Posterior Fossa Volume Research Articles

Primary trigeminal neuralgia is associated with posterior fossa crowdedness: A prospective case-control study

Neurovascular conflict (NVC) has been postulated to be the underlying cause of trigeminal neuralgia (TN). Does the posterior fossa crowdedness increase the chance of NVC? The aim of this study was to quantitatively measure the posterior fossa crowdedness in patients with TN and to perform a comparison with healthy controls. We conducted a prospective case-control study of 46 patients diagnosed with primary TN and 46 sex- and age-matched healthy controls. All subjects underwent high-resolution three-dimensional MRI, and the 3D Slicer software was used to measure the posterior fossa volume (PFV) and hindbrain volume (HBV). The posterior fossa crowdedness index (PFCI) was calculated as HBV/PFV × 100%. The results showed that patients with TN had a larger HBV (155.4 ± 23.2 cm3 versus 152.9 ± 13.5 cm3, P = .16) and a smaller PFV (182.7 ± 18.3 cm3 versus 186.1 ± 11.7 cm3, P = .42) as compared to control subjects, but these values were not significantly different. The mean PFCI was significantly higher in patients with TN than in controls (85.1% ± 3.4% versus 82.2% ± 5.3%; P = .03). Women had a more crowded posterior fossa than men (85.8% ± 2.1% versus 84.1% ± 2.6%; P = .023) in patients with TN. The correlation analysis showed that a higher PFCI was associated with younger age (P = .02), woman (P = .014), and TN disease (P = .001). From this study, we conclude that patients with TN have a more crowded posterior fossa than healthy subjects. Women, younger age and TN disease are associated with a higher PFCI. The posterior fossa crowdedness may be a risk factor of NVC, and thus more likely to result in the genesis of TN.

Microvascular decompression for adolescent-onset cranial neuropathies

Objective To analyze the epidemiology, pathogenesis, clinical features of adolescent-onset cranial neuropathies (ACN) and to discuss the surgical effects and complications of microvascular decompression (MVD). Methods We retrospectively analyzed 37 ACN patients (including 34 successfully followed up) who underwent MVD at Department of Neurosurgery, China-Japan Friendship Hospital from March 2001 to October 2016 (ACN group) and compared them with 210 cases of adult cranial neuropathies (CN) which included 188 successfully followed up and were extracted by random sampling method during the same period (adult CN group). Results The symptoms of 37 cases in ACN group were unilateral. The average age of onset was 16.7±1.5 years. Compared with the adult CN group, the rate of affecting the right side (56.7% vs. 41.0%), the ratio of arachnoid thickening in cerebellopontine angle (CPA) region (78.4% vs. 25.7%), the rate of narrow posterior fossa (54.0% vs. 28.6%) and incidence of acoustic complications (14.7% vs. 4.3%) were all higher in ACN group, which however demonstrated a lower rate of AICA oppression (24.3% vs. 42.8%). Those differences were statistically significant (all P 0.05). Conclusions ACN seems to be rare and more commonly affect the right side. Arachnoid thickening in CPA region and the small volume of posterior cranial fossa may be the pathogenic factors. MVD could be a safe and effective surgical method for ACN in which the operational efficiency is almost the same as that of adult CN, while the incidence of complications in ACN group is suggested to be higher than that in the group of adult CN. Key words: Cranial neuropathy; Microvascular decompression; Adolescent; Disease attributes

The association between the pulse pressure gradient at the cranio-cervical junction derived from phase-contrast magnetic resonance imaging and invasively measured pulsatile intracranial pressure in symptomatic patients with Chiari malformation type 1.

In symptomatic Chiari malformation type 1 (CMI), impaired intracranial compliance (ICC) is associated with an increased cranio-spinal pulsatile pressure gradient. Phase-contrast magnetic resonance imaging (MRI) represents a non-invasive modality for the assessment of the pulse pressure gradient at the cranio-cervical junction (CCJ). We wished to explore how the MRI-derived pulse pressure gradient (MRI-dP) compares with invasively measured pulsatile intracranial pressure (ICP) in CMI, and with healthy controls. From phase-contrast MRI of CMI patients and healthy controls, we computed cerebrospinal fluid (CSF) flow velocities and MRI-dP at the CCJ. We assessed bidirectional flow and compared the flow between the anterior and the posterior subarachnoid space at the CCJ. We computed total intracranial volume (ICV), ventricular CSF volume (VV), and posterior cranial fossa volume (PCFV). We analyzed the static and pulsatile ICP scores from overnight monitoring in CMI patients. Five CMI patients and four healthy subjects were included. The CMI group had a significantly larger extent of tonsillar ectopia, smaller PCFV, and a smaller area of CSF in the FM. The pulsatile ICP (mean ICP wave amplitude, MWA) was abnormally increased in 4/5 CMI patients and correlated positively with MRI-dP. However, the MRI-dP as well as the CSF flow velocities did not differ significantly between CMI and healthy subjects. Moreover, bidirectional flow was observed in both CMI as well as healthy subjects, with no significant difference. In symptomatic CMI patients, we found a significant association between the pulse pressure gradient at the CCJ derived from phase-contrast MRI and the pulsatile ICP (MWA) measured invasively. However, the MRI-dP was close to identical in CMI patients and healthy subjects. Moreover, the CSF flow velocities at the CCJ and the occurrence of bidirectional flow were not different in CMI patients and healthy individuals. Further studies are required to determine the diagnostic role of phase-contrast MRI in CMI patients.

Simultaneous total intracranial volume and posterior fossa volume estimation using multi-atlas label fusion.

Total intracranial volume (TICV) is an essential covariate in brain volumetric analyses. The prevalent brain imaging software packages provide automatic TICV estimates. FreeSurfer and FSL estimate TICV using a scaling factor while SPM12 accumulates probabilities of brain tissues. None of the three provide explicit skull/CSF boundary (SCB) since it is challenging to distinguish these dark structures in a T1-weighted image. However, explicit SCB not only leads to a natural way of obtaining TICV (i.e., counting voxels inside the skull) but also allows sub-definition of TICV, for example, the posterior fossa volume (PFV). In this article, they proposed to use multi-atlas label fusion to obtain TICV and PFV simultaneously. The main contributions are: (1) TICV and PFV are simultaneously obtained with explicit SCB from a single T1-weighted image. (2) TICV and PFV labels are added to the widely used BrainCOLOR atlases. (3) Detailed mathematical derivation of non-local spatial STAPLE (NLSS) label fusion is presented. As the skull is clearly distinguished in CT images, we use a semi-manual procedure to obtain atlases with TICV and PFV labels using 20 subjects who both have a MR and CT scan. The proposed method provides simultaneous TICV and PFV estimation while achieving more accurate TICV estimation compared with FreeSurfer, FSL, SPM12, and the previously proposed STAPLE based approach. The newly developed TICV and PFV labels for the OASIS BrainCOLOR atlases provide acceptable performance, which enables simultaneous TICV and PFV estimation during whole brain segmentation. The NLSS method and the new atlases have been made freely available. Hum Brain Mapp 38:599-616, 2017. © 2016 Wiley Periodicals, Inc.

Influence of Posterior Fossa Volume on Clinical Outcomes After Vestibular Schwannoma Resection.

To determine the influence of posterior fossa dimensions on surgical outcomes after vestibular schwannoma management. Retrospective chart review. Tertiary care academic medical center. Patients with sporadic vestibular schwannomas who underwent surgical resection via retrosigmoid or translabyrinthine techniques. Diagnostic. One and two-way analysis of variance (ANOVAs) of the cohort based on volumetric and clinical outcome groupings. A total of 95 patients were identified. A one-way ANOVA looking at the entire cohort of patients showed that outcomes like surgical time (p < 0.001) and whether a Good Outcome (House-Brackmann score of 1 or 2, no complications, and a complete resection) was achieved (p = 0.009) correlated very well with preoperative tumor volume, but not with posterior fossa volume (p = 0.412 and p = 0.345, respectively). However, in medium sized tumors, House-Brackmann group was correlated with posterior fossa volume (p = 0.032). The present data suggest a small effect of posterior fossa size on some clinical outcomes in medium sized vestibular schwannomas.

Two distinct populations of Chiari I malformation based on presence or absence of posterior fossa crowdedness on magnetic resonance imaging.

OBJECTIVE A subset of patients with Chiari I malformation demonstrate patent subarachnoid spaces around the cerebellum, indicating that reduced posterior fossa volume alone does not account for tonsillar descent. The authors distinguish two subsets of Chiari I malformation patients based on the degree of "posterior fossa crowdedness" on MRI. METHODS Two of the coauthors independently reviewed the preoperative MR images of 49 patients with Chiari I malformation and categorized the posterior fossa as "spacious" or "crowded." Volumetric analysis of posterior fossa structures was then performed using open-source DICOM software. The preoperative clinical and imaging features of the two groups were compared. RESULTS The posterior fossae of 25 patients were classified as spacious and 20 as crowded by both readers; 4 were incongruent. The volumes of the posterior fossa compartment, posterior fossa tissue, and hindbrain (posterior fossa tissue including herniated tonsils) were statistically similar between the patients with spacious and crowed subtypes (p = 0.33, p = 0.17, p = 0.20, respectively). However, patients in the spacious and crowded subtypes demonstrated significant differences in the ratios of posterior fossa tissue to compartment volumes as well as hindbrain to compartment volumes (p = 0.001 and p = 0.0004, respectively). The average age at surgery was 29.2 ± 19.3 years (mean ± SD) and 21.9 ± 14.9 years for spacious and crowded subtypes, respectively (p = 0.08). Syringomyelia was more prevalent in the crowded subtype (50% vs 28%, p = 0.11). CONCLUSIONS The authors' study identifies two subtypes of Chiari I malformation, crowded and spacious, that can be distinguished by MRI appearance without volumetric analysis. Earlier age at surgery and presence of syringomyelia are more common in the crowded subtype. The presence of the spacious subtype suggests that crowdedness alone cannot explain the pathogenesis of Chiari I malformation in many patients, supporting the need for further investigation.

Chiari I Malformation in the Adult

Chiari malformation is a designation for a group of alterations in the posterior fossa architecture having the effect of reducing posterior fossa volume, causing descent of the cerebellar tonsils and occasionally the hindbrain as well as an associated disturbance of normal cerebrospinal fluid (CSF)

Vascular tortuosity in relationship with hypertension and posterior fossa volume in hemifacial spasm

BackgroundHemifacial spasm (HFS) is a disabling neurological condition. Vascular tortuosity in HFS patients has not been quantified objectively and its relationship with hypertension and posterior fossa volume (PF) is unknown. In a case control magnetic resonance imaging and angiographic (MRI/A) study, we quantified and compared the vascular tortuosity in HFS and controls, and evaluated its relationship with hypertension and PF.MethodsUsing a commercially available vessel probe tool, an index of tortuosity based on vessel over chord length was employed to quantify vascular tortuosity of the vertebral (VA) and basilar arteries (BA) in 79 subjects (40 HFS, 39 controls).ResultsThe tortuosity index of the BA (1.09 vs 1.16, p = 0.26, 95 % CI 1.07, 1.23), RVA (1.15 vs 1.15, p = 0.83, 95 % CI 1.06, 1.38) and LVA (1.14 vs 1.288, p = 0.16, 95 % CI 1.14, 1.44) was not different between HFS and controls, with adjustments for PF volume and hypertension.ConclusionsContrary to popular belief, our study showed that taking into account hypertension and PF volume, vascular tortuosity of the vertebrobasilar arteries is unlikely to be a major etiologic factor in HFS, though its role in select individuals cannot be excluded. The complex interplay of facial nerve hyperexcitability, genetic predisposition, vascular tortuosity, posterior fossa volume and hypertension needs to be further evaluated.

Human Foramen Magnum Area and Posterior Cranial Fossa Volume Growth in Relation to Cranial Base Synchondrosis Closure in the Course of Child Development.

To date, no study has compared the evolution of the foramen magnum area (FMA) and the posterior cranial fossa volume (PCFV) with the degree of cranial base synchondrosis ossification. To illustrate these features in healthy children. The FMA, the PCFV, and the ossification of 12 synchondroses according to the Madeline and Elster scale were retrospectively analyzed in 235 healthy children using millimeter slices on a computed tomography scan. The mean FMA of 6.49 cm in girls was significantly inferior to the FMA of 7.67 cm in boys (P < .001). In both sexes, the growth evolved in a 2-phase process, with a phase of rapid growth from birth to 3.75 years old (yo) followed by a phase of stabilization. In girls, the first phase was shorter (ending at 2.6 yo) than in boys (ending at 4.33 yo) and proceeded at a higher rate. PCFV was smaller in girls (P < .001) and displayed a biphasic pattern in the whole population, with a phase of rapid growth from birth to 3.58 yo followed by a phase of slow growth until 16 yo. In girls, the first phase was more active and shorter (ending at 2.67 yo) than in boys (ending at 4.5 yo). The posterior interoccipital synchondroses close first, followed by the anterior interoccipital and occipitomastoidal synchondroses, the lambdoid sutures simultaneously, then the petro-occipital and spheno-occipital synchondroses simultaneously. The data provide a chronology of synchondrosis closure. We showed that FMA and PCFV are constitutionally smaller in girls at birth (P ≤ .02) and suggest that a sex-related difference in the FMA is related to earlier closure of anterior interoccipital synchondroses in girls (P = .01). AIOS, anterior interoccipital synchondrosesFMA, foramen magnum areaLS, lambdoid suturesOMS, occipitomastoidal synchondrosesPCFV, posterior cranial fossa volumePIOS, posterior interoccipital synchondrosesPOS, petro-occipital synchondrosesSOS, spheno-occipital synchondrosisyo, years old.

3-D simulation of posterior fossa reduction in Chiari I.

We proposed a 3D model to evaluate the role of platybasia and clivus length in the development of Chiari I (CI). Using a computer aided design software, two DICOM files of a normal CT scan and MR were used to simulate different clivus lengths (CL) and also different basal angles (BA). The final posterior fossa volume (PFV) was obtained for each variation and the percentage of the volumetric change was acquired with the same method. The initial normal values of CL and BA were 35.65 mm and 112.66º respectively, with a total PFV of 209 ml. Ranging the CL from 34.65 to 29.65 - 24.65 - 19.65, there was a PFV decrease of 0.47% - 1.12% - 1.69%, respectively. Ranging the BA from 122.66º to 127.66º - 142.66º, the PFV decreased 0.69% - 3.23%, respectively. Our model highlights the importance of the basal angle and clivus length to the development of CI.

Morphometric Analysis of Predictors of Cervical Syrinx Formation in the Setting of Chiari I Malformation

Background/Aims: We performed a morphometric analysis of Chiari I malformations to look for predictors of cervical syrinx formation. Methods: Eighteen patients with Chiari I malformation and associated cervical syrinx and 16 patients with Chiari I malformation without associated cervical syrinx were included in the study. Chiari I size was obtained from the radiology report; foramen magnum diameter, cerebellar volume, posterior fossa volume and intracranial volume were calculated using OsiriX software, and average measurements were compared between the two groups. Results and Conclusion: Patients with Chiari I with syrinx had an average tonsillar descent of 13.03 ± 5.31 mm compared to 9.25 ± 3.31 mm in the Chiari I without syrinx group (p < 0.05). Patients with Chiari I and syrinx also showed increased cerebellar crowding with a higher cerebellar volume to posterior fossa volume ratio; however, this difference was not significant (0.83 vs. 0.81; p = 0.1872). No difference between groups was found in posterior fossa volume, intracranial volume and foramen magnum diameter. Therefore, only Chiari I size based on the extent of tonsillar herniation was found to be a determinant of cervical syrinx formation.

Diffusion tensor imaging assessment of microstructural brainstem integrity in Chiari malformation Type I.

OBJECTIVE The diagnosis of Chiari malformation Type I (CM-I) is primarily based on the degree of cerebellar tonsillar herniation even though it does not always correlate with symptoms. Neurological dysfunction in CM-I presumably results from brainstem compression. With the premise that conventional MRI does not reveal brain microstructural changes, this study examined both structural and microstructural neuroimaging metrics to distinguish patients with CM-I from age- and sex-matched healthy control subjects. METHODS Eight patients with CM-I and 16 controls were analyzed. Image postprocessing involved coregistration of anatomical T1-weighted with diffusion tensor images using 3D Slicer software. The structural parameters included volumes of the posterior fossa, fourth ventricle, and tentorial angle. Fractional anisotropy (FA) was calculated separately in the anterior and posterior compartments of the lower brainstem. RESULTS The mean age of patients in the CM-I cohort was 42.6 ± 10.4 years with mean tonsillar herniation of 12 mm (SD 0.7 mm). There were no significant differences in the posterior fossa volume (p = 0.06) or fourth ventricular volume between the 2 groups (p = 0.11). However, the FA in the anterior brainstem compartment was significantly higher in patients with CM-I preoperatively (p = 0.001). The FA values normalized after Chiari decompression except for persistently elevated FA in the posterior brainstem compartment in patients with CM-I and syrinx. CONCLUSIONS In this case-control study, microstructural alterations appear to be reliably associated with the diagnosis of CM-I, with a significantly elevated FA in the lower brainstem in patients with CM-I compared with controls. More importantly, the FA values normalized after decompressive surgery. These findings should be validated in future studies to determine the significance of diffusion tensor imaging-based assessment of brainstem microstructural integrity as an adjunct to the clinical assessment in patients with CM-I.

Measuring posterior fossa with neuronavigation in chiari type 1 malformation patients and the relation between syrinx cavity and posterior fossa volume

Measuring posterior fossa with neuronavigation in chiari type 1 malformation patients and the relation between syrinx cavity and posterior fossa volume

Volume change theory for syringomyelia: A new perspective.

Background:The etiopathogenesis of syringomyelia is still an enigma. The authors present a novel theory based on fluid dynamics at the craniovertebral (CV) junction to explain the genesis of syringomyelia (SM). The changes in volume of spinal canal, spinal cord, central canal and spinal subarachnoid space (SSS) in relation to the posterior fossa have been analysed, specifically during postural movements of flexion and extension. The effect of fluctuations in volume of spinal canal and its contents associated with cerebrospinal fluid (CSF) flow dynamics at the CV junction have been postulated to cause the origin and propagation of the syringomyelia. The relevant literature on the subject has been reviewed and the author's theory has been discussed.Conclusion:Volume of spinal canal in flexion is always greater than that in extension. Flexion of spine causes narrowing of the ventral subarachnoid space (SAS) and widening of dorsal SAS while extension causes reverse changes leading to fluid movement in dorsal spinal SAS in flexion and ventral spinal SAS in extension. Cervical and lumbar spinal region with maximum bulk hence maximum area and volume undergo maximum deformation during postural changes. SSS CSF is the difference between the volume of spinal canal and spinal cord, varies in flexion and extension which is compensated by changes in posterior fossa (CSF) volume in normal circumstances. Blocked SAS at foramen magnum donot permit spinal SAS CSF exchange which during postural changes is compensated by cavitatory/cystic (syrinx) change at locations in cervical and lumbar spine with propensity for maximum deformation. Augmentation of posterior fossa volume by decompression helps by normalization of this CSF exchange dynamics but immobilizing the spinal movement theoretically will cease any dynamic volume changes thereby minimizing the destructive influence of the fluid exchange on the cord. Thus, this theory strengthens the rational of treating patients by either methodology.

Application of neuroendoscopy in primary hemifacial spasm surgery

Objective To investigate the application value of neuroendoscope techniques in primary hemifacial spasm of the local complex anatomy aiming to the difficulties of primary hemifacial spasm surgery. Methods Forty-two patients with hemifacial spasm of the local complex anatomy were treated with neuroendoscope-assisted microvascular decompression at the Department of Neurosurgery, Wuxi No. 2 Hospital Affiliated to Nanjing Medical University from January 2008 to January 2012. When the blind area appeared during the operation, a neuroendoscope was used to perform comprehensive observation of the surgical area, exploring the facial nerves from the brain stem area and distal end, and carefully distinguishing the relationship between the nerves and the blood vessels. The offending vessels were confirmed. After decompression, a neuroendoscope was use to reexamine whether there were omission of offending vessels and vascular excessive stretch, twisting in an angular shape, or formation of a new compression. The clinical and imaging data of all patients were analyzed retrospectively and were followed up after procedure. Results All patients were followed for 18-30 months (mean 25 months). Forty-one patients had complete remission after procedure without recurrence (98%), 3 recovered from Shorr grade Ⅰ to grade 0 at discharge, 1 with hearing loss was fully restored within 6 months, and 1 with Shorr grade Ⅱ did not obviously aggravate at the end of follow-up. Conclusions Neuroendoscopy is an useful complement to microvascular decompression for the treatment of hemifacial spasm, particularly in patients with local anatomical complex or unusual hemifacial spasm, such as combining the advantages of neuroendoscopy may improve the operation effect under the conditions of small posterior fossa volume, full cerebellum pompoms, rock bone block, local arachnoid adhesions and thickening, and identifying offending vessels with difficulty. Key words: Facia1 spasm; Neuroendoscope; Microvascular decompression

Comparison of pulsatile and static pressures within the intracranial and lumbar compartments in patients with Chiari malformation type 1: a prospective observational study.

In Chiari malformation type 1 (CMI), the obstruction of cerebrospinal fluid (CSF) flow through the foramen magnum is believed to cause alterations of intracranial pressure (ICP) pulsations. Foramen magnum decompression (FMD) is therefore considered a treatment of choice. However, the pathophysiology of CMI is poorly understood and it remains unknown how ICP alterations relate to symptoms and radiological findings. This study was undertaken to measure pulsatile pressure and its gradient between intracranial and lumbar compartments, and to determine its relationship to clinical and radiological findings. In patients with symptomatic CMI, we simultaneously measured ICP and lumbar CSF pressure, with particular focus on analysis of pulsatile pressure. Ventricular CSF volume (VV), intracranial volume (ICV) and posterior cranial fossa volume (PCFV) were calculated using volumetry software. In 26 patients (median 35 years), we found clearly abnormal or borderline values of pulsatile ICP in 18/26 patients (69 %; median 4.5 mmHg) and abnormal pulsatile pressure gradient in 17/24 patients (71 %; median 2.6 mmHg). The correlation between pulsatile ICP and the pulsatile pressure gradient was significantly positive (p < 0.001). We found no significant correlation between pulsatile or static pressure and extent of tonsillar ectopy, VV, ICV or PCFV. The pulsatile pressure gradient was significantly higher in patients with syringomyelia (p = 0.02). In this cohort, the pulsatile ICP was elevated in 69 %. The intracranial-lumbar pulsatile pressure gradient was abnormal in 71 % and significantly higher in patients with syringomyelia. The elevated pulsatile ICP significantly correlated with pulsatile pressure gradient; no similar correlation was found for static ICP. We interpret the results as providing evidence of impaired intracranial compliance as an important pathophysiological mechanism in CMI.

MB-16 * BOOST VOLUME EFFECTS ON INCIDENTAL HIPPOCAMPAL DOSE IN PEDIATRIC MEDULLOBLASTOMA

INTRODUCTION: Radiation treatment for medulloblastoma, which involves craniospinal radiation with posterior fossa boost, has been associated with neurocognitive sequelae, possibly due to impaired hippocampal neurogenesis. The posterior fossa has recently been shown to be radiation tolerant with respect to neurocognitive outcomes, though previous studies highlight effects of tumor bed vs. whole posterior fossa boost volumes. The incidental dose to the hippocampus may account for the boost volume effects, and despite traditional boost volume categories, the relative volume of posterior fossa treated may vary significantly. This study analyzes the association of hippocampal dose and boost volume as a continuous variable. METHODS: Bilateral hippocampi and posterior fossa were contoured on T1 axial images for 25 medulloblastoma patients. Minimum dose received by 100% of each hippocampus (D100) and percent volume of posterior fossa receiving 100% of boost dose (V100) were extracted from dose-volume histograms. Analysis of covariance was used to investigate the effect of V100 as a continuous variable while controlling for total craniospinal dose categorically (Low Dose 18-23.4Gy, High Dose 36Gy). RESULTS: Patient characteristics were as follows: age 3-21Y; 5 female, 20 male; 15 average risk (7 18Gy, 8 23.4Gy), 10 high risk (36Gy). Right and left total incidental hippocampal biologically effective doses were both greater for the high dose group by 13.9Gy (p < 0.0004) and 14.0Gy (p < 0.0001) respectively. Right and left D100 significantly varied with V100 by 0.18Gy (p < 0.0218) and 0.15Gy (p < .0322) per percent volume respectively. CONCLUSION: Incidental hippocampal doses are positively associated with boost volumes and may account for neurocognitive decline in medulloblastoma patients. An estimated 11-13% boost volume increase may further impair neurogenesis for a 2Gy threshold. Posterior fossa V100 can be a useful metric to more accurately describe boost volumes, and new hippocampal sparing techniques may allow for greater posterior fossa coverage.

The role of the posterior fossa in developing Chiari I malformation in children with craniosynostosis syndromes

ObjectivePatients with craniosynostosis syndromes are at risk of increased intracranial pressure (ICP) and Chiari I malformation (CMI), caused by a combination of restricted skull growth, venous hypertension, obstructive sleep apnea (OSA), and an overproduction or insufficient resorption of cerebrospinal fluid. This study evaluates whether craniosynostosis patients with CMI have an imbalance between cerebellar volume (CV) and posterior fossa volume (PFV), that is, an overcrowded posterior fossa. MethodsVolumes were measured in 3D-SPGR T1-weighted MR scans of 28 ‘not-operated’ craniosynostosis patients (mean age: 4.0 years; range: 0–14), 85 ‘operated’ craniosynostosis patients (mean age: 8.0 years; range: 1–18), and 34 control subjects (mean age: 5.4 years; range: 0–15). Volumes and CV/PFV ratios were compared between the operated and not-operated craniosynostosis patients, between the individual craniosynostosis syndromes and controls, and between craniosynostosis patients with and without CMI. Data were logarithmically transformed and studied with analysis of covariance (ANCOVA). ResultsThe CV, PFV, and CV/PFV ratios of not-operated craniosynostosis patients and operated craniosynostosis patients were similar to those of the control subjects. None of the individual syndromes was associated with a restricted PFV. However, craniosynostosis patients with CMI had a significantly higher CV/PFV ratio than the control group (0.77 vs. 0.75; p = 0.008). The range of CV/PFV ratios for craniosynostosis patients with CMI, however, did not exceed the normal range. ConclusionVolumes and CV/PFV ratio cannot predict which craniosynostosis patients are more prone to developing CMI than others. Treatment should focus on the skull vault and other contributing factors to increased ICP, including OSA and venous hypertension.

Demographic confounders in volumetric MRI analysis: is the posterior fossa really small in the adult Chiari 1 malformation?

Measurement of posterior fossa volume has been proposed to have diagnostic utility and physiologic significance in the Chiari malformation type 1. This study evaluated the effects of demographics on posterior fossa volume and total intracranial volume in adult control subjects, adult patients with Chiari malformation type 1, and adult patients with idiopathic intracranial hypertension, who may share some imaging features of patients with Chiari malformation type 1. Twenty-eight patients with Chiari malformation type 1, 21 patients with idiopathic intracranial hypertension, and 113 control subjects underwent brain MRI including contrast-enhanced 3D gradient-recalled echo (GRE) T1-weighted imaging. Linear measurements of the posterior fossa and intracranial space were obtained. Manual segmentation of the posterior fossa and intracranial space was performed to yield posterior fossa volume and total intracranial volume. Age, sex, race, and body mass index (weight in kilograms divided by the square of height in meters; BMI) were controlled for when comparing cohorts. Three of the 12 linear measurements significantly predicted total intracranial volume (accounting for 74% of variance), and four predicted posterior fossa volume (54% of variance). Age, race, sex, and BMI each statistically significantly influenced posterior fossa volume and total intracranial volume. No statistically significant differences in posterior fossa volume, total intracranial volume, or ratio of posterior fossa volume to total intracranial volume were seen between the Chiari malformation type 1 group and control group after controlling for demographics. Patients with idiopathic intracranial hypertension were more likely than control subjects to have smaller posterior fossa volumes (odds ratio [OR]=1.81; p=0.01) and larger total intracranial volumes (OR=1.24; p=0.06). Linear measurements of the posterior fossa are not strong predictors of posterior fossa volume. Age, race, sex, and BMI have statistically significant effects on intracranial measurements that must be considered, particularly with respect to posterior fossa volume in Chiari malformation type 1. Even when these demographic variables are appropriately accounted for, other similarly presenting diseases may show small posterior fossa volumes.

Skull Base Morphology in Fibroblast Growth Factor Receptor Type 2-Related Faciocraniosynostosis

Children with faciocraniosynostosis present skull base abnormalities and may develop hydrocephalus or cerebellar tonsils ectopia (CTE). Several pathophysiological hypotheses were formulated in the past decades to explain these associations. However, no study has described in a genetically homogeneous population with confirmed fibroblast growth factor receptor type 2 (FGFR2) mutation eventual correlations between skull base abnormalities and hydrocephalus or CTE. To illustrate these features in children <2 years of age with a genetically confirmed FGFR2-related faciocraniosynostosis. We measured the foramen magnum area (FMA) and its sagittal and transversal components: the right, left, and mean area of the jugular foramen; the posterior fossa volume; and the cerebellar volume on preoperative millimetric computed tomography scan slices in 31 children with an FGFR2 mutation (14 with Crouzon syndrome, 11 with Apert syndrome, and 6 with Pfeiffer syndrome). They were compared with 17 children without synostosis. All children were <24 months of age. We correlated all these measures with the presence of hydrocephalus or CTE. We observed a significantly small FMA in children with Crouzon (P = .03) and in children with Pfeiffer (P = .05) resulting from a reduced sagittal diameter (P = .02 for Crouzon and P = .002 for Pfeiffer). Hydrocephalus was associated with small FMA (P = .02). The jugular foramen area, posterior fossa volume, and cerebellar volume were not associated with hydrocephalus or CTE. Hydrocephalus and CTE were statistically associated (P = .002). Hydrocephalus in FGFR2-related Crouzon and Pfeiffer syndromes is statistically associated with a small FMA. Hydrocephalus is statistically associated with CTE.