|Year : 2012 | Volume
| Issue : 2 | Page : 58-61
Spontaneous CSF otorrhea, hidden and serious condition necessitate recognition of its presentation and the modalities of treatment
Bandar Al Qahtani
Department of Otolaryngology, King Saud Medical City, Riyadh, Kingdom of Saudi Arabia
|Date of Web Publication||6-Sep-2012|
Bandar Al Qahtani
Consultant Otologist Neuro-otologist, King Saud Medical City, Riyadh
Kingdom of Saudi Arabia
Source of Support: None, Conflict of Interest: None
Spontaneous cerebro spinal fluid (CSF) otorrhea or temporal CSF leakage represents a difficult and serious condition to deal with, in term of its diagnosis, especially with intact drum, management and its sequalae if not treated or diagnosed. the clinical presentation, the types of leaks and which are the best approaches to repair it are reviewed.
Keywords: CSF otorrhea, Temporal bone CSF, Temporal encephalocele
|How to cite this article:|
Al Qahtani B. Spontaneous CSF otorrhea, hidden and serious condition necessitate recognition of its presentation and the modalities of treatment. Indian J Otol 2012;18:58-61
|How to cite this URL:|
Al Qahtani B. Spontaneous CSF otorrhea, hidden and serious condition necessitate recognition of its presentation and the modalities of treatment. Indian J Otol [serial online] 2012 [cited 2019 Apr 23];18:58-61. Available from: http://www.indianjotol.org/text.asp?2012/18/2/58/100694
| Introduction|| |
Cerebro spinal fluid (CSF) middle ear effusion/otorrhea can develop in adults without a prior history of meningitis or head trauma or any apparent proximate cause. Although, presenting symptoms can be subtle, early suspicion and confirmatory imaging aid in establishing the diagnosis, Galbarriatu and other et al. ,,
It can be associated with or without skull-base defect  or cephalocoeles and can be further classified into congenital, spontaneous, or secondary (traumatic, iatrogenic or due tumor) Connor et al.  The majority is due to secondary related to trauma. 
Iatrogenic CSF leaks can seldomly be seen in any middle ear surgeries, especially with eroding pathology, Wootten et al.  CSF gusher is low, encountered in approximately 1% of patients undergoing cochlear implantation surgery.  Both, in cochlear malformations and in cochlear without apparent malformation, the incidence is almost the same but luckily successful implantation in these patients without short-term or long-term complications is expected. 
In the other hand, spontaneous CSF otorrhea is rare entity, , can be easily missed in adults and often not diagnosed until a myringotomy or tympanostomy tube is placed.
Any patients, especially adult older than 50 years with a negative history of otologic disease who have recurrent serious otitis media should be evaluated for this pathology.  There is no doubt that the condition needs immediate attention and correction because of risk of devastating meningitis infection. Usually, CSF leak can result from a defect in the dura and skull base.
CSF leakage can trickle down through the Eustachian tube More Details to be presented as CSF otorhinorrhea or nasal discharge.
Any clear pulsating fluid through myringotomy should arise the suspicion of CSF otorrhea and encephalocele, which is a protrusion of intracranial contents can include brain matter and meninges through a defect in the cranium or skull base.  Temporal lobe encephalocele protruding through a middle fossa defect is even more rare with few distinctive features, so is difficult to identify if not investigated thoroughly. 
Kutz et al. in his study of 19 cases underwent operations for repair of spontaneous CSF otorrhea or rhinorrhea. The mean age was 61 years and the male to female ratio was 5:12. All female patients had a body mass index (BMI) greater than 30 mg/kg.  The most common presenting symptom was otorrhea after a myringotomy or placement of a tympanostomy tube. 
In some rare presentation of spontaneous CSF otorrhoea, it can be presented as chronic otitis external with middle ear encephalocele. 
Review of literatures in the last 5 years with focus on different presentation and modalities of treatment.
| Discussion|| |
The most common site of CSF leakage is through the floor of the anterior fossa, which communicates with the ethmoid or frontal sinuses, or with the nasal fossa.  The sphenoid sinus is rarely implicated as a source of spontaneous CSF fistula.  Communication with the middle cranial fossa and CSF fistula is more likely if the sphenoid sinus is laterally pneumatized. 
The majority of patients presented with symptoms of aural fullness, middle ear effusion, hearing loss and for certain extent with headache and meningitis.  Some patients had multiple defects in the tegmen and dura, and some can have meningoencephaloceles, can be seen behind the tympanic membrane.
CSF pressures and the hydrostatic pulsative forces may lead to the development of pit holes on the middle fossa at the sites of arachnoid villi with herniation of dura/ arachnoid or brain tissue.  If such defects are located over the underlying well pneumatized middle ear, encephalocele can develop and lead to CSF leakage into the affected site. Therefore, erosion of bone by arachnoid granulations is only significant when it affects a pneumatized part of the skull  [Figure 1] and [Figure 2].
|Figure 1: Arachnoid granulation tissue can erode and cause CSF otorrhea if temporal bone is well pneumatized (axial CT scan of one of king saud medical city patient with arachnoid pit or granulation tissue)|
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|Figure 2: MRI for the same patient with arachnoid granulation tissue (white arrows)|
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Laboratory testing of nasal or aural fluid drainage or aspirate for the presence of the protein Beta (beta)-2 transferring plays a key role in establishing the presence of a CSF leak. Such assays are not always available, making imaging pivotal in the diagnosis of this entity. The development of minimally invasive endoscopic repair, further underscores the importance of imaging as precise anatomic localization is important for repair. 
Both, computer tomography (CT scan) and magnetic resonance image (MRI) play an important role for the diagnosis. The high resolution CT scan with fine cuts (1 mm or less) can shows the direct anatomy and the relationship in different parts of temporal bone and can give indirect evidence of the CSF fistulae by revealing bony defects and opacification of middle ear or mastoid air cells. As a role, if there is a bony defect at base of skull with fluid in the dependent area or sinus then CSF leakage should be rolled out. Magnetic resonance cisternography in particular can non-invasively reveal CSF leakage in multiple planes without the disadvantage of ionizing radiation. On the T2-weighted spin-echo image sequence, is a superior to other modalities, the bright signal from the CSF column is well visualized against the middle ear cleft or sinus background of air.  MRI is essential to confirm the nature and extension of the lesion and any associated abnormalities. MRI is able to provide multiplanner images of the encephalocele that is useful for both diagnosis and surgical planning.
There are two well known surgical approaches, the middle fossa craniotomy and the transmastoid approach.  The management require adherence to the principles of establishing a safe ear as a primary goal, then with hearing restoration as a secondary goal.  As it is difficult in its presentation, the planning to repair it is also difficult with the middle fossa craniotomy provides the best exposure for defects involving the middle fossa floor, but not all neuro-otologist or the neurosurgeons are familiar with it. Wootten et al. showed that, both alloplastic and autologous materials are highly successful in repairing the defect(s) responsible for CSF otorrhea. No infections of the alloplastic bone cement occurred in their 17 cases series.
Surgical repair is mandatory to seal these non-traumatic CSF leaks. A multi-layered closure technique in which autologous and artificial materials are combined is considered to result in the highest rate of success.
A middle fossa craniotomy was used in 17 approaches. The most common defect sites were located over the tegmen mastoideum and tegmen tympani. Multiple materials were used in most repairs including allogenic bone cement and autologous materials. One patient had persistent otorrhea after a transmastoid approach and required a middle fossa craniotomy to repair a tegmen mastoideum defect. 
Transmastoid approach in the other hand with fascia, bone paté and fibrin glue is more popular to otologist and it is an easy to visualized the defect from the roof of middle ear.
Kari et al. demonstrated in his large group study in management of 56 consecutive patients with mastoid encephaloceles and CSF leaks. The majority were managed through the transmastoid route with temporalis fascia and calvarial bone graft. Among the patients operated on at least 18 months before analysis, there was a median follow-up of 54 months with no intracranial complications.  The average BMI of patients with spontaneous CSF leaks was found to be statistically significantly greater than that of patients with CSF leaks with traumatic, infectious or iatrogenic etiologies. ,
In this interesting study of these 56 cases, 12 had CSF otorrhea alone, 19 had encephaloceles, and 23 had both. Two patients presented with spontaneous pneumocephalus without CSF leak. Thirty-three patients had spontaneous onset of their lesion without any history of temporal bone surgery or trauma. This difference was found to be statistically significant. Six of these had either preceding or subsequent contralateral mastoid or anterior fossa (sphenoid, ethmoid) CSF leak. Intracranial pressure was evaluated in these cases and most underwent ventricular-peritoneal shunting to reduce their intracranial pressure. Sixteen cases followed previous mastoid surgery or chronic ear disease and 7 were attributed to previous temporal bone trauma. Lesions were repaired with the transtemporal approach (42), middle fossa (4), and combined (5) approaches. Five cases required petrosectomy and fat obliteration. There was one recurrent CSF leak 4 years after initial repair; the leak was anterior to the previously placed bone graft.
In other study of 28 cases with CSF leaks, operated by Stenzel et al. from 1983 to 2002. After a mean follow-up of 8 years, patients were interviewed concerning otorrhea or rhinorrhea and meningitis.  In this context, the CSF leak had arisen spontaneously (n = 3), traumatically (n = 6) or postoperatively (n = 19). The surgical CSF leak repairs were performed via a transmastoid (n = 13), a middle fossa (n = 11) or a combined (n = 4) approach. Surgical outcome was independent on the used graft. CSF leak could be sealed in 25 of 28 cases. Only 3 patients suffered from recurrences but meningitis or other complications did not occur. 
Comparing different techniques and grafts, there were no differences in the surgical outcome.
Brain herniation and/or CSF leak appear to be rare complications of surgery for revision chronic ear diseases.
CSF leak occurs when there is an osseous and dural defect at the skull base,  with direct communication of the subarachnoid space to the extracranial space, usually a paranasal sinus. Recognition of this leak site and source and appropriate treatment are necessary to avoid rhinorrhea or otorrhea, low-pressure headaches, and meningitis, known complications of CSF leak. The imaging evaluation has evolved over the past several decades. Description of current techniques available to direct treatment options, including multi-detector thin section computed tomography, and imaging recommendations are presented. Five patients underwent combined middle cranial fossa/transmastoid repair. Materials used in repair included temporalis fascia, free muscle graft, oxycel cotton, calvarial bone, pericranium, bone wax, and fibrin glue.
Because surgical repair by way of a mastoid approach alone can be inadequate if there are multiple tegmen defects, a middle fossa approach alone, or in combination with a transmastoid approach, should be considered in such cases. 
| Conclusion|| |
Recognition of CSF otorrhea can be difficult and challenging as the complication can be devastating and serious. Repairing the defect is mandatory to prevent these complications.
Different technique and approaches had been attempted with varieties of materials with almost similar result and success.
For otologist, transmastoid approach can be considered a good approach for repairing, except if there are multiple defects where middle cranial fossa approach will be adequate.
| Acknowledgements|| |
Otolarynglolgy and Radiology Departments in King Saud Medical City, Riyadh.
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[Figure 1], [Figure 2]