Cerebral Venous Thrombosis as a First Vaso-Occlusive Crisis in a Sickle Cell Disease Patient
Eman Almusallam, Eman Alhassan, Ali Alsaeed, Aqeela Alhashim, Mohammed Alhazzaa*
Department of Neurology, National Neuroscience Institute, Saudi Arabia
Submission: March 12, 2019; Published: March 26, 2019
*Corresponding author: Mohammed Alhazzaa, Department of Neurology, National Neuroscience Institute, King Fahad Medical City, Riyadh, Saudi Arabia
How to cite this article: Eman A, Eman A, Ali A, Aqeela A, Mohammed A. Cerebral Venous Thrombosis as a First Vaso-Occlusive Crisis in a Sickle Cell
Disease Patient. Open Access J Neurol Neurosurg. 2019; 10(3): 555790. DOI: 10.19080/OAJNN.2019.10.555790.
Sickle Cell Disease (SCD) is a well-known precipitant of vascular occlusions when triggers of SCD crisis are active. This process commonly impacts the arterial circulation in different organs. SCD has not been recognized as a leading cause of Cerebral Venous Thrombosis (CVT) and the crisis does not typically start within the venous system. In this report, we present a case of SCD patient presenting with venous thrombosis as an initial vascular attack.
Cerebrovascular complication of sickle cell anemia is well established in the literature. However, the main focus has been directed toward arterial vaso-occlusive crisis and other rare associated arteriopathies such as Moyamoya syndrome [1,2]. Intracerebral Hemorrhage (ICH) is a known complication of SCD and it typically happens in adult patients due to different reasons such as aneurysmal formation . Recently, a number of reports have been published about venous thrombosis occurrence in the setting of SCD [4-7]. This probably reflects the nature of the disease and on the other hand the advancement in diagnostic methods. Cerebral Venous Thrombosis (CVT) can cause ICH. This might implicate that CVT could be a potential mechanism of ICH among SCD patients where provoked or unprovoked blood flow abnormalities are likely to generate thrombosis within veins similar to the arterial crisis. In this brief encounter, we will discuss venous thrombosis in a young patient with sickle cell anemia who never had any prior vascular crisis.
An 18-year-old right-handed male presented to the Emergency Department (ED) with right-sided weakness. He is known to have sickle cell anemia on folic acid. He was a smoker. He had no history of previous neurological manifestations or systemic vaso-occlusive crisis. His physical examination revealed aphasia and right-sided hemiplegia. During ED visit, his consciousness level deteriorated and he required intubation. His CT scan of the brain revealed a left temporal
lobe large hemorrhage with significant vasogenic edema and midline shift (Figure 1). He required immediate decompressive hemicraniectomy. Intraoperatively, a thrombosis of the left vein of Labbe was noted. Although Magnetic Resonance Venography (MRV) showed patent main venous sinuses, a four-vessel cerebral angiography demonstrated the paucity of the left convexity cortical veins, confirming a cortical and/or deep venous thrombosis (Figure 2).
His Complete Blood Count revealed hemoglobin (Hb) (8.7)
and platelets (306). He had normal thrombophilia testing
including antiphospholipid antibodies, Factor V Leiden mutation,
antithrombin, protein C, and protein S level. Hemoglobin
electrophoresis showed HbS 73% and Hb A 20%. He was
evaluated by Hematology and started on hydroxyurea 500 mg
twice daily and exchange transfusion where Hb S was lowered
to 26%. He had smooth hospital course with slow recovery.
Then, he required extensive rehabilitation where he showed
significant improvement. He was discharged on aspirin 81 mg
daily and hydroxyurea 1000 mg daily. He received multiple
exchange transfusion. After two years of follow up, there has
been no recurrence of any vascular crisis.
Sickle cell disease is prevalent in some regions of Saudi Arabia
where it has unique presentations involving different systems.
This hematological condition produces its symptoms following
a complicated pathological cascade involving disfigured sickled
red blood cells, endothelium, and coagulation system [1,2]. As
a result, hyper viscosity and coagulopathy promote occlusive
vasculopathy, which is considered the most serious hallmark
of this disease. Hence, SCD is associated with increased risk
of ischemic strokes secondary to acute thrombosis and other
known vasculopathies such as Moyamoya pattern [1,2].
Although Intracerebral hemorrhage (ICH) is less commonly
seen, it is considered as another vascular complication of SCD
[1,3]. Adults with SCD are more likely to develop ICH due to
unclear mechanisms. Some suggested associated aneurysms
and Moyamoya disease as potential etiologies , but those
do not provide a satisfactory explanation in several ICH cases.
There have been few reports about CVT in SCD after certain
precipitants [4-7], and CVT can lead to a brain hemorrhage.
In this case, we postulate a new pathophysiological concept
underlying this devastating disease in which CVT could be a
variant of occlusive disease among SCD patients where it can
be the first presenting vascular crisis. Therefore, we suggest
a similar sickling mechanism that may predominantly affect
venous system causing acute occlusion. Arterial infarctions are
well understood in SCD and easy to diagnose, however, CVT might
have been overlooked in cases of ICH. As a result, CVT should
be diligently sought since it can be a potential cause of ICH. In
our case, intraoperative recognition of the venous thrombus was
a key in diagnosis although MR venogram was not suggestive.
This also raises another observation of whether SCD selectively
involves the deep venous system which adds another layer of
complexity in identifying thrombosis with the use of regular
imaging modalities. Hence, catheter cerebral angiography would
be of great help in diagnosis.
There are reported precipitating factors that might have
played a role in the development of CVT in patients with SCD [5-
7]. Dehydration and infection are common provoking factors for
the occlusive crisis in SCD. Our patient had no definite triggers
apart from being a smoker, which probably contributed to
increased blood viscosity. He has no family history of thrombosis
and his thrombophilia workup is unremarkable. This may also
add to the notion of CTV as a potential occlusive variant. There
have been other factors reported as possible triggers of CVT
in SCD patients such as exchange transfusion and following
hydroxyurea initiation [4,5]. CVT in those cases was attributed
to rapid correction of hemoglobin level. However, this could be
a coincidence and we don’t have a strong evidence to reach a
level of association or causation. Our patient had no vascular
exacerbation after exchange transfusion or use of hydroxyurea.
In conclusion, we are presenting a case of venous thrombosis
among adult sickle cell patient as a first clinical vascular attack.
CVT might be underestimated condition in SCD and therefore
missed in patients with acute ICH, unless specifically sought with
advanced imaging modalities. Diagnosis certainty will impact
the treatment plan. There is growing evidence that supports
the presence of a venous variant of acute occlusive crisis.
However, there are several questions remain unanswered. It is
unclear if this venous variant has unique provoking factors and
pathogenesis. It is also uncertain whether exchange transfusion
is efficacious in preventing future venous events as compared
to the arterial variant. Further studies are required to recognize
SCD patients at high risk of CVT and identify ideal diagnostic and