Intradural Pathology Causing Cerebrospinal Fluid Obstruction in Syringomyelia and Effectiveness of Foramen Magnum and Foramen of Magendie Dredging Treatment.

OBJECTIVE: This article discusses the procedure of foramina magnum and Magendie dredging, summarizing the pathologic changes in the intradural region of the craniocervical junction in patients with syringomyelia and the pathophysiologic mechanism of cerebrospinal fluid (CSF) circulation obstruction. METHODS: Clinical data from 50 adult patients with syringomyelia treated at Xuanwu Hospital from July 2018 to January 2019 were collected and retrospectively analyzed. All operations were performed with foramina magnum and Magendie dredging, and all intradural factors that may have induced the obstruction of CSF circulation were recorded. RESULTS: Intradural pathology was found in all patients. The pathologic changes that may have caused obstruction of the CSF circulation include tonsil occupying the foramen magnum and overlying foramen of Magendie in 88% (44/50), intertonsillar arachnoid adhesions in 36% (18/50), tonsil to medulla arachnoid adhesions in 18% (9/50), medialized tonsils in 70% (35/50), vermian branch of posterior inferior cerebellar artery in 22% (11/50), arachnoid veil in 16% (8/50), cisterna magna cyst in 4% (2/50), and tonsil to dura mater arachnoid adhesions in 8% (4/50). Mean duration of follow-up was 13.3 months. The long-term effective rate was 96.0%. Postoperative magnetic resonance imaging revealed that the size of the syringomyelia was reduced or completely resolved in 88% of patients. The mean preoperative Japanese Orthopaedic Association score was 12.9 ± 3.1, which improved to 14.7 ± 3.2 (P < 0.05) at last clinical follow-up. CONCLUSIONS: Intradural pathology that causes CSF circulation obstruction exists in many forms. Relieving the obstruction of the foramen magnum and foramen of Magendie is key to surgical treatment.

Understanding Subdural Collections in Pediatric Abusive Head Trauma.

Life-threatening physical abuse of infants and toddlers is frequently correlated with head injuries. A common variant of the abusive head trauma is the shaken baby syndrome. The present review article sheds light on subdural collections in children with abusive head trauma and aims at providing a recent knowledge base for various medical disciplines involved in diagnostic procedures and legal proceedings. To this end, the different subdural collection entities are presented and illustrated. The pathophysiologic background is explained. Differential and age-diagnostic aspects are discussed and summarized by tabular and graphic overviews. Two problematic constellations frequently occurring during initial CT investigations are evaluated: A mixed-density subdural collection does not prove repeated trauma, and hypodense subdural collections are not synonymous with chronicity. The neuroradiologic analysis and assessment of subdural collections may decisively contribute to answering differential diagnostic and forensic questions. In addition to more reference data, a harmonization of terminology and methodology is urgently needed, especially with respect to age-diagnostic aspects.

Posttraumatic Bilateral Epidural Hygroma of the Posterior Cranial Fossa: Case Report and Brief Review of Literature.

BACKGROUND: Posttraumatic posterior fossa epidural hygroma is a rare entity, and a clear management has not been established in the medical literature. We present 1 case and review the literature relevant to this unusual entity. The mechanism of formation and management of posterior cranial fossa epidural hygroma are also outlined. CASE DESCRIPTION: A 2-year-old child presented after a rooftop fall injury with symptoms of headache, drowsiness, vomiting, and brief loss of consciousness. Computed tomography scan demonstrated swelling in the left occipital region and epidural hygroma. After conservative management failed, surgical repair of the dura mater was performed. The child was discharged postoperative day 11 in stable condition with marked improvement in occipital swelling. CONCLUSIONS: It is imperative to consider epidural hygroma in very small children presenting with occipital injury. As a result of loose adhesion of dura mater and internal cranial lamina layers in younger pediatric patients, potential epidural space may be easily created secondary to injury, and small breaches in meningeal integrity near the cisterna magna may favor cerebrospinal fluid leak. During surgery, if watertight repair of a visible dural tear is performed, duro-periosteal hitching or vacuum drain placement may not be required.

A calvarial acute subdural hematoma migrating into the spinal canal in a young male.

It is not common for an acute subdural hematoma (SDH) in the supratentorial region to show rapid resolution or migration during the clinical course. In this report, we present a rare case where the SDH in the supratentorial region was observed to rapidly migrate into the lumbar spinal canal, leading to severe radiculopathy. A 20-year-old male patient was admitted to the emergency department with severe headache after head trauma. The patient's overall condition was good, whereas his Glasgow Coma Scale score was 15 and blood pressure was normal. He had vomited 3 times after the onset of pain. No stiff neck was found, and the computed tomography showed an ASDH over the outer layer of the right hemisphere, causing a 7- to 8-mm shift. During the follow-up, the headache regressed and eventually resolved after 12 hours; however, another severe pain occurred in the lumbar region and in both legs. The pain worsened over time, progressing to sciatica in both legs. Acute SDH associated with a minor head trauma may migrate from the supratentorial compartment into the spinal canal by the help of elastic cerebral tissues in young adults and children.

False localizing sign of cervico-thoracic CSF leak in spontaneous intracranial hypotension.

OBJECTIVE: Spontaneous spinal CSF leaks are an important cause of new-onset headaches. Such leaks are reported to be particularly common at the cervico-thoracic junction. The authors undertook a study to determine the significance of these cervico-thoracic CSF leaks. METHODS: The patient population consisted of a consecutive group of 13 patients who underwent surgery for CSF leak repair based on CT myelography showing CSF extravasation at the cervico-thoracic junction but without any evidence of an underlying structural lesion. RESULTS: The mean age of the 9 women and 4 men was 41.2 years. Extensive extrathecal longitudinal CSF collections were demonstrated in 11 patients. At surgery, small leaking arachnoid cysts were found in 2 patients. In the remaining 11 patients, no clear source of CSF leakage could be identified at surgery. Resolution of symptoms was achieved in both patients with leaking arachnoid cysts, but in only 3 of the 11 patients with negative intraoperative findings. Postoperative spinal imaging was performed in 9 of the 11 patients with negative intraoperative findings and showed persistence of the longitudinal intraspinal extradural CSF. Further imaging revealed the site of the CSF leak to be ventral to the thoracic spinal cord. Five of these patients underwent microsurgical repair of the ventral CSF leak with resolution of symptoms in all 5 patients. CONCLUSIONS: Cervico-thoracic extravasation of dye on myelography does not necessarily indicate the site of the CSF leak. Treatment directed at this site should not be expected to have a high probability of sustained improvement of symptoms.

Acute enlargement of subdural hygroma due to subdural hemorrhage in a victim of child abuse.

An 11-month-old female baby was found dead by her mother. Cranial postmortem CT prior to the forensic autopsy showed dilatation of bilateral extra-axial spaces and ventricles. The autopsy revealed a new linear fracture of the left parietal bone and occipital bone, and a healed linear fracture of the right parietal bone and occipital bone like a mirror image of the left one as well. Intracranially, 230ml of subdural fluid were collected, which was mixed with blood. There was a fresh hemorrhage around a bridging vein of the left parietal lobe and the dura mater. Moreover, the outer side of the cerebrum and the inner side of the dura mater were covered by a thin membrane, which mater might have been previously formed because of being positive for Fe-staining and anti-CD68 antibody. A subdural hematoma might have been developed when the right side of the skull was previously fractured, which was transformed into a subdural hygroma. Subsequently, it is likely that, after the left side fracture of the skull occurred, the subdural hygroma rapidly enlarged due to hemorrhaging from the bridging vein, which resulted in intracranial hypertension, because microbleeding was detected in the brain stem. Accordingly, we diagnosed the cause and manner of death as intracranial hypertension due to subdural hemorrhage in subdural hygroma, and homicide, including child abuse, respectively.

Bibrachial amyotrophy and ventral spinal cyst associated with myelomalacia and intracranial hypertension.

It has been recently recognised that patients with ventral intraspinal fluid collections secondary to cerebrospinal fluid leaks can present with bibrachial amyotrophy or mimic Hirayama disease. Here we present two further patients that expand the clinical spectrum of this disorder to include association with myelomalacia and intracranial hypertension.

Electron microscopy of astrocyte changes and subtypes in traumatic human edematous cerebral cortex: a review.

The astrocyte subtypes in moderate and severe human brain trauma complicated with subdural hematoma or hygroma are described. Clear and dense edematous and hypertrophic reactive astrocytes are distinguished in severe vasogenic brain edema. Swollen perineuronal astrocytes appear compressing and indenting dark, degenerated pyramidal and nonpyramidal nerve cells. Glycogen-depleted and glycogen-rich astrocytes also are seen. Reactive hypertrophic astrocytes exhibit increased amounts of dilated smooth and rough endoplasmic reticulum, microtubules, and gliofilaments. Perisynaptic astrocyte ensheathments of neuropil synaptic contacts are lost, and the perivascular astrocyte end-feet appear dissociated from the capillary basement membrane. The interastrocytary gap junctions appear fragmented.

Subdural effusions and lack of early pontocerebellar hypoplasia in siblings with RARS2 mutations.

Mutations in the recently described RARS2 gene encoding for mitochondrial arginyl-transfer RNA synthetase give rise to a disorder characterised by early onset seizures, progressive microcephaly and developmental delay. The disorder was named pontocerebellar hypoplasia type 6 (PCH6) based on the corresponding radiological findings observed in the original cases. We report two siblings with the RARS2 mutation who displayed typical clinical features of PCH6, but who had distinct neuroimaging features. Early scans showed marked supratentorial, rather than infratentorial, atrophy, and the pons remained preserved throughout. One sibling also had bilateral subdural effusions at presentation. The deceleration in head growth pointed to an evolving genetic/metabolic process giving rise to cerebral atrophy and secondary subdural effusions. RARS2 mutations should be considered in infants presenting with seizures, subdural effusions, decelerating head growth and evidence of cerebral atrophy even in the absence of pontocerebellar hypoplasia on imaging.

An MRI rating scale for amyloid-related imaging abnormalities with edema or effusion.

BACKGROUND AND PURPOSE: Immune therapy against amyloid-ß appears to be a promising target in Alzheimer disease. However, a dose-related risk for ARIA on FLAIR images thought to represent parenchymal vasogenic edema or sulcal effusion (termed "ARIA-E"), has been observed in clinical trials. To assess the intensity of ARIA-E presentation, an MR imaging scale that is both reproducible and easily implemented would assist in monitoring and evaluating this adverse event. MATERIALS AND METHODS: On the basis of a review of existing cases from a phase II bapineuzumab study, a scale was constructed with a 6-point score for the 6 regions on each side of the brain (range, 0-60). Scores would be obtained for both parenchymal and sulcal hyperintensities and frequently co-occurring gyral swelling. Inter-rater reliability between 2 neuroradiologists was evaluated in 20 patients, 10 with known ARIA-E and 10 without, by using the intraclass correlation coefficient. RESULTS: The 2 raters had excellent agreement in the identification of ARIA-E cases. A high inter-rater agreement was observed for scores of parenchymal hyperintensity (ICC = 0.83; 95% CI, 48-96) and sulcal hyperintensity (ICC = 0.89; 95% CI, 63-97) and for the combined scores of the 2 ARIA-E findings (ICC = 0.89; 95% CI, 62-97). Gyral swelling scores were observed to have lower inter-rater agreement (ICC = 0.54; 95% CI, -0.06-0.86). CONCLUSIONS: The proposed rating scale provides a reliable and easily implemented instrument to grade ARIA-E imaging findings. We currently do not recommend including swelling.

A new evaluation method for the intracranial volume changes and subdural effusion of patients following endoscopic third ventriculostomy.

OBJECTIVE: Endoscopic third ventriculostomy (ETV) is a procedure commonly applied in the treatment of non-communicating (obstructive) hydrocephalus. One of the rare complications that can occur following ETV is a subdural effusion, even though this procedure is considered to be a more controlled and natural method of cerebrospinal fluid drainage compared to external drainage. In this study, we evaluated the intracranial volume changes and subdural effusion of patients following ETV using Cavalieri method. METHOD: Volumes analysis of the cranial cavity, brain, ventricles and subdural effusions of two patients after ETV were performed on computed tomography images using the Cavalieri principle, one of the stereological methods. RESULTS: The preoperative total intracranial volumes and the preoperative brain volumes decreased for both patients during the postoperative 3rd, 10th and 30th days. Following ETV, the volumes of the lateral ventricles of both patients initially decreased during the postoperative 3rd and 10th days, however, the volumes returned almost to their preoperative size by the end of the 30th day. The effusions were seen on the postoperative 3rd and 10th days resolved by the end of the 30th day. CONCLUSION: Our results show that the Cavalieri method can be used to unbiased prediction of intracranial volume changes and to follow the subdural effusion after the ETV surgery.

MR imaging evaluation of endoscopic cranial base reconstruction with pedicled nasoseptal flap following endoscopic endonasal skull base surgery.

INTRODUCTION: Postoperative CSF leak is the most common complication of endoscopic endonasal approach (EEA) to skull base lesions. Endoscopic multilayer closure of skull base defect using pedicled nasoseptal flaps (NSF) based off the sphenopalatine artery reduces CSF leaks. EEA robustly expands in surgical arena, yet postoperative imaging evaluation remains poorly studied. This work illustrates normal MR imaging appearance of skull base reconstruction utilizing NSFs during immediate postoperative period. METHODS: We retrospectively identified patients who had skull base reconstructions utilizing NSFs following EEAs and immediate postoperative-enhanced brain MRI. NSFs and free grafts were evaluated for signal intensity, thickness, configuration, enhancement, vascular pedicle of NSF, relationship in multilayer reconstruction, and defect coverage. Imaging findings were correlated with surgical technique and CSF leaks. RESULTS: Twenty-eight patients had 26 multilayer reconstructions and 34 NSFs. Twenty-nine NSFs showed enhancing C-shaped arc at the skull base. Of those, 26 flaps (90%) were confidently identified by visualization of their vascular pedicles, 3 were not distinguishable from adjacent mucosa and pedicles were not identified. Five NSFs showed no enhancement (1 CSF leak). Twenty-seven enhancing NSFs approximated defects with close abutment to free grafts. One flap was displaced; one incompletely covered the defect (2 CSF leaks). Fisher exact test demonstrated an association between incomplete defect coverage and displacement of NSFs with CSF leak (P=0.05). CONCLUSION: Endoscopic skull base reconstruction utilizing NSF has characteristic MR imaging appearance. Non-enhancing mucosal gap or displacement of NSF may indicate incomplete defect coverage, identifying patients at risk for CSF leak.

Fluid outflow in a large-animal model of posttraumatic syringomyelia.

BACKGROUND: Posttraumatic syringomyelia affects approximately 28% of spinal cord injury patients, and current treatments are often ineffective. The pathogenesis of this condition remains poorly understood. Previous reports have focused on pathways and mechanisms of fluid inflow; however, disturbances of fluid outflow mechanisms and pathways may be important in syrinx formation and enlargement. OBJECTIVE: To determine the route of fluid outflow from a syrinx in an animal model of posttraumatic syringomyelia. METHODS: A model of posttraumatic syringomyelia using excitotoxic amino acid and kaolin-induced arachnoiditis was created in 12 Merino wethers. Six weeks after syrinx induction, the cavities were localized and a cerebrospinal fluid tracer, horseradish peroxidase (HRP), was injected into the syrinx under ultrasonic guidance. After 10 minutes, the animals were killed and the spinal cords harvested for microscopy. RESULTS: An extracanalicular syrinx developed in 6 of the 12 sheep. HRP was successfully injected into 5 of the 6 syrinx cavities. HRP reaction product was observed in gray and white matter adjacent to the syrinx in a diffuse pattern. There were moderate amounts of HRP around the central canal and perivascular spaces and minimal amounts in the dorsal subarachnoid space. CONCLUSION: In this model of posttraumatic syringomyelia, fluid outflow occurred in a diffuse manner into the surrounding extracellular space and toward the central canal and perivascular spaces. Fluid outflow may be an important consideration in the pathogenesis of syringomyelia and the development of new therapies.

Pathophysiology and management of spontaneous intracranial hypotension--a review.

Spontaneous Intracranial Hypotension is a syndrome involving reduced intracranial pressure secondary to a dural tear which occurs mostly due to connective tissue disorders such as Marfans Syndrome, and Ehler Danlos Syndrome. Patients with dural ectasias leading to CSF leakage into the subdural or epidural space classically present with orthostatic headaches and cranial nerve deficits mostly seen in cranial nerves V-VIII. Diagnosis of SIH is confirmed with the aid of neuroimaging modalities of which Cranial MR imaging is most widely used. SIH can be treated conservatively or with epidural blood patches which are now widely being used to repair dural tears, and their effectiveness is being recognized. Recently epidural injection of fibrin glue has also been used which has been found to be effective in certain patients.

Serious head injury in young children: birth trauma versus non-accidental head injury.

Birth injury of the skull and central nervous system can be a complication of a difficult delivery, especially following forceps or vacuum-assisted delivery. Birth trauma of the head can also mimic the appearance of a non-accidental head injury and is therefore an important differential diagnosis. We report on two young infants with serious head injuries. The difficult differential diagnosis birth trauma versus non-accidental head injury is discussed and the necessity for cooperation between clinicians, forensic doctors and specialized neuroradiologists is emphasized.

The role of MR myelography with intrathecal gadolinium in localization of spinal CSF leaks in patients with spontaneous intracranial hypotension.

BACKGROUND AND PURPOSE: Localization of spinal CSF leaks in CSF hypovolemia is critical in directing focal therapy. In this retrospective review, our aim was to determine whether GdM was helpful in confirming and localizing spinal CSF leaks in patients in whom no leak was identified on a prior CTM. MATERIALS AND METHODS: Forty-one symptomatic patients with clinical suspicion of SIH were referred for GdM after undergoing at least 1 CTM between February 2002 and August 2010. A retrospective review of the imaging and electronic medical records was performed on each patient. RESULTS: In 17 of the 41 patients (41%), GdM was performed for follow-up of a previously documented leak at CTM. In the remaining 24 patients (59%), in whom GdM was performed for a suspected CSF leak, which was not identified on CTM, GdM localized the CSF leak in 5 of 24 patients (21%). In 1 of these 5 patients, GdM detected the site of leak despite negative findings on brain MR imaging, spine MR imaging, and CTM of the entire spine. Sixteen of 17 patients with previously identified leaks underwent interval treatment, and leaks were again identified in 12 of 17 (71%). CONCLUSIONS: GdM is a useful technique in the highly select group of patients who have debilitating symptoms of SIH, a high clinical index of suspicion of spinal CSF leak, and no demonstrated leak on conventional CTM. Intrathecal injection of gadolinium contrast remains an off-label use and should be reserved for those patients who fail conventional CTM.

Anterior horn cell loss from subdural hygroma: a consequence of spontaneous spinal fluid leak.

We describe a case of a 50-year-old man with bilateral shoulder girdle weakness caused by anterior subdural hygroma secondary to a previous spontaneous CSF leak. The CSF leak occurred and resolved 16 years prior to presenting with a 6-year progressive painless, asymmetric proximal muscle weakness involving both upper extremities. Current examination reveals remarkably restricted atrophy and weakness in bilateral C5-6 muscles and absent biceps and brachioradialis reflexes. Neuroimaging shows a subdural CSF collection extending from C1 to L2 anteriorly causing thecal sac effacement at the C4 level and secondary Chiari deformity. The clinical picture demonstrates severe weakness in C5-6 muscles with sparing of all other myotomes. The acute clinical features as well as neuroimaging characteristics of spontaneous CSF leak are well known but the late effects are less described. The development of a subdural fluid collection secondary to a spinal fluid leak can cause damage to the anterior spinal cord years after the leak. The underlying pathophysiology of the motor neuron loss remains unclear but there appears to be a pressure effect localized to the C4-5 region. The possibility that intervention to prevent or treat the subdural CSF collection might have avoided the shoulder girdle weakness is considered.