CEREBROSPINAL FLUID RHINORRHEA

Nino Zaya, MD May 4, 2006

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.

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.

Definition

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

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.

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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A.

B.

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

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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

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 mL=20 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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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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Conditions with elevated ICP and associated CSF rhinorrhea.

1. 2. 3.

Nontraumatic CSF rhinorrhea Benign Intracranial Hypertension (BIH) Empty Sella Syndrome (ESS)

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Abnormalities bony architecture of skull base and CSF rhinorrhea.

1.

Lateral lamellar of the cribriform plate (LLCP)

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

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Differential Diagnosis

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

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.

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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Diagnostic Testing

2 types of testing:
1.
2.

Identification substance serves as marker CSF. Agent administration that documents communication (intradural and extradural space).

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Chemical markers
1.
2.

Glucose Beta-2 transferrin

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)

CSF Tracers
1. 2.

3.

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Localization Studies

Limitations
1.
2.

Radionuclide cisternography

Poor spatial resolution. Long scan acquisition times required that produce thick image slices that cannot identify small skull base defects. Difficult to reliably interpret, even with slices of 1 mm.

MR cisternography

3. 4.

CT cisternography (Metrizamide)

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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Management

Multidisciplinary approach:
1.
2. 3. 4.

Otolaryngologist Neurosurgeon Neuroradiologist Infectious disease specialist

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CONSERVATIVE TREATMENT OF CSF RHINORRHEA

1. 2. 3. 4. 5.

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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Transcranial Techniques
1.

2.

3.

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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5.

6.

7.

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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Extracranial Techniques
1.
2.

3.
4. 5.

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

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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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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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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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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.

Management Strategy

Indications
1.
2.

3.

4.

5.

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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Traumatic (Nonsurgical) Etiology

1.

2.

3.

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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Intraoperative Injury with Immediate Recognition

1.

2.

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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Operative Injury with Delayed Recognition

1.

2.

3.

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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Nontraumatic Leaks
1.
2. 3.

4.

5.

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.

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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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.

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.

Bibliography

Halo sign http://connection.lww.com/Products/timbyess entials/Ch41.asp Cummings Otolaryngology: Head and Neck Surgery. Chapter 55. CSF Rhinorrhea Fluorescin CSF Leak http://www.geocities.com/shouser144/csf.html