Cerebrospinal Fluid Leak


Article Author:
Michael Severson


Article Editor:
Margaret Strecker-McGraw


Editors In Chief:
Jessica Snowden
Asif Noor
H Davies


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Frank Smeeks
Kristina Soman-Faulkner
Trevor Nezwek
Radia Jamil
Patrick Le
Sobhan Daneshfar
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Navid Mahabadi
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
3/16/2019 1:14:07 PM

Introduction

Cerebrospinal fluid (CSF) is a clear protein- and glucose-rich liquid in the subarachnoid space of the central nervous system. It is found in the ventricles, surrounding the brain, and within the central spinal column. CSF regulates central nervous system temperature, cushions the brain and spinal cord, and provides a delicately balanced buoyant force that allows the brain to retain its shape and circulatory integrity despite its weight and lack of intrinsic rigid support.[1] A leak in this system, therefore, can be detrimental to brain blood supply and function and can increase the risk of direct trauma to brain parenchyma due to loss of fluid cushion. Additionally, the presence of CSF leak indicates the need for further evaluation and management as it may be due to a frontobasilar or temporal skull fracture. Open communication of the subarachnoid space with CSF leak presents a pathway for life-threatening CNS infection including meningitis. 

Etiology

A Cerebrospinal fluid leak can occur whenever there is open communication between the subarachnoid space and other spaces via meningeal disruption. The most common cause of leaking cerebrospinal fluid is a structural compromise secondary to craniofacial trauma, making up 80% of CSF leaks. Iatrogenic causes make up 16% of CSF leaks, with the last 4% of leaks due to varied etiologies, including spontaneous leak and congenital defects.[2]

Craniofacial trauma can lead to varied presentations of CSF leak, determined primarily by injury location and mechanism of action. Anterior skull base fractures are frequently associated with moderate-to high-velocity impact. They are, thus, more commonly associated with CSF leak compared to injuries to other locations due to the generally more extensive force of trauma, such as involvement in a motor vehicle accident. The cribriform plate, ethmoid bone, and frontal and sphenoid sinuses are thin and closely associated with the dura. Thus, trauma can easily disrupt both osseous structures and dura. Less frequently, fractures of the temporal bone are associated with dural disruption which can also result in CSF leak. Rarely, injury and disruption to the orbit can result in CSF occulorrhea.[3] 

Iatrogenic CSF leaks occur most frequently as sequelae to functional endoscopic sinus surgery with the cribriform plate and ethmoid bone being the most commonly injured, followed by the frontal and sphenoid sinuses.[2][4] Neurosurgical interventions also contribute to iatrogenic leaks, especially with the increased prevalence of endoscopic transnasal pituitary surgeries. In one study, pituitary tumor resection made up nearly half of cases where tumor removal led to confirmed CSF leak.[4] Spinal CSF leaks may also occur after certain procedures such as lumbar punctures, epidural anesthesia, or spinal surgery.

Epidemiology

Spontaneous CSF leaks occur without an obvious inciting event. Increasingly, spontaneous leaks are attributed to underlying conditions that result in increased intracranial pressure (ICP) such as idiopathic intracranial hypertension (idiopathic intracerebral hypertension -pseudotumor cerebrii)). This condition is frequently associated with obesity and female sex; In some studies, 60-70% of subjects presenting with spontaneous leak were female and BMI was overweight or obese; in two studies the mean BMI was approximately 35.[4][5][6] These leaks were most likely secondary to erosion of thin bony structures discussed above due to chronically increased ICP.

History and Physical

As most CSF leaks are secondary to either accidental or iatrogenic trauma, the history of a patient presenting with rhinorrhea or otorrhea should raise suspicion for CSF leak; thus one should obtain a recent history of trauma or a surgical procedure. The most common presenting symptom across all causes is clear rhinorrhea that may be accompanied by a headache. In those with spontaneous leaks, presentation frequently includes a pressure-like headache that may be positional in nature as well as pulsatile tinnitus.[7]

Evaluation

Evaluation of a suspected leak should include testing of rhinorrhea or otorrhea for beta-2 transferrin, a compound found only in CSF and perilymph making it a highly specific and sensitive test. If negative, there is a low likelihood of a leak.[8][9]

Glucose testing of rhinorrhea liquid was more frequently done in the past but has poor sensitivity and specificity when compared to beta-2 transferrin.

If beta-transferrin is positive during an acute leak or if there is a high index of suspicion, imaging is indicated to localize the source. High-resolution CT (HRCT) of paranasal sinuses and temporal bone is typically sufficient for identifying single osseous defects. If multiple defects are suspected on HRCT, CT cisternography is useful to localize the lesions further If initial CT is suspicious for meningoencephalocele, magnetic resonance cisternography is highly sensitive for soft tissue findings. If the leak is intermittent but active, HRCT is still the first line imaging test of choice, but if the suspected leak is inactive at the time of imaging, consider contrast-enhanced MR cisternography or radionucleotide cisternography.[10] 

In cases of chronic CSF leak or intracranial hypotension, MRI of the brain may show intracranial pachymeningeal thickening and enhancement with contrast, subdural fluid collections, and downward displacement of the brain. MRI of the spine can also show dural collapse and may demonstrate CSF leakage from spinal dural defects.[11]

Treatment / Management

Cases of CSF leak are managed according to their etiology. In cases of craniofacial trauma, it has been posited that since a number of these resolve with no intervention that conservative management and observation should be employed. However, the risk of developing meningitis in these patients is up to 29%, so this course should be pursued with caution.[12] For cases of spontaneous leak due to elevated intracranial pressures, multiple therapies have been employed. These include acetazolamide, the use of lumbar shunts or repeat lumbar punctures to lower ICP, endoscopic repair, and in cases of refractory or particularly high ICP, ventriculoperitoneal (VP) shunts. VP shunts can be effective but have relatively high complication rates.[4]

In cases of iatrogenic injury during intracranial surgery, the obvious treatment is repair of the affected site which may require multiple procedures to fix.[13] In cases with basilar skull fractures that are surgically managed, an endonasal endoscopic approach has a first attempt success rate ranging from 80 to 91%. The remaining cases may need further endoscopic revision with less than 10% of cases requiring open surgical revision.[6] Overall success rates range from 99 to 100%.[4][6] The surgical failure rate is higher among those with elevated ICP.[14]

Differential Diagnosis

The presentation of clear rhinorrhea and/or a headache is common to many conditions. Those that should be specifically considered are allergic rhinitis, infectious etiologies such as the common cold, vasomotor rhinitis, spontaneous intracranial hypotension, subarachnoid hemorrhage, carotid or vertebral artery dissection, benign intracranial hypotension, posttraumatic headache, and meningitis.[15][16]

Prognosis

As discussed in Treatment/Management, overall prognosis of CSF leak is very favorable, with one study citing 90% first attempt success rate of endoscopic repair of CSF rhinorrhea, and another study citing an overall success rate of 98% across traumatic, iatrogenic, and congenital causes.[17][4]

Complications

The most serious potential complication of CSF leak is meningitis. This risk appears to be highest in the preoperative period for those with confirmed CSF rhinorrhea with those sustaining traumatic injuries being at the highest risk of around 30%. However, the risk of meningitis remains at 19% in those with persistent CSF leak and such persons remain at risk until successful operative closure.[18]

Deterrence and Patient Education

Usefulness of deterrence and patient education is limited due to the varied etiologies as well as the common presenting symptoms of a headache and clear rhinorrhea being nonspecific. However, public health education regarding the prevention of trauma such as motor vehicle accidents as well as reduction of obesity would likely lead to a decreased number of traumatic or obesity-related CSF leaks.

Enhancing Healthcare Team Outcomes

CSF leak is a condition that involves many areas of healthcare. Initial presentation of a patient may occur at any level of care center and any provider, including physicians, nurse practitioners, and physician assistants. Laboratory technicians interpret CSF samples collected for testing. Radiologic technicians are instrumental in obtaining proper studies which are then interpreted by radiologists. Surgeons, interventional radiologists, and surgical technicians then provide definitive operative management. Throughout the presentation, workup, and treatment of a patient, pharmacists review and nurses administer medications and monitor patient vital signs. Because many of these patients' hospital stays may be prolonged, effective communication is important for preventing medical errors and reducing patient harm. 


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Cerebrospinal Fluid Leak - Questions

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After a facial fracture, what is the best way to differentiate cerebrospinal fluid from nasal secretion?



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Which cause of cerebrospinal fluid (CSF) leakage is the least likely to close spontaneously?



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A 5-year-old boy is referred with middle ear fluid on one side. He has had no acute infections. When the physician performs a myringotomy in preparation for tube placement, a profuse flow of spinal fluid ensues. What is the best course of action for this patient?



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A 5-year-old boy is referred with middle ear fluid on one side. He has had no acute infections. When the physician performs a myringotomy in preparation for tube placement, a profuse flow of spinal fluid ensues. The child is found postoperatively to have only a conductive hearing loss with no sensorineural component. Which of the following is MOST likely to be noted on CT scan?



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A patient presents complaining of several days of rhinorrhea. In which condition would one expect this rhinorrhea to be unilateral?



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If a trauma patient has clear fluid draining from the nose the provider should do which of the following?



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Which of the following may occur with spinal CSF leak?



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A 28 year old female presents with a severe generalized headache that started when she got out of bed this morning. The pain is much worse when upright and she lies down in the waiting room. She complains of mild nausea and slightly decreased hearing but no other symptoms. The exam is normal. Select the most likely diagnosis.



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A 28 year old female who is 23 weeks pregnant presents with a severe generalized headache that started when she got out of bed this morning. The pain is much worse when upright and she lies down in the waiting room. She complains of mild nausea and slightly decreased hearing but no other symptoms. The exam is normal. Select the best test for diagnosis.



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A patient presents two days after a motor vehicle collision with airbag deployment. The patient is complaining of persistent rhinorrhea and positional headache. What is the best initial imaging?



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Select the correct statement about traumatic CSF leaks and meningitis.



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Detection of a cerebral spinal fluid leak would not be reasonable with which imaging modality?



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A trauma victim with a head injury has clear fluid coming from his right nare. A glucose test of the fluid is ordered. What is true about the sensitivity and specificity of this test?



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A 40-year-old woman presents to the emergency department for a headache described as worse when she sits or stands upright and relieved when she lays down. Her headache began one day ago, a few hours after being involved in a motor vehicle crash where she experienced severe whiplash. An MRI of her head shows an increase in intracranial venous volume and a downward displacement of the brain. What is the diagnosis?



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Cerebrospinal Fluid Leak - References

References

Huff T,Dulebohn SC, Neuroanatomy, Cerebrospinal Fluid null. 2018 Jan     [PubMed]
Baugnon KL,Hudgins PA, Skull base fractures and their complications. Neuroimaging clinics of North America. 2014 Aug     [PubMed]
Le C,Strong EB,Luu Q, Management of Anterior Skull Base Cerebrospinal Fluid Leaks. Journal of neurological surgery. Part B, Skull base. 2016 Oct     [PubMed]
Banks CA,Palmer JN,Chiu AG,O'Malley BW Jr,Woodworth BA,Kennedy DW, Endoscopic closure of CSF rhinorrhea: 193 cases over 21 years. Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery. 2009 Jun     [PubMed]
Englhard AS,Volgger V,Leunig A,Meßmer CS,Ledderose GJ, Spontaneous nasal cerebrospinal fluid leaks: management of 24 patients over 11 years. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery. 2018 Oct     [PubMed]
Martínez-Capoccioni G,Serramito-García R,Martín-Bailón M,García-Allut A,Martín-Martín C, Spontaneous cerebrospinal fluid leaks in the anterior skull base secondary to idiopathic intracranial hypertension. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery. 2017 May     [PubMed]
Wang EW,Vandergrift WA 3rd,Schlosser RJ, Spontaneous CSF Leaks. Otolaryngologic clinics of North America. 2011 Aug     [PubMed]
Hiremath SB,Gautam AA,Sasindran V,Therakathu J,Benjamin G, Cerebrospinal fluid rhinorrhea and otorrhea: A multimodality imaging approach. Diagnostic and interventional imaging. 2018 Jun 14     [PubMed]
Meco C,Oberascher G,Arrer E,Moser G,Albegger K, Beta-trace protein test: new guidelines for the reliable diagnosis of cerebrospinal fluid fistula. Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery. 2003 Nov     [PubMed]
Bachmann G,Achtelik R,Nekic M,Michel O, [Beta-trace protein in diagnosis of cerebrospinal fluid fistula]. HNO. 2000 Jul     [PubMed]
Platt MP,Parnes SM, Management of unexpected cerebrospinal fluid leak during endoscopic sinus surgery. Current opinion in otolaryngology     [PubMed]
Bernal-Sprekelsen M,Bleda-Vázquez C,Carrau RL, Ascending meningitis secondary to traumatic cerebrospinal fluid leaks. American journal of rhinology. 2000 Jul-Aug     [PubMed]
Mirza S,Thaper A,McClelland L,Jones NS, Sinonasal cerebrospinal fluid leaks: management of 97 patients over 10 years. The Laryngoscope. 2005 Oct     [PubMed]
Knight A, The differential diagnosis of rhinorrhea. The Journal of allergy and clinical immunology. 1995 May     [PubMed]
Schievink WI, Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA. 2006 May 17     [PubMed]
Daele JJ,Goffart Y,Machiels S, Traumatic, iatrogenic, and spontaneous cerebrospinal fluid (CSF) leak: endoscopic repair. B-ENT. 2011     [PubMed]
Daudia A,Biswas D,Jones NS, Risk of meningitis with cerebrospinal fluid rhinorrhea. The Annals of otology, rhinology, and laryngology. 2007 Dec     [PubMed]
Chiapparini L,Ciceri E,Nappini S,Castellani MR,Mea E,Bussone G,Leone M,Savoiardo M, Headache and intracranial hypotension: neuroradiological findings. Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology. 2004 Oct;     [PubMed]

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