Independent Researcher, Paterson, NJ 07533, USAUnited States
Also available as PDF (figures and tables appear in the PDF).
Objective: The objective of this study was to present a review article about petrous apex cere-brospinal fluid (CSF) leak. Data Sources: Published English-language literatures in PubMed and Google Scholar. Review Methods: PubMed and Google Scholar were systematically searched using search terms: petrous, apex, cerebrospinal and leak. Temporal, bone, cerebrospinal and leak. Study Selection: We included studies about petrous apex CSF Leak. Results: Seventeen studies were included in this study. The results showed that 72% of patients are adult and 28%of patients are children. Meningocele is the most common cause of petrous apex CSF leak in pediatric patients, while iatrogenic trauma is the most common cause in adult patients. Seventy-seven percentage of pediatric patients have active leak, while 96% of adult patients have active leak. Nose is the most common site of CSF leak in both adult and pediatric patients. Sixty-six percentage of pediatric patients have meningitis while only 20% of adults have meningitis. Most cases need surgical procedure. Eleven percentage of pediatric patients have a recurrence, while 20% of adult patients have a recurrence. Conclusion: Petrous apex is a rare location for CSF leak.
Keywords: Middle fossa approach; Transmastoid approach; Meningocele; Gorham-stout; Spontaneous CSF leak
Cerebrospinal fluid leak (CSF) from an intracranial source is rare, as it is a life-threatening
condition that can have difficulties in localization, diagnosis, and management. CSF leaks from
the petrous apex are extremely rare, as only few cases are reported in the world literature.
Surgery of petrous apex area has potentially high morbidity rate due to complex anatomy. Mul-
tiple surgical approaches have been developed for reaching petrous apex region (subtemporal,
transtemporal, endoscopic transnasal), all of them aiming to increase the anatomic exposure,
reduce the complication rates, and result in high successful treatment. Each approach has its
advantages and disadvantages. We in this review article discuss about the English literatures of
petrous apex CSF leak.1
All petrous apex CSF leak articles published after 1990 were
included in the study.
Sixteen studies about petrous apex CSF leak were available in
PubMed (MEDLINE) and Google Scholar in English literature
Rhinorrhea First approach MFA sealed with bone wax and cov-
1 B/l hearing loss ered with fascia second operation MFA The meningo- Kou et al 61 F spontaneous recurrent cele was reduced and the defect was plugged with an
meningitis abdominal fat graft and fibrin glue.
2 CSF Right Extradural MFA the defect packed with fat, covered Warade et al 26 M Rhinorrhea Meninogcele with fascia lata graft and fibrin glue.
Vertigo,
3 Headache, Right Morimoto et al 11 F fascia, periosteum flap, and sealed with fibrin glue. No Pharyngeal Pulsatile tinnitus. Gorham-stout
Hearing loss
4 Recurrent Grant et al 53 F meningitis
Profuse right-sided
56 M rhinorrhea, Right iatrogenic
Meningitis
CSf otorrhea Right Trauma
28 M Heaing loss
profound Acoustic neuroma
CSF 57 F transcochlear packed with abdominal fat. Proplast No Nose rhinorrhea
5 Recurrent MFA stuffed soft tissue and fascia in air cells with Motojima et al 6 F Right meningocele Meningitis fibrin glue for repair
Otorhinorrhea the fistula and CSF leak, obliteration of the eusta-
Dzaman et al 60 M Profound Right choleasteoma chian
hearing. canal, and obliteration of the middle ear and mastoid
Headache
Nausea 7 Right Tympanomastoidectomy, eustachian tube plugging, zygmotic Cushing et al 12 M Vomiting
Hearing loss
Facial palsy
(Table 1).
There were 33 patients of age ranged from 5 to 71. There were 9
pediatric patients, 6 patients of them were male 66%, while the
other 3 patients were female 33%. There were 24 adult patients,
12 patients of them were male 50% and 12 patients were female
50% (Chart 1).
Left cephalocele
Yes Nose
CSF leak
No Nose
Extradural MFA packed with superficial temporal Cervical
Medical treatment; interferon-alpha 2b area
First operation: an excision of the mastoid-cutaneous
Left Iatrogenic fistula tract, and the wound was closed with a tempo- Nose roparietal fascia flap.
Postauricul Yes ar CSF Acoustic neuroma Second operation: modified transcochlear petrous fistula apex. The distal eustachian tube was obstructed with
Proplast and abdominal fat placed in the cavity.
Conservative therapy. First:translabyrinthine oblitera-
tion of the mastoid with an abdominal fat graft. Head
of the malleus was packed into the Eustachian tube.
Yes Nose Second; transcochlear obliteration of the petrous
apex, Proplast was packed into the tube and fat used
to obliterate the petrous apex.
Lumbar drainage rest Ventriculoperitoneal shunt. First
oberation: This was managed with a radical
mastoidectomy and eustachian tube obliteration, Yes Middle ear Second: Transcochlear approach obliteration was
done with fat. The eustachian tube was occluded with
Proplast.
The CSF leak did not resolve with bed rest and
Right iatrogenic lumbar drainage. She underwent a right-sided
Acoustic neuroma was packed into the medial end of the eustachian
tube and abdominal fat packed into the cavity.
No Nose
Combined MFA and Trans-mastoid. Temporal fascia
graft, fibrin glue, and collagen patch tachoSil treated
tubal orifice, closure of the external auditory No Nose
clefts were essential in this procedure. Bony defects
were repaired through the use of an acrylic mass.
surgical site
No Gorham-stout middle ear and mastoid obliteration with fat root, TMJ
area
8 Right Isaacson et al 55 F Meningitis Middle fossa approach packing wax, fascia and muscle No Not active
Hearing loss
9 Latrogenic right Danner et al 49 F Otorhinorrhea Lumbar drain rest Transient No Nose
10 Latrogenic Sekhar et al 37M CSF Leak Lumbar drain No
42 F CSF Leak obliteration with fat, ET plugging, bone graft for defect, No
53 M Lumbar drain, rest peritoneal shunt (palliative) No One
46 F CSF Leak fascia, obliteration with fat, ET plugging, bone graft for No external ear
33 F CSF Leak
58 F CSF Leak Lumbar drain No
64 F CSF Leak fascia, obliteration with fat, ET plugging, bone graft for No
35 M CSF Leak Chondrosarc Lumbar drain wound No
43 M CSF Leak fascia, obliteration with fat, ET plugging, bone graft for No
46 F CSF Leak obliteration with fat, ET plugging, bone graft for defect, No
40 F Lumbar drain packing peritoneal shunt No
38 F CSF Leak Lumbar drain No
Ota et al 34 M Rhinorrhea Right iatrogenic Hydroxiappetiate for packing then fiber glue then piece No Nose
59 F Rhinorrhea Right iatrogenic Hydroxiappetiate for packing then fiber glue then piece No Nose
32 M Rhinorrhea Left iatrogenic hydroxiappetiate for packing then fiber glue then piece No
12 meningocele Hervey-Jumper et al 14 M meningitis fibrin glue. Second operation endoscopic trans nasal Yes
Headache approach
meningitis Extradural MFA, Multilayer defect coverage the
Facial and herniated brain and dura were resected and the dura
13 abduces palsy Left was closed primarily. The petrous apex was packed with Pross et al 5 F
hearing loss The defect was covered with another piece of temporalis
rhinorrhea fascia,
14 Right Iatrogenic Oyama et al 71 M Rhinorrhea Lumbar drainage, rest MFA muscle free flap No Nose
Otalgia
cephalocele
Meningioma
One throw
Mhordoma wound
Lumbar drain, rest reoperation packing multilayer, fascia, Latrogenic Nine throw
Meningioma middle ear fibrin glue for sealing
CSF Leak Latrogenic
Meningitis Chordoma
Lumbar drain, rest reoperation packing: multilayer, Through Latrogenic
Meningioma defect, fibrin glue for sealing one throw
Latrogenic Lumbar drain, rest nose repacking foreman flap for No Sphenoid Chordoma sphenoid
Latrogenic
Meningioma
Lumbar drain, rest reoperation packing multilayer, Latrogenic
Meningioma defect, fibrin glue for sealing
Latrogenic
oma
Lumbar drain, rest reoperation packing multilayer, Latrogenic
Meningioma defect, fibrin glue for sealing
Lumbar drain, rest reoperation packing multilayer, fascia, Latrogenic
Aneurysm fibrin glue for sealing
CSF Leak Latrogenic
meningitis Meningioma
Latrogenic
Meningioma
Lumbar drain trans-petrosal multilayer, HAC
of dura then fat then fiber glue
Lumbar drain trans-petrosal multilayer, HAC
of dura then fat then fiber glue
Lumbar drain Trans=petrosal multilayer, HAC Nose
middle ear of dura then fat then fiber glue
Bilateral Recurrent MFA Pericranium patch and fat graft, dural repair, and, Retro
s (larger on pharyngeal
the left)
Recurrent
No Nose Sudden meningocele gelfoam as support for temporalis fascia graft underlay.
calvarial bone graft, and synthetic dural substitute
Chiari
cholesteatoma
15 RIGHT CSF Right Moore et al 5 M cia graft underlay. The defect was covered with another No Nose
16 CSF LEAK Right obtained from the left ear. As a second layer, the Schick et al 12 M Meningitis meningocele prepared mucosal flap was used to cover the site of
17 Right Mulcahy et al 6 M Meningitis fascia graft underlay. The defect was covered with No Not active
9 M Meningitis fascia graft underlay. The defect was covered with No Not active
Side of CSF leak: Seven pediatrics patients had CSF leak from
right side 77%, one from left side 11%, and one had B/L CSF
leak 11%. Nine adult patients had CSF leak from right side 75%,
while the other 3 patients had CSF leak from left 25%.
Pediatrics patients: Four patients had CSF leak from nose, 3
patients had CSF leak in petrous apex surrounding areas (neck,
retropharyngeal, and zygomatic area), while the other 2 patients
had no active CSF leak (Chart 2).
MFA the dura was closed primarily. The petrous apex
was packed with gelfoam as support for temporalis fas-
leak meningocele piece of temporalis fascia, calvarial bone graft, and
synthetic dural substitute
Endoscopic transnasal approach perichondrium
No Nose
repair
MFA, TM The dura was closed primarily. The petrous
apex was packed with gelfoam as support for temporalis
meningocele another piece of temporalis fascia, calvarial bone graft,
and synthetic dural substitute
MFA, TM The dura was closed primarily. The petrous
apex was packed with gelfoam as support for temporalis Right
meningocele another piece of temporalis fascia, calvarial bone graft,
and synthetic dural substitute
Adult patients: Twelve patients had CSF leak from nose, 10 pa-
tients had CSF leak from middle ear, 2 patients had CSF leak
from the surgical wound, one from external auditory canal, and
one had no active CSF leak (Chart 3).
Pediatric patients: Seventy-seven percentage of patients had me-
ningocele (the most common cause in pediatric patient), while
the 22% patient had Gorham-stout syndrome which is lympho-
vascular proliferation malformation of bones (Chart 4).
Adult patients: Eighty-three percentage of patients had iatro-
genic trauma (the most common cause in adult patients), 8% of
patients had cephalcele, 4% of patients had external trauma, and
4% had spontaneous CSF leak (Chart 5).
Pediatrics: Seventy-seven percentage of patients had active CSF
leak, 66% of patients had history of meningitis, 33% of patients
had headache, and 33% of patients had hearing loss (cranial 8
nerve involvement) (Chart 6).
Adults: Ninety-six percentage of patients had active CSF leak,
20% of patients had history of meningitis, 8% of patients had
headache, and 16% of patients had cranial nerve palsy (CN VI,
CN VII and CN VIII) (Chart 7).
Pediatrics: Five patients had middle fossa approach (MFA), one
of them had a recurrence and had another endoscopic trans-nasal
approach to repair the CSF leak. Two patients had a combined
MFA and trams-mastoid (TM), one had transmastoid approach
(TMA) and one patient had trans-nasal approach (Chart 8).
Adult: Twenty-one patients had conservative management and
CSF leak continued in 17 patients of them, 2 patients had lum-
boperitoneal shunt, 6 patients had re-exploration of surgical
site with repacking, 4 patients had MFA and one of them had
recurrence and treated with MFA approach, and 6 patients had
trans-petrosal approach and 3 patients of them had recurrence
and treated with tras-petrosal approach (Chart 9).
Five patients had multilayer repair using fascia, fibrin glue, fat
packing to obliterate middle ear, Eustachian tube (ET) plugging
and bone graft to support defect. Five patients had multilayer re-
pair using primary dura closure, fascia, gelfom packing, and ET
pulgging and synthetic dura. Five patients had a fascia for du-
raplasty, ET plugging, fat obliteration and fibrin glue. Three pa-
tients had multilayer repair using hydroxyapatite packing, fiber
glue, fascia and fat. Three cases had fascia for durplasty, patched
with fascia and fibrin glue for sealing. One patient had only fas-
cia and bon wax, 2 had free flab. One patient had perichondrium
graft and nasal mucosal flap. One had fascia and packing with
muscle and bone wax sealed by fibrin glue. One patient had fis-
tula removal and packed with fascia (Table 2).
There was one recurrent case in pediatrics group 11%, and 4
recurrent cases in adult group 16% (Chart 10).
Cerebrospinal fluid (CSF) leak develops when there is a fistula
between the subarachnoid space and the aerated areas of the
temporal bone. Spontaneous leaks are commonly misdiagnosed
as chronic serous otitis media. Generally, the diagnosis is done
by the presence of β2-transferrin in the middle ear and nasal flu-
id. Meningitis is the most significant complication of persistent
CSF otorrhea or rhinorrhea. Common causes of petrous apex
CSF leaks include iatrogenic injury, congenital malformation,
meningocele, trauma, and choleasteoma and spontaneous CSF
leak 1.
Pelosi et al18 reported 14 cases of temporal bone CSF
leak, one of them was from petrous apex area. Patients with
spontaneous CSF leaks are usually females aged 40-60 with
body mass index (BMI) greater than 30. They should also be
evaluated for other problems associated with idiopathic intracra-
nial hypertension (IIH) (ophthalmologic, neurologic, and empty
sella). We should suspect this disease in patients with multiple
Multilayer
Multilayer fascia Fascia HAT One duraplasty,
oblitartion with fat, and fat for patient had Perichondrium packing with
ET plugging, bone packing, ET only fascia Free flab graft and nasal muscle and
graft, fibrin glue for plugging, and
sealing sealing with wax sealed by fibrin
fibrin glue
Patients 5 3 4 3 1 2 1 1 5 1
Recurrence 0 0 0 1 1 0 0 0 2 1
Table 2. Fistula repair.
meningitis and we review their imaging even if they have a neg-
ative β2 transferrin.
Cephalocele of petrous apex (PAC) is a rare lesion, it
extends into the petrous apex from Meckel’s cave. It is usually
asymptomatic in adults. CSF leak of PAC has only been seen in
children. PAC might make up of either one or all 3 layers of the
meninges. It could be congenital or acquired. The symptoms of
PAC are CSF rhinorrhea, otorrhea, trigeminal neuralgia, head-
ache and recurrent episodes of meningitis in children. Chronic
pulsations against the thin anterior wall of a pneumatized pe-
trous apex and raised intracranial pressure leading to dehiscence,
herniation of meninges and CSF leak through weak points in the
petrous apex. These lesions may be either unilateral or bilateral.
Magnetic resonance imaging (MRI) has a key role in diagnos-
ing these lesions as they follow CSF signal on all sequences and
that these directly communicate with Meckel’s cave. Treatment
is surgical removal for symptomatic cases and surgical approach
whether MFA or tranpetrosal is determined by patients hearing
ability (Figures 1 and 2).2
Gorham-Stout syndrome is a lymphovascular prolif-
eration of unknown etiology, lymph vessels usually do not pen-
etrate the temporal bone. Computed tomography (CT) findings
are helpful to assess the extent of bone destruction, while T2-
weighted MRI can show the extent of abnormal lymphovascular
proliferation. Contrast lymphangiography can be used to find
exactly the site of leakage. Meningitis secondary to CSF leakage
is a life-threatening complication of Gorham-Stout syndrome.
Surgical treatment does not prevent progression of the disease,
but it is effective. Gorham-Stout osteolytic lesions should be re-
moved minimally and carefully since the leakage increases in
some cases after biopsy. Anti-interleukin-6 (Anti-IL6) recep-
tor antibody and anti-vascular endothelial growth factor (anti-
VEGF), antibodies (bivacizumab) that decrease angiogenesis,
bisphosphonates that decrease osteoclast activity and bone re-
sorption, interferon propranolol can be used to treat this disease
(Figure 3).3
Iatrogenic trauma is the most common cause of petrous
apex CSF leak in our study, it is usually seen in pneumatized pe-
trous apex that have anatomic pathway between the petrous apex
and the medial ET. It is recommended to close ET and obliterate
middle ear and mastoid cavity when the patient has pneumatized
Multilayer, Fascia for primary dura Fascia for Fascia for
closure, durplasty, duraplasty ET Fistula
fascia, gelfom patched plugging, fat removal,
packing, fascia and obliteration, and packing bon mucosal flap bone wax, ET bulging, fibrin glue fibrin glue with fascia
synthetic for sealing sealing glue dura
petrous apex with that abnormal pathway. Surgical repacking us-
ing multilayered obliteration technique is recommended for the
cases that do not respond to conservative treatments (Figure 4).4
Petrosal cholesteatoma is rare entity, it may be primary
or acquired in the origin. Primary congenital cholesteatoma arise
from embryonary ectodermal inclusions but the pathogenesis
is not clear. Congenital cholesteatoma may be asymptomatic
or manifest as a conductive hearing loss, otalgia, vertigo and
facial palsy. CSF rhinorrhea is extremely rare manifestation of
cholesteatoma. Treatment is surgery via MFA or transpetrosal
depending on hearing abilities in symptomatic cases, a multi-
The most common cause Meningocele
Table 3. Petrous apex CSF leak conclusion.
layered obliteration technique in which artificial and autologous
materials are combined is the best modality of treatment with the
4 6 highest rate of success.6
Petrous apex CSF leak is more common in adults, iatrogenic
trauma is the most common cause in adult patients while me-
ningocele is the most common cause in pediatrics, CSF leak is
more from right side. Meningitis, cranial nerves involvement
and headache can also be associated with CSF leak. Multilayer
fistula repair is least likely to recurre (Table 3).
Percentage 27% 73%
Iatrogenic
Sex M<F M=F
Side Right<Left Right<Left
Active leak 77% 96%
Meningitis 66% 20%
Treatment Surgery Conservative management then Surgery
Recurrence 11% 16%
Dana library.
The author declared that he has no conflicts of interest.