Factor X Deficiency Presenting with Bilateral Chronic Subdural Hematoma

Department of Radiology, Medical School, Dicle University, Diyarbakir, Turkey.
Pediatric Neurosurgery (Impact Factor: 0.33). 06/2010; 46(1):54-7. DOI: 10.1159/000315004
Source: PubMed
ABSTRACT
Factor X deficiency is a rare coagulation defect that can result in several hemorrhagic manifestations including central nervous system hematomas in infants and children. In this case report, we present computed tomography (CT) and magnetic resonance (MR) imaging findings of bilateral chronic subdural hematomas due to factor X deficiency. Cranial CT and MR imaging in a hypoactive 7-month-old male infant with right hemiparesis revealed bilateral chronic subdural hematomas at different stages. Laboratory findings showed a severe factor X deficiency, with a level of 0.7%. After fresh frozen plasma replacement, the patient was operated and the large hematoma on the left side evacuated. The patient recovered uneventfully and remained asymptomatic during the 1-year follow-up.

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Case Report
Pediatr Neurosurg 2010;46:54–57
DOI: 10.1159/000315004
Factor X Deficiency Presenting with
Bilateral Chronic Subdural Hematoma
SenemSenturk
a
EbruGuzel
c
AylinHasanefendiogluBayrak
a
YasarBukte
a
AslanGuzel
b
Departments of
a
Radiology and
b
Neurosurgery, Medical School, Dicle University, and
c
Department of Radiology,
Veni Vidi Hospital, Diyarbakir , Turkey
er children [1] . Patients with intrauterine subdural hema-
tomas have been reported
[25] . Most pediatric cases are
caused by child abuse
[1] . Trauma, vitamin K deficiency,
thrombocytopenia and other coagulation disorders can
lead to subdural hemorrhage
[2, 4, 6, 7] . Although factor
X has been a well-known etiology for intracranial bleed-
ing, intracranial localizations of hematomas have not
been well documented. Reports of intraparenchymal
hemorrhage have been relatively more common
[8–12] .
Subdural hematomas have been reported in congenital
factor X deficiency
[5, 9, 13] . Fujimoto et al. [14] docu-
mented an infant who had intraventricular and epidural
hematomas. To our knowledge, recurrent chronic subdu-
ral hematoma has not been documented in the literature.
In this case report, we present computed tomography
(CT) and magnetic resonance (MR) imaging findings of
bilateral chronic subdural hematomas at different stages
in a patient with severe factor X deficiency.
C a s e R e p o r t
A 7-month-old male infant was referred to the neurosurgery
clinic of our institution with a history of general malaise and di-
minished movements on his right side. On admission, he was le-
thargic and his neurological examination revealed right hemipa-
Key Words
Factor X deficiency Hemorrhage Subdural hematoma
Abstract
Factor X deficiency is a rare coagulation defect that can
result in several hemorrhagic manifestations including cen-
tral nervous system hematomas in infants and children. In
this case report, we present computed tomography (CT)
and magnetic resonance (MR) imaging findings of bilateral
chronic subdural hematomas due to factor X deficiency. Cra-
nial CT and MR imaging in a hypoactive 7-month-old male
infant with right hemiparesis revealed bilateral chronic sub-
dural hematomas at different stages. Laboratory findings
showed a severe factor X deficiency, with a level of 0.7%.
After fresh frozen plasma replacement, the patient was oper-
ated and the large hematoma on the left side evacuated. The
patient recovered uneventfully and remained asymptomatic
during the 1-year follow-up.
Copyright © 2010 S. Karger AG, Basel
Introduction
Chronic subdural hematomas are usually encountered
in the elderly, and rarely seen in infants and children.
Their etiology is not always clear, but generally associated
with trauma. The incidence of chronic subdural hemato-
mas in children under 1 year of age is higher than in old-
Received: July 6, 2009
Accepted after revision: January 9, 2010
Published online: May 27, 2010
Senem Senturk
University of Dicle, Medical School
Department of Radiology
TR–21280 Diyarbakir (Turkey)
Tel. +90 412 248 8158 4314, Fax +90 412 248 8115, E-Mail snmsenturk
@ gmail.com
© 2010 S. Karger AG, Basel
1016–2291/10/0461–0054$26.00/0
Accessible online at:
www.karger.com/pne
This study was presented as an electronic poster at the 29th National
Radiology Congress Turkrad in Antalya, Turkey, November 1–5, 2008.
Page 1
Factor X Deficiency and Bilateral
Chronic Subdural Hematoma
Pediatr Neurosurg 2010;46:54–57
55
resis. He had no history of trauma; however, it came to our atten-
tion that the patient had been evaluated with the same symptoms
in another medical center 1 month earlier and his cranial CT had
been obtained. Bilateral isodense chronic subdural hematomas,
more predominant on the left side, had been detected on the ini-
tial CT ( fig.1 ). His symptoms resolved on follow-up; however,
they recurred 1 month thereafter. Cranial CT was repeated with
a preliminary diagnosis of recurrent chronic subdural hematoma.
The CT revealed that the amount and density of subdural hema-
tomas were increased bilaterally. The focal hyperdense area in the
left frontal region represented a fresh hemorrhage ( fig.2 ). Unlike
on the previous CT, there was a mass effect on the left hemisphere
with an obliteration of the frontal horn of the left lateral ventricle.
The falx cerebri was mildly shifted to the right.
The laboratory tests revealed a hematocrit level of 35.4%, he-
moglobin at 11.9 g/dl, white blood cell count of 17,100/mm
3
, plate-
let count of 365,000/mm
3
, prothrombin time of 103.0 s (control:
14.6 s) and partial thromboplastin time of 83.4 s (control: 26.4 s).
The factor X level was found to be 0.7%, which represented a se-
vere deficiency. The levels of factors II, V, VII, VIII, IX, XI and XII
were within normal limits.
Four days after the CT scan, MR imaging was performed be-
fore the operation. On T1-weighted images (repetition time/echo
time: 444/15 ms), bilateral hyperintense chronic subdural hema-
tomas were observed ( fig.3 ). There was a small right subdural he-
matoma and a large, growing subdural hematoma on the left. On
T2-weighted images (repetition time/echo time: 4,540/113 ms), the
small right subdural hematoma was mildly hyperintense relative
to the gray matter. However, the large left subdural hematoma was
markedly hypointense, representing the early subacute phase of a
recurrent hemorrhage ( fig. 3 ). The midline shift was increased,
and the left lateral and third ventricle were obliterated. There was
a focal T1- and T2-hypointense area in the hematoma, at the same
location where hyperdensity was observed on CT ( fig.3 ). Hypoin-
tensity represented an acute hemorrhage. Moreover, the hemato-
ma on the left side included T1- and T2-hyperintense components
adjacent to the brain parenchyma, which represented the old hem-
orrhage and multilayered structure of the hematoma.
ab
ab
cd
Fig. 1. Axial CT revealing small bilateral isodense subdural hema-
tomas.
Fig. 2. Axial CT obtained 1 month after the first CT. A left sub-
dural hematoma grew (
a ), causing a midline shift and compres-
sion of the left lateral ventricle (
b ). The hyperdense area on the left
side represented an acute hemorrhage.
Fig. 3. MR imaging obtained 4 days after CT. The hematoma on
the left was larger. Most of the hematoma was hyperintense on
T1-weighted images. Focal T1 (
a ) a n d T 2 ( c ) hypointensity (ar-
rows) represented an acute hemorrhage. The small T1- (
b ) and
T2-hyperintense (
d ) component (asterisks) adjacent to the brain
parenchyma was in concordance with the older part of the hema-
toma.
Page 2
Senturk /Guzel /HasanefendiogluBayrak /
Bukte
/Guzel
Pediatr Neurosurg 2010;46:54–57
56
After fresh frozen plasma replacement, the patient was oper-
ated and the hematoma on the left side evacuated. The capsule and
membranes observed at operation confirmed the diagnosis of
chronic subdural hematoma at different stages.
Burr hole craniotomy was used to evacuate the hematoma. Af-
ter a skin incision of 3–4 cm over the maximum thickness of the
hematoma, a burr hole was made and the dura cauterized and
opened by cross-shaped incision. Approximately 20 ml of hemor-
rhagic fluid under pressure was let out, and the hematoma cavity
was irrigated with normal saline. A silicone drain was left in the
subdural space. Postoperative CT revealed a minimal subdural
collection on the left ( fig.4 a). On the third postoperative day, a
clear fluid consistent with the cerebrospinal fluid was observed,
and the drain was moved out. The patient recovered uneventfully
and was discharged 10 days after surgery. Prophylaxis with a pro-
thrombin complex concentrate would have been conceivable;
however, the patient was followed up without any medication be-
cause the agent was not available. Two months after surgery, CT
showed no evidence of subdural hematoma ( fig.4 b).
The patient has been followed up monthly with physical and
neurological examinations and laboratory tests including total
blood count, prothrombin time and partial thromboplastin time
in the pediatric and neurosurgery outpatient clinics of our hospi-
tal. He has remained asymptomatic without recurrence of intra-
cranial hemorrhage for 1 year.
Discussion
Factor X deficiency is a rare autosomal recessive co-
agulation disorder. It may be manifested during the first
few days of life by umbilical stump bleeding. Subsequent
hemorrhagic problems such as easy bruising, spontane-
ous hematoma, hemarthrosis, epistaxis, menorrhagia,
hematuria, intracranial hemorrhage and gastrointestinal
hemorrhage are frequent
[15] . Intracranial hemorrhage is
the most dreadful complication and an important cause
of death in factor X deficiency. Patients with intracranial
hemorrhage are between 1 and 5 months of age, and they
generally do not have a history of prior trauma
[9] . In
these patients, early diagnosis is crucial for the preven-
tion of intracranial bleeding by aggressive replacement
therapy.
Intracranial localizations of hematomas due to factor
X deficiency have not been well documented. Intraparen-
chymal hematoma appears to be the most common type
in patients with factor X deficiency
[8–12] . Subdural he-
matomas have been reported in congenital factor X defi-
ciency
[5, 9, 13] but, to our knowledge, recurrent chronic
subdural hematoma has not been documented.
The localization of an intracranial hematoma is im-
portant for the treatment strategy. Patients with intrapa-
renchymal hematomas are managed conservatively by
factor concentrate/plasma replacement therapy, whereas
surgery is required for patients with subdural hematomas
who have deteriorating neurological features in spite of
adequate factor support
[9] . Surgery was indicated in the
treatment of our patient to evacuate the large subdural
hematoma and to relief intracranial hypertension. There
is no specific factor X concentrate, thus either fresh fro-
zen plasma or prothrombin complex concentrate is used
as prophylaxis. Bowles et al.
[12] recommend the use of
prothrombin complex concentrate as prophylaxis in chil-
dren with severe congenital factor X deficiency. Prophy-
laxis with prothrombin complex concentrate would have
been conceivable for the patient we report on; however,
we did not perform it because the agent was not available
at the time of hospitalization. We used fresh frozen plas-
ma as prophylaxis before the operation.
CT remains the most important imaging modality in
the initial evaluation of intracranial hemorrhage. MR im-
aging provides more precise localizations and delinea-
tions of hematoma margins and of a hematomas mass
effect on adjacent structures. Chronic cerebral intrapa-
renchymal hematomas appear hypodense on CT, and hy-
pointense on T1- and T2-weighted MR images. However,
the density and intensity patterns of chronic subdural he-
matomas differ from those of intraparenchymal hemato-
mas owing to the absence of the blood-brain barrier in
the subdural space and the tendency to recurrent bleed-
ing
[16] . Mixed intensity and T1 hyperintensity are com-
monly observed on MR imaging due to recurrent hemor-
rhage
[17] .
The right subdural hematoma of our patient was small
and hyperintense on T1- and T2-weighted MR images.
The left subdural hematoma was large and of mixed in-
ab
Fig. 4. P o s t o p e r a t i v e C T s c a n s . a Two days after surgery. Minimal
subdural collection with air bubbles.
b Two months after surgery.
The subdural hematoma and collection had completely disap-
p e a r e d .
Page 3
Factor X Deficiency and Bilateral
Chronic Subdural Hematoma
Pediatr Neurosurg 2010;46:54–57
57
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tensity. It was predominantly hyperintense on T1-weight-
ed images and hypointense on T2-weighted images, in
concordance with the subacute phase of a hemorrhage.
On the left there was also a small T1- and T2-hyperin-
tense hematoma component adjacent to the brain paren-
chyma with an intensity pattern similar to that of the
chronic right subdural hematoma. This component was
the older hematoma component, which represented
membrane formation and the multilayered structure of
the chronic subdural hematoma. The hyperdense area on
CT, in the left frontal subdural hematoma of the patient,
was showing an acute hemorrhage. On MR imaging, T1
and T2 hypointensity at the same site also represented the
acute hemorrhage, and the growing nature of the chron-
ic subdural hematoma.
In addition to child abuse, accidental trauma, throm-
bocytopenia, vitamin K deficiency and other coagulation
disorders, factor X deficiency should also be kept in mind
when chronic subdural hematoma is encountered in in-
fants. Factor X deficiency can present with intracranial
hemorrhage at an early age. An intracranial hemorrhage
may be the primary bleeding episode leading to the de-
tection of factor X deficiency. Most hematomas are intra-
parenchymal, even if subdural, epidural and intraventric-
ular hematomas can be seen.
It must be remembered that nonaccidental injury is
the most common cause of subdural hematomas in chil-
dren under 2 years of age
[18] . The presence of retinal
hemorrhages, bone and rib fractures, a delay in presenta-
tion and the young age of the infants suggests child abuse
is the most likely cause of these injuries. Most of the un-
explained infant subdural hematomas are caused by child
abuse, and coagulation disorders including factor X defi-
ciency are rarely encountered.
Conclusion
Factor X deficiency is a rare coagulation defect that
can result in large chronic subdural hematomas in in-
fants and children. In these patients, due to recurrent
hemorrhages, the density and intensity patterns on CT
and MR imaging are different from those of intraparen-
chymal hematomas.
Page 4
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