CEREBROSPINAL FLUID RHINORRHEA

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CEREBROSPINAL FLUID RHINORRHEA

• Nino Zaya, MD
• May 4, 2006

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Objectives

• Understand the classification system for various
  causes of CSF rhinorrhea.
• Understand the pathophysiology and diagnosis of
  CSF rhinorrhea.
• Review diagnostic testing techniques (chemical
  markers and CSF tracers) as well as localization
  studies.
• Review both medical and surgical strategies in
  treatment of CSF rhinorrhea.

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Case EA

• EA is a 55 y.o. female referred to Dr. Garcia
  with Sx suggesting ETD. She also c/o unilateral
  rhinorrhea occurring on the left side. No
  previous history of head and neck surgery, or
  trauma. She has had intermittent headaches
  present. The unilateral rhinorrhea has been
  present for 3 years with no improvement with
  allergy medications. Other history
  non-contributory.
• Physical exam Well-nourished female NAD, 160
  pounds, 52
• Ears weber-left ear, minimal effusion on
  left ear. R ear nl.
• Nose Anterior rhinoscopy negative. Prone,
  head-down position with valsalva lead to
  significant left-sided rhinorrhea. Fluid was
  collected for analysis. Remainder of patients
  exam was negative.

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Definition

• Cerebrospinal fluid (CSF) rhinorrhea results from
  a direct communication between the CSF-containing
  subarachnoid space and the mucosalized space of
  the paranasal sinuses.

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Historical Perspective

• First reported in the 17th century.
• Dandy in 20th century, reported first successful
  repair utilizing a bifrontal craniotomy for
  placement of a fascia lata graft.
• Extracranial approaches introduced mid-20th
  century.
• Endoscopic approaches were introduced and
  popularized in the 1980s and early 1990s.

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Classification of CSF Rhinorrhea

• Based on established pathophysiology of CSF
  rhinorrhea
• This has important clinical implications for the
  selection of treatment strategies and patient
  counseling about prognosis.
• Initial schemes-traumatic leaks and nontraumatic
  leaks.
• Accidental Trauma-80 of all CSF rhinorrhea
• Non-traumatic-4 of all CSF rhinorrhea.
• Procedure related-16 of all CSF rhinorrhea.

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Continued.

• Traumatic  
• Accidental    
• 1. Immediate   
• 2. Delayed  
• Surgical    
• 1. Complication of neurosurgical
  procedures      
• a. Transsphenoidal hypophysectomy     
• b. Frontal craniotomy      
• c. Other skull base procedures    
• 2. Complication of rhinologic procedures      
• a. Sinus surgery     
• b. Septoplasty      
• c. Other combined skull base procedures

8
Continued.

• Nontraumatic  
• A. Elevated intracranial pressure    
• 1. Intracranial neoplasm    
• 2. Hydrocephalus      
• a. Noncommunicating      
• b. Obstructive    
• 3. Benign intracranial hypertension  
• B. Normal intracranial pressure    
• 1. Congenital anomaly    
• 2. Skull base neoplasm      
• a. Nasopharyngeal carcinoma      
• b. Sinonasal malignancy    
• 3. Skull base erosive process      
• a. Sinus mucocele and Osteomyelitis    
• 4. Idiopathic

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Pathophysiology

• CSF produced by choroid plexus (20 mL/hour).
• CSF circulates from ventricles through foramina
  Luschka and Magendie to subarachnoid space.
• Total CSF volume is 140 mL20 mL (ventricles)
  50 mL (intracranial subarachnoid space) 70 mL
  (paraspinal subarachnoid space).
• CSF pressure ranges 40 mm H2O (infants) - 140 mm
  H2O (adults).

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Continued.

• CSF pressure maintained by relative balance
  between CSF secretion (choroid plexus) and CSF
  resorption (arachnoid villi).
• CSF resorption rate plays major role in
  determining CSF pressure.
• CSF rhinorrhea requires disruption of barriers
  that normally separate the contents of the
  subarachnoid space from the nose and paranasal
  sinuses
• Pressure gradient is also required to produce
  flow of CSF.

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Continued.

• Conditions with elevated ICP and associated CSF
  rhinorrhea.
• Nontraumatic CSF rhinorrhea
• Benign Intracranial Hypertension (BIH)
• Empty Sella Syndrome (ESS)

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Continued.

• Abnormalities bony architecture of skull base and
  CSF rhinorrhea.
• Lateral lamellar of the cribriform plate (LLCP)

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Continued.
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Continued.
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Continued.

• Meningocele or meningoencephalocele may occur in
  association with CSF rhinorrhea.
• Obtain imaging studies prior to blind biopsies.

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Continued.

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Continued.
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Differential Diagnosis

• CSF otorrhea presents as CSF rhinorrhea
• Sinonasal saline irrigations
• Seasonal perennial allergic rhinitis
• Vasomotor rhinitis

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History

• Unilateral watery nasal discharge (laterality)
• Salty taste.
• Positional variation.
• History trauma or surgery.
• Weight loss.
• Presence of inflammatory paranasal sinus disease.
• Headache.
• History of single or multiple episodes bacterial
  meningitis.

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Physical Examination

• Position testing.
• Halo sign.
• Glistening moist nasal mucosa on side of CSF
  leak.
• Clear fluid stream.
• Papilledema.
• Abducens nerve palsy.
• Traumatic CSF rhinorrhea and physical stigmata of
  recent or distant maxillofacial trauma.

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Continued..
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Diagnostic Testing

• 2 types of testing
• Identification substance serves as marker CSF.
• Agent administration that documents communication
  (intradural and extradural space).

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Continued.

• Chemical markers
• Glucose
• Beta-2 transferrin
• CSF Tracers
• Visible dyes (Intrathecal fluorescin)
• Radionuclide markers (Radioactive iodine (I131)
  serum albumen (RISA), technetium (99mTc)-labeled
  serum albumen and diethylenetriamterinepentaacetic
  acid (DTPA), and Indium (In111)-labeled DTPA)
• Radiopaque dyes (metrizamide)

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Continued.
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Continued.
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Continued.
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Localization Studies

• Limitations
• Radionuclide cisternography
• Poor spatial resolution.
• MR cisternography
• Long scan acquisition times required that produce
  thick image slices that cannot identify small
  skull base defects.
• CT cisternography (Metrizamide)
• Difficult to reliably interpret, even with slices
  of 1 mm.
• All above studies assume presence active CSF flow
  (intermittent or very small leaks may not be
  identified)
• Nasal endoscopy after intrathecal fluorescin
  infusion

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Continued.
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Continued.
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Continued.
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Management

• Multidisciplinary approach
• Otolaryngologist
• Neurosurgeon
• Neuroradiologist
• Infectious disease specialist

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Continued.

• CONSERVATIVE TREATMENT OF CSF RHINORRHEA
• Subarachnoid drainage through a lumbar catheter
• Strict bed rest
• Head elevation
• Stool softeners
• Patient advised to avoid coughing, sneezing, nose
  blowing, and straining

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Continued.

• Transcranial Techniques
• After craniotomy, defect site identified, and
  tissue graft placed to close the defect.
• Materials used Fascia lata grafts, muscle plugs,
  and pedicled galeal flaps may be used.
• A tissue sealant, such as fibrin glue, may be
  used to hold the grafts into position.

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Continued.

• Access to the cribriform plate region and roof of
  the ethmoid requires a frontal craniotomy.
• Extended craniotomy and skull base techniques
  with even greater brain compression provide
  access to the sphenoid sinus defects.
• Potential morbidities include brain compression,
  hematoma, seizures, and anosmia.
• High failure rates (25) despite direct access.

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Continued.

• Extracranial Techniques
• Endoscopic repair of CSF rhinorrhea provides
  adequate visualization of defect.
• Intrathecal fluorescin facilitates defect
  identification.
• Prepare defect site for grafting.
• Bipolar cautery or KTP laser used to fulgurate
  any coincidental meningoencephalocele.
• Mucosa within 5 mm of the margins of the skull
  base defect must be removed to facilitate mucosal
  grafting.

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Continued.

• Graft material
• Temporalis fascia, fascia lata, muscle plugs,
  pedicled middle turbinate flaps (mucosa alone or
  mucosa and bone), autogenous fat, free cartilage
  grafts (from the nasal septum or the
  cartilaginous auricle), and free bone grafts
  (from the nasal septum or calvarium).
• Acellular dermal allograft.
• Higher failure with with pedicled intranasal
  grafts versus free grafts.

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Continued.

• Underlay technique
• Larger defects require layered reconstruction
  less risk of delayed recurrence and
  meningoencephalocele formation.

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Continued.

• Never place mucosal grafts intracranially
  (intracranial mucocele after repair can occur).
• Surgical sealant (fibrin glue) may be used to
  help hold the grafts in place.
• Absorbable nasal packing is placed adjacent to
  the grafts, and nonabsorbable packing used to
  support absorbable packing.

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Continued.
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Continued.
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Continued.

• Pure endoscopic approaches provide excellent
  access to the ethmoid roof, cribriform plate, and
  most of the sphenoid sinus.
• Lateral sphenoid leaks may require an extended
  approach, which incorporates endoscopic
  dissection of the medial pterygomaxillary space.
• Osteoplastic flap or a simple trephine might be
  required for repair of defects through the
  posterior table of the frontal sinus.

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Continued.

• Postoperative care includes strict bedrest for
  several days and antistaphylococcal antibiotics.
• Observation in ICU for first 24 hours.
• Continue lumbar drain for 4 to 5 days.
• Nasal packing removed after several days.
• Operative site may be checked through serial
  nasal endoscopy.
• Patients advised to avoid strenuous activity,
  sneezing, coughing for 6 weeks after repair.
• Primary cases successful repair 85-90
• Secondary endoscopic repair also has high
  likelihood of success.

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Continued.

• Endoscopic techniques offer several advantages.
• Excellent visualization afforded by nasal
  endoscopy facilitates identification of the
  defect and graft placement.
• Endoscopic repair is also well tolerated,
  especially compared with intracranial techniques.
  
• Report outcomes are excellent for both primary
  and secondary endoscopic repairs.

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Management Strategy

• Indications
• Failed conservative management
• Intraoperative recognition of a leak (during
  sinus surgery, skull base surgery, and
  craniotomy)
• Large defects/leaks (especially in association
  with pneumocephalus)
• Idiopathic leaks (spontaneous leaks)
• Open traumatic head wounds with CSF leakage

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Continued.

• Traumatic (Nonsurgical) Etiology
• Conservative measures (reduces ICP and promotes
  spontaneous closure).
• Persistent rhinorrhea-explore and repair.
• Extracranial endoscopic techniques and open
  transcranial procedures (massive head injury
  requiring urgent operative exploration) might be
  warranted.

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Continued.

• Intraoperative Injury with Immediate Recognition
• CSF leaks noted intraoperatively should be
  repaired immediately during FESS.
• Intracranial and skull base procedures include
  deliberate violations of the dura provide a
  watertight seal at the end of the procedure.

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Continued.

• Operative Injury with Delayed Recognition
• Conservative therapy for a few days warranted
  since some leaks will close.
• Can pursue operative intervention for massive
  leaks early.
• Significant delay between time of surgery and CSF
  leak diagnosis-conservative measures less
  successful, and early surgical intervention
  warranted.

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Continued.

• Nontraumatic Leaks
• Usually require surgical repair.
• Can attempt conservative measures.
• Treat underlying etiology along with CSF
  rhinorrhea (neoplasm, hydrocephalus, etc.).
• Always consider unrecognized elevation of ICP
  (ESS or BIH) in cases of spontaneous CSF leaks.
  
• Operative repair in ESS and BIH usually
  necessary.

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Case EA Revisited..

• Patient EAs fluid analysis-positive Beta-2
  transferrin.
• CT-Scan showed fluid/soft tissue in left sphenoid
  sinus.
• CSF tracer study utilizing intrathecal omnipaque
  along with CT scanning-positive in left sphenoid
  sinus for CSF leak.

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Case Continued.
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Case Continued.

• Patient taken to operating room and underwent
  left sphenoidotomy with closure of CSF leak.
• Small pinpoint defect in left sphenoid sinus had
  been identified.
• Fascia lata and lateral rectus muscle were
  utilized for closure along with fibrin glue.
• Patient had intraop lumbar drain placed for
  decompression by Neurosurgery
• Post-operatively-CSF leak resolved and area where
  leak located healed.

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Conclusions

• Categorize leaks
• Beta-transferrin assay and several CSF tracer
  studies available, but have limitations.
• High-resolution CT provides detailed information
  about the bony skull base anatomy
• MR assesses soft tissue issues, including
  unrecognized tumors and coincidental
  meningoencephaloceles.
• Many CSF leaks respond to conservative management
  (observation plus measures to minimize ICP).
• Traumatic CSF rhinorrhea tends to resolve with
  conservative measures alone.
• Nontraumatic CSF rhinorrhea require operative
  repair.
• Extracranial techniques are first line for CSF
  rhinorrhea.

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Bibliography

• Halo sign http//connection.lww.com/Products/timby
  essentials/Ch41.asp
• Cummings Otolaryngology Head and Neck Surgery.
  Chapter 55. CSF Rhinorrhea
• Fluorescin CSF Leak http//www.geocities.com/shous
  er144/csf.html