Persistent Falcine Research Articles

Dural arteriovenous fistulas in the falx cerebri: case series and literature review.

Dural arteriovenous fistulas (DAVFs) within the falx cerebri are infrequently documented and may be linked with the falcine sinus/venous plexus. The falcine sinus/venous plexus, often regarded as a normal venous structure, can exhibit pathological characteristics, differing from the persistent fetal falcine sinus. A retrospective analysis was conducted at a single center to identify all cases of DAVFs within the falx cerebri spanning from 2002 to 2022. Demographic data, fistula features, treatment modalities, clinical outcomes, and fistula closure were collected and analyzed. Additionally, relevant literature on DAVFs in this location was reviewed. Ten cases were identified at our center, supplemented by 13 cases reported in the literature. In our cohort, patients had an average age of 49.4 ± 8.1 years, with a male predominance of 90%. Trans-arterial embolization (TAE) alone achieved immediate complete occlusion in eight cases, while conservative treatment was pursued in two cases. No treatment-related complications or fistula recurrences were observed. In the literature, seven patients underwent direct surgery, three underwent TAE, and one underwent both direct surgery and radiosurgery for complete fistula closure. No instances of fistula recurrence or treatment complications were reported. Dural arteriovenous fistulas within the falx cerebri are rare, with limited literature available. They typically present as aggressive lesions. Treatment options include direct surgery or TAE. However, due to a lack of long-term DSA follow-up, the cure and recurrence rates are unknown for endovasdcular therapy. Further investigation is warranted to elucidate the involvement of the falcine sinus/venous plexus in falx cerebri DAVFs.

Persistent falcine sinus with associated atretic encephalocele: Diagnosis with ultrasound

Persistent falcine sinus with associated atretic encephalocele: Diagnosis with ultrasound

Atretic cephalocele and differential considerations: A small case series.

Any posterior midline cystic or soft tissue scalp mass in an infant needs evaluation with MRI brain and MRV brain to look for intracranial extension of the lesion. One of the differential considerations is an atretic cephalocele, which can be a difficult diagnosis, especially when the cystic lesions are small. The presence of a small calvarial defect adjacent to the cystic mass and a persistent primitive falcine vein, which points towards the cystic mass, are imaging clues to this diagnosis. Correct diagnosis is needed prior to surgical resection to avoid potentially serious postoperative complications.

Aneurysmal dilatation of vein of galen associated with thalamic arteriovenous malformation and straight sinus agenesis

Straight sinus agenesis is a rare congenital anatomical variation that is usually accompanied by persistent falcine sinus. Concomitance of straight sinus agenesis and absence of falcine sinus are associated with thalamic arteriovenous malformation by the disturbance of the venous flow. In this report, a very rare case of aneurysmal dilatation of the vein of Galen associated with thalamic arteriovenous malformation and straight sinus agenesis is presented.

Sinus Pericranii associated with Hypoplastic Straight Sinus and Persistent Falcine Sinus

Sinus Pericranii associated with Hypoplastic Straight Sinus and Persistent Falcine Sinus

A persistent falcine sinus in a child with venous sinus thrombosis.

A persistent falcine sinus in a child with venous sinus thrombosis.

The spectrum of venous anomalies associated with atretic parietal cephaloceles: A literature review.

Background: Parietal atretic cephalocele (PAC) is a small, subscalp lesion with underlying extracranial meningeal, neural, and glial tissues. In this paper, we analyze the related literature on the continuum of PAC-associated venous anomalies and report an exemplary case.Methods: The PubMed Medline database was searched using the following search algorithm: (Atretic encephalocele) OR (Rudimentary meningocele,) OR (Atypical meningocele) OR (Meningocele manqué) OR (Meningeal heterotopia). Only papers detailing the venous anomalies associated with PACs have been included.Results: A total of 30 papers in our search documented PAC-associated venous abnormalities. The overall number of cases reported was 68 (including our exemplary case). The most frequently identified associated venous anomaly was the presence of a “fenestrated superior sagittal sinus” recorded in 48.5% of cases (n = 33), followed closely by “persistent falcine sinus” in 47% (n = 32) and vertical embryonic positioning of the straight sinus (SS) in 44% (n = 30). The complete absence of a SS was reported in 39.7% (n = 27) and various anomalies of the Galenic system were reported in 26.8% of cases (n = 12).Conclusion: Although benign in nature, PACs are often a marker for the presence of complex and variable cerebral venous malformations, requiring extensive preoperative imaging workup for both the superficial and deep venous systems to obtain an accurate understanding of the anatomy of the venous system and guide surgical planning.

Foramina parietalia permagna: descrizione di un caso clinico e revisione della letteratura

The purpose of the present work is to expose the defects of parietal bone ossification and to identify the criteria for differential diagnosis and brain changes related to the condition, with particular attention to the venous developmental anomalies and the pathological features associated. Foramina parietalia permagna (FPP) are caused by an insufficient intramembranous ossification around the parietal notch that is normally obliterated in the fifth month of normal foetal development. During the first few years of life as calvarial growth continues, cranium bifidum tends to resolve into two distinct, large parietal foramina. Most people with FPP have a positive family history as the condition is inherited in an autosomal dominant fashion with high, but incomplete penetrance. Mutations of either MSX2 or ALX4 genes are associated with enlarged parietal foramina. Meningeal cortical vascular malformation of the straight sinus and persistent falcine sinus have also been reported in the literature as possible associated anomalies.

Chiari I malformation with craniosynostosis and persistent falcine sinus draining into the straight sinus

Owing to the interconnected nature of the brain, anatomical variations in one area often coincide with, or are caused by, abnormalities in another. During dissection of a specimen with both Chiari I malformation and craniosynostosis, a persistent falcine sinus was observed to drain into the straight sinus. Such a variant should be noted by physicians as it could alter treatment plans and require more detailed imaging procedures prior to surgical correction. Herein, we report the case and discuss the possible embryological origins and clinical significance of the variant.

Variations of Intracranial Dural Venous Sinus Diameters from Birth to 20 Years of Age: An MRV-Based Study.

The role of the dural venous sinus system in cerebrovascular pathology and the understanding of normal developmental patterns and sizes of the dural venous sinus system continue to expand. The purpose of this study was to review MR venograms to elucidate developmental patterns and diameters of the major dural venous sinuses from 0 to 20 years of age. All available MR venograms of patients 0-20 years of age who presented to our institution were retrospectively reviewed. Patient age at the time of image acquisition was noted, and measurements were taken of the diameters of the major dural venous sinuses. The presence of embryonic sinuses including the persistent falcine sinus and the occipital sinus was noted. Dominance patterns of the transverse sinus system were determined. Mean diameters of each sinus were plotted as a function of age. The prevalence of persistent prenatal sinuses and transverse sinus-dominance patterns was compared across ages. A total of 429 MR venograms from 429 patients were reviewed. All dural venous sinuses demonstrated a maximal growth rate from 0 to 7 years of age and reached maximal diameters around 5-10 years of age. The prevalence of falcine sinuses and occipital sinuses trended downward across increasing age categories (P = .09 and, <.0001, respectively). Dural venous sinuses demonstrate maximal growth between 0 and 7 years of age and reach adult size around 5-10 years of age. Involution of the prenatal sinuses continues to take place after birth into childhood but is largely absent in early adulthood.

4q27 deletion and 7q36.1 microduplication in a patient with multiple malformations and hearing loss: a case report

BackgroundChromosome deletions of the long arm of chromosome 4 in 4q syndrome are characterized by mild facial and digital dysmorphism, developmental delay, growth retardation, and skeletal and cardiac anomalies, which is regarded as an autism spectrum disorder. Moreover, some scarce reports indicate that patients with 4q interstitial deletion and 7p duplication may present symptoms associated with hearing loss.Case presentationA boy with a severe developmental delay not only post-natal but also intrauterine and several dysmorphic features including microcephaly, ocular hypertelorism, exophthalmos, low-set ears, single palmar flexion crease, and overlapping toes presented discontinued cyanosis and recurrent respiratory infections. MRI, BAEP, echocardiogram and bronchoscopy revealed that he had persistent falcine sinus with a thin corpus callosum, left auditory pathway disorder, patent foramen ovale (2 mm), and tracheobronchomalacia with the right superior bronchus arising from the lateral posterior wall of the right main bronchus. Finally, the patient died with severe pneumonia at 10 months. Array CGH revealed a 23.62 Mb deletion at chromosome 4q27, arr [hg19] 4q27-q31.21 (121, 148, 089–144, 769, 263) × 1, and a 0.85 Mb duplication at chromosome 7q36.1, arr [hg19] 7q36.1-q36.2 (152, 510, 685–153, 363,5 98) × 3. It is rare for 4q syndrome cases or 7q duplications previously reported to have a hearing disorder, pulmonary dysplasia, and pulmonary arterial hypertension.ConclusionsThe phenotype of our patient mainly reflects the effects of haploinsufficiency of FGF2, SPATA5, NAA15, SMAD1, HHIP genes combined with a microduplication of 7q36.1.

Abstract TP407: Transvenous Pressure Monitoring Guides Endovascular Treatment of Vein of Galen Malformation

Background: Vein of Galen Malformation (VGM) is a complex vascular disorder with high morbidity and mortality. Mainstay management involves staged endovascular embolizations. Determining when to stop an embolization during any single intervention is challenging. If embolization is excessive, it precipitates thrombosis on the VGM, increase in cardiac post-load, and worsening on heart failure, as well as hemorrhages. Conversely, insufficient embolization may be futile. We report the use of transvenous pressure monitoring as an adjuvant for guiding the extent of embolization. Method: Arterial and venous accesses were obtained through the common femoral artery and vein, respectively. After diagnostic angiography, the best working projection was selected in early and late venous phases. A microcatheter was placed at the VG, and an intermediate 5 Fr. catheter was located at the persistent falcine sinus. The microcatheter was used for coil embolization, while the intermediate catheter provided support and was connected for continuous venous pressure monitoring. Continuous arterial pressure monitoring was obtained via an A-line and transduction of a glide catheter located in the carotid artery. Arterial and venous SBP were recorded. Result: Serial angiograms during treatment showed reduction of flow within the VGM. Concomitantly, there was an increase in the A-V-gradient over time (Figure 1). The difference between the A-V SBP went from 36 mmHg at the start of the case to 51 mmHg after the final coil. The average pressure gradient went from 38.3 mmHg in the first quarter to 43.5 mmHg in the last. Conclusion: The continuous assessment of transvenous pressure monitoring during embolization of VGM provides measurable changes that correlate with degree of flow. This quantifiable metric may be used as an adjunct to guide adequate reduction of flow during endovascular treatment of VGM.

A review of extraaxial developmental venous anomalies of the brain involving dural venous flow or sinuses: persistent embryonic sinuses, sinus pericranii, venous varices or aneurysmal malformations, and enlarged emissary veins

This paper is a narrative review of extraaxial developmental venous anomalies (eDVAs) of the brain involving dural venous flow or sinuses: persistent embryonic sinuses, sinus pericranii, enlarged emissary veins, and venous varices or aneurysmal malformations. The article highlights the natural history, anatomy, embryology, imaging, clinical implications, and neurosurgical significance of these lesions, which the authors believe represent a continuum, with different entities characterized by distinct embryopathologic features. The indications and surgical management options are discussed for these individual intracranial pathologies with relevant illustrations, and a novel classification is proposed for persistent falcine sinus (PFS). The role of neurointervention and/or microsurgery in specific cases such as sinus pericranii and enlarged emissary veins of the skull is highlighted. A better understanding of the pathophysiology and developmental anatomy of these lesions can reduce treatment morbidity and mortality. Some patients, including those with vein of Galen malformations (VOGMs), can present with the added systemic morbidity of a high-output cardiac failure. Although VOGM is the most studied and classified of the above-mentioned eDVAs, the authors believe that grouping the former with the other venous anomalies/abnormalities listed above would enable the clinician to convey the exact morphophysiological configuration of these lesions, predict their natural history with respect to evolving venous hypertension or stroke, and extrapolate invaluable insights from VOGM treatment to the treatment of other eDVAs. In recent years, many of these symptomatic venous malformations have been treated with endovascular interventions, although these techniques are still being refined. The authors highlight the broad concept of eDVAs and hope that this work will serve as a basis for future studies investigating the role of evolving focal venous hypertension/global intracranial hypertension and possibilities of fetal surgical intervention in these cases.

DEVELOPMENTAL PERSISTENT FALCINE SINUS WITH CRANIUM BIFIDUM OCCULTUM, CRANIOSYNOSTOSIS, AND ASSOCIATED ANOMALIES: A UNIQUE CASE

The falcine sinus is an intrauterine anatomic structure located in the falx cerebri that is closed after birth and persistence of thisembryologic falcine sinus is considered as a rare variation of the venous pathway which is associated with defect in the developmentof the straight sinus leading to formation of an alternate venous pathway served by the persistent falcine sinus. Cranium bifidumoccultum is another rare skull ossification disorder referred to as the Catlin mark characterized by ossification defects in theparietal bones. Many other associated anomalies can be seen with persistent falcine sinus apart from cranium bifidum occultumwhich include absent or dysplastic tentorium cerebelli, agenesis of the corpus callosum, apert syndrome, atretic occipital/parietalencephalocele, vein of galen malformation, osteogenesis imperfecta, and chiari malformation Type II. We describe a rare case of a10-year-old girl who presented with short stature, intermittent headache, and abnormal skull shape. On radiological examination,persistent falcine sinus was detected with large midline skull defect at the high parietal area. The straight sinus was absent, and therewas dysplastic tentorium cerebelli. In addition to these, craniosynostosis was also present with many other associated anomalies.Persistent falcine sinus and cranium bifidum occultum are very rare, and when found, they are associated with many anomaliesraising the possibility that they may represent the benign end of the same developmental spectrum. To the best of our knowledge,these constellations of anomalies have been reported in very few children.

Falcine Sinus: Incidence and Imaging Characteristics of Three-Dimensional Contrast-Enhanced Thin-Section Magnetic Resonance Imaging.

ObjectiveTo evaluate the incidence, characteristics, and variations of the falcine sinus with contrast-enhanced three-dimentional (3D) thin-section magnetic resonance (MR) images.Materials and MethodsA retrospective review identified 1531 patients (745 males and 786 females, 2 months to 85 years) who underwent cranial MR imaging including T1-weighted imaging, T2-weighted imaging, T2-weighted fluid-attenuated inversion recovery, contrast-enhanced 3D thin-section sagittal scans, and MR venography, from June 2014 to January 2016. The incidence, characteristics of the falcine sinus, and coexisted intracranial lesions were confirmed by two neuroradiologists.ResultsFalcine sinuses were identified in 81 (38 males and 43 females) cases (5.3%, 81/1531, 5 months to 76 years of age) with calibers ranging from 2.3 mm to 17.0 mm. Three major forms of falcine sinuses were defined: arch-like (n = 47), stick-like (n = 22), and bifurcated (n = 12). Persistent falcine sinuses were found in 57 cases, among which 3 cases showed complicated cerebral anomalies, and 2 cases showed smaller straight sinuses. Recanalization of falcine sinuses were found in 24 cases, including 17 cases with tumor compression, 6 cases with cerebral venous sinus thrombosis, and one case with hypertrophic meningitis.ConclusionFalcine sinus is not as rare as has been reported previously. Most falcine sinuses are not associated with congenital cerebral abnormalities. Diseases that cause increased pressure in the venous sinus may lead to recanalization of falcine sinus. Illustrating the characteristics of falcine sinus may prompt a more comprehensive understanding and diagnosis of associated diseases, and avoid potential surgical damage in the future.

Rare combination of a persistent anterior falcine sinus with developmental venous anomaly: A case report and literature review

Abstract A 21-year-old woman was admitted with a 1-day history of headache. Her neurological examination findings were normal. Computed tomography angiography revealed an oval, well-enhanced dilated vascular lesion in the anterior interhemispheric fissure with a developmental venous anomaly characterized by caput medusae centripetally in the right hemisphere. On the magnetic resonance image, an anterior persistent falcine sinus was connected with the middle portion of the superior sagittal sinus. Cerebral angiography revealed the persistent falcine sinus located in the anterior falx and the parenchymal developmental venous anomaly with caput medusae. In addition, the venous phase of cerebral angiography demonstrated the obstruction of both the inferior sagittal sinus and the bridging vein between the superior thalamostriate and the internal cerebral vein. To our knowledge, this is a very rare case of the combination of an anterior persistent falcine sinus and a developmental venous anomaly.

Vein of Galen malformation presenting as congestive heart failure

A 2.5-month-old boy presented with failure to thrive and an increasing head circumference since birth. Clinical examination revealed features of congestive cardiac failure. Multiplanar magnetic resonance imaging (MRI) of the child was performed on 1.5 T MRI scanners (Magnetom Sonata, Siemens, Germany) with dedicated head coil. MRI shows the markedly enlarged median prosencephalic vein of Markowski, draining into the sagittal sinus, via a persistent falcine vein; with absence of the straight sinus. The dilated veins show flow void on spin echo sequences, characteristic of vein of Galen malformation (VOGM). There was no hyperintense signal within these dilated veins to suggest thrombosis. Arterial feeders are seen along the anterior wall of the vein. A complex arterial maze is well seen on the magnetic resonance angiography, with arterial feeders arising from the perforating branches of right middle cerebral artery. Bilateral cerebral parenchyma was otherwise normal (Figs 1‐4). Patient was referred to pediatric cardiology unit of the hospital where he was initially managed with medications like diuretics to control the congestive cardiac failure and is currently doing well on diuretics and angiotensin-convertase enzyme inhibitors.

An Uncommon Presentation of a Rare Disorder - Ruptured Vein of Galen Malformation with Atrial Septal Defect

Vein of Galen aneurysmal malformations (VGAMs) are rare intracranial anomalies constituting 1% of all the cerebrovascular malformations. Its association with congenital heart disease has been described. Most reported cases are in the postnatal period associated with cardiac failure. Rupture of this malformation is uncommon. Here we describe a case of an otherwise healthy 25 year old presenting with sudden change in his mental status. Unenhanced CT brain showed intracranial hemorrhage secondary to rupture of the VGAM. Cerebral angiography showed stenosis of a persistent falcine sinus, which likely delayed his presentation. Endovascular embolization of the varix was performed. Echocardiogram showed a large sinus venosus ASD with anomalous pulmonary venous return. He sustained residual neurological deficits and is currently undergoing rehabilitation. This is a rare case of delayed presentation of this disease despite the underlying cardiac defect, with an uncommon complication of rupture and an unfortunate outcome.

Prevalence of persistent falcine sinus as an incidental finding in the pediatric population.

Persistence of the embryologic falcine sinus can be a congenital or acquired phenomenon related to a defect of the straight sinus. Previous studies have stated that it is rare for the falcine sinus to persist without an additional abnormality of the brain such as a vein of Galen malformation or venous sinus thrombosis. The purpose of this article is to show that persistent falcine sinus is not an uncommon isolated finding in the pediatric population. We performed a retrospective review of pediatric brain MRIs to identify patients with a persistent falcine sinus and subsequently recorded whether there were findings in addition to the straight sinus defect. Thirty-one patients were identified as having a persistent falcine sinus, five of whom had no other congenital or acquired abnormality of the brain other than a defect in the straight sinus. Our data support the hypothesis that persistent falcine sinus is not an uncommon isolated finding in the pediatric population.

MRI findings of intracranial anomalies associated with cephalocele—a case series

Cephalocele is a relatively rare cranial dysraphism characterised by herniation of intracranial structures through the skull. Surgical management is primarily necessary where a risk of infection through communication of the lesion with the intracranial space exists, a risk of rupture, or for cosmetic purposes. Cephalocele is often associated with venous anomalies such as vertical embryonic positioning of the straight sinus, splitting of the superior sagittal sinus, vein of Galen elongation, along with tenting of the tentorium [Morioka et al. Childs Nerv Syst 25:309-315, 2009] Here, we report four cases of cephalocele with pre-operative MRI imaging retrospectively studied, demonstrating associated venous anomalies. Three of these patients went on to have uncomplicated, corrective surgery, while one was managed conservatively. All four cases demonstrated the main venous drainage going through a persistent falcine sinus to drain into the superior sagittal sinus. Upward tenting of the tentorium was observed in three cases (cases 1, 3 and 4). Two of our cases demonstrated other venous anomalies frequently reported in the literature, namely splitting of the superior sagittal sinus and absence of the transverse sinus (case 1) and communication of the cephalocele with the superior sagittal sinus and absence of the straight sinus (case 2). The association between cephalocele and venous anomalies suggests that pre-operative MRI should be mandatory for a full evaluation of a suspicious midline cranial lesion in order to evaluate the safety of corrective surgery.