Paravertebral and Retroperitoneal Vascular Tumour Presenting with Kasabach-Merritt Phenomenon in Childhood, Diagnosed with Magnetic Resonance Imaging

Gonca Keskindemirci,Arzu Akçay,Nuray Aktay Ayaz,Bilge Bilgiç,Ali Er,Ensar Yekeler,Gönül Aydoğan,Deniz Tuğcu

doi:10.1155/2015/537530

Abstract

Kasabach-Merritt phenomenon (KMP) is characterized by vascular tumour and consumptive coagulopathy with life-threatening thrombocytopenia, prolonged prothrombin time and partial thromboplastin time, hypofibrinogenemia, and the presence of high fibrin split products. We report a case of 3-year-old boy with local aggressive vascular lesions associated with KMP. Magnetic resonance imaging revealed an extensive lesion at paravertebral and retroperitoneal regions that was infiltrating vertebrae. Although we did not get any response to steroid or propranolol treatment, partial response was observed radiologically with interferon-alpha treatment. Unfortunately, the patient died because of the uncontrolled consumptive coagulopathy that led to intracranial hemorrhage which was caused by huge knee hematoma after minor trauma.

Highlights

Vascular anomalies include a spectrum of disorders from simple lesions to life-threatening entities [1]
KasabachMerritt phenomenon is characterized by vascular tumour and consumptive coagulopathy with life-threatening thrombocytopenia, prolonged prothrombin time (PT), and activated partial thromboplastin time, hypofibrinogenemia, and the presence of D-dimer and fibrin split products [2]
We describe a 3-year-old boy with unusual localization of extensive vascular lesions involving spinal vertebrae from T11 to L5 that caused fatal consumptive coagulopathy

Summary

Case Report

Paravertebral and Retroperitoneal Vascular Tumour Presenting with Kasabach-Merritt Phenomenon in Childhood, Diagnosed with Magnetic Resonance Imaging. Kasabach-Merritt phenomenon (KMP) is characterized by vascular tumour and consumptive coagulopathy with life-threatening thrombocytopenia, prolonged prothrombin time and partial thromboplastin time, hypofibrinogenemia, and the presence of high fibrin split products. We report a case of 3-year-old boy with local aggressive vascular lesions associated with KMP. Magnetic resonance imaging revealed an extensive lesion at paravertebral and retroperitoneal regions that was infiltrating vertebrae. We did not get any response to steroid or propranolol treatment, partial response was observed radiologically with interferonalpha treatment. The patient died because of the uncontrolled consumptive coagulopathy that led to intracranial hemorrhage which was caused by huge knee hematoma after minor trauma

Introduction

Discussion