Non-traumatic CSF leak: protocol of
management according to clinical
presentation
Retrospective clinical study
:‫رشح السائل الدماغي غير الناتج عن الصدمات‬
.‫الخطة العالجية بناءا على االعراض السريرية‬
‫دراسة سريرية باثر رجعي‬
Authors and co-authors:
1-Dr.Amer Abdelrazzaq Alshurbaji (Corresponding Author)
Consultant neurosurgeon.
Chief of neurosurgery department royal medical services.
Amman-Jordan.
.‫عامر عبدالرزاق الشربجي‬.‫د‬-1
.‫مستشار جراحة دماغ و اعصاب‬
.‫الخدمات الطبية الملكية‬/‫رئيس اختصاص جراحة الدماغ و االعصاب في مدينة الحسين الطبية‬
.‫االردن‬/‫عمان‬
:‫رقم تلفون‬00962777243998
:‫ ايميل‬[email protected]
2-Dr.Zuhir Abu Salma.
Specialist of neurosurgery in royal medical services.
Amman-Jordan.
.‫زهير ابو سلمى‬.‫د‬-2
‫اخصائي جراحة دماغ و اعصاب في مدينة الحسين الطبية‪/‬الخدمات الطبية الملكية‪.‬‬
‫عمان‪/‬االردن‪.‬‬
‫‪00962779128836‬رقم تلفون‪:‬‬
‫‪[email protected]‬ايميل‪:‬‬
‫‪3-Dr.Raed Aljboor.‬‬
‫‪Specialist of neurosurgery in royal medical services.‬‬
‫‪Amman-Jordan.‬‬
‫‪-3‬د‪.‬رائد الجبور‪.‬‬
‫اخصائي جراحة دماغ و اعصاب في مدينة الحسين الطبية‪/‬الخدمات الطبية الملكية‪.‬‬
‫عمان‪/‬االردن‪.‬‬
‫رقم تلفون‪00962795053638:‬‬
‫‪[email protected]‬ايميل‪:‬‬
‫‪4-Dr.Rakan Ahmed Allozi.‬‬
‫‪Specialist of neurosurgery in royal medical services.‬‬
‫‪Amman-Jordan.‬‬
‫‪-4‬د‪.‬راكان احمد اللوزي‬
‫اخصائي جراحة دماغ و اعصاب في مدينة الحسين الطبية‪/‬الخدمات الطبية الملكية‪.‬‬
‫عمان‪/‬االردن‪.‬‬
‫‪00962779551100‬رقم تلفون‪:‬‬
‫‪[email protected]‬ايميل‪:‬‬
Non-traumatic CSF Leak: Protocol of Management according to clinical
presentation
Amer Al-Shurbaji,Zuhair Abu Salma,Raed Al-Jboor,Rakan AL-Lawzi
Royal Medical Services, King Hussien Hospital Amman-Jordan
Objective: Spontaneous CSF leak is a relatively uncommon disease as compared
to other causes of CSF leak, and can lead to meningitis; our aim in this study is to
share our experience in the evaluation and management of these cases.
Patients and methods: This is a retrospective study of 50 consecutive patients
(21 male :29 female)who presented with spontaneous CSF leak from March 2003
to March 2013 and were managed in KHMC, the investigations, site of leakage,
line of management taken, and mortality and morbidity were reviewed and
studied.
Results: All our patients presented by spontaneous rhinorreah, six female
patients were diagnosed to have Idiopathic Intracranial Hypertension (IIH) and
one male had obstructive hydrocephalus and all were treated by CSF diversion.
43 patients had CT/MR cisternography: in 16 patients the site of leak was
apparent and were subjected to direct repair, 27 patients were treated medically
and with limitation of activity for 7to10 days, seven cases needed external
lumbar drainage (ELD) and of these two needed permanent CSF diversion. No
treatment related mortality nor morbidity.
Conclusion: Good results can be achieved in managing cases of spontaneous CSF
leak and can preclude the occurrence of meningitis, this can be achieved by
proper evaluation of the cause and site of leakage. Leaks secondary to high
intracranial pressure are managed by diversion procedure, while detectable
defects are best managed by direct repair and in other cases a conservative
management is appropriate.
Introduction: Galen1 was the first physician to describe a traumatic CSF leak in
the second century AD but it wasn’t until 1826 when Miller2 reported the first
non-traumatic CSF leak. Its relative rare occurrence (4% of all CSF leaks 1)
compared to the majority of leaks due to trauma or recently to iatrogenic causes
(3% of traumatic head injuries have leaks3 and 0.5% of all endonasal procedures
are complicated by leaks4) makes the optimal management of these cases
challenging, we present our experience in dealing with these cases, our protocol
and results.
Patients and Methods:
This is a retrospective study of 10 years duration (from March2003 to March
2013) of patients presenting with CSF spontaneous rhinorrhea, patients who had
previous trauma, radiation, recurrent sinus infection, endo -nasal surgery or
proven to have invasive base skull tumors were excluded from the study.
Fifty patients were included in this study 21 males (age 16-55 years mean age
42 years) and 29 females (age 18-65 years mean age 43 years), all of which had a
general neurological examination, fundoscopy and visual field assessment. All
patients had a general MRI to rule out a sellar lesion, signs of sinus infection,
hydrocephalus or indirect signs of raised intra cranial pressure such as: slit
ventricles, empty sella, flattening of the posterior sclera, or thrombosis of dural
sinuses.
Patients who did not meet criteria of CSF leak secondary to tumors, infections or
Idiopathic Intracranial Hypertension (IIH) were subjected to radiological
localization of the defect, from 2003 to 2012 the localization was done using CT
cisternography; LP injection of a water soluble contrast agent (lopamidol, 8ml
diluted with 10ml of N/S), patient in prone position, Water’s view CT acquisition
3x3mm/0.9x0.9mm reconstruction. Since 2012 non –invasive MRI
cisternography were done with the patient in the prone position, thin T2
weighted coronal slices and CISS sequence were taken.
Patients who showed signs of IIH were managed by Acetazolamide 250 mg x3
and thecoperitonal shunt insertion,Patients who didn’t show signs of IIH and had
no apparent defect on cisternography were put also on Acetalzolamide for one
week as inpatient and for another four weeks as out patient, lumbar drain was
inserted for non responders for a period of 7-10 days with broad spectrum
antibiotics coverage and discharged on Acetazolamide for four weeks, patients
who had recurrence of the leak after discontinuation of the drain were subjected
to theco-peritoneal shunt insertion.
Direct surgical repair was done only for those patients who didn’t show signs of
IIH and had an obvious defect on cisternography, two approaches were used: an
endo-nasal approach for leaks related to the sphenoid sinus and a frontolateral
approach for defects in the anterior cranial fossa, patients post op were started
on Acetazolamide for 4 weeks and no external lumbar drain was inserted except
for one redo case and one patient had an external ventricular drain.
Results: Six female patients met the criteria of IIH both clinically and
radiologicaly and were treated by theco-peritoneal shunt, one male patient was
found to have obstructive hydrocephalus and a medium pressure ventriculoperitoneal shunt was inserted.
Forty-three patients underwent CT/MRI cisternography; twenty seven didn’t
show an obvious defect and didn’t meet the criteria of IIH, they were managed by
the tiers of conservative management described above, of these seven did not
stop the leak after the third day of initiating the treatment and needed an
external lumbar drain for seven to ten days, two of these patients had recurrence
of the CSF leak after discontinuation of the drain and had theco-peritoneal shunt.
Sixteen had obvious defects on cisternography ; six patients had a defect
related to the sphenoid sinus: 4of which were from the lateral sphenoid wall
(figure 1) and were approached via microscopic endo-nasal approach and the
sinus was packed with abdominal fat after patching the defect with Surgicel and
Gelfoam ,one of these cases had a persistent leak which was apparent after
removing the nasal pack he underwent a redo surgery in which a fascia lata graft
was applied to the defect packed with abdominal fat and needed an external
lumber drain (ELD) for 5 days. one case had the defect in the posterior wall of
the sinus i.e. :the upper third of the clivus and had a large aerocele, the sinus was
packed with abdominal fat and two external ventricular drains were kept for 10
days5,in one case the defect was in the planum sphenoidale (figure 2) and was
managed by the same route. In the other ten cases the defect was in the anterior
cranial fossa two were in the fovea ethmoidalis (figure 3)and in eight they were
lateral to the cribriform plate, all were approached by Transcranial route
(frontolateral approach) extra dural dissection, no post operative external
drainage was needed.
No treatment related mortality or morbidity were observed in either the
conservatively treated patients or the surgically treated ones, no late CSF leak
recurrence was observed. Table 1 summarizes the line of management.
Discussion: Non traumatic, non lesional spontaneous CSF rhinorrhea is probably less
than 4%1,though the exact pathophysiology is unknown speculations include a long
standing continuous or intermittent intracranial pressure6, or congenital weak points7,
the resulting osteodural defect must communicate with a preexisting cavity such as the
nasal sinuses to give rise to the clinical condition.
The necessity of treating CSF leaks arises from the risk of meningitis, with the risk in
acute post traumatic leaks of 25% and 57%8 for the delayed leaks, the rarity of the
spontaneous leaks precluded knowing the exact percentage of meningitis but it should
be laying between these two values.
The radiological workup in these patients aims at recognizing leaks associated with
increased intra-cranial pressure and the site of leakage, CT cisternography has 40-90%
sensitivity if the CSF leak is active9 and in inactive leaks the sensitivity drops to 27%10,
non invasive MRI cisternography raises the sensitivity to 87%11,in our study we
detected 16 leaks out of 43 (37%) these cases were investigated mainly by CT
cisternography while our adaptation of non invasive MRI cisternography is recent .
Our policy in the treatment is first to rule out a treatable cause of the leak such as IIH,
or hydrocephalus which was the case in one of our patients. The other objective is to
find a source of the leak so as to choose the appropriate route to approach it and to seal
the defect. In cases without a detectable defect they are managed first by diuretics
mainly carbonic anhydrase inhibitor (Acetazolamide) which reduces the CSF production
by 50%, and if there isn’t a response, an external lumbar drain is inserted for seven to
ten days the rational of which is to decrease or divert the CSF away from the defect
giving the defect time to heal, in non-responders a permanent diversion of the CSF is
needed in the form of theco-peritoneal shunt.
In 1884, Chiari was the first to demonstrate a fistulous connection between the
subarachnoid space and an ethmoidal defect in a patient who died from meningitis .12
Surgical repair was not done until Dandy first repaired a defect in 1926 13, our
experience in treating defects in the anterior cranial fossa is to isolate the dural defect
via extradural dissection and freeing the dura of the bone, fulgurating the part of the
brain herniating through the defect, and then the dural defect is primarly repaired if
possible , if not, a Surgicel is applied on the brain surface and a large piece of Gelfoam
covers the defect in the dura ,the bony defect is plugged by a piece of abdominal fat.
Sphenoid sinus related leaks are managed by endo-nasal approach, in which the
sphenoid sinus is opened, detecting the defect and applying Surgicel to the exposed
dura, then packing of the sinus with abdominal fat.
The defects in the anterior cranial fossa were first approached via the endonasal route
by Dohlman in 194814, frequent studies showed efficacy of this approach in cribriform
and ethmoidal defects15,16. Our opinion dealing with defects in the anterior cranial fossa
are best approached by transcranial route because there is no need for a long osseous
work to delineate the defect, while exposing the defect via endonasal route needs
ethmoidectomy and might need a soft tissue pediculated graft 17.
Number of patients with their corresponding line of management shown in table 1
The strategy of dealing with spontaneous is summarized in algorithm 1
Conclusion: Spontaneous CSF leak is an uncommon disease and is difficult to manage;
appropriate diagnosis of leaks secondary to increased intra cranial pressure directs
management towards CSF diversion, while in other cases the ability to localize o the
defect directs surgical versus conservative approach which proved to be successful in
many cases.
Appendix 1: Figures
Figure 1: A defect in the left lateral wall of the sphenoid sinus
Figure 2: A defect in the planum sphenoidale
Figure 3: A defect in the left fovea ethmoidalis
Appendix 2: tables
Table 1
Medications
External Theco-peritoneal shunt Direct repair
or ventriculo-peritoneal
drain
shunt
Hydrocephalus
0
0
1
0
IIH
6
0
6
0
+ve defect
0
2
0
16
-ve defect
27
7
2
0
Appendix 3: algorithm
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