Cerebrospinal Fluid Rhinorrhea: Diagnosis and Management: Julie T. Kerr, MD, Felix W.K. Chu, MD, Stephen W. Bayles, MD
Cerebrospinal Fluid Rhinorrhea: Diagnosis and Management: Julie T. Kerr, MD, Felix W.K. Chu, MD, Stephen W. Bayles, MD
Cerebrospinal Fluid Rhinorrhea: Diagnosis and Management: Julie T. Kerr, MD, Felix W.K. Chu, MD, Stephen W. Bayles, MD
38 (2005) 597–611
Cause
Traumatic leaks are subdivided as surgical (whether planned or
unplanned) or nonsurgical (whether blunt or penetrating). Seventy percent
to 80% of CSF rhinorrhea is caused by accidental trauma [3]; 2% to 4% of
acute head injuries result in CSF rhinorrhea [4].
Nontraumatic CSF rhinorrhea includes high-pressure and normal-
pressure leaks. High-pressure CSF rhinorrhea comprises 45% of non-
traumatic CSF rhinorrhea, and 84% of these leaks result from tumor
obstruction. The remainder is caused by either benign intracranial
hypertension or hydrocephalus. Normal-pressure leaks may result from
bony erosion by tumor, tumor treatment with radiation therapy, arachnoid
granulations, infection, empty sella syndrome, or congenital defects such as
preformed pathways, fistulas, meningoceles, meningoencephaloceles, or
encephaloceles.
* Corresponding author.
E-mail address: [email protected] (F.W.K. Chu).
0030-6665/05/$ - see front matter Ó 2005 Elsevier Inc. All rights reserved.
doi:10.1016/j.otc.2005.03.011 oto.theclinics.com
598 KERR et al
Localization
Key in management of CSF leakage is localization of the dural defect,
which can originate from the anterior, middle, or posterior cranial fossas.
The most common site of accidental traumatic fracture seems to be at the
cribiform plate where the bone is thick, the area adjacent is thin, and
the dura is very adherent [25]. Congenital defects most commonly arise from
the superior or lateral walls of the sphenoid sinus or from the cribriform
niche adjacent to the middle turbinate vertical attachment.
Multiple imaging studies are available to help localize sites of dural
defects. The sensitivities and specificities of these studies vary with patient
population, defect size, operator interpretation, and leak flow rate.
600 KERR et al
Conservative management
Most CSF leaks resulting from accidental and surgical trauma heal with
conservative measures over the course of 7 to 10 days. Less likely to heal
spontaneously are leaks in which CSF rhinorrhea develops days or weeks
after surgical or accidental trauma, massive leaks that develop immediately
after surgery, leaks caused by sustain gunshot wounds, or normal-pressure
CSF leaks. CSF fistulae found at the time of endoscopic sinus surgery
require repair at the time of initial surgery [4]. Leaks noted 5 to 7 days after
surgery may close spontaneously; if there is not resolution in 1 to 2 weeks,
surgery is indicated.
First-line treatment includes bed rest with head elevation, avoidance of
straining activity such as nose blowing, sneezing, and coughing, and the use
of stool softeners.
Antibiotic prophylaxis remains controversial. Most authors avoid
antibiotics to reduce development of resistant organisms [40,41]. Friedman
[42] found the incidence of meningitis after accidental trauma to be 10% in
those treated with antibiotics versus 21% in those not treated. Pappas [6]
602 KERR et al
found that, given the greater incidence of resistant organisms with use of
prophylactic antibiotics, surgical management is the only definitive treat-
ment. If there is gross contamination along a fluid pathway, such as with
a comminuted fracture of the paranasal sinus resulting from acute trauma,
antibiotic prophylaxis does have a role.
A nontraumatic, high-pressure CSF leak caused by increased intracranial
pressure will probably resolve if the intracranial pressure is normalized. The
intracranial pressure can be normalized by use of diuretics such as
acetazolamide or with ventriculoperitoneal shunting. Leaks that do not
resolve with normalization of intracranial pressures warrant surgical
management [43].
Surgical management
Numerous factors are involved in the surgical management of CSF leaks.
These factors include use of a lumbar drain, the approach for repair, the type
of graft or flap and its placement, and the use of sealant and nasal packing.
Lumbar drain
Controversy surrounds use of a lumbar drain [44–48]. Use of a sub-
arachnoid lumbar drain or serial lumbar punctures is controversial, as well.
If resolution has not occurred after 72 hours in a patient managed
conservatively, draining 150 cm3/day of CSF for 4 additional days before
entertaining surgical options may be beneficial [49]. Lumbar drainage is not
without risk, however. Overdrainage may create a siphon effect with
resultant pneumocephalus. Additional complications include headache,
nausea, vomiting, vocal cord paralysis, occlusion of the posterior cerebral
artery, and lumbar radiculopathy. Hegazy’s [46] meta-analysis documents
that lumbar drainage does not affect success rates. Casiano [44] reports
a 97% success rate in more than 30 patients without use of lumbar drain.
Sixty-seven percent of otolaryngologists use lumbar drainage routinely and
drain for an average of 4 days [48].
Komisar et al [50] recommend drainage if there is suspicion for increased
intracranial pressure. In long-term or congenital leaks, a lumbar drain may
offset the initial rise in intracranial pressure. Suspicion for increased
intracranial pressure is warranted with leak recurrence [51]. Preoperative
intracranial pressure reading will be inaccurate because of the leak, but an
accurate reading can be taken postoperatively.
Surgical approach
Transcranial
Dandy [52], in 1929, was the first to document successful repair of a CSF
leak using an intracranial approach. Success rates ranging from 60% to
CEREBROSPINAL FLUID RHINORRHEA 603
95% have been reported [53–55]. Spetzler et al [56] experienced a failure rate
of 27% on initial operation and a 10% overall failure rate with multiple
procedures.
Advantages of this approach include improved exposure, ability to
identify multiple defects, and ability to tamponade a leak in a high-pressure
situation. Drawbacks include the inherent increased morbidity, increased
length of hospitalization, and permanent anosmia.
Extracranial
Dohlman [57] was the first to document a successful extracranial repair of
a CSF leak in 1948. Success rates of 86% on initial operation with a 97%
success rate overall have been documented [58,59].
Extracranial repair results in decreased morbidity, no anosmia, improved
endonasal exposure of the sphenoid, parasellar and posterior ethmoids,
cribriform plate, fovea ethmoidalis, and the posterior wall of the frontal
sinus. Inherent to this procedure is a facial scar, risk for facial numbness,
and orbital complications. The procedure can be quite cumbersome. Cere-
bral damage and the lateral extensions of the frontal and sphenoid sinuses
cannot be assessed.
Transnasal
A transnasal approach for closure of CSF rhinorrhea was first described
by Hirsch [60] in 1952. Lehrer and Deutsch [61] improved visualization with
use of the microscope, but visualization of the lateral and superior walls of
the sphenoid sinus is limited. Transnasal approaches risk facial numbness as
well as septal perforation. With use of endoscopes, these approaches are
rarely used today.
Endoscopic
In 1981 Wigand described closure of a cerebrospinal fistula using an
endoscopic approach. Endoscopic intranasal fistula repair is now the
preferred approach, with higher success rates and less morbidity than
intracranial surgical repair [62,63].
Multiple studies have documented high success rates with a wide variety
of grafting materials and adjuncts to closure such as lumbar drain, tissue
sealant, and nasal packing. Regardless of materials used, success rates of
92% to 96% have been documented [38,62,64,65]. Hegazy’s [46] meta-
analysis of 575 cases demonstrates a 90% success rate on first attempt and
a 96% success rate on second attempt.
Generally, a small defect can be closed with an overlay free mucosal graft or
a free fascial graft. The free mucosal grafts can be acquired from the inferior
turbinate or the septum. Fascia can be obtained from the temporalis region or
fascia lata. It is important that, after identification of the bony defect, mucosa
surrounding the perimeter of the defect be removed to stimulate osteogenesis,
thus thickening bone around defect and improving graft incorporation.
604 KERR et al
Sealant
Fibrin, created by a combination of fibrinogen, thrombin, and calcium
cofactor, is the most commonly used sealant today. Tisseel is a fibrin sealant
that includes components of autologous cryoprecipitate from single-donor
plasma, bovine thrombin, and an antifibrinolytic agent that yields a more
stable clot. This fibrin sealant provides a temporary watertight closure and
creates an additional barrier to CSF leakage during wound healing and
fibrosis.
Packing
Packing materials can be either absorbable or nonabsorbable. Whether
packing is used and which type is used is the surgeon’s choice. Senior et al
[48] reported that 86% of rhinologists use packing to repair CSF leaks.
Hegazy’s [46] meta-analysis reported that 60% of patients were packed
postoperatively. No study to date has determined a difference in outcome
with use of packing.
Special considerations
Spontaneous encephaloceles have a higher rate of treatment failure [80]. In
these cases, cranial bone graft may be more successful, because intracranial
pressure is likely to be elevated whether the defect is small or large. Bone graft
606 KERR et al
Follow-up
When repair of a defect is near a sinus outflow tract, there is risk that
normal sinus drainage will be obstructed. This obstruction may sub-
sequently result in mucocele formation. Postoperative follow-up with CT
scanning is appropriate to rule out development of a mucocele when there is
concern for obstruction [82]. Meticulous care to ensure that the non-
epithelialized surface of the graft is in contact with the intracranial cavity
can prevent unplanned outflow obstruction. The risk of obstruction is
unavoidable, however, when the leak approximates an outflow tract.
Summary
Advances in imaging and endoscopic techniques have improved the
ability to diagnose, localize, and treat in a less morbid fashion CSF leaks of
the anterior skull base.
An appreciation for the mechanism of leak and of the relationship
between CSF production and absorption must be kept in mind when
individualizing a repair. Increased CSF pressure caused by overproduction
or underabsorption may result in persistence of a leak despite one’s best
efforts.
Numerous advances in dural replacement grafts and tissue sealants have
improved the ability to achieve watertight closure of the cranial vault.
Microvascular techniques have allowed larger defects previously not
reconstructable to be handled with relative ease by trained personnel. With
expanded reconstructive techniques, the ability to handle larger disease
processes of the skull base continues to expand.
608 KERR et al
References
[1] Har-El G. What is ‘‘spontaneous’’ cerebrospinal fluid rhinorrhea? Classification of
cerebrospinal fluid leaks. Ann Otol Rhinol Laryngol 1999;108:323–6.
[2] Ommaya AK, Di Chiro G, Baldwin M, et al. Non-traumatic cerebrospinal fluid rhinorrhea.
J Neurol Neurosurg Psychiatry 1968;31:214–55.
[3] Bernal-Sprekelsen M, Bleda-Vazquez C, Carrau RL. Ascending meningitis secondary to
traumatic cerebrospinal fluid leaks. Am J Rhinol 2000;14:257–9.
[4] Mincy J. Post-traumatic spinal fluid fistulas of the frontal fossa. J Trauma 1966;6:618–22.
[5] Rice DH. Cerebrospinal fluid rhinorrhea: diagnosis and treatment. Curr Opin Otolaryngol
Head Neck Surg 2003;11:19–22.
[6] Pappas DG, Hammerschlag PE, Hammerschlag M. Cerebrospinal fluid rhinorrhea and
recurrent meningitis. Clin Infect Dis 1993;17:364–8.
[7] Sibler H. The normal cerebrospinal fluid proteins identified by means of thin-layer isoelectric
focusing and crossed immnunoelectrofocusing. J Neurol Sci 1978;36:273–88.
[8] Ridley F. The intraocular pressure and drainage of the aqueous humor. Br J Exp Pathol
1930;11:215–40.
[9] Skedros DG, Cass SP, Hisrch BE, et al. Sources of error in use of beta-2 transferrin analysis
for diagnosing perilymphatic and cerebral spinal fluid leaks. Otolaryngol Head Neck Surg
1993;109:861–4.
[10] Oberashcer G. A modern concept of cerebrospinal fluid diagnosis in oto and rhinorrhea.
Rhinology 1988;26:89–103.
[11] Ryell RG, Peacock MK, Simpson DA. Usefulness of beta 2-transferrin assay in the detection
of cerebrospinal fluid leaks following head injury. J Neurosurg 1992;77:737–9.
[12] Roelandse FWC, Van de Zwart AZJ, Didden JH, et al. Detection of CSF leakage by
isoelectric focusing on polyacrimide gel, direct immunofixation of transferrins and silver
staining. Clin Chem 1998;44:351–3.
[13] Seiler RW, Mariani L. Sellar reconstruction with resorbable Vicryl patches, gelatin foam,
and fibrin glue in transphenoidal surgery: a 10 year experience with 376 patients. J Neurosurg
2000;93:762–5.
[14] Ciric I, Ragin A, Baumgartner C, et al. Complications of transsphenoidal surgery: results of
a national survey, review of literature and personal experience. Neurosurgery 1997;40:
225–36.
[15] Black PM, Zervas NT, Candia GL. Incidence and management of complications of
transphenoidal operation for pituitary adenomas. Neurosurgery 1987;20:920–4.
[16] Koltai PJ, Goufman DB, Parnes SM, et al. Transphenoidal hyophysectomy through the
external rhinoplasty approach. Otolaryngol Head Neck Surg 1994;111:197–200.
[17] Jane JA, Laws ER. The surgical management of pituitary adenomas in a series of 3093
patients. J Am Coll Surg 2001;193:651–9.
[18] Jho H. Endoscopic transphenoidal surgery. J Neurooncol 2001;54:187–95.
[19] Lawson W. The intranasal ethmoidectomy: evolution and assessment of the procedure.
Laryngoscope 1994;104(Suppl 64):1–25.
[20] May M, Levine HL, Mester SJ, et al. Complications of endoscopic sinus surgery: analysis of
2108 patientsdincidence and prevention. Laryngoscope 1994;1040:1080–3.
[21] Park JI, Strelzow VV, Friedman WH. Current management of cerebrospinal fluid
rhinorrhea. Laryngoscope 1983;93:1294–300.
[22] Schlosser RJ, Bolger WE. Spontaneous nasal cerebrospinal fluid leaks and empty sella
syndrome: a clinical association. Am J Rhinol 2003;17:91–6.
[23] Levin S, Nelson KE, Spies HW, et al. Pneumococcal meningitis: the problem of the unseen
cerebrospinal fluid leak. Am J Med Sci 1972;264:319–27.
[24] Jacob JB, Persky MS. Traumatic pneumocephalus. Laryngoscope 1980;90:515–20.
[25] Som ML, Kramer R. Cerebrospinal rhinorrhea pathological findings. Laryngoscope 1940;
50:1167.
CEREBROSPINAL FLUID RHINORRHEA 609
[26] Lloyd MNH, Kimber PM, Burrows EH. Post traumatic cerebrospinal fluid rhinorrhea:
modern high definition computed tomography is all that is required for the effective
demonstrated of the site of leakage. Clin Radiol 1994;49:100–3.
[27] Bateman N, Jones NS. Rhinorrhoea feigning cerebrospinal fluid leak: nine illustrative cases.
J Laryngol Otol 2000;114:462–4.
[28] El Gammal T, Brooks BS. MR cisternography: initial experience in 41 cases. AJNR 1994;15:
1647–56.
[29] Chow JM, Goodman D, Mafee MF. Evaluation of CSF rhinorrhea by computerized
tomography with metrizamide. Otolaryngol Head Neck Surg 1989;100:99–105.
[30] Manelfe C, Cellerier P, Sobel D, et al. Cerebrospinal fluid rhinorrhea: evaluation with
metrizamide cisternography. AJR Am J Roentgenol 1982;138:471–6.
[31] Stone JA, Castillo M, Neelon B, et al. Evaluation of CSF leaks: high resolution CT
compared with contrast enhanced CT and radionuclide cisternography. AJNR Am J
Neuroradiol 1999;20:706–12.
[32] Manelfe C, Guirand B, Tremoulet M. Diagnosis of CSF rhinorrhea by computerized
cisternography using metrizamide. Lancet 1997;2:1073.
[33] Flynn BM, Butler SP, Quinn RJ, et al. Radionuclide cisternography in the diagnosis and
management of cerebrospinal fluid leaks: the test of choice. Med J Aust 1987;146:82–4.
[34] Stammberger H. Endoscopic sinus surgery. Philadelphia: BC Decker; 1991.
[35] Lund VJ, Savy G, Lloyd GAS. Optimum imaging and diagnosis of cerebrospinal fluid
rhinorrhea. J Laryngol Otol 2000;114:395–7.
[36] Stammberger H. Special problems in functional endoscopic sinus surgery. Philadelphia:
B Dekker; 1991. p. 437–40.
[37] Keerl R, Weber RK, Draf W, et al. Use of sodium fluorescein solution for detection of
cerebrospinal fluid fistulas: analysis of 420 administrations and reported complications in
Europe and the United States. Laryngoscope 2004;114:266–72.
[38] Zweig JL, Carrau RI, Celin SE, et al. Endoscopic repair of CSF leaks to the sinonasal tract:
predictors of success. Otolaryngol Head Neck Surg 2000;123:195–201.
[39] Sillers MJ, Morgan E, El Gammal T. Magnetic resonance cisternography and thin coronal
computerized tomography in the evaluation of cerebrospinal fluid rhinorrhea. Am J Rhinol
1997;11:387–92.
[40] Rathor MH. Do prophylactic antibiotics prevent meningitis after basilar skull fracture?
Pediatr Infect Dis J 1991;10:87–8.
[41] Stankiewicz JA. Cerebrospinal fluid fistula and endoscopic sinus surgery. Laryngoscope
1991;101:250–6.
[42] Friedman JA, Ebersold MJ, Quast LM. Post traumatic cerebrospinal fluid leakage. World
J Surg 2001;25:1062–6.
[43] Briant TDR, Bird R. Extracranial repair of cerebrospinal fluid fistulae. J Otolaryngol 1982;
11:191.
[44] Casiano RR, Jassir D. Endoscopic cerebrospinal fluid rhinorrhea repair: is a lumbar drain
necessary? Otolaryngol Head Neck Surg 1999;37:33–6.
[45] Marshall AN, Jones NS, Robertson IJA. An algorithm for the management of CSF
rhinorrhea illustrated by 36 cases. Rhinology 1999;37:182–5.
[46] Hegazy HM, Carrau RL, Snyderman CH, et al. Transnasal endoscopic repair of
cerebrospinal fluid rhinorrhea: a meta analysis. Laryngoscope 2000;110:1166–72.
[47] Chin G, Rice D. Transnasal endoscopic closure of cerebrospinal fluid leaks. Laryngoscope
2003;113:136–8.
[48] Senior BA, Jafri K, Benninger M. Safety and efficacy of endoscopic repair of CSF leaks:
a survey of the members of the American rhinologic society. Am J Rhinol 2001;15:21–5.
[49] Cooper PR. Skull fracture and traumatic cerebro-spinal fluid fistulas in head injury. In:
Cooper PR, editor. Head injury. Baltimore (MD): Williams and Wilkins; 1982.
[50] Komisar A, Weitz S, Ruben RJ. Cerebrospinal fluid dynamics and rhinorrhea: the role of
shunting in repair. Otolaryngol Head Neck Surg 1983;91:399–403.
610 KERR et al
[77] Disa J, Rodriquez VM, Cordeiro PG. Reconstruction of lateral skull base oncological
defects: the role of free tissue transfer. Ann Plast Surg 1998;41:633–63.
[78] Thompson JG, Restifo RJ. Microsurgery for cranial base tumors. Clin Plast Surg 1995;22:
563–72.
[79] Fisher J, Jackson IT. Microvascular surgery as an adjunct to craniomaxillofacial
reconstruction. Br J Plast Surg 1989;42:146–54.
[80] Gassner HG, Ponikau JU, Sherris DA, et al. CSF rhinorrhea: 95 consecutive surgical cases
with long term follow up at the Mayo Clinic. Am J Rhinol 1999;13:439–47.
[81] Jones DT, McGill TJ, Healy GB. Cerebrospinal fistulas in children. Laryngoscope 1992;
102:443–6.
[82] Delfini R, Missori P, Iannetti G, et al. Mucoceles of the paranasal sinuses with intracranial
and intraorbital extension: report of 28 cases. Neurosurgery 1993;32:901–6.
[83] Spetzler RF, Herman JM, Beals S, et al. Preservation of olfaction in anterior craniofacial
approaches. J Neurosurg 1993;79:48–52.
[84] Shiley SG, Limonadi F, Delashaw JB, et al. Incidence, etiology, and management of
cerebrospinal fluid leaks following trans-sphenoidal surgery. Laryngoscope 2003;113:
1283–8.
[85] Cappabianca P, Cavallo LM, Esposito F, et al. Sellar repair in endoscopic endonasal
transsphenoidal surgery: results of 170 cases. Neurosurgery 2002;51:1365–71.
[86] Dessi P, Castro F, Triglia JM, et al. Major complications of sinus surgery: a review of 1192
procedures. J Laryngol Otol 1994;108:212–5.