You are in: eMedicine Specialties > Radiology > BRAIN/SPINE Cerebrospinal Fluid, LeakArticle Last Updated: Feb 7, 2007AUTHOR AND EDITOR INFORMATIONAuthor: Hugh J Robertson, MD, DMR, FRCPC, FRCR, FACR, Professor Emeritus, Department of Radiology, Section of Neuroradiology, Louisiana State University School of Medicine; Clinical Professor, Department of Radiology, Tulane University School of Medicine, Consulting Staff, Department of Radiology, University Hospital Hugh J Robertson is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Neuroradiology, Louisiana State Medical Society, Radiological Society of North America, Royal College of Physicians and Surgeons of Canada, Royal College of Radiologists, and Royal Society of Medicine Coauthor(s): Michael D'Antonio, MD, Associate Professor of Clinical Radiology, Department of Radiology, Section of Neuroradiology, Louisiana State University Health Sciences Center in New Orleans Editors: Lucien M Levy, MD, PhD, Director of Neuroradiology, Professor of Radiology, Department of Radiology, George Washington University Medical Center; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; C Douglas Phillips, MD, Professor, Departments of Radiology, Neurosurgery, and Otolaryngology, University of Virginia Health Sciences Center; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; L Gill Naul, MD, Professor and Head, Department of Radiology, Texas A&M University College of Medicine; Chair, Department of Radiology, Chief, Section of Magnetic Resonance Imaging, Scott and White Memorial Hospital and Clinic Author and Editor Disclosure Synonyms and related keywords: CSF leak, dural tear, dural leak, CSF rhinorrhea, CSF otorrhea, pneumocephalus, spinal CSF leak, intracranial hypotension, spontaneous intracranial hypotension syndrome, SIHS, traumatic CSF fistula, double-ring sign, lumbar extradural blood patch INTRODUCTIONBackgroundCerebrospinal fluid (CSF) leak may occur from the nose (rhinorrhea), from the external auditory canal (otorrhea), or from a traumatic or operative defect in the skull or spine. The fluid leak is a result of meningeal dural and arachnoid laceration with fistula formation. Blunt trauma is the most common cause. PathophysiologyNormal adult subarachnoid fluid has a circulating volume of 90-150 mL. Approximately 500 mL of CSF is produced daily, primarily from the ventricular choroid plexuses. Circulating CSF is absorbed into the venous circulation, mainly through the cranial arachnoid granulations and spinal arachnoid villi. Normal CSF pressure is 100-200 mm of water. The normal CSF protein content is 20-45 mg/dL, and the normal CSF glucose range is 50-100 mg/dL, which is 60% of the measured serum glucose value. However, nasal mucous secretions and tears also have detectable glucose content. Therefore, tests used to identify CSF by its glucose content are often false positive (in 45-75% of cases). The absence of glucose tends to exclude CSF as the leaking fluid. The enzyme beta-2 transferrin is present in CSF and perilymph but not sinonasal mucous secretions and tears. This feature is the basis for a specific test for CSF based on immunoelectrophoresis. CSF rhinorrhea Causes of CSF rhinorrhea include (1) blunt head trauma; (2) sequelae of skull-base surgery, commonly functional endoscopic sinus surgery (FESS), transphenoidal pituitary surgery, translabyrinthine acoustic schwannoma, and mastoid surgery with intact tympanic membrane; (3) destructive skull-base lesions, including neoplasms (both benign and malignant), and empty sella; (4) developmental defects of the ethmoid, sphenoid, frontal, or petrous temporal bones with the formation of a meningocele or meningoencephalocele (with an intact tympanic membrane); and (5) fracture of the petrous temporal bone or other destructive processes in which CSF in the middle ear drains to the nose in the presence of an intact tympanic membrane. Less than 5% of all cases of CSF rhinorrhea are spontaneous. Most cases of CSF rhinorrhea begin soon after a head injury and cease spontaneously within 7-180 days. CSF otorrhea CSF otorrhea occurs in the presence of a perforated tympanic membrane in the following settings: (1) fractures of the petrous temporal bone, (2) translabyrinthine and/or mastoid surgery, (3) developmental defects of the tegmen tympani or petrous apex with meningocele formation and spontaneous or posttraumatic meningeal laceration, (4) perilymphatic fistula from trauma with stapes fracture and torn round or oval window membrane, (5) translabyrinthine fistula due to the Mondini developmental defect of the cochlear modiolus and/or lamina cribrosa, and (6) wide cochlear aqueduct syndrome. With a translabyrinthine fistula CSF mixes with perilymph in the cochlea or vestibule and forms perilymphatic hydrops with displacement or perforation of the maldeveloped stapes footplate; the fluid leaks into the middle ear. Wide cochlear aqueduct syndrome is a controversial and doubtful entity in adult patients because the aqueduct is filled with fibrous tissue and not functional beyond childhood. Pneumocephalus Pneumocephalus can occur in up to one third of all patients with posttraumatic or spontaneous CSF leak. This condition is likely the result of the pressure gradient created during respiration, sneezing, or nose blowing. Spinal CSF leak Spinal CSF leaks can occur as a result of (1) blunt or penetrating trauma; (2) postoperative sequela with leakage through a dural tear or incision; (3) lumbar puncture; (4) inadvertent meningeal puncture during epidural anesthesia; (5) spontaneous leakage from 1 or more spinal nerve root sleeves, particularly in the thoracic and lumbar areas; and (6) Valsalva maneuver during excessive weight lifting. Increased intracranial pressure facilitates the development of CSF leaks. Meningeal dysplasia (as in Marfan syndrome) may also contribute to the development of CSF leak in some patients. Spontaneous intracranial hypotension syndrome Spontaneous intracranial hypotension syndrome (SIHS) can result from a persistent CSF leak. SIHS is usually spinal and seldom originates from the skull base (eg, ethmoidal defects). Frequently, SIHS and persistent orthostatic headache after lumbar punctures can be successfully treated by lumbar epidural blood patch. FrequencyUnited StatesCSF rhinorrhea occurs in 2-6% of patients with head injury. Rhinorrhea or otorrhea occur in up to 30% of patients with a skull-base fracture. Head trauma accounts for 50-80% of all cases of CSF leak, and up to 16% are iatrogenic. Postoperative CSF leak has been noted in 0.5-15% of patients with transphenoidal surgery, particularly after reparative operations. CSF leak has been reported in 5-12.5% of translabyrinthine acoustic schwannoma surgeries. FESS is a common procedure, with CSF leak occurring in 1.0-2.5%, but 90% of leaks are detected and repaired intraoperatively. About 4% of CSF leaks are of spontaneous and nontraumatic causes (eg, developmental skull-based defects with meningocele, skull-base tumor, empty sella, osteomyelitis). Mortality/MorbidityMeningitis occurs in 25-50% of untreated traumatic CSF fistulas and in 10% of patients in the first week after trauma with head injury. The incidence of meningitis up to several years after spontaneous cessation of posttraumatic CSF leak is 10%. Meningitis-related mortality rates up to 20% have been reported, particularly when meningitis is due to antibiotic-resistant organisms.
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AnatomyCSF leak resulting from trauma occurs usually with fractures of ethmoid, sphenoid, or petrous temporal bones. The ethmoid bones are particularly vulnerable to trauma. The orbital plates of the frontal bone do not cover the ethmoid bones completely; therefore, the thin and perforated cribriform plates are partially unprotected. The dura is thinnest at and adherent to the cribriform plates and adjacent ethmoid sinus medial segments. The anterior ethmoidal arteries course in grooves on the surface of the ethmoid bones. In addition, multiple developmental defects occasionally occur in the sphenoid bone and in the floor of the middle cranial fossa. Because of these vascular grooves in the ethmoid bones and cribriform plates and because multiple anatomic defects are frequently present, it is sometimes difficult to demonstrate a fracture or developmental defect of the ethmoid bone in association with a CSF fistula. Clinical DetailsCSF leak after trauma CSF leak from the fistula occurring after head trauma consists of watery, bloodstained fluid that abruptly leaks from 1 or both nostrils or an external auditory canal. Two thirds of patients with these fistulas present within 48 hours of their head injury. Almost all of these fistulas occur within 3 months of injury. Occasionally, the fistula appears many months or years after injury, with a sudden gush of fluid or meningitis; these episodes are sometimes recurrent. CSF leak occurs often without nasal congestion, sneezing, lacrimation, or aural discharge. Rhinorrhea may occur intermittently and can increase on bending forward, with a Valsalva maneuver or jugular vein compression. Nasal vasoconstrictor or antihistamine therapy does not affect the leak. Headache is sometimes but not always present. The patient may have physical signs of skull-base fracture, including periorbital ecchymosis or edema, mastoid-area skin ecchymoses, and cranial nerve deficits. Frontal trauma may result in anosmia from an injury to the olfactory nerves, tracts or orbitofrontal cortex; visual deficit from an injury to the optic nerve, optic globe or extraocular muscle; or fractures of the orbit medial wall and floor. A fracture of the temporal bone may be associated with a blood clot and hemorrhagic fluid in the external auditory canal, a perforated tympanic membrane, and conductive or sensorineural hearing loss. Transverse fractures of the petrous temporal bone result in injury to cranial nerves VII and VIII in 50% of patients. In addition, deafness may result from ossicular disruption. Longitudinal fractures of the temporal bone result in injury to cranial nerve VII in 25% of patients. Labyrinthine injury may result in vertigo. Otitis media and meningitis may occur. Increased intracranial pressure and hydrocephalus may prolong a CSF leak that might otherwise cease spontaneously. A ventriculostomy catheter in a patient with CSF leak is associated with an increased incidence of meningitis. Pneumocephalus Pneumocephalus can sometimes give an audible succussion splash with shaking of the head. Tension pneumocephalus occurs in rare cases and can result in an emergency with an acute change in the level of consciousness. The air must then be drained by inserting a needle through a twist drill hole in the cranial bone. Spontaneous intracranial hypotension syndrome Patients with SIHS typically present with orthostatic headaches, which are maximal in intensity in upright position and diminished in the recumbent position. Other symptoms include neck pain and stiffness, nausea, diplopia, dizziness, hearing loss, photophobia, visual field defects, facial numbness, and (occasionally) radicular pain in the arms. Patients may have a history of recent lumbar puncture, spinal trauma, or surgery. In SIHS, CSF pressure is usually 40-60 mm of water, but it is sometimes normal. Preferred ExaminationA suggested algorithm for the diagnosis of a CSF fistula follows.
Fluid leaking from the nose or external auditory canal must first be positively identified as CSF. Drops of fluid from a CSF leak placed on absorbent filter paper may result in the double-ring sign, which is a central circle of blood and an outer clear ring of CSF. Results of glucose, chloride, and total protein tests of the fluid are not specific or conclusive for CSF. All methods of cisternography—radionuclide, CT, and MR—provide improved or optimal CSF fistula detection when the fistula is active and when a Valsalva maneuver or jugular venous compression is added to the imaging protocol. CSF fistula can usually be demonstrated by using some method of cisternography, but localization of the leak to the right or left nasal cavity may be difficult because of the tendency of the fluid to cross sides and flow from both nostrils. Methods for detecting CSF fistulas with intrathecal injections of dye pose a risk of chemical meningitis. Methylene blue, indigo carmine, and phenolsulfonphthalein (PSP) dyes are no longer in use. Some otolaryngologists use a dilute solution of fluorescein to localize CSF fistulas both preoperatively and during surgery. Typically, 0.5 mL of a 10% fluorescein solution is injected into the lumbar subarachnoid space over more than 1 minute. Cotton pledgets are placed in the nose, as for radionuclide cisternography. The dye reaches the skull base in 6 hours and is present over the cerebral convexities in 24 hours. The pledgets are examined for green fluorescence in a dark room with ultraviolet light 6 hours after the intrathecal PSP injection. Limitations of TechniquesSkull radiographs are of limited diagnostic use, but may show a skull fracture or suggest the presence of empty sella. Computer-reconstructed coronal images are less accurate and acceptable only until direct coronal images can be obtained. DIFFERENTIALS
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| Media file 1: Lateral 24-hour cranial scintigraphic image from a nuclear medicine cisternographic study in a patient with clinically evident right-sided cerebrospinal fluid rhinorrhea. Image demonstrates increased tracer accumulation in the nasal region (arrow). | |
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| Media file 2: Anterior 48-hour scintigraphic image in the same patient as in Image 1 demonstrates tracer accumulation in the right nasal region. Imaging findings were correlated with both the clinical findings and nasal pledget counts obtained as part of this study. | |
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| Media file 3: Acute posttraumatic cerebrospinal fluid rhinorrhea. This coronal magnetic resonance cisternogram demonstrates a left-sided cerebrospinal fluid leak through the cribriform plate (small arrows), which was clinically suspected. The image also shows a right-sided meningocele (large arrow) protruding through the cribriform plate, which was not suspected but was surgically repaired at the same time as the left cribriform cerebrospinal fluid leak site. | |
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| Media file 4: This patient presented with a spontaneous onset of cerebrospinal fluid rhinorrhea 10 years after a head injury. This coronal CT cisternogram was obtained after an intrathecal injection of contrast material (Omnipaque 300, 8 mL) into the lumbar thecal sac and subsequent positioning of the contrast agent in the head. The image demonstrates dense contrast medium layering in the empty sella and contained within the meningocele (arrow). | |
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| Media file 5: Axial CT image was obtained with the patient (same patient as in Image 4) in the supine position. Contrast medium has drained out of the meningocele, but a small amount remains in the sphenoid sinus around the meningocele. | |
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| Media file 6: Magnetic resonance cisternogram in the same patient as in Image 4 with cerebrospinal fluid rhinorrhea demonstrates a meningocele extending into the left lateral recess of the sphenoid sinus (arrows). | |
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| Media file 7: Axial magnetic resonance cisternogram of the same patient as in Image 4 demonstrates the connection of the meningocele to the middle cranial fossa (arrows). Fluid contained in the meningocele and leaked fluid in the sphenoid sinus outline the meningocele membrane. | |
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| Media file 8: Sagittal magnetic resonance cisternogram in the same patient as in Image 4 demonstrates the connection of the meningocele to the middle cranial fossa; this finding facilitated surgical planning. | |
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| Media file 9: Fast spin-echo T2-weighted coronal image of a patient with a spontaneous onset of cerebrospinal fluid rhinorrhea demonstrates an empty-sella configuration. | |
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| Media file 10: Axial CT image of the same patient as in Image 9 demonstrates pneumocephalus in association with the spontaneous cerebrospinal fluid rhinorrhea and a septal bone defect in the left posterior ethmoid air cell. | |
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| Media file 11: Coronal CT image of the temporal bone demonstrates a bone defect (small arrows) in the tegmen tympani with a protruding soft-tissue meningoencephalocele (large arrows). This patient had cerebrospinal fluid otorrhea after mastoidectomy. | |
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| Media file 12: Coronal fast spin-echo T2-weighted image in the same patient as in Image 11 demonstrates herniation of meninges and brain tissue (arrows) with adjacent cerebrospinal fluid into the postmastoidectomy tegmen tympani defect. This finding is consistent with a meningoencephalocele of the temporal bone. | |
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| Media file 13: Artist's rendering of a tegmen tympani bone defect with a herniated meningoencephalocele. | |
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| Media file 14: Sagittal magnetic resonance myelogram demonstrates a traumatic cerebrospinal fluid leak (small arrows) with disruption of the ligamentum flavum posteriorly (large arrow). | |
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| Media file 15: Magnetic resonance myelogram in a patient with a brachial plexus injury and pseudomeningoceles (arrows). | |
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| Media file 16: Magnetic resonance myelogram demonstrates pseudomeningoceles secondary to a stretch injury of the lumbosacral nerve roots. | |
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| Media file 17: Spontaneous intracranial hypotension syndrome in a patient with chronic headaches, which began after lumbar puncture. Axial fast spin-echo T2-weighted MRI demonstrates widened extra-axial fluid spaces but no focal extra-axial fluid collection. | |
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| Media file 18: Coronal fast spin-echo T2-weighted MRI in the same patient as in Image 17. | |
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| Media file 19: Gadolinium-enhanced T1-weighted axial MRI in the same patient as in Image 17 shows diffuse moderate dural thickening with contrast enhancement. | |
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| Media file 20: Gadolinium-enhanced, coronal, T1-weighted MRI in the same patient as in Image 17 shows dural and tentorial thickening with contrast enhancement. | |
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| Media file 21: Nuclear cisternogram obtained at 24 hours in the same patient as in Image 17 demonstrates diffuse epidural accumulation of the tracer in the midlumbar region. This finding is suggestive of a site of cerebrospinal fluid leak. | |
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| Media file 22: Gadolinium-enhanced, T1-weighted axial MRI in the same patient as in Image 17 obtained 2 weeks after a 7-mL extradural blood patch was applied to the midlumbar region. This image shows complete resolution of the previous dural thickening and contrast enhancement. The patient's severe postural headaches were markedly decreased in intensity | |
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| Media file 23: Gadolinium-enhanced, coronal, T1-weighted MRI in the same patient as in Image 17. | |
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Cerebrospinal Fluid, Leak excerpt
Article Last Updated: Feb 7, 2007