Posterior Fossa Crowding Research Articles

Atypical Posterior Reversible Encephalopathy Syndrome with Obstructive Hydrocephalus and Impending Tonsillar Herniation. (P6-5.030)

<h3>Objective:</h3> <h3>OBJECTIVE AND IMPORTANCE:</h3> Rhombencephalic edema with hydrocephalus has rarely been described as a manifestation of hypertensive emergency. We present an unusual case of brain edema with hydrocephalus secondary to hypertensive emergency. <h3>Background:</h3> <h3>CASE DESCRIPTION:</h3> A 33-year-old male with uncontrolled hypertension and CKD Stage IV presented to hospital with occipital headache and vomiting. His BP was 230/160. He was treated with Nicardipine infusion. CT head showed cerebellar vasogenic edema and mild tonsillar herniation with partial effacement of the fourth ventricle. MRI confirmed these findings, there was disproportionate dilatation of the lateral and third ventricles with periventricular T2 hyperintense signal consistent with transependymal CSF flow. The headache and neck pain were persistent. The patient was upgraded to Neurocritical Care Unit for monitoring and continued IV BP treatment. Given concerns for incipient tonsillar herniation, Mannitol and Hemodialysis [HD] were added to his medical treatment. After these interventions, his headache and blood pressure management improved. Two days later, CT showed significant interval improvement of hydrocephalus, cerebral edema and posterior fossa crowding. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> <h3>DISCUSSION:</h3> This was an unusual case of extension of PRES to the posterior fossa, leading to hydrocephalus and a vicious cycle of worsening rise in intracranial pressure, leading to worsening refractory systemic hypertension, end organ damage, need for hemodialysis, impending tonsillar herniation and risk of death. <h3>Conclusions:</h3> <h3>CONCLUSION:</h3> This presentation and improvement were compatible with the final diagnosis of an atypical presentation of Posterior Reversible Encephalopathy Syndrome. In this case, reduction of blood pressure, hyperosmolar therapy and dialysis resulted in improvement of the symptoms as well as radiological resolution of hydrocephalus. Aggressive medical treatment was the best approach in this case, but neurosurgical intervention would have been necessary if the patient had not responded to these measures. <b>Disclosure:</b> Dr. Solano has nothing to disclose. Dr. Zafar has nothing to disclose. Dr. Newman has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Clyde Bergstresser, LLC. Dr. Newman has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Maria Rubio LLC. The institution of Dr. Newman has received research support from Albert Einstein Society.

Traumatic vs Spontaneous Cerebrospinal Fluid Hypotension Headache: Our experience in a series of 137 cases

ObjectivesTo analyze and compare differences between epidemiological and clinical aspects, as well as radiologic findings and treatment, in a series of adult patients with traumatic intracranial hypotension (TIH) and spontaneous intracranial hypotension (SIH) treated at our institution in order to identify predictors of recurrence. BackgroundCerebrospinal fluid hypotension headache (CSF-HH) is often caused by orthostasis and relieved by recumbency. Etiology can be either traumatic or spontaneous. Indirect signs of CSF hypotension are often observed on brain MRI. The most common therapeutic approach is conservative management and, when necessary, the use of an epidural blood patch. MethodsMedical history and brain MRI of adult patients consulting our institution with a diagnosis of CSF-HH between January 2010 and March 2019, were retrospectively reviewed. Clinical criteria as per the International Classification of Headache Disorders, 3rd edition, were applied. Presence of typical MRI findings were assessed by two experienced neuroradiologists, previously informed of patients’ clinical characteristics. Patients were divided into two different groups, namely: Group A: Spontaneous Intracranial Hypotension (SIH) and Group B: Traumatic Intracranial Hypotension (TIH). Recurrence was defined as return of symptoms after one month of remission. In order to find predictors of recurrent intracranial hypotension the patients were divided into three groups: Recurrent Orthostatic headache (ROH); (33 cases; 25%); Non-Recurrent Orthostatic headache (NROH) (84; 61%) and Patients missing follow-up (20; 15%). The latter were excluded from the regression analysis. Results137 patients with CSF-HH were identified: 80 traumatic (54 women, age 33.8 ± 10.4 years) and 57 spontaneous (31 women, age 43.9 ± 15.2 years). Median follow-up was 35 months (range: 8 months–9 years).Compared with TIH, patients with SIH showed lower frequency of orthostatic headache and higher frequency of aural fullness.Also, in patients with SIH, brain MRI showed a higher frequency of pachymeningeal enhancement, ventricular collapse, cisternal obliteration, posterior fossa crowding, brainstem distortion, and a more likely presence of subdural collections.Patients with SIH required an epidural blood patch treatment more often, showed higher recurrence rates, and were more prone to develop subdural hematomas. RecurrenceAs more days elapsed between headache onset and clinical consultation, the presence of spontaneous intracranial hypotension, tinnitus, and thoraco-lumbo-sacral pain were all more common in patients with recurrence.Findings on brain MRI suggesting higher recurrence rates in patients included: ventricular collapse, brainstem distortion, and posterior fossa crowding.Patients treated with invasive therapy (epidural blood patch) presented a higher recurrence rate.In the multivariate regression analysis, the only independent predictor of recurrence after adjusting for age, sex and traumatic vs spontaneous cause of IH, was brainstem distortion diagnosed on MRI (OR 5.13, 95% CI: 1.2-21.7; p = 0.026). ConclusionsSIH and TIH can no longer be likened, since there is considerable variability in clinical presentation, imaging findings, response to treatment and recurrence rates.Anatomical abnormalities underlying SIH leaks are often complex and not simply a disruption of normal structures as encountered in TIH, which could explain why treatment success is poor and recurrence rates remain high.

Experiences in Performing Posterior Calvarial Distraction.

The use of posterior calvarial distraction (PCD) for the management of craniosynostosis is well recognized. The advantages of using this technique include increased cranial volume, decreased intracranial pressure, relief of posterior fossa crowding, improved cerebrospinal fluid (CSF) circulation at the cranio-cervical junction with cessation, and possible resolution of syrinx.The authors retrospectively review their first 50 patients who have undergone PCD under the senior author's care in our unit.The demographics, diagnoses, intraoperative approach with techniques in distractor placement and outcomes of each patient were obtained through an electronic craniofacial database and written patient records. Analysis of complication rates (bleeding, distraction problems, CSF leaks, and infection) was included.A total of 31 boys and 19 girls underwent the procedure between October 2006 and September 2015 with a median age was 17.7 months (range 4 months to 19 years). Of those 50 children, 34 of the cohort were proven to be syndromic by genetic testing.The median length of inpatient stay was 9.4 days (range 3-43 days). Average distraction distance was 24 mm.Complications including CSF leaks, bleeding, distractor problems, and severe complications (recorded in 3 patients) are discussed. Our overall complication rate was 50%.Favorable outcomes included resolution of Chiari, syrinx, and raised intracranial pressure in the majority of patients where distraction was successful.The authors recommend that PCD should be considered the primary treatment for increasing calvarial volume. The authors discuss our experiences and technical innovations over the past decade.

Costello syndrome: Analysis of the posterior cranial fossa in children with posterior fossa crowding.

This study aimed to assess changes in the posterior cranial fossa (PCF) to shed light on the mechanism of cerebellar herniation in children with Costello syndrome (CS) and posterior fossa crowding. We performed a morphovolumetric PCF analysis on brain magnetic resonance imaging (MRI) in seven children with CS (mean age 31 ± 16 months) comparing the MRI scans with those of seven age-matched healthy subjects.PCF volume (PCFV), PCF brain volume (PCFBV) and cerebellar volume (CeV) were assessed on axial T2-weighted MRI. Morphometric parameters (diameters of the foramen magnum, tentorial angle, basiocciput, supraocciput, basisphenoid and exocciput lengths) were measured on sagittal T1-weighted MRI. The volume of the cerebrospinal fluid (CSF) spaces was calculated as PCFV minus PCFBV.Five out of seven CS children showed tonsillar herniation in the upper cervical canal; no child had hydrocephalus but three out of seven children showed ventriculomegaly. In addition, the PCFV/PCFBV ratio, PCFV, CSF spaces volume, basiocciput, basisphenoid and exocciput lengths and latero-lateral and antero-posterior diameters of the foramen magnum were significantly reduced, whereas no significant changes were found in supraocciput length, PCFBV, CeV or hindbrain volume. The volumetric reduction of the PCF due to bony posterior fossa hypoplasia is a predisposing factor for developing cerebellar tonsillar herniation through the foramen magnum in children with CS. The altered anatomy of the foramen magnum and upward expansion of the PCF secondary to an increased tentorial slope serves to explain the possible mechanism of cerebellar herniation in patients with CS.

Low-pressure/spinal fluid leak headache.

Low cerebrospinal fluid (CSF) pressure results in neurologic deficits, of which the most common manifestation is headache. Typically, the headache is postural - and specifically, orthostatic - in presentation. There are three hypotheses to explain the occurrence of headache associated with low CSF fluid. The first is traction on pain-sensitive intracranial and meningeal structures; the second is CSF hypovolemia; and the third is spinal loss of CSF resulting in increased compliance at the caudal end of the CSF space. Spontaneous intracranial hypotension (SIH), once believed to be rare, is now more commonly recognized. It is typically associated with orthostatic headache (although initially it may not be) and one or more other symptoms such as alterations in hearing, nausea, vomiting, neck stiffness, diplopia, and visual field cuts. Magnetic resonance imaging (MRI) of the brain with gadolinium is the first study of choice, which typically reveals diffuse pachymeningeal enhancement and, frequently, cerebellar tonsillar descent and posterior fossa crowding. Epidural blood patch (EBP) is the treatment of choice. Surgery and epidural fibrin glue injection are options for those who fail conservative therapy and/or EBP.

Phenotypic analysis of individuals with Costello syndrome due to HRAS p.G13C

Costello syndrome is characterized by severe failure-to-thrive, short stature, cardiac abnormalities (heart defects, tachyarrhythmia, and hypertrophic cardiomyopathy (HCM)), distinctive facial features, a predisposition to papillomata and malignant tumors, postnatal cerebellar overgrowth resulting in Chiari 1 malformation, and cognitive disabilities. De novo germline mutations in the proto-oncogene HRAS cause Costello syndrome. Most mutations affect the glycine residues in position 12 or 13, and more than 80% of patients share p.G12S. To test the hypothesis that subtle genotype-phenotype differences exist, we report the first cohort comparison between 12 Costello syndrome individuals with p.G13C and individuals with p.G12S. The individuals with p.G13C had many typical findings including polyhydramnios, failure-to-thrive, HCM, macrocephaly with posterior fossa crowding, and developmental delay. Subjectively, their facial features were less coarse. Statistically significant differences included the absence of multifocal atrial tachycardia (P-value = 0.033), ulnar deviation of the wrist (P < 0.001) and papillomata (P = 0.003), and fewer neurosurgical procedures (P = 0.024). Fewer individuals with p.G13C had short stature (height below -2 SD) without use of growth hormone (P < 0.001). The noteworthy absence of malignant tumors did not reach statistical significance. Novel ectodermal findings were noted in individuals with p.G13C, including loose anagen hair resulting in easily pluckable hair with a matted appearance, different from the tight curls typical for most Costello syndrome individuals. Unusually long eye lashes requiring trimming are a novel finding we termed dolichocilia. These distinctive ectodermal findings suggest a cell type specific effect of this particular mutation. Additional patients are needed to validate these findings.

Iatrogenic complications following shunting the subarachnoid space in infancy

Background Of those patients who require extracranial shunting to manage CSF circulation disorders, some of the most challenging are those whose shunts have been placed in the subarachnoid space in infancy or childhood. While clinically well in childhood, with few if any revisions, a symptom complex may develop in mid to late childhood that is characterized by chronic, often non-specific headaches and intermittent intracranial hypertension associated with abducens palsy and/or papilloedema. Imaging shows some combination of decompressed CSF spaces, acquired hindbrain herniation or posterior fossa crowding, diploic hypertrophy, narrow spinal canal and meningeal thickening. ICP monitoring does not always provide clarification of the clinical ICP correlation.

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High incidence of progressive postnatal cerebellar enlargement in Costello syndrome: Brain overgrowth associated with HRAS mutations as the likely cause of structural brain and spinal cord abnormalities

Costello syndrome is a rasopathy caused by germline mutations in the proto-oncogene HRAS. Its presentation includes failure-to-thrive with macrocephaly, characteristic facial features, hypertrophic cardiomyopathy, papillomata, malignant tumors, and cognitive impairment. In a systematic review we found absolute or relative macrocephaly (100%), ventriculomegaly (50%), and other abnormalities on brain and spinal cord imaging studies in 27/28 individuals. Posterior fossa crowding with cerebellar tonsillar herniation (CBTH) was noted in 27/28 (96%), and in 10/17 (59%) with serial studies posterior fossa crowding progressed. Sequelae of posterior fossa crowding and CBTH included hydrocephalus requiring shunt or ventriculostomy (25%), Chiari 1 malformation (32%), and syrinx formation (25%). Our data reveal macrocephaly with progressive frontal bossing and CBTH, documenting an ongoing process rather than a static congenital anomaly. Comparison of images obtained in young infants to subsequent studies demonstrated postnatal development of posterior fossa crowding. This process of evolving megalencephaly and cerebellar enlargement is in keeping with mouse model data, delineating abnormal genesis of neurons and glia, resulting in an increased number of astrocytes and enlarged brain volume. In Costello syndrome and macrocephaly-capillary malformation syndrome disproportionate brain growth is the main factor resulting in postnatal CBTH and Chiari 1 malformation.

Hydrocephalus in Paget’s disease

Neurological manifestations in Paget's disease are rare. Hydrocephalus is infrequent, its etiology in Paget's disease being multi-factorial. Surgical and anesthetic procedures may be challenging, requiring special precautions. We describe one such case and discuss these peculiarities. An elderly lady, diagnosed to have Paget's disease presented with features of recent onset dementia, gait disturbances with urinary incontinence. Hydrocephalus secondary to posterior fossa crowding was present. A ventriculo-peritoneal shunt was inserted. Endotracheal intubation proved to be difficult. Modification of the surgical technique was required to ensure a safe procedure. She improved immediately following CSF diversion. Timely CSF diversion yields good results. Certain precautions during anesthesia and surgery can ensure a safe and uneventful peri-operative period.

Low Cerebrospinal Fluid Pressure Headache.

Alterations in cerebrospinal fluid (CSF) pressure lead to neurologic symptoms, the most common clinical manifestation of which is headache. Typically, the headache is orthostatic and related to traction on pain-sensitive intracranial and meningeal structures, distention on periventricular pain-sensitive areas, and direct pressure on pain conveying cranial nerves. Low CSF headache is a distinct and familiar syndrome that is seen most frequently following lumbar puncture. In this clinical scenario, the diagnosis and proposed plan of treatment are obvious. Over the past decade, however, an emerging syndrome of spontaneous intracranial hypotension (SIH) is being recognized with increasing frequency. Most of these patients are found to have spontaneous CSF leaks and have unique, clinically distinct imaging findings, which confirm the diagnosis leading to appropriate treatment. Spontaneous intracranial hypotension is a relatively benign and usually self-limiting syndrome of orthostatic headache in association with one or more of numerous symptoms including nausea, vomiting, horizontal diplopia, unsteadiness or vertigo, altered hearing, neck pain/stiffness, interscapular pain, and occasionally visual field cuts. The headache itself, while often orthostatic, may initially be non-positional, may lose its orthostatic features, or rarely or never be orthostatic. It may be gradual, subacute, or thunderclap in onset. There may be a history of minor, antecedent trauma. By very definition, the opening CSF pressure is low, below 60 mm H(2)O, and often a "dry" tap is encountered. However, the pressure may be normal, especially with intermittent leaks and may vary tap to tap. Fluid analysis is normal. Brain (and occasionally spinal) MRI studies, with gadolinium enhancement should be undertaken. In patients with SIH, studies typically reveal diffuse pachymeningeal enhancement, frequently in association with "sagging"of the brain, tonsilar descent, and posterior fossa crowding. Spinal MRI is an up and coming investigational technique, which may be helpful even in the case of a normal brain MRI. Computed tomography myelography is the diagnostic study of choice and may follow radiocisternography, which often shows absence of activity over the convexities and early appearance of activity in the renal/urinary tract. Although conservative measures are often undertaken first, epidural blood patch (EBP) is the treatment of choice. For those who fail EBP, surgery may need to be undertaken in those cases with clearly identified leaks.