelioma of the spine associated with fixed hyperlordotic deformity and Kasabach–Merritt Syndrome: a case report and literature review

Authors: Leanne H. Q. Chin, Kevin K. F. Fung, Joyce P. K. Chan, Amanda N. C. Kan, M. K. Yuen

Published in: Skeletal Radiology | Issue 4/2023

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Abstract

Kaposiform haemangioendothelioma (KHE) is a rare childhood disease classified by the International Society for the Study of Vascular Anomalies (ISSVA) as a locally aggressive vascular tumor. It has been reported to affect any site, with a predilection for the extremities and trunk. Although it typically manifests as an enlarging cutaneous or soft tissue lesion, less than 10% of cases have no skin involvement, with the retroperitoneum being the most frequently involved extracutaneous site. Approximately twenty cases of KHE with bony involvement have been reported in the literature to date, with only five of those cases involving the spine specifically.
We present a, rare case of KHE who presented with progressive fixed hyperlordotic deformity, multiple non-specific spinal lesions, and abnormal blood tests, posing a clinical and radiological diagnostic challenge. Additionally, we conducted a thorough review of the literature to compare and contrast the various multimodality imaging manifestations of KHE involving the spine.
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Metadata
Title
Kaposiform haemangioendothelioma of the spine associated with fixed hyperlordotic deformity and Kasabach–Merritt Syndrome: a case report and literature review
Authors
Leanne H. Q. Chin
Kevin K. F. Fung
Joyce P. K. Chan
Amanda N. C. Kan
M. K. Yuen
Publication date
10-08-2022
Publisher
Springer Berlin Heidelberg
Published in
Skeletal Radiology / Issue 4/2023
Print ISSN: 0364-2348
Electronic ISSN: 1432-2161
DOI
https://doi.org/10.1007/s00256-022-04152-z

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