Hypofibrinogenemia Revealing Localized Intravascular Coagulopathy in a Child with Venous Malformations: A Case Report

Aims: VMs are slow-flow vascular malformations present at birth. Only 1% are multifocal. These lesions are complicated by LIC characterized by elevated D-dimer and decreased fibrinogen levels.

The aim of this article is to report a rare case of multifocal VMs complicated with LIC, revealed by low fibrinogen level. To the best of our knowledge, no case has been reported to date in the Maghreb countries.

Presentation of Case: A 14-year-old male patient with multifocal VMspresented for appearance of a lateral cervical mass, rapidly increasing in size. Cervical CT scan revealed a tumor-like right latero-cervical formation, measuring (67x65x96.8) mm. The patient underwent an excision of a vascular-like latero-cervial mass, complicated by a postoperative hematoma for which he was reoperated to ensure hemostasis. Haematological investigations have essentially demonstrated an elevation of the D-dimer, with a very low fibrinogen level. These biological abnormalities occurring in association with venous malformations has been termed LIC.

Discussion: The correlation between LIC and VMs is known. It is characterized by the elevation of D-dimers and fibrin degradation products, low levels of fibrinogen, and sometimes minor-to-moderate thrombocytopenia.

Low molecular weight heparin can be used for prevention from decompensation of severe LIC into disseminated intravascular coagulopathy (DIC).

Conclusion: Recognizing LIC in patients with VMs is essential for detecting clinical and biological abnormalities, optimizing therapeutic management and thus preventing hemorrhagic and thromboembolic risks which may be life-threatening.