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Traumatic Cerebrospinal Fluid Leak: Diagnosis and Management

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Traumatic Cerebrospinal Fluid Leak: Diagnosis and Management

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pISSN 2234-8999 / eISSN 2288-2243

REVIEW ARTICLE
Korean J Neurotrauma 2017;13(2):63-67 https://doi.org/10.13004/kjnt.2017.13.2.63

Traumatic Cerebrospinal Fluid Leak: Diagnosis and Management


Ji-Woong Oh, So-Hyun Kim, and Kum Whang
Department of Neurosurgery, Wonju Severance Christian Hospital, Yonsei University, Wonju, Korea

Cerebrospinal fluid (CSF) leaks are one of the common complications after traumatic brain injuries (TBI). The risks of
CSF leaks can be detrimental to the outcomes of the patients. Early diagnosis and proper management is imperative for it
is strongly associated with a better long-term prognosis of the patients. Diagnostic tools for CSF leaks are still under de-
bate. Nevertheless, many reports of successful treatments for CSF leaks have been published with introduction of various
repair techniques for leakage sites even though it is surgically challenging. Hereby, we review about the pathophysiology,
manifestations as well as the update of the clinical diagnosis and current management of CSF leaks.
(Korean J Neurotrauma 2017;13(2):63-67)

KEY WORDS: Brain injuries ㆍ Cerebrospinal fluid leak ㆍMeningitis.

Introduction resolved.12) The traditional treatment involves intravenous


antibiotics treatment as well as primary repair of dural de-
Cerebrospinal fluid (CSF) is a physiologic fluid for pro- fect if the definite injury is suspected. Thus, early detection
tecting brain and maintaining intracranial pressure (ICP). of CSF leaks is important as it determines the outcome of
It is produced at choroid plexus and a total volume of 140 the patient. The decision of whether to observe or to surgi-
mL are actively circulating and turned over daily. After se- cally intervene is most likely to be dependent on the cause,
vere craniomaxillofacial trauma, the destruction of the site of leak, and timing of the leak.4,11)
meningeal structure may lead to the CSF leak from the sub-
arachnoid space. Post-traumatic CSF leaks are seen 1% to Clinical Pathophysiology
3% of all closed traumatic brain injuries (TBI) in adults of Traumatic CSF Leak
and 80% to 90% of all the causes of CSF leaks in adult pa-
tients are due to head injuries.22,28) The risk of meningitis Traumatic CSF leak is reported to be approximately 10%
from the traumatic CSF leak can present with high mor- to 30% of the skull base fractures in adults.28) More than half
bidity and even mortality depending on the cause and site of these CSF leak is presented within 48 hours of the trau-
of CSF leak. Except the cases with spontaneous diseases, ma while almost all CSF leaks occur within 3 months in de-
traumatic CSF leak can be potentially detrimental with vari- layed manner. The most common fracture sites leading to
ous complications such as bacterial meningitis if not self- CSF leaks following TBI are the frontal sinus (30.8%), sphe-
noid sinus (11.4-30.8%), ethmoid (15.4-19.1%), cribriform
Received: September 4, 2017 / Revised: September 29, 2017 plate (7.7%), frontoethmoid (7.7%) and sphenoethmoid
Accepted: October 8, 2017 (7.7%).3,20)
Address for correspondence: Kum Whang
Department of Neurosurgery, Wonju Severance Christian Hospital,
Yonsei University, 20 Ilsan-ro, Wonju 26426, Korea Blunt injury of anterior fossa
Tel: +82-33-741-0593, Fax: +82-33-746-2287 Fractures of anterior cranial fossa were more common
E-mail: [email protected]
cc This is an Open Access article distributed under the terms of Cre-
than temporal bone fractures. And dura of these sites, was
ative Attributions Non-Commercial License (http://creativecommons. very adherent to the anterior cranial fossa. Therefore, the
org/licenses/by-nc/4.0/) which permits unrestricted noncommercial use,
distribution, and reproduction in any medium, provided the original work
CSF rhinorrhea caused by anterior cranial fossa fractures,
is properly cited. was more common than caused by temporal bone fractures.

Copyright © 2017 Korean Neurotraumatology Society 63


CSF Leakage

And fractures of ethmoidal bone and junction between Delayed onset or recurrence
cribriform and ethmoid were most common cause of CSF The delayed onset group is defined as patients with CSF
rhinorrhea.2) Especially because the ethmoidal artery pen- leak presented at least 1 week after trauma. CSF leakage
etrates the lateral part of the cribriform, it is the most vul- may be delayed even if there is no CSF leakage at first. Usu-
nerable part of the trauma. In the situation of anterior cra- ally they can be healed spontaneous or lumbar drainage
nial fossa fractures caused by head trauma, bony defect may be required for further treatment. Even if the CSF leak-
size and degree of dural disruption, arachnoid tearing and age was healed, there were also possibility of delayed CSF
ICP were influenced the development of CSF rhinorrhea. leakage. There were two reasons of the delayed onset or re-
currence of CSF leakage: 1) blood clot disappears; 2) sep-
Blunt injury of middle and posterior fossa aration of dura and arachnoid caused by cerebral edema
Fractures that extend from the petrous bone to the middle subsides.15)
ear can cause otorrhea if the tympanic membrane is torn.
Otorhinorrhea can also occur along to the tract of Eusta- Very-Late Onset or Infection
chian tube. Temporal bone fractures are transverse type in CSF leakage may also occur after a considerable period of
10% to 30% and longitudinal type in 70% to 90%. Frequen- time, and even before CSF leakage occurs, such as rhinor-
cies of CSF fistula are constant regardless of each fracture rhea, infection may occur. The reason for this is that brain
type. And if a fracture extending from the greater wing of shrinkage caused by age may cause CSF leaking site reopen-
the sphenoid to the sphenoid sinus occurs, a rhinorrhea may ing, growth fracture on ethmoidal bone may lead to leakage
also occur, even though it is a middle fossa fracture. to fracture site, CSF leaking site has a barrier, which can’t
work as an infection barrier, could be considered.
Penetrating injury
Although, penetrating injuries are rare, CSF leakage is the Diagnosis and Investigation
common complication of penetrating injuries. When pen-
etrating injury was occurred, Aarabi1) and Meirowsky et Clinical presentation: symptoms and signs
al.16) reported that at the time of penetrating injury occurred, The most common clinical symptom is the leak of clear
CSF leakage occurred at a frequency of 8.7% and 8.9% re- and watery drainage from the nose and ear with a position-
spectively. And in the situation of CSF leakage caused by al dependency.14) If the patient is alert, a complaint of the
penetrating injury, infection rate was increased. Aarabi1) salty postnasal drip is presented. The clear and non-mu-
in a study of 379 patients with missile wounds in Iraqi war- coid fluid drainage from nose and ear can be presented with
fare, reported CSF leakage in 33 patients, of whom 12 (36%) mixed nature of bleeding, however, this can be further test-
had infection. ed for a ‘double-ring’ or ’halo’ sign on a filter paper. Fur-
thermore, other otolaryngeal diseases must be differentiat-
Time of CSF Leakage ed such as allergic rhinitis or vasomotor rhinitis prior to the
diagnosis of CSF leak. Patients may experience a salty taste
The timing of CSF leak is important that it will affect the or may have ear fullness or hearing loss. There may also
long-term prognosis of the patients with other complications be a ‘Reservoir sign’ in which the CSF goes out when tak-
such as infection. ing a head up position in the lying position. Most patients of
the CSF leakage complained of headache. The headache
Early Onset could be classified as a high pressure type and low pressure
The early onset group includes the patients with CSF leak type. High-pressure type is a symptom in which headache
within 48 hours from trauma.10,19,21,24) In most cases of blunt continues to increase and relived when CSF was drained
injury, CSF rhinorrhea usually begins within 48 hours, and out. Early detection of CSF leak will be critical for the pa-
if it is not large, 60% to 70% is naturally blocked.17) How- tient in order to prevent possible bacterial meningitis and
ever, in cases of large bone or dura defect, medical disease intracranial abscess formation.
such as diabetes mellitus and situation of increased ICP,
spontaneous healing of CSF leakage site had a high possi- Identifying of CSF leakage
bility of fail. They may be required imminent treatment in- Target sign: When the CSF is mixed with a blood or na-
cluding surgical repair. sal discharge, the CSF moves away on the filter paper, and
the blood moves closer, so two rings are visible. This is

64 Korean J Neurotrauma 2017;13(2):63-67


Ji-Woong Oh, et al.

called a target sign, a double ring sign, or a Halo sign imaging (MRI) with intrathecal contrast or cisternogra-
Handker chief test: When the discharge from the nose is phy.18,25,26)
buried in a handkerchief or dry gauze, the CSF is more like- High resolution CT: This method would give a detailed
ly to be clear if it is not sticky The Handker chief test is a test structural information on the bone details. It is perhaps the
to determine the nasal discharge, which is unclear and sticky best and fast method in viewing the skull base structures.
due to mucin secretion from the nose. The 3-dimensional structures of anterior and middle cra-
Glucose oxidized test: The CSF glucose from nasal or nial fossa in thin sections (usually 1-2 mm) are acquired
ear secretions has long been a classical method in testing and provide details on the fractures of those structures. It
CSF leak. In general, the glucose oxidase strips show pos- has a sensitivity of 89% due to high false positivity with ar-
itive result when the sample has a concentration over 20 tifacts. Nonetheless, it is known to be a good method in de-
mg/dL. Nasal discharge has a normal concentration of 10 ciding treatment plan rapidly. CT cisternography is rather
mg of glucose, thus, if the glucose test is negative then it an invasive method as lumbar puncture is required for the
can be ruled out. However, it is only to be used as reference study. The sensitivity of finding CSF leak with this method
as it has high false positive and negative rates depending is approximately 60% to 80%.13)
on the patients’ other medical conditions.6) Moreover, the MRI: MRI can be used to detect the CSF leak in multiple
lacrimal secretion can also be tested even if the concentra- imaging planes. Its accuracy with active CSF rhinorrhea is
tion is less than 5 mg/dL. Meanwhile, a false positive result about 90%.26) While the CT is useful in showing the bone
can be observed in the bloody nasal discharge whereas a details of fractures, MRI can provide details of the content
false negative results are seen if the meningitis is already of the CSF fistula or sacs of CSF content if necessary with
progressed in the patients. All these clinical conditions have contrast information. MRI is a useful tool to differentiate
to be considered before the interpretation and confirma- CSF leak and to diagnose arachnoid herniation through
tion of the CSF leaks. bone defect. CSF is observed as a high signal intensity in
β2 Transferrin: β-1 transferrin is found in serum tears, T2-weighted images while the peri-mucosal discharge is
nasal secretion and saliva ubiquitously while β-2-transferrin seen in low signal intensity where mucosal diseases also
is only observed in CSF, perilymph, and vitreous humor. come along with contrast enhancement.
Since the β-2 transferring is specific in CSF, it is a well-known
marker with extremely high sensitivity and specificity. Trace tests
However, it is also present in the vitreous humor, hence, when Due to the high risks of anaphylaxis, it is not common to
there is an eyeball rupture, CSF leaks can be false positive use radionuclide tracers. However, many surgeons use intra-
in the test. And it is also very expensive and takes longer thecal fluorescein by using endoscopic skills. Again this
time for the results to be reported. Therefore, in South Ko- is not yet acknowledged by the Food and Drug Adminis-
rea, it is not yet a common test to run in the clinical situations tration, in South Korea, thus, it is not used commonly yet.
Glucose and Chlorine Concentration: If the serum glu- In 2009, Banks et al.3) have reported a research in the use
cose level is 0.5 to 0.67, then there is a higher possibility of intrathecal fluorescein in the CSF leakage. The 0.1 mL
that there is a CSF leak. CSF glucose level is undoubtedly of 10% fluorescein is mixed in the 10 mL of the patient's
affected by the glucose levels in serum, therefore, it is im- CSF and it was injected intrathecally for 5 minutes. Then the
portant to consider the two parameters together when endoscopic study was carried out after 1 and 2 hours of in-
confirming the CSF leaks. Furthermore, if the chlorine jection. One out of 193 patients had a complication of pre-
concentration level is 100 mEq/L, then one must consider mature ventricular contraction, but the rest of patients showed
the situations with CSF leaks as well. no neurologic deficits.

Identification of CSF leakage site Management of CSF Leak


As the physical examinations are not always reliable, lab-
oratory tests are necessary to supplement the diagnosis. There are two major ways in treating CSF leak: conserva-
However, the radiologic findings are also important in iden- tive management or surgical repair. Surgical treatment will
tification of leaking point and decision making for the be subdivided into three methods: intracranial, extracra-
treatment. The radiologic evaluation will include plain films nial, and transnasal endoscopic method.
of skull and facial bones, high resolution computed tomog-
raphy (CT), CT cisternography, and magnetic resonance

http://www.kjnt.org 65
CSF Leakage

Meningitis management Intracranial repair of CSF leakage


Meningitis is seen in 19% of persistent CSF leakage with The indications of intracranial repair of CSF leakage are
10% of mortality.7) The delayed CSF leakage and the lon- as follows: 1) accompanied craniofacial injuries; 2) a large
ger duration of the leakage with concomitant infection bone defect which cannot be solved by the endoscopic re-
have a higher risk of meningitis. The most common patho- pair method only; and 3) in a situation where the leaking
gens of meningitis due to CSF leakage are Streptococcus fistula site is not obvious via endoscopic examination. In
pneumoniae and Hemophilus influenza. Meanwhile, there general, if the leakage site is involved in the anterior fossa,
is still a controversy in the use of prophylactic antibiotics then the anterior fossa craniotomy is carried out via bicor-
with these infections. Brodie5) reported that there is a risk onal incision while the subtemporal craniotomy is consid-
of meningitis about 2.5% and 10% with and without pro- ered in the CSF leakage of middle fossa. The advantage of
phylactic antibiotics, respectively. Yet, whether it is clini- intracranial approach is that the operation field is widely
cally significant or not, it is still a controversial issue.27) The exposed, hence, it is convenient to repair multiple defect of
most common antibiotics used are ceftriaxone and ampi- CSF leakage. Another advantage is that it is possible to re-
cillin/sulfadiazine, but there are no significant difference pair the leakage site even if the ICP is high due to severe
in the overall incidence rate of meningitis, according to the brain injury. On the other hand, the disadvantages include
type of antibiotics.9) anosmia, retraction-related brain injury and longer hospi-
tal stay. The open craniotomy procedures should consider
Conservative management the following key points during the repairment: 1) preser-
The indications of conservative management are the pa- vation of draining vein and olfactory nerve; 2) knowing
tients with linear fractures on facial bones. The patient ed- the first intradual sign which is an area of adherence of brain
ucation is included with head elevation with 30 degrees and arachnoid to the site of fistula; 3) if no fistula site is
without blowing nose, coughing or deliberate yawning or found gayer a careful exploration, then a thorough review
staining of stools. Absolute bed resting for at least 3 days of of radiologic studies is compulsory to look for other possi-
clinical observation will decide whether further treatment ble leakage sites (e.g. middle ear, posterior fossa etc.); 4) a
is required such as lumbar drainage or immediate surgical large bone graft is necessary with inner calvaria in the pres-
repair.8) One has to be cautious that the Over-drainage of ence of a large bone defect; 5) the temporalis fascia or fas-
CSF will result in intracranial aeroceles and brain displace- cia lata is placed intradually in the presence of dural tear-
ment with herniation followed by comatose mentality. It is ing; 6) the coverage of the entire anterior fossa floor with
usually common to drain the CSF in the rate of 10 to 15 mL fascia is not recommended in the avulsion of intact olfac-
per hour with a total drainage volume to be ranged in 150 tory nerve; and 7) lumbar drainage shunt is required if there
to 250 mL.8) is no other sites of CSF leakage or if there is only a small-
sized leakage.
Surgical management
The indications for early surgery is as follows: 1) penetrat- Endoscopic endonasal approach
ing injury; 2) intracranial hematoma; 3) meningitis; 4) large As there has been an advancement in the endoscopic tech-
intracranial aerocele; 5) herniation of brain tissue from niques, the endoscopic method is chosen as the first choice
nose and ear; and 6) low probability of natural dural repair. of repairing CSF leakage. However, it is important to have
The indications of delated surgery are as follows: 1) per- a good knowledge of the leakage site of fistula prior to the
sistent CSF leakage after 10 days of conservative manage- repair operation during the endoscopic endonasal approach.
ment; 2) recurrence of delayed CSF leakage after 10 days The major advantages of endoscopic endonasal repair are
of conservative management; 3) recurrent aerocels after a low risk of retraction injury of brain cortex and anosmia
10 days of conservative management; and 4) the presence and a relatively convenient approach to the sphenoid para-
of meningitis and abscess formation. Surgical methods are sellar and posterior ethmoid region. Banks et al.3) reported
classified into two groups. The first is a classical intracra- the overall success rate of 98% in the treatment of 193 pa-
nial approach and the second is extracranial approach. In tients with CSF rhinorrhea. And complications of endoscop-
the past, the transfacial extracranial approach was the main ic surgery are reported to be very low. According to the re-
method in extracranial approach. However, nowadays, it is port by Senior et al.23) in 2001 with the compilation of 522
more popular to go for the endonasal repair by endoscopic cases with retrospective questionnaires, the complication
approach. rate was 2.5% meanwhile the overall success rate was 90%

66 Korean J Neurotrauma 2017;13(2):63-67


Ji-Woong Oh, et al.

in the first attempt of endoscopic approach in a single insti- neurosurgical patient. Infect Dis Clin North Am 4:677-701, 1990
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