Spontaneous intracranial hypotension: updates from diagnosis to treatment.

Spontaneous intracranial hypotension (SIH) is caused by spinal cerebrospinal fluid (CSF) leaks, which result in continued loss of CSF volume and multiple debilitating clinical manifestations. The estimated annual incidence of SIH is 5/100,000. Diagnostic methods have evolved in recent years due to improved understanding of pathophysiology and implementation of advanced myelographic techniques. Here, we synthesize recent updates and contextualize them in an algorithm for diagnosis and treatment of SIH, highlighting basic principles and points of practice variability or continued debate. This discussion includes finer points of SIH diagnosis, CSF leak classification systems, less common types and variants of CSF leaks, brain MRI Bern scoring, potential SIH complications, key technical considerations, and positioning strategies for different types of dynamic myelography. The roles of conservative measures, non-targeted or targeted blood patches, surgery, and recently developed endovascular techniques are presented.

Osseous spicules of the posterior elements causing fast cerebrospinal fluid leaks.

PURPOSE: Dural tears are a common cause of spontaneous spinal CSF leaks. The majority of such leaks occur ventrally along the thecal sac, typically due to ventral osseous spicules that cause a rent in the dura. A minority of dural leaks are posterolateral in location. These leaks usually do not have an identifiable anatomic cause. We have anecdotally observed cases of posterolateral leaks caused by osseous spicules and sought to describe this phenomenon. METHODS: We retrospectively reviewed our imaging database, searching for cases of posterolateral CSF leaks caused by osseous spicules. We identified and included three such patients and reviewed imaging and clinical information from each patient. RESULTS: All three patients had been diagnosed using hyperdynamic CT myelography or conventional CT myelography. Their imaging showed dorsal epidural fluid collections that were related to posterolateral leaks adjacent to dorsal osseous spicules. CONCLUSION: Dorsal osseous spicules have the potential to cause posterolateral CSF leaks.

Lateral decubitus dynamic CT myelography for fast cerebrospinal fluid leak localization.

Dynamic CT myelography is used to precisely localize fast spinal CSF leaks. The procedure is most commonly performed in the prone position, which successfully localizes most fast ventral leaks. We have recently encountered a small subset of patients in whom prone dynamic CT myelography is unsuccessful in localizing leaks. We sought to determine the added value of lateral decubitus dynamic CT myelography, which is occasionally attempted in our practice, in localizing the leak after failed prone dynamic CT myelography. We retrospectively identified 6 patients who underwent lateral decubitus dynamic CT myelography, which was performed in each case because their prone dynamic CT myelogram was unrevealing. Two neuroradiologists independently reviewed preprocedural spine MRI and all dynamic CT myelograms for each patient. Lateral decubitus positioning allowed for precise leak localization in all 6 patients. Five of six patients were noted to have dorsal and/or lateral epidural fluid collections on spine MRI. One patient had a single prominent diverticulum on spine MRI (larger than 6 mm), whereas the others had no prominent diverticula. Our study suggests that institutions performing dynamic CT myelography to localize fast leaks should consider a lateral decubitus study if performing the study in the prone position is unrevealing. Furthermore, the presence of dorsal and/or lateral epidural fluid collections on spine MRI may suggest that a lateral decubitus study is of higher yield and could be considered initially.

Benefits of Photon-Counting CT Myelography for Localization of Dural Tears in Spontaneous Intracranial Hypotension.

Photon-counting CT is an increasingly used technology with numerous advantages over conventional energy-integrating detector CT. These include superior spatial resolution, high temporal resolution, and inherent spectral imaging capabilities. Recently, photon-counting CT myelography was described as an effective technique for the detection of CSF-venous fistulas, a common cause of spontaneous intracranial hypotension. It is likely that photon-counting CT myelography will also have advantages for the localization of dural tears, a separate type of spontaneous spinal CSF leak that requires different myelographic techniques for accurate localization. To our knowledge, prior studies on photon-counting CT myelography have been limited to techniques for detecting CSF-venous fistulas. In this technical report, we describe our technique and early experience with photon-counting CT myelography for the localization of dural tears.

Incremental diagnostic yield and clinical outcomes of lateral decubitus CT myelogram immediately following negative lateral decubitus digital subtraction myelogram.

INTRODUCTION: Spontaneous intracranial hypotension (SIH) caused by a spinal cerebrospinal fluid (CSF) leak classically presents with orthostatic headache. Digital subtraction myelography (DSM) has a well-established diagnostic yield in the absence of extradural spinal collection. At our institution, DSM is followed by lateral decubitus CT myelogram (LDCTM) in the same decubitus position to increase diagnostic yield of the combined study. We evaluated the incremental diagnostic yield of LDCTM following negative DSM and reviewed patient outcomes. METHODS: Retrospective review of consecutive DSMs with subsequent LDCTM from April 2019 to March 2021 was performed. Combined reports were reviewed, and studies with positive DSMs were excluded. Of the exams with negative DSM, only studies with LDCTM reports identifying potential leak site were included. Interventions and follow-up clinical notes were reviewed to assess symptoms improvement following treatment. RESULTS: Of the 83 patients with negative DSMs, 11 (13.2%) had positive leak findings on LDCTMs, and 21 (25.3%) were equivocal. Of 11 positive LDCTMs, 6 leaks were nerve sheath tears (NSTs) and 5 were CSF-venous fistulas (CVFs). 10/11 (90.9%) had intervention and follow-up, with 9/10 (90%) having positive clinical outcome. Of the 21 equivocal LDCTM patients (19 CVFs and 2 NSTs), 15 (71.4%) had interventions and follow-up, with 3/15 (20.0%) with positive clinical outcomes. CONCLUSION: LDCTM following negative DSM has an incremental diagnostic yield up to 38.6%, with up to 14.5% of positive patient outcomes following treatment. LDCTM should be considered after DSM to maximize diagnostic yield of the combined exam.

Diagnostic Yield of Intrathecal Gadolinium MR Myelography for CSF Leak Localization.

PURPOSE: Intrathecal gadolinium magnetic resonance (MR) myelography can be used to localize various types of spinal cerebrospinal fluid (CSF) leaks; however, its diagnostic yield is not well known. We sought to determine the diagnostic yield of MR myelography in patients with spontaneous intracranial hypotension. METHODS: A retrospective review was performed on all patients who had undergone intrathecal gadolinium MR myelography at our institution from 2002 to 2020 for suspected spinal CSF leak. The MR myelography images were reviewed for the presence or absence of a spinal CSF leak site. Images were also evaluated for the presence an extradural fluid collection. RESULTS: A total of 97 patients were included in the final cohort. The average age was 52.6 years; 67.0% were female, 4 patients underwent 2 examinations each, yielding a total of 101 MR myelograms. The source of a spinal CSF leak was localized in 14 patients. The diagnostic yield for CSF leak localization on intrathecal gadolinium MR myelography was 14/101 (13.9%) per GdM examination and 14/97 (14.4%) per patient. Among the subset of patients without extradural fluid collections, the yield was 15.7% per examination. All detected leaks were either CSF-venous fistulas or distal nerve root sleeve tears. CONCLUSION: Intrathecal gadolinium MR myelography is capable of localizing CSF-venous fistulas and distal nerve root sleeve tears; however, our data show that it has a limited diagnostic yield. We suggest that other modalities may be a better first step before attempting intrathecal gadolinium MR myelography.

Utility of CT venography in monitoring stent patency in idiopathic intracranial hypertension: retrospective single-center study.

BACKGROUND: Cerebral venous sinus stenting is an established treatment for patients with idiopathic intracranial hypertension (IIH), refractory to medical management and with stenotic venous sinus on conventional cerebral venography. Currently, there are no clear guidelines on optimal noninvasive imaging modality for routine post-stenting follow-up. We investigated diagnostic yield of CT venography (CTV) for evaluation of stent patency. METHODS: We reviewed our clinical database of patients with a diagnosis of IIH, who underwent stenting of stenotic transverse or transverse/sigmoid sinus junction. Patients who had follow-up CTVs after more than 30 days were included in the final study group. All CTVs were reviewed by two readers for in-stent thrombosis, extrinsic stent compression, juxta-stent stenosis, and new contralateral venous sinus stenosis. Inter-observer agreement and association of stent patency with clinical outcomes (headache and papilledema) were assessed. RESULTS: In all 36 included patients with 38 cerebral venograms for stenting, follow-up CTVs demonstrated adequate opacifications of the venous sinuses and stents for confident evaluation for in-stent thrombosis, extrinsic stent compression, juxta-stent stenosis, and new contralateral venous sinus stenosis, with inter-observer agreement coefficient of 0.7, 1.0, 0.8, and 1.0 respectively. Association between abnormal CTV and higher rates of persistent headache and lower rates of headache improvement/resolution was statistically significant (P-value of 0.01). CONCLUSION: CTV is a reliable noninvasive imaging modality for evaluation of cerebral venous sinuses and stent patency following treatment of idiopathic intracranial hypertension with venous sinus stenting and could be used as a routine follow-up study.

Transplanted Endothelial Progenitor Cells Improve Ischemia Muscle Regeneration in Mice by Diffusion Tensor MR Imaging.

Endothelial progenitor cells (EPCs) play an important role in repairing ischemia tissues. Diffusion tensor imaging (DTI) was applied to detect the architectural organization of skeletal muscle. This study investigated the feasibility and accuracy of using the DTI to evaluate effectiveness of EPCs treatment. Mouse bone marrow-derived EPCs were isolated, cultured, characterized, and transplanted to hindlimb ischemia mice model. DTI was performed on the hindlimb at postischemia time points. The edema regions of diffusion restriction (high signal in diffusion weighted imaging) were decreased in the ischemic muscle of EPCs treated mice after 14 days compared with the controls. These results from DTI show the lower apparent diffusion coefficient and eigenvalues (λ1, λ2, and λ3) and the higher fractional anisotropy and fiber counts of ischemic muscle on 7 and 14 days after EPCs treatment compared to the controls. There was a significant correlation between fiber counts calculated by DTI and survival fibers evaluated by histological section (r = 0.873, P < 0.01). Our study demonstrated that the time frame for muscle fiber regeneration after EPCs transplantation was significantly shortened in vivo. DTI could be a useful tool for noninvasive evaluation of muscle tissue damage and repair in animal models and patient with ischemic diseases.