G. Michael Lemole Jr., MD
Jeffrey S. Henn, MD
Joseph M. Zabramski, MD
Volker K.H. Sonntag, MD
Department of Neurological Surgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, Arizona
The term CSF leak refers to any disruption of the arachnoid and dura that allows CSF to escape into an extradural space. CSF leaks can occur anywhere along the craniospinal axis. The most common clinical manifestation of cranial CSF leak is rhinorrhea or otorrhea. Leakage along the spinal pathways can cause severe postural headaches, such as those that aggravate a spinal tap. Occasionally, recurrent bouts of bacterial meningitis are the only clue to the diagnosis. Treatment options range from bed rest and CSF drainage to direct surgical repair through a variety of approaches. Treatment depends on the suspected location, cause, and symptoms of the CSF leak. The details of the lead authors' approach to the assessment and management of cranial and spinal CSF leaks are reviewed.
keywords: Liquor cerebrospinalis (CSF), Liquorfistel, Liquorleck
Diagnosis and treatment of a patient with a CSF leak depends on the location of the leak, its etiology, and the patient's symptoms. When a leak is associated with external drainage of CSF via the paranasal sinuses, external ear, or a skin tract, it is more appropriately referred to as a CSF fistula. However, in the literature, the distinction between these terms is blurred, and CSF fistula and CSF leak are used interchangeably.
CSF leaks can result from various etiologies, including trauma, hydrocephalus, tumor, infection, and iatrogenic and idiopathic causes. Clinical manifestations range from overt CSF drainage, which is easy to spot, to slow, intermittent leaks, which can be difficult to diagnose. Locating a CSF leak can also be difficult. The relevant anatomy and mechanisms of cranial and spinal CSF leak formation must be understood in order to properly diagnose and treat these lesions. A clear understanding of their natural history is also imperative to make informed management decisions.
This article examines the natural history, diagnosis, and treatment of CSF leaks involving the skull base and spine. The first section focuses on cranial CSF fistulas; The second section addresses spinal fluid leaks, including their relationship to symptomatic intracranial hypotension.
In the second century AD Galen described the leakage of cerebrospinal fluid after head trauma, but it was not considered a pathological process until the mid-17th century. In 1826 Miller noticed a nasal flow of spinal fluid in a child with hydrocephalus. In 1899, St. Clair Thomson coined the term rhinorrhea in a report describing a group of patients with spontaneous nasal CSF leakage. In 1923, Grant first suggested closing a traumatic dural defect. He reported on a 19-year-old man with rhinorrhea and pneumocephalus after a car accident. He stated, "We felt that an attempt should be made to locate and close the tear in the dura through which the air had entered." Excessive bleeding from the dura thwarted Grant's proposed surgery. In 1926, Dandy reported the first successful surgical repair of a CSF leak. He closed a traumatic dural tear over a frontal sinus fracture using muscle and fascia lata.
Over the next several decades, a better understanding of the natural history of traumatic and spontaneous CSF fistulas was gained. The earliest attempts to identify the location of a leak involved the introduction of dyes into the cerebrospinal fluid space. However, the dyes proved to be neurotoxic and are no longer recommended.[5,60,85]
Other intrathecal markers to document CSF leakage have been developed. In the 1950s, radioactive isotopes were first used and injected into the cerebrospinal fluid for diagnostic purposes. Since then, refinements in the radioactive tracers used for these procedures have improved the overall efficiency of the technique.
The development of computed tomography (CT) and the advent of water-soluble intrathecal contrast agents such as metrizamide have greatly improved the diagnosis and localization of CSF leaks. Diagnostic techniques are still evolving, and recent advances in magnetic resonance imaging (MR) suggest that it, too, may become a valuable tool.
CSF is formed in the cerebral ventricles by the choroid plexus and by transependymal flow of parenchymal fluid. CSF communicates directly with the basal subarachnoid cisterns through the Magendie and Luschka foramina in the posterior fossa. The subarachnoid space is defined by the potential volume between the pial lining overlying the neural parenchyma and the arachnoid layer of the meninges. Several trabeculae traverse the space between these two layers. The arachnoid layer does not directly follow the conformations of the underlying brain parenchyma, thus forming pockets or cisternae through which CSF seeps. CSF exiting the fourth ventricle flows freely down and up through the basal cisterns around the spinal cord and over the cerebral convexities to be reabsorbed into the venous blood stream through arachnoid granulations in the superior sagittal sinus (Fig. 1).
CSF is thought to act as a physiological shock absorber that protects the neuroparenchyma from direct trauma. It has also been suggested that CSF is analogous to lymph for the nervous system. The process of formation, flow and reabsorption of CSF is dynamic. The total volume of the cerebrospinal fluid space is about 150 ml and almost 500 ml of cerebrospinal fluid is produced daily. Therefore, the volume of CSF is replaced about three times a day.
Classification of cranial CSF leaks
In 1937, Cairns offered the first classification of CSF rhinorrhea, dividing CSF fistulas into acute, delayed, traumatic, operative, and spontaneous groups. This scheme was further refined by distinguishing between primary spontaneous or idiopathic rhinorrhea and secondary spontaneous rhinorrhea (i.e. rhinorrhea with an underlying etiology such as a tumor or hydrocephalus). Ommaya classified all CSF leaks as traumatic or non-traumatic, based on whether the underlying CSF dynamics reflected high or low pressure. Traumatic etiologies included accidental trauma to the skull or spinal axis, and iatrogenic injuries. Non-traumatic CSF leaks included those caused directly or indirectly by tumors, those caused by hydrocephalus, those induced by infection, and those thought to be from congenital abnormalities or focal atrophy (Fig. 2). Groups within Ommaya's classification scheme overlap (eg, when occult traumatic skull fractures manifest with delayed CSF leak), but the system is useful when the natural history of each group is considered.
Traumatic cranial CSF leaks
Trauma is the most common cause of cranial CSF leakage[5,60] and is responsible for up to 90% of CSF rhinorrhea cases. Fractures through the floor of the anterior cranial fossa can extend directly into the paranasal sinuses. If the dura and arachnoid are also torn by the trauma, a CSF fistula presenting with rhinorrhea may result. Fractures through the petrous bone can cause CSF leaks due to the proximity of the middle ear, particularly longitudinal fractures (Fig. 3) passing through the middle ear. Eighty percent of traumatic CSF leaks involve nasal pathways, while 20% involve auditory pathways. Penetrating head injuries can also cause CSF leaks anywhere.
Post-traumatic CSF leaks occur in 2% to 3% of head injury patients. The rate is highest in patients with anterior skull base fractures. Brodie and Thompson reported a 14.5% incidence of CSF leaks in a review of 820 cases of temporal bone fractures. In patients with facial fractures, the incidence of CSF rhinorrhea is up to 25%. The demographics of this patient population are consistent with trauma statistics, with the majority of cases affecting young adult males aged 18 to 25 years. Post-traumatic CSF leaks are uncommon in young children and rare in children under the age of 2.[5,15,44] The apparent immunity of infants to traumatic CSF leaks probably results from the flexibility of the skull base, particularly the cartilaginous nature of the ethmoid bones, and the poor development of the forehead - and sphenoid sinuses. Beyond the age of 5 years, the frontal sinuses progressively enlarge, reaching adult dimensions by 14 years. Interestingly, there is little correlation between the severity of head injury and the occurrence of a CSF leak. Mincy reported that nearly 50% of patients with post-traumatic CSF leak experienced brief or no loss of consciousness and had no neurological deficits.
Post-traumatic CSF leaks are divided into two categories: (1) those in which the leak is evident immediately or shortly after trauma, and (2) those in which the leak begins weeks to months after a head injury. CSF leak occurs in 60% of patients within the first few days of injury and is diagnosed within 3 months in 95% of patients. The delayed onset of CSF rhinorrhea or otorrhea after head trauma may reflect resolution of a hematoma, cerebral edema, or both at the site of the fracture. CSF leaks can also complicate delayed reduction of nasal or facial fractures. In a small number of patients, the onset of post-traumatic CSF leakage can be delayed by years, with the longest reported delay being 36 years.
Symptoms of a cranial CSF leak include frank rhinorrhea or otorrhea. CSF leakage can also occur intermittently or only with a change in posture. Anosmia is a common complaint, particularly when the lamina cribrosa is involved. Meningitis is the most significant risk associated with traumatic CSF leaks and has been reported to complicate 25 to 50% of untreated cases.[1,11,60] The risk of meningitis increases with the duration of the CSF leak. In a review of 122 cases of post-traumatic CSF leak, meningitis was reported in only 3% of patients when the leak was successfully treated within a week, compared to 23% in patients with leaks lasting longer than a week. Pneumococcal infection is the most common cause of meningitis in this population.
In contrast to adult patients with meningitis from other causes, the course of post-traumatic meningitis is relatively benign. Prompt treatment with appropriate antibiotics can minimize morbidity and mortality rates and allows for a rational approach to CSF fistula management without the need for “emergency” surgery.
According to the scheme proposed by Ommaya, CSF leaks resulting from surgical intervention are classified as traumatic (Fig. 2). CSF leak complications are more common in procedures involving the ethmoid and air cells of the mastoid. CSF leak rates associated with acoustic neuromas are 5 to 10%,[39,83] and those associated with transsphenoidal surgery are 3 to 6%.[19,80] proximal internal carotid artery can result in postoperative CSF leak. Careful waxing of exposed air cells in combination with the use of fibrin glue and multilayer closures can help minimize these risks.
Non-traumatic or spontaneous cranial CSF leaks
Ommaya divided spontaneous CSF leaks into normal and high pressure categories (Fig. 2). High-pressure leaks associated with long-standing increases in intracranial pressure (ICP) account for approximately 45% of spontaneous CSF leaks. Specific etiologies include congenital and acquired hydrocephalus, as well as other abnormalities associated with elevated ICP, including Crouzon's disease, Albers-Schönberg disease, and mass effect of slow-growing tumors. High pressure CSF leaks are not the result of direct invasion of the skull base. The remaining 50 to 60% of spontaneous CSF leaks occur with normal ICP.[84,85]
Nontraumatic causes of CSF leaks at normal pressure include intracranial tumors directly invading the skull base, infections such as osteomyelitis and tuberculosis, meningocele and meningoencephalocele, and defective development or atrophy of the olfactory bulb, which exposes the thin perforated bone of the cribriform plate to pulsations of CSF . Even in the presence of a normal ICP, it is postulated that the constant pulsation of CSF can cause the gradual dilation of these sacs with their eventual rupture and leakage of CSF. An elevated ICP for some reason can be expected to speed up this process. Kaufman et al. have emphasized the importance of pneumatization of the floor of the middle cranial fossa in the etiology of spontaneous CSF leaks. The authors hypothesize that pneumatization of the floor of the middle cranial fossa, combined with the normal CSF pulsations, thins the dura and bone, resulting in small pits and holes. Continuation of this process could lead to bony and dural dehiscence with herniation and rupture of arachnoid diverticula.
Non-traumatic or spontaneous CSF leaks are much less common than their traumatic counterparts. Females are more commonly affected than males (2:1), and presentation is most common in the fourth to fifth decade. A runny nose, triggered by coughing or sneezing, is the most common symptom. Patients may also complain of a salty or metallic taste in their throat. Intermittent symptoms can last for years and be misdiagnosed as allergic rhinitis or postnasal drip. Meningitis is rare but may be the only clue to the diagnosis. Unlike traumatic CSF leaks, anosmia is rare.
Diagnosis and localization of cranial CSF leaks
Management of patients with a CSF fistula requires confirmation of the diagnosis and localization of the responsible defect. Sometimes the presence of a CSF leak is obvious, as in the case of acute trauma associated with pneumocephalus, but it can also be extremely difficult to diagnose. Subtle CSF leakage can often be demonstrated by the patient sitting up, leaning forward, and flexing the neck after lying supine overnight. If CSF has accumulated in the sphenoid sinus overnight, flexing the head in an upright position drains the fluid through the sphenoid orifices located halfway up the anterior wall of the sphenoid sinus. This temporary heavy discharge (called a positive "reservoir sign") can aid in diagnosis and allow copious amounts of fluid to be collected for chemical analysis.
If adequate blood-free drainage can be collected, measuring the glucose level of the patient's fluid can be used to confirm the diagnosis of a CSF leak. CSF glucose levels are typically above 30 mg/100 mL.[30,42] After trauma, glucose analysis results are unreliable because the drain becomes contaminated with blood. Under such circumstances, the presence of the specific CSF marker b2-transferrin is diagnostic of a CSF fistula. Assays for this marker usually have to be sent to specialized laboratories and therefore it may take a day or two before results are available. A crude but relatively reliable test is to observe the pattern created by suspected leakage on clean linen. Bloody CSF characteristically creates a ring or "halo" pattern, with blood being carried to the periphery of the fluid spot.
All patients with head injuries and basal skull fractures should be evaluated for a possible CSF leak. As mentioned above, patients with specific fracture patterns that involve the frontal or ethmoid sinuses and those that extend parallel to the long axis of the petrous bone are at highest risk of developing CSF fistula. CT imaging with bone window settings is the method of choice for evaluating skull base fractures. Coronal CT imaging can be helpful in patients with facial fractures that extend to the base of the skull. Intracranial air is easily seen on CT and, in the absence of an obvious leak, provides evidence of the presence of a CSF fistula.
In patients with a spontaneous CSF leak, the diagnosis can be more difficult to confirm because the leak can be small and intermittent drainage is common. In such cases, radionuclide cisternography combined with the use of nasal compresses and maneuvers to increase ICP (eg, head positioning and clenching) can allow diagnosis of the CSF leak.[52,85] Cotton compresses are placed adjacent to the large ostia of the paranasal sinuses . The marked pledgets are removed after 12 to 24 hours and the radioactivity counted. Elevated levels confirm the presence of a CSF leak and can even help identify its location.
Accurate localization of the site of the CSF fistula is essential whenever surgical intervention is contemplated. High-resolution CT imaging will identify the bony defect and the site of the CSF fistula in a large percentage of cases. If there are questions about the location of the fistula, CT imaging can be combined with metrizamide cisternography. The metrizamide contrast is injected either via the lumbar subarachnoid space or a lateral C1-C2 puncture. The contrast medium is injected under fluoroscopic guidance and the patient is positioned head down to bring the contrast medium into the basal cisterns. If possible, the patient is placed in the prone position in the CT scanner with the neck slightly hyperextended to encourage gravity drainage of CSF through the fistula. Fine 3mm x 3mm coronal CT sections are obtained. Additional cuts in the coronal and axial planes may be required to visualize the fistula. Delayed imaging may be useful in patients with low-flow fistulas. Recent reports have advocated MR imaging as a noninvasive method for locating the site of a CSF fistula. Several groups have suggested that its sensitivity rivals that of CT cisternography; [24,27,58] However, this view is not yet widely accepted.
If the diagnosis of a CSF fistula has been confirmed but its location remains elusive, endoscopic techniques can be helpful. Endoscopy combined with intrathecal or topically applied fluorescein allows visualization of the CSF leak site.[14,41,45] In extreme cases, open surgical exploration without prior localization may be required to identify the site of the CSF leak. Direct repair of the fistula is usually attempted at the time of endoscopic or surgical exploration.
Treatment of cranial CSF leaks
The treatment of CSF fistulas can be divided into conservative and operative management. The choice of treatment depends on the etiology and severity of the CSF leak, as well as the timing and mode of presentation. The general rule of thumb is to start with the least invasive treatment and escalate as needed. This approach is particularly useful for traumatic CSF leaks.
Initial management of patients with a traumatic CSF leak typically includes bed rest and head elevation. Patients are encouraged to avoid coughing, sneezing, blowing their nose, and any exertion that would increase ICP. Stool softeners and laxatives are given as needed to avoid straining during a bowel movement. With this simple approach, 70-85% of traumatic CSF rhinorrhea cases heal within 7 days and almost all traumatic otorrhea cases heal.[11,55,60]
Traumatic CSF leaks that do not respond to conservative measures within 5 to 7 days should be considered for more invasive management.[1,60,73] Intermittent spinal taps and placement of a spinal drain are effective methods to reduce ICP and to promote the closure of the CSF fistula. All patients with spinal drains should be monitored in an intensive care unit. Rapid or excessive CSF drainage is dangerous and must be avoided. We prefer to start draining at 5 to 10cc per hour. The drain height is titrated to maintain a slow, steady flow rate. The drain will be closed once the desired volume has been reached for a given hour. The drain can also be closed to allow the patient to sit by the bed to eat. Excessive CSF drainage is associated with headache, nausea, and vomiting, and can rapidly progress to coma with evidence of tentorial herniation. If the patient's condition worsens, the drain should be closed immediately and the patient placed in a flat or slight Trendelenburg (head down) position. Oxygen (100%) is given and a CT scan is done to assess the presence of intracranial air and more precisely define the cause of deterioration. The presence of pneumocephalus is a contraindication for continued lumbar CSF drainage.
The prophylactic use of antibiotics in patients with traumatic CSF fistulas remains controversial. In 1997, Brodie published a meta-analysis suggesting that prophylactic antibiotics might be beneficial; Numerous other studies, including a second meta-analysis by Villalobos et al. from 1998, could not demonstrate that they reduce infection rates. The emergence of drug-resistant bacteria and reports of infections with more virulent organisms have heightened concerns about the indiscriminate use of antibiotics.[17,25] In an intensive care unit, the widespread use of prophylactic antibiotics led to an epidemic of Klebsiella meningitis that resulted in eight fatalities. Based on the available literature, the prophylactic use of antibiotics in patients with CSF leakage appears ineffective and cannot be recommended. This caveat does not include the short-term perioperative use of antibiotics beginning immediately prior to surgery to repair a CSF fistula.
Surgical treatment is reserved for traumatic CSF leaks that do not respond to conservative measures. Surgical interventions can be divided into intracranial and extracranial approaches. Extracranial approaches are limited to the anterior cranial fossa and paranasal sinuses. Endoscopic techniques have expanded the indications and improved outcomes associated with extracranial CSF leak repair.[57,66,78] Success rates of up to 90% have been reported. Under direct endoscopic vision, a leak can often be identified and the sinus into which it empties obliterated by fat or muscle filling (Fig. 4).
Other techniques described in connection with extracranial techniques include mobilization of mucosal flaps and combinations of autologous tissue with fibrin glue.[2,20,59,79] A theoretical disadvantage of extracranial approaches is that the patch is less secure than when it is placed intradurally. In the intradural repair, the patch is held firmly against the defect by the brain and normal CSF pressure. Wormald and McDonogh recently addressed these concerns by describing the endoscopic repair of CSF leaks using a "bath-plug" technique to seal the defect from the intradural side.
Typically, intracranial procedures are reserved for patients with defects not amenable to extracranial endoscopic techniques, including patients with extensive skull base fractures, comminuted fractures with displaced fragments that require reduction, and fractures associated with intracranial hemorrhage or contusion that usually require craniotomy would be treatment.
A bicoronal incision is typically used (Fig. 5) and the craniotomy is tailored to the extent of the bony defects and associated intracranial pathology. If bony defects are lateralized on one side, a frontal or pterional craniotomy can be used. The patient's head is positioned so that gravity assists in retracting the frontal lobes. Lumbar drainage of CSF and intraoperative application of mannitol can further minimize the need for retraction. Depending on the location of the skull base defect, the surgical microscope is an invaluable addition. Dura defects can be repaired directly by suturing, but autologous tissue grafts are often used.
The authors prefer a vascularized periosteal graft, but fascia lata or various commercially available dural substitutes can be used. Grafts can be sutured in place, but most surgeons use fibrin glue to strengthen the seal (Fig. 6). In clinical and laboratory studies, fibrin sealant has been shown to significantly improve dural closure and reduce the risk of CSF leakage.[35,59,67]
Surgical treatment of CSF leaks may require repair of bone defects. Small defects can be filled with bone dust and fibrin glue. For larger defects, split bone autografts fixed with fibrin glue or one of the available microplating systems are ideal. Facial fractures should be reduced before or in combination with procedures to close a traumatic CSF fistula. [22,26,75] Early treatment of facial fractures can enhance the spontaneous resolution of traumatic CSF leaks, while delayed reduction can lead to recurrence.
The same principles apply to the closure of CSF fistulas that arise in the middle or posterior fossa. Leakage in the middle cranial fossa may occur through air cells in the inferior sphenoidal wing or in the floor of the temporal fossa. In the latter cases, an extradural approach can be used to close the defect.[37,65] Ultimately, combined intra- and extradural approaches may be required. In rare cases, a mastoidectomy may be required to obliterate the offending air cells.34,36 The correct surgical approach is that which permanently stops the CSF leak with the least risk to the patient.
Postoperative CSF leaks often respond to conservative measures.
CSF leakage after transphenoidal surgery should initially be treated with maximum rest and lumbar CSF drainage. If the leak persists despite lumbar drainage, repacking of the sphenoid sinus is indicated.
CSF leaks complicate 10 to 20% of cerebellopontine angle tumor resections, particularly when the approach involves extensive petrous bone drilling. [9,28,31,50,72] The risk of CSF leakage can be minimized by carefully packing open air cells with wax or fat, and by filling bone defects with adipose tissue. Bed rest and spinal drainage are usually sufficient to stop these leaks. Subgaleal accumulations may respond well to aspiration and a simple compression bandage. A percutaneous technique for aspirating fluid and injecting fibrin glue to close a subgaleal fistula has also been described. Recurrent subgaleal accumulations should raise suspicion of hydrocephalus.
Unlike traumatic CSF leaks, spontaneous CSF leaks are unlikely to respond to nonsurgical intervention. Treatment aims to identify the site of the fistula and treat the underlying cause (ie, resection of a mass lesion causing increased ICP or insertion of a shunt for hydrocephalus). The presence of hydrocephalus can be difficult to assess in patients with an active CSF leak. Careful follow-up with serial CT imaging studies can reveal evidence of previously unsuspected hydrocephalus. The delayed failure of an apparently successful repair should raise clinical suspicion of hydrocephalus. In such cases, radiolabeled cisternography may show altered CSF dynamics such as isotopic reflux into the ventricles.
Leaks in the spinal fluid
Like their cranial counterparts, CSF leaks affecting the spinal axis can be divided into traumatic and non-traumatic (or spontaneous) categories. Traumatic spinal CSF leaks include iatrogenic lesions resulting from surgical, therapeutic, or diagnostic procedures. The diagnosis is often straightforward, as in the case of the patient with penetrating trauma and cutaneous CSF leak or the patient with spinal headache after a lumbar puncture. Traumatic CSF fistulas from the spinal subarachnoid space to the pleural space have been described.[3,32,69] Symptoms of intracranial hypotension (severe postural headache) and the presence of pleural fluid accumulation should suggest the diagnosis. The presence of b2-transferrin in the pleural fluid is a diagnostic clue to a CSF leak.
Spontaneous spinal CSF leaks can be difficult to diagnose, but the similarity of symptoms to those of post-lumbar puncture spinal headache should suggest the diagnosis. Severe headaches, nausea, and vomiting often prompt MR imaging of the brain. Contrast-enhanced MR images show diffuse, intense dural enhancement that can be confused with meningitis or metastatic or inflammatory disease.[8,12,18] Subdural fluid collections consistent with hygromas are reported in 60 to 70% of patients.  A caudal displacement of the cerebellar tonsils and obliteration of the basal cisterns are usually present.[8,12,18,70]
Other clinical features may be more alarming: cranial neuropathies, typically unilateral or bilateral abducens palsy, transient visual disturbances, photophobia, hearing disturbances, facial deafness or weakness, and drowsiness from traction and downward displacement of the brainstem.[6,38,49,71] The literature to spontaneous leakage of CSF is somewhat confusing. Despite the recognition that these symptoms are the result of spontaneous spinal CSF leaks, patients are grouped under the diagnosis of spontaneous intracranial hypotension.
Radionucleotide cisternography is quite characteristic in patients with spontaneous CSF leak. In patients with an active leak, the escape of CSF from the spinal subarachnoid space leads to rapid uptake of the tracer into the bloodstream, as shown by the early appearance of the tracer in the kidneys and bladder [46,56,64,70] uncommonly identifies the site of a spinal CSF leak, but often the leak is below the resolution of the study.[46,70]
CT myelography is required to locate the leak if surgery is contemplated and can reveal the underlying anatomical deficit causing the leak, such as: B. a meningeal diverticulum leak for subsequent CT scans.
If myelography cannot locate the area of the leak, CT imaging should be done along the entire spinal axis. MR imaging studies of the spine can show extra-axial CSF collections that help localize the leak site, but experience with spinal MR imaging in patients with spontaneous spinal CSF leak is limited.
Treatment of spinal cerebrospinal fluid leaks
Treatment of spinal CSF fistulas follows the paradigm described for cranial CSF leaks. In the case of traumatic and postoperative CSF leaks, bed rest and brief lumbar drainage are often sufficient. Postoperative leaks often respond to simple suturing over the wound, but patients should be closely monitored for signs of persistent leaks, such as: B. postural headache or the development of a pseudomeningocele. If the fistula is unresponsive to conservative measures, surgical exploration should be considered.
In postoperative cases, careful inspection often reveals a previously unnoticed dural tear that can be repaired with direct suturing, application of fibrin glue, or both. For spinal fluid leaks associated with penetrating trauma, access is dictated by the site of the fistula, which is best defined by CT myelography. Percutaneous techniques to deliver fibrin glue to the leak site have been successful.[40,61]
Treatment of spontaneous CSF leak begins with fluid replacement and bed rest to relieve symptoms of intracranial hypotension. Medical treatment options include intravenous caffeine infusions, glucocorticoids and various nonsteroidal anti-inflammatory drugs, mineralocorticoids, and saline infusions. A more direct and, in our experience, more effective treatment is a lumbar epidural blood patch. The success of this procedure can be confirmed by the findings of Szeinfeld et al. be explained. that blood injected into the lumbar epidural spaces spreads rostrally and caudally to involve eight or more spinal segments. Fortunately, 60 to 70% of patients respond to these conservative measures.[8,43,70] Surgery should only be considered if symptoms recur after treatment with two appropriate patches of blood.
Most documented leaks are at the cervicothoracic junction or in the thoracic spine. Where surgical confirmation was available, the leaks were the result of ruptured meningeal diverticula. Surgical ligation of leaky meningeal diverticula has been associated with good results.[43,71] Recently, epidural patches containing fibrin glue have been used successfully to treat patients with spontaneous CSF leaks who have failed to respond to epidural blood patches.
The etiology of most meningeal diverticula is unknown, and it is unclear whether these lesions are congenital or acquired. However, it is likely that, in at least some cases, an underlying weakness of the spinal cord membranes is involved, predisposing the patient to developing meningeal diverticula.
CSF leaks can occur anywhere along the craniospinal axis. Trauma is the most common cause of cranial CSF leak, but spontaneous cases are increasingly recognized, particularly along the spinal axis, where they clinically manifest as spontaneous intracranial hypotension. The choice of treatment depends on the etiology and severity of the CSF leak; However, the general rule is to start with conservative measures and escalate to more invasive procedures as needed. Exceptions requiring direct surgical intervention may be patients with large amounts of intracranial airflow, extensive skull base fractures, or chronic CSF leakage.
- Aarabi B, Leibrock LG: Neurosurgical approaches in CSF rhinorrhea. Ear, nose and throat J 71: 300-305, 1992
- Anand VK, Murali RK, Glasgold MJ: Surgical decisions in the management of CSF rhinorrhea. Rhinology 33:212-218, 1995
- Assietti R, Kibble MB, Bakay RA: Iatrogenic liquor fistula to the pleural cavity: Ca
- Beckhardt RN, Setzen M, Carras R: Primary spontaneous liquorrhinorrhoea. Otolaryng Head Neck Surg 104:425-432
- Bergman TA, Rockswold GL: CSF fistulas, in Youmans JR (ed.): Youmans Neurological Surgery. A comprehensive guide to the diagnosis and treatment of neurosurgical problems. Philadelphia: W.B. Saunders, 1996, pp. 1840-1852
- Berlit P, Berg-Dammer E, Kühne D: Abducens nerve palsy in spontaneous intracranial hypotension. Neurology 44:1552, 1994
- Binhammer RT: CSF anatomy with emphasis on relationships to the nasal cavity and labyrinthine fluids. Ear, nose and throat J 71: 292-299, 1992
- Blank SC, Shakir RA, Bindoff LA, et al: Spontaneous intracranial hypotension: clinical and magnetic resonance imaging features. Clin Neurol Neurosurg 99: 199-204, 1997
- Brennan JW, Rowed DW, Nedzelski JM, et al: CSF leakage after acoustic neuroma surgery: influence of tumor size and surgical approach on incidence and treatment response. J Neurosurg 94:217-223, 2001
- Brodie HA: Prophylactic antibiotics for post-traumatic CSF fistulas. A meta-analysis. Arch Otolaryngol Head Neck Surg 123:749-752, 1997
- Brodie HA, Thompson TC: Management of complications in 820 temporal bone fractures. Am J Otol 18: 188-197, 1997
- Bruera OC, Bonamico L, Giglio JA, et al: Intracranial hypotension: the nonspecific nature of MRI findings. Headache 40:848-852, 2000
- Cairns H: Injuries to the frontal and ethmoid sinuses with special reference to liquorrhinorrhoea and aeroceles. J Laryngol Otol 52: 589-623, 1937
- Calcaterra TC: Extracranial surgical repair of cerebrospinal rhinorrhea. Ann Otol Rhinol Laryngol 89: 108-116, 1980
- Caldicott WJ, North JB, Simpson DA: Traumatic CSF fistulas in children. J Neurosurg 38:1-9, 1973
- Casiano RR, Jassir D: Endoscopic CSF rhinorrhea repair: is lumbar drainage necessary? Otolaryngol Head Neck Surg 121:745-750, 1999
- Choi D, Spann R: Traumatic CSF leakage: risk factors and the use of prophylactic antibiotics. Br. J. Neurosurg 10:571-575, 1996
- Christoforidis GA, Mehta BA, Landi JL, et al: Spontaneous intracranial hypotension: report of four cases and review of the literature. Neuroradiology 40:636-643, 1998
- Ciric IS, Tarkington J: Transsphenoidale Mikrochirurgie. Surge Neurol 2:207-212, 1974
- Citardi MJ, Cox AJ3, Bucholz RD: Acellular dermal allograft for sellar reconstruction after transsphenoidal hypophysectomy. Am J Rhinol 14:69-73, 2000
- Crow HJ, Keogh C, Northfield DWC: The localization of CSF fistulas. Lancet 2:325-327, 1956
- Daly DT, Lydiatt WM, Ogren FP, et al.: Extracranial approaches to repair of cerebrospinal fluid rhinorrhea. Ear, nose and throat J 71: 311-313, 1992
- Dandy WE: Pneumocephalus (intrakranielle Pneumatozele oder Aerozele). Arch Surg 12:949–982, 1926
- El Gammal T, Sobol W, Wadlington VR, et al: Cerebrospinal fluid fistel: Detection with MR cisternography. AJNR Am J Neuroradiol 19:627-631, 1998
- Eljamel MS: Antibiotic prophylaxis in unrepaired CSF fistulas. Br. J. Neurosurg 7:501-505, 1993
- Eljamel MS: Fractures of the middle third of the face and rhinorrhea of the cerebrospinal fluid. Br. J. Neurosurg 8:289-293, 1994
- Eljamel MS, Pidgeon CN: Lokalisation inaktiver Liquorfisteln. J Neurosurg 83: 795-798, 1995
- Fishman AJ, Hoffman RA, Roland JT, Jr., et al: CSF drainage in the management of CSF leaks after acoustic neuroma surgery. Laryngoscope 106:1002-1004, 1996
- Fitzgerald LF, Sandlin M, Carrier D, et al: Spontaneous intracranial hypotension: myelographic findings. case presentation. J Neurosurg 92:188, 2000
- Gadeholt H: The reaction of glucose oxidase test paper in normal nasal secretions. Acta Otolaryngol (Stockh) 58:271-272, 1964
- Gillman GS, Parnes LS: Acoustic Neuroma Management: A Six-year rieview. J. Otolaryngol 24:191–197, 1995
- Godley CD, McCabe CJ, Warren RL, et al: Traumatic subarachnoid pleural fistula: case report. J Trauma 38:808-811, 1995
- Grant FC: Intracranial aerocele after skull fracture: case report with literature review. Surg Gynecol Obstet 36:251-255, 1923
- Grant IL, Welling DB, Oehler MC, et al.: Transcochlear repair of persistent CSF leakage. Laryngoscope 109:1392-1396, 1999
- Hadley MN, Martin NA, Spetzler RF, et al: Comparative transoral dural closure techniques: A canine model. Neurosurgery 22:392-397, 1988
- Hamilton JW, Foy PM, Lesser TH: Subtotal petrosectomy in the treatment of lateral skull base CSF fistula. Br. J. Neurosurg 11:496-500, 1997
- Harner SG, Laws ER, Jr.: Translabyrinthine repair for cerebrospinal fluid otorhinorrhea. J Neurosurg 57: 258-261, 1982
- Horton JC, Fishman RA: Neurovisual findings in the syndrome of spontaneous intracranial hypotension due to dural cerebrospinal fluid leak. Ophthalmology 101:244-251, 1994
- House WF, Hitselberger WE: Surgical complications of acoustic tumor surgery. Arch Otolaryngol 88:659-667, 1968
- Huch K, Kunz U, Kluger P, et al: Epidural blood patch under fluoroscopy: non-surgical treatment of a lumbar cerebrospinal fluid fistula after implantation of an intrathecal pump system. Spinal Cord 37:648-652, 1999
- Hughes RG, Jones NS, Robertson JJ: The endoscopic treatment of CSF rhinorrhea: the Nottingham experience. J Laryngeal Vet 111: 125-128, 1997
- Hull HF, Morrow G: Reunion with Glucorrhea. Longer spread of another plastic bead. JAMA 234:1052-1053, 1975
- Inenaga C, Tanaka T, Sakai N, et al: Diagnostic and surgical strategies for intractable spontaneous intracranial hypotension. case report. J Neurosurg 94: 642-645, 2001
- Jones DT, McGill TJ, Healy GB: Cerebrospinal fistulas in children. Laryngoscope 102:443-446, 1992
- Jones ME, Reino T, Gnoy A, et al: Identification of intranasal CSF leaks by topical application of fluorescein dye. Am J. Rhinol 14:93-96, 2000
- Kadrie H, Driedger AA, McInnis W: Persistent dural-cerebrospinal fluid loss demonstrated by retrograde radionuclide myelography: case report. J. Nucl. Med. 17:797-799, 1976
- Kamada M, Fujita Y, Ishii R, et al: Spontaneous intracranial hypotension successfully treated by epidural patch with fibrin glue. Headache 40:844-847, 2000
- Kaufman B, Nulsen FE, Weiss MH, et al: Acquired spontaneous non-traumatic normal-pressure CSF fistulas originating in the middle cranial fossa. Radiology 122:379-387, 1977
- Kosmorsky GS: Spontaneous intracranial hypotension: A review. J Neuro-Ophthalmol 15: 79-83, 1995
- Magliulo G, Sepe C, Varacalli S, et al: Management of CSF leak after cerebellar pontine angle surgery. J. Otolaryngol 27:258-262, 1998
- Matsumura A, Anno I, Kimura H, et al: Diagnosis of spontaneous intracranial hypotension by magnetic resonance myelography. case report. J Neurosurg 92: 873-876, 2000
- McKusick KA, Malmud LS, Kordela PA, et al: Radionuclide cisternography: normal values for nasal secretion of intrathecally injected 111In-DTPA. J. Nucl. Med. 14:933-934, 1973
- Menovsky T, de Vries J, Bloss HG: Treatment of postoperative subgaleal CSF fistulas with fibrin glue. Technical Note. J Neurosurg 90: 1143-1145, 1999
- Miller C: A case of Hydrocephalus Chronicus with some unusual symptoms and presentations on dissection. Trans Med Chir Soc 2: 243-248, 1826
- Mincy JE: Post-traumatic CSF fistula of the frontal fossa. J. Trauma 6:618-622, 1966
- Molins A, Alvarez J, Sumalla J, et al: Cisternographic pattern of spontane liquoral hypotension. Cephalalgia 10:59-65, 1990
- Nachtigal D, Frenkiel S, Yoskovitch A, et al: Endoscopic repair of CSF rhinorrhea: is it the treatment of choice? J. Otolaryngol 28:129-133, 1999
- Nakayama Y, Tanaka A, Ueno Y, et al: Spontaneous leakage of cerebrospinal fluid detected by magnetic resonance cisternography - case report. Neurol Med Chir (Tokyo) 39:251-254, 1999
- Nishihira S, McCaffrey TV: The use of fibrin glue to repair experimental CSF rhinorrhea. Laryngoscope 98:625-627, 1988
- Ommaya AK: Liquorfistel und Pneumozephalus, in Wilkins RH, Rengachary SS (Hrsg.): Neurosurgery. New York: McGraw-Hill, 1996, S. 2773-2782
- Patel MR, Louie W, Rachlin J: Postoperative CSF leaks of the lumbosacral spine: management with percutaneous fibrin glue. AJNR Am J Neuroradiol 17:495-500, 1996
- Price DJ, Sleigh JD: Control of Klebsiella aerogenes infection in a neurosurgical unit by discontinuation of all antibiotics. Lancet 2:1213-1215, 1970
- Reisinger PW, Hochstrasser K: The diagnosis of CSF fistulas based on the detection of beta 2-transferrin by polyacrylamide gel electrophoresis and immunoblotting. J.Clin. Chem. Clin. biochem. 27:169-172, 1989
- Renowden SA, Gregory R, Hyman N, et al: Spontane intrakranielle Hypotonie. J Neurol Neurosurg Psychiatry 59:511-515, 1995
- Robson AK, Clarke PM, Dilkes M, et al: Transmastoid extracranial repair of CSF leaks after acoustic neuroma resection. J Laryngol Otol 103: 842-844, 1989
- Ross IB, Colohan AR, Black MJ: Extrakranielle Reparatur von Liquor-Rhinorrhoe. Can J Neurol Sci 17: 320-323, 1990
- Rossitch E, Jr., Wilkins RH: Use of fibrin glue in neurosurgery, in Wilkins RH, Rengachary SS (eds): Neurosurgery. New York: McGraw-Hill, 1996, pp. 623-624
- Salca HC, Danaila L: Onset of an uncomplicated CSF fistula 27 years after a head injury: case report. Surgeon Neurol 47: 132-133, 1997
- Sarwal V, Suri RK, Sharma OP, et al: Traumatische Subarachnoidal-Pleural-Fistel. Ann ThoracSurg 62:1622-1626, 1996
- Schievink WI, Meyer FB, Atkinson JL, et al: Spontaneous spinal-cerebrospinal fluid leaks and intracranial hypotension. J Neurosurg 84: 598-605, 1996
- Schievink WI, Morreale VM, Atkinson JL, et al: Surgical treatment of spontaneous leakage of spinal cerebrospinal fluid. J Neurosurg 88:243-246, 1998
- Sluyter S, Graamans K, Tulleken CA, et al: Analysis of the outcome of 120 patients with large acoustic neuromas treated surgically via the translabyrinthine-transtentorial approach. J Neurosurg 94: 61-66, 2001
- Spetzler RF, Wilson CB: Treatment of recurrent liquorrhinorrhea of the middle and posterior cranial fossa. J Neurosurg 49: 393-397, 1978
- Stone JA, Castillo M, Neelon B, et al: Assessing CSF leaks: High-resolution CT versus contrast-enhanced CT and radionuclide cisternography. AJNR Am J Neuroradiol 20:706-712, 1999
- Stranc MF, Harrison DH: Primary treatment of craniofacial injuries. Rev Stomatol Chir Maxillofac 79: 363-371, 1978
- Szeinfeld M, Ihmeidan IH, Moser MM, et al: Epidural blood stain: assessment of the volume and spread of blood injected into the epidural space. Anesthesiology 64:820-822, 1986
- Thomson St C: The cerebrospinal fluid: its spontaneous escape from the nose. London: Kassel, 1899
- Tolley NS: A clinical study of spontaneous CSF rhinorrhea. Rhinology 29:223-230, 1991
- Van Den Abbeele T, Elmaleh M, Herman P, et al: Transnasal endoscopic repair of congenital skull base defects in children. Arch Otolaryngol Head Neck Surg 125:580-584, 1999
- VanGilder JC, Goldenberg IS: Hypophysectomy for metastatic breast cancer. Arch Surg 110: 293-295, 1975
- Villalobos T, Arango C, Kubilis P, et al: Antibiotic prophylaxis after basilar skull fractures: A meta-analysis. Clin Infect Dis 27:364-369, 1998
- Wormald PJ, McDonogh M: "Bath-plug" technique for endoscopic treatment of CSF leaks. J Laryngol Otol 111: 1042-1046, 1997
- Yasargil MG, Fox JL: The microsurgical approach to acoustic neuroma. Surge Neurol 2:393-398, 1974
- Yerkes SA, Thompson DH, Fisher WS 3rd: Spontaneous CSF rhinorrhea. Ear, nose and throat J 71: 318-320, 1992
- Zlab MK, Moore GF, Daly DT, et al: Cerebrospinal fluid rhinorrhea: A review of the literature. Ear, nose and throat J 71: 314-317, 1992
How do you treat cranial CSF leak? ›
Surgery is often the best treatment option for cranial CSF leaks. Surgical approaches are tailored to the exact location of the leak. Once surgeons reach the leak site, they repair the hole by plugging it with tissue or fat.What is the difference between a spinal CSF leak and a cranial CSF leak? ›
A spinal CSF leak occurs anywhere in the spinal column. A cranial CSF leak occurs in the skull. The most common symptom of a spinal CSF leak is a headache, while a cranial CSF leak causes symptoms such as clear fluid leaking from the nose or ear. Some CSF leaks may heal with conservative treatments such as bed rest.What is the conservative treatment for cranial CSF leak? ›
Conservative management consists of a 7-10 day trial of bed rest with the head of the bed elevated approximately 15-30°. This angle of inclination is sufficient to reduce the CSF pressure at the basal cisterns. Coughing, sneezing, nose blowing, and heavy lifting should be avoided as much as possible.How serious is a cranial CSF leak? ›
A CSF leak is a very serious condition, and patients who have tears in their dura with persistent CSF leaks need repair as soon as possible to reduce headache pain and the chance of meningitis.How long does it take for a spinal CSF leak to heal? ›
Postoperatively, patients are placed on bed rest in the hospital, typically for two to three days, to help facilitate the healing process and closely monitor for leak recurrence. The repair site can take four to six weeks to heal completely.Can a cranial CSF leak heal on its own? ›
Most cases heal by themselves with no lasting symptoms. If the CSF leak keeps coming back, high pressure of the CSF (hydrocephalus) might be the cause and should be treated.What are the symptoms of leaking spinal fluid? ›
- Positional headaches, which feel worse when sitting upright and better when lying down; caused by intracranial hypotension.
- Nausea and vomiting.
- Neck pain or stiffness.
- Change in hearing (muffled, ringing in the ears)
- Sense of imbalance.
- Photophobia (sensitivity to light)
Two likely places that CSF can leak into are your sinuses or your nose. In either case, you'll have a runny nose (rhinorrhea) with thin, clear fluid.How common is cranial CSF leak? ›
How common is a CSF leak? Spontaneous CSF leaks (ones that are not triggered by a medical procedure such as a lumbar puncture, or by a serious injury such as a gunshot wound) occur in at least five in 100,000 people per year, making them fairly rare.What is the success rate of CSF leak surgery? ›
The primary surgical success rate was 79.4%, with a significantly higher success rate for lateral than for anterior skull base defects.
Do neurologists treat CSF leak? ›
Mayo Clinic offers a multidisciplinary, specialized team of CSF experts including neurologists, neurosurgeons, ear nose and throat (ENT) specialists, imaging specialists (radiologists), and other specialty professionals who work together to provide exactly the care you need.What type of doctor treats CSF leak? ›
Our sinus specialists, otologists/neurotologists, and neurosurgeons collaborate to diagnose and treat cranial CSF leaks. These are most often treated with surgery.Can a cranial CSF leak cause back pain? ›
All patients presented with a postural headache; however, most had additional symptoms, including nausea, emesis, sixth cranial-nerve paresis, or local back pain at the level of the CSF leak.What happens when spinal fluid goes to the brain? ›
Cerebrospinal fluid normally flows through the ventricles and bathes the brain and spinal column. But the pressure of too much cerebrospinal fluid associated with hydrocephalus can damage brain tissues and cause a range of brain function problems.Can you live with a spinal leak? ›
Outlook is usually good depending on the cause. Most cases heal by themselves with no lasting symptoms. If the CSF leak keeps coming back, high pressure of the CSF (hydrocephalus) might be the cause and should be treated.What should I avoid with a CSF leak? ›
You will need to stay in bed with your head raised and avoid activities that cause pressure. These activities include coughing, vomiting, blowing your nose, and straining to have a bowel movement. Your provider may give you medicines to stop any coughing and vomiting.Is spinal CSF leak an emergency? ›
CSF Leaks and Skull Base Defects
If a CSF leak is suspected, you should see a physician as soon as possible. If symptoms of meningitis (high fever, light sensitivity, neck stiffness) are suspected, you should go to the emergency room.
The caffeine in coffee is thought to increase CSF production, thereby helping to reduce headache severity in those with spinal CSF leaks.Does caffeine increase spinal fluid? ›
Conclusion. The results of this study show that long-term consumption of caffeine can induce ventriculomegaly, which is mediated in part by increased production of CSF.What does a CSF headache feel like? ›
The classic symptom is a headache that becomes severe when the patient is upright and quickly disappears when the patient is lying flat. So, the headaches are typically absent first thing in the morning, and start or worsen shortly after getting out of bed.
How long can a spinal fluid leak last? ›
Spinal fluid leaks also can lead to serious complications, including seizures. Patients may have a CSF leak for years or decades before it is diagnosed.Does CSF leak show on MRI? ›
Magnetic resonance imaging (MRI) accurately detects CSF leaks and associated complications such as the encephaloceles and meningoceles.How long does spinal fluid take to replenish? ›
Depending on the rate of production and absorption (which varies individually), the supply of CSF can be replaced about every 7.5 hours. Most of this fluid is produced in the ventricles of the brain by the choroid plexus—however, the ependymal cells, which line the ventricles, produce a smaller portion.Is CSF leak curable? ›
An epidural blood patch
Epidural blood patches have high success rates but may not cure all types of CSF leaks.
Cerebrospinal fluid (CSF) leakage is a hazardous complication after spine surgery because it can cause symptoms of intracranial hypotension, meningitis and other complications.Can CSF be cured without surgery? ›
Sealant. A special sealant may be used alone or mixed with your blood in the same technique described above to cover the hole and stop the CSF leak. Surgery. Some CSF leaks need surgery, though surgery is only performed if the other treatment options don't work and the precise site of the leak is known.Can a CSF leak come back after surgery? ›
A recurrent CSF leak developed in five (38%) of 13 patients who had undergone surgical CSF leak repair, compared with none (0%) of five patients who had been treated non-surgically (p = 0.249).Can a CSF leak cause memory loss? ›
The memory loss from a spinal CSF leak can be so severe that it resembles dementia. It's much more debilitating than 'brain fog'.How does cerebrospinal fluid affect memory? ›
Conclusion: Patients with lower CSF flow had significantly worse memory, visuo-constructive capacities, and verbal fluency. Alterations in CSF flow could contribute to some of the cognitive deficit observed in patients with AD.Can you live with fluid on the brain? ›
Hydrocephalus is a chronic condition. It can be controlled, but usually not cured. With appropriate early treatment, however, many people with hydrocephalus lead normal lives with few limitations. Hydrocephalus can occur at any age, but is most common in infants and adults age 60 and older.
How do you fix fluid on the brain? ›
A shunt (tube) is surgically inserted into the brain and connected to a flexible tube placed under the skin to drain the excess fluid into either the chest cavity or the abdomen so it can be absorbed by the body. Endoscopic Third Ventriculostomy (ETV) improves the flow of CSF out of the brain.How long can you go with a CSF leak? ›
Most of posttraumatic CSF leakage resolves within six months after trauma. CSF fistula should be closely evaluated because of fatal complication. CSF leakge is the first symptom usually noticed by the patients. Therefore, it can usually be diagnosed before the development of meningitis as its complication.What is the best sleep position for CSF leak? ›
Sleep with your head slightly elevated for 7 days. Stay in a bed or chair with your head up during the day for 7 days. You may go to the restroom. No heavy lifting, bending over, or straining for 30 days.What happens if a CSF leak is not treated? ›
A cranial CSF leak results in a runny nose and carries a high risk of meningitis, an inflammation of the membrane around the brain and spinal cord, which can be fatal and requires emergency attention.What type of doctor treats spinal fluid leak? ›
Our sinus specialists, otologists/neurotologists, and neurosurgeons collaborate to diagnose and treat cranial CSF leaks. These are most often treated with surgery.What makes a CSF leak worse? ›
The most common symptom of intracranial hypotension from a CSF leak is a postural headache, which means a headache that changes depending on your posture. A postural headache with a CSF leak worsens when you sit up or stand and improves when you lie down.Does a CSF leak show up on MRI? ›
CT cisternography, though invasive, helps accurately identify the site of CSF leak, especially in the presence of multiple bony defects. Magnetic resonance imaging (MRI) accurately detects CSF leaks and associated complications such as the encephaloceles and meningoceles.