Cerebral Vascular Accidents (CVA) in children, a term used to describe neurological accidents caused either by decreased blood flow (obstruction and ischemia) or rupture of intracranial blood vessels (hemorrhage) [1]. CVAs are considered to be rare, but they retain a significant morbidity and mortality rate nonetheless [1]. Almost 50% of strokes in children are caused by acute ischemic events [2,3]. To be specific, the posterior circulation is mainly affected in about 30–40% of all childhood strokes, a percentage that is significantly more frequent than that in adults [4]. There are several etiologies for ischemic and hemorrhagic strokes in children: cardiac, hematologic (Sickle cell disease, Prothrombotic disorders), infectious, vascular, syndromic, and metabolic disorders, vasculitis, oncologic, trauma, or drugs [1]. In this article lies the case of a child that presented with headache and found to have Intracranial Bleeding (ICB) associated with Antithrombin III deficiency. To our knowledge, this is the first case reported at this age group from Lebanon.

Case Description

A 10 years old girl with no significant past medical history other than chronic sinusitis presented to our emergency department for sudden onset of stabbing headache, mainly at the right temporal region while attending an online class. The headache was associated with two episodes of non-projectile vomiting, slurred speech, and ataxia. It was the first episode. There was no history of trauma or loss of consciousness. Parents denied fever, weight loss, or night sweats. Vital signs were within normal range, and the patient was hemodynamically stable. On physical exam, she appeared somnolent with a Glasgow Coma Scale (GCS) of 14/15 (absence of spontaneous speech, and preserved comprehension). 

Figure 1: CT scan of the brain without contrast at day 1 of presentation, axial view showing bilateral cerebellar hematomas

She was conscious, able to identify all the parts of her body, obeying commands, and oriented to time, place, and person. Further examination showed nystagmus in both eyes. The patient was unable to walk without support despite motor power being 3/4 in the four limbs. All reflexes were preserved with no sensory loss. An urgent CT scan of brain was done showing bilateral cerebellar hematomas reaching 45 mm at the right side and 34 mm at the left side with a hypodense aspect of the superior part of the cerebellar vermix at the right, denoting edema (Figure 1) with caudal displacement of cerebellar tonsils at the level of foramen magnum without herniation at presentation (Figure 2). No hydrocephalus is seen in Figure 3.

Figure 2: CT scan of the brain without contrast at day 1 of presentation, sagittal view showing caudal displacement of cerebellar tonsils at the level of foramen magnum without herniation

Figure 3: CT scan of the brain without contrast at day 1 of presentation, axial view showing no hydrocephalus

The patient was transferred to the pediatric intensive care unit (PICU). Primary laboratory workup showed normal hematological values (Prothrombin time, Partial thromboplastin time). Platelets were 369000x10⁹/L, Hemoglobin level was 14 g/dl, and C - reactive protein level was 2 mg/L. Pan Cultures (Blood and Urine) were no growth. PCR for neuro9 was ordered; it didn’t detect any germs. The patient was started on Ceftriaxone, Vancomycin, and Acyclovir for coverage of possible meningitis or encephalitis. A Brain MRI with and without contrast revealed bilateral cerebellar hemorrhage (Figure 4) with no evidence of pathological enhancement (Figure 5). The cerebral tonsils are close to the foramen magnum. MRA and MRV sequences (Figure 6,7) were realized and ruled out any dural fistula, venous thrombosis or arterial aneurysm. No surgical correction was attempted on the first day due to the absence of hydrocephalus and the bilateral aspect of the hemorrhage.

Figure 4: MRI of the brain, T2 gradient echo sequence axial view showing bilateral cerebellar hemorrhage

Figure 5: MRI of the brain with intravenous contrast administration, axial view showing no evidence of pathological enhancement

Figure 6: Magnetic Resonance Venography (MRV) showing no evidence of venous thrombosis

Figure 7: Magnetic Resonance Angiography (MRA) showing no evidence of arterial aneurysmal dilatation

Figure 8: CT scan of the brain without contrast at day 2 of presentation, sagittal view showing cerebellar tonsils herniation

Early in the morning of the second day of admission, the patient developed disorientation with GCS = 3. No response to any stimuli was documented. Blood pressure was 200/90. Heart rate decreased gradually reaching 55 beats per minute with desaturation and cyanosis. Thus, the patient was intubated and antihypertensive medication was started. Repeated CT brain showed herniation of cerebellar tonsils cross the foramen magnum (Figure 8) with increase in cerebellar hemorrhage at the left side (Figure 9) reaching 42 mm versus 34 mm in the previous day. A hydrocephalus developed (Figure 10).

Furthermore, mydriasis developed. A surgical decompression with Ventriculo-Peritoneal shunt (VPS) was done, but with no improvement. A flat EEG was recorded, and the patient developed cardiopulmonary arrest three days later and passed away. To note, an autoimmune workup, to rule out vasculitis, had been ordered including Anti Ds-DNA, ANA, ANCA, P-ANCA, C3, C4, Anti-Phospholipids. However, no antibodies were detected. Urine toxicology screen was negative. The result of Neuro 9 PCR and respiratory panel PCR were also negative. Echocardiography showed normal heart architecture with no vegetation. Finally, the hematologic workup (Protein C and S, Factor V Leiden, Anti-Thrombin 3, Factor 12, and Factor 13) detected an Antithrombin III deficiency. Thus, it’s a case of a child with intracerebral bleeding coupled with anti-thrombin 3 deficiency.

Figure 9: CT scan of the brain without contrast at day 2 of presentation, axial view showing increase in the bilateral cerebellar hematomas, especially on the left side

Figure 10: CT scan of the brain without contrast at day 2 of presentation, axial view showing hydrocephalus

This is a case of a child who was diagnosed to have a sudden onset of cerebellar hemorrhage, a relatively uncommon event, where 85% of posterior fossa strokes in pediatric population are ischemic in nature [5]. However, 15% of strokes do exist due to hemorrhagic events in an autopsy series. The presentation of the patient was very confusing and paused a big mystery about the etiology of the stroke. Acute cerebellar hemorrhage accounts for around 20-30% of all hemorrhages in children. The most common cause of the aforementioned strokes is arteriovenous malformation (AVM). 15–20% of all AVMs in children are cerebellar in location. Other possible causes may be cavernomas or aneurysm [6]. Back to our patient, the MRA and MRV images revealed no vascular malformation: no aneurysm, no AVM, and no cavernoma thus eliminating the most common causes of hemorrhagic stroke after infancy; i.e. vascular malformation [1]. In addition, strokes can be due to other causes such as post-viral encephalitis (HIV, Varicella, Enterovirus, Parvovirus 19, Influenza virus type A, and Coxsackie virus), or post-bacterial (Tuberculosis, Mycoplasma, Chlamydia) [1]. However, the patient had a negative viral panel and negative blood cultures. Moreover, other causes like vasculitis (idiopathic, arteritis), drug-induced (Heroin, Cocaine, Amphetamine, etc.), tumor or trauma [1] can lead to hemorrhagic or ischemic strokes. However, in our patient all autoimmune workup eliminated any possible vasculitis, and a negative urine toxicology screen was obtained. Also, with the absence of any history of fall down or documented fracture in the CT brain, drug abuse and trauma were eliminated. Cerebellar hemorrhage in children can be associated to blood dyscrasias or hematological causes such as prothrombotic events, coagulation disorders (Factor VII or Factor VIII deficiency) [1]. To note, prothrombotic events such as Protein C, Protein S, Factor V Leiden, or Antithrombin III deficiency have been associated to acute ischemic strokes in previous case reports [1]. In addition, there is an association between pediatric ischemic strokes and hereditary thrombophilia, but hemorrhagic stroke and thrombophilia are usually seen in perinatal life not later on during childhood [7]. In contrast, this patient had a cerebellar hemorrhage and Antithrombin III deficiency. This finding is so contradictory, for Antithrombin III deficiency is usually associated with thrombotic ischemic events, and in addition, most of these events manifest early around perinatal period. It’s uncommon to manifest at childhood which makes this case so rare. After eliminating the common causes of cerebellar hematoma in this patient, it was suggested that the etiology is idiopathic cerebellar hemorrhage in childhood associated with Antithrombin III deficiency. In the end, something to keep in mind is that the patient was under stress during the online course, as stated by the parents, with a context of thrombophilia. From this, a question may be imposed: Can stress induce a vessel rupture on top of thrombosis in a predisposed patient?

Pediatric stroke is a serious condition. It needs rapid diagnosis and intervention, whether medical or surgical, to decrease the high morbidity and mortality. A golden hour is present for intervention to decrease sequelae such as cerebral palsy.

1. Tsze, D.S., and J.H. Valente. “Pediatric Stroke: A Review.” Emergency Medicine International, 2011, pp. 1–10.

2. Earley, C.J. et al. “Stroke in Children and Sickle-Cell Disease: Baltimore-Washington Cooperative Young Stroke Study.” Neurology, vol. 51, no. 1, 1998, pp. 169–176. https://doi.org/10.1212/WNL.51.1.169.

3. Carvalho, K.S., and B.P. Garg. “Arterial Strokes in Children.” Neurologic Clinics, vol. 20, no. 4, 2002, pp. 1079–1100. https://doi.org/10.1016/S0733-8619(02)00012-9.

4. Mackay, M.T., P. Monagle, and F.E. Babl. “Improving Diagnosis of Childhood Arterial Ischaemic Stroke.” Expert Review of Neurotherapeutics, vol. 17, no. 12, 2017, pp. 1157–1165. https://doi.org/10.1080/14737175.2017.1395699.

5. Shenkin, H., and M. Zavala. “Cerebellar Strokes: Mortality, Surgical Indications, and Results of Ventricular Drainage.” The Lancet, vol. 320, no. 8295, 1982, pp. 429–432. https://doi.org/10.1016/S0140-6736(82)90453-6.

6. Kondziolka, D. et al. “Arteriovenous Malformations of the Brain in Children: A Forty Year Experience.” Canadian Journal of Neurological Sciences, vol. 19, no. 1, 1992, pp. 40–45. https://doi.org/10.1017/S0317167100042517.

7. Torres, V.M., and V.A. Saddi. “Systematic Review: Hereditary Thrombophilia Associated to Pediatric Strokes and Cerebral Palsy.” Jornal de Pediatria (Versão em Português), vol. 91, no. 1, 2015, pp. 22–29. https://doi.org/10.1016/j.jpedp.2014.07.005.