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Spontaneous intracranial hypotension. (A) Pre-operative T1-weighted MRI demonstrates dural thickening (thick arrow), sagging of the pons (diamond head) and draping of the optic chiasm (thin arrow). (B) Pre-operative T2-weighted axial. (C) Pre-operative T1-weighted gadolinium enhanced coronal MRI.
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Introduction:
Spontaneous intracranial hypotension (SIH) is an uncommon syndrome widely attributed to CSF hypovolemia, typically secondary to spontaneous CSF leak. Although commonly associated with postural headache and variable neurological symptoms, one of the most severe consequences of SIH is bilateral subdural hematomas with resultant neurolo...
Contexts in source publication
Context 1
... left-handed man who initially pre- sented with severe positional headache, blurry vision, and tinnitus. He was discharged with a diagnosis of benign positional vertigo but subsequently presented to the emergency room with wors- ening symptoms. On MRI a non-enhancing, crescentic, extraaxial mass was visualized in the anterior right temporal lobe (Fig. 1A). A lumbar puncture was unsuccessful under fluoroscopy. The patient improved on bed rest and hydration and was discharged with a diagnosis of ...
Context 2
... of SIH is made on the basis of symptoms, history, and MR findings. Although lumbar puncture is generally unnec- essary, a CSF opening pressure of less than 60 cm of water is a classic finding. MR findings of pachymeningeal enhancement, venous engorgement, pseudo-subarachnoid hemorrhage, pituitary hyperemia, and/or cerebellar tonsil "sagging" (Fig. 1A) are help- ful diagnostic features. Spinal imaging is generally reserved for patients with SIH who do not respond to non-directed EBP. Precise localization of the leak is best accomplished with CT myelography, though there is increasing interest in gadolinium myelography. In our case, high resolution CT myelography not only confirmed ...
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Background
Spontaneous intracranial hypotension (SIH) is a rare pathology caused by a cerebrospinal fluid (CSF) leak. If intractable by conventional methods (i.e. bedrest, analgesics, or epidural blood patching) it may lead to the inability of the patient to cope with daily life and eventually to life-threatening complications. Recently, calcified...
Citations
... There is another one case report reported where there is a giant growth of osteophytes in anterior surface of thoracic spine that compress the greater splanchnic nerve and need surgical decompression with improves of symptoms [14]. There are also cases in the literature of Spontaneous Intracranial Hypotension (SIH) due to proliferation of osteophytes in the anterior thoracic site with development of important neurological symptoms such as: postural headache subdural hematoma and neurological cognitive impairment [15]. In the cases reported in the literature, the treatment of hypotension was in most cases conservative with a partial and progressive clinical remission after at least 6 weeks of drug therapy and epidural patches [16]. ...
The main purpose of this article is to provide an extensive review of the literature about cervico-thoracic marginal osteophytosis with pathophysiological, histological
evidence and complications emerging from the reported literature (esophageal perforation, Spontaneous Intracranial Hypotension (SIH), dysphagia, dyspnea etc.); and to correlate the alterations present in 5 cases reported by our centre with reported images and prevalence of radiological patterns found. The iconographic description is taken from the images that have been archived respecting the rules on privacy and before publishing them the informed consent of the patient was obtained and signed.
... A thorough review of the existing English literature between 1970 and 2020 revealed only 24 reported cases (Table 1). [10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28] Osteophyte involvement was seen in 13 of the 24 cases. The diagnosis was more common in women (ratio 1.67:1), and the mean age at diagnosis was 41.8 years (range 25 to 57); 79.2% patients (n = 19) had pathology in the thoracic spine versus 20.8% patients (n = 5) with cervical spine pathology. ...
BACKGROUND
Intracranial hypotension (IH) manifests with orthostatic headaches secondary to cerebrospinal fluid (CSF) hypovolemia. Common iatrogenic etiologies include lumbar punctures and spinal surgery. Although much rarer, structural defects such as osteophytes and herniated calcified discs can violate dural integrity, resulting in CSF leak.
OBSERVATIONS
The authors reported the case of a 32-year-old woman who presented with progressively worsening orthostatic headaches. During an extensive examination, magnetic resonance imaging of her thoracic spine revealed a cervicothoracic ventral epidural collection of CSF, prompting a dynamic computed tomography myelogram, which not only helped to confirm severe cerebral hypotension but also suggested underlying pathology of a dorsally projecting disc osteophyte complex at T2–3. Conservative and medical management failed to alleviate symptoms, and a permanent surgical cure was eventually sought. The patient underwent a transdural thoracic discectomy with dural repair, which resulted in resolution of her symptoms.
LESSONS
Clear guidelines regarding the management strategy of IH secondary to disc osteophyte complexes are yet to be established. A thorough literature review noted only 24 reported cases between 1998 and 2019, in which 13 patients received surgery. There is a 46% symptom resolution rate with conservative management, lower than that for iatrogenic etiologies. For patients in whom conservative management failed, surgical intervention proved effective in resolving symptoms, with a success rate of 92.3%.
... As shown in the chart, conservative treatment always fails to yield persistent improvement in such patients, and currently, whether to perform surgical SDH evacuation or EBP therapy as the initial procedure is still controversial [12,13]. Through the review of those reports above and other published reports, we believe that IH patients with both SDH and cranial nerve injury require emergency surgery under the following conditions: Moderate to large hematoma causing brain August 6, 2021 Volume 9 Issue 22 hernia and neurological deterioration [14], failure to improve after applying the Trendelenburg position in comatose SDH patients with a mass effect and pupil dilation [6], and progressive SDH or rapid cognitive deterioration after EBP therapy [15,16]. Furthermore, surgical repair is indispensable in patients with evidence of cranial August 6, 2021 Volume 9 Issue 22 or spinal anatomic abnormalities (i.e. ...
... Thus, EBP therapy may be a priority since it is much safer, with less adverse reactions. Last, compared with injecting blood into multiple targeted sites of CSF leakage at one time, putting the patient at a risk of deterioration after EBP therapy due to ICP elevation [16], it is safer to perform repeated targeted EBP procedures after some interval of time with careful follow-up. ...
Background:
Intracranial hypotension (IH) is a disorder involving cerebrospinal fluid (CSF) hypovolemia due to spontaneous or traumatic spinal CSF leakage and is easily being misdiagnosed or missed, especially in these patients without the prototypical manifestation of an orthostatic headache. At present, the management of IH with both cranial nerve VI palsy and bilateral subdural hematomas (SDHs) is still unclear.
Case summary:
A 67-year-old male Chinese patient complained of diplopia on the left side for one and a half mo. Computed tomography revealed bilateral SDHs and a midline shift. However, neurotrophic drugs were not effective, and 3 d after admission, he developed a non-orthostatic headache and neck stiffness. Enhanced magnetic resonance imaging revealed dural enhancement as an additional feature, and IH was suspected. Magnetic resonance myelography was then adopted and showed CSF leakage at multiple sites in the spine, confirming the diagnosis of having IH. The patient fully recovered following multiple targeted epidural blood patch (EBP) procedures.
Conclusion:
IH is a rare disease, and to the best of our knowledge, IH with diplopia as its initial and primary symptom has never been reported. In this study, we also elucidated that it could be safe and effective to treat IH patients with associated cranial nerve VI palsy and bilateral SDHs using repeated EBP therapy.
... Connective tissue abnormality such as Marfan's syndrome has been shown to be associated with IIH [7,8]. A few cases of spinal osteophytes causing spontaneous dura puncture and intracranial hypotension have also been reported [9][10][11][12][13][14][15]. MRI and CT myelogram are the two most commonly employed imaging tests to identify site of CSF leakages [16]. ...
... However, this may not be the case for SIH that is caused by spinal osteophytes. A review of all reported cases of SIH secondary to spinal osteophytes [9][10][11][12][13][14][15] showed that all but one [10] patient had inadequate responses to EBP procedures and required surgical removal of osteophytes and dural repair. Outcome after surgical intervention was favorable in all cases [9,[11][12][13][14][15]. ...
... A review of all reported cases of SIH secondary to spinal osteophytes [9][10][11][12][13][14][15] showed that all but one [10] patient had inadequate responses to EBP procedures and required surgical removal of osteophytes and dural repair. Outcome after surgical intervention was favorable in all cases [9,[11][12][13][14][15]. ...
... Mokri et al. [8] reported one such case of tonsillar herniation down to C2 level in a 9 year chronic case of spinal CSF leak. Veeravagu et al. [9] suggest that, of all the effects of SIH the most severe are due to subdural hematoma, and they almost always require neurosurgical intervention. Also, diplopia has been demonstrated to be the strongest positive predictor of CSF leaks if an SIH is suspected [9]. ...
... Veeravagu et al. [9] suggest that, of all the effects of SIH the most severe are due to subdural hematoma, and they almost always require neurosurgical intervention. Also, diplopia has been demonstrated to be the strongest positive predictor of CSF leaks if an SIH is suspected [9]. In this article we propose the institution of a protocol which we practice to diagnose and treat SIH patients in our facility. ...
... Degenerative lesions in the spine are a rare cause of secondary IH [12]; a review of literature shows less than ten reported cases of intracranial hypotension secondary to such lesions (Table 1), and SIH due to a thoracic column pathology has been only rarely reported in the literature. One case described a calcified T7-8 disc indenting the spinal cord, with an extradural collection of CSF at the same level [13]; one case involved a thoracic osteophyte associated with a CSF fistula [7], two other cases described single dorsal osteophytic spurs [9,14], and one series illustrated two cases of intra-dural thoracic osteophyte with superimposed disc herniation [15]. Mechanical stressors are important for the pathogenesis of this particular secondary SIH. ...
PurposeIn this article, we aim to describe the presentation and management of a case of spontaneous intracranial hypotension caused by a dural tear from a ventral thoracic osteophyte at the T12 level that was refractory to non-surgical treatment modalities. A review of the literature has been performed. Also a proposal of diagnostic and treatment algorithm is presented. Intracranial hypotension and CSF leak as a result of dural tear is a common phenomenon. However, the detection of the source of CSF leak from a thoracic spinal osteophyte has rarely been reported. Methods
Diagnostic workup including MRI and CT Myelogram as well as application of epidural blood patches and surgical technique of hemilaminectomy and osteophytectomy by transpedicular approach have been described. Literature review was conducted using relevant search terms in PubMed. ResultsThe patient’s spontaneous intracranial hypotension symptoms resolved and this persisted on follow up visits. Review our experience as well as similar cases in the literature pointed us towards a diagnostic and treatment algorithm. Conclusions
Spontaneous resolution is the norm for intracranial hypotension of most etiologies and management of all such cases begins with fluid resuscitation coupled with bed rest. On failure of conservative therapy, autologous epidural blood patches into the spinal epidural space should be tried, which often produce an immediate relief of symptoms. Osteophyte-induced dural tear and consequent intracranial hypotension may require surgical intervention if the symptoms are refractory to conservative treatment. Under all circumstances a careful step-wise approach for diagnosis and treatment of spontaneous intracranial hypotension needs to be followed, as we have proposed in our article.
Cerebrospinal fluid (CSF) leakage due to large ventral dural tears (VDT) often requires surgical intervention. Surgical closure of a high thoracic VDT is challenging and associated with high morbidity, especially if it is performed after multiple epidural blood patch (EBP)/fibrin glue injections. A 44-year-old woman was diagnosed with spontaneous intracranial hypotension due to VDT at T1-T2, causing CSF leakage. Multiple EBP and fibrin glue injections failed to treat her symptoms; hence, the patient underwent surgical closure using the posterior approach. The patient was anesthetized using standard anesthetic drugs and was maintained under total intravenous anesthesia to facilitate continuous motor-evoked potential (MEP) monitoring. The surgical course was complicated by bleeding, hypotension, and MEP loss. Continuous MEP monitoring, effective team communication, quick restoration of blood pressure(BP) and BP augmentation with fluid, blood, and vasopressor helped to restore the MEP back to baseline. Hence, the patient recovered without neurological morbidity. This case report highlights the importance of adequate vascular access, multimodal spinal cord monitoring, and BP augmentation during a high thoracic VDT repair.
Objective/background:
Spontaneous spinal cerebrospinal fluid (CSF) leaks are a rare entity that can lead to intracranial hypotension and associated headaches, meningismus, and patient debility. Surgical treatment may be necessary for patients that do not respond to conservative management. Surgical repair of CSF leaks located in the ventral thoracic spine traditionally requires an invasive, open approach.
Methods:
We describe the case of a patient with a ventral thoracic spontaneous spinal CSF leak associated with a ventral bony osteophyte successfully treated with spinal endoscopy. We also provide a systematic review of the literature to better understand outcomes of this approach.
Results:
A total of 55 patients were included in the systematic review. The study designs found in the literature review included case reports (66.7%), retrospective cohorts (22.2%) and prospective cohorts (11.1%). Of the studies reporting data, 50% of studies stated they used an open posterior approach to the dural defect, while 37.5% reported using an open anterior approach to the pathology. Only 1 (12.5%) study reported using an endoscope. Most studies (62.5%) utilized primary closure of the dura in their technique, while 37.5% reported using a local tissue graft (fat or muscle) or a dural sealant for their closure technique, and 25% of studies reported using a dural substitute for their closure technique. Overall mean clinical follow-up with 19.8 months.
Conclusion:
The endoscopic approach described here for treatment of this rare entity allows for removal of bony spicules/osteophytes and dural repair without the morbidity associated with traditional open dorsolateral or ventrolateral approaches.
Intracranial hypotension is a rare cause of chronic subdural hematomas (CSDH). These patients often present with headaches, but unlike other etiologies of CSDH, surgical evacuation can often result in worsened neurologic symptoms. Careful clinical and radiographic evaluation is needed in order to efficiently and accurately diagnose and treat intracranial hypotension. Diagnosis often requires multimodal imaging in order to identify a source of a spinal fluid leak. Treatment options include epidural blood patches, fibrin glue patches, and open surgical repair. Subdural evacuation should only be considered once the source of the spinal fluid leak has been identified and repaired or in patients presenting in extremis.
Background:
Intracranial hypotension secondary to spontaneous spinal cerebrospinal fluid (CSF) fistula is a rare condition which can have serious sequelae. Early diagnosis and treatment can be challenging.
Case description:
We present a case of a 17-year-old man who presented with a history of sudden onset, postural headaches, associated with upper thoracic back pain. Magnetic Resonance Imaging (MRI) demonstrated a thoracic extradural fluid collection and slumping of the brain within the posterior fossa. The patient was initially managed with a period of bed rest, followed by a thoracic epidural blood patch. Symptoms recurred and subsequent operative exploration found a large arachnoid cyst with CSF egress through a linear split in the axilla of the right T7 nerve root. The arachnoid cyst was resected and the defect was closed primarily. All symptoms completely resolved. MRI at three months post-operatively demonstrated normal spinal configuration and resolution of brain sagging.
Conclusions:
Spontaneous CSF leaks are a rare cause of postural headache. Although epidural blood patching is an easy and safe intervention, early serial imaging to ascertain the evolution of the pathology may identify cases that are amenable to early surgical management.
