ABSTRACT
A 5 month-old baby developed non-ceasing intra-peritoneal bleeding after extensive surgical biopsy for an hepatoblastoma. A single recombinant activated factor VII injection following enlarged hepatectomy helped to resolve quickly this life-threatening haemorrhagic syndrome.
Subject(s)
Coagulants/therapeutic use , Factor VIIa/therapeutic use , Hepatoblastoma/surgery , Liver Neoplasms/surgery , Postoperative Hemorrhage/prevention & control , Biopsy/methods , Hemostasis, Surgical , Humans , Infant , Injections , Liver/pathology , Male , Recombinant Proteins , Treatment OutcomeABSTRACT
Factor XIII (FXIII) deficiency is a rare autosomal recessive congenital disorder of haemostasis, associated with a high risk of intracranial haemorrhage. Intracranial haemorrhage can result in neurological sequelae including seizure disorders. In some cases, medically intractable epilepsy led to epilepsy surgery. Little has been reported on the management of FXIII deficiency during surgery, and there is only a few data on the management, safety and efficacy of epilepsy surgery in the patients with haemostatic disorder. We report here an epilepsy neurosurgery in a case of severe FXIII deficiency.
