Cerebrospinal fluid
rhinorrhoea Cerebrospinal fluid rhinorrhoea: diagnosis and management
Allan Abuabara DDS,
Specialist in Dental and Maxillofacial Radiology, Health Division,
Joinville City Hall, Joinville, Santa Catarina, Brazil
Correspondence:
Dr. Abuabara Rua
Quintino Bocaiúva, 102, apto 206, Joinville, SC, Brazil. 89204-300;
E-mail:
allan.abuabara@gmail.com
Received: 25-07-2006
Accepted: 30-01-2007
Abuabara A.
Cerebrospinal fluid rhinorrhoea: diagnosis and manage-ment
.Med Oral Patol Oral
Cir Bucal 2007;12:E397-400.
© Medicina Oral S.
L. C.I.F. B 96689336 - ISSN 1698-6946
Abstract
A cerebrospinal
fluid (CSF) rhinorrhoea occurs when there is a fistula between
the dura and the skull base and discharge of CSF from the nose. CSF
rhinorrhea or liquorrhoea commonly occurs following head
trauma (fronto-basal skull fractures), as a result of intracranial
surgery, or destruction lesions. A spinal fluid leak from the
intracranial space to the nasal respiratory tract is
potentially very serious because of the risk of an ascending
infection which could produce
fulminant
meningitis.
This article
reviewed the causes, diagnosis and treatment of CSF leakage. A
PUBMED search of the National Library of Medicine was conducted.
CSF leak most
commonly occurs following trauma and the majority of cases
presenting within the first three months. CSF rhinorrhoea have
significantly greater incidence of periorbital haematoma. This
suggests that patients with head injuries and features of
periorbital haematoma are at greater risk of unobserved dural tear
and delayed CSF leakage.
In the presence of a
skull base fracture on computed tomography and a clinical CSF leak,
there is no need for a further confirmatory test. In cases where a
confirmatory test is needed, the beta-2 transferrin assay is the test
of choice because of its high sensitivity and specificity.
A greater
proportion of the CSF leaks in the patients resolved spontaneously.
CSF fistulae persisting for > 7 days had a significantly
increased risk of developing meningitis. Treatment decisions should
be dictated by the severity of neurological decline during the
emergency period and the presence/absence of associated intracranial
lesions. The timing for surgery and CSF drainage procedures must be
decided with great care and with a clear strategy.
Key words:
Liquorrhoea,
rhinorrhoea, cerebrospinal fluid, head injury.
Indexed in:-Index
Medicus / MEDLINE / PubMed-EMBASE, Excerpta Medica-SCOPUS-Indice
Médico Español-IBECS CONCEPt
Surgical
management of multiple traumatized patients with head trauma is
highly individualized and depends on a number of factors including
etiology, intracranial pressure,concomitant injuries, patient age and
the possibility of an interdisciplinary procedure. Severe head and
neck trauma are often connected with fractures of the frontal
skull base or nasoethmoido-orbital complex and cerebrospinal fluid
(CSF) leakage (1).
CSF leak is
an escape of the fluid that surrounds the brain and spinal cord, from
the cavities within the brain or central canal in the spinal cord. A
CSFrhinorrhoea occurs when there is a fistula between the dura and
the skull base and discharge of CSF from the nose. A spinal fluid
leak from the intracranial space to the nasal respiratory tract is
potentially very serious because of the risk of an ascending
infection which could produce fulminant meningitis (2). CSF leaks
have been associated with abouta 10% risk of developing meningitis
per year (3).
CSF rhinorrhea
commonly occurs following head trauma (fronto-basal skull
fractures) or as a result of intracranial surgery.
Others conditions
include paranasal sinuses along with osteomyelitis of the
adjacent bone, congenital anomalies of the brain and its
coverings such as meningoceles or meningoencephaloceles, and
destruction lesions along the skull base (4). Pituitary tumors cause
erosion of the sella turcica floor and are frequently associated with
CSFrhinorrhea (5).
This article
reviewed the causes, diagnosis and treatment of CSF leakage. A PUBMED
search of the National Library of Medicine was conducted.
EPIDEMIOLOGY CSF rhinorrhea can be divided in traumatic and
non-trau-matic: the traumatic group can be divided in accidental and
iatrogenic. The non-traumatic group is associated to braintumors
(intracranial and extracranial tumors, cholesteatoma, or tuberculoma
are know to erode the bone directly) (6), skull base congenital
defects and meningoceles or meningoencephalocles (7).
CSF leak most
commonly occurs following trauma (80-90% of cases) and the
majority of cases presenting within the first three months. Other
etiologies include: postoperative defect (10 %), spontaneous leak
(3-4 %), tumor, and inflammation (8). Usually the fracture
involves some portion of the anterior cranial fossa floor with
the leaks occurring through the cribriform plate or ethmoid sinus
roof into the nose.
Another frequently
seen anterior fossa fracture site is the posterior wall of the
frontal sinus through which CSF can escape into the nose via
the nasofrontal duct.
Less common are
middle cranial fossa fractures that can cause leakage to the nose
via the sphenoid sinus or eustachian tube (2).
Nontraumatic
cerebrospinal fluid fistulae tend to occur less frequently, and most
of them are related to diseases that cause increased intracranial
pressure or local skull destruction. Such conditions include
hydrocephalus, tumors, osteomyelitis of the skull and brain cysts.
Congenital
defects of the skull can also serve as the source of fistulae,
usually occurring in the anterior cranial fossa (2).
Fain et al. (9)
presented a classification of trauma to the cranial base, based on
observation in 80 cases. There were five types.
Type I: involves
only the anterior wall of the frontal sinus.
Type II: involves
the face (craniofacial disjunction of the Lefort II type or crush
face) and extend upward to the cranial base and, in occurrence, to
the anterior wall of the frontal sinus, because of the facial
retrusion.
Type III: involves
frontal part of the skull and extend down to the cranial base.
Type IV: is a
combination of types II and III.
Type V: involves
only ethmoidal or sphenoidal bones.
CSF leak is
unfrequent in types II, and transitionnal, if it occurs; but it often
occurs in types III, IV and V which include in every case a dural
tear. Correct diagnosis facilitates treatment. Fractures of types I
and II can be fully treated by maxillo-facial surgeons, whereas for
types III, IV, and V,
they need the help
of a neuro-surgeon.
CLINICAL PICTURE AND
PATHOGENESIS
CSF rhinorrhea after
intracranial or intranasal surgery is a known potential complication
with significant morbidity and mortality. Accurate identification of
the site of CSF leakage is necessary for a successful surgical
repair. The most reliable methods of distinguishing between a
traumatic
or neoplastic lesion
and a spontaneous CSF rhinorrhea are high-resolution computed
tomography (CT) and magnetic resonance (MR) tomography (10). MR
imaging is reserved for defining the nature of soft tissue i.e.
inflammatory tissue, meningoencephalocele or tumor (11). In MR images
we can find brain herniation into the ethmoid or frontal sinuses
(12). CT with or without intrathecal contrast and preoperative nasal
endoscopy are frequently used to preoperatively localize the site of
the leak (13).
CSF rhinorrhoea have
significantly greater incidence of periorbital haematoma. This
suggests that patients with head injuries and features of periorbital
haematoma are at greater risk of unobserved dural tear and delayed
CSF leakage. Frontal and ethmoid fractures in particular are also
associated with CSF leakage (14). Radiographic exams like simple
skull X rays are quite ineffective. However it can demonstrate
indirect signs like fractures and pneumoencephalus (7). Various
combinations of planar tomography and CT, contrast-enhanced CT
cisternography, and radionuclide cisternography, and, more recently,
MR cisternography have been used in the diagnosis of CSF leak.
Radionuclide
cisternography and
contrast-enhanced CT cisternography techniques require injections
into the intrathecal space, most often via lumbar puncture. Although
cisternography has minimal inherent risks, such as infection and
lumbar CSF leak, it significantly increases expense and adds
patient discomfort.
Radionuclide studies do not provide precise anatomic localization of
CSF leaks (15). Stone et al. (15) suggest that high-resolution CT is
a useful screening examination for the initial workup of CSF
rhinorrhea or otorrhea. When the clinical and imaging findings
coincide,
further evaluation
using CT cisternography and radionuclide cisternography is often
unnecessary. Computerized cisternography and radionuclide
cisternography should be used if MR imaging is contraindicated or if
a clinically and biologically proven CSF fistulae is not visualized
by CT or
MR imaging (8).
Diagnosis through nasal inspection and performance of laboratory
tests of the fluid can be conducted. In some cases, there is
contamination of the material with blood or
other secretions, so
the test with beta-2 transferrine becomes mandatory (7). Beta-2
transferrin is a carbohydrate-free (desialated) isoform of
transferrin, which is almost exclusively found in the CSF (16) and
blood or nasal secretion does not disturb the test (17). Beta-2
transferrin is not present in blood, nasal mucus, tears or mucosal
discharge.
This protein was
first described by Irjala et al in 1979 (18). Intense research
over the last decade has validated its characteristics and value in
clinical use as a specific CSF marker
(19). Beta-2
transferrin was reported to have a sensitivity of near 100% and a
specificity of about 95% in a large retrospective study (20).
Detection of glucose
in the sample fluid using Glucostix test strips has been a
traditional method for detection of the presence of CSF in nasal
and ear discharge. Glucose detection using Glucostix test strips
is not recommended as a confirmatory test due to its lack of
specificity and
sensitivity (19).
Interpretation of the results is confounded by various factors such
as contamination from glucose-containing fluid (tears, nasal
mucus, blood in nasal mucus) or
relatively low
CSF glucose levels (meningitis) (19). Studies have shown (21)
that glucose can be detected in airways secretions from people with
diabetes mellitus, stress hyperglycaemia and people with nasal
epithelial inflammation due to viral colds.
In the presence of a
skull base fracture on CT and a clinical CSF leak, there is no
need for a further confirmatory test.
In cases where a
confirmatory test is needed, the beta-2 transferrin assay is the test
of choice because of its high sensitivity and specificity (19).
MANAGEMEN AND
PROGNOSIS
Most of CSF leaks
close spontaneously within 7 to 10 days
(19, 22, 23).
Although most trauma-related CSF leaks resolve without intervention,
conservative treatment of CSF leaks may lead to bacterial meningitis,
therefore surgical closure of leaks or defects at the skull base
should be considered treatment of choice to prevent ascending
meningitis
(24). CSF fistulae
persisting for > 7 days had a significantly increased risk of
developing meningitis (23). The goal of surgical therapy is repair of
the dural defect contributing to the CSF leak (15). The surgical
management of CSF leak has changed significantly after the
introduction of functional endoscopic sinus surgery in the management
of sinusitis.
The clear
anatomical exposure of the roof of the nasal and the sinus cavities
by the endoscope offers the surgeon an opportunity to identify the
area of the CSF leak, which enables one to adequately plan the
treatment (25).
It is currently
accepted that endoscopic intranasal management of CSF rhinorrhea is
the preferred method of surgical repair, with higher success rates
and less morbidity than intracranial surgical repair in selected
cases (13). Endonasal endoscopic approach can be preferred for the
closure of uncomplicated CSF fistula, located at the
anterior or posterior ethmoid roof and in the sphenoid sinus,
due to its minimal postoperative morbidity. Uncomplicated CSF
fistula, located at the posterior wall of frontal sinuses can be
repaired extradurally with osteoplastic frontal sinusotomy.
Intracranial
approaches should be reserved for more complicated CSF rhinorrhea
which results from extensive comminuted fractures of the anterior
cranial base and is accompanied
with intracranial
complications (26). Anosmia is the most frequent permanent
complication mentioned.
The value of
antibiotic prophylaxis in patients with CSF leakage is debatable. In
a literature review, Brodie (27) concluded that individually, each of
the studies evaluated demonstrated no significant difference
in the incidence of meningitis with prophylactic antibiotic
therapy. The reason
for this is that
inadequate numbers of patients were available at each institution.
Pooling the data from the past 25 years revealed a statistically
significant reduction in the incidence of meningitis with
prophylactic antibiotic therapy. It is ethically justifiable to keep
antibiotic prophylaxis in patients with CSF fistulae until other
studies settle the question.
COMPLEMENTARY
EXPLORATION
Post-traumatic
CSF leaks are uncommon and will usually resolve without
surgical intervention. Successful management in refractory cases
often involves a combination of observation, CSF diversion, and/or
extracranial and intracranial procedures (28). The factors that had a
critical influence on outcome are level of consciousness on admission
and presence of additional intracranial pathology associated with CSF
leakage within cases of traumatic CSF fistulae
due to skull base
fractures. Patients with CSF leaks that persist greater than 24 hours
are at risk for meningitis, and maybe require surgical
intervention. Prophylactic antibiotics may be effective and should be
considered in this group of patients (29). Treatment decisions should
be dictated by the
severity of
neurological decline during the emergency period and the
presence/absence of associated intracranial lesions.
The timing for
surgery and CSF drainage procedures must be decided with great care
and with a clear strategy (22)
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