Posterior fossa extradural haematoma with cerebral venous sinus thrombosis precipitates haemophilia a diagnosis: a paediatric case report and literature review

Journal article : Review

Background: Posterior fossa (PF) fractures are often associated with cerebral venous sinus thrombosis (CVST) yet rarely require treatment beyond anticoagulation. When observed with synchronous, atypical extradural haematoma (EDH) over the transverse sinuses, the management is more equivocal. We present a rare paediatric case of traumatic PF EDH with CVST incidentally unveiling an inherited bleeding disorder (IBD). Observations: A 3-year-old boy presented following an unwitnessed head injury. Serial imaging confirmed marginal growth of the EDH with stable CVST and an undisplaced occipital skull fracture. A clotting profile sent via PICU admission bloods informed a new diagnosis of haemophilia A. Conservative management monitored EDH evolution against operative risks over the sinus with a clotting disorder. Haematological treatment navigated the quandary of FVIII replacement to prevent bleeding against the need for CVST anticoagulation. The latter was not initiated. A full recovery was made without neurological deficit. Conclusions: A unifying diagnosis for these incongruent radiological features presented distinct diagnostic and therapeutic challenges. Haematological screening can be misleading in paediatric patients with unclear implications as a diagnostic measure. Excluding coagulopathies is more significant if neurosurgical intervention is indicated, evaluated against individual clinical correlates and symptomatology. In the absence of standardised guidelines for the management of traumatic paediatric EDH with intercurrent CVST and IBDs, the case presented an invitation to thought.

Authors: Akhbari, M; Owen, W; Honeyman, SI; Badle, S; Calisto, A

• Publication status: Published
• Peer review status: Peer reviewed
• Publisher: Springer
• Journal: Child's Nervous System
• Volume: 41
• Issue: 1
• Article number: 394
• Publication date: 2025-12-02
• Acceptance date: 2025-11-10
• DOI: 10.1007/s00381-025-07040-8
• EISSN: 1433-0350
• ISSN: 0256-7040
• Language: English
• Keywords: Posterior fossa; Haemophilia; Coagulopathy; TBI; Haematoma; Extradural; Paediatric neurosurgery; Cerebral venous sinus thrombosis
• Subtype: Review