'Choroid bar': easy-to-seek marker of normal posterior fossa at 12-14 weeks' gestation.

OBJECTIVES: Our objectives were: (1) to assess the visualization rate of the choroid bar in a consecutive series of 306 first-trimester scans; (2) to verify, in this cohort of fetuses, the normality of the posterior fossa later in pregnancy; and (3) to confirm the non-visualization of the choroid bar in a retrospective series of fetuses with posterior fossa malformations. METHODS: This study included a prospective and a retrospective series. The former comprised 306 fetuses undergoing routine obstetric ultrasound at our unit in both the first and second trimesters over a 6-month period, while the latter comprised 12 cases of posterior fossa malformations. In the prospective study, the presence of the choroid bar, which is defined as a visually continuous, homogeneously hyperechogenic, thick structure bridging the cisterna magna from side to side, was evaluated at the end of the first-trimester nuchal translucency scan. In the retrospective study, previously acquired three-dimensional volume datasets were processed in order to assess whether the choroid bar could be visualized in cases of open spinal dysraphisms and vermian cystic anomalies. In the prospective study, confirmation of a normal posterior fossa was based on the sonographic features of this anatomical region at the time of the second-trimester anomaly scan at 19-21 weeks' gestation, while, in the retrospective study, it was based on autopsy results, when available, or further direct imaging of the defect later in pregnancy. RESULTS: In the prospective study, the choroid bar could be visualized in all 306 fetuses, on transabdominal ultrasound in 287 (93.8%) cases and on transvaginal ultrasound in 19 (6.2%) cases. The choroid bar was displayed with a ventral/dorsal approach in 67 (21.9%) cases, with a lateral approach in 56 (18.3%) cases and with both in 183 (59.8%) cases. All 306 cases were confirmed to have a sonographically normal posterior fossa at 19-21 weeks. On the other hand, in the retrospective study, it was not possible to visualize the choroid bar in any of the fetuses with posterior fossa malformations. CONCLUSIONS: We have described a new sign, the choroid bar, consistent with a normal posterior fossa at 12-14 weeks' gestation. The choroid bar provides the option of screening for major abnormalities of the posterior fossa, since its absence raises suspicion of both open spinal dysraphisms and posterior fossa cystic malformations. At the same time, it is easy to visualize, as it can be seen with all lines of insonation. © 2023 International Society of Ultrasound in Obstetrics and Gynecology.

Prenatal diagnosis of vermian cyst: a new type of posterior fossa cyst.

BACKGROUND: Prenatal diagnoses of cystic malformations of the posterior fossa mainly encompass arachnoid cysts, Blake's pouch cysts and Dandy-Walker syndrome. To date, vermian cysts have not been reported prenatally. OBJECTIVES: To report a series of fetuses with a vermian cyst. MATERIALS AND METHODS: This was a single-center retrospective study conducted from 2012 to 2021. We included all fetuses presenting with a vermian cyst and excluded all other types of posterior fossa cyst. The cyst was visible at prenatal ultrasound (US) and/or magnetic resonance imaging (MRI). Postnatal imaging and/or clinical outcome data were available. RESULTS: Sixteen fetuses fulfilled the inclusion criteria with a strong female predominance (n=13). US and MRI were performed at a mean gestational age of 29+5 and 33+1 weeks, respectively. In all patients, the cyst was in the vermian horizontal fissure. The mean longest dimension was about 10 mm. The vermis and other posterior fossa structures were otherwise normal. At postnatal imaging, 13 children underwent brain imaging including 11 MRIs with complete regression (n=9), stability (n=1) and increase in size (n=3) of the cyst. Psychomotor development was normal in 14 children. One child (with an inner ear malformation) showed a slight delay in walking and language acquisition. Slight walking ataxia was present in another child. CONCLUSION: We report 16 fetuses with posterior fossa cysts located within the vermis at the level of the horizontal fissure, diagnosed at US and/or MRI and carrying an overall excellent neurological prognosis.

A myriad of posterior fossa cysts: A single center experience.

Antenatal posterior fossa cystic lesions are intimidating due to overlapping imaging features of benign and severe malformations. Sonographic assessment of the posterior fossa with good resolution median sagittal and axial views, either primary or secondarily reconstructed, plays the lead role in antenatal evaluation, further enhanced when sequential assessments are added. We present 10 cases of fetal posterior fossa cystic lesions diagnosed in the first and second trimesters that were sequentially analyzed and followed up till delivery or termination. The ultrasound imaging appearance, evolution, and morphometry have been presented in this article.

Biometric assessments of the posterior fossa by fetal MRI: A systematic review.

BACKGROUND: Posterior fossa abnormalities (PFAs) are commonly identified within routine screening and are a frequent indication for fetal magnetic resonance imaging (MRI). Although biometric measurements of the posterior fossa (PF) are established on fetal ultrasound and MRI, qualitative visual assessments are predominantly used to differentiate PFAs. OBJECTIVES: This systematic review aimed to assess 2-dimensional (2D) biometric measurements currently in use for assessing the PF on fetal MRI to delineate different PFAs. METHODS: The protocol was registered (PROSPERO ID CRD42019142162). Eligible studies included T2-weighted MRI PF measurements in fetuses with and without PFAs, including measurements of the PF, or other brain areas relevant to PFAs. RESULTS: 59 studies were included - 6859 fetuses had 62 2D PF and related measurements. These included linear, area and angular measurements, representing measures of PF size, cerebellum/vermis, brainstem, and supratentorial measurements. 11 measurements were used in 10 or more studies and at least 1200 fetuses. These dimensions were used to characterise normal for gestational age, diagnose a range of pathologies, and predict outcome. CONCLUSION: A selection of validated 2D biometric measurements of the PF on fetal MRI may be useful for identification of PFA in different clinical settings. Consistent use of these measures, both clinically and for research, is recommended.

Comprehensive review of the mastoid foramen.

Anatomical variations of the mastoid foramen have been observed to vary in a number of qualities including size, number, and location. These variants have the potential to become problematic during surgical approaches to the posterior cranial fossa and mastoid part of the temporal bone, and should thus be appreciated by the surgeon. Herein, we discuss the mastoid foramen in detail including issues with such foramina that should be known to the neurosurgeon.

Anatomical variations of clivus: a descriptive anatomical study.

PURPOSE: The clivus is a part of the sphenoid bone. It may show some anatomical variations such as fossa navicularis magna (FNM), canalis basilaris medianus (CBM) and craniopharyngeal canal (CPC). These variations have been associated with conditions like meningitis and tumors of skull base. Inadequate information about these structures may pose a risk of inaccurate diagnosis resulting in unwarranted interventional procedures. Hence, the knowledge about the prevalence of these variations is important. Thus, the objective of this study was to determine the prevalence of fossa navicularis magna, craniopharyngeal canal and canalis basilaris medianus and its types utilizing cone-beam computed tomography (CBCT) images. METHODS: Retrospectively, a total of 350 CBCT scans were evaluated for the presence of FNM, CBM, its types and CPC. The analysis was done by two observers independently. Cohen's kappa statistics was used to determine the interobserver agreement. RESULTS: FNM was noted in 19.4% cases, CBM in 9.7% cases, whereas CPC was not identified in any case. Type 5 was the most common type of CBM. There was no significant difference (p > 0.05) between genders and age groups for the prevalence and a highly significant (p < 0.01) substantial agreement between observers for the prevalence of FNM and highly significant (p < 0.01) moderate agreement for the prevalence of CBM and its types was obtained. CONCLUSION: FNM, CBM, CPC, albeit rare anatomical variations of clivus, knowledge of these structures is important for radiologists, anatomists and surgeons to avoid misdiagnosis and provide awareness to the individual of a higher possibility of meningitis or tumors of the skull base.

Quantitative fetal magnetic resonance imaging assessment of cystic posterior fossa malformations.

OBJECTIVE: Normal cognitive development usually requires a structurally intact and complete cerebellar vermis. The aim of this study was to evaluate whether quantification by fetal magnetic resonance imaging (MRI) of vermis- and brainstem-specific imaging markers improves the definition of cystic posterior fossa malformations (cPFM). METHODS: Fetuses diagnosed with cPFM that had an available midsagittal plane on T2-weighted MRI were identified retrospectively and compared with gestational-age (GA) matched brain-normal controls. Fetuses with cPFM were assigned to three groups, according to standard criteria (vermian size and brainstem-vermis (BV) angle): normal vermian area and BV angle < 25° (Group 1); reduced vermian area and/or BV angle of 25-45° (Group 2); and reduced vermian area and BV angle > 45° (Group 3; Dandy-Walker malformation (DWM) group). The number of differentiable vermian lobules and the areas of the vermis, mesencephalon, pons and medulla oblongata were quantified, correlated with and controlled for GA, and compared between the study groups. RESULTS: In total, 142 cases of cPFM were included, with a mean GA of 25.20 ± 5.11 weeks. Cases comprised Blake's pouch cyst (n = 46), arachnoid cyst (n = 12), inferior vermian hypoplasia (n = 5), megacisterna magna (n = 35) and classic DWM (n = 44). In the control group, 148 fetuses were included, with a mean GA of 25.26 ± 4.12 weeks. All quantified areas and the number of differentiable vermian lobules had a significant positive correlation with GA. The number of vermian lobules and the areas of all quantified regions, except for that of the medulla oblongata, differed significantly between the study groups (P ≤ 0.015 for all). The control group had the highest number of differentiable vermian lobules and the DWM group had the lowest (P < 0.01). CONCLUSIONS: Prenatal MRI assessment of vermian lobules is a useful addition to standard neuroradiological and neurosonographic techniques. The quantification of vermian lobules using fetal MRI allows further differentiation of cPFM into subgroups and thereby improves the classification of hindbrain malformations. © 2019 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology.

Arteriovenous malformations of the posterior fossa: a systematic review.

BACKGROUND: Posterior fossa arteriovenous malformations (pfAVMs) can be challenging lesions to manage. AVMs in this location may have distinct features compared with supratentorial AVMs. Our aim was to systematically review the literature on the presenting characteristics of pfAVMs and compare clinical and angiographic outcomes after the various types of treatment employed. METHODS: The review was conducted according to the Cochrane Collaboration guidelines. Electronic databases from 1900 to March 2018 were searched and complemented by hand-searching and cross-referencing. Articles were categorized into (i) AVM studies that included those in the posterior fossa, (ii) those that focused exclusively on pfAVM, and (iii) those that further specified a cerebellar or brainstem location of the AVM. RESULTS: Seventy-seven articles with 4512 pfAVM patients were retained for analyses. Compared with historical supratentorial controls, pfAVMs were reported to more frequently present with rupture, to more commonly have associated arterial aneurysms, and to more frequently lead to poor clinical and angiographic outcomes. The quality of the literature and lack of standardization of outcome reporting precluded performing a meta-analysis on the results of the various different treatment modalities. CONCLUSIONS: Posterior fossa AVMs may have some distinct features compared with supratentorial AVMs. The available reports on pfAVMs are not sufficiently standardized to provide reliable guidance for patient management decisions. This goal will require future studies to be multicentric and to focus on standardized, repeatable clinical and angiographic outcomes.

Hindbrain morphometry and choroid plexus position in differential diagnosis of posterior fossa cystic malformations.

OBJECTIVE: To assess the differential diagnostic significance of a series of quantitative and qualitative variables of the cerebellar vermis in fetuses with posterior fossa cystic malformation, including Dandy-Walker malformation (DWM), vermian hypoplasia (VH) and Blake's pouch cyst (BPC). METHODS: This was a retrospective study of confirmed cases of DWM, VH and BPC, diagnosed at the Fetal Medicine and Surgery Unit of the Federico II University between January 2005 and June 2013 or the Fetal Medicine and Surgery Unit of G. Gaslini Hospital between July 2013 and September 2017. All included cases had good-quality three-dimensional (3D) volume datasets of the posterior fossa, acquired by transvaginal ultrasound through the posterior fontanelle. The midsagittal view of the posterior fossa was the reference view for the study. We assessed brainstem-tentorium angle and brainstem-vermis angle (BVA), as well as craniocaudal (CCVD) and anteroposterior (APVD) vermian diameters and vermian area (VA), which were normalized by biparietal diameter (BPD) to take into account gestational age (CCVD/BPD × 100, APVD/BPD × 100 and VA/BPD × 100, respectively). Finally, the position of the fourth ventricular choroid plexus (4VCP) was defined as normal ('up') or abnormal ('down'), relative to the roof/cyst inlet of the fourth ventricle. RESULTS: We analyzed 67 fetuses with posterior fossa malformations (24 cases of DWM, 13 of VH and 30 of BPC). The mean gestational age at diagnosis was 23.6 weeks. Regardless of gestational age, the BVA differed significantly between the three groups, and the VA/BPD was able to differentiate between VH and BPC. In differentiating between VH and BPC, the greatest areas under the receiver-operating characteristics curve were those for VA/BPD ratio. The 4VCP position was down in all cases of DWM and VH, while it was up in all cases of BPC. CONCLUSIONS: Our data support the concept that VA/BPD ratio and 4VCP position may be used to differentiate between DWM, VH and BPC in the fetus. In our series, the position of the 4VCP had the highest accuracy, but a larger number of VH cases should be evaluated to confirm that an up position of the 4VCP indicates BPC while a down position indicates DWM or VH. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Quantitative diagnostic advantages of three-dimensional ultrasound volume imaging for fetal posterior fossa anomalies: Preliminary establishment of a prediction model.

OBJECTIVES: To quantitatively assess prenatal diagnostic performance of three-dimensional ultrasound (3D-US) for posterior fossa anomalies (PFA) and establish a preliminarily 3D-US prediction model. METHODS: Sixty singleton fetuses suspected of PFA by 2D-US presented their detailed 3D-US evaluation. The surface area of vermis (SAV), brainstem-vermis, and brainstem-tentorium angles were measured by 3D-US. The good prognosis was defined as normal neurodevelopmental outcome. MRI and autopsy were the diagnostic reference standard. RESULTS: There was a significant difference between 2D-US (60.0%, 36/60) and 3D-US (94.8%, 55/58) for the diagnostic accuracy (P < .01). Prenatal 3D-US prediction model was established with observed/expected SAV as the main predictor (area under the curve [AUC]: 0.901; 95% CI, 0.810-0.992, P < .001). When it was more than 107.5%, the prognosis seemed to be good (sensitivity: 96.4%, specificity: 26.7%), which led to consideration of mega cisterna magna, Blake pouch cyst, or small arachnoid cyst. The prognosis appeared to be poor when it was less than 73% (sensitivity: 71.4%, specificity: 100%), and the diagnosis tended to be a Dandy-Walker malformation, vermian hypoplasia, and cerebellar hypoplasia. Brainstem-vermis and brainstem-tentorium angles were the secondary indicators (AUC: 0.689 vs 0.761; 95% CI, 0.541-0.836 vs 0.624-0.897, P = .014 vs.001). CONCLUSIONS: It seems that the exact types of PFA can be effectively diagnosed by quantitative indicators of 3D-US.

The reconstruction of skull base defects in infants using pedicled nasoseptal flap-a review of four cases.

INTRODUCTION: Benign lesions of the skull base are common in the paediatric population, and are usually congenital in aetiology. Majority of these lesions are treated transcranially exposing the patients to a number of risks. Although endoscopic endonasal surgery (EES) helps avoid many of these potential morbidities, CSF leak with its attendant complication remains a big concern. This study reports the use of the Hadad flap in the reconstruction of skull base defects in infants to prevent this problem. The study was conducted on four infants with a mean age of 7 months, who underwent repairs for CSF leaks associated with congenital lesions like meningocele or meningoencephalocele, using the Hadad flap. Of the four patients, three cases were revision cases and one was primary where the patients presented with complaints such as nasal obstruction and watery nasal discharge. Post surgery, the infants were monitored for a mean period of 23 months and no major complications or recurrent CSF drainage were observed. Minor complications that were observed include vestibulitis and crusting in the nose. The utility of the Hadad flap in the reconstruction of skull base defects in the paediatric age group has been controversial while its utility in infants has not been studied in literature so far. We report here in our series four infant patients in whom we believe that the nasal septum and the skull base will develop proportionally to each other, hence lowering the chances of a short flap and eliminating the occurrence of future complications. CONCLUSION: The nasoseptal flap is an effective and safe technique for reconstructing skull base defects in infancy. It can be concluded that this technique does not have any potential effect on septal or craniofacial growth as the flap is harvested only on one side with normal mucosal cover on the other side. There is no posterior septectomy or any form of bony or cartilaginous resection that is performed, hence avoiding any effects on bony growth. No studies have been published in literature so far and to the best of our knowledge, this is the first report describing the efficacy of the nasoseptal flap in infants.

Carotid canal and optic canal at sphenoid sinus.

In the present study, we investigated the relationship between sphenoid sinus, carotid canal, and optic canal on paranasal sinus computed tomography (PNSCT). This study was performed retrospectively. PNSCT images of 300 adult subjects (159 male, 141 female). Sphenoid sinus (pneumatisation, dominancy, septation, inter-sinus septa deviation), anterior clinoid process pneumatisation, Onodi cell, carotid and optic canals (width, dehiscence, classification) were measured. In males, type 3 pneumatised sphenoid sinus (in both sides) and in females type 2 pneumatised sphenoid sinus (right side) and type 3 pneumatised sphenoid sinus (left side) were detected more. Anterior clinoid pneumatisation was present 47.2% in males and 39.7% in females. In male group, more septation (i.e. 22.6%, ≥ 3 septa) in sphenoid sinus were detected. Onodi cell was present 26.6 and 19.1% in males and females, respectively. Carotid canal protrudation to the sphenoid sinus wall was present 23.9-32.1% in males and 35.5-36.2% in females. Dehiscence in carotid canal was detected more in females (34%) compared to males (22%). Optic canal protrudation was 33.3 and 30.5% in males and females. Type 4 optic canal was detected more in both gender. Optic canal dehiscence was detected 11.3 and 9.9% in males and females. Carotid and optic canal diameters were higher in males. In pneumatised sphenoid sinuses and in females, type 3 carotid canal (Protrudation to SS wall) (bilaterally) and type 1 optic canal type (No indentation) (ipsilateral side) were detected more. In elderly patients, carotid and optic canal width increased. When carotid canal protrudation was detected, there was no indentation in optic canals In pneumatised SS, carotid canal protrudation was observed with a greater risk in surgery. However, type 1 (non indentation) optic canal was present in highly pneumatised SS with lower risk for the surgery. In women, the risk of carotid canal protruding (about 1/3) is greater than that of males, and carotid canal dehiscence rates are also higher in females. Therefore, physicians should be very careful during the preparatory stages of the sphenoid sinus surgery. Otherwise, it may not be possible to prevent lethal carotid artery bleeds.

Stability-Sparing Endoscopic Endonasal Odontoidectomy in a Malformative Craniovertebral Junction: Case Report and Biomechanical Considerations.

BACKGROUND: The craniovertebral junction (CVJ) is often involved in a wide range of congenital, developmental and acquired pathologies that can create bony and ligamentous instability or cause direct compression on the medulla and cervical spine cord, resulting in significant impairment. Atlas assimilation is the most common malformation in the CVJ and can be frequently associated with basilar invagination (BI) and Chiari malformation (CM) type I. Posterior atlas assimilation more frequently leads to BI type II with a mass effect on neural structures but usually no signs of biomechanical instability. Operative approaches to the CVJ have undergone a remarkable evolution and can be divided into ventral, lateral and dorsal ones. In this kind of surgery, it is vital to detect and eventually treat any CVJ instability. CASE DESCRIPTION: We present a case of CVJ malformation comprising assimilation of the posterior arch of the atlas, BI type II and CM, treated by endoscopic endonasal odontoidectomy and partial clivus removal to spare CVJ stability. CONCLUSION: Neurological and biomechanical analysis of all CVJ malformations permits stratification and selection of those cases that can be managed by simple, direct, minimally invasive decompression with no need for surgical fusion.

Utilization of neurosonography for evaluation of the corpus callosum malformations in the era of fetal magnetic resonance imaging.

AIM: We evaluated the ability of fetal neurosonography and magnetic resonance imaging (MRI) to asses callosal anomalies (CA) and associated cranial malformations. We also aimed to determine the long-term prognosis of the cases. METHODS: Thirty-six cases of CA diagnosed combined with neurosonography and MRI between January 2012 and October 2017 were retrospectively reviewed. RESULTS: Seventeen of 36 fetuses were diagnosed complete agenesis of corpus callosum (CACC) (47.2%), 9 had partial agenesis of corpus callosum (PACC) (25%) and 10 was dysgenesis of the corpus callosum (DCC) (27.2%) at ultrasonography (US) examination. Fetal MRI reported 16 of cases as CACC (44.4%), 11 PACC (30.5%) and nine (25%) DCC. The overall consistency between neurosonography and MRI in the definition of CA were 91% of cases. Sulcation anomalies were present in 9 cases in the US (25%) and 11 cases in MRI (30.4%). Seven of cases showed posterior fossa abnormalities in the US (19.4%) and eight cases in MRI (22.1%). Neonatal MRI added new findings to fetal MRI and neurosonography including grade-1 intraventricular hemorrhage and periventricular leukomalacia in two cases (12.5%). Eighteen cases were terminated (50%), 17 cases were followed up and mean follow up interval was 39 ± 5.1 months. The neurologic outcome was abnormal in seven (41.7%) patients. Presence of associated brain anomalies worsened the prognosis. CONCLUSION: Fetal neurosonography has a comparable performance with MRI in the diagnosis of CA and associated anomalies. It should be used in collaboration with MRI to achieve accurate diagnosis which is crucial for counseling.

Canalis basalis medianus with cerebrospinal fluid leak: rare presentation and literature review.

Canalis Basalis Medianus is a rare congenital normal anatomically variant of the clivus .We report a very rare case of cerebrospinal fluid leak from a canalis basilaris medianus in a 22-year-old male, who presented to our hospital with frontal headache and running nose two weeks after an elective septoplasty.

Non-visualization of choroid plexus of fourth ventricle as first-trimester predictor of posterior fossa anomalies and chromosomal defects.

OBJECTIVE: To assess non-visualization of the choroid plexus of the fourth ventricle (CP-4V) as a simple, qualitative and reproducible first-trimester ultrasound feature of the posterior fossa for the prediction of central nervous system (CNS) anomalies and chromosomal defects. METHODS: First-trimester three-dimensional ultrasound datasets of the fetal brain were obtained prospectively from 65 consecutive normal singletons and retrospectively from 27 fetuses identified as having an abnormal posterior fossa on first-trimester ultrasound examination, and randomly combined to form the final study group. The stored ultrasound volumes were analyzed offline by two accredited sonologists, who were not aware of the final diagnoses. The CP-4V was assessed by multiplanar navigation and classified as visible or non-visible in its normal position depending on whether or not the echogenic structure that separates the fourth ventricle from the cisterna magna was identified in both midsagittal and axial planes. Correlation with subsequent second-trimester ultrasound, fetal magnetic resonance imaging, or postmortem or postnatal findings was performed to determine the predictive value of the first-trimester findings. RESULTS: Among the 92 ultrasound datasets analyzed, 73 (79%) were acquired transabdominally and 19 (21%) transvaginally. The CP-4V was classified as visible in 64 cases and non-visible in 28 cases, with agreement between the two observers in both sagittal and axial planes in all but one case. Twelve of the 28 (43%) fetuses with non-visible CP-4V were subsequently diagnosed as having a CNS malformation (open spina bifida (n = 6), Dandy-Walker malformation (n = 2), Blake's pouch cyst (n = 2), cephalocele (n = 1) and megacisterna magna (n = 1)). In addition, 20 of these 28 (71%) fetuses had aneuploidy (trisomy 18 (n = 10), triploidy (n = 5), trisomy 13 (n = 3), Turner syndrome (n = 1) or trisomy 21 (n = 1)). There was only one false-positive case, in which the CP-4V was classified as absent in a normal fetus. CONCLUSIONS: Non-visualization of the CP-4V in the first trimester appears to be a strong marker of posterior fossa anomalies and chromosomal defects. Qualitative evaluation of this anatomic structure is simple, feasible and reproducible, and its routine assessment during the first-trimester scan may facilitate the early detection of CNS anomalies and associated fetal aneuploidy. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Quantitative evaluation of facial hypoplasia and airway obstruction in infants with syndromic craniosynostosis: relationship with skull base and splanchnocranium sutural pattern.

PURPOSE: Craniosynostostic syndromes are due to multisuture synostoses and affect the entire craniofacial skeleton. This study analyzed the facial complex and airways to quantify the relationship between insufficient facial growth, airways obstruction, and the sutural pattern of the splanchnocranium and cranial fossae. METHODS: Preoperative high-resolution CT images in 19 infants with syndromic craniosynostosis were quantitatively analyzed. Because all children showed involvement of minor sutures/synchondroses coursing in the posterior cranial fossa, they were divided into three groups according to the synostotic involvement of "minor" sutures/synchondroses coursing in anterior (ACF) and middle (MCF) cranial fossae: group 1 (ACF), group 2 (MCF), and group 3 (ACF-MCF). Analysis of the facial complex and airway was performed. Each group was compared with age-matched healthy subjects. RESULTS: Premature closure of skull base synchondroses of ACF and MCF was found only in groups MCF and ACF-MCF. Group MCF showed synostosis in the posterior branch of the coronal ring and reduced anterior hemifossae lengths while group ACF-MCF showed synostosis in the anterior branch of the coronal ring and reduced middle hemifossae lengths. No group showed reduced maxillary or mandibular volumes but group MCF showed synostosis of the zygomaticomaxillary sutures and maxillary retrusion. All groups showed reduced airway volume but group 2 had a higher degree of airway hypoplasia. CONCLUSION: The skull base synostotic process drives the changes in facial complex growth and airway obstruction. Premature closure of synchondroses/sutures in the posterior branch of the coronal ring causes insufficient facial growth, maxillary retrusion, and more severe airway reduction.

A morphometric assessment of type I Chiari malformation above the McRae line: A retrospective case-control study in 302 adult female subjects.

PURPOSE: Type I Chiari malformation (CMI) is a radiologically-defined structural dysmorphism of the hindbrain and posterior cranial fossa (PCF). Traditional radiographic identification of CMI relies on the measurement of the cerebellar tonsils in relation to the foramen magnum with or without associated abnormalities of the neuraxis. The primary goal of this retrospective study was to comprehensively assess morphometric parameters above the McRea line in a group of female CMI patients and normal controls. MATERIAL AND METHODS: Twenty-nine morphological measurements were taken on 302 mid-sagittal MR images of adult female CMI patients (n=162) and healthy controls (n=140). All MR images were voluntarily provided by CMI subjects through an online database and control participant images were obtained through the Human Connectome Project and a local hospital system. RESULTS: Analyses were performed on the full dataset of adult female MR images and a restricted dataset of 229 participants that were equated for age, race, and body mass index. Eighteen group differences were identified in the PCF area that we grouped into three clusters; PCF structures heights, clivus angulation, and odontoid process irregularity. Fourteen group differences persisted after equating our CMI and control groups on demographic characteristics. CONCLUSION: PCF structures reliably differ in adult female CMI patients relative to healthy controls. These differences reflect structural abnormalities in the osseous and soft tissue structures of the clivus, odontoid process, and cerebellum. Clinical and pathophysiological implications are discussed.

Post Mortem Diagnosis of Blake's Pouch Cyst: A Presentation of Distended Cyst at Necropsy.

BACKGROUND: Blake's pouch cyst forms from non-permeabilization of Blake's pouch. It is difficult to visualize at necropsy as the cyst ruptures easily into the 4th ventricle during dissection. CASE REPORT: Based upon prenatal imaging, delicate dissection allowed post-mortem confirmation of the Blake's pouch cyst. CONCLUSIONS: This highlights the importance of utilizing premortem imaging to help guide the postmortem dissection and documentation of a posterior fossa cyst.

Anatomical subgroup analysis of the MERIDIAN cohort: posterior fossa abnormalities.

OBJECTIVE: To assess the diagnostic and clinical contribution of fetal magnetic resonance imaging (MRI) in fetuses of the MERIDIAN cohort diagnosed with abnormalities of the posterior fossa as the only intracranial abnormality recognized on antenatal ultrasound. METHODS: This was a subgroup analysis of the MERIDIAN study of fetuses with abnormalities of the posterior fossa (with or without ventriculomegaly) diagnosed on antenatal ultrasound in women who had MRI within 2 weeks of ultrasound and for whom outcome reference data were available. The diagnostic accuracy of ultrasound and MRI is reported, as well as indicators of diagnostic confidence and effects on prognosis and clinical management. Appropriate diagnostic confidence was assessed by the score-based weighted average method, which combines diagnostic accuracy with diagnostic confidence data. RESULTS: Abnormalities confined to the posterior fossa according to ultrasound were found in 81 fetuses (67 with parenchymal and 14 with cerebrospinal fluid-containing lesions). The overall diagnostic accuracy for detecting an isolated posterior fossa abnormality was 65.4% for ultrasound and 87.7% for MRI (difference, 22.3% (95% CI, 14.0-30.5%); P < 0.0001). There was an improvement in 'appropriate' diagnostic confidence, as assessed by the score-based weighted average method (P < 0.0001), and a three-fold reduction in 'high confidence but incorrect diagnosis' was achieved using MRI. Prognostic information given to the women changed after MRI in 44% of cases, and the overall effect of MRI on clinical management was considered to be 'significant', 'major' or 'decisive' in 35% of cases. CONCLUSIONS: Our data suggest that any woman whose fetus has a posterior fossa abnormality as the only intracranial finding on ultrasound should have MRI for further evaluation. This is on the basis of improved diagnostic accuracy and confidence, which impacts substantially on the prognostic information given to women as well as their clinical management. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.