Intracranial Anomalies Research Articles

Occipital encephalocele: a retrospective analysis and assessment of post-surgical neurodevelopmental outcome.

Encephalocele represent a group of disorders which is characterised by extracranial herniation of the leptomeninges, brain, and CSF through a structural defect in the cranium. They are usually associated with other intracranial anomalies which may impact the neurological development. This study aimed to assess the predictors of neurological development of patients undergone surgical excision of occipital encephalocele. All patients with occipital encephaloceles operated over the last decade (2012-2022). The sac size, presence of hydrocephalous, and associated anomalies were noted. The biopsy of these patients were reviewed and categorised as those which contains mature neural tissue and those without. The neurological outcomes were assessed by social, language, cognitive, and motor milestone and has been stratified into no delay, mild (1 of 4), moderate (2 or 3 of 4), and severe development delay (4 of 4). Total of 35 patients were included with median age of 10months (IQR = 5-20months). Fifteen (42.9%) patients had sac size of ≥ 5cm, and 23 (65.7%) patients had mature neural tissues on biopsy. The median follow-up period was 6.4years (IQR = 4.38-10.65) years. Seventeen (49.6%) patients had moderate to severe developmental delay. The sac size of ≥ 5cm (AOR = 33.5; 95%CI = 3.35-334.8) (p = 0.003) and presence of mature neural content in the sac (AOR = 13.32; 95%CI = 1.1-160.36) (p = 0.041) were associated with significant neurodevelopmental delay. The presence of a large sac of ≥ 5cm and the presence of mature neural tissues on histopathological specimen of patients with encephalocele point towards the possibility of poor neurological development.

Ectopic Posterior Pituitary and Its Associations with Extrapituitary Intracranial Anomalies.

Posterior pituitary ectopia is a very rare entity in the development of the pituitary gland. Several factors and multiple genes are associated with this entity causing both pituitary and extrapituitary abnormalities. Pituitary abnormalities can be various endocrine problems and extrapituitary abnormalities can be optic nerves and cerebellar hypoplasia, heterotopia, and abnormal vessels. This pictorial review represents the imaging manifestations of extrapituitary intracranial anomalies that can be associated with posterior pituitary ectopia.

Associations and outcomes of prenatally detected rhombencephalosynapsis.

To describe the association between prenatal imaging and neurodevelopmental outcomes of fetuses with rhombencephalosynapsis (RES). Thirty-four pregnancies complicated by RES were identified from our institutional databases based on US and/or MRI findings. Genetic testing results were gathered. In cases of termination of pregnancy, we studied the association between prenatal imaging and neuropathologic findings. For those who opted for expectant management, comprehensive developmental assessments and postnatal MRI imaging were evaluated. Over one third of fetuses in our cohort had complete RES. Common intracranial anomalies identified were mesencephalosynapsis, aqueduct stenosis and diencephalosynapsis. The degree of RES was not associated with the frequency of additional central nervous system anomalies. MRI had a good correlation with neuropathologic findings with regard to the degree of RES, aqueduct stenosis and mesencephalosynapsis. Postmortem autopsy showed that one third of our cases had VACTERL-H and almost all of those had complete RES. All liveborn neonates(n=6) had aqueduct stenosis requiring ventriculoperitoneal shunting within days of delivery (median 5days). While a large proportion of prenatally suspected complete RES were found to have partial RES on postnatal imaging, prenatal diagnosis of aqueduct stenosis remained unchanged. All children that were at least 2years old (n=3) had global developmental delay. Prenatal assessment of the RES severity is challenging and may be unreliable. Nevertheless, postnatal prognosis is poor for both complete and partial RES. Associated aqueductal stenosis, can be reliably assessed prenatally and this may contribute to worse postnatal prognosis than the degree of RES.

Magnetic resonance imaging of intracranial anomalies in pregnancies complicated by twin anemia-polycythemia sequence

PurposeTo describe fetal brain Magnetic Resonance Imaging (MRI) findings in a large series of monochorionic (MC) pregnancies complicated by Twin Anemia-Polycythemia Sequence (TAPS) prenatally diagnosed, so to characterize the potential intracranial complications associated with this condition, their frequency and potential treatment options.MethodsThis is a retrospective study of MC twin pregnancies complicated by TAPS and undergone fetal MRI in a single institution from 2006 to 2023.MRI control was performed and post-natal ultrasound (US) or MRI were available.Results1250 MC pregnancies were evaluated in our institution. 50 pregnancies (4%) were diagnosed with TAPS, 29 underwent a fetal brain MRI.13/29 pregnancies (44.8%) demonstrated brain findings at MRI in at least a twin. Neuroradiological findings were detected in 14/57 twins (24.6%).We detected four main categories of findings: hemorrhagic lesions, T2-weighted white-matter hyperintensities (WMH), brain edema-swelling and venous congestion. Nineteen findings were present in the anemic and three in the polycythemic twins, with a statistically significant ratio between the two groups (p-value = 0.01).Intrauterine MRI follow-up demonstrated the sequalae of hemorrhagic lesions. A complete regression of brain swelling, veins prominence and T2-WMHs was demonstrated after treatment. Postnatal imaging confirmed prenatal features.ConclusionsOur work demonstrates that TAPS-related MRI anomalies consisted in edematous/hemorrhagic lesions that occur mostly in anemic rather than in polycythemic twins. Fetoscopic laser surgery could have a potential decongestant role.Therefore, prenatal MRI may help in counselling and management in TAPS pregnancies, especially for the planning of therapy and the monitoring of its efficacy.

MRI Evaluation of Vasculopathy and Additional Intracranial Manifestations in Morning Glory Disc Anomaly

Morning glory disc anomaly (MGDA) is rare, but its fundoscopic findings are well documented in the ophthalmologic literature. It is sporadic, without sexual predisposition, and usually unilateral. Furthermore, it is associated with numerous central nervous system anomalies, including vasculopathy. This case series reports four pediatric patients over a 3-year period in whom ophthalmologic evaluation identified MGDA. Magnetic resonance imaging and magnetic resonance angiography were subsequently performed to assess for associated intracranial vascular anomalies. This report is of significance because it demonstrates the spectrum of intracranial vasculopathy in this rare entity.

Management of sporadic intracranial vascular malformations in pregnancy: a retrospective analysis

BackgroundSporadic intracranial vascular malformations can pose significant risk to parturients, and additional reports of management may inform patient care. Here we describe the peripartum management of parturients with intracranial vascular malformations. MethodsAfter Institutional Review Board approval, we performed a retrospective analysis of parturients with a known sporadic intracranial vascular malformation including cavernous malformation, developmental venous anomaly, or arteriovenous malformation who delivered at our institution between 2007 and 2020. ResultsWe identified 10 parturients (five cavernous malformations, three developmental venous anomalies, and two arteriovenous malformations) with 16 deliveries. Among all deliveries, 13 (81.3%) were cesarean deliveries without trial of labor; 11 of these (84.6%) received a single-shot spinal and two (15.4%) received an epidural for surgical anesthesia. Two deliveries (12.5%) began with attempted trial of labor but ultimately required cesarean delivery for failure to progress; one of these cases received epidural anesthesia and the other received combined spinal-epidural anesthesia. One delivery was via spontaneous vaginal delivery with epidural analgesia. Overall, our study’s cesarean delivery rate was 93.8% and spontaneous vaginal delivery rate was 6.2%.Three of 16 pregnancies were complicated by seizure, obstructive hydrocephalus, or intracranial hemorrhage. There were no intensive care unit admissions or maternal deaths. ConclusionsIn our case series of 16 deliveries, there were no complications directly resulting from neuraxial procedures. It remains unclear whether intracranial developmental venous anomalies or unruptured arteriovenous malformations impart increased risk during pregnancy. Antepartum planning with a multidisciplinary team approach enables risk stratification and optimal management.

Evolution of cerebrovascular imaging and associated clinical findings in children with Alagille syndrome.

Alagille syndrome (ALGS) is a multisystem autosomal dominant disorder with highly variable expression. Intracranial arterial and venous anomalies have a reported prevalence of 30-40% and can increase the risk of stroke by 16%. Few reports document the frequency and evolution of cerebrovascular abnormalities (CVAs) in children with ALGS. We aimed to define the spectrum, frequency, and evolution of CVAs in a series of children with ALGS using magnetic resonance angiography (MRA). We conducted a single-center, retrospective study in a large tertiary pediatric hospital. CVAs were grouped into 4 categories: 1) Stenosis or narrowing; 2) Aneurysms and ectasias; 3) Tortuosity; and 4) Vascular anomalies and anatomical variants. Thirty-two children met the inclusion criteria. The median age at initial diagnosis was 6 (3.8-10.3) years. Thirteen (40%) had follow-up MRI at a mean of 55 (31.5-66) months. Eighteen (56%) had CVAs; the most frequent fell into group 1 (n = 12, 37.5%). CVAs were stable over time, except for one patient with Moyamoya arteriopathy (MMA). One patient developed a transient ischemic attack secondary to an embolic event. Three (9.3%) had microhemorrhages at the initial diagnosis secondary to Tetralogy of Fallot. Another patient had recurrent subdural hematomas of unknown cause. CVAs were stable except in the presence of MMA. Vascular strokes, which are reported in older patients with ALGS, were not a common feature in children under 16 years of age, either at presentation or over the 31.5-66 month follow-up period.

Evaluation of Cerebral and Spinal Abnormalities on Magnetic Resonance Imaging in Infants with Myelomeningocele: A Retrospective Observational Study

Introduction: Spinal dysraphism includes a diverse collection of congenital spinal abnormalities that occur due to flawed closure of the neural tube and can be classified as open or closed types. The most common form of open spinal dysraphism is Myelomeningocele (MMC), which is characterised by a bony spinal defect and a sac in the back region containing dysplastic neural tissue and cerebrospinal fluid. MMC is associated with various intracranial and spinal anomalies that can lead to neurological, intellectual, and cognitive impairments. Imaging is necessary for the diagnosis of these anomalies and for surgical management planning and follow-up. Aim: To evaluate the associated intracranial and spinal anomalies in infants with MMC who presented with low back swelling since birth. Materials and Methods: The retrospective observational study was conducted in the Department of Radiology at Gajara Raja Medical College, Gwalior, Madhya Pradesh, India, from January 2020 to December 2022. A total of 117 subjects under one year of age, clinically diagnosed with MMC, were included in the study. Brain Magnetic Resonance Imaging (MRI) images were available for 84 patients, and spine MRI images were available for 113 patients. The MRI images were evaluated for bony and spinal anomalies. Data analysis was performed using IBM’s Statistical Package for Social Sciences (SPSS) version 20.0. Results: The mean age of the study participants was 1.7±2.9 months, with 70 males and 47 females (M:F=1.5:1). The most common location of spina bifida was lumbosacral 36 (31.9%), followed by lumbar 31 (27.4%), dorsolumbar 30 (26.5%), and sacral 16 (14.2%). A low-level spinal defect was observed in 39 cases (34.5%), and a high-level spinal defect was observed in 74 cases (65.5%). The spinal anomalies detected on MRI included syringomyelia 80 (70.8%), low-lying cord 59 (52.2%), bony abnormalities 26 (23.01%), diastematomyelia 16 (14.2%), and fatty filum terminale 3 (2.7%). Among the intracranial anomalies, the most common was dilated lateral ventricles 62 (73.8%). The most common infratentorial anomaly was Chiari II malformation 58 (69.1%). Corpus Callosum (CC) abnormalities were found in 40 cases (47.6%), with complete agenesis seen in three cases (3.6%), cerebellar hypoplasia in 20 cases (23.8%), absent septum in 15 cases (17.9%), and colpocephaly in 10 cases (11.9%). Conclusion: Spinal dysraphism, such as MMC, and cerebral anomalies often co-exist and can have an impact on neurological, intellectual, and cognitive functions. Chiari II malformation, corpus callosal anomalies, ventriculomegaly, and syringomyelia are frequently associated with MMC. Therefore, imaging evaluation of both the spine and brain is necessary for surgical treatment planning and long-term follow-up in MMC.

Prenatal evaluation of genetic variants in fetuses with small head circumference: A single-center retrospective study

Objectives: To comprehensively evaluate the contributions of numerical chromosomal abnormality, copy number variant (CNV), and sequence variant (SV) to fetuses with small head circumference in a Chinese cohort using chromosome microarray analysis and whole exome sequencing. Methods: A total of 157 fetuses with small heads defined as head circumference < − 2 standard deviation (SD) were recruited between October 2014 and March 2023. We used the ultrasonic measurement parameter Z-score to define small head as possible microcephaly (3 < Z ≤ -2), microcephaly (-5 < Z ≤ -3), or pathologic microcephaly (Z ≤ -5). Ultrasound findings and genetic results were analyzed. Results: The overall diagnostic yield of chromosomal abnormalities by microarray analysis was 13 %. Whole exome sequencing revealed eight novel variants and two interesting candidate genes and provided a 25.4 % incremental yield compared with microarray analysis. Of the detected SVs, 56 % were de novo and the most common inheritance pattern was autosomal dominant inheritance presented in 11/16 fetuses. Compared with isolated small heads, non-isolated small heads had a significantly higher detection rate of chromosomal abnormalities (16 % vs. 3.0 %, P = 0.049) but not SVs (24 % vs. 5.5 %, P = 0.126). Subgroup analysis showed that intracranial anomalies had a similar high detection rate of SVs in fetuses with all small heads subgroups while no chromosomal abnormalities and causative SVs were found in fetuses with isolated possible microcephaly. Conclusions: Ultrasound findings of small fetal head circumference < 3 SD below the mean, especially those with intracranial structural abnormalities, indicate the need for genetic counseling. Genetic variants, mainly copy number variants and SV, may be responsible for the substantial proportion of small fetal head circumference, while most are de novo. Whole exome sequencing and microarray analysis are effective diagnostic approaches for this population.

Anomalies of the Corpus Callosum in Prenatal Ultrasound: A Narrative Review for Diagnosis and Further Counseling.

The corpus callosum is the major interhemispheric tract that plays an important role in neurological function. Understanding the etiology and embryology development helps the ultrasound diagnosis for disorders of the corpus callosum and further counseling. The nonvisualization of cavum septum pellucidum or dysmorphic cavum septum pellucidum in axial view are indirect signs for beginners to diagnose complete agenesis of corpus callosum (cACC) and partial agenesis of the corpus callosum (pACC). Further coronal view, sagittal view, and fetal magnetic resonance imaging are also important for evaluation. Genetic testing plays an essential tool in anomalies of corpus callosum by revealing the underlying genetic pathophysiology, such as chromosomal anomalies and numerous monogenetic disorders in 30%-45% of ACC. Diagnosis and prediction of prognosis for hypoplasia or hyperplasia of the corpus callosum are more difficult compared to cACC and pACC because of the limited reports in the literature. However, the complex types often had poorer prognostic outcomes compared to the isolated types. Hence, it is important to evaluate and follow fetal conditions thoroughly to rule out intracranial or extracranial anomalies in other systems.

Chiari Malformation (CM) type III surgical management in infant: a case report

Background: Chiari Malformations (CM) is a collection of hindbrain abnormalities ranging from simple to complete agenesis of the cerebellum. Type III CM (CM III) is the rarest CM, with herniation of the cerebellum and brainstem into a posterior encephalocele and other intracranial anomalies. Reduction of the viable herniated brain, along with providing intracranial space and inadequate closure, often presents as the main problem. This case study aims to evaluate the Chiari malformation type III surgical management in infants. Case Presentation: A 16-day-old male infant with an intact congenital mass in the occipital, with no neurological deficit. Radiological examination shows a defect in the occipital skull consisting of cystic non-contrast enhancing lesion and parenchymal tissue herniated through the defect. The cele excision and defect closure were performed, and viable neural tissue was found inside the sac. Blossom flower-like trim expanded the fossa posterior space and put the viable neural tissue inside the intracranial room. The defect was then closed with a dura, periosteal patch, and tension-free two-layered scalp closure. Post-operative follow-up presented a good outcome, and 1-year follow-up showed good recovery, with developmental delay, following developmental rehabilitation. Conclusion: CM III is rare and has poor outcomes because neurological deficits and respiratory problems usually follow it. Operative management patient with CM III was presented with occipital encephalocele with no post-operative complication. Occipital bone reconstruction using a blossomed-flower-like technique could be considered to create adequate room for viable neural tissue.

Can Craniosynostosis be Diagnosed on Physical Examination? A Retrospective Review.

Craniosynostosis is a developmental craniofacial defect in which one or more sutures of the skull fuse together prematurely. Uncorrected craniosynostosis may have serious complications including elevated intracranial pressure, developmental delay, and blindness. Proper diagnosis of craniosynostosis requires a physical examination of the head with assessment for symmetry and palpation of sutures for prominence. Often, if craniosynostosis is suspected, computed tomography (CT) imaging will be obtained. Recent literature has posited that this is unnecessary. This study aims to address whether physical examination alone is sufficient for the diagnosis and treatment planning of single suture craniosynostosis. Between 2015 and 2022, the Divisions of Pediatric Neurosurgery and Pediatric Plastic Surgery at UTHealth Houston evaluated 140 children under 36 months of age with suspected craniosynostosis by physical examination and subsequently ordered CT imaging for preoperative planning. Twenty-three patients received a clinical diagnosis of multi-sutural or syndromic craniosynostosis that was confirmed by CT. One hundred seventeen patients were diagnosed with single suture craniosynostosis on clinical examination and follow-up CT confirmed suture fusion in 109 (93.2%) patients and identified intracranial anomalies in 7 (6.0%) patients. These patients underwent surgical correction. Eight (6.8%) patients showed no evidence of craniosynostosis on CT imaging. Treatment for patients without fused sutures included molding helmets and observation alone. This evidence suggests that physical examination alone may be inadequate to accurately diagnose single suture synostosis, and surgery without preoperative CT evaluation could lead to unindicated procedures.

Neurointerventional Treatment of Vein of Galen Malformation (VGM): A Structured Review with a Proposal for the Comparison of Outcome Quality

Background: Vein of Galen malformation (VGM) is a congenital intracranial vascular anomaly consisting of arteriovenous fistulas and/or malformations between various arterial feeders and the median prosencephalic vein of Markowski (MPV). Despite its rare occurrence, VGM is of particular clinical relevance, as the excessive intracranial shunt volume leads to high mortality without appropriate treatment. Methods: The objective of this article is to review the published data on neurointerventional treatment and compare outcome quality in the included studies. Eight studies were included and synthesized. One study was multicentric and the rest were retrospective monocentric (level 4 evidence studies according to the Oxford Centre for Evidence-based Medicine). Results: The total number of included patients was 480 and patient age ranged from 1 day to 18 years. Mild or severe heart failure, hydrocephalus, and other reasons led to the indication for neurointerventional treatment, which was performed in all studies in the form of embolization. Under consideration of the introduced semiquantitative multidimensional scoring system, the highest total score, i.e., the best outcome quality, was found for the study “Houston” 2002–2018 (19 points) and the study “Duisburg” 2001–2010 (19 points). Conclusions: Neurointerventional treatment represents the essential pillar in the interdisciplinary management of patients with VGM, although standardization is lacking—based on the results of the structured review. As complementary treatments, pediatric critical care is mandatory and includes medical hemodynamic stabilization.

Volume Fractionation Stereotactic Radiosurgery for Large Volume Intracranial Arteriovenous Malformations.

Arteriovenous malformations (AVMs) are common intracranial vascular anomalies. Common treatment modalities used to manage AVMs are surgical excision, embolization, and stereotactic radiosurgery (SRS). Large AVMs are defined as AVMs larger than 10 cm3 and pose a therapeutic challenge with high rates of treatment-related morbidity and mortality. Single-stage SRS is a good option for small AVMs but carries high risks of radiation-induced complications in large AVMs. Volume-staged SRS (VS-SRS) is a newer strategy used in large AVMs that allows one to deliver an optimal radiation dose to the AVMs while reducing the risk of radiation damage to the normal brain. It involves the division of AVM into multiple small sectors which are irradiated at different time intervals with high radiation doses. Good obliteration rates with less risk of radiation-induced complications have been described in the literature with VS-SRS.

Imaging the Cerebral Veins in Pediatric Patients: Beyond Dural Venous Sinus Thrombosis.

The range of intracranial venous anomalies in children differs from that in adults. As a commonly encountered highly morbid disease, sinovenous thrombosis has been discussed extensively in the literature, and the associated imaging considerations are similar in pediatric and adult patients. The authors shift the focus to less frequently discussed cerebral venous diseases in pediatric patients. First, the practical embryology pertinent to malformations, syndromes, and variants such as vein of Galen aneurysmal malformation, Sturge-Weber syndrome, and developmental venous anomalies are discussed. Second, anatomic considerations that are applicable to neuroimaging in pediatric patients with cerebral venous anomalies are reviewed. In the discussion of anatomy, special attention is given to the medullary venous system that serves the cerebral white matter, superficial cortical veins (tributaries of the dural venous sinuses), and bridging veins, which carry blood from the superficial cortical veins through the potential subdural space into the dural venous sinuses. Third, the selection of imaging modalities (US, CT and CT venography, and MRI) is addressed, and various MR venographic pulse sequences (time-of-flight, phase-contrast, and contrast-enhanced sequences) are compared. Finally, a broad variety of congenital and acquired superficial and deep venous diseases in children are reviewed, with emphasis on less frequently discussed entities involving the medullary (eg, deep medullary venous engorgement and thrombosis, periventricular hemorrhagic venous infarction due to germinal matrix hemorrhage), cortical (eg, cortical venous thrombosis), and bridging (eg, acute and chronic manifestations of injury in abusive head trauma) veins, as well as the deep veins and dural venous sinuses (eg, varix). © RSNA, 2023 Quiz questions for this article are available through the Online Learning Center. Online supplemental material and the slide presentation from the RSNA Annual Meeting are available for this article.

Idiopathic Intracranial Hypertension and Vascular Anomalies in Chiari I Malformation.

Mounting evidence has suggested a relationship between Chiari I malformation and idiopathic intracranial hypertension, with some studies implicating anomalies of the cerebral venous system in the development of these conditions. However, precise mechanisms explaining these associations are not well described. There is a clear need to clarify the interplay between these conditions to guide further study in this area. In tandem with these efforts, it is necessary to review proper diagnosis and management to improve outcomes in patients suffering from these diseases.

Chiari malformations: not every disability is visible

In Chiari malformation (CM), portions of the brain or spinal cord is pushed down through the foramen magnum, which is located at the bottom of your skull. This causes a portion of the brain to be in contact with the part of the spinal column-usually the spinal cord. The cerebrospinal fluid, however, may be pushed up through the hole making it difficult to detect in this condition. These are a collection of malformations affecting the cerebellum, pons, and medulla oblongata in the posterior fossa and hindbrain. Problems caused by these malformations range from the lack of the cerebellum to the cerebellar tonsils protruding through the foramen magnum, with or without additional intracranial or extracranial anomalies such as hydrocephalus, encephalocele, syrinx, or spinal dysraphism. CMs can be of several types and are more common in females than in males and in children than in adults. This is because they are growing and developing at a rapid rate, which leads them to have a greater amount of skull growth. In this review article, we discuss the various types of CMs, their pathophysiology, clinical features, imaging characteristics, treatment, and why they form an important complex of rare disorders necessary to be diagnosed early.

A study on role of DSA in intracerebral vascular anomaly comparison with computed tomography angiography/magnetic resonance angiography

Background: Brain imaging techniques provide the ability to noninvasively map the structure and functions of the brain. Brain vascular malformation mainly affects people in the 5th decade followed by 4th and 3rd decade. Aims and Objectives: The aim of the study was to compare the diagnostic supremacy of computed tomography angiography (CTA)/magnetic resonance angiography (MRA) and Digital subtraction angiography (DSA) for the detection of intracranial vascular anomalies. Materials and Methods: An observational descriptive study with cross-sectional design was performed among 50 patients of both sexes undergoing DSA test at Medical College and Hospital, Kolkata with acute stroke syndrome or any other symptoms suggesting intracranial vascular lesion, who were investigated with one or more index tests and a reference standard diagnosed by computed tomography (CT) or MR scanning or other parameters. DSA served as the standard of reference for presence of intracranial vascular anomalies. Results: Out of the 50 patients included in the study, 41 were diagnosed with vascular malformations by DSA. Moya moya disease was diagnosed in three and distal AV fistula in six patients. In the 41 patients with vascular malformations, CTA could correctly identify 17 (41.5%) cases whereas MRA could identify 73.2% cases. Conclusion: DSA can be used for both diagnostic and interventional angiography. Its high spatial and temporal resolution have maintained DSA as a very important tool. The study reveal DSA is more superior to accurate angioarchitectural delineation of different intracerebral vascular malformation.

The Course and Neonatal Outcome of Choroid Plexus Extension to the Anterior Horn at the Routine Anatomy Scan.

Our objective was to examine the pregnancy course and immediate neonatal outcome of fetuses with an isolated extension of choroid plexus (CP) to the anterior horn during the second trimester. We prospectively collected the cases referred to us between July 2012 and January 2021 with isolated finding of CP extension to the anterior horn. Relevant clinical and demographic information was recorded, and a full anatomy scan including a comprehensive neurosonogram was performed. In cases of confirmed isolated extension of CP to the anterior horns, women were offered further investigation including fetal MRI, and ultrasound follow up. We collected 29 eligible cases for analysis. The mean gestational age (GA ± SD) for diagnosis and referral was 19.24 ± 2.3 weeks. No other intracranial anomalies were detected in any of the cases, and the finding resolved at 25 ± 2.6 weeks. The average extension length and width to the anterior horn were 0.7 ± 0.3cm, and 0.5 ± 0.1cm, respectively. Eleven fetuses (38%) had choroid plexus cyst (CPC) in addition to the extension. Ten patients (35%) completed a fetal brain MRI, with no identified abnormalities. Gross neurological exam and Apgar score at birth were normal. Extension of CP to anterior horn with or without CPC at mid-trimester seems to have spontaneous resolution with likely a good prognosis and no further implications.

Perinatal observation of large congenital pial arteriovenous fistula before and after surgery: A case report

Pial arteriovenous fistula (PAVF) is an extremely rare type of intracranial vascular congenital anomalies. The presented clinical case is a unique example of intrauterine diagnosis of PAVF using fetal MRI at 30 weeks of gestation, which allowed successful surgical treatment in the early neonatal period. The case demonstrates the capabilities of fetal MRI in the diagnosis of PAVF and estimation of accompanying brain changes, which are fully consistent with the results of postnatal cerebral angiography. Based on neuroimaging data, endovascular embolization of the fistula with detachable microcoils was successfully performed on the 2nd day of the child’s life. A good neurologic outcome of the surgery was stated. Taking into account the known unfavorable outcome of PAVF in the case of untimely surgical treatment, this observation demonstrates the need to use fetal MRI for prenatal differential diagnosis of vascular malformations in order to reduce the risk of possible complications and mortality in the early neonatal period.