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Cerebrospinal Fluid Leak, Meningocele and Meningoencephalocele 95
In case of a cephalocele, signs and symptoms de­pend on its location. Transethmoidal cephaloceles herniate into the nasal cavity and may be character­ized by unilateral nasal obstruction; less frequently, this is the heralding symptom of transsphenoidal encephaloceles. Endoscopic evaluation may reveal a smooth isolated polypoid mass coming from the olfactory fossa (Fig. 7.1) or sphenoid sinus. In this setting, removal or biopsy of the lesion is contrain­dicated unless appropriate radiologic evaluation has been obtained. Fronto-ethmoidal and spheno-orbital cephaloceles herniate into the soft tissues of the nose region and of the orbit(s), respectively, and are char­acterized by swelling, proptosis, and hypertelorism. In these cases, an association with facial deformities and cleft palate is frequently observed (Mahapatra and Suri 2002).
In a patient with persistent CSF leak, rarely nasal endoscopy identifi es the site of CSF leakage. Watery rhinorrhea and mucosal bulging at the olfactory fossa is the most favorable situation suggesting a CSF leak coming from a cephalocele. An unexpected sinonasal neoplasm can also be detected by endoscopy.
Recurrent meningitis, even in the absence of rhi­norrhea, should raise a suspicion of CSF leak. The prevalence of meningitis and brain abscess is re­ported to be up to 40% in traumatic non-intermit­tent fi stulae, whereas their incidence greatly varies in spontaneous leaks (Beckhardt et al. 1991; Eljamel 1994; Wa x et al. 1997).
7.3 Diagnosis
Physicians can be faced with two main different scenarios: patients with persistent or intermittent watery rhinorrhea highly suggestive for CSF leak and patients who had one or more bouts of men­ingitis apparently without any specifi c cause. In the fi rst situation, which is the most frequent, diagnosis should include fi rst chemical methods to analyze the nasal discharge in order to obtain confi rmation of its nature, while in the second an immediate imag­ing examination and fl uorescein test are indicated (Fig. 7.2).
Fig. 7.1. Meningocele of the left nasal fossa. Endoscopic eval­uation with a 0°-angled rigid endoscope: an isolated bluish polypoid mass (P) projecting from the left olfactory fossa is visible. Nasal septum, NS; middle turbinate, MT
Fig. 7.2. Work-up in the case of suspected spontaneous or trau­matic CSF leak. High resolution CT (HRCT)
Chemical methods for glucose, protein, or chloride have been used for many years to confi rm CSF leak. However, they are nowadays considered highly non­specifi c (Oberascher and Arrer 1986) and beta-2 transferrin (B2T) assay has taken their place in the confi rmation of CSF rhinorrhea diagnosis. B2T is a polypeptide involved in ferrous ion transport: while beta-1 transferrin is present in serum, nasal secre­tions, tears, and saliva, B2T has been demonstrated in CSF, perilymph, and aqueous humor only. The re­ported sensitivity and specifi city for B2T test in the diagnosis of CSF rhinorrhea is 100% and 95%, re­spectively (Nandapalan et al. 1996; Skedros et al.
1993). Furthermore, the procedure is absolutely non­invasive, the amount of fl uid necessary for analysis is
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very small (0.5 ml), detection of B2T can be achieved within 3 h, and contamination by other body fl uids does not invalidate the method. Beta-trace protein (BTP) is another brain specifi c protein that is pro­duced mainly in the leptomeninges and the choroid plexus; it is the second most abundant protein in CSF after albumin. This protein has also been detected in other body fl uids such as serum and perilymph at much lower concentrations than in CSF. Recently, a BTP nephelometric assay for the quantifi cation and detection of CSF in nasal fl uid has shown sensitiv­ity of 91.17% and specifi city of 100% (Bachmann et al. 2000). Compared with B2T assay, BTP assay is less time-consuming . Therefore, B2T and BTP assays are nowadays considered the fi rst line test in confi rming the diagnosis of CSF rhinorrhea.
The recommended fl uorescein test protocol is based on the employment of 1 ml of 5% sodium fl uo­rescein solution diluted with 10 ml of patient’s CSF. The patient is put in Trendelenburg position and the solution is slowly injected intrathecally through a lumbar puncture (Stammberger 1991). Rare adverse effects of intrathecal injection of fl uorescein have been reported (temporary paresthesias of the lower limbs, weakness of the extremities, dizziness, dyspha­sia, hemiparesis and status epilepticus) (Moseley et al. 1978); however, the occurrence of these symptoms seems to be related to the use of higher concentrated solutions of the dye (Senior et al. 2001).
External approaches are still the mainstay in the treatment of frontoethmoidal, spheno-orbital and spheno-maxillary cephaloceles, while nasopharyn­geal lesions may be amenable with a microendo­scopic approach.
A large spectrum of materials can be used for du­raplasty: abdominal fat, septal mucoperichondrium, turbinate bone, temporalis muscle and fascia, ca­daver pericardium, lyophilized dura, fascia lata, and hydroxyapatite (Zweig et al. 2000).
Transnasal surgical repair involves mainly three techniques: underlay, overlay and tobacco pouch (Schick et al. 2001). The underlay technique is ideal for defects located in the fovea ethmoidalis (Fig. 7.3). Graft material (bone from the middle turbinate or cartilage from the septum) is positioned between the dura and the skull base or intracranially over the dura. A free mucoperichondral graft, harvested from the septum, is subsequently placed, as a second layer, on the endonasal surface of the skull base to reinforce the plasty.
7.4 Treatment Guidelines
Although most traumatic CSF fi stulas can spontane­ously heal with conservative measures such as bed rest, head supraelevation, administration of laxa­tives and antiemetics, and positioning of a lumbar drainage, a surgical corrective procedure is recom­mended in case these measures fail within 10–15 days (Hegazy et al. 2000).
Immediate surgical correction is instead indicated in the case of traumatic fi stulas associated with intra­cranial lesions requiring craniotomy and in all cases of iatrogenic leaks occurring during skull base sur­gery and sinus surgery (Hegazy et al. 2000).
Wi ga nd (1981) fi rst reported the endoscopic en­donasal approach for CSF leaks occurring during mi­croendoscopic sinus surgery for infl ammatory con­ditions. From this fi rst experience, microendoscopic repair gained popularity and is now considered the treatment of choice.
Fig. 7.3. Underlay technique. Coronal view of the anterior skull base showing the repair of a defect in the fovea ethmoidalis with “underlay technique.” Grafting material (autologous car­tilage from nasal septum or bone from middle turbinate) is positioned between the skull base and the dura. A mucosal graft from nasal septum or middle turbinate is placed as a second layer to reinforce the plasty
The overlay technique is generally employed for de- fects located in the lamina cribra, where the presence of olfactory phyla makes it diffi cult to dissect dura from the adjacent skull base (Fig. 7.4). A free cartilaginous or bony graft is fi rst placed on the extracranial sur­face of the skull base to close the bony gap; similarly to underlay technique, duraplasty is then completed
Cerebrospinal Fluid Leak, Meningocele and Meningoencephalocele 97
with a second layer of free mucoperichondrium. This technique can be also used in the lateral wall of exten­sively pneumatized sphenoid sinuses, where the leak is usually coming from a defect in the fl oor of the middle cranial fossa. In this setting, extensive drilling of the pterygoid process is required to ensure an adequate exposure (Casiano and Jassir 1999).
The tobacco pouch technique is an alternative pro­cedure for sphenoid sinus defects: after careful re­moval of the entire mucosa investing the sinus, fascia lata plus Gel foam or abdominal fat is positioned into the sinus and then sealed by a mucoperichondral graft (Weber et al. 1996; Schick et al. 2001) (Fig. 7.5).
A review of the literature indicates high success rates for microendoscopic procedures, varying from
75.9% to 97% after primary repair, up to 100% af­ter revision surgery. These results are independent
Fig. 7.5. Tobacco pouch technique. Axial view of the skull base showing the repair of a defect in the posterior wall of a sphenoid sinus with “tobacco pouch technique.” A double layer packing with fascia lata and abdominal fat or Gelfoam is positioned into the sinus
a
b
Fig. 7.4a,b. Overlay technique. a Coronal view of the anterior
skull base showing the repair of a defect in the olfactory fossa with “overlay technique.” Autologous cartilage or bone and mucosal graft are positioned endonasally under the dural defect. b Coronal view of the middle skull base showing the repair of a defect in the lateral wall of a hyperpneumatized sphenoid sinus with “overlay technique.” After drilling of the pterygoid process a double layer plasty is positioned
from the surgical technique and grafting materials (Dodson et al. 1994; Burns et al. 1996; Lanza et al. 1996; Castillo et al. 1999; Marshall et al. 1999; Nachtigal et al. 1999; Mao et al. 2000; Zweig et al. 2000; Schick et al. 2001).
Important clues for a successful repair are repre­sented by: precise location and exposure of the de­fect with adequate removal of the surrounding mu­cosa, removal of unstable bony fragments around the breech, and use of an oversized graft (Gassner et al. 1999; Castelnuovo et al. 2001; Schick et al. 2001).
An elevated body mass index, a fi nding frequently associated with spontaneous CSF leak (Schlosser and Bolger 2003), has also been recently shown to be related with failure of repair (Lindstrom et al.
2004).
Although some authors consider perioperative antibiotic therapy unnecessary, or even contrain­dicated, for potential positive selection of resistant bacteria, it is generally recommended for at least 48 h after surgery to decrease the incidence of postopera­tive meningitis (Choi and Spann 1996; Nachtigal et al. 1999; Hegazy et al. 2000; Zweig et al. 2000).
Some authors recommend the use of a lumbar drainage and its maintenance for at least 72 h af­ter surgery (Mccormack et al. 1990; Persky et al. 1991; Mao et al. 2000). According to more recent and numerous series, its use should be limited to those patients who have an associated hydrocephalus and/ or intracranial hypertension (Dodson et al. 1994;
Casiano and Jassir 1999; Hegazy et al. 2000).
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The success rate of the microendoscopic approach (superior to external techniques) and the low inci­dence of postoperative complications make it the treatment of choice. External approaches are nowa­days justifi ed in the case of repeated failures of mi­croendoscopic techniques, multifocal fronto-basal fractures, association with brain lesions requiring craniotomy, and fi stulae not endoscopically treatable (posterior wall of the frontal sinus).
7.4.1 Follow-Up
Although most CSF leak recurrences generally occur within days or months after surgical repair, late recur­rences have been described even after years. Periodic endoscopic evaluations are therefore mandatory, while BTP or fl uorescein tests and imaging techniques are indicated in the case of suspicious symptoms such as persistent rhinorrhea and meningitis. Patients with intracranial hypertension should be considered at risk for a recurrent lesion or a second meningocele at a different site; periodic MR may therefore be indicated for early detection of this occurrence.
directly demonstrating the leakage, as evidence of its existence, to different – though not less challenging – tasks.
As a direct consequence, there is less need to use invasive “cisternographic” imaging techniques, which have the purpose of tracking the fi stulous tract by means of radiopaque or radioactive “markers” previ­ously injected into the CSF. Therefore, nowadays, the identifi cation of intra- and extracranial lesions possi­bly causing CSF leak and the detailed demonstration of the anatomic/topographic features of the fi stula(e) are the main objectives of imaging.
This information may be achieved by “plain” non- invasive imaging techniques, aimed at identifying lesions involving the skull base or to detect osseous/ dural defects. Interestingly, MR – the more recently introduced imaging technique – enables the identi­fi cation of the fi stula by means of special sequences that enhance the CSF signal (MR cisternography), therefore providing fi ndings similar to those obtained by invasive “cisternographic” techniques.
7.6.1 Integration of Imaging into the Diagnostic Work-Up
7.5 Key Information to Be Provided by Imaging
Presence of intra- and extracranial pathology
possibly related to the CSF fi stula (empty sella, paranasal sinuses diseases, skull or head and neck tumors or infections) and/or contraindicat­ing lumbar puncture possibly necessary for fl uo­rescein or contrast-medium injection (acute post­traumatic lesions, hydrocephalus, brain tumors)
Radiological confi rmation of the CSF leakSide, site, size and possible number of the
fi stula(e)
“Nature” of the lesion primarily causing CSF rhi-
norrhea (bony dehiscence, meningocele, menin­goencephalocele)
7.6 Imaging Findings
The development of highly accurate chemical meth­ods to confi rm CSF leak has recently shifted the use of imaging from its traditional primary purpose of
The use of imaging – as for the employment of either fl uorescein test or BTP test – is dictated by the clinical presentation of CSF leak that can be separated into persistent or intermittent rhinorrhea vs. recurrent meningitis (Fig. 7.2)
7.6.1.1 CSF Leak Associated with Persistent or Intermittent Rhinorrhea
When persistent or intermittent watery rhinorrhea suggests CSF leak, endoscopy is the fi rst line exami­nation. An endonasal expansile lesion (cephalocele? glioma?) may be detected or endoscopy may give a negative result. In the fi rst case, BTP test is un­necessary, as CSF leak is assumed to be related to the endonasal lesion. Additionally, having detected a nasal mass, the main clinical issue is to defi ning its relationship with intracranial structures. In this set­ting, MR is preferable to high resolution CT. In fact, T2 and MR cisternographic sequences are superior in demonstrating the uninterrupted CSF signal ex­tending from the subarachnoid space into the mass, or into paranasal sinuses/nasal cavity or into middle ear, with or without brain tissue (Stafford et al.
1996) (Fig. 7.6).
Cerebrospinal Fluid Leak, Meningocele and Meningoencephalocele 99
a
Fig. 7.6a,b. Small spontaneous meningocele through the right cribriform plate. Coronal TSE T2 before (a) and after (b) surgi-
cal repair. a A small rounded fl uid collection (proved to be a meningocele at surgery) is seen under the right cribriform plate (asterisk); both olfactory bulbs are clearly visible over the cribriform plate in this patient with a “deep” olfactory fossa (the left one indicated by arrows). Subtle mucosal thickening in both maxillary sinuses, with retention cyst on the right side. b After successful endoscopic repair with overlay technique, the CSF collection in the right olfactory cleft is no longer visible. Note the post-surgical changes on the right side due to middle turbinectomy, uncinectomy, middle antrostomy, and ethmoidotomy
CT has a complementary role, related to its supe­rior depiction of bony detail. At MR, the cephalocele appears as a round “mass” with sharp margins, isoin­tense to CSF in all sequences (meningocele) or con­taining some tissue isointense to brain parenchyma (meningoencephalocele), protruding from the in­tracranial cavity into the nasal cavity or paranasal sinuses. The lumen of the lesion is continuous with the subarachnoid space and often has a constriction in the portion passing through the bone defect (the “neck” of the cephalocele) (Fig. 7.7). CT clearly shows the sclerotic margins of the bone defect. Cerebral MR may show the associated brain anatomy “distortion”, presumably due to the effect of the brain pulsation in utero, which “pushed” the pliable unmyelinated brain outward through the defect (Truw it et al. 1996). The result is a general tendency of the ventricles and sub­arachnoid spaces, which subtend the cephalocele, to be stretched and elongated, “pointing” toward the calvarial defect. This fi nding is usually observed in patients with large occipital or parietal cephaloceles.
Cephaloceles may be isolated anomalies, but may also be seen in conjunction with other congenital brain malformations or as a part of a syndrome. The radiologist must therefore also look for associated anomalies, such as agenesis or hypogenesis of the
corpus callosum, Dandy-Walker malformation, or malformations of cortical development (Naidich et al. 1992).
Among lesions to be differentiated from cephalo­celes is the so-called nasal glioma (heterotopic brain tissue). It consists of a variable amount of dysplastic brain tissue (especially glia cells, whereas neurons are present in only about 10% of cases), located within the nasal cavity or in the nasal subcutaneous tissue. It is thought to result from the herniation of brain tissue into a dural projection that normally extends through the foramen cecum during the embryologic develop­ment. Regression of the more superior portion of this dura projection leads to complete separation of the nasal glioma from the intracranial contents. If infe­riorly the dural projection remains adherent to the skin of the nose and fails to involute, but no brain tissue herniates through it; a dermal sinus tract is present. It is usually suggested by a small dimple on the surface of the nose. Along the path of the dermal sinus tract (epi)dermoids may develop.
MR is clearly superior to CT in the evaluation of nasal gliomas, because it directly shows the lack of communication with the subarachnoid space, which is the hallmark of cephaloceles. Furthermore, the dysplastic brain tissue of nasal glioma appears hyper-
b
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a
c
intense to gray matter in both T1- and T2-weighted images (Fig. 7.8).
When no endonasal mass is detected by endos­copy, BTP test is indicated, its sensitivity for CSF leak being more than 90% (Bachmann et al. 2000).
If post-traumatic rhinorrhea is investigated, positivity of BTP will confi rm the diagnosis. In this setting, high resolution CT is the fi rst line imaging technique, having a reported sensitivity ranging from 50% up to 100% (Dietrich et al. 1993; Lloyd et al. 1994, Zapalac 2002). It is obtained without intra­thecal or intravenous contrast agent administration, using thin (1- or 2-mm) contiguous axial and coro­nal planes. Scans or multiplanar reconstructions are orientated parallel and perpendicular to the anterior cranial fossa fl oor, respectively. The whole skull base needs to be examined, including the mastoid cells.
Because CSF leak is not directly detectable on high-resolution CT, the identifi cation of a bony de­fect is indirectly taken as the possible site of the CSF leak, even if the bone defect itself does not necessar­ily correspond to the site of dural tear (To ll e y et al. 1992). However, bony dehiscences are not infre­quent in the skull base (Tol l ey et al. 1991), having
b
Fig. 7.7a−c. Fronto-ethmoidal spontaneous meningocele in an
8-year-old girl. Coronal high resolution CT (a), TSE T2 in the coronal (b) and sagittal (c) planes. a Bone defect in the left cribriform plate (arrow), associated with soft-tissue density in the left nasal cavity (asterisk). b The outpouching of CSF in the left nasal cavity is clearly identifi ed as fl uid signal (hy­perintense) on TSE T2 images (asterisk). Even if the signal intensity of CSF is slightly brighter than that of nasal mucosa, an infl ammatory polyp could be a differential diagnosis. c On sagittal image, the direct communication between the fl uid collection in the nasal cavity and the frontal subarachnoid space is clearly depicted (arrow). There is no brain paren­chyma extending into the endonasal CSF collection
been demonstrated in about 14% of ethmoid bones (Ohnishi 1981). Similar dehiscences have been de­scribed in the sphenoid and, less commonly, in the frontal sinus. As a consequence, a simple bony defect cannot be considered a reliable sign of CSF fi stula. Conversely, when a bony defect, located at the edge between skull base and paranasal sinus/nasal cavity, is associated with fl uid collection and/or mucosal thickening within the adjacent sinus/nasal cavity, it can be assumed to be located close to the dural breach site (Fig. 7.9). By combining these fi ndings, the speci­fi city of high-resolution CT raised to 86% in a series of 15 patients with traumatic (accident or iatrogenic) fi stulae (Lloyd et al. 1994).
However, precise location of the fi stula(e) may be rather diffi cult, if not impossible, in the presence of comminuted fractures, particularly when scar tissue partially replaces the skull base or invests its endona­sal interrupted surface. In this case, CT fi ndings may indicate more than a single potential site of CSF leak, therefore not enabling a suffi ciently tailored surgical approach. MR may provide additional fi ndings, like showing signal intensity consistent with CSF within the scar tissue or across the interrupted skull base.
Cerebrospinal Fluid Leak, Meningocele and Meningoencephalocele 101
a
shows intermediate signal. e On off-midline sagittal plane, the nasal glioma (asterisk) appears bordered superiorly by the lateral aspect of the crista galli (black arrows). A normal nasofrontal suture is present (white arrow)
d
cb
ed
Fig. 7.8a−e. Intranasal heterotopic brain
tissue (nasal glioma). Coronal (a−c) and sagittal (d−e) TSE T2. a The mass (aster- isk) arises from left olfactory fi ssure, the left nasal cavity is completely occupied. b The nasal glioma abuts the inferior sur­face of the cribriform plate (1) without any connection with subarachnoid space. Middle (2) and inferior (3) turbinates are compressed. c The vertical lamina of the middle turbinate limits the lateral aspect of the nasal glioma. d On midline sagit­tal plane, the crista galli (black arrows)
In particular, MR cisternography has been proposed as a non-invasive cisternographic technique. It con­sists of turbo spin echo sequences with fat-suppres­sion (to null signal from bone marrow) that greatly increase CSF signal intensity and suppress the back­ground, providing heavily T2 images with detailed anatomic information of the subarachnoid cisterns. If necessary, a small number of images (usually be­tween two and eight) may be compressed into com­posite images using maximum intensity projection algorithms. Composite images may be useful to de­tect fi stulae with irregular and tortuous tracks (El Gammal and Brooks. 1994). In detecting CSF fi s­tulae, MR cisternography yielded sensitivity of 87%, specifi city ranging from 57% up to 100%, and accu­racy of 78%–89% (El Gammal et al. 1998, Shetty et al. 1998). Although MR cisternography may be added
Figure 7.9. Traumatic iatrogenic CSF fi stula, post-microendo­scopic sinus surgery. Coronal high resolution CT at the level of the olfactory fossa. A large bone defect is demonstrated to involve the horizontal (short arrows) and the lateral lamella (arrowhead) of the right cribriform plate. The right lamina papyracea is not recognizable (long arrows). Note the absence of the middle turbinate and ethmoid labyrinth on the right side, due to previous endonasal surgery
to the diagnostic work-up, at present precise identifi ­cation of the fi stula(e) is made possible at surgery by fl uorescein leakage detection, which is more accurate than imaging techniques.
In fact, even fi stulae due to incomplete damage of the meninges causing permeation rather than a com­plete breech are shown with this technique. The fl uo-
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roscein tracer is administered via lumbar puncture, and a special light fi lter is necessary to endoscopically detect small fi stulae. In case of spontaneous fi stula, a positive BTP test requires the patient to be inves­tigated either by CT, adding the high resolution CT technique to the standard examination of the head, and by MR. Detection of paranasal sinuses or tem­poral bone hyperpneumatization will prompt the accurate assessment of the whole skull base to rule out bone dehiscences. The presence of an empty sella with or without a fluid-like broad-based le­sion on sphenoid walls may arouse the suspicion of benign intracranial hypertension. In this set­ting, intermittent CSF leak may be associated with other imaging findings suggesting idiopathic in­tracranial hypertension, as increased CSF amount surrounding the optic nerves along their intraor­bital course (Suzuki et al. 2001). Furthermore, any fluid-like broad-based lesion hanging on the ex­tracranial surface of the skull base may indicate a cephalocele located beyond the limit of diagnostic endoscopy (Stone et al. 1999).
However, the presence of a high signal on T2 im­ages within paranasal sinuses or mastoid cells or adjacent to the skull base may be due to thickened mucosa, mastoiditis or rhinosinusitis, and not nec­essarily to CSF accumulation. Therefore, a CSF fis­tula may be suspected whenever the high signal of the fistulous tract appears to be in direct continu­ity with the intracranial subarachnoid space. This point is relevant, as high signal intensities on T2 sequences are shown in up to 25% of patients ex­amined by MR for non-sinonasal diseases (Moser et al. 1991).
Indirect evidence of CSF fi stula is provided by a low-lying gyrus rectus when the leak is located at the cribriform plate area (“gyrus rectus sign”), probably related to the negative pressure created by the fi stula (Shetty et al. 1998).
Finally, unexpected intracranial or extracranial tumors, hydrocephalus, or skull base infl ammatory lesions may be demonstrated by CT or MR.
When BTP test, high resolution CT, and MR are all negative, the diagnosis of CSF has to be considered unlikely or spontaneous healing of the dural defect(s) might have occurred during the diagnostic work-up.
In a series of 42 spontaneous and post-traumatic CSF leaks examined by high-resolution CT, radionu­clide cisternography, and CT cisternography, sponta­neous resolution of CSF leakage within 1 month from imaging studies was observed in all patients negative at high resolution CT (29%) (Stone et al. 1999).
7.6.1.2 CSF Leak Presenting with Recurrent Meningitis
In this second group of patients, CSF leak is sus­pected because of recurrent meningitis in the ab­sence of rhinorrhea. In this setting, the fi rst line ap­proach entails both endoscopy and imaging. BTP is, of course, unhelpful.
Endoscopy, CT and/or MR may succeed in dem­onstrating lesions both in post-traumatic and spon­taneous fi stulae. Compared to patients with CSF leak and rhinorrhea, the main difference consists in the absence of imaging fi ndings of active leak (i.e., fl uid collection within sinonasal cavities or focal mucosal thickening adjacent to bone dehiscences or to skull­base fractures).
Being based on indirect fi ndings, high-resolu­tion CT may be positive in inactive and active leaks with similar rates because it does not depend on the amount of CSF leakage at the time of investigation (Fig. 7.10).
Recently, a three-dimensional (3D), heavily T2­weighted sequence (3D CISS, constructive interfer­ence in steady state), has been proposed for CSF fi stula detection (Jayakumar et al. 2001). 3D-CISS, being heavily T2-weighted with better CSF-brain­bone-air contrast, and allowing thinner (sub-mil­limeter) sections and multiplanar reconstructions, seems to be ideally suited for the demonstration of CSF leak (Fig. 7.11). Its very short TE enables a mini­mization of signal loss from magnetic susceptibility effects at the air–bone interface. The main advantage of this sequence is its ability to reduce artifacts from CSF pulsations. Unfortunately, in CISS sequence fat tissue appears moderately bright, so that anatomi­cal details at the skull base/CSF interface may be ob­scured. Jayakumar et al. (2001) reported a sensitivity of 100% in a series of six patients: larger series have to be provided to establish the role of this promising technique.
If both endoscopy and non-invasive imaging are negative, the fl uorescein test is considered more use­ful than invasive cisternographic techniques, because their sensitivity is insuffi cient in patients with inac­tive leak.
In the case of a positive fl uorescein test, endoscopy may or may not precisely identify the fi stula, as the defect can be located beyond diagnostic endoscopy limits. Microendoscopic surgery with fl uorescein in­jection is indicated.
A negative fl uorescein test makes the diagnosis of CSF leak unlikely.
Cerebrospinal Fluid Leak, Meningocele and Meningoencephalocele 103
a b
Fig. 7.10a,b. Traumatic (due to car accident) CSF fi stula. Coronal high resolution CT (a), with reformatted sagittal section (b).
On the left side, the posterior aspect of the fovea ethmoidalis is not visible (arrows): a soft tissue density is clearly demonstrated (arrowhead) strictly adjacent to the large bone defect
a
c
Fig. 7.11a−e. Small traumatic (due to
car accident) meningocele through the right cribriform plate. Coronal TSE T2
d
e
the CSF possibly present in the ethmoidal cells from the thickened mucosa and/or sinus secretions. Only the left olfactory bulb is seen (arrow). b On coronal CISS sequence – 0.7 mm of thickness, obtained at the same level as (a) − the small meningocele (white arrows) shows the same signal as intracranial CSF, brighter than mucosal thickening. The contralateral olfactory bulb is demonstrated (black arrow). c−e The CISS sagittal reformatted sections show the small meningocele “neck” (arrows)
(a); coronal (b) and sagittal (c−e) images from a CISS sequence. a The hyperin­tense signal of CSF appears to continue with the hyperintense signal within the right ethmoid cells, through a presumed bone defect in the right cribriform plate, raising the suspicion of a meningocele. However, it is impossible to distinguish
b
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7.6.2 Invasive Imaging Techniques
In the past, several invasive “cisternographic” nuclear medicine and radiologic techniques have been devel­oped to confi rm and locate the site of CSF leaks. Since “cisternographic techniques” rely on demonstrating the passage of some “marked” CSF through the bone/ dural defect, their sensitivity strictly depends on the amount of CSF crossing the defect at the time of the examination. Nowadays they are indicated in very few cases.
Among invasive techniques, radionuclide cis- ternography has been the fi rst to successfully de­tect the site of CSF rhinorrhea (Crow et al. 1956). Unfortunately, most CSF leaks are intermittent, caus­ing its accuracy to reduce from approximately 70% in patients with active leaks to 28% in patients with inactive leaks (Eljamel et al. 1994).
CT cisternography has been considered one of the most reliable techniques in CSF fistula detec­tion, although the rate of positive studies has been quite variable in large reported series, ranging from 36%–40% in inactive leaks, and up to 81%–92% in active leaks (Manelfe et al. 1982; Colquhoun 1993; Eljamel et al. 1994). It requires intrathe-
cal injection of contrast agent (usually via lumbar puncture). A few minutes after contrast agent in­jection, thin CT slices (1–3 mm) are obtained, with both soft tissue and bone algorithms, preferably in the coronal plane (Drayer et al. 1977). Not to miss any potential CSF leakage site, scans should extend from the anterior frontal sinus wall to the poste­rior surface of the petrous bone. In fact, CSF rhi­norrhea due to leakage through the petrous bone into the middle ear and then, via Eustachian tube, into the nasopharynx – where the CSF may emerge via the nasal cavity – has been reported (Shetty et al. 1997). CT cisternography may directly dem­onstrate the contrast agent passage through bony and dural defects and/or the pooling of the con­trast agent within nasal cavity or paranasal sinuses (Fig. 7.12).
Positive fi ndings consist of direct demonstration of contrast agent passage through the bone/dural defect or of bone defect(s) associated with contrast agent pooling within ipsilateral sinus. Equivocal fi ndings entail detection of bone defect(s) and/or the presence of contrast agent within the ipsilateral nasal pledget. Negative fi ndings require no extracranial contrast agent or bone defect demonstrated (Manelfe et al.
1982).
a
Fig. 7.12a,b. Multiple traumatic CSF fi stulae due to fractures of the anterior skull base fl oor. CT cisternography, coronal sec-
tions (a,b). a Contrast agent fl ows from the right frontobasal subarachnoid space into the ethmoid cells through the right fovea ethmoidalis fracture (black arrows). The superfi cial lining of the mucosa investing nasal structures is hyperdense (arrowheads) due to layering and absorption of contrast agent leaking from the fi stula. On the left side, a thicker collection of contrast agent fi lls a cleft in the middle turbinate (white arrow). These fi ndings prompt a careful examination of the CT scan set, looking for other CSF fi stulae. b A more posterior coronal section discloses a fovea ethmoidalis fracture on the left side (arrows), with leak of a small amount of contrast agent into the ethmoidal cells
b