In our practice, epidural blood patching or conservative treatment consisting of bed rest, oral hydration. A csf leak stems from a tear or abnormality in the dura, the. Diagnosis of a csf leak generally begins with a physical exam and history. Spontaneous intracranial hypotension the spine hospital at. Mri demonstrated diffuse leptomeningeal and pachymeningeal enhancement figure, a, suggesting meningitis and intracranial hypotension due to. Spontaneous intracranial hypotension is believed to result from development of a spontaneous csf leak after minor trauma. According to the international classification of headache disorders, second edition, spontaneous intracranial hypotension is diagnosed primarily on the basis of the presence of positional headache, resolution of headache after blood patching, low csf pressure at lumbar puncture, the presence of csf leak at myelography, or a combination of these. In general, the brain is a sterile compartment and is separated from the sinuses.
A csf leak can be difficult to detect, causing misdiagnosis or delayed diagnosis. There are no concrete guidelines available for management. Spontaneous intracranial hypotension duke radiology. A csf leak located in the spine is today thought to be the prevailing etiology. There is virtually no awareness of spontaneous cerebral spinal fluid leaks in the medical establishment.
The most common cause of spontaneous intracranial hypotension is spontaneous spinal fluid leak. The objectives of the current study were to assess outcomes regarding spontaneous. Not all cases show a low csf pressure on lumbar puncture, and not all of the csf leaks are spontaneous as trauma may play a role in some cases. Traumatic cerebrospinal fluid leaks johns hopkins medicine.
Iatrogenic causes make up 16% of csf leaks, with the last 4% of leaks due to varied etiologies, including spontaneous leak and congenital. Criterion b, cranial mr imaging changes of intracranial hypotension ie, presence of subdural fluid collections, enhancement of the. Cerebrospinal fluid leak statpearls ncbi bookshelf. Cisternography rarely, however, identifies the site of leak.
December 11, 2009 doug reh, left, hopes that by studying csf leaks and working with neurologists and neurosurgeons, physicians can better treat the condition. What is the pathophysiology of cerebrospinal fluid csf. The operative management of cerebrospinal fluid leak is advised in the following circumstances. These diagnostic criteria were applied to a group of consecutive patients evaluated by us between january 1, 2001, and december 31, 2006, for spontaneous spinal csf leaks and intracranial hypotension. Nov 20, 2015 at the clivus spontaneous csf leak can be secondary to ecchordosis physaliphora, which should be taken into consideration in differentiation. It is an important cause of new headaches in young and middleaged individuals, but initial misdiagnosis is common. A ventriculoperitoneal shunt remains the standard method of bih treatment in patients with spontaneous nasal csf leak. Csf is a clear fluid that surrounds and protects the brain and the spinal cord, and cushions it from injury or damage. At the clivus spontaneous csf leak can be secondary to ecchordosis physaliphora, which should be taken into consideration in differentiation. Successful treatment of spontaneous cerebrospinal fluid leak. When the connective tissue known as dura that holds csf in around the spinal cord has a hole or tear, the result is a loss of csf volume, known as intracranial hypotension. Dec 11, 2009 a multidisciplinary approach to treating spontaneous csf leaks date. The pathophysiology of spontaneous csf leak is not completely understood, although the presence of meningeal diverticulum have been postulated and seems to be a prerequisite. Based on the results of this study, further research is warranted to establish the role of elevated csf pressure in spontaneous csf otorrhea.
Lumbar puncture showed low pressure 50 mm h 2 o, raised levels of protein 4. Connective tissue spectrum abnormalities associated with. Spontaneous intracranial hypotension nord national. Do spontaneous csf leaks represent a distinct clinical entity and a variant of idiopathic intracranial hypertension. The relief of unilateral painful thoracic radiculopathy. Her prolactin level improved, however within 4 weeks of initiation of therapy 15 months after surgery she was confirmed to have a csf leak. This supports a role of iih in the pathogenesis of spontaneous csf otorrhea. Other, extremely rare, causes of spontaneous intracranial hypotension include diabetic coma or a leakage of csf through the ears or nose. None of these terms are completely satisfactory, however. Spontaneous spinal csf leaks cause spontaneous intracranial hypotension but no systematic study of the different types of these csf leaks has been reported. Spinal csf leak may occur as a result of medical procedures or trauma or may occur spontaneously. Csf leaks focusing on premorbid factors, surgical technique, and. Low cerebrospinal fluid csf pressure headache is frequently encountered in pain clinics following dural puncture. Information on this site is provided in good faith and is intended to inform discussion and promote research.
After intrathecal fluorescein is administered, an exposed frontal recess. Csf leaks, such as trauma and postsurgical leaks, and to discuss the various imaging modalities used in their detection, to assist the reader in the selection and interpretation of imaging in this condition. Spontaneous csf leak is considered a disorder with a variety of clinical manifestations and imaging. A spinal cerebrospinal fluid csf leak is an underdiagnosed cause of a debilitating headache that is treatable. Spontaneous spinal cerebrospinal fluid leaks schievink. Management protocol according to clinical presentation.
The underlying cause is a spontaneous spinal cerebrospinal fluid csf leak. A 35yearold man presented with fever, headache, and vomiting. Diagnostic criteria for spontaneous spinal csf leaks and. Based on our experience with spontaneous intracranial hypotension, we propose a classification system of spontaneous spinal csf leaks. A classification system of spontaneous spinal csf leaks. Women are affected more commonly than men and most are in the fifth or sixth decade of life. Spinal cerebrospinal fluid csf leak brain foundation. Spontaneous intracranial hypotension is secondary to a cerebrospinal fluid csf leak at the level of the spine and the resulting loss of csf volume to support the brain and spinal cord.
Successful treatment of spontaneous intracranial hypotension. In seven patients, spontaneous csf leak was the first noted manifestation of the genetic disorder. Intracranial hypotension an overview sciencedirect topics. Dec 12, 2019 radionuclide cisternography may be helpful in the situation where spontaneous csf leak is suspected, but mri has no suspicious findings for leak as a way to confirm the presence of csf leak. Spontaneous csf leaks may be associated with elevated spinal fluid pressure resulting in bone thinning in key areas of the sinuses. Recurrent spontaneous spinal cerebrospinal fluid leaks and. If you have symptoms of cerebral spinal fluid leaks, or love someone who does, please check out the spinal csf leak foundations web site at. Based on the successful treatment of this patients spontaneous intracranial hypotension, we advocate that patients undergo epidural blood patches to target the site of any csf leak identified by imaging studies to improve the efficacy of this intervention. Mri demonstrated diffuse leptomeningeal and pachymeningeal enhancement figure, a, suggesting meningitis and intracranial hypotension due to spontaneous skull. Sc046319 promoting awareness of cerebrospinal fluid csf leaks, with a particular focus on spinal csf leaks. Information on this site is provided in good faith and is intended to. This series introduces a correlation between radiographic empty sella, increased bmi, and spontaneous csf otorhinorrhea.
The causes of spontaneous spinal fluid leak are not completely understood. The most common sites were cribriform plate 51%, sphenoid lateral pterygoid recess 31%, and ethmoid roof 8%. Spontaneous intracranial hypotension the spine hospital. Using a whitacre or sprotte atraumatic needle for lumbar punctures also may decrease the risk of csf leak compared with standard needles. Primary spontaneous transclival cerebrospinal fluid leak. A spinal fluid leak from the intracranial space to the nasal res. Cerebrospinal fluid fistula, commonly known as csf leak, occurs when there is an abnormal communication between the csf containing space around the brain subarachnoid space and either the sinuses or the ear. An axial ct of a patient with a spontaneous csf leak reveals a defect in the posterior table of the left frontal sinus. A spontaneous cerebrospinal fluid leak scsfl is a medical condition in which the cerebrospinal fluid csf surrounding the human brain and spinal cord leaks out of the surrounding protective dural sac for no apparent reason. Spontaneous spinal cerebrospinal fluid csf leaks from the intradural to the epidural space without history of dural puncture or surgery are rare. For example, loss of csf volume also occurs with spontaneous csf rhinorrhea or otorrhea, but the typical imaging and clinical features of spontaneous intracranial hypotension rarely, if ever, occur under those circumstances.
When this fluid volume is reduced, there is less fluid available to support the normally floating brain inside the skull. Csf rhinorrhea may present spontaneously or following trauma and if untreated. About csf leaksspontaneous intracranial hypotension. Spontaneous intracranial hypotension sih is a debilitating and often invisible medical condition resulting from a spontaneous spinal cerebrospinal fluid csf leak. Contemporary approach to the diagnosis and management of. Spontaneous csf leak headache, however, is a rare entity, and its diagnosis and management can pose serious clinical challenges. These confront neurologists and create many challenges. Spinal imaging findings in spontaneous intracranial hypotension. Aug 29, 2012 in seven patients, spontaneous csf leak was the first noted manifestation of the genetic disorder. Cerebrospinal fluid csf leak occurs when csf escapes through a small tear or hole in the tissue that surrounds the brain and spinal cord and holds in the csf. We conclude that spontaneous csf leaks are associated with a spectrum of connective tissue abnormalities and may be the first noted clinical presentation of the genetic disorder. Recurrent spontaneous spinal csf leaks are not rare, and the recent successful repair of such a leak at another site may be an important risk factor. Pdf traumatic, iatrogenic, and spontaneous cerebrospinal fluid. That aspect might represent a weakness at the spinal meninges, creating a favorable condition to the csf leaks and, as consequence the origin of intracranial hypotension 1.
The csf leak association is a scottish charitable incorporated organisation charity no. A spinal csf leak occurs when a tear or hole occurs in the spinal dura and the csf leaks out. Spontaneous spinal cerebrospinal fluid leaks and intracranial. Learn more from cleveland clinic about causes, symptoms, treatment, recovery and more. What is spontaneous cerebrospinal fluid rhinorrhea. Although upper arm pain or paresthesia is reportedly associated with sih from spontaneous spinal csf leak in the presence of orthostatic headache, low thoracic radicular pain due to spontaneous spinal csf leak unassociated with postural headache is extremely. Experts such as those in the cedarssinai csf leak program can be pivotal in providing relief. Myelographic techniques for the detection of spinal csf leaks. Causes of spontaneous csf leak leakage of csf into the epidural space through a defect in the thecal sac has been. May 22, 2012 a 35yearold man presented with fever, headache, and vomiting. Review article spontaneous intracranial hypotension. To the best of our knowledge, there has only been 1 article with a large series of patients that looked into medically induced, spontaneous csf leaks complicating da therapy in prolactinomas. We report our experience with two cases of primary spontaneous csf leaks through the clivus, managed via an endoscopic transsphenoidal approach.
Spontaneous intracranial hypotension sih caused by spontaneous spinal cerebrospinal fluid csf leaks produces orthostatic headaches. Spontaneous csf leaks often result from skull base dehiscence and bony. Most neurologists will go their whole career without seeing a patient like me. Fast effective treatment of spontaenous csf leak causing. Either through rupture of the arachnoid membrane or tarlov cysts opening the dura or spontaneous development of a venous fistula. Postural headache is the distinctive symptom of lowpressure headaches, but other headache patterns nonpositional, exertional occur as well. Spontaneous intracranial hypotension is an uncommon but not rare cause of new onset daily persistent headaches.
Spontaneous intracranial hypotension is an increasingly recognized cause of new daily persistent headaches, particularly among young and middleaged people, but an initial misdiagnosis remains common. Context spontaneous intracranial hypotension is caused by spontaneous spinal cerebrospinal fluid csf leaks and is known for causing orthostatic headaches. Men and women of all ages are affected but the diagnosis is more common in women. The recurrent leak occurred between 10 and 77 months after the initial csf leak, but within 2 or 3 months of successful surgical repair of the leak in all patients. The clinical spectrum of spontaneous intracranial hypotension is expanding. The risk of iatrogenic csf leak after lumbar puncture may be decreased with the use of a 29 gauge needle instead of 26 gauge needle, along with orienting the bevel parallel to the axis of the spine.
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