Teaching Case

3-month old twin boy (full term) presented to A&E with fevers, lethargy and was noted to have a bulging fontanelle.
A CT scan was requested to exclude possibility of intracerebral abscess. Coagulation studies returned as abnormal at this point. The child deteriorated further. PICU and neurosurgical input were then required.

Abutting the falx cerebri posteriorly on the right there is an extra-axial low/high attenuation fluid-fluid level. The high attenuation represents clotted subdural blood (HU 60) and the low attenuation area is representative of unclotted blood.

This is an example of the “Swirl Sign” [1] which is suggestive of hyper-acute extra-axial haemorrhage. Previous descriptions of the swirl sign have described active and chronic components. The active component is often a circular lesion that is low or iso-attenuating to the brain, representing exuding unclotted blood.

A repeat scan (to assess internal changes following the child developing focal seizures) did not demonstrate significant interval change and the child was transferred to the tertiary centre for paediatric neurosurgical input – craniotomy and evacuation.
Post operatively a repeat scan showed a reduction in the extra-axial blood around the cerebellum but also noted a new finding of blood in the transverse sinus.

A.Background

Most intracranial haemorrhages in infants are due to Non-Accidental Injury (NAI) - e.g. shaken baby syndrome [2]. Occasionally brain haemorrhage in non-mobile infants is due to inherited coagulopathy. There have also been published case reports describing rare metabolic conditions, such as glutaric aciduria and Menkes disease, presenting with ICH [7 & 8]. Other causes include arteriovenous malformations and choroid plexus abnormalities.

Haemophilia is the second most common congenital bleeding disorder. 85% of cases are due to Factor VIII deficiency (Haemophilia A), an X-linked recessive disorder [3]. Up to 30% of children who present have a negative family history [3].

Despite this, haemophilia presenting with intracranial haemorrhage is uncommon. It is particularly uncommon in the infantile age group and is usually associated with a history of head trauma [4].

In a retrospective study of 30 children with haemophilia who presented with ICH, only 4 had severe neurological deficits and one died [5].

C. Imaging Perspective

The literature suggests that the radiological finding of subtentorial subdural haemorrhage is highly specific for NAI [5].

Thus any isolated radiological finding of subdural haemorrhages in this age group should be correlated with clotting studies before making a diagnosis of NAI . It is essential coagulation studies are done as part of the initial paediatric assessment.

D. Outcome

In our case, without a previous history of haemophilia and trauma, NAI was initially suspected until the coagulation study results were made avaliable. The coagulation results coupled with the imaging helped suggest a diagnosis of ICH secondary to haemophillia.

Post neurosurgical intervention, the infant had a slight residual left-sided weakness and was discharged on anti-epileptic medications with follow up by the haematology services planned.