4. DEFINITION
Leakage of CSF into the nose
Clear fluid or fluid mixed with blood
Rare but potentially devastating condition
History:
Dandy(1926): first surgical repair of CSF leak via frontal
craniotomy approach
Wigand (1981): use of endoscope for the first time to
assist with repair of skull base defect
[ 90-95% success rate with decreased associated morbidity – thus preferred] 4
6. ETIOLOGY
A. Congenital:
Defective anterior neuropore closure
Embryologic defect-patent fonticulus frontalis or
foramen cecum
B. Acquired:
1. Traumatic (9O%)
2. Iatrogenic
3. Idiopathic/ Spontaneous
4. Certain tumors
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7. Traumatic CSF Rhinorrhea:
90% of CSF rhinorrhea
Anterior > middle > posterior cranial fossa
can be:
Immediate (< 48 hours) - Motor Vehicle Accidents (MVA)
Delayed (in 3 months) - proposed different theories
Intact dural layer slowly herniated through bony defect
Finally tearing & CSF leak
Another Theory:
Bony defect & dural tear +nt from the time of original injury
Leakage once masking hematoma resolves
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8. Iatrogenic CSF Rhinorrhea:
Any surgical manipulation near skull base
i. Neurosurgical:
most commonly craniotomy & trans-sphenoidal pitutary resections
ii. Otolaryngological:
FESS (lateral lamella of cribriform plate, fovea ethmoidalis, posterior
aspects of frontal recess)
Septoplasty
Present as:
Simple cracks in bony architecture
Large (>1 cm) defects
Presents in within 1st week of insult 8
9. Idiopathic/ Spontaneous CSF Rhinorrhea:
Without antecedent cause
Site: anterior cranial fossa mostly
subjected to wide variations of CSF pressure due to normal arterial
and respiratory fluctuations
Cause: secondary to raised ICP
Idiopathic Intracranial Hypertension (IIH)
Obstructive Sleep Apnea (OSA)
Stressors: Valsalva like manoeuvers during nose blowing or straining
Other causes: Focal atrophy, persistent embryonic olfactory lumen,
rupture of arachnoid projections accompanying olfactory nerves
9
10. Congenital Tumors:
1. Locally aggressive: Inverted papilloma
2. Malignant neoplasms: eg. Glioma
Erode bone of anterior cranial fossa
Inflammation and potential violation of dura
10
11. PRESENTATION TO HOSPITAL
Clear watery unilateral/ bilateral nasal discharge
Anosmia- in some traumatic cases
Congenital:
Furstenberg sign:
Meningoencephaloceles in child as intranasal/ extranasal mass
Transilluminates and expands with crying (high index of suspicion with
midline mass) No biopsy till complete imaging
CSF otorhinorrhea: in some traumatic/ iatrogenic cases
Idiopathic/ Spontaneous: headache 11
12. PHYSICAL EXAMINATIONS
o Multimodality approach:
o Reservoir sign:
headache relieved due to CSF drainage externally intermittently
from one of the accumulated paranasal sinuses with the change in
head position
Queckenstedt Stooky test:
On endoscopy, CSF drainage is elicited by patient having perform
Valsalva manoeuver or by compressing both jugular veins
Double ring/ Halo sign:
CSF detected from mixed blood when placed on filter paper with clinically
detectable ring
Rhinologic: Endoscopy Otologic Head and neck Neurologic evaluation
12
14. DIFFERENTIAL DIAGNOSES
Allergic Rhinitis
Vasomotor Rhinitis
Differences between CSF Rhinorrhea & Nasal secretions:
Features CSF Rhinorrhea Nasal secretions
History Trauma/ Surgery/ Tumor Sneezing, stuffinesss
Flow Drops/ stream, not sniffed back Continuous, sniffed back
Character Clear, watery Slimy
Taste Sweet Salty
Sugar +nt >30 mg/dl <10 mg/dl
Beta2
transferrin
Always present Always absent
14
15. LABORATORY VALUES
Rapid but highly
unreliable
Not for screening or
confirmation
False +ve: d/t
reducing substance
in nasolacrimal
secretions
False –ve: acute
meningitis
92% sensitive, 100%
specific
PG-D synthase
Production: Arachnoid
cells, oligodendrocytes
& choroid plexus
Altered by:renal
failure, multiple
sclerosis, cerebral
infarction, CNS tumors
Glucose Content Beta-trace protein Beta-2 transferrin
Highly sensitive & specific
Rapid, Non-invasive
Currently single best lab
test to identify +nce of
CSF in sinonasal fluid
Produced by
neuraminidase activity
Present in CSF +
Perilymph + Aqueous
humor
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16. IMAGING TECHNIQUES
1. CT-Scan:
o HRCT
imaging of choice
Axial plane with <1 mm thickness & reformatted into coronal and
sagittal plane
Congenital defects- best diagnosed with 3-D reconstruction
o CT-cisternography: intrathecal contrast to identify leak easily
2. MRI: less diagnostic
3. Nuclear Medicine Studies (Serial scanning/ Scintiphotography):
using DTPA, Radioactive Iodine-131
16
22. Endoscopic technique:
Most widely used
General concepts:
Decongestion to maximize visualization (1:1000 epinephrine or
4% cocaine solution)
Specific endoscopic approach:
Designed to gain access to the area of interest in most efficient
fashion
Approaches:
Transfrontal
Transcribriform
Transplanum
Transsellar
Transclival
Transpterygoid
22
23. FOLLOW UP
Non absorbable packing: removed on day 7-10 of operation
Regular endoscopic examination & minimal debridement of
surgical site to r/o recurrence of encephalocele/ CSF leak
Close f/u in raised ICP
Acetazolamide use:
Close electrolyte monitoring
Ventriculoperitoneal shunt if intolerable S/Es
23
24. COMPLICATIONS
1. Meningitis: most feared & severe
i. Bacterial:
S. pneumoniae, H. influenzae
In first 3 weeks with trauma (10%) & non-trauma(40%)
ii. Aseptic:
Meningeal irritation due to manipulation during surgical repair
2. Anosmia
OUTCOME & PROGNOSIS:
Recurrence type Average time from repair
Most leak 2 years
Spontaneous leak 7 months
Traumatic leak 4 months (50% in 2 weeks) 24
25. REFERENCES
1. Image-Based Case Studies in ENT and Head &
Neck Surgery by Rahmat Omar & Prepageran
Narayanan
2. Diseases of Ear, Nose & Throat by PL Dhingra &
Shruti Dhingra 6th Edition
3. An illustrated text book Ear, Nose & Throat and
Head & Neck Surgery by Rakesh Prasad
Shrivastav 2nd Edition
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