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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.70 no.7 São Paulo July 2012 



Nontraumatic clival cerebrospinal fluid rhinorrhea


Rinoliquorreia espontânea secundária à fístula de clivus



Luana Antunes Maranha; Renata de Angeli Corredato; João Cândido Araújo

Serviço de Neurocirurgia do Hospital de Clínicas – Universidade Federal do Paraná, Curitiba PR, Brazil




Cerebrospinal fluid (CSF) fistula is characterized by a leak through a communication between the subarachnoid space (SAS) and the external environment. Three main factors are necessary to be present for the production of a fistula: bone defect, meningeal tear, and pressure gradient.

Spontaneous fistulae, as opposite to the posttraumatic variant, are less common. The ones submitted to a high pressure gradient are usually secondary to tumors or hydrocephalus, whereas the low-pressure gradient type may be related to congenital defects, bone erosion, excessive aeration, or encephaloceles1.

The characteristics of unilateral leak of colorless fluid through the nostril, varying with postural changes and followed by headache, must not be overlooked. While posttraumatic leaks often heal spontaneously, around 70% of the nontraumatic variant persist, with recurrences after surgical repair ranging from 25 to 87%2.

A patient with an upper clivus spontaneous CSF fistula is presented, among only other four previously reported cases.



A 49 year-old woman with a history of CSF rhinorrhea since 2002, had been submitted to three different attempts to surgical correction up to 2007, always through bifrontal craniotomies, with episodes of recurrent meningitis. On admission, she had no focal neurological signs except for olfactory loss. Her high blood pressure had always been poorly controlled. Magnetic resonance imaging (MRI) revealed fluid level within the sphenoidal sinus, connected to the pre-pontine cistern, through an upper clival defect (Figure).



She was then submitted to a transnasal endoscopic approach. A bone defect was identified at the upper clivus, which was repaired through the use of nasal septal cartilage secured with a mucosal flap rotation and fibrin glue. After five days, her lumbar drain was removed. She has been followed for the last four years without recurrence.



Idiopathic spontaneous CSF rhinorrhea would represent from 14 to 46% of all cases3. Skull base malformation, excessive sinus aeration, primary or secondary empty sella syndrome, and obesity would be risk factors. The association between increased weight and benign intracranial hypertension applied to a thinned skull floor has been speculated as a possible cause3. The origin of spontaneous clival fistulae remains to be elucidated.

Coitero et al.4 described the first two cases of CSF clival fistulae, mentioning the diagnostic challenges.

Aside from the primitive synchondrosis, the process of endochondral ossification for clival formation is continuous and does not allow weakened fusion points, which could eventually lead to bone defects5.

Coitero et al.4 suggested that, in one of their cases, repeated basilar artery pulsations over a thinned bone structure could have produced the defect. Similar explanation could eventually be suggested for this particular case, considering in addition the associated condition of poorly controlled arterial blood pressure.

Advances in technological imaging tools have provided better facilities for the identification of CSF leaking sites, whereas improvements in surgical techniques, including the introduction of skull base endoscopy, have led to better surgical results3. However, attempts to explain the pathophysiology of CSF clival fistulae remain speculative.



1. Ommaya AK. Cerebrospinal fluid rhinorrhea. Neurology 1964;14:106-113.         [ Links ]

2. Telera S, Conte A, Cristalli G, Occhipinti E, Pompili A. Spontaneous cerebrospinal fluid rhinorrhea as the presenting symptom of sellar pathologies: three demonstrative cases. Neurosurg Rev 2007;30:78-82.         [ Links ]

3. Elrahman HA, Malinvaud D, Bonfils NA, Daoud R, Mimoun M, Bonfils P. Endoscopic management of idiopathic spontaneous skull base fistula through the clivus. Arch Otolaryngol Head Neck Surg 2009;135:311-315.         [ Links ]

4. Coiteiro D, Tavora L, Antunes JL. Spontaneous cerebrospinal fluid fistula through the clivus: report of two cases. Neurosurgery 1995;37:826-828.         [ Links ]

5. Ommaya AK, DiChiro G, Baldwin M, Pennybacker JB. Nontraumatic cerebrospinal fluid rhinorrhea. J Neurol Neurosurg Psychiatry 1968;31:214-225.         [ Links ]



Luana Antunes Maranha
Rua Desembargador Motta 2.198 / apto. 130
80420-190 Curitiba PR - Brasil

Received 08 December 2011
Received in final form 30 December 2012
Accepted 06 January 2012



Conflict of interest: There is no conflict of interest to declare.

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